Pediatric Cystic Fibrosis Patients Research Articles

The Effectiveness of Viktor Frankl's Logo therapy on the Mental Health of Pediatric Patients with Cystic Fibrosis

Background Individuals with cystic fibrosis (CF) are at high risk of clinically significant anxiety, which can be related to lower treatment adherence and poorer health outcomes. So, it is associated with poor psychosocial and quality of life outcomes. The aim of this study was to assess the effectiveness of Viktor Frankl's Logo therapy on the mental health of pediatric patients with CF. Materials and Methods: This research was a quasi-experimental based on pretest and posttest with a control group. The population under study includes pediatric patients with CF referring to Emam Khomeini hospital, Tehran, Iran, during 2018-19. The range of the participants’ age was between 12 and 16 years. The sample size was selected based on the convenience sampling method. The participants divided randomly into two groups: experimental, n=11 and control n=11. Participants in both experimental and control group completed questionnaires on The General Health Questionnaire (GHQ) before and after training. Treatment process for 45 minutes a week was applied among experimental group. Results: The mean and standard deviation of the patient's age in this study were 15.83± 0.87 years old. The results of this research showed that the score of mental health in the experimental group (25±6.8) increased after the intervention training (p <0.01), compared the control group. Conclusion Based on the results of this study, the effectiveness of logo therapy in fostering mental health among pediatric cystic fibrosis patients with a mean age of 13-18 years was approved after nine sessions, 45 minutes once a week. According to the results, logo therapy helped children with cystic fibrosis improve their mental health.

Pulmonary Function Testing in the Coronavirus Disease Era: Lessons and Opportunities.

Pulmonary Function Testing in the Coronavirus Disease Era: Lessons and Opportunities.

The prevalence of aberrations in body composition in pediatric cystic fibrosis patients and relationships with pulmonary function, bone mineral density, and hospitalizations

BackgroundBody mass index (BMI) correlates with clinical outcomes in cystic fibrosis but has limitations. Body composition aberrations in CF are multifactorial. We sought to evaluate body composition and relationships with pulmonary function, bone health, and hospital admissions. Other aims included defining body composition indices in a cohort of children with CF. MethodsWe conducted a retrospective review of patients with CF, age 8-18 years, seen at Nationwide Children's Hospital (2015-2020). Indices of body composition measured by dual-energy x-ray absorptiometry(DXA) scans. Data included fat mass, fat-free mass (FFM), forced expiratory volume in one second (FEV1%), bone mineral density (BMD), and hospital admissions. NWA was defined as BMI 5th-85th percentile, body fat percentage >85th percentile. FFMD defined as FFMI <10th percentile. StatisticsT-tests compared NWA, FFMD and clinical measurements. Pearson correlations analyzed fat-free mass index (FFMI), fat mass index (FMI), BMI and clinical measurements. ResultsThis study included 114 patients. Mean age 12 years, 72 female. A high prevalence of FFMD existed (n=66, 38.6%). FMI and FFMI correlated with FEV1% (r: 0.23, p:0.01, r: 0.36, p<0.001, respectively) and BMD (r: 0.29, p:0.002). FMI and hospital admissions were related (r:-0.23,p:0.01). FFMD was associated with 9.5% lower FEV1% (p=0.001) and lower BMD Z-score by 1.1 (p<0.001) when compared to no FFMD. ConclusionThis cohort of children with CF had a high prevalence of FFMD and low prevalence of NWA. FFMD was associated with worsened clinical measurements. Patients with FFMD need additional exercise or nutritional intervention. Heterogeneity of body composition definitions creates need for more research.

Bronchodilator responsiveness in cystic fibrosis children treated for pulmonary exacerbations.

Cystic fibrosis (CF) pulmonary exacerbations (PEx) are associated with a significant drop in pulmonary function. The clinical value of measuring bronchodilator (BD) responsiveness during treatment for PEx to monitor or predict recovery of lung function is unclear. A retrospective analysis of spirometry with BD response testing obtained during hospital admissions for PEx in pediatric CF patients. Repeated events were included for patients with BD testing during multiple admissions. Two hundred forty-nine spirometries with BD testing in 102 patients were completed around Day 7 (Days 4-10) of hospital admission for treatment of CF PEx. Median (IQR) forced expiratory volume in 1 s (FEV1 ) was 70.6% predicted (58.1, 84.6) before the PEx event (best FEV1 in 6 months before admission), 54.4% (41.5, 66.9) at admission, 62.3% (48.4, 74.7) around Day 7 of admission and 67.1% predicted (53.8, 78.2) at the end of treatment. BD response around Day 7 correlated poorly with FEV1 before PEx (r = -.16, p = .02) and did not correlate with recovery to baseline FEV1 at end of treatment (r = .08, p = .22). Only 23/249 (9%) individual tests had a BD response in FEV1 of ≥12% and 200 ml. BD response was not related to age or severity of lung disease and led to an immediate change in clinical management in only four cases. Significant BD response in CF patients treated for PEx is rare, shows poor correlation with baseline pulmonary function and does not correlate with the recovery of FEV1 with treatment. These data suggest that routine testing for BD response is not indicated during PEx.

Prevalenţa alergiilor alimentare mediate IgE la pacienţii pediatrici cu fibroză chistică – studiu retrospectiv monocentric

Malnutrition continues to be a major issue concerning pediatric cystic fibrosis patients’ medical care. Supplementary diagnostic association with food allergy in a cystic fibrosis patient leads to worse nutritional status decline. This particular association determines low quality of life and life expectance impairment. Low caloric intake, recurrent pulmonary infections, maldigestion and malabsorption, low treatment adherence, genetic susceptibility and chronic infections are all risk factors and/or causes of malnutrition in cystic fibrosis patients. The aim of our study was to evaluate the prevalence of IgE mediated food allergies in cystic fibrosis patients. A secondary objective was to assess nutritional status in cystic fibrosis patients with food allergies after exclusion diet. In order to accomplish our objectives, we conducted a descriptive, retrospective study on a 68 pediatric patient cohort with cystic fibrosis (CF). All children were diagnosed and followed in the Regional center for CF of INSMC “Alessandrescu-Rusescu”, Bucharest, between 2011 and 2016. After data collection and statistic analysis we observed a prevalence of 11.7% of food allergies in our cystic fibrosis patients cohort. Also, specific exclusion diet impact on nutritional status was evaluated.

Predictors of sinonasal disease onset, progression, and severity in pediatric cystic fibrosis patients

PurposePediatric cystic fibrosis (CF) patients have a variable onset, severity, and progression of sinonasal disease. The objective of this study was to identify genotypic and phenotypic factors associated with CF that are predictive of sinonasal disease, recurrent nasal polyposis, and failure to respond to standard treatment. MethodsA retrospective case series was conducted of 30 pediatric patients with CF chronic rhinosinusitis with and without polyps. Patient specific mutations were divided by class and categorized into high risk (Class I-III) and low risk (Class IV-V). Severity of pulmonary and pancreatic manifestations of CF, number of sinus surgeries, nasal polyposis and recurrence, age at presentation to Otolaryngology, and Pediatric Sinonasal Symptom Survey (SN-5)/Sinonasal Outcome Test (SNOT-22) scores were examined. Results27/30 patients (90%) had high risk mutations (Class I-III). 21/30 (70.0%) patients had nasal polyposis and 10/30 (33.3%) had recurrent nasal polyposis. Dependence on pancreatic enzymes (23/27, 85.2% vs 0/3, 0.0%, p = 0.009) and worse forced expiratory volumes (FEV1%) (mean 79, SD 15 vs mean 105, SD 12, p = 0.009) were more common in patients with high risk mutations. Insulin-dependence was more common in those with recurrent polyposis (5/10, 50% vs 2/20, 10%, p = 0.026). There was no statistical difference in ages at presentation, first polyps, or sinus surgery, or in polyposis presence, recurrence, or extent of sinus surgery based on high risk vs. low risk classification. ConclusionCF-related diabetes was associated with nasal polyposis recurrence. Patients with more severe extra-pulmonary manifestations of CF may also be at increased risk of sinonasal disease.

Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies.

In cystic fibrosis (CF), defective biogenesis and activity of the cystic fibrosis transmembrane conductance regulator (CFTR) leads to airway dehydration and impaired mucociliary clearance, resulting in chronic airway infection and inflammation. The most common CFTR mutation, F508del, results in a processing defect in which the protein is retained in the endoplasmic reticulum and does not reach the apical surface. CFTR corrector compounds address this processing defect to promote mutant CFTR transfer to the apical membrane. When coupled with potentiators to increase CFTR channel activity, these drugs yield significant clinical benefits in CF patients carrying the F508del mutation. However, processing of CFTR and other proteins can be influenced by environmental factors such as inflammation, and the impact of airway inflammation on pharmacological activity of CFTR correctors is not established. The present study evaluated CFTR-rescuing therapies in inflamed CF airway epithelial cultures, utilizing models that mimic the inflammatory environment of CF airways. Primary bronchial epithelial cultures from F508del/F508del CF patients were inflamed by mucosal exposure to one of two inflammatory stimuli: 1) supernatant from mucopurulent material from CF airways with advanced lung disease, or 2) bronchoalveolar lavage fluid from pediatric CF patients. Cultures inflamed with either stimulus exhibited augmented F508del responses following therapy with correctors VX-809 or VX-661, and overcame the detrimental effects of chronic exposure to the CFTR potentiator VX-770. Remarkably, even the improved CFTR rescue responses resulting from a clinically effective triple therapy (VX-659/VX-661/VX-770) were enhanced by epithelial inflammation. Thus, the airway inflammatory milieu from late- and early-stage CF lung disease improves the efficacy of CFTR modulators, regardless of the combination therapy used. Our findings suggest that pre-clinical evaluation of CFTR corrector therapies should be performed under conditions mimicking the native inflammatory status of CF airways, and altering the inflammatory status of CF airways may change the efficacy of CFTR modulator therapies.

Impact of airway Exophiala spp. on children with cystic fibrosis

IntroductionIsolation of Exophiala species from sputum samples has become increasingly reported in Cystic Fibrosis (CF). However, the clinical significance of Exophiala spp. with regards to the paediatric CF population is unknown. MethodsA case control study was undertaken to compare CF children with and without chronic Exophiala spp. in their sputum samples. Demographic and clinical data were collected retrospectively for each case from the date of Exophiala isolation and for 12 months preceding isolation. Each case was compared to three age and year-matched controls. To determine the effect of Exophiala on clinical course, patients were then followed for 12 months post isolation. ResultsIn total, 27 of 244 eligible paediatric CF patients (11%) isolated Exophiala spp. on more than one occasion. There were no significant differences in the key clinical parameters: spirometry, mean number of intravenous (IV) antibiotic days and body mass index (BMI), between cases and controls (p = 0.91, p = 0.56 and p = 0.63 respectively). A higher proportion of cases isolated Candida spp. (67% vs 21%, p < 0.0001) and Aspergillus fumigatus (37% vs 26%, p = 0.37). There was no clinically significant difference in spirometry, mean number of IV antibiotic days and BMI in cases pre and post Exophiala spp. isolation. Posaconazole was the only drug used that successfully eradicated Exophiala. ConclusionDespite the frequent isolation of Exophiala spp. in this cohort, in most patients it is not associated with significant clinical deterioration. It does however seem to be associated with isolation of other fungi.

Reflex zone stimulation reduces ventilation inhomogeneity in cystic fibrosis: A randomised controlled cross-over study.

The reflex zone stimulation technique (RST) activates complex motor responses and has a positive impact on the locomotor system. This technique may also indirectly affect breathing; however, the use of this technique as adjunct of the treatment of cystic fibrosis (CF) has not yet been characterised. We performed a randomised controlled single-centre interventional trial to evaluate the short-term effects of RST on lung function in 21 paediatric CF patients with normal baseline spirometry. The effect of 30 min of RST was compared to that of sham therapy in a crossover design. The interventions were performed in random order and planned 6 months apart. The primary outcome was a change in global ventilation inhomogeneity after intervention, assessed by lung clearance index (LCI2.5 ) derived from a nitrogen multiple breath washout test. Secondary outcomes included changes in regional ventilation inhomogeneity (indices of acinar [Sacin*Vt] and conductive airway [Scond*Vt] inhomogeneity) and spirometry parameters (inspiratory capacity, forced vital capacity, and forced expiratory volume in 1 s). Trunk deformity was assessed by physiotherapists at study entry. After the RST intervention, the LCI2.5 (p = .004) and Scond*Vt (p = .009) decreased significantly, while inspiratory capacity increased (p = .012). In the sham-therapy group, none of the parameters changed significantly. Trunk deformity was seen in 76.5% of all patients, and 92.9% of those with trunk deformity showed a decrease in LCI2.5 after RST. RST has multiple positive short-term effects on lung function, especially in CF patients with trunk deformities.

The clinical effects of combining postural exercises with chest physiotherapy in cystic fibrosis: A single-blind, randomized-controlled trial.

Objectives This study aims to investigate the effects of postural exercises as an adjunct to chest physiotherapy program on respiratory function, exercise tolerance, quality of life (QoL), and postural stability in patients with cystic fibrosis (CF). Patients and methods In this single-blind, randomized-controlled trial, 19 pediatric CF patients (11 males, 8 females; mean age: 9.36 years; range, 6 to 14 years) were randomly allocated to chest physiotherapy and postural exercise program (Group 1, n=10) or chest physiotherapy program alone (Group 2, n=9) between March 2017 and October 2017. Respiratory functions were assessed with pulmonary function tests, whereas exercise tolerance with the Modified Shuttle Test (MST), quality of life with the Cystic Fibrosis Questionnaire-Revised Child Version (CFQR), and postural stability with the Limits of Stability Test (LOS). All tests were performed before treatment and six weeks, three months, and six months after treatment. Results Respiratory functions were improved in both groups; however, these changes were not statistically significant. The MST increased after treatment in both groups (p<0.001 and p=0.003 respectively), without a significant difference between the groups. Emotional function and treatment difficulties subdomains in CFQR were significantly increased only in the group with postural exercises (p<0.05). Conclusion The postural exercise program in addition to chest physiotherapy in pediatric CF patients whose postural changes were not taken place did not cause significant changes in respiratory function, exercise tolerance, and postural stability; however, it affected the emotional state well and improved the compliance with the treatment.

The effects of nutritional status and intervention on pulmonary functions in pediatric cystic fibrosis patients

Cystic fibrosis (CF) causes malabsorption of nutrients that exacerbate pulmonary problems. Nutritional interventions can improve pulmonary functions. We aimed to evaluate the effects of nutritional intervention in CF patients with malnutrition, and to determine ifthere is a correlation between nutritional status and pulmonary functions. The study included 143 CF patients (67 females) with a mean 2year follow-up time. Patients' sociodemographic data, presenting symptoms and history were recorded. Height-for-age, weight-for-age, weight-for-length/height (WFL/H), and body mass index (BMI) were calculated in all patients. Patients were grouped as well nourished, mild malnutrition, moderate malnutrition, and severe malnutrition. These four groups were compared in terms of pulmonary function test results, lung infections, and the hospitalization rate. Among the patients with a WFL/H or BMI z-scores that decreased, the frequency of lung infection was 74.1% and the hospitalization rate was 40.7%, versus 34% and 12.3%, respectively among the patients with a WFL/H or BMI z-scores that increased. The difference was significant (P=0.02 and P=0.01, respectively). The difference in bacterial lung infections differed significantly between the four nutritional status groups (P=0.002). Patients in the well-nourished group had significantly higher pulmonary function test scores than the other groups. The forced expiratory volume in the first second differed significantly between the patients with and without an increase in the WFL/H or BMI z-scores (P=0.001). The appropriate nutritional intervention to pediatric CF patients with malnutrition, decrease the frequency of lung infections, and improve respiratory function.

Subgroup causal effect identification and estimation via matching tree

Inferring causal effect from observational studies is a central topic in many scientific fields, including social science, health and medicine. The statistical methodology for estimating population average causal effect has been well established. However, the methods for identifying and estimating subpopulation causal effects are relatively less developed. Part of the challenge is that the subgroup structure is usually unknown, therefore, methods working well for population level effect need to be modified to address this. A tree method based on a matched design is proposed to identify subgroups with differential treatment effects. To remove observed confounding, propensity-score-matched pairs are first created. Then the classification and regression tree is applied to the within-pair outcome differences to identify the subgroup structure. This nonparametric approach is robust to model misspecification, which is important because it becomes much harder to specify a parametric outcome model in the presence of subgroup effects. In addition to describing assumptions under which our matching estimator is unbiased, algorithms for identifying subgroup structures are provided. Simulation results indicate that the proposed approach compares favorably, in terms of the percentage of correctly identifying true tree structure, with other competing tree-based methods, including causal trees, causal inference trees and the virtual twins approach. Finally the proposed method is implemented to examine the potential subgroup effect of the timing of Tobramycin use on chronic infection among pediatric Cystic Fibrosis patients.

Comparative genomics of ST5 and ST30 methicillin-resistant Staphylococcus aureus sequential isolates recovered from paediatric patients with cystic fibrosis

Staphylococcus aureus chronic airway infection in patients with cystic fibrosis (CF) allows this pathogen to adapt over time in response to different selection pressures. We have previously shown that the main sequence types related to community-acquired methicillin-resistant S. aureus (MRSA) infections in Argentina – ST5 and ST30 – are also frequently isolated from the sputum of patients with CF, but in these patients they usually display multi-drug antimicrobial resistance. In this study, we sequenced the genomes of MRSA from four paediatric CF patients with the goal of identifying mutations among sequential isolates, especially those possibly related to antimicrobial resistance and virulence, which might contribute to the adaptation of the pathogen in the airways of patients with CF. Our results revealed genetic differences in sequential MRSA strains isolated from patients with CF in both their core and accessory genomes. Although the genetic adaptation of S. aureus was distinct in different hosts, we detected independent mutations in thyA, htrA, rpsJ and gyrA – which are known to have crucial roles in S. aureus virulence and antimicrobial resistance – in isolates recovered from multiple patients. Moreover, we identified allelic variants that were detected in all of the isolates recovered after a certain time point; these non-synonymous mutations were in genes associated with antimicrobial resistance, virulence, iron scavenging and oxidative stress resistance. In conclusion, our results provide evidence of genetic variability among sequential MRSA isolates that could be implicated in the adaptation of these strains during chronic CF airway infection.

Intestinal Dysbiosis in Young Cystic Fibrosis Rabbits.

Individuals with cystic fibrosis (CF) often experience gastrointestinal (GI) abnormalities. In recent years, the intestinal microbiome has been postulated as a contributor to the development of CF-associated GI complications, hence representing a potential therapeutic target for treatment. We recently developed a rabbit model of CF, which is shown to manifest many human patient-like pathological changes, including intestinal obstruction. Here, we investigated the feces microbiome in young CF rabbits in the absence of antibiotics treatment. Stool samples were collected from seven- to nine-week-old CF rabbits (n = 7) and age-matched wild-type (WT) rabbits (n = 6). Microbiomes were investigated by iTag sequencing of 16S rRNA genes, and functional profiles were predicted using PICRUSt. Consistent with reports of those in pediatric CF patients, the fecal microbiomes of CF rabbits are of lower richness and diversity than that of WT rabbits, with a marked taxonomic and inferred functional dysbiosis. Our work identified a new CF animal model with the manifestation of intestinal dysbiosis phenotype. This model system may facilitate the research and development of novel treatments for CF-associated gastrointestinal diseases.

Cyproheptadine as an appetite stimulant in children with cystic fibrosis.

Cystic fibrosis (CF) is a multi-organ genetically inherited disease that leads to progressive lung disease and nutrient malabsorption. The aim of this study was to assess the effectiveness of cyproheptadine (CH) (Periactin®) as an appetite stimulant on improving the nutrition status of paediatric patients with CF. We conducted a retrospective study of 15 patients with a suboptimal nutrition status prescribed CH for ≥12 months from 2013 to 2018. Change in Body Mass Index (BMI) z-score and lung function before vs. after treatment with CH were measured as well as dose-response relationship. The mean change in BMI z-score over 12 months of treatment with CH was+0.91 compared to-0.52 in the previous 12 months (p∗∗∗=0.0002). There was also a trend towards an improvement in lung function over the 12 months of CH treatment compared to the 12 months prior (+2.79 vs-6.2% (p=0.07)). No dose-response relationship was observed. These results suggest that CH is effective at improving the nutrition status of paediatric CF patients with suboptimal nutrition.

Cost-effectiveness of implementing routine hearing screening using a tablet audiometer for pediatric cystic fibrosis patients receiving high-dose IV aminoglycosides.

BACKGROUND: Cystic fibrosis (CF) patients who receive high-dose aminoglycosides can acquire inner ear damage and subsequent hearing loss. There is no current standard protocol for assessing ototoxicity in CF centers in the United States. OBJECTIVE: To evaluate the cost-effectiveness of a pharmacist-implemented routine hearing screening for ototoxicity among pediatric patients using a clinically validated tablet audiometer to allow for earlier detection of hearing loss in an exploratory analysis. METHODS: A Markov decision-analytic model was developed to assess the cost-effectiveness of implementing routine screening with monthly cycles over a 3-year time horizon. The model measured the difference in promptly detected hearing loss, delayed detected hearing loss, and undetected hearing loss, compared with current screening practices. Model inputs were obtained through a comprehensive literature review. Primary model outcomes included total health care costs and quality-adjusted life-years (QALYs) gained with a 3% yearly discount. One-way, two-way, and probabilistic sensitivity analyses were conducted to evaluate model uncertainty. RESULTS: In a hypothetical cohort of 100 patients, routine screening using a tablet audiometer increased promptly detected hearing loss by 8 patients. There was an incremental gain of 3.2 QALYs at an increased cost of $333,826 compared with current screening practices. This resulted in an incremental cost-effectiveness ratio (ICER) of $103,771 per QALY. In the 1-way sensitivity analysis, the ICER ranged between $64,345 and $258,830 per QALY. CONCLUSIONS: Using a tablet audiometer for routine hearing screening appears to be a cost-effective option at a $150,000 per QALY willingness-to-pay threshold when only considering the immediate benefits gained. This analysis did not examine the long-term effects of early detection in language development for pediatric patients. DISCLOSURES: Huang reports funding from the University of North Carolina and GlaxoSmithKline Health Outcomes Fellowship. GlaxoSmithKline had no involvement in the study creation, analysis, or manuscript composition. The other authors have nothing to disclose.

P056 Intestinal current measurement (ICM) in cystic fibrosis paediatric patients - a pilot study

P056 Intestinal current measurement (ICM) in cystic fibrosis paediatric patients - a pilot study

P219 Before and during the COVID-19 pandemic: a review of health of our paediatric cystic fibrosis patients

Objective: To explore the impact of the COVID-19 pandemic on our cystic fibrosis (CF) patients’ health. Method: A retrospective review of data for 20 paediatric CF patients between 01/03/2020 to 30/11/2020 compared with 01/03/2019 to 30/11/2019. This included: • Days requiring intravenous antibiotics (IVAB) • Course of oral antibiotics (abx) • Routine vs acute IVAB • Chest x-ray (CXR) Northern score • Lung function tests (LFT) • Body mass index (BMI) • COVID-19 antibody test • Acute presentations Results: Oral abx courses for exacerbations or positive cultures decreased from 29 to 14;total IVAB days decreased from 177 to 107. Patients requiring rescue IVAB was the same (2). Of 19 patients had CXR done, score was unchanged in 8, reduced in 5 and increased in 6. 5 patients (under 6 years) did not complete LFTs, no data for 4 patients due to phone appointments, 10 patients FEV1 remained stable and 1 patient's FEV1 decreased by 8%. 15/20 patients had stable or improved BMI and 3 had decreased BMI. 17 patients had COVID-19 antibody tests. Only 1 tested positive. Number of acute presentations halved to 5 in 2020 compared to 10 in 2019. Conclusion: While our ability to deliver care for CF patients was significantly impacted due to redeployment and service reconfiguration, we succeeded in completing annual review investigations for our 20 patients, which also included COVID-19 antibody testing. Overall, our patients remained in good health as evidenced by decreased acute presentations, reduced need for abx therapy, stable radiology, lung functions and BMI. This could be due to patient shielding, reducing exposure to potential bacteria and viruses, and access to CF care remaining available. The introduction of NuvoAir in 2020 allowed us to remotely monitor LFTs and weight and respond to changes promptly. However, this is relying on patient technique and accuracy of measurements. The forced reconfiguration of services does not appear to have had an impact on patient health.

P110 A soft, flexible, wearable device for cough detection in paediatric cystic fibrosis patients

Objectives: New or increased cough is an important sign of pulmonary exacerbation. There is unmet need to objectively quantify and analyse cough trends in early life. Advances in flexible electronics and materials allow development of soft, skin-like, accelerometer-based wearable devices that seamlessly interface with the human body in unique locations, enabling the simultaneous capture of low-frequency body, chest, and throat motion along with high-frequency vocal, throat, and lung sound signals associated with various body processes including coughs and vital signs. Methods: A small, flexible, fully wireless, accelerometer-based mechano-acoustic sensor (MAS) was applied using gentle adhesives to the suprasternal notch of 10 paediatric CF subjects during regular clinic visits. A 15-minute protocol consisting of eliciting cough, throat clearing, speech, and laughs in various head/body orientations, ambient environments, and physical activity intensities. The MAS recorded at a high bandwidth 1.6kHz rate and automatically uploaded to a cloud server upon replacement on a wireless charging platform. Results: The captured sensor data was analysed using an existing machine learning algorithm developed for COVID-19 symptom tracking. Preliminary results show the device differentiates cough from other vocal, respiratory noises and motion artifacts, both at rest and during activity. A majority of children found the device to be acceptable, though device size and adhesive removal caused minor discomfort for some. Conclusion: The extraction of well-classified cough events is feasible, facilitating further analysis of cough episode duration, force, and clinically relevant features associated with early decompensation, response to treatment, and daily symptom tracking. Objective measurement of cough may be useful as a clinical study outcome measure and for clinical monitoring. Future work will include evaluation for longer time periods during stability and pulmonary exacerbations.

EPS2.07 How did the COVID-19 lockdown influence the nutritional status and body composition of cystic fibrosis patients in Poland?

Background: The global COVID-19 pandemic has changed daily life, due to restrictions affecting day-to-day functioning. The strict lockdown changed habits of cystic fibrosis (CF) patients as well. The aim of our study was to evaluate the impact of the one-month strict spring lockdown in Poland on the nutritional status and body composition of CF paediatric patients. Methods: All CF patients between 8 and 18 years old who had both in- and outpatient visits with completed data at the Cystic Fibrosis Centre in Warsaw were included in the study. BMI z-scores (CDC calculator), as well as the fat free mass index (FFMI) and the fat mass index (FMI) were calculated according to the following formulas: FFMI = FFM/height2, FMI = FM/height2. The strict lockdown (SL) in Poland was between 16.03.20 and 20.04.20. The measures were evaluated on average: 3 months before SL (M1);3 months after SL (M2), and 3 months after M2 (M3). Results: 101 CF subjects (51% female), with a mean age of 11.53 ± 3.29 years were assessed. 48% of them were heterozygotes F508del and 80% had pancreatic insufficiency. The highest significant increase in values of BMI z-score and FMI was observed after SL (M2) with decrease in M3 (F = 7.18;p = 0.001;F = 4.71;p = 0.01, respectively). FFMI increased significantly and continually from M1 to M3 (F = 14.17;p < 0.001). The mean values for BMI z-score, FMI and FFMI were, respectively, as follows: M1: [–0.20 ± 0.86;4.00 ± 1.51;13.81 ± 1.86];M2: [–0.05 ± 0.82;4.19 ± 1.54;14.05 ± 1.91];M3: [–0.14 ± 0.84;4.07 ± 1.46;14.17 ± 1.91]. The mean 3 months differences between measures [M2 vs M1] and [M3 vs M2] were negative for BMI z-score [–0.17 ± 0.49;p = 0.005];FMI [–0.21 ± 0.77;p = 0.006];FFMI [–0.07 ± 1.15;p = 0.56]. Conclusion: CF paediatric patients improved nutritional status after strict lockdown with increase both FMI and FFMI, nevertheless a decline with time was observed in BMI z-scores and FMI, but not for FFMI.