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Sickle-cell disease
Why is it in news?
- Researchers are testing strategies for correcting the problem at the genetic level.
- Patients under observation who have endured an illness that causes excruciating bouts of pain, strokes and early death, no longer show signs of the disease.
More in news
- Alternative to present techniques:
- At present the only remedy for sickle-cell disease is a rarely used, dangerous and expensive option- Bone marrow transplant.
- Now gene therapy which is not a simple or inexpensive, but it could change the lives of tens of thousands of people.
- More prevalence in sub-Saharan Africa: The disorder is most common in sub-Saharan Africa, where an estimated 70% of children with it die before adulthood.
Sickle cell disease
- What is Sickle cell disease?
- Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body.
- People with this disorder have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.
- Crises condition:
- The misshapen cells get stuck in blood vessels, causing strokes, organ damage and episodes of agonizing pain- called crises- as muscles are starved of oxygen.
- Children usually return to normal between crises, but teenagers and adults may suffer chronic pain.
- The misshapen cells don’t survive long in the blood- 10 to 20 days, compared to the usual 120 days.
- Signs and symptoms of sickle cell disease:
- It usually begins in early childhood.
- Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain.
- The severity of symptoms varies from person to person. Some people have mild symptoms, while others are frequently hospitalized for more serious complications.
- What causes sickle cell disease?
- The cause of SCD is a defective gene, called a sickle cell gene.
- People with the disease are born with two sickle cell genes, one from each parent.
- Person 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.
Source
The Hindu, nih.gov