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SICKLE CELL DISEASE REGISTRY AND
PREVALENCE OF SICKLE CELL DISEASE
IN KENYA
IS IT FEASIBLE?
BY
DR. CONSTANCE N.TENGE
SENIOR LECTURER/PAEDITRICIAN
MOI UNIVERSITY-CHS-SOM
Contents
About sickle cell disease
Burden of sickle cell disease
Public health concern about SCD
A National SCD control programme
Academic mission
SCD registry and prevalence of SCD in
Kenya.
Is it feasible?
References
About sickle cell disease
Sickle cell Anaemia / Drepanocytosis.
Various cell genotypes or variants of the sickle cell syndrome:
Hb AS (SCT) Hb SD- Punjab
Hb SS etc
Hb SC
Genetic or hereditary blood disorder
Inheritance –Autosomal recessive
Perpetuation of the sickle cell gene can be controlled.
Percentage Chance
Normal
Hb AACarrier
Hb AS
Disease
HB SS
Hb AA Hb AA 100 - -
Hb AA Hb AS 50 50 -
Hb AA Hb SS - 100 -
Hb AS HB AS 25 50 25
Hb AS HB SS - 50 50
Hb SS HB SS - - 100
Burden of sickle cell disease
Millions of people throughout the world are affected.
Ancestors lived in tropical and subtropical -Subsaharan
regions
–African, African-American Mediterranean (Italian, Sicilian,
Greek), Middle East, East Indian, Caribbean ,Central or South
American descent.
75-85% of cases occur in Africa.
Affects up to 3% births.
6-9 million infants are born with SCD in Africa each year.
10-40% carrier frequency across equatorial Africa (Kenya 28-
35%).
U.S.A U.S.A
1:5000 affected :90 000 cases : 2.5 million
herogenous carriers.
FRANCE
1 in 2500 affected : 8750 carriers
UNITED KINGDOM
1 in 2000 births with SCD
Public Health concern Public health Implications of sickle cell disease are
significant :
-High morbidity and mortality (5% under five deaths)
- Socio-economic burden
No firm data on burden and survival of patients with
SCD on the African Continent.
Management of SCD in most African countries remains
inadequate.
National SCD control programmes do not exist.
The need for a National Control Programme
Provide a comprehensive approach to prevention
and management of SCD.
Simple affordable and accessible technology that
is feasible so as to benefit a large proportion of
the community
Healthcare system able to provide basic
requirements
EDUCATION and RESEARCH activities to provide
evidence based practice and fill the knowledge gap.
Academic Mission EDUCATION
-Training of health care workers on prevention, diagnosis and management.
-Public Education and awareness on genetic risks and carrier detection before
marriage or pregnancy
CARE
-regional working group experts to coordinate activities and develop
guidelines and work closely with primary care providers.
-Health care system that should provide the basics to patients( Pen V, Folate,
Hydroxyurea )
- Carrier detection screening programme ( Sickling test )
- Neonatal screening
RESEARCH
-Research and surveillance
- Vital statistics reporting systems to guide changes in health policy.
-Planning and evaluating appropriate interventions.
SCD registry and prevalence of SCD
Aim
To set up a sickle cell registry and determine the
prevalence of sickle cell disease in Kenya
Objectives
To set up a sickle cell registry in Kenya
To describe the socio- demographic characteristics of
patients with sickle cell disease in Kenya
To evaluate the care received by the patients
To provide a data base for other studies on Sickle Cell
Disease
NAMES
Constance N. Tenge 1, Wilson K. Bett 2, Mercy Mulaku 3, Festus M.
Njuguna 4, Meshack Liru 2, Teresa C Lotodo 1 , Pamela A Were 4 ,
Sarah Awino 4, Juliana Otieno 5, Evallyne S Sikuku 6, Rachael Nyamae2, Anne Wamae 2, Fatuma Abdalla7 , Walter Mwanda 7, Mike English3.
AFFILIATIONS
1 . Moi University – College of Health Sciences( MU – CHS )
2 . Ministry of Health – Government of Kenya ( MOH – GOK )
3 . Kenya Medical Research Institute ( KEMRI )
4 . Moi Teaching and Referral Hospital ( MTRH )
5 . Jaramogi Oginga Odinga Teaching and Referral Hospital ( JOOTRH )
6. Academic Model Providing Access To HealthCare ( AMPATH )
7. University of Nairobi - College of Health Sciences ( UON – CHS )
METHODS Preparation of relevant education material and
dissemination.
- Health care providers-conferences and workshops,
CMEs. etc.
- Communities - baraza’s hospitals ,health camps etc.
Gradual and target the different regions/ provinces
systematically
- Level 4,5 and 6 government hospitals
- Mission and private hospitals
Identify the patients and profile them-use of
questionnaires
-demographic characteristics
-family information
-Mode of Diagnosis of SCD
-Medical information.
?? SCD Registry
?? Prevalence of SCD in Kenya.
?? National SCD control programme.
IS IT FEASIBLE? ??
What role can you play??
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