Assessing the health impacts of parenthood on people with cystic fibrosis: the HOPeCF prospective cohort protocol.

People with cystic fibrosis (CF) are living longer and healthier lives with a growing number considering and pursuing parenthood. The decision of whether to become a parent is complex for people with CF, and CF is a major factor in reproductive decision-making. Unfortunately, in people with CF who become parents, there are no prospective studies of disease trajectory, no data on the impact of parenthood on mental health, disease self-management, or quality of life, and no research regarding non-genetic parenthood. Health Outcomes of Parents with CF (HOPeCF) is a prospective, multicentre observational cohort study which will enrol 146 new parents with CF of children less than 5 years of age. The primary aim of this 60-month study is to assess the rate of lung function decline as impacted by mental health, parental stress and responsibility, and the use of CF transmembrane conductance regulator modulators. In addition, we will conduct dyadic interviews with a subset of study participants and their key supports (partner/family/friend) to inform future interventions. This longitudinal, observational multicentre study is a necessary and timely step in understanding parental health outcomes in CF and will provide data essential for care guidance to people with CF, their partners, and healthcare providers. The University of Pittsburgh Institutional Review Board approved this study (STUDY23080161). As people with a variety of paediatric-onset chronic diseases are living longer and considering parenthood, these results may have widespread applicability and will be distributed at international meetings and submitted to peer-reviewed journals.

Kazmerski TM ，Stransky OM ，Lee M ，Prangley KA ，Jain R ，Georgiopoulos AM ，Shaw DS ，Taylor-Cousar J ... -  《BMJ Open Respiratory Research》

Parenthood impacts short-term health outcomes in people with cystic fibrosis.

People with cystic fibrosis (CF) are living longer, healthier lives. A growing number are considering parenthood. There is a substantial knowledge gap regarding the health impacts of parenthood on people with CF. Using data from the United Kingdom CF registry from 2015 to 2019, we evaluated the impact of having a child on percent predicted forced expiratory volume in 1 second (ppFEV1), body mass index (BMI), and pulmonary exacerbations via multivariable longitudinal data analysis adjusting for age and sex in the year before a person with CF became a parent to the first year following parenthood. We examined whether changes from pre- to post-parenthood differed by sex or CF transmembrane conductance regulator (CFTR) modulator use. Among 296 people with CF who became parents, we found a significant decrease in ppFEV1, (adjusted mean difference -3.19, CI: -4.31, -2.07; p<0.01) and BMI (adjusted mean difference -0.28, CI: -0.51, -0.05; p<0.02) and a significant increase in pulmonary exacerbations (adjusted rate ratio 1.3, CI: 1.13, 1.49; p<0.01) in the year following the birth of a child compared to the year prior. Further analysis showed that CFTR modulator use mitigated parenthood impacts on ppFEV1, but not on BMI and pulmonary exacerbations. Females experienced significantly worse impacts on BMI from pre- to post-parenthood compared to males. Parenthood adversely impacts short-term health outcomes for people with CF and CFTR modulator use mitigates such effects. To better understand the impact of the widespread use of highly effective CFTR modulators, longer-term, prospective parenthood studies are needed.

Kazmerski TM ，Jain R ，Lee M ，Taylor-Cousar JL ... -  《-》

A qualitative exploration of parenthood experiences and needs among people with cystic fibrosis.

As highly effective modulator therapy improves the lives of many people with cystic fibrosis (pwCF), more are considering or pursuing parenthood. This study explores the interplay between having CF and being a parent. We recruited pwCF with >1 child ≤10 years from CF Foundation Community Voice and participating CF centers to complete interviews exploring their decision-making process to become a parent, adjustment to parenthood with CF, the impact of CF on parenting, and the impact of parenting on CF care/adherence. We transcribed and thematically analyzed interviews using a deductive approach. Twenty-one mothers and 16 fathers participated (age 22-46 years). Key themes included: 1) The responsibilities of parenthood and the responsibilities of CF care often conflict, requiring creative multitasking and alterations to other aspects of life; 2) Delegating tasks to partners/family can alleviate conflicts between parenting and CF care; 3) While CF teams do not play a major role in decisions to become a parent, pwCF desire support/resources specific to parenting from their CF team; 4) It is logistically and emotionally difficult to avoid illnesses transmitted from children, but some parents use precautions to mitigate risk; and 5) Parents with CF desire clinic-facilitated connections with other parents with CF to share strategies and for emotional support. Parents with CF have unique challenges both as pwCF and as parents, and the intersection of these roles can impact their health outcomes and care adherence. Identifying common challenges may allow CF care teams to improve support of parents with CF.

Om S ，M P ，M B ，Jl TC ，Tm K ... -  《-》

Pursuing parenthood with cystic fibrosis: Reproductive health and parenting concerns in individuals with cystic fibrosis.

As life expectancy for cystic fibrosis (CF) has increased in recent decades, more individuals with CF are becoming parents. The objectives of this study were to describe the parenting and reproductive health concerns of individuals with CF and to identify the psychosocial and educational needs related to parenthood with CF. Twenty adults with CF, including parents and non-parents, participated in one-on-one, semi-structured interviews about reproductive health and parenting. Questions pertained to reproductive health knowledge, psychosocial adaptation to CF related to fertility and parenthood, parenting concerns in the context of CF, and psychosocial care needs. We performed thematic content analysis on interview transcripts and descriptive statistical analysis on participant demographics and health variables. A majority of participants (ten women and ten men, of whom half were parents) described their health as "stable" and "good/fair"; median FEV 1 was 66% predicted (range, 30-105). Participants shared a range of experiences related to reproductive health discussions with CF care providers and expressed concerns about pregnancy, infertility, and adoption. Parents and non-parents expressed concerns about balancing roles as parent and patient, the impact of anticipated health decline and early mortality on children, and communication with children. Participants identified a need for earlier, improved education for potential parents and resources for parents with CF. Individuals with CF may not receive sufficient CF-related reproductive health education, and they have wide-ranging concerns about the intersecting roles of patient and parent. Results from this study can provide guidance for CF care providers to improve their understanding and response to the needs of individuals and families affected by CF.

Hailey CE ，Tan JW ，Dellon EP ，Park EM ... -  《-》

Family-building and parenting considerations for people with cystic fibrosis.

As people with cystic fibrosis (CF) live longer and healthier lives, increasing numbers are considering the full range of reproductive options for their futures, including parenthood, pregnancy, or pregnancy prevention. As the face of CF changes, the CF care model must adapt to meet the reproductive health needs of both parents and nonparents with CF. This article summarizes the reproductive goals and family-building concerns faced by people with CF, including fertility, pregnancy, and alternative paths to parenthood, the impact of parenthood on mental and physical health, and important future research.

Kazmerski TM ，West NE ，Jain R ，Uluer A ，Georgiopoulos AM ，Aitken ML ，Taylor-Cousar JL ... -  《-》