Today marks the World Sickle Cell Day, a condition arising from a genetic mutation in the haemoglobin molecule.
This leads to a loss of structural and functional integrity of affected red blood cells, impairing their oxygen transport.
Sickle cell manifests in various ways but its hallmark feature is “painful crises” that affect multiple organs.
Though a hereditary condition, not all offspring are affected, with some expressing milder forms of the disease and labelled “carriers”.
No accurate data is available on sickle cell anaemia prevalence in Kenya but it seems to be clustered around Lake Victoria, western Kenya and the coastal regions. One survey reported 80 percent of patients were of Luo or Luhya origins (Luo 58.4 percent, Luhya 23.9 percent) with the rest being Mijikenda community. However, migration has seen these communities resettle in urban areas.
In Nairobi, for instance, ethnic clustering in some settlements has created pockets of such patients in Mathare, Kibera and Kariobangi.
Intermarriages have also over time generated intercommunal inheritance of the trait beyond these communities.
Estimates in a past study showed 77 per cent of sicklers were below 15 years, with a paediatric to adult ratio of 3:1. Because of this, children bear the brunt of the condition, and, therefore, the need to have an entity championing for this group’s special needs.
The Children Sickle Cell Foundation is dedicated to raising awareness, supporting parents, children and caregivers of those affected with sickle cell anaemia. Key advocacy areas are the provision of holistic child-friendly services and supporting clinicians, parents and caretakers to achieve this.
Outside the endemic zones, not many clinics have expertise, diagnostics or facilities to care for such children. To address this, the foundation is spearheading efforts to raise awareness about sickle cell anaemia and collaborate with health facilities to better care for such patients.
As the majority of sicklers are school-going, disruption of their education is common. The foundation works with education institutions to understand their special needs in hygiene. Clean toilets and drinking water are crucial, given oral hydration is part of preventive management of crises.
Teachers are also encouraged to understand when these children are sick or unable to do strenuous exercise that could dehydrate them.
For those in boarding schools, environments conducive for their learning and avoiding infections that could trigger crises is vital.
Presently, the foundation is advocating for clinical guidelines to standardise care across facilities and to help clinicians be aware of the condition, its diagnosis as well as treatment. It is also advocating cost reduction of medications and for insurers not to exclude sickle-cell anaemia from their covered conditions or raise premiums for those afflicted.
Given its chronicity and economic costs, there is also a need for early diagnosis and screening support like happens in other countries.
Finally, there is a need to have access to hydroxyurea, a drug that significantly improves the quality of life, according to the foundation.