Keywords

“I’m Out of Breath”

This phrase, commonly voiced by a patient to a clinician, demands pause and deep exploration. These words often begin a conversation that is fraught with challenges for both the clinician and the suffering patient. Assessing the patient’s concerns, diagnosing the etiology of the complaint, developing a care plan, and providing ongoing support through the continuum of what may turn out to be a severe and fatal respiratory condition can be difficult for even the most expert practitioner. With these daunting challenges of serious pulmonary disease, both patients and clinicians can find ways to “breathe easier” through the integration of palliative care into pulmonary medicine.

Respiratory symptoms can be caused by a wide variety of physical, mental, social, and environmental factors [1,2,3,4]. Primary pulmonary problems, extrapulmonary diseases that directly or indirectly impact the lungs and airways, as well as psychological conditions can all lead to complaints of trouble breathing. In the most benign cases, “running out of breath” represents a limitation to otherwise healthy physical exertion, a signal that despite normal physiology, someone is not able to reach the level of functioning that they desire and deem necessary for quality of life. In the most severe cases, being truly without breath is followed shortly by being without life. Pulmonary disease is for many a constant reminder of the possibility of death.

Respiratory disease and the end of life are healthcare experiences that are universally a part of the human condition. While virtually everyone has had a minor upper respiratory infection with mild respiratory symptoms, others develop advanced pulmonary diseases with severe symptoms that impact quality of life and ultimately limit one’s life span. Heart disease, cancer, chronic lower respiratory disease, and respiratory infection comprise four of the top ten causes of death in the United States [5], and all cause respiratory symptoms. Worldwide, primary respiratory tract cancers and tuberculosis also enter the top ten causes of death, extending the prevalence of chronic respiratory concerns around the globe [6]. While modern medicine has been able to reduce morbidity and mortality in many respiratory conditions, the COVID-19 pandemic has presented yet another pulmonary disease challenge worldwide. Whether caring for a patient in the clinic or at the ICU bedside, the combined efforts of those trained in both pulmonary medicine and palliative care can improve both quality of life and the quality of death.

Palliative care has evolved as a model of health care focused on quality of life concerns in serious illness [7]. It builds on the foundation of hospice care, focused on the end of life, and extends the principles of this care across the trajectory of disease from the time of diagnosis. Palliative care began with a focus on cancer and late-stage disease, but it has rapidly expanded to include chronic illnesses such as heart failure, renal disease, neurological diseases, and dementia. As described below, there are many characteristics of pulmonary disease that make it an ideal target for palliative care, with the ultimate aim of improving quality of life for patients and families [8,9,10].

As palliative care has developed and expanded its focus across many diseases, there are common characteristics identified, or key domains that apply across serious illnesses. These are reflected in the National Consensus Project for Quality Palliative Care, Clinical Practice Guidelines [7]. These guidelines, now in the fourth edition, have guided the field through the eight domains of care, including Structure and Processes of Care; Physical, Psychological and Psychiatric, Social, and Spiritual, Religious, and Existential Aspects of Care; Cultural Aspects of Care; Care of the Patient Nearing the End of Life; and Ethical and Legal Aspects of Care [7].

Table 1.1 depicts these domains with application to pulmonary disease. There are many features of pulmonary disease that make these domains of care even more relevant. Pulmonary diseases are associated with high symptom burden, with often severe respiratory symptoms but also many other associated physical symptoms such as fatigue and cachexia. Psychological symptoms are also common including anxiety and depression. A common feature of these patients is concurrent chronic illnesses such as cardiac disease, adding to the symptom burden and quality of life impact.

Table 1.1 Palliative care domains applied to care of patients with pulmonary disease
Full size table

The guideline domain of social aspects of care is very relevant given the family caregiver demands in pulmonary disease, including managing multiple respiratory treatments and medications, oxygen, and care by multiple clinicians. The Spiritual domain is important for patients with severe illness, as they face their mortality and often reflect on religious, spiritual, or existential concerns.

The Cultural domain, described in more detail later in this chapter, is increasingly important as the population becomes more diverse and as clinicians recognize the influence of cultural factors on patient and family values, beliefs, and healthcare decisions.

The seventh domain, Care of the Patient Nearing the End of Life, has important implications for patients with pulmonary disease. Extensive literature has documented issues central to this population such as ventilator withdrawal, control of dyspnea in the final hours, and the many issues specific to care in the ICU setting [1,2,3,4].

The final domain, Ethical and Legal Aspects of Care, is relevant in all serious illnesses but also particularly relevant in pulmonary disease where conflicts often arise related to withdrawal of life support, lung transplant decisions, code status, or chronic respiratory management.

In the chapters to come in this text, the reader can apply these domains through the individual patient’s clinical course: from initial assessment, to diagnosis, to symptom management, through the end of life. The concluding chapters of this book expand these domains from the individual level to broader societal impact and healthcare policy, including future directions, and contemporary challenges, including the COVID-19 pandemic.

“I Need Air”: Palliative Care Assessment in Lung Disease

The initial evaluation of a patient with respiratory symptoms can be a daunting task. The American Thoracic Society defines dyspnea as “a term used to characterize a subjective experience of breathing discomfort that is comprised of qualitatively distinct sensations that vary in intensity. The experience derives from interactions among multiple physiological, psychological, social, and environmental factors, and may induce secondary physiological and behavioral responses” [11]. Similarly, other common symptoms of respiratory disease including cough, fatigue, pain, depression, anxiety, insomnia, and anorexia can each have multifactorial causes, with complex exacerbating and alleviating factors. In 1 study of 85 patients presenting to a pulmonary unit with a complaint of chronic dyspnea, the initial impression of the etiology of dyspnea based upon the patient history alone was correct in only 66 percent of cases [12]. Classically, the primary care or pulmonary clinician will use history, physical exam, laboratory results, imaging studies, and cardiopulmonary function tests to reach an initial diagnosis and then determine a disease-focused treatment plan. As symptoms progress, clinicians struggle further to understand refractory symptoms and prognosticate heterogenous diseases. Integrating palliative care into pulmonary disease assessment from initial presentation through the course of illness can help both the patient and clinician through this challenging process. Chapters 1, 2, 3, 4, 5, 6, and 7 of this textbook provide an outstanding resource for navigating patient symptoms, needs, and outcomes, with tools to evaluate and improve quality of life for patients and families with lung disease.

Integrating palliative care consultation also provides an interdisciplinary, whole person assessment. The initial evaluation by a palliative care social worker or nurse may add tremendous insight about the patient and family needs. One strong point of agreement across sources is the critical need to implement palliative care early in the course of the disease [3, 13,14,15,16].

“From One Breath to the Next”: Palliative Care Approaches to Diverse Pulmonary Diseases

Practitioners in pulmonary medicine encounter many patients with similar initial presentations but ultimately different diagnoses and courses of care. Pulmonary diseases are often divided into broad categories such as obstructive lung disease, restrictive lung disease, pulmonary vascular disease, and lung cancer, with unique pathophysiologic processes defining distinct pulmonary diagnoses within each group. Clinicians can use these diagnostic categories to not only guide disease and treatment but also symptom management, communication, and advanced care planning within specific patient populations. The disease trajectory of chronic lung diseases is often uncertain. For example, patients with chronic obstructive pulmonary disease (COPD) usually decline gradually, whereas patients with pulmonary fibrosis have an unpredictable disease course with median survival less than 4 years. For both patients, the decline can be more rapid if the patient’s underlying lung disease worsens or if the patient has other comorbid conditions. In the SUPPORT, data showed that 5 days prior to death, patients with lung cancer were predicted to have a <10% chance of surviving for 6 months while patients with COPD were predicted to have a >50% chance of this survival [17, 18].

Chapters 8, 9, 10, 11, 12, 13, 14, 15, and 16 of this textbook focus on the unique needs within special chronic lung disease patient populations, including those with COPD, interstitial lung disease, lung cancer, neuromuscular disease, pulmonary arterial hypertension, and pediatric pulmonary disease. While these diseases vary in many aspects, the domains of palliative care apply as they reflect universal concerns. Pulmonary diseases are also, in many ways, similar to other serious illnesses such as heart failure, end-stage renal disease, advanced cancer, or late-stage dementia. There are also unique aspects of lung disease including the profound impact of lung diseases on patient function and QOL. People living with lung disease also face social and cultural issues such as the blame associated with smoking-related diseases and the isolation often imposed on those with oxygen dependence.

“Clearing the Air”: Contemporary Challenges and Future Directions for Palliative Care in Pulmonary Disease

The twenty-first century has brought astounding advances as well as staggering challenges to pulmonary disease care. Advances in pharmacologic therapies for lung cancer, idiopathic pulmonary fibrosis, and pulmonary hypertension have impacted patient experiences with varied outcomes. Non-small cell lung cancer mortality in men decreased by 6.3% annually from 2013 through 2016, while lung cancer-specific survival increased from 26% for men diagnosed in 2001 compared to 35% who were diagnosed in 2014 [19]. This analysis based on SEER data showed improvement in survival across racial and ethnic groups and was thought to be in large part due to the development of targeted therapies.

Idiopathic pulmonary fibrosis, a subtype of interstitial lung disease, has a notoriously poor median survival of 3 years. The development of two new anti-fibrotic medications, pirfenidone and nintedanib, both approved by the US FDA in 2014 for treatment of idiopathic pulmonary fibrosis, has been shown to slow progression of the disease, though has not shown an improvement in mortality [17, 20].

Pulmonary arterial hypertension research has led to the development of multiple new medications targeting three pathways of the disease [21]. However, patients continue to have progression of disease with significant symptom burden and high rates of healthcare resource utilization. Despite numerous advances in basic science and clinical pulmonary medicine over the last two decades, there remains a need for improved patient care through focus on quality of life, symptom management, and advanced care planning which palliative care can provide.

Furthermore, pulmonary diseases are more prevalent in populations with decreased access to health care and increased environmental exposures contributing to disease. Care is increasingly provided to an aging population, in outpatient settings, by telehealth and care is dependent on family caregivers. Many of these family caregivers are also elderly with their own chronic illnesses, often with pulmonary diseases. Healthcare systems are keenly interested in models of care which can increase patient and family satisfaction, reduce hospital admissions, and provide the most cost-efficient care [22, 23]. As is true with all serious illnesses, there is a need to devote attention to underserved communities and minority populations who are especially vulnerable to inadequate care, late diagnosis, and limited access to supportive care [23].

The year 2020 has brought issues of public health crisis, healthcare justice, and racial disparities to the forefront of pulmonary disease. In December 2019 the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was first recognized in Wuhan, China. By January 2020, the virus was declared a public health emergency of international concern by the WHO, and in March 2020 it was recognized as a pandemic. As workgroups came together to diagnose and manage the profound pulmonary disease associated with this virus, palliative care practitioners assumed a central role in helping with bedside patient care, remote family communication, and end-of-life preparation. Key tenets of palliative care, including advanced care planning to ensure goal-concordant care, decision-making regarding life-prolonging treatments, and ethical/legal issues regarding chronic ventilator support, have been essential in the management of critically ill patients with COVID-19 infection [24].

Furthermore, palliative care team members have brought important skills to aid healthcare system management, including ethical distribution of healthcare resources, and use of alternative care environments outside of traditional healthcare settings such as home care. The COVID-19 pandemic has been perhaps the greatest healthcare challenge in a century; that challenge has been met at the front lines by both pulmonary medicine and palliative care providers working side by side.

During this time of pandemic, a domestic crisis also occurred. On May 25, 2020, a video captured an African-American man, George Floyd, and his words “I can’t breathe” shortly before his death in police custody. His death ignited an international Black Lives Matter movement, not only focusing on racial injustice involving police brutality but also extending the discussion of racial and ethnic inequalities throughout society. Advocacy groups including White Coats for Black Lives have highlighted the passion that healthcare providers have in recognizing and addressing racism, inequality, and diversity in health care. Palliative care practitioners bring a strong foundation focused on social, cultural, and spiritual aspects of health care. Chapters 16, 17, and 18 of this textbook discuss multidisciplinary models and healthcare policies for improving palliative care in advanced lung disease through the current pandemic and beyond. Pulmonary disease, as all other chronic illnesses, is greatly impacted by social factors and changes in healthcare delivery.

“Breathing Easier”: A Collaborative Future for Palliative Care in Lung Disease

The field of pulmonary medicine continues to make significant advances in diagnosing, managing, and treating lung disease. Similarly, the field of palliative care has a growing evidence base demonstrating effectiveness in symptom management, psychosocial and spiritual support, as well as enhanced survival [25,26,27,28] and cost implications of aggressive care at the end of life [24, 29, 30]. This textbook could not be timelier in bringing together the fields of pulmonary disease and palliative care. The chapters in the text address the key experiences of patients living with a broad range of pulmonary diseases and their daily challenges of breathlessness, anxiety, diminished function, and uncertainty, from initial assessment through end of life. The chapters represent common factors across pulmonary diseases, as well as those unique to specific diagnosis of COPD, lung cancer, interstitial lung disease (ILD), pulmonary hypertension (PH), and many others.

There is an expanding body of literature specifically addressing the benefits of palliative care in pulmonary disease [31,32,33,34,35,36,37], including better symptom management [38]. Extensive additional work is needed to test models of palliative care delivery across pulmonary diseases [2, 4, 39,40,41,42,43].