Cystic Fibrosis Care Research Articles

Cystic Fibrosis-Related Diabetes in Poland.

Cystic fibrosis (CF) is the most common autosomal recessive inherited monogenic disease in Caucasians. As medical technology progresses and the quality of patient care improves, the survival time of patients with CF has increased, which results in more frequent comorbidities such as cystic fibrosis-related diabetes (CFRD). CFRD is the result of abnormal glucose metabolism characterized primarily by insulin deficiency, exacerbated periodically by insulin resistance. The aim of our study was to analyze the epidemiology of patients with CFRD in Poland on the basis of data collected from six CF treatment centers. Analyses were performed on 1157 CF patients who were treated at one of the six CF care centers. CFRD was diagnosed according to standard criteria. All data including demographics, types of CFTR mutations, CFRD duration, and microorganisms in the sputum were obtained from the patients’ medical history. Our study indicates that the prevalence of CFRD in Poland is 12.9%. CFRD was most often diagnosed between the ages of 11 and 20 (60% of patients), while 23% of patients were diagnosed between 21 and 30 years of age. Furthermore, we observed that approximately 3–5% of patients under the age of 10 had CFRD. We found out that the type of mutation did not affect the frequency of CFRD development. Factors that increased the risk of developing CFRD include underweight and chronic Pseudomonas aeruginosa infection. Due to the extended lifespan of CF patients, the number of CFRD patients is currently increasing. We believe that the results of our study may complement information from other studies or may be useful in planning health policy in Poland.

Exploring the barriers and enablers experienced by people with Cystic Fibrosis and their healthcare professionals in accessing, utilising and delivering maternity and Cystic Fibrosis care during the pre-conception to post-partum period: A mixed methods systematic review protocol.

Background Cystic Fibrosis (CF) is an autosomal recessive inherited multi-system disease that primarily affects the lungs and digestive system. New drug therapies and treatments are improving the lives of many people with CF. With improved life expectancy and increased quality of life, many people with CF are now contemplating parenthood and becoming pregnant, an aspiration that decades ago was almost unheard of. Given this quickly evolving and more positive health landscape, it is vital to understand how people with CF experience the care they receive whilst accessing and utilising fertility and maternity services. It is also important to explore the experiences of healthcare professionals involved in providing care during this period. The overall aim of the mixed-methods systematic review will be to explore the barriers and enablers experienced by people with CF and the healthcare professionals involved in their care in the pre-conception to post-partum period. Methods The proposed review will be conducted in accordance with the Joanna Briggs Institute (JBI) methodology for convergent integrated mixed methods systematic reviews. A systematic search of Medline (Ebsco), Cinahl, Embase, APA PsychINFO and Cochrane Library from inception to February 2022 will be conducted. Quantitative, qualitative and mixed methods studies pertaining to the experience of pre-conception to post-partum care for people with CF and their healthcare professionals will be included. Two independent reviewers will screen titles, abstracts and full texts with disagreements being resolved by a third reviewer. Conclusion This review will help to determine the potential barriers and facilitators experienced by people with Cystic Fibrosis and the health care professionals involved in their care during the pre-conception to post-partum period. The results will be of benefit specifically to the CF population and their healthcare providers when planning further studies in the area of fertility and pregnancy for this population and when delivering care.

"The Stakes Are Higher"- Patient and Caregiver Perspectives on Cystic Fibrosis Research and Personalized Medicine.

IntroductionMaking bench to bedside advances in cystic fibrosis (CF) care requires the sustained engagement and trust of people living with CF. However, there is a scarcity of studies exploring their concerns and priorities regarding research and its end products. The aim of this qualitative study was to generate empirical evidence regarding patient and caregiver perspectives on cystic fibrosis research and personalized medicine to foster developments in translational research in Canada.MethodsA total of 15 focus groups were conducted, engaging 22 adults with CF and 18 caregivers (e.g., parents, siblings and partners) living in Canada. Inductive thematic analysis relied on an iterative process involving themes derived from both participant meaning-making and existing scientific literature. Participant perspectives were considered along intrapersonal, intracommunity, interpersonal, and structural lines.ResultsOverall, participants described a relationship to CF research inextricable from the lived experience of CF as a lifelong progressive and terminal disease and from the goal of advancing medical science. They were enthusiastic and excited about the emergence of CFTR modulators, although they had some knowledge gaps regarding the associated research. They largely spoke to positive experiences with researcher communication but had feedback regarding informed consent processes and the return of study results. Participants also voiced concerns about structural access barriers to research and to its end products. Extensive histories of research participation, a relatively small and intercommunicative CF community, and structural overlap between research and care settings contributed to their perspectives and priorities.ConclusionStudy findings are valuable for researchers and policy-makers in CF and rare or progressive diseases more broadly. Continuing to solicit and listen to the voices of patients and caregivers is crucial for research ethics and the translation of new therapies in the area of personalized medicine.

Collaborative research protocol to define patient-reported experience measures of the cystic fibrosis care pathway in France: the ExPaParM study

IntroductionIn France, the cystic fibrosis (CF) care pathway is coordinated by multidisciplinary teams from specialised CF centres or transplant centres. It includes the care provided at home or out of hospital, risk prevention in daily life and adjustments to social life, which together contribute to the person’s quality of life. Patient experience is used to describe and evaluate the care and life of patients living with the disease.ObjectivesOur collaborative research aims to identify the most significant areas and criteria that characterise the CF pathway. It will lead to the development of a questionnaire to collect patients' experience, which can be administered to all patients or parents of children registered and followed in the centres. The article describes the protocol developed in partnership with patients and parents of children living with the disease.MethodA multidisciplinary research group brings together researchers, patients, parents of children with CF and health care professionals. The patient partnership is involved in the 4 phases of the protocol: (1) setting up the study, recruiting patient and parent co-researchers, training them in qualitative research methods, defining the situations and profiles of patients in the study population, elaborating the protocol; (2) selecting the study sites, recruiting participants, carrying out semi-structured interviews, analysing verbatims using the grounded theory approach; (3) co-elaborating Patient-Reported Experience Measures (PREM) questionnaires adapted to the 4 types of participants: parents, adolescents, non-transplanted adults and transplanted adults; (4) validating the construct with participants and professionals from the study centres.ResultsThe protocol obtained a favourable opinion from the Ethics Evaluation Committee of INSERM (IRB00003888—no. 20-700). Training was provided to the 5 patients and 2 parent co-researchers to enable them to participate effectively in the research. Eleven centres participated in the recruitment of participants in mainland France and Reunion Island. Eighty hours of interviews were conducted.DiscussionThe PREM questionnaires to be elaborated will have to undergo psychometric validation before being used by the actors of the CF network to assess the impact on the care pathways of quality approaches or new therapies available in cystic fibrosis.Trial Registration Registry: IRB00003888 – no. 20-700. Issue date: 06/09/2020.

Clinical outcomes in cystic fibrosis at 6 years of age with tricare insurance coverage

Health insurance coverage is associated with outcomes in cystic fibrosis (CF). A fraction of individuals in the United States are covered through Tricare, a federally funded program for military members and their dependents. The role of Tricare on CF health outcomes is unknown. Using a retrospective CF Foundation Patient Registry cohort born 2000–2011, insurance status was defined as any Tricare (n = 328) with reference groups of always private (n = 3,455) and exclusively public (n = 2,669) during the first 6 years of life. Subjects with Tricare coverage attended more CF care centers and lived in more zip codes by age 6 than their counterparts. BMI did not differ between groups. Subjects with Tricare had a higher FEV1 at age 6 compared to those with always public insurance. Overall, outcomes for those with Tricare insurance appeared more similar to those with always private insurance. Future research should consider treating Tricare coverage similar to private insurance.

What Do We Know about the Microbiome in Cystic Fibrosis? Is There a Role for Probiotics and Prebiotics?

Cystic fibrosis (CF) is a life-shortening genetic disorder that affects the cystic fibrosis transmembrane conductance regulator (CFTR) protein. In the gastrointestinal (GI) tract, CFTR dysfunction results in low intestinal pH, thick and inspissated mucus, a lack of endogenous pancreatic enzymes, and reduced motility. These mechanisms, combined with antibiotic therapies, drive GI inflammation and significant alteration of the GI microbiota (dysbiosis). Dysbiosis and inflammation are key factors in systemic inflammation and GI complications including malignancy. The following review examines the potential for probiotic and prebiotic therapies to provide clinical benefits through modulation of the microbiome. Evidence from randomised control trials suggest probiotics are likely to improve GI inflammation and reduce the incidence of CF pulmonary exacerbations. However, the highly variable, low-quality data is a barrier to the implementation of probiotics into routine CF care. Epidemiological studies and clinical trials support the potential of dietary fibre and prebiotic supplements to beneficially modulate the microbiome in gastrointestinal conditions. To date, limited evidence is available on their safety and efficacy in CF. Variable responses to probiotics and prebiotics highlight the need for personalised approaches that consider an individual’s underlying microbiota, diet, and existing medications against the backdrop of the complex nutritional needs in CF.

Opportunity for pharmacogenomic testing in patients with cystic fibrosis.

Patients with cystic fibrosis (CF) are exposed to many drugs in their lifetime and many of these drugs have Clinical Pharmacogenetics Implementation Consortium (CPIC) guidelines that are available to guide dosing. Contemporary CF treatments are targeted to specific mutations in the CF transmembrane conductance regulator (CFTR) gene, and thus, require patients to have genetic testing before initiation of modulator therapy. However, aside from CFTR genetic testing, pharmacogenomic testing is not standard of care for CF patients. The aim of this study is to determine the number of non-CFTR modulator medications with CPIC guidelines that are prescribed to patients with CF. We identified all patients with a diagnosis of CF and queried our hospital electronic medical records (EMR) for all orders, including inpatient and prescriptions, for all drugs or drug classes that have CPIC actionable guidelines for drug-gene pairs that can be used to guide therapy. We identified 576 patients with a diagnosis of CF that were treated at our institution during this 16-year period between June 2005 and May 2021. Of these patients, 504 patients (87.5%) received at least one drug that could have been dosed according to CPIC guidelines if pharmacogenomic results would have been available. Patients with CF have high utilization of drugs with CPIC guidelines, therefore preemptive pharmacogenomic testing should be considered in CF patients at the time of CFTR genetic testing.

Cystic fibrosis telemedicine in the era of COVID-19.

The coronavirus disease 2019 pandemic has resulted in large-scale changes to incorporate telemedicine for the delivery of care. People with cystic fibrosis (CF) have care considerations that pose challenges to telemedicine; they include frequent visits for pulmonary disease progression, medication management, and evaluation by a multidisciplinary team of providers. We share our center’s experience with video visits replacing in-person clinic evaluation, using quality improvement strategies to create a replicable workflow. Key considerations include incorporation of the multidisciplinary team into the visit, limitations of remote delivery of care, as well as patient and staff perceptions of this care model. Results revealed that video visits were convenient, efficacious, and comparable to in-person visits, with interest for its continued incorporation into the traditional CF care model.

Exploring provider attitudes and perspectives related to men's health in cystic fibrosis

BackgroundNew modulator therapies have markedly improved the health of people with cystic fibrosis (CF), allowing an increased focus on quality-of-life improvements for men with CF, including those related to sexual and reproductive health (SRH). This study explored CF providers’ attitudes and experiences with addressing men's health in CF. MethodsWe interviewed geographically diverse adult and pediatric United States (U.S.) CF program directors via semi-structured telephone interviews exploring their perspectives and practices related to men's SRH in CF. Two coders analyzed transcribed interviews and created a codebook to identify key themes. ResultsWe interviewed 20 providers and identified the following themes: 1) Men's SRH is important to address within CF care, but there is no standardization around this aspect of care; 2) There is no consensus about the recommendation or utilization of semen analysis to assess men's infertility; 3) There are many barriers to men's SRH care provision in CF centers, including the low priority of SRH concerns and provider discomfort and lack of expertise in SRH; 4) Providers desire clear evidence-based guidelines and patient resources related to men's SRH in CF; and 5) Providers believe future research should focus on testosterone and the impact of modulators on men's SRH. ConclusionsCF center directors acknowledge the importance of addressing SRH with men with CF, but there is a lack of standardization and research in this aspect of care. Existing barriers to optimal SRH care and identified facilitators in this study can serve as targets for interventions in the CF care model.

Real-World Outcomes in Cystic Fibrosis Telemedicine Clinical Care in a Time of a Global Pandemic

BackgroundDuring the COVID-19 pandemic, the University of Virginia adult cystic fibrosis (CF) center transitioned from in-person clinical encounters to a model that included interdisciplinary telemedicine. The pandemic presented an unprecedented opportunity to assess the impact of the interdisciplinary telemedicine model on clinical CF outcomes.Research QuestionWhat are the clinical outcomes of a care model that includes interdisciplinary telemedicine (IDC-TM) compared with in-person clinical care for patients with CF during the COVID-19 pandemic?Study Design and MethodsAdults with CF were included. The prepandemic year was defined as March 17, 2019, through March 16, 2020, and the pandemic year (PY) was defined as March 17, 2020, through March 16, 2021. Patients were enrolled starting in the PY. Prepandemic data were gathered retrospectively. Telemedicine visits were defined as clinical encounters via secured video communication. Hybrid visits were in-person evaluations by physician, with in-clinic video communication by other team members. In-person visits were encounters with in-person providers only. All encounters included previsit screening. Outcomes were lung function, BMI, exacerbations, and antibiotic use. FEV1 percent predicted, exacerbations, and antibiotic use were adjusted for the effect of elexacaftor/tezacaftor/ivacaftor treatment.ResultsOne hundred twenty-four patients participated. One hundred ten patients were analyzed (mean age, 35 years; range, 18-69 years). Ninety-five percent had access to telemedicine (n = 105). Telemedicine visits accounted for 64% of encounters (n = 260), hybrid visits with telemedicine support accounted for 28% of encounters (n = 114), and in-person visits accounted for 7% of encounters (n = 30). No difference in lung function or exacerbation rate during the PY was found. BMI increased from 25 to 26 kg/m2 (t100 = –4.72; P < .001). Antibiotic use decreased from 316 to 124 episodes (z = 8.81; P < .0001).InterpretationThis CF care model, which includes IDC-TM, successfully monitored lung function and BMI, identified exacerbations, and followed guidelines-based care during the pandemic. A significant decrease in antibiotic use suggests that social mitigation strategies were protective.Trial RegistryClinicalTrials.gov; No.: NCT04402801; URL: www.clinicaltrials.gov.

Financial impacts of the COVID-19 pandemic on cystic fibrosis care: lessons for the future

BackgroundChronic care delivery models faced unprecedented financial pressures, with a reduction of in-person visits and adoption of telehealth during the COVID-19 pandemic. We sought to understand the reported financial impact of pandemic-related changes to the cystic fibrosis (CF) care model. MethodsThe U.S. CF Foundation State of Care surveys fielded in Summer 2020 (SoC1) and Spring 2021 (SoC2) included questions for CF programs on the impact of pandemic-related restrictions on overall finances, staffing, licensure, and reimbursement of telehealth services. Descriptive analyses were conducted based on program type. ResultsAmong the 286 respondents (128 pediatric, 118 adult, 40 affiliate), the majority (62%) reported a detrimental financial impact to their CF care program in SoC1, though fewer (42%) reported detrimental impacts in SoC2. The most common reported impacts in SoC1 were redeployment of clinical staff (68%), furloughs (52%), hiring freezes (51%), decreases in salaries (34%), or layoffs (10%). Reports of lower reimbursement for telehealth increased from 30% to 40% from SoC1 to SoC2. Projecting towards the future, only a minority (17%) of program directors in SoC2 felt that financial support would remain below pre-pandemic levels. ConclusionsThe COVID-19 pandemic resulted in financial strain on the CF care model, including challenges with reimbursement for telehealth services and reductions in staffing due to institutional changes. Planning for the future of CF care model needs to address these short-term impacts, particularly to ensure a lack of interruption in high-quality multi-disciplinary care.

Barriers and facilitators to implementing telehealth services during the COVID-19 pandemic: A qualitative analysis of interviews with cystic fibrosis care team members

BackgroundThe COVID-19 pandemic forced cystic fibrosis (CF) care programs to rapidly shift from in-person care delivery to telehealth. Our objective was to provide a qualitative exploration of facilitators and barriers to: 1) implementing high-quality telehealth and 2) navigating reimbursement for telehealth services. MethodsWe used data from the 2020 State of Care CF Program Survey (n=286 U.S. care programs) administered in August-September to identify two cohorts of programs, with variation in telehealth quality (n=12 programs) and reimbursement (n=8 programs). We conducted focus groups and semi-structured interviews with CF program directors and coordinators in December 2020, approximately 9 months from onset of the pandemic. We used the Consolidated Framework for Implementation Research to identify facilitators and barriers of implementation, and inductive thematic analysis to identify facilitators and barriers of reimbursement. ResultsFactors differentiating programs with greater and lower perceived telehealth quality included telehealth characteristics (perceived advantage over in-person care, cost, platform quality); external influences (needs and resources of those served by the CF program), characteristics of the CF program (compatibility with workflows, relative priority, available resources); characteristics of team members (individual stage of change), and processes for implementation (engaging patients and teams). Reimbursement barriers included documentation to optimize billing; reimbursement of multi-disciplinary team members, remote monitoring, and telephone-only telehealth; and lower volume of patients. ConclusionsA number of factors are associated with successful implementation and reimbursement of telehealth. Future efforts should provide guidance and incentives that support telehealth delivery and infrastructure, share best practices across CF programs, and remove barriers.

Telehealth use in cystic fibrosis during COVID-19: Association with race, ethnicity, and socioeconomic factors

BackgroundDue to the COVID-19 pandemic, there was an uptake of telehealth in cystic fibrosis care. Previous studies show disparities in telehealth use based on socioeconomic status (SES). We aimed to: (1) understand telehealth use and perceptions and (2) identify the facilitators and barriers to telehealth use among people with CF and their families (PwCF) from diverse racial/ethnic and socioeconomic backgrounds. MethodsWe conducted an analysis of the 2020 Cystic Fibrosis State of Care surveys completed by PwCF (PFSoC), CF Care Programs (SoC1) and the CF Foundation Patient Registry (CFFPR). ResultsA total of 424 PwCF and 286 programs responded to the PFSoC and SoC1. Among PwCF, 90% self-identified as White, 6% as Hispanic/Latino, and 2% as Black. Racial/ethnic minorities were less likely to have had a telehealth visit (p=.015). This difference was pronounced among the Hispanic/Latino population (p<.01). Telehealth use did not differ by health insurance and was similarly offered independent of financial status. Compared to PwCF who denied financial constraints, those who reported financial difficulties found telehealth more difficult to use (p=.018) and were less likely to think that their concerns (p=.010) or issues that mattered most to them (p=.020) were addressed during telehealth. Programs perceived lack of technology, language barriers, and home conditions as barriers to telehealth in vulnerable populations. ConclusionPFSoC and SoC1 identified differences in telehealth use and care perceptions by ethnicity, race, and socioeconomic characteristics. Further studies are needed to understand how telehealth can change access to CF care in diverse subpopulations.

Design and methods for understanding the state of cystic fibrosis care amid the COVID-19 pandemic

BackgroundNovel therapies have dramatically changed cystic fibrosis (CF) and innovative care delivery systems are needed to meet future patient needs. Telehealth has been shown to be an efficient and desirable form of care delivery. The COVID-19 pandemic caused a rapid shift to telehealth, and this presented a unique opportunity to study facilitators, barriers, and satisfaction with this mode of care delivery. We aim to report survey methods, demographics and telehealth use among CF care programs, patients, and families during the pandemic. MethodsCF programs completed two surveys between July 29 and September 18, 2020, and between April 19 and May 19, 2021. Patients and families completed a similar survey between August 31 and October 30, 2020. The surveys addressed topics assessing the pandemic's financial impact, telehealth modes and experiences, licensure and reimbursement issues, health screening, and remote monitoring. Quantitative data were analyzed with descriptive statistics and were compared to the CF Foundation Patient Registry. ResultsMost programs (278 at timepoint one and 274 at timepoint two) provided telehealth during the pandemic. The percent of visits containing either telephone or video components changed from 45% to 25% over the time periods. Additionally, 424 patients and families from various ages and backgrounds responded to the survey and 81% reported having a telehealth visit. ConclusionsThe pandemic accelerated telehealth adoption and these datasets are a valuable source for exploring telehealth barriers and facilitators, the quality-of-care experience, financial and workforce implications, the impact on underrepresented populations, and implications for coverage and reimbursement.

Remote monitoring in telehealth care delivery across the U.S. cystic fibrosis care network

BackgroundCystic fibrosis (CF) programs and people with CF (PwCF) employed various monitoring methods for virtual care during the COVID-19 pandemic. This paper characterizes experiences with remote monitoring across the U.S. CF community. MethodsThe CF Foundation (CFF) sponsored distribution of home spirometers (April 2020 to May 2021), surveys to PwCF and CF programs (July to September 2020), and a second program survey (April to May 2021). We used mixed methods to explore access, use, and perspectives regarding the use of remote monitoring in future care. ResultsBy October 2020, 13,345 spirometers had been distributed, and 19,271 spirometers by May 2021. Programs (n=286) estimated proportions of PwCF with home devices increased over seven months: spirometers (30% to 70%), scales (50% to 70%), oximeters (5% to 10%) with higher estimates in adult programs for spirometers and oximeters. PwCF (n=378) had access to scales (89%), followed by oximeters (48%) and spirometers (47%), often using scales and oximeters weekly, and spirometers monthly. Over both surveys, some programs had no method to collect respiratory specimens for cultures associated with telehealth visits (47%, n=132; 41%, n=118). Most programs (81%) had a process for phlebotomy associated with a telehealth visit, primarily through off-site labs. Both PwCF and programs felt future care should advance remote monitoring and recommended improvements for access, training, and data collection systems. ConclusionsPwCF and programs experienced unprecedented access to remote monitoring and raised its importance for future care. Improvements to current systems may leverage these shared experiences to augment future care models.

Impact of the COVID-19 pandemic: How our response is shaping the future of cystic fibrosis care

The findings of this body of work are presented in the eight articles included in this supplement. The impact and perspectives of adult and pediatric care teams and patient/families are covered with special attention to mental health care, the financial and personnel impacts within care programs, the experiences of vulnerable and underrepresented patient populations, and implementation of remoting monitoring. Commentaries from colleagues provide a broader perspective, offering reflections on the findings and their implications regarding the future CF care model.

Eating disorders and body image in cystic fibrosis

Eating disorders and disturbed body image have been reported in individuals with cystic fibrosis (CF) and may contribute to poor weight gain, reduced lung function and increased mortality. CF individuals often look and feel different from their peers and bear the additional burden of body-altering side effects of treatment. As a result, the impact of disorders such as binge eating, anorexia nervosa, and bulimia nervosa may adversely affect the social, emotional, and physical development of those with CF.Multiple risk factors may contribute to the development of an eating disorder in CF. Growth failure is affected by the physical impairments of CF, including pancreatic insufficiency, high energy demands, respiratory infections, and delayed and stunted growth and puberty. Psychological factors, such as CF associated depression and anxiety, intense focus on BMI, lack of control in a chronic disease, and preoccupation with morbidity and mortality, likely further contribute. Exercise inefficiency, secondary to poor lung function, low BMI and pulmonary exacerbations, and the potential for medication manipulation are also additional risk factors.The intense scrutiny around BMI may lead to a poor relationship with food, including disordered eating habits, abnormal mealtime behaviors, and stressful caregiver-patient interactions regarding meals. This further contributes to a discrepancy between ideal CF nutritional standards and the reality of the challenges of appropriate daily energy intake for an individual with CF.It is imperative that CF providers are equipped to identify potential eating disorders and disturbed body image in their CF patients. Improved screening and monitoring practices should be developed and implemented, with multidisciplinary support from all CF care team members, including dietitians, mental health professionals, and social workers, to best support holistic care and optimize outcomes. Increased attention to these concerns may help reduce CF related morbidity and mortality.

The effect of the cystic fibrosis care center on outcomes after lung transplantation for cystic fibrosis

The purpose of this study was to evaluate outcomes in people with cystic fibrosis (CF) who underwent lung transplant (LT) at a transplant center with an accredited Cystic Fibrosis Care Center (CFCC) in the United States. We reviewed the Scientific Registry of Transplant Recipients for all adult patients with CF who received a first-time LT from 2005 to 2018. The primary outcome was graft failure. Unadjusted Kaplan-Meier analysis and adjusted multilevel Cox proportional hazards models were used to evaluate outcomes in CF patients undergoing lung transplantation at a CFCC. 2,573 patients with CF underwent a first time LT during the study period. Of the 68 lung transplantation centers, 50 were CFCCs (73.5%). After adjustment for potential confounders, patients who underwent lung transplantation at a hospital with an accredited CFCC had a 33% reduction in risk of death or re-transplantation compared to those transplanted at a hospital without an accredited CFCC (HR: 0.67, 95% CI: 0.56-0.82, p < 0.001). People with CF who undergo LT at a transplant center with a CFCC have improved graft survival and decreased need for re-transplantation compared to those who undergo LT at a non-CFCC, independent of volume.

Fertility preservation in women with cystic fibrosis pre-lung transplantation: A mixed methods study.

Explore the knowledge, experiences, preferences, and concerns related to fertility preservation as an option for building a biological family among women with cystic fibrosis. Convergent mixed methods study design. We recruited women with cystic fibrosis of childbearing age in the United States through cystic fibrosis centres, snowball sampling, and social media. Participants completed an anonymous survey about fertility and fertility preservation (n=50). We also conducted audio-recorded, semi-structured interviews with a subset of women to gain a better understanding of their perspectives (n=20). We transcribed the interviews verbatim and analysed them using thematic analysis. For the quantitative arm, 78% of women indicated that they would like to have a child in the future; however, 74% reported never having had conversations about fertility preservation with their providers. For the qualitative arm, four major themes emerged: (1) Women with cystic fibrosis have inadequate knowledge about fertility and fertility preservation; (2) fertility is a low priority area for the cystic fibrosis care team; (3) women with cystic fibrosis recommend that the cystic fibrosis care team provide specific fertility resources; and (4) providers and literature lack information on fertility and cystic fibrosis. Integrated findings identified that while the majority of women with cystic fibrosis want to become mothers in the future, including post-lung transplantation, they have not received education on fertility preservation, and there is a general lack of knowledge on the topic of fertility in cystic fibrosis. Women with cystic fibrosis desire to have children but have little knowledge about fertility preservation, and cystic fibrosis providers do not initiate family planning discussions. Findings from the study support that additional education is needed for women with cystic fibrosis who are considering parenthood. Clinical care models should include early, regular, and thoughtful discussions about reproductive health issues, including fertility preservation.

Management of Cystic Fibrosis during COVID-19: Patient Reported Outcomes based remote follow-up among CF patients in Denmark – A feasibility study

BackgroundPatients with cystic fibrosis (CF) are considered to be a COVID-19 risk group. In March 2020, a fast-track Patient Reported Outcome (PRO) solution was developed to ensure access to CF care without in-person hospital visits. This study investigated the feasibility of urgently replacing in-person appointments with remote monitoring using telephone consultations combined with PROs. We investigated patients and health care professionals' (HCPs) acceptance, recruitment rate, response rate, missing data, and attrition.MethodsWe included adult CF patients from the Department of Infectious Diseases at Aarhus University Hospital, Denmark between April and June 2020. Patients filled in a disease-specific questionnaire including relevant clinical aspects, performed home spirometry, and sent in a sputum sample before a scheduled telephone consultation. Twelve participants who completed the questionnaire and had a telephone consultation were interviewed. Three physicians and three nurses from the CF clinical team participated in a focus group interview.ResultsEighty patients were recruited for remote monitoring, and 41 patients filled in at least one questionnaire. Overall, both patients and HCPs found remote monitoring and use of PROs acceptable and useful. Patients experienced greater flexibility and found the questionnaire relevant and understandable but pointed out the need for items regarding mental health status and more adequate information about changes in follow-up and workflow.ConclusionUrgent reorganization of outpatient follow-up among CF patients due to COVID-19 was feasible in routine clinical practice. However, patient involvement should be a future point of attention to ensure a sustainable telehealth PRO solution.