Sickle cell disease

Document Type

Book

Publication Date

2009

Abstract

Sickle cell disease (SCD) is a collection of autosomal recessive genetic disorders involving the abnormal production of hemoglobin. In SCD, red blood cells are short-lived and brittle, assuming a sickled shape that hinders their ability to effectively deliver oxygen throughout the body. In addition, impaired red blood cells often aggregate to occlude smaller blood vessels and significantly reduce the amount of oxygenated blood to the lungs and other tissues (Serjeant, 1997). Several SCD variants of graded severity exist including homozygous SCD (HbSS), which is noted to be the most severe, and heterozygous SCD associated with more benign symptoms. In addition, three heterozygous thalassemia deviations occur with varying degrees of clinical manifestation (Helps, Fuggle, Udwin, & Dick, 2003). Individuals who carry the sickle cell trait generally do not experience symptoms associated with the disease (Rees et al., 2003). © 2009 Springer Science+Business Media, LLC.

Publication Title

Behavioral Approaches to Chronic Disease in Adolescence: A Guide to Integrative Care

First Page

253

Last Page

267

Comments

This chapter was published in Behavioral Approaches to Chronic Disease in Adolescence: A Guide to Integrative Care, Volume , Issue , Pages 253-267.

The published version is available at http://dx.doi.org/10.1007/978-0-387-87687-0_19.

Copyright © 2009 Wiley.

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