Cystic fibrosis Nutritional, Metabolic, Endocrine Cystic fibrosis
Conditions
Interventions
Participants will complete two questionnaires at the beginning and end of the study. The first asks about the medications and treatments currently taken and any reasons they may have difficulty in tak
Sponsors
Leeds Teaching Hospitals Trust (UK)
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. Patients with a diagnosis of CF, attending Leeds Adult CF unit for their complete care 2. Male or female patients age 16 - 60 years old 3. Consecutive volunteers recruited at time of clinical stability [end of in-patient treatment or at out-patient clinic] 4. Patient must be prescribed a minimum of three specified medications (see below) for at least 6 months prior to signing the informed consent form: azithromycin hypertonic saline, TOBI®, Pulmozyme®, pancreatic enzyme replacement therapy, oral antibiotics, fat-soluble vitamins, insulin, inhaled compounded tobramycin, oral nutritional supplements, insulin, AZLI
Exclusion criteria
Exclusion criteria: 1. Pregnancy 2. Acceptance on the lung transplant list (note: participation in this study will not delay or exclude patients from being placed on the transplant list in the future or receiving a transplant once enrolled in the study)
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Medication Possession Ratio (MPR) derived from two sources (self-report and pharmacy refill records) at baseline and 12 months | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. CF medication knowledge [time frame: baseline, 6 months, 12 months] 2. Health Related Quality of Life measured by the validated CF-QoL [time frame: baseline, 12 months] 3. Lung Function [time frame: baseline, 6 months, 12 months]. Forced expiratory volume in one second (FEV1) percent predicted and forced vital capacity (FVC) – used to calculate rate of decline for 1 year pre and 1 year post start intervention 4. BMI [time frame: baseline, 6 months, 12 months] 5. Vitamin A, D, E levels [time frame: baseline, 6 months, 12 months] 6. Coefficient of variation for lung function [time frame: baseline, 6 months, 12 months]. For each measure the coefficient of variation will be calculated from the variation of the highest and lowest values of lung function over the previous 6 months 7. Pulmonary exacerbation [time frame: number of exacerbations requiring intravenous therapy from 1 year prior to baseline until start; and from baseline to 1 year] 8. CF hospitalisations [time frame: 1 year prior to baseline until start; and from baseline to 1 year] – including iv treatment days | — |
Countries
England, United Kingdom
Outcome results
None listed