Retinitis Pigmentosa and Blindness

How to help blind and partially-sighted people (video)

The technics how to guide a visually impaired person (video)

Sports and Activity Rules for those with Visual Impairments

Alpine skiing

Alpine skiing is a winter sport which requires excellent endurance and communication skills between the visually impaired skier and the guide. The skier controls his/her descent by traversing the face of the hill. The guide facilitates this activity by calling out turns to be made etc. This process is the same as for any skier except that a sighted guide assists in navigation.

Events competition among the blind/visually impaired occurs at regional, national and international levels in the following events:

Slalom - A race in which each competitor, with navigational assistance from a guide, skies as fast as possible down a zig zag course around tightly placed markers.

Giant slalom - A race that is similar to slalom but on a longer course and the markers are spread further apart. Thus turns are less frequent but at higher speeds.

Super giant slalom - Similar to the giant slalom but on a much longer course with fewer turns which results in very fast racing.

Downhill - The fastest of all the ski races. The skier and guide negotiate a course with even fewer turns that the Super G at exhilarating speeds.

Special Equipment - Skies, boots, poles goggles and helmets must be worn at all times in competition.


Track consists of running short, middle and long distance events which are excellent for quickness, strength and improving the cardiovascular system. The use of guides depends entirely on the athlete's visual classification and the particular event. Guides facilitate the activity by running alongside the visually impaired athlete, both runners holding on to a tether. Alternatively, stationary guides positioned around the track call to the runner giving directional signals.


100 meter (Male & Female), 4x100m Relay (M&F), 200 meter (M&F), 400 meter (M&F), 800 meter (M&F), 1500 meter (M&F), 3000 meter (F) , 5000 meter (M), 10k road race (M&F), Marathons (M&F), Pentathlon (M&F)

Proper running shoes are strongly recommended. Track spikes are allowed. Tethers for use with guide runners shall be a flexible, non-elastic material not to exceed 50 cm in length.

Beep ball

The pitcher and the catcher of each team are sighted players. These pitch at, and catch from, their own team, the opposite of what happens in ordinary baseball, where these players pitch and catch against the opposing team. All the players, except those just mentioned, wear blindfolds. Why? To avoid those with residual vision having an advantage over the totally blind players.

When a player takes his place to bat, the pitcher and catcher of his team also come on. The pitcher will be at a distance of twenty feet (6 meters) from the home plate and will do all he can to ensure the batter hits the ball. For a shot to be considered good, it must pass a line situated forty feet (12 meters) from the home plate. If it does not pass this line, it will be considered void. Neither the pitcher nor the catcher may touch the ball once it has been batted. Should this occur, the batter will be declared out. The batter will strike out after four strikes and not three as is the case in ordinary baseball. The pitcher will use two signals, warnings or commands to help the batter to hit the ball: first, he will shout "ready", which means that both himself and the batter are ready to play and, second, "ball", at which the batter will attempt to hit the approaching ball. Should the batter hit the ball the required distance, he then runs towards the base activated for him by the umpire - first or third. The base emits a continuous beeping sound to distinguish it from the ball and thus avoid confusion for the players between one and the other. The base is made of a soft material (foam), covered with plastic and is three feet (approx. 1 meter) high. There are only two bases on the pitch, compared with the three in ordinary baseball. They are situated at a distance of 90 feet (27 meters) from the batting area, one along the right-hand line and one on the left, similar to the first and third bases in ordinary baseball. They will be placed ten feet (3 meters) outside the foul line. The base is reached whenever the player touches it with any part of his body before the defender gains possession of the ball. So, if the batter hits the ball towards the first base, the umpire will activate the base corresponding to the third base area and the batter will run towards this base, and vice versa. This method is adopted in order to avoid accidents. If the runner reaches the base before the defending player gets the ball, a run will be awarded. If, on the contrary, the defending player regains possession of the ball before the runner reaches the base, the latter will be declared out. If any defending player catches a ball in the air, the innings will be declared over. That is, that play may be considered as one, two or three "outs", depending on the number remaining to finish the innings.

There is a line marking a distance of 180 feet (54 meters) from the home plate. If a batter hits a ball and it goes beyond this line, he will be awarded two runs.

The match will last for six innings, each consisting of two halves with three "outs" per team. The visiting team will bat first. The local team will not bat in the sixth innings if it is already winning when the visiting team finishes its batting turn in this innings.

There is a special ruling known as the "twelve-run rule" which states that, if, at the end of a complete innings, either team is leading by twelve runs or more, the opposing team will continue batting after its three "outs" are completed. Every three "outs" will count as an innings and the next one will then commence, and so on until they catch up with the winning team's score. Should this not occur until the sixth innings is finished, the game will be declared over. If, on the contrary, they draw level or surpass the winning team's score, the game will continue as normal. Nevertheless, the team that previously had a twelve-run lead, will not lose those turns which it did not take previously so as to permit the losing team to bat. If the umpire observes that play endangers some players at a given moment, he may stop the match. Said play will be declared void and the game will resume as if it had not taken place.


Goalball is a team sport played exclusively by the visually impaired. The object of the game is to roll a ball which contains bells past the opposing team. There are two teams of 3 players which alternate rolling and defending. The offensive team rolls the ball in a manner that is either hard or soft, depending upon player style, in an attempt to get the ball past the opposing three players. The defensive team listens for the approach of the ball and attempts to prevent or block the ball from crossing the line. The ball is rolled back and forth with the offensive and defensive team alternating until time expires for the half. The game is played in two five or seven minute periods and the team with the most points wins. There are women's and men's teams with no variations in equipment or rules.

Events - The USABA offers local, regional, national and international competition.

Special equipment - Clothing, gym shirt, shorts or sweats, a goalball, blindfolds, set of goals (not mandatory), tape for special markings on the floor.

To purchase goalball equipment can be purchased through USABA.


Judo is a competitive sport contested by two players from the same classifications. Judo matches can last as long as two or five minutes or as quickly as the time it takes to score a pin. The match is started with the contestants gripping or holding the shirt of the opponent. When the contestants are in proper starting position the referee will call "Hajime" (begin). Judo is similar to the sport of wrestling, the differences lie in the rules, the clothing and how points are scored.

Points may be scored by throwing your opponent to the mat, pinning your opponent to the mat, or through holds like arm bars which makes the opponent plea submission.

When the referee determines that a contestant has executed a proper action to score a point he will call "Ippon". Ippon signifies that one point has been scored and the match has ended.

Events - Events are conducted in local, regional, national and international tournaments. Athletes participate in specific weight classifications.

Special equipment - Mats (foam rubber), clothing in the form of a robe (judogi).

Nordic Skiing

Nordic or cross country skiing is a winter recreational/competitive sport that is pursued over generally flat areas or a combination of flat and hilly terrain. Nordic skis are long and narrow and are fastened to a cross country ski boot at the toe. The skier is also equipped with poles to help with balance and forward motion. Cross country skiing is much like jogging, alternating left and right while gliding over the terrain. Wax is applied on the bottom of each ski to grip on the snow as the alternating motion takes place. Cross country skiing generally takes place on surfaces in which tracks have been made by previous skiers. This facilitates cross country skiing for the blind/visually impaired and decreases the dependency upon a guide. The guide, however, is needed to assist avoiding obstacles and in navigating turns, and does so by calling directions to the skier.

Events - Nordic skiing can be recreational outing or a competitive event. The competitive events at the local, regional, national, and international level are:

Men 15k, 30k; Women 5k, 10k and 4x10 relay races

Special equipment - In addition to cross country ski equipment, it is important that each skier dress in layers of clothing. Skiers rapidly generate enough heat to stay warm and will want to remove a layer at a time to avoid overheating.


Powerlifting is a sport that has no age, sex or weight restriction on participants. There are three basic lifts that are conducted, both in recreational and competitive lifting. These three are described below:

Squat - Consists of squatting down and then rising again to a standing position while holding a bar with weights behind the head resting on the back of the shoulders.

Bench press - Lifter lies on his/her back on a horizontal bench, lowers a weighted bar to his/her chest and pushes it back up to arm's length by extending the arms.

Dead lift - A lift in which the weight is lifted from the floor to hip level with straight arms by the power of the back and legs, and then lowered to the ground again.

The object is to lift as much weight as possible in each of the three basic lifts. Generally, people start with lighter weights and improve their strength through practice, repetition and improved technique.

Events - Events are conducted at local, regional, national, and international levels. Classifications are made according to sex, age, and weight of each participant. The age categories are 14-19, 20-39, 40-49 and 50 years and up.

Special equipment - Weights or access to a weight room is necessary, and cooperation from one or more 'spotters' who assist in safety aspects. A lifting belt is strongly recommended to protect the lifters back.


Swimming is a sport with no age restrictions and may be pursued for fitness, recreation, or competition. Swimming is an outstanding sport for increasing overall fitness and body strength.

Special equipment - To aid the athlete in judging turns, a coach may tap the competitor when he/she nears the end of the pool. For this we have created a special device called a 'tapper'. A verbal count is given to inform the swimmer of the number of remaining laps in competition. A common practice of many of the USABA swimmers has been to count the strokes it takes to go from one end of the pool to the opposite end. This technique assists the swimmer in knowing when the end of the pool is being approached.

Events - USABA offers competition at the regional, national, and international level:

Butterfly 100 & 200 meters (50 & 100 for Masters)

Backstroke 100 & 200 meters (50 only for Masters)

Breaststroke 50, 100, 200 meters (50 only for Masters)

Freestyle 50, 100, 200, 400, 800 (women), 1500 (men) (50 &100 for Masters)

Individual medley or all 4 strokes in one race 200 & 400m (200 only for Masters)

Tandem Cycling

Tandem cycling is a sport for individuals interested competitive bicycle racing. The sport has two disciplines. Track racing and road racing. Track racing takes place on steeply banked track called a velodrome and road racing is as it sounds, on the road.

Track bikes have no brakes and only one gear, which is sized according to the riding style of the cyclist and the event. Road bikes vary widely between events and are specialized in nature, often incorporating aerodynamic design elements and sporting as many as 27 gears.

Both types of racing, however, share a common ground - the winner is not always the fastest rider. Strategy and knowing an opponent's strengths and weaknesses, can be important as speed.

Tandem cycling consists of two riders, the rider on the front, the pilot or captain, is sighted. The rear rider, the stoker, is visually impaired or totally blind. Events range from a few minutes for the kilometer on the track to a few hours on the road. Track events include the kilo (kilometer timed trial), sprints and pursuit races. Road events consist of road, time trials and criterium races.

Cycling is a sport that improves an individuals strength, endurance, balance, and conditioning, as well as developing character, commitment and dedication, which is used well beyond the sport and into an athletes life.

Requirements - Helmet (ANSI or Snell approved), high quality cycling shorts and jerseys and shoes with a cleat/pedal system must be worn. Ownership of, or ability to borrow a tandem is also very important.


Wrestling is for every size and shape person, both men and women. It is the most natural sport for the visually impaired because the two competitors must maintain contact with each other throughout the competition.

The sport is performed similarly to "sited" wrestling. The rules for international wrestling competitions have some modifications to render conditions more suitable for the visually impaired.

From the beginning of the competition contact is established between the two contestants in the standing position by the gentle overlapping of each hand over the hand of the opponent. Although each contestant may subsequently move and change this starting position, as long as there is some type of contact between the two contestants wrestling continues. Once contact is broken the referee will blow the whistle to reestablish.

Visually impaired wrestling was included in the 1984 Paralympic Games. Unfortunately the sport has not been since then due to a lack of interest. It is our goal to reinstate its inclusion in the 2004 Paralympic Games. In order to accomplish this, specific requirements must be met. There must be 18 countries, representing 3 continents participating in National Championships, Continental Championships, and World Championships. With the amount of interest received, our goal looks very attainable.

National Championships have been held in 15 countries for blind wrestling. There have also been three regional championships in which an average of three countries U.S., Canada, and Puerto Rico, have participated. World Championships have been organized in the Netherlands and the U.S. The interest in the sport of blind wrestling has greatly increased. Many countries, Algeria, Canada, Chile, Czech Republic, Ghana, Indonesia, Iran, Italy, Kenya, Mexico City, Nigeria, Republic of South Africa, Russia, Sri Lanka, U.S.A and Zambia, have shown interest in developing programs for visually impaired wrestlers.


What the letter "p" can cause. Jillian's story.

by Robin Benoit

It all started with the letter P. It was that one special day in preschool when the focus was on the letter P. They had planned to wear pajamas, paint pictures in purple and pink, eat popcorn and pretzels and pretend to be pirates. Perfect!

I had dropped my daughter Jillian off at school and about two hours later received a call from her teacher. She called to say that Jillian had “freaked out” in class. The teacher explained that the class had been pretending to be pirates and when she put a patch on Jillian’s eye, she had thrown her hands out in front of her as if trying to reach out for something and fallen to the floor screaming for help.

My husband and I were troubled by Jillian’s reaction to the pirate patch and went immediately to see her pediatrician. He was skeptical, as we hadn’t previously noticed any vision issues with Jillian, but he said we would take her into the hall and check it out. I stood with Jillian at one end of a long hallway while our doctor stood by the eye chart at the other end. A nurse covered one of Jillian’s eyes and asked her to read the letters on the chart, which she did with no problem. Then, the nurse covered the other eye.

“Mommy, help me!”
My heart nearly stopped as Jillian instantly threw her hands up in front of her and started calling out for me. “Mommy, Mommy, help me!” I was standing right next to her within arms reach of her right shoulder. I spoke softly to her, telling her I was right there and she looked in my direction as if searching for me. I grabbed her hand and started to cry.

The doctor looked completely shocked. He said, “Jillian, can you see the eye chart on the wall?” and she moved her head all around as if searching the sky and ground and everywhere in between for it. Our doctor then said, “Jillian, can you see me?” and she stood very still and softly murmured, “I hear you.”

The doctor left us in the examination room and called to get her in immediately at the children’s hospital. I was terrified. What was wrong with my baby girl? Had she always been blind in one eye? If not, why was she not able to see—all of sudden—out of one eye? How could we have missed that?

A week later, the ophthalmologist told us that Jillian had amblyopia. I had never heard the word but I soon learned that amblyopia is “partial or complete loss of vision in one eye caused by conditions that affect the normal development of vision.”* With amblyopia, the brain favors one strong eye over the weaker one. The weak eye is eventually ignored by the brain, and therefore the brain cells related to eyesight do not mature normally. Amblyopia is the most common cause of monocular blindness, partial or complete blindness in one eye. It affects an estimated two to three percent of children in the United States.

So, yes, Jillian had basically become blind in one eye because her brain had all but ceased communicating with the weak eye. The doctor explained that there is no surgery to correct ambloypia. The most common treatment is to force the brain to start using the “bad” eye by putting a patch over the “good” eye.

The best we could expect
Jillian wore an eye patch for ten to eleven hours a day for three years. Her limited vision led us to the decision to home school during that time. At the end of third grade, our ophthalmologist said that Jillian had made as much progress as she could with the eye patch. The vision in her weak eye had improved to 20/40 with the assistance of eyeglasses. We were told that was the best we could expect.

Jillian was so excited to return to public school. She started fourth grade feeling a little nervous, but with high hopes. Her fourth grade teacher was fantastic. She was quick to point out Jillian’s strengths, but equally concerned about her weaknesses. She told me that Jillian could answer questions verbally and recite her times tables perfectly, but then she would fail the written math exam. I assumed that Jillian needed to see the ophthalmologist about adjusting her eyeglasses prescription. When we saw him, he agreed that she needed a slight change, but said it shouldn’t be causing her problems in school. He had nothing else to offer.

That afternoon I sat at my computer and typed into Google the words vision and learning. I found pages and pages of information about something called vision therapy. I didn’t know if this would help Jillian, but I was willing to try anything. I clicked on one of the links to find a vision therapist in our area. With a silent prayer, I dialed the number.

The first thing I discovered about vision therapy is that 20/20 eyesight is not 20/20 vision. Eyesight is merely the basic ability to “see.” Vision is the ability to identify, interpret, comprehend, and act on what is seen. Visual memory, visual concentration, eye teaming, and focusing skills all come into play with vision therapy.

At Jillian’s first vision therapy appointment, I was drawn to a wall in the hallway. It was plastered with photographs of patients of all ages accompanied by their success stories. I enjoyed learning about a high school student who, after overcoming an eye muscle weakness that caused double vision, went on to be the pitcher on her university’s softball team. I was also inspired to hear about the man who was able to achieve his dream of becoming a Navy Seal.

I learned that vision problems are as troubling for adults as they are for children and teens. Undiagnosed and untreated vision challenges follow adults into their later years, sometimes disrupting careers. As it turns out, many adults have solved serious problems through vision therapy.

No Teamwork
After Jillian’s initial consultation, we were told that one of her most worrisome and immediate problems was that her eyes were not teaming together. They were working independently of each other. The therapist explained that this is a common result of eye patching.

With normal vision, both eyes work cooperatively, sweeping easily from side to side. However, as Jillian would read a line in a page of text, her left eye would read a few words to the middle of the line and then her right eye would try to take over for the other half of the line. Her vision was causing not only reading difficulties, but troubles with math and handwriting as well.
Jillian embraced the challenge of vision therapy. She knew that schoolwork was harder for her than most others in her class. She had told me she wished her teacher would not hang up her drawings or stories on the wall because they were not as pretty as other students’ work. It was a relief to her to find out there was someone specially trained who could help her.

Jillian did vision therapy exercises four or five nights a week at home, and worked an hour every week in the vision therapist’s office.

Many vision therapy instruments are unique and creative. Some are unique in that they have been engineered specifically for vision problems. Others are creative: simply a ball on a string, a newspaper, a chalkboard, a mirror, golf tees, a low balance beam.
One unique tool is the trans-lid binocular interactor (TBI), which flashes a bright white light, alternating between the left and right eye at nine cycles per second. In Jillian’s case, the goal of the TBI was to stop the brain from suppressing her weak eye. Suppression comes from the brain’s desire to have a clear image and to protect against double vision.

Another tool used to correct amblyopia is the standing cheiroscope. The cheiroscope looks something like binoculars or an old-fashioned viewfinder. Jillian would use a pencil to trace a drawing while looking through this device, which immediately revealed if Jillian was suppressing her weak eye or not. The picture would be incomplete if she was only using one eye.

Little Bo Peep with no sheep
One of Jillian’s favorite exercises involved the near-vision vectogram. In this exercise, images of storybook characters like Humpty Dumpty, Little Bo Peep, and Old King Cole are presented on transparencies. The vectogram has polarized lenses. The result is that when Jillian would look at the images and then tell the vision therapist, for example, that Humpty Dumpty’s hat was missing or Little Bo Peep had no sheep, the therapist knew that Jillian’s brain was suppressing her right eye.

Jillian graduated from vision therapy fifteen months after she began. What an amazing transformation had taken place! When she recently turned 11, we went to see her pediatrician for an annual physical. He was so pleased with how well Jillian was doing. He said he was glad that the ophthalmologist had been so helpful. I said, “He wasn’t. We found vision therapy.”

That was, perhaps, a little unfair to the ophthalmologist. The eyeglasses and eye patching did help Jillian to make progress, but at that point Jillian was almost like a car with a good engine but poor steering. Jillian also needed the development that the vision therapist was able to help her accomplish.

Jillian now wears one contact lens instead of glasses. She is doing so well in school and is much more aware of her surroundings. She loves riding her bike and scooter and I don’t worry as much as I used to about her safety. It’s nice to be on par with all mothers who worry about skinned knees and elbows instead of the gut-wrenching fear that she won’t see a car until it is too late.

Years ago, we wondered why our purple-and-pink-pajama-wearing princess would be so distraught over wearing a pirate eye patch. Now we know. It took a six-year roller coaster ride of good days and bad days to find answers, and what a journey it has been.

Jillian’s odyssey may have started with the letter P, but it has now reached the letter A. That is, with the exception of a B+ in math, all the A’s she got on her last report card.

*Webster’s New World Medical Dictionary


Hunnah has Retinitis Pigmentosa
One morning in 1987 Stephen Jones and his wife, Lesley, drove from their home in Osterley, west London, to Moorfields Eye Hospital in the City so their son Ben could have his eyes tested.

A nurse had picked up ‘some problem’ during a routine health check at Ben’s primary school. She was not sure exactly what it was, only that he needed to be seen by a specialist.

Stephen and Lesley were not overly worried because Ben’s eyesight was not noticeably bad. He had had some problems reading the blackboard, and occasionally tripped over his toys, but his parents put that down to clumsiness.

Otherwise Ben was a normal eight-year-old boy: he loved football, watching television and hanging out with his friends.

The consultant examined Ben’s eyes, then delivered the news.

He was consoling and deliberately used careful wording so as not to sensationalise the condition, but said that at some point in the future – maybe in two years or 20, it was impossible to predict exactly – Ben would start to lose his sight. Ben had retinitis pigmentosa, a group of inherited eye conditions that lead to incurable blindness.

‘It was a severe shock because it came out of the blue,’ Stephen recalls.

There was no history of blindness in their family.

‘I remember the assistant saying, with luck his eyesight will remain good throughout his education. So, in one visit you’re going from thinking, oh, he may have to wear glasses to being told he’ll probably lose his sight.’

Now they faced the dilemma of what to tell Ben. Although he had been sitting across the table from the consultant during the diagnosis, what had been said barely registered with him. (Ben says now he has no memory of that day; he says he was probably thinking about football – he was devoted to Everton.)

Should Stephen and Lesley tell their son what lay ahead and allow him to prepare for the future? Ben was obsessed with sport and his dream was to play competitive rugby or football. Or should they keep quiet about the timebomb in store? After all, how do you explain to a child that he has been sentenced to inevitable darkness?

The issue of disclosure, of what to reveal to whom and when, is increasingly being discussed as advances in genetic testing mean that people can be forewarned of their future. The success of the Human Genome Project, which mapped man’s entire genetic code, has made it possible to detect hereditary diseases that develop later in life. Huntington’s disease, for example, which is non-treatable and fatal, and which does not typically develop until middle age, can now be picked up in the womb and, in the case of IVF, even before with ‘pre-implantation genetic diagnosis’ – embryo screening; likewise, BRCA, the gene known to dramatically increase the risk of breast cancer.

Dr Ruth Newbury-Ecob, a consultant in clinical genetics at University Hospitals Bristol, points out that the dilemma facing Ben’s family ‘is where a child has a diagnosis and it’s a question of the age at which they’re made aware of that.’

What she sees in her clinic are parents with a strong family history of a genetic disease seeking a ‘predictive’ test: has their child inherited the disease or not? The technicalities of a genetic test are fairly simple, but its human implications are painful and complex and often lead to heart-rending decisions.

Individuals with a serious genetic disease in the family now have a choice. Do they live in hope? Or do they take the test, and then, if it is positive, spend the next 20 years knowing that death or incapacity approaches? How does knowing affect an individual’s decision about a job, a partner, having children? There is soul searching for the parents of children who test positive, without a doubt. But what of those who do the testing?

‘It can be difficult to stand back and observe a parent withholding information from their child, particularly in early teens when they are thinking about their future and you are aware they don’t know the full picture,’ Sophie Devery, a genetic counsellor at Moorfields, says. Most parents do tell their children, she adds, ‘but obviously we can’t get involved because that is the parents’ decision.’

‘It’s very difficult for parents to balance the difficulty of telling their child they have tested positive,’ Dr Newbury-Ecob acknowledges, ‘the distress that may cause, the anxiety for the child, and how it affects the parent/child relationship, against the long term, where if they choose not to discuss things and the child finds out through a third party, that can be very damaging for the relationship because of the breakdown of trust.’

Retinitis pigmentosa (RP) causes the slow disintegration of retinal cells: first the rods, which enable vision in low-light conditions (the first symptom of RP is typically night blindness); then the cones, which detect light and colour. As time goes on, many sufferers are left with narrow tunnel vision. There is a lot of individual variation in the disease. Some sufferers are blind by their early thirties; others can still have good eyesight into their forties and fifties. About 25,000 people in the UK suffer from RP (one in 3,000), and there is currently no cure.

There are three main hereditary patterns: ‘dominant’ requires only one abnormal gene from either parent; ‘recessive’ requires an abnormal gene from both parents; and X-linked, where only males are affected after inheriting one abnormal gene from their mother. Genetic testing for the disease was first developed in 2003, but still only about half of all RP cases can be genetically tested (the dominant and X-linked forms). And although it is inherited, half of all cases come as an abrupt shock.

‘Most of those are recessive but some are X-linked, where there hasn’t been a male born for some time with it,’ Anthony Moore, a professor of ophthalmology and an honorary consultant at Moorfields, explains. The other slightly unusual aspect of RP is that it is not always necessary to do a genetic test to get advance warning. It can be stumbled on during routine eye examinations and confirmed by an optician looking at the back of the eye (the tell-tale sign is changes in pigment).

Ben Jones is now 31 and in his second year training to be a vicar at Trinity College, Bristol. He is tall and slim with messy dark hair and an endearing boyish buoyancy. We meet in his light-filled flat in a residential suburb of the city, where he lives with his wife, Victoria, a stay-at-home mother, and their children, Evie, two and a half, and Caleb, four months. His father, Stephen, is visiting for the day (his parents now live in Newbury, where the family moved soon after Ben was diagnosed). Both father and son exude a wry humour, which suggests that this is their way of coping with the seriousness of the condition.

Ben was registered blind aged 17, and has used a white stick for the past five years. Yet he is not completely blind. He cannot see anything in his right eye, but has narrow tunnel vision in his left, as if seeing through a straw. Normal vision is 180 degrees; Ben’s is two degrees.

‘So from here, if I look at your right eye I can’t see your nose,’ he tells me. ‘In fact, if I look in your eye, I can’t see your eyebrow. So at any given moment I can only see a tiny area, and I make up the picture by not just looking at one place all the time.’

In good light, he can still read, work on a computer and watch television. It transpires that both of his parents were carriers of the RP gene (the recessive type), but did not know it. Consequently, Ben had a one in four chance of getting the disease. (His sister has been examined, and has no signs of RP on her retina, but she has a 50/50 chance of being a carrier.)

Stephen and Lesley decided to tell their son as soon as he was diagnosed. ‘I’m not very keen on secrets and holding things back from people,’ Stephen explains. ‘Obviously we didn’t spend our time saying to Ben, “You’re going to go blind.” You can protect your child by being entirely open, but not laying it on thick, and doing it as gently as possible. So when something arose like a forthcoming visit to Moorfields or if Ben trod on the cat, we would talk about it with him. The thing we did debate in the family was the extent to which we should tell his friends, and we all agreed from the outset that we should be very upfront about it, so when the need arose, people were aware.’ Stephen spoke about RP at Ben’s secondary school.

At first Ben’s only symptom was night blindness at the age of nine – in dim light, he struggled to find the door, for example. But by the age of 14 Ben had lost the sight in his right eye and the sight in his left eye was slowly closing in. At 17 he could no longer play in the school first team for rugby, basketball and football (he was in all three), and he was also noticeably stumbling into objects during the day. But he went on to study theology and computing at the University of Kent in Canterbury, and then trained as an RE teacher, which he did part time while also being a student worker at his church (where he met his wife).

‘The fact that I’ve always known [about the RP] has made it easier to bear,’ Ben says. ‘So at every stage I’ve known it’s going to get worse, but two years is the distant future when you’re 10. I can still see my kids and watch football on the telly, and at some point that is probably not going to be the case, but I don’t really think about that because I don’t know when that’s not going to be the case. I suspect that everything about this was much more disturbing for Mum and Dad than it was for me. And maybe that’s partly because I am relatively chilled out anyway, but also because I was told when I was eight and when you’re eight you just take stuff in your stride.’

Ben’s having RP also means he is a carrier and therefore has the potential, if Victoria is a carrier too (which they don’t know and don’t want to know at this stage), to have passed it on to their children. Does he want to get his children tested? ‘If one of them starts showing signs or even the vaguest problem, then I’d want to, but I’m not in a desperate rush – not because I don’t want to, but because it seems like an enormous amount of effort to go to for an off-chance.’

RP meant Ben abandoned his dream of being a professional footballer, and he regrets not being able to go to nightclubs – ‘not being able to see or hear is a very bad combination’. Walking without a white stick in an area he doesn’t know makes him feel vulnerable, but in familiar surroundings such as his home, which is unmodified, he moves freely – he puts a glass of water directly in my hand. When talking of the difficulties of RP, Ben is predisposed to focus on the comedy: bollards are, painfully, ‘always at knee height’; he walked up to his waist in a small lake at Kew Gardens; he fell down a manhole while on a school trip to the theatre. ‘I did the whole cartoon thing, legs twirling in the air, before plummeting.’

‘Ben has taken RP in his stride all his life,’ Stephen says, ‘even when he was going through his “Kevin” years’ – Ben’s teenage rebellion included riding motorbikes and quad bikes around a friend’s farm and subsequently doing badly in his A-levels – ‘but in terms of RP I’ve never heard him say, “It’s not fair” or “Why me?” or “This shouldn’t have happened.” He has just got on with life.’

Ben says his decision to become a vicar wasn’t dictated by his sight, but by the fact that he had always wanted to be a vicar. ‘In fact there will be issues – I won’t be able to be in a rural parish, for example, because I can’t drive and a lot of churches are quite dingy.’ Likewise his decision to marry young (he was 25 when he met Victoria) and have children was simply down to ‘finding the person I knew I wanted to spend the rest of my life with’.

Has knowing the future made Ben want to see as much as he can while he can?

‘I think so, but it’s hard to say what is me and what is the eyesight problem,’ he says. ‘I’ve always been someone who wanted to experience life.’ He aims to continue bungee jumping and white-water rafting no matter what. ‘But there has been some stuff I wanted to do before going blind. I will be forever grateful that whatever happens, I got to see my children.’

Not long after meeting Ben I went to see Tom Walker, a pharmacist, 39, in Newcastle. He is married to Elizabeth, a graphic designer, and they have three children, Charlie, 10, Hannah, eight, and Emily, four. Hannah was diagnosed with RP when she was three and a half. Both Tom and his wife are carriers of the recessive form of RP, and so have a one in four chance of having a child with RP.

‘I noticed Hannah didn’t see particularly well in the dark,’ Tom says. When he read her a bedtime story and the light was low, she couldn’t see the pictures on the page. If her teddy bear dropped off her bed, she couldn’t find it. Their GP referred them to a consultant at the eye hospital who diagnosed fast-acting RP, where the degeneration is rapid. ‘They told us she would be severely visually impaired by school age.’ Tom and Elizabeth were devastated. Hannah had just started nursery.

The vexing question was what to tell Hannah. ‘You carry the responsibility for a child at that age,’ Tom says. ‘She wasn’t old enough to understand.’ So they did not discuss it with her. And they still have not discussed it with her, partly because Hannah’s predicted deterioration has yet to happen. ‘She is long-sighted but no more or less than any other child in her school who wears glasses,’ Tom says. Hannah is the sort who loves to put on shinpads and kick footballs. ‘It’s only a matter of time, we know that,’ he says.

So will they discuss it with her? ‘What’s the trigger?’ Tom says. ‘Is it four and a half, five and a half, six? When would you consider a child to be old enough to cope with knowing that she will go blind?’ Tom’s eyes fill with tears. He is heartbroken and confused. ‘It’s hard for us to accept and that’s why I’d like to tell her on the day they find a cure, because that is easier to deal with.’

There are two treatments currently in clinical trials in Britain: gene therapy, where good copies of the gene that is faulty are injected into the retina; and artificial retinas or the ‘bionic eye’, where an electronic chip sits on the retina stimulating light-sensitive cells. Neither offers a complete cure and neither is imminently available on the market, but both are the focus of widespread hope.

Tom says that he and his wife rehearse telling Hannah in their minds. But not telling her has advantages. ‘The innocence of childhood,’ he says, ‘Protecting your child. Would you want to know that you were about to step out of that door and get killed by a bus? Most people wouldn’t want to know the future, you just want to live life to the full. That is what I want to do with my daughter. I want her to live life to the full.’

He adds that both sets of grandparents agree that not telling Hannah yet is the right thing to do. As do friends and other members of the family. The only person who disagrees is the consultant who diagnosed Hannah’s condition. ‘He’s advised what he would do in my situation – tell her – but respects my decision not to,’ Tom says.

Most parents explain in a simple way once their child is at school, Prof Moore says, but ‘there is a minority who do not like to discuss it. There are some families where even the grandparents do not want to talk about it. They feel very guilty.’

But not telling opens up other problems. Hannah has to be removed from the consulting-room during discussions at her yearly check-ups and has started to ask why ‘Daddy has gone back to talk with the man’. And the fact that they have confided in close family, friends and teachers (so Hannah can sit at the front of the class to see the blackboard) means that there is the potential for information to leak out.

‘It’s a bit of a tightrope we walk because we don’t want Hannah to find out in the playground from another child whose parents have discussed it with someone else,’ Tom says. And not telling requires ingenuity. Hannah has started to ask questions about why she cannot see as well as her friends at night. So far her parents have answered by saying everyone is different – ‘kind of skirting around the issue’.

‘It would be very easy to bury your head completely and that would not be the right thing,’ Tom says, ‘because when we do talk to Hannah about her eye condition she is going to want to know what we did. Did we try to look for a cure? Did we try to raise money to help with research? How far did we go on her behalf knowing what we knew? So we do sponsored events and charity things and I’m involved in the RP society for that reason.’ Tom swallows hard. ‘Just to explain to her when she’s older that we tried to do things… tried to help her, even though she didn’t know.’


Retinitis Pigmentosa Support Group on "The RP Family on Facebook" (video)

Retinitis Pigmentosa by Lorelei Rabishaw

I was 18 years old when I began to notice little floaters and my parents began to notice that I was tripping over things. I went to several doctors in both Calgary, where I was living, and in Ottawa, where I moved at age 19. I was finally diagnosed with RP in Kingston when I was 21. It was somewhat of a relief to know that it "wasn't in my mind" as some had suggested. I was referred to an opthamologist in Ottawa who immediately told me I was legally blind and must stop driving. Of course I felt I could still drive, and continued to do so during the daytime for a few years. At the time I was employed as a secretary at City Hall in Pembroke, Ontario and had a new sports car. Up until that time my life was happy and I was untouched by the grim realities of the world. I decided that I could no longer work as a secretary. I sold my car and quit my job and returned to university where I got an undergraduate degree in law with a concentration in criminology and criminal justice. I married and became employed as a probation and parole officer. I was still driving and life was great. I forgot about my problem until it started deteriorating further. When I quit driving I was confined more to the office and I became very frustrated. I had difficulty with paperwork and in concentrating. After my daughter was born in 1994 I was exhausted and became depressed. I worried that I might not be a good mother because of what I thought were my limitations. I left work in 1995 and thankfully had a long term income protection plan. I remained quite depressed and felt isolated after leaving work. As a Christian I felt guilty that I could not see the good things in my life. In 1997 I decided to get on with life and started some rehabilitation with the C.N.I.B. Then my dad was diagnosed with prostate cancer, two weeks later my horse died, two weeks later my husband was diagnosed with malignant melanoma, then during my dad's lengthy hospitalization in Ottawa my mom had heart problems and was hospitalized in Pembroke. Through all of this I became a walking zombie but my brothers and sister continued to help me with my burdens and said they would always look after me. In November 1997 my youngest brother, with whom I was especially close, was killed tragically in a car accident. For over a year the grief consumed me ....but I sure put my RP in perspective. He did alot of fundraising for RP and always tried to get me to see the positive things in my life. During the last couple of months I have felt stronger..from my faith in God, the prayers of others, and I think strength from my brother. I still hate the fact that I can't drive but I'm beginning to enjoy life a bit more now. I have recently purchased another horse who is trained by voice commands. Her previous owner is completely deaf and she's the most positive and energetic person I have ever met. I only have to watch the horse's ears with my tunnel vision and she'll do the rest. I know I'll start to feel more independent and alive again. My brother's death has taught me that having RP certainly isn't the worst thing that can happen to a person. I have recently returned from a trip to Toronto to see my opthamologist at Sick Kids and found out my field has narrowed again. I'm not even upset!!!"I can do anything through Him who gives me strength."


Retinitis Pigmentosa by Richard King

Life seems to have taken a whole new direction.I suppose this began about 6 months ago when I woke up to the fact that I have a peculiar "condition", not a disease as far as I am concerned nor do I refer to it as a problem. Just this condition. A condition that requires thought and caution in an ordinarily simple regime of day to day life.A condition that also needs some of my attention to try to understand what is going on, and to consider the various options open to me as a newly labeled "disabled" person.

I became aware of my condition at aged 19 but for some reason I can't honestly remember how or what led us (my brother and I) to Moorfields Eye Hospital in London on this particular day, all I remember is being there as a whole family, my parents, (having divorced 5 years prior) brother, sister and me sat in this dull room confronted by a big nob medical Professor and his cronies. "Retinitis" and "Pigmentosa" were the words being banded about. "What the hell is that?" is this guy having a laugh or what? "Just tell me why I'm not seeing very well in the dark and tell me how I can get it fixed". No chance. No cures, no treatments, no vision is what you have to look forward to. From that day and for the next 11 or so years, this has always been on my mind of course, I had more or less ignored it. Perhaps a denial thing, perhaps I just wanted to consider myself as a 'normal' person.

Recently I have been thinking more of my condition, constantly thinking of my future; as a provider, a father and a husband and towards the end of last year I was almost at breaking point; "What is happening to me?" I was in some kind of major rut. I still manage to do normal things; I go to work every day, work out at the gym, ride my bike, play games with the kids, but I have certainly noticed differences in things and I can and can't do as well as I did 10 years ago. I suppose I decided now was the time I wanted to look into what the future lies in store and began researching this condition. This has helped in the way I now deal with things, my life has changed for me now; being open and honest, not hiding anymore. It is one of the best thing that has happened in my life in years. I am communicating with some great people through RP and it is in encouraging to know so much support and information is available.


Retinitis Pigmentosa by Geof Collis

At this writing, I am 42 years old, and have been legally blind for 15 yrs. I was diagnosed when I was 27. I first found out I had R.P., through a simple field vision test. Boy what a shocker. One day your feeling on top of the world, next, it all comes crashing down around you.

My opthalmologist wasn't exactly very kind in his delivery of my condition, either. It's like they say, "What you don't know, won't hurt you." One day your playing the best hockey of your life, next, you can't do anything right. I spent the next 3 months asking all the usual questions. Why me, blah blah blah. I would try to make my eyes work. Finally I just accepted it and went back to the way I was, sort of. Once you find out, it's never the same again.

I was driving at the time, so it was easy to get back to a somewhat normal life. As I grew older my eyesight started to deteriorate, and so I had to change my life with it. At 33 my night vision was getting so bad, I wasn't sure if I was turning into a side street or the ditch. I was driving a lot for my job, so I decided to ask them to help me to adjust, by not having to drive and just work in the one store. Yeah right! Their answer was to fire me. Luckily I had a Long Term Disability plan to fall back on (later on the insurance Co. would put the screws to me as well). Insurance Companies, or Government for that matter, should not be allowed to tell the disabled what to do with their lives. Until they walk a mile in our shoes they should respect our condition, and all the obstacles that go with it.

Life is always dealing you curveballs, but you have to be able to deal with it. In retrospect, the Insurance Company treating me like crap, turned out to be a blessing in disguise. Settling with them allowed me to set my own agenda, I was able to get married and buy my dream home in the country. I find the country to be less stressful, no utility poles to walk into, no cars trying to run me down in intersections, no crowded malls, nothing but wide open spaces, and fresh air. Aaaaahhh!!!!!

My eyes are getting worse, but it's easier to deal with, when life is not so 'In your face'. I feel living in the rural area has bought me a little more eyesight


Inspirational Story of a Young Woman with Retinitis Pigmentosa

Elizabeth Troutman is 25 and going blind.

She can’t see the sun rise or the stars at night. She hopes to have children one day, but will never know their smiles or their frowns.

She rides the city bus uptown to work in the mornings, a pretty woman in high heels, with nothing to show she’s almost blind until she steps off the bus and opens a collapsible white cane with a red tip. She tap-taps the pavement, ears alert to approaching cars, feet intuitively following the curve of the sidewalk, then through the glass door and up the elevator to her office. There, a computer reads aloud the words on her screen.

You might expect someone with a progressive disability like hers to be overcome with depression or anger, and some people are. Not Elizabeth. She has faced this future for 20 years, gradually losing eyesight the older she got until all that’s left is a tiny keyhole of sight in her right eye.

Though she became legally blind in high school, she lived in Paris for a semester during college and graduated with honors from Princeton, where some classmates thought at first she drank a lot because of the way she stumbled around campus at night. She now plans to go to law school.

There are, Elizabeth believes, much worse misfortunes than not seeing. In her job as assistant director for development at the Council for Children’s Rights, she meets boys and girls who have been sexually abused, who struggle with autism, whose parents neglect them.

Elizabeth would rather be blind.

Still. If she could see.

Elizabeth – Lizzie to friends – was 5 when her parents found out.

At her annual doctor’s visit, she failed the eye exam. Her parents, Liz and Haynes Lea, weren’t alarmed. Haynes wore glasses and they suspected Elizabeth might need glasses, too. At an Easter egg hunt she ran right past eggs her 2-year-old sister spotted.

They took her to a pediatric ophthalmologist, an appointment that changed their lives so dramatically they remember the date: April 6, 1989.

As parents, we envision endless possibilities for our children, who we hope they become, what they might achieve, the adventures they’ll have along the way. Perhaps unfairly, we project our own expectations on them.

The doctor’s diagnosis was beyond anything Haynes and Liz ever imagined. Or had ever heard about.

Elizabeth, they learned, has a disease called retinitis pigmentosa. It was destroying the retina on the back inside walls of her eyes, where images are captured. She would gradually lose her ability to see.

There was no way to predict how far her eyesight would deteriorate. No way to stop the progression. No cure.

That night, Haynes retreated to the basement of their house so Elizabeth wouldn’t see him cry. He felt weighted down by incredible sadness. And fear. What, he wondered, would become of Elizabeth’s life?


A child’s perseverance

Elizabeth, the oldest of three girls, was too young to understand.

 They told her as much as they felt a 5-year-old could grasp: You’ve got a problem with how you see. And it’s going to get worse. We don’t how bad, but we want you to know we will always be here for you. And we want you to know everything is going to be OK.

Not long after she was diagnosed, Elizabeth was playing in her bedroom, stringing a necklace of beads. It took her much longer than it would have taken another child, as she struggled to thread the tiny string through the tiny holes. Finally, she added the last bead. She was so proud, she held up the necklace for her mother to see. That’s beautiful! Just then all the beads scattered across the floor. Elizabeth had forgotten to knot the end of the string.

Her mother teared up.

Don’t worry, Elizabeth said, I’ll just do it again.

And Elizabeth did.

That perseverance would carry her through the next 15 years, through the ups and downs, through the scrapes and bruises, the sadness and anger, of going blind…..



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