Cystic fibrosis dating other cf patients

For patients, learning to work with a new team of adult specialists can be a difficult transition, Champion said: “You get used to seeing the same faces and seeing the same people.” In addition, some cystic fibrosis patients have difficulty obtaining health insurance after they age out of their parents’ plans, Champion said.Meanwhile, patients are dealing with the challenges that every teenager must confront: relationships, education, leaving home and choosing a career, said Elisabeth Dellon, MD, MPH, assistant professor of pediatrics in the division of pulmonology.Patients with CF have mutations in the CFTR gene, which is supposed to create a protein that regulates the flow of water and chloride in and out of the cells that line the lungs, pancreas, and other organs.However, CFTR mutations can lead to the production of defective proteins or to producing no protein at all.Drugs are designed to target proteins based on specific CFTR gene mutations.These drugs currently are available in oral tablet form.Doing so can help patients understand that graduating to adult care is a normal part of growing up, Champion said.

Pediatricians are also learning to help their young patients transition to adult medicine.This results in thick secretions that can cause infections, damage, and problems with the lungs, pancreas, and sinuses among other organs.CFTR modulators target the defective proteins and work to control their effects by moving them to the cell surface and helping them function properly.But a new classification of drugs – cystic fibrosis transmembrane conductance regulator modulators (CFTR modulators) – provides an opportunity to slow progression of the disease for more than 90 percent of CF patients.CF is a genetic condition that causes thick secretions in the lungs, pancreas, and other organs.

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