Sickle cell anaemia

Sickle cell anaemia

Why in news :

  • Finance Minister Nirmala Sitharaman said that the government will work in “mission mode” to eliminate the condition of sickle cell anaemia in by 2047.
  • India is the second-worst affected country in terms of predicted births with sickle cell anaemia i.e. chances of being born with the condition.

What is sickle cell anaemia ?

  • Sickle cell disease (SCD) is a group of blood disorders typically inherited.
  • The most common type is known as sickle cell anaemia.
  • It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells.

  • This leads to a rigid, sickle-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6 months of age.
  • Haemoglobin which is tasked with carrying oxygen to all parts of the body, has four protein subunits — two alpha and two beta.
  • In some people, mutations in the gene that creates the beta subunits impact the shape of the blood cell and distorts it to look like a sickle.

  • A round red blood cell can move easily through blood vessels because of its shape but sickle red blood cells end up slowing, and even blocking, the blood flow.
  • Moreover, sickle cells die early, resulting in a shortage of red blood cells that deprive the body of oxygen.
  • These obstructions and shortages may cause chronic anaemia, pain, fatigue, acute chest syndrome, stroke, and a host of other serious health complications.
  • Without treatment, quality of life is compromised and severe cases can become fatal in the initial years of life.

Who are more vulnerable ?

  • Research and screening programmes have found that the prevalence of haemoglobinopathies disorders of the blood is more common among tribal populations than non-tribal communities in India.
  • Research has shown that SCA is prevalent in communities residing in areas where malaria is endemic.
  • Around the middle 1940s, doctors found that those with sickle red blood cells were more likely to survive malaria.
  • Those with the trait in some African countries were found to be potentially resistant to lethal forms of malaria and had a survival advantage.
  • The sickle cell trait thus gave an evolutionary advantage, offering immunity to some people during malaria epidemics.
  • In India, States and UTs with tribal populations contribute a significant malaria case load.
  • Additionally, the documented prevalence of SCA is higher in communities that practice endogamy, as the chances of having two parents with sickle cell trait is higher.

Treatment of sickle cell anaemia :

  • Sickle cell anaemia is a genetic disorder, making complete “elimination” a challenge that requires major scientific breakthrough.
  • The only cure comes in the form of gene therapy and stem cell transplants both costly and still in developmental stages.
  • Blood transfusion, wherein red blood cells are removed from donated blood and given to a patient, is also a trusted treatment in the absence of permanent cures.

Syllabus : Prelims + Mains; GS3 – Health

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