Pathophysiology
Autosomal recessive hemoglobinopathy. HbS polymerizes when deoxygenated, RBCs sickle, vaso-occlusion, hemolysis, ischemia. Shortened RBC lifespan (10-20 days), chronic hemolytic anemia. Common in African descent (1 in 365).
Acute Complications
Vaso-occlusive pain crisis (most common): ischemic pain in bones, chest, abdomen. Triggers: cold, dehydration, stress, infection. Acute chest syndrome: new infiltrate + fever/chest pain — leading cause of death. Splenic sequestration. Stroke. Aplastic crisis. Fever: emergency (functional asplenia).
Nursing Management
Pain crisis: aggressive opioids (morphine, hydromorphone), hydration 1.5x maintenance, oxygen if hypoxemic, warmth. Acute chest: antibiotics, transfusion. Hydroxyurea reduces crisis frequency. Pneumococcal vaccine critical. Prophylactic penicillin until age 5.
Important Notes
- Fever in child with SCD is medical emergency — functional asplenia puts them at high risk for sepsis.
- Acute chest syndrome is leading cause of death in SCD — recognize and treat immediately.
Clinical Tips
- Avoid cold and dehydration — common triggers for vaso-occlusive crises.
- Treat pain aggressively — opioid dependence is rare in acute sickle cell pain management.
Summary
Sickle cell disease requires aggressive pain management, infection prevention, transfusion therapy, and hydroxyurea to reduce crisis frequency.
