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