-
-
-
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.
The CF Foundation supports research to
discover and develop new CF treatments
and maintains apipeline of potential
therapies that target the disease from
every angle.
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 arrival of this
group of drugs, called CFTR modulators,
signals a historic breakthrough in how CF is
treated. It's expected that CFTR modulators
could add decades of life for some people
with
CF.
Today, the Foundation is focused on
developing life saving new therapies for
larger numbers of people with CF and
pursuing daring, new opportunities to one
day develop a lifelong cure.
5. How is cyst ic f ibr osis r elat ed t o t h e
cell m em br an e?
Cyst ic f ibr osis is a disease t h at cau ses an
excess of f lu id pr odu ct ion in t h e lu n gs
du e t o an ef f ect in t h e calciu m -ion
ch an n el. Th is ch an n el con t ain s a pr ot ein
t h at
is im por t an t
f or
t he
cell
m em br an es of t h e lu n gs an d con t r ols
t h e level of f lu ids an d m u cu s w it h in
t h em . Wh en t h e ch an n el m u t at es
t ow ar ds
cyst ic
f ibr osis
m u cu s
accu m u lat es w h ich m ak es it ver y
dif f icu lt t o br eat h e.