This refers to a series of disorders that affect haemoglobin. People with this condition have haemoglobin molecules called haemoglobin S which distort the shape of red blood cells from biconcave to a sickle or crescent shape, hence the name. The sickle shape (dominant) causes these red blood cells to die early, leaving a small amount of healthy red blood cells (sickle cell anaemia) and can even cause blockage of blood vessels when they come together causing immense pain (sickle cell crisis).
Signs and Symptoms
Include;
- A low number of RBCs leading to dizziness and fatigue
- Periodic episodes of pain
- Repeated infections
- Dark- coloured urine (like coke)
- Delayed growth and development
- Yellowing of the skin and eyes (jaundice)
Complications
- Organ damage due to oxygen deprivation
- Pulmonary hypertension
- Heart failure
Treatment
- Medication (folic acid, pain relievers)
- Blood transfusions
- Bone marrow transplant (rare)