Biomaterial Database

Quality of life in cystic fibrosis. 1995

D E Tullis, and G H Guyatt

Adult Cystic Fibrosis Programme, Wellesley Hospital, Toronto, Ontario, Canada.

Cystic fibrosis is a chronic, multisystem genetic disease with a wide variability in clinical severity. In recent years, advances in therapy have led to improved patient survival into adulthood. New treatments are rapidly being developed and require evaluation to determine their efficacy. The measurement of health-related quality of life in cystic fibrosis provides additional information about the impact of this disease that cannot be obtained by physiological tests such as pulmonary function. An instrument to measure health-related quality of life is especially useful as an outcome measure for clinical trials. To date, only a few general quality-of-life (QOL) measures have been used in people with cystic fibrosis. There has been some demonstration of validity in 2 measures (the Quality of Well-Being Scale and the Functional Status Index) but the responsiveness of these instruments in this population has not been established. A cystic fibrosis-specific QOL instrument would be valuable as an outcome measure because of its potential for increased responsiveness, but no published measures exist as yet.

UI	MeSH Term	Description	Entries
D011788	Quality of Life	A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral, social environment as well as health and disease.	HRQOL,Health-Related Quality Of Life,Life Quality,Health Related Quality Of Life
D003550	Cystic Fibrosis	An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.	Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D017063	Outcome Assessment, Health Care	Research aimed at assessing the quality and effectiveness of health care as measured by the attainment of a specified end result or outcome. Measures include parameters such as improved health, lowered morbidity or mortality, and improvement of abnormal states (such as elevated blood pressure).	Assessment, Outcome,Outcome Assessment,Outcome Assessment (Health Care),Outcomes Research,Assessment, Outcomes,Outcome Measures,Outcome Studies,Outcomes Assessment,Assessment, Outcome (Health Care),Assessments, Outcome,Assessments, Outcome (Health Care),Assessments, Outcomes,Measure, Outcome,Measures, Outcome,Outcome Assessments,Outcome Assessments (Health Care),Outcome Measure,Outcome Study,Outcomes Assessments,Research, Outcomes,Studies, Outcome,Study, Outcome