The FDA expanded the approval of lumacaftor and ivacaftor to include the treatment of children with cystic fibrosis aged 1 year to younger than 2 years, according to a manufacturer-issued press release.
This approval applies to children who are homozygous for the F508del mutation (F/F genotype) in the CFTR gene.
Harboring two copies of the F508del mutation leads to a deficiency of the CFTR protein at the cell surface. The agent combines lumacaftor, which targets the defect of the F508del-CFTR protein that inhibits processing and trafficking, thereby increasing the amount of the protein at the