title: Understanding the impact of pulmonary arterial hypertension on patients' and carers' lives.
abstract: Pulmonary arterial hypertension (PAH) is a rare, debilitating and rapidly progressive disease. Although there have been important medical advances in PAH management, the search for a cure continues. Despite an increased understanding of the disease, data on the wider effect of PAH on patients and carers, beyond the clinical symptoms, are still limited. In order to explore this, a large-scale international survey investigated four key areas affected by PAH (physical and practical, emotional, social, and information needs) and provides new insight into patients' and carers' experiences of living with the disease. The results from the survey highlight not only the limited ability of patients to carry out everyday tasks, but also the financial impact and social isolation experienced by both patients and carers. The study confirmed that a decline in a patient's World Health Organization functional class, which indicates an increase in clinical severity of the disease, is associated with greater limitations. Results from the survey demonstrate the need for multidisciplinary PAH management and a comprehensive standard of care to assess and improve all aspects of well-being for both patients and carers. In addition, they underline the need for updated PAH guidelines that address these needs.
authors.affiliation: Service de Médecine Interne, Hôpital Cochin, Paris, France. 2 Pulmonary Hypertension Association UK, Rotherham, and 4 National Pulmonary Hypertension Service, Hammersmith Hospital, Imperial College London, London, UK. 3 Pulmonary Hypertension Association US, Silver Spring, MD, 6 Division of Rheumatology, Dept of Medicine, National Jewish Health, Denver, CO, 7 UCSD Medical Center, San Diego, CA, and 8 Woodhull Medical Center, New York University, New York, NY, USA. 5 Lung Allergy Clinic, Karolinska University Hospital, Stockholm, Sweden. 9 Pulmonary Hypertension Association Europe, Vienna, Austria.
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.forename: Iain
authors.initials: I
authors.lastname: Armstrong
authors.forename: Rino
authors.initials: R
authors.lastname: Aldrighetti
authors.forename: Luke S
authors.initials: LS
authors.lastname: Howard
authors.forename: Henrik
authors.initials: H
authors.lastname: Ryftenius
authors.forename: Aryeh
authors.initials: A
authors.lastname: Fischer
authors.forename: Sandra
authors.initials: S
authors.lastname: Lombardi
authors.forename: Sean
authors.initials: S
authors.lastname: Studer
authors.forename: Pisana
authors.initials: P
authors.lastname: Ferrari
mesh_terms: D000203:Activities of Daily Living; D017028:Caregivers / Q000523:psychology*; D017281:Cost of Illness; D065627:Familial Primary Pulmonary Hypertension; D019538:Health Care Surveys; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000191:economics / Q000523:psychology* / Q000628:therapy; D007398:Interpersonal Relations; D010361:Patients / Q000523:psychology*; D011379:Prognosis; D011788:Quality of Life; D012720:Severity of Illness Index; D012919:Social Behavior; D012959:Socioeconomic Factors; D011795:Surveys and Questionnaires