I never wanted to write this post, but feel I must. I haven’t blogged in nearly 5 years, but have chatted with many who have found my site looking for information. Aren’t we all?
In short, I had bilateral, epi-off collagen cross-linking (CXL) surgery in 2012 in Los Angeles via a clinical trial that I paid a fortune for. I have long-term side effects from CXL such as severe dry eye, corneal neuralgia (nerve pain), light sensitivity, and corneal capacities. I saw the best of the best. But, I also learned from that keratoconus specialist that I ought to see a geneticist where I finally got the correct diagnosis for my chronic pain and health problems: Ehlers-Danlos syndrome, classical type or the former type II. I believe the latter is what has caused so many issues with my CXL surgery and possibly its ultimate failure, but nobody knows and my doctors have various theories.
In 2016, 4 years post-CXL, I noticed I had very poor vision in my left eye without my scleral lens in. This had been the eye with the best correction via a lens and no longer was. It was my better eye pre-CXL as it was affected last and it completely flattened after CXL and was actually too flat and that and the central scar from CXL were what made it necessary for me to wear a lens in it to see. In essence, this eye no longer had any trace of keratoconus (KC) and was the last of my eyes I was worried about. I wasn’t getting the best follow-up care locally as the years went on, but found a new optometrist ASAP when I noticed the diminished vision. She did a topography that showed major steepening in my left eye and I saw a new corneal specialist after that and he confirmed that my KC had indeed come back and was progressing rapidly. I wanted to just hang my head after all I’d been through. He was stumped and told me to see my KC specialist/CXL surgeon in Los Angeles. It is very hard for me to get a ride, but I did and my doctor there was just as confused and suggested not wearing my scleral lens for a few months in the event it was distorting my entire eyeball due to having Ehlers-Danlos, which causes me to have stretchy tissue and weak collagen and a million other issues (the thought was the lens could be squeezing the stretchy tissue in my eye and causing my cornea to cone which sounds very odd). I followed his orders and drove all winter with no lens on an eye with advanced KC and have no idea how I didn’t get in an accident at night with the fireworks show going on in my left eye while trying to focus out of my right eye via my scleral lens. Neither eye was ever 20/20 with lenses, either.
My left eye seemed to stabilize, however, and I thought I was out of the woods and got a new pair of scleral lenses by a fabulous, local lens fitter (optometrist) I stumbled across. He noticed the acquired strabismus I had in both eyes from KC and it was very bad in my now legally-blind left eye. The strabismus did go away with good lenses in, even if I didn’t have perfect vision (phew). He also realized I had a 5th nerve palsy, likely from Ehlers-Danlos, which also causes my left eye to mildly turn down and to the left, although who knows how far to the left when I look like Biggie Smalls from the loss of vision. Even better, he told me I always had intermittent strabismus (100% and Ehlers-Danlos thing) after I told him I spent my pre-KC days watching TV sideways and that my vision would get very messed up when I got tired and spaced out. I told him I always thought I was crossing my eyes, but as I have exotropia, which is the opposite, my eye or eyes were really floating tot the sides. I really hope I didn’t ever do that in public, but I did get a lot of comments about that sideways TV watching for over 30 years. He was even able to make me a pair of glasses for nearsightedness and astigmatism with prism (for strabismus) that helped me see a little better while not wearing my lenses, even if mainly out of my better eye. They were very off at first due to my high level of irregular astigmatism, hence needing to wear scleral lenses, but I got used to them within a month and they helped a bit. The lens for the left eye was thicker than the frame and I actually cried when I first saw them as I had near 20/20 vision until this debacle started in my late 30s and was already so sick and now the KC was more than the icing on the cake as the visual image of the glasses through my scleral lenses screamed disabled at me.
Yet, I had finally found a great doctor here and things had stabilized and I was seeing better, albeit with painful eyes, and that was a positive, despite my emotional breakdown after seeing those glasses that didn’t even allow me to leave home with them on. I should have held back my tears. I could still use my adapted laptop, text on my phone on the largest font, read larger print on paper, take notes while on the phone, and watch TV and read the ticker tape–even if it was far from HD quality. After all, I was the one who had very wonky vision when I started this blog, but could read 20/40 out of each eye post-CXL without lenses (I still question that as I couldn’t drive to save my life until I got lenses). This was all about to change.
In 2017, once again in the summer, my right eye could no longer read the TV. I thought it was from an eye drop I was using, but I stopped it and I still couldn’t read the TV. My vision seemed worse all around, but it was a mild progression if any. I made an appointment with my wonderful lens fitter and got the bad news that this eye appeared to be progressing based on some tests he did, but my cornea wasn’t any steeper so he said I needed a test from my corneal specialist as he suspected something else was going on. My corneal specialist confirmed the progression of keratoconus in my right eye and while my left eye was still stable over 6 months later, it had thinned a lot when it started to go again and the right eye was now doing the same. My corneas were just over 400 microns for my CXL surgery in 2012, but were now in the 200s and 300s. Additionally, my right eye was actually thinning from the back, so posteriorly, as my lens fitter had suspected. The topographies of my right eye are rather worthless, as a result.
I was closely monitored again and things stabilized for a few months. In the fall of 2017, the vision in my right eye became poor and I could no longer function without lenses while home, not that I actually have a home, and as someone who produces no tears and is lens intolerant, this was the worst I could imagine as I functioned lens-free at home for over 5 years. I actually wrote every past post on this blog without lenses in and with eyes coated in ocular ointment due to the severe dryness. Now I couldn’t see my phone, my laptop, the TV, or much of anything else. I clung to my ugly glasses hoping to see a tiny bit better and have been frustrated beyond belief as I was already disabled and can no longer live a normal, full life like others and am home in the latest motel for hours or days at a time in my ghost-y world of white light and faded shapes and colors during the hours before I shower and force my lenses in.
I did get yet another pair of sclerals in February of 2018 and had the best vision I have had with them via my great optometrist. He saw me again in the early summer and I read 20/25 in both eyes still (I think that was a stretch and I took my time and had a lot of guesses). I had moved out of a moldy condo in November of 2017 and he thought that my horrible allergies there had possibly set off the KC progression and now that I was back in an overpriced (post-recession) motel, maybe things had settled. I had seen my corneal specialist in early 2018 and all seemed stable so he told me to see him in 6 months. I’ve had chronic sinusitis for 3 years from that moldy condo and was healing from failed sinus surgery and dealing with the never ending infection. I was trying to focus on that, my Ehlers-Danlos issues, my dwindling bank account, my loss of housing again, and my attempt at volunteering again so I had other fish to fry. Little did I know that once again, the crap was about to hit the fan.
I had noticed the vision in my left eye was non-existent without a lens and diminished with my scleral lens in come July of this year (2018). I do my makeup via a 20x magnifying mirror and also saw the my tear duct plug had popped out of my left eye and when that happened in my right, my vision got much worse so I presumed that was the issue. My optometrist wasn’t in but I saw his colleague for a new plug and this time, my vision didn’t clear up right away when I reread the eye chart. The doctor told me it might take a few days to settle, but I was nervous. The vision never improved and I told my corneal specialist when I saw him for my 6-month follow up last month in August, just a month after noticing the huge change. My topography didn’t seem to register (maybe the tech wasn’t the best), but like my right eye, the left is now progressing from the back of my cornea. The black hole (area of extreme thinness) on the pachymetry for my left eye is much worse and in just 6 months, has thinned another 80 microns or so. My doctor spent more time looking at my cornea via the slit lamp exam than ever before. He told me the central scar had gotten worse. As that eye has been staged advanced since 2016, it also has Vogt’s striae and a Fleisher’s ring. Despite his saying that everything that can go wrong with a transplant will back in 2016, he now told me the time had come to do a transplant in my left eye. These were the words I never thought I would hear.
While he’s a younger doctor who trained at UCLA (Jules Stein) and so on, he only does PK (full thickness) transplants which he feels give the best vision post-op. I want DALK (partial thickness) as I heal terribly and it’s a speedier recovery. I also have absolutely no help locally, so I need to be able to drive even if via 1 eye and be back in a lens ASAP and I know that can be over 6 months in someone who heals normally. The mere thought of bad post-op vision and having the world see my cocked, Biggie Smalls eye makes me want to cringe and that’s if things go relatively well, as in the transplant doesn’t fail or reject. I would need to get to Los Angeles for DALK and no one from my oh-so-helpful family in California is willing to drive me. I also know how terribly painful corneal surgery is from CXL and while some do fine with transplants, I don’t think I will get lucky based on my CXL experience and I can’t take narcotics so the thought of traveling out-of-state with horrendous pain and light sensitivity is giving me pause.
This is where I am today. I haven’t scheduled surgery yet and am waiting for my optometrist to go to a big eye conference this month to seek out a doctor who has knowingly done a transplant on someone with keratoconus and Ehlers-Danlos syndrome to get their take. I have no choice as I’m at risk for corneal hydrops due to the thinness which is now well below 200 microns. The lack of any help post-op and all that can go wrong mainly due to my Ehlers-Danlos have made me want a Valium drip 24/7. I don’t know if my KC came back after several years of dormancy due to having Ehlers-Danlos, severe environmental allergies from the moldy condo, from the never ending sinus infection and antibiotics that have taken a huge toll on my body, a looming diagnosis of primary immunodeficiency disease which would explain the latter, or if I am just one of the roughly 30% of people who don’t get lasting results from epi-off CXL as the latest and longer-term studies now suggest, per my doctor and what I’ve read in studies, as well. I also wonder if anyone in that roughly 30% have any of the above.
I still recommend epi-off CXL as a first line of defense for those with mild-to-moderate progressive keratoconus as no one has heard of or written about the long-term side effects I got from CXL and I still feel it is safe and largely effective. However, as hindsight is 20/20 (yes, a pun), I would rather not have painful, bone-dry eyes and excruciating corneal neuralgia and I’d really like that kidney I had to sell for bilateral CXL back (that’s a sad joke and illegal in the US).
The only thing that hasn’t changed much since I started and stopped posting on this blog is that my posts are still around 2000 words long. Sigh.