Sky's Story

If you’re new to this space, start here. I’m going to summarize Sky’s story here in the best way I can, so if you only read one post, you’ve got the basics. This is a little more factual and less feely--there have been SO MANY feelings through this journey--but I'll share those in other places.

When Sky was 4 months old, his pediatrician noticed that he wasn’t tracking objects with his eyes. He referred us to a pediatric ophthalmologist (a rare find, turns out), and even called him after hours to get him squeezed in later that week. This was on a Tuesday. We saw the ophthalmologist on Friday. He gave a preliminary diagnosis of either FEVR or Retinopathy of Prematurity (ROP). Sky didn’t have an eye assessment in the NICU because he didn’t meet the criteria to look for ROP since he was born after 30 weeks and was just over 1500 grams, so it was unlikely that it was ROP. (If you’re a This is Us fan, that’s what a certain character has…)

We were referred to OHSU in Portland to see a pediatric retinal specialist–an even more rare find. We had a phone call with Dr. Campbell the following Monday, and Sky’s first evaluation under anesthesia (EUA) and potential surgery was scheduled for the next week. It all moved SO fast.

We drove to Portland on June 30, 2021 for Sky’s first procedure. Dr. Campbell confirmed the diagnosis of FEVR–Familial Extrudative Vitreoretinopathy–and did surgery that day on his right eye, which was more severe. We went back a week later for him to do his left eye.

What is FEVR?

I’ll explain it as best I can–you can google it and the first thing that comes up is a rarediseases.com page. I can’t actually find a prevalence rate, it’s so rare. I’m part of a Facebook group that is international and it only has a couple hundred members.

As I understand it, Sky’s blood vessels didn’t completely form all the way around his eyes, so they leak “exudate”, or blood byproducts, into the space around his eye. The leaking caused the retina to detach from his eye. He also has a fold in his retina that reached from his lens all the way to the back of the eye, which was kind of pulling his retina away even more, and he has a hole in his right retina as well.

In the procedures at OHSU the surgeon removed both of his lenses to release that tension, cleaned up the exudate with a laser, and put a silicone band (a “scleral buckle”) around his eye to try and push the retina back so it reattached. We just went back on July 6, 2022 to have the buckles released in another procedure, and at this point he’s done with surgeries for the foreseeable future. At all of his follow up appointments we’ve heard the same thing: stable to improved. The procedures worked. The disease is under control, and there has been no more leaking. 

I want to be clear that these procedures were not to restore his vision, but to try and stop the progression of the disease. FEVR is a progressive genetic disorder that he will have his whole life. It caught us totally by surprise–there’s no family history on either side of retinal disease. Dr. Campbell did do a quick assessment on Graham at one visit and was able to see that he does in fact have signs of it, though at a much lesser scale in that he doesn’t have any symptoms or vision impairments from it. 

I remember asking Dr. Campbell what Sky's prognosis is after his second procedure. He said “if we win”, meaning if the surgeries were successful, he thinks Sky will be able to navigate his environment and read on an ipad. I remember wanting more–wanting to realize what that means for his life, what it would be like. I wanted answers that he just wasn’t able to give. 

Blindness

Sky is blind. Over time we have learned that he can certainly see things–he could see light up toys early on and now crawls around the house without knocking into things, or chases after his toys that aren’t lit up. It turns out most people who are blind can see something--light and shadows or just peripheral vision or just central vision. Only a small percentage of people are completely blind. We won’t really know what all Sky can see until he’s able to tell us, but we know he will have deficits. He has permanent structural damage to the retina, and since he doesn’t have lenses he won’t be able to focus without glasses or contacts. At this point he doesn’t like his glasses and pulls them off every chance he gets, but we’re waiting for a new prescription. They say kids like to keep them on when they realize they can see better, so we’re hoping an updated pair will be more motivating. But he will always have impairments. It took us some time and practice to start using the word “blind”--but I’ll go more into that story in another post.

That’s the beginning of our journey with Sky’s vision. Someday he’ll have the words to tell us what he can and can’t see. In the meantime, we’ve got an amazing Early Intervention team that is helping us support him in the best ways that we can. And we’ve got friends and family that are helping us along the way too. We’re grateful for this kid that is teaching us humility in ways we could never have imagined. I’d be lying if I said I’m grateful for his disability, but I’m grateful for the things I’m learning through the challenges it brings.

We grow the most through the things that are the hardest. I have to remind myself of that some days. And other days, I just get to be his mom, and he's just our kid. Many of our challenges are just the ones that come with raising a baby into a toddler and nothing to do with his blindness. This is our parenting journey, and this is his childhood.