Should all children with cystic fibrosis who have responsive CFTR mutations be prescribed CFTR modulators?

•The decision to use ETI should be one shared between the clinician, parents and child.•The best interests of the child should be central to the decision to use ETI. The evidence supports the foreseeable possibility of decades of additional life and improved QOL.•The risks of long-term side-effects or lack of long-term efficacy do not hold great moral weight as they are distant and not clearly foreseeable.•The harms of not using ETI in a particular case should guide the effort to which one might try to ensure it's use.•Clinical ethics consultation may assist clinicians, children and parents if there is sustained refusal to use ETI.