Risk factors for sickle cell disease and potential complications

Risk factors for sickle cell disease and potential complications

Risk factors for sickle cell disease and potential complications

Who is at higher risk of SCD?

Endemic malaria regions do appear to have a higher rate of sickle cell trait carriers due to the complex nature of both SCD and the infectious condition caused by the P. falciparum parasite. The two have a complex relationship, even though the red blood cells in the body are affected in different ways. Areas with a higher rate of carriers include Africa, India, Saudi Arabia and the Mediterranean.

One or more adults who carry the sickle cell trait place future biological children at risk of sickle cell disease. A blood test known as ‘haemoglobin electrophoresis’ can be performed to determine which type a parent may carry and likely pass on to a child.

What are the most common complications of SCD?

Complications can be severe and stem from the various parts of the body which are deprived of normal blood flow and oxygen.

  • Chronic anaemia (severe anaemia)
  • Hand-foot syndrome and leg (skin) ulcers
  • Delayed growth during childhood (delayed puberty or sexual maturation)
  • Splenic sequestration (a sudden and painful enlargement of the spleen – which can also shrink and cease to function at all - due to a blockage of the splenic vessels)
  • Seizures, stroke, coma and other neurological complications
  • Blindness and vision problems (retina damage)
  • Heart problems (heart failure, heart attack and abnormal heart rhythms)
  • Pulmonary hypertension and pulmonary fibrosis (scarring of the lungs) – lung disease
  • Priapism (in males) – this is a painful, lingering erection (without sexual stimulation and not relieved by ejaculation) that occurs when blood vessels in the penis are blocked. A long-lasting erection can occur for several hours at a time and this condition is regarded as a urologic emergency in order to avoid complications such as impotence.
  • Gallstones (due to high levels of bilirubin)
  • Damage to nerves and organs – kidneys, spleen and liver
  • Sickle chest syndrome (severe variation of sickle cell crisis) or acute chest syndrome – cough, mucus or sputum production, fever, low blood oxygen levels and shortness of breath are common signs other than severe pain in the chest area.

Another serious risk individuals with SCD should be aware of is the potential for the development of fifth disease (‘slapped cheek disease’), caused by the parvovirus (which is an airborne infection). Parvovirus can result in a temporary halt in the production of new red blood cells. For a person with sickle cell disease, this can become a life-threatening situation as it can lead to a severe case of anaemia (aplastic anaemia).

A person with SCD will need to be well aware of airborne infections and their contagious consequences, and do their utmost to reduce their exposure to these conditions so as to avoid serious complications that will arise if contracted.

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