Care of the Critically Ill Pediatric Sickle Cell Patient

  • Tolulope Rosanwo
  • Jennifer A. McArthur
  • Natasha ArcherEmail author


Sickle cell disease is the most common inherited disease in the United States. Through the effects of hemolysis and vaso-occlusion, it has the potential to incite critical illness involving multiple organ systems. Children with sickle cell disease are at risk of multiple types of shock resulting in a need for ICU care. Our youngest patients with sickle cell disease are at highest risk of infection due to lack of splenic function, and this can present with septic shock. Hypovolemic shock can occur secondary to severe acute anemia as seen with splenic sequestration or a delayed transfusion reaction. As one ages, the risk of cardiac dysfunction – diastolic and systolic dysfunction as well as pulmonary hypertension – can result in cardiogenic shock. In addition to shock, patients with sickle cell disease are at risk for respiratory failure from acute chest syndrome as well as acute neurologic deterioration from stroke. For these reasons, critical care physicians must be familiar with the unique management of sickle cell complications in order to provide the best possible care for this vulnerable population.


Sickle cell disease Acute chest syndrome Pulmonary hypertension Stroke Splenic sequestration Vaso-occlusive crisis Sepsis 


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Copyright information

© Springer International Publishing 2019

Authors and Affiliations

  • Tolulope Rosanwo
    • 1
  • Jennifer A. McArthur
    • 2
    • 3
  • Natasha Archer
    • 4
    Email author
  1. 1.Case Western Reserve University School of MedicineClevelandUSA
  2. 2.Department of Pediatrics, Division of Critical Care MedicineSt. Jude Children’s Research HospitalMemphisUSA
  3. 3.Medical College of WisconsinMilwaukeeUSA
  4. 4.Department of Pediatric Hematology and OncologyDana-Farber Boston Children’s Cancer and Blood Disorders Center, Harvard Medical SchoolBostonUSA

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