Barbara Kimbell

Living and dying well with end-stage liver disease: Time for palliative care?†

C hronic liver disease is a major cause of mortality worldwide. In the United Kingdom, liver deaths have been increasing for 30 years and currently constitute the fifth highest cause of mortality. Liver transplantation is only available to a subset of patients who meet strict criteria. Comorbidities and limited donor organ supplies mean that many patients will not receive a graft and so stand to benefit from palliative care delivered in parallel with optimal disease-focused treatment. End-stage liver disease (ESLD) is the final decompensation phase in the liver trajectory. It is characterized by episodic, acute exacerbations, often requiring hospitalization. Life-threatening complications, such as variceal hemorrhage or hepatoma, combine with multiple debilitating symptoms, including ascites, extreme fatigue, pruritus, and cachexia. Patients may also experience cognitive decline, ranging from mild chronic impairment to severe hepatic encephalopathy and coma. Many suffer from psychological distress and depression. Advanced liver disease generally affects younger people of working age. Financial and social problems as well as the stigma of liver disease can have a profoundly negative impact on the quality of life of the patient and their family. Given the plethora of complex physical, psychological, existential, social, and family problems that are the norm in advanced liver disease, it is perhaps surprising that so little attention has been paid to understanding and addressing the wider illness experience of these patients and families. The hepatology literature has largely focused on the clinical complications of liver disease and treatment options that may improve both prognosis and quality of life. Health-related qualityof-life research is starting to provide insights into the burden of advanced liver disease and how people cope with waiting for transplantation. The liver illness trajectory is very different from that found in a typical progressive cancer, the disease paradigm for which most palliative care provision has been developed. These patients face an uncertain illness trajectory with the possibility of improvement, if they can stop drinking, or rapid deterioration and death during an acute admission for a life-threatening complication. Palliative care may be viewed incorrectly as only applicable once the patient is in the final days of life and all other treatment options have been exhausted. Palliative care policies in the United Kingdom and internationally aim to ensure that patients with any lifelimiting illness who are approaching the last months of life are identified and assessed. Given the lack of a clear ‘‘terminal phase’’ and the difficulties of accurate prognostication at an individual level, patients who are likely to be ‘‘at risk of dying’’ from advanced liver disease in the next year are candidates for palliative care. We suggest that clinical judgement informed by generic indicators, such as recurrent, unplanned hospital admissions and multimorbidity, can help trigger a review. Disease-specific prognostic tools, such as the ChildPugh or Model for End-stage Liver Disease scores, are evidence-based markers of some of the major complications of decompensated disease, and patients on the transplant waiting list also have end-stage disease. Palliative care has well-defined, supportive care goals related to optimizing quality of life and addressing information needs about the illness and prognosis alongside symptom control, psychosocial support, and spiritual care of the patient and their family. Continuity of care consistent with the patient’s wishes should then be provided through effective care planning. Goals, interventions, and plans for managing progressive deterioration or a potential episode of acute decompensation can be agreed upon with patients and families and communicated to all those providing care. In addition, expertise in symptom control for patients with other types of organ failure is more advanced, whereas a robust evidence base for safe prescribing in liver disease, with its complex pathophysiology and pharmacokinetics, is lacking. Pain may be caused by comorbidities, liver disease complications, or hepatoma. There are no long-term studies of paracetamol (acetaminophen) use in patients with cirrhosis, but at a reduced dose, it is the safest option for mild pain. Opioids can be problematic in a population Abbreviation: ESLD, end-stage liver disease. Address reprint requests to: Kirsty Boyd, M.B.Ch.B.(Hon.), F.R.C.P. (Edin.) M.Med.Sci., The University of Edinburgh, Centre for Population Health Sciences, Primary Palliative Care Research Group, Medical School, Teviot Place, Edinburgh, EH8 9AG, UK. E-mail: Kirsty.Boyd@ed.ac.uk; fax: (0131)-650 9119. CopyrightVC 2012 by the American Association for the Study of Liver Diseases. View this article online at wileyonlinelibrary.com. DOI 10.1002/hep.25621 Potential conflict of interest: Nothing to report.

Different Experiences and Goals in Different Advanced Diseases: Comparing Serial Interviews with Patients with Cancer, Organ Failure or Frailty and Their Family and Professional Carers

CONTEXT Quality care for people living with life-limiting illnesses is a global priority. A detailed understanding of the varied experiences of people living and dying with different conditions and their family and professional caregivers should help policymakers and clinicians design and deliver more appropriate and person-centered care. OBJECTIVES To understand how patients with different advanced conditions and their family and professional caregivers perceive their deteriorating health and the services they need. METHODS We used a narrative framework to synthesize eight methodologically comparable, longitudinal, and multiperspective interview studies. We compared the end-of-life experiences of people dying from cancer (lung, glioma, and colorectal cancer), organ failure (heart failure, chronic obstructive pulmonary disease, and liver failure), and physical frailty and those of their family and professional caregivers in socioeconomically and ethnically diverse populations in Scotland. RESULTS The data set comprised 828 in-depth interviews with 156 patients, 114 family caregivers, and 170 health professionals. Narratives about cancer typically had a clear beginning, middle, and an anticipated end. Cancer patients gave a well-rehearsed account of their illness, hoping for recovery alongside fear of dying. In contrast, people with organ failure and their family caregivers struggled to pinpoint when their illness began, or to speak openly about death, hoping instead to avoid further deterioration. Frail older people tended to be frustrated by their progressive loss of independence, fearing dementia or nursing home admission more than dying. CONCLUSION These contrasting illness narratives affect and shape the experiences, thoughts, and fears of patients and their carers in the last months of life. Palliative care offered by generalists or specialists should be provided more flexibly and equitably, responding to the varied concerns and needs of people with different advanced conditions.

Managing uncertainty in advanced liver disease: a qualitative, multiperspective, serial interview study

Objective To understand the experiences and support needs of people with advanced liver disease and those of their lay and professional carers to inform improvements in the supportive and palliative care of this rapidly growing but currently neglected patient group. Design Multiperspective, serial interviews. We conducted up to three qualitative in-depth interviews with each patient and lay carer over 12 months and single interviews with case-linked healthcare professionals. Data were analysed using grounded theory techniques. Participants Patients with advanced liver disease of diverse aetiologies recruited from an inpatient hepatology ward, and their lay carers and case-linked healthcare professionals nominated by the patients. Setting Primary and secondary care in South-East Scotland. Results 37 participants (15 patients, 11 lay and 11 professional carers) completed 51 individual and 13 joint patient-carer interviews. Nine patients died during the study. Uncertainty dominated experiences throughout the course of the illness, across patients’ considerable physical, psychological, social and existential needs and affected patients, lay carers and professionals. This related to the nature of the condition, the unpredictability of physical deterioration and prognosis, poor communication and information-sharing, and complexities of care. The pervasive uncertainty also shaped patients’ and lay carers’ strategies for coping and impeded care planning. While patients’ acute medical care was usually well coordinated, their ongoing care lacked structure and focus. Conclusions Living, dying and caring in advanced liver disease is dominated by pervasive, enduring and universally shared uncertainty. In the face of high levels of multidimensional patient distress, professionals must acknowledge this uncertainty in constructive ways that value its contribution to the persons coping approach. Pervasive uncertainty makes anticipatory care planning in advanced liver disease challenging, but planning ‘just in case’ is vital to ensure that patients receive timely and appropriate supportive and palliative care alongside effective management of this unpredictable illness.

What is the patient experience in advanced liver disease? A scoping review of the literature

Background There has been a dramatic increase in liver disease over recent decades, with morbidity and mortality rates predicted to rise significantly. In order to effectively support this growing patient population we need to understand the experiences, key issues and priorities of people living and dying with advanced liver disease. Aims To establish what is currently known about the patient experience of advanced liver disease. Methods Scoping literature review. We searched MedLine, Web of Science, CINAHL and PsychINFO databases. Eligibility criteria: original research and review papers written in English since 1990 relating to the adult patient experience of advanced liver disease. Papers focusing on treatment development, acute liver failure or post liver transplantation were excluded. Results 240 abstracts were identified and 121 articles reviewed in full. Patients with advanced liver disease experience many physical and psychosocial challenges, often of a greater severity than those reported in other advanced conditions. They are affected by gastrointestinal problems and fatigue, with both linked to higher levels of depression. Socio-demographic variations in the patient experience occur. Only two studies explored the holistic patient experience. Conclusions Our knowledge of the patient experience in advanced liver disease is mainly from quality of life instruments and quantitative research. There are methodological limitations in the existing research such that the holistic patient experience is not well described. Qualitative longitudinal research has great potential for contributing to our understanding of the complex needs and experiences of patients living with advanced liver disease.

A “good death” with irreversible liver disease: Talking with patients and families about deteriorating health and dying

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Embracing inherent uncertainty in advanced illness

B Kimbell and colleagues argue that we should be helping people plan for possible future needs rather than trying to provide certainty

Palliative care for people with advanced liver disease: A feasibility trial of a supportive care liver nurse specialist:

Background: Liver disease is an increasing cause of death worldwide but palliative care is largely absent for these patients. Aim: We conducted a feasibility trial of a complex intervention delivered by a supportive care liver nurse specialist to improve care coordination, anticipatory care planning and quality of life for people with advanced liver disease and their carers. Design: Patients received a 6-month intervention (alongside usual care) from a specially trained liver nurse specialist. The nurse supported patients/carers to live as well as possible with the condition and acted as a resource to facilitate care by community professionals. A mixed-method evaluation was conducted. Case note analysis and questionnaires examined resource use, care planning processes and quality-of-life outcomes over time. Interviews with patients, carers and professionals explored acceptability, effectiveness, feasibility and the intervention. Setting/participants: Patients with advanced liver disease who had an unplanned hospital admission with decompensated cirrhosis were recruited from an inpatient liver unit. The intervention was delivered to patients once they had returned home. Results: We recruited 47 patients, 27 family carers and 13 case-linked professionals. The intervention was acceptable to all participants. They welcomed access to additional expert advice, support and continuity of care. The intervention greatly increased the number of electronic summary care plans shared by primary care and hospitals. The Palliative care Outcome Scale and EuroQol-5D-5L questionnaire were suitable outcome measurement tools. Conclusion: This nurse-led intervention proved acceptable and feasible. We have refined the recruitment processes and outcome measures for a future randomised controlled trial.

DANCING TO A DIFFERENT TUNE: LIVING AND DYING WITH CANCER, ORGAN FAILURE AND PHYSICAL FRAILTY

Introduction The “danse macabre” has been depicted in Europe since medieval times. Providing good end-of-life care for all patients, whatever form their last dance takes, is a global challenge. Aim(s) and method(s) To explore typical narratives of living with and dying from advanced illnesses, to provide insights into providing effective care. Secondary analysis of data from 8 qualitative longitudinal studies: 3 cancer; 3 organ failure; 1 frailty; 1 with South Asian participants from all 3 trajectories. Patients were interviewed up to 4 times over 18 months. Researchers from each study reviewed the data using a narrative analytic framework. The narratives were initially synthesised by illness trajectory, then compared and discussed at analysis workshops. Results The dataset was 828 in-depth interviews with 156 patients. Cancer narratives had a well-rehearsed beginning, middle and anticipated end, with dual themes of hope for recovery alongside fear of dying. People with organ failure struggled to tell their story, being unclear when the illness began, or how one event linked to another. Fewer spoke about death, hoping instead to avoid further deterioration. Frail older peoples narratives often began with a specific event in combination with ageing, yet lost salience amidst increasing losses and future fears. Death was only fully anticipated very near the end, and feared less than nursing homes or dementia. Conclusion(s) Patients from different illness groups gave very different accounts. Consequently the cancer-based model of end-of-life care seems poorly suited to the needs of those dying in other ways. Understanding how different patient groups perceive their deteriorating health and approaching death will inform appropriate future palliative care for all.

Liver disease in the UK

We welcome the publication of the Lancet Commission (Nov 29, p 1953) on liver disease in the UK and its important recommendations to improve care for this growing population of patients. However, we were surprised to see minimum reference to the provision of high quality supportive and palliative care for the many patients dying with advanced liver disease. The report makes clear how common death from liver disease is. The population with liver disease is younger and growing faster than populations with other types of organ failure, and has many complex needs. We fully support the directed measures to reduce preventable deaths, but the fact remains that for the foreseeable future many patients will continue to die. A clear and immediate need exists for evidence-based guidance about best practice in care of people with advanced liver disease to ensure that they are identifi ed as being at risk of further deterioration and death, and then receive appropriate supportive care alongside optimum treatment of their disease. This issue is emphasised in Getting it Right: Improving End of Life Care for People Living with Liver Disease, a report by the NHS Liver Care published in 2013. Clear guidance exists for the palliative care of non-malignant end-stage disease in the kidney, lung, and heart. However, a pressing need and opportunity exist to develop programmes of research to inform the development of such guidelines for liver disease too.

DIFFERENT DYINGS: LIVING AND DYING WITH CANCER, ORGAN FAILURE AND PHYSICAL FRAILTY

Background Recent controversy around the Liverpool Care Pathway has highlighted the need to listen to patient and carer perspectives. Better understanding of how different patient groups perceive their deteriorating health and approaching death can inform palliative care developments for all conditions. Aim To establish whether there are typical narratives of living with and dying from a variety of progressive conditions, that might provide insights into how best to provide effective, patient-centred care. Methods We used a narrative framework to synthesise eight methodologically comparable, longitudinal, interview studies undertaken by our research group. We examined the narratives of people dying from cancer (lung, glioma and colorectal cancer), organ failure (heart, liver and chronic obstructive pulmonary disease) and physical frailty, in socioeconomically and racially diverse populations in Scotland. Results The dataset comprised 828 in-depth interviews with 156 patients, 114 family caregivers and 170 health professionals. The cancer narratives typically had a clear beginning, middle and anticipated end. They gave a well-rehearsed account of the illness, with the dual themes of hope for recovery alongside fear of dying. In contrast, people with organ failure struggled to pinpoint when their illness began, or see meaningful patterns between acute events. Fewer spoke about death, hoping instead to avoid further deterioration. Some attributed their poor health to ‘old age’, others struggled to identify any specific illness. Frail older people were frustrated by their progressive loss of independence, fearing dementia or nursing home admission more than dying. Conclusions The typical cancer narrative is very different from those in frailty or organ failure. Palliative care as a concept may be too inextricably linked to imminent death to be acceptable and appropriate for these patients. We must diversify palliative care provision to meet the range of holistic needs of all patients approaching the end of life.