We were told days after Ethans diagnosis that there was a glimmer of hope…
There was a drug, a man made enzyme to be exact, the very same enzyme that our Ethan was missing. The very same enzyme that our boy needed. It was, at the time, the most expensive drug in Europe.
This drug was not a cure. It was however, a little vile of hope. The drug itself was in its infancy, boys (& 2 girls!)all over the world with Hunter Syndrome were receiving this drug. There was not enough data to tell us how, in the long term this drug would help our boy, but, but it did show enough data in the here and now to prove, yes prove, that this drug was indeed slowing down the progression of Hunter syndrome. That was hope.
We, very naively, thought Ethan would get this drug a month or so after his diagnosis. We were wrong. Very wrong. Money makes the world go around~ well, ain’t that the truth.
We were told, due to Ethan’s age, that it was unlikely the drug would help him, as the syndrome had clearly progressed. He was now 6.
We were sat across from the metabolic specialist in Dublin.
“But it’s your fault he wasn’t diagnosed earlier” I screamed at the nicest doctor Ethan had ever had – Batman. “I’ve been up and down to my gp, ENT in Galway and to the public health nurse, telling you something was wrong since he was a year and a half” I knew I was crying, I didn’t care, I let the tears run down over my nose and into my mouth.
Batman titled his head, took a deep breath and slid a piece of paper to me. In his soft Australian accent, he confided, “And you’re right. It is the HSE’s (Health Service Executive) fault. Not yours. And I won’t have Ethan denied his treatment ” he stood up.
He was a tall man with a tight haircut who’s office was decked out in ‘Batman’ memorabilia, “You got spunk kid” he patted Ethan’s wiry hair.”Batman” Ethan giggled as he continued to play with his toys unaware of everything we had just been told.
“As ye know folks, the drug is expensive” he sat back down,” But, we can and will get it. Here’s how…”
Batman, six months later, returned to his native country, where he is still working fighting for children like Ethan, we still email to this day.
I cannot for reasons, I’m sure you can imagine, type what his plan was, but this was clearly not Batmans first rodeo.He knew the law like no doctor I have ever met (and I’ve met a lot!). He fought each step of the way with us, silently but very very strongly.
It took four months from this meeting to get Ethan this drug.
Batman was there with us for Ethans very first Enzyme Replacement Therapy~ ERT. It was very emotional for us all, especially Batman, as Ethan was his last ever Hunter boy to help in Ireland.
This drug is infused into Ethans body over a three and a half hour period, once a week, every week.( It looks a little like dialysis, to the untrained eye!)
We travelled from Galway to Dublin, once a week for a year until our local hospital were trained and ready for ERT. We then attended our local hospital, under the care of the wonderful Dr. Browne for another five years. We are now one year, since September, doing Ethan’s ERT at home.
Without Batman, I’m sure the outcome would have been different. Very different.
It is not a cure.
But Ethan is now 12.
Boys with Hunter Syndrome, as severe as Ethans, had been up until ERT, living into their early teenage years, maybe 14. Now though, more and more of the boys are living into their 20’s.
No it’s not a cure but fuck it’s some amount of hope.
Imagine what the next few years may bring in research?
Currently there are trials going on, (which Ethan was unsuitable for-that’s another story) they are now trying to get this ERT into the brain, it is by all accounts successful.
Right now ERT only helps the body, meaning the brain will still regresses. I hate explaining this part of Hunters. So when I type things like Ethan forgets words, sadly that is why. If this drug could get passed the blood brain barrier, well, folks, that’s as close to a cure as we need.
Will Ethan see this in his lifetime …I live with that hope
Hope is a powerful thing.
Batman is real.