Diagnosed with Eye Disease I Couldn’t Spell, Pronounce or Find Online: Vogt Koyanagi Harada Syndrome

Editor’s note: In observance of Rare Disease Day on February 28, Empish Thomas, VisionAware Peer, has written  this post about her rare autoimmune condition characterized by chronic, bilateral uveitis. An image of the parts of the eye most affected is shown below.

Parts of the eye are most affected by uveitis. image by National Eye Institute.  uvea is the middle layer of the eye which contains much of the eye’s blood vessels (see diagram). This is one way that inflammatory cells can enter the eye. Located between the sclera, the eye’s white outer coat, and the inner layer of the eye, called the retina, the uvea consists of the iris, ciliary body, and choroid:

Iris: The colored circle at the front of the eye. It defines eye color, secretes nutrients to keep the lens healthy, and controls the amount of light that enters the eye by adjusting the size of the pupil.

Ciliary Body: It is located between the iris and the choroid. It helps the eye focus by controlling the shape of the lens and it provides nutrients to keep the lens healthy.

Choroid: A thin, spongy network of blood vessels, which primarily provides nutrients to the retina.

Uveitis disrupts vision by primarily causing problems with the lens, retina, optic nerve, and vitreous (see diagram):

Lens: Transparent tissue that allows light into the eye.

Retina: The layer of cells on the back, inside part of the eye that converts light into electrical signals sent to the brain.

Optic Nerve: A bundle of nerve fibers that transmits electrical signals from the retina to the brain.

Vitreous: The fluid filled space inside the eye.
Parts of the Eye Most Affected by Uveitis

Getting the Diagnosis

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 where it comes from or 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 also experienced tearful eyes like you get from cutting up raw onions. Next came intense aversion to both indoor and outdoor light like a vampire. I kept the lights off and the blinds and curtains closed and when I went outside, I wore dark wraparound sunglasses keeping out all light from entering my eyes. Prior to this, I had never had problems with my vision and had no idea what was happening to me. I had just moved to Atlanta after college graduation to start my career and had no insurance, so I was reluctant to seek medical attention and relied on home remedies and over the counter solutions. Additionally, there was no real internet at the time and therefore no way to do a quick Google search. After about a week of these symptoms my roommate convinced me to go to the emergency room, because my vision was getting worse. Once there, the doctor tried to administer an eye exam but my eyes were too inflamed so he referred me to an ophthalmologist. I went there but they didn’t know what to do because they had never seen eyes in the condition that mine were in. That kind of freaked me out. They referred me to a specialist and that’s where I got the proper diagnosis and started my treatment.

What Is Vogt Koyanagi Harada Syndrome?

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 affect 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.”   

Eye Condition Considered a Rare Disease

I also learned that my eye condition is considered a rare disease or disorder, because only 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 doubt the number have changed much since 1995. Additionally, at the time I was hard pressed to find any information on VKH when doing research. The only good source I found was the National Organization for Rare Disorders, NORD which still lists 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, but on the other hand I was dealing with a rare medical condition that had no cure and was not common or top priority in the medical field. For National Rare Disease Day I want to shed light on my still rare eye condition and how it has impacted my life. 

Tried Various Treatment Options

When I was diagnosed the doctor couldn’t tell me where my condition came from or why I got it, only that I had it and there was no cure. We started a treatment regimen immediately. To stop the inflamation I started taking a steroid called prednisone as both eye drops and pills. I noticed an improvement pretty quickly. No more headaches, tearing, or sensitivity to light. I was not able to regain total 20/20 eyesight because I had suffered some damage, but my eyesight was pretty good and I could manage my life and go to work every day. This went on for a year or so, but the good times didn’t last because prednisone is a powerful 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. My doctor referred me to a specialist who had more experience with my eye condition and treatments that might work better than prednisone. The new doctor also began to reduce the dosage and saw the same problems. We tried other kinds of medications and even prednisone eye injections, but they were not as effective as the drops and pills and they had other kinds of side effects. During this time my lenses became infected and had to be removed. I ended up having emergency eye surgery where they operated on both eyes at the same time which is not standard eye surgery procedure. After surgery, I had to wear thick bifocals to see and I lost my peripheral vision.

Sought Vision Rehabilitation Services

Around this time, I sought help from a vision rehabilitation center for services. I learned how to use a white cane, adaptive technology and daily living skills. I also got assistance to keep my job and tools to work with my employer. 

Empish using white cane to cross street

Decided to Go Blind and Stop Medication

My vision only got worse with the detachment of both my retinas. So, one day I decided to have a heart-to-heart talk with my eye doctor. 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 and I wanted to be happy and healthy. I told him that I would rather be blind and healthy than sighted and sick. I felt I was going nowhere fast and I was young and had my whole life ahead of me. At the time, I was about 28 years old. I also had no idea of how all these medicines would impact my body long term and I was very concerned about that. My doctor told me that he had never had any of his patients go blind so he was not sure how things would go for me but he respected my decision. He started tapering me off the meds slowly. This resulted in withdrawal pain as my body detoxed from three years of daily steroid use. It was not pretty, but I made it through. 

Totally Blind and Living Well 

That was some 20 years ago. I am doing well and living my best life. I am totally blind and wear ocular prosthetics, but that is a whole other story! I am working from home as a writer and am healthy, happy and well. VKH Syndrome is still a rare disease that I still struggle to spell and pronounce; yet it’s easier to find online as well as its generic label, uveitis. Now when I search on the internet, I can find information. There is even the American Uveitis Society that has excellent information on the condition and a list of referring doctors for treatment. These things were not available to me back then. As advancements in medical technology and treatment increase, I am hopeful that my rare eye disease will become commonplace.