Journal History

Click here to view older journal entries (diagnosis through transplant)

Monday, July 7, 2008 3:05 AM CDT

Just a quick note to let you know that no news is stable news, with, of course, a handful of hiccups thrown in for good measure. Much to report, yet not much has changed. An update is in the works ...

Thanks, as always, for your unending support, even amid our annoying silence.

Love,

The Whites

Monday, June 2, 2008 3:03 AM CDT

HAPPY 39th BIRTHDAY KLANE - the best daddy, husband and friend a girl could ever ask for!

WE LOVE YOU,

Your 3 Girls


Sunday, June 1, 2008 10:58 PM CDT

Hello Again,

Miss Susannah continues to make nice progress on the ventilator. Things have moved a bit slower over the weekend due to some issues with the CRRT circuit (dialysis), but she has continued to move forward with decreased vent settings nonetheless. Baby steps.

We did have a big hiccup mid-week with a bout of bleeding from Susannah’s stomach. In a matter of 24 hours, her red blood cell count dropped an alarming eleven points (a far more rapid fall than we typically see with blood draws and hemolysis). Without getting too graphic, Susannah had an enormous emesis, immediately followed by diarrhea, which was suggestive of a GI bleed. On Thursday she had an endoscopy, which revealed several polyps in her stomach, one of which was bleeding significantly. This was cauterized and samples were sent to pathology. Thus far, her blood counts have stabilized and she is doing well. As best we can tell, the nasogastric (NG) tube that was inserted when she went on the vent, agitated these polyps, actually knocking one off, causing it to bleed.

In conjunction with the endoscopy, Susannah also had a high-resolution ultrasound done to insure the bleeding had nothing to do with her recent splenectomy. Findings on the ultrasound prompted the team to follow-up with a CT scan to further access the situation. Seeing that she is already intubated, we decided this would be a good opportunity to also reassess her lung nodules. So on Friday night, Susannah and her entourage (including the ICU attending – leave it to Amy to insist), paraded down the hall to radiology. Too bad she snoozed through the whole trip because taking a ride in the bed is one of our girl’s all time favorite hospital pastimes! For the most part the CT checked out fine.

The good news is that despite the bleeding episode, not to mention the recent VRE sepsis (by the way, she’s been fever free since starting the appropriate antibiotics), Susannah has continued to show steady improvement on the lung front. The lung CT showed what we are interpreting to be significant improvements in the appearance of her fungal nodules. We will, of course, follow her lead, but as long as she is tolerating it, the goal is to press on with lowering her vent settings as expeditiously as possible, in spite of the other extraneous medical issues that persist. The chief goal at the moment is to get her off the vent!

Susannah’s CRRT has gone far more smoothly than anyone could have hoped. These circuits are known for clotting, getting infected and wreaking all sorts of other havoc with the dialysis process. Susannah's course, up until now, has been unflawed. However, on Friday, literally a minute before we were due to remove her from the circuit for a routine filter change, Susannah’s catheter clotted off. While ultimately remedied several hours later, this resulted in a good deal of hoopla and stress getting things back up and running. Since then, her catheter pressures have been a good bit more finicky requiring quick attention and coddling. The renal team assures us that this is the norm and that actually most kids would have already had their catheters replaced by now. Good to know, that Susannah has had some streak of luck.

On the hematological front, it has been a promising week. Today, Susannah went a record 36 hours between platelet transfusions, made even more impressive in light of the fact that we’ve been keeping her transfusion threshold at 75,000 because of the recent bleeding episode. Her red cells are also holding on nicely, in spite of frequent blood draws, and we now find ourselves asking if the polyps in her tummy could have been a contributing factor in her falling hematocrit these last few months. Susannah’s white blood cell count has significantly decreased since we began treating the VRE, combined with the hydroxyurea therapy, and in fact, the more worrisome cells present in her total white count, have shown marked decline.

In Madeline news, we took a trip to the hair salon this weekend where she had TEN inches cut from her beautiful blonde hair, which have since been donated to Locks of Love. She is now sporting an adorable sassy bob, great for summer! The ballerina decided to capitalize on the moment, knowing she will not need the perfect bun again until her next stage performance in December. End of school year festivities are officially underway, ramping up for the next couple of weeks and culminating in a special 5th grade graduation ceremony … believe it or not, Madeline will be heading to middle school next year!

All in all we’ve been blessed with a quiet and calm weekend here in the PICU. Susannah especially enjoyed having Grammy sing to her and her big sister read her stories, particularly the new Fancy Nancy Bonjour Butterfly book that Madeline brought her from the school book fair. Madeline and Mommy were treated to a pedicure when a dear friend arranged for a local salon to come spoil us at the hospital. It was quite a sight when the doctors came by for evening rounds and found the girls with their feet up! Fear not, we shared the wealth with Susannah, who is now also perfectly painted with pretty pink polish on both her fingers and toes. The coming week brings about a grandmother swap, as well as Klane returning to clinical work. We continue to take each day as it comes. Baby steps.

Much love,

The Whites

PS. Congratulations to our nephew and cousin, Anthony, who graduated from high school this weekend. We wish we could have been there to help you celebrate!


Tuesday, May 27, 2008 11:19 PM CDT

Greetings from the PICU, where yesterday we actually hit the 50 day mark. Time flies, I guess. The first 4 weeks up here were spent with a spunky Susannah, on BiPAP, awaiting surgery. Our girl is now nearly 3 weeks out from the removal of her spleen and gallbladder, very comfortable and sedated on the ventilator. Although she is heavily medicated, she does have periods of wakefulness and will engage us with her beautiful brown eyes, listen to stories, music or even watch an occasional video … until her lids again become heavy and she drifts back off. Many of her teachers and friends visit regularly and go through their usual routines, which is really fabulous for her morale. Today her clown pals came with their squeaky “butt horn” and honked her some songs. She also had a visit from the hospital music therapist, who was even able to help her move her hand to play the “omnichord” while he sang several songs, including, of course, “Oh! Susannah”.

With our most recent updates primarily focused on the surgery and with current circumstances being relatively mellow, it is probably a good time to step back and recap just how we got here … to the PICU and to the hospital in general for that matter … and at the same time provide a little glimpse into the world of Susannah. For some of you this will likely be redundant, but for others, due to our being so out of touch these last months, it will hopefully help to fill in the gap.

Since moving to Seattle almost two years ago, Susannah’s GVHD, as manifested by hemolytic anemia, has progressively become a much more difficult issue to deal with. This is likely due to decisions that were made regarding her medication regimen upon our arrival here (a whole story, in and of itself). Because of this, we had to go at her with a series of treatments that further suppressed her immune system. Subsequently, we started noticing, about this time last year, that she was having difficulty holding onto her platelets and she also began to require frequent intervention (GCSF) in order to keep her neutrophils in a safe range. As the summer progressed both of these problems became more and more significant, with the anemia issue further revving up as well. In what at the time seemed to be unrelated, Susannah’s oxygen saturations were also becoming a little sluggish at night.

As a result of these developments, last August, Susannah had a bone marrow biopsy, which showed her to not be making platelets and CT scan of her chest, which revealed two nodules consistent with a fungal infection. To be absolutely sure of what these nodules represented, Susannah, on August 15, underwent a needle biopsy, which confirmed the fungal infection (Aspergillus for those of you interested in the specifics). She was admitted after the procedure, for what was meant to be a one-day admission, and has yet to leave the hospital. Her story has been even more complicated and convoluted since then. Fortunately, the fungal infection showed immediate improvement upon starting the latest and greatest antifungals. However, she has had progressive worsening of her lung function, which seems to be entirely independent of the fungal infection (the exact etiology remains a puzzle). Concurrently, she also began to have increasing transfusion requirements, both of red blood cells and platelets. Her previously plummeting white count became increasingly erratic, shooting both up and down in a crazy, and completely unexplainable fashion.

Beginning in January, she started having recurring bouts of abdominal pain, which further compromised her ability to take deep breaths. This was thought to be related to “sludging” and stones in her gallbladder and resultant inflammation in her pancreas. By early February, it became very clear that Susannah’s spleen was enlarging at a visibly appreciable rate. The concern with this was several-fold. Firstly, it could have been a site for red cell and platelet sequestration. Secondly, as her spleen enlarged it was starting to act as a physical barrier to Susannah taking full, deep breaths. Lastly, there was likely a component of stomach discomfort with this big, football-sized organ sitting in her little abdomen. Given this constellation of issues, that is how we came to the decision for surgery to remove her spleen and gallbladder.

By early April, while waiting for the surgery to be scheduled, Susannah was having more and more difficulty breathing related to the stints of abdominal pain and thus was transferred to the PICU, where she was put on BiPAP. For those of you not familiar with BiPAP, this is a pressurized mask that helps facilitate good, deep breathing. Given her somewhat tenuous situation at the time and because it is typically best to exercise the utmost caution when dealing with Miss Susannah, she remained in the PICU for monitoring and adjustments to the BiPAP. Up until the day before surgery, Susannah tolerated the BiPAP extraordinarily well, keeping up most of her usual activities in spite of the mask. She’d happily work her puzzles, participate in school and to the shock of all of the staff, the girl would even eat and drink! The BiPAP clearly made her feel good and thus she tolerated it marvelously. This compliance came as a shock to many, particularly the many who’d spent the previous nine months trying to convince Susannah that using a nasal canula, as opposed to a bulky face mask or cumbersome blow-by oxygen would be a good thing – she would have no part either! Folks were so elated with her compliance that the BiPAP was affectionately deemed “Snuffy” (short for Sesame Street’s Snuffleupagus – a favorite of Susannah’s) and Susannah wore it proudly … and actually looked pretty darn cute in the contraption. The nurses, RTs and even the doctors referred to “Snuffy”, in the room, on the phone and even in rounds!

As we alluded to in our previous postings, Susannah’s spleen grew, her pain increased and as surgery drew near, she was really struggling to keep up from a pulmonary standpoint, slowly becoming truly uncomfortable. It seemed that her spleen was literally growing with every single blood product she received, and she was receiving many. So the day prior to her scheduled surgery, we decided to put her onto the ventilator. Surgery was then delayed by two days due to a surprise infection in her central line, but this was likely a blessing in disguise, as Susannah really did seem to improve from being on the ventilator, both from the standpoint of breathing and in terms of her discomfort. At the same time, with the start of a new med (hydroxyurea), we also saw a marked decrease in her elevated white count (which had reached an all time, nerve wracking high), an added safety feature heading into surgery.

During these long months, even with everything going on, little Susannah has continued with a happy go-lucky, albeit somewhat peculiar, lifestyle. Up until the day before she went on the ventilator, she’d wake up bright and early each morning to greet her teacher with a smile – even when her mother wasn’t yet smiling at 8am! This was typically followed by several more teachers and therapists throughout the day. The hospital volunteer corp has also come out in full force, with frequent visits from the music and art therapists, a variety of musicians, and of course, the crazy clowns. Aside from charming every adult she encounters, Susannah has also befriended several of the other children on the transplant unit. Whether group music class, afternoon strolls or PT in pairs on the stairs, the kiddos really have developed a bond. Big kids, little kids, it makes no difference, Susannah loves them all the same. And they love her. One little girl, also admitted last August, decided she did not think Susannah should have to push around a big wagon full of her babies, so for Christmas she gave Susannah a fancy pink baby stroller! When a baby sister was born to one of the kids on the unit, Susannah decided this baby needed to be her own personal doll, so the mom would bring her out to see Susannah every day and boy did Susannah want to put that baby in HER stroller!

Speaking of, Susannah is famous for her daily and nightly walks around the hospital pushing said stroller, and yes sometimes the entire wagon, garbed in a variety of tutus, tiaras and fancy shoes. Her regular outings with pals and her primary nurses are a huge part of her routine. The adventures vary from short strolls around the unit with her dolls and various animals in tow, to hour long treks all over the campus, at times even venturing outdoors, or visiting the Mickey Mouse and Pinocchio statues in the outpatient center (always taking time for the ritualistic stepping on Mickey’s toes!). In the event of a low energy day, the alternative of lounging in the wagon, or a ride on the bottom of her IV pole provide nearly as much excitement. Susannah’s all time favorite trip is her late night rendezvous to the snack machine to choose the perfect bag of chips. We have gone through countless rolls of quarters as Susannah has contemplated exactly which snack she wants from the vending machine!

It is important to point out that many of Susannah’s daily rituals have been made possible by her own very special team of nurses, who dote on her and indulge her every whim. They each further spruce up her routine in their own special ways, and believe me, Miss Susannah has her own specific expectations from each one of them. Wrapped around her finger that they are, they always indulge! Whether this is conjuring up materials for leis, following Susannah’s repeated requests to watch hula girls, dressing dollies in crazy outlandish outfits, dressing the nurse in crazy outlandish outfits, repeatedly doing Dora or Cinderella puzzles (which we all can now do in our sleep), or letting her splash in the bath until the floor is an inch deep in water - this has been quite entertaining, as Susannah will gleefully douse anyone in the line of fire with wave after wave of bathwater.

Thanks to some special friends from both near and far, Susannah’s room has been decorated to the nines for every holiday, big or small. We trick-or-treated around the unit in full costume (she made an adorable Dorothy, with Madeline as the perfect Glenda) and managed enjoy a true Thanksgiving feast right in the hospital room. We’ve tried to maintain some lighthearted fun with New Year’s, Mardi Gras and St. Patrick’s Day celebrations. Both girls even attended their first “prom”, complete with fancy dresses and hair-dos, when the unit held a prom for one of the teenage girls who was going miss out on her own prom because of being hospitalized. Both Santa Claus and the Easter Bunny managed to sneak their way into the room, despite Big Sister actively guarding the stockings and baskets as she slept on a pallet next to Susannah’s bed. Thanks to Madeline’s fabulous letter to Santa, Susannah was granted a four-hour pass for Christmas, and Easter, when we took her home for short visits and the ever popular car rides there and back.

That finally brings us back to where we are now. Change has been slow over the last week or two, but for the most part in the right direction. Susannah is much more svelte these days, and has almost attained her fighting weight of about 15 kilos. The CRRT (dialysis) has gone as smoothly as it could possibly go, so this has been very encouraging. Her lungs, on the other hand, have been slow but sure in their improvement. She has definitely taken a circuitous route in this regard (but really, does this child know any other way?). Overall though, we have been able to make appreciable gains in decreasing her vent settings. At one point there was discussion about a bronchoscopy, but as long as she has remained stable and does not regress too much, we have been reluctant to proceed with this. If all goes well, Susannah should remain on the vent for at least a couple more weeks, and the duration of the CRRT remains an unknown, but should also last at least a few more weeks, depending on her ability to pee.

Her white count remains high, but has stabilized. We reinitiated the hydroxyurea after about a week’s hiatus, which has kept her counts in check. She perhaps would be making even more headway, but has battled near persistent fevers over the last week and just yesterday presented with a new positive blood culture, which we now know represents a blood stream infection with vancomycin resistant enterococcus (VRE). We also had a scare last week with a flare in the levels of her long dormant cytomegalovirus (CMV). This actually first arose the night before surgery (not to be confused with the line infection that cropped up the day prior), but immediate start-up of antiviral meds quickly got this under control. However late last week, after a steady decline in her CMV titers for the previous two weeks, her levels suddenly shot through the roof. After much deliberation, paranoia and hand-waving, it was determined that the CRRT machine was actually filtering out the bulk of the antiviral medication. The dose was promptly doubled and lo and behold, her numbers dropped back down in a strikingly fast manner. This has provided us all with a huge sigh of relief.

Her platelet transfusions are back to averaging once a day (which is much improved compared to her perioperative time). Considering her recent fevers, this is actually quite a feat as typically a single fever can cause her to chew through a batch of platelets in no time flat. Regarding her red blood cell needs, this seems to be improved, but is difficult tell because she has had a significant amount of blood drawn for her recurrent cultures needed for the fevers, not to mention the multitude of daily labs required for CRRT.

On the home front, Madeline and I were able to get out last weekend and plant our summer vegetable garden, while this weekend I managed to get most of the summer annuals in the ground. These projects amounted to about 10 hours of normalcy for us. Amy and I have gotten out once or twice as well, as there have been several end-of-the-year school events for Madeline. We took advantage of these opportunities to get dinner as well, while the grandmothers and the one to one PICU nurse looked after Susannah. Speaking of, Grammy (Jackie) remains on the job keeping the house running and managing Madeline’s affairs. Grandma Dona returned home last week with plans to return in June. Their help has been enormous and much appreciated.

For now, Susannah continues to remain sedated, but very comfortable. Consequently, her peacefulness has allowed us to remain remarkably at ease through this period as she slowly but steadily improves, however the long-term concerns still hang over us. We do miss her smiling and laughing, and eagerly await the return of “little miss personality”.

Our warmest appreciation to all,

Klane, Amy, Madeline and Susannah

Thursday, May 15, 2008 7:10 PM CDT

Good Evening,

Well, despite everyone’s valiant efforts in praying for pee, Susannah has just not been going enough to keep up. This is likely from a host of nephrotoxic medications that she has received over the last month, since being in the PICU. Her kidneys are NOT shot, however; we have been assured of that. They can recover, but will need a little extra help for the moment. Consequently, we placed a dialysis catheter in her leg yesterday (all in her room) and last night she started on a special kind of gentle and temporary dialysis (CRRT). When things finally got started she weighed in at 21.5 kilograms, up from her baseline 15.5. She is pretty much a little, or rather, a big marshmallow. The renal docs want to move relatively slowly, but still predict that we can get her back to her baseline in a relatively short time, perhaps in about a week (nice diet, eh?). The trouble is, kidneys can be sort of stupid, so once you help them out, there is a pretty high likelihood that they will then (temporarily) forget how to do their job on their own. Thus, it is expected that once the fluid is off, she will still need to be dialyzed to some degree for a couple of weeks to months. No one knows for sure how long. The good news is that once most of the fluid is removed, we can go to work on weaning the ventilator. If this is tolerated, she can actually be extubated with the dialysis still on board.

The nephrologists have been a very optimistic addition to Susannah’s team. They believe that Susannah’s kidneys are not permanently damaged and that the present situation is reversible. This hospital tends to be very aggressive with the use of this type of dialysis (we’ve been told they are the third most frequent users of CCRT in the country) and has much success. On the flip side, with Susannah’s main Seattle doc out of town and the hem/onc physician on service this week not terribly familiar with the in and outs of our girl, there has been a fair amount of naysaying going on. Although he never outright objected to proceeding with dialysis, he pretty much dragged, and redragged us through the multitude of other issues that we are up against, unnecessarily reminding us that we are between a rock and a hard place. None of this, of course, was news to us, but nonetheless, he seemed to feel it his obligation to rehash these issues as we moved forward with the next step. This did not do a lot for our morale, but we remain steadfast in believing that we know what is best for Susannah. Fortunately, the current ICU attending is very in the loop and has taken over the helm, this week, even signing on as one of Susannah’s continuity people. He is awesome, a tremendously thoughtful and calming individual. We have grown quite fond of him and his uncanny insight into patient care, and our situation in particular. He has been incredibly patient and spent a great deal of time with us and our girl. He has definitely helped keep us at ease with this latest decision to proceed with dialysis. It is nice to have the moral and intellectual support that he provides.

With all that said, the decision to start dialysis was pretty easy for us. We certainly are not to the point of giving up on Susannah, and without the dialysis, we are pretty much stuck in the mud. All in all, there continues to be much reason to be optimistic. All of her issues remain addressable. We just need to continue with the proverbial one step at a time. Susannah is comfortable and very relaxed. We really do think the surgery helped her in that regard. She also continues to go on without any fevers and she seems to be holding onto her red cells quite well. Although premature to say with confidence, there are certainly signs that her hemolysis has slowed by a significant degree.

In other news, platelets are still an issue, but the dust has yet to settle. Today was the first day that the surgeon allowed us to drop her parameters – he had us keeping her greater than 100,000 in the first five postoperative days. Now we have decreased the threshold to 50,000, and we are hoping for longer stints between transfusions. She is certainly in a much better place than she was pre-op, considering that for the few days prior to surgery she was needing platelets every couple of hours, sometimes with barely even a break between transfusions. Our hope is that she can now equilibrate at a lower threshold. One doesn’t need a normal platelet count after all, just enough to get the job done.

Susannah’s bizarre white blood cell count has begun to escalate once again, still without any concrete etiology. We have lost some of the progress made in the preoperative period. But, then again, we discontinued the medication (hydroxyurea) that was reducing it so effectively almost a week ago, due to concerns about her kidneys. We restarted it today, but at a lower dose. A huge bonus of dialysis is that it will help her body accept this medication without the worry of its effect on the kidneys.

The surgeon is quite happy with his handiwork, and we must say, her incision is quite pretty, considering. He did, however, have to do some things that irritated the pancreas when he was removing the gallbladder, thus elevating her pancreatic enzymes. This is relevant because he wants us to hold off on feeding her through her belly until the values normalize. As her pancreatic enzymes have gradually come down, we are now quite close to restarting feeds through the NG tube, perhaps even starting them today. Susannah is currently being fed via IV, but we are hoping to capitalize on her sedated state to give her some nutrition by NG tube. As you can probably guess, the minute she is awake, the tube will cease to stay put for more than a nanosecond! The little Houdini actually tries to remove it now, any chance she gets, whenever her sedation becomes even remotely light.

Madeline is doing very well. She is in her normal routine and actually has the attention of both of her grandmothers this week. She remains annoyed at the fact that, not only could she not take her sister’s spleen to school for show and tell, but more so, that no one even bothered to take a photo of it! In unrelated news, she got word this week that she is being promoted to “Pointe” in the fall! This is very exciting for the little ballerina and all of us. We are very proud of her hard work and dedication. Her dance studio has really become a second home for her. Speaking of Madeline’s ballet, Susannah has been watching/listening her big sister almost constantly during the last week (of course, with a bit of Barney mixed in for good measure). Madeline’s recital DVDs have always proved very relaxing for Susannah, and now seems to be no different. She certainly loves her big sister!

Klane and I are tired, but find new energy every time we look at our sweet girl(s). We learned last week that his partners actually cleared his schedule at least through May, so that he can dedicate his time to his family. No call, no clinic, no OR (not that he is thrilled about the latter - “man, I had some really cool cases this month”) - pretty amazing. It is nice to have their support along with the support of the rest of the Orthopaedics department.

In a similar vein, in anticipation of Susannah’s surgery last week, some special folks at the hospital threw her a very fun Cinco de Mayo party. Not only did they provide decorations, music and Mexican food, they did so in a conference room, out of the ICU complete with a big screen TV playing non-stop Barney! Grammy, our friend Denise and even one of our favorite nurses from downstairs were invited and as we paraded our way out of the ICU, oxygen and IV pole in tow, everyone donned sombreros, sang the Mexican Hat Dance with Miss Susannah shaking maracas to the beat. Afterward, we took a detour back to her room, making for an extra long wagon ride, which was probably the highlight of the evening for the guest of honor. Afterward, an exhausted Susannah and I snuggled down in her bed for the night. The evening was such a treat and an incredible gesture of kindness.

In the last two weeks we have blessed by an amazing stream of out of town visitors. Not only did we have a host of grandparents with us as we anxiously waited for Susannah to return from surgery, but Aunt Kerri also made a sleepless detour through Seattle on her way home from New York. My best friend Wendy flew in from Boston for most of last week, successfully spoiling us all and bringing along her steadfast good karma for the big events. In what I can only describe as the nicest surprise I’ve ever received, our wonderful friend and Dallas neighbor, Lori, just appeared at the ICU door two Friday mornings ago! She spent two amazing days with us, lifting our spirits beyond belief. Just seeing her and hearing her voice, made Susannah smile and shimmy in delight; she even stomped her foot for the first time in weeks. It was great to see Susannah in such sheer joy, especially knowing what was in store for the coming week.

We continue to look forward to everyone’s notes of support and encouragement. Susannah’s local fan base continues to stream through the ICU. The nurses are constantly commenting on the sheer number of visitors that she receives. It truly warms our hearts to know that this little girl has engaged so many people in her life. Her spirit has touched many, and the devotion and love shown back to her is remarkable.

Peace (and pee) to all,

The Whites



Sunday, May 11, 2008 10:17 PM CDT

Hi All,

Susannah came through surgery on Friday with flying colors! Her little body is now sans gallbladder and one very large spleen. There have been many smiles and looks of relief floating around these parts for the last forty-eight hours. The entire surgery took about an hour and a half. She lost very little blood and had no problems with anesthesia. We were very fortunate to have the “A” team taking care of her for the big event. We are not out of the woods yet, however.

As of now, Susannah is quite sedated, but very comfortable on the ventilator. At first glance after surgery, Susannah’s respiratory needs were immediately improved. So far her platelet requirements have decreased some, but not the homerun we were hoping for. Regarding her red cell counts, these do seem to be holding on nicely, but this issue is confounded by the fact that she has been extra getting transfusions for various reasons, not directly due to her hemolytic anemia. The next weeks should be telling.

While we wait for the dust to settle in these areas, a new issue has moved to the forefront. The main concern at the moment is Susannah’s renal function. She has had major fluid shifts and her kidneys have become rather unhappy and remiss about doing their job. Fortunately for Susannah, she is supported by the vent and thus we can be more aggressive about fluid resuscitation, without having to worry as much about her respiratory status. A bit more time should give us a better handle of where we are on this issue. The hope over the next couple of days is that all of the fluids we’ve given Susannah will shift back into her vasculature and head back to the kidneys and out the door. Then we will be able to start the task of getting Susannah off of the ventilator. We expect that this will take at least a week. In the meantime, we will support her needs as they dictate and continue to keep her comfortable. In a refreshingly light moment yesterday when our priest was visiting, Amy suggested praying for “pee”, which was initially mistaken for “peace” by the priest and myself. So, we then decided both were in order!

Thanks to everyone for the wonderful notes of support. We have passed it all along to Susannah who is listening quite intently. Oh the wonder of some good narcotics and a captive audience!

With love,

Klane, Amy, Madeline and, of course, Susannah



Friday, May 9, 2008 1:40 PM CDT

Just a quick note to let you all know that Susannah is currently en route back to the ICU. She did very well in the OR, losing only about 25cc of blood. For the first time this week, the surgeon cracked a smile, certainly a site for sore eyes. More details to follow – we expect to be called back to her momentarily ...



Thursday, May 8, 2008 11:59 PM CDT

Dear Family and Friends,

As most of you may know, Susannah has been in the hospital for the past 10 months. Unfortunately, she continues to have considerable troubles with her lungs and blood function. Without getting into too many details, we wanted to pass on some news about where Susannah is at this time.

She is currently scheduled to undergo surgery tomorrow morning, at 9am, to remove her spleen and gallbladder. It is our hope that this surgery will stem the current tide, which has largely been a slow, but steady flow in the wrong direction. In anticipation of surgery, Susannah was placed onto a ventilator Tuesday. She was working harder to breathe and was experiencing pain, and so we made this decision with the hope of avoiding an emergency situation prior to surgery, and also to “tune her up” a little. In the meanwhile we have been able to keep her more comfortable, letting her rest as well.

So far she is holding up with the strength and stubbornness that has so exemplified her life thus far. Though she has lived within the hospital campus for many months, we want everyone to know that her days have been uniquely normal and filled with much happiness and typical little girl playtime, and even school time. She has made many new friends and is undoubtedly the hospital darling. Susannah is known to all for her nightly strolls and bike rides throughout the building with her dollies, all the while dressed in full princess regalia! We do not know exactly what to expect after the surgery, but we have much hope that it will give her the break that she so needs.

We apologize to all of you that we have not been in contact with for some time, and hope you understand. We will be sure to update.

All our love,

Klane, Amy, Madeline and Susannah

Sunday, April 15, 2007 11:33 PM CDT

Our sweet Susannah is EIGHT years old!

HAPPY BIRTHDAY TO YOU,
HAPPY BIRTHDAY TO YOU,
HAPPY BIRTHDAY MISS SUSANNAH ...
HAPPY BIRTHDAY TO YOU!!!

Yes, yes, I know ... you probably think you are having deja vu, asking yourself, "isn't this the very same entry I've been looking at for the last year?" Nope, it really is new! I honestly cannot believe I have been remiss for this long in catching everyone up on the happenings with our little lady. Many apologies. Our year has been filled to the brim and seems to be passing by with lightening speed. Honestly, so much has happened that I am not quite sure where to begin. I do know though, at this hour, stuffed with birthday cake and ice cream, that there is no possible way I can do it justice. I promise, however, to return with full details and new photos (really, this time I mean it!) very soon.

In the meantime, the much abridged version:

We arrived safely in Seattle in mid-August, where we were welcomed by our amazing neighbors (you will hear much more about them in the future) and loads of aunties, uncles and cousins galore! September brought about the start of school (Susannah still in the home program) and, drum roll please, Klane’s first REAL job! Madeline, being Madeline, settled right in: making friends, dancing ballet, playing soccer ... it’s like she’s lived here all her life. Of course, she’s still mastering the art of the umbrella, as are we all — it really does rain like crazy here! Klane loves his job. He too dove right in and is already sporting a busy surgical schedule. We are fortunate to live VERY close to the hospital, so he actually walks to work. Yes, even in the rain. Susannah is happy as ever and has a wonderful group of teachers and therapists working with her. She is eager to learn and has been showing them her stuff! One of her latest passions is ballet; she loves to wear it, watch it and actually has some pretty slick moves in her repertoire. She now has her very own swing set, fit for a queen, which she absolutely adores ... yes, in the rain too! As winter came about and the area beneath the swings began to get muddy, we thought it might be time to resort to indoor activities. Nope, not Susannah ... a pair of new duck galoshes and a yellow rain slicker, and she was back in action!

As you may have surmised from my reference to Susannah’s home bound schooling, she continues to be incredibly immunocompromised. In fact, after a relatively smooth year in Dallas, the Seattle scene has not been kind to Susannah from a medical standpoint. Her hemolytic anemia has been on a wild tear, and to add spice to the mix, our old friends neutropenia and thrombocytopenia (low platelets) have also been rearing their heads. Susannah has endured such episodes before, but experiencing them all at once is new. We have been treating things as they arise, which seems to be almost weekly, adding and subtracting so many meds that our world is reminiscent of her immediate post-transplant days. Some big guns have been added to try to quell the issues, yet these have brought about a host of new side effects to deal with. As always though, she is a trouper and keeps right on smiling. Susannah continues to show us that she will do things in her own time. Her unique rendition of chronic GVHD certainly has the folks here scratching their heads. Being in a new place, with new people involved, it seems everyone has a new idea. Thus, there has been much temptation to stir things up (and yes, some actual attempts), but thanks to the ever present, albeit long distance, guidance of Dr. Davies, we are managing to keep things in check. To know Susannah’s issues is to accept them, treat them delicately and coddle them with infinite amounts of patience. An acquired skill indeed. There is (obviously) not a quick fix, nor a simple answer. I cannot tell you how many times I have heard, since arriving in Seattle, “Susannah doesn’t do things by the book, does she?”

Thank goodness no, or we wouldn’t be here today!

To be continued ...

Saturday, April 15, 2006 1:32 AM CST

HAPPY BIRTHDAY TO YOU,
HAPPY BIRTHDAY TO YOU,
HAPPY BIRTHDAY DEAR SUSANNAH ...
HAPPY BIRTHDAY TO YOU!!!

Our sweet Susannah celebrates her 7th birthday today. We're looking forward to a lovely day together, filled with her favorite things ... swings, slides, dollies, doggies and, of course, CHOCOLATE!

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Tuesday, March 14, 2005 11:10 PM CST

BREAKING NEWS!!!

We are moving to SEATTLE!!! Yes, it is finally, final ... as of August our family will be relocating to the beautiful Pacific Northwest. Klane has accepted an attending position at Seattle Children's Hospital and the University of Washington. We are so excited to move on to this next phase of our lives. It's been a long time coming!

Below is Madeline's "Top Ten List" for moving to Seattle:

10. Learning to ski in the winter
9. Playing on boats with friends in the summer!
8. Actually getting to use your umbrella
7. View from the top of the Space Needle (without Mommy!)
6. Salmon coming out of your ears!
5. VERY cool Doll Museum
4. Going to the USC vs. UW game every other year
3. Riding the school bus!
2. A Starbucks on EVERY corner
1. 10 cousins within 10 miles!!!

And so the real count down begins ... just under 5 months to go!

Much love,

The Whites

PS. Sorry about the pictures ... STILL having technical difficulties. Check back soon.

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Monday, January 23, 2006 11:23 PM CST

Howdy Y’all!

Yes, yes, yes, we arrived safely in Dallas ... back in July! Yes, we DO have internet access here in Texas! Yes, I DO still intend to send out Christmas ... err New Year’s (possibly Valentine’s Day at this rate) cards. And, NO, I have not forgotten you and do check in to read your always supportive, uplifting and usually comical messages. I apologize profusely for being remiss with staying in touch. Believe it or not, I have continued, throughout these long months, to chronicle Susannah and her sister’s goings-on, so one of these days you WILL get the blow by blow account, but for now, based on the messages I have been receiving, I figure it is high time to at least get the abridged version posted!

Getting settled into a new city, new house, new job and new school have kept us all very busy. We are adapting nicely and the time is really flying by. It’s hard to believe we are already nearly half of the way through our year here. Susannah has adjusted nicely to her new team of therapists and teachers ... all still here in our home. She continues to struggle with her speech, but her communication is blossoming and she is actually beginning to read short words, spell her name and even use the computer. She has a wonderful loving teacher and many other incredible therapists and members of her educational team. Health-wise, she is faring pretty well. We are fortunate in that her medical care is being overseen by a great transplanter, who trained at none other than the University of Minnesota. Being in a temporary situation, we couldn’t ask for a physician more versed in Hurler Syndrome and BMT, able to step right into Susannah’s care without missing a beat. Of course, we remain in close contact with the folks at U of M, as well as Dr. Davies. Until viral season hit, we had been making huge gains in Susannah’s steroid wean ... reaching a record low dose. Then, December arrived, and our old friend neutropenia decided to wreak havoc on Susannah’s body. Likely due to the viruses floating around, Susannah began having trouble maintaining her white count and at times her hemoglobin dipped, causing us to hike up her prednisone dose. After a couple of months of frequent blood draws, stepped up isolation and a few doses of GCSF, it appears we may just be turning the corner. Today’s lab values were much improved over recent weeks, although there was a noticeable decline in her platelet count. Hopefully, this shift is just a blip and nothing more will come of it.

Madeline is really enjoying her new school and has meshed right in with her new surroundings. She misses her San Diego friends, but has made many new pals, including our next door neighbors, Allie and Grace. Since day one the girls have been putting on plays, having sleepovers and all in all having tons of fun together. We LOVE our neighbors and we are already all lamenting at how sad it is going to be to part come July! She is busier than ever, immersed in accelerated classes at school, ballet of course, a musical theater class, piano lessons (thanks to one of Klane’s pals for loaning us a keyboard for the year) and even voice lessons! She is about to begin her second season of Texas soccer ... with her daddy as one of the coaches! Her “Explorations” class at school recently dissected hogs’ hearts (yes, this really IS third grade) and dear old Dad was invited as the guest instructor. Madeline, as you can imagine, was beaming!

While the following, by no means does justice to the excitement of the last several months, I will at least attempt a very brief recap. We spent the month of July on a multi-state car trip, totaling over 4000 miles upon our arrival in Dallas. Some of the highlights were a stops at Papa White’s childhood home in Green River, WY and also a sunset trip to Mount Rushmore; a walk in the Omaha Fourth of July Parade with our good friend and now US Senate Candidate David (and Beth!) Kramer; four glorious days in the (not so windy) city of Chicago with our friends the Ciacciarellis and Birminghams, while Klane studied and sat for his orthopaedic surgery board exam (which he passed!); a week long jaunt to Minneapolis for visits with Joanie, Kelsey and of course, Susannah’s semi-annual check-up (for which all reports were favorable); a midnight ferry ride across Lake Michigan to Grand Rapids and a special visit from a fellow Minnesota "veteran" and friend, Amy Jastrzembski, whom both girls were promptly spoiled by! Driving, driving and more driving (the girls were absolute troupers ... never even once complaining ... and Susannah STILL loves to drive!) until we finally reached our destination three plus weeks later, and were greeted by our moving truck and the lovely Dallas heat.

August passed quickly as we all got settled with doctors, therapists, etc. and prepared for the start of school. One of the month's highlights was a party, just for Madeline, hosted by a colleague of Klane’s in order to introduce her to other third graders before the start of school! Talk about a warm welcome! Klane started work August 1st and school began August 22nd. Poppy, was kind enough to drive our second car out and Grammy followed later to ensure we all got settled.

September brought about much heartache as we said goodbye to our dear friend Julia Jean Moran. September 24th was a sad, sad day, one that we’d hoped, with all our being, would simply never arrive. Julia and Susannah were soul mates, Susannah just five days her senior. Although often separated by a multitude of states, the bond shared between the girls and their parents would be impossible to even attempt explaining. While the pain is still raw and appropriate words continue to escape me, there is peace in the knowledge that sweet Julia is now is even more beautiful and more perfect than her body would allow her to be on earth. She deserves nothing but the best. Jules nestled her way into the hearts of more people in her life, than most people could in a hundred years.

October brought about Halloween and a visit from our Alaskan (via London and San Diego) friends, the Smiths. We carved pumpkins, shared lots of laughs, feasted on the tradtional "ghoooul-lash" and "bones", and even trick-or-treated together. The girls, of course, were USC Song Girls and despite venturing into enemy territory managed to collect enough chocolate (even by Susannah standards) to last months! We also assisted two other local families in hosting the Dallas-Fort Worth MPS Run for Their Lives, to raise awareness and funds for research into Susannah’s family of diseases. A tremendous and long over-due thank you to so many of you for your generous support of such a worthwhile cause.

November took us to Arizona (yes, another LONG drive ... told you she still loves the car!) for the Annual MPS Family Conference. A good time was had by all as we met up with many old and new friends. The long weekend also graced us with a visit from Susannah’s beloved June, who was kind enough to drive over to Scottsdale and meet up with us! The reunion was long overdue and much enjoyed. We spent a quiet Thanksgiving with another of the fellows and his wife. They have not only become great friends, but also our “in a pinch” babysitters ... who could ask for more -- an orthopaedist and an anesthesiologist to look after our children!

That brings us to December. Klane had an exciting month as he and the other fellows competed in the Dallas White Rock Marathon (don’t be too impressed, 1- it was a relay and 2- they were forced ... it is a mandatory tradition), which was certainly an adventure. When they did not out run the much more aged attending team, the fellows quickly claimed sabotage and forever dubbed it, the “Black Rock Marathon”! Klane also joined Erik in Los Angeles for their annual pilgrimage to the USC-UCLA game, which was of course, phenomenal. Somehow he even managed to get himself down on the field following the game, which was a feat we never even managed during college. Scary! Viral season and low blood counts set in just in time to keep us from attending the annual TSRH Christmas Party, but we did manage to run into Santa in the elevator one night while Klane was on-call. Madeline performed a spontaneous solo of Winter Wonderland for all of the orthopaedic family and staff at the Herring’s annual “Cocoa and Caroling party”, which was most impressive! We had a great Christmas with Grammy and Poppy in for a visit. Klane was on-call both Christmas Eve and Christmas Day, but oddly enough, this was quite a treat. We ALL (the grandparents included) ventured over to the hospital and stayed in the bank of apartments there for the holiday. We had loads of space, a lovely view of the lights and the Dallas skyline and more Christmas trees than you could ever begin to imagine! Santa (aka “Ho Ho” in Susannah speak) managed to find the only chimney in the place and spoil the girls rotten. Susannah was most enamored, however, by a gift of ballet slippers from Big Sister and refused to even take them off for two days! A great time was had by all.

Klane absolutely LOVES his job and is learning a ton. Everyone at TSRH (Texas Scottish Rite Hospital for Children) aka the "Crayon Hospital" has not only been wonderful and welcoming to Klane, but to our entire family. This will certainly be a year we will never forget. The fellows were recently honored at a luncheon and Klane was asked to speak on their behalf about what this fellowship ... this year in Dallas ... is all about. Unbeknownst to him (and I am sure he is going to be “oh so happy” with me when he discovers what I've done), I have pasted his speech below for you to read because I really think it speaks to our year, what we are doing here and why we chose to embark on this adventure.



from 12/8/05
"Good afternoon, and on behalf of the Dorothy and Bryant Edwards Fellows, welcome! It is a distinct honor and pleasure for me to stand before you today and talk to you about OUR place here at Texas Scottish Rite Hospital for Children. We are here due to the gracious endowment made by Mrs. Dorothy Edwards many years ago. I would like to start by thanking her for making this fellowship a reality.

"The four of us have come to Dallas from great distances, for the opportunity to learn the art of pediatric orthopedic surgery.

"We have picked up and moved our lives and our families for the opportunity to spend a single year in a city that, until now, we have had little familiarity. Our loved ones came here not knowing anyone, leaving schools, jobs, friends and family behind. We have endured the Dallas mid-summer (hmm ... October) 100 degree heat, the tank columns of SUV’s, the country music on every other radio station, and the throngs of Texas Longhorn fans!

"Why? To come to Texas Scottish Rite, to be a part of its mission, and to continue the legacy of clinical and academic excellence that IS the Edwards Fellowship in Pediatric Orthopaedics and Scoliosis.

"So you might ask, “what is it about this fellowship, that makes the experience here, different than any other fellowship?”.

"First of all is the camaraderie provided by your “fellow” fellows. Personally speaking, I could not have asked for a group that is more collegial, thoughtful, and myself excluded, downright impressive. We are sharing experiences that, I feel, no other fellowship can provide. We are learning together, growing together, perhaps stumbling together, with an intenseness that can only create bonds that will last through our careers and through our lifetimes.

"Secondly, well actually this should be first, but I do have hang out with these three in the fellow’s office when we’re done here! Anyway, secondly, there is the orthopedic faculty. Starting with Dr. Tony Herring -- with a pervasiveness through the ranks of seniority, down to the most junior partners, there is nowhere in the country where the orthopedic faculty participates and contributes to a fellow’s education in such an remarkable way.

"Their dedication to our future is evidenced by their impressive attendance to each and every conference. I am truly astounded by how week in and week out, they show up to teach us. They eat the same roast beef sandwiches, chips and cookies EVERY Monday night, and the same cinnamon rolls EVERY Tuesday morning, and the same muffins EVERY Friday Morning. Clearly, they’re not attending for the food!

"But seriously, each one of them imparts their experience and expertise on most every case that is presented. And, always in a way that is insightful, thoughtful and educational, but never derogatory or demeaning.

"On top of this, they are always willing to take time from their personal lives. We are invited to their homes for holidays and get-togethers. They are always willing to discuss whatever may be on our minds ... jobs, research ideas, family, football ... it doesn’t matter. Their commitment to our personal growth is a unique and extraordinary asset to this fellowship, unmatched anywhere else in the world. And for this, we are extremely grateful.

"I would also like to extend our appreciation to the TSRH staff, including the advanced practice nurses, the OR and floor nurses, the orthopedic administrative staff, the therapists, orthotists and research staff, and the people from every department, from audiovisual to environmental services, all of whom make this hospital a wonderful place to be and to learn.

"Thank you to the volunteer staff, who grease the wheels of our everyday existence here, and have so kindly dedicated their time to coordinating this event. Finally, I would like to thank the hospital administration, led by Mr. J.C. Montgomery, for assembling and fostering such a focused, caring environment.

"As you well know, in this hospital's 80 years in existence, not once has a patient (or insurance company for that matter) ever received a bill. This is simply incredible. Even more so, the staff here goes above and beyond that mission each and every day to make the children (and employees) feel at home. Where else can you enter a hospital and be greeted by the overwhelming smell of freshly popped popcorn? TSRH is frequently compared, in a tongue-in-cheek sort of way, to Disneyland,. But, I’m not so sure that is such a bad thing, after all, Disneyland IS the “happiest place on earth”.

"Being the parent of a young child with a chronic illness, who is now a patient here, and having had spent many months-on-end in the hospital, I can greatly appreciate as a parent, THAT atmosphere and the collective mission promulgated by this hospital with the administration at its heart. This is the only hospital that I have ever been to, where the CEO actually makes rounds with you, and personally gets to know the patients. Now that is a life lesson, which I assure you, is not taught in other fellowships.

"So, this is our place, as the Dorothy and Bryant Edwards Fellows, so generously endowed as such, here at TSRH: To learn not only pediatric orthopedics and the treatment of scoliosis, but how to treat people, and how to give back to the community, which has given, so much, so graciously to us.

Thank you."



So, there you have it ... the reason that we are in Texas this year. What’s next, you ask? Well THAT is the million dollar question! We are currently in the final stages of ironing out just exactly where we will call home come August. I believe, and trust me this changes almost daily, we have narrowed our options down to three cities. Each of the opportunities are super, we just need to decide which of the three is best for our entire family. We’ll certainly keep you posted (in a timely manner) and no doubt, it will be exciting!

Just to clarify one thing, we may be in Texas and despite near daily harassment and a most disturbing finale to the Rose Bowl ... our home is absolutely NOT graced by a UT flag (and believe me, they are popping up by the hundreds!), there are no “horns” on our cars, “burnt orange” gives us a headache and the words “hook ‘em” are NOT part of our vocabulary! Nope, no fair weather fans here! Even Miss Susannah proudly sports her cardinal and gold and has learned to chant her very own version of “U-S-C” and raise her arms for Trojan “touchdowns”.

“Fight-On”,

The Whites


PS. Hold onto your hats, there are new photos too!!! (oops, I spoke too soon ... technical difficulties ... check back Wednesday or so!)

PSS. As we go to press this evening, I would like to welcome our newest Godson to the world ... Jackson “Jack” Walters was born this morning at 8:10am, weighing in at 9.3 pounds (20 inches). Heather and baby are doing well, as is big sister, Jessica!

Wednesday, June 29, 2005 5:14 AM CDT

We are presently up to our ears in boxes, with plans to head out of San Diego tomorrow! After taking the scenic route and visiting many friends along the way, we will ultimately arrive at our new home in Dallas on or about July 23rd.

It is very exciting for us to be starting this new chapter in our life, although we will very much miss the many wonderful people we are leaving behind here in California. Visitors are always welcome ... although we can no longer provide our guests with picture perfect weather and ocean breezes (it was a mere 107 degrees when we visited Texas in May!), we can offer true southern hospitality!

With any luck, the time in the car over the next few weeks (yes, this is one HUGE road trip!) will afford me the chance to post a proper update once and for all. There is so much to catch you up on! In short, Susannah continues to do VERY well and is happy as a clam; Madeline completed 2nd grade, with nothing less than a PERFECT report card; and of course, I can't forget, Klane FINALLY finished residency!!!

More to come ...

With love,

Amy and the gang

Thursday, April 14, 2005 4:00 AM CDT

HAPPY BIRTHDAY TO YOU,
HAPPY BIRTHDAY TO YOU,
HAPPY BIRTHDAY DEAR SUSANNAH ...
HAPPY BIRTHDAY TO YOU!!!

Our sweet Susannah turns six years old tomorrow! Wow, where has the time gone?!? She is becoming a real little lady right before our eyes. Her sparking personality and zest for life (not to mention her adorable belly laugh!) are a constant source of joy for those of us lucky enough to be in her life.

I know it has been absolutely eons since I last made an official entry to this page ... many apologies. I have however, continued to keep her journal, so I promise to get those entries added here very soon.

In short, our gal is doing quite well, pleasing her parents, sister, doctors, nurses, therapists and teachers in so many ways. Her health is much improved (the new meds seem to be just what her blood had in mind, details forthcoming) and her development is progressing along splendidly. She even got her first "kindergarten" report card which was super! This past Sunday, Susannah enjoyed a very special birthday celebration with her favorite friends and a visit from a real choo choo! We even got permission to ditch her mask for a couple of hours, a true taste of freedom for the birthday girl! It was a wonderful day and she was in her glory.

With the frenzy of "Birthday Month" in full swing (not to mention a coffee soaked keyboard that is causing much havoc, ahem Klane!), my computer access is a bit meager at the moment. So, while the full scoop will have to wait, I have posted some NEW PHOTOS to tide you over. Enjoy - they are but a small preview of the happenings around here (and don't worry, I have not forgotten about the Halloween and Christmas pics I promised), with many more to come.

Stay tuned ...

Sunday, November 14th, 2004

The Princess and the PICC


Whew!

It has certainly been an eventful week around the "White House", one we are happy to have behind us (and no, I'm not referring to the election). Despite the stress and chaos leading up to things, Miss Susannah came through both her line placement surgery and her induction dose of Rituxan with flying colors! Proving yet again, that she is one extraordinarily tough little lady.

As incredible as it may sound, in order to get Susannah safely put to sleep and her line inserted, it took no less than two hospitals and two doctors, including one from a third institution (and this wasn’t even taking into account all of the BMT docs involved)! Here’s how it all transpired. First and foremost, we imported the anesthesiologist from UCSD, as none of the Children’s Hospital anesthetists familiar with Hurler Syndrome were available. Then, since at Children’s only “PICC Nurses” are allowed to place PICC lines, and because Susannah’s history of multiple central lines required x-ray assistance, it was necessary to utilize an interventional radiologist from Sharp Memorial, the hospital adjacent to Children’s. So, while the procedure was technically handled by Children’s Hospital, it was actually performed next door at Sharp, to which Sis was transported via an underground tunnel and then back again to recover in the PACU at Children’s. Sound crazy? It certainly was, but the bizarre set-up actually ensured she was in the most capable hands. Boy, did the experience make us long for the efficiency of the University of Minnesota.

With that said, we are so thankful to Mark Greenberg for coming over from UCSD, on his vacation no less, and taking charge of not only the anesthesia, but of the situation in general. On top of him being an awesome pediatric anesthesiologist, we knew he’d put Susannah first and foremost above anything else, bureaucratic issues included. And he certainly did! Never mind that when he paged Klane the next morning to check on the princess, he said he was really glad it was over as he didn’t sleep the night before the case because he was so nervous!

Speaking of nerves, folks were a little on edge the morning of the procedure, as neither the anesthesiologist nor the interventional radiologist felt comfortable proceeding without a minimum platelet count of 30,000. A compromise was made and the BMT team convinced them that a platelet transfusion at the precise time of the line placement would serve to prevent any untoward issues from arising. In the end, all involved breathed a huge sigh of relief when Susannah’s pre-operative labs came back showing the high dose steroids to have taken hold, sending her platelets skyrocketing (okay, so it’s all relative) to 70,000, just in time for the case. This was definitely a load off everyone’s mind. Leave it to Sis to hold out until the eleventh hour!!!

The procedure itself, as unconventional as the set-up may have been, went very smoothly. Because of the pre-op IV being placed in the same arm as the PICC was set for (the left, to leave the right hand free for thumb sucking!), the radiologist was able to use contrast to lead the way up her arm vein into the subclavian. Sissy’s numerous previous lines did make it more complex in that her veins had narrowed substantially, making the task of poking the wire through more difficult than usual. The best news, of course, was that she was able to be put to sleep safely without being intubated, just as we had planned. The anesthesiologist was able to get away with using an LMA (laryngeal mask airway) and propofol. For safety reasons, he opted to wake her up over at Sharp before transporting her back to Children’s to recover. This unorthodox decision made for one very groggy, but gruff Susannah on the trip back, but there was definitely no worry about her protecting her own airway, which is all that matters! As Sis rolled around the gurney screeching, Dr. Greenberg apologized profusely saying this was the part they usually kept the parents from seeing. Klane and I just looked at each other and smiled, as a little post anesthesia rage paled in comparison to all we’d witnessed in the last few years.

Aside from getting to leave the hospital that evening, the highlight of the day was the transport trip over to Sharp Memorial. At first Sis was reluctant to get on the gurney (laden with all the requisite anesthesia monitors), as the events of recent weeks have led her to associate them with painful procedures (multiple IV placements, blood draws and freshest in her mind, the IV put in that very morning). But as soon as she saw her baby “happily” sitting on the “bed”, our gal was game! She had the time of her life traveling though the tunnel, up and down ramps, on her rolling “car”! It was so precious that the orderly gladly paused to allow for a quick photo shoot!

The following morning we headed straight back to Children’s for yet another long day. I am happy to report that Susannah’s first dose of Rituxan is under her belt and she handled it like a champ. The Rituxan was infused via the brand new PICC line, which worked beautifully. She did begin to have a reaction about halfway through, but stopping the infusion for a bit and slowing things down, seemed to alleviate the problem and the next few hours were uneventful. Compared to her previous ATG reactions, this was truly a walk in the park. Added to her usual regimen of pre-meds was IV hydrocortisone, which may have played a roll in the lackluster reaction. We will follow the same protocol each week and hope to be as fortunate.

Now Sis just needs to warm to the idea that accessing this PICC is not going to hurt, but rather make her life so much less traumatic. After all the blood letting ordeals of the past couple of months, she is not exactly trusting. Who can blame her?!? The area itself is still tender, but once that heals up and she realizes needles are a thing of the past, I’m certain it won’t take her long to remember that a line is actually an ally. At present, however, she lets anyone coming near her arm have it! In clinic, her frustration runs so deep that she is even immune to the distraction of Darrell’s famous Chicken Dance Elmo, which she, until recently, thought was the cat’s meow! As such, we’ve arranged it so that homecare sends only Susan out for dressing changes (believe it or not, it is a Children’s Hospital policy NOT to allow parents to dress, flush or draw from PICC lines -- ironic considering we’ve managed her lines for years now, but who’s complaining about extra help?!?), so hopefully having a steady person whom she already trusts will help smooth things over and get her back in her groove. Her new line is clearly at the forefront of her thoughts, as the first thing she does when I walk into her room in the morning is point to her little wrapped arm, as if to say, “WHAT is this???”

Regarding her ever fluctuating blood counts, subsequent to her pre-operative boost, Susannah’s platelets did drop back down, coinciding with the first day of lowered steroids. This was not a surprise and we expect the trend to continue until the Rituxan (which is more slow acting) kicks in. The fact that her counts responded to the steroids is encouraging that there is nothing additional going on besides the antibody situation. Her hemoglobin and reticulocyte count seem to have also appreciated the boost of steroids and are up and down respectively, to more acceptable levels. Her white count on the other hand may be up to its old tricks yet again. Friday morning’s labs showed her ANC to be all the way down to 1050, quite a marked decrease from recent results, and even down a good deal from just Wednesday. Hopefully this is just her body trying to even itself out after what were obviously inflated white blood cell counts these last couple of weeks.

As I’ve mentioned previously, the Rituxan (along with the additional prednisone) will leave Sissy’s body significantly more immunocompromised that it already is, leaving her particularly susceptible to viral and fungal infections. Fortunately (or unfortunately, depending on how one looks at it) she is already on all of the necessary prophylactic medications that would otherwise need to be added with the Rituxan now on board. On the bright side, with the existing prophylactic anti-viral, anti-biotic, anti-fungal, anti-pneumocystis coverage, as a result of her on-going requirement for cyclosporine and steroids for chronic GVHD, and val-gancyclovir for CMV retinitis, she won’t be forced to endure any noticeable additions to her already complex daily regimen of oral medications. She is, however, back in the business of being admitted to the hospital for IV antibiotics, blood cultures and the requisite 72 hour stay for any and all temperatures greater that 100.5 degrees. With the winter months upon us, we’re keeping our fingers crossed that fevers keep their distance and in turn we can keep our distance!

As always, we couldn’t have managed this week without a little help from our friends. Special thanks to the Sennes family for entertaining Madeline both Tuesday and Wednesday and ensuring she was in the right place at the right time. Also, a huge thank you to June for not only providing me with some much needed relief during the long days at the hospital, but also for her steadfast commitment to Susannah and our family. Of course, it goes without saying that Mark Greenberg was the man of the hour! Finally, I am grateful beyond words to the Ortho Trauma Team at Hillcrest for spelling Klane and going “chief-less” on Tuesday so that he could be with Susannah and I on such an emotionally draining day.

Love to all,

Amy, Klane, Madeline and Susannah

Friday, November 5th, 2004

A Change in Plans …


Hello All,

Our appointment schedule was set, vacation request granted, airline tickets purchased, hotel reservations made; we could practically feel the cold Minnesota wind on our faces, BUT our trip to the Twin Cities has been postponed. It seems that Miss Susannah’s little body has other plans for the moment.

The last couple of weeks have shown a marked decrease in Susannah’s platelets to dangerous levels. As of today, her platelet count is a mere 12,000. It has become clear that she is producing massive amounts of anti-platelet antibodies, a condition called idiopathic thrombocytopenia (ITP). We spent the day in clinic on Wednesday giving her an infusion of IVIG in hopes of getting things under control (IVIG, in this instance, can help neutralize antibodies, be they attacking red cells or platelets). The effort was for not, however, as her follow-up blood test from this morning showed an additional decrease in her platelet count as opposed to the rise we’d hoped for.

After tri-state communication between Dr. Davies in Cincinnati, Dr. Grewal in Minneapolis and Dr. Roberts here in San Diego we’ve decided to tackle this situation head on and, “we have a plan” (can you tell who’s taken charge?!?). As of tonight, Sis’s steroids have been increased to pulse doses (25mg) for three days. We will then cut her back to 6mg daily and see how she fares. Meanwhile, she will start on Rituxan infusions this Tuesday. This new drug is akin to ATG (only targeting b-cells, as opposed to t-cells) and likewise has the potential for severe reaction, so it will need to be given at the hospital each week. She will continue the IVIG therapy to replace the “good antibodies” destroyed by the Rituxan as I’ve mentioned previously. The hope is that the steroid boluses will tide her over until the Rituxan kicks in and we won’t have to resort to higher on-going steroid doses (and perhaps, even get her back to her alternating day regimen, sooner than later). For the moment, she will not be receiving platelet transfusions as any new platelets would not only be immediately destroyed by her body, but would serve to induce additional antibodies in her bloodstream. The last thing she needs with such a low count is more antibodies! Although, this management strategy makes perfect sense, it sure feels odd not to be tending to a low platelet count in the way that has become so ingrained in us these past few years. Should Susannah show any signs of abnormal bleeding from the low platelets, then obviously that would be a different story and we’d need to consider resorting to the continuous infusion of platelets as an inpatient. I should clarify that ITP differs from typical post-transplant low platelets, in that the platelets currently circulating in our gal's body (as few as they may be) are actually normal functioning, as opposed to being ravaged from the effects of chemo; thus they should be providing at least some benefit even with the unusually low numbers.

As much as we’d like to be enveloped in the comfort of the University of Minnesota during such a tumultuous time, with her platelets at such a precarious level, it is just too dangerous for Sis to travel. Apparently flying with platelet levels under 50,000 leaves one much more susceptible to bleeds of various natures, even in a pressurized aircraft. Since we do live in a major metropolitan city, with a competent institution, it is felt by all that with the directives of her transplant physicians from afar, Susannah would be best off sticking close to home. If she should reach a point where she would be better served in Minneapolis, we will get her there one way or another. Both Dr. Davies and Sat have assured us they won’t hesitate to make such a call. Otherwise her “routine” check-up, has obviously been put on the back burner until she can safely travel.

With all of the required lab draws, Susannah’s veins have taken a beating throughout the summer. Essentially every point of entry has been used, abused and bruised beyond immediate repair. Even the amazing Susan had to make three attempts before getting blood this morning! Now with the addition of the IVIG and Rituxan, both of which are administered intravenously, the issue of more permanent access is now back on the table. The least invasive option obviously is a PICC line, although past efforts at placing such a line in Susannah have proven difficult. Her current status of “pin cushion” will likely not help matters any, but they assure us the team here has much success working with adverse conditions. Aside from the physical process of actually placing the line, the issue weighing more heavily on our minds is, of course, anesthesia. Susannah has yet to be intubated since her stints on the vent and this has been a very deliberate decision for obvious reasons. These were not the circumstances that we had anticipated to change this position. Here and now, in San Diego, is certainly not the time or place we’re most comfortable with, so there will be lengthy discussions about alternate sedation options with the anesthesia departments at both Children’s Hospital and the University Hospital to see what the best options and location are. The plan is still unfolding, but Klane is on the case.

As far as the rest of Sissy’s blood counts go, her retic also peaked at a sky high 18 this week, followed by a three gram drop in her hemoglobin (although it still remains in acceptable range). She’s one very pale and yellow little girl at the moment, but the therapies planned for her platelet situation should also address any potential hemolysis that may be in the works. The silver lining in all of this is that her white count seems to have rebounded. The last couple of weeks have shown a steady increase with today’s numbers being a stellar 9,500 total white count with 7,220 neutrophils. Yippee!!!

The trickiest part of this new “adventure” will be keeping the little miss in check. I can’t tell you how many times in the last week we’ve heard the words “Try to keep her from being too rambunctious and bumping herself.” Ahhh, easier said than done! Susannah is one very active little girl (who regularly konks her head), with a mind of her own, not to mention that mind is now under the influence of major steroids! So we are going to be watching her like a hawk. Additionally, this new regimen of meds certainly kicks Susannah’s level of immunosuppresion up a notch, forcing her into further isolation. Cabin fever is already setting in and she is not a happy camper -- poor thing, just wait until she realizes how frequently she’s going to be tethered to that IV pole. I suspect tolerating the long days “hooked up” at the hospital will come back to us both like riding bike; compared to all the meds, pumps and time we endured in Minneapolis, this is nothin’!

As we venture into these uncharted waters, I have to be honest and say we are nervous. This is an issue very new to us, not to mention new even to our physicians, in a child so far post transplant and they have expressed their concern. Neither of these things are terribly reassuring, but we are clinging tightly to the words of Dr. Davies, delivered in her classically self-assured tone, “Not to worry, we’ll take care of this … no freaking out!” Ever the voice of reason. It goes without saying of course, that we welcome any, all and additional prayers, intentions and good karma you can spare for our little lady.

In closing this evening, I leave you with a special request. As the holiday season rapidly approaches, so does a tremendously painful time of year for a number of the friends we have met along this journey. November, in particular, is a month filled with difficult days for both our dear friends the Ciacciarellis and the Leightons. So as you hold our sweet Susannah close to your hearts, please remember these two families, as well as the many others that we hold close to our hearts, who have endured trials far more arduous than ours, yet continue to enrich our lives.

With love and thanks,

Amy, Klane, Madeline and Susannah

Wednesday, October 20th, 2004

A “Spook-tacular” Month!!!


Happy Fall One and All,

The reign of the antibody queen persists! While Susannah is doing exceptionally well from an outward perspective, on the inside her blood is completely out of whack. Her platelet count continues to dwindle, her reticulocyte count is higher than ever with her hemoglobin bouncing around (although remaining at decent levels) and her white count has seen better days, with her ANC teetering just below the 1000 mark. Ironically, her skin looks better than ever! Of course, all of this blood business is happening unbeknownst to the little miss and she is as happy (and feisty) as ever!

We continue to play the waiting game. But, as frustrating as it can be, the alternative of intervening is disturbing, so alas we wait, happily. For the sole purpose of “warding off the need for intervention” aka “the evil spirits”, Dr. Davies and I had a long chat last week about the course of action, should Susannah’s antibody situation not remedy itself, quickly. She remains positive that Sis can still turn things around on her own, but with her platelets continuing their movement, albeit slowly, downward, her neutrophils hovering at precarious levels and her reticulocyte count sky high (although, her hemoglobin did increase some this week, yeah!) it seemed prudent to at least discuss the options. First, as I’ve mentioned before, if her platelets (currently 50,000) drop below 40,000, give or take 5,000, we will need to increase Sissy’s prednisone dose. And, while due to the antibodies, transfusions will basically be immediately “eaten up”, we will need to transfuse her nonetheless. Obviously if her ANC doesn’t start behaving, then G-CSF will be back in the picture. Finally, if her hemoglobin begins to drop or if her retic remains at current levels (regardless of her hemoglobin), both of which would indicate significant hemolysis, we would likely start her on a weekly IV medication called Rituxin (or Rituximab) in order to suppress her system, by targeting and destroying the b-cells which produce antibodies. Unfortunately, there are both “good and bad” b-cells, so on top of the weekly Rituxin, we would also need to give her IVIG to add back the antibodies from the “good” b-cells. Unlike the chemotherapeutic meds Susannah was given during her transplants, Rituxin, is able to target only the b-cells. The idea behind this therapy is that it could once and for all put her antibody issue to rest and ultimately allow us to wean her steroids. Although Rituxin is also used in conjunction with other medications to combat secondary lymphomas, it is not classified as chemotherapy. It is safe, just cumbersome. Obviously this is a lot to swallow and not something to be entered into lightly. But, remember, I am only describing the options in order to prevent them from coming to fruition; sort of the transplant patient’s version of knocking on wood. So, please knock away!

On a far more positive note, October 9th marked Susannah’s transplant anniversary! It’s hard to believe four years have passed since she received the life saving bone marrow donated by a perfect stranger. Although we may never meet him, we will forever be grateful for his generosity and selflessness. Sometimes it seems like just yesterday that we began this journey, but then looking back, it also seems like we’ve been living it forever; just a way of life. A life that despite tragedy and heartache, has richly blessed us in ways we never before could have imagined. In spite of the challenges continually facing her, our sweet Susannah continues to amaze us and bring us joy every single hour, of every single day! As I’ve said so many times before, she is our hero.

Fall has been and will continue to be a pretty busy around here. October seems to be the month of field trips and thespians for the Whites! Klane and I surprised Madeline with tickets to the musical version of Peter Pan, starring Cathy Rigby. When we realized the show was coming to San Diego, we knew there was no way she could miss it! This is a child that has seen and can detail every single version, both stage and screen, of Peter Pan ever produced (including the now “politically incorrect” stage version starring Mary Martin from 1956!). Her copy of the show starring Cathy Rigby from a few years back is one of her most prized possessions. This obsession began when she was three years old back in Minnesota, and continues in full force to this day. The kid’s endurance is just remarkable. So, needless to say, we managed to secure (fabulous) seats and we drove our darling daughter (blindfolded) to the Civic Theatre downtown for the big surprise! Madeline was truly in her glory, simply awestruck, singing along to every word, having the time of her life!

Next week, I have the pleasure of accompanying Madeline’s class on a field trip to see “Seussical the Musical” at the local Jewish Community Center. One of her fellow second graders (and fellow Brownie), Celia, will be starring as Whoo in the show! It will be a real treat and Madeline is elated that HER mommy actually gets to go. One of the mom’s helping out in her class the other day, called to tell me that as the class was discussing the upcoming event, Madeline proudly announced that because her sister’s name is Susannah (“Sus” for short) and because she loves music, that perhaps the perfect nickname for her would be “Musical Sus-ical”! Apparently the mom and Mrs. Loebig could hardly contain themselves. Leave it to Madeline!

This past weekend, with Klane and Madeline off at their first Indian Princess campout of the year (on the beach at Camp Pendleton, complete with “Surf Diva” lessons) and Susannah safe in the care of June (with a visit from Abby on the docket), I headed north to the Los Angeles area (LaVerne, for those familiar with the geography) with my friend Julie Leighton (Christina’s mom) for a “field trip” of my own to the annual MPS Walk/Run LA. With all of the issues of the years since Susannah’s diagnosis, this was the first time I was actually able to attend one of these events and I must say it was great! There was a super turnout, with over $40,000 raised for research! If I’m not mistaken, it was the most successful year yet! We really enjoyed ourselves, at the race and at the family event following. While the parents listened to a motivational speaker and dined on a delicious lunch, the kids were entertained by a face painter and magician, not to mention the playground! Tami Slawson, the race director and fellow MPS-1 mother, did a fabulous job of putting the events together! There were loads of MPS families in attendance not only from California, but Washington, Minnesota, North Dakota, and Indiana as well! Nearly all forms of the MPS diseases were represented, some of the children we already knew and many others we were fortunate enough to meet. Although, Miss Susannah’s immune system precluded her from actually attending, she was there in spirit, as posters with photos of the children lined the finish line. Hopefully next year she too can join in the fun. I hear there are several different walks in the great state of Texas!

Speaking of the Lone Star State, the time is really flying by until our departure … nine months and counting! I have begun to research (in further detail) the logistics of moving Susannah’s care out of California. As I well anticipated, it is, ummm, rather involved to say the least, from both a medical, as well as an educational standpoint. Fortunately, or unfortunately, one of the current fellows at TSRH has children with special needs as well, so his wife has been most helpful in advising me on the inter-workings of the Dallas area school system. Of course, Susannah’s needs are not identical to their children’s, so there is still much investigating on the horizon, but at least the groundwork has been laid. I have been assured that we will be transitioning to a wonderful transplant center and an equally wonderful physician, very familiar with post-transplant Hurler children … she trained at the University of Minnesota! As you can imagine, that was music to our ears.

To add even more spice to the mix, we are beginning to think about life beyond fellowship, i.e., a REAL job and just where we want to settle our family. The coming months should bring about much information and contemplating; the bottom line is we could end up literally anywhere, there are lots of opportunities in pediatric orthopaedics, we just need to decide what is best for Klane’s career and most of all our family’s future. It will be our choice to make. So, in the meantime, while searching for housing in Dallas and mentally packing our home, we’ll also be considering the idea of storing some of our belongings, so as not to have to move them all twice, and pondering whether to rent or to sell our house here on Mount Tami Drive. Decisions, decisions, decisions. They are both scary and exciting at the same time, but this is the crossroad we’ve been striving to reach nearly all of our adult lives. We are more than ready and good thing, as it’ll be here before we know it!

With Halloween rapidly approaching, the girls are busily preparing. Thanks to a class field trip to Bates Nut Farm last week, the perfect pumpkins have already been picked and are just waiting to be carved into Jack-o-Lanterns. The house is decorated and sufficiently scary (although apparently we’re awaiting some “bones” that Daddy promised to bring home from work!). Madeline is ensuring all the finishing touches of their costumes are in order and Sis is dutifully trying hers on in anticipation of, what else? Chocolate! In honor of our trip to Disneyland earlier this year, the girls will be dressing up as none other than Cinderella and Snow White … princesses, of course! According to Madeline, the hair color should tell you who’s who. Susannah loves twirling for us in her big skirt and telling us how pretty she is. Modest, our girls are not! The big white petticoat meant to be worn UNDER her dress has been a staple outfit for little Snow White, all on it’s own, these last few weeks. It is quite a sight to see her attempt PT in the thing! Madeline, I mean Cinderella, is very excited about having her hair “done up” like a real princess and is anxiously awaiting the arrival of the prince (also known as the UPS man) with her glass slippers for the big day!

Halloween this year will be a weekend long event for our family. It all begins Friday with a parade at Holmes Elementary School, followed by a second grade class party. Fear not, for Susannah, I am sure we will come up with some version of a parade of our own here at “Home” Elementary School -- her therapists and teachers (and sister) would allow nothing less! Then on Saturday, after a busy morning of soccer, Pele, err Madeline, will transform into Cinderella once again for the annual Holmes Halloween Carnival. Then the princesses will each spend a fun-filled evening with their favorite ladies, Snow White at home with June and Cinderella off with Auntie Brenda, while Mommy and Daddy attend Heather and Erik’s (Aunt Mousie and Uncle Erik) annual Halloween Haunt. This is an event that our dear friends have been hosting since 1993, the year we headed east for medical school, and we have yet to be able to experience it, for one reason or another. We’ve been “warned” that we must attend this event before we dare leave the state again, so this is finally the year! Keep your fingers crossed that it actually materializes and nothing unexpected crops up. Finally, we’ll culminate the weekend with our traditional “spooky dinner” followed by trick-or-treating with some neighborhood pals … and lest I forget, loads of chocolate!

With November on the horizon, we look forward to another trip back to Minneapolis for Susannah’s four year post-transplant studies. We will return to our second home the week of November 14th. The appointment schedule has already been prepared and it is quite a site, but that is certainly no surprise. No doubt Miss Madeline’s (social) schedule will rival her sister’s and in fact, I hear an evening at the Children’s Theatre may even be in the works! We can’t wait to see our Minnesota family and maybe even some snow!

Sending our love, both near and far,

The Whites

Friday, September 17th, 2004

The Antibody Queen


Greetings!

School has officially started! The new academic years brings about much change for Miss Susannah. New teacher, new therapists, new curriculum … she is a kindergartner! While the changes are exciting, they are changes nonetheless and we are very fortunate to have all involved in her education (outgoing, incoming and, of course, returning) around to provide a smooth transition. It promises to be an exciting and productive year!

As for Madeline, she also has a new teacher who is awesome! Mrs. Loebig is sure to provide an incredible 2nd grade experience. Madeline absolutely loves her. She is lucky enough to be in a 2/3 combination class, which, I confess, I had some initial reservations about, but once the year started they quickly evaporated. There are only 16 kids in the class and lots of perks: Teaching up to the next level, double PE time and even double field trips! While the emphasis is obviously academic, Mrs. Loebig makes a point of introducing the children to the arts and teaching them to draw. She is incredibly fun, while at the same time appropriately firm. According to Madeline, her teacher’s two best qualities, are 1) being a fellow “lefty” and 2) her status as a USC alumna. Hard to argue there!

The start of the school year brings about the usual busy fall schedule. Soccer season is underway (Go Blue Thunder!), as is a ballet/jazz combo class, Brownies, Indian Princess, another ballet class, Sunday School and, of course, the continuation of swim team. Anyway you slice it, Madeline is one busy gal, but she loves every minute of it! With Susannah’s equally busy home schedule, I have many people to thank for helping make Big Sister’s attendance at these activities a reality. As it is so often said, “It takes a village to raise a child” and oh, am I forever grateful for our village!

We did have a bit of a “tragedy” around here last week, when, Ruby, Madeline’s fish moved on to greener pastures, or more appropriately, bluer seas. During a routine bowl change, while waiting the 30 minutes for the water to acclimate, Ruby jumped right out of her holding tank, landing across the kitchen floor! By the time she was found (thankfully Madeline was already in bed), it was too late. Although extremely upset, Madeline took the news relatively well. If you recall, she earned Ruby nearly a year ago when she gave up her thumb, so the fish had a soft spot in our hearts. Obviously Ruby lived a looooong and very active life for a fish. Of course we held a nice service for her before sending (ahem, flushing) her out to “sea”.

Onto happier news, Madeline finally lost her first top-side tooth; she was convinced she was the last second grader on the planet to do so! She looks awfully cute and is working diligently at loosening up the other one so that she can officially sing: “All I want for Christmas is my two front teeth …”. This marked the first time she lost a tooth at school and she was treated like quite the celebrity. As she tells the story, “I was just minding my own business, eating a shrimp popper, and all of a sudden I felt something hard in my chew and poof, there was my tooth! Out! Right there in the lunch arbor! All my friends saw and there was hardly even any blood. Then Mrs. Loebig gave me a lost tooth award certificate and I even got to go over and show Mrs. Wright! She gave me a tooth treasure chest and it was pink, can you believe she remembered that was my favorite color?! Oh, and Mommy, I want to try to call the Tooth Fairy as soon as we get home because I am tired of getting her answering machine, so maybe if I call early enough, she will still be home and not out on her route.” Of course the Tooth Fairy did visit that night, fairy dust, gold coin and all, but even though we called early in the day, we didn’t manage to catch her in time and once again got her answering machine! Hmm, must’ve just missed her!!!

Back to Susannah, voice therapy is going well. Carol has made significant headway in getting Susannah to produce some long forgotten sounds. Her techniques are simple, yet unique and most of all successful. She has been able to help discourage some of the more “voice threatening” guttural sounds in lieu of more of the higher pitched variety needed for good speech. Since Carol’s arrival on the scene, Sis is more regularly engaging in spontaneous vocal play, as the pros refer to it (Mom here calls it cooing and babbling). Regardless of the name, it is definitely another step in the right direction! We also intensified her speech therapy for the late summer/ early school year to seven visits per week! Although not permanent, but rather more of a fluke due to a pregnant therapist and a variety of make-up sessions, but whatever the reason, we welcomed the extra intervention with open arms. It certainly helped bridge the gap as we bid farewell to our beloved Kellee, Susannah’s school district speech therapist. We can’t thank her enough for the fabulous work she did with our daughter and wish her well as she begins her new life as a mother with her own daughter.

Susannah spent the later part of August bouncing around with her white count. However, she was able to maintain on her own without any intervention and for the last two weeks her counts have been phenomenal (WBC over 7,000)! Great blood counts, decreased need for labs (down to once a week), her skin looking absolutely awesome … the thought of lowering her steroids was just beginning to cross our minds. But, yes there is usually a but, just as her white count was heading upward, her retic was doing the same, suggesting that although her hemoglobin level remains steady, her body continues to hemolyze. To add even more spice to our lives, her platelet count is also trending downward, steadily declining (now 60,000 from a previously VERY stable 300,000 ), with her body showing the ramifications of significant bruising. The docs have decided she is now likely producing antibodies against her platelets. Of course, this scenario would not be complete if I didn’t also have to report that Susannah’s neutrophil count is back on the decline, with her ANC teetering on barely acceptable levels.

Without a doubt, our girl has become the antibody queen! Let’s see, she now produces antibodies against her platelets, to compliment her on-going red cell antibodies and how could one forget those lovely anti-neutrophil antibodies?!? The good news is we’ve now pretty much covered the entire bloodstream, so hopefully there is no where to go but up! Seriously, on a positive note, thus far all of these antibodies have been able to be managed with minimal (relatively speaking of course) intervention. We well know, that the situation has the potential to be far worse. The plan for now is to see what the next lab draw brings and should her platelets dip below 40,000, we will have to add more steroids to try to boost things up. I imagine we will also consider transfusing her, but that has yet to be discussed. Beyond the steroids there are other interventions on the table, but they are quite significant and not yet necessary, so for now I’m attempting to simply file them away in the back of my mind. Call it a healthy dose of denial. The plan for the moment is to just monitor things closely and see what the proverbial tomorrow brings.

On behalf of her royal highness, we thank you all for your continued support and devotion. It means the world to us and we’d be lost without you!

Thinking positive in San Diego,

Amy, Klane, Madeline and “The Queen”

Tuesday, August 10th, 2004

Wrapping up a splendid summer …


Howdy Everyone!

Wow, we’re already down to the final month of summer … where does the time go??? In just a few short weeks Miss Susannah will be in kindergarten (okay, so kindergarten will come to her, but still a big milestone nonetheless!) and her big sister will start second grade! I cannot believe our girls are growing up so fast. At the risk of sounding like a broken record, where does the time go???

Our summer has been great, filled to the brim with lots of family, fun and friends (including the four legged variety). The girls have been surrounded by cousins, cousins and more cousins over the past couple of months with great fun had by all. We did have to make some accommodations so Susannah could participate in the cousin extravaganzas, but everyone was more than happy to oblige. In addition to all the camps and activities we’d preplanned, Madeline also managed to sneak in a week of clay camp in Balboa Park and a week up at Grammy and Poppy’s, complete with tennis lessons and swim stroke coaching at the club. She performed as a “Lost Boy” in the musical Peter Pan with the Christian Youth Theatre (the Peter Pan aficionado that she is, she already knew every single song by heart before the week ever began!). The girls have been spending a good amount of time in the (blow-up) pool and even rigged up the slide, so that they could maximize their splashing! We were also able to attend Girl Scout Night at the Padre game with the Tarmans, and despite contracting severe nose bleeds (very last seats, very last row, very top tier), had a terrific time. Susannah, of course, skipped out on the night at the new ball park, but thanks to June, she’s been treated to several special visits from Abby, aka “the pup-pup”, as well as Champagne, the therapy dog, throughout the summer. Yes, she still loves those doggies!

As far as Susannah’s blood counts and the anti-neutrophil antibodies go, things are essentially status quo. The trial of weekly G-CSF worked reasonably well. Each week it would sufficiently jack things up, and then we’d watch the numbers slowly fade, just in time to give another dose. During this course, her ANC never dropped too low and in fact, nearing the we noticed her bumps from the G getting smaller and smaller with her actually going up on her own some near the end of the last week. All involved took this as a positive sign of her body trying to tell us the medication was no longer needed and nature was ready to regain control. After coming off though, she failed to maintain stellar counts and often dipped below acceptable levels, resulting in the need to intervene with doses of G-CSF here and there. But, just as the yo yo-ing began to get out of hand and talk of returning to serial injections was on the table, she rebounded and managed to hoist herself, just over the hump, narrowly escaping another series of shots. Then, in typical Susannah fashion, as talk again died down, we found her neutrophils to be back in the 700s. Go figure.

Out of the blue last week, all on her own (no G-CSF), we saw her ANC to jump over 3000! No explanation, but we were obviously elated, thinking she’d finally kicked this. Upon emailing Dr. Davies with the good news, I was treated to a chuckle when her response contained only two words: “Susannah rocks!!!” I guess we were all feeling a bit giddy! The excitement was somewhat short lived as her next draw did show a significant drop, but I am told these things wax and wane, so we’re just holding our breath and holding out hope that things are indeed on the mend. The good news is that the little pin cushion is down to twice weekly blood draws, which may sound like a lot, but we’ll take it over daily labs, that’s for certain! Although, I must say, we have grown very attached to Susannah’s “personal blood draw nurse” Susan, who has been affectionately nicknamed “Sue” (with the emphasis on the “uuuuue”). It is nice to have someone special who turns such an unpleasant task into a happy experience. The minute Susan walks through the door, Sis runs to her and greets her with a big hug and then launches into her very own “commentary” where she indicates the sign for needle poke, mouths the word band-aid and walks around repeating “Suuuuuue, Suuuuuue”. She is an amazing trooper, sitting very still and never even flinching as she gets stuck. Perhaps it has something to do with the fancy Strawberry Shortcake band-aids that Susan brings especially for her!

Our gal spent the last few months at war with the potty. Basically she wanted absolutely nothing to do with it! We’re not quite sure what prompted this “anger” at the potty, but Susannah was certainly adamant about it. Recent weeks, however, have brought about a subtle change of heart and she has had a good deal of success. Perhaps it is her sweet tooth getting the best of her and she is motivated by the thought of a chocolate reward. Or maybe it is her interest in her new toilet book that actually makes a real live flushing sound. Or still yet, her recent discovery that her babies too can sit on the potty! One can only guess her rationale, but regardless we are embracing every small step in the right direction and will just continue to follow her lead.

Sis is REALLY into her doll house these days. We were lucky enough to locate an accompanying playground and some additional people (and dogs!) for it, which have added a whole new element. The house has become such a big part of Susannah’s day that Grandma Dona went out and found a play mat with streets and a train track on it -- add the bus she received last Christmas and Big Sister’s train to the mix and she practically has her own little city! She has also now completely mastered “Lucky Ducks” and is coming along nicely with Candy Land. I think the rest of us are ready for a change and welcome the chance to officially move on to Candy Land (and perhaps even others that Santa might bring), as frankly, we have ducks coming out of our ears!

Susannah continues to show a keen sense of fashion, going through at least six outfits each day. She removes the ensembles from her dresser and even buttons and unbuttons her tops all by herself! Of course, she much prefers dresses from the closet, but since she can’t get to them on her own (Mommy has it booby-trapped!), she’ll settle for the items within her reach. Some of the get-ups she comes up with are hysterical, as she regularly digs in other family members’ bureaus for bathing suits, underwear, shirts and shorts ten times her size, to top off her ensembles. She obviously inherited Aunt Tami’s ability to accessorize!

Thanks for checking in on our little lady. As you can see, she does her best to keep things exciting around here, but best of all she keeps us smiling! Enjoy the final weeks of summer … stay cool!

Hugs and Kisses,

Amy, Klane, Madeline and Susannah

Monday, July 5th, 2004

Anti-WHAT? WHY? WHEN?


Happy Fourth of July (just a tad bit late)!

It seems Susannah’s back in the old two steps forward, one step backward mode. It’s now been over two months since we’ve been able to budge on her steroids and it doesn’t appear we’ll be making a change any time soon; her retic count remains elevated; we’re again doing twice weekly blood draws; G-CSF is back in the picture, regularly and yes, believe it or not, this child is almost four years post transplant! And now, drum roll please, we have a brand new diagnosis to add to her list.

Following the last update and several weeks of chasing her neutrophils with G-CSF, we watched, we waited, we bit our nails and went stir crazy as Susannah’s counts continued to drop. At one point she was down in the 300s. WHY is this happening you ask??? This far out of transplant one would think we would be focusing on issues other than her white count. Well, it seems the consensus is that Susannah is likely making anti-neutrophil antibodies. WHAT is this you ask? From what I have learned (WHEN will Miss Susannah realize, I am not interested in learning anything more about the intricacies of the human body’s blood cells???) this gets lumped under the general heading of immune dysregulation that can occur as part of chronic GVHD, and is similar in principal to hemolytic anemia; it is not uncommon to see both together. Some normal kids will even get this in response to infection, alone or in addition to hemolytic anemia. There is a blood test to check for such antibodies but it is not entirely reliable -- helpful if it is positive but doesn’t rule out if negative. Typically the anti-neutrophil antibodies tend to go away on there own and one does not wind up giving extra steroids for this. As we are well aware, however, Susannah is anything but typical. Yet, considering she is already on increased steroids because of her hemolytic anemia, in addition to her GVH, we are hopeful that this may speed up remedying her newest issue. To help move things along, not to mention keep her safe, we’ve begun giving her weekly injections of G-CSF. We will continue on this course for a few weeks, all the while monitoring her counts. The thought is, that after this prolonged artificial boost, her system will kick back into gear on its own and be able to maintain … keep your fingers crossed.

We had a nice visit with our friend Preston, in town from Portland, last week and must take a moment to congratulate he and Lori on their LONG awaited (16 years long!) engagement. We’re looking forward to the big event this fall! Preston, err Dr. Sullivan, just so happens to be an ophthalmologist, so of course while in town he got an earful about Susannah’s eyes. While he doesn’t specialize in peds, he was quick to consult several colleagues who do (in pediatric cataract surgery specifically), and proved to be wealth of information. It was good to hear from someone with a personal, as well as professional interest. He agreed that, contrary to her acuity exam scores, Susannah is indeed visually navigating extremely well. While her other doctors observed this too, it was reassuring to hear it from one who spent a good portion of the day with her, as opposed to a couple of minutes! At any rate, he is still researching the situation for us, but at least we have decided we will likely do both eyes independently when the time comes, as the risk (of blindness) is just too real to put both eyes in jeopardy simultaneously.

Madeline and I left Klane and Susannah holding down the fort last weekend as we headed off to Boston for baby J.J.’s Christening. We had a wonderful time visiting with the Calareso clan (and of course, the O’Hara contingent in town for the occasion). I was able to spend a lot of time getting to know my Godson and even got to babysit the little guy one morning, no small feat if you know his mom! Wendy and I managed to sneak out for a ladies lunch (plus J.J.), thanks to Papa O’Hara offering to entertain the big kids. Speaking of, Madeline, Justin and Brianna had a blast together and essentially spent the entire weekend in the pool, swimming their little hearts out … poor Mad even has the green hair to prove it!

We had a great Fourth of July holiday, spent with our old pals the Tarmans, who are now living in the San Diego area as well. It was reminiscent of our days back on the East Coast when we’d stay the weekend together. Of course, it’s a little different now with four kids added to the mix, but certainly just as fun. Although the fireworks over Mission Bay didn’t quite measure up to the shows we once saw in DC, Susannah loved every minute of them. We were able to find a vantage point up on the hill, close enough for viewing but far enough away such that the noise didn’t scare her. While the grown-ups reflected the “old days”, Madeline and Andrew played fabulously and Susannah, as usual, was infatuated with Jack, who was quite the sport! She couldn’t believe her eyes when she woke up the next morning to find him at the breakfast table. It made her week!

Next weekend, Klane heads to Toronto to give a presentation on the orthopaedic issues associated with Hurler Syndrome at the annual conference of The Canadian Society for Mucopolysaccharide and Related Diseases. While it would have been a nice family event, it’s just too risky with Susannah (for many reasons) right now. Still, we’re sure to have lots of excitement at home, as June will be joining Sis here at the house for a sleepover while Madeline and I enjoy a sleepover of our own, at Sea World. The event is billed as an “extreme adventure” and we will be bunking with the Manatees … on the floor! Ahhh, the perks of selling 550 boxes of Girl Scout cookies!

Thanks for checking in. Hope you’re enjoying the summer sunshine!

Sending our love,

Amy, Klane, Madeline and Susannah

Saturday, June 19th, 2004

Whacky White Counts


Happy Summer!

As far as Susannah’s health goes, it has been quite a bizarre month. Since the week following our return from Minnesota, we’ve been busily chasing her ANC of all things. Upon routinely checking her labs (which were “perfect” only the week before in the Twin Cities), we found Susannah’s absolute neutrophil count to have dipped down into the precarious range below 1000. In fact, she was neutropenic at a level of 403. We quickly treated her with a little G-CSF, and with three days of the white cell boosting medication on board, she rebounded nicely. Well, exactly one week later, we had a repeat performance, only this time the G-CSF intervention more than worked, bumping her white count all the way to 30,400! As amazing as this was, it was short-lived and within days she was back down to an acceptable, but much lower 4,900, with an ANC of 1,617. We’ve been peeking twice a week since (remember all this sticking is sans line) only to watch a slow and steady fall -- 1,200 to 777 and finally last night she was down to 477. Dr. Davies has not been terribly concerned because although Sissy’s neutrophils are low, there are other elements of her white count with acceptable ranges (monos, metas, myleos) and suggested we hold off intervening this time and see if things settle out on their own. Apparently the use of G-CSF can sometimes cause the counts to “fall hard” upon coming off of it. All bets are off however, if she doesn’t rebound soon, as with an ANC below 500 Susannah is technically considered neutropenic, where even a minor fever warrants hospitalization with all the bells and whistles. Far too many things can happen in the face of an infection with such a low count. So, for now we’re on pins and needles waiting for her body to kick back in on its own, hoping and praying for no fevers in the meantime. Sis, for her part, is totally oblivious to the goings on within her little body, other than the fact that she is NOT a happy little girl seeing that she has been banished from the outdoors until her counts decide to rebound!

As if that were not enough, Miss Susannah’s reticulocyte count has also been drifting upward, indicating her hemolytic anemia remains active. Although her hemoglobin has been holding steady (albeit at a lower number since we drained her for her bi-annual labs back in May), her retic has increased rather dramatically in recent weeks. Since the anemia issue first cropped up last fall, Susannah has continually tested positive for red cell antibodies, even though her hemoglobin has remained in check since the initial increase in her steroids. This raise in her retic indicates that they may be becoming more prevalent than in recent months. Hopefully the antibodies are just a little revved up because of her decreased white count and will settle back down once that gets back under control. With all the activity surrounding her ANC and the elevated retic, we wound up holding tight on the steroids for May and did not drop her dose as anticipated. Obviously we haven’t made a move in June either … no need to rock the boat with so much going on.

Otherwise, things have been going very well. Susannah is blossoming in so many ways. It seems we always see such a burst from her following our trips to Minnesota. We sometimes wonder if it is all the outside stimulation she is exposed to on those trips, and thus long for the day that she can attend school. We’re certain that being with her peers would do wonders for her development. Even just playing with her one pal Chloe, brings her such joy and teaches her so much.

Sissy’s language is really progressing with her initiating “words” with very little prompting. She is very into “telling” us all kinds of things about her environment, ranging from the fact that the moon is visible at night to pointing out that she has a dirty diaper. We are beginning voice therapy in conjunction with her existing speech therapy, on Monday. Hopefully, this will help with her articulation and give us some answers as to her lack of verbalization, when she clearly understands everything we say! As far as I’m concerned, the biggest news in regard to her speech has to do with one VERY important word! Susannah now says MaMa!!! Of course, it is not perfect and comes out as more of “mmmm MMMM”, but hey, I’ll take it! She has much difficulty with her M sounds, often substituting a B sound, so this is definitely progress, not to mention warms this mommy’s heart! She hasn’t uttered my name since just before she was intubated, following her second transplant in December of 2000.

She now knows her numbers to 20, by sight, as well as having a word approximation for each one. The same goes for ALMOST all of her alphabet letters. Susannah has even been working on the fundamentals of writing them, although this is not one of her more favored activities. She has mastered her colors, as well. So much so, that we are just beginning to introduce her to simple board games. We started with one of Madeline’s old favorites “Lucky Ducks”, which was an immediate hit, thanks to the battery operated duck pond! Big Sister is now determined to teach her how to play Candy Land!

The favorite Susannah-ism of late has to do with signing and let me tell you it is just adorable! As you know Sis has become quite the proficient signer, but the little smarty pants has really taken signing to a new level. She has taught her babies to sign! Yup, you just read right, with the “help” of their little mommy, Susannah’s babies know how to use sign language. Of course the babies’ communication often has to do with the wants and desires of their mother; for example, if a snack is called for, well then our gal maneuvers the dolls’ little hands/arms to sign “eat” then “snack” then “please”. It is absolutely hysterical to watch!

As you probably guessed, Susannah still LOVES her dolls. Her favorite thing to do these days is undress them. No matter how many times we put on their clothes, the little lady promptly removes them. The other day Madeline and I spent the good part of an hour dressing every single baby in our house (no small feat) and before we knew it, we were surrounded by naked babies, yet again. Apparently this is somewhat of a developmental milestone, so we’ll take it, but geez, sometimes it feels like a nudist colony in our playroom! Speaking of dressing, not only is Susannah proficient at removing her dolls clothes, but also her own! This we are very happy about and, while she may lack the fine motor skills to redress her dollies, she is making wonderful progress in dressing herself! She can put her shirt on virtually unassisted, while she flies completely solo on her socks and pants (minus a little assistance on getting them over her backside). The trouble is, now that she has mastered these items, she has decided that she has no use for them and only wants to wear dresses! Some mornings she will flat out refuse to get her clothes on, until I retrieve a dress (of her choosing, no less) from the closet! Hmmm, wonder where she could possibly have developed that little fetish?!?

Still on the subject of clothing, although Susannah has mastered sock donning, shoes have proven a bit more difficult. Perhaps that is because her mommy has not been kind in the selection of footwear for the little princess. So, I finally broke down and got the poor thing her first pair of velcro fastening sneakers. Her newest OT goal is to learn to put on her shoes by herself (off is not a problem!). A work in progress …

A couple of years ago Santa brought Susannah a child’s chair for the dining table. Not a highchair like a baby would use, but rather more of a regular chair with a raised seat. She has been eating in it for sometime now, but only recently has learned to climb in and out all by herself. Well, her latest kick is to tell us she is hungry, not by signing or speaking necessarily, but by getting herself a bowl (have I mentioned that she refuses to eat her favorite chips unless they are in a bowl?) and climbing up to the chair by herself. We’ll be wondering where she’s gone to and then walk into the kitchen to find her sitting at the table with a very smug look on her face!

Our girl is now a proficient jumper, hopping all over the place, well clearing the floor. She is jumping on her trampoline without holding on, jumping down off of steps, jumping over things, and is all around a little jumping bean! Now she is working on hopping on one foot, but has a ways to go before she is strong enough to fly solo. The other day in PT she was able to step over a 10” stack of books for a “Susannah World Record”, quite a feat considering the fact that her legs are not much longer than that! She just loves stairs and is able to both climb and descend her adapted staircase reciprocally, without holding on, but try as she might on regular sized stairs, her little legs are just not long enough. So for now she’s happy to grasp a nearby finger or opt for the handrail as long as she can keep on climbing! As Poppy would say, “Any port in a storm!”.

With June now upon us, Madeline is out of school and officially a 2nd Grader! What a wonderful year she has had and while she is definitely ready to move on to bigger and better things, we will ALL certainly miss Mrs. Wright. The two were soulmates and had such an amazing relationship! On tap for the summer: Her annual dance recital, ballet camp, acting camp, vacation bible school, swim team and a week at the beach with her cousins! The school year has also officially ended for Susannah and she has now begun her summer school program (simply a continuation of her normal school schedule) which will take us through the middle of July. Although school will break for the month of August, her private therapies will continue, as well as, increase some to fill the gap. Never a dull moment!

With love,

Amy, Klane, Madeline and, of course, Susannah

Sunday, May 23rd, 2004

Minnesota in May


Dear Friends,

It has certainly been a week of reunions! In fact, before we ever left San Diego, we were treated to a wonderful visit from the Ciacciarellis, who decided to take a Mother’s Day get-a-way to our fine city! It was great to see them again and it felt so incredibly natural to be spending time together, as if our two families hang out all the time. Of course, the absence of Max was an indescribable void and although he was not physically with us, his presence was definitely felt. As you can imagine, Madeline and Grace picked up right where they left off last year, carrying on like the best of friends. Those are two amazing little ladies! Our visit was far too short.

Once we arrived in Minneapolis, we enjoyed another long distance reunion. Our East Coast friends, the Morans, were in the Cities for the WORLD meeting and we were able to spend three lovely days catching up with them. Jules was beautiful as always and it was great to see our baby girls together (Susannah was infatuated with Julia’s belly!); it was nice to be able to spend such quality time with Patty and, how could I forget, Jay who kept us in stitches the entire time! With the meeting being held in Minneapolis, we were also reunited with many old transplant pals from near and far, including the Moores and Wigglesworths, and connected with some new Hurler families, as well.

Naturally, Madeline’s social calendar was chock-full. She essentially spent the week with Joan, Gary, Primo and their newest addition, Fratello (it seems Primo was in need of a puppy pal), but did squeeze in a sleepover at Kelsey’s (which included a visit from Miss Sue!) and a play date with Jessica Moore (who is doing awesome and going into first grade!). This was no small feat, considering the amazing agenda she and Joan had planned for the week. The two took a trip out to Pepin, Wisconsin, to see the Little House in the Big Woods (the real-live home of Laura Ingalls Wilder); spent an evening at the Children’s Theatre taking in “Mrs. Piggle Wiggle”, not to mention a slew of other fun activities.

After a very busy week, we all headed home bright and early Thursday morning for the Holmes Elementary Annual Talent Show. Madeline had decided many months ago that she wanted to be part of the show and little did we know it would coincide with our trip. Still, we managed to make it just in time and, in fact, went straight from the airport to the school! Dressed as a mermaid, surrounded by seashells and with bubbles blowing past her out into the audience, our little star belted out “Part of Your World” from the Little Mermaid like it was old hat. Adorable. Not only was she was one of the youngest participants, but also one of the few brave enough to perform a solo act.

Regrettably, our trip wasn’t completely social, but the medical portion was actually pretty low key in many ways. Since this was only a semi-annual visit, the appointment schedule was relatively light, and we were actually finished by four o’clock on many days, and twice were even able to sneak back to the hotel for Susannah’s afternoon nap! QUITE a change from our usually hectic visits. Even so, it wasn’t all fun and games for our girl; Susannah was still seen by BMT, ENT, Ophthalmology, Pulmonology, Orthopedics and the hand therapist. She also underwent skeletal x-rays, a dexascan, a kidney ultrasound with doppler, a laryngoscope in the ENT’s office and an eye exam (complete with speculum, resulting in a full blown, pin-down exam of her retinas and cataracts) in the Ophthalmology clinic.

As we’ve come to learn in our situation, no new news if often the best news. That said, things with Susannah remain status quo in many ways, with only a few blips on the radar screen. Overall, all specialists were very pleased with what they saw. Most notable was her increased level of interaction and cognitive ability. While we didn’t learn any new Earth shattering info, there were a few things to note regarding the various subspecialties. Starting with the “Uncles” …

Dr. Milla aka “Uncle Carlos” was pleased, but did add back her Pulmicort nebulizer now that we are again heading downward on the steroids. Even though she is not yet on alternate day dosing, he felt it would be a good idea to restart the nebs to keep things moving in the right direction. Of course, he really wants to see her grow and from that standpoint, encouraged starting on the growth hormone. While there is some wiggle room, apparently there is a narrow time period (up until about 7 years of age) in which she has to develop new lung tissue; after that window passes, it will just be a matter of repairing the damaged areas, as opposed to growing them anew. The endocrinologist was out of town, so we have yet to finalize our decision in the area of growth hormone, but are planning a conference call with her in the near future, as there are clearly multiple reasons for us to seriously consider its use.

Susannah also saw “Uncle Frank”, who thought her ears looked great as always, and other than a little wax removal, she was good to go. After discussing the unusual quality of her voice and her lack of use of it, despite a full vocabulary of signs, Dr. Rimmel decided to scope her. That is, he performed a laryngoscope in the office, which showed swelling and scar tissue resulting from her stints on the ventilator and abnormal use of her vocal folds with supraglottic phonation and epiglottis. In English, that means she has basically developed a set of false vocal cords which she uses to concoct a wide array of sounds that you and I, using our actual cords, could never begin to reproduce. As impressive as this may seem, it is actually inhibiting her from using her own vocal cords to properly produce sounds. This COULD explain, in part, the lack of verbal words despite her increasing ability to sign. After discussing his findings with Dr. Shapiro, the two decided Sis may well benefit from voice therapy (in addition to the traditional speech therapy she is already receiving) in order to improve the quality of her voice and help her produce viable sounds.

Speaking of Neuropsych, Dr. Shapiro was very happy with Susannah’s progress. Yes, believe it or not, the woman actually uttered those words! She felt Sis showed signs of intellectual ability, far beyond her ability to speak. She was most impressed with her sense of humor, as apparently humor is a more advanced cognitive function. As I mentioned above, she’s a strong advocate of voice therapy in this case, as she thinks it is quite unusual that a Hurler child posses such a significantly better ability to sign than speak. While many of these kids begin to communicate by using signs, eventually they progress into speech well before they are communicating in full sentences with sign. Dr. Charnas was also happy with Susannah from a neurological standpoint, with no areas of concern. He even admitted that there was no need for an MRI. Now, Dr. Davies has held fast on this for years, but without fail every time we visit Dr. Charnas, he asks for one. Nice to know the man’s finally on board.

We also met Dr. Schewender, the new orthopaedist in charge of the Hurler kiddos. It was an interesting visit, as he thoroughly went over each and every orthopaedic abnormality found in Hurler Syndrome and its respective surgical treatment. Klane sat listening quietly and after about 20 minutes, I gave the poor guy a break and told him what Susannah’s father’s profession was. Funny thing, the parent friendly layman’s explanations I was getting instantly digressed to shop talk and medical terminology, but were informative nonetheless. All kidding aside, he was really nice and very knowledgeable, obviously well mentored by Dr. Ogilvie. Susannah’s x-rays were quite stable, with her kyphosis holding steady at around 67 degrees. Her hips actually look scantly better than on previous films, showing a bit more development of her acetabulum. As for her knees, they appear to be worsening, the left in particular at 17 degrees, to a stage where they will likely need intervention. The catch is, her bones are so small that she would not be a candidate for the preferred treatment, knee stapling, and would require osteotomies instead. Unfortunately this procedure is much more invasive and often necessitates being re-done down the road. At any rate, she’s not there just yet, so we’ll continue to watch and wait. Dr. Schewender did ask us to contact him should we notice them worsening.

Of course, Susannah also had her eyes evaluated. We skipped our visit with Dr. Olsen this trip and had Dr. Christensen do the retina exam, as well as, the general exam and cataract evaluation. Boy was that fun! Seriously though, she did indeed get a thorough exam and in general things pretty much remain status quo. Her retinas continue to be stable thanks to the on-going val-gancyclovir therapy and her corneal clouding is holding steady at “plus 1” since her initial post-transplant improvement. Obviously, the big interest this time was in her cataract progression. They appear to be continuing to progress and Dr. Christensen feels they are now bad enough such that it will be difficult to decipher when more progression occurs. Basically, in any other patient, he would have already operated, but in Susannah’s case, with all the confounding factors and since her vision still appears functional (despite decreasing scores on her acuity exams), we have a little wiggle room. That said, on the docket for our next trip is an anesthesia consult with the our old pal Dr. Sweeny, who knows Susannah well not only from an anesthesia standpoint, but also from her days in the ICU (meaning, he’s very well acquainted with her lungs), to get his take on just what we can expect if and when she heads to surgery. While no one can know for certain, his insight will go a long way in helping us make a decision about proceeding with surgical anesthesia. In addition to the intubation issues, there are also a few other concerns about operating on Susannah’s eyes specifically. For instance, whether to risk doing both eyes at once (not usually done to keep at least one eye safe from complications) in lieu putting her through two separate procedures in a short time, as is customary. Additionally, some of the post-op care requires compliance on Susannah’s part or we risk injury to the eye(s). Finally, we need to decide when and where this procedure will take place. There are pros and cons to doing it locally versus Minnesota; not to mention, while we’d like to wait as long as possible, by delaying too much longer we may run into the further confounding issue of having to make a decision between doing the procedure(s) in Dallas or Minnesota.

Last, but not least, we obviously met with the folks from BMT. We had our farewell appointment with Dr. Ramsay early in the week (she is retiring this month!) which was very bittersweet, as she has been such a steady presence since Dr. Davies moved, not to mention throughout out entire MN experience. In her absence, good ole Dr. Orchard will be joining our team as the new GVH guru, with the added bonus of his involvement in the metabolic program. Of course, Satkiran Grewal will continue as Susannah’s primary Minnesota physician, and yes, we have forbid him from retiring or relocating! On our last day we met with “Sat” for an exit appointment to recap the week’s events. The overall consensus of everyone: Our little lady is doing very well. In addition to the subtle changes in her care previously mentioned, the plan is to continue to wean her steroids as tolerated, with a few holds at critical points (basically, we’re going to try doing it monthly, yet holding steady for a bit longer at the transitions right before and after moving to alternate day dosing). Finally, Susannah remains 100 percent engrafted with a whopping enzyme activity level of, get this, 98.5!!! On that positive note, I think I’ll sign off …

Love always,

Amy, Klane, Madeline and Susannah

Tuesday, April 30th, 2004

The Birthday Princesses


Hello, Hello!

Whew! The whirlwind of celebrations is behind us and still the excitement has yet to wear off! Birthday Month, otherwise known as April to the rest of the world, was loads of fun this year; in fact, it was better than ever!

Before the birthday business could even begin, we began the month’s celebrations with an exciting Easter. The Easter Bunny treated the girls well this year and Susannah quickly discovered that the Bunny was a very good source for chocolate! Once again, the kind people of St. David’s reserved the Cry Room for our family so Susannah could join us for church on Easter Sunday. They were even nice enough to arrange for Susannah to have her own private Easter Egg Hunt and gather a few eggs herself, away from the other children. She really got the hang of things and was pretty much an expert by day’s end, especially at opening the plastic eggs and retrieving the goodies from inside! Later in the afternoon, we enjoyed a festive dinner in our backyard with Grandma Dona, and Uncle Joel’s and Auntie Brenda’s families. Madeline orchestrated yet another egg hunt with the cousins, and Grandma Dona made a very cool jelly bean cake!

Onto the birthdays! First up was Miss Susannah. Five years old and pleased as punch about it! On April 15th, yes that’s right, she was born on Tax Day, the four of us celebrated quietly at home. Sissy’s two claims to fame this year were, blowing out her birthday candles, all by herself, and her latest phrase “eyyyyy ffffiiii” (translation: “I’m five”). Three days later, Susannah had her first “real” birthday party! Okay, so she only was able to have two friends attend, but it was a party nonetheless. As you know, Susannah LOVES to slide, so we had a fabulous thing called a “mini bounce and slide” delivered to our yard for the day, completely disinfected it and let her and her buddies, Chloe and Maya, jump their little hearts out! It was just precious to watch. She had an AMAZING time and her happiness was apparent to all. The top three birthday list for Susannah was: 3) going down the inflatable slide, again and again and again … 2) the feel of the grass under her bare feet for the first time (shhh, it was a special occasion!) and 1) licking the frosting of her birthday cake, BEFORE it was cut!

Five years old is sort of a monumental age with kindergarten on the horizon and real big kid activities like dance classes and sports teams now in the picture. Knowing come September, Susannah won’t be joining her peers in such things is a little hard to swallow at times. However, when we think back to where things stood just three short years ago, the changes in our little lady are absolutely incredible. She couldn’t maintain eye contact, let alone recognize numbers and letters. While this milestone birthday certainly had potential to prove difficult on Mom and Dad, it took but one look at her sweet face to be reminded that she’s Susannah, a super star in her own right!

Madeline turned seven this year and to celebrate her Earth Day birthday her classroom had a “planting party”. Since they’d been studying the holiday all week, and one of her schoolmates was also born on April 22nd, we turned the day into a big event. The kids got to plant petunias in their own miniature flower pots and then feasted on “dirt” cupcakes with gummy worms. The following Sunday, the princess celebrated in royal fashion, as the kingdom gathered at the park for a birthday bash. Big girl cousins - turned real live princesses - polished nails, painted faces and “crowned and “knighted” each guest. Even the palace teacher, Mrs. Wright was in attendance! All the royal subjects alike had a wonderful time and slept well that night, thanks to the giant pink jumpy castle!

Health wise, Susannah continues to hold her own. With her hemolytic anemia remaining in check, we plan to drop her steroids another notch come May. After that, it’ll be just two more weans until she’s back on every other day dosing. Having a day off from the prednisone will certainly be a much safer situation for our gal. We head to Minneapolis on May 13th for Sissy’s check-up. The appointment schedule is relatively light since it is only her semi-annual evaluation, so we were able to coordinate things such that Klane can attend the WORLD Lysosomal Disease Research Symposium (sort of the parent “company” of Hurler Syndrome) meeting during our visit. As always, we look forward to returning to our second home.

As we delight in another incredible year with our amazing girl(s), we are ever so grateful for the unending support of our family and friends. You have walked many miles with us these past five years, steadfastly offering your love, encouragement and strength along the way, and the rewards have been great. We recognize them each and every day as we witness Susannah’s incredible progress and hear her precious giggle. It is our hope that by continuing to share these anecdotes, but a small glimpse of her ever expanding world, we allow you too to see the joy that is Susannah.

All our love,

Amy, Klane and the Birthday Princesses

Tuesday, April 6th, 2004

Doctors, Teachers, Leprechauns … and a Mom Who Can Flip!


Hi There!

All in all, we had a pretty mellow month of March. As planned we were indeed able to drop down, yet again on Susannah’s prednisone dose. While she continues to be on daily dosing, we are certainly chipping away at that alternate day.

Susannah and I made the rounds with a few of the specialists here locally that she hadn’t seen in a while. For the most part no new issues were uncovered; we just like to show our faces periodically in case something acute was to arise. Dr. Eichenfeld, the dermatologist, was very pleased with her skin and the way her hair has filled in since last we saw him. Of course, he commented on her remarkable ability to grow so much hair elsewhere on her body as well! Then it was onto Endocrine, where Dr. Gottschalk discussed the pros and cons of growth hormone therapy for Susannah. His opinions, while welcomed, somehow managed to further confound the issue for us. We still have lots to chew over on the subject and something tells me we won’t be making a decision in May, after all.

We have been noticing some subtle changes in Susannah’s peripheral vision and while we’re certain she is still seeing, we do have concern about the increased rate at which she bumps into things and trips over items not in her direct line of vision. Given the way her cataracts have been progressing, we made a visit to Dr. Granet, her San Diego ophthalmologist. While he didn’t get the best look (thanks to a slightly uncooperative patient) he did feel secure that we should be able buy her at least a little more time before proceeding with surgery. In a healthy child he’d operate in a heartbeat, but with the risks as they are and the fact that her vision is still functional, he thinks it best to remain in a holding pattern. This is the news we hoped to hear, and basically just wanted some input as a precursor to our May visit to Minnesota, so as to ensure that we weren’t blindsided by news of an immediate need for surgery at that time. Thankfully, although surgery is imminent, we can stave things off for the moment.

Susannah’s educational team met to discuss her annual IEP with the school district. The reports were glowing across the board, with Susannah far exceeding many of her goals from last year. The pride and caring exuding from her teachers and school therapists (seven in all) was heartwarming. We are truly blessed with a wonderful team. Their knack for working together in the best interest of Susannah and the pride they show at her accomplishments was incredible. She will move into the next school year by adding another “day” of classroom time since technically she will be a kindergartner -- her teacher will visit five days a week, come September. Otherwise she will continue her current levels of service with DHH and vision services, and physical, occupational, speech and music therapies. Of course, her medical based therapies will all remain in place as well.

Lucky the leprechaun paid a visit to Mrs. Wright’s first grade classroom, toppling chairs, scattering books, sprinkling confetti and glitter galore and best of all leaving loads of treats in all shades of green and gold. Back at home our St. Patrick’s Day was made most festive when another little (blonde) leprechaun, ahem, collected much of Lucky’s remnants from school and decided to sprinkle a little Irish cheer ALL OVER OUR LIVING ROOM!

Without a doubt the highlight of the last few weeks, for ALL of us, was our Minnesota friend, Kelsey’s, spring pilgrimage to San Diego. To add even more excitement to what appears to have become an annual event, Kelsey brought along a very special guest … her mom, Sue. We ALL had such a fun week. Klane and I were even treated to an overnight get-a-way on our 12th anniversary thanks to our pals from up north. Both Kelsey and Sue are nurses (Kelsey of course, now working on 4-A), so Susannah was in all knowing hands and we didn’t have to think twice about her care. Madeline had the time of her life during the “girl’s weekend” and both of our ladies were doted on like no tomorrow. Dress-up, karaoke shows, EZ Bake Oven treats, gymnastics clinic and park outings galore were all on the agenda. Under the watchful tutelage of Sue, Madeline even managed to perfect her cartwheel. The highlight for of the trip was when we coaxed Sue into demonstrating her world famous flip! A mom who can flip??? How cool is that???

With April Fool’s Day behind us (boy, was Madeline full of jokes this year!) we’re gearing up for the frenzy of Birthday Month and all the excitement that goes on with April at our house. Speaking of, we’ll actually kick things off NOW … Happy 68th Birthday Papa White!!!

Love,

Amy, Klane, Madeline and Susannah

Sunday, March 14th, 2004

“The Happiest Place on Earth”


Happy Spring!

With Susannah’s health plugging along at a steady pace these days, I’ll forego the medical info this time and skip straight to the good stuff!

Even though Disneyland, and now Disney’s California Adventure, parks are just over an hour away from our home, due to the events of recent years, our family has never been able to venture there. Klane and I obviously visited as children, but of course Susannah had never been and even Madeline had yet to go. Due to the circumstances of late, Madeline has experienced many firsts without us present, but Disneyland was the one activity which we’ve been hoarding for ourselves, “depriving her” even when others have kindly offered to take her, as we really wanted to witness her first visit to the Magic Kingdom.

Well, thanks to the coordination and kindness of a great number of wonderful people, our family managed to enjoy a fantastic trip to Disneyland … together!!! Yes, Susannah too, believe it or not! With some accommodations for her medical condition, special arrangements, lots of string pulling and calling in favors, we were able to spend four magical days taking in the sights and sounds of the Disneyland Resort, as a family.

We were treated like royalty throughout our stay. From the minute we drove up to the Grand Californian Hotel, it was clear that this was going to be one very special experience. We were personally greeted at the car by our own personal trip coordinator, who then whisked us directly up to our room, completely bypassing the crowds (aka germs) at front desk. This is where the spoiling really began! The standard room we’d booked had been upgraded to a beautiful and spacious suite, with the famous Monorail running right under our window (literally through the hotel!). There were balloons everywhere and autographed photos of the girls’ favorite characters (how did they know???) sent over via the Disneyland mail. It was awesome.

Once settled in, we took the girls back down to the lobby for “story time”. Every evening, in front of a huge open hearth fireplace, children sit and listen to stories told by a vibrant young “miner” about the Gold Rush and living in the forest of the Sierra Nevada. While Madeline was entranced by the stories, Susannah and I found a quiet corner where she explored a spiral staircase, over and over and over again.

Then it was onto Goofy’s Kitchen where we enjoyed an exciting character dinner. Special arrangements were made for our family to sit in a secluded, glass enclosed corner where we could take in the whole restaurant, while at the same time avert the crowd. While Madeline was in her glory with all of the attention and affections of the characters, Susannah was a little more tentative, and would have absolutely nothing to do with the furry characters. No matter how cute Chip and Dale tried to be, or however cautiously they approached the table, as soon as they were within six feet, Susannah would flip out. Once the music and dancing began however, it was another matter! As the music played all the characters and the kids danced like crazy, and of course, Miss Madeline was front and center with none other than Goofy himself as her dance partner. Though, she still refused to get too close, Susannah thought this was the neatest thing she’d ever seen and eventually warmed up to Princess Aurora from Sleeping Beauty and decided she just HAD to have her crown! Minnie Mouse did receive some looks of interest, as long as she stayed on the other side of the table! It was a fabulous evening and quite an incredible feeling to be able to ALL dine out together again.

Upon returning to our room, we were further spoiled by finding the beds turned down with a stuffed “Brother Bear” for each girl peeking out from behind the pillows. For the grown-ups there were chocolate character coins and rustic containers of California Redwood seedlings. Still there was more! On the coffee table, was a platter of the most decadent chocolate peanut butter cookies you’ve ever seen, accompanied by four ice cold glasses of milk. We were sooo spoiled!

Even though it was the quietest time of year to be at Disneyland, and some of the slowest days of the week at the park (and even slower due to predicted rain!), there were still far more people than we’re accustomed to having around Susannah. Aside from our usual precautions, we were, however, able to make special arrangements to alleviate some of the risks posed to Susannah. When Sis was with us, her special pass allowed her to avoid people and enter the rides through the exits; on any rides with community seats, for example Pirates of the Caribbean and It’s a Small World, we were given our very own boat! Everyone was so accommodating -- when Madeline really wanted Sis to join us in the Haunted Mansion (we’d been on it three times already without her), the cast members were gracious enough to allow Sis and I to enter from a secret side door, avoiding the crowded “stretching room”, which would have otherwise precluded her from enjoying the ride. We were also selective about the timing of the rides and made certain to avoid peak hours, having her join us at the beginning and ends of each day, the quietest times in the park. It took some coordination, but thanks to June and Grammy, Susannah was able to spend the busier mid-days back at the hotel on exciting little adventures of her own.

Our first day at the park began early the next morning and was approached with both excitement and apprehension. Armed with disinfectant wipes and hand sanitizer, spare masks and a backpack full of other goodies, we watched them drop the ceremonial rope and then made a B-line to Fantasyland, where we rode what became Susannah’s all time favorite, Casey Jr’s Circus Train. She squealed with glee, signed “more” profusely and let out great big belly laughs throughout the ride and brought us all so much pleasure that we returned to that train more times than I can count over the course of our stay! We then took some time to procure the requisite Minnie Mouse Ears, red bow and all! What Disney trip would be complete without them? It was then off to Dumbo, on which BOTH girls tormented their mother by insisting on flying very high! We, of course, made the rounds to Mr. Toad’s Wild ride, the good ole fashioned merry-go-round, Pinocchio’s Adventure and, who could ever forget (not a chance with Madeline present!) Peter Pan’s Flight. As the crowds started to roll in, I took Susannah back to the hotel while Madeline and Klane discovered Tom Sawyer’s Island and all the “big kid” rides -- Haunted Mansion, Splash Mountain, Pirates of the Caribbean, and the Matterhorn. That afternoon we saw the new Snow White musical. With Susannah rejoining us at the end of the day, we hit Susannah’s other favorite Disneyland attraction: It’s a Small World! Susannah loved the music and couldn’t get over all of the little kids singing the words again and again. She was mesmerized such that all she could do was point and say “bababababa” while signing “boy” and “girl”, time and time again. We also made it to Toontown that night and saw Mickey in his own mouse house!

The next morning we made our way to the new Disney’s California Adventure. What a wonderful day! Because our hotel was literally attached to this park, we were allowed the special privilege of early admission, and a pre-opening ride on the amazing “Soaring Over California”. Once the park officially opened, Madeline and Klane dashed off to Paradise Pier, where they boarded California Screamin’, a huge upside down roller coaster. Not only did they brave it once, but ran back in line for back to back rides! Madeline is still telling her cousin how brave she was and how much fun she had (apparently he was a little intimidated by the roller coaster and refused to ride it, and he’s two years her senior!). After a few more rides, Madeline and Klane went to see Playhouse Disney Live, where they were given front row VIP seats and Madeline danced in the aisle with Rollie Pollie Ollie. Later in the morning, Susannah joined us for the Bug’s Life rides (I think we rode the Caterpillar train, seven times straight!) and Brother Bear’s adventure park, where she giggled as Madeline swung from ropes and waived down from above bridges. The girls had a great time together on the rock slide and on our way out even got to meet Brother Bear, himself. Lunch was spent at Ariel’s Grotto, again with a safe haven table arranged so Susannah could join us. We were able to meet even more Disney characters there, with Susannah still keeping her distance, although she did play peek-a-boo with Minnie. Klane and Madeline saw a couple of shows and toured the Mission Tortilla factory and Boudin Sourdough bakery while I took Susannah back to the hotel for a nap. With Susannah sacked out and in June's capable hands, I snuck back just in time to enjoy the awesome seats the hotel had arranged for the Aladdin Musical -- a great performance, complete with flying carpets! At about six o’clock it started to rain! Now the place was virtually empty and we had the run of the parks, so we headed back over to Disneyland, and rode ‘til our hearts’ content … there were absolutely no lines!

A private “character meet and greet” with Princess Aurora at City Hall was arranged for the girls on our last day. Madeline was able have the princess all to herself for close to an hour and the two chatted non-stop with Aurora even teaching Madeline the “secret princess handshake”. Needless to say, she was enamored! It took a little re-warming up, but ultimately Susannah blew her kisses and bid her farewell with a great big hug. The princess visit, combined with the events of the last few days wiped our little lady out, so Susannah and I again headed back to the Grand Californian for a break while Madeline and Klane went on a princess hunt. It took some careful scouting, but Madeline managed to secure the autographs and photographs of ALL of the other princesses, not to mention many other characters.

I think the highlight of our trip, was the very last ride, on the very last day. With the parade keeping the rest of the guests occupied, we headed back to It’s a Small World. Not only were we the only people on the boat, but believe it or not, the only people on the entire ride! It was an opportunity we couldn’t resist and would likely never have again, so we removed Susannah’s mask held her high on our laps and watched her dance throughout the entire ride … not once, not twice but three times in a row! Her pure joy was apparent to all and I think the cast members were nearly as excited by her fascination as we were! We exited the ride, with her blowing them kisses, grinning from ear to ear. A perfect ending to a perfect trip!

This vacation was truly a dream come true for our family! True, there was risk involved, but with Dr. Davies’ blessing and every precaution taken and every courtesy extended, Susannah was kept as safe as humanly possible. We are so grateful for the time and effort invested by so many to ensure she was protected at all costs, not to mention the lengths they went to indulge and pamper the whole lot of us. Ironically, my mother’s friend Bill who was the driving force (although he had many co-conspirators!) behind making this incredible experience a reality, announced his retirement after 30 years with Disneyland on the very day we were leaving. The timing of our trip could not have been better and was clearly a once in a lifetime opportunity! We made magical memories that we will cherish always.

With much love (and a little pixie dust!),

Amy, Klane, Madeline and Susannah

Saturday, February 21st, 2004

A Month of Memorable Moments


Hello All,

As planned, we began the month by dropping Susannah’s prednisone another notch. The steroid taper is going extremely well, with little to no adverse reaction. So well in fact, that we’ve been given the go-ahead to decrease it by another milligram (on alternating days) come March! This is great news, as the sooner we can get her back to an every other day dose the better. Of course, eventually, we need her off entirely, but first things first.

Otherwise, her labs and whatnot all remain stable and her retic count is even dropping down some, in spite of the lessened steroids. This is very good news with regard to the hemolytic anemia. If we can omit the use of steroids for that little issue, then we can return to putting all our efforts back into wheedling away her dose for the pesky chronic GVHD.

Since her line came out last summer, we have virtually performed circus tricks in order to draw Susannah’s blood, with at least three and often four people required to pin her down for the process (the little lady screaming all the while). Ironically, it isn’t the pain, but rather the pinning that irritates her; in fact, often by the time the needle is in, Sis is “calm”. Well, today at clinic, as she was sitting on the gurney, glancing at the TV, Darrell the master phlebotomist, set up his stuff, put on the tourniquet and began feeling for the vein, while I sat at the ready for the moment to lay her down and begin. Well, seizing the moment, Darrell uncapped the needle with one hand while holding Sis’s arm with the other … next thing we knew he was in, had his blood and Susannah was sporting a Bugs Bunny band-aid! I think the three of us are still in shock! Susannah was sooo brave (and Darrell was sooo slick)! I have to say she showed off her band-aid, like never before, I really think she knew it was something to be proud of!

We had a very special visitor this month. Jenny Birmingham flew in from Chicago for a weekend vacation. Even though she left Molly and TJ at home, Jenny was not at all short of children’s affections. Susannah and Madeline both developed an immediate fondness for their newest friend and instantly became her shadows. Susannah had no qualms about following her into the bathroom and Madeline was more than comfortable plopping herself down on the bed as our guest readied herself each morning. Madeline is still relishing the visit by finishing up Jen’s yummy flavored creamer (in her milk, not her coffee!) claiming to be “just like Miss Jenny!” We had a great time and got in loads of visiting. The only complaint: Our time together was WAY too short

Just days after Jenny headed home, Susannah decided to follow in her pal Molly’s footsteps and perform the infamous “poop and paint” stunt. I walked in to check on a supposedly sleeping Susannah, to find her naked in her crib, with some very smelly artwork surrounding her! Yuck! I’d actually been through this escapade once before when Madeline was a toddler, but boy oh boy, did adding a CSA hair-covered body to the mix ever spice things up! Nothing short of a scouring shower had the little princess back to her sweet self. I suppose I should be happy about Sissy achieving such a normal right of passage, yeah sure, as long as it only happens once! Needless to say, we are now double securing her diapers and clothing at naptimes!

On a much more pleasant note, February 11th already marks the celebration of two birthdays in our family, for both Uncles Steve and Tom. This year, also on the 11th, we welcomed (from long distance, unfortunately) the newest member to our family, as “Auntie Wendy” gave birth to my Godson, little J.J. Calareso. We can hardly wait to meet him and see his sweet face in person! In June, Madeline and I will be traveling to Boston for his Christening -- that day can’t come soon enough!

This month, Madeline and Klane attended the annual Indian Princess Daddy-Daughter Dance and had a blast. The theme this year was a Luau. As I helped get Madeline dressed in her grass skirt and lei, unbeknownst to us, a little someone in the other room was busily digging through the dress up trunk looking for a costume of her own. Miss Susannah had conned Daddy into getting her suited up in her Hawaiian finest and was giddy with excitement, twirling around in her festive skirt. She was none too pleased however, when it came time to leave and Madeline and Klane exited the house without her! Real tears flowed and she stomped around frantically saying/signing over and over “please, drive, Su Su, me!”. It was so sweet, but it broke all of our hearts. Next year, Sissy, next year!

Extra special congratulations to Miss Madeline who won her classroom Spelling Bee, landing herself a spot in the finals of the 1st Grade Spelling Bee … which I’m pleased to report, she also WON!!! This was quite the event, big stage, microphones, judges and much to Madeline’s delight a huge trophy! We are so very proud of her.

XXOO to all,

Amy, Klane, the Queen “Bee” and her very brave Lil’ Sis

Saturday, January 31st, 2004

News for the New Year


Hi Everyone!

The New Year has kept things busy around the White House. It has been an exciting month, with several things to highlight.

First things first, Susannah continues to do well with no adverse effects from the low ANC issue earlier this month, and in fact at present, her neutrophils are exceptionally elevated with no known cause. Her hemoglobin remains steady and although her retic count remains a little on the high side, after four long months, Dr. Davies thinks we should give a minor wean of her prednisone a shot. Now, don’t get too excited, we’re only dropping her steroids by one milligram every other day (beginning tomorrow) but it is a step in the right direction.

Susannah has had a busy couple of weeks of appointments. A routine clinic visit allowed Dr. Kadota to lay eyes on her and confirm what we already knew -- she looks great! Next, she had her first ever dental appointment. With all of her ongoing issues, it was basically just a look-see to be sure there was nothing major going on inside that little mouth of hers. Dr. Poisette was very gentle and Susannah handled things really well, especially once she noticed the television playing on the ceiling above her head! Her teeth checked out beautifully in terms of decay (he found none!), but of course there were the anticipated issues of the effects of radiation on her enamel and prolonged ventilation on her bite. Nothing to do for now, other than keep up the good hygiene!

She also saw Dr. Wenger for her annual (San Diego) orthopaedic appointment. He was very pleased with her progress and delighted with her level of activity. Susannah was more than happy to show off her new jumping abilities … have I mentioned she now clears the floor by four inches?!? Her x-rays looked good, stable. Hips remain unchanged and her spine and knees have shown marginal improvement, however likely only do to slightly varied positioning, in other words within the margin of error. We will continue with our watch and wait motto for now, which will probably remain the course of action until she is off steroids and her chronic GVHD is resolved. Other than giving the use of growth hormone his blessing and approving the new foot orthotics her physical therapist recommended, he sent us on our way with a few of his typical narratives and an edict to return in a year.

The highlight of Susannah’s month happened by chance, but boy was it exciting. Klane and the girls were waiting in the car one Saturday evening while I ran a quick errand at the local outdoor mall. With the usual perfect timing, Madeline had to use the bathroom, so Klane put on Susannah’s mask and carried her to the door of the store, sending Madeline in to find me. As the two were waiting outside, Susannah spotted a child-size choo choo train chugging around a track near the playground. She was entranced. Of course our initial response was to attempt to divert her attention and get the heck out of there, but, with it being dusk and the all the stores closing, the children seemed to be evaporating. The kind lady working the attraction, instinctively knew this was a special situation and invited Susannah and Madeline on board the train. She proceeded to give the girls three private rides, back to back. Susannah was beside herself with glee and the smile beneath her mask was brighter than I’ve ever seen. With Big Sister as her guide, she quickly learned the appropriate “woo woo” sound, complete with hand motions. She had the time of her life! It was an absolutely heartwarming site and truly made our night.

The other big Susannah-ism of late is her newfound interest in saying her prayers. Every night, during her bedtime ritual, she dutifully clasps her hands and awaits the words “Now I lay me down to sleep …” (on a good day, she will even attempt to repeat them, one by one), then proceeds to sign all of the special people in her life …Madeline, Daddy, Mommy, Grammy, Poppy, Grandma Dona and who ever else catches her fancy at the moment. Madeline has even been working on teaching her the sign of the cross, which is a pretty funny sight. While I am sure she has no idea of the meaning of her nightly ritual, she dutifully clasps her hands together anytime her crib is calling!

Madeline has some big news of her own this month. After deciding she was finally old enough to give up her beloved thumb, she was rewarded (Dr. Poisette’s ingenious idea) with her very first pet, a red beta fish. She is very a very responsible mother to “Ruby” and proudly shows her off to anyone who walks through the door. Currently, she is devising a scheme to take her to school for show-and-tell! On the subject of fish, Madeline also made the swim team and has been dutifully practicing 2-3 times a week, depending on her Brownie schedule. Speaking of, she is quite the little salesperson, setting the record for the most Girl Scout Cookies sold in her troop. Thin Mints, anyone??? Spring soccer starts next month, as does practice for her annual ballet recital. She is one busy little lady … I just hope I can keep up!

For my part, I was treated to luxurious long weekend in Massachusetts with my childhood friend Wendy (ready to pop with her third child) Calareso, at a phenomenal spa in the Berkshires. While I was technically there to celebrate Wendy’s impending motherhood, she was kind enough to let me sneak in a visit with three other lovely ladies. Julia and, of course, Patty Moran, along with Hurler-internet matriarch Lisa Post, drove up from Connecticut to meet us for lunch on our way to the mountains. It was a short but sweet, long overdue visit during which I was treated to many snuggles and giggles by the incredible Miss Jules.

Last but certainly not least, I am delighted to say that Klane has accepted a fellowship position. We are so proud of him, that we are ready to don our cowboy boots and move to Dallas for the year! He will be training at Texas Scottish Rite Hospital for Children, a center devoted exclusively to pediatric orthopaedic surgery. Not only is it a phenomenal opportunity for him clinically, but they have even offered to fund a research project for him, focusing on the some of the orthopaedic aspects of Hurler Syndrome.

Although the big move will not take place until next summer (2005), Madeline is already brushing up on “The Yellow Rose of Texas” with her karaoke machine and I am busily looking into insurance and therapy issues, as moving Susannah for the year will certainly be an endeavor. Klane, of course, is very excited to be given such an awesome position. We are ALL really looking forward to this new adventure. And the best part is, fellowship signals the beginning of the end … there really is a light at the end of the tunnel!

Yee Haw!

Amy, Klane, Madeline and Susannah

Tuesday, January 6th, 2004

The 12 Days of Christmas …


Happy New Year!

The four of us rang in the New Year quietly here at home, joined by Grammy and Poppy. At the New York midnight, pots and pans were banged, clanged and rattled, toasts were made with kid “champagne” (and of course, the real deal too!) and kisses were given. By the time the 12th bell tolled here in San Diego, the whole lot of us were fast asleep!

Backing up a bit …

With our families spread out across the country this year, we spent a relatively quiet Christmas close to home. Grammy and Poppy came down on the 23rd and we shared an early Christmas Eve celebration on the morning of the 24th, before they headed north to my sister’s house. Of course our Christmas Eve would not be complete without the annual Christmas Pageant at St. David’s. This year Madeline had a speaking role as a shepard, and what a star she was! She started the night with three very important lines “Whose turn is it to watch?”, “Mercy on us!” and “Let’s go find Him.” Then when one of the other shepards fell ill at the last moment, she acquired three more and recited them like a champ. As the third and final Shepard suffered from a sudden case of stage fright, Madeline just moved right in and fed her the words. It was precious and she stole the show. While this was Susannah’s third year witnessing the pageant (not including her role as Baby Jesus the day following her diagnosis four years ago), it was the first year she really enjoyed herself. Although she was still secluded in the cry room, she managed to dance throughout the service to Christmas carols and wave to “Sis Sis” through the glass (and okay, she did her fair share of banging on it as well!).

Susannah still has not grasped the meaning of the season, or even the concept of Santa, but she certainly enjoys partaking in the excitement. She quickly became and expert at unwrapping packages and even snuck in a few when we weren’t looking, never mind that they weren’t for her! In fact, much to Big Sister’s chagrin, the little lady no longer requires assistance, and is quick to express her displeasure at unwanted fingers on HER packages. She absolutely loved driving around the neighborhood looking at the Christmas lights; we racked up quite a few miles the month of December and each time, Susannah had no qualms about expressing her displeasure when we’d finally pull into our own driveway.

Santa was very good to the girls this year, although he seemed to underestimate Susannah’s interest in her big sister’s toys. You see, Mr. Claus brought a karaoke machine intended for Madeline, and while she is belting out the tunes like there’s no tomorrow (look out American Idol!), Susannah is utterly obsessed with it and insists on “singing” everyday. All of her therapists, nurses, teachers and of course, her parents, have been coerced into showing off their singing ability. Truthfully, the machine has really brought many hours of family fun, more so than we could have ever imagined. It is not often there is an activity that both girls can equally enjoy.

Susannah latest antic is climbing into Madeline’s new doll stroller. We continually find her sitting in it, looking like the cat that caught the canary. Madeline’s beside herself that Sis has no interest in heeding the warning label, which, as we are reminded daily by our six-year-old, “plainly states that it is not intended for real live children!”. The stroller, along with its accompanying twin dolls, ranks up there as one of the biggest hits of the holiday for BOTH girls.

Sissy has also spent countless hours playing with two of her own newest toys. No surprise following Christmas in most households, but in this case, we were never sure if Susannah would be able to utilize such items. She received a three wheel scooter from June and she can actually ride it all on her own. Of course, she has a ways to go before she is proficient, but she is well on her way! We are also pleased to see her putting her new Sit-n-Spin to use. Perfect for Susannah, this traditional toy has a new twist in that it is musical. Regularly throughout the day, we hear the sound of music coming from the playroom, and are delighted to find a smiling Susannah, using her arms to spin herself around in circles!

Much to our delight, she is also beginning to outgrow many of her toys and we find ourselves packing away many of the more babyish items in lieu of more challenging gadgets. That said, Susannah is really blossoming in her intellectual development. She understands absolutely everything, it is just matter of coaxing her to get the actual words out. Her comprehension is truly incredible and she is doing a great job following commands and showing initiative. With her receptive language skyrocketing, our goal, as always, is to beef up her expressive component. Her signing has reached new levels and now includes numerous prepositions and more abstract words like “forgot”, “oh my” and “hurt”. While she will readily repeat sentences (one word at a time), verbally she continues to require significant prompting. As frustrating as this can be, we know the words are there, and just await the day they come flowing out.

Not to be outdone, Madeline continues to excel in first grade with an exemplary report card from the fall semester. She received all but one E and that only because Mrs. Wright said she didn’t want her head to swell, so she gave her a G in penmanship and something to work towards! She is a champion reader, well on her way to reading “chapter books”, although her favorite subject is still recess! Needless to say, she is looking forward to returning to school from this long winter break.

Susannah’s overall health has been stable and without surprise. She seems to be holding her own this cold and flu season, although we are well aware that it is still early in the game. We will however, have a little more lee-way this year as she is sans line, so where as in the past, a fever meant an automatic hospital admission, this year there will be a little more discretion as to the course of action. This will be a welcome change. Regarding the hemolytic anemia issue, Susannah continues to remain steady on her daily dose of steroids, with her hemoglobin stable and her reticulocyte count marginally high. We did, however, have a scare recently when her ANC dropped down to an alarming 750, with an incredibly elevated eosinophil count. Unfortunately, due to a glitch in the system, we were not made aware of the low level for several days, at which point it had actually crept back up over 1000, all on its own. While there was no explanation for the low ANC (the high eos are being attributed to a mini flare of her chronic GVH -- a bit odd considering the significant level of steroids she continues to be on), we are just glad things are back where they belong and she is looking good.

Wishing you and yours a healthy and prosperous 2004!

Much love,

Amy, Klane, Madeline and Susannah

Friday, December 19th, 2003

Facts from the Frozen Tundra


Happy Holidays One and All,

We’ve returned from the winter wonderland of snowy Minnesota, very much in the holiday spirit. I must say after braving the 10 inch snowfall and sub zero temperatures (no, I am NOT exaggerating), a warm, sunny California Christmas sounds pretty darn inviting! All kidding aside, we had a great visit.

With Klane tied down at the hospital and unable to join us this trip, the girls and I embarked on the week’s adventure all on our own. And what a nice week it was! Despite my anxiety, the flights went very well, no small thanks to our friends, the Walters, for the loan of a miniature DVD player, complete with an array of Baby Songs and Barney episodes. The distraction of this incredible machine, coaxed Susannah to handle wearing the oxygen mask, with out much of the usual raucous. Madeline of course, was the quintessential big girl helper and really pitched in to make things run smoothly. If you could have seen the three of us trekking through the airport with all of our paraphernalia, you would have been skeptical, as we were quite a site, but to the amazement of all, we did just fine.

We were met at the airport in the Twin Cities, by our faithful friend Karen and her husband, in their very necessary minivan. Since it was just us girls, we thought we’d try staying at the Ronald McDonald House this trip. In all of our time in Minneapolis, we never had the privilege of staying at this amazing place. Due to our “short” stay, we didn’t have the most spacious of accommodations, yet it was abundantly clear that the facilities and amenities, not to mention kindness and support, offered at the Ronald McDonald House are top notch.

Almost as soon as we’d unpacked, we found ourselves repacking smaller bags for the weekend jaunt out to Albany, Minnesota for Kelsey’s wedding. Thanks to our friend and former “college girl”, Kim, for acting as chauffer, babysitter and companion, the adventure went off without a hitch. We could have never managed without her. With a snowy December evening as the backdrop, Kim, Madeline, Susannah and I, arrived at the church to a warm greeting and were immediately whisked off to see the bride, as she had requested the pre-ceremony presence of her “honorary flower girls”. Madeline was invited to sit up front with the groom’s parents and was the perfect princess. I snuck Susannah into the back of the church to watch Kelsey make her entrance and then she and Kim (now an elementary school teacher) went off to play in a side room. It was a beautiful ceremony, to compliment a beautiful day! Congratulations Kelsey and Adam, we’re so happy for you and we were honored to be part of such a special occasion.

Following the ceremony and getting Kim and Sis situated in the hotel, I braved the winter roads and headed out to the reception. Although there were more than 300 guests in attendance, the evening was very personal with loads of special touches and we were able to spend some quality time with Kelsey. She even presented Madeline with a special package (and one for Susannah, to go), containing a miniature replica of her own veil … after all, every princess needs a crown! The hit of the evening was the dance floor with Madeline, who had never before attended a wedding, dancing her little heart out.

On Sunday, we attended the post wedding brunch and then made our way back to the Cities, where we were greeted by our CarePartner, Sue. It was great to see her and the four of us had a nice evening together, enjoying loads of laughs. As usual she spoiled the girls silly. Just as Sue, was walking out the door, Joan walked in and Madeline could hardly contain herself. We loaded her bags into the car and they were off like they’d never even been apart, to enjoy an incredible week together. Some of the highlights of their week included an incredible day of sledding, a trip to the Children’s theatre to take in the Wizard of Oz, play dates with old friends, playing non-stop with Primo the puppy and lots and lots of quality time.

While Madeline was off at her second home, Susannah and I managed to get in some visiting of our own. We were able to catch up with old friends and even meet some new ones, as there are several Hurler families currently going through the transplant process. The only void being, of course, the absence of the Ciacciarellis. For months we’d been planning the reunion of Madeline and Grace and in fact, Margaret and Mike were the driving force behind our decision to stay at the Ronald McDonald House this trip. Not only did we miss them, but it was clear that the staff and families who’d had the privilege of sharing a roof with them did so as well. Their family definitely made a lasting impression and will forever be a part of “the House”.

Despite the frigid temps and dumping of snow, Susannah and I made the audacious walk to clinic each day. Thankfully I left our usual travel stroller behind, and decided to lug our old tried and true Minnesota winter pram, because we were essentially “four-wheeling” it for a few of the days. It was cold, (some mornings below zero as I later was told), but we bundled up and made our way, just like we did in the old days. As I saw my breath, literally freeze on my scarf, I knew for certain that Susannah would not have a fever when we arrived in clinic! Hee hee! Seriously (although the breath part was true), we were actually both quite warm in our bundled layers and Sis was entranced with the snowflakes falling on her mittens and the white covered ground. I, on the other hand, may have been slightly more entranced had I actually packed, snow friendly shoes, as opposed to backless mules! Live and learn.

Okay, onto the medical stuff …

All and all, our girl is doing very well. She remains 100 percent engrafted with an incredible enzyme activity level, which as you well know was the whole reason we first ventured to Minnesota so long ago. All of her other labs were stable, including her IGG, which was in the low normal range.

Of course, her chronic GVH and hemolytic anemia, remain at the forefront of her care, but even those two issues are relatively stable at the moment … no small thanks to good ole prednisone. As stable as things are, however, Dr. Ramsay is in no hurry to begin tapering. She also stressed the fact that should Susannah require more transfusions, the likelihood of finding a compatible match is slim and we need to be prepared to transfuse with mismatched blood. Although, not at all desirable, given the proper preparations in the appropriate setting, it is do-able. Otherwise, she was pleased overall with the reports from the various appointments and happy to see Susannah so interactive and social. Although, "Sat" was doing his stint as attending on the unit during our visit, we did get the chance to visit with him as well, and he was also able to join Dr. Ramsay for our exit conference. As always, it was great to see our trusted team and receive their reassurances.

What the specialists had to say:

Eyes: Once again, the major issue this visit played out to be Susannah’s eyes. While her retinas (CMV retinitis) remain stable and inactive, her cataracts have continued to significantly progress. Dr. Christensen was actually quite surprised that her vision remains so functional and stressed that it is just a matter of time before the cataracts “take over”. Apparently this will just happen overnight, so when it does we need to be prepared to act quickly and intervene surgically. We will watch things closely and reassess her eyes her in May, if not sooner. The school system, here at home, continues to remain involved with their vision services program to help in this area. On a more positive note, Susannah’s refractory exam came back favorable, showing no need for correction. Translation: No glasses at this time!

Ears: As has been the case in recent visits, Susannah’s ears checked out beautifully on all accounts. With nothing more than a little wax removal and social visit with “Uncle Frank” in the ENT clinic, we were on our way to Audiology. Despite her annoyance with the head phones, the audiologist was able to determine that Susannah continues to hear within the normal range on all frequencies.

Heart: Susannah’s heart remains in tip top shape and according to Dr. Braunlin and Jim Berry, they’d be hard pressed to even know she has Hurler Syndrome by looking at her heart alone. In other words, as of now, her heart and heart function are essentially “normal”.

Tummy: We saw the GI folks, who were more than satisfied with our gal’s nutritional status and even signed off on her, barring any new issues. She is eating well (with a little extra boost from steroids) and her weight is actually on the upper end for her height. Although, it her scrawny steroid arms and legs make it difficult to actually visualize.

Endocrine: Speaking of height, we also had an appointment with Dr. Petryk, who seriously visited the issue of growth hormone therapy for Susannah. Give or take a centimeter, her growth has been stagnant for the last 2 ½ years. Since it appears prednisone will continue to be on board for sometime to come, the Endocrinology team is very interested in introducing growth hormone. The use of synthetic growth hormone has shown reasonable results in children on long-term steroid therapy, with no real side effects, aside from the annoyance of daily subcutaneous injections. Still, its use is relatively uncharted territory in children with Hurler Syndrome, which makes us a tad bit hesitant. So, we’ll chew on the idea over the next couple of months and make a decision by our next visit in May.

Lungs: Our visit with Pulmonology was much of the same ole same ole. Growth continues to be the best medicine for Susannah’s lungs, so needless to say Dr. Milla is very much on board with starting growth hormone therapy. Otherwise, Sis’s pulmonary status essentially remains unchanged. He did hear a few “crackles” from her lower lobes and she presented with some rather low saturations in the office, so he sent us packing with a Pulmocort script and loaned us a nebulizer machine to use during our stay, just to be safe.

Kidneys: Another renal ultrasound was performed, but there continues to be no consensus as to what to do (if anything) about the discrepancy in Susannah’s kidney size. After a quick chat, with Susannah all the while obsessing over Dr. Mauer’s moustache (he’s taken over for Dr. Kim), it was decided that he’d take her chart and review “this interesting case” with his colleagues. On the table for our next visit, is a possible shift in her antihypertensive medications to make them more “nephro friendly”. Of course, the doctor also expressed his desire to see her off of the cyclosporine (wouldn’t we all!?!), but we’ll leave that battle up to the Bean Team and the BMT folks –- should be interesting!

Bones: We saw Dr. VanHeest who reviewed the EMG from our last visit, and after a quick look-see was pleased with the functional ability of Susannah’s hands and fingers. Of course, the GVH has left them less than aesthetically pleasing, but as far as carpal tunnel syndrome and range of motion, she remains in pretty good shape. We did see the hand therapist and she was refitted for her hand splints, this time one is pink and one is blue, with her thumb still left free for sucking! As far as general orthopaedics (hips, knees and spine), with the retirement of Dr. Ogilvie, we opted to table this appointment until next time since she’s already booked to see Dr. Wenger here in San Diego in January.

Brain: While our appointment was officially with Dr., Charnas, we of course were not permitted to pass through the neurology clinic without a little once over by the whole gang. Drs. Charnas and Lockman were their usual comical selves, doting on Susannah and bragging about her recovery to their latest team of medical students. They continue to be very pleased with Susannah’s upwards trajectory.

Development: I tried my hand at not micromanaging the appointment schedule this year and as a result, Susannah’s neuropsych evaluations wound up smack in the middle of her naptime (so much for my laissez faire approach, I’ll be back in action next time!). Despite her sleepy uncooperative state, Miss Susannah’s developmental testing showed progress in all areas, some more than others, but all and all, she is moving in the right direction. As always, Dr. Shapiro would have liked to see more, but we have learned that no amount of gain will ever be enough and have learned to take the commentary with a grain of salt. It was abundantly clear to all that Susannah is a much different child from even 6 months ago … we’ll gladly take that!

The highlight of our clinic visits for Miss Susannah was the discovery of what came to be known as “Pat’s House”. The waiting room of the BMT clinic has always had a little enclosed play house, but it has never really interested Susannah. WELL, let me tell you, this time she couldn’t get enough of it! While she became an expert into conning anyone and everyone into taking her over to the house, she was especially fond of playing inside it with our nurse coordinator (and friend) Pat, hence the nickname “Pat’s House”. Promises of visiting “Pat’s House” were key in getting our little lady through many uncomfortable procedures throughout the week.

We are so very thankful for all the positive reports on Susannah. Especially this time of year, when we remember all too well those dark days when we were not at all sure what the next day held. While we still don’t have a crystal ball, and know the future is always uncertain, we know at least that the chance for a future is real. Susannah’s health, in whatever shape it may be, is the greatest gift we will receive this Christmas. While her road has been long, and continues to be uphill, we are so very aware that there are far worse alternatives as we hold close to our hearts our many dear friends who no longer have their children in their arms.

From our house to yours, wishing you all wonderful holiday season, filled with peace, joy and the love of family.

Merry Christmas,

Amy, Klane, Madeline and Susannah

Thursday, December 4th, 2003

Farewell Sweet Boy


Dear Friends,

I had so hoped that the next entry to Susannah’s Story would have followed our trip to Minneapolis and been filled with details, not only of Susannah’s appointments, but also of our reunion with the Ciacciarellis. Instead, I find myself writing with tremendous sadness to tell you about my sudden trip to Chicago to attend Max’s funeral.

On a crisp December morning Mighty Max was laid to rest, but it was six days earlier that he found true peace. This courageous little boy fought with every morsel of his being from the moment he entered this world, until the moment he left. Max was a hero to everyone who made his acquaintance, as well as many others who never actually had the pleasure. While the pain of his passing is felt across the globe and so many are left to mourn, Max is finally free from the limitations of Hurler Syndrome, running and singing, as only his angel status could allow.

As devastating as Max’s loss is to his family, they did a beautiful job honoring his life. The incredible dignity and tremendous hospitality shown by Margaret and Mike, on what was no doubt one of the most difficult days of their lives, personified courage. The pride they both exuded as they spoke of their beloved son was testament to their unparalleled love for him. And then there’s Grace. The love and laughter emanating from this amazing three-year-old, as she passed out photos of her baby brother to those she saw crying, brought comfort to all. Her unabashed adoration, an inspiration. It was easy to see, surrounded by these three, not to mention his extended family, just where Mighty Max ascertained his strength and valor.

I was greatly privileged, along with my friend Jenny Birmingham (Molly’s mom), to present the gifts at Max’s mass. The bond shared by Hurler and transplant families is truly like none other. Whether our friendships are fostered over years or weeks, in person or from afar, our bond is strong and vast, and I imagine, incomprehensible to those who have not walked in our shoes. True the triumphs we share are often tempered by tragedy and, to be honest, at times it would be far less painful were we to distance ourselves from one another, but certainly our lives would be far less rich. I will be forever indebted to Max, Susannah and the others for such a tremendous gift.

Farewell sweet Max. Your spirit will eternally shine and light the lives of your family and the many friends, known and unknown, whom you touched in your short life. May you rest in peace dear boy and as you are watching over your family from above, know there are a great many people looking after them here on earth.

With deepest admiration,

Amy, Klane and Girls

Sunday, November 23rd, 2003

November News


Hello There,

First and foremost, with all the excitement surrounding last month, I was remiss in congratulating Susannah on achieving a very important milestone. October 9th marked the third anniversary of her (second) bone marrow transplant. She is an amazing girl, who has traveled an amazing road and we couldn’t be more proud of her.

When one enters into the world of transplant and statistics are given, it is often said that once a patient reaches the two year post transplant mark, they are “in the clear”. Well, now three years later, with transplant issues still playing a major roll, we’re continuously amazed at the resilience, determination and zest, at which Susannah plods through each and every day. Regardless of what life throws her way, her attitude never ceases to amaze us. For instance, no doubt due to the many uncomfortable procedures she’s endured, Sis absolutely abhors being pinned down or confined in any way. Thus she is very squirrelly and adamantly shakes her head “NO” at the nurses who come to the house to draw her blood each week, yet within minutes, even seconds of them completing the task, she has composed herself and forgiven all, even blowing them kisses and making the sign for love. What a gem!

From a medical standpoint, Susannah continues to hold her own. It seems that we will be remaining on the current course for sometime, to allow her hemolytic anemia time to level out. Once all factors (retic count, bilirubin and hemoglobin being the front runners) are in check and remain there for a while, then and only then will we move toward the much anticipated prednisone taper. The plan is to get Susannah back to alternate day dosing, with the ultimate goal, of course, being to wean her entirely, but first things first. For now, as long as we have the steroids in full force, we will just sit back and watch them do their magic.

All of her other labs remain stable and her IGG is actually holding its own. This is great news, not only for the obvious reason that it is helpful to her immune system, but also because it makes managing her hemolytic anemia a little less complicated. While IVIG is a common treatment for hemolysis, in some cases it can actually have an adverse affect. Since it is unclear what exactly triggered her hemolytic anemia so late in the transplant process and considering she had only recently started IVIG therapy, all involved feel if it is not required due to low IGG levels or active hemolysis, it might be wise to steer clear just in case.

Last week, the girls were treated to a private meeting with the Dragon Tales. With the live show in town for the week, and knowing how much Susannah loves to dance to the Dragon Tales tunes, one of her nurses, arranged for a private rendezvous with “”Cassie” and “Ord”. Backstage at the Sports Arena, both Susannah and Madeline were presented with a box full of Dragon Tales paraphernalia, complete with miniature stuffed versions of the characters (and although, Sis couldn’t join us, Madeline was even given special tickets to that weekend’s show!). A few minutes later, the big moment finally came, as the long awaited dragons came out to meet the girls. While Madeline was enamored with the six foot tall creatures, enveloping them in huge hugs, Susannah preferred to admire from a distance, a BIG distance. In fact, she wouldn’t get closer than 15 feet from them! We managed to get a picture by holding her on my lap with the dragons far, far away and then having them sneak up behind us. However, as soon as the camera snapped, so did Susannah, as she realized just what we had tried to pull over on her and she was off in the other direction like a bolt of lightening. Minutes later, from her safe vantage point, across the corridor, she giggled and pointed to her newest friends. I must admit, while it was sad to see her afraid, I couldn’t help but relish the fact that Sissy was exhibiting behavior of a typical toddler, as I have yet to see a child her age be fond of anything in costume!

Madeline, once again, enjoyed a very fun-filled soccer season as a member of the Golden Retrievers. This is not only the first year of a girls only team, but also the first year with a goalie, as well as assigned positions, and the various other things that “real live soccer” entails (can you tell by my vagueness, I am still not quite the ultimate soccer mom?). Madeline played an awesome defender, dutifully maintaining her position on the field at all costs … a coach’s dream. However this new league ranges in age from 6-9 years, and we just so happened to have a team of twelve six-year-olds, while the other teams were sprinkled with a large number of returning players. As you might imagine, with such a “young team” there were many a sad face following the games, although all was generally forgotten by the time the snack was passed out! Madeline, like her father, is the master score keeper, so you can imagine our relief, when in the last few minutes, of the very last game, the Golden Retrievers finally WON!

Speaking of physical activity, Miss Susannah continues to be more and more active each day with her endurance and stamina making great strides. She can now make the walk around our entire block (hills included) with her respirations remaining in safe territory. She has also mastered the art of standing up from the floor with out holding onto anything or anyone for support. In the short time since my last update, she is now able to climb DOWN off her horse all by herself! Of course it comes with a price, as I greeted her this morning in her room to find her with one leg swung over the rail of her crib! Hmm, do I see a big girl bed in her future???

Sis has also mastered the art of donning her socks all by herself! Ahh, if she could only convince her big sister to do the same -- Madeline has to be the world’s biggest procrastinator when it comes to putting on her socks! While Susannah still needs help with her shoes, she has become quite proficient at wearing everyone else’s. Her favorite pastime of late is trying on people’s shoes and parading around the foyer in them! One more stage of “normal” development, that we are so grateful to witness.

The girls and I will soon be heading to Minneapolis for Susannah’s anniversary appointments. Yes, we are technically a couple of months behind schedule, but despite the winter weather, it was a conscious decision to coordinate her three year check-up with Kelsey’s wedding. We fly out on December 5th, attend the big event on Saturday and begin our week of fun, bright and early Monday morning. Then, 19 appointments later, we’ll head back to San Diego on Saturday the 13th. As always, we are very much looking forward to visiting our Minnesota family.

In the meantime, we plan to spend the Thanksgiving holiday at Grandma Dona’s new house with all the San Diego Whites. It will surely be a nice relaxing day filled with family, food, fun, and most importantly, as far as the girls are concerned, COUSINS! Decorations for this festive day have already been handmade by none other than our darling daughters. Susannah, for her part colored and “cut” out her very own turkey during a recent OT session. While Madeline, created a beautiful wall hanging of a paper plate pilgrim girl (complete with blonde hair and a bow!) adorned with the following sentiments: “Pilgrim Madeline says: this Thanksgiving, I am thankful for Ballet, Susannah, my parents, Chinese food, lions and The Circle of Life”. Ahh, the priorities of youth (and a little Lion King overload?)!

Happy Turkey Day, one and all ... we are thankful for YOU!

Love,

The Whites

Wednesday, November 5th, 2003

Tricks and Treats and Prayers Too!


Good Morning,

We had very nice Halloween here at our house and hope you too had a happy and haunted holiday. Madeline dressed up as an angel and very apropos, this year, Susannah’s costume was a little devil! Both girls’ costumes were chalk full of sparkle and fluff very befitting of the princess in them both. Trick-or-treating was a huge hit! Susannah donned her matching mask (thanks to Grammy -- hopefully one of these years she won’t have to make THAT part of the costume) and we set out about the neighborhood with three grandparents in tow. We did bring along Susannah’s wagon, but after the first house she wanted no part of it and elected to scurry from door to door with the other kids. She quickly figured out what all the fuss was about, and definitely knew the ultimate goal was CHOCOLATE! Once home, the girls dove into their treats and we all feasted on our traditional fare of Grandma Dona’s “Ghoooul-ash” in a pumpkin and bread “bones”! Madeline declared it to be “the best Halloween ever” and is certain they collected enough loot to last "at least two and a half months!". Susannah on the other hand is still too busy with her chocolate to comment.

On a more somber note, the recent fires in our fare city have been truly devastating. Our family was fortunate to squeak by with nothing more than a thick coating of soot and ash. The entire county essentially shut down for a day and school was cancelled for the entire week due to the poor air quality. Thankfully, the hepa filters we regularly have running were able to keep Susannah and her already fragile lungs in a safe position. As lucky as our own family was, we are painfully aware of the many others who lost their homes and memories and even their lives. It was a very scary time for San Diego. It hits a little too close to home when Madeline’s Brownie Troop made signs for those who lost their homes, along with the brave firefighters who worked to save them, and we were able to personally deliver two of them.

Far from San Diego, but equally close to our hearts, our friends, the Ciacciarellis, remain in Minnesota with their sweet Max suffering from complex post transplant issues. While this remarkable family continually exhibits indomitable spirit and resolve, they could certainly use some extra prayers as Max fights to overcome the turmoil going on within his little body. He has a long road ahead of him, but his steadfast determination is tantamount to none! As you so often did for Susannah during our tenuous days on Unit 4-A, we ask that you please remember Max, as well as Margaret, Mike and Grace, with special intention. Presently, up in Room 7, Mighty Max proudly sports a button which reads, “I Believe in Miracles” … we know, first hand, that miracles do happen and in fact, witnessed one ourselves in that very room! Max is undeniably another.

Never stop believing,

Amy, Klane, Madeline and, of course, Susannah

Saturday, October 25th, 2003

New endeavors all around …


Hello All,

Steroids -- our best friend and our worst enemy. Thankfully the situation with Susannah’s blood has been holding steady, no doubt due to the increased dosing of her prednisone. While we weren’t forced to resort to enormous doses to get things under control, we did have to switch her steroid dose to daily, further suppressing her immune system and leaving her more vulnerable to infection, not to mention the many other adverse effects of long-term steroid use. The alternating day regimen that was being used to treat her chronic graft versus host disease was far more gentle to her system, as it essentially allowed her body a day off, thus helping to reduce the side effects. Unfortunately, weaning her is going to be a very slow process with no plans to do so in the near future and the steroids will continue to take their toll. Regrettably, any hope for vertical growth has again been placed on the back burner. The bonus, however, is that the prednisone is keeping her skin in check. On a much more trivial note, just as she was beginning to lose those infamous steroid cheeks, they are back again in full force. On the flip side, Sis is eating like a champ.

Just when we thought we knew all there was to know about Susannah’s labs, we have recently had a new value introduced to our vocabulary. We now follow something called a reticulcyte count, which essentially is a measure of immature red cell growth. The ultimate goal in beating the anemia is to have her “retic” count down to 5.0 and her hemoglobin steady above 10.0, combined, of course, with a normal bilirubin. This is going to take some time, to be sure, and we will not even begin to dink with the steroids until we are in a good place with all of these numbers. Sissy is, however, looking good clinically, essentially rid of the yellowish cast to her skin and very energetic. Her platelet count continues to hold steady for the most part, which is also a reassuring sign. We have been able to cut back on blood draws to twice a week, which is definitely a good thing for Susannah’s psyche, not to mention her delicate veins, which have been pretty well tapped-out.

The onset of her hemolytic anemia has also spawned some changes to Susannah’s medications. She continues her complex daily regimen of somewhere in the neighborhood of 20 plus doses, but there has been some fine tuning as of late. With the steroids being upped as mentioned above, there is no need to supplement her systemic dose with a topical form as prophylaxis for her lungs; so for now the nebulizer has been put on hold pending her prednisone being tapered back down. Also, we have added a folic acid supplement, as the process of ongoing hemolysis tends to eat up the body’s normal supply. Other additions also include calcium carbonate and a daily vitamin D supplement, as recommended by the endocrine folks due to her chronic steroid use. As long as I’m on the subject of vitamins, I should also add that with Susannah’s vitamin A level back in the normal range following the long hiatus from her multivitamin, we have once again started her on an iron free vitamin. The reason this is worth mentioning is that it actually demonstrates progress, as last year when we put the supplement on hold, Susannah was taking a liquid version, or as Madeline would say, “a baby vitamin”. Now, she joins her big sister each morning in consuming a “big girl vitamin”, a ritual even more exciting since we discovered Gummi-vites!

In spite of all that is going on medically with Susannah, she presses on, none the wiser. Her days now completely revolve around her babies. She is becoming quite maternal, pushing her many charges around in their strollers, feeding them in the highchair and insisting they do absolutely EVERYTHING she does. If she eats, her baby eats, if she goes to the park, same for the baby. We have outdoor babies, indoor babies, bathtub babies; we have big babies, small babies and every possible baby in between. Our house has become utterly inundated with dolls and to be honest we are unsure as to just where and when we acquired all of said babies, nor do we have an exact head count (we are beginning to wonder if her therapy pals are sneaking them in to her!). No matter, for in Susannah’s eyes, the more the merrier! Her babies have become a part of every therapy session, mealtime and even diaper change. They brush their teeth, dance around the kitchen and go to the park to swing on the swing. Sis very much tends to these babies and is becoming a very good little mommy. We just love to see this pretend play. If per chance, the girl lays her eyes upon a real live baby, she shivers with excitement becomes absolutely giddy!

Susannah’s infatuation with the potty has taken an unprecedented step forward. Twice now she's insisted she sit down on it, concentrated for a bit and then stood up to reveal a very exciting and smelly little present! Yep, our gal pooped on the potty, TWICE. Of course, we all danced around singing her praises (and the famous song, a la Aunt Tami) and once she got over the shock, she too applauded herself. Susannah was so intrigued by flushing her deposits down the toilet that she headed back to the bathroom later for a little fun. Apparently she’d determined that the toilet bowl was the perfect place to take her babies for a swim! Needless to say, we now have one less baby in our possession.

October also marked another milestone, as Madeline FINALLY lost her first tooth! When weeks (upon weeks) of wiggling, were getting her nowhere, and the new tooth was more than half way in, Grandma Dona intervened. She tied a string on Madeline’s tooth and after Susannah gave it a few tugs, it finally gave way and shot across the living room floor. It took three adults on our hands and knees with two very giggly little girls, but we finally found the darn tooth! Of course, it was promptly deposited in the pocket of the special “tooth pillow”, and after placing a call to the Tooth Fairy and leaving a message on her answering machine, it was time for bed. The next morning, aside from a fancy gold coin, the Fairy also left a sprinkle of fairy dust in the bed!

This month Klane began the interview process for his fellowship in pediatric orthopaedics. While he will not assume his new position until the summer of 2005, we will have to solidify the location for this next phase in our lives by mid November, this year. It is a little strange already ironing out the details for something so far in the future, but that’s the way the system works, and we are quickly realizing that with all the nuances of relocating Miss Sis, the lead-time will actually be welcomed. At this point, we have no idea where we will end up for fellowship year, but in the running are: Dallas, Toronto, Boston, Cincinnati, Los Angeles, or even remaining in San Diego. There are, as one might imagine, pros and cons to each program and location, so it will be a difficult decision process, which will no doubt include the availability and accessibility of suitable care for Susannah. We’ll surely keep you posted ...

We appreciate your continued prayers for Susannah and our family. Your unending love and encouragement mean the world to us!

Sending our best,

The Whites

Thursday, October 9th, 2003

Loads of Excitement


Hi Everyone!

Things appear to be holding relatively steady in regard to Susannah’s hemolytic anemia. The increased steroid dose seems to be just enough to keep things in line for our little lady. While her hemoglobin continues to experience peaks and dips, she has managed to avoid further transfusions, although sometimes only by the skin of her teeth. Her bilirubin remains elevated, which is evidence that there is still some hemolysis occurring, but the everyday steroid regimen is keeping things at bay. We continue to take things one day at a time, literally, letting her body direct our actions.

Despite the goings on inside her body, and the additional pokes and prods, Miss Susannah remains as happy as a clam. She is continuing to learn new things each day and really beginning to express her personality. The girl is absolutely adamant about her wants and needs these days, or at least what SHE thinks her needs are! Sis is very much exhibiting typical toddler behavior and as frustrating as that sometimes can be, in the end it always brings a smile to our faces. It’s all about perspective.

Much to our delight, she has finally started to babble. This is a phase that has been very much remiss from Susannah’s development, but recently she has begun to “chat” to herself as she flutters about the house. Of course, the moment she realizes we are observing, she becomes completely tight lipped, but the couple of minutes that we are privy to, are truly music to our ears. This is a really big milestone, as up until now, Susannah would "speak" when spoken to, but otherwise made little to no noise (we literally would not know she was awake in the mornings were it not for the beeping of her pulse ox, when she stands up and wreaks havoc in her crib!). We are hopeful that this is the beginning of many good things to come in terms of her speech. She is also making lots of progress with sounds and signs in her therapies, but is very selective on what she says and for whom. While she’ll say one thing for one therapist, teacher or nurse, she’ll flat out refuse for another. It is definitely on her terms! With family, she is even more selective, however yesterday, as I removed her from the bathroom where Klane was showering (have I mentioned her obsession with the shower?) we were treated to a very boisterous, “Bye, bye Da Da!”.

Sissy has also become extremely agile these days, quite the little daredevil! Recently, I walked down the hall, having only had her out of my site for a few seconds, to find Susannah sitting proudly atop her spring horse, feet in the stirrups and all. She’d climbed up all by herself and was beaming from ear to ear! As I got closer, she began riding it like a banshee! Now, whenever she happens to find herself alone in the room, the girl makes a B-line for that horse! I think she likes the shock value. Her riding style has become so fast, fearless and furious that we joke about needing some sandbags to keep her from getting air (probably not a bad idea!). Next on the agenda, procuring a safe dismount!

In other exciting news, we just returned from a very special trip abroad. When Klane was asked to speak (from a parent's perspective) at a meeting on the orthopaedic management of Hurler Syndrome, we decided it was a wonderful opportunity to reward Madeline for being such a wonderful and understanding big sister, by having her join us for some special time together. She has given so freely of herself and continually amazes us with the care, compassion and concern she directs toward her sister, never harboring even an ounce of resentment. As a matter fact, when we told her about the special surprise we had in store, the very first words out of her mouth were, “And, Susannah too?”

The meeting was in Manchester, England, just a few hours from London. The timing and location could not have been more perfect considering that on top of its many exciting sites, London is also host for the year to one very important six year old … Madeline’s best friend, Lara. The three of us spent five amazing days exploring the city with the Smiths and then, while Klane and I attended the meeting up north, Madeline had an extended sleepover with Lara and her family.

We thoroughly enjoyed our trip with some of the high points being (as transcribed from “Madeline’s London Journal”): “Spending the night on the airplane; seeing and hearing Big Ben; a cruise on the River Thames to the Prime Meridian, where time begins; evening singing service at Westminster Abbey; the changing of the guard at Buckingham Palace; high tea at the Savoy; an afternoon at Lara’s school to see how school works in London; visiting Kensington Palace and the Peter Pan statue in the park; seeing Peter Pan’s statue at Great Ormond Street Hospital where Lara’s daddy works; helping my daddy hail a black taxi; riding atop a classic London red bus; locating Platform 9 ¾ at Kings Cross (only to find it “sealed” because there wasn’t a Hogwartz train coming); touring the Tower of London; and my very favorite part, seeing the Lion King, the play.”

The meeting itself was phenomenal, providing a vast amount of information to professionals of both the MPS and orthopaedic worlds, as well as, parents. Among the highlights were discussions on the treatment of spine, hip and knee deformities in Hurler Syndrome; the potential of enzyme replacement therapy; and the role of various mutations on skeletal outcomes. It was a very productive forum and some consensus was built on appropriate timing and techniques for treating these children’s orthopaedic issues. Specifically, what has worked so far and what hasn’t, as a great deal has been learned by trial and error over the years. We look forward to this consensus statement being published by the MPS Society of the United Kingdom, in the near future. Hopefully, such a tool will enable others to learn from the pitfalls and triumphs of those who have already gained some orthopaedic experience in treating post-transplant Hurler children. On a more personal note, we were able to meet several teenagers who had undergone BMT (some even twice) early in life and are doing just beautifully -- definitely good for the soul!

With Susannah’s still compromised immune system and her oxygen requirements at altitude, unfortunately she was not able to join us. Rest assured however, she had an absolutely incredible time back at home, with June moving in for the week and doting on her every whim. There were art projects, baking sessions, pizza parties, park outings galore and even a visit from a little dog! Rumor has it that the little princess didn’t even miss us! While the opposite was certainly not the case, we were very confident Susannah, even with her newfound anemia issue, was being perfectly tended to stateside by our two favorite Brits. June was in contact with Dr. Davies throughout the week, ensuring that absolutely every “i” was dotted and every “t” crossed. Susannah received unparalleled care from both a medical and personal standpoint. We are so very blessed to have such an exceptional nurse (not to mention, friend and member of the family) in June. In our absence Sissy could not have been in more capable and caring hands.

All our love,

Amy, Klane, Madeline and Susannah

Friday, September 26th, 2003

Needles, Nurses and Nerves


Hello, Hello,

This week has pretty much been a fact finding mission in regard to Susannah’s new found hemolytic anemia. Our days have been filled with finger sticks and venopunctures (ouch … no line!), awaiting lab results and conversing with doctors to determine how best to proceed. We are literally taking things one day at time, checking daily labs and then altering course accordingly. Fortunately, save for one morning when she was seen in clinic, Susannah has been able to maintain her normal home routine, with the addition of homecare nurses who visit the house each afternoon to play vampire. So far she has been able to stave off another blood transfusion, but we are still waiting to see where things are going to settle out.

Following last week’s mega transfusion, Susannah’s hemoglobin has been on a downward slide. She did receive an incredible bump from that batch of blood, so luckily she does have a ways to fall. Overall she has been dropping a gram or so a day over the last week, but by week’s end things had settled down somewhat and her hemoglobin appeared to actually be holding steady. Our hopes were dashed, however, when today’s results showed an overnight drop from 10.6 to 9. On a more positive note, our girl’s platelet count is back on the rise, which Dr. Davies tells us could be a very good sign, as platelet activity can often be an indicator of things to come. We will just have to see where things go from here to determine if her steroids need to be further increased or if additional medications will be required. At this point we continue evaluating things day by day, watching her hemoglobin and making a daily plan from there.

For the time being we have been able to hold course with the increased steroid dose implemented last week. Prior to the onset of this issue, Susannah’s prednisone had been weaned down to five milligrams every other day. Last Thursday when the hemolytic anemia set in she was increased to an everyday dose of five milligrams. While daily steroids are much more dangerous than an alternating day regimen, we are just thankful we haven’t had to increase the actual dose even more. Normally in a situation such as this, high dose steroids are called for, however it was thought that since Susannah has been on them chronically, perhaps a more modest boost would do the trick. As the issues continue to play out, we may well need to increase her steroids again in order to get things under control. Alternatively, once things begin to settle back down with her hemoglobin, we should be able to return to her taper, albeit from a much greater starting point. When this will be however, remains a mystery.

This evening we had the pleasure of hosting two very special visitors, Cathy and Jake Peters from Edmonton, Alberta, Canada, the parents of precious Isobel who became an angel back in April. On top of our daughters’ common diagnoses of Hurler Syndrome, our two families have shared some incredible connections. One of our physicians in Minnesota became Bella’s transplanter in Calgary. Meanwhile, their good friend, who works as a travel nurse, often cared for Sissy during her hospital admissions here in San Diego and subsequently moved to Minneapolis and worked as a nurse on Unit 4-A. We know it was a bittersweet visit for them, but we admire their courage and dedication immensely. Although, we had never met in person before tonight, I think I speak for all of us (including Susannah, who was absolutely enthralled with Jake!) when I say it was as if we’d known each other for years.

Thanks, as always, for checking in on Miss Sis. We are forever grateful to have such a loving and loyal bunch of friends and family. Your unwavering support truly does wonders for our spirit, particularly in the face of new challenges.

Much love,

Amy, Klane, Madeline and Susannah

Saturday, September 20th, 2003

Something New and Different


Hi Again …

As always, just when we least expect it, our littlest princess decides to throw us a curveball. Suppose she will ever realize that we really don’t care for baseball???

Susannah’s routine monthly clinic visit turned into an all day affair, ultimately resulting in an overnight admission to the hospital. It seems her body has developed a condition called, hemolytic anemia, whereby her body produces antibodies that destroy her own red blood cells. This issue has varying degrees of severity and only time will tell which path Susannah is going to take. For now, we are going to begin to treat things, assuming the best case scenario, and add further intervention as things play out.

Sometime since her blood draw last month, Susannah’s bilirubin shot up … hence, the yellow eyes I mentioned on Wednesday. In and of itself this is not terribly concerning, but when her labs also came back with a very elevated white count and a hemoglobin of 5.7, hemolysis (breakdown of red blood cells) was pretty much a given. Just to rule out lab error, we did draw a second specimen, only to have that value return at 5.4. Normal is 14.0 and Susannah’s bottom-line parameter is 8.0. Basically, she needed a blood transfusion and she needed it NOW.

Further testing on her blood showed extensive antibodies, making it incredibly difficult to find an adequate match to transfuse her with. More blood samples, on the order of 40cc, were necessary to perform testing to locate a suitable match, very problematic considering her already low levels. By about seven o’clock it was apparent that red cells were a long way off, so Susannah was admitted to the hospital in order to be more closely monitored.

It took until the following morning to locate a useable match, by which time Susannah was white as a sheet, undoubtedly dropping her level even further with all the additional draws. The transfusion really pinked her up, not to mention tanked her up and she was discharged last night with a hemoglobin of 12.2. Quite a bump although not surprising considering the enormity of the transfusion she received, 400cc, twice the normal size.

The plan for now is to do daily blood draws here at home, to monitor her hemoglobin, and act accordingly upon those results. We should know in a few days the degree to which this is going to affect Susannah’s body. The basic treatment for hemolytic anemia is the following: 1) specially typed blood transfusions, 2) IVIG, which ironically she was in the process of getting when we happened upon this issue, 3) steroids. Normally the steroid dose necessary to treat this is quite significant, but seeing that Sis has been on them chronically, Dr. Davies wants to first try just increasing her normal dose to every day, as opposed to every other. Tolerance will be very low for going up, but there is really no harm in starting slowly, as it appears that the onset of this whole thing was actually rather slow. If things do not become controlled with increased steroids, there are more significant interventions, both medically and surgically, but we will address those issues when and if the time comes. For now we’re trying to remain optimistic.

It is rather rare to develop this situation so far out of transplant, but then again Susannah has a propensity for doing the unusual. In the transplant population in general, it is a somewhat “common” occurrence, but usually within the acute transplant phase or at least in the first year. It turns out, however, that hemolytic anemia is far more prevalent in metabolic children, a category in which Hurler Syndrome certainly falls. Interestingly, however, it can even manifest in healthy children (and adults) following a viral illness. Obviously, it tends to be more severe in the immunocompromised.

To add more salt to the wound, it seems Susannah is now having a major flare of her chronic graft versus host disease. Hemolytic anemia is an auto immune reaction, much like chronic GVHD, so we are now wondering if one thing set off another. We did, however, make that move downward on her steroid just last week, so things are a little foggy. What is rather surprising, however, is that she has presented with such a significant outbreak in the face of additional steroids. Hmmm …

All and all it was a trying two days. In the absence of her central line, Miss Susannah was forced to endure two separate IVs, plus vampire pokes in the arm, twice in the fingers, as well as in the foot and heal. Through it all Susannah was a trooper … never mind that she herself was the cause of that second IV! When the two of us finally strolled out of the hospital at six o’clock last night, we were quite a site -- dirty, bloody and rumpled, she wearing mismatched hospital PJs and a cardigan sweater (white, when we arrived) and me wearing scrub pants and sandals.

On a much happier note, we had a bit of excitement earlier this week when Susannah used the potty for the first time ever!!! Hooray for the big girl! Of course WE all celebrated like crazy and SHE wondered what all the hoopla was about. While Aunt Tami was not here to sing Sissy her famous “Pee pee on the pot –tay!” song, “Ta Ta” most certainly did call up and serenade her over the telephone!

We’ll keep you appraised as things develop. Hopefully the situation will become more clear in the next couple of days. In the meantime, thanks for checking in our little lady and saying an extra prayer on her behalf.

Love,

Amy, Klane and Girls

Wednesday, September 17th, 2003

Back to School …


Hello Friends,

The school year started with a bang … Miss Susannah hit the ground running and is right back in the swing of things. She was elated to welcome her teachers back from their summer vacation and picked up right where she left off. Hopefully by next year she can GO to school instead of having it coming to her. She is learning quite a lot here at home, but the socialization of a school setting would surely do wonders for her, language and otherwise. We’re keeping our fingers crossed for next year!

We have also added another service to Susannah’s IEP. Beginning this month, she will start to receive input from the low vision program through the school district. While Susannah can see quite well, the trio of Hurler Syndrome, CMV retinitis and now progressive cataracts places her in the at-risk category. Thus, the school will now be following her as a precautionary measure. In our eyes, no pun intended, there is no such thing as too much intervention with our gal.

Another move downward on the steroids finds Sis getting closer and closer to making her debut back into the outside world a reality. Now don’t get too excited just yet, we are realistically at least a year away, but we are really beginning to see the light, dim as it may be. We still have several steps downward on the prednisone, before we even can begin to tackle the cyclosporine and then are looking at a few months to get that out of her system. Once all the meds are behind us, we then proceed onto the project of re-immunizing her, which will also take some time. Seems like a lot, but every step downward on the meds is a step in the right direction! In the meantime we’re holding out hope that the wean will continue to progress steadily and smoothly with little side effects and the recent IGG issues will remain in check.

Regarding the IGG, we’re off to clinic this week for another dose of IVIG. With her levels stable after her last draw, it seemed prudent to all involved to try extending the intervals to five weeks between doses. So far so good, but the levels will ultimately tell the story. This week’s scheduled visit is timely, as we’ve been noticing a yellow-ish, alternating with blue-ish tint to the sclera of Susannah’s eyes. Everyone is a little stumped as her latest bilirubin level was normal. And there are no clues at all to the blue-ish part of the issue. There have been hints that an ophthalmology appointment may be in our future to help get to the bottom of this.

Over the last month, we’ve added yet another med to Susannah’s regimen. As I have mentioned previously, the pulmonary folks in Minnesota decided to introduce an inhaled steroid since her systemic dose has decreased so significantly in recent months. While this has been the plan all along, the steroid dose only recently cooperated. So, now she is receiving a twice daily nebulizer treatment, Pulmocort. For those of you who are familiar with both of my children’s distaste for mechanical noise (hairdryers, vacuums, electric toothbrushes and the list goes on) this was not exactly something Susannah was fond of, or even wanted a part of, for that matter. However, several weeks later (thanks to lots of coaching and coaxing by June), Sissy has really become a champ at taking her breathing treatments. Dolly, of course has to take the first puff, and then, with the music blasting, Susannah dutifully holds the thing to her mouth, all the while dancing around the room!

Big sister Madeline is LOVING the first grade and in her own words thinks “it is WAY more fun than kindergarten!” There is even a “real live piano” in her classroom and they have been promoted to the “big kid playground”, where they can “play jump rope” every day! She has decided that school may actually be survivable without her best friend Lara … barely! Madeline thinks it is incredibly amusing that she is in Grade 1, Room 1 with her name being White and her teacher’s name, Wright. She and Mrs. Wright, have hit it off famously; we’re now just trying to determine which of the two possesses the driest sense of humor!

We are off to spend the weekend at Grammy and Poppy’s house to celebrate my dad’s birthday … HAPPY 70th BIRTHDAY, POPPY!!! Seventy years young and still playing tennis every other day! We love you ~ Poppy, Tom, and of course, Dad!!!

XOXO,

Amy, Klane, Madeline and Susannah

Monday, August 25th, 2003

The Dog Days of Summer


Hey There!

Susannah is lovin’ life without her line! Not only has she rediscovered the shower (giggling all the while as the water splashes her face!), she gets to enjoy lots of long tubbies with her big sister. She has even taken a few “dips” in the inflatable kiddie pool and has learned the sign for swimming and definitely uses it!

Otherwise, the little miss is completely obsessed with playing dress-up. Madeline graciously passed down some of her outgrown crowns, tutus, hats, costumes and of course, “clickies” (fancy shoes) to Susannah so she could have a dress-up chest of her own. We cannot get her out of the pink leotard and she is entranced with the way it’s skirt billows as she turns in circles. She literally insists on napping in it and lets us know she disapproves when we insist she remove it. Her therapists all come to the door each day in anticipation of what get-up she will have on. It really is a crack-up. While the whole dress-up idea started as pure fun, it has actually become a great OT tool, helping Susannah learn to dress herself and button and zip her clothing.

Move over Barney and the Wiggles, Elmo is here! Susannah’s latest pastime is her daily exercise routine with her new friend Elmo. She LOVES to “Elmocise” and gleefully moves her body to the rhythm and follows the steps as Elmo and others call them out. She is by no means a TV junkie, yet she does now enjoy a variety of different videos. While I am not a big advocate of television, I have to admit that I actually see my darling daughter’s expanding repertoire as a sign of her emerging intellectual skills. It used to be that she’d only watch Barney, and then only episodes she was familiar with. Nowadays, she not only shows interest in a variety of subjects, but she actually correlates what she sees, with things in her daily life. The other day while watching Teletubbies, she walked over to her dress-up chest and retrieved a tutu, identical to the ones the characters were wearing. After asking Klane for help putting it on, she proceeded to dance around, just as she saw them doing on the show!

Perhaps fostered by her fascination with Elmocise, although actually a precursor to it, jumping and hopping have become a favorite activity around our house. Susannah is constantly jumping in place (okay, so she only gets an inch off the floor, but it is air nonetheless!) and hopping like a bunny, and all and all keeping the rest of us on our toes! She thinks this is great fun and really delights in the response she gets from those of us who are always willing to cheer her on. It is so cute to see her elation when she knows she has actually cleared the floor! We are working hard during PT at getting her to actually jump off of a small step, where she still insists on going one leg at a time. All in due time, as I recall not to long ago one of her therapists saying, “Well, she’ll never run and jump, right?” Oh, how sweet it is to see Sissy prove them wrong!

As for potty training, that mission was put temporarily on hold. While summer would have been the perfect time (for running around naked outside and all!), our little sojourn to the Sheraton made things a little more complex. Hence, we nixed the commencement of this lovely task until Susannah was back home in a more normal setting. Once we get settled back into our school year routine, we again plan to try our hand at things. In the meantime she still loves the potty book and, of course, accompanying us to the bathroom.

In spite of the departure of our two wonderful speech therapists, Susannah continues to make tremendous gains in her language skills. She is signing like crazy and only seems limited in this area by the adults in her life having to continually search for new signs to teach her. Her receptive skills are constantly emerging and she seems to understand more and more each day. She is even beginning to master two-step directions, which is a huge milestone. As is quite common in children with Hurler Syndrome, her verbal communication continues to lag far behind, yet she is becoming more and more comfortable making sounds and putting together primitive words. As best we can tell, she has a sign or a “word” for more than 150 different words and this list is constantly evolving. She knows, recognizes and verbalizes sounds for her numbers 1-10 as well as the 6 colors of the rainbow, and of course, pink! Today, she actually asked for a drink, with words, SPONTANEOUSLY! She said “I want drink please” with the only sign in the bunch being “drink”!

As I mentioned, we bid farewell this summer to two wonderful speech therapists, Nicole and Josh, both of whom have relocated to different a different country and state, respectively. While we are ALL very sad to see them go, we are very happy for the opportunities they have been offered. Both have gone out of their way to make certain that Susannah will be well cared for in their absence and for this we are enormously grateful. We will miss them both and wish them well.

Susannah and I headed to Children’s Hospital last week for her first IVIG infusion here in San Diego. What was to be a four hour infusion quickly became an all day affair, but that was pretty much par for the course. While they were actually able to get her IV in on the first try (no small feat with Sissy’s tiny veins), they did have to stick her numerous times to get enough blood for her labs. With any luck things will go smoother next month, although, considering, Susannah did quite well. The big glitch of the day came when we realized the IV had been placed in her right hand aka her thumb-sucking hand! This did make for a long day without a nap (no napping without that thumb!), but the two of us spent lots of time snuggling and, of course, Elmocising! At one point, when things got a little desperate, and because she asked so nicely, I did treat the princess to a chocolate bar, and all was forgiven!

Susannah’s lab results proved to be very stable. Her hemoglobin still has not quite recovered from last month’s blood letting, but it was acceptable nonetheless. As for her IgG level, it did show improvement from last month, but not enough to warrant cutting back or stopping the IVIG. On the day that the lab was drawn, it had not quite been four weeks since her last infusion, and since the drug has a six-week half life, it is likely that she had a good amount of residual still left in her system from her infusion in Minnesota.

It seems Susannah’s body didn’t quite handle last month’s drop on the steroid dose as well as we’d hoped. At first all seemed fine and her skin/hands actually looked better than they had in a long time, but about three weeks into the new dose, Sis actually exhibited a mini-flare, complete with a peel; so we’ve decided to wait it out another month before attempting to proceed with the taper. It may be a fluke, or we may be to the point where we have to revert to proceeding slowly once again. We did notice after both IVIG doses that the condition of her hands seemed to improve, but we have been told that this is likely just a coincidence; still we will continue to keep a mental tab on this, as Susannah so often has had her own way of doing things. As for continuing the wean, we will hold steady at the six milligram dose for a while longer, at least a few weeks. We continue to remain hopeful that this is just a bump in the taper road.

Back on the home-front, we finally had to bid farewell to my sister, Tami (aka “Aunt Tami” or in Susannah-speak, “Ta Ta”), as she boarded the plane to join Steve and the boys for their year long stint in Rhode Island. We were lucky enough to have her as our houseguest these last few weeks while she tied up a few loose ends at work here in San Diego. We will surely miss having her so close by, but are keeping our fingers crossed that her family will be making their way back west once the year is out.

As summer is winding down, the new school year is upon us. Beginning September 2nd, Miss Susannah’s busy schedule will kick back into high gear. Truthfully, this month has been a nice break with a few less people coming through our door each day. Still, we know how very much the services she gets help her progress and thus, we welcome the school crew back with open arms.

Also on the second of the month, we will officially have a first grader in our midst. Madeline has had a wonderful summer, and is very much looking forward to moving onto fall. She has already started soccer practice (and is now in the big girl league, complete with goalies!) and ballet will begin again next week. At her annual physical earlier this month, Dr. Boies found her to be the picture of health. She is really growing up (literally) and topped the growth charts by plotting out in the 95th percentile in both height and weight! She certainly takes after her daddy, and at this rate should be looking down on me by the fourth grade! Perhaps the biggest news however, does not have to do with her height at all, but rather her mouth. Madeline FINALLY has a loose tooth! This is big news for her, as she is convinced that she is the very last person on this earth who has not had a visit from the tooth fairy and certainly the world’s only six year old! Stay tuned for further reports, as the darn thing has already been wiggling for three weeks and still does not appear to want to budge!

Wishing you all a relaxing and Happy Labor Day weekend!

All our love,

Amy, Klane, Madeline and Susannah

Monday, July 28th, 2003

Another Busy Week in the Twin Cities!


Hi All,

After six weeks of living in a hotel, we packed our bags and headed to … another hotel! Yep, that’s right, our house was still being repaired when it came time for Susannah’s bi-annual check-up in Minnesota, so off we went! We had a nice stay in the Twin Cities and, as always, Susannah handled things like a trooper!

All and all the reports on Miss Susannah were favorable. First things first, she remains 100 percent engrafted with donor cells and her enzyme activity level came back at a solid 48.2. In general her counts and chemistries looked good, with the exception of a low-ish hemoglobin, no doubt attributed to all the extra draws throughout the week.

Her ears looked wonderful, as always and we even staved off the audiologist until this fall when we will return for her three-year post transplant studies. She also was seen by both Drs. Charnas and Lockman, who were pleased as punch with her neurological status. While Susannah did a great job keeping them entertained, as usual THEY were equally impressive at entertaining Klane and I! Those two are quite the characters. Their latest prank involved convincing the poor medical student that it was imperative that he locate and review Susannah's entire inpatient chart ... all 16 volumes!

Moving onto her heart, things here checked out just fine and in fact the reports in this area just keep getting better and better! And to top things off, the echocardiogram was the only test of the week that Susannah appreciated. Her pal Jim, the echo tech (they go WAY back), popped in Barney in Concert and she lay perfectly still for him (to give even more kudos to Jim, he even managed to get the EKG tech to come to the echo room, so as not to ruin a good thing!). Quite a feat, considering it followed three solid days of testing torture!

Seriously, overall this visit, was exceptionally irritating for our little lady. The pain started at Monday morning’s EMG, with which she was none too pleased. Tuesday began bright and early with a growth hormone stimulus test, in which Sis was kept NPO for the majority of the day and was given medications to make her drowsy, but was prevented from sleeping due to the need to draw blood every 20 minutes for about six hours (and all this after she had already been drained with the usual Monday labs).

The eye guys were next and their torture actually spanned two full days. The good news opthalmologically is that her retinas are stable with no signs of active CMV. Dr. Olsen was thrilled to see her increased awareness, although it certainly made for a tricky exam. On the flip side, Dr. Christiansen found Susannah’s cataracts to be increasingly worsened since our last visit and feels the need to surgically intervene will present itself sooner rather than later. Additionally, although her acuity exam was essentially the same as last time, he feels that with the increasing fuzziness that the cataracts are contributing, it may be wise to consider glasses. At present, however, the issue has been tabled and we will access things again in the fall. In the meantime, Dr. Olsen actually felt that introducing Susannah to sunglasses may help her with the uncomfortable glare the cataracts are undoubtedly creating, while at the same time helping her adapt to wearing something on her face. As for the usual Hurler issue of corneal clouding, that remains in check at "one plus" -- Christiansen lingo for not too shabby.

Sissy had the usual x-ray panels for pulmonary and orthopaedics with a few extra ones thrown in for endocrine. While these are normally routine and well tolerated on her part, Susannah was none to happy to enter the Radiology Department this go-around. To make matters worse, she was subject to two different kidney ultrasounds, as ordered by nephrology. Dark rooms and big machines, were not her friends this week, to say the least! She did however, hold true to her usual bright personality, when after bawling her eyes out and wildly squirming away from all of these folks, she’d sign “all done” and with tears still in her eyes, walk over and offer the guilty party one of her beloved Cheese-its!

We met up with Dr. Kim, the nephrologist, this trip to address the convoluted issue of Susannah’s kidneys. As expected, he was not at all concerned that their size was smaller than normal, since she too is smaller than normal for her age. He did, however, find the discrepancy in their size, as related to one another, a bit bothersome. He ordered additional ultrasonic testing to rule out the possibility of reflux and it seems things with her “beans” are just fine, albeit a little unusual. However, he will now become part of her regular follow-up.

Susannah was also evaluated by Dr. Milla and, our favorite pulmonary fellow, Spike. While her lungs do sound “stronger” with lessening crackles, “Uncle Carlos” again reminded us that in order to see any marked improvement, she really needs to grow and expand her chest cavity. With her steroid dose being lowered significantly since our last visit (we were able to drop the dose again this month!), the team did prescribe inhaled steroids (via a nebulizer) to help fill the gap and keep things on track. They also stressed the need to keep her saturations up and have a low threshold for administering supplemental oxygen. Of course, the need for oxygen while flying was a given as she’d already more than demonstrated her requirement on the flight out.

As for orthopaedics, things remain status quo in that there has been essentially been no progression in Susannah’s spine, hips and knees. Of course, this was not terribly surprising since she also has not grown a stitch in over a year. We were fortunate to get to be one of Dr. Ogilvie’s very last appointments, as he is retiring next month and will certainly be missed. Speaking of growing, or lack there of, we also saw the endocrinologist this visit. Dr. Petryk discussed the benefits of growth hormone for children on chronic steroids, as well as the potential benefit to kids with Hurler Syndrome. She did mention that growing could and would likely cause some of the orthopedic issues to worsen and be brought to the forefront, but Dr. Ogilvie was not concerned about this, stating that growth was very important and we’d deal with the ortho issues when and if they were affected by it. Since her growth hormone studies revealed that Susannah was actually producing a normal amount of hormone (there was some fear that due to radiation, she may not be), Dr. Petryk felt that the main contributor to Susannah’s lack of growth was indeed the steroids, although the her underlying diagnosis certainly plays a role as well. A number of other radiographic bone studies were performed, but will mainly be used as benchmarks and comparisons in years to come. She did want us to supplement Sis with extra calcium and vitamin D. While we are still awaiting her final recommendations, we will sit tight for now and readdress the growth hormone topic at our next visit. Along these lines, with Dr. Schwartzenburg out of town, the GI follow-up was also tabled until the fall. Ditto for Dr. Van Heest, although, she will be evaluating the EMG in the meantime and contacting us with any concerns; we do not expect any, as Susannah’s EMG was actually stable, if not improved.

Now for the big news ... drum roll please! Susannah is LINE-FREE, for the first time in three years!!! One of the highlights of our trip to Minneapolis this summer was the anticipated removal of Susannah’s two-year-old PICC line. In fact, Madeline was so excited at the prospect of her sister being line-free that she made certain that everyone on the plane, in the airport and at the hotel was well aware that, “Sissy is getting her wires out at her work in Minnesota and then we can take a bath together, finally!”

While the line was still being utilized regularly for blood draws, it’s past history of infection, the fact that it had been sutured down so many different times (Susannah’s poor forearm was beginning to look like hamburger), and the sheer idea that it was so old, the risks of keeping it in were really beginning to outweigh the benefits. As Dr. Davies put things, “It's been a good friend to us for a very long time, but it can only endure so much, so better to get it out on our terms than have it backfire on us.” Thus, even before leaving California, we had predetermined that removing Susannah’s line during our check-up would be a prudent plan and we’d go ahead and give monthly sticks for labs a shot. Not exactly enjoyable for the little one, but the hope was that the other freedoms she would enjoy, sans line, would more than make up for the added pokes.

As is so often the case with Miss Susannah, the issue of her PICC line was not as simple as we’d expected. When one of her immune studies, IgG, came back quite low, our plan came to a screeching halt. While a low value in this area is quite common in post transplant children, believe it or not, it had never before been an issue with regard to Sis. The concern now was that this low level could potentially be a sign of her chronic GVHD worsening. On the other hand, it could also be a transient thing that would remedy itself. Still, we had to be safe; Susannah would need to receive monthly infusions of IVIG, and to do so with a line would be so much simpler. Her first infusion was set-up for Friday morning. The catch, however, was that we were to be in Dr. Saltzman’s office on Thursday afternoon to remove her line (it had been in place so long that everyone else was afraid to remove it, as they didn’t know what to expect). After canceling our appointment with Dr. Saltzman, at literally, the eleventh hour, we conferred with our trusted trio, Drs. Davies, Ramsey and Grewal (aka Sat), and after much debate decided the line needed to go. It’s age (actually, a record for a PICC line) and precarious anchoring made it both a high risk for infection or otherwise failing. Finally, after much ado, the line removal, with our favorite Barney singing surgeon, was back on for the last appointment of the week, immediately following our trip to clinic for a little IVIG. Alas, Madeline was not disappointed!

So, as far as the IgG goes, the plan is this. Susannah will continue her monthly clinic visits, at which time she will have a peripheral IV placed (and hopefully draw labs in the same stick), through which she will be infused with IVIG. If her pre-infusion IgG levels come back in the normal range, then we will space out the infusions to determine if she can actually produce enough IgG on her own without the boost. If, in a few months, it looks as if her need for IVIG will be long-term, then we will discuss having Dr. Saltzman place some sort of access during our next visit to Minnesota (likely a port-a-cath). Hopefully, our gal can kick this of her own accord and it is indeed just a passing thing.

Whew! Now that I’ve rehashed all of the details, I’ll move on to the more exciting stuff. As jam packed as our visit was with medical business, we did manage to squeeze in lots of socializing! Madeline, who declared herself as “tired of being Eloise at the Plaza”, immediately retreated to Joan’s house for the week, only to be lured back briefly for visits with Jessica (Moore) and Kelsey and Gayle, her big-girl college friends. As usual, she and Joan had a “lovely” time doing all of their favorite things (swimming, tea parties, going to the library and just being together!) and especially some new and exciting things too -- playing with Joan’s new puppy, “Primo”, and visiting Gibbs Farm, a real-life “Little House on the Prairie”. Madeline was kind enough to share her special friend with us and we all enjoyed a very nice evening at Joan and Gary’s house together. On Sunday morning, Joan and Lyn Lawyer from St. Matthew’s were even able to make special arrangements for our entire family to attend church. With Joan and Madeline in the pews and Klane, myself and a masked Sis hidden away in the side chapel, it was the first time our family had been to St. Matthew’s at the same time since before Susannah’s first transplant! It was so nice to see all of the wonderful people who took such good care of our family during our stint in the Twin Cities and for them to see Susannah out and about.

Like her sister, Susannah had her share of fun as well. Of course, we visited with many of our nurse friends and therapists up on the unit and in clinic, which, as always, was a treat. Back at the hotel, we got to catch up with Jane and Maureen (and their boys), our therapists and friends who are no longer working at the hospital. We too got to spend a lot of time with Kelsey and Gayle, which we wouldn’t have missed for the world. Special thanks to Kelsey for squeezing us in between studying for her nursing boards and the first week of her new job on the pediatric bone marrow transplant floor. Yep, our Kelsey is now a full-fledged nurse up on Unit 4-A!

Our hotel room was Hurler central this trip as we not only snuck in our usual visit with the Moore’s, but also got to spend the afternoon with the Wigglesworth’s whose daughter, Maddy, is a year older than Susannah and four years post transplant. Like Jessie, Maddy is a star! We also had a wonderful visit with a new family, just beginning the transplant process, the Ciacciarelli’s. Mighty Max is a real cutie and he and his big sister Grace had a fun time playing with the girls. Madeline and Grace even roped Max’s daddy into playing “beauty shop” with them!

We also made a new discovery this trip to the Radisson. The hotel has converted their fine dining restaurant into a lounge with gourmet hors d’oeuvre. I know this doesn’t seem very exciting or even relevant, but the deal is that the new lounge leaves one entire room of the former restaurant abandoned. Well, on our second day in the hotel, we approached the bartender and asked if he’d be able to set a special table up for us in the private room. Sure enough he could and he did (complete with highchair) and voila, we had our own private restaurant! It was quite a luxury, nearly an entire week of being able to dine “out” with Susannah in tow, something we never get to do. Needless to say, we became regulars and knew the little menu by heart by week’s end!

Upon our return from Minnesota, we couldn’t help but do a little celebrating. We left the airport (having flown into Orange County) and headed straight to Grammy and Poppy’s house. Sans line and with Dr. Davies blessing, Susannah had her first adventure in the swimming pool! It was a blast to watch her and see the look on her face as she splashed about. Of course our fun was limited only to the steps (we’d been given explicit instructions to keep any water from getting into her sinuses or digestive tract and only to venture into private pools), but it was indeed great fun for all. Susannah is a master splasher and quite fond of her swimming suit and proudly models it for anyone in sight. Let me tell ya, it’s quite a look she’s got going with her "fur"!

And, we had even more to celebrate! Thanks to LOTS of work by Grammy, in our absence, we were finally able to move back into our house! While we were busy in Minneapolis, Grammy oversaw workmen, painters, plumbers, tile and carpet layers and ensured that everything was all set for our return. After seven long weeks, we are finally home!!! Ahhh, what a wonderful feeling ...

Home at last,

The Whites

Friday, June 27th, 2003

Where to begin???


Wow!

What a wacky, wild, weird, yet wonderful whirlwind the last few weeks have been! First and foremost, Susannah is doing exceptionally well, even tolerating another move downward on her steroid dose with little to no effects (going on four weeks now). We are finally beginning to make some headway in getting the dose down to a less toxic level, relatively speaking. In fact, her dose of prednisone has been reduced more in the last six months than it has in two years! Otherwise, our girl’s blood counts and chemistries have been very stable with no cause for concern. I dare say she weathered this year's viral season quite well. Onto summer ... a much safer time of year!

June began with much excitement in anticipation of welcoming home Uncle Steve and his troops from Iraq. We ALL donned our red, white and blue and made the trip up to Camp Pendleton for the midnight reunion. Despite the crowds, even Susannah was able to share in the moment (she waited in his office), and proudly helped us display our fancy homemade banner: “Welcome Home, Uncle Buzz!” Just two weeks after his homecoming, we were back on base for Steve’s change of command ceremony, which unfortunately for us, means that the Ferrando clan will once again be on the move soon -- this time all the way to Newport, Rhode Island!

The next big event was Madeline’s graduation from Kindergarten! It was a big day, the highlight being our daughter’s drawing gracing the cover of the “Celebration of Learning” program. Unbeknownst to us until that day, the teachers had held a drawing contest and Madeline won. She’d sketched a little girl (complete with big bow!) singing, just as the children did at the ceremony. And sing she did, during the program, she not only sang her little heart out, but she was also in charge of leading the flag salute! Other end of the school year milestones, included “bridging to Brownies” (from Daisy Girl Scouts), a soccer medal and lots of assists, or “insists” as she refers to them, amazing performances at her ballet recital on the big stage, as well as the Del Mar Fair. On tap for the summer are swimming lessons (which have already begun and boy, is she a little fish!!!), vacation bible school, ballet camp and, of course, sports camp up at Grammy and Poppy’s house.

Susannah’s summer schedule is busy as usual! While, the regular school year ended on June 13th, three days later summer school began. The eight week summer program ends just in time for our trip to Minnesota and upon our return, there is a month long gap in her program, providing a little added free time (aka park time!) until the school year begins again in September. During this time will have to tack on some of our own “school time” to be sure that she continues to progress during the summer hiatus. Thankfully, her medical therapies will continue throughout.

This month also marked what was to be the end of Klane’s residency. It was a bittersweet evening as we bid farewell to the other three graduating chief residents, and their families, who we began this adventure with six years ago. While it was quite strange not having Klane up at the podium along with Roger, Tony and “Uncle Phil”, we would not trade our time together as a family for anything. Yes, part of me (and I, of course, speak for Klane here too) wishes that the grueling demands of residency were behind us, but a far bigger part knows that we made the right decision in him to choosing the path he did. Not only did we have invaluable family time when we needed it most, with Susannah still juggling transplant issues, the extra time here in San Diego will surely be quite a blessing.

To make our exciting life even more so, we are currently living in a hotel! One of our toilets overflowed and subsequently flooded a good portion of our house. On doctor’s orders (not to mention common sense), we packed our gear and headed to the Sheraton Hotel Harbor Island, where we have been living since June 3rd, with no definite end in sight. Both girls LOVE the excitement of our new digs and have developed quite a fondness for hotel life. Madeline is convinced she has inspired a sequel to the book “Eloise at the Plaza” … “Sisters at the Sheraton” (pronounced, in Madeline jargon as, “Shera-taaaaawn”).

Now that school is out, things have become much more simplified, but in the beginning the added commute from downtown was a little tricky. Susannah hasn’t skipped a beat, however, as all of her therapists, nurses, teachers, and of course, medical paraphernalia have been redirected to the 11th floor! Although I won’t say it is convenient, things have actually fallen into place quite well and we’ve developed a sort of routine. Truthfully, after spending so many months in 10x10 hospital room, our adjoining rooms with a harbor view weren’t too difficult to get used to. Our experience in Minnesota has made our family quite adaptable, so honestly, after living in a hospital for so long, hotel life isn’t all that bad!

As I said, Susannah has transitioned well to being cooped up in the hotel, making the most of the restaurant style high chair, pack-n-play and the changing area, formerly known as a desk -- it’s amazing the adaptations one can come up with in a pinch. The hotel stairs have quickly become the highlight of Susannah’s stay, not to mention the highlight of many a guests’ stay too, as she is particularly fond of entertaining the breakfast diners as she climbs the dining room stairs each morning during PT! I am certain that she knows exactly where every single set of stairs are in the entire hotel and she would go up and down them all day if we’d let her! She often gets such a work out that we have to force her to call it quits as her respirations go way up. Still, day in and day out, all day long, the little miss begs to climb them and has even learned a new word, “staaaa” aka stairs!

The girls have definitely won the hearts of the hotel staff. Miss Susannah has made quite a name for herself as she rides the hallways on her tricycle waving to the passers by. Naturally, Madeline is no different as she comes and goes from her many activities attracting lots of attention in her tutus, girl scout uniform and swimming suit! It seems everyone in the lobby knows her social schedule! The room service staff is most accommodating and gets quite a kick of sending up kid friendly snacks.

Madeline’s favorite thing about the hotel is, believe it or not, sharing a bedroom with her sister! They are so cute … after the nighttime routine, when Susannah stands up in her bed, Madeline calmly says, “Lay down Sissy, it’s time for night-night,” and Susannah dutifully obeys. Definitely not the same reaction her daddy and I get! The other nice thing about the shared room is that it seems to have cured Susannah’s fear of the dark. Just before we had the flood, Susannah was having a really hard time getting to sleep at night and we deduced that she'd become afraid of the darkness. Solution: We got her a little duck nightlight and presto, she was happy as a clam. The cutest part is, every night she reminds us to turn it on! Now, with Madeline in the room too, it is an added bonus.

In other quirky Susannah news, she has developed a love for the Wiggles and even has created a sign of her own to indicate her preference to listen to her newfound Aussie friends. While she loves listening to them on CD, she is completely entranced by the video. She dances like crazy and follows along with all the actions and has really improved on her hopping skills since discovering these guys. While her favorite character is clearly the Captain, for some reason she is convinced that the big goofy guy is Klane and incessantly refers to him as “Da Da”!

Well that about sums up our June … never a dull moment for the Whites! We return to Minneapolis the week of July 13th for Susannah's bi-annual appointments/evaluation. We’re looking forward to lots of good news and visits with good friends.

With love from the “Shera-taaaaawn”,

Amy, Klane, Madeline “aka Eloise” and Susannah

Thursday, May 15th, 2003

Happenings Around Here


Greetings!

It has now been better than a month since Susannah’s last admission and she couldn’t be feeling better! Her post antibiotic blood cultures came back free of infection, so it appears that we were able to nip things in the bud this time. With any luck she’s closed the door on this year’s viral season once and for all. Still, recollecting last June’s late breaking fever, I think we’ll lay low and keep our fingers (and toes!) crossed for a while longer. The transition to monthly clinic visits has gone smoothly. While we still deliver weekly blood work, it’s sure been a nice break having extended time away from hospital appointments. Susannah’s lab values have been quite stable and her skin looks very good. So good, in fact, that if the trend continues over the next couple of weeks, we plan to proceed with her steroid taper at the end of this month. Slowly, but surely she is getting there.

Susannah is now showing an interest in the potty and more precisely, the video “Once Upon a Potty”, to which she delights in dancing along with the singing kids to the catchy theme song. She is more than happy to invite herself into the bathroom with anyone willing to tolerate her presence and wave bye-bye to whatever they happen to be flushing down the toilet. If per chance, there is nothing to flush, Sis is quite willing to help out by ripping paper off of the roll and tossing it in the toilet. Needless to say, we’ve been going through A LOT of extra toilet tissue lately! The little lady is also quite keen on removing her pants and then running around the house clapping in search of the potty, although we’ve yet to see any official action. In light of these events, however, we have acquired a box of pull-ups and a potty chair to see exactly where this new infatuation might be headed. With summer and warmer weather approaching, who knows what will happen?

On that note, the joys of toddler-dom continue in full force. In addition to her perpetual response of “NO”, which comes in the form of an adorable little headshake, Susannah has also developed this lovely habit of whacking people on the arm. She doesn’t do it out of malice, but rather excitement and to gain one’s attention; still, it is a little unladylike nonetheless. Most often the recipient of these wallops is Madeline, who, ever the protector, insists that the not so little pats are actually “signs of love”. Yet, in spite of Big Sister’s defensive protests, we now find ourselves constantly reiterating the importance of gentle and soft touches to our youngest daughter. Her newfound defiance is most amusing and any trace of aggravation she might insight, she makes up for in cuteness … particularly when it comes in the form of her newly acquired, very precocious foot stomp!

This week we finally wrapped up Susannah’s IEP for the upcoming year. Dealing with the bureaucracy and administrative hoopla was as tedious as ever, but in the end, Sissy came out on top, with the school district actually increasing the services in her educational plan. She will continue to receive preschool instruction, speech therapy, DHH services (a compliment to speech therapy), additional occupational therapy and the newly added music and physical therapies through the district’s Home Hospital Program. She will, of course, continue with her intensive private therapy sessions here at home, as well. Busy, busy, busy, but definitely worth the results!

Our spring trip to Minnesota, originally planned for May, has been postponed until this summer. Due to scheduling conflicts at Klane’s work (too many residents taking vacations the same week), we will push back Susannah’s bi-annual check up to mid-July. This actually works out well, as we were already planning our fall visit a couple of months later than usual so that it could coincide with Kelsey’s wedding in December. Much to Madeline’s dismay, we just miss the Fourth of July parade in Joan’s neighborhood, but by going a week later, we will be able to catch not only all of the necessary doctors, but most of our friends will be in town as well.

Speaking of Madeline, she is adjusting to life at school without her pal Lara. The first few days were pretty traumatic, as the two had been completely joined at the hip for so long. She gained a new perspective when I reminded her that my grade school friend, “Auntie Wendy”, also moved off to England way back when and we are still the best of friends to this day. On her first day back at school after Lara’s send off, the other little girls in the class really came together to support her, all 19 of them! One little friend actually made her a card and came running up and presented it to Madeline as she walked into class. The other girls lined up to hug her and remind her they were her friends too. It was so sweet. Even the mommies were asking about Madeline, as it was obvious to everyone just how close she and Lara had become. Needless to say, Madeline has now been introduced to the wonderful world of e-mail and the two girls have been sending each other messages across the Atlantic.

So far May has proved to be pretty eventful for our family and much to Susannah’s delight it’s even included a bit of “traveling”. Considering a simple ride in the car usually makes her day, our recent excursions have really brought about excitement for Sis, who continues to remain pretty isolated from the outside world. We began the month by heading up to Carlsbad to celebrate our youngest nephew/ cousin Nicholas’s First Holy Communion. My Godson was so handsome in his spiffy suit standing tall and regal reciting the day’s reading from memory. It was a really nice service, dedicated solely to honoring the children and this important occasion in their lives. Although Susannah could not attend the actual mass, she was ready and waiting back at Aunt Tami’s house for the family party that followed. To make a special day more so, we were treated to a phone call from Iraq in honor of Nick’s big event. Miss Susannah even got to talk to her Uncle Steve and the word is … he’s coming home in the beginning of June!

Then the following Friday, we headed up to Grammy and Poppy’s house for Mother’s Day weekend. While we all enjoyed our time with Poppy, we unfortunately missed celebrating this year with Grammy … in an odd twist of fate, my mother traveled to Louisiana to lay her own mother to rest on Saturday. Madeline and Susannah’s great-grandmother, my “Gramma Tom” passed away last Thursday at the age of 92. She’d lived a long and full life, leaving her imprint on the hearts of many. It makes me sad to realize that the girls will never have the opportunity to know her, but her memory will live on in all of us and it is that which I will strive to impart on my children.

As always, we appreciate you checking in, it means so much. Susannah sends big hugs, as do the rest of us!

Love to all,

Amy, Klane, Madeline and Susannah

Sunday, April 27th, 2003

Let them eat cake!


Hi Ho,

Whew! I think it’s fair to say that we are all “caked out” here at the White house! In celebration of the month’s events, our family has enjoyed a total of no less than eight different cakes! Madeline made out on the deal, claiming five of her very own. There has been a “tea pot cake”, a “duck cake”, a “bunny cake”, a “grown-up cake”, ice cream cakes and cupcakes of multiple varieties. We’ve lit so many candles I’ve lost count -- they have been placed atop cakes, cupcakes, left over cake, bagels, sandwiches, pizza and anything and everything else that could possibly hold them!

We began the festivities of “Birthday Week” a few days early this year with Madeline’s Tea Party on Saturday the 12th. With her best friend Lara’s family moving to London on the 18th, and the girls’ beside themselves at the prospect of missing out on each other’s birthdays (like Susannah, Lara’s birthday is April 15th) that we decided to push the celebration up a bit.

Now, not only did she want to share the day with her friend, Madeline also specifically requested that her party be here at home this year; but with Susannah’s still compromised immune system this presented somewhat of a challenge with 20 six year olds on the guest list. Fortunately, San Diego in April is quite conducive to outdoor activity, so between sending Susannah off on an extra long park outing and setting up a good portion of the tea party in our backyard, things really managed to fall into place. And just to be safe, Klane, being the trooper that he is, donned his tuxedo and played butler for the day, escorting the kids from the front door directly to the restroom to wash their hands. Our main intention was, of course, functionality, but it had the added bonus of making the kids feel pretty special!

Mrs. Crumpet arrived with a flare and dressed up the “ladies and gentlemen” in formal attire, from their hats all the way down to their shoes and afterward the parents were treated to the most adorable fashion show you can imagine! For the tea party itself, the children were enraptured by Mrs. Crumpet’s hysterical tales and fun-filled etiquette lesson, while they sipped “apple tea” from real grown up china. As if Mrs. Crumpet’s appearance wasn’t exciting enough, Madeline was absolutely thrilled when Miss Langfus, her kindergarten teacher, arrived to help her celebrate. It was truly a day she will never forget!

Next on the agenda was Susannah’s actual birthday. Her birthday this year was such a treat for all of us. For the first time, she really understood the concept of opening gifts and delighted in ripping the paper and pulling the ribbons. A little to Madeline’s dismay, Susannah no longer required her assistance with the presents! Naturally, the cake (and more importantly, the frosting!) was the highlight of the day. The birthday girl not only devoured her slice, but asked for more and more and more! Running a close second to her cake was the “kid champagne” aka sparkling cider … I’m still not sure if it was the bubbles or the fact that she got to drink it from a stemmed glass that was more exciting, but Sis just thought it was the cat’s meow! While Susannah didn’t quite muster up the wind to blow out her candles completely on her own, she did blow AT them and insisted they be relit again and again. By the end of the month, she had really caught on and at the mere mention of the word cake, would puff up her cheeks and blow!

The weekend brought about another celebration of Susannah’s birthday, along with Easter, and a family party at my sister Kerri’s house. With all the cousins present, it made for quite the fête and fun was had by all. We even took advantage of (almost) everyone being together and had a photographer stop by for family photos. It was a bittersweet occasion as the only person missing was Uncle Steve, but we were able to e-mail him some of the shots that very day, including his very first glimpse of little Nicholas in his new glasses. The highlight of the day for the kids was an egg hunt, which Susannah got the hang of very quickly, filling her basket to the hilt. Much to the exasperation of the big kids, she even spotted one of the two “sparkly eggs” (prize eggs) and conned Grammy into reaching it for her. Fear not, however, with Aunt Kerri in charge, everyone won a prize!

We culminated the month’s festivities the following Tuesday as we again celebrated Miss Madeline’s day. She started out her “real” birthday with a wonderful surprise -- the big six year old received her first overseas telephone call! Even far off in Italy, Joan couldn’t let the 22nd pass without sharing in her very special friend’s birthday and boy, did it make Madeline smile! As queen for the day (complete with a pink crown) she was honored at school, ballet class and once again here at home with Mommy, Daddy and Sissy. It was quite a busy day.

To add even more excitement to the month, we welcomed a new member to the April birthday bunch this year. Baby Jessica Walters arrived on April 9th and what a beauty she is! Jessica’s mommy is my life long friend Heather who just so happens to celebrate her special day one day before mine. Jessica was actually due on Susannah’s birthday, but decided to surprise us all by arriving a little early.

Finally, the month of April would not be complete without sending out our birthday wishes to the many wonderful friends we have met through Susannah’s journey who, ironically, were also born this month. Just what is it about April?!? Perhaps one of these years we will all be able to get together and celebrate with a very special cake and LOTS of candles!

Cheers,

Amy, Klane and the Birthday Girls

Tuesday, April 15th, 2003

Happy Birthday, Susannah!!!


Dear Friends and Family,

Sometimes in the day-to-day flurry of activity of all that goes on around our house, it is difficult to see the big picture. It is easy to get bogged down in medications, nursing needs, insurance, state and county bureaucracy, lab reports and never ending therapy issues. While we know Susannah continues to make progress and gains each and every day, when we look back to a year ago or even just six months, we are truly amazed. Of course, we’d be kidding ourselves if we began thinking all was normal here, but when the changes in Susannah’s personality and physical ability come full circle and look us straight in the eye, it is quite astounding. Yes, she is now four years old, and in no way, shape or form is she on equal ground to her peers, but she continues to make slow and steady progress in the right direction each and every day.

A year ago she would scarcely eat anything other than cheese and did so with only her fingers. Now she insists on using the appropriate utensils at all meals and she has a far more well balanced diet than most of America. If she is hungry or thirsty, she has no qualms about letting us know and is even able to lend a hand with the preparation (okay, so it isn’t always the most helpful hand, but a hand nonetheless). Of course, she will also express her dissatisfaction if our meal choices aren’t exactly what SHE had in mind. She not only insists on sitting in her big girl chair for meals, all but dumping the her high chair, but she gets quite perturbed if anyone else sits in HER chair!

Susannah now holds herself with confidence as she ambulates around the house, not only walking, but “running” from here to there. A little more than a year ago, crawling was her only means of transportation, yet now the only crawling she does is on top of things! Her little body gets stronger each and every day allowing her to continue to master the little milestones most of us take for granted. Our once distant wishes to see her standing on her tippy toes, squatting and hopping in place, are all now considered short-term goals. One day she longs to climb up the face of the slide like all the big kids do and within a week, and with the help of some proper shoes, she is doing it on her own. Of course, we watch her like a hawk, because she is different, she is Susannah. When she first set foot on her trampoline four months ago, she gave only a tentative bounce, now we can’t get her off! She bounces high and giggles again and again, and is only willing to stop in exchange for a ride on her “pony”. The very same spring horse she was unsteady on a few months back, is now eagerly ridden with one hand. Of course, another four year old would climb up on her own, and we have to lift Susannah’s tiny frame up upon the big horse, but it is those sort of differences that we have become accustomed to, making a simple pony ride all the more special.

She is really beginning to understand concepts and consequences. Her comprehension and cognition have improved ten fold. Sometimes this can be difficult to recognize, as her verbal communication is still remedial at best, but when we stop to count her signs, discuss her understanding and evaluate her progress, we are truly amazed. She is constantly “telling” us things and makes wonderful use of the emerging communication skills she has. If we could just get her immunosuppression under control, she would benefit greatly from interaction with other children, in some sort of a classroom setting. Right now, even her limited contact with the outside world makes all the difference. Her walks to the park help with not only her physical development, but her intellectual advancement as well. Once she is able to again be immunized and get back out in the world, we just know the sky’s the limit on the strides she will make.

In the meantime, this little girl is certainly not going to site back and watch life pass her by! She has already defied the odds to be here with us today and the medical world continually marvels at the accomplishments she has made, medically, emotionally, developmentally, let alone the sheer fact that she is alive. Susannah continues to make progress in all of her therapies, almost daily, and continually succeeds in melting the hearts of each and every individual who joins her team. She brings joy to all who work with her because her potential is so great and the results readily apparent, but most of all because of her love for learning and zest for life.

With Susannah turning four today, we are but a week away from Madeline’s 6th Birthday. Naturally, the older she gets the more aware she becomes that there are differences between her sister and the siblings of her friends. She longs for the day when Susannah can accompany us freely about town, to birthday parties and even to ballet class. Madeline has an amazing sense of compassion and loves Susannah unequivocally, making the questions that come from her curious mind all the more heart wrenching and awakening for her parents. We often ponder our responses, as we ourselves are unsure of just what lies ahead for our sweet Susannah. One thing is clear, however and that is the fact that Madeline is a doting big sister who loves Susannah without pretense. She is over-protective, understanding, defensive and yes, just a little bit bossy -- all the qualities an elder sibling should possess. When another child asks why Susannah wears that mask on her face, Madeline answers matter of factly, “because she had a transplant, silly!” The other day when one of her good friends inquired as to why her sister did not talk, Madeline, without hesitation replied, “She sure does talk, she talks with her hands, you know, sign language AND she’s teaching me too.”

While we know not what the future holds, we have learned to cherish the todays and yesterdays and not to worry about the tomorrows. Uncertainty can be crippling, thus we find much comfort in the old adage, “take one day at a time”. And on this particular day, we rejoice in celebrating our sweet Susannah’s 4th Birthday. Happy Birthday Sissy … you are our hero!

Love,

The Whites

Monday, April 7th, 2003

This time with a twist!


Good Morning,

Susannah and I returned Friday evening from yet another stay in the “Get Well Hotel”. It was much of the same old drill, only this admission our gal kept things exciting by throwing in a few unexpected twists and turns along the way. You know Sissy, she likes to keep everyone on their toes!

Just as the dust was settling from last Monday’s IEP meeting, Miss Susannah surprised us all by spiking a fever in lieu of taking her afternoon nap. Apparently the day had not yet provided enough excitement for her. With all the fireworks, it’s a wonder the morning’s meeting was not the cause of her fever, as it was surely the root cause of my headache! In all seriousness, the IEP actually went reasonably well, with a decision to table our discussions until further evaluations from the school district could be obtained. The long and short of it is that the IEP team is willing to increase Susannah’s intervention, adding school based physical therapy and augmenting her OT services. When we reconvene in May, we will have a clearer picture of her complete educational plan, but as of now it looks to be shaping up pretty darn well!

Okay, now back to the fever. Once all the necessary docs were contacted and the wheels in motion, Sis and I gathered our gear and headed for our home away from home, none other than Children’s Hospital. While she looked well and was acting relatively normal, during the admission work up, her temperature peaked at 103.1 and the pulse oximeter was alarming incessantly with consistent readings in the low 80s. After making a few adjustments to her toe probe, it was determined that the low saturations were indeed real, prompting the start of oxygen. I have to admit, it was quite unnerving, as I haven’t seen sats such as these for a long while, but perhaps more so because I was the only one calling the shots in such a precarious situation, “ordering” the O2, antibiotics on the double and even the Tylenol, because the admitting resident was more concerned with obtaining another H&P than the more pressing issues at hand, like breathing! Luckily our nurse, recognized the absurdness of the situation, followed my lead and got the job done, despite the inquisitive looks of the doctor. While Susannah’s need for oxygen continued through the night, with a little Tylenol on board and her fever at bay, the alarms from the monitor all but silenced. Amazing what reducing one’s fever can do, eh? I think our resident got a little life’s lesson that night about the wonders of Tylenol, not to mention mother’s intuition.

The daylight hours brought about a new challenge. Seeing that it was the first of April, I had really hoped someone playing an April Fool’s joke, but no such luck. When I reached in to get Susannah out of her crib, I discovered a very wet baby and a very wet bed. Initially, I assumed that she’d soaked through her diaper in the night, but upon further investigation realized that her left arm and the surrounding bandages were particularly drenched and the dampness was accompanied by an odd stench. Then it dawned on me: Ah ha! Her IV must be leaking! Well not only was it leaking, but much to my dismay, it was not even attached to her! To make matters worse, the awful smell of cefipime and the sogginess of her bed and dressing all but assured us that her overnight doses of vanco and cefipime never actually reached Susannah, but rather flowed directly into her bed. Adding even more salt in the wound, my next discovery was that her line was not even connected to the IV and in fact, was wide open to the environment, without a cap or even a valve! So, of course, more blood cultures were ordered, additional antibiotics given and her soaked dressing was replaced. And all this by 8:00am!

As I if we hadn’t had enough catheter ordeals for one day, when the nurse returned to draw the cultures from Susannah’s lines, she discovered that white port had not only been left open to the elements, but it was now completely clotted off and unable to be infused through or drawn from. Ugh! TPA was ordered, carefully eased into the line and, thankfully, a couple of hours later, it was back in business. Whew! Quite a close call and one we certainly could have done without, but all’s well that ends well.

The following day we were treated to an early morning visit from Dr. Roberts, bearing the lovely news that one of Susannah’s blood cultures had turned positive. She had grown “gram positive cocci in clusters” from her blue lumen; ironically, it was the white lumen that was compromised in the previous day’s events. The days that followed brought about the further specification and drug sensitivities, showing the final result to be our old friend, Staph epi, which of course, would need to be treated with a ten-day course of IV antibiotics. This latest version of the bug was actually sensitive to both Ancef and vancomycin, so in the interest of sparing Susannah’s kidneys, it was decided that Ancef would be the drug of choice.

Naturally, our stay at our winter retreat would not be complete without shift in lab values. The next morning, Susannah’s 4:00am labs showed her white count dump in half, dropping her ANC below the magic number of 1000. A little GCSF was ordered and proved to do the trick, but still we had to serve our time and be certain that things were stable before we could head home.

Finally, by Friday, the dust had settled, subsequent cultures were negative, her counts were up and Susannah was raring to go. What a good girl, just in time to spare her mommy and daddy from spending yet another anniversary in the hospital! Seriously though, she was released with the caveat of an every eight-hour antibiotic regimen, labs ordered every other day and follow-up blood cultures to be drawn once the Ancef therapy was complete. Quite a busy home schedule, but much better than the alternative, not to mention a drop in the bucket compared to some of our previous discharges back in Minnesota.

As seems to be the norm of late, juggling the schedules of Klane, Madeline and Susannah proved to again be quite a challenge during this admission. Special thanks to all of our amazing friends and family who stepped up and hosted spur of the moment sleepovers and extended play dates, not to mention got thrust into carpooling duties and homework patrol. Now, if only I can get Madeline to understand that school night sleepovers are a special treat only, and not a weekly occurrence!

Speaking of Madeline, as usual, she took it all in stride and she and her friend Lara even penned a special book during their free time at school entitled, “We Hope You Feel Better Susannah”. While the absence of their accompanying, adorable illustrations just do not do the book justice, their work is far too precious not to share. The following is the raw text:

We Hope You Feel Better Susannah
by Madeline White and Lara Smith

We hope you feel better Susannah.
Are you feeling better Susannah?
We hope you do.
We never want you to catch another fever, okay?
We hope you never have to go to the hospital again.
We love you Susannah.
Love, Madeline and Lara -- The End.

We busted out of the hospital just in time to find ourselves in the midst of the frenzied flurry of plans for the rapidly approaching “birthday week”. Thus far on tap is a tea party for 20 six year olds, as well as a family party for Sissy, not to mention Easter and the girls’ actual birth dates … look out April here we come!

Much love,

Amy, Klane, Madeline and Susannah

Sunday, March 30th, 2003

It’s a Grand Ol’ Flag …


Hello All,

While some Americans choose to wave their flags in support and others opt to protest US involvement in Iraq, both Susannah and Madeline have found their own ways to showcase their patriotism. At the mere site of an American Flag or when she is asked, “What do you do when you see the American Flag?” Susannah promptly slaps her right hand across her heart. Oh, how she’d make her uncle proud!

Miss Langfus’ kindergarten class, having already memorized both the Flag Salute and the Star Spangled Banner, brushed up on their letter writing skills by producing two dozen letters, addressed as follows: “Dear Uncle Steve”, “Dear Uncle Steve’s Men”, “Dear Troops of Uncle Steve”, “Dear Madeline’s Uncle Steve”. The children’s words of wisdom (complete with “kid spelling”) and detailed pictures are sure to bring about some serious smiles out there in the desert.

As you can tell, the safety and well being of Steve is weighing heavily on our hearts and minds. The reality of what is transpiring half a world away has really hit home and brings about the realization that the sinking pit we feel in our bellies has become our reality for the next few months.

Upon “decoding” very cryptic conversation between Klane and myself, about a downed Marine helicopter, Madeline piped in with “Boy, I sure hope Uncle Steve was not in that helicopter, because if he was he is going to have A LOT of boo boos when he comes home.” Oh, the innocence of a child.

Steve’s parting words to me, “Ammes, don’t you worry …” offer little comfort as the reports of the first Marine casualties trickle across the airways. While we hold our breath for word, we realize that “word” is exactly what we do not want and thus we live by the theory that no news is good news. Yet it is heart wrenching to realize that our own relief, often is another family’s tragedy.

Please say an extra prayer for “no boo boos”. God bless America!

With love,

Amy, Klane, Madeline and Susannah

Wednesday, March 26th, 2003

Déjà vu


Hey There!

Well, it seems our week with Kelsey reminded Susannah of her time in Minnesota ... a little too much! Late Saturday, the very day Kelsey boarded the plane for Minneapolis, our darling daughter spiked a fever, more than worthy of an admission to Children’s Hospital. When is she ever going to learn that friends from the frozen tundra and hospital life do NOT go hand in hand?!?

Fear not, this time we were admitted directly to the floor, no questions asked! Can’t imagine why? Once inside the very familiar, Room 111, I couldn’t help but experience a feeling of déjà vu. Things unfolded much the way they did last month, only this time Susannah’s fevers persisted for a good 24 hours and were accompanied by an ugly cough. Fortunately, her chest x-ray, as well as her lungs, proved clear and her viral battery came back negative. Despite these favorable findings, however, Sis did have a few incidents of unusually low sats, nothing too dangerous, but enough to cause some raised eyebrows and prompt a few questions. As for her counts, after remaining stable for the first 36 hours, we were hopeful they would hold their own, but instead they dumped in half, pretty much across the board. Fortunately, the decline in counts leveled off and her labs have since remained stable, albeit at a fraction of what they had been, but manageable nonetheless.

Still, with nearly 72 hours of negative blood cultures, the return of her upbeat personality and, of course, a little vanco and ceftaz running through her veins, Susannah was granted her discharge orders on Tuesday afternoon. However, not before earning herself a much deserved visit from her old pals, the doggies. Oh how she loves the Canine Care Program! This time she was actually able to wear her mask out into the hallway and see the dogs face-to-face without the glass barrier. The little lady was so excited she could hardly stand it. She giggled nonstop and, of course, barked right back at them!

This particular admission was a logistical nightmare, with Klane being on in-house call on two of the nights and in the OR during the days, but thankfully, Grandma Dona and Auntie Brenda were able to put their own lives on hold and lend a much needed hand. The two of them drove many a mile over the last few days to ensure that Madeline was tended to day and night, while I stayed at the hospital with Susannah. We simply couldn’t have managed without them!

Now, back to our visitors. All the drama surrounding Sissy’s hospitalization, could not overshadow the events of the previous week. Our time with Kelsey (and her friend Melissa) was just great. As expected, Madeline’s “big girl to-do list” kept things quite eventful -- there was dress-up, ballet class, visits to kindergarten, lots of baking, and, who could forget the, now annual, “Early Birthday Party”. Naturally, there was lots of wedding talk and we were even granted a sneak preview of Kelsey in her dress! Amid all the action, the real big girls did manage to sneak out and take in the sunshine and San Diego’s main attractions. Susannah, for her part, dazzled our guests with her newest achievements, kept them smiling at her silly antics and basked in the added attention. It was a delightful visit all around!

Much love,

Amy, Klane, Madeline and Susannah

Monday, March 10th, 2003

Rolling along ...


Hello Susannah Fans!

And she’s off! After months of rigging up every contraption imaginable to keep Susannah’s feet on the pedals, one day the little miss just climbed on her tricycle and rode down the driveway! She’s still working on steering, not to mention her dismount, which will surely come with practice, but in the meantime, thank goodness for helmets, on both accounts! When she received the trike for her birthday last year, I think we all quietly wondered if she’d ever really be able to ride it unassisted. In true Susannah form, however, our youngest daughter has once again reminded us just what she’s made of! She never ceases to amaze us.

From a medical perspective, things are rolling along, as well. Susannah’s labs have been stable since her admission last month and she’s managed to steer clear of the hospital. In fact, with all the winter bugs flying around, we’ve elected to decrease her bi-weekly clinic appointments to monthly visits in hopes of keeping her exposure to a minimum. Last week, we were able to drop her steroid dose down another notch, to eight milligrams. While she has shown some adverse effects of the wean, we remain optimistic that we can keep things from escalating with the addition of some extra topical steroids and avoid having to go back up on the systemic dose. Of course, neither the reduction in her clinic visits, nor the scheduled taper of her prednisone, would be possible if Susannah were not doing so well. Two more baby steps in the right direction!

Susannah’s achievements on her tricycle are just the tip of the iceberg. We see new lights going on almost daily. She is well on the way to learning her colors (she’s even starting to say them!) and is also beginning to understand the concept of counting. We regularly find her silently “counting” on her little fingers, upon hearing numbers spoken by others. Imaginary play in the girls’ mini kitchen is by far the latest craze. Susannah will happily con anyone willing, to sit at her table and watch her feed her baby with the wooden food from her fridge. She is so into this food game that her speech therapists have decided to use it to their advantage and embark on some new oral motor exercises using straws, peanut butter and even a chocolate milkshake! Yes, I said a milkshake! Lucky for the therapists their session only lasted 60 minutes, since the hours that followed made for one wild and crazy little girl! It must have done some good however, as while Susannah ran around the house like a banshee that night, she was making all sorts of new sounds!

Assertiveness seems to be Susannah’s new middle name. I dare say, she has officially entered toddlerhood. She has no qualms stating her preferences about EVERYTHING! From the food she eats, to the chair in which she sits, to the activities she engages in, our little lady has a definite opinion. Along these lines, Sis has taken a very active role in the dressing process, making her desires known in this area as well. In fact, just last week she insisted on wearing a t-shirt and a pair of shorts on top of her dress all day long. Selecting the perfect hat has become a staple in her daily routine and our girl emphatically refuses to walk out the door until she has the hat of HER choosing atop her head! The fashion bug has also developed a shoe obsession, fostered by a package we received from the Calaresos a few weeks back filled with at least ten pairs of Brianna’s outgrown shoes. Basically, Susannah now feels the need to try on a new pair every few hours!

Music therapy is going well and has proven to be a nice compliment to her more conventional speech therapy sessions. This intervention has really helped with stringing sounds together; so instead of getting an “mmm” and an “eee”, we are actually able to elicit a nice “meeeee” from Susannah ... when she wants to, of course! As such, this therapy will now be added as a permanent fixture in her upcoming educational plan. We’ve always known Sissy has a unique love of music and now the school district has officially recognized it too.

As I alluded to above, we are approaching the time to renew Susannah’s IEP with the San Diego City School District. We are scheduled to meet with school system administrators at the end of this month to evaluate and finalize her educational plan for the coming year. The assessments from her various teachers and therapists have already begun trickling in and I am pleased to report that Susannah has shown progress in all areas, and in many cases has left her goals from last year in the dust.

Not to be outdone by her baby sister, Madeline has become a wonderful little reader, right under our noses! She loves to read us book after book and constantly amazes us at the titles she has mastered. The level of performance expected out of mere kindergartners these days is mind boggling, yet Madeline does not disappoint. Miss Madeline has also achieved a cycling goal of her own, officially mastering the two-wheeler, sans training wheels. Since we live on a hill there hasn’t been much opportunity to attempt this, but after one afternoon on Grammy and Poppy’s cul de sac and she was riding like an old pro! And, thanks to her cousin Nick’s encouragement, she cannot only ride, but she can ride FAST!

Spring soccer is now upon us. The Red Dragons are reunited, only this time they have been transformed into the Blue Dolphins. I am not sure who is more excited about the season beginning, the parents or the players! We are looking forward to enjoying the next few months with this great group of people. Even Susannah is excited, as soccer practice had become the true highlight of her week last fall. Of course, the games are too crowded for her to attend, but the practices are so low key that she can safely don her mask and hat and have free run of the field -- yes, it makes for a lot of chasing on our part, but more importantly it makes her day!

Madeline and Klane, aka “Princess Tiger Lily” and “Brave Tiger Bone”, will be embarking on another Indian Princess campout this coming weekend. In search of snow, the two will head up to the mountains of Big Bear with their tribe for two fun-filled days of daddy-daughter bonding. Meanwhile, back here at home, Susannah and I will be anxiously awaiting the Saturday evening arrival of our Minnesota pal, Kelsey, who will once again be joining us for her Spring Break week. Madeline may be otherwise engaged on Kelsey’s first night in San Diego, but don’t be fooled, she already has a full blown agenda for her “big girl college friend” all mapped out! Needless to say, we’re looking forward to a great visit.

Thanks for continuing to keep tabs on our little lady. You’re unending support means the world to us.

Lots of love,

Amy, Klane, Madeline and Susannah

Friday, February 14th, 2003 ~ Valentine’s Day

Winter’s First Adventure


Happy Heart Day!

Well we may not have loads of snow or frigid temps, but February is definitely upon us. This fact was made poignantly clear this week as Susannah and I took our first trip of the season to our “winter home”. We returned today from a glorious three-night stay at the lovely Children’s Hospital, San Diego. The following is a recap of our itinerary.

Our girl woke herself up with an axillary temperature to 101.6 around 9:00pm on Tuesday night. Despite the added heat, she was full of energy and raring to go. Klane and I, on the other hand, were far less eager to set out on this impending adventure, well aware of the final destination. After telephoning the on-call doc, one new to the practice, he decided Susannah should first be seen in the Emergency Room, as opposed to the usual direct admit to the floor, in hopes that she may actually avoid an admission. While I knew this option was by no means part of “the plan”, I was too caught off guard to argue (and it was now 2:00am in Cincinnati). With Madeline fast asleep and Klane due in at the crack of dawn, Sis and I hopped in the car and headed for Children’s. And so the adventure began.

After spending five tedious hours in the ER (and that was with Klane calling ahead for speedy service), an elevated white blood cell count with an abundance of bands, landed us right where we should have gone all along -- the Hem/Onc Unit, for an all expenses paid stay in Room 111. Susannah remained bright eyed and bushy tailed throughout all of the admission rigmarole and only begrudgingly went to sleep at 5:00am! As for me, the five o’clock hour in California, meant it was eight o’clock in Cincinnati, now a more civilized time to ring up Dr. Davies and give her the low-down. Klane arrived shortly thereafter with a sleeping Madeline in one arm and all of her class Valentine goodies and her, all important, pink overalls and heart socks in the other (the kindergartners were celebrating on Wednesday, as they were off from school on Thursday and Friday).

An early morning call to the ever adaptable June, allowed me to grab Madeline a little breakfast from the hospital cafeteria and, despite the pouring rain and an empty gas tank, get her to school in the nick of time. A quick recap to her friend Lara’s mom, and arrangements were made for an extended play date at the Smith house, until either Daddy or Grandma Dona could fetch her. Although, I did have a chance to catch a shower before heading back to the hospital, I was on such an adrenaline rush, making all the necessary phone calls to facilitate our unexpected “vacation”, that I returned to the hospital, without remembering to gather our belongings. As for sleep? Well, that too would to have to wait. On the contrary, however, the little sister snoozed the morning away, and never even noticed my absence.

Susannah’s admission via the ER, or rather the weak attempt to stave it off, left us to arrive on the unit without our usual hospital “comfort” items. Still, we somehow managed to persevere in the tiny room, with borrowed toys from Child Life, the clothes on our backs and the lovely pediatric toothbrushes that the hospital provides. I learned to be quite creative with the treasures found deep in the pockets of the diaper bag. Actually, I owe my sanity to Susannah’s new “We Sign” video, an early Valentine’s Day gift from her pal Molly, which I tossed into the bag on a whim before leaving the house Tuesday night. Thank goodness for small favors (and thoughtful friends)!

In spite of it all, Susannah remained in wonderful spirits throughout our stay ... I wish the same could be said for mother. It took a while to get back in the groove of the impatient routine. With Klane working at the University and VA hospitals these days, Daddy sightings were few and far between. This was our first admission here in California where he wasn’t physically working at Children’s and I’ll be the first to admit that I had become a little spoiled. Of course, our Minnesota nurses are no longer traveling here, so they weren’t at the ready to get everyone in line and seeing as how it had been eight months since her last admission, the staff was a bit rusty on the nuances of our little lady. It was a vivid reminder that Susannah is the exception at this hospital and not the rule, something not everyone appreciates. As you’ve probably guessed, I wasn’t at all shy about speaking my mind, or in more politically correct terms, being an advocate for my daughter. I tell you though, “advocating” can sure be exhausting!

The good, and most important, news in all of this is that the fever proved to be benign, and the stay didn’t keep our girl down. In fact, did I mention that Sissy is MUCH more mobile than she was during her past admissions?!? She paid no heed whatsoever to the three foot cord of IV lines to which she was perpetually attached and simply went about her business, making for quite a bit of untangling and demonstrating just how strong that plastic tubing can be! Other than the elevated white count, Susannah’s labs remained relatively stable during this escapade. While the thought of a potential bacterial infection wasn’t exactly comforting, the fact that we weren’t dealing with the bottomed out counts and severe neutropenia that we were last winter certainly made things less stressful and our stay briefer. Now, with her blood cultures proving to be negative, we are hopeful that the rapid rise in her neutrophils was a sign of improved marrow function and are excited at the possibility that her new bone marrow could mount such a response to stress.

Although, Susannah’s fever subsided almost simultaneously to our arrival at the hospital and physically, she looked wonderful, as you know, there is no cancellation policy on these little trips. But three nights later, with her ANC back down, a clear chest x-ray and 48+ hours of negative cultures, Sis was granted an early check out time, provided we were willing to administer a few more doses of vanco and ceftaz here at home. An option we wholeheartedly embraced.

As such, Susannah and I arrived home Friday afternoon to a house festively decorated with pink and red hearts, courtesy of Miss Madeline. Once Klane joined us and received his special Valentine, father and daughter were off to spread more of Cupid’s cheer. Somehow, Klane had gotten roped into delivering cards, decorated by Madeline’s Daisy Troop, to the Veteran’s Hospital. When Madeline got wind that he’d forgotten them at home this morning, she insisted on accompanying him back to the VA to pass them out. So instead of Klane just dropping them off at the nursing stations, Madeline hand delivered the cards to all the vets herself. Albeit, coy at times, she truly enjoyed passing them out and giving the men her Valentine’s wishes and as you might imagine, they were most delighted to see her smiling face!

We hope you all enjoyed a lovely Valentine’s Day with your sweetheart(s) … we sure did!

Hugs and Kisses,

The Whites

Friday, January 31st, 2003

January in a Nutshell


Hi Everyone,

Our little lady has been keeping herself busy with her usual gamut of therapy sessions, preschool and music class, all within the confines of our home, of course. At last count she was up to a whopping 18 visits per week all told, not including nursing business. Without fail, however, Susannah rises to the challenge and enjoys every minute (well, okay, maybe not the range of motion exercises!). The highlight of her days is a mask clad walk to the neighborhood park. Susannah is really gaining endurance and actually does most of the walking on her own, only allowing us to use the stroller by force, or out of pure exhaustion! She longs to play with the other children on the playground and has no qualms about making this fact known, but in her own way seems to understand that her day WILL come and is contented with watching from afar.

Susannah’s latest pastime is jumping on the mini trampoline that Santa brought her. She just loves it and looks absolutely adorable bouncing up and down, especially when her hair is up in pig tails, as they “jump” right along with her! Albeit a little late for the holiday season, she has learned to express her gratitude for the man in the red suit, by whispering “Ho, Ho, Ho”, in response to inquiries about where she got her “jump toy”. It is very cute they way her little lips pucker when making this new sound.

While her speech is still clearly delayed, her communication is coming along beautifully. Susannah continues to use a combination of sign language and primitive words. Her favorite word these days is, without a doubt, Daddy, and every time she hears the front door open, she jumps up and chants “Da Da”; unfortunately, most of the time it is actually someone else. If she finds one of Klane’s shirts in the laundry basket, she picks it up and proudly announces who the owner is. At the hospital, whenever she sees someone in scrubs, she points to them and says, “Da Da”, which gives everyone quite a chuckle. I must say, I do long for the day when I once again hear the words “Ma Ma” -- at present Susannah does mouth them, but no sound comes out, and of course, there’s still the infamous “Ba Ba” -- but in the meantime I’ll take what I can get!

The big new activity around here these days involves her old pal, water. Susannah regularly goes to the cupboard and retrieves a big girl cup (paying no heed to the child locks, mind you) and promptly walks it over to the ice and water dispenser on the front of the refrigerator. Since she is not quite tall enough to actually push the button herself (phew!), she gently tosses her cup up onto the ledge and sets about to find someone to indulge her by filling it. She loves drinking out of an open cup and often will flat out refuse any sort of sippy contraption. She is as tidy as can be in this new endeavor, unless per chance, she has quenched her thirst and there still is water in the glass … ah, what better to do, than dump it out?!?

From a medical standpoint, Susannah is holding her own. Her labs are relatively steady in the “normal for post-transplant/ chronic GVHD range” and her skin seems to behaving itself in such a way that another move on her steroid taper may actually be on the horizon (in the meantime, however, she’s holding steady at 9mg). She does seem to be less interested in eating these days. While this may seem like normal toddler behavior, it comes just on the heels of a follow-up call from the GI doc in Minnesota recommending Susannah intake 100 additional calories each day. Nurse June, naturally, has long since calculated our gal’s k-cals down to the necessary amount per hour, so the increase itself will not be tricky, it’s just a matter of convincing Sis to ingest them. I suppose, if all else fails, there’s always chips and chocolate!

Susannah and I got the chance to spend some special one on one time together this weekend while Madeline and Klane set sail on a most exciting adventure to Catalina Island. They went on their first Indian Princess campout, only daddies and (over five year old) daughters allowed! They took the boat over on Friday afternoon and returned Sunday, in time for the Super Bowl (can you tell the fathers made the arrangements?). It was quite clear that they had the time of their lives when they walked into the house utterly exhausted, but giddy to recant the tales of their fun-filled weekend. There was kayaking, hiking, shell hunting, tide pooling, skits, a bonfire, bow and arrow shooting, and of course, the “Polar Bear Swim”, for which they both earned a coveted bear claw! The two are already looking forward to their next campout in March and the activity on next month’s agenda, a Daddy-Daughter Dance.

In closing tonight, I leave you with a special request. As you have so graciously done for our littlest princess these past few years, we ask that you say a special intention for her Uncle Steve, who was deployed overseas tonight to lead his battalion on a reconnaissance mission; the details of course, we are not privy to, but we know for certain it is not routine. As a career military officer, Lt. Colonel Ferrando undertakes this assignment with great pride, yet we, his family, enter into it with a lump in our throats. As much as we commend the bravery and acknowledge the expertise of our armed forces, it’s so very hard not to worry. Please pray for his safety and swift return, as well as for strength for my sister, Tami and her three boys.

Thank you,

Amy, Klane, Madeline and Susannah

Tuesday, January 7th, 2003

Move Over 2002 … 2003 Here We Come!


Happy New Year to Our Wonderful Friends and Family,

We hope this finds you and yours well and embracing all that 2003 has to offer. The girls and I rang in the New Year with a little get-a-way to Grammy and Poppy’s house, where we relaxed and, well, we pretty much just relaxed. A post-call Klane joined us on New Year’s Day, for, you guessed it, more relaxing and lots of football. Of course, for us the important game didn’t occur until the next day when USC socked it to Iowa in the Orange Bowl! Go Trojans!!! It was a big day in our house with everyone donning their cardinal and gold. Madeline, who was proudly sporting her new 'SC sweatshirt, even got poor June into the action by insisting she wear one of her red headbands, complete with big red bow. As you can imagine, it was quite the site and June, of course, was oh so excited … heck, the Brits don’t even believe in “that kind of football!”

YIPPEE!!! HOORAY!!! WOOHOO!!! The New Year also brought about Klane’s return to civilization; after three loooooong months, he is officially off of the Spine Service. This past weekend, with Madeline occupied at a slumber party and June at the helm at home, I whisked Klane off on an overnight escape for some much needed R&R, and especially SLEEP! Many, many thanks to our friends, the Spencers, who set us up with the most amazing royal treatment -- it was truly awesome! Now that he has had a chance to recuperate, the girls and I are abundantly excited about the prospect of reclaiming our daddy and husband! Well, okay, as much “reclaiming” as residency allows, but we can dream, can’t we?

On the Susannah front, thankfully things have been fairly quiet as of late, just the way we like ‘em!. We have seen some periodic dipping of her blood counts, but nothing terribly alarming and whatever the culprit, it seems to have remedied itself each time. As we enter viral season, Dr. Davies feels we will likely see a good bit of wavering in her lab values, but anticipates that Susannah’s responses this year will be far milder than last. What a blessing that would be!

Although a month later than “scheduled” (is there such a thing when it comes to our gal?), I am pleased to report that we have been able to knock another milligram off of Susannah’s steroid dose. Yes, the taper is proceeding, albeit at a snail’s pace. Susannah seems to be tolerating this most recent decrease fairly well, although it has only been a couple of weeks. She essentially needs to remain flare free for an entire month before making any additional moves, which with Susannah can be very tricky. In the interest of getting her dose down to limit the side effects of the prednisone, we have made the concession to tolerate itching, but rashes or lesions we will not. Time will tell, but we remain hopeful that we will be able to continue the taper close to the every other month schedule. Stay tuned …

In the meantime, Sissy continues to experience many of the adverse results of long-term steroid therapy. While we have grown quite fond of those trademark cheeks, it would sure be nice to see even a smidgen of vertical growth on the rest of her body. And, it’s a good thing that jacket weather is upon us (yes, we DO own those in California!), because it seems that the ongoing combo of CSA and prednisone has decided to add yet another layer of “fur” to, what we like to refer to as, her “winter coat”. The hair on her arms and back has literally begun to curl. While the steroids help her skin so much in one regard, they also make it very thin. Recently, the sutures anchoring in her PICC line have not been holding and have been pulling though the skin at less than weekly intervals. Previously these stitches were lasting two months or more at a stretch. Not only do we have to pin Sis down and sew her up (not fun for any of us, the least of which her), for obvious reasons, it’s been trickier than usual for Klane to be available for these little procedures. Fortunately, however, Daddy has somehow managed to come to the rescue, so Susannah and I have been able to steer clear of the ER. We are hopeful that once viral season is behind us, we may actually be able to pull the line and attempt life without permanent access. Wouldn’t that be a treat? If you recall, this line was actually only intended as a temporary fix “for a few weeks or so” and it has been, well, I better not elaborate for fear of jinxing it. At any rate, let’s just say we are probably setting a longevity record for PICC placement!

Otherwise, Susannah is deep into her dollies and their paraphernalia. She loves to feed them, rock them and of course, push them. The various strollers around the house, and oh there are multiples, are by far the most utilized toys these days. Her latest treasure is the pram she discovered in Big Sister’s bedroom. Daily, she sneaks into Madeline’s room and maneuvers the bulky thing out the door and down the hall, all the while looking like the cat that ate the canary!

She is keeping busy getting into lots of mischief and all around turning into a real little busy body! Susannah is becoming aware of new things everyday and seems much more in tune with her environment, both indoors and out. She is also learning all kinds of tricks from her big sister and is now able to scale the upright ladder to her play structure and come down the wavy slide, all by herself. As exciting as this is, it is actually sometimes a little scary, for if we leave her alone for even a minute, she is up on the platform, dangling over the railing. Just to add a little more fuel to the fire, Miss Madeline taught her to go down the slide head first, on both her stomach and her back! After all, what’s a big sister for?!? Needless to say, we are now working on positive reinforcement for sliding in the correct position! Besides herself, Susannah is very fond of sending anything and everything she can get her hands on for a ride down the slide, as well! Yes, I know, I know, we really need to work on our playground etiquette!

The little miss has also mastered the art of child cabinet locks and can now be found emptying any and every cupboard or drawer at her level, then lining the contents up in a row. She loves to “escape” to her bedroom and close the door behind her. If not followed, she can wreak havoc in a matter of minutes! Her neatly folded clothes are now found out of her drawers and scattered all about her room on a regular basis. Her favorite pastimes of late include turning her sat monitor on and off, unplugging her nightlight and throwing everything out of her bed. Ahh, she’s coming of age.

Susannah’s speech therapist for the school district has recommended her for Music Therapy services. As such, the music therapist will be working on a consultative basis with the speech therapist and preschool teacher, here at home, to introduce more music into her curriculum, as a means of enhancing verbal communication through song. As most of you know Susannah LOVES music, so this is going to be right up her alley.

And who could forget, the best big sister in the world? The soccer season ended with a bang. While we won’t scrutinize the Red Dragons actual record, their hearts were in it and that’s what counts! Madeline’s most prized possession is now her very own soccer trophy. In addition to being showcased in her room and brought out for any and all visitors, the trophy even managed to get an invitation to Kindergarten Show and Tell. She was also presented with the “Most Determined Girl” award for her unending drive to “never give up”. We met a lot of wonderful families and are looking forward to spring soccer with the very same team to play one more season before the ages of the children abolish the co-ed teams. She continues her ballet/ tap class once each week and participates in Daisy Girl Scouts every other Wednesday. With soccer on hiatus for a stint, we are looking into an intersession gymnastics or music class to bridge the gap, but who knows maybe we’ll just take a break. Stay tuned for more details on our little butterfly’s social calendar.

Speaking of socializing, as you all know, Susannah doesn’t get out much. In fact, I cannot recall how long it has been since she has been to a restaurant. Certainly long enough for her to have absolutely no recollection of it, probably somewhere on the order of 2½ years or so. Well, we finally did something about that! Call us resourceful, some would say nutty, but we found our own way around all the constraints of her compromised immune system. With Klane on-call and loaded down with cafeteria passes, we girls met him at the Thornton Cafeteria! Okay now, stop your smirking, Thornton just happens to be UCSD’s private hospital, fondly deemed the “Ritz Carlton”. As the nickname denotes, its lobby very much resembles a luxury hotel. Now don’t get me wrong their cafeteria is still a cafeteria, but it was deserted and equipped with a private dining room adjacent to it, complete with mahogany chairs and crown molding. Bet’cha never encountered a cafeteria like that! At any rate, we scoured the high chair with disinfectant wipes, put a plastic placemat on the table and voilà, Susannah had her very own restaurant! She was in complete awe and Madeline was beside herself with excitement at the idea of us all going “out to lunch” together. Now, as you might expect, the fare did not quite equate to the décor, but hey, at least we were out!

Hmmm, that about sums things up here in sunny San Diego. It is, of course, a balmy 75 degrees today -- just a little hint to all of our mid-west and east coast friends who we seem to be missing more than ever these days! We are looking forward to 2003 being a year of HEALTH and happiness for our family and certainly wish you all the very same. Susannah’s steadfast progress is due, in no small part, to the fact that she still has so very many dedicated people praying for her. We are forever grateful for your perpetual support, love and loyalty.

All our love,

Amy, Klane, Madeline and Susannah

PS … In light of being a little lonesome for our distant pals, I’ve updated the photos (yes, twice in one month!) to include some great shots taken during our last trip to Minnesota. Hope you enjoy them as much as we have!

Sunday, December 22nd, 2002

Tis the season …


Merry Christmas from Our House to Yours!

The tree is trimmed, the cards are out, the presents wrapped (well, almost!), the house is decorated, and boy, are we in the holiday spirit! Madeline has been going full steam ahead since December 1st when she made her very own Advent wreath. We’ve been hearing Christmas carols non-stop and in the mind of a five year old, the 25th of December can’t come soon enough! Miss Susannah is busily trying to figure out just exactly what all the hoopla is about, but one thing is certain, she very much understands the importance of her CHOCOLATE Advent calendar!

Today, we began our Christmas celebration a few days early with a very special visit from the “Washington Whites”. Uncle Randy, Aunt Susie and crew arrived in force for a wonderfully festive afternoon. To make this special gathering even more exciting, we were joined by both Auntie Brenda’s and Uncle Joel’s families, and of course, Grandma Dona. By some small feat, everyone was healthy! With nine cousins in all, Madeline was in her glory; there were art projects, nail polishing parties, costume parades, and, perhaps the biggest hit of all, dance performances! Susannah was doted on every minute and was in complete awe of the plethora of children, particularly her teenage cousin, Jonathan. A great time was had by all, we only wish they lived closer.

Arrangements have been made for Susannah to once again accompany us to Christmas Eve Mass for the annual St. David’s Children’s Pageant. It will be a nice family outing, with the added bonus of watching Big Sister perform. Like last year, we will need to be safely tucked away in the privacy of the "crying room", but we will be there nonetheless, and together! After lamenting to anyone who would listen about the fact that she has been “cooped up as an angel” for the past three pageants (including the one in Minnesota), Madeline will be making her debut this year as a sheep. Of course the real reason behind her whining, was the hope of playing the Virgin Mary (rumor has it there will be a real live baby Jesus to hold -- this will be the first year since Susannah had the honor), but alas we explained to her that the role of the blessed Mother was reserved for older girls, of at least ten! Her pouting about her only “speaking” line being “Baa” was short lived when, in the mail yesterday arrived a genuine letter from Santa Claus! While the letter she and Susannah sent him was merely addressed “Santa at the North Pole”, apparently the guy in the red suit paid attention to the return address and actually replied! As you might imagine, this absolutely made her week!

Following the pageant, we plan to celebrate our traditional Christmas Eve at home and are grateful for another opportunity to share the night with, both Grammy and Poppy, as well as, Grandma Dona. Since we’ve had two VERY good girls this year, we anticipate an early morning awakening in search of the treasures Santa leaves behind. Aunt Tami has been kind enough to “Susannah-tize” (translation: fake tree) her house again this year, so we will then venture up their way for dinner on Christmas day with the Ferrando “boy cousins”. Otherwise our plans and the timing of the holiday will remain relatively flexible, as Klane has somehow managed to be find himself on-call for all of the November, December and January holidays. While this will surely make this Christmas unique, barring any emergencies, a little creative scheduling should allow him to spend the much of the day with us.

There is something about this time of year that causes us to reflect. We cannot help but be reminded of just how far Susannah has come, how much she has endured and the opportunities that lie ahead for her. She has had an incredible journey in her short life. Tomorrow marks the third anniversary of her diagnosis of Hurler Syndrome, a day that will forever be engrained in our memory. As if the prior year didn’t impose enough change in our lives, during Christmas 2000 (nearly 100 days after her second transplant) we began the roller coaster ride that we are still on today. At that time, never did we believe we would be “coasting” along as we are now, at much slower speeds with more benign turns. And finally, who could forget last year when we were so very fortunate to celebrate Susannah’s homecoming to San Diego, after 18 tumultuous months away. As we bask in the joy of all she’s overcome, however, we are painfully aware of our many friends who have not been so blessed and are enduring the holidays, and everyday, with aching souls and empty arms. We hold them close to our hearts now, as always.

In this season of miracles, we especially thank God for the miracle we have been given in our sweet Susannah. Never a day passes that we are not reminded of how lucky we are to have her in our lives. She is truly a precious gift who makes every day richer with her warm smile and contagious laughter.

May your Christmas be merry and bright, and may the gifts of love joy and family fill your heart and home this season.

With much love,

Amy, Klane, Madeline and Susannah

Saturday, November 30th, 2002

Wishes for a Happy Thanksgiving


Dear Ones,

We hope you and yours had a wonderful Thanksgiving! Due to the combination of Susannah’s still compromised immune system and her father’s compromised availability (in other words, Klane was on call), our little family shared a very quiet holiday together, just the four of us. Thankfully, the pager was relatively quiet and Klane did indeed get to spend most of the day at home.

As you all are aware, our blessings this past year have been plentiful, our thanksgivings many and the promises for the future continue to be bright. However, I must say having a kindergartner in the house has really reiterated the history and traditions behind this particular holiday. We have made Pilgrim masks, Indian headbands and incorporated turkeys into every educational process you can imagine; they have been colored, cut, used as sentence builders, art projects and we have all mastered the art of tracing our hands and “converting” them into this festive feathered friend. And oh, less I forget the cornucopia. Madeline was so enchanted by this symbol and she so vibrantly shared her joy with us (as well as, everyone in the grocery store), that I too will share it with you. Fear not, there is even a recipe: “Bugles” for the baskets, “Ritz Crackers” for the base, “Trix” for the fruit spilling out of the baskets and to top it off, this delectable combination is all held together with, none other than, peanut butter! These innovative “cornucopias” were such a hit at the “Kinder Feast” that she just had to make them again at home!

Speaking of feasting, a very giddy Madeline insisted on assisting in the preparation and planning of our own family’s feast. Literally, it was a feast, in the true sense of the word. In fact, we shall be eating it for the next week! I do believe the two of us prepared enough food for the entire neighborhood and NO, even Klane could not make a dent in it! At Madeline’s suggestion, we set our table up in the living room and dined in front of the fireplace. She was truly in her glory as she, herself, lit the “turkey taper candles” and sipped special holiday “champagne” aka sparkling cider.

Susannah for her part, decided that turkey gravy is delicious on absolutely anything … potatoes, turkey, cranberry sauce and if there is nothing else available, it tastes just as good licked directly off of one’s own fingers! The big news of the day was that Susannah actually sat at the table with us. On a whim we decided to ditch her highchair in exchange for a booster and she did beautifully -- such a little lady. She did find great delight in discovering the sounds she could produce by clanging her fork on the china!

Everyday is a day to be grateful, yet this Thanksgiving we are especially thankful for the progress Susannah continues to make and for the love, prayers and support our family continues to receive from all of you.

With thanks,

Amy, Klane, Madeline and Susannah

Monday, November 11th, 2002 ~ Veteran’s Day

Happy “Boo-lated” Halloween!!!


Hi There,

BOO! I realize it’s now Veteran’s Day and half way to Thanksgiving, but our Halloween this year was such fun that it bears sharing. And, besides, there are even PHOTOS!!!

Other than her stint as a bumble bee when she was six months old, this was Miss Susannah’s first real Halloween experience outside of the hospital. And she loved it! Her affection for doggies fit in perfectly with the costume theme Big Sister had concocted for this year (you do realize that she plans these things a year in advance?!?). Thanks to Grammy’s handiwork with a needle and thread, Susannah looked cute as a button in her “Genevieve” costume, while Madeline followed close at hand as the French storybook character, “Madeline”, complete with the classic blue cloak and yellow hat. Mommy even got into the action and donned a gray habit to chaperone as "Miss Clavel".

During the day, while “Madeline” was off at school marching in the annual Holmes Halloween Parade and enjoying a sugar fest (aka her classroom party), “Genevieve” and I spent the day at the hospital for her bi-weekly clinic appointment. Other than the remnants of her recent rash, Susannah received a “clean” bill of health and spent the remainder of her visit being ooh’d and ahh’d over by the nurses and her therapists, who dropped by our room to see her in costume. She left the hospital that day with more treats than I could carry!

Once dark, we bent the rules a bit and took Susannah out trick-or-treating to a few houses in the neighborhood. We did, however, keep her at arms length from others and she wore her coordinating brown mask, a la Grammy, the entire time. We brought the red wagon along, thinking Susannah may get tired during the outing. Boy, were we wrong! In the beginning she was excited to ride in it, but after the first house, she ditched the wagon and demanded (and DO I mean demanded) to walk; she wanted to be like the big kids. Ever the social butterfly, she’d scuttle right up to the doors and try to ENTER every single house! Despite being covered in brown fur from head to toe, as we went from house to house, Susannah was known to all from her daily walks around the neighborhood and was more than happy to oblige everyone with her signature bark.

It took her a while to catch on, but thanks to Madeline, Sissy quickly learned the ropes of taking the candy and tossing it into her pumpkin. In fact, at the last house, she kept signing “more” followed by a pathetic sounding little “peassse”, until she had completely wiped out our neighbor’s supply! It was irresistible. Thankfully we don’t have a very large turnout here on Mount Tami Drive. Both girls had a blast (as did Mommy and Daddy, watching in the wings).

In Susannah’s humble opinion, the best part about the whole evening was, unequivocally, the CHOCOLATE! Until recently, the little miss would have nothing to do with sweets, but now that she’s been introduced to chocolate, she sure has changed her tune! I am beginning to think we should start using candy as part of her speech therapy because on Halloween night, we managed to elicit full sentences (word and sign combos) begging for more chocolate!

Happy Halloween one and all ... better late than never!

Love,

The Whites

Wednesday, November 6th, 2002

A True Hero


Hello All,

It is with an aching heart, through a veil of tears that I write about our dear friend, Christina, who passed away early this morning. One might think that having composed similar words so often over the last few years would make this a little easier, but in actuality, it becomes increasingly difficult each time. Perhaps even more so in this instance, as we are now home ... here in San Diego things are supposed to be safe, a haven away from the hospital and tragedy we so often experienced during our stint in the mid-west. THIS just isn’t supposed to happen here. Or so I allowed myself to think.

Today, Christina Leighton lost her valiant battle with Hurler-Scheie Syndrome. It was sudden, unexpected and just on the heels of her 14th birthday. She was a trailblazer, a hero, in the true sense of the word. Christina paved the way for children around the world and advanced MPS research by participating in the first ever drug trial on humans whereby a synthetic enzyme was infused to compensate for the one lacked. Additionally, because of Christina, the anesthesiologists and surgeons here at Children’s Hospital San Diego are no longer strangers to the anomaly of Hurler airways and anatomy of these special children. Her sacrifices and struggles have made the local doctors and nurses better prepared to care for Susannah and the others who will undoubtedly come their way. On top of it all, Christina managed to endear herself to the souls of everyone she encountered.

The Leighton Family has been an incredible inspiration to us. We were introduced to them two short days after Susannah’s diagnosis and became fast friends. They are an amazing family, whose strength and confidence are utterly contagious. I so wish each and every one of you could have had the opportunity to meet their sweet Christina, as she would have made your heart sing.

Christina entered our life and gave us hope at a time when our world seemed dark. She played, she laughed, she was a kid! It was remarkably uplifting. Yes, things were more difficult for her than most -- she was no stranger to hospitals and doctors; she had physical limitations; she was a child who lost many friends in her lifetime, not to the usual childhood squabbles, but to the very disease she shared with them. It didn’t phase her. She was a young lady who, despite all of the challenges she faced, truly loved life.

One of my fondest memories of Christina was the look of sheer joy on her face as she demonstrated the art of the “Chicken Limbo” to a, then, two year old Madeline and an infant Susannah. From the smile in her eyes, an outsider would have never guessed the strain on her body to perform such a task. She fought daily with this ravenous disease we have all now come to know so well, but her positive attitude, sparkling personality and hysterical sense of humor always shined through.

Just last month, Christina sat in our living room and brightened our day with her infectious laughter. She longed for the day when Susannah would be healthy enough to accompany her to Sea World. In fact, during our visit, she could talk of little else. We never made it. Standing at her bedside earlier this week, I squeezed her hand and made a silent promise. She spoke no words, but I can assure you of one thing, with Christina cheering us on, Susannah WILL make that trip one day, in her honor, and our special guardian angel will be right there with us in spirit (probably making certain those dolphins get us really wet!).

We will be forever grateful for the privilege of having known Christina. Heaven is now blessed with another perfect angel. Our love and prayers surround her wonderful parents Julie and Ken, as well as, her older sister Megan; may they find peace and solace during the difficult days ahead. We appreciate you remembering them as well.

All our love,

Amy, Klane and Girls

Sunday, October 27th, 2002

Minnesota on My Mind


Dear One and All,

We are once again home, safe and sound after a wonderful trip back to Minneapolis. It was a whirlwind week, which was at the same time, relaxing and exhausting, informative and exciting. I have a ton to report, so sit back and relax (you may even want to put up your feet!), this is going to be a doozie.

True to form, our favorite mid-western city welcomed us with a dramatic change of season. We left the warmth of San Diego, where sundresses are still appropriate in October and were greeted by the vibrant colors and crispness of autumn. Honorary Minnesotans that we are, we simply broke out our coats and enjoyed. The girls were so excited about collecting the gorgeous leaves and we all savored the familiar crunch beneath our feet as we walked around our old stomping grounds. Well, two short days after we entered fall, we ran smack into winter! Yep, that’s right, it actually SNOWED during our visit and twice at that!

As always, Miss Susannah received a full and comprehensive evaluation from the folks at the University of Minnesota. On the agenda were eyes, ears, bones, belly, lungs, heart, kidneys, skin, brain … you name it, it was checked, rechecked scrutinized and discussed. And, for the most part things are coming along very well. Overall, the docs could not have been more delighted with her progress in every aspect of her being.

First things first, let me get to the good stuff. Susannah’s heart not only continues to be in great shape, it has actually improved. As far as the tickers go in Hurler children, Dr. Braunlin reports that from all accounts Susannah has one of the strongest she’s ever seen. Her EKG and echocardiogram were essentially normal. Amazingly, the minor leakage she has had in her valves in the past is miraculously gone! This is supposed to be one of the areas the transplant does not remedy, but as we well know, stranger things have happened and we are certainly not complaining.

On to the ears. The news here gets better and better! Our visit to the ENT clinic, proved completely non-eventful and other than removing a hunk of wax the size of a hazelnut from Susannah’s little ear, Dr. Rimmel had nothing “medical” to contribute. Of course, we had our usual chit chatting session and, as he has done so many times before, he reminded us that he never thought he would see the day that Susannah would be thriving and kicking, and off the vent!

The trauma of the wax removal earlier in the day, proved to be beneficial as we moved onto our Audiology appointment. With clear ears, and the help of Jim our former speech therapist as a distraction, Susannah’s hearing test came back NORMAL! Yes that’s right, our little lady even heard things in that booth that neither Jim nor I did! As you may recall, Susannah did not suffer from the classic Hurler conductive hearing loss, her loss was actually sensorineural in nature and thus, not typically helped by the transplant process, not to mention the numerous ototoxic antibiotics she’s received over the years. The audiologist was shocked and had no explanation, but assured us that the test had been double and triple checked. Our gal continues to defy the odds, in so many ways. And so it seems, that hearing aides are off the agenda for now, something that I assure you Susannah will not be complaining about, nor will those of us who have struggled all this time to get them in those little ears (and keep them there!).

We also visited with the neurologist and neuropsychologists. With Dr. Charnas out of town, we got to see our old friend Dr. Lockman, who was pleased as punch with Susannah’s progress and was gloating over her to his team of residents and medical students (some things just never change, as usual, he was accompanied by his entourage). It seems the neurologists think of Sis as their poster child, you know, the one they never expected to do this or that. Unfortunately, their all too common role as prognosticators when children are at death’s door, doesn’t usually provide this kind of ending. Thus they get very excited to see the likes of Susannah.

From Neurology, it was off to the lovely three hour Neuropsychology evaluation. As we anticipated, Susannah’s developmental assessment showed tremendous improvement all around. In the last year, she has gained tons of milestones and many months in development. Since she “awoke” in May of 2001, 17 months ago, our girl has gained more than 23 months in some areas. Dr. Shapiro was grinning from ear to ear (yes, this actually IS possible) at all of her achievements and even let Susannah sit on her lap and “share” her pen. Ever the performer, Miss Susannah then wowed her with fancy down lines and beginning circles.

Of course, she also was evaluated by Dr. Ogilvie, the orthopaedic surgeon, as well as, Dr. Van Heest, the hand surgeon. As we pretty much knew from our interim visits with Dr. Wenger here in California, things with Susannah’s skeletal system remain status quo. At this point, her kyphosis is essentially stable and we can safely stave off any spine surgery until the curve exceeds 70 degrees. As you already are aware, her hips are typical of her diagnosis and exhibit significant dysplasia, but it is generally not until four to five years of age that the hip elements are large enough to tolerate the necessary hip reconstruction. Now that she is up and walking, Susannah’s knees are beginning to show very early signs of the classic knock knee deformity, but are no where near the surgical benchmark of 15 degrees; not to mention that her knees are so tiny that at this point it would be impossible actually perform stapling. As a side note here, we also hooked up with our buddy Todd, the orthotist, who added more arch supports to Susannah’s shoes to help with any pronation that may contribute to the knee issues.

As for her hands, this was a more controversial area of the week. On physical exam, and based on her age, Dr. Van Heest was concerned that we were approaching the time to look at performing carpal tunnel releases on Susannah. With her EMG from last year showing mild nerve conduction loss, she ordered another EMG to reevaluate the situation. Of course, this could not be scheduled until Friday and we saw Dr. Van Heest on Monday, so we were on pins and needles all week, as to whether Susannah would require surgery in the next couple of months. With her compromised immune system, a la the steroids, operative procedures are something we would like to put off as long as possible. However, the implications of waiting in this case could result in a loss of use of her hands, so we wouldn’t have much choice but to proceed with surgery if indicated. Aside from being totally annoyed with the electrical stimulus of the test, Susannah managed to defy the odds for the third time in one week by showing improvement in her nerve conduction velocity testing and the EMG. Once again, this is not something that the transplant generally rectifies. Of course, this is by no means a permanent improvement, but it at least allows us to avoid the operating room for now. To help alleviate any discomfort in the wrist, as well as, to try to stretch Susannah’s fingers, Dr. Van Heest did have us see the hand therapist to remake some new splints in order to allow for better digit extension while she sleeps. Don’t fret though, the therapist was kind enough to leave her little thumbs free for sucking and the splints are even pink!

From a pulmonary perspective, for better or worse, things continue to remain on an even keel. Our flight a few days earlier, was proof positive that Susannah’s lungs still need to heal. Once again, as the altitude increased, her oxygen saturations did a nose-dive. Fortunately, this time the oxygen was ready and waiting. Dr. Milla reiterated that growth would be her best medicine; during our spring visit, he plans to obtain a follow-up x-rays to monitor the size of her chest cavity. In the meantime, with winter (and more importantly viral season) upon us, he suggested keeping the inhaled steroids, as well as the oxygen, close at hand.

We also swung by the Dermatology clinic where Dr. Hordinsky offered two new creams to try on Susannah’s hands, which seem to be the first place to show signs of her chronic GVHD flares. While her head and hair do look better overall, the persistent scales are still not under control (it’s been almost a year now). Although a long shot, some skin samples from her scalp were obtained and sent off for analysis to see if the lab could shed any light on the situation. For now, we’ll continue with the regimen of medicated shampoos and her fancy steroid mousse.

Our appointment with the eye guys was two fold, two days long and two torture sessions for poor Susannah. On the retina front, Dr. Olsen found her eyes to be free of any active CMV lesions, which has thankfully been the case since her gancyclovir injection two springs ago. Still, as long as she continues to be immunosuppressed, she will remain on her val-gancyclovir to prevent any re-activation and as we expected, the poor baby will stay on the every four month retina exam schedule.

We also saw Dr. Christensen, who reported that per testing, Susannah’s vision has markedly improved since May. Since children do not reach an adult level of vision until about age seven, the rating for system for kids does not equate to the familiar 20/20 rating, so do not be alarmed by the following numbers; they are actually quite good. In May Susannah tested at 20/130, which was within normal limits for her age, and this week she jumped to 20/63, which obviously is a dramatic improvement. So still, she has no need for glasses. They did also perform a refractory exam while her eyes were dilated for the retina doc, and she exhibited a slight nearsightedness, but again this was within normal limits. As for her corneas, they continue to remain in great shape, with her post transplant improvement in the clouding holding steady. Unfortunately, Dr. Christensen did notice the formation of some early cataracts on her lenses. While this is disappointing, it is not yet affecting her vision, nor is it a real surprise. You see the radiation Susannah received as prep for her second transplant was fractionated, which does reduce the risk of this typical side effect, however, her long history on steroids certainly adds fuel to this fire. All involved agree that the steroids are likely the culprit, and since her body seems intent on keeping them on board for a while, the cataracts very well may progress to the point of requiring intervention. Which means, you guessed it, surgery; in children the common laser approach is not possible and she will actually have to go under the knife to remedy the problem. This being said, we all know Sissy does things her own way, so perhaps, she will wind up surprising us all on this one too, although it is not probable.

With her ongoing regimen of val-gancyclovir for the CMV retinitis, as well as her prior bacteremia issues, we had a quick visit to the Infectious Disease clinic. Dr. Van Burik marveled at the changes in Susannah since she’d first come to know her unconscious, infected and sedated up on 4-A. We went over her many meds (14 in all, not including the numerous creams or “prn” medications) and discussed the importance of a flu shot for Susannah and the rest of us coming into close contact with her. With her continued immunosuppression, we will obviously be holding off on revaccinating her for a while. Yet another reason why she needs to continue to remain isolated from others.

Susannah’s thyroid studies (blood work) done this go-around, showed the need to increase her synthroid back to every day dosing. Growth and brain development partially depend on thyroid support, so these are two areas in which we really don’t want to sell Susannah short. During our next trip, we will also meet with the endocrinologist in Minnesota to assess Susannah’s growth more in depth. The BMT docs want to be certain that the reason behind her lack of vertical growth is not due to a deficiency in growth hormone subsequent to her exposure to radiation. As we well know, Hurler Syndrome can also be a contributing factor, but not usually to this degree at such a young age. For the moment, all fingers point to the good ole steroids, but it makes sense to give the Endocrinology folks a chance to throw in their two cents.

Our appointment with the GI team was relatively uneventful, but left us feeling reassured. Dr. Schwartzenberg felt Susannah’s current weight was actually right in line with her height and opted against delving deeper into the malabsorbtion issue until her steroid dose was down and the dust had settled. She thinks it is a distinct possibility that the prednisone may be a contributing factor in her lack of weight gain and mediocre nutrition labs, despite her fabulous eating habits of late. However, she does want GI put on the agenda early on during our spring visit, so she can do a more in depth evaluation.

Susannah also had another ultrasound of her kidneys which showed that while they were no longer shrinking, they certainly were not growing. Her overall kidney function is quite good considering the level of insult they have withstood, but there is some concern over their lack of growth. Now, the question on everyone’s mind is that since Susannah is not exactly growing herself, could this be a plausible explanation for her stunted kidneys? This will be followed up on with the renal doctors during our next check up in Minnesota, unless anything further arises in the meantime.

We wrapped up our appointments at a visit with Sat and Dr. Ramsay on Friday afternoon. While both had seen Susannah several times throughout the week and reported back to Dr. Davies, with all of her results in hand they were now able to officially give her their blessing. They, like everyone else were very happy with Susannah’s progress. Her counts look great, with a near normal hemoglobin of 12.0 and a whopping platelet count of 242,000! Now, for the numbers you all have been waiting for: Susannah’s engraftment continues to hold steady at 100% donor with a super duper enzyme activity level of 50.7.

Of course, we also addressed the age old issue of chronic graft versus host disease with these two. As you may have noticed, one thing is becoming more and more clear. Any and all pitfalls in Susannah’s current health status can basically be attributed to one thing, the ever-present steroids. In other words, while prednisone is her best friend in controlling her auto-immune dysregulation (aka chronic GVHD), it is quickly becoming her worst enemy from an overall medical perspective. So, despite, a few splotchy patches on Susannah’s skin, which seem to be responding quite well to the topical creams, we all agreed that the change was not severe enough to warrant an increase in her oral steroid dose. After consulting with Dr. Davies, Dr. Ramsay and Sat recommended holding tight for now and attempting to continue a loose wean plan of one milligram every other month, assuming of course, Susannah’s body can tolerate it. While this may seem ultra slow, with small kiddos even the slightest budge in the wrong direction, things can really get out of hand. Thus, it will be a very painstaking and precise process and puts the light at the end of this isolation tunnel at least another year away. On the positive side, at least we again have an end in sight!

While our days were jam packed with appointments, we managed to get lots of visiting done. With the entire city sold out of hotel rooms due to a convention, and the Ronald McDonald house packed to the gills, the kind folks at Dinnaken Properties (our former landlords), were able to sneak us into an empty apartment in one of their buildings. It was so great, with lots of room to roam and plenty of space for entertaining! Speaking of which, our apartment was like a revolving door, but it sure was great to get the chance to visit and catch up with all of our pals. We really appreciate everyone working their schedules around ours. Hopefully, as the years go by and Susannah’s health progresses, these trips will become a little less hectic with appointments, leaving MUCH more time for socializing!

We were also treated to a visit from our Care Partner, Sue, who managed to sneak out of the north woods of Wisconsin to spend the afternoon with us. It was really great to have this time together, since we were unable to connect during our last trip. In true Sue fashion, she came bearing all kinds of goodies for the girls. Special thanks to her “groom” for sharing her with us (not to mention driving her down)! On that note, a huge thank you to Kelsey and Gayle, as well as, the Moore family for outfitting our apartment with the necessities and stocking our fridge. Also, to our ever-reliable airport chauffeurs, Karen and Joan -- you’re the best.

It took several trips to the unit (which looks fabulous, by the way!), but we actually managed to catch up with ALL of our favorite nurses from inpatient life who are still at the hospital. It was great to hear the news about everyone’s engagements, marriages, families, et cetera. Many of these people really became a part of our life during those long, long months up on 4-A. Not surprisingly, Susannah felt right at home and had no qualms about running the halls and knocking on the doors. She was particularly taken by the new animal murals and delighted all with her latest bunny imitation!

As you probably already guessed, Miss Madeline was completely indulged by all and had an absolute blast. While we were off at clinic, she had all kinds of fun with Kelsey and Gayle, collecting leaves, doing crafts, playing “make believe” and even going to the movies. She was also invited for a sleepover at the Moore’s house and as always, she and Jessica (who is doing great!) hit it off and rekindled their friendship in nothing flat. Of course, Madeline had special visits from all of her old babysitters and wowed them with her oh so grown up kindergartener persona. Later in the week, Joan returned from a trip and drove straight from the airport to pick up her girl. Naturally, the two were inseparable for the remainder of the trip!

All in all it was a wonderful week. Even amid all of the poking and prodding, Miss Susannah managed to have a great time and really show her stuff. Just like on her last trip “home” she really blossomed during our visit. Appropriately, she even mastered a new phrase this week. With a warm smile she would happily sign “hat”, followed by “mask”, then “stroller” and then finally “all done”. Then, just to drive her point home, she would sum things up with very boisterous “bye bye”. Can you blame her?!?

Thanks for checking in and for all the prayers and good wishes you sent our way during this important week of testing. We love you.

Blessings,

Amy, Klane, Madeline and Susannah

Wednesday, October 9th, 2002 ~ Two Years Post Transplant

Happy Transplant Anniversary to You ...


Good Morning!

Perhaps it’s about time we stop counting this journey in days … today, Susannah is two years post bone marrow transplant, transplant number two that is! What a road our girl has traveled, although you’d never know it by her vibrant attitude and zest for life. In some ways October 9th, 2000 feels like a lifetime ago, yet at other times it seems like just yesterday. While we never thought we’d still be juggling transplant complications this far out, we are just grateful to have her smiling face amongst us. She is a miracle child.

We are scheduled to be in Minneapolis the morning of October 14th to begin Susannah’s anniversary visit for her two-year studies. She has a full week of appointments on tap, spanning nearly all specialties, and then some. Again, we will refrain from any of the usual imaging studies at this time, as we continue to shelter her little lungs from any non-essential anesthesia. Of course, we will be extending our stay through the weekend to be certain there is plenty of time for visiting. We are looking forward to catching up with our “mid-western family” and touring the newly renovated Pediatric BMT Unit.

There is however, one individual who will be noticeably absent from our visit this time around. Over the last couple of months, Dr. Davies has been transitioning to her new position as head of Peds Bone Marrow Transplant at Cincinnati Children’s Hospital Medical Center. As such, she will be sorely missed this trip. In her absence, we will of course be in wonderful hands as two new, yet very familiar faces are reincorporated into Susannah’s care. Our favorite former fellow “Sat” (oops, Dr. Satkiran Grewal), now an attending specializing in metabolic disorders will head up her Hurler related follow-up, while Dr. Norma Ramsay will oversee her needs in regard to the chronic graft versus host disease. Both of these physicians are more than familiar with Susannah, not to mention her high maintenance parents, and we trust them implicitly. Of course, Dr. Davies will remain in very close communication with everyone involved and will always be a mere phone call away. As she so poignantly has informed us “You’ll never get rid of me!” Nor would we ever want to!!!

On the medical front, with Susannah’s skin looking better than ever, we took the brave step of attempting a steroid taper. Okay, so it was a baby step, as we dropped her dose a whopping milligram from 11mg to 10mg every other day, but hey, it’s a start and we have to start somewhere! Thus far, she is tolerating if fairly well, but we have had to step up her topical steroids somewhat. While it is much easier to give the medication orally, than to add to our creaming routine, the topical route is much safer for Susannah. Should she continue to tolerate things at this dose, without evidence of a flare, we may just re-attempt our monthly wean schedule we were to begin nearly a year ago! By the time we get to Minnesota, she will be into her fourth week on the new dose, so we will have a clearer picture of the trend and will make the decision to proceed or hold steady at that time.

In other news, kindergarten is going really well and Madeline is picking things up like a sponge. While there is lots of learning going on, I have to tell you the real hit of school is the monkey bars! Even after splitting her lip on the way down, Madeline was right back on the very next day. She is addicted and let me tell you you’d have thought there had been a trauma last week when she had to skip a few days due to blisters! Luckily, Daddy managed to tend to them with his little bag of tricks and she was again back in business a few days later.

Madeline continues to enjoy soccer. Although this is her first year, she has really taken to it, getting right in the thick of things, making her presence known. Her infamous red bow has become somewhat of a trademark for her team. You see, she plays in a co-ed league, and there have been numerous times when she was the only girl on the field and the opposing coach directed his boys to kick it to "the girl", only to realize too late the mistake of his misjudgment. For all her feminine qualities, Miss Madeline is quite the go-getter on the soccer field. We are ever so grateful to the other parents on the team, virtual strangers, who have pitched in and made this work for our daughter, no small feat with Klane's schedule and Sis's crowd restrictions. They have truly been wonderful.

Our trip to Minnesota will be a family affair. Madeline’s teacher has kindly complied her school assignments so that she too can join us for the week. Although deep in the trenches of the Ortho Spine Service, Klane will actually be able to sneak away for a week’s vacation, as his chairman will conveniently be out of the country at the same time. It will be a nice opportunity for the four of us to spend some time together, since the grueling schedule of his latest rotation has really taken its toll on everyone. The 20 hour work days, combined with round the clock call, find the poor guy going several days at a time without ever seeing the girls awake (not to mention being asleep on his feet!). With three more months to go at such a pace, this trip cannot come soon enough.

As always, we thank you from the bottom of our hearts for your immeasurable support throughout this journey. This latest milestone is as much yours to celebrate as it is ours!

God bless you all,

Amy, Klane, Madeline and Susannah

PS ... Just a reminder that if you have the address for this site saved in your “favorites” or “bookmarks” be sure you’ve changed the stored address from the .com to .org format. The correct address for this site is now:

http://www.caringbridge.org/page/susannah

Saturday, September 14th, 2002 ~ Day +704

Growing up, times two!


Hi Ho,

Susannah is definitely becoming a toddler, in mind, body and spirit! She is really developing an attitude and an independent streak. And we LOVE it! Well, most of the time, anyway. She is now in the habit of dropping to her knees and becoming a limp noodle when she is not interested in whatever we might be trying to accomplish. The scary part of this is that when she goes down, she often also bangs her head with a loud thud! I cringe every time, but she just looks back as if to say, “See Mommy, I told you I didn’t want to do that, why did you try to make me!?!” Seconds later, she is back up and at ‘em.

We are now working on the art of toothbrushing. Upon hearing the words “tee-teeth”, Susannah scuttles off toward the bathroom, opens the drawer, retrieves her toothpaste and climbs up on her stool. After we put on the paste, she holds her brush, runs it under the water and then brings it to her mouth. Now, if we could just convince her to keep it in her mouth, we’d be all set! Ultimately, with lots of silly teeth songs, we manage to get the job done. It is really cute and very refreshing to see her understanding such a concept.

The other day as Klane and Madeline left the house, little Susannah stood at the door and actually cried, signing Madeline constantly for about ten minutes. She didn’t want her sister to leave her!!! As strange as it may sound, this little show of emotion really made our day. Of course, we do not want her to be sad, but the event was just one more reminder of how cognizant she is getting of the world around her. While her expressive language skills have a long way to go, she is growing by leaps and bounds in her receptive language and cognition. Now, if we could only get her to TELL us what she’s thinking!

Lab-wise things remain very stable. With all of the medications Susannah continues to be on, her values are likely as good as they are going to get for now. So, hopefully these numbers will just dig in their heels and hang tight through the fall and winter months. Speaking of fall, plans are now in full swing for our return trip to Minneapolis in mid-October. We will continue our twice yearly visits for as long as her chronic graft versus host disease remains an issue. Truth be told however, the every six month schedule is quite conducive to curbing our periodic need for a Minnesota fix!

With all quiet on the Susannah front, the real newsmaker this month is Madeline. It’s official, we now have a kindergartner in our midst! Where has the time gone? It seems like just yesterday … okay, sorry to get sappy on you, onto the excitement. Miss Madeline lined up in front of her classroom on the first day of school, eagerly holding hands with her new buddy Lara, whom she’d become fast friends with at the “meet and greet” two days earlier (coincidentally, Lara’s father just so happens to be the first person Klane met on his first day of residency, way back when). She kissed us goodbye and was on her way, never even looking back! As we walked away from the classroom, we had a good chuckle: There on the appropriate hooks, outside of the classroom, were 20 brand new backpacks -- and all but two of them were PINK! Yep, 18 little girls, all in one class, with only two lone boys to balance them out. Yikes, Miss Langfus is surely going to have her hands full, not to mention those poor little boys!

Madeline also started soccer this last week. Go Red Dragons! Seeing as I know nothing at all about the game, getting our girl outfitted for her new sport was quite interesting. Luckily the other mothers stepped right in and gave me a quick mentoring session on the art of soccer gear, kindly letting me know that the shin guards go INSIDE the socks, not on the outside! Madeline did catch some flack for her red hair bow on game day, until she informed the dad in question that she actually got the okay from her cousins (“big girl soccer players”) who also wear team colored ribbons to their games! She did really well at the game and even scored a goal. The only trouble was that whenever the crowd cheered for her (Auntie Brenda, the ultimate soccer mom was there, so you can guess that Madeline received more than her fair share of fan support!), the ever social Madeline would turn to see who was calling her name!

We are so proud of both of our little ladies ... as if you couldn’t tell!!!

With much love and gratitude,

Amy, Klane, Madeline the Kindergartner and Susannah

PS … While the main purpose of starting (and continuing) to write these updates has been to keep a journal of sorts for our girls, the other obvious bonus is that it has doubled as a way of keeping our family and friends informed, especially those of you we don’t get to see often enough. As many of you know, however, upon returning home in November of last year we created this site in lieu of the individual e-mail updates we’d been sending throughout the transplant processes. We made this decision with the hope that other families of children with Hurler Syndrome might benefit from our unique, and extended, journey. With this in mind, we have recently added ALL of our PAST UPDATES (from diagnosis to present) to the “History” section of this site, so as to have a full account of “Susannah’s Story” all in one place. If our experiences can, in some small way, help lighten the load of even one family following in our footsteps, then it will all have been worthwhile.

Thursday, August 22nd, 2002 ~ Day +681

Appointments Galore


Hello All,

As the saying goes, NO NEWS IS GOOD NEWS! This has certainly been the case these days in the wonderful world of Susannah.

The word of the week is "wa wa" aka "water" and boy does Susannah enjoy it! Water is not only her new favorite thing to drink, it is also her favorite thing to say. To take it even one step further, it is now one of her favorite things to play. Play? Yes, Susannah loves the bathtub, particularly splashing, thanks to Daddy! The little lady no longer sits calmly during her brief (PICC line style) baths, she splashes like crazy, giggling all the while.

Now, for the beauty report: Susannah got a haircut! Her hair has fallen out and grown back so many times I've nearly lost track, but this most recent batch had gotten so long that when all of the curls were wet, it was halfway down her back! With the summer heat, I decided to give it a little snip. The result is sort of a curly bob style and she sure does look cute. It makes it appear a lot more full and healthy, but the curls in the back seem to have relaxed quite a bit. Perhaps the hair just needs a little time to adjust to its new style and those ringlets will reappear. Still, the top remains quite curly and those locks make for perfect bow material! As for the color, it too has changed again and that classic dark CSA hair has lightened up significantly, giving way to a light brown shade with blond highlights. While the drug induced color may be lessening, the body fur is increasing. Hmmm, there is really no explanation here, as her drug levels have been stable, but according to Dr. Davies, these things have a way of fluctuating when we least expect it. Back to the hair on her head, while it has thickened up a bit since her last episode of falling out in January, it remains on the thin side. Careful styling makes this less evident, but Dr. Davies says Susannah is unlikely to regain that famous "Teichmann mop" until her chronic GVHD fully resolves. It's just one of those things that comes with the territory.

August has sure been a month for doctors' appointments. First and foremost, our visits to the Hem/Onc (aka BMT) docs have been blissfully uneventful (and yes, they are now examining her at each visit). Susannah's blood counts are really hanging in there and have remained stable for several weeks now. Her platelets, hemoglobin, ANC, and even her electrolytes, are all looking stellar. We are still seeing elevated eosinophil levels, however, which are coinciding with the somewhat steady flare in her chronic GVHD.

We did visit the endocrinologist this month to recheck Susannah's thyroid levels, which I am happy to report came back normal, and this is with her synthroid dose down to every other day. Dr. Gottschalk felt we could actually, experiment with stopping it completely, but Dr. Davies said she'd prefer to continue the dose as it stands for a few more months. As I've mentioned before, since thyroid function has a good deal to do with brain development, Dr. Davies prefers to over shoot than undershoot; the effects of under treating are significant, while over treating a bit in this area has no real effect. Dr. Gottschalk, again, suggested trying to wean the steroids to help her growth. Susannah's growth has essentially been at a stand still for the last year, save for a centimeter, maybe two. Now, you know, Hurler Syndrome in and of itself does have an impact, but this dramatic drop off is more likely due to the considerable dose of steroids she remains on for her chronic GVHD. Anyway, you slice it, the best thing for Susannah would be to get her steroid dose weaned down to a safer, less toxic level. If only it were so simple.

We also finally managed to be squeezed in for an "urgent follow-up appointment" with Peds GI to review Susannah's blood and poop studies (YES, the original appointment was back in April; let me tell you this is one efficient subspecialty!). There appears to be no blatant evidence of malabsorption, but another, more specialized, study is in the works for when we return to Minnesota in the fall (the machine to run the test is not available in San Diego). Her labs did show deficiency in zinc and folic acid, so we will begin with a new combined supplement. Oh joy, another med! At any rate, the bottom line is that Susannah continues to show only a very subtle weight gain, despite her excellent intake of late. To be blunt, she has gained, at best, a pound since November, and that's on a good day -- although you'd never guess it by just looking at those adorable and very pinchable steroid cheeks! Obviously her lack of vertical growth, has some impact on her stagnant weight, but with the amount she is eating these days (June has her necessary caloric intake calculated down to the hour and in a nut shell, she is eating constantly!) we should definitely be seeing a little extra meat on her bones.

Lastly, there was a Pulmonology appointment ... well, sort of anyway. After waiting in the exam room for two hours yesterday, the doctor finally came in. It wasn't actually the wait that bothered me, it was the fact that it wasn't even OUR doctor! Dr. Pian was in Mexico, but it seemed that no one felt it was important to tell us. Since all the of the main decisions in this area are deferred to Dr. Milla in Minneapolis, and Dr. Pian is more of a local "just in case" contact, there is really no point in involving yet another physician. Anyhow, we will hook up with Dr. Pian again sometime in the next week to touch base, but I really do not anticipate any changes or new information to report, since as you already know, Susannah's steroid taper is at a standstill and the pulmonologists feel a little growth is the key to improving her pulmonary status. Gee, are you beginning to see a pattern here?!? However, unlike other parts of her body, the continued healing of her lungs is also aided by steroid therapy, but as I've said before, once her systemic dose is lowered, she can be treated "topically" with an inhaled steroid. So, again anyway you look at it, the goal is to taper, taper, taper, then grow, grow, grow!.

Well, enough of the doctor talk! On to the fun stuff ...

The kindergarten countdown is on! Her action-packed summer is winding down and Miss Madeline will start elementary school in two weeks. Of course, she is very excited! It is so hard to believe she is such a big girl already. Kindergarten in San Diego is a full day program, so she will be going to school five days a week from 9:00am to 3:30 pm (someone is sure going to sleep well at night!), with a minimum day each Wednesday. We are looking forward to attending a kindergarten picnic next Saturday and getting to know her new classmates and teacher before school begins on September 3rd.

Thanks for checking in on Susannah (and the rest of us). We wish you all a nice and relaxing weekend!

Love to all,

Amy, Klane, Madeline and Susannah

Wednesday, July 31st, 2002 ~ Day +660

Feeling Good and Having Fun!!!


Greetings, Susannah Fans!

It's Christmas in July! Well, still no Christmas card, but to make up for it, I actually posted some photos. Enjoy! Also, with Susannah's labs and whatnot status quo for the moment, I thought I'd give everyone a good old fashioned feel good update.

All is going well around here these days, although, we are contemplating the idea of installing a revolving door to accommodate the many new therapists in Susannah's daily routine. Hee hee! Coordinating Susannah's schedule has practically become a full time job (the running joke is that she needs her own social secretary!), but the rewards are well worth it and Susannah loves every minute. When the doorbell rings, several times each day, she dashes over to greet her "guests" with a smile and giggle, eager to play their "games". Madeline has officially deemed herself a "junior therapist" and has become an invaluable tool in distracting Susannah from the "yucky" (range of motion) parts of therapy, while at the same time, being a roll model for the activity portions. Of course, there are those days when Madeline politely asks the therapists to treat Susannah in her room as opposed to the playroom, so that they do not "bug" her big girl playing!

Susannah's signing is really beginning to gain momentum. She seems to be learning and wanting to learn new signs almost every day. She is now stringing as many as six signs, interspersed with primitive words, together. Also, she has started to initiate signing on her own to make her needs and desires known, without constant prompting from us. Not surprisingly, her favorite thing to ask for is "Madeline", and has now perfected her own personal sign for her big sis (with her fingers in the sign for the letter "M", she pats her hand across her chest). From the moment she wakes up in the morning, until she goes to bed at night, we are constantly being barraged with requests for big sister; she was, literally, up in arms last week with Madeline off at Grammy and Poppy's! Running a close second to the sign for Madeline, is the sign for "eat". While Susannah does seem to be perpetually hungry, she has also realized that this request can get her out of doing something she's doesn't care for or has lost interest in, and trust me, she's always looking for a sucker! Some other hit signs of the moment are, "cracker", "fish" (Goldfish crackers), "milk", "juice", "cat", "crying", "swing" and "car", just to name a few. She is vocalizing new approximations for "water", "cheese", "car", "please" and "chip", as well as, making many more new sounds each day. Additionally, she has begun labeling objects with both signs and words; this is very new for Susannah and quite a big step in her communication efforts.

Her fine motor skills are also coming along nicely. Susannah can now feed herself an entire meal with a spoon (yes, this is just a tad messy). To make things a little easier, she does have a special sticky placemat from the occupational therapists that we put under her dish to keep it from sliding around. She is also doing really well spearing with a fork and actually prefers this method of eating. Often, if we give her what she used to eat as finger food, she will now refuse it until we give her a fork. She is quite accomplished at making her requests for particular food choices known and has become very adept at specifying precisely what type of music she wishes to accompany her meal, sometimes down to the exact song. As for cup drinking, our little lady does a nice job with an open cup, but to be honest doesn't get much opportunity because she also has learned how to pour things out of a cup and I prefer to keep that to a minimum! Coloring and scribbling have become a favorite pastime of late, and Susannah will diligently sit at the table and scribble away until she has used every color crayon in the box. She is really doing a good job in this area and her grasp is near perfect (in the therapists' words, not mine). We are also seeing nice progress in other OT areas such as, puzzles, beading, stacking (especially knocking down!), and how could I forget, Mr. Potato head -- a big thank you to Sue, our friend and Care Partner from Minnesota who sent her the super duper deluxe version!

It seems there is a higher power determined to establish a British colony in our home. First, there are the two staple Brits in our life: Along with June's presence, we obviously have the influence of Dr. Davies (if these two happen to wind up on the phone together, it's like old home week with talk of tea and Cadbury chocolates!). Now, to add a bit of spice to the mix, both Lee and Wendy, Susannah's physical therapists, are from South Africa, as is the new school district occupational therapist! Daily our home is a buzz with British nursery rhymes and "proper English". Under their tutelage Susannah has completely mastered the motions to the their famous children's song, "Miss Molly had a Dolly"! The running joke is that once Susannah finally begins speaking, she will likely do so with an English accent!

In addition to "Miss Molly", Susannah has become quite fond of many other action songs. She wows us everyone by performing the hand movements to "The Wheels on the Bus", "Itsy Bitsy Spider", "If You’re Happy and You Know It" and "Five Little Monkeys Jumpin' on the Bed". With her new love for an audience, she may just give Madeline a run for her money! Actually, the girls often do little duets together with Madeline singing the words and Sissy acting out the movements. It is precious. The physical therapists have even taken her love for "the monkey song" literally and have begun to teach Susannah how to jump ... on the bed! Shhh, don't tell Daddy! Actually, we're in pretty good shape, as we take the mattress of a roll away bed and lay it on the floor, so both the therapists and I, remain in Klane's good graces while still allowing Susannah the experience. She loves it.

As you can tell, things are rather lively around this place. Miss Susannah certainly keeps us on our toes, in more ways than one!

We love you,

Amy, Klane, Madeline and, of course, Susannah

Tuesday, July 23rd, 2002 ~ Day +652

Milestones


Hi There,

Yesterday marked another milestone event in the world of Susannah. Her platelet count topped 205,000! YIPPEE!!! Believe it or not, the number is finally within the NORMAL range! This is cause for celebration, as we haven't seen a count like this since April of 2000. In fact, I am so used to focusing on the highlighted sections (meaning "out of the normal limits"), which generally make up the entirety of her CBC, that I skipped right over this most impressive value and didn't even notice the super duper platelet count. It wasn't until Klane picked up her lab report late last night and gave a big cheer that I realized what I'd missed! As for Susannah's other blood counts, at first glance her total white count looked equally impressive, but with nearly 10% of it being eosinophils, another flare of her chronic GVHD seems evident. Thus, again we've stepped up the creams and ointments and put the steroid taper on the back burner.

In other news, Susannah's clinic visits have been reduced from weekly to twice each month. While her improved platelet count surely had an impact on this decision, the major reason behind our reprieve had more to do with the fact we spent several Wednesdays in a row at the hospital, only to leave without the physicians ever even laying a hand on Susannah! Nothing, nada, nil! As you might imagine, this little scenario didn't bode well with any of the other doctors in Susannah's life. With the lack of any exam and because we already draw all of her labs here at home, the office visits each week suddenly seemed pointless. So, since her care is essentially being quarterbacked from Minnesota anyway (thank goodness!) and we are obviously very in tune with Susannah's needs, Dr. Davies felt we could just communicate via phone on the off weeks, unless something was awry. This change saves us the hassle of making the trek to Children's every single week and also frees up an occasional morning in Susannah's crazy schedule. We are definitely enjoying our time away from the hospital; these two week intervals are the longest we have gone between doctor's appointments since this all began.

The girls and I are slowly adapting to Klane's return to residency. In time, we will surely re-adjust, it is just that we became so accustomed to having him around, that the erratic schedule and long hours of clinical residency are somewhat of a culture shock. It's funny, the things you let yourself forget! At any rate, he is really enjoying being back in the operating room and considering that the rest of his internship class is now in their chief year, Klane is certainly ready to get his show back on the road! For those of you keeping track, he has five years down, three to go! It is a long haul, but this last year and a half in the lab, allowed us the opportunity to share invaluable time together as a family. It was well worth every moment and something we will always be grateful for.

I am without my right hand at the moment. Big sister Madeline is off at Grammy and Poppy's house for the week to participate in a sports camp at their club. She and her Teichmann cousins are having a blast! The agenda each day varies, but the highlights include tennis, gymnastics, swimming, karate and, as Madeline calls it, "dodger ball" aka dodge ball. As if the days are not exciting enough, my dad has also arranged evening trips to the Orange County Fair and even front row seats at the circus, not to mention dinner at Poppy's favorite restaurant. Who could ask for a more fun-filled summer?!?

All of this, on the heels of a wonderful visit last week from the Calareso clan. Wendy, Joe, Brianna and, of course, "Justy" flew in from Boston for Wendy's father's retirement party (congratulations, Papa O'Hara!). Although the festivities were up north in Monterrey, as luck would have it, San Diego just so happened, ahem, to be on their way to the airport! Our two families had a great time together, and the five days just sped by. Wendy and I even managed to sneak in a long overdue girls' night (it has been more than a year since we've seen one another outside of a hospital room), while the daddies stayed home with the little ones. Though Susannah missed out on the touristy tidbits, just the excitement of having the kids in the house was a huge event in her book; she followed them around in awe. As for our guests, they were absolutely elated with the transformation in Miss Susannah. Although they've had the opportunity to visit with Madeline more recently, the last time they spent time with Susannah, she was on a ventilator, the morning after her major "event" and things were looking pretty grim. Thinking back to those tenuous days, it is incredible just how far she has come ... the unspeakable fear and sadness of that time have given way to renewed hope and joy.

Susannah is truly a miracle child and we count each and every day with her as a blessing. Sure, there are ups and downs, and we know not what the future holds, but time and again we are reminded of the quote that so personifies our sweet Susannah, "There is no chance, no destiny, no fate that can circumvent or hinder or control the firm resolve of a determined soul."

All our love,

Amy, Klane, Madeline and Susannah

Monday, July 8th, 2002 ~ Day +637

Living on the edge ...


Hello One and All,

Well, what d'ya know!?! Susannah actually made it through the holiday without an admission! So we can still say we've never spent the Fourth of July in the hospital, one of the last holidays we've managed to avoid! Truth be told, however, she did have a few close calls that left us feeling as if we were living on the edge ... of a huge cliff! However, with careful maneuvering she has managed to keep at least one foot on stable ground at all times. While it was not my intent to leave you all in the dark, I must confess, I've sat down to compose an update numerous times of late, only to think better of it. Yep, you guessed it, I was afraid of spoiling a good thing!

Overall, Susannah has felt pretty darn good in recent weeks, but as I just alluded to, her temperature and blood counts did creep into dangerous territory on several occasions. I'll spare you the daily ups and downs, but the following is a brief recap. With a sagging white count shortly after her last admission, our little lady was put back on G-CSF to get those neutrophils a-pumping. When, what everyone assumed would be a one time dose left her ANC still droopy, Susannah's Val-Gancyclovir was temporarily held and a week's worth of IV garb was delivered to the house; and so began again the all-too-familiar ritual. There were also many uncertain periods in the temperature department, yet she managed to squeak by and stay just cool enough to remain at home under close observation. Finally, in the last couple of days things seem to be back to "normal".

Oddly enough, Susannah had no other outward symptoms of infection throughout this recent bout with the "mystery bug". Additionally, there seemed to be no significant impact on the hemoglobin and the platelet front, with both values remaining stable at +10.0 and 150,000ish, respectively. I must boast and tell you though, that at one point just before all this started, Susannah's platelet count briefly peaked at an all time high of 195,000! We hadn't seen values like this since before her first transplant! Her (relatively) high platelet count is timely considering she has become quite the daredevil, climbing on chairs and even attempting the kitchen table. Yesterday, Susannah scaled her highchair and pulled it right down on top of herself! Thankfully, she was just scared, but needless to say, we cannot take our eyes off of her!

After having it rescheduled twice, we finally had our follow-up meeting with the school district to finalize Susannah's Individualized Education Plan (IEP) and for the most part, they have seen the light. They are still arguing amongst themselves on a technicality regarding medical versus educational therapy, but regardless, Susannah will receive the services she deserves. She will continue to receive both physical and occupational therapy through Children's Hospital, with the school system tacking on an additional session of occupational therapy each week. The speech therapy discrepancy was settled and she will maintain her five weekly sessions. Fortunately, our insurance companies were willing to pitch in and fill in the gaps (even though speech therapy technically becomes the sole responsibility of the school system after the age of three), so this particular discipline will now be divvied up between the three agencies. As an enhancement to her speech therapy, the district will also provide twice weekly intervention from the Deaf and Hard of Hearing Program. Since her immunosuppression prohibits Susannah from attending preschool, the school district's Home Hospital Program will be sending out a teacher four times each week. As with the teacher, all therapists will continue to treat Susannah in our home to reduce her exposure to infection. We did reach an impasse with the district regarding Susannah's adaptive PE program. Apparently, they only see the need to offer it "at school", but since Susannah's school is in the home and because she is entitled to this service, she should not be penalized by her homebound status. Thus, we will mediate this final detail of her IEP, but otherwise we are very pleased with the intervention and level of service Susannah will be receiving, both from San Diego City Schools and our insurance companies via Children's Hospital.

On a lighter note, Susannah has found a new love. His name is Wilbur and let me tell you he is certainly giving good ole Barney a run for his money! Wilbur is a cow puppet who talks and teaches kiddos the parts of the body; his monologues are interspersed with snippets of children singing and dancing to tunes about eyes, ears and even tails. Susannah sits happily in her little rocking chair acting out the movements and pointing to the appropriate areas as Wilbur makes the requests. I am not sure if this odd little puppet is a national phenomenon or not, as some of the footage is filmed at a local beach park here in Del Mar. At any rate, if you can get your hands on a copy (Aunt Tami came across it in the Nordstrom shoe department, of all places!), Susannah highly recommends it.

Now for the most exciting news of the last few weeks! Let me tell you, we have in our house the most dedicated little ballet dancer around, not to mention the biggest ham of them all! Miss Madeline performed, and boy do I mean performed, in not one, but three separate dance recitals in one week! Her ballet class had its annual recital on the "big big stage", after months of practicing and what seemed like endless dress rehearsals. The very next day, her Wee Dance class from preschool finished out the year with a performance at the fair -- yes, believe it or not, San Diego actually has a county fair (never mind that it is sandwiched between the beach on one side and the interstate on the other!). Then, just five days later, it was back to the fair for an encore performance with her ballet class! With the brightest smile you've ever seen and an unmistakable bounce in her step, our little ballerina danced her heart out. We’ve never been prouder!

Thank you so much for so faithfully checking up on Miss Susannah (and her big sister!). Your continued support, prayers and well-wishes mean more than you can imagine and truly give us the strength to keep on keeping on!

Love Always,

Amy, Klane, Madeline and Susannah

Wednesday, June 19th, 2002 ~ Day +618

The Gift that Keeps on Giving


Good Morning to You!

Miss Susannah decided to give her daddy an early Father's Day present. It seems she wasn't keen on the gift she and her sister had planned on giving Klane and so she surprised everyone with a gift all her own! On Saturday evening, our darling daughter spiked a doozie of a fever and earned herself a three day stint at Children's Hospital. I suppose she wanted to be sure she wasn't missing out, since this is one of the few holidays she has never spent as an inpatient.

Thankfully, this admission was pretty uneventful, with Susannah's fever subsiding after only 12 hours. Her blood counts did take a dip warranting G-CSF, but not nearly as drastic as in months past; in fact, they rebounded nicely after just one dose. Thus, with 72 hours worth of antibiotics flowing through her veins, a stable chest x-ray and negative blood cultures (and, oh yes, how could I forget, a visit from those doggies!), Susannah was cut loose yesterday afternoon and is now back in the comfort of her own home.

However, just to keep us jumping and add a little more adventure to our lives, as we tucked Susannah into bed last night, her temperature was teetering on the brink at 100.4 degrees, completely out of the blue. As the night went on, she did cool down a bit, but needless to say, there was a fair amount of nail biting going on around this place. Nope, life here at The White House, is never dull!

We hope all of the dads out there had a very Happy Father's Day!

Much love,

Amy, Klane, Madeline and Susannah

Tuesday, June 11th, 2002 ~ Day +610

Summertime and Sunshine


Hi Everyone!

As I alluded to in my last update, our summer began with unprecedented excitement. For six glorious days Klane and I took in the beautiful sites and charm of Victoria, British Columbia. JUST THE TWO OF US! Yes, we actually left the country! Okay, so it was only Canada, but we did cross a border nonetheless! These plans, of course, had been in the making for some time, but we kept the trip under wraps to prevent a certain little lady from pulling one of her famous stunts. Superstious? Perhaps, but can you blame us?!?

While we had many reasons to celebrate, the official reason for trip was credited to Klane's receiving a research award at the annual meeting of the American Orthopaedic Association. An elegant hotel, first class air travel, wonderful dinners and even a black tie gala to top things off ... and best of all, it was all courtesy of the AOA and Zimmer Orthopaedics! Ahh, yes, and how could I forget to mention my wonderful spa treatments! I think I was even more spoiled than the guest of honor. Klane did have a few daytime obligations, but we managed to take in all of Victoria's can't miss attractions -- whale watching, afternoon tea at the renowned Empress and of course, the beautiful Butchart Gardens. The entire trip was absolutely delightful, and to top it off, we actually were welcomed by six straight days of crystal clear sunshine. Quite a stroke of luck for the Pacific Northwest!

We definitely missed our girls, yet we were remarkably relaxed and truly able to enjoy ourselves. It was a nice break, nearly an entire week free from the responsibilities of meds, IVs, blood draws, therapies, dressing changes, and doctor appointments. These things have become such a part of our daily lives that I never dreamed their absence could be so liberating. As we boarded the plane, I realized that this was the first time in 26 months that our days could begin without mixing up medication cocktails, but fear not, just incase we missed the routine, those ever friendly Canadians kindly introduced us to "the Caesar", another sort of morning cocktail!

Seriously, the real credit for our peace of mind goes to two amazing ladies. Vowing to protect Susannah with her life, June essentially moved into our house for four of the six days, only to be relieved by Kristy, our primary nurse and friend from Minnesota (now living in Colorado), who graciously flew in for a long weekend to make this trip a reality. The two made a perfect team and our baby could not have been in more capable and knowing hands. Not only did they manage Susannah's medical needs with ease, but they meticulously coordinated her intense appointment schedule, all the while showering her with their love and attention. And, yes, Kristy did some serious spoiling; would you have expected anything less?!? Sure, a couple of mini-crises did pop up (we are dealing with Susannah, after all), but even then, our "super nurses" handled everything, never even blinking an eye. We had absolutely nothing to worry about! Without June and Kristy, our wonderful getaway would never have been possible.

Also, we are grateful to our many friends and family members for their combined effort in ensuring Madeline's well being during our absence. She had quite the "vacation" of her own, visiting her buddies, having sleepovers and going on all kinds of adventures. We were greeted at the airport by one very happy five-year-old, full of smiles, as she recounted tales of her fun-filled week!

As for Miss Susannah, she is doing quite well. Her counts remain stable and she even seems to be staving off a runny nose and cough without allowing them to become anything more ominous. We are still waiting for the skin manifestations of her recent GVHD flare to cease, but the corresponding blood counts and pulmonary issues seem to be a thing of the past. Keep those fingers crossed!

We met with the school district for Susannah's IEP and, needless to say, we already have another meeting scheduled. As anticipated, they did offer a considerable amount of intervention; still, it was not nearly up to par in regard to speech therapy. There is a basic model which the district follows and to get them to deviate from it is like pulling teeth. However, everyone else involved in Susannah's care finds it unacceptable that their arrival onto the scene actually decreased this particular therapy, as opposed to enhancing it. Round two begins next week.

Summer is definitely upon us. While Big Sister has been splashing about in her new inflatable flower pool (pink of course), Susannah has been busy mastering her new slide. She can now climb up the three steps on the plastic Little Tykes slide, stabilize herself at the top and then slide on down -- ALL BY HERSELF! She is becoming such a big girl.

One final thing, we have a star in our midst! Our little friend, Molly, will grace the pages of the July 16th issue of Woman's Day, yep, the magazine! Not only does the article showcase Molly's sweet face, but also Hurler Syndrome and her journey through the transplant process. Way to go, Mol, now we can say we knew you when!

Happy summer to all! Be sure you take some time and enjoy the sunshine!

Sending love your way,

Amy, Klane, Madeline and Susannah

Wednesday, May 29th, 2002 ~ Day +597

Here's the scoop!


Greetings All,

Many apologies for the long silence, but we've been on the go! No sooner did we return from Minneapolis than did we pack our bags again (no small feat with all of Miss Susannah's paraphernalia) for a long weekend at Grammy and Poppy’s with a little sojourn to up to Redlands to celebrate our oldest nephew/ cousin's graduation from college. It required a good deal of tag teaming to attend the big event with Susannah in tow, but with a bit of creativity we managed to pull it off. We wouldn't have missed it for the world ... congratulations to Darren, we are very proud of him!!!

As for our trip to the Twin Cities, it was great, in every aspect. It was an amazingly refreshing feeling to be back in the U of M clinic; it really made us long for their well-oiled machine. Everyone knew Susannah, who she was, and exactly what she has been through. They knew her routine, and better yet, they understood all of our preferences and idiosyncrasies. There was no explaining, no "how to's". From the moment we arrived, everything fell right into place and everyone knew just what Susannah needed, and exactly what to do and when to do it. Things went so smoothly with the clinic and other appointments, it was almost as though we had never left.

Nevertheless, after lots of hugs and hellos and a little showing off by Susannah (walking performances were the major attraction), we got down to business. Medically speaking, the timing of the visit could not have been better, as Susannah had a flare-up of her chronic GVHD just days before we headed for Minnesota. Thus, Dr. Davies and others were able to visualize things firsthand and make appropriate recommendations. A far cry from the quick tummy touch and obligatory lung listen that Susannah has become accustomed to at home, within minutes her clothes were off and Dr. Davies was examining every inch of her little body, from head to toe, and in the midst of it all, managed to introduce Susannah to the joys of a good back scratch! We discussed the week to come, visited, and of course came up with "a plan"!

Backing up a bit, the usual skin issues related to a flare in Susannah's GVHD were also accompanied by a puffiness, reminiscent of her early bouts with the disease last spring, and coincidentally, or not, she began having some early evening desaturations, as well as, a slight cough. As conversations with the pulmonologists ensued, it became apparent that these two factors would put a bit of a glitch in our travel plans. It was determined that it would be a good idea to secure supplemental oxygen for our flight. Unfortunately, this is an entity that is not permitted when flying with Corporate Angel Network. So, with much chagrin, we gave up our seats on a luxurious corporate jet (which we'd have had all to ourselves), for seats on an oversold 757. You can imagine our excitement at embarking on such an adventure with two children, but add an immunocompromised toddler to the mix, and boy do you have a treat!

While the flights went as well as could be expected (considering we had to try persuade a three year old to wear a mask for hours on end), we did have a bit of a scare on the outbound leg when Susannah's oxygen saturations dropped to 78%. Despite the low sats, she looked good and did not appear to be in any distress and as the altitude decreased, her numbers went up accordingly. Regardless, we did keep the crew's emergency oxygen tank close at hand. Once back on the ground, Susannah was back up to 100%. Thankfully, our return trip was much less eventful. Clear skies enabled the pilot to fly at a lower cruising altitude, which seemed to stress Susannah's system a little less. Bedsides, we had unlimited canisters of oxygen readily available which we blew at her the entire way. I must say however, my nerves were calmed immensely when, as we boarded the flight home, we bumped into two of Klane's colleagues who were returning from a continuing ed course at the Mayo Clinic. While I am well aware that bones and power tools are their business, I figured that in a pinch, the three of them could pull out the necessary stops to halt a crisis.

With all that said, you can probably guess that our visit with Dr. Milla, the pulmonologist, aka "Uncle Carlos", was informative. His feeling was that Susannah's pulmonary status had not changed a whole lot in the past six months. What Susannah needs, is to GROW! Easier said than done considering her substantial steroid dose and, of course, the Hurler Syndrome issues. Until her body grows, her pulmonary reserve will remain limited, leaving her on the precarious ledge that she slipped from during the airplane flight. This also likely explains her continued mild desaturations at night. When she reaches a very deep sleep, her breathing rate drops low enough that her body cannot compensate, and then we see the desats. Despite the ominous matters at hand, we still had a nice visit, and we were able to catch-up with his new fellow, Spike, who we know well from those ICU days up on Unit 4A, when he was a second and third year peds resident. Yikes, time flies! In the end, "Uncle Carlos" sent Susannah on her way with an edict to grow and, for the meantime, a few puffs of an inhaler each night. He, like everyone, was elated to see all of the progress that she has made in so many of the other aspects of her being.

Our visit, with the dermatologist, was somewhat of a social visit, as Dr. Hordinsky's original involvement with Susannah stemmed from a lack of hair growth last Spring. Other than a small patch on her left eyebrow, the CSA is now back in full force and Susannah exhibits an abundance of hair virtually everywhere on her body. As for the hair on her head, she is now sporting lots of curls, long enough for a variety of barrettes and bows, and finally again, has enough hair to actually style! Dr. Hordinsky did, however, provide a suggestion for a new steroid "mousse", as opposed to the unappealing oil with which we are currently treating her scalp, an area very sensitive to the GVHD flares. We'll give it whirl!

Unfortunately, Dr. Olsen, the ophthalmologist, was out of town, but his associate found Susannah's eyes to be in fine shape. Her CMV retinitis continues to be inactive and her visual acuity has improved since her exam last Fall and is essentially normal for a child her age. As usual, Susannah had a particular dislike for the "eye guy". Who could blame her? All the drops and lights, and ohhh yes, that lovely eye speculum, what a delight! Well, at least he was the bearer of good news.

After the traumatizing time up in ophthalmology, we moved onto a more friendly appointment with the ENT, another of Susannah's infamous "uncles". Dr. Rimmel, errr "Uncle Frank", basically chit chatted the appointment away and was pleased as punch to see Susannah's success and willingly ate his words (if you recall, he as much as told us, on several occasions, that she would never come off the ventilator). As for her ears, not to mention her airway, things on the inside look shipshape. Susannah still boasts one ear infection in her lifetime and no tubes ever (knock on wood!), quite a feat for a Hurler babe. From an auditory standpoint, Susannah's hearing once again showed subtle improvement, with only a mild high frequency loss present. Basically, she can hear most everything, but with the help of her aides, she should be able to pick up the subtleties at the ends of words with silent consonants, something very beneficial to her speech. Now, if only we can find a way to get her to keep the aides in her ears!

Our appointment with Dr. Charnas, the neurologist was uplifting. He was, of course, elated with Susannah's progress and optimistic about her future. What you have to understand about Dr. Charnas, is that he did not know Susannah before she was comatose for months on end. So for him to see her alert, interactive and displaying all the charm that she is famous for was a real treat. Dr. Shapiro, the neuropsychologist, made an informal, cameo appearance and sat with us, and Dr. Charnas, to discuss many of the aspects of Susannah's development, particularly her speech and her therapy needs. Susannah really showed them her stuff and was especially intrigued by Dr. Charnas's socks! Dr. Shapiro, too, was quite pleased with Susannah's progress, and expressed as much optimism as she was capable of (she doesn't get excited about much!). We agreed that in the Fall, Susannah would undergo the usual formal battery of tests to gage her progress. They were also interested in seeing an MRI or CT scan of Susannah's brain, but they'll have to get clearance from Dr. Davies, who was not nearly as interested in this imaging, before subjecting Susannah to general anesthesia.

The biggest news of the week was in the labs! Susannah's engraftment remains steady at 100% donor and her enzyme activity level of 69.1 continues to knock everyone's socks off! She has far exceeded a normal enzyme level, surpassing not only her mommy and daddy, but also her non-carrier donor. Yipee! Susannah's platelet count reached a record high of 154,000 and her hemoglobin topped off at 10.8 (and that was after giving up a ton of blood to lab tests earlier in the week). Her white count and ANC were also rock solid. Hooray! Was there something in that Minnesota air?!?

A number of labs pertaining to her chronic graft versus host disease were also drawn and, as anticipated, came back with rather elevated results (although, if it helps at all, they have certainly been much worse). As this particular variety of GVHD varies from the acute form that most people are familiar with, and functions more like an autoimmune disorder, for now her regimen of immunosuppression is here to stay and her limited exposure to the outside world remains status quo. In fact, while a few weeks back there had been whispers about beginning a wean of her steroids, with this recent flare, Dr. Davies is actually toying with the idea of adding an additional dose of prednisone on Susannah's off day. However, for now we will just watch and wait and use that only as a last resort, since it is far safer, as far as side effects go, to keep her to the every other day regimen.

Susannah really brightened up during the week in Minneapolis. She seemed to take one of those developmental leaps that she does every now and then. It was clear that she remembered certain people who were really involved in her care. She also very much remembered the physical therapy gym and it's play structure. We literally had to peel her off the slide/ climber each time we went by to visit someone there! Perhaps the exposure to her former environment and all of the familiar faces and places, triggered a little fire in her mind. It really was astonishing and she did look wonderful.

The week was not all doctors and labs however! Our hotel room was like a revolving door; I think our social calendar was more full than our appointment schedule. We had a chance to visit with our many old friends, including the nurses, doctors, therapists, families and special individuals who really represented the silver lining of our BMT cloud. We also made it over to the floor, now "Temporary Unit 4A" located on Unit 4E due to a renovation. We saw many of the other individuals who helped take care of Susannah. Everyone was so happy to see her upright and mobile -- again, the walking was the major attraction! We did get to meet several of the new families going through the transplant process, some of whom were having a pretty rough going of it at the time. Hopefully they were given some inspiration by seeing Susannah and hearing the staff marvel over her remarkable story.

Of course, Madeline had a very busy week as well. She kissed us goodbye at the airport, jumped into Joan's waiting arms and never even looked back! We did manage to lure her to the hotel for a night here and there, but not without promises of fun and frolic. She came over one evening to play with her buddy, Jessica, who is now nearly three years post-transplant for Hurler Syndrome (and doing beautifully!), and also the Dinnaken crew, including Kelsey, Gayle and Paula. Thursday night, Joan treated Madeline and I to a night on the town for a performance of "Alice and Wonderland" at the Minneapolis Children's Theatre. The next evening we all shared a wonderful evening with Joan and her family, where we enjoyed a delicious dinner, as well as, an original marionette performance by Joan and Madeline! With all of these friends, it is no wonder Madeline came through our time in Minnesota with such a great demeanor and growth.

As always, thanks for checking in on Susannah, and for your patience during our silence. We are delighted to have positive news to report on our little one and look forward to sharing more of the same in the coming months. With May quickly drawing to a close, this Friday, we will culminate our month of traveling with one final excursion ... stay tuned!

Lots of love,

Amy, Klane, Madeline and Susannah

Friday, May 10th, 2002 ~ Day +578

Knock on wood ...


Hello There,

No news is good news ... okay, knock on wood right now, please! The recent weeks find Susannah's blood counts creeping back toward "normal". Platelets are over 100,000 (yippee!) and white blood cell/ absolute neutrophil counts are way up to 9,000/ 5,000, give or take. At 9.0 her hemoglobin is the only thing lagging behind, but it is still more than acceptable. That darn runny nose and goopy eyes seem to be the last remnants of her recent escapade and hopefully, they too are on their way OUT!

Now that Susannah is three, she will begin school! Or rather, school will begin coming to her. While she has officially graduated from the Birth to Three Program, because California has a unique set-up, many of her existing therapists and services will remain in place and for now the school district will only supplement. She will however, be transitioning to a new teacher who is from the district over the next couple of weeks and we will sadly say goodbye to Miss Phyllis, who we will truly miss. Of her own accord, she is planning on staying a few weeks extra just to ensure that the change over is not unsettling to Susannah. It seems we will now be adding another subject to Susannah's "curriculum". In addition to physical therapy, the school system will offer her PE! Yes, as in adapted physical education, apparently the goal of this discipline is to focus on "physical play" so that when it is medically appropriate for her to integrate into an actual school setting, she can do so without fear of being overrun by the other students.

We could potentially have a major shift in Susannah's speech therapy as the school enters the picture and currently I am working out the details to minimize any disruption to Susannah and the progress she is making in this area. There are several avenues to explore regarding speech therapy and certainly I will leave no stone unturned in deciding what is best in this delicate situation. We have a meeting on the 22nd to sit down with eight different people from the school district to formalize an Individualized Education Plan (IEP) for Susannah and establish some educational goals for the next year. We have already bombarded them with heaps of reports and letters documenting the intricacies of Susannah's disease and current condition in an effort to by-pass the almost assured bureaucratic uproar regarding Susannah's intense therapy requirements. Still, we anticipate there will be need for some significant parental advocacy on the part of Susannah. Ahhh, you may think we are old pros at this by now, but I assure you it gets to be quite tiring and we would certainly welcome an uneventful transition. Wish us luck!

Regarding her speech, Susannah is once again beginning to string two and three signs together. She is even now able to combine some words and sounds with signs. Of course, she often needs a little prompting, but if we ask her what she wants, for example, she will vocalize "want", followed by the item (usually a sign) and then she'll politely complete her thought with either the sign for "please" or even a verbal "psssss". A few of her favorite combos these days are " 'waaa' more music 'psssss' ", " 'Dada' book please" and "eat more 'psssss' " Granted many of her vocalized words sound the same, but they are definitely becoming distinguishable, especially in context; it just so happens that many of her new words begin with "B", so "bye bye", "ball", "banana", "book" and "bird" all have a similar sound. Susannah is also beginning to make choices. When asked whether she'd prefer an apple or banana, she is able to point to one or the other and then speak her primitive version of the chosen word, "baa na" or "aaa pu". Slowly, but surely she is heading in the right direction and communicating more each day.

On other fronts, Susannah is quickly becoming a master at the game of catch. She just loves to play with balls these days, tossing them every chance she gets. Large balls, tennis balls, golf balls, beach balls, no matter, any old ball will suffice. Her physical therapists, Lee and Wendy, have even managed to teach her to kick a ball! It is still a very delicate and tenuous kick, but a kick nonetheless. Each time she does it she gets so excited, claps her hands like crazy and, of course, we all cheer! Madeline is especially happy about this new trick, as she longs for the day when Susannah can join her at Sunday soccer "matches" with their buddies.

Susannah has also taken up "bird watching". June is an avid birdwatcher and we happen to have a resident blue jay (or more precisely, a scrub jay) in our backyard. So the two have a daily ritual of placing peanuts upon our fence and then rushing inside and waiting to see their little blue pal land on the slats and scoop them up. As you might guess, our little feathered friend played a big roll in the addition of "bird" to Susannah's vocabulary. June's chief goal of late is to coax Susannah into saying "tweet" when she sees the bird, as opposed to the barking noise she currently favors (it seems any outdoor animal, is a dog in Susannah's eyes). Now, I must tell you Madeline takes much of the credit for discovering this jay, as well as his propensity for peanuts. A while back she had made a bird feeder from a paper cup, peanut butter and birdseed. Well, we attached it to a tree out back and the next thing we knew it was gone. Yes, the whole feeder, cup and all, were nabbed, leaving only the lone red string. At first she was distraught, but now boasts, "Sissy, your bird friend liked my peanut butter so much that now he comes to visit you!"

Speaking of Miss Madeline, her five year old check-up found her in perfect health. She is growing like a weed and is definitely following in Klane's footsteps in the height department. She is in the 95th percentile in both height and weight. While she received two of the necessary shots for entering Kindergarten this Fall, she lucked out, for the time being and got to skip one -- she has her little sister to thank for the reprieve.

On Monday, we head for Minneapolis. It will surely be a busy week of appointments, but we have allowed plenty of extra time for visiting! Ever the socialite, Madeline's schedule is nearly as full as her sister's. We are all really looking forward to the coming week, catching up with all of our old friends in the Twin Cities. After spending a year and a half there, it will always be a huge part of our lives ... strange as it may sound, it almost feels like we are going "home".

We'll certainly touch base upon our return, as we will surely have lots of news to report. As always, we thank you for keeping our little lady in your prayers.

Love to all,

Amy, Klane, Madeline and Susannah

Wednesday, May 1st, 2002 ~ Day +569

Another April in the Books


It's May Day!

Wow, we made it through another April! It's hard to believe that we managed to squeeze in two hospital admissions amidst all of the month's celebrations! Then again, we always knew our Susannah thrived on excitement.

In the last several days, all of Susannah's blood counts have crept back up to a more desirable level. Her bone marrow has really started working overtime to produce all of the new cells needed to help her body fight off infections and stabilize her counts, such that we were even able to discontinue the G-CSF ahead of schedule. With any luck, this time those neutrophils will actually dig their heals in and stick around for a while ... better yet, permanently! After a ten day hiatus, we will now re-start her val-gancyclovir at a lower dose to see if that will take some of the pressure off of her already stressed marrow. Otherwise, she is feeling well, aside from a new bout of itching and dry skin. Cream, cream and more cream! Her weight is climbing and has peaked at a record 12.3 kilos. We are still awaiting results from all of the blood and poop studies done by the GI docs on our visit with them two weeks ago. They stole a whopping 21 cc's of blood from our little one, so let's hope they can come up with some helpful suggestions.

We ended the month of April with a bang! The long awaited "fairy party" was a smashing success. Madeline was truly queen for the day and reveled in every moment of it. June was kind enough to stay behind with Susannah so all the "big kids" could travel to the enchanted forest for an afternoon of fairy dust wishes and magical dreams. The little fairies and princes were dressed in costume as they hunted for fairy treasure, dined on fairy cakes and fairy nectar, listened to fairy stories, played fairy instruments and sang fairy songs. There was even a visit from a real fairy!

This past weekend we also had a little dinner party to bid farewell to Casey and Jill, our nurse friends from Minnesota who have been traveling nurses here at Children's Hospital San Diego since our return home. We will really miss having them around and are grateful for the enormous roll they played in easing our transition, or better yet educating the staff here on Miss Susannah.

With all of the April festivities now behind us, I have set aside my party planning hat and donned my travel agent hat. May will be a traveling month in our household, the highlight being our family's trip to Minneapolis/ Saint Paul for Susannah's 18 month post BMT check-up. Arrangements are still in the works, but as things stand now, we will be heading back the week of May 13th -- hopefully, we can avoid a spring snowstorm! We are really looking forward to visiting with our "Minnesota family". Speaking of, the other day I was on the phone with a complete stranger who asked me if I was from the mid-west. When I said no, I was a native Californian, she replied, "Oh, I was asking because my daughter goes to school at the University of Minnesota and you have the same accent as all of her roommates there!" Yikes!!! And, for all you Minnesoooooootans out there, I didn't even use words like "ramp", "yet" and "pop"! I guess I need to bone up on my California lingo!

Klane is flying to Salt Lake City on Thursday to present some of his research to the Pediatric Orthopedic Society of North America (POSNA), so the girls and I will be going it alone this weekend. We will surely miss having him around, but are very proud of all the work he has accomplished this last year. Time has really flown by; he will be reentering his residency program in mid June, hence we are really trying to savor the next several weekends!

Today, I had the treat of watching Madeline in her ballet class where she learned the tradition of wrapping the May Pole. It was precious to watch a dozen little girls intently listening as the ceremony was explained and then to see their smiling faces as they actually got to skip in circles and wrap the ribbons round. While it didn't snow here last month, like it did in some ... ahem ... other states, we certainly did have our fair share of April showers, so now let's bring on those May flowers!

Love and hugs,

Amy, Klane, Madeline and Susannah

Friday, April 26th, 2002 ~ Day +564

There's No Place Like Home!


Good Morning,

We're happy to report that Susannah is now back in the comfort of her own home and, let me tell you, she couldn't be happier about it! She just can't get enough of her own toys and has been busy exploring her turf from the moment we walked through the door. It is exciting to see her show so much enthusiasm and even more so to witness her increased recognition that this is her home and this is where she belongs. Things in that sweet little head of hers are really beginning to click these days.

In the last couple of days, Susannah's absolute neutrophil count (ANC) has climbed 950. Although still not stellar, it was high enough to safely discontinue the IV antibiotics (her cultures were negative, but with her counts so low they'd been extended as prophylaxis), thus making her care manageable as an outpatient. Considering the fact that there were not any filtered transplant rooms available, it was decided that Susannah was probably just as safe, if not more so, being at home than in the hospital.

While her counts continue to leave a lot to be desired, the differential does show lots of new growth and immature cells, which is promising. We are optimistic that they will take off any day. In the meantime, she remains of G-CSF twice daily, which we will now be administering here at home. Unfortunately, her platelets finally decided to get in on the action and dropped quite a bit yesterday, so we will be monitoring them daily, along with her white count. Hopefully, all of these blood draws will not cause her hemoglobin to follow suit.

In spite of her low counts, Susannah was feeling quite chipper this admission and was certainly well entertained. She was treated to a few visits from her new pals at the Canine Care Program. These dogs are part of a volunteer organization to cheer up children in the hospital ... very official, the dogs even wear ID badges with their photos, just like regular employees! Now, they couldn't actually enter Susannah's room, but that certainly did not matter to our gal. She shrieked with utter delight each time they appeared outside her window and even started crying once in excitement when she couldn’t get there fast enough. The moment she caught sight of the furry critters at the glass, Susannah took off for the door as fast as her little feet would carry her, never mind all of the IV lines impeding her. On her way, she would slap her leg frantically in the sign for dog, giggling all the while and on occasion she'd even vocalize a very boisterous, "dog"! She’d bang on the window, let out great big belly laughs, then press her face up against the glass and "kiss" the dogs. Then the barking would start. Barking you ask? Well, let me just tell you our little darling does a mean dog impression! She can bark with the best of 'em. And bark she did, causing passers by to do a double take to see whether it was actually the dogs barking or Susannah. It was hysterical.

While the coming days are sure to be tedious, we are hopeful that home life will agree with Susannah. We are just thankful that her little body managed to rebound enough to don her ruby red slippers, as there truly is, "no place like home"!

With love,

Amy, Klane, Madeline and Susannah

Tuesday, April 23rd, 2002 ~ Day +561

Rock Bottom Counts


Hello Again,

It appears Susannah is in no hurry to leave the hospital. While her fever remains in check, her absolute neutrophil count today is down to 24 -- no that is not a typo, nor did I mean to write 2,400. Her ANC is actually a frighteningly low 24.

While such a drop is obviously disturbing, it is the all the more so considering that the rapid decline in Susannah's counts has occurred in the wake of twice a day G-CSF. We have indeed been holding the val-gancyclovir in hopes that it will give her battered marrow a little reprieve. Still, her neutrophils have yet to show any real sign of responding. The good news, however, is that her platelet count and hemoglobin are hanging in there, with only a slight decrease. Additionally, a viral battery done yesterday, was negative for a multitude of viruses and there is no growth on the blood cultures drawn Sunday night.

So, for now, we wait ... wait for the giving and holding of drugs to do the trick and stimulate her bone marrow. And, we pray ... pray for those cells to grow and that Susannah's counts return quickly, so as not to subject her already fragile body to any further risks for infection.

The uncertainty of the situation at hand is incredibly stressful, especially considering that Susannah and her complex condition are still an enigma to the folks here in San Diego. Thankfully, we are blessed with the amazing support of Dr. Davies, who, although half way across the country, continues to remain abreast of even the most intricate details. The doctors and nurses here are constantly amazed by her availability and responsiveness. Regardless of the day or time, our pages are always answered immediately, often within seconds; if she happens to be traveling, she makes it a point to check in. Of course, her medical expertise is very much appreciated, but her calming and reassuring demeanor also go a long way for morale in these unnerving circumstances. She is a true gem and we would be lost without her.

As you are all well aware, Susannah is one tough little lady, but as has been proven time and again, your thoughts and prayers are instrumental in seeing her through even the most difficult times. Thank you so very much for continuing to hold her close.

Love,

Amy, Klane, Madeline and Susannah

Monday, April 22nd, 2002 ~ Day +560

She almost made it ...


Hi Everyone,

Oh, she was close, so very close! Susannah nearly managed to make it all the way through birthday week without medical incident. She even held on until the very last hour of the "cousins party". In fact, as the double celebration was winding down and the birthday cakes were cut, she still seemed fine, but when she repeatedly refused to even sample the frosting, something was clearly awry.

Sure enough, as we removed the thermometer from underneath her little arm, staring back at us were the numbers 101.1. So, as we bid farewell to our guests, we gathered up a three day supply of gear and headed for what seems to be becoming our home away from home. Susannah was admitted to Children's Hospital on Sunday evening, the fourth time in two months.

To tell the whole story, her labs from Friday unexpectedly showed a low neutrophil count, so we began G-CSF at home with plans of continuing it daily over the weekend. As anticipated, on Saturday her labs showed a slight improvement, so you can imagine our surprise when, in the midst of the birthday party, we received a call from Dr. Roberts reporting that Sunday's labs showed a significant decline in neutrophils, to 324. With no overt signs of infection present – clear eyes, goop free nose and completely afebrile - we were all a bit stumped, not to mention worried. A quick call to Dr. Davies provided much piece of mind, as well as, instructions to increase the G-CSF administration to twice daily and to temporarily hold the val-gancyclovir, as it can suppress white cell production.

What a difference an hour makes. By 7:00pm the fever had reared its ugly head and we now had a plausible answer to the mysterious low counts. Another pesky virus? Likely, but nonetheless, the usual neutropenic fever work-up was ordered, complete with all the bells and whistles. Ironically, by the time we arrived at the hospital, Susannah's temperature had come down a bit and by the next set of vital signs it had subsided completely. Still, she had spiked and in her situation, once that happens, there is no turning back. While the absence of fever, potentially qualifies Susannah for an early discharge, for now she will be staying put until she shows an increase in her white count. Unfortunately, this morning's ANC dropped even further, down to 150. With her counts so low, there are certainly no plans to discontinue IV antibiotics, nor send her anywhere.

As mentioned earlier, Susannah ALMOST made it through birthday week ... today, Miss Madeline turned five years old. Grandma Dona and Mommy joined her at preschool this afternoon for the long awaited (she has the second to the last birthday in her class) "classroom cupcake party" and boy, did she wear that birthday crown well! Then, just like her sister the week before, she was treated to our famous, err infamous, birth time serenade, promptly at 1:07pm. Tonight, we celebrated with a yummy picnic at Susannah's "work", which included cake and candles, and of course, frosting! Madeline was given the option of going out for dinner, but she would have absolutely no part of it, insisting her birthday would not be complete without Sissy right by her side. So, together we ate, sang, blew out candles and celebrated in the unique way we have become so accustomed to in the last couple of years. While Madeline's actual birthday was somewhat reserved, the grand finale of birthday week, the long awaited "fairy party", is still to come this Saturday. She can hardly wait!

While we so wish Susannah were home and this update filled merely with details of pinata busting and birthday fun, we know full well that under the circumstances Sissy is exactly where she needs to be right now. Please pray that her little body stays strong and that those cells grow, grow, grow!!!

All our love,

Amy, Klane, Madeline and Susannah

Monday, April 15th, 2002 ~ Day +553

Happy 3rd Birthday,Susannah!


Dear Friends and Family,

Our littlest princess turned three years old today! As is tradition in our family, the birthday girl was serenaded with "Happy Birthday to You!" promptly at 8:45 this morning, her birth time. She giggled with excitement and immediately responded by walking over to her toy piano and pushing the corresponding button to play the very same song!

This is the first birthday, ever, that Miss Susannah has celebrated in our home; both her first and second birthdays were in Minnesota. An intimate gathering around a birthday cake lit with three glowing candles completed the big day. Although we have been practicing her blowing skills, Susannah was so mesmerized by the flames that she needed a little assistance from Madeline to actually blow out the candles. The highlight of the evening was definitely the frosting! Susannah delighted in sticking her fingers into the cake and then licking the frosting off, bit by bit. Apparently she thought the cake was so scrumptious that she wanted to share it with her sister, by repeatedly coating her little fingertips with frosting and then sticking them into Madeline's mouth.

Not surprisingly, Madeline was the official party coordinator, leading all the festivities and especially making certain that Susannah had the necessary "big girl" assistance in opening her birthday presents (after all, "That tied up ribbon can be pretty tricky for a three year old!"). First and foremost, was the special gift she'd chosen and wrapped all by herself. To top it off, literally, Madeline also presented Susannah with her very own birthday crown, handmade today at pre-school. Naturally, it was yellow!

Throughout the day, one by one, each of Susannah's therapists appeared at our door bearing gifts and birthday cheer. It truly warms the soul to witness the soft spot they have already developed for this little girl. While she has only been home and in their care for five months, it is very apparent that she has touched their lives, in that special way Susannah is so famous for.

Since Susannah has never officially had a birthday party, she will actually have TWO celebrations this year. In addition to the festivities of today, we also have a second event planned for this weekend -- a "cousins party". Really the party is two fold. Given that a celebration for Susannah was long overdue and Madeline so desperately wanted a "double birthday party" with her sister this year (which was not feasible considering we must still limit Susannah's contact with the outside world), we decided on a small family (aka cousins) gathering, complete with two birthday cakes. Voila! A joint birthday party! Of course, Madeline will still have a "fairy party" with her friends the following weekend.

This is surely a week chalk full of celebrations, as such, we send great big birthday wishes out to two of our very special friends ... Molly will turn two the 19th and Julia will turn three on the 20th (both of their web site links are listed below). And, of course, who could forget, big sister Madeline's 5th birthday on April 22nd!

Sending you all much love,

Amy, Klane, Madeline and The Birthday Girl

Sunday, April 7th, 2002 ~ Day +545

Home Where She Belongs


Hi All!

I am happy to report that after another, relatively short inpatient stay, Susannah is again home where she belongs! As is so often the case, there are no definitive answers to the cause of her fever, but with her blood cultures still negative after 72 hours, her fever gone since early Thursday and her white count sky high (gotta love that G-CSF!), she received the green light on Saturday afternoon. She was more than ready to bust out of the hospital and, in fact, was going a bit stir crazy in her little room -- I never believed it possible, but I actually think Susannah was on Barney overload!

As luck would have it, the timing of Susannah's discharge coordinated perfectly with the surprise get-a-way Klane had planned for our 10th wedding anniversary. There was just enough time to unpack, repack and grab a quick shower, before he whisked me off to celebrate. Thankfully, June was waiting in the wings to take over and Susannah was so happy to be home that she didn't even miss us! We had a delightful "trip" downtown, dining out, taking in a show and best of all, spending some long overdue time together. And so, our April festivities began ... one down, three to go!

As for Miss Madeline, she spent a fun-filled extended weekend with Aunt Tami, Uncle Steve and the boys. She had such a blast that she never even missed the suitcase she'd left behind. Aunt Tami to the rescue; a trip to the PX took care of the toothbrush obstacle and as for clothes, Madeline attended mass this morning with her Godparents and cousins, happily wearing none other than Nicholas's pants and her auntie's underwear! Quite a coup, as I can barely get the girl out of a dress, but then again, I've always said my sister could sell ice to an Eskimo!

Much love,

Amy, Klane, Madeline and Susannah

Thursday, April 4th, 2002 ~ Day +542

Another Ride on the Merry-Go-Round!


Hello Again,

I should have known better. Really, I should have. Literally, less than an hour after posting yesterday's update, I found myself frantically packing Susannah's bags for yet another stay in the hospital. Oh my, let me back up a bit ...

We had just spent the morning at her weekly clinic appointment where Susannah received a clean bill of health. She looked fantastic, her labs had finally turned the corner and her vital signs were stable; her temperature was a stellar 98.5 degrees. Who could ask for more? As we strolled out of the hospital, she was giggling and waving bye-bye to everyone we passed.

The next thing I knew, Susannah was trying to take her afternoon nap in the middle of the living room floor! Hmmm, suddenly an alarm went off in my head. Something was definitely not right. Sure enough, the thermometer read 103.8, and that was merely under her arm (add the extra degree for axillary and she was pushin' 105 degrees!). She was shivering and lethargic and her heart rate was skyrocketing. Within two hours of our departure, we were headed back to the hospital, only this time, unfortunately, our destination was not just the clinic.

So, off we dashed ... to Children's ... to the BMT floor ... and here we wait. By now, even you all know the routine: Blood cultures, chest x-rays, big gun antibiotics, viral cultures, the works. No chances are ever taken and every avenue must be explored. All the while we patiently await the results and at the same time pray that none are found.

With Susannah's fever being so high and accompanied by chilling, not to mention it's sudden onset, the idea of an infection in her line quickly came to the forefront of everyone's mind. Of course, another viral process is certainly not out of the realm of possibility. The fevers point to a bacterial infection, yet the low counts tend to indicate viral. She is having drainage from her eyes and nose, as well as a cough, all of which could go either way. The coming days (and the results on her cultures) should provide more information and allow us to formulate a plan.

Despite, some lower than desirable oxygen saturations, Susannah's lungs remain clear. Through the night, with her sats hovering in the low nineties, a respiratory consult was ordered, but the RT actually thought she sounded great and passed on any intervention. She did, however, ready some blow by oxygen at the bedside, just to "ward off the demons". We are still awaiting the official results of her chest films, but are assuming no new is good news.

As if the nasty fever didn't wreak enough havoc on her little body, Susannah's newly stable blood counts also lost their footing in the crossfire. Her admission labs showed a dramatic decline in her counts. Specifically, her ANC dove down to a mere 153, yikes! Accordingly, a little G-CSF was added to her present IV cocktail and thankfully, by this morning her marrow had already shown some response.

Fortunately, Susannah's fever also broke during the night and there is no growth to date on her blood cultures. The question remains, did the fever subside due to the presence of antibiotics or would it have done so on it's own? Time will give us more answers, but the good news is, either way, her fever is down, thus increasing the likelihood that Susannah's line is salvageable. Right now, and for the foreseeable future, she really needs this PICC line; it is our best friend and replacing it would not be fun.

With the fever down, Susannah has returned to her sweet and smiley self, albeit noticeably less energetic than usual. Despite feeling a little under the weather, she still manages to charm the nurses and passers by as she knocks at the glass window on the door to her room. She is developing quite a name for herself around this place!

Please say a special prayer for Susannah as she endures yet another ride on this merry-go-round that her little body seems so fond of. We will do our best to keep you posted in a timely manner.

Love always,

Amy, Klane, Madeline and Susannah

Wednesday, April 3rd, 2002 ~ Day +541

The Latest and Greatest


Greetings,

Susannah's counts have finally begun to slowly creep upward and settle in, back to where they belong. Her white blood cell and absolute neutrophil counts have reached a nice equilibrium and her platelets and hemoglobin are following in their footsteps. As you can imagine, we are breathing a sigh of relief. Additionally, her weight has again stabilized and she has even managed to gain a few grams. As for the hair, well, it is still very thin, especially on either side, but seems to have stopped actively falling out. And, if we happen to catch her head in just the right light, we can even see some early signs of fuzz in the previously hairless patches. Thankfully, the top is quite long, which allows us to coiffure a discreet little "comb-over" and even sneak in a bow here and there!

Physically, Susannah is progressing by leaps and bounds. Still, by no means an average almost three year old, but she is getting stronger and making advancements each week. She has certainly not allowed her recent stints in the hospital to keep her down and has mastered the art of standing up in the middle of the room all by herself. Her latest trick is trying to escape. She sees us coming for her, wants to run, thinks she can, but as soon as she takes off, she falls forward and reverts to crawling as fast as her little body will carry her. Otherwise, walking has definitely become her preferred mode of transportation. She seems to have so much energy, but it is amazing to realize how little endurance she actually has. We are working on this by taking her on little walks around the yard. The short walk from the front door to the sidewalk has her panting. We have to keep a close eye on her respirations, so we are talking this very slowly. Her physical therapists are really working on getting Susannah to step over and up things and are constantly setting up little obstacle courses around the playroom. Susannah is too smart for them however, and instead just takes the easy way out and walks around things. Still, they press on because she really has no fear of falling and when she doesn't see something in front of her, she tends to trip and do a great big belly flop. She is making such strides in her gross motor skills that she is getting a little reprieve and we have decreased her PT from four times each week to three.

Klane hung a swing in the back yard, which is, of course, a huge hit. Susannah alternates between great big belly laughs and chants of "Weeeeeeeeeeee" the entire time she is in it. We have actually been a little adventurous lately and taken a few trips to the park! Naturally, precautions are taken to keep Susannah safe -- you know, those "normal" things like wiping off the swing, laying down a barrier before placing her on the slide and keeping her away from the sand (not to mention the other children)! This new exposure to the outside world has made Susannah crave it all the more. We are barraged daily with endless signs for "mask", "hat" and "shoes" which are all precursors to an outdoor excursion. She has even begun to vocalize "bye bye"; I am sure the timing of this is no coincidence. This little girl knows what she wants and has no qualms about requesting it!

Susannah is making tons of sounds these days. The tricky part, however, is that she only makes them when SHE feels like it! Of course, the favorite is "da da" and she is indeed referring to Klane as she chants it. We are still working on "ma ma" and with a lot of coaxing she will muster an "mmmmmm", followed by a high-pitched "ahhhhhhh". However, her most recent greeting for her mother is a very unladylike raspberry sound ... and yes, only mommy is privileged enough to receive such a special greeting. Nice! The interesting thing about Susannah's speech is that often she will vocalize more advanced words, totally out of the blue. The catch is that once she does it, she will not do it again! So, in the last month we have very distinctly heard, "red ball", "dolly", "apple" and a few others, but then as quickly as they came, they were gone. Hopefully this means she has a ton of words inside that little head, just waiting for the perfect moment to bust out! It is becoming very apparent to her therapists that Susannah's skills are very scattered, where she is above age level in some skills, but at or below in many others. So, perhaps these random words are a part of this phenomenon.

Thank you so very much for your unending support of our family and for continuing to keep Susannah in your thoughts and prayers. While the road she is traveling is certainly a long and winding one, it means the world to us to know you are right there beside her every step of the way.

Our love to all,

Amy, Klane, Madeline and Susannah

Sunday, March 31st, 2002 ~ Day +538

In her Easter bonnet ...


Good Evening Dear Ones,

May this note find you well and enjoying your Easter Sunday. Words cannot convey the depth of our sentiments at having our little family home to celebrate the miraculous day. Our blessings this year have been unique, but plentiful and we are ever so grateful.

This is the first Easter in two years that we have spent in San Diego, not to mention outside the confines of the BMT unit. Thinking back to last year at this time, it is stunning to realize just how far our sweet Susannah has come. Twelve months ago on this day she sat propped up and fully supported, staring past us with a distant gaze, virtually unresponsive. She was completely unaware of the world around her. Her Easter meal flowed into her body through a line in her chest and a tube down her nose. While Susannah's journey toward restored health is far from complete, she has made remarkable strides and continues to progress in every aspect of her being.

Not only did she join us at the table this Easter, she ate like a champ and even made several attempts to climb right out of her highchair! Susannah delighted in finding the not-so-hidden Easter eggs that the bunny left behind and tore into her basket with true toddler expertise. She spent the day walking from person to person, sharing her wonderful giggles and testing out the various laps around the house; Uncle Steve was definitely the man of the hour. Running a close second, were her cousins and big sister, whom she lovingly tailed everywhere, well almost anyway ... when the play moved to the backyard, Susannah stood guard at the patio door, nose pressed up against the glass, knocking politely to be let out!

This morning, two precious girls, adorned in pretty pink dresses, sat on the laps of Klane and myself, as we attended Easter Mass, together. Once again, the wonderful people of St. David's graciously prepared and reserved the "crying room" for Miss Susannah's special visit (an extra thank you to our neighbor, Gail, for the super duper vacuum job!). Though separated by windows, there was no mistaking the loving smiles that were shared between Susannah and her fellow parishioners. Our hearts were warmed by their spirited applause when Father Brent announced Susannah's presence, not to mention the glistening eyes of many as he laid his hands upon her for an Easter blessing. We may have had the little room to ourselves, but I am quite certain that Madeline was heard by all as she sang "Alleluia" at the top of her lungs (she'd been waiting for 40 whole days, after all).

Wishing you and yours a very blessed and joyous Easter season!

With love,

Amy, Klane, Madeline and, of course, Susannah

Monday, March 25th, 2002 ~ Day +532

Spring is in the Air


Happy Spring!

What a nice week! You know things have been pretty quiet when I can begin an update without immediately recounting the latest labs and such on Susannah.

Our visit with Kelsey and Gayle, two of the famous "college girls" from our apartment building in Minneapolis, was just great. We were able to explore nearly all of San Diego's attractions, some even I had never before seen. It just so happened that Klane was on vacation this last week, so he got to join in the fun too. For the most part, Susannah missed out on all of the tourist action, but was well entertained at home by her nurse, June, not to mention, the usual parade of therapists. We did manage to sneak her out for a quick stroll along the boardwalk and even found a nearly deserted carousel for her to ride on ... shhhh! You can imagine that we received a few stares from passers by as we went through the anti-bacterializing ritual, but it was well worth it -- we couldn't leave her out of all the excitement!

We really enjoyed spending time with our guests. Susannah was glad to perform her new walking and eating skills for the girls. They had spent many an hour trying to persuade her to do these things while we were still in Minnesota, so it was a treat for them to finally see the fruit of their labors. Without a doubt, Madeline was on cloud nine, showing them her dolls and playing beauty parlor and treasure hunt, just to name a few. Rest assured, she kept very close tabs on precisely whose turn it was to sit by her, read to her, ride next to her in the car and sleep in her "magic (trundle) bed". In fact, on their last night in town, Madeline realized that Kelsey and Gayle had each spent two nights in her room and there was still one night left. So, she walked out of her bedroom with an alarm clock and informed Gayle that when she heard the bell ring in the middle of the night, it meant that it was time for her to go switch beds with Kelsey; "That way you'll both get half a night and it will be even!" Needless to say, we were all very sad to see them go (although, not nearly as sad as they were to trade our sunny and 75 degree weather for the frozen tundra and chilling temps back home!).

As I mentioned, things have been relatively mundane on the medical front, so there is not much new information to report. Susannah did get a nice bump from last week's platelet transfusion and so far they have been hanging in, but slowing inching their way back down. If she stays true to her course, we anticipate her needing another batch of platelets in the next couple of days. Like her platelets, we keep waiting her white count to rev back up. She is consistently needing an extra boost of G-CSF every couple of days, give or take. In fairness, I must say that Susannah's counts are not THAT bad, but in light of last month's ordeal, we are taking the proactive approach and pumping her up any time her ANC droops below 1,500 or so. We have tested the waters a bit on this, but each time we deviate from the plan, we see the trend quickly head in the wrong direction, south. Dr. Davies feels she may just need these little "kick starts" once or twice weekly for the next several weeks, until she has completely cleared this latest infection. Hopefully now that spring is upon us, Susannah can bid farewell to winter's pesky viruses, once and for all.

On second thought, there actually was a bit of action in terms of health care. Twice this week we turned Susannah's changing table into a make shift procedure area. On two separate occasions the stitches that anchor the PICC line into Susannah's arm broke, first one side, then three days later, the other. Thank goodness for dear ole Dad! I guess all that ER call he takes does come in handy, after all. While I held her arms and June her legs, Klane re-secured Susannah's line with a couple of sutures (fear not, he was kind enough to numb her up with a little shot of lidocaine). She was a champ and things went very smoothly. Not only was the line able to be salvaged, but we also managed to avoid what would have been some pretty lengthy visits to the emergency room!

Now, for the big news! Madeline achieved a huge milestone this week; she can tie her shoes, all by herself! Hooray!!! She has become such the pro that she has even taken over the role of tying Susannah's shoes as well. With the shoe tying business under her belt, Madeline is now on mission to learn to jump rope and insists that she needs to wear nothing but "jumpers", in order to accomplish this feat (apparently, mere dresses don't help one jump). From what I understand, time telling is next on the agenda. She is becoming such a little lady.

Not to be outdone, Miss Susannah has reached a milestone of her own: Crying. Crying, you ask? Yes, you see, Susannah has taken up the art of crying when whenever she hears "NO". If anyone utters even the slightest inkling of the word, regardless of their tone, Susannah's lower lip begins to quiver, her eyes close tight and the drama begins. It all stops almost as quickly as it starts, but it is so pitiful to watch that it immediately turns us to mush (mind you, we have yet to see a tear!). It does, however, give us a little glimpse of the fact that, despite everything she has been through, Susannah is still a toddler at heart and can play us with the best of 'em. Since she really has his number, our littlest princess gets the most mileage out of her antics when her daddy is present.

As for the two of them together, the girls have recently rekindled their old past time of reading books in Susannah's crib. Madeline climbs right over the rail and the two will sit endlessly reading books with one another and snuggling with their special blankies. Of course, Madeline "reads" most of the stories and is often heard singing sweet songs to her little sis. Ever the helper, if she happens to be the first to hear the pre-dawn stirrings from Susannah's room, she makes a B-line for the crib. This morning, I was awakened to the familiar sound of beeping from Susannah's pulse oximeter, but was then surprised to hear Madeline's voice over the baby monitor, "Is it the button with the circle on it, Mommy?" Before I could speak, there was silence, as she had indeed found the right button to turn off the sat machine, which was beeping from all of Susannah's wiggling. I chuckled to myself as I then heard, "Whew, I did it. Sissy, your beeping was sure driving me crazy! Now, let’s get on with our stories."

Thanks so much for checking up on Susannah's progress. We really appreciate you sticking with us through yet another season and continuing to send all those positive thoughts our way. May the sunshine of springtime brighten your every day!

Warmest wishes,

Amy, Klane, Madeline and Susannah

Saturday, March 16th, 2002 ~ Day +523

Guess who's been listening?!


Hi There,

Just when it looked like Susannah was beginning to produce her own platelets and her white count appeared to be hanging steady, she decided to surprise us. Late last evening we received a call from Dr. Kadota saying her platelet count had dropped from 45,000 to 15,000. So, I'll give you one guess where we spent our Friday night? Apparently Susannah must have been listening in on my pre-weekend chat with Dr. Davies and overheard the words "counts on the rise" and "lab free weekend". Accompanying Susannah's low platelets, was a lower than desirable ANC, so we will continue to supplement her with intermittent doses of G-CSF until she can finally kick this virus once and for all.

Now, there is actually a case of her eavesdropping being helpful. Earlier in the week, with Susannah's weight dipping way down, despite her healthy eating habits of late, Dr. Davies suggested we make contact with a gastroenterologist who specializes in nutrition for an evaluation and some suggestions. Well, within the hour, little Susannah began eating like there was no tomorrow ... she was eating well before, but nothing like this! Even so, she will still see the GI doc later this month as she has been hovering at or below 12 kilos since back in the fall.

We have added a couple of new medications in the last several weeks. It seems the aerosolized pentamidime we transitioned Susannah to back in November when she was having such trouble with Bactrim, has the folks here at Children's a bit nervous. Since they are not used to it, they do not like it! The result: Instead of inhaling a medication once each month, Susannah now takes yet another oral medication, dapsone, every evening. Ugh! Upon conferring with Dr. Davies, we decided, despite the inconvenience, to throw 'em a bone and give in on this one. Fortunately, after calling Minnesota, the pharmacy here was able to prepare the new medication into a pre-mixed suspension. I spent the first week agonizing over evenly splitting the three millimeter pills in order to achieve a Susannah size dose, until finally they agreed to pursue the suspension. The tedious preparation of Susannah's 14+ medications already involves much crushing, mixing, dissolving, but I drew the line at splitting! Thank goodness for the wonderful compounding pharmacists in Minnesota, even 2000 miles away, they still look out for Susannah, and her parents too!

Susannah also started on a new cream, Elidel (pimecrolimus), for her chronic GVHD. She is the first child (what's new?!) that it has been prescribed to, as it only received FDA approval in the last couple of weeks. The dermatologist here was instrumental in getting it approved and Dr. Davies is really excited about it. It is a new topical form of the immunosuppressive, FK506. As you may recall, we did try another form of this drug, tacrolimus cream, last spring only to stop it because Susannah was achieving systemic levels in her blood (something that is supposed to be impossible); since she was already on cyclosporin, it was like double dosing and, among other things, would not have been friendly to her already fragile kidneys. Well, the new Elidel is also not supposed to be absorbed, but the difference is that, if by some fluke it happens to be, it does not suppress the immune system. Still, Dr. Davies is taking no chances, so we will be monitoring Susannah's creatnine (kidney function) twice each week and checking levels as warranted. We are hopeful that this new cream will provide Susannah much relief and allow us finally work on tapering her prednisone back down to a safer level. Oh yes, one last note on the med front -- the final decision on her thyroid medication (following our recent visit to the endocrinologist) was to decrease Susannah's Synthroid dose by half and check the appropriate labs in five weeks' time.

Our family is looking forward to an exciting week with two very special visitors from Minnesota. Shhh, please, please do NOT tell Susannah! When their original plane was snowed in by an unexpected storm on Thursday, the running joke was that Susannah had gotten wind of their impending arrival, but instead of landing herself in the hospital, this time she decided to just keep them in Minnesota! Hopefully, last night's brief stint on the unit for platelets, was enough to fool her into staying home for their visit.

Here's wishing everyone a Happy St. Patrick's Day. In closing, I offer some words of wisdom from Miss Madeline (who, by the way, thinks she is a leprechaun today). "Whatever you do, please just don't forget to wear green tomorrow, or else you might get pinched!"

Blessings,

Amy, Klane, Madeline and Susannah

Tuesday, March 12th, 2002 ~ Day +519

Short and Sweet


Hello All,

Susannah managed to sneak out of the hospital late last night! As you know, the usual stay for a fever is a minimum of 72 hours, but this time she was lucky enough to make her get-a-way after only two days. With her fever down since early Sunday morning, her blood cultures negative to date and her lungs sounding MUCH better, Dr. Roberts, Dr. Pian (the pulmonologist) and Dr. Davies all agreed that an early discharge was in order.

Now, of course, nothing is that simple. Susannah was sent packing with an additional 36 hours worth of IV antibiotics, until we're absolutely certain that there is no late breaking growth on her cultures. We'll continue to monitor her with daily blood draws; the promise to return immediately at any hint of an elevated temperature was understood. She will also attend clinic tomorrow.

While Susannah is still coughing at times, her lungs are much improved since Saturday evening -- the wheezing is gone, leaving only some slight crackles in the bases. In general, she is feeling well and hopefully, with a little time, will be as good as new. As often is the case, there are no definitive answers for the reason behind her fever, but a viral process is again highly suspect. Fortunately, we're almost through the viral season, because poor Susannah could sure use the break.

Well, our weekend didn't exactly go as planned, but we are just thankful that Susannah is feeling better and this admission was short and sweet. Believe it or not, two days is actually a record for our little lady!

Love,

Amy, Klane, Madeline and Susannah

Saturday, March 9th, 2002 ~ Day +516

Here we go again!


Hello from Room 117,

No sooner did I post the last update than did Susannah decide she had plans of her own. A toasty temp of 102.4 was her kind way of telling us that she wanted to spend the weekend at The Get Well Hotel. So we packed our bags, and alas, here we go again!

Susannah is on the usual cocktail of IV antibiotics, with a slight variance, substituting Cefapime for the old stand-by ceftazidime, in addition to vancomycin. With a bit of a cough present and somewhat junky sounding lungs, albuterol has also been added to the mix. We are still awaiting the results of her chest x-ray. Please say an extra prayer that it is clear and there are no signs of anything sinister.

Another platelet transfusion is in the works for tomorrow, as the combination of Tuesday’s washed platelet fiasco and today’s fever has really done a number on her count. With any luck, our friends at the blood bank will find it in their hearts to go the extra mile and round up some proper volume-reduced platelets for our little patient.

Fortunately, the dose of G-CSF Susannah received yesterday did indeed give her white count the boost it needed, sending her WBC and ANC from 3,000 and 1,100 all the way to 19,000 and 17,500, respectively. This is certainly the silver lining of the day's cloud cover. Maybe tomorrow we'll see a rainbow!

At present, Susannah is sleeping soundly, seemingly oblivious to all the extra work her body is doing to compensate for this nasty fever. Meanwhile, big sister Madeline is off at Auntie Brenda's for a "special sleep-over" and Daddy is sacked out down the hall in the ortho call room (hopefully some poor sleep deprived resident notices him there before plopping down onto the bed). Speaking of beds, I hear my lovely sleeper chair calling me ... sweet dreams everyone.

All our love,

Amy, Klane, Madeline and Susannah

Friday, March 8th, 2002 ~ Day +515

Her Lucky Number?


Hi Everybody,

WHEW, what a week! Two transfusions, coupled with two marathon clinic visits, two doctor's appointments and two missed appointments, find us relishing the idea of the upcoming weekend with two relaxing days around the house. Two seems to be the number of the week! While we do anticipate a quiet couple of days, we will still need to do daily blood draws and drop them off at the hospital. We had originally expected the next two days to be "lab free", but sweet Susannah had something else in mind. This morning's results showed another sudden drop in her white blood cell count. Not as drastic as the dip last week, but it was down nonetheless. Hopefully this time we are catching things early and a little G-CSF will send her on her way back up.

After a platelet count that continually hovered around the 20,000 level for close to a week, combined with some pretty bruised little shins, Dr. Davies felt a transfusion was warranted. The fact that Susannah was able to maintain roughly the same level, without dumping off, meant that she was actually producing her own platelets to a degree, but just wasn't doing so with the expediency we had hoped. So a little help was in order and Tuesday we were off to Children's Hospital for a transfusion. Our 11:30am arrival and 7:00pm departure from clinic should attest to the efficiency of getting the platelets into her! On top of that, someone from the blood bank decided it would be a good idea to "wash" her platelets in addition to volume-reducing them. As you might imagine, platelets are not particularly fond of being washed, so we didn't get much bang for our buck, and Susannah's platelet count has been dropping rapidly; she may well be in for another transfusion within a couple of days.

Today, Susannah received her first red cell transfusion on California soil. Like her platelet count, her hemoglobin had been tenuous for the last week. However, each time we were prepared to transfuse her, she'd rally out of nowhere and produce just enough red cells to squeak by. Well, after hanging out for two consecutive days at 7.7 and acting a bit punky, we finally bit the bullet and headed back to CHHC for the sixth time this week. The blood was just what she needed, as even an hour into the transfusion (despite a mega dose of Benadryl) she had her energy back. And her color too! Susannah had been anemic for so long, that we'd almost forgotten what a nice rosy color she has to her skin. The only thing we did not bargain for was another clinic visit lasting until 7:30pm! Okay, I admit it; I am spoiled and long for the efficiency of dear ole Minnesota.

On to the doctors' appointments. The above details should give you an idea of the goings-on at her weekly hematology appointment. We also saw the endocrinologist, Dr. Gottschalk, who, based on Susannah's most recent endocrine labs, had some recommendations for decreasing her thyroid medication. He's going to run things by Dr. Davies and then get back to us. He also evaluated Susannah's growth, or lack there of, over the last year and felt that the primary culprit was likely the prednisone regimen she is on as treatment for her chronic GVHD (of course, the Hurler Syndrome and radiation she received as prep for her second transplant certainly play a role as well). Dr. Gottschalk really felt that it would be helpful to get her steroid dose lowered to six milligrams every other day ... hmm, easier said than done!

In light of the unexpected transfusions, Susannah's dermatology and pulmonology visits were postponed for another week. Regarding ophthalmology, well to be honest, I completely blanked and forgot all about it! Now, before you scoff, we didn't actually no-show, but rather, I was supposed to have called the doctor's secretary on Tuesday to be worked in for a Thursday appointment. Let's just say, I finally remembered that plan on Friday afternoon. Oops!!! I am losing my touch -- I guess my hard-drive is just too full! Obviously, she will have her eyes evaluated next Thursday instead.

All in all Susannah is hanging in there. Since her discharge from the hospital last week, she has yet to get her appetite back. She is still eating, just not nearly the volume of recent months and her weight is reflecting it. She has dropped about a pound and a half in the last two weeks, which may not sound like a lot, but considering she only weighs in at around 27 pounds, it does make a big difference. She also has the beginnings of a cough (although it is intermittent at best), and a slightly runny nose, both of which we will surely be keeping an eye on. In spite of everything, Susannah continues to be a sweet, giggly and social little girl, constantly bringing joy and smiles to those around her. We are always hearing her therapists and nurses chuckling about her silly antics, adorable dance moves and loveable kisses. Of course, WE know all about those!

On the social side of life, tonight was a very special event for Madeline. As most of you are aware, Miss Madeline is quite the Peter Pan aficionado and after weeks of anticipation, the day finally arrived for our trip to the theatre to see "Return to Neverland", the Peter Pan sequel. This was such a huge deal in our house that both Klane and I really wanted to be able take Madeline to see the movie, together. Susannah's unexpected red cell transfusion today nearly foiled our second attempt in as many weeks (the first being Susannah's admission two weekends ago) and we just couldn't bear the thought of letting her down again. June to the rescue! June was kind enough to come to the hospital/ clinic and sit with Susannah, while we snuck out to a quick dinner (none other than Madeline's favorite, Chinese buffet) and the movie. Madeline, in all her glory, refused to leave the theatre until the very last credit rolled up the screen!

Susannah sends her wonderful kisses out to each and every one of you (and a sprinkle of fairy dust from her sissy too!).

Much love,

Amy, Klane, Madeline and Susannah

Monday, March 4th, 2002 ~ Day +511

Moving Right Along ...


Good Morning!

Home life certainly agrees with Susannah -- she not only remains fever free, but her counts are incredible and have decided to rebound with a vengeance! After my last update, her total white count continued to climb all the way up to 22,000 and her absolute neutrophil count to 16,500. We came home with a week's worth of G-CSF and, needless to say, we have yet to administer it. Dr. Davies is extremely pleased that Susannah's bone marrow is able to mount such a terrific response to stress.

With any luck, Susannah's platelets and red cells will follow the lead of her white count, so we can head off any transfusions. Her hemoglobin is certainly testing the limits, hanging out at a nerve wracking 8.0 and her platelets are well below her usual transfusion parameter of 40,000. However, with the amazing response she managed to mount in the white blood cell area, Dr. Davies really feels if we push things a bit, Susannah may just manage to turn things around on her own. Of course, if she becomes at all symptomatic (bruising, bleeding, pale or lethargic) we would head for the hospital immediately and transfuse her with the appropriate blood product. Obviously, we are back to drawing daily labs to follow her blood counts extra closely.

We have also resumed the val-gancyclovir. Last week, when Susannah's counts were not responding at all to the G-CSF, Dr. Davies felt it would be wise to try holding her Val-Cyte, since the drug is technically a chemotherapeutic and could have potentially been a catalyst in suppressing her counts. Her eyes will be checked this week by the retina doc to be safe, but since CMV retinitis is relatively slow in reactivating, Dr. Davies figured it was worth the gamble to hold the drug, if it could help Susannah's counts rebound. And, rebound did they ever! We will never know for sure whether Susannah's marrow finally just revved up on it's own, decided to respond to the mega doses of G-CSF or appreciated the reprieve from the val-gancyclovir, but things certainly turned around only two days after we stopped the medication.

On the agenda for this week are a whole slew of doctor's appointments. Aside from the usual hematology, dermatology and ophthalmology re-checks, Susannah will also visit the pulmonologist and endocriniolgist. While the specialists, who know her so well, will continue to follow her in Minnesota, it seemed wise to at least make contact with the doctors here, as well. On that note, plans are underway for a return visit to Minnesota at the end of April/ beginning of May, for Susannah's 18 month post BMT check-up. We will be sure to keep you posted as we iron out the details.

Speaking of Minnesota, despite the chaos of the last week, we did manage to sneak in a wonderful visit with Joan. Yes, Joan, from St. Paul, came all the way to California just to visit Madeline! Madeline was in her glory, soaking up every minute and reveling in Joan's affections, as she had been counting down the days for an entire month! She even insisted on baking a cake "all by herself" to welcome Joan. Madeline treated her guest to visits to her "San Diego ballet class", her "San Diego park", her "San Diego church" and unfortunately, even her sister's "San Diego work (hospital)". Next time we are expecting a mid-west visitor, we are going to be sure to keep the news from Susannah, as apparently she thinks the words hospital and Minnesota go hand in hand. We so wish we could have all been home and together during Joan's trip, but still we managed a very nice visit in spite of everything.

Otherwise, things on our end remain quiet and that's how we like 'em, quiet and uneventful! Thanks a million for continuing to check in with us and also for signing the guest book ... your entries really add smiles to our day! We hope you all had an enjoyable weekend.

We love you,

Amy, Klane, Madeline and Susannah

Thursday, February 28th, 2002 ~ Day +507

Back Home, Safe and Sound


Hi There!

It seems Susannah must have heard everyone's chants for cell growth, because grow them she did!!! After stagnating in dangerous territory for nearly a week, overnight, Susannah's counts went through the roof! Not only did she grow cells, but she did so in true Susannah style.

Things had been quite tense these last several days as Susannah's ANC continually hovered in the 200 range, showing no real signs of improvement. Of course, we were in daily contact with Dr. Davies, who gave several recommendations to help Susannah's marrow recover more quickly, but for a while even these didn't appear to be doing the trick. So you can imagine my sheer relief when I awoke this morning to the news that Susannah's neutrophil count was a staggering 4,100, and her total white count was 8,300! No one could believe their eyes; in fact, the doctor here even had the labs redrawn just to be certain the blood sample was indeed Susannah's! Well, the second set of counts proved to be even more impressive, as in just five hours time, her ANC had jumped to 5,900 and her WBC to 13,300. Dr. Roberts, the current the unit attending, said he'd never quite seen anything like it. Incredible is the only way to describe it!

With such phenomenal counts, Drs. Davies and Roberts felt there was absolutely no reason to keep Susannah in the hospital, for even another day. As such, the IV antibiotics were discontinued, home nursing arrangements were made, and she was discharged early this evening and is now sleeping soundly in her own crib. I can't tell you how wonderful it is to have her home again. It feels as if a lead weight has been lifted off of our shoulders ... I think we will all sleep a little easier tonight.

Thank you, thank you, thank you for all of your extra thoughts and prayers -- they really seem to be working. It has been a draining six days, so I think I will follow Susannah's lead and turn in as well. Nighty-nite!

Love to all,

Amy, Klane, Madeline and Susannah

Monday, February 25th, 2002 ~ Day +504

Grow Cells Grow!!!


Hello All,

As you may already be aware, Susannah was admitted to the hospital on Friday afternoon for a fever and neutropenia. After teetering on the brink at 100.4 for two days, she finally surpassed the magical fever mark and spiked an axillary temperature to 101.3. Coupled with this, her labs from Friday morning showed a neutrophil count of 435, yikes! So, we packed our bags and headed for Children's Hospital.

The good news is that she has been afebrile since Saturday and all blood cultures and preliminary viral screens have been negative. After a few days of feeling punky, she is no longer a "snuggle bug", but rather back up and scooting around the room. It's amazing how much more difficult it is keeping her hooked up to an IV pole is, now that she can walk!

The not so great news is that her blood counts have not yet recovered. In fact, they are all around rotten. Her platelet count was so low today that she was actually transfused with random donor platelets, as opposed to waiting for her cross-matched unit to arrive. Fortunately, she did receive a very respectable bump, so she is out of the danger zone in that area. As for her hemoglobin, it is also trending downward, but this could very well be attributed to the numerous blood draws she has received since being admitted.

The most worrisome piece of the puzzle, however, is Susannah's progressively lower white blood cell counts. Despite maximum dosing and double dosing of G-CSF, Susannah's absolute neutrophil count continues to fall. Her total white count has remained steady at ~1400, but her ANC has gotten lower and lower with each draw, tonight's being a very disturbing 180. The consensus is that she is reacting to a viral process and will eventually come around, but even so, it is quite frightening to witness and some reassurance from her labs would be nice.

Being in the hospital here in San Diego has been, well, it has been an adventure. Thankfully, the two traveling nurses that are here from Minnesota have been on duty, which has made things much smoother and really helped ease the transition. After becoming very accustomed to care at a single center, it has been challenging adapting to a new hospital environment, especially considering we are a little rusty with inpatient life these days. We are ever so grateful for the timely presence of Casey and Jill!

Obviously Amy and I have both been on hospital duty, alternating night shifts and relieving one another as often as possible. Amid the chaos, Madeline has been graciously entertained by her cousins from both sides of the family. We've learned from past experience that keeping her busy as possible is what works best for Madeline in this situation. As always, her visits to Susannah's hospital room bring a smile to everyone's face, most importantly, Susannah's. Madeline's reaction to her sister's latest admission was precious ... upon learning of Susannah's return to "work", Madeline said, "Ohhhhh noooo, not again!" Realizing she was under the impression that Susannah had returned to Minnesota without her, we explained that she was merely down the street at Daddy's work. At that, Madeline dramatically swept her hand across her forehead and exclaimed, "Wheeeeeew!!! When can I go visit her?" It was priceless.

It has been an exhausting couple of days, but we are prepared to do what it takes to see Susannah through this latest hurdle. It would be untruthful to say that we are not nervous and concerned, but we also know that Susannah is one very strong little lady who knows how to avert adversity. Please say some extra prayers that Susannah's counts recover quickly -- grow cells grow!

With love,

Klane, Amy, Madeline and Susannah

Sunday, February 24th, 2002 ~ Day +503

Admitted Again ...


Hello Everyone,

Just a brief update to let you know that Susannah was admitted to the hospital on Friday afternoon, for fever/ neutropenia. She is on the BMT unit, receiving IV antibiotics, G-CSF for her low counts and is being monitored very closely, as we await the results of her blood and viral cultures.

While she is not quite herself, Susannah is in good spirits. Nonetheless, we can never be too careful where our little girl is concerned, so a few extra prayers certainly wouldn't hurt. Thank you and we promise to give a full update soon.

Love,

Klane, Amy and Girls

Thursday, February 14th, 2002 ~ Day +493

Sisters are Special


Happy Valentine's Day!

On this day when love abounds, I find it only fitting to reflect on the incredible bond shared between Susannah and Madeline ... a love so unconditional that it melts your heart. While Susannah is in complete awe of her elder sibling and this admiration is unmistakable, we continuously marvel at the tenderness and compassion Madeline exhibits toward her sister. She adores Susannah unabashedly and without pretense. I want to take a moment to share with you all just a few of the wonderful sisterly moments that we enjoy on a daily basis.

One of Susannah's latest pastimes is walking from room to room "investigating" anything and everything she shouldn't. Once inside, she will close the door, sit right in front of it and then knock on it to be let back out. Well, you might imagine, it is a bit tricky to get the door open while she is sitting there, so we often keep the doors to the bedrooms closed. One Sunday, while trying to help keep Susannah out of mischief, Madeline inadvertently closed our bedroom door on Susannah's pinky finger. Susannah shrieked, but recovered pretty quickly. Madeline, on the other hand, began to sob hysterically. When we asked her what was wrong, she told us she was worried that Susannah would be angry with her and "When Sissy is four, she might not want to play with me and I will be soooo sad." Later, when the two of them were alone in the kitchen together, Klane overheard Madeline asking Susannah, "You will forgive me, right Susannah? It really was just an accident, I promise." Of course, Susannah responded with her signature hand covering the mouth giggle and all was forgotten!

Madeline is so protective of Susannah, always looking out for her baby sister's best interest, doting on her and catering to her every whim. She never needs to be reminded to wash her hands, leave her shoes outside and all around thrives on being Susannah's chief advocate. In fact, Madeline really takes pride in her new self proclaimed role as the "hand-washing police". Last week when a neighbor stepped into our foyer to hand me some paperwork, Madeline came barreling around the corner, hands on her hips, looked straight into her eyes and said, "You didn't wash your hands, did you?" When our neighbor innocently mentioned that she was just dropping something off and not planning on touching anything, Madeline told her, "You mustn't come inside the house without washing those hands, for my sister, you know."

Recently, when talk turned to the subject of Madeline's upcoming birthday in April (yes, she is her mother, always planning ahead), as she was naming all of her friends for her guest list, she stopped suddenly and said, "Wait, is that enough friends?" Not understanding, we asked what she meant and she replied, "I want to be sure there are only enough friends so it's safe for Susannah to come to my party, she really has to come, I want her there the most!"

Just yesterday while the girls and I were laying down together catching a quick snuggle, Susannah began to scratch at her ear. As you all know the poor little thing always seems to be itching one part of her body or another. Well, in the sweetest voice I've ever heard, Madeline took Susannah's hand and began to rub it saying, "It's okay, Sissy, I'll scratch for you -- listen to my words Susannah, you don't need to worry about that itchy ear, Big Sister will take care of it for you."

Of course, being the little sister, Susannah does manage to do her fair share of pestering and interrupting of "big girl activities", but even then Madeline takes things with a grain of salt. At times we do hear, "Sissssssssssy", but more often than not Madeline will just redirect Susannah to one of her own toys, all the while explaining, "These are fragile toys Susannah, you see I am four years old, so I get to play with them, but don't worry, when you are four or six or seven, I will save them for you."

The daily giggles shared between our girls truly make everything worthwhile. We feel so very blessed to have two wonderful daughters to love and who love each other so completely. We send our love and great big hugs to each and every one of you, far and near, and wish you the happiest Valentine's Day ever!

XOXO,

Amy, Klane, Madeline and Susannah

Wednesday, January 30th, 2002 ~ Day +478

An Update from Head to Toe


Hi There!

I am happy to report that Susannah has responded appropriately to the G-CSF and her white count has rebounded. Now we will just watch and wait to make sure they actually stay up on their own. She narrowly escaped a platelet transfusion last week, when her platelet count plummeted. Fortunately, they too are on the rise, slowly but surely. Her hemoglobin is now in the hot seat, showing a slight downward trend, but we are hopeful that this is just a response to the increased blood draws. On that note, we continue to draw labs daily, to be certain things are moving in the right direction with no surprises.

On other fronts, due to Susannah's increased itching and skin breakdown of late, we have been unable to proceed with the tapering of her oral steroids which treat the chronic GVHD. At this rate there is no telling how long we will have to keep her on the immunosuppressives, perhaps another year or more; it's just too soon to make predictions. She needs to be stable at a much lower level of prednisone for some time before we can even begin a wean. Generally, the treatment for chronic graft versus host disease is a nine month course, but this is Susannah, after all -- nine months would have been next month!

We continue to see daily hair loss, much like Susannah experienced last May when her chronic GVHD was first diagnosed. Although somewhat of a minor issue in the scheme of things, it does tug at my heart strings to see those beautiful thick and curly locks looking patchy and thin once again. Hopefully, as her skin settles down her hair loss will follow suit.

Curiously, despite Susannah's increasing appetite and incredibly balanced diet these days, we have begun to see subtle a decline in her weight. With all she is eating, it seems she should be really putting on the pounds ... hmmm, another strange Susannah phenomenon. Dr. Davies had us check a few additional labs last week to see if that would shed any light on the issue. For now, we'll just continue to watch her closely and see where things settle out. As an added measure, we have also reintroduced a high calorie juice supplement into her diet.

In spite of everything going on with her medically, Susannah continues to be a happy giggly little lady who is becoming more and more aware of her surroundings. With all of the progress she is making in her fine and gross motor skills, her speech has taken somewhat of a backseat these last couple of months. She has however, mastered a couple new signs ("finished" and "shoe"); also, she will now answer us by saying "yes", well actually, it is more of an "sssssssss", but still, it gets her point across. Supposedly, she will actually verbalize "want" (or "waaaaa") for one of her speech therapists, but so far it seems to be their little secret and Susannah is content keeping the rest of us out of the loop! She did have us all cheering at dinner last week, when she clearly told us who the most important person in her life was by shouting, "Mad" at the top of her lungs. Just incase we were unsure of her meaning, she then pointed toward her big sister, began clapping like crazy and shouted it again. As you might imagine, this made our day!

Thanks so much for continuing to keep up with Susannah and our family.

All our love,

Amy, Klane, Madeline and Susannah

Friday, January 25th, 2002 ~ Day +473

A Tribute ...


Good Evening,

Since my last update, we have seen marked improvement in Susannah's blood counts, which, of course, has brought about a huge sense of relief. It seems the offending virus, has passed and things are on the upswing. Although Susannah is doing better, it has been a heartbreaking couple of days.

Our dear friend Alexis moved to heaven yesterday afternoon. She was such a special little girl who had endured so very much in her short life. Susannah and I first met she and her parents, Jody and Tom, more than a year ago on their initial trip to Minnesota and have since shared in every step of their journey. We hold close to our hearts many wonderful memories and feel blessed by the indelible mark their friendship has made on our lives.

While we will forever miss Lexy's sweet smile, we find some peace in knowing that she is now free from the ravages of Hurler Syndrome and bone marrow transplant ... dancing and singing with all of our other precious angel friends, already waiting up there to greet her. Our love and prayers for continued strength and courage go out to her mommy, daddy, sister, brother and the entire Wiggins family. Thank you all for remembering them as well.

We will give a more thorough update on Susannah in the coming days.

Love to all,

Amy, Klane and Girls

Sunday, January 20th, 2002 ~ Day +468

She keeps us on our toes!


Hello All,

It seems little Susannah thought life at home was becoming a bit too mundane, so she decided to pull a few tricks out her hat to keep us on our toes!

In the last week, Susannah's blood counts took a dramatic dip. After three months of rock solid white blood cell and absolute neutrophil counts in the 7000 and 3000 range, respectively, her ANC plummeted to a low 313. Unfortunately, Susannah's previously stable platelet count has also followed suit.

While things here at Children's are handled quite a bit differently than in Minnesota, after a few phone calls to Dr. Davies, "we had a plan" -- would you have expected anything less?! We immediately began infusions of G-CSF (a white blood cell stimulant) and of course, daily lab draws to follow her counts. Thankfully, as of now, her platelets remain at an acceptable, yet lower, level so as not to require intervention.

While this is all likely due to the fact that we have had a virus running around our house in the last month, it is still concerning. The lower her white count, the more susceptible Susannah becomes to infection. This scenario is a bit nerve wracking as only twice before have Susannah's counts reached such a low and both times were immediately following her actual transplants when she was still confined to an isolation room. Needless to say, our "quarantine" has been stepped up a notch in the last week, but we are still in our own home continuing with everyday life.

Despite her low counts, Susannah's runny nose has remained just that. In the last few days she has developed a slight cough, but so far it remains benign. Her lungs sound clear and her oxygen saturations remain favorable with only a few dips here and there. Of course, we will continue keep a close eye on things. As for Madeline and I, we are now feeling much better and are grateful to be able snuggle Susannah again.

We had a special treat at clinic on Wednesday when we got a visit from two of the nurses who cared for Susannah when she was on the step-down unit this past summer. They are spending the year as traveling nurses here in San Diego. I can't tell you how nice it was to see some familiar faces! We also had the chance to chat with three of our favorite nurses from 4-A in the last week, Kristy, "Missy" and Mary. It was great to hear from them and catch up with their lives. We miss them tons, as well as, the many others who became like part of our family.

The recent happenings around here, once again, remind us that Susannah has a long road ahead of her and will undoubtedly blaze new trails the entire way. We are just so very thankful to have such a loyal and loving group of people who continue to keep her in their thoughts and prayers. Also, as you have so often done for Susannah, we ask that you say a prayer for our dear little friend, Alexis ... she remains in Minnesota nearly a year post-transplant and is now facing yet another major hurdle. I know she and her mommy (as well as, her daddy, sister and brother back at home) will gain much strength from the knowledge that they are being held close during this trying time.

Love Always,

Amy, Klane, Madeline and Susannah

Saturday, January 12th, 2002 ~ Day +460

Ringing in the New Year


Happy New Year!

The New Year brings us much progress and good news. In general, Susannah is quickly expanding her horizons on all fronts! She's walking, eating, communicating and playing in progressively more advanced ways each day. This is in no small part due to the everyday grind of advocation which her mother must continuously endure. Although we are absolutely delighted to have everyone home, obtaining what we feel is the best care for Susannah has been much more difficult here than in Minnesota. Hassles with the clinic, pharmacy, home care, and occasionally therapy, occur almost daily. Additionally, things have been pretty hectic with all of the extra people around the house (anywhere from three to six professionals visit on any given day). I know Amy and Susannah sometimes miss their daily Minnesota routine; it was relatively predictable and the faces were more than familiar. All in all, being back in San Diego is everything we could have hoped for and more, but it has also been a bit psychologically draining, especially with the extremely limited number of outlets. Nonetheless, Amy and the girls press on!

Life with June (aka Mary Poppins) continues to bring developmental improvements for Susannah on a daily basis. Most notable of these is Susannah's growing appetite. She is not only eating more food in general, but her taste buds are going crazy. Her range of food variety has expanded exponentially -- she is eating everything from Cheerios to grilled eggplant, as well as meat, fruits and numerous dairy products, all with great delight! More impressively, Susannah will now feed herself with a spoon or fork, with only a little assistance in loading. These developments are certainly due in large part to June's infinite patience. They'll sit at the high chair for 2-3 hours in a day and enjoy every minute. She loves to give Susannah interesting little snacks, and now Susannah loves to eat them! June's timely entrance into our lives has truly been a gift from God! Having that extra set of hands around a few days each week to help with meds, line care, blood draws, not to mention allowing Amy to run errands and simply leave the house, has made such a difference. June not only nurses Susannah, but her parents as well. She all but insists that Amy and I take an evening for ourselves, every couple of weeks, in which she kindly looks after both girls.

While Susannah has made great strides with her eating and gross motor skills since returning to California, she seems to have had a bit of a setback in the language department. Prior to leaving Minneapolis, she was using a few words and making several sounds. Now, however, she has really clamed up. It's not that she has forgotten the things she knows, she just isn't willing to vocalize them. For instance, when asked what a sheep says, Susannah used to reply with a boisterous "Baaaaa", but now the same question yields only the appropriate lip movements, without any sound. She will gladly mimic all kinds of sounds and even some words, the catch, however, is that little or no noise actually comes out! Her adorable and friendly "Hiiiiii" greeting that everyone is so fond of has essentially gone by the wayside; unless of course, we are lucky enough to be watching at exactly the right moment to catch her tiny lips silently part. We have been told that when children are making rapid gains in other developmental areas (i.e. learning to walk), their speech tends to take a back seat, for a period. Another contributing factor could be the change in therapists. While all of Susannah's new therapists are excellent and very well qualified, we expect that the transition from Jane, Jim and Rebecca has really thrown her for a loop. These three had become such a huge part of her life (heck, she spent more time with them than me these last months) that she must notice their absence. Her life in Minnesota is likely the only one she remembers, so in a sense, she has been uprooted and may just miss her friends (I know her mother does!). This would be tough on anyone, let alone a two year old. Hopefully with a little time, Susannah will adapt and come out of her shell. In the meantime, I am happy to report that she has exchanged that unladylike grunting she acquired when she first came home, for a very refined pointing when she wants something, which is certainly a step in the right direction.

Susannah had several doctor's appointments over the holidays. She was seen by her new vitro-retinal surgeon (the opthalmologist, or even better yet, the retina doc), who reassured us that her CMV retinitis remains inactive. She seems to be responding well to the oral med, Val-gancyclovir. This is likely due to the fact that her gancyclovir levels, which we have now checked twice, are therapeutic. Dr. Freeman felt confident enough to change her monthly visit to every other month. Let me tell you, this is a wonderful thing because as nice as he was, Susannah was none too fond of him, his staff, his drops or his tools and machines. In fact, the minute she caught wind of the dilating drops, it was all over! Dr. Freeman was kind enough to try to examine her visually, but in the end, had to resort to the usual "technique". This is not a very pleasant exam for anyone, not Susannah, Amy or the doctor. It basically involves four people pinning her down, while the doctor pries her eyes apart with a wire speculum and looks inside. It's pretty difficult to shake Susannah, so you know the ordeal must be awful. Needless to say, we are more than happy to give her a month's reprieve.

Her orthopedic appointment was far less eventful. Susannah did flinch for a moment when she caught sight of the x-ray machine, but with a little coaxing to re-direct her, she quickly regrouped, and was even willing to perform her new walking skills for Dr. Wenger. Orthopedically, her hips and spine are status quo (surgery at some point down the line), but we'll need to keep a close eye on her knees. For now, however, we will just watch and wait.

Susannah's weekly hematology visits have typically been uneventful. Her labs have been solid, with not much changing, except her skin. The chronic GVHD has flared a bit, with worsening itching and thinning of her hair. Dr. Davies (who is keeping a close eye on things via Aunt Tami's digital camera) upped the potency and frequency of her steroid creams which really seems to have helped. Susannah also had a visit to the dermatologist, Dr. Eichenfeld, who was quite knowledgeable about GVHD, and gave some good recommendations for her skin and some different shampoos to try for her scalp. As I mentioned, Susannah seems to be responding well on the skin front, but we continue to find fine locks of her hair everywhere.

As for Madeline, she enjoyed the holiday break from preschool. Unfortunately, her "vacation" was extended by a week because she has been sick with an upper respiratory virus and an ear infection (poor Madeline now has to endure daily doses of medications like her sister). In turn, Amy became sick, and thus both have been trying to keep away from Susannah, which is pretty much impossible. So far, good hand washing, mask wearing and extra help from Grandma Dona, have kept things at bay, resulting in only a runny nose for Susannah. We'll keep our fingers crossed, as no one is looking forward to an admission to the hospital for a fever.

We welcomed the New Year quietly. Our New Year's Eve celebration was pretty low key, but certainly a welcome change from last year when Susannah went on the ventilator. I took emergency room call, while Madeline, Susannah and Mommy celebrated on their own. Well actually, Susannah slept, but Madeline stayed up late and watched the Times Square countdown, and at "midnight" (nine o'clock our time), celebrated by clanging pots and pans outside and toasting with a little "champagne" a la Martinelli's. I managed to arrive home about 30 seconds after midnight, to kiss my bride ... we do party hard here at the White house!

We look forward to 2002 being a year of recuperation and growth for our family. Amy and I are continually grateful for all of the love and kindness that we are blessed with from our amazing family and friends. In turn, we wish each and every one of you a year of health, love and happiness!

Best wishes and all of our love,

Klane, Amy, Madeline and Susannah

Wednesday, December 26th, 2001 ~ Day +443

On the 2nd Day of Christmas ...


Merry Twelve Days of Christmas to All!

We hope you have been enjoying the magic of this blessed season as much as we have. As you might imagine, this year the holiday has taken on added meaning for our family.

The biggest treat by far was Christmas Eve; the four of us were able to attend church at St. David's, together! Yes, Susannah too ... Father Brent made arrangements for the "crying room" to be reserved exclusively for our family on this special night, so Susannah, Klane and myself were able to enjoy the service and watch Madeline in her role as an angel. Of course, Madeline looked beautiful in her halo and was front row center, belting out the words to every song! She was totally enamored by Baby Jesus and is already trying to finagle herself into the roll of The "Firgint" Mary for next year's pageant. It was such a joy for us to be together and it was so nice for all of the parishioners to once again see (albeit through the glass window) the miracle child they have prayed for all these months.

Afterward, we spent a quiet evening at home with my parents and Grandma Dona opening gifts by the fire and sharing Christmas cheer. We had a great time and the girls were beside themselves with excitement. Susannah, sporting her first pair of party shoes, was all over the place walking from person to person and back again. The wrapping paper and ribbons really caught her fancy, but most of all, she fell in love with Grammy's lap -- perhaps it had something to so with the yummy artichoke dip she kept sneaking her!

Christmas morning came bright and early. Why is it the older the girls get, the earlier it comes?! We must have been really nice this year because Santa was very good to us all. Madeline insists it was the glitter and oats she sprinkled on the lawn to lure the reindeer, not to mention the dozen pink cookies and milk she set out on the hearth.

Aunt Tami and Uncle Steve were kind enough to completely "Susannah-tize" their home (artificial tree and all!) so that we could share Christmas dinner with my two sisters' families, as well as, Grammy and Poppy. Thankfully, everyone remained healthy and sniffle free, thus making the gathering possible for Susannah. This is the first time in 13 years that we have all lived on the same coast. It was wonderful to be able to celebrate this extra special Christmas together.

It is incredible to think about everything that has transpired since last year at this time, not to mention since Susannah's diagnosis two Decembers ago. Reflecting back, we find ourselves constantly counting the blessings that have been bestowed upon us. At the same time, we hold close to our hearts our many friends whose lives have been forever changed and those who are struggling during this holiday season.

As we celebrate the miracle of Christ's birth, we also thank God for our sweet Susannah whose strength and courage are an inspiration. May the joy she brings continue to unfold in our lives and the lives of everyone she has touched.

Merry Christmas,

Amy, Klane, Madeline and, of course, Susannah

Friday, December 14th, 2001 ~ Day +431

She's on the move!!!


Hi Everyone!

I know it's rather unprecedented for us to update twice in one week, but we have BIG news to share. Susannah has found her legs! Yes, it's official, the little miss is now walking! Yesterday morning, amid chants of "Go Sissy go!", she surprised us all and just walked across the room, as if she'd been at it for years. She has come close several times in the past, only to be set-back by illness or transplants, but this is the first time Susannah has ever crossed over into the world of toddling. We are so very proud of her.

Since returning home, Susannah's taste buds have really blossomed. Naturally, she has developed a love for Mexican food, especially sticking her fingers into guacamole and then licking it off. The other day, she devoured a taco and refried beans and even ate two tablespoons of salsa, straight! Along those lines, she is working on using a spoon and coming along well. Now, if only we could keep her from throwing that cup!

Madeline, who by the way takes sole credit for her sister's newfound walking, continues to be the model elder sibling when it comes to looking out for Susannah. After spending a busy, napless day with friends, she fell asleep in the car on the drive home; as I carried her into bed, she sleepily said, "Wait, Daddy, I have to wash my hands, we need to keep Sissy well!" Madeline is still working hard on Susannah's vocabulary and is always more than willing to "assist" the various therapists in their duties.

Just a little note to all of our Minnesota nurses, especially those who have been calling in a frenzy upon learning of our new nurse: Fear not friends, even though we are now blessed with "Mary Poppins", you can never be replaced! Although, June did want us to remind you that possession is nine-tenths of the law, so you better come visit soon! All kidding aside, while we are obviously elated to be home and together, not a day goes by that we don't miss ALL of our Minnesota friends.

We hope you are enjoying the holiday season. We have already received the greatest gift of all!

With love and warm wishes,

Klane, Amy, Madeline and Susannah

Monday, December 10th, 2001 ~ Day +427

Home for the Holidays


Seasons Greetings!

Susannah took two steps this week!!! We are now on pins and needles waiting for an encore performance, since Lee, her new PT, was the only one to witness the big moment. She has also stood on her own for several seconds in the middle of the room on numerous occasions. Susannah is indeed much more interested in "walking" these days than any other mode of transportation. She will grab for your finger and walk around the whole house barely holding on, screeching "waak, waak". If by chance there are no fingers available, she will push anything around to get where she pleases -- I am not just talking about toys, but also chairs and even the coffee table. It's just amazing to watch her go, go, go. She has come so far since May, when she wasn't even able to hold her head up on her own.

On the medical front, Susannah's labs continue to be favorable and very stable. She is, however, contending with a good deal of dry and flaky skin these days, reminiscent of previous flares in her chronic GVHD. As such, we have increased her regimen of potions and lotions to see if they help calm things down. Dr. Davies wants us to watch and wait, but feels we may be holding off on the steroid taper which was due to drop down a bit this week. We are going to borrow a digital camera and e-mail off some photos to Minnesota, so she can "see" things in person.

Lately Susannah is totally enamored with her daddy. I think she has realized just what she was missing these last months. From the minute Klane walks in the door she is crawling all over him, literally. Susannah has quickly figured out that he is a morning person and has decided to follow in his footsteps. She is now Klane's personal alarm clock and the two spend the wee hours of the morning reading books and drinking their respective brews together (his, very strong coffee and hers, "beefed up" milk). Pretty perceptive of her to find a way to get her daddy all to herself!

I was lucky enough to sneak off for a little welcome home weekend with friends last Saturday and Sunday. It was such a treat! We had a great time dining out, shopping and most of all relaxing and catching up! Big thanks to all who made it possible, especially the dads who between them watched a total of 12 little ones! It goes without saying that Klane did a wonderful job looking after both girls on his own.

This weekend was Madeline's turn as she joined her cousins at Grammy and Poppy's to celebrate Aunt Tami's "milestone" birthday. Since I had been gone the previous weekend, I asked her if she was sure she wanted to spend two nights away ... her reply: "Why only two nights, Mommy?" With that, she was out the door! The kids played so hard that after dinner was served, Madeline laid her head down in my mom's lap and went to sleep in the middle of the restaurant! When the waiter came by later to take a birthday group shot, she was so zonked that they had to hold her up for the photo!

Madeline returned home last night to find our house twinkling with lights. We narrowly escaped being the last ones on the block to get into the spirit! On Saturday, Susannah donned her mask and the two of us directed from the lawn, while Klane dangled from the rooftop with his staple gun. We have enjoyed just being in our own home so much that we've been a little slow out of the gate getting festive for the season, but we do have our advent calendar, as well as, the snowmen we received as parting gifts from Minnesota, prominently displayed. A Christmas tree and stockings are next on the agenda! Hint: You probably shouldn't look for our holiday card before February!

Thank you for checking up on us; we love reading your guestbook entries. We are so happy to have such good news to pass on these days. Home for the holidays has really taken on new meaning this year!

Love,

Amy, Klane, Madeline and Susannah

Friday, November 30th, 2001 ~ Day +417

Getting settled in ...


Hello There,

We are settling in nicely, unpacking slowly, sifting between boxes from this lot and those packed and sent back in July. As we attempt to essentially merge two households, we are constantly shifting things from one room to another, trying to find a place for everything. It is amazing how much paraphernalia one little girl can accumulate.

Speaking of Susannah, it goes without saying that she loves her newfound freedom, roaming about everywhere, including many places she should not be. We have quickly become reacquainted with baby gates and cabinet locks. Even though I trip over them daily, I find myself elated that we actually have need for them again!

Other than a slight battle with the time difference, Sleeping Beauty made the transition to her old room without a hitch. She really seems to enjoy the comfort of her own crib, sleeping twelve hours straight each night and hanging on to her two nap a day schedule. She has taken up the unladylike habit of grunting when she wants something (to get out of her crib, for instance) -- perhaps it is because there is so much to want and try in her new surroundings, but we are trying to break the habit nonetheless.

On Monday, we had our first official appointment at Children's Hospital San Diego. Dr. Kadota gave Susannah a complete one over and was very pleased with what he saw. He will be in close contact with Dr. Davies in managing Susannah's care; in fact he has already shown us first hand that he does not hesitate page her for the official word on things (nor do we, for that matter). As much faith as we have in the hem/onc staff here, it is nice to know our Minnesota security blanket is only a phone call away.

This week brought many new faces to our house. All three of Susannah's therapists officially began working with her this week. PT and OT will both visit four days each week and she will continue to have speech therapy everyday; scheduling these appointments around naps, meals, clinic and nurse visits is quite a feat! She is definitely trying to make a good impression on these ladies, doing the right tricks for the right discipline – as many of you know, little Susannah is quite famous for walking during speech, stacking during PT and "talking" during OT – it's only a matter of time until she shows them her sneaky side!

The most exciting new face we welcomed (and boy, do I mean welcomed!) into our home is Susannah's new nurse, June. By all accounts, she is our very own version of Mary Poppins and even has the accent to prove it! June is a PICU trained nurse with BMT experience and she will be coming to the house 16 hours each week to help me with Susannah's care. She is wonderful with Susannah and Susannah has quickly grown fond of her as well.

June's arrival allowed Madeline and I the luxury of spending some special time alone together. We even managed to sneak off for an afternoon of ice skating with friends (yes, we do have ice rinks here!). After hearing of the snow dumping, uh I mean falling in Minneapolis, Madeline suddenly became "homesick" for winter weather, hence the ice skating. You could tell our blood was still thick from last winter, as we were the only two in the rink without gloves on!

Even with all the action around the house, this week did provide us with glimpses of normalcy. I, for one, am thoroughly enjoying the little things ... picking up my pre-schooler at class, combing ponytails and tying hair bows, reading bedtime stories, cooking in my own kitchen and, of course, sleeping in a real bed. Ahh, the wonders of home.

Thank you for your continued prayers for Susannah. True, she has successfully completed a huge part of her journey, but in another sense, things have just begun.

With love,

Amy, Klane, Madeline and Susannah

Sunday, November 25th, 2001 ~ Day +412

Home at Last!


Hello from San Diego!

Home at last ... how sweet it is! After much fanfare, not to mention a few tears, we said goodbye to our many well-wishers at the St. Paul Airport and joined our hosts, Masco Corporation (via Corporate Angel Network), aboard their luxurious jet. The businessmen were very gracious and accommodating and didn't even mind riding next to a seat filled with a box of meds and a baby stroller! We had so much luggage that it didn't all fit under the plane! Their chairman loved children and even brought a toy for Susannah.

It was after dark when we touched down at the Newport Jet Center, but we could still make out a beaming little girl, flanked by Grammy and Poppy, waiting patiently at the edge of the tarmac, well, okay, they were holding her back! The instant the doors opened, Madeline rushed out into our arms -- what a wonderful reunion, our family was together again at long last.

On the car ride home, Klane and I were treated to the sound of laughter. The girls giggled in the backseat the entire way, thoroughly enjoying one anothers' company. At one point we could hear Madeline calling out random words, probably twenty in a row, "car, book, blanket, truck, doll ...". When we asked her what she was doing she firmly responded, "Shhh, I'm teaching Sissy how to talk!".

An hour later, we were finally back home on Mount Tami Drive. We were welcomed by a giant painted banner, cheerful balloons, goodies and champagne. Our freezer had been filled with home cooked meals and every nook and cranny of our house had been completely "Susannah-tized". The grandmas had spent the last few weeks cooking, washing curtains, lining cabinets and scrubbing anything and everything they could get their hands on, all in preparation for our arrival. Together with Klane, they had arranged for hepa filters, carpet cleaning, car detailing, you name it, it had been done. As if returning home was not enough of a treat, much to my delight, Klane and company had also given the place a complete facelift; he had painted virtually every surface inside the house, laid lawn in our backyard, installed a new floor in our kitchen and much more! Amazing, is the only way to describe it!

Our first few days home were chalk full of visits from nurses and therapists to get things squared away for Susannah's continuum of care. Thanks to the advance work of the teams, both back in Minnesota and here, everyone was very well prepared for her arrival. The transition has gone quite smoothly and things have really fallen into place.

On Wednesday morning we were off to Children's Hospital for a brief, "get reacquainted visit" and a quick check before the holiday weekend. The staff was happy to finally have Susannah back, as she's quite famous for dancing to their singing flower pots! Susannah's labs were very stable and her line was flushing perfectly ... uh, did I happen to mention the four hour stint in the ER, on our second night back, to remedy a clot in her PICC line? Never a dull moment.

Following those busy first days, we spent the long weekend enjoying the comforts of our own home and most of all just being together again as a family, under one roof. Although Susannah certainly does not remember this wonderful place we call home, she is adapting beautifully, enjoying the space and room to roam. She hasn't stopped exploring since we walked in the door and is, of course, totally enamored by her big sister. Madeline is extremely patient with her little sister and the idea of having a shadow in tow everywhere she goes.

We hope you all had a very Happy Thanksgiving. I can not think of a more appropriate holiday to follow our long awaited homecoming!

Love to all,

Amy, Klane, Madeline and Susannah

Click here to view older journal entries (diagnosis through transplant)

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