Adrenal Insufficiency and my son

In 2014, we made an appointment for our eleven year son Cody to see his pediatrician. We were concerned because he was not growing like a normal eleven year old boy. He had been at the zero percentile on the growth charts for about 2 years.  He weighed about 60 pounds and was 4’4” tall.  He was way behind most his friends. His pediatrician didn't think it was a reason for concern. He ordered a bone age scan, that came back at about a year and a half younger than he was.  At the time of the scan he was 11 ½ and the scan show he had the bones of a 10-year-old child.   Our pediatrician said he was just a slow grower and to check back with him, if there was no improvement, in 6 months.

We waited the 6 months until his birthday last year in February 2015 and returned to his normal doctor.  The doctor examined him and all was “normal” in the doctor’s eyes.  My son was not gaining weight nor was he growing, but the doctor said he was still just “delayed”.  His friends were all shooting up.  He was now a head shorter than almost every child his age.  We left there again not feeling right, but not sure where to go.

In March of 2015 during our spring break, the mother of my son’s best friend, who is a nurse practitioner pulled my wife aside after a sleepover with the boys. She told us that she was concerned about Cody.  She said “Something is not right with him.  His color is off and he should be growing and gaining weight, but he seems to be losing weight.”  She said she didn’t think it was just that he was slow to grow. She believed there was something else going on.  

We contacted Children’s Mercy to make an appointment, but they are so back logged in the endocrine department that we couldn’t didn’t get in for our first visit until almost July.

The doctors at Children’s Mercy were concerned right away by his size.  They scheduled a growth hormone test as soon as possible.  The testing was hard on a 12-year-old.  It is a 3-hour long IV blood draw. They were coming in constantly to give him medicine and then draw blood to see how is body was reacting to the testing.  

A week later they called us in for the results and didn’t really talk about his growth hormone issue, which they admitted he had, but talked to us about a Cortisol deficiency.   They let us know that day that his tests came back and showed an unusual result.   They weren’t sure the results were correct because the test said his body was producing zero cortisol.  They were baffled by it and didn’t fully trust the test.  They asked us to come back in and do a focused 1-hour test on his pituitary/adrenal glands to show what his results really were.  We rescheduled for the next possible appointment.

We went back in about two weeks. This time they did a similar IV blood draw test that lasted over an hour.  They did several other blood draws to check other levels including his thyroid.   He has been a trooper through all of it to this point.  In an 8 week period of time they did 10 different blood draws.

They called us back a few days later on a Friday and said his cortisol level still measured zero.  They told us to IMMEDIATELY schedule the first available MRI on his head (which is where the pituitary gland is located).  They let us know that we needed to rule out a tumor of some kind causing the issue.  We got a scan scheduled for the next day on Saturday morning  at 7 am.  They let us know as soon as the results came back they would call us.  Of course being a Saturday no doctor was available to examine the scan so we weren’t going to find out over the weekend.  Finally, Monday morning at 8 AM sharp they let us know that his scan was clear with the exception of a tiny cyst that should be of no consequence.

So now what.  They asked us to come in that day.  In an almost life or death fashion they explained the solu-cortef.  Solu-cortef is a shot you give in an emergency situation, much like an Epipen for people with food allergies.  They gave us some instruction on how to use it but no real instruction on when to use it.  They were very vague.  They gave us hydrocortisone (a pill) to take 3 times a day and kind of rushed us out the door, at least that how it felt.  We were thinking we were just coming to help him grow.  We were looking for answers to why he was so small.  We didn’t know he had a life threatening disease.

We did get him on growth hormones eventually and that is working well.  He’s grown about 2” since he started taking those, but more importantly he has gained about 20 pounds in weight which is mainly attributed to his cortisol being back in his body.  His pep is back, his happy face is back, his charm and wit, his color is back, and his life is mostly back.  We didn’t know it but he was slowly dying.  His body was slowly breaking down and without the love of his best friend’s mom and the love of God we could have lost him.  We couldn’t see the small changes that were happening to him every day as we were with him every day.  I hard to see someone change slowly when you are looking at them every day.

Adrenal Insufficiency is such a rough disorder.  Have you ever felt stressed out? I'm willing to bet Yes. Stress on the body effects on all of your systems. When you're under stress, your body responds by releasing a hormone called cortisol “the stress hormone”.   As with my son’s case of Secondary Adrenal Insufficiency when he gets “stressed out” he doesn’t have the means to pump more into his body because his body doesn’t make any.  So we have to monitor him and try to predict when he will and won’t be stressed out.  It’s hard to do.  We have a plan but that plan can change on any given day.  Cortisol regulates so much more than just stress.

Cortisol has many function we didn’t know about until this disease.  Cortisol functions to reduce inflammation in the body. So if he gets hurt we have to give him more medicine because his body won’t help him heal.  If he goes unchecked his immune system responding to persistent inflammation can lead to slew problems: an increased susceptibility to colds and other illnesses, an increased risk of cancer, the tendency to develop food allergies, an increased risk of an assortment of gastrointestinal issues, and possibly an increased risk of autoimmune disease.  Overall it regulates Metabolic response, Immune response, Bone and collagen, wound healing, acts as a diuretic by balancing electrolytes and water, Sleep, stress, and depression.  This is just about half of what cortisol does for our body.  Cortisol is an amazing hormone in our body.

Not knowing what can cause an episode is what is the hardest thing about this disease.  Just today he is feeling “low”.  Can’t get enough sleep, dragging, and just not feeling well.  We don’t know why it’s just one of those days.  We let him sleep in after his dose for the morning and he got a couple extras hours and we are going to head off to school.  That’s just a normal day.

Another normal day happened this past fall.  My wife, as loving as she is, found a way to tell my son’s story to Chevy.  She sent an email to a customer service rep and prayed it would happen.  I didn’t know what she was doing at the time but I found out later that our baseball team the Kansas City Royals does a program where they have some kids, on Sunday home games, run out on the field with the players during introductions.  They are called Junior Royals.  My wife unaware of how to get my son in on this, to give him some joy in his tough life, contacted the sponsor of the event Chevy through their contact us form on the Chevy website.  That email went to an hourly employee in Michigan.  She is paid to read every email that comes in and she was so touched by the story she passed it on to her manager.  We later found out that the email went to 12-15 different people trying to figure out how to make this happen.  Since they are just the sponsor not the organizer of the Junior Royals Program it was a difficult task.  They finally got in touch with the Royals but there was no room left.  It just wasn’t going to happen, but somehow they made it happen.

Several weeks went by and we thought nothing was going to happen, but one day we got a phone call from a general manager at Chevy telling us they were touched by his story and what he had been through this past summer.  They worked with the Royals to get him on the field as a Junior Royal for the last home game of the year.  He was very excited when we shared the news with him.  He prayed for a couple weeks up to the event that Alex Gordon, his favorite player, would be playing that day and he could run out on the field with him.

The day of the event arrived.  We got there to the stadium early for the event as directed by the Royals.  We waited in an area where several other Junior Royals players stood for about 45 minutes.  He and I kept checking my phone for the lineup to be released for the day.  I could see the excitement in his face.  Slugger, the Royals mascot, came by to say hi. Finally, we moved to a new spot.   They moved us to the Royals Press room to wait to go on the field.  Again we were in there for a while as the Royals people explained how it would work.  While we waited he and I discussed that it was cool to be in the same press room that Ned Yost sits at after every game.  Again they moved us to the tunnel between the locker room and the field.  On one side you could hear the crowd on the other Terrance Gore, a player for the Royals, ran by.  Still I could see my son was on cloud nine.  That day Alex Gordon was playing DH so we found out that he would be running out with Shortstop Alcides Escobar.  My son was excited and disappointed (only because he loves Alex so much) but overwhelmed too.  You see we didn’t stress dose (increase his medicine) our son that day because we were so new to the process we didn’t think this day was going to be hard on him.  He seemed like he was ok during it all.  He ran out on the field that day with a big smile on his face.  He talked with the player and got his autograph.  A few of the players waved to him that day but he just ran off the field when it was over.  The other kids stopped and got autographs from the other players and he just ran off.  I don’t think he could handle it all that day.  I am sure it was stressful for him to be on that field in front of a sold out stadium and meeting players he idolizes.   

Anyway we went back up to our seats and watched the game.  The Royals won that day and we went back to our car happy and excited about the day.  Our son got in the car and just after he put on his seat belt he laid his head down on the center arm rest in the back seat.  We didn’t know it but he was crashing.  He said he was just a little tired.  We tried talking to him but he just said I’ll sleep on the way home.  I knew at that time something wasn’t right we been to a lot of games and he’s never wanted to lay down after any game.  I gave him some of my left over soda from the game and we told him to take a dose of medicine.  We gave him some water and I started the car to get out of the stadium in case we needed to rush him to the hospital.  He was still not responding well.  We had his emergency shot of solu-cortef if we needed it but I felt like I needed out of the stadium parking lot.  We got on the road and about 15 minutes after his dosage he started coming back to normal.  He was starting to be ok again.  We gave him one more dose and another 12 or 20 minutes later it was like the day never happened.  He was fine again.  But we knew at that time he just had a crisis or event.

What we didn’t know or understand is anything can cause an event.  That day was the most exciting day in his life.  He talks about it still, but frankly, un-managed it could have killed him too.  When an adrenal patient crashes their body is literally shutting down and without treatment it can lead to death.

Since that day we look at life even more different then day 1 of his diagnosis.  What is he doing today, and how do we handle it?  There needs to be more education and awareness.  This may be a rare disease but there are people not diagnosed every day that need to know.  This picture embodies his spirit and his love but at the same time shows his new life with his emergency pack over his shoulder.

A couple weeks ago a different general manager at Chevy called us back to ask how our son Cody was doing.   They were so touched by his whole story that they still use him, his story of a junior Royal, and his disease in meetings as examples of how they can the community and reach out.  That got me thinking is there something more I could be doing?

I am not an expert I’m just a parent, a computer programmer by trade.  I’m just looking to get some awareness out there.  Trying to do whatever I can to help.  So I'll use this blog.  Not only to tell his story but maybe relieve some stress that we live through every day.