Presentation on Thalassemia s Management and Prevention By: Hemant Bellani, On behalf of Thalassemics India Date : 08th Dec 2011
Disclaimer Please be aware, that the sole purpose of this presentation is to spread awareness on prevention and management of Thalassemia. Please contact a medical practitioner or doctor, before initiating any medical action upon reading this presentation. Thalassemics India will not be responsible for any action initiated with prior advice from a medical practitioner or doctor.
Agenda What is Thalassemia ? Cause, Myth and Reason How a Thalassemia child lives ? Way to Stop this What you can do for us ?
What is Thalassemia ? Thalassemia Major is defined as an inherited blood disorder in which the affected children are not able to maintain Hemoglobin (Hb) in the normal range. Blood disorder in which the bone marrow cannot form sufficient red cells and red cell survival is also reduced . The disease is particularly prevalent among Mediterranean peoples, and this geographical association was responsible for its naming: Thalassa is Greek for the sea, Haema is Greek for blood. It is more prominent in certain parts of the world like the Mediterranean Countries , Asia ,the Middle East etc . In India , it is estimated ,3.4% (Range upto 17%) of our population is carrier/minor . Every year 7000-10,000 children with Thalassemia Major are born in India . It is more prevalent in communities like Sindhis,Punjabis, Gujaratis ,Marwardis ,Bengalis ,Saraswats ,Gaurs etc.
What is Thalassemia Major? Thalassemia Major is serious disorder, People with the Thalassemia major have insufficient hemoglobin, the critical oxygen carrying component of blood Patients with Thalassemia major need blood transfusions every 2-4 weeks to maintain hemoglobin levels With regular transfusion and removal of iron (chelation therapy), Thalassemics can live and grow into adulthood. During Blood transfusion they are exposed to infections like Hepatitis C, hepatitis B and HIV. Due to regular transfusion the Iron overload in the body increase if not cheated it damages to the liver, heart, pancreas and other glands. Untreated Thalassemia Major eventually leads to death. The only curative treatment is bone marrow transplant. Thalassemia Major (& intermedia) needs medical attention. Thalassemia Minor is not a disease
Inheritance of Thalassemia When both parents are Thalassemia Minors or Carriers there is a 25% chance of the birth of a Thalassemia Major child , 25%chance of a normal child and 50% chance of a Thalassemia Minor child .
What are the possible types of Thalassemia ? Each person can be either of the following three : 1 People who live a normal life and do not carry the gene for Normal Thalassemia Majority of us belong to this group People who live normal lives but carry the gene for Thalassemia There is also an 2 Thalassemia minor is not a diseaseintermediate condition Over 6 crore1 Indians are Thalassemia minors and are living normal called “Beta Thalassemia Thalassemia lives Minor Intermedia” – a condition where Many notable people are known to be Thalassemia minors children maintain Hb – Amitabh Bachchan, Amisha Patel, Pete Sampras, Zinedine levels of 6 - 9 gm Zidane (Wikipedia, Times of India) 3 Thalassemia People who have two abnormal genes are called Major. Major
Cause, Myth and Reason There are three type of human being : Normal ,Minor and Major carrier Thalassemia. Thalassemia is of two types : Minor and Major Thalassemia minors are people who carry one defective gene of Thalassemia .They are normal people except that during stress situations in life (like some serious surgery /pregnancy etc ) they may have low hemoglobin. Thalassemia Major is a blood disorder which causes severe anemia and related complications .Thalassemia major patients have two defective genes of Thalassemia ,acquired one from each Thalassemia minor parent .To survive the affected children require repeated blood transfusions (every two-four weeks )for their whole life.
How a Thalassemia child lives ? Blood Infections : Though the donor blood is tested for various transfusion transmitted still the patient are prone to infections like HIV,HBV,VDRL and Malaria. Thalassemia patients are always at a risk of getting these infections, for the test may not always pickup the infection as it might be at an early stage (window period) in the donor . Iron Overload : As a result of repeated blood transfusions,Thalassemia patients develop Iron Overload. Unfortunately the human body has no natural mechanism of excreting the excess iron from the body . This excess iron gets deposited in and around the various vital organs of the body and ultimately causes organ failure . It is very important to remove the excess iron from the patients body .This can be achieved by medicines only . This process of excess iron removal is called Iron Chelation . There are different medicines called Chelators available in the market to remove this excess iron from the body . Treatment with Desferal is very expensive and traumatic .This makes compliance difficult . Each vial of Desferal costs around Rs.165/- .Each person needs 2 vials every day In addition to this the infusion pump costs Rs.15,000/- . Monthly costs goes upto Rs.10,000 Per child. Other Diseases : Jaundice, Osteoporosis, Heart problems.
Way to Stop this There are 8000-10,000 new Thalassemic Majors born in our country every year . We need to take some important steps to control the birth of more Thalassemics .The only way this can be done is through : Public Awareness Carrier Detection Antenatal Diagnosis We need to take lessons from countries like Greece , Cyprus and Sardinia where the prevalence of Thalassemia minors is upto 17% and yet they have controlled the birth of Thalassemics Majors . Public awareness about this disorder and related management and complications is very vital. The carrier rate in our country is 3-17 %. The media needs to be proactive in spreading this message and the government needs to make testing for Thalassemia carrier status mandatory at the pregnancy stage
What you can do for us ? Awareness – Spread as much awareness as you can for Thalassemia prevention Blood Donation Camps – Organize/Participate in Voluntary Blood Donation Camps, as it’s a perennial requirement for all thalassemics. Supporting Under Privileged Thal children to enable them receive requisite medical facilities and management Join hands with volunteers in activities of society.