Cystic fibrosis

doi:10.1017/CBO9780511543579.156

Cystic fibrosis

from Medical topics

Published online by Cambridge University Press: 18 December 2014

Edited by

John Weinman and

Claire A. Glasscoe: Affiliation:
Royal Liverpool Children's Hospital
Susan Ayers: Affiliation:
University of Sussex
Andrew Baum: Affiliation:
University of Pittsburgh
Chris McManus: Affiliation:
St Mary's Hospital Medical School
Stanton Newman: Affiliation:
University College and Middlesex School of Medicine
Kenneth Wallston: Affiliation:
Vanderbilt University School of Nursing
John Weinman: Affiliation:
United Medical and Dental Schools of Guy's and St Thomas's
Robert West: Affiliation:
St George's Hospital Medical School, University of London

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Summary

The disease

Cystic fibrosis (CF) is the most common fatal hereditary disease in the Western world; although not confined to those of European ancestry, CF is rare in oriental and native African people (Tsui, 1990). The recessive gene is carried by 4% of the populations in the UK and US affecting 1:2000–3000 live births (Bobadilla et al., 2002). The disease is characterized by generalized dysfunction of the exocrine glands. These produce excessively viscous mucus secretions that block ducts and are prone to infection. Lung infections are the most serious complication with 90% of those affected with CF eventually succumbing to pulmonary disease. Pancreatic insufficiency is present in 85–90% of patients with malabsorption of fat and protein. Sterility occurs in 98% of males whereas female fertility is near normal. Puberty is generally delayed by two years in both sexes reflecting the reduced height and weight potential.

During 1969–90 the median age of survival doubled in the US from 14 to 28 years (FitzSimmons, 1993) and was reported to be 30 years in the UK in 1994 (Dodge et al., 1997). This improved prognosis is attributable to specialist multidisciplinary CF centres, early diagnosis and breakthroughs in management. Neonatal screening for CF has been piloted and implemented in some parts of Europe and in the US and Canada and scheduled to start nationally in the UK in 2006 (see ‘Screening: antenatal’).

Type: Chapter
Information: Cambridge Handbook of Psychology, Health and Medicine , pp. 662 - 665

DOI: https://doi.org/10.1017/CBO9780511543579.156 [Opens in a new window]

Publisher: Cambridge University Press

Print publication year: 2007

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Cystic fibrosis

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