Pediatric Cystic Fibrosis Center Research Articles

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.

Elimination of Australian epidemic strain (AES1) pseudomonas aeruginosa in a pediatric cystic fibrosis center.

In this cohort study spanning an 18-year period, we evaluated the prevalence and associated mortality rate of epidemic strains of pseudomonas aeruginosa (PsA), especially Australian Epidemic Strain Type 1 (AES1), in a pediatric cystic fibrosis center practicing cohort segregation and early PsA eradication. Cohort segregation was introduced in January 2000. PsA clonal strain was determined by pulse-field-gel-electrophoresis (PFGE) at the time of routine collection of airway specimens. Children with PsA underwent eradication treatment with anti-pseudomonal antibiotics over 2-3 months. We analyzed changes in prevalence and mortality from 1999 to 2016. The prevalence of AES1 declined from 69 (20%) in 1999 to 16 (5.4%) in 2006, to 1 (0.4%) in 2016. The prevalence of PsA overall diminished less over the same period, from 128 (37%) patients in 1999 to 57 (23%) in 2016. New acquisition of AES1 became less common over time, with no new cases identified from 2011. Those who contracted AES1 had a greater risk of death than those who did not (Odds Ratio 4.9, 95%CI 2.5-9.6). Patients with other AES PsA types were uncommon (AES2 n = 5, AES5 n = 2, AES14 n = 3, AES19 n = 1). Cohort segregation was associated with reduction in AES1 prevalence ascertained by PFGE surveillance for patients in a single large pediatric cystic fibrosis center. Other alterations in practice such as early eradication treatment may also have contributed to reduced PsA prevalence. These factors combined with the transition of chronically infected patients over time to adult centers has eliminated AES1 from our clinic, with an accompanying mortality decrease.

Sinonasal computed tomography in pediatric cystic fibrosis: do we know the indications?

ObjectivesTo investigated the correlation between sinonasal computed tomography (SNCT) findings and sinonasal symptoms, genotype, chronic colonization by Pseudomonas aeruginosa, forced expiratory volume in the first second (FEV1), body mass index (BMI), Shwachman-Kulczycki (SK) score, and Bhalla score in patients with cystic fibrosis. MethodsWe retrospectively reviewed the medical records of 61 patients aged 2–16 years who received care at the Pediatric Cystic Fibrosis Center of our institution over a 10-year period. SNCT findings were graded using the Lund-Mackay scoring system. For patients who had SNCT scans performed at two different time points, the findings from both examinations were compared. ResultsPatients with chronic P. aeruginosa colonization and patients with atelectasis had higher Lund-Mackay scores (p = 0.04 and p = 0.01, respectively). There was no difference in Lund-Mackay scores between sinonasal symptomatic and asymptomatic patients (p = 0.45). Among patients who had two SNCT scans available (n = 11), those with no evidence of bronchiectasis on chest CT had decreased Lund-Mackay scores compared to those with evidence of bronchiectasis, who had an increase in their scores (p = 0.03). Variations in the Bhalla score were positively and variations in the SK score were negatively correlated with variations in the Lund-Mackay score (r = 0.74, p = 0.01; and r = −0.85, p < 0.01). ConclusionsAssociations between SNCT findings, chronic P. aeruginosa colonization, SK score, and chest CT findings were demonstrated. Further studies with larger sample sizes are needed to evaluate patient follow-up and assess the benefits of the sinonasal treatment strategy adopted for patients who exhibit pulmonary deterioration despite controlling for other factors associated with exacerbation.

P122 Change in the Lung Clearance Index (LCI) with microbiological status in children with cystic fibrosis - pilot study in a new paediatric cystic fibrosis centre in Poland

P122 Change in the Lung Clearance Index (LCI) with microbiological status in children with cystic fibrosis - pilot study in a new paediatric cystic fibrosis centre in Poland

P101 Clinical data of patients with nontuberculous mycobacteria in a paediatric cystic fibrosis centre in Slovenia

P101 Clinical data of patients with nontuberculous mycobacteria in a paediatric cystic fibrosis centre in Slovenia

EPS6.03 Hypertonic saline use in our paediatric cystic fibrosis centre: using the UK cystic fibrosis Registry to reflect on practice

EPS6.03 Hypertonic saline use in our paediatric cystic fibrosis centre: using the UK cystic fibrosis Registry to reflect on practice

P273 Human papilloma virus vaccination among female patients attending the Auvergne-Rhône Alpes paediatric cystic fibrosis centres

P273 Human papilloma virus vaccination among female patients attending the Auvergne-Rhône Alpes paediatric cystic fibrosis centres

A quality improvement program for adolescents with cystic fibrosis: focus on psychosocial skills

BackgroundThe two pediatric cystic fibrosis centers (CFCs) in Paris (Robert Debré) and Nantes, France, have been developing therapeutic patient education (TPE) programs since 2006 and have been engaged in the pilot phase of the quality improvement program (QIP) named the Hospital Program to Improve Outcomes and Expertise in Cystic Fibrosis (PHARE-M) since 2011. The objective was to improve the FEV1 of the cohort of adolescents to prepare them for their optimal transition to an adult CFC.MethodsThe two CFCs formed a multidisciplinary quality team and used the analysis of causes of insufficient respiratory function taking into account the adolescents’ psychosocial factors. At the Nantes CFC, the approach was centered on adolescents’ body image and their motivation to take care of themselves by assigning specific aspects of patient follow-up to each professional in the team. At R. Debré, an individual cause-and-effect diagram identified for each patient the medical and psychosocial factors that could account for insufficient respiratory function. Personalized actions were offered to each patient.ResultsIn 2014, the median FEV1 (Forced Expiratory Volume in 1 Second) of the adolescent cohort exceeds 90% at the 2 CFCs (Nantes and R. Debré). Between 2011 and 2014 both centers improved their ranking for FEV1% in adolescents in the Registry histograms. At R. Debré, the personalized process allowed to reinforce equality of care, offering to all the opportunity to benefit from TPE sessions and coaching with an adapted physical activity teacher. The psychologist developed a specific tool to support the patient-centered process.ConclusionThe link between TPE and QIP was strong at our two centers enhancing patient centered care and targeting an optimal transition to an adult program.

Stenotrophomonas maltophilia: A marker of lung disease severity.

BackgroundWhile the prevalence of Stenotrophomonas maltophilia lung infection in cystic fibrosis (CF) patients has increased in the last decades, its pathogenicity remains controversial. The aim of this study was to investigate the effects of S. maltophilia initial infection on the progression of lung disease in CF children.MethodsThis case‐control retrospective study took place in a pediatric CF center. A total of 23 cases defined by at least one sputum culture positive for S. maltophilia, were matched for age, sex, and CFTR mutations to 23 never infected CF controls. The clinical data were collected for 2 years before and after S. maltophilia initial infection and comprised lung function analyses, rates of exacerbations and of antibiotic courses.ResultsCompared with controls, cases had lower lung function (P = 0.05), more frequent pulmonary exacerbations (P = 0.01), hospitalizations (P = 0.02), and intravenous antibiotic courses (P = 0.04) before S. maltophilia acquisition. In the year following S. maltophilia initial infection, lung function decline was similar in cases and controls but cases remained more severe, with more frequent pulmonary exacerbations (P = 0.01), hospitalizations (P = 0.02) and intravenous antibiotic courses (P = 0.02).Conclusions S. maltophilia seems to be a marker of CF lung disease severity and international recommendations to reduce lung infection by this pathogen should rapidly emerge.

Cystic fibrosis newborn screening: outcome of infants with normal sweat tests

Newborn babies positively screened for cystic fibrosis (CF) (high serum immunoreactive trypsin (IRT) with DNA analysis) are referred for a diagnostic sweat test, which may be normal (sweat chloride <30...

Impact of pharmacy services on cystic fibrosis medication adherence.

The purpose of this study is to characterize the impact of pharmacy services on medication adherence and hospitalizations for pediatric cystic fibrosis (CF) patients. A retrospective health insurance claims analysis and patient medical charts review from January 1, 2014 to December 31, 2016 of patients from the Pediatric Intermountain CF Center was performed. Adherence to dornase alfa and hospital admissions for pulmonary exacerbations pre and post the implementation of an integrated pharmacy team were reviewed. Dornase alfa adherence was measured by the medication possession ratio (MPR) both monthly and yearly. Fifty-four patients met inclusion criteria. The mean dornase alfa yearly MPR improved from 0.75 (2014) to 0.92 (2016). Patients were 2.8 times more likely to be adherent to dornase alfa when followed by integrated pharmacy team model (P < 0.001), and 2.4 times more likely to be adherent when followed by a dedicated CF clinic pharmacist only (P = 0.001). The study demonstrated that pharmacy services improved adherence to dornase alfa.

381 A survey of TIVAD management and complications in UK paediatric and adult cystic fibrosis centres

381 A survey of TIVAD management and complications in UK paediatric and adult cystic fibrosis centres

254 Feasibility of implementing cardiopulmonary exercise testing in a regional paediatric cystic fibrosis centre in the UK

254 Feasibility of implementing cardiopulmonary exercise testing in a regional paediatric cystic fibrosis centre in the UK

Early severe anemia as the first sign of cystic fibrosis.

Severe anemia is reported to occur rarely in patients with cystic fibrosis (CF). This study aimed to determine the factors associated with early severe anemia in infants with CF. This study included 231 infants with CF from 3 pediatric CF centers ten year period that were retrospectively reviewed in terms of severe anemia as the first sign of CF. Factors that could affect anemia, such as age, pancreatic insufficiency, mutations, vitamin A and E, and albumin level were evaluated. Clinical and laboratory findings in CF patients that presented with severe anemia and no respiratory symptoms were compared to those in CF patients that did not present with severe anemia. Severe anemia as the first sign of CF was noted in 17 of 231 patients. Patient age, prolonged PT/INR and the albumin level differed significantly between the 2 groups of patients (P<0.001). Feeding pattern, pancreatic insufficiency, vitamin E and A levels, and the types of genetic mutations did not differ between the 2 groups. The mean hemoglobin level was 5.59±0.21g/dL and respiratory symptoms began a mean 6.3months after diagnosis of CF in the anemia group. In early infancy severe anemia in the absence of respiratory symptoms can be the first sign of CF. CF should be considered in the differential diagnosis of severe anemia in infants. Anemia can occur several months before respiratory symptoms in patients with CF and may be caused due to several reasons. • Severe anemia as a first sign is reported to occur rarely in patients with cystic fibrosis. • Although anemia is well known in cystic fibrosis, factors that cause severe anemia are not known clearly. What is New: • This study shows the importance of severe anemia as the first sign of cystic fibrosis. • Anemia can occur several months before respiratory symptoms in patients with CF.

The social network of cystic fibrosis centre care and shared Pseudomonas aeruginosa strain infection: a cross-sectional analysis

Person-to-person transmission is a potential pathway of Pseudomonas aeruginosa acquisition in cystic fibrosis. Reports of cross-infection of shared cystic-fibrosis-specific P. aeruginosa strains across large geographical distances are concerning. Therefore, we aimed to assess the extent to which patient movement between cystic fibrosis centres contributes to dissemination. We did a cross-sectional study to assess movement of patients with cystic fibrosis who were infected with P. aeruginosa between Sept 3, 2007, and June 16, 2010, at 18 Australian cystic fibrosis centres. We applied social network analysis to patient movement data from P. aeruginosa-infected patients to assess the role of patient mobility in P. aeruginosa genotype prevalence. We generated networks linking treatment centres based on the movement of patients attending adult and paediatric cystic fibrosis centres, and compared these with the movement of patients infected with all P. aeruginosa strains, unique strains, and predominant Australian shared strains (AUST-01 and AUST-02). We summarised connectivity using degree centrality, in-degree centrality, out-degree centrality, and k-core estimates. Infection control and surveillance practices were also assessed by use of a questionnaire. 983 patients (mean age 25 years [SD 10]; 551 [56%] male) provided 2887 P. aeruginosa isolates for ERIC-PCR genotyping, which yielded 531 distinct genotypes: 493 unique strains in 373 patients and 38 shared strains in 610 patients. AUST-01 infections were associated with higher in-degree centrality (p=0·004) and k-core (p=0·005) estimates and AUST-02 infections with higher degree centrality (p=0·002), out-degree centrality (p=0·002), and k-core (p=0·007) estimates for the previous health-care facilities; associations for the present cystic fibrosis centre were not significant. These findings were significant for adult patients (AUST-01 in-degree centrality p=0·004 and k-core p=0·005; AUST-02 degree centrality p=0·004, out-degree centrality p=0·003, and k-core p=0·007), but not for paediatric patients. By contrast, infections with unique strains were associated with a lower k-core estimate for the present cystic fibrosis centre overall (p<0·0001); this finding was significant in adults (p<0·0001), but not in paediatric patients. Our results show that the connectivity of cystic fibrosis centres, as measured by the movement of patients, seems to be an important risk factor for the acquisition of shared P. aeruginosa strain infections. These results show the importance of prioritising infection control interventions (eg, prospective molecular surveillance for shared P. aeruginosa strains, strict universal infection control precautions, and hospital design and ventilation) to limit P. aeruginosa cross-infection between patients with cystic fibrosis. Australian National Health and Medical Research Council; Children's Health Foundation Queensland; Office of Health and Medical Research, Queensland Health; European Respiratory Society-European Union; Australian Cystic Fibrosis Research Trust; Prince Charles Hospital Foundation; and Rotary Australia.

Adherence to treatment in children and adolescents with cystic fibrosis: a cross-sectional, multi-method study investigating the influence of beliefs about treatment and parental depressive symptoms.

BackgroundAdherence to treatment is often reported to be low in children with cystic fibrosis. Adherence in cystic fibrosis is an important research area and more research is needed to better understand family barriers to adherence in order for clinicians to provide appropriate intervention. The aim of this study was to evaluate adherence to enzyme supplements, vitamins and chest physiotherapy in children with cystic fibrosis and to determine if any modifiable risk factors are associated with adherence.MethodsA sample of 100 children (≤18 years) with cystic fibrosis (44 male; median [range] 10.1 [0.2-18.6] years) and their parents were recruited to the study from the Northern Ireland Paediatric Cystic Fibrosis Centre. Adherence to enzyme supplements, vitamins and chest physiotherapy was assessed using a multi-method approach including; Medication Adherence Report Scale, pharmacy prescription refill data and general practitioner prescription issue data. Beliefs about treatments were assessed using refined versions of the Beliefs about Medicines Questionnaire-specific. Parental depressive symptoms were assessed using the Center for Epidemiologic Studies Depression Scale.ResultsUsing the multi-method approach 72% of children were classified as low-adherers to enzyme supplements, 59% low-adherers to vitamins and 49% low-adherers to chest physiotherapy. Variations in adherence were observed between measurement methods, treatments and respondents. Parental necessity beliefs and child age were significant independent predictors of child adherence to enzyme supplements and chest physiotherapy, but parental depressive symptoms were not found to be predictive of adherence.ConclusionsChild age and parental beliefs about treatments should be taken into account by clinicians when addressing adherence at routine clinic appointments. Low adherence is more likely to occur in older children, whereas, better adherence to cystic fibrosis therapies is more likely in children whose parents strongly believe the treatments are necessary. The necessity of treatments should be reinforced regularly to both parents and children.Electronic supplementary materialThe online version of this article (doi:10.1186/s12890-015-0038-7) contains supplementary material, which is available to authorized users.

Disparities in Parental Health Literacy at a Pediatric Cystic Fibrosis Center

Background: Hispanic cystic fibrosis (CF) patients experience higher morbidity and mortality than non-Hispanic patients, even after adjustment for socioeconomic status. Reduced parental health literacy is associated with adverse child health outcomes in chronic disease, but has not been characterized in CF. Methods: Twenty-five parents of Hispanic children and 63 parents of non-Hispanic children with CF participated in the study. Health literacy was assessed using the English or Spanish version of a validated instrument according to each parent's preference. Results: Average health literacy fell in the adequate range for parents of both Hispanic and non-Hispanic CF patients, and was not significantly different between groups (p=0.17). Four parents of Hispanic CF patients (16%) demonstrated inadequate or marginal health literacy compared to zero parents of non-Hispanic CF patients (p=0.005). Conclusions: While parents of Hispanic children with CF were at higher risk for reduced health literacy, median heal...

Fat soluble vitamin deficiency in children with cystic fibrosis in NSW

Aims To evaluate fat-soluble vitamin status and prothrombin time (PT) in children with cystic fibrosis (CF) aged ≤18 years in NSW and to assess rates of deficiency from 2007–2010. Methods A retrospective analysis of fat-soluble vitamin levels and PT in children aged ≤18 years who lived in NSW and attended any of the three paediatric CF centres from 2007–2010. An audit of demographic and clinical data during the first vitamin level measurement of the study period was performed. Results Deficiency of one or more fat-soluble vitamins was present in 240/530 children (45%) on their first vitamin test in the study period. The prevalence of vitamin D and E deficiency fell from 22.11% to 15.54% and 20.22% to 13.89%, respectively, from 2007–2010. The prevalence of vitamin A deficiency increased from 11.17% to 13.13%. Vitamin K was measured infrequently. Prolonged PT was common. Low vitamin levels were associated with poor clinical status. Discussion This is one of few studies of fat-soluble vitamin deficiency in children with CF in Australia. Testing of vitamin K-dependant factors needs consideration. Further studies are needed to continue monitoring rates of vitamin deficiency in the CF community.

Improving nutritional status in a pediatric cystic fibrosis center.

The nutritional status of patients with cystic fibrosis (CF) is strongly associated with pulmonary function, respiratory status and survival. Malnutrition could result from a discrepancy between energy needs and food intake while malabsorption results from pancreatic insufficiency which occurs in 85% of people with CF. A quality improvement (QI) project was designed to improve the nutritional status of patients with CF with low Body Mass Index (BMI) between 3 and 19 years of age. An algorithm was developed which included clinic-based assessments of patients' nutritional status and periodic assessment by a dietitian, social worker and/or psychologist during the project. Gastrostomy tube placement and feeding was offered as a last resort to improve caloric intake. 173 patients seen during January-June, 2010, were included in this project. They were classified into four BMI groups and data were collected quarterly through June, 2012. The project target population (BMI percentile ≤ 24) had a median BMI percentile at the start of the project of 11.8. At the end of the project median BMI percentile was 22 (46% improvement). Improving nutrition and BMI for patients with CF is achievable. There must be a motivated, multi-disciplinary team that includes patients and families. A patient-specific combination of interventions must be used. These interventions could be quite basic for patients with BMI percentile ≥ 25, yet more elaborate for patients with BMI percentile <25. Clinic-based algorithms such as ours can successfully improve the BMI percentile in patients with CF.

219 A review of hospital and hospital in the home (HiTH) services at a paediatric cystic fibrosis (CF) centre

Objectives: The Royal Children’s Hospital in Melbourne cares for approximately 250 patients with CF. Patients admitted to hospital may spend some of the admission in HiTH whilst others remain in hospital. Routine physiotherapy service in hospital includes twice daily supervised sessions on weekdays, and once daily on weekends. HiTH physiotherapy service includes once daily supervised session on weekdays only. This review aimed to examine HiTH utilisation and compare HiTH outcomes to hospitalisation in CF. Methods: A retrospective audit of patient records was performed covering a 24 month period. Results: 246 patient records were reviewed. 338 admissions occurred for 117 patients. 13 admissions involved inter-hospital transfer and were not included further leaving 375 admissions. 221 (59%) admissions were conducted solely in hospital and 154 (41%) included some period of HiTH (mean 9.9 days, range 3–174). When 16 admissions of excessive length (>20 days) were excluded, average length of stay was 12.6 days for hospital-only admissions, and 14.4 days for those including HiTH (p = 0.00). Hospital-only admissions had significantly lower FEV1 (% predicted) at admission (60.6% vs 70.7%, p = 0.00) but resulted in a greater absolute percentage increase in FEV1 from admission to discharge (15.7% vs 8.7%, p = 0.02). Conclusion: This review shows that CF admissions when they involved were longer and resulted in a smaller increase in FEV1. However, these patients had higher FEV1 at admission. With increasing reliance on home-based provision of hospital services it is important to ensure that these services are similarly effective, and beneficial in the short and long-term for patients.