Definition (CDC)
A genetic disease of the mucus and sweat glands that causes problems with breathing and digestion. It mainly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs. People with CF have mucus that is too thick and sticky, which
- blocks airways and leads to lung damage
- traps germs and makes infections more likely
- prevents proteins needed for digestion from reaching the intestines
Causes
Cystic fibrosis (CF) is a genetic disease. People with CF inherited two copies of the defective CF gene. Both parents must have at least one copy of the defective gene.
People with only one copy of the defective CF gene are carriers but do not have the disease.
Statistics
- 40,000 children and adults are living with cystic fibrosis in the U.S
- Approximately 1,000 new cases of cystic fibrosis are diagnosed each year
Risk Factors
Both parents must be CF carriers for a person to have Cystic Fibrosis. About 1 in 20 people are a CF carrier. If both parents are a carrier, there is a
- 3 in 4 chance (75%) the child won’t have CF
- 1 in 2 chance (50%) the child won’t have CF but will be a carrier
- 1 in 4 chance (25%) the child will have CF
- 1 in 4 chance (25%) the child will not have CF and not be a carrier
Treatment
There is no known prevention for cystic fibrosis, but there are steps that can be taken to improve breathing and digestion, prevent and treat infections, and thin mucus:
- Airway Clearance Techniques loosen thick, sticky mucus so it can be cleared from the lungs. Clearing the airways may help decrease lung infection and improve lung function
- Physical Activity can help improve lung function and prevent additional chronic disease development
- Medications may be prescribed to help keep the lungs clear, prevent or fight infections, and improve digestion
- Nutritional Plan to consume the correct amount of calories and nutrients
Vaccines
The CDC recommends that all adults keep their vaccinations up to date. Childhood immunizations may wear off after time and need a “booster shot,” and you are at risk for other diseases as an adult.
All adults need:
- Influenza – recommended every year, age 6 months and older
- Tdap – (if an adult did not receive it as an adolescent to protect against pertussis (whooping cough), and then a Td (tetanus, diphtheria) booster shot every 10 years.
Additionally, the CDC recommends the following vaccines for individuals suffering from Cystic Fibrosis (talk to your doctor about which vaccines are right for you):
- Pneumococcal Vaccine
COVID-19 and Cystic Fibrosis
People with cystic fibrosis are more likely to get very sick from COVID-19, especially lung or other solid lung transplant recipients.
For more information, visit Cystic Fibrosis Foundation’s CF and COVID-19 webpage.
Resources
- Cystic Fibrosis Foundation
- Centers for Disease Control and Prevention
- American Lung Association: Cystic Fibrosis
- National Institutes of Health, National Heart, Lung, and Blood Institute