Sickle cell Anaemia
Sickle cell anemia / sickle cell disease (SCD), is a genetic disease of the red blood cells (RBCs).
In a normal circumstance, RBCs resemble discs in shape, thus give them the flexibility to travel through even the smallest blood vessels. However, when this disease occurs, the RBCs have an abnormal crescent shape resembling a sickle. This makes them sticky and rigid and prone to getting trapped in small vessels, which blocks blood from reaching different parts of the body. This can cause pain and tissue damage.
Types of Sickle Cell Disease
Several types of sickle cell disease are known. However, the most common ones are:
- Sickle Cell Anemia (SS),
- Sickle Hemoglobin-C Disease (SC),
- Sickle Beta-Plus Thalassemia,
- Sickle Hemoglobin-D Disease and
- Sickle Beta-Zero Thalassemia.
Sickle Cell Anemia (SS): When a child inherits one substitution beta globin genes (the sickle cell gene) from each parents, the child has Sickle Cell Anemia (SS).
Sickle Hemoglobin- C Disease (SC): Individuals with Sickle Hemoglobin-C Disease (SC) have a slightly different substitution in their beta globin genes that produces both hemoglobin C and hemoglobin S.
Sickle Beta-Plus Thalassemia: Sickle beta-plus thalassemia affects beta globin gene production. The size of the red blood cell is reduced because less beta protein is made. If inherited with the Hb S gene, you will have haemoglobin S beta thalassemia. Symptoms are not as severe.
Sickle Hemoglobin-D Disease: Through research, hemoglobin D, which is a different substitution of the beta globin gene, has been found to interact with the sickle hemoglobin gene.
Sickle Hemoglobin-O Disease: Hemoglobin O, another type of substitution in the beta globin gene, also interacts with sickle hemoglobin.
Causes of sickle cell anemia
The basic cause of sickle cell anemia involves hemoglobin, a component of the red cells in the blood. The hemoglobin molecules in each red blood cell carry oxygen from the lungs to organs and tissues and then bring back carbon dioxide for removal by the lungs. In sickle cell anemia, this process is disrupted. After the hemoglobin molecules give up their oxygen, some of them may cluster together and form long, rod-like structures that become stiff and assume a sickle shape and gene mutation is also leads to inherited one gene for hemoglobin S from each parent.
Symptoms of sickle cell anemia
- Yellow eyes
- Painful swelling of hands and feet
- Frequent pain episodes
- Stunted growth
- Anemia (looking pale)
- Dark urine
Treatment and medications
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Prevention of sickle cell anemia
• Drinking plenty of water (hydration).
• Avoiding extremely hot or cold temperatures.
• Avoiding places or situations with low oxygen, such as high altitudes, military boot camp, or strenuous athletic training.
• Getting plenty of rest and taking frequent breaks during exercise.