Advertisement

Palliative Care and Intellectual Disability

  • Irene Tuffrey-WijneEmail author
Reference work entry

Abstract

People with intellectual disabilities make up an estimated 1–3% of the population. This is an aging population, with an associated increasing need for palliative care provision. However, many do not have equitable access to palliative care services, for a variety of reasons. They often have issues, challenges, and circumstances that make it particularly difficult to meet their palliative care needs. This includes communication difficulties which affect all the aspects of palliative care provision; difficulties around insight and the ability to participate in decision making; unconventional ways of expressing signs and symptoms of ill health and distress; multiple comorbidities; complex family and social circumstances; and higher levels of behavioral or psychiatric problems.

This chapter describes the unique challenges in meeting the needs of people with intellectual disabilities at the end of life. It is based on the White Paper on Intellectual Disabilities, published by the European Association of Palliative Care in 2015. It addresses the following key areas: equity of access; communication; recognizing the need for palliative care; assessment of total needs; symptom management; end of life decision-making; involving those who matter; collaboration; support for families and carers; preparing for death; bereavement support; education and training; developing and managing services. This provides a comprehensive overview of the current state of the art.

Keywords

Intellectual disabilities End of life Palliative care Advance care planning Bereavement 

References

  1. American Association on Intellectual and Developmental Disabilities. Definition of intellectual disability. 2013a. Available at http://aaidd.org/intellectual-disability/definition#.WD2pNk0SGUk. Accessed 29 Nov 2016.
  2. American Association on Intellectual and Developmental Disabilities. Frequently asked questions on intellectual disability. 2013b. Available at https://aaidd.org/intellectual-disability/definition/faqs-on-intellectual-disability#. Accessed 20 Dec 2016.
  3. Baile W, et al. SPIKES – a six-step protocol for delivering bad news: application to the patient with cancer. Oncologist. 2000;5(4):302–11.PubMedCrossRefGoogle Scholar
  4. Bittles A, Glasson E. Clinical, social, and ethical implications of changing life expectancy in down syndrome. Dev Med Child Neurol. 2004;46:282–6.PubMedCrossRefGoogle Scholar
  5. Blackman N. Loss and learning disability. London: Worth Publishing; 2003.Google Scholar
  6. Blackman N. The development of an assessment tool for the bereavement needs of people with learning disabilities. Br J Learn Disabil. 2008;36:165–70.CrossRefGoogle Scholar
  7. Connecticut State Department of Developmental Services. Mortality annual report 2014. Hartford: CT.GOV State of Connecticut; 2015.Google Scholar
  8. Cross H, et al. Practical approaches toward improving end-of-life care for people with intellectual disabilities: effectiveness and sustainability. J Palliat Med. 2012;15(3):322–6.PubMedCrossRefGoogle Scholar
  9. Department of Health. Valuing people: a new strategy for learning disability for the 21st century. A white paper, London: Department of Health; 2001.Google Scholar
  10. Department of Health. Valuing people now: a new three-year strategy for people with learning disabilities. 2009. Available at https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/250877/5086.pdf. Accessed 29 Nov 2016.
  11. Disability Discrimination Act. Chapter 13, Disability discrimination act 2005. London: The Stationery Office. 2005. Available at http://www.legislation.gov.uk/ukpga/2005/13/introduction. Accessed 29 Nov 2016.
  12. Doka K. Disenfranchised grief: new directions, challenges and strategies for practice. Champaign: Research Press; 2002.Google Scholar
  13. Dowling S, et al. Bereaved adults with intellectual disabilities: a combined randomized controlled trial and qualitative study of two community-based interventions. J Intellect Disabil Res. 2006;50(Pt 4):277–87.PubMedCrossRefGoogle Scholar
  14. Emerson E. Deprivation, ethnicity and the prevalence of intellectual and developmental disabilities. J Epidemiol Community Health. 2012;66:218–24.PubMedCrossRefGoogle Scholar
  15. Emerson E, Baines S. The estimated prevalence of autism among adults with learning disabilities in England. Cambridge: Public Health England; 2010.Google Scholar
  16. Emerson E, Hatton C. Health inequalities and people with intellectual disabilities. Cambridge: Cambridge University Press; 2013.CrossRefGoogle Scholar
  17. European Association for Palliative Care. EAPC definition of palliative care. 1998. Available at http://www.eapcnet.eu/Corporate/AbouttheEAPC/Definitionandaims.aspx. Accessed 29 Nov 2016.
  18. Evenhuis H, et al. Healthy ageing – adults with intellectual disabilities: physical health issues. J Appl Res Intellect Disabil. 2001;14(3):175–94.CrossRefGoogle Scholar
  19. Friedman SL, Helm DT, Woodman AC. Unique and universal barriers: hospice care for aging adults with intellectual disability. Am J Intellect Dev Disabil. 2012;117(6):509–32.PubMedCrossRefGoogle Scholar
  20. Greenswag L. Adults with Prader-Willi syndrome: a survey of 232 cases. Dev Med Child Neurol. 1987;29:145–52.PubMedCrossRefGoogle Scholar
  21. Haveman M, et al. Report on the state of science on health risks and ageing in people with intellectual disabilities. Dortmund: University of Dortmund: IASSID Special Interest Research Group on Ageing and Intellectual Disabilities/Faculty Rehabilitation Sciences; 2009.Google Scholar
  22. Heslop P, et al. Confidential inquiry into premature deaths of people with learning disabilities (CIPOLD). Bristol; 2013. Available at http://www.bris.ac.uk/cipold/reports/index.html. Accessed 29 Nov 2016.
  23. Hogg J, Tuffrey-Wijne I. Cancer and intellectual disability: a review of some key contextual issues. J Appl Res Intellect Disabil. 2008;21(6):509–18.CrossRefGoogle Scholar
  24. Hollins S, Esterhuyzen A. Bereavement and grief in adults with learning disabilities. Br J Psychiatry. 1997;170:497–501.PubMedCrossRefGoogle Scholar
  25. Hubert J, Hollins S. Men with severe learning disabilities and challenging behaviour in long-stay hospital care. Br J Psychiatry. 2006;188:70–4.PubMedCrossRefGoogle Scholar
  26. Iacono T, Johnson H. Patients with disabilities and complex communication needs. The GP consultation. Aust Fam Physician. 2004;33(8):585–9.PubMedGoogle Scholar
  27. Johnson B. Practical guide to health care decision making. In: Friedman S, Helm D, editors. End-of-life care for children and adults with intellectual and developmental disabilities. Washington, DC: American Association on Intellectual and Developmental Disabilities; 2010. p. 133–46.Google Scholar
  28. Kaye P. Breaking bad news: a 10 step approach. Northampton: EPL Publications; 1996.Google Scholar
  29. Learning Disabilities Observatory. People with learning disabilities in England 2015: Main report. London: Public Health England; 2016. Available at https://www.improvinghealthandlives.org.uk/securefiles/161129_1604//PWLDIE%202015%20final.pdf. Accessed 29 Nov 2016.
  30. Mash E, Wolfe D. Abnormal child psychology. Belmont: Thomson Wadsworth; 2004.Google Scholar
  31. McEnhill LS. Breaking bad news of cancer to people with learning disabilities. Br J Learn Disabil. 2008;36(3):157–64.CrossRefGoogle Scholar
  32. McHale R, Carey S. An investigation of the effects of bereavement on mental health and challenging behaviour in adults with learning disability. Br J Learn Disabil. 2002;30:113–7.CrossRefGoogle Scholar
  33. McLaughlin D, et al. Developing a best practice model for partnership practice between specialist palliative care and intellectual disability services: a mixed methods study. Palliat Med. 2014;28(10):1213–31.PubMedCrossRefGoogle Scholar
  34. McLaughlin D, et al. Service user perspectives on palliative care education for health and social care professionals supporting people with learning disabilities. BMJ Support Palliat Care. 2015;5:531–7.PubMedCrossRefGoogle Scholar
  35. Mencap. Death by indifference. London Mencap; 2007. Available at https://www.mencap.org.uk/sites/default/files/2016-06/DBIreport.pdf. Accessed 29 Nov 2016.
  36. Michael J. Healthcare for all: report of the independent inquiry into access to healthcare for people with learning disabilities. London; 2008. Available at http://webarchive.nationalarchives.gov.uk/20130107105354/http:/www.dh.gov.uk/prod_consum_dh/groups/dh_digitalassets/@dh/@en/documents/digitalasset/dh_106126.pdf. Accessed 29 Nov 2016.
  37. Moss A, et al. Prognostic significance of the “surprise” question in cancer patients. J Palliat Med. 2010;13(7):837–40.PubMedCrossRefGoogle Scholar
  38. Murray Parkes C. Bereavement in adult life. Br Med J. 1998;316(7134):856–9.CrossRefGoogle Scholar
  39. Oswin M. Am I allowed to cry? A study of bereavement amongst people who have learning difficulties. London: WBC Print; 1991.Google Scholar
  40. Patja K, et al. Life expectancy of people with intellectual disability: a 35-year follow-up study. J Intellect Disabil Res. 2000;44(5):591–9.PubMedCrossRefGoogle Scholar
  41. Payne S, Morbey H. Supporting family carers report on the evidence of how to work with and support family carers. Lancaster, England; 2013.Google Scholar
  42. PCPLD Network. Linda McEnhill Award 2012 winner. 2013. Available at http://www.pcpld.org/linda-mcenhill-award/2012-winner/. Accessed 20 Dec 2016.
  43. PCPLD Network. Palliative care for people with learning disabilities network. 2016. Available at http://www.pcpld.org/. Accessed 20 Dec 2016.
  44. Read S. Loss, bereavement and learning disabilities: providing a continuum of support. Learn Disabil Pract. 2005;8(1):31–7.CrossRefGoogle Scholar
  45. Read S. Communication in the dying context. In: Read S, editor. Palliative care for people with learning disabilities. London: Quay Books; 2006. p. 93–106.Google Scholar
  46. Read S. Bereavement counselling for people with learning disabilities: a manual to develop practice. London: Quay Books; 2007.Google Scholar
  47. Regnard C, et al. Understanding distress in people with severe communication difficulties: developing and assessing the disability distress assessment tool (DisDAT). J Intellect Disabil Res. 2007;51(4):277–92.PubMedCrossRefGoogle Scholar
  48. Reiss S, Syzszko J. Diagnostic overshadowing and professional experience with mentally retarded persons. Am J Ment Defic. 1983;87:396–402.PubMedGoogle Scholar
  49. Ryan K, et al. Communication contexts about illness, death and dying for people with intellectual disabilities and life-limiting illness. Palliat Support Care. 2011;9(2):201–8.PubMedCrossRefGoogle Scholar
  50. Satgé D, Vekemans M. Down syndrome patients are less likely to develop some (but not all) malignant solid tumours. Clin Genet. 2011;79(3):289–90.PubMedCrossRefGoogle Scholar
  51. Schalock R, et al. Intellectual disability: definition, classification, and systems of supports. 11th ed. Washington, DC: American Association on Intellectual and Developmental Disabilities; 2010.Google Scholar
  52. Strydom A, et al. Dementia in older adults with intellectual disabilities – epidemiology, presentation, and diagnosis. J Policy Pract Intellect Disabil. 2010;7(2):96–110.CrossRefGoogle Scholar
  53. Swinton J. Spirituality and the lives of people with learning disabilities. London: Foundation for People with Learning Disabilities/Mental Health Foundation; 2001.Google Scholar
  54. Symons F, Shinde S, Gilles E. Perspectives on pain and intellectual disability. J Intellect Disabil Res. 2008;52(4):275–86.PubMedCrossRefGoogle Scholar
  55. Thomas K et al. The gold standards framework prognostic indicator guidance. 4th ed. 2011. Available at http://www.goldstandardsframework.org.uk/cd-content/uploads/files/General Files/Prognostic Indicator Guidance October 2011.pdf. Accessed 29 Nov 2016.
  56. Thurman S, Jones J, Tarleton B. Without words – meaningful information for people with high individual communication needs. Br J Learn Disabil. 2005;33(2):83–9.CrossRefGoogle Scholar
  57. Todd S. Surprised endings: the dying of people with learning disabilities in residential services. Int J Palliat Nurs. 2005;11(2):80–2.PubMedCrossRefGoogle Scholar
  58. Todd S. Silenced grief: living with the death of a child with intellectual disabilities. J Intellect Disabil Res. 2007;51(Pt 8):637–48.PubMedCrossRefGoogle Scholar
  59. Todd S. “Being there”: the experiences of staff in dealing with matters of dying and death in services for people with intellectual disabilities. J Appl Res Intellect Disabil. 2013;26(3):215–30.PubMedCrossRefGoogle Scholar
  60. Tuffrey-Wijne I. The palliative care needs of people with intellectual disabilities: a literature review. Palliat Med. 2003;17(1):55–62.PubMedCrossRefGoogle Scholar
  61. Tuffrey-Wijne I. Living with learning disabilities, dying with cancer: thirteen personal stories. London: Jessica Kingsley Publishers; 2010.Google Scholar
  62. Tuffrey-Wijne I, McEnhill L. Communication difficulties and intellectual disability in end-of-life care. Int J Palliat Nurs. 2008;14(4):192–7.CrossRefGoogle Scholar
  63. Tuffrey-Wijne I, McLaughlin D. Consensus norms for palliative care of people with intellectual disabilities in Europe: EAPC White Paper. London/Milan: European Association for Palliative Care (EAPC); 2015. Available at http://www.eapcnet.eu/LinkClick.aspx?fileticket=Iym7SMB78cw%3d. Accessed 29 Nov 2016.
  64. Tuffrey-Wijne I, Bernal J, et al. Using nominal group technique to investigate the views of people with intellectual disabilities on end-of-life care provision. J Adv Nurs. 2007a;58(1):80–9.PubMedCrossRefGoogle Scholar
  65. Tuffrey-Wijne I, Hogg J, Curfs L. End-of-life and palliative care for people with intellectual disabilities who have cancer or other life-limiting illness: a review of the literature and available resources. J Appl Res Intellect Disabil. 2007b;20(4):331–44.CrossRefGoogle Scholar
  66. Tuffrey-Wijne I, Curfs L, Hollins S. Providing palliative care to people with intellectual disabilities and cancer. Int J Disabil Hum Dev. 2008;7(4):365–70.CrossRefGoogle Scholar
  67. Tuffrey-Wijne I, et al. People with intellectual disabilities who are affected by a relative or friend with cancer: a qualitative study exploring experiences and support needs. Eur J Oncol Nurs. 2012;16(5):512–9.PubMedCrossRefGoogle Scholar
  68. Tuffrey-Wijne I, et al. Developing guidelines for disclosure or non-disclosure of bad news around life-limiting illness and death to people with intellectual disabilities. J Appl Res Intellect Disabil. 2013;26(3):231–42.PubMedCrossRefGoogle Scholar
  69. Tuffrey-Wijne I, et al. Defining consensus norms for palliative care of people with intellectual disabilities in Europe, using Delphi methods: a white paper from the European Association of Palliative Care (EAPC). Palliat Med. 2015;30(5):446–55.PubMedPubMedCentralCrossRefGoogle Scholar
  70. Tuffrey-Wijne I, Wicki M, et al. Developing research priorities for palliative care of people with intellectual disabilities in Europe: a consultation process using nominal group technique. BMC Palliat Care. 2016a;15(36)Google Scholar
  71. Tuffrey-Wijne I, Abraham E, et al. Role confusion as a barrier to effective carer involvement for people with intellectual disabilities in acute hospitals: findings from a mixed-method study. J Adv Nurs. 2016b;72(11):2907–22.PubMedCrossRefGoogle Scholar
  72. Tuffrey-Wijne I, Rose T. Investigating the factors that affect the communication of death-related bad news to people with intellectual disabilities by staff in residential and supported living services: an interview study. J Intellect Disabil Res. 2017;61(1):727–736.PubMedCrossRefGoogle Scholar
  73. United Nations. Convention on the rights of persons with disabilities. 2006. Available at http://www.un.org/disabilities/convention/conventionfull.shtml. Accessed 29 Nov 2016.
  74. Vrijmoeth C, et al. Physicians’ identification of the need for palliative care in people with intellectual disabilities. Res Dev Disabil. 2016;59:55–64.PubMedCrossRefGoogle Scholar
  75. Wagemans A, et al. End-of-life decisions: an important theme in the care for people with intellectual disabilities. J Intellect Disabil Res. 2010;54(6):516–24.PubMedCrossRefGoogle Scholar
  76. Wiese M, et al. “If and when?”: the beliefs and experiences of community living staff in supporting older people with intellectual disability to know about dying. J Intellect Disabil Res. 2013;57(10):980–92.PubMedCrossRefGoogle Scholar
  77. Yang Q, Rasmussen S, Friedmann J. Mortality associated with Down’s syndrome in the USA from 1983 to 1997: a population-based study. Lancet. 2002;359(9311):1019–25.PubMedCrossRefGoogle Scholar
  78. Young H, Garrard B, Lambe L. Bereavement and loss: supporting bereaved people with profound and multiple learning disabilities and their parents. Dundee: PAMIS; 2014. Available at http://www.pamis.org.uk/_page.php?id=70. Accessed 29 Nov 2016.

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Faculty of Health, Social Care and EducationKingston University and St George’s, University of LondonLondonUK

Personalised recommendations