Sickle-cell anaemia is a disease passed down through families in which red blood cells form an abnormal crescent shape. (Red blood cells are typically shaped like a disc.) The distorted red blood cells are shaped like crescents or sickles. These sickle or crescent-shaped blood cells are fragile and are unable to deliver adequate oxygen to the body’s tissues. They clog easily in the smaller blood vessels, and even break easily. They can experience a disruption in the healthy flow of blood.
This condition is inherited from both parents. Sickle-cell disease is much more common in people belonging to the Mediterranean and African heritage. It is also seen in people from South and Central America, the Caribbean, and the Middle East.
It occurs when an individual inherits the Hemoglobin (S) gene from a parent and the haemoglobin (A) gene, which is normal, from another parent. Such an individual has the sickle-cell trait. However, those who the sickle-cell trait do not experience the symptoms of sickle-cell anaemia.
The symptoms of sickle-cell Anaemia show up in childhood only, especially between the ages of 5 and 15. Pain is a significant indicator of this disease. Most people begin having pain in their abdomen, hips, knees, and elbows. A severe pain crisis may require hospitalization until the pain is under control. Some of the other common symptoms include:
- Attacks of abdominal pain
- Bone pain
- Delayed growth and puberty
- Ulcers on the lower legs (in adolescents and adults)
Tests to detect Sickle-cell Anaemia
Complete blood count (CBC) – this test screens for anaemia, a condition that occurs when not enough oxygen is delivered to the cells of the body due to the presence of abnormal haemoglobin. To cure this, HPLC or high-performance liquid chromatography, Hemoglobin electrophoresis, and even DNA testing are used to know what type of Hemoglobin is present in one’s blood.
Patients with the disease need ongoing treatment, even when they are not having a painful crisis. They should take supplements of folic acid (essential for producing red blood cells) because red blood cells are turned over so quickly. The purpose of treatment is to manage and control symptoms and to limit the frequency of crises.
During a sickle cell crisis, you may need specific treatments. Pain is then treated with painkillers and intake of fluids. Pain should not be ignored. Patients will need medication in large doses. Hydroxyurea (Hydrea) is a drug some patients use to reduce the number of pain episodes (including chest pain and difficulty breathing). It does not work for everyone.
In such a situation, vaccines and antibiotics are given to the patients to avoid bacterial infections. Such problems are common in children who suffer from sickle-cell Anaemia. Blood transfusions are also recommended and may be done on a regular basis to help avoid strokes.
Today the life expectancy of patients with this disease is 50 years or more compared to a mere 14 years before.