@Article{info:doi/10.2196/59686,
author="Moody, Louise
and Clarke, Samantha
and Compton, Matt
and Hughson-Gill, Rachael
and Boardman, Felicity
and Clark, Corinna
and Holder, Pru
and Bonham, R. James
and Chudleigh, Jane",
title="Development of an Online Scenario-Based Tool to Enable Research Participation and Public Engagement in Cystic Fibrosis Newborn Screening: Mixed Methods Study",
journal="J Particip Med",
year="2025",
month="Mar",
day="6",
volume="17",
pages="e59686",
keywords="extended genetic testing",
keywords="next-generation sequencing",
keywords="cystic fibrosis",
keywords="decision-making",
keywords="engagement",
abstract="Background: Newborn screening aims to identify babies affected by rare but serious genetic conditions. As technology advances, there is the potential to expand the newborn screening program following evaluation of the likely benefits and drawbacks. To inform these decisions, it is important to consider the family experience of screening and the views of the public. Engaging in public dialogue can be difficult. The conditions, screening processes, and associated moral and ethical considerations are complex. Objective: This study aims to develop a stand-alone online resource to enable a range of stakeholders to understand whether and how next-generation sequencing should be incorporated into the CF screening algorithm. Methods: Around 4 development workshops with policymakers, parents, and other stakeholders informed the design of an interactive activity, including the structure, content, and questions posed. Stakeholders were recruited to take part in the development workshops via purposeful and snowball sampling methods to achieve a diversity of views across roles and organizations, with email invitations sent to representative individuals with lived, clinical, and academic experience related to CF and screening. Ten stakeholders informed the development process including those with lived experience of CF (2/10, 20\%), clinicians (2/10, 20\%), and representatives from relevant government, charity, and research organizations (6/10, 60\%). Vignettes constructed using interview data and translated into scripts were recorded to provide short films to represent and provoke consideration of families' experiences. Participants were recruited (n=6, adults older than 18 years) to test the resulting resource. Study advertisements were circulated via physical posters and digital newsletters to recruit participants who self-identified as having a reading difficulty or having English as a second language. Results: An open access online resource, ``Cystic Fibrosis Newborn Screening: You Decide,'' was developed and usability and acceptability tested to provide the ``user'' (eg, a parent, the general public, or a health care professional) with an interactive scenario-based presentation of the potential outcomes of extended genetic testing, allowing them to visualize the impact on families. This included a learning workbook that explains key concepts and processes. The resulting tool facilitates public engagement with and understanding of complex genetic and screening concepts. Conclusions: Online resources such as the one developed during this work have the potential to help people form considered views and facilitate access to the perspectives of parents and the wider public on genetic testing. These may be otherwise difficult to obtain but are of importance to health care professionals and policymakers. Trial Registration: ClinicalTrials.gov NCT06299566; https://clinicaltrials.gov/study/NCT06299566 ",
doi="10.2196/59686",
url="https://jopm.jmir.org/2025/1/e59686",
url="http://www.ncbi.nlm.nih.gov/pubmed/40053726"
}


@Article{info:doi/10.2196/62931,
author="Moumjid, Nora
and Gotte, Constance
and Hommey, Sophie
and Poupon Bourdy, St{\'e}phanie
and Haesebaert, Julie
and Durieu, Isabelle
and Reynaud, Quitterie",
title="Mixed Comparative Evaluation of a Training Program Dedicated to Cystic Fibrosis Reference Centers: Protocol for the Pilot Implementation of Shared Decision-Making in the Treatment of Diabetes in Adult Patients With Cystic Fibrosis",
journal="JMIR Res Protoc",
year="2025",
month="Jan",
day="28",
volume="14",
pages="e62931",
keywords="shared decision-making",
keywords="implementation",
keywords="training",
keywords="decision aid",
keywords="cystic fibrosis",
abstract="Background: Diabetes affects half of the patients with cystic fibrosis who are aged 30 years and older. Diabetes progresses asymptomatically over a long period of time. Two treatment options are possible: start insulin as soon as cystic fibrosis diagnosis is made with the additional constraints of cystic fibrosis or wait while monitoring the patient's clinical condition and start insulin when diabetes symptoms develop and therefore later. This situation is particularly well suited to shared decision-making (SDM) between the physician (health care team) and patient/relatives. Objective: The aim of this study was to perform qualitative and quantitative analyses for evaluating the outcomes and experience of SDM implementation between the physician/health care team trained for SDM and patients/their relatives for cystic fibrosis--related diabetes. Methods: A quasi-experimental with a comparison study will be developed. Three cystic fibrosis reference centers (CFRCs) will be trained in SDM by using a web-based training, including a validated decision aid and coaching for physicians and the medical team. Two control CFRCs will maintain their usual practices. A qualitative analysis through observation of consultations, individual semistructured interviews with patients, and focus groups in CFRCs will be conducted based on a thematic content analysis. Questionnaires related to decision-making and experience of decision-making with and without SDM implementation will be administered to patients and physicians. Results: Forty patients will be included (8 patients in each center), that is, 60 consultation observations (2 consultations per patient in the intervention groups given the modalities of the SDM process) will be conducted in 2025. Eight focus groups will be conducted in the 5 centers (2 groups in each intervention CFRC and 1 group in each control CFRC). This qualitative corpus plus responses to the patient and physician questionnaires will make it possible to know whether the practice of SDM in CFRCs is increased by an implementation strategy and to analyze the experience of patients and their relatives regarding decision-making modalities. Analysis of the outcomes and experience of the implementation of SDM are of importance to identify the facilitators and barriers to SDM from patients' and CFRCs' point of views. Conclusions: Our study will give us keys to adapt, improve, and disseminate SDM more widely in the context of cystic fibrosis therapy. SDM could thus be used in routine clinical practice in CFRCs at the national level. Trial Registration: ClinicalTrials.gov NCT04891159; https://clinicaltrials.gov/study/NCT04891159?id=NCT04891159 International Registered Report Identifier (IRRID): PRR1-10.2196/62931 ",
doi="10.2196/62931",
url="https://www.researchprotocols.org/2025/1/e62931"
}


@Article{info:doi/10.2196/49941,
author="Duncan, L. Christina
and Muther, F. Emily
and Lindwall, J. Jennifer
and Durkin, Kristine
and Ruvalcaba, Elizabeth
and Williamson, Eliza
and Ahrabi-Nejad, Corrine
and Bord, Evelyn
and Green, Angela
and Harrison, L. Megan
and Polineni, Deepika",
title="Using Community Engagement to Create a Telecoaching Intervention to Improve Self-Management in Adolescents and Young Adults With Cystic Fibrosis: Qualitative Study",
journal="J Particip Med",
year="2025",
month="Jan",
day="20",
volume="17",
pages="e49941",
keywords="cystic fibrosis",
keywords="telecoaching",
keywords="self-management",
keywords="community engagement",
keywords="community partner",
keywords="intervention development",
abstract="Background: Adolescents and young adults (AYA) with cystic fibrosis (CF) are at risk for deviating from their daily treatment regimen due to significant time burden, complicated daily therapies, and life stressors. Developing patient-centric, effective, engaging, and practical behavioral interventions is vital to help sustain therapeutically meaningful self-management. Objective: This study aimed to devise and refine a patient-centered telecoaching intervention to foster self-management in AYA with CF using a combination of intervention development approaches, including an evidence- and theory-based approach (ie, applying existing theories and research evidence for behavior change) and a target population--centered approach (ie, intervention refinement based on the perspectives and actions of those individuals who will use it). Methods: AYA with CF, their caregivers, and health professionals from their CF care teams were recruited to take part in focus groups (or individual qualitative interviews) through a video call interface to (1) obtain perspectives on the overall structure and logistics of the intervention (ie, Step 1) and (2) refine the overall framework of the intervention and obtain feedback on feasibility, content, materials, and coach training (ie, Step 2). Qualitative data were analyzed using a reflexive thematic analysis process. Results were used to create and then modify the intervention structure and content in response to community partner input. Results: For Step 1, a total of 31 AYA and 20 clinicians took part in focus groups or interviews, resulting in 2 broad themes: (1) video call experience and (2) logistics and content of intervention. For Step 2, a total of 22 AYA, 18 clinicians, and 11 caregivers completed focus groups or interviews, yielding 3 major themes: (1) intervention structure, (2) intervention materials, and (3) session-specific feedback. Our Step 1 qualitative findings helped inform the structure (eg, telecoaching session frequency and duration) and approach of the telecoaching intervention. Step 2 qualitative results generally suggested that community partners perceived the feasibility and practicality of the proposed telecoaching intervention in promoting self-management in the face of complex treatment regimens. Extensive specific feedback was used to refine our telecoaching intervention before its efficacy testing in subsequent research. The diverse community partner input was critical in optimizing and tailoring our telecoaching intervention. Conclusions: This study documents the methods and results for engaging key community partners in creating an evidence-based behavioral intervention to promote self-management in AYA with CF. Incorporating the lived experiences and perspectives of community partners is essential when devising tailored and patient-centered interventions. ",
doi="10.2196/49941",
url="https://jopm.jmir.org/2025/1/e49941"
}


@Article{info:doi/10.2196/51753,
author="Le Roux, Enora
and Ursino, Moreno
and Milovanovic, Ivana
and Picq, Paul
and Haignere, Jeremie
and Rault, Gilles
and Pougheon Bertrand, Dominique
and Alberti, Corinne",
title="Home-Based Connected Devices Combined With Statistical Process Control for the Early Detection of Respiratory Exacerbations by Patients With Cystic Fibrosis: Pilot Interventional Study With a Pre-Post Design",
journal="JMIR Form Res",
year="2024",
month="Oct",
day="28",
volume="8",
pages="e51753",
keywords="connected devices",
keywords="cystic fibrosis",
keywords="patient education",
keywords="self-management",
keywords="medical device",
keywords="home monitoring",
keywords="remote monitoring",
keywords="statistical process control",
keywords="connected health",
keywords="alerts",
abstract="Background: Currently, patients with cystic fibrosis do not routinely monitor their respiratory function at home. Objective: This study aims to assess the clinical validity of using different connected health devices at home to measure 5 physiological parameters to help prevent exacerbations on a personalized basis from the perspective of patient empowerment. Methods: A multicenter interventional pilot study including 36 patients was conducted. Statistical process control---the cumulative sum control chart (CUSUM)---was used with connected health device measures with the objective of sending patients alerts at a relevant time in order to identify their individual risk of exacerbations. Associated patient education was delivered. Quantitative and qualitative data were collected. Results: One-half (18/36) of the patients completed the protocol through the end of the study. During the 12-month intervention, 6162 measures were collected with connected health devices, 387 alerts were sent, and 33 exacerbations were reported. The precision of alerts to detect exacerbations was weak for all parameters, which may be partly related to the low compliance of patients with the measurements. However, a decrease in the median number of exacerbations from 12 months before the study to after the 12-month intervention was observed for patients. Conclusions: The use of connected health devices associated with statistical process control showed that it was not acceptable for all patients, especially because of the burden related to measurements. However, the results suggest that it may be promising, after adaptations, for early identification and better management of exacerbations. Trial Registration: ClinicalTrials.gov NCT03304028; https://clinicaltrials.gov/study/NCT03304028 ",
doi="10.2196/51753",
url="https://formative.jmir.org/2024/1/e51753"
}


@Article{info:doi/10.2196/54942,
author="Oppelaar, C. Martinus
and Emond, Yvette
and Bannier, E. Michiel A. G.
and Reijers, E. Monique H.
and van der Vaart, Hester
and van der Meer, Renske
and Altenburg, Josje
and Conemans, Lennart
and Rottier, L. Bart
and Nuijsink, Marianne
and van den Wijngaart, S. Lara
and Merkus, M. Peter J. F.
and Heinen, Maud
and Roukema, Jolt",
title="Potential, Pitfalls, and Future Directions for Remote Monitoring of Chronic Respiratory Diseases: Multicenter Mixed Methods Study in Routine Cystic Fibrosis Care",
journal="J Med Internet Res",
year="2024",
month="Aug",
day="6",
volume="26",
pages="e54942",
keywords="telemonitoring",
keywords="digital health",
keywords="chronic respiratory diseases",
keywords="telespirometry",
keywords="interviews",
keywords="mixed methods",
keywords="qualitative study",
keywords="remote monitoring",
keywords="evaluation",
keywords="cystic fibrosis",
keywords="pediatrics",
keywords="mixed method",
keywords="observational study",
keywords="health care professionals",
keywords="semistructured interview",
keywords="psychosocial",
keywords="clinicians",
keywords="researchers",
keywords="policy makers",
keywords="telehealth",
abstract="Background: The current literature inadequately addresses the extent to which remote monitoring should be integrated into care models for chronic respiratory diseases (CRDs). Objective: This study examined a remote monitoring program (RMP) in cystic fibrosis (CF) by exploring experiences, future perspectives, and use behavior over 3 years, with the aim of developing future directions for remote monitoring in CRDs. Methods: This was a mixed methods, multicenter, observational study in 5 Dutch CF centers following a sequential explanatory design. Self-designed questionnaires using the technology acceptance model were sent out to people with CF who had a minimum of 12 months of experience with the RMP and local health care professionals (HCPs). Questionnaire outcomes were used to inform semistructured interviews with HCPs and people with CF. Qualitative findings were reported following the COREQ (Consolidated Criteria for Reporting Qualitative Research) checklist. Anonymous data on use frequency of all people with CF were analyzed. Results: Between the second quarter of 2020 and the end of 2022, a total of 608 people with CF were enrolled in the program, and a total of 9418 lung function tests and 2631 symptom surveys were conducted. In total, 65\% (24/37) of HCPs and 89\% (72/81) of people with CF responded to the questionnaire, and 7 HCPs and 12 people with CF participated in semistructured interviews. Both people with CF and HCPs were positive about remote monitoring in CF care and found the RMP a good addition to daily care (people with CF: 44/72, 61\%; HCPs: 21/24, 88\%). Benefits ranged from supporting individual patients to reducing health care consumption. The most valued monitoring tool was home spirometry by both people with CF (66/72, 92\%) and HCPs (22/24, 92\%). Downsides included the potential to lose sight of patients and negative psychosocial effects, as 17\% (12/72) of people with CF experienced some form of stress due to the RMP. A large majority of people with CF (59/72, 82\%) and HCPs (22/24, 92\%) wanted to keep using the RMP in future, with 79\% (19/24) of HCPs and 75\% (54/72) of people with CF looking forward to more replacement of in-person care with digital care during periods of well-being. Future perspectives for the RMP were centered on creating hybrid care models, personalizing remote care, and balancing individual benefits with monitoring burden. Conclusions: Remote monitoring has considerable potential in supporting people with CF and HCPs within the CF care model. We identified 4 practice-based future directions for remote monitoring in CF and CRD care. The strategies, ranging from patient driven to prediction driven, can help clinicians, researchers, and policy makers navigate the rapidly changing digital health field, integrate remote monitoring into local care models, and align remote care with patient and clinician needs. ",
doi="10.2196/54942",
url="https://www.jmir.org/2024/1/e54942",
url="http://www.ncbi.nlm.nih.gov/pubmed/39106098"
}


@Article{info:doi/10.2196/50527,
author="Mazzocato, Pamela
and Luckhaus, Linnea Jamie
and Malmqvist Castillo, Moa
and Burnett, Johan
and Hager, Andreas
and Oates, Gabriela
and Wannheden, Carolina
and Savage, Carl",
title="A Patient-Driven Mobile Health Innovation in Cystic Fibrosis Care: Comparative Cross-Case Study",
journal="J Med Internet Res",
year="2024",
month="Jul",
day="31",
volume="26",
pages="e50527",
keywords="chronic illness",
keywords="implementation",
keywords="adoption",
keywords="spread",
keywords="patient-driven innovation",
keywords="mHealth",
keywords="mobile health",
keywords="innovation",
keywords="health care provider",
keywords="motivation",
keywords="interdependency",
keywords="adaptability",
abstract="Background: Patient-driven innovation in health care is an emerging phenomenon with benefits for patients with chronic conditions, such as cystic fibrosis (CF). However, previous research has not examined what may facilitate or hinder the implementation of such innovations from the provider perspective. Objective: The aim of this study was to explain variations in the adoption of a patient-driven innovation among CF clinics. Methods: A comparative multiple-case study was conducted on the adoption of a patient-controlled app to support self-management and collaboration with health care professionals (HCPs). Data collection and analysis were guided by the nonadoption, abandonment, spread, scale-up, and sustainability and complexity assessment tool (NASSS-CAT) framework. Data included user activity levels of patients and qualitative interviews with staff at 9 clinics (n=8, 88.9\%, in Sweden; n=1, 11.1\%, in the United States). We calculated the maximum and mean percentage of active users at each clinic and performed statistical process control (SPC) analysis to explore how the user activity level changed over time. Qualitative data were subjected to content analysis and complexity analysis and used to generate process maps. All data were then triangulated in a cross-case analysis. Results: We found no evidence of nonadoption or clear abandonment of the app. Distinct patterns of innovation adoption were discernable based on the maximum end-user activity for each clinic, which we labeled as low (16\%-23\%), middle (25\%-47\%), or high (58\%-95\%) adoption. SPC charts illustrated that the introduction of new app features and research-related activity had a positive influence on user activity levels. Variation in adoption was associated with providers' perceptions of care process complexity. A higher perceived complexity of the value proposition, adopter system, and organization was associated with lower adoption. In clinics that adopted the innovation early or those that relied on champions, user activity tended to plateau or decline, suggesting a negative impact on sustainability. Conclusions: For patient-driven innovations to be adopted and sustained in health care, understanding patient-provider interdependency and providers' perspectives on what generates value is essential. ",
doi="10.2196/50527",
url="https://www.jmir.org/2024/1/e50527",
url="http://www.ncbi.nlm.nih.gov/pubmed/39083342"
}


@Article{info:doi/10.2196/54322,
author="Ladune, Raphaelle
and Hayotte, Meggy
and Vuillemin, Anne
and d'Arripe-Longueville, Fabienne",
title="Development of a Web App to Enhance Physical Activity in People With Cystic Fibrosis: Co-Design and Acceptability Evaluation by Patients and Health Professionals",
journal="JMIR Form Res",
year="2024",
month="Jul",
day="30",
volume="8",
pages="e54322",
keywords="cystic fibrosis",
keywords="decisional balance",
keywords="digital app",
keywords="acceptability",
keywords="physical activity",
keywords="mobile phone",
abstract="Background: Cystic fibrosis (CF) is a genetic disease affecting the respiratory and digestive systems, with recent treatment advances improving life expectancy. However, many people with CF lack adequate physical activity (PA). PA can enhance lung function and quality of life, but barriers exist. The Cystic Fibrosis Decisional Balance of Physical Activity questionnaire assesses the decisional balance for PA in adults with CF, but it is not optimal for clinical use. A digital app might overcome this limitation by improving the efficiency of administration, interpretation of results, and communication between patients and health care professionals. Objective: This paper presents the development process and reports on the acceptability of a web app designed to measure and monitor the decisional balance for PA in people with CF. Methods: This study comprised two stages: (1) the co-design of a digital app and (2) the evaluation of its acceptability among health care professionals and people with CF. A participatory approach engaged stakeholders in the app's creation. The app's acceptability, based on factors outlined in the Unified Theory of Acceptance and Use of Technology 2, is vital for its successful adoption. Participants volunteered, gave informed consent, and were aged >18 years and fluent in French. Data collection was performed through qualitative interviews, video presentations, surveys, and individual semistructured interviews, followed by quantitative and qualitative data analyses. Results: In total, 11 health care professionals, 6 people with CF, and 5 researchers were involved in the co-design phase. Results of this phase led to the coconstruction of an app named MUCO\_BALAD, designed for people with CF aged ?18 years, health care professionals, and researchers to monitor the decisional balance for PA in people with CF. In the acceptability evaluation phase, the sample included 47 health care professionals, 44 people with CF, and 12 researchers. The analysis revealed that the acceptability measures were positive and that app acceptability did not differ according to user types. Semistructured interviews helped identify positive and negative perceptions of the app and the interface, as well as missing functionalities. Conclusions: This study assessed the acceptability of an app and demonstrated promising qualitative and quantitative results. The digital tool for measuring the decisional balance in PA for people with CF is encouraging for health care professionals, people with CF, and researchers, according to the valuable insights gained from this study. ",
doi="10.2196/54322",
url="https://formative.jmir.org/2024/1/e54322",
url="http://www.ncbi.nlm.nih.gov/pubmed/39078689"
}


@Article{info:doi/10.2196/55718,
author="Morrison, Lisa
and Saynor, Louise Zoe
and Kirk, Alison
and McCann, Lisa",
title="Revolutionizing Care: Unleashing the Potential of Digital Health Technology in Physiotherapy Management for People With Cystic Fibrosis",
journal="JMIR Rehabil Assist Technol",
year="2024",
month="Jul",
day="15",
volume="11",
pages="e55718",
keywords="cystic fibrosis",
keywords="physiotherapy",
keywords="digital technology",
keywords="telehealth",
keywords="cystic fibrosis transmembrane regulator modulators",
keywords="telemedicine",
keywords="digital health technology",
keywords="DHTs",
keywords="digital health",
keywords="physical therapy",
keywords="physical activity",
keywords="exercise",
keywords="monitoring",
keywords="physiotherapists",
keywords="user",
keywords="experience",
keywords="remote",
keywords="virtual care",
keywords="consultation",
keywords="consultations",
keywords="eConsultations",
keywords="preferences",
keywords="digital divide",
keywords="access",
keywords="accessible",
keywords="accessibility",
keywords="attitude",
keywords="perception",
keywords="attitudes",
keywords="opinion",
keywords="perceptions",
keywords="perspectives",
keywords="eHealth",
keywords="online health",
keywords="therapy",
doi="10.2196/55718",
url="https://rehab.jmir.org/2024/1/e55718"
}


@Article{info:doi/10.2196/38064,
author="Morsa, Maxime
and Perrin, Am{\'e}lie
and David, Val{\'e}rie
and Rault, Gilles
and Le Roux, Enora
and Alberti, Corinne
and Gagnayre, R{\'e}mi
and Pougheon Bertrand, Dominique",
title="Experiences Among Patients With Cystic Fibrosis in the MucoExocet Study of Using Connected Devices for the Management of Pulmonary Exacerbations: Grounded Theory Qualitative Research",
journal="JMIR Form Res",
year="2024",
month="Jan",
day="23",
volume="8",
pages="e38064",
keywords="cystic fibrosis",
keywords="mobile health",
keywords="mHealth",
keywords="patient education",
keywords="chronic disease",
keywords="empowerment",
keywords="devices",
keywords="patients",
keywords="detection",
keywords="treatment",
keywords="respiratory",
keywords="education",
keywords="monitoring",
keywords="care",
abstract="Background: Early detection of pulmonary exacerbations (PEx) in patients with cystic fibrosis is important to quickly trigger treatment and reduce respiratory damage. An intervention was designed in the frame of the MucoExocet research study providing patients with cystic fibrosis with connected devices and educating them to detect and react to their early signs of PEx. Objective: This study aims to identify the contributions and conditions of home monitoring in relation to their care teams from the users' point of view to detect PEx early and treat it. This study focused on the patients' experiences as the first and main users of home monitoring. Methods: A qualitative study was conducted to explore patients' and professionals' experiences with the intervention. We interviewed patients who completed the 2-year study using semistructured guides and conducted focus groups with the care teams. All the interviews were recorded and transcribed verbatim. Their educational material was collected. A grounded analysis was conducted by 2 researchers. Results: A total of 20 patients completed the study. Three main categories emerged from the patients' verbatim transcripts and were also found in those of the professionals: (1) task technology fit, reflecting reliability, ease of use, accuracy of data, and support of the technology; (2) patient empowerment through technology, grouping patients' learnings, validation of their perception of exacerbation, assessment of treatment efficacy, awareness of healthy behaviors, and ability to react to PEx signs in relation to their care team; (3) use, reflecting a continuous or intermittent use, the perceived usefulness balanced with cumbersome measurements, routinization and personalization of the measurement process, and the way data are shared with the care team. Furthermore, 3 relationships were highlighted between the categories that reflect the necessary conditions for patient empowerment through the use of technology. Conclusions: We discuss a theorization of the process of patient empowerment through the use of connected devices and call for further research to verify or amend it in the context of other technologies, illnesses, and care organizations. Trial Registration: ClinicalTrials.gov NCT03304028; https://clinicaltrials.gov/ct2/show/results/NCT03304028 ",
doi="10.2196/38064",
url="https://formative.jmir.org/2024/1/e38064",
url="http://www.ncbi.nlm.nih.gov/pubmed/38261372"
}


@Article{info:doi/10.2196/39450,
author="Perkins, C. Ryan
and Sawicki, S. Gregory",
title="Author Reply to: Empowering Without Misinforming Adolescents and Young Adults with Cystic Fibrosis. Comment on ``Perceptions of Social Media Use to Augment Health Care Among Adolescents and Young Adults With Cystic Fibrosis: Survey Study''",
journal="JMIR Pediatr Parent",
year="2022",
month="May",
day="25",
volume="5",
number="2",
pages="e39450",
keywords="Cystic fibrosis",
keywords="Social media",
keywords="mobile health",
keywords="adherence",
keywords="adolescents",
keywords="young adults",
keywords="Medical misinformation",
doi="10.2196/39450",
url="https://pediatrics.jmir.org/2022/2/e39450",
url="http://www.ncbi.nlm.nih.gov/pubmed/35612884"
}


@Article{info:doi/10.2196/33457,
author="Thumber, Navandeep
and Bhandari, Prerana",
title="Empowering Without Misinforming Adolescents and Young Adults with Cystic Fibrosis. Comment on ``Perceptions of Social Media Use to Augment Health Care Among Adolescents and Young Adults With Cystic Fibrosis: Survey Study''",
journal="JMIR Pediatr Parent",
year="2022",
month="May",
day="25",
volume="5",
number="2",
pages="e33457",
keywords="cystic fibrosis",
keywords="social media",
keywords="mobile health",
keywords="adherence",
keywords="adolescents",
keywords="young adults",
keywords="medical misinformation",
doi="10.2196/33457",
url="https://pediatrics.jmir.org/2022/2/e33457",
url="http://www.ncbi.nlm.nih.gov/pubmed/35612889"
}


@Article{info:doi/10.2196/14552,
author="Morsa, Maxime
and Perrin, Am{\'e}lie
and David, Val{\'e}rie
and Rault, Gilles
and Le Roux, Enora
and Alberti, Corinne
and Gagnayre, R{\'e}mi
and Pougheon Bertrand, Dominique",
title="Use of Home-Based Connected Devices in Patients With Cystic Fibrosis for the Early Detection and Treatment of Pulmonary Exacerbations: Protocol for a Qualitative Study",
journal="JMIR Res Protoc",
year="2021",
month="Aug",
day="18",
volume="10",
number="8",
pages="e14552",
keywords="cystic fibrosis",
keywords="pulmonary exacerbation",
keywords="connected devices",
keywords="patient education",
keywords="self-management",
abstract="Background: Early detection of pulmonary exacerbations (PEx) in patients with cystic fibrosis (CF) is important to quickly trigger treatment and reduce respiratory damage. We hypothesized that using home-based and wearable connected devices (CDs) and educating patients to react in case of abnormal variations in a set of parameters would allow patients to detect and manage their PEx early with their care team. Objective: This qualitative study aimed to assess the feasibility and appropriate conditions of a new PEx management process from the users' point of view by analyzing the experience of patients and of CF center teams regarding the education program, the use of CDs, and the relationship between the patient and the care team during PEx management. Methods: We have been conducting a multicenter pilot study involving 36 patients with CF aged ?12 years. The intervention was divided into 3 phases. In phase 1 (3 months), patients were equipped with CDs, and their parameters were collected on 3 nonconsecutive days each week. Phase 2 involved the development of a ``React to PEx'' educational program aimed at providing patients with a personalized action plan. A training session to the educational program was organized for the physicians. Physicians then determined the patients' personalized alert thresholds by reviewing the data collected during phase 1 and their patients' clinical history. In phase 3 (12 months), patients were educated by the physician during a clinic visit, and their action plan for reacting in timely fashion to their PEx signs was defined. Education and action plans were revised during clinic visits. At the end of the project, the patients' experience was collected during semistructured interviews with a researcher as part of the qualitative study. The experience of CF teams was collected during focus groups using a semistructured guide once all their patients had finished the study. The interviews and focus groups were recorded and transcribed verbatim to be analyzed. Data from educational sessions were collected throughout the educational program to be put into perspective with the learnings reported by patients. Analyses are being led by 2 researchers using NVivo (QSR International). Results: The study received the favorable reception of the Committee for the Protection of Persons (CPP NORTH WEST III) on June 10, 2017 (\#2017-A00723-50). Out of the 36 patients included in phase 1, 27 were educated and entered phase 3. We completed collection of all data from the patients and care providers. Qualitative analysis will provide a better understanding of users' experience on the conditions of data collection, how useful CDs are for detecting PEx, how useful the PEx action plan is for reacting quickly, what patients learned about PEx management, and the conditions for this PEx management to be sustainable in routine care. Conclusions: This study will open new perspectives for further research into the implementation of an optimal PEx care process in the organization of care teams in order to support patient self-management. Trial Registration: ClinicalTrials.gov NCT03304028; https://clinicaltrials.gov/ct2/show/results/NCT03304028 International Registered Report Identifier (IRRID): DERR1-10.2196/14552 ",
doi="10.2196/14552",
url="https://www.researchprotocols.org/2021/8/e14552",
url="http://www.ncbi.nlm.nih.gov/pubmed/34406124"
}


@Article{info:doi/10.2196/25014,
author="Perkins, C. Ryan
and Gross, Rachel
and Regan, Kayla
and Bishay, Lara
and Sawicki, S. Gregory",
title="Perceptions of Social Media Use to Augment Health Care Among Adolescents and Young Adults With Cystic Fibrosis: Survey Study",
journal="JMIR Pediatr Parent",
year="2021",
month="Aug",
day="16",
volume="4",
number="3",
pages="e25014",
keywords="cystic fibrosis",
keywords="social media",
keywords="mobile health",
keywords="adherence",
keywords="adolescents",
keywords="young adults",
abstract="Background: For individuals with cystic fibrosis (CF), adolescence and young adulthood are times of significant vulnerability and have been associated with clinical and psychosocial challenges. Social media may offer innovative care delivery solutions to address these challenges. Objective: This study explored motivations and attitudes regarding current social media use and preferences for a social media platform in a sample of adolescents and young adults (AYA) with CF. Methods: A cross-sectional survey was administered to 50 AYA with CF followed at a large pediatric-adult CF center. The survey included questions regarding social media platform utilization, attitudes toward general and CF-specific online activities, and preferences for a CF-specific care delivery platform. Results: YouTube, Snapchat, and Instagram were the most commonly used social media platforms. AYA with CF do not report routinely using social media for health-related information acquisition, social support, or help with adherence. However, their perceptions of social media utilization and preferences for platform development suggest interest in doing so in the future. Conclusions: AYA with CF use social media and expressed interest in the development of a social media platform. Platform development will allow for gaps in health care delivery to be addressed by improving social support and adherence while augmenting current methods of health information acquisition. ",
doi="10.2196/25014",
url="https://pediatrics.jmir.org/2021/3/e25014",
url="http://www.ncbi.nlm.nih.gov/pubmed/34232121"
}


@Article{info:doi/10.2196/21270,
author="Dauber-Decker, L. Katherine
and Basile, Melissa
and King, D'Arcy
and Polo, Jennifer
and Calise, Karina
and Khan, Sundas
and Solomon, Jeffrey
and Dunne, Daniel
and Hajizadeh, Negin",
title="Developing a Decision Aid to Facilitate Informed Decision Making About Invasive Mechanical Ventilation and Lung Transplantation Among Adults With Cystic Fibrosis: Usability Testing",
journal="JMIR Hum Factors",
year="2021",
month="Apr",
day="14",
volume="8",
number="2",
pages="e21270",
keywords="usability",
keywords="medical informatics",
keywords="clinical decision support",
keywords="cystic fibrosis",
keywords="advance care planning",
abstract="Background: Cystic fibrosis (CF) is a life-limiting genetic disease that causes chronic lung infections. We developed an internet-based decision aid (DA) to help patients with CF make better informed decisions regarding treatments and advance care planning. We built the DA around two major treatment decisions: whether to have a lung transplant and whether to agree to invasive mechanical ventilation (intubation). Objective: This study aims to conduct usability testing of the InformedChoices CF DA among key stakeholder groups. Methods: We performed a patient needs assessment using think-aloud usability testing with patients with CF, their surrogates, and CF clinicians. Think-aloud participants provided feedback while navigating the DA, and after viewing, they answered surveys. Transcripts from the think-aloud sessions and survey results were categorized into common, generalizable themes and optimizations for improving content, comprehension, and navigation. We assessed the ease of use of the DA (System Usability Scale) and also assessed the participants' perceptions regarding the overall tone, with an emphasis on emotional reactions to the DA content, level of detail, and usefulness of the information for making decisions about either intubation or lung transplantation, including how well they understood the information and were able to apply it to their own decision-making process. We also assessed the DA's ease of navigation, esthetics, and whether participants were able to complete a series of usability tasks (eg, locating specific information in the DA or using the interactive survival estimates calculator) to ensure that the website was easy to navigate during the clinic-based advance care planning discussions. Results: A total of 12 participants from 3 sites were enrolled from March 9 to August 30, 2018, for the usability testing: 5 CF clinicians (mean age 48.2, SD 12.0 years), 5 adults with CF, and 2 family and surrogate caregivers of people with CF (mean age of CF adults and family and surrogate caregivers 38.8, SD 10.8 years). Among the 12 participants, the average System Usability Scale score for the DA was 88.33 (excellent). Think-aloud analysis identified 3 themes: functionality, visibility and navigation, and content and usefulness. Areas for improvement included reducing repetition, enhancing comprehension, and changing the flow. Several changes to improve the content and usefulness of the DA were recommended, including adding information about alternatives to childbearing, such as adoption and surrogacy. On the basis of survey responses, we found that the navigation of the site was easy for clinicians, patients, and surrogates who participated in usability testing. Conclusions: Usability testing revealed areas of potential improvement. Testing also yielded positive feedback, suggesting the DA's future success. Integrating changes before implementation should improve the DA's comprehension, navigation, and usefulness and lead to greater adoption. ",
doi="10.2196/21270",
url="https://humanfactors.jmir.org/2021/2/e21270",
url="http://www.ncbi.nlm.nih.gov/pubmed/33851921"
}


@Article{info:doi/10.2196/19413,
author="Rutland, B. Sarah
and Bergquist, Palmer Rikard
and Hager, Andreas
and Geurs, Robin
and Mims, Cathy
and Gutierrez, H. Hector
and Oates, R. Gabriela",
title="A Mobile Health Platform for Self-Management of Pediatric Cystic Fibrosis: Qualitative Study of Adaptation to Stakeholder Needs and Integration in Clinical Settings",
journal="JMIR Form Res",
year="2021",
month="Jan",
day="26",
volume="5",
number="1",
pages="e19413",
keywords="cystic fibrosis",
keywords="mHealth",
abstract="Background: Cystic fibrosis (CF) is an inherited chronic condition that requires extensive daily care and quarterly clinic visits with a multidisciplinary care team. The limited exchange of information outside of the quarterly clinic visits impedes optimal disease self-management, patient engagement, and shared decision making. Objective: The aim of this study is to adapt a mobile health (mHealth) app originally developed in Sweden to the needs of patients, families, and health care providers in a CF center in the United States and to test it as a platform for sharing patient-generated health data with the CF health care team. Methods: Focus groups with health care providers of patients with CF, adolescents with CF, and caregivers of children with CF were conducted to determine what modifications were necessary. Focus group data were analyzed using a thematic analysis, and emergent themes were ranked according to desirability and technical feasibility. The mHealth platform was then modified to meet the identified needs and preferences, and the flow of patient-generated health data to a secure Research Electronic Data Capture database was tested. Protocols for data management and clinical follow-up were also developed. Results: A total of 5 focus groups with 21 participants were conducted. Recommended modifications pertained to all functionalities of the mHealth platform, including tracking of symptoms, treatments, and activities of daily care; creating and organizing medication lists and setting up reminders; generating reports for the health care team; language and presentation; sharing and privacy; and settings and accounts. Overall, health care providers recommended changes to align the mHealth platform with US standards of care, people with CF and their caregivers requested more tracking functionalities, and both groups suggested the inclusion of a mental health tracker as well as more detailed response options and precise language. Beta testers of the modified platform reported issues related to translatability to US environment and various bugs. Conclusions: This study demonstrated the importance of identifying the needs and preferences of target users and stakeholders before adopting existing mHealth solutions. All relevant perspectives, including those of clinicians, patients, and caregivers, should be thoroughly considered to meet both end users' needs and evidence-based practice recommendations. ",
doi="10.2196/19413",
url="http://formative.jmir.org/2021/1/e19413/",
url="http://www.ncbi.nlm.nih.gov/pubmed/33496667"
}


@Article{info:doi/10.2196/medinform.9979,
author="Jing, Xia
and Hardiker, R. Nicholas
and Kay, Stephen
and Gao, Yongsheng",
title="Identifying Principles for the Construction of an Ontology-Based Knowledge Base: A Case Study Approach",
journal="JMIR Med Inform",
year="2018",
month="Dec",
day="21",
volume="6",
number="4",
pages="e52",
keywords="cystic fibrosis",
keywords="knowledge base",
keywords="knowledge representation",
keywords="molecular genetics information",
keywords="ontology",
keywords="OntoKBCF",
keywords="phenotypes",
abstract="Background: Ontologies are key enabling technologies for the Semantic Web. The Web Ontology Language (OWL) is a semantic markup language for publishing and sharing ontologies. Objective: The supply of customizable, computable, and formally represented molecular genetics information and health information, via electronic health record (EHR) interfaces, can play a critical role in achieving precision medicine. In this study, we used cystic fibrosis as an example to build an Ontology-based Knowledge Base prototype on Cystic Fibrobis (OntoKBCF) to supply such information via an EHR prototype. In addition, we elaborate on the construction and representation principles, approaches, applications, and representation challenges that we faced in the construction of OntoKBCF. The principles and approaches can be referenced and applied in constructing other ontology-based domain knowledge bases. Methods: First, we defined the scope of OntoKBCF according to possible clinical information needs about cystic fibrosis on both a molecular level and a clinical phenotype level. We then selected the knowledge sources to be represented in OntoKBCF. We utilized top-to-bottom content analysis and bottom-up construction to build OntoKBCF. Prot{\'e}g{\'e}-OWL was used to construct OntoKBCF. The construction principles included (1) to use existing basic terms as much as possible; (2) to use intersection and combination in representations; (3) to represent as many different types of facts as possible; and (4) to provide 2-5 examples for each type. HermiT 1.3.8.413 within Prot{\'e}g{\'e}-5.1.0 was used to check the consistency of OntoKBCF. Results: OntoKBCF was constructed successfully, with the inclusion of 408 classes, 35 properties, and 113 equivalent classes. OntoKBCF includes both atomic concepts (such as amino acid) and complex concepts (such as ``adolescent female cystic fibrosis patient'') and their descriptions. We demonstrated that OntoKBCF could make customizable molecular and health information available automatically and usable via an EHR prototype. The main challenges include the provision of a more comprehensive account of different patient groups as well as the representation of uncertain knowledge, ambiguous concepts, and negative statements and more complicated and detailed molecular mechanisms or pathway information about cystic fibrosis. Conclusions: Although cystic fibrosis is just one example, based on the current structure of OntoKBCF, it should be relatively straightforward to extend the prototype to cover different topics. Moreover, the principles underpinning its development could be reused for building alternative human monogenetic diseases knowledge bases. ",
doi="10.2196/medinform.9979",
url="http://medinform.jmir.org/2018/4/e52/",
url="http://www.ncbi.nlm.nih.gov/pubmed/30578220"
}


@Article{info:doi/10.2196/11080,
author="Longacre, Meghan
and Grande, Stuart
and Hager, Andreas
and Montan, Meera
and Bergquist, Palmer Rikard
and Martensson, Maria
and Kotzbauer, Greg",
title="Clinical Adoption of mHealth Technology to Support Pediatric Cystic Fibrosis Care in Sweden: Qualitative Case Study",
journal="JMIR Pediatr Parent",
year="2018",
month="Dec",
day="10",
volume="1",
number="2",
pages="e11080",
keywords="cystic fibrosis",
keywords="mHealth",
keywords="mobile phone",
keywords="pediatrics",
keywords="qualitative case study",
keywords="technology",
abstract="Background: Mobile health (mHealth) technologies have potential to improve self-management and care co-ordination of pediatric chronic diseases requiring complex care, such as cystic fibrosis (CF). Barriers to implementation include the lack of support and infrastructure to use mHealth in the clinical microsystem. Coproducing mHealth technology with patients, clinicians, and designers may increase the likelihood of successful integration into the clinical setting. Objective: This study explored the development, adoption, and integration of a new, co-produced mHealth platform (Genia) for the management of pediatric CF in Sweden. Methods: A retrospective, qualitative case study approach was used. The case was defined as the process of introducing and using Genia at the Pediatric Cystic Fibrosis Center at Sk{\aa}ne University Hospital in Lund, Sweden. Data sources included interviews, presentations, meeting notes, and other archival documents created between 2014 and 2017. To be included, data sources must have described or reflected upon the Genia adoption process. Iterative content analysis of data source materials was conducted by 2 qualitatively trained researchers to derive themes characterizing the mHealth clinical adoption process. Results: In total, 4 core themes characterized successful clinical integration of Genia in Lund: cultural readiness to use mHealth; use of weekly huddles to foster momentum and rapid iteration; engagement in incremental ``Genia Talk'' to motivate patient adoption; and co-design approach toward pediatric chronic care. Conclusions: Principles of quality improvement, relational co-ordination, user-centered design, and coproduction can facilitate the integration of mHealth technology into clinical care systems for pediatric CF care. ",
doi="10.2196/11080",
url="http://pediatrics.jmir.org/2018/2/e11080/",
url="http://www.ncbi.nlm.nih.gov/pubmed/31518297"
}


@Article{info:doi/10.2196/11189,
author="McCreery, L. Jessica
and Mackintosh, A. Kelly
and Cox, S. Narelle
and McNarry, A. Melitta",
title="Assessing the Perceptions of Inspiratory Muscle Training in Children With Cystic Fibrosis and Their Multidisciplinary Team: Mixed-Methods Study",
journal="JMIR Pediatr Parent",
year="2018",
month="Oct",
day="25",
volume="1",
number="2",
pages="e11189",
keywords="cystic fibrosis",
keywords="health perceptions",
keywords="inspiratory muscle training",
keywords="mobile phone",
keywords="pediatrics",
keywords="qualitative",
abstract="Background: Little is known about the opinions or perceived benefits of an inspiratory muscle training intervention in patients with cystic fibrosis and their multidisciplinary team. Objective: The aim of this qualitative study was to examine patients' and multidisciplinary teams' views on inspiratory muscle training to inform and tailor future interventions. Methods: Individual, semistructured interviews were conducted to evaluate participants' perspectives of a 4-week inspiratory muscle training intervention. In this study, 8 of 13 individuals involved in the inspiratory muscle training program (5 children aged 11-14 years; 2 physiotherapists; and 1 respiratory physician) participated. Interviews were transcribed verbatim, analyzed using thematic analyses, and then coded into relevant themes. Results: Four key themes emerged: acceptability, facilitators, barriers, and recommendations. While fun, enjoyment, and improved perceived physical ability were reported by children and their multidisciplinary team following the inspiratory muscle training program, the multidisciplinary team identified factors such as time and cost as key barriers. Conclusions: A short inspiratory muscle training program was perceived to have positive effects on the physical ability and psychosocial health of children with cystic fibrosis. These findings highlight the importance of obtaining participants' and multidisciplinary teams' perceptions and recommendations to ensure the efficacy and optimal design of future inspiratory muscle training protocols. ",
doi="10.2196/11189",
url="http://pediatrics.jmir.org/2018/2/e11189/",
url="http://www.ncbi.nlm.nih.gov/pubmed/31518290"
}