The big players; part 2 - endocrine/metabolics; tomato/tomahto

This is a mixed bag because he has two endocrine issues, well sort of. The first one, which is definitely endocrine, is hypothyroidism. Tomas has congenital hypothyroidism. meaning he failed his newborn screen and has been on replacement hormone (Synthroid) since NICU.

At his last appointment I told his enocrinologist that during an inpatient stay the rounding team had mentioned that when a child turns 3 they like to trial them off of Synthroid and see what happens. That sometimes it is not a true congenital issue and the children do just fine on there own. She said while that is true, Tomas' levels are normal on the Synthroid (meaning he is not getting too much), he is on a fairly large dose for someone his size, she increased it not that long ago because the smaller dose wasn't sufficent, and that it was discovered during his newborn screen - so truly was congenital. Ok, I tried, but it really isn't that big of a deal, just make sure he gets his meds and like I said his levels are stable.

This would've gone in the minor catagory if there wasn't another endocrine issue to write along with it. Well, maybe it is an endocrine issue and maybe it isn't. The metabolics team says it is; the endocrine team says it isn't. It is the bad boy Hypoglycemia (low blood sugar). Everyone else (all the other docs) think it is metabolic as well, but since I don't know where else to put it, here it stays. And until recently it was the single most urgent issue we dealt with.

If Tomas fasts for more than 4 hours his blood sugar starts to drop. That doesn't sound too bad, right? Most of us eat something at least every 4 hours, right? Nope we don't - we go to sleep, prayerfully for a lot longer than just 4 hours. Plus, if we had to skip a meal we could. Typically a toddler can go at least 18 hours without getting sick from not eating (hungry yes, sick no). Tomas can't. His body is either running out of sugar stores or unable to use them. He begins to make ketones as an alternative fuel source, but doesn't use those effectively either. They build up in his bloodstream, and since they are an acid, if enough time passes without intervention his blood ph changes and he goes into a state called metabolic acidosis. Without intervention this could be fatal.

Notice all the "without interventions" in there. For the first year and half of his life Tomas never fasted, he was always continuously fed. Remember he had his feeding tube since he was born. There was a brief 2 week period where I was able to nurse him (when he was a month old) but even then he was nursing through the night. So up until the spring of 2010 he had never gone more than approximately 2 hours without food.

One night that spring his feeding tube came out, the entire button dislodged from his stomach. I was exhausted and could not bear the the thought of driving to ER in the middle of the night. So I put in another tube, but not one that fed into his intestines - just his stomach. Because I didn't think his stomach could handle his regular formula I just ran pedialyte until morning. That was when we found out about the hypoglycemia, and I found out pedialyte doesn't have nearly as much sugar as one would think. By the time I got him to ER he was lethargic and unresponsive; a rag doll. His blood sugar was dangerously low, and thus began another road of test after test after test.

To date no specific reason has been found for the hypoglycemia. He has had 4 fasting studies done and during all of them his endocrine response (in the way of cortisol and insulin levels) have been perfect. Which is why endocrine insists it is not their problem. Also during all 4 he has had various levels of ketones in his blood and urine, sometime high enough to change his blood ph, which is why everyone thinks it is a metabolic condition. Not so says his metabolic doctor - because the ratios of blood/urine ketones are not consistant she thinks it is due to a hyperinsulin response - except - not once has his insulin level been high - so kind of hard to buy that theory.

After going round and round between the two clinics, after 2 hypoglycemic seizures, and after countless phone calls from me to the GI doctor (because he is in charge of Tomas' nutritional state) telling him that Tomas' blood sugar was really low again, that doctor had pretty much had it. He started Tomas on the gold standard for metabolic hypoglycemia - cornstarch.

Yup, your grandmother's Argo cornstarch is simply magic. Not only can it make a kick ass gravy, but it can keep a kid out of a coma. The reason the hypoglycemia was such a problem for me as his caregiver was that because of liver issues (another post) Tomas' TPN only ran for 17 hours, leaving a 7 hour gap without a steady stream of sugar. 7 is definitely more than 4. How to compensate? Well, mostly with G feeds (formula into his tummy). Unfortunately, most of the time he wasn't tolerating enough volume at a fast enough rate to keep his blood sugars stable, and some days he simply couldn't take anything in at all (another post). This left me or his nurse scrambling to get sugar into him somehow. Sometimes we'd try orange juice, but if he can't take formula orange juice usually didn't sit so well either, and other times pedialyte mixed with sugar. Finally, the GI doctor ordered IV sugar water (D10) for us to run during the TPN down time when his sugars started to drop. That worked great unless the central line wasn't working. And if you are on FB you know his central line stopped working a lot. Then it became urgent to get to the hospital and get a regular old IV started. So, after one too many hypoglycemic episodes the GI trumped the metabolics doctor and started the cornstarch therapy. It gets mixed in with his regular formula twice a day. Cornstarch is a very long chain carbohydrate and therefor takes a long time for your body to break down, causing a steady stream of carbs (aka - sugar) to be released. 

And the most amazing things happened. It worked - we were all so unsure of how his GI tract would handle having to digest something that required that much work. He still has days (and days and days) where he can't take anything in through his stomach, but the cornstarch has not made it any worse. His blood sugars went from being in the 40s at the 7 hour mark to being around 100. Just absolutely perfect. And another amazing thing happened - his liver enzymes normalized (another post), meaning that all the liver damage we were seeing, which we thought was from the TPN, was really from his body being in a metabolic crisis so often. Which brings me to the last miracle cornstarch brought about - GI and neurology are now convinced Tomas has a metabolic condition. I have no idea what the metabolics doctor would make of it as I have simply stopped going to her. But this was a major component in the "let's get a diagnosis" push that is now on the table, so for that I am very, very grateful.