Educating the Adolescent and Young Adult With Cystic Fibrosis About Their Reproductive Risks and Options

Abstract

We conducted a follow-up to our 2008 study assessing the genetic and reproductive knowledge of adolescents and young adults with cystic fibrosis (CF).1Houser GH Holt CL Clancy JP et al.Genetic and reproductive knowledge among adolescents and adults with cystic fibrosis.Chest. 2008; 133: 1533Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar The original study showed that only 33% of patients with CF knew that two carriers had a 25% chance of having a child with CF and that 25% knew that two carriers have a 50% chance of having a carrier child. Here, we report our effort to determine the efficacy of recent educational efforts made by organizations like the Cystic Fibrosis Foundation, who launched their 2010 webcast series to educate patients and families about CF.2Marshall BC Penland CM Hazle L et al.Cystic fibrosis foundation: achieving the mission.Respir Care. 2009; 54: 788-795Crossref PubMed Scopus (23) Google Scholar We used a 24-question survey. Twelve questions assessed demographics, such as age, sex, education level, and baseline health. Twelve questions assessed patient knowledge of genetics, inheritance, reproduction, and health concerns for patients with CF wanting to reproduce. We then provided each patient (N =40) with a trifold, color brochure addressing these topics and reassessed patient knowledge with patients able to reference the brochure. All study protocols and materials were approved by The University of Alabama (UAB) Institutional Review Board (Protocol X100704010). We found that 23% of patients knew that two carriers have a 75% chance of having a child who does not have CF and that 40% knew that the recurrence risk for two carriers to have a second child with CF was 25% (Table 1). These numbers increased to 38% and 65%, respectively, with the brochure. Fifty percent of patients knew that among patients with CF it is more difficult for the male sex to have children than for the female sex, and 47.5% knew that the male sex are infertile because sperm are unable to exit the testicles. These numbers increased to 75% and 87.5%, respectively, with the brochure.Table 1Survey Questions and Number of Correct Answers Before and After Access to Educational BrochureQuestionBefore Brochure CorrectAfter Brochure CorrectPercent ChangeIf you are a carrier of CF, what are the typical medical problems you will have?17 (42.5)24 (60)41.2If you have CF and your partner is a healthy noncarrier, what is the chance that your child will be a carrier?11 (27.5)24 (60)118What is the chance for parents who are both carriers to have a child that does not have CF?9 (22.5)21 (52.5)133If two parents with no sign of having CF already have a child with CF, what is the chance their next child could have CF?16 (40)26 (65)62.5When considering pregnancy, an individual with CF should:34aMore than one answer was deemed correct. The total sum of both answers is used in this table. (85)38 (95)11.8Is the chance of having children without help different for males and females with CF?20 (50)30 (75)50What is the main reason that a male with CF would be infertile?19 (47.5)35 (87.5)84.2What is the main reason that a female with CF would be infertile?13 (32.5)36 (90)177When considering pregnancy, what is the biggest risk for women with CF?13 (32.5)28 (70)115If you are infertile because of CF, what are your options to have a child?7 (17.5)18 (45)157If you have CF, can you adopt a child?28 (70)33 (82.5)17.8If you have CF and your partner is also a carrier, what technology can you use to ensure that your child does not have CF?5 (12.5)31 (77.5)520Data are presented as No. (%), unless otherwise indicated. CF = cystic fibrosis.a More than one answer was deemed correct. The total sum of both answers is used in this table. Open table in a new tab Data are presented as No. (%), unless otherwise indicated. CF = cystic fibrosis. The total percentage of correct knowledge responses showed an average of 40% for patients for the first survey assessing baseline knowledge. An average of 71% was achieved for the second survey given with the brochure as a reference, a difference of 31% (P < .0001). This study showed that despite recent efforts to improve patient education, there is no change in patients' baseline knowledge of their disease, at least in the UAB CF population. In 2010, 47.5% of patients with CF were adults.3Cystic Fibrosis Foundation Patient registry: annual data report 2010. Cystic Fibrosis Foundation website.http://www.cff.org/UploadedFiles/LivingWithCF/CareCenterNetwork/PatientRegistry/2010-Patient-Registry-Report.pdfGoogle Scholar As survival rates improve and patients with CF desire to have children, it is important that they be educated on the risks and challenges reproduction poses. This study showed that inexpensive paper brochures are a potentially effective way to address this deficit in patient knowledge. Role of sponsors: The sponsor had no role in the design of the study, the collection and analysis of the data, or in the preparation of the manuscript.