What is cystic fibrosis?

Cystic fibrosis is a progressive, genetic disease that affects ~30,000 people in the US and ~70,000 people worldwide. In people with CF, there is a malfunction of chloride transfer at the cell surface which leads to the buildup of sticky mucous in multiple organs.

 

In the lungs, this residual thick mucous can lead to chronic infection and inflammation which progressively limits one's ability to breathe over time. 

 

In the pancreas, this buildup of mucous inhibits the release of digestive enzymes critical for absorption of key nutrients. This can lead to poor weight gain and malnutrition. The current life expectancy is around 40 years of age. While great advancements are being made, there still is not a cure. 

What is the

treatment of CF?

The treatment of cystic can often feel as complex as the disease itself. Since cystic fibrosis affects everyone a bit differently, the treatment typically varies from person to person as well. Due to the effect on the pancreas, most individuals with CF must take synthetic enzymes in the form of pills every time they eat to facilitate the breakdown of fat, protein and other essential nutrients. Because of this pancreatic insufficiency, people with CF typically have a hard time gaining weight and therefore are prescribed diets high in fat and protein. Most people with CF also take a daily vitamin supplement.

Airway clearance, or chest PT, is also an important part of the treatment regimen. This involves connecting to a percussive vest for 30 minutes two or more times a day. The vibration of the chest loosens the mucous from the lungs so that the individual can cough it out. The goal of this treatment is to regularly clear any buildup of mucous from the airways which helps reduce the chance of bacteria settling in and causing trouble. Another option is manually performing chest PT, which involves strategically pounding on the chest over the lung fields.  This technique is used in infants or those unable to use the vest.

Inhaled therapies are also a common treatment of CF. These are often utilized along with chest PT to help open the airways and hydrate the mucous, making it thinner and easier to shake loose and cough out.

Many people with CF are now adding a new twice daily pill to their treatment plan. These new drugs called gene-modulators are the first to target the underlying cause of this disease rather than just treat a symptom. The use of these are specific to an individual’s mutation which means the same drug doesn’t work for everyone with CF. The newest, and most effective, however, was just approved by the FDA for people 12 and up and works in 90% of people for CF. This major breakthrough is being hailed a “miracle drug” and is showing remarkable success in improving lung function and overall well-being. The CF Community continues to celebrate this monumental milestone but recognizes this is not a cure and there is still so much work to be done.

What causes CF?

Cystic fibrosis is an inherited disorder that occurs when the baby receives two copies of a recessive CF gene. Each parent must be a carrier of the CF gene to pass it to their offspring. Carriers are unaffected and do not have any symptoms. Unless there is a known history of CF in their family and they have been tested for the gene before getting pregnant, they typically do not even know they are carriers. When parents are carriers of the CF gene, there is a 25% the baby will have CF, a 50% chance the baby will be a carrier, and a 25% chance the baby will not even carry the CF gene at all.

Infographic courtesy of Cystic Fibrosis Foundation

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