Sickle cell anaemia

• What is sickle cell anaemia?

  Autosomal recessive condition -> synthesis of abnormal haem chains
• When does sickle cell anaemia become symptomatic and why?

  In 4-6 months when abnormal HbSS molecules take over from fetal HbF
• When does sickle cell crisis occur?

  Deoxygenated HbS molecules polymerise -> RBCs sickling -> fragile sickle cells haemolyse -> block small vessels = infarction
• What are some signs and symptoms of sickle cells anaemia?

  Anaemia symptoms

  Inc risk of serious infections

  Painful episodes lasting days/weeks (sickle cell crisis)
• What are some risk factors for sickle cell anaemia?

  African descent
• What is the definitive diagnostic test for sickle cell anaemia?

  Haemoglobin electrophoresis
• How does crizanlizumab help sickle cell anaemia?

  Prevents blood cells sticking to inside walls of vessels = reduces pain
• How does hydroxyurea therapy help sickle cell anaemia?

  Increases amount of fetal Hb = milder symptoms
• How can sickle cell anaemia be managed surgically?

  Regular blood transfusions (for worse symptoms)

  Emergency blood transfusions (severe anaemia)

  Stem cell/bone marrow transplant (curative)
• How is sickle cell crisis managed?

  Analgesia (opiates)

  Hydration

  Oxygen

  Antibiotics (if infection)

  Blood transfusion
• How is chronic sickle cell anaemia managed?

  Hydroxyurea

  Vaccine every 5 years (pneumococcal polysaccharide)