Diagnosis and Management of Acute Pain Episodes in Sickle Cell Disease

Clinical Diagnostic Approach

The diagnosis of an acute pain episode in sickle cell disease is based entirely on the patient's self-report of pain, without requiring any specific laboratory abnormalities, as recommended by the American Academy of Pediatrics 1
Acute painful episodes are diagnosed clinically based on the patient's description of pain, without requiring any specific laboratory abnormalities, and 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 2

Rapid triage and assessment are essential when patients report acute pain consistent with their typical crisis pattern, and aggressive parenteral analgesia with opioids should be administered promptly by healthcare providers 2
Scheduled around-the-clock dosing or patient-controlled analgesia (PCA) should be used for severe pain, and close observation for development of complications is necessary after initial presentation 2
The American College of Cardiology recommends excluding acute chest syndrome in patients presenting with chest pain, characterized by new infiltrate on chest radiograph with respiratory symptoms 3, 4

A drop in hemoglobin >2 g/dL characterizes splenic sequestration crisis, not a typical vaso-occlusive pain episode, according to the American Academy of Pediatrics 1
Sickle cells are chronically present on peripheral smears in patients with sickle cell disease and do not distinguish acute pain episodes from baseline, and a decreased reticulocyte count characterizes transient aplastic crisis 1, 5

Pain typically affects the fingers in infancy (dactylitis), and long bones, sternum, ribs, or back in older children and adults 6

Elevated laboratory markers in priapism, such as lower hemoglobin and elevated lactate dehydrogenase, bilirubin, aspartate aminotransferase, reticulocyte count, white blood cells, and platelet counts, are not used to diagnose the acute pain episode itself 7

The American College of Cardiology recommends excluding life-threatening complications, such as acute chest syndrome, in patients with sickle cell disease presenting with acute chest pain, with a Class I recommendation 8, 9
Emergency transfer by EMS to an acute care setting is recommended for all patients with sickle cell disease who report acute chest pain to ensure rapid evaluation and exclusion of acute chest syndrome, which can be life-threatening 8, 9

The American College of Cardiology gives a Class I recommendation to exclude acute chest syndrome in patients presenting with chest pain, characterized by new infiltrate on chest radiograph with respiratory symptoms, which is a leading cause of death with up to 13% all-cause mortality 10
In recurrent ischemic priapism, such as in sickle cell disease, the diagnosis may be abundantly clear by history and examination alone, and additional testing may be omitted at the clinician's discretion, with laboratory markers such as lower hemoglobin, elevated lactate dehydrogenase, bilirubin, aspartate aminotransferase, reticulocyte count, white blood cells, and platelet counts not used to diagnose the acute pain episode itself 10