Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2022 Mar 7;27(1):50-62.
doi: 10.1093/pch/pxab096. eCollection 2022 Mar.

Acute complications in children with sickle cell disease: Prevention and management

[Article in English, English]
Carolyn E Beck  1 , Evelyne D Trottier  1 , Melanie Kirby-Allen  1 , Yves Pastore  1
Affiliations
Review

Acute complications in children with sickle cell disease: Prevention and management

[Article in English, English]
Carolyn E Beck et al. Paediatr Child Health. .

Abstract

Sickle cell disease (SCD) is a chronic, multi-system disease that requires comprehensive care. The sickling of red blood cells leads to hemolysis and vascular occlusion. Complications include hemolytic anemia, pain syndromes, and organ damage. Patterns of immigration and an increase in newborn screening mean that paediatric health care providers across Canada, in small and large centres alike, need to be knowledgeable about SCD. This statement focuses on principles of prevention, advocacy, and the rapid treatment of common acute complications. Guidance includes the current status of newborn screening, recommendations for immunizations and antibiotic prophylaxis, and an introduction to hydroxyurea, a medication that reduces both morbidity and mortality in children with SCD. Case vignettes demonstrate principles of care for common acute complications of SCD: vaso-occlusive episodes (VOE), acute chest syndrome (ACS), fever, splenic sequestration, aplastic crises, and stroke. Finally, principles of blood transfusion are highlighted, along with indications for both straight and exchange blood transfusions.

Keywords: Hemoglobinopathy; Hemolytic anemia; Hydroxyurea; Newborn screening.

PubMed Disclaimer

References

    1. Canadian Haemoglobinopathy Association (CanHaem). Consensus Statement on the Care of Patients with Sickle Cell Disease in Canada. Version 2.0. Ottawa; 2015: https://www.canhaem.org/wp-content/uploads/2018/05/Sickle-Cell-Consensus... (Accessed May 25, 2021).
    1. Corriveau-Bourque C, Bruce AA. The changing epidemiology of pediatric hemoglobinopathy patients in Northern Alberta, Canada. J Pediatr Hematol Oncol 2015;37(8):595–9. - PubMed
    1. Kim E, Graves E, Ajayi LT, PendergrastJ.. How Many Sickle Cell Disease Patients are there in Ontario? Results of a query of health services administrative databases. Sickle Cell Disease Association of Canada, Presentation; 2019: https://sicklecellanemia.ca/wp-content/uploads/pdf_public/ahrq_sickle_ce... (Accessed May 25, 2021).
    1. National Institutes of Health. Evidence-Based Management of Sickle Cell Disease. Expert Panel Report, 2014: https://www.nhlbi.nih.gov/health-topics/evidence-based-management-sickle... (Accessed June 25, 2021).
    1. Ballas SK, Lieff S, Benjamin LJ, et al. ; Investigators, Comprehensive Sickle Cell Centers . Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol 2010;85(1):6–13. - PMC - PubMed