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Current situation in control strategies and Health
Systems in Asia

SINGAPORE
                                                             8 February2012



       < single image >
            g      g

       4.3cm x 5.5cm

                          Law Hai Yang
                          Deputy Director
                          Natiional Thalassaemia Reigistry
Demography              (2010)
                        (2010)


    Land area                    712.4 km2
    Temperature                  24.9 – 31.9 OC
    Total Population             5,076,700
                                   076,
       Resident Population       3,771,700
                                 3,771,
    Density                      7,126/km2
    Ethnic Groups
       Chinese                   74.1%
       Malay                     13.4%
       Indian                     9.2%
       Others                     3.3%

2
Demography                    (2010)
                              (2010)


    Annual Birth Rate
    A      l Bi th R t                       9.3/1 000
                                               3/1,000
                                               3/1
                                                 1,000
    Total Live-Births
          Live-                              37,967
    Infant Mortality Rate                    2.0 /1000 Live-Birth
                                                       Live-
    Life Expectancy at Birth                 81.8 Years
    Doctor per 1,000 Population              1.7
    Average Income*
         g                                   $4,800 (US$3,713)
                                                    (    ,   )
    Total Health Budget*                     $4.1 (US$3.17) billion
     Subsidies for medical care*             $2.2 (US$1.7) billion
                                                  (      )

     *$1 (USD) = $1.2926 (SGD) 16 Jan 2012


3
Health System – Public/Government (1)
   Affordable, Good Quality Healthcare

  Hospitals
     • 5 general hospital
     • 1 women’s and children’s hospital
     • 1 psychiatry hospital
  6 Specialty Centres
     • Cancer, cardiac, eye, skin, neuroscience
       and dental care
  Total 185 – 2064 beds
  18 polyclinics
        l li i
Health System – Public/Government (2)

   • Medisave Medishield ElderShield and
     Medisave, Medishield,
     Medifund schemes exist to help
     Singaporeans “co-pay” their medical
     expenses.
   • $20 million allocated to Medication
     Assistance Fund to help needy patients
     with high cost drugs.
            g          g
   • No special policy on Hb disorders.
Health System – Private


   16 Hospitals
       • 20 – 505 beds

   ~2000 Private Clinics

   Medisave, Medishield, ElderShield and
   Medifund not applicable
Diagnostic Services

Population Screening
    • National Thalassaemia Registry
    • Cascade screening – first degree relatives
    •GGenetic C
            i Counseling
                     li
Clinical laboratory
                  y
     • Full blood count, Hb electrophoresis
     • All major hospitals and private labs
              j     p          p
     • Outpatient and prenatal screening
Molecular Diagnostic Lab (Carrier & Prenatal testing)
    • KK Women’s and Children’s Hospital
    • National University Hospital
National Thalassaemia Registry (NTR)

  Established:1992年
  Location:KK Women’s and Children’s Hospital
  Aim :    • Register all thalassaemia carriers
           • Free counseling
           • Subsidized screening

  Referral :• Government and private hospitals, Clinics
            • Consent
            • Self referral

  Effective in identifying carriers
Screening


                Full blood count



     Hb electrophoresis   (HbH inclusion bodies)



               Mutation analysis
Prenatal screening
                                   FBC of couple


  MCV < 80 for one or both                              MCV > 80 for both
  MCH < 26 for one or both                              MCH > 26 for both

   Hb electrophoresis    for                            No further action
  HbH inclusion body     both


   HbE +ve              HbA2             HbH +ve        HbH -ve & both
                                                           MCV < 80
  HbE trait         -thal trait         -thal trait

                    If spouse is         If spouse is
                    -thal trait         -thal trait


                             Consider -thalassaemia minor
                                      -
                               Do DNA and family studies
Thalassaemia carrier rate


       2000 unrelated individuals:

        Thalassaemia                2.91 %
        Thalassaemia                0.93
                                     0 93 %
       HbE                           0.64 %

       Total                         4.49 %
Number of allele screened



                      No of alleles   No of mutation

    - thalassaemia       4971             26

    -th l
      thalassaemia
                i         3105             53
NUMBER OF NTR REGISTRANTS
2010

   • Number of Registrants 43,471
   • Number Screened 24,989
   • Number of Carrier 30,679

                          NTR       Population
                                      p

   Chinese                72.6%      74.1%
   Malay                  17.6%      13.4%
   Indian                  4.8%
                           4 8%       9.2%
                                      9 2%
   Others                  5.1%       3.3%
Type of thalassaemia
2010



  -thalassaemia carrier                  14129 (45.97%)
  -thalassaemia
   thalassaemia carrier                  11550 (37 58%)
                                                (37.58%)
  HbE                                     3652 (11.88%)
  HbH disease                             521   (1.70%)
  Concurrent -thalassaemia carrier
              thalassaemia           352   (1.15%)
                                                (1 15%)
  HbE with -thalassaemia                 101   (0.33%)
  -thalassaemia                           89   (0.29%)
  major
Number of -thalassaemia majors born

 30



 25



 20



 15



 10



  5



  0
Before Year   1971-1975   1976-1980   1981-1985   1986-1990   1991-1995   1996-2000   2001-2005   2006-2010
   1970
   19 0
-thalassaemia majors




     Chinese
      66.3%




                                      Malay
                                      23.6%



                             Indian
                    Others
                              4.5%
                     5.6%


                                              n = 89
-thalassaemia minors




   Chinese
                               Malay
    77.5%
    77 5%
                               14.3%




                            Indian
                              5.2%
                        Others
                         3.0%




                                     n = 11550
HbE Carriers



     Malay
     M l
     75.5%

                          Indian
                          I di
                           2.2%



                         Others
                         12.7%


               Chinese
                9.6%

                              n = 36 2
                                  3652
HbE -thalassaemia


       Malay
       74.3%




                                  Indian
                                   4.0%

                               Others
                                4.0%

                     Chinese
                      17.8%


                                        n =101
-thalassaemia minors



    Chinese
     82.8%




                                    Malay
                                    8.2%
                                 Indian
                                  4.1%
                        Others
                           h
                         5.0%


                                          n = 14129
 and -thalassaemia carriers




                                   Malay
  Chinese                          8.5%
   79.5%


                                    Indian
                                     6.5%
                                Others
                                 5.4%




                                           n = 352
HbH Disease



     Chinese
      86.2%




                          Malay
                           7.1%
                     Indian
               Others 0.8%
                6.0%

                                  n = 521
Treatment

Blood transfusion
   • Non-remunerated
   • US$21 27 (RCC) or US$52.22 (Washed Cells)
     US$21.27            US$52 22
   • Average 4 weekly transfusion
   • Target at Hb Level of 9 g/dL
 Iron chelation
   •Deferroxamine (Desferal)
    D f        i (D f l)
   •Deferiprone (L1)
   •Deferasirox (Exjade)
   •Combination therapy (Desferal & L1)
• Bone marrow transplant
Monitor Cardiac and other complications


Cardiac
   • T2* Technology available at National Heart Centre

Other complications
   • Singapore General Hospital
   • National University Hospital
Conclusion

 • The strategy of screening family members of index
   cases has been effective in identifying new carriers.

 • The incidence of the major form of the disease has
   decreased significantly with effective screening and
   preventive strategies in place.

 • The reduction in the number of affected
   homozygotes has allowed expensive optimal
   treatment to be available to nearly all those afflicted
   with the major form of the disease.
Thank you
      y
                          < single image >

                           4.3cm x 5.5cm




This presentation contains information which is confidential and/or legally privileged. No part of this presentation may be disseminated, distributed, copied, reproduced or relied upon without the expressed authorisation of SingHealth.

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Singapore - Current Situation in Control Strategies and Health Systems in Asia

  • 1. Current situation in control strategies and Health Systems in Asia SINGAPORE 8 February2012 < single image > g g 4.3cm x 5.5cm Law Hai Yang Deputy Director Natiional Thalassaemia Reigistry
  • 2. Demography (2010) (2010) Land area 712.4 km2 Temperature 24.9 – 31.9 OC Total Population 5,076,700 076, Resident Population 3,771,700 3,771, Density 7,126/km2 Ethnic Groups Chinese 74.1% Malay 13.4% Indian 9.2% Others 3.3% 2
  • 3. Demography (2010) (2010) Annual Birth Rate A l Bi th R t 9.3/1 000 3/1,000 3/1 1,000 Total Live-Births Live- 37,967 Infant Mortality Rate 2.0 /1000 Live-Birth Live- Life Expectancy at Birth 81.8 Years Doctor per 1,000 Population 1.7 Average Income* g $4,800 (US$3,713) ( , ) Total Health Budget* $4.1 (US$3.17) billion Subsidies for medical care* $2.2 (US$1.7) billion ( ) *$1 (USD) = $1.2926 (SGD) 16 Jan 2012 3
  • 4. Health System – Public/Government (1) Affordable, Good Quality Healthcare Hospitals • 5 general hospital • 1 women’s and children’s hospital • 1 psychiatry hospital 6 Specialty Centres • Cancer, cardiac, eye, skin, neuroscience and dental care Total 185 – 2064 beds 18 polyclinics l li i
  • 5. Health System – Public/Government (2) • Medisave Medishield ElderShield and Medisave, Medishield, Medifund schemes exist to help Singaporeans “co-pay” their medical expenses. • $20 million allocated to Medication Assistance Fund to help needy patients with high cost drugs. g g • No special policy on Hb disorders.
  • 6. Health System – Private 16 Hospitals • 20 – 505 beds ~2000 Private Clinics Medisave, Medishield, ElderShield and Medifund not applicable
  • 7. Diagnostic Services Population Screening • National Thalassaemia Registry • Cascade screening – first degree relatives •GGenetic C i Counseling li Clinical laboratory y • Full blood count, Hb electrophoresis • All major hospitals and private labs j p p • Outpatient and prenatal screening Molecular Diagnostic Lab (Carrier & Prenatal testing) • KK Women’s and Children’s Hospital • National University Hospital
  • 8. National Thalassaemia Registry (NTR) Established:1992年 Location:KK Women’s and Children’s Hospital Aim : • Register all thalassaemia carriers • Free counseling • Subsidized screening Referral :• Government and private hospitals, Clinics • Consent • Self referral Effective in identifying carriers
  • 9. Screening Full blood count Hb electrophoresis (HbH inclusion bodies) Mutation analysis
  • 10. Prenatal screening FBC of couple MCV < 80 for one or both MCV > 80 for both MCH < 26 for one or both MCH > 26 for both Hb electrophoresis for No further action HbH inclusion body both HbE +ve HbA2  HbH +ve HbH -ve & both MCV < 80 HbE trait -thal trait -thal trait If spouse is If spouse is -thal trait -thal trait Consider -thalassaemia minor - Do DNA and family studies
  • 11. Thalassaemia carrier rate 2000 unrelated individuals:  Thalassaemia 2.91 %  Thalassaemia 0.93 0 93 % HbE 0.64 % Total 4.49 %
  • 12. Number of allele screened No of alleles No of mutation - thalassaemia 4971 26 -th l thalassaemia i 3105 53
  • 13. NUMBER OF NTR REGISTRANTS 2010 • Number of Registrants 43,471 • Number Screened 24,989 • Number of Carrier 30,679 NTR Population p Chinese 72.6% 74.1% Malay 17.6% 13.4% Indian 4.8% 4 8% 9.2% 9 2% Others 5.1% 3.3%
  • 14. Type of thalassaemia 2010 -thalassaemia carrier 14129 (45.97%) -thalassaemia  thalassaemia carrier 11550 (37 58%) (37.58%) HbE 3652 (11.88%) HbH disease 521 (1.70%) Concurrent -thalassaemia carrier  thalassaemia 352 (1.15%) (1 15%) HbE with -thalassaemia 101 (0.33%) -thalassaemia 89 (0.29%) major
  • 15. Number of -thalassaemia majors born 30 25 20 15 10 5 0 Before Year 1971-1975 1976-1980 1981-1985 1986-1990 1991-1995 1996-2000 2001-2005 2006-2010 1970 19 0
  • 16. -thalassaemia majors Chinese 66.3% Malay 23.6% Indian Others 4.5% 5.6% n = 89
  • 17. -thalassaemia minors Chinese Malay 77.5% 77 5% 14.3% Indian 5.2% Others 3.0% n = 11550
  • 18. HbE Carriers Malay M l 75.5% Indian I di 2.2% Others 12.7% Chinese 9.6% n = 36 2 3652
  • 19. HbE -thalassaemia Malay 74.3% Indian 4.0% Others 4.0% Chinese 17.8% n =101
  • 20. -thalassaemia minors Chinese 82.8% Malay 8.2% Indian 4.1% Others h 5.0% n = 14129
  • 21.  and -thalassaemia carriers Malay Chinese 8.5% 79.5% Indian 6.5% Others 5.4% n = 352
  • 22. HbH Disease Chinese 86.2% Malay 7.1% Indian Others 0.8% 6.0% n = 521
  • 23. Treatment Blood transfusion • Non-remunerated • US$21 27 (RCC) or US$52.22 (Washed Cells) US$21.27 US$52 22 • Average 4 weekly transfusion • Target at Hb Level of 9 g/dL Iron chelation •Deferroxamine (Desferal) D f i (D f l) •Deferiprone (L1) •Deferasirox (Exjade) •Combination therapy (Desferal & L1) • Bone marrow transplant
  • 24. Monitor Cardiac and other complications Cardiac • T2* Technology available at National Heart Centre Other complications • Singapore General Hospital • National University Hospital
  • 25. Conclusion • The strategy of screening family members of index cases has been effective in identifying new carriers. • The incidence of the major form of the disease has decreased significantly with effective screening and preventive strategies in place. • The reduction in the number of affected homozygotes has allowed expensive optimal treatment to be available to nearly all those afflicted with the major form of the disease.
  • 26. Thank you y < single image > 4.3cm x 5.5cm This presentation contains information which is confidential and/or legally privileged. No part of this presentation may be disseminated, distributed, copied, reproduced or relied upon without the expressed authorisation of SingHealth.