World Sickle Cell Day 2016 : A time for appraisal

sickle cell disease (SCD), a heritable blood disorder with devastating effects, was recognized as a global health problem by the World Health Organization. Two years later, the 63 rd United Nations Assembly designated June 19 as the " World Sickle Cell Day " , in an attempt to improve public awareness of the disease and to improve the prospects for patients. Now in 2016, do we detect any changes in public awareness and are the patients more research aware? Has research funding for SCD increased, and has advocacy for SCD improved? Why is SCD a global health problem? From its origins in sub-Saharan Africa, the Arabian peninsula and the Indian subcontinent, population migration has increased SCD prevalence in areas not previously associated with the disorder, such as the USA, western and northern Europe 1. In the USA, it affects close to 100,000 people with 3000 affected newborns each year, while in the United Kingdom, it is estimated that 12,500 individuals have SCD with an annual birth rate of 300 affected newborns 2. SCD is said to be the fastest growing serious genetic disorder in the UK and Western Europe. These figures pale into insignificance when compared with Africa and India. It is estimated that more than 300,000 children are born each year with SCD, about two thirds of them in Africa; Nigeria, India and the Democratic Republic of Congo bear half the global burden of SCD 3. Numbers are expected to climb projecting that by 2050, there will be about 400,000 babies born with SCD annually 4. How is SCD a global health problem? In well-resourced countries (e.g. USA, UK and France) 94 to 99 per cent of newborns with sickle cell anaemia can now expect to survive into adulthood 5,6 but they face emerging complications and morbidity, as they grow older. The adolescent with HbSS also face a crucial period of transitional care to adult services. But while survival estimates have continued to improve from a median survival of 42-48 years in 1994, to 58 years in 2014, in the USA, the life expectancy of patients with SCD is still shortened by more than two decades compared to the general population 7-9. The early mortality comes from several sickle-related complications affecting multiple organs from the damage inflicted by years of ongoing inflammation and vasculopathy. In well-resourced settings, adults with SCD are screened for potential complications including pulmonary hypertension, renal …