After signing the informed consent form, you will be enrolled in this long-term study that evaluates the safety and effectiveness of a medication combination for cystic fibrosis.

This study is specifically for people with cystic fibrosis who do not have the F508del genetic mutation.

The study consists of two parts: Part A and Part B, which will evaluate how well you tolerate the medication combination over an extended period.

You will take a combination of three medications called elexacaftor/tezacaftor/ivacaftor (also known as ELX/TEZ/IVA or by the brand name Kaftrio).

Depending on your age and weight, you will receive either Kaftrio 37.5 mg/25 mg/50 mg film-coated tablets or Kaftrio 75 mg/50 mg/100 mg film-coated tablets.

You will also take ivacaftor (brand name Kalydeco) as either 75 mg or 150 mg film-coated tablets.

These medications will be taken daily according to the schedule provided by your study doctor.

Throughout the study, you will attend scheduled clinic visits to monitor your health and response to treatment.

During these visits, the study team will perform various assessments to track your progress.

You must maintain your regular cystic fibrosis treatments (except for other CFTR modulators, which are medications that target the defective protein in cystic fibrosis) throughout the study period.

The study team will regularly monitor your health through several tests:

Lung function tests to measure your FEV1 (the amount of air you can forcefully exhale in one second)

Sweat chloride tests to measure the amount of salt in your sweat, which is typically elevated in people with cystic fibrosis

Completion of the CFQ-R questionnaire, which asks about your respiratory symptoms and quality of life

Measurements of your weight and BMI (body mass index, which is a calculation based on your height and weight)

Tracking of any pulmonary exacerbations (episodes when your lung symptoms get worse)

Throughout the study, your safety will be closely monitored through:

Adverse event reporting (any unexpected medical problems you experience)

Blood tests and other laboratory assessments

ECGs (electrocardiograms, which record the electrical activity of your heart)

Vital signs measurements (like blood pressure and heart rate)

Pulse oximetry (measuring the oxygen level in your blood)

After completing Part A of the study, if you meet the criteria, you will continue to Part B.

Part B will follow a similar format to Part A, continuing to evaluate the long-term safety and effectiveness of the medication.

The study is scheduled to run until April 2027.

Upon completion of the study, your study doctor will discuss next steps for your cystic fibrosis treatment.