Paper
Thursday, July 22, 2004
9:30 AM - 10:00 AM
Thursday, July 22, 2004
2:30 PM - 3:00 PM
This presentation is part of : Posters I
Genetics and Ethics: Parents, Children and Neurofibromatosis 2
John G. Twomey, PhD, PNP, Graduate Program in Nursing, Institute of Health Professions at Mass. Gen Hosp, Boston, MA, USA, Deborah Cassidy, PhD, RN, Boston College, Chestnut Hill, MA, USA, and Catherine Bove, MEd, MGH Molecular Neurogenetics Unit, Charlestown, MA, USA.

Objective: To determine the factors that parents of children at risk for Neurofibromatosis 2 (NF2) describe as affecting their decision to have their child(ren) tested for the NF2 gene mutation Background: Genetic Disorder of Interest Neurofibromatosis 2 (NF2) is an autosomal dominant disorder that manifests itself primarily in the acoustic and cranial nerve systems. NF2 represents the type of genetic disorder that has theoretical arguments for and against presymptomatic illness in children. While the benefits of presymptomatic testing for this gene may be unquestioned by the families with the illness, the picture may not be so clear in reality. Given the fact that there is a high degree of somatic mosaicism in mutant NF2 genes and that expressivity is so variable, an uncritical acceptance of widespread genetic testing in asymptomatic children may not be advisable. Design: Descriptive survey method through use of a semi-structured interview guide Population: The group of families seeking care for NF2 at the Massachusetts General Hospital Neurogenetics Clinic, Boston, MA Methods: Using a questionnaire developed by two of the investigators a convenience sample of ten parents from nine families with NF2 with children at risk for the mutation were recruited by one of the investigators for the study. Findings: Parents described personal as well as past family medical history as driving their decision to test their children. Having coped with the illness, the overriding factor cited to test was the hope that the knowledge gained (particularly if the child was positive for the NF2 mutation) would allow the parents to better make decisions for their children regarding surveillance and treatment. Results of the decision-making process did not vary between those who did or not carry out molecular testing nor were there differences depicted between those whose children who tested positive or negative.

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Sigma Theta Tau International
July 22-24, 2004