Sickle Cell Disease

Sickle Cell Disease Latest News

A recent study by the AIIMS Bhopal has stressed the need for early and advanced health screening in children suffering from sickle cell disease (SCD).

About Sickle Cell Disease (SCD)

• It is a group of inherited blood cell disorders that affect hemoglobin, the molecule in red blood cells (RBCs) that delivers oxygen to cells throughout the body. 
• SCD can cause episodes of severe pain and lead to life-threatening complications. 
• The most common and severe type of SCD is sickle cell anemia.
• How Does it Affect Blood Flow?
  • Normally, RBCs are disc-shaped and flexible enough to move easily through the blood vessels.
  • People with SCD have atypical hemoglobin molecules called hemoglobin S, which can distort RBCs into a sickle, or crescent, shape.
  • When RBCs sickle, they do not bend or move easily and can block blood flow to the rest of the body.
  • The sickle-shaped cells can also stick to vessel walls, causing a blockage that slows or stops the flow of blood.  
• What causes it?
  • The cause of SCD is a defective gene, called a sickle cell gene.
  • A person will be born with SCD only if two genes are inherited—one from the mother and one from the father.
  • If you are born with one sickle cell gene, it’s called sickle cell trait. People with sickle cell trait are generally healthy, but they can pass the defective gene on to their children. 
• Symptoms:
  • Signs and symptoms of sickle cell disease usually begin in early childhood. 
  • Early stage: Extreme tiredness or fussiness from anemia (low number of RBCs), painfully swollen hands and feet, and jaundice.
  • Later stage: Severe pain, anemia, organ damage, and infections.
• Treatments:
  • A bone marrow transplant (stem cell transplant) can cure SCD.
  • However, there are treatments that can help relieve symptoms, lessen complications, and prolong life.
  • Gene therapy is also being explored as another potential cure.
  • The UK recently became the first country to approve gene therapy treatment for SCD.

Source: NDTV

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