Table 1

Modulator therapy and classes of CF variants

Trade nameGeneric nameVariant classCommon variants
KalydecoIvacaftorClass III–VG551D, S549N, G1349D, R117H, 3849+10kbC→T
OrkambiLumacaftor-ivacaftorClass IIPhe508del homozygous
Symkevi/SymdekoTezacaftor-ivacaftorClass II–VPhe508del homozygous, Phe508del and a residual function variant—L206W, 3272-26A→G, P67L, heterozygous for one of 154 responsive variants
Kaftrio (UK)/Trikafta (USA)Elexacaftor-tezacaftor-ivacaftorClass II–VPhe508del homozygous, Phe508del and any other variant, heterozygous for one of 177 responsive variants