Sense advice and information Living with Usher syndrome Facts and fiction about Usher I got Usher from my mother or my father. False Usher is a recessive genetic condition. This means that both parents either have Usher, or carry a specific gene for Usher, in order for you to be affected. I have Usher because my mother was ill during pregnancy. False When a woman has an infection such as rubella during pregnancy, this may cause sight and hearing problems in some children. Usher is different. It is not caused by illness during pregnancy. It is a genetic condition. I have Usher. If I have children, they will also have Usher. It is possible, but very unlikely In order for your children to have Usher, your partner must either have the same type of Usher as you or be a carrier of the same gene. A child will not get Usher syndrome from only one parent so it is very unlikely that your children will be affected. Anyone with Usher thinking about having a family may wish to get advice from a genetic counsellor. There is no cure for Usher. True At present there is no cure for Usher and no way of preventing the condition. However, much research is taking place and as doctors and scientists start to understand more about the condition and the genes that cause it, treatment may become available. This may take several years. Researchers are currently trying to find treatments to stop retinitis pigmentosa (RP) from getting worse. People with Usher will definitely go blind. False Usher is an individual condition – everyone is different. The extent to which vision changes and the time this takes is variable, but is probably linked to the type of Usher you have. Many people with Usher have tunnel vision and problems seeing at night, but their central vision may remain good for a long time. This means that while they may have difficulty with mobility, they can still see to communicate, read and do other tasks. A person with Usher may be registered bind (or severely sight impaired) because of their tunnel vision, but may still have useful central vision. An ophthalmologist (eye doctor) will be able to tell you more. People with Usher syndrome may get other eye problems. True Some people with Usher may get other eye problems as well as RP. Such problems can include cataracts or macular oedema. When the clear lens in the front of the eye becomes cloudy, this is called a cataract. Although cataracts can affect anyone, people with Usher are more likely to develop them at an earlier age - maybe in their thirties or forties. Symptoms of a cataract include increased problems with glare, “halos” around, for example, street lamps and an increased fogginess of vision. Cataracts can be surgically removed which will usually improve vision. However, the symptoms of the underlying RP will remain. Macular oedema is rarer than cataracts. It occurs when part of the retina swells with fluid. This may be treated by medication but success is not guaranteed. The swollen part of the retina will not function properly and will cause some loss of sight in that part of the visual field. It is important to have your eyes checked regularly and to report any problems to your ophthalmologist. If you develop eye problems suddenly, go straight to your nearest hospital accident and emergency department. Sense and the National Deaf Children’s Society have published useful guidance for families and professionals about how the vision of children with hearing impairments can be assessed and looked after. For a copy of Vision Care for your Deaf Child contact Sense or visit our website. People with Usher will always be dependent on other people. False Many people with Usher lead independent and fulfilling lives. They may go to college or university, have a partner and a family and have a job or career. However, they may require additional support at times - for example a sign language interpreter, or a sighted guide when travelling after dark. People with Usher cannot go out on their own due to their poor eyesight. False Many people with Usher travel independently. As their vision changes, some people benefit from using a mobility aid, such as a red and white cane, to help them move around safely and independently. Your local Rehabilitation Worker with Visually Impaired People (ROVI) will be able to provide advice and safety tips and also give you a mobility aid if you agree that this will help. It is your choice whether you use an aid. Some people with Usher should not drive. True It is dangerous to drive if you cannot see well at night, have tunnel vision or your eyes take time to adapt between different lighting conditions. The eye clinic or the Driver and Vehicle Licensing Agency (DVLA) can provide more information and also advise on whether you are legally entitled to drive. People who continue to drive after they have been told to discontinue driving will invalidate their insurance in the event of an accident and are also putting other road users, and themselves, at risk of injury or death. People with Usher must give up their job when their sight deteriorates. False Access to Work is a scheme operated by the government to support disabled people to get employment, or stay in their job if their condition changes. Depending on the assessment, funding can be available to pay for equipment, such as a special computer, or to pay for support, such as a BSL interpreter or a Communicator Guide. Employers must make “reasonable adjustments” in the workplace to enable the disabled person to do their job. Disability Employment Advisors (contactable through the local Job Centre) can provide more information. People with Usher cannot care for themselves or look after their own children. False Most people with Usher continue to care for their children, prepare meals, clean their home and manage their own personal care (washing and dressing). If any of this becomes difficult, the person (or their partner or family) may ask for an assessment by their local Social Services or Adult Services Department. They may be eligible for aids or adaptations to help (for example, a vibrating baby monitor) or additional support (for example, a Communicator Guide or a home care worker). People with Usher are not entitled to the higher rate mobility component of the Disability Living Allowance (DLA). False The assessment for DLA should take into account how your vision, hearing and balance - together with your specific communication needs - affect your mobility and ability to care for yourself. Some people with Usher are entitled to the higher rate mobility component of the DLA. Before applying for DLA, or if you have been turned down, you may wish to seek advice from a specialist organisation, such as Sense. The DLA rules changed in spring 2011. As well as deafblind people, people who registered blind or severely sight impaired can now claim for the higher rate of the mobility component of the DLA. Please note that your DLA will not be automatically upgraded; you will have to claim. The Government is also planning to change DLA into Personal Independence Payment (PIP), there will be new assessment criteria. Most people on DLA who are between 16 and 64 will be re-assessed from 2013 to see if they are eligible for PIP. Sense is campaigning to make sure PIP meets the needs of deafblind people, including people with Usher 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 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