IMPENDING DOOM: MY VISION IS WORSE & ALL ABOUT MY PSEUDO-KERATOCONUS

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Photo Source: 40ans.ehess.fr

I was in deep thought about my collagen cross-linking (CXL) surgery awhile ago.  I’ve read every journal article online about epi-off CXL, poured over the interviews with the top surgeons and their speculations on whether CXL will last in the long-term for keratoconus (KC), and thought about everything my top-notch surgeon told me at my pre-op appointment about the efficacy of CXL.

Since epi-off CXL was first performed in Dresden, Germany in 1998, the studies have shown there is no progression of KC and in the small percentage of people who don’t benefit from CXL from the get-go, it is effective when the surgery is repeated. This abstract from 2006, while not long-term, demonstrates the halt in progression.  So, why was I worried?

Well, it wasn’t until I found my great optometrist this summer that I learned that although I look like I have KC per my scans and tests, my vision is that of someone with keratoconus, and despite all of the medical literature stating that keratoconus, a primary corneal ectasia, or thinning, can be caused by Ehlers-Danlos syndrome (EDS)—I really have secondary corneal ectasia caused by EDS.  Should I change the tagline on my blog? As my optometrist stated, “It’s the same thing more or less.”

Per the “more or less,” here are the differences:

The true cause of KC is not known but there is a connection, at least in part, to faulty collagen in the cornea, but KCers don’t have a systemic disease that causes faulty collagen throughout the body like EDS does.  A dry abstract discussing collagen fibrils in KC.

KC tends to show up in one’s teens or early 20s and runs a course of progression before burning out—usually within 20 years.  With my secondary corneal ectasia, which apparently has never been researched, there is obviously no burn-out cycle as my body will continue to produce faulty collagen V for the rest of my life due to EDS type II.  Therein lies the big problem, or so I think.

With CXL, the weak collagen fibrils in the cornea are tightened up by creating cross-links, much like adding rungs to a ladder. That sounds like a win-win for true KCers and for me.  By creating all the cross-links, the cornea is stiffened and aged in essence (see abstract), which burns out the KC as cross-linking and stiffening are naturally occurring processes in normal, aged corneas.  That’s good for KCers and in my mind, has no affect on my ectasia with no burn-out cycle and continued production of faulty collagen.

So, why all the worry again?  I began to think about cell turnover.  Most of the cells in our body turnover in time. What would happen to those nice, cross-linked collagen fibrils when the cells in my cornea, or within the stroma more precisely, turned over?  With true KCers, I don’t think it matters as the disease has burned out, but for me, I believe that all the good collagen fibrils will be replaced by the old and it will only be a matter of time before the pseudo-keratoconus—what I’m calling this disease—shows up again.

Then, what are the options?  I see none.  When KC is severe enough, corneal transplants are the only option, but they reject sooner or later and the healing process is very long.  I also have abnormal connective tissue due to EDS, which would make finding a match rather difficult, but that’s an assumption. Lastly, transplanted corneas in KCers who are still progressing can occasionally develop keratoconus again since the disease process is still active.  I believe that would be a huge cause for concern in my case due to my faulty collagen production, not to mention my healing and scarring issues from EDS which would make transplants so problematic.  A single case report highlighting the recurrence of keratoconus post-transplant.

Now, I was just thinking about all of this and then I noticed my vision got worse all of a sudden, rather like when I first noticed the psuedo-keratoconus.  I thought it was from the sclerals I have to wear a few times a week to drive and that it would go away in a couple of days, per usual.  That was about a month ago, so it’s not from wearing the sclerals.  I don’t have any symptoms aside from more ghosting in my right eye, which was my better eye post-CXL.  Now, my left eye with the scar is the better eye and my brain has made that the dominant eye. As the vision in my right eye is worse than the left, it means I’ve lost more than 2 lines of vision in a month or so, which is how this whole debacle started by losing a line every 4-6 weeks on the Snellen chart.

I did have a lot of testing at my 1-year follow-up in late September for the FDA clinical trial, but due to my corneal specialist being the worst ophthalmologist known to humankind, he came into the room for 30 seconds and didn’t say a thing.  The reports, which are required by the FDA, never seem to make it to my surgeon in L.A., so who knows if anyone even reviewed them.  No one told me anything was awry and I had no reason to suspect anything at the time.  I do remember that my VA was the same, not that they’re very accurate at that practice or that it truly represents keratoconic vision.

I spoke with my optometrist about my eye and my concerns and have an appointment next week for an eye exam and corneal topography to see if there is progression.  The CXL did work as I got 6 lines of vision back, which is very rare as most get 1 to 2 lines back or none at all.  I do know that the cell turnover of the collagen in the stroma is several years and I’m shy of that at about 1 year post-op, but I have no idea what else it could be unless the very mild scar I have in my right eye that’s been stable for 8 months suddenly went wild, in which case I still won’t get my former vision back, and that’s all I want right now, even though most people would be crying in the corner if they had to see the world through my eyes.

I wish I could just get a break and I had to pay so much money for CXL that I didn’t have—I’m sure more than most Americans have in their savings account.  When I had the talk with my optometrist about my concerns with CXL, EDS, and the pseudo-keratoconus, he agreed and said, “It’s a whole other monster.”  Why didn’t he say that the first time? Maybe they could state that in the medical literature: that a condition that looks, but doesn’t exactly act, like keratoconus can be caused by EDS and it’s a whole other monster, rather than stating that keratoconus can be caused by EDS, period.  Perhaps the inept corneal specialist who has M.D. after his name could have told me that after I was diagnosed with Ehlers-Danlos syndrome.

I also found out from my optometrist that if I had had an EDS diagnosis in September of 2012 when I had CXL, instead of in November, that I would have been excluded from the clinical trials.  That’s doing wonders to quell my nerves while I sit and twiddle my thumbs and ponder writing a letter to the German ophthalmologist who developed CXL to get his take on its efficacy on my pseudo-keratoconus.

Theo Seiler, M.D., Ph.D., the creator of CXL who also looks exactly like my late grandfather.  Hmmm…

Photo Source: http://www.meaco.org
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19 thoughts on “IMPENDING DOOM: MY VISION IS WORSE & ALL ABOUT MY PSEUDO-KERATOCONUS

  1. Actually, why don’t you write the guy??? I’m sure he’d be more than interested in your case. Plus you are so smart it’s scary – and he’s probably smart too – and I’m sure would love to speak to you.

    • Thanks Sheep, but I think Dr. Seiler is way too busy for my issue and he’s beyond smart as he created a treatment for an incurable eye disease, which makes me look like I have a low IQ here. He’s in Zurich now and presents at conferences all the time and they’re running trials for CXL with other ocular diseases. What really irritates me is my surgery was done by the top keratoconus specialist in the US and he knows the docs in the EU and travels to the conferences, but due to my idiotic, local doctor not doing X, Y, and Z, I feel the relationship is strained and it’s not my fault that reports weren’t sent or that I had to contact my surgeon for all the odd complications that my doc wouldn’t even attempt to treat. Most people see their surgeon for the follow-ups, but I didn’t have the luxury since he’s in another state and I didn’t have any way to get there or the ability to pay cash. There was a verbal agreement that my local doc would do my follow-up care and he dropped the ball. So, here we are and I have more faith in an optometrist who did a fellowship in ocular disease than an ophthalmologist who is a corneal specialist. Well, as mentioned, if it has progressed, CXL is a moot point now. Sigh…
      A

  2. dyspatient says:

    Aw geez. Sounds like you’ve fallen victim to heuristics. “If it looks like a duck…” But in your case, it’s not a duck, it’s a zebra. The lack of awareness of what EDS is rears its ugly head again. I hate that you’re surrounded by incompetent doctors and that these are who must safeguard your vision. :/ I wish I could put you on a plane up here. I know there are crappy docs everywhere but you seem to have a real epidemic out there in the wild southwest.

    • Thanks D. It’s just so worrisome and my doc doesn’t know why I’ve lost so much VA in 1 month, but my Rx has totally changed and this is not normal. I don’t know if anyone in your area would know as no research has been done, but I’m sure I wouldn’t be dismissed without an explanation of some sort. Ugh! I called my surgeon over a week ago and no call back. I had to contact him every time there was a post-op issue due to the corneal specialist not knowing what to do and think he’s tired of me. What pisses me off is that I paid so much money for this surgery and the docs who do CXL and other surgeries not covered by insurance are making so much money–like they found a loophole in the insurance game. I think he has an obligation to follow-up with me since I did all the follow-ups for his clinical trial AND I mentioned my concerns with EDS to him in my pre-op exam, so he knew it was a possibility. He’s the one who said that he’d only seen a handful of KC patients like me and also that it was worth seeing a geneticist. He still did the bilateral surgery and how would I know it’s not true KC? The optom was calling it KC again when I saw him because it is, but isn’t at the same time: beyond a zebra here!!!

      Anyway, I’m just so frustrated with my vision. I could only blow things up a little more on here and I was able to adjust the font size, but it’s so faded and ghosted that I’m worried my days on the web are coming to an end. No!

      • dyspatient says:

        He does have an obligation to follow up with you. Failing to do so is physician abandonment.

      • Hmmm. I looked that up as it seems like doctors around here just drop off the map all the time, although this was the surgeon in LA. So, the last time I contacted him, he said I would have to see him in LA, which isn’t possible and he’s extremely expensive (plus how to get there, renting a motel, etc.). Come to think of it, that was over the opacity issue in March or so that my local doc refused to treat until I gave him the journal article written by my surgeon. To confuse matters, my newer optom said the corneal doc had the right to refuse treatment and refer me to my surgeon in LA, which does seem to make sense now with that law if it’s beyond their scope of practice, but again, the local MD is a damn corneal specialist. Argh! Anyway, that was the only time I contacted my surgeon post-CXL–all other communication was via his assistant and when she ignored my last e-mail this spring or so which was like a cry for help due to the fiasco here, I was just done. I forgot that I was e-mailing her and not him (although anything medical was forwarded to him), but I did call 2 wks ago and left a message for HIM and nothing. I think I got confused as we had several e-mails before CXL. So, I think he’s within the law if I can see him in LA (right?), but should have returned my call and I really don’t feel I need to go to LA when all the tests where done here, especially due to my circumstances. My optom could easily send my refraction/scans/notes and he could advise him if he even can as to what is going on. I believe people with EDS are getting CXL in the EU (etc.), I just can’t find them online and if they’re males, they probably don’t know they have it. As my surgeon is a top KC specialist in the US, he would have some insight. Again, what I have gathered is that with my KC friends in the EU, the docs share info with each other and here, they don’t. Back to the crappy, for-profit system again.

        Thanks for the tip, but what to do if the doc is in another state and I’m too sick and don’t have the means to pay cash and travel? Maybe they should teach compassion in medical school. Sorry for the long reply… :/

      • dyspatient says:

        They definitely need to focus more on the “soft skills” of doctoring in med school. I was just talking to a good friend about this. We proposed that there should be an entire “medical mystery” rotation, where you see patients day after day with difficult to diagnose problems. And where you have to keep a journal about how you react to them. I have found it’s such a key point of departure in care, both professional and compassionate, when a doctor doesn’t know what to do. I’m sure it raises all kinds of unpleasant feelings for them, and they should be encouraged (forced?) to encounter and examine those feelings during their training….before they are the last line of treatment for patients.

      • I agree! I also think that if you are not interested in patients with chronic illness and/or complicated cases, then you should not become a physician, period. Of course, with 15 minutes (more like 5), how can anything get accomplished? Argh! I just want to bulldoze the entire system.

      • dyspatient says:

        You’re so right. The ridiculous time pressure doesn’t help, especially if the encounter is one that brings with it feelings of incompetency, insufficiency, and helplessness for the doctors. Also not helpful, doctors don’t get paid for thinking and reading about a patient, just doing procedures and as many office visits as one can cram into a day.

      • You got it! That’s why I want to smack everyone who thinks a for-profit healthcare system is the only way to go. I’m sure it is if you’re healthy, but we’re the ones paying for this system that doesn’t meet our needs in the slightest. Argh!

      • dyspatient says:

        It’s such a sticking point for me. Yeah, add the people who get on their “you’re costing me money!” high horses to the list of people wanting a smack.

        When those people think about costs, they only think in terms of “healthy young people subsidizing” the financial, the premiums, deductibles, etc. “all you sickies!” It’s always short sighted and never includes outcomes, costs for repeat visits due to turfed patients, incomplete encounters, complications, progression of something that might have been treated more effectively if doctors had more compensation for time spent thinking….

      • Absolutely! I saw my PM&R today and had a 5 minute appointment, which also included dry needling and I really want to do that for the myofascial pain. They’re supposed to feel for all the knots and take their time when dry needling (had it back home once), but he just asked where it hurt and I rubbed my scalenes (starting behind my ears) down to my shoulders and upper back. Then, he randomly poked me maybe 15 times (hit a nerve once and my arm flew out) and was done–and he never even did my neck! It took me 45 mins. to drive there and I’m so pissed. So, that just cost my insurance and me a lot of money and for what? I’m also exhausted from having to get up and deal with it all and seeing that checkout girl who was so rude to me on the phone when I was 5 mins late due to an accident (last post). She tried to charge me nearly $100 for a no-show and I knew she was the one who was on the phone with me and I said that I had an appt. with the nurse and there’s a 10 min. grace period and they should have notified me that I’d be seeing the doctor, not that it mattered. If I get a 5 min. appointment and was 5 mins late, then he had plenty of time to see me before the appt with his attorney that he so needed to get to. I better not hear a thing about that charge again. Getting the bulldozer…

  3. It sounds like it would be a good idea to write him! And you’re so well versed in everything that you’d be able to keep up with him and comprehend the doctor lingo so he could just give it to you straight (instead of giving you the “dumbed down” usually less-than-helpful version docs will try to hand us before realizing we know as much if not more than them on those specific subjects lol).

    Sorry that you have so much to worry about! 😦 I may not have gone through or am going through the exact same thing in regards to eyes but this post and your worries still really resonate and hit home for me. So many of your thoughts and fears really echo my own.

    Anyways, what I’m trying to say is within the ramblings is; I do understand and am always here for you.

    Big hugs xx

    • Hey Katie,

      Well, I don’t know about writing to the world-famous doctor/professor. Sheep thinks I should do that too, but I think getting ignored by my rather famous surgeon in LA has ruined my self-esteem. I know the CXL guru is with a university in Switzerland now, but it would feel like writing to someone really famous at this point. Haha. I think the EDNF should get the scoop since he’ll take them more seriously.

      I do remember the nightmare you had with your eyes so early in life, so you do get it. Keratoconus is really not that common in EDS, nor is classical/type II it appears, so I feel like most people with EDS have no clue that you can actually lose your vision from this disease. Well, my good friends on here do since you are all so smart!

      Big hugs back at you and I will drop by this weekend after my eyes get a break–the endless appointments continue through Thursday.

      A xxx

  4. Ilian Andreev says:

    “So, why all the worry again? I began to think about cell turnover. Most of the cells in our body turnover in time. What would happen to those nice, cross-linked collagen fibrils when the cells in my cornea, or within the stroma more precisely, turned over? With true KCers, I don’t think it matters as the disease has burned out, but for me, I believe that all the good collagen fibrils will be replaced by the old and it will only be a matter of time before the pseudo-keratoconus—what I’m calling this disease—shows up again.”

    So why do you think it doesn’t matter for true KCers (like me)? Wouldn’t their collagen fibrils be replaced too after cell turnover? Or maybe their fibrils will be replace with healthy ones … I don’t understand please could you explain?

    Hope you’re doing good 3 years after this post.

    • Hi Ilian,

      I’m almost 4 years post-CXL now and no signs of progression, or so I think (I last had a topography about 8 months ago). Thanks for asking and I hope your KC is under control, as well. Per what I wrote, let me say I have no degrees in the medical field, just a mind that wanders. But, it would be true that everyone’s collagen fibrils turnover every few years. It seems that people with EDS–my genetic disorder–get keratoconus that never burns out but I can’t source that; I just believe that due to chatting with EDS/KC hybrids like myself who all ended up with horrendous cases of KC and that’s what got me worried as I developed KC in my late 30s and it progressed like crazy until I got CXL. It seems that normal KCers progress for about 20 odd years and then, as I’m sure you know, the disease burns out due to the collagen fibrils naturally becoming stronger via the aging process which naturally crosslinks them. In essence, CXL instantly burns out KC by toughening the cornea and Dr. Seiler, the German physician/professor who developed CXL, stated that he got the idea for CXL from tanning hide to make leather (yikes). I wish I knew where that interview was as it was rather fascinating. Does that make any sense? My worry is that maybe my KC didn’t truly burn out for good and since I’m still pumping out faulty collagen V day and night due to my EDS, who knows what could happen? My only hope is that some new studies indicate that a percentage of normal KCers probably produce faulty collagen in the corneas (only) due to a genetic defect, but that still leaves me puzzled on the burn-out effect. Do you have an ophthalmologist you can ask? I asked mine years ago and he got a worried look on his face. That’s never a good thing.

      Thanks for dropping by,
      A 🙂

    • I also don’t worry about normal KCers due to the CXL studies out of Europe that show virtually no progression of KC post-op and some of those studies are from the late ’90s. 🙂 I hope that helps.

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