Cystic Fibrosis mucoviscidosis
Conditions
Interventions
None listed
Sponsors
HagaZiekenhuis
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: - Signed informed consent form (ICF ) - 18 years or older on the date of signed informed consent - Diagnosis of cystic fibrosis confirmed by genotype analysis - Current use of tezacaftor/ivacaftor in combination with ivacaftor - If pancreas insufficient, current use of amylase/lipase/protease, creon 10.000
Exclusion criteria
Exclusion criteria: - Use of drugs that have a known influence on the CYP3A enzyme (inducers or inhibitors) - Pulmonary exacerbation with hospital admission in the month before study participation (defined as need for intravenous antibiotics) - Pregnancy or breast feeding
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| - Pharmacokinetic paramaters (AUC, Tmax, Cmax and T*) of ivacaftor for pancreas sufficient patients and pancreas insufficient patients. | — |
Secondary
| Measure | Time frame |
|---|---|
| - Pharmacokinetic parameters for absorption and exposure of ivacaftor (AUC, Tmax, Cmax and T*) for pancreas insufficient patients with and without pancreas enzymes. The RAUC of ivacaftor (AUC with pancreas enzymes divided by AUC without pancreas enzymes) for pancreas insufficient patients. - Number of adverse and serious adverse events Other study parameters: - Age - Gender - Height - Weight - Body mass index (BMI) - Fat free mass index (FFMI) - Use of co-medication | — |
Countries
Netherlands
Outcome results
None listed