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.
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.
I saw my former ophthalmologist today for a corneal topography, which is the gold standard in diagnosing keratoconus (KC). My optometrist had diagnosed me with the disease on July 13, 2012, roughly 9 months after my vision started to fail. That was a Friday, so I suggest not going to a doctor on Friday the 13th.
General information on keratoconus can be found here.
My ophthalmologist is a corneal specialist and I feel comfortable putting my eyes in his hands, so to speak. I only wish I had not been shifted around in his practice, as I feel I would have gotten a diagnosis earlier and before my vision progressed to its current state. I ended up seeing him after killing myself—body and eyes—doing online research non-stop following the initial diagnosis last month. Forget the worst-case scenarios out there unless you have advanced KC. I discovered a procedure known as corneal collagen cross-linking (CXL) that is awaiting FDA-approval in the U.S.
CXL is approved everywhere but the U.S., per usual. This procedure stops the progression of keratoconus by strengthening the cornea and has been available in the EU (European Union) since 2007. After I discovered this, I had to find a way to get the procedure without flying to Europe since disability does not allow for the jet-set lifestyle. I scoured the internet and came upon some posts from people who were getting the procedure via clinical trials. I figured I would have to go to L.A., but to my surprise my former ophthalmologist was participating in a clinical trial and my prayers were answered.
If you are searching for a clinical trial for CXL near you, the U.S. government site is here.
So, today I not only went in for the official diagnosis, but also for the screening process to see if I am a candidate for the trial. I should also mention that since this is a treatment trial, there is a charge of $2,500 per eye, but each trial is different it appears. Well, what are my options really? The other bad news is that my doctor is not sure if I am a candidate as they still do not have clear exclusionary guidelines from the FDA, but he thinks my chances of being approved are at about 80%. There is a waiting period of 45 days per the guidelines and considering the fact that my vision is progressing for the worse so rapidly, we have decided to do the CXL—if I am approved—on my worse eye and I will be getting surgically implanted rings (2 semi-circles) called Intacs in my better eye. This will flatten my cornea and allow me to have better vision with glasses for now.
Due to my insurance, I need to fail a pair of contact lenses before I will be approved for Intacs surgery. So, I go back to the optometrist later this week to get fitted for contact lenses and then next week I see my ophthalmologist’s optometrist to get measured for Intacs.
If all goes well after I fail the contact lenses—my severely dry eyes will make me toss them in a day, the plan is to get CXL in the better eye after FDA-approval as Intacs will disqualify that eye from the trial. In other words, I will get CXL in my right (worse) eye to stop the progression and Intacs in my left (better) eye to improve my vision temporarily until I can get CXL in that eye once it is approved, covered by insurance, and the trial restrictions are a none-issue. If you are confused, so am I, but the bottom line is that I should be getting Intacs in my left eye within a month if I can get through the insurance loopholes in a timely manner.