Blood safety

The Beginnings of Transfusions


This week I am off to the National Hemophilia Foundation meeting in San Francisco, and I thought an historical look at blood might be in order. I can’t think of a better story about blood than one that starts with a madman running naked through the streets of Paris in the 17th century. True.

Chapter 1 of the wonderful book Blood by Douglas Starr starts with poor Antoine Mauroy, who suffered “phrensies.” From time to time, he would take off his clothes, run through streets and set fires. Eventually, doctors experimented on him to try to cure him. Mauroy became the guinea pig in an experiment that forever changed medicine. In 1667 Jean-Baptitste Denis, physician to king Louis XIV, transfused half a cup of blood from a calf into Mauroy. He hoped the “gentleness” of the calf would infuse as well. Despite the discoveries of the Renaissance and the advances made in science, doctors still believed the blood somehow carried the characteristics of the creature, a concept known as “vitalism.” For example, a stag’s blood carried courage; a calf gentleness. Since the ancient Greeks, the body was not yet viewed as a system, and doctors knew nothing of hormones, genes or viruses. It would be 200 more years before they discovered that water transported disease! In the 17th century, doctors believed that in the “humors”: phlegm, choler, bile, blood. The Greeks believed that good health meant maintaining a balance of the humors in the body, so draining blood and purging digestive system should help. This is where blood-letting as a medical treatment evolved.

Blood– it has a colorful past! And worth reading about. Be sure to read Blood by Douglas Starr.

Other doctors and researchers dabbled in finding out the secrets of the blood. William Harvey found valves in blood vessels, which led him to think that the body might be a system, more mechanical. Christopher Wren (the famous architect whose beautiful cathedrals I just glimpsed when I was in London last week) and Robert Boyle, founder of modern chemistry, dabbled in circulation. Richard Lower tried transfusing blood from one dog to another: he discovered how to transfuse from an artery into a vein and it worked.

What happened to our madman? Antoine Maury died from the procedure. What doctors didn’t know is that proteins in the blood from one animal–even from another person– are not always accepted by the body. The immune system may attack the foreign proteins. Dr. Denis was accused of murder. He in turn sued Antoine Mauroy’s widow in 1668 for slandering his reputation. Turns out Mauroy actually died from arsenic poisoning– by his wife! Still, the French Parliament’s banned all transfusions involving humans. Similar actions follow in England and Rome. And 150 years would pass before it was tried again.

A Week That Shook the Hemophilia World

I am writing from Europe, which is an interesting place to be right now as a member of the hemophilia community. Just this past week the first evidence of variant Creutzfeldt-Jakob disease (vCJD) transmission in an 70+ year old British person with hemophilia was confirmed, according to leaked information. The United Kingdom Health Protection Agency (HPA) has since confirmed this. There are a lot of emails and news wires being fired off. It’s important to note the facts:

1. The patient died of other causes, not vCJD.
2. The patient had not shown any symptoms of vCJD.
3. The HPA says it is still unclear how the man became infected with the prion protein that causes vCJD.
3. The patient had received British plasma-derived clotting factor concentrates before 1999, before rules were introduced to limit contagion, when the risk of transmission was not known.
4. The patient was treated with a British made product that was manufactured from the plasma of a British donor who went on to develop symptoms of vCJD.
5. It is the first time that vCJD has been found in the approximately 4,000 British hemophilic patients who received plasma-derived clotting factor concentrates between 1980 and 2001.

Up to 4,000 British hemophilia patients have been warned by authorities they could be at risk of vCJD. Authorities also state they run a low risk of developing the disease.

What we know about vCJD:

1. CJD is a fatal brain disease first classified in the 1920s.
2. In 1996, doctors reported a variant of the disease, vCJD, the result of exposure to the agent that causes Bovine Spongiform Encephalopathy (BSE, or mad cow disease) in cattle.
3. There is no test that can screen for vCJD.
4. There is no treatment for vCJD.
5. vCJD is rare.
6. Since 1995, 164 people in Britain have died from vCJD, with just one death last year.

UK plasma has not been used in the UK or elsewhere since 1996 for the production of the clotting treatment.

A statement excerpt from David Allsop, professor of neuroscience at Lancaster University, UK: “The only real cause for concern is for other hemophilia patients who received clotting factors from the same infected human donor, at around the same period of time; and they can presumably be traced and given specialist advice.”

A statement excerpt from Chris James, Chief Executive of the Haemophilia Society (of the UK): “Unfortunately, the risks are higher than they might have been because Health Authorities in England and Wales continued to use products derived from human blood to treat haemophilia long after most other developed countries had switched to safer, synthetic recombinant treatments.” (Note that all clotting products, both plasma-derived and recombinant, that are licensed for sale and distribution in the US are currently considered safe)

A statement excerpt from NHF’s MASAC, in its Medical Advisory #409 on February 18: “No Added Risk Seen for U.S. Patients. It is important to note that only individuals exposed to UK-sourced plasma infused between 1980 and 2001 are considered to be at elevated risk for vCJD compared to the general population… there should be no change in the safety profile of US-licensed products or in any treatment recommendations.”

A statement excerpt from the Committee of Ten Thousand (COTT): “This means that all 4,000 persons with hemophilia in the UK are at risk of contracting vCJD from the plasma-derived clotting factor they used during the 1990s. It also raises serious questions about the risk of CJD and variant CJD transmission in the American hemophilia/bleeding disorders community… COTT is angered that the US Department of Agriculture has failed to conduct a substantive and wide reaching surveillance program for cows in the US.” (Note: the COTT statement implies but does not specficially state that the product used by the patient was a brand of British plasma-derived clotting factor, created from pooled British plasma, which included the blood donation from a British donor infected with vCJD. These products have never been used in the US)

A statement excerpt from the Plasma Protein Therapeutics Association (PPTA): “… the industry has implemented the deferral of donors from areas of high risk of bovine spongiform encephalopathy (BSE) and its human equivalent, vCJD, for the past ten years… Therefore, this incident involved products that do not reflect the safety profile that has underpinned PPTA plasma protein therapies for the past ten years. Patients receiving these products can rest assured of their enhanced safety. The PPTA supports the conclusions of patient groups and government authorities that the use of current products to preserve the health of plasma protein recipients should be maintained.”

There you have it: an event that shook the hemophilia world this week. Facts should be read completely. Various statements by leading patient organizations, reflecting nuances in opinion, which our readers should analyze carefully for content, history, perspective, source and purpose. Read more by going to: www.hemophilia.org, www.ppta.com, www.cott1.org, www.haemophilia.org, and above all, speak with your hematologist and HTC staff if you have questions about the news reports.

Also see: http://www.hpa.org.uk/webw/HPAweb&HPAwebStandard/HPAweb_C/1234859690542?p=1231252394302

COTT: Working to keep us safe

I just received another issue of the COTT Washington Update, Vol. 11 No. 1. It’s one of the most intelligent reads you can get in the hemophilia community. COTT is the Committee of Ten Thousand, a national nonprofit that was formed in response to the HIV infections of the 80s. It helped to bring closure and settlement to the community affected by HIV. But its work wasn’t done then; it has since become our nation’s watchdog for the blood supply.

Below is just a quick sample of what’s in the Update. Note the scope of topics, the relevance to hemophilia, the timeliness of it:

1. …the unexpected withdrawal of Tom Daschle, the White House candidate for Secretary of Health and Human Services, [was a setback to health reform] who was to have also directed the Office on Health Care Reform in the White House.

2. The current economic stimulus bill drafted by the House Democratic majority includes an $80 billion for Medicaid, the same for education, and smaller amounts for unemployment insurance, COBRA costs, law enforcement, military construction and homeland security.

3. A disturbing part of the Stimulus bill language was proposed, in a section supporting outcomes- or evidence-based medical research, being called ‘comparative effectiveness’ when applied to pharmaceuticals, biologics, and medical devices. The language allowed government bodies such as the National Institutes of Health to convene expert panels to review results of such studies, and in effect endorse the winners – only those most effective – with dire ramifications for others. The problem is that cost is factored into such rankings, and while as we know new products from fractionators increase safety, they may not currently reflect improvements in cost-effectiveness. Moreover, should a panel select one brand of factor over others, there is no built-in assurance as yet that the need for all factor products to be available to all patients would be guaranteed. Alerted to this by the fractionators’ association, PPTA, COTT and others wrote to relevant committee chairs in the Senate to soften this language.

4. Vermont: The Health Department office operating the Medicaid program in Vermont contracted for hemophilia pharmacy effective November 1. Although the regional and national associations were not aware of this plan until less than a week before, HTCs in the state were contacted in advance. The regional association convened a number of input conference calls, and COTT as well as others urged that the MASAC-approved Standards of Service document be shared with that office, and that Vermont be asked to respond whether its terms were met in the contract.

5. New York: The state Medicaid office has announced contracts for hemophilia pharmacy – multiple contracts, so concern about the sole sourcing actions in other states is not present, however the process exempts 340b HTCs (Hemophilia Treatment Centers which sell factor). Thus, those currently using one of the state’s centers can continue there; those in more rural areas must however subscribe to one or the other of the state’s new contractors, regardless of whatever more hemophilia-focused pharmacies they have been using.

6. The Advisory Committee on Blood Safety and Availability met in December. The only topic of discussion was “The Responsibility of Blood and Plasma Centers to Donor and Public Health.” In COTT testimony we pointed out that conspicuously omitted from these concerns was recipient health… perhaps because the blood banking community has no direct contact with recipients but can track and work to improve care provided to, and health of, their donors.

7. Wellpoint has continued to expand the number of states in which its for-profit subsidiary payers (Blue Cross) require clients with hemophilia to use either an HTC or its own mail-order pharmacy in Indiana; the total now stands at 10 of their 14 states.

This is just a sample of the breathtaking information and vast scope of COTT’s activities. The volunteers and committee members work hard to stay on top of anything related to blood safety. While many in the community use recombinant products and think this doesn’t apply to them, COTT also monitors all activities regarding hemophilia business, such as the narrowing of choice, forced homecare switching, and research.

This summer marks COTT’s 20th year. It’s planning the construction of an AIDS/Hemophilia memorial, and the celebration of COTT’s 20th Anniversary this summer with an event in Washington.

COTT writes: “Out of the ashes of the devastation of 10,000 lives emerged a grassroots advocacy group that has brought the hemophilia community a federal advisory committee now in its 12th year, a $600 million relief bill, and plans for a national blood policy, maximizing blood safety well into the 21st century.”

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www.cott1.org

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