From the Guidelines
For a sickle cell pain crisis, I would order a complete blood count (CBC) with differential to assess hemoglobin levels, white blood cell count, and platelet count, as well as other essential tests to identify complications and guide treatment decisions. The management of sickle cell disease has improved markedly in recent decades, with a focus on prevention and management of complications from birth, and death in childhood is uncommon in the UK 1.
Essential Diagnostic Tests
- Complete blood count (CBC) with differential to assess hemoglobin levels, white blood cell count, and platelet count
- Reticulocyte count to evaluate bone marrow response
- Basic metabolic panel (BMP) to check electrolytes, renal function, and hydration status
- Liver function tests to assess for hepatic involvement
- Blood cultures if infection is suspected as a trigger
- Chest X-ray if respiratory symptoms are present to rule out acute chest syndrome
- Urinalysis to identify urinary tract infections or renal complications
- Oxygen saturation monitoring via pulse oximetry to detect hypoxemia, aiming for an oxygen saturation of 94–98% or the saturation level that is usual for the individual patient 1
Additional Studies
- Hemoglobin electrophoresis if diagnosis is uncertain
- LDH and bilirubin to assess hemolysis
- Targeted imaging based on pain location
- Consideration of multimodal postoperative analgesia techniques for pain management 1
These tests help confirm the diagnosis, identify complications or triggers, guide treatment decisions, and establish a baseline for the patient's current crisis. Early identification of complications like acute chest syndrome or infection is critical as these can rapidly worsen and become life-threatening in sickle cell patients 1.
From the Research
Diagnostic Studies for Sickle Cell Pain Crisis
The following diagnostic studies may be ordered for a sickle cell pain crisis:
- Complete Blood Count (CBC) to assess hemolytic and inflammatory processes, although its usefulness in evaluating acute vaso-occlusive crisis is debated 2
- Reticulocyte count to evaluate bone marrow response to hemolysis, which may be increased in patients with vaso-occlusive crisis (VOC) development 3
- Erythrocyte parameters, such as immature reticulocyte count and medium fluorescence reticulocytes, which may be useful in predicting VOC development 3
- White Blood Cell (WBC) count, which may be elevated in patients with acute vaso-occlusive crisis, although its true value in admission decisions is unclear 2
Laboratory Biomarkers
Certain laboratory biomarkers, such as:
- Reticulocyte count >189.4 10^9/L
- Medium fluorescence reticulocytes (MFR) >19.75% may be used to predict VOC development in patients with sickle cell disease 3
Trends in CBC Values
Trends in CBC values, including parallel changes in CBC values and pain intensity scores, may be monitored during acute painful episodes in children with sickle cell disease 4