Background - Non-invasive ventilation has significantly increased life expectancy for individuals with neuromuscular diseases (NMDs). Research is now suggesting that tidal volume ventilation alone is insufficient to manage the vicious cycle of increasing load and progressive weakness that presents. In NMDs, muscle weakness renders ineffective spontaneous sigh breaths, yawns, and coughs. Proposed to maintain lung expansion, compliance, and secretion clearance, the absence of these supratidal inhalations leaves individuals at elevated risk of respiratory tract infections. This is especially evident in childhood-onset NMDs, in which progressive muscle weakness occurs in the context of both pulmonary and musculoskeletal development.
Lung volume recruitment (LVR) is a simple inexpensive technique used to augment inspiration, either before a cough or on a regular basis to mimic lost spontaneous deep breathing activities. LVR has demonstrated effectiveness in improving assisted peak cough flow values across the spectrum of adult onset NMD’s
Purpose - The focus of this systematic review was to consider whether LVR had an effect on pulmonary function test parameters in individuals with progressive childhood-onset NMDs.
Methods A systematic search was undertaken in January 2019 considering LVR in the respiratory management of childhood-onset NMDs. Studies were critically appraised using tools from the Joanna Briggs Institute and data extracted using an adapted tool from the Cochrane effective practice and organization of care group.
Results - Results were compiled using a narrative synthesis approach focused on peak cough flow, forced vital capacity, and maximum inspiratory capacity outcomes.
Conclusions - Though limited, evidence suggests an immediate positive effect of LVR on peak cough flow and a potential long-term effect on the rate of forced vital capacity decline.
Implications - Considering the accepted correlation between forced vital capacity and morbidity, this review suggests that LVR be considered for individuals with childhood-onset NMDs once forced vital capacity starts to deteriorate.

Background:
Bronchiectasis guidelines encourage children to participate in regular physical activity, but there is minimal guidance supporting these recommendations and limited knowledge surrounding current physical activity participation.

Purpose:
To investigate how physically active children with bronchiectasis living in Counties Manukau, are compared to their healthy peers, and how often they achieved daily recommendations of at least 60 minutes of moderate to vigorous physical activity (MVPA) across the week. Secondary aims explored associations between MVPA and demographic or disease severity markers, and the mode of activity and time of day children with bronchiectasis engage in physical activity.

Methods:
A quantitative, cross sectional, observational study was undertaken. Thirty-one children aged 7 to 12 years participated in the study: Bronchiectasis group n=18; Control group n=13. Time spent in MVPA was measured over seven consecutive days using wrist-based ActiGraph wGT3X+ accelerometers. Mode of activity and time of day children engaged in physical activity were measured using the Physical Activity Questionnaire for Children (PAQ-C).

Results:
The Bronchiectasis group completed 31.6 (p=0.034) less minutes of MVPA per day than the Control group, with both groups demonstrating significantly higher (p=0.002) MVPA minutes on weekdays compared to weekend days. On average, 62.3% of the Bronchiectasis group and 86.4% of the Control group achieved daily MVPA recommendations. Weak to moderate associations were found between MVPA minutes and body mass index and socioeconomic hardship. Both groups were most active at school during the week, participating in more informal school yard games then organized activities.

Conclusion:
Children with bronchiectasis are less active than their peers and achieve daily MVPA recommendations less often. Children are most active during the week at school.

Implications:
In-depth physical activity assessment needs to be incorporated in routine bronchiectasis management to proactivity identify and manage inactivity and its associated comorbidities.

Background:
Evidence surrounding extubation markers in a paediatric population is sparse. P0.1 is defined as the negative pressure measured 100ms after the initiation of an inspiratory effort performed against a closed respiratory circuit and correlates with central respiratory drive and effort. A literature review surrounding its use in this population was completed, retrieving two papers, one of which was discarded due to age. The remaining paper, published in 2023 by Charernjiratragul et al., investigated indices to guide if respiratory support was required post-extubation (PE). The study found a P0.1 figure > 0.9 showed higher sensitivity for predicting respiratory support escalation PE.

Purpose:
To determine if a relationship existed between pre-extubation P0.1 values and repository support post extubation, in the paediatric intensive care unit (PICU) population, at the Evelina Children’s Hospital.

Method:
Data was collected over a thirty-day period. Patients suitable for extubation were identified by the physiotherapy team. Three pre-extubation P0.1 measurements were collected before subsequent treatment, if required. At forty-eight hours post-extubation, notes were reviewed to identify the maximum respiratory support required.

Results:
Twenty-seven patients met the inclusion criteria; however, one was unsuccessfully extubated, and their data was excluded. Eighteen patients in total required respiratory support within forty-eight hours PE; eight required high-flow oxygen. The median P0.1 value for these eight patients was 0.9. This was thus used as a threshold value, providing binary data to determine if a significant relationship existed. A chi-squared distribution test identified that a statistically significant relationship (p = 0.0011) existed between P0.1 > 0.9 and the subsequent need for high-flow oxygen PE.

Conclusions:
These results reinforce the potential utility of P0.1 as a reliable marker for predicting respiratory support post-extubation in paediatric cases.

Implications:
A proportion of our participants were extubated post cardiac surgery, which may differ from those in less specialised PICUs.