1. Prevalence of Thalassemia Major and Hepatitis C March 2013 In collaboration with Children’s Liver Foundation (Sukhbir Kaur) THINK Foundation (Vinay Shetty) Thalassemia and Sickle society, Hyderabad (Suman Singh ) DMC Hospital, Ludhiana (Ajit Sood) SGPGI, Lucknow (Shubha Phadke, Anjurani) Thalassemia Welfare Association, Chennai (Revathi Raj)

2. Thalassemia Major and Hepatitis C Average carrier rate of thalassemia in India is 3%. Approx 100,000 children with β thalassemia major in India Estimated prevalence of HCV in general population-0.08 - 4 % Thalassemia major patients is a high risk group on account of being multi-transfused Mandatory testing of HCV was only implemented in 2001

3. Thalassemia Major and Hepatitis C AIM Prevalence of Hepatitis C in multi blood transfused patients of Thalassemia Major DATA COLLECTED FROM THINK and Children’s Liver Foundation, Mumbai Thalassemia and Sickle Society, Hyderabad DMC Hospital, Ludhiana SGPGI, Lucknow Thalassemia Welfare Association, Chennai

4. Anti HCV Positive (%)in current survey CENTERS THALASSEMIA MAJOR PTSANTI HCV POSITIVE (%) MUMBAI (12 DAY CARE)953118 (12.4) CHENNAI20631 (15) HYDERABAD15008 (0.5) LUCKNOW30828/242 (11.6) LUDHIANA19258 (30.2) TOTAL3159243 (7.7)

5. PUBLISHED DATA AuthorYear of PublicationGeographic Location % anti HCV positive Bhattacharya DK1991Calcutta14.3 Amrapurkar D1992Mumbai17.5 Williams TN1992Delhi11.1 Aggarwal MB1993Mumbai16.7 Chopra K1994Delhi62 Choudhry UP1998Lucknow30 Mohammed I2002Delhi30 Marwaha RK2003Chandigarh54.4 Mishra D2004Delhi27 All data prior to mandatory HCV screening of blood, which started in June 2001

6. Pre and post mandatory testing Prior to 2001Now West India17.5 (Mumbai) 12.4 (Mumbai) North India35.75 (Chandigarh, Delhi, Lucknow) 21 (Ludhiana/Lucknow) South IndiaNA7.5 (Chennai, Hyderabad) East India14.3 (Calcutta) NA Overall22.513.6

7. Sex distribution Centers (Anti HCV positive patients analysed) MALE %FEMALE % MUMBAI (46)60.939.1 LUCKNOW (22)63.636.4 LUDHIANA (40)7030 CHENNAI (30)46.753.3 HYDERABAD (8)7525 MEAN63.136.9

8. Age Age in years (Number of patients analysed) Mumbai (46) Chennai (30) Hyderabad (8) Lucknow (22) Ludhiana (58) Mean (Range)21 (19-35)15 (5-35)16 (9-21)19 (6-32)12 (3-37) Median1912162010 Overall mean (range) 16.6 (3-37)

9. Age (in months) at FIRST TRANSFUSION Age (In months) of first transfusion MumbaiChennaiHyderabadLucknowLudhiana Mean (Range)9 (2-60)11 (6-36)9.7 (3-16)18 (4-48)NA Median6612

10. Religion CENTERS (No. of patients analysed) MUMBAI (46) CHENNAI (30) HYDERABAD (8) LUCKNOW (22) LUDHIANA (40) OVER ALL% Hindu (%)40 (89)28 (93.4)4 (50)21 (95.5)22 (55)78.8 Muslim (%)3 (6.5)1(3.3)4 (50)1 (4.5)6.2 Sikh(%)2 (4.5)18 (45)13.6 Christian (%) 1 (2.2)1(3.3)1.4

11. CO-INFECTION MUMBAICHENNAIHYDERABADLUCKNOWLUDHIANA HBV10010 HIV20000

12. MODE OF DETECTION MUMBAICHENNAIHYDERABADLUCKNOWLUDHIANA Routine Screening42 (91.2)27 (90)8 (100)22 (78.6)NA Abnormal LFT1 (2.2)3(10) Decompensated liver disease 1 (2.2) Pre op check1 (2.2) Not available1 (2.2)6 (11.4)

13. HCV GENOTYPE GENOTYPEMUMBAI (12) CHENNAI (15) HYDERABAD (1) LUCKNOW NA LUDHIANA (40) I63-6 III28--23 NA33--11 NON TYPEABLE 11---

14. TREATMENT OF HCV INFECTION Poor documentation of treatment data Few were tested for HCV RNA Fewer were started with the treatment From the data available significant drop in Hb, requiring increased transfusion rate and volume

15. TREATMENT OF HCV INFECTION MUMBAICHENNAIHYDERABADLUCKNOWLUDHIANA TOTAL TREATED 6/4610/271/83/2740/58 % TREATED133712.511.169 SVR2/6NA 26/40 TREATMENTPeg-Inf α 2a with Ribavirin Peg-Inf α 2a + Rib – 7/10, Peg-Inf α 2b + rib -3/10 Inf α 2bInterferon α 2a with or without Ribavirin Peg-Inf α 2b alone v/s with Ribavirin RIBAVARIN MINIMUM mg/day 200200 mg alternate day 00 RIBAVARIN MAXIMUM mg/day 600 800 *Sood A et al., Indian J Gastroenterol. 2010 Mar;29(2):62-5Indian J Gastroenterol.

16. Chelation Status S. Ferritin ng/mL MUMBAICHENNAIHYDERABADLUCKNOWLUDHIANA Minimum2839352898900NA Maximum75007167160008000NA Mean4078620154893017NA Median4147221655782560NA

17. CHELATORS DESFERALKELFERDESIROXASUNRA Mode of Administration SC with syringe driver over 8 – 10 hours Oral Cost / month in Rupees 15000300040009000 ManufacturerNovartisCipla Novartis AdvantageRemoves iron from other parts of body Removes iron from heart Water soluble, iron excretes through faeces DisadvantagePainful SC with syringe driver Joint pains, neutropenia Not known

18. In Mumbai Earlier patients receiving transfusions in public hospitals in the city eg. St. George, KEM, Wadia, Sion Hospital However most of these were unmonitored where patients went to the blood banks and receive transfusion THINK Foundation set up 12 day care centre in Mumbai to facilitate transfusions, close to the residence and under medical supervision, with regular yearly screening for infections, monitoring chelation, growth etc THINK Foundation - advocacy for NAT testing of blood which has been implemented in some Centres

19. Summarising… Thalassemia major and HCV infection Overall reduction in HCV prevalence compared to a decade back However, continuing infection despite mandatory testing Higher incidence in North India compared to the rest of the country Small proportion of patients receive treatment SVR is better achieved with combination therapy of Pegylated Interferon with Ribavarin v/s Pegylated Interferon alone Most patients are not adequately chelated; Effect of chelation on treatment response needs to be studied Need for overall focused care of thalassemic children including growth, iron overload related heart problems, infections and psychological problems