Early and Integrated Palliative Care to Achieve a Home Death in Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia characterized by progressive fibrosis. Chronic refractory breathlessness is the clinical hallmark and the most debilitating symptom of IPF; it is strongly associated with poor quality of life and mortality. Dyspnea in IPF remains grossly undertreated. The current IPF treatment guidelines recommend palliative care referral and a symptom-based approach as an adjunct to disease-focused care. However, it is well recognized that most IPF patients do not receive palliative care during the course of their illness or at end of life. As a result, these patients suffer from not only a poor quality of life but also a poor quality of dying and death. Although most patients express the preference for a home death, unfortunately the majority die in hospitals, including intensive care units. In fact, outside of a clinical feasibility trial setting, home deaths have not been described in the literature, which quotes 100% of deaths occurring in acute care. Most patients and families believe that their symptoms at the end of life will be managed more effectively in hospital. Concurrently, respiratory specialists are not familiar with facilitating patient care and management in the home or to providing palliation of their symptoms at home. In addition, it is generally believed that patients with end-stage lung disease are bed bound. To our knowledge, there are limited published reports describing an approach that allows patients with IPF to die at home without being bed bound. We describe a case where patient-centered multidisciplinary care provided early integrated palliative care and supported a very active end-of-life experience for a patient with IPF, including dying at home which