How Not to See the World: Living with Keratoconus

by Jake Whalen

I approached the checkout counter in my local ShopRite as I had done a thousand times over the years. The beeps from items being scanned from a dozen checkout lanes bounced in one ear – and rattled around a few times in the void, my wife would add – and out the other. It seems we can’t escape those incessant sounds, as they’ve become part of the modern shopping experience.

But this particular experience was different because on that day, and all month long I’ve since learned, the checkout counter was lined with little pieces of white paper with “ONE DOLLAR” written across the top. I’ve seen these types of charity support cards many times before, and I’ve “purchased” a few on occasion, but this one was different. Not only did it concern children, which, for me, is the greatest concern on the planet, but it related specifically to eye and vision disorders, which I have experienced since I was nine years old.

It All Started around Age Nine

It was around that time that I realized I had a hard time seeing the blackboard in class and also began experiencing extreme sensitivity to light. Thus began my lifelong acquaintance with keratoconus, a condition which misshapes the corneas and, in most cases, is not correctable with glasses. Interventions include specialized contact lenses and corneal implants, and, in severe cases, corneal transplantation.

Editor’s note: Keratoconus is a bilateral (both eyes) degenerative condition of the cornea, a transparent dome-shaped tissue that forms the front part of the eye. Keratoconus gradually causes the cornea to thin, bulge/protrude outward, and become cone-shaped. This creates an abnormal curvature of the eye called astigmatism that can cause blurry vision, glare, or light sensitivity (photophobia). People with keratoconus typically begin to exhibit symptoms in their teens, and the disease gradually progresses over time. In the later stages, scarring can develop in the area of the corneal bulge or protrusion, which can further impede vision. Occasionally, people with keratoconus can develop hydrops, which is a sudden break in the internal surface of the cornea that allows fluid to enter the cornea and cause it to swell. This can cause pain and a sudden decrease in vision.

My First Prescription Glasses

Squinting at the blackboard seemed to rectify the situation, as did moving closer to the front of the class. The added benefit to the latter was that I could see Miss Maxon more clearly. She was, without doubt, the hottest teacher that this fifth grade student had ever laid his visually challenged eyes upon.

Unfortunately, Miss Maxon soon began to blur into that same blackboard and – while I wouldn’t lose any sleep over not being able to see the various types of triangles clearly – not being able to see Miss Maxon was clearly and completely unacceptable.

Naturally, I told my parents that my vision was affecting my school work. Within a few weeks, glasses were adorning my fifth-grade face so that I could get back to the serious matter of adoring Miss Maxon’s face.

For the next twenty years or so, changes in my eyeglass prescriptions desperately tried to keep up with the shape changes that were occurring in my corneas, but they finally lost that battle when I was 28. I was told that the keratoconus had worsened and that hard contact lenses would be the only option. Glasses would no longer offer any help.

My First Experience with Hard Contact Lenses

In theory, the contact lens doesn’t actually sit directly on one’s eye, but on the layer of tears that coats the eye. In practice, however, not only did my hard lenses feel like they were sitting directly on my eyes, they felt like an evil lab technician in a dark, dank lair had fashioned them from tiny pieces of sandpaper that rubbed across my corneas every time I blinked.

I was tempted to insert toothpicks into my eyelids to keep from blinking, but I suspected that my wife would object, particularly when we were out in public. I learned to just endure the pain and try to function as best I could. On most days, however, I felt like ripping the lenses out after just a few hours and lying on the couch for the rest of the day with a warm compress over my eyes. On more than one occasion, I actually did just that.

After consulting with several eye specialists, including those at the renowned Wills Eye Hospital in Philadelphia, all of whom conceded that life on the couch with a warm compress was most likely my future, a co-worker mentioned that she’d heard about “someone” in Harrisburg, Pennsylvania who had helped a friend with a difficult eye condition.

The Piggyback Lens

That “someone” was Dr. Robert Morrison, a renowned soft contact lens pioneer, who, upon reading my hastily-written and faxed letter describing sandpaper, toothpicks, and warm compresses, indicated that he believed he could help me!

His waiting room looked like the lobby of a Hollywood talent agency or a restaurant known for its famous patrons. There was even a photo of a Rolls-Royce on the dock in Philadelphia, given to him by a sheikh as a present for being the only doctor who had been able to figure out how to improve his son’s vision.

Dr. Morrison and his associate on Park Avenue in New York experimented over the next several years with hybrids, Rose K lenses, and other solutions until we finally agreed that a piggyback lens system, which involves wearing two lenses in each eye – a hard lens over a soft lens – provided the best compromise in vision and comfort.

Until the abrasions started.

Corneal Abrasions

For most of the population, for whom eye discomfort involves a wayward eyelash or some other benign irritant, I’m here to tell you that a corneal abrasion makes the aforementioned sandpaper scenario seem like a Swedish massage in comparison.

A healthy cornea is a perfectly smooth parabolic shape. A cornea with keratoconus is more like a Thomas’s English Muffin with nooks and crannies. Those nooks (or is it the crannies?) can come in contact with the contact lens and therein lies the rub – literally.

That rubbing action causes a corneal abrasion, which in turn causes pain so severe that men have been know to offer up their Sports Illustrated Swimsuit Issue to the eye gods for relief. Of course, no such relief comes until the abrasion heals. In my case, I would get one in my left eye and a few weeks later, another in the right.

Of course, in order to heal, I couldn’t wear the lenses; thus, I couldn’t see, sometimes for several weeks at a time. Not until those offending, malformed corneas were replaced with new corneas, via a corneal transplant, would it be possible to enjoy a normal life.

After putting up with this life-altering condition on and off for several years, the final straw occurred during the Masters Golf Tournament. I had abrasions in both eyes at the same time. Not able to wear any contacts, and in earth-wobbling pain, I was lying on the floor in front of the TV, a hot compress over my eyes, listening to the play action of Tiger Woods, occasionally peeking out from under the towel only when seeing another great shot was worth the agony. I called my doctor the next day and said “Tell me more about corneal transplants.”

Corneal Transplants

I had my corneal transplants done about a year apart and am extremely grateful to the donors. The most arduous part was going back in for the removal of the stitches which takes place gradually over time. An assistant prepared me for the doctor by putting a numbing drop in the eye. A few minutes later, the doctor came in, measured the pressure, and determined exactly which stitch or two needed to be removed.

He held his knife in place next to the stitch, touching my eye, and then would tell me to look in a specific direction. Upon his three-count, I moved my eye and the stitch would be cut as it passed over the knife blade. He would then remove the stitch.

One time an assistant accidentally handed me lens cleaner, which was NOT intended for use in the eye. Imagine pouring Tabasco sauce in your eye! Mine literally felt like it was on fire and I swear on all that is holy that I would have tried to put it out with an ice pick if one had been within reach.

I’ve not had any more abrasions since the corneal transplants about ten years ago. The vision and sensitivity issues remain, however, and I’m still wearing hard lenses. I can no longer drive safely at night on dark roads, and overhead fluorescent lighting – the kind found in most stores – is quite uncomfortable.

I have tried hybrid contact lenses, but they were brittle where the hard component meets the soft part, and they kept splitting. My vision was good, but I found that it was just as good and comfortable with the piggyback lens system. I’ve never tried scleral lenses.

The hard lenses are not that bad. I’ve got great vision and they’re not that uncomfortable. Yes, I have visual fatigue and at times I most certainly wish I could pull them out, but after 25 years, I’m more or less conditioned to just deal.

Editor’s note: Hybrid contact lenses contain a soft and hard contact lens component. Scleral lenses are larger-diameter gas permeable contact lenses that cover the entire corneal surface and rest on the sclera, which is the white part of the eye.

I’ll manage as I’ve always done. It’s been said that the eye is the most spectacular device in all of God’s creation. I think He must have been so impressed with this beautiful world He put us on, it was His desire to give us the ability to see it for ourselves.

Additional Information on Keratoconus