Creutzfeldt-Jakob Disease: The Problem of Recipient Notification

Masters C.L. Harris J.O. Gajdusek D.C. , “Creutzfeldt-Jakob Disease: Patterns of Worldwide Occurrence and the Significance of Familial and Sporadic Clustering,” Annals of Neurology, 5 (1978): 177–88.

See id. For example, Creutzfeldt-Jakob disease (CJD) is known to have a higher incidence among Libyan Jews and in Slovakia, Italy, and Chile.

Roos R. Gajdusek D.C. Gibbs C.J. , “The Clinical Characteristics of Transmissible Creutzfeldt-Jakob Disease,” Brain, 96 (1973): At 7–11.

Health Canada, Canada Communicable Disease Report (Ottawa: Queen's Printer, vol. 22–8, Apr. 15, 1996): At 57–60.

Lantos P.L. , “From Slow Virus to Prion: A Review of Transmissible Spongiform Encephalopathies,” Histopathology, 20 (1992): At 2.

Masters C.L. Richardson E.P. Jr. , “Subacute Spongiform Encephalopathy (Creutzfeldt-Jakob Disease): The Nature and Progression of Spongiform Change,” Brain, 101 (1978): 333–44.

The view that the infectious agent of CJD is viral is defended in Manuelidis L. , “The Dimensions of Creutzfeldt-Jakob Disease,” Transfusion, 34 (1994): 915–28; and in Manuelidis E.E. Manuelidis L. , “A Transmissible Creutzfeldt-Jakob Disease-Like Agent is Prevalent in the Human Population,” Proceedings of the National Academy of Science, 90 (1993): 7724–28.

DeArmond S.J. , “Overview of the Transmissible Spongiform Encephalopathies: Prion Protein Disorders,” British Medical Bulletin, 49 (1993): 725–37.

The term prion was first introduced in Prusiner S.B. , “Novel Proteinaceous Infectious Particles Cause Scrapie,” Science, 216 (1982): 136–44.

Rosenthal N.P. , “Familial Neurological Disease Associated with Spongiform Encephalopathy,” Archives of Neurology, 33 (1976): At 258; and Prusiner S.B. Hsiao K.K. , “Human Prion Diseases,” Annals of Neurology, 35 (1994): At 389–91.

For a helpful summary, see Will R.G. , “Epidemiology of Creutzfeldt-Jakob Disease,” British Medical Bulletin, 49 (1993): At 962–65.

Brown P. Preece M.A. Will R.G. , “‘Friendly Fire’ in Medicine: Hormones, Homografts, and Creutzfeldt-Jakob Disease,” Lancet, 340 (1992): 24–27.

Manuelidis E.E. , “Experimental Creutzfeldt-Jakob Disease Transmitted Via the Eye with Infected Cornea,” N. Engl. J. Med., 296 (1977): 1334–36.

Thadani V. , “Creutzfeldt-Jakob Disease Probably Acquired from a Cadaveric Dura Mater Graft,” Journal of Neurosurgery, 69 (1988): 766–69.

Bernouilli C. , “Danger of Accidental Person-to-Person Transmission of Creutzfeldt-Jakob Disease by Surgery,” Lancet, (1977): 478–79.

Fradkin J.E. , “Creutzfeldt-Jakob Disease in Pituitary Growth Hormone Recipients in the United States,” JAMA, 265 (1991): 880–84.

See Manuelidis , supra note 7.

Evatt B.L. , “Current Status of Creutzfeldt-Jakob Disease and Blood Product Safety,” Haemophilia World, 2, no. 2 (1995): 3–4. But see Créange A. , “Creutzfeldt-Jakob Disease After Liver Transplantation,” Annals of Neurology, 38 (1995): 269–72. Reports of earlier studies suggesting the possibility of transmission by blood transfusion include Manuelidis E.E. Gorgacz E.J. Manuelidis L. , “Viremia in Experimental Creutzfeldt-Jakob Disease,” Science, 200 (1978): 1063–65.

See Esmonde T.G.F. , “Creutzfeldt-Jakob Disease and Blood Transfusion,” Lancet, 341 (1993): 205–07; and a similar study by Heye N. Hensen S. Muller N. , “Creutzfeldt-Jakob Disease and Blood Transfusion,” Lancet, 343 (1994): 298–99.

Dodd Roger , American Red Cross, Remarks at Special Meeting of Food and Drug Administration Special Advisory Panel on Creutzfeldt-Jakob Disease and Blood Products (June 22, 1995). See Council of Community Blood Centers, CCBC Newsletter, June 23, 1995, at 9–10.

This was reported at a meeting on December 15–16, 1994. See “BPAC Recommends Component Retrieval for Previous Donations from CJD-Infected Donors,” AABB Blood Bank Week, 11, no. 45 (1994).

It was passed by a vote of six to five, with two abstentions, at a special meeting held on June 22, 1995. For a report of the proceedings, see Council of Community Blood Centers, supra note 20.

Memorandum from the European Agency for the Evaluation of Medicinal Products (Feb. 13, 1995) (on file with author); and Position Paper or the European Association of the Plasma Products Industry (July 18, 1995) (on file with author).

Information Letter from Health Canada (Oct. 20, 1995) (on file with author).

Letter from Aye Bert Dr. , National Director of Blood Services, to all Blood Bank directors and chief executive officers (July 17, 1995) (on file with author); and Memorandum from Aye Bert Dr. , National Director of Blood Services, to all Blood Bank directors and chief executive officers (July 24, 1995) (on file with author).

For example, the Hospital for Sick Children in Toronto and the Calgary Regional Health Authority both undertook notification campaigns. However, Vancouver General Hospital, Alberta Capital Health Authority, and Winnipeg's Health Sciences Centre have each decided not to notify specific recipients.

This point was raised by Rothman David , Remarks at the Meeting “Creutzfeldt-Jakob Disease: Decision-Making in Conditions of Uncertainty,” Toronto (June 6, 1996) (on file with author).

In addition, in the event that life or health insurance companies include questions about exposure to CJD in application questionnaires, those notified may find life or health insurance either unavailable or more expensive.

Walker R. , “Blood Recipients Joining Forces,” Herald (Calgary), May 1, 1996, at B1.

Immen W. , “Hospital Warns Parents of Blood Risk,” Globe and Mail (Toronto), May 4, 1996, at A1.

I acknowledge that the analogy is not as persuasive as hoped. Aside from young children, those tested for Huntington disease already know that they are at some risk of developing the disease. The test simply makes the prognosis more or less certain. Notifying of possible CJD exposure introduces this risk to persons theretofore totally unaware of it.

Jankovic J. Beach J. Ashizawa T. , “Emotional and Functional Impact of DNA Testing on Patients with Symptoms of Huntington's Disease,” Journal of Medical Genetics, 32 (1995): 516–18. The authors found no significant differences in psychological scores, depression, functional capacity, symptom interference, independence, or other measures of mood and behavior two weeks and three months following testing. However, see Bloch M. , “Diagnosis of Huntington Disease,” American Journal of Medical Genetics, 47 (1993): 368–74; and Tibben A. , “DNA Testing for Huntington's Disease in the Netherlands,” American Journal of Medical Genetics, 44 (1992): 94–99. Both studies found profound effects when patients were advised of their Huntington disease status.

Lipe H. Schultze A. Bird T.D. , “Risk Factors for Suicide in Huntington's Disease,” American Journal of Medical Genetics, 48 (1993): 231–33; and Farrer L.A. , “Suicide and Attempted Suicide in Huntington Disease,” American Journal of Medical Genetics, 24 (1986): 305–11.

Bloch M. , “Predictive Testing for Huntington Disease in Canada,” American Journal of Medical Genetics, 42 (1992): 499–507.

Codori A. Brandt J. , “Psychological Costs and Benefits of Predictive Testing for Huntington's Disease,” American Journal of Medical Genetics, 54 (1994): 174–84.

See, for example, Stanback v. Parke, Davis & Co., 657 F.2d 642 (4th Cir. 1981); Doe v. Miles Laboratories, 927 F.2d 187 (4th Cir. 1991); and Tarasoff v. Regents of the University of California, 551 P.2d 334 (Cal. Sup. Ct. 1976).

For example, Christopher v. Cutter Laboratories, 53 F.3d 1184 (11th Cir. 1995) (alleging that the defendant supplier of blood products failed to warn prior to the transfusion that might have transmitted HIV to the hemophiliac patient).

927 F.2d 187.

In re Sealed Case, 61 F.3d 965 (D.C. Cir. 1995).

See id.; and Pittman Estate v. Bain [1994] 112 D.L.R.4th 257, 403–12 (Ont. High Ct.).

927 F.2d at 193; and 112 D.L.R.4th at 310.

Arato v. Avedon, 858 P.2d 598, 605 (Cal. 1993) (noting duty to disclose “dire prognosis”).

112 D.L.R.4th at 372.

Daly v. United States, 946 F.2d 1467 (9th Cir. 1991).

Union Carbide & Carbon Corp. v. Stapleton, 237 F.2d 229 (6th Cir. 1956).

M.M.H. v. United States, 966 F.2d 285 (7th Cir. 1992).

Miles, 927 F.2d at 194–95.

Hoemke v. New York Blood Center, 912 F.2d 550 (2d Cir. 1990).

See id. at 554.

Spann v. Irwin Memorial Blood Centers, 40 Cal. Rptr. 2d 360 (Cal. Ct. App. 1995).

American College of Legal Medicine, Legal Medicine (St. Louis: Mosby, 3rd ed., 1995): At 283.

Miceikis v. Field, 347 N.E.2d 320, 324 (Ill. App. Ct. 1976) (“excessive disclosure of remote risks would tend to do more harm than good to the patient”); see also, Pardy v. United States, 783 F.2d 710 (7th Cir. 1986); and McInerney v. McDonald [1992] 93 D.L.R.4th 415, 429–30 (Can.).

This is the manner of notification used, for example, by the Calgary Regional Health Authority and Toronto's Hospital for Sick Children.

Kessler S. , “Psychiatric Implications of Presymptomatic Testing for Huntington's Disease,” American Journal of Orthopsychiatry, 51 (1987): 212–19.

Quaid K.A. Wesson M.K. , “Exploration of the Effects of Predictive Testing for Huntington Disease on Intimate Relationships,” American Journal of Medical Genetics, 57 (1995): 46–51.

This is the approach adopted by Winnipeg's Health Sciences Centre when it faced this dilemma. See Free Press (Winnipeg), May 11, 1996, at A2.

This is the method preferred by Health Canada. See Information Letter from Health Canada, Health Protection Branch (Oct. 20, 1995) (on file with author).

Pittman Estate v. Bain suggests that it may not. See Pittman, 112 D.L.R.4th at 382–83.

This point was made by McLelland Gary , Remarks at Meeting “Creutzfeldt-Jakob Disease: Decision-Making in Conditions of Uncertainty,” Toronto (June 6, 1996) (on file with author).

This point was made by Paltiel David , Remarks at Meeting “Creutzfeldt-Jakob Disease: Decision-Making in Conditions of Uncertainty,” Toronto (June 6, 1996) (on file with author).