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      Sickle cell anemia in sub-Saharan Africa: advancing the clinical paradigm through partnerships and research

      Author(s): 1 , 1 , 1
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      Journal: Blood
      Publisher: American Society of Hematology

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          Abstract

          <p class="first" id="d10997785e139"> <b>Publisher's Note:</b> There is an <span class="generated">[Related article:]</span>Inside <i>Blood</i> Commentary on this article in this issue. </p>

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          Sickle cell trait and the risk of Plasmodium falciparum malaria and other childhood diseases.

          Neal D E Alexander, Moses Kortok, Kevin Marsh … (2005)
          The gene for sickle hemoglobin (HbS) is a prime example of natural selection. It is generally believed that its current prevalence in many tropical populations reflects selection for the carrier form (sickle cell trait [HbAS]) through a survival advantage against death from malaria. Nevertheless, >50 years after this hypothesis was first proposed, the epidemiological description of the relationships between HbAS, malaria, and other common causes of child mortality remains incomplete. We studied the incidence of falciparum malaria and other childhood diseases in 2 cohorts of children living on the coast of Kenya. The protective effect of HbAS was remarkably specific for falciparum malaria, having no significant impact on any other disease. HbAS had no effect on the prevalence of symptomless parasitemia but was 50% protective against mild clinical malaria, 75% protective against admission to the hospital for malaria, and almost 90% protective against severe or complicated malaria. The effect of HbAS on episodes of clinical malaria was mirrored in its effect on parasite densities during such episodes. The present data are useful in that they confirm the mechanisms by which HbAS confers protection against malaria and shed light on the relationships between HbAS, malaria, and other childhood diseases.
          Article 0 comments Cited 124 times – based on 0 reviews     Review now
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            The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS).

            E. Rivas Plata, George Stamatopoulos, A Balassopoulou … (2010)
            The aim of this prospective study was to evaluate the long-term efficacy and safety of hydroxyurea (HU) in patients with sickle cell disease (SCD). Thirty-four patients with sickle cell anemia (hemoglobin S [HbS]/HbS), 131 with HbS/beta(0)-thal, and 165 with HbS/beta(+)-thal participated in this trial. HU was administered to 131 patients, whereas 199 patients were conventionally treated. The median follow-up period was 8 years for HU patients and 5 years for non-HU patients. HU produced a dramatic reduction in the frequency of severe painful crises, transfusion requirements, hospital admissions, and incidence of acute chest syndrome. The probability of 10-year survival was 86% and 65% for HU and non-HU patients, respectively (P = .001), although HU patients had more severe forms of SCD. The 10-year probability of survival for HbS/HbS, HbS/beta (0)-thal, and HbS/IVSI-110 patients was 100%, 87%, and 82%, respectively, for HU patients and 10%, 54%, and 66%, for non-HU patients. The multivariate analysis showed that fetal hemoglobin values at baseline and percentage change of lactate dehydrogenase between baseline and 6 months were independently predicted for survival in the HU group. These results highlight the beneficial effect of HU, which seems to modify the natural history of SCD and raise the issue of expanding its use in all SCD patients.
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              The cost of health care for children and adults with sickle cell disease.

              Teresa L Kauf, Thomas D Coates, Liu Huazhi … (2009)
              Although sickle cell disease (SCD) is marked by high utilization of medical resources, the full cost of care for patients with SCD, including care not directly related to SCD, is unknown. The purpose of this study was to estimate the total cost of medical care for a population of children and adults with SCD. We used data from individuals diagnosed with SCD enrolled in the Florida Medicaid program during 2001-2005 to estimate total, SCD-related, and non-SCD-related cost per patient-month based on patient age at the time of health care use. Across the 4,294 patient samples, total health care costs generally rose with age, from $892 to $2,562 per patient-month in the 0-9- and 50-64-year age groups, respectively. Average cost per patient-month was $1,389. Overall, 51.8% of care was directly related to SCD, the majority of which (80.5%) was associated with inpatient hospitalizations. Notably, non-SCD-related costs were substantially higher than those reported for the general US population. These results suggest a discounted (3% discount rate) lifetime cost of care averaging $460,151 per patient with SCD. Interventions designed to prevent SCD complications and avoid hospitalizations may reduce the significant economic burden of the disease.
              Article 0 comments Cited 102 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Title: Blood
                Publisher: American Society of Hematology
                ISSN (Print): 0006-4971
                ISSN (Electronic): 1528-0020
                Publication date Created: January 12 2017
                Publication date (Print): January 12 2017
                Volume: 129
                Issue: 2
                Pages: 155-161
                Affiliations
                [1 ]Division of Hematology, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, Cincinnati OH
                Article
                DOI: 10.1182/blood-2016-09-702324
                PMC ID: 5234214
                PubMed ID: 27821508
                SO-VID: fd770d07-05dc-4796-84a5-c03a3a12e6ca
                Copyright © © 2017
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