Carol M.Hi Sue,
I’m glad to hear that your daughter is feeling better. What you went through sounds like a nightmare.
Like you, I feel the term "adrenal crisis" is a little vague and hard to get a handle on. I asked my son’s endo for a more concrete definition and he gave me, "A condition where the lack of adrenal steroids (either cortisol or aldosterone) results in either inadequate circulation, electrolyte abnormalities, OR low blood sugar (hypoglycemia.)" Given that, this is my opinion re. the questions that you asked:
1) Did she actually have a crisis ? The doctors say it was not a crisis but hypoglycemia and any child could have gotten into one , CAH or not. Because she was vomitting and not had anything to eat since the previous day. In little ones the liver does not store enough sugar and that’s what sends them downhill real quick. There was no way to anticipate or prevent this.
Given that hypoglycemia is one of the indicators of adrenal crisis, I think the answer to the first question would actually be yes. I think the doctors are right that hypoglycemia can happen to anyone. But the point is that hypoglycemia is a particular danger, for someone with CAH. Since we know ahead of time that someone with CAH may be more prone to hypoglycemia during illness, we take extra precautions to prevent it from happening. That is partly what the need for stress doses is all about.
One of the major roles of cortisol in the body is to maintain normal blood sugar levels. Cortisol does this by triggering a process called gluconeogenesis. Gluconeogenesis is a way that the body can produce glucose from the fat stores in our bodies.
Glucose is essentially the brain’s fuel. When we are ill, the brain needs extra glucose to help handle the additional stresses of illness. One of the ways that it can get more glucose is by producing more cortisol, thus triggereing gluconeogenesis.
2) If indeed it was hypoglycemia, then how could it happen at 7am when we got back from the ER at 2 am after injecting IV fluids and dextrose. Should the low blood sugar levels at the ER been a red flag.
In my opinion, if your daughter already had a low blood sugar reading on the first trip to the hospital, that should have been a BIG red flag. I think they should have been aware that hypoglycemia was a particular danger for someone with CAH.
3) About our endo advising us not to give the shot, he says, the way cortisol works is by enabling the release of sugar stored in the liver into the blood stream. If there is no stored sugar to begin with, the the cortef cannot do much. Anyone else been in a similar situation.
To be blunt, I am shocked that an endo would advise you NOT to give the shot, knowing that you had just found your daughter unresponsive and almost unconscious. Those symptoms should have clued him to the fact that your daughter’s blood sugar levels were dangerously low, a sign of too little cortisol in someone with CAH. We are told to give a shot IMMEDIATELY, if we see those sypmtoms.
As for what he said about cortisol not being able to do much if there isn’t much sugar stored in the liver to begin with: I’m not sure that that is completely right. But don’t take my word for it...I’ll paste in some links for you to read below.
I believe what is stored in the liver is glycogen, which is a by-product of glucose. When the body needs energy, it first looks to use up glycogen. If there is no glycogen ( e.g. the person hasn’t eaten for a while), then the body looks for other sources of fuel. This is where gluconeogenesis comes in. Through the secretion of cortisol, the body is able to convert fat stores to glycogen, which is then converted back to glucose. Thus, the extra cortisol helps to maintain the blood sugar levels where they are supposed to be.
Anyway, I may not have all the details right, but I believe the gist of this is correct. If what your endo said was right, then, in a way, extra cortisol would not do any good anytime there was vomiting and diarrhea, or the individual had not eaten for awhile. So, then, what would be the purpose of giving extra cortisol in those situations? To me, what he says doesn’t make any sense.
It’s so scary to think what might have happened had we not caught her when we did.
Somewhere in one of these articles, it says that normal blood sugar levels are in the 60-120 mg/dl range. It also says that levels below 30 are considered life-threatening and can cause coma and brain damage. I don’t know if these are the same units in which they measured your daughter’s levels, but if they are, then I think you are right that it was an extremely close call. I’m glad she made it through all right.
Take care,
Carol
http://stress.about.com/library/weekly/aa012901a.htm
http://www.fred.net/slowup/hcauses.txt
http://www.niddk.nih.gov/health/diabetes/pubs/hypo/hypo.htm