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.
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
7. Objectives
Objectives
• To establish national prevalence data
•TTo evaluate the pattern of drugs
l t th tt fd
usage by institution
usage by institution
• To evaluate serum ferritin levels
• To predict the minimal incidence
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; ……..
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
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