Sickle Cell Diseases
Anna Soubry (Broxtowe, Conservative)
Sickle cell disease is a group of genetic blood disorders caused by a mutation of the haemoglobin gene, the most common of which is sickle cell anaemia. In this country, approximately 12,000 people have sickle cell anaemia. Around 25% of patients with sickle cell anaemia will require regular transfusions as part of their treatment, and it is essential that they receive suitably matched blood.
The British Committee for Standards in Haematology’s (BCSH) “Guidelines for pre-transfusion compatibility procedures in blood transfusion laboratories” (2012) require more stringent procedures to ensure compatible blood is provided for sickle cell patients. NHS Blood and Transplant (NHSBT), which provides blood to hospitals in England and North Wales, has in place a number of measures to maximise the availability of suitably typed blood for sickle cell patients. NHSBT is also evaluating new methods of improving the match between blood donor and recipient from the start of treatment, which would help people who have regular transfusions, such as those with sickle cell anaemia.
The UK haemovigilance scheme SHOT (Serious Hazards of Transfusion) recorded 19 adverse events related to blood transfusion in 2011 for people with sickle cell disease, some of which might have been avoided by better communication of information, but some of which were unpredictable.
New guidelines for transfusion in sickle cell and other haemoglobinopathy patients are currently being prepared by BCSH.
NHSBT is working with the Sickle Cell Society, the patient organisation for sickle, on education and blood donor recruitment. An information leaflet on sickle cell covering screening, blood donation, patient support and receiving blood products is due to be ready for distribution by the end of 2012.