An unwanted and hopefully temporary title : Adrenal insufficiency

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

Did I just use the word 'healthy'?

The question I get most often is “How is Mason doing?”.  This is a challenging question for me.  Mason, like most chILDren, is not a black or white case so to speak; he is always gray.  I could easily answer why he is doing well or not so well but that always would come with some sort of qualifying “but….”. That is why such a seemingly simple question is such a challenge for me. Not knowing how much to say or how in detail I should get with each individual often left me not answering very well; and for that I’m sorry.

Today, however, the question of “How is Mason doing?” brings on a whole new emotion/issue for me. The question doesn’t seem very challenging at all in fact. Mason is doing awesome.  Mason is eating well, drinking well, growing, learning, laughing; I could go on for days it seems like when I think of how well he is doing at the moment. Mason is OFF OF STEROIDS (as of October) and his body is finally functioning with a high enough cortisol level that we don’t have to live in constant worry of him going into shock. Mason is my little rock star! So what’s my issue with the question of how Mason is doing? This question makes me a nervous anxious wreck at times because I catch myself almost blurting out “he is doing great, he is really healthy”.

*Insert Gasp Here

*also please knock on any wood you have nearby

Healthy. Did I just call my son…that word? This simple word, a word that seems like a perfectly acceptable way to describe someone when asked how they are doing, is a very emotional word for me.  My sweet boy has a lung disease, he will always have a lung disease, so dare I call him healthy? I know this seems very silly to many of you and sometimes I would agree with you; but here is my reasoning. Although Mason is doing so very well I must always keep my guard up. It is my job as his Mom to protect and take care of him and that means I must always remember that he is my Mason chILD. Every single day when I tape the oxygen onto his sweet little cheeks I am reminded that no matter how well he is doing his lungs are not healthy; they are compromised so to speak. So while the world sees this amazingly happy, healthy young boy I have to see that too but also remain on the offense. I know how quickly that picture of health can change with one cold, one virus, one illness. Maybe I fear using the word will throw me off my game, make me slack or become complacent. So please know, Mason isn’t doing terrible just because I didn’t say he was healthy. Mason is doing phenomenal right now, I just want to make sure I can be even halfway phenomenal in keeping up with him and his health.

Thank you all for your many thoughts and prayers for Mason. He is in a truly great and blessed place right now! I pray he continues down the path he is on because I see nothing but bright things in my sweet boys future!


Here are some more recent photos of our sweet Mason!