My collapsible arm rests against the front door. I only use it outdoors and in unfamiliar territory, never at home. The soft glow of the computer monitor is the only illumination in my spacious, one-bedroom apartment. The screen, however, doesn’t hold my attention as I write this. Rather, Lisa is helping me with the spelling and grammar while those keyboarding classes I took in high school are guiding my fingers. Lisa, along with that extra appendage by the door, is now fixtures in my life because of recalcitrant illness, its treatment, and its fallout.
I had been diagnosed with juvenile rheumatoid arthritis (sometimes shortened to JRA) when I was five years old. Arthritis occurs when the body’s own immune system mounts an unprovoked and unwanted attack on tissues and organs. Often the result is inflammation, and for the majority of my childhood I had experienced classic symptoms: stiff, painful, or swollen joints. The resilience of youth prevailed; the disease didn’t limit my physical activities too much. However, an extremely rare pathology had started to manifest as adolescence dawned.
Shortly before my fifteenth birthday, my eyesight became cloudy. I had difficulty reading printed material and the chalkboard at school. Writing appeared faded and the characters indistinct. I needed to hold my beloved comic books mere inches from my eyes in order to discern the pictures and words. With justifiable concern, my parents booked an appointment with our ophthalmologist (an eye doctor).
My parents and I were shocked by the results of the eye exam. My left eye could detect light and little else, and I could read a few lines on the vision chart with my right eye. The right had compensated for the left, and I hadn’t noticed that the latter was blinded. The ophthalmologist explained that the arthritis had spread to my eyes. White blood cells, proteins, and other mediators of inflammation had infiltrated the organs and blocked vision. Eventually this process could also cause irreparable damage to the delicate ocular structures. This had already happened – with startling speed – to the left eye and the sight would never be recovered. I now had only one functioning eye, and it was sick. After visiting other specialists and my rheumatologist, I had learned that this complication can occur in late-teenage females but is extremely rare in teenage males.
Treatment focused on preserving and restoring vision in the right eye. It consisted primarily of steroidal anti-inflammatory medicines, taken as eye drops and pills. Some of the former had to be instilled every hour while awake. An ointment, applied to the bottom of the eye inside the lower eyelid, was used overnight. The oral immune system suppressant of choice was prednisone. Prednisone is used to treat a variety of inflammatory conditions, from rashes to multiple sclerosis. It is very effective, but it can also be very harmful. Typically, doctors try to use the drug at the lowest dose possible and for a limited time. I would need to take these tiny white pills daily for the next 17 years, sometimes at doses five times higher than what is considered “safe.” Thus, I would discover firsthand the dangers of excessive prednisone use.
Shortly after the diagnosis, my vision deteriorated. I couldn’t function at school, and I was tutored at home for the remainder of the ninth grade semester. The vision in my right eye was fully restored by the end of the summer, however, and I returned to school in the fall.
I was able to reduce the number of medications and the doses when my vision improved and the disease was under control. But arthritis is chronic and often cyclical; flare-ups of inflammation occur periodically. Accordingly, I needed to modify the dose and frequency of the eye drops and prednisone throughout my teenage years to keep the illness under control. Unusually persistent or severe bouts of inflammation required injections of cortisone (an anti-inflammatory steroid like prednisone) just below the eyeball. I had to consult the ophthalmologist monthly to ensure early detection of an immune system attack on my eye. Once or twice weekly visits were necessary during flare ups.
The medicines and vigilance provided me with one well-functioning eye, which was sufficient to get a driver’s license and finish high school. Before the end of my final semester of school however, I would have the first of many eye surgeries.
Four years of intermittent inflammation, eye drops, and prednisone had traumatized the eye with a consequent rapid decline in vision. The disease process caused fluid accumulation in the eye, and a cataract was growing because of the steroid use. Surgery restored my vision, but I now sported thick glasses. The new apparel replaced the lens in my eye, which had been removed along with the cataract.
The surgery had been done shortly before finishing the thirteenth grade. I was now anxious to start university in the fall. I wanted to be a forensic scientist; this was even before the television show CSI glamorized the occupation. To attain this goal, I wanted to enroll in the Honours Pharmacology and Toxicology program at the University of Western Ontario in London, Ontario. However, my doctors were skeptical. They argued that although the medications had helped reduce the inflammation, I had just had surgery and the arthritis was still active and prone to flare ups. London was over 700 kilometers away from home and the help and support of my parents. Perhaps I should wait a year to let my condition stabilize. Or maybe I could take some courses at the local college. No, these were not options! Presently I had good vision, and I knew how to control the illness. Six months after the surgery, I abandoned the nest and started on my preferred path.
The condition of my right eye still required constant monitoring. During the first month at the university, my ophthalmologist back home referred me to a local specialist who would guide my treatment for the four years I was at school. The strategy would be similar to that which had maintained my vision during high school: monthly or weekly doctor appointments and as-needed adjustments to the dose and frequency of medications, including prednisone.
The school made accommodations in the event that I could not read standard print. I was provided with large print exams and tests, and all my professors were notified of my illness. Unlike the other students, I was allowed to write exams in empty classrooms or offices. I was also given extra time to complete the tests. These steps were necessary because high doses of prednisone, which I had been taking for most of my first year, made me anxious and unable to concentrate. I sometimes had to read a question four or five times before selecting an answer from the multiple choice list.
However, I did not need these concessions throughout my undergraduate study because the disease stabilized by the start of the third year, and my vision was good for the remaining two years.
I made the Dean’s Honour List every year. I enjoy learning and wanted to continue my education at the graduate level. My fragile eyesight forced abandonment of the dream of scientific crime investigation. I wasn’t very disappointed because I had become keenly interested in the field of pharmacology and toxicology and wanted to pursue my own research. But before I could begin graduate work, I would need to have my second eyesight-saving surgery.
The barrage of steroids throughout adolescence had caused glaucoma in my right eye. Eyes contain fluid that gives them their shape. This so-called aqueous humor puts pressure on the back (retina) and front (cornea) of the eye. Glaucoma results when that pressure is elevated to dangerous levels. Despite being treated with eye drops for the disorder, the pressure in my right eye had been consistently too high, sometimes rocketing to more than double what is healthy. Glaucoma, in its early stages, does not hinder vision. Sight can be lost permanently if the pressure remains elevated, and this had begun to occur in the corner of my eye nearest my nose.
The glaucoma treatment, called a trabeculectomy, was performed within a month of writing my final exam. The surgery involves carving a flap into the eye. This “trapdoor” allows the excess fluid to flow into the white part of the eye and eyelids where it is absorbed. The improvised drainage hole successfully lowered the pressure, but I would never regain the peripheral vision nearest my nose, and I was stuck with a restricted visual field.
With the arthritis under control and the glaucoma cured, it was time to decide how to proceed. The recent surgery had planted some seeds of doubt. What if the inflammation flared or pressure rose? What if another complication developed? Could I permanently lose more sight? I would be expected to work independently in a laboratory for several years to get a graduate degree. Would my eye remain stable? Ever the optimist, I enrolled in the Pharmacology and Toxicology graduate program at Queen’s University in Kingston, Ontario, and began studies a year after the surgery.
My Kingston-based ophthalmologist informed me that the pressure in my right eye was rising to dangerously high levels once again as the first year at Queen’s progressed. The trapdoor was sealing closed; I would need a second trabeculectomy. Fortunately, she could operate in the summer, a time when work in the laboratory slowed, and I could take a holiday to recover.
My eye never rebounded from this third insult. It could no longer maintain any pressure, and it softened. Extremely low pressures are just as harmful as those that are too high. The part of the retina called the macula began to degenerate. Given my medical history, I was not a good candidate for any therapeutic intervention. I permanently lost central vision.
The bouts of inflammation increased in frequency and severity. Often the cloudiness could only be cleared by using exceedingly high prednisone doses because I had developed tolerance to doses that would otherwise be effective. Further, the world was darkening. An opaque membrane had formed in my eye, which filtered out much of the light.
I needed to adapt if I was going to finish the graduate work and snag the carrot: a doctorate degree.
I needed to use various magnifying devices, sometimes placing one atop another, to make out words in print and glowing on computer monitors. I learned to read using peripheral, rather than central vision. In effect, I would read a line of text by “looking” at the line below it. This required some practice, but eventually I began to view things without staring directly at them. Despite this strategy, I often struggled to read, write, watch television or movies, and do many day-to-day duties. Fellow graduate students in the laboratory helped me with the benchwork. Though grateful, I had always liked to work independently, and the reliance on other people frustrated me.
I graduated with a Ph.D. in Pharmacology and Toxicology in five years, which is a typical amount of time needed to get a doctorate in one of the sciences. At a doctor visit several months after the graduation ceremony, I could only read the big “E” on the eye chart, and I would never be able to see any better. I was legally blind. My academic pursuits had ended, but post doctorate training was no longer an option.
I returned home to my parents after graduation. I could use a computer and read large print. Surely with my background I could find a job, I thought. I just won’t be able to do research.
I scoured job listing websites daily after graduating. I had to be very selective; office-based computer work was about the only thing I could handle. Local employment was very unlikely because I live in a small city with no large academic institutions or pharmaceutical companies.
A few weeks before Christmas I found a classified ad on one of my ten bookmarked job websites. A biotechnology company in Montreal was looking for a medical writer with a Ph.D. in Pharmacology and Toxicology! Montreal is a short road trip (or in my case a train ride) from where I live, and I fulfilled all of the qualifications. The ad stated that the deadline for applications was the previous day, but I figured I had nothing to lose. My resume was e-mailed eight hours after the cutoff date and two hours later a representative called and we set up an interview.
I never thought that my father would accompany me on my first job interview. My parents drove me to Montreal, and I needed assistance to find the appropriate offices. My father waited in the waiting room while I faced the inquisition.
I was questioned by a darkly-dressed blur with a male voice and a white cloud with a female voice. My vision wasn’t a subject of inquiry, and I didn’t raise the issue. Instead, we discussed the research and writing I had done at university. Towards the end of the process, I mentioned that perhaps the job could be done from home. They hadn’t considered this possibility but they seemed to take the suggestion seriously. The meeting ended after I handed over the research articles I had written as part of my doctorate work.
The interview had been short, and I thought I fed them the “right” answers. The distorted figures guided me back to my waiting father. I must have confirmed any suspicions they had had about my eyesight when I had not shaken their proffered hands.
Two weeks later the company offered me the job of medical writer, but only after me reassuring them that my eyesight would not hinder the work. In addition, they liked my suggestion about telecommunication, and I could do all the research and writing from home. This would save me from having to relocate or make daily train commutes.
A couple of months before my thirtieth birthday, and after three months on the job, I had a routine consultation with the ophthalmologist. I asked if my vision would deteriorate further, perhaps to total blindness. No, he reassured me, your eyesight could not get much worse.
He was wrong.
My vision, what little remained, clouded over the two months after the prognosis. Even the brightest setting on the computer monitor could not penetrate the fog. Work became near-to impossible. The preceding 16 years of inflammation, medications, and surgeries had culminated in a fully detached retina in the right eye.
The operation to reattach the retina was done five years ago. My right eye has not recovered. It now contains a capsule designed to apply pressure to the retina and promote reattachment. The cornea, the front cover of the eye, is swollen, cloudy, and mushy. No light can penetrate, and no further interventions are possible at this time.
I have adapted fairly well to the total darkness. I tend to focus on what I can do, rather than on what I cannot. I read copiously (using a scanner and computer) and listen to audio books instead of watching movies or television. Working can be tedious and frustrating but not impossible. The biotechnology company had to let me go because of financial constraints, but I have managed to scrounge up work here and there as a telecommunicating freelance medical writer. Not being able to see the computer monitor, I use text-to-speech software called Zoom Text. The program is compatible with any Windows applications. It vocalizes text on the screen as well as everything I type. The Lisa voice is the most pleasant. The white cane resting by the door is a necessary mobility aid, and I use it everywhere I go. I remain optimistic. Advances are already being made with stem cells and artificial eyesight, and I wouldn’t be surprised if the veil is lifted within the next decade.
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