Creutzfeldt-Jakob Disease (CJD)

CJD is a rare degenerative and fatal nervous system disorder. It is diagnosed in about one person per million per year in the US and worldwide. There are three forms of CJD that can affect humans: sporadic CJD has no known risk factors and accounts for 85 percent of CJD cases; hereditary CJD occurs only in individuals with a family history of the disease and/or tests positive for specific genetic mutations; and acquired CJD is transmitted by exposure to brain or nervous system tissue. Acquired CJD accounts for less than 1 percent of CJD cases and can occur in individuals who have received injections of human pituitary gland growth hormone, or who have had their brain’s outer lining (dura mater) repaired with dura mater from someone else who had CJD.

Individuals who will develop CJD can remain without symptoms for decades and then progress rapidly to dementia, severe loss of coordination and death. Scientists believe abnormal brain proteins that have undergone a peculiar shape change can cause other brain proteins to do the same and cause CJD.

Currently, there is no screening test for the disease, and while blood transfusions have never been shown to transmit any form of the disease, as a precaution the Food and Drug Administration (FDA) prohibits blood donation by individuals who may be at risk. These include potential donors who have received injections of human-derived pituitary hormone, those with a family history of CJD, or those who have had surgeries that involved transplanted dura mater.

variant Creutzfeldt-Jakob disease (vCJD)

Similar to CJD, vCJD, commonly known as the human form of “mad cow” disease, is a rare degenerative and fatal nervous system disorder. There is reason to believe that vCJD occurs when humans eat beef contaminated with bovine spongiform encephalopathy (BSE or “mad cow”). This new form of CJD has appeared in residents of the United Kingdom (UK) and France, and a single individual in Italy. Cases have occurred in other countries, including one in the United States, that have been tracked to a UK origin. Currently, there is no screening test in humans for the disease.

The UK has reported two presumptive transfusion transmitted cases of vCJD. FDA blood donor deferral policies seek an optimal balance between vCJD risk reduction and blood supply preservation. These policies are are under constant review by FDA as we learn more about vCJD and BSE.

The FDA recommends that the following donors be deferred indefinitely due to vCJD risk:

• Donors who spent a total of three months or more in the United Kingdom (UK) from the beginning of 1980 through the end of 1996;
• Donors who have spent a total of five years or more in Europe from 1980 to the present;
• Current or former US military personnel, civilian military employees and their dependents who resided at US military bases in Northern Europe (Germany, UK, Belgium, and the Netherlands) for a total of six months or more from 1980 through 1990, or elsewhere in Europe (Greece, Turkey, Spain, Portugal, and Italy) from 1980 through 1996.
• Donors who have received any blood or blood component transfusions in the UK between 1980 and the present;
• Donors who have injected bovine insulin since 1980, unless it is possible to obtain confirmation that the product was not manufactured after 1980 from cattle in the UK.

Department of Defense (DoD) has a slightly different policy summarized here:

The DoD implemented its set of donor deferral rules in October 2001. All active-duty military personnel, civil service employees, and these two groups’ family members will be deferred indefinitely due to vCJD risk if they are:

• Donors who traveled or resided in the UK for a cumulative total of three months or more at any time from 1980 through the end of 1996;
• Donors who have received a blood transfusion in the UK at any time from 1980 to the present;
• Donors who have traveled to or resided anywhere in Europe for a cumulative total of six months or more at any time from 1980 through the end of 1996;
• Donors who traveled to or resided anywhere in Europe for a cumulative total of five years or more at any time from Jan. 1, 1997, to the present.

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