Awareness among youth and their role in combating Thalassemia By Tripti Agarwal (U310054)

Introduction:
Most of our physical characteristics are inherited through the genes we take from our parents; for example, the shape of our nose, the color of our skin and eyes. We also inherit our hemoglobin type from our parents, through the genes. Hemoglobin is the substance in the blood which gives it its red color. It carries the oxygen we breathe to all our body parts; thus is vital for life. Thalassemia is an inherited blood disorder in which the body is unable to make adequate hemoglobin. Hemoglobin is present in the red cells and is made from proteins. These proteins consist of alpha() and beta() chains. Normally 2 alpha and 2 beta chains are essential to form hemoglobin. Imbalances in these chains results in reduced red cell survival. The amount of beta and alpha chains a person makes is controlled by the hemoglobin gene they inherit from their parents. Normally red cells survive for 120 days but in Thalassemia red cell survival is reduced. Thalassemia can be categorized into three classes depending on clinical expression: thalassemia major (TMA), a severe disorder leading to transfusion dependence; thalassemia intermedia (TI), relatively milder involving less transfusion dependence; and thalassemia minor (TMI), where individuals carry the heterozygous mutation but do not exhibit any of the symptoms and usually do not require blood transfusion.1 If both parents have TMI there exists a 25% probability in each pregnancy that their child will have TMA, a 50% probability that the child will have TMI and a 25% probability that a child will have neither mutation . The classification of TMA or TI also depends on the age of onset of symptoms, degree of anemia, age when first transfused, transfusion requirements per year, and growth or enlargement of the spleen. Survival of Thalassemia patients depends upon repeated blood transfusion and costly medicines.

Importance of Topic for Research:

Thalassemia is a hematological disorder that belongs to a heterogeneous group of single gene disorders occurring due to an absence or reduction of hemoglobin synthesis. Thalassemia is not uncommon among people of Asian descent, occurring at a higher rate among certain communities including Punjabis, Assamese, Gujaratis, Sindhis, Marwaris and Bengalis. It is estimated that globally there are 200 million people with thalassemias or similar hemoglobinopathies, primarily in the Mediterranean countries, Asia, parts of North and West Africa, and parts of Eastern Europe. India has nearly 30 million Thalassemia carriers and of the 100,000 children born with Thalassemia annually, 10,000 are born in India. 2 Though there are many variants of Thalassemia, beta Thalassemia is most commonly found in the Indian subcontinent. Thalassemia cannot be cured, only managed; bone marrow transplantation has been successful in the management of TMA and reduces (sometimes eliminates) dependency on transfusions. However, this procedure is expensive, risky, and difficult to perform, and success rates are discouraging in India. Even with the advantages of good medical care and a high standard of living, patients cannot expect to have the full life expectancy of an average adult, and premature deaths are not uncommon. In India the incidence of beta Thalassemia trait in general population is about 3%. There are a number of communities in India such as Sindhi, Lohana, Khoja, Bhanushali, Punjabi, Jain, Muslim and Bengali in whom the incidence of beta thalassemia trait ranges between 8% to 15%. The high incidence can be attributed to consanguinity and endogamy practiced in these communities. On the basis of earlier reports published since last 20 years, several tribal groups of India have been identified as high-risk groups for Thalassemia and other haemoglobinopathies. It causes high degree of morbidly and mortality among them. It is evident that if awareness about the disease is not created in these communities, the number of beta Thalassemia major children born will be much higher than the present estimate of about 8,000 to 10,000 per year. Every year 10,000 children with Thalassemia major are born in India, which constitutes 10% of the total number in the world, and one out of every 8 carriers of Thalassemia worldwide lives in India.

Research Question in broad sense:

Which are the communities mostly affected by Thalassemia and how can they be made aware about it? What all attempts are made in the area of Thalassemia Awareness and hence studies carried to analyze their impact?

Literature Review:
There are camps held in the high risk communities under awareness programs. In view of the hereditary nature of the disease, the families do not want to get themselves branded as thalassamics due to the fear of social stigma and the difficulties it would create in getting the girls married. Considering the Indian social and cultural pattern, awareness programs and genetic counseling in high risk communities is the only powerful and economic tool to control Thalassemia. In an aim to reduce the birth of children with -thalassemia and other haemoglobinopathies with immediate effect a study was undertaken to screen the thalassemia carriers from a population of pregnant women attending the antenatal clinic of Ramakrishna Mission Seva Pratisthan hospital over a period of 5 years (1995-1999). From a total of 1962 female pregnant patients 7.04 % thalassemia carriers were identified. The husbands of these carrier mothers (138 in number) were offered carrier detection and 68.84% responded from which 15.8% carriers (15 in number) were identified. From these high risk couples only 6 agreed for prenatal diagnosis. 19 other couples were also identified as couples at risk from 133 referred antenatal cases who came for investigation of anaemia In 1998, the governments of West Bengal, Maharashtra, Punjab, Gujarat and Karnataka in collaboration with the Indian Council of Medical Research (ICMR) launched thalassemia prevention campaigns. These states have a particularly high incidence of thalassemia, with official estimates of TMI ranging from 5% to 10% incidence in the general population (Indian Council of Medical Research Bulletin, 1993). An ICMR pilot study conducted during 1998 with schoolchildren revealed that 2.7 percent of 5682 tested in Bombay, 5.5 percent of 5408 tested in Delhi and 10.4 percent of 952 children tested in Calcutta had beta TMI. Based on these figures, the pilot study concluded that thalassemia was sufficiently prevalent to warrant a prevention program in the study areas. In West Bengal the program is called Joi Vigyan: Community Prevention of Thalassemia which literally means Victory to Science. This project aims to test 10,000 people, focusing on college students and pregnant women, within the next five to seven years.The project conducts camps in colleges and tests students free of charge. Along with this Delhi government is also making an attempt to spread awareness as awareness is the most important preventive measure for thalassemia. In a drive to create awareness about thalassemia and provide blood transfusion facilities to thalassemics in the capital, the Delhi government has opened a daycare centre for thalassemics at the Hindu Rao hospital. Dr Madhur Kudesia, medical superintendent at Hindu Rao said that the five-bed centre has specially been designed for making patients stay comfortable. Along with blood transfusion, the centre also provides supportive therapies for other haematological disorders and iron chelation therapy. Lamenting the lack of awareness about thalassemia, Dr Kudesia said, There is need for a sustained mass awareness campaign along with ante-natal testing and voluntary testing by all. Experts say that India should launch a national programme to curb the disease. According to Dr Gautam Bose, vice president, Thalassemics India the fact that parents of almost all thalassemic children dont know about this genetic disorder proves that theres ample need to have a mass awareness campaign. He suggests that India should emulate the Cyprus model, where timely

intervention of the government, including sustained mass awareness campaigns has helped to contain the widely prevalent disease. In the past 20 years, no new case has been reported in Cyprus because thalassemia minor carriers were identified through a simple blood screening and were not allowed to marry another carrier. The Delhi government has woken up to this need and has started services like screening, free checkups and medication in a few government hospitals. Awareness will help people know that they need to get a test done in the right age group," Abbi said at a function organised by Thalassemia Control Project and India Population Project-VIII to mark an awareness week from May 2 to May 8 at a Health Centre in Dilshad Garden, east Delhi. The Municipal Corporation of Delhi (MCD) is planning to collaborate with the students of Delhi University (DU) to spread awareness on Thalassemia. The MCD will collaborate with Delhi University students to spread awareness about the disease in their locality through street plays, and other ways. Of Gujarat's total population, 17% are carriers of thalassemia genes, said Dr Samir Melinkeri, consulting hematologist and transplant physician at Sahyadri Hospital and Deenanath Mangeshkar Hospital, Pune. She added that 2000 children in Gujarat are born with thalassemia major. As part of its efforts to spread awareness about the disease and to let more doctors know about its treatment, Stemcyte India offered a fellowship programme, in Taiwan, to two transplant physicians-Dr Uday Deotare and Dr Melinkeri. Deotare is the director of bone marrow transplant unit at Sterling Hospital, Ahmedabad. Another awareness program was organized by Rotary Club of Porbandar and Shri Ram Sea Swimming Club of Porbandar jointly organized Rotary Coastal Marathon-2011 for the Thalassemia Awareness on 12th June 2011 for the first time in the history of Porbandar. The aim behind this was to save lot of financial loss and emotional stress to the family. Around more than 1000 participants joined this event from entire state. The event established good print and electronic media coverage throughout Gujarat. As compared to Porbandar, Mumbai took an initiative for awareness on this cause a long back. A programme for education, screening and genetic counselling of high school students was undertaken between 1984 and 1988. In total, 5682 school children (age 1118 years) from 75 schools in Mumbai city were screened after obtaining informed onsent from their parents. Of the 5682 children, 153 (prevalence 2.7%) were found to be b-thalassaemia heterozygotes. All of them were given report cards as well as a booklet giving information on thalassaemia. After a gap of 20 years, an attempt was made to follow-up these 71 families to evaluate the impact of screening this group. The students screened were now in the age group of 3038 years. Only 12 of the 47 individuals contacted (26%) recollected that they were b-thalassaemia carriers. The others vaguely remembered that some blood test was carried out in school but had lost their reports. None of the 41 individuals who were now married had revealed their carrier status or had their partners tested before marriage. . A thalassaemia major child was born in one family in spite of screening. There are attempts made to make people aware through social networking sites. Below are the links for the facebook page established for this cause.

http://www.facebook.com/pages/The-Sickle-Cell-Awareness-FoundationTSCAF/161579440528554?sk=wall http://www.facebook.com/pages/Thalassemia-Awareness/142428282476134

NO More Thalassemia- with this slogan, the students of the Faculty of Business Studies, University of Dhaka organised an awareness programme on Thalassemia disease on 8th May, the International Thalassemia Day. The programme was organised by TSI (Thalalassemia Support Initiative), a voice for Thalassemia awareness, support and care, members of which are Md. Raquibul Islam Russeau, Tanzina Alam Monty, Tasnia Safdar and Sonia Rezina. They have been conducting multimedia presentation about the disease in all the classes of Business Studies Faculty for the last one month to aware the students about Thalassemia disease, the reason, consequences and ways of prevention. TSI's vision is to stop the increasing number of Thalassemia patients in Bangladesh. With the purpose in mind, they arranged an Electrophoresis Test (a simple blood test) among a few students of DU to identify whether they are Thalassemia carriers or not. The test was conducted by DMCH (Dhaka Medical College Hospital) medical team and sponsored by Novartis (Bangladesh) Ltd and Dr. Mahmood Osman Imam, Professor, Department of Finance. Later on, it was found that 4 among 52 students are Thalassemia carriers! The awareness programme was organised at the Faculty of Business Studies, University of Dhaka. The programme started with a seminar on "Mobilizing Youth to Prevent Thalassemia". The programme also included a Blood Donation part for the Thalassemia patients. Blood transfusion is the lifeline for patients suffering from severe Thalassemia. In 2006 on World Blood Donor Day, Dr R N Makroo, Secretary General of Indian Society of Blood Transfusion and Immunohaematology (ISBTI) said that India has a requirement for 8.5 million units of blood every year whereas only 6 million units are collected in the country and for this there were several blood donation camps set up in association with Baxter India. Over 80 million units of blood are donated every year, but only 38 per cent are collected in developing countries, where 82 per cent of the global population lives. There are certain gaps in the awareness programs carried in India as compared to similar models in other countries. Thus there is need to fill these gaps by alternatives. Screening for bthalassaemia and other haemoglobinopathies is a prerequisite for a community control programme. In India, both retrospective and prospective screening had been carried out in different target groups.35 However, the long-term impact of screening has never been evaluated. This has immense relevance as a lot of time, effort and money goes into such programmes. A similar programme on screening over 25,000 high school students, in Montreal, Canada showed that virtually all the carriers identified in the high school programme remembered their status, had their partners tested and opted for prenatal diagnosis if required. In another study from the Marseille region in France, evaluation of a screening programme for secondary school children was carried out by assessing the partners uptake for testing by sending a letter along with an anonymous questionnaire to all the carriers. In total, 86% of them were aware that they had to test their partners. Six carrier couples were identified and four of them requested prenatal diagnosis. Although these reports from Canada and France are success stories, our experience in India has shown that screening high school children with one-time counselling of their families is insufficient. A periodic effort would be necessary to increase awareness and make such programmes more successful.

It has been seen that generating awareness among school children proves effective in long run as compared to other communities. In an article by C R Scriver, M Bardanis, L Cartier, C L Clow, G A Lancaster, and J T Ostrowsky, they evaluated a program for thalassemia-disease prevention, comprising education, population screening for heterozygotes, and reproductive counseling; the evaluation includes cost analysis. A preprogram survey in 1978 of 3,247 citizens in the high-risk communities (85% were high-school students) showed that 88% favored a program but that only 31% considered fetal diagnosis as an acceptable option. Screening in high school or before marriage was preferred by 56%. In a 25-month period (December 1979-December 1982), they screened 6,748 persons, including 5,117 senior high-school students, using MCV/HbA2 indices. The participation rate was 80% in the high-school group. Most carriers told parents (95%) and friends (67%) the test result. Sickle cell disease (SCD) affects many students worldwide. Central nervous system complications are widespread among students with SCD and include stroke, silent cerebral infarction, and cognitive impairment. The effects of these complications may lead to academic failure, limited career options, and for some, total disability. Despite studies describing the significant academic and cognitive impact of sickle cell disease, reports describing interventions are limited. There is a lack of awareness among educators of the academic risks associated with sickle cell disease and a lack of appropriate resource allocation. The school nurse, as community health advocate, should be called upon to bridge the gap among healthcare providers, parents, students, and educators. She should serve the role of the school nurse as an advocate, liaison, and educator. Living with thalassemia can be a challenge but following the recommended treatment plan, getting medical care regularly and adopting measures to stay healthy has definitely shown to increase life expectancy. Genetic counseling plays a very important role in disease management. Genetic counseling is a communication process to provide information and support to families that are in some way impacted by inherited diseases such as thalassemia.A genetic counselor usually conducts a pedigree analysis(detailed family medical history)which can help family members who maybe at risk of having thalassemia disease or trait to be tested and ensure their status. Partner testing and prenatal testing options would be considered for those families who have a famility history of the disease and who are planning to have a child. This could certainly help in early recognition and prevention of this disease. Research in genetics and genomics has opened up several molecular genetic tests, which have entered our healthcare system. However, in a country like India, more than 75% of the people live in rural areas and do not get the benefit of this advanced testing. In fact their needs are different. In high-risk communities for -thalassemia or in tribal pockets with a high prevalence of sickle hemoglobin, the main requirements are, creating an awareness of the disorders and genetic counseling, which can help to prevent the transmission of these disorders to the next generation. Efforts should be targeted toward identifying the affected families and their needs, and then offering them proper counseling. These are the preliminary goals of integrating genetics into the public health in India. Once they are achieved one can think of genomic medicine and other advanced studies to detect genetic disorders, novel genetic association, and the like, at the population level.

Research Question:
What is the level of awareness among sample studied about Thalassemia? In what way can they intervene for the benefit of society?

Research methodology:
This research is basically concentrated towards qualitative research. Qualitative researchers aim to gather an in-depth understanding of human behavior and the reasons that govern such behavior. The qualitative method investigates the why and how of decision making, not just what, where, when. Hence, smaller but focused samples are more often needed, rather than large samples. Thus keeping these points I will look forward to understand the level of awareness among the selected sample size. Also there will be an attempt made to see how students can generate awareness on the topic and prospective impact on the target population. The design frame used to carry research is Evaluation Research. This is selected because evaluation is the comparison of actual impacts against strategic plans. It looks at original objectives, at what was accomplished and how it was accomplished. Evaluation is inherently a theoretically informed approach (whether explicitly or not), and consequently a definition of evaluation would have be tailored to the theory, approach, needs, purpose and methodology of the evaluation itself. Having said this, evaluation has been defined as:

A systematic, rigorous, and meticulous application of scientific methods to assess the design, implementation, improvement or outcomes of a program. It is a resource-intensive process, frequently requiring resources, such as, evaluator expertise, labour, time and a sizeable budget A study designed to assist some audience to assess an objects merit and worth'

As defined above, there are several attempts made to generate Awareness on Thalassemia. These attempts are made among various communities, in different age groups and sexes. This all incorporates extensive resources and expertise knowledge on the subject in order to aware people about the precautions and consequences of thalassemia on individual and society as a whole. The sample to be studied for this research will be containing:

Students of XIMB Married couples School students College students

This sample is selected as there is need to generate awareness in these communities as:

Understanding the consequences they will be hopefully taking preventive measures Take part in events to generate awareness among other communities like tribals, poor etc. Also they can serve to change the traditional mindset of people regarding thalassemia and remove social stigma.

There will be collection of data from these individuals by adopting certain techniques like:

Questionnaire filling In depth interview Focus group discussion

Qualitative research often categorizes data into patterns as the primary basis for organizing and reporting results. My research will rely on the following methods for gathering information: Structured Interview, Semi-structured Interview, Unstructured Interview, and Analysis of documents and materials. These methods of data collection are selected as they will cover the following aspects:

Views of individuals on thalassemia and their understanding on it Level of awareness among them, Their willingness towards the subject knowledge. Attempts and their potential to contribute to the society

There will be a questionnaire designed which will be covering the research objectives and scope. Some questions will be like; 1. 1. Have you donated blood? Yes /No 2. 2. Do you know about the Thalassemia? Yes/ No 3. 3. Do you know how this disease spreads? Yes/ No 4. 4. Do you know about treatment? _________________________________________ 5. 5. Do you think that Thalassemia test is necessary before marriage? Yes No 6. 6. Do you think you have to join this awareness program of Thalassemia? Yes/ No 7. 7. Have you heard about any such campaign? Yes/ No 8. 8. Do you know any person with Thalassemia? Yes/ No 9. 9. Have they taken any preventive measure? Yes/ No 10. 10.How do you think that you can contribute to this _______________________________________

cause?

There will be an extensive questionnaire be built on these grounds and thereby analysis made. After collecting data from various sources there will be analysis carried with help of certain techniques like: Top of the mind/awareness SWOT Analysis

Timeline:
Overwhelmed by the amount of information while doing research for a report, one way to organize research materials and outline paper at the same time is to write a timeline report. Choose a topic for project: This involves brainstorming and selecting a topic on which some studies are conducted and approaches my thoughts. This took me 5 days to zero down on Thalassemia after studying several topics Conduct research: It requires to read multiple books, conduct interviews, or travel to observe people. Decide on research method(s) and then determine how much time will be needed to complete the research. So it is expected to complete this work in a duration of 15 days due to time constraint Analyze the results of research: Decide how much time is needed to organize the research you have conducted and analyze the results. This will take approximately 5 days Prepare your research for presentation: Determine how much time will be needed to write up or otherwise prepare to present research and analysis. This will take around next 3 days Receive feedback from project adviser, professor or anyone else who will critique do work: this depends on the professor but is expected to be done within 5 days Proof read and edit work: This will be done in 2 days Cushion time: Keeping some unavoidable circumstances I take this as 2 days.
0-5 days Choose the topic Conduct research Analyse the results Research presentation/write-up Feedback Proof read and edit work Cushion time 10-15 days 15-20 days 20-25 days 25-30 Days 30-35 days

5-10 days

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