NAIROBI, Kenya, Aug 6 – Kenya has developed its guidelines for control and management of sickle cell disease (SCD) which will be used to bring to light the interventions needed to control and manage the disease.
The first edition also lays emphasis on early diagnosis and the measures required to reduce the impact of complications and mortality.
“The emphasis must now move towards early detection and prevention of long-term complications of sickle cell disease. This early diagnosis of Sickle Cell Disease is important to initiate prompt management,” the new guidelines state.
Sickle Cell Disease is caused by a disorder in the haemoglobin component of blood leading to abnormal sickle haemoglobin (HbS). Individuals with sickle cell disease exhibit significant morbidity and mortality.
At least 240,000 children in Africa are born each year with Sickle Cell Disease of which an estimated 6,000 are in Kenya alone (one in every 150 new-borns) in 2010 and this number would rise to over 10,000 (one in every 100 new-borns) per year by 2050 given the projected population growth.
The distribution reflects the fact that Sickle Cell Trait confers a survival advantage against malaria and that selection pressure due to malaria has resulted in high frequencies of the mutant gene especially high malarial transmission areas.
In the absence of new-born screening and appropriate treatment, majority of such children die undiagnosed in early childhood from preventable causes such as malaria and bacterial infections.
In Sub-Saharan Africa, an estimated 50-90% of those born with the condition die undiagnosed before their 5th birthday.
The disease is common across Kenya with high disease burden pockets in Western, Nyanza and Coastal regions.
In the western region, it is estimated that as high as 18% of children are born with a Sickle Cell Trait and 4.5% will end up developing SCD. In the lake region, it is estimated that about 17% children are carriers of the trait with 0.6% having SCD while in the coastal region, using inpatient data, almost 1% of inpatient children have SCD and are almost 20 times likely to die compared to admissions of other morbidities.
Movement in search of better livelihoods leads to a wider spread of the areas previously considered endemic zones.
Health Cabinet Secretary Mutahi Kagwe lauded the development of Kenya’s premier Sickle Cell Disease Control and Management Guidelines. “The guidelines propose delineation of unique levels of service delivery for patients with Sickle Cell Disease aligned with the Kenya Essential Package for Health levels,” said Kagwe.
Globally, it is estimated that Sickle Cell Disease (SCD) causes between 6-15% deaths in children aged less than 5 years. There is paucity of data in Kenya, but in general, malaria endemic areas have a higher prevalence of SCD.
Interventions to control the disease include provision of prompt and effective management, advocacy, communication and social mobilization for screening, genetic counselling during premarital courtship. A child born with sickle cell disease is ten times likely to die than a normal child.
Morbidity and mortality have been high in young children with sickle cell disease. However, recent studies have shown that early diagnosis and supportive care have significant impact in the reduction of complications, mortality and improved quality of life.
The guidelines on the management of SCD in the country include new born screening including having nationwide screening of all newborns for SCD but this comes with challenges of limited resources.
Inclusion of children born at home has a financial implication to the health budget. A targeted approach where all newborns in high endemic areas are tested shortly after birth and early screening and testing of suspected children in potentially low endemic areas may be used as an alternative.
Penicillin Prophylaxis – Patients with SCD are prone to infection and death from encapsulated bacterial organisms like Streptococcal pneumonia due to splenic dysfunction that occurs within the first 5 years of life. It is therefore, recommended that children with SCD should have penicillin prophylaxis started within 2 months of life and continued until 5 years of age.
Inoculation practices – Children with SCD are required to get all the vaccines as per the Kenya Expanded Programme on Immunization which covers infectious diseases that are relevant to SCD like Streptococcal pneumonia, Hemophilus influenza type B and Hepatitis B.
Hydroxyurea Use – The Realizing Effectiveness Across Continents with Hydroxyurea (REACH) trial, in which Kenya was one of the four participant countries, confirmed the clinical benefits of hydroxyurea in SCD like reducing painful crisis, blood transfusion and infection in patients.
Other guidelines include Blood Transfusion, Pain management, Psychosocial and Nutritional support as well as management of acute complications.
“Overall improvement of quality of life is addressed. The rational for this guideline takes cognizant of the challenge’s patients with sickle cell disease face and the complexities in provision of health care services thus calling for significant health system changes,” Acting Director-General, Ministry of Health Patrick Amoth said.
He further noted that the guidelines position Sickle Cell Disease care on a life course platform addressing patient needs at different stages from the newborn through to adulthood.
