Sickle Cell Disease

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Authors: Lisa Sabir, Roshan Cherian / Editors: Frances Balmer / Codes: / Published: 25/03/2024

Sickle cell disease (SCD) is a group of hereditary haemoglobinopathies. Patients suffering from SCD presenting to the emergency department (ED) most commonly present due to complications such as painful crises and are at risk of life-threatening complications. It is important for the emergency team to promptly recognise and treat these patients.

In this learning session we will run through the pathophysiology that leads to the clinical manifestations of the disease and outline the assessment and management of these patients.

Learning objectives

After completing this session, you will be able to:

  • Understand the pathophysiology leading to the symptoms the patient may present with
  • Describe the key features of the assessment
  • Describe the common sickle cell disease related complications
  • Describe the emergency management of these patients

References

  1. National Institute for Health and Care Excellence (NICE). Sickle cell disease | Health topics A to Z | CKS | NICE [Internet]. [Accessed Oct 19 2023].
  2. NHS Digital [Internet]. Hospital Admitted Patient Care Activity, 2022-23. [Accessed Oct 18 2023].
  3. National Institute for Health and Care Excellence (NICE). What is the prognosis? | Background information | Sickle cell disease | CKS | NICE [Internet]. [Accessed Oct 26 2023].
  4. Paula. No Ones Listening – A Report Sickle Cell Society [Internet]. Sickle Cell Society. 2021 [cited 2023 Oct 20].
  5. Simon E, Long B, Koyfman A. Emergency Medicine Management of Sickle Cell Disease Complications: An Evidence-Based Update. The Journal of Emergency Medicine. 2016 Oct 1;51(4):37081.
  6. Sickle cell anaemia – Symptoms, diagnosis and treatment | BMJ Best Practice [Internet]. [Accessed Oct 20 2023].
  7. Sundd P, Gladwin MT, Novelli EM. Pathophysiology of Sickle Cell Disease. Annu Rev Pathol. 2019 Jan 24;14:263-292.
  8. DeBaun MR, Armstrong FD, et al. Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Blood. 2012 May 17;119(20):458796.
  9. National Institute for Health and Care Excellence (NICE). Sickle cell disease: managing acute painful episodes in hospital | [CG143] | NICE [Internet], 2012. [Accessed Oct 20 2023].
  10. Royal College of Emergency Medicine (RCEM). Clinical Guidelines. [Accessed Oct 20 2023].
  11. Vichinsky EP, Neumayr LD, et al. Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease. New England Journal of Medicine. 2000 Jun 22;342(25):185565.
  12. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014 | NHLBI, NIH [Internet]. [Accessed Oct 26 2023].
  13. DeBaun MR, Jordan LC, King AA, Schatz J, et al. American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. Blood Advances. 2020 Apr 16;4(8):155488.
  14. Lee MT, Piomelli S, Granger S, Miller ST, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood. 2006 Aug 1;108(3):84752.
  15. Sickle Cell Disease in Childhood: Standards and Recommendations for Clinical Care Sickle Cell Society [Internet]. Sickle Cell Society. [Accessed Oct 26 2023].
  16. Aljuburi G, Laverty AA, Green SA, Phekoo KJ, Bell D, Majeed A. Socio-economic deprivation and risk of emergency readmission and inpatient mortality in people with sickle cell disease in England: observational study. J Public Health (Oxf). 2013 Dec;35(4):5107.
  17. Health Inequalities. elearning for healthcare (ELFH), NHS England [Internet]. [Accessed Oct 25 2023].
  18. Davies SC, Oni L. Fortnightly review: Management of patients with sickle cell disease. BMJ. 1997 Sep 13;315(7109):65660.

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