Current Situation in Control Strategies and Health Systems in Asia - Sri Lanka by Rasnayaka M Mudiyanse, Senior Lecturer in Paediatrics at the University of Peradeniya and Consultant Paediatrician at the Teaching Hospital in Peradeniya.

1. Thalassaemia in Sri Lanka;
Prevalence and Prevention
l d i
Rasnayaka M Mudiyanse
M Mudiyanse

2. Country Sri Lanka
Country – Sri Lanka
• Size – 65 610 km2
• Population – 20 653 million
• Per capita income 2399 USD
Per capita income 2399 USD
• Nine province 25 Districts  Grama Seva divisions
one officer per 500 – 1000 f ili
ffi 500 1000 families
• Nine Provincial Directorates of Health  291 Medical
Officer of Health (MOH) 43 PHM per 100 000
population
• Total Births (2010) ‐ 364 565
• Total Marriages (2010) ‐ 200 985
Total Marriages (2010) 200 985

3. Country Sri Lanka
Country – Sri Lanka
• Life expectancy – M‐68.1, F ‐76.6 yrs
Life expectancy – M 68 1 F 76 6 yrs
• Literacy ratio ‐ 92%.
y
• Vaccination coverage ‐ 90%.
• CMR – 10.4 per 1000 live births
• IMR – 8.5 per 1000 live births
IMR 8 5 per 1000 live births
• NMR ‐ 5.9 per 1000 live births
p
• MMR ‐ 14.2 per 100 000 births

4. History of Thalassaemia in Sri Lanka
History of Thalassaemia in Sri Lanka
• 1950 – four cases of Cooley’s anemia in
y
Sinhalees by C C De Silva at el
• 1959 – 15 cases of HbE beta thalassaemia C C
De Silva and Nagarathnam
De Silva and Nagarathnam
• 1962 – Three families with Hb S
• 1995 – 2011 – more advanced studies
1. C. C. de SILVA and C. E. S. WEERATUNGE , COOLEY'S ANAEMIA IN SINHALESE
1 C C d SILVA d C E S WEERATUNGE COOLEY'S ANAEMIA IN SINHALESE
CHILDREN , Achieves of disease of childhood 1950
2. N. SAHA and B. BANERJEE idence of Abnormal Haemoglobins in Different Ethnic
Groups of Indians Humangenetik 11,300‐‐303 (1971)
G f I di H tik 11 300 303 (1971)
3. C. C. DE SILVA, M.D., F.R.C.P. D. T. D. BULUGAHAPITIYA, M.B., B.S. JUSTIN DE SILVA
SINHALESE FAMLY WITH HAEMOGLOBIN S BY BRITISH MEDICAL JOURNAL June 2 1962

5. Thalassaemia in Sri Lanka ‐ Prevalence
Sri Lanka Wayamba
Beta thalassaemia 2350 220
Hb E /b t th l
E /beta thal.. 1060 260
Total number of pts
Total number of pts 3410 480
Gene frequency beta
frequency beta 0.011
0 011 0.0125
0 0125
Gene frequency e bata 0.0025 0.0075
1. Anuja Premawardana, Life Sciences & Medicine, Human Molecular Genetics Volume13,
Thalassemia in Sri Lanka: a progress report ,
2.
2 Shanthimala de Silva, Lancet 2000: 355: 786 91 Thalassaemia in Sri Lanka:
Silva 786–91,
implications for the future health burden of Asian populations

6. National Survey on Prevalence of
National Survey on Prevalence of
thalassaemia in Sri Lanka ‐ 2011

7. Objectives
Objectives
• To establish national prevalence data
•TTo evaluate the pattern of drugs
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usage by institution
usage by institution
• To evaluate serum ferritin levels
• To predict the minimal incidence

8. Methodology
• Permission from the ministry of health SL
• Contacted all the institutions with facilities for
blood transfusion
• Collect data
Collect data
– Age or date of birth
– Sex
– Diagnosis (beta thalassaemia, E beta, HB S or other)
– Last ferritin level
– Amount of chalation drugs used by the institution
g y

9. Results
Results
• Total number of institutions contacted –
42 out of 81
42 out of 81
• Hospitals with at least one patient ‐19
• Total number of patients traced ‐ 1547
• Details available up to date – 1379 ( 11
(
hospitals)

10. Evaluation of 1379 patients
Evaluation of 1379 patients
• Male ‐ 668 (48.4%)
• Female ‐711 (51.6%)
F l 711 (51 6%)
• beta thalassaemia – 926 (67.1%)
beta thalassaemia – 926 (67 1%)
• e beta thalassaemia 305 (22.1%)
e beta thalassaemia ‐ 305 (22.1%)
• Others – 148 ( 10.7%)

11. Average age after evaluation of 1231
patients
• Global average age ‐ 12.6 (SD 5.9) yrs
• Beta thalassaemia
Beta thalassaemia
– male 11.9 ( SD 3.8) yrs
– female 11.4 (SD 4.8) yrs
• HbE/Beta thalassaemia
HbE/Beta thalassaemia
– Male ‐ 5.3 (SD 6.0) yrs
– Female 16.1 (SD 5.6) yrs.

12. Thalassaemia in Sri Lanka ‐ Prevalence
Lancet 2000 National survey
2011
2011 ‐ 2012
Beta thalassaemia 2350 1020 ( 64.8%)
Hb E /beta thal
/ 1060 348 ( 22.1%)
( )
Total patients
Total patients 34 0
3410 1574
574
Gene frequency 0.011 0.0073
Beta
B t (CR 2 2%)
2.2%) ( C.R. – 1.45%)
( C R 1 45%)
Gene frequency E 0.0025 0.0013
beta (C.R. ‐0.5%) ( C.R.‐ 0.257%)
Incidence 60‐80 66
Shanthimala de Silva at el Lancet 2000: 355: 786–91, Thalassaemia in Sri Lanka; ……..

13. Age Frequency Distribution
1231 patients
300
250
200
150
Series1
100
50
0
0‐4 yrs 5‐9 yrs 10‐14 15‐19 20‐24 25‐29 30‐34 35‐39 40‐44 45‐49 50‐54 55‐59 60‐64 65‐69 70‐74 75‐79
yrs yrs yrs yrs yrs yrs yrs yrs yrs yrs yrs yrs yrs yrs

14. Incidence of thalassaemia in SL?
Incidence of thalassaemia in SL?
450
400
350
300
250
200
150
100
50
0
5‐9 yrs 10‐14 yrs 15‐19 yrs
Live Deaths

15. Thalassaemia Prevention
Th l i P ti
Teenage screening and promoting
safe marriages by motivating the
public through education
bli th h d ti

16. Thalassaemia Prevention
Thalassaemia Prevention
• Education campaign
• Screening protocol
• Strategy of Prevention
• Monitoring the progress

17. Education to Promote Screening
Education to Promote Screening
• School children – Examination question
• Higher education institutions
Higher education institutions
• General public
– Media
– Lectures
• Professionals
– H lth
Health
– Registrar of marriages, GS

18. Screening Protocol
Routine process
• Step 1 – FBC – above 15 yrs, volunteer
– Green card if MCV > 80 fl and MCH > 27 pg
– Iron therapy if MCV = or < 80 fl or MCH = or < 27 pg
• Step 2 – Repeat FBC after 3 mo Iron therapy
Step 2 Repeat FBC after 3 mo Iron therapy
– Green card if MCV > 80 fl and MCH > 27 pg
– HPLC if MCV
HPLC if MCV = or < 80 fl or MCH = or < 27 pg
80 fl MCH 27
• Step 3 – HPLC for confirmation
– Pink card and counseling for confirmed carriers
– If HPLC is negative  hematologists opinion
g g p

19. Screening Protocol
who need early results
• Step 1 – FBC and HPLC
– Green card if MCV > 80 fl and MCH > 27 pgpg
– Red card for HPLC positive thal traits
– Iron therapy if MCV or < 80 fl or MCH or < 27
Iron therapy if MCV = or < 80 fl or MCH = or < 27
pg
• Step 2 – Repeat FBC and HPLC after 3 mo of
Iron therapy
– Evaluate for false positive HPLC or MCV/MCH

20. Counseling and Education
Counseling and Education

21. Monitoring the Prevention Program
Monitoring the Prevention Program
• Percentage of screen coverage (by
PHM division)
• Incidence of high risk marriages
Incidence of high risk marriages
• Incidence of high risk pregnancies
Incidence of high risk pregnancies
• Incidence of thalassaemia births

22. Percentage of Screening
Percentage of Screening
• At PHM level
• 1000 families per PHM
1000 families per PHM
• Indicator of success of educational
campaign
• Indicator of success
f
• 50% screening  75% reduction of
50% screening  75% reduction of
incidence high risk pregnancies

23. Incidence of High Risk Marriages
Incidence of High Risk Marriages
• To be reported by the registrar of marriages
• One registrar of marriage register 500 – 1000
One registrar of marriage register 500 1000
marriages per year
• When to inquire about thalassaemia status
q
• Maintaining confidentiality
• Both partners have the right to know

24. Incidence of High Risk Pregnancies
Incidence of High Risk Pregnancies
• All the mother are registered
• From antenatal clinic
From antenatal clinic
• Late to know but help to monitor

25. Incidence of Thalassaemia Births
Incidence of Thalassaemia Births
• To be reported by pediatricians
• Late indicator
Late indicator
• Helps to plan the management

26. Thank you