Isabella Griggs's Journal
Pre-school Tune Up
Written Aug 16, 2013 10:18amBella and I spent most of our day yesterday at or in the vicinity of Children's Hospital as she had multiple appointments in what we will call a little 'tune up' before school starts in a couple of weeks.
After spending the past few weeks watching Bella wretch and gag on nearly any amount of food that passes through her esophagus, we were concerned about a possible stricture of the esophagus. A stricture is excessive scar tissue which forms in the esophagus at the site where it was connected after she was born. In the past, stricture has caused her to aspirate and develop pneumonia. After expressing our recent concerns to her surgeon's nurse, an esophagram (radiologic exam of the esophagus using barium contrast) was ordered and that's where we started our day at 9am.
Bella was a champ and, despite some understandable resistance, she took several large swallows of that yucky, disgusting, chalky orange (seriously, why even bother flavoring it?) barium beverage. I watched the monitor as it slowly, but steadily crept down her esophagus which showed no signs of stricture. Unfortunately, the contrast made it painfully obvious that her swallowing is uncoordinated and, to be frank, looks crappy. Her muscles, as with many patients with esophageal atresia at birth, just never developed peristalsis. No surprises there. What was surprising is that her nissen (surgery where part of the stomach is wrapped around the top of the esophagus to prevent vomiting/reflux) was really, really tight - allowing only small amounts of barium to slowly pass through. The combination of poor muscle motility and a tight Nissen is what has led to the discomfort with swallowing. Thankfully, no surgery is necessary (or even possible) to remedy this problem. Bella just needs to work harder at chewing and swallowing and drink after each bite.
I'm proud to say that despite overscheduling and even though the radiology nurses were very skeptical, we did make it to our 10am appointment on time. Now under normal circumstances, this wouldn't be so impressive however, radiology NEVER runs on time and the second appointment, was in a completely different clinic building. We had to drive to from the hospital in Wauwatosa to downtown Milwaukee for a dental appointment. Dr. Fischer is definitely worth the drive tonight as he has a good rapport with Bella and has taken the time to read anything on Fanconi Anemia that he can get his hands on. So at 10 o'clock on the dot we walked into the Dental office of the downtown CHW clinic building.
Bella got an official thumbs up from Dr. Fischer for her recent teeth brushing. Herr teeth were cleaned, a sealant applied to one tooth, and a thorough examination for any oral lesions or lumps was completed within reasonable time which means Bella was cooperative. All is good and we were assured that the tooth fairy should be visiting our home soon as two teeth were very loose.
With a break in the action, we decided some retail therapy was necessary and headed over to Toys R Us where Bella quickly spent her remaining birthday money on a new set of Lego Friends and some accessories for her bike before promptly telling me she is now "totally broke." Then we stopped for lunch at Q'doba and hit the CHW pharmacy for some candy for dessert.
Just before our final appointment for the day, we stopped by the surgery clinic to see our favorite nurse, Lynn, and discuss the results of the esophagram. Although Dr. Oldham isn't in today, Lynn had already reviewed the test and agreed with my assumptions. Of course the doctor has to give his official agreement, but the results are pretty clear cut. Regardless, it was great to see Lynn again and catch up with her for a few moments, even if we weren't on the schedule. My apologies to those patients who had to wait a bit longer while Lynn chatted with us!
Our final appointment was with the Ear, Nose, and Throat specialist. It should come as no shock that Bella has yet another ear infection. I think it would be more shocking if she didn't have one as summer and frequent swimming seem to bring on ear infections regularly for Bella. We got a refill of her ear drops prescription and Dr. Robey suggested that we should look into ear plugs when Bella goes swimming. We will discuss this with her audiologist.
Fortunately, these appointments are all she needs before school starts unless something else comes up in the mean time. That means we will hopefully be able to set aside Bella's medical issues and just enjoy the last few remaining days of SUMMER!
Another Day, Another Doctor
Written May 22, 2013 12:10amBella's dental appointment this morning went much more smoothly than the endocrine fiasco of yesterday thanks in part to a wondrous little intervention called nitrous oxide. Dr. Fisher gave Bella just a bit of happy gas to help calm her anxiety prior to putting sealants on her teeth to prevent cavities (9 years cavity free and still going strong!). In fact, her appointment went so well that I barely had time to finish my discussion with the receptionists about The Great Gatsby book vs. Movie when Dr. Fisher reappeared and told me he was done. Wow....that must be a record!
Dr. Fisher wants to continue to follow up every 4 months to closely monitor for oral lesions and reduce the anxiety and oral aversions which are so ingrained in Bella from the past 9 years! So we will return in August, just before school starts again.
Because I possess the patience of a 2 year old and didn't want to wait until the rest of the endocrine blood test results came in to hear about Bella's CBC, I made Ben call the transplant clinic today and get them. I don't recall the exact numbers for her whites, reds, and hemoglobin but I do know her platelets were 180 and her ANC is just over 1000. Yea! We're sticking around that range that I am comfortable in which always includes at least 4 digits. Still mild bone marrow failure, but not falling into the moderate range, yet.
And now for those of you I left hanging yesterday, here's the rest of the BIN story.....As it turns out, Bella DIDN'T push this other kid down on the playground yesterday. See, I knew she was telling the truth, sort of. You see, she did KICK him.....that little stinker does have a way with her words, doesn't she? Apparently the kid kept singing his version of Carly Rae Jepson's song over and over and over to Bella "Call me maybe, call me maybe..." That song is soooo last year and frankly it's well past the annoying point so I think I might have kicked him too! Just kidding. Well instead of just walking away, Bella got annoyed and hauled off and kicked the kid in the foot to get him to stop harassing her. Apparently Bella is destined to be an NFL kicker because the kid went and told the teacher, showed her a 'bruise' already forming on his leg (even though Bella kicked him in the foot), and the teacher punished Bella for it. She gave her a BIN which finally made it home in her backpack today. I'm not saying she didn't deserve it as she should never kick someone, but the kid shouldn't be harassing Bella either, especially after the rough day she had. So for the next week, Bella won't be having any friends over nor will she be playing on her iPad, watching TV, or using other electronics. Life is going to be pretty rough for Bella for the next 7 days as she'll be singing the jailhouse blues.
Written May 21, 2013 12:59amAfter having the time of her life and feeling like a princess yesterday at the very successful More Birthdays for Bella fundraiser for Fanconi Anemia Research Fund, it was back to reality today for Bella. She kicked off her morning with a marathon appointment in the Endocrine clinic for a check up on how things are going with her growth hormones. The good news is, she can start giving herself injections in the thigh she has had to avoid for the past 6 months due to a build up of scar tissue. The scar tissue has dissolved and is doing much better.
Unfortunately, that's about the only good news we got today. I mistakenly stayed home from this appointment and just sent Ben with Bella so I could finish up some stuff from the fundraiser. Generally Endocrine appointments consist of a quick weight, height, and a conversation of
'Nope, no random side effects from the injections but she's growing like a weed'
"Great, see ya in 6 months."
Sounds easy, right. Not so much this time. The height and weight showed that Bella's growth has slowed considerably....like waaaaayyyy slowed down. Last year she was growing at a rate of 8 centimeters per year and now she's only growing at half that rate. 4cm per year. Boo.
What does this mean? Well, it could be one of two things. First, it could mean that they simply need to up her dose. Maybe, because of her increase in height and weight, she isn't getting enough HGH in he injection. Or it could mean that it just isn't working anymore. The possibility exists that Bella's body has developed a tolerance for the HGH and it is no longer effective. That would suck. Ugh.
The only way to make this determination is to draw blood and see what Bella's levels are at. We were not prepared for this today and Bella was NOT happy about it. She may be tiny, but she has a lot of fight in her. Daddy (oh poor daddy) had to hold her sitting on his lap with his legs wrapped around hers (to prevent her from KICKING the guy doing the blood draw) and his chin restraining her shoulder. One of Ben's arms was wrapped around her chest, holding him against her and his other arm prevented Bella from using her free arm to give the phlebotomist and upper cut. Then the guy drawing the blood had to hold down her other arm while simultaneously trying to stick a needle into a miniscule blood vessel. Thankfully, after just a bit of digging, he found the vein and drew the vials necessary.
The results will take a few days to come back, but if her levels are normal or high, then we are done. It means Bella's body simply isn't responding to the injections anymore and continuing to give them to her every day wouldn't do any good. She's still a good 4 inches shorter than her peers and stopping the injections doesn't mean she won't grow any more, but it does mean she likely will never reach the height of an average adult. The whole reason why we chose to go this route is because we didn't want her height to be a disability for her when she is an adult and I must admit that I am going to be quite disappointed if 2 and a half years of daily injections will not help us reach this goal. She will have enough challenges as a teenager and adult. I don't want her height to be one of them.
We are praying that the results will come back low because that simply means that her dose needs to be increased. There really is no way to guess what the results will be and we simply must just patiently wait to hear from the doc. Because blood was already being drawn, they took additional vials for immunology studies. This was recommended by Dr. Wagner from Minnesota as Bella seems to get sick a lot more than other kids, even others with FA. I will be interested to see if her immune system is working as well as it should be.
Because of the long endocrinology appointment (oh, and did I mention we also found out that our regular endocrinologist is moving to Chicago soon and the only other endo doc with a good koweldge of Fanconi Anemia at CHW left last year? Yea, that was great news too. They did say they are getting an endo doc from Cincinnati Children's soon though and Cincy is an FA center so we're hoping that doc comes with some background in FA otherwise we'll be travelling 3-4 times per year to Minnesota for endo appointments. Won't that be fun?) and having to coordinate the immunology study and the difficult blood draw, Bella didn't get to school until 1:00 this afternoon.
As if her day wasn't bad enough, during recess another child on the playground accused Bella of pushing him down(um, kid, have you SEEN Bella? She's like half the size of you - oh wait, it did just take 2 adults to hold her down, lol) at some time during the whopping 2 hours she was at school and even though Bella and her friend Ainslee both swear it didn't happen, and the teacher didn't even see it, Bella was told to stand against the wall and informed she was going to be given a B.I.N. (Behavior Infraction Notice = basically a note home telling your parents you were naughty) if she didn't tell the teacher why she pushed him. Bella wouldn't admit to it and I believe she didn't do it. So now she's scared to go to school tomorrow because she thinks she's getting a BIN.
Well guess what? Little Miss is getting her wish. She gets to play hooky in the morning again because she has yet another appointment. Tomorrow she visits the dentist at CHW to have sealants put on her teeth. At least she will be able to delay facing the music for a little bit.....and that will give Momma Bear a little time to make some phone calls to school and figure out what's going on with that BIN. Talk about a rough day for my little Princess. Let's hope and pray tomorrow is easier for her!