First, a little background for those of you who may be just
tuning in…..
We here at the Bush house have had an ongoing love/hate
relationship with oral steroid, in our case prednisolone. Before Mason was
given his diagnosis of NEHI he was believed to have pneumonia or other lung
conditions and was given oral steroids time and time again as treatment. Even after we had his diagnosis he had times
where he was in such respiratory distress our pulmonologist thought giving some
steroids in hopes of giving him some respiratory relief was necessary. There
were times it did seem to help him and he was only on them a few short days.
Then there were the times we have no idea if it did him any good and he would
be on them for so long he would then get “stuck” on the dreadful stuff. Now do
you understand the love/hate? That’s the short version of what Mason’s steroid
experience has been.
Secondly, lets have a little endocrinology lesson.
(I feel it’s my duty
to first say I am by no means qualified to give this lesson. This profession is
a LOT about numbers and I willingly admit I’m terrible with numbers. I know I’m
about to totally dumb this down. I just want to get enough information across
that we can get to talking about the stuff I’m good at; Mason. )
Your brain sends a signal to your adrenal glands telling
them to produce cortisol. The signal that is sent is the hormone ACTH. If you
don’t produce cortisol you receive oral steroids as a replacement. So when you
receive a lot of oral steroid and for an extended period of time your adrenal
glands can shut down or go to sleep because there is no need for them to
produce, you are giving the body all it needs. Cortisol is known as the “stress
hormone”. Your body needs cortisol to regulate many things and to respond to
stress. Without it you could go into shock.
Lastly, but definitely not least lets get to Mason.
Mason’s cortisol levels have always been on the lower end
when we test him. I’m not going to name names but lets just say we had some
doctors willing to sit on it and others not ok with it. Jonathan and I were not
ok with it and wanted to find out more and why. We have learned that when it
comes to the rare and unknown we want to, need to, push to know as much as
possible. Where better to go then to our right hand man, in this case a
hospital. We set up an appointment with the endocrinology department in Denver
and went for a second opinion. As always, Children’s Hospital Colorado
delivered. The doctor agreed his levels
were low and she too felt like we should look into it more. She wanted to do an
ACTH stimulation test. Basically they put in an IV and inject him with some
ACTH and then test him at 30 and 60 minutes to see how his adrenal glands
respond, do they stress respond and produce cortisol like they should. They
take a baseline cortisol level first thing before injecting the ACTH and Mason
was at a 6.1 which is not stellar, but acceptable. At 30 minutes the response
should be at least a 10 and Mason was at 13.1. Go Mason Go! At 60 minutes the
level should be 18 and Mason was only at a 13.9. Boo Mason Boo! In the doctors
notes she said “he still has evidence of iatrogenic adrenal insufficiency.”
Since he is insufficient we will have to stress dose him if he gets ill. He can
be stress dosed by oral steroid as long as he can keep it down, if he can’t
keep it down I have an injection that I can give him. In 3-4 months we will
repeat the ACTH test to see where he is. The hope is that this is all because
of the steroid and that his adrenal glands are not damaged and will eventually
wake up and produce at the level they should.
I want to address two things that I’m sure many of you are
thinking.
1. This would have never happened if the doctors
didn’t put him on steroid for no reason or for so long! (or something like
that). I don’t blame the doctors and I
don’t hate the medicine. This road that we have been on and continue to be on
with Mason isn’t straight and narrow. Mason has a rare disease; one with no
treatment, no cure and little known about it at all. Steroids have been known
to help pneumonia respiratory issues, asthma and other lung conditions. When Mason was prescribed oral steroid it was
because it was truly believed it could help him. It’s not always fun feeling
like your son is the guinea pig or that you are paving the road. The truth is
I’d much rather have some beautiful road with all the answers laid out for me.
That’s not reality though and so I’m not mad we sometimes pave the road I just
hope that we can look back and see some others go straight over bumps because
of pavement we laid. There is simply no point to look back and be mad.
2. Adrenal
insufficient sounds crappy and like a bad test result. However it isn’t
adrenal failure, now that would be a bad test result! I look at it as if he got
a C maybe even C+ on this test. His adrenal glands are functioning and that is
great news! So they aren’t functioning at the level they should be, we’ve all
not functioned at the level we should from time to time right? We will wait it
out and live life for the next few months and then do testing again. If the
results are still insufficient then we start to look into if something other
than the medication has caused this or if it’s more permanent than we thought.
For now I’ll make sure the adrenal glands know a C is unacceptable and there
will be some grounding, no TV for a week for sure!
As always, Thank you all SO much for your love and support
through all of this. Having Mason wrapped in your prayers and your strength is
so comforting. His smile and his spirit take all my worry away. Mason is such a
light and a blessing in my life, I know whatever the road ahead holds he will
pave it with greatness!
Love you all!
Carly
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