Slowing Lung Failure in Cystic Fibrosis Patients is the Only Prevention

Cystic fibrosis (CF) is a life-threatening genetic disease that causes mucus to build up and clog the lungs, making breathing very difficult. The thick mucus also causes bacteria to get stuck in the airways, which causes inflammation and infections that leads to lung damage. At this time, there is no proven way to prevent lung failure in cystic fibrosis (CF) patients. However, there are many studies that have proven that the deterioration of the lungs can be slowed down in some CF patients.

The body naturally produces mucus to stop germs and bacteria from reaching susceptible areas and causing infection. Mucus is regarded as impenetrable, making it is very difficult to treat CF patients that have an excessive amount of mucus built up. Researchers have studied a way to develop a coating that will enable drugs to get through the mucus layer faster.

High levels of ceramide, a chemical produced by the body, are thought to cause increased inflammation and death of lung cells. It is believed that it may be possible to halt this chain reaction with the use of certain anti-depressant drugs in many CF patients. Elavil is one of the drugs believed to help prevent lung infection. However, the dose must be exactly right to avoid harm and may not be beneficial to all CF patients.

Cigarette smoke can worsen lung diseases, including CF. If you have CF and you smoke, stop right now!! The sooner you stop the better chance you have of slowing down the progression of lung failure. Avoid secondhand smoke, which has been proven to cause just as much damage as actually smoking. It has been discovered that CF patients have a mutated TGFbeta1 gene that is highly susceptible to secondhand smoke. It can weaken lung function and shorten the lifespan of people with cystic fibrosis who are exposed to secondhand smoke.

High doses of ibuprofen have shown a significantly slower rate of reduction of lung function in children and adolescents with CF. According to emaxhealth.com, researchers found that CF patients who took high doses of ibuprofen had a 29 percent reduction in loss of lung function compared with those who did not use the anti-inflammatory drug twice daily, over a period of 2 to 7 years. Although ibuprofen is reported to cause internal bleeding in the stomach and intestines, the benefits of ibuprofen therapy outweigh this small risk if therapy is started before the age of 13.

Inhalation therapy is the breathing in of saline, which is basically salt water, to help break up the mucus. This therapy seems to work better on adults than on children.

When lung failure progresses to a critical stage, it will be time to think about a lung transplant. At first, it was not recommended for children to undergo the lung transplants because of the dangers and no proven beneficial results. However, lung transplants have come a long way since the beginning, allowing CF patients, young and old, to live many more years.

In the UK, a portable artificial lung that works the same way as a human lung is being developed to give CF patients more freedom. Although it may be years before this is perfected, it is something to look forward to.

The outlook for people with cystic fibrosis continues to improve steadily each year.