Renowned hematologist Dr. Edward Shanbrom dies

The Associated Press

Posted:   03/04/2012

TUSTIN, Calif.—Dr. Edward Shanbrom, a pioneering hematologist who invented a widely used process for removing viruses from blood plasma and helped develop a breakthrough treatment for hemophilia, has died in Southern California.

Shanbrom died of natural causes Feb. 20 at his home in Tustin, according to a statement from his family. He was 87.

While on the executive staff at Baxter Laboratories in the 1960s the Connecticut native helped come up with a method, still in use today, to produce large quantities of a blood clotting factor that is absent in hemophiliacs. This breakthrough therapy made it possible for those suffering from hemophilia to lead more normal lives.

“Hemophilia patients and blood product recipients worldwide have benefited from Dr. Shanbrom’s work,” Samuel D. Anderson, his colleague and a former biotechnology executive, said in a statement to the Los Angeles Times.

In the 1980s, while conducting research at his home, Shanbrom developed a blood purification technique that uses mild detergents to scrub viruses, bacteria and other contaminants out of plasma.

Shanbrom held 49 U.S. patents and 91 international patents in areas ranging from nutraceuticals to antiseptics to blood products, his family said. In 1988 the New York Blood Center bought his patented processes for scrubbing viruses from transfusion blood.

He practiced hematology and oncology at the City of Hope Medical Center in Duarte, Orange County General Hospital and St. Joseph Hospital in Orange.

Shanbrom was also a clinical instructor at the University of California, Los Angeles and at UC Irvine. In 2007 the Edward Shanbrom, M.D. Hall was dedicated on the UC Irvine campus in recognition of his pioneering hematology research and support of the university. The building is also home to the Edward Shanbrom, M.D. Laboratory for the Study of Blood and Natural Products.

For more inforamation on Edward Shanbrom , M.D. click here.

Conflicts in medical management of hemophilia

entire Article can be found here: Conflicts in medical management of hemophilia.

 By Everett Winslow Lovrien, M. D  (Author of “Doctor Guilt?“)
WordPress blog

Hemophilia is a medical disorder that is part of our genetic load as humans. It will never disappear. Therefore, the only way to live with it is to receive adequate medical management. Its effects on those males who have inherited the disorder that affects their blood clotting without treatment includes recurrent hemorrhages (bleeds) into joints as well as internal bleeding. The bleeds are painful, resulting in disuse, disability and crippling. The first signs usually appear in early childhood. A child who suffers a mouth bleed can slowly lose enough blood to result in death.

Prior to 1960, the expected life of a child born with severe hemophilia in the USA was less than 11 years. A common cause of death in childhood was bleeding into the brain, an intracerebral hemorrhage. This agonizing threat could be overcome with the discovery of Cryoprecipitate in 1964 by Dr. Judith Pool at Stanford University. The missing precursor to deficient clotting factor, antihemophilia factor VIII (AHF VIII) was obtained from the blood plasma of normal donors. A further step led to the production of AHF in the form of a lyophilized powder (Concentrates) that could be re-constituted and infused at home to treat bleeding. The introduction of concentrates was a marvelous innovative form of treatment that considerably improved the lives of those born with hemophilia by eliminating agonizing suffering.

Constraint in hemophilia treatment by HIV and hepatitis viruses

Despite the great success of Concentrate therapy for replacement of missing AHF in persons who have hemophilia, two conflicts have clouded the brightness of its use. The first major hazard of replacement therapy was the discovery that the magic medicine was contaminated with hepatitis viruses and HIV, the virus that cause AIDS. The drug companies that manufactured AHF Concentrates derived from plasma sacrificed safety bypassing purification in their drive for profitability. Thousands of boys and men who had hemophilia died all over the world from liver failure and AIDS as a result of the polluted medicine they infused to treat their bleeding episodes. More than 10,000 persons with hemophilia became HIV infected in the USA, which could have been prevented.

High costs limit availability of hemophilia treatment

The second conflict in AHF replacement is the cost of the medicine. AHF infusions to treat or prevent bleeds in hemophilia are not infused just once; instead infusions are required every few days in childhood. A person with severe hemophilia often exceeds 1,000 infusions by adulthood. The cost of treatment depends on the amount of AHF infused. A child requires less than an adult. A single treatment costs $1,000 to $2,000 for an adult. In the USA, estimates reveal that 12,000 persons with severe Type A hemophilia infuse an average of 171,760 units of AHF each year totaling 2,060,000,000 AHF units, including surgery. The average maintenance per person without surgery is less. However, recent recommendations for the prevention of bleeding in hemophilia include scheduled prophylactic infusions of Factor VIII, three times each week. A person who receives 150 infusions each year at 750 units per infusion will receive 112,500 AHF units; if the amount is 1,000 units three times each week, the yearly infusions total 150,000 units. The pharmaceutical manufacturing of AHF concentrates, as plasma derivatives and by genetic recombinant methods, world-wide is a multi-billion dollar industry. Production of AHF in 1995 equaled $2.4 billion, and amounted to $6 billion by 2011. AHF concentrates are sold primarily in countries with high incomes (RE: J.S. Stonebraker et al, Hemophilia (2010), 16, 33-46).

Hemophilia appears in males from all of the world’s races. With a world population of 7,000,000,000 (CIA, 2010) and an average birth rate of 20 per thousand individuals, there are 140,000,000 births each year in the world. One half are males, (70,000,000). One in 5,000 newborn males has hemophilia, equaling 14,000 new cases of hemophilia that are born in the world each year.

Within the U.S., with a population of 310,000,000, a birth rate of 14 per thousand equates to 4,340,000 births each year. In the U.S. 2,170,000 males are born annually. Experience has revealed that one in 5,000 will have hemophilia, equaling 434 new cases of hemophilia in the USA each year. Approximately 20,000 persons living in the USA have hemophilia ( RE: J.S. Stonebraker et al Hemophilia (2010), 16, 20-32). Of these, 12,000 are males who have severe type A hemophilia requiring Factor VIII replacement infusions.

Thanks to the federal government’s establishment of Hemophilia Treatment Centers, whose goal is to identify all of the persons who have hemophilia within the center’s health region, most cases of hemophilia in the USA, as well as in other high income countries are identified. Recognition is a necessary step that leads to treatment and relief from suffering.

But, that’s not the case in the major parts of the world. It is estimated that one–half, or even three-fourths of the children born in the world who suffer from hemophilia receive inadequate or no treatment (World Federation of Hemophilia, WFH). Often, they are not identified. The lack of treatment in economically poor counties of the world is the result of the high cost of the replacement AHF medicine, made in America without cost controls, that is not affordable in many countries.

Information provided by Mr. Patrick Robert of Marketing Research Bureau, Orange, Connecticut, for 2009 revealed the following:

Factor VIII sales in the USA (2009)
Plasma derived AHF Factor VIII	360,000,000 units
Recombinant AHF Factor VIII	1,700,000,000 units
	Total 2,060,000,000 units

 

Factor VIII worldwide sales (2008)
Plasma derived AHF Factor VIII	2,900,000,000 units
Recombinant AHF Factor VIII	4,000,000,000 units
	Total 6,900,000,000 units

 

Price of Factor VIII
Recombinant Factor VIII	$.90 -$.99 / unit
Plasma derived AHF Factor VIII	$.60/ unit within the USA
	$.50/ unit outside the USA

The USA includes only 4.7% of the world’s population but it uses 30% of the Factor VIII concentrates produced in the world. 12,000 males (Type A hemophilia) using 2.06 billion units of AHF annually average 171,670 units for each person. This amount of AHF appears to be excessive but considering the large amounts of AHF utilized during surgery, prophylactic infusion therapy three times each week, and induction of immune tolerance to overcome inhibitors the amount may be even higher. Mr. Mark Skinner, president of WFH, believes the utilization of AHF will increase as children in the USA, who have been maintained on prophylaxis become adults. An adult male weighing 175 lbs who infuses 2,000 units of Factor VIII prophylactically three times each week uses 312,000 units each year, at a cost of $187,200 to $308,880 depending on whether recombinant or plasma-derived Factor VIII is infused.

Donations of AHF from pharmaceutical manufacturers to underserved person have been welcome. Pfizer has provided 10 million units of its ReFacto AHF to persons suffering from hemophilia in underserved countries, valued at $13 million (Medical.net/news 2010-02-03). Medical insurance providers may question the high prices of AHF Factor VIII they pay to pharmaceutical manufacturers for the benefits of their subscribers while they give away medicine to others without a cost. There remains a dilemma – a marvelous treatment exists but it is not affordable for most persons in the world. AHF treatment is available for persons who live in rich countries—so, a societal issue arises. If medicine exists to provide treatment, relieve pain and suffering, prevent disability and prolong life expectancy, is it a basic human right that the medicine should be available to everyone who needs it?

WFH and the National Hemophilia Foundation (NHF) continue their advocacy for all persons born with hemophilia. The Hemophilia Treatment Centers (HTC) in the USA have improved the management of hemophilia resulting in an improved quality of life and increased life expectancy within the USA. Other countries that have a high average income have also experienced the improvement of hemophilia care as the result of the pharmaceutical manufacturers’ entrepreneurship. The innovative production of AHF for the treatment of hemophilia as a safe plasma derivative and by genetic recombinant methods by industry has been remarkable. The challenge ahead for treating hemophilia has shifted from production of medicine to distribution.

Blood Donor Deferral Policy

March 19, 2010

The National Hemophilia Foundation (NHF) believes that any decision to revise current blood donor deferral policies must put safety first and be made solely on the basis of scientific evidence. For this reason, NHF will be an active participant in any review and discussions on changing the donor deferral policy for men who have had sex with men (MSM) even once since 1976.

The bleeding disorders community is heavily reliant upon blood and plasma donations for the manufacture of the essential life-saving therapies on which we depend. We are grateful to all those who donate—without their generosity, treatment would not be possible for many in our community. We welcome discussions on ways to improve the safety of the nation’s blood supply not only for the bleeding disorders community, but for all who may need a blood transfusion at some time, be it for surgery or an emergency situation.

NHF asserts that any proposed changes to current donor deferral policies must satisfy the Precautionary Principle.  In this context, the Precautionary Principle implies that decisions must err on the side of caution in the absence of a scientific consensus that harm would not result from changing these policies. Unless there is evidence that a proposed change will not elevate the risk to the nation’s blood supply, we must maintain current policies to protect the recipients of blood and blood products.

It is critical to remember that our community has been devastated by death and illness resulting from contaminated blood products.  People with bleeding disorders, including hemophilia A and B, von Willebrand disease and other rare bleeding disorders, continue to depend on a safe blood supply to lead healthy lives.  We must avoid repeating our tragic history, which will only be possible through constant vigilance and application of the Precautionary Principle to scientific review.

NHF looks forward to participating in the discussions of the Advisory Committee on Blood Safety and Availability of the Department of Health and Human Services and with others on the existing donor deferral policies related to MSM to determine whether the current state of science supports modifications to the existing policy.

NHF Statement, here.

WHF Statement, here.

Life of the Edge – Hemophiliac living life

The article below just goes to show how much progress has been made in Hemophilia research and how much farther we have to go.  Three cheers for those like Justin are taking the lead!

Hemophiliac happy to live on the active edge

Taken from Straight.com<

By Gail Johnson

Justin Tanguay works hard and plays hard. At 29, the former RCMP computer programmer is a heli-ski guide who also works on avalanche control. When he’s not cruising down steep grades or mountain biking down equally treacherous terrain, he works as a contractor in the Kootenays, where he’s building dozens of homes.

With his penchant for rough-and-tumble activities, the Ontario native might seem like any other hard-core outdoorsy type. But what’s staggering about Tanguay’s ability to conquer mountains—and his desire to live on the edge—is that he has hemophilia.

Contrary to popular thinking, people with the hereditary condition do not bleed more profusely than others. However, they lack certain proteins (called factors) that are necessary for clotting blood. So what would be a minor injury to anyone else could cause a hemophiliac to experience serious, even life-threatening, internal bleeding in the joints, muscles, tissues, and organs.

Among the symptoms in a baby is bleeding in the gums and tongue during teething. In older kids and adults, some of the signs are bleeding in the mouth after losing a tooth or biting the tongue, and blood in the urine.

Tanguay’s mom is a carrier of the genetic disease, which is more common in men than women, so she knew there was a 50-50 chance her first son would acquire it. (Tanguay’s younger brother doesn’t have it.) But she wasn’t about to let the diagnosis deprive her little boy of a fun-filled childhood.

“The doctor didn’t encourage me to do any physical activity, not even ride a bike,” Tanguay says on his cellphone. “My mom basically flipped him the bird and said I was going to do whatever all the other kids were doing. It was as if I should have been on a leash tied to my mom to make sure I wouldn’t get hurt.”

Tanguay got onto a bike and into ski boots at an early age, even going along with his elementary-school class for its weekly trips to the slopes. His parents were often by his side, teaching him to play it safe—but to still play.

“It was, ‘Mountain biking is okay, but maybe it’s not okay to jump six feet in the air off the deck,’ ” Tanguay explains. “It was about knowing my limitations and my capabilities. All my friends and coworkers knew about my hemophilia, so nobody ever egged me on to do something I didn’t want to do. If I was mountain biking and there was an area where I could crash and get seriously hurt, I just wouldn’t do that part.

“If I hit a tree with my knee, it would hurt just as much as the other guy; we’d both get internal bleeding, but mine isn’t going to stop and his is.”

There are two types of hemophilia—A, which is more common, and B (which Tanguay has)—and the condition as a whole is classified as mild, moderate, or severe. The extreme forms affect mostly men.

According to the Canadian Hemophilia Society, there are about 2,500 with hemophilia A and roughly 500 with type B.

Tanguay’s active lifestyle hasn’t been without mishaps. As a child, he regularly had to go for three-day stays at the hospital, where he’d receive blood transfusions.

But treatment has come a long way since he was a kid. What used to take 72 hours in hospital now can be done in 15 minutes in his own home, Tanguay says, referring to the freeze-dried concentrates of clotting factors that he can administer himself intravenously. He’s usually back up and at ’em within a day.

Tanguay—who says he skis about 150 days a year and has guided snowcat-skiing expeditions in the Selkirk Mountains and led three-week-long ski trips in Alaska—says that what hasn’t changed much is so many misconceptions about the condition. As a result, he doesn’t tell most people right away that he has hemophilia.

“There were times when I’d go for a job on a construction site and they’d say, ‘Here’s a mop.’ I’d restrain myself from telling people until they could see what I could do.

“As hemophiliacs, we’re big bleeders, but we know how to handle our situations, and as adults we know our capabilities,” says Tanguay, who was declared a “hemophilia hero” by the World Federation of Hemophilia on last month’s World Hemophilia Day. “I still try to push the limits within a safe realm.…When people meet people with hemophilia, they instantly judge them. Ask them about it instead. Really talk to them and ask about their situation and their case.”

In the late ’70s and early ’80s, Tanguay says, hemophilia was often hush-hushed because of the tainted-blood scandal, in which viruses like HIV and hepatitis were spread through blood products.

Hemophilia “was a bad word”, Tanguay says, quickly adding that he feels completely safe using the blood products available today. In the early 1990s, safer concentrates were developed; they’re either purified with monoclonal antibodies or genetically engineered.

The Canadian Hemophilia Society encourages hemophiliacs to get regular exercise. Physical activity helps keep the bones and joints strong, and being in good shape can help diminish the number of bleeding episodes.

The society categorizes different sports according to their risk factors on its Web site. Golf, tennis, and swimming are low-risk, for instance, while hockey, in-line skating, and alpine skiing fall in the high-risk zone.

For Tanguay, it could all be condensed into the words of that B.C. Lottery Corporation motto: “Know your limit; play within it.” He plans to keep on skiing, within bounds, of course—the mountains’ and his own.

Jonathan Wadleigh, a co-founder of the Committee of Ten Thousand (COTT), died

Wadleigh, along with Tom Fahey, founded COTT in 1989. Both men had severe hemophilia, and contracted HIV and hepatitis C from blood products. Wadleigh served as president until 1996. The grassroots organization was established to be the voice of the growing number of people with hemophilia who contracted HIV/AIDS through contaminated blood products.

For more information about Jonathan Wadleigh see the Boston Globe Article, here.

Without Men like him and others in our community where would we be.  Not as strong and not as wise as we would be without them.  it is now our turn to take up the call to action and be as he was – active and involved in pushing for our rights as a community.