Conference: 2009 PH Resource Network Symposium
Release Date: 09.24.2009
Presentation Type: Abstracts
Ann Gihl, MA, RN-C
LifeSource Upper Midwest Organ Procurement Organization, Inc. St. Paul, MN
BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare incurable disease. Patients report living with a significant degree of uncertainty and burdens. There is limited research in understanding health-related quality of life (HRQOL) in PAH.
PURPOSE: The purpose of this literature review was to critically appraise the available literature on HRQOL in PAH.
RESULTS: Twenty-three studies published between 1996 and July 2008 were reviewed with content relating to HRQOL in PAH. Measurement of HRQOL showed profound impairment in PAH patients; however, this was confounded by small samples, undefined HRQOL conceptualizations, and lack of PAH disease specific tools. Two nursing qualitative studies identified patient perspectives on coping with the uncertainty of disease and therapy. Pharmaceutical therapy may improve physical dimensions and functional ability, yet other domains such as psychosocial domains of HRQOL remain impaired. Non-pharmaceutical interventions to improve HRQOL are minimally studied, but exercise suggested improvement in all dimensions of HRQOL. Recent HRQOL studies in PAH have sought to validate tools to measure HRQOL. The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is the first tool developed from the PAH patient perspectives to measure symptoms, functioning, and quality of life.
CONCLUSIONS: Research in HRQOL for PAH is underdeveloped. Unmet needs in psychological and social domains may require interventions to help the person cope with uncertainty and daily needs of living with therapy. Assessing needs from the patient perspective using tools such as CAMPHOR is helpful to discover each person's unique experience with PAH.
IMPLICATIONS FOR RESEARCH AND PRACTICE: Nursing can help facilitate the adaption needed to cope with PAH. Specific areas for nursing to assess and target include role changes, self-image, spirituality, and sexuality. Nursing has potential to improve HRQOL in PAH by identifying key influences, determining patient needs, and applying interventions to improve the quality of life for those with PAH.
KEYWORDS: pulmonary hypertension, quality of life, health-related quality of life, uncertainty.