Sickle Cell Disease in Nigeria: Why We Must Focus on Advocacy

17Sep 2024 by Kiara Ekeigwe

The authors—Adeniyi Adesola, David Akoki, Joshua Lawal, Abigail Oyedokun, Anuoluwapo Babawale, and Oluwatomisin Agboola—are part of a Mentored Child Health Research Project led by the Duke Center for Policy Impact in Global Health and the University of Ibadan Medical Students’ Association.

Background: the scope of the disease

Imagine a world where your own blood turns against you. That is the reality for millions of people living with sickle cell disease (SCD), a genetic condition that turns red blood cells into rigid and fragile, crescent-shaped obstacles. These cells can clog blood vessels, causing severe pain and life-threatening complications. Globally, Nigeria bears the highest burden—about 2% to 3% of the population has the disease—with about 150,000 newborns affected yearly and 50 million people carrying the sickle cell trait.1 Nearly half of affected children die before their fifth birthday.2 Those who survive face a life expectancy that hovers around 21 years, much lower than the life expectancy of people with SCD in high-income nations, which is 54 years.3,4

Beyond causing frequent bone pain crises, SCD causes a range of complications such as stroke, swelling of the hands and feet (dactylitis), anemia, leg ulcers, infections, osteonecrosis, and priapism. There is a high prevalence of depression and anxiety, exacerbated by the stigma and stress of frequent hospitalizations. For example, a systematic review of prevalence studies estimated a prevalence of depression in people with SCD of around 29-35%.5 This disease often disrupts education and career aspirations, as frequent absences from school or work can hinder academic and professional achievements. Socially, individuals may face relationship challenges. The implications go beyond individuals as the high costs of managing the disease, coupled with the emotional toll of losing children, can strain families, sometimes leading to disharmony.‌6,7

How did we get here?

The high prevalence of SCD in Nigeria, and its associated morbidity and mortality, is driven by many factors.  Neonatal screening has proven to be an effective means of reducing mortality in SCD through early identification for supportive care, which includes early initiation of prophylactic penicillin, pneumococcal vaccination, and counseling for the parents.8 Nigeria, however, lacks a coordinated national neonatal screening programme, which leads to late diagnosis, usually at the onset of complications.1,9 These delays in diagnosis and intervention often lead to preventable complications and death.10

SCD presents a significant public health challenge in Nigeria—the prevalence is high, yet public awareness remains inadequate.11 The condition is further complicated by pervasive health-related stigma, which manifests in various forms. Patients with SCD report experiencing stigmatization from family members, the general public, and even healthcare professionals.12 This stigma significantly impedes access to care and contributes to the perpetuation of misconceptions about the disease. One such misconception is evident in the colloquial term ‘sicklers,’ used to describe individuals with SCD. While this term originates from the characteristic sickle shape of red blood cells during a crisis, it is often misinterpreted as a reference to frequent hospital admissions. This stigmatizing language, coupled with other forms of discrimination, can lead to patients attempting to conceal their true genotype. In some cases, individuals go as far as deliberately falsifying their hemoglobin genotype test results to avoid the stigma associated with SCD.

Another offshoot of the ailing health infrastructure that feeds into the perpetuation of SCD is the incidence of false results. The low adoption of quality assurance management systems in the running of laboratories in Nigeria has led to otherwise careful individuals having children with SCD,13 with even the little progress made in the area of premarital screening being undermined by the menace of false laboratory results. Additionally, the decision by informed carriers to take risk has contributed to children being born with SCD. On the other hand, ignorance about the implication of a union between two carriers of the sickle cell gene has led to the continued perpetuation of the disease among the unknowledgeable.

The economic cost of tackling the disease burden is a major hindrance to a successful execution of any strategy to rein in the disease. Government allocation is stretched thin between many health priorities. Universal health coverage is still a pipe dream, and families are left to seek care out of pocket, with some paying for healthcare. One study in Nigeria found that 61% of households in the poorest quintile experienced catastrophic health spending due to SCD, where such spending was defined as over 40% of the income that is available after basic needs have been met.14

Despite the national house of assembly’s passage of a SCD bill focused on prevention, control, and effective management, wider implementation has been stalled by a delay of presidential assent. The government, despite its far-reaching capacity for wider impact, has not invested in awareness programmes, leaving a large proportion of the population unaware of SCD and its prevention.

Steps towards change

We need a comprehensive approach to curbing the burden of SCD that includes awareness, research, and advocacy. Targeted awareness efforts need to be adopted and intensified across various levels of society. For a medical condition as specific and burdensome to Nigeria as SCD, there should be robust representation in educational curricula as early as primary education through to tertiary education and even the one-year national youth service. This education should be programmed to enable individuals to make truly informed decisions regarding their own sickle cell status, especially when considering conjugal union compatibility.

In 2021, the Nigerian Senate passed the Sickle Cell Bill, which aimed to tackle SCD through a three-pronged approach of prevention, control, and management. However, as mentioned, there are significant gaps in implementation and execution of the bill, especially with regards to control and prevention. To effectively address SCD as a public health crisis, a comprehensive strategy is imperative, one that prioritizes raising awareness about SCD and establishing robust control and prevention measures nationwide. While non-government organizations (NGOs) have been at the forefront of advocacy efforts, the government has a critical role—indeed, a collaborative effort involving all stakeholders is essential. It is time to revisit the 2021 bill and establish a clear roadmap to reducing the burden of SCD in Nigeria.

The already high prevalence of SCD is further exacerbated by inaccurate genotype testing. Therefore, it is crucial to enhance advocacy for stringent quality control measures in genotype testing. Currently, the predominant testing method in Nigeria, hemoglobin electrophoresis, is susceptible to errors, underscoring the need for more advanced and reliable diagnostic techniques.15 Also, to ensure the highest standards of care, only licensed health professionals should conduct SCD tests within accredited facilities, with a comprehensive list of these facilities publicly accessible to facilitate informed patient choice.

At the intersection between awareness and research emerges the importance of advocacy to translate the insights from research into more precise awareness initiatives and more practical policy making and implementation. This advocacy should be targeted at the entire spectrum of relevant stakeholders in the fight against SCD. These stakeholders include the ministries of health and education, NGOs, the National Youth Service Commission, religious organizations, public and private employers, and local and national policymakers, amongst others.

Conclusion

While there have been great strides in treating SCD, such as hematopoietic stem cell transplantation and gene therapy, they are currently impractical on a large scale. These options are both costly and inaccessible to most Nigerians, given the country’s lower middle-income status. Thus, prevention remains the most effective strategy to drive down the disease burden, which will require a comprehensive and concerted advocacy effort. Acknowledging the burden of SCD is a starting point but much more needs to be done. Complementary efforts geared towards awareness, research, and advocacy need to be made in an iterative manner in pursuit of significant improvement in SCD indices and outcomes in Nigeria.

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