Hidden lives and deaths: the last months of life of people with intellectual disabilities living in long-term, generic care settings in the UK.

RATIONALE: This paper concerns mortality and needs for end-of-life care in a population of adults with ID living in generic care homes. METHODS: Various sampling strategies were used to identify a difficult to find a population of people with ID in generic care homes. Demographic and health data were obtained for 132 people with ID. This included the Surprise Question. At T2, 12 months later, data were obtained on the survival of this sample. FINDINGS: The average age was 68.6 years, and the majority were women (55.3%). Their health was typically rated as good or better. Responses to the Surprise Question indicated that 23.3% respondents might need EoLC. At T2, 18.0% of this population had died. The average of death was 72.2 years. The majority died within the care setting (62.9%). IMPLICATIONS: The implications for end-of-life care and mortality research are discussed.

Maximising engagement and participation of intellectual disability staff in research: Insights from conducting a UK-wide survey.

AIM: This article explores ways of maximising engagement of intellectual disability staff as research participants, research advisers and research implementers. METHOD: The authors describe and reflect on a three-phased strategy in recruiting front-line staff (n = 690) working for intellectual disability service providers (n = 25) to participate in a UK-wide anonymous online survey about death, dying and bereavement. RESULTS: Important elements in engaging participants were: involving stakeholders at all stages of the research process, which includes: building relationships with participating organisations; enlisting organisational management support at all levels; an attractive and well laid-out collection tool; a well-structured recruitment strategy; time and flexibility; and a varied and targeted dissemination strategy. However, the recruitment method had limitations, in particular around representativeness, bias and generalisability. CONCLUSIONS: Staff in intellectual disability services can be enthusiastic and invaluable research participants. Active engagement between researchers, participating organisations and stakeholder groups is key to ensuring involvement of intellectual disability staff with research.

Place of death of people with intellectual disabilities: An exploratory study of death and dying within community disability service settings.

This article describes an exploratory study of deaths of people with intellectual disabilities (IDs) that had occurred in group homes managed by an ID service provider in Australasia. Such settings are increasingly recognised as places for both living and dying. Little is known about the extent to which they encounter the death of a person with ID and with what outcomes. Data were obtained from service records and telephone interviews on 66 deaths occurring within a 2-year period. The findings suggest that death is an important but relatively rare event within ID services. This rate of death was influenced by the age structure of the population. Most of the deaths occurred within a hospital setting. Cause of death did not have much impact upon place of death. However, setting characteristics seemed to have some influence. As an exploratory study, lessons for future population-based research in this area are addressed.

Expecting the unexpected: measures, outcomes and dying trajectories for expected and unexpected death in adults with intellectual disabilities in social care settings in the UK.

BACKGROUND: Considerable attention is rightly paid to preventable deaths in adults with intellectual disabilities. The pattern of these deaths and the implications for services have been less considered. METHOD: This paper forms part of a larger cross-sectional study of deaths in a defined population of adults with intellectual disabilities. Data from VOICES-SF were obtained from intellectual disability service providers for each death within their supported population over 18 months, 149 decedents in all. RESULTS: Only 34.2% of deaths were anticipated, death was often sudden or followed a short illness. Four dying trajectories were identified, and categorized by length of illness, service use, care outcomes, symptom control and cause of death. CONCLUSIONS: Recognition that most deaths in this group were unanticipated implies a need for better preparation for less expected dying and better anticipation. A descriptive epidemiology of dying among people with intellectual disabilities is needed.

Last months of life of people with intellectual disabilities: A UK population-based study of death and dying in intellectual disability community services.

BACKGROUND: Population-based data are presented on the nature of dying in intellectual disability services. METHODS: A retrospective survey was conducted over 18 months with a sample of UK-based intellectual disability service providers that supported over 12,000. Core data were obtained for 222 deaths within this population. For 158 (71%) deaths, respondents returned a supplemented and modified version of VOICES-SF. RESULTS: The observed death was 12.2 deaths per 1,000 people supported per year, but just over a third deaths had been deaths anticipated by care staff. Mortality patterns, place of usual care and availability of external support exerted considerable influence over outcomes at the end of life. CONCLUSION: Death is not a common event in intellectual disability services. A major disadvantage experienced by people with intellectual disabilities was that their deaths were relatively unanticipated. People with intellectual disabilities living in supported living settings, even when their dying was anticipated, experienced poorer outcomes.

Communicating about death and dying with adults with intellectual disabilities who are terminally ill or bereaved: A UK-wide survey of intellectual disability support staff.

BACKGROUND: Illness and death are part of life for everyone, including people with intellectual disabilities. This study investigated the extent to which staff communicate about death with people with intellectual disability facing terminal illness or bereavement. METHOD: Staff who support people with intellectual disability in the UK (n = 690) completed an electronic survey. Detailed data were obtained from staff where a client had died in the past 12 months (n = 111), was terminally ill (n = 41) or had been bereaved (n = 200). Analysis included descriptive and chi-squared statistics. RESULTS: 52.6% of people with intellectual disability who were terminally ill were told about their illness, and 18.1% were told they would die. Of those experiencing an anticipated bereavement, 32.4% of staff said no one talked about this with them beforehand. A quarter of staff had received training on end of life or bereavement. CONCLUSION: Death affects many people with intellectual disability. Staff require training and support in communicating death.

End-of-life care in intellectual disability: a retrospective cross-sectional study.

BACKGROUND: Adults with intellectual disability (ID) experience inequality in access to healthcare that is considered to extend to end-of-life care. Their experiences of healthcare at the end of life and how these compare with the general population are unknown. AIM: To describe the end-of-life care outcomes for adults with ID living in residential care in the UK using the VOICES-SF questionnaire and compare these with the general population. DESIGN: Nationwide population-based postbereavement survey. PARTICIPANTS: 38 ID care providers took part in the study. The supported over 13 000 people with ID. Over the 18-month period of data collection, 222 deaths were reported. The survey was completed, by care staff, for 157 (70.7%) of those deaths. RESULTS: Decedents had complex health, functional and behavioural needs. Death was unanticipated in a high proportion of cases. Quality of care provided across care settings was generally well rated. However, hospital care and care provided at the time of was less well rated, particularly in comparison with the general population. Respondents reported low levels of involvement in care and awareness of approaching death among adults with ID, and lower than in the general population. CONCLUSIONS: Access to end-of-life care for adults with ID may be constrained by a failure to identify approaching the end of life. The high proportion of unexpected deaths in this population warrants further study. There is a need to increase and support the involvement of adults with ID to be active partners in planning care at the end of their lives.

Nursing care at end of life: a UK-based survey of the deaths of people living in care settings for people with intellectual disability.

BACKGROUND: People with intellectual disability are believed to be at risk of receiving poor end-of-life care. Nurses, given their advocacy role and duty to provide compassionate end-of-life care, have the potential to change this situation but research regarding this aspect of their role is limited. AIMS: This paper thus seeks to answer the question 'How and when are nurses involved in providing care at end of life for people with intellectual disability?' METHODS: A total of 38 intellectual disability care providers in the UK providing support to 13,568 people with intellectual disability were surveyed. Data regarding 247 deaths within this population were gathered in two stages and subsequently entered into SPSSX for analysis. RESULTS: Findings revealed that the majority of deaths occurred between the ages of 50 and 69 years, the most commonly reported cause of death being respiratory problems. Both community and hospital-based nurses were involved in supporting individuals during their final 3 months of life, and sometimes more than one type of nurse provided support to individuals. Generally nursing care was rated positively, although room for improvement was also identified. CONCLUSIONS: Nurses are involved in supporting people with intellectual disability at end of life and appropriate education is required to undertake this role. This may require change in curricula and subsequent research to determine the impact of such change on nursing support to this population.

Impact of cholinesterase inhibitors or memantine on survival in adults with Down syndrome and dementia: clinical cohort study.

BACKGROUND: There is little evidence to guide pharmacological treatment in adults with Down syndrome and Alzheimer's disease. Aims To investigate the effect of cholinesterase inhibitors or memantine on survival and function in adults with Down syndrome and Alzheimer's disease. METHOD: This was a naturalistic longitudinal follow-up of a clinical cohort of 310 people with Down syndrome diagnosed with Alzheimer's disease collected from specialist community services in England. RESULTS: Median survival time (5.59 years, 95% CI 4.67-6.67) for those on medication (n = 145, mainly cholinesterase inhibitors) was significantly greater than for those not prescribed medication (n = 165) (3.45 years, 95% CI 2.91-4.13, log-rank test P<0.001). Sequential assessments demonstrated an early effect in maintaining cognitive function. CONCLUSIONS: Cholinesterase inhibitors appear to offer benefit for people with Down syndrome and Alzheimer's disease that is comparable with sporadic Alzheimer's disease; a trial to test the effect of earlier treatment (prodromal Alzheimer's disease) in Down syndrome may be indicated. Declaration of interest A.S. has undertaken consulting for Ono Pharmaceuticals, outside the submitted work. Z.W. has received a consultancy fee and grant from GE Healthcare, outside the submitted work.

Predictors of Age of Diagnosis and Survival of Alzheimer's Disease in Down Syndrome.

BACKGROUND: People with Down syndrome (DS) are an ultra-high risk population for Alzheimer's disease (AD). Understanding the factors associated with age of onset and survival in this population could highlight factors associated with modulation of the amyloid cascade. OBJECTIVE: This study aimed to establish the typical age at diagnosis and survival associated with AD in DS and the risk factors associated with these. METHODS: Data was obtained from the Aging with Down Syndrome and Intellectual Disabilities (ADSID) research database, consisting of data extracted from clinical records of patients seen by Community Intellectual Disability Services (CIDS) in England. Survival times when considering different risk factors were calculated. RESULTS: The mean age of diagnosis was 55.80 years, SD 6.29. Median survival time after diagnosis was 3.78 years, and median age at death was approximately 60 years. Survival time was associated with age of diagnosis, severity of intellectual disability, living status, anti-dementia medication status, and history of epilepsy. Age at diagnosis and treatment status remained predictive of survival time following adjustment. CONCLUSION: This study provides the best estimate of survival in dementia within the DS population to date, and is in keeping with previous estimates from smaller studies in the DS population. This study provides important estimates and insights into possible predictors of survival and age of diagnosis of AD in adults with DS, which will inform selection of participants for treatment trials in the future.

Developing guidelines for disclosure or non-disclosure of bad news around life-limiting illness and death to people with intellectual disabilities.

BACKGROUND: There is insufficient evidence to guide decisions around (non-)disclosure of bad news of life-limiting illness and death to people with intellectual disabilities. AIM: The aim of this study was to develop guidelines for decisions about (non-)disclosure of bad news around life-limiting illness and death to people with intellectual disabilities, by examining stakeholders' preferences (and reasons) around disclosure and non-disclosure. MATERIALS AND METHODS: Focus groups and interviews were held with 109 participants: people with intellectual disabilities, family carers, intellectual disabilities professionals and medical healthcare professionals. RESULTS: People with intellectual disabilities had wide-ranging views about disclosure. Reasons for non-disclosure included: preventing distress; too difficult for the bearer of bad news; the bearer of bad news lacks knowledge; inability to understand; no sense of time; conflicting views among stakeholders. Reasons for disclosure included: a right to know; knowledge helps the person cope; need for involvement. CONCLUSION: Disclosure of bad news could cause harm in some situations, but this needs careful assessment. The authors present guidelines for disclosure that now need to be tested in practice.

Exploring the lived experiences of people with learning disabilities who are dying of cancer.

BACKGROUND: Growing numbers of people with learning disabilities are living longer and dying of age related illnesses such as cancer. AIM: To explore the experiences of people with learning disabilities who have cancer. METHOD: The study used participant observation with 13 people with learning disabilities. All had a cancer diagnosis and 10 were terminally ill. Participants were visited regularly at home and in other settings, including hospitals. RESULTS: The main themes were: dependent lives; deprived lives; truth telling and understanding; the importance of families; inexperienced carers and unprepared services; and resilience. CONCLUSION: To understand the experiences of people with learning disabilities who are dying of cancer, it is important to understand their previous life experiences and key relationships. Healthcare professionals who treat people with respect, dignity and openness can make a positive difference to their care.

Disclosure and understanding of cancer diagnosis and prognosis for people with intellectual disabilities: findings from an ethnographic study.

PURPOSE: Growing numbers of people with intellectual disabilities are diagnosed with a life-limiting illness such as cancer. Little is known about disclosure of diagnosis and prognosis to this group. The study aim was to explore how much people with intellectual disabilities who have cancer understand about their diagnosis and prognosis, and to explore how much they are told about their cancer. METHOD: 13 people with intellectual disabilities and cancer took part in a 3-year ethnographic study. Data collection consisted mostly of participant observation. Participants were visited regularly for a median of 7 months. RESULTS: Eleven participants were told that they had cancer, but most were not helped to understand the implications of this diagnosis or their prognosis. Decisions around disclosure, as well as the task of truth-telling, rested mostly with relatives and paid carers. Those with severe/profound intellectual disabilities were most likely to be protected from the truth. Understanding was affected by cognitive ability, life experience and truth-telling. Lack of understanding affected the ability to take decisions about treatment and care. CONCLUSIONS: Existing models for breaking bad news are inadequate for people with intellectual disabilities. The findings suggest that more open communication is needed, but further studies are needed to establish best practice in this area.

People with learning disabilities who have cancer: an ethnographic study.

BACKGROUND: Cancer incidence among people with learning disabilities is rising. There have been no published studies of the needs and experiences of people with learning disabilities and cancer, from their own perspective. AIM: To provide insight into the experiences and needs of people with learning disabilities who have cancer. DESIGN OF STUDY: Prospective qualitative study, using ethnographic methods. SETTING: Participants' homes, hospitals, nursing homes, and hospices in London and surrounding areas. METHOD: The participants were 13 people with learning disabilities ranging from mild to severe, who had a cancer diagnosis. The main method of data collection was participant observation (over 250 hours). The median length of participation was 7 months. RESULTS: Participants' cancer experiences were shaped by their previous experience of life, which included deprivation, loneliness, and a lack of autonomy and power. They depended on others to negotiate contact with the outside world, including the healthcare system. This could lead to delayed cancer diagnosis and a lack of treatment options being offered. Most participants were not helped to understand their illness and its implications. Doctors did not make an assessment of capacity, but relied on carers' opinions. CONCLUSION: Urgent action is warranted by findings of late diagnosis, possible discrimination around treatment options, and lack of patient involvement and assessment of capacity in decision making. There are significant gaps in knowledge and training among most health professionals, leading to disengaged services that are unaware of the physical, emotional, and practical needs of people with learning disabilities, and their carers.

Using Nominal Group Technique to investigate the views of people with intellectual disabilities on end-of-life care provision.

AIM: This paper is a report of a study using the Nominal Group Technique as a method to elicit the views of people with intellectual disabilities on sensitive issues, in this example end-of-life care provision. BACKGROUND: Establishing consumer views is essential in providing appropriate end-of-life care, yet people with intellectual disabilities have historically been excluded from giving their opinion and participating in research. METHODS: Nominal Group Technique was used in three groups, with a total of 14 participants who had mild and moderate intellectual disabilities. This technique involves four steps: (1) silent generation of ideas, (2) round robin recording of ideas; (3) clarification of ideas and (4) ranking of ideas (voting). Participants were presented with an image of a terminally ill woman (Veronica), and were asked: 'What do you think people could do to help Veronica?' FINDINGS: Participants generated a mean of nine individual responses. The highest rankings were given to issues around involvement in one's own care, presence of family and friends, offering activities to the ill person, and physical comfort measures. CONCLUSION: People with mild and moderate intellectual disabilities are capable of expressing their views on end-of-life care provision, and should be asked to do so. The Nominal Group Technique presents an effective and acceptable methodology in enabling people with intellectual disabilities to generate their views.

People with intellectual disabilities and their need for cancer information.

Many people with intellectual disabilities will be affected by cancer in their lifetime. There is a lack of available knowledge about people with intellectual disabilities' understanding and experience of cancer, and about their need for cancer information. The principal aim of the study was to explore the information needs of people with intellectual disabilities who are affected by cancer. This was a qualitative, hypothesis generating study. Nine people with intellectual disabilities who were affected by cancer were identified using a purposive sampling method; five of these participated in the study. Data collection included observation of the use of a pictorial cancer information book designed for people with intellectual disabilities, and semi-structured interviews. Data analysis revealed three themes: (1) "Nobody told me": the desperate need for cancer information; (2) "That reminds me": telling my own story; and (3) "I don't know much about cancer": the difficulties of the staff supporting people with intellectual disabilities to read the cancer information book. The people with intellectual disabilities in this study were not provided with information about cancer. They wanted this information, and appeared to be capable of comprehending it. Further development of accessible information materials around cancer and palliative care is needed.