The purpose of this presentation is to review the transition of CF patients from pediatric to adult care, to palliative care, and to lung transplant. CF is a genetic, inheritable disease, usually diagnosed in childhood, caused by a mutation in the CFTR (Cystic Fibrosis Transmembrane conductance Regulator) protein which codes for a chloride channel. When the chloride channel is mutated, there is loss of mucociliary clearance resulting in long term bronchiectasis and frequent bacterial infections. CF is a systemic disease, affecting not only the lungs but also the GI tract, pancreas, biliary tree, reproductive organs, and others.

Patients with CF are living longer thanks to newer therapies such as CFTR modulators that directly target specific CFTR mutations. With survival improving, more patients will require transition to adult providers. While our Adult CF clinic provides a multidisciplinary approach to care with physicians, nurses, respiratory therapists, dieticians, and social workers, we are lacking in CF related subspecialty providers for our patients. The biggest limitation to transition of care remains to be insurance and funding for our patients and many are on Medicaid.

Though survival is improving for some patients, there will still be a few who develop CF Advanced Lung Disease (CFALD) and may require transition to Palliative Care or Lung Transplant.