Palliative Care in Pulmonary Fibrosis (PULFIP)

Effect of Palliative Care in Patients With End Stage Pulmonary Fibrosis: a Randomized Control Pilot Study

The investigators will examine the effect of introducing palliative in patients with end-stage idiopathic pulmonary fibrosis, refractory to the pharmacological treatment or not deemed to be treated, on patient-reported outcomes and end-of-life care. The investigators will randomly assign patients who receive either early palliative care integrated with standard respiratory care or standard respiratory care alone. Quality of life and symptoms will assessed at baseline and at 12 weeks The primary outcome will be the change in the quality of life and symptoms at 12 months.

Advanced fibrosing interstitial lung disease (ILD) is a group of often progressive and incurable conditions. The most common form of ILD, idiopathic pulmonary fibrosis (IPF), is associated with poor survival, and high symptom burden and poor quality of life as the disease progresses. ILD represents an increasing proportion of patients with chronic hypoxemic respiratory failure. Despite this poor prognosis, palliative care remains underused in patients with ILD. This may be due to under-recognition of the palliative care needs and symptom burden, or unfamiliarity and discomfort with palliative therapies. Though oncology has largely embraced earlier integration of palliative care, which has translated into improvements in end-of-life (EOL) care for patients with lung cancer,palliative and EOL care for non-malignant diseases are now gaining increased attention. In pulmonary disease, this research has mainly focused on COPD and demonstrated a significant burden of unmet palliative care needs and lower quality of EOL care compared with patients with cancer. Similar attention is only beginning to be paid to ILD, but symptom burden and quality of EOL care in patients with ILD have yet to be quantified, and furthermore none has so far investigated the possible role of palliative care in these patients. The investigators will examine with the present study the effect of introducing palliative in patients with end-stage idiopathic pulmonary fibrosis, refractory to the pharmacological treatment or not deemed to be treated, on patient-reported outcomes and end-of-life care. The investigators will randomly assign patients who receive either early palliative care integrated with standard respiratory care or standard respiratory care alone. Quality of life and symptoms will assessed at baseline and at 12 weeks The primary outcome will be the change in the quality of life and symptoms at 12 months.

The patients will receive the usual respiratory care that included both the "classical" treatments with antifibrotic drugs and oxygen therapy

The patients will receive the usual respiratory care that included both the "classical" treatments with antifibrotic drugs and oxygen therapy PLUS a palliative care program that includes paid to assessing physical and psychosocial symptoms

Inclusion Criteria: diagnosis of idiophatic pulmonary fibrosis resting partial arterial oxygen pressure (PaO2) < 60 mmHg Decline in Forced Vital Capacity (FVC) > 10% in the last 6 months stage 3 according to the GAP index Exclusion Criteria: active treatment with antifibrotic drug concomitant cancer

Bassi I, Guerrieri A, Carpano M, Gardini A, Prediletto I, Polastri M, Curtis JR, Nava S. Feasibility and efficacy of a multidisciplinary palliative approach in patients with advanced interstitial lung disease. A pilot randomised controlled trial. Pulmonology. 2021 Dec 27:S2531-0437(21)00223-3. doi: 10.1016/j.pulmoe.2021.11.004. Online ahead of print.