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Sickle Cell Anemia in the Female Patient

Dauphin-McKenzie, Nathalie MD*; Gilles, Jerry M. MD; Jacques, Elvire MD; Harrington, Thomas MD§

Obstetrical & Gynecological Survey: May 2006 - Volume 61 - Issue 5 - p 343-352
doi: 10.1097/01.ogx.0000216976.15100.29
CME Program: CATEGORY 1 CME REVIEW ARTICLES 13, 14, AND 15: CME REVIEW ARTICLE 15
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Seventy-two thousand Americans are homozygous for the sickle cell gene and 2 million are carriers. The gene offers protection against malaria but can be a cause of chronic pain and early death. Life expectancy is 48 years for females. Some people with sickle cell anemia live into their 60s and beyond. The purpose of this article is to review and summarize evidence from clinical, translational, and epidemiologic studies that have examined the clinically relevant aspects of sickle cell anemia as it relates to the female patient. Studies were identified through a MEDLINE search for articles in English between the years 1966 and 2005. References from identified reports were also used to identify additional articles. Women with sickle cell disease experience multiple complications. These complications can affect each and every organ system and are often worse in pregnant women. Progestins, hydroxyurea, and bone marrow transplant appear to ameliorate sickle cell anemia. Other therapies being evaluated include those that increase fetal hemoglobin concentration and prevent dehydration of the sickle red blood cells. More than one third of pregnancies in women with sickle syndromes terminate in abortion, stillbirth, or neonatal death. Recently, a number of genes modifying the clinical severity of sickle cell anemia have been identified. Sickle anemia is associated with immense suffering and multisystemic complications. In addition to the now-established therapy with hydroxyurea and bone marrow transplants, there are multiple investigational treatments that offer the hope of extending life expectancy while diminishing associated morbidities. Whether any of these new agents are safe in pregnancy has yet to be determined.

Target Audience: Obstetricians & Gynecologists, Family Physicians

Learning Objectives: After completion of this article, the reader should be able to summarize the multiple complications that women with sickle cell anemia (SCA) endure, explain that many of the complications worsen during pregnancy and increase the risk of an adverse pregnancy outcome, and state that there are treatment modalities that extend life and diminish morbidities.

*Resident Physician, Department of Obstetrics and Gynecology, University of Miami–Jackson Memorial Hospital, Miami, Florida; †Associate Professor and ‡Assistant Professor, Department of Obstetrics and Gynecology, Division of Maternal–Fetal Medicine, and §Assistant Professor, Department of Medicine, Division of Hematology/Oncology, University of Miami, Miami, Florida

Chief Editor’s Note: This article is part of a series of continuing education activities in this Journal through which a total of 36 AMA/PRA category 1 credit hours can be earned in 2006. Instructions for how CME credits can be earned appear on the last page of the Table of Contents.

The authors have disclosed that they have no financial relationships with or interests in any commercial companies pertaining to this educational activity.

Lippincott Continuing Medical Education Institute, Inc. has identified and resolved all faculty conflicts of interest regarding this educational activity.

Reprint requests to: Nathalie Dauphin-McKenzie, MD, 2451 Brickell Avenue Suite 17B, Miami, FL 33129. E-mail: ndauphin@um-jmh.org.

© 2006 Lippincott Williams & Wilkins, Inc.