Sickle cell disease

Last Updated on by FRCEM Intermediate

Overview

Sickle cell disease is caused by HbS hemoglobinopathy which produces rigid, distorted and dysfunctional erythrocytes called sickle cells

Pathophysiology

Vaso-occlusive crisis – Abnormal beta-globin chain that polymerizes when deoxygenated- distorting the erythrocyte into a sickle shape -clumping of sickle cells leads to the impaired blood supply

Types of sickle cell disease

  • sickle cell anemia (usually homozygous SS genotype)
  • sickle beta thalassemia
  • sickle HbC disease

Precipitating factors to sickling

  • Hypoxia e.g. due to exertion, high altitude
  • Dehydration
  • Pregnancy
  • Cold
  • Psychological stress
  • Intercurrent illness

Types of sickle cell crisis presentations

  • fever
    • consider veno-occlusive disease, acute chest syndrome, osteomyelitis, local or systemic infection
  • Acute painful crisis –  veno-occlusive disease
    • assume this is the cause of any painful presentations
    • Bone infarction – painful swollen joints, dactylitis
  • Acute abdominal complications
  • Acute anaemia
    • Acute splenic sequestration
      • Acute drop in Hb, high reticulocyte count, splenomegaly
    • Acute aplastic crisis secondary to infection with human parvovirus B19
      • Acute drop in Hb, low/absent reticulocyte count
    • Acute hepatic sequestration
  • Acute chest syndrome
    • Infection and/or infarction
    • assume if hypoxia + chest pain
  • Acute osteomyelitis
    • Most commonly caused by Salmonella species, Gram-negative enteric bacteria, and Staphylococcus aureus
  • Acute priapism
  • Acute renal impairment
  • Acute stroke
    • Infarction or haemorrhage

Management

  • Analgesia-aggressive pain management e.g. paracetamol, +/- NSIADs, opiates (Morphine
  • Oxygen – O2 and aggressive pain management (prevent hypoventilation)
  • IV fluids – rehydrate with  IV fluids
  • Avoid triggering factors
  • Blood transfusions
    • For emergency top-up in aplastic crisis or acute splenic sequestration
    • For emergency exchange transfusion in acute stroke, acute chest syndrome, severe sepsis, acute hepatic sequestration or progressive intrahepatic cholestasis
  • Antibiotics
  • Consult hematology
  • Refer to Pediatric team

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