Learning objectives

• Sings and symptoms and management of sickle cell disease

Definition and mechanisms

• Sickle cell disease is a group of inherited hemoglobinopathies 
• The most common type is known as sickle cell anemia, other types are sickle cell anemia (HbSS) and the double heterozygote conditions sickle C (HbSC) and sickle thalassemia (HbSThal) 
• The average life expectancy is 40 to 60 years
• The dominant hemoglobin (Hb) is replaced by the unstable hemoglobin S resulting in the deformation of the red cell membrane into the characteristic sickle shape
• These sickle cells become rigid and aggregate, thereby occluding small blood vessels and leading to tissue infarction
• Hemolysis occurs due to the damaged cell membrane leading to an inflammatory response
• The major features of sickle cell disease are chronic anemia and the occurrence of sickle cell ‘crises’ in which multiple episodes of tissue infarction occur
• Crises can be provoked by temperature changes, stress, dehydration, and high altitude

Signs and symptoms

• Symptoms of sickle cell anemia usually appear around 6 months of age
• Vary from person to person and may change over time
• Anemia
• Episodes of pain
• Swelling of hands and feet
• Frequent infections
• Delayed growth or puberty
• Vision problems

Complications

• Stroke
• LV hypertrophy
• High-output cardiac failure (anemia)
• Myocardial infarction without coronary artery disease
• Acute chest syndrome
• Pulmonary hypertension
• RV hypertrophy
• Cor-pulmonale
• Renal failure or infarction
• Splenic sequestration
• Blindness
• Leg ulcers
• Gallstones
• Priapism
• Deep vein thrombosis
• Pregnancy complications
• Acute pain crisis

Management

• Avoid precipitants of sickle cell crisis
  • Hypoxia
  • Vascular stasis
  • Hypothermia
  • Hypovolemia/hypotension
  • Acidosis

Suggested reading

• Pollard BJ, Kitchen, G. Handbook of Clinical Anaesthesia. Fourth Edition. CRC Press. 2018. 978-1-4987-6289-2.
• Wilson, M., Forsyth, P., Whiteside, J.. Haemoglobinopathy and sickle cell disease. Continuing Education in Anaesthesia Critical Care & Pain. 2010. 10, 24–28.

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