Diagnosing Acute Pain Episodes in Sickle Cell Disease
The diagnosis of an acute pain episode in sickle cell disease is based entirely on the patient's self-report of pain—no laboratory tests or objective findings are required to make the diagnosis. 1, 2
Core Diagnostic Principle
The patient's self-report is the gold standard for diagnosis. If the patient states they are experiencing pain typical of their sickle cell crisis, aggressive treatment must be initiated immediately without waiting for confirmatory testing. 2
The American Academy of Pediatrics explicitly states that acute painful episodes are diagnosed clinically based on the patient's description of pain, without requiring any specific laboratory abnormalities. 1
There are rarely any objective physical findings even with severe pain, making the patient's report the only reliable diagnostic criterion. 2
Trust the Patient's Experience
Patients with sickle cell disease and their caregivers know their disease best. Their report of typical pain symptoms should be trusted and acted upon promptly. 1
Pediatric and adult providers must foster trust that patients will be heard when reporting pain, particularly to mitigate effects of systemic racism that causes mistrust of the healthcare system. 2
Delays in addressing and undertreatment of sickle cell pain are common pitfalls that must be actively avoided. 2
Typical Pain Patterns (Not Required for Diagnosis)
While not necessary for diagnosis, pain typically affects:
- Fingers in infancy (dactylitis). 1
- Long bones, sternum, ribs, or back in older children and adults. 1
Essential Evaluation to Exclude Life-Threatening Complications
Once pain is diagnosed based on patient report, the following evaluations are mandatory to exclude complications that require different management:
Immediate Assessment Required
Acute chest syndrome: Evaluate for new segmental infiltrate on chest radiograph with lower respiratory tract symptoms, chest pain, and/or hypoxemia. Chest radiograph is required if any respiratory symptoms, chest pain, or hypoxemia are present. 2
Infection: Check temperature; if fever ≥38.0°C, obtain blood cultures and initiate prompt antibiotics, as patients are functionally asplenic. 2
Splenic sequestration: Assess for rapidly enlarging spleen with hemoglobin decrease >2 g/dL from baseline. 1, 2
Baseline hemoglobin measurement: Compare with the patient's known baseline to detect potential complications like sequestration. 2
Oxygen saturation: Measure and compare to the patient's baseline. 2
Other Complications to Consider
- Stroke, priapism, or acute renal insufficiency must be considered in the appropriate clinical context. 2
What Laboratory Tests Do NOT Diagnose Acute Pain Episodes
Sickle cells on peripheral smear are chronically present in patients with sickle cell disease and do not distinguish acute pain episodes from baseline. 1
Elevated lactate dehydrogenase, bilirubin, reticulocyte count, white blood cells, or platelet counts are not used to diagnose the acute pain episode itself. 1
A decreased reticulocyte count characterizes transient aplastic crisis, not a typical vaso-occlusive pain episode. 1
Critical Management Implication
Rapid triage and aggressive parenteral analgesia with opioids should be administered promptly once the patient reports acute pain consistent with their typical crisis pattern. 1
Scheduled around-the-clock dosing or patient-controlled analgesia (PCA) should be used for severe pain. 1
Never assume opioid dependency, as opioid dependency is rare in sickle cell disease, while opioid sensitivity is more common. 3, 2