CMC to do successful BMT for thalassaemia

Thalassemia (also spelled Thalassaemia) is an inherited autosomal recessive blood disease. In thalassemia the genetic defect, which could be either mutation or deletion, results in reduced rate of synthesis or no synthesis of one of the globin chains that make up hemoglobin. This can cause the formation of abnormal hemoglobin molecules, thus causing anemia, the characteristic presenting symptom of the Thalassemias. 

According to Wikipedia Thalassemia is a quantitative problem of too few globins synthesized, whereas sickle-cell anemia (a hemoglobinopathy) is a qualitative problem of synthesis of an incorrectly functioning globin. Thalassemias usually result in underproduction of normal globin proteins, often through mutations in regulatory genes. Hemoglobinopathies imply structural abnormalities in the globin proteins themselves. The two conditions may overlap, however, since some conditions which cause abnormalities in globin proteins (hemoglobinopathy) also affect their production (thalassemia). Thus, some thalassemias are hemoglobinopathies, but most are not. Either or both of these conditions may cause anemia.

The two major forms of the disease, alpha- and beta- are prevalent in discrete geographical clusters around the world - probably associated with malarial endemicity in ancient times. Alpha is prevalent in peoples of Western African descent, and is nowadays found in populations living in Africa and in the Americas. Beta is particularly prevalent among Mediterranean peoples, and this geographical association was responsible for its naming: Thalassa (θάλασσα) is Greek for the sea, Haema (αἷμα) is Greek for blood. In Europe, the highest concentrations of the disease are found in Greece, coastal regions in Turkey, in particular, Aegean Region such as Izmir, Balikesir, Aydin,Mugla and Mediterranean Region such as Antalya, Adana, Mersin, in parts of Italy, in particular, Southern Italy and the lower Po valley. The major Mediterranean islands (except the Balearics) such as Sicily, Sardinia, Malta, Corsica, Cyprus and Crete are heavily affected in particular. Other Mediterranean people, as well as those in the vicinity of the Mediterranean, also have high rates of thalassemia, including people from the West Asia and North Africa. Far from the Mediterranean, South Asians are also affected, with the world's highest concentration of carriers (16% of the population) being in the Maldives.

The thalassemia trait may confer a degree of protection against malaria, which is or was prevalent in the regions where the trait is common, thus conferring a selective survival advantage on carriers (known as heterozygous advantage), and perpetuating the mutation. In that respect the various thalassemias resemble another genetic disorder affecting hemoglobin, sickle-cell disease.you may see more detail with a click here.

This Article is to create awareness in the public regarding thalassaemia and stem sells transplant Dr.Joseph John said that there is need for proper education of masses to create awareness regarding thalassaemia. 

Beta-thalassemia (β-thalassemia) is a form of thalassemia due to mutations in the HBB gene on chromosome 11 ^[1], inherited in an autosomal recessive fashion.

The severity of the disease depends on the nature of the mutation.

• Alleles without a mutation that reduces function are characterized as (β).
• Mutations are characterized as (β^o) if they prevent any formation of β chains.
• Mutations are characterized as (β⁺) if they allow some β chain formation to occur. (Note that the "+" in β⁺ is relative to β^o, not β.)

In either case there is a relative excess of α chains, but these do not form tetramers: rather, they bind to the red blood cell membranes, producing membrane damage, and at high concentrations they form toxic aggregates.Wikipedia also informs in detail about Thalassemia major and Thalassemia minor. Click on the link Please.

 Talking about this problem  Dr M Joseph John. MD. DM  of CMC Hospital  says that the thalassaemia prevalence in India is 2-14% in different regions of the country.  In Punjab the prevalence reported is 5 - 6.5%.  Although measures are being taken to curtail the incidence of thalassaemia major babies born in the state, the existing patients find it difficult to sustain themselves with regular blood transfusions and iron chelation as the average cost of transfusion-chelation in the country is about 2-4 lakhs/year depending on the age and weight. The average life span of a thalassaemia major patient in India is approximately 20-25 years before he succumbs to health problems related to iron overload or infections related to blood transfusions. 

                     

The only curative treatment for thalassaemia is allogeneic stem cell transplantation if there is a HLA identical sibling or family member.  If done between the ages 2-7 years, the success rate for this procedure is 80 -90%.  The cost of the transplant which is approximately Rs 8-10 lakhs per patient is equivalent to looking after a thalassaemia major patient for 5-8 years with supportive care.

                      

CMC Ludhiana is first institution in Northern India to do successful allogeneic stem cell transplant for thalassaemia patients and the only institution to offer transplants for paediatric patients. So far CMC done a total of 17 transplants between the ages 1 ½ to 60 years.  Of them 5 were for patients with thalassaemia patients aged, 8, 13, 14 and 18 years. 

Common questions and answers about thalassaemia transplant.

Ques 1:  Who can be a donor of stem cells for thalassaemia transplant?

Ans:   Patient’s brother or sister has a 25% chance of being an HLA identical stem cell donor. There is approximately 5% chance that a parent can be a match.

Ques 2:  What are the possible problems in the donor?

Ans :  Stem cell donation is a safe procedure and the donor need to be hospitalized for only 1 day. Stem cells are either taken through the peripheral vein in the hand or directly from the bone marrow.

Ques 3:  What is the hospital stay required for transplant?

Ans:   Patient needs to be hospitalized in specially constructed HEPA filtered room for approximately 1 to 1 ½ months.

Some other organisations are working for this noble cause.

Thalassemics India,an NGO founded in 1986, is striving to ensure that every thalassemic child gets proper medical treatment.It is an organisation that helps thalassemics to LIVE not just EXIST. 

 Foundation Against Thalassaemia is a registered NGO based at Faridabad Haryana India. Foundation was set up in 1995 and was registered under societies act 1860/21 on 24 April 2001 by a group of parents & charitable members of community. Foundation received no subvention [grant] from Government over the years, Foundation had been inspired to work towards improving clinical care, enhance public awareness of Thalassaemia. This is to ensure that children born with Thalassaemia major can grow in the healthy and happy environment. Its members are engaged in various activities aimed for 'Prevention and Treatment' of a dreaded disease Thalassaemia. 

The U.K. Thalassaemia Society has been in existence for 30 years and has amassed a wealth of experience in Thalassaemia not only in the U.K. but through its network and associations with other countries.

For More detail in Ludhiana and other parts of northern parts of India, needy persons may  contact Dr M Joseph John, MD, DM

Associate Professor
Clinical Haematology, Haemato-Oncology
& Bone Marrow Transplant Unit
Christian Medical College
Ludhiana-141 008
(M): 08054959525
(O): Direct: 0161-5037957 or 0161-2600270 Ext 4823/5022
Fax:0161-2600270

Details compiled and presented by Rector Kathuria & Shalu Arora

Amnesty International Urges Italy

Washington, DC : Amnesty International has urged the Italian authorities to deal with the humanitarian needs of thousands of migrants and asylum seekers leaving Tunisia following recent unrest.
More than 5,000 migrants have landed on the Italian island of Lampedusa, according to authorities, who have described the events as a "humanitarian emergency."
"A 'humanitarian emergency' demands a humanitarian response, not a law and order one," said Nicola Duckworth, Amnesty International's Europe director.
"This means that boats carrying migrants and asylum seekers from Tunisia, Egypt or other North African countries must not be pushed back," continued Duckworth. "Everyone arriving is entitled to be treated with dignity, to be granted assistance and access to a fair asylum procedure."

However, the country's foreign ministry has suggested that Italy and other E.U. countries should "stop the Tunisian wave," and recently deployed more boats to patrol the country's coasts.

Amid increasing tension over the issue, on Monday night Italian border police reportedly shot at a boat carrying Egyptian migrants and asylum seekers, wounding the vessel's pilot.

Italian authorities have reinforced the patrolling of the coast following the wave of migration in the Mediterranean.

Large numbers of people were forced to sleep outdoors for several days before the Italian authorities reopened the reception center on Lampedusa.

Italy routinely detains irregular migrants, and in the past has conducted mass expulsions, in violation of international law.

"Detention should only ever be a last resort, not an automatic response," said Duckworth. " No one should be sent back before they are able to make their case or explain their circumstances before the relevant authorities, and certainly there should not be any mass forced expulsions."

Frontex, the European Union's agency for external border security, has agreed to support Italy's calls for assistance in stemming the flow of migrants from North Africa.

"The main objective of any patrolling of national or international waters carried out by Italy, Tunisia or any other force, such as Frontex, should be the safety and security of those found at sea," added Duckworth.

Amnesty International is a Nobel Peace Prize-winning grassroots activist organization with 3 million supporters, activists and volunteers in more than 150 countries campaigning for human rights worldwide. The organization investigates and exposes abuses, educates and mobilizes the public, and works to protect people wherever justice, freedom, truth and dignity are denied.

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DOD Strives to Strengthen, Empower Military Families

Fri, Feb 18, 2011 at 5:13 AM By Elaine Wilson of American Forces Press Service WASHINGTON, Feb. 17, 2011 - From education opportunities to spouse employment, Defense Department officials are expanding military family support programs to better meet families' current needs, as well as to empower them for the challenges that lie ahead, the DOD official who oversees military family programs said today. Robert L. Gordon III, deputy assistant secretary of defense for military community and family policy, and Karen Guice, executive director of the Federal Recovery Coordination Program, take questions during the Congressional Military Family Caucus Kickoff in the U.S. Capitol building in Washington, D.C., Feb. 17, 2011. DOD photo by Elaine Wilson  "It's not just about providing fish -- it's teaching to fish as well," Robert L. Gordon III, deputy assistant secretary of defense for military community and family policy, told an audience of politicians, spouses and service organization leaders during the Congressional Military Family Caucus Kickoff in the U.S. Capitol building here. The caucus' goal is to educate Congress members and staff about the unique challenges –- including mental health, wounded warrior care, health care, education and disability –- service members and their families face. The military community is diverse and vast, Gordon said, with more than 2 million service members, 1.59 million military-connected children and nearly 800,000 spouses. "The question is: How do we look at that community and address their needs, but also empower them for the future?" he said. The nation is an era of "fiscal austerity," Gordon acknowledged. However, he added, families continue to have the same needs and concerns, including education, behavioral health support, employment and community reintegration. Gordon called for an increase in DOD partnerships to bolster family support in the years ahead. The department must continue to foster relationships with nonprofit and military spouse groups and the commercial and entertainment sectors, he added. Leadership also is "absolutely key" when addressing the military community's most-pressing issues and concerns, he said, but it will take more than just Defense Department leaders to do the job. Government and nonprofit group leaders and senior military spouses, Gordon said, must be "focused like a laser beam on the issues and concerns of our military community." Families have a plethora of support programs available to them, but leaders must ensure those programs are effective and adequately address needs, he added. Toward that end, Gordon touched on a few of the programs and initiatives the department is looking at to increase education and career opportunities. The Defense Department, for example, plans to conduct an education review to ensure a "world-class" education for military children. Of the 1.2 million military-connected children in schools, roughly 85,000 are in DOD schools, about 70,000 are in public schools on military installations and the rest, Gordon noted, attend off-base public schools. The 172-day review will look at science, technology and engineering, math, early child education and languages, he said, to ensure all 1.2 million military children are being adequately prepared for 21st-century demands. For younger children, Gordon said the department is working to expand the number of child care slots within communities. The Defense Department has 923 child development centers with 200,000 young children needing care and a shortfall of about 30,000 slots, he said. To alleviate the shortfall, Gordon said, DOD officials have partnered with 13 states to increase military family access to community-based, quality child care. This focus on community care makes sense, he noted, since roughly 70 percent of families live off base. "We have to have partnerships where we provide those sorts of resources where the communities are," he said. Officials also are focused on improving spouse employment opportunities, Gordon said. Of the 800,000 military spouses, about 77 percent would like work, he said. However, he acknowledged, spouses are hindered by frequent transitions and deployments. The department is engaging in a spouse education and career opportunity initiative to alleviate some of those employment challenges, Gordon said. The initiative includes expanding the Army Spouse Employment Program into the Military Spouse Employment Program so all spouses can benefit, he said. The Army's program helps connect spouses with opportunities in Fortune 500 and Fortune 100 companies. These are just a few of the programs the department is working on, Gordon said, noting many others are outlined in the White House report, titled: "Strengthening our Military Families: Meeting America's Commitment." This report describes the sweeping interagency effort under way to better support military families. Federal agencies, he added, have made nearly 50 commitments in terms of family support. Moving ahead, Gordon said it will take a mix of innovation and creativity to meet military families' needs, while ensuring they're empowered "to be the best they can be."

Biographies: Robert L. Gordon III Related Sites: Special Report: Military Family Support  Special Report: Strengthening Our Military Families  Related Articles: DOD Expands Community-based Child Care Options

Marine Plans Rebound From Injuries

Thu, 17 Feb 2011 10:01:00 -0600

By Marine Corps Pfc. Franklin E. Mercado of 1st Marine Logistics Group CAMP LEJEUNE, N.C., Feb. 17, 2011 - Marja, Afghanistan, was the site of many deaths and injuries while Marines and other coalition forces seized the city early last year to rid it of Taliban forces. Even now, a year after the initial assault, the urban center remains a dangerous region of Helmand province. Marine Corps Lance Cpl. Brian K. Steele, left, speaks with Marine Corps Col. Kenneth Enzor, chief of staff for the 1st Marine Logistics Group, during a ceremony in which Steele received the Purple Heart Medal at Camp Lejeune, N.C., Feb. 14, 2011. Steele was a vehicle commander during a convoy near Forward Operating Base Hansen, Afghanistan, when his vehicle struck a roadside bomb. U.S. Marine Corps photo by Pfc. Franklin E. Mercado  Marine Corps Lance Cpl. Brian K. Steele, a native of Paris, Ill., can testify to the still-dangerous environment around Forward Operating Base Hansen, one of the many coalition outposts that now dot the city. On Jan. 22, Steele, who commander the sixth vehicle in a 17-vehicle convoy, was near Forward Operating Base Hansen when his vehicle struck a roadside bomb. He was wearing all of his protective equipment, but the blast left him in serious condition. Steele, a combat engineer with the 1st Marine Logistics Group's 8th Engineer Support Battalion, suffered injuries to his cheekbone and hip joint area, among other fractures. The experience is something he says he will never forget. "Getting blown up will stay with me for the rest of my life," Steele said. "It's a life-changing experience, obviously, but I'm fine, and that's what's important." After the explosion, Steele was taken to Camp Bastion. Soon thereafter, he was admitted to Landstuhl Regional Medical Center in Germany, and he subsequently was taken to the National Naval Medical Center in Bethesda, Md. Now on convalescent leave, he said he's taking the rest of his life one step at a time as he continues to recover from his injuries. Despite his injuries, Steele said, his morale has not been shaken. The self-proclaimed trail blazer, who received the Purple Heart Medal on Feb. 15, said he has a plan and is not going to let something like a combat wound keep him down for long, and that he hopes to make a full recovery and return to duty. "I like following my own path," he said. "I make my own decisions. Even growing up, I liked to do my own thing."

Related Sites: NATO International Security Assistance Force

During a joint operational access exercise

U.S. Army paratroopers with the 82nd Airborne Division sit in an Air Force C-17A Globemaster III before an airdrop during a joint operational access exercise (JOAX) at Pope Air Force Base, N.C., Feb. 9, 2011. JOAX is a joint Army and Air Force training exercise held to practice large-scale personnel and equipment airdrop missions. (DoD photo by Staff Sgt. Greg C. Biondo, U.S. Air Force/Released)

During reaction force training

U.S. Navy Information Systems Technician Seaman Erick Martinez provides security for his teammates during reaction force training aboard the U.S. 7th Fleet command ship USS Blue Ridge (LCC 19) in Yokosuka, Japan, Feb. 7, 2011. The scenario is part of the final exercise during ship's reaction force Bravo Training, a force protection exercise to prepare Blue Ridge Sailors to react to threats to the ship. 














(DoD photo by Mass Communication Specialist 2nd Class Steven Khor, U.S. Navy/Released)

Top official warned of a disruption in war funding

Wed, 16 Feb 2011 08:13:00 -0600

By Matthew Mientka of Emerging Media, Defense Media Activity

WASHINGTON, Feb. 16, 2011 - While acknowledging a tougher financial reality, the Defense Department's top financial official yesterday warned of a disruption in war funding and other likely effects if Congress continues to force the military to operate on continuing resolutions instead of fully funded budgets for fiscal 2011 and 2012.

In a "DOD Live" bloggers roundtable yesterday, Pentagon Comptroller Robert F. Hale explained President Barack Obama's $671 billion fiscal 2012 budget request for the Defense Department and detailed potential effects if the military must continue operating without a fully funded fiscal 2011 budget.

Although DOD officials continue to look aggressively for opportunities to cut and save, Hale said, Congress must give the department sufficient funding. Budgetary delays with continuing resolutions, he added, would pose a national security problem.

"We face a significant problem in the current fiscal year," he said. "We are on a continuing resolution, ... and a year-long continuing resolution would pose serious problems for us. It wouldn't provide enough resources to properly meet our national security requirements [and] would feature a $23 billion cut compared to our proposal."

Hale said funding via continuing resolution would limit the military's discretionary spending power, reducing managerial flexibility and the procurement of assets. Some 50 "shovel-ready" military construction projects continue to be delayed by continuing resolution funding, he noted, and the Army recently froze hiring of civilian personnel, leaving positions vacant for tank mechanics and the like.

Hale said the department requires "reasonable funding" to avert a fiscal crisis.

The fiscal 2012 budget request includes $533 billion in discretionary spending authority, with another $118 billion to support operations in Afghanistan and Iraq, compared with an authorization this year of $725 billion that included an additional $41 billion for the war effort.

Total defense spending would decline by $15 billion to $35 billion, depending on whether Congress funds war spending — beyond the normal budget — with a continuing resolution.

At the very least, the Pentagon requires $540 billion in fiscal 2012, Hale said, noting that the "reform agenda" of the new Congress has proposed funding of about $532 billion.

"While we're accomplishing our national security goals," he said, "we recognize the country has got serious economic problems, and we are working to tighten our belt."

Toward that end, Hale said, Pentagon officials continue to reform the budget and acquisitions process. Aside from a 10 percent cut in contracting services that would save $20 billion or so per year, he said, the military proposes cuts to ground forces to save another $6 billion over a five-year period. In a multi-year plan, Hale added, the military wishes to cut Army end strength by 27,000 while shedding 15,000 to 20,000 positions in the Marine Corps.

In addition, he said, the department intends to cut the Marine Corps' expeditionary fighting vehicle, but would maintain the service's amphibious capability with a cheaper vehicle and upgrades to the existing fleet.

Hale also said the military would continue to modernize equipment as planners continue to focus on current wars, ensuring proper resources for intelligence, surveillance, reconnaissance, rotary-wing aircraft and whatever it takes to build "partnership capacity" in the war zones of Afghanistan and Iraq.

"We're modernizing broadly for future conflicts, [with] aggressive modernization of our tactical air fleet, our ships, a new bomber program, a ground vehicle program and, finally, supporting our deployed troops — especially those in war zones — with all the resources they need, including financial resources," he said.

In fact, he noted, the imperative to "support the troops" represents the most dramatic change to the defense budget from this year to the next. The Pentagon proposed spending $8.8 billion next year for family support programs, with about $500 million in funding shifted from existing wartime funds.

"As we invest these dollars to meet our national security requirements, we follow several themes, [but] our most important goal is to take care of our people," Hale said. "We propose a pay raise of 1.6 percent, about $8.3 billion in family support spending, and substantial funding for our military health care."

Still, Hale added, Pentagon officials would be looking for cost savings within the Military Health System budget, noting officials likely would increase TRICARE Prime health plan enrollment fees for working-age military retirees modestly -- by about $5 per month for families and $2.50 per month for single beneficiaries. The fees, which haven't changed in 15 years, would then be tied to a health care index for future inflationary increases, Hale said.

Also, he said, the military gradually would eliminate subsidies for civilian hospitals providing military health care, creating a single tier of payment for hospitals. Officials also would increase pharmacy users' co-payments while encouraging beneficiaries to fill their prescriptions by mail and to request generic drugs.
 

Biographies:

Robert F. Hale 

Related Sites:

"DOD Live" Bloggers Roundtable