Chronic Illness

All patients diagnosed with progressive ILD share problems common to those with a chronic illness. Chronic illness, a permanently altered health state, results from a nonreversible pathologic condition. The consequence is a residual disability that cannot be corrected by a surgical procedure or cured by medical therapy. The diagnosis of a chronic illness can produce an emotional response, sometimes in a manner disproportionate to the extent of physical disability and major life changes. Major challenges include coping with progression of the disease and the associated disability, which vary across patients and diagnoses, and attempting to best maintain quality of life.

Idiopathic Pulmonary Fibrosis

IPF is one of the most common, relentlessly progressive, and best-studied ILDs regarding symptom burden and perceived benefits and barriers to use of palliative care (PC). Therefore, the focus of this chapter will be on use of PC in IPF.

IPF is a progressive life-limiting lung disease that affects ∼128,000 newly diagnosed individuals in the United States annually. IPF, a disease of aging associated with intense medical and financial burden, is expected to grow in incidence within the US population. The prognosis is poor. Median survival from diagnosis is 3.8 years, with some patients succumbing to a rapid death within 6 months. New therapies have recently become available. These medications slow the rate of deterioration of lung function, but have no proven impact on ultimate survival or quality of life. Although transplantation is an effective surgical therapy, less than 20% of patients ever receive a lung transplant. The remaining 80% have few treatment options and are predicted to experience a progressive downhill course. Patients report great symptom burden and impaired quality of life. As fibrosis advances and lung function deteriorates, patients experience a progressive increase in shortness of breath, cough, and fatigue. These symptoms are distressing to patients and family caregivers and present a challenge in maintaining quality of life as the disease relentlessly progresses. Despite the fatal prognosis, patients and caregivers often fail to understand the poor prognosis.

Palliative Care—Goals and Timing

PC is a comprehensive treatment of the discomfort, symptoms, and stress of serious illness and should be offered at the time of diagnosis of a serious illness ( Fig. 8.1 ).

Conceptual framework for the role of palliative care in the course of chronic illness.

Adapted from Dahlin C. Clinical Practice Guidelines for Quality Palliative Care On behalf of the National Consensus Project for Quality Palliative Care Task Force. National Consensus Project for Quality Palliative Care . 2009, with permission.

PC involves management of symptoms, which is relevant even in patients with mild to moderate disease. Patients receive emotional and spiritual support focused on enabling them to live better with the consequences of an incurable illness. PC can be a means to assist patients and families through the process of reflection, discussion, and communication of treatment preferences for end-of-life (EOL) care. This process, termed advance care planning, is “a more deliberate, organized, and ongoing process of communication to help an individual identify, reflect upon, discuss, and articulate values, beliefs, goals, and priorities to guide personal care decisions up to and including EOL care.” The mantra “It is wise to hope for and expect the best, but it is also wise to prepare for the worst” is a way to introduce advance care planning to patients with IPF and their caregivers.

PC can be delivered by an interdisciplinary team, referred to as specialty PC, or a member of the clinical care team, referred to as primary PC. PC practitioners are trained in conducting discussions regarding EOL planning and, therefore, may be helpful in initiating and facilitating such discussions. Often being confused with hospice ( Table 8.1 ), PC has different goals. Because PC focuses on assisting patients/family caregivers to better manage symptoms associated with disease progression, primary and/or specialty PC should ideally occur early following diagnosis. For patients with IPF, this is particularly important, as disease progression is difficult to predict.

Consensus guidelines for implementation of PC have been developed through the work of several professional organizations. The National Consensus Project for Quality Palliative Care evolved from the work of four PC organizations: the American Academy of Hospice and Palliative Medicine; Center to Advance Palliative Care; Hospice and Palliative Nurses Association; and the National Hospice and Palliative Care Organization. The mission of the National Consensus Project for Quality Palliative Care was to create guidelines that improved the quality of PC in the United States. Stakeholders from these organizations developed clinical practice guidelines that cover the eight domains identified as being crucial to the delivery of comprehensive PC ( Box 8.1 ). The goal is to promote quality PC, foster consistent and high standards in delivery of PC, and encourage continuity of care across settings.

Since introduction to the United States, PC has grown rapidly. Referral to PC has become an integral part of the care of patients with malignant disease and is commonly part of the care of critically ill patients because of high risk for mortality. In this setting, referral has the additional benefit of providing support to family caregivers who are asked to serve as surrogate decision-makers.

Palliative Care Versus Hospice

In 1967, Dame Cicely Saunders (who trained as a nurse, a social worker, and a physician) founded the St. Christopher’s Hospice in London, the first inpatient, home care, research, and teaching hospice. The hospice movement came to the United States in the mid-1970s, when Dr. Florence Wald, a nursing pioneer, led an interdisciplinary team to create the first American hospice. Hospice care became a Medicare benefit in the United States in the 1980s. While both PC and hospice share the same principles of providing comfort and support for patients, hospice is designated for patients with a life expectancy of 6 months or less certified by a physician. An important difference between hospice and PC relates to the use of life-prolonging therapy. This is not allowed as part of hospice, but is fully permitted as part of PC ( Table 8.1 ).

Palliative Care in Patients With Malignant Disease

In patients with metastatic lung cancer, early and integrated PC, with a focus on communication regarding disease severity and treatment choices, has been found to improve quality of life, reduce symptom burden, and improve survival in comparison to usual care. In one study comparing healthcare utilization and quality of care for patients with cancer who received early versus late PC, early PC was associated with less intensive medical care, improved quality outcomes, and cost savings at EOL. Blackhall et al. measured timing of referral to outpatient PC and impact on EOL care in patients with advanced cancer and found that referral to outpatient patient care occurred in approximately half of patients with incurable cancer. Those patients referred had improved EOL care and reduced costs with fewer hospitalizations at the EOL and were less likely to die in hospital.

Despite these positive findings, studies continue to indicate unmet needs. A 2016 survey conducted in the United States that enrolled a diverse sample of patients diagnosed with cancer in terms of ethnicity, income, education, geography, age, insurance, cancer type, and treatment stage revealed serious communication problems that compromised their EOL care. The findings of the survey entitled the CancerCare Patient Access and Engagement Report included (1) less than half of respondents said their care team knew their EOL wishes; (2) approximately one-third or less of the respondents said they felt adequately informed about other treatment options their care team considered, the responsibilities of their caregivers, and clinical trial opportunities; and (3) between 22% and 37% of respondents said that additional information about PC, living wills, or hospice care would have been helpful to them.

Palliative Care in Patients With Advanced Lung Disease

Despite higher symptom burden, worse quality of life, and more social isolation, patients with chronic lung disease have been found to receive PC less likely than patients with cancer. In a study of 2400 deaths in patients with chronic illness, Beernaert and colleagues reported that patients with chronic obstructive pulmonary disease (COPD) (20%), heart failure (34%), and severe dementia (37%) were less likely to receive a PC referral than patients diagnosed with cancer (60%). This and other studies reinforce the conclusions that discussion of PC by clinicians who manage the care of patients with advanced lung diseases, such as COPD, occurs less frequently than for other life-limiting conditions, such as cancer. Brown and colleagues in an opinion editorial posed several reasons for fewer referrals. These included uncertainty in prognosis, lack of provider skill to engage in discussions about PC, fear of using opioids among patients with chronic lung disease, fear of diminishing hope, and perceived and implicit bias against patients with smoking-related lung disease.

Supporting Research

Prior studies support that, as in other advanced lung diseases, referral to PC occurs infrequently and late in patients with IPF. In a retrospective study, Lindell and colleagues identified the location of death and frequency of referral to PC for patients with IPF managed at a specialty lung center from 2001 to 2012. Of 277 IPF decedents whose location of death could be determined, over half died in the hospital (57%). Only 38 (13.7%) had a formal PC referral. Most referrals (71%) occurred within 1 month of death, often after admission to the intensive care unit (ICU). The most common reason for referral was “to discuss goals of care.” From a study of 45 patients with IPF, Bajwah and colleagues reported the majority (76%) died in a hospital setting and a minority (38%) had PC team involvement, supporting limited referral.

To elicit perspectives of patients and family caregivers, Lindell conducted focus groups consisting of patients currently living with the disease, caregivers for patients living with the disease, and caregivers of a deceased family member. Findings revealed overwhelming symptom burden, with cough being a particularly debilitating symptom, frustration with the need to use oxygen therapy, and worry about finances and the cost of medications. Participants also verbalized hesitance to engage in advance care planning, which was recognized as a need, but avoided because it was perceived as loss of hope. Caregivers of the patients living with the disease were vocal about “only wanting positive options,” including research participation opportunities. Patients were not aware of the scope of support offered through PC, for example, reducing symptom burden. In addition, there was confusion with hospice. Family members of decedents were more likely to receive a formal PC referral, suggesting clinician reluctance to suggest this option early in the disease course. This outcome, while logical, can be counterproductive as symptoms of IPF may escalate rapidly, resulting in acute respiratory failure and the need to make EOL care decisions at the time of critical illness.

Several additional qualitative studies have attempted to identify reasons for hesitancy in initiating referral to PC. Bajwah and colleagues reported findings from a survey of 124 general practitioners that attempted to elicit barriers to implementation. Respondents viewed PC services positively, but felt the support the care provided was sufficient and the referral was not indicated. To gain further understanding, Bajwah and colleagues found in a qualitative study of 18 patients with fibrotic ILD and their informal caregivers and health professionals that patients experience substantial distress from the impact of their symptoms and psychosocial needs were often not being met. Health professionals had varied knowledge and confidence in managing these concerns and tended to underestimate patient concerns.

Findings from this study prompted the authors to develop an educational plan using a case conference approach entitled Hospital2Home . In a randomized control trial of 53 patients with advanced fibrotic lung disease, 26 of whom were randomized to receive the intervention, findings supported benefits. A PC specialist nurse delivered the intervention in patient homes. Prior to the conference, the nurse telephoned the patient and carer to identify current concerns and goals for the visit. Patients related physical, emotional, social, spiritual, and EOL concerns. A mutual action plan was developed with telephone follow-up at 2 weeks, 1 month, and 2 months, and as needed. Patients reported that the sessions improved their ability to manage their symptoms and quality of life, demonstrating this type of intervention can improve patient care.

Sampson and colleagues conducted a qualitative study of IPF patients and their caregivers to explore their perspectives across the IPF spectrum and inform the development of clinical pathways and multidisciplinary service interventions. Because the nature of disease progression in IPF is uncertain, specialists in PC and ILD generated a disease typology to classify patients into four stages related to their disease progression. The intervention focused on using clinical visits to identify changes in health status and functional activity, patient understanding of symptoms, interventions to improve symptoms, coping strategies, and caregiver roles. Three important areas for improvement were identified: (1) including a focus on supportive interventions and symptom self-management in discussion during clinic visits, (2) recognition of importance of family caregivers, and (3) appreciation of differences in patients and caregivers regarding need for information and change in needs over time. Findings revealed that patients initially had a clear understanding of their prognosis, but were uncertain about how their disease would progress and be managed. This study is important as it addresses ways to incorporate concepts of PC into the clinical care continuum.

Caregiver Perspective

Decades of research have identified the stress experienced by caregivers of patients with high care needs. Patient suffering, whether physical, psychosocial, or spiritual, has been found to have a major impact on family caregivers. Family caregivers are essential to the well-being of patients with serious illness In testing an interdisciplinary PC intervention in family caregivers in lung cancer, caregivers experienced improvement in social well-being and psychological distress and less caregiver burden after participating in interdisciplinary care meetings and receiving four educational sessions.

Caregivers of patients with IPF are a unique and critical population in which to study approaches for improving the quality of PC because of their critical role in physical and emotional assistance. In testing a disease management intervention that included a session on advance care planning, Lindell found the content reduced perceived stress among caregivers who participated in the study. Belkin and colleagues conducted a study capturing caregiver perspectives on the effects of IPF and reported that caregivers experienced hardships throughout the disease course, including dealing with their own emotional issues and the patient’s physical limitations, which were increased by the need to use supplemental oxygen.

Critical Care Perspective

The Study to Understand Prognoses and Preferences for Outcomes and Risks of Treatment (SUPPORT) conducted in the early 1990s produced findings that prompted the discussion of the need for more proactive measures to improve quality of care for seriously ill and dying patients. As a result, hospitals created PC service to provide specialty consult services to address symptom burden and quality of life. Team PC is now available in more than 70% of US hospitals with at least 50 beds.

To evaluate impact, Roczen and colleagues conducted a review of 12 studies evaluating the effects of PC programs in ICUs from 2000 to 2013. Common findings included that PC interventions did not necessarily change hospital or ICU mortality, but length of stay was often significantly reduced, especially ICU length of stay. Nevertheless, evidence continues to suggest that for patients with advanced lung disease, including those with IPF, referral is infrequent. From a study that examined medical record documentation of PC in patients admitted to 15 hospitals, Brown and colleagues reported that, compared with patients with metastatic cancer, patients with ILD, including those with IPF, and COPD who died in an ICU were less likely to have documentation of a discussion of their prognosis or a “do not resuscitate” order at the time of their death. This is concerning because the onset of acute respiratory failure in patients with IPF is associated with extremely high mortality.

Lung Transplant Perspective

Lung transplantation remains the only treatment that has the potential to extend life for patients with IPF. A significant percentage of patients hospitalized with advanced lung fibrosis or an acute exacerbation of their ILD will develop respiratory failure. In the absence of discussion of advance care planning, the consequence of this outcome may be admission to the ICU and mechanical ventilation. If so, mortality is extremely high and few patients survive to ICU discharge. Even fewer survive months and there are almost no survivors at 12 months. The need for ICU admission and mechanical ventilation typically precludes selection for transplant because of time constraints involved in the evaluation process, organ availability, and risk for complications during the ICU. Therefore, it is advisable to introduce the potential of evaluation for transplant early following diagnosis. A book, written by a transplant recipient and entitled Partners for Life, provides a patient’s perspective of this journey and can be of assistance in answering patients’ questions.

PC is often not prescribed for lung transplant candidates. Barriers include fear that referral will signal abandonment by the transplant team and concern over opioid use. Colman and colleagues did a retrospective case series reviewing outcomes of 64 lung transplant candidates comanaged by PC. There were no episodes of opioid toxicity or respiratory depression, and they concluded that PC could be an important means to reduce symptom burden in lung transplant candidates.