Creutzfeldt-Jakob disease (CJD) is a rare fatal neurodegenerative disorder caused by abnormal prion agents. Transmissibility of the classic forms of CJD by blood transfusion is currently unknown. The proposed project will continue the only active, nationwide look-back study on recipients of blood products from donors subsequently diagnosed with classic CJD. This study began in 1995, and has been working for the past 20 years on the key objective of carefully and systematically assessing the risk of blood borne transmission of this disease in the United States. The major components of the study include collaboration with multiple sources to identify blood donors diagnosed with classic CJD in the United States; working with blood centers to trace the blood components from donors diagnosed with classic CJD to final disposition; collecting vital status and cause of death information (if applicable) on the recipients of blood components; utilizing the data from the study to continually monitor and assess the risk of blood-borne transmission of CJD; and disseminating results of the study to relevant stakeholders. To date, the study has enrolled 54 blood donors who died of classic CJD and 666 recipients of their blood. Through 2013, 125 of these recipients were presumed to be still alive, 520 were deceased and 21 were lost to follow-up. Post transfusion, these three groups had survived a total of 2719.5 person-years. A total of 187 recipients survived five years or longer post transfusion and 86 of them had received blood donated 60 or fewer months before the onset of CJD in the donor. No recipients with CJD have been identified. The current results of this large, ongoing look-back study show no evidence of transfusion transmission of CJD. They reinforce the conclusion that the risk, if any, of transfusion transmission of prion disease by classic CJD donors is significantly lower than the comparable risk of such transmission by vCJD donors.
This study collects blood recipient outcome data to assess the potential risk of transmission of classic Creutzfeldt-Jakob disease (CJD) by blood components from donors who subsequently developed CJD. There are two phases to this study, retrospective and prospective. The retrospective phase identifies the CJD infected blood donors and the recipients of those blood components while the prospective phase that tracks these recipients for the development of CJD. During the retrospective phase, we receive reports about blood donors who subsequently developed CJD from blood centers, family members, physicians and the Centers for Disease Control and Prevention (CDC). Once we receive the report we notify the blood center (if previously not notified) and they contact the consignees of the blood components, the consignees identify the recipients and report the survival status and if deceased the date and cause of death of that recipient. If the recipient is listed as deceased, we verify the date and cause of death with the National Death Index (NDI). If the recipient is listed as alive we move onto the prospective phase of the study. This consists of tracking the recipient to see if they are currently alive (as of the last available NDI data set) and if not, document the date and cause of death. We track the recipients by using the NDI Plus database and other databases as needed. We match the recipient's information to the information in the database to determine if it is a match. The risk to the human subjects is minimal. For donors who are reported by the blood centers, only the donor initials are available. For those individuals whose family members report the case, the family receives an information letter and Consent for Participation and Release of Information form to fill out. Investigation does not proceed unless the consent for participation is signed. Names of the family reported donors are kept in a secure cabinet with very limited personnel access. Only the donor initials are used when imputing the data in the database. The risk is also minimal for the recipients. The surviving recipients are not notified of the tracking because to date there has not been a transfusion transmission of classic CJD. Notifying the recipient would cause undue stress and mental anguish. This would be an unnecessary harm to the recipient. All recipient information is kept in a secure cabinet with very limited personnel access. The name, date of birth and Social Security number of the recipients are reported in the database and the database can only be accessed by specific co- investigators.
