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Patient with Down's syndrome speaks out

Thirty-year-old James is a new patient; you are surprised he has come to consult you unaccompanied as he clearly has Down's syndrome. When you ask if there is anyone he would like to bring in with him, he tells you he is quite capable of speaking for himself. Dr Melanie Wynne-Jones advises.

What is self-advocacy?

Self-advocacy is a fairly new term which emphasises that people with intellectual disabilities have a right to a 'voice' and identity of their own, and to have their wishes respected1.

This may be something as simple as what they choose to eat, or as complex as deciding where to live, and with whom, deciding whether to take risks, consenting to medical treatment, forming relationships (including sexual relationships), cultural issues and the right to be consulted about services and public policies that affect them.

Self-advocacy can only operate within the limits of practicality and often requires help and support from others. Some people with Down's syndrome are able to work, marry or live semi-independently, but are still likely to require legal, financial and general advice.

GPs can help to solve medical problems and challenge attitudes and obstacles where appropriate ­ it's important to look beyond the Down's syndrome and see the person.

How should we communicate with someone who has a learning disability?

The 'rules' are the same as in any other consultation:

 · Correct setting ­ this may include a longer appointment time and the presence or removal of others who may facilitate (or obstruct) both the practicalities and the subtext of communicating with the patient

 · Correct rapport ­ focus on the patient, involving a third party only where appropriate

 · Correct tools ­ words aimed at correct educational level, deliberate use of body language, use of sign language or pictures

 · Correct consulting techniques ­ establish patient's agenda (and the carer's, which may be different), signpost, check understanding and summarise frequently, ask open questions, explore ideas, concerns and expectations, discuss options, help patient choose management plan.

What are the health needs of adults

with Down's syndrome2?

Better medical care and social changes have tripled average life expectancy for people with Down's syndrome in the last few decades ­ someone who reaches the age of five (by which age most cardiac deaths have occurred) has an 80 per cent chance of living into their 20s and a 60 per cent chance of reaching their 50s.

About 40 per cent of children with Down's have cardiac abnormalities, many of which can be successfully treated. Without surgery these will continue to cause problems throughout life. Some 12 per cent will have suffered gastrointestinal problems including duodenal atresia or Hirschsprung's disease.

Up to 15 per cent will have radiological evidence of instability of the atlantoaxial joint; this rarely causes neurological abnormalities due to impingement on the spinal cord but some may need to avoid certain activities.

Cataracts (congenital or acquired), refractive errors, squint, glaucoma, hearing impairment, lax joints including dislocation of the hip, constipation and dental decay are also common.

The skin is dryer and more at risk from infections. Menstruation and sexual functioning are usually normal, and pregnancy is rare but possible. Hypothyroidism is more common (up to 30 per cent lifetime risk) ­ a low index of suspicion and perhaps five-yearly screening are appropriate. Osteoporosis is also more common.

Behavioural problems may occur at any age, and will reflect life events and the behaviour and attitudes of other people, as well as the person's intellectual capacity and communication skills. Depression or psychosis may mask physical illness or present as behavioural problems. Mental illness is harder to assess and may reflect past or ongoing abuse. Referral to a psychiatrist experienced in caring for patients with learning disabilities is helpful.

Alzheimer's disease appears to be more common in people with Down's and may be associated with late-onset epilepsy. Reversible cause of dementia should be sought.

The family of someone with learning disabilities will have separate, changing or conflicting needs throughout the person's life.

What lifestyle advice should be given?

People with Down's are more likely to be obese and advice on diet, exercise, dental care, osteoporosis prevention, smoking and drinking should be given, as it would to anyone else. They should be encouraged to accept appropriate immunisations and to participate in screening for cervical cancer, hypertension and so on. Regular hearing, vision and dental checks are advisable.

Can adults with learning disabilities give medical consent?

People with learning disabilities are increasingly outliving their parents, who may have protected and campaigned for them throughout their lives.

There is evidence that health care professionals frequently expect a relative, carer or support worker to make decisions on behalf of people with learning disabilities, and do not even check the individual's capacity to consent3. This can mean the third party's knowledge, preferences and even prejudices determine what actually happens to the patient.

Examples include suppression of menstruation, contraception and screening procedures.

Assuming that people with learning disabilities would not be interested or could not understand is discriminatory, although they may reject interventions that are presented too simplistically ('this will hurt'). Wherever possible, they should be counselled in appropriate language and without undue influence.

This may require extra time or the involvement of a trusted relative, carer, social worker or other health professional. The Department of Health4,5,6 and the Down's Syndrome Association in collaboration with St George's Hospital Medical Schoo · 7 offer advice to patients and health professionals.

Where the patient does not have capacity to consent (or refuse) and may be in danger, the Court of Protection, or in an emergency the Mental Health Act or even a court injunction, can be used to safeguard their interests.

References

1 Club NDSS, a US website for people with Down's syndrome and other developmental disabilities includes advice on

self-advocacy.

www.clubndss.org

2 Down's Syndrome Association (aimed at adults). www.downs-syndrome.org.uk

3 Keywood Ket al. Best practice? Health care decision making by, with and for adults with learning disabilities. Manchester, National Development Team and Institute of Medicine, Law and Bioethics (1999)

4 DoH. Reference guide to consent for examination or treatment. London, 2001. www.doh.gov.uk/consent/refguide.pdf

5 DoH. Seeking consent: working with people with learning disabilities. London, 2001. www.doh.gov.uk/consent/guidance.htm

6 DoH. Advice on consent for people with learning disabilities. www.doh.gov.uk/consent/guidelearningdisabilities.htm

7 Down's Syndrome Association in collaboration with St George's Hospital Medical School, Department of Mental Health. Learning disability.

www.intellectualdisability.info

Key points

 · Adults with learning disabilities are entitled to 'have their own voice', self-advocate and have their capacity to (withhold) consent respected

 · Relatives and professional carers must not be automatically presumed to know what someone with learning disabilities wants/needs

 · There is a wide range of physical and learning disability in Down's syndrome; many adults outlive their parents

 · People with learning disabilities should not be discriminated against in health care or health promotion, although there may be practical difficulties in providing equivalent care

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