Prescribing information

 

   

SCD is a group of autosomal recessive disorders characterised by the predominance of HbS that results in the production of abnormal RBCs. Due to mutations of the beta-globin gene,1 Hb are ‘sickle’ shaped and inflexible; this can increase blood viscosity and block or limit blood flow to the limbs and organs.2

SCD is most common in people living or originating from sub-Saharan Africa, but also affects people of Mediterranean, Caribbean, Middle-Eastern and Asian origin. The WHO estimates that 220,000 babies in Africa are born with SCD every year, many of whom die before the age of 5 years. In the UK, SCD is the most common serious genetic disorder and affects over 1 in 2,000 live births.3

SCD is commonly expressed in patients in different forms:

List of the different forms of SCD

SCD is a multi-system disease with unpredictable acute and chronic components, including:

List of the acute and chronic components of SCD

 

SCD increases the risk of stroke, organ damage, bacterial infections, and complications of blood transfusion. As a result, SCD is associated with lifelong morbidity and a reduced life expectancy, especially in young children. About 10% of children with HbS may develop a stroke, and more than 50% of these may suffer recurrent strokes.3

SCD and the risk of iron overload

Frequent blood transfusions are recommended to help treat SCD-associated complications (most notably anaemia), predisposing SCD patients to iron overload.4–6

Abbreviations: HbS, sickle haemoglobin; RBC, red blood cell; SCD, sickle cell disease; WHO, World Health Organization.

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 2016:01:2402.
  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 February 2020.
  5. Claster S and Vichinsky EP. Managing sickle cell disease. BMJ 2003;327:1151–1155.
  6. Davis BA, et al. Guidelines on red cell transfusion in sickle cell disease. BJH 2017;176:179–191.
HCP20-C005q June 2020.

 

For more information, refer to the EXJADE® (deferasirox) Summary of Product Characteristics:
https://www.medicines.org.uk/emc/product/7775/smpc.

Legal Category: POM.

PVC/PVDC/Aluminium blisters. Unit packs containing 30 or 90 film-coated tablets or multipacks containing 300 (10 packs of 30) film-coated tablets. Not all pack sizes may be marketed. Marketing Authorisation (MA) number, quantities and NHS price: EU/1/06/356/011 – 90 mg film-coated tablets 30 pack, £126.00. EU/1/06/356/014 – 180 mg film-coated tablets 30 pack, £252.00. EU/1/06/356/017 – 360 mg film-coated tablets 30 pack, £504.00 pack £1,540, EU/1/10/612/008 – 75 mg x 28 tablet pack £2,310.

 

 

For more information, refer to the DESFERAL® (desferrioxamine mesilate) Summary of Product Characteristics:
https://www.medicines.org.uk/emc/product/3813#PHARMACEUTICAL_PARTS.

Legal Category: POM.

Each vial contains a white to practically white lyophilisate supplied in a clear glass vial in a pack size of 10 (500 mg) or 1 (2 g). Marketing Authorisation (MA) number, quantities and NHS price: PL 00101/0523. Boxes of 10 £46.63.

 

×

Ask Speakers

×

Medical Information Request

Adverse events should be reported. Reporting forms and information can be found at yellowcard.mhra.gov.uk. Adverse events should also be reported to Novartis via [email protected] or online through the pharmacovigilance intake (PVI) tool at http://www.report.novartis.com/
If you have a question about the product, please contact Medical Information on 01276 698370 or by email at [email protected]