SICKLE-CELL disease (SCD) is a genetic blood disorder that affects the haemoglobin within the red blood cells. The recurrent pain and complications caused by the disease can interfere with many aspects of the patient's life, including education, employment and psychosocial development in which believed by among some traditional tribes attributes the sickness to a blood 'Vampire' that sucks human blood, hence causing blood deficiency to the person.
It is a major genetic disease in most countries in Sub- Saharan Africa. In recognition of this, the World Health Organisation (WHO) under the DNC Department, supervise and coordinate interventions relating to the prevention and management of SCD. The WHO intends to support primary prevention (genetic counselling, general public knowledge); Early detection - screening; reduce morbidity; chemoprophylaxis, vaccines, clinical care of special groups (children, pregnant women); capacity building Human resource; improve quality of life of patients and build partnerships.
Tanzania ranks fourth in the world after Nigeria, India and DR Congo to have the highest number of people having the disorder. With sickle cell becoming a threat to the world, specifically in Sub-Saharan Africa, researchers and scientists are hoping to find better ways of managing and improving services related to treatment of the disease.
Sub-Saharan Africa has the highest number of people suffering from genetic blood disorder (sickle cell anaemia). Children are at more risk of dying of the inherited disease if it is not contained. According to coordinator at the Muhimbili Sickle Cell Research Programme, Dr Deogratias Soka, said between 8,000 and 11,000 new babies born annually are affected by the disease.
"The rate is high and without treatment, between 50 to 90 per cent of these children will die in childhood, the prevalence of the threat caused by the disease in Tanzania can be reduced by 70 per cent in childhood if newborn screening and prevention of infections and mortality are emphasised," says the coordinator.
The World Health Organisation (WHO) estimates that over 300,000 babies with severe forms of the disease are born worldwide each year, the majority in low and middle income countries, that 5 per cent of the world's population are healthy carriers of a gene for sickle-cell disease. The country has established a biomedical research programme in sickle cell disease to find interventions that are locally-appropriated and have global significance.
With local and global partnership, Tanzania has developed a systematic framework for comprehensive research with prospective surveillance of over 2,500 SCD patients. The Muhimbili Wellcome Programme, which is part of Muhimbili University of Health and Allied, works within the Muhimbili National Hospital (MNH).
With support from the Wellcome Trust, it has provided descriptions of the clinical spectrum of disease, defining the major causes of illness and death. This programme is one of the largest, biomedical sicklecell resources in the world. Tanzania is building mass of technologically- literate professionals who will ensure sustainability and competitive advantage.
Tanzania intends to use sickle cell disease as a model to illustrate, that with effective collaboration, significances in health and biomedical science can be archieved in Africa. Management of sickle-cell disease at different levels of the health-care system should emphasize programmes that use simple, affordable technology and are accessible to a large proportion of the community; such programmes are preferred instead of a parallel system which may be too expensive and unsustainable. The programme should be developed at the primary care level with appropriate technical and patient referral support from higher levels of care. Training of health personnel in prevention, diagnosis and case management should ensure that the health-care system is able to provide the basic requirements of these services.
Family and community- based care should be an integral part of the national programme Education and research are important components of the programme for the reason that the information generated should be disseminated and used as evidence in policy-making, as well as in day-to-day decision-making in the management of the programme.
It is also necessary to study the natural history of the disease and its effects on clinical manifestations and transmission of malaria. For prevention and care programmes the disease should be identified during the prenatal period or at birth as part of a routine screening programme.
At this stage such services should be available alongside counselling and health education services while genetic counselling and screening can lead to substantial reduction in the number of children born with the trait. Furthermore, partnerships should be fostered between health professionals, parents, patients, relevant community interest groups and the media, where appropriate. Partnerships will facilitate public education, identification of genetic risks in the community by recording family disease histories, genetic counselling, awareness and active participation in prevention.