This week, we have been called upon to make a tough decision about Carys.
She’s very small, about the size of a five or six-year-old, yet in nine weeks’ time she’ll be nine years old. Her hands and feet are tiny; she wears a shoe in an infant size 10 (Euro size 28, Us size 11), and it still has plenty of growing room. I believe that size equates roughly to the size of the average two-year old. Told you she was small.
All the blood tests have consistently indicated that she is producing growth hormone; it’s at the lower end of the scale, but it’s still within normal limits. She has grown in the past 18 months… by a whopping 6.5cm – woohooooo! But it’s way below where she should be.
In that time, she has also gained two kilos. That’s great news, because prior to that, whilst she was suffering so badly with chronic constipation and impaction, she lost weight, fluctuating between 17 and 19 kilos (37 and 41lbs). But the average nine-year old girl should weigh 28kgs. She’s the weight of a five-year old.
I have learned over the years not to get hung up on stats where Carys is concerned. The normal rules and milestones don’t apply. All that matters has always been whether she’s healthy and happy. Everything else is just ‘stuff’.
So, what her endocrinologist wanted to know, was how we wished to proceed from here.
It’s not as if we didn’t already know all this. It’s something we have been monitoring for a few years now. It’s not uncommon for children with Cardiofaciocutaneous Syndrome to be short of stature. Many of these children don’t seem to grow much beyond 4ft. Many don’t go through puberty. Their pretty faces age, but their bodies seem stuck in a time warp. the no-man’s land of the perma-child.
Not being very tall might not seem like much of an issue. I’m rather on the small side, myself. But in the extreme, it brings additional health and emotional concerns.
Like I say, if she doesn’t grow enough, her body might not be prompted to enter puberty. Good, I hear you cry, there’s nothing worse than having a moody teenager in the house! (I’ve already got one of those, and another imminently metamorphosing, so I know what you mean!).
Being small makes her easier to manage, from a parent’s point of view, in terms of lifting, carrying, and so on. But no growth means no puberty. What if she falls in love one day, and wants children? Highly unlikely, I know. But what if?
What if one day she realises how different she is from her peers? She doesn’t give a fig right now, if she has even noticed. But who knows how she’ll feel, or what she will be capable of thinking in the future.
Regardless of stature, it is not advisable to allow puberty not to happen. But looking after a disabled child with periods looks pretty daunting to me right now. Puberty can be ‘forced’ to happen with drug and hormone treatment, I am reassured by the doctor. Only, I’m not feeling very reassured.
What she needs to do is GROW. And therein lies the whole problem. She eats well, but it’s just not happening. Does she have lots of energy? Yes. Does she sleep well? Yes. Should we start Growth Hormone Therapy?
Does she really need it?
Probably. Yes. I don’t know.
Conor and I have talked about this a lot. We can’t get Carys’s opinion, so we have to decide for her. Growth Hormone treatment involves a daily injection. She won’t understand that. All she will understand is pain. Yesterday, she had blood taken for some tests. It took three of us to hold her down, and one to administer the needle. She fought really hard and screamed blue murder.
I would have to give her the daily injection. Me. At home. Sometimes by myself, if Conor wasn’t around. I’ve never given an injection in my life. Of course I would do it for Carys if I had to. I’ve had to do some crazy things for my kids that are well above and beyond anything a parent expects to have to do for their child. But I couldn’t restrain her and give the injection by myself.
I know people who have used GHT for their CFC child. In some cases it has worked. In some, it hasn’t. In some, muscle tone and strength improved,as well as a few centimetres of growth. But there is no guarantee of success. Although failure is not a given, either.
Yet again, Conor and I must play God with Carys’s life.
Rightly or wrongly, we chose against Growth Hormone Treatment. For now.