Today many pharmaceutical companies have or are getting into the business of manufacturing Factor product...Read More
Sitting in front of my television set one night, I saw my reality on screen: A young man named Ryan White taking clotting factor for a bleed. Having never seen a movie showing someone with hemophilia having factor injected into his arm, just as I had done so many times...Read More
The extreme reaction of the Japanese government this summer to the discovery of a human vCJD case, in a person who visited the United Kingdom, albeit briefly, showed the strong desire of governments to minimize the risk of infection, particularly infection of national blood supplies.
Between what seems like a steady drumbeat of new countries and parts of the world revealing the presence of human or animal TSEs, we at COTT are reminded of one of the fundamental rules in science: in the face of uncertainty, do not presume to be too restrictive.
In 1999 essentially all donor deferrals were dropped concerning individuals and families with diagnosed or symptomatic classical CJD, as it was not proven to be cattle-related, remained a disease of older age groups, and was not found in blood.
Statistics from the UK CJD Surveillance Unit show a “bell curve” for the rise and fall of bovine disease, peaking in about 1985, and of human vCJD there as well, peaking about ten years later. However, the “tail” of that curve cannot be correctly perceived due to the multi-year process of case investigation. Some experts also note that it won’t go to zero since some infection continues to be found.
Families of classical CJD victims, the disease widely held to be a background-level concern, with a incidence rate of 1:1,000,000, now feel more strongly than even a few years ago that change is occurring, that the background level is rising. UK data shows an increase in the trendline of such cases, from 40 deaths per year in 1992 to 60 in 2004, a 50% rise in just over a decade.
To those who would say it is time to relax the CJD-related geographic donor deferral requirements of recent years, we at COTT say, it’s too soon. We point to the scientific principle above, we point to the troubling variations in cases of related diseases, in humans and in animals, we point to the lack of conclusive evidence regarding the transmission of vCJD to man, and we point, as other countries have with increasing urgency of late, to the importance of a pristine blood supply.
The two published studies of blood transmissibility of prion disease came out only in the last two years, causing rethinking of many of our protections to date, and the Hunter study on transmission from asymptomatic animals completed the alarm. Two years well after the peak of the UK vCJD curve, and almost twenty after the discovery of the prion. We obviously are still learning, have not taken the measure of this agent yet at all, and are likely to be dealt more surprises in the next five years. It would be very shortsighted to assume we knew everything about this threat and relax our guard anytime soon.
The large-scale USDA program to not find BSE cases in US cattle, and its refusal to permit tracing of infected meat when one does pop up, serve their intended purpose of maintaining complacency in the population, reinforcing as with many other things American the perceived differences of the USA from the rest of the developed world. Although it will be a bitter pill to swallow, we at COTT would hope that public pressure will force USDA to a more stringent screening, and the number of cases found will more realistically rise into the range of 20-40. At those numbers, we will not need to plead as loudly for actions to protect blood; all will respond.
VOL. 12 NUMBER 2 Originally published on 10-24-2005
The Food Chain from the Slaughterhouse to the Infusion Needle: The confirmation of the transmission of Mad Cow disease (CJD and variant CJD) to humans is now accepted based on events in Great Britain. Given that we have now transitioned from the “hypothetical” dangers
of Mad Cow transmission from cattle to humans to the reality of TSE (transmissible spongiform encephalopathy) transmissibility, it is imperative that the connection between food and blood be prioritized by those tasked with the regulation of America’s blood supply. At the ground floor is the reality that those who consume beef products also donate blood. The importance of that basic reality falls squarely on the shoulders of the Food & Drug Administration. With that in mind, the relationship between the regulation of the blood supply and the regulatory tracking of America’s cattle herds must be elevated to a much more integrated and comprehensive threshold. We also must be concerned about our deer herds and those that hunt and consume deer meat. These animals are also showing TSE and there is concern and those that hunt and consume them. While persons involved in public health cannot be expected to learn the specifics of agricultural animal management, some familiarization with these events is warranted.
Starting from the bottom or end result of the chain, for a person to receive the infectious agent for the disease from blood, it (the agent) must be:
- Present in donated blood (and, for plasma product users, not sufficiently removed in fractionation;
- Present in the donor’s bloodstream via consumption of infected food;
- Present in the food – beef cattle – through horizontal contagion from others, linking eventually back to the United Kingdom.
This last gives most people pause – the US stopped importing British beef years and years ago. But the Canada link was responsible for the first US case found. The second case was all ours. It’s here. And if our sampling system caught two, a fuller test might catch hundreds …
Most countries protect their populations from this disease by thorough testing of all or most cattle before slaughter. Usually this means testing only those older than 30 months, the time when detectable symptoms begin to appear. Determining the age of cattle in slaughter is based on presence of certain molars, not an ironclad system.
However the US doesn’t test all cattle over 30 months. We test less than one percent of them, plus any other beef cow which cannot walk or otherwise shows possible symptoms of BSE.
Instead, the US requires slaughterhouses to make extensive changes in the process, to assure that all cerebral and spinal materials are removed, so that theoretically even infected cattle can be processed into meat and sold in US stores but the meat will not be infectious.
This is similar to cutting the bad part out of an apple, or tearing the mold off the corner of a piece of bread, and consuming the remainder with no concern. Except for two things: 1) discovery, research on and handling of prions is so new it may be naïve to conclude which parts are infectious and which aren’t, and 2) the age determination and the removal of the potentially infectious material are performed by low wage employees, often with minimal training.
Oversight of these functions by USDA has been the linchpin in holding public confidence in the USDA plan to successfully protect our health. However, its efficacy is not widely reported, but anecdotal evidence abounds of its shoddy implementation. As one brief example, workers assigned to mark cattle when the indicated molars are found miss many so the packages coming out of the other end of the plant are wrongly labeled “USDA Inspected and Passed” and taken to market.
A few other examples of in-plant procedural inadequacies that allow risk materials to pass into the food chain:
Check every carcass for age
Requirements [Other Staff/Vets]:
Verify on paper and spot checks
Document violations of USDA rendering requirements
Provide line inspections when vacancies due to illness, etc.
Actual Procedure [Inspectors]:
Not trained or told to check every carcass
Actual Procedure [Other Staff/Vets]:
Not trained or told how to verify.
Spot checks as seldom as once a day.
Not allowed to record frequency of spot checks.
Only permitted if seen in person. For others, take word of employees over inspectors.
Not permitted to record violations observed when filling in for line staff.
Source: Public Citizen
So USDA inspectors are tightly constrained, subsidiary to slaughterhouse employees versus in a regulatory role over them, and, essentially, complicit in permitting much potentially infectious material to pass. There is no post-production test for presence of this material before packaging.
When you put into your arm weekly or monthly the distillation of the blood of tens of thousands of donors, you go to extreme lengths to advocate for its purity. USDA does not.
VOL. 12 NUMBER 1 Originally published on 10-24-2005
From the beginning, COTT’s guiding philosophy has been, Action=Life. The perspective is ongoing as COTT moves into its third decade as a grass-roots advocacy, support and policy organization serving the hemophilia and larger bleeding disorders communities. We are one of the few community advocacy organizations who do not accept support of any kind from the manufacturers of blood products, drugs or medical devices. This prohibition reflects our belief that conflict of interest played a key role in the AIDS/Blood epidemic that devastated the hemophilia community in the 1980s.
The survival of COTT is imperative if we are to continue to have an independent community voices participating in the regulatory structure as full stakeholders and partners. Your support is critical to our continued ground breaking work in blood safety and in the significant impact of trauma associated psychosocial challenges such as depression and Post Traumatic Stress Disorder, which our community members confront on a daily basis.
What can you do? Plenty. Send COTT a donation, by check in the mail or by clicking on "Donate" on the home page of our website, www.cott1.org. All amounts are welcome. Then talk to friends, neighbors, relatives about blood safety and ask them to donate with you.
Click here to download the latest issue of the COTT Washington Update, volume 14, number 4.
COTT was pleased to learn that Corey Dubin, President and Board Chair of COTT, has been appointed again to the consumer representative seat on the FDA's Blood Products Advisory Committee (BPAC). Corey was originally appointed to a term on the committee in 1995, just after the Institute of Medicine's report HIV and the Blood Supply: An Analysis of Crisis Decisionmaking -- calling for increased consumer voice on committees such as this one.
It was then-FDA Commissioner David Kessler who specifically instructed the committee's staff secretary to declare a consumer seat and place Mr. Dubin in it. His service there -- despite much early skepticism by the academic and industry-linked other members -- paved the way for all subsequent consumer representation, on BPAC and also on the Advisory Committee on Blood Safety and Availability, in the DHHS Office of the Secretary (on which Corey Dubin has also served).
For the second year in a row, COTT has served on the FDA committee to review nominations for Consumer Representative seats on all FDA Advisory Committees.
This installment of the hemophilia "archive e-newsletter" is dedicated to the memory of Loras Goedken and the Goedken family. I've also included an attachment containing a scanned image of the COTT memorial brochure. Contained in the "archive" is a video tape of a program entitled "Ed Bradley Street Stories", which aired on CBS in 1992. In the program, CBS reporter Richard Roth interviews Loras Goedken, from which the following exchanges are excerpted:
R. R. "This is factor eight and it is what infected Loras Goedken with HIV."
L. G. [seated in an examination room having blood drawn] "Every time I get an infusion, I'm tired of being a hemophiliac. Every time I have to run for a blood test, I'm tired of having HIV. But, ya' gotta' go on."
R. R. [narrative] "Goedken is from a large Iowa farm family."
L. G. [seated next to his mother, Mary at their Iowa home] "Mother had to write all the names down so she wouldn't forget us."
R. R. [narrative] "Six of Mary Goedken's seven sons were born with hemophilia. For much of their lives, the boys depended on transfusions of whole blood or plasma to stop severe bleeds. In the early '70s, factor eight changed everything. An infusion only took ten or twenty minutes, and it could be done at home."
R. R. [walking by Loras' side in a cemetary] "Who was the first to die?"
L. G. "From HIV? My brother Ernie. He was 47."
R. R. "And then?"
L. G. [standing in front of a long row of tombstones] "And then my nephew, Clayton at four and a half months old. Then Carl died; and Clayton's father, Denny; and then Jan, my wife, in April of '90; and then Clayton's mother; and then Jay died in August of '91."
L. G. [speaking from a residence, presumably his home in Houston] "If I had to point a finger, I would probably blame the factor eight producers."
R. R. "Because?"
L. G. "Because I think they were forwarned. And I think they were forwarned early enough to prevent a lot of us from getting HIV."
R. R. [narrative] "Loras Goedken was diagnosed HIV positive just before Christmas, 1985. Then, the doctors tested his wife."
L. G. "I took her in in January of '86, and they called me at the office and gave me the results of her test. And I just... [pausing] I left the office, walked around the block three times, and I'd never been around the block downtown. [pause] -did a lotta' cryin', did a lotta' kickin', and had to come home and tell her she was positive."
R. R. "You told her?"
L. G. "Yep. It was my place to tell her."
R. R. "Why?"
L. G. "She was my wife. And I gave it to her."*
"Loras Goedken died at age 52 on August 24, and a singular family nightmare ended.
Altogether, eight Goedkens died. In the two decades of this [AIDS] epidemic, authorities know of no other American family that has suffered so much...
Year after year, the Goedkens died.
As Loras' wife Jan deteriorated, she spent days in front of the television with a credit card in hand, ordering stuffed animals and dolls from television shopping channels. Dementia set in. She no longer recognized Loras, and when she came home from the hospital before her death on April 18th, 1990, she did not know it was her house...
Loras Goedken was by all accounts a vibrant man, ambitious and energetic-even when hemophilia twisted his knees and elbows into swollen knobs. And he brought passion to his fight against AIDS.
He spoke at schools. He testified before a U.S. Senate committee hearing on HIV. He traveled to Japan last year as a guest speaker at the International Conference on Hemophilia and HIV...
Mary Goedkin is alone now-her husband died of stroke earlier this year. Now, in her little white house, dozens of family photographs line the walls and the shelves. The Goedkin brothers look out of their tinted graduation portraits, young, handsome, their eyes soft.
Half a mile away, there is a row of small pink headstones in the Sacred Heart cemetary in Monticello for the Goedken family.
"I didn't have to look for heroes outside my family," said [Loras' sister, Judy]. "I had them right here."**
* Ed Bradley Street Stories, CBS, 1992. ** "Family Watches Eight Die of AIDS", The Associated Press, 50 Rockefeller Plaza, New York, NY 10020, November 1, 1997.
Below are two links to other articles written about the Goedken family:
It is the intent of the "archive e-newsletters" to present historical hemophila-related material in an accurate and objective manner which is why I generally refrain from personal commentary and rely almost entirely on quoted sources from previously published material.
I would like to add one personal thought to this edition, however.
As I looked through the archive videos and articles re: Loras Goedken, I was truly struck by the monumental sacrifice made by Loras and his family. Even after losing a brother and brother-in-law to HepC and HIV in my own family, I still could not begin to conceive of the staggering tragedy the Goedkin family endured.
Because of the enormous price paid by hemophiliacs and tainted blood transfusion recipients during the AIDS crisis, we all now enjoy a much safer blood supply. Regrettably, I do not ever recall anyone in the general public saying "thank you".
To this day, there still exists no national memorial, visible to the general public, to honor our dead. There is a sculpture and fountain within NHF headquarters which does not include names, which few will ever see. Some local hemophilia chapters have planted trees or display quilts or photos of our lost, but those are the only efforts I am aware of. Engand, Canada, and Japan are among those countries who have established national memorials, and I can only wonder why it has never been done in the U.S.
Once more, the hemophilia community must "do for itself". In that regard, I have included an attachment containing the COTT memorial brochure. Will YOU help us to remember our lost by supporting the Committee of Ten Thousand's effort to establish a national memorial? Information on how to help can be found in the brochure.
This installment of the "archive e-newsletter" includes an attachment containing two articles regarding the research behind and introduction of factor concentrates. The first article, "Hemophiliac Doctor Helps Find Way to Save Bleeders" is dated May 14th, 1966 and references Dr. Edward Shanbrom, a researcher with Hyland labs, and his collaboration with biochemist and hemophiliac, Murray Thelin, of the University of North Carolina at Chapel Hill.
The second article, "New Drug to Give Hemophiliacs Aid", is dated 1969, and heralds the arrival of the new factor concentrates. I'm also including some biographical information about Dr. Ed Shanbrom and his role in the development of factor concentrates.
"In the late 1960s, a new and more concentrated form of Factor VIII was developed. Drs. Kenneth Brinkhous of the University of North Carolina and Edward Shanbrom of Hyland laboratories produced the Factor VIII concentrate by pooling hundreds-later thousands-of units of plasma. From these pools they made large quantities of cryo[precipitate]. Then they redissolved the cryo, treated it with chemicals, fitered it, and centrifuged it-all to produce a white crystalline powder of pure, highly concentrated Factor VIII. *
"Hemophiliacs and their families greeted the new product with jubilation. Carried in a vial the size of a salt shaker, the concentrate had one hundred times the clotting power of raw plasma-so concentrated that the patient could inject it with a syringe if he wanted, instead of with a blood bag...By injecting 'early and often,' as manufacturers suggested, hemophilia patients could quickly control their bleeds, avoid the devastating episodes of joint pain, and gain enough freedom to go on vacations."*
"After completing his studies [at Chapel Hill], [Murray] Thelin was hired by Hyland, and he brought the Chapel Hill method with him...Shortly after he began to test this substance, he had a brain hemorrhage and decided to risk using the new concentrate on himself. After the first transfusion, his Factor VIII levels 'soared, and in ten days 'he walked out of the hospital smiling'...he and medical director of Hyland, Dr. Edward Shanbrom, decided to test whether the concentrate could be used to prevent prolonged bleeding. Shanbrom administered daily and weekly injections to Thelin. 'The results seemed miraculous: weeks passed without a bleed.' **
"Soon after they began producing the substance, [Dr. Shanbrom] noticed that the lab workers developed jaundice after breathing the plasma mist in the cold rooms. Suspecting hepatitis, he conducted tests on the liver enzymes of experimental patients, which showed pathological changes. 'It was obvious there was some kind of virus there', he said later. He did not consider withdrawing the product; like everyone who had seen its miraculous effects on hemophiliacs, he had no doubt that the benefits exceeded the risk. Yet he felt that, given the product could spread a chronic disease, the company should try to minimize the risk. He notified his superiors to take preventive measures, such as closing its collection centers in hepatitis hot spots. They ignored his suggestion, and he eventually left Hyland." *
Dr. Shanbrom "tried to persuade the four manufacturers of clotting-factor concentrates to use a detergent process for viral inactivation as early as the mid-to late 1970s. Though some of the companies demonstrated interest in the process, there were no takers because, as Dr. Shanbrom recalls, 'They said, We don't have a problem.' ***
"Dr. Shanbrom approached the [CDC] hoping to interest them in using and studying his detergent process of viral inactivation. The response was a letter from the CDC that he has saved to this day. The CDC expressed interest in Dr. Shanbrom's process but regretted that it did not have enough chimpanzees to enable researchers to conduct experiments with it. For want of adequate funding to the CDC for chimpanzees, clotting factor remained infectious." ***
"Once AIDS became an acknowledged reality, the drug firms quickly developed screening tests and virus-killing technologies...If they had heeded the warnings of factor VIII pioneer, Ed Shanbrom and others who issued early warnings about viruses, they would have abandoned the high-risk collection centers sooner and worked more intensively on virus-killing technology." *
"Shanbrom himself addressed this issue at a 1996 conference of AIDS victims in Japan. He had not taken the podium in order to defend himself; indeed, the moderator had introduced him as the man whose advice the industry should have taken. Nevertheless, Shanbrom lowered his head in a long ceremonial bow. 'I would like to officially and openly apologize for the pain and suffering to all the hemophiliacs and their families,' he said. 'While we attempted to do good we also did harm. For this I apologize."*
The following testimony was given by Richard Valdez, then president of "HIV Peer Association (California), at the September 12th, 1994 Institute of Medicine hearings:
"Ed Shanbrom, I met with him February of 1992. We talked for an hour just like two fathers because he lost a son too in a tragic traffic accident...He said to me, 'Dick, [HIV contamination of factor] was an accident waiting to happen.' Remember, Dr. Shanbrom was one of the co-founders of the process to make factor. His own workers [were] getting sick at Hyland from hepatitis...Ed Shanbrom said to me, 'Dick, the present and the future is bioengineering. Anytime that you grow a culture you have the potential for a virus.' "****
Ed Shanbrom was born in West Haven, Connecticut in 1924. He received his B.S. in biology from Alleghany College and his M.D. from the University of Buffalo School of Medicine. He served as physician, specializing in hematology, in addition to his work with Hyland/Baxter. Much more information about Dr. Shanbrom, the develpment of factor concentrates, and information on other key researchers can be found on PBS's website at the following URL:
Still more information can be found in the following books, from which I have quoted material in this edition of the "e-newsletter":*"Blood, An Epic History of Medicine and Commerce", Douglas Starr. Alfred Knopf, Inc., 1998, page 224, 296, 340. **"Blood Saga, Hemophilia, AIDS, and the Survival of a Community", Susan Resnik. University of California Press, 1999, page 48. ***"Cry Bloody Murder, A Tale of Tainted Blood", Elaine DePrince. Random House, 1997, pg. 51. ****"Committee to Study HIV Transmission Through Blood Products", U.S. Department of Commerce/National Technical Information Service, September, 1994.