Diagnosis of Acute Pain Episodes in Sickle Cell Disease
The diagnosis of an acute pain episode in sickle cell disease is entirely clinical, based solely on the patient's self-report of pain, without requiring any laboratory abnormalities or diagnostic tests. 1
Clinical Diagnostic Criteria
Patient self-report is the gold standard: The diagnosis relies completely on the patient describing pain consistent with their typical crisis pattern, as patients with sickle cell disease and their caregivers know their disease best and their report should be trusted and acted upon promptly. 1
No laboratory tests are required: Acute painful episodes are diagnosed clinically without needing any specific laboratory abnormalities to confirm the diagnosis. 1
Location patterns: Pain typically affects the fingers in infancy (dactylitis), and long bones, sternum, ribs, or back in older children and adults. 2
What Laboratory Tests Do NOT Diagnose Acute Pain Episodes
It is critical to understand that certain laboratory findings indicate different complications, not typical vaso-occlusive pain episodes:
Sickle cells on peripheral smear: These are chronically present in patients with sickle cell disease and do not distinguish acute pain episodes from baseline status. 1
Hemoglobin drop >2 g/dL: This characterizes splenic sequestration crisis, not a typical vaso-occlusive pain episode. 1
Decreased reticulocyte count: This characterizes transient aplastic crisis, not acute pain. 1
Elevated laboratory markers in priapism: While acute ischemic priapism in sickle cell disease may show lower hemoglobin and elevated lactate dehydrogenase, bilirubin, aspartate aminotransferase, reticulocyte count, white blood cells, and platelet counts, these are not used to diagnose the acute pain episode itself. 2
Essential Clinical Actions Following Diagnosis
Once the patient reports acute pain consistent with their typical pattern:
Rapid triage and assessment are essential, with aggressive parenteral analgesia using opioids administered promptly. 1
Scheduled around-the-clock dosing or patient-controlled analgesia (PCA) should be used for severe pain rather than as-needed dosing. 1
Close observation for complications is necessary after initial presentation, particularly for acute chest syndrome in patients with chest pain (characterized by new infiltrate on chest radiograph with respiratory symptoms). 1
Common Pitfalls to Avoid
Do not delay treatment waiting for laboratory confirmation, as no specific lab abnormalities are required for diagnosis. 1
Do not dismiss patient reports of typical pain symptoms, as patients are the experts on their own disease presentation. 1
Do not confuse acute pain episodes with other acute complications like splenic sequestration or aplastic crisis, which have distinct laboratory findings. 1