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Article Reviews & Commentary

Article Reviews by Thomas J. Mancuso, MD, FAAP

Health Supervision for Children with Sickle Cell Disease.
Section on Hematology/Oncology, Committee on Genetics Pediatrics 2002:109;526-535

This statement from the AAP is intended to provide general pediatrician and subspecialty practitioners with an overview of the genetics, diagnosis, clinical manifestations and treatment of Sickle Cell Disease (SCD). The description of the illness, SCD, captures in one sentence the varied clinical picture seen in children afflicted and the extent the illness affects their lives. "…SCD describes a group of complex, chronic disorders characterized by hemolysis, unpredictable acute complications that can rapidly become life-threatening, and the variable development of chronic organ damage." The statement goes on to distinguish the various forms of SCD such as SS, SC and the 2 sickle-thalassemias. The genetics and diagnostic test for the various forms as well as the protean clinical manifestations are reviewed.

The importance of adequate, comprehensive pain treatment is mentioned in the section dealing with acute illnesses in these children. The section devoted to health maintenance includes discussions of immunizations, prophylactic antibiotics and innovative therapies for SCD. However, the statement does not mention the importance of outpatient analgesia in the care of these children.


This document is a useful reference on SCD, including many references and mention of new, innovative therapies becoming available for these children in addition to a discussion of routine medical, psychosocial, and family care of children with SCD. A glaring absence is perioperative management of these patients, many of whom will come to the OR for procedures. For example, most children with SCD, a hemolytic anemia, have gallstones. I will now get on my soapbox and encourage the members of SPA to become advocates for children with SCD and educate their primary caregivers about the issues surrounding anesthesia and surgery for these patients as well as the contributions we can make to their pain management.

The interested reader should review the following paper wihch evaluated various preop transfusion regimens for patients with sickle-cell disease.
Vichinsky EP.  Haberkern CM.  Neumayr L et al. New England Journal of Medicine. 333(4):206-13, 1995 Jul 27, comment 333(4):251-2
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