Cystic fibrosis related diabetes is not associated with maximal aerobic exercise capacity in cystic fibrosis: a cross-sectional analysis of an international multicenter trial.

Background: Previous studies have reported differences in aerobic exercise capacity, expressed as peak oxygen uptake (VO), between people with and without cystic fibrosis (CF) related diabetes (CFRD). However, none of the studies controlled for the potential influence of physical activity on VO. We investigated associations between CFRD and VO following rigorous control for confounders including objectively measured physical activity.

Effects of a Partially Supervised Conditioning Program in Cystic Fibrosis: An International Multicenter, Randomized Controlled Trial (ACTIVATE-CF).

The long-term effects of vigorous physical activity (PA) on lung function in cystic fibrosis are unclear. To evaluate effects of a 12-month partially supervised PA intervention using motivational feedback. In a parallel-arm multicenter randomized controlled trial (ACTIVATE-CF), relatively inactive patients aged at least 12 years were randomly assigned (1:1 ratio) to an intervention group or control group.

Prevalence and clinical associations of Staphylococcus aureus small-colony variant respiratory infection in children with cystic fibrosis (SCVSA): a multicentre, observational study.

Background: Staphylococcus aureus is the bacterium cultured most often from respiratory secretions of people with cystic fibrosis. Both meticillin-susceptible S aureus and meticillin-resistant S aureus (MRSA) can adapt to form slow-growing, antibiotic-resistant isolates known as small-colony variants that are not routinely identified by clinical laboratories. We aimed to determine the prevalence and clinical significance of S aureus small-colony variants and their subtypes among children with cystic fibrosis.

Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial.

Rationale: Cystic fibrosis (CF) is characterized by dietary antioxidant deficiencies, which may contribute to an oxidant-antioxidant imbalance and oxidative stress.

Objectives: Evaluate the effects of an oral antioxidant-enriched multivitamin supplement on antioxidant concentrations, markers of inflammation and oxidative stress, and clinical outcomes.

Methods: In this investigator-initiated, multicenter, randomized, double-blind, controlled trial, 73 pancreatic-insufficient subjects with CF 10 years of age and older with an FEV between 40% and 100% predicted were randomized to 16 weeks of an antioxidant-enriched multivitamin or control multivitamin without antioxidant enrichment.

Cost(s) of caring for patients with cystic fibrosis.

Purpose Of Review: Cystic fibrosis (CF) has received a lot of attention in the past few years because of increased longevity and the emergence of ground-breaking new drugs targeting the molecular and cellular defects, making a huge clinical difference, and - not incidentally - carrying massive price tags. The prices of these new drugs make the question of overall costs of CF care highly relevant.

Recent Findings: This article reviews recent developments in CF science and treatment, and highlights areas that contribute to costs of CF care, emphasizing how these costs have increased.

Effects of a partially supervised conditioning programme in cystic fibrosis: an international multi-centre randomised controlled trial (ACTIVATE-CF): study protocol.

Background: Physical activity (PA) and exercise have become an accepted and valued component of cystic fibrosis (CF) care. Regular PA and exercise can positively impact pulmonary function, improve physical fitness, and enhance health-related quality of life (HRQoL). However, motivating people to be more active is challenging.

Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis.

Background: This study aimed to describe and compare the sexual and reproductive health (SRH) care utilization among young women with cystic fibrosis (CF) with the general United States (U.S.) population.

Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis.

Background: The prevalence of general and disease-specific sexual and reproductive health (SRH) concerns is unknown in the United States (U.S.) female CF population.

Provider Attitudes and Practices toward Sexual and Reproductive Health Care for Young Women with Cystic Fibrosis.

Study Objective: To investigate the attitudes and practices of cystic fibrosis (CF) providers toward sexual and reproductive health (SRH) care in young women with CF.

Design, Setting, Participants, And Interventions: Adult and pediatric US CF providers were sent an online survey exploring their attitudes toward SRH importance, SRH care practices, and barriers/facilitators to SRH care in adolescent and/or young adult women. Descriptive statistics and logistic regression were used to analyze results.

Provider and Patient Attitudes Regarding Sexual Health in Young Women With Cystic Fibrosis.

Objective: To explore the attitudes, preferences, and experiences of patients with cystic fibrosis (CF) and CF providers toward sexual and reproductive health (SRH) care for young women with CF.

Methods: Young women with CF aged 18 to 30 years from a US CF care center and pediatric and adult CF program directors from a national sample participated in qualitative interviews investigating their experiences regarding SRH care and their attitudes and preferences toward SRH care provision in the CF setting. Interviews were audio-recorded, transcribed, and coded by using a thematic analysis approach.

Cystic fibrosis program directors' attitudes toward sexual and reproductive health in young women with CF.

Introduction: Advancements in therapy have led to dramatic increases in the life expectancy of patients with cystic fibrosis (CF). As survival improves, young women with CF will have expectations for their sexual and reproductive health (SRH) futures similar to their counterparts without CF. As they face unique CF-specific SRH concerns, they may rely on CF care providers for disease-specific SRH care provision.

How does heart rate recovery after sub-maximal exercise correlate with maximal exercise testing in children with CF?

Background: Disease progression in cystic fibrosis (CF) is marked by worsening exercise tolerance. Further, maximal exercise capacity (VO2 peak) correlates with survival in CF, but maximal tests are uncomfortable and resource-intensive. A three-minute step test (STEP) has been validated in CF.

Parent and child perceptions of a self-regulated, home-based exercise program for children with cystic fibrosis.

Background: Despite recognized benefits, many children with cystic fibrosis (CF) do not consistently participate in physical activities. There is little empirical literature regarding the feelings and attitudes of children with CF toward exercise programs, parental roles in exercise, or factors influencing exercise experiences during research participation.

Objectives: The aim of this study is to describe the exercise experiences of children with CF and their parents during participation in a 6-month program of self-regulated, home-based exercise.

Interval exercise training in cystic fibrosis -- effects on exercise capacity in severely affected adults.

The aims of the present study were to investigate the effects of IT on lung function power (P) and oxygen uptake (VO2) at peak performance (peak) and ventilatory anaerobic threshold (VAT) in CF patients who were unable to participate in a standard exercise program (SEP) and to compare these IT responses with corresponding effects in CF patients performing SEP. 20 patients (FEV1 25.5 ± 7.

Exercise intensity self-regulation using the OMNI scale in children with cystic fibrosis.

Prescribing exercise at intensities that improve fitness is difficult in children with cystic fibrosis (CF) due to ventilatory limitations and fluctuating health status. Our aim was to determine if children with CF could regulate the intensity of cycle ergometer and treadmill exercise using target ratings of perceived exertion (RPE) derived from the Children's OMNI Scale. We examined prescription congruence (similar oxygen consumption [VO₂] and heart rate [HR] for target RPE) and intensity discrimination (different VO₂ and HR for different RPEs), from cycle to cycle and cycle to treadmill.

The ease of breathing test tracks clinical changes in cystic fibrosis.

Introduction: The EOB is a measure of dyspnea that correlates with CF pulmonary function and exercise tolerance, but has not been shown to track clinical changes.

Methods: The EOB was administered before and after treatment for twelve episodes of exacerbations in eleven pediatric CF subjects. Each subject performed the EOB Sustained Phonation and Single Breath Counting measures before and after treatment both at rest and after exercise.

Randomized trial of biofilm testing to select antibiotics for cystic fibrosis airway infection.

Rationale: In cystic fibrosis (CF), conventional antibiotic susceptibility results correlate poorly with clinical outcomes. We hypothesized that biofilm testing would more accurately reflect the susceptibilities of bacteria infecting CF airways.

Methods: A multicenter randomized pilot trial was conducted to assess the efficacy and safety of using biofilm susceptibility testing of Pseudomonas aeruginosa sputum isolates to guide antibiotic regimens for chronic airway infections in clinically stable adolescent and adult CF patients.

Effects of an exercise program in children with cystic fibrosis: are there differences between females and males?

Objective: To investigate the adaptive responses of an in-patient exercise program in children with cystic fibrosis (CF) and evaluate the effects of sex.

Study Design: In total, 158 female and 186 male subjects with CF (age, 12 to 43 years) were studied during a 6-week rehabilitation course. A maximal incremental cycling test was used to determine exercise capacity and responses after 6 weeks of exercise training.

Motor performance is better than normal in preschool children with cystic fibrosis.

The aim of the present study was to assess the motor performance in preschool children with a reliable and valid test battery developed to identify motor dysfunction and normal motor development in children aged from 4 to 6 years. Several aspects of motor performance were examined in 29 preschool children with cystic fibrosis (CF) age range 4-6 years (mean 5.2 +/- 0.

Update on the role of exercise in cystic fibrosis.

Purpose Of Review: The role of exercise in patients with cystic fibrosis has been recognized for as long as cystic fibrosis has been identified as a clinical syndrome, as exercise intolerance has always been a hallmark of disease progression.

Recent Findings: Work published in the past year has shed new light on several aspects of this field, including physiologic responses to exercise, responses to exercise programs, and the epidemiologic and prognostic implications of activity and exercise testing.

Summary: Barker's work is perhaps the most compelling, as it highlights the overwhelming interest and belief in the utility of exercise testing and prescription among cystic fibrosis physicians and the contrasting paucity of programs that test their patients and prescribe exercise for them.

Strength vs aerobic training in children with cystic fibrosis: a randomized controlled trial.

Study Objective: Exercise has the potential to improve the ability of a patient with cystic fibrosis (CF) to cope with the physical demands of everyday life, and may improve prognosis. The purpose of this study was to compare the effects of a home-based, semi-supervised, upper-body strength-training regimen with a similarly structured aerobic training regimen.

Design: Data were collected during a 1-year randomized clinical trial.

Liver transplantation in children with cystic fibrosis: a long-term longitudinal review of a single center's experience.

Background: Improved long-term survival in cystic fibrosis (CF) has led to an increased incidence of extrapulmonary complications of this disease. Of these, end-stage liver disease is a significant cause of morbidity and mortality with liver transplantation being the only effective therapy.

Methods: Records of all CF pediatric liver transplant recipients were reviewed.