John C. Fletcher 
to patients, but it should be kept in a perspective of restraint that 
avoids raising unrealistic hopes. 
Secondly, sonic parents may have feelings of guilt about the in- 
herited mode of transmission of the disease. Guilt might increase 
the willingness of some parents to consider gene therapy. Although 
it is likely that few families are free from guilt, the key issue would 
be to protect against irrational choices made largely on the basis of 
guilt or denial of the child’s condition. Wide variations have been 
found (Hewett, 1 976, p. 44) in studies of this problem among parents 
of handicapped children. Generalizations are dangerous about the 
role of guilt in parental choices concerning their genetically affected 
children. Indeed, some parents of handicapped infants are overly 
protective of their general welfare, rather than being excessively 
willing to take risks. The issue, however, ought to be a source of 
caution for researchers. How familiar will researchers be with the 
psychological aspects of the parents’ relationship to the child? 
Thirdly, if possible, the assent of the child for the study should 
be required. Extreme immaturity may prevent assent in some cases. 
However, if there is a choice between older and younger children 
as the first subjects in a trial, one should select the older children 
first, especially if they have some awareness of their situation and 
can participate in giving assent. Will the older children suffer from 
any cognitive or mental deficits that may impair their ability to 
assent? Children with Lesch-Nyhan syndrome suffer from mild to 
moderate retardation and dysarthric speech (Emery and Rimoin, 
1983, p. 1289). The children with ADA and PNF deficiency might 
be too young to assent. 
For these reasons, the IRB may want to consider adding special 
protections to assure that the consent of the parents, or next-of-kin, 
is well-considered and that the child’s best interests have been care- 
fully weighed. One available option is to assign a member or a con- 
sultant from the IRB to fulfill this function. Another method is to 
assign a group consent auditor (GCA). The Clinical Center, NIH 
(Report of a Working Party, 1984), has recently discussed this option 
to assure the soundness of next-of-kin substitute consent in research 
with cognitively and mentally impaired human subjects of research. 
Originally planned for patients who suffer from dementias due to 
Alzheimer’s disease, Korsakoff’s psychosis, or schizophrenia, the 
practice could easily be adapted to research with children. In the 
event that an impaired subject is both incompetent to give consent 
and also unable to designate a representative to consent for a study, 
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