What is Sickle Cell & Thalassaemia?
Sickle cell disease (SCD) and thalassaemia are serious inherited conditions that affect red blood cells. They are passed down from parents through genes and cannot be "caught" like an infection. These disorders alter how oxygen is carried in the body, impacting health significantly.
Differences Between Sickle Cell & Thalassaemia
Both sickle cell and thalassaemia affect haemoglobin, the oxygen-carrying part of blood:
- Sickle Cell: Affects the quality of haemoglobin, causing red blood cells to become stiff, sickle-shaped, and prone to blocking small blood vessels.
- Thalassaemia: Affects the quantity of haemoglobin, resulting in fewer red blood cells to transport oxygen effectively.
Genetics and Inheritance
Sickle cell and thalassaemia are genetic diseases determined by genes inherited from parents. A child must inherit the sickle cell gene from both parents to have SCD, typically when both are carriers (sickle cell trait) or one has SCD and the other is a carrier. Genes influence traits like height, skin color, and susceptibility to these conditions.
Symptoms & Complications
Symptoms of SCD often emerge after 5 months of age and vary over time, leading to complications such as:
- Recurrent infections
- Anemia
- Periodic episodes of pain
- Yellowing of eyes and skin
- Organ damage due to inadequate oxygen
- Heart failure
- Stroke
Normal red blood cells circulate for 120 days, while sickled cells last only 20-30 days and can get stuck in small vessels, causing chronic pain.
Treatment for Sickle Cell
SCD is often treated with blood transfusions, which increase normal red blood cells, deliver oxygen, unblock vessels, and alleviate pain. Treatments are improving, allowing people to live longer with proper care. The NHS notes that sickle cell patients requiring regular transfusions need about 100 blood donors per year to survive.
Living with Sickle Cell & Thalassaemia
Living with these conditions involves challenges such as:
- Finding the right care and support
- Managing school life
- Securing suitable housing
- Facing stigma and lack of understanding
- Getting employer support
- Frustration of overprotection when well
Myths & Facts
Myth
People with sickle cell or thalassaemia will die young.
Fact
Treatments are improving rapidly, enabling people to live long lives with proper care.
Facts & Figures
SCD predominantly affects people of African and Caribbean ancestry (1 in 4 West Africans, 1 in 10 Afro-Caribbeans). In England:
- 450,000 people carry an unusual gene for sickle cell or thalassaemia.
- 13,000 have sickle cell disease; 700 have beta thalassaemia major.
- 2016/17: 274 babies identified with SCD, 8,530 as carriers.
- 2020/21: 24,573 hospital admissions due to SCD, 35% emergencies, average age 29, 25% under 18.
- 2021/22: 84,489 red cell units requested for sickle cell patients (up from 55,652 in 2015/16).
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