Diagnosing 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 objective findings. 1
Clinical Diagnostic Standard
Trust the patient's report completely. The American Academy of Pediatrics explicitly states that acute painful episodes are diagnosed clinically based on the patient's description of pain alone. 1 Patients with sickle cell disease and their caregivers typically know their disease best, and their report of typical pain symptoms should be trusted and acted upon promptly without delay. 1
Key Diagnostic Principles
- No laboratory tests are required to diagnose an acute pain episode—the diagnosis is purely clinical. 1
- Patient self-report is the gold standard for diagnosis, and healthcare providers must accept this without demanding objective evidence. 1
- Rapid triage and assessment are essential when patients report acute pain consistent with their typical crisis pattern. 1, 2
Typical Pain Patterns by Age
The location of pain varies predictably with age: 1
- Infants: Pain typically affects the fingers (dactylitis)
- Older children and adults: Pain commonly involves long bones, sternum, ribs, or back
What Laboratory Tests Do NOT Diagnose
It is critical to understand what laboratory findings do not indicate an acute pain episode: 1
- Sickle cells on peripheral smear: These are chronically present in patients with sickle cell disease and do not distinguish acute pain episodes from baseline
- Elevated hemoglobin, lactate dehydrogenase, bilirubin, reticulocyte count, white blood cells, or platelet counts: These markers may be elevated but are not used to diagnose the acute pain episode itself
- Decreased hemoglobin >2 g/dL: This characterizes splenic sequestration crisis, not a typical vaso-occlusive pain episode
- Decreased reticulocyte count: This characterizes transient aplastic crisis, not acute pain
Critical Exclusions Required
While the pain episode itself is diagnosed clinically, you must exclude life-threatening complications: 3, 1, 4
Acute Chest Syndrome (ACS)
In patients with sickle cell disease presenting with acute chest pain, acute chest syndrome must be excluded. 3 This is a Class I recommendation from the American College of Cardiology. 3
ACS is characterized by: 4
- New segmental infiltrate on chest radiograph
- Lower respiratory tract symptoms
- Chest pain and/or hypoxemia
Emergency Transfer Protocol
Emergency transfer by EMS to an acute care setting is recommended for all patients with sickle cell disease who report acute chest pain. 3 This ensures rapid evaluation and exclusion of ACS, which can be life-threatening.
Management Implications of the Diagnosis
Once the diagnosis is made based on patient report: 1, 2
- Aggressive parenteral analgesia with opioids should be administered promptly for severe pain
- Scheduled around-the-clock dosing or patient-controlled analgesia (PCA) should be used rather than as-needed dosing
- Close observation for development of complications is necessary after initial presentation, particularly for acute chest syndrome
Common Pitfalls to Avoid
Do not delay treatment while waiting for laboratory confirmation—there is no laboratory test that confirms or rules out an acute pain episode. 1 The most significant barrier to appropriate care is the failure to trust the patient's self-report and the tendency to seek objective evidence that does not exist for this diagnosis. 2, 5
Do not undertreate pain due to lack of objective physical findings—studies have documented disparities in pain management, with patients of color receiving less pain medication despite similar pain severity. 2