It's a life threatening disease

This is what my son has, Secondary Adrenal Insufficiency. It's a life threatening disease that no one understands and I can't explain. This article was written by researchers in Germany. It explains a lot but still leaves me wondering if there is more that can be done. It's a lot of medical jargon that is hard to read but still helpful to me.

The hard part for me and really anyone I explain this to is that we don't know when he will need more medicine and possibly go into a crisis. There is no normal dose. Well of course unless we put him in a bubble and not allow him to live his life.

It's the "Adjustment for stress, illness, life and surgery" that has us baffled. How does anyone know when the next time you will be stressed, excited, too happy, too sad, what day are you going to be ill next? How long is too long to play outside before it becomes a "prolonged exercise"? How long will it take for a teacher to be disciplining her class before it's too much "increased psychological stress" and he needs a dose or he might go in to a crisis?

This is the learning part. He takes a normal does everyday just to function daily. Just like your own body does you just don't know it. His baseline is 12.5 mg in the morning and 10 mg in the afternoon. This is just to be able to live day to day. You get similar dosages from your own Adrenal glands every morning noon and night. You just don't know it is happening. If your day gets crazy stressful your body gives you more. Cody's does not. Not only does it not adjust throughout the day it gives him nothing ever. He is broken completely.

Now let's add crazy, not crazy for a normal child but crazy for Cody. Let's say it's Tuesday and it is time for practice for Cody. Well that is categorized as prolonged physical activity so we bump his intake of 5 to 10 mg hydrocortisone. So depending on if that was enough, because there are many factors involved and it has everything to do with the fact that each patient is completely different he may be ok that night. Add to it a sleep over the night before and then we have the making of a crisis. We can do just what the doctor orders and still he goes in to a crisis. This was last week and it took him 3 days to recover and triple his medicine, which should be reserved for surgery and life-threatening illnesses. But I guess the doctor felt 3 days in crisis was life threatening.

Adrenal crisis—Acute adrenal crisis is a life-threatening condition, which will affect approximately 1 in 13 patients over the course of each year for many reasons. Despite established glucocorticoid replacement therapy, adrenal crisis remains one of the most common causes of death among patients with chronic adrenal cortical insufficiency. The symptoms of adrenal crisis include fatigue, nausea and vomiting, and hypotension and are thus non-specific, which can lead to misdiagnosis.

Quality of Life—In patients with adrenal cortical insufficiency, quality of life is significantly restricted compared to that of the healthy general population. However, this seems not to depend on the type of glucocorticoid taken or the frequency of hydrocortisone dosing. Instead, it appears that dose quantity and the non-physiological timing in administration of the glucocorticoid are responsible. So again it's about timing and knowing how much to give before you can even know that he is going to need his medicine.

The stress of it all for us is at times unbearable. I just want for my child to be able to be a kid. Can he go to a friends house? Not with out his emergency pack and a conversation with the family. Can he play in the back yard? Yes but not too long he may be pushing too hard toward an event if he has "too much fun". What about school and tests and gym class. Do we dose him there?

Well shit why does all this matter anyway. The doctor can just give us a plan. Why don't we just follow it? I mean don't the doctors know what to do? Secondary AI happens at a rate of 20 people per million (.00002%). In case you're wondering that is pretty rare. So no the doctor's don't completely know because there is not a single test available to do on a daily basis to check his levels, like a diabetic. We have to guess be cause every single patient is completely different, not a single one is the same. So we have to monitor him on a daily basis and plan a head. There are no last minute things in our house anymore. Want to go to your best friends sleep over we need to know 48 ours in advance to get him dosed properly. Honestly it is all a guess.

So what happens if we don't guess right? He goes in to a crisis (1 in 13 people go in to a crisis every year). Cody has had 2 in the past year. Some are mild and some are acute. Either way every crisis there is a chance we could lose our son. So if you want to know what keeps me up at night. This is what keep me up at night.

So again if you want to read the article feel free it is very interesting, but be prepared it is translated from German and written in medical speak so it's not super fun to read.

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3928535/