Exploring Novel Interventions to Improve Adherence in Children With Cystic Fibrosis

This is a randomized controlled trial to assess the impact of home lung function monitoring and medication reminders on adherence, clinical outcomes and quality of life in children with cystic fibrosis (CF) who are between 10 and 21 years of age.

Participants will be randomly assigned to either an intervention group or a control group. Those in the intervention group will receive a personal spirometer device that provides medication reminders and allows for lung function monitoring at home. The control group will receive the current standard of care. All participants will be aware that their adherence is being monitored over the course of the study using prescription refill data. Changes in lung function, body mass index and rate of hospitalization will be used as measures of clinical outcome. Responses to age-appropriate well-validated patient questionnaires will be used to assess the impact of the investigators interventions on quality of life and perceptions of treatment burden.

Since this device has never been previously studied in this patient population, the investigators would like to do a pilot study with 5 patients (3 in the intervention group and 2 in the control group) over a 3-month period to determine feasibility. This will also help us with the investigator power calculations and ultimately deciding the number of participants that will be needed for the larger study.

Inclusion criteria

• Age 10-21 years
• Confirmed diagnosis of cystic fibrosis (CF) either by a sweat chloride ≥ 60mEq/L or the presence of two disease-causing mutations
• Patients must be clinically stable with at least 1 month from their last hospitalization or use of oral antibiotics for a pulmonary exacerbation
• Signed informed consent from the patient and/or from the parent/legal guardian, if younger than 18 years.

Exclusion criteria

• Age less than 10 years or greater than 21 years
• Clinically unstable