Cystic Fibrosis

What Are the Signs and Symptoms of Cystic Fibrosis?

The signs and symptoms of cystic fibrosis (CF) vary from person to person and over time.
Sometimes you'll have few symptoms. Other times your symptoms may become more severe.
One of the first signs of CF that parents may notice is that their baby's s!in tastes salty when
!issed or the baby doesn't pass stool when first born.
"ost of the other signs and symptoms of CF happen later. They're related to how CF affects the
respiratory digestive or reproductive systems of the body.
Cystic Fibrosis
Figure # shows the organs that cystic fibrosis can affect. Figure $ shows a cross%section of a
normal airway. Figure C shows an airway with cystic fibrosis. The widened airway is bloc!ed
by thic! stic!y mucus that contains blood and bacteria.
Respiratory System Signs and Symptoms
&eople who have CF have thic! stic!y mucus that builds up in their airways. This buildup of
mucus ma!es it easier for bacteria to grow and cause infections. 'nfections can bloc! the airways
and cause fre(uent coughing that brings up thic! sputum (spit) or mucus that's sometimes
bloody.
&eople who have CF tend to have lung infections caused by unusual germs that don't respond to
standard antibiotics. For e)ample lung infections caused by bacteria called
mucoid Pseudomonas are much more common in people who have CF than in those who don't.
#n infection caused by these bacteria may be a sign of CF.
&eople who have CF have fre(uent bouts of sinusitis (si%nu%S'%tis) an infection of the sinuses.
The sinuses are hollow air spaces around the eyes nose and forehead. Fre(uent bouts
of bronchitis (bron%*'%tis) and pneumonia (nu%"O%ne%ah) also can occur. These infections can
cause long%term lung damage.
#s CF gets worse you may have more serious problems such as pneumothora) (noo%mo%
T+O,%a!s) or bronchiectasis (brong%!e%-*%ta%sis).
Some people who have CF also develop nasal polyps (growths in the nose) that may re(uire
surgery.
Digestive System Signs and Symptoms
'n CF mucus can bloc! tubes or ducts in your pancreas (an organ in your abdomen). These
bloc!ages prevent en.ymes from reaching your intestines.
#s a result your intestines can't fully absorb fats and proteins. This can cause ongoing diarrhea
or bul!y foul%smelling greasy stools. 'ntestinal bloc!ages also may occur especially in
newborns. Too much gas or severe constipation in the intestines may cause stomach pain and
discomfort.
# hallmar! of CF in children is poor weight gain and growth. These children are unable to get
enough nutrients from their food because of the lac! of en.ymes to help absorb fats and proteins.
#s CF gets worse other problems may occur such as/
&ancreatitis (&#0%!re%ah%T'%tis). This is a condition in which the pancreas become
inflamed which causes pain.
,ectal prolapse. Fre(uent coughing or problems passing stools may cause rectal tissue
from inside you to move out of your rectum.
1iver disease due to inflamed or bloc!ed bile ducts.
2iabetes.
3allstones.
Reproductive System Signs and Symptoms
"en who have CF are infertile because they're born without a vas deferens. The vas deferens is a
tube that delivers sperm from the testes to the penis.
4omen who have CF may have a hard time getting pregnant because of mucus bloc!ing the
cervi) or other CF complications.
Other Signs, Symptoms, and Complications
Other signs and symptoms of CF are related to an upset of the balance of minerals in your blood.
CF causes your sweat to become very salty. #s a result your body loses large amounts of salt
when you sweat. This can cause dehydration (a lac! of fluid in your body) increased heart rate
fatigue (tiredness) wea!ness decreased blood pressure heat stro!e and rarely death.
CF also can cause clubbing and low bone density. Clubbing is the widening and rounding of the
tips of your fingers and toes. This sign develops late in CF because your lungs aren't moving
enough o)ygen into your bloodstream.
1ow bone density also tends to occur late in CF. 't can lead to a bone%thinning disorder called
osteoporosis (OS%te%o%po%,O%sis).