1. Thalassaemia Issue 34 • July 2009
4th 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 & Puberty
Thalassemics India Sir Ganga Ram Hospital Thalassaemia International Federation
Blood Donation Camps
Readers’ Queries
www.thalassemicsindia.org

2. 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 ffff
marathon to help raise funds for three
thalassemia children on 5th April 2009. Aarti Zaveri donated one of her
Corporate Gurgaon was involved by artworks to Thalassemics India on 8th
Genesis 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 also
organized 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 events
helped in saving the lives of three
to
thalasssemia children in need of Bone
Marrow Transplant.
You All
ffff ffff

3. aemia
SECRETARY’s Message
Thalassemics India Dear Friends
A-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
3
www.thalassemicsindia.org

4. 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

5. THALASSAEMIA UPDATE_______________________________________________ Health Care Services
Future 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 Hospital
We wish to continue providing beds for our Thalassemia unit.
comprehensive care for Thalassemia We would sincerely like to thank Dr
THALASSAEMIA UPDATE
patients improving their life Acknowlegment Mohini Kumar , Head of Department
expectancy and quality of life. We of Paediatrics, DDU , hospital for her
wish 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, Delhi
Dr. Amita Mahajan, Sr. Consultant Paediatric Oncology / Haematology
The Thalassemia Unit at Apollo which is much more sensitive and workup of these children especially
Hospital, Delhi was set up in 2003. further minimises the risk of acquired in terms of growth and pubertal
Mrs 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 provides
Medical services at Apollo Hospital, gastroenterology backup. In
Delhi were very keen that a service Again for over eight years all blood addition, every lady registering
is available for thalassemic children at was one log leucodepleted at the for the antenatal clinic at Apollo
our hospital and on my joining Apollo time of collection. However, as hospital is automatically screened for
Hospital, I was asked to organise the it is recommended for thalssemic Thalassemia carrier state and services
same. 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 last
colleagues in the Blood Bank, Dr year, all the blood products undergo These are, however, the basics of any
R. N. Makroo and Dr V. Raina, we three-log leucodepletion at the time Thalassemia service. What we had
started enrolling patients in 2003. of collection prior to storage, which is hoped to achieve was that in addition
A four-bedded area was set up. A the ideal way for thalassemic patients. to satisfactory hemoglobin and good
subsidised package was devised to Now we do not use any bedside overall health, each and every child
make an all-inclusive package that filters. with Thalassemia has confidence,
was much more affordable than the a sense of pride and self-esteem
standard charges in the corporate and can think beyond Thalassemia.
sector. The management also helped For all families coping with chronic
us further by giving us discounts in disorders, very often their entire
investigations. But the most important lives run around this disorder. What
help came from the Blood bank, which I had hoped to accomplish was
agreed to provide blood through the to encourage families to take this
help of voluntary donation. As it is disorder in their stride so that their
our hospital policy to not use blood children have a sense of normalcy in
from any other external source, and their lives and can achieve their true
it is not really feasible for families potential.
of thalassemic children to provide Over the last six years, we have had
donors regularly, the Blood bank took to move the area twice because of Every time one of our members
it upon themselves to arrange the various renovations in the hospital shows me their report card with lots
blood supply and their support has and due to constant demand for of stars in it or their trophy for being
been unwavering. However, it has more bed space. Yet, all patients an all-rounder at school, I feel that we
meant 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 and
As a service, our biggest advantage The annual testing, ongoing every family coping with this disorder
is the quality of our blood in terms monitoring, counselling for future to instill positivity and optimism in
of safety. Since the inception of this pregnancies all are a continuing their home environment so that these
hospital, all blood products have process in all Thalassemia units and the children can truly bloom. For, the
been screened for Hepatitis C in same holds true for us. Every patient flower that blooms in adversity is the
addition to Hepatitis B, much before is on chelation. Dr Anju Virmani, rarest and the most beautiful of all
it was legally required to do so. Two Pediatric Endocrinologist provides
ffff
years ago, we moved to NAT testing the back up for endocrinology
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www.thalassemicsindia.org

6. THALASSAEMIA UPDATE___________________________________________________ Scientific Article
Growth and Puberty in Thalassaemia
Nicos Skordis, MD and Andreas Kyriakou, MD
Pediatric Endocrine Unit, Makarios Hospital, Nicosia, Cyprus
THALASSAEMIA 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

7. THALASSAEMIA UPDATE___________________________________________________ Scientific Article
Alternative oral chelation agents liver function tests, fer ritin, progression –once puberty has
are 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 UPDATE
remain 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 and
Protocol for investigation of poor receptor levels (10). LH, where the response in
growth in thalassaemic children hypogonadism is low
30
1. 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 depot
2. Assess bone maturation IM 25-50mg monthly for 6 months
Arrested puberty is defined as
3. Routine blood tests including the absence of further pubertal (Continued on page 16..................)
7
www.thalassemicsindia.org

8. 4th International Conference on Thalassemia
31st October - 1st November, 2009
New Delhi, India
THALASSAEMIA 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

9. THALASSAEMIA UPDATE_________________________________________________________ Activities
World Thalassemia Day, 8th May, 2009
THALASSAEMIA UPDATE
May 8th, The World Thalassemia Day performed beautifully on “Des to the society.
was celebrated with great fervour Mera Rangeela”. A group of 14
this year at Sri Sathya Sai International thalassemics from Delhi gave a vibrant Ms. Shovana Narain encouraged
Centre with around 600 Thalassemia medley performance. Tushar Arora an the children, their families and the
children, parents, doctors, supporters upcoming singer was applauded by society. She offered all possible help
and well wishers. one and all. in her capacity. Ms Anjana Kuthalia
also promised all possible help for
Dr. Kanwar Sain, Mayor of Delhi Mrs. Shobha Tuli, Secretary our children.
was the Chief Guest. Ms. Shovana Thalassemics India, welcomed the
Narain and Ms Anjana Kuthalia were Chief Guest and invited dignitaries. She The cultural programme continued
the 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, Vandana
Asunra by Dr. V.K.Khanna, Head of sites and open Thalassemia Centers
Thalassemia 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 were
and 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 for
The cultural programme began with in sports, academics and extra sponsoring the May 8th function. We
the lamp lighting and vandana sung curricular activities. He appreciated are also grateful to our well wishers
by 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 Temple
We were approached by where delicious lunch was served or the Gaint Screen Film that
thalassemia children and their with great affection as the vividly potrays Neelkaanth's epic
parents expressing their desire prasadam. pilgrimage of India. 3rd Phase
to see Akshardham Temple. was the Sankriti Vihar. The most
Thalassemics India decided to Children and parents seemed very fascinating boat ride experiences
take a group of 221 people to excited and full of enthusiasm. India's glorious Heritage.
Akshardham Temple. Exhibition of the Akshardham
temple was divided in three phases. Darshan at Akshardham was
After the security check we all truely a breathtaking experience
proceeded towards a huge hall 1st Phase was Sahajanand as the Indian culture in all its
where our group of 221 people Darshan or the Hall of Values - grandeur beauty, wisdom and bliss
was divided into 2 groups and here the values for the mankind was depicted and all the people
were allocated 2 guides as group are presented though audio- took beautiful spiritual messages
leaders. After the exhibition all animatronics and light and sound along with them at their homes.
the children and the parents were diorama presentations from life
taken for lunch in the Cafetaria of Bhagwan Swaminarayam.2nd
Phase was the Neelkanth Darsham
9
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10. 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
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10

12. World Thala
Celebra
Lamp Lighting
Ms. Shovana Narain (Guest of Honour)
lauding the efforts of our children
Dr. 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

13. assemia Day
ations
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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15. THALASSAEMIA UPDATE_______________________________________________________ Awareness
Towards Awareness
THALASSAEMIA UPDATE
Talk on Thalassemia Gupta, Inner wheel club Delhi Main.
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
Sr. Sec. School, Old Willington Camp.
awareness and prevention of
Mrs. Menon Principal appreciated
Thalassemia 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
15
www.thalassemicsindia.org

16. 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 induced
THALASSAEMIA 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

17. 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."
aemia
TIF 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

20. THALASSAEMIA UPDATE_________________________________________________ News Across India
NEWS ACROSS INDIA
THALASSAEMIA 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

21. THALASSAEMIA UPDATE
®
21
www.thalassemicsindia.org

22. 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 World
THALASSAEMIA 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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