Author name: Rabfoundyydheadmin

ICKLE CELL DISORDER – A NATIONAL EMERGENCY! BACKGROUND Sickle Cell Disorder (SCD) is the most common inherited blood disorder in the world. It is associated with severe shortage of blood, severe pain, serious complications such as stroke in children, chronic leg ulcers, degeneration of the hip joint, multiple organ failure, as well as social stigma, emotional and mental trauma and untimely death. Sickle Cell disorder (SCD) affects nearly 100 million people in the world. Of the 300,000 children born annually worldwide with SCD, more than 70% are in Sub-Saharan Africa – the vast majority in Nigeria. Nigeria is the country with the highest burden of sickle cell disorder in the world!: In Nigeria, SCD is among the 10 priority non-communicable diseases (NCDs) and it contributes significantly to both child and adult morbidity and mortality. By virtue of its population, Nigeria stands out as the most sickle cell-endemic country in the world; an estimated 150,000 babies are born every year in Nigeria with SCD and sadly, 50 – 90% do not live to celebrate their 5th birthday, mostly due to ignorance and lack of access to appropriate diagnosis and care. It is also estimated that over 25% of Nigerians have the sickle cell trait, implying that more than 40 million Nigerians are healthy carriers of the sickle cell gene (Hb AS) – about a quarter of the entire nation’s population – and can therefore transfer the gene to their offspring. Life Expectancy: The average life expectancy of persons with sickle cell in Nigeria remains very low – less than 20 years; whereas in the US for instance, it is now over 60 years. These grim statistics are predicted to worsen, should sickle cell disorder not be addressed as the serious public health problem that it is. It is indeed high time SCD is indeed seen as a NATIONAL HEALTH EMERGENCY WHICH MUST BE HANDLED WITH A HIGH SENSE OF URGENCY. Unfortunately, SCD continues to be of low priority for government and critical stakeholders, who should work in a coherent manner in order to bring SCD to heel. Sickle Cell Foundation Nigeria posits that we need a targeted approach to addressing the sickle cell problem. High mortality due to SCD in children under 5 years has been virtually eliminated from North America through policies and legislation that ensure ready access to a number of simple interventions, including newborn screening and prevention of infection through the provision of penicillin prophylaxis and polyvalent pneumococcal vaccination. We can get there as a country! The 63rd session of the UN General Assembly in December 2008 adopted a resolution on “recognition of sickle-cell anemia as a public health problem,” and urged Member States and UN organisations to raise awareness of SCD on June 19 of each year. Despite the fact that the UN has called for global efforts “to bring the disease out of the shadows,” relatively little attention has been given to assessing the burden of SCD and how to reduce it in sub-saharan Africa, where about 85% of children with SCD are born. In 2010, the WHO Regional Office for Africa proposed a SCD strategy officially recognising SCD as an important cause of child mortality in many African countries. Enough is already known to justify investment in public health SCD programmes, such as those already in place for other conditions (e.g., HIV or malaria), for promoting widespread screening, health education, and treatment to prevent or manage SCD. The Sickle Cell Pyramid of Care Ensures that everyone can play a role in bringing adequate intervention to the eco-system. No measure of support is too small to move the needle towards improved narratives. SCD PYRAMID OF CARE Prevention, Basic Treatment, Comprehensive Care, Support, Management of Complications: These are at the base and mid-section of the pyramid; interventions that focus on reduction in incidence of the SCD as well as provide the most care for the most people with the disorder, ensuring that persons with SCD can adequately manage the condition and live pain-free and fulfilling lives Government and other Stakeholders should invest resources and efforts in advocacy, awareness, education, genetic counselling, newborn screening, research-oriented projects and programmes that can bridge the knowledge gap that exists. Cure: The cure for SCD is at the apex of the pyramid. Whilst it behoves Nigeria as the sickle cell capital of the world to have centres that have centres that offer cure solutions for SCD (such as Stem Cell Transplantation), not everyone with SCD is a good candidate for these interventions and not everyone can afford them – with a huge percentage of the SCD demographic living below the poverty line and struggling to access basic routine medications. Applying resources to the Prevention, Basic Treatment, Comprehensive Care, Support, Management of Complications bands of the Care Pyramid will go a long way in alleviating the burden of sickle cell disorder in Nigeria. Without intensifying Education and Awareness, Nigeria will continue to bear the huge burden of SCD with high morbidity and mortality – with huge socio-economic implications for the country. More than ever before, there is a need to deploy a collaborative, coherent and synergistic approach to bringing the necessary intervention for this marginalized demographic of persons with SCD. Working in silos will not produce the expected results we are looking for. Synergy is the new energy required to get the job done. Avoiding confronting the challenge or ignoring it will not solve the problem. Government, corporate bodies, religious institutions, individuals, the medical community and all other relevant stakeholders must give impetus to initiate the deployment of relevant resources and interventions for persons with SCD. SCD and the SDGs: Addressing SCD presents Nigeria with a low-hanging fruit opportunity for achieving SDG 3/Target 2 – Reducing Under 5 mortality (approximately 10% of children born in Nigeria die before reaching the age of five – a statistic that we can deal a blow with interventions targeted at SCD). This will take strong political will and intentionally prioritising SCD as a public health problem, but