Sense Information and Advice Living with Usher syndrome Mobility and travel Mobility is important in our everyday lives, as we all need to move around our homes and communities, to get to work or education and to socialise with our friends. A diagnosis of Usher syndrome, or a change in vision, can cause a loss of confidence and present challenges with mobility. This, in turn, may lead to someone with Usher syndrome feeling that they are losing their independence. Understanding something of the effects of Usher syndrome may help, as may considering some basic strategies which can help people with impaired vision to move around more safely and independently. Clearly everyone with Usher syndrome is different - both in how the condition affects them and how they respond to changes in their vision. This factsheet offers some broad guidelines and strategies to help maintain confidence and independence. Anyone with Usher syndrome who is concerned about their mobility should speak to a Mobility Officer or Rehabilitation Officer for Visually Impaired People (ROVI), who may be contacted through their local authority Social Services Department. The effects of Usher syndrome Loss of peripheral vision People with Usher syndrome have an eye condition called retinitis pigmentosa (RP). The retina, at the back of the eye, has two types of photoreceptor cells which respond to light. Rod cells are found on the outer edges of the retina and are responsible for night vision and peripheral vision (awareness of objects to the side). Cone cells are found at the centre of the retina and help us to see detail and colour. In RP changes to the rod cells cause a person’s peripheral vision (or field of vision) to reduce. The result is often called tunnel vision, as it is similar to looking down a tunnel or tube. When looking forward, people with “normal” vision are aware of objects and movement within about a 180-degree field. If they lift their arms up to the side of their body in line with their shoulders and then move their fingers they should be aware of the movement to both sides, when looking straight ahead. For people with Usher, this view is often much reduced. Consequently they might bump into pedestrians or lamp posts or miss overhanging tree branches when walking outside, or trip over furniture, small children or the family pet at home. Difficulty seeing at night or in low light Rod cells require less light to function than cones and are the main source of visual information in poor light. Changes to the rod cells will therefore also cause difficulty seeing at night and adapting between different lighting conditions. People with Usher commonly have difficulty walking down a dark street unaided, or moving from a sunny outdoor area into a dark building. Somebody with “normal” vision will find that their vision adapts from light to darker conditions within a few seconds. For somebody with Usher this adaptation may take several minutes, or even longer. Balance People with Usher Type 1 often have very poor balance, as the condition causes a malformation of the vestibular organs in the inner ear which are responsible for balance. Generally, the balance of people with other types of Usher is not affected in this way, although recent research indicates that up to 50 per cent of people with Usher Type 3 will develop some balance problems. Balance problems can have a significant impact on mobility. For example, someone with Usher Type 1 might have difficulty walking along a straight, narrow path or standing in one position without support for any period of time, particularly in the dark. Making the most of your mobility If you have Usher syndrome and your vision changes gradually, you may make adjustments without being fully aware that you are doing so. For example, you might hold on to a friend or partner when coming out of a theatre or pub at night, or you might move your head from side to side more than usual, in an attempt to scan for obstacles on the pavement. There are some other simple things that you can consciously do to improve your own confidence and help you to move around more safely and independently. Walking outside Get to know regular routes in daylight Familiarising yourself with a route over time will enable you to learn where obstacles are, such as street signs, steps, crossings, bollards and kerbs. Plan your route Try to plan a route to avoid heavy traffic and busy pedestrian areas, particularly during the rush hour. One-way streets may be easier to cross, as may streets with wide traffic islands. Tactile surfaces (bumps) on the pavement indicate crossing points. Avoid areas with low light Even in busy urban areas outside lighting levels vary considerably. For example, it might be easier to walk at night along a shopping street with brightly lit window displays, than along a residential road. Use pedestrian crossings Pedestrian crossings offer a safer alternative to crossing a road than elsewhere. They are generally well lit and allow drivers to anticipate that somebody might be crossing. When using a Pelican crossing, ensure that the green man has appeared (this is often accompanied by an audible signal) and double check that traffic has stopped. Pelican crossings sometimes have a small rotating cone underneath the push button box. You can hold the cone and when it starts to turn, you know that the green man is showing and it should be safe to cross. However, it is also important to beware of such hazards as emergency vehicles or cyclists coming up the inside lane of the traffic. Walk at a steady and even pace We are all liable to bump into people on the street who weave from side to side or stop suddenly in the middle of the pavement. Walking at a steady and even pace allows others to anticipate your movements and keep out of your way. Try to walk down the middle of the pavement, as this is more likely to be free from obstructions. Use residual vision Many people with Usher or RP have some useful central vision. Moving your head to scan from side to side and up and down may allow you to anticipate obstacles. However, this also requires a lot of concentration and can be very tiring. Consider using a cane or mobility aid Some people with Usher syndrome are at first quite reluctant to consider using a cane, as it is a sign that they have a visual impairment. However, when used correctly, a cane can be of great benefit in supporting people with impaired sight to move around safely and independently. A qualified Rehabilitation or Mobility Officer will be able to assess which type of aid is appropriate and provide guidance and training in its use. There are three main types of cane: a symbol cane is a short cane that can be folded and kept in a pocket or bag. It can be brought out in busy pedestrian areas, or when crossing a road, to indicate to others that you have a visual impairment; a guide cane is longer and is used to help detect steps, kerbs and other obstacles immediately in front of you; a long cane is guided along the ground at a distance in front of you to provide information about upcoming obstacles. It is a specialist mobility aid, which should only be used by people who have received training from a Rehabilitation or Mobility Officer. Additionally, a solid walking stick may be of benefit to people with particularly poor balance. All of these aids are available in red and white, which indicates that the person using them has both a hearing and sight impairment (as mentioned in Section 207 of The Highway Code). Moving around outside after dark Walking around outside in dull or dark conditions can be particularly challenging for people with Usher. However, there are some simple things that can help. Give your eyes time to adjust When leaving a brightly lit place at night, give your eyes time to adjust. This may take five to six minutes or longer for somebody with RP. Be visible to other road users It is important to ensure that road users and other pedestrians can see you. It helps to wear something that is white, brightly coloured or reflective, for example a pair of fluorescent trainers or a jacket. The fluorescent armbands or belts often worn by cyclists are particularly visible and are widely available at a low cost. Carry a torch Some people with Usher like to carry a small torch with them. This can be useful, for example, when walking in dark areas, reading the timetable at the bus stop or finding the keyhole on your front door. Bright conditions Bright sunny conditions can be equally challenging for somebody with Usher. An optometrist - either at the hospital eye clinic, or at a high street optician – should be able to advise on dark or tinted glasses that can help in these conditions. Red lenses are particularly helpful in blocking out glare. Information on glasses that can be purchased privately is available from the company Mediview - see www.mediviewspecs.co.uk/ A hat or visor may also be helpful. Using a communicator guide, guide or support person Many people with Usher use a sighted guide at some time, perhaps at night or when travelling in an unfamiliar area. This person might be a paid worker who has had formal training as a sighted guide or Communicator Guide, or it might be a friend or family member who offers to help. Here are some tips for walking with a sighted guide. Your guide should have an understanding of Usher syndrome and your individual needs. For example, it is important that they understand that you may see well in certain lighting conditions and have greater difficulty in other conditions, or that you have a problem with balance that affects your mobility. You and your guide should agree on how to communicate while moving about. For example, if you have Usher Type 2 and use hearing aids, you may not be able to follow speech in a busy outdoor area and may prefer to receive communication using the deafblind manual. You also need to negotiate a signal system. For example, when the guide’s arm is raised, this means that they will be going upstairs; when they move their guiding arm behind their back this indicates that they are approaching a narrow gap. Agree a guiding position with your guide. For example, you may prefer to take the guide’s arm gently at the elbow or may prefer to put your hand on the guide’s shoulder. It is important for you to trust the person guiding you and feel that the guide is confident in what they are doing. This trust may be established through training and by individual discussion, negotiation and constructive feedback. Formal sighted guide training is provided by most local and national organisations working with visually impaired people. Please contact the Usher Team at Sense, or your local Sense office, for advice on where to access such training. Guide dogs or assistance dogs Some people with Usher are eligible for a guide dog. If you are interested you should initially talk to your local authority Rehabilitation Worker who will refer you to the Guide Dogs for the Blind Association. A specialist worker from Guide Dogs will then carry out a detailed assessment and, if eligible, you will be matched with a dog and provided with full training and follow-up support. A small number of people with Usher have been provided with dual trained dogs, which carry out the additional role of responding to audible alerts in the home such as the doorbell, telephone or smoke alarm. Transport Most people with Usher will be eligible for support with the cost of travel. For example: Disabled Persons Railcards These allow a companion to travel at a reduced rate too Bus and travel permits Dial-a-Ride or community car services Blue Badge disabled parking permits. Information is available from Social Services, libraries and the UK government website www.direct.gov.uk Individual airlines and railway companies will offer assistance to disabled travellers by arrangement. It is advisable to request this assistance well in advance of your journey. Sources of help Sense Sense is a national charity that supports and campaigns for children and adults who are deafblind. We provide expert advice and information as well as specialist services to deafblind people, their families, carers and the professionals who work with them. Sense aims to help people with Usher to live as full a life as possible, and provides a range of services: * Information, advice and support to people with Usher and their families - particularly when the condition is first diagnosed. * Opportunities for people with Usher and their families to meet together - both face to face and online. * Training for education, health and social service professionals to understand Usher syndrome and its effects. Please contact the Information and Advice Team for further information: Tel: 0845 127 0066 Fax: 0845 127 0061 Text: 0845 127 0062 Email: info@sense.org.uk www.sense.org.uk Sense 101 Pentonville Road London N1 9LG Usher Life www.usherlife.co.uk This is an independent, dedicated, UK website for people who have Usher syndrome. The site includes details of forthcoming social events for people with Usher and their partners. Hearing and Sight Impaired Group (HSI) The Hearing and Sight Impaired Group brings people together to combat isolation and provide mutual support to its members. It holds regular meetings as well as social events. It also campaigns on local and national issues. For more information contact Sense. Counselling GPs may know of suitable counsellors working locally. The British Association for Counselling & Psychotherapy can provide a full list of accredited counsellors working in your region - see www.bacp.co.uk or phone 01455 883316 Any comments? This is one of a series of factsheets that provides information and advice for people with Usher and their families. They were put together with the help of people with Usher, their families and professionals experienced in this area. If you have any comments or suggestions about the content of this factsheet, do please let us know.   Sense 2011