Journal of Cystic Fibrosis最新文献

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Strategies used to access CFTR modulators in countries without reimbursement agreements
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-03-01 DOI: 10.1016/j.jcf.2025.02.010
Jonathan Guo , Grace Hennessy , Benedict Young , Andrew Hill
{"title":"Strategies used to access CFTR modulators in countries without reimbursement agreements","authors":"Jonathan Guo ,&nbsp;Grace Hennessy ,&nbsp;Benedict Young ,&nbsp;Andrew Hill","doi":"10.1016/j.jcf.2025.02.010","DOIUrl":"10.1016/j.jcf.2025.02.010","url":null,"abstract":"<div><div>CFTR modulators represent the international standard of care for the treatment of cystic fibrosis (CF). Yet due to prices of over $250,000 per year they are functionally inaccessible for people with CF (pwCF) unless reimbursed by healthcare systems. Current prices are unaffordable for payors in almost all low- and middle-income countries (LMICs) worldwide, and resulting disparities in access are widening existing global health inequities. In comparable situations in other therapeutic areas, patients have successfully developed strategies to bypass national reimbursement systems and gain access to treatment. We therefore undertook an international survey of CF clinicians in 15 countries where CFTR modulators are not reimbursed, to characterise alternative means of accessing modulator therapy.</div><div>Successful methods were identified in 11 countries, and could broadly be categorised into legal challenges to access originator modulators, use of generic formulations, and access via donations. Aside from domestically produced generics used in Argentina and an originator-led donation program in Ukraine, these methods were only able to provide treatment to limited proportions of the local CF population due to significant associated financial costs. Accordingly, they are generally not sustainable or widely applicable, and fail to address the underlying structural issues driving international disparities in access.</div><div>Twelve years after the initial marketing of CFTR modulators, pwCF in LMICs are being forced to take extraordinary measures to access disease-modifying treatment. Corrective measures are urgently required to overcome barriers posed by restrictive patents and prohibitively high prices, and to promote global health equity for pwCF.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 2","pages":"Pages 290-294"},"PeriodicalIF":5.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143476590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Monitoring cystic fibrosis airway infections with Pseudomonas aeruginosa with anti-OprF serum antibodies 用抗 OprF 血清抗体监测铜绿假单胞菌引起的囊性纤维化气道感染。
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-03-01 DOI: 10.1016/j.jcf.2024.06.001
Burkhard Tümmler , Christiane Bürger , Peter Kubesch
{"title":"Monitoring cystic fibrosis airway infections with Pseudomonas aeruginosa with anti-OprF serum antibodies","authors":"Burkhard Tümmler ,&nbsp;Christiane Bürger ,&nbsp;Peter Kubesch","doi":"10.1016/j.jcf.2024.06.001","DOIUrl":"10.1016/j.jcf.2024.06.001","url":null,"abstract":"<div><h3>Background</h3><div>The management of cystic fibrosis (CF) requires knowledge of the patient's microbiological status. The serology of anti-<em>Pseudomonas aeruginosa</em> antibodies against exoenzymes or water-soluble antigens has gained diagnostic value, particularly to detect the onset of colonization with <em>P. aeruginosa.</em> However, the diversity and variable expression of these antigens, which was unknown when the ELISAs became common diagnostic procedures at CF clinics, prohibits the quantitative evaluation of bacterial antigen load during intermittent and chronic infection.</div></div><div><h3>Methods</h3><div>An ELISA was developed to measure the serum IgG antibody levels against <em>P. aeruginosa</em> porin OprF, a species-specific, conserved, immunogenic and constitutively expressed protein present in the outer membrane and extracellular vesicles.</div></div><div><h3>Results</h3><div>Serial serum samples were collected from 310 people with CF (pwCF) over a period of up to 15 years. Compared to a reference of <em>P. aeruginosa</em> – negative CF sera set to 1, OprF antibody titers ranged from 0.3 to 13.2 (median: 1.7) in 56 intermittently colonized patients and from 0.5 to 51.2 (median: 11.8) in 176 chronically colonized pwCF showing higher anti-OprF antibody levels during chronic than during intermittent colonization with <em>P. aeruginosa</em> (<em>P</em> = 0, <em>Z</em> = - 21.7, effect size 0.62). Inhalation with twice daily 80 mg tobramycin decreased OprF antibody titers (<em>P</em> = 5 × 10<sup>−5</sup>), particularly during the third and fourth year of chronic colonization.</div></div><div><h3>Conclusion</h3><div>The OprF ELISA should be an appropriate tool to monitor Pseudomonas serology at all stages of infection and disease severity and to study the impact of short- and long-term therapeutic interventions.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 2","pages":"Pages 353-358"},"PeriodicalIF":5.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141283827","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Pain in adults with cystic fibrosis – Are we painfully unaware?
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-03-01 DOI: 10.1016/j.jcf.2025.01.009
Anastasia Ward , Ramil Mauleon , Gretel Davidson , Chee Y. Ooi , Nedeljka Rosić
{"title":"Pain in adults with cystic fibrosis – Are we painfully unaware?","authors":"Anastasia Ward ,&nbsp;Ramil Mauleon ,&nbsp;Gretel Davidson ,&nbsp;Chee Y. Ooi ,&nbsp;Nedeljka Rosić","doi":"10.1016/j.jcf.2025.01.009","DOIUrl":"10.1016/j.jcf.2025.01.009","url":null,"abstract":"<div><h3>Background</h3><div>A previous Australia-wide pilot study identified pain as a significant burden in people with CF (pwCF). However, the prevalence, frequency and severity have not been evaluated using validated tools.</div></div><div><h3>Methods</h3><div>Australian adults, pwCF and healthy controls (HC) were invited to complete an online questionnaire from July 2023 – February 2024, consisting of four validated tools: Brief Pain Inventory, Pain Catastrophising Scale, PAGI-SYM and PAC-SYM. The questionnaire, disseminated via Cystic Fibrosis Australia, CF Together and online social media groups, explored experiences surrounding pain and its management using closed and free text entries.</div></div><div><h3>Results</h3><div>There were 206 respondents, consisting of 117 CF patients and 89 HC. Over 70 % (<em>n</em> = 69) of pwCF reported pain compared to 28 % (<em>n</em> = 21) of HC (<em>p</em> = &lt;0.001). Further, significantly higher pain frequency per month was reported for pwCF than HC (40 % vs. 10 %; <em>p</em> &lt; 0.001). Symptom clustering was also observed where at least three other locations of pain were reported, and pain was reported to trigger other physiological and psychological symptoms. Notably, there was no significant difference in the locations, occurrence, frequency or severity of pain between those on a CFTR modulator or not (<em>p</em> = 0.625). PwCF also reported significantly lower relief from over-the-counter therapies (<em>p</em> = 0.002) and expressed themes of unmet symptom and management needs.</div></div><div><h3>Conclusions</h3><div>This study identified a high prevalence of pain affecting multiple body parts in pwCF compared to HC and suggests that pain is sub-optimally managed, impairing their quality of life. Increased awareness and early recognition within the CF clinics and the development of clinical pathways are critically needed to better manage and monitor pain in pwCF, leading to improved quality of life and health outcomes.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 2","pages":"Pages 236-245"},"PeriodicalIF":5.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143046795","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Association of race and ethnicity with the development of cystic fibrosis-related diabetes 种族和民族与囊性纤维化相关糖尿病发病的关系。
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-03-01 DOI: 10.1016/j.jcf.2024.07.018
Maria S. Rayas , Blessed Mbogo , Andrea Kelly , Phuong Vu , Amalia Magaret , Tanicia Daley
{"title":"Association of race and ethnicity with the development of cystic fibrosis-related diabetes","authors":"Maria S. Rayas ,&nbsp;Blessed Mbogo ,&nbsp;Andrea Kelly ,&nbsp;Phuong Vu ,&nbsp;Amalia Magaret ,&nbsp;Tanicia Daley","doi":"10.1016/j.jcf.2024.07.018","DOIUrl":"10.1016/j.jcf.2024.07.018","url":null,"abstract":"<div><h3>Background</h3><div>CF-related diabetes (CFRD) is a common, life-expectancy limiting complication of CF. While Black race and Hispanic ethnicity in youth-onset type 1 and type 2 diabetes are well-recognized risk factors for worse diabetes complications, the potential for racial/ethnic disparities in CFRD has received limited attention.</div></div><div><h3>Methods</h3><div>We conducted a retrospective cohort study utilizing the CF Foundation Patient Registry from 2010 to 2019 to determine the prevalence and incidence of CFRD by race/ethnicity. Three age cohorts were identified at baseline in 2010 (11–20y, 21–30y, and 31–40y). Logistic regression and Cox regression stratified by age group were used to determine the prevalence and incidence, respectively, among Hispanic, non-Hispanic Blacks (NHB), and non-Hispanic whites (NHW) after adjustment for relevant confounders, including demographics, socioeconomic status, clinical factors, and chronic medication use.</div></div><div><h3>Results</h3><div>Among 14,660 registry participants, 510 were NHB and 890 Hispanic. NHB associated with higher odds of CFRD baseline prevalence in all age cohorts (11–20y: OR 2.53 (95 % CI: 1.88–3.41, <em>P</em> &lt; 0.05), 21–30y: OR 1.80 (1.25–2.59, <em>P</em> &lt; 0.05), and 31–40y: OR 1.93 (1.00–3.73, <em>P</em> &lt; 0.05)) relative to NHW. In the 11–20y cohort, the hazard of new-onset CFRD was 40 % higher in NHB (HR 1.40 (1.09–1.8, <em>P</em> &lt; 0.05)) and 19 % higher in Hispanics (HR 1.19 (1.01–1.41, <em>P</em> &lt; 0.05)).</div></div><div><h3>Conclusion</h3><div>NHB had a higher prevalence of CFRD across all age groups, with NHB and Hispanics showing higher incidence of CFRD in the youngest group. Multicenter studies performed in diverse CF populations are warranted to identify modifiable factors influencing earlier CFRD development in minoritized groups and their potential contribution to diabetes complication disparities.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 2","pages":"Pages 263-270"},"PeriodicalIF":5.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141889388","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
GRASPing for answers: The shortfalls of our current understanding of the effects of GI-related aspiration on the lungs in CF (GRASP-CF) GRASPing for answers:我们目前对消化道相关吸入对 CF 肺部影响的认识存在不足(GRASP-CF)。
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-03-01 DOI: 10.1016/j.jcf.2024.09.014
Christopher Vélez , Isabel Neuringer , Jeffrey King
{"title":"GRASPing for answers: The shortfalls of our current understanding of the effects of GI-related aspiration on the lungs in CF (GRASP-CF)","authors":"Christopher Vélez ,&nbsp;Isabel Neuringer ,&nbsp;Jeffrey King","doi":"10.1016/j.jcf.2024.09.014","DOIUrl":"10.1016/j.jcf.2024.09.014","url":null,"abstract":"","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 2","pages":"Pages 398-400"},"PeriodicalIF":5.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142347592","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Disparities in outcomes by race and ethnicity in the Canadian cystic fibrosis population 加拿大囊性纤维化人群中不同种族和族裔的治疗效果差异。
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-03-01 DOI: 10.1016/j.jcf.2024.09.009
Rachel Holland , Hanna Stewart , Stephanie Y. Cheng , Miriam Schroeder , Sanja Stanojevic
{"title":"Disparities in outcomes by race and ethnicity in the Canadian cystic fibrosis population","authors":"Rachel Holland ,&nbsp;Hanna Stewart ,&nbsp;Stephanie Y. Cheng ,&nbsp;Miriam Schroeder ,&nbsp;Sanja Stanojevic","doi":"10.1016/j.jcf.2024.09.009","DOIUrl":"10.1016/j.jcf.2024.09.009","url":null,"abstract":"<div><h3>Background</h3><div>Cystic Fibrosis has historically been described as a disease that affects people of European ancestry. Consequently, much of what we know about CF is based on evidence generated from data collected in white individuals. This may lead to systematic bias in how non-white people with CF are diagnosed and treated. In this study we compared clinical outcomes between the white and non-white people with CF in Canada.</div></div><div><h3>Methods</h3><div>Canadian CF Registry data collected between 2000 and 2019 were used in this population-based cohort study. Demographic characteristics and clinical outcomes of people with CF identified as white and those identified as non-white were compared. Analyses were adjusted for cohort effects but not socioeconomic status.</div></div><div><h3>Results</h3><div>Between 2000 and 2019, 5516 people with CF in the Registry were identified as white and 323 were identified as non-white. At diagnosis, the white and non-white groups were similar with respect to sex at birth, age at diagnosis, prevalence of pancreatic insufficiency, and meconium ileus. The non-white group had similar rates of CF-related complications and bacterial infections compared to the white, but worse lung function, worse nutritional status, lower treatment rates, and higher rate of hospitalizations. During the 20-year study period, the non-white group had a 1.85 higher risk of death compared to the white group (HR 95 %CI 1.39; 2.47).</div></div><div><h3>Interpretation</h3><div>There is an urgent need understand why outcomes for Canadians with CF differ between white and non-white individuals, including the role of socioeconomic circumstances.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 2","pages":"Pages 271-277"},"PeriodicalIF":5.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142288299","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Infection prevention and control in cystic fibrosis: An update of a systematic review of interventions 囊性纤维化的感染预防和控制:干预措施系统回顾的更新。
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-03-01 DOI: 10.1016/j.jcf.2024.08.004
Nicola J Rowbotham , Sherie Smith , Nikki Jahnke , Sarah Milczanowski , Zoe C Elliott , Andrew P Prayle , Alan R Smyth
{"title":"Infection prevention and control in cystic fibrosis: An update of a systematic review of interventions","authors":"Nicola J Rowbotham ,&nbsp;Sherie Smith ,&nbsp;Nikki Jahnke ,&nbsp;Sarah Milczanowski ,&nbsp;Zoe C Elliott ,&nbsp;Andrew P Prayle ,&nbsp;Alan R Smyth","doi":"10.1016/j.jcf.2024.08.004","DOIUrl":"10.1016/j.jcf.2024.08.004","url":null,"abstract":"<div><div>Preventing transmissible infection is a priority in cystic fibrosis (CF) care. This is an update of a systematic review of the evidence for infection prevention and control interventions in CF.</div><div>Our full protocol can be found on PROSPERO (CRD42018109999). We searched for studies and guidelines which included interventions for infection prevention and control in CF.</div><div>We included 39 studies and 7 guidelines. Strategies included: cohort or individual segregation, hand hygiene, facemasks, equipment, enhanced adherence or a combination of these. Many studies showed a reduction in transmission with segregation. However, the certainty of evidence (using GRADE) was low or very low. Most guideline recommendations have little evidence to support them, with no updates since our original review.</div><div>Undertaking RCTs in this area is ethically difficult. Large-scale registry-based studies may be the best pragmatic approach. Benefits of infection control must be balanced against the intrusion in the lives of people with CF.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 2","pages":"Pages 359-363"},"PeriodicalIF":5.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142093218","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Pf bacteriophage is associated with decline in lung function in a longitudinal cohort of patients with cystic fibrosis and Pseudomonas airway infection 在囊性纤维化和气道假单胞菌感染患者的纵向队列中,噬菌体Pf与肺功能下降有关。
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-03-01 DOI: 10.1016/j.jcf.2024.09.018
Elizabeth B. Burgener , Aditi Gupta , Kayo Nakano , Sophia L. Gibbs , Maya E. Sommers , Arya Khosravi , Michelle S. Bach , Colleen Dunn , Jacquelyn Spano , Patrick R. Secor , Lu Tian , Paul L. Bollyky , Carlos E. Milla
{"title":"Pf bacteriophage is associated with decline in lung function in a longitudinal cohort of patients with cystic fibrosis and Pseudomonas airway infection","authors":"Elizabeth B. Burgener ,&nbsp;Aditi Gupta ,&nbsp;Kayo Nakano ,&nbsp;Sophia L. Gibbs ,&nbsp;Maya E. Sommers ,&nbsp;Arya Khosravi ,&nbsp;Michelle S. Bach ,&nbsp;Colleen Dunn ,&nbsp;Jacquelyn Spano ,&nbsp;Patrick R. Secor ,&nbsp;Lu Tian ,&nbsp;Paul L. Bollyky ,&nbsp;Carlos E. Milla","doi":"10.1016/j.jcf.2024.09.018","DOIUrl":"10.1016/j.jcf.2024.09.018","url":null,"abstract":"<div><h3>Background</h3><div>The Pseudomonas filamentous bacteriophage (Pf) infects <em>Pseudomonas aeruginosa</em> (<em>Pa</em>) and is abundant in the airways of many people with cystic fibrosis (CF) (pwCF). We previously demonstrated that Pf promotes biofilm growth, as well as generates liquid crystals that confer biofilms with adhesivity, viscosity and resistance to clearance. Consistent with these findings, the presence of Pf in sputum from pwCF has been linked to chronic <em>Pa</em> infection and more severe exacerbations in a cross-sectional cohort study.</div></div><div><h3>Methods</h3><div>We examined the relationships between Pf and clinical outcomes in a longitudinal study of pwCF. Sputum <em>Pa</em> and Pf concentrations were measured by qPCR, as well cytokines and active neutrophil elastase by standardized assays. Recorded clinical data, including spirometry and microbiological results, were analyzed for associations with Pf. Finally, lung explants from pwCF in this cohort who underwent lung transplantation were examined for presence of liquid crystals within secretions.</div></div><div><h3>Results</h3><div>In explanted lungs from pwCF with known Pf infection we demonstrate areas of birefringence consistent with liquid crystalline structures within the airways. We find that high concentration of Pf in sputum is associated with accelerated loss of lung function, suggesting a potential role for Pf in the pathogenesis of CF lung disease. We also find Pf to associate with increased airway inflammation and an anti-viral cytokine response.</div></div><div><h3>Conclusion</h3><div>Pf may serve as a prognostic biomarker and potential therapeutic target for <em>Pa</em> infections in CF.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 2","pages":"Pages 345-352"},"PeriodicalIF":5.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142566485","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Active parents, active youth? Exploring the association between physical activity of youth with Cystic Fibrosis and their parents 积极的父母,积极的青年?探讨青年囊性纤维化患者体育活动与其父母之间的关系。
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-03-01 DOI: 10.1016/j.jcf.2024.11.007
Manon Kinaupenne , Stephanie Van Biervliet , Kim Van Hoorenbeeck , Heidi Schaballie , Kristof Vandekerckhove , Marieke De Craemer , Heleen Demeyer
{"title":"Active parents, active youth? Exploring the association between physical activity of youth with Cystic Fibrosis and their parents","authors":"Manon Kinaupenne ,&nbsp;Stephanie Van Biervliet ,&nbsp;Kim Van Hoorenbeeck ,&nbsp;Heidi Schaballie ,&nbsp;Kristof Vandekerckhove ,&nbsp;Marieke De Craemer ,&nbsp;Heleen Demeyer","doi":"10.1016/j.jcf.2024.11.007","DOIUrl":"10.1016/j.jcf.2024.11.007","url":null,"abstract":"<div><h3>Background</h3><div>Parents play a major role in shaping their children's physical activity (PA) behaviour. This study aimed to investigate the association between PA of youth with Cystic Fibrosis (YwCF) and their parents.</div></div><div><h3>Methods</h3><div>PA was measured by an ActiGraph GT3x-BT for seven consecutive days. Data were processed by GGIR and PA intensities were based on the age-specific Hildebrand equations. Moderate-to-vigorous PA was chosen as primary outcome.</div></div><div><h3>Results</h3><div>26 YwCF-parent dyads participated. A significant positive association was found between parental PA behaviour and YwCF's PA behaviour for both moderate-to-vigorous PA and total PA (R<sup>2</sup> = 0.60; <em>p</em> = 0.001; R<sup>2</sup> = 0.64; <em>p</em> &lt; 0.001). Furthermore, YwCF with less active parents perform 16 min/day less moderate-to-vigorous PA compared to YwCF with more active parents (<em>p</em> = 0.004).</div></div><div><h3>Conclusion</h3><div>Higher parental PA levels are strongly associated with higher YwCF's PA levels. This association needs to be confirmed in a larger cohort to explore whether parental behaviour is an effective strategy to improve YwCF's PA levels.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 2","pages":"Pages 418-420"},"PeriodicalIF":5.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142754934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Impact of UPLIFT, a group telehealth intervention, on symptoms of depression and anxiety in adults with CF 集体远程医疗干预——UPLIFT对CF成人抑郁和焦虑症状的影响
IF 5.4 2区 医学
Journal of Cystic Fibrosis Pub Date : 2025-03-01 DOI: 10.1016/j.jcf.2024.11.008
Michael S. Schechter , Andrea Molzhon , Robin S. Everhart , Le Kang , Rachel Weiskittle , Brittany Castleberry , Nancy J. Thompson
{"title":"Impact of UPLIFT, a group telehealth intervention, on symptoms of depression and anxiety in adults with CF","authors":"Michael S. Schechter ,&nbsp;Andrea Molzhon ,&nbsp;Robin S. Everhart ,&nbsp;Le Kang ,&nbsp;Rachel Weiskittle ,&nbsp;Brittany Castleberry ,&nbsp;Nancy J. Thompson","doi":"10.1016/j.jcf.2024.11.008","DOIUrl":"10.1016/j.jcf.2024.11.008","url":null,"abstract":"<div><h3>Background</h3><div>Despite high rates of anxiety and depression, research regarding the effect of psychological interventions on people with CF (pwCF) is limited. We evaluated the impact of UPLIFT (Using Practice and Learning to Increase Favorable Thoughts), a group telehealth intervention using mindfulness-based cognitive behavioral therapy (MBCT), on symptoms of anxiety and depression in pwCF.</div></div><div><h3>Methods</h3><div>This multicenter randomized trial compared changes in symptoms of anxiety and/or depression in adult pwCF who participated in the 8-week UPLIFT intervention to a treatment-as-usual (TAU) group. Follow up assessments occurred immediately after and 6- and 12-months post-intervention. Primary outcome measures were change in Patient Health Questionnaire (PHQ-9) and Generalized Anxiety Disorder (GAD-7) scores modeled in separate linear mixed-effects models.</div></div><div><h3>Results</h3><div>Sixty-six pwCF participated. At baseline, 43 (65.15%) had some minimal symptoms of depression (PHQ-9≥5) and 44 (66.67%) had some minimal symptoms of anxiety (GAD-7≥5). During the 12 month follow up period, the overall change in PHQ-9 was greater in the UPLIFT group compared to TAU (<em>p</em> = .049). Analysis of individual time points showed a statistically significant difference between groups in change from baseline immediately post-treatment (-2.321, <em>SD</em> 0.684 vs 0.362, <em>SD</em> 0.656, <em>p</em> = .005); differences persisted but were not statistically significant at 6 and 12 months. Similar trends for changes in GAD-7 were non-significant.</div></div><div><h3>Conclusions</h3><div>Participation in UPLIFT, a group telehealth intervention using MBCT, provides short-term improvement in symptoms of depression, as measured by changes in PHQ9. Improvement in symptoms of anxiety were suggested but could not be statistically confirmed.</div></div>","PeriodicalId":15452,"journal":{"name":"Journal of Cystic Fibrosis","volume":"24 2","pages":"Pages 319-325"},"PeriodicalIF":5.4,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142818290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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