|
CANCER, PALLIATIVE CARE AND INTELLECTUAL DISABILITIES
Irene Tuffrey-Wijne RGN, RNMH, BSc Nursing (Palliative Care)
Introduction
The life expectancy of people with intellectual disabilities
has increased dramatically during the last century. Correspondingly,
conditions such as cardiovascular disease and cancer are becoming
more common (Jancar, 1990; Patja et al, 2000). Studies have shown
that around one in ten people with intellectual disabilities now
die of cancer (Cooke, 1997; Hollins et al, 1998). This means that,
like the rest of the population, many people with intellectual
disabilities will require palliative care at the end of their
lives.
Palliative care is defined as 'the active total care of patients
whose disease is not responsive to curative treatment; the goal
of palliative care is achievement of the best possible quality
of life for patients and their families' (World Health Organisation,
1990).
As most people with intellectual disabilities now live in the
community, they rely on primary and secondary health care services
to meet their health needs. Recent health care strategies recognise
that people with intellectual disabilities have a right to a uniformly
high standard of health care. They may need focused support to
enable them to access mainstream services (Department of Health,
1995, 1998, 2001; Lindsey and Russell 1999).
This paper looks at the issues surrounding cancer and palliative
care needs of people with intellectual disabilities. Problems
include the risk of delayed diagnosis; issues around assessment
and control of symptoms; issues around consent; communicating
about the illness and impending death; family dynamics; and the
importance of good support services.
Delayed diagnosis
There is a growing body of evidence to suggest that people with
intellectual disabilities have a high level of previously undiagnosed
and unmanaged health problems, some of them serious (Barr et al,
2001; Beange et al, 1995; Howells, 1986; Webb and Rogers, 1999;
Wilson and Haire 1990). A serious illness may not be picked up
in its early stages when the chances of a positive outcome may
be greater.
There are various possible reasons for this failure to receive
adequate medical help. These include:
- Lack of health screening: Many people with intellectual disabilities
do not access screening programmes that are generally available
to the wider population. (Mencap and the NHS, 1998; Pearson,
1998; Stein and Allen, 1999)
- Communication problems: Many people with intellectual disabilities
are unable to give an accurate medical history or describe their
symptoms. (Beange et al, 1995; Dodd, 1999; Howells, 1997
- Diagnostic overshadowing: Behavioural manifestations of discomfort
may be attributed to the patient's challenging behaviour or
their intellectual disability, rather than to an undiagnosed
physical illness. Carers may trivialise complaints. (Beange
et al, 1995; Dodd, 1999; Howells, 1997; Hendren et al, 1990).
- Carers may lack the skills to support a person with an intellectual
disability through the health system. They may not recognise
the problem, or they may lack confidence in communicating with
health professionals. (Howells, 1997; Tuffrey-Wijne 1997)
- Negative attitudes of health professionals, possibly caused
by lack of confidence, lack of experience and assumptions. (Howells;
Slevin and Sines, 1996; Cumella and Martin, 2000)
Assessment and Control of Symptoms
Pain is often the first indicator of injury and illness, but
in a person with intellectual disabilities this warning sign could
be easily missed. If a person is unable to communicate with words,
pain and other symptoms (such as nausea, dysphagia, fatigue) may
be communicated in different ways. These include:
- Vocal responses (crying, moaning)
- Adaptive behaviour (eg rubbing of the affected area, avoiding
certain movements, keeping area still)
- Self-distracting behaviour (eg rocking, pacing, biting hand,
gesturing)
- Facial expressions (eg grimacing)
- Withdrawal, low mood
- Sleep disturbance
- Self-injurious behaviour
- Hyperactive behaviour
- Autonomic changes (increased/decreased pulse, blood pressure,
sweating) (Astor, 2001; Biersdorff, 1994; Bosch et al, 1997;
McGrath et al, 1998; Regnard et al, 2002.)
Special skills of observation, together with a close knowledge
of what is normal behaviour for an individual with intellectual
disabilities, are needed to pick up signs and symptoms related
to the illness. This can only be achieved by a close cooperation
between health professionals and the person's carers.
Consent
The issue of consent to tests and treatments can cause anxiety
and confusion among clinicians and carers alike. Clinicians may
be reluctant to consider and provide the same range of treatment
options for people with intellectual disabilities as for the rest
of the population, because of perceived difficulty obtaining informed
consent, or for fear of litigation (Howells, 1997; Cumella and
Martin 2000). There is also evidence to suggest that clinicians
do not seek the consent of people with intellectual disabilities
when providing tests and treatments, and base decision-making
on an assumption of incompetence (Northfield and Turnbull 2001;
Keywood et al, 1999). This is a complicated issue. People with
intellectual disabilities may have difficulties with understanding
risks and possible treatment outcomes, which require abstract
thinking. This can lead to denied opportunities to give consent
(Curran and Hollins, 1994).
It is important to understand the law, which is different in
different countries. In England no one, not even parents or medical
staff, can consent on behalf of an adult who is not competent
to give consent. The guiding principle should be that doctors
must act in the patients' best interest if they cannot choose
for themselves. It may be negligent to withhold treatment because
the patient cannot give consent (Department of Health, 1998; Tännsjö,
1999). MENCAP (a UK organisation campaigning for the rights of
people with intellectual disabilities) proposes that decisions
around treatment should be based on the patient's medical situation,
not on health professionals' interpretation of the patient's quality
of life (MENCAP, 2001).
Communicating about illness and death
Carers and health professionals are often unsure whether they
should talk to a person with an intellectual disability about
his or her illness. There is a danger of creating a 'conspiracy
of silence', where professionals, family and friends all know
about the illness and impending death but will not talk about
it in the presence of the patient. Arguments such as 'he won't
understand' or 'the truth is too upsetting' are often used (Tuffrey-Wijne,
1997). Such behaviour is unlikely to result in a death with comfort
and dignity, and will probably add to the patient's confusion
and distress.
There is a need to assess the patient's understanding of the
illness, as this could affect the way he or she copes with it.
One study found that people with intellectual disabilities tended
to relate to illness in terms of observable symptoms and behaviour,
rather than explanations of its underlying cause; knowledge of
the course of illness was sufficient explanation of its cause
(March, 1991).
Talking openly about the illness can help the patient make sense
of his or her situation. Questions must be answered directly,
honestly and simply. Health professionals should try to use the
patient's own vocabulary. It is also possible to use aids such
as simple demonstrations, or pictures. A series of picture books
has been published for this purpose by St George's, University of London in London (Donaghey et al, 2002; Hollins et al, 1996, 1997,
2000). It is important to establish what the patient's history
has been. Has anybody in the patient's social circle had a similar
illness? Has there been any previous negative experience of hospitalisation?
It may also be important to explain that this illness is not necessarily
the same as others. Likewise, the patient may have formed certain
images of what death will be like. Has anyone in his or her social
circle died suddenly or in pain? Such images are not always expressed
and they are not always realistic. Gentle probing could bring
out the patient's worries.
Family Dynamics
Health professionals need to understand the nature of the relationships
that the individual with intellectual disabilities has with his
or her family, carers and close friends. Many people who have
a life-threatening illness (whether they have intellectual disabilities
or not) and their families have a strong need to have some deep
and meaningful communication together. This can be one of the
most difficult needs to fulfill. People often do not know how
to begin to communicate. Family patterns of behaviour can emerge
more strongly during a terminal illness. Thus, a family's tendency
to protect or be jolly may become a barrier to open communication.
There may also be profound feelings of guilt, rejection, or over-identification.
People with intellectual disabilities often have a very firm and
important place in their social environment, and the impending
death will mean a profound loss and a complete change in the family
dynamics. In addition, if the person has left the family home
to live in another care setting, carers may also have difficulty
coming to terms with the impending loss (Botsford, 2000; Tuffrey-Wijne,
1998). Health professionals need to be aware of these issues;
they may be needed to provide sensitive support and aid honest
communication.
Support services
Case histories reported in the literature all conclude that effective,
pro-active facilities and support services are needed to help
terminally ill people with intellectual disabilities and their
carers manage the situation (Hendren et al, 1990; Tuffrey-Wijne,
1997, 1998). Health professionals may have limited awareness of
the needs of people with intellectual disabilities (Lindop et
al, 2000; Thornton, 1996). It is important to establish in each
situation what support or training is needed for the health professionals
involved, and who could best provide such support. Ongoing training
could have its place, but ad hoc practice support measures from
the palliative care team or the intellectual disabilities team
may be more useful in many instances (Minihan et al, 1993).
It is likely that many different individuals and agencies will
be involved in the care of a person with intellectual disabilities
who has a terminal illness. A UK government report which investigated
the experiences of cancer services by people with intellectual
disabilities, their families and staff has found that satisfaction
with services was greatest when there was good collaboration between
the different organisations involved in providing services (Northfield
and Turnbull, 2001). It is also important to establish who co-ordinates
the care around the patient. This should be someone who keeps
the patient's best interest at heart at all times.
Conclusion
Health professionals are increasingly likely to work with a terminally
ill patient who has intellectual disabilities. Whilst the principles
of palliative care remain the same regardless of who the patient
is, special issues may arise when the patient has intellectual
disabilities. Focused attention is needed to deal with those issues.
Health professionals need to take time to understand the way in
which an individual communicates, to ensure that signs and symptoms
are identified correctly and to address underlying worries in
a sensitive manner. They also need to be aware of consent issues,
and ensure that the patient is kept central in any decision making.
This can only be achieved if everyone involved in the patient's
care, including the family, carers and advocates, shares information
and co-operates closely. Finally, as is imperative in providing
good palliative care, the patient should be the most important
member of the team.
References
Astor R. Detecting pain in people with profound learning disabilities.
Nursing Times 2001; 97(40):38-39.
Barr O, Gilgunn J, Kane T, Moore G. Health screening for people
with learning disabilities by a community learning disability
nursing service in Northern Ireland. Jounal of Advanced Nursing
2001; 29(6):1482-1491.
Beange H, McElduff A, Baker W. Medical disorders of adults with
mental retardation: a population study. American Journal of Mental
Retardation 1995; 99(6):595-604.
Biersdorff K. Incidence of significantly altered pain experience
among individuals with developmental disabilities. American Journal
of Mental Retardation 1994; 98(5):619-631.
Bosch J, Van Dyke DC, Milligan Smith S, Poulton S. Role of medical
conditions in the exacerbation of self-injurious behavior: an
exploratory study. Mental Retardation 1997; 35(2):124-130.
Botsford AL. Integrating end of life care into services for people
with an intellectual disability. Social Work in Health Care 2000;
31(1):35-48.
Cooke LB. Cancer and learning disability. Journal of Intellectual
Disability Research 1997; 41(4):312-316.
Cumella S, Martin D. Secondary healthcare for people with a learning
disability. 2000. London, Department of Health.
Curran J, Hollins S. Consent to treatment and people with learning
disability. Psychiatric Bulletin (Royal College of Psychiatrists)
1994; 18(11):691-693.
Department of Health. The health of the nation. A strategy for
people with learning disability. London: HMSO, 1995.
Department of Health. Signposts for success in commissioning and
providing health services for people with learning disabilities.
Wetherby: 1998.
Department of Health. Valuing people: A new strategy for learning
disability for the 21st century. A White Paper. London: Department
of Health, 2001.
Dodd K. 'Feeling poorly': Report of a pilot study aimed to increase
the ability of people with learning disabilities to understand
and communicate about physical illness. British Journal of Learning
Disabilities 1999; 27:10-15.
Donaghey V, Bernal J, Tuffrey-Wijne I, Hollins S. It's not all
bad news. London: Gaskell/St George's, University of London,
2002.
Hendren R, Stokes Taylor W, Lemke B, Lemke P. Case study: an autistic
boy copes with a terminal illness. Journal of the American Academy
of Child and Adolescent Psychiatry 1990; 29(6):901-904.
Hollins S, Attard MT, von Fraunhofer N, McGuigan S, Sedgwick P.
Mortality in people with learning disability: Risks, causes, and
death certification findings in London. Developmental Medicine
& Child Neurology 1998; 40(1):50-56.
Hollins S, Bernal J, Gregory M. Going to the doctor. London: Gaskell/St
George's, University of London, 1996.
Hollins S, Downer J. Keeping healthy 'down below'. London: Gaskell/St
George's, University of London, 2000.
Hollins S, Perez W. Looking after my breasts. London: Gaskell/St
George's, University of London, 2000.Howells G. Are the medical
needs of mentally handicapped adults being met? Journal of the
Royal College of General Practitioners 1986; 36:449-453.
Howells G. A general practice perspective. In: O'Hara J, Sperlinger
A, editors. Adults with learning disabilities: A practical approach
for health professionals. Chichester: John Wiley and Sons, 1997:
61-79.
Jancar J. Cancer and mental handicap: a further study. British
Journal of Psychiatry 1990; 156:531-533.
Lindop P, Read S. District nurses' needs: palliative care for
people with learning disabilities. International Journal of Palliative
Nursing 2000; 6(3):117-122.
Lindsey M, Russell O. Once a day: One or more people with learning
disabilities are likely to be in contact with your primary health
team. Can you help them? Wetherby: 1999.
March P. How do people with a mild/moderate mental handicap conceptualise
physical illness and its cause? The British Journal of Mental
Subnormality 1991; 37, part 2(73):80-90.
McGrath P, Rosmus C, Canfield C, Campbell M, Hennigar A. Behaviours
caregivers use to determine pain in non-verbal, cognitively impaired
individuals. Developmental Medicine & Child Neurology 1998;
40:340-343.
MENCAP. The NHS: Health for all? People with learning disabilities
and health care. London: Mencap National Centre, 1998.
MENCAP. Considerations of 'quality of life' in cases of medical
decision making for individuals with severe learning disabilities.
2001. London, Mencap. 2001.
Minihan P, Dean D, Lyons C. Managing the care of patients with
mental retardation: a survey of physicians. Mental Retardation
1993; 31(4):239-246.
Patja K, Iivanainen M, Vesala H, Oksanen H, Ruoppila I. Life expectancy
of people with intellectual disability: a 35-year follow-up study.
Journal of Intellectual Disability Research 2000; 44(5):591-599.
Pearson V. Only one quarter of women with learning disabilities
in Exeter have cervical screening. British Medical Journal 1998;
316:1979.
Regnard C, Gibson L, Jenson C. Understanding and helping the person
with alternative communications. 2: Identifying distress. In:
Regnard C, editor. CLIP Worksheets. Oxford: Radcliffe Medical
Press, 2002.
Slevin E, Sines D. Attitudes of nurses in a general hospital towards
people with learning disabilities: influences of contact, and
graduate-non-graduate status, a comparative study. Jounal of Advanced
Nursing 1996; 24:1116-1126.
Stein K, Allen N. Cross sectional survey of cervical cancer screening
in women with learning disability. British Medical Journal 1999;
318:614.
Tännsjö T. Informal coercion in the physical care of
patients suffering from senile dementia or mental retardation.
Nursing Ethics 1999; 6(4):327-336.
Thornton C. A focus group inquiry into the perceptions of primary
healthcare teams and the provision of healthcare for adults with
a learning disability living in the community. Jounal of Advanced
Nursing 1996; 23:1168-1176.
Tuffrey-Wijne I. Palliative care and learning disabilities. Nursing
Times 1997; 93(31):50-51.
Tuffrey-Wijne I. Care of the terminally ill. Learning Disability
Practice 1998; 1(1):8-11
Webb OJ, Rogers L. Health screening for people with intellectual
disability: the New Zealand experience. Journal of Intellectual
Disability Research 1999; 43(6):497-503.
Wilson D, Haire A. Health care screening for people with mental
handicap living in the community. British Medical Journal 1990;
301(1379):1381.
World Health Organisation. Cancer pain relief and palliative care.
Geneva: WHO, 1990.
This article was first published on the site in 2002.
Back to top
|
