Sickle Cell Anaemia

What is sickle cell anaemia?

Sickle cell anaemia is a blood condition that mostly affects people of African descent. It is inherited from parents and changes the shape of red blood cells from round and flexible to a stiff, crescent or banana shape. These abnormally shaped cells can block blood flow, causing pain and damage to body organs.

How common is sickle cell anaemia in Africa?

Sickle cell disease (SCD) is a major health issue in Africa. According to the world health organization (WHO), about 1,000 babies are born every day in Africa with SCD, and over 66% of all cases worldwide occur in Africa. Countries like Nigeria, the Democratic Republic of Congo, and Ghana have the highest numbers. Unfortunately, many children with SCD die before the age of five due to a lack of proper treatment.

Sickle cell anaemia vs. sickle cell disease

Sickle cell disease (SCD) refers to a group of inherited blood disorders, with sickle cell anaemia (SS genotype) being the most severe form. Other forms include SC, SE, and sickle beta-thalassemia.

  • People who inherit one normal gene and one sickle gene are called carriers (sickle cell trait). They do not have the disease but can pass it to their children.
  • It is wrong to call people with sickle cell anaemia “sicklers.” The correct term is “persons living with sickle cell disease.”

Symptoms of sickle cell anaemia?

Symptoms usually start appearing in babies as early as six months old and can include:

  • Extreme tiredness (fatigue)
  • Yellowing of the eyes and skin (jaundice)
  • Swelling and pain in hands and feet
  • Frequent infections
  • Bedwetting due to kidney problems
  • Severe pain in different parts of the body (sickle cell crisis)

Types of sickle cell disease?

SCD comes in different forms based on the types of abnormal haemoglobin inherited:

  • Hemoglobin SS disease: The most common and severe form, caused by inheriting the sickle cell gene from both parents.
  • Hemoglobin SC disease: A milder form but still causes significant symptoms.
  • Hemoglobin S beta-thalassemia: Less severe but can still cause complications.
  • Other rare forms: Hemoglobin SD, SE, and SO.

Who is at risk?

If both parents carry the sickle cell gene, there is a 25% chance their child will be born with sickle cell anaemia. SCD is more common in people from Africa, India, the Mediterranean, and Saudi Arabia due to historical exposure to malaria, which led to the survival of carriers.

Complications of sickle cell anaemia?

SCD can lead to serious health problems, including:

  • Severe anaemia: Rapid breakdown of sickle cells reduces oxygen supply.
  • Painful swelling of hands and feet: Called “hand-foot syndrome.”
  • Infections: The spleen (which fights infections) is often damaged, increasing the risk of serious illnesses.
  • Delayed growth and puberty: Due to reduced oxygen supply to the body.
  • Stroke: Blocked blood flow to the brain.
  • Blindness: Due to damaged eye blood vessels.
  • Heart and lung problems: Can lead to heart failure or lung disease.
  • Priapism (in men): A painful, prolonged erection that can lead to infertility.

How is sickle cell anaemia diagnosed?

A simple blood test called haemoglobin electrophoresis confirms the presence of sickle cell disease. Many African countries are now introducing newborn screening to detect SCD early.

How is sickle cell anaemia treated?

Although there is no universal cure, several treatments help manage the condition:

  • Staying hydrated: Drinking plenty of water prevents sickle cell crises.
  • Pain relief medications: To manage pain episodes.
  • Blood transfusions: To increase oxygen supply in severe cases.
  • Vaccinations and antibiotics: To prevent infections.
  • Hydroxyurea medication: Helps reduce pain episodes and hospital visits.
  • Bone marrow transplant: The only known cure but not widely available.

Living with sickle cell disease?

Many people with SCD live long and fulfilling lives with proper care. Here’s how:

Regular check-ups: Seeing a doctor regularly helps prevent complications.

Eating healthy: A balanced diet with plenty of fruits, vegetables, and protein.

Avoiding extreme temperatures: Both cold and hot weather can trigger a crisis.

Reducing stress: Managing stress levels through relaxation and support groups.