Objective Measures of Vest Therapy Adherence Among Pediatric Subjects With Cystic Fibrosis.

BACKGROUND: Airway clearance techniques (ACTs), including high-frequency chest wall oscillation (vest therapy), are important for maintaining lung function for patients with cystic fibrosis, but daily completion of ACTs is time-consuming and cumbersome. Patient adherence is a persistent challenge, and adherence data are largely patient reported, which may reduce accuracy. To provide definitive adherence data, this study utilized a Bluetooth-enabled vest therapy system to remotely collect objective adherence data from a cohort of pediatric subjects. METHODS: Vest usage data were collected over a 12-month period and compared to each subject vest prescription. Because vest prescriptions are multifaceted, we developed metrics to examine adherence to the various facets (eg, daily treatment, treatments per day, treatment duration, frequency settings, and frequency and pressure settings combined) as well as an overall adherence metric. RESULTS: Among the 73 enrolled subjects, 62 (50% male, age range 2-19 y) completed the study. Among adolescent subjects age 13-19 y, average adherence to daily vest therapy (44.0%) was significantly lower than that among subjects 0-6 y old (77.8%) and subjects 7-12 y old (89.5%). As more prescribed therapy components were combined into a single adherence metric, all age groups declined in mean adherence rates, with overall adherence a decreasing function of age. CONCLUSIONS: Employing new technology to remotely collect vest usage data allows for a granular examination of vest therapy adherence. While maintaining high levels of treatment adherence becomes increasingly difficult as children age, we also found substantial reductions in adherence rates among all age groups when more complex aspects of therapy prescriptions, such as frequency and pressure settings, were examined. These data illustrate areas providers and care teams can focus on to improve patient adherence to vest prescriptions.

Yoga Therapy in Children with Cystic Fibrosis Decreases Immediate Anxiety and Joint Pain.

This study was designed to determine whether yoga might alleviate symptoms of pain, sleep disturbance, anxiety, and depression in children with cystic fibrosis (CF). CF is the most common genetic, life-limiting chronic disease among Caucasian populations. It primarily affects the lungs but also many other secretory organs and consequently leads to significant morbidities. Research has shown that children with CF have significantly increased depression, anxiety, and pain compared to their healthy counterparts. Subjects participated in six one-on-one sessions over a 10-week period with a certified instructor who designed each yoga practice based on a preestablished list of 30 yoga asanas. Questionnaires evaluating pain, sleep disturbance, sustained anxiety, immediate anxiety, and depression were administered. Differences between premeasures and postmeasures were evaluated using a two-sided test. Twenty subjects were assessed (12 females/8 males), median age of 11 (7-20) years. Mean immediate anxiety scores decreased (before session to after session 29 to 23.6, respectively, p < 0.001). Joint pain improved (3.25 to 3.65, p = 0.028). CFQ-R emotion subscale improved from 79.2 to 85 (p = 0.073), and the respiratory subscale improved from 66.7 to 79.2 (p = 0.076). Other results were less notable. We conclude that yoga may reduce immediate anxiety and joint pain in patients with CF.

Bordetella bronchiseptica in a pediatric Cystic Fibrosis center.

BACKGROUND: Bordetella bronchiseptica is a common pathogenic or colonizing organism of domestic mammals. In dogs, it causes an infectious tracheobronchitis known as Kennel Cough. Human infections are unusual and almost exclusively described in immunocompromised patients who have had contact with a known animal reservoir. It is rarely reported in Cystic Fibrosis (CF), possibly hampered by low recovery from culture and organism misidentification. We describe the incidence and characteristics of B. bronchiseptica in our CF population. METHODS: A retrospective cohort study was conducted of our center's CF patient population. Patients were included if they had B. bronchiseptica isolated on one or more occasion. RESULTS: Seven children with CF isolated B. bronchiseptica on 23 occasions, frequently associated with the symptoms of a pulmonary exacerbation. Four patients required hospitalization. CONCLUSION: These results suggest that B. bronchiseptica may be more common than previously reported and may play a potential pathogenic role in CF.