Cystic Fibrosis, or CF, is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time. In people with CF, mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause the CFTR protein to become dysfunctional. When the protein is not working correctly, it’s unable to help move chloride -- a component of salt -- to the cell surface. Without the chloride to attract water to the cell surface, the mucus in various organs becomes thick and sticky.
In the lungs, the mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications. For this reason, minimizing contact with germs is a top concern for people with CF.
In the pancreas, the buildup of mucus prevents the release of digestive enzymes that help the body absorb food and key nutrients, resulting in malnutrition and poor growth. In the liver, the thick mucus can block the bile duct, causing liver disease. In men, CF can affect their ability to have children.
People with CF can have a variety of symptoms, including very salty-tasting skin, persistent coughing, at times with phlegm, frequent lung infections including pneumonia or bronchitis, wheezing or shortness of breath, poor growth or weight gain in spite of a good appetite, frequent greasy, bulky stools or difficulty with bowel movements, and/or male infertility.
Mucus also can block the digestive tract and pancreas. The mucus stops digestive enzymes from getting to the intestines. The body needs these enzymes to break down food, which provides important nutrients to help people grow and stay healthy. People with CF often need to replace these enzymes with the medicine they take with their meals and snacks, which helps them digest food and get proper nutrition.
CF is a complex disease with various types and severity of symptoms that can vary widely from person to person. Many different factors, such as age of diagnosis, can affect an individual's health and the course of the disease. Therefore, although treatment plans can contain many of the same elements, they are tailored to each individual's unique circumstances.
When the Cystic Fibrosis Foundation was established in 1955, people born with the disease weren't expected to live to attend elementary school. Today, because of the efforts of the Cystic Fibrosis Foundation, people with CF are living into their 30s, 40s, and beyond. Faced with insurmountable odds, but determined to save the lives of their children, a group of concerned parents came together to form the Cystic Fibrosis Foundation.
At the time, very little was known about cystic fibrosis, but the tenacity and strength of CF families laid the groundwork for monumental progress in research, care, and treatment of this rare disease. Today, because of their foresight and the hard work and dedication of thousands of families and volunteers across the country, the CF Foundation is the world's leader in the search for a cure for cystic fibrosis.
Since 1955, the CF Foundation has added decades to the lives of people with the disease. These gains are the direct result of advances in treatment and care made possible through their innovative business model, called venture philanthropy.
The mission of the Cystic Fibrosis Foundation is to cure cystic fibrosis and to provide all people with CF the opportunity to lead long, fulfilling lives by funding research and drug development, partnering with the CF community, and advancing high-quality, specialized care.
“65 Roses” is what some children with Cystic Fibrosis call their disease because the words are much easier for them to pronounce. Mary G. Weiss became a volunteer for the Cystic Fibrosis Foundation in 1965 after learning that her three little boys had CF. Mary’s son, Richard, listened closely to his mother as she made calls seeking financial support for CF research. Later, Richard told his Mom, “I know what you are working for.” Mary was dumbstruck because Richard did not know what she was doing, nor did he know that he had CF. With some trepidation, Mary asked, “What am I working for, Richard?” He answered, “You are working for 65 Roses.” He could not see the tears running down Mary’s cheeks as she stammered, “Yes Richard, I’m working for 65 Roses.” Since 1965, the term “65 Roses” has been used by children of all ages to describe their disease. But making it easier to say does not make CF any easier to live with. The “65 Roses” story has captured the hearts and emotions of all who have heard it. The rose, appropriately the ancient symbol of love, has become a registered trademark of the Cystic Fibrosis Foundation.