Trikafta for Patients With Non-cystic Fibrosis Bronchiectasis

Study participants with non-cystic fibrosis bronchiectasis will be given Trikafta for four weeks. The researchers will monitor clinical endpoints, quality of life, and weight. Additionally, cutaneous punch biopsy material will be collected from each participant to test cellular response to Trikafta.

Inclusion Criteria:Provision of signed and dated informed consent formStated willingness to comply with all study procedures and availability for the duration of the studyRadiologic and other clinical evidence leading to a diagnosis of NCFBE1 CF-causing mutation and/or sweat chloride measurement ≥ 30 mEq/L and < 60 mEq/LAble to perform spirometry meeting American Thoracic Society (ATS) criteria for acceptability and repeatability, and FEV1 40-90% predictedClinically stable in the past 4 weeks with no evidence of bronchiectasis exacerbationWillingness to use at least one form of acceptable birth control including abstinence or condom with spermicide. This will include birth control for at least one month prior to screening and agreement to use such a method during study participation for an additional four weeks after the last administration of Study DrugAbility to take TrikaftaAgreement to adhere to all current medical therapies as designated by the study physicianExclusion Criteria:Diagnosis of cystic fibrosisDocumented history of drug or alcohol abuse within the last yearPulmonary exacerbation or changes in therapy for pulmonary disease in the 4 weeks prior to screeningListed for lung or liver transplant at the time of screeningCirrhosis or elevated liver transaminases > 3 times the upper limit of normal (ULN)Pregnant or breastfeedingInhibitors or inducers of CYP3A4, including certain herbal medications and grapefruit/grapefruit juice, or other medicines known to negatively influence Trikafta administrationHistory of solid organ transplantActive therapy for non-tuberculosis mycobacterial infection or any plan to initiate non-tuberculosis mycobacterial therapies during the study periodKnown allergy to TrikaftaTreatment in the last 6 months with an approved CFTR modulatorAny other condition that in the opinion of the lead investigators might confound results of the study or pose an additional risk from administering Study DrugTreatment with another investigational drug or other intervention within one month prior to enrollment, throughout the duration of study participation, and for an additional four weeks following final drug administrationEvidence of cataract/lens opacity determined to be clinically significant by an ophthalmologist at or within 3 months prior to the Screening Visit