Cystic fibrosis

Claire A. Glasscoe

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

Book contents

Get access

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

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Bartholomew, L.K., Czyzewski, D., Parcel, G.S.et al. (1997). Self-Management of cystic fibrosis: short-term outcomes for the Cystic Fibrosis Family Education Programme. Health Education & Behaviour, 25, 652–66.Google Scholar

Bluebond-Langner, M. (1991). Living with cystic fibrosis: a family affair. In Morgan, J.D. (Ed.). Young people and death (pp. 46–62). Philadelphia: Charles Press.

Bobadilla, J.L., Macek, M. Jr., Fine, J.P. & Farrell, P.M. (2002). Cystic fibrosis: a worldwide analysis of CFTR mutations – correlation with incidence data and application to screening. Human Mutation, 19, 575–606.Google Scholar

Davis, M.A., Quittner, A.L., Stack, C.M. & Yang, M.C.K. (2004). Controlled evaluation of the STARBRIGHT CD-ROM program for children and adolescents with cystic fibrosis. Journal of Pediatric Psychology, 29, 259–67.Google Scholar

Delk, K.K., Gevirtz, R., Hicks, D.A., Carden, F. & Rucker, R. (1994). The effects of biofeedback assisted breathing retraining on lung functions in patients with cystic fibrosis. Chest, 105, 23–8.Google Scholar

DeLambo, K.-E., Ievers-Landis, C.E., Drotar, D. & Quittner, A.L. (2004). Association between observed family relationship quality and problem-solving skills with treatment adherence in older children and adolescents with cystic fibrosis. Journal of Pediatric Psychology, 29, 342–53.Google Scholar

Dodge, J.A., Morison, S., Lewis, P.A.et al. (1997). Incidence, population and survival of cystic fibrosis in the UK, 1968–95. Archives of Disease in Childhood, 77, 493–6.Google Scholar

FitzSimmons, S.C. (1993). The changing epidemiology of cystic fibrosis. The Journal of Pediatrics, 122, 1–9.Google Scholar

Glasscoe, C.A. & Quittner, A.L. (2002). Psychological interventions for cystic fibrosis (Cochrane Review). The cochrane library, Issue 3. Oxford: Update Software.

Hodson, M.E. & Geddes, D.M. (2000). Cystic fibrosis. London: Arnold.

Kerem, B., Rommens, J.M., Buchanan, J.A.et al. (1989). Identification of the cystic fibrosis gene: genetic analysis. Science, 145, 1073–80.Google Scholar

Koocher, G.P., McGrath, M.L. & Gudas, L.J. (1990). Typologies of nonadherence in cystic fibrosis. Developmental and Behavioural Pediatrics, 11, 353–8.Google Scholar

Patterson, J.M., McCubbin, H.I. & Warwick, W.J. (1990). The impact of family functioning on health changes in children with cystic fibrosis. Social Science and Medicine, 31, 159–64.Google Scholar

Pearson, D.A., Pumariega, A.J. & Seilheimer, D.K. (1991). The development of psychiatric symptomatology in patients with cystic fibrosis. Journal of the American Academy of Child and Adolescent Psychiatry, 30, 290–7.Google Scholar

Quittner, A.L., DiGirolamo, A.M., Michel, M. & Eigen, H. (1992). Parental response to cystic fibrosis: A contextual analysis of the diagnosis phase. Journal of Pediatric Psychology, 17, 683–704.Google Scholar

Quittner, A.L., Esplanage, D.L., Opipari, L.C.et al. (1998). Role strain in couples with and without a child with a chronic illness: associations with marital satisfaction, intimacy, and daily mood. Health Psychology, 17, 112–24.Google Scholar

Simmons, R.J. & Goldberg, S. (2001). Infants and preschool children. In Bluebond-Langner, M.Lask, B. & Angst, D.B. (Eds.). Psychosocial aspects of cystic fibrosis (pp. 10–24). London: Arnold.

Stark, L.J., Bowen, A.M., Tyc, V.L., Evans, S. & Passero, M.A. (1990). A behavioral approach to increasing calorie consumption in children with cystic fibrosis. Journal of Pediatric Psychology, 15, 309–26.Google Scholar

Stark, L.J., Millar, S.T., Plienes, A.J. & Drabman, R.S. (1987). Behavioral contracting to increase chest physiotherapy: a study of a young cystic fibrosis patient. Behavior Modification, 11, 75–86.Google Scholar

Thompson, R.J., Hodges, K. & Hamlett, K.W. (1990). A matched comparison of adjustment in children with cystic fibrosis and psychiatrically referred and nonreferred children. Journal of Pediatric Psychology, 15, 745–59.Google Scholar

Tsui, L.-C. (1990). Population analysis of the major mutation in cystic fibrosis (Editorial). Human Genetics, 85, 391–2.Google Scholar

Wilson, J., Fosson, A., Kanga, J.F. & D'Angelo, S.L. (1996). Homeostatic interactions: a longitudinal study of biological and family variables in children with cystic fibrosis. Journal of Family Therapy, 18, 123–39.Google Scholar

Book contents

Cystic fibrosis

Summary

Access options

References

Save book to Kindle

Save book to Dropbox

Save book to Google Drive