What Special Considerations Are Needed for Patients With Advanced Lung Disease?

Epidemiology and Illness Trajectories

COPD and IPF vary in prevalence, risk factors, typical age of onset, and disease trajectories, as summarized in Table 52.1 . There is also considerable variability in trajectories between and within each disease, with some patients progressing slowly through severity stages, others progressing rapidly, and some experiencing frequent and severe acute exacerbations. This variability introduces significant challenges for clinicians in knowing when to integrate palliative care.

COPD is the fourth leading global cause of death, with around 3.2 million deaths annually, and the third leading cause of global disability. In the United States, COPD is the fourth leading cause of mortality, with about 120,000 deaths annually, and rises to third among adults over 65 years of age. Approximately 16.4 million or 7% of Americans report a COPD diagnosis, but there is likely significant underdiagnosis due to limited awareness and availability of diagnostic modalities such as spirometry. Survival is varied and dependent on the stage of COPD, severity at diagnosis, and smoking status; some patients live for decades, while others die within 1 year of diagnosis. Other factors such as rural residence, weight loss, and presence of anxiety and depression also increase mortality risk.

The burden of COPD is projected to increase due to the rise in our aging population and exposure to COPD risk factors such as tobacco smoking and air pollution. It is estimated that over half of adults with COPD will be over 75 years of age by 2030. COPD tends to be higher in smokers compared to nonsmokers and in adults over 40 years of age, with prevalence increasing in later decades of life, and generally more prevalent in males than in females, although there is some evidence that trends in gender differences may be reversing. People with COPD have a history of risk factors including tobacco smoke, smoke from cooking or heating fuels, occupational exposures, genetic factors, or low birthweight.

People living with COPD experience breathlessness, chronic cough, and recurrent lower respiratory tract infections. Breathlessness is the most characteristic symptom and is generally progressive, worse with exertion, and persistent, and can be refractory to multiple different inhalers. As COPD progresses, people often develop fatigue, unintentional weight loss, and anxiety or depression, which can be associated with higher mortality. The clinical course of COPD is often long and punctuated by exacerbations, or periods of acute worsening of respiratory symptoms that often require antibiotics and steroids and may need hospitalization. Hospitalizations for exacerbations of COPD are generally treated with antibiotics, steroids, noninvasive ventilation, or invasive mechanical ventilation, and symptoms typically resolve in days. The risk of death following an acute exacerbation is increased, and as many as 1 in 4 adults with COPD may die in the year following these hospitalizations.

IPF, on the other hand, has a US prevalence of 100,000 with an incidence of 15,000 to 30,000 cases per year. A diagnosis of IPF carries a very poor prognosis, with a median survival of 2.8 years. IPF is more common in males and in people over 60 years of age, is rare in those younger than 50, and accounts for 47% to 64% of idiopathic interstitial lung diseases (ILD). Patients present either as an exacerbation or a more indolent presentation over the course of months to years, and symptoms include breathlessness, dry cough, fatigue, and unintentional weight loss.

The clinical course of IPF can have a slow progression, but exacerbations lead to a rapid deterioration in 5% to 10% of patients annually. These exacerbations may be thought of as sentinel events, and unlike in COPD, they are much more difficult to treat. Patients often require weeks in the hospital to recover and are placed on high levels of supplemental oxygen support, which complicates safe discharges and transitions of care. Mortality is high during and following an exacerbation of IPF, with an in-hospital mortality of up to 50% and, for those discharged, a median survival of 3 to 4 months.

Experiences Living With COPD and IPF

The experience of living with COPD and IPF from numerous qualitative studies illustrates significant burden that extends beyond refractory breathlessness into the wider toll of dealing with anxiety, depression, social isolation, loss of hope, and difficulty maintaining meaning, all while dealing with a limited understanding of the disease. Overall, patients with serious respiratory illnesses carry a symptom burden at least as severe as those with lung cancer but with less prognostic certainty about the illness course. Symptom burden at the time of palliative care consultation is similar when comparing those with serious respiratory illnesses and those with lung cancer. In the last year of life for those who died from COPD, almost half were admitted to the hospital at least 2 times, two-thirds knew they might die, and two-thirds died in the hospital. When compared to patients with lung cancer, those with COPD preferred comfort-focused care at similar rates but were treated with life-sustaining interventions more often, such as mechanical ventilation, cardiopulmonary resuscitation, and tube feeds, and many died in the intensive care unit (ICU).

People with COPD experience alternating good and bad days with symptom burden and may not necessarily attribute exacerbations to progression of disease but instead as temporary setbacks. Given the difficulty with prognostication in COPD, clinicians often feel ill-equipped to discuss difficult topics regarding end-of-life, and even when they do, patients have limited understanding. Patients may gain a better understanding of prognosis when examples that are relevant to their lives are used. For instance, explaining that COPD may lead to difficulty walking around the house or playing with grandchildren has a greater impact than explaining that the disease will reduce life expectancy by 10 years.

Breathlessness in COPD is the most pervasive and debilitating symptom present in all aspects of a person’s life, impacting activities such as toileting and bathing and leading to distress. Patients also become frustrated by the limitations of fatigue, described as unrelenting tiredness and exhaustion, that becomes a barrier to social participation. The psychological impact due to respiratory symptoms leads to anxiety over fear of breathlessness and coughing, distress that they will not wake up the next morning, and social isolation, which creates a loss of hope, meaning, and connectedness. Toward the end of life, patients often struggle with restrictions in their mobility and other aspects of life, while dealing with increasing vulnerability, unpredictable setbacks, and daily symptom burden.

For people living with IPF, the median time from initial presentation of breathlessness, cough, or fatigue to diagnosis can be 1.5 years but sometimes takes up to 12 years with multiple health care visits and “misdiagnoses” of COPD or asthma. Even after diagnosis, the majority of patients and care partners report a lack of information and resources about IPF, especially regarding benefits of pulmonary rehabilitation, managing high doses of supplemental oxygen, and the risks and benefits of lung transplantation. As with COPD, breathlessness and a dry cough that can range from being an annoyance to being unpredictable and leaving the patient exhausted will eventually impact every aspect of life in those with IPF. Even simple activities, such as watching television, require significant effort. As in COPD, patients also struggle with anxiety or depressive symptoms and live with a constant uncertainty of the illness trajectory. Beyond symptom burden, IPF leads to decreased functional independence due to inability to perform daily tasks and oftentimes significant oxygen requirements, limitations in relationships given isolation and perception of their burden on family and society, and increased financial stress from health care costs and loss of ability to work. Patients and family caregivers develop constant awareness of symptoms and progression, fearing when an overwhelming coughing attack may be fatal, with many caregivers feeling insecure and alone due to the responsibility. Long-term oxygen use is seen as a sign of disease progression and deterioration, and while it provides relief for breathlessness there is still the limitation of having a tether.

Symptom Management