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.

Tagged , , , , , , ,


  1. I know it can be tough when doctors can’t give you exact answers. Hang in there =)

    • Thanks. It’s so frustrating to have a rare type of a rare disorder, not that the docs here know about any type of EDS! Lol. Keratoconus really isn’t that common in EDS and from the little research out there, it seems to only show up in type II and type VI (using old nosology). I was lucky enough to find out I had type II last year and only came across EDS due to getting KC. I spent 11 years thinking I had fibro due to the docs not having answers. Argh. Well, hope you could get through the Greek in that post!

      Hope you are hanging in there too!
      A 🙂

  2. dyspatient says:

    I keep hearing the Louis C.K. “sh****y ankle” skit as I’m reading your paragraph about your “effed up” eye diagnosis. I wish I could make it better. I hate that this loss is still happening.

    • Lol! I had to YouTube that skit and yes, that’s my entire life in general with doctors, but I don’t even get the scans. I’m the one who has to pay for them! I was getting wobbly, EDS ankles (speaking of) this winter due to those knitted, boot slippers that I grabbed at Wally World and was wearing non-stop (avoid those), not that I knew that was the cause until I put 2 and 2 together. I mentioned it to my doc last month, as my left hip and knee now seem to be going as well, and he pretty much just ignored me but gave me a packet with stretches for a sprained ankle on the way out. I can’t see the pictures and I don’t think stretching lax tendons is going to help the problem. :/

      I’ve been super depressed about my vision and it’s scary that I have no answers. After he gave me the “effed up” line, I just sort of sat and stared at him like, “Did you really just say that?” Jesus. No wonder I keep getting worse.

      Thanks for the laugh and reading through all the Greek of optometry–it’s been a super crappy couple of weeks. I hope you fared better with your eye appointment. (Btw, I’m posting that clip!)

      • dyspatient says:

        “You and your sh***y ankle…” totally love that. I liked Louis CK already but that officially elevated him to prophet level in my book.

        I would be super depressed about the vision too. No, my appointment was not good actually. I can push, but not now. Slight, very mild cataract. Eh, I knew that. Everything else, dry eyes, eff off. And treated like a criminal, you know the routine. They act like your being there is somehow an imposition. I actually heard the fellow and the attending laughing in the hall about EDS. Nice, huh?

      • Oh no re: your eyes! And laughing about EDS–that sounds like something that would happen here if they even knew what it was. So sorry! I’ve heard cataracts are more common in EDS, but I’m not totally sure on that. It freaks me out to no end with the (possible) elevated risk from the UV light used in CXL and having to have surgery on my eyeballs again with my severe dryness and just the fear of lens replacement in general with the crappy connective tissue and then how to get lenses right since they affect your VA. Oh, that is not good news at all. I hope you can find another doc–there’s always more going on but they never seem to find it until you end up like me.

      • dyspatient says:

        I know, it’s very frustrating to be the only one on your medical team who is like “hey this isn’t right….” Next time I need an eye exam, I’m going to a good community doc. Just need to do my research. I picked this clinic based on insurance ease, that’s what I get for wimping out on the research I guess.

      • I’m in the same boat, but out of docs. Now I have 3 who are just worthless! The dry eye one has one condition under control (meibomitis), but it appears I’ll never really produce tears anymore or something. I just read more and more about all the crazy, ocular issues we can have and my exam doesn’t even involve dilating my eyes, so how much are they really seeing. And what the hell is wrong with my vision?! I so need to get out of here, but am just stuck. Argh! I hope you can find a good doc–at least there are a lot to choose from out there.

Share your thoughts... If you are weird in a bad way, read my disclosure first.

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s

%d bloggers like this: