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.
I had near-perfect vision less than 2 years ago. It was somewhere close to 20/20, aside from some mild myopia, or nearsightedness, that I had developed in my early 20s and did not even wear glasses for.
Then, the keratoconus (KC) developed and went wild and you can read my blog for that saga. I was at my corneal specialist’s recently as he wanted to see me for a 6 week follow-up after discovering central corneal opacity, or scarring, in my right eye months after my bilateral cross-linking (CXL) surgery.
Despite what I have felt has been rather negligent care, I got the full workup at the appointment: reading 2 eye charts, a refraction, a corneal topography, a corneal pachymetry, a check of my ocular pressure, and who knows what else I forgot. I was in a bad mood due to the disorganized clinic and was not really sure why I needed all the tests when I just had them done, but asked if I could at least have a copy of my topographies since nothing was initially sent to my CXL surgeon in L.A. for the clinical trial.
The techs are nice and had no problem with my request of having 4 papers copied, as opposed to the last time when I asked the front desk girl, who said she would have to charge me. Now, I finally had the scans I needed to see what my corneas and KC really look like at this stage, although my surgeon confirmed the other week that per my scans, the CXL was successful in both eyes and the progression of KC has been stopped in its tracks. Thank the Lord for modern medicine.
After all the testing and finally seeing what I could of those scans myself, the doctor rushed in talking a million miles a minute. He never seems to remember who I am or what my post-op issues are, which is really frustrating. He asked if I had any improvement since I had last seen him and I replied that I had some intermittent watering in my left eye—possibly due to the Restasis starting to work after 5 months—and that my right eye with the scarring had the same vision, which is a good sign, as well as the fact that the horrid light sensitivity (sun burning my retinas) was better, but not gone. He did a quick check of my eyes and said, “Good. Good.” That told me nothing, per usual, so I began with the questions that he hates, as he is already making notes in my chart at this point.
Here we go with the issues to preface all this. I read 2 eye charts—from right to left my visual acuity (VA) is now 20/40, 20/40 on one chart and 20/30, 20/40 on another. My left eye had gone up to 20/60 at my last appointment, so I was dealing with a post-op fluctuation that has cleared up. To err on the side of caution, I will say my vision is 20/40 with great difficulty on those last 2 lines, as no one has had me read the charts with both eyes, or binocular vision, which often gives a better VA. Would my true visual acuity not be of importance post-CXL? Well, of course, but I am dealing with sub par healthcare here. However, the big question is: why can I not see?
I should add that I do have a bizarre zone where I can see somewhat normally—it seems to start 1 foot in front of me and stop at 4 feet or so and there can be no sunlight or bright, artificial lights or the whiteout sets in. This is the safe zone and aside from a plasma-type TV on a not-too-far wall, I get staggered vision of everything outside that space in small rooms. For a description of how I see outside, in big spaces, and in the world in general—also known as the Ghosted Mess—refer to this post.
I have to ease my way into questions or my doctor gets irritated and the appointment ends, so I started with the basics. I wanted to know if the Restasis was causing the intermittent watering in my left eye, or if that was just from my corneas finally starting to heal from the surgery, as Ehlers-Danlos syndrome (EDS) is causing very poor and slow wound healing. The doctor could not answer that one. He kept saying my eyes were dry due to my meibomitis (MGD), which causes a lack of lipid (oil) layer in the tears, and that I just need more IPLs. I told him I had my last IPL 2 months ago and his associate—the doctor I like who specializes in dry eye—said more IPLs would do nothing at that point and I would not need a touch up for possibly a year.
I had 5 IPLs with no improvement that I noticed. However, the doctor thought they were successful in terms of the meibomitis, but as my eyes stopped producing the water layer in my tears post-op, they were still dry as a bone. Yes, this is all confusing, but for more information on MGD, as well as post-op dry eye, refer to this post. The corneal specialist gives me an odd look every time I explain that my complaints are related to my lack of a water layer and he says that I could barely open my eyes due to the meibomitis before the IPLs—although I only saw him once since 2010 and never had that symptom so I think he has me confused with another patient. Can I just see the dry eye specialist instead?
That leads to the big question—if my vision is roughly 20/40, why am I visually impaired? 20/40 vision is legal to drive in most, if not all, of the U.S. and I am not too sure I should even be on the road late at night, let alone left to wander in a big box store, which I would like to peruse with a stiff drink to offset the anxiety from the chaotic mess. Why do people look like shapes and colors and nothing more, unless they are in my safe zone? Well, the doctor is not sure, of course. What is he sure of? Why is he a doctor? Why is he the best corneal specialist in this horrid city? I may have better luck getting answers from the local fortune teller, who happens to be in the jenky strip mall next to my motel.
Now, I am not an ophthalmologist, but I am quite capable of interpreting my scans. Per the topographies, my better eye pre-op has no evidence of KC now in either the scan—showing no corneal steepening and 0% severity—or the K-readings, which are in the normal range now. My worse eye pre-op decreased in severity by 32.5%, although my K-readings are relatively the same in that eye, thus showing keratoconus—although it will not progress further due to CXL. K-readings measure the curvature of the cornea and there is still a significant steepening, which I can also see in the color-coded scan, but it appears to be much less severe, which makes no sense, especially since I got 6 or 7 lines of vision back in that eye. Both topographies do look quite different from my pre-CXL ones, which is rather fascinating. So, it would appear that I would have no keratoconic vision in my better eye, yet there is from my vantage point and I do not have my old 20/20 vision, regardless. May I also point out that at my last appointment, my doctor could not even tell me if the CXL had worked in that eye. Can I please move to L.A. with a giant dehumidifier and go see my surgeon for follow-ups instead? As mentioned, even I could interpret that scan with my impaired vision!
Back to the question at hand—my doctor asked when I would be seeing my surgeon again, as he seems to think I am able to go to L.A. at the drop of a hat—even though I would need an all day ride, have to pay a huge cash sum to see my doctor, and rent a motel room there, while living in one here. I reiterated for the 100th time that I have not seen him since my surgery in September of last year due to X, Y, and Z—not to mention that my local doctor referred me there and said he would handle all my follow ups. My patience was wearing thin.
Then, he asked how soon I could have laser vision correction surgery done there, even though that is my local doctor’s specialty (hence, corneal specialist). Is he crazy? I am 7 months post-op and a poorly healing, scarred mess due to my EDS and he thinks laser vision correction—namely PRK, which to my knowledge is still being studied for safety in post-CXL eyes (please fact check that), is a good idea? Due to the weak collagen structure in keratoconic corneas prior to the development of CXL, laser vision correction is contraindicated and is why all laser vision correction patients are given a topography to look for any evidence of KC before LASIK or PRK surgery. My eyes are so dangerously dry, plus the central corneal opacity that is no worse 6 weeks later but has not resolved, and he is even considering this? Furthermore, upon researching this issue just for the hell of it, the only scholarly, research articles regarding PRK following cross-linking involve or reference this ophthalmologist in Greece, who is on the payroll:
Financial Disclosures: Dr. Kanellopoulos is a consultant for Alcon, Inc., maker of the Wavelight excimer laser that he uses for topography-guided photorefractive keratectomy [PRK].
Maybe it is best to just see the local shaman wandering in the desert at this point. Perhaps some native shrub, like tumbleweed, can fix my vision—or is the local shaman in on the hustle to further destroy my corneas for some exorbitant fee, as well?
I told my doctor—who I now have absolutely no trust in—that there was no way I would go through surgery again with all the complications I have had and he mentioned something about my vision being bad from astigmatism. What astigmatism? Irregular astigmatism aka keratoconus? 9 months before my diagnosis, the optometrist I saw had misdiagnosed my keratoconus as mild astigmatism in my worse eye. Was this something new that my doctor pulled from the refraction done that day that I was not given a copy of? Add it to the list of copies I will be paying for very soon.
So, aside from residual keratoconic vision and little improvement in my K-readings in my worse eye, which I still don’t understand if the cone is so much flatter per the topography, is massive astigmatism in both eyes—the normal kind—contributing to the significant visual impairment now? If so, why can I not see any better with a refraction, just like when I had one before my diagnosis. I just answer, “Same” every time they flip the lens and ask, “Which is better—1 or 2?” Is the KC cancelling out any hope of lenses correcting the astigmatism? Why can I not get correction in my now non-keratoconic eye like my brother who has astigmatism and gets by fine with glasses? I am so utterly confused and Dr. Corneal Specialist is of no help.
As I have no choice but to wait and hope my vision improves a little more as my corneas continue to heal, patience has now been added to the survival box, and while I twiddle my thumbs, I will keep crunching the numbers in attempts to resolve the elusive mathematical equation involving my VA: 20/40 = X.
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.