Critical Alerts

• Acute Chest Syndrome (ACS) is life-threatening: New infiltrate + respiratory symptoms
• Fever in SCD = Medical emergency: Functional asplenia → overwhelming sepsis
• Opioids are appropriate for pain crisis: Do not undertreat pain
• Exchange transfusion for severe ACS, stroke, multi-organ failure: Simple transfusion may not be enough
• Think stroke in any neurological change: Low threshold for imaging
• Splenic sequestration can cause rapid death: Especially in children

Key Diagnostics

Emergency Treatments

Overview

Sickle cell disease (SCD) is an inherited hemoglobinopathy caused by a single nucleotide mutation resulting in abnormal hemoglobin (HbS) that polymerizes under low oxygen conditions, causing red blood cells to become rigid and sickle-shaped. This leads to vaso-occlusion, hemolysis, and progressive organ damage. Sickle cell crisis refers to acute complications requiring emergency care.

Types of Crises

Epidemiology

• SCD prevalence: 100,000+ in US; 1 in 365 African American births
• Life expectancy: 40-60 years (improved with modern care)
• ED visits: SCD patients have high ED utilization
• ACS is leading cause of death: And second most common cause of hospitalization
• Stroke incidence: 11% by age 20 (without prevention)

Genotypes

Life-Threatening Complications

Pain Crisis Mimics

Acute Chest Syndrome vs Pneumonia vs PE

• Often cannot distinguish clinically
• Treat empirically for all
• ACS may evolve from pain crisis

Laboratory Studies

Imaging

Special Considerations

• Know patient's baseline Hgb
• Extended RBC phenotype matching (reduces alloimmunization)
• Higher incidence of iron overload (if chronically transfused)

ICU Admission

• Severe ACS (high O2 requirement, worsening)
• Stroke
• Multi-organ failure
• Hemodynamic instability (sepsis, splenic sequestration)
• Exchange transfusion in progress

Floor Admission

• Pain crisis requiring IV opioids
• Mild/moderate ACS
• Fever requiring IV antibiotics
• Aplastic crisis needing transfusion

Discharge Criteria

• Pain controlled on oral medications
• Afebrile
• Adequate oxygenation
• Able to maintain hydration
• Close follow-up arranged

Follow-Up

Condition Explanation

• "Sickle cell disease causes your red blood cells to become stiff and sticky, which can block blood flow and cause pain."
• "We are giving you strong pain medicine and fluids to help with this crisis."
• "It's important to recognize the signs of serious complications like chest problems or fever."

Prevention

• Stay hydrated
• Avoid extreme temperatures, high altitude
• Manage stress
• Take hydroxyurea as prescribed
• Get vaccinations (pneumococcal, meningococcal, flu)
• Prompt medical attention for fever

Warning Signs

• Fever (>38.5°C)
• Chest pain, difficulty breathing
• Severe headache, vision changes, weakness/numbness
• Abdominal swelling
• Priapism (erection >4 hours)

Pregnancy

• High-risk pregnancy
• Increased frequency of crises
• Risk of preterm labor, IUGR
• Hydroxyurea contraindicated
• May need prophylactic transfusions
• Obstetrics and hematology co-management

Pediatric

• Higher risk of splenic sequestration
• Stroke prevention (transcranial Doppler screening)
• Penicillin prophylaxis until age 5
• Spleen may be palpable (before autoinfarction)

Surgical Patients

• Preoperative transfusion to Hgb ~10
• Aggressive hydration, oxygenation, warming
• Pain management planning

Sickle Cell Trait (HbAS)

• Usually asymptomatic
• Rare complications: Splenic infarct at altitude, hematuria
• Do not treat like SCD

Performance Indicators

Documentation Requirements

• Pain score and treatment response
• Baseline hemoglobin
• Prior complications (ACS, stroke)
• Opioid history and regimen
• Transfusion history
• HbS level (if available)

Diagnostic Pearls

• Pain crisis is a diagnosis of exclusion: Rule out infection, ACS, etc.
• ACS may evolve from pain crisis: Repeat CXR if worsening
• Fever = emergency in SCD: Functional asplenia
• Low reticulocytes with anemia = aplastic crisis: Think parvovirus
• Know the patient's baseline Hgb: Drop of >1-2 g/dL is significant
• Stroke can be subtle: Any neuro change warrants imaging

Treatment Pearls

• Do not undertreat pain: Opioids are appropriate
• Transfuse to Hgb ~10, not higher: Hyperviscosity risk
• Exchange transfusion for severe ACS/stroke: Target HbS <30%
• Incentive spirometry prevents ACS: 10 breaths q2h
• Cover atypical organisms in ACS: Chlamydia, Mycoplasma
• Don't give supplemental O2 unless hypoxic: May suppress erythropoiesis

Disposition Pearls

• Most pain crises need admission: Unless rapid resolution
• Low threshold for ICU with ACS: Can deteriorate quickly
• Stroke requires immediate hematology and exchange: Do not delay
• Ensure sickle cell follow-up: Chronic disease management reduces crises