On Thursday Alex had an appointment at the hospital, but it was the start of a new chapter in our journey, we were starting with our fourth department at WCH – endocrinology.
It’s a somewhat blurred starting line, since technically we have been visiting the endocrine department for a year or two, it’s just been for Jenny. This time it was Alex’s turn to meet Dr Jan, a very lovely doctor, who in fact brought forward Alex’s appointment (it was scheduled for October) after a chat I had with her during Jenny’s last appointment!
A quick exam and a slightly longer chat about Alex’s history brought forth a few conclusions. The little pot belly Alex has gained after he finally started to put on some weight is apparently a classic physical symptom of growth hormone defficiency, his lack of growth since radiation therapy was a definite clue too 🙂
Long story short, he’s pretty much a slam dunk for being growth hormone deficient but there are still some tests to run before we can make a start on treatment. Thyroid and cortisol levels need to be checked as well as the growth hormone levels, as the pituitary gland in the brain is responsible for their production too, and the pituitary gland is what the radiation therapy has apparently done some significant damage to. Thyroid and cortisol are checked by blood tests but checking growth hormone levels mean a day in hospital with a line in. Drugs are given to stimulate the body’s normal production of growth hormone (this usually happens overnight so to see it during they day they have to artificially trigger it) and then periodically more blood samples are taken to see what the levels of growth hormone are.
The good news is that thyroid and cortisol balancing drugs are tablets – no worries there. The bad news is that the growth hormone treatment is a daily injection, which Phil or myself get to administer.
There is an increased risk of cancer with growth hormone therapy, however if you are growth hormone deficient and the treatment is just to bring you up to ‘normal’ levels then apparently that risk doesn’t necessarily apply, it’s when you go past your normal levels that you have to worry.
Before we can start on the treatment a few baseline levels need to be checked, so we left Dr Jan with forms for bone growth x-rays and blood tests.
Getting the x-ray of Alex’s hand was fairly straightforward, no big dramas, but Alex was a little on edge because we had to leave Dr Jan’s office before he’d finished doing all the puzzles. One of the quirks of Alex’s behaviour is that generally speaking he’s pretty calm and happy but he’s very procedure driven. He sets out a list of things he wants to do, and in what order, and if that list is disrupted he can have a bit of a melt down. We have learned how to talk him down off the ledge as it were, but he’s usually pretty emotionally fragile for a time afterwards, and if subsequent events upset him, what in other circumstances would be a bit of a fuss but nothing major, results in another meltdown.
Interestingly when I mentioned this to Dr Jan she said that it’s quite common to see behaviours like this in growth hormone deficient kids and that sometimes the application of treatment can have significant positive effect on behaviour.
Fingers crossed.
From x-ray it was on to the blood test. I had a form for both Alex and myself to have blood drawn, since there was a test we needed to have run to answer a question with regards to Jenny. There’s only one more disorder to rule out before the experts can say ‘we don’t know why, she’s just little’, it’s called Silver-Russell syndrome. Jenny had a blood test a while ago which didn’t show evidence of it, but there’s a rare (5-10% of cases) version of it, and to test for it you need a sample of the patient’s parent’s blood. As far as I understand it, they are checking to see if, instead of getting one version of a chromosome 7 from each of us, Jenny got both of hers from me.
<https://www.healthline.com/health/russell-silver-syndrome>
Alex decided I could go first and he would help me be brave. When it came to his turn however things didn’t go quite so smoothly, being a little fragile already didn’t help and he really didn’t like the tourniquet – that bothered him more than the needle I think. In the end the first needle was moved to much by his jumping around to get a blood draw and they had to do a second one. Thankfully that one went smoothly and the tests were done for the day.
It was not however the end of our adventures at the hospital. As you may know Alex has two perforated eardrums as a result of the grommets he had a while back. Unfortunately the damage done to his tissues by the radiation treatment mean that they simply won’t heal on their own, and at this point, nothing much can be done about it. The big problem for us, as well as not being able to hear, is that the perforations allow bacteria in, and Alex is living with more or less constant ear infections. We keep them at bay as best we can with antibiotic drops, but we were literally down to the last few drops and we had run out of repeat prescriptions.
We went in search of nurse Lorraine in the ENT department and were exceedingly lucky and found her. I need to say at this point that there aren’t enough ways to describe how amazing, wonderful, fantastic and increadible nurse Lorraine is. She knew that the ENT doctors wouldn’t be in until 2pm (it was currently 12.45) so she rung and paged until she found one of the registrars, and rung and paged until she arranged for the delivery of Alex’s notes, managed to get both of them to the ENT outpatients desk where we were, and then gave the lovely doctor who arrived enough information that he was happy to write us a script.
We then lodged the script at the pharmacy, had a half hour wait for it to be filled, which we spent in the cafe, and then finally made it back to the car with 5 minutes left before the time on our park expired.
Thinking we were done until the test results had been returned and assessed I was a bit surprised to get a call from the oncology registrar on Friday afternoon. Since Alex is still an oncology patient, his blood results had been sent to them as well as the endocrine department, and they had a concern about his haemoglobin levels. Alex’s last blood test in January had shown levels of around 120 (normal levels for kids his age are around 140 – 160) but Thursdays test had come back with his level currently sitting at 88. To put this in perspective, when Alex was having chemo if his levels hit 60 he’d be given a blood transfusion.
Obviously a level of 88 was a concern and the doctor asked if we could bring him back in for another test, just to see if the haemoglobin level was still low and to see if any other parts of his blood spectrum were low as well. He said there was no rush, as long as it was done by Monday or Tuesday it would be fine. :/
So Phil took Alex in on Saturday before taking him on to his restless dance lesson, and since Dr Jan had given me a form for Phil’s blood test for Jenny, Alex was able to help daddy be brave too before having to have his blood taken again.
So now we wait, hopefully not to long, for the results of 3 blood tests and one x-ray.
While we are waiting we get to experience one of the little joys which came from Alex’s day as a firefighter, we are taking Alex to an Adelaide Symphony Orchestra rehearsal where he will get to meet some of the musicians and the conductor!
Alex is looking forward to it, and has already been practicing his conducting skills.
We’ll let you know what happens on this ride, hopefully there won’t be any loop-de-loops on this one!
