Abstract
Dear Editor:
Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease of unknown etiology. The median survival of patients with IPF ranges from 2.5 to 3.5 years 1 and the rate of progression to death may take several clinical forms: slow deterioration, rapid deterioration and progression, or periods of relative stability interposed with periods of acute respiratory decline; acute exacerbation of IPF has a very poor outcome. 2 Evidence on how in-hospital IPF patients are cared for in a respiratory unit during the last period before death are lacking; 4 we wanted to report our experience studying the available organizational support related to the dying process and quality of care in the last days of life of these patients; in particular we considered patients with acute exacerbation who did not respond to treatment and were referred to our palliative care unit.
Medical records of 11 patients suffering from acute exacerbations of IPF admitted to our department in 2011 were collected retrospectively. Criteria for acute exacerbations of IPF were (1) acute worsening of dyspnea with the course of one month; (2) bibasilar honeycombing with newly developing ground glass attenuation and/or consolidation on high resolution computed tomography scans; (3) deterioration of PaO2 of more than 10 mmHg; and (4) exclusion of other known causes of exacerbation, such as pulmonary infection, pneumothorax, malignancy, pulmonary thromboembolism, and heart failure.
Cardiologic evaluation was performed to exclude evidence of heart failure, and bronchoalveolar lavage excluded concomitant pulmonary infection. Patients were treated with broad-spectrum antibiotics (starting before obtaining cultures) and high doses of methylprednisolone (125 mg to 500 mg a day for three days); corticosteroid therapy was followed by a tapered dosage and mechanical ventilation was not administered. Five patients (45%) did not respond to this treatment, showing progressive deterioration, and met as early as possible a member of the palliative care team to set an appropriate treatment to minimize symptoms (dyspnea, cough, malaise, fatigue, etc). As soon as beds became available, patients were transferred to the hospice for better medical assistance during the last days of life; 8 to 10 days after initiation of therapy, two of these five patients were transferred to the hospice. A single room location was available in all cases and doctors prescribed sedative and morphine with significant benefit in palliating dyspnea and cough. Patients died after 16 days (range 5–30).
It is very difficult to establish criteria concerning the best place to address the needs of respiratory patients in end-of-life stages. 4 IPF should be more appropriately managed by mixed models of care that offer both active and palliative treatments right up to the time of death. Integration of palliative care principles into IPF treatment is essential, including advance care planning, relief of physical and psychological burden, and patient and carer education. 5 Early integration of palliative care with standard care results in meaningful improvement of life and mood; improving quality of life and mood in patients with IPF is a formidable challenge, given the progressive nature of the illness. To have a meaningful effect on patients' quality of life and end-of-life care, palliative care services must be provided earlier in the course of the disease.