KEY POINTS Complications of end-stage liver disease include ascites, HE, variceal bleeding, spontaneous bacterial peritonitis, and coagulopathy—all irreversible conditions without liver transplantation. Pain is one of the most common symptoms experienced by patients with end-stage liver disease. Advanced care planning should be a standard part of caring for patients with decompensated cirrhosis. INTRODUCTION Cirrhosis is a progressive chronic illness that develops as a result of irreversible scarring and fibrosis of the liver parenchyma, ultimately disrupting liver architecture and function. Although it often begins as an asymptomatic condition when compensated, the clinical trajectory can deteriorate rapidly as patients develop decompensated cirrhosis, also referred to as end-stage liver disease (ESLD). Complications of ESLD include ascites, HE, variceal bleeding, spontaneous bacterial peritonitis, and coagulopathy. Physical and psychological symptoms associated with these conditions are often distressing and decrease both patient and caregiver quality of life.2 Commonly reported symptoms in the ESLD population include pain, fatigue, breathlessness, muscle cramps, anxiety, depression, sexual dysfunction, insomnia, and pruritis.6 The only curative treatment for cirrhosis is liver transplantation. In the absence of liver transplant, median survival for those with decompensated cirrhosis is 2 years, with improvement to 12 years in compensated cirrhosis. The remainder of the treatment arsenal is therefore aimed at palliation (Figure 1).4 Given the life-limiting nature of this disease, the high symptom burden, and the caregiver burden, this population has the potential to benefit greatly from palliative care (PC).4FIGURE 1: Approach to care for patients with end-stage liver disease.Palliation of symptoms PC is a comprehensive approach to patient care focused on symptom management, quality of life, and the psychosocial needs of patients with chronic illness and their caregivers (Figure 2).6 PC is not equivalent to hospice care and can be provided to patients with decompensated cirrhosis at any stage of illness, regardless of active medical therapy or transplant status.5 PC can be delivered by the primary medical team and by specialized PC physicians as cases become more complex. The medical treatment of portal hypertensive complications, such as HE and ascites, is itself a palliation of symptoms and an example of primary PC.FIGURE 2: Toolkit for palliation and end of life care in patients with decompensated cirrhosis. Abbreviations: MELD, model for end-stage liver disease; SSRI, selective serotonin reuptake inhibitor.Pain management Pain is one of the most common symptoms experienced by patients with ESLD, with prevalence noted to be 30%–77%.3 It is often localized to the abdomen in relation to ascites or spontaneous bacterial peritonitis. Generalized muscle cramping is another common source of pain in this population, with prevalence noted to be 56%–68%.3 Pharmacologic pain management is challenging in patients with liver disease due to changes in drug absorption, distribution, and metabolism.7 Management should be directed toward underlying causes of pain, such as volume management in ascites. If pain persists despite disease-directed therapies, then nonpharmacologic modalities, such as physical therapy, heat/ice, and massage therapy, should be used, followed by nonopioid pharmacologic therapy, such as topical therapies, acetaminophen (up to 2 g/d) and neuropathic pain medications, including gabapentin and pregabalin.7 NSAIDs are relatively contraindicated due to the increased risk of bleeding and renal dysfunction. When opioids are used, they should be started at the lowest dose to decrease the risk of accumulation and subsequent encephalopathy.2 Oral morphine and hydromorphone and IV fentanyl are preferred, as oxycodone and hydrocodone are largely metabolized by the liver.7 Hepatic encephalopathy Confusion secondary to HE is another highly distressing symptom associated with ESLD, for both the patient and the family. It presents on a spectrum and can be overlooked when patients with cirrhosis begin having subtle sleep disturbances or difficulty at work.7 HE is associated with decreased independence, loss of employment, increased hospitalizations, and increased caregiver distress, resulting in decreased quality of life. Therefore, it is crucial to diagnose and treat HE. Lactulose remains the first-line therapy for HE, but polyethylene glycol is an appropriate substitution for those who cannot tolerate lactulose due to abdominal bloating. Rifaximin can be used in conjunction, but use is often limited by cost.7 Ascites Ascites is one of the most common complications of ESLD and indications for hospitalization.4 The mainstay of therapy is diuresis with lasix and spironolactone along with dietary sodium restrictions (≤2 g sodium/d). Medical management is often limited by hyponatremia, hypotension, and renal dysfunction as ESLD progresses. Considerations for the next line of therapy for refractory ascites include repeated paracenteses versus transjugular intrahepatic portosystemic shunting. The development of refractory ascites not only increases the symptom burden but is also associated with an increased 1-year mortality of ~70%.2 Therefore, patients in this position may benefit from specialty PC referrals, given their increased mortality and higher symptom burden. Psychological burden Accompanying physical complications is a significant psychological symptom burden among ESLD patients. A multicenter observational study done in 2020 demonstrated that there are high rates of depression (16%) and anxiety (43%) in this population.7 There are a multitude of contributing factors to the disruption of mental health, including the financial burden of health care, the perceived burden on caregivers, and substance use. Selective serotonin receptor inhibitors remain the first line in ESLD for depression and anxiety. Sedating medications such as tricyclic antidepressants and benzodiazepines are avoided because of their sedating effects and association with worsened HE.2 Role of advanced care planning Hepatologists routinely practice components of PC by managing complications of decompensated cirrhosis. As part of this approach, it is imperative to have advanced care planning discussions. Given the unpredictable trajectory of cirrhosis, it is important to have these conversations early on, if possible, before the development of HE or another acute decompensation event.7 Data from the SUPPORT study demonstrated that 43% of patients with at least 2 features of decompensated cirrhosis would prefer to die than live in a nursing home and that the majority would prefer to die rather than be sustained on life-supporting measures.6 Despite this, patients with ESLD tend to have higher hospitalization rates and more aggressive care in their last year of life, which underscores the importance of early advanced care planning conversations.7 Caregiver fatigue and support Overall, decompensated cirrhosis is a complex chronic disease process that places significant physical and psychosocial burdens on the patient and caregiver, which makes this population appropriate candidates for specialized PC. Despite this, PC is underutilized in decompensated cirrhosis, and when used, the referrals are often delayed, defined as <30 days before death.1 There is often pause before referring to PC in decompensated cirrhosis because of the possibility of liver transplant, but nonhospice PC can be used concurrently with active treatment and transplant evaluation. The early involvement of PC improves physical and psychological well-being as well as quality of life.2 Qualitative studies show that caregivers also experience decreased quality of life, anxiety, and depression, all of which can benefit from a multidisciplinary PC team.4 Role of hospice care Lastly, as the disease course continues to progress, hospice care should be considered for patients who prefer to pursue comfort and have a prognosis of <6 months. Although the Child-Pugh and model for end-stage liver disease (MELD) scores are used for transplant allocation, they can be helpful for hospice referral as well.7 Per the American Association for the Study of Liver Diseases (AASLD) guidance statements on palliative management in decompensated cirrhosis, a MELD score>21 or Child-Pugh score ≥12 is associated with a life expectancy of ~6 months, which can be used for prognostication when considering the end of life care.5 Hospice care is associated with improvement in physical and psychological symptoms along with caregiver stress and bereavement. Ultimately, PC can be integrated into the care of ESLD patients in a variety of ways and should be considered both early and often.
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