The Juice on Wheatgrass & thalassaemia

There has been recently much discussion on the potential benefits of wheat grass juice in relation to thalassaemia. The interest in wheatgrass juice has been instigated by a pilot study from India reporting significant benefit in some patients (RK Marwaha et al.Wheat grass juice reduces transfusion requirement in patients with b-thalassemia major: a pilot study. Indian Pediatrics 2004, in press). The original article should hopefully be published very soon. For anybody interested to look at the original article, it should become available through the website of the Indian Pediatrics journal (http://www.indianpediatrics.net/), as soon as it is published.

The study has involved 16 patients who satisfied the strict requirements of taking 100 ml of wheat grass extract every day for at least 18 months. Patients were monitored only by measuring the frequency and amount of blood transfused. In the course of the study 50% of the patients showed a reduction in blood consumption, with 3 of them showing a much stronger benefit. However, it is important to emphasise that all patients continue to be transfusion dependent. Unfortunately the paper does not include any information on the types of mutations found in these patients, or their iron chelation or whether the reduced consumption of blood was accompanied by an increase in fetal hemoglobin.

These are all important questions that need to be carefully documented, in order to helpus understand the mechanism by which some patients are deriving benefit and others are not responding. Prompted by this study, we hypothesised that wheat grass juice might be exerting its beneficial effect by increasing the production of fetal hemoglobin.

To address this question, we have recently examined in our lab whether wheat grass concentrate can increase the production of fetal hemoglobin. The method of our assay was published earlier this year (Human Molec Genet 13 (2004) 223-233. Vadolas J,Wardan H, Orford M, Williamson R and Ioannou PA. Stable cellular genomic reporter assays for screening and evaluation of inducers of fetal hemoglobin) and depends on using a sensitive fluorescent protein to detect production of fetal hemoglobin in human erythroleukaemic cell cultures.

The wheat grass concentrate we have used is of course prepared in a very different way from the wheat grass juice that was taken by patients in India. Nonetheless, we were very encouraged to see in our preliminary studies that wheatgrass concentrate has a significant effect on fetal hemoglobin production. I cannot say much more at this stage, except that we are planning to extend our studies to hopefully identify the factor(s) responsible for this induction.

I understand that wheat grass extract is already being promoted as an elixir for many diseases without any solid scientific evidence, so I want to caution people from rushing to buy it from natural product stores as an elixir for thalassaemia. We have no information which patients can respond to it and which patients may not benefit from it. Similarly, it is not possible to predict from the Indian study what dosage of wheat grass extract or concentrate may be most effective for those patients that could benefit from it.

Further studies are clearly necessary and warranted by these initial observations. It is only through careful and systematic studies that we will eventually get an effective therapy for this disease and not by patients experimenting individually without proper assessment.

In the meantime, we are also interested to examine any other natural products which may have beneficial effects on thalassaemia. We would therefore be pleased to hear from any patient who may have "experimented" with other natural products and noted a significant difference in their transfusion requirements.

Panos Ioannou (VIC)
Head, Cell & Gene Therapy
Research Group
Murdoch Childrens
Research Institute
Flemington Road,
VIC 3052Australia

ATA Objectives

The objects for which the Association is established are:

*To establish coordination between members with regard to interchange of ideas, diagnostic techniques, treatment and other matters pertaining to thalassaemia and related conditions (haemoglobinopathies).

*To liaise with the Australian Government, other bodies and institutions on any National issue of relevance to haemoglobinopathies.

*To liaise with international organisations and groups and to coordinate Australian representation to such bodies.

*To promote education, welfare and scientific endeavors in the field of haemoglobinopathies.

President

Dear Friends,

It is my pleasure to welcome you to our first National Newsletter for the Australian Thalassaemia Association. The National newsletter will be a platform for Health professionals,mscientists, patients, families and friends to share their experiences and intellect.

The aim of the newsletter is to generate public awareness and provide national and international information on Thalassaemia and the Haemoglobinapathies to the individual, those close to them and the general public. This will be achieved through State updates, lifestyle stories, scientific research, government information, diet and nutrition.

There is also a less formal fun page for children. The newsletter is structured to provide a holistic approach about thalassaemia and the
haemoglobinapthies to the general public especially. It then follows with information about recent developments that may be of more immediate interest to patients, families and friends.

I invite you to send me your input about what you would like to see in the next newsletter

Josie Stockden ATA Vice President

ATA Executive

The responsibilities of the Australian Thalassaemia Association (ATA) are rotated every two years between the State organisations.

New position holders are as follows:

The roles of the Committee of Management for the ATA 2003 to 2005

The ATA Committee includes:
President: Martha Gerolemou - NSW
Vice President: Josie Stockden - WA
Secretary: Maria Avarantinos - NSW
Treasurer: Carol Fotheringham - NSW
Councillor: Isabella Agius - QLD
Councillor: Maria Kastoras - VIC
Public Officer: Boyd Webster - NSW

ATA Thalassaemia International
Federation Representative: Maria Avarantinos - NSW

Delegates from each state organisation are also encouraged to attend ATA meetings.

Following are ATA subcommittees with the following coordinators:
Website subcommittee - Isabella
Newsletter subcommittee - Isabella
Conference/AGM organising subcommittee - yet to be assigned
Funding/fundraising subcommittee - Maria
Constitution subcommittee - yet to be assigned
Health professional relations subcommittee - Maria
Marketing subcommittee - yet to be assigned

International Thalassaemia Day May 8th

Operation Code Red - Proposal

AIM:
To incress awareness of thalassaemia nationally. By releasing 2000 red balloons at the same timein all major capital cities around Australia, along city river banks.

RESULT:
Gain national exposure and raise much needed awareness.

WHEN:
May 8th International Thalassaemia day 2005

TIME:
12 midday in each state

LOCATION:
Your capital city river eg Brisbane river, Melb-south bank, Major capital city, easy acess point and likely to receive the best media exposure

RATIONALE:
The river location to release the balloons, was chossen beause part of the word thal in greek means water. In greek the words thalasseamia means Water and blood so you see water blood make the word thalssaemia. So you see the significance of your local river. The red balloons symbolise the blood.

Sotirios - living with thalassaemia

‘I was born in Melbourne, to Greek Australian parents. Part of my heritage included thalassaemia. Although it was not diagnosed at the time of my birth, my mother had heard of thalassaemia in Greece. Later, when I was appearing rather ill and anaemic as a four year old, it was diagnosed at the hospital.

‘An illness that made me feel very tired as a child, unable to walk long distances or to play with the energy of other children soon provided other ‘nightmares’ as part of the treatment. Around five years old, I began having regular blood transfusions at hospital. These varied from fortnightly to eight weekly. My haemoglobin level was around six before I began transfusions. After I was initiated into the transfusion regime, doctors aimed to maintain my haemoglobin level at or above nine prior to transfusion. Due to the lack of volume of blood in my body and the weak development of my veins, transfusions were particularly traumatic for me as a child. The painful search for a suitable vein to put the drip into and the stays in hospital became part of the experience of living with thalassaemia.

‘Around seven years old, I had my spleen removed as it was using too much of my valuable red cells (it was trying to do its job of removing the sick thalassaemic red cells and had become bloated with its desperate drive to destroy them). Without a spleen my immune system was further compromised and penicillin was prescribed. To this day, however, I have not suffered a major

infection other than the regular cold.‘Regular transfusions now provided another challenge. The extra iron left from all those blood transfusions could not be removed by my body and was beginning to store itself in my vital organs. An iron-chelating agent, Desferal (desferioxamine) was prescribed to be administered as a single intra-muscular injection daily. This became another painful daily reminder of thalassaemia. The Royal District Nurse would come to school on weekdays and I would be called to have the injection. Other children saw this and it added to their curiosity. I did not reveal to them what I had to live with.

‘During my early teen years the Desferal injection was replaced by a more effective administration regime. Using a device called a slow-infusion pump, I now administered the Desferal injection myself. The pump administered the injection over a ten-hour period (overnight). Of course, as a teenager, this meant a severely curtailed nightlife at home. I did not want to have this thing strapped to me while going out. I didn’t want people to see me as different or pity me, and I did not want to deal with their questions, no matter how well intentioned.

‘Diet was not a real issue for me apart from abstaining from high-iron foods like lentils, which I did not care too much for. As long as I kept up with regular blood transfusions and using my pump, I could lead a very normal life. But using the pump is painful physically and a hassle socially. If I was not vigilant in its use I was warned that other complications could arise such as heart and liver problems, diabetes and eventually death. Ironically, the transfusions would kill me.

‘It’s a life long-condition but one that is not impossible to deal with. Excellent support at hospital by people that I now have known for over 25 years means I have an extended family to support me. Plans for the future need to be realistic. In finding a life partner I have to be conscious of the possibility that thalassaemia could be passed on to my children and its severity depends on my partner’s carrier status.

‘Over 30 years ago, my parents were first confronted with thalassaemia in a language and environment that was very difficult. They suffered as much or more than I have. I was asked once whether I regret being born with this condition. I sincerely say that I would live through it all again because I have learned so much and it has made me a stronger-willed person.’

Tax Laws - for patients Pt. 1

Australian Tax rebates - Medical expenses rebate

You may be entitled to a medical rebate if:- you reside in Australia, and
- your net medical expenses (after refunds from Medicare and health funds) exceed $1250

The rebate is allowable for medical expenses for:
- yourself, your spouse, a child under 21, a dependant child, housekeeper, a dependant child or student, a dependant invalid relative, a dependant parent or parent-in-law

The rebate amount is 20% of the excess of net medical expenses over$1250.

Example:

You incur medical expenses of $2000 for your spouse, and $1500 for your child. You are a member of a health fund, and total refunds received are $1100

Net medical expenses ($3500 - $1100) $2400
Less threshold $1250
----------
$1150

Rebate 20% x $1150 $ 250

TIP

Ensure you keep records of all valid medical expenses not only for yourself but for all of those for whom you can claim rebate. You will be surprised as to how these items may add up to.

Rodney fisher, 'MAKING TAX LAWS WORK FOR YOU - a simple guide to tax planning',McGraw-Hill, 2001, AUSTRALIA pg49-51

Tax Laws - for patients Pt. 2

CHECKLIST OF ALLOWABLE MEDICAL EXPENSES PAYMENTS

- medical practitioner, nurse, chemist, dentist, dental technician
- therapeutic treatment, artificial limbs, artificial eyes, hearing aids
- public or private hospital, medical or surgical appliance
(please see checklist below)
- eye testing, prescription and supply of spectacles, attendant for blind
-person - attendant for person permanently confined to bed or invalid chair, maintenance of a guide dog

CHECKLIST OF ITEMS THAT ARE MEDICAL/SURGICAL APPLIANCES

- wheelchairs, crutches, car controls for the disabled- kidney dialysis machines, Maximyst machines, teletypewriters, medical wigs, surgical braces

CHECKLIST OF NON-ALLOWABLE MEDICAL PAYMENTS

- Private health fund, Chiropractor, Naturopath, For special dietary foods

CHECKLIST OF ITEMS THAT ARE NOT MEDICAL/SURGICAL APPLIANCES

- breathing monitors, household air conditioners, cosmetic wigs, hydrotherapy pools, spa pools, swimming pools, wheelchair ramps, language kits

Doc Enquirer

A national forum for doctors

Please send questions you would like doctors around australia to respond too via email: Isabella Agius

As ATA would love to include this information for you in the next issue

Awareness Update - Diet For Thalassaemia Pt. 1

Reducing the iron absorbed from food.

Diet is not just about losing weight. Being a Dietician, I often have to try and convince people I meet socially that, no, I don’t spend my time handing out diets to perfectly healthy ladies who need to lose 3kg in order to fit into their evening dresses! Diet is the way we need to eat to optimise our health. This is different for different people and different ages. Thalassaemia is a complex condition and the ideal diet would need to take account of many factors. This article concerns the iron present in food.

In Thalassaemia, although most of the iron overload is due to blood transfusion, increased absorption of iron from the diet is also important. Only a small amount of iron from the diet is absorbed into our body.

The amount absorbed is higher when haemoglobin in the blood is low. People with a low haemoglobin such as those with Thalassaemia Intermedia or those with Thalassaemia Major, in between transfusions could therefore adapt their diet so that not only the total amount of iron in their diet is low but also the amount of iron in their body is low.

There are two kinds of iron in the diet: iron, which is present in red meat (Meat iron) and iron, which is widely distributed in the diet (Non-meat iron).

MEAT IRON
Meat iron is present in red meat such as beef, lamb and pork and the dark meat of chicken as well as in seafood such as sardines, cockles and mussels. Liver is a very rich source of meat iron. Try to cut down on these and perhaps substitute meat with soy protein. It is not, however, a good idea to exclude meat, chicken and fish completely from your diet because they contain other important nutrients, particularly for children.

Choose the white part of the chicken rather than red meat as this contains less iron.On average, after a meal with red meat, about 35% of iron will be absorbed into our body.

However, this may vary between 10-40%, depending mainly on whether the meal contains milk or milk products. The calcium, present in milk, cheese, yoghurt, cream decreases the absorption of meat iron. Try to drink a glass of milk with a meat-containing meal and to use milk in cooking.

Good examples are the white cheesy sauces in lasagne, pasticcio, mousaka and cannelloni, adding lots of cheese in spaghetti Bolognaise and using yoghurt and milk to cook your curries. Milk intake should be at least one pint daily, particularly because it helps to pre vent osteoporosis. If you are worried about your weight, semi-skimmed milk or skimmed milk are =just as rich sources of calcium as whole milk.

NON-MEAT IRON
Non-meat iron is widely distributed in the diet, present in eggs, chocolate, cereals, vegetables, fruits, roots (potatoes, parsnips), beans and lentils. In the UK several foods are fortified with iron, such as breakfast cereals, wheat flour and bread. However, this may not be the case in other countries.

The absorption of non-meat iron from the diet into our body is much less than that of meat iron, but it may vary more than 20-fold, depending on the composition of a meal.

The foods, which decrease its absorption, are:
1) cereals
2) dairy products

The foods which increase its absorption are:
1) fruit and vegetables rich in Vitamin C
2) meat, fish, shellfish and poultry
3) pickles, sauerkraut, soy sauce, vinegar and alcohol

It is difficult to avoid taking non-meat iron because it is present in most foods. However, diet can be modified by taking more of the foods which decrease and less of the foods which increase the amount of iron absorbed into our body.

Awareness Update - Diet For Thalassaemia Pt. 2

FOODS WHICH DECREASE NON-MEAT IRON ABSORPTION

1. Cereals, wheat bran, maize, oats, rice and soy, decrease the iron absorbed into our body and fight the effect of vitamin C. Foods rich in vitamin C increase iron absorption. It is good to eat a lot of cereals in your diet, but remember not to take a vitamin C-rich food with them, like orange juice.

Try to combine milk and cereals (e.g., cheese sandwich, French toast, macaroni cheese, cereals and milk). In the UK, all wheat flour other than wholemeal is required by law to be fortified with iron. The fortification of break fast cereals is voluntary.

It may therefore be better to choose unfortified wholemeal wheat flour and bread, and to look care fully at the label of your favourite break fast cereal. Unfortified breakfast cereals include porridge oats and some cereals in health shops but look at the label to make sure you choose an unfortified variety.

In other countries, flour and breakfast cereals may not be fortified. Is this a good excuse for more holidays abroad then? Soy protein also decreases the amount of iron absorbed into your body. Soy protein can work wellin many recipes (e.g., spaghetti Bolognaise, stews and casseroles) and the taste can be improved by adding spices.

2. Tea, coffee and some spices (e.g., oregano) decrease iron absorption. Drink plenty of tea and coffee daily, particularly with your meals. Better yet, if you take it with milk. Tea is also a very good source of antioxidants as will be discussed later. And, keep adding oregano to spice-up your souvlaki!

3. Dairy products- Milk, cheese and yoghurt decrease the iron absorbed into our body. Calcium is also important for osteoporosis, so it is good to include as many dairy products as you can in your diet. Lower fat varieties of milk (skimmed or semi-skimmed) and cheese are just as high in calcium and may be preferred if you are watching your weight. At least one pint of milk should be taken every day.

FOODS WHICH INCREASE NON-MEAT IRON ABSORPTION

1. Vitamin C is present in fruit, fruit juice and vegetables. It is better to avoid drinking fruit juice, such as orange juice, with your meal or your toast in the morning. Instead, cups of tea or coffee are better options as they inhibit iron absorption.

Alternatively, have a glass of milk! Beer increases iron absorption so it is better to avoid drinking it with your meal too often, but you could always have it on its own with some nuts! Fruit and fruit juice are, however, good sources of antioxidants and should be taken on their own as snacks. Boiled vegetables contain much less vitamin C because the vitamin leaks in the water.

2. Meat, poultry, fish and seafood not only contain a lot of meat iron but they also help to absorb more of the non-meat iron from your food! It would be unwise, how ever, to omit them from the diet altogether as they contain other vital nutrients, particularly important for children and adolescents.

3. Pickles, sauerkraut, soy sauce, vinegar, alcohol, pickled onions, tunips and carrots as well as fermented soy products (e.g., miso and soy sauce) enhance iron absorption. The amount of iron absorbed is even higher when the pickled vegetables are added to bread and rye-containing meals.

In general, a low iron diet would contain cereals (maize, whole-grain flour, beans) and root vegetables with little meat, fish or foods rich in vitamin C. A moderate iron diet would consist of cereals and root vegetables, but would also contain some vitamin C-rich foods and meat. High iron diets contain generous quantities of meat, poultry and fish.
They also contain foods with high levels of vitamin C such as citrus fruits and some vegetables. A high iron diet can be reduced to a moderate one by the regular consumption of foods which decrease the amount of iron absorbed by our body, such as dairy products, cereals, beans, coffee and tea. "Right", says Fred, lets have a cup of tea!!.

By Dr. Dona Hileti-Telfer

Awareness Update - Glossary

What The Words Mean?????

chelation =a chemical reaction in which there is a combination with a metal to form a ring-shaped molecular complex in which the metal is firmly bound and isolated.

deferriprone =a chelating agent which can be prescribed in the treatment of acute iron intoxification and chronic iron overload.

Please email any other words you would like to know the meaning via email to Isabella Agius

Awareness Update - Diet For Thalassaemia - In Summary

Present in:

MEAT IRON - Red meat: :beef, lamb, pork, chicken (dark meat), seafood (sardines, cockles, mussels)

NON-MEAT IRON - Widely distributed in the diet.

IRON Absorption Decreased By:
milk
cheese
yogurt
cereals
tea
coffee
spices

IRON Absorption Increased By:
vitamin C (fruit, fruit juice and vegetables)
meat
chicken
fish
seafood
pickles
sauerkraut
soy sauce
vinegar
alcohol

Awareness Update - Linda's Story

My daughter was diagnosed with B Thalassemia Major at 21 months of age. She will be 2 in a week’s time.

I knew I was a carrier but we only found out about my husband just before we got married. Our GP sent us to genetic counseling. Unfortunately they did not tell us much about what was involved with Thalassemia just that they could do nothing until I fell pregnant.

When I did fall pregnant we knew the odds were a 1 in 4 chance of having a Thal major child. We told our Obstetrician we were carriers but he strongly advised that a CVS test would put too much strain on the baby and as we were young and healthy it was probably better not to do the test.

I carried well and did not have any problems but still had to have an emergency caesarean to deliver her. My daughter began going off her food at about six months of age and never slept well from day one. I was always told to try different techniques to get her to eat and no-one seemed very worried about her not gaining much weight.

At about 19 months she had the flu and my doctor asked if she always had a pale complexion. I said yes, but knowing that my husband and I were both Thal carriers, he insisted on blood tests. Two hours after the tests, he had the results and I knew in my heart straight away that she was positive for Thal major.

She is now having transfusions every five weeks. She is not on Desferal yet because she has just started her treatment. We have not discussed her life expectancy and quite frankly, I don’t want to know.The medical staff who treat her are great especially her nurses.

We have been given a book from the Thalassemia Foundation but I have learnt a lot more from the Thalassemia Patients and Friends web site than anywhere else.

I think it is so important to bring this blood disorder to the attention of people at risk. Although my husband and I knew we were carriers we did not know exactly what was involved until it was too late.

All we can do for our daughter now, is to pray that one day through technology and the doctors efforts we can find a cure.

Written by
Linda Modellino, New South Wales

Awareness Update - Useful Links

Thalassaemia International Federation established in 1987, as an International non-governmental organisation co-ordinating the various issues of Thalassaemia Associations globally,
T.I.F. = http://www.thalassaemia.org.cy

England = http://www.ukts.org

The Pakistan Society was established in 1994 to Provide the best care and treatment for children with Thalassaemia.
Pakistan = http://www.thalassaemia.org.pk

The Sarawak Thalassaemia Society was establishedon the 5th feb 1994 as a non-profit organization in Sarawak, a state of Malaysia located on the Borneo Island.
Malaysia = http://www.thalassaemia.cdc.net.my

The Cooley's Anemia Foundation is dedicated to serving people afflicted with various forms of thalassemia.USA -
New York = http://www.thalassemia.org

Sickle Cell/Thalassemia Patients Network (SCTPN) began in 1989 at Brooklyn Jewish Hospital located in Brooklyn, NY, as an adult and parent support groups for individuals with inherited blood disorders such as sickle cell disease and thalassemia.USA -
New York = http://www.sctpn.org

The Northern California Thalassemia Center at Children’s Hospital Oakland was established in 1991 the center is at the forefront of research, developing and evaluating treatments aimed at improving quality of life for thalassemia patients.USA -
Northern California = http://www.thalassemia.com/

The Thalassemia Foundation of Canada is a non-profit organization dedicated to providing awareness, knowledge and support to patients, members and friends of Thalassemia.
Canada = http://www.thalassemia.ca

Awareness Update - World Arena

Whats Happening Around The World

08 -10/08/2004 Australia, Fremantle
The Australian Public Health Genetics Consortium - Genetics & Population Health

25 -27/09/2004 Saudi Arabia, Riyadh
9th Symposium on Genetics in Health and Disease - Implications for the Individual, Family and Community

15 -17/10/2004 Italy, Sestri-Levante
Scientific Meeting 'Disorders of Erythropoiesis and Iron Metabolism'05 -

07/11/2004 India, New Delhi
3rd International Conference on Thalassaemia

18- 22/11/2004 Cyprus
9th International Workshop on Clinical Management of Thalassaemia17-

19/03/2005 USA, Orlando
New York Academy of Sciences 8th Cooley's Anaemia Symposium

Research Update - The Panos Report

Affordable oral iron chelation for all - Date: Thu, 04 Mar 2004 16:45:38 +1100

Dear Friends,

It is a long time since I was actively involved in getting UKTS established in London. Much has happened since then, but our common dream of an effective therapy for thalassaemia still remains to be achieved, while most patients in the world still do not have access even to the minimum standard of acceptable therapy. UKTS played a leading role for many years in the funding of basic research that led to the development of L1, as a cheap oral iron chelator.


For reasons beyond the immediate scope of this message, but still worthy of exploration at a more appropriate time, UKTS and patient representative organisations have subsequently been denied any role in determining the availability and price structure for L1. Furthermore, despite many studies supporting an important role of L1 in oral iron chelation therapy of thalassaemia patients, the protracted dispute between Nancy Olivieri and Apotex continues to create confusion in the minds of many patients, clinicians, ethicisits and regulatory authorities, hindering the development of a wide consensus on the conditions under which L1 may be used to improve iron chelation therapy for thalassaemia.


The limited licensing of L1 in Europe and the recent rejection of the case of Dr Olivieri by the European Court are important steps forward, but they do not seem to have daunted Dr Olivieri in her dispute with Apotex over L1. We would be all be clapping in favour of Dr Olivieri if her dispute with Apotex was focused on safeguarding patients' interests and research integrity against interference by big pharma.
Unfortunately, the dispute has reached the stage where patients interests are being abused and harmed in the name of safeguarding "research integrity". I have copied to some of you in the past my correspondence urging Dr Olivieri to make a public reassessment of L1, not only on the basis of her initial observations that led her to suspect lack of efficacy and increased risk of liver fibrosis, but also taking into account all the other studies that have been published since then.


Unfortunately she has failed to respond directly to my messages.
Furthermore, in a public statement released last month, she raised new claims that several "premature deaths" of patients have occurred since the licensing of deferiprone in Europe. These new claims, in a non-scientific circular, contrast with the increasing evidence from a number of respected groups in peer-reviewed scientific journals, that have failed to find any evidence of increased risk of liver fibrosis and support a valuable role of L1 in reducing intracellular iron damage, the most likely mechanism of continuing iron damage in patients that are well chelated with desferal. I have therefore taken the initiative to ask Dr Olivieri to clarify the basis for her new claims. Again she has failed up to now to respond directly to my messages.


However, Prof David Nathan has responded on her behalf, proposing that the claimed "premature deaths" have happened among patients "after a short time on L1". Were it not for the respect that we all have for Prof David Nathan, I would have said that this is the most un-scientific statement I have ever heard in my life. That Prof Olivieri and Prof Nathan can make such claims after 15 years of clinical studies on L1 and when thousands of patients are currently receiving L1 without a single scientific report of "premature deaths" indicates to me that the "murky story of deferiprone", as has been recently called by Prof Savulescu, is far from over.


The only clear beneficiary out of this "murky" story up to now has been Novartis who has fully exploited the situation to expand its desferal monotherapy into more and more markets at exhorbitant prices and is now "rolling out" its long-heralded oral iron chelator, without providing the slightest evidence that it canprotect against intracellular oxidative damage, in the way L1 does.


Besides the general obligations that UKTS shares with TIF and national patient support groups in other countries towards thalassaemia patients, UKTS also has the moral authority and justification to ASSERT a key role in resolving the continuing dispute over the use of L1 and DEMAND a voice in determining the price structure for L1 in various countries in favour of patients.


The early development of L1 was funded exclusively by UKTS, so it is immoral and unethical for Apotex or any other drug company to claim a unique right in determining the price structure of L1.


UKTS can speak and demand a saying on these issues with the moral authority that comes out of its role in the early development of L1, as well as on behalf of all the thalassaemia patients that are participating selflessly in clinical trials, often at considerable risk and great discomfort to themselves.


TIF and other national patient organisations have of course a role in this as well. As a first step in this direction, I therefore propose for UKTS to establish a comprehensive web page on "Iron Chelation".

This should include sections containing:

a) Key documents (grant applications, reports & correspondence) demonstrating the role of UKTS in the early development of L1 and documenting its new role to get drug companies to make iron chelators available to thalassaemia patients at affordable prices in all countries;

b) Key scientific publications on iron chelation therapy (with permission from scientific journals, where necessary);

c) Key documents relating to the Olivieri/Apotex and Olivieri/European Court cases;

d) Information on new iron chelators at various stages of development;

e) A professional forum for publishing correspondence and open discussion on oral iron chelators;

f) A patient forum to provide authoritative guidelines and information to patients in various countries on the use of oral iron chelators.

Oral iron chelation is a job half-complete. While we all hope that even more efficient and safer oral iron chelators than L1 may still be developed, UKTS should take again a leading role on oral iron chelation, as it is demanded by the new circumstances. I am sure that an initiative to make desferal and oral iron chelators available at affordable prices to thalassaemia patients in all countries will be greatly applauded by the medical and scientific communities and patients all over the world.
As always dedicated to the struggle for the effective therapy of thalassaemia,

Yours sincerely,

Panos Ioannou,PhD Head, Cell & Gene Therapy (CAGT) Research Group The Murdoch Childrens Research Institute,
Royal Children's Hospital Flemington Road,
Parkville 3052, Melbourne, Australia

Associate Professor, Dept of Paediatrics, University of Melbourne Honorary Senior Scientist, The Cyprus Institute of Neurology & Genetics

State Update - OLD

He is only 10 years old but what an impact.....

In Queensland we are only a small group but we have a huge impact. In early March 10-year-old Zachary, accompanied his father Anthony, to an International Internet mini conference in Sydney. The organisers of the seminar were made aware of Zachary's blood disorder "Sickle cell thalassaemia".

In attendance were many corporate executives from states in and around Australia, and countries from around the world. Zachary's positive attitude and openess of his blood disorder made a huge impact on the organisers of the event.

It was a great surprise for Zachary who has been receiving Blood transfusions since he was three years old when a cheque for $22,000 was presented for the Starlight Foundation in his name. Money raised by the organisers of the internet seminar in just one weekend.

Zachary's Dad, Anthony was thrilled by the enthusiasm, compassion and understanding of the people for his son "this is such a special day for us all. thank you. the money raised will help children just like Zachary, wishes come true' The presentation put a smile on the boys face that took a week for it to disappear.

A wish is an opportunity to change tomorrow....

Isabella Agius ATA-Queensland Delegate

State Update - VIC

The Thalassaemia Society of Victoria

Last year was a very busy one for the Thalassaemia Society of Victoria. Some large projects for 2003 included updating our website at TSV, attending the Thalassaemia International Federation conference in Italy and organizing the Australian Thalassaemia Association conference in Brisbane (with much help from Isabella in Queensland).

This year’s major focus is on the provision and update of our resources. We will shortly launch 3 new brochures on alpha thalassaemia, sickle cell and haemoglobin E. These brochures will be available for the Australian Thalassaemia Association to distribute across Australia. We have also drafted fact sheets specifically targeted at the lay person with little knowledge of medicine or genetics. These fact sheets are to be translated into 8 languages for those populations at high risk of haemoglobinopathies. We will then target educational presentation to these multicultural groups.

Ongoing website updates will include a new streamed presentational on thalassaemia. This presentation will be located on our ‘education’ page so please visit and provide us with your feedback.

Sandra has already booked a number of educational presentations at Secondary Colleges and community organizations. Last year she found it difficult to keep up with the increasing demand but please feel free to contact her if your know anyone who would like to arrange a presentation.

We are also looking to update our policies at the Thalassaemia Society. Such policies will address issues such as grievances, privacy, volunteering and project structure and evaluation.

Sotrios Katakouzinos TSV- President

State Update - SA

Thalassaemia Society of South Australia

The Thalassaemia Society of SA is a non-profit organisation that aims to:

* Raise public awareness for the genetic blood condition Thalassaemia in South Australia;

* Ensure that people with Thalassaemia in SA receive the optimum standard of treatment;

* Provide advocacy on behalf of patients;

* Support and encourage medical and social research projects designed to improve the quality of life for people with haemoglobin conditions; and

* Obtain and distribute the latest scientific and medical information on Thalassaemia

The society relies on fund raising and donations to carry out its work. In 2004, the Society will be holding a dinner dance on Saturday, May 8th 2004 as its major fund raising event.

May the 8th was declared International Thalassaemia Day by the World Health Organisation in 1983, therefore this year marks the 11th anniversary of this day.

We look forward to strong community support for this event. For further information, please contact Irene Ponias on 0402 358 006.

Irene Ponias TSSA - President

State Update - WA

Thalassaemia Association of Western Australia

*FUND RAISING The Calabrese Club inc held a quiz night on Friday May 7th 2004, Which we are helping to get prizes for and to also get as many people as we can to come along and form tables. The Calabrese Club has been so kind to donate part of the proceeds to the Thalassaemia Association of WA. Which is a great help considering we do not have to organize and outlay much towards the night. We are also using the Entertainment Books as another form of fund raising for this year. It's a fairly easy way to raise money as most people know about these books and are waiting for them to come out again.

*MRI's Tim St Pierre is still conducting research with MRI's to test amounts of iron in the liver without the need of a liver biopsy. A few patients in Perth have just volunteered to have another MRI scan on their liver as part of the ongoing research and study. Tim is also looking alot further into testing amounts of iron in the heart using the MRI machine. There is alot of this happening in Adelaide for any patients who are interested to participate in this form of research. Teresa volunteered to have her heart and liver scanned in Adelaide with the help of our association and Tim St Pierre's research group.

*CONFERENCE In the second week of August 2004, there will be a conference on Genetics and Population Health. The big focus will be Thalassaemia because of its growing impact on individuals, families and communities, and on the national and community health resources. This conference should be a big help towards providing awareness to the public and health professionals in Perth. Due to this conference being held in Perth, Perth will host the AGM for the ATA on Friday August 6th.

*NEEDLES I dont know if this news is too old as it happened early last year. We pushed one of our big hospitals (Sir Charles Gardiner Hospital)to provide needles for all our Thal patients attending that hospital. This was done with a letter that we received from (I think it was Rosa in Sydney) Sydney to give to the hospital's CEO. So now patients do not have to pay for their needles.

Josie Stockden ATA-WA Delegate

Kids Corner - Thalassaemia Song

On the outside we look different
on the inside we are the same

It's hard for people with thalassaemia
and it's hard for people who live with thalasaemia

When you are out of school, you miss your friends
and they miss you

It's not nice to take a needle and I am scared
the Doctor will make a mistake

You are different but everyone thinks you are normal......
it's ok being special but i would rather be normal

WE ARE ALL BRAVE SPECIAL
AND HAVE GOOD FRIENDS.

Written by:Alexis, Zac, Adrienne, Raani, Rajeev and Jessica.

National Thalassaemia Conference,
Brisbane November 2003

Kids Corner - Jessica's ATA Conference Report

News from one of the kids at the Conference. Jessica attended the kids program during the QLD ATA conference, and had this to say;

The first day that I went to the children Thalasseamia conference I was quite nervous, but after about 3 minutes Alexis and Zack and Adrian made me feel at home. Then we started to make up a Rap song about Thalasseamia.

Zack had to do the beat and Shannon played the guitar and then we had a really neat song. Then we went out to morning tea and got to see our dads and mums, and then we went back inside. Then some of the adults came in and we played hang-man.

After theat we went to the hospital and visited some people. Then we went to the Starlight room. While we were there we made things and played on the Nintendo and then we played memory with the Captain Starlight's.

After this we had to go back to the conference and made Christmas decorations, and then the Captain Starlight's came and painted our faces and then they done some magic tricks. We then all played pictionary.

I would like to say it was the best time in QLD, and hope to meet everyone and more kids at other conferences.

Jessica Christo, 9 years old S.A

Calendar Of Events Pt. 3

The Eighth Cooley's Anemia Symposium
March 17-19, 2005
Hilton Hotel in the Walt Disney World Resort,
Orlando, FL

The Cooley's Anemia Foundation is collaborating with the prestigious New York Academy of Sciences (NYAS) The goal of the symposium is the presentation and dissemination of current findings and the setting of future directions regarding basic and clinical research in thalassaemia.

The program will address recent advances in:

1. The natural history of thalassaemia worldwide
2. Gene regulation and gene therapy
3. Mechanisms of cell injury of iron overload in thal
4. Development and therapy of iron chelators
5. Stem cell transplantation
6. New therapies for thalassaemia
7. The adult thalassaemia patient
8. Non-invasive iron measurements

The symposium will integrate basic science and clinical research so that representatives of both of these disciplines as well as clinicians can develop a mutual understanding of recent progress in thalassaemia. Conference attendees should achieve an understanding of the molecular biology, pathophysiology, epidemiology, clinical care and psychosocial management of thalassaemic disorders.

A combination of keynote addresses, state-of-the art lectures that provide an overview of progress in different aspects of thalassaemia, oral presentation of recent findings by leading investigators, and poster presentations will provide a comprehensive overview of progress in the understanding and treatment of thalassaemia.

CAF and NYAS have collaborated on the seven previous Cooley's Anemia Symposia, beginning in 1964. As with previous conferences, the proceedings of this conference will be published as a volume in the series Annuals of the New York Academy of Sciences.

*Reprinted with permission from the Cooley's Anemia Foundation website,*

Calendar Of Events Pt. 2

Conference on family & sibling issues 18th -19th Nov 2004 Adelaide

Great things are happening around Australia in terms of sibling and family support. It would be good to have people come together and share examples of such programs and to share what is happening in terms of research. Such an event would allow us to look at future directions for this work, covering such issues as mental health, education, community responses, research, family strengths, early intervention, policy and political agendas.

Siblings Australia Inc.www.siblingsaustralia.org.au
email: info@siblingsaustralia.org.au
Fax: 08 8161 7032

Calendar Of Events Pt. 1

The Conference Genetics and Population Health 8th - 10th August 2004 The Esplanade Hotel Fremantle

The aim of the Conference is to provide an interactive forum for people with specific health interests, clinicians, researchers, genetic counsellors, and staff involved in medical outreach programs, to meet and collectively discuss topics of challenge and concern.

Recent advances in the diagnosis, treatment and overall care of people with thalassaemia will be the lead topic, and in addition to the Conference there will be a free access Workshop on World Best Practice in the Management of Thalassaemia 7th August At the The Esplanade Hotel Fremantle. With 26 invited speakers from 14 countries this will be a very important international meeting.

Sponsored by The Australian Public Health Genetics Consortium, The World Health Organization, The U.K.-based Welcome Trust, and many associated national and state bodies.

As Co-Organizers, Dr. Wendy Erber and myself warmly welcome the active participation of ATA and its members,and we look forward to welcoming you to Fremantle in August 2004.
www.geneticsangpopulationhealth.com