What are the typical symptoms of a sickle cell crisis?

Symptoms of Sickle Cell Crisis

Sickle cell crises present with severe pain (most commonly in the lumbar spine, abdomen, femoral shaft, and knees), often accompanied by fever, and may progress to life-threatening complications including acute chest syndrome, stroke, splenic sequestration, or priapism. 1, 2

Acute Pain Crisis (Vaso-Occlusive Crisis)

The hallmark symptom is severe pain affecting multiple body regions 1, 3:

• Most common locations: Lumbar spine (49%), abdomen (32%), femoral shaft (30%), knees (21%) 3
• Pain characteristics: Ranges from mild to excruciating; often bilateral in limbs and ribs 3
• Timing: Most frequently develops between 3 PM and midnight 3
• Associated symptoms: Fever is common even without infection 3

Abdominal Pain Presentations

• May include abdominal distention (31% of cases) 3
• Referred rib pain occurs in 26% of abdominal crises 3
• Can mimic acute surgical abdomen 4

Pediatric-Specific Manifestations

• Dactylitis (hand-foot syndrome) in infants: painful swelling of hands and feet 1
• Symptoms typically first appear near the end of the first year of life 5

Life-Threatening Acute Complications

Acute Chest Syndrome

This is a medical emergency characterized by 1:

• New segmental infiltrate on chest X-ray (may not be visible initially)
• Lower respiratory tract symptoms
• Chest pain and/or hypoxemia
• Can present with or without fever
• May deteriorate rapidly to pulmonary failure and death

Stroke

Any acute neurologic symptom beyond transient mild headache requires urgent evaluation 1:

• Hemiparesis
• Aphasia or dysphasia
• Seizures
• Monoparesis
• Severe headache
• Cranial nerve palsy
• Stupor or coma

Splenic Sequestration

Rapidly life-threatening, presenting with 1:

• Rapidly enlarging spleen (palpable on examination)
• Hemoglobin drop >2 g/dL below baseline
• Mild to moderate thrombocytopenia
• Can progress rapidly to shock and death
• Most common in children <5 years with HbSS and adolescents with HbSC

Priapism

Occurs in two forms 1:

• Stuttering episodes: <4 hours duration, often recurrent
• Severe acute ischemic episodes: ≥4 hours (irreversible tissue damage begins after 4 hours)
• Often starts during early morning hours

Other Acute Manifestations

Transient Aplastic Crisis

• Exacerbation of baseline anemia
• Reticulocyte count drops to <1%
• Usually caused by parvovirus B19 infection (often without characteristic rash) 1

Fever and Infection Risk

• Any fever requires urgent evaluation for potential bacteremia 1
• Increased susceptibility to pneumococcal sepsis and meningitis, especially in young children 5

Common Pitfalls

1. Fever in pain crisis does not necessarily indicate infection—it can be characteristic of the crisis itself 3
2. Abdominal pain may mimic surgical emergencies but is often vaso-occlusive in nature 4
3. Chest pain requires high suspicion for acute chest syndrome, which may not show infiltrates on initial imaging 1
4. Priapism lasting >4 hours is a urologic emergency requiring immediate intervention 1
5. Parents of young children should be taught daily spleen checks to detect enlargement early 1

Associated Symptoms Requiring Urgent Evaluation

Per guidelines, patients and families should seek immediate medical attention for 1:

• Pallor: potential severe anemia from hyperhemolysis, splenic sequestration, or aplastic crisis
• Difficulty breathing: potential severe anemia or acute chest syndrome
• Any neurologic symptoms: potential stroke