Cystic fibrosis mucoviscidosis
Conditions
Interventions
Elexacaftor/tezacaftor/ivacaftor combination with ivacaftor
(Elexacaftor/tezacaftor/ivacaftor).
All subjects will be treated from day 1 of the study with the standard dose
elexacaftor/tezacaftor/iv
Sponsors
HagaZiekenhuis
Eligibility
Age
18 Years to 64 Years
Inclusion criteria
Inclusion criteria: - Males and females aged 18 years or older on the date of informed consent - Diagnosis of cystic fibrosis with a genotype registered for the use of elexacaftor/tezacaftor/ivacaftor confirmed by genotype analysis - Kidney or liver transplantation; at least 1 year ago - Current use of tacrolimus - Signed informed consent form (ICF)
Exclusion criteria
Exclusion criteria: - Use of drugs that are metabolized by the CYP3A enzyme or have a known influence on the CYP3A enzyme (inducers or inhibitors) - Having a contra indication for the use of elexacaftor/tezacaftor/ivacaftor - Organ rejection within the last 3 months - Pulmonary exacerbation within one month before the study period (defined as need for intravenous antibiotics) - Pregnancy or lactation -Pregnancy wish
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| - Pharmacokinetic parameters: T1/2, Tmax, Cmax, Cmin , AUC all measured without and with co-administration of elexacaftor/tezacaftor/ivacaftor in order to measure RAUC of tacrolimus (RAUC=AUCtacro with elexacaftor/tezacaftor/ivacaftor/AUCtacro) in week 1, 2, 3 and 4 after starting elexacaftor/tezacaftor/ivacaftor. - Change in pk parameters after co-administration of elexacaftor/tezacaftor/ivacaftor per individual : T1/2 (T1/2 tacro with elexacaftor/tezacaftor/ivacaftor * T1/2 tacro), Tmax (Tmax tacro with elexacaftor/tezacaftor/ivacaftor * Tmax tacro), Cmax (Cmax tacro with elexacaftor/tezacaftor/ivacaftor * Cmax tacro) , Cmin (Cmin tacro with elexacaftor/tezacaftor/ivacaftor * Cmin tacro) measured in week 1, 2, 3 and 4 after starting elexacaftor/tezacaftor/ivacaftor - Number and level of dose adjustments made in order to stay within target tacrolimus values. | — |
Secondary
| Measure | Time frame |
|---|---|
| - Absolute change in lung function (FVC and FEV1), absolute change in quality of life (CFQ), , absolute change in nutritional status (weight, BMI) in week 1, 2, 3 and 4 after starting elexacaftor/tezacaftor/ivacaftor. Absolute change in sweatchloride from baseline through end of the study. - Through concentrations of elexacaftor, tezacaftor and ivacaftor measured in week 1, 2, 3 and 4 after starting elexacaftor/tezacaftor/ivacaftor. - Safety and tolerability based on the number and type of (S)AEs, laboratory values and vital signs. | — |
Countries
Netherlands
Outcome results
None listed