Today I'm wearing my "researcher hat" and spending my afternoon researching growth hormones as they relate to a person with Noonan Syndrome.
Wednesday, my little guy goes to the endocrinologist for an assessment. I'm not so sure that I would have entertained this issue except for the fact that he's falling off the "normal" growth chart (damn) and he does have a "thriving" issue. Although, I must say since January my little guy has managed to pack on 4 lbs. Just wished some of that weight was because the body was growing vertically too.
I understand that the doctor we are going to see is a bit "dry" and very "to the point" and "clinical" in nature. I'm not expecting a "warm and fuzzy" visit. (damn) I was told by the referring physician to enter this appointment armed with information on growth hormones. So I've done my research, what little bit I can find on the correlation to Noonan Syndrome and the growth hormones available to treat "short of stature" as it relates to Noonans.
It appears that most of the research has been done over in Europe - another thing that ruffles my panties - why is it that all of the research on Noonans seems to be driven in Europe? Canada is even better suited with information on Noonan Syndrome, come on U.S. - lets jump into the game too!
So after deciphering two different research papers, and re-reviewing one given to me while my guy was in the hospital in February, I'm still at a stale-mate as to what to do. I sort of wanted to go into the appointment with a strong opinion one way or another so that the endocrinologist had to sell me on the opposite of my opinion. (Again I say damn, because I'm the pickle in the middle.)
So based on the research I did find - and from Great Britain I might add - basically it didn't give me a rousing you got to do this, it's the best thing since the invention of (you fill in the blank)! Some people did well on the therapy and were lucky enough to add on a few more inches to make them a bit taller than they might have been without the therapy others didn't fair quite as well. Then there are side effects to take into consideration - some of them are not so nice side effects either. The thought of having to inject via a needle medication daily is also quite overwhelming to me, a needle-phobic, and I'm sure my son would rather not be stuck by a needle daily. I also need to take into consideration my guy's heart issue, and what type of stress will this do to his already "slightly taxed" pulmonary valve? I haven't found anything to support an issue either way, but I know that adding hormones especially ones that accelerate growth have to have some sort of adverse effect on the heart, even if it's so minimal that a researcher wouldn't need to report it. The other thing that scares me is that you're adding hormones to force growth, god-forbid something "bad, like cancer" is laying dormant in his body? Adding the growth accelerator could also help accelerate the growth of the "un-want-ables" too.
BUT then .... I look at my guy and see how little he is in comparison to his peers. Its cute now, but what will it be like in 5, 10, 15 years from now for him? I'm going to admit something horrible here, but I tried to stay away from dating short guys (shhhh, don't tell my husband), but I always hated when short guys approached me when I was a young single person because I was short like them. I felt like a short guy magnet. Horrible of me to have ever felt that way, but I spent a period of time feeling that way - and I'm not proud of it. I'm glad I shed that attitude because I would never have met my husband. My life wouldn't be so fulfilled and happy! In fairness, my husband isn't really that short, he's just not basketball playing material!
Then I remember being in college and out at a bar and some random guy stopped me and asked me how tall I was. I told him 4'10 3/4" and he asked me if I knew that if I was 3/4" of an inch shorter, I would be considered handicapped and a midget and I would be eligible to go to college for free for my disability! Of course, the sassy person that I am turned to him and said, "If you are using that as a pick up line, you failed miserably" and I walked away. Who knows what his agenda was, but I wasn't planning on sticking around to find out. I'll be honest though, that comment has stuck with me to this day. Which is why I worry about my guy and the type of comments he will be forced to hear along the way.
Being short is a huge challenge. Finding clothing that fits that you don't have to hem or have tailored is nearly impossible. Thank goodness for capri pants! I can never find shoes to fit my feet because my feet are so small for an adult woman. I miss the cut-off by most shoe makers by 1/2 a shoe size if not 1 whole size. It plain sucks!
Needing a step stool to reach things is a pain too!
For me, though, being a woman, being short doesn't hold as much of a stigma as it will for my little guy. I can strap on a pair of stiletto heals and add a few inches, he can't! He will be teased and this already breaks my heart. I'm sure he'll run across bitchy girls like me who wanted nothing to do with a "short guy". These are things I can't stomach and I can't protect him from. So the thought of having the ability to administer growth hormones to help "average" him out is very tempting. But would that be the right reason?
I'm hoping the endocrinologist can shed some light on this "topic" for us. I want to make the right decision for my son. I wish he could be a part of this decision-making, but being only 3 years old - its hard for him to understand this topic. All I know is that if it is recommended, it will have to be a strong recommendation and my son's cardiologist will need to be in on this as well.
Good luck on Wednesday!
ReplyDeletePS: Health Canada has not approved GHT for Noonan Syndrome. It's considered "off-label" and therefore an out of pocket cost if we pursue it. We're looking at $2,500 per year for the first few years, increasing with growth (because it's by weight) to upwards of $25,000 per year. I have heard that there are a lot of new technologies in terms of injection systems that make it easier and less painful. Lots of parents on the forum/list serv have also said it gets easier and less painful as their kids get used to it.
You didn't ask for these but here are the questions I'd want answers to... whether it HAS to start now or if you can wait. What the expected growth addition per year should be? When is the latest you can start and still expect it to be effective? What additional benefits are there besides height? What would be his anticipated final height if you start now, in two years, in five, 10?
And ask yourself if you want him to be part of the decision and can you wait that long?
As well, is he maintaining on his own growth curve? Our endo isn't pushing GHT right now because, while Lauren is off the standard charts, she is consistent on her own curve. Slow and steady wins the race to 4' 8"...
Ask how the determine whether he's growth deficient or, as with most NS kids, producing GH but not using it effectively.
It was also suggested by one of our other practitioners that Lauren would have to pass a sleep study before she'd be approved for GHT anyways. Not going to happen any time soon. Guess that decision is made for us huh.
Looking forward to hearing (reading?) how it goes!!