Sorry it took me a bit to respond here. Quite a busy weekend. Warning to everyone - this post is a long one!

Here is the address for the site that can help you calculate m2 medicine.ucsd.edu/cystinosis/bodysurf.html

To get your child’s meters squared (m2), also called body surface area by some, you will need to know height and weight. This site will calculate the m2 from those numbers.

To get your child’s mg/m2/day, take the total mg per day dose and divide it by the m2.    mg/m2/day is nice to work with because it allows you to compare dosing. You can compare your child to your child as he/she grows. (For example, my son has been on as low as 3 mg/m2 and as high as 21 mg/m2. Seeing this wide variation helped us realize that the liquid Cortef from a few years back was defective.) 

You can also compare your child to other children and to the treatment guidelines. Although the treatment of CAH varies widely (as I’m sure you’ve discovered from this board), there are some guidelines about the best mg/m2 ratio. I don’t have the articles in front of me anymore so I hope someone will correct me if I am remembering wrong. When my son was first diagnosed almost 5 years ago, I read the optimal dose was between 15mg/m2/day and 21 mg/m2/day. I believe they have since lowered it a bit. But I can’t confirm that. Anyone else?

A note on the liquid Cortef - I think Anne-Marie and Sally mentioned this also - several years ago, Pharmacia Upjohn produced an orange liquid Cortef. The suspension agent was defective and it was ultimately recalled. The active ingredient (hydrocortisone) was sticking to the bottle sides and was nearly impossible to shake and re-suspend. (Even Upjohn recommended shaking at least 20 minutes prior to each dose - right!). Since then, as far as I know, they have stopped mass producing a liquid Cortef. However, a fair number of people have their local pharmacist make a batch of liquid Cortef suspension especially for them. From postings on the board, it seems people are pleased with the local pharmacy made kind.

FYI: I think one of the posts in this thread was asking about 17 OHP levels. What is high etc. This is the range that I was given by my son’s ped endo.  "normal range" for 17 OHP is 3-90 ng/dl. Two comments on that measurement. First, be sure you are comparing ng/dl to ng/dl. Some labs return the numbers in a different format. Also, it seems to me there are many different thoughts on what is the "ideal range" for a CAH patient. I think Maria New looks for anything less than 1000. My son’s endo likes to see the numbers much lower.  (We seem to be shooting for more about the 300 range). Although my son tends to stay oversuppressed quite easily.

I thought it was very interesting that your daughter’s levels went up after you triple dosed her. It made me think of a post many months (years ?) ago where someone talked about their child’s endo liking to kind of jump start a dose change to sort of shock the adrenals. The dr. said something about how the adrenals don’t like to be sneaked up on. I wonder if (and this is just a guess from a non-medical person whose only claim to expertise is many nights analyzing and worrying about her son’s over/under suppression vacillation)  . . . I wonder if prior to the tripling, you daughter was producing some cortisol, this combined with her regular dose kept her levels a little high, but not extremely so. Then, when she was given the triple dose, her adrenals shut down and she was relying solely on the hydrocortisone dose, which maybe wasn’t enough to keep her levels down.

I am making that guess because of a conversation I had with my son’s previous endo and observing changes in my son’s levels and doses. When my son was coming off of the defective liquid, he was doing very well on a low dose. When we switched him to the Cortef pill he became very oversuppressed and it took him a long time to come back. It seemed like once we oversuppressed him, his adrenal function shut down and it took a much higher dose to maintain him. Once his adrenals kicked in again, we could lower the dose and he would do better. In fact, his previous endo said our goal was to completely suppress his adrenal function because it was easier to calculate/titrate a complete replacement dose, then to figure out what he was making already and replace the necessary remainder.

This kind of ties into Kristin’s question as well - why her child’s symptoms seem to be getting worse when treatment first begins. I remember other parents with children diagnosed in their toddler/childhood years saying the same thing. (Carol once posted a very good message on this topic, saying it is actually a very dangerous time just as you start treatment).  It would make sense that the symptoms would get worse as you start treatment if the treatment is supressing the partial adrenal function that was getting the child along prior to treatment.

One last thing on Elizabeth’s question about tripling then finding higher levels. Were all other factors the same during the draw? e.g. was it at the same time of day, same time frame before or after the last dose etc. Also, stress can raise 17 OHP levels. Does you dr. evaluate other blood levels as well?

Sorry for this extremely long post, but I hope it helps.

-Chris