We met with Hannah’s liver doctor today, Dr. Carter at TCH. We actually had not seen her since before Hannah was diagnosed. I brought her up to date on everything that has been going on, including how Dr. V was concerned about some of her elevated liver function levels from as far back as Sept of 2008, which were still a bit high at the NIH. What they mean, we don’t know yet, but Hannah had another set of blood work today so she can review it.
My main concern is that Hannah has been on the Cerezyme treatments for one year (next week), and her spleen and liver should have shrunk back to normal size by now. They definitely aren’t. Dr. V, her GI doc, is actually concerned that it seems a bit bigger. This along with the elevated numbers makes this something that needs to be looked into.
So we will be doing the liver function tests (blood work), and we are going to schedule a abdominal ultrasound downtown at TCH to get measurements to see where we are at. Then, depending on the results of these tests along with the advise from Dr. Goker-Alpan at NIH, they may do a liver biopsy to see if there is any liver disease that may not be common with GD. Apparently there are a few rare cases of “liver scarring” with GD type 1, so that is in the back of her mind. The thing that sucks with the liver biopsy is that what would normally be a quick outpatient procedure would be an overnight stay downtown at the hospital to be monitored because 1] of her lower platelets and 2] because of the quality of platelets (which may not be as functionable as normal platelets).
We then talked about Hannah’s nutrition and how we have seen regressing in finger feeding. I wanted to know “what to do” because we have been just winging it lately with milk, pediasure, and stage 2 baby foods in the bottle. She brought in her dietician, and her dietician recommended a product called “Compleat Pediatric,” which is basically all the required food elements (proteins, fats, vitamins, etc) pureed together using natural foods.
She warned me that it is mostly used for g-tube feedings (feeding tubes), but she does have some patients that can drink it orally. Apparently, it doesn’t taste that great, but since Hannah is used to stage 2 meats, veggies, and fruits mixed together, we would give it a shot.
We came home, and I put together her first bottle of 4 oz whole milk, 4 oz Compleat Pediatric, and one stage 2 fruit (pears and pineapple). She downed the entire bottle! Not only that, she drank most of her second attempt with it tonight (again 4 oz milk, 4 oz CP, and stage 2 fruit). I’m going to email her in a couple of days and take her up on her office to get a rx for this.
Even though we have had to “give up” on feeding her normal foods, there is a sense of comfort knowing that we are a step closer into getting her the nutrition she needs. After talking to another of her doctors, it is time to stop her feeding therapy now and just move on and accept this is where we are at this time. I guess there is a sense of comfort knowing that we aren’t needing a feeding tube at this point, but there is such a sense of loss/grief trying to accept this.
How frustrating. It was a major struggle for us to get Connor to eat too, and we finally ended up with a g-tube placement. I felt like him not eating typical foods was not only something that would set him apart from the other children but was also a direct reflection on my ability as a mother. Getting a g-tube was one of the most difficult things we’ve had to do with him so far, but now we are 100 percent happy we did it, as he has filled out considerably and has so much more strength to develop other skills. It’s also taken a lot of the stress and worry out of eating, allowing him to enjoy food instead of having it be a chore.
I’m glad that Hannah isn’t there yet as far as a g-tube goes (and hopefully won’t get there) but know that if she does eventually need one they can really make a tremendous difference and allow you both to focus on other things.
I hope the tests go well– how nervewracking. I’ll keep Hannah in my prayers as always.
~Jess
Don’t worry to much with the whole food thing. My niece Savannah has type 2 and 3 and she didn’t start eating regular food till she was about 3 1/2 to 4 and we still had to cut it up very small. But now that she is 6, she eats whatever all the other kids eat, even though we do cut up some of it but that is because she trys to take big bites. It is a slow process, as with anything these children do. Never give up, just be patient. When it happens, It is feels even better than it would if they was right on time!
Drinking from a bottle is still a long way from a G-tube! In many ways drinking fluid is actually harder than eating solids. I probably should’ve taken Bertrand’s transition to an all liquid diet (his KetoCal formula is for tube fed kids too) harder than I did, but I was so exhausted from months of struggling to get him to eat “solids” (yogurt and baby food). Now I put all my energy into trying to get him to learn how to communicate–which is a lot more fulfilling. You’ll come up with some great alternatives to fill the time saved from fighting the food wars! Hannah **and you** will be much happier for it. 🙂
.-= Cristina´s last blog ..We’re alive =-.