Sickle Cell Disease in the ED

Vaso-occlusive Phenomena

Stroke

• Ischemic

• Hemorrhagic

Acute Chest Syndrome

• Diagnostic Criteria

  • New pulmonary density on chest imaging involving at least one complete lung segment and ≥1 of the following:

    • Temperature ≥38.5°C

    • >3 percent decrease in SpO2 (oxygen saturation) from a documented steady-state value on room air

    • Tachypnea (per age-adjusted normal)

    • Intercostal retractions, nasal flaring, or use of accessory muscles of respiration

    • Chest pain

    • Cough

    • Wheezing

    • Rales

• Symptoms

  • Fever

  • Cough

  • Chest pain

  • Shortness of breath

  • Hemoptysis

• Triggers

  • Infection

  • Vaso-occlusive Crisis

  • Pulmonary Embolism

  • Asthma

  • Pulmonary Edema

  • Atelectasis

  • Opioid induced hypoventilation

• Treatment

  • Antibiotics

  • Maintaining oxygenation >95%

  • fluid hydration

  • pain control

  • inhaled incentive spirometry

Acute Pain Crisis

• Affected Systems

  • Pulmonary: CP, SOB

  • CNS: HA

  • Musculoskeletal: Tender + warm joints, bone pains

  • GI: Abdominal pain

• Other findings: Fever, Leukocytosis

• Consider other differentials:

  • CP/SOB: ACS, ARDS, cor pulmonale, high output heart failure, pneumonia, pulmonary embolism

  • headache: Stroke, memingitis, CV thrombosis, ocular pathologies

  • Arthralgias: septic arthritis, acute trauma, avascular necrosis

  • Abdominal Pains: Splenic sequestration, acute intrahepatic sequela, mesenteric vascular occlusions, renal infarcts.

Sequelae of hemoglobinopathies:

• Priapism

• Sickle Cell Nephropathy

• UTI/Pyelonephritis

• Renal meduallary carcinomas

• Splenic Sequestration

• RUQ/Abdominal pain

  • Symptomatic cholelithiasis

  • cholecystitis

  • acute sickle hepatic crisis

    • acute intrahepatic cholestasis

  • Acute hepatic Sequestration

Microbiology reminders to watch out for in asplenic sickle cell patients

• Streptococcus Pneumoniae

• Haemophilus Infleunza

• Non-typhoidal salmonella species

• mycoplasma

• C. Pneumoniae

• Yersinia enterocolitica

Amanda Baroutjian, MD