What is Cystic Fibrosis (CF)?

Jens was born with Cystic Fibrosis (CF). He was diagnosed when he was about 1 year old. CF is an inherited disease that affects about 30,000 people in the United States. A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:

• clogs the lungs and leads to life-threatening lung infections; and
• obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food. (http://www.cff.org/AboutCF/)

Each case of CF is different. Jens has a tougher case than most because he also has a bacteria called B. Cepacia. Because it is dangerous for CF patients with this bacteria complex to be around each other, Jens cannot be in physical proximity to anyone with CF. Jens spends over 4 hours a day doing breathing treatments of inhaled medicines, including time using an airway clearance device called “The Vest.” He must also take enzymes before every meal and snack to help his body digest his food. (For more info, stay tuned for an upcoming blog, “A Day in the Life of Jens”)

There is no known cure for CF at this time. A lung transplant will not cure CF because the defective gene that causes the disease is in all of the cells in the body, not just in the lungs. At this time, scientists are not able to “fix” genes permanently (see gene therapy). While a transplant does give a person with CF a new set of lungs, the rest of the cells in the body still have CF and may already be damaged by the disease. Further, organ rejection is always possible and drugs that help prevent organ rejection can cause other health problems. (http://www.cff.org/AboutCF/Faqs/ )