About Cystic Fibrosis

What is Cystic Fibrosis?
Cystic Fibrosis is a genetic disease in which the body produces an abnormally thick, sticky mucus. This mucus clogs the airways, leads to chronic and fatal lung infections, and impairs digestion. Currently, there is no cure.

What causes Cystic Fibrosis?
A defective gene alters a protein that regulates the normal movement of salt in and out of cells, which causes normally thin, slippery secretions like sweat and saliva to become thick and sticky.

How many people have Cystic Fibrosis?
Approximately, 30,0000 children and adults in the United States have CF, and almost 1,000 new cases of CF are diagnosed each year. While diagnosis typically occurs before an individual is three years old, as many as 10% of new cases are people over the age of 18.

What are the symptoms of Cystic Fibrosis?
People with CF have a variety of symptoms:

• Persistent coughing (at times with phlegm)
• Wheezing or shortness of breath
• Excessive appetite but poor weight gain
• Very salty-tasting skin
• Greasy, bulky stools

Symptoms can vary widely from person to person, however, because of the many mutations of the CF gene.

How is Cystic Fibrosis treated?
Treatment depends on how much the disease has progressed and the organs it has affected, but all strategies focus on preventing infections, reducing the mucus in the lungs, and maintaining nutrition. Current treatment options include:

• Antibiotics like Tobramycin solution and Azithromycin
• Mucus thinning drugs like Pulmozyme
• Bronchodilator drugs like albuterol (proventil, Ventolin), which help keep the bronchial tubes clear
• Chest physical therapy (clapping on the back and chest to dislodge mucus from the lungs at least twice a day for 20-30 minutes)
• Oral enzymes and supplemental high-calorie nutrition, which counter the effect CF has on the pancreatic enzymes that prevent food from being absorbed in the small intestine
• Lung transplantation (for severe breathing problems, life-threatening lung complications, or increasing resistance to medication)

While many advances in treatment have occurred over the years, the median age of survival for individuals with CF is mid-30s. In addition, adults with CF face other challenges besides the symptoms listed above such as diabetes and osteoporosis. CF can also cause reproductive problems. In men, more than 95% of those diagnosed with CF are sterile, but new technology is making fatherhood a possibility. Meanwhile, though many women with CF are able to conceive, carrying a baby to term is hard for them because of their limited lung function and other health problems.

Today, research is being conducted into a number of new treatment technologies. Some of these technologies seek to deliver copies of the normal gene into cells of the respiratory tract of CF patients. Others modify the protein produced by the CF gene, so that the movement of salt and water in and out of cells can be normalized.

What can I do to help find a cure for Cystic Fibrosis?
Register to ride your motorcycle or car with us on Amy's Ride for CF, and enjoy good food and fun while benefitting a great cause. If you cannot ride, you can join us for the pig roast we'll be having at the conclusion of the ride, make a donation, or be an event sponsor.

You can also make a secure online donation on the Cystic Fibrosis Foundation website or contribute by phone or mail. For more ideas (such as donating your vehicle or shopping online through selected retailers), visit the Cystic Fibrosis Foundation's Ways to Give page.

If you are unable to make a donation, just raising awareness about CF and Cystic Fibrosis Foundation in your community can be a great contribution to the cause! You can also visit the Volunteer page of the Cystic Fibrosis Foundation website to sign up to help with fundraising drives and events.

With your help, we can accomplish our ultimate goal of making CF stand for Cure Found.