How is CF treated?

Treating a complex disease like CF requires therapies that address problems in different parts of the body, especially the lungs and the digestive system.

Because the type and severity of CF symptoms can differ widely from person to person, there is no typical treatment plan for people with the disease. CF Foundation-accredited care centers work closely with people with CF and their families to create individualized treatment plans.

However, each day, most people with CF typically:

  • Do some form of airway clearance to help loosen and get rid of the thick mucus that can build up in the lungs. Some airway clearance techniques require help from family members, friends or respiratory therapists. Many people with CF use an inflatable vest that vibrates the chest at a high frequency to help loosen and thin mucus.
  • Take inhaled medicines — liquid medicines that are made into a mist or aerosol and then inhaled through a nebulizer. These medicines include antibiotics to fight lung infections and therapies to help keep the airways clear.
  • Take pancreatic enzyme supplement capsules with every meal and most snacks to improve absorption of vital nutrients. People with CF also usually take multivitamins.

The most recent drug approved for CF, ivacaftor (Kalydeco™), treats the underlying cause of CF in a small number of people with specific mutations of the CF gene. All other CF therapies available today treat the symptoms of CF.

Work is ongoing to find additional CF therapies that could help improve key symptoms of the disease by targeting the disease at its root.

 

What is the life expectancy for people with CF?

It is not possible to accurately predict how long a person who has CF will live. Many different factors — for example, severity of disease and age at diagnosis — can affect an individual’s health and the course of the disease. Recent research has shown that the severity of CF symptoms is based partly on the type of CF gene mutations a person has.

 The CF Foundation Patient Registry collects information on the health of the more than 27,000 people treated at CF Foundation-accredited care centers. According to the most recent Patient Registry data, the median predicted age of survival for people with CF is close to 40. Median predicted age of survival is the age by which half of the people tracked in the Patient Registry would be expected to survive, given the ages of the patients in the Registry and the distribution of deaths in a particular year.

In the 1950s, children with CF were not expected to live long enough to attend elementary school. Today, approximately half of all people with CF in the United States are 18 years or older. The steady “aging” of the CF population reflects the remarkable progress that has been made in understanding and treating CF. Thanks to CF Foundation-supported research and care, an increasing number of people with CF are living well into adulthood and leading healthy lives, pursuing careers, getting married and having children of their own.

 

Is there a cure for CF?

Currently, there is no cure for CF. However, specialized medical care, aggressive drug treatments and therapies, along with proper CF nutrition, can significantly lengthen and improve the quality of life for those with CF.

In 2012, the U.S. Food and Drug Administration approved ivacaftor (Kalydeco™) — the first drug to treat the underlying cause of CF in a small group of people with a specific mutation of the CF gene. In 2014, ivacaftor was approved for people ages 6 and older who have eight additional CF mutations. 

It is too early say whether ivacaftor will be an actual cure for those who are taking it. However, the drug has shown that it is possible to target the defective CFTR protein that causes CF and improve key symptoms of the disease. Ivacaftor is now being studied in combination with other potential therapies to treat people with the most common CF mutation.

Ivacaftor also offers a roadmap that could help advance the discovery and development of more lifesaving therapies for all people with CF. The CF Foundation continues to support cutting-edge research to address the root cause of CF, and maintains a robust pipeline of potential therapies targeting the disease from every angle. Research to find a cure for CF has never been more promising.

Please visit CFF’s Patient Assistance Resource Center to learn more about assistance programs available to those with CF. 

 

Source: CFF.org