Made of Gray

Hello Boston - Boston Children's Hospital Update

Raising the Rare ChildHolly D Gray2 Comments

We are now home from our week long trip to visit the CAIR team at Boston Children's Hospital. I am now properly medicated with steroids, antibiotics and a hopped up version of decongestant that is supposed to be healing my massive sinus infection. I don't have a voice at all so writing it will be! 

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We were in Boston for a week this time. Each weekday we had appointments at the hospital and spent more than half the day there. The rest of the time we were on the move sight seeing, dealing with behavior issues that would rival any toddler you've ever seen and eating all the good food we could find. 

Since this post is about the medical updates and specifically what we found out for Caleigh, I'll save our Boston adventures for another post. 

The first appointment was with the CAIR Clinic (Center for Advanced Intestinal Rehab.) This team is the reason for transferring Caleigh's care to Boston and we still love them and their ideas. The are very impressed with Caleigh's growth. She has gained 10 lbs, up to 48lbs, and grown to 3ft. 7 inches since the end of April. She is still small, but now back on the growth charts. If I remember correctly 3% height and 30% weight on the chart. So the plan is to cut back her calories and slow the growth down a bit. We will check her weight locally in a month. 

Caleigh has been urinating a lot since we went up on fluids and calories. We are basically in the restroom every 30 minutes to an hour and if she has an accident she gets very upset about it. Like screaming bloody murder anywhere we happen to be. This includes public restrooms, parking lots, therapy clinics... anywhere. We are going to cut out one pedialyte bolus in the morning to see if that helps. So far it hasn't slowed down though. They checked her urine to make sure there wasn't any glucose in it and it came back fine. There was a trace of protein so we did another urine sample several days later to recheck the amount and right now there isn't enough protein to cause concern. Something to watch. 

The CAIR team ordered a Dexa Scan, which is a bone density scan, and we did that the following day. Overall I'm glad we made the trek to the clinic again. 

The next day we had a genetics appointment. This was our first visit to this clinic. We really liked the doctor. We talked about Gastroschisis, family history and anything that might be lurking in our DNA. She told us that they feel like there may be some "traits" that are linked to couples that have a baby with Gastroschisis, but they haven't found any genetic markers "yet." For couples that have already had a baby with this birth defect the chances of it happening again are raised 2.75% on top of the 1 in 1683 births (in 2009) based on the Texas Gastroschisis rate. This rate is higher than most other states in our country. This throws in the environmental factors and I wont get into that here. So you can do the math, but the chances are it could happen again and I know several families that hit the jackpot more than once on this. 

She ordered labs for a rare cholesterol depletion disorder. This genetic syndrome tends to lend itself to birth defects and certain characteristics that Caleigh has. She was doubtful that Caleigh has this, but wanted to do her due diligence.  So I'm not going to get into specifics until the tests come back in a few weeks... hopefully negative.

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We took the weekend off to run around the city and as you can see in the photo above Caleigh is in an umbrella stroller. This is a whole crazy story, but we have been working on getting a new manual wheelchair since March and insurance has denied it twice so far. We are working on the third appeal and I feel like crying about it everyday. I also feel like taking my child that is falling out of her current chair to the person making that denial and showing them the impact it is making. The new chair is 5-6K so I get it, but she has outgrown her old one. There is no repair or fix for a kid out growing her chair. Ok, off the soapbox. Hopefully, we will get this pushed through.

On Monday we had an appointment with Endocrinology. We are watching Caleigh's premature adrenarche and so far it isn't progressing to puberty. During this appointment we got the results of the Dexa scan. This is a full body scan that measures bone density. The reason behind checking Caleigh's bone density is her past nutritional deficiencies and the healed fracture that was found in her femur when she was in the NICU. Also, this is a common test for people with cerebral palsy because of the lack of daily weight baring. It turns out Caleigh's spine and hips are the worst of the measurements. She's always had the diagnosis of osteopenia but this moves her into osteoporosis zone. Essentially this means that fractures can and probably will happen. I find this pretty scary and it was something I had in the back of my mind but wasn't expecting so soon. I think the spine part really surprised me. I'm pretty bummed about it. This throws kinks into Caleigh's future hip surgery or any surgery she might have. It's more to think about and plan for. 

Our CAIR team ordered more lab tests like vitamin C and D. This is a good baseline and the chance to up Caleigh's supplements. The endocrinologist told us that if Caleigh starts puberty early it wouldn't be such a bad thing because the increased estrogen would actually help her bones. Other than that endocrinology is in the wait in watch mode. 

Early Tuesday morning we had our last appointment of the trip with the tube clinic. For the past six months we've had major issues with Caleigh's g-tube site. It's been terrible. Constant leaking around the stoma, skin breaking down, yeast and then random days where it was completely dry and fine like nothing happened. A lot of it has to do with intestinal motility, but as long as it was leaking or pouring stomach acid around the stoma there wasn't a chance for the weeping skin to heal. When we got to the clinic they were impressed with everything we were doing to try to heal the site and on that day the site looked good. I brought photos of the skin break down, oral thrush and pictures of her bed soaked in Elecare and stomach contents. They offered to hire me to do button care in the clinic, but I declined... Boston winters aren't for me. 

The nurse measured Caleigh's stoma at 1.5cm. Her button was an AMT Mini One Balloon Button 2.0cm 18 french. So we changed it to 1.7cm 18 french and switched to a Mickey Balloon Button. We've used the Mini One for years because it is much smaller, but the hospital only stocks Mickeys. I'm ok with it for right now. It's much larger than the Mini One, but the leaking has slowed down to a crawl and the tenderness is decreasing. We added some extra steps to our arsenal and if they continue to go well, I'll do a post on our routine. The clinic was a success and really made the whole trip. 

So as of now we are waiting on lab work to come back from genetics. Next week we will make a plan for upping vitamin supplements that will support bone density. Right now it looks like we will be going to Boston every six months, so twice a year as long as things stay great. Cramming all of these visits into one week is actually pretty brilliant. Clustering the care helps the different departments work together as a team. It's also very exhausting for the family. Overall though, this was an informative and helpful trip. 


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