Articles

Recent Articles

* These CDC scientific articles are listed in order of date published from 2006 to present.

Health Care Utilization and Expenditures for Privately and Publicly Insured Children with Sickle Cell Disease in the United States 
Pediatric Blood Cancer, October 2009; 53(4) 642-6.
Mvundura M, Amendah D, Kavanagh PL, Sprinz PG, Grosse SD

No national estimates currently exist on health care utilization and expenditures for US children with sickle cell disease.  The Centers for Disease Control and Prevention, the Boston University School of Medicine, and the Boston Medical Center estimated health services use and expenditures in a sample of 2,428 Medicaid-enrolled and 621 privately insured children with SCD.
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Trends in Pediatric Sickle Cell Disease-Related Mortality in the United States, 1983-2002 
The Journal of Pediatrics; April 2009; 154(4), 541-5
Yanni E, Grosse S, Yang Q, Olney RS

The objective was to analyze trends in sickle cell disease (SCD)-related mortality among black children during 1983-2002. The conclusion was that recent decreases in SCD mortality in black children under age 4 years coincided with the introduction of the 7-valent pneumococcal conjugate vaccine in 2000, although temporal association is not evidence of causation.
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Impact of Hurricane Katrina on Newborn Screening in Louisiana
Pediatrics; October 2007; 120(4):e749-e755
Lobato MN, et al

The Louisiana Office of Public Health and the Centers for Disease Control and Prevention assessed the extent to which newborn screening was disrupted from August 15 to September 21, 2005, the immediate period before and after Hurricane Katrina.
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Progestogen-Only Contraceptive Use among Women with Sickle Cell Anemia: A Systematic Review
Contraception; February 2006; 73(2):195-204
Legardy J, et al

The use of progestogen-only contraceptives among women with sickle cell anemia has generated concerns about possible hematological and other clinical complications. Although data are limited, these studies suggest that progestogen-only contraceptives are safe for women with sickle cell anemia.
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Additional Selected Articles

Relation between the Response to Iron Supplementation and Sickle Cell Hemoglobin Phenotype in Preschool Children in Western Kenya
American Journal of Clinical Nutrition; 2004; 79:466-72
Terlouw D, et al

The objective was to determine the influence of the HbAS phenotype on hematologic responses and malaria after iron supplementation in anemic (hemoglobin: 70-109 g/L) children aged 2-35 months. The conclusion was that there was no evidence for a clinically relevant modification by the hemoglobin S phenotype of the effects of iron supplementation in the treatment of mild anemia. The benefits of iron supplementation are likely to outweigh possible risks associated with malaria in children with the HbAA or HbAS phenotype.
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Increased Efficacy of Sulfadoxine-Pyrimethamine in the Treatment of Uncomplicated Falciparum Malaria among Children with Sickle Cell Trait in Western Kenya
The Journal of Infectious Diseases; 2002; 186:1661-8
Terlouw D, et al

The role of the sickle cell hemoglobin type as a determinant of treatment outcome with sulfadoxine-pyrimethamine was retrospectively studied in young children with uncomplicated falciparum malaria who lived in an area with intense perennial malaria transmission. The results suggest that the HbAS phenotype should be included among factors that determine sulfadoxine-pyrimethamine treatment outcome.
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Protective Effects of the Sickle Cell Gene against Malaria Morbidity and Mortality
The Lancet; 2002; 359:1311-1312
Aidoo M, et al

The high frequency of the sickle-cell haemoglobin (HbS) gene in malaria endemic regions is believed to be due to a heterozygote (HbASp) advantage against fatal malaria. Data to prospectively confirm the protection associated with HbAS against mortality are lacking. We show that HbAS provides significant protection against all-cause mortality, severe malarial anaemia, and high-density parasitaemia.
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Update: Newborn Screening for Sickle Cell Disease - California, Illinois, and New York
Morbidity and Mortality Weekly Report; August 18, 2000; 49(32); 729-731
CDC

This report summarizes the results of this study, which demonstrate the difficulty in retrospectively finding children who were screened at birth so that data for evaluating program effectiveness can be assessed.
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Newborn Screening for Sickle Cell Disease:  Public Health Impact and Evaluation in Part IV
Genomics and Public Health in the 21st Century; July 2000 
Olney RS

Sickle cell disease is an example of a common genetic condition for which preventive strategies have been particularly effective at reducing rates of complications, and the historical precedents of sickle cell newborn screening and treatment may serve as a public health model for other conditions considered for population screening in the future.
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Sickle Hemoglobin (Hb S) Allele and Sickle Cell Disease: A HuGE Review 
American Journal of Epidemiology; 2000 May 1; 151(9): 839-845 
Ashley-Koch A, Yang Q, Olney RS

Sickle cell disease is caused by a variant of the beta-globin gene called sickle hemoglobin (Hb S). In recent years, newborn screening, better medical care, parent education, and penicillin prophylaxis have successfully reduced morbidity and mortality due to Hb S.
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Preventing Morbidity and Mortality from Sickle Cell Disease: A Public Health Perspective 
American Journal of Preventive Medicine; 1999; 16(2):116-121
Olney RS 

The objective is to discuss current public health issues associated with sickle cell disease, and approaches to preventing complications from these conditions in the United States.  The author concludes that although the efforts of preventive medicine providers in public health programs are not solely responsible for the improved survival of children with sickle cell disease, such programs remain an important component in preventing sickle cell complications.
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Mortality Among Children with Sickle Cell Disease Identified by Newborn Screening During 1990-1994 - California, Illinois, and New York
Morbidity and Mortality Weekly Report; 1998; 47:169-172
CDC 

This report focuses on mortality in California, Illinois, and New York. In California and Illinois, mortality from all causes among black children born during 1990-1994 with SCD was slightly less than overall mortality for all black children born in the same time period.
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Sickle Cell and Thalassemia Health and Safety, A Guide to School Policy, January 2011.
Simon Dyson BSc; MPhil; PhD
Unit for the Social Study of Thalassaemia and Sickle Cell,
De Montfort University, Leicester, United Kingdom
sdyson@dmu.ac.uk

This booklet has been produced based on research examining the experiences of young people with sickle cell disease in schools in England. An important part of school inclusiveness is recognizing the importance of offering care to young people with long-standing illness, particularly since a major part of childhood is spent attending school. [Booklet]

 

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