Tag Archives: ghosted vision

A FALSE ALARM, BUT KEEP YOUR SEATBELTS SECURELY FASTENED

Image Source: http://www.thesun.co.uk

I don’t like to fly.  I get extreme anxiety and have had full-blown panic attacks at every elevation imaginable, even after taking a fear of flying course nearly 20 years ago.  So, when I went to see my optometrist a couple of weeks ago to find out why my vision was worse and more ghosted in my right eye, I had that fear-of-flying feeling the whole time: being completely out of control and waiting to crash and burn at any second. After all, if I’m not holding that plane up, then who is?

As usual, an assistant had me read the Snellen chart—a useless tool for assessing the visual acuity (VA) in anyone with keratoconus (KC).  I just wanted to know how much my VA had changed in my right eye.  Well, this woman didn’t want to tell me, but I do know that the VA in my left eye was unchanged because I turned around and read it on the wall when she walked out and saw 20/60 written next to the letters. I couldn’t see that while staring straight at it in the mirror. Again, the Snellen chart is worthless and there is no way I really have 20/60 vision in my formerly better eye pre-op and then formerly worse eye post-op.  I didn’t get near that line with my right eye, so I believe I lost around 4 lines of vision over a month in that eye.  I’m sure that sounds impossible, but I was progressing so quickly pre-op that it really is possible.  I had that awful feeling of sitting in a plane knowing we would be taking off very shortly.

Next, I had to have the dreaded corneal topography done, as that is the gold standard for diagnosing and tracking KC.  I looked into the machine and stared at a tiny hot air balloon that I saw multiples of with each eye while a photo was taken. My fate was sealed, just like when they close the door to the plane.

We headed back to the exam room and I waited for my doctor with my heart pounding.  What did the topography show? Was I progressing 13 months after collagen cross-linking (CXL)? Why doesn’t anyone research people who get keratoconus from Ehlers-Danlos syndrome?

My doctor came in and he knew why I was there as we had spoken on the phone, which is a rarity in the States.  He looked over a few things on the computer and then my topographies came up, but I couldn’t see them in detail.  I think he spent 20 seconds comparing them to my previous ones from this June, but it felt like 20 minutes and I could hear those huge, jet engines starting up: the clear sign of impending doom.

Finally, he spoke and said that there was no difference between my topographies.  I actually cried right there in the exam room out of relief, which is how I feel when a plane firmly touches down on the runway.  I was horribly embarrassed and it only lasted 5 seconds and I wiped my eyes and apologized. But, what was wrong with my eye?

My doctor checked my eyes and looked to see if the slight scar I have in the right eye was worse, but it wasn’t.  Another thing that can initially cause ghosted vision is a cataract, which may be a side-effect of CXL due to the UV light, but my lens was clear.  He thought everything else looked normal in both eyes, aside from the scar partially blocking my vision in my left eye from CXL.

He decided to do a refraction and apparently my prescription has totally changed since I saw him a few months ago, but not for the worse.  I’m still confused on what that means exactly, but that is precisely what he told me and I wish I had gotten copies of my refractions, but you have to pay for records.  I can still drive with my scleral contact lenses, but either due to the sun or the lights at night it’s really hard.  As I rarely wear them due to the pain, I noticed that my vision had gotten worse without my lenses in, which is the bulk of the time.

I told my doctor again my concerns about CXL lasting due to Ehlers-Danlos.  He told me that he had no idea about that or what was wrong and that I had surgery that totally effed up my eyes—that would be both eyes due to the prescription change and yes, I cleaned up his language.  What did he mean by “effed up my eyes?”  I believe he was referring to the fact that my corneas are changed at the molecular level, not truly effed up, but maybe a casual relationship with a doctor isn’t as great as I thought.  He said that maybe my left eye had gotten better, but I’d been doing the cover-one-eye-and-then-the-other test for weeks and just looked at him like he was yet another inept doctor in this city.  The free fall was starting.

I can’t even read on my hugely blown-up laptop with my right eye, so that is the one that is worse.  That was my better eye 6 weeks ago and I was also right eye dominant due to that, so my visual acuity was based on that eye which used to read the 20/40 line with great difficulty, but previously read 20/100 pre-CXL.  I am now left eye dominant as my brain is trying to compensate for the vision loss in my right eye.  Do I need to have fancy initials after my name to figure that out?

Again, the Snellen-based VA means nothing with KC as you can get a driver’s license with 20/40 vision and I flunked the test almost 2 years ago when the KC was starting in my left eye and I could read 20/30 from that eye without correction. I got a license as I could get enough correction with glasses in my better eye to read it back then, but I have a huge restriction stating that I am blind in my right eye, which was at 20/60 at that time: my current VA.  Legally blind is defined as 20/200 in the States; I rest my case on the idiotic Snellen chart.

So, my optometrist doesn’t know what is wrong or if the CXL will continue to halt the progression of keratoconus.  Oh, he called it keratoconus three times so I’m going back to that. A Google search for “secondary corneal ectasia from Ehlers-Danlos syndrome” yields zero results, unless my posts come up, so keratoconus it is.  Many disorders are associated with KC and until the medical world catches up and comes up with new names, I’m staying in the KC club. My topographies give a diagnosis of keratoconus and the big specialist who did my CXL had no clue I had Ehlers-Danlos syndrome, although I did mention the fact that I thought I might have it and he told me to see a geneticist.  If the only real difference is the typical age of onset and the lack of disease burn-out, I’m sticking with keratoconus and not changing my tag line.

What is wrong with my eyes again?  My vision is a bit better today so I wrote this post.  Who effing knows?  The plane crashed and there are no survivors.  I’ll post an update when they recover the black boxes.

Where all the answers are.

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

dominos-1

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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