Cooley's Anemia (Thalassemia)

Recently, I bought some presents from a commercial site for Christmas,
all the products can enjoy a big discount, it is worth visiting:
fallinele.com ,
for the Christmas more one choice. $--.

:The Daily Star: Internet Edition: "Treatment Of Thalassaemia
In quest of comprehensive treatment facilities
Star Health Correspondent

Thalassaemia is an inherited blood disease. In thalassemia, the genetic defect results in the formation of abnormal haemoglobin molecules, and this in turn causes the anaemia which is the characteristic presenting symptom of the thalassemias.

Other consequences of the disease are deposition of iron from the haemoglobin affecting the reticuloendothelial system of the body.

So the mainstay of thalassaemia treatment is blood transfusion at a regular interval and removing of iron from body by iron chelating agents.

If this can be maintained strictly, thalassaemia patients can also lead almost a normal and productive life. But most of the cases it does not happen. There remains various causes behind it.

First of all, patients seriously lack from proper knowledge about how to manage the disease and other important issues. Secondly comes the crisis of quality blood frequently. Sometimes patients get infected from poor quality blood. Thirdly, many patients cannot maintain the iron chelating agents. Sometimes availability of drug is a problem, while most often not maintaining a proper guideline is the serious issue.

Above all, handling all these issues from the childhood, patients and their families are very exhausted. Many patients are quite in a fix what to do,"

Health care: Leading woman

* Dr Mariam is the first woman to become Undersecretary at the Ministry of Health, where she supervised initiatives designed to make primary health care available to the biggest share of population in the northern emirates.
* In 2005, Dr Mariam was the leader of the executive team dedicated to research key social development issues described in the "Dubai Strategic Plan 2008-2015".
* She is the founder and executive director of two non-government and non-profit organisations - the UAE Down's Syndrome Association and the UAE Genetic Diseases Association.
* Dr Mariam initiated many community programmes such as "UAE Free of Thalassemia by 2012", and the "Wellness Centre".
* She graduated from Dubai Medical College in 1999 as top of her class and specialised in community medicine.

Efforts to empower UAE nationals

By Abbas Al Lawati, Staff Reporter
Published: July 26, 2008, 23:24


Dubai: A new authority under the Dubai government was recently set up to act as an umbrella for social service organisations and was also tasked with promoting national identity and empowering UAE nationals.

Gulf News spoke to Dr Mariam Mattar, Director General of the Community Development Authority (CDA), about the authority's plans and challenges ahead.

Gulf News: What is the objective of CDA?

Dr Mariam Mattar: CDA was launched to develop a framework for social development in line with the social objectives of the Dubai government as highlighted in the Dubai Strategic Plan 2015.

Through specialised entities, we aim to create a strong social safety net for everyone living in Dubai and enhance social cohesion between different nationalities and groups as well as promote our national identity.

Our objectives are to encourage social cohesion, social inclusion and social protection, to promote the national identity, and to empower nationals.

We would like to become the umbrella organisation for social service organisations and be able to come up with a legal framework under which these organisations would fall.

It has come to our attention that many social service organisations in the UAE are not licensed and we would organise the sector better by coming up with additional guidelines.

As you know, some of the accusations that the UAE face are lack of data and credible sources [to back them up]. We have been emphasising [becoming a source of data on Dubai] from day one.

The Dubai Statistics Centre has helped with this ...

But we didn't rely on the centre alone. Our research and strategy department has specialists in social studies and social analysis ... [to] help us work with some of the social trends.

We [plan] to have an annual report ... but a lot of it relies on surveys that will be conducted and help us assess the needs of every individual resident in Dubai according to geographical locations. The services resulting from that will be tailored to individual needs.

How significant a role is CDA going to be playing?

Ours is a monitoring and policy related role. We don't devise new policies but enforce and renew current policies in Dubai that help us implement our objectives. As for the federal level, we coordinate with [federal bodies] to enforce the federal laws that weren't being enforced previously ...

We ... provide services based on studies [on future] social requirements and how the different bodies in Dubai can cooperate to fulfil the requirements, whether it is legal, administrative, security-related or financial.

It was mentioned with the launch of the programme that CDA would be tasked with strengthening the national identity. Is the establishment of an official body with that objective indicative of a realisation that there's a problem?

I wouldn't call it a problem. Let's say that there are some needs and we as the government are fulfilling these.

The media often says there is a need to preserve or protect the national identity. The national identity doesn't need protection because it is here and so are the nationals, the natives of this country?

Our objective is to promote national identity, to ensure that the UAE national is distinguishable and noticeable. ...

We at the authority ensure that the national identity is visible in society. This is not just about how we cook, eat or dance, or our portrayal in the media. We emphasise empowering Emiratis in Dubai with skills and expertise to allow them to compete and contribute in the development of society on par with non-nationals.

We've had more than a workshop and brainstorming session with all types of [people], ... and found mainly that many Emiratis need support to be able to participate in the fierce competition [for work in Dubai]. And we can help - whether through enabling them to speak a global language, teach them skills, and help them ... communicate with [other cultures].

How do you expect to deal with some of the concerns UAE nationals have about the rapidly changing population make-up of the UAE that is making them a minority?

We are working on research about the social needs of Dubai's population ... The mere presence of expatriates here who are specialised in their fields reflects positively on the development of society. Their presence here is of a consultative nature and we need to utilise [their expertise] to [learn from them].

I'm not denying that some Emiratis might feel that expatriates are given priority. But we at the CDA take note of these observations. [Our] survey will give the respondent the chance to [express their views on these issues].

What role does the Arabic language play in the Emirati identity at a time when it is seemingly being phased out in education and in some government entities?

The Arabic language is one of the [basic] elements of Emirati identity and we are trying to promote Arabic to non-nationals. There will be a number of initiatives from CDA to preserve the language and promote it. The programme will focus on three levels - legislation, auditing and service levels.

'It is important to push for change'

Dubai: When Gulf News asked Dr Mariam Mattar why she would be the most suitable person to head the Community Development Authority (CDA), she spoke of a personal experience that made her feel an obligation to contribute to society.

"In 1999, two weeks before my final year exams at Rashid Hospital, a tragedy hit my family, and me particularly. I lost my beloved father to artery block, something that could have been prevented with proper medical care. Back then, I decided not to take my exams, however, everyone at Rashid Hospital gathered to show me support and reminded me of the impact that family physicians have on the individuals and communities they treat.

"The death of my father was a turning point in my life following which I discovered the importance of taking the initiative and pushing for change on all levels, mostly at individual but also social, political and regulatory [levels]."

Striking the right chords
27 Jul 2008, 0000 hrs IST,TNN

Melodious music, lilting voices and different surs and taals filled a room at the Trident on Friday evening as the annual ghazal concert ‘Khazana’ — the proceeds of which go to the Cancer Patients Aid Association and to the Parents Association of Thalassemia Unit Trust — took place.

The roomfull of guests were treated to ghazals sung by some of the country’s best in the genre — Pankaj Udhas, Talat Aziz, Penaaz Masani, Anup Jalota and Mitalee and Bhupinder Singh, Pt Ajay Pohankar and his son Abhijit and Rajendra Mehta who performed the next evening.

This year’s two-day fest being in honour of ghazal legend Begum Akhtar, there was a short speech on her and her contribution to the field. Rakshanda Khan anchored the evening which was attended by Shatrughan Sinha who was seen having a colourful conversation with Nana Chudasama, Vijay Kalantri who caught up with Vasant Bhandari, Satish Mathur and Chhaya Momaya who was seated with Pankaj’s wife Farida Udhas.

Over cocktails and some soulful music, Mumbai’s musically inclined enjoyed an evening that was significant not just for its performances, but also for the heartening cause behind it. Cheers to that!

Australian Life Scientist - Resonance tests FerriScan: "Resonance tests FerriScan
Resonance Health applies for reimbursement for FerriScan
Dylan Bushell-Embling 01/07/2008 10:57:38

WA-based diagnostics company Resonance Health (ASX: RHT) has lodged an application with the Australian Medical Services Advisory Committee seeking reimbursement for its FerriScan iron test in Australia.

The company has already had some public funding success overseas, with the Whittington Hospital in London receiving funding approval from the British National Health Service [NHS].

FerriScan is used in to test for and measure levels of iron overload in the liver, caused by regular blood transfusions received by patients with sickle cell disease, thalassemia major and myelodysplastic syndromes.

* Login or register to post comments"

Blood website comes to patients' help: "Blood website comes to patients' help
With lack of awareness about blood types, many lives are lost. In an emergency, the relatives and friends of the patients are often helpless when blood shortage crisis occurs. A website on blood groups has been launched to help those in need.."

Yemen marks World Blood Donor Day - Yemen Times: "Yemen marks World Blood Donor Day
Yemen Times Staff




This past Saturday, Yemen marked World Blood Donor Day, a World Health Organization-sponsored event focusing on the role of voluntary unpaid blood donations in the fight to save lives across the globe.

“This annual event highlights the role blood donors play in saving lives and improving the health of millions. It creates awareness about the availability, safety and appropriate use of blood and blood products,” a WHO statement said about the event.

Voluntary blood donation in Yemen still is somewhat uncommon, as most patients needing blood get it from relatives who donate whenever the need arises, but the Ministry of Health is trying to change this."

I'm sorry ,but I inadvertanly deleted the subscriber file. Please put your email address in the subscriber box or send me an email and you will be updated on all new releases of the blog. Thanks for your patience.
Peter Mulieri pmulieri@gmail.com

Avoiding Spleen Removal for Cooley's Anemia Sufferers: "Avoiding Spleen Removal for Cooley's Anemia Sufferers


Weill Cornell Researchers May Have Identified Gene Responsible for Mutated Red Blood Cells


Preclinical Study Provides Fresh Perspective on Disease, with Potential New Therapeutic Options


NEW YORK (MAY 22, 2008) — Researchers from Weill Cornell Medical College may have discovered the precise role of a gene in one of the world's most common blood disorders, beta-thalassemia, commonly known as Cooley's anemia. Along with sickle-cell anemia, Cooley's anemia is the most commonly inherited disease in the world, affecting many people of Mediterranean descent, and 20 out of every 100,000 African-Americans. The World Health Organization estimates that between 50,000-100,000 children are born with the disease each year.

The research is published in the latest online issue of the journal Blood, the official publication of the American Society of Hematology (ASH).

In Cooley's anemia, hemoglobin — the oxygen-carrying molecule on red blood cells — is mutated and non-functioning, resulting in a low red-blood-cell count. Common symptoms of the disease include fatigue, shortness of breath and an enlarged spleen, called splenomegaly, caused by a buildup of malformed red blood cells within the body. The spleen works to filter out these unhealthy cells in order to protect"

‘Hope Continues’ for UAE Thalassemia patients | Novartis: "International Thalassemia Day takes place each year on 08 May, but the UAE event was scheduled for a date and time believed to be more convenient and accessible for a greater number of UAE participants.

Thalassemia patients and their families participated in an educational lecture presented by Senior Doctors and Medical Advisors from across the UAE, followed by a question and answer session where they learnt about treatment updates.

The evening was devoted to entertainment and leisure activities.

“Thalassemia Day is a time to reflect on the seriousness of the disease and ask ourselves how we can extend and improve the lives of Thalassemia patients. It also provides an opportunity to educate the public on successful prevention programmes,” said Dr. Khawla Belhoul, Director, Thalassemia Center, Dubai Health Authority.

“The UAE’s Thalassemia community shows consistent resilience and positivity when faced with the challenges associated with the treatment of this chronic disease, and the ongoing support from the public contributes greatly to this,” said Dr. Fatma Sajwani, Specialist Haematologist, Al Qassimi Hospital, Sharjah."

‘Hope Continues’ for UAE Thalassemia patients | Novartis: "‘Hope Continues’ for UAE Thalassemia patients
The UAE Thalassemia Community gathered at Dubai Creek Park on Saturday 03 May to celebrate International Thalassemia Day and mark the conclusion of ‘Hope Continues,’ the campaign designed to raise awareness of the blood disorder, and the need for increased pre-marital blood testing and blood donation.

* United Arab Emirates: Tuesday, May 13 - 2008 at 14:39
* PRESS RELEASE"

ekathimerini.com | Thalassemia units lack staff: "Thalassemia units lack staff

Thousands of Greeks suffering from thalassemia – a type of anemia affecting people of Mediterranean descent – are unable to get the regular blood transfusions they need due to an acute shortage of qualified staff at specialist centers across the country, experts told a press conference yesterday.

Of the country’s 38 centers for treatment of thalassemia – also known as Mediterranean anemia – several lack doctors, meaning that nurses must carry out transfusions, the experts said ahead of a panhellenic conference on the disease starting on Friday. There are also space restrictions. Often patients are cramped into tiny rooms, sometimes in the basement, for transfusions, experts said.

A new law voted through Parliament last November had aimed to improve the operation of the units. But problems with staffing and infrastructure persist."

-
Wednesday, August 30, 2006; Posted: 05:13 AM

Find out where NVS is going

Use our most popular trading tool to find
out how NVS will move in the next
5 days.


Stocks rated 9 and 10 have outperformed the average stock by approximately 14 to 1 since 1995 and ideally are the stocks to focus on each day. Stocks rated 1 and 2 on average have lost money and you want to know these stocks in order to avoid them.

Click here for more details

(RTTNews) - Early Wednesday, Basel, Switzerland-based healthcare company Novartis AG (NVS | charts | news | PowerRating) announced the European Commission or EC's approval for Exjade as a new treatment for patients with transfusional iron overload. The company said that the EC approved the drug in all 25 member states of the European Union or EU.

According to the company, iron overload is a potentially life-threatening consequence of frequent blood transfusions. After a few transfusions, iron starts to build up in the body as the body cannot remove it by its own. In iron chelation, an agent binds to iron in the body and helps remove it through the urine or feces.

Novartis said that Exjade is the first oral iron chelator approved in the EU for the treatment of chronic iron overload due to frequent blood transfusions in patients age six and older with beta thalassemia major, myelodysplastic syndromes, sickle cell disease and other anemias. Exjade is administered once-daily as a drink. This drug provides continuous chelation with the single daily dose. The drug also removes highly toxic labile plasma iron from liver and heart, the company noted.

Novartis also said that deferoxamine, the standard iron chelator used globally, requires nightly infusions by needle and pump, which often takes eight to 12 hours per night for five to seven nights in a week.

Novartis further stated that during the clinical trials for Exjade, data from more than 1,000 patients with a broad range of underlying diseases showed that the drug is effective in reducing excessive body iron, especially in patients with moderate to severe iron overload. While the main Phase III study demonstrated that Exjade as effective as deferoxamine, a sub-study indicated the drug's efficacy in reducing the content of iron in the heart.

The company also stated that the drug was generally well tolerated in the clinical trials. The most frequently reported adverse events were nausea, vomiting, diarrhea, abdominal pain, skin rash and increases in serum creatinine.

As per the company, before granting the approval, the Committee for Medicinal Products for Human Use or CHMP of the European Medicines Agency or EMEA issued a positive opinion recommending marketing authorization. The drug has already been approved in 29 countries and has been designated an orphan drug in the EU, U.S., Switzerland and Australia. The company also stated that additional regulatory submissions for Exjade have been made around the world.

On the NYSE, NVS closed Tuesday's regular trading session at $57.50, up $0.52, on a volume of 1.08 million shares.

Copyright(c) 2006 RealTimeTraders.com, Inc. All Rights Reserved

AN 11-year-old boy who suffers from a life-threatening blood disorder has become one of the youngest people in the country to learn the Koran off by heart.

Junaid Jubar, who lives with his family in Chiltern Avenue, High Wycombe, has memorised the contents of the Koran in just five years - devoting two hours a night to learning several pages of the Islamic holy text at a time.

The former Castlefield Primary School pupil and Chelsea fan can now recite this book off by heart and has achieved this on top of his everyday studies and amid fortnightly trips to north London's Whittington Hospital.
continued...

He said: "Learning the Koran was hard. But it feels amazing.

"I really enjoyed it. And now that I know it off by heart I want to study it now - properly. It's really inspiring.

"My parents really supported me along the way, especially my mum."

Junaid has a medical condition known as Thalassemia Major, which means he cannot produce his own haemoglobin - the protein in red blood cells which carries oxygen around the body.

He receives vital blood transfusions and crossmatches to treat his medical condition and five nights a week he also has to take tablets to reduce the amount of iron in his blood.

Junaid also gets very tired and agitated a week or so before each transfusion - which is why his parents Shamim and Abdul are so proud of his achievement.

Mrs Jubar, 33, said: "It's a miracle. Our friends and family are shocked that he has done it, especially with his condition.

"Even if he won the lottery it would mean nothing compared to this. It's as if he has special powers - like superman or spiderman."

Junaid's ability to absorb information has even earned him a place at the Royal Grammar School.

His teacher Hafiz Hamid-ud-Din Qureshi, says he has never known a person of Junaid's age learn the whole Koran by heart.

Mr Qureshi said: "To recite it is a big achievement. It's something to expect us just to read it but to be able to recite it is a powerful thing. It's a marking of our religion."

To celebrate Junaid's achievement a special ceremony is being held on the evening of September 10 at the Castlefield mosque in Rutland Avenue when guest Pir Habib-Ul-Rehman of Niyaran Sharif is flying over from Pakistan.

10:20am Sunday 3rd September 2006

U.S. Newswire : Releases : "New Drug Poised to Radically Change Treatment of Severe Anemias": "New Drug Poised to Radically Change Treatment of Severe Anemias

4/21/2006 9:05:00 AM

To: Medical and Health reporters

Contact: Laura Stark of the American Society of Hematology, lstark@hematology.org or 202-776-0544

WASHINGTON, April 21 /U.S. Newswire/ -- Those with severe chronic anemias need frequent blood transfusions to remain healthy, but such frequent transfusions can cause a potentially deadly buildup of iron in the body, leading to heart and liver failure. The traditional treatment to remove excess iron is so onerous that many patients choose to forgo it, putting their own lives at risk. The results of an international study on deferasirox, a new drug that may revolutionize the way chronic iron overload is treated, will be published in the May 1, 2006, issue of 'Blood', the official journal of the American Society of Hematology.

The current standard therapy to rid the body of excess iron is deferoxamine, administered for as long as the patient continues to receive blood transfusions, which, for many patients, can be for the rest of their lives. Although its effectiveness and safety are well-established, the necessity for the drug to be delivered by slow subcutaneous or intravenous infusion for eight to 12 hours a night over a period of five to seven"

Cyprus first lady meets thalassemia patients in Delhi: "Cyprus first lady meets thalassemia patients in Delhi

By Indo Asian News Service

New Delhi, April 12 (IANS) First Lady of Cyprus Fotini Papadopoulou Wednesday interacted with over 100 thalassaemic children in a private hospital here and donated speciality equipment used by patients of the ailment.




'India like Cyprus is a country highly affected by the disease, which, if not addressed properly, can amount to a major public health problem,' Papadopoulou said during her visit to the Sir Ganga Ram Hospital.

She gifted 10 infusion pumps used to administer medicine on thalassemia patients and received a painting of the Taj Mahal made by a 12-year-old child afflicted with the ailment.

The meeting was organised by Thalassemia India, an NGO.

Citing an estimate of the World Health Organisation (WHO), the hospital's vice chairman P.K. Khanna said around 300 million people globally, nearly 30 million of them in India, are carriers of the defective thalassemia genes.

Around 8,000 children are born with the disease in India every year with a high degree of prevalence among Punjabis, Sindhis, Gujaratis and Bengalis, he added.

Thalassemia India secretary Shobha Tuli requested Papadopoulou to organise a doctors' exchange programme between the two countries so that Indian physicia"

commercialappeal.com - Memphis, TN: Local: "Scientists 'on path to a cure' for sickle cell

By Mary Powers

April 13, 2006
Amid a national meeting packed with reports on strategies for easing symptoms and extending lives of sickle cell patients, a Memphis investigator stepped to the podium Wednesday to talk about a possible cure.

Dr. Arthur Nienhuis of St. Jude Children's Research Hospital said although progress has been more gradual than some predicted, gene therapy still holds the promise of cure.

Advertisement
'Hopefully in our lifetime or that of those we train, it will reach its full potential,' said Nienhuis, who has returned to research after retiring as St. Jude's director.

Scientists have already used gene therapy to cure the disease in mice. That milestone was reported in 2001. The first human trial began last year in Paris. Plans call for enrolling five patients with sickle cell and five more with beta thalassemia, another inherited blood disorder.

'They may not fully succeed, but we hope they will start us on a path to a cure,' he said.

The study comes as treatment advances during the past 25 years have pushed average life expectancy for Americans with sickle cell from their 20s to their 40s or 50s. But Nienhuis said the national sickle cell mee"

Tracking birth defects: "Tracking birth defects
State registry would provide scientific data, assist in planning education, social services
Monday, March 06, 2006
TROY GOODMAN
News staff writer

The term 'birth defect surveillance' rarely grabs much attention - that is until the data it generates is used to explore issues like rising autism rates and an outbreak of babies born without fully formed brains.

Right now, though, birth defects registries are a hodgepodge of regional or university-based programs that leave major gaps in science's ability to know what kinds of defects are happening and where, said Dr. Russell Kirby, a professor of public health at the University of Alabama at Birmingham.

Alabama is among 15 states and the District of Columbia without a comprehensive birth defect registry, according the Centers for Disease Control and Prevention in Atlanta. That could change in the coming years, Kirby said. He is leading a push to start a birth defects monitoring system that would track cases at the hospital and clinic level throughout every city and county. Now, only one birth defects registry exists for a 20-county region in South Alabama.
Advertisement





'For example, has the rate of spina bifida declined statewide during the last few years? We can't answer that question,' Kirby said.
"

Singapore's leading doctors set to arrive in Middle East | SingaporeMedicine: "A delegation of Singapore's top doctors - including experts in 'fusionless' technology, stem cell research, robotic surgery and the treatment of thalassemia - are set to arrive in Dubai this weekend for a range of meetings and to participate in the region's largest medical exhibition."

WAM: "Detailed thalassemia study to be conducted

Jan 9, 2006 - 01:10 -

Dubai, Jan 9th, 2006 (WAM) - A detailed study of the prevalence of a genetic blood disorder among UAE nationals will be conducted soon to replace a random study made in 2002, said a leading doctor on thalassemia.

Dr Abdullah Al Khayyat, director of the Al Wasl Hospital and head of its Thalassemia Centre, told Gulf News the statistic of 1 in 12 nationals was based on a random sample, according to the Gulf News.

'Our plan is to have a real study. The eight per cent [prevalence rate) was based on a random sample,' he said.

He added the hospital would begin the study after the end of the international conference on thalassemia and other blood disorders taking place at the Dubai World Trade Centre tomorrow. He was speaking after attending a press conference on exjade, a recent FDA-approved (Food and Drug Administration) deferoxamine drug to treat excess iron in the blood. Thalassemic patients suffer excess iron in the blood and internal organs due to the frequent blood transfusions they must undergo. Thalassemia is a potentially life-threatening genetic blood disorder characterised by the breakdown of haemoglobin in the blood.

According to maker Novartis Oncology, exjade, which is taken"

FDA Advisory Committee: "Novartis Exjade Phase IV Commitments To Be Discussed By Committee

FDA's Pediatric Oncology Subcommittee will discuss Phase IV commitments for Novartis’ oral iron chelating agent Exjade (deferasirox) on March 14.

The once-daily oral iron chelator was given an accelerated approval in November based primarily on data from adult patients with ß-thalassemia.

At the Sept. 29 meeting of FDA’s Blood Products Advisory Committee, members requested that additional studies be done with Exjade in patients with sickle cell disease, myelodysplastic syndromes, or other hemotrophic disorders.

Novartis’ post-marketing commitments for Exjade required under accelerated approval include: establishing a registry for 200 children ages two to six years and following them for five years; completing ongoing four-year extension studies; and conducting a single-arm study in patients with congenital or acquired anemias and chronic iron overload.

The accelerated approval commitments also include the submission of a full study report (including safety and efficacy data) from a study in patients with sickle cell disease and a proposal for ascertaining iron concentration and cardiac function in patients on Exjade.

Other postmarketing studies requested by FDA for Exjade, which are not a condition of accelerated approval, include: a study t"