Cystic Fibrosis.
Conditions
Interventions
All subjects nebulized daily both rhDNase (2.5 mg of rhDNase in 2.5 ml buffered solution: 8.77 mg/ml sodium chloride and 0.15 mg/ml calcium chloride) and a placebo (2.5 ml of a buffered solution: 8.77
Sponsors
Roche Nederland BV
PO Box 44
3440 AA WOERDEN
The Netherlands
Eligibility
Inclusion criteria
Inclusion criteria: 1. Proven CF, as evidenced by an abnormal sweat test or an abnormal rectum potential difference measurement or by the presence of two CF mutations and at least one clinical feature of CF. 2. Treated at the Erasmus MC - Sophia, and a. Five years and older; b. Able to perform reproducible manoeuvres for spirometry; c. Maintenance treatment with rhDNase for at least one month d. Clinically stable for at least one month (no intravenous antibiotics and / or hospitalizations within one month before enrolment); 3. Willing to participate in and comply with study procedures, and willingness of the parent or guardian and subjects >12 years to provide written informed consent.
Exclusion criteria
Exclusion criteria: Admission: 1. FVC <40%; 2. Using rhDNase more than once daily; 3. Mental retardation; 4. Having a history of non-adherence to treatment advice known to the physician.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Pulmonary function test: MEF25. | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. Pulmonary function tests: FVC, FEV1, Rint; 2. Frequency and duration of coughing measured with audiorecording; 3. Oxygenation at night recording transcutaneous oxygen saturation; percentage with saturation below 95%; 4. Severity of cough with a VCD score; 5. Sputum characteristics: amount, viscosity with a VAS-score; 6. Quality of sleep and appetite with a VAS-score; 7. Presence of morning sickness. | — |
Contacts
Public ContactLianne Giessen, van der
Erasmus Medical Center, Sophia Children's Hospital, Department Pediatric Physiotherapy, SK 0327, Dr Molewaterplein 60
Outcome results
None listed