Image Source: http://www.buddhaweekly.com

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.

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  1. ShimonZ says:

    Sorry to hear of your difficulties.Doctors and other experts have their limitations too… and we can’t always expect them to know how to deliver us from nature.

    • Thanks, Shimon. You’re right that sometimes doctors and/or science don’t have the answer and that is true with some of my ailments–including my EDS that has no cure or real treatment. Until a few years ago, the clinical trials for the CXL surgery I had to hopefully halt the progression of my newly diagnosed corneal disease weren’t even available in the US, although the surgery is available around the globe. So, if I’d developed keratoconus sooner, I wouldn’t even be chatting with you on my blown-up laptop but on the other hand, if the doctors hadn’t taken so long to diagnose me, I could have had the surgery when my vision was much better and partially correctable with glasses in one eye.

      In my case, I’ve had to be the doctor way too many times–and I primarily blame the very poor healthcare in the city I’m stuck in. Who should have to figure out they have a genetic disease that is believed to affect 1 in 45,000 people on their own? That’s what scares me and I have no trust in doctors as a result.

      I hope you had a nice Shabbos. xx

  2. chloezub154 says:

    I have nothing to offer but the scratching of my head as well… And I get the same thing with my doctors. And people I see that I haven’t seen since I got sick that don’t recognize me. I lost 30 lbs. and I wasn’t overweight to begin with. My positive thoughts are with you.. I guess that’s what I have to offer 🙂

    • Thanks for the positive thoughts and sending some your way, as well. I don’t recognize myself in photos at all anymore–my face looks like a skull with big eyeballs at this point and a PT told me my head is too big for my body. :/ I remember when I first got sick in ’01 and then turned into the skeletal zombie over the course of a year and lost half or more of my huge hair in the process. Anyway, I was in a waiting room in ’02 and saw a guy I knew and had seen about 1.5 years prior. He was literally sitting adjacent to me and when I said hello to him, he had no idea who I was until I kept talking and he had the scariest look on his face the whole time.

      It’s odd that I didn’t quite realize how awful I looked and I was somehow still working and going to grad school (while throwing up in the bathroom half the day). The weight loss wasn’t just from that. I also couldn’t push a grocery cart so I barely had any food and I had zero appetite, I presume from the pain. I had to keep buying new clothes and accused the Gap, where I could afford to shop in my former life, of vanity sizing (funny).

      I was always fit and never overweight just like you mentioned and as I’m still extremely underweight after all this time due to odd, food allergies that I know are related to EDS (negative for MCAD) and horrid, lower GI issues from EDS, I suppose this is just my new normal. It’s really hard when you have almost no control over what is happening to your body and per the EDS, no one here knows about it and I don’t think my eye docs are going to take it on!

      Thanks for dropping by and hang in there…
      A 🙂

      • chloezub154 says:

        I’m getting sick of ppl telling me how great I look. I guess I was a fat lard before?!? They are almost making me anorexic.. Like I need to keep up this low weight… And my dermatologist told me the other day.. You ARE anorexic… Not on purpose… Because you are only eating 500 calories a day… If I don’t vomit it up. Wow.. I’m really sorry I never saw this until it was too late :/

      • My blog is your blog; comment whenever. 🙂 Yes, I have the anorexic look due to insane allergies (EDS-related I presume) and massive food intolerance, albeit no gastroparesis. I hate it and want my old body back. Whoever said that thin was in was just weird. I was normal before and liked it! No apologies… We’re all in this garbage together.

      • chloezub154 says:

        So does it seem I wrote this? oh grr.. I need to look up the word blog 😉 lol. Ya.. I’m pretty damn sick of being told how awesome I look. Apparently I was a fatty before ??? Wonder what they will say when I’m being tube fed.. Sexy? Right? 😉

      • Wait, wrote what? I got lost there! If you mean reblogging, I’m not sure exactly how to do it, but I have a reblog button on my posts so people can reblog if they wish. Usually, it says it’s reblogged and from where, but perhaps it depends on which theme you have? My friend Cissy reblogged the same post as you–you can go to her blog (I think she has a few on her Gravatar) to see what it looks like–she reblogs quite a bit, as do others. It’s not a problem for me either way. 🙂

        Anyway, the weight loss is awful and I’ve been there. I’m up about 8 lbs as I went to the doc’s today (what’s new?) and am grateful I found a couple more foods I can eat. I hope you can find some more or your gastroparesis calms down a bit. GI issues are really hard to live with. 😦

  3. RAD says:

    Hello, How are you doing with your keratoconus now that it’s been a few years since CXL? I saw that you are wearing scleral contact lenses. There are a lot of new and good things in scleral contact lenses now. They are much more customizable and comfortable now than they were even a few years ago. Here is a link to some of the latest info on scleral contacts. https://keratoconusdoctors.com/scleral-contact-lenses-keratoconus/

    • Hello,

      I don’t have great news, but it seems that per the latest studies, I’m not alone as you probably know that 11%-30% of epi-off CXL patients show progression in the new, longer-term studies. My left eye rapidly progressed to the advanced stage in 2015 about 4 years post-CXL and now we are trying to figure out if my right eye is also progressing (I noticed a mild change in June and then I couldn’t even see the TV one day in the fall) and ALSO if the left is following suit again as it has thinned down to the 200s and the Kmax readings are up again. My vision is so poor in that eye that I don’t notice a difference. My newer corneal specialist will be redoing my scans in a few weeks to verify. As my scans have been off before and then gone back to a steady pattern, he just wants to double check. I did go back to CA to see my CXL surgeon last year about my right eye and he felt my Ehlers-Danlos had thrown a wrench in things which I do tend to believe. I also started wearing my sclerals (with much discomfort) 10-12 hours/day up from about 4 hours a few days a week so that could play a role (I moved and was no longer exposed to mold and severe eye allergies), but that is only the opinion of my CXL surgeon who thinks my eyeballs as a whole are likely more malleable due to having EDS and that even sclerals could change my cornea. No one locally believes this theory, but there’s always hope.

      Per the sclerals, I have severe dry eye post-CXL and already had dry eye due to dysautonomia, as well as meibomitis, pre-CXL. I managed before via a side-effect of Klonopin which makes me produce saliva (100%) and tears to an extent (I’m rather like a Sjogren’s patient), and even though the new test (unsure of name) that can test your tear film, see you meibomian glands, etc. shows I am still producing tears via my Klonopin, the tears are not in my eyes and I’m not sure where they are! My lens fitter says I look totally normal on paper, but then look like a mess when he examines me with dry spots, etc. Due to that debacle, I am absolutely lens intolerant but my vision is so bad now that I can’t function w/o lenses. I currently wear Jupiters that have a HyrdaPeg coating and after numerous lens fitters and lenses, I have only been able to see with Jupiters since 2013 so about a year post-CXL. I have a great fitter now, finally, and am getting fitted for ABB Optical–ICD Flex Fit Sphere Visitin+ lenses (with HydraPeg) to see if the thin, contoured edge makes any difference. I am not very confident after the 1st trial pair, as my eyes are still dry as a bone. I use Cliradex on my lids, restasis, IPL 2 x year, Azasite on my lids e/o month and go through tubes of ocular ointment and irritating gel drops over my sclerals and just try to get to the next day with all my EDS pain to boot!

      If you have any other questions, feel free to ask. I recognize your name, but am not sure if we’ve chatted on my defunct blog. There is almost no research on people with EDS (classical/type II) and keratoconus but the few people I have run into online seem to have bad cases of KC. Mine showed up in my late 30s and produced rapidly until I got into a trial in LA about a year later as it took that long to get Dx. I was probably 20/20 before that so this was really the icing on the cake.

      Thanks for stopping by and for the link you provided. I still have a lot of hits and it will help out my readers.

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