Ординатура / Офтальмология / Английские материалы / Retinal Degenerations biology, diagnostics, and therapeutics_Tombran-Tink, Barnstable_2007

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LIVING WITH RETINAL DEGENERATION

how the situation today is different, especially because of the efforts of Retina International and all of our member countries in raising awareness of our condition. Furthermore, given the tremendous advances in technology to aid partially sighted people, and legislative and environmental improvements, we conclude with a view toward the future. We hope you enjoy our contribution.

COPING WITH RETINAL DEGENERATION: JAMES CAPE

Born in 1951, as the youngest of three children, I enjoyed a normal and happy childhood. In 1958, my sister, the eldest of us siblings who was 12 at the time, was diagnosed with MD. As little was known at the time, my brother—13 months older than me—and I were duly examined but were found to have normal vision.

During the ensuing years, I adapted to life with a visually impaired family member, but found it strange that there did not appear to be any magical cure. Throughout my teens and early adult years, I participated in all levels of ball sports, representing my region at the highest level.

At the age of 27 and at the pinnacle of my sporting career and duly making progress in my career as a bank official, I was called up for a military camp as part of the country’s national defence force. Subject to a full medical examination, the military optometrist could not fully understand why I was having problems reading the normal optometrist chart. Consequently, I was simply overlooked on the basis that I was endeavouring to be exempt. Knowing my sister’s condition, I immediately identified my shortcoming and the feeling of dismay remained with me for the duration of my 3-mo call up.

Immediately on my dismissal, I visited my ophthalmologist for a full assessment. After a full diagnosis, he referred me to a colleague for a second opinion. Both concluded that I had MD and were somewhat bewildered by the fact that I had been examined on an annual basis and that the condition had only reared its head at this later stage of my life. At the time, it had only been diagnosed in patients under the age of 25 and after the age of 55 this is known as age-related MD (AMD).

As a young adult enjoying the fruits of life with ambitions and aspirations, the news came as a devastating blow. My immediate reaction was one of anger and bewilderment. I had many questions that needed to be answered by the medical fraternity, my parents, and my faith. This forced me into a state of denial and I almost became an introvert, not sharing or prepared to divulge my inner feelings and hurt. Part of the thought process I experienced was perhaps that there was no light at the end of this tunnel and, therefore, giving up the aspects of living seemed to be the only alternative.

Because my parents had experienced my sister’s condition, they found themselves in an awkward position, as they still did not have what seemed to be the right answers or solutions. Dealing with the emotions and the reality of the situation is not easy to describe as my loss of sight was gradual at the time. But what it did do was start to place an emphasis on the day-to-day aspects with which I was having difficulty. I noticed that I was perhaps not as competitive as I started to focus on the negative aspects of my sporting activities rather than the positive.

At the workplace (I wore spectacles being far sighted in one eye and near sighted in the other for clerical work), I had experienced problems with certain printed material and had merely put it down to an adjustment required to the lenses of my spectacles.

At the time, this did not bother me, but, rather, the daunting thoughts of the future did, based on what my sister had experienced. Slowly, I started to restrict my sporting activities and denied my problems at the workplace. It was during this time that I perhaps received the best advice from my local physician and one that I continue to share with others: Do not give up on your eyes, but let your eyes give you up. What he meant by this was simply, do not give up your day-to-day activities, sports, and work, but rather let them give you up and let logic prevail. With this in mind and a fresh outlook, I approached my day-to-day activities in a more optimistic manner, focusing on the positive aspects rather than the negative. I continued with my sporting activities, taking the opportunity to use my experience and skills rather than relying totally on my sight. In the working environment, it was merely a matter of adjusting to conditions and looking at a smarter approach rather than the norm. I continued to enjoy and play sports and have only recently given up playing indoor hockey. The first of the sports that I retired from was softball and baseball, which I found became more and more difficult. Because the ball spends much of its time airborne and blends in with the background, I was unable to see it and position myself accordingly. Furthermore, the expectations of your teammates continue to be in line with one’s performance and they could not understand or appreciate why my performance had declined. It is worth mentioning that I normally batted at third or fourth in the line up and was considered to be one of the leading run scorers. This was indeed a sad moment.

My other passion was that of squash, which I had played actively from my school days and thoroughly enjoyed playing men’s league squash. With the deterioration of my sight, I found the need to adjust my style of play and particularly focused on keeping the ball at a low to middle projection, which seemed to fit in with my field of vision. The high drop shots caused me the most problem and more than often I would forfeit the point as I was unable to track the ball. At the same time, I was finding that I was being hit by the ball, especially when the ball came off the back wall. Those who know the game will appreciate how much this can hurt, leaving one with bruise marks. My friends typically took advantage of the situation despite the fact that they had empathy for me. I continued to play squash until the early 1990s when it became apparent that I was no longer competitive and perhaps was endangering myself.

I have continued to play field hockey and now have the pleasure of having my two sons on my team. I must mention that four or five of the players have played hockey with me since 1972 and they continuously prompt me throughout the game. This has often lead to confusion with those at the back prompting me to move left whereas those facing me are prompting me to move to the right. I have become known in the league as that deaf player as my teammates were in the early years congratulated for accommodating a deaf player because of the shouting/calling that took place. So you are probably wondering how (or do) I see the ball. With my instinct for the game and my ability to see movement and duly assisted by the prompting, I am able to follow the play of the game. It is only when play is within 1 m or so that I am able to “pick up” the ball provided the ball is in a good, clean condition. When the ball is traveling at a speed, I am unable to “pick it” up resulting in a couple of bumps and bruises. Any free hit taken close to the goal mouth, I am kindly asked to move out of the area.

The team always calls on me to take free hits as I have a natural ability to hit the ball extremely hard. As you can appreciate in field hockey, the ball can be lifted fairly easy depending on the condition of the field and this, combined with me not being able to distinguish between my team and the opposition, depending on the team colors, has unfortunately meant that I often hit players with the ball. My reputation in this regard has certainly exceeded me, resulting in players reluctant to stand too close to me when free hits are awarded. On the subject of the team colors, I have often been generous in passing the ball to the opposition, which often has my team in a tizz.

From the time that I was diagnosed, I immediately informed my employer of the situation and it was mutually agreed that we would address the situation on a year-to-year basis. I have now worked for the same company for 34 yr and have gone through many trials and tribulations.

Owing to the deterioration of my sight, I have tried many forms of glasses. At one stage, the one lens of my glasses was almost 1-cm thick and my colleagues used to comment jokingly that I should not sit with the sun behind me as it could cause the documents I was working on to catch on fire. It certainly does require an adjustment to the many comments from customers and alike regarding how closely I read and that perhaps I should change my optometrist. Naturally, this restricted me in my career development because of the limitation of such matters as transport. I also learned that if you silently teach people to adapt to your needs, they do so willingly and without realizing it. A good example of this is that when staff extract fax messages, they will automatically read the fax and not just merely place it in my in tray. They will ensure that work presented to me is formatted and printed in a manner in which they know I will be able to read. To assist me with computer work, I make use of a software package called ZOOMTEXT, which has a variety of features, such as magnification and text reading. I also make use of a micro reader, which facilitates the reading of documents and books. Despite the fact that I have been overlooked for certain positions, I was recently asked to visit certain African countries to consult on operational issues. This was certainly a challenge and not only from a consultancy aspect, but also the whole aspect of dealing with mobility and obtaining appropriate assistance from people within these countries.

Let me share with you some of my trials and tribulations. Shortly after my diagnosis, I realized that I had to read with my nose closer to the print. As mentioned, my first set of spectacles had one lens that was extremely thick and of course very heavy to the extent that I would have marks on my nose from the frames. As the spectacles were for reading only and the fact that I would have to take them on and off, I often misplaced the glasses resulting in such comments as: “you will have to get a pair of spectacles to find your spectacles.”

Ensuring that the light conditions were favorable became a priority. Too much light meant a reflection off the documents, resulting in strain on my eyes; simultaneously, too little light resulted in difficulty in reading the material. It also became difficult to write, taking into account the closeness of my face to the writing material. To accommodate this, I naturally adjusted my head to a more suitable position with the added consequence of my handwriting deteriorating. My two sons often criticized my handwriting, stating that it was similar to that of a medical physician’s and they would always suggest I scribe letters to the school.

At about this time, I started to experience problems when driving my motor vehicle, depending on a host of conditions such as the time of day, the condition of traffic signs, the condition of the façade, and the amount of traffic on the road. I found that I was not adhering to road rules, going through red lights and not stopping at stop signs. In order to compensate, I found it in my interest to travel routes that I was familiar with and would follow the vehicle in front of me on the basis that they were adhering to the road rules. If there were no vehicles, the cautionary action taken would be to turn left at a traffic light rather than proceed through. (Do not forget we travel on the left side of the road.)

My family and I were preparing to leave on a short vacation and as we left our abode, the motor vehicle stalled and we were unable to get the vehicle started. As it was our intention to call on our dentist on the way because my eldest son was experiencing toothache, my wife decided to proceed to the dentist using our second vehicle, leaving me to sort out the problems. Shortly after my wife departed, I was able to get the car started and, with my youngest son as a guide, made my way to the nearest service center (which was luckily up the road). The motor mechanic requested that I pull the motor vehicle into the garage in order to undertake an examination of the problem. Despite my appeal, the mechanic insisted I drive the vehicle, which I duly did under guidance from my son seated in the back seat. The workshop was a hive of activity and took some careful maneuvering to reach the allocated service bay. It did not take the mechanic all that long to attend to the problem. After which, he suggested we take the car for a test drive. Once again he insisted that I drive, so with the mechanic in the passenger seat and my son in the back seat, off we set. My son was quite clear on his instructions, which went something like—slow down you are about 5 m from the stop street, okay you should now stop, it looks clear you can proceed, you are coming towards a pedestrian crossing, etc. This prompted the mechanic to reply that it was most encouraging to hear a youngster so involved and knowledgeable on our local road ordinance. When we returned to the workshop, the only means of payment was by cheque, which my son duly completed on my behalf, requesting me to sign in the appropriate space. The owner of the workshop was somewhat bewildered and also commented on how nice it was to see a youngster being taught at such an early age.

After some minor accidents and having nearly written the motor car off, I decided it was time to stop driving because I realized that I was not only becoming a danger to myself but the consequences of my actions could be severe. This news I knew would delight my ophthalmologist, who on each visit cautioned me not to drive. The loss of one’s independence certainly takes a huge amount of adjustment, especially in a country like ours where public transportation is almost nonexistent. Part of the adjustment entailed re-organizing getting to and from work, visiting customers, attending conferences, and even on the domestic front. At the time, it was difficult to engage the full impact, but this certainly placed an additional burden on my wife and friends. To this day, this remains one of my stumbling blocks of life, as it does not matter what arrangements I make, I always need to take into account the whole transportation matter. Strange but true, I have a good sense of direction and, once I have been on a particular route, I have the ability to memorize it. As a result, I am invariably requested to direct people from point A to B.

The nature of my job has required that I undertake trips to outlying areas, necessitating the use of air travel. I do not experience difficulty in getting around airports as one only needs to ask for directions in order to get to the various areas requiring departure, e.g., obtaining boarding passes, security, and access to airlines. I do experience problems when boarding the aircraft. The location of my designated seat is indeed a challenge and has often resulted in confusion with fellow passengers.

When arriving at new destinations, it is always important for me to familiarize myself with the lie of the land. What I mean by this is finding out where the dining room, toilets, lifts, and reception desk are located. This action also extends to hotel rooms— making sure that I fully appreciate all the small things. I identify which bottle has the shampoo vs hand cream. To make my life easier, the better I understand or familiarize myself with the environment, the easier it is to adjust. On a recent visit, I exited the hotel carrying a briefcase and, as the stairs leading to the pavement were not clearly marked, I ended up sprawled all over the pavement. Pedestrians passing by were to my amazement reluctant to assist me and I could sense from their remarks that they considered whether or not I was intoxicated.

Living with a disabled person I do believe takes quite an adjustment and does impact on all family members, from time to time. My two sons grew up with my condition from the onset and to them this was the way of life. When playing with their toy cars, the lady always did the driving and it was only when they went to school that they realized that there was in fact a difference. However, being the ages they were, this did not deter them and they played on. They would automatically do certain things differently, e.g., they would look for me after school or school events rather than me looking for them. Without hesitation, they would guide me up or down stairs and describe my immediate surroundings. When walking through the school grounds, they would politely advise me of any teacher that was approaching in order that I could formally greet them. Of course, it was difficult for me to help with school homework, particularly reading.

I would, from time to time, participate with my sons at certain scout outings like day trips, camps, and hiking. Without their help and assistance, these outings would not have been possible. During an overnight hike through some unforeseen circumstances, the boys got split into two groups and for all in tense and purposes were lost. I was in charge of the group and the hike itself was in a nature reserve, which stretched over many kilometres. It took me quite a while to overcome the feeling of absolute helplessness and gather my wits and attend to the immediate problem. The area was covered in tall grass, which compounded the problem. Through applying some good logic and some lady luck, we were able to regroup. As this was on the first day of the hike, it taught me just how observant and careful I had to be.

This reminds me of one incident when my wife was at work one Saturday morning. The boys asked me to read them a story and, not wanting to disappoint them, I chose a book that I was familiar with, not knowing it happened to be a favorite of theirs, which my wife had read on several occasions to them. With my sons sitting on either side of me, I pretended to read the book by telling them the story. It was not long before one of them notified me that I was deviating from the story as read by their mother.

I took the liberty of cleansing my sons inside by default. This came about when my wife went out and I was mowing the lawn and I was left instructions as to provide them

with cool drinks and biscuits as and when the need arose. Youngsters being who they are, it was not long before they requested that I pour a cool drink. I went into the kitchen where the cool drink was kept and unfortunately on this occasion the dishwashing liquid stood next to it (both were green). I took the dishwashing liquid by mistake and poured it into their mugs, added water, and told them to drink up. Naturally the liquid created a burning sensation and, in order to resolve this quickly, I dosed them with milk. I asked them not to repeat this to their mom on her return, but this was to no avail. You can imagine the first words they spoke to their mother and in what detail!

My wife has adapted to the situation and has been a pillar of support throughout the years. She was fully aware of my eye condition at the time of our marriage, but I do not believe anybody can anticipate the impact and the personal sacrifices that are associated with a partner having this condition. With the loss of my sight being gradual, my wife subconsciously takes on more menial day-to-day tasks. I am unable, for example, to read the price of items when shopping. Although I can memorize where the items are in a shop, the condition and otherwise of the article requires assistance of a sighted person. When eating at a restaurant, menus remain a stumbling block and always require somebody in your company to assist you. It can be quite daunting to be told by restaurant staff that the bathroom “is over there,” followed by pointing in the general direction. It does not end there: when you locate the bathroom, the signage is invariably small and difficult to decipher. In order to return to your table, one has to memorize the environment.

Attending to domestic accounts and such matters can also be difficult, especially without the assistance of your partner. I have been fortunate that my family has adapted so well, ensuring that small furniture items are not placed haphazardly in the room. Drinking glasses are stacked in manner that I am able to see them and take them out of the appropriate cupboard without any damage being caused. The watching of television is somewhat unusual as my family sits in their normal positions on the couch and I sit to the side of the television and in close proximity, i.e., almost on top of it. Sliding doors are clearly marked.

The family has become almost experts at describing the countryside when we travel on location. On arrival, I get a full description of the lie of the land, which certainly helps me to orientate myself and enjoy and share their enthusiasm. This is done invariably without having to prompt them and seems to come naturally from them. Typically, this type of behavior has been adopted by friends and family, who invariably are more than willing to assist without making me feel disabled.

Once a year, I visit my local ophthalmologist for a full eye examination. Despite the fact that there is no cure or treatment available, it is important to ensure that the rest of my eye is healthy. During the examination my vision acuity is measured. I am actively involved with Retina South Africa, which gives me the opportunity to keep abreast of the latest developments both on a local and international basis. This includes research treatment and technology enhancements. Public awareness remains an ongoing challenge and, in particular, in our country. This has a distinct disadvantage in that it is difficult to explain to people that you have sight but cannot see. The yellow cane concept, which was muted some years ago, (as a means of indicating that the individual is partially sighted as opposed to the white cane, which is interpreted that the person is blind), still carries merit and should be reconsidered. Because of the lack of awareness, employers