Palliative care and end of life management in patients with idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic disease with an unknown etiology that causes deterioration of the structure of the lung parenchyma, resulting in a severe and progressive decline in respiratory function and early mortality. IPF is essentially an incurable disease, with a mean overall survival of 5 years in approximately 20% of patients without treatment. The combination of a poor prognosis, uncertainty about the disease's progression, and the severity of symptoms has a significant impact on the quality of life of patients and their families. New antifibrotic drugs have been shown to slow disease progression, but their impact on health-related quality of life (HRQoL) has to be proven yet. To date, studies have shown that palliative care can improve symptom management, HRQoL, and end-of-life care (EoL) in patients with IPF, reducing critical events, hospitalization, and health costs. As a result, it is essential for proper health planning and patient management to establish palliative care early and in conjunction with other therapies, beginning with the initial diagnosis of the disease.

Right heart and pulmonary thromboembolism from extensive splanchnic vein thrombosis after splenectomy for myeloproliferative disease.

BACKGROUND: Splenectomy is a risk factor for both portal-vein and chronic thromboembolic pulmonary hypertension. The underlying mechanism is unclear, but may involve a hypercoagulable state. METHODS: We describe 1 patient with polycythemia vera who developed extensive portal thrombosis of the portal, suprahepatic, and inferior cava veins, leading to right heart thromboembolism, with a resultant pulmonary embolism subsequent to splenectomy despite heparin prophylaxis. RESULTS: In this patient, several mechanisms may have played a role, including perioperative stress, thrombocytosis, thrombophilia, and associated chronic liver disease. Nevertheless, combined treatment with intravenous heparin and thrombolysis and the myeloproliferative inhibitor hydroxyurea was associated with a favorable outcome. CONCLUSION: The risk of pulmonary thromboembolic complications and their management after splenectomies for hematologic disease warrant further study.