The purpose of this study was to explore the Cystic Fibrosis (CF) community’s use of, goals for, and perspective about social media networks, as well as interest in including CF medical professionals within these forums. Understanding the dynamics within these networks is important for medical professionals to better meet patient needs, address gaps in care, and utilize this major support system to improve patient care and wellbeing. Sixty-six individuals in the CF community participated in an anonymous survey circulated through social media. The survey addressed both social media use and individuals’ current CF care teams. Most respondents had positive feelings towards their social media networks, CF care teams, and overall quality of life. Making friends and being part of a community, obtaining medical advice, learning from experiences of others with CF, tracking others’ health, fundraising, and having an outlet were considered important goals of social media use. Respondents felt most supported by their CF care teams in regards to answering medical questions, and least supported emotionally. There is a low level of involvement of medical professionals on these social media platforms, however there were strong variations in respondents’ interest in this involvement. These results support the need for future research into the dynamics of these social media networks and exploration into how medical professionals can best utilize them. These platforms have the potential to positively impact patient engagement, baseline knowledge, expectations for future disease progression, compliance, and psychosocial wellbeing, if we can play to their strengths.

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Framing Conversations Around Fertility: Experiences of People with Cystic Fibrosis, Their Parents, and Their Clinical Teams

ByNicole Charlotte Nevitt Master of Science in Genetic Counseling University of California, Irvine, 2023 Professor Eliezer Nussbaum, Chair

Cystic fibrosis (CF) is an autosomal recessive genetic condition caused by biallelic pathogenic variants in the CFTR gene. The condition is characterized by thick mucus that progressively causes damage to the respiratory and digestive systems. CF also affects fertility in both males and females. Approximately 95% of males with CF experience infertility, largely due to complete or partial congenital bilateral absence of the vas deferens (CBAVD), and even those with biallelic CFTR gene variants without clinical CF features can have CBAVD. CBAVD prevents mature sperm from leaving the body, rendering these males infertile by natural conception methods. However, assisted reproductive technology can help them achieve fertility. About 35% of females with CF face infertility due to altered cervical mucus, ovulation issues, or nutritional problems. Those with significant lung disease must also weigh the medical risks of pregnancy. With highly effective modulator therapy, pregnancy rates in the CF population have risen dramatically, but there remains a knowledge gap regarding individual fertility capabilities, likely due to inadequate fertility education. Currently, no guidelines exist on discussing fertility with people with CF (pwCF), and it is unclear how well available resources are utilized. An anonymous survey was distributed to pwCF, parents of pwCF, and healthcare providers from CF care centers via Exchange Forums through the CF Foundation, on social media platforms including Facebook, Reddit, Instagram, and LinkedIn, and online support group forums including CysticFibrosis.com and HealingWell.com. Participants were at least 18 years old, could read/write in English, and lived in the United States (US). Participants completed a fertility knowledge assessment and were asked to describe their experience with fertility discussions as part of their CF care, as well as how they hope fertility will be discussed in the future. A total of 116 pwCF and 24 parents from 27 states participated, along with 111 providers from all US regions. Responses to short answer questions revealed a strong desire for improved fertility care practices from all groups. Only about half of pwCF indicated satisfaction with their fertility care. Nearly two thirds of pwCF and parents reported fertility conversations were initiated by pwCF or parents rather than providers. Although various care team members engaged in fertility discussions, approximately one in six pwCF stated that no one had discussed fertility with them. Pulmonologists, specialty physicians, and genetic counselors were identified by all groups as best suited for fertility discussions. Most pwCF and parents preferred fertility conversations to occur during teenage years, contrasting with their reported experiences of discussions happening mostly after transitioning to adult care. Provider responses on current practices varied, but a consensus leaned towards discussing fertility during teenage and transition years. This study highlights a gap between current practices and the expectations of pwCF, parents, and providers regarding CF fertility care. All groups emphasized the importance of this issue and expressed a strong motivation to improve fertility care for pwCF and their families.

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