What is wrong with my little boy?

[theglink]

My son is 3yrs. He has a rare chromosome disorder, seizure disorder, and a yet undiagnosed metabolic disorder. He is not diabetic but he does suffer from acute onset hypoglycemia. He has had a full endocrine work up, IEM workup, and urine organic acids. We have not found an answer yet. My son has been having more frequent and severe attacks. He has been hospitalized 5 times in the last 3 months. His LBS is not related to fasting. He will sleep 12 hours at night and his sugars remain steady. It is not reactive hypoglycemia. He eats a special formula (not yet able to eat solids) and his sugars remain level afterwards. His other significant hx includes: inability to regulate body temperature, acrocyanosis, partial complex myoclonic seizures, aortic insufficiency, hypertension, asthma and GER. He just had a PEG done on 8/6 so I can try and control his hypoglycemia without all the hospital admissions. Oh, and when I give him Glucagon IM, it barely makes a dent in his sugar level. Last episode he was 36, then after Glucagon he went to 55. Shouldn't it raise his levels more? Does this make sense to anybody? Is there a connection I am missing? I am hesitant to say what chromosome abnormality he has because my experiance has been that doctors prefer to lump all of his problems under that umbrella. Needless to say it is extremely rare (100 cases). His docs are wonderful but also stumped. My son can't be the only person in the world who has had this problem. If you can help at all it would be greatly appreciated. Thank you - Heather

[JDRF-Team-sgg]

I am not a physician, and am unfamiliar with your son's rare disorder, so of course I can only make suggestions for you to check out with his doctor. Since he is not on solid foods, I wonder if juices or sports drink are possible for him to digest. I have read that the sports drink that you can buy in the grocery store for athletes (something as simple as Gatorade, for example) that replenish electrolytes, are abosrbed the quickest of all drinks into the body to raise glucose levels during a severe low. These drinks are diluted for quick absorption and don't have the citric acids of natural fruit juices, so you may want to check out the possibility of using something like this after his glucagon shot to help elevate the blood sugars. I offer this warning though: frequently after a very severe low, the stomach is a little upset, so he may get a tummy ache after drinking anything. But if he can drink this kind of liquid, his sugars may stabilize without depleting the emergency stores in the liver.

Even after a glucagon shot, it can take a half hour or more for the sugar to rise significantly, so don't panic if he doesn't rise much in the first 15 minutes or so. I am assuming that his liver functions are working, and that he does respond to this stimulus provided by the glucagon. LRS's suggestion that he may not be having enough time in between lows to replenish the liver's emergency stores may be indeed the problem. Adding something like a sports drink may do the trick and may give his liver a break from the demands. If he can digest this, and if he is truly NOT a reactive hypoglycemic, this would not make his hypo situation worse by stimulating his pancreas to overwork.

The other possible explanation that comes to mind about why his glucose levels do not rise much after glucagon is that the liver may not respond properly to the stimulus provided by the glucagon. I do know that some type 1 diabetics do have limited response from the liver when they have lows, and there doesn't seem to be much in the way of help for those people other than keeping food and drink close by at all times, for they will not rise to the challenge of a low by producing much in the way of emergency sugars. I seem to be one of those people, and so lows are a threat I take very seriously. If I have a severe low in my sleep and need a glucagon shot, I do need to eat or drink something afterwards to stabilize me, for the glucagon itself won't do the trick for very long.

It is very important to try to catch those lows as soon as possible, so I would make sure to always have some glucagon or sports drink if his doctors say he can digest this with you at all times. Severe PROLONGED lows are dangerous and can cause damage to brain cells in a growning child. So immediate treatment is the key to his future health. Are you aware of all the possible symptoms to look out for in a young child? If he looks sleepy or yawns without reason or mentions that he feels cold, you probably should check his glucose, for these are all symptoms that sometimes are not mentioned in the diabetes books, but are easy to recognize in a young child. Irritability, unusual grumpiness, or sudden tears for no reason are also symptoms to look out for. Maybe knowing some symptoms to watch for will help catch these episodes before they get severe, and may help break the downward spiral you seem to be describing.

Maybe some other folks will jump in here and have other suggestions for you. We do wish you the very best.

[JDRF-Team-LRS]

TheGlink,
What an enormous challenge you're all facing.  Seems that you're doing all the right things and yet I understand your yearning for more answers & solutions to help your little guy.

We're not physicians here; we're largely folks with a lot of experience with Type 1 diabetes either as patients or parents of patients -- or both.  Maybe others will have somme additional suggestions for you.

As your son gets a bit older, there's a chance he'll be able to *detect* and communicate his falling blood sugar.  That's something that diabetics count on & when we lose that ability, it's frightening indeed.  

As you point out, I would expect more of a BG rise from glucagon than just getting to 55 -- tho' I'm not sure what interval of time you've measured, but if your son has had frequent low BGs, perhaps his liver is doing all it can ....  As I understand it, glucagon works by causing our liver to dump glucose.  Then, it takes some time for our liver to rebuild its stores so that it can perform that job again when/if needed.  Perhaps your little guy hasn't been able to rebuild his stores?

Can you treat his low BG with sugary "foods" (cake icing and other glucose forms)?

My heart goes out to you and I'll keep my ears open for other resources that might have some bearing.  I'm sure you'll get other responses, so do check back.

[theglink]

Thank you both for your comments. In response to some things mentioned:
I do keep drinks close by for his attacks. I use unflavored Pedialyte. It helps his sugars and does not interfere with his allergies. I wish my son was verbal because I would be able to detect things faster. I check his sugars regularly throughout the day. In his situation I rarely get advanced warning. He can be right in the middle of playing and walking around and then instantly throw up and scream. I will check his sugars again and they are instantly bottomed out. It just happenes so fast. We are hoping that his PEG will help us to control his sugars more effectively from now on. He gets very nauseous with LBS and can hardly toerate even looking at his bottle without gagging. I hope one day we will be able to tie all of his sx and hx together and discover how to treat the root problem and not just the results. Thank you.

[JDRF Team SGG]

Nausea can also be a normal sign of a low, although we don't necessarily get nauseated every time it happens. Gatorade may be easy on his digestive tract -- I would ask the doctor about it. It may not cause him to feel as nauseated as a formula even one such as Pedialyte, for it is deliberatley created for easy and quick digestion. Of course, I know nothing about his condition and just throw this out for discussion with his doctor. It may be a very bad idea.

As he grows older and more able to communicate, this may get easier for you. Keep your chin up, for treating the symptoms now may be the best that you can do, as frustrating as it seems right now. Don't worry overly much over odd behavior during the lows, for as soon as his glucose is back up, he feels fine, and many of us don't even remember the grumpiness or depression or odd behavior that we exibited that alarmed you. This is probably harder for you to see happen than for him to experience.