have the disease. Each time two CF carriers have a
child, the chances are:
-
-
-
25 percent (1 in 4) the child will have CF
50 percent (1 in 2) the child will be a carrier
but will not have CF
25 percent (1 in 4) the child will not be a
carrier and will not have CF
The defective CF gene contains a slight
abnormality called a mutation. There are more
than 1,700 known mutations of the disease. Most
genetic tests only screen for the most common CF
mutations. Therefore, the test results may indicate
a person who is a carrier of the CF gene is not a
carrier
3. Wh at t o Expect
Cystic fibrosis is a complex disease and the types
and severity of symptoms can differ 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.
People with cystic fibrosis are at greater risk of
getting lung infections because thick, sticky mucus
builds up in their lungs, allowing germs to thrive
and multiply. Lung infections, caused mostly by
bacteria, are a serious and chronic problem for
many people living with the disease. Minimizing
contact with germs is a top concern for people
with CF.
The buildup of mucus in the pancreas can also
stop the absorption of 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.
4. Tr eat m en t s
The type and severity of CF symptoms can differ
widely from person to person. Therefore, although
treatment plans can contain many of the same
elements, they are tailored to each individual's
unique circumstances.
Gavin, a 15-year-old with CF, wearing his vest.
People with CF need to work closely with their
medical professionals and families to create
individualized treatment plans.
The CF Foundation accredits more than120 care
centers that are staffed by dedicated health care
professionals who provide expert CF care and
specialized disease management.
Each day, people with CF complete a combination
of the following therapies:
-
-
-
Airway clearanceto 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.
Inhaled medicinesto open the airways or
thin the mucus. These are 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.
Pancreatic enzyme supplement capsules to
improve the absorption of vital nutrients.
These supplements are taken with every
meal and most snacks. People with CF also
usually take multivitamins.
Break through treatments have added years to the
lives of people with cystic fibrosis. Today the
median predicted survival age is close to 40. This is
a dramatic improvement from the 1950s, when a
child with CF rarely lived long enough to attend
elementary school. The CF Foundation supports research to discover
and develop new CF treatments and maintains a
pipeline of potential therapies that target the
disease from every angle.
Because of tremendous advancements in research
and care, many people with CF are living long
enough to realize their dreams of attending
college, pursuing careers, getting married,
andhaving kids. In 2015, the United States Food and Drug
Administration approved the second drug to treat
the root cause of cystic fibrosis, a defective protein
known as CFTR. The first drug targeting the basic
genetic defect in CF was approved in 2012. The