BURA Collection:

Prioritising cardiopulmonary exercise testing for adults with cystic fibrosis: a service evaluation

2026-03-06T03:00:59Z

Title: Prioritising cardiopulmonary exercise testing for adults with cystic fibrosis: a service evaluation Authors: McDowell, R; Ogbonnaya, C; Shannon, H; Douglas, H Abstract: Background: Cystic Fibrosis is an inherited, life-limiting condition causing a range of symptoms including lowered exercise tolerance. Approximately 95% of people with cystic fibrosis in the United Kingdom are now eligible for new genetic modulator therapies. As a result, cystic fibrosis centres are treating older populations in greater numbers. Cardiopulmonary exercise testing measures aerobic capacity, however it is resource intensive. Identifying whether routinely collected clinical measures are associated with reduced aerobic capacity is needed to aid prioritisation of cardiopulmonary exercise testing. Methods: Maximal cardiopulmonary exercise testing data were collected from July 2022 to January 2024, alongside routine clinical data (spirometry, body mass index, diabetic status, Pseudomonas aeruginosa colonisation status, modulator status, age and sex). Peak oxygen uptake was analysed as a percentage predicted value (VO2peakpp). Results: Overall aerobic capacity at the centre was low (mean peak oxygen uptake 79.16% predicted). No relationship was identified between body mass index and aerobic capacity (β = 0.23, 95%CI -0.91, 1.37, p = 0.69). When adjusting for other clinical measures, having cystic fibrosis related diabetes (β=-17.56, 95%CI -27.17, -7.95, p < 0.001) and younger age (β = 16.62, 95%CI 4.13, 29.12, p = 0.01) were associated with a reduction in VO2peakpp. Conclusion: Annual CPET for all pwCF may not be necessary or available. This service evaluation found associations with younger age and CFRD and reduced VO2peak who could be targeted for exercise testing and training intervention in the future. Description: We are providing an unedited version of this manuscript to give early access to its findings. Before final publication, the manuscript will undergo further editing. Please note there may be errors present which affect the content, and all legal disclaimers apply.; Data availability: The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.

Access to water and sanitation for people with paraplegia living in rural communities in Bangladesh

2026-03-06T03:01:00Z

Title: Access to water and sanitation for people with paraplegia living in rural communities in Bangladesh Authors: Alam, MJ; Bryant, W Abstract: Purpose: To explore how people in rural Bangladesh with paraplegia accessed water and sanitation in their home environment. Method: Based on a qualitative design, semi-structured interviews were used to gather direct experiences. Six people with complete paraplegia were recruited and their interview data were subject to thematic analysis. Findings: There were different ways of accessing water and sanitation. Experience varied depending on accessibility within the home environment, design of assistive devices, and individual skills. The dynamics between these factors determined how much time and energy were required. Easier access was associated with higher life satisfaction and a sense of occupational balance. Barriers to access have serious implications for health and wellbeing, but independence can be achieved through environmental adaptation or modification, appropriate design of assistive devices and development of individual skills. Conclusions: Community-based rehabilitation and other campaigns could use these findings to advocate for improved access to water and sanitation for people with paraplegia. At an individual level, occupational therapists can address barriers to access, by enabling people to gain skills, and adapt their environment and routines. Combining individual and community approaches could have many benefits for occupational balance, health and well-being.

The relationship between stigma and quality of life of children with rare diseases in Romania

2026-03-02T03:01:02Z

Title: The relationship between stigma and quality of life of children with rare diseases in Romania Authors: Grunebaum, S; Parnis, N; Nagy, L Abstract: The current study examines the impact of parental perceived stigma on the quality of life (QoL) of children with rare diseases in Romania. As rare diseases habitually affect a small portion of the population, they are frequently under-researched, underfunded and patients are often unsupported. Additionally, people with rare diseases often experience high levels of stigma. The present study uses the Paediatric Quality of Life Measure (PedsQL) and the Parental Perceptions of Public Attitudes Scale (PPPAS) to ascertain the parent/primary caregiver reported QoL in children with rare diseases (CwRD) and explore the relationship between stigma and QoL. Using two validated measures, this study identified that stigma has a statistically significant negative impact on QoL whilst controlling for the age, gender, education and employment status of the parent. Every SD increase of stigma (M = 3.35, SD = 1.12) results in a decrease of 0.268 SD of QoL (M = 36.04, SD = 18,13). Over 75% of responders reported physical QoL and low psychosocial health. Since parent/primary caregivers who experience higher levels of stigma are likely to report a lower (QoL) for their CwRD, coproduced interventions to address stigma should be considered by future research to support the physical and psychosocial QoL of CwRD in Romania. Description: Data availability statement: The data that support the findings of this study are available on request from the corresponding author, SG. The data are not publicly available due to the data containing information that could compromise the privacy of research participants.

Power, cardiorespiratory fitness and physical activity in young people with cerebral palsy

2026-03-02T03:01:01Z

Title: Power, cardiorespiratory fitness and physical activity in young people with cerebral palsy Authors: Mavrommati, F; Collett, J; Cossington, J; Coe, S; Theologis, T; Nagy, L; Meaney, A; Wade, W; Dawes, H Abstract: Objectives: Physical activity (PA) levels in young people with cerebral palsy (YPwCP) remain consistently low. Previous research suggests that fitness parameters such as muscular strength and cardiovascular capacity are interrelated with mobility and PA levels in YPwCP. This study aimed to (1) describe fitness parameters and PA levels in YPwCP, (2) explore associations between fitness parameters and PA, and (3) evaluate the reliability of accelerometer-based PA measurement. Methods: A cross-sectional study was conducted with an embedded test–retest reliability assessment of accelerometer-measured PA over a one-month interval. Participants included 36 YPwCP (19 males, 17 females; mean age 15.5 ± 0.7 years), classified within GMFCS levels I–III. Cardiorespiratory endurance (VO2max) was assessed using an incremental cycle ergometer test, and leg power was measured using the Five-Times Sit-to-Stand (5xSTS). PA was measured via self-report using the Physical Activity Questionnaire for Adolescents (PAQ-A) and via device-measured data using a wrist-worn accelerometer over seven consecutive days. Results: We found low levels of PA. The 5xSTS completion time showed moderate to strong correlations with self-reported PA (ρ=−0.384, p≤0.01) and device-measured vigorous PA (ρ=−0.566, p≤0.01). VO2max was positively but not significantly associated with total PA (ρ=0.173) and moderate-to-vigorous PA (ρ=0.115). Accelerometry demonstrated good reliability (ICC=0.796). Conclusions: These findings suggest that accelerometer has good reliability in YPwCP but highlight low levels of PA measured by self-report and accelerometer. Higher lower-limb power, assessed by faster 5xSTS test, was associated with greater PA. Description: Data availability: Research data are not shared.