Editor’s Note: Feb 28 is National Rare Disease Day and I am reposting an article I wrote for VisionAware on my rare eye condition and how it caused my blindness.

What is Vogt Koyanagi Harada Syndrome?

I am sure you have heard of common eye conditions like glaucoma, cataracts and diabetic retinopathy. You are probably familiar with Age-Related Macular Degeneration  that impacts our older population. But have you ever heard of Vogt Koyanagi Harada (VKH) Syndrome? Do you know how to pronounce it? Where it came from? How a person gets it? Well, neither did I when I was diagnosed in 1995.

The discovery started out so strangely with violent headaches like someone was hammering on my head non-stop. I experienced tearful eyes like you get from cutting raw onions. Next came intense aversion to both indoor and outdoor light like a vampire. I kept the lights off with the blinds and curtains closed. When I went outside, I wore dark wraparound sunglasses avoiding all light from entering my eyes.

I never had problems with my vision in the past. I had no idea what was happening to me. I had just moved to Georgia after college graduation. I was starting my career. I had no medical insurance at the time. So, I was reluctant to seek medical attention and relied on home remedies and over the counter solutions. Visine might get the red out but it wasn’t working for my situation. Additionally, there was no internet. Therefore no way to do a deep Google search.

VKH Syndrome Explained

After a week, my roommate convinced me to go to the emergency room. My vision was not improving and getting worse. The doctor tried to administer an eye exam but my eyes were too inflamed so he referred me to an eye doctor. I went there but they didn’t know what to do because they had never seen eyes in the condition mine were in, which kind of freaked me out. They referred me to a specialist and it was there where I got the proper diagnosis and started my treatment. I learned that VKH Syndrome is an autoimmune disease characterized by chronic, bilateral uveitis.

The National Eye Institute says that “uveitis is a general term describing a group of inflammatory diseases that produces swelling and destroys eye tissues. These diseases can slightly reduce vision or lead to severe vision loss. The term “uveitis” is used because the diseases often affects a part of the eye called the uvea. Nevertheless, uveitis is not limited to the uvea. These diseases also affect the lens, retina, optic nerve, and vitreous, producing reduced vision or blindness.”  

I also learned that my eye condition is considered a rare disease or disorder because about 1.5 to 6 per one million patients are diagnosed in the United States. This is a current statistic from the US Department  of Health and Human Services  but I would dare to say the numbers haven’t changed much since 1995. Additionally, I was hard pressed to find any info when doing research. The only good source was the National Organization for Rare Disorders, (NORD). They still list my eye condition today in their database. The discovery was bittersweet. On one hand I was glad to find a trustworthy authority besides my doctor. However, on the other hand I was now dealing with a rare medical condition that was abnormal, had no cure  and not top priority in the medical field.

Tried Various Treatment Options

When I got my diagnosis the doctor had no explanation only that I had it and there was no cure. We started a treatment regimen immediately. I was on steroids called prednisone, both eye drops and oral pills, to stop the inflammation. Improvements came fast. No more headaches, tearing or being a vampire. I didn’t regain total 20/20 because of nerve damage but my eyesight was pretty good. I could manage my life and go to work.

This went on for a year or so. However, the good times didn’t last because prednisone is a super strong drug with awful side effects and I had pretty much all of them. The goal was to reduce and eventually get off the medication but each time my doctor reduced the dosage my vision decreased. We tried this schedule multiple times with no success. In the meantime, I was suffering from violent mood swings, ravenous appetite, hair loss, major weight gain, and rapid facial hair growth.

He referred me to a more experienced specialist. The new doctor also saw the same problems. We tried other kinds of medications and even prednisone eye injections but they were not as effective and had other kinds of side effects. My lenses became infected and had to be removed. I ended up having emergency eye surgery where both eyes were operated on at the same time. This is not standard eye surgery procedure. After surgery, I had no peripheral vision and only saw blurry blobs. I  wore thick bifocals   for clarity and focus. Around this time, I sought help from a vision rehabilitation center. I learned how to use a white cane, adaptive technology and daily living skills. I also got assistance to keep my job with tools for the workplace.

Decided to go Blind and Stop Medication

Yet my vision only got worse with both retinas detaching. It was time for the hard talk. My doctor and I had a heart-to-heart discussion. I told him I was ready to let it all go and let the eye disease run its course. We both knew that my vision was not improving even though I was taking several medications and the side effects were getting worse. I wanted my life back. I wanted to be happy and healthy. I told him that I would rather be blind and healthy then sighted and sick. I was going nowhere fast and I was young and had my whole life ahead of me. At this time, I was about 28 years old. I also had no idea how all these meds would impact my body long term. I was worried about my future.

My doctor said he never had a patient go blind. He was unsure how things would go yet respected my decision. He started tapering me off the meds slowly. This resulted in withdrawal pain that I had to endure. What was initially supposed to be 6 months of medication ended up being 3 years. That’s a lot of chemicals in your body. Mine was detoxing. It was not pretty but I made it through.

Totally Blind and Living Well with This Condition

That was over 20 years ago. I am doing well, living my best life. I am totally blind and wear ocular prosthetics. But that’s a whole other story for another day! I am working from home as a writer and am healthy, happy and well.

VKH Syndrome is still a rare disease I still struggle to spell and pronounce. Yet I know more  about it. It’s easier to find online as well as its generic name, uveitis. Today when I search on the internet, I can find information. There is even the American Uveitis Society that gives all kinds of good stuff on the condition and a list of referring doctors for treatment. This was not available back then. As advancements in medical technology and treatment increases, I am hopeful that my rare eye disease will become common place.