Introduction and Objectives Idiopathic pulmonary fibrosis (IPF) is a rare, fibrotic, progressive lung disease that results in shortness of breath and difficulty in breathing. IPF commonly presents with crackling sounds in the lungs discernable via stethoscopic auscultation. IPF is most prevalent in patients >50 years of age. Risk factors include smoking and environmental exposures (metal, wood dust and other airborne pollutants). Disease progression features clubbing of the fingers, increasing difficulty in breathing, requirement for supplemental oxygen and/or lung transplantation. IPF is ultimately fatal. We conducted a European survey of diagnosed IPF patients to identify unmet needs in the management of IPF and opportunities to improve care.
Methods Patients in Germany, France, Italy, Spain and the UK with a confirmed diagnosis of IPF underwent a structured interview at home. In some instances, involved carers were also interviewed. The interviews featured associative questions where images were selected to express feelings, and constructive questions where factual recall was tested. Patient emotions were mapped according to four principle stages of the patient journey: 1. Symptoms, 2. Initial diagnosis, 3. After diagnosis, and 4. Disease management.
Results Forty-five patients (median age 67 years) and 18 care givers were interviewed for the survey. In 58% of cases, diagnosis was protracted due to dismissal of symptoms and misdiagnosis. Patients could be categorised in one of four ways (combative, serene, dejected and stoic) based chiefly on their age and attitude to disease management. Patients expressed a range of emotions at the point of diagnosis, and diagnosis was recognised as a time at which the patient-doctor relationship could break down. Patients treated in specialist centres reported better satisfaction with care than those treated by generalists. The patients cited negative impacts of IPF on almost all aspects of their lives, which could lead to depression. Keeping fit was acknowledged as a means to control disease symptoms and maintain a positive outlook.
Conclusion IPF has an enormous impact on patient quality of life, and patients are affected differently by the disease and its diagnosis. Emotional support and participation in patient support groups should be considered a key component of IPF management.
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