"In terms of an enzyme deficiency such as 21 hydroxylase, you are looking at a difference of a SW having 0-1% remaining enzyme activity compared to a SV having approximately 2% remaining enzyme activity. Not much difference at all."

Not sure what you mean above Rosalie...but aldosterone is a very potent hormone.  If your lacking a tiny speck maybe you’ll get by.  If your lacking it full stop, there is a hell of a huge difference in what happens when vomiting and diarhea which are typical symptoms of a crisis.   In total enzyme block in fact absolutely no ability to retain salt, no ability to retain water. 

When sick and vomiting, and losing fluid’s in diahorea and vomiting that in itself makes a huge difference.  In fact half the time when ill and  Ashley just get’s the cortisol and is just plied with replacement cortisol, and we were unable to get florinef down---recovery time from that illness takes way to long in my opinion.    We have had  Doctor’s that don’t seem to realise this, and he ends up being in hospital longer than a few days trying to get his electrolyte levels back to where they should be.  Any florinef in Sw’s body is quickly expended, and since he makes zero % aldosterone---and there is no way to replace it (and they seldom THINK replace it in a drip in the hospital;--except at birth)  it often means these longer stays.  They always seem to be more mithered about cortisol replacement and electrolytes half the time---but unfortunately this does not help to retain sodium right away.  In non CAH folks and SV aldosterone levels would shoot up in response to the illness.  This means adrenals can at least respond and more fluids are retained.  Whetreas when your losing what you put in orally where hormone is concerned, cannot replace it and do not replace it, sodium levels in fact ONLY start to come back up and fluid levels when the vomiting stops and you can get the hormone down them orally.  Why on earth they don’t use their skills and give straigh away in a drip is beyond me.  Probabaly because they have the same opinion as jim’s Doctor and not enough research into what should be happening for SW’s.  Would not surprise me at all.   

What disturbs me is that some Doctors see it as pretty much the same, when it can be quite life threatening really for the child or should I say "Quality of Life" threatening because should they make it through a bad crisis and have never been plied with the correct fluids to such a great degree, it could possibly lead to fit’s and brain damage no less.    Not to mention the fact that it is everything to be hydrated and nothing short of catastrophe for kids when they don’t see to this.  Because as much as they shove fluid and electrolytes in a drip, without the drug to help "retain" it being present in the blood serum some of these kids just pee it right out the other end and their body’s retain absolutely zilch...except for a tad from the extra cortef they are getting.  

I have seen the symptoms of dehydration in Ashley so many times and especially when ill it is not true really, but they don’t seem to fuss about it ...it seems to sit second place to getting his cortisol levels right when they could be getting BOTH right at the same time.  Simple.  When he is dehydrated  he loses the "thirst" mechanism that we all have normally, and this goes for many kids with SW---if you don’t feel thirsty..chances are you won’t drink or won’t want to drink either.  I literally have to ply him with drinks and he sips them and sometimes I get so furious with him..but he still will not drink.  Technically he should really be on a drip--but can these guys get their heads around how it affects the natural drive to drink fluids.  NOPE.  They think he can control this himself and that much more even if he does...that it will not be peed out the other end.  LOL!  Very frsutrating.  If they would think MORE about getting his Florinef med’s into him at those times and actually paid a little attention to detail, he would certainly recover so much more quickly has he does at times and would get his natural thrist back.  one of the symptoms of dehydration is losing that "thirst" instinct.  Imagine your body crying out for fluid and you don’t know about it.   That is how it is. 

I imagine that SV’s whilst losing cortisol, still have the ability to produce aldosterone and they also can up the anti in an emergency where that hormone is concerned, so  because their adrenals are still replacing aldosterone that has been expended in fluid loss...their pituitary’s sense the imbalances and can make the normal repairative response where fluids are concerned. 

Cortisol itself contains only 1-2% mineralocorticoid properties from what I read...is that what you meant above?  It’s leaving an SW child wide open for very little in the way of sodium and electrolyte retaining ability basically.  I don’t know why they do it in a crisis.

Anne-Marie