On this year’s World Sickle Cell Day, Dr. Noelle Orata, the Chair of Board for The Children’s Sickle Cell Foundation in Kenya sat down with Lizz Ntonjira, our Communications & Engagement Director and had a candid conversation on what can be done to increase public knowledge and understanding of the sickle cell disease, and the challenges experienced by patients and their families and caregivers.
Sickle cell disease has been recognized by the UN as a public health hazard and one of the world’s main genetic illnesses, yet investment in its prevention and management remains low. Why is this the case?
Investment in Sickle cell disease has been minimal. Some of the factors that hinder Investment in Screening, Prevention and Management of SCD globally are, lack of awareness. This is because Sickle cell disease (SCD) is an inherited non-communicable chronic illness that primarily affects populations in sub-Saharan Africa, the Middle East, and parts of India, where access to healthcare and awareness about SCD may be limited. Some challenges experienced are stigma, myths, misconceptions, religious beliefs and poverty that also leads to a low turnout of patients seeking care in the formal healthcare system.
Limited Health Funding in Public Health facilities contribute to poor health seeking behaviour, hence low prioritization of SCD based on various factors such as disease burden, prevalence, mortality rates, and cost-effectiveness of interventions. With the competing demands for limited resources, SCD does not receive the necessary attention and funding compared to other diseases with higher mortality rates or wider geographic reach. This results in a low level of attention and investment as compared to other diseases that have a broader global impact and has had a lot of research contributing to the global pool of knowledge.
There is little research done on SCD compared to other diseases. Research attracts alot of funding and unfortunatley for sickle cell disease there has been minimal funding. “Hydroxyurea” was first used on SCD patients in 1984 and it has taken more than 3 decades for other drugs to be introduced into the market for clinical trials. Compared to other diseases like cancer, infectious diseases, malaria research due to the sustained interest in infectious diseases and funding, there’s been remarkable strides in research and developmement including malaria vaccines as the latest initiative as a result of that.
Policy can influence investment and funding. In Kenya for instance, SCD has been on the radar for more than 100 years and yet the treatment guidelines that are SCD specific were launched in 2021. This tells a lot about how policy and advocacy has contributed greatly in delay of funding and investment on prevention and management of SCD in Kenya. This is the same story in most of the affected countries. Insufficient advocacy for SCD prevention and management can result in lower prioritization and resource allocation.
Finally, legal avenues can be used to develop a Sickle Cell Act that can ring fence funds with a specific focus on management of sickle cell patients from birth to adulthood taking away the burden of healthcare costs from the patients where patients seek care at sickle cell health centres of excellence and this ensuring they continue to contribute to our economy well into their old age and live a full wholistic life.
According to the World Health Organisation (WHO), more than 66% of the 120 million people affected worldwide by sickle cell disease live in Africa. Approximately 1,000 children are born with the disease everyday on the continent, making it the most prevalent genetically acquired disease in the region. What factors make the African continent shoulder such a great burden of this disease?
The high burden of sickle cell disease (SCD) in Africa can be attributed to:
- Sickle cell disease is a genetic disorder where one inherits two abnormal blood genes, each from both parents. In Sub-Saharan Africa, particularly the areas with a high malaria burden, has historically and today recorded a higher prevalence of the sickle cell gene (Hbs), leading to greater incidences of SCD. It is believed that those who carry only one gene of sickle cell (Carriers/ trait) have a higher resistance to malaria. This mutation advantage has led to the persistence of the HbS gene in populations living in malaria-endemic regions. Consequently, the African continent, where malaria has been prevalent for centuries, has a higher prevalence of the HbS gene and subsequently a higher burden of sickle cell disease.
- Stigma associated with sickle cell disease can hinder awareness, acceptance, and proper care-seeking behavior. Misconceptions and negative attitudes towards the disease can lead to discrimination, isolation, Mental Health issues and neglect of individuals with SCD, further exacerbating their health outcomes.
- Poverty, limited education, and inadequate access to healthcare services contributes to the burden of sickle cell disease in Africa. These factors contribute to delayed diagnosis, limited availability of essential medications, and reduced access to supportive care, all of which are crucial for managing the disease effectively, avoiding or delaying complications.
- Many countries due to resource constraints in Africa do not invest in genetic counseling and screening, this has contributed to the high burden of children born with SCD. Without proper screening and counseling, individuals who carry the sickle cell trait may unknowingly pass it on to their children, leading to a higher incidence of the disease.
- Most countries in Africa lack Primary healthcare services (which are Available, Affordable and Accessible), including comprehensive care for individuals with chronic diseases like SCD. Lack of diagnostic tools, specialized treatment centers, and trained healthcare professionals hampers early diagnosis, appropriate management, and preventive measures for sickle cell disease
In 2022 African health ministers launched a campaign to increase awareness, bolster prevention and care to curb the toll of sickle cell disease on the continent on the side-lines of the 72nd WHO Regional Committee for Africa. Has this campaign had any impact on the prevalence of the disease in Africa?
It is certainly work in progress and it is encouraging to take note of these efforts however it’s too early to talk about the impact though. There has been a lot of deliberate effort made towards diagnosis, newborn screening programs now launched in 5 countries in Africa, patient and care giver education, Health Care providers training on SCD and community engagements. Long-term evaluation studies and surveillance programs are needed to assess the impact of awareness campaigns and measure changes in disease prevalence over time. These studies can help determine the effectiveness of interventions and guide further efforts to reduce the toll of sickle cell disease on the African continent.
What barriers, in your opinion, have stood in the way of the campaign’s success?
- Political good will is key and more can be done to achieve more gains in this area.
- Advocacy, legal and policy change to give more room to research
- Low funding
- Awareness of SCD is still very low both at healthcare worker and community levels
- Low focus in our training programmes at primary, secondary and tertiary level institutions
Globally, what policies need to be designed and implemented, and what actions need to be taken, to address these barriers and increase access to prevention mechanisms, early screening and care for sickle cell disease?
It’s important to recognize that policies and actions should be customized to the specific needs and contexts of each country or region. A comprehensive and multi-sectoral approach, involving government agencies, healthcare systems, advocacy groups, community organizations, and affected individuals, is crucial for successfully addressing the barriers to SCD prevention and care on a global scale. To address the barriers and increase access to prevention mechanisms, early screening, and care for sickle cell disease (SCD) globally, several policies and actions can be considered:
- Governments should develop and implement national policies that are specific and focused on SCD
- Establish and expand newborn screening programs for SCD
- Strengthening healthcare infrastructure increasing the availability of specialized clinics, healthcare professionals trained in managing SCD, diagnostic tools, and essential medications and commodities
- Governments should develop and implement comprehensive awareness campaigns targeting communities, healthcare providers, and policymakers
- Ensure that essential medications and treatments for SCD are affordable and accessible.
- Governments should invest in research to advance the understanding, prevention, and treatment of SCD.
How can these policies be implemented in resource-constrained settings at a time when global health funding remains focused on infectious diseases, and development funding for health to low-and middle-income countries is shrinking?
While the challenges are significant, it’s important to emphasize the long-term benefits of addressing SCD, including improved health outcomes, reduced healthcare costs, and enhanced productivity. Collaborative efforts involving governments, civil society organizations, healthcare providers, and affected communities can help drive policy change and resource mobilization even in resource-constrained settings. However, there are several strategies that can be employed to navigate these constraints:
- governments and policymakers should prioritize SCD as a public health concern. This involves allocating a portion of the healthcare budget specifically for SCD prevention, early screening, and care. By highlighting the impact of SCD and the need for increased funding, policies can be influenced to allocate more resources for SCD prevention and care in resource-constrained settings.
- Collaborative research projects can generate evidence, inform policy decisions, and attract additional funding for SCD prevention and management.
- Collaboration with private sector entities, including pharmaceutical companies, can facilitate access to affordable treatments, donations, and technology transfers
- Engaging with international organizations, such as the World Health Organization (WHO) and non-profit organizations focused on genetic disorders, can help draw attention to the importance of addressing SCD and leverage resources and technical expertise for resource-constrained settings.
What role does gender equity in health leadership play in spotlighting the burdens of this disease and advocating for more inclusive and equitable approaches to its prevention and management?
Promoting gender equity in healthcare leadership creates an enabling environment for addressing the burden of SCD in a more comprehensive and equitable manner. By amplifying the voices of women and advocating for their rights and needs, gender-equitable health leadership can drive change, increase awareness, and improve access to prevention mechanisms, early screening, and care for SCD.
If you had the chance to speak to stakeholders and policymakers in health regarding the urgent need for greater investment and stronger collaboration to combat the disease, what would you say?
To combat this disease effectively, we need increased investment. SCD must be recognized as a priority area for policy, research, prevention, screening, and continuum of care. Adequate funding is required to support the development of innovative approaches, diagnostic tools, and treatment options. Investment in health infrastructure, including specialized clinics and trained healthcare professionals and community health workers, is crucial for ensuring timely and comprehensive care for those affected by SCD. Kenya has made significant strides in the work done by community health workers now called community health promoters. However, strong collaboration among stakeholders is equally vital. We must foster partnerships between governments, healthcare systems, private sector, non-governmental organizations, pharmaceutical industry, research institutions, innovators and affected communities.
Combating sickle cell disease requires unwavering commitment, collaboration, and investment. By coming together, we can make a difference in the lives of millions affected by SCD for both patients and their caregivers. Let us seize this opportunity to prioritize SCD on the global health agenda, strengthen health systems, foster collaboration, and ensure equitable access to prevention and care. Together, we can bring about lasting change and improve the lives of those living with sickle cell disease.
