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Creutzfeldt-Jakob Disease: The Problem of Recipient Notification

Published online by Cambridge University Press:  01 January 2021

Extract

In the past twelve to eighteen months, another perceived threat to the safety of America's blood supply has arisen. The fear is that Creutzfeldt-Jakob disease (CJD) will join hepatitis, HW and AIDS in the public and medical consciousness as the 1990s next infectious disease epidemic. A particular kind of ethical dilemma has arisen causing much debate and consternation for hospitals, regulators, and blood suppliers, and has elicited a remarkably varied response.

CJD is a rare but uniformly fatal neurological disease: it affects the brain and the rest of the central nervous system. Its incidence in the population of the developed world is about one per million per year, although some geographic anomalies exist. CJD manifests itself in two broad ways. First, the disease is associated with symptoms of mental deterioration and dementia, including memory loss, anxiety, and cognitive impairment. Second, CJD is also associated with a range of neurological symptoms, including muscle rigidity, seizures, tremors, muscle twitching, and spasticity, as well as problems with muscle coordination and unsteady gait, sometimes resulting in total disability.

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Article
Copyright
Copyright © American Society of Law, Medicine and Ethics 1997

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References

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.CrossRefGoogle Scholar
See id. For example, Creutzfeldt-Jakob disease (CJD) is known to have a higher incidence among Libyan Jews and in Slovakia, Italy, and Chile.Google Scholar
Roos, R. Gajdusek, D.C. Gibbs, C.J., “The Clinical Characteristics of Transmissible Creutzfeldt-Jakob Disease,” Brain, 96 (1973): At 7–11.Google Scholar
Health Canada, Canada Communicable Disease Report (Ottawa: Queen's Printer, vol. 22–8, Apr. 15, 1996): At 57–60.Google Scholar
Lantos, P.L., “From Slow Virus to Prion: A Review of Transmissible Spongiform Encephalopathies,” Histopathology, 20 (1992): At 2.CrossRefGoogle Scholar
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.CrossRefGoogle Scholar
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.CrossRefGoogle Scholar
DeArmond, S.J., “Overview of the Transmissible Spongiform Encephalopathies: Prion Protein Disorders,” British Medical Bulletin, 49 (1993): 725–37.CrossRefGoogle Scholar
The term prion was first introduced in Prusiner, S.B., “Novel Proteinaceous Infectious Particles Cause Scrapie,” Science, 216 (1982): 136–44.CrossRefGoogle Scholar
Rosenthal, N.P. et al. , “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.CrossRefGoogle Scholar
For a helpful summary, see Will, R.G., “Epidemiology of Creutzfeldt-Jakob Disease,” British Medical Bulletin, 49 (1993): At 962–65.CrossRefGoogle Scholar
Brown, P. Preece, M.A. Will, R.G., “‘Friendly Fire’ in Medicine: Hormones, Homografts, and Creutzfeldt-Jakob Disease,” Lancet, 340 (1992): 2427.CrossRefGoogle Scholar
Manuelidis, E.E. et al. , “Experimental Creutzfeldt-Jakob Disease Transmitted Via the Eye with Infected Cornea,” N. Engl. J. Med., 296 (1977): 1334–36.CrossRefGoogle Scholar
Thadani, V. et al. , “Creutzfeldt-Jakob Disease Probably Acquired from a Cadaveric Dura Mater Graft,” Journal of Neurosurgery, 69 (1988): 766–69.CrossRefGoogle Scholar
Bernouilli, C. et al. , “Danger of Accidental Person-to-Person Transmission of Creutzfeldt-Jakob Disease by Surgery,” Lancet, (1977): 478–79.CrossRefGoogle Scholar
Fradkin, J.E. et al. , “Creutzfeldt-Jakob Disease in Pituitary Growth Hormone Recipients in the United States,” JAMA, 265 (1991): 880–84.CrossRefGoogle Scholar
See Manuelidis, , supra note 7.Google Scholar
Evatt, B.L., “Current Status of Creutzfeldt-Jakob Disease and Blood Product Safety,” Haemophilia World, 2, no. 2 (1995): 34. But see Créange, A. et al. , “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.Google Scholar
See Esmonde, T.G.F. et al. , “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.CrossRefGoogle Scholar
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.Google Scholar
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).Google Scholar
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.Google Scholar
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).Google Scholar
Information Letter from Health Canada (Oct. 20, 1995) (on file with author).Google Scholar
Letter from Dr.Aye, Bert, National Director of Blood Services, to all Blood Bank directors and chief executive officers (July 17, 1995) (on file with author); and Memorandum from Dr.Aye, Bert, National Director of Blood Services, to all Blood Bank directors and chief executive officers (July 24, 1995) (on file with author).Google Scholar
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.Google Scholar
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).Google Scholar
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.Google Scholar
Walker, R., “Blood Recipients Joining Forces,” Herald (Calgary), May 1, 1996, at B1.Google Scholar
Immen, W., “Hospital Warns Parents of Blood Risk,” Globe and Mail (Toronto), May 4, 1996, at A1.Google Scholar
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.Google Scholar
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. et al. , “Diagnosis of Huntington Disease,” American Journal of Medical Genetics, 47 (1993): 368–74; and Tibben, A. et al. , “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.CrossRefGoogle Scholar
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.CrossRefGoogle Scholar
Bloch, M. et al. , “Predictive Testing for Huntington Disease in Canada,” American Journal of Medical Genetics, 42 (1992): 499507.CrossRefGoogle Scholar
Codori, A. Brandt, J., “Psychological Costs and Benefits of Predictive Testing for Huntington's Disease,” American Journal of Medical Genetics, 54 (1994): 174–84.CrossRefGoogle Scholar
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).Google Scholar
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).Google Scholar
927 F.2d 187.Google Scholar
In re Sealed Case, 61 F.3d 965 (D.C. Cir. 1995).Google Scholar
See id.; and Pittman Estate v. Bain [1994] 112 D.L.R.4th 257, 403–12 (Ont. High Ct.).Google Scholar
927 F.2d at 193; and 112 D.L.R.4th at 310.Google Scholar
Arato v. Avedon, 858 P.2d 598, 605 (Cal. 1993) (noting duty to disclose “dire prognosis”).Google Scholar
112 D.L.R.4th at 372.Google Scholar
Daly v. United States, 946 F.2d 1467 (9th Cir. 1991).Google Scholar
Union Carbide & Carbon Corp. v. Stapleton, 237 F.2d 229 (6th Cir. 1956).Google Scholar
M.M.H. v. United States, 966 F.2d 285 (7th Cir. 1992).Google Scholar
Miles, 927 F.2d at 194–95.Google Scholar
Hoemke v. New York Blood Center, 912 F.2d 550 (2d Cir. 1990).Google Scholar
See id. at 554.Google Scholar
Spann v. Irwin Memorial Blood Centers, 40 Cal. Rptr. 2d 360 (Cal. Ct. App. 1995).Google Scholar
American College of Legal Medicine, Legal Medicine (St. Louis: Mosby, 3rd ed., 1995): At 283.Google Scholar
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.).Google Scholar
This is the manner of notification used, for example, by the Calgary Regional Health Authority and Toronto's Hospital for Sick Children.Google Scholar
Kessler, S., “Psychiatric Implications of Presymptomatic Testing for Huntington's Disease,” American Journal of Orthopsychiatry, 51 (1987): 212–19.CrossRefGoogle Scholar
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): 4651.CrossRefGoogle Scholar
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.Google Scholar
This is the method preferred by Health Canada. See Information Letter from Health Canada, Health Protection Branch (Oct. 20, 1995) (on file with author).Google Scholar
Pittman Estate v. Bain suggests that it may not. See Pittman, 112 D.L.R.4th at 382–83.Google Scholar
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).Google Scholar
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).Google Scholar