Frequently Asked questions

• Over 10,000 patients with sickle cell disease (SCD) and ß thallasaemia major (and intermediate) across London

• The majority are domiciled in North West or East London.

• A number of hospitals have appointed (are appointing) specialists in the field including Central Middlesex, Hammersmith, St Mary’s (Charing Cross has an associate specialist), University College, the Whittington, the North Middlesex, the Royal London and Newham, Kings College, Guy’s, Lewisham. This has generally resulted from high patient throughput and perceived need.

• Medium life expectancy of SS men and women is mid forties and rising.

• As patients live longer chronic organ damage becomes a greater problem so many require end stage renal services, replacement hips, specialist ophthalmic services etc.

• 10% of children suffer stroke (most common cause of stroke in children) with a further 20% suffering sickle damage to their brains such that they will under perform.

• There is therefore a need for specialist rehabilitation services, imaging and neuro educational services.

• Most patients complain that a major need is an integrated social care support packaged along with housing, education and health provision.

• Psychological support can reduce dependence on the health services e.g. cognitive behavioural therapy (CBT).

• Over 90% of acute hospital admission are for painful crisis – the pain protocols are varied and frequently not evidence based.

• Pregnant women with SCD or ß thallasaemia major need specialist and seamless haematological and obstetric care.

• For the severely affected children bone marrow transplants are curative and are available at St Mary’s Hospital.

• For severely affected adults (and selected children) hydroxyurea can be helpful and should only be managed by a specialist.

• There are a number of other complications including delays in growth and development, the onset of chronic organ damage etc. which recommend a care model of at least annual review in a specialist centre with shared care or locally where appropriate.