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??

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