I think the thread originally started by Chris D. and Laura K. is much too interesting to be continued on page 2---and hasn't yet been explored fully---so I think I'll continue it here.  Thanks to all who posted their thoughts, ideas, and personal experiences.  There were so many good questions and subtopics that came out of that last thread....where to start?  Since you started the original thread, Chris D., and---as Laura mentioned---continued with some great questions, maybe I'll just start where you left off with your last post, and try to address each question, one by one.

 

I can't remember if I have asked you this yet - what do you think Nick's levels are doing now? Do they seem stablized, or are you expecting undersuppression with the next set of levels? You mentioned you "have had very subtle dose incresaes, and (we) have stomped". I would love to hear more about that . I am assuming the "subtle" includes the recent dose reductions. When did you guys try the stomping? How did it work?

 

First, I should probably reiterate that Nick is on dexamethasone, not HC.  For treatment of CAH, dex is considered to be 80 times more potent than HC, and stays in the system much longer.  As such, I think dex is a drug that needs to be treated with much more care than HC, to begin with.  As a general rule of thumb, our endo tends to increase slowly and decrease slowly, usually no more than 10% of previous dose.  Because of the nature of the medication, I think a "slow and steady" approach would be more adviseable with dex, anyway, in comparison to HC, as part of using dex successfully is being able to very carefully control its use.

 

With that said, we have "stomped" twice, both times to combat undersuppression.  We have never "reverse stomped" for oversuppression.  You're right that the "subtle" approach includes the recent dose reductions.  Out of 6 reductions, the first was backed down by 5%, the remaining five, 10% each.  It's hard to explain why I feel that the subtle reductions were the correct approach to take with Nick, without getting into the whole history of why he became oversuppressed, to begin with.  I will say, however, that physical symptoms actually took about 2 /12 months to appear, after we had made some changes in his treatment---they didn't happen overnight, as was the case with Laura's daughter, Claire.  As such, I don't think a sudden and dramatic cut would have been warranted for us, anyway.  And, again, since Nick is so advanced in bone age, the danger of his pendulum swinging back towards undersuppression is a much bigger worry for us, than if he were to stay slightly oversuppressed, even if it's for a bit onger than we'd like.

 

Another reason why I didn't push more to "reverse stomp" is that, after each dose reduction, there was an initial reaction and slight waning of symptoms, so I fully expected that each decrease would "do the trick." I'm sure our doctor probably thought I was completely schizophrenic.  One minute, I'd be telling him, "Great news....it looks like we're losing the chipmunk cheeks!"  The next, I'd be saying, "Ooops....spoke too soon....looks like they are coming back!"  The part of the last 8 months that I find the most interesting is that I can pinpoint things that seemed to change, after almost every reduction.  Even more curious is that some changes were sustained, while others teetered on the edge, then reverted back.  Through all this, however, Nick's test results remained rock steady, giving us room to make continued decreases.  Nick's dose now is the lowest it's ever been, by far.  He has actually been both well controlled and under- suppressed, on much higher doses of medicine. 

 

In some ways, if we try to compare what is happening now with where he was at a completely different point in time, it doesn't seem to make much sense.  On the other hand, if we look at what's been happening, as a kind of continuum, the whole thing doesn't seem quite as illogical.  I guess that's why I've recently adopted the idea of looking at control and levels more from the point of view of momentum, rather than trying to compare and rationalize differences in numbers from vastly different times.  As I mentioned to you earlier, it seems to me that once you start leaning undersuppressed or oversuppressed, it becomes very difficult to stop the freight train.  Laura's idea of "stomping," I find very interesting, because it has the effect of stopping the "train" dead in its tracks.

 

Which brings me to the two times when we did "stomp," in order to reverse the momentum towards undersuppression.  One time, it was because an initial, more modest increase (similar to ones that we had utilized successfully in the past) had absolutely no effect on Nick's numbers, which had climbed to the highest levels we'd seen since starting treatment.  After no movement whatsoever with a 10% increase, our doctor resorted to "stomping" with an additional 33% bumpup. This is probably the only time that I have known him to approach the situation in a heavy-handed fashion.   Did I think it was warranted, given our situtation?  Yes.  And did it work? Again, the answer is Yes.

 

The other time that we "stomped" was a slightly different situation.  This was when we made the switch from HC, back to dexamethasone.  At the time, Nick's numbers (on HC) did not necessarily reflect horrible control, though they were starting to, again, make the shift.   Though the numbers had not yet caught up to the physical symptoms, it was obvious to me that Nick was starting to, very quickly, spin out of control.  He was a complete basket case, during this period, and symptoms of androgen overproduction---the most obvious being body odor---which had nicely gone away after being on dex for three months, started coming back in full force.  Because of an equivalency error made by the doctors who switched him to HC, his dose had been inadvertently halved.  So when we returned him back to a correct dose of dex, we effectively "stomped" by doubling the amount of meds he had been getting with the HC.  Did it have the effect of stopping the freight train?  In a nutshell, again, the answer is Yes.

 

As far as where I think Nick's levels are now, my prediction is that we will actually see his 17-KS move for the first time in 8 months (we test urine, not blood), BUT I do not expect it to move all the way to undersuppression.  Unlike before, this last, sixth reduction, seems to have produced more prolonged changes in Nick's physical symptoms.  Maybe it is wishful thinking, but I am thinking that we might even be headed into that state of grace where growth, numbers, and dose all seem to correlate.  We have been there before and I hope we are going there again.  Maybe, this time we'll even stay awhile.  We'll see, I guess.

 

I am also interested in your findings about growth during the first two years. I have asked myself the same question many times  - knowing both under and oversupppression are not desirable, is there one side we should lean to? I have read similiar research suggesting the first two years are absolutely critical for growth.....If I understand you correctly, from what you read . . . because the first two years of growth are very critical, the newborn diagnosed and treated from infancy should be cautious of oversuppression especially during those critical growth years....

 

That's what I have also concluded from reading those articles.  It was a while ago, and I have everything saved haphazardly in my computer, so will try to find the abstracts for you, so you can compare it to the ones that you've seen.  I find that my interpretation of things is constantly changing, so often it pays to reread things, at different points in time, anyway.

 

Assuming I follow you on that point, I am struggling trying to apply that to Jack. He was pretty oversuppressed between 18 months and 2 1/2 years, yet he stayed fairly close to the 50% height curve, and, his 2 and 3 year bone ages were interpreted as very close to chronological age. This leads me to wonder (1) Did we just get lucky so far with height? or (2) Do kids have different growth spurts regardless of the "curve" and did we oversuppress, hence miss, one of his?  (3) Could his bone age interpretations have been incorrect (although advanced bone age wouldn't be consistent with oversuppression - I don't think?) (4) Does his system maybe do better slightly oversuppressed since his height wasn't as affected as may be typical? I would love to hear your analysis.

 

Wow...these are such tough questions and I wish I had the answers for you. There are so many factors that go into determining how each child grows---many of them are not even related to CAH or androgen production---so I think it would be difficult for anyone to state exactly why Jack has grown in the way that he has, and what would have happened had he not been being treated for CAH.  I think, in some situations, it is absolutely clear that changes are related to the condition and medication levels.  Other times, the picture is a bit more fuzzy.  Given that Jack was oversuppressed for a good bit of this time, but still managed to grow well, I would have to say, yes, I think you're very lucky!

 

Research is only as good as the people doing it, and in reading only abstracts and studies, sometimes I find it difficult to evaluate stated conclusions, without understanding the validity of what is trying to be proven or disproved.  So.... I went to the library once and xeroxed some pages out of a textbook on pediatric endocrinology.   Not that I understand any more than would fit on a pinhead, but I did find some interesting tidbits on growth, that I will try to gather together to pass along.  They won't necessarily get you any closer to getting the answers that you're looking for, but I think you'll find them interesting, just the same.  As this post is already getting way too long, I'll have to pick that up at another time....sorry to keep you hanging.

 

Your "newest idea" is definitely something to ponder. Do you know more about why shorter lasting is advocated? I guess I have heard the conclusion "it doesn't negatively impact growth like longer acting steroids", but never the reasoning.

I have a TON to say on this subject.....OH NO!!!....so am glad to see that this topic has stirred some discussion.  But, again, it'll have to wait till another post, another time....got to go join the land of the living, at some point.  (Soon, though!!)