Sickle cell disease

Sickle cell disease is an inherited condition which affects the structure of the red blood cells and their ability to transport oxygen around the body. 

Normal red blood cells contain haemoglobin which gives them the red colour. These cells perform their function because they are flexible and are round in shape, which allows them to move freely within blood vessels. In sickle cell disease, many of the red cells are curved in shape like a “sickle”. This makes them rigid, easily getting stuck within blood vessels and causing symptoms such as pain.

What causes sickle cell disease?

Sickle cell disease is an inherited condition which means it is passed down from parents to a child. If both parents carry the gene for sickle cell disease, then there is a chance of passing it down to the child. However, it is important to note that a child can only inherit the condition if both parents carry the gene for the condition. 

How can I test for sickle cell disease?

To test for the presence of sickle cell disease, one needs to test for one’s haemoglobin genotype. There are different genotypes namely AA, AS, AC, SC, SS, and CC. Sickle cell disease is associated with the SS genotype. Prospective parents ought to check their genotype so as to determine their chances of having a child with sickle cell disease.

What are the symptoms of sickle cell disease?

  • Low blood cell count (Anaemia). Because blood cells affected by sickle cell disease do not last long in the blood, people with the condition usually experience anaemia which in itself can cause symptoms such as tiredness, difficulty breathing.
  • Pain crisis. Sickle cell disease can often cause periodic pain in different parts of the body, particularly the hands and legs, chest and abdomen. This pain crises usually occurs because the sickle-shaped blood cells get stuck within the blood vessels, blocking off the blood supply.
  • Growth delay. Children with sickle cell disease often experience slow growth as compared to other children of the same age. This is because there is no adequate number of red cells to transport oxygen and nutrients around the body.
  • Repeated infections. Sickle cell disease makes people more prone to repeated infections, so it is important that children are sufficiently immunized and monitored by a doctor.
  • There could also be many other symptoms which can vary from one individual to another.

How is sickle cell disease diagnosed?

If you suspect that a child has sickle cell disease, speak to a doctor who will examine the child and request blood tests to check the child’s genotype.

What is the treatment for sickle cell disease?

Unfortunately, sickle cell disease is a lifelong condition which requires careful daily management. The following measures are important to prevent periods of sickle cell crisis.

  • Keeping up-to-date with recommended immunizations helps to reduce the chance of having infections which can trigger a sickle cell crisis.
  • Keeping well hydrated at all times as this allows the blood to flow better especially on very warm and dry days.
  • Having regular checks with the doctor will ensure that other illnesses are picked up early and treated.
  • Some children might need to take regular antibiotics in order to prevent them from getting infections.

What complications can arise from sickle cell disease?

Sickle cell disease, unfortunately, is a serious medical condition which can sometimes lead to complications such as the following.

  • Stroke
  • Organ damage
  • Blindness
  • Leg ulcers
  • Chest crisis

Is there any support for people with sickle cell disease?

There are a number of organizations that provide information and support for people with sickle cell disease and their families.