It is important to focus on the support people with Usher need to lead an active life – for example, the communication and mobility a person needs to make friends and the information they need to plan for the future.

Early intervention, as well as individual and personalised support is vital.

Communication and language

Some people with Usher will use British Sign Language to communicate. As their sight narrows they will still be able to use and understand sign language if people sign to them within their remaining visual field.

If this remaining vision fails some people may then use tactile, or hands-on signing, by placing their hands over the hands of the signer so that they can feel the signs being used.

Others will usually continue to use speech to express themselves. Some may learn finger-spelling and signing to receive communication.

Whatever the communication method used, early intervention is extremely important. A child needs to take advantage of the window of time during which the brain is most receptive to learning language; an adult needs time and support to adapt their communication.

Balance and mobility

Balance problems are usually most apparent when walking without a guide or a mobility aid, or when standing without support, particularly in darker conditions.

Emotional reactions

When someone discovers they have Usher syndrome the news can be devastating for them, and their family. Many people do not realise they have the condition until they are teenagers and begin to have problems with their sight.

As their field of vision reduces, people with Usher can feel isolated. As it becomes more difficult to lipread or see sign language communication difficulties may emerge and need further adaptations.

Some people may benefit from ongoing counselling, which aims to help people to adjust to their decreasing vision and changed life circumstances. They may also benefit from counselling at times of significant change.

Meeting other people who have Usher, or who understand the condition, can break down the sense of isolation and provide support.

Parents are always shocked to discover that their deaf child also has retinitis pigmentosa (RP), and it may take a long time to really accept what is happening. The whole family and friends may go through a period of emotional upset before coming to terms with the situation. Many parents find it very helpful to meet up with other parents in a similar position.

Education

Children and young people will need access to specialist support and adaptations to their learning if they are to make the most of the opportunities their education gives them.

Planning for employment

By their twenties, most people will be in training for employment, or will already be in work. Those who have RP and deafness may well be doing work which will eventually prove difficult or even dangerous to carry out.

The rate at which someone's vision declines in their twenties is hard to predict, and so it may not be easy to decide when to change course. It is important that the young person with Usher receives career guidance that is realistic but not too restrictive.

Technology

There is a range of technology, aids and equipment that can help people with communication, access to information and mobility. This might include hearing technologies such as cochlear implants and hearing aids, communication devices, mobility technology and a range of aids to daily living.

Getting the right support

It is important that people with Usher get the right training and support to learn the skills they need to cope with life when their sight deteriorates.

People with Usher may also be entitled to support from their local authority. The Deafblind Guidance, issued by the Department of Health, requires local authorities to ensure that individuals with combined sight and hearing impairments are assessed by a professional who has received training in deafblindness - in particular to assess the need for one-to-one support, assistive technology and rehabilitation.