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Sickle-cell Anaemia (SCA) is a blood disorder typically inherited from a person's parents, who are carriers of sickle-cell anaemia trait (i.e. both parents are AS genotype, or one of the parent has SS genotype).  

It results in an abnormality in the oxygen-carrying protein haemoglobin (hemoglobin S) found in red blood cells. Thus, causing the affected red blood cells to be rigid and have sickle-like shape.

The haemoglobin gene is located at chromosome 11, Sickle-cell anaemia occurs when a person inherits two abnormal copies of the haemoglobin gene, one from each parent or both abnormal copies from one parent, resulting to SS genotype individual.

Symptoms of sickle-cell anaemia typically begin around 5 to 6 months of age, following the replacement of fetal haemoglobin with adult haemoglobin.  A number of health problems may develop, such as;

  • Pain attacks ("sickle-cell crisis")
  • Anemia
  • Swelling in the hands and feet
  • Bacterial infections
  • Stroke 

The incidence of sickle cell anaemia as of 2015 by (Global Burden of Disease Study 2015) about 4.4 million people have sickle-cell anaemia, while an additional 43 million have sickle-cell trait. About 80% of sickle-cell disease cases are believed to occur in sub-Saharan Africa (Rees, et al, 2010).  In 2015, it resulted in about 114,800 deaths (Global Burden of Disease Study 2015).

Management of people with sickle-cell anaemia may include;

  • Infection prevention with vaccination and antibiotics
  • High fluid intake
  • Folic acid supplementation throughout the person’s life span
  • Pain medication
  • Blood transfusion and the medication hydroxycarbamide (hydroxyurea).
  • Transplant of bone marrow cells

At JAFC we believe that in this day and age, no couple should bring forth a child with SS genotype. Take advantage of the science & technology available to avoid it. Couples with AS genotype should by all means avoid giving birth to a child with SS genotype. It is worth preventing.

 

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