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Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
Dr. rashid merchant presentation new era for thalassemia
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Dr. rashid merchant presentation new era for thalassemia

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Dr. rashid merchant presentation new era for thalassemia

Dr. rashid merchant presentation new era for thalassemia

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  • 1. Thalassemia “Bad Blood By Mutations” Dr Rashid Merchant World Hepatitis Day
  • 2. 5
  • 3. 6
  • 4. 60 Million 112 Million 72 Million • MAHARASHTRA • GUJARAT • MADHYA PRADESH
  • 5. Sindhis Bhanushalis Bhatias Khojas Bhoras Marathas Luwanas Kucchis Communities:
  • 6. screen for thalassemia New births of beta-thalassemia major can be prevented do it the right way The screening test needs to done only once in a person’s life but done the right way urgent need to identify all carriers screen for thalassemia
  • 7. Lab Investigations - Carriers Hb < 10-11gms RBC > 5 million MCV < 76 MCH < 26 Mentzer’s Index <13 (MCV/RBC) RDW Normal PBS - micro, hypo Suspect Thal. minor/IDA Hb Electrophoresis (HPLC) HbA2 > 3.5% -------Thal. Minor
  • 8. You could be a Thalassaemia Minor There is nothing wrong in being a Thalassaemia Minor
  • 9. Picture of thal minor
  • 10. There is everything wrong in not knowing that you are a Thalassaemia Minor
  • 11. 16 Demography Worldwide: 5 million people – symptomatic clinically 240 million carriers of β -thalassemia gene India: 30 million carriers of β-thalassemia thalassemia gene 10,000 cases added every year Carrier rate: 4% carrier rate in India One thalassemic is born every hour
  • 12. Let’s look at Cyprus……
  • 13. Can we replicate Cyprus model Cyprus has highest Thal. prevalence No child with Thal. maj born in last 10 yrs Prenatal: awareness/ counselling/ testing Couple getting married needs church license for marriage, which is given after testing for Thalassemia
  • 14. Transfusion Transmitted Infections HIV Screening antibodies /Confirmed PCR HBV HCV CMV Malaria Syphilis Yersinia Bacteria
  • 15. Heart is the lethal target organ in Thalassemia Cardiac complications are >75% cause of mortality Adequate iron chelation is mandatory to prevent cardiac disease Quantitative MRI T2* best evaluates cardiac iron overload
  • 16. No Bone Deserves A Break Cardiac : Major cause of mortality Bone : Major cause of morbidity (Osteopathy) (pain/fractures/deformities) Incidence 80-90% (>15 yrs)
  • 17. 2 - D ECHO
  • 18. OUR EXPERIENCE IN CARDIAC/LIVER IRON QUANTIFICATION MRI T2* Cardiac/Liver Iron in Thalassemia T2* - 1.5 Tesla, SEIMENS machine,Thal. Tools software CENTRE : JHANKARIA CLINIC, PIRAMAL DIAGNOSTICS , MUMBAI Rashid Merchant Aditi Joshi Pradeep Krishnan Bhavin Jhankaria
  • 19. MRI IS ABLE TO DETECT SINGLE ORGAN IRON IRON LOAD TO TAILOR CHELATION TREATMENT ON SINGLE ORGAN DAMAGE HYPOPHISYS HEART LIVER PANCREAS Normal Iron overloading NormalIron overloading Normal Iron overloading NormalIron overloading
  • 20. Hepatic Evaluation METAVIR STAGING FIBROSIS • F0 – no fibrosis • F1 – portal fibrosis without septa • F2 – portal fibrosis with few septa • F3 – numerous septa without cirrhosis • F4 – cirrhosis ACTIVITY • A0 – no activity • A1 – mild activity • A2 – moderate activity • A3 – severe activity
  • 21. TRANSIENT ELASTOGRAPHY (Our Experience) Ultrasound based technique Measures the liver stiffness Measures the propagation velocity of SHEAR WAVES Harder tissue Faster propagation Higher velocity
  • 22. FIBROS CAN Fibroscan
  • 23. Discussion Liver biopsy is the gold standard for fibrosis staging Not feasible to monitor progression & treatment response Liver elastography is a noninvasive alternative It is shown to correlate with liver enzymes Variable correlation with S. Ferritin & MRI T2* liver
  • 24. No Bone Deserves A Break Cardiac : Major cause of mortality Bone : Major cause of morbidity (Osteopathy) (pain/fractures/deformities) Incidence 80-90% (>15 yrs)
  • 25. Osteoporosis Treatment Vitamin D 1000 IU/day Calcium 1000-1500 mg/day Bisphosphonates:  osteoclastic activity Hormone replacement treatment rPTH (Forteo):  osteoblastic activity Calcitonin nasal spray (inhibits osteoclasts)
  • 26. Treatment Calcitonin nasal spray (inhibits osteoclasts) Duration = 6 weeks 200 IU / day Especially vertebral fractures, severe bone pains
  • 27. Chelator MetalChelator Toxic ExcretionMetal What is chelation therapy?
  • 28. Overview of iron chelators Property Deferoxamine (DFO) Deferiprone (DFP) Deferasirox Usual dose 25–60 mg/kg/day 75 mg/kg/day 20–30 mg/kg/day Route s.c., i.v. 8–12 h, 5 days/week p.o. 3 times daily p.o. once daily Half-life 20–30 min 3–4 h 8–16 h Excretion Urinary, faecal Urinary Faecal Adverse effects Local reactions, ophthalmological, auditory, growth retardation, allergic GI disturbances, agranulocytosis/ neutropenia, arthralgia, elevated liver enzymes GI disturbances, rash, mild non-progressive creatinine increase, ophthalmological, auditory, elevated liver enzymes Status Licensed Not licensed in USA or Canada Licensed Approved indications Treatment of chronic iron overload due to transfusion-dependent anaemias Thalassaemia major Treatment of chronic iron overload due to frequent blood transfusions GI = gastrointestinal; i.v. = intravenous; p.o. = per orum; s.c. = subcutaneous.
  • 29. Desferal is effective Compliance is the issue
  • 30. By 15 years By 50 years 250 units PCV 2000 units PCV 4000 inj of DFO 15,000 inj of DFO Rs 16.5 lacs expenses (DFO & L1) Rs 90 lacs expenses (DFO & L1) 40,000 hours needle stuck in 1.5 lacs hours needle stuck in
  • 31. DEFERIPRONE (L1) India - first country to launch in 1994. Available in 40 countries. Used in more than 7500 patients. More powerful cardiac iron chelator than DFO DFO + L1 : best combination for chelation
  • 32. ICL 670
  • 33. How to take DFX ? STEP 1:  DROP the tablet(s) into a glass of orange juice, apple juice, or water (100 ml or 200 ml)
  • 34. How to take DFX ? STEP 2:  STIR until the tablet(s) are completely dissolved  The liquid in the glass will look cloudy  The cloudy liquid means the medicine is mixed in
  • 35. WHAT & HOW WE MONITOR?? TYPE OF TEST ORGAN FREQUENCY ECG/ 2D ECHO HEART ANNUALY MRI T2* LIVER/ HEART ANNUALY SONOGRAPHY ABDOMEN/PELVIS ANNUALY XRAY CHEST ANNUALY DEXA(BMD) BONE ANNUALY (Ca,P,Alk-Poshphatase), Blood Sugar BLOOD ANNUALY VIRAL MARKERS HBV/HCV/HIV ANNUALY RBC ANTIBODIES DCT/ICT ANNUALY HORMONAL ANALYSIS THYROID (T3+T4+TSH), IGF1 FSH/LH& ER/TESTOSTERONE ANNUALY BUN/Sr. Cr, URINE,LFT BLOOD 1-3 MONTHLY SERUM FERRITIN BLOOD 3 MONTHLY
  • 36. Dr. Rashid Merchant Pediatrician • Former Dean & Prof. Pediatrics B.J. Wadia Child Hospital Mumbai • Presently Consultant Pediatrician Nanavati Hospital Mumbai
  • 37. Hydroxyurea(Hu) Therapy Hu is hydroxamic acid compound Hu inhibits ribonucleotide reductase Hu stimulates Y chain synthesis(B gene) Increased Fetal Hb Used in Rx - Sickle / Hb-pathies Cytodrox(Cipla) 500mg/caps @ 10-20mg/kg/day Effective minimal S/E. Safe for long term. S/E:Anemia, Thrombocytopenia, Neutropenia.
  • 38. Dr. Rashid Merchant Stem cell is a single cell that can give rise to progeny (progenitor cells) that can differentiate into specialized cells of various tissues. In other words, it is origin of life.
  • 39. Persevere through the difficult patches and better times are sure to come some time.

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