What are the signs and symptoms of a sickle cell crisis in a patient with sickle cell disease?

Signs and Symptoms of Sickle Cell Crisis

Sickle cell crisis presents with acute, severe pain that is diagnosed entirely based on the patient's self-report, without requiring any specific laboratory abnormalities, and commonly affects the back, legs, knees, arms, chest, and abdomen. 1

Primary Clinical Manifestations

Pain Characteristics

• Pain is the hallmark symptom, typically described as throbbing, sharp, dull, or stabbing in character 2
• Pain severity averages 9.5/10 on visual analog scale upon presentation, indicating excruciating intensity 2
• Pain location varies by age: fingers (dactylitis) in infancy; long bones, sternum, ribs, or back in older children and adults 1
• Pain may be localized to one area, involve multiple sites simultaneously, be diffuse throughout the body, or migrate between locations 3
• Episodes occur very abruptly and are often localized around joints 4

Vaso-Occlusive Crisis Features

• Chronic hemolytic anemia is present at baseline, with typical hemoglobin levels between 60-90 g/L in severe disease 5
• Painful vaso-occlusive crises result from micro-vessel occlusion causing bone marrow necrosis, edema, and increased pressure 4
• Approximately 70% of all patients with sickle cell disease experience pain crises 4

Life-Threatening Complications to Recognize

Acute Chest Syndrome

• Occurs in more than 50% of hospitalized patients with vaso-occlusive crisis and is a leading cause of death with up to 13% all-cause mortality 1, 6
• Presenting signs include: new segmental infiltrate on chest radiograph, lower respiratory tract symptoms, chest pain, and/or hypoxemia 6
• Any patient presenting with chest pain requires urgent exclusion of acute chest syndrome through chest X-ray 1, 6

Neurological Complications

• Any acute neurologic symptom other than transient mild headache requires urgent stroke evaluation 6
• Common presentations include hemiparesis, aphasia, seizures, severe headache, cranial nerve palsy, stupor, or coma 6
• Stroke occurs in up to 10% of children with sickle cell disease 6

Splenic Sequestration Crisis

• Characterized by rapidly enlarging spleen and hemoglobin decrease >2 g/dL below baseline 6
• Can progress rapidly to shock and death 6
• Distinguished from typical vaso-occlusive crisis by the significant drop in hemoglobin 1

Infectious Complications

• Fever (≥38.0°C) or signs of sepsis require immediate blood cultures and antibiotic initiation 6
• Patients are particularly vulnerable to gram-negative sepsis, including urinary tract infection, biliary sepsis, and non-typhi salmonella infection due to hyposplenism 6

Abdominal Manifestations

• Abdominal pain is an important component of vaso-occlusive crises and presents a substantial diagnostic challenge 7
• Often attributed to micro-vessel occlusion and infarcts of mesentery and abdominal viscera 7
• Abdominal pain from sickle crisis is often indistinguishable from acute cholecystitis, acute pancreatitis, hepatic infarction, ischemic colitis, or acute appendicitis 7

Associated Systemic Features

• Acute and chronic end-organ damage develops progressively 5
• Patients may present with priapism, characterized by laboratory markers including lower hemoglobin, elevated lactate dehydrogenase, bilirubin, aspartate aminotransferase, reticulocyte count, white blood cells, and platelet counts 1

Critical Diagnostic Pitfall

The most important pitfall is failing to trust the patient's self-report of pain. Patients with sickle cell disease and their caregivers often know their disease best, and their report of typical pain symptoms should be trusted and acted upon promptly without waiting for laboratory confirmation 1. Sickle cells are chronically present on peripheral smears and do not distinguish acute pain episodes from baseline 1.