Advance Care Planning and Goals of Care Discussions in Advanced Liver Disease

Abstract

Purpose of Review

Advance care planning (ACP) has been shown to improve multiple outcomes in patients with serious illnesses; however, patients with advanced liver disease, or cirrhosis, rarely receive timely ACP. The purpose of this review is to evaluate the current state of ACP for patients with advanced liver disease and to provide practical guidance for integrating early ACP into routine outpatient hepatology care.

Recent Findings

Due to multiple patient-, physician-, and systems-level barriers, patients with advanced liver disease have low rates of timely ACP engagement. Early identification and preparation of a surrogate decision-maker, enhancing clinician ACP communication skills, use of patient-centered educational tools, optimizing outpatient processes for ACP documentation, and early referral to specialty palliative care may help to improve current deficits in ACP for this population.

Summary

Provision of early ACP to patients with advanced liver disease may better ensure that patients receive medical care that is consistent with their goals and values.

Introduction

Advanced liver disease, or cirrhosis, affects over 600,000 adults and is the 11th leading cause of death in the USA [1, 2]. Once patients with advanced liver disease develop complications such as ascites, hepatic encephalopathy, or variceal bleeding, they also face limited life expectancy with a median survival of 2 years in the absence of liver transplantation [3]. Despite the option of liver transplantation as curative therapy, fewer than 1 in 12 patients with advanced liver disease are ever transplanted [4]. Furthermore, patients with advanced liver disease often experience frequent hospitalizations over the course of their illness, including at end of life [5]. Advanced liver disease is a diagnosis marked by substantial morbidity and mortality which underscores the critical need to optimize advance care planning (ACP) for this population.

Defining Advance Care Planning

ACP is the iterative and longitudinal process by which health care providers prepare patients and families for their medical decision-making over the course of their illness trajectory, including at end-of-life, to ensure that their approach to future medical care reflects their personal goals, values, and preferences [6]. ACP involves many components, including illness education, prognostic disclosure, assessment of goals and values, shared decision-making, designation of a surrogate decision-maker, and formal documentation of patients’ goals for medical and end-of-life care through medical orders and written documents. Timely ACP has been associated with improved goals of care (GOC) documentation and satisfaction with medical care, increased palliative care consultations and hospice utilization, and reduced emotional distress and healthcare utilization at the end of life for patients with serious illnesses [7].

Current State of End-of-Life Care in Advanced Liver Disease

Patients with advanced liver disease experience high healthcare utilization as they approach end of life. Between 61 and 78% of patients with advanced liver disease die in the hospital [8–12]. While rates of hospice utilization have been gradually increasing over time, only 10% of patients with advanced liver disease receive hospice care services at death [8, 13]. In addition to dying in hospitalized settings, patients with advanced liver disease also receive intensive medical care as they approach end of life. In a retrospective study of 102 patients with cirrhosis removed or declined from the liver transplantation waiting list, patients had an average of 2 hospital admissions after delisting with 48% utilizing intensive care unit (ICU) care [14]. In a population-based study, patients with advanced liver disease were twice as likely to require ICU care or die in the ICU in the last 90 days of life compared to patients without advanced liver disease [12]. The intensity of end-of-life care that these patients receive is also reflected in their costs of care; median heath care costs in the last 3 months of life for patients with advanced liver disease outpace those for patients with renal and cardiovascular disease [12]. Despite the high healthcare utilization of patients with advanced liver disease, it is not clear that these invasive treatments lead to meaningful changes in their life expectancy or quality of life. For one, patients with advanced liver disease often receive invasive life-sustaining therapies during their terminal hospitalizations, with >58% receiving mechanical ventilation, 45% receiving renal replacement therapy, and 25% receiving endoscopy at end of life in observational studies [15, 16]. Secondly, approximately 6% of hospitalized patients with advanced liver disease receive cardiopulmonary resuscitation (CPR), despite only 11% of these patients surviving to hospital discharge and only 3% able to be discharged home [15, 17]. Lastly, in a large multicenter study of 575 patients with advanced liver diseases, 43% of patients reported that they would rather die than be placed in a nursing home, and 75% reported that they would rather die than live in a coma [18••]

Current State of ACP in Advanced Liver Disease

The current state of end-of-life care in advanced liver disease highlights current deficiencies in ACP for this population. In a prospective study of outpatients with advanced liver disease, only 33% of patients had identified a surrogate decision-maker [19•]. In a study of patients awaiting liver transplantation, 0% had legal ACP forms or end-of-life care wishes documented in the medical chart despite 85% of patient reporting readiness to discuss and document their preferences for EOL care [20•]. These rates of advance directive completion are much lower compared to the national average; approximately 40% of patients with chronic illnesses have completed a living will, health care power of attorney, or both [21]. Similar findings have been reported among transplant-ineligible patients with advanced liver disease; in one retrospective study, 0% of transplant-ineligible patients had ACP discussions documented in their medical charts during their outpatient hepatology clinic visits [22]. In another series, only 28% of patients with cirrhosis who were delisted for liver transplantation had a “do not resuscitate” designation in their medical chart [14]. In a survey study of hepatology providers nationally, 81% reported that ACP discussions with patients with advanced liver disease occur too late [23••]. Over 60% of hepatology providers in this study reported that initial ACP discussions regarding resuscitation status, preferred site of death, or hospice care occurred either during a patient’s acute hospitalization or when a patient’s death was clearly imminent [23••]. A qualitative study of 46 transplant center clinicians and 42 patients with decompensated cirrhosis revealed that patients are open to conversations about their values and types of care they would like to receive at end of life [24••]. Despite this, transplant clinicians viewed their role as getting patients to transplant and avoided proactive discussions regarding death and dying with patients.

Barriers to ACP for Patients with Advanced Liver Disease

Multiple barriers to early advance care planning for patients with advanced liver disease have been reported in prior studies. The unpredictable illness trajectory of advanced liver disease, in which patients can experience acute and severe clinical deteriorations that require hospitalizations, complicates the timing and setting of ACP discussions [18••, 25]. Over 40% of patients with advanced liver disease will ultimately develop hepatic encephalopathy, which substantially impairs their capacity to make their own medical decisions [3]. In qualitative studies, hepatology providers reported that they most often conduct GOC discussions with family members of patients with advanced liver disease during the patients’ last month of life [26]. This finding underscores the impact of hepatic encephalopathy in precluding ACP discussions with patients regarding their preferences for medical and EOL care if conducted late in the disease course. The unpredictability of advanced liver disease also leads to uncertainty amongst patients and their families regarding planning for the future [27, 28]. This can cause prognostic discordance among hepatology providers, patients, and families, particularly regarding end-of-life care decision-making [29, 30]. This leaves patients and their families at high risk for emotional distress, caregiver burden, and complicated bereavement in the absence of early ACP discussions to support them in their medical decision-making over the course of their illness experience, including at end of life [30–32].

The option of curative liver transplantation may also delay end-of-life care conversations for patients with advanced liver disease who are transplant-eligible. Patients with advanced liver disease have no well-accepted bridge to liver transplantation, unlike the availability of renal replacement therapy for patients with end-stage renal disease or mechanical circulatory support therapy for patients with advanced heart failure. Qualitative studies of patients with advanced liver disease and transplant clinicians have highlighted that the central focus on liver transplantation leads to limited opportunities for goals of care or end-of-life care discussions and may contribute to continued escalation of medical care for these patients in the face of poor prognosis [24••, 25, 29]. This is further reinforced by the requirement of most liver transplant centers that patients on the liver transplantation waiting list should be full code, which necessarily precludes ACP discussions that are centered on end-of-life care planning [23••, 33, 34].

Hepatology clinicians have reported a lack of confidence and skills to engage in ACP discussions, specifically regarding prognosis, medical decision-making, future planning, and end-of-life care with patients with advanced liver disease and their families in prior qualitative and survey-based studies [23••, 26, 35]. A recent study using an objective structured clinical examination to assess the communication skills of gastroenterology fellows highlighted additional deficiencies in GOC communication. In this simulation study, less than half of the trainees were able to successfully conduct an ACP discussion to discuss components of a health care proxy, end-of-life care planning, and importance of advanced directives, and describe hospice care with standardized patients [36]. Additional barriers to engaging patients in ACP reported by hepatology providers have included competing time demands in clinic and limited reimbursement for this process [23••].

Recommended Strategies to Improve ACP in Advanced Liver Disease

The American Gastroenterological Association (AGA) commissioned a Clinical Practice Update on Palliative Care Management in Cirrhosis that was published in 2020 that included an evidence-based review of ten Best Practice Advice (BPA) statements addressing key issues regarding palliative care in cirrhosis care [37••]. The BPA statements that specifically focus on ACP are included in the following recommendations:

Early Identification, Documentation, and Preparation of a Surrogate Decision-Maker

Early identification and documentation of a surrogate decision-maker is critical given the high risk of incapacity in patients with advanced liver disease. Interviews of patients with advanced liver disease reveal that most would prefer to have ACP discussions early in their disease course and prior to the onset of hepatic encephalopathy with a provider that they had a longitudinal, trusting relationship with [25]. Interviews of informal caregivers of patients with advanced liver disease have highlighted that surrogate decision-makers are often unprepared for end-of-life decision-making [24••]. The process of advance care planning can help patients clarify their choice for a surrogate decision-maker through identifying an individual who would be able to make health care decisions for them if they lose their decisional capacity. It is important to note that a patient’s designated surrogate decision-maker may be different from their primary informal caregiver. After designating a surrogate decision maker, it is critical that hepatology clinicians support patients in documenting this process through completing an advanced directive using a Durable Power of Attorney for Health Care (also known as a health care proxy) or a living will.

However, the process of advance care planning in hepatology must go beyond identifying and documenting a surrogate decision-maker to also preparing that individual for this role. Prior studies have shown discordance between patients and their surrogate decision-makers on preferences for medical care [38, 39]. This highlights that surrogates must be prepared for the responsibility of making medical decisions that best reflect the patients’ values and preferences, as opposed to their own, by practicing substituted judgment [40]. Hepatologists must engage both patients and their surrogate decision-makers in a series of conversations to assess their medical knowledge, prognostic awareness, personal values, and goals and preferences for care. Reviewing disease management, trajectory, and complications should be an important aspect of these conversations to allow for anticipatory care planning for patients and their surrogate decision-makers.

Assessing Goals and Values to Guide-Shared Decision-Making

Patients with advanced liver disease experience many complications from their illness and will in turn face many decisions for their medical care outside of considering their candidacy for liver transplantation or preferences for CPR. A key component of ACP is shared decision-making, and hepatologists should support patients, caregivers, and surrogate decision-makers in their medical decision-making in anticipation of these complications through discussing the risks, benefits, burdens, and tradeoffs of their options for future medical care. Treatment options for complications such as ascites, hepatic encephalopathy, variceal bleeding, and more may vary based on a patient’s transplant candidacy, severity of liver disease, underlying comorbidities, available caregiver supports, and, most importantly, their personal values and goals. Integrating options for future medical care is a central component of ACP that allows patients to decide on treatments that best align with their personal values and preferences.

To assess patients’ values and goals for their health, hepatologists should explore their perspectives on what they find most important in life, their fears and worries about their future health, their personal assessment of their quality of life, and what tradeoffs, if any, they are willing to make regarding their physical and mental function as they approach future decisions for their medical care [41••, 42, 43]. For example, the risk of hepatic encephalopathy after a transjugular intrahepatic portosystemic shunt procedure may be unacceptably high for a patient with refractory ascites who values maintaining intact cognition above all else. Communication strategies and skills to facilitate these conversations will be discussed in a later section.

Identifying Clinical Milestones and Major Life Events as Triggers for Prognostic Assessment and Advance Care Planning Discussions

Prognostic assessment and ACP should be iterative processes, and hepatology clinicians should revisit goals of care regularly but especially after sentinel events during which a patient’s values and goals for care may change (Table 1). BPA statement #6 from the 2020 AGA Clinical Practice Update recommends that “prognosis should be evaluated by gastroenterology/hepatology providers during routine care visits and at sentinel events” [37••]. Traditional liver-specific prognostic metrics such as the Model for End-Stage Liver Disease (MELD) score and the Child-Pugh classification have been used to predict mortality for patients with advanced liver disease, but these metrics may not capture additional outcomes that matter to patients and families such as quality of life or functional independence. Tools such as the Liver Frailty Index, 6-min walk test, activities of daily living/instrumental activities of daily living, and Karnofsky performance status may be better able to provide a global assessment of physical function or overall health reserve for patients with advanced liver disease [44].

Table 1.

Suggested clinical milestones and life events that should trigger ACP discussions for patients with advanced liver disease

Liver-specific factors	Patient factors	Major life events
New liver-related complications (ascites, hepatic encephalopathy, variceal bleeding, hepatocellular carcinoma)	Advanced multimorbidity	Recent ICU admission
New diagnosis of refractory ascites	Poor performance status and/or progressive frailty	Recent unplanned hospitalization for a liver-related complication
New diagnosis of acute kidney injury or hepatorenal syndrome	Ongoing substance use disorder	Loss of a spouse or primary caregiver
Recently delisted or declined for liver transplantation	New comorbid diagnosis of cancer, cardiovascular disease, neurological disease	Advanced age

BPA statement #7 from the 2020 AGA Clinical Practice Update emphasizes that GOC discussions should occur at sentinel events such as “hospital or intensive care admission, before initiation of life-supporting therapies, before surgery, on new onset of cirrhosis-related complications, and after determination of transplant eligibility” [37••]. In addition to sentinel clinical events, patients experiencing multimorbidity, progressive functional decline or frailty, escalating healthcare utilization, or significant change in their caregiver supports should be quickly re-engaged by their hepatologists in conversations revisiting their goals for medical care. These conversations should ideally occur in a non-acute setting when the patient is stable and well enough to participate and should involve the patient’s primary caregivers and surrogate decision-maker [25]. During these conversations, clinicians should support patients in updating their advance directives, particularly related to their preferences for rehospitalization and receipt of invasive procedures such as endoscopy in the event of acute illness, resuscitative care, location of death, and hospice care.

How to Integrate Early ACP into Outpatient Hepatology Clinical Practice

Hepatology clinicians face many barriers to engaging in timely ACP with their patients in routine clinical practice. It is important to note that ACP in cirrhosis care can be a shared responsibility among a multidisciplinary care team that may include primary care providers, social workers, palliative care clinicians, and other relevant subspecialists in additional to hepatology clinicians. The following suggestions are practical suggestions for hepatology clinicians who wish to integrate early ACP into the care of patients with advanced liver disease:

1. Communication Skills Training: High-quality communication is a critical component of ACP in cirrhosis care as highlighted in BPA statement #4 from the 2020 AGA Clinical Practice: “Across the spectrum of cirrhosis, excellence in communication is integral to high-quality advance care planning, goals of care conversations, and the cultivation of prognostic awareness with patients and caregivers” [37••]. Hepatology clinicians report not feeling comfortable or adept at having ACP discussions with patients and families [23••, 35]. Structured clinical communication training such as Vital Talk (www.vitaltalk.org), the Serious Illness Conversation Guide (www.ariadnelabs.org/areas-of-work/serious-illness-care), and the Veterans Affairs Life-Sustaining Treatment Decisions Initiative (https://www.ethics.va.gov/LSTDI.asp) can empower hepatology clinicians to engage in GOC discussions with patients and families and assess prognostic awareness and readiness. Outside of formal training, hepatology clinicians can also use frameworks for GOC conversations such as SPIKES (Setting, Perception, Invitation, Knowledge, Empathy/Emotions, Summarize) and REMAP (Reframe, Expect emotion, Map out patient goals, Align with goals, and Propose a plan) to guide difficult conversations [43, 45]. Brisebois et al. provided suggested conversation starters that can be used to guide ACP discussions with patients with advanced liver disease (Fig. 1) [41••].

2. Patient-Centered Educational Tools: Patients with advanced liver disease have reported a preference for patient-facing tools that improve their illness and prognostic understanding and engagement in ACP [25]. The PREPARE (www.prepareforyourcare.org/welcome) and National POLST Office (www.polst.org) websites provide educational brochures, videos, and toolkits for patients to guide patients and their families in identifying a surrogate decision-maker, expressing their medical care preferences, and completing an advance directive [46]. Use of a question prompt list, a recommended set of questions that patients and their family can review prior to an outpatient visit regarding their medical care, has been shown to improve communication among providers, patients, and families [47]. A question prompt list has recently been developed for patients with advanced liver disease and could provided to patients in the outpatient setting prior to a clinic appoint to empower them to express their unmet informational needs [48]. Use of visual aids such as the “Best case/Worst case” communication tool to provide anticipatory guidance for patients with advanced liver disease should be considered and has been shown to be effective in improving shared decision-making and ACP in patients with serious illnesses [41••, 49, 50].

3. Optimizing Outpatient Processes for ACP: Hepatology providers have reported limited time and reimbursement as important barriers for engaging in ACP in clinical practice [23••]. The use of a standardized ACP note template in one quality improvement study helped to streamline the process of ACP documentation in the electronic health record and led to improved completion of advanced directives for outpatients with advanced liver disease [51••]. To recognize ACP as an integral and valuable component of care, Medicare adopted new billing codes for ACP using Current Procedure Terminology (CPT) codes 99497 and 99498 to provide reimbursement to clinicians for this service [52].

4. Collaboration with Specialty Palliative Care: Specialty palliative care clinicians are experts in complex symptom management, care coordination, and communication. Collaborating with specialty palliative care services in the outpatient setting may be helpful to clarify patients’ goals and values early in their disease course. Early referral to specialty palliative care services has been shown to improve ACP documentation for patients undergoing liver transplantation evaluation [53]. Specialty palliative care clinicians may be particularly helpful in supporting hepatologists to navigate complicated cases through providing assistance in conflict resolution within families, managing emotional and spiritual distress, caregiver support, and hospice referral [54].

Figure 1.

Recommended conversation starters for advance care planning discussions with patients with advanced liver disease (Brisebois et al. Hepatology 2018)

Conclusion

Patients with advanced liver disease experience limited life expectancy and high healthcare utilization, making them vulnerable to poor quality of life and end-of-life care. Early integration of ACP into the management of patients with advanced liver disease may improve patient outcomes and ensure that they receive care that is consistent with their goals and values.

Abbreviations

ACP

Advance care planning

CPR

Cardiopulmonary resuscitation

GOC

Goals of care

ICU

Intensive care unit

MELD

Model for End-Stage Liver Disease