 |
Haven Library |
(The following are questions and answers I personally dealt with as a member on the panel at the 1976 Regional Workshop of the National RP Foundation held in New Orleans, October 15. At the time I was president of the Corpus Christi Chapter.) Question 1: What would you recommend to counteract depression as an RP Family member? Answer: As a person with RP I would say: "keep busy". At first you might feel that you have to keep busy in order to survive in a mentally healthy way. Later, your small accomplishments and developing self-confidence will challenge you to want to channel your energies toward constructive goals. In other words, you will want to make RP work for you, not against you. Question 2. What should you tell your children about why your sight is poor? Answer: I would tell them "like it is," as a fact -- and with objectivity. Their attitude toward the RP situation will be reflected largely by the way in which it is presented. Question 3. What can you do with home decorations that will help your husband or wife see better -- (lighting, contrasting light and dark colors)? Answer: I find that fluorescent lighting to be especially helpful in my kitchen. Plain colored tablecloths are better for me than plaids, flowers and bright multi-colored designs. Drinking glasses are seen more easily if there is a pattern on the glass, which is colorful. A family room or den will appear more cheerful if the room has subdued lighting. If bright colors are used in decorating, It will also help "spot" certain objects and furniture in the room. And please, no furniture mover fanatics! Another note about fluorescent lighting: it is cool light, does not cast shadows, and proven more economical if left turned on. Question 4. How can you persuade your or your family member who has RP, to give up driving? Answer: No one could effectively persuade me to stop driving. It was not because I was stubborn or didn’t care. I really did not think I was not seeing everything I should be seeing while driving my car. I rationalized that since I had had no accidents I was just as good a driver as the next person who did not have RP. It was not until our transfer to New Jersey did I realize that the familiar territory of my "driving range" in Corpus Christi was camouflaging the real situation. I realized then that I had been stretching my good luck far too thinly, and that if I had caused an accident, which resulted in bodily harm, I wouldn’t be able to live with the guilt. I stopped driving at age 32, with a peripheral field of 15 degrees. (Legal blindness is also assessed as a result of loss of peripheral sight, at 20 degrees or less in both eyes. Question 5. From parents of RP children: Should I explain to each of the teachers? Should the teacher explain about your child’s vision in their prescience? Answer: This question did not apply to me personally, but from observation of trends in society today, openness and frankness are the tools for understanding. Question 6. Have you developed any special techniques, aids, or methods of overcoming your sight limitations? Answer: Definitely. You learn to become an expert manipulator of your body. My neck muscles get a good workout while crossing streets or walking in crowds, and I have learned to feel comfortable about slipping my arm underneath someone else's when I need to go somewhere less familiar to me. I've learned that slowing down my pace of walking decreases the chance of running into objects or tripping over things my peripheral vision can no longer pick up. Question 7. Would a directory for RPers looking for work or information about specific careers be helpful? Should this be made available to agencies or institutions interested in placing handicapped persons? Answer: I feel it is definitely needed. Agencies such as the Commission for the Blind have dealt largely with congenitally blind and those becoming blind suddenly. These agencies are not familiar with the specific, long-range needs of a person with RP losing sight gradually. Question 8. Should the Retinitis Pigmentosa Foundation, with the assistance from professionals in the field of placement, prepare literature of general advice covering such subjects as how to answer questions on an application, and provide legal information relating to discriminating employment practices? Answer: Yes. It would be helpful. At this time, an agency counselor provides this assistance from beginning to end of a client's caseload. But, if I may add, not too many people with RP seek help from these agencies. Question 9. Do you feel it is important for a person to know whether he/she has Retinitis Pigmentosa? Answer: Absolutely. How can you begin to understand or justify the stupid and clumsy things you do? There is reluctance among some doctors to sit down and talk with their RP patients. They feel that if they can’t treat RP or cure it, why burden them with the anxiety of knowing about it. Question 10. Should children in my family be told about the inheritance of RP as soon as they are old enough to understand? Answer: I believe so. But again, first of all, the attitude of the parents has to be stable and objective; always supportive. Question 11. Is it important to make the diagnosis of RP as soon as possible in a child (although he/she may not be told immediately)? Answer: Definitely. Mothers will want to know the probability risk of another child having RP. When parents have been informed through genetic counseling, the decision whether to have another child always remains with the parents. When it is known early that a child has RP, the "special" educational process can begin early and slowly. Children are more adaptable than we give them credit for. It is the adult who many times causes problems with guilt feelings and overprotectiveness for the child who has RP. I realize it is difficult for parents not to have such feelings, but I also realize only too well that the attitudes of parents toward a child with RP are critical to his or her well being. Remember, it’s not what he has (RP) that is going to bother him half as much as the negative attitudes of others toward him as a person with RP. Question 12. Is it best to tell a child about his/her diagnosis as soon as he or she is old enough to understand, or is it better to wait until symptoms appear and the child is concerned about what is happening? Answer: It is always better to let the child know what is happening so that proper adjustments can be made. When symptoms have already been occurring, the child could be experiencing night blindness without the parents realizing it. I was 16 years old when I first noticed something was wrong. Even then I wasn't sure that something was amiss. If there has not been any knowledge of RP in the family, a child may go through the entire 12 years of school without Incident or problems enough to warrant a visit to the ophthalmologist. Had I been aware of what RP is at an earlier date, I would not have had such a feeling of self-condemnation. I was 25 years old before I went to a specialist. Ironically it was not for the clumsiness or lack of sight at night – I thought everyone else had the same problem). Instead, it was cataracts causing blurring vision while trying to read. In my case, there was no public education about RP for my mother to be aware of. Today there is. If a mother chooses not to tell her youngster about RP, she may have to face an accusing question later on: "Mother, why didn't you tell me? Are you ashamed? "TWENTY-
FIVE YEARS By Dave Bakker There is a very old saw that says: "If life gives you nothing but lemons, then make lemonade." Whether or not Dorothy Stiefel of Corpus Christi took that ancient admonishment to heart, she was handed by life what a lesser woman would would regard as a "lemon" – and she has dedicated her life to the making of her own special kind of "lemonade" for the past 25 years! Stiefel is an internationally recognized lay authority on the dread malady retinitis pigmentosa (RP), a genetic disorder which can rob a person vision, and in some instances hearing. "I had been diagnosed as having RP when I was 25, -- just days before my wedding day," she said. "I was told that I would be blind by the time I was 40." Now, many decades later, this mother of seven and grandmother of 16 has fought an ongoing battle to retain some degree of her vision. "And here I am, a full-fledged senior citizen, and by the grace of God, I still have some vision left. I use two hearing aids and very powerful eyeglasses, so I can still look you in the eye when we talk, she chuckled. "Did life hand me a lemon? I suppose so, but I know a whole lot of other people far worse off than I. You see, God gave me the talent to write, the skill to use a computer, and the patience to listen to others," she commented. "He also gave me a good sense of humor." Since her first symptom appeared – failing night vision – Dorothy has been "making lemonade." For 25 of those years she has been the spark plug for advocacy, and has lent her years of experience and understanding to others that have sought her counsel. With consummate skill she developed the non-profit Texas Association of Retinitis Pigmentosa (TARP), a support group that she founded in Corpus Christi in 1979. Her TARP hotline and fax machine bring in calls from all over the world. Because Stiefel sees and seizes life with a gusto that most people can never approach, and because of her passion and understanding of others and their difficulties, she is in contact with people around the globe who are affected by RP and other vision impairments. Her four published books are available internationally, and she is sought out as a guest speaker and counselor wherever her travels take her here and abroad. "It was in New Zealand that I received the finest compliment of my life. My hostess said to me, "Dorothy, you are truly a catalyst for change," she recalled. Although she sees the world with only a few degrees of vision, Dorothy sees more profoundly than most people do. Each detail is more precious and precise for her; she writes and lectures with a clarity that bespeaks a much greater awareness of the world and its beauties than most people possess. Perhaps that is because each moment is a spiritual experience for her. Instead of bewailing her sealed fate, Dorothy has worked for more than two decades to make the world more palatable for herself (including two powerful hearing aids and strong magnifying prescription glasses for reading) and for other RP victims. She has given a whole new significance to the concept of advocacy. Her Texas Association of Retinitis Pigmentosa (TARP) is a support group and clearing house of information and advice regarding RP and many other disabilities. Under Dorothy’s astute guidance, TARP has achieved worldwide recognition through her publications. As with many disabilities, RP knows no political or ethnic boundaries. As a seasoned homemaker, Dorothy’s house is neatly and carefully kept with every piece of furniture, every cooking utensil, every gardening tool stored and returned and stored in a specific location. In her kitchen you will find markings on the function knobs of appliances making them accessible to a visually impaired person. The same is true of her laundry room. Most surprising of all is her office and equipment room that fills what was once a formal dining room. The computer, printer, fax and copy machines you would find in any well managed office – with the exception of the computer screen that enlarges text to any size she needs for working, and the enlarging viewer, a Closed Circuit Television (CCTV.) Dorothy can place a printed page on the base plate of her viewer and then be able to magnify the words to any size comfortable for her to read … and she is a voracious reader. It is from this office that a stream of works has flowed. Including her four published books. Her "Knotholes are for ‘Seeing’" is an allusion to her narrowed field of vision (referred to as tunnel vision) as viewing life through a knothole. In this book she has recorded her life and observations in extensive poetry, prose, and remembrances. "With my narrowed field of vision (I have absolutely no peripheral vision), much of my sight and hearing is triggered by thought imagery," she explains. "I have to go after what I want to see and hear. I have been "knotholing" for years and have learned to turn it to my advantage in terms of concentration and comprehension. I have had to because I am blessed with an insatiable curiosity about life and living. "Knotholes are for Seeing" covers a major part of my life span in which the world of movement and color has become smaller and dimmer, and always with the threat of closing in on me completely. But as my sight has diminished, my writing has reflected more of how my personal growth and awareness of life have expanded! "It seems to me that it is not so important how much one sees, but rather the quality and meaning of what the mind’s eye envisions," she said. And maybe that’s what "making lemonade" is all about.
Dorothy adds: "but when someone calls the Hotline, they get my full attention. I guess that’s because I’m a "people person" she concluded. WHAT‘S
IN A By Dorothy H. Stiefel Support services have many names for different folks. Some just need a listening ear. Many want to know how to find the most information about RP. Others have a spousal problem. Each case is very different from another but one factor binds us all: we need to know we are worthy and that we can get through this lifelong struggle to keep our vision intact for as long as possible.
A diagnosis of Retinitis Pigmentosa is viewed by some as having received a death sentence. It becomes an emotionally charged change from normalcy to lifelong captivity. From the instant of diagnosis and chilling prognosis, RP never leaves a person alone without fear of what the future will hold. In some way or another RP touches the lives of everyone involved with the individual who has just received the shocking news. Because RP is not a visible problem it is difficult to understand why a person is behaving so strangely: bumping into large objects such as posts and people, or misjudging distance and tripping over objects in full view. The affected individual really *thinks* all things within the parameters of a wide field of vision are being exercised. Instead, a huge chunk of the field used for spotting moving objects is gone, having slowly disappeared over a number of years. Without a way to measure or "see" what is missing, a person with RP finds it easy to fool himself into thinking that he really has no problem at all and blames such incidents on clumsiness. THE LONG, WHITE CANE The "long white cane" is one of the most important mobility aids to effectively augment safe mobility within a person's home or work environment. However, this tool of independence is often resisted by many who fear that using a cane will "let the cat out of the bag." Individuals worry about losing their jobs, their friends, and their loved ones. Such delay in utilizing a mobility aid often leads to more serious falls and injuries. It also leaves a person with a feeling of despair and loss of self-esteem. This is why support groups have become so essential to people with RP. GUIDELINES
FOR FACILITATING CONFIDENTIALITY: Everything that is said during the meeting is to remain in the meeting; no one who is seen there or anything that is said there should be discussed there. This means no talk of "So and so said in the Support Group meeting last week…." PARTICIPATION: It is important to the Group that each person attending consider himself or herself as an active participant. No one likes the feeling of being "observed" by someone who is not willing to share about himself. This does not mean that everyone must speak. Rather, it is the attitude of being part of the Group that counts. Everyone is welcome whether or not he/she is ready to talk. LEADERSHIP: It is the responsibility of the facilitator to start the meeting, to see that everyone has the opportunity to share, to guide the flow of discussion and to set limits when necessary. However, all Group members are responsible to be open and to say so if the Group is getting off course or if something that is going on is not in accordance with the purpose of the Group. AVOID TALKING ABOUT "THEM" This is essential. The focus needs to be on constructive ways to support each other and ourselves on our thoughts, feelings, and-behaviors, rather than on the behaviors of others outside the Group. Some mention of outside pressures is inevitable, but should be kept to a minimum. NO ATTACK: No one should be criticized about what he or she does or does not do, either when he is present or absent from the Group. Although members are free to ask for feedback from each other, this should be given in a caring way along with expression of concern for the person. LIMIT ADVICE-GIVING All are welcome to share their experiences and what has worked to help them feel better, but no one should presume to know what is best for another. This means... tell what works for you, but don't say, "You should do such and such." STICK TO THE TOPIC AT HAND Virtually any topic dealing with quality of life, health, self-care, stress management, human potential, growth and development, or ways to support each other is fine. Refrain from politics, religion or therapeutic modalities. There is no room for debate or indecisiveness in a support group. Remember, remember, you are not here to gripe about groups of people such as doctors, churches, officials, etc. You are here to participate in your own growth and healing. You are here to share how you are developing new tools for coping with old problems and unhealthy ways of living. GIVE YOURSELF PERMISSION It is difficult to share one's thoughts and feelings in front of new people. However, this is one of the best ways to grow, as long as the setting is a caring atmosphere. Decide that you want to gain all that you can from being part of the Group. Pat yourself on the back for speaking up when it feels scary or awkward. It WILL get easier! STAY IN TOUCH WITH YOURSELF: Pay attention to how you are feeling within the Group. Be aware of your physical sensations as well as your emotional responses. Use this as an opportunity to get to know yourself better. Without criticizing yourself, keep track of how you react and respond to certain topics and issues in group sessions. Keep aware of behaviors of others as well as your own In the Group. IT DOESN'T HAVE TO BE HEAVY: Even though the topics of discussion are important, and your purpose for being in the Group is very crucial to your well being, you can still allow yourself to see the light side and the humor in yourself, your situation, and those of others. Laughter heals! Before Group Starts 1. First take care of yourself and your needs. Share with your teammates anything that may keep you from being fully present. (i.e., communicate any upset: fears, competition feelings, etc.) 2. Arrive 15-30 minutes prior to group to connect with your co-facilitators, share whatever needs to be shared, get centered, check the room for arrangement and necessities, set the tone of warmth and peace. 3. At the end of your group meetings, stay for 5-10 minutes extra to de-brief with your co-facilitators. During Group Process:
From the 1997 Archives of the RPM
Ó 1999 By Dorothy H. Stiefel Digital technology promises to revolutionize the hearing aid industry and the quality from which wearers will ultimately benefit. But don't hold your breath nor be in a hurry to be a recipient of the new digital devices which are mostly worn in the ear canal but may be also be found in behind the ear shells. Other considerations should be noted because these programmable marvels are not for everyone. Digital instruments are programmed by the hearing health professional to meet the acoustic needs of the patient. One must be cautioned from trying to reach unrealistic conclusions based on the information presented by the media. Digital technology does not resolve all problems associated with hearing loss and the processing of acoustic information. For example, digital hearing aids do not resolve those problems of aging and the possible reduction of one's ability to process auditory information efficiently. For some individuals, the aging process and other conditions create a central auditory processing disorder: the inability to understand speech efficiently under various conditions of listening. Some older individuals with Ushers 2 may find themselves in this range. When speech is delivered rapidly and background noise is present, it is much more difficult for some than others even though the actual degree of hearing loss may be the same. As improvements continue and programming becomes more fine-tuned, the jury is still out. It will take the brave few who also find the cost affordable to be the pioneers (and guinea pigs) before all the rough edges of tomorrow's technology have been smoothed away. Meanwhile, it is prudent for us who need and want to hear better, to be become well informed and keep up with progress in the works. Some day soon digital technology will offer a new beginning and great promise to the hearing-impaired person. Significant
advantages of a true
(Source: Robert E. Sandlin, Ph.D., Adjunct Professor of Audiology, San Diego State University, San Diego, California.) Go To Top Confrontation Testing A Family Project Ó 1999 by Dorothy H. Stiefel For many years after I found out that I had Retinitis Pigmentosa (RP), I did not understand my kind of "seeing." Why did I sometimes see things and at other times not? I felt that my eyes were playing tricks on me or that perhaps I wasn't paying attention to what I was doing. Doctors talked to me about peripheral or field vision. I did not relate those words to the way I was functioning with my eyesight. Most literature about RP describes the loss of vision as "tunnel vision." I would make two little round circles with my hands and look through them as one would peer through a telescope. Nope. It still wasn't what I was seeing. I was seeing more than that, but yet I wasn't seeing *all* of it. In 1977, when I was involved in trying to educate the local Board of Education about the importance of vision screening for children. I wanted to focus on peripheral vision, rather than just acuity, or straight-ahead vision. I went to several training classes and learned about simple screening for peripheral vision . . . confrontation testing. At one of the rap sessions (get-togethers for people who have RP and related vision problems) we discussed about how much each of us could see in the periphery while looking straight ahead. The five of us at that particular session tried out the confrontation method on ourselves. This little technique helps everyone involved to understand how a person with RP is actually seeing. I think this exercise is especially effective to use on small youngsters because it can be performed in game-like fashion. The usefulness of this idea was verified by an optometric/psychologist who was giving a lecture at a seminar in Dallas I attended in the fall of 1977. I believe it is important that all of the family members participate in the confrontation session. No tools or devices are needed. If small children will be watching and following the exercise, it will prove useful because they will then understand why it is important to pick up their toys off the floor and to put things back where they belong. When an adult with RP does the testing, another participant should be facing him/her approximately 6 to 8 feet away as a target individual (also assures no cheating! Follow the procedure outlined below: Lift your arms and hold them straight out from your sides. Make a clenched fist except for the index fingers which you position vertically, pointing toward the ceiling. The person who has a peripheral vision problem will be sharply aware at the start that the moving hands are not seen far out to the sides, and as they move in to a mid-range point, they seem to come into focus (the mid range) Others doing this exercise will have no difficulty and will seem mesmerized at the "now you see, now you don't situation of the RP participant. The person with RP continues to state when the fingers are no longer seen until all of a sudden at about shoulder distance away (if the person still has a fair amount of vision, will see their fingers coming in to touch the nose. The smaller the "tunnel," the further in toward the nose the person has to come before the fingers are seen. If this confrontation exercise is to be used for a youngster, for the sake of this description we will assume that a child has RP and is ready and willing to follow your instructions. Place your hands in your lap and ask your child to go through the motions given above, reminding the youngster to keep their eyes fastened on your nose. For comfort and to level the position between the two of you, a stool or cushion for the child may be necessary. It is important that the youngster is not looking up or down at the target: your nose. Ask your child to let you know when the moving fingers can be seen, making sure that eyes remain fixed on your nose. If the child seems to become confused by using both hands simultaneously, doing one side at a time may be easier. If the child gets to a spot where movement of fingers cannot be seen, ask the child to wiggle them a little more during the progression toward the nose. (This is to make the test more representative of an active environment.) After the exercise you will be better equipped to explain the reason why there are "blind spots" in the vision which are responsible for the accidents (falling and bumping into things), not clumsiness. Children are smart and if approached in an intelligent manner to "help solve the mystery of ..." they will get into the game-playing mode. You as a parent will learn how much side vision is missing which will assist you in telling your child what to watch for. After this procedure, the same test can be done with up and down movements, but with holding the hand horizontally close to the body overhead and coming down slowly, wiggling fingers until the hand is seen while the child is focusing on your noise. Reverse the procedure, coming from the lap to overhead. After you learn what the child with RP can or cannot see in the periphery at close range, you can check what can be observed from a closer or farther distance away from your position. As you perform the hand movements it will become obvious what area of the immediate environment is a "blind spot"... or more accurately ... a ring scotoma of lost rod and cone cells. If you try this procedure on yourself, you may be surprised to notice a tiny area off to the side that seems to have active cells. This is not unusual. However, the most critical peripheral vision for engaging in active sports is an area from 15 to 25 degrees from center. This field of sight is necessary for walking around safely and easily, also imperative for defensive driving. This information becomes very useful for children and adults alike because the "missing" area becomes more concrete, something that can be dealt with in more tangible terms. Training oneself to dart the eyes back and forth and scan with neck movements is a compensation technique that will give persons with RP a better view of their immediate environment for a longer period of time. At whatever age the child, teenager or adult discovers where this "seeing" is consistent and dependable, and where it is not, much stress and anxiety will be alleviated. If and when young children ask why they can't see their fingers at a particular place in the field of vision, a simple but truthful response can be given: "The little cells of the eye are responsible for what you are seeing, There are so many cells that they are like a special army with a job to do. They send messages to your brain to make sure you see everything you are supposed to see, Some of these little messengers can not do their job any longer. (An older youngster could be told that the rods and some cones affected have deteriorated or for some reason are not working.) Doctors are trying to find out why they cannot do the work they are supposed to do. Right now, they do not know how to make these cells work." Remember, persons with RP, whether they be children or adults, hove the right to know what is happening to them—*they* will be living with it 24 hours a day. Give them the tools to work with the problem so they can get on with their lives. If they better understand themselves they will be less hesitant to talk about their problems to others. As more and more people become informed about Retinitis Pigmentosa, schools, agencies and doctors will be better equipped to do their share in dealing with its functional difficulties. THE USHER SYNDROMES © 1999 by Dorothy H. Stiefel The Usher syndrome is a genetic disorder involving congenital sensorineural deafness, Combined with 'a form of retinitis pigmentosa (RP), a hereditary retinal degeneration that manifests symptoms in the early teen years. First recorded as early as 1859, this dual sensory deprivation was named in 1925 after C. H. Usher, a British ophthalmologist. It was Dr. Usher who first described deafness occurring with "retinitis pigmentosa," a Latin term meaning "inflammation of die retina," an unfortunate rnisnomer which has prevailed for more than a century. Four types of Usher syndrome are well documented in medical history, but current literature primarily refers to two basic types: Usher Syndrome type I, and Usher Syndrome type 2. USHER 1 consists of the combined neurosensory deficits of profound hearing impairment and a progressive RP-like retinal degeneration. The hearing loss is usually severe and stable but vision impairment progresses to "legal blindness." defined as 20/200 (or worse) acuity (central) vision and/or 20 degrees or less peripheral (side) vision. Usher 2 is considered much more variable in severity and in rate of progression. It is not uncommon for persons with mild hearing loss and no visible symptoms of RP to remain undiagnosed for many years. Progression of field vision loss is slow and erratic, sometimes stabilizing for a number of years. These subtle, seemingly non-threatening symptoms frequently remain unnoticed by family members and friends until the classic symptoms of RP become bothersome --or until a serious accident suddenly occurs. THE SYMPTOMS Nightblindness is the first major symptom. RP primarily affects the photoreceptor rod cells that govern all that we see at night and in low illumination. This 120-million plus "army" of light-sensing cells also provide peripheral vision--all of what we normally see to the sides while looking straight ahead. Cone cells, clustered heavily in the acute, five-degree fovea (central vision), are less likely to be affected until much later in life. The presence of cataracts, not RP, more often is the fore-running indicator that sends a person scurrying to an eye doctor for another pair of glasses. Such was my case. Instead of being fitted with another pair of lenses, I received a prognosis of impending blindness. "Tunnel vision" is usually the second major symptom. As rod cells continue to degenerate, unexplained mishaps increase. Stumbling and bumping into objects and people become commonplace. The individual feels stupid and not understanding what is happening becomes frustrated. To make matters worse, it is not unusual for the person to be called "clumsy, even by well-meaning family members and friends. It is the beginning of anxiety, self-doubt, and losing self-esteem. WHEN THE DIAGNOSIS IS USHER SYNDROME
|
||||
