Made of Gray

Hello Boston

Raising the Rare Child, Traveling w/ DisabilitesHolly D GrayComment

Caleigh's appointment at Boston Children's Hospital was at 12:30 on Thursday. We were told to expect a 4 hour appointment and we got that and then some. Which is a good thing. 

Eric and I have decided to transfer Caleigh's care to Boston. After the third hour with the very organized intestinal care team we knew that this was the place for Caleigh. As much as we connected with the surgeon in Pittsburgh, Boston was the complete package and that made all the difference. 

The traffic that we experienced in Boston was terrible, the weather isn't our cup of tea and the hospital is heavily under construction but we are here for Caleigh's care and that was the most important part of our decision.

Another big factor that we will have to deal with is that our insurance company is willing to pay for travel and lodging for Pittsburgh where they aren't willing to do the same for Boston. It's all about coding and department specifics. Pittsburgh would classify Caleigh's case as a transplant candidate because their transplant and intestinal rehab departments are grouped together and use the same doctors. Boston isn't willing to code her case that way which is probably accurate considering her health right now.  Boston's transplant and CAIR departments are separate. So if we are in Boston for long periods of time we wouldn't have that added monetary support. We will definitely be fine without it, but it would have made the deal sweeter.  I'm glad we aren't talking transplant one hundred percent. 

Boston had already requested Caleigh's medical records including the operative notes from her STEP procedure in 2008. They had called me prior to us coming up and asked a lot of questions. I sent them her formula recipe, medical history timeline and medication/allergy list. They knew so much about Caleigh before we even walked in that it made the appointment really easy. The questions we were asked were basically to fill in the small gaps. The whole five hour appointment we were rarely left in the room alone. There was always someone with us learning more about Caleigh or giving their opinion on how we should proceed. 

They were immediately concerned with Caleigh's weight, growth and overall nutrition. We know she is small and I've noticed it more over the past six months or so that she is skinny. She's been 38 pounds for over a year now, but still growing in height. They are worried about her vitamins, minerals and bone health. 

The surgeon that we met with was extremely knowledgable. He immediately pointed out the surface veins that are all over Caleigh's torso. This could be a sign of an old blood clot. This gets complicated, but it goes back to damage to her liver from her being on TPN for so long. As the doctors in Pittsburgh suggested this could be causing the veins inside her intestines. A portal vein issue. 

Boston doesn't believe that the veins are rupturing causing the bleeding episodes that Caleigh is having. They are guessing that there are a lot of sources. They have recent research that some kids that have had the STEP procedure actually bleed along the staple lines. They think this may be her case as well as ulcers, irritation etc. around the known strictures. 

The "Granulation Tissue" that Caleigh has inside her g-button stoma that is so painful isn't actually that. Granulation tissue is something that happens on the outside of the body that is painful. It's a different kind of tissue that is, we are told, a different kind of pain to painless. In Caleigh's case she actually has a severe irritation of the gastric mucosa, which is the inner lining of the stomach. This makes me cringe because our GI has been cauterizing this off during scopes which must be extremely painful for Caleigh. They are going to give us some tricks for stabilizing the g-button and the option of moving the stoma to a different location if all else fells. Again, this is rare and they really haven't seen it. 

Hello Boston via Caleigh's Corner

Hello Boston via Caleigh's Corner

MTHFR... we recently found out that both Eric and I are carriers of this gene. Caleigh got two strands. In simple terms it means that you may have clotting issues and that you can't break down folic acid in its inactive form. Eric and I both are on methylfolate, which is the broken down version of folic acid, and a B12 supplement. It's made a huge difference in our energy. They want Caleigh on the same. 

I also found out in the last year that I have the auto immune disease Hashimotos. This is basically my body attacking my thyroid. Everyone told me that my symptoms were caused by stress and it took 7 doctors and 4 years to figure this out and that is the short story. Since this is an auto immune disease it means that it is genetic. Somewhere, although I don't know where, the auto immune genes were passed on to me.

All of this to say that Boston is concerned that Caleigh has something metabolic/genetic possibly happening. Mostly because of her ongoing dumping syndrome that raises its hand every now and then. Her blood sugars get into the 300s and within an hour of her feed being done her glucose would drop to the 40s-50s causing Caleigh to feel terrible. Typical dumping syndrome involves stooling out, but Caleigh doesn't budge from her normal twice to three times a day routine. With the MTHFR, premature adrenarche and my auto immune disease they want to investigate her endocrine system to see what is going on.

What was impressive was the fact that they were very aware of MTHFR and how it would affect Caleigh during surgery. I'm pretty sure that was the hook that pulled us in. It's still a fairly new finding and most doctors don't know about it yet so this was wonderful and reassuring to hear from them. 

So the plan... Once we told them that we would like to transfer care to Boston they started processing everything. They ordered blood work for that afternoon and an abdominal ultrasound for the next morning. We will be planning on returning to Boston in the near future for about a week and a half for more tests. This will probably be inpatient so that we can easily add in hepatology (study of the liver, gallbladder, etc.), endocrine and genetics as well as the intestinal tests that she will need. Caleigh may also be put back on TPN at this time to get her body in the best nutritional condition possible before surgery. 

So yes, surgery is in the near future for Caleigh. They want to remove the strictures, lay out her intestines and do an intra operative scope to really see the inside surface of her intestines and proceed from there. It will basically be a very long exploratory surgery. 

They are very confident that Caleigh's food intolerances aren't really intolerances, but more of an anatomy issue. They feel like they can rehab her intestines and there was mention of attempting real food again after things are up and running again. We will have blood work back this coming week and we will have a plan of increasing calories and moving over to continuous feeds for more absorption. 

The ultrasound that they ordered was to see Caleigh's liver, veins and abdominal blood flow as well as veins for future central line access. Caleigh was nervous since she doesn't remember having an ultrasound. At the end I asked her if it was the easiest test ever and she said yes with a smile. I wish all medical tests could be like ultrasounds. We should have these results this week as well. 

Caleigh was in agreement with us about the Boston team and we happily finished off our visit with a trip to the aquarium which she absolutely loved. She shared so many smiles while we were there. As for Eric and I... we aren't on our short gut game like we used to be, but it is coming back fast enough. We're happy with our decision and glad that it was an easy and clear one to make. 


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