The comprehensive care of the patient with IPF involves balancing the three pillars of disease-centered management, symptom-centered management, and patient education and self-management upon a solid foundation of provider-patient partnership. Constant reassessment of the individual patient's goals of care, based on their values and preferences, is essential to the constant recalibration of these various interventions.
Supplemental oxygen users experience frequent and varied problems, particularly a lack of access to effective instruction and adequate portable systems. Initiatives by professional and patient organizations are needed to improve patient education, and to promote access to equipment and services tailored to each patient's needs.
Idiopathic pulmonary fibrosis (IPF) is a debilitating, progressive, and fatal fibrotic lung disease with a poor prognosis. Antifibrotic therapy slows but does not halt disease progression. Patient education and management needs change during disease progression. Management is complicated by comorbidities, adverse events associated with antifibrotic therapy, and difficulties with long-term oxygen therapy and pulmonary rehabilitation. Treating IPF requires coordination between physicians and nurses in community and interstitial lung disease center settings. This review provides guidance for the healthcare professional who manages the essential aspects of care in IPF from diagnosis, through disease progression, and to the end of life.
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