Palliative care in interstitial lung disease: living well

Summary

Progressive fibrotic interstitial lung diseases (ILDs) are characterised by major reductions in quality of life and survival and have similarities to certain malignancies. However, palliative care expertise is conspicuously inaccessible to many patients with ILD. Unmet patient and caregiver needs include effective pharmacological and psychosocial interventions to improve quality of life throughout the disease course, sensitive advanced care planning, and timely patient-centred end-of-life care. The incorrect perception that palliative care is synonymous with end-of-life care, with no role earlier in the course of ILD, has created a culture of neglect. Interventions that aim to improve life expectancy are often prioritised without rigorous assessment of the individual's health and psychosocial needs, thereby inadvertently reducing quality of life. As in malignant disorders, radical interventions to slow disease progression and palliative measures to improve quality of life should both be prioritised. Efficient patient-centred models of palliative care must be validated, taking into account religious and cultural differences, as well as variability of resources. Effective implementation of palliative care for ILD will require multidisciplinary participation from clinicians, specialist nurses, psychologists, social workers, and, in some countries, non-governmental faith and community-based organisations with access to palliative care expertise.

Introduction

Chronic interstitial lung disease (ILD) is a heterogeneous group of mainly fibrotic diseases including idiopathic pulmonary fibrosis (IPF), sarcoidosis, hypersensitivity pneumonitis, and connective tissue disease-associated ILD. Although often perceived as rare, ILD is the 40th most common cause of death globally,¹ with a 52% increase in mortality during the past 10 years. Despite therapeutic advances, a cure is usually unattainable in fibrotic ILD and only a minority of patients meet eligibility criteria for lung transplantation. IPF, the most prevalent fibrotic ILD, has a worse outcome than most malignancies, with an average survival of roughly 3 years from diagnosis.² Fibrotic ILDs are characterised by cough, fatigue, anxiety and depression, and increasingly disabling dyspnoea, ultimately requiring supplemental oxygen. The psychosocial burden of IPF is substantial for both patients and caregivers.³ As the disease advances, the need for support often increases exponentially.

Patients with ILD need timely diagnosis, comprehensive information, early treatment access, and holistic and palliative care (figure 1), as advocated by patients and their treating physicians in the European IPF Charter.⁴ Given that the needs of patients and caregivers in progressive ILD are strikingly similar to those in malignant disease, it is surprising that palliative care in ILD remains in its infancy.

In view of the absence of clear national and international guidelines on palliative care in ILD, we established an international group working in different health services and political and socioeconomic environments made up of ILD specialists, palliative care specialists, ILD nurses, a patient, and a caregiver, from various cultural and religious backgrounds. The working group identified important topics regarding palliative care in patients with ILD and a consensus statement was generated.

Here, we discuss the urgent need for the involvement of palliative care in ILD, both earlier in the disease course and in end-stage disease. We share our perceptions of the most important questions of palliative care in ILD, and consider the religious and cultural differences that might affect decisions around palliative care, obstacles to implementing a palliative approach to care, and future research considerations.

Key messages

• The main goal of palliative care for patients with interstitial lung disease (ILD) is to improve and maintain quality of life

• Palliation is holistic and includes all aspects of patient care in ILD

• A disease behaviour-based strategy might be helpful in identifying the patients' need for palliation because of the unpredictable course of most fibrotic ILDs

• Assessment of needs is an iterative and dynamic process throughout the disease course in ILD

• Training of ILD teams on palliative care and of palliative care teams on ILD must be improved

• Patients and caregivers should be realistically educated on all aspects of the disease from the moment of diagnosis depending on the individual patients' needs and preferences

• Palliation should be discussed and initiated early in the disease course depending on the individual patients' needs, preferences, culture, and religion

• Caregivers should be supported throughout the disease trajectory and bereavement

• Advanced care planning, including discussion of treatment limitations and preferences on dying should be offered to all patients with ILD in whom it is evident or likely that the disease will be progressive despite treatment

• Further research is needed to identify the most appropriate models for palliative care and discussions with patients, possible markers to guide care, and suitable outcome measures for clinical trials of palliative care for ILD