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Monday, November 19th, 2001 ~ Day +406

Farewell Minnesota and Thanks!!!


Greetings All!

The day has finally arrived. We really are going HOME! Klane flew in on Friday, we spent the weekend shoring up details and this morning Dr. Davies gave Susannah her final blessing. Late this afternoon, we will board a corporate jet bound for southern California and will all spend the night in our own house, together!

But first, before we go ...

Over the last year and a half, Minnesota has truly become our second home, and surely the only one Susannah remembers. As we walk the halls of the U of M hospital and clinics, Stadium Village and Dinnaken House, there is rarely a face we fail to recognize. We are all too familiar with every shortcut, tunnel, building, skyway and restaurant. In preparing for our return to San Diego, we are not only packing up our belongings, but also memories that we will treasure for the rest of our lives. While times here have been trying to say the least, through it all there has also been much laughter and joy to coincide with the uncertainty and fear, and often a mixture of both at once.

Saying goodbye has not been easy to the myriad of people here who have become such a big part of our daily life. So many wonderful individuals have given us the strength to persevere and managed to brighten even our darkest days, especially our amazing nursing team -- Mary Andersen, Kristy Faulkner and Kent Nygaard; "Trish", Jen, Erica, "Missy" and Andrea; Lisa, Yuri, Kris, Theresa and Rollie; Sarah, Rich, Andrea and Debbie. Never again will the following words pass our lips without a smile and fond memory: "Wisconsin", "Sheetrock", "Benefits", "The Meter", "Bop", "Little Man", "Frappe" and, oh, I could go on and on. We will always cherish ... the great lengths you went to in order to put on Susannah's dresses, amidst all of the tubes, lines and wires, just to make us smile; the many heartfelt and also silly conversations we shared during those long days and nights at her bedside; the way you read my baby stories and sang to her when you thought I wasn't listening; the way you polished her tiny fingernails, tended to her every need, preserved her dignity at all times and held her in your arms as if she were your most precious possession. Thanks for the memories, the friendship and, of course, the exceptional care and unparalleled sense of compassion you showed, not only Susannah, but Madeline, Klane and myself as well.

We came here knowing that the University of Minnesota was the place to offer Susannah the best and safest care, and we leave here having experienced every single aspect of that glowing reputation. We could not have entrusted our daughter's care and life to a more brilliant and benevolent group of physicians, Drs. Davies, Peters and of course, "Sat"; the remainder of the BMT team, Drs. Wagner, Orchard, Ramsay, MacMillian, Baker, Blazar and Kersey; also Drs. Sweeny, Cornfield, Hadad, Wendorf aka Rich, Milla, Charnas, Lockman, Lobo, Braunlin, Olsen, Rimmel, Leonard and Saltzman; and, who could ever forget, "Myank" and Dr. Steiner on that cold, snowy night back in February. Some have been with us through every step of this remarkable journey, while others have stepped in in times of need and trial, yet we are equally indebted to all for their input, counsel and expertise. We know full well the number of gray hairs little Susannah has cost so many of them.

To the incredible people of Saint Matthew's Episcopal Church, especially Grant Abbott, Lyn Lawyer, Karen Stoddard and Joan Hershbell and the countless others, who welcomed us with open arms and embraced our entire extended family from the moment we arrived here in the Twin Cities. We can never thank you enough for taking us under your wing and making us part of your special family.

Speaking of Joan, she and her husband Gary Johnson, have become such an important part of our own family. Words simply cannot express our gratitude for the impact they have made on Madeline's life. In no small part because of their influence, she has managed to endure this entire ordeal while remaining a happy, well-adjusted, confident and empathetic little girl.

Then there is Sue Reichert, far more than just our Care Partner, also a dear friend. She always knew exactly what we needed, no matter what the circumstances, even when we did not. She truly made our situation bearable. Not only did Sue indulge and spoil us, she was also instrumental in ensuring a sense of normalcy in Madeline's life, when things were anything but normal.

Jane, Jim, Rebecca, Maureen and Teresa, the extraordinary therapists who have worked so hard and brought Susannah so far. Whether they started with us, ended with us or were around for the entire ride, they have become such an integral part of our lives. They have been by our side through thick and thin. Even when Susannah was too ill to participate, they were still right there holding her hand, whispering in her ear, not as a therapist, but as a friend. We are thankful that the lines between professional and patient have become blurred and will miss the many hours a week we spend together.

The college girls from our apartment building, Kelsey, Gayle, Paula, Kim, Michelle and Danie, have been absolute lifesavers. They did a tremendous job looking after Madeline, and most importantly, they found their way into the hearts of both of my children. These girls have gone above and beyond the call of duty on so many occasions, rearranging their busy schedules in order to accommodate mine. I was able to count on them day in and day out, for anything imaginable, when I am certain there were far more exciting ways they could have spent so much of their time.

Cyndie Hibbs and Pat Fidler, by official titles Susannah's nurse practicioner and nurse coordinator, respectively, but they are much more than just that. These two ladies have personally come to my rescue more times and in more ways than I can ever count. Together they have counseled, consoled, confided, conspired, reassured and all-around preserved my sanity. I couldn't have possibly managed without their friendship.

Of course, this tribute to our time in Minnesota would not be complete without also acknowledging the many relatives and friends, not to mention perfect strangers, from back home and virtually every other corner of the world, who have so graciously supported us throughout these long months. Over the past year and a half, people have given of themselves in every way imaginable, emotionally, physically and financially. They have visited, called, written, e-mailed, sent cards, gifts and care packages, and done everything possible to brighten our days; they have cooked, cleaned, laundered, shopped, babysat and pampered us; they have loved, sacrificed, listened, hugged, hand-held, cried, comforted and above all, they have prayed -- prayed for Susannah, for our family, for our situation and for our strength. We have been humbled by the sheer magnitude of kindness that has been bestowed upon our family and will be eternally grateful for each and every good deed done, contribution made and prayer offered in Susannah's name.

Last, but certainly not least, there are the other families, the parents, spouses, children and siblings, who also come to Minneapolis in search of hope. We are not unique here, but rather one of many, part of an exclusive group to which no one chooses to belong. Through our joys and trials we have strengthened one anothers' lives with bonds and friendships, some lasting and others only for the moment. So many shoulders have been cried on, so many hugs given. Words simply do not do justice to the distinctive feeling of closeness found here. More than once have I reached out to another mother in her time of sorrow, only to realize later that I scarcely even knew her name ... it didn't matter, I knew her child, her situation, her feelings. It is about the children, the patients, about living for the moment. Time here stands still. There is so much to be said for those silent glances and knowing embraces that speak volumes, but utter no words. Throughout our stay we have seen waves of families come and go, some who saw happy endings, and so many others who did not; some were here for months on end, while others had their stays abruptly shortened. Each of their faces will be forever engrained in our memory.

So long, farewell dear friends and thank you all from the bottom of our hearts. At long last we are headed home ... you are welcome in sunny San Diego anytime!

All our love,

Amy, Klane, Madeline and Susannah

Tuesday, November 13th, 2001 ~ Day +400

The Countdown Has Begun ...


Hi Everyone!

The light at the end of the tunnel is definitely getting brighter -- it shouldn't be long now! This time, however, no one, and I mean no one, is letting Susannah in on the news. All kidding aside, the superstition around this place is really bubbling over, as everyone, from doctors to nurses to friends to neighbors, is mumbling under their breath, spelling and even covering Susannah's ears when the subject comes up! Knowing how attached she is to Minnesota, we're not taking any chances.

Regarding the diabetes insipidus issue, after two separate admissions, numerous cancellations and reinstatements, a change in physicians, two nights in the PICU and a comedy of errors, which was far from comical, we actually have the answer. Susannah has a mild renal component, which thankfully, affects only the kidneys and should be easily kept under control by removing the offending medications, giving additional fluids and modifying her diet. Alas, an answer and one with a simple solution!

Following what seemed like endless appointments and tests, Susannah's one year post transplant evaluation is finally complete. Over the last several weeks, we made the rounds to Pulmonology, Neurology, ENT, Ophthalmology, Neuropsychology, Orthopaedics, Endocrinology, Dermatology, Cardiology, Audiology, GI, and of course, Hematology. There were also x-rays, EKGs, an echo, a sleep study and CT scans of every body part imaginable; we did nix the MRI, though, due to anesthesia issues. Since Susannah's course has been so convoluted that most of these tests and visits were also just done in July, there was not too much new information, but all specialists were unilaterally pleased.

There were, however, a few new things to report on ... an EMG showed early signs of carpal tunnel syndrome, consistent with her age. While not of urgency, at some point in the future, possibly a year or two, the tendons will need to be released. Although these orthopaedic changes were new, the news was not at all unexpected. Susannah's lungs continue to improve and although she is still monitored while she sleeps and has occasional mild desaturations at night, it has been months since she has required any supplemental oxygen at all. The pulmonologist did prescribe some inhalant steroids to use, once her oral steroids are tapered back down to every other day. The cardiologist could not have been more pleased with Susannah's heart function. The PDA is indeed gone, closed up on it's own! I believe the words actually used to describe her heart were, "not just good, but great!". As for her episodes of low heart rate that I referred to previously, the situation quickly resolved on its own and has not reoccurred (we are chalking it up to the idea that a time change in the administration of Susannah's thyroid med, had her body acting up for the night). Just to be on the safe side, we did proceed with the Holter Monitor test, which fortunately proved to be unremarkable. We also received encouraging news from the audiologist in that Susannah's hearing has shown substantial improvement, even since her improved test back in May. At this point testing shows her hearing to be borderline normal and the issue of aids is now questionable. We will, however, continue to check her regularly because it seems her level of impairment tends to wax and wane. Neurology and Neuropsychology were both impressed with Susannah's overall steady progress. While her speech is still slow in coming, her understanding of things and the world around her has improved immensely in the last couple of months. As I mentioned a few weeks back, she is beginning to stand on her own a bit and is cruising everywhere, climbing up and down and is into absolutely everything!

Of course, her one year visit also included a boatload of labs! The big news is that Susannah's engraftment studies once again came back at 100% donor and her enzyme level remains in the high end of the normal range! While these results are not expected to change this far out of transplant, it is always reassuring to hear that 100%. Overall, Susannah's blood counts are very stable and she continues to produce her own red cells and platelets; her nutrition labs are also quite favorable.

Little Susannah is a celebrity of sorts around here. There has been such a vast change in her these past few months that everyone just oohs and ahhs over her wherever we go. During our anniversary appointments, all of the doctors marveled at the amazing transformation she has made. In fact, on one visit to the Peds clinic where we had an appointment with the gastroenterologist, we were stopped four different times by the neurologist, the pediatric surgeon, the pulmonologist and the geneticist, all before we ever made it into the exam room! Everyone wanted their chance to see the new Susannah! The beaming smiles of these men, spoke volumes about Susannah's resolve and just how far she has come.

While Susannah's incredible strength and tenacity continue to amaze everyone around her, her immune status remains extraordinarily tenuous. Although her blood counts are encouraging, the medications she is taking for her chronic graft versus host disease, keep her immune system nearly as compromised as it was in the early stages following transplant. This is somewhat deceiving since most children at this stage post transplant are weaned off their immunosuppressives and beginning to reintegrate into society. However, as we are all very much aware, Susannah does things her own way and in her own time.

As we begin preparations for our departure to San Diego, the subject of her fragile immune system comes to the forefront. For the foreseeable future, we will follow her body's lead, and keep Susannah's exposure to the outside world at a minimum. As if she weren't already a princess, once back home most things in Susannah's life will continue to be brought right to her. Therapies, lab draws and any other services will all be performed in our home, with clinic visits being her only real adventure out, and even then she will need to be hidden under her mask and whisked in the back door. The people here in Minnesota have gone into high gear and are busy making the preparations to help insure a smooth transition.

Please forgive us if we seem a bit reclusive upon our return home for a time, but we will need to be extremely cautious where Susannah is concerned. Don't get me wrong, we are not planning on completely sequestering ourselves on Mount Tami Drive and we are very, very excited to see you all again, but we will need to take things slowly with gatherings and visitors for Susannah's sake.

It really seems the countdown has begun ... we can hardly wait!!!

Love,

Amy, Klane, Madeline and Susannah

Tuesday, October 23rd, 2001 ~ Day +379

What now, you ask?!?


Hello Again,

Just when we thought we'd heard it all ... what now, you ask?! Or better yet, what else could there possibly be? Well, the diagnosis du jour is a pesky thing called diabetes insipidus. And, yes, you guessed correctly, our Susannah has it. Rest assured, you are not alone if you're asking yourself, where in the world did this come from and why now? My sentiments, exactly! I will try to explain, but I am certainly no expert, so please bear with me.

Several weeks back, I mentioned that Susannah began having cyclical bouts of dehydration, accompanied by weight loss, nausea and vomiting. Well, every time we thought the issue was resolved, it reoccurred again. After a few weeks, a strange pattern began to immerge. The symptoms became apparent on Tuesdays and Wednesdays, but after loading her up with IV fluids, Susannah was always on the upswing by Fridays. As the weekends came and went, her weight recovered, her electrolytes were perfect, the wretching disappeared and her appetite returned with a vengeance. Right on cue, however, every Tuesday the cycle began again. At first the symptoms were attributed to her chronic GVHD and if you recall, the vomiting seemed to be remedied by an increase in her steroid dose. Well in retrospect, the steroid increases were made on Thursdays, about the time she usually began to turn the corner anyway.

The biggest piece of the puzzle was the huge, unexplainable dip in weight. Then, there was the sudden change in electrolytes, immediately followed by the wretching and subsequent vomiting. Some additional lab tests showed that Susannah's blood had an extremely elevated sodium concentration, yet the very same test performed on her urine, showed things to be normal. Generally, if someone is showing such major signs of dehydration, their pee should be very concentrated. It seemed Susannah was peeing out most of the fluid in her body, causing her sodium level to skyrocket, thus inducing vomiting ... but the question remained, why only on Tuesdays and Wednesdays?

A renal consult was called and the nephrologists immediately tried to pass it on to GI, saying vomiting was their area and it was causing the wacky labs. But Dr. Davies and her fellow had a hunch that kept nudging at them, so they basically arm wrestled "the bean team" into delving deeper into the situation and ordered a kidney ultrasound. As soon as the renal folks realized the vast difference in the urine and blood osmalalities, the answer became evident to them as well -- diabetes insipidus, which causes Susannah’s kidneys waste urine in a crisis situation, as opposed to concentrating it as it should.

Let me stress that this condition is NOT the traditional type of diabetes most people are familiar with and this particular type is easily treated. In order to treat her appropriately, though, it must be determined which variation of diabetes insipidus Susannah is afflicted with. It may either be a renal form, or a central form, or a combination of the two. A renal version could be attributed to two of her antibiotics, Bactrim and penicillin. Whereas a central version, which results from an inability to produce a specific hormone, is likely a sequela of the radiation she received as prep for her second transplant. In both cases, the remedy is simple, we either stop the antibiotics or give her a medication to replace the missing hormone. Given the fact the her symptoms mainly occur on Tuesdays and Wednesdays, Bactrim seems to be the most likely culprit, since she takes it only on Mondays and Tuesdays, which might explain the weekly pattern.

In the next week, Susannah will be admitted to the hospital for a day of intensive testing to further investigate the cause. Since she will require twice hourly labs and receive medication which has the potential to cause either severe dehydration or fluid overload very quickly, Susannah will need to be closely monitored over the twenty-four hour period, in an ICU setting. While those three letters are anything but comforting, I have been assured and reassured that the reasoning behind the added precautions is only to provide the safest possible environment for Susannah, because after all, she is Susannah and no one is willing to leave things to chance.

Believe it or not, there is some good news in all of this. For once, we were actually able to isolate a problem and rather quickly at that (as you know, this is no small feat where Susannah is concerned). Although it may seem strange, we are happy to have an answer, and a treatable one no less. We should have more definitive confirmation and a plan of action by the middle of next week. Also, since it seems that Susannah's chronic GVHD is not the catalyst in this whole issue, we can now begin slowly tapering her steroids back down to the every other day regimen, which is a far more gentle on her body.

Just in case it wasn't obvious, this new development will push back our departure from Minneapolis, which was scheduled to be this coming weekend, just in time for Halloween. True, we were so close, but it is in Susannah's best interest to have the present issue resolved before making the transition to a whole new group of doctors. Besides, the situation seems to be straight forward enough that the delay should be brief, only about two weeks or so. This does, however, put somewhat of a glitch in our Halloween plans. You see, there is one very special Peter Pan who will now be without her precious Tinkerbell next Wednesday night. Fear not, Madeline and I have already begun planning a belated costume party for the four of us upon our return (Klane, err, I mean Captain Hook had better brush up on those sword fighting skills ... hmm, I wonder who I'll get to be?!?), complete with "Ghoooul-ash" baked in a real pumpkin and bread "bones"! At any rate, our absence will allow Madeline to spend the evening at her annual playgroup trick-or-treating party ... where she always has a ball!

On an exciting note, the gancyclovir trial was a success. Val-gancyclovir (Val-Cyte), it is -- this particular oral form actually produced higher blood levels than the IV form. This is wonderful news! Susannah will transition to the oral drug this week and we can finally put the issue of the eye implants versus intravenous administration to rest. It will be so nice to go home without daily IV meds and it will be much safer for Susannah in the long run, as we will eventually be able to remove her line. Of course, we will continue to monitor the levels regularly, but they can be sent directly from San Diego.

There was a funny twist in finding a lab to actually perform the assay for gancyclovir levels. Since this is not commonly needed, there was difficulty finding a lab willing to do the test. After much investigation, a lab was finally engaged. However, when that lab fell though on the day before the blood was to be sent, every contact involved was calling everywhere to see about getting levels and getting them quickly (ironically, we didn't want this to delay our departure!). With what we all thought was no where else to turn, Klane began working on a lead in Norway, of all places. Yes, I mean the country! The blood was going to have to be packed on ice and shipped abroad to be tested, since according to the drug manufacturer, no lab in the states was currently performing the assay. Well, last Tuesday, Dr. Davies danced into clinic waving a sheet of paper containing the results! What??? How??? When??? It seems the send-out lab had taken it upon themselves (which is amazing in and of itself) to contract someone to perform the assay and not only that, the results were already back. Again, what??? To everyone's surprise, Susannah's blood samples had been sent down to the Mayo Clinic, where they routinely perform the assay for gancyclovir drug levels. Realizing that Mayo is a giant research entity, this may not seem like a big deal, but what you must understand is that the Mayo Clinic was the very first place called upon when this whole process began back in August and had told Dr. Davies that they could not be of service. Very strange, but we'll take it nonetheless. You can bet, a certain lab tech received a big box of chocolates that day!

As for Susannah herself, she has a few new tricks up her sleeve this week. Both of which I am somewhat appalled to report, but should give you all a good chuckle. At the very mention of the word "Daddy", Susannah now promptly brings her hand to her head in the sign for "phone". I am not sure where she learned this, but it is certainly perceptive of her. Boy, do we need to get home! While the report of the former didn't exactly make Klane smile, the following certainly did. Lately, at the slightest "rumble" from her diaper, Susannah lifts her little leg, then covers her mouth with her hand and lets out a great big belly laugh. Quite the little lady, eh?! I try not to laugh, but it is pretty funny. Naturally, Klane thinks this is hysterical.

Finally, please say a special prayer for the family of little Bryanna ... another baby girl who lost her battle with Hurler Syndrome. She had just returned home to Indiana after a successful second bone marrow transplant and was doing well, when she passed away very unexpectedly. Needless to say, her parents and siblings are devastated, as is everyone else whose life she touched.

All our love,

Amy, Klane, Madeline and Susannah

Thursday, October 4th, 2001 ~ Day +360

Change of Season


Hi There!

Fall has officially unfolded here in Minnesota -- the leaves are changing, the students are back, apples are ripe for the picking, the air is crisp and coats are now a necessity. Yikes, I have no coat here ... I sent it home back in July! Who'd have thought Susannah and I would be spending our second autumn in a row in the Twin Cities?!? While the change of season is certainly lovely, it would be untruthful to suggest that I don't long for that year-round beach weather San Diego is so famous for. The goal is to get out of dodge before the first snowfall!

Things here have been moving forward as planned and we are still shooting for a target date of late October, just in time for Halloween. Yes, I am aware that it has snowed here in October before, but I'm not focusing on that!

Susannah is in the midst of her third and final week of the gancyclovir trial. We are anxiously awaiting the labs demonstrating what kind of blood levels she can obtain from the oral gancyclovir and the val-gancyclovir, as opposed to the IV form of the drug. The final specimen will be drawn tomorrow and samples from all three weeks will be sent out this coming Monday.

Assuming Susannah achieves adequate absorption from either of the oral forms, the wheels here will really begin spinning toward discharge to San Diego. With her recent platelet transfusion independence, we could be on the road with nothing more than a PICC line (and that for only as long as it lasts) and oral medications to contend with. Wouldn't that be a treat!?! Don't let me kid you, there would still be a variety of other issues, but at least we would be free from the risk and worry of a central line, not to mention avoid another surgery.

Just to keep things lively, Susannah decided to provide us all with a few minor scares over the last couple of weeks, all of which were quickly eradicated, but worth mentioning nonetheless. First, one night she experienced some significant bradycardia (low heart rate), which didn't appear to have an effect on her, but did manage to keep me up all night as the alarm on her monitor was perpetually being triggered, even though I continued to lower the limits. At one point her heart rate was dipping into the low 60s. This event, although it has not repeated itself as drastically since, prompted Dr. Peters to consult the cardiologist, who ordered a test in which Susannah will wear a contraption called a Holter Monitor for a single 24 hour period. Basically, on Monday, they will hook her up to the heart monitor for one day to get a more in depth look at all of her cardiac "wiring", just to be on the safe side. They tell me this is just precautionary to make certain everything checks out and they have no reason to believe it won't.

We also spent seven days straight in the clinic this past week ... anywhere from 6-8 hours each day, giving the little miss some much needed fluid resuscitation. For some reason, which remains up for debate, she decided to begin vomiting and become severely dehydrated. Luckily, we were on top of it right away and she managed to avoid a stint in the hospital, but she still lost an entire kilo in a matter of days and her labs got pretty out of control (for those of you who are interested, her creatinine reached 1.0, her BUN 80 and her sodium was somewhere in the high 150's). Anyway, after a boatload of fluids, which her lungs handled phenomenally, she was back in business by Friday. She did wonderfully over the weekend, eating and drinking like a champ, but had a brief, unexplained relapse on Tuesday. Things were again back in control by this morning. Without evidence of anything concrete, the running theory by most is that Susannah's chronic GVHD was acting up a bit and irritating her gut. There was a definite elevation in her eosinophil count, and that combined with a recent move downward on her steroids, was enough to make a fair case. With Dr. Peters out of town, Dr. Davies elected to make a move back up on the prednisone (just incase it wasn't obvious, there has been some quibbling over Susannah's steroid dosing and how it should be handled). While the increase was ever so slight, it really seemed to make a difference. Dr. Davies has had several patients with "chronic G", as she calls it, and swears even the tiniest movement one way or the other on the steroids can have significant effects. The vomiting and wretching did prompt an essentially useless visit to the GI clinic. We walked away with an appointment for a lovely barium swallow/upper GI, which was promptly cancelled by the BMT folks when the symptoms subsided and Susannah was on the mends.

We have now begun the countless appointments related to Susannah's one year post transplant evaluation/check-up. Wow, what a schedule! However, since we have the luxury of not having to pack all the appointments and tests into a single week (yes, most families come BACK to Minneapolis for a week on their anniversary visit), our schedule is not nearly as tiresome and stressful as is tradition. All of the results and impressions will be compiled in the coming weeks and we will surely fill you in on things at that time, but so far things have been quite positive.

The weekend brought our two favorite visitors, Madeline and Klane, along with some much needed family time. Susannah and I needed a little fix to sustain us through the next few weeks. It was a relaxing couple of days filled with lots of lounging and "ham and cheese sandwiches", Madeline's invention of a four-way snuggle with Klane and I on the outside as "bread" and she and Susannah in the middle. I'll let you guess which girl is the ham! We said goodbye to Klane on Tuesday evening, but Grammy arrived tonight for a visit, so Madeline will get to spend an extra week with Mommy and Sissy (and of course, Grammy too)! The plan is for the two of them to fly home together on Tuesday evening.

Within an hour of Grammy's arrival tonight, she took charge and plans were underway for Elvis, uh, I mean Susannah to receive a haircut. Well, actually more of a touch-up. While the overall length is coming along splendidly (in fact, Madeline is quite excited about her ability to put a bow in Sissy's hair ... just like her own), Susannah could stand a little trim on the sides. As fond as we have become of her "lamb chops", really, she could give Elvis a run for his money, we are anxious to see more of her pretty little face. Her sideburns literally connect together under her chin! With the CSA still on board for the foreseeable future, we will likely have to invest in some shears for monthly trimmings to keep ahead of the game, or should I say the five o'clock shadow. For those of you familiar with the loveable fuzz, fear not, we have no intention of touching that famous "pet-able" back hair, nor the classic hairline!

The girls are so cute together! Susannah just adores watching Madeline's every move and Madeline is the model big sister, catering to Susannah's every whim doing anything and everything to produce a giggle. She has also become an expert in the two-man diaper change, singing verse after verse of "Oh Susannah, now don't you cry for me, I really want to play with you, so don't you cry for me ...". Susannah absolutely loves "Itsy Bitsy Spider" these days and Madeline has successfully taught her most of the corresponding moves. The little sister also dutifully obliges her role in the game of "Beauty Parlor", enduring endless combings and stylings at the hands of the "beautician". Madeline is the apple of her eye! It is so sweet to see the bond they share.

As always, we are grateful for the love and prayers you continue to send our way. They and you mean the world to us!

Much love,

Amy, Klane, Madeline and Susannah

Friday, September 21st, 2001 ~ Day +347

Time to Catch-Up!


Hello All,

With the leaders of our nation asking for a return to normal routines, I figured it was time to finally write an update. While pale in comparison to the tragic events of the last couple of weeks, there have been some happenings here in Minnesota, as well as California, to catch you all up on.

Medically speaking, things with Susannah have been steadily progressing with no real setbacks to speak of. Then why, you may ask, are we still here?!? More on that in a moment ...

After six long weeks on multiple IV antibiotics here at the apartment, Susannah remains infection free and our life has suddenly become vastly simplified without the familiar five daily dosings of the intravenous meds. In fact, I don't quite know what to do with myself during this hiatus from flushes, syringes and pumps, and Susannah is enjoying her virtual freedom from being "hooked-up". While she still sports a PICC line in her left forearm, she is officially disconnected for the first time in eons. Ahhh, freedom!

For the most part, we have stabilized to a twice weekly clinic schedule, seeing Dr. Peters on Mondays and Dr. Davies on Thursdays. This is a nice break and makes Susannah's five day a week schedule of all three therapies much easier to manage. Although, on the occasion that we do get delayed in clinic, Jim, Jane and Rebecca have been really wonderful about venturing over to clinic in order to treat Susannah. Our weekends are free, but quiet, relaxing and house bound -- as you may recall, our car went back to San Diego in July, not to mention Susannah's need to be secluded due to her continued immunosuppression.

There has been a bit of flaring on the chronic GVHD front, followed by our old friends itching and wretching. Hopefully, some hold off on her steroid taper and other med adjustments will calm things back down soon. Truth be told, however, the best eliminator of Susannah's incessant scratching, is to keep every inch of her body covered in clothing so it in inaccessible to those quick little fingers. Naturally, this makes diaper changes a real treat. The instant a piece of clothing is removed her fingers get right to work. So, as you might imagine, any poor soul passing by gets recruited to do a little arm holding and sing-songing. It's definitely a two person job!

Along those lines, Gayle, Paula and Kelsey, three of the college girls, have absolutely been my saviors these days. They have been essential in getting the PICC line dressing changed (talk about a two person job!), making midnight milk runs, vacuuming the apartment while Susannah and I are at clinic, allowing me to do laundry down the hall and all around making my life a little easier. The girls have also been a huge help in getting Susannah to eat. This has even spawned a sort of competition between them: "She drinks her milk for me.", "Well, she eats rice for me." or "Let me try, I can get her to eat anything!". I don't know how I would manage without them.

This week we had a "platelet party" in clinic! That is to say that for the first time in nearly 12 months, Susannah is making her own platelets --yes, she is platelet transfusion independent! Yippee!!! This is great news as it is one more sign that her new bone marrow is maturing. It will also make our transition home much more simplified. As such, this news put plans for a central line on hold (pending the need for IV gancyclovir). For now the plan is to leave well enough alone with the PICC line, maximize it's potential and hope it gets us through. While it would have been nice to be in California by now, never did we expect the opportunity to leave here without the need for transfusions or even TPN, and if we play our cards right, we may just get to take Susannah home without a line as well! So, I guess you could say ... good things come to those who wait.

Speaking of going home, at this point we are delayed again and looking toward mid-October. Ugh! With all the positive news and non-events, you are all probably wondering, what in the world is the hold-up? Well, once again we are being held captive by the age old issue of Susannah's CMV retinitis, aka the gancyclovir dilemma (which, as you may recall, we were forced to place on hold until her infections were finally clear and she was off antibiotics). Some new information has come to light in the last month which has prompted us to pursue another route.

With Dr. Peters still on his implant crusade, and the rest of us continuing to have reservations, news of a brand new form of oral gancyclovir, known as val-gancyclovir, equivalent to the IV form, sparked Klane to do a bit of investigating on the issue. Conversations ensued between both the retina surgeon here, as well as, in San Diego and pharmacologists and infectious disease docs across the country. Ultimately all agreed that this new drug was definitely worth pursuing, if not a viable alternative for Susannah's treatment.

If only things were that simple. The first major glitch lies in the fact that there is absolutely no pediatric dosing for this drug, much less for a child of merely 12 kilos. Secondly, the only form val-gancyclovir manufactured is in the form of a coated tablet; seems like a small issue, but there is much more involved. The key to both issues, as well as a few others, is testing the in drug levels in the blood. Easier said than done ... let me explain.

Immediately, between the group of experts Klane had assembled, they were able to ascertain a reasonable dose by comparing adult and IV ratios. Still they needed to double check themselves -- answer, levels. As for the issue of the tablet, at first it seemed like a non-issue since many of Susannah's meds consist of crushed pills, opened capsules or suspensions compounded especially for her in the depths of the U of M pharmacy. Well, without attempting to get scientific (why didn't I make Klane write this update?), keeping this particular pill intact is likely the key to it's potency. Unlike the regular form of oral gancyclovir (whose absorption is questionable in relation to treating the retina), val-gancyclovir is known as a pro-drug, which means it bypasses the stomach and does not get broken down until it reaches the liver. It is this mechanism that makes it equivalent to the IV form of gancyclovir. Thus, crushing it or suspending it could likely impact it’s effectiveness, essentially turning it into plain old gancyclovir by diminishing it's capacity to withstand stomach acids. Back to the levels. If blood levels can be obtained then both gancyclovir and val-gancyclovir (even if only partially effective due to breakdown from crushing), could simply be dosed according to levels in Susannah's blood.

So, as you might guess, the answer is, LEVELS. Well, at first we were told that it would be impossible to obtain such levels. While it is common for blood levels to be run on many medications, apparently not gancyclovir. Back to Klane's investigation ... it seems a big study on pediatric use of regular old oral gancyclovir, using drug levels, was conducted at none other than UCSD! Yep, right in our own backyard. Well, the study is long over and the assay is no longer up and running, but the doctors affiliated with UCSD have been very instrumental in working with the ID docs here in obtaining levels elsewhere. Finally, this is going somewhere!

Now that I have gone into more detail than you could have ever wanted, while the docs are busy finding a lab to run the levels, our little patient is involved in her own mini drug trial. Susannah will have three sets of blood levels drawn over the course of three weeks. She will be treated with one week of oral gancyclovir, one week of val-gancyclovir and also one week of IV gancyclovir. The hope is that the levels will allow either the oral or val-gancyclovir to be titrated based on the comparison of blood levels achieved on the IV form. Although certainly not our goal, or our intent for that matter, but while helping herself, Susannah will also be laying the ground work for a potential new treatment for all the children who follow her. Of course, the docs are intrigued, which, to be honest, is probably the reason behind their enthusiasm. Whatever works, I suppose, as long as Susannah is the beneficiary.

The final day of the trial will be October 5th. All frozen blood samples from the previous weeks will be sent off together on Monday the 8th. Realistically, we need to allow for a week or two for the results to be completed and analyzed. Once we know adequate absorption can be obtained, any future adjustments can be made from San Diego. Assuming this to be the case and allowing a few days to shore details up here, we should be able to head home sometime in the fourth week of October. Keep your fingers crossed!

If things work out such that we can actually take Susannah home on all oral meds, with out a line and avoid the risks of surgery, then it will have been worth the wait. Although, I would be lying if I said the delay wasn't beginning to take its toll. This coming Sunday marks the one year mark of our second arrival in Minnesota, and the October 9th anniversary of Susannah's second transplant is fast approaching. We are setting a few records around this place that I could stand to do without. The people here have become our friends, not just our caregivers -- you know it's been a while when you hear a therapist call herself Auntie Rebecca and the echo tech is known as Uncle Jim or better yet, when Susannah shuns a video only to turn her head to watch him perform her echo-cardiogram instead!

Okay, enough of the medical mumbo jumbo and onto the fun stuff. Susannah is making more and more progress with each passing day, physically, emotionally and developmentally. Of course, she still has a long way to go, but we have nothing but time and patience where Susannah is concerned. She is all over the place, cruising everywhere and even standing alone for a few moments here and there. Her latest past time is climbing up onto the couch, the chair, the coffee table, the bookshelf, basically anything and everything that is the right height. Luckily, she has also mastered getting back down! Go, go go -- she is constantly on the move. I think all would agree, Madeline has coined it best, "My sister is a wiggle worm!".

The signs for "baby", "book", "flower", "phone" and "hat" have been added to Susannah's repertoire, along with a few others that are escaping me at the moment. She also now verbalizes the word "cheese" clear as day, but it is not quite as clear whether she is requesting cheese specifically, or simply food in general when she uses it. At any rate, it sounds adorable. "Hi" continues to be her favorite word, although it is now accompanied by a wave. Susannah really loves her books of late, especially one with large pictures of babies on each page. She is currently working on simple puzzles and stringing large beads; she has become an ace at stacking and stacks absolutely everything! It seems someone must have mentioned to her that two year olds have a reputation to uphold, because Susannah certainly has a mind of her own these days.

Back on the home front, things are going very well. With Madeline back in California for over a month now, Klane is handling the job of solo parent like a champ. He has become quite the expert in hair bows and ponytails. Many thanks to all the grandparents, aunts, uncles and friends who have pitched in to help Klane balance work and Madeline, as well as a home renovation.

Yes, the mold has been completely removed from our family room and the necessary tear down has begun. Reconstruction is scheduled to begin on the 15th of October. While the work will not be completely finished, should we arrive home as scheduled at the end of October, a large portion (and more importantly the part dangerous to Susannah) will be finished and totally sealed off from the rest of the house. So, we will indeed be able to move directly into our own house once Susannah is finally discharged to San Diego.

The biggest news back home is that Madeline started preschool at St. David's this month. She attends on Monday, Wednesday and Friday each week and is absolutely loving it! Besides classroom time and recess, preschool includes weekly chapel and a dance class among the activities. Madeline also began her new ballet class with her little friend Dana, which has been a big hit. Of course she still spends a lot of time with her buddies, going to the beach, playing imaginary games and having tons of fun. A backyard "daddy-kiddo campout" at the Jacobs' house was the hit of this week ... yes, they both actually slept (and fit!) in the famous tent.

While it was difficult not being with Madeline on her first day of school, knowing she is enjoying herself so much brings me such happiness and piece of mind. Each night she recounts the details of her day to me over the telephone and we sing our special bedtime songs. She is such wonderful, fun-loving little girl and I couldn't be more proud of her. Anticipating the day when our little family can all be together again under one roof is what keeps me going during these long separations.

Susannah and I did have the pleasure of a visit from Madeline and Klane over the Labor Day weekend. Not only did we get to spend some special time together as a family, but Sissy and Daddy also enjoyed some alone time with each other, while Madeline and I went out on the town. Undoubtedly, our most exciting outing was a trip to the Minnesota State Fair. Let me tell you this was definitely a fair like no other and we did it in style. Joan, a veteran fair-goer, was our personal guide and made certain we had the true state fair experience. From the famous foods-on-a-stick to deep fried cheese curds, to watching bees make honey to animals being born before our very eyes, we did it all. Madeline even milked a cow all by herself!!!

Wow, talk about a marathon update -- there certainly was a lot to catch you all up on. Thank you so very much for your continued love and support of Susannah and our family. Your thoughts and prayers continue to give us strength each and every day of this journey.

Our love to you all,

Amy, Klane, Madeline and Susannah

PS … Happy Birthday, Poppy! We love you and wish we were there to help you celebrate your special day!!!

Tuesday, August 21st, 2001 ~ Day +312

Flew the Coup


Hello Everyone!

Really, this is a short one! I just wanted to let everyone know (somewhat delinquently) that Susannah was finally discharged from the hospital last Wednesday. So far, so good without a "bounce-back" admission.

Susannah is apparently enjoying the elbow room of the apartment. Even though we brought the crib home last month, the little fur-ball is sleeping like a rock in her makeshift pack-n-play bed. She is continuing to make progress in all of her therapies. Amy thinks that she is on the verge of budding in terms of her signs.

Other than the nonstop administration of IV antibiotics, Amy is hanging in there as well. I think she is enjoying the quiet of the apartment. Truth be known, however, that outpatient life is sometimes more difficult than being in the hospital, especially when you're on your own. So, Madeline and I will head back next week for Labor Day weekend.

Speaking of Madeline, we are having a wonderful time together. She spent the week catching up with her many cousins and friends. Grandma Dona is staying with us about four days a week to help out as well.

Generally, the plan still remains the same. Hopefully we can have the girls home by the end of September, exactly one year from the beginning of the second transplant! Whewwwww!

We'll keep in touch.

Love,

Klane, Amy, Madeline and, of course, Susannah

Thursday, August 9th, 2001 ~ Day +300

Ay yi yi!!!


Hello Again,

First of all, another milestone: Post Transplant Day +300 ... from BMT #2! Ay yi yi!!!

I had barely sent off Tuesday’s update when Amy called to tell me the plan had already changed and in a big way. On Tuesday afternoon, Amy got wind that Susannah's latest culture was not the same run of the mill Strept infection, but rather ... music please, (a la police thriller/Beethoven's 7th) dum, dum, dum, dum ... VRE!!! Yes, the big bad VRE (vancomycin resistant Enterococcus) was back, and in Susannah's line and blood stream. No wonder she couldn't clear it!

Like I mentioned in the last update, Susannah's peripheral line had already blown by Monday night, and into Tuesday she was still not feeling well and having low grade fevers. Her leg and foot were blown up like a balloon from the bad peripheral IV. In the mean time, we were covering her with twice daily shots in the leg of ceftriaxone, poor baby! Probably to little avail given the VRE. With the new urgency in treating her infection, and the clear trouble that we were already encountering with the peripheral IV's (even the pediatric anesthesiologists were having difficulties finding veins), we all decided that Susannah needed a PICC line. A PICC line is sort of half way between a peripheral IV and a Hickman catheter, and goes in her arm. But, it requires a trip to the operating room. The last time Susannah went to the OR while I was home in San Diego, it took five months for her to wake up enough to recognize me! So, of course, this was a little disconcerting for both Amy and I. In light of this, as well as other issues, we've tried to make it a policy to not take Susannah to the operating room without both of us being present. Unfortunately, she needed to go right away, as they needed access, and there was no way I could get back there in time.

Dr. Peters made certain that all the ducks were in a row, with the anesthesia and otherwise, which made us feel a little better. We like the fact that he is as compulsive as we are (yes, that is possible!), and details are rarely overlooked with him on service. Susannah went to the OR yesterday. It took the interventional radiologist TWO HOURS to put the PICC line in! We do total knee replacements in two hours. I'm not sure what took so long, but as you can imagine, we were both kind of stressing out. In fact, we weren't the only ones -- after an hour and thirty minutes, Dr. Peters went down to the OR himself to make sure everything was okay. Despite the lengthiness of the procedure, Susannah was able to get by, again, with the LMA and avoid being intubated. Whewwww!!! Ultimately, Susannah did great, and came out just fine. Amy had to laugh, though, because not fewer than three anesthesiologists came over in the pre-op area, just to say "Hi"! It's pretty scary that we can't go anywhere in the hospital without knowing a majority of the physicians, nurses and staff. We're regulars alright! The Norm and Cliff of Fairview University Hospital; straight out of Cheers.

In general, the plan remains the same, but is still not fully clear. Susannah will remain on IV synercid (for VRE and Staph epi) for 3 weeks and IV ceftriaxone (for the Strept) for two weeks; then we'll watch and wait. If all is clear, then we will likely proceed with the gancyclovir implants and some kind of more permanent line, such as another Hickman or possibly a port (which stays under the skin). Time will tell.

On a sad note, the same day that Susannah went to the OR, Amy and I were saddened to learn that yet another Hurler child lost her battle with the BMT process. This was extremely sudden and unexpected. It's never easy to take, and as a matter of fact, I think it's only getting harder -- extraordinarily gut wrenching. We have gotten to know this family quite well, as they too have been here since last September. They, if anyone, although for very different reasons, have had it just as rough as we have. Now I know, they have it even worse. Our hearts break for their family. It's scary how suddenly things can turn on a dime! This is the kind of event that just re-nforces our hyper-vigilance. So be it, if it drives the medical staff and nurses crazy ... you just can't let your guard down, because tragedy is always hiding right around the corner.

On the brighter side, I am very much looking forward to Madeline's return to San Diego this coming Monday. Yeah!!! Poppy just arrived in Minnesota to escort her back home (as you probably already guessed there was a slight swap in grandparent scheduling). I can't wait to have her here with me, but I know the girls will miss the special "sleepovers" at the hospital they have been having these last few days.

As usual, all our love to all of you,

Klane, Amy, Madeline and Susannah

Tuesday, August 7th, 2001 ~ Day +298

And the saga continues ...


During the last episode, young Susannah Skywalker and her rebel fleet had turned the tide against the BMT empire. Escape from the Minnesota Imperial cruiser seemed imminent, but the dark side was not to be deterred so easily ... as the rebel forces loaded onto the Millennium Falcon, they were besieged by Staph epi and Streptococcal storm troopers and escape was denied. And the saga continues ...

UUGGGGGGGGGHHHHHHHH!!!!!!!!!

I think you all get the point. I really am becoming convinced that Susannah actually likes Minnesota and wants to stay! Well, here is the latest news.

At present, Susannah, Amy and Madeline are all in Minnesota and all in the hospital. Susannah's newest line was finally removed yesterday because she continues to spike fevers, and again grew out bacteria from her blood cultures. The current plan is to TRY to give her two weeks of IV antibiotics sans line, and then put in a new one. Complicating the issue are two other problems. Her chronic GVHD is again out of control and going crazy, undoubtedly spurned on by the line infections. Her kidney function is also showing signs of getting beat-on a little too much. Given these issues, we're looking at a minimum of five to six more weeks in Minnesota.

Let me back track a little. When we last updated, we were trying to save Susannah's line, the second in less than a month. We knew this probably was not going to work, but worth a try nonetheless. She actually went a week without positive cultures and things were beginning to look promising. Then, about the middle of last week, Susannah began developing low grade fevers and growing a new bacteria in her blood stream. Strangely, the original infection, the Staph epi, seems to have resolved. However, she is now contending with recurrent Streptococcal infections. With all the steroids and CSA on board, she just is not capable of clearing the infection with the line in there. Yesterday, we got wind of the latest positive culture, from Saturday, and the decision was made for us. The line had to come out. Within hours, the surgery chief resident (Dr. Saltzman was on vacation) and Dr. Sweeney (our favorite PICU/Anesthesia attending) removed the Hickman catheter at bedside with a little lidocaine and Versed (kind of like valium). As I mentioned, the plan now is to give her two weeks of IV antibiotics through a wimpy peripheral IV, the first of which has already blown. This will be a real trial, as a peripheral IV lasts, at best, three to five days in the most optimal of circumstances. Then we need to wait several days off antibiotics to be more confident that this pesky infection won't come back again.

The line infection business has obviously been very trying on Amy. First was the uncertainty of what was going to happen next, but also there was the torture of being placed on Unit 5A, where the nursing staff had absolutely no idea what they were doing when it came to BMT patients. The BMT floor (4A) was, and will, remain full and 5B, the Hem/Onc floor (where post transplant patients are usually admitted), was closed for new carpet, or something stupid like that. Not only were the nurses uncomfortable with Susannah, but Amy got to contend with the brand new interns, who roam 5A. Speaking from experience, the quality of interns on that floor is a little spotty. Some are good, and some are not. One thing I've learned from this experience is to take some time, just a little, and listen to your patients and/or their parents, because they may just lead you to where you need to go. The new interns, of course have little experience to work from, especially in the case of a complicated BMT patient; the ones who do well are those who listen, and don't pretend to know everything. By now, Amy knows far more about BMT patients, particularly Susannah, than even a majority of the third year pediatric residents! So when they don't listen to her, it can become a particularly frustrating experience. I should, and am happy to say, that we have had some very good house staff over the last year and a half as well, but back to my point.

Unfortunately the combination of being on 5A, by herself for three weeks (and for the last two weeks with an attending who didn't really care to listen either), was sending Amy over the edge. On top of that, she was sleep deprived, getting about five hours of broken sleep a night, on a good night. This all came to a head on Thursday evening. Susannah had been running low blood pressures all day, and Amy kept bugging anyone who would listen to make sure that Susannah was okay. Of course, a little low blood pressure, in and of itself, is no big deal, but that evening, Susannah developed a high fever, with low blood pressure, a very elevated heart rate and rapid breathing. The first call was made to the intern, who came in, saw Susannah VERY briefly and thought she looked fine; she shrugged her shoulders and left, but not before directing the nurse to go ahead and give Susannah her scheduled anti-hypertensive medications! After about an hour Amy called me to tell me what was going on, and that Susannah didn't look so good. The first thought that came to my mind was gram negative bacterial sepsis. For you non-medical types, this sort of infection can be lethal in a matter of hours, but on the flip side, with early intervention, can be easily averted. So of course, I was flipping out over the phone, half way across the country. Amy and her nurse recalled the intern, who this time consulted her senior resident, who decided that Susannah was appropriately covered by antibiotics (which was not the case!) and that NOTHING needed to be done. Not even increased observation! By now, I was livid and made them call the fellow. The response here was a little better, but far from optimal, until I chewed her out. She called Dr. Peters, who fortunately had just come on service, and he made them transfer Susannah to a floor where the nurse didn't have FOUR OTHER patients on a night shift! He was very concerned, and as such, Susannah was started on triple coverage broad spectrum antibiotics, given fluid resuscitation and transferred to 4A, where she could get ICU level care and observation as needed. By the next morning she was thankfully doing much better. We're still not exactly sure what was going on that night. Probably a combination of the Strept infection and too many anti-hypertensives. Strept, in and of itself, however, can release toxins which can mimic a septic type of picture as well. Fortunately nothing came of it. Susannah stayed on 4A for two days. Once we were sure that she was going to be okay, she was then transferred, again, this time to 5B (new carpet and all), which has been much better than 5A.

Luckily, I had planned on being there this weekend anyway, because I could tell, even last weekend, that Amy was near the end of her rope. I arrived Friday night and stayed until Monday. I think she got some decent rest, if not at the very least, some relief and a little company. Susannah looked really good. This last month in hospital has not kept her down. She could, however, definitely be casted as a baby on Planet of the Apes. The CSA hair is rampant! Our little fur-ball is really playful, standing, extraordinarily active, and chatting up a storm. She has about five different meanings for the word "ba", which are decipherable by pointing and context, and four meanings or "da". Best of all, she has a wonderful "Hiiiii", with a long drawn out "i". Very cute indeed. She has also once again discovered an old past time (from way back in December) of standing up in her crib and flicking on the (very bright) overhead exam lights, in the middle of the night ... perhaps this has something to do with Amy's lack of sleep! Truly, the most exciting news of the last week is Susannah's rekindled desire to eat! She is finally back to eating on her own and enough that she doesn't even need TPN anymore! Hooray!

One other medical issue that people have been asking about is the gancyclovir, and the issue of implants in Susannah's eyes. Dr. Peters is still lurking in the shadows with his plan of gancyclovir eye implants. I know this is kind of confusing, so I'll try to re-explain it. Back in March, we discovered that Susannah had vision threatening CMV retinitis, a viral eye infection. Now that she is on CSA and steroids for the chronic GVHD, and will be immunosuppressed for some time, she needs the drug gancyclovir to prevent recurrence of the infection. At present, she gets this five days a week intravenously, so that it not only goes to the eyes, but also to everywhere else in her body. As a side effect, gancyclovir adversely affects kidney function and also suppresses bone marrow function. Well, Susannah's bone marrow is having a hard enough time as it is after the transplant, but the gancyclovir makes it more difficult to produce platelets and to mount a cellular immune response, therefore making her even more immunocompromised. Thus, with the gancyclovir and the CSA and steroids, she is having a very tough time clearing her bacterial infections. Since she will have to stay on the CSA and steroids indefinitely, we would like to reduce the side effects of the systemic gancyclovir; one way to do this is to put gancyclovir impregnated implants in her eyes, which deliver very high doses in the eyes, but very little to the kidneys and bone marrow. Up to now we have been reluctant to do this because of the anesthesia risk, but I think that we are headed in that direction. The risk of anesthetic for Susannah is lessening somewhat with each passing day, and the risk of the systemic gancyclovir is only increasing. We were hoping to hold off until October, but now are potentially looking toward the end of August or early September. Before going this route, however, we have to be certain that her infections are TOTALLY gone, or otherwise the implants themselves could become infected, which would be a nightmare. One upside to this scenario is that when we do this, we will likely do all of Susannah's one year post BMT tests, and may avoid having to return to Minnesota in October, which is right around the corner! This plan is still in evolution and far from being totally thought out, so we'll be sure to keep you all abreast of this issue, as it will surely come to the forefront sometime in the next month.

As for the ever gregarious Madeline, do I have a story for you! Shortly before her recent trip to Boston, Madeline somehow became fixated on going camping. She wanted to sleep in a tent. Enter stage left, our Care Partner, Sue. Sue decided that this was a task fit for her! In the process of planning one of her famous "adventures", she realized she didn't have any camping equipment, particularly a tent and this to Madeline was the most important part. This little glitch, however, would never stop Sue; she and her daughter asked around to see if they could borrow one. No luck. A few days later, a friend of Sue's daughter called up to say that her boss at the Target corporate offices was donating a tent, a backpack and various other camping supplies for the "adventure"! Apparently she was so touched by the stories of Sue and Madeline and their "adventures" (and how Madeline REALLY wanted to go camping), that she came to the office the next day loaded up with all the gear for Madeline to keep, courtesy of Target! Such a touching example of the generosity and kindness that we continue to be blessed with, even from people that have never met our family. As for the actual camping trip, the campers spent the day setting up camp on the shore of Lake Minnetonka, playing in the tent, catching ants, swimming in the lake and roasting marshmallows and hotdogs over a campfire (never mind the 100 degree heat wave!). Leaving no detail undone, Sue even managed to swing a boat ride for the two of them! Madeline had an absolutely wonderful time and I know Sue did too.

As I just mentioned, Madeline the jet-setter, spent the last eleven days with Justin, Brianna, Wendy and Joe Calareso back in Boston. Again they stepped up to the plate in amazing fashion. Joe flew from Boston just to retrieve Madeline! Talk about going above and beyond the call of duty. Of course Madeline had a great time with their family. One of the highlights was a trip to Storyland, a theme park in New Hampshire, where Madeline actually got to meet Cinderella! She was completely star-struck and had a fabulous time. The kids played so well that Wendy tried to coax us into having Madeline stay for an extra week. Amy and I got a little selfish though, and brought her back to Minnesota yesterday. Actually, Wendy's mother, Patty aka Nana, flew her back to Minnesota for us. She flew through Chicago to Minneapolis, dropped off Madeline, then back to Chicago and then on to home in California. Again, absolutely amazing! I just can't speak enough about the overwhelming measure of kindness that has been continually heaped upon us throughout the past 16 months.

Which reminds me ... many thanks to Dana Jacobs and her friends (who also had never met us until last week), Martin and Mary Jo Stowe, for providing me with the American Airlines vouchers, which I used to fly back and forth to Minnesota for my last two trips. They were a Godsend!

Thanks again to everybody for your unending support! Peace and love to you all.

Until next time,

Klane, Amy, Madeline and, of course, Susannah

Tuesday, July 24th, 2001 ~ Day +288

Where are they?!?


Okay, okay already!

Sorry for the delay in updating. I suppose the question on everybody's mind is "WHERE ARE THEY?!?" Well, unfortunately Amy and the girls are still in Minnesota. I did go back at the beginning of the month with the intention of bringing them all home, but as Amy has pointed out, Susannah remains in charge, and quite frankly, doesn't appear to be in any hurry to come back to San Diego. I guess we can't really blame her since she has spent far more time in Minnesota than she has in California, so perhaps she just thinks that Minneapolis is home!

Here's the gist of the situation. As you all know Susannah developed a line infection, the week she was supposed to come home. Well, three days after pulling the line out, Dr. Saltzman put in a beautiful new line without a hitch. That was Friday, two weekends ago. Much to our dismay the infection stirred up Susannah's chronic GVHD, so the plan was revised. We would watch her for about another week in clinic, and then hopefully send her on home. As such, Susannah was discharged on Saturday the 14th and looked wonderful. Since I had to be to work on Monday, I flew back to San Diego alone that Sunday. When I got home, Amy called to tell me that Susannah didn't look so hot, but actually she was -- 100.9 degrees hot. So you guessed it, readmitted for another fever, and as it turned out, another line infection. On top of that, her chronic GVHD was still trying to rear it's ugly head, so we had to increase her steroids, causing her blood pressure go sky high and making her even more immunocompromised.

Because the line is brand spankin' new, and we're running out of sites to place them, nobody is in a hurry to put in a new one. Consequently, the plan has been to treat through the infection with antibiotics and hope the problem goes away. The issue is slightly clouded, however, since even in the presence of appropriate antibiotics, Susannah has managed to produce positive blood cultures. At this point, thankfully, there have been no positive cultures since Friday, and Susannah hasn't spiked a fever in over a week. So everything remains on course, and every day that goes by without a positive culture, is a step in the right direction.

At present, Susannah and Amy are still in the hospital. The blood pressure issue is finally getting under control. We had to increase the anti-hypertensive medication that she was on and then max out a second medication, with still marginal results. Currently, we are attempting to taper her steroid dose back to a maintenance level, at which point it should be much easier to control her blood pressure. With the flare in her chronic GVHD, we have had to restart Susannah on her antihistamine (Periactin), four times a day, as well as, numerous steroid creams. As for the cultures, we need at least three consecutive days of negative cultures to feel good about the infection being cleared, but we will likely want even more. We continue to check them daily. Since her last positive culture was Friday, that means that she needs negative cultures through Monday. It takes three to four days to really say that a culture is negative, so we need to wait until at least Thursday to really say that everything is good to go (keep your fingers crossed!).

As for Madeline, she has taken up residence with Joan ... thank God for Joan, she has truly been a miracle! Being with Joan, and her husband Gary, Madeline couldn't be in better hands. She and Joan are best buddies now, for sure, and their house is like a second home for Madeline. There are lots of kids for her to play with there, and Madeline gets to swim in the pool and eat "pastina" everyday! Of course we miss her, but Joan does take Madeline to the hospital each evening for some Mommy time. We are so grateful and fortunate to have had these two wonderful people come into our lives!

In light of the unexpected change in our schedule, the plan for Madeline is this: Wendy Calareso (actually her husband Joe) is going to fly into Minnesota this Thursday to take Madeline back to Boston for a week. Then Grammy is going to Massachusetts to retrieve the princess. They'll stop in Minneapolis for a few days afterward, and then Grammy will bring Madeline home, hopefully for good. With any luck, the other two will follow shortly thereafter.

We've been trying to remind Susannah that the weather in San Diego is much nicer than in Minneapolis, but so far she hasn't taken the bait. We'll keep trying to convince her!

All our love and best wishes,

Klane, Amy, Madeline and Susannah

Tuesday, July 10th, 2001 ~ Day +274

She’s Still in Charge!!!


Hello Again!

Just as I was shoring up the final details for our journey back to California, little Susannah decided to remind everyone of the most important detail of all ... SHE'S STILL IN CHARGE!!! The fevers began yesterday afternoon and have yet to subside. Never a dull moment!

Yes, you guessed it, Susannah was readmitted to the hospital early last evening! With her temperature holding steady between 103 and 104 degrees, even in the wake of vancomycin, et al, we waited on pins and needles for the results of the first blood cultures. By 8:00am this morning we'd received word of a positive culture and not thirty minutes later, "We had a plan!". I'll give you one guess who the doc on service is?!?

As only she could do, Dr. Davies had Dr. Saltzman up to the floor in the blink of an eye to yank the pesky line -- with the fevers persisting, in spite of the vanco, she was taking no chances. Once the Hickman was out, the Barney-singing surgeon, placed a peripheral IV line in Susannah's foot. She was a trooper, clapping and smiling throughout, never mind the two milligrams of Versed, he attempted to sedate her with!

Assuming that tomorrow's line-free cultures come back negative and Susannah's fever curve decides to trend downward (she is still spiking to the 102's), she has a date to visit the OR this week, after all. If things bode well, a new central line will be placed on Friday and Susannah will hopefully be discharged on Saturday. The plan is for Dr. Saltzman to put in the catheter, with none other than Dr. Bellani providing anesthesia, via an LMA. This should allow them to put Susannah to sleep and safely control her airway without actually having to intubate her. While the idea of going to the operating room is very unnerving, knowing that she will be in such capable hands does help ease the tension a bit.

Although we are anxious to get home, the fact that this happened while still here in Minnesota is really a blessing. With her complex history, it is reassuring to face this obstacle with the safety net of those who know Susannah and her quirks, inside and out. Besides, this way, she will return to San Diego with a brand new line, free of infection. A fresh start, if you will.

We tentatively have a flight scheduled for Sunday morning the 15th into Camarillo, California (it's as close as the Corporate Angel folks could get us) and believe it or not, Dr. Davies told us to hold off on canceling it just yet. Both she and Dr. Peters feel that it is not completely outside the realm of possibility for us to actually be on our way back to San Diego by then!

Love to all,

Amy, Klane, Madeline and Susannah

Monday, July 9th, 2001 ~ Day +273

Wrapping Things Up


Hi There,

Just a relatively quick note, to fill you in on the last couple of days. For now, pending flight arrangements, our plan is to tie up any loose ends and fly home later this week!

After much thought and discussion, we opted to forego the surgery on Susannah's eyes. The anesthetic risk was just too great. Dr. Bellani felt there was a ten percent chance that Susannah would experience complications, prohibiting her from being immediately extubated. Having been down that road before, where one day on the ventilator led to six weeks, from which she is still recovering, we decided to put the surgery off for now. After she has come so far, we were not willing to take that kind of chance, when there was another option available. Perhaps during her one year post transplant visit, when she will likely already be anesthetized for an MRI, we will reconsider. Especially because, if at that time she is no longer needing to be transfused (she went a record seven days between platelet transfusions this week, yippee!), we can replace her central line with a port then as well. Since the implants are only effective for eight months and have to be removed no later than a year from implantation, this actually works out best from a timing aspect. She will probably need gancyclovir coverage for close to a year and by placing the implants in October, that should bide us a little time, so as not to have to replace them. Also, this way they can be implanted when Susannah will already be "under" and removed the following October at her two year visit, saving her additional procedures.

On a more exciting note, Thursday's appointment with the ophthalmologist, was quite reassuring. Susannah's visual acuity is very acceptable and Dr. Christensen sees no reason, at this point, for her to need glasses. Now, I have saved the best for last. Despite her unfortunate luck with the CMV retinitis, Susannah does indeed have some luck in the eye department. Her corneal clouding resulting from Hurler Syndrome has improved with transplant! This is one of the areas that the transplant does not always benefit; more often than not, the children either show no improvement or get worse. In just nine months, the degree of Susannah's clouding has decreased by half. This is phenomenal news.

Susannah's GI studies last week looked basically normal. There is a bit of slowed motility in her esophagus, but that is somewhat expected and hopefully something that will get better with time. So, for now we'll just concentrate on reacquainting her with the desire to swallow textured foods. Everyone agrees that Susannah is actually eating far better than anyone could have imagined at this point, in light of everything she went through. So, perhaps we were just expecting a little too much, a little too soon.

Between this update and the last, I think I have now given you more information that you ever wanted on every organ system in Susannah's body! Aside from all of the medical jargon, our littlest princess continues to improve more and more each day. Susannah, is once again becoming Susannah. She is constantly showing us wonderful glimpses of, not only her returning, but also her new found personality. Her smile and her laugh are back with a vengeance and the joy she brings us is overwhelming.

Poppy flew in on Sunday, with plans on driving back to San Diego. The Explorer is packed to the gills and he is armed with his famous books on tape ... after we visit with him for a couple more days (the girls are not ready to let him go just yet) he will be on his merry way. We have accumulated a great deal of stuff over the last 15 months, so even after donating quite a bit, we will be shipping some back home as well.

More to come on our impending departure!

Love,

Amy, Klane, Madeline and Susannah

Wednesday, July 4th 2001 ~ Independence Day ~ Day +268

Happy 4th of July!


Happy Independence Day, Everyone!

In the name of patriotism, we decided to break the rules today. Susannah joined us to watch her big sister ride in the local Fourth of July parade! Now, don't think we were that brave, Madeline's grown-up friend Joan just so happens to live one block off of the parade route, making for a quick and easy getaway. Also, Susannah wore her mask the entire time and remained a safe distance from the crowd. Both girls were beside themselves, Madeline beaming as she rode by on her festively decorated tricycle and Susannah as she waved incessantly at her sister when she passed. Afterward, we returned to Joan and Gary's house for a traditional Minnesota lunch of "brats and beer", followed with an afternoon by the pool. It was a delightful day.

Susannah never ceases to amaze us and everyone around her these days. Last week she began pulling up to stand and is now cruising everywhere, transitioning from one thing to another. To the observer, her legs still appear very weak, but she manages to maneuver herself around anyway. This most recent development makes for quite an interesting bedtime. Where before she would obligingly lay down and go to sleep, she now prefers to stand up and move from one side of her crib to the other, paying no attention whatsoever to the two IV lines and pulse oximeter she is attached to! The other night it took her five hours to go to sleep and I untangled her at least ten times. She has also, once again, become smitten with her big sister and is completely obsessed with saying "hi". We continue to count our blessings.

In fact, after her evaluation by Neuropsychology this week, they determined that she had gained three months in development, in just three weeks time! Both the neuropsychologist and the neurologist were ecstatic with her progress. As you may recall, Dr. Charnas only came to meet Susannah back in February, so this visit was really the first time he had the pleasure of truly knowing her; he was just beside himself at her recent transformation. Of course, she is one of a kind, as no other patient has had quite the same course, so they were unable to make any real predictions. However, we were given much hope and will just take things one day at a time.

Speaking of specialists, after a very hectic week of appointments, here are the other reports:

Cardiology: Overall, Susannah's heart looks healthy with no major areas of concern. The PDA that was seen in October, and on several subsequent echocardiograms, was not evident this time, but it is possible that she was just moving too much for it to be detected. This will be monitored.

Pulmonology: Susannah's lungs continue to improve and with her increased activity, not to mention the steroid therapy for chronic GVHD, they should only get better. Dr. Milla did say, however, that once she is tapered off the current steroids, he suspected she may require some sort of inhalant steroids to keep things in check. He recommended that we continue supporting her with supplemental oxygen as necessary and did caution us that any increase in oxygen requirements, accompanied by elevated respirations, would warrant an immediate trip to the Emergency Room for a full pulmonary work-up.

Endocrinology: The plan remains to support Susannah with synthroid (synthetic thyroid hormone replacement). In a child her age it is far better to overshoot than to skimp, which can inhibit brain development. So for now we will keep with the medication and follow her labs accordingly, while addressing the issue of an experimental wean at a later date.

Otolaryngology (ENT): Susannah's ears look fine and there appears to be no permanent damage to her airway from the prolonged intubations. The scar tissue in her upper airway has improved significantly. Dr. Rimmel feels that her voice, although still soft and raspy, should only improve with time.

Gastroenterology: It seems Susannah may have a touch of reflux, as she is showing several symptoms, including her discomfort in swallowing textured foods. There was evidence of this on her upper GI series back in May, but until recently it seemed to remain in check. Tomorrow, she will undergo a swallow study and esophogram to see if any further information can be obtained in that regard. Her lack of interest in swallowing solid foods (she will take in almost anything, but then chews it up and promptly spits it out), leaves her getting the majority of her oral calories from whole milk. Thus, we are all but resigned to the fact that her IV nutrition will accompany us home to California, at least for a short while.

Dermatology: Susannah's skin looks wonderful and the itching has almost totally subsided. In fact, she was even able to be taken off the Periactin last week. We have also cut back on her creams substantially. It appears that the chronic GVHD is now under control. As for the hair situation, after leaving the hospital as a virtual cue ball (no eyebrows, eyelashes or hair to speak of), the cyclosporine (CSA) kicked in once again and is quickly producing a little teddy bear, back hair (yes, it's back!) and all. She does have a strange bald spot on her now VERY thick eyebrows, along with lessened growth on either side of her head (if only we could transfer the black fuzz from the rest of her face to these areas!). For now, Dr. Hordinsky will take the conservative approach and just watch and document with photographs, since the usual treatment for such patchy hair growth is actually CSA!

Regarding Orthopaedics, I suppose this specialty warrants it's own paragraph in our household. As a matter of fact, I am not even going to attempt to relay the information, so for the remainder of this topic, I'll turn you over to Klane ... For the time being Susannah's orthopaedic situation is stable. Yet, as time passes and we get past this first year post transplant, ortho issues will enter the forefront of Susannah's challenges. Of primary concern are her hips and spine (these, as you may recall were the original two anomalies leading to her diagnosis). As evidenced by her x-rays last week, already she has significant underdevelopment of her hips, which according to Dr. Ogilvie, will require surgery in two to three years. Her spine deformity, or gibbus, has also worsened some in the last year. We fully expect this to progress, eventually requiring a fairly involved surgical treatment as well. The hope is to delay spinal surgery for as long as possible to allow her body maximum growth potential. In Hurler Syndrome, the hands and knees are also commonly affected, but for now Susannah's look relatively stable. She does have a locked trigger digit in her left thumb. This and an EMG will likely be addressed when we return to Minnesota in October for her one year post transplant visit.

As for the reports from Ophthalmology, this is a somewhat complicated issue. First for the easy part, Susannah will have her eyes examined for visual acuity tomorrow morning by the pediatric ophthalmologist, Dr. Christensen. Now, for the more complex issue of her CMV retinitis. For starters, I am happy to report that the CMV remains inactive in both retina, so apparently the gancyclovir is doing it's job. Now this is where it gets tricky. The Infectious Disease service suggested yesterday that Susannah have gancyclovir implants placed in her eyes, in lieu of the IV gancyclovir therapy she is currently receiving. In light of Susannah's ongoing immunosuppression, she will need to continue some sort of gancyclovir treatment for a minimum of six months, in order to keep the CMV retinitis in check. Additionally, she will need to have her retina monitored monthly at least.

Dr. Peters feels that the implants would preserve her long term kidney function and better maintain Susannah's marrow reserve (in the case of an overwhelming infection), while simultaneously helping to reduce the number of blood and platelet transfusions she is currently receiving (platelets every four to six days and red cells every two to three weeks). Not surprisingly, this, like everything else with Susannah, is a topic for debate. Dr. Davies feels that Susannah's body has handled the gancyclovir extremely well thus far, and does not anticipate a change in this area. Her platelet situation has only improved with time and her white count has never really been affected. Not to mention, her marrow has more than proven itself with it's extreme reaction to her chronic GVHD. In this case, she strongly believes in the old adage, "If it ain't broke, don't fix it!". As you might imagine, we are very torn between two people we trust implicitly.

While the implants would certainly simplify Susannah's care back home, they are not without risk. First and foremost, both the implantation and removal of the devices would require general anesthesia, via intubation. Obviously, this is no small issue for Susannah in her current state, never mind the added anesthetic risks already associated with her disease. On top of this, there are a few ophthalmologic risks involved as well. Neither option is ideal. Therefore, tomorrow we plan to meet again with Drs. Peters and Davies, as well as, Dr. Bellani, the chief of anesthesiology and Dr. Olsen, the vitro-retinal surgeon to further discuss the risks and benefits as they pertain to Susannah specifically.

Tentatively, she is on the OR schedule for Tuesday morning, the 10th, but we have certainly not committed ourselves to this route and very well may opt against it. Should the procedure take place, Susannah would need to remain in Minnesota between one and four weeks post-op, depending on who we arranged to follow her back in California. Fortunately, there happens to be a CMV retinitis specialist at UCSD, so the time frame would likely be the former, assuming everything goes smoothly.

On that note, we have been striving toward a departure date of July 11th. Health-wise, Susannah should be ready and everyone here has been working like mad arranging for all of the necessary specialists, homecare, in-home therapies, equipment and supplies, back in San Diego. However, we have run into a few other obstacles this week. First, the obvious glitch being the gancyclovir issue, which will hopefully be resolved soon. Secondly, we plan on flying back home with Corporate Angel Network, yet they seem to be having a lack of fights to Southern California next week. We have certainly not given up hope, however, since they were able to arrange that amazing, last minute flight for our trip out here last October. Keep your fingers crossed, as not only will it be difficult to arrange commercial tickets at this late date, but Susannah's level of immunosuppression makes a plane full of strangers less than desirable.

Now for the big obstacle! It was determined last week that the back section of our house had some sort of leak this past winter, which has now produced a mold, none other than Aspergillus! This could be lethal for Susannah so, in no uncertain terms can she be anywhere near the house. Obviously the fungus has to be removed; this will be no small task and will likely involve some tear down and reconstruction (another thing Susannah is prohibited from being around). Currently, our homeowners’ insurance is assessing the situation to determine what sort of leak caused the damage and whether or not it is covered. Regardless, we will have to proceed, and no matter how quickly things can be lined up, it is unrealistic to expect the work to be completed anywhere near our tentative return date. Ugh! As such, Klane visited the Ronald McDonald House in San Diego, and somehow got them to agree to allow us to stay there, even though Susannah will not be an inpatient and we live only five miles away. Obviously, I would much rather leave our apartment here in Minneapolis for the comfort of our own home, but at least this way, we will be one step closer. On the bright side of things, Susannah and I will still be able to walk to and from the hospital for a little while longer and Klane will be working right across the street!

Regarding the home-front, Klane headed back to California last week to begin his fulltime research at Children's Hospital (although he did fly back in to celebrate the holiday with us and watch Madeline in the parade), so he was able to be on hand to facilitate the insurance adjusters, leak detectors and the like. I was fortunate to have Grandma Dona here for ten days to help me juggle all of Susannah's appointments and readjust to life here without Klane's help. Having him around these last few months has really spoiled me. During her visit, Grandma Dona and Madeline hit all of the usual hot spots, including a new favorite, the Dinkytown Park wading pool. Madeline continues to keep us all going with her inspiring energy and she continues to make us laugh with her silly antics and hysterical one-liners. I can't resist sharing two of her most recent quotes: After walking across a wobbly wooden suspension bridge constructed by the Boy Scouts at today's Fourth of July festivities, she returned to tell me, "Mommy, I just climbed on a Boy Scout! Can we build a Boy Scout in our backyard?" and last week while she was sitting on the potty, I asked her if she was going poo-poo, to which Madeline replied, "No Mommy, I'm having a bowel movement. That's poo-poo in Italian. Joan teached me that." Of course, Joan, who does study Italian, denies anything of the sort!

We will certainly keep you all abreast of things as they transpire around here. With any luck, we should have some more definite answers by week's end. As always, thank you for sending your thoughts and prayers in our direction -- which will hopefully be West very soon!

Much love,

Amy, Klane, Madeline and Susannah

Tuesday, June 19th, 2001 ~ Day +253

Our little blossom ...


Hi Everyone!

Well let me tell you, the last two weeks have been pretty amazing. Since being discharged, Susannah has made incredible strides toward getting back to her old self! Every day she blooms more and more.

Within three days of returning to "temporary house", Susannah began to crawl, again. Better yet, this week, she is actually standing on her own two feet with very little assistance and is able to take a few steps around the coffee table (this is now the fourth time in her life that she is on the road to walking). She has also nearly mastered the art of pulling up to stand and spends a lot of time on her knees, grabbing things off of low shelves or tables and tossing them every which way.

In addition to all of the physical activity, Susannah's personality is finally returning to that of the little girl we know and love. Happy, smiling, giggly and just a bit stubborn! And, she definitely still has the beat ... at even the slightest hint of music, we find her dancing and bobbing. It has been wonderful to hear her laugh and smile again. Really, we don't even mind her waking up in the middle of the night to play with the musical toys in her crib! I guess she just needed a change of environment.

Cognitively, Susannah has been a little slower to rebound, but this was somewhat expected. Regardless, she is indeed making progress. Her vocabulary has picked back up to "Dada" (of course!), "baba" (which we think may be her latest version of Mommy), "hi", as well as, a slew of other cooing and babbling sounds. Her sign language is also showing some small signs of returning. She seems to be signing "more" and "want", and she certainly knows how to point and grunt for anything in sight that she might find desirable. She has also re-achieved several important developmental milestones in the last couple of weeks. She is now able to put objects into a bucket and place rings on a stacker. Currently, we are working on mastering the shape sorter and trying to relearn animal sounds. All small potatoes for most two year olds, but very significant achievements for Miss Susannah.

I almost forgot! Even more good news is the report from Susannah's six month engraftment studies. She continues have 100% donor marrow, with a phenomenal enzyme level of 68. Geez, mine's only twenty-something; maybe she could donate a little to me!

Life on the outside has been sufficiently hectic. Susannah now goes to clinic about three or four times a week. It was a nice reunion with the BMT clinic staff, who were happy and quite relieved to have Susannah back in their domain. As you know, Susannah is on a pharmacoepia of meds, which she takes throughout the day, as well as needing TPN every night (we are still working on getting enough calories into her by mouth). Additionally, Susannah has three hours of therapy each day, Monday through Friday. Fortunately, Susannah has continued with the same therapists (Jane - PT, Jim - Speech, and Rebecca - OT) as an outpatient that she had as an inpatient. She knows these three very well and responds wonderfully to them. Indeed, Susannah keeps us busy, but given her outstanding progress, we are more than happy to oblige her.

Hectic or not, we are just all grateful to back in the "luxurious" apartment again. Pretty scary when dorm-style cinder block walls, industrial carpet and two twin beds pushed together seem plush! Imagine what home on Mount Tami Drive is going to feel like. Amy has been away for nine months straight (and fourteen months combined) -- I think she has forgotten what home is like. Hopefully that sweet reunion is on the horizon.

On the social front, Madeline, our star socialite, performed in the long awaited ballet recital. The girls were absolutely adorable! Five four year old girls danced beautifully and proudly, to nothing other than, "Thank Heaven for Little Girls", in front of what must have been at least 300 people. Not a one of them even winced! They were very "bwave".

Madeline has been spending one to two days a week with her "grown-up friend" Joan, who it turns out, has a swimming pool. Although summer has been slow to present itself in Minnesota, the weather has permitted numerous swim days. Joan has been instrumental in honing Madeline's swimming skills. Madeline seems to have met up with a contingent of kids from Joan's neighborhood, with whom she swims fairly frequently. We know that she must be having a great time, because these are the nights that she sleeps extra soundly.

Madeline was kind enough to swing an invitation for good ole Mom, Dad and Sissy to Joan's house, as well. We managed to get ourselves out for our first outing as a family in about ten months. Madeline and I spent the better part of a day cleaning and sanitizing the Explorer (Grandmother grime!) and packing all of the necessary gear for Susannah to make the trip. Joan and her husband, Gary, were very gracious hosts and barbecued some delicious steaks for us. Madeline and I swam in the pool, while Mommy, Susannah and our hosts watched. Susannah was so worn out from all the excitement that she passed out on their living room floor! We had a great time.

This past weekend, Amy and I managed to get away for a night to the quaint little town of Stillwater, just 25 miles northeast of here. It was our first ever "vacation" without the girls. Madeline went for a sleepover at Joan's house, while Susannah's long-time nurse, Kristy, stayed here at the apartment with her. Over the past umpteen months, both Kristy and Joan have come to know and love their respective charges inside and out, so both girls were in incredible hands and we could not have felt more secure. As my belated birthday gift (we had to wait until Susannah was stable enough to skip a day of clinic), Amy arranged for us to stay at a gorgeous turn-of-the-century bed and breakfast. Our room was amazing, with a real bed and everything. There was even a hot-tub for two! We walked into town for dinner and treated ourselves to an excellent French restaurant and lots of wine. Maybe too much! I think we were both a bit under the weather the next morning, if you know what I mean.

Tentative plans are underway for Susannah to bust out of here sometime in early to mid-July. We need to make sure that the steroids and CSA are doing their job on the chronic GVHD. So far, so good (in fact, this will mark her first full week without bolus steroids). Also, we need to make our rounds with each of the sub specialists over the next couple of weeks; literally twelve specialty appointments have been scheduled thus far. We'll be sure to keep you updated.

Keep your fingers crossed and please keep up all those thoughts and prayers. They are certainly doing the trick!

All our love as always,

Klane, Amy, Madeline and Susannah

Friday, June 1st, 2001~ Day +235

She’s been sprung!!!


Hello All,

She did it!!! After nearly six straight months in the hospital, Susannah is now back home at "temporary house"! She was discharged early yesterday afternoon and I cannot express how wonderful it is to have our little family back together under one roof.

A great deal of medical paraphernalia accompanied Susannah back to the apartment, some necessary and some precautionary. At the moment, she remains on supplemental oxygen and is being monitored very closely. Hopefully as the weeks progress, we will be able to widdle down the gear significantly. In the meantime, we are easily adjusting to our new found freedom and are busy reestablishing our daily routine of clinic visits, home nursing, oral and IV medications, as well as, outpatient therapy appointments.

Medically speaking, Susannah basically maintained steady state in the week preceding discharge. The one new development was a dramatic decrease in her blood counts: Sudden drops in hemoglobin and platelets, and lowered absolute neutrophil counts (ANC), necessitating growth factor (G-CSF). All fingers point to gancyclovir as the culprit, thus the doctors are considering reducing her maintenance regimen to five times per week, as opposed to seven. At his exam this week, the ophthalmologist confirmed that Susannah's CMV retinitis remains in check, so reducing the dose seems like a safe option.

In the last week alone, Susannah has progressed by leaps and bounds, from a neurological and social standpoint. When Madeline and I returned from our trip to San Diego (which by the way was wonderful -- thanks to all for making it such an enjoyable stay for us both), Susannah was almost a different child. She now shows great interest in her toys, claps her hands, points to what she desires, takes off her socks (ugh!) and babbles constantly. She is also regaining her strength at an amazing rate and is now pulling to her knees, taking steps with assistance and even riding her scooter, albeit backwards! We could not be more pleased with her progress.

Our departure from Unit 4A was bittersweet, with the emphasis, of course, on the sweet. It was difficult to say goodbye to the wonderful people who were such an integral part of our lives, especially these past several months. Many have become like family to us and we truly owe Susannah's life to them. We will miss seeing them everyday, but are anxious to move on to the next step of this journey.

As for a time frame for Susannah's return to San Diego, only time will tell. At this point, no one is willing to even speculate. Right now we will just continue to take things one day at a time and let Susannah's body tell us when she is ready ... we all know she does things her own way!

Love Always,

Klane, Amy, Madeline and Susannah

Monday, May 21st, 2001 ~ Day +224

The Wondergirl


Hi Everyone!

Time for yet another update on Susannah, the wondergirl. She's the wondergirl for many reasons, not the least of which is still being alive, but mostly because we continue to wonder what to expect next, and wonder when we are going to get out of here! Yes, we're still in the hospital. After tentatively planning on being discharged this weekend, Susannah decided to keep us all guessing by turning blue again last Monday evening. Thankfully, it was a much less dramatic demonstration than the major episode, which now occurred more than two months ago. Susannah's oxygen saturations dropped into the 50-60% range briefly and has since required more supplemental oxygen than usual. As a result, she had a CT scan of her chest on Wednesday that showed diffuse lung reaction, fortunately without signs of anything sinister. We think she probably contracted a virus that was too wimpy for anyone else to notice, but was enough to significantly impair Susannah's lung function. Now, apparently on the mend from this latest bump in the road, the new tentative date for Susannah's discharge from the hospital is May 31st. However, as we have grown accustomed to accepting, nothing with Susannah can be planned for ... the only certainty is uncertainty itself. So we will continue to take things one day at a time, while doing a little discharge planning on the side.

Fortunately, since last updating, there have been many positive developments as well. In the last two weeks, Susannah's itching has nearly ceased, her vomiting has stopped, she is sleeping through most of the night and she is really beginning to enjoy eating solid foods again. This has all correlated with a virtual disappearance of her crazy eosinophil counts. Another positive note is the report from ophthalmology that the CMV retinitis is now completely inactive. Most exciting of all is that in the last week Susannah has become much more interactive. She has smiled, given a couple of small giggles and even pushed up to a sitting position from lying, all on her own! It is safe to say that Susannah has officially lost her "rag doll" status. Yeah!

Many of these new improvements can be attributed to Susannah being restarted on steroids and cyclosporine (CSA) for what has been loosely termed chronic GVHD. Although not exactly classic graft versus host disease, in either the acute or chronic form, it was determined that Susannah has had enough symptoms of the chronic variety to warrant treatment. The usual course is eight rounds of high dose IV steroids given weekly, plus daily doses of oral steroids and CSA. This treatment can last for anywhere from two months to many years, depending on how Susannah responds to the therapy, as well as who you happen to ask.

As you might have guessed from the last update, this diagnosis was not made without a healthy dose of controversy. After nixing the idea of a lip biopsy, everyone was content to wait and see if we could make any headway in finding an allergen, to which Susannah may have been exposed. Meanwhile, as Dr. Wagner suggested, we also pursued a second skin biopsy to see if any further information could be gained in that regard. So, two weeks ago today, we took Susannah to the Dermatology clinic (don't get me started on why their service couldn't get their pansy #!@$! over to the hospital). The dermatologists were convinced that Susannah did NOT have GVHD of any sort from the appearance of her skin. Dr. Wagner basically forced them to do the biopsy anyway, to which they obliged. The next day the pathologist called up by phone and reported that the biopsy result was absolutely negative for graft versus host disease. One day later, she called again and asked, "Could this by any chance be GVHD?". UGGHHH!!! Apparently she had reviewed the slide with a second pathologist who thought it was actually consistent with chronic GVHD. Dr. MacMillan firmly agreed with the pathologist that Susannah has chronic GVHD, and as such, should be treated with the full year of therapy. After reviewing the slide himself, Dr. Peters' explanation was that it is not actually GVHD, but some variant of bone marrow dysregulation, allergic or otherwise, and that we should treat Susannah accordingly. Dr. Davies says it’s really a matter of semantics. Thus, we ended up with a compromise that makes sense to almost everyone: Start chronic GVHD treatment per the protocol, and if Susannah's body responds quickly (which it has already shown signs of doing), then we can consider tapering the treatment early, perhaps within a month or two.

There is of course a major downside to this treatment. Susannah will have to remain in Minnesota for at least as long as it takes to be sure that the chronic GVHD is under control and that her course is stable enough to transfer care to San Diego. As a result of her treatment, she will again become quite immunocompromised, much in the same way as she was many months ago right after her transplant. In essence, Susannah is starting all over again from square one in this area. Isolation and her mask, will continue to be a part of our lives for sometime. Also, she has already been restarted on anti-hypertensive medications to control the high blood pressure that is associated with the steroids and CSA. Due to the re-introduction of immunosuppressives, she will continue on gancyclovir throughout her treatment, in order to keep the CMV retinitis in check. Additionally, Susannah has been restarted on all of the other prophylactic medications associated with immunosuppression. At least she is MUCH more comfortable, and now able to concentrate on redeveloping all of the milestones that she lost with her illness.

Much to our dismay, Susannah's therapy for the chronic GVHD did not start in time to prevent her hair from falling out, AGAIN! One of the indications for her most recent diagnosis is hair loss. At present, Susannah has a Mohawk type hairdo from sleeping on her sides. We haven't yet had the heart to finish the job -- what is left of her new hair is already three inches long! When we finally do get out the clippers, rest assured that the CSA will kick in and supply, once again, all the hair she needs and then some. We had been thinking that one benefit of being here for so long would be that by the time we returned to California, Susannah would have shedded (literally) the side effects of her many medications, not to mention the drugs themselves! Needless to say, with hair, without hair and even with hair everywhere, we are just thankful to still have her with us.

This week we also bid a fond farewell to our friends, the Seabrooks, who are headed home to Pennsylvania. Tyler, also on his second transplant for Hurler Syndrome, is doing so well that Dr. Peters actually sent him home early! So, yes, incase you were beginning to wonder, people do actually get to leave this place! We wish Chris, Matt and little Tyler the best of luck, and look forward to seeing them again soon.

As for the bi-weekly social report, Grammy made it into town, just in time for Mother's Day. She was nice enough to stay at the hospital with Susannah and her nurse, while Madeline and I took Amy out to dinner. Hopefully next year we can all go out together! The weekend also brought another visit from our friend, Beth Kramer who drove up from Omaha with her mom. Madeline's biggest adventure of late was to see a performance of "Pippy Longstocking" at the Minneapolis Children's Theatre with her grown-up friend, Joan. Now we have to guess who she is on a daily basis, Pippy or Peter? The little stinker keeps all of us on our toes. Madeline has also been on numerous other play dates in the last two weeks, including farewell adventures with Kelsey and Michelle, two of her college babysitters. Sadly, most of Madeline's "big girl friends" are leaving for the summer, so she probably won't see them again for a long time, if ever. They have really become a part of her life here.

Speaking of Madeline and her friends, she is currently on a little vacation back in San Diego. She and Grammy flew out on Saturday and I will follow on Wednesday. We thought that Madeline could use a little time with her buddies, and I have a presentation to give next Saturday at UCSD. She is spending the early part of the week in the care of the Jacobs and the deBoucauds and is having a great time. The long holiday weekend should make it easier for Madeline and I to make the rounds and see everyone. Amy and Susannah will stay behind alone for the week. The expectation is that upon our return, Susannah will be discharged and we can all settle back into the apartment together. By then, we should have a better idea of what to expect in terms of getting everyone home to San Diego. I've learned better than to make predictions, but a nice goal to strive for would be to have the girls home by the Fourth of July.

Thanks for all the positive thoughts and prayers!

All our love,

Klane, Amy, Madeline and Susannah

Saturday, May 5th, 2001 ~ Cinco de Mayo ~ Day +208

Cinco de Mayo ... what’s that?


Hi Everyone!

Feliz Cinco de Mayo! Now that we have decorated Susannah's room here on 4A for virtually every holiday, tonight we plan on introducing a few of our Midwestern raised nurses to an old San Diego tradition ... the Mexican food is en route, but we're still trying to figure out a way to blend those margaritas up here!

Despite continued rumblings of discharge, little Susannah seems to have plans of her own. She apparently is not a child who is in a hurry to go home. Frustratingly, Susannah continues to have fevers and also vomits at least once or twice a day, with her favorite time being around 4:00am. After a four day fever hiatus, we stopped Susannah's antibiotics yesterday. Of course, last night she decided that it was time for another fever. Every time we do a trial without antibiotics, Susannah quickly obliges us with another fever. Unfortunately, each fever buys us at least another week in the hospital, so this is becoming a real pain in the @#$!

The debate about chronic graft versus host disease (chronic GVHD) continues. Despite a vast improvement over the last few weeks, Susannah's skin still has a "thickened" or "leathery" texture to it, and her eosinophil count continues to wax and wane, but is always high. For the most part, her hair seems to have stopped falling out, but is much thinner than it was. Also, after her eyebrows and eyelashes rubbed out, they have slowly started to re-grow. The itching persists, but is much less severe with a strict regimen of antihistamines and topical ointments.

Earlier in the week, Dr. MacMillan, who is now back on service, strongly suggested that we take Susannah to the operating room for a lip biopsy and a tearing test (Schermer’s test) to check for evidence of chronic GVHD. This, of course, went over like a ton of bricks. It seems that she wasn't clearly recollecting past issues involving anesthesia for Susannah. You might recall that Dr. MacMillan was the attending physician back in December when Susannah rushed into surgery, and look where that got us ... and still has us!

Dr. Peters strongly believes that graft versus host disease is not Susannah's problem and is firmly against taking her to the OR for any procedure, unless absolutely necessary. So, we did the tearing test at the bedside, which was very negative for chronic GVHD. For now we are watching and waiting. Since Dr. Peters basically vetoed the idea of a lip biopsy, it's likely that we will instead do a second skin biopsy Monday or Tuesday, in an attempt to resolve the issue. This procedure is done with only local anesthetic and is much safer for Susannah. We feel even better about this plan after talking it over with Dr. Wagner. Dr. Wagner, who is covering for the weekend, thinks this is the best test to answer the chronic graft versus host disease question, and he is the expert, after all. Dr. Peters, of course, is the expert on Hurler Syndrome and is very familiar with every nuance of Susannah's history. It's all about trust. If Susannah does indeed have chronic GVHD, she could be committed to a nine month course of immunosuppression with high dose steroids and cyclosporine. Blaaahhh! This treatment may not bode well for her CMV retinitis situation. So, we want to be absolutely certain that we are correct in the diagnosis before committing her such a course.

Otherwise, Susannah becomes a little more alert each day. Progress of note this week is her increased general awareness of the surrounding environment. She now turns her head when someone opens the door, kicks her feet with excitement and actually opens her mouth for food. As I am typing, she is sitting in her high chair eating saltine crackers on her own. Actually, she is putting them in her mouth, chewing them up and then spitting them out, but we won't complain! We are still taking Susannah down to physical, occupational and speech therapy each afternoon in the wagon. She really enjoys the ride, looking all around and taking in her surroundings. Yesterday, was a very, very big day, as Susannah actually put weight on her feet! It wasn't really standing, but leaning over a block and keeping weight on her legs. Still, it's the first "step" back to standing on her own two feet.

We enjoyed a visit this week from the Mannings. They flew in from San Diego on Wednesday and it is safe to say that Madeline and Samantha didn't stop playing and giggling the entire time; the two were virtually inseparable. Madeline played tour guide to the highlights of Minneapolis, which naturally included a trip (actually two!) to, where else, The Mall of America! Their family's visit was yet another example of the huge gestures of kindness that our friends and family have continually showered upon us.

For the upcoming week, we will again be alone until Friday when Grammy arrives. We are looking forward to seeing her and showing off all of Susannah's progress since her last visit. Our college babysitters are in the midst of finals, so we will keep Madeline busy here at the hospital with the various Child Family Life activities, visits with her friend Nancy, the librarian, as well as, with the Care Partner unit volunteers, who she affectionately refers to as her "Play Characters". Now that spring has actually arrived (yes, there are once again leaves on the trees and tulips a bloomin'), the playground downstairs will definitely be a hot spot!

And now, for the biggest news of the past week ... "A Nite for Susannah" was a smashing success! Amy and I would like to thank everyone who attended the benefit last Sunday in support of Susannah and our family. We'd also like to thank the many people who were unable to attend, but who we know were there in spirit. By all accounts it was a wonderful evening. I will now attempt to provide a recap of the entire event, but as those of you who attended will surely attest, my description can in no way do justice.

The Entertainment:
The event began with a slide show of Susannah, accompanied by the song "In a Heartbeat", which was written especially for Susannah. The presentation featured photos of her from birth to the present, especially highlighting the last year. From what we understand, there was not a dry eye in the house! Our heartfelt thanks go out to Nicole and Dave Franco and all of the other artists who contributed to the album, "Songs for Susannah". Both the album and the song, "In a Heartbeat" are indeed inspirational and a lovely tribute to our daughter. I don't think anyone left that evening without purchasing one of these fantastic CDs.

The Auction:
With over 100 prizes, the silent auction and raffle were phenomenal. Much fun was had by all, visiting and bidding on the many fabulous items in the silent auction -- apparently there were even a couple of friendly bidding wars! The prizes ranged from pieces of furniture to clothing certificates to weekend getaways at great hotels and dinners out, and just about anything else you can imagine. Many, many people generously donated and collected various goods and services for both the auction and the raffle.

The Faire:
While mingling with friends and taking in the auction and the evening's events, everyone enjoyed a variety of wines and delicious hors d'oeuvre, which were so graciously donated. The staff and management of The Westin Hotel, Horton Plaza in San Diego did an exceptional job and deserve tremendous thanks for their generosity in hosting this fabulous event.

The Guests:
There were approximately 150 friends and family members who actually attended the reception, not to mention the many others who participated from afar. There were people there from all aspects of our lives, including many childhood friends. A welcome treat was the appearance of Father Grant Abbott, who just happened to be in San Diego for the weekend. He spoke to the crowd about the past year here in Minneapolis with us and gave everyone a first hand account of the most recent Susannah happenings.

Last, but certainly not least, there are four very special families without whom the evening would not have been possible. We are ever so grateful to Dana and Warren Jacobs, Penny and Troy Thayer, Ruth and Rob Manning and Sharon and Gary Spencer for realizing the need and making the benefit a reality. They worked day and night in preparation for "A Nite for Susannah" and did an amazing job, leaving no absolutely detail undone. We feel very fortunate to have such a wonderful group of friends behind us. No amount of thanks will ever be sufficient!

Love,

Klane, Amy, Madeline and Susannah

Thursday, April 25th, 2001 ~ Day +199

Heading in the Right Direction


Hello Everyone,

Susannah seems to heed warnings. Just as the docs came around and began barking up the hydrocephalus tree again due to her sleepiness, she decided to prove them wrong. In the last week, our little sleepy head has suddenly become significantly more alert and even began babbling dadadadada and bababap bababap -- we have yet to determine whether she is referring to Mommy or Baby Bop with this last one ... I'll let you guess my opinion! Susannah also once again began taking little bits of food by mouth. She does continue to battle day to day bumps in the road, such as fevers, spells of sleepiness, breathing issues, strange blood counts, itching and rashes, but overall she is definitely on the upswing.

For the most part the experiment with removing the NJ tube in exchange for the NG feeding tube has proved beneficial. Susannah does continue to vomit about once each day, but this is most likely related to the sudden increase of volume to her stomach (which has been inactive for so long), rather than reflux from her intestinal tract. The vomiting is of a vastly different variety than the multiple wretching episodes of weeks past.

We have however, begun to slow down a bit on the amount of her daily tube feedings in order to encourage more oral intake. Yes, Susannah is eating again! So far the focus has mainly been on re-exposing her to various tastes and textures, rather than nutritional value. Thus, she has been dining on such delicacies as chocolate, kiwi, pudding, Texas Grill Fritos and, her current favorite, popsicles, with her new speech therapist, Jim (Maureen, or "Mo" to Susannah, is out on maternity leave). She has also been reintroduced to two of her old favorites, yogurt and Pediasure. When handed a spoon, Susannah will bring it to her mouth, as well as, drink from her sippy cup independently without prompting. Our hope is to, sooner than later, eliminate the tube feedings altogether and as such, perhaps eliminate the vomiting.

Susannah continues to have daily occupational therapy and twice daily speech and physical therapies. We did make a significant change this week, in that she is now allowed to travel off the floor for some of her therapy sessions. Each day, we load her and all of her equipment up in a little red wagon and take a jaunt downstairs to the Pediatric Rehab area for her afternoon sessions. This gives everyone, especially Susannah, a nice change of pace and scenery. Some days she is very sleepy, but on others she is fully interactive, pushing buttons, turning to her own name and kissing her dolls on command. While Klane and I are still chopped liver compared to Baby Faces and Barney, Susannah seems to have rediscovered her big sister. At times, she takes great interest in Madeline and even smiled several times over the weekend in response to her silly antics. While Susannah is still not making all of the appropriate connections, she is certainly making progress in the right direction.

As for the rash, while it now seems to have receded to her hands, feet, knees and head, it still is very much an issue. The itching continues, but Susannah has started yet another new antihistamine (Periactin) which seems to have calmed it a bit more. Dr. Wagner, who specializes in GVHD (as well as many other things), is now on service (By the way, we have managed to catch him three times this last year ... he is only on service twice per calendar year. We all joke that this isn't exactly a record to be proud of!) and is not yet willing to totally discount the idea of chronic graft versus host disease, although all tests so far have resulted in no hard findings. The only possible signs are thickening of the skin on her knees and hands, combined with the timing of the onset of the rash and the discontinuation of her cyclosporine (CSA); also, the increase of eosinophils in her blood counts. Susannah has had substantial hair loss (she has shed ALL of her furry CSA hair and then some, including her eyebrows and eyelashes), but this more likely the result of her incessant scratching, rather than GVHD. So for now we will just watch and wait, as the treatment for chronic GVHD is far from benign and not something to be entered into lightly. Without getting into a lot of detail, it is important to note that chronic GVHD, as well as its treatment, is a completely different beast than acute graft versus host disease, which one worries about earlier in the transplant process. In its chronic form, GVHD is a sort of auto immune disorder.

By the end of last week Susannah's counts had come down significantly, but this week they are back up in the 20,000 range with a high percentage being eosinophils. The cause of the rash and the presumably related wacky counts, remain the million dollar questions.

After being fever free for several days, last Friday Susannah was again taken off all antibiotics. In response, or perhaps independently, she promptly spiked two lower grade, yet "magical" fevers which eventually led to Tuesday evening's high spike and the restarting of double coverage antibiotics. She currently remains febrile. Naturally, all resulting blood cultures have been negative.

Although unlikely related to the current fever trend, we did receive word from the virology lab of a strange virus that has been brewing in Susannah's stool since January. Yes, I did say January! Apparently they isolated the little bug way back when, but did not alert anyone to its discovery because, well, they could not identify it. It has appeared intermittently since then and has been sent over to both the Minnesota Department of Health and the Center for Disease Control ... still no clue as to its identity. I suppose no one should be surprised, this is Susannah we are talking about after all. The Infectious Disease team, who is all too familiar with little Susannah, recommended a course of oral immunoglobulin (IgG) in hopes of killing off the mystery offender. There have been anecdotal reports where this has helped in immunosuppressed populations. The risks are slight and everyone agrees the therapy is worth a shot.

With regard to her breathing, we have managed to fend off the CPAP and Susannah continues to get by with oxygen via the nasal cannula. We just started yesterday with an experiment in removing her oxygen during waking hours. So far so good, but we remain cautious, as we have attempted this before only to have her lungs tell us to forget it. Overall, she has been maintaining very good saturations these days and often we find the cannula blowing into her eye balls with her sats still 95-100%. She does, however, continue to have periodic episodes of drastic desaturation, almost always while sleeping. Ultimately, we will experiment during naps and nighttime in an attempt to wean her from the oxygen completely. For now, though, we will take things slowly. We have also cut back on her nebulizers and BDs, to make things simpler on us once she is eventually discharged. So far Susannah seems to be tolerating these adjustments well -- her chest films are improving and her lungs sound great.

Yes, I did just utter the word, "discharged". Slowly, but surely we are moving in that direction. Later this week we will meet with Dr. Wagner, Cyndie, our nurse practioner and Satkiran (whom we affectionately refer to as, "Sat"), our all-time favorite BMT fellow, in anticipation of Susannah's eventual discharge from the hospital back to the apartment. The five of us will develop a plan of action for getting Susannah sprung! There are many details to be ironed out in order to make this a reality, but we plan to meet and establish the necessary goals to be obtained over the next couple of weeks! It has been a long four months on this floor so, as you can imagine, we are all very much looking forward to finally being back at "temporary house" together again, and of course, eventually back home in San Diego.

Well, "Birthday Week" was definitely exciting! We had a quiet little celebration for Susannah on Easter Sunday ... she appropriately had a cake decorated like a giant Easter egg and even enjoyed the tiniest taste of the frosting! Madeline's "Garden Party" the following Sunday was a big hit. Since her friends here in Minneapolis run the gamut, from "kid friends", to "big girl-college friends" to "grown-up friends", we had activities for all. While it was raining cats and dogs outside, indoors we planted flowers in hand-painted pots (thanks to Grandma Dona), decorated flower cookies (thanks to Sue Reichert), played "Pin the Tinkerbell on the Flower" (thanks to Paula, one of our college babysitters) and broke open a huge flower pinata full of goodies. We snacked on theme appropriate treats including flower shaped sandwiches (thanks to Kelsey, another of our sitters), terra cotta pots of fruit, baskets of vegetables and a garden birthday cake complete with a picket fence! Since her little sister could not be there to share in the festivities in person, at Madeline's request, we placed a photo of she and Susannah on the table ... in a garden frame, of course!

As much fun as Madeline's party may have been, I am actually the one who had the best birthday ever! Everyone around here decided I needed a little rest and relaxation, so Klane and all of our wonderful nurses (Kristy, Mary, Kent, Andrea, Melissa, Patty, Sarah, Erica and Jen) sent me for a day at the spa! After enjoying a massage, facial, manicure and pedicure, and thinking the day could not get any better, I returned to the apartment to hear, "SURPRISE!!!" Yes, Klane had orchestrated an absolutely amazing surprise birthday party! There was delicious food from our favorite Italian restaurant (Buca di Beppo), wine, music, decorations, cake, flowers ... he even sent out invitations! He left no detail undone and even made certain that there was trusted care for Susannah back at the hospital, so we could both fully enjoy ourselves. There was quite a turnout and everyone had a great time. Apparently he'd had this in the works for weeks and with lots of sneaking, whispering and cahooting with Sue, the college girls and our nurses, he managed to do the unthinkable, surprise ME! Oh, incase you're wondering, Klane only told Madeline about the party, AFTER I left for the spa!

As always, thanks so much for your continued support of our family. Both girls were spoiled this year by the many wonderful birthday cards and gifts sent by those of you near and far. Although your gestures were very unnecessary, thank you for thinking of them.

All our love,

Amy, Klane, Madeline and Susannah

PS ... Susannah made the front page! Yesterday, The North County Times featured an article on Susannah and this Sunday's benefit in her honor. You can read it by clicking on the following link:

http://www.nctimes.com/news/2001/20010425/c.html

Sunday, April 15th, 2001 ~ Easter Sunday ~ Day +188

Happy Easter Birthday!!!


Happy Easter from Susannah the Birthday Girl!

Today Susannah turns two! We are celebrating Susannah's big day and the Easter holiday from the confines of Unit 4A, Room #7. Of course, Amy has prepared wonderful Easter baskets for the girls, and Susannah will have a beautiful birthday cake and loads of presents. My mother will be bringing over Easter dinner this evening, which we will share as a family here in Susannah's room.

The Easter Bunny was very smart this year and managed to visit both "Susannah's work" and "temporary house". Madeline awoke bright and early this morning to a trail of carrots and an elaborate egg hunt, which she loved! She had decided earlier in the week that she wanted to have her Easter basket with Sissy, so the Bunny dropped those off at the hospital. After a breakfast of Easter eggs (she has been dying them for two weeks!), Madeline joined her "grown-up friend", Joan, for church at Saint Matthew’s, followed by a brunch back at Joan's house. After a quick nap, she will return to the hospital with Grandma Dona for our Easter celebration and Susannah's birthday party.

As for Susannah, the last two weeks have resulted in only baby steps of progress. We can at least say that she is heading in the right direction! Most frustrating is her very limited level of awareness. We still await the bubbly, smiling, ever so chatty ("signy") little girl that we knew four months ago. Susannah's sleep-wake cycle is getting close to normal, but what she is doing while awake is a far cry from who she really is. She spends the majority of her awake time fidgeting and scratching. She seems to intently watch any of the numerous videos we have here, such as Barney, Baby Songs, Baby Shakespeare and so forth. What is concerning, however, is that she fails to have any real awareness of people around her. Even if we get right in her face, there is only a blank stare. Of course this is breaking our hearts, but all we can do is wait and hope that she comes around. We miss her dearly! Today we will celebrate her second birthday. She will have no idea what is going on, and unfortunately will not get to enjoy any of the usual festivities that a normal two year old would, like blowing out candles, eating cake, opening presents and playing with her new toys.

On a brighter note, we have received several pieces of good news. First of all, Susannah's donor engraftment remains strong at 100%! The chances of her losing her engraftment at this point are close to nil. The second piece of good news is that Susannah's hearing has improved. It seems that the transplant has rectified the worst aspects of her hearing loss, specifically in the right ear. She will still require hearing aids for good speech development, but her hearing loss is now only very mild in either ear.

The breathing saga goes on. Susannah continues to require supplemental oxygen. In this regard, she actually seems to have gotten worse. Her baseline oxygen saturation levels have been on the decline this week. She still has significant desaturations while sleeping. Over the past two weeks she has undergone a battery of tests and consultations for her breathing. She has had two formal sleep studies, which indicated that Susannah probably has obstructive apnea. Dr. Rimmel (our ENT here) saw her in clinic last week (it took two nurses, Amy, myself, two monitors, an oxygen tank and a wagon capable of pulling an IV pole to transport her!) and said that Susannah had significant collapse of the soft tissues around the back of her throat. From the appearance of things, he thought that this problem was temporary and should resolve itself within a few weeks, and perhaps was related to her current eosinophilia and fluid issues. Her weight has been up significantly and she has continued to have problems with extra fluid in the soft tissues of her skin and presumably her throat. In effect, when she sleeps deeply, she has trouble generating enough pressure to overcome the collapsed tissues in her upper airway. He recommended putting her back on a pressurized mask, the old, dreaded BiPAP/CPAP, for the mean time. He said that if Susannah were healthier, he would have recommended operating on her to remove the extra tissue (a UP3 for you medical folks). He kept mentioning the fact that she may tire out and need to go back on the vent. When he saw her, though, she looked worse than she had in weeks -- with the fluid overload and lower oxygen saturations, the vomiting, as well as her being tired at that time of day. Since then we have only had her on nasal cannula, foregoing the BiPAP due to her vomiting issues. She doesn't seem to be overtired with this approach, and if it truly is only temporary, this is a hell of a lot easier.

Having mentioned the throwing up, it appears that we may have finally found an answer to the problem. Don't hold your breath though, because it has only been about 24 hours since she last vomited. In an act of desperation, Dr. Peters called in the gastroenterologists. On Friday, Susannah had an x-ray GI study done to evaluate how things move through her gut. It turns out that she has phenomenally fast transit of gut contents in the proper direction -- South, if you know what I mean. The one interesting finding was that she pooled some of the contrast just past her stomach and some of the dye seemed to be slipping backward into the stomach around her feeding tube, the NJ. So yesterday we decided to experiment and removed the NJ and started feeding her through an NG, which goes only to the stomach and not past, into the small intestine. So far, so good! If this approach fails, then we may be talking about taking Susannah to the OR for an upper endoscopy and biopsy, i.e. a fiber optic scope to take a tissue sample. The biopsy could tell us if the eosinophils are affecting her stomach in the same way that they are affecting her skin, thus making her nauseous. This procedure would require general anesthetic, and of course, given the ramifications of Susannah's surgery in December, we will avoid anything of the sort, if at all possible.

Susannah's skin has dramatically improved over the last week. Since our previous update Susannah's skin became much worse. Initially she just had a rash and itching, but as it seemed to persist, the dermatologists were called to come and biopsy her skin. They took a small piece of skin, which didn't really give us much information. Susannah continued to peel all over; her elbows and knees were becoming like leather and she was getting really slimy. Virtually every place she scratched, she would then bleed. Her white cell count was as high as 35,000, with 12,000 being eosinophils, so the immunologists were called. They reviewed her case, made a few random suggestions and then punted to the dermatologists. The derm folks were re-consulted, since they had arbitrarily decided that they didn't need to see her anymore. It was a little frustrating to see them work. They came into the room, for the second time, knowing nothing about Susannah or her history. They didn't even want to look at her chart. Of course, this set Amy on fire! The attending, who came back with the resident later that day, tried to explain that "they didn't want to bias their exam by reading the chart. This is how dermatologists are trained to work". So anyway, what they saw, they pinned as eczema. Regular old eczema, with an early bacterial superinfection causing all the sliminess. Quite frankly, everyone was and still is pretty skeptical of their diagnosis, but the regimen of topical ointments and creams (especially an immunosuppressive ointment – FK506 or Protopic) that they prescribed, as well as, the bathing regimen made a dramatic improvement. Although she looks much better, her eosinophil counts continue to linger, albeit at much lower levels, and the rashes on her hands and feet remain persistent and itch incessantly. We will keep a close eye on this and hope it goes away, while continuing to search for an answer. There is still some suspicion that this may all be related to an antibiotic allergy, particularly to one of the cephalosporins (penicillin relatives).

Over the last two weeks, Susannah has also experienced some drastic swings in her body temperature, going from one extreme to another. Most recently she has been spiking fevers, but prior to now she was so cold that we had to resort to warming blankets and devices. After she dropped her temp to 93.1 degrees, despite warming, we called in the endocrinologists (yes, I do think Susannah has now been seen by every subspecialty in this hospital!). They studied her labs, ordered some others and discovered that she had increased TSH and decreased T4 levels, both indicating that her thyroid function was on the low side. As such, Susannah is now on synthroid for thyroid replacement. This seems to have rectified the problem, but they will repeat the labs again in a few weeks to determine if it is necessary to continue the therapy. Naturally, questions are still circulating as to whether her hypothermia was in some way related to the eosinophilia.

Another breath of good news is Susannah's response to the gancyclovir. The ophthalmologists returned again this week and said that the CMV retinitis is resolving. She is now back down to once a day dosing of gancyclovir, but will remain on the poison indefinitely. We can deal with that any day over her going blind!

This coming week promises to be entertaining. With Amy's and Madeline's birthdays yet to come, there should be plenty of excitement. Amy has been busily planning Madeline's "Garden Party", with lots of help from Grandma Dona and our Care Partner, Sue. It is sure to be quite festive! Madeline is very much looking forward to her turn as birthday girl ... we have had many a conversation this month about how Susannah’s birthday falls on Easter, and she too wants her birthday to be on a holiday, specifically Easter. I guess that Earth Day doesn't quite cut it for Madeline.

Amy and I did manage to get out to celebrate our 9th wedding anniversary. We had a wonderful dinner at Minneapolis' finest seafood restaurant, The Oceanaire. I know most people wouldn't equate Minnesota with seafood, but this was truly some of the best seafood I have ever had. I should note that this dinner out was a Christmas gift from Father Abbott and Saint Matthew's, so to them, thank you again. It only took us three months to get out and use it!

My mother has been visiting for the past two weeks and will be flying home on Tuesday. This marks her third trip in the last three months, all of which we couldn't have managed without! We will then be on our own for a bit until our next visitor arrives, sometime after "A Nite for Susannah". As many of you are probably aware, our friends back home are hosting a reception and silent auction benefit, in honor of Susannah on Sunday, April 29th. Thus, most of our usual visitors will be remaining in San Diego to attend this wonderful event on Susannah's behalf.

Wishing all of you a joyous Easter!

Love,

Klane, Amy, Madeline and, of course, Susannah

Sunday, April 1st, 2001 ~ April Fools Day ~ Day +174

An Anniversary of Sorts ...


Hello All,

It was one year ago today that we arrived in Minnesota. Last April 1st we came here anticipating the usual post transplant stay of 100 days, boy were we in for quite a surprise. Ironically, today also marks the hundredth day of this admission alone -- Susannah has been in the hospital for 100 days straight since December. I tell you all of this not to complain, but because it is incredible how time flies. Now, with Susannah's continual improvement, albeit at a slow but steady pace, there is actually light at the end of the tunnel. With any luck, we'll be back home before we know it.

We left San Diego exactly 365 days ago, never dreaming what would lie ahead. We were both excited and scared at the same time -- we knew what to expect, but then again we didn't. Our lives have since changed in ways we could never have imagined. We have learned so much this past year. We have learned to take one day at a time, be grateful for the little things and especially each other. We have learned to take nothing for granted and to be thankful for what we have. We have seen so much and met so many. We braved our first Minnesota winter, and actually learned to enjoy it. Spring is upon us and it is almost sad to see the snow melting. We have met many new friends with whom we have shared much joy, but also much sadness. Our lives are richer for it.

In spite of everything, the challenges of this year have also brought happiness and tender moments we will never forget. I would, however, be lying if I said it has been easy. All in all, the last twelve months have flown by. It seems like our "five week vacation” to California last summer was a lifetime ago. The fact that Susannah is already Day +174 from her second transplant and Day +339 from her first, is almost unthinkable. It feels like just yesterday that we were out ordering birthday cakes for the girls at a bakery in Uptown Minneapolis ... who'd have thought then that we'd be about to do it again. When we arrived here Madeline was a mere toddler, still two and using a booster chair at the dinner table. She has now grown into a little girl, four in just a few weeks, who has given up her car-seat in lieu of a seat belt booster. Madeline has become a wonderful, compassionate little lady right under our noses and we are very proud of the way she has handled everything. Then there is Susannah. She was admitted to the hospital for her first bone marrow transplant only two days after her first birthday and now it appears she may well spend her second as an inpatient. She has been hospitalized for more than one-third of her short life. The Twin Cities and The University of Minnesota have truly become her second home. She has endured so much and done very well through it all; she is an inspiration. When we arrived here, Klane was a third year resident, is still a third year resident and will be for the next two years. Over the past year, he has lived in two states simultaneously, traveled thousands of miles in between, put his career on hold and missed some things I know he wishes he hadn't. As for me, I have essentially been relocated to the Midwest, for the most part without my husband, leaving behind my happily hectic life as a mommy in exchange for the new role of mother, nurse and social worker all wrapped up in one. I have seen my children through some times that I would wish on no other mom, but the rewards have been well worth it.

Back in December, I jokingly established a goal of returning to California by Valentine's Day -- that way we could say we spent at least two holidays at home this past year. After Susannah's surgery, the goal was quickly amended to Saint Patrick's Day, hoping then for one holiday, which at the time seemed realistic. Well, April is now upon us, as is Easter, our 9th wedding anniversary and of course, all three April birthdays ... there is something to be said for familiarity of one's surroundings! These are not sad things to us, I suppose they could be if we let them, but instead they are the things that make us stronger, the things that keep us going.

There is a something else that keeps us going, perhaps the most integral component ... all of you. I know we have said this many times before, but no matter how often we utter the words, it will never be enough. The strength, support and love we have received and continue to receive from our family and friends, near and far, never ceases to amaze us. We have been showered with an outpouring of kindness and generosity this last year that has been absolutely amazing and we could not have managed without you.

Would we do it all over again? In a heartbeat!!!

With love from Minnesota,

Amy, Klane, Madeline and Susannah

Wednesday, March 28th, 2001 ~ Day +170

Busy Little Girl


Hi Everyone!

Well, everything has been pretty quiet around here ... until yesterday.

Big news -- Susannah woke up! Yes indeed, for about three or four hours yesterday, Susannah's eyes were wide open, she was watching Barney, kicking her legs, waving her arms and SMILING. This was by far the most alert that she has been since before Christmas! Needless to say we were all very excited. Kent and Kristy, two of our favorite nurses, saw to it that all of the nurses on our team, made it in to see her awake. Kent was even standing outside of the room waving people in as they passed by! It was a complete gawking session; no one could believe their eyes.

Unfortunately the remainder of the day yesterday was very busy for little Susannah. She was poked, prodded, fitted, futzed with, and even went on a little excursion, all for which she received repeated doses of sedation. Thus, her energy quickly fizzled and she snoozed the evening away. Her sudden wakefulness was, however, truly amazing, shocking, relieving, and a slew of other adjectives while it lasted.

Backing up a bit, Susannah had a relatively calm week last week. She did create a little commotion when she decided to take up the new past time of vomiting. It has been a bit puzzling to all, since she really doesn't have anything in her stomach. Poor baby had some pretty miserable nights, one in particular where she threw up 19 times! This unexplained bout of nausea, caused her to have yet another tube placed down her nose ... this time an NG (naso-gastric, as opposed to the NJ into the jejunum from which she is fed) to help drain the junk she was throwing up. This combined with several stomach motility meds, seems to have helped a bit, although she continues to gag incessantly. Poor little thing has been pretty miserable these days.

During the past week, Susannah also began to have a tremendous amount of skin breakdown and several different rashes on her body. It seemed that each day a new rash appeared, totally confusing everyone. She also began itching like crazy and peeling all over. Yes, peeling, or molting as Amy refers to it. Sort of like a lizard shedding her skin, although Dr. Davies chastised us for this comparison, saying she prefers to think of Susannah as a bear cub shedding for the spring. Anyway, all of these skin changes are, for the moment at least, being attributed to a likely drug allergy. It remains up for debate whether the allergy might be to Bactrim or drugs in the cephalosporin family (Ancef, ceftazidime and a prior related suspect, penicillin). While the allergy seems somewhat odd, considering she has been taking some of these medications for months, she did have a significant increase in her blood count for eosinophils, which are the cells generally elevated in the event of an allergic response. Despite her symptoms, it is encouraging to note that Susannah's new bone marrow is able to mount such a response.

While the nausea and rashes seemed to be unrelated, it was hard to dismiss them as two separate entities when they began almost simultaneously. Especially when they were followed by Susannah spiking intermittent fevers on Sunday and Monday. While they were "official" fevers (>100.5 degrees axillary) warranting blood cultures and the like, they are certainly not as impressive as her toasty temps of January and February. Nevertheless, she was started on the usual regimen of antibiotics. Still, I can't help but think that this all is related to some sort of viral process. She has vesicles (sores) on her tongue and in her mouth, fevers, vomiting, itching and the various rashes. The only thing that I can't tie in is the weird white count.

Speaking of viral processes, remember good ole gancyclovir? The eight week course of IV anti-viral for CMV, that Susannah was on back in December and January ... well, it's back! Since Susannah was still not really tracking with her eyes, on Monday we decided to get an ophthalmology consult, just to see if the eye guys had any thoughts. Low and behold, when they looked back into her retina, they found active CMV (or CMV retinitis). The infection was pretty severe in both eyes, but in her left eye it was actually vision threatening. This was very scary as, while she is tested weekly for CMV in her blood, all recent tests have been negative. Apparently, CMV in the eye generally shows no symptoms until vision becomes impaired and Susannah is obviously unable to warn us of this. So, the ophtho consult was very serendipitous. Anyway, Dr. Davies was in the room in an instant, ordering systemic treatment of gancyclovir immediately. Because it takes sometime for the meds to get to therapeutic levels, the ophthalmologist felt it was also necessary to treat the left eye locally -- translation, stick a needle full of medication directly into Susannah's eye. Dr. Davies actually wanted to do the procedure on the spot, but the solution had to be specially formulated and because Susannah needed to be anesthetized (propofol) for the procedure, we also had to coordinate with our friends from the Peds ICU. So things were scheduled for Tuesday afternoon. The procedure went well and Susannah handled it all like the trooper she is. As for the IV gancyclovir, she will need it for at least eight weeks. Depending on how she responds, another injection may be required and at the very least her retina will need to be checked weekly for a while. Whether this will lengthen her stay in the hospital or ours in Minnesota for that matter, remains uncertain. This issue is about as clear as mud right now, so as usual, we will have to wait and see.

Yesterday also brought an Audiology consult. The original intent was just to have the molds for Susannah's hearing aids re-fitted. For obvious reasons, she has not really worn her aids since December and she has definitely grown since they were last fitted back in September. Considering that we want to stimulate her brain as much as possible, we need to get a handle on what she is actually hearing. Consequently, the neurologists and audiologists have decided to repeat a BAER (brain auditory evoked response test). This tests the nerve response between her ear and brain to give us an idea of how much Susannah hears (considering her current state, it would be difficult to test her hearing in the traditional sense). So, this test will be performed under sedation sometime later this week; hopefully it will show no change from her last BAER a year ago.

Speaking of neurologists, the new week brought old controversies back to the forefront. On Monday, Dr. Lockman, despite the results of the lumbar puncture and last week's CT scan (which was unchanged), is still convinced that Susannah has high pressure hydrocephalus. His take on the situation is that the measured pressure was artificially low due to the sedation she received for the procedure. He maintains that Susannah's vomiting and continued lethargic state are signs of high pressure. Drs. Charnas, Peters and Davies are all skeptical of this theory because Susannah does continue to slowly improve. Yesterday, to appease Dr. Lockman, it was decided to do an MRI of her brain to see if we could glean anymore clues as to the real cause of her slow recovery. Dr. Lockman seems set on shunting her, but he is pretty much alone on this one. Now, given the brief miraculous turn around in Susannah's alertness, I think he is, more than ever, flying solo. Nonetheless, everyone agreed that an MRI is at least safe, and may be helpful. Dr. Charnas came by this morning and told us that last night's MRI actually showed smaller ventricles (i.e. less chance for high pressure), but at the same time also showed some swelling around the ventricles as well as some fluid collections around the periphery of the brain. These results are a little contradictory, but the smaller ventricle size is extremely encouraging. The neurologists and Dr. Peters have another conference scheduled, and should be able give us a more thoughtful explanation later today or tomorrow. The plan for now, per Dr. Charnas, is "to actively sit on our hands and do nothing".

Susannah's lungs continue to grow stronger by the day. It has now been nearly three weeks since she last came off the ventilator and she is requiring little to no oxygen. The majority of her day is spent on room air, with the occasional use of blow-by oxygen. Her chest CT last week showed subtle improvements, but essentially remained the same. She did have a slight back slide one night early last week, where she began taking very shallow quick breaths and an x-ray showed that her lungs looked somewhat "wet" with a little more atelectasis (collapsing) in the lower lobes. The ICU folks thought she may once again need a little CPAP, but with the addition of more bronchodialators, she was back to business in a couple of days. Her films are now back to looking better than ever.

On the social side of life, yesterday started off bright and early as Madeline and I took Auntie Megan and Nils to the airport at 6:00am! We really enjoyed having them and they were a huge help. Megan took the kids on the usual rounds of Camp Snoopy, ice skating and so forth. It did take them two trips to actually make it to Camp Snoopy -- the first time that they tried to go they were turned away. I guess when they got to the mall, the place was barricaded with cop cars and the like. There was a "bad man" in the Mall of America! Apparently, an escaped convict was seen entering the mall on a surveillance camera. Megan and the kids sat in the car for almost an hour before aborting for an ice rink. As a side note, the guy got away and has yet to be apprehended. The kids could not stop talking about the "bad man". Boy, will they have a story to tell!

Well, that about sums things up around here. And to think when I originally started this update on Sunday, the first line read, "Let's see, not too much to report this week ...". It is amazing just how quickly things can change.

Love and warm wishes,

Klane, Amy, Madeline and Susannah

Sunday, March 18th, 2001 ~ Day +160

“We have a plan!”


Hello All,

Sorry for the not updating before now, but this has been a week full of uncertainty and we wanted to have all our ducks in a row before we filled you in on things. At present, Susannah is doing well and continues to slowly improve.

As you already know, Susannah had an EEG of her brain on Monday morning. There was some delay in getting these results back because the various physicians could not seem to agree on the meaning of the findings. The official report showed slowed brain activity, with no evidence of seizures. Again, the speculations as to the reason for this slowing varied dramatically between specialists, so on Friday another lumbar puncture was performed in order to help resolve some unanswered questions and formulate a plan.

Ahhh, where to begin? Well, the neurologist on service this week, Dr. Lockman, was adamant that the slowing was due to increased pressure in the ventricles in Susannah's brain, and wanted to consult the neurosurgeons again about placing a shunt or ventriculostomy, to help relieve some of the pressure. In case you’re wondering what ever happened to the non-surgical approach, the Diamox therapy got tossed by the wayside following Susannah’s code (on the off chance the med could have been a contributing factor), and since it never seemed to prove a difference clinically, no one was terribly interested in restarting it. At any rate, Dr. Lockman felt the changes on the EEG were definitely not related to sedation. Then came Dr. Charnas, the neurologist on last week who actually ordered the test. For weeks now, he has dismissed the idea that the problem is pressure or hydrocephalus related and chalked it up to the drugs. But, with the sedatives being weaned and Susannah still not waking up, he decided to order the EEG just to be certain there was no residual brain damage resulting from her respiratory arrest. Next there was Dr. Kersey, the BMT attending of the week, who thought Susannah may indeed be having seizures, despite what the EEG results showed. He felt her agitation and jittering could be the result of small, recurrent seizures; the nurses felt these symptoms were related to withdrawal from sedation. Finally, Dr. Peters chimed in. He felt there were no seizures or hydrocephalus. He was more convinced than ever that the slowing was the combined effect of long term sedation, prolonged illness and the associated sleep deprivation that has occurred over these past few months.

So, on Wednesday, there was a meeting of the minds. The four doctors got together to discuss the issues and their varying opinions. Knowing the four personalities involved, surely it was quite a meeting -- would have loved to have been a fly on the wall for that one! The conclusion: They would do a spinal tap (lumber puncture) to settle the high pressure issue once and for all, and then follow up with another CT scan of Susannah's head (brain) next week. From there a plan would be developed as necessary.

The LP was done Friday afternoon by Dr. Lockman and his entourage. Since such important decisions were to be made based on the results, they wanted Susannah positioned very precisely, so the ICU team came down to help with added sedation for the procedure. Susannah, of course, was a champ! Her opening pressure was 15, which was her lowest reading ever. Excellent news! With that result, the hydrocephalus and high pressure theories went right out the window.

Did I mention Dr. Davies came on service Friday? So naturally, "We have a plan!" For those of you who know her, I know you are smiling as you read this, and for those of you who don't, the above quote is classic Stella. She is wonderful, very much on the ball and knows everything there is to know about Susannah! She has the perfect combination of compassion and vigor -- absolutely nothing gets past her. Just in case it wasn't obvious, we LOVE her and are thrilled to have her on service during such an important time.

Immediately after the tap she called Dr. Peters. Needless to say he too was ecstatic about the pressure reading. Within the hour, Dr. Davies was in our room to discuss "the plan": Continue to aggressively wean Susannah's sedation. The theory is that she has been on so many sedatives for so long that, even though we have cut back significantly, there may continue to be residual effects for some time. Apparently, these drugs can remain in the system for long after the patient is completely weaned from them. Given the parameters of the plan, Susannah should be completely off her drips by midweek.

A few hours later, Dr. Peters stopped by. We spent some time talking about what to expect out of Susannah, given what we know. As she has basically been idle for the last two and a half months, both physically and developmentally, Susannah has a long road ahead of her. To lose several months of one's life at her age means that she has certainly missed some important developmental milestones. It is a given that she will require intense rehabilitative therapy for at least the next six months to a year. Hopefully this well help her to regain the lost ground, as well as, maximize whatever potential she has. At this point, we have no idea of what that is. There are just too many variables related to, not only her recent illness, but also to the Hurler Syndrome with her pre-existing mild delay, as well as variability in the effectiveness of the transplant itself. As both Drs. Davies and Peters were very quick to point out, however, Susannah's engraftment and enzyme levels are great, and kids are extraordinarily resilient. So, with a lot of love and care, we can certainly hope for the best.

By far the most exciting event of the week, was Susannah's re-finding her thumb! We have been thinking of Susannah's recovery process as a reconnecting of wires that have been left in a mess after her illness. Well, one of the first wires to be reconnected was the thumb to mouth wire. Done in the classically aggressive Susannah manner, she has not stopped sucking since she found it three days ago! Although still "asleep" a majority of the time, Susannah seems to be opening her eyes more and possibly even fixing her gaze on our faces. She still doesn't "track", or follow us with her eyes. She has been drinking from a sippy cup and even held it herself for a short while. Also, she once again "tripods" with little to no assistance, sitting for several minutes at a time. Susannah is much like a newborn, starting over and re-learning all of the most basic skills. Every "new" skill is a step in the right direction.

As far as her lungs go, while she sounds quite clear and her films look better than ever, Susannah has started requiring more oxygen in the last couple of days. Since there is no obvious explanation for this change, the plan is to just continue to monitor her closely and add an additional nebulizer (Atrovent) to her breathing treatments. When she has her follow up head CT next week, we will also take a peek at her lungs.

Susannah continues to tolerate her new tube feeding formula. Even though we have been steadily increasing the volume, because she has been without proper nutrition for so many weeks, we decided on Tuesday to re-begin TPN supplementation until she reaches adequate levels on her tube feeds. Since she is tolerating liquids by mouth (a few meds and a little water from the sippy cup), we are also hoping to begin feeding her some Pediasure, or the like, from a cup soon. Susannah actually attempted to breastfeed yesterday for the first time since December. Amy has been diligent about pumping each day these past few months (our nurses have jokingly deemed the bathroom, Wisconsin, as in the land of dairy farms and Patty even brought in a state map for the door), so that when she finally woke up, Susannah could still nurse if she were unable to eat otherwise. While she seemed to remember the gist of it, the addition of several new teeth made the process slightly more tricky and likely to be phased out quickly. I think it is fair to say that both mother and daughter have given it their all! At any rate, if we can get Susannah to take the majority of her calories by mouth, then perhaps we can be done with the feeding tube business. Miss Feisty has managed pull out three NJ tubes (these must be placed by a radiologist, under fluoroscopy, which involves traveling) in the last few weeks!

Grandma Dona flew home on Monday, despite the seven inches of snowfall that morning. Then the temperature warmed up (a whopping 40 degrees -- yes, believe it or not, we now consider this balmy), just in time for Grammy's arrival on Wednesday. The gap in grandmothers allowed Madeline to spend a little extra time with us at the hospital during the day this week. Our nurse, Patty, very well aware of Madeline's Peter Pan fascination, brought in a special treat to help entertain her during the long days ... the movie, "Hook". Needless to say, it was a big hit and she was entranced. Madeline also found a new friend in the librarian from the peds library. Her name is Nancy and she and Madeline have spent hours together reading books. A big treat is in store for the coming week: My sister and nephew, arrive tomorrow for a visit. Auntie Megan and little Nils, who is also three and a half, are sure to provide loads of fun and entertainment.

Well, that's about all there is on this end. We hope you all had a nice and relaxing weekend.

As always, we send our love,

Klane, Amy, Madeline and Susannah

Sunday, March 11th, 2001 ~ Day +153

Nature, time and patience ...


Good Day & Good Weekend to All!

This has been a positive week indeed. After expectantly waiting through the beginning of the week, Susannah was finally extubated on Thursday morning! Since then we have all been watching her like hawks and so far she seems to be holding her own very well, requiring little to no supplemental oxygen.

Although still quite lethargic, Susannah has been opening her eyes far more in the past few days than in the all of the last two months. As it stands now, however, "the lights are on, but nobody is home". Whether this state is drug induced or due to an injury to her brain is yet to be determined. Tomorrow morning she will undergo an EEG (electrical study of the brain) to look for any abnormalities. The EEG can tell us two things: 1) whether there is an injury to the upper control areas of the brain (the cortex) or 2) whether or not she is having any seizures. According to the neurologists, seizures are very unlikely and the suspicion for a brain injury remains quite low. But, given her respiratory arrest and prolonged course, they want to make sure that they aren't missing anything. If Susannah's lethargy is indeed drug induced, as she still remains on Versed and morphine drips equivalent to about one or two beers an hour, then the EEG should be normal.

Aside from the neurological and respiratory issues, Susannah is doing great. Her kidney function is the best it has been since the beginning of her FIRST transplant. This is because the BMT team has completely stopped her immunosuppressive, cyclosporine (CSA). Because she has shown absolutely no signs of graft versus host disease (knock on wood) and her kidneys have continued to take a beating, it was decided that she might be better off without the CSA. So far this seems to be the case -- only time will tell.

Although still slightly jaundiced, Susannah's liver functions are now back to normal. With regard to the rest of her GI system, she has been restarted on tube feeds at a very slow rate, and thus far she seems to be tolerating them quite well. We did restart her on a different nutritional supplement (Vivanex), so perhaps this is contributing to her greater tolerance for the feeds. Once again, time will tell!

As for the fevers, GONE! Susannah has now been completely afebrile since Monday. Hopefully her line change was the answer. As such, she has been downgraded to only prophylactic antibiotics for the first time in months. She was however placed on Ancef, as opposed to the usual penicillin regimen, due to the question of an allergic reaction a few weeks back.

Madeline and Grandma Dona have kept themselves busy this week with the usual routine of events that we have established for Madeline. The game of the week seems to be "Go Fish". Madeline has a pretty good understanding of the game with the exception that she has a hard time not showing everyone her cards. The other big event this week was Madeline's introduction to ice skating. Michelle, one of our more frequent college sitters, accompanied Madeline and Grandma Dona to the indoor park where there is an itty bitty ice rink. Although not Dorothy Hamill, Madeline had a great time and even skated short distances on her own. Not bad for a first timer. Just two days later, Sue Reichert took her to see "Disney on Ice", which of course, she loved. Madeline now practices her "skating" in the apartment. She removes all of the rugs and such from the kitchen and glides around the floor in her socks. Susannah's high-chair is too heavy for her to remove from the kitchen by herself, but instead of letting it's presence cramp her style, Madeline just refers to it as the zamboni. It's scary that she even knows that word -- way too long in Minnesota!

As for Amy and I, I think the move to the larger room has lifted our spirits. Room #7 receives immensely greater amounts of sunlight and we again have a view of the street, bustling with college students and the like (as opposed to a cold brick wall continuously doused in shade). We also now have futon that gives us a couch on which to relax. This is definitely good for the soul. Additionally, we have many fewer bruises to our shins from tripping over the equipment, nurses and each other! I think the move has helped us through the last ten days in which we have seen four little boys pass away on the unit (Aaron, Yossi, Ben and of course, Jaxon). While it is not unusual for children to die here, four in just over a week is quite a chill.

On a brighter note, we learned that our friends the Portwoods and the Torglers are both heading home on Tuesday (Jeremy, who you all know, and big brother Joshua to Kentucky, and Dalton, who like Susannah is on his second transplant for Hurler Syndrome, to Oklahoma). This is a bag of mixed emotions, as we are extraordinarily excited for them to move on and reestablish their lives, but we will definitely miss them as well. It's difficult to see other families leave while we stay behind, but we know our time too will come. As things can change daily here, we don't ask for predictions, but it will certainly not be before the end of April. Appropriately, I have placed a fortune cookie message at the foot of Susannah's bed, "Nature, time and patience are the three great healers". So to this wisdom we abide.

Keep up all the positive thoughts and continued prayers and we'll keep plugging away!

Love,

Klane, Amy, Madeline and Susannah

Sunday, March 3rd, 2001 ~ Day +146

Weekend Update


Good Morning,

Susannah's trip to the operating room went very smoothly. The preliminary readings of her CT scans, showed improvement in the chest and no new issues in the brain. We should have more details by early next week.

We have a new telephone number at the hospital. After 70 days in our little shoebox, the increase in Susannah's supportive equipment has warranted a move to a larger room. So, we are back in the room we stayed in during her second transplant. We can now be reached at (612) 273-0224.

I apologize for not updating sooner, but it has been a difficult couple of days. Little Jaxon passed away late Friday afternoon. He fought so very hard these last months, but his fragile body finally just became too tired to continue. Our hearts and prayers are with Jenn, Brent and the girls -- we will miss them very much. Susannah now has two extra special angels watching over her, Jaxon, and of course Maddie.

All our love,

Amy, Klane, Madeline and Susannah

Friday, March 2nd, 2001 ~ Day +144

A Trip to the OR


Hello Again,

We wanted to let everybody know that Susannah continues to improve little by little each day. Her ventilator support is steadily declining and her liver function is nearly back to normal, as are her kidneys. Slow steady progress in the right direction!

Susannah is scheduled to head the OR today to have her Hickman catheter (IV line) moved to the opposite side of her chest. For weeks now, Susannah's line placement has been sub optimal, as well as a questionable source for her fevers. Since the line needs to be replaced regardless, the team feels it would best be addressed before she is extubated, as there is always a risk that she would wind up being re-intubated during the procedure, which we would obviously like to avoid.

Incidentally, after a tiny crack was discovered last week on one of the outer lumens of her Hickman, as a quick fix, Susannah's line was repaired at the bedside. The external part of her line has now been repaired twice, once on each port. Remarkably, since the second repair was done last Wednesday, Susannah has been afebrile. Perhaps this was the source of her fevers all along -- just another reason to get the thing out. As you probably recall, this procedure has been scheduled and cancelled three times in the last month for various reasons, but today is a definite go.

Susannah will also undergo CT scans of her head and chest to help evaluate what damage, if any, may have resulted from her hypotensive episode the other night. Since she is vented, the anesthesiologist will transport her directly from the operating room to radiology for the scans. We should be back on the floor by late afternoon.

Klane's mother arrived on Monday for an already scheduled visit. Madeline is enjoying time with Grandma Dona and our neighbor in the apartment, Joshua (Jeremy's big brother). They have just discovered an amazing indoor park, complete with trees and a playground -- apparently a Minnesota thing! Madeline has also been working hard on her other favorite past time, ballet. At the present course, she may still be here for her ballet recital in June. Hopefully not, but in any event, she has already practically mastered the whole routine. Last week she actually asked her teacher, Miss Barb, if she could be "the prima ballerina". Confidence, she does not lack! With Grandma Dona in town, Madeline will, once again, begin her music class this week, which she has always loved. She continues with her fascination for Peter Pan and now Buzz Lightyear, from Toy Story. Madeline's other big news is that she is learning to spell short words like cat, dad, mom and most impressive of all, her own "very long" name. She is anxious to go to school and loves to play "pretend homework" with her "big girl friends", the college babysitters. She has even started saving her "pennies for college" in her piggy bank. With any luck, we will be home and settled in time for her to start preschool this year; she can hardly wait. Fortunately, with all the wonderful helpers that we've had since being here, Madeline has been able to continue to grow and push her developmental boundaries even though we've had to keep her somewhat secluded. With luck and perseverance, hopefully the time in which we can all return to a more normal life will come soon.

Please remember to keep our little Susannah in your thoughts and prayers today as she undergoes her surgery and scans.

Love,

Amy, Klane, Madeline and Susannah

Tuesday, February 27th, 2001 ~ Day +141

A Turn for the Better …


Hello All,

First, thank you to everyone for your wonderful outpouring of support. The tremendous number of notes we received these past few days really reminded us that we are not alone, and certainly went a long way for morale.

After an extraordinarily scary weekend, Susannah is now recovering with the strength that we have all grown to expect. She is definitely a champ! Without exaggeration, she was truly at death's door. While we are still waiting for the dust to settle, we now have hope that, with time, she may yet gain a full recovery.

She continues to be quite sick, particularly with regard to her lungs and liver, which seem to have taken the biggest hits. Fortunately, her kidneys and heart appear to have come through relatively unscathed. It is too early to determine what, if any, effects there were on her brain. The good news is that she is moving all four of her extremities and ironically, is now opening her eyes far more than in recent weeks. There is some question as to whether she has asymmetry in her pupils, representative of a focal brain injury, however this finding is confounded by one of the medications (atropine) that they had to give her during the resuscitation.

While we may never know the cause of Susannah's dramatic change in condition, we do know that the result was an acute onset of pulmonary edema throughout both lungs. In other words, her lungs just filled up with fluid, preventing the oxygenation of her blood.

On Friday, prior to this event, Susannah had experienced her best day in a long time. Her oxygen requirements were down to a minimum, she had been stone-cold afebrile for 36 hours and her fluid status had finally reached a nice equilibrium. That evening Amy and I had just finished taking turns holding her, while sitting in the rocking chair. Things were truly serene, and we were just thinking about leaving Susannah with the nurse, after shift change at 11:30pm, to take Madeline (who had fallen asleep on the pull-down bed in the hospital room) home.

That's when we noticed that Susannah began breathing much faster than usual. This lasted approximately 20-30 minutes, but her oxygen saturations remained good. Suddenly, her sats dropped from the 98-100% that she had been maintaining all day, to the low 90’s. We tried to compensate by increasing her oxygen supply, but her sats continued to drop to the high 80's. At this point Amy ran to get the nurse, while I put Susannah on the bed and strapped on her CPAP mask, which can supply far more oxygen than the nasal cannula she was wearing.

Within the one to two minutes that it took for the nurse to get there, she was requiring 100% oxygen by mask with her sats still dropping. Within another minute Susannah was turning blue and was moving very little air. Luckily our nurse, Yuri, had enough wits about her to start blowing air into her lungs with an ambu bag. Meanwhile, Amy fetched the resident who was across the hall. We managed to keep her sats up in the 80's and 90's by bagging until the ICU fellow arrived. Fortunately, he did a wonderful job of taking control of the situation. By this time there were about ten people in our little shoebox of a room, all buzzing around fetching this and checking that, grabbing things from the crash cart. At this point we were now having trouble getting blood pressures and picking up sats on the monitor. Susannah was still extremely pale, but the ICU fellow was telling me that he could feel a carotid pulse.

Although we tried to stay calm, I think Amy and I were both completely panicked inside. Relatively quickly, at least it seemed that way, the ICU fellow was intubating Susannah in front of my very eyes. I made Amy leave the room before Susannah was intubated, because I didn't think that she needed to see this happen. I've seen many code situations before, but this time I was frozen, it was very surreal. I tried to hang around for a few more minutes moving furniture out of the way and such, but quickly found that I wasn't much needed anymore, so I too stepped out to be with Amy.

As Amy and I watched from outside the room, we could see that the team continued to have trouble getting a blood pressure. We would come to find later that they were also having a hard time finding a pulse. Two femoral lines were placed; a venous for added access and an art line to facilitate an accurate blood pressure. After some time, perhaps an hour or so, it's hard to say, the BMT fellow (who came in from home) and the ICU fellow came out to tell us that they were in a very difficult situation. The highest blood pressures that they could obtain were something on the order of 25/15, in the face of maximal dosing of both dopamine and epinephrine. Additionally, they were having a lot of trouble ventilating Susannah and felt they were going to need an oscillating ventilator to in order to adequately oxygenate her blood. They explained, however, that they were unable to resort to this machine because it would push down her blood pressure even more, further preventing perfusion of her brain and other vital organs. In their opinion, the outlook was grim at best. It didn't appear as if Susannah would make it through the night. Not knowing what else to do, I called our priest from Saint Matthew's, Grant Abbott, and asked him to come down, which he did without blinking an eye.

By now Dr. Steiner, the ICU attending, had come in from home. She gave Amy a big hug, and then went straight to work. Father Abbott arrived a few minutes later, as did Dr. Orchard, the current BMT attending. You have to understand, that there were six inches of fresh snow on the ground, and the driving conditions were awful, so for all these people to make it in so quickly was no small feat. We then went with Grant to the lounge and said a few prayers for Susannah.

When we returned to the room, they were finally getting a peripheral pulse and decent blood pressures. Dr. Steiner did what classically happens, she took measures that no resident or fellow would be brave enough to do. She turned up the epinephrine to a dose five times the usual maximum dose. Once a blood pressure was obtained, they reduced her dose back to the usual maximum dose. After about five hours of work, Susannah was maxed out on her pressors, as well as her vent settings and her core temperature was down to 92 degrees, but thankfully she was alive.

Most of Saturday was spent catching up with Susannah's resuscitation (I don’t think our nurse, Kent, stopped moving once during his 12 hour shift). She was still requiring large amounts of fluids, blood products of every type, electrolyte replacements, and whatnot. She seemed to be holding her own, at least not getting worse. She started to make urine, late Saturday morning. This was a good sign that she had perhaps adequately perfused her organs throughout this ordeal. On Sunday morning, the tide started to turn for the better. She was requiring less oxygen from the ventilator (she had been requiring 100% oxygen combined with nitric oxide for the previous 24 hours), we were able to wean her from the dopamine and epinephrine and get her temperature back up to normal.

At present, Susannah remains on a ventilator with significant support. Her kidneys continue to be workhorses, trying to remove the kilogram and a half of extra weight (10% of her body weight) that she gained over the weekend. Her liver temporarily stopped making clotting factors, which had to be replaced with every blood product under the sun (FFP, cryo, platelets, PRBCs), and she is extremely jaundiced again because her liver is unable to clear the blood appropriately. We also continue to give her extra platelets, to prevent her from bleeding into her lungs, or anywhere else for that matter, until her clotting factors are normalized. As I mentioned earlier, her neurological status is promising, but we still need more time to tell.

I would like to express a few words of gratitude to some people who we couldn't have done without this weekend: Wendy and Joe Calareso, who for the second time, flew out to tend to Madeline at a moment's notice; Joan Hershbell, who came to the hospital at 1:30am and took Madeline to her home for a "sleep over" (Madeline has been so busy playing with everyone that she has not even missed us and is none the wiser to the situation); and Amy's sister, Kerri, who was in Minneapolis by 10:00am the next morning. She was a tremendous source of support throughout the weekend, filling in nicely for Amy's mother, who was unable to travel, as she had just suffered a TIA two days earlier (Grammy is doing much better now and appears to be making a full recovery).

Again, we are very grateful to have such support and friendship from all of you. Your love and prayers have carried us through this last year and especially the recent days. As always, we will continue to keep you updated.

With love,

Klane, Amy, Madeline and Susannah

PS … Many of you have been asking about "the boys". On a wonderful note, little Jeremy was finally discharged from the hospital yesterday, after more than five months. He is recovering nicely ... a true miracle, after five months on a ventilator and nine pulmonary hemorrhages. As for Jaxon, although still on the ventilator, his spirit remains stronger than ever and little by little his tumor is shrinking and his enzyme level is rising. He is a true fighter!

Saturday, February 24th, 2001 ~ Day +138

Prayers for Susannah!


Hello Everyone,

Last night at around eleven o'clock, Susannah suddenly went into respiratory arrest. After more than five hours of intensive intervention, she was finally stabilized on a ventilator, requiring full blood pressure support. There was much uncertainty as to whether or not she would make it through the night.

At this point, she appears to be sustaining, but her condition is still quite tenuous. We have no idea what set off this turn of events, nor can we anticipate what the future holds.

Susannah is a very sick little girl right now. We ask that you keep her in your prayers, now more than ever.

All our love,

Klane, Amy and Girls

Monday, February 12th, 2001 ~ Day +126

Our Mystery Girl


Greetings All!

Since last Monday's update Susannah has slowly improved, but it's been somewhat of a rocky course. She still has not awakened enough to open her eyes for more than a few seconds each day, and is certainly not yet signing, talking or taking anything by mouth. She basically lays in bed "asleep" all of the time. At present, she is still alternating time on the facemask and CPAP, but her lungs continue to show improved function. As for the fevers, they persist without an identifiable cause.

The scariest part of the week was Tuesday. That morning, Susannah seemed quite a bit more lethargic than she had been the previous four days off the ventilator, and was suddenly extremely jaundiced. This was followed later in the day with a sudden four point drop in her hemoglobin. While it didn't appear that she was bleeding anywhere externally or internally, that evening she underwent a CT scan of the head to see if there was any bleeding into her brain. Thank God this turned out not to be the case. Incidentally, however, Susannah was found to have a moderate amount of ventricular dilation in her brain (hydrocephalus). It was unclear whether this was the result of a high pressure obstruction, typical of children with Hurler Syndrome, or if it was due atrophy (mild shrinking) of the brain because of Susannah's recent illness. The neurologists were consulted on Thursday, but were of little help in making the determination. To quote, "I'm just a neurologist, I don't know much about hydrocephalus". Hmmm.

Today the neurosurgeons stopped by to give their two bits. Their rendering of the scans was that there didn't appear to be much evidence of high pressure, but that we should do a lumbar puncture (spinal tap) to settle the situation. The tap was done this afternoon and revealed a pressure of 20cm H2O (basically no different than before). We have yet to hear anything official from the powers that be, but from the preliminary results, it appears very unlikely that Susannah will require any surgical intervention. Dr. Lamb, who is perhaps the most experienced neurosurgeon around when it comes to these kids, strongly feels a shunt placement is best avoided unless absolutely necessary and only then as a last resort. His opinion, correlates with the theory that the best treatment for high pressure hydrocephalus in patients with Hurler Syndrome, is a BMT, which in time generally rectifies the problem.

This hydrocephalus situation did catch us quite by surprise. Susannah has had multiple MRIs and CTs of her brain throughout the last year, and up to now has not shown any signs of hydrocephalus. The dilation of her ventricles on last Tuesday's scan was fairly acute and had changed substantially since the scan of her sinuses only five days earlier. So, the sudden progression makes it unlikely that the dilation is a result of the Hurler Syndrome disease process, and instead related to Susannah's current medical situation. For now, she has been placed on a new medication, Diamox, just in case it is a high pressure problem. This drug is meant to slow the production of CSF (cerebral spinal fluid or brain fluid), and therefore prevent build up of pressure. We do not know yet whether this will be continued, considering the results of the lumbar puncture.

You may be a little confused as to why this is even an issue. The situation at hand is that Susannah has been rather slow to wake up after her extubation. As I said before, she is pretty much obtunded all of the time. She will occasionally arouse enough to scratch her nose and eyes, or tug at her CPAP mask, but that's about it. The question is: Is this the result of her remaining sedation (which is not at all insignificant, but has been dramatically decreased since coming off the ventilator), or is it a result of too much pressure in the brain? Like I said, no one seems to know the answer, so we just have to wait and see. Although, given the results of the tap, it is most likely the result of her sedation. Only time will tell.

Other than that, not much has changed since last Tuesday. Susannah has continued to spike fevers as high as 103.4 degrees, axillary. So far the synercid and ciprofloxacin do not seem to be helping. Susannah still remains on ICU status due to her fragile state, with oxygen dependency and constant need for electrolyte replacement, positioning, and respiratory therapy. Her jaundice that I mentioned earlier is slowly dissipating, with no clear etiology.

Susannah is the mystery girl. We have no idea what has been wrong with her lungs all this time, why she continues to have fevers, why she was jaundiced, why her blood level dropped out of nowhere on last Tuesday, or why she is taking so long to wake up. Just a little frustrating! For now though, we'll just need to be patient and pray that things change for the better.

More later as things develop ...

All the best,

Klane, Amy, Madeline and Susannah

Monday, February 5th, 2001 ~ Day +119

Off the Vent!!!


Hello Everyone!

Susannah is now off the ventilator!!! Many apologizes for not updating on this big news sooner, but things have been a little exciting around here and only now have I had a moment to collect my thoughts.

Anyway, back to the main event -- Susannah was extubated on Friday morning, and now, more than 72 hours out, appears to be holding her own! She is still being watched very closely, on ICU status, but is making the necessary strides toward recovery. Since coming off the vent, she continues to require significant supplemental oxygen, without which she quickly desaturates. Susannah has a decent cough, but the respiratory therapists have also been administering nebulizers and BD treatments (aka “chest beatings”) to help mobilize the secretions in her lungs until she, again, has a strong enough cough to do so on her own. Her chest film yesterday did show more atelectasis (collapsed lung sacs) than on Saturday, so she was placed on a machine that pushes air into the lungs via a facemask to help things expand. She has, however, been downgraded from BiPAP (which forces air into and out of the lungs, a sort of mini-vent) to CPAP (which forces air only into the lungs, requiring Susannah to exhale on her own), and today the ICU docs decided that she could alternate one out of every four hours between a regular oxygen mask and the CPAP. Hopefully, this is the first step to the road back home.

Aside from her lung issues, Susannah continues to have persistent fevers, in fact, as of late, they have been higher than usual and generally unresponsive to Tylenol or even Vioxx. So once again, we are playing the antibiotic juggling game. The antibiotic du jour is synercid, one we have been antsy to use for sometime due to Susannah's past history of VRE. The ID staff had been holding out on using this one for the past month, but in the absence of any other resolution, they are finally giving it a whirl. With any luck, this will be the one! As far as her blood pressure goes, since coming off the vent, she has actually been having hypertension and requiring frequent doses of nifedipine. Quite a switch from the dopamine titration of the last several weeks.

Now for a little background information. Until this past week, not much had changed in terms of Susannah's need for ventilatory and blood pressure support, relative to what Amy wrote last time. She completed the course of Etanercept and while it went off without incident, the drug didn’t produce the benefits everyone had hoped. In spite of this, however, the little lady did appear to be heading in the right direction, with the exception of another episode of bleeding into her endotracheal tube. Subsequently, she underwent yet another bronchoscopy on Monday the 22nd. They found what is called alveolar hemorrhaging, bleeding from the breathing sacks into the lungs. Fortunately, they considered Susannah's bleeds to be relatively minor, but still bleeds nonetheless. The reassuring factor, though, was that she continued to maintain adequate oxygen saturation levels despite what was going on in her lungs. All of her cultures from the bronch were negative.

When I arrived in Minneapolis on Saturday the 27th, Susannah looked better than when I saw her two weeks prior, requiring, on average, less oxygen and less medication to support her blood pressure. She was still pretty labile though, particularly with regard to her pressures.
Starting on Sunday, we were able to wean her off the blood pressure support, outright stopping the dopamine on Monday morning. Later that day we started slowly weaning her sedation, with the goal of getting her awake enough to take her off the ventilator by week's end. At this point Susannah's vent settings were not terribly high, leaving her to do a majority of the work. As such, we felt confident that this was an achievable goal, barring any further setbacks and assuming that the stars would correctly align.

So throughout the week, baby-step by baby-step, Susannah's ventilatory support was reduced. She tolerated all of the vent changes very well. It appeared that time had finally begun to heal her sick lungs (although, what exactly they were healing from still remains a mystery). On Thursday, she went down to the CT scanner to get some new pictures of her lungs, sinuses and abdomen. Of course, they wanted an idea of the status of her lungs before taking her off the vent, but the scan also offered one more opportunity to search for answers to her still unresolved fevers. Her lungs showed much improvement from the previous scans, and there was still no evidence of an abscess in her abdomen or elsewhere. Her sinuses appeared congested, but according to the ENT service this was nothing to get too excited about, although the jury is still out on this one until we hear from Dr. Rimmel himself. So still nothing to help explain away her fevers, but with the encouraging information on her lungs, we proceeded with the extubation plans for the following day.

On Friday morning, we turned off most of Susannah's sedation, so that she was quite awake; this way she would be somewhat agitated and have the strongest chance at breathing on her own. By this time she was on the most minimal ventilator settings, and still was not requiring any blood pressure support. So at about noon, the stars aligned, and the ICU doctor and a respiratory therapist removed the endotracheal tube. Susannah did very well. Since then she has continued to do well, requiring oxygen with BiPAP/CPAP breathing support, frequent turning and nebulizers. She remains pretty sedated on enough morphine, Versed and chloral hydrate to knock several grown men on their butts. She is certainly not yet her old self, but hopefully we will be able to finish weaning her sedatives soon (this process will be pretty slow, so as to help cut-down on withdrawal). Susannah is still too out of it to sign, play or even watch videos, so we continue to just play her cassette tapes and hold her hand. As I mentioned earlier, she is coughing fairly well, but is extremely hoarse and sounds very much like a sea lion. The nice difference is that it is now easier to get her out of bed to hold her, and we have already done so multiple times.

Over the next week or two we expect to completely wean her off of the sedation, hopefully requiring less and less oxygen support. It will probably be several months before she returns to her previous level of gross motor skills and activity. As she comes out of this, it will be difficult for her to even hold her own head up or lift her arms, much less stand and cruise. So therapy and lots of patience are going to be very important. The Hurler issues aside, she has a very long road ahead of her, but Susannah is definitely up to the challenge.

As for me, the issues with my work have been mostly resolved. I have officially been placed on "research" status, allowing me the flexibility to spend more time with the girls. I arrived last weekend and plan on staying for some time. I have been given an NIH research training grant which lasts until June 2002. Due to the many strings attached (gotta love the government), there is no real possibility of me returning to full-time residency before then. As painful as this might seem, it has actually been a great relief for me. I am now free to focus on Susannah, Madeline and Amy -- we now will have time to recover and hopefully reestablish a relatively normal life. Furthermore, it gives me an opportunity to pursue some interests that I have been chewing on for the last year or so. These may, in the long run, help my career goals as a researcher, as well as further the cause in the treatment of Hurler Syndrome related orthopaedic problems, of which there are many.

Grandma Dona was here for a couple of weeks. She and Madeline took it upon themselves to bring Valentine's Day to the apartment and the hospital room. We are now blessed with hoards of tacky, inescapable decorations. They also did numerous other fun activities, such as the Mall of America. During her visit, Amy and I did get to sneak out on Tuesday to take Madeline to ballet class. It was great to finally see her in action. She really enjoys the class, and it seems that the teacher thinks pretty highly of her (of course, she is a star pupil!).

Speaking of our little ballerina. Madeline's new favorite past-time is playing "Peter Pan". She is absolutely obsessed with Peter Pan. Her current goals in life are to, in chronological order, be a bride, be a mommy, and then become a "Peter-boy". Her doll, Kayla, is presently dubbed Michael (the small boy in the Peter Pan movie), and most of her imaginary play and dreams revolve around this Disney classic. I have been woken up on numerous nights/early mornings to hear about Madeline's Peter Pan dreams. She has quite a vivid imagination!

At present, Grammy is here for an extended weekend and our friend Heather Kinkead is returning for a third tour of duty on Thursday. We still haven't even dented the freezer from her last visit. She is a true blessing! Since I will be here this time, hopefully she and Amy can escape for a little while sans children. Amy really could use the break; with me back home until a week ago, she has basically been cooped up in the hospital for the last 45 days.

We will continue to keep you posted, hopefully in a more timely manner. Thanks once more for all of your notes, thoughts and prayers ... they are working and are very much appreciated.

Love,

Klane, Amy, Madeline and Susannah

Thursday, January 18th, 2001 ~ Day +101

The Week in Review


Hello All,

First to update on Susannah, things here basically remain status quo. She is still ventilated and on ICU status; tomorrow marks three long weeks. Not too much has changed in recent days, as she continues to spike sporadic fevers and experience significant dips in her blood pressure. It seems just about the time Susannah gets weaned from the inotropic medications, her BP begins to slide downward again, thus requiring them to be restarted. Everyone is stumped and exasperated. To quote Dr. Peters, "While there is definitely an answer to all of this, we are just not yet smart enough to figure it out." Soon, hopefully, soon.

I should point out that while Susannah is in intensive care, thankfully she remains in her protective isolation room on the BMT floor. A very special feature of Unit 4A is that all of the transplant rooms convert to ICU rooms, should the need arise, and the floor is staffed with not only RNs, but also CCRNs (critical care nurses). This way, patients requiring ICU level care do not have to be transferred to the Pediatric Intensive Care Unit, where there is often more than one patient per room and a lot of hustle and bustle. Also, the doctors from the Peds ICU physically come to 4A, to care for the patients. This is an added bonus because it allows the patients to be managed not only by these excellent intensivists, but also the hematologists who are so familiar with all of the nuances specific to transplant patients.

We are very fortunate in that all of the wonderful individuals already on Susannah’s nursing team happen to be ICU trained, so we have also been able keep continuity with our nurses, who already know Susannah inside and out. This really provides a sense of security and goes a long way for morale, not to mention our comfort level. When you’re spending 24 hours a day in a 12x12 room with these people, such things are invaluable.

After I last wrote, Susannah's fever broke just in time to save her that trip to the operating room. Since no one was terribly excited about her "traveling" on epinephrine and dopamine, and in the absence of fever, it was determined that the removal of her line was no longer urgent. On Sunday evening, Susannah had an episode in which her oxygen saturations dropped a bit, her blood pressure actually went up very high and following this, blood was suctioned from her endotracheal (breathing) tube. Nobody was quite sure what to make of this -- it could have been a very small pulmonary hemorrhage or just irritation from the tube. Although her subsequent x-rays did not indicate a major problem, the fact that her sats continued to remain a bit lower for several days was somewhat suspect. On Monday, another CT scan was performed, this time of Susannah's entire body. With this scan they were able to rule out the possibility of an abscess and reevaluate her lungs. Everything, except her lungs, looked fine. While the area in the left upper lobe continued to be concerning, there was still no explanation. On Tuesday night, Susannah again had a septic episode, spiking yet another fever and as you probably have guessed by now, her pressures dropped and then became very labile. During this time her blood gasses indicated both respiratory and metabolic acidosis. A few increases on her vent settings and several fluid flushes calmed things down a bit. All blood and other cultures continue to be negative, despite the cyclical pattern of fevers that persist. On Wednesday, the ICU team placed an arterial line in Susannah to help keep a closer eye on her blood pressure and allow the staff to more precisely titrate the inotropic medications. The fact that she continues to require regular use of pressors (most often dopamine) to maintain adequate blood pressures, both with and without fevers, is an unsettling mystery to all.

This evening we are trying a new medication on Susannah called, Etanercept (anti TNF alpha). The idea behind this trial is that it may help reduce any inflammation that may be in her lungs. The drug is generally used in rheumatoid arthritis patients, but has only once before been used to treat a situation similar to Susannah's. It is a bit unnerving, as all of the literature the doctors have presented to me on Etanercept clearly states that it is not for use in immunosuppressed patients and it can foster infections. However, Dr. Blazar, who is currently on service, feels strongly that in Susannah's case, since she is being monitored so closely and is already thoroughly covered with multiple antibiotics, the potential benefits would outweigh the risks. Lacking another true diagnosis, it is thought that inflammation may be causing some of Susannah's pulmonary troubles and producing the infection-like symptoms. So, after much discussion, the decision has been made to give the Etanercept therapy (a total of six doses, via sub-cutaneous injection) a chance and if we notice any problems at all, it will simply be stopped. Hopefully, this medication is the answer we have all been praying for -- now, we just have to wait and see. It’s a shot in the dark, but lacking any other solutions, we’re willing to try.

Perhaps the biggest turn of events this week has to do with Klane. It looks like he will be spending a little more time here in Minnesota with us. While this is probably the best thing for our family right now, it comes at no small price. In light of Susannah's worsened condition, Klane's department has encouraged him to put his residency on hold and spend the next 18 months in the laboratory. While this move will give him much more flexibility, it will add two additional years onto his already six year residency program. Additionally, because this new position is grant funded, his salary will be significantly impacted and the effect it will have on our health insurance remains unclear. Everything has happened very suddenly, so we are still digesting it and working out the logistics.

Back at the apartment, we did a grandmother swap this week. Grammy left for California, after a ten day stay, on Monday afternoon and Grandma Dona arrived late Monday night. Madeline, who returned from her trip West last Friday, did not skip a beat, when she awoke Tuesday morning exclaiming, "Okay, now it's time for another grandma." She is such an easy going little girl.

Many of you have asked how Madeline is handling Susannah's latest situation ... amazingly well, is the only way to describe it. At first we were hesitant to let her see Susannah with all of the tubes and machines. After a few days passed, she would tolerate it no longer and demanded to see her little sister. The first time she entered the room, she climbed right up onto a chair, looked straight at Susannah and inquired as to why she had that "pa-sketti" (aka spaghetti aka vent tubing) in her mouth. The following day, Madeline barreled through the door and announced that she had just washed her hands. She then kicked off her shoes and walked over to our nurse and said, "Kent, please carry me up into Sissy's bed and Mommy, don't forget to take a picture." She laid there for thirty minutes, singing to Susannah and patting her on the head. It was absolutely adorable and we literally had to peel Madeline out of the bed.

Susannah continues to be a little fighter and faithfully squeezes my finger every few hours as her sedation wears thin. This little gesture, along with a few blinks of her eyes makes my day. The only downside of it is that her movements are an indication that it is time again for more sedatives, so our interactions are very brief. The one evening last week when Susannah was relatively stable and her blood pressure medications were at a minimum, I did get to hold her for about half an hour. After much preparation and propping of pillows, our primary nurse, Mary, placed Susannah, with all of her attachments, carefully in my lap. It was so nice just to have my baby in my arms ... it had been more than two weeks!

Before I close, I want to mention two other very sick babies that are with us on the floor right now. "The boys", as they are so often referred to, both also have Hurler Syndrome and are on ventilators, as well -- in fact, they occupy the two rooms flanking Susannah's. In an attempt to lighten the mood up here, the staff has deemed our little corridor, "Hurler-Vent Row". While each of our children's current situations are remarkably different, to say our three families share a common bond would be an understatement. As you so fervently have for Susannah, please also keep both Jeremy and Jaxon in your thoughts and prayers, while their friends and families do the same for our little princess.

All our love,

Amy, Klane, Madeline and Susannah

Wednesday, January 10th, 2001 ~ Day +93

More Ups and Downs


Hello Again,

Well, it seems Susannah misses her Daddy. Klane returned to California early Sunday morning and he was not gone more than a half an hour before Susannah's blood pressure again plummeted and her temperature spiked. Just like last Wednesday she was started on pressors and given additional antibiotics. Things resolved and they were able to wean the dopamine on Monday. Unfortunately, by yesterday morning, the cycle had started all over again. Today is Wednesday and she is now requiring both dopamine and epinephrine to maintain adequate blood pressures and remains febrile. Of course, she is also still on the ventilator and will continue to be for sometime. Although, her lungs do seem to be oxygenating better and the ICU doctors are in the process of tapering down some of the vent settings. It appears that Susannah is suffering from some components of ARDS - Acute Respiratory Distress Syndrome, but beyond that no one is very certain of anything.

There is still no clear cut answer to what is making Susannah so sick. A repeat CT scan on Monday showed both good news and bad news. It seems one of the problem areas (the nodule in the upper right lobe) in her lungs has resolved, while the lower lobes continue to show some sort of infectious fluid. Unfortunately, a new concerning patch in the upper left lobe has now appeared. No one is quite sure what to make of the new finding and in fact, the different specialists all have varying ideas as to the cause, as well as to the necessary treatment. Their proposed diagnostic approaches vary from blood cultures for numerous obscure organisms to a repeat broncoscopy to a desire to perform a second thoracotomy, this time on her left side. As you might imagine, their speculated diagnoses are equally as conflicting. To stump everyone even more, all results from the lung biopsy have come back negative, as have all blood cultures to date. Dr. Peters has made it very clear that while he is eager to solve this mystery, he does not want to do anything that will further compromise Susannah's stability unless it is absolutely warranted. He is very concerned.

For now, however, the plan is as follows. Tomorrow, Susannah will head back to the operating room to have her central line removed and replaced in the event that it is harboring the offending bacteria. While this is probably not the case, it is a relatively easy way to eliminate a potential source. Also, her chest tube was removed this morning for the same reason. Several vials of blood were also drawn this morning for a huge array of tests, some of which we will know the results later today and others not for a week or so. This afternoon they repeated an echocardiogram of her heart to ensure it was functioning properly -- thankfully, it remains unaffected. It seems we will be doing a lot of "ruling out" before the exact problem can be detected. As proven time and again, with little Susannah, nothing is simple.

On a more positive note, in fact a wonderful note, Dr. Peters stopped by yesterday to report on Susannah's Day +60 enzyme studies. Susannah's enzyme activity level is a whopping 44! This is above average and in fact, Klane and I are at 24 and 25 respectively. So, 44 is obviously enough to do the trick. Also, because Susannah's engraftment level continues to be 100%, Dr. Peters feels that her enzyme level will eventually approach or equal that of her donor, 63 -- a little extra never hurt.

Surely there will be more news to report shortly as things continue to unfold around here and we will certainly be in touch. Until then, thank you for continuing to hold Susannah in your thoughts and prayers, she needs all of the added strength she can get.

With love,

Amy, Klane, Madeline and Susannah

Friday, January 5th, 2001 ~ Day +88, followed by Days +79 and +80

Now for the details ...


Hello All,

I realize I blindsided many of you with my last update and I apologize for not giving more details, but things were happening very quickly and time was limited. Since you may be a little confused as to how Susannah has found herself in such a precarious situation, following this update, I am including the two updates I wrote (but never managed to send) last week just before things got crazy.

For now however, I will fill you in on where things stand at the moment. Unfortunately, Susannah remains on a ventilator in intensive care. Following her surgery last Friday, Susannah has been slow to "wean" from the vent. Also some other issues have now developed.

Since she appeared to be stable and close to being extubated, I returned to San Diego on Monday night because I was running out of people to ask to cover my absence, and I was starting a new service on Wednesday. Leaving, of course, was very difficult for me to do, but with little choice, I flew home.

On Wednesday morning, Amy called me to tell me that Susannah had suddenly become very sick, spiking fevers and being unable to maintain her blood pressure. It appears that she was septic (a disseminated bacterial infection and quite life threatening), requiring inotropic blood pressure support medications (pressors) and increased ventilator support. So, I jumped on a plane that afternoon, and am now back in Minnesota ... my third trip in twelve days.

As of today, she is again stable but still requiring a fair amount of support from the ventilator and remains on ICU status. Needless to say, these past few days have been extraordinarily scary.

Susannah is now on six different antibiotics to keep whatever this new bacteria is in check, not to mention a host of sedatives and other medications to counteract the side effects of one another. The exact underlying causes of both her lung troubles and her fevers are still unknown, so Dr. Peters (who, thankfully, came on service the first of the month) and the ICU team are hesitant to stop any one drug. They are relatively certain that this recent septic episode, is unrelated to the original lung disease which prompted them to do the biopsy last week.

Until Wednesday, Susannah was heavily sedated while on the ventilator, but as she became more unstable, and her support needs increased, the doctors felt it necessary to keep her totally immobile, and as such are now treating her with paralytic medications as well. She is unable to move at all, but is still able to hear. So, we are constantly playing her favorite music from a little cassette player by her pillow. Music has always been soothing to Susannah and prior to the paralytics, in between doses of sedation, she would arouse slightly and sign for her music to be played. It was very sweet. It's really difficult to see her lay there unable to respond, but we know that resting her body is very important in helping her heal.

As for Amy, she has amazingly maintained her composure throughout this ordeal. Since Madeline and Grammy left for California on Tuesday afternoon, she has been able to focus on Susannah. A woman on a mission! She is truly amazing.

As I mentioned, Madeline is now back in California. She is having fun with her "old" friends in the warm sunshine. Originally, the plan was for her to stay at our house with Poppy and I for a week or so. When the plan changed suddenly, our friends didn't skip a beat and the Thayer, Jacobs and Spencer clans stepped right in. So far, she doesn't seem to miss us a lick. I wish I could say that the opposite were true.

Finally, I just wanted to extend a warm thanks to the Calaresos, who at the drop of a hat flew to Minnesota on Wednesday afternoon to give Amy a hand. Unsure of my ability to make it here and with Amy suddenly alone with a critically ill child, they arrived in Minneapolis before I did! Also, thanks to all of you for all of the notes and prayers. Our e-mail has been disabled for the last week, and only today have we been able to read your messages.

Right now the plan is to continue to let Susannah's lungs and body rest and recuperate. The doctors are scratching their heads as there seems to be no clear-cut answer to what is happening. We will certainly keep in touch as things change. Please continue to keep Susannah in your thoughts and prayers.

All our love,

Klane, Amy, Madeline and Susannah

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From Thursday, December 28th, 2000 ~ Day +79


Hi Everyone,

I wish I had better news to report. Susannah has been in the hospital since last Friday due to difficulty breathing and increased swelling in her left arm.

On Friday morning the 22nd, Susannah went to clinic and received a clean bill of health. Just two hours later, however, she began to have some difficulty breathing with swelling in her left arm, so Amy took her back to the clinic. From there she was admitted and given supplemental oxygen. That night she had a couple of episodes of oxygen "desaturations", or reductions in oxygen carrying of her blood. An ultrasound of her arm was performed to rule out the possibility of a blood clot and none was found. Still unsure of her diagnosis, she had a CT scan the next day showing fluid in both of her lower lungs and a nodule in the upper lobe of her right lung.

Fortunately, I was scheduled to arrive for Christmas on Saturday anyway. Within three hours of my being in Minneapolis, the pulmonologist was performing a bronchoscopy (a fiber optic scope to look inside the lungs) to get cultures. Dr. MacMillan, our newest favorite Canadian and the current attending BMT doctor, was concerned about a fungal infection because of the appearance of the second patch in the right lung. Susannah tolerated the procedure fairly well, but the scope seemed to have induced some spasm in the upper airways. That night Amy and I both stayed over in the hospital because Susannah was having a lot of difficulty breathing. Luckily she responded to a treatment of high dose steroids, and narrowly avoided being intubated. Notably, this all transpired on the one year anniversary of Susannah's diagnosis of Hurler Syndrome. Some have suggested we erase December 23rd from our calendar as it just doesn't seem to be Susannah's day!

It is unclear at this time what the exact etiology of Susannah's problem is, but it is likely a viral pneumonia and croup. At present the pulmonologist is considering another entity known as BOOP (bronchilitis obliterans with organizing pneumonia) or possibly Aspergillus (fungal) pneumonia.

As for Christmas, we were unable to get Susannah out of the hospital on a pass -- Dr. MacMillan just did not feel it would be safe. So, instead we brought Christmas to Susannah. Grammy and Poppy were in town and we basically moved Christmas to Unit 4A, one day late, because things were so hectic. Fortunately no one was the wiser, especially the girls. It was amazing how Santa Claus even knew to pick up Susannah's stocking from the "fireplace" at temporary house and bring it to her hospital room ... what a smart guy! Anyway, we feasted on Honeybaked Ham and delicious side dishes thanks to Amy's sister Kerri and our Care Partner, Sue. While we obviously would have preferred to all be "home" for the holiday, we were just grateful we could all celebrate Christmas together.

Speaking of Sue, she was instrumental in assuring Santa'a arrival. One day several weeks ago, Madeline was inquiring as to how the big guy was going to get into our apartment on Christmas since there is no fireplace. Well, leave it to Sue, after much research, she was able to commission a couple of local art teachers to construct the girls their very own fireplace on which to hang their stockings! It was very cute and Madeline, needless to say, was very relieved.

Another highlight of the holiday weekend was the Christmas Eve Pageant at Saint Matthew's. Madeline played the perfect angel and was adorable. We really appreciate them including her in the event. Amy, Poppy and I were able to sneak off for a couple of hours to watch her in the pageant, while Grammy stayed back at the hospital to look after Susannah.

Since Sunday, Susannah has progressively improved, but seems to have plateaued at a point where she is still requiring supplemental oxygen, and remains in the hospital. With things relatively stable, I returned to work in San Diego as scheduled on Tuesday, the 26th.

A repeat CT scan was performed today and showed that her lungs didn't really look all that much better, and in fact the second patch looked worse. As I mentioned earlier, there are still three possible diagnoses, each of which is treated differently. The possibility of her problem being BOOP, which is a condition associated with a preceding viral infection, such as croup, is weighing more heavily on the pulmonologists' minds. The treatment for this is high dose steroids. This treatment is contraindicated in an active viral or fungal infection, because it will prevent Susannah from being able to fight off the infection.

Susannah is scheduled to undergo a CT scan guided needle biopsy tomorrow to help make the diagnosis. As such, the plan is to obtain the biopsy of her lung. It's a small procedure, but has a fairly significant (5-10%) chance of collapsing her lung. This potential complication, as well as, her already compromised pulmonary status and Hurler upper airway makes for a particularly scary time. We have confidence that everything will be fine with the procedure, this is the reason we chose to take Susannah to Minnesota, as opposed to somewhere else, where the experience of the BMT staff and the support staff (i.e. the anesthesiologists, pulmonologists, radiologists, etc.) with children like Susannah is far greater.

We truly hope you all had a wonderful holiday season and wish you a very Happy 2001!

Love,

Klane, Amy, Madeline and Susannah

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From Friday, December 29th, 2000 ~ Day +80


Good Evening,

This morning, Amy had still not been given a time for the needle biopsy. Within a few short minutes, the doctors came in and informed her that the interventional radiologist, who performs such procedures, did not feel comfortable doing it. They were worried about the increased risk of bleeding if the problem were indeed Aspergillus. So at 9:00am, it was decided that the biopsy would not only be done in the OR, but instead of it being a minimally invasive procedure, it would be an open lung biopsy through a full thoracotomy. No small difference. They wanted the procedure done ASAP. The limiting factor was that our trusty surgeon, Dr. Saltzman, was out of town. Dr. MacMillan did not feel it wise to wait the three days until his return, so they consulted his associate, Dr. Leonard, who wanted to do the surgery later this afternoon.

Well, an unexpected cancellation, suddenly turned later this afternoon into 10:00am! Things happened so quickly that Amy barely had time to get her shoes on. The surgery itself went smoothly and relatively quickly. There should be some news on the biopsy results tomorrow and we just pray it is not Aspergillus.

Unfortunately, I was still back in California during all of this. We had agreed long ago that it was very important for both Amy and I to be present for any operative procedures, due to Susannah's high risk airways and fragile post transplant lungs. The doctors as much as said with Susannah's recent breathing difficulties it was a sure bet that she would have to remain intubated after leaving the OR and require ventilator support for a night or two. This being the case, it was more important than ever that I be present. However, since things transpired so quickly, I wasn't able to catch a flight in time for the surgery, so I finished up things at work and caught the last flight out of San Diego today. This is quite possibly the worst weekend for me to be away from work as it is the New Year's holiday and I was to be responsible for most of the call. So, many many thanks to the guys who volunteered to cover for me during this time. Both Amy and I appreciate it immensely.

Love always,

Klane, Amy, Madeline and Susannah

Saturday, December 30th, 2000 ~ Day +81

Important News on Susannah


Hello Everyone,

I just wanted to give a brief update on Susannah. A great deal has happened in the last eight days and I was in the middle of preparing a detailed message yesterday morning recounting these events, when Amy called to tell me that Susannah was unexpectedly going to the OR -- in an hour. I will fill you in on all of the details leading up to this point in a day or two, but for now let me give you the crux of the situation as it currently stands.

Yesterday morning, Susannah underwent a right thoracotomy/open lung biopsy to determine whether or not she was harboring a fungal infection in her lungs, Aspergillus pneumonia. Today, the results of the biopsy, thankfully, showed no evidence of fungus or bacteria, indicating that the causative organism is likely viral.

At present, Susannah remains intubated from the surgery, requiring the help of a ventilator to breath. She seems to be slowly improving, and we are hopeful that she will be weaned from the vent sometime tomorrow. She also has a chest tube in place, which will be removed after she comes off of the ventilator. She is in good hands, and we are confident that she'll get through this situation with the help of the staff here at the U of M. It was the possibility of problems like this that drove us to come to Minnesota in the first place.

We'll keep you up to date as events unfold. Keep your fingers crossed and please say an extra prayer for little Susannah.

With all our love,

Klane, Amy & Girls

Tuesday, December 12th, 2000 ~ Day +64

Burrrrr, it’s cold!


Hi All!

Yet another update on the crew in the now frozen Gopher State! The temperature was a balmy five degrees today. All I can say is, as much as I miss those girls, I'm glad that at least I'm at home in beautiful, warm San Diego. The temperature in Minneapolis has been near or below zero for the better portion of the last week. Amy and Susannah, being the troopers that they are, bundle-up and continue to WALK to clinic or therapy everyday! Crazy? I think so.

One of our dear friends, Heather Kinkead, flew in from Washington, DC Thursday night to stay for an extended weekend. As you may recall from her last visit, she is a wonderful cook, and otherwise knows how to baby Amy, who is in great need of such attention about now. This was a long anticipated visit, as Amy has now been alone for the better part of a month, and will continue to be so until Christmas. Unfortunately, Heather had to leave today, however, she did leave behind a fully stocked freezer and a variety of homemade holiday treats to help ease the pain. Madeline was as disappointed as Amy to see her go, as she had grown quite attached to her cooking buddy.

I also made it back to Minnesota for the weekend, or more accurately for 32 hours! I flew out Saturday morning and returned Sunday evening, quite a whirlwind tour. On Saturday afternoon, we decorated our Christmas trees. We actually have two trees in the tiny apartment, one in the living room and a small one for Madeline's room. Since Susannah cannot be around any live plants, a couple of our friends in the area graciously donated their artificial trees to our cause. The girls had been anxiously awaiting my arrival for this event and Madeline could barely stand it she was so excited. She had made several Christmas ornaments earlier in the week and was eager to get to work. She and Heather had also picked up a couple boxes of candy canes (pink ones if you can believe that!) and popcorn for stringing. None of us were sure how the trees would turn out with the limited decorations, but as luck would have it, some of our friends from home sent a package that very day filled with Christmas cheer -- complete with handmade ornaments bearing each of our names. Many thanks to the Jacobs, Spencers and Thayers! When all was said and done, the tree turned out to be quite beautiful and fits perfectly in the spot in front of the window in the living room. On Sunday, we didn't accomplish much other than eating a lot of delicious food, a la Heather, and spending a little down time together. It was far too cold to even step outside the apartment. As always, it was difficult to leave.

Several new developments on the BMT front. Susannah was retested for her engraftment levels, and remains strong at 100%, yeehaw! So it seems that Dr. Peter's prediction is so far holding true. The results from her Day +21 enzyme studies provided the excellent news that Susannah is producing the enzyme necessary to combat the Hurler Syndrome. The levels were not yet normal, but well on their way. This first test was performed only days after engraftment had occurred and it is expected that as the engraftment digs in it's heels, the enzyme activity levels will continue to rise. Yesterday, Susannah saw Dr. Peters for her 60 Day follow-up appointment, and once again engraftment and enzymes studies were drawn. The results should be back in three days and three weeks respectively. Overall Dr. Peters was very pleased with her progress and has begun developing a very tentative plan for her eventual return to California.

On a less happy note, Susannah tested positive for early CMV (Cytomegalovirus) infection, supposedly the bane of a transplant's existence. At this point she is not overtly infected, but is colonized with the little buggers. The test that is used to detect CMV is extraordinarily sensitive, so at least we have some lead time to combat the virus. Consequently, Susannah was taken off oral acyclovir and put on a more potent drug called gancyclovir. The bummer about this change is that gancyclovir is dosed in IV form and is known to reduce white blood cell counts. This means that Amy has to wear gloves and use "chemotherapy" precautions while administering it to Susannah at home, twice a day, through her hickman catheter. Fortunately, Susannah is far enough along that it shouldn't affect her engraftment, but it will impact her counts such that she will likely require G-CSF (growth factor) and more frequent platelet transfusions. Since her hemoglobin is currently stable, she should be able to maintain her red cell transfusion independence. The other major bummer is that the medication course is anywhere from eight to ten weeks which may keep the girls in Minnesota for at least two to four weeks longer than we had initially hoped. It now looks like they won't be home until the early part of February.

As for life in Minnesota, Madeline continues to go to ballet classes once a week and loves every minute. She has a new grown-up friend, Joan Hershbell, who has taken Madeline to "tea" and was also the force behind the homemade Christmas ornaments, among other exciting activities. To quote Madeline, "Joan and I have a lovely time together.". Last week, she also went to lunch and to see "102 Dalmatians" with our Care Partner, Sue and, of course, had a blast. Otherwise she has been spending a great deal of time with her "big girl friends", aka the college babysitters, playing games and making crafts while Amy and Susannah are at appointments.

Susannah, for her part, is slowly regaining the strength that she lost with the second transplant, not to mention the Adenovirus. She has once again returned to the chubby state induced by the steroids, although she actually completed her prednisone taper today, so the puffiness should dissipate within a month or so. This bloating, along with the beginnings of the cyclosporine (CSA) induced black facial hair and her bald head, have her looking vastly different from the cute little thing she was eight months ago, before all of this started. Of course, she's always beautiful in our eyes, and her sweet personality is still the same. Besides, no one can resist running their fingers through that soft CSA hair!

Otherwise, her sign language vocabulary continues to grow and she appears to be getting closer to actually muttering a few more words instead of making animal sounds like "baaa" "roar" and "quack". In fact, this weekend she actually verbalized "mama" for the first time ever!!! This made Amy's day, as it has come long after "daddy", "hat", "hi" and a couple others. Her favorite word is actually "yeah", although, thanks to the folks around here, it is very distinctly said with a Minnesota accent!

As for her eating, she continues to take some food (and Pediasure) by mouth, but for the most part has been overcome by the post-chemo salt fetish. She is currently very fond of the "Toes Diet" -- Fritos, Doritos and Cheetos. Just to help wash down all that salt, she is usually happy to chase the wonderfully nutritious line-up from Frito-Lay with a little chicken broth. Truthfully, she continues to receive the majority of her nutrition from her TPN, which we will continue to give her for the next five to six weeks to help give her a boost and create some reserve.

As for me, life in San Diego is quiet. I spend most of my time buried in work in some form or another. It makes the time pass more quickly, and it allows me to make up for some of my time away. I should note that I did make it to the Carlsbad vs. Torrey Pines CIF semifinals, which Carlsbad won! Back to the championships for the second year in a row. They are so lucky to have had athletes like me pave the way for them! Ha! Seriously though, it was very exciting. Since I went back to Minnesota for the weekend, I wasn't able to go to the finals, which unfortunately the Lancers lost.

Anyway, not much else to report at this time. Keep up the prayers and support, there's light at the end of the tunnel!

Love,

Klane, Amy, Madeline and, of course, Susannah

Thursday, November 23rd, 2000 ~ Thanksgiving Day ~ Day +45

Happy Thanksgiving!


Happy Thanksgiving to All,

Our little family will be spending a quiet holiday together. After 16 days, Susannah was discharged from the hospital on Tuesday afternoon and Klane arrived in Minneapolis yesterday. We are all now finally back at the apartment again and for this, we truly are thankful.

Despite Susannah's "Saturday night fever", Dr. Peters kept his promise to have us out of the hospital before Thanksgiving. We will however, be expected in clinic daily, although we do get a special holiday reprieve today. The doctors believe the Adenovirus to be the root of Susannah’s fevers and expect her to continue to run intermittent low grade temps until the virus finally clears. They have even raised her "magic number" for readmission to 101 degrees, as opposed to the usual 100.5, assuming, of course, there are no other symptoms involved. The Adenovirus could linger with Susannah for months, but there are much worse things to have, and in time as her body adapts, it will become a mere annoyance.

Once again, Susannah really handled being cooped up well. Although, it's pretty scary how much she has learned to enjoy procedures. Twice this past visit, she spiked fevers, felt miserable, yet perked right up as soon as she caught sight of the red wagon that was to take her out of her little room down to radiology for chest x-rays! It's amazing how well she adapts. As for Madeline, she was a little less enthusiastic about her sister and mommy returning to the hospital so quickly. However, she did have a very nice time with Grammy, who was instrumental in looking after Madeline these last two weeks.

For the most part, Susannah is back to her old self. She is once again happy and playful. She is still having trouble absorbing fluids and therefore nutrients, and as such was sent home on intravenous nutrition (TPN). As Madeline puts it, Susannah will be "hooked up" for 14 hours per day and will be guaranteed the fluids and nutrition her body needs to heal. You may recall, we had in the past tried to avoid TPN because of the adverse effects of bypassing the stomach and intestines. Susannah, remarkably, continues to eat and drink which will keep her gut active and her hunger instinct intact and help avoid any long-term issues. Because of her absorption difficulties, some of her meds have been left in IV form. Luckily for me, the timing is such that between clinic and the home nurse, I will be free from most of this responsibility. However, regardless of what it entails, I am just glad we are back "home" again.

We wish you all a very happy and peaceful Thanksgiving Day. We have much to be thankful for this year, especially the strength we gain from the continual love and support from you, our family and friends.

All our love,

Amy, Klane, Madeline and Susannah

Friday, November 10th, 2000 ~ Day +32

Return to “The Get-Well Hotel”


Hello Everyone,

As usual, a pretty lengthy update to follow, so first let me give you the latest ...

I am now back at work in San Diego. Just two hours before my plane departed the Twin Cities last Sunday, Susannah (along with Amy) was re-admitted to the hospital for fever and dehydration. After several days as an inpatient, we found out today that Susannah has a bug called Adenovirus. Hopefully, we can credit this as the sole source of her current relapse.

Okay, now I'll backtrack a bit, fill in the gaps and get you up to speed.

Grammy arrived the eve before Halloween with homemade costumes in tow. Madeline was, of course, a princess. Originally, Susannah was slated to be a pea (get it, "The Princess and the Pea"?), but in light of her current infatuation with "quack quacks", she was a duck. Grammy created a masterpiece of white fur with yellow webbed feet. She was even able to transform Susannah's mask into a yellow beak for the day. The Child Family Life department at the hospital set-up a very festive afternoon of events, in which both girls were able to participate. On the unit, they had "reverse trick-or-treating", in which the staff knocked and delivered candy to all of the kids stuck in their rooms. Since Susannah's counts were so high by Tuesday (WBC-5900 and ANC-2600), she was given a pass to leave her room. So, while Madeline was up at the Halloween Party getting her face painted, Grammy decked Susannah out in her duck suit and they joined us upstairs for the trick-or-treating tour. Susannah rode around in a red wagon watching in awe as Madeline and her new friend, Joshua, collected candy from several stations around the hospital. They all had a great time.

I should take a moment to talk about Joshua. He is the big brother to Jeremy, yet another Hurler Syndrome patient on the floor. The two have become fast friends, providing each other with countless hours of companionship and, best of all, energy release! We met his parents, Susie and Ian, back during Susannah's first transplant when they were looking into having Jeremy transplanted here. Little did we know then that we would not only be neighbors on the floor, but also next door neighbors in our apartment building!

On Wednesday, Day +24, with her counts up and her transition to oral meds complete, Susannah was discharged. Leaving Unit 4A is quite a process, so finally at 5:00pm that evening Amy, Susannah and I (and everything we'd collected over the last month) headed for "temporary house". Madeline and Grammy were eagerly awaiting our arrival and the girls were so happy to be together, on their own turf, free of IV lines, that they played together until we had to force them both to bed. They truly have a special relationship and love each other dearly.

As you might imagine, after more than 30 days of being poked and prodded at all hours, Susannah's first couple of nights at home were not exactly rest filled. Also the fact that she was sent home on Q8 antibiotics (synercid, for the VRE), didn't particularly help matters. Since the drug was to be administered every eight hours, there was inevitably a dose to be given in the late night, early morning hours.

Of course, the best day of the week (the month, the year!) was Thursday, the day we learned of Susannah's RFLP results. Thanks so much to everyone for the tremendous number of messages we received celebrating this wonderful news. Getting engrafted was, of course, the first major hurdle. According to Dr. Peters, once engrafted on the second try, he has never had a child actually lose it (unlike the first attempt when it is fairly common for engraftment levels to slide drastically and even disappear completely).

While we remain very optimistic, I must emphasize that Susannah is not out of the woods yet. The next sixty days or so are fraught with potential complications, as indicated by the minor one that we are enduring at present. We are now just entering the stage at which graft versus host disease (GVHD) becomes evident. This can be as benign as a skin rash or as life threatening as to require ventilation. Infections and sepsis are always lurking around the corner as well, which is why Susannah will need to be admitted for any fevers over the next six months to a year. We just need to keep in mind that Susannah's condition is still quite friable and that she could take a very fast turn for the worse at any time. And that is exactly why she will have to remain in Minnesota, where she can be closely monitored, until late January, at the earliest. So please, don't hold back on those prayers yet!

Since being discharged last Wednesday, Susannah had been seen daily in clinic, as she was having some difficulty with vomiting and diarrhea. In light of her recent love affair with Pediasure, the problems were being chalked up to transplant induced lactose intolerance. This seemed like a fine explanation until Saturday when Grammy went on a hunt for lactose free Pediasure only to discover that all Pediasure is made without lactose! Anyway, Susannah seemed to be on the mends Saturday, so much so that we were even given the day off from clinic on Sunday. However, when she awoke Sunday morning her temp reached the magical mark of 100.5, earning her a trip to the "Get-Well Hotel".

In a manner of hours she had become severely dehydrated. By the time she was admitted her fontenelle (soft spot) was sunken in and she had lost an entire kilo, which is 10% of her body weight. She also had blood in her stools. Basically, she looked terrible. They immediately began fluid resuscitation and antibiotics. Also, they transferred most of her oral meds back to IV in order to give her gut a break, as well as to make sure they were being absorbed properly since things were moving through her so rapidly. In the absence of any obvious explanation, the doctors were tentatively implicating a viral infection, however, they ordered all of the usual bacterial cultures. Of course GVHD, as always, was in the back of everyone's minds, but the absence of any other signs made it seem unlikely. Dr. Peters felt it could be attributed to the cumulative effects of three rounds of chemotherapy and the radiation taking their toll on her intestinal tract. Still the viral aspect weighed the heaviest on everyone's mind. Additionally, her counts took a dive on Tuesday (WBC-1200 and ANC-800), which in an immunocompromised patient, is often indicative of a virus, as opposed to a bacterial infection which tends to drive counts upward. At any rate, a little growth factor (G-CSF), which she had been off of for a week, seemed to do the trick and by Thursday her white blood cells were in the 7000 range. Finally today, we have an answer. One of the cultures taken in clinic last week, grew out a pesky little thing called Adenovirus.

For the first time in two transplants, Susannah is really looking and acting sick. She is pretty listless and not interested in much other than sleeping and being rocked. This is quite a change for her and certainly does not go unnoticed by the nurses and doctors who know her well. The good news is she has been fever free since Sunday night and has not vomited since Monday, although as best as one can tell in a baby, she continues to have nausea and stomach cramps. So, as it stands, she will remain in the hospital until her stool output is under control and her appetite, along with some weight, returns. Since Adenovirus can often suppress the appetite, and because she is already in the middle of her steroid taper, the doctors really want her to build up some reserve. Adenovirus can be difficult to shake, often taking weeks or even months, so they are also evaluating the need for supplemental nutrition during this time (IV or tube feedings). Dr. Ramsay, the current attending, said it could easily be another week before Susannah will be able to leave the hospital and she is certainly not making any promises. In someone her size, as things can change so very quickly, they tend to err on the side of conservatism.

As for the social butterfly, Madeline, she and Grammy have been busy about town. Amy did manage to escape the hospital on Tuesday to take her to her first ballet class (thanks to Grammy staying with Susannah). Madeline was a star pupil and loved every second. I guess it was a pretty adorable sight, all of those little girls running around in their pink ballet gear. Tuesday also marked Madeline's first snowfall. She ran straight out of class and began making snowballs in her tights, never mind that it was freezing! Thankfully, our Care Partner, Sue Reichert accompanied (and navigated) the girls that day, so she was able to instruct Madeline on the art of making and throwing snowballs. Oh, speaking of Sue, the five of us made the hospital newsletter! They were writing an article on the Care Partner program, and used us as their model family. It was pretty neat, our picture was in it as well. Madeline is quite excited about being in the "newspaper" and, of course, we have plenty of copies.

I will not be returning to Minneapolis until Thanksgiving. It's amazing how quiet the house is here without the constant whirl and swirl of Amy and the girls. At least I'm not stuck in the hospital like Amy. She is truly a trooper. No one ever said that this process would be easy, but it certainly takes a toll on your patience and emotional well-being. Luckily, Grammy was still around when I left, otherwise we wouldn't have had anyone to care for Madeline at night, and that would have been really tough.

Well, that's the latest and we'll continue to keep everyone up to date.

With all our love,

Klane, Amy, Madeline and Susannah

Thursday, November 2nd, 2000 ~ Day +24

100% Donor!!!


Hi All,

Just a quick update to share our wonderful news.

We learned this morning that Susannah is 100% engrafted with her donor's marrow!!! This is the exactly news that we have been hoping and praying for and it comes as a great relief. While she still has a long road ahead of her, we now know that she is heading in the right direction.

Of course, the other big news is that we received Susannah's engraftment results as outpatients. She was discharged from the hospital yesterday afternoon! We are all back at the apartment now, still settling in at this point, but very happy to be "home" and together.

We will send a more thorough update this weekend, with details of Halloween and otherwise.

Love always,

Klane, Amy, Madeline and Susannah

Saturday, October 28th, 2000 ~ Day +19

Never a Dull Moment


Hello Again from Minneapolis,

As life is rarely dull here on Unit 4A, this last week was no exception. Where to start?

Well, on Monday afternoon I flew to Phoenix for the WOA meeting. As I mentioned last time, I was able to stay with Eric and Nancy who were extraordinarily gracious hosts. Nancy drove me to and from the airport, they took me to dinner, and even let me park myself at their place overnight. Eric and I poured a few Guinesses, and the three of us had a nice visit.

It was truly a whirlwind trip for which the final details only came together the week prior, because I couldn't predict what was going to happen with Susannah. I bought my ticket six days out. First, we called a travel agent who informed us that it would cost $900 round trip, which was of course, out of the question. So, I put a bid into Priceline.com for $200. Believe it or not, I actually got it -- a round trip ticket on America West, non-stop. Now, if any of you have ever used Priceline.com, then you know that you don't exactly have a choice of flight times and if your bid is accepted, then the tickets are yours. Period, no exceptions, with little flexibility. The only glitch was that my return trip was scheduled to depart Phoenix at 12:26pm and my talks were scheduled to end at 11:30am. The talks themselves went very smoothly, and seemed to be well received, but the meeting ran 15 minutes behind! So, with Nancy waiting in the getaway car, we made our way to the airport. I ran to the gate, in my suit, two suitcases in hand, arriving at 12:20pm -- "Is it too late to get on the plane?!". The gate attendant then informed me that our plane had actually just arrived and would be ready to board in a few minutes, I almost kissed her! Anyway, I made it back to Minnesota, on my scheduled flight, early Tuesday evening.

Meanwhile back at the barn, err, hospital, Susannah's central line seemed to have had some problems during my absence. During a routine blood draw, Jen, one of our night nurses, heard a "pop" and then noticed that one of the ports on the external part of the line had busted. Amy awoke the next morning to find the line adorned with two clamps, one on each end. Dr. Saltzman came up a few hours later and repaired the line in the room. It now seems to be working again and hopefully we won't need to replace it. Much to our nurse's dismay, he did not perform his infamous Barney rendition. He did, however, again go above and beyond the call of duty by recommending a ballet school for Madeline, convinced she should be in the same class as his three year old. He even took the time to call around for the phone number!

The real bummer about this incident was that two nights later, Susannah spiked a fever. As usual, routine blood cultures were ordered. By Thursday, we had a preliminary reading. We were told that it was "gram positive cocci, probably an indolent Staph species, Staph epi". Well if this were true then it would have been no big deal. Being nosey, I looked on the computer myself and read, "gram positive cocci in chains and pairs". As we learned in the first year of medical school, this is not classically Staph, but Strept. I reminded the staff that Susannah was colonized with (the dreaded) VRE. Enterococcus is a cousin of Streptococcal bacteria (in fact, otherwise known as Group D Strept, or Strept faecium, which on gram stain classically appears "in chains and pairs"). Well, regardless of what I thought, they thought that it was still likely to be Staph. Of course, the next day the culture was speciated as Enterococcus, and today as vancomycin resistant Enterococcus. So, Susannah indeed has a VRE blood infection. The most likely cause being contamination by skin flora when the line ruptured on Tuesday.

The good news in this turn of events, if that is possible, is that Susannah's white blood cell count has continued to climb, now at 1500 cells/mm3 with an ANC of 1400 cells/mm3. She has
remained afebrile since starting her antibiotics (vanco, tobramycin and ceftazidime) and all of her subsequent blood cultures have been negative to date. With this high of a white count and the addition of a fairly new antibiotic called synercid (she is now off the vanco, as there is obviously no need for it), we will hopefully be able to treat her infection without removing the line. As a parent, I'm happy to avoid the OR and anesthetic risk for a line change, yet as a surgeon it makes me nervous to leave the line in with an infection brewing. I called Dr. Saltzman on Friday, before we knew for sure what was going on with the VRE, and he was comfortable leaving the line in place and attempting to treat through the infection, but wants to evaluate it on Monday. The Infectious Disease people seemed to agree with this plan as well. As long as everyone is on the same page, I guess that I can go with it, but the tension level here is stepped up a notch or two. Surprisingly, Amy has taken all of this pretty well. You know how she feels about VRE. I'll tell you what though, she sure knows how to keep the staff on their toes, in a way that only Amy could!

Throughout this crazy week, Dana, "Sugar" and Matthew have been keeping Madeline occupied with a list of fun activities. We're especially lucky that they're here now, because we've had very little time this week to focus solely on Madeline. We still make every attempt to sneak in a snuggle here and there though. She's a tough little trooper, just like her mom. On top of everything, the poor thing has had a stomach flu and has thrown up three of the last five nights. Also, it appears that last night, her eardrum burst. Around 10:00pm she began complaining to Amy that, "My left ear hurts, Mommy, not my right one, just my left one.". She later woke me up at 4:00am, with more pain, but this resolved with Tylenol. Then this morning when she awoke, there was pus draining from her ear. She had tubes placed just seven months ago, so hopefully the drainage is coming through those and her eardrum didn't actually perforate again. We'll have her seen by the ENT this week, who just so happens to be friends with her ENT back in San Diego -- small world. Otherwise, Madeline has had so much fun with Dana and the kids that she really is taking all of this in stride.

So, life presses on here. If events progress smoothly without any further surprises, Susannah may be discharged to "temporary house" this upcoming week. She seems to once again be eating fairly well, and has developed a new love for Pediasure, with all of its protein and calories. Her blood pressure is still somewhat of an issue. She is back on Lasix and now is on another blood pressure medication, enalapril. This cocktail of nifedipine, amlodipine, prazosin, enalapril and Lasix was the same one she ended up on last time. So, hopefully this will again be just the right mix.

Monday is Day +21, the day they draw the RFLP, which is the DNA test for engraftment. Although Susannah's counts seem to be doing all the right things, in light our last experience with infection and engraftment, we can't help but be nervous. We should have results by Thursday or Friday, so keep up those prayers!

On that note, I just want mention one of our little neighbors. Jeffrey Shannon, an eleven year old boy with Adrenoleukodystrophy, passed away today. Jeffrey had undergone two transplants as well, and was last admitted about the same time as Susannah was for her FIRST transplant. As such, we had gotten to know his father, "Big Jeff", pretty well. Please keep Jeffrey's family in your thoughts and prayers, as well as all of the other kids up here on the unit, for they all have equally, if not more, tragic stories than our own. It's a skewed world when you are simply thankful that your child is alive, and not on a ventilator. It certainly makes you appreciate what you have.

Anyway, sorry for the downer. We'll just keep pluggin' along here with all of the wonderful support and positive vibes that everybody is sending our way; you're definitely keeping our spirits up!

Love,

Klane, Amy, Madeline and Susannah

Sunday, October 22nd, 2000 ~ Day +13

Cells, Cells, Cells!!!


Hello Everyone!

The last week has been pretty much a waiting game, hence the lack of updates. We know a few of you were getting anxious, so we apologize for the long silence ... but just remember, in our current situation, no news is generally good news. Better news, of course, requires a note of some kind. So, here goes it.

On Friday, Day +11, Susannah started showing the first sign of new bone marrow, heralded by the detection of circulating white blood cells. She went from the lowest detectable amount of 50 cells/mm3 (normal = 3000 cells/mm3) on Friday, to 100 cells/mm3 yesterday and today. We just hope that they continue to come back a little quicker this time, and from the right person! All of our doctors had bets going as to what day her counts would begin to come in -- Dr. Baker, the current ward attending, was going with Monday, Day +14, while Dr. Peters was leaning toward Sunday. Bright and early Friday morning, Amy awoke to a beaming Dr. Davies standing over her bed and I believe the quote went something like this, "I've just come to gloat, Day +11 is very tasteful indeed -- not too early, not too late."

Aside from the excitement of counts, the other big news of the week occurred Thursday, Day +10. Susannah is, once again, bald! After a couple of days of shedding, she woke up Thursday morning having rubbed off all of the hair from the crown of her head, thus leaving her with a mohawk! Needless to say, we decided it was finally time to help her along and engaged one of our nursing aides, Sarah, to play barber. Susannah sat patiently in the high chair, while Sarah buzzed away what was left of her hair. The new style is quite becoming and, if possible, is even cuter than last time she was bald. An added treat this go around is that, while her head is now bald, she continues to have the CSA fur all over her little back. There are three other families on the floor right now who have kids undergoing their first transplant for Hurler Syndrome, and every few days one of the parents inevitably comes by asking to show a visiting relative or friend the famous fur that their child is in store for! Little Susannah, oblivious to their good natured gawking, loves the attention from all of the people at her window.

Although things have been going well for the most part, this round has definitely been a little tougher on Susannah than last time. She has thrown up quite a bit more and is suffering from open sores on her behind, requiring morphine at times to help her sleep. As for eating, the last few days have been slow on the nutrition front. Fortunately, Amy has been a trooper and, on the advice of Drs. Peters and Wagner, has continued nursing Susannah up to now, just to get her through this difficult phase. You have to understand that if it were not for the transplant, Susannah would have been off the boob eight months ago. Thanks to this perseverance, good ole mother nature has allowed Susannah to make it this far with out requiring any supplemental nutrition (TPN or NG feeds) and Dr. Wagner is convinced that breast milk definitely has a diminishing effect on mucositis, hence Susannah's relatively minor complications in this area. Amy has, however, had to step up the number of feedings to compensate for Susannah's current lack of appetite for solid foods. This involves late night and early morning feedings, so the two are basically on the schedule of a newborn. Realizing that both of our girls slept through the night at two weeks old, this new schedule is really draining on Amy. Luckily, I am able to relieve her during the day so she can catch a few extra winks. I compare Amy to the mother octopus who locks herself up with her babies never eating or sleeping; doing everything possible to ensure the best outcome for Susannah.

Just like last time, Susannah is again battling CSA and steroid induced high blood pressure. She has already maxed out one anti-hypertensive medication (amlodipine) and another was added today (prazosin). She also continues to need prn doses of nifedipine to help manage her spikes. Previously, it took five different meds (all of the above, as well as, enalapril and Lasix) to get Susannah's pressures under control. While there is a set regimen for managing high blood pressure up here, Dr. Baker is looking into some alternative options. Hopefully, by introducing different 2nd and 3rd line drugs early, we can get things under control more quickly and use less medications.

As far as transfusions go, since we already knew from before that Susannah had some platelet antibodies, we were able to begin using the single donor cross-matched platelets immediately. This has, for the most part, kept her platelet transfusions to every other day. She has also been maintaining a relatively stable hemoglobin and as such has only been receiving red cells every seven to ten days.

This morning Amy had a little scare when, while eating toast, Susannah started bleeding from her mouth. It seems that she probably scraped a sore on the back of her throat, and with her platelet count extremely low, had a small bleed. Blood always seems to appear more substantial than it really is, but maybe that is just the fear factor of seeing it. Until today, Susannah really showed no signs of mouth sores or persistent mucositis, but perhaps this sheds some light on a few recent bouts of otherwise unexplained fussiness and discomfort which were severe enough to require morphine.

While an inpatient, Susannah, although tired, continues to do well with occupational therapy and physical therapy. She is on the verge of walking independently. OT has declared her "age appropriate" and as such, they are now only going to see her three times per week. When not working with therapy, Susannah remains quite active and requires three extensions on each IV line to allow her the necessary freedom to roam around her room. We've coined her activity about the room as "dinking". She moves from here to there dinking with this and then dinking with that. It's sort of a putzing around that she does and is quite cute.

The video "Baby Faces" is again the hit it was last time we were in the hospital. Now, however, Susannah is able to ask for it by signing, and sign she does! She constantly is pointing at the TV and signing "want", followed by "babies" and then "please". She is not only quite good at asking for what she wants (and she wants everything!), but is very polite about it. You know how Amy feels about the television, but even she finds her little "please" impossible to resist. In true 18 month old fashion, Susannah also definitely knows "yes" and "no", usually with quite an attitude. Her communication skills are coming along really well, particularly since returning to Minnesota.

For the first time in three weeks we are grandmotherless. Grammy left last Saturday and Grandma Dona flew home on Friday afternoon. Madeline had a wonderful time and we really appreciated all of their help and enjoyed their company. Yesterday our friend Dana Jacobs arrived from California with her little ones, Casey (aka "Sugar") and Matthew, in tow! To say Madeline is ecstatic would be an understatement and we are pretty excited to have a friend around too. Susannah is very attached to "her Dana", so it will be a real treat for her as well.

I will be leaving for Arizona tomorrow afternoon to present two papers at Western Orthopaedic Association's annual meeting. This engagement, planned months back, was originally intended to coincide with a week of vacation with Amy and the girls. Obviously our plans have changed since then, but the speaking schedule has not, so I will be making a very quick trip out West. As Susannah's condition continues to be very tenuous, I will fly out on Monday afternoon and then leave Phoenix immediately following my presentations on Tuesday to be back in Minneapolis by Tuesday evening. It will be quite a whirlwind trip, but I will at least get to stay Monday night with my long time friend Eric Stoermann and his main squeeze, Nancy. It will be great to see them.

We will spend the next week or so anxiously awaiting further return of Susannah's white blood cells. Her present white count is too low to even calculate the differential (ANC portion), but just as before, she will need to at least have an ANC of >500 for three consecutive days in order to be discharged from the hospital. This can happen relatively quickly, or the counts can drag their heels a bit. We can't help but be superstitious and hope for an opposite course from the one in May, so at this point we are hoping the cells continue to multiply at a rapid and steady rate. Engraftment testing (RFLP), to determine to whom the growing marrow belongs, is usually completed on Day +21, with the results following a few days later. So, in the meantime, please continue to pray for 100% donor cells, as it is these cells that are ultimately needed to provide the enzyme that Susannah lacks ... the whole reason for doing the transplant.

Love,

Klane, Amy, Madeline and Susannah

Tuesday, October 10th, 2000 ~ Day +1

The Marrow is In!


Hello All,

Transplant number two is in! Susannah's donor marrow made it into the Twin Cities at 4:00pm yesterday afternoon. It took about six hours of transport and processing before the marrow made it's way up to 4A. At 10:55pm the infusion of bone marrow began and was complete by about 11:30pm -- the star of the show was awake and playful throughout.

As for the weekend, Susannah started radiation therapy early Friday morning. We met anesthesia and nursing at 6:00am. She then went down to Radiation Oncology and received her first course of TBI without a hitch. Since she is so young, it was necessary to sedate Susannah for each session. As I mentioned in the last update, Susannah was also scheduled to have her central line replaced on Friday. In order to avoid subjecting her to three anesthetics in one day, Surgery, Radiation Oncology and Anesthesia were able to coordinate things in the afternoon, so that immediately following the line change, Susannah, still sedated, was whisked back down to Radiation Oncology for her second session of the day. As no news is good news, you can guess that there weren't any complications from the surgery, and as of now her line still seems to be working. Dr. Saltzman said that he found a clot at the tip of the catheter and thought that perhaps the line hadn't been appropriately flushed at some point. As a surgeon, I am sure he was looking for a reason why "his" line failed. The nurses, of course, blamed the doomed Hickman on its placement. Whatever the cause, it is sort of irrelevant, because what is done, is done, and the line is now working. As for Susannah, she found the day exhausting and slept from 4:00pm until 7:00am, never even waking for nighttime vitals.

On Saturday and Sunday, Susannah continued the twice daily course of radiation, as well as the ATG. Happily, this went very smoothly. She did develop a fever immediately following her first dose of ATG (the same thing happened last transplant) and as such was started on double coverage antibiotics (vancomycin and ceftazadime). While everyone is 99.9% certain that the cause of the fever was just a reaction to the ATG, as it is derived from horse serum and as such commonly induces allergic responses, the motto around here is better safe than sorry.

Susannah continues to be in good spirits, but is growing increasingly fatigued as is made apparent by her pre-nap irritability, followed by longer than usual naps. Part of this may also be related to the fact that, as we discovered the other morning while she was sedated (thus she actually allowed Amy and I to look in her mouth), Susannah has finally cut some teeth, more precisely five! Poor thing, what a time to finally get her teeth. On top of the new teeth, Susannah's mouth tissue is beginning to break down (mucositis) and she is also developing a "sun burn" all over her body from the radiation. In a way, the shorter regimen of cytoxan/TBI almost seemed easier than dealing with the oral Busulfan/cytoxan prep from last time, but I suppose in a few days when the real side effects of the radiation kick in we'll change our minds.

Father Grant Abbott came by last evening to bless Susannah prior to her transplant and celebrated communion with us. Laura Kelley, the hospital chaplain, wrote a nice prayer ceremony for the occasion, which we read together and integrated into the communion. As I've mentioned before, Grant, Lyn Lawyer and the rest of the St. Matthew's Parish have been a great source of strength and support for us throughout this process by providing us with a family away from home. We truly appreciate everything they have done and continue to do.

The BMT itself was a festive occasion as well. Our primary nurse, Kristy, was not able to be there because the marrow arrived earlier than expected, before her shift. You may recall, she gave Susannah her bone marrow last time. Jen, also a favorite member of our nursing team, filled in nicely, and was even able to take a Polaroid shot with us centered in the picture! Kristy, although a great nurse, is not known for her photographic prowess! Susannah received a teddy bear with a balloon and a BMT Day poster from Child Family Life. Also present for the big event was Grammy, who probably provided the most entertainment for all of us. I think that the excitement of being present at the transplant had her running on all cylinders. She was clapping and singing, making duck noises, anything to get Susannah to smile. It surely was special for her and for Susannah to have her present for such an important occasion.

This morning Dr. Wagner told us that this bone marrow dose was five fold greater than the last. We'll take every advantage that we can get! This morning the lab completed the marrow analysis and determined that the elutriated marrow that Susannah received last night didn't include quite enough T-cells, so she received an "add back" of marrow consisting of a few extra T-cells and better yet, more stem cells. "Add backs" are very common, especially in the case of second transplants when they allow for a greater number of T-cells in the donor marrow, so as to increase the chances for engraftment. This, as you might remember, does come with an increased risk for graft versus host disease (GVHD), but the second time around it is even more important that the transplant engraft; the risk is carefully weighed. The number of T-cells that are actually added back is quite minimal and is calculated tediously to try to add only the amount necessary to do the trick and not induce GVHD. The elutriation or T-cell depletion process is the main reason we decided to come to Minnesota, since GVHD is especially problematic for patients with Hurler Syndrome and the process has cut down on the problem immensely. Dr. Wagner happens to be the one who developed the technique, so we are in the best possible hands right now.

Madeline returns to Minnesota in two days. Many thanks to Joe for flying back with her. He will do an immediate turn-around back to Boston and having done this myself a week ago I feel for him, so we really appreciate it. Madeline is very excited about her travel partner. In fact, I think he even beats me -- from what we understand, Madeline has been quite infatuated with Uncle Joe this trip, preferring him to do everything for her. We continue to receive phone calls each evening with Madeline being completely exhausted. She is ecstatic as she recounts the fun-filled events of her days with "Justy", before she literally falls into bed. We are anxiously awaiting her return on Thursday.

Grandma Dona will be arriving tomorrow evening and Grammy will leave later this week. The two may overlap by a couple of days -- should be interesting to see who is lucky enough to get the top bunk! I'll give ya one guess?! On second thought, Grammy will probably just sleep on the floor where she is happiest. Two Grandmas surely will be very exciting for Madeline. She loves them both very dearly, and we really appreciate all of their help. Susannah, of course, enjoys the extra cuddles.

Before I close, we would like to take a moment to express our appreciation to the kind, anonymous man who has so selflessly given our little girl this wonderful gift. Words simply cannot express our gratitude. We would also like to, again, thank all of you for the outpouring of love, kindness and prayers you have constantly been providing us. We will, of course, continue to keep everyone informed and up to date as events unfold. Usually at this point, it is a watch and wait game, with symptomatic care until we start to see counts sometime between Day +7 and Day +21. In the meantime, please pray for 100% donor cells!

Love always,

Klane, Amy, Madeline and Susannah

Thursday, October 5th, 2000 ~ Day -4

Susannah’s BMT, Take Two


Hi Everybody,

Time for an official update on Susannah and the rest of the White family here in Minnesota (and Massachusetts). This will be a particularly long update, so for those of you interested in the short version, here it is:

Susannah made it through work-up week relatively uneventfully. Her heart was found to have a small residual communication between the aorta and the pulmonary artery known as a patent ductus arteriosus (PDA). Her neuropsychological assessment showed that although she is still mildly delayed, she has made up a lot of ground recently and the therapies seems to really be helping her development. Otherwise everything else checked out okay, and generally everyone was pleased with her current status. On Monday she was admitted to the hospital, after having her central line placed, an ophthalmological exam, an MRI and a CT scan of her chest. She tolerated all of this well and Tuesday started chemotherapy, with no real complications to speak of so far. Tomorrow, Susannah begins radiation, but unfortunately she will also have to make a trip to the OR to have her central line re-wired as it is not drawing properly.

Now for the whole story ...

Amy, Madeline and Susannah flew back to Minnesota on Sunday, September 24th. Let me tell you, this was no ordinary plane flight. Through a charitable foundation known as Corporate Angel Network, the girls were hooked up with the sweetest of planes. Corporate Angels works with the hospital to connect families of sick children with private corporate jets that are flying to similar destinations, so as to provide families with a free flight, away from the crowded cattle cars of commercial airlines. As it goes, Amy was set up with the CEO of American Express's Financial Services division and the company's Gulfstream jet. This plane was plush to the gills. The girls even dined on specially prepared meals, Caesar Salad with Filet Mignon for Amy and Mac & Cheese for Madeline -- they even asked what brand of yogurt Susannah preferred! As she ate the Kraft out of china with silver, Madeline watched Mary Poppins on her own personal video monitor. The hosts and crew were extremely gracious, and even brought small gifts for Susannah and Madeline. They had a great time.

After arriving in the private hanger of American Express, the girls made their way back to "temporary house" and settled in without much ado. A big thanks to Karen Stoddard from St. Matthew's for getting them (and their luggage!) there safely. Starting Monday of last week, Susannah went through a somewhat reduced battery of pre-transplant tests and consults. She was also fitted for lung and brain protective blocks for radiation therapy.

Work-up week results:

Pulmonology - No obvious sequelae of the chemotherapy from last time. The pulmonologist ordered a CT scan of her chest to better delineate what damage, if any, was inflicted on her lungs. We still don't have the results from that test yet.

Cardiology - Her EKG remains normal. She had an echocardiogram which showed some new findings. In addition to the slight mitral valve insufficiency that they found last time, she has some new aortic valve insufficiency. It's unlikely that the transplant will help these issues and in the distant future, she may need open heart surgery to replace one or both of these valves. As I mentioned earlier, they also found that Susannah had a patent ductus arteriosus, which is a vestigial remnant of her prenatal circulatory system. It was apparently small, 1-2 mm in diameter, and of minimal consequence at this time. Dr. Braunlin, the cardiologist, did indicate, however, that at some point after the transplant, we would probably want close it to prevent infection. This would likely be done with a "coil" placed by the interventional radiologists. A relatively small procedure, although we are coming to realize that such a thing doesn't really exist for these kids.

Neurology - Susannah is a little behind with the development of her gross motor skills, but is continuing to improve at a good pace. Dr. Lockman is a pretty funny guy. I understand that he and Susannah had a nice meeting and shared a NutriGrain bar as they "chatted".

Neuropsychology - As usual, they planned to test Susannah in the middle of her naptime, but Amy, knowing better this time, cancelled the appointment and made them reschedule for a more conducive time to Susannah's circadian rhythm. What a surprise! She suddenly is performing near age appropriate levels -- it's amazing what a little nap will do. What you have to understand is that these people are notoriously cynical, and as such, rarely have anything nice to say. So this meeting was actually a pleasant surprise, with which we were pleased. Truthfully, Amy and I both believe that Susannah's continuation of intensive therapy has really helped in all aspects. She has excellent fine motor skills, although her fingers are a little tight; she continues to improve in her gross motor skills; and she now signs/expresses about 30-40 different words.

Ophthalmology - The exam under anesthesia revealed slightly increased corneal clouding, but no evidence of glaucoma. Dr. Christensen didn't necessarily think the clouding was affecting her visual acuity, but said it definitely makes her more sensitive to bright lights (no wonder she hates to have her picture taken). He recommended that she be formally evaluated for visual acuity at some point following her transplant, just to be certain.

MRI/Lumbar Puncture - Susannah's spinal pressure was in the normal range, several points below it was in April. Her MRI showed no evidence of hydrocephalus, but we are still awaiting the final reading.

ENT/Audiology - Her middle ears still look really good, with no evidence of effusions. This is pretty unusual for Hurler Syndrome, as chronic ear infections are a hallmark of the disease. As for the audiologist, she just reaffirmed what was assessed before leaving Minnesota, as well as in San Diego -- that the hearing aids seem to help a great deal, putting her within the normal range.

All specialists agreed that Susannah is in good shape to proceed again with the transplant process. Her liver and spleen are only slightly enlarged and the effects of the Hurler Syndrome on her little body seem to be relatively mild, especially considering her age. As far as her heart goes, while there are definite findings, these are common issues of the disease and are often much more pronounced in other kids her age.

During the busy work-up week, Madeline was cared for by our contingent of college student neighbors/sitters, as well as our Care Partner, Sue Reichert. Madeline got to go on her first apple picking adventure, among other activities and had a really fun week.

I arrived on Friday evening. That was the day that we learned about little Maddie Moore. Amy was alone when she found out, but luckily I arrived within a couple of hours so that we could be together at such a terrible time. Of course we're still devastated by her death. She was doing so well, just days from going home when this all began. Also, the fact that all this ensued as the result of a "minor" procedure, makes one realize just how fragile these kids actually are. Things happened very quickly, so we didn't get a chance to say a real goodbye to Maddie or her parents, all of whom with which we have become quite close. We really miss them, but at this time want to give them some space to separate from the transplant process and Minnesota, and allow them to grieve as they need to. Anyway, I don't want to dwell on the issue, but it's sort of hard to overlook something that is always on your mind.

On Saturday, Madeline and I flew to Boston. She is becoming such a big girl -- as we started to say goodbye to Amy, Madeline said "Let's go Daddy and don't worry Mommy, I won't cry." and she didn't. We had a very nice trip together and played lots of "Go Fish", although she was a little puzzled as to why she couldn't watch a video on this airplane! I got quite a workout during our transfer at O'Hare, as our gates were about ten miles apart and Madeline was asleep on my shoulder. As Amy elaborated on in a previous message, we feel that Madeline's being with the Calaresos will be very good for her while Susannah undergoes the most time intensive part of the BMT process.

I was in Boston for just over 12 hours, before I headed back to Minneapolis. Madeline was already having such a blast that she didn't even want to see me off at the airport! She has steadily continued at this pace, as every night when we talk to her she is completely exhausted and says she wants to go to bed. Madeline NEVER wants to go to bed so, this a good sign indeed.

I returned back to "temporary house" Sunday evening and we left for the hospital at 5:30 the next morning. Susannah had her big day in the OR and we were up on 4A by 2:00pm. Apparently we have been given the 2nd transplant bonus -- our room, last time called the shoebox, is now affectionately referred to as the "ballroom". Seriously though, last time we had a pretty small room with a "view" of a brick wall. Our new digs, in addition to being huge, have a nice view of campus and the beautiful fall leaves. Which reminds me, Amy and I were talking the other night and realized that by the time this is all through, we will have experienced all four seasons here in Minneapolis ... of course, the best is still ahead of us with the 20 below winters! At least we will be assured a white Christmas.

So, back to Monday. We weren't on the floor for 30 minutes before we found out that Susannah is colonized with VRE (vancomycin resistant Enterococus). VRE is a bacteria that is resistant to basically all antibiotics, but is not very infectious, unless of course, someone is immunocompromised, as Susannah will be after her chemotherapy, radiation and immunosuppressive drugs. The bitter irony of this situation is that the last time we were here, there were four or five kids who had active VRE infections, and we were complaining to anyone who would listen, that some of the families were not adhering to the simple gowning and hand washing precautions. We were, of course, scared to death that Susannah would become infected and then we would have no real recourse for treating her. A few years back I lost a patient to VRE and of course, Amy remembered this (imagine that) and thus became the VRE police. As a result, there was a change in policy and the current admission work-up now includes swab cultures for VRE. Low and behold, Susannah is colonized -- gee, I wonder why? Now we get to wear gowns and gloves at all times in the room. Since Amy is still nursing Susannah, this is somewhat impractical. As a result Amy has gained a special exemption from the Infectious Disease folks to ditch the gown and gloves, but is required to take certain precautions when leaving the room, particularly when using common areas such as the kitchen and lounge. I still wear my gown at all times in the room, but have been cleared to only wear gloves when changing Susannah's diaper, and I make sure I wash my hands regularly, especially when leaving the room. VRE is a really tough little bacteria and can live on hard surfaces for up to seven days, so it is unlikely that this little isolation thing will be short lived. Hopefully, Susannah will continue to only be colonized for VRE and the situation will remain a mere nuisance, as opposed to it becoming an active infection.

On Tuesday, Susannah began chemotherapy with high dose cytoxan. Of course, this would not be complete without adding all of the other meds to counteract the side effects and to prevent toxicity. So, Susannah is already sporting quite an impressive IV pole. Also, along with cytoxan, comes every two hour diaper changes, day and night. I'm sure you can imagine that this little routine makes nighttime a real treat for Amy, so she is a little tired, but seems to be holding up.

Today is our day of rest ... a day off, so to speak. On Friday, Susannah will begin her TBI (total body irradiation) regimen. This is done in seven segments, over the course of four days and she will be anesthetized for each session. So, essentially, twice a day for three days and once more on Monday. No one is looking forward to it, but it must be done. Radiation is much scarier than the chemo regimen from last time, due to the anesthesia risk and both the short and long term side effects. Susannah remains in great spirits, but it shouldn't be long before things begin to take their toll. Hopefully her discomfort will be short lived and the chemo/TBI combination will do its job and prepare her little body to accept the new marrow.

There has been one minor glitch since being admitted. As of last night, Susannah's central line has been giving poor blood return. This means that the nurses are unable to use the line for the many blood draws that are required for monitoring Susannah's course. Dr. Saltzman (you remember, the Barney singing pediatric surgeon) placed the line in a somewhat unusual location near Susannah's left armpit. He put it here so as to hide the scar for, as he put it, future bikini wear. I'm not even sure that my girls are going to be allowed to wear a bikini, or date before the age of 30 for that matter, but it was a nice idea nonetheless. Whether or not this location is a factor in the line problems is debatable, but the nurses have been in a ruckus about the unorthodox location (as well as his reasoning) since they first laid eyes on it. Anyway, tomorrow, just before her second TBI session, Susannah will be making a quick trip to the OR to have her line replaced. Fortunately, the doctors were able to coordinate the two procedures, so as to not subject her to a third anesthesia in one day. The surgery is relatively simple and should take only about 30 minutes, but it is still a surgery and something we are not taking lightly.

As far as getting in touch with us, here's the deal. While our address and telephone at the apartment remain the same, we do have new numbers here in the hospital. We enjoy hearing from you all, so feel free to get in touch of us any of the following ways:

In the hospital --

Susannah J. White
Fairview University Medical Center
University of Minnesota
BONE MARROW TRANSPLANT UNIT, Room 4-207
Harvard Street at East River Parkway
Minneapolis, MN 55455

(612) 273-0224 (direct dial to our room)
(858) 967-4895 (local for those in San Diego)
(612) 273-3000 (switchboard - ask for 4-207)

At our apartment --

Amy, Klane, Madeline and Susannah White
900 Washington Avenue, SE
Apartment 104
Minneapolis, MN 55414

(612)362-9378
(858)967-4894 (local for those in San Diego)

Via e-mail --

kkwhite@ucsd.edu

By the way, in case anyone is confused, since we have now begun the transplant process once again, our calendar starts fresh. We are back to counting days in negative numbers, Day -7 through Day -1 for the preparative regimen. As you can see from above, today is Day -4. Once transplant day, Day 0, has come and gone, we will again begin counting upward from Day +1.

We hope all is well on the outside world, and please keep in touch.

Love,

Klane, Amy, Madeline and Susannah

Sunday, October 1st, 2000

Update from Minnesota


Hello Everyone,

We write tonight with very heavy hearts. Our friends, the Moores, lost their little Maddie on Friday afternoon. While we can't even begin to imagine their pain, our hearts break for them. No parents could have loved their child more.

Heather and Rocky have been a tremendous source of strength for us these past months. Their companionship truly made our first experience here in Minnesota bearable, as our friendship reached well beyond the boundaries of our common situation. We will miss them terribly and especially their little angel.

We had planned on writing an update this weekend, but now simply cannot find the words. As our heads clear, we will update you on the details of Susannah's work-up week, as well as her upcoming preparative therapy. In the meantime, we appreciate you keeping Susannah in your thoughts and prayers as she goes to the operating room tomorrow. Please also remember the entire Moore family during this most difficult time.

All our love,

Klane, Amy and Girls

Saturday, September 21st, 2000 ~ Day +147

We’re Off!


Hi All,

Well, our plans have indeed been pushed back by a week, but they are actually finalized and that is a great relief. The new donor has received final physical clearance and Susannah's second bone marrow transplant is scheduled for October 9th.

The girls and I will leave San Diego on September 24th and begin work-up week on Monday the 25th. The pre-admission week tends to be very busy and emotionally draining, so Klane's absence is certainly going to take its toll. However, with his limited time off, it is far more critical that he be with us throughout the inpatient stay. Klane will definitely fly into Minneapolis in time to be present for Susannah's admission and OR procedures on Monday, October 2nd. She will begin preparative therapy the following Tuesday. The regimen, this time, consists of two days of chemotherapy (cytoxan), followed by four days of total body irradiation (TBI), with three more days of chemotherapy (ATG) interspersed with the radiation. As I mentioned earlier, the actual transplant date will be October 9th.

Just before Susannah is admitted, Madeline will head East for a very exciting adventure. She will spend the next week or so in Boston with my grade school friend, Wendy Calareso, her husband Joe, their baby Brianna and most importantly their three year old son Justin. She is quite excited to see "Justy" again and can't wait to play on both his indoor (gotta love the East Coast!) and outdoor swings with all the Calareso cousins. She will no doubt develop a deep love for true Italian cuisine during her stay!

Undoubtedly, we will miss Madeline something terrible. But, as the radiation adds a whole new and even more unpleasant element to the transplant process, it is more important than ever that she have distraction during this stressful week. While seeing Madeline's smiling face would certainly help make things easier on us, it wouldn't be fair to her. During radiation, Susannah will undergo anesthesia twice each day, once in the morning and once in the late afternoon, with the early afternoon and evening spent recovering the effects of the sedation. Also, because of the late afternoon anesthesia, she will not be allowed to eat anything for a good portion of the day. Sleep will be scarce and the side effects unpleasant. Needless to say, Susannah will require an inordinate amount of care during these periods and consequently, Madeline will be much better served in a fun and carefree environment. Of course we realize she will miss us and being separated will certainly be difficult on her, as it would on any 3 year old, but at least most of her day will be spent playing with her buddy, distracted from thinking about us. Unfortunately this is a case of the lesser of two evils and a decision we have not made lightly.

No sooner did I write the last update, than did Susannah develop another set of fevers. As fate would have it, Grammy happened to arrive for an overnight visit, just as the first fever spiked and just in time to spend Saturday evening with Madeline while we got to spend it back in the ER. Fortunately, Susannah had received a dose of G-CSF that morning, so her ANC was well over 1000 and she was able to narrowly escape admission. More cultures, more ceftriaxone, but still no clear cut answers ... just the mystery viral infection. The fevers continued steadily until Monday and then, like last week, they just disappeared. The blood cultures remain negative.

By the way, I keep forgetting to mention that Susannah has been completely transfusion independent since we returned home. Apparently, just removing her central line (and thus the bacteria colonizing it) gave her the boost she needed. Both her platelets and red cells have been stable at ~100,000 and ~10.5, respectively. When we left Minnesota her platelet count was just over 40,000 and by the second week in San Diego, it had jumped to 105,000. So, thankfully she ended up not requiring the transfusion center at Children's after all; this was quite a surprise to everyone, as she was fully expected to need at least one. We are, however, now quite familiar with the doctors and the process there, so things will already be completely in place and ready to go when we return next time. Ultimately, Children's truly is the best place to care for Susannah, since they are the only pediatric BMT center in San Diego County.

Some of you have been inquiring about the new donor. Like before, he must remain anonymous until one year post transplant, but the few details we are allowed, I am happy to share. This kind hearted soul is a 45 year old male who lives somewhere in North America. He is one of the original ten people (out of 40 matches) that were pursued for Susannah's first transplant, only his initial screening was not completed until after she was already in the hospital and another donor had been selected. We have been told on several occasions that he is very eager about donating to Susannah. To say we are grateful would be an understatement.

As we mentioned back in July, we are fortunate to be returning to the same apartment we lived in during our last stay in Minneapolis. It will be nice for all of us, especially Madeline, to have a familiar place with familiar faces to go back to. Since we left many toys and other essentials, all we will be packing this time is our clothing and a few other miscellaneous items. The move should be pretty uneventful, as "temporary house" will have been completely cleaned (thanks to the staff at Dinnaken House!) and waiting for us upon our arrival. We will have the same address and phone numbers as last time and once Susannah is admitted I will forward you her new hospital address and telephone.

Our next update should be from Minnesota!

Much Love,

Amy, Klane, Madeline and Susannah

PS … The following actually isn't a post script, but rather a subject matter warranting its own space. I didn't feel it appropriate to sandwich this between such trivial things as travel plans, meaningless fevers and address updates.

We would like to take a moment to ask you to keep our friends the Moores in your thoughts and prayers. You may remember them as our neighbors on 4A ... their little girl Maddie received her second transplant for Hurler Syndrome at the same time Susannah was going through her first. Well, just about the time they were finally scheduled to return home to Oklahoma from their stint in Minneapolis, Maddie unexpectedly experienced some serious complications, following a simple procedure. She is now in intensive care and requiring a ventilator to help her breathe, as well as dialysis to keep her kidneys functioning. Our hearts ache for Heather, Rocky and their baby -- they have been through so much and had come so very far. Maddie is a true fighter, however, and we have faith that she will win this latest battle tossed her way, but a few extra folks holding their family close, certainly wont hurt.

Thank you.

Tuesday, September 10th, 2000 ~ Day +136

Upcoming Departure ...


Hello Everyone,

With our stay here at home quickly drawing to a close, we thought it was time to bring you all up to date on our impending return to Minneapolis.

We received wonderful news last week in regard to the new donor for Susannah's second transplant. His enzyme activity level (the same enzyme that Susannah is missing and the whole reason for doing the transplant) is a whopping 57! Dr. Peters said this is one of the highest levels he has ever seen in a donor. This news was quite a relief, since as you may recall, for the first transplant we ended up changing donors at the last minute due to low levels.

At the moment, the plan is for Susannah, Madeline and myself to return to "temporary house" sometime in the next two weeks. While we originally planned on leaving San Diego on Sunday the 17th, there is a chance that the donor's physical exam clearance will not be completely finalized by then. This being the case, things may be pushed back one week and we will then leave on the 24th. If we leave on the 17th, we will begin work-up week (you may remember, this is the week in which Susannah is evaluated by virtually every medical specialist known to man) on the 18th. She will be admitted to the hospital for preparative therapy (this time she will undergo both chemotherapy and radiation) the week of the 25th, with her transplant being the week of October 1st. Should work-up week be delayed until the 25th, obviously everything would be pushed back one week. Hopefully we will have a much firmer handle on our departure date on Monday, when the donor's physical is complete.

Since Klane has very limited time off this go around, he will not leave for Minnesota until the week following us. Unfortunately, this means he will not be present for work-up week, but he will arrive just in time for Susannah's admission, which is a very big day in the operating room for her (about eight hours). He will remain in Minnesota for the next few weeks and, with any luck, Susannah will be out of the hospital before he is scheduled to return to work at the end of October. Of course, the best laid plans ...

As for the events of the last few weeks, although we don't get out much, there is certainly no lack of excitement here. While we intended Susannah's time home to be somewhat isolated, the whole seclusion thing was stepped up a notch after an unexpected exposure to the chicken pox. Fortunately, we knew the exact date of the exposure, so we were able to treat her with VZIG, an immunoglobulin shot intended to prevent an outbreak. This combined with her already hefty preventative dose of acyclovir, seemed to do the trick and she was able to ward off any sign of the pox. The perk in all this is that she earned herself a front row seat at clinic, bypassing the waiting room and receiving a private escort to the back.

Just as her 21 day Varicella quarantine was drawing to a close, our littlest princess spiked a fever and earned herself a late night visit to the Emergency Room. Thus we spent the better part of an evening (and early morning -- luckily, Anne Williams and girls had come down that day and were able to stay the night at our house with Madeline) at Children's Hospital running the usual battery of chest films, blood cultures and the like. Thankfully, all remain negative for bacteria, to date. However, she did receive an injection of ceftriaxone, a broad spectrum antibiotic, just in case.

Now, finally on the mends from her latest little illness, Susannah's white blood cell count has decided to take a dip. As of yesterday, her ANC dropped to 780, kicking her right back into full isolation (apparently, someone doesn't want us to become at all lax)! This came as somewhat of a shock as she has been well above 1000 for months now, but the doctors feel pretty confident that it is only a response to her recent fevers and a few doses of G-CSF (white blood cell growth stimulant) should do the trick. Unfortunately, since she no longer has a central line, the drug has to be given by injection. Poor thing has been getting more than her fair share of shots this past month. As it stands, her counts will be rechecked on Tuesday, after three consecutive days of G-CSF, and hopefully they will have rebounded. Of course, her counts will continue to be monitored very closely, especially considering she will soon be on an airplane, and if they are anywhere near 1000 as our travel date approaches, she will receive another shot just to be safe.

As for the mundane day to day stuff, we have been keeping quite busy in our house. Susannah is up to her eyeballs in therapy appointments, but the results have been tremendous! She is nearly walking on her own, cruising everywhere! She is pulling up on anything she can get her hands on, climbing up onto the couch using only our leg as a step and is currently obsessed with climbing in and out and around and under her car. There seems to be something about those covered plastic cars, as Madeline and her buddies had the exact same obsession at this age! Susannah also received her hearing aids this past month. While she is not exactly ecstatic about wearing them, she is quickly growing more accustomed each day. As long as she is wearing one of her hats (yet another obsession) she seems to forget they are in her ears. The great news is that when tested with the aids on, her hearing results were normal!

As for Madeline, she too has been very busy -- only she is the lucky one who actually gets to leave the house! Our friends and family have been absolutely wonderful in our efforts to keep Madeline's days full of excitement, so she does not become overwhelmed by the influx of therapists and medical types doting over her little sister. Her brief summer at "regular house" has been filled with trips to the beach (her favorite -- a true California girl), Sea World, the zoo, music class, Balboa Park and just about every other park in town. She is really enjoying swimming lessons and is quite the little fish! She has also become acquainted with a new favorite, a pool with a playground in it (what more could a kid need?)! Without a doubt, one of the most difficult things about our return to Minnesota will be taking Madeline away from her friends. She, however, is actually looking forward to returning to "temporary house" and is excited about the prospect of trick-or-treating and seeing Santa Claus there. Unfortunately, due to the numerous bugs and germs she would acquire, and thus bring home, the doctors have advised us against enrolling her in any kind of preschool setting. We will, however, continue to keep her involved in the weekly music class she attended during our last stay, as well as a dance class and Sunday school, to maintain some sense of normalcy in her life.

Just a side note ... while many of you may appreciate the very descriptive account of this whole process, I'm sure many others would be just fine without the trivial details and rather the straight facts. Please know that we are certainly not trying to bore you! These updates serve not only as a means of keeping our family and friends up to date, but we are also writing them so that both of our girls will be able to look back and understand this part of their life in the years to come -- a journal of sorts. We feel it is important for Madeline and Susannah to know exactly what transpired throughout this entire experience as it pertains to each of them individually and our family.

As our plans become finalized in the next few days, we will certainly keep you appraised. Until then, as always, we thank you all for your unending love and support.

Love always,

Amy, Klane, Madeline and Susannah

Monday, August 14th, 2000 ~ Day +109

Home Sweet Home


Hello All,

Home at last ... and how wonderful it is! After a uneventful flight home Susannah and I are finally back in San Diego and our family is together again. She did great on the flight, only taking her mask off to eat! Susannah is in her glory, thoroughly enjoying her big sister and all of the room to roam!

Susannah had her first appointment with the UCSD Pediatric Hematology Group last Monday and boy, were we in for a shock! Things started off smoothly enough as they knew immediately who we were and escorted us in through the back door to avoid the crowded waiting room. Four hours later, they finally drew her blood and informed us it would be two more hours before her counts would be back from the lab. Keep in mind, in Minnesota the first order of business is to draw the blood, so by the time the doctor comes in, they have the counts to refer to (on a good day there the counts take 15 minutes, on a bad day 45). Next we were informed that all of Susannah's transfusions would be given on an inpatient basis, as opposed to in the clinic. For platelets she would be required to be admitted for 12-24 hours and for red cells she would need to stay for 24-36 hours! Needless to say our jaws nearly dropped to the floor -- we are accustomed to her receiving all transfusions as an outpatient, with the time frame for platelets being 20 minutes and red cells not exceeding two hours. We were warned about the "culture shock" once we returned home, but certainly no one expected this. The last thing we expected during our time home were trips to the hospital.

Well, now one week later Susannah's interim care has been transferred to Children's Hospital San Diego. The antiquated transfusion process at UCSD was just too much to take. After a bit of research, we found that Children's actually performs pediatric transfusions on an outpatient basis. Although we really liked the physician at UCSD, the decision to move hospitals was unanimous by all involved, including the insurance company! If you are wondering why no one inquired about the possibility of admission for transfusions prior to our return, well, as Dr. Davies put it, no one thought it was even an option in this day and age!

Tomorrow, Susannah resumes her physical, occupational and speech therapies. These will now be conducted in our home due to her continued immunosuppressed status. She will see each discipline three times per week, as well as begin receiving weekly visits from the Early Childhood Education Program. With all this, added to her weekly clinic and audiology visits, she will surely be one busy little lady! The therapy appointments, however, will be a rather nice diversion since Susannah’s exposure to the outside world must still be very limited. She is permitted outside only when wearing her mask and must avoid stores, restaurants or groups of people.

Now that we are home and settled, for the time being anyway, I hope to find the time to catch up on the many long overdue personal thank yous for the encouragement, prayers, cards, meals, care packages and all around support we have received from everyone over the past several months. In the meantime, please know that not a day goes by that we don't think of you and how grateful we are for your unending support.

All our love,

Amy, Klane, Madeline and Susannah

Wednesday, August 2nd, 2000 ~ Day +97

Susannah’s Returning Home!


Hey Everybody!

Just a relatively quick update on Susannah (and Amy). It appears that they a will be returning home this weekend, on Friday night!

As fortune would have it, Susannah has staved off any evidence of a fever. She was discharged back to the apartment on Sunday and has been well since. Of course, not one of these updates would be complete without a bizarre twist -- nothing is ever simple.

It turns out that blood cultures drawn at the time of Susannah's initial admission to the hospital last week grew out a strange little bacteria known as atypical Mycobacterium. No one is quite sure what to make of it. It's a bacteria that is really only an issue in patients who are severely immunocompromised, and otherwise it is usually what we refer to as a "colonizer", a bacteria that's there but doesn't result in any harm. The fact of the matter is that the Hematology staff in Minnesota is split in their approach to treating it. So on Monday Dr. Peters, after consulting the Infectious Disease specialist, decided that we should pull Susannah's central line, just in case the bacteria had colonized it. That way when she returns to Minnesota in September, infection shouldn't be an issue. Also to help insure this, she will now be on yet another antibiotic, which she will need to take for the next two months and probably throughout the second transplant. The line was pulled on Tuesday in the Peds Surgery clinic and as an added bonus the surgeon was able to do so without subjecting her to general anesthesia. He did, however, subject her to his rendition of the Barney song, "Barney is a dinosaur from our imagination...". As usual, Susannah was a trooper.

This unexpected freedom from the central line will make Susannah's care at home much easier for Amy, as well as alleviate the risks for any future line infections. Since she is still platelet transfusion dependent, she will unfortunately require weekly sticks to check blood levels and a new peripheral IV each time she needs to be transfused. This is why everyone had hoped that she would have recovered her platelet counts on her own by now.

We also received news this week that a new donor has been identified for Susannah's second transplant. Again we are blessed with an optimal six of six HLA tissue type match, and this time the donor is a 45 year old male. Apparently male donors are preferred because of their inability to get pregnant. There appears to be a relationship between multiple pregnancies of the donor and a higher incidence of graft versus host disease in the recipient. And besides, we all know that the Y chromosome is far superior to an extra X chromosome!

As for our apartment, the hospital has agreed to continue renting it to us during our brief hiatus. This will allow Amy to pack only the essentials, so she and Susannah will be able to manage flying home alone. When we return in September, our phone numbers and address will remain the same -- it will really be nice for the girls to have a familiar place to go back to.

With that, we anxiously await their return home.

Until next time,

Klane, Amy, Madeline and Susannah

Thursday, July 27th, 2000 ~ Day +91

Back in the Hospital ...


Well, Well, Well,

Just when we thought that the specter of good luck was looking our direction, reality decided to rear its ugly head. Susannah developed a fever for the first time after two months of outpatient care, just about the time we were making arrangements to bring her home. She did this early last Thursday morning, and continued until Saturday afternoon. So she was admitted to the hospital on Thursday for evaluation and observation, just in case the fever was something evil. The good news is that she was discharged on Monday. The bad news is that she was readmitted early (like middle of the night early) Wednesday morning for a recurrent fever. So far she doesn't seem particularly ill, but is quite the fuss-bucket. Cultures, x-rays and the like have all been negative, but she is still being treated with broad spectrum IV antibiotics, just in case bacteria are lurking. Both Madeline and Amy have had some cold/flu symptoms, so Dr. Kersey, the current ward attending, is fairly confident that Susannah's infection is viral and should resolve in due time. As is standard of care, however, she must remain hospitalized until she is afebrile for 48 hours, with 24 hours off the antibiotics to ensure that she is not harboring a line infection or something of the like. With any luck, Susannah and Amy should get back to the apartment over the weekend.

As for Amy she's holding up pretty well, but being back in the hospital is never fun, with the IV's beeping, the nurses checking and the new interns doing, well, what interns do. Yes, interns ... the BMT unit was completely full so she was put on the general peds floor which is a story in and of itself, but rest assured that Amy is keeping them on their toes (as if you had any doubt)! Thankfully, our friend Beth Kramer and her able bodied ten year old niece, Lauren, were still in town and could stay with Madeline when Susannah was admitted last week. They were planning to leave early Thursday morning, but hung around until mid-day, when Amy was able to recruit someone from her list of college babysitters for emergency watch. Grammy flew in on Thursday night for an already planned visit, and took care of Madeline for the remainder of the week. Fortunately, Madeline was in San Diego, when Susannah's second admission rolled around, so there was no scrambling for a sitter in the middle of the night. Although, from what I understand, it wasn't pretty gathering Susannah, enough paraphernalia for three days and arranging for a shuttle at 3:00am, all in the midst of a tornado watch! Ahhh, the Midwest.

As I alluded to above, on Sunday, Grammy brought Madeline back to California, and she is currently in the care Poppy and myself, with a load of help from our good friends Dana Jacobs and Sharon Spencer. So far, Madeline has been to the beach twice, to music class with me, visited friends and frequented "new park" (in her absence, a park opened up 1/8 mile from our house). She even got to spend some time with her big boy cousin, Anthony, who was in from Washington, DC. Sharon came over yesterday with her girls Rylee and Chloe, and the kids had a great time, and she left dinner to boot! Major score indeed! Madeline, with Poppy in tow, went to play at Casey "Sugar" Jacob's house today and will go to Legoland tomorrow with the Jacobs and Spencer clans. Her agenda for next week includes swimming lessons, playgroup, music class and a visit with her friend "New Dana" Hemingway and her mom Stacy. She is one busy little lady!

The highlight of Madeline's return is her new big girl bed. In true Amy fashion, this bed is about four feet high, fluffy and pink! Unmistakably intended for the likes of a princess. So far Madeline loves it and has only fallen out once, a fall from which she didn't even wake. To say she is adapting well to returning to "regular house" would be an understatement. Hopefully, Amy and Susannah can come home soon as well, so we can all be together.

As for Susannah's line issue, it now appears that Dr. Peters is backing down on his insistence that the line come out prior to returning to California. I think that he is seeing the need to get my ladies home for a stint. We are currently making arrangements for follow up with UCSD's Pediatric Hematology group. Things look promising so far. I spoke with Dr. Peters yesterday, and even in light of the recent hospital admissions, he remains optimistic that Amy and Susannah may still come home late next week (though Amy is not holding her breath). The catch is that Susannah will indeed come home with her central line. Prior to the fevers, transfusion independence was clearly on the horizon, but with the infection and all of the subsequent blood draws, her platelet count and hemoglobin really took a hit.

We will try to be better about sending more frequent updates. Let's all hope for a resolution of Susannah's fevers and a prompt return home.

Love,

Klane, Amy, Madeline and Susannah

Thursday, July 13th, 2000 ~ Day +77

More News From the Gopher State


Hi Everybody!

Klane here in San Diego. Amy is stuck in the apartment these days and unable to get to the computer room to compose a note, so I guess that makes it my turn to write. She has been able to hauncho a couple of babysitters recently, so perhaps her exile won't be so long lived.

So far I've been able to get back to Minnesota twice since coming home. I did make it out for the 4th of July weekend, thanks to the guys here covering clinic for me on Monday afternoon. Although I'm now at the VA where things in general are slower, the clinics have been extremely busy and any absence is duly noted, as it leaves a bit of a burden on the guys being left behind. So, to them I owe a great deal.

As for the three girlies, I had a great visit with them. When I first got to the car at the airport, however, I'm not sure that Susannah recognized me, and to be honest I wasn't sure that I recognized her. I was trying to figure out who put the little werewolf doll in Susannah's car seat. But then as soon as we each figured out who the other was, I saw that adorable grin that she shares with her big sister and all was cool.

Susannah has grown a tremendous amount of jet black hair all over her body. She has big bushy eyebrows, a moustache, sideburns and a back that would make many of my Persian and Italian friends envious. At least now she is losing some of her steroid fat and her face is slimming down. Poor little thing, so cute yet so funny looking! I was just glad to hold her. Anyway, she stopped the cyclosporine on the 4th, so the hair growth should slow down (it will be another few months, though, before it goes away completely). Also, now that she is off of the CSA, we have begun tapering her blood pressure medications, so now she takes two rather than three. By the time that she gets home, she should be weaned from all three; this will make dosing her meds quite a bit easier.

Of course, Madeline was a joy to see as well. We spent the weekend doing puzzles, reading books, taking tricycle rides and watching her newest, most "favoritest" video, "Annie" (it is really cute to see her dancing and singing along with the movie -- she even changes clothes/costumes accordingly). We also took walks and went for "coffee" together. The usual good stuff, quality time. She has really been missing her buddies lately, so a little daddy time was a nice diversion for that as well.

As for Amy and I, we did manage to get out on Saturday night for Japanese food (thanks to Grammy's babysitting). There was a huge national AA convention, but luckily the restaurant still served us. Japanese food without the large Ichiban beer is like going to the beach without going in the water. Otherwise, it was a pretty quiet visit. We weren't able to get out on the 4th due to Susannah's requirement to avoid crowds, and then I flew out before the fireworks started. Nonetheless, it was still a nice visit, and just being together was enough. It's amazing how quiet it is around here at home without the girls.

As for their return to San Diego, this remains uncertain. Susannah is still recovering from the transplant, requiring red cell and platelet transfusions every seven to ten days. My goal is to have the girls back home by the end of July. The end was actually looking nearer, as Susannah, for the second week in a row, managed to go seven days between platelet transfusions ... then boom, she needed red cells and growth factor, as her ANC dipped to 900. It usually takes four to eight weeks for the body to begin producing these blood products on its own, with the platelets being the very last thing to kick in. As she is now ten weeks out, Susannah seems to be taking her own sweet time recovering. She is approaching Day +100 (August 4th), which under the usual circumstances is the magical number at which patients are allowed to return home. Dr. Peters, with whom we met last Monday (the 3rd), now feels this is a more realistic date to plan around for Susannah's homecoming (so much for that early release they were initially planning for the beginning of July). Of course, coming home is dependent on Susannah being transfusion independent. The limiting factor is Susannah's central line. Dr. Peters again expressed his desire to have it removed before she leaves. This is because if she's not using it, it only serves as a potential site of infection.

The idea of transfusion independence and line removal seemed like a pretty reasonable plan -- a month and a half ago. But now, with my impatience getting the best of me, I am working on an alternative plan, which will allow the girls to return at the end of the month either way. If Susannah still needs the central line for transfusions, I think that we can arrange for that here (in spite of her need for cross-matched platelets, growth factor, etc.). Although, the process may be more tedious here than in Minnesota. Then, perhaps later in August we could fly back for a four day weekend to have her line removed. Right about now, a bunch of you are probably asking yourselves why the central line has to be removed in Minnesota and not here. Well, particularly now that Susannah's disease has surely progressed, we worry a lot more about her anesthetic risks. The anesthesiologists at the University of Minnesota are much more familiar with the kids with Hurler Syndrome and their compromised airways. So anyway, Amy discussed this option with Dr. Davies yesterday, and today, when she met with Dr. Peters, he too indicated that this is indeed is a very plausible scenario. Especially since this week Susannah went a record eight days between platelet transfusions. Also, beginning next week the plan is to try testing her body by letting her counts fall a bit below the current parameters to see if she has the ability to recover on her own. They think that she might just surprise us. If she doesn't become transfusion independent in the next few weeks, both doctors realize the emotional need for everyone to return home to California for a little break before things begin again in September. As such they will help set-up the necessary arrangements.

As I mentioned at the beginning of the note, Amy is at present, and for the foreseeable future, stuck by herself in Minneapolis. This means that she is on her own to change Susannah's dressings, clean the apartment, feed the kids, do the laundry, take Susannah to two clinic appointments per week, go to therapy everyday, and God forbid sit down for five minutes and catch her breath. Keeping the apartment clean is the tricky one, because it has to be cleaned daily, and Susannah can't be there while the carpet is vacuumed or the furniture is dusted. Although quite overwhelmed, Amy has managed to get most of these things accomplished with the aid of several college students whom she is paying to watch the kids while she works. This has been going on for about two weeks now, and she is near the end of her rope.

I will be going out this weekend to give her a hand, and there is some talk that our friend Beth Kramer (another one of our DC friends, who now happens to be our Midwest neighbor in Omaha, Nebraska) may be able to go out next week. Also in the works is a plan for Amy's mother to fly in and bring Madeline home the week of July 23rd to stay with me (and some helpers, yet to be recruited). With any luck, Amy and Susannah will come home the following week and Madeline will have one less flight back to Minnesota.

As usual, thanks for all the continued support and prayers. We will update again as our plans become clearer.

Best wishes,

Klane, Amy, Madeline and Susannah

Friday, June 30th, 2000 ~ Day +64

Just Chuggin’ Along …


Hi Everyone,

After much confusion at the airport and nearly missing his flight, Klane did indeed arrive for his first return visit to Minneapolis on Friday, the 16th. It was so great to see him that he was immediately "pardoned" for forgetting his ticket at home and having to purchase one at the gate!

We met with Dr. Peters bright and early Saturday morning and now have a time frame for Susannah's second transplant. The current plan is to begin the process once again in mid-September, exactly five months from the time of her first transplant. Susannah's body needs time to recover from the first round of chemotherapy, before we subject her to radiation and further chemotherapy and five months is really the minimum amount of time necessary. Any sooner could be harmful, but waiting much longer would allow even further progression of her disease. So, September it is ... and the 100 days to follow. Apparently we are destined to experience a Minnesota winter after all.

Clinically, Susannah is doing well and remains free of infection. Unfortunately, she is still requiring platelet transfusions every three to five days and red cell transfusions about every ten days. Also, her counts have taken a dip, with her ANC to falling below 1000 on several occasions, so she has been receiving G-CSF (or growth factor, a white blood cell stimulant) about every five days. Realistically, these things push our departure for California back a bit. We probably will have to remain here until at least July 15th, but it really just depends on how soon she can start producing these things on her own. Dr. Peters would really like her to be transfusion independent prior to our leaving. This would allow for her central line to be removed, which not only would make care at home much simpler, but would also eliminate a potential source of infection.

At her clinic visit on Monday, Susannah was seen by Dr. Wagner, the engraftment expert, who was one of our attendings on the unit. He and I discussed the failed engraftment and the possible explanations, as well the fact that each of our physicians has a different theory (infection, Hurler Syndrome or just plain old bad luck) as to why it occurred. Bottom line ... there is no clear cut answer, other than it was a fluke. He assured me that they would do everything humanly possible to insure that it does not happen again and explained the precise steps he would take to do so. This is truly his area of expertise and he is a very kind man, thus his reassurances were quite comforting.

Later in the week we had a bit of a scare when Susannah's fontanelle (soft spot) became protruded. Upon consulting Dr. Peters, he explained that this was fine, as long as the area remained soft. He also said it is fortunate that her fontanelle is still open, so if her spinal pressures do increase, there is some release available. He said it would only become concerning if she began vomiting frequently and was constantly irritable ... neither of which was occurring. Of course, just for good measure, two days later Susannah had to vomit twice in one morning. Fortunately, these were isolated incidents and nothing more came of them. At clinic the following Tuesday, Dr. Davies reevaluated the fontanelle and likewise felt that, although the it was indeed protruding, with the absence of any other symptoms, there was no reason for concern. By the way, Susannah is now being followed jointly by both Dr. Peters and Dr. Davies. Officially, as he is the Hurler expert, Dr. Peters remains our primary physician, but Dr. Davies has, for lack of a better term, adopted us. We were very happy with this development, as she is both a wonderful doctor and person who is very up front and easy to talk to. And, she loves babies!

Susannah is still very fuzzy ... our own little teddy bear. As Klane says, she is as cute as a bug in a rug (only she is actually wearing the rug!). Aside from all of the hair growth in other areas, the chemo finally caught up to Susannah's beautiful eyelashes and they have fallen out. For those of you who don't know, the child had eyelashes that any female would die for -- they were literally about a half inch long! The CSA however is working its magic and her old lashes are quickly being replaced with very thick new ones. Although the new, at one centimeter, just don't compete -- especially, if you compare them to the three remaining original lashes on her eye lids.

Also, at long last Susannah has finally stopped eating. She went from steroid gorging three egg breakfasts to consuming nothing but milk overnight. She has much less appetite now than she ever did in the hospital, even during the chemotherapy, which is somewhat disturbing. This began shortly after Klane left from his weekend visit, but thankfully Grammy the "master feeder" arrived soon after and had the patience to literally shove a little in each day. The doctors are not overly concerned, as her labs remain on track and her weight steady. Klane thinks she is just using up the steroid storage and her normal appetite will return shortly. Speaking of steroids, she has finally lost most of the puffiness that accompanied them. Her cute little face is indeed little once again.

As far as our endless stream of visitors go, both Grandma Dona and Klane left for California on Sunday, Father's Day. That same night Heather Kinkead arrived and we had a great visit. Not only did she out do herself cooking (she and Madeline even prepared kid recipes -- I admire her patience), but she also stocked my freezer! In all seriousness though, food aside, it was great to have a friend around. We did what friends do best and stayed up until the wee hours of the morning having good ole fashioned "girl talk". She left Wednesday afternoon and Grammy arrived Wednesday night. Since we will now be here for a while, she spent some time with her needle and thread adding a few personal touches to the apartment. Also, aside from her usual role as "ironing queen", Grammy was very helpful with bath time and dressing changes which was a great relief. By far the highlight of Grammy's visit, for Madeline, was "the parade". One morning, the two rode the bus downtown (a favorite pastime in itself of Madeline's) and happened upon a big parade impeding traffic. Unbeknownst to Madeline, she attended the Minneapolis Gay Pride Parade! She left the event armed with at least one of every giveaway that was offered, and had a great time.

Klane is now at the VA (Veteran's Hospital) and has a bit more flexibility with his call schedule (he is the one making it!), so he will be joining us for the 4th of July weekend. He arrives on Saturday, the 1st and will stay through Tuesday (and yes, his ticket has already been placed in his suitcase!). As far as the ladies in his life are concerned, he can't arrive soon enough. We really miss having him with us; the kids' faces just light up when he is around and can not get enough of their daddy.

As always, thank you to all of you for your continued support. I know we have said this before, but we can't tell you enough how much your love, prayers and kindness mean to us.

Have a wonderful weekend and Fourth of July!

Lots of love,

Amy, Klane, Madeline and Susannah

Thursday, June 15th, 2000 ~ Day +49

Recent Happenings


Hello All,

Yes, we girls are still in Minnesota. Klane returned home to California last Sunday, the 4th, and we have been adjusting to life without him around ... hence, the unusual silence.

Dr. Peters returned last Thursday and Susannah and I met with him on Friday -- actually it was I who had the pleasure as she snoozed through the entire appointment, physical exam and all! While we discussed a lot of details, still no concrete plans have been set for transplant number two, other than the fact that we plan to proceed. Details will come with time, but we will probably have a better idea once Klane and I can meet with him together, after Dr. Peters has had time to process the news more. As for Susannah's current health, she is doing remarkably well. Were it not for her bald head (and regular need for platelet transfusions and medications), one would be hard pressed to know she just had a transplant. In fact, the other day as I entered the elevator a man looked at Susannah, smiled and said, "Does she have allergies?" I, of course, said, "no" and then exited the elevator. It wasn't until we were outside the building that I realized he was referring to her mask. Not only was it not obvious that she'd just had a BMT, but he actually thought I was a neurotic mother who had my healthy baby wear a mask for the heck of it! It's funny, being on the unit and in clinic, you begin to take for granted that everyone knows what the mask is all about.

Speaking of clinic, one perk to Susannah's lack of engraftment is that we rarely have to go! Seriously though, she is still very closely monitored and we do have appointments two or three times a week to see the doctor and/or receive platelet transfusions, but for the most part, her daily blood work is done at the apartment by our home health care nurse, Jane. This is not how things usually work, but somewhere in the process of receiving news of Susannah's engraftment, I mentioned to Dr. Davies that, in light of the situation, I really had no desire to be in clinic. She was more than happy to make the arrangements to accommodate this request. Yes, I got the sympathy vote, but it actually works out well since I already had a nurse scheduled to come daily to flush her central line (I know ... I'm a wimp!). The blood draws and vitals make her visits even more legitimate.

The decrease in the number of clinic visits definitely makes the nine therapy appointments per week much more manageable. Susannah continues to progress and is really flourishing with the speech, occupational and physical therapies, so all the running around is indeed worthwhile. Specifically, Susannah is now crawling on all fours! She has done her own version of crawling for sometime now, but she has recently decided to show up her big sis and do so in the traditional manner (Madeline never actually crawled). Despite her new preference to being on her hands and knees, she continues to improve on her leg strength and standing and cruising ability. As far as occupational therapy, Susannah is now an ace at shape sorters and stacking toys, it is really very impressive to watch her give Madeline a run for her money! To keep her from getting frustrated at her own lack of vocalization (children with Hurler Syndrome are frequently late talkers), the speech therapist has begun to teach Susannah some signs, to give her a jump on communicating. She is now quite expressive and is constantly indicating her desire for "more" by signing it. She also quickly picked up the signs for "want", "all done" and "baby". Madeline has become quite the advocate for her little sister, constantly informing me that Susannah "wants more" of this or that!

Medically speaking, a few other noteworthy events have transpired since our last update. We began last week tapering Susannah's immunosuppressive/anti-rejection drug, cyclosporine (CSA). Completing the taper should take about four weeks and is the first step in our returning home to California (hopefully in early-mid July). CSA has many side effects, one of which is high blood pressure. Once the taper is complete, we will also be able to begin weaning her from her three hypertension medications. As of today, she has officially finished her steroid course, which should also help in reducing her blood pressure, not to mention the puffy look she is currently sporting! While on the subject of her looks ... I have to mention the hair! Another side effect of CSA is lots and lots of jet black hair (or better yet, fur) all over the body. Since Susannah began being tapered so early, we didn't really expect to see much hair growth. So, last week when I noticed some "bruising" on her eyebrows one evening, imagine my surprise when, by morning, those “bruises” actually became a giant five o'clock shadow all over her body! Yes, our little princess now has full body fur and the shadows of a mustache, goatee and raccoon eyes! Thankfully, this look will be short lived and she will return to her normally beautiful state in a few weeks.

Susannah was also fitted for hearing aids last Friday. She will continue to be followed and tested by an audiologist in San Diego while we are home to help determine the precise amount and type of loss she is experiencing. Both Klane and myself, as well as all three therapists, struggle to notice a lack of hearing on a day to day basis. The thought is that she is able to hear environmental sounds, which are complex sounds, but has difficulty with pure tones. It is the pure tones that have the greatest impact on speech, so the sooner she receives amplification, the sooner her speech should progress. Since her loss is indeed subtle, she will be monitored very closely to assure she is receiving only the necessary amount of amplification and no excess.

The search for a new donor is underway. Since Susannah was blessed with so many potential matches to begin with, the doctors feel we should try a different donor next time around. Who knows the precise reason the transplant failed, but we might as well eliminate as many potential reasons as possible. It is unlikely that the graft failure was due to the donor's marrow, but better safe than sorry. The time frame for clearing a new donor is compatible with the tentative plans to transplant again sometime in the early fall, so things should work out well. The goal is to proceed with the second transplant in an expedient, but safe amount of time. While re-tranplanting too soon can be dangerous, time is also of the essence in regard to the progression of the Hurler Syndrome. It is the combination of these two things, along with Susannah's own body recovering, that will be heavily weighed by the doctors in determining exactly when to proceed again. In the meantime, we will pray for a minimal amount of disease progression and do everything in our power to keep her healthy, as she will continue to be somewhat immunosuppressed in the months to come. In returning home, we will be able to exercise some discretion, but for the most part, Susannah will need to stay close to home, wear her mask and minimize her exposure to others. Although life will certainly not be returning to normal this summer, we will at least be back home with our family and friends close by.

Now, for the update on the social side of life here in Minneapolis. As I mentioned earlier, Klane returned home on Sunday, the 4th and Grandma Dona (his mother) arrived in his place. Before his leaving, the four of us were able to spend some quiet time together as a family. Madeline returned from her road trip that Friday and was elated to find her little sister back at "temporary house" with us. She and I had a little one-on-one time together as we planned a birthday celebration for Daddy. Not only were we able to find the gifts we were looking for, but also Klane's favorite, an ice cream cake. Okay, just a tip ... a three year old cannot keep secrets! It was bad enough that she walked into the apartment boasting about the "secret cake", that I had so carefully concealed, but Klane also knew the contents of every package he was opening, down to the color! She was very excited and it was really cute, but I just thought I'd warn you of her secret keeping abilities! This past week, Madeline and Grandma Dona have been tearing up the town keeping themselves busy. They have ventured out to the Children's Museum, the Bell Museum and also hit many of the old favorites too! Poor Grandma Dona actually thought she'd be able to get some shopping done while at Mall of America -- she obviously did not realize that the only thing of importance in that massive structure, as far as Madeline is concerned, is Camp Snoopy!

We are looking forward to spending Father's Day weekend with Klane, who flies in tomorrow evening. It took much shuffling, swapping and promises of additional call nights, but he managed to get the weekend off to come for a visit. We girls are quite grateful, as this is the only weekend that would work in his schedule until July! Both Klane and Grandma Dona fly out on Sunday and that same evening my friend Heather Kinkead, from our days in DC, flies in. Quite the busy airport day! It will be great to see Heather and I am definitely looking forward to the delicious gourmet food I know she will spoil me with. Klane is already jealous, although he has no reason to be, as the wonderful folks from St. David's have graciously been stocking the refrigerator at home for him!

Well, now that I have rambled on forever, I suppose I should close. Klane and I will be meeting with Dr. Peters together on Saturday, so we should have more information to report following that appointment. Susannah will also be seen by both Dr. Wagner and Dr. Davies next week, so that will give us even more perspective. As the details continue to unfold, we will certainly keep you posted. If you notice an unusual silence, please don't worry, it is just more difficult for me to escape to the computer room these days without Klane to run interference for me with the girls. By the way, Klane's current schedule has brought me out of my retirement (aka boycott) in the update area, so you're stuck with me again for a while!

You all continue to be a tremendous source of strength for us and we are truly grateful for everything you have done to see our family though this time. It is your love, support and prayers that have given us the courage and where-with-all to continue to move forward. We cannot thank you enough!

All our love,

Amy, Klane, Madeline and Susannah

Wednesday, May 31st, 2000 ~ Day +34

Update on Susannah


Hello Everyone,

Today was not a good day. I'm not quite sure how to sugarcoat this, so I'll be blunt. We learned the results of Susannah's RFLP test for donor DNA levels today. As of now, it appears that Susannah's transplant has failed.

After a week and a half of waiting for a result, it turns out that Susannah's blood has 100% of her own DNA and no donor DNA. As you might imagine, this is obviously very shocking to Amy and I and quite upsetting. We were not the only ones to be surprised, but the staff here was as well. Susannah continues to defy the odds! First, she was born with an extremely rare hereditary disease that neither of us were aware that we carried. Now, Susannah is only the fourth bone marrow transplant patient, of over a thousand, to outright reject her graft from the beginning. At least she was one of the few who would actually eat throughout the entire transplant process! As I mentioned in my last note, this is not how things usually progress. Patients with Hurler Syndrome who lose their engraftment generally do engraft and then lose their graft over the next few months. Susannah is such a little fighter that even through vigorous chemotherapy, her own bone marrow was able to win out, never even giving her donor's a chance.

Because we only learned of this problem today, the exact plan is not yet clear. Additionally, Dr. Peters is out of town until June 8th, and we will need to further discuss with him the possible options. For now though, it appears that Susannah will require a second transplant, as we had quietly feared. Unfortunately, we have to wait between four and six months for Susannah to fully recover from the insult of the chemotherapy before subjecting her to a second round of chemo and possibly radiation therapy. These plans will become more clear with time.

As for the immediate future, Amy and Susannah (and of course, Madeline) will need to stay in Minnesota for about another month. This time is necessary to slowly and safely reduce the many medications that she is on right now. We also have to wait for Susannah's marrow to recover to a sufficiently healthy status such that she will not require regular transfusions. As for me, I still intend to return home on Sunday night. I feel terrible about leaving Amy at this point, but my staying will not help Susannah, and now I will need to save my leave for later. My mother is flying out for a two week stay on Sunday, at about the time I leave, so this will relieve some of the burden from Amy.

Despite this disappointing news, Susannah seems to be doing very well. We have gotten into a routine at the apartment now. Susannah is presently taking 13 medications by mouth which are administered at 8:00am, 12:00pm, 4:00pm, 8:00pm and 11:00pm. She is tolerating this superbly and is getting back to a normal sleeping schedule at night. Our clinic visits have indeed been long, but we have been given the luxury of skipping a day here and there.

On the lighter side, our first night back from the hospital was quite a fiasco. First, is the tub story. Our apartment is handicap accessible, meaning that there is no bathtub, only a wheelchair accessible shower. Seeing as to how we need to bathe Susannah daily, we needed a bathtub. Amy, being Amy, realized this before Susannah was even admitted to the hospital. So we went to Target and Home Depot in search of a plastic basin to use for a bathtub, but could not find anything that worked. So, on the day of admission, Amy asked if we could get a tub from the homecare company or from the hospital after discharge. As usual (and I'm as guilty of this as anyone!), everyone blew her off, and said that they would worry about it later. Well later, a couple of days before being discharged, no one could manage to solve our problem of no tub for Susannah. So, we hijacked our hospital tub while our primary nurse, Mary (bless her heart), turned a blind eye. We did this in the middle of the night before leaving the hospital. Somehow, the morning clerk found out and hit the roof! How, we have no idea, because we were pretty darn slick about the whole thing. Well, that day there was a big to do about how we needed to return this tub, but seeing as to how possession is nine-tenths of the law, the tub is still in our apartment (and Susannah is clean)!

Secondly, there were the medications. Susannah was discharged on 13 medications! Some were pills, some were solutions, some were refrigerated, some we had to mix ourselves and, as I mentioned before, they are given at many different times throughout the day. Without getting into the details, this was very confusing for the first night back at the apartment. Amy, however, is now a little alchemist sitting there with her syringes and pill crushers and bottles of potions, coming up with two skillfully mixed syringes of orange and pink. Somehow she has condensed what appears to be liquor store full of medications into about 16 cc's of medicine, which Susannah dutifully takes upon command.

Well anyway, I wish I could have been the deliverer of better news. While we are crushed by the revelation of Susannah's failed transplant and our impending return to Minnesota in the fall, we are trying to remain upbeat. Although completely heart-wrenching every moment that I think about it, I only have to take one look at Susannah and see her smile and giggle, in the goofy way that she does, to make me feel better. There are still many unknowns about how we are going to deal with this new challenge, both practically and emotionally. Please keep Susannah and the girls in your thoughts and prayers, for that is what has made this trip tolerable up to now. We will keep in touch and God bless all off you for being with us.

With all of our love,

Klane, Amy, Madeline and Susannah

Friday, May 26th, 2000 ~ Day +29

Today is the day!


Hello All,

Just a note to let everyone know that Susannah will be discharged from the hospital later this afternoon. Her counts continue to rise, with a WBC of 1700 and an ANC of 1200. We are just waiting to receive the final clearance, then we'll be free to go. Just in time for the holiday weekend!

I have been busy today packing up our little home here on 4A (its amazing just how much one baby can accumulate in a month!), while Klane is back at "temporary house" disinfecting and cleaning in preparation for Susannah's return. Susannah has been entertained all morning, playing on her play mat with Jane, Teresa and Maureen (her physical, occupational and speech therapists), as well as, the many nurses and aides who have fallen in love with her during our stay here.

Yesterday was a very big day for Susannah. She was actually given permission to leave her room for the first time in 38 days! She donned a tiny mask and new hat and cruised the halls of 4A in a little push car. One of the aides even gave her a little tape player to hold onto so she had tunes for the ride, which, of course, she just loved. After an hour or so inside, we were onto bigger and better things -- the outside world! Klane and I took turns pushing Susannah's car along the river road enjoying, what you could call, a "Chamber of Commerce day" in Minneapolis. She had a ball and savored every moment, never once even touching her mask!

We are still awaiting the result of Susannah's RFLP to determine her engraftment percentage. Apparently, there was a great deal of confusion in the lab and the test was somehow cancelled twice (its a very long story). So, on Wednesday we had to have the test redrawn and now we will not know the results until Tuesday, at the earliest. Remember ... think and pray 100%. We did, however, receive news today from the lab that Susannah is inactive for CMV, a respiratory virus to which she and most of the population have been exposed. Earlier in the week, a blood test (which, incidentally, was also cancelled twice!) revealed very early signs that this virus may be in the process of activating in her body. We were very relieved to hear today's results, for had she tested positive, Susannah would have required several weeks of gancycolvir, a heavy duty IV antiviral medication known to suppress grafts. Hopefully, her growing white count and prophylactic antiviral medication (acyclovir) will continue to keep this virus in check and the IVs away!

Madeline left on her road trip yesterday morning. She was elated to see Tessie and is looking forward to a fun-filled week! We sure are going to miss her, but it will give us a chance to establish a routine before Klane goes back to work next week. Once he leaves for California, there will be a lot to juggle between appointments, medications, catheter care, as well as entertaining Madeline. It will be nice to be well adjusted when she returns -- we want to make things as easy as possible on both girls. Madeline can hardly wait for her little sister to be back "home" with us!

Once she is discharged, Susannah (and I) will begin our daily visits to the BMT clinic, tomorrow! Yes, on Saturday, in fact the clinic is open seven days a week and 365 days a year. This will be her opportunity to receive any blood transfusions or IV fluids or medicines without having to be in the hospital. Although, better than being in the hospital, clinic visits can often take on the upward of six hours per day. Susannah will also continue all three of her therapies as an outpatient. Nothing like a busy schedule for Mommy!

Before I close, I would like to take a moment to let everyone know how very much we appreciate all of the cards, e-mails, packages, and all-around well wishes we have received over the past weeks. Although we have not yet had the opportunity to respond to each of you individually, please know that every piece of correspondence we get helps brighten our day and strengthen our hope.

All our love,

Amy, Klane, Madeline and Susannah

Monday, May 22nd, 2000 ~ Day +25

The Latest on Susannah


Hi Everyone!

Once again, sorry for the lapse in updates. Issues around here have been developing rather slowly these days and I was waiting for something big to happen. Finally, it did!

The good news -- Susannah's WBC count is 900 today, her ANC is 400. What does this mean? Well, in order to be discharged she needs an ANC of greater than 500 for three consecutive days. So, by these criteria alone, Susannah will likely be discharged by the end of the week.

Since I've been bad about writing, I must now backtrack and explain, as there has been a bit of drama around here surrounding Susannah's white count. As I wrote last time, we expected her WBC count to pick up a few days after first appearing on Day +13, but this didn't go quite as planned. Up until Friday, Susannah's counts were bouncing up and down between 150 and 400. As you might imagine, the anticipation and anxiety were killing us. On top of the waiting, Dr. Peters (the Hurler expert) came by on Thursday and told us that one possibility as to why her counts weren't increasing was because the transplant didn't take. This, of course, sent Amy over the edge.

Thankfully, the current ward physician, Dr. Wagner (the expert on engraftment), was less pessimistic. He told us that it's not unusual for cell counts to take 21-30 days to increase, although Hurler patients often do engraft much sooner. The combination of Susannah's recent bacterial infection and her course of imipenem (a potential graft suppressant) probably didn't help the situation. However, on two separate occasions Dr. Wagner went down to the lab to personally inspect the blood smears himself. Both times he thought that the cells looked good, with a majority of neutrophils present. Apparently, when the graft begins to take it produces a majority of neutrophils. If the host cells are proliferating instead, then there tends to be a majority of lymphocytes. So, these results were promising.

To truly know the origin of the cells now in Susannah's body (graft or host), we need to do a DNA test called RFLP. The catch here is that in order to do the RFLP (routinely done on Day +21), there needs to be a minimum count of around 700 cells, which Susannah had not had until today (Day +25). The only alternative to peripheral blood RFLP is to take cells from the actual marrow, by bone marrow biopsy. This is a fairly painful procedure and as such, requires sedation. The plan was, if Susannah's counts were not high enough by today, that we would do a biopsy. Luckily, good ole Susannah came through just under the wire!

The RFLP was drawn today and the results should be back by Friday. This is the big test and we're very anxious! Obviously, we hope Susannah will be 100% engrafted with her donor's marrow along with its necessary enzyme. Initially, most children with Hurler Syndrome do engraft at 90-100%. Unfortunately, it is not uncommon for some of these kids (about 40%) to show a decline in engraftment over time. This is usually a gradual process and often takes several months. Some patients eventually lose their graft entirely, requiring a second transplant. Others stabilize at a level of partial engraftment, which still enables their bodies to produce enough enzyme to fight the disease. We are becoming increasingly aware of this potential, as we meet more families of children who have undergone BMT for Hurler Syndrome. It is tough enough to go through this process once, but to do it again, is at this point too difficult to imagine. Susannah, however, is a fighter and we have faith that she will be one of the lucky ones!

Otherwise, Susannah continues to have a smooth course. It has been two weeks since she has had a fever. She also is only requiring a minimal amount of transfusions -- about one unit of red cells, and two units of cross-matched platelets per week. Her blood pressure is still rather difficult to control and remains a daily topic of discussion on morning rounds. Finally, in preparation for discharge, we are trying to transition Susannah to all oral medications, which so far, she is tolerating pretty well. This can be troublesome for some kids, as there are many medications given at many different intervals throughout the day.

Madeline has been in the care of my sister, Brenda, and my niece, Holly, for the last week. They've had a great time enjoying the spring weather here in Minnesota, jaunting around Minneapolis. One of the highlights of their visit was that Brenda made her world famous enchiladas, twice! This helped to satisfy our homesick craving for good Mexican food ... not much of that in Minnesota, and certainly there isn't a Roberto's down the street, like at home.

This week on the list of Madeline's visiting dignitaries is Carey, Jim, James and most importantly, Tess Cimino. They arrive today and will be in the area for about two weeks. On Friday they will take Madeline to Wisconsin for cheese and to visit Jim's sister. At some point they will continue on to Rochester to visit Tessie's Uncle "Matty" and then return about ten days after their departure from Minneapolis. It's the Cimino whirlwind tour of the Midwest, which I'm positive Madeline will absolutely enjoy.

On that fun note I will quit. Since we expect several significant events to occur this week, there should be more frequent updates over the next few days. Please keep thinking and praying (and hoping and wishing) for continuously increasing donor cells!

Love,

Klane, Amy, Madeline and Susannah

Monday, May 15th, 2000 ~ Day +18

Smooth Sailing


A Big Hello from Minnesota!

Well, we've made it to Day +18 and everything, so far, seems to be coming along quite smoothly.

Here's the scoop medically:

1) WBC counts are hanging in there at about 200-400. We expect them to take off any day now.

2) No fevers since last Monday! All subsequent cultures have been negative. So now we just continue the course of IV antibiotics until Sunday, and hope that Susannah does not spike any more fevers.

3) Only one red cell transfusion since last week and two platelet transfusions. She actually only required one, but apparently at midnight on Saturday, the blood bank decided they had a surplus of her cross-matched platelets and somehow talked the charge nurse (who was a “float pool” nurse) into giving them to Susannah versus "wasting" them. Needless to say, the doctors were less than pleased the next morning since the transfusion was unnecessary, as were we.

4) Susannah's blood pressure still remains an issue. It is the staff's desire to have her BP under control prior to discharge. At present, her first two medications are maxed-out in terms of dosage, so it appears likely that we will add a third anti-hypertensive. Hopefully we can have this issue resolved by the end of the week, so as to not hold up Susannah's discharge.

Susannah remains very playful and one of the most popular patients on the unit (but she still has no tolerance for stethoscopes and the like). It does seem that her stamina is now reduced (imagine that!), as she poops-out pretty quickly with physical therapy and other such play-mat activities. She is still eating very well (apparently one of the few patients ever to eat throughout the entire transplant process) and on a regular nap schedule -- two things everyone told us would be impossible. We are very proud of her.

On the social side of life, Grammy left yesterday for California. We were sad to see her go, despite her well known idiosyncrasies. She and Madeline have been playing "make-up" in the morning, running errands, going to music class and doing other fun activities. Next in line is Auntie Brenda (my sister) and cousin Holly. They arrive tomorrow and will surely provide loads of entertainment for Madeline.

Speaking of Madeline, she has her first official crush. She is in "love" with Rich, one of the male nursing aides who has glowing red hair! She follows him everywhere and waives when he passes and says "Hi Rich" in a sweet little voice over and over again. Her new game is "restaurant". We set-up a miniature table and chairs in the hall outside Susannah's room and the secretary made her a sign, "Chez 4A" and gave her a tablet and pen. She spends hours taking people's orders and making pretend food. Dr. Davies (the attending covering this weekend) says she is only missing evening margaritas at her cafe, otherwise she'd be set. Kayla is the most frequent patron, although virtually everybody (doctors, nurses and even Ronald McDonald) has "eaten" there. Everyone who passes by gets quite a chuckle.

Well, I'll sign off for now and try to improve on the update frequency front (Amy is currently on strike in the writing area). As you might imagine, things have become much busier around here since the return of Madeline, thus cutting into our computer time.

With our best wishes and love,

Klane, Amy, Madeline and Susannah

Thursday, May 11th, 2000 ~ Day +14

The counts are in!


Hi All!

We have much good news to share today. First and foremost, SUSANNAH HAS COUNTS! Primordial signs of a returning white blood cell count in Susannah's body first appeared yesterday morning. Now, you know that I can't relay any information without a quick, little medical lesson. I like to try to make sure that everyone is on the same page, so you will either find this helpful or very boring or both.

Since the topic of counts will continue to be big issue in the coming months, here's how it works. First, a normal white blood cell count is somewhere between 3,000 and 10,000. Susannah's is currently 50-150, very low, but better than zero. Second, a certain percentage of these white blood cells are called neutrophils (also known by a variety of other names such as granulocytes, polys, PMN's, segs). Neutrophils are very important in the attack, and particularly, the destruction of bacteria. The reason I tell you this is that the number of neutrophils that Susannah has will be the primary determinant of when she can leave the hospital. The number of neutrophils in the blood is measured as the absolute neutrophil count (or ANC). As long as the ANC is less than 500, Susannah is considered severely immunocompromised and cannot leave her room. When the ANC reaches 500 for at least three consecutive days, then she is eligible for discharge, assuming all other factors are favorable. Counts tend to come in very slowly at first, hovering around 100 for a few days, then suddenly shooting up into the thousands. From what we have been told, it is possible for Susannah to reach 500 by the end of the weekend.

About the "other factors" I mentioned above ... for Susannah this would mean no more fevers, as well as, control of her blood pressure. Regarding her fevers, she has now gone more than 36 hours without one. Prior to that, she'd had a febrile episode at least once, each day between Monday and Wednesday (she spiked immediately following our last update). Since the antibiotics Susannah is on should have been fighting her infection and halting the fevers, the doctors were pretty close to ordering a CT scan of her chest (to look for a hidden infection), and adding a stronger antifungal medication (amphotericin B -- better known as "ampho-terrible"), but thankfully Susannah pulled through without a fever last night. Even though she is now afebrile, Susannah will still have to complete the full 14 day course of antibiotics because of that positive blood culture (you know, the ugly Enterobacter), which will put her earliest possible date of discharge at May 22nd.

As for her blood pressure, Susannah is now on two regularly scheduled anti-hypertensives, amilodipine and prazosin, as well as her prn dose of nifedipine (I told you she might give Poppy a run for his money). As of now, she is still requiring intermittent doses of nifedipine, so the doctors continue to increase the doses of the other two medications in an attempt to regulate her pressure.

Otherwise, Susannah continues to be pretty happy and playful and, in general, we think she feels well. She can be somewhat temperamental at times, thanks to the steroids! She now despises the thermometer probe and the stethoscope. Being in orthopaedics, I can certainly understand that! I'm still not sure what those silly things do anyway! She had a great day today with all three of her therapists and continues to demonstrate advancement in development. She was very interactive with the speech therapist and tried to mimic her sounds. My approach to this was to try to make her oink like pig (alright, no “Deliverance” jokes out there) and it worked! I think that she is really catching on. During her physical therapy session, Susannah cruised, for about three steps, on her own initiative.

As for Amy and I, we actually went out to dinner the other night. Grammy has been staying with Susannah in the evenings so that we are able spend a little quality time with Madeline. We took advantage of this situation on Tuesday, when we were invited to dinner by some other families in the area (of course, I gave Susannah her bath and did the catheter dressing change before leaving). We went to TGI Friday's with the Moores, the Moores and the Richardsons. No I didn't just stutter, there are actually two Moore families here, each with a little girl who has undergone a bone marrow transplant for Hurler Syndrome, Maddie and Jessica. The Richardson's we have mentioned before; their son Regan also recently had a BMT and their daughter Fayth is Madeline's age. We have all gotten to know each other fairly well since being here, and we had a nice time at dinner and Madeline loved playing with the other little girls! I must mention that little Maddie was admitted today to undergo her second transplant, so please keep her in your thoughts and prayers too, as we hope for a successful transplant this time around. I hate to be selfish about this, but since she has to be here anyway, we were extraordinarily excited that Maddie was assigned the room right next to ours. It will be nice to have friends with whom to share these long, monotonous days. The two mommies appear to be two peas in a pod, and will surely keep everybody in this place on their toes!

Again, I have ended up being much more long winded than I intended, so I will sign off for now. Of course, we will continue to keep everyone up to date on Susannah's progress as things unfold.

With much love,

Klane, Amy, Madeline and Susannah

PS … For those of you who may have noticed, I must exonerate myself. In my last message, phenergan and Benadryl were listed as "anti-medics". No, I don't believe that these are medications used to ward off enlisted military health corpsmen, but I was lazy enough to let Amy type in the list for me and was half asleep when she asked me to look at it. I may not be a flea, but I do know that these medications are "anti-emetics".

Monday, May 8th, 2000 ~ Day +11

Plugs, Bugs and Drugs


Hello Everybody!

Klane here again. Amy asked me to write the update today because things have gotten a little technical around here.

As you know, Susannah had her Hickman catheter (aka plugs) replaced this morning. The procedure itself was relatively uneventful. The hardest part was for Amy and Susannah last night. At midnight our nurse, Lisa, informed Amy that the baby needed, not one, but three antibiotic baths (otherwise known as Hibiclens scrubs) over the next six hours. As you might guess, Susannah was ecstatic about this, and she and Amy where up a good portion of the night partying away with Hibiclens! So today, both are pretty hung over and, at present, are taking naps (of course, this was after Susannah woofed down two jars of baby food, two crackers and some of Mommy's milk -- she had been NPO since 2:00am).

By the way, many of you have been asking about the sleeping arrangements around here. For the most part, Amy stays in Susannah's room on a futon bed and I stay at the apartment with Madeline. On occasion we trade off, for Madeline's sake, but the above arrangement works best because of Susannah's feeding schedule. Also, for some reason I can't understand, Amy is able to sleep very soundly in the hospital, whereas my body has been conditioned over the years to remain light, in anticipation of those dreaded pages.

On the more concerning side, we found out today that Susannah's cultures from her Saturday night fever (to keep the party theme going) are growing bacteria, which have been identified as Enterobacter cloacae. These "bugs", happen to be resistant to the two antibiotics which she is already on to cover this type of infection -- ceftazadime and tobramycin. So, now we're changing these two drugs to cefotaxime and imipenem, not exactly my choice for a party cocktail. These are definitely big gun antibiotics. In fact, imipenem requires a special sign off from the infectious disease specialists before it can be prescribed so as to reserve it for only the most resistant strains of bacteria. In addition to these two drugs, she will continue on vancomycin (also a pretty heavy hitter in and of itself). Hopefully, the three meds will do the trick. I do think that these antibiotics are pretty safe, just not your typical "take two pills and call me in the morning". I am, however, concerned about breeding more antibiotic resistance, which may be how we ended up in this position in the first place (and you all wonder why I'm so uptight about giving antibiotics!). Still, it is very reassuring to see that they don't take any chances around here; particularly considering that, as of this morning, Susannah has yet to show a white blood cell count.

For those of you keeping track, Susannah has officially been deemed a walking pharmacy. The following is her current med list; it's pretty impressive.

o vancomycin (ABX)- q6 or 4x daily
o cefotaxime (ABX)- q6 or 4x daily
o imipenem (ABX)- q6 or 4x daily
o G-CSF (WBC stimulant)- q24 or 1x daily
o Diflucan (antifungal)- q24 or 1x daily
o Lasix (diuretic)- q24 or 1x daily
o acyclovir (antiviral)- q8 or 3x daily
o cyclosporine (immunosupressant)- q8 or 3x daily
o Solu-Medrol (steroid/immunosupressant)- q12 or 2x daily
o amilodipine (blood pressure)- q24 or 1x daily
o nifedipine (blood pressure)- prn
o Tylenol (fever/transfusion pre-med)- prn
o Benedryl (transfusion pre-med/anti-medic)- prn
o phenergan (anti-medic)- prn

As I mentioned, still no sign of new white blood cells. So we wait expectantly. As for Susannah's platelet counts, these appear to be responding very well to the type specific transfusions. She did, however, have a fair amount of oozing from her catheter site today, so I had to hold pressure for a while until it stopped (unquestionably, this was due to her low platelet count). After a second transfusion this afternoon, her platelet count reached 86,000, well above the required 40,000. Perhaps she is now on her way to every other day transfusions rather than daily.

The good signs so far are that she has not spiked a fever in 36 hours and, for better or for worse, she has a new central line, which may have removed the place where the little buggers were hiding. So as usual, we remain optimistic. I do believe in a small, but healthy dose of denial. The plan for now is to draw blood cultures daily until no more bugs are found for three consecutive days, and hope that those white cells grow up quickly.

So, on with the new plugs, bugs and drugs, we trudge! I promise that next time Amy will write and spare you all of my corny witticisms. Of course, as always, your continued thoughts and prayers are very much appreciated.

With love,

Klane, Amy, Madeline and Susannah

Sunday, May 7th, 2000 ~ Day +10

Off to the OR ...


Hello Again,

Well, like Klane said, no news is good news. Unfortunately, I do have some news.

Just a quick update to let you know that Susannah will be going back to the operating room tomorrow to have her central line (Hickman) replaced. We are not exactly sure what happened, but the bottom line is that it somehow pulled out.

The story goes ... Klane and I went back to the apartment last night for a few hours to put Madeline to bed, while Grammy stayed with Susannah. We decided to let the nursing aide give Susannah her bath and change her dressing (Klane usually does this every evening, but figured since it is actually the aide's job, he would leave it for her this time so we could spend a little more time with Madeline). Upon our return, we noticed that the dressing was lifting off and thus needed to be re-done. So, Klane and the nurse removed the bandages and began to change it. Everything was fine one minute and then the next, the two of them simultaneously said, "Does that look like it is loose?!?" No one tugged, no one pulled, it just mysteriously came out. A chest film revealed that the line was indeed displaced, although still usable for the time being (it was now in the jugular vein, rather than the heart). Needless to say, we will go back to having Klane be the sole bather and central line caregiver. In case you're wondering, I'm a big chicken and tend to stay far away from anything involving the Hickman. After all, it took several days for me to even become comfortable holding Susannah with the line protruding from her chest!

Just to add a bit more excitement to the evening, Susannah also decided it was time to spike a temperature (101.2). Since she surpassed the magical 100.5 mark, we began the whole fever work-up. Blood cultures were drawn to check for an infection and she was started on more antibiotics. She was already on two prophylactic antibiotics, ceftazadime and Ancef (or "Orthocillin", as Klane refers to it). Anyway, she will continue with the ceftazadime, but vancomycin ("the big gun") will replace Ancef for three days, while we await the results of her cultures. Of course a fever at anytime during the transplant process is alarming, but apparently it is also quite common for patients to spike fevers, just as the white cell counts are coming in. Hopefully, this is indeed the only cause for Susannah's fever and we can expect to see some counts very soon!

On a more positive note, Madeline conned Grammy into her very first pair of roller skates last night. They are quite the sight -- white with pink flowers and hot pink wheels! Of course, Madeline thinks they are just beautiful. A number of the students around campus use skates as their mode of transportation and Madeline has been eyeing them for weeks now. She was requesting them daily, but Klane and I kept thinking she was far too young to have the coordination and balance to skate. Apparently we were very wrong. Within minutes of putting them on, she was cruising the halls of the apartment building. The students already get a kick of her riding her tricycle around the foyer, but the skates have really won their hearts. It really is adorable.

We just received word that Susannah's surgery has been scheduled for 7:30am tomorrow. Although the operating room is obviously sterile, her immunocompromised state, as well as the added risk of anesthesia with Hurler Syndrome, makes leaving our little bubble on Unit 4A a very unsettling event. However, she is certainly not the first post transplant patient, nor will she be the last, to need her Hickman replaced. Susannah should be back in her room by mid-afternoon and we will be in touch then.

Thank you for your continued thoughts and prayers, today, tomorrow and always.

All our love,

Amy, Klane, Madeline and Susannah

Saturday, May 6th, 2000 ~ Day +9

Susannah the Bald Baby


Hi Everyone,

It's now my turn to write again (Klane). Not a great deal has transpired in regard to Susannah's health since our last update. However, in our current situation, the old adage "no news is good news" is definitely appropriate.

Madeline and Grammy returned this past weekend. Of course, we were delighted to have Madeline back with us -- it was a long, and very quiet, two weeks without her. She thoroughly enjoyed her "vacation" in California. A very big thank you to all of you who made this possible. We saw some photos of her birthday party and it certainly looked like everyone enjoyed themselves.

Also this week, Amy's long time (as in grade school long time) friend, Wendy Calareso arrived with Justin, her almost three year old son, and her baby Brianna. The three of them and Grammy have managed to keep Madeline very well entertained with picnics, hopscotch, running in the sprinklers and trips to the park. We have also discovered that daily walks to and from the hospital to visit Susannah make for nice long afternoon naps and early bedtimes for Madeline! By the way, winter is officially over and the weather in Minnesota has suddenly become nice and warm, hence all of the outdoor activities.

As you may have guessed from the title of this message, Susannah has finally lost her hair. It began to fall out Monday afternoon. At first we noticed a few strands around the crib and by evening her hair was starting to look pretty thin. On Tuesday morning it was coming out by the handful and there was literally hair everywhere! It was in her crib, on her clothes, in her food, in the CD player, in her mouth, in our mouths and in our food ... well, you get the idea. It is amazing that a one year old baby could have so much hair. So, after her nap on Tuesday, I took the liberty of taking a pair of scissors and some clippers and finishing the job for her. I must say, it turned out pretty well, so if orthopaedics doesn't work out, I may have a future in barbering. Better yet, it could be like the old days of the surgeon-barber -- fix your grade 3B open tibia fracture and give you a trim to boot!

Anyway, when Madeline came to visit later that day, she walked into the room, looked straight at her sister and said "Where's Susannah?". She didn't even recognize her! Once we convinced Madeline that the bald headed baby in the crib was indeed her little sister, she then exclaimed, "It's Susannah! Her hair blew out! She looks like my Kayla!". Kayla, for those of you who may not be aware, is Madeline's favorite baby doll, who does bear a remarkable resemblance to Susannah in her “follicly” challenged state. Madeline now insists on bringing Kayla to the hospital every time she comes to visit. As far as the term "blew out" goes, its origin is a mystery to both Amy and I. Madeline came up with it on her own when we first began preparing her for Susannah's impending hair loss (I guess kids just come up with these things to help process what they don't quite understand). Since Susannah was born with a FULL head of hair, none of us have ever had the opportunity to see her bare head! Although it took a bit of getting used to, she looks pretty cute. It is amazing just how much Susannah now looks like Madeline did as a baby.

Now, when it comes to Susannah, there is of course always something medical to talk about. The issues of platelets and blood pressure have been the major discussions of late. Platelets are the little guys in the blood responsible for aiding in blood clot formation. Since platelets are manufactured in the bone marrow, along with white cells and red cells, she no longer has the ability to produce them on her own. Because of this, Susannah has required multiple transfusions to replace her platelets. Platelet transfusions, as well as red cell transfusions, are very common in post transplant patients, as it generally takes a minimum of six weeks for platelet production to return to normal. Susannah, however, has been requiring more platelets than usual (up to six transfusions per day, as opposed to the usual one). Thus, she now must receive platelets that have been "typed and cross-matched", rather than the random donor platelets most patients receive. These special platelets appear to have helped the situation, and hopefully will continue to lessen the number of platelet transfusions that she will need. As for red blood cell transfusions, fortunately Susannah is on the usual schedule of requiring transfusions only every three or so days.

Regarding Susannah's blood pressure, this is somewhat of an expected problem as well. Her blood pressure has risen from her usual 70/35 to as high as 170/95. This increase is due to the cyclosporine and steroids (immunosupressant/anti-rejection drugs), both of which are known to cause an elevation in blood pressure. She now is on a pair of meds called nifedipine and amilodipine, which treat the high blood pressure (for you medical types, the calcium channel blockers apparently work best for cyclosporine induced hypertension). Interestingly, it is not uncommon for small children to require doses rivaling those of adults to treat this problem. I can see it now, Susannah and Poppy (Tom) traveling to Tijuana together to pick up their blood pressure medications! What a sight. In reality, as soon as Susannah stops the cyclosporine, she should no longer have any problems with high blood pressure. I did suggest minoxidil (you know, Rogaine) to treat the elevated pressure; this way Susannah could gain on the hair front too. Rogaine, for those of you who do not know, was originally developed to treat high blood pressure and it had the serendipitous side effect of causing unwanted hair growth. Needless to say, this did not go over very well and the "pine drugs", as Amy refers to them, ultimately won out.

Also of significance this week, we began checking for a white blood cell count on Day +7. This is the marker we are looking for to evaluate the success of the transplant. The white blood cells are the whole basis of this therapy. So far we have yet to see any sign of growth. This is quite normal, however, and we truly don't expect to see anything for at least another week (between Days +14 and +21). The return of her white blood cell count also heralds the end of her severely immunocompromised state (although she will continue to have problems fighting off infections for at least six months). Of course, we will be sure to keep everyone appraised of this important issue as it develops.

On the food front, Susannah's appetite continues to improve and she is requiring less of the song and dance routine. She has actually returned to opening her mouth willfully (sometimes). When she needs a bit of coaxing, however, all we have to do is turn on the VCR to "Baby Faces", a videotape which we came across in the hospital library. Instantly, she becomes mesmerized and opens her mouth like a machine. It's actually quite funny because this video is really just a low-budget collection of home video clips of babies, doing nothing in particular, put to classic children's songs. You know that Amy is dieing inside -- Madeline didn't watch any TV until she was about two, and now Susannah literally points at the television and squeals the instant she is placed in her high chair in demand of the video. Susannah has also become somewhat feisty with the medical staff whenever they attempt to examine her (those steroids really do make her feisty), so the video is now serving the dual purpose of taming her for exams and eating. The real question is, who is manipulating who?

Susannah continues to be seen daily by the physical, occupational and speech therapists. Remarkably, she is progressing regardless of all the trauma her little body is currently undergoing as an inpatient. She can now pull-up to stand by herself and is in the early stages of cruising. The therapists are all very impressed. Her verbal skills are also improving, although this remains an area of more delay for her. I must boast though that her favorite word is still "Dada", but is beginning to take second place to "Daddy" (much to Amy's dismay).

As usual, we must thank all of our family and friends here, back home and everywhere else (that's you!). I truly believe that the relatively easy course that Susannah has taken thus far is because Amy and I have been able to be here for her. This commitment, of course, would have not been possible without all of the support that we've received in the form of cards, baby sitting, house sitting, meals, work coverage, frequent flyer miles, thoughts, prayers, and the thousands of other ways that everyone has contributed. Susannah is indeed a very fortunate little girl to have so much love. You all are always in our thoughts and prayers.

With love,

Klane, Amy, Madeline, Kayla and Susannah

Thursday, April 27th, 2000 ~ Day 0

Transplant Day!


Dear Family and Friends,

Today is the big day -- April 27th, aka Transplant Day, aka Day 0. The event we have long been awaiting has finally arrived. As the actual transplant will not occur until very late this evening and I know many of you will be anxiously awaiting word, I thought I would get a jump start on this update so I am not up typing until the wee hours of the morning. For now, I will take a moment to fill you in, explain the process and then will eventually conclude with a report of the big event once it actually occurs.

With all the chemotherapy finished and Susannah fully immunosupressed, it is now time for the main event. As exciting, and at the same time scary, as this day really is, the bone marrow transplant is apparently quite uneventful. Contrary to popular belief, the transplant is not actually an operation, but rather more along the lines of a very special blood transfusion. I am sure you are wondering ... if it is so simple and Susannah is ready, then what is the hold up? Well, a lot must happen today, before she actually receives the marrow. Most importantly, that wonderful, generous person we all have come to know as Donor B, must make a trip to the operating room at her hometown hospital, undergo anesthesia and have her bone marrow extracted. Our thoughts and prayers will certainly be with this kind woman today as she graciously gives Susannah this special gift.

Once harvested, the marrow is packed in a cooler, handed off to a waiting University of Minnesota representative and transported by air to Minneapolis. After the plane touches down, it takes about seven hours for the marrow to be elutriated or processed. The goal of elutriation (T-cell depletion, for you medical types) is to remove a portion of the donor cells that have a high likelihood of reacting against Susannah's own cells. This process significantly reduces the incidence of future graft versus host disease (GVHD), where the new donor cells attack the host's (Susannah) cells. While most transplant recipients endure some degree of GVHD, obviously the less attacking going on the better. Thus, the importance elutriation. So, with all this said, sometime late tonight, this newly processed bone marrow, will finally be ready for Susannah.

As for the little gal, she remains in excellent spirits. Her days are spent playing, reading books, flirting with the staff and all in all keeping to her regular schedule. She managed to endure the entire chemotherapy regimen like a champ! All of those nasty drugs and only two "incidents"; we could not ask for more. Susannah continues to eat and drink, but has completely boycotted baby food, in lieu of more flavorful people food. She is craving salt! Poor Madeline never had a speck of junk food until the age of two (and now only in the presence Aunts, Grandmas and the like) and yesterday we found ourselves digging pretty deep into the junk food trenches (salt-n-vinegar potato chips -- yikes!) trying to find the item du jour that was compatible with Susannah's chemo affected taste buds. Anything for calories! I bet you all wish you had that problem. At any rate, regardless of the nutritional value (and the fact that it goes against all of my child-rearing principles), it is food and food wards off the TPN Police! Everyone here is very pleased with Susannah's progress.

The moment has come ...

Susannah's new marrow arrived in the Twin Cities at 7:00 this evening and after many hours in the laboratory, it was finally delivered to Unit 4A. At 1:20 am, Susannah received her bone marrow transplant. It was quick and simple, lasting only 20 minutes. Our nurse, Kristy, hung the bag of marrow (which, by the way was clear and pale yellow in color, rather than red as you might expect) on the IV pole and the three of us, as well as the star of the show (who was awake for the occasion), watched as this gift of life entered Susannah's body. We took lots of photos and another nurse came into video tape the event and present Susannah with a balloon, card and stuffed animal from all of her friends at the hospital. For those of you keeping track, although the transplant actually occurred after midnight on the 28th, the official date remains Thursday, April 27th and Friday is still considered Day +1.

And now we wait ... wait for the donor cells to begin growing in Susannah's body. This could take several weeks and is a very critical time due to the fact that her own immune cells have been destroyed by the chemotherapy. It seems she has already endured so much these past two weeks, yet it is really only the beginning. The upcoming week is usually the most difficult of the entire process, as the effects of the chemotherapy are still in full force, while at the same time the lack of an immune system leaves her very vulnerable and tired. Susannah, so far, has defied the norm with her bubbly and energetic demeanor, we hope and pray she retains her strength and continues to have a smooth course.

As always we want to thank everyone for the tremendous outpouring of love and support you all have shown Susannah and our family. Your thoughts and prayers truly do make our days brighter.

All our love,

Amy, Klane, Madeline and Susannah

Tuesday, April 25th, 2000 ~ Day -2

Day –2 and counting!


Hello Everyone!

Amy says it is now my turn to write an update. Hopefully I can manage to inform you and yet entertain you just as well as Amy has been doing.

Today is Day -2. Since our last note Susannah has finished (without incident) the Busulfan and has started two other chemotherapeutic drugs: ATG (antithymocyte globulin) and cytoxan, as well as about seven others to counteract their effects.

Susannah continues to do quite well, given the amount of toxic chemicals that have been floating around her little body. Everyday we hear a different comment, from those taking care of her, about how happy she is and what a joy she is to be around. I have to agree. If there is a bright side to this whole experience, it is that Amy and I have been able to spend more one-on-one time with Susannah than ever before (even though we miss Madeline like nothing else).

Anyway, since beginning the cytoxan, we have noticed a significant drop in Susannah's appetite. With this said, she still probably eats as much, if not more, than most kids her age and continues to nurse as well. However, we have had to become more creative in order to find ways to coax her into eating. My first trick was to turn on the Laker game ... it turns out that Susannah is a huge Lakers fan. She was so interested in the game that she would open her mouth without realizing it, and I could quickly shovel a spoonful of food in before she knew it.

Even funnier, was what we had to resort to last night (no Laker game on). I would hold the spoon of food while Amy would divert her attention so that I could again get the spoon in her mouth before she knew what happened. Now, imagine Amy as a cheerleader, clapping, tap dancing and singing: "rah rah ree, kick 'em in the knee, rah rah rass, kick 'em in the ... other knee". Or for those of you who know this one, "Across the wind swept desert, where nature knows no man..." or even some of my old fraternity songs, "Give a cheer, give a cheer, for the boys who brew the beer, in the cellars of old Chi Phi...". Anyway, in the midst of this side show, we both looked up, laughing our butts off, to find half of the nursing staff standing at our window applauding! What a sight, one I am sure we will not live down for some time. The show was good for about one jar of Gerber's, but well worth it -- anything to ward off IV nutrition (total parenteral nutrition or TPN). If her appetite continues to drop, then we may have to revert to a nasal feeding tube. Then, if all else fails the TPN police will then come knocking. Hopefully not! We still have a week or two of potential eating difficulty ahead, so keep your fingers crossed.

Another reason that we felt so compelled to feed Susannah last night was because she had her roughest day thus far, by a long shot. Yesterday, she received her first dose of the ATG. This is an immune globulin (protein) derived from horse serum, which specifically targets Susannah's white blood cells, as opposed to the other two drugs which generally affect all fast dividing cells, such as the bone marrow, hair follicle cells, gut and mouth cells. Because ATG is derived from horse serum, it is almost always associated with some level of allergic response. Thus, Susannah felt terrible yesterday afternoon. She spiked a fever to 104 degrees (anything over 100.5 automatically requires a full fever work-up with blood cultures and a chest film) and then proceeded to unload a large projectile vomit onto my shirt. After this and some Tylenol with Benadryl, she perked up and felt much better. This morning she was back to her old self and thankfully, this first bump in the road was behind us. Of course, we are still awaiting the results of the blood cultures, but everyone feels the odds are that her reaction was indeed allergic.

We have settled into a nice routine with the nursing staff. We are allowed to and have already made a list of our favorite nurses and aides to be part of our patient care team. This way, we pretty much always are assigned one of the nurses that we get along with well (despite popular misconception, I actually do get along with nurses!). The entire staff here (nursing and physician) is very competent and friendly. Susannah is in excellent hands.

One of the highlights of the week was meeting our Care Partner, Sue Reichert. The hospital has a program for BMT families, called Care Partners, where a volunteer from the community “adopts” the patient and their family during their stay in the Twin Cities (this same program also provides the computers in the patient rooms, as well as, the specially trained volunteers on the unit who help entertain the children and their siblings). These individuals are very familiar with the transplant process and thus are especially in tune with the needs of people going through this situation. The program coordinators really go out of their way to pair people with similar interests and within minutes of meeting Sue, we knew they’d found the perfect person! The moment she walked into the room, we just clicked. She immediately fell in love with Susannah and we fell in love with her famous chocolate chip cookies!


As for big sister Madeline, she will return to us on Saturday with Grammy. We will be very glad to have her back after what seems like forever. She had a fantastic 3rd Birthday Party this past Saturday. Thanks to all of you who could make it, especially Dana, Penny, Carey, Tiffany and Aunt Kerri who put a lot of time and effort into making it so special for her. She has had such a wonderful time back in California with Tessie and Co. that we decided that it might be best if she stay a few more days, until the actual transplant is behind us. So, today she left San Diego for Los Angeles to spend a few days with her cousin Terese (and Aunt Kerri & Uncle Tom). On Sunday, Madeline spent most of the day with my family at my brother and sister-in-law's ranch in Fallbrook. She had a great time hunting Easter eggs and playing with her many cousins.

The cards and care packages we have been receiving, literally from all over the globe, really help to brighten our days. Everyone has been so thoughtful and we cannot thank you all enough. Also, the folks at St. Matthew's continue to be extraordinarily kind and generous (and feed us well, too -- apparently someone told them the way to MY heart)! Father Abbott came by today, and gave us Easter communion, as we did not feel we could leave Susannah alone on Sunday. We are grateful to have such a wonderful support system.

I've been a little more long winded than I like, so I'll sign off here. The transplant is scheduled for Thursday and we will certainly fill you in on the details as they transpire. As always, thanks to everybody for your love, support, thoughts and prayers.

All the best,

Klane (Amy, Madeline and Susannah, too!)

Friday, April 21st, 2000

In the hospital ...


Hello Everyone,

A great deal has transpired in the ten days since our last update. Let me begin with a brief synopsis, and then for those interested, I will get to the details.

Susannah was admitted to the hospital bright and early Monday morning. After five hours in the operating room and seven procedures, our little patient finally settled into Unit 4A, her new residence for the next six weeks or so. She received her first dose of chemotherapy at 4:00am on Tuesday. Now, 14 doses later, she seems to be tolerating it well, but much still lies ahead. Her bone marrow transplant is definitely scheduled for April 27th. Oblivious to all of this, big sister Madeline is off "vacationing" in sunny California.

Many of you have inquired as to the best way to reach us. It brightens our day to hear from people, so please do not hesitate. You are more than welcome to try any of the following:

At our apartment --

Amy, Klane, Madeline and Susannah White
900 Washington Avenue, SE
Apartment 104
Minneapolis, MN 55414

(612) 362-9378
(858) 967-4894 (local for those in San Diego)

In the hospital --

Susannah J. White
Fairview University Medical Center
University of Minnesota
BONE MARROW TRANSPLANT UNIT, Room 4-202
Harvard Street at East River Road
Minneapolis, MN 55455

(612) 273-0219 (direct dial to our room)
(858) 967-4895 (local for those in San Diego)
(612) 273-3000 (switchboard - ask for 4-202)

Via e-mail --

kkwhite@ucsd.edu

Please note, Minnesota is on Central Time, two hours ahead of California.


Now for the details ...

We received the long awaited enzyme activity levels on Donor B this past Friday. Her levels are indeed normal and she is not a carrier of the disease. This news was quite a relief and certainly made us all feel that our delay was worthwhile.

We spent the weekend celebrating the girls' birthdays. While it was technically only Susannah's big day (Madeline's is not until next week), Madeline sweet talked her way into celebrating her "pretend" birthday, as well. It was a two day celebration, complete with two cakes, tons of presents, balloons and special visitors, Grammy and Poppy (aka Jackie and Tom aka my parents). On Friday, we returned to Camp Snoopy at the Mall of America (this time Susannah stayed awake to enjoy the fun) and on Saturday, we saw Barney in Concert -- yes, LIVE! Both Susannah and Madeline had a blast and "danced" the entire time. It was very enjoyable for us too ... no, not the annoying purple dinosaur, but rather the looks of sheer joy and amusement on the girls' faces. Afterward, at the party, all six of us (even Poppy) wore party hats and played games. The high note of the day was the expression on Susannah's sweet face as she savored every morsel of her birthday cake.

Sunday was spent getting Madeline and Poppy off to the airport and preparing for our move to the hospital. Susannah surprised us all by "cruising" a few steps and actually "riding" her new scooter (backwards). She has really blossomed this past week, developing many new talents, including saying "Hi".

We arrived at the hospital at 7:30am Monday for a final meeting with the hematologist. Susannah was a real trooper, never once complaining about the fact that she was being starved because of the impending anesthesia. At 10:45am Susannah was carried off to the operating room, waiving bye-bye as she went. And then, the waiting began. One by one, each of the doctors filed out and gave us their reports ... The central line (Hickman) placement, back-up bone marrow harvest, and skin biopsy procedures all went off without a hitch. Ophthalmology (eyes) - very slight corneal clouding, with otherwise excellent vision. Pulmonology (lungs) - perfect function. Cardiology (heart) - slight thickening and trace leakage at the mitral valves, but otherwise unremarkable. Otolaryngology (ears)- clear membranes, free of fluid, no tubes necessary. Neurology (nerves/brain) - very normal function with no evidence of carpal tunnel issues. Neuropsychology (development) - on target with fine motor skills; some gross motor delay (more than likely due to her wearing of the hip brace); her speech, although difficult to assess in a 12 month old, appears to be somewhat delayed (see audiology). MRI (brain) - no evidence of hydrocephalus and only very slight changes. Lumbar puncture (spinal tap) - pressure was borderline high (probably due to anesthesia), but still within normal limits. All of these reports were excellent news.

The audiologist (hearing) was the last to make her report and unfortunately, the news was somewhat disappointing. Apparently, Susannah has some sensorineural hearing impairment. This came as quite a shock to us all, including the audiologist and the otolaryngologist, as she appears to hear quite well. While Hurler Syndrome can cause hearing loss, it does not cause sensorineural loss so early. Ironically, the deficiency is NOT related to Susannah's disease. It is caused by either inherited genetics or by an antibiotic sometimes given at birth, gentamicin. Susannah not only has a family history, but also received this medication. Testing showed that Susannah has a functional level of hearing, but without intervention, she will likely have some trouble learning advanced speech (a possible explanation for her delay in this area). The loss is mild in the right ear and moderate in the left, but it is across all frequencies, so hearing aids will do wonders. This type of hearing deficiency usually does not get better and it is not expected to get worse. However, most people do not receive bone marrow transplants, thus there is some uncertainty as to whether or not the transplant may have a positive effect in this area as well.

Finally, after all the procedures, reports and a brief stint in the recovery room, Susannah made her way to the bone marrow transplant floor (Unit 4A) -- the place she will call home for the next couple of months. She slept peacefully as she received her first dose of Busulfan (chemotherapy drug) at 4:00 am Tuesday. Busulfan is given orally or through an NG (nose) tube every six hours for four days. Once this course is completed, it is followed by a second chemotherapy drug, cytoxan, which is administered intravenously twice a day.

Time and calendars on Unit 4A revolve around Day 0, or transplant day. Patients are admitted to the hospital on Day -10, and the count down to Day 0 begins. Preparative chemotherapy is administered on Day -9 and continues through day -1. Finally, the bone marrow is given on Day 0 and the subsequent days are counted in positives, with the first day after transplant being Day +1.

Today is Day -6 and so far Susannah has been doing quite well. She has now received 14 of 16 doses of Busulfan and has tolerated them nicely. The first few were given through her NG tube, but after she twice pulled it out, we opted to try dosing the medication orally. Thus far, there have been no incidents to speak of. Busulfan tastes terrible and children often vomit after receiving it. If vomiting occurs within the first 30 minutes, it must be dosed again. Susannah has had one close call (she spit-up at 25 minutes), but so far so good. She is also receiving three medications prophylactically to prevent nausea, fungal infections and seizures. There is a tremendous amount to keep track of, so we have made a drug schedule to help keep things straight. This should really come in handy in a few days when the number of medications Susannah is receiving will multiply times five!

Through all of this, Susannah continues to eat, play, laugh and smile. We hope and pray that her course remains smooth and uneventful. She has a long road ahead, but she is a strong little girl who is deeply loved and she is at the leading treatment center in the world -- a combination that cannot be beat.

As always, we thank you all for your continued encouragement, support, love and prayers.

All our love,

Amy, Klane, Madeline and Susannah

Tuesday, April 11th, 2000

Update from the Twin Cities


Hello Everyone,

We arrived in Minneapolis Saturday evening, April 1st, after an uneventful flight. Both Madeline and Susannah did very well on the airplane, thus we all did very well. What was eventful, however, was the fact that we had so much luggage (nine GIANT bags, even by Amy standards -- the people at the baggage claim were laughing!) that it took two oversized cabs (a wagon and a van!) to transport us to our apartment.

Our apartment ... very spacious and convenient to everything. The furnishings are comfortable, yet very collegiate; we feel as though we are back in school and should have nothing but an empty case of beer and a half eaten pizza in the fridge! In fact, we are quite certain that all of our neighbors live by this creed. Klane wants to join them. I am just thankful the apartment has those beautiful "dorm style" cinder block walls, so we too are not up partying until 3:00am! The students are actually all very nice and very helpful (never mind that they look at us like we are 80!). Some have even offered to watch Madeline for us. The building has an exercise room, a laundry room, a computer lab and off-street parking. We are just blocks from the hospital and within walking distance to virtually anything else we might need. It really is going be a nice "temporary house" for us.

Everyone here in Minnesota has been so friendly and helpful. Prior to our arrival, I arranged to rent (for next to nothing) a crib for Susannah and a "big girl bed" (toddler bed) for Madeline, as well as, a highchair. When we walked into the apartment, they were not only already assembled for us, but they were all new; the highchair still even had tags on! The people at Baby Grand really out did themselves -- they even called the next day to see if we needed anything else!

Madeline was not only elated at the sight of her "big girl bed", but also at the BIG package of presents that was waiting for her from all of her friends back home. Susannah made herself right at home scooting from room to room inspecting the cleaning crew's vacuuming job and helping herself to any microscopic tidbits they might have missed.

My sister, Tami, arrived on Monday the 3rd and we had big plans. She is rented a car at the airport, swung by to pick us up and then, we were off to Mall of America! For those of you who are scoffing, it is not JUST a mall. There is an aquarium, an amusement park, lots of bars and
much, much more! Madeline had a blast and cannot wait to return (a warning to those of you coming to visit). As for Susannah, she decided it was naptime and snoozed through the entire adventure.

We had a meeting at the hospital Tuesday morning regarding the schedule for Susannah's treatment. We are now in the process of "work-up week" and doing all of the baseline testing required for transplant. She is being evaluated by many specialists: Neurology, Pulmonology, Cardiology, Otolaryngology, Radiology, Ophthalmology, Neuropsychology and, of course, Hematology. So far so good! We should have the official results by week's end. Susannah has been a real trooper through it all and has been endearing herself to the medical staff with her latest trick of blowing kisses. Fortunately, her blood work went off without a hitch this time. In the past, it has not been uncommon for her to endure as many as seven needle pokes in order to get one vile of blood. However, this time they were able to get all that they needed (eight tubes) in just one stick!

Her work-up will continue through Wednesday the 12th, then we should have a few days free until she is admitted on April 17th. Susannah's first morning as an inpatient will be spent in the operating room, at which time she will have her central line (Hickman) placed, as well as an MRI, lumbar puncture (spinal tap) and a complete eye exam. They will also remove a small amount of her own bone marrow to have on reserve, just as a precaution. Chemotherapy will begin the following day and her transplant is scheduled for Thursday, April 27th.

This past Saturday evening we had dinner with The Richardson's. Their son Regan, also has Hurler Syndrome, and is just recovering from his transplant. Although we have been corresponding for the last couple of months, this was the first time we had actually met. It was a nice time and we received a great deal of much needed advice. Madeline and their three year old daughter, Fayth, became fast friends. The support and advice we have been receiving from the other families who share our predicament has been phenomenal. It is quite amazing, that with everything each of them is also going through, they still have found the time for us.

We have found tremendous support from St. Matthew's here in the Twin Cities. Our dear friend, Father Newman, the priest who married us and baptized both girls, happens to be from the Minneapolis area. With one telephone call to an old friend, he established an instant "family" for us. Their parish has been awaiting our arrival and praying for Susannah for weeks. They have been so very kind to our family -- opening their homes to us, offering rides, babysitting, meals, furniture and anything else we might need. We cannot begin to thank them enough for their generosity and hospitality.

My mom and dad arrive tonight and Wednesday, respectively. On Saturday, April 15th we will celebrate Susannah's 1st Birthday with a special party, planned by big sister Madeline. On Sunday, Madeline and her "Poppy" will board a plane for California. She will be spending the next ten days in San Diego with her friend Tess. It is sure to be a fun-filled time for her. She will be back "home" with all of her buddies and in her regular routine. They will even be throwing a party for her 3rd birthday on the 22nd; she can hardly wait! We will certainly miss having Madeline around (much more than she will miss us), but since we will both need to be at the hospital so much during those first two weeks, we really feel it is best for her. We know our friends will dote on her and be able to give her far more attention than we could even begin to think about. She has grown up with these kids, loves their families as if they were her own and is more than comfortable in their homes. We are lucky to have such wonderful people in our life.

Thanks again to everyone for all you have done to make this situation easier on the four of us. We appreciate your love, kindness, encouraging words, as well as your thoughts and prayers, more than you can imagine.

Love Always,

Amy, Klane, Madeline and Susannah

PS … Still no word on Donor B's enzyme activity level, but they are expecting some information on Tuesday. We will let you know once we hear something.

Friday, March 31st, 2000

Minneapolis here we come!!!


Hello All,

It's official. Our bags are all packed and we are actually leaving for Minnesota tomorrow, April 1st.

Donor B it is. We received her physical exam clearance today and she is the picture of health. Ironically, however, her enzyme activity level also came back on the low side (nearly identical to that of Donor A). The doctors feel confident that her low level was the due to specimen mishandling. For the most accurate results, blood should be drawn and tested within one day; hers took three. Thus, on Monday, Donor B will have her blood re-drawn and her enzyme level re-checked.

We are scheduled to meet with the folks in Minneapolis on Monday. Susannah will most likely not begin her work-up until Wednesday, when the enzyme level is back. This two day delay will push back the actual transplant to April 27th (Donor B's donor center only harvests marrow once a week, on Thursday). To accommodate this, we will now have two or three days between the completion of work-up and the beginning of chemotherapy to explore the Twin Cities. This little reprieve will allow Susannah to celebrate her 1st Birthday outside the confines of the hospital.

Susannah, Madeline, Klane and I will be flying from San Diego directly to Minneapolis. My dad has graciously offered to drive our car out, so we will not be without "wheels". We will arrive Saturday evening and my sister Tami arrives on Monday morning to help care for Madeline. She will be with us for a week and will help get us settled in while we are busy with appointments.

For those of you still unfamiliar with the timing of this whole thing, let me explain. The first week or two that we are in Minneapolis, is called work-up week. Susannah will undergo a variety of medical tests to establish baselines on her heart, lungs, brain, eyes, ears and virtually anything else you might imagine. At the end of this time she will undergo surgery to have a central line placed in her chest. As this line goes directly into her heart, it will allow medications to be given and blood to be drawn without having to stick her several times a day. Eventually, she will even receive her donor's bone marrow through this same line. The day or so following this surgery, chemotherapy (to destroy her own bone marrow) will begin and this regimen lasts ten days. Once completed, she will then receive the bone marrow transplant that will save her life! This should occur on or around April 27th. Following transplant, she will remain in the hospital until the donor's marrow begins to grow inside her and she starts to acquire a new immune system. Once discharged, we will visit the doctor's office every day, eventually tapering off to every week, until she is well enough to return home to California (approximately 100 days).

Many of you may be wondering, who is this Donor B they keep referring to? Since the identity of unrelated marrow donors is kept confidential until one year following transplant, recipients and their families are only permitted to know the donor's age and sex, nothing else. Susannah's generous donor is a 29 year old female. So, now you know everything that we do.

We finally have an address. One of the apartments that the hospital leases to families such as ours has become available. We were number 80 on the waiting list, so we were surprised, yet very relieved when our name actually came up. It is a two bedroom, fully furnished (down to the linens) and within walking distance of the hospital. As of April 1st, our address will be:

Amy, Klane, Madeline and Susannah White
900 Washington Avenue, SE
Apartment 104
Minneapolis, MN 55414

The telephone number at the apartment will be:

(612) 362-9378

Since the apartment telephone, as well as the telephone at the hospital, only allows outbound calls to be local, (all incoming calls are fine), we have also established the following numbers:

(858) 967-4894 at the apartment
(858) 967-4895 at the hospital

One of these will be at the hospital and the other at the apartment. For those of you in the San Diego area, calls placed to these two numbers will be considered local calls. Any incoming calls, regardless of where they are from, are not charged to us. Also, our home number will be forwarded, just in case you forget. Please feel free to call us on any or all of these numbers. We will look forward to keeping in contact with everyone.

As soon as we are settled and have any further news to report, we will certainly be in touch. Thank you all for keeping Susannah in your thoughts and prayers.

All our love,

Amy, Klane, Madeline and Susannah

Thursday, March 23rd, 2000

Still in California …


Hi Everyone,

We just wanted to send a brief note to let everyone know we are still in California.

We were set to leave for Minnesota on Saturday the 18th, but were informed on Thursday the 16th that, while she passed her physical exam with flying colors, Susannah's bone marrow donor had an enzyme level that was lower than anticipated. Since the enzyme in question is the same one that Susannah is lacking and since there is another equally matched donor waiting in the wings, everyone involved felt it would be best to explore the possibility of using the second donor. So, as it stands, the original donor (Donor A) has been placed on hold and the secondary donor (Donor B) has been asked to give blood for enzyme testing, as well as undergo a complete physical exam. The doctors have been assured that this process can be expedited such that the delay will not exceed three weeks. Should things become at all delayed with Donor B, we will revert to using Donor A and proceed with the original plan. Because time is of the essence when dealing with Hurler Syndrome, we do not want to wait very long. Two to three weeks is acceptable, two to three months is not. It is also important to note that the donor having a low enzyme level is not necessarily indicative of a poorer outcome for Susannah, in fact some of the best results in transplants have come from related donors who were carriers of the disease. Although, given the choice, a greater level of enzyme in the donor cannot hurt, thus we will pursue Donor B.

Aside from the roller coaster of "going then not going then going then not ..." (our plans literally changed nine times in 24 hours), we are all doing well -- very tired and mentally exhausted, but well. Most likely we will not leave for Minneapolis before the first of April, but as I just mentioned plans can change on a moments notice. We will certainly keep you posted as things develop.

Thank you again to all for your wonderful words of encouragement and support, as well as your thoughts and prayers.

Much love,

Amy, Klane, Madeline and Susannah

PS ... Madeline is doing fine following Monday's surgery to place tubes in her ears (who ever heard of a kid requiring tubes after having only two ear infections in her life!). Other than Klane being out of town and the girls and I spending nine hours at UCSD, the procedure itself was simple and went off without a hitch.

Sunday, February 20th, 2000

To All Our Family and Friends,

Because the number of you who know, equals the many of you who still do not, Amy and I felt it necessary to send this letter to inform everyone of our younger daughter, Susannah’s situation. We apologize for the impersonal format, but as there are some of you, who as much as you are dear to our hearts, we do not get the chance to see or communicate with as often as we would like, thus we felt this was the best way to reach the greatest number of people.

In December Susannah, then eight months old, was diagnosed with a rare (there are only about 200 cases in the states) genetic condition called Hurler Syndrome (Mucopolysaccharidosis Type 1H). Since the disease is progressive, babies appear to be unaffected at birth (it often goes undiagnosed until the second year of life) and continue to develop normally until enough time has lapsed for the disease to do its damage. Basically, her body is lacking in an enzyme responsible for breaking down cell by products called mucopolysaccharides. Without the enzyme around to clean things up, these by products deposit themselves throughout the body -- in the heart, lungs, eyes, ears, throat, liver, spleen, bones and unfortunately brain. There is virtually no area of the body that goes untouched … there is no cure. Without treatment, the disease is fatal by the age of 11 and usually much sooner.

After much research, contemplation and soul searching, Amy and I have determined the best possible course of treatment for Susannah is a bone marrow transplant (BMT). We want to give her the chance not only to live, but to live well and in her lifetime benefit from a potential cure. Although the benefits are great (most notably, it minimizes the effects to the brain), the procedure is not without risk. In fact 20% of the patients do not survive past two years post BMT. Those who do, however, usually experience a near normal childhood and are often in their age appropriate grade in school. BMT for Hurler Syndrome has been used as treatment since 1980; thus the oldest survivor is only 20 years old. What happens to these kids after age 20 is uncharted water. But with the advances being made in the field of gene therapy, there is great potential for an actual cure in the next decade or two. By the time Susannah reaches her twenties, perhaps she will already be cured; this is our greatest hope!

Thus, in mid-March, Amy, Susannah, Madeline and myself will pack our bags for the University of Minnesota, the leading center for pediatric BMTs (and more importantly for Hurler BMTs -- of the 80 or so Hurler Syndrome transplants done in the states, they have conducted over 60). Amy and the girls will be in Minneapolis for a minimum of four months. I am able to take two months off from my residency during the most critical phase of the treatment and then I plan to commute to Minnesota every other weekend until they return. We are fortunate to have wonderful family and friends who have agreed to rotate in and out each week to help care for Madeline (and us) while we focus on caring for Susannah.

Although it has been a wild couple of months, I’d say we are doing reasonably well under the circumstances. Obviously the first few days after Susannah’s diagnosis were spent in shock and disbelief and a wide array of other emotions. However, we quickly began devoting our energy to researching and finding the best possible treatment for her (within two weeks of that life altering phone call, we were already in Minnesota for a consult). As strong as we are trying to be through this, when we find ourselves alone it really hits home, so we are trying to surround ourselves with loved ones and most of all each other. As for big sister Madeline, she is still blissfully unaware of the magnitude of the situation. She looks forward to visiting the snow again and staying in her “temporary house”. Thankfully, she equates hospitals and doctors’ offices to “Daddy’s work” and not some place traumatic. The many appointments already associated with Susannah’s illness have been deemed, in three-year-old lingo, “Susannah’s work”. Fortunately, aside from her serious skeletal problems (whoever said specializing in orthopaedics wouldn’t come in handy), our sweet Susannah is showing no outward signs of the disease. She is a happy little ten month old who loves to eat Cheerios and is in awe of her big sis. She continues to progress in her physical, intellectual and social development and has not been afflicted with the multiple ear, upper respiratory, eye and heart problems usually common in the early stages of Hurler Syndrome. Were her diagnosis not documented, she would appear to be your average, healthy ten month old.

In closing, I really want to thank everyone for being so supportive. The first week was incredibly difficult on everyone, but now with the spiritual, emotional and, of course, meal aid that you all have provided to us, we are stronger than ever and totally committed to getting through this with the minimum of scars to Madeline, Amy and myself (thankfully, Susannah is too young to remember). Though at times I still find Amy breaking down and myself lost in thought, I know that with the continued support of all of you, we will endure and see Susannah thrive. We are truly grateful to be surrounded by such a large community of wonderful people.

With all our love and best wishes to you and yours,

Klane, Amy, Madeline & Susannah White

PS … Realizing the great number of folks we’ll need to keep posted on Susannah’s progress while we are in Minnesota, the most efficient and convenient way to do so is via e-mail. Those of you wishing to be included in these updates, please e-mail your addresses to me at: kkwhite@ucsd.edu -- thanks!


For more details, please read on . . .

For those of you who are very detail oriented, I have included some excerpts from my original (much longer) draft of this letter. Although somewhat redundant, it gives a much more detailed account of the disease, the BMT process and the events leading up to Susannah’s diagnosis.

At about four weeks of age, we noticed that Susannah had an abnormal forward bend in her spine. After several months of evaluation and observation by one of my bosses at Children’s Hospital (Dr. Dennis Wenger), we discovered that Susannah also had underdeveloped hip joints. Since then she has had to wear a special brace to aid her hips in proper development. With this discovery of multiple bony “dysplasias” (literally bad growth) we were referred to a very respected dysmorphologist (a doctor who specializes in the evaluation of abnormal features to make the diagnosis of various genetic, developmental diseases) named Dr. Ken Jones at UCSD. After some going back and forth we finally concluded that we had better check Susannah for a series of diseases consistent with her specific bone abnormalities called MPS (mucopolysaccharidoses).

There are seven such described diseases, which are the product of a protein deficiency passed on by one or both parents. Patients with this protein (or enzyme) deficiency are unable to break down certain specific byproducts within their body called, you guessed it, mucopolysaccharides. These molecules are normal connective tissue elements found in everybody, but in the face of one of these protein deficiencies they build-up to abnormal levels causing mechanical problems within the parts of the body that they deposit. On December 23, 1999, we were informed that Susannah was diagnosed with a disease known as Hurler Syndrome (or MPS I), the most serious of the seven.

The natural history (medical lingo for untreated disease progression) of this disease is as follows. This is an autosomal recessive genetic disorder, meaning that Amy and I are both carriers of the disease (as are potentially 25% of our brothers and sisters). As infants, these children may have curves in their spine as does Susannah, they commonly have multiple ear infections, colds and in severe cases, early heart, lung or liver problems and possibly developmental (cognitive and motor) delay. As I mentioned, Susannah, so far has only manifested the skeletal problems. Almost all of these kids by age two have progressed to the list of problems I just mentioned to a more or less severe degree. The most profoundly affected kids end up dieing by age five (this makes up about 50% of children afflicted by Hurler Syndrome), the other half die by age eleven (this means all of them). Most notably these children start rapidly regressing in their cognitive and motor skill development by three to four years of age. If allowed to progress, they end up with the skills of a six month old infant at age eight or nine, if not sooner. They have a remarkably short stature and require surgeries for removal of their tonsils/adenoids, as well as, placement of shunts in their brains to alleviate fluid build up (blocked by the deposition of the mucopolysaccharides) or hydrocephalus. Most of them pass away, however, as a result of either airway obstruction (difficulty breathing because their wind pipes become blocked), or they die from heart failure (because their heart becomes too rigid, or the blood supply is blocked). It is our impression that if left untreated, these children are not terribly uncomfortable, but require an inordinate amount of specialized medical care and intervention.

As any parents would do, faced with this situation, we wanted to know what we could do to give Susannah a chance to live, and live well. At this time there are two possible treatments for this disease, neither of which is totally curative. The first is a bone marrow transplant (BMT) or umbilical cord blood transplant, and the second is enzyme replacement therapy. Enzyme replacement therapy, first of all is not available to us as an option at this time, due to FDA restrictions. Basically, as the name implies, patients are given a dose of the enzyme that they lack on a once or biweekly basis. These children have shown good response to the therapy with resolution of many of their symptoms. The one major caveat to this treatment is that it has not been shown to affect their neurological (cognitive and motor) deterioration. (There is a variant of this disease that is less severe called Hurler-Scheie Syndrome, for which this treatment is more appropriate because these kids often have normal cognitive development. We are nearly 100% certain, unfortunately, that Susannah has the real deal and not this more mild form of the disease.) As such, we of course looked into the benefit of a bone marrow transplant.

BMT has been performed on children with Hurler Syndrome since 1980, with the oldest living survivor being around 22 years of age. This young man, although greatly incapacitated by severe musculoskeletal problems, works at home from a computer, and has near normal mental capacities. BMT consists of giving the patient bone marrow cells (immature immune cells) from another person. These new cells from another person have normal levels of the enzyme that children with Hurler Syndrome lack, and because the job of these cells is to sort of “clean things up” anyway, they are perfectly adapted to the situation of breaking down most of the unwanted mucopolysaccharides.

In order for this to take place, the patients must undergo chemotherapy to kill all of their own bone marrow cells. Just like patients who undergo chemotherapy for cancer, this is a fairly dangerous and uncomfortable procedure. With the donor being anybody other than Madeline (who was not a match for donation), there is a 20% chance that Susannah will not survive beyond two years from the time of the transplant. This would be due to complications related to the transplant such as infection (because her immune system will be temporarily compromised) or graft versus host disease (GVHD), when the donor cells attack the recipient’s body). Additionally, the longer that you wait, the more the disease progresses and increases the risks of BMT. Children under two years of age have a much better prognosis and outcome than those kids transplanted after age two.

It is important to understand that BMT is not curative. These kids still have significant orthopaedic problems and they do not regain skills already lost (so far Susannah is still progressing and not yet regressing). Some of the kids require eye surgery as well, and it is still not clear whether or not they will all require heart valve replacements. Common orthopaedic surgeries for post transplant children include: Spine, hips, knees, hands and sometimes neck procedures. Of the children who have survived the transplant, most all seem to be doing fairly well. Many of the kids are teenagers now, and some are in age appropriate school grades. What happens as these children become adults, living into their twenties is completely uncharted territory. As for Susannah, she will at the very least require major spine surgery when she gets a little older, and hopefully if she is transplanted soon enough will have normal cognitive development. With all of this said, there is in the near future (maybe 10-15 years) the possibility of gene therapy. Gene therapy would consist of replacing Susannah’s defective DNA with new, working DNA. This would hopefully be curative, but as I said is a long way off.

So after much discussion, research and thought Amy and I have decided to risk going ahead with the BMT. We are very fortunate to have the advice and experience of the many prominent physicians involved in Susannah’s care who have been advising us, not only as our doctors, but also as friends. BMT is performed all over the country at many university and non-academic medical centers. The University of Minnesota, in Minneapolis, is the location where more pediatric transplants are done than anywhere else in the world. More importantly, they are the academic center where the majority of research is being done on Hurler Syndrome, and have done more transplants for patients with the disease than all other centers in the country combined. Being in medicine, it has been my experience that if you are going to have a potentially life threatening procedure, that to a certain extent it makes a huge difference how comfortable a center, its physicians, nurses and other support staff are with the disease entity that they treat. As for Hurler Syndrome, it is well recognized that these kids have a much greater problem with GVHD than say kids with leukemia or other diseases that require BMT, and at the University of Minnesota they take special steps to help reduce this risk.

As of now, we are blessed in that there are 40 potential matches for Susannah (many people never find any). We are presently waiting for the logistics to be worked out and our tentative date for transplant is April 3rd. We will go back to Minnesota the week of March 20th. I will need to take a minimum of two months off from work, and Susannah, Amy and Madeline will be required to stay in Minnesota for at least four months. Fortunately for us, the folks at UCSD (the staff attendings and other residents) have been extraordinarily supportive, and have given me a lot of flexibility. As for all of our friends and family this is equally true.

So from here, you may be asking what you can do, if anything. There have been several bone marrow drives arranged by friends with Susannah as a “poster child”. But, as I mentioned above, she already has a long list of potential donors, so we are really only doing this for the greater cause. If you want to be a part of this, that is great and we can let you know about available opportunities to do so for free, but understand that this is not for us, but you. Also understand that you will be agreeing to be a donor for anyone who might match your specific bone marrow type. The test is quick and easy; it looks and feels like your average blood test. Donors must between the ages of 18 and 60 years old.

Luckily our insurance company has agreed to pay for the transplant, as well as a few of the travel expenses and a limited amount of housing costs. On that note, if things go well, I plan to commute every weekend or two back and forth after the first two months. Some friends and family have graciously offered us their frequent flier miles. This, we think is a greatly practical and necessary gift that will help us immensely. Other issues may come up, but for now that is where things stand.

Finally, we cannot begin to express our gratitude to those of you who have been providing us with meals, miles, babysitting and all around support, as we sort through this difficult process. The outpouring of love and kindness we have received has been phenomenal. Our family and friends have truly been instrumental in seeing us through this. Amy’s parents and/or my mom have visited nearly every weekend -- without Jackie, our home would have fallen to pieces during those early weeks. The delicious meals that everyone has delivered to us have been invaluable. Were it not for you all, there would have been many nights that we simply would have forgotten to eat. Thankfully, due to the many people who love her as if she were their own, little Madeline has been kept entertained and virtually oblivious to the seriousness of the things going on around her. Your many cards, letters and calls have touched us deeply and are very much appreciated.

All our love,

The White Family

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