title: Sickle cell anemia in northern Israel: screening and prevention.
abstract: BACKGROUND
  Sickle cell anemia is a hemolytic anemia caused by a single mutation in position 6 of the beta globin molecule. About 80 patients with SCA in northern Israel are currently receiving treatment.
  
  
  OBJECTIVE
  To assess a screening program in northern Israel aimed at detecting couples at risk for having offspring with SCA.
  
  
  METHODS
  Since 1987, screening for beta thalassemia in pregnant women in northern Israel has been conducted, and from 1999 all the samples were also tested for hemoglobin S, Hgb C, Hgb D, Hgb O Arab and others.
  
  
  RESULTS
  During the 20 year period 1987-2006 a total of 69,340 women were screened; 114 couples who carried Hgb S were detected and 187 prenatal diagnoses were performed in couples at risk for having an offspring with Hgb S. The mean gestational age was 13 +/- 4 weeks. Fifty-four of those diagnoses revealed affected fetuses and in 4 cases the couple declined to perform therapeutic abortion.
  
  
  CONCLUSIONS
  The economic burden to the health services for treating SCA patients is about U.S.$ 7000 per year, and the institution of prevention programs has proven cost-effective in populations with a high frequency of carriers. Since our program is aimed to also detect beta thalassemia, a disease that is more frequent in this area (> 2.5%), the added cost for the prevention of SCA is less significant despite the low incidence of the S gene in our population, namely < 1%.
authors: Pediatric Hematology Unit and Department of Pediatrics B, HaEmek Medical Center, Afula, Israel. koren_a@clalit.org.il
authors: Ariel
authors: A
authors: Koren
authors: Lucia
authors: L
authors: Zalman
authors: Haya
authors: H
authors: Palmor
authors: Ronit Bril
authors: RB
authors: Zamir
authors: Carina
authors: C
authors: Levin
authors: Ariella
authors: A
authors: Openheim
authors: Etty
authors: E
authors: Daniel-Spiegel
authors: Stavit
authors: S
authors: Shalev
authors: Dvora
authors: D
authors: Filon
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000517:prevention & control*; D001609:Beta-Globulins / Q000235:genetics; D005260:Female; D005817:Genetic Counseling; D005820:Genetic Testing; D006451:Hemoglobin, Sickle / Q000235:genetics; D006801:Humans; D007557:Israel / Q000453:epidemiology; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012307:Risk Factors; D055815:Young Adult; D017086:beta-Thalassemia / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000517:prevention & control
chemical_list: D001609:Beta-Globulins; D006451:Hemoglobin, Sickle