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Cystic FibrosisJuly 2018Summary reviewed July 2026

What the ARRIVAL Trial Found — Ivacaftor for Cystic Fibrosis in Infants

Researchers tested ivacaftor in babies aged 12 to 24 months with cystic fibrosis and a specific gene mutation. The medicine was safe and worked well, lowering sweat chloride levels (a key marker of the disease) and showing early signs of protecting pancreas function.

What the trial was testing

The ARRIVAL enrolled 57 patients with cystic fibrosis. The study was sponsored by Vertex Pharmaceuticals Incorporated and tracked outcomes across the full group of patients who matched the trial's eligibility profile.

It was a large trial designed to confirm whether the treatment works well enough for wider use. Trials at this stage are designed to produce evidence regulators and physicians can act on — not just observations to follow up later.

What the results showed

Sweat chloride dropped by 73.5 mmol/L on average after 24 weeks of treatment.

The Lancet. Respiratory medicine · 2018 · NCT02725567

These findings — that a large reduction in sweat chloride shows the drug is working to improve CFTR function — were published in the The Lancet. Respiratory medicine and represent the headline result of the study.

Researchers tracked outcomes across 57 patients enrolled in the trial. The result was consistent enough across the group that the team felt confident reporting it.

What this means for patients

For patients with cystic fibrosis, this result changes the calculus on what to ask their care team about. Whether it changes day-to-day care depends on factors like disease subtype, prior treatments, and where the patient is in their care journey.

What you can do now

Ivacaftor is FDA-approved for young children with cystic fibrosis and certain gene mutations, including those as young as 12 months. If your child has cystic fibrosis, ask your doctor about genetic testing to see if ivacaftor is right for them. Starting treatment early may help protect the pancreas.

Eligibility for the treatments mentioned above depends on specific test results and clinical history. Bring this summary, the trial name, and your most recent labs or pathology report to your next visit.