Sickle Cell Disease, a hereditary blood disorder characterised by the presence of abnormal haemoglobin in red blood cells, is prevalent in Kenya.
Individuals affected by this condition exhibit misshapen red blood cells with a significantly reduced lifespan, causing complications such as blood vessel blockages and damage to tissues and organs. SCD is the most common severe monogenic disorder in humans.
For the first time in Kenya, the Ministry of Health has issued National Guidelines for the Control and Management of Sickle Cell Disease. While data in Kenya remains limited, globally, SCD accounts for between six to 15 per cent of deaths in children under five years old.
The prevalence of SCD is particularly higher in malaria-endemic areas. Alarmingly, by 2010, up to 6,000 children were born with SCD annually, and approximately 50-90 per cent of them passed away undiagnosed before their fifth birthday.
The disease is widespread across Kenya, affecting 18 counties, with the highest disease burden observed in Western, Nyanza and coastal regions.
Children under the age of five are at increased risk of death due to infections, anaemia, and other life-threatening sickle cell anaemia complications.
However, many of these complications can be prevented through cost-effective prophylaxis methods, including the administration of penicillin, pneumococcal immunisations and the distribution of malaria bed nets.
Educating parents on the importance of seeking medical attention for fever and other preventive measures for SCD complications is vital, especially if diagnosed early in life.
Inheritance of the HbSS mutation from both parents increases the risk of severe malaria and mortality. Prevalence rates vary across regions in Kenya, mirroring malaria endemicity.
In the Western region, it's estimated that up to 18 per cent of children carry the sickle cell trait, with 4.5 per cent at risk of developing SCD. In the Lake region, approximately 17 per cent of children are carriers, and 0.6 per cent have SCD, while in the coastal region, nearly one per cent of inpatient children have SCD and face a 20-fold increased risk of mortality compared to admissions for other conditions.
Managing SCD patients necessitates a multidisciplinary approach to address both acute and chronic complications. Comprehensive care should begin with early diagnosis, preferably during the newborn period, followed by penicillin prophylaxis initiated before two months of age and continued until five years.
Immunisation practices, safe transfusion procedures, psychosocial support for patients and families, nutritional support with folic acid supplementation, pain management, regular follow-up clinics, the use of hydroxyurea and screening for chronic complications are all integral components of SCD care.
Newborn screening for SCD, particularly in malaria-endemic areas, is crucial and should be universally implemented. Immunisation for children with SCD must align with the Kenya Expanded Programme on Immunisation, covering infectious diseases relevant to SCD.
Blood transfusion plays a pivotal role in SCD treatment, both as a simple top-up transfusion and exchange transfusion, which aims to reduce HbS concentration in circulation to less than 30 per cent.
One of the hallmark symptoms of SCD is excruciating pain due to blood vessel blockages. Parents can be educated on the proper use of analgesics like paracetamol and painkillers.
Psychosocial support is essential as SCD is a chronic condition with numerous challenges for both patients and their families. These challenges include periods of pain, inpatient care, physiological changes, and the potential for complications.
SCD patients are highly susceptible to malaria infections, which can have severe consequences. Prophylactic antimalarial drugs are recommended for those living in high-transmission malaria areas, in addition to general precautions such as the use of insecticide-treated mosquito nets.
Regular reviews, at least every eight to 12 weeks, are necessary for SCD patients, including screening for chronic complications like kidney disease, hypertension, heart disease, pulmonary hypertension, retinal disease, stroke and iron overload.
In conclusion, neonatal screening, along with timely diagnostic testing, parental education, and comprehensive care, significantly reduces morbidity and mortality from SCD in infancy and early childhood.
All children with unexplained acute illnesses, including pain, anaemia, neurological symptoms, vision loss, respiratory issues, organ enlargement, jaundice, swollen limbs and sepsis, should be tested for SCD.
