Most CF patients eventually die, accumulation of sticky, thick, slimey mucus in the lungs
- From: "Jason P" <jaspetr@xxxxxxxxx>
- Date: Wed, 10 Oct 2007 07:45:00 -0700
Cystic Fibrosis
Signs and Symptoms
Physician developed and monitored.
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Original source: www.pulmonologychannel.com
Original Date of Publication: 01 Jun 2000
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Signs and Symptoms
CF develops in most patients in the first year of life. Usually at least
some symptoms are present at birth, although sometimes symptoms do not
appear until adolescence.About 3% of all CF patients are not diagnosed until
adulthood.
The lungs and pancreas produce the most common symptoms, usually respiratory
and gastrointestinal complications. Most CF patients eventually die from
advanced lung disease. CF also affects, to a lesser extent, the reproductive
tract and the sweat glands.
Respiratory Symptoms
Almost all CF patients begin to experience respiratory symptoms within the
first couple of years of life. They include:
a.. Repeated infections: The accumulation of sticky, thick mucus in the
lungs creates a favorable environment for infectious microorganisms to
inhabit and flourish.
b.. Chronic cough: Patients with CF often have a persistent cough that
results from repeated respiratory infections. Over time, patients begin
coughing up a viscous (thick, sticky), purulent (pus-filled), green-colored
sputum.
c.. Recurrent bronchitis: The repeated infections that afflict the lungs
of CF patients often lead to bronchitis, inflammation and swelling of the
bronchi that obstructs the airways and stimulates the production of mucus.
The bronchitic mucus creates yet more obstruction and further increases the
chances of infection.
d.. Recurrent pneumonia: Pneumonia is a lung infection that can be caused
by a number of microorganisms. Patients with CF are especially susceptible
to pneumonial infections because the abnormal mucus and reduced water
content in the airways promotes bacterial growth.
e.. Bronchiectasis: Continual respiratory problems can eventually lead to
bronchiectasis and permanent lung damage, which is the usual cause of death
for CF patients. Bronchiectasis is the chronic dilation of the airways. The
enlarged airways become filled with mucus, which makes them prone to more
infection, enlarging and damaging them even more. The damage caused by
bronchiectasis is permanent, and patients usually cough up a great deal of
infected mucus.
f.. Pneumothorax: Pneumothorax is the trapping of gas or air between the
lung and the chest wall that occurs as a result of a leaky lung or rupture.
More than 10% of all CF patients experience pneumothorax.
g.. Hemoptysis: Hemoptysis is the coughing up of blood and is a symptom of
many different underlying disorders, including cystic fibrosis. Minor
hemoptysis is common in CF patients with advanced lung disease.
h.. Digital clubbing: Digital clubbing is enlargement and bulbous, shiny
appearance of the ends of the fingers and toes. It occurs in nearly all CF
patients who have advanced lung disease.
i.. Cor pulmonale: Cor pulmonale is an enlargement of the right side of
the heart caused by a high resistance to blood flow through the lungs. It is
often a prominent feature in patients who have advanced lung disease and
leads to heart disease.
j.. Sinusitis: Sinusitis is inflammation of the nasal sinuses. The sinuses
are hollow cavities inside the skull that produce mucus and help keep the
nasal passageway moist. Chronic sinusitis is common in children with CF and
can lead to nasal obstruction and a runny nose.
k.. Allergic bronchopulmonary aspergillosis: Sputum is the substance that
is expelled when someone coughs or clears his or her throat. Nearly half of
all CF patients have a fungus known as Aspergillus fumigatus in their
sputum, and about 10% develop a condition known as allergic bronchopulmonary
aspergillosis. Aspergillosis is an infection in the bronchi or lungs that
can lead to the formation of inflammatory lesions.
l.. Nasal polyps (nasal polyposis): Nasal polyps are fleshy growths inside
the nose. Nasal polyposis occurs in about 15% to 20% of all CF patients and
often requires surgery.
Gastrointestinal Complications
In CF patients, nearly all of the pancreatic tissue is destroyed because of
the disturbed ion transport, creating a multitude of problems related to
pancreatic dysfunction. Symptoms related to pancreatic insufficiency,
especially poor weight gain and bulky, foul-smelling stools, are experienced
in over 90% of all CF patients.
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In the intestines, the epithelial cells are not able to flush away all of
the various secretions and macromolecules from the intestinal crypts (pits
in the intestinal epithelium), eventually causing obstruction in both the
small and large intestines.
Pancreatic complications and intestinal obstruction can manifest in a
variety of ways, including:
a.. Poor weight gain and growth, also known as "failure to thrive": Babies
and children with CF have a difficult time gaining weight, even those with a
hearty appetite, because the pancreas is not absorbing and digesting fats
and fat-soluble vitamins (vitamins A, D, E, and K).
b.. Greasy, foul-smelling stools (steatorrhea): All of the fat that the
pancreas is not adequately digesting and absorbing is passed out of the body
in the stools, which take on a greasy appearance and smell very bad.
Fat-soluble vitamins are flushed out of the body in the stools, and in
severe cases, a patient can suffer complications due to fat-soluble vitamin
deficiency (e.g., visual disturbances due to a lack of vitamin A).
c.. Meconium ileus: Meconium is the term given to a newborn's first
stools, which are usually thick, sticky, dark green, and odorless. Ileus
refers to an obstruction of the intestines. In babies with meconium ileus,
the bowel is blocked with very thick meconium resulting from a deficiency of
enzymes, including trypsin, that are normally secreted amply by the
pancreas. Meconium ileus occurs in about 10% of all newborns with CF. Its
symptoms include emesis (vomiting) and the inability to pass stool.
d.. Distal intestinal obstruction: Distal intestinal obstruction, also
known as meconium ileus equivalent, is a syndrome that occurs frequently in
children and young adults with CF and, because of its symptoms, is sometimes
confused with appendicitis. Symptoms include pain in the right lower
quadrant, a loss of appetite, and emesis (vomiting). Distal intestinal
obstruction results from blockage by poorly digested fats (and proteins)
which have clumped into a mass of firm sticky material.
e.. Hyperglycemia: Hyperglycemia is a high blood sugar that results when
the body does not have enough insulin to convert sugar (specifically
glucose) into energy. Patients with hyperglycemia usually are very thirsty,
have a dry mouth, and need to urinate often. The pancreas is the organ
responsible for producing insulin. Only a small percentage of CF patients
develop hyperglycemia, and if they do, it usually does not appear until they
are in their 20s or 30s.
f.. Abdominal discomfort: The belly is usually swollen with gas and the
patient feels abdominal discomfort.
g.. Rectal prolapse: Rectal prolapse is a condition in which the lining of
the rectum pushes out through the anus. It usually occurs during a bowel
movement. This is a well-recognized but rare complication in young children
with CF.
In the hepatobiliary system (the liver and the secretions from the liver),
which is damaged in about 25% to 30% of all CF patients, malfunctioning
secretion can cause liver disease and gallstones, leading to pain and
jaundice (yellowing of the skin):
a.. liver disease (liver cirrhosis): Liver complications include a fatty
liver, which usually doesn't create problems except in infants; focal
biliary fibrosis, which causes the liver to first enlarge and then shrink
like a typical cirrhotic liver - this is caused by blockage of the bile
ducts, which leads to inflammation, which leads to fibrosis; and development
of cholesterol gallstones.
b.. prolonged natal jaundice: Prolonged natal jaundice, also known as
prolonged obstructive jaundice, is a yellowing of the skin and mucous
membranes caused by excessive amounts of bile pigments in the blood.
Normally, bile pigments are filtered through the liver and excreted in the
feces. When the ducts connecting the liver to the intestines are blocked,
obstructive jaundice develops.
Reproductive Tract Complications
Reproductive tract abnormalities that people with CF often experience
include:
a.. Delayed puberty: Puberty is often delayed a couple of years in both
boys and girls with CF. The delay is probably due to nutritional
inadequacies.
b.. Male infertility: Azoospermia is the absence of sperm in the semen.
More than 95% of men with CF are azoospermic due to an obliterated vas
deferens (the tube in the male reproductive tract that carries the sperm
from each testis to the urethra).
c.. Female fertility complications: About 20% of women with CF are
infertile. Female infertility is due to the effects of chronic lung disease
on the menstrual cycle, as well as a thick cervical mucus that blocks the
migration of sperm.
Sweat Gland Complications
Patients with CF secrete normal amounts of sweat, but the sweat has
abnormally high levels of salt (NaCl) due to the malfunctioning Cl-
transport, causing very salty skin. Indeed, the symptom is nearly universal
and the sweat test is used as the number one diagnostic test for CF. Only
about 1% to 2% of all people with CF show normal sweat test results.
The imbalance of salt in the sweat can lead to abnormal heart rhythms.
.
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