BILATERAL EPI-OFF COLLAGEN CROSS-LINKING (CXL) FAILED

when_the_shit_hits_the_fan_3215951

Image Source: emergency-room-nurse.blogspot.com

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.

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5 thoughts on “BILATERAL EPI-OFF COLLAGEN CROSS-LINKING (CXL) FAILED

  1. ShimonZ says:

    think of you often, and always hope you are doing well. Sorry about the trials you’ve been having. Hope that the new year will bring you relief and alternatives… we would like things to be one way, and often they work out another… But then, it’s learning to make the most of life even with the other… sending you my very best wishes.

    • Hello my dear friend! I must say omein to your comment and I, too, think of you and what has transpired in your life over the years. Life likes to keep throwing wrenches my way and it is wearing me down day by day, which is why my Hebrew name means the weary one. My real name, which is Anglicized Hebrew, means joyous one. I don’t think my mother researched either that much.

      I hope you are doing okay as I saw a while back that you had stopped blogging when I dropped by your site and I sure can relate to the need to do so and of course, your reasons were different than mine but I could deeply understand why you did it. I was going to comment on a couple of your posts, but the comments were closed and you needed your peace and space. I figured I would just write that post of mine late last night, without much thought process or creativity in all honesty, to inform the masses of what many doctors are not saying to patients like me. As the first person to have a blog dedicated to this newer CXL surgery in the US, I figured I owed the world that much.

      I wish you a very sweet New Year. The sun has not yet set here, but Erev Yom Kippur is upon us and it has been a long, trying day.

      Shana Tova and Good Yontif to you, Shimon. May you be inscribed in the book of life, along with all of your feline friends. My beloved cat passed away in early 2015 from acute renal failure, but I still talk to her and miss her company and all she gave me in her 13 years by my side.
      I just couldn’t bring myself to write about her death, which I’m sure you understand.

      Much love from afar and thank you for the holiday visit,
      A (aka Leah) ❤

  2. I feel lucky to have found your blog just as you posted a recent article… but I’m sad it’s not with good news.
    I found your blog while looking for info about intermittent strabism and a possible link to keratoconus… and what a surprise to read you also have EDS. Well, should I really be surprised? Maybe not.

    I haven’t been diagnosed with the strabism, but people have started to notice my left eye going inward on some pics… and I’ve felt like I was cross-eyed even since my KC symptoms started, a bit over a year ago. As I’m sure you know with EDS, after a while we get pretty good at autodiagnosis, sadly, so I’m pretty sure we are not dreaming (and if it’s not strabism it’s something along those lines, and probably related to keratoconus).
    My KC is in fact post-LASIK ectasia, which is just a fancy way to say it’s not genetic keratoconus, but brought on by the surgery. Almost definitely because of the EDS, which I just had been diagnosed with at the time of the LASIK, and my doctor didn’t know the danger of… still not sure it would’ve changed anything. Would I really have said no to perfect vision for the off chance of a problem? Today I wouldn’t… but back then EDS seemed “the end” diagnosis. It explained problems I’ve always had and it had taken so long to get the damn diagnosis, I would not have dreamed I would get so many new diagnosis on top of it, develop so many comorbidities. So to have someone tell me I would be at risk of something more, something rare at that… I probably would’ve thought it was not really possible.
    Now I know if there is a small risk of something, I’ll get it.

    I started seeing fuzzy in late June 2017, eye doctor finally saw something in the fall, but wasn’t sure… I had my topography in March 2018 and a small KC could be seen. Got glasses in April to help (a bit) while they waited for a second topography, to gauge the progression and decide to do CXL or not… and now it’s much worse. I’d say I see as bad in the glasses as I was seeing without 6 months ago, and that strabismus started.

    I’ve been worried about the CXL from the start because of EDS. I’ve tried prolotherapy in shoulders, and it didn’t work, I’m guessing because the goal is to make the body produce new collagen… and ours is faulty. And isn’t that the same process with CXL? But when I told the eye doctor about that worry, she laughed (patients are so stupid, right?).
    I agree with you that it’s still better to try it, especially if the progression is there… but I’m worried.
    As I would be worried about a transplant. I would try it, like I tried a wrist surgery when most doctors wouldn’t want me to because of EDS, and it worked! I’ll soon undergo ankle surgery as well… sometimes we just don’t have a choice.
    But it’s worrying. And people without EDS or who don’t understand how it works can’t understand why it worries us so much.

    I hope you can find people to help you (I’m so, so sorry you don’t have help, it makes everything so much harder!), hope that you have your transplant soon, and that it goes well.

    You can always chat me up through my blog, but I understand how hard it is when vision is bad.

    Hang tight and good luck!

    • Salut Annie-Danielle!

      Sorry for the delay. I had to (dread) get a new phone after nearly 5 years and it was a real mess between my vision and the price and now my cramped-up hands from setting it up for hours. I actually wrote a post about getting the last one in early 2014 and my horrible anxiety (yes, so much worry due to our health about everything). Typing on my laptop helps my fingers so figured I’d come on here in the wee hours of the night and I found your comment! Btw, I bookmarked your blog and I do understand some French due to classes long ago. Yay! I speak Spanish (now rusty) as I lived in Mexico several times, but need to practice my French and I have been to your lovely city but back in the early ’90s. 🙂 I recall I could understand a lot of what was written in French back then due to my Spanish, but without any classes, I couldn’t understand a thing.

      I am so sorry to hear that you are also an EDS/KC hybrid (we can still call it keratoconus). A former doc called what you have secondary corneal ectasia and thought people who got it from EDS could maybe fall into that category, too. Who knows? I think we are not so rare, actually. We are totally misdiagnosed, like you said, and I was told I had fibro for 11 years before getting KC led me to see a geneticist in 2012. Strabismus of all kinds is more common in EDS so I think your assessment is likely correct. I have exotropia so my eyes go out (not in) and the worse the vision, the worse the turn. Exotropia, per my new doc, is seen with vision loss but one can have it without, of course. This 5th nerve palsy may have been around pre-KC but my great lens fitter (aka “my doc”) found it to be more common in EDS. So, something else to look for. My nerve palsy is mild, but the outward turn from KC (sans lenses) is very noticeable due to vision loss. It was apparently intermittent before the KC showed up in my late 30s (also very weird to get KC at that age).

      I have heard of post-LASIK corneal ectasia due to all my research. My sincere apologies as it is just like KC. My concern with those like us is I have never seen a mild case of KC in EDSers. It seems to progress and THIN like mad and I was worried even before I stopped blogging about stromal cell turnover post-CXL. The stroma is where CXL really makes the collagen fibrils nice and normal, but what about the cell turnover when my body starts producing faulty collagen and making new cells full of garbage collagen? That was my worry and it happens within a few years and I progressed again at year 4 and 5. Hmmm. No research on that one. Also, the thinness is what is making a transplant 6 yrs later (that eye was hit a bit later) a must as it will rupture soon.

      If you want CXL, I wouldn’t wait. I would honesty due it and just pray you don’t get teh neuralgia which has caused the bone-dry eyes. I could still see fairly well with lenses (and so-so without) even with corneal opacities from it (those are scars). We already have thin corneas due to EDS and one must have a corneal thickness above 400 microns for a safer, epi-off procedure. There are some drops they can use to plump things up for surgery, but CXL will thin you even more (happens to everyone) so I’m not sure I’m a fan of that. My KC specialist in LA didn’t want to re-CXL my left eye 2 years ago via the plumping route plus epi-on CXL (usually ineffective) so here I am 2 years later! Ugh.

      I’m glad you found me and I’m glad I found you, even if we are both a mess. I know socialized healthcare can be tricky as far as options go, but I fired all of my local eye docs and only see the best I can find now, just like with the other 99 specialists I have (haha). My corneal specialist is iffy as he doesn’t do the latest and greatest for transplants which makes me want to go to LA, but how? Yes, help and lots of money here in the US are a good thing! Otherwise, I’d take socialized healthcare any day! Anyway, I really owe it to my lens fitter who has figured all of this weird stuff out in the last 2 years. Strabismus is easy to Dx in office by quickly flipping a long spoon thing in front of your eyes to confuse them, btw. I have no idea what it’s called, but the spoon is the thing you hold over each eye while freaking out about all the letters you can no longer read. Lol. I also have written about scleral lenses somewhere on here (scroll to bottom I think) and you should be able to see pretty well out of them, but I’d stay away from standard RGPs due to poor fit and rubbing on a steep cornea. I presume sclerals would be covered by your healthcare system as they are very pricey. It’s trial and error to get them right and not all lens fitter can fit me. Best advice I can give on that. Also, if you don’t have it, try our national KC foundation for good advice and resources: http://www.nkcf.org.

      I wish you luck and I will likely blog again post-op about my ordeal (surgery is maybe around Dec 1st or so), so at least you’ll have a reference in case it comes to that, but I hope not as I am a ball of anxiety between the pain (can’t take opiates) and the light sensitivity for weeks to months (yikes) and then my wonky eye and terrible bruising. I have a broken blood vessel in my other eye today for no reason, as usual! My severe dry eyes and then EDS will throw a wrench it it all, though, so take some of what I write in the future with a grain of salt if you need info in the future. I have EDS, classical type, which is the type primarily seen with KC. I have never found any articles indicating it shows up in the hypermobility type (annoying names if you ask me), but the post-LASIK does make you wonder. Hang in there and thanks for stopping by! I’ll come visit your site in a few days (promise). Excuse any typos between my long day of lenses and my cramp-y hands *and* my mini-post in response, but it’s quite rare to meet someone with both of these awful things. 😦

      ((Câlins))
      A 🙂

    • This site is credible and explains how epi-off CXL works to strengthen the collagen fibrils (see Background). Everyone with KC or post-LASIK corneal ectasia has bad collagen fibrils, not just EDSers, so it seems good in theory. Hmmm.
      http://eyewiki.aao.org/Corneal_Collagen_Cross-Linking

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