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.
Being in a maze is chaotic. There is sense of confusion in your surroundings, an urgent need to find your way, frustration at the pure craziness of it, and there never seems to be anyone else in the maze with you.
This is how having keratoconus (KC) feels to me.
I know that a picture says a thousand words—and there are many computer-generated photos of keratoconic vision around the web—but does that really describe the reality of having keratoconus in its entirety? I don’t believe it does. Most people who don’t have keratoconus can understand what they see in a simulated photo full of ghosting or streaky, sparkly lights, but they can’t grasp a never-ending life of the distorted vision portrayed in a still photograph.
A keratoconic, blogger friend and I have been discussing all the oddities that go along with our vision lately. We have different stages of KC and my vision has improved in many ways as a result of cross-linking (CXL), but I still have keratoconic vision even if my visual acuity is much better than hers. We realized that keratoconus has never taken away our ability to see color, for example, although I have problems seeing clear objects and knock over water bottles at least twice daily. Why are they always full when that happens?
We also compared notes on what visual acuity (VA) really means when you have keratoconus. We decided it’s not the same as someone with simple myopia. For me, the lowest lines I can read are so faded and full of ghosting, or multiple images, that I get the lowest line I can see based primarily on knowing which letters my ophthalmologist doesn’t use. It also takes me about a minute to read that last line and I read the letters in no particular order. Starting in the middle and working my way out seems to work best for me. Go figure!
Then there is the big, scary world, which involves so many things the non-KC group takes for granted. Let’s begin with attempting to drive a car. This is akin to trying to navigate your way through a maze, but with massive anxiety. My doctor’s assistant told me yet again that I can read 20/40 with each eye—very questionable, so I’m technically legal to drive my car. With keratoconus, vision dramatically gets worse as the day goes on so most have trouble driving at night—the 1st symptom I noticed, but CXL stabilized my vision and I no longer have this problem.
My biggest issue with driving is due to post-CXL light sensitivity. If I am forced to drive during the day, it will only be to the eye doctor since the clinic is less than a mile away. I see multiple images of everything: other cars, pedestrians, buildings, signs, stray dogs, big trucks and buses, panhandlers, broken palm fronds, and whatever else is within my sight.
Due to this, I drive when it’s pitch black, but only once a week or so. There is literally less to see at night, so ghosting is less of an issue. I can stay in my lane, but I do feel I’m very focused when I drive, as if I were contemplating some abstract concept involving something I don’t understand, like physics. Good thing for the tinted windows to hide the bizarre expression on my face.
The bulk of my night vision is more like the mild stage of keratoconus. I can’t read street signs anymore, have trouble seeing where to turn to get into a parking lot, and the streetlights and headlights of oncoming cars are bothersome and have halos. They used to look like fireworks, so I try not to complain. It is absolute madness, but I feel I can safely drive at night now as I averted getting sideswiped by a possible drunk driver a few weeks ago. The Department of Motor Vehicles may dispute that, but I’m sticking by my story—and my 20/40 VA.
The most frustrating activity involves a store. I refer to this as being utterly lost in the maze and going down every dead end in attempt to find the exit. This would usually be the big-box stores that are so common in the US, especially in the wide-open SW, but even smaller stores pose a problem. The trick is to always go to the same store and memorize every aisle and exactly where every item you need is before losing any vision, but that isn’t always practical or convenient, especially when they decide to move things around in the store for no reason.
I will put this in perspective. I had to go to a beauty supply—a smaller store by US standards—for more products to put in my increasingly unruly hair due to the post-CXL steroid drops. I knew where the hair products were and I could easily identify what I needed by the shape and color of the container, since I can’t read the small print on most items. Maybe the price had doubled? Well, that’s part of the fun, of course. I also needed eyeliner. Why would I need that when I can’t see well enough to apply it with any precision? I really don’t know, but I could see just fine less than 2 years ago and know I look a hell of a lot better with makeup. I might look like a clown now, but no one has stopped me and told me so.
In search of the aforementioned eyeliner, I missed the aisle and ended up in the lipstick section, which I could tell was lipstick from the shape of the tubes. I headed to the next aisle and saw all the rows of colorful sticks. Eyeliner! Luckily, the brands are written on big signs and since I can see color, I grabbed my golden olive and headed to the cashier. 6 months post-CXL, I can now read the debit machines for some reason and can see money, but that was impossible before. I was about to start using singles to pay for everything like Ray Charles did to avoid getting scammed.
Another bizarre place is the center of the maze: sitting in a doctor’s waiting room. I’m not having to navigate or search for anything—I just have to sit forever and do nothing. I can’t read a magazine, after all. Unless someone is quite close to me, I can’t see their face and it’s an odd feeling. I don’t mean this in any offensive way, but everyone looks like the pre-surgery photos of the man who was the 1st face transplant patient. Just skin over a skull, except I can see the hair and skin color. Just Google it.
It’s in these waiting rooms—my home away from motel room—that the anxiety from driving dissipates, the frustration of trying to see through the distorted stores goes away, and I’m left all alone in a bubble of utter isolation. I feel that no one can see me, since I can’t see them. It’s as if I’m in my own world at these times—the crazy, maddening world of keratoconus that got dealt to me in that crappy game of poker.
It seems like it was just yesterday that I had bilateral, “epi-off” cross-linking (CXL), but I am just shy of 5 months post-op this week. My recovery has been beyond slow and rather atypical, so please don’t assume you will have the same issues. If you are having them and found my post, then great—there are more of us out there.
As I’ve mentioned in prior posts, I have an unusual form of KC that hits later in life and progresses extremely fast. Also note that thanks to keratoconus, which did have a silver lining, I was finally diagnosed with Ehlers-Danlos syndrome (EDS)—a genetic connective tissue disorder that presumably caused my KC and is also causing the slow healing.
Aside from the pesky dry eyes, which I wrote a whole post on, my biggest gripe at this point is with light sensitivity. It has slowly improved with time, but it just seems abnormal to be dealing with this problem still, although a fellow blogger who had her CXL around the same time as mine has the same issue still, too.
Following CXL, any light source was a problem and I saw sparklers and halos around anything that emitted light. Then there is glare, which I lump into the same category and is still a bit of an annoyance now. However, I recall not being able to even look into the silverware drawer the week I recuperated from CXL at my mother’s house. So, as long as I’m not dealing with glare from the sun, I won’t complain about trying to sort through my coins.
At this stage of the game, I have two big grievances with light sensitivity. The first involves using a computer due to the bright, white screen. For weeks after CXL, I couldn’t even open my laptop, but it’s still a nuisance now even if things are getting better. I’m not an IT person, but one of my fellow bloggers—who doesn’t even have KC—really helped me out with her comments. She suggested I adjust my screen’s brightness and other tips. Seriously, if you’ve never dealt with this, why would you think such an option existed on your computer?
Needless to say, one problem has been semi-resolved and I also learned I could blow up my entire operating system! That has been great since I can’t wear contacts still. If you have a PC, you can easily tinker around with your computer via Control Panel > Appearance and Personalization > Display (or at least that’s how it is in Windows 7 land).
My second issue involves seeing things outdoors during the day and driving. Regarding the latter, I can drive at night now although headlights are irritating, but I absolutely cannot drive in the day due to the sun. I live in the desert SW of the U.S. and the sun is usually a big ball of intense light, even though it’s freezing cold now. I get a “whiteout” effect—almost as if I were constantly driving west as the sun were setting.
The other odd thing is that while I’ve had this massive improvement in my visual acuity, I have typical, keratoconus vision with ghosting outside in daylight, which just adds to my reluctance to drive. This has improved drastically too, but before CXL, I could get one good drive in 1st thing in the day. After that, I shouldn’t have even been on the road.
I am still healing and my vision is still fluctuating a bit, but I have stable vision throughout the day due to the cross-linking and that is wonderful. I also am noticing that I am starting to be able to read some “fine print” on things, although if it’s too small I struggle and get a headache between my eyes.
While my visual acuity jumped to 20/400 post-CXL, I’m currently at about 20/40 in both eyes, although that last line is a struggle to read. Pre-CXL, I had monocular vision and was only using my better eye—now my slightly worse eye, which was at 20/60 and rapidly progressing like my other eye, which was no longer functioning. Sort of confusing. I wish I could explain it all in diopters, but the clinical trial follow-ups don’t seem to include a refraction.
So, aside from the horribly dry eyes, my hair that is still falling out from the steroid drops months after stopping them, and the light sensitivity and inability to drive a car during the day (huge problem!)—I am so glad I was able to max out my credit card and be one of the lucky few to get into a CXL clinical trial in the good ol’ US of A.
I need to “fail” a pair of contact lenses in order for my medical insurance to cover surgery for Intacs for keratoconus in my right eye. I was lucky enough to get in today to see my optometrist, just a few days after learning I needed this surgery. Of course, none of the records from my ophthalmologist’s office were sent, the receptionist never recorded exactly why I was coming in today, and I can’t recall exactly what else went wrong.
I was escorted back to the exam room after having more corneal measurements taken and I explained to the medical assistant why I was there and that I needed my optometrist to prescribe “the cheapest contact lenses possible” (repeating what the ophthalmologist said), as they were just going to go in the garbage since my eye couldn’t go much longer without surgery. She looked completely perplexed, but I don’t make up these ridiculous rules. Blame the insurance companies who are now paying for two things instead of one.
My doctor came in and I explained the whole situation once again. I was having Intacs in the worse eye and corneal collagen cross-linking (CXL) via a clinical trial in the better eye and I needed to “fail” a pair of contact lenses for my insurance to cover the Intacs (let’s repeat this one more time). I was a little worried that he’d think I was making him lie, but I had copies of my corneal topography—thank the Lord I thought to get those—and he could see my eyes’ progression since this all began about 9 months ago, or at least compare the current topography with my K-readings. He agreed that Intacs were the way to go and would help him in fitting me with the hybrid contact lenses that I will eventually be wearing, since my cornea will be so much flatter in that eye.
My doctor did an eye exam and refraction and prescribed a trial pair of soft contact lenses, but with a stronger prescription than my glasses. I’ve never worn contacts—and never wore glasses except for really long drives until this year, so his assistant taught me everything I needed to know about cleaning and storing them and how to put them in and take them out. I had no idea it was such a big deal and figured it was a good primer for the hybrid contact lenses I’ll be wearing in the future.
After that, I went back into the exam room to make sure the prescription and fit were fine. I wasn’t aware that I could see better, but I got to 20/25 on the eye chart with them in—using both eyes, and I’m at 20/50—using both eyes—with my glasses, which is no longer legal for driving. Yikes! However, everything close-up looked fuzzy and I still had a lot of ghosting on the eye chart. Ghosting is seeing the faint, double, or multiple, images of everything you look at. I could also really feel the contacts in my eyes, but my doctor said that would go away. Again, best to get used to it now.
Then I had to deal with getting samples of new eye drops since I have very dry eyes from a separate ocular condition I’ll write about at another time. Apparently everything I currently use will fog up the contacts, but I barely get relief from my “liquid gel” drops as it is and now I’m back to the “regular” dry eye drops. More frustration and I’d really like to just have my old myopia, or nearsightedness, back and wear my beautiful D & G glasses that cost a fortune a mere 6 months ago.
I headed to the front to check-out and asked what insurance this was going through. Vision. I said I thought these were “medically necessary” so my medical insurance would cover it at 85%. Negative. Well, there went close to $100 on a trial pair that provide better—but unstable—vision and are good for one month. Then I asked who would tell my medical insurance that I failed these. Oh, that needs to come from the ophthalmologist.
I asked for a copy of my records from today so that he’ll have it in writing. Today’s records were about as botched as my last two, since there is nothing about failing the contacts and the “plan” is to just get medically necessary contacts; it actually says “plan.” Sounds like he just negated what my insurance needs, but I sure hope it will suffice.
As I mentioned in my ABOUT section, I live in the desert SW and it was probably 110°F today and the sun/glare is a killer, whether you have keratoconus or not. So, I reached for my sunglasses, but forgot there’s a prescription in the lenses. What to do? Contact lenses in my eyes and now no sunglasses and it’s due west to get home at 5 pm.
Well, I did make it and noticed that things were clearer, aside from the blinding sun, which was odd. I came home and could see the TV for the first time in months and the computer is much easier to see, too. Of course, I have a stronger prescription and I could also see pretty well with my glasses prescribed 6 months ago before they failed me in less than 90 days, which is before anyone suspected I had keratoconus (can I get my $500 back?).
I see the optometrist at my ophthalmologist’s office in 3 days to get measured for Intacs and hope the insurance loophole can be cleared up at that time via the bizarre medical record I got today. This will also be the third time I’m getting my eyes dilated—sans driver—in 2 months and I’m not looking forward to it. I had the worst headache the last time, which I blame on the desert sun.
Now I wonder how I will be able to see after Intacs surgery since it’s only on one eye and isn’t a cure. Do I pay for another trial pair of contacts until I get the cross-linking in the better eye, which hopefully will be within 45 days? Once both procedures are done, I can get the hybrid contact lenses, but how do I manage in the meantime?
I think I need an Ocular Liaison if one exists. I also need a rich benefactor, as all this “treatment” is adding up rather quickly.