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

ContentsAbout sickle cell diseaseBurden of sickle cell diseasePublic health concern about SCD A National SCD control

programmeAcademic missionSCD registry and prevalence of

SCD in Kenya. Is it feasible?References

About sickle cell diseaseSickle cell Anaemia /

Drepanocytosis.Various cell genotypes or variants of

the sickle cell syndrome:

Hb AS (SCT) Hb SD- Punjab

Hb SS etc

Hb SCGenetic or hereditary blood disorderInheritance –Autosomal recessive Perpetuation of the sickle cell gene

can be controlled.

Percentage Chance

Normal Hb AA

Carrier 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 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.

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

 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 Abdalla8 , Walter Mwanda ,8

Mike English3.

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 )

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.

?? 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

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

May our choices reflect our hopes, not our fears…

REFERENCES Herrick J. B. (1910). “Peculiar elongated sickle-

shaped red blood corpuscles in a case of severe anaemia arch. Intern. Med. 6(5): 517-521

Savitt T. L., Goldberg M. F. (Jan1989). “Herrick’s 1910 case report of sickle cell anaemia. The rest of the story”. JAMA 261 (2): 266-271

Mason V R (1922). ‘Sickle Cell Anaemia’ J A M A 79 (14) : 1318 – 1320.

Malowany, J. I., Butany J. (2012). Pathology of sickle cell disease. Seminars in diagnostic pathology 29(1):49-55

Lazarus, Hillard M.; Schmaier, Alvin H. (2011). Concise Guide to Haematology, Wiley – Blackwell. P.8. ISBN 1-4051-9666-1

Cont.. Kumar, Vinary; Abbas, Abul K.; Fausto, Nelson;

Aster, Jon (May 2009). Robbins and cotran pathologic basis of disease professional edition: expert consult – online (Robbins patho

Clarke G. M., Higgin, T. N. (August 2000). “Laboratory Investigation of haemoglobinopathies and thalasaemias: review and update” Clin.Chem. 46(8 pt 2): 1284-1290. PMID 10926923

Allison A. C. (October 2009). “Genetic Control of resistance to human malaria”. Current opinion in immunology 21(5): 499 – 505 PMID 19442502

Kwiatkowski D. P. (August 2005). “How malaria has affected the human genoma. What human genetics can teach us about malaria’ American Journal of Human Genetics. 77(2): 171-192. ISSN 002 – 9297. PMC 1224522. PMID 16001361

Cont..

Weatherall D. J., Clegg J. B. (2001) inherited haemoglobin disorders; an increasing global health problem. Bull world health organ 2001; 79: 704-712. PMID 11545326.

Angastiniotis M., Modell B. Global epidemiology of haemoglobin disorders. Ann N Y. Acad SCI 1998; 850: 251-269. PMID 9668547

Roberts I., de Montalembert m. (July 2007). Sickle cell disease as a paradigm of immigration haematology: new challenges for haematologists in Europe. Haematologica 92(7): 865-871. Doi.3324/haematol 11477. PMID 176 06434

Grosse SD, Odame I, Atrash H. K., Amenda D. D., Plel F. B., Williams T. N. Sickle cell disease in Africa: A neglected cause of early childhood mortality. AM. J prev. Med. 2011 Dec; 41 (suppl 4): S 398 – 405

Cont.. Suchdev. P. S., Ruth L. J., Early M., Macharia A. Williams T. N.

The burden and consequences of inherited blood disorders among young children in Western Kenya. Matern Child Nutri. 2012 Sep 13

National Heart, Lung and Blood Institute ‘Sickle cell anaemia, key points. Retrieved 2010-11-27

Cinnchinsky E. P., Mationey D. H., Landlaw S. A. (Nov.2011). “Uptodate: sickle cell trait’

Bardakdjian J., Wajcman H. (September 2004) Epidemiology of sickle cell anaemia. Rev. prat (in French/54(14): 1531-3 PMID 15558961

http://www.nhs.uk/conditions/sickle-cellanaemia/pages/introduction.aspx

Jastanian W. (2011). ‘Epidemiology of sickle cell disease in Saudi Arabia’. Annals of Saudi Medicine 31(3): 289-293 doii 10.4103/0256 – 4947.81540. PMC 3119971. PMID 21623060

WHO. ‘Sickle Cell Anaemia’ – Report by the Secretariat’ (PDF) retrieved 2010-11-27

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

Cont… Steinberg M. H., Barton F. Castro O., et al. Effect of

hydroxyurea on mortality and morbidity in adult sickle cell anaemia: risks and benefits up to 9 years of treatment. JAMA 2003; 289 (13): 1645-1651.

Strouse J. J., Lanzkron S., Beach M. C. et al. Hydroxyurea for sickle cell disease: A systematic review for efficacy and toxicity in children. Paediatrics 2008: 122(6): 1332-1342

Walters M. C., Patience M., Leisenring W. et al (August 1996); Bone marrow transplantation for sickle cell disease’ New England Journal of Medicine 335(6): 369-376 doi:10.1056/NEJM 199608083350601 pmid 8663884.

Sickle cell anaemia. Agenda item 11.4 in: 59th World Health Assembly, 27 May 2006. WHA 59.20. Available from http://who.int/gb/ebwha/pdf files/WHA 59 - RECI/e/WHA 59 2006 REC - en pdf p.26

Creary M., Willaimson D., Kulkarni R. Sickle cell disease: current activities, public health implications, and future direction. J. Women's Health (Larchmt/. 2007 June 16(5): 575-582