1. SICKLE CELL DISEASE REGISTRY AND PREVALENCE OF SICKLE CELL DISEASE IN KENYA IS IT FEASIBLE? BY DR. CONSTANCE N.TENGE SENIOR LECTURER/PAEDIATRICIAN MOI UNIVERSITY-CHS-SOM

2. 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

3. 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.

4. Percentage Chance Normal Hb AA Carrier Hb AS Disease HB SS Hb AA 100- - Hb AAHb AS50 - Hb AAHb SS-100- Hb ASHB AS255025 Hb ASHB SS-50 Hb SSHB SS--100

5. 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%).

6. 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

7. Public Health concern  Public health Implications of sickle cell disease are significant : -High morbidity and mortality (5% under five deaths) - Socio-economic burden  No reliable 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.

8. 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.

9. 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.

10. 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

11. Constance N. Tenge 1, Wilson K. Bett 2, Mercy Mulaku 3, Meshack Liru 4 Festus M. Njuguna 5, Teresa C Lotodo 1, Pamela A Were 5, Sarah Awino 5, Juliana Otieno 6, Evallyne S Sikuku 7, Rachael Nyamae 4, Anne Wamae 4, Fatuma Abdalla 8, Walter Mwanda, 8 Mike English 3. AFFILIATIONS  1. Moi University – College of Health Sciences( MU – CHS )  2. Egerton University- School of Medicine ( EU – SOM )  3. Kenya Medical Research Institute ( KEMRI )  4. Ministry of Health – Government of Kenya ( MOH – GOK )  5. Moi Teaching and Referral Hospital ( MTRH )  6. Jaramogi Oginga Odinga Teaching and Referral Hospital ( JOOTRH )  7. Academic Model Providing Access To HealthCare ( AMPATH )  8. University of Nairobi - College of Health Sciences ( UON – CHS )

12. METHODS  Preparation of relevant education material and dissemination. - Health care providers-conferences and workshops, CMEs. etc. - Communities - baraza’s,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.

15.  ?? SCD Registry  ?? Prevalence of SCD in Kenya.  ?? National SCD control programme. IS IT FEASIBLE? ?? What role can you play?? Intellectual support(ideas) Technical support(personnel) Material support(funds ) Social support

16. There is no passion to be found playing small, in settling for a life that is less than the one you are capable of living

17. May our choices reflect our hopes, not our fears…

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22. Cont…  Awasthy N., Aggarwal K. C., Goyal P. C., Prassad.M. S., Saluza S., Sharma M. (2008). ‘Sickle cell disease. Experience of a tertiary care centre in a non endemic area’ Annals of Tropical Medicine and public health 1(1) 1-4 dos.10.4103/1755-6783.43069.  Aluoch J. R., Aluoch L. H. Survey of sickle cell disease in Kenya. Trop. Geoga Med 1993 Mar; 45(1): 18-21  Glassberg J. (August 2011). ‘Evidence-based management of sickle cell disease in the emergency department’ emergency medicine practice 13(8):1020; quiz 20. PMID 22164362  Akinyanzu O. O., Otaigbe A. L., Ibidapo M. O. Outcme of holistic care in Nigerian Patients with sickle cell anaemia. Clin Lab Haemaful 2005; 27:195-199 doi.10.1111/5. 1365-2257.2005 00683. X PMID 15938726

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