Mobile health platform for self-management of pediatric cystic fibrosis: Impact on patient-centered care outcomes.

BACKGROUND: Previously, we adapted a mobile health platform (Genia) to the needs of patients and families in a pediatric CF center in the United States. In this feasibility study, we tested the impact of Genia on measures of patient-centered care. METHODS: In a one-group pre-post study with adolescents with CF and caregivers of children with CF, we tested Genia's effect over 6 months on patient satisfaction with chronic illness care (PACIC) and shared decision-making (CollaboRate). Feasibility and acceptability were assessed with exit interviews and app analytics. RESULTS: The intervention included 40 participants: 30 caregivers of children with CF age ≤14 years and 10 patients with CF age ≥15 years. The use of Genia was associated with increased satisfaction with care (p = 0.024), including delivery system and decision support (p = 0.017), goal setting (p = 0.006), and shared decision-making (p<0.001). The use of Genia was associated with nominal improvements in all QOL domains and symptom scales. The platform was feasible, with participants recording more than 4,400 observations (mean 84.2) and submitting 496 weekly reports (mean 13.8) and 70 quarterly reports (mean 1.8), and acceptable (95% retention rate). For participants, the most useful app feature was pre-visit reports (66.7%), and the top symptom trackers were those for cough (23.7%), appetite (21.1%), energy (18.4%), and medicines (18.4%). CONCLUSION: The use of Genia over 6 months was feasible, acceptable, and associated with improved measures of patient-centered care. Study results support wider use of Genia in clinical settings. Efficacy for clinical outcomes should be assessed in a randomized clinical trial.

Outcomes of children with cystic fibrosis screen positive, inconclusive diagnosis/CFTR related metabolic syndrome.

Background: Some infants undergoing newborn screening (NBS) tests have inconclusive sweat chloride test (SCT) results that lead to the designation of Cystic Fibrosis Screen Positive, Inconclusive Diagnosis/CFTR-related metabolic syndrome (CFSPID/CRMS). Some proportion of them transition to a CF diagnosis, but no predictive markers can stratify which are at risk for this transition. We report single-center outcomes of children with CRMS. Methods: We retrospectively identified all infants born in Alabama from 2008 through 2020 referred to our CF Center with an elevated immunoreactive trypsinogen level (IRT) associated with a cystic fibrosis transmembrane conductance regulator (CFTR) mutation (IRT+/DNA+) who had at least one SCT result documented. Infants were classified per established guidelines as Carrier, CRMS, or CF based on the IRT+/DNA+ and SCT results. The electronic health record was reviewed for follow-up visits until the children received a definitive diagnosis (to carrier or CF) according to current diagnostic guidelines for CF, or through the end of the 2020 year. Results: Of the 1,346 infants with IRT+ and at least 1 CFTR mutation identified (IRT+/DNA+), 63 (4.7%) were designated as CRMS. Of these infants, 12 (19.1%) transitioned to Carrier status (CRMS-Carrier), 40 (63.5%) of them remained CRMS status (CRMS-Persistent) and 11 (17.5%) of them transitioned to a diagnosis of CF (CRMS-CF). Of the 11 children in the CRMS-CF group, 4 (36%) had an initial SCT 30-39 mmol/L, 4 (36%) had an initial SCT 40-49 mmol/L and 3 (27%) had an initial SCT 50-59 mmol/L. These children also had higher initial sweat tests and greater yearly increases in sweat chloride values than others with CRMS. We found that in comparison to children in the CRMS-P group, a greater proportion of children in the CRMS-CF group cultured bacteria like methicillin-resistant Staphylococcus aureus, Stenotrophomonas maltophilia, and Pseudomonas aeruginosa, had smaller weight-for-height percentiles and remained smaller over time despite slightly greater growth. Conclusion: Infants with an inconclusive diagnosis of CF should continue to receive annual care and management given their potential risk of transition to CF. Further research is needed to assess whether certain phenotypic patterns, clinical symptoms, diagnostic tests or biomarkers could better stratify these children.

A Mobile Health Platform for Self-Management of Pediatric Cystic Fibrosis: Qualitative Study of Adaptation to Stakeholder Needs and Integration in Clinical Settings.

BACKGROUND: Cystic fibrosis (CF) is an inherited chronic condition that requires extensive daily care and quarterly clinic visits with a multidisciplinary care team. The limited exchange of information outside of the quarterly clinic visits impedes optimal disease self-management, patient engagement, and shared decision making. OBJECTIVE: The aim of this study is to adapt a mobile health (mHealth) app originally developed in Sweden to the needs of patients, families, and health care providers in a CF center in the United States and to test it as a platform for sharing patient-generated health data with the CF health care team. METHODS: Focus groups with health care providers of patients with CF, adolescents with CF, and caregivers of children with CF were conducted to determine what modifications were necessary. Focus group data were analyzed using a thematic analysis, and emergent themes were ranked according to desirability and technical feasibility. The mHealth platform was then modified to meet the identified needs and preferences, and the flow of patient-generated health data to a secure Research Electronic Data Capture database was tested. Protocols for data management and clinical follow-up were also developed. RESULTS: A total of 5 focus groups with 21 participants were conducted. Recommended modifications pertained to all functionalities of the mHealth platform, including tracking of symptoms, treatments, and activities of daily care; creating and organizing medication lists and setting up reminders; generating reports for the health care team; language and presentation; sharing and privacy; and settings and accounts. Overall, health care providers recommended changes to align the mHealth platform with US standards of care, people with CF and their caregivers requested more tracking functionalities, and both groups suggested the inclusion of a mental health tracker as well as more detailed response options and precise language. Beta testers of the modified platform reported issues related to translatability to US environment and various bugs. CONCLUSIONS: This study demonstrated the importance of identifying the needs and preferences of target users and stakeholders before adopting existing mHealth solutions. All relevant perspectives, including those of clinicians, patients, and caregivers, should be thoroughly considered to meet both end users' needs and evidence-based practice recommendations.

Tobacco smoke exposure in pediatric cystic fibrosis: A qualitative study of clinician and caregiver perspectives on smoking cessation.

OBJECTIVE: Tobacco smoke exposure has negative impacts on the lung health of children with cystic fibrosis (CF), yet evidence-based strategies for smoking cessation have not been tested with or tailored to CF caregivers. This qualitative study identified barriers and facilitators of smoking cessation in this population and outlined potential interventional approaches. METHODS: We conducted semi-structured interviews with CF familial caregivers who were current or former smokers, and with members of the CF care team. We asked about experiences, practices, and prerequisites for a successful program. Interviews were recorded, transcribed verbatim, and coded by two investigators. Analysis used a thematic approach guided by the PRECEDE model, which identifies predisposing (intrapersonal), reinforcing (interpersonal), and enabling (structural) factors relevant to health behaviors and programs. RESULTS: Seventeen interviews were conducted-eight with familial caregivers and nine with CF team members. Whereas caregivers provided greater insight into internal difficulties and motivators to quit smoking, clinicians offered more extensive input on barriers and solutions related to the clinical environment. Based on study recommendations, a successful tobacco cessation program should include (a) family education about the harms of smoke exposure for children with CF; (b) screening for exposure, ideally with biochemical verification; (c) access to trained tobacco counselors; (d) affordable pharmacotherapy; and (e) outpatient follow-up of those undergoing tobacco treatment. CONCLUSION: This qualitative study revealed intrapersonal, interpersonal, and structural barriers to eliminating tobacco smoke exposure in children with CF, outlined opportunities to address these barriers, and made recommendations for a comprehensive tobacco cessation strategy.

Prevention of severe respiratory syncytial virus infection in the pediatric population in Mexico: position of a group of experts.

Respiratory syncytial virus (RSV) is the agent that causes more hospitalizations and deaths due to lower acute respiratory infection. Its distribution is widespread, and almost every child has been infected by the age of two years. Different risk populations have been identified: preterm newborns (NB), children with congenital heart disease, bronchopulmonary dysplasia, Down syndrome, cystic fibrosis, asthmatics, neuromuscular diseases, among others. However, preterm NBs, children with congenital heart disease or bronchopulmonary dysplasia show higher rates of hospitalization and death from RSV. In the late 90s, monoclonal antibodies against RSV were developed, with demonstrated efficacy and safety for the prevention of RSV hospitalizations in these populations. Currently, the American Academy of Pediatrics recommends this therapy for the prevention of severe infection in the population at higher risk. Economic evaluations have been conducted to determine the effectiveness of immunization, resulting favorable for palivizumab. Immunization in Mexico has resulted cost-effective in NBs under 32 gestation weeks. Mexican authorities should discuss the inclusion of palivizumab in their clinical guidelines.

El virus sincicial respiratorio (VSR) es el agente que ocasiona más hospitalizaciones y muertes por infección aguda de vías respiratorias bajas. La mayoría de los niños ya han sido infectados a los 2 años de edad. Se han identificado diferentes poblaciones de riesgo: recién nacidos pretérmino y niños con cardiopatía congénita, displasia broncopulmonar, síndrome de Down, fibrosis quística, asma y enfermedades neuromusculares, entre otras. Sin embargo, las tasas de hospitalización y de muerte por VSR son más altas en los recién nacidos pretérmino y en los niños con cardiopatía congénita o displasia broncopulmonar. A finales de los años 90 se desarrollaron anticuerpos monoclonales contra el VSR, los cuales demostraron ser eficaces y seguros en la prevención de hospitalizaciones por VSR en estas poblaciones. Actualmente, la American Academy of Pediatrics los recomienda para la prevención de la infección grave en la población de mayor riesgo. Se ha recurrido a evaluaciones económicas para determinar la efectividad de la inmunización, las cuales han sido favorables para el palivizumab. En México se ha demostrado que la inmunización es costo-efectiva en los recién nacidos menores de 32 semanas de gestación. Las autoridades mexicanas deben discutir la inclusión del palivizumab en sus guías de práctica clínica.

Tobacco smoke exposure and socioeconomic factors are independent predictors of pulmonary decline in pediatric cystic fibrosis.

BACKGROUND: Pulmonary decline in CF is heterogeneous, with socio-environmental factors contributing to this variability. Few studies have attempted to disentangle the effects of tobacco smoke exposure and socioeconomic factors on lung function deterioration in pediatric CF. The current study evaluates their contributions longitudinally across the entire U.S. CF care network population. METHODS: Data from the CF Foundation Patient Registry were obtained on all individuals who at the end of 2016 were 6-18 years old. Lung function measures (ppFEV1) for each person were calculated at each attained age. Multivariable analyses used mixed modeling to assess the impact of smoke exposure and socioeconomic factors on initial lung function and change over time. RESULTS: The sample included 10,895 individuals contributing 65,581 person years. At age 6, ppFEV1 of smoke-exposed children was 4.7% lower than among unexposed. The deficit persisted through age 18. In adjusted mixed models, smoke exposure and socioeconomic factors had independent, additive associations with lung function. Median ppFEV1 declined 2.4% with smoke exposure, 4.9% with lower paternal education, 0.3% with public insurance, and increased 0.2% with each $10,000 annual household income. The effect of smoke exposure on ppFEV1 was larger in disadvantaged children compared to privileged counterparts (3.2% vs 1.2%). CONCLUSIONS: Smoke exposure and socioeconomic factors are independent risk factors for decreased ppFEV1 in pediatric CF. Smoking cessation strategies should be emphasized at the time of CF diagnosis and reiterated during infancy and early childhood. Interventions may be prioritized in disadvantaged families, where the exposure has a disproportionately large effect.

Area Deprivation as a Risk Factor for Methicillin-resistant Staphylococcus aureus Infection in Pediatric Cystic Fibrosis.

BACKGROUND: In US cystic fibrosis (CF) patients, methicillin-resistant Staphylococcus aureus (MRSA) rates have tripled in the past 2 decades. Known clinical risk factors include exposure to a healthcare setting, Pseudomonas aeruginosa and CF-related diabetes. Area-level socio-environmental exposures have not been evaluated. We explored the association of area-level deprivation with MRSA prevalence in a pediatric CF Center in the Southeastern United States. METHODS: Patients' residential addresses were geocoded and linked to a composite Area Deprivation Index and Rural-Urban Commuting Area scores. The association of MRSA with Area Deprivation Index and Rural-Urban Commuting Area scores was evaluated using logistic regression with robust standard errors adjusted for sociodemographic covariates (age, sex, race, mother's and father's education and household income), clinical risk factors (P. aeruginosa, CF-related diabetes, hospitalizations and number of clinic visits) and clustering. RESULTS: The study included all pediatric patients (N = 231; mean age 12) at a single CF Center. MRSA was present in 44% of subjects. Higher area-level deprivation was correlated with rural residence, lack of parental college education and lower household income (P < 0.001 for each). In a multiple regression model fully adjusted for patient-level sociodemographic covariates, clinical risk factors and clustering, neighborhood deprivation was associated with more than 2-fold increase in the odds of having MRSA [OR 2.26 (1.14-4.45), P < 0.05]. CONCLUSIONS: Neighborhood deprivation is a risk factor for MRSA in pediatric CF, doubling the odds of infection. Community-level socioeconomic risk factors should be considered when developing prevention strategies and treatment plans for MRSA infection in pediatric patients with CF.

Objective Versus Self-Reported Adherence to Airway Clearance Therapy in Cystic Fibrosis.

BACKGROUND: Historically, studies of adherence to airway clearance therapy in cystic fibrosis (CF) have relied on self-reporting. We compared self-reported airway clearance therapy adherence to actual usage data from home high-frequency chest wall compressions (HFCWC) vests and identified factors associated with overestimation of adherence in self-reports. METHODS: Pediatric patients who perform airway clearance therapy with a HFCWC vest were eligible to participate. Objective adherence data were obtained from the HFCWC device, which records cumulative utilization time. Two readings at least 5 weeks apart were collected. Objective adherence was recorded as a ratio of mean-to-prescribed daily use (%). Self-reported adherence data were collected with a caregiver survey at enrollment. Adherence rates were categorized as low (< 35% of prescribed), moderate (36-79% of prescribed), and high (≥ 80% of prescribed). An overestimation was present when self-reported adherence was at least one category higher than objective adherence. RESULTS: In the final sample (N = 110), mean adherence by usage data was 61%. Only 35% of subjects (n = 38) were highly adherent, and 28% (n = 31) were low adherent. In contrast, 65% of subjects (n = 72) reported high adherence and only 8% (n = 9) reported low adherence (P < .001). Nearly half of self-reports (46%) overestimated adherence. In a multiple regression analysis, overestimation was associated with multiple airway clearance therapy locations (odds ratio 7.13, 95% CI 1.16-43.72, P = .034) and prescribed daily use ≥ 60 min (odds ratio 3.85, 95% CI 1.08-13.76, P < .038). Among subjects with prescribed daily airway clearance therapy ≥ 60 min, the odds of overestimating adherence increased 3-fold (odds ratio 3.04, 95% CI 1.17-7.87, P = .02) in a lower-income (< $50,000/y) environment. CONCLUSIONS: Self-reports overestimated actual adherence to airway clearance therapy, and the overestimation increased with treatment occurring in multiple households and prescribed therapy duration. Among participants with prescribed airway clearance therapy ≥ 60 min, overestimation increased with lower income. Objective measures of adherence are needed, particularly for lower-income children and those receiving treatments in multiple locations.

Risk stratification model to detect early pulmonary disease in infants with cystic fibrosis diagnosed by newborn screening.

OBJECTIVE: The clinical benefit of newborn screening (NBS) for cystic fibrosis (CF) has been primarily nutritional, with less overt respiratory impact. Identification of risk factors for infant CF lung disease could facilitate targeted interventions to improve pulmonary outcomes. METHODS: This retrospective study evaluated socioeconomic information, clinical data, and results from routine infant pulmonary function testing (iPFT) of infants diagnosed with CF through NBS (N = 43) at a single CF center over a 4-year period (2008-2012). A five-item composite clinical score was developed and combined with socioeconomic indicators to facilitate identification of CF infants at increased risk of early-onset respiratory impairment. RESULTS: Paternal education was positively associated with lung function (P = 0.02). Clinical score <7 (on a scale of 0-10) predicted diminished pulmonary measure (P < 0.005). Retrospective risk stratification by clinical score and paternal education identified CF infants at low, intermediate, or high risk of pulmonary disease. Forced expiratory volume (FEV0.5 %, mean ± SD) averaged 115 ± 19% in the low-risk group, 97 ± 17% in the intermediate-risk group, and 90 ± 8% in the high-risk group (P < 0.005). Results were similar for mid-expiratory flows (FEF25-75 %). Multiple regression analysis confirmed the predictive value of this risk stratification model of CF infant pulmonary health. CONCLUSION: We combined socioeconomic and clinical data to risk-stratify CF infants for early-onset lung disease as quantified by iPFT. Our model showed significant differences in infant pulmonary function across risk groups. The developed tool offers an easily available, inexpensive, and non-invasive way to assess risk of respiratory decline in CF infants and identify those meriting targeted therapeutic attention. Pediatr Pulmonol. 2016;51:1168-1176. © 2016 Wiley Periodicals, Inc.

Implementación de atención multidisciplinaria del paciente con fibrosis quística / Implementation of multidisciplinary care for patients with cystic fibrosis

Cystic fibrosis (CF) is the most common recessive genetic disease in the Caucasian population. Unanimously, all CF care guidelines in developed countries require that management be provided by a network of specialized CF care centers that cover the entire population of patients, pediatric and adult. The establishment of such centers of excellence requires a level of experience that is achieved only by the conformation of a multidisciplinary team of trained and experienced health professionals, caring for a sufficient number (critical mass) of patients to achieve the best clinical outcomes and survival rates at a lower cost which is achieved by distributing health resources to this small network of centers of excellence. The experience of the health team of the Hospital San Borja Arriaran participating in the project promoted by the COA/UAB CF Center in Birmingham, Alabama in the United States shows that it is possible to transfer knowledge and quality management know-how, creating a cutting-edge, world-class, “CF Center” which performs a multidisciplinary care management of high quality, that is finally reflected in significant improvements in nutritional parameters and lung function of the patients.

La fibrosis quística (FQ) es la enfermedad genética recesiva más común en la población caucásica. De manera unánime todas las normativas de cuidado de FQ de países desarrollados requieren que el manejo sea dado por una red de centros especializados de cuidado en FQ que cubra toda la población de pacientes, pediátricos y adultos. El establecimiento de dichos centros de excelencia requiere de un nivel de experiencia que se logra solo por la constitución de un equipo multidisciplinario entrenado y experimentado de profesionales de la salud, a cargo de un número suficiente (masa crítica) de pacientes para lograr los mejores resultados de sobrevida, a un costo menor, lo cual se logra al distribuir los recursos de salud a un número reducido de centros de excelencia. La experiencia del equipo de salud del Hospital San Borja Arriarán en el proyecto impulsado por el COA/UAB CF Center de Birmingham, Alabama de Estados Unidos, demuestra que es posible la transferencia de conocimientos y de gestión de calidad, formando un “Centro FQ” de vanguardia y de categoría mundial, que realiza un manejo multidisciplinario de primera calidad, reflejado en la mejoría nutricional y de función pulmonar.

Adherence to airway clearance therapy in pediatric cystic fibrosis: Socioeconomic factors and respiratory outcomes.

OBJECTIVES: The evidence linking socioeconomic status (SES) and adherence in cystic fibrosis (CF) is inconclusive and focused on medication uptake. We examined associations between SES, adherence to airway clearance therapy (ACT), and CF respiratory outcomes. STUDY DESIGN: Socioeconomic, clinical, and adherence data of CF patients (N = 110) at a single CF Center were evaluated in this cross-sectional observational study. SES was operationalized as maternal and paternal education and household income. Adherence to ACT was measured with utilization data from the high-frequency chest wall oscillation (HFCWO) device over 4-6 weeks. Statistical modeling was used to test three hypotheses: (H1) Higher SES is associated with higher ACT adherence; (H2) Higher SES is associated with better respiratory outcomes; and (H3) ACT adherence mediates the relationship between SES and respiratory outcomes. RESULTS: In multinomial logistic regression, maternal college education, annual income >$50,000, and more adults in the household were independently related to better adherence (P < 0.05). Paternal college education, income >$100,000, and lack of exposure to smoking were independently related to higher lung function (P < 0.05). Current adherence to ACT with HFCWO was not associated with lung function over 12 months. CONCLUSIONS: SES is associated both with ACT adherence and respiratory outcomes in pediatric CF patients. However, the link between SES and respiratory outcomes in this study was not mediated by adherence to ACT with HFCWO. These data emphasize the importance of socioeconomic resources and household environment for CF health. Family socio-demographic profiles can help identify patients at increased risk for ACT nonadherence.

Sustained improvement in nutritional outcomes at two paediatric cystic fibrosis centres after quality improvement collaboratives.

OBJECTIVE: To describe the characteristics of sustained improved nutritional outcomes through the use of quality improvement (QI) methodology. DESIGN: Retrospective analysis of a QI intervention in two institutions, implemented as part of larger national collaboratives. SETTING: Paediatric cystic fibrosis (CF) programmes in academic centres in Alabama and Illinois. PARTICIPANTS: All paediatric patients enrolled in the CF Foundation (CFF) Patient Data Registry were included. INTERVENTIONS: Improved and sustained nutrition outcomes occurred through implementation of the CFF practice guidelines for CF nutrition management via care delivery processes, nutritional interventions, team engagement and data display. MEASUREMENT: Mean body mass index (BMI) percentile, percentage of patients less than 50th percentile and percentage less than 10th percentile for all patients aged 2-20 years were tracked through run charts and statistical process control charts. Mann-Whitney U and χ(2) tests were used to determine significance between each centre and national outcomes. RESULTS: Each centre achieved rapid improvement in mean BMI percentile in patients, one centre rising from the 40th percentile in 2001 to the 49th percentile in 2003, the other rising from the 37th percentile in 2003 to the 45th percentile in 2004. These centres have also maintained improved nutritional outcomes, so that they were at the 60th and 55th percentiles, respectively, in 2011. Sustained improvement was accomplished through QI methodology, use of data as a driver for improvement and a change in culture. CONCLUSIONS: Participation in collaboratives led to improved nutrition outcomes while a strong culture of QI facilitated sustained improvement.

Baseline characteristics and factors associated with nutritional and pulmonary status at enrollment in the cystic fibrosis EPIC observational cohort.

SUMMARY BACKGROUND: The EPIC Observational Study is an ongoing prospective cohort study investigating risk factors for and clinical outcomes associated with early Pseudomonas aeruginosa (Pa) acquisition in young children with cystic fibrosis (CF). OBJECTIVES AND HYPOTHESIS: To describe the baseline characteristics of the cohort and evaluate associations between potential risk factors and nutritional and respiratory characteristics at enrollment. We hypothesized that distinct demographic and environmental risk factors could be identified for poorer nutritional status and lung function at enrollment. METHODS: During 2004-2006, 1,700 children with CF were enrolled at 59 US CF centers. Children

Improving the quality of care for patients with cystic fibrosis.

PURPOSE OF REVIEW: Improvement in cystic fibrosis (CF) outcomes over the past 50 years has been dramatic. This article describes the factors that have contributed to the recent acceleration in this improvement and the important role of the Cystic Fibrosis Foundation. RECENT FINDINGS: Initiatives to improve CF care over the past decade include the refinement of a sophisticated patient registry that allows a comparison of center processes and outcomes; development of evidence-based and consensus-based guidelines regarding standards of care; cultural transformation, including the training of care center teams in a systems-oriented approach to quality improvement; data transparency; and encouragement of a patient-centered and family-centered orientation. Cystic Fibrosis Foundation initiatives have promoted an increase in the consistent provision of evidence-based care and the promulgation of innovative, proactive therapeutic approaches. Unfortunately, so far most of these accomplishments have not been documented in peer-reviewed research articles, but rather in platform presentations at national meetings, meeting abstracts, data reports to center directors, and published reports from individual centers. SUMMARY: While several new medications for CF lung disease have been introduced in the last decade, improvements in outcomes have been largely due to refinements in the delivery of care. At this point, the most efficient and reliably effective technique for promoting further improvement still needs to be identified. Recent discussions have centered on attempts at new innovative approaches that utilize more selective teaching of relevant systems-based quality improvement methods in the specific CF environment.

Cystic fibrosis care in Chile.

PURPOSE OF REVIEW: Cystic fibrosis (CF) has been underdiagnosed and undertreated for many years in Latin American countries, including Chile. This article describes the evolution of CF care in view of recent reforms in healthcare delivery in Chile, and the opportunities that exist to improve outcomes. RECENT FINDINGS: The characteristics of the population of CF patients are described using recently collected data. Unfortunately, current data do not allow an accurate assessment of CF care in Chile. The situation is similar elsewhere in Latin America. Further, we describe the obstacles to achieving optimum CF care, among them lack of expertise among healthcare professionals, dispersion of patients between private and public health systems, and limited laboratory infrastructure despite significant economic growth and improvement in national health indices. We discuss different avenues to enhance CF care in Chile and Latin America. SUMMARY: Centralization and organization of CF care within centers of excellence employing 'best practices' and engaging in quality-improvement initiatives, and the creation of national data registries represent important steps to improve survival, diminish morbidity, and allow more extensive participation of Latin American CF patients in multicenter clinical research.