Cystic Fibrosis is the second most common
life-shortening, childhood onset inherited disorder in the United States, behind
sickle cell anemia. Approximately
30,000 people in the United States have Cystic Fibrosis (CF).
fibrosis (CF) is a chronic and
progressive disease usually diagnosed in childhood that causes mucus
to become thick, dry, and sticky. The mucus builds up and clogs passages in many
of the body's organs, but primarily the lungs
and the pancreas.
In the lungs, the mucus can lead to serious breathing problems and lung disease.
In the pancreas, the mucus can lead to malnutrition and problems with growth and
People with CF have an average life expectancy of about 32 years, although
new treatments offer hope for longer and healthier lives.
Cystic fibrosis occurs most commonly among
whites. It is estimated that 1 in 3,200 white births are affected in
comparison to 1 in 10,500 Native Americans, 1 in 11,500 Hispanics, 1 in
14,000 to 17,000 African Americans, and 1 in 25,500 Asians
More than 10 million Americans are unknowing,
symptomless carriers of the defective CF gene. An individual must
inherit two defective CF genes -- one from each parent -- to have CF.
Each time two carriers conceive, there is a 25 percent chance that their
child will have CF; a 50 percent chance that the child will be a carrier
of the CF gene; and a 25 percent chance that the child will be a
The most common symptoms of CF include very
salty-tasting skin, persistent coughing, wheezing or shortness of
breathing, an excessive appetite but poor weight gain and greasy, bulky
stools. Respiratory infections increase with age.
Nearly 1,000 new cases of the disease are
diagnosed in the United States each year. Over 80 percent of CF cases are
diagnosed by the age of three; however 10 percent of new cases are
diagnosed in those over 18.The sweat
test is the standard diagnostic test for CF.
The treatment of CF depends upon the stage of
the disease and the organs involved. Chest physical therapy to clear
mucus from the lungs is an important part of the daily CF treatment
regimen. Other types of treatments include TOBI, an aerosolized
antibiotic used to treat lung infections; Pulmozyme, a mucus-thinning drug
shown to reduce the number of lung infections and improve lung function;
and azithromycin, an antibiotic that improves the lung function of people
with CF whose lungs are chronically infected with Pseudomonas aeruginosa
bacteria, the most common cause of respiratory infection in people with
People with cystic fibrosis have a problem with the way salt�sodium and
chloride�and water move in and out of mucus-producing cells. Normally, salt
and water move easily across cells, keeping mucus>
secretions thin and fluid, lubricating organs and keeping the lungs and air
tubes clean by carrying off germs and dust. The defect in the CFTR gene causes
the mucus to become dry, thick, and sticky. The mucus can clog organ passages,
hinder organ secretions, and attract bacteria. The symptoms and complications of
CF are largely a result of this abnormally thick mucus.
Carriers of CF may be at higher risk for some
mild complications, such as inflammation of the pancreas (pancreatitis)
Symptoms of CF are usually caused by the production of thick, sticky mucus.
Symptoms vary from person to person and are not always present at birth; in some
people, symptoms may be very mild and not be noticed or develop until later in
childhood or early adulthood.
Early signs and symptoms
Symptoms of CF in a baby or young child may
To Call a Doctor
- A blockage of the small intestine at birth,
called a meconium
plug or meconium ileus. This blockage affects approximately 17% of
newborns with CF.
- Salty sweat or skin, a classic sign of CF.
- Poor appetite, lack of energy, and weight loss
during infancy, sometimes called failure
to thrive. Usually this occurs if a mucus blockage prevents digestive
enzymes produced in the pancreas
from reaching the intestines, where they are needed for digestion. Pancreatitis
may be the first symptom in some children and adults.
- Irregular bowel movements. This may include
persistent diarrhea; large, greasy stools; or constipation. Stools may be
unusually bad-smelling. If the intestines become blocked, the child may have
a bloated stomach and may not be able to have a bowel movement.
- Difficulty breathing and respiratory system
infections. Children with CF may have frequent infections in the lungs,
nose, and sinuses, such as bronchitis
A child may tire easily while playing.
- Persistent cough and wheezing. These symptoms
may appear along with respiratory system infections and last longer than
normal in children with CF. Coughing is usually worse at night and when
waking up in the morning.
Report any symptoms of cystic
fibrosis (CF) to your health professional right away. The risk for
developing permanent lung damage is reduced if CF is promptly diagnosed and
Call a health professional if your child:
- Has frequent lung infections (pneumonia),
colds, an ongoing cough, shortness of breath, or wheezing.
- Coughs up mucus that contains blood.
- Does not gain or maintain weight despite
- Has foul-smelling, large, greasy stools or
- Has salty-tasting skin or sweat.
- Tires easily during activity.
- Has rounded, flat fingertips (clubbing).
Call your health professional if your child
diagnosed with CF experiences even slightly worsening symptoms. Typically, this
is when your child:
- Has increased or worsening coughing.
- Has new or worsening wheezing.
- Experiences breathing difficulties with less
exertion or otherwise worsening lung function.
- Has unexplained weight loss or failure to gain
weight as expected.
- Experiences any symptoms or complications that
you and your doctor have earlier established as requiring attention.
Source: Healthwise, Wrong Diagnosis.com and American Lung Association
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