IntroductionThe social and political implications of Sickle Cell Disease (SCD) are complicated and unique. It is an example of a genetic disease that is now considered a global health problem. The fact that it is a genetically inherited disorder renders it incurable by conventional means. Although research in stem cell and gene therapy have shown promise in mice, this treatment option would be expensive and not readily deployable to the millions of patients who suffer with SCD in developing nations, where the majority of the cases are concentrated. The fact that much of the burden of disease is felt in these nations creates an inequitable distribution of worldwide research spending, as those people in poverty-stricken nations do not have the income to afford the high prices pharmaceutical companies may charge for their products. This divide is illustrated by the amount of money spent on research of chronic diseases, such as heart disease, diabetes and atherosclerosis more prevalent in rich countries, versus the amount spent on tropical disease research on malaria, Dengue and tuberculosis, which are almost exclusively found in poorer nations. SCD prevention needs to be addressed differently based on region, but common themes include prevention through prenatal screening coupled with dedicated and comprehensive SCD treatment facilities. In the majority of countries where sickle cell anemia is a major public health concern, management has unfortunately been inadequate due to a lack of national control programmes, according to a report published by the World Health Organization. It was reported that basic facilities for patient care are usually absent and systematic screening for sickle cell anemia is not widespread. Another concern is that the diagnosis of sickle cell disease is only made when a patient has a major and severe complication. The use of penicillin to prevent infections is a cost-effective and simple solution for patients, but unfortunately the use of this medicine is not widespread in many countries that lack policies for preventative measures. The challenge which demands the most attention is the prognosis improvement for patients with sickle cell anemia in developing countries which lack basic policies. However, more effective policies are also required in developed countries. Preventative medicine has been suggested by the WHO and researchers as one of the top priorities for public policy measures for sickle cell anemia. Intervention at an early stage for preventable problems with the help of pain medications, antibiotics, nutrition, folic acid supplementation or counselling to increase fluid intake are all measures that can reduce costs, improve prognosis for patients, and decrease the number of severe cases that require immediate attention. Prevention eliminates the need for emergency cures. Another important factor to consider is new-born screening which, along with timely diagnostic testing, parental counselling, education, and comprehensive care, can significantly reduce the morbidity and mortality which results from this disease in infancy and early childhood. Counseling and a holistic approach that takes into account economic challenges can greatly improve the quality of life for sickle cell patients, both in developing and in developed countries. In the past decade, many advancements have been made in the field of sickle cell treatments, but most of these cures are only available to patients in high resource (developed) countries. The challenges faced by patients in developing countries are different from the ones faced by patients in developed countries. A general improvement in health services is first required in developing countries to ensure better treatment for sickle cell patients. In developing countries, concerns of public policy for sickle cell pertain to which treatment types are most effective or which type of patient care model is most effective. Models of Care: Developed CountriesResearch has proven that a dedicated facility is the kingpin for effective and rapid painful crisis management as it reduces hospitalizations, and facilitates integration of the approach into other areas of care. Comprehensive Care Centres Increase Cost Effectiveness:
Costs and Hospital Care Dissatisfaction
Models of Care: Developing Countries
Bacterial SepsisMore than 90% of children with SCD die prior to diagnosis in sub-Saharan Africa. Williams (see references) posits that bacterial sepsis is a significant cause of death in children with SCD. In fact, the odds of a child with SCD having bacteraemia was much higher than a normal child. There is a similar bacterial profile in those SCD patients with bacterial sepsis in rural Kenya and developed countries. Vaccines against Streptococcus pneumoniae and Haemophilus influenzae b, part of normal immunization programs around the globe, are an effective preventative measure because they are almost exclusively found in children with SCD. Vaccination for H influenzae b was shown to be effective for those with and without SCD. In Kilifi, Kenya there was an 88% reduced incidence of bacteraemia caused by this bacterium. Newborn ScreeningNewborn screening for sickle cell anemia has emerged as a crucial public health approach towards the identification of sickle cell anemia patients before they develop complications. After a newborn baby is screened for sickle cell anemia, this can lead to the implementation of further strategies for patient care, such as parent education, immunizations and penicillin administration for prevention of infections. Furthermore, identification of families at risk via newborn screening, or community screening programs has led to the ability for genetic counselling for patients and couples planning for children. However, the benefits of prenatal/neonatal testing are felt in developed countries because appropriate counselling for parents or primary care initiatives for the patients are available. The aforementioned approaches require that attention be paid to the three core principles of medical genetics: that the autonomy of the individual or the couple is respected; their right to complete and adequate information is fulfilled and that strict confidentiality is required. Resources for Patients and Parents:
Future DirectionsNational control programs which exist in developed countries are not appropriate in most low-resource countries. However, sickle cell disorders should be covered by health service planning in all countries where the disease is prevalent. Various components of prevention and treatment should be considered simultaneous and support should be provided to existing initiatives. Research and systematic gathering of information for the least expensive approaches for prevention and treatment should be encouraged and promoted. Essential areas of work include:
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