SCD

What is SCD?

SCD refers to a group of blood disorders caused by genetic faults that affect the production of healthy red blood cells (RBCs). This causes faulty RBCs to form a ‘crescent’ shape which can stick together and form blockages within blood vessels.1 These blockages may prevent oxygenated blood from reaching muscles and organs, potentially causing serious damage to the affected areas.2

SCD is the most common serious genetic disorder affecting over 1 in 2,000 live births in the UK. However, SCD most commonly affects people originating from sub-Saharan Africa.3

What are the symptoms of SCD?

The symptoms of SCD can be complex and varied. Typical complications include chronic pain, delayed growth, acute chest syndrome, as well as heart, liver and kidney problems.4,5

Treatment of SCD and the risk of iron overload

In the UK, SCD can be diagnosed shortly after birth through genetic screening.6,7 A key component of SCD management is the use of frequent blood transfusions, however this is not without complications and can lead to iron overload.6,8

Iron overload in SCD and how it can be treated

Iron overload can lead to serious health implications such as heart, liver and hormone complications.9 In order to help control the build-up of excess iron in SCD patients, doctors can suggest the use of iron chelators.

 

Abbreviations: RBC, red blood cell; SCD, sickle cell disease.

References

  1. Quinn CT. Clinical severity in sickle cell disease: the challenges of definition and prognostication. Exp Biol Med (Maywood) 2016;241(7):679–688.
  2. Yawn BP and John-Sowah J. Management of sickle cell disease: recommendations from the 2014 Expert Panel report. Am Fam Physician 2015;92(12):1069–1076.
  3. Meremikwu MM and Okomo U. Sickle cell disease. BMJ Clin Evid 2016;2016:2402.
  4. McCavit T and Desai P. National Heart, Lung and Blood Institute. 2014. Available at: www.hematology.org/Clinicians/Guidelines-Quality/Quick-Ref/3466.aspx. Last accessed May 2020.
  5. Claster S and Vichinsky EP. Managing sickle cell disease. BMJ 2003;327:1151–1155.
  6. National Health Service. Sickle Cell Disease. Available at: https://www.nhs.uk/conditions/sickle-cell-disease. Date accessed: May 2020.
  7. National Heart, Lung and Blood Institute. Sickle Cell Disease. Available at: https://www.nhlbi.nih.gov/print/4933. Date accessed: May 2020.
  8. Davis BA, et al. BJH 2017;176:179–191.
  9. Lambing A, et al. The dangers of iron overload: Bring in the Iron Police. J Am Acad Nurse Pract 2012;24(4):175–183.
ONC20-C044c June 2020.
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