Sickle Cell Anaemia

Sickle Cell

Sickle cell anaemia is an inherited condition where the red blood cells which carry oxygen around our body develop into an abnormal shape. Instead of being round they form into a sickle shape i.e. a semi-circle. These abnormally shaped blood cells are sticky and stiff and they can block blood flow into your tissues and organs. The reduction in blood flow to the organs can cause pain, organ damage  and increase a person’s chance of developing infections.

Sickle cell affects millions of people worldwide and is significantly more common in people who are of African ancestry.

Causes 

Sickle cell anaemia is an inherited disease. People with sickle cell will have inherited one copy of the gene from their father and another copy of the gene from their mother. If you only inherit one of the sickle cell genes you will not develop full-blown sickle cell, you will be a carrier of the sickle cell gene, but you can pass this gene down to your children. If you are a carrier you will not experience any symptoms as you have enough normal blood cells to ensure that your blood flows adequately throughout your body but as a carrier you do have to be careful in situations where oxygen levels are low like when mountain climbing and scuba diving.  Carriers also have some protection against malaria.

Complications

People with sickle cell may experience a number of symptoms and complications; everyone is different so each individual will have a different experience with sickle cell.  You may experience any of the following:

  • Episodes of pain (Sickle cell crisis)

This is the most common complication of sickle cell and occurs when sickle shaped blood cells block the blood vessels that supply a particular tissue. This means oxygen is unable to get to tissues and therefore they are starved of their energy source; this causes tissues to become damaged and then die – this causes the pain. Repeated episodes eventually lead to organ damage. The episodes can affect your ribs,  spine, pelvis, abdomen, chest, legs and arms.

  • Anaemia

Anaemia is a decrease in the number of red blood cells in the body. Sickle cells do not live for a long time and when they die they are not replaced quickly enough so sickle cell sufferers will have a low red blood cell count which can lead to:

  • Shortness of breath
  • Feeling tired all the time
  • An irregular heart beat
  • Infections

Children with sickle cell are prone to developing infections such as:

  • Pnemonia
  • Bone infection
  • Meningitis

If your child has sickle cell they may need to have some additional vaccinations or be given antibiotics as a precaution.

  • Jaundice and Gallstones

The rapid break down of the sickled cells can lead to the build up of a  yellow waste product called bilirubin. This causes the yellowing of the skin and the whites of the eye, this is called jaundice.  The bilirubin can also solidify forming gallstones.

  • Stroke and Acute Chest Syndrome

If sickle cells block the blood flow to the brain this can lead to a stroke, and if it blocks the blood flow to the lungs this can result in acute chest syndrome where patients experience chest pain and difficulty breathing.

Treatment

Sickle cell cannot be cured but a treatment plan is developed to help sickle cell sufferers manage their disease. The aim of any treatment is to:

  • Prevent episodes of pain
  • Relieve pain
  • Minimise any complications
  • Treat any symptoms

Diagnosis

Sickle cell is diagnosed through a blood test.  When a person has sickle cell this will become apparent when they are a child.  If you are worried that your child has sickle cell you can arrange for them to have the blood test by making an appointment to see your GP or family doctor.  For couples who are expecting a baby and may be concerned that their baby may inherit sickle cell, your unborn baby can be tested from eleven weeks into your pregnancy. You can speak to your GP or obstetrician about this.

More Information

For more information about sickle cell, please visit the following websites: