End-stage chronic liver disease: Time to define a good death
Authors: John Iredale
Journal: Hepatology (2008)

Much has been written about the epidemic of chronic liver disease faced in the West as a result of hepatitis C, alcohol use, and nonalcoholic steatohepatitis and in the East as a result of chronic viral liver disease. For each of these examples, the end stage of years of repetitive cycles of inflammation and repair is advanced fibrosis and cirrhosis with the attendant complications, which are challenging to treat and which seriously affect patients' quality of life. Additionally, end-stage cirrhotic liver disease is associated, of course, with the development of hepatocellular cancer. Our approach to this challenge has been the development and refinement of hepatic transplantation. Transplantation represents a real success story of the latter part of the 20th century because it has transformed the prognosis of diseases which until comparatively recently were considered fatal.1 But for the cohort for which we can successfully perform transplantation, we also manage a cohort of patients who are either unsuitable for transplantation, because of the presence of comorbid factors, or who are unable to undergo transplantation for other technical reasons or because of the shortage of donor livers. By definition, this group of patients has an appalling prognosis. Indeed, disease models, the Model for End-Stage Liver Disease and Child-Pugh scores, and the development of significant complications such as ascites and spontaneous bacterial peritonitis frequently predict an outlook for these patients worse than many advanced cancers. Our patients with end-stage liver disease require increasingly complex medical support and manifest a spectrum of complications that significantly impact on quality of life, such as the development of ascites, encephalopathy, and cachexia.2, 3 At the same time, our flexibility to deploy pharmaceutical-based approaches is limited by the capacity of the end-stage diseased liver to metabolize many drugs commonly used in advanced disease, such as opiates.2-4 The analogy with end-stage cancer is an important one. In many developed countries, the cancer services have established effective and dynamic systems of palliative care ensuring that the final illness of the patient is characterized by as high as possible a quality of life. The approaches taken in this setting are becoming increasingly based on evidence from studies in community and hospital settings. These studies encompass the effects on quality of life of specific interventions or treatments. But to evaluate the effects on quality of life after an intervention, a baseline undertaking of patient needs and expectations is required. A key tool which has proven valuable in defining this evidence in the palliative core setting has been qualitative in-depth interviews with patients and their caregivers during their final illnesses.6, 7 This structured approach; defining the needs and requirements of patients with nonmalignant terminal diseases, together with clinical and epidemiological studies that allow a plotting of illness trajectory, has been successfully deployed in the pulmonary and renal fields to provide evidence-based guidelines to trigger specific interventions and the institution of specific services and treatments as the patient deteriorates.8 In the area of palliation for nonmalignant end-stage disease, we are falling behind our pulmonary, cardiology, and renal colleagues. The recently published Gold Standard Framework for prognostic guidance and engagement of palliative care published in the United Kingdom, for example, contains clear guidelines for identifying those with terminal (life expectancy less than 1 year) illnesses based on clearly definable parameters applicable in primary and secondary core settings for: cardiac failure, pulmonary disease, renal disease, and degenerative neurological disorders.8 Liver disease is notable by its absence. With a few key exceptions, there is little in the literature on the appropriate palliative approach for patients with end-stage liver disease. While those publications are valuable and authoritative,2-5 there is huge scope for us as a community to define what we need to do for these patients and to identify and evaluate the most appropriate way of treating the very distressing symptoms that occur to those with end-stage liver disease in the terminal setting. Indeed, defining when to pull out of active treatment with such patients can be an extremely difficult and challenging clinical decision. In his excellent book "Hippocratic Oaths", Raymond Tallis illustrates the possible futures of old age in the form of a series of graphs.9 This type of plot could be used to illustrate what we should be aiming for in our patients with end-stage liver disease. If we were to plot quality of life on the y axis against time on the x axis, then I suspect the experience of our end-stage liver failure patients would be represented as an undulating line wandering (with variable steepness) from the top lefthand corner to the bottom right, indicating a relentless decline in quality of life. Whereas our aim should be that the plot runs to as great an extent as possible horizontal and as close to the top of the y axis as possible before suddenly plummeting to the x axis in the days around death. We are not, of course, able to alter the illness trajectory of the diseases which underpin terminal hepatic failure in this setting. However, as clinicians in the 21st century, we need to make it our business to understand the expectations, needs, and requirements of patients dying of end-stage hepatic disease and, on the basis of this data, define the best and most effective approaches to make the final illnesses of our patients as symptom-free and fulfilling as possible.