Pediatric Cystic Fibrosis Care Research Articles

Identifying Disparities in Pediatric Cystic Fibrosis Care Between Low-Middle and Middle-High Income Countries in the Middle East

Cystic Fibrosis (CF) care has advanced tremendously in the United States (US), North America, Australasia, and Europe in recent decades. Given the concern for global disparities in pediatric medical care in other parts of the world, we wanted to assess the availability of key aspects of current pediatric CF care in the Middle East as-compared to the US. We collected electronic surveys from pediatric CF providers in the Middle East and surrounding countries assessing whether treatments known to be widely available in the US were also available in surveyed countries. Many countries in the Middle East, as-compared to the US, do have less overall availability of many key CF diagnostic and treatment modalities. Within the Middle East, 80% of higher-income countries had inhaled tobramycin available compared to none in lower-income countries. This study highlights the additional global collaboration needed to ensure all children with CF receive optimal care globally.

P428 Experiences of paediatric cystic fibrosis care in the UK

P428 Experiences of paediatric cystic fibrosis care in the UK

Patient Attitudes Regarding Health Care Clinician Communication in Adolescents with Cystic Fibrosis.

The quality of health care clinician (HCC) communication varies, yet few studies evaluate ways to improve communication among adolescents with cystic fibrosis (CF). We sought to characterize the attitudes of adolescents and young adults (AYA) with CF about HCC communication and describe the components important for high-quality communication. AYA with CF aged 12-20 years from a single large pediatric CF care center participated in a brief survey and semi-structured individual and group virtual interviews that were recorded, transcribed, coded and analyzed with a combined deductive and inducive approach. Discrepancies were resolved by consensus. Among the 39 survey respondents, most were white (77%), male (51%), and averaged 15.51 years (range 12 - 20 years). Many (40%) perceived their health status as "neutral" and over half (61%) were "very satisfied" with HCC communication. Overall, among the 17 interviews (averaged 53.6 minutes, range 31.5-74 min), participants reported a desire to be actively engaged in discussions about their health and included in the decision-making process with HCC to support adolescent autonomy and cultivate trust. Some factors detract (loss of control and fear of diagnosis), and others strengthen (transition to adult care and external motivators) adolescent autonomy. Some factors detract (perceived lack of interdisciplinary communication, statements of non-compliance, and being compared to others) and others strengthen (inherent trust and familiarity over time) the cultivation of trust. The development of adolescent autonomy and the cultivation and maintenance of trust between the patient and HCC are two essential components of quality communication that should inform future communication-focused interventions.

Lung function and secondhand smoke exposure among children with cystic fibrosis: A Bayesian meta-analysis

BackgroundSecondhand smoke exposure, an important environmental health factor in cystic fibrosis (CF), remains uniquely challenging to children with CF as they strive to maintain pulmonary function during early stages of growth and throughout adolescence. Despite various epidemiologic studies among CF populations, little has been done to coalesce estimates of the association between secondhand smoke exposure and lung function decline. MethodsA systematic review was performed using PRISMA guidelines. A Bayesian random-effects model was employed to estimate the association between secondhand smoke exposure and change in lung function (measured as FEV1% predicted). ResultsQuantitative synthesis of study estimates indicated that second-hand smoke exposure corresponded to a significant drop in FEV1 (estimated decrease: -5.11% predicted; 95% CI: -7.20, -3.47). The estimate of between-study heterogeneity was 1.32% predicted (95% CI: 0.05, 4.26). There was moderate heterogeneity between the 6 analyzed studies that met review criteria (degree of heterogeneity: I2=61.9% [95% CI: 7.3–84.4%] and p = 0.022 from the frequentist method.) ConclusionsOur results quantify the impact at the pediatric population level and corroborate the assertion that secondhand smoke exposure negatively affects pulmonary function in children with CF. Findings highlight challenges and opportunities for future environmental health interventions in pediatric CF care.

351 Rounding out the pediatric cystic fibrosis care team: Including the profession of occupational therapy

351 Rounding out the pediatric cystic fibrosis care team: Including the profession of occupational therapy

68 Increasing care coordination between the primary cystic fibrosis team and gastroenterology in a pediatric cystic fibrosis care center using the model of improvement

68 Increasing care coordination between the primary cystic fibrosis team and gastroenterology in a pediatric cystic fibrosis care center using the model of improvement

97: Optimizing oral glucose tolerance test completion at a pediatric cystic fibrosis care center: A 10-year continuing quality improvement effort

97: Optimizing oral glucose tolerance test completion at a pediatric cystic fibrosis care center: A 10-year continuing quality improvement effort

Safety and effectiveness of a risk-stratified venous thromboembolism prophylaxis algorithm in young people with cystic fibrosis

BackgroundPeople with cystic fibrosis (CF) have an increased risk of thrombosis due to acquired thrombophilia secondary to chronic systemic inflammation and central venous catheter use for treatment of pulmonary infections. The objective of this study is to determine the safety and effectiveness of a risk-stratified, venous thromboembolism (VTE) prophylaxis intervention. MethodsThis single-center, IRB-approved, retrospective study assessed patients with CF admitted to our institution for treatment of a pulmonary exacerbation from 2017 to 2019. Data and outcomes were manually extracted from the electronic medical record and internal CF clinical database. Subject characteristics, calculated VTE risk, prophylaxis interventions prescribed, VTE incidence, and adverse events were captured. ResultsA total of 135 CF patients had 354 admissions for pulmonary exacerbations in the time frame of the study. The majority of admissions (88.7%) were classified as moderate or high risk for VTE using the algorithm. Overall, VTE prophylaxis intervention determined by the algorithm was initiated in 36.2% of admissions. During the study period, no VTE events occurred. Four minor bleeding adverse effects were reported in patients receiving VTE chemical prophylaxis with enoxaparin (4.2%). ConclusionsThis study provides the first reported outcomes following implementation of a risk-stratified VTE prophylaxis algorithm in hospitalized young people with CF. In this population at increased risk, use of risk-stratified prophylaxis was safe and effective in preventing VTE. Additional work to improve and maintain adherence to the algorithm and VTE prophylaxis interventions at our institution is planned and similar care should be considered at other pediatric CF care centers.

P230 A review of paediatric cystic fibrosis care during the COVID-19 pandemic

P230 A review of paediatric cystic fibrosis care during the COVID-19 pandemic

P288 Evaluation of dietetic service provision in UK paediatric cystic fibrosis care: how are we doing?

P288 Evaluation of dietetic service provision in UK paediatric cystic fibrosis care: how are we doing?

Palliative care skills in CF: Perspectives of adults with CF, caregivers, and CF care team members

The role of cystic fibrosis (CF) care team members in delivering palliative care (PC) remains undefined. We aimed to understand the PC skills of CF care teams. CF care team members ("clinicians"), adults with CF ("patients"), and family caregivers ("caregivers") rated the ability of CF clinicians to provide aspects of PC using a five-point scale ("poor" to "excellent"). Median ratings were compared between groups. A total of 70 patients, 100 caregivers, and 350 clinicians participated. Clinicians consistently rated their PC skills higher than patients or caregivers rated them, particularly for advanced PC skills. While clinicians, patients, and caregivers rated clinicians as "very good" at basic pain assessment and "good" at discussing prognostic uncertainty, clinicians rated themselves more highly at providing most skills, including simultaneous PC and standard CF care (P < .0001), basic depression assessment (P < .001), and discussing transplant, advance directives, end of life, code status, and hospice (all P < .0001). Respondents affiliated with adult CF care teams rated clinicians more highly than respondents affiliated with pediatric CF care teams at discussing lung transplant (P < .001), end of life (P = .006), advance directives (P < .001), code status (P = .012), and hospice (P = .016). Most patients (69%) and caregivers (60%) felt CF clinicians should receive more PC training. Discrepancies exist among patient/caregiver and clinician perceptions of PC skills in CF, and skills of adult and pediatric teams may differ. Patients and caregivers feel clinicians' more advanced PC skills are lacking. CF clinicians may benefit from PC training to enhance skills and to understand how and when to utilize specialty PC services.

Complete Genome Sequence of the Multidrug-Resistant Pseudomonas aeruginosa Endemic Houston-1 Strain, Isolated from a Pediatric Patient with Cystic Fibrosis and Assembled Using Oxford Nanopore and Illumina Sequencing.

Hybrid de novo assembly of Illumina/Nanopore sequence data produced complete circular sequences of the chromosome and a plasmid for the multidrug-resistant Pseudomonas aeruginosa Houston-1 strain. This provides a high-quality representative sequence for a lineage endemic to a pediatric cystic fibrosis care center at Texas Children's Hospital.

An Exploration Into Knowledge, Attitudes, and Beliefs Towards Risky Health Behaviours in a Paediatric Cystic Fibrosis Population.

Risky behaviours are prevalent within the cystic fibrosis (CF) population; however, there is a lack of research which has investigated risky behaviour engagement among adolescents with CF, with reasons for initiation currently being unknown, as no qualitative studies have been conducted. This research therefore examines knowledge, attitudes, and beliefs towards risky behaviours at an age commonly associated with initiation. Ten paediatric participants were recruited. Thematic analysis illustrated several psychological factors associated with risky behaviours. A desire for normalcy was evident, with this been associated with a desire to engage in normalised risky behaviours. Evidence of a life-orientated illness perspective was also prevalent, with participants believing that many individuals engage in risky behaviours for fun. Overall, there was a reported lack of knowledge on consequences of risky behaviours, with many participants not being informed of these by health care professionals (HCPs). This research provides insight into an area of CF paediatric care which could be improved on, with the provision of awareness regarding risky behaviours not being embedded within paediatric CF care. Consequently, this research demonstrates the need for interventions to be integrated into paediatric CF care for the prevention and reduction of risky behaviours.

Clinical Adoption of mHealth Technology to Support Pediatric Cystic Fibrosis Care in Sweden: Qualitative Case Study

BackgroundMobile 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.ObjectiveThis study explored the development, adoption, and integration of a new, co-produced mHealth platform (Genia) for the management of pediatric CF in Sweden.MethodsA 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 Skåne 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.ResultsIn 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.ConclusionsPrinciples 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.

217 Medical outcomes post transition of clinical care from a paediatric cystic fibrosis care model to an adult cystic fibrosis care model – an Irish perspective

217 Medical outcomes post transition of clinical care from a paediatric cystic fibrosis care model to an adult cystic fibrosis care model – an Irish perspective

Health Outcomes Associated With Transition From Pediatric to Adult Cystic Fibrosis Care

Almost half of individuals who have cystic fibrosis (CF) are over 18 years old, thus safely transferring patients from pediatric to adult care is a priority. The purpose of this study is to compare youth transferred from pediatric to adult CF care versus those remaining in pediatric CF care and quantify the relationship between transfer status and health outcomes. Patients who transferred from pediatric to adult CF care were identified from the CF Foundation Patient Registry from 1997 to 2007. Transferred patients were compared with individuals who have similar baseline characteristics who remained in pediatric care throughout the same time period. The main outcome measures include pulmonary function, nutritional status, care use, and home intravenous antibiotic events per year. A propensity-matched analysis was performed. Fifty-eight percent of patients remained in pediatric programs throughout the study period. The mean age at transfer to adult care was 21.2 (1.3) years. In the 2 years after transfer there was a less rapid decline in percent predicted forced expiratory volume in 1 second of 0.78 percentage points per year among transfer-positive patients compared with transfer-negative ones (95% confidence interval; 0.06-1.51); there were no other significant health related changes. The current study contradicts reports of other chronic childhood conditions, in which transfer between the pediatric and adult health system was associated with adverse health outcomes. Further research is needed to explore the long-term relationship between transition practices and health status outcomes to establish a systematic, evidence-based transition process.

Molecular Epidemiological Surveillance of Multidrug-Resistant Pseudomonas aeruginosa Isolates in a Pediatric Population of Patients with Cystic Fibrosis and Determination of Risk Factors for Infection with the Houston-1 Strain

Multiyear molecular epidemiological surveillance of multidrug-resistant Pseudomonas aeruginosa (MRPA) in a pediatric cystic fibrosis care center identified an endemic MRPA strain (Houston-1). Recent hospitalization was found to be a statistically significant risk factor for acquisition of the endemic strain. Multiple infection control improvements led to the reduced incidence of the Houston-1 strain in the CF population.

464 Paediatric Cystic Fibrosis care — at a District General Hospital

464 Paediatric Cystic Fibrosis care — at a District General Hospital