Update: CPIC ivacaftor-CFTR guideline

The CPIC guideline for ivacaftor treatment based on CFTR genotype has been updated on PharmGKB, in light of changes to the FDA-approved drug label.

Originally, ivacaftor was indicated only in patients with the G551D variant. This has now been extended to other variants that result in CFTR gating defects:

• G1244E (rs267606723)
• G1349D (rs193922525)
• G178R (rs80282562)
• G551S (rs121909013)
• S1251N (rs74503330)
• S1255P (rs121909041)
• S549N (rs121908755)
• S549R (rs121909005, rs121908757)

> View the updated CPIC ivacaftor-CFTR guideline.

Figure 1: Treatment algorithm for clinical use of ivacaftor for cystic fibrosis patients based on CFTR genotype.