Key Points
- Stabilize ABCs; begin targeted evaluation without delaying life-saving therapy.
- Use system-specific risk tools to guide testing and disposition.
- Order high-yield tests first; escalate imaging when indicated.
- Start evidence-based initial therapy and reassess frequently.
Algorithm
- Primary survey and vitals; IV access and monitors.
- Focused history/physical; identify red flags and likely etiologies.
- Order system-appropriate labs and imaging (see Investigations).
- Initiate guideline-based empiric therapy (see Pharmacology).
- Reassess response; arrange consultation and definitive management.
Clinical Synopsis & Reasoning
For Sickle Cell Disease Acute Crisis Disease Modifying Therapy, frame the differential by acuity and pathophysiology, then align diagnostics to the leading hypotheses. Prioritize stabilization while obtaining high‑yield studies such as CBC with diff (Cytopenias/leukocytosis), Coags (Bleeding/clotting), Smear (Morphology). Incorporate bedside imaging and targeted labs to define severity and identify complications; synthesize results with clinical trajectory to refine the working diagnosis and disposition needs.
Treatment Strategy & Disposition
Initiate disease‑directed therapy alongside supportive care, titrating to objective response. Pharmacologic options commonly include Anti-pseudomonal β-lactam. Use validated frameworks (e.g., Disease‑Modifying Options) to guide escalation and site of care. Address precipitating factors, de‑escalate empiric therapies with data, and arrange follow‑up for monitoring and risk‑factor modification; admit patients with instability, high risk of deterioration, or needs for close monitoring.
Management Notes
Establish individualized pain plans; address stigma and bias in care. Coordinate with SCD centers for advanced therapies.
Epidemiology / Risk Factors
- Cytotoxic chemotherapy, neutropenia; lines
Investigations
| Test | Role / Rationale | Typical Findings | Notes |
|---|---|---|---|
| CBC with diff | Cytopenias/leukocytosis | Abnormal counts | |
| Coags | Bleeding/clotting | Abnormalities | |
| Smear | Morphology | Abnormal cells |
Disease‑Modifying Options
| Therapy | Use |
|---|---|
| Hydroxyurea | Frequent pain/ACS; increases HbF |
| L‑glutamine | Reduces pain episodes |
| Crizanlizumab | Prevents VOC via P‑selectin blockade |
| Voxelotor | Increases Hb in hemolytic phenotypes |
| Transplant/gene therapies | Curative in select; specialist centers |
Pharmacology
| Medication | Mechanism | Onset | Role in Therapy | Limitations |
|---|---|---|---|---|
| Acetaminophen | Analgesic/antipyretic | Hours | Symptom control as appropriate | Hepatotoxicity (overdose) |
| Ondansetron | 5-HT3 antagonism | Minutes | Antiemesis if needed | QT prolongation |
Prognosis / Complications
- Tied to depth/duration of neutropenia and comorbidities
Patient Education / Counseling
- Explain red flags and when to seek emergent care.
- Reinforce medication adherence and follow-up plan.
References
- ASH Sickle Cell Guidelines — Link