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Blood Rev. 2013 Nov;27(6):279-87. doi: 10.1016/j.blre.2013.09.001. Epub 2013 Sep 19.

Management of sickle cell disease from childhood through adulthood.

Author information

1
Department of Pediatric Hematology-Oncology, Sickle Cell Research, Medical University of South Carolina, Charleston, SC, USA. Electronic address: kanter@musc.edu.

Abstract

Sickle cell disease (SCD) is a genetic disorder characterised by anaemia and "sickling" of red blood cells, leading to chronic haemolytic anaemia, vascular injury, and organ dysfunction. Although children and adults experience many similar symptoms and problems, complications increase with age, leading to early mortality. Hydroxyurea (hydroxycarbamide), the only US Food and Drug Administration-approved treatment, continues to be under-utilised and other treatments available to children are often inaccessible for adults. Haematopoietic stem-cell transplantation is a curative option, but is limited by a lack of donors and concerns for transplant-related toxicities. Although comprehensive programs exist in paediatrics, affected adults may not have access to preventative and comprehensive healthcare because of a lack of providers or care coordination. They are often forced to rely on urgent care, leading to increased healthcare utilisation costs and inappropriate treatment. This problem highlights the importance of primary care during the transition from paediatrics to adulthood.

KEYWORDS:

Adult; Haematology; Paediatric; Sickle cell disease

PMID:
24094945
DOI:
10.1016/j.blre.2013.09.001
[Indexed for MEDLINE]
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