ThalassaemiaIssue 34 • July 2009

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

Our Worthy Donors

News Across India

Towards Awareness

Article on Growth & Puberty

Blood Donation Camps

Readers’ Querieswww.thalassemicsindia.org

4th InternatIonal ConferenCe on thalassemIa31st october - 1st november, 2009

New Delhi, India

“It Helps To Know”

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Organised by: In collaboration with:

Thalassaemia International FederationThalassemics India Sir Ganga Ram Hospital

Thanks to

You All

May 8th World Thalassemia Day was sponsored by Torrent Pharmaceuticals Ltd. Thalassemics India acknowledges their kind contribution with deep gratitude.

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Aarti Zaveri donated one of her artworks to Thalassemics India on 8th May as her token of love & regards. We are extremely grateful to her for her kind gesture.

Genesis Foundation organized a marathon to help raise funds for three thalassemia children on 5th April 2009. Corporate Gurgaon was involved by Genesis Foundation to help raise awareness and to collect funds.

An annual street festival was also organized at API Centre Gurgaon on 4th & 5th April,09 offering entertainment, Shopping, Street theater, Street Food, Music, Films & more to the visitors. Proceedings from both the events helped in saving the lives of three thalasssemia children in need of Bone Marrow Transplant.

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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.

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Special Thanks

www.thalassemicsindia.org

Dear Friends

We are preparing very hard for the upcoming 4th International Conference on Thalassemia. Our last conference held in 2004 was a great success. We would like this conference to be a landmark event.

We are trying to spread the information about the conference to as many people as we can through mails, advertisements, post and posters. You will agree that it is a good opportunity for Thalassemia patients, parents, doctors, social workers, medical students and Thalassemia experts to meet on a common platform for discussing and sharing their knowledge and experiences.

The Scientific and Organising committees are putting in great efforts to make this conference a reality. We are confident that you will show a keen interest in the conference by not only registering for the conference but also by taking an active part in the conference.

At present there are a number of new challenges for us in the field of health policies, prevention of Thalassemia and collection of funds to meet the ever increasing demands of our patients. We hope to push these issues very seriously in the coming months of the year.

You will find details of some of our past activities, news across the country, new members and our forthcoming events in this latest issue of ‘Thalassemia Update’. I hope you will enjoy reading it. Please continue sending us your news, stories and suggestions.

I would like to take this opportunity to thank all our friends, collaborators and donors for their valuable support and contribution to the success of our events.

With Warm Regards,

Yours Sincerely,

Shobha Tuli

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Thalassemics India A-9, Nizamuddin West, New Delhi-110 013, INDIA

Tel : 91-11-41827334 Fax : 91-11-24353871E-mail : thalcind@yahoo.co.in

website : www.thalassemicsindia.org

PRESIDENTDeepak Chopra

Tel. : (R) 26511199

VICE PRESIDENTDr. V.K.Khanna

Dr. Gautam Bose

SECRETARYShobha Tuli

(M): 9810019950

JT. SECRETARYRekha Arora

TREASURER Arun Sehgal

Tel. : (R) 28751677

JT. TREASURERDeepak Dhingra

EXECUTIVE MEMBERSRita jain

Vasu PahwaPoonam AnandAshwini Malik

Gagandeep SinghDr. Sabrina Sabharwal

Gaurav Bose

Editorial Board

Chief editorDr. V.K. Khanna

Co-editorsShobha Tuli & Dr. G. Bose

MemberPoonam Anand

Published by Thalassemics IndiaPrinted at Process & Spot (M) 9811222771

www.thalassemicsindia.org

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THALASSAEMIA UPDATE ______________________________________________ Health Care Services

Deen Dayal Upadhyay Hospital is one of the major health care providing hospitals of Govt of Delhi. Being situated in West Delhi it provides major health care supply for this region.

Deen Dayal Upadhyay hospital has been running a Thalassemia day care center successfully since 1999. Started initially with few patients this center has evolved into major transfusion center run by the Govt. of Delhi .

Six beds are available for Thalassemia day care center at ward 4 in indoor patient building. It is situated in a quiet atmosphere with a good indoor facility. Patients have access to television for entertainment in the ward. Facility for fridge and microwave is also available in the ward.

Nearly 148 patients of all age groups are registered for regular transfusion at this center. Patients are transfused on day care basis regularly in the hospital. Patients come here in the morning at 9am. Blood sample is acquired and sent for cross match. Patients’ blood to be transfused is available by 2 hours from blood bank. Bed side leucocyte filters are also made available by the hospital. This reduces the transfusion reaction usually observed during transfusion. Under supervision of doctor and trained nurses blood transfusion is carried out during day time. After transfusion patients are observed for some time and discharged same day.

D.D.U. Hospital has been made Regional Blood Transfusion Centre

(West Zone) since September, 1994 which is catering to the needs of this hospital as well as other hospitals/private nursing homes of this area for their blood requirement. Blood bank is very efficient in its provision of blood for various blood groups for thalassemia patients regularly without much problems . Patient registered for regular blood transfusion gets initially his blood group antigen both major and minor blood group tested. It is one of the very few centers in Delhi which is providing blood group cross matching with minor antigens also. This prevents the formation of various antibodies on long term basis.

Thalassemia is a disease which requires not only blood transfusion on regular basis but also requires medication for reduction of iron levels. DDU Thalassemia Center has been providing drugs for chelation regularly in full doses . Both deferiprone and desferral are provided in sufficient doses from the hospital for free of cost. Patients are regularly monitored for their iron levels with serum ferritin levels which has been started in hospital. Patients are also helped for their requirements of pump with the help of hospital and associations like Thalassemics India. Patients are regularly monitored for side effects of the drugs. Indeed good chelation helps in improving life expectancy of these patients as observed by us.

Patients are kept under supervision and monitored with blood investigation regularly. Patients growth is monitored on every visit . Liver function test , kidney function test Thyroid function test are done from the hospital.

Tests for blood transfusion infections like HIV, HBsAg and HCV are also done regularly.

2 D Echo is also done on regular basis in the hospital to rule out cardiac involment.

Nearly 25 patients were found to have HCV antibody titers positive . With the coordination with G.B.Pant hospital gastroenterology department these patients were tested for HCV RNA titers . Six patients were found to be HCV RNA titers positive in high range. These patients have undergone liver biopsy and have been started on interferon and ribavarin combination therapy.

Patients are also being screened for hyperparathyroidism with the help of calcium, phosphorus, alkaline phosphate, parathyroid hormone levels and a dexa scan and treated accordingly .Patients in pubertal age group are also been investigated for various endocrine sex hormones and treated accordingly.

Treatment of thalassemia does not mean just treatment of disease but also prevention of disease spread in community. Parents are counseled regarding the disease and spread. Carrier status detection is carried out in the Hospital. DDU is one of the three Govt hospitals in Delhi in which HIGH PERFORMANCE LIQUID CHROMATOGRAPHY is done for carrier detection free of cost. Antenatal screening in pregnant women for Thalassemia is being carried out in the hospital. Antenatal diagnosis of the fotus is also done with the help of higher centers.

Thalassemia patients and parents are already burdened with multiple responsibility and we try our best not to load them with further financial burden.

Thalassemia care in DDU HospitalDr Ritu Chawla CMO NFSG, Incharge Thalassemia Unit , DDU Hospital.

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THALASSAEMIA UPDATE ______________________________________________ Health Care Services

Future prospectives.

We wish to continue providing comprehensive care for Thalassemia patients improving their life expectancy and quality of life. We wish to see our patients enjoying life with no complications and to expand

our services to whole of West Delhi. We wish to increase the strength of beds for our Thalassemia unit.

Acknowlegment

We hereby acknowledge Dr Aveender Prasad, M.S.,DDU HOSPITAL, for his

constant , enthusiastic support and guidance for our unit in DDU Hospital

We would sincerely like to thank Dr Mohini Kumar , Head of Department of Paediatrics, DDU , hospital for her constant inspiration, invaluable advice, suggestions and help for this unit.

The Thalassemia Unit at Apollo Hospital, Delhi was set up in 2003. Mrs Tuli, Secretary, Thalassemics India and Dr Anupam Sibal, Director Medical services at Apollo Hospital, Delhi were very keen that a service is available for thalassemic children at our hospital and on my joining Apollo Hospital, I was asked to organise the same.

With the help and support of our colleagues in the Blood Bank, Dr R. N. Makroo and Dr V. Raina, we started enrolling patients in 2003. A four-bedded area was set up. A subsidised package was devised to make an all-inclusive package that was much more affordable than the standard charges in the corporate sector. The management also helped us further by giving us discounts in investigations. But the most important help came from the Blood bank, which agreed to provide blood through the help of voluntary donation. As it is our hospital policy to not use blood from any other external source, and it is not really feasible for families of thalassemic children to provide donors regularly, the Blood bank took it upon themselves to arrange the blood supply and their support has been unwavering. However, it has meant being limited to a number so that we can sustain this service.

As a service, our biggest advantage is the quality of our blood in terms of safety. Since the inception of this hospital, all blood products have been screened for Hepatitis C in addition to Hepatitis B, much before it was legally required to do so. Two years ago, we moved to NAT testing

which is much more sensitive and further minimises the risk of acquired viral infections. The blood is also tested for Malaria antigen routinely.

Again for over eight years all blood was one log leucodepleted at the time of collection. However, as it is recommended for thalssemic children to receive three-log leuco-depleted blood, we were using bedside filters till last year. Since last year, all the blood products undergo three-log leucodepletion at the time of collection prior to storage, which is the ideal way for thalassemic patients. Now we do not use any bedside filters.

Over the last six years, we have had to move the area twice because of various renovations in the hospital and due to constant demand for more bed space. Yet, all patients have co-operated in our endeavour to provide a satisfactory service.

The annual testing, ongoing monitoring, counselling for future pregnancies all are a continuing process in all Thalassemia units and the same holds true for us. Every patient is on chelation. Dr Anju Virmani, Pediatric Endocrinologist provides the back up for endocrinology

workup of these children especially in terms of growth and pubertal development. Dr Anupam Sibal, Pediatric Gastroenterologist provides gastroenterology backup. In addition, every lady registering for the antenatal clinic at Apollo hospital is automatically screened for Thalassemia carrier state and services for Chorionic Villus Sampling (CVS) are available.

These are, however, the basics of any Thalassemia service. What we had hoped to achieve was that in addition to satisfactory hemoglobin and good overall health, each and every child with Thalassemia has confidence, a sense of pride and self-esteem and can think beyond Thalassemia. For all families coping with chronic disorders, very often their entire lives run around this disorder. What I had hoped to accomplish was to encourage families to take this disorder in their stride so that their children have a sense of normalcy in their lives and can achieve their true potential.

Every time one of our members shows me their report card with lots of stars in it or their trophy for being an all-rounder at school, I feel that we have achieved our goal.

I urge every child with Thalassemia and every family coping with this disorder to instill positivity and optimism in their home environment so that these children can truly bloom. For, the flower that blooms in adversity is the rarest and the most beautiful of all

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Thalassemia Unit at Apollo Hospital, DelhiDr. Amita Mahajan, Sr. Consultant Paediatric Oncology / Haematology

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THALASSAEMIA UPDATE __________________________________________________ Scientific Article

Introduction

Treatment of beta-thalassaemia major is based on regular blood transfusions to maintain the pre-transfusional Hb level above 9 gr/dl and appropriate chelation therapy to avoid the consequences of iron overload. The metabolically active iron catalyses the formation of free radicals, which damage membrane lipids leading to cell death and eventually organ failure. The endocrine glands are particularly vulnerable to the excess iron, so that the appearance of endocrine dysfunction in thalassaemia major (TM) is a common and disturbing complication, which requires prompt recognition and treatment. The contribution of the underlying molecular defect in TM to the development of endocrinopathies, and particularly hypogonatotrophic hypogonadism, is significant because the patients with the more severe defects have a greater rate of iron loading through higher red cell consumption (1).

The child with thalassaemia major has a particular growth pattern, which is relatively normal until age of 9–10 years; after this age a slowing down of growth velocity and a reduced or absent pubertal growth spurt are observed. The pathogenesis of growth failure is multifactorial (2), as shown below.

• Chronic anemia, hypersplenism, chronic liver disease (HBV, HCV)

• Zinc and folic acid deficiency, skeletal dysplasia,

• Desferrioxamine toxicity• Emotional disturbance• E n d o c r i n o p a t h i e s :

hypothyroidism, delayed puberty, hypogonadism,

• dysfunction of the growth hormone (GH) – IGF 1 axis

Growth Hormone (GH)

Chronic anaemia is no longer a factor

in well-treated children in developed countries. The response of growth hormone to stimulation tests has been found to be normal or reduced. The presence of neurosecretory dysfunction is supported by the impaired 24 hour GH secretion. Evidence for partial GH resistance is based on the fact that children with thalassaemia major have normal GH and GHBP, with low levels of IGF-1 and IGFBP-3, which are not always properly increased following GH stimulation. Moreover the therapeutic administration of GH did not fulfil all our expectations and often supraphysiological doses of GH are required to overcome this resistance and lead to an improvement in linear growth (3,4,5). The response to growth hormone treatment cannot be predicted based on known parameters such as growth velocity, age, height SDS, bone age SDS, IGF-1 levels and the type of abnormality in the GH-IGF-1 axis, suggesting that additional factors such as skeletal dysplasia and Desferrioxamine (DFX) toxicity are implicated in the child’s growth retardation.

Desferrioxamine (DFX) toxicity and body disproportion

Short stature with disproportionate body composition due to desferrioxamine toxicity has been observed. Desferrioxamine exhibits its toxic effect on growth by inhibiting DNA synthesis, fibroblast proliferation and collagen formation, causing flattening of the vertebral bodies (platyspondylosis) and eventually poor spine growth. Both sitting and standing heights are normal until the age of 6-9 years but gradually decreased in older ages with particular shortening of the sitting height as shown in figure 1(6). Body disproportion with short trunk has been reported in patients who have been poorly chelated during childhood and adolescence, so

that other contributing factors like haemosiderosis and deficiency of trace elements should influence spine growth (3). Sex steroid replacement therapy cannot adversely affect body disproportion, as truncal shortening at final height is evident in patients with either spontaneous or induced puberty (7). Body disproportion therefore is independent of pubertal or prepubertal period of greater height gain.

Figure 1: Standing height and sitting height is SDS in different age-groups 6.

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Management

Can children with thalassaemia major attain normal stature and develop normally with early and reasonable Desferrioxamine treatment? Although iron chelation can decrease the frequency of endocrinopathies, early DFX treatment may result in growth impairment. On the other hand poor compliance with DFX may eventually lead to severe iron burden, gonadal dysfunction and eventually growth failure (8). The benefits of treatment should be weighted against the potential adverse effects and the caring physician should balance between the efficacy and the injudicious use of Desferrioxamine. An ideal therapeutic regimen, which will avoid the toxic effects of iron overload and that of continuous subcutaneous chelation therapy, has yet to be found. It is therefore recommended that growth in both standing and sitting position should be assessed at 6-month intervals in order to detect early growth failure. Long-term observations on the effect of therapy are needed before this mysterious puzzle is solved.

Growth and Puberty in Thalassaemia Nicos Skordis, MD and Andreas Kyriakou, MD Pediatric Endocrine Unit, Makarios Hospital, Nicosia, Cyprus

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THALASSAEMIA UPDATE __________________________________________________ Scientific Article

Alternative oral chelation agents are often an option in cases of DFX toxicity, although some bone lesions remain irreversible.

• Prevention of growth retardation is essential.

• Monitoring growth in all children by using growth charts for both standing and sitting height is mandatory.

• The mean hemoglobin levels must be kept near to 9 gr/dl.

• Prompt initiation of iron chelation therapy prevents pituitary haemosiderosis, which is the main cause of growth hormone insufficiency.

• Treatment with growth hormone is recommended when GH deficiency is established. In poor responders such treatment should be discontinued. Therapeutic response with GH administration in cases with GH deficiency, is often non satisfactory.

• Growth acceleration is mostly promoted with sex steroids in children with pubertal delay.

• Sexual complications present a significant issue in thalassaemics. These include: delayed puberty, arrested puberty and hypogonadism. Transfusional haemosiderosis in the pituitary gonadotroph cell causes gonadotrophin deficiency, which is the underlying abnormality. Histological examination of the gonads shows minimal siderosis, so that the ovarian and testicular function is well preserved.

Protocol for investigation of poor growth in thalassaemic children

1. Measure current height both standing and sitting and plot on the growth chart. Calculate the target height based on parental heights. Compare with previous measurement to es t imate the growth velocity. Examine pubertal status. Note any physical disproportion. Review emotional and social status.

2. Assess bone maturation3. Routine blood tests including

l iver function tests, fer r it in, biochemical profile, and zinc

4. Urine analysis5. Thyroid function tests (Free T4,

TSH)6. IFG-1 and IGFBP-37. Stimulation tests to assess GH

secretion. At least two tests are required. Priming with sex steroids is necessary in boys older than 10 years with testosterone and in girls older than 9 years with Estrogens.

8. IGF-1 generation test in patients with low levels of IFG-1 and IFGBP-3 and normal GH secretion to exclude GH resistance.

Puberty

Puberty is the biological phenomenon, which results from the activation of the hypothalamic–pituitary–gonadal axis and is clinically manifested by the appearance of sexual characteristics. Delayed puberty is defined as the absence of any pubertal sign in girls (breast enlargement) and in boys (testicular enlargement) by the age of 13 and 14 years respectively. Delayed puberty in thalassaemia is almost always due to hypogonadotrophic hypogonadism, which still remains the most common endocrine and stressful complication in thalassaemia major (9) (figure 2). The association of hypogonadotrophic hypogonadism with the genotype has already been proven (1). An additional contributing factor, whose role is thought to be weak, is the impaired synthesis of leptin in thalassaemic patients which seems to be related to transferrin receptor levels (10).

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Figure 2. FSH (a) and LH levels (b) after GnRH stimulation in thalassaemic women with normal menstrual cycles, primary amenorrhea and secondary amenorrhea. X-axis: time in minutes. Y-axis: FSH and LH in miu/l 6

Arrested puberty is defined as the absence of further pubertal

progression –once puberty has started –for more than one year, where testicular volume in boys never exceeds 6 to 8 ml and breast size in girls remains unchanged. Failure of sexual development by the age of 15 to 16 years in both sexes is defined as hypogonadism. Secondary hypogonadism appears later in life, and is manifested in women as secondary amenorrhea and in men as decline in sexual drive and azzoospermia.

Adolescent girls with TM often present with primary amenorrhea and boys fail to become well virilized. The damage to the hypothalamus and pituitary is progressive, even when intensive chelating therapy is given and the appearance of hypogonadism in both sexes is often unavoidable. Most women with TM manifest secondary amenorrhea at some stage in their life and men develop hypogonadism in their 3rd decade after being normal for some years and even becoming fathers (11, 12, 13).

Protocol for investigation of pubertal disorder

The absence of any clinical pubertal signs in a boy (testicular enlargement) older that 14 years and in a girl (breast development) older than 13 years requires investigation.

1. Measure testosterone in the boy and oestradiol in the girl. DHEA-S in both sexes is often helpful

2. Perform the GnRH test to evaluate the pituitary capacity to secrete the gonadotropins FSH and LH, where the response in hypogonadism is low

3. Bone age is helpful for the treatment decision options

Therapeutic approach in delayed puberty should mimic biological and biochemical pubertal changes, aiming on promotion of linear growth as well (14,15,16,17).

Induction of puberty in boys can be achieved with testosterone depot IM 25-50mg monthly for 6 months

(Continued on page 16..................)

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Invited FacultyDr. 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

PatronsMr. Panos Englezos - President, Thalassaemia International FederationDr. B.K. Rao - Chairman Board of Management, Sir Ganga Ram Hospital

Dr. Anupam Sibal - Group Medical Director, Apollo Hospitals Group

T.I.F. Advisory CommitteeDr. Androulla Eleftheriou - Executive Director, TIF

Dawn Adler - Board Member, TIFGeorge Constantinou - Secretary, TIF

Gargi Pahuja - Board Member, TIFMrs. Fatemah Hashemi - Board Member, TIF

National Advisory CommitteeDr. M. B. Agarwal

Dr. Manorama BhargavaDr. Sharmila Chandra

Dr. V.P. ChoudharyDr. Sunita Mittal

Dr. I.C. Verma

For registration or any other information contact:

Shobha TuliSecretary, Organising Committee

M: 9810019950

4th InternatIonal ConferenCe on thalassemIa31st october - 1st november, 2009

new Delhi, India

Conference secretariat:

Thalassemics India A-9, Nizamuddin West, New Delhi-110013, India

Tel. (O) : 41827334, Fax : 24353871E-mail: thalcind@yahoo.co.in

Website: www.thalassemicsindia.org

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THALASSAEMIA UPDATE ________________________________________________________ Activities

May 8th, The World Thalassemia Day was celebrated with great fervour this year at Sri Sathya Sai International Centre with around 600 Thalassemia children, parents, doctors, supporters and well wishers.

Dr. Kanwar Sain, Mayor of Delhi was the Chief Guest. Ms. Shovana Narain and Ms Anjana Kuthalia were the Guests of Honour.

Before the cultural programme a short one hour session was conducted on Asunra by Dr. V.K.Khanna, Head of Thalassemia Department. SGRH and Dr. Sunil Goomber Head of Thalassemia Department , G.T.B Hospital. Parents and patients cleared their doubts about the usage and effects of the new drug.

The cultural programme began with the lamp lighting and vandana sung by Shivangi Amrit.

Thalassemia children from Faridabad

performed beautifully on “Des Mera Rangeela”. A group of 14 thalassemics from Delhi gave a vibrant medley performance. Tushar Arora an upcoming singer was applauded by one and all.

Mrs. Shobha Tuli, Secretary Thalassemics India, welcomed the Chief Guest and invited dignitaries. She talked about thalassemia scenario in Delhi and requested the Mayor to get all the thalassemics of Delhi registered, give space for awareness at specific sites and open Thalassemia Centers at all the new upcoming hospitals having blood bank facilities.

Dr. Kanwar Sain praised the performance by thalassemia children. He very kindly felicitated some of the young thalassemics who had excelled in sports, academics and extra curricular activities. He appreciated the work of Thalassemics India. He agreed to extend all possible support

to the society.

Ms. Shovana Narain encouraged the children, their families and the society. She offered all possible help in her capacity. Ms Anjana Kuthalia also promised all possible help for our children.

The cultural programme continued with Salaam Balaak Theater Group performance.

Later the audience enjoyed the performance by Sanjay Raina, Vandana Vadhera, Garima and Guitarists from Amit Upadhyay Guitar School.

All the thalassemia children were given back gifts.

Thalassemics India is thankful to Torrent Pharmaceuticals Ltd for sponsoring the May 8th function. We are also grateful to our well wishers and friends - AOC Monitors, Pepsi, SDIPA and others.

World Thalassemia Day, 8th May, 2009

We were approached by thalassemia children and their parents expressing their desire to see Akshardham Temple. Thalassemics India decided to take a group of 221 people to Akshardham Temple.

After the security check we all proceeded towards a huge hall where our group of 221 people was divided into 2 groups and were allocated 2 guides as group leaders. After the exhibition all the children and the parents were taken for lunch in the Cafetaria

where delicious lunch was served with great affection as the prasadam.

Children and parents seemed very excited and full of enthusiasm. Exhibition of the Akshardham temple was divided in three phases.

1st Phase was Sahajanand Darshan or the Hall of Values - here the values for the mankind are presented though audio-animatronics and light and sound diorama presentations from life of Bhagwan Swaminarayam.2nd Phase was the Neelkanth Darsham

or the Gaint Screen Film that vividly potrays Neelkaanth's epic pilgrimage of India. 3rd Phase was the Sankriti Vihar. The most fascinating boat ride experiences India's glorious Heritage.

Darshan at Akshardham was truely a breathtaking experience as the Indian culture in all its grandeur beauty, wisdom and bliss was depicted and all the people took beautiful spiritual messages along with them at their homes.

Special Darshan to Akshardham Temple

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THALASSAEMIA UPDATE ____________________________________________________New Members

Torrent Pharmaceuticals Ltd. Rs.3,75,000.00

Trent Co. Rs.47,000.00

Surya Foundation Rs.30,000.00

Ashwani Kumars Rs. 45,000.00

Anita Mahajan Rs.25,000.00

Brindavan Agro Industries Rs.25,000.00

Rajarshri Guha Rs.24,930.00

P&G Enterprises Pvt. Ltd. Rs.16,188.00

Sonal Gupta Rs.12,800.00

Rita Jain Rs.6,620.00

Alka Nath Rs.6,620.00

Uma Ashok Bansal Rs.6,000.00

Educare Rs.6,000.00

Shobha Tuli Rs.5,000.00

Sunita S. Singh Rs.6,80.00

Saroj Choudhary Rs.5,00.00

Our Worthy Donors

M.L.Ahuja Delhi

Manish Bhai Patel Ahmedabad

Shafique Ahmed Khan Ahmedabad

Malay Paul West Bengal

Vikas Jain Ghaziabad

Aseem Jain Delhi

WE WELCOME THE FOLLOWING NEW MEMBERS

Subhash Khanna Haryana

Vikas Takral Girsa

Susanta Sahu Kolkata

Pramod Anandrao Maharashtra

Mayank M. Patel Guajarat

Pyari Praneeta Mittal secured 73% in Class XII Commerce.

Nanhi Niyati secured 76.4% in Class X Boards, 09.

Daring Dipin secured 68% in Class XII Boards despite all odds.

Brave & Beautiful Bitosta Bit topped humanities stream in her school by

securing 81% in class XII.

Our Shining Stars

We at Thalassemics India are proud of you and wish you all the success in life

Would you like to help those who are in need of Infusion Pumps?

There are many thalassemic patients who are in need of Infusion Pumps. At the moment they are either sharing their pumps with others or they are forced to take the treatment in the hospital.

Your donation will be duly acknowledged by us.

ACKNOWLEDGEMENT

Thalassemics India would like to thank all those who have donated desferal injections to the society. With your help

we have been in a position to help some of our thalassemia children in need of chelation.

Thanks once again !

Change of Address Please inform Thalassemics India if there is any change in your postal address.

Thalassemics India A-9, Nizamuddin West, New Delhi-13

email:- thalcind@yahoo.co.in

Lamp Lighting

Ms. Shovana Narain (Guest of Honour) lauding the efforts of our children

“Desh Mera Rangeela” by thalassemics from Foundation against Thalassemia Society

Dr. V.K. Khanna and Dr. Sunil Gomber taking questions from the parents

World Thalassemia Day Celebrations

Vandana sung by Shivangi Amrit

Performance by Salam Balak Trust troupe

Ms. Anjana Kuthalia (former Ms India) promisingall possible help

Children enjoying the evening withMs. Vandana Vadhera

Children on the stage dancing and enjoying.

World Thalassemia Day Celebrations

Tushar Arora Singing on the tunes of Guitarist from Amit Upadhyay school of Guitar

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Towards Awareness

Talk on Thalassemia

Dr.V.K. Khanna addressed 60 students of class XIth at Air Force Bal Bharti School, Lodhi Road. The talk was followed by Q/A session, Vice Principal Mrs. Malhotra proposed a vote of Thanks.

Talk on Thalassemia

Mrs. Shobha Tuli gave a talk on awareness and prevention of Thalassemia to the students of Air Force

Sr. Sec. School, Old Willington Camp. Mrs. Menon Principal appreciated 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

THALASSAEMIA UPDATE ______________________________________________________ Awareness

Gupta, Inner wheel club Delhi Main.

Blood Collection Drive

Blood Donation Camps organized by Thalassemics IndiaVenue Units CollectedPepsi Co, Gurgaon 30Huges, Gurgaon 51Nbrc, Gurgaon 36Jass, Gurgaon 56Oracle, Gurgaon 29Airforce Station, Hindon 70Airforce Station Basant Nagar 29Airforce Station Subroto Park 21Airforce Station Rajokari 25Airforce Station , Aya Nagar 38Airforce Station, Palam 33

Blood Donation Camps Supported by Thalassemics IndiaVenue Units CollectedDwarka 43Janak Puri 49South Ext. 226

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and reassessment. Pubic hair will appear and penile size will increase. Increase in testicular volume indicates activation of the axis and release of gonadotrophins (FSH and LH), where no further treatment is needed except for close observation. In case where testicular size is unchanged, then treatment is continued for 6 months and subsequently the dose is increased to 100mg monthly for one year. Therapeutic schedule is determined by the growth potential, clinical response and emotional factors. For testicular enlargement, the therapeutic regime is altered to the combination of hCG and hMG, both of which mimic the pituitary gonadotrophins. The final adult dose is testosterone depot 50mg/weekly IM or alternatively transdermally in patches 5 mg/daily. The oral route (testosterone undeconate) should be avoided due to liver toxicity.

For induction of puberty in girls oral ethinylestradiol is preferred at the dose 100ng/kg/day for 6 months, where increase in breast size and growth acceleration is noted. This dose is continued for additional 6 months and increased to 200ng/kg/day for the subsequent year. Therapeutic schedule is determined by the same factors as in boys. The adult dose is 400ng/kg/day, where the uterine size is satisfactorily increased for the induction of menarche. Induction of puberty can be successfully achieved by the transdermal use of estrogens.

Menarche is achieved by the addition of Medroxyprogesterone 10 mg/day for 10 days when the size of the uterus exceeds 5cm. When menstrual bleeding occurs spontaneously during estrogen treatment, the regime should be adjusted. For maintenance of the menstrual cycle the use of estrogens (Conjugated Estrogens 0.625 ή 1.25 mg, Ethinyl Estradiol 20 μg) from day 1st to 25th and Progesterone from day 14th to 25th is required. The transdermal use of Estradiol and Norethisterone is advantageous due to decreased liver toxicity.

Prompt chelation therapy before pubertal age and before extremely high levels of ferritin are reached is the fundamental tool to help children with thalassaemia major to attain normal stature and sexual maturity and to improve their bone mass. Poor pubertal growth in adolescents with thalassaemia major does not solely depend on gonadal failure. No difference has been observed in pubertal growth and final height between treated hypogonadal patients compared to those with spontaneous puberty (18,19). Failure to progress normally through puberty is associated with failure of adequate bone mineralization and achievement of peak bone mass, which is a contributing factor to the ultimate bone disease in thalassaemia (20).

References

1. Skordis N, Michaelidou M, Savva SC, Ioannou Y, Rousounides

A , K leanthous M, Skordos G, Christou S. The impact of Geno t ype on Endoc r i ne complications in Thalassaemia major. Eur J Hematol 2006

2. Skordis N. The growing child with Thalassaemia. J Pediatr Endocrinol Metab 2006; 19: 467-9.

3. De Sanct is V. Growth and puberty and its management in Thalassaemia. Horm Res 2002; 58(S1): 72-79

4. Raiola G, Galati MC, De Sanctis V, Caruso-Nicoletti M, Pintor C, De Simone M, Arcuri VM, Anastasi S. Growth and puberty in Thalassaemia major. J Pediatr Endocrinol Metab 2003; 16: 259-266

5. De Sanctis V, Urso L. Clinical expe r i ence w i t h G rowth Hormone treatment in patients with beta-Thalassaemia major. Bio Drugs 1999; 11:79-85

6. Toumba M, Sergis A, Kanaris C , S ko rd i s N . Endoc r i ne complications in patients with Thalassaemia major. Pediatric Endocrine Reviews 2007;5:642-648

7. Filosa A, Di Maio S, Baron I, Esposito G, Galati MG. Final

height and body disproportion in Thalassaemic boys and girls with spontaneous or induced puberty. Acta Paediatr 2000; 89:1295-1301

8. De Sanctis V, Roos M, Gasser T, For tini M, Raiola G, Pintor C. Impact of long-term iron chelation therapy on growth and endocrine functions in Thalassaemia. J Pediatr Endocrinol Metab 2006;

9. I t a l i an Work ing Group on Endocrine Complications in Non-Endocrine Diseases. Multicentre study on prevalence of endocrine complications in Thalassaemia major. Clin Endocrinol 1995; 42: 581-586.

10. Dedousis GVZ, Kyrtsonis MC, Andrikopoulos NE, Voskaridou E, Loutradis A. Inverse correlation of plasma leptin and soluble transferrin receptor levels in β-thalassaemia patients. Ann Hematol 2002; 81: 543-547.

11. De Sanctis V, Vullo C, Katz M, Wonke B, Tanaw R , Bagni B. Gonadal function in patients with B–Thalassaemia Major. J Clin Pathol 1988; 41:133-137.

12. Skordis N, Gourni M, Kanaris C, Toumba M, Kleanthous M, Karatzia N, Pavlides N, Angastiniotis M. The impact of iron overload and genotype on gonadal function in women with Thalassaemia major. Ped Endocrinol Rev 2004; 2(S2): 292-295

13. Skordis N, Petrikkos L, Toumba M, Simamonian K, Hadjigavriel M, Sitarou M, Kolnakou A, Skordos G, Pangalou E, Christou S. Update on fertility in Thalassaemia major Pediatric Endocrine Reviews. 2004; (S2):296-302

14. Pozo J, Argente J. Ascertainment and treatment of delayed puberty Horm Res. 2003; 60 (S3):35-48

15. Mac Gill ivray MH. Induction of puber ty in Hypogonadal children. J Pediatr Endocrinol Metab 2004;17(S4):1277-1287

16. Ca r u so –N ico le t t i M , De Sanctis V, Cavallo L, Raiola G, Ruggiero L,Skordis N, Wonke B. Manegement of puberty for

(...............Continued from page 7)

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optimal Auxological results. J Pedia t r Endocr ino l Metab 2001;14(S2):939-944

17. De Sanctis V, Vullo C, Katz M, Wonke B, Nannetti C, Bagni B. Induction of spermatogenesis in Thalassaemia. Fertility Sterility 1998; 50: 969-975.

18. Caruso-Nicoletti M, De Sanctis V, Raiola G, Skordis N, Manusco M, Coco M, Wonke B. No difference in pubertal growth and final height

between treated Hypogonadal a n d n o n - H y p o g o n a d a l Thalassaemic patients. Horm Res 2004;62:17-22

19. Filosa A, Di Maio S, Lamba M, Baron I, Saviano A, Esposito G. Bone age progression during five years of substitutive therapy for the induction of puberty in Thalassaemic girls-effects on height and sitting height J Pediatr Endocrinol Metab 1999;12:525-

53020. Bielinski BK, Darbyshire PJ, Mathers

L, Crabtree NJ, Kirk JM, Stirling HF, Shaw NJ. Impact of disordered puberty on bone density in beta-Thalassaemia major. Br J Haematol. 2003; 120:353-358

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.

Readers’ Queries

Answered by Dr. V.K Khanna (Head, Thalassaemia Unit, SgRH)

Ackowledgement "According to TIF Magazine, the

official newsletter of the Thalassemia International Federation."

TIF Board meeting in progress

TIF Board Meeting was held in Damascus, Syria. Out of the18 board members, 11 members were present.

The minutes of the last board meeting and the minutes of the last general Assembly were approved. TIF N

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Dr. Nica Cappellini, Mrs. Fatemah Hashemi & Shobha Tuli in Syria

Audited accounts and management accounts for 2008 were presented and approved. Annual TIF report, Involvement of TIF in health related policies, fund raising, regional priorities, MSC fellowship course. hosting of next TIF conference and a number of other issues were discussed.

TIF website www.thalassaemia.org.cy

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THALASSAEMIA UPDATE ________________________________________________ News Across India

Jamshedpur

“Dr. S.P Foundation” successfully observed “International Thalassaemia Day” at “Hotel Boulevard” Bistupur, Jamshedpur.

Chief Guest, Mr. Saryoo Rai, M.L.A, 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 was read out by Madhumita. Then Dr. T.K. Chatterjee, Director of Dr. S.P Foundation elaborately explained about Thalassemia and its genetic inheritance.

Dr. Chatterjee said that every Thalassemic should be monitored properly for their better health care. He said “Undetected Cases” are much more than “Detected Cases” of Thalassemia. So he appealed the State Govt. to provide an H.P.L.C. Machine for Mass Screening and data based prevalence. Dr. Chatterjee said that Jharkhand State is a Malaria Prone area so here Thalassemia may be in higher count. He informed that free Thalassaemia Help Line 0657-2425357 and Thalassemia Care Unit for counselling is gaining popularity. In this occasion Mr. G.M. Sharan, Rony D’ Costa also delivered their speech.

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Bangalore

On the occasion of World Thalassemia Day on May 8th, 2009 public awareness and education program on Thalassemia was organized through Jai Vigyan mission project Phase II at St. John’s Medical College hospital. The theme of the day was “Together we Care” and efforts of Dr. Cecil Ross and Dr. Karuna Rameshkumar to organize the programme were ably supported by staff from laboratory, blood bank and medical social work department and faculty from general medicine and pediatrics and project staff.

On May 8th, the programme started with a prayer at 2.30 pm. Thalassemia children who are being treated in St. John’s medical college hospital welcomed the executives. II MBBS students performed a skit which highlighted the clinical features of Thalassemia and a take home message on inheritance. Pamphlets about inheritance were distributed.

Dr. Sitalakshmi from the laboratory, Dr. Cecil Ross from general medicine and Dr. Indumathy, Dr. Ranjani and Dr. Anand from Pediatrics participated

in the program and answered the questions raised by the patients and their parents.

Dr. Karuna Rameshkumar, thanked the management for their constant support and appreciated the involvement of faculty, staff and the industry.

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Ludhiana

To mark the World Thalassemia Day, Dayanand Medical College & Hospital, Ludhiana held a medicos voluntary blood donation camp on

8th May, 2009. the Students, Interns and consultants donated 207 units of blood at this camp.

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Kerala

Thalassaemia day was observed in the auspicious of Blood patients protection council and Malabar THAS society .In this part a population blood screening programme, Hepetitis.B vaccination programme free distribution of medicines, Haematology medical camp, pamphlet distribution were organized. Eminent youth leader of Kerala and the member of Legislative Assembly Adv. A.Pradeep Kumar MLA inaugurated the function. Giving his own blood sample the inauguration of population blood screening

NEWS ACROSS INDIA

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THALASSAEMIA UPDATE ________________________________________________ News Across India

programme were also inaugurated by Mr. Pradeep Kumar MLA. In his speech Mr.Pradeep Kumar stated that all organizations and NGOs should 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.

M. Sudeendra Kumar president, Press Club, Calicut, distributed the medicines. Noted eye specialist 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 Blood Patients Protection Council and the secretary of Malabar THAS society gave the key note address.

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Ajmer

Ajmer Region Thalassemia welfare Society (regd) collected 281 units of blood on 12th April,2009 at a blood Donation Camp organized at Zonal blood Bank, Jawahar Lal Nehru

Chikitsalay Ajmer.

Ajmer Region Thalassemia welfare Society (regd) celebrated World Thalassemia Day on 8th May,2009 at city water Park.

85 thalassemic children celebrated World Thalassemia Day by cake cutting.

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Gujarat

Seminar on Thalassemia Organized by Indian Red Cross Society, Vadodara at K.G. Children Hospital, Vadodara, Gujarat.

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Kolkata

2nd workshop on Thalassaemia was organized by the Hematology Foundation, Kolkata.

(...............Continued from page 20)

Thank You !We are thankful to all those who have contributed in this issue of

Thalassaemia Update.

The conference was well attended by nearly 300 delegates comprising of Physicians, Patients and Parents.

Prof. Ratna Chatterjee from U.K, Prof. S. Fucharoen from Thailand, Prof. V.P Chodhury from Delhi , Prof. M.B Agarwal from Mumbai, Prof. N. Marwah from Chandigarh, Prof. G. Choudhury from Lucknow, Prof. Dr. Andrew Wu from Singapore were the main speakers at the conference.

Mrs. Shobha Tuli and Retired Chief Justice, Mr. Chittotosh Mukherjee had the honour to open the conference.

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The Editorial Committee reserves the right to change the text of the articles sent for publication where necessary, in good faith.

The Editorial Committee or Thalassemics India do not accept any responsibility for any inaccuracies or omissions.

The views expressed are not necessarily that of Thalassemics India.

Reproduction of the material published in Thalassaemia Update for Educational purpose is encouraged, provided it is accompanied by the following “According to Thalassaemia Update, the official newsletter of Thalassemics India”.

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