Parental attitudes regarding carrier testing in children at risk for fragile X syndrome.

Sixty-five parents of individuals affected by fragile X syndrome who attended the National Fragile X Conference in Portland, Oregon (1996), were asked to complete a survey assessing parental level of concern about carrier testing in children at risk for fragile X syndrome. All subjects completed a 15-item paper and pencil Likert response scale measure that was developed specifically for this study. The items included parental rights and duties, psychological adjustment, adaptation, discrimination, harm, childbearing, and interpersonal relationships. The major concern of the parents was that their children have knowledge of their carrier status prior to becoming sexually active and that their children be able to marry informed of their genetic risk. Mothers were significantly more concerned than fathers about raising their children with the knowledge of their carrier status. A sense of parental right to make the decision regarding carrier testing for children was associated with concerns about (1) behavioral or educational problems, (2) knowledge of carrier status prior to sexual activity or marriage, and (3) adjustment of the children to knowledge of their carrier status. As the sample was drawn from a unique population of parents, the results of this survey should be interpreted with caution. The findings of this study suggest a model of parents providing anticipatory guidance for their children to help them adjust to carrier information and for their children to have this knowledge prior to the possibility of reproduction.

[1]  G. Spiridigliozzi,et al.  Carrier testing in the fragile X syndrome: attitudes and opinions of obligate carriers. , 1997, American journal of medical genetics.

[2]  T. Webb,et al.  Prevalence of fragile X syndrome. , 1996, Journal of medical genetics.

[3]  B. D. de Vries,et al.  Mental status of females with an FMR1 gene full mutation. , 1996, American journal of human genetics.

[4]  É. Khandjian,et al.  Prevalence of carriers of premutation-size alleles of the FMRI gene--and implications for the population genetics of the fragile X syndrome. , 1995, American journal of human genetics.

[5]  A. Clarke,et al.  The genetic testing of children. Working Party of the Clinical Genetics Society (UK) , 1994, Journal of medical genetics.

[6]  P. Reilly,et al.  Genetic testing for children and adolescents. Who decides? , 1994, JAMA.

[7]  C. Gullion,et al.  Aberrant behaviors of young boys with fragile X syndrome. , 1994, American journal of mental retardation : AJMR.

[8]  N. Sharpe Presymptomatic testing for Huntington disease: is there a duty to test those under the age of eighteen years? , 1993, American journal of medical genetics.

[9]  C. Williams Papers presented at the 2nd North American Conference on Angelman syndrome: Introduction , 1993 .

[10]  R. Hagerman,et al.  Learning disabilities and attentional problems in boys with the fragile X syndrome. , 1985, American journal of diseases of children.

[11]  D. Brodzinsky,et al.  Children's understanding of adoption. , 1984, Child development.

[12]  T. Marteau,et al.  Long-term cognitive and emotional impact of genetic testing for carriers of cystic fibrosis: the effects of test result and gender. , 1997, Health psychology : official journal of the Division of Health Psychology, American Psychological Association.

[13]  S. Michie Predictive genetic testing in children: paternalism or empiricism? , 1996 .

[14]  M. Stacey The troubled helix: The new genetics: a feminist view , 1996 .

[15]  A. Clarke,et al.  The troubled helix: The genetic testing of children: a clinical perspective , 1996 .

[16]  S. Shiloh,et al.  Perception of risk in genetic counseling , 1989 .