THALASSAEMIA

PREVENTION & CONTROL

STRATEGIES IN MALAYSIA
Dr Hajah Hayati Mohd Radzi
Senior Consultant Public Health Physician
Director
Family Health Development Division
Ministry of Health

Thalassaemia Conference
Sunway Putra, 20th May 2017
 International Framework of Thalassaemia Prevention and Control
Presentation
Outline Elements of Prevention and Control Policy

Success Story

Status of Thalassaemia in Malaysia

Thalassaemia Control Strategy in Malaysia

Thalassaemia Score Card

Issues and challenges

Conclusion
Thalassaemia Cost of Thalassaemia treatment is
Prevention costly and each patient requires
lifelong treatment
and Control is
essential for
Blood transfusion and chelation has
the following prolonged patient survival
reasons;

Failure adhering to treatment regime

resulted in additional cost for
managing complication and infections
related to Thalassaemia
Net effect is patients live longer but
poor quality of life

Mechanism must be in place to Reduce new birth of Thalassaemia patient

Reduce complication related to the Disease
Improve survival and quality of life
WHO resolution on thalassemia and other hemoglobinopathies (EB118.R1, May
2006) adopted by the 63rd World Health Assembly in May 2010

WHO Commitment : Member states:

1. Increase awareness of the
international community of 1. To design, implement and reinforce in a systematic, equitable
the global burden of these and effective manner, comprehensive national, integrated
disorders; programme for prevention and management of thalassaemia
and other hemoglobinopathies
2. Promote equitable access
to health services; 2. Including surveillance, dissemination of information,
3. Provide technical support awareness-raising and screening,
to countries for the
prevention and
management of these 3. Tailored to specific socioeconomics and cultural contexts and
disorders; and
4. Promote and support
research to improve quality 4. Aimed at reducing the incidence, morbidity and
of life for those affected.
mortality associated with these diseases.”
Thalassaemia Situational Analysis in
Malaysia
 117
DISTRIBUTION OF THALASSAEMIA
681 PATIENTS AS OF 31st DECEMBER 2016
446 357 260

548
Total : 7112

1081 355 20 1694

537 162
23
211 559

Thalassaemia patients by diagnosis

188
Others 6.3

Beta thalassaemia intermedia 10.1

Hb H disease 14.1

Beta thalassaemia major 32.2

HbE-beta thalassaemia 33.3

 Thalassaemia Prevention and Control Program Goals

Short term goal

Awareness on importance of Thalassaemia Patient Education on compliance
Prevention

Medium term goal

Increase acceptance and uptake of Thalassaemia
Reduce complication related to Thalassaemias
carrier screening among target population

Long term Goal

Reduction of new Thalassaemia birth among
screened population Increase survival and quality of Life
 Thalassaemia Prevention and Control Program Components
Comprehensive
Population awareness Population screening Thalassaemia registry
patient management
• Health Education Division • Family Health • Medical Division • Medical Development
• Family Health Development Development Division • Blood Bank Division
Division • Radiology and Lab
• Pharmaceutical Division
 Create public awareness  Define population  Comprehensive  Hospital based patient
about disease and screening policy patient care registry
 Motivate attitude and  Setup screening  Access to affordable  Provide profiles on
behaviour change standards and treatment for all Thalassaemia disease
towards carrier guidelines patient in Malaysia
screening  Monitor carriers and  Blood safety  Capture patient care
 Develop understanding evaluate reduction in  Standard of patient plan and progress
on diseasea linage in new bith of care and guidelines  Monitor individual
prevention Thalassaemia patients  Transition from patient progress
 Empower and tap young  To Set up children to adult care  Monitor and evaluate
talents in developing HE Thalassaemia  Prenatal Diagnosis and patient survival and
materials Screening System to TOP quality of Life
 Develop patient facilitate Result tracing  Access of Oral
Education module and client monitoring Chelating agents for
better compliance
 Projection : Impact Of Introducing School Based
Screening On New Thalassaemia Birth
No change New policy
250
T
E
h
s
a 200
t
l
i
a
m
s
a o
s 150
t f
a
e
e
d n
m
e
i 100
n w 95% reduction
a
u
m
achieved in 2035
b
b 50
i
e
t
r
h
0
2015 2017 2019 2021 2023 2025 2027 2029 2031 2033 2035 2037

Assumption:
1. Without young people screening: reduction of 1 case/ year
2. Introduction of compulsory 16 yrs old screening in 2015: Reduction of 1 case /year/ birth
cohort once these population started family at age of 18 and above.
Thalassaemia score card after introduction of
Prevention and Control Program
 Thalassaemia Score Card
Source: My Thal (2017)
Hematology Lab, HKL (2016)
700 Cases in MyThal versus Hematology Laboratory Returns 2008 - 2015
Difference between New
600

Number of cases/ results

500

400

300

200

100

0
2008 2009 2010 2011 2012 2013 2014 2015
New Thalassaemia Birth ( My Thal) 293 247 232 188 180 149 139 64
β-thalassaemia major ( Lab) 87 95 101 99 114 31
β-thalassaemia intermedia (Lab) 64 22 47 89 47 71
Alpha Thalassaemia (Lab) 486 608 396 531 590 581
Hb E β-thalassaemia (Lab) 363 282 Year 349 627 427 496
 Patient survival in 1999 and 2016
1600 1999
1400

1200
No of patients

1000

800

600

400

200

0
5 10 15 20 25 30 35 40 45 50 55 60 65 Above
65
Age Group Source: My Thal ,2017
 What do Population Screening Tell Us

Number of people screened between

2008 - 2016
900000
789421
800000
700000 654946 641234
600000
500000 436225
400000
300000 267236
209356 199174
200000 141758 137423
100000
0
2008 2009 2010 20111 2012 2013 2014 2015 2016
 Conclusion

Thalassemia is a - Universal access to Rx

Optimal care - Compliance to Rx
disease
- Comprehensive Registry

Thalassemia is Net reduction

preventable Thalassaemia patient
Reduction of new
cases

Optimal Prolonged quality life

patient of Thalassaemia
Effective patient
preventive
management
measures

Continous, Targeted cohort

structured, Undirectional carrier screening and
and targetted preventive measures genetic counseling
health
awareness