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Sickle cell disease

from Medical topics

Published online by Cambridge University Press:  18 December 2014

By
James Elander
Edited by
Susan Ayers ,
Andrew Baum ,
Chris McManus ,
Stanton Newman ,
Kenneth Wallston ,
John Weinman  and
Robert West
James Elander
Affiliation:
University of Derby
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

Sickle cell disease (SCD) is a group of blood disorders caused by genetically determined amino acid substitutions in the beta polypeptide chains of the haemoglobin molecule. The abnormality causes haemoglobin to polymerize, or gel, when oxygen tension is lowered, making the red cells stiffen and elongate into a characteristic sickle or crescent shape, causing episodic vaso-occlusion and tissue infarction. It is a lifelong chronic condition and although survival rates have improved significantly over the last 50 years, those affected continue to suffer from chronic anaemia, susceptibility to infections, a shortened life expectancy and recurrent episodes of severe pain. In the UK, the condition affects over 10 000 people, with over 140 babies with SCD born every year (Hickman et al., 1999). In the USA, one in every 400 African-American new births are babies with SCD (Consensus Conference, 1987), and many more are affected in Africa, the Caribbean and parts of Asia. Worldwide, as many as 300 000 babies are born every year with sickle cell disorders (Angastiniotis & Modell, 1998).

Genetic testing and screening, coping and adjustment and pain management are all important psychological issues in SCD, and sickling episodes in the brain have potential neuropsychological consequences (Kral et al., 2001; Prengler et al., 2002). It was often said that psychological aspects of SCD were under-researched, but this is now less true than in the past.

Type
Chapter
Information
Cambridge Handbook of Psychology, Health and Medicine , pp. 877 - 880
Publisher: Cambridge University Press
Print publication year: 2007

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