Living with Vision Loss

As a 12-year-old young man I was struck in the left eye with a stone while participating in a "friendly" neighborhood rock fight. You can say that I learned my lesson the hard way. Upon being treated for that injury, the physician noticed abnormalities in my retina. After further testing, it was determined that I had a rare eye disease known as Retinitis Pigmentosa. This was 1982.

Now it is 2010, and I am approaching my 40th birthday. After 39 years of living with a rare disease, I continue to learn first-hand about adjusting to the harsh reality of gradually losing your vision. That adjustment has been far from easy. Up until this very article, I have never written about this aspect of my life. It's time to change that.

According to the Foundation Fighting Blindness, Retinitis Pigmentosa, commonly referred to as RP, "is a group of inherited diseases causing retinal degeneration." Over the course of a lifetime, individuals with RP will notice different stages of progression, as they lose the function of retina cells called rods and eventually healthy cones in the retina. Retinitis Pigmentosa often begins with early childhood night blindness, graduating to a loss of peripheral vision in early adulthood, and sometimes resulting in legal blindness later in life. In some severe cases blindness can occur earlier. Exposure to bright lights and sunshine can accelerate the disease, so those affected are encouraged to wear protective sun glasses, and to stay out of sunlight for extended periods of time. The U.S. National Library of Medicine indicates that this genetically inherited affects about 1 in 4,000 people in the United States.

I guess you could say that I am one of the fortunate ones. My form of RP was not the severe type, as I made it through my early years with no real symptoms beyond night blindness. It's fair to say that most of my friends had no idea that I had an eye disease. I played sports, went to movies, hung out, and pretty much enjoyed the active life of a young person. The only time that my RP manifest itself was when we had night activities like hay rides, laser tag, or outdoor sports. Besides those occasions, I was not much different than my peers. I knew that I had a disease and that my ophthalmologist required me to come in every three months to test my visual field, but I had little understanding of the implications. I did not fully comprehend the impact of this disease that had been passed down to me. That would come later.

As a genetically inherited disease, Retinitis Pigmentosa runs in families, with parents and grandparents passing along these genes through the generational pool. Though it is not the only way to detect RP, one way that may shed light is to interview other family members for symptoms. In many cases you will find a parent or grandparent who is either a carrier or affected. There is not a single genetic defect that is classified RP, as this disease can be the result of a variety of genetic conditions. Still, it is worthwhile to pursue genetic testing, as this can provide information on the severity of the disease and the specific genetic strand.

A little after my 33rd birthday I began to notice problems with my left eye. I visited my doctor and explained to her that it felt like a part of my visual field in my left eye was "hazy". This was the eye that was injured years earlier and had always been the weaker of the two. She set up testing for me with a nationally recognized opthamologist in Philadelphia. After the hour-long marathon of very uncomfortable tests, I was informed that the RP in my left eye was progressing faster than that in my right eye. I was told that there was nothing that could be done medically to change this. I eventually began to notice subtle changes in my "good" eye. That was just the beginning.

According to the U.S. National Library of Medicine, "as the disease gets worse, side (peripheral) vision is gradually lost. The condition may eventually lead to blindness, but usually not complete blindness. Signs and symptoms often first appear in childhood, but severe vision problems do not usually develop until early adulthood."

As my vision began to fade over the next handful of years, I experienced a wide range of emotions. At first, I lived in denial. This couldn't be happening to me. I convinced myself that it would pass, sort of like a cold. As things continued to worsen, I became desperate, spending countless hours pouring over the Internet, joining a forum, looking for solutions. I read that the only scientifically verified treatment was taking high doses of Vitamin A. This was not a cure, as there is no medical cure for RP, but it would slow down the progress of the disease, buying me more time. I spent hundreds of dollars on bottles of synthetic Vitamin A. I was taking over 15,000 IU a day, which is well beyond the threshold of what our daily intake should be. My hair began to fall out and I would experience frequent headaches. As I further researched this treatment, I came upon information that seemed to warn against the use of high doses of Vitamin A. I decided to discontinue usage at the age of 38 years old.

The National Institute of Health says, "Controversial studies have suggested that treatment with antioxidants (such as vitamin A palmitate) may slow the disease from getting worse. Several clinical trials are in progress to investigate new treatments for retinitis pigmentosa."

Today, as I write this article, I am still blessed with a very productive life. Though my left eye is severely restricted, my right eye is okay. I can no longer play sports competitively, but I have great memories and I own several sports games for my Sony PS 2 (lol). I serve as a Middle School teacher in a public school in Florida, and outside of the occasional missed handshake, or clumsy stumble into a desk, I would say that the average student or colleague is not aware of my daily challenges. I can still drive a car, though that may be short-lived as the disease progresses. Most importantly, I've made adjustments, like learning to visually scan a room or new environment like a market. I have even adjusted my classroom for familiarity, and changed the setting on my computers to enlarge the cursor and font type. This has helped. I've also released my fears to God, and chosen to focus on what I have, instead of what I've lost. The bottom line is that I can still look into the face of my wife, watch the sun set, and find great joy in watching our young son grow. It's important to count your blessings. I genuinely enjoy my job teaching young people, and I dare say that I have lived a great life so far. I can still hope.

The Foundation Fighting Blindness says, "The Foundation Fighting Blindness (FFB) has funded many important RP research and clinical advances. A nutritional therapy using vitamin A and docosahexaenoic acid (DHA) has emerged as an effective treatment for many patients; gene therapies are progressing through preclinical trials; technologies for delivering therapeutic agents to rod and cone cells are being studied in Phase II/lll clinical studies; an implantable microchip to enhance retinal function is under development.

Living with RP is not something I've chosen for myself. You can say that it chose me. Yet, as I move forward with my life and continue to learn how to deal with RP, I make every effort to protect the vision that I still have. A summary of the research and real-life feedback that I've collected has convinced me to protect my eyes through use of sunglasses, implement a diet rich with cold-water fish like salmon, avoid high doses of vitamin E, practice emotional health by managing stress, and utilize any visual aids and techniques available. Though these are not cures, they do help. Finally, we can help those with RP is by informing others. Please join us as we support the many research opportunities that are being initiated as we search for a cure. You can make a difference.