Reproductive and sexual health in boys with cystic fibrosis: what do parents know and say?

OBJECTIVE: Improved life expectancy in cystic fibrosis (CF) alters the significance of developmentally relevant aspects such as sexual and reproductive health (SRH). Men with CF report parents are an important source of information about infertility, but parent perspectives remain unknown. The aim of this study was to systematically explore parents' knowledge, attitudes and behaviors regarding the SRH education of their sons. PATIENTS AND METHODS: Eligible participants were parents of boys with CF aged at least 6 years who completed a 61-item written questionnaire that was developed to measure parent knowledge and education about CF-specific aspects of SRH, and parent attitudes and behaviors about the CF-specific sexuality education of their sons. RESULTS: Eighty-four mothers (82%) and 64 fathers (70%) completed the questionnaire. All but one knew of probable infertility, being primarily informed at the time of diagnosis. Since then, 19% of parents reported subsequent discussions of SRH with their son's CF specialist, mostly initiated by parents. Adolescence was considered the most appropriate time for the initial education of boys. Sixty percent of parents worried how their sons would react. Ninety-five percent of parents said that they (alone or together with the CF specialist) should first inform their sons of probable infertility. Only 30% of parents were satisfied with their current knowledge. CONCLUSION: These parents are well informed about male infertility in CF. However, they have had little opportunity for subsequent discussion of SRH with health professionals since their son's diagnosis. As boys mature, subsequent SRH discussions by health professionals with parents are indicated.

Changing their minds with time: a comparison of hypothetical and actual reproductive behaviors in parents of children with cystic fibrosis.

OBJECTIVE: Newborn screening for cystic fibrosis, with appropriate counseling, enables carrier parents to be informed early about future reproductive choices. Previous studies have assessed attitudes toward reproductive decisions in a hypothetical pregnancy or have measured reproductive behaviors. We aimed to measure parent attitudes to reproductive technologies and to compare prospectively these attitudes with later reproductive behaviors. METHODS: Parents of children who had cystic fibrosis and were aged 2 to 7 years were surveyed at baseline using a written questionnaire that explored attitudes to prenatal testing and termination of pregnancy in a hypothetical pregnancy. Parent knowledge and access to genetic counseling services also were assessed. Five years later, we compared attitudes with actual reproductive behaviors. RESULTS: Fifty-six mothers participated at baseline, and 43 were resurveyed 5 years later. Parent knowledge of cystic fibrosis and genetics was very good. A total of 93% had met a genetic counselor at the time of diagnosis, and more than half had on at least 1 subsequent occasion. At baseline, 82% reported that they would be likely to have prenatal diagnosis in a subsequent pregnancy, and 56% reported that they would be likely to terminate an affected pregnancy. Twenty-seven mothers since had been pregnant, with prenatal diagnosis used in 33 of the 55 pregnancies. In 67%, the hypothetically reported behavior regarding use of prenatal testing was the same as their actual behavior. Five of the 33 tested pregnancies were affected; all ended in termination. Reproductive choices in relationship to the number of children wanted, together with attitudes toward prenatal diagnosis and termination of pregnancy, were dynamic over time, with decisions having changed in both directions. CONCLUSIONS: This cohort of parents has actively used reproductive technologies since the birth of a child who has cystic fibrosis that was diagnosed by newborn screening. The dynamic aspect of reproductive choices highlights the importance of ongoing access to genetic counseling beyond the initial period of diagnosis and education, regardless of whether parents report that they expect to use reproductive technologies.