James Finklea, M.D. Answers Questions On: Cystic Fibrosis

Vitamin D is not only a hormone that helps build bone and muscle; it also helps fight infections. It’s an antimicrobial peptide.

Studies have found that patients with higher vitamin D levels have fewer infections. That’s why we push to make vitamin D levels higher in cystic fibrosis patients.

Cystic fibrosis transmembrane protein, the gene that causes cystic fibrosis, is damaged not only in the lungs but also in the pancreas.

The damage in the pancreas eventually leads to a chronic inflammation. It damages the cells that secrete insulin.

Because of that, CF patients produce less insulin, causing diabetes. It’s not the classic type I or type II diabetes, but a category in itself. It’s treated with insulin injections, like other diabetes.

If CF patients live long enough, many will need a lung transplant. To be considered for a lung transplant, they need to have significant loss of lung function. If their lung function falls to 25 to 30 percent (out of 100), then they would need to think about a lung transplant.

Cystic fibrosis patients make good candidates for lung transplantation because they are generally younger than most lung transplant patients. The statistics show CF patients live longer after transplant than other lung transplant patients. The median survival of all lung transplant patients is five years. The median survival of a cystic fibrosis patient who receives a lung transplant is eight years.