Top FAQ's about Mad Cow Disease

Q1.What is variant Creutzfeldt-Jakob Disease (kroits ' felt - yä ' kôp) (vCJD)?
A1.
  • vCJD is the human form of bovine spongiform encephalopathy (BSE), known as "mad cow disease." Both the human and bovine disorders are fatal brain diseases.
  • This rapidly progressive, degenerative neurologic disease is silently carried within the body for years before it attacks and destroys the brain, causing rapid deterioration and death usually within a year.
  • The disease process has been described in ancient history, but the pathogen itself is a newly recognized agent, a highly resistant infectious protein called a prion. Prion diseases are now known to be a group of uniformly fatal, brain-destroying conditions affecting both humans and animals. Prion transmission has been traced to ingestion in a number of animals.
  • Direct inoculation of infected material has also spread the disease. It is undetectable in the infectious, preclinical state and is sturdy, surviving routine sterilization.

Q2.What is "classic" CJD?
A2.

  • A degenerative, invariably fatal brain disorder. CJD affects about one person in every 1 million people per year worldwide.
  • In the United States, there are about 200 cases per year. CJD usually appears in later life and runs a rapid course.
  • Typically, onset of symptoms occurs about age 60, and about 90 percent of patients die within a year. In the early stages of the disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities and coma may occur. There is no cure for the disease.
Q3.How are people infected with vCJD?
A3.
  • The cause of this new vCJD, which has now killed more than 100 people in Europe (mostly Great Britain), has now been traced to the consumption of meat and meat products infected with BSE.
  • Research suggests that the cause of vCJD is a variant form of proteins called "prions," which are self-replicating proteins that attack the brain and central nervous system. This is not a bacteria or a virus, which are much more familiar to scientists; it is an entirely new pathogen, and we are actively researching methods to deal with it.
  • There is enormous scientific uncertainty surrounding vCJD. Although there is no evidence that vCJD has been transmitted through human blood, blood products or plasma, some studies using laboratory animals indicate that transmission through blood and blood components may be possible.

Q4.What is BSE?
A4.

  • Bovine Spongiform Encephalopathy (BSE), widely referred to as "mad cow disease," is a chronic degenerative disease affecting the central nervous system of cattle. The disease was first diagnosed in 1986 in Great Britain.
  • Affected animals may display changes in temperament, such as nervousness or aggression, abnormal posture, incoordination, decreased milk production or loss of body weight despite continued appetite.
  • There is no treatment or vaccine to prevent the disease and affected cattle die. BSE may have been caused by feeding cattle rendered protein produced from the carcasses of scrapie-infected sheep or cattle with previously unidentified Transmissible Spongiform Encephalopathies (TSE).
Q5.Are patients receiving blood transfusions at risk of contracting the disease through blood?
A5.
  • There have been no known cases of humans receiving vCJD through a blood transfusion.
  • However, there is some risk the disease may be transmissible through blood, so the Red Cross is applying its best judgment by tightening the restrictions to exclude some blood donors who have lived in Europe.
  • The cause of this new vCJD, which has now killed more than 100 people in Europe (mostly Great Britain), has now been traced to the consumption of meat and meat products infected with BSE.
  • Vulnerable patients' lives depend on the safest possible blood available. The American Red Cross must be prudent and cautious to implement a more strict deferral in the face of scientific uncertainty, because there is a long latency period with this disease, evidence in animal models that the disease may be contracted through blood and currently no blood test exists.

Q6.How did you arrive at this new deferral policy?
A6.
    This policy was developed with serious consideration and deliberation involving leading scientists and epidemiologists from the United States and around the world. A geographic deferral is a necessary step in the absence of a blood-screening test for vCJD. The Red Cross policy will restrict the following people from donating blood to the Red Cross:

  • anyone who has lived in the United Kingdom for a cumulative total of three months since 1980 or
  • anyone who has lived in any European country or combination of countries (including the United Kingdom) for a cumulative total of six months since 1980 or
  • anyone who has received a blood transfusion in the United Kingdom since 1980.

    • Several factors call for a cautious, prudent approach.

    • There is enormous scientific uncertainty about the disease.
    • There is no test available to screen for vCJD, although the American Red Cross and others are working hard to develop one.
    • There is a long latency period (10 to 20 years) before vCJD can be detected.
    • There is enormous scientific uncertainty surrounding vCJD. Although there is no evidence that vCJD has been transmitted through human blood, blood products or plasma, some studies using laboratory animals indicate that transmission through blood and blood components may be possible.
    • The disease continues to spread. There are now more than 100 cases of vCJD deaths throughout Europe.
Q7.How safe is America's blood supply?
A7.
  • America's blood supply today is among the safest in the world. The American Red Cross is committed to the safety of our volunteer donors, our patients and the public we serve. We make every effort to continually improve blood safety.
  • The American Red Cross is at the forefront of implementing blood safety measures, including some that go above and beyond what is required by the Food and Drug Administration (FDA). For example, the Red Cross is implementing universal leukocyte reduction for all of its blood products, a process of depleting contaminating white blood cells from blood. The Red Cross is a leader in the effort to implement nucleic acid testing (NAT) on a nationwide scale. NAT is being studied under an investigational new drug protocol, which is designed to detect the genetic material of a virus like HIV or hepatitis C much faster than the current test.
  • Since 1991, the American Red Cross has made enormous investments in blood safety and availability:
  • Converting from a patchwork of 54 local cottage industry blood banks into a single blood services enterprise to improve safety, increase efficiencies, standardize performance and establish accountability.
  • Building nine state-of-the-art National Testing Labs where the Red Cross performs increasingly sophisticated tests seeking to ensure blood safety.
  • Developing a national computer system for databasing donor records, blood-products tracking and labeling, clinical evaluations, constant quality assessment and monitoring the nation's blood inventory on a daily basis.
Q8.What will the American Red Cross do if "mad cow disease" or its human equivalent (vCJD) is found in the United States?
A8.
  • The American Red Cross is implementing prudent, cautious donor deferral criteria, no matter how difficult, to help protect the safety of the blood supply, because it is possible that "mad cow disease" could appear in the United States. The Red Cross will constantly evaluate the emerging science being conducted on this disease and when there is more definitive science and/or a test for vCJD will re-evaluate the criteria.
  • If BSE is some day found in the Untied States, the American Red Cross will use the information available at that time to implement appropriate donor guidelines to help protect America's blood supply.
Q9.Will donors who are deferred under the new criteria be allowed to donate in the future?
A9.
  • Donors who are no longer eligible to give blood because of the new policy will be deferred for an indefinite period of time. The American Red Cross will re-evaluate this criteria within one year or sooner should more definitive scientific evidence become available.
  • In the absence of a blood test, the Red Cross must be cautious. A test is a necessary and defining step to deal with the public health and public fear problems associated with "mad cow disease" and it will help lead to a solution. A blood screening test is the beginning of Red Cross efforts and may lead to medical breakthroughs and understanding.
  • The Red Cross continues to actively research CJD and vCJD to determine if there is a link between those diseases and blood transfusion.
Q10.How will this decision affect the availability of the blood supply?
A10.
  • It is the responsibility of the American Red Cross to ensure the safest possible blood supply is available to all patients whenever and wherever needed.
  • The American Red Cross recognizes this will have an impact on blood availability, reducing the current number of Red Cross donors by about 3-5 percent. The importance of protecting the safety of the blood supply given this emerging threat is paramount, and the Red Cross confident it will continue to increase blood collections.
  • The Red Cross is significantly expanding efforts to recruit, retain, cultivate, honor and recognize volunteer blood donors and make it as easy and pleasant as possible to give lifesaving blood.
  • The Red Cross is investing in targeted marketing campaigns, leveraging direct mail, telemarketing and advertising to raise public awareness of the need for blood and encourage people to donate regularly.
Q11.Why is the American Red Cross issuing a more cautious deferral than the FDA?
A11.
  • In the face of scientific uncertainty, different experts will reach different conclusions. It is impossible to say what is the best approach. The American Red Cross believes that until there is a blood test for vCJD, it must take a prudent, cautious approach.
  • The Red Cross and others have to make decisions based on what is known. In this case the data are incomplete, and the science is uncertain.
  • The FDA was aggressive in banning blood donors from the United Kingdom. This was a very wise and courageous move by the FDA. The American Red Cross has an obligation to take the next steps.
Q12.Does this mean Red Cross blood will be safer?
A12.
  • The American Red Cross is taking prudent actions to ensure the safety of America's blood supply. It does not claim its blood is safer than any other blood centers; simply, the Red Cross is making the judgment to act cautiously.
  • America's blood supply is extremely safe. Vulnerable patients depend on blood generously given by volunteer donors who entrust the American Red Cross and others to turn every donation into the safest possible transfusion.
Q13. Define what countries are included in the deferral.
A13.
  • People who have lived in the United Kingdom (England, Northern Ireland, Scotland, Wales, Falkland Islands, Gibraltar, Isle of Man or the Channel Islands) for a cumulative total of three months or more between 1980 and the present will be restricted from donating blood to the American Red Cross.
  • Anyone who received a blood transfusion in the United Kingdom since 1980 will be deferred.
  • Additionally, anyone who has lived in the following European countries for a cumulative total of six months or longer since 1980 will be prevented from donating blood to the Red Cross:
  • Eastern Europe: Albania, Belarus, Bosnia/Herzegovina, Bulgaria, Croatia, Czech Republic, Estonia, Hungary, Latvia, Lithuania, Macedonia, Poland, Republic of Moldova, Romania, Slovak Republic (Slovakia), Slovenia, Ukraine, Yugoslavia (Federal Republic includes Kosovo, Montenegro and Serbia)
  • Western Europe: Andorra, Austria, Azores (Portugal), Belgium, Denmark Faroe Island (Denmark), Finland, France, Germany, Greece, Greenland, Iceland, Italy, Liechtenstein, Luxembourg, Madeira Islands (Portugal), Malta, Monaco, Netherlands, Norway, Portugal, Republic of Ireland, San Marino, Spain, Svalbard (Norway), Sweden, Switzerland, Vatican City
Q14. I have traveled extensively to Europe. Do I have this disease?
A14.
  • The American Red Cross is not suggesting that blood donors who have traveled to Europe have this disease. Traveling to these locations may have exposed you to the potential contaminant for this disease. The people who have developed this disease to date have been long-term (years) residents of these countries. The Red Cross cannot pinpoint exactly when or how their exposure occurred.
  • Currently about 100 people throughout the world have been diagnosed with vCJD.
  • There is tremendous scientific uncertainty about vCJD, and the importance of protecting the safety of the blood supply given this potential emerging threat must be paramount.

Q15. Do you need me to donate blood?
A15.

  • The American Red Cross depends solely on the generosity of our volunteer blood donors to help ensure safe and adequate blood is available for patients wherever and whenever needed.
  • Your generous blood donation may save the life of a new father injured in an accident, a teenager undergoing cancer treatment, or a child with sickle cell anemia. Each day, thousands of people depend on blood – the precious gift of life.
  • If you are eligible to donate blood, please call 1-800-GIVE-LIFE to schedule a blood donation appointment in the coming weeks.
  • If for any reason you cannot give blood, there are other ways to continue contributing to saving lives. Volunteer to help with a blood drive in your community. Or make a financial donation to help support ongoing American Red Cross research efforts to identify and eradicate threats to America's blood supply.
Q16. Does this restriction apply to vegetarians?
A16.
  • Yes. All individuals who lived in the United Kingdom for a total three months or in any European country for six months cumulative since 1980 are deferred.
  • There is no test available at this time to screen for vCJD, although the American Red Cross and others are working hard to develop one.
  • There is tremendous scientific uncertainty about vCJD, and the importance of protecting the safety of the blood supply given this potential emerging threat must be paramount.
Q17. Does the Red Cross differentiate between "living in" and "traveled to"?
A17.
  • No. The deferral excludes anyone who spent time, whether resided in or traveled to the countries included in the new restrictions.
  • For example, if someone's European vacations total six months since 1980 to the present, he or she will not be eligible to donate to the Red Cross.
Q18.Since there is no blood test for vCJD, how can you be sure that volunteer blood donors will be honest about their possible exposure at the time of donation or remember their travels in the last 10 years?
A18.
  • The American Red Cross relies on the honesty of each and every blood donor. It trusts its blood donors.
  • The Red Cross receives only voluntary donations, encouraging people to give out of altruistic motivations to support the safest possible blood supply in the United States.
  • The Red Cross does not provide monetary or other incentives, such as free blood tests or tax donations to potential blood donors. There is no incentive to be dishonest when answering the questions.
  • In addition, the thorough donor-screening procedures of the American Red Cross include a written donor history. When questions on the written donor history indicate further evaluation, an oral history is done. During the verbal donor history, trained Red Cross personnel interview potential donors to determine their risk factors.
  • At the conclusion of the donation, each person is provided the option to place a private, highly confidential "do not use my blood" sticker on the blood donation form before giving blood.

Q19. Does the Red Cross import blood from Europe?
A19.

  • No. The American Red Cross does not import any blood from outside the United States.
Q20. How much meat would someone have to eat to worry about getting vCJD?
A20.
  • The Red Cross doesn't know how much meat a person would have to eat before he/she is at risk of developing vCJD. It is thought that the risk is extremely low on a per-meal basis.
  • The Red Cross recommends you visit the Centers for Disease Control and Prevention (CDC) at www.cdc.gov or the Food and Drug Administration's website at www.fda.gov
    • for more information.

Q21. Can vCJD be sexually transmitted?
A21.

  • There is no evidence that vCJD can be sexually transmitted. In fact, this seems to be true for all forms of spongiform encephalopathies.

Q22. I am now ineligible to give blood? Since CJD is genetic, will vCJD affect my offspring -- i.e., will any of my children be at risk for vCJD?
A22.

  • It is important to remember that classic CJD and variant CJD are different diseases. Variant CJD is thought to be an infectious disease related to BSE, or mad cow disease. Classic CJD is not a new disease. Most of the cases are sporadic but some forms of CJD are genetic. Genetic CJD occurs in families and appears to be caused by a specific mutation that can be inherited. The requirements for blood donation cover both classical CJD and variant CJD. If a person is deferred from donation because of travel to countries with BSE, that decision is based on concerns about variant CJD - the acquired non-genetic form. This is not an inherited risk, and you do not need to be concerned about your children.
Q23. Can people be infected with vCJD by drinking milk?
A23.
  • No. Researchers have tried to transmit related TSEs through milk and have not been able to do it. Calves of cows infected with BSE do not themselves have BSE. Milk probably does not contain enough blood cells or plasma to be infectious for the prions. The European authorities have addressed this question specifically and agree that milk is not a source of vCJD in humans or BSE in cattle.
Q24.Can Nucleic Acid Testing (NAT) detect vCJD?
A24.
  • No. Nucleic Acid Testing (NAT) looks for the actual presence of specific material from the nucleus, in HIV and HCV viruses. Prions do not have genetic (DNA or RNA) material and are therefore undetectable via NAT testing. NAT is performed on all donated blood under an Investigational New Drug protocol submitted to the FDA.

Q25. If the Red Cross is so concerned about the risk posed by donors who will be deferred, why wait until September to implement the new criteria?
A25.

    • The Red Cross is regulated as a drug, a biologic and a medical device. All staff must undergo extensive training on all of our operating procedures.
    • Each blood donor must complete a blood donation record before giving blood. We must revise the questionnaire to reflect this change, reprinting and distributing more than 7 million forms and related documents.

Q26. If a current donor told a Red Cross staff member during the health history interview that he/she thought the deferral would apply to him/her, would the staff member encourage the donor to self-defer or would the staff member encourage the donor to donate now while there's still time?
A26.

  • Because the Red Cross operates in a strictly regulated environment, collections staff members follow detailed procedures. Until an official change is made to those procedures, Red Cross staff will not deviate from them. The decision to donate blood is a personal one and a donor must decide for himself or herself whether he or she will donate blood.

Q27. Will adding questions to the blood donation record confuse donors or turn them away from giving blood?
A27.

  • Every question the Red Cross asks donors and every step in the donation process is designed to ensure the safest possible blood supply is available for our hospital patients. And because volunteer blood donors have only one motivation to give blood--they want to save lives. The Red Cross believes that donors will be willing and even eager to help the Red Cross ensure the safety of the community blood supply in this way.
  • The Red Cross is participating in a American Association of Blood Banks program to create a more unified blood donation record to make the donation process better.