STIM Test, Twitter Famous, and a Surprise Diagnosis Change!

So, here's the thing...

Sometimes life gets crazy and some stuff has to take a backseat to surviving. For our family, the past year or so has been about surviving and Jonah's treatment was one of the things that was put on hold. His rate of growth has slowed way down again and he has dropped back down from the 12th percentile to the 7th.  He has grown in a million other ways this year, however. Jonah has "graduated" out of Special Ed services at school, attended Robotics Camp, has made his first best friend, joined the Coding Club and is learning to play the trombone.  His teacher says he is doing very well both academically and socially.  His size appears to be a non-issue for him at the moment and he has definitely enjoyed taking a break from his nightly injections.  

  Super cute, right?!







Awwwwww!! So cute!


What were we talking about?



Oh, yeah...

Since my last post, we moved from Tennessee to Maryland and have been transitioning out of the ministry.  A few months ago, we transferred Jonah's records from Vanderbilt Children's Hospital to a pediatric endocrinologist at Children's National Medical Center in Washington DC.  She went through his records and was surprised to find that he never did a growth hormone stimulation test.  We did lots of other testing, but not a STIM test!  She ordered a STIM test for one day during Spring Break, and Jonah and I got up while it was still dark to get down to the children's hospital by 7:30am.

Children's National Medical Center

Let me first say that I was super unhappy about this whole thing.  Jonah has had a sedated MRI of his pituitary, multiple blood tests per year, regular bone age scans and check-ups with the endocrinologist every four months.  I was positive that this new test, which involved an intramuscular injection, an IV, and four hours of not being able to get out of bed to pee, would yield little new information. It turned out to be a game changer!

We were taken back to a small room around 8am and a nurse started an IV on a very nervous Jonah who was concentrating on looking brave and fighting tears. The nurse drew a baseline blood sample and then administered an intramuscular injection of Glucagon, which is a hormone that raises the blood sugar and makes your child forget that he's supposed to be looking brave. If you are preparing for this test with your own child, know that when the nurse says that this shot may cause vomiting, what she means is that this shot ABSOLUTELY WILL  cause vomiting.  Blood was drawn from the IV every half hour and another medicine, called Arginine, was administered about an hour and a half into the test.  Arginine is a an amino acid that's supposed to stimulate the secretion of growth hormones.  Supposed to.  The whole ordeal took about four hours and caused drowsiness, nausea, vomiting, blood sugar crashes and super low blood pressure.  Let me tell you, it was awesome.  At least we had How To Train Your Dragon on Netflix, the soundtrack to Into the Woods on my phone, and various Percy Jackson books.

SPEAKING OF PERCY JACKSON!!!!  The highlight of our otherwise long and stressful morning was the three hours that we were totally Twitter-famous!  Jonah is a completely obsessive Rick Riordan fan.  He has read all of the Percy Jackson books, the Kane Chronicles, the Heroes of Olympus Series, and is eagerly awaiting the second Magnus Chase book and the release of the new Trials of Apollo series. He has read all of the supplemental books about Greek gods and heroes over and over.  He brought Riordan's giant Book of Greek Heroes with him for the test and spent a couple hours either giggling to himself or reading the funny parts out loud to the nurse and me.  I took a picture of him reading and tweeted it Rick Riordan who almost immediately retweeted it.

 You guys, my phone went crazy for like an hour.  Our tweet has been retweeted sixty-something times and liked 510 times.  Rick Riordan's tweet of our tweet (I've never used the word "tweet" this many times in my whole life) has been liked 202 times and retweeted about thirty.  My phone buzzed every time either of them were liked or shared.  It was madness, but Jonah was psyched to have made contact with his favorite person on the planet.

Our new endocrinologist called yesterday with results.  Four years after being diagnosed with ISS, we received an official diagnosis of Growth Hormone Deficiency.  The endocrine nurse will be working this week on getting all of our stuff resubmitted to our insurance in an effort to appeal their rejection of Jonah's treatment. The doctor expects that it will be accepted this time around in light of this new information.


I know that you lost almost two full years of sleep awaiting this post. :)  I promise not to go this long again, especially since there will soon be exciting updates about insurance and beginning treatments again.  I started writing to keep a record of this journey for Jonah to see when he gets older, but I have gotten lots of messages from other growth disorder parents who are searching the internet for stories from other families.  So, share your story!!!!

A Pep Talk About Side Effects

One of the things that made the Idiopathic Short Stature diagnosis so difficult for me was feeling like it was "only" a cosmetic issue, and medication (especially an injection!! ew!!!) seemed like a dramatic intervention to correct a cosmetic problem.  Of course, our insurance company reinforced the idea that ISS is really nothing to an otherwise healthy child.  We discussed it at length with our family and doctors, and concluded that, while ISS kids usually have no other symptoms, it IS a medical issue.  ISS doesn't mean that nothing is wrong.  It means that something's wrong, they just don't know what.  The body doesn't use the GH it produces and the resulting growth failure is significant.  Jonah's growth chart showed that he wasn't just a mini-kid, but rather a kid falling farther and father off the bottom of the chart.  In terms of percentiles, he was getting shorter!  He started in the 25th percentile, dropped to the 12th and so on until he was below the first. Now he is back to the 12th percentile and holding steady.  He is still a mini-kid, but he is a kid whose growth rate is consistent instead of getting slower.

Parents whose kids receive this diagnosis are often and appropriately concerned about side effects.  Jonah was a couple months into his treatment when he randomly decided to bite some kid in his kindergarten.  Just bit him. WTH, kid?!  Don't bite your friends!! 

I immediately pictured some roid-ragey baseball player and called the doctor.  Nope. This is not a known side effect.  In fact, Norditropin side effects are rare in otherwise healthy kids and usually very mild.  They include headaches, stiffness, redness at the injection site, etc.   You can check out the Norditropin safety information here! I also learned that growth hormone therapy has been studied for over forty years, focusing on thousands of children and following them into adulthood.  Every time we see the endocrinologist at Vanderbilt, he asks Jonah about headaches, joint pain, whether or not the injection hurts and a few other questions.  They check his vitals after the weigh and measure him.  Every other visit includes blood tests and a bone age scan.  Everything is carefully monitored.

   

 Most recently, we had a nurse practitioner check his spinal alignment and think she detected signs of kyphosis, an abnormal curvature of the spine.  Some quick googling indicated that growth hormone therapy can worsen an already existing curvature of the spine, but the endocrinologist assured me that it does not cause it.  Further examination showed that his spine is perfectly fine, but the fear of side effects came crashing back into my consciousness in that moment.

Checking out his bone age scan

I know that I sound like a commercial for Norditropin and I'm just itching to get you to stick your kid.  I just want you to know that it's safe and well-studied.  Obviously, I'm not a doctor.  I'm not even a very good blogger! (One post in 2013?! Really?!)  I'm just a mother of an ISS kid who KNOWS that roughly 9 zillion people felt it necessary to tell me that I am putting my kid "at risk" over something as "inconsequential" as his height.  If you are a parent, then this phenomenon of people feeling free to share their opinions with you is nothing new!  Share away, busy-bodies of the world!!  We know that our kids deserve the best care and we don't give a crap what you think!! So There!! 

A Few Little Hiccups

We headed back to the endocrinologist at Vanderbilt for Jonah's regular check-up last month.  We have gone every four months for the past 21 months or so.  It's hard to believe that it has been almost two years already!  Only, what?  Ten more years.  Man.  We have hit a few bumps in the road the past few months with his treatment and results.  First, Jonah has randomly re-scared himself (re-scared? re-frightened? re-freaked?) of the injection.  A few months ago I was able to give him his shot while he happily played Angry Birds or read a book.  The Thanksgiving before last, he called the entire extended family in to the living room  to watch him get a shot in his butt to show off how brave he was.  It was a weird moment, and he was super proud.  Sometime during this spring, he started trying to wiggle away again.  He would cry and struggle to stay still and tell us that it was going to hurt.  He wasn't comforted when it was over and hadn't been painful. He was really stressing himself out, and it was so confusing.  For a few days, we were back to having to hold him down like we did in the very beginning.  The endocrinologist is stumped.

I should add that, while the shots are usually painless (I tried one on myself before we began treating him), there have been some instances over the past 21 months when something unusual has caused them to sting.  For example, if he gets nervous and tightens the muscles in his butt cheeks, it will hurt a little.  Once, he moved just as I hit the button and the fluid gathered into a little painful lump just under the skin.  There was also the night that the hair-thin needle mysteriously bent and I didn't notice before giving him the injection.  That was a rough one.  These problems are EXTREMELY rare, but still suck.  We went back to deep breaths and ice packs, and they helped a little bit.  Jonah refuses to try getting the shots in a different location, so we just alternate between butt cheeks.

I have no idea why, but one night I taught him the "bum bum buuuuuuuummmm" part to Neil Diamond's Sweet Caroline.  They should teach Neil Diamond lyrics in Medical School.  Seriously.  Who doesn't feel better after a few choruses of Sweet Caroline?! It's a scientifically proven fact that Neil Diamond makes everything better.  It's enough to Crackle your Rosie.

So now, Jonah lays down across my lap and it goes like this:

Me: Sweet Caroliiiiiine,
Jonah: Bum Bum Buuuuuum!!!
Me: Good times never seemed so gooooood
Jonah: So Good! So Good! (Deep Breath)
Me: I've been inclined
Jonah: Bum bum buuuum!! <--- inject during bum's and hold.
Me: To believe they never would.
Remove Needle
Again, I have no idea, but this is bizarrely comforting and Jonah requests to do it on nights I try to just count to three.  Thank YOU, Mr. Neil Diamond.  To show my gratitude, I promise not to tell anybody that your real middle name is "Leslie."

The other little hitch is that Jonah's rate of growth has slowed way down.  He was growing at a rate of 12 centimeters per year, and now he's down to about 6.  It's still much better than the less than two he was doing before treatment began, but it was kind of a bummer to see.  He has dropped down from the 12th to the 11th percentile.  He also weighs a pound less than he did last year at this time.  The doctor suspects that his other medication is killing his appetite, and his lack of weight gain is slowing his growth.

After an exam and some blood work, it was determined that Jonah is receiving a high dose of synthetic growth hormone for his weight but it is not producing the results "they" expected.  The doctor assures us that we still have TONS of time, and that if this trend continues we can increase his dose.  Right now, we're just trying to get him to eat a little more and celebrating the continued progress.

   <---first day of 1st grade

                   end of 1st grade ---->

One Year Later!

Next week will mark one year since Jonah received his first Norditropin pen in the mail and started treatment!  Look at this!  It's a giant apple juice bottle filled with discarded needles, spent pens and dirty alcohol swabs.  Gross, I know, but jeez! Look how many needles are in there!  

Before we threw it away (don't even get me started on what you're allowed to just throw in the trash in Tennessee), Jonah noticed this little sticker floating around in the bottle.  We don't remember how it got in there, but I like it.  It's a little treasure in with all the medicine and needles and scraps of swabs.  Just a little note to remember the reward at the end of the journey! 

  It has been a crazy year of x-rays, blood tests, injections, finishing kindergarten and lots of growing up!  For his birthday last month, Jonah received age-appropriate, size six jeans!  They need to be rolled a little (a lot) at the bottom, but at least they don't look like the legs on one of those inflatable dancing carwash guys.  

While Jonah's progress can't be overstated, I just noticed that the shirt he wore to the endocrinologist yesterday is the same shirt he is wearing in the picture from last year's blog post about his fifth birthday.  LOL! I guess it's just a reminder that we have a long way to go even though we have already come so far.  

How about a quick update first?  Jonah is up to 43 inches and is in the 11th percentile in height for his age.  This is completely unbelievable to me. I so clearly remember the day we were sitting in the pediatricians's office learning that he had dropped from the 25th to the 12th percentile.  It's amazing that in one year, he has gone from below the first to the eleventh.  It's so unbelievable that you are probably sitting there refusing to believe it.  I don't blame you.  Here's the graph.  You can't argue with graphs!  I love graphs.  

We also learned that his rate of growth has more than doubled.  He was growing about 5cm per year and now he is growing 12cm (ABOUT FIVE INCHES!!) per year.  The doctor doesn't expect him to be able to maintain that rate, but he described it as "quite awesome" for right now.  Quite awesome.  

We also did another bone age scan that involved a simple x-ray of his hand.  

  It turns out that his bone age is a year and a half behind his real age.  So, he has the bones of a four and a half year old.  When we started, his bone age was only a year behind.  This is actually good news for us!  Since his bone age is a year and a half behind his real age, he will continue to grow for a year and a half after boys his age would normally stop growing.

 As an aside, nothing makes people peg you as a "helicopter parent" faster than knocking down an x-ray tech to take a phone picture of your kid's scan.  Yelling, "It's okay! I have a blog!" doesn't help.

Any lingering questions I had about whether or not we're doing the right thing by treating Jonah's ISS with growth hormones have been wiped out by just one year of school.  The other kids haven't been intentionally mean, but they have certainly noticed that he is different and have been quick to remind him.  For example, there was a boy in Jonah's kindergarten class whom he absolutely adored.  He talked about this kid every single day after school like they were absolutely the best of friends, but Jonah said that they didn't play together at recess.  When I asked why not, he said that the kid had informed him that he was too little and that he would get hurt playing with the boys. 

We also had some discussion with Jonah's teachers and pediatrician about whether or not it would be in his best interest to put him back in kindergarten after he had already started first grade.  Jonah's teacher described him as one of the top three students in her class of twenty, but still recommended that we consider placing him back in kindergarten because of his size and age.  His guidance counselor and pediatrician agreed.  I called the Magic Foundation, and one of Magic's co-founders called me and assured me that short stature is no reason to hold Jonah back when he is obviously doing just fine.  We discussed simple accommodations the school could make (like making sure Jonah has a chair that allows his feet to touch the floor) and how to encourage them to teach inclusiveness to students who may reject him because of his size. Yesterday, Jonah's pediatric endocrinologist agreed that Jonah should be allowed to continue normally in school.  

So, that's where we are now.  A year into his journey, Jonah is rocking this whole thing.  He is growing in stature and confidence and joy!!  Stay tuned! He has nowhere to go but up!! 

The World's Most Wonderful Graph

Okay, I know this picture is pretty bad.  I'm not going to tell you how long it took me to get a decent picture of this graph with my phone.  I'm not going to tell you how many times I carried it from room to room looking for the best light.  I'm not going to point out that I accidentally took a picture of my own shadow.  I won't tell you how long it took me to edit it online or how stupid it is to have to edit a picture of black dots on white paper in the first place.  All I will tell you is that you HAVE to see this!!!

The first two dots indicate Jonah's height percentile (according to the CDC) before treatment.  It was below the first percentile.  Awesome, right?  You can also see that the line flattens out a little bit between the second and third dot, indicating that his rate of growth was actually slowing down.  If his rate of growth had continued to slow, he would have just kept falling farther and farther away from the curve until he wound up having to wear Gymboree clothes to post-college job interviews.  The third dot was the last time he was measured before starting growth hormone treatment, then WHA-BAM!!!

  The fourth dot jumps enthusiastically toward the bottom line of the graph!  Unfortunately, the heartless slobs at the CDC don't think that a kid deserves a line until they are in the third percentile, so it still appears that Jonah's height is below the curve, but it's not!  I promise!! Jonah is firmly in the second percentile.  That means that in a room full of 100 kids his age, there could be two kids who don't want to pat him on the head like the kids at school.  Those kids drive me crazy.   According to this, my favorite graph in the world, Jonah was roughly 37 inches when we started the shots in October and was 40 inches when he was last measured by the endocrinologist in February.  This graph has earned a place of honor in the long-neglected baby book.

My favorite part of the whole process is how FAST it's happening!  Jonah has gone up three shoe sizes this month!  At our last appointment, I addressed my concern that Jonah was just growing too fast and that maybe we need to reduce his dose.  The doctor asked Jonah if his knees or hips ever hurt, and he said no.  The doctor asked him if he ever had headaches, and Jonah said no.  Then the doctor smiled at me and explained that everything looked perfect and that because the dose is determined by Jonah's weight, it would actually be INCREASING since Jonah has gained weight as he has grown taller!

  Because the change has been so quick and significant, it has been easy for Jonah to see the results.  It is exciting for him and it makes dealing with the injections every night a little easier.  For the first time, Jonah will not be wearing last year's summer clothes this year.  He can reach the remote on top of the entertainment center.  He is much taller than his little brother.  He is brushing his teeth without needing a step stool at the sink.

You should see the difference it is making in his confidence.  Rarely does he ask if he's "big enough" to do something the way he used to.  He plays on the "big" playground equipment and flies down slides that used to terrify him.  Obviously, that's a normal part of growing up, but I like to think that feeling like he is finally "big enough" to do things is encouraging him to actually DO them. 

What we are NOT seeing is the uncertainty and self-esteem issues that concerned me back in October.  I was worried that Jonah would think he was "sick" since he needed medicine, or that he wasn't good enough the way he was.  I was worried that we were over-emphasizing his appearance rather than celebrating the fact that he was a perfectly healthy kid.  But none of that seems to have entered that adorable little head.  All he knows  is that he is growing up like everybody else.  Finally! 

Kudzu and Giants!

According to our super-scientific and highly accurate pencil marks on the wall, Jonah grew approximately one inch in January alone!  That brings his grand total up to around 2.5 inches since the beginning of the school year.  I swear, he's growing like kudzu.  I have heard that, in Georgia, kudzu grows so fast you can actually hear it growing.  How's that for full service?!  Information on growth disorders and random vegetation trivia all in one place.  Thank God for the internet.

  

The nurse who taught us how to give the injections told us not to expect much progress in the first six months of treatment, so you can imagine how surprised we are to see him shooting up like this.  It makes me a little nervous to think about the rack-like effect this could be having on his joints.  In fact, it made me so nervous that I began to think that I was administering the growth hormones incorrectly.  When a child starts growth hormones, they begin at a low dose and work up to what their actual dose will eventually be.  I started wondering if maybe I was supposed to start each Norditropin pen at the low dose and work up to the .8mg.  I wasn't doing that.  After a few days of obsessing about it, I began to have visions of Jonah's poor joints popping as they try to keep up with his insane growth.  I imagined him ultimately looking like this guy.

A quick call to the Pediatric Endocrinology clinic allayed my Jolly Green Jonah fears.  We are doing the shots right, and if Jonah is growing too quickly we could reduce his dose at his appointment in two weeks.  So, that takes care of that. :) 

Our other HUGE piece of news (besides the fact that i'm not ODing my kid) is that we have been accepted to the Novo Nordisk Patient Access Program!  This means that we will be receiving Norditropin pens, needles, and alcohol swabs at no cost for as long as Jonah's insurance refuses to cover it!!  

From the Novo Nordisk website:

The Norditropin^® Patient Access Program (PAP) reflects our commitment to social responsibility and our belief that a lack of financial resources should not stand in the way of necessary medical care. Through the Norditropin^® PAP, those who qualify may receive free medicine.   Eligibility is based on financial resources, cost of living, size of household, and out-of-pocket medical costs.

Upon approval, NordiCare^® will ship a free 30-day supply of Norditropin^® directly to the patient’s home.  Patients are eligible to remain in the program and receive monthly shipments as long as their financial and insurance situation does not enable them to afford Norditropin^®. Every 6 months, a NordiCare^® Case Manager will contact the patient to evaluate whether assistance is still required.   

So, that's where we are now.  Thanks to Novo Nordisk, we will be able to continue our kudzu-like march toward being back on the growth chart!  Look out, growth chart!  Here he comes!! 

  

Little Victories!

It has been so long since my last post, that you probably figured that Jonah is 6'5" and has been recruited to play college basketball by now.  Not quite, but close!

We have finished Jonah's third Norditropin pen and his fourth is supposed to come on Thursday.  He continues to improve in his willingness to take the injection and his level of calm about the whole thing.  He assembles the injection himself, counting off the steps from taking off the cap to snapping on the needle and dialing up the dose.  He picks which leg he wants to use (he has decided that his belly and butt are fully off-limits), and I do the actual injecting.  When I press the button, he counts down from five, we yell "BLAST OFF!" and the pen flies away like a rocket.  Because of the New Year holiday, there will be a longer gap than usual between pens, and I anticipate that he will need some time to get back to being such a pro.

The literature claims that we shouldn't expect to see growth for the first six months, but I have to show you something after only three months.  Check this out...

The bottom line is Jonah's height at his fifth birthday, September 26, 2011.  The top line is his height yesterday January 1, 2012.  It's about a half an inch of growth!!  Not bad for a kid who usually only grows a few centimeters per year!  Jonah has also moved up to 4T pants.  He was firmly a 3T when he started kindergarten.  Now he can reach the remote on top of the entertainment center.  His teacher, Ms. Ashley (whom Jonah LOVES) noticed that he was growing when he stopped soaking his shirt in the water fountain! 

Our insurance company firmly rejected our claim and our appeal for the Norditropin synthetic growth hormones.  We are working with the NordiCare program to see if we qualify for their patient assistance program.  We have to submit all kinds of tax forms and medical forms as well as apply for Medicaid and submit our anticipated rejection.   So, I have filled out an application for Medicaid and have an interview on January 10.  I have left a message for the case manager handling our interview to ask if we could do the interview over the phone, since the goal is to be rejected.  Understandably, she never responded.  She is entirely too busy with people who need help to mess with people who waste her time seeking a rejection letter.

  I have also contacted the Magic Foundation and spoken with a wonderful woman named Melissa whose entire job is to get appeals approved by insurance companies.  So, if patient assistance through NordiCare doesn't work out, there are still other options. 

In the meantime, we are enjoying watching Jonah's pants get a little shorter and his confidence get a little bigger!