Sickle Cell Disease at 100 Years.

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    • Subject Terms:
      SICKLE cell anemia; BLOOD disease treatment; HEMOGLOBINOPATHY; HEMOGLOBINS; SUBSTITUTION reactions; BIOCHEMISTRY; ALPHA fetoproteins
    • Abstract:
      The article discusses sickle cell disease (SCD) and the progress that science has made in SCD treatment. A brief overview is presented which includes the discovery of SCD 60-years ago, an explanation of the substitution reaction in the β-globin chain of hemoglobin (HbA) that causes SCD, and statistics on how many people die in Africa and the U.S. from SCD. Also discussed are the various kinds of therapy patients with SCD receive, including hydration, antibiotics, and blood transfusions, the role of fetal hemoglobin (HbF, α2γ2) in the variation of SCD severity, and how an understanding of the hemoglobin switch can be used to design mechanism-based approaches to treating SCD.