Why does Ehlers-Danlos syndrome (EDS) have to be a freak show disease? My skin doesn’t stretch enough to be the India Rubber Woman and I’m not hypermobile enough to be the Human Pretzel, but I look like a hybrid of marbled, blue cheese and the Purple People Eater from the venous pattern and purpura and petechiae covering my body from nearly head to toe. The icing on the cake is the beyond frustrating keratoconus, which turned my corneas into little cones. Alas, the latest problems that have been discovered could surely guarantee me an act in the freak show.
I’ve been working with my great, new optometrist over the past couple of months to try and get scleral contact lenses, which can correct keratoconic vision to a point by creating artificial corneas in essence. As I’m post-CXL surgery, the progression of my keratoconus (KC) has been halted and my vision is stable, not that it means I can see well. The trial pair of scleral lenses had about 3 pluses and a lot of minuses, which I typed up in a list with bullet points for my doctor. He thought that was very amusing, but in a good way, and it actually helped to narrow down why I was having so many issues with the sclerals, aside from my dry eyes which will prevent me from wearing them daily it seems.
My doctor got down to business getting the prescription just right as it was a hair off and he can get my far vision a little clearer, although that is where things are very good for me with sclerals. I mentioned in my bullet points that I still can’t see well close-up and that while the T.V. image is crisp, it often has a double image, as do all the illuminated signs at night which makes it hard to drive still and is different from the multiple, faded images I see with keratoconus. My doctor thought that the better prescription would help, as sclerals work on both nearsightedness and farsightedness. I wasn’t that positive, though.
I had also mentioned in my list that the intermittent strabismus, or intermittent exotropia to be exact, that I developed when my brain shut off vision to my worse eye pre-CXL wasn’t gone as I suspected. It reared its ugly head again when I started inserting the new sclerals. As the contacts are akin to plastic bowls filled with saline solution, I have to use a rubber holder of sorts to put them into my eyes while looking into a mirror laid flat on the vanity with my head parallel to the floor. It’s really not that easy as the lenses are so huge and I have to hold my eyelids open very wide.
The right contact goes in well, but when I go to put in the left one, I can literally see my right eye turning out—see Figure B in the 1st image—and then my left eye that I’m trying to work with does the same! In other words, both of my eyes are turned out and it’s a little freaky to say the least. Now, I’m not making fun of anyone but myself here—I dated someone in high school who had exotropia and it wasn’t the intermittent kind. Regardless, a scleral lens has to fit over the cornea, so if my left eye has wandered to the far left, out falls the lens and saline solution and then I have to repeat the process 10 more times. Suffice to say, I’ve been a little late to appointments the last month.
My setup for scleral contacts lenses
It’s a very good thing I made a bullet point about that strabismus issue because my doctor did a couple of tests. He had me focus on a letter on the wall and then flipped something over my eyes in a rapid motion, so that every second I was using either the right or left eye to read.
“You do have strabisumus!” he told me, as he’s always amazed to have a patient who speaks doctor talk. Apparently, the test made my eye turn out as I started to lose vision in my right eye and couldn’t see the letter, only a blank blur. Yet, I can somehow make both eyes turn out when putting in the sclerals for some odd reason. That sounds like a freak show act if I ever heard one.
“Great,” I said, as I thought this was just some transient thing from having low vision for 2 years and thus some atrophying of my extraocular muscles, which control the movement and alignment of the eyes.
“Am I going to need surgery?” I asked my doctor. “You know about my scarring issues with EDS.”
He told me, “Not now at least.”
Then, he was on a mission and started rifling through my purse, which is fine as we spend most of the time joking like we’re old friends. I asked him what he was doing and he told me that he was looking for my phone. I grabbed my cell and he told me to type a text. So, I started to and he noticed how close I hold the phone to my face and he pulled my arms down. Well, that’s not fair. I knew he was trying to figure out my difficulty with seeing close-up and I mentioned that the screens on cell phones and my blown-up laptop aren’t horrible with my slcerals in, but print text was awful. That was in a bullet point, but I’ll cut him some slack since he’s my favorite doctor and knows all about EDS.
He gave me the paper I had typed up in 12 font at 200% in Word to read, which now looked very small and wonky. With the sclerals, the letters tend to move around a bit and make me feel sick; that’s the best way I can describe it, unless it’s very small text and then my head starts to hurt. He kept putting different lenses in front of my eyes and asking if it was better, but I kept saying that I didn’t notice a difference. I figured this was the best I would ever be able to see if none of the lenses over my sclerals helped. However, I must admit I like the rows of little lenses lined up in long drawers—it’s all so old-world and I got to wear the opera glasses again for a minute. You sure wouldn’t get any of this from the optometrist in the mall.
Then, my doctor had me look up and read the paper with the bullet points directly in front of me. He must have been watching my eyes because he had a rather deflated look on his face when he stopped me.
“You have convergence insufficiency,” he said in a rather sad voice.
“Noooo!” I whined. “No more diagnoses. What is that?”
He grabbed something and told me to watch his eyes while he brought it closer to his face.
“Do you see my eyes moving?” he asked me.
“Oh,” I replied. “Your eyes are moving inwards as it gets closer to your face.”
“Right,” he told me, “But your eyes don’t do that and they aren’t in perfect alignment in general, either—they both turn out a bit and all of that is causing double vision.”
“So, I have more vision problems than just the keratoconus?” I asked.
“Yes,” replied my doctor, “But convergence insufficiency is not that rare.”
More information on convergence insufficiency can be found here.
Per my eyes not being in perfect alignment, he tried to reassure me that it wasn’t noticeable, but I swear that one eye looked a little off when the KC first started. I demanded to know if I looked like Jaws from James Bond, and my doctor couldn’t believe that he was the image I associated strabismus with. Jaws actually had acromegaly and I don’t think he had strabismus. I should have said Marty Feldman, who was only in my favorite movie ever growing up and does the Abby Normal line I use all the time. Here’s the video clip. He had strabismus from Graves’ disease, but I see a resemblance, and it’s not just the eyes. He may have been a second cousin. How long until the freak show comes to town?
My doctor put lenses with prism in front of my eyes and I could see the text, but my head started to hurt. Prism bends light so that people with convergence insufficiency can see close-up and I could get readers made with them, but my doctor said they can be hard to get used to. Contacts and glasses are just too much right now.
I remembered that when I had the intermittent strabismus pre-CXL, I did some eye exercises I found online as I was so worried my wandering eye would prevent me from getting the surgery. I asked if that might help to tighten up my muscles, just like I do in physical therapy for my loose joints. My doctor handed me a plastic, Popsicle stick with a tiny E on it. He told me to focus on the E and pull it towards my face and then away, which I was able to do. I will now be doing this for 5 minutes every day, as it sometimes can help, but now I can’t remember with which issue, so hopefully it’s both.
My doctor also confirmed this could all be secondary to Ehlers-Danlos, but that maybe I had had it since childhood like most people. I never had problems with my near vision or any strabismus before keratoconus showed up, which is around the time my EDS started to affect more of my body, so I’m betting on EDS being behind all of this, or that and and KC combined.
I left with more health matters on my mind and then got a call from the assistant of yet another specialist I need to see as I was sitting in the parking lot of CVS. How many body parts can fall apart from faulty collagen? I went in to get my pricey prescription and got $5.00 in Extra Bucks—free money to spend at CVS for those not familiar with the chain pharmacy. I almost make money from all my prescriptions.
Then, I remembered I needed a Popsicle stick, but couldn’t find any, until I found the little freezer that had real, lime Popsicles, one of the last fruits I can still eat. Eating the lime Popsicle that I got for free in the desert heat made the bad news a little sweeter, especially if an employment opportunity where I can just stand while people gawk at me may be in my future.