Management of Usher syndrome

There are currently no available treatments to prevent, cure or slow the progression of Usher syndrome. Research continues but for now the emphasis is on early identification and management of the condition.

Early identification and management

Early identification of Usher is important so that the right individual support can be identified. Useful support could include:

  • Emotional support - from friends, family, mutual support and professionals
  • Orientation and mobility  
  • Communication – While some people with Usher syndrome will retain sufficient sight and hearing to continue with more typical communication techniques, others will benefit from learning new methods
  • One-to-one support – Some people with Usher can benefit from the support of a communicator guide, essentially someone acting as the individual’s eyes and ears, to help them get out and about and maintain their independence
  • Hearing and vision aids – Hearing aids or implantable devices will be beneficial to many people with Usher syndrome, as are various kinds of magnifiers
  • Assistive technology – There is a wealth of technology now available to help people with vision and hearing loss go about their everyday lives and maintain their independence. Find out more at Sense’s dedicated technology web pages.

Getting support from your local authority

People with a significant combined visual and hearing impairment are entitled to an assessment, under guidance issued by the Department of Health issued in 2014. This assessment is carried out by an individual’s local authority. 

If you are considered eligible then the assessment could lead to you receiving the kind of support mentioned above. Read more about this kind of assessment and the duties of a local authority in our dedicated web pages.

Nutrition

The benefits of eating a healthy, balanced diet are well established. The NHS provides detailed information on how to go about this, explaining how to get that balance right and what foods to avoid. A balanced diet should give you all the nutrients you need.

Being overweight in itself does not seem to be a direct risk factor for any particular eye condition or hearing condition, but being overweight does put you at risk of a number of health problems including:

  • high blood pressure
  • type 2 diabetes
  • heart disease
  • stroke
  • some cancers

These problems could lead to further issues and eye conditions such as glaucoma and cataracts, jeopardising the remaining sight that someone with Usher has.

Annual eye tests

Routine ophthalmic check ups are important to monitor any changes in your vision. They will also aid detection of any potential further complications such as cataracts.

Vitamin A and fish oil supplements

There have been claims that the use of vitamin A supplements and/or fish oil supplements can slow the progression of retinitis pigmentosa. The claims come from a single trial in the USA in 1993 but unfortunately nobody has since been able to replicate those results in true randomized control trials.

While vitamin A and oily fish can have health benefits in the natural qualities of certain foods, there can actually be dangers to some people using supplements containing high doses of either, including people under 18, pregnant women and women considering pregnancy. Sense does not recommend the use of either supplement without discussing the situation with your GP first.

Vitamin E supplements

Although only very limited research has been carried out there is some suggestion that the use of vitamin E supplements may actually speed up the deterioration of the eyesight of people with retinitis pigmentosa. Therefore, Sense strongly recommends that people with Usher syndrome do not take vitamin E supplements.

Smoking

Smoking can cause damage to eye tissue and should be avoided. It may not contribute to retinitis pigmentosa but it doubles the likelihood of developing age-related macular degeneration, again jeopardising the remaining sight of someone with Usher syndrome.

Eye safety in the sun

UVA and UVB rays in sunlight can harm your eyes and increase the risk of cataracts and age-related macular degeneration. Wearing sunglasses or glasses with built in UV filter will protect your eyes. You should only use sunglasses that have a CE mark or carry British Standard BSEN 1836:1997.

 

Support available to people with Usher from Sense

The Sense Usher Service specialises in the field of Usher syndrome and supports people with Usher, their families and professionals. Support covers advice, assessments, training, mentoring and professional support.  Contact info@sense.org.uk or usher@sense.org.uk for more information. You can also follow us on Facebook.

Usher Peer Support Project: ‘Live confidently with Usher’

The Usher Peer Support Project is for people, aged 18 and older, who have Usher syndrome, or for adult family members of those with Usher Syndrome across England, Wales and Northern Ireland. We are looking for people who will use their experience of living with Usher syndrome to give support to other individuals to enable them to make changes in their lives.  

We would also like to hear from anyone who is looking for support from those who know what it’s like to live with Usher syndrome. 

If you are interested in finding out more about the Usher Peer Support Project, please contact Ann Copson in the Sense Usher Service Team.

 

Created: February 2016

Review due: February 2018

First published: Wednesday 2 December 2015
Updated: Monday 16 January 2017