ABOUT THE DISEASE

Sickle cell disease also known as sickle cell anemia is caused by a type of faulty hemoglobin in red blood cells. Normal red blood cells are round. In people with sickle cell anemia, hemoglobin – a substance in red blood cells – becomes defective and causes the red blood cells to change shape from disc like shape to a sickle or crescent moon like shape. The faulty hemoglobin is called hemoglobin S, (HbS) and it replaces normal hemoglobin called: hemoglobin A (HbA). Hemoglobin is responsible for carrying oxygen in the blood. The differently shaped cells don`t move well through the smallest blood vessels. This can stop or slow blood flow to parts of the body, causing less oxygen to reach these areas. The sickle cells also die earlier than normal blood cells, which can cause a shortage of red blood cells in the body.

SYMPTOMS

People with sickle cell anemia can have painful episodes called crises, when sickle cells block blood flow to the organs. These can last from hours to days. Crises can cause pain in the lower back, leg, joints, and chest.

• • Swollen hands or feet
• • Sudden paleness of the skin or nail beds
• • Yellow color of the skin or eyes
• • Fever due to some infection
• • Swelling in the abdomen
• • Sudden tiredness
• • Erection of the penis that won`t go away due to blockage in small blood vessels
• • Trouble hearing or seeing
• • Weakness on one side of the body or a sudden change in speech due to a mild stroke
• • Headache
• • Trouble breathing
• • Joint, stomach, chest or muscle pain, or limping

CAUSES

This is a hereditary disease, which means that the disease is passed on to kids from their parents as part of their genetic makeup. Parents cannot give sickle cell anemia to their children unless they both have the faulty hemoglobin in their red blood cells. It is not necessary for both the parents to be affected by this disease, they could also be carrying a sickle cell trait (People with sickle cell trait have enough normal hemoglobin in their red blood cells to prevent the cells from showing any symptoms). One in four such kids with both parents carrying a sickle cell trait are affected by this disease.

DIAGNOSIS

- Sickle cell test

TREATMENT METHODS

The treatment plan revolves around managing and limiting the number of crises. Folic acid supplements are prescribed as it helps make new red blood cells.

Treatment for a sickle cell crisis includes:
- Blood transfusions (may also be given regularly to prevent stroke)
- Pain medicines
- Plenty of fluids
- Antibiotics to prevent infections

And in case to case basis might require a kidney transplant or a hip replacement or any other treatment related to the affected organ.

Bone marrow transplants can cure the disease in a small number of people, but this treatment is not an option for most people.

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