"CJD Lookback Study" (Research Study to Assess the Risk of Blood Borne Transmission of CJD)

1. "CJD Lookback Study"(Research Study to Assess the Risk of Blood Borne Transmission of CJD) Roger Y. Dodd, PhD Presented by Peter L. Page, MD American Red Cross Blood Services TSEAC October 14, 2004

2. Classic Creutzfeldt-Jakob disease (CJD) a rapidly fatal degenerative disorder of the central nervous system characterized by cerebral spongiform changes caused by a distinct group of agents called prions In 1996, a new variant of CJD (vCJD) described in the UK Possible transmission of vCJD by blood transfusion reported from UK in 2003 Second possible case of vCJD transmission via transfusion, reported from UK in July 2004 Creutzfeldt-Jakob disease (CJD)

3. Designed in 1994 by ARC and CDC and implemented in 1995 To assess the risk of transmission of CJD by whole blood and blood components In 1997, the coordinating responsibility for the study transferred to the former NBDRC of AABB Late 2003, the study management returned to ARC September 2004, a cooperative agreement on the study reached between CDC and ARC for the next 5 years CJD Lookback Study

4. Upon a U.S. blood center learning that a blood donor has been diagnosed with CJD, recipients of whole blood and blood components from prior donations are identified (not notified*). Each year since 1995, the names of these recipients are checked against the NDIPlus (National Center for Health Statistics) for multiple causes of death. Methods *FDA new guidance

5. Both CDC IRB and ARC IRB approved the study Changes have been made to the study protocol following the reporting of possible transmission of vCJD through transfusion and FDA’s new guidance no notification necessary for this study medically appropriate notification and counseling may be performed at the discretion of health care providers Both CDC IRB and ARC IRB will be consulted when a vCJD should occur in the US, a test becomes available or classic CJD should be associated with blood transmission. Methods

6. Enrollment as of June 2004 28 donors (CJD patients) 368 recipients of blood 102 alive (as of February 2003) 241 deceased 0 CJD 240 not CJD 1 pending 25 being investigated Results

7. Post transfusion survival in person-years Alive 966 Deceased 430 Total 1396 Results

8. Number of Long Term (>= 5 years) Survivors # years Living Deceased Total after txn >=5 84 32 116 5-10 47 25 72 11-15 24 3 27 16-20 10 3 13 21+ 3 1 4 Results

9. No cases of CJD have occurred in 342 recipients of whole blood and blood component from donors who subsequently developed CJD, representing nearly 1,400 person-years of follow up. Long term follow up of survivors will allow for more accurate estimate of the risk, if any, of transmission of CJD by blood components. Summary

10. When a CJD patient has been a volunteer blood donor, family members should contact the collecting blood center. Blood centers learning of a blood donor having developed CJD are encouraged to participate in the study by contacting: 301 738-0495 fax Karen Fujii, 301 738-0774 phone, fujiik@usa.redcross.org Edward Notari, 301 738-0646, notarie@usa.redcross.org Shimian Zou, PhD, 301 738-0644, zous@usa.redcross.org Contact Information

11. Marian T. Sullivan, MS, MPH: currently with Research Triangle Institute CDC: co-investigator (Dr. Larry Schonberger) and funding support Blood Regions/Centers: support from many ARC Blood Services Regions and non-ARC blood centers including the military blood program ARC Staff: Karen Fujii, Edward Notari, Shimian Zou Acknowledgment