As Josh’s condition is so rare, the treatment options are limited, and so is the information about them. We talked to a lot of people and did a lot of research on our own to get our heads around what we needed to do. Our PO presented surgery as an option, but she didn’t push us one way or the other. Surgery went against all my natural instincts as a mother, I hated the idea of it, and really wanted to take the more appealing “wait and see” approach. But the more we researched, the more we realised that surgery was our only option.
So the wheels were set in motion for Josh to undergo a lensectomy. This involves removing the lens completely. It’s a common procedure, usually performed in people with cataracts. In a standard lensectomy, an artificial lens is placed into the sac that holds the lens, and there’s no more to it. In Josh’s case, the sac is unstable so it would never be as straightforward as all that. The option we were presented with was contact lenses. An eye without a lens is “Aphakic” and there are special contacts made for just this purpose. I imagined that trying to put a contact lens in a toddler is like trying to walk a cat on a lead. After a little more research we learned that Aphakic contact lenses cost around $250 USD each, and they’re not the kind of thing you can easily pick up at your local optician or drugstore. Factoring in how often he will lose or damage a lens, and the struggle we’d have taking them out and cleaning them and then trying to put them back in, this was not a favourable option.
Needless to say we were more than a little apprehensive. Then we came across an online community for parents just like us, with kids dealing with eye conditions. After a year of no one really knowing what we were talking about, and constantly having to explain ourselves, we found others who knew exactly what we meant. Little Four Eyes is the name of the site, and finding it felt like someone holding out their hand to help us up. Finally, we found people speaking the same language, and what they were saying was that Intraocular implants were the way to go. An IOL is an artificial lens, but it’s attached to the eye itself, either with stitches or a clip. There are a couple of different types of lenses, and different ways of attaching them, but they’re permanent, and, if this type of surgery was a success, it would open up a world of possibilities for our boy. We had a lengthy discussion with the PO, determined that this was how the surgery was going to go. She agreed and we planned accordingly.
I was still anxious about the surgery after this decision was made, but ultimately knew it was the right thing to do, and I really felt like we had done our homework. The surgery was booked, and we were committed.
In order to correctly fit the IOL, Josh needed to have an examination under a general anaesthetic, where the surgeon would measure his eye. We planned to include a minor procedure called an “Oblique Recession” on his left eye. His left eye “drifts” up a little when he’s using it, and this surgery would adjust the muscle so that the eye stays where it is supposed to. Today was the day.
It was day surgery, but he needed to fast from 7.30am. We arrived at the hospital at midday, and were told the surgery was scheduled for 3pm. We were advised that they had pulled the pin on the muscle procedure, but couldn’t tell us why. It was already shaping up to be a long day, and by 2.30, after 4 hours in the waiting room, the cracks were starting to appear. At 3, our PO arrived, she commented on Josh’s glasses, saying “I didn’t know he had glasses,” despite the fact that she had prescribed them – it was not a good start… By 4pm when they finally came to take us through to theatre, Josh had had enough, and he put up quite a fight with the anaesthetist. Sitting there holding him as he kicked and screamed and held his breath, I wondered what on Earth we had done. 45 minutes later and they were done. The team of surgeons, our PO, a registrar and the Professor overseeing it all, came to talk to us. The news wasn’t good.
They had found when they measured his eye, that it was small and flat, not a good fit for an IOL. Not only that, they simply don’t make them to the strength that Josh would need. So we are back to the contact lenses, right where we didn’t want to be. It was a disappointing setback, and the road ahead seems longer than it was 24 hours ago, but our boy has bounced back today and all he cares about is when we’re going to the playground! You can’t really ask for more than that.