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    Thalassemic update Thalassemic update Document Transcript

    • Thalassaemia Issue 34 • July 20094th International Conference on Thalassemia 31st October - 1st November, 2009 New Delhi, India “It Helps To Know” Dr. Kanwar Sain Mayor of Delhi felicitating Thalassemics on World Thalassemia Day Scintillating performance by Thalassemia children In This Issue................ Thalassemia Unit at Apollo Hospital Thalassemia Unit at DDU Hospital Special Darshan to Akshardham Temple New Members aemia Our Worthy Donors Organised by: In collaboration with: News Across India aemia Towards Awareness Article on Growth & PubertyThalassemics India Sir Ganga Ram Hospital Thalassaemia International Federation Blood Donation Camps Readers’ Queries www.thalassemicsindia.org
    • Special Thanks Air Force Wives Welfare Association extended their assistance to our society for conducting blood donation camps at Palam, Rajokari, Hindon, Subroto Park, Basant Nagar & Aya Nagar. May 8th World Thalassemia Day was sponsored by Torrent Pharmaceuticals Ltd. Thalassemics India acknowledges their kind contribution with deep gratitude.Genesis Foundation organized a ffffmarathon to help raise funds for threethalassemia children on 5th April 2009. Aarti Zaveri donated one of herCorporate Gurgaon was involved by artworks to Thalassemics India on 8thGenesis Foundation to help raise May as her token of love & regards.awareness and to collect funds. We are extremely grateful to her for her kind gesture.An annual street festival was alsoorganized at API Centre Gurgaon on 4th Thanks& 5th April,09 offering entertainment,Shopping, Street theater, Street Food,Music, Films & more to the visitors.Proceedings from both the eventshelped in saving the lives of three tothalasssemia children in need of BoneMarrow Transplant. You All ffff ffff
    • aemia SECRETARY’s Message Thalassemics India Dear FriendsA-9, Nizamuddin West, New Delhi-110 013, INDIA We are preparing very hard for the upcoming 4th International Conference Tel : 91-11-41827334 Fax : 91-11-24353871 on Thalassemia. Our last conference held in 2004 was a great success. E-mail : thalcind@yahoo.co.in website : www.thalassemicsindia.org We would like this conference to be a landmark event. PRESIDENT We are trying to spread the information about the conference to as many Deepak Chopra people as we can through mails, advertisements, post and posters. You Tel. : (R) 26511199 will agree that it is a good opportunity for Thalassemia patients, parents, VICE PRESIDENT doctors, social workers, medical students and Thalassemia experts to Dr. V.K.Khanna meet on a common platform for discussing and sharing their knowledge Dr. Gautam Bose and experiences. SECRETARY Shobha Tuli The Scientific and Organising committees are putting in great efforts to (M): 9810019950 make this conference a reality. We are confident that you will show a keen JT. SECRETARY interest in the conference by not only registering for the conference but Rekha Arora also by taking an active part in the conference. TREASURER At present there are a number of new challenges for us in the field of health Arun Sehgal policies, prevention of Thalassemia and collection of funds to meet the Tel. : (R) 28751677 ever increasing demands of our patients. We hope to push these issues JT. TREASURER very seriously in the coming months of the year. Deepak Dhingra EXECUTIVE MEMBERS You will find details of some of our past activities, news across the Rita jain country, new members and our forthcoming events in this latest issue Vasu Pahwa of ‘Thalassemia Update’. I hope you will enjoy reading it. Please continue Poonam Anand sending us your news, stories and suggestions. Ashwini Malik Gagandeep Singh Dr. Sabrina Sabharwal I would like to take this opportunity to thank all our friends, collaborators Gaurav Bose and donors for their valuable support and contribution to the success of our events. Editorial Board With Warm Regards, Chief editor Dr. V.K. Khanna Yours Sincerely, Co-editors Shobha Tuli & Dr. G. Bose Shobha Tuli Member Poonam Anand Published by Thalassemics India Printed at Process & Spot (M) 9811222771 3www.thalassemicsindia.org
    • THALASSAEMIA UPDATE_______________________________________________ Health Care Services Thalassemia care in DDU Hospital Dr Ritu Chawla CMO NFSG, Incharge Thalassemia Unit , Ddu Hospital.THALASSAEMIA UPDATE Deen Dayal Upadhyay Hospital is one (West Zone) since September, 1994 Nearly 25 patients were found to have of the major health care providing which is catering to the needs of this HCV antibody titers positive . With the hospitals of Govt of Delhi. Being hospital as well as other hospitals/ coordination with G.B.Pant hospital situated in West Delhi it provides major private nursing homes of this area gastroenterology department these health care supply for this region. for their blood requirement. Blood patients were tested for HCV RNA bank is very efficient in its provision titers . Six patients were found to be Deen Dayal Upadhyay hospital has of blood for various blood groups for HCV RNA titers positive in high range. been running a Thalassemia day thalassemia patients regularly without These patients have undergone liver care center successfully since 1999. much problems . Patient registered for biopsy and have been started on Started initially with few patients regular blood transfusion gets initially interferon and ribavarin combination this center has evolved into major his blood group antigen both major therapy. transfusion center run by the Govt. of and minor blood group tested. It is Delhi . one of the very few centers in Delhi Patients are also being screened for which is providing blood group cross hyperparathyroidism with the help Six beds are available for Thalassemia of calcium, phosphorus, alkaline day care center at ward 4 in indoor matching with minor antigens also. This prevents the formation of various phosphate, parathyroid hormone patient building. It is situated in a levels and a dexa scan and treated quiet atmosphere with a good antibodies on long term basis. accordingly .Patients in pubertal age indoor facility. Patients have access Thalassemia is a disease which group are also been investigated for to television for entertainment in the requires not only blood transfusion various endocrine sex hormones and ward. Facility for fridge and microwave on regular basis but also requires treated accordingly. is also available in the ward. medication for reduction of iron levels. DDU Thalassemia Center Treatment of thalassemia does not has been providing drugs for mean just treatment of disease but chelation regularly in full doses . also prevention of disease spread in Both deferiprone and desferral are community. Parents are counseled provided in sufficient doses from regarding the disease and spread. the hospital for free of cost. Patients Carrier status detection is carried are regularly monitored for their iron out in the Hospital. DDU is one of levels with serum ferritin levels which the three Govt hospitals in Delhi in has been started in hospital. Patients which HIGH PERFORMANCE LIQUID are also helped for their requirements CHROMATOGRAPHY is done for of pump with the help of hospital carrier detection free of cost. Nearly 148 patients of all age groups Antenatal screening in pregnant are registered for regular transfusion at and associations like Thalassemics India. Patients are regularly monitored women for Thalassemia is being this center. Patients are transfused on carried out in the hospital. Antenatal day care basis regularly in the hospital. for side effects of the drugs. Indeed good chelation helps in improving diagnosis of the fotus is also done Patients come here in the morning with the help of higher centers. at 9am. Blood sample is acquired life expectancy of these patients as and sent for cross match. Patients’ observed by us. blood to be transfused is available Patients are kept under supervision and by 2 hours from blood bank. Bed monitored with blood investigation side leucocyte filters are also made regularly. Patients growth is monitored available by the hospital. This reduces on every visit . Liver function test , the transfusion reaction usually kidney function test Thyroid function observed during transfusion. Under test are done from the hospital. supervision of doctor and trained nurses blood transfusion is carried Tests for blood transfusion infections out during day time. After transfusion like HIV, HBsAg and HCV are also patients are observed for some time done regularly. Thalassemia patients and parents and discharged same day. are already burdened with multiple 2 D Echo is also done on regular basis responsibility and we try our best D.D.U. Hospital has been made in the hospital to rule out cardiac not to load them with further financial Regional Blood Transfusion Centre involment. burden. 4
    • THALASSAEMIA UPDATE_______________________________________________ Health Care ServicesFuture prospectives. our services to whole of West Delhi. constant , enthusiastic support and We wish to increase the strength of guidance for our unit in DDU HospitalWe wish to continue providing beds for our Thalassemia unit.comprehensive care for Thalassemia We would sincerely like to thank Dr THALASSAEMIA UPDATEpatients improving their life Acknowlegment Mohini Kumar , Head of Departmentexpectancy and quality of life. We of Paediatrics, DDU , hospital for herwish to see our patients enjoying life We hereby acknowledge Dr Aveender constant inspiration, invaluable advice,with no complications and to expand Prasad, M.S.,DDU HOSPITAL, for his suggestions and help for this unit. Thalassemia Unit at Apollo Hospital, DelhiDr. Amita Mahajan, Sr. Consultant Paediatric Oncology / HaematologyThe Thalassemia Unit at Apollo which is much more sensitive and workup of these children especiallyHospital, Delhi was set up in 2003. further minimises the risk of acquired in terms of growth and pubertalMrs Tuli, Secretary, Thalassemics viral infections. The blood is also development. Dr Anupam Sibal,India and Dr Anupam Sibal, Director tested for Malaria antigen routinely. Pediatric Gastroenterologist providesMedical services at Apollo Hospital, gastroenterology backup. InDelhi were very keen that a service Again for over eight years all blood addition, every lady registeringis available for thalassemic children at was one log leucodepleted at the for the antenatal clinic at Apolloour hospital and on my joining Apollo time of collection. However, as hospital is automatically screened forHospital, I was asked to organise the it is recommended for thalssemic Thalassemia carrier state and servicessame. children to receive three-log leuco- for Chorionic Villus Sampling (CVS) depleted blood, we were using are available.With the help and support of our bedside filters till last year. Since lastcolleagues in the Blood Bank, Dr year, all the blood products undergo These are, however, the basics of anyR. N. Makroo and Dr V. Raina, we three-log leucodepletion at the time Thalassemia service. What we hadstarted enrolling patients in 2003. of collection prior to storage, which is hoped to achieve was that in additionA four-bedded area was set up. A the ideal way for thalassemic patients. to satisfactory hemoglobin and goodsubsidised package was devised to Now we do not use any bedside overall health, each and every childmake an all-inclusive package that filters. with Thalassemia has confidence,was much more affordable than the a sense of pride and self-esteemstandard charges in the corporate and can think beyond Thalassemia.sector. The management also helped For all families coping with chronicus further by giving us discounts in disorders, very often their entireinvestigations. But the most important lives run around this disorder. Whathelp came from the Blood bank, which I had hoped to accomplish wasagreed to provide blood through the to encourage families to take thishelp of voluntary donation. As it is disorder in their stride so that theirour hospital policy to not use blood children have a sense of normalcy infrom any other external source, and their lives and can achieve their trueit is not really feasible for families potential.of thalassemic children to provide Over the last six years, we have haddonors regularly, the Blood bank took to move the area twice because of Every time one of our membersit upon themselves to arrange the various renovations in the hospital shows me their report card with lotsblood supply and their support has and due to constant demand for of stars in it or their trophy for beingbeen unwavering. However, it has more bed space. Yet, all patients an all-rounder at school, I feel that wemeant being limited to a number so have co-operated in our endeavour have achieved our goal.that we can sustain this service. to provide a satisfactory service. I urge every child with Thalassemia andAs a service, our biggest advantage The annual testing, ongoing every family coping with this disorderis the quality of our blood in terms monitoring, counselling for future to instill positivity and optimism inof safety. Since the inception of this pregnancies all are a continuing their home environment so that thesehospital, all blood products have process in all Thalassemia units and the children can truly bloom. For, thebeen screened for Hepatitis C in same holds true for us. Every patient flower that blooms in adversity is theaddition to Hepatitis B, much before is on chelation. Dr Anju Virmani, rarest and the most beautiful of allit was legally required to do so. Two Pediatric Endocrinologist provides ffffyears ago, we moved to NAT testing the back up for endocrinology 5www.thalassemicsindia.org
    • THALASSAEMIA UPDATE___________________________________________________ Scientific Article Growth and Puberty in Thalassaemia Nicos Skordis, MD and Andreas Kyriakou, MD Pediatric Endocrine Unit, Makarios Hospital, Nicosia, CyprusTHALASSAEMIA UPDATE Introduction in well-treated children in developed that other contributing factors like countries. The response of growth haemosiderosis and deficiency of Treatment of beta-thalassaemia major hormone to stimulation tests has trace elements should influence spine is based on regular blood transfusions been found to be normal or reduced. growth (3). Sex steroid replacement to maintain the pre-transfusional Hb The presence of neurosecretory therapy cannot adversely affect body level above 9 gr/dl and appropriate dysfunction is supported by the disproportion, as truncal shortening chelation therapy to avoid the impaired 24 hour GH secretion. at final height is evident in patients consequences of iron overload. The Evidence for partial GH resistance is with either spontaneous or induced metabolically active iron catalyses based on the fact that children with puberty (7). Body disproportion the formation of free radicals, which thalassaemia major have normal GH therefore is independent of pubertal damage membrane lipids leading to and GHBP, with low levels of IGF-1 or prepubertal period of greater cell death and eventually organ failure. and IGFBP-3, which are not always height gain. The endocrine glands are particularly properly increased following GH vulnerable to the excess iron, so Figure 1: Standing height and sitting height is stimulation. Moreover the therapeutic SDS in different age-groups 6. that the appearance of endocrine administration of GH did not fulfil 12 dysfunction in thalassaemia major all our expectations and often 10 (TM) is a common and disturbing supraphysiological doses of GH are 8 6 complication, which requires required to overcome this resistance 4 Normal Menses prompt recognition and treatment. and lead to an improvement in linear 2 Primary 0 amenorrhea The contribution of the underlying growth (3,4,5). The response to 0 30 60 90 120 Secondary amenorrhea molecular defect in TM to the growth hormone treatment cannot development of endocrinopathies, be predicted based on known and particularly hypogonatotrophic Management parameters such as growth velocity, hypogonadism, is significant because age, height SDS, bone age SDS, IGF- Can children with thalassaemia major the patients with the more severe 1 levels and the type of abnormality attain normal stature and develop defects have a greater rate of iron in the GH-IGF-1 axis, suggesting that normally with early and reasonable loading through higher red cell additional factors such as skeletal Desferrioxamine treatment? Although consumption (1). dysplasia and Desferrioxamine (DFX) iron chelation can decrease the The child with thalassaemia major has toxicity are implicated in the child’s frequency of endocrinopathies, early a particular growth pattern, which growth retardation. DFX treatment may result in growth is relatively normal until age of 9–10 impairment. On the other hand poor Desferrioxamine (DFX) toxicity and compliance with DFX may eventually years; after this age a slowing down body disproportion of growth velocity and a reduced lead to severe iron burden, gonadal or absent pubertal growth spurt Short stature with disproportionate dysfunction and eventually growth are observed. The pathogenesis of body composition due to failure (8). The benefits of treatment growth failure is multifactorial (2), as desferrioxamine toxicity has been should be weighted against the observed. Desferrioxamine exhibits potential adverse effects and the shown below. its toxic effect on growth by inhibiting caring physician should balance • Chronic anemia, hypersplenism, DNA synthesis, fibroblast proliferation between the efficacy and the chronic liver disease (HBV, HCV) and collagen formation, causing injudicious use of Desferrioxamine. • Zinc and folic acid deficiency, flattening of the vertebral bodies An ideal therapeutic regimen, which skeletal dysplasia, (platyspondylosis) and eventually will avoid the toxic effects of iron • Desferrioxamine toxicity poor spine growth. Both sitting and overload and that of continuous • Emotional disturbance standing heights are normal until subcutaneous chelation therapy, • E n d o c r i n o p a t h i e s : the age of 6-9 years but gradually has yet to be found. It is therefore hypothyroidism, delayed puberty, decreased in older ages with recommended that growth in both hypogonadism, particular shortening of the sitting standing and sitting position should • dysfunction of the growth height as shown in figure 1(6). Body be assessed at 6-month intervals in hormone (GH) – IGF 1 axis disproportion with short trunk has order to detect early growth failure. been reported in patients who Long-term observations on the Growth Hormone (GH) effect of therapy are needed before have been poorly chelated during Chronic anaemia is no longer a factor childhood and adolescence, so this mysterious puzzle is solved. 6
    • THALASSAEMIA UPDATE___________________________________________________ Scientific ArticleAlternative oral chelation agents liver function tests, fer ritin, progression –once puberty hasare often an option in cases of DFX biochemical profile, and zinc started –for more than one year,toxicity, although some bone lesions 4. Urine analysis where testicular volume in boys never THALASSAEMIA UPDATEremain irreversible. 5. Thyroid function tests (Free T4, exceeds 6 to 8 ml and breast size TSH) in girls remains unchanged. Failure of• Prevention of growth retardation is 6. IFG-1 and IGFBP-3 sexual development by the age of 15 essential. 7. Stimulation tests to assess GH to 16 years in both sexes is defined• Monitoring growth in all children secretion. At least two tests are as hypogonadism. Secondary by using growth charts for both required. Priming with sex steroids hypogonadism appears later in standing and sitting height is is necessary in boys older than life, and is manifested in women mandatory. 10 years with testosterone and as secondary amenorrhea and in• The mean hemoglobin levels must in girls older than 9 years with men as decline in sexual drive and be kept near to 9 gr/dl. Estrogens. azzoospermia.• Prompt initiation of iron chelation 8. IGF-1 generation test in patients therapy prevents pituitary with low levels of IFG-1 and Adolescent girls with TM often haemosiderosis, which is the IFGBP-3 and normal GH secretion present with primary amenorrhea and main cause of growth hormone to exclude GH resistance. boys fail to become well virilized. The insufficiency. damage to the hypothalamus and• Treatment with growth hormone Puberty pituitary is progressive, even when is recommended when GH intensive chelating therapy is given and deficiency is established. In poor Puberty is the biological phenomenon, the appearance of hypogonadism in responders such treatment should which results from the activation of both sexes is often unavoidable. Most be discontinued. Therapeutic the hypothalamic–pituitary–gonadal women with TM manifest secondary response with GH administration axis and is clinically manifested by the amenorrhea at some stage in their life in cases with GH deficiency, is appearance of sexual characteristics. and men develop hypogonadism in often non satisfactory. Delayed puberty is defined as the their 3rd decade after being normal• Growth acceleration is mostly absence of any pubertal sign in girls for some years and even becoming promoted with sex steroids in (breast enlargement) and in boys fathers (11, 12, 13). children with pubertal delay. (testicular enlargement) by the age of• Sexual complications present a 13 and 14 years respectively. Delayed Protocol for investigation of significant issue in thalassaemics. puberty in thalassaemia is almost pubertal disorder These include: delayed always due to hypogonadotrophic hypogonadism, which still remains The absence of any clinical pubertal puberty, arrested puberty and signs in a boy (testicular enlargement) hypogonadism. Transfusional the most common endocrine and stressful complication in thalassaemia older that 14 years and in a girl (breast haemosiderosis in the pituitary development) older than 13 years gonadotroph cell causes major (9) (figure 2). The association of hypogonadotrophic hypogonadism requires investigation. gonadotrophin deficiency, which is the underlying abnormality. with the genotype has already been 1. Measure testosterone in the boy Histological examination of the proven (1). An additional contributing and oestradiol in the girl. DHEA-S gonads shows minimal siderosis, factor, whose role is thought to be in both sexes is often helpful so that the ovarian and testicular weak, is the impaired synthesis of 2. Perform the GnRH test to evaluate function is well preserved. leptin in thalassaemic patients which the pituitary capacity to secrete seems to be related to transferrin the gonadotropins FSH andProtocol for investigation of poor receptor levels (10). LH, where the response ingrowth in thalassaemic children hypogonadism is low 301. Measure current height both 25 3. Bone age is helpful for the standing and sitting and plot on 20 treatment decision options 15 the growth chart. Calculate the 10 Normal Menses Therapeutic approach in delayed target height based on parental 5 0 Primary puberty should mimic biological and amenorrhea heights. Compare with previous 0 30 60 90 120 Secondary amenorrhea biochemical pubertal changes, aiming measurement to estimate on promotion of linear growth as well Figure 2. FSH (a) and LH levels (b) after GnRH the growth velocity. Examine stimulation in thalassaemic women with normal (14,15,16,17). pubertal status. Note any physical menstrual cycles, primary amenorrhea and disproportion. Review emotional secondary amenorrhea. X-axis: time in minutes. Induction of puberty in boys can be and social status. Y-axis: FSH and LH in miu/l 6 achieved with testosterone depot2. Assess bone maturation IM 25-50mg monthly for 6 months Arrested puberty is defined as3. Routine blood tests including the absence of further pubertal (Continued on page 16..................) 7www.thalassemicsindia.org
    • 4th International Conference on Thalassemia 31st October - 1st November, 2009 New Delhi, IndiaTHALASSAEMIA UPDATE Invited Faculty Dr. M.B. Agarwal, India Dr. Rekha Bajoria, United Kingdom Dr. Nica Cappellini, Italy Dr. Mammen Chandy, India Dr. Ratna Chatterjee, United Kingdom Prof. Renzo Galanello, Italy Dr. Antonio Piga, Italy Dr. Michel Sadelain, U.S.A Dr. Farrukh Shah, United Kingdom Dr. Malcolm Walker, United Kingdom Patrons Mr. Panos Englezos - President, Thalassaemia International Federation Dr. B.K. Rao - Chairman Board of Management, Sir Ganga Ram Hospital Dr. Anupam Sibal - Group Medical Director, Apollo Hospitals Group T.I.F. Advisory Committee Dr. Androulla Eleftheriou - Executive Director, TIF Dawn Adler - Board Member, TIF George Constantinou - Secretary, TIF Gargi Pahuja - Board Member, TIF Mrs. Fatemah Hashemi - Board Member, TIF National Advisory Committee Dr. M. B. Agarwal Dr. Manorama Bhargava Dr. Sharmila Chandra Dr. V.P. Choudhary Dr. Sunita Mittal Dr. I.C. Verma For registration or any other information contact: Shobha Tuli Secretary, Organising Committee M: 9810019950 Conference secretariat: Thalassemics India A-9, Nizamuddin West, New Delhi-110013, India Tel. (O) : 41827334, Fax : 24353871 E-mail: thalcind@yahoo.co.in Website: www.thalassemicsindia.org 8
    • THALASSAEMIA UPDATE_________________________________________________________ Activities World Thalassemia Day, 8th May, 2009 THALASSAEMIA UPDATEMay 8th, The World Thalassemia Day performed beautifully on “Des to the society.was celebrated with great fervour Mera Rangeela”. A group of 14this year at Sri Sathya Sai International thalassemics from Delhi gave a vibrant Ms. Shovana Narain encouragedCentre with around 600 Thalassemia medley performance. Tushar Arora an the children, their families and thechildren, parents, doctors, supporters upcoming singer was applauded by society. She offered all possible helpand well wishers. one and all. in her capacity. Ms Anjana Kuthalia also promised all possible help forDr. Kanwar Sain, Mayor of Delhi Mrs. Shobha Tuli, Secretary our children.was the Chief Guest. Ms. Shovana Thalassemics India, welcomed theNarain and Ms Anjana Kuthalia were Chief Guest and invited dignitaries. She The cultural programme continuedthe Guests of Honour. talked about thalassemia scenario in with Salaam Balaak Theater Group Delhi and requested the Mayor to get performance.Before the cultural programme a short all the thalassemics of Delhi registered,one hour session was conducted on Later the audience enjoyed the give space for awareness at specific performance by Sanjay Raina, VandanaAsunra by Dr. V.K.Khanna, Head of sites and open Thalassemia CentersThalassemia Department. SGRH and Dr. Vadhera, Garima and Guitarists from at all the new upcoming hospitals Amit Upadhyay Guitar School.Sunil Goomber Head of Thalassemia having blood bank facilities.Department , G.T.B Hospital. Parents All the thalassemia children wereand patients cleared their doubts Dr. Kanwar Sain praised the given back gifts.about the usage and effects of the performance by thalassemia children.new drug. He very kindly felicitated some of the Thalassemics India is thankful to young thalassemics who had excelled Torrent Pharmaceuticals Ltd forThe cultural programme began with in sports, academics and extra sponsoring the May 8th function. Wethe lamp lighting and vandana sung curricular activities. He appreciated are also grateful to our well wishersby Shivangi Amrit. the work of Thalassemics India. He and friends - AOC Monitors, Pepsi,Thalassemia children from Faridabad agreed to extend all possible support SDIPA and others. Special Darshan to Akshardham TempleWe were approached by where delicious lunch was served or the Gaint Screen Film thatthalassemia children and their with great affection as the vividly potrays Neelkaanths epicparents expressing their desire prasadam. pilgrimage of India. 3rd Phaseto see Akshardham Temple. was the Sankriti Vihar. The mostThalassemics India decided to Children and parents seemed very fascinating boat ride experiencestake a group of 221 people to excited and full of enthusiasm. Indias glorious Heritage.Akshardham Temple. Exhibition of the Akshardham temple was divided in three phases. Darshan at Akshardham wasAfter the security check we all truely a breathtaking experienceproceeded towards a huge hall 1st Phase was Sahajanand as the Indian culture in all itswhere our group of 221 people Darshan or the Hall of Values - grandeur beauty, wisdom and blisswas divided into 2 groups and here the values for the mankind was depicted and all the peoplewere allocated 2 guides as group are presented though audio- took beautiful spiritual messagesleaders. After the exhibition all animatronics and light and sound along with them at their homes.the children and the parents were diorama presentations from lifetaken for lunch in the Cafetaria of Bhagwan Swaminarayam.2nd Phase was the Neelkanth Darsham 9www.thalassemicsindia.org
    • THALASSAEMIA UPDATE_____________________________________________________New Members Our Shining Stars Our Worthy Donors Torrent Pharmaceuticals Ltd.THALASSAEMIA UPDATE Rs.3,75,000.00 Trent Co. Rs.47,000.00 Surya Foundation Pyari Praneeta Mittal secured 73% in Daring Dipin secured 68% in Class XII Rs.30,000.00 Class XII Commerce. Boards despite all odds. Ashwani Kumars Rs. 45,000.00 Anita Mahajan Rs.25,000.00 Brindavan Agro Industries Rs.25,000.00 Nanhi Niyati secured 76.4% in Class X Brave & Beautiful Bitosta Bit topped Rajarshri Guha Boards, 09. humanities stream in her school by Rs.24,930.00 securing 81% in class XII. P&G Enterprises Pvt. Ltd. We at Thalassemics India are proud of you and Rs.16,188.00 wish you all the success in life Sonal Gupta Rs.12,800.00 WE WELCOME THE FOLLOWING Rita Jain NEW MEMBERS Rs.6,620.00 Alka Nath M.L.Ahuja Delhi Subhash Khanna Haryana Rs.6,620.00 Manish Bhai Patel Ahmedabad Vikas Takral Girsa Uma Ashok Bansal Rs.6,000.00 Shafique Ahmed Khan Ahmedabad Susanta Sahu Kolkata Educare Malay Paul West Bengal Pramod Anandrao Maharashtra Rs.6,000.00 Shobha Tuli Vikas Jain Ghaziabad Mayank M. Patel Guajarat Rs.5,000.00 Aseem Jain Delhi Sunita S. Singh Rs.6,80.00 Saroj Choudhary Would you like to help those who ACKNOWLEDGEMENT Rs.5,00.00 are in need of Infusion Pumps? Thalassemics India would like There are many thalassemic patients who are in need of to thank all those who have Infusion Pumps. At the moment donated desferal injections to Change of Address they are either sharing their the society. With your help Please inform Thalassemics India pumps with others or they are we have been in a position to if there is any change in your forced to take the treatment in the postal address. hospital. help some of our thalassemia Thalassemics India children in need of chelation. A-9, Nizamuddin West, New Delhi-13 Your donation will be duly acknowledged by us. Thanks once again ! email:- thalcind@yahoo.co.in 10 10
    • World Thala Celebra Lamp Lighting Ms. Shovana Narain (Guest of Honour) lauding the efforts of our childrenDr. V.K. Khanna and Dr. Sunil Gomber taking questions from the parents “Desh Mera Rangeela” by thalassemics from Foundation against Thalassemia Society Vandana sung by Shivangi Amrit
    • assemia Dayations Performance by Salam Balak Trust troupe Ms. Anjana Kuthalia (former Ms India) promising all possible help Children enjoying the evening with Ms. Vandana Vadhera Tushar Arora Singing on the tunes of Guitarist from Amit Upadhyay school of Guitar Children on the stage dancing and enjoying.
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    • THALASSAEMIA UPDATE_______________________________________________________ Awareness Towards Awareness THALASSAEMIA UPDATETalk on Thalassemia Gupta, Inner wheel club Delhi Main.Dr.V.K. Khanna addressed 60 studentsof class XIth at Air Force Bal BhartiSchool, Lodhi Road. The talk wasfollowed by Q/A session, VicePrincipal Mrs. Malhotra proposed avote of Thanks.Talk on ThalassemiaMrs. Shobha Tuli gave a talk on Sr. Sec. School, Old Willington Camp.awareness and prevention of Mrs. Menon Principal appreciatedThalassemia to the students of Air Force the efforts of Thalassemics India. Thalassemia Screening Camp A Thalassemia Screening Camp was organized in MCD Primary School, Malviya Nagar. Total of 52 blood samples were collected & sent to Sir Ganga Ram Hospital, for testing. Thanks to Ms. Nirmal Blood Collection Drive Blood Donation Camps organized by Thalassemics India Venue Units Collected Pepsi Co, Gurgaon 30 Huges, Gurgaon 51 Nbrc, Gurgaon 36 Jass, Gurgaon 56 Oracle, Gurgaon 29 Airforce Station, Hindon 70 Airforce Station Basant Nagar 29 Airforce Station Subroto Park 21 Airforce Station Rajokari 25 Airforce Station , Aya Nagar 38 Airforce Station, Palam 33 Blood Donation Camps Supported by Thalassemics India Venue Units Collected Dwarka 43 Janak Puri 49 South Ext. 226 15www.thalassemicsindia.org
    • THALASSAEMIA UPDATE___________________________________________________ Scientific Article (...............Continued from page 7) Prompt chelation therapy before height and body disproportion and reassessment. Pubic hair will pubertal age and before extremely in Thalassaemic boys and girls appear and penile size will increase. high levels of ferritin are reached is with spontaneous or inducedTHALASSAEMIA UPDATE Increase in testicular volume indicates the fundamental tool to help children puberty. Acta Paediatr 2000; activation of the axis and release with thalassaemia major to attain 89:1295-1301 of gonadotrophins (FSH and LH), normal stature and sexual maturity 8. De Sanctis V, Roos M, Gasser where no further treatment is needed and to improve their bone mass. T, For tini M, Raiola G, Pintor except for close observation. In case Poor pubertal growth in adolescents C. Impact of long-term iron where testicular size is unchanged, with thalassaemia major does not chelation therapy on growth then treatment is continued for 6 solely depend on gonadal failure. and endocrine functions in months and subsequently the dose No difference has been observed Thalassaemia. J Pediatr Endocrinol is increased to 100mg monthly for in pubertal growth and final height Metab 2006; one year. Therapeutic schedule is between treated hypogonadal 9. I t a l i a n Wo r k i n g G r o u p o n determined by the growth potential, patients compared to those with Endocrine Complications in Non- clinical response and emotional spontaneous puberty (18,19). Failure Endocrine Diseases. Multicentre factors. For testicular enlargement, to progress normally through puberty study on prevalence of endocrine the therapeutic regime is altered to is associated with failure of adequate complications in Thalassaemia the combination of hCG and hMG, bone mineralization and achievement major. Clin Endocrinol 1995; 42: both of which mimic the pituitary of peak bone mass, which is a 581-586. gonadotrophins. The final adult dose contributing factor to the ultimate 10. Dedousis GVZ, Kyrtsonis MC, is testosterone depot 50mg/weekly bone disease in thalassaemia (20). Andrikopoulos NE, Voskaridou IM or alternatively transdermally in E, Loutradis A. Inverse correlation References of plasma leptin and soluble patches 5 mg/daily. The oral route (testosterone undeconate) should be 1. Skordis N, Michaelidou M, Savva transferrin receptor levels in avoided due to liver toxicity. SC, Ioannou Y, Rousounides β-thalassaemia patients. Ann A , Kleanthous M, Skordos Hematol 2002; 81: 543-547. For induction of puberty in girls oral 11. De Sanctis V, Vullo C, Katz M, G, Christou S. The impact of ethinylestradiol is preferred at the dose Wonke B, Tanaw R , Bagni B. Genotype on Endocrine 100ng/kg/day for 6 months, where Gonadal function in patients complications in Thalassaemia increase in breast size and growth with B–Thalassaemia Major. J Clin major. Eur J Hematol 2006 acceleration is noted. This dose is Pathol 1988; 41:133-137. 2. Skordis N. The growing child with continued for additional 6 months 12. Skordis N, Gourni M, Kanaris C, Thalassaemia. J Pediatr Endocrinol and increased to 200ng/kg/day for Toumba M, Kleanthous M, Karatzia Metab 2006; 19: 467-9. the subsequent year. Therapeutic N, Pavlides N, Angastiniotis M. 3. De Sanctis V. Growth and schedule is determined by the same The impact of iron overload and puberty and its management in factors as in boys. The adult dose genotype on gonadal function in Thalassaemia. Horm Res 2002; is 400ng/kg/day, where the uterine women with Thalassaemia major. 58(S1): 72-79 size is satisfactorily increased for the Ped Endocrinol Rev 2004; 2(S2): 4. Raiola G, Galati MC, De Sanctis induction of menarche. Induction of 292-295 V, Caruso-Nicoletti M, Pintor puberty can be successfully achieved 13. Skordis N, Petrikkos L, Toumba C, De Simone M, Arcuri VM, by the transdermal use of estrogens. M, Simamonian K, Hadjigavriel M, Anastasi S. Growth and puberty Menarche is achieved by the addition in Thalassaemia major. J Pediatr Sitarou M, Kolnakou A, Skordos of Medroxyprogesterone 10 mg/ Endocrinol Metab 2003; 16: G, Pangalou E, Christou S. Update day for 10 days when the size of the 259-266 on fertility in Thalassaemia major uterus exceeds 5cm. When menstrual 5. De Sanctis V, Urso L. Clinical Pediatric Endocrine Reviews. bleeding occurs spontaneously during experience with Growth 2004; (S2):296-302 estrogen treatment, the regime should Hormone treatment in patients 14. Pozo J, Argente J. Ascertainment be adjusted. For maintenance of the with beta-Thalassaemia major. Bio and treatment of delayed puberty menstrual cycle the use of estrogens Drugs 1999; 11:79-85 Horm Res. 2003; 60 (S3):35-48 (Conjugated Estrogens 0.625 ή 1.25 6. Toumba M, Sergis A , Kanaris 15. Mac Gillivray MH. Induction mg, Ethinyl Estradiol 20 μg) from C, Skordis N. Endocrine of puber ty in Hypogonadal day 1st to 25th and Progesterone complications in patients with children. J Pediatr Endocrinol from day 14th to 25th is required. Thalassaemia major. Pediatric Metab 2004;17(S4):1277-1287 The transdermal use of Estradiol and Endocrine Reviews 2007;5:642- 16. Car uso –Nicoletti M, De Norethisterone is advantageous due 648 Sanctis V, Cavallo L, Raiola G, to decreased liver toxicity. 7. Filosa A , Di Maio S, Baron I, Ruggiero L,Skordis N, Wonke Esposito G, Galati MG. Final B. Manegement of puberty for 16
    • THALASSAEMIA UPDATE_________________________________________________________ TIF News optimal Auxological results. J between treated Hypogonadal 530 Pe d i a t r E n d o c r i n o l M e t a b and non-Hypogonadal 20. Bielinski BK, Darbyshire PJ, Mathers 2001;14(S2):939-944 Thalassaemic patients. Horm Res L, Crabtree NJ, Kirk JM, Stirling HF, THALASSAEMIA UPDATE 17. De Sanctis V, Vullo C, Katz M, 2004;62:17-22 Shaw NJ. Impact of disordered Wonke B, Nannetti C, Bagni B. 19. Filosa A, Di Maio S, Lamba M, puberty on bone density in beta- Induction of spermatogenesis in Baron I, Saviano A, Esposito G. Thalassaemia major. Br J Haematol. Thalassaemia. Fertility Sterility Bone age progression during 2003; 120:353-358 1998; 50: 969-975. five years of substitutive therapy 18. Caruso-Nicoletti M, De Sanctis V, for the induction of puberty Ackowledgement Raiola G, Skordis N, Manusco M, in Thalassaemic girls-effects on "According to TIF Magazine, the Coco M, Wonke B. No difference height and sitting height J Pediatr official newsletter of the Thalassemia in pubertal growth and final height Endocrinol Metab 1999;12:525- International Federation." aemiaTIF NEWS TIF Board meeting in progress Dr. Nica Cappellini, Mrs. Fatemah TIF NEWS TIF Board Meeting was held in Hashemi & Shobha Tuli in Syria Damascus, Syria. Out of the18 Audited accounts and management board members, 11 members were accounts for 2008 were presented present. and approved. Annual TIF report, The minutes of the last board meeting Involvement of TIF in health related and the minutes of the last general policies, fund raising, regional Assembly were approved. priorities, MSC fellowship course. hosting of next TIF conference and a number of other issues were discussed. TIF website www.thalassaemia.org.cy Readers’ Queries Answered by Dr. V.K Khanna (Head, Thalassaemia Unit, SGRH) Q.1 My son is using Asurna from the last one and half years but his serum ferritin is 2600. Should I switch back to Desferal as Kelfer doesn’t suit him? Ans. You have not mentioned the level of serum ferritin before staring the Asunra. For the time being you continue Asunra. It is a safe and effective medicine. Q.2 I am a mother of an adult Thalassemic who is 22 years old. I want to know if Asurna has any effect on sperm count in males and menstrual cycle in females. What about Kelfer and Desferal? Do we have any studies done on them? Ans. None of the medicines like Asunra, Desferal or kelfer effect the sperm count in males and fertility / menstrual cycle in females. 17 www.thalassemicsindia.org
    • THALASSAEMIA UPDATE_________________________________________________ News Across India NEWS ACROSS INDIATHALASSAEMIA UPDATE Jamshedpur Bangalore in the program and answered the questions raised by the patients and “Dr. S.P Foundation” successfully On the occasion of World Thalassemia their parents. observed “International Thalassaemia Day on May 8th, 2009 public Day” at “Hotel Boulevard” Bistupur, awareness and education program on Dr. Karuna Rameshkumar, thanked Jamshedpur. Thalassemia was organized through the management for their constant Jai Vigyan mission project Phase II at support and appreciated the St. John’s Medical College hospital. involvement of faculty, staff and the The theme of the day was “Together industry. we Care” and efforts of Dr. Cecil ffff Ross and Dr. Karuna Rameshkumar to organize the programme were ably supported by staff from laboratory, Ludhiana blood bank and medical social work department and faculty from general To mark the World Thalassemia medicine and pediatrics and project Day, Dayanand Medical College & staff. Hospital, Ludhiana held a medicos Chief Guest, Mr. Saryoo Rai, M.L.A, voluntary blood donation camp on Jharkhand Govt.inaugurated the programme by garlanding the portrait of Dr. S.P Chatterjee and litting the inaugural lamp. Welcome song was presented by Dr. Rachita Roy. Welcome address was given by Mrs. Madhumita. After that message of Tanushree Chatterjee, International Thalassaemia Programme Coordinator of Dr. S.P Foundation and Medical Student of Southern Medical University, China On May 8th, the programme started was read out by Madhumita. Then with a prayer at 2.30 pm. Thalassemia 8th May, 2009. the Students, Interns Dr. T.K. Chatterjee, Director of Dr. S.P children who are being treated in and consultants donated 207 units of Foundation elaborately explained St. John’s medical college hospital blood at this camp. about Thalassemia and its genetic welcomed the executives. II MBBS ffff inheritance. students performed a skit which highlighted the clinical features of Dr. Chatterjee said that every Thalassemia and a take home message Kerala Thalassemic should be monitored on inheritance. Pamphlets about properly for their better health care. inheritance were distributed. Thalassaemia day was observed He said “Undetected Cases” are in the auspicious of Blood much more than “Detected Cases” patients protection council and of Thalassemia. So he appealed the Malabar THAS society .In this part State Govt. to provide an H.P.L.C. a population blood screening Machine for Mass Screening and data programme, Hepetitis.B vaccination based prevalence. Dr. Chatterjee programme free distribution of said that Jharkhand State is a Malaria medicines, Haematology medical Prone area so here Thalassemia may camp, pamphlet distribution were be in higher count. He informed that organized. Eminent youth leader of free Thalassaemia Help Line 0657- Kerala and the member of Legislative 2425357 and Thalassemia Care Unit Assembly Adv. A.Pradeep Kumar MLA for counselling is gaining popularity. In Dr. Sitalakshmi from the laboratory, inaugurated the function. Giving his this occasion Mr. G.M. Sharan, Rony D’ Dr. Cecil Ross from general medicine own blood sample the inauguration Costa also delivered their speech. and Dr. Indumathy, Dr. Ranjani and Dr. of population blood screening Anand from Pediatrics participated (Continued on page 22..................) ffff 20
    • THALASSAEMIA UPDATE ® 21www.thalassemicsindia.org
    • THALASSAEMIA UPDATE_________________________________________________ News Across India (...............Continued from page 20) Chikitsalay Ajmer. The conference was well attended programme were also inaugurated by nearly 300 delegates comprising Ajmer Region Thalassemia welfare of Physicians, Patients and Parents. by Mr. Pradeep Kumar MLA. In his Society (regd) celebrated WorldTHALASSAEMIA UPDATE speech Mr.Pradeep Kumar stated that Thalassemia Day on 8th May,2009 at all organizations and NGOs should city water Park. help the thalassaemia screening movement and participate to the process of prevention programme till the eradication is a reality not only in Malabar area but also in the entire state of Kerala. He advised the organizers to approach all like minded organizations and youth bodies to achieve the goal. Prof. Ratna Chatterjee from U.K, Prof. S. Fucharoen from Thailand, Prof. V.P Chodhury from Delhi , Prof. 85 thalassemic children celebrated M.B Agarwal from Mumbai, Prof. N. World Thalassemia Day by cake Marwah from Chandigarh, Prof. G. cutting. Choudhury from Lucknow, Prof. Dr. ffff Andrew Wu from Singapore were the main speakers at the conference. Gujarat Seminar on Thalassemia Organized by M. Sudeendra Kumar president, Indian Red Cross Society, Vadodara Press Club, Calicut, distributed the at K.G. Children Hospital, Vadodara, medicines. Noted eye specialist Gujarat. Prof:Chandrakanth inaugurated the HepatitisB vaccination program. Dr P.M.Kutty, president of Malabar THAS society presided over the function. Kareem Karassery Gen.convener of Mrs. Shobha Tuli and Retired Chief Blood Patients Protection Council and Justice, Mr. Chittotosh Mukherjee had the secretary of Malabar THAS society the honour to open the conference. gave the key note address. ffff ffff The Editorial Committee reserves the right to change the text of the articles sent for publication where necessary, in good faith. Kolkata The Editorial Committee or Thalassemics India do not accept any responsibility for 2nd workshop on Thalassaemia any inaccuracies or omissions. was organized by the Hematology Foundation, Kolkata. The views expressed are not necessarily   that of Thalassemics India. ffff Reproduction of the material published in Thalassaemia Update for Educational purpose is encouraged, provided it is Ajmer accompanied by the following “According to Thalassaemia Update, the official Ajmer Region Thalassemia welfare newsletter of Thalassemics India”. Society (regd) collected 281 units of blood on 12th April,2009 at a Thank You ! We are thankful to all those who blood Donation Camp organized at have contributed in this issue of Zonal blood Bank, Jawahar Lal Nehru Thalassaemia Update. 22 22
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