A decade or so before my keratoconus showed up, I recall seeing my former internist back home when I became so horrible ill from what I now know was classical Ehlers-Danlos syndrome. I had lost 30 lbs and was well below 100 due to throwing up from the pain all day and had this gaunt face with dark under eye circles when my doctor walked into the exam room and said to the half-dead person in front of him, “What happened to you?” in a very concerned tone. My internist was a smart and good doctor, but he was scratching his head like a monkey over what could possibly have made a fit and healthy young woman turn into a zombie seemingly overnight. In the end, he ordered blood work and sent me a copy with a referral to a rheumatologist. My doctor hand wrote the following on my report: You look good on paper.
I happened to have my annual eye exam this week and it’s been about 18 months since I had bilateral collagen cross-linking (CXL) to hopefully halt the progression of my keratoconus (KC), as well. The majority of the side effects from CXL have not gone away, and I still have the odd, right eye that originally got 6 lines of visual acuity (VA) back and then started to lose vision last October. I was sure the KC was back and had seen my optometrist to figure out what was going on, but my topographies were unchanged, which left him scratching his head like a monkey and then swearing, which was a first. That exciting appointment is detailed in this post.
The vision in my right eye has become even more ghosted recently and the prescription in my right scleral lens that I need to wear to drive is not correcting the ghosting anymore, so I figured I’d have my ophthalmologist redo my topographies to hopefully shed some light on the problem. After all, two heads are better than one and both my optometrist and ophthalmologist are well-versed in keratoconus and Ehlers-Danlos. For those who know my KC ordeal, this is my dry eye specialist who I actually like as I fired the inept corneal specialist 6 months ago. Good riddance.
Once again, I found out that there is no progression of the keratoconus in either eye, although the cone on my left cornea was blasted off during CXL for some odd reason and my scan still shows no keratoconus, although the resulting scar, or corneal opacity, has resulted in keratoconic vision in that eye that can’t be fully corrected. My doctor checked the opacity for progression and it’s the same, but he said that I had something almost like an iron line in that cornea. I’m not sure if that is a Fleischer ring as that eye technically doesn’t have KC anymore and he said “line” and not “ring,” but I didn’t think to ask at the time and he sounded a little stumped by it.
Also, the tech had me read the Snellen chart with my lenses in—or with correction—but then forgot to have me reread it with my lenses out. Therefore, I have no idea what my actual VA really is, but with great difficulty, I could read 20/30 in each eye and a poor 20/25 with both eyes with correction. Mind you, I can barely wear my sclerals due to severely dry eyes, so I live in ghost-y land the rest of the time. In the right lighting, I’d maybe believe I’m a weird 20/30 with my sclerals, but my near vision is still terrible and if any bright light is around, I tend to get the whiteout effect, holes in my vision, pain, and all sorts of bizarre things. When I got my final pair of scleral lenses in September from my optometrist, my corrected VA was 20/20 in my right eye and 20/30 in my left due to the opacity, so I have lost a line in the former eye with correction and much more without.
Therein lies the problem. Per my topographies over the last 18 months, the severity of KC in my right eye dropped from 76% to 32%. My K-readings, which measure the curvature of the corneas at various spots, have gone down from 51 diopters to 48 diopters at the steepest area, and if you look at my colorful topographies, the pink area (very bad) is now gone and the red area (bad) is extremely small. It’s improved since this time last year, in fact. Now, my CXL surgeon told me, “If you get improvement after cross-linking, you’ll continue to improve for the rest of your life.” Per my scans, it sure seems that is the case and that I’m the poster child of CXL, yet my vision in the keratoconic eye is getting worse—and it’s in terms of ghosting, which should mean an increase, not a decrease, in my irregular astigmatism: the hallmark of keratoconus.
My ophthalmologist was clearly mulling over this and throwing out theories. He wondered if the Ehlers-Danlos was causing changes to the shape of my eye’s lens, which would definitely create wonky vision, but I don’t think he had a test for that. And so in the end when the exam was complete, I got the monkey scratching his head while telling me, “Your eyes really do look good,” which brought back that old memory of the note from my former internist about looking good on paper.
If it took me a decade to figure out Ehlers-Danlos syndrome was what had turned me into the walking dead, should I pay more attention to this oddity with my eye that looks good—minus the keratoconus, etc., etc.—as well?
At this point, I feel like a monkey scratching my head, too.