For two years, we flew our younger son to Melbourne each three to 6 months to participate in a drug trial. There isn’t a higher place than Melbourne’s Royal Youngsters’s hospital for such a gruelling exercise. Monumental fairy dioramas hold from the roof. A cylindrical fish tank as large as a whale’s gullet and three storeys excessive is full of brightly colored tropical fish. An ice-cream store within the hospital’s lobby is a superb supply of sugary bribes.
Each second journey was the worst. That’s when medical doctors inserted a cannula into the again of my son’s hand or foot so they might take a number of bloods over the course of the day. As soon as, when my son was two, the nurses and anaesthetist struggled to seek out our son’s veins. He has significantly tough veins to seek out: they’re smaller than different youngsters his age and hidden below extra flesh. It was my job to carry him in my Wolverine grip whereas the anaesthetist ran his ultrasound machine over his wrists and ft. He seemed panicked. I used to be crying, my son was wailing and me with massive eyes that stated: Why on earth would you let anybody do that to me? They tried many times till his onesie was coated in contemporary blood and our tears and the nurse stated, “You may inform us to cease, you understand”.
“Then cease!” I stated.
The anaesthetist seemed relieved. “I’d have finished the identical factor,” he stated. “I’ve a daughter the identical age, and I’m unsure I might watch her undergo that.”
I took my son exterior on to the wind-blasted balcony, surrounded by white stones and succulents. We held one another and cried nice unfastened sobs till I might regular my voice sufficient to name my companion.
“I’m unsure I can do that any extra,” I stated to him.
He fell silent.
I defined: the entire level of enrolling our son on this drug trial was to minimise medical intervention in the long term, however what if it wasn’t medical intervention? To place him via this each six months for the following 17 years? He’d you should definitely develop a phobia of medical doctors and hospitals – and in that second I used to be not satisfied the payoff was that nice.
However I by no means did pull the pin. Like all troublesome choice sophisticated by lengthy lists of professionals and cons, my companion and I in the end determined that the professionals outweighed the cons.
Right here’s the background:
At his beginning, our son was identified with achondroplasia, a congenital situation attributable to a mutation of the FGFR3 gene. It’s the tiniest change, nearly too tiny to think about. At one level within the fibroblast progress issue receptor 3 protein chain, the amino acid glycine is switched out for the amino acid arginine. That’s it. However this tiny change makes the protein very energetic. The lengthy bones of an individual with achondroplasia ossify sooner, whereas the identical bones in an average-statured particular person keep cartilaginous for longer and develop longer because of this. And that’s why essentially the most seen symptom of achondroplasia is brief stature, or dwarfism.
Persons are much less acquainted with the suite of different potential side-effects. Youngsters with achondroplasia have smaller respiratory passages, smaller veins, and the foramen magnum – the outlet at the back of the cranium via which the backbone passes – is way smaller than in average-statured youngsters, whereas their skulls are a lot bigger and heavier. Some research have proven the toddler mortality charges of youngsters with achondroplasia are 5 occasions larger than common, and that is largely the results of extreme obstructive respiratory, or sleep apnea, and compression of the foramen magnum.
Different joys await an grownup with achondroplasia. Their life expectancy is 10 years decrease than for average-statured individuals. The composition of their bones results in a better threat of spinal stenosis (a narrowing of the spinal canal) which could be debilitating. Many adults with the situation depend on mobility scooters and painkillers to alleviate power ache. Some select to bear surgical procedure. I spoke to at least one younger girl who had 5 surgical procedures in 10 months when she was in her 20s. She stated:
In August 2016 I had two spinal surgical procedures throughout the week which had been each eight hours lengthy … I bear in mind describing the seven days in between surgical procedures as absolute hell, it was so painful and so arduous to do something … I simply began to show a nook after which I needed to go in and have one other one the next week. The second was so painful as effectively. The surgeon instructed me that it could be a month till the ache settled …
Regardless of the ache these surgical procedures inflicted, she believes they saved her life. Earlier than the surgical procedure she would get pins and needles in her ft as she walked round. If she lay down for brief intervals of time, ache from the arthritis in her again made her seize up and battle to rise up once more.
As soon as the surgical procedure was finished and I had recovered, none of that occurred any extra and I might stroll a lot additional than I ever used to. It was wonderful!
When our son was 14 months outdated, he was given a chance to be one of many youngest candidates in a trial for a brand new drug – Vosoritide – that promised to remove the necessity for this sort of surgical intervention. We leapt on the probability.
Since making that call, what continues to hang-out me is the thought that our son will flip 15 and ask: Why did you attempt to change me? Didn’t you like me the best way that I used to be?
However what haunts his father is the thought that if we don’t proceed the trial our son would possibly say: Why didn’t you do every little thing you would to maintain me out of the surgical ward; to assist me dwell pain-free?
The truth is, we do not know what our son will assume when he’s 15, 20, 50, 80. We’ve needed to weigh the choices, take a punt, and hope he’ll imagine we made the best selection.
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How the media deals with our choice, though, is another matter altogether. They want cowboys. Pistol shootouts at sunset. Otherwise, where’s the story?
Over the last four years, Vosoritide’s piecemeal coverage in the media has become increasingly polarised. Recently on Channel 9 news, the drug was celebrated as an alternative to “limb-lengthening surgery” – a breakthrough “cure” for a “disorder”, something to be used to help dwarves grow taller. While in an interview on an SBS Dateline documentary with a member of the Little People’s Association in the US, the drug was compared to “genocide” – an example of doctors trying to erase an entire category of people.
In her Sydney festival play Othering, producer-performer and short-statured woman Debra Keenahan recalled how a doctor advocating a medical trial asked her, “But don’t you want to be taller?”
“No,” was her exasperated answer.
According to Keenahan, this was a cultural position the doctor seemed unable to comprehend. Why didn’t she want to be “fixed”?
Because, she argued, not every difference is a problem waiting to be solved.
In all these stories, whether for or against the drug, the emphasis is placed on growth, and growth is equated to length. But there are so many other aspects of growth to consider – how cartilaginous are bones? How dense? How brittle? How prone are they to causing pain in the human they support?
Some doctors who publicly campaign for Vosoritide frame it as a “cure”; a chance for longer limbs. They fail to see that a culture, an identity and a community exist around people of short stature. This is the main concern I have around our decision. What if the drug ends up placing our son between two cultures: the short-statured community, and the average-statured community, and he finds he doesn’t quite fit into either?
My partner isn’t as rattled by the quandary. “I was there when our son struggled to breathe. I know we made the right choice.”
It is predicted that Vosoritide will open up the foramen magnum, the spinal canal and airways. Despite these medical benefits, it’s the claim that it will likely add 10cm to the average height of 130cm for an adult male with achondroplasia that seems to be the sticking point in the cultural debate about the drug. What were the reasons behind the development of Vosoritide? Were they medical or cosmetic? Is it a moral problem if the answer is both, even if we only enrolled our child for the medical benefits?
As firm believers in the social model of disability, it was important to us that our son keep his identity as a person of short stature. When the time comes for our son to learn to drive, I would rather use NDIS support to adapt our car so that he can reach the pedals than medically tinker with his body so that he can fit the car we already own! But it was also important to us that we do our best to reduce his chances of experiencing debilitating spinal surgery or living with chronic pain in the future.
Our son is now five-and-a-half. We’ve been giving him daily injections at home for a little over four years, and even though my doubts persist we will probably continue to do so until he stops growing at age 18. He still snores – badly – and may still have to have his tonsils removed. He may end up needing grommets to drain fluid from his smaller hearing passages at some later stage. But since enrolling in the trial, he hasn’t needed to undergo surgery. He still looks like a short-statured person. He has a larger head (setting off his fabulous head of hair!) He is still a whole head shorter than his peers and the difference is growing every day. He needs a scooter to get around school, and adjustments need to be made in his classroom to make things easier to reach. But, so far, he can walk, run and play pain-free, with relative ease.
My partner would tell this story differently, of course, and so will our son, when he is old enough to decide how he feels about the decisions we have made on his behalf. I hope we will be able to cut through the noise and listen to each other’s versions with open hearts, and our cowboy hats set to the side.
