s' Check-ups

[Guest guest]

So, I figured it was about time to update on .

We went to Minneapolis last week for 's checkups with his endo and GI. Got a mixture of news. So, I'll start at the beginning.

Tuesday afternoon we saw his endocrine doctor. That went VERY well.

First, has grown an inch since his last visit with them, 6 months ago. This is WONDERFUL. He is not catching up with other kids his age, but he is growing at the same rate now, after 3 years of not growing. But with that lost 3 years, his height is 50th% for a child who is 8 years old. This being said, , Dr. and I are all very anxious to get his approval back from the state on starting on his growth hormone shots. With the fact that he will be on prednisone for the rest of his life, he will not be able to catch up like other kids might, and might be lucky to even reach his full height potential from where he is now - by age 18 or 20, he's expected at this point to be about 5' 4", when it should have been 5' 10". Anyway, I'm calling Genentech tomorrow to find out where things are with that.

We discussed his Diabetes Insipidus situation, and decided, after making him be NPO all night Tuesday night before his labs Wednesday morning, that currently has Partial Central Diabetes Insipidus. He CAN concentrate his urine, but only to a certain extent. It's not as bad as it was before, but it DOES disrupt his sleep (up 2-3 times per night to go to the bathroom and 4-5 glasses of water in the night) and his day in school. We decided to try him on just a dose of Desmopressin at bedtime to help him concentrate his urine over night, since his body can't as well as it should on it's own.

His thyroid numbers are a bit off still, tho better than they were when we checked them back in December. At that time, we switched him from 125 mcg of Levothyroxine a day to that dose every other day, and 112 mcg on the opposite days, because one dose is too big, the other too small. This is still giving him too much, so we've gone to 112 mcg 5 days a week, 125 2 days a week, and will recheck in a couple of months.

Dr. considers his Diabetes to be a non-issue at this point, with it having been so long since we've had a blood sugar issue of any kind. YIPPEE!!!!! So, all in all, Endocrine appointment was good.

Labs were done Wednesday morning, and I have to say, I was ecstatic with those. EVERY one of his CBC numbers were in range for the first time EVER. His LFT's were fabulous, Renal panel fabulous, pretty much everything was where it should be, with the exception of his thyroid and the ones to check for his DI returning. His growth hormone numbers and a few others were towards the extreme end one way or the other, but still in range. And those I expect because they are pituitary related and he's got that thing on it (micro-adenoma).

Wednesday afternoon, we went for his GI appointment with Dr. Sharp. Here's where the downs come in.

First, he walked in, announcing to the Fellow that saw us first that " had Primary Sclerosing Cholangitis, secondary to Langerhan's Cell Histiocytosis. This disease also caused the problems with his lungs, his thyroid, and his pituitary gland." He went on to say that this disease (LCH) increases the chances that will reject his liver. AND that, contrary to what the Hem/Onc doc at U of M (who is one of the nation's specialists on LCH) told me, LCH CAN come back/reappear after organ transplant, but would attack the tissues that it left alone the first time around. So his liver, lungs, thyroid (already dead anyway) and pituitary glad would likely be fine. But that still leaves his skin, bones, pancreas, and intestines open game. UGH. Just when I thought I didn't have to worry AS much!!! And the symptoms of LCH are so vague, and it's so difficult to chase, this worries me. This disease is

already quite rare. Solid organ involvement and multi-system involvement with this disease in children is even more rare. So it figures.

THEN he tells me that he absolutely does NOT want being treated with Growth Hormone. He said there is a "theoretical chance of increasing his risk for rejection of his liver". In my mind, there is a theoretical chance of him being hit by a meteor, too, but I don't keep him underground! There is a REAL risk of him dying in an automobile collision on our way to the store, but I still take him in the car. Children have become so mean to him this year at school about his height. When you're in 5th grade and the size of a 2nd grader, children are not nice!! We've gotten him to a pretty good place physically. And he has already had so much mental and emotional stress and trauma the last 4+ years, I just want him to have every benefit he can. So I talked to his endocrine doc, who is going to talk to his GI and try to find out where his concern is coming from and we'll go from there.

Other than that, he just wants us to get some more weight on him. Same thing we hear every time. I'm 5'7 and weigh 113 pounds. My husband was 135 pounds until he was 30 years old (at 5' 11). Emma was "puny" until about 12 years old, when she hit puberty. He is not going to be a big kid. Period. But, we continue to try.

We will return in 6 months to see GI again, along with Endocrine and Pulmonary.

So, that's pretty much it. At least, what I can think of right now. Will update on the rest of the family soon.

Ami

Ami - Migraines, FMS

mom to - 10 yrs - Double Lung Tx 2/26/2006, PSC - Tx 7/16/2007, Diabetes, Hypothyroid, GERD, ADHD, Osteopenia http://caringbridge.org/visit/seanfox

mom to Emma - 14 yrs - Migraines

stepmom to - 15 yrs - ADHD, ODD

~~~~~~~~~~~~ ~~~~~~~~~ ~~~~~~~~~ ~~~~~~~~~ ~~~~~