Download - Cystic Fibrosis
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Fitango EducationHealth Topics
Cystic Fibrosis
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Overview
Mucus is a substance made by tissues that
line some organs and body cavities, such as the lungs and nose. Normally, mucus
is a slippery, watery substance. It keeps the linings of certain organs moist
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Overview
and prevents them from drying out or getting infected.
If you have CF, your mucus becomes thick
and sticky. It builds up in your lungs and blocks your airways. (Airways are
tubes that carry air in and out of your lungs.)
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Overview
The buildup of mucus makes it easy for
bacteria to grow. This leads to repeated, serious lung infections. Over time,
these infections can severely damage your lungs.
The thick, sticky mucus also can block
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Overview
tubes, or ducts, in your pancreas (an organ in your abdomen). As a result, the
digestive enzymes that your pancreas makes can't reach your small intestine.
These enzymes help break down food. Without
them, your intestines can't fully absorb fats and proteins. This can cause
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Overview
vitamin deficiency and malnutrition because nutrients pass through your body
without being used. You also may have bulky stools, intestinal gas, a swollen
belly from severe constipation, and pain or discomfort.
CF also causes your sweat to become very
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Overview
salty. Thus, when you sweat, you lose large amounts of salt. This can upset the
balance of minerals in your blood and cause many health problems. Examples of
these problems include dehydration (a lack of fluid in your body), increased
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Overview
heart rate, fatigue (tiredness), weakness, decreased blood pressure, heat stroke,
and, rarely, death.
If you or your child has CF, you're also at
higher risk for diabetes or a bone-thinning condition called osteoporosis
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Overview
(OS-te-o-po-RO-sis).
CF also causes infertility in men, and the
disease can make it harder for women to get pregnant. (The term
"infertility" refers to the inability to have children.)
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Causes
A defect in the CFTR gene causes cystic
fibrosis (CF). This gene makes a protein that controls the movement of salt and
water in and out of your body's cells. In people who have CF, the gene makes a
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Causes
protein that doesn't work well. This causes thick, sticky mucus and very salty
sweat.
Research suggests that the CFTR protein
also affects the body in other ways. This may help explain other symptoms and
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Causes
complications of CF.
More than a thousand known defects can
affect the CFTR gene. The type of defect you or your child has may affect the
severity of CF. Other genes also may play a role in the severity of the
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Causes
disease.
**How Is Cystic Fibrosis Inherited?**
Every person inherits two CFTR genes—one
from each parent. Children who inherit a faulty CFTR gene from each parent will
have CF.
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Causes
Children who inherit one faulty CFTR gene
and one normal CFTR gene are "CF carriers." CF carriers usually have
no symptoms of CF and live normal lives. However, they can pass the faulty CFTR
gene to their children.
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Causes
The image below shows how two parents who
are both CF carriers can pass the faulty CFTR gene to their children.
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Symptoms
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.
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Symptoms
One of the first signs of CF that parents
may notice is that their baby's skin tastes salty when kissed, or the baby
doesn't pass stool when first born.
Most of the other signs and symptoms of CF
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Symptoms
happen later. They're related to how CF affects the respiratory, digestive, or
reproductive systems of the body.
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Treatment
Cystic fibrosis (CF) has no cure. However,
treatments have greatly improved in recent years. The goals of CF treatment
include:
-- Preventing and controlling lung infections
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Treatment
-- Loosening and removing thick, sticky mucus
from the lungs
-- Preventing or treating blockages in the
intestines
-- Providing enough nutrition
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Treatment
-- Preventing dehydration (a lack of fluid in
the body)
-- Depending on the severity of CF, you or
your child may be treated in a hospital.
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Treatment**Specialists Involved**
If you or your child has CF, you may be
treated by a CF specialist. This is a doctor who is familiar with the complex
nature of CF.
Often, a CF specialist works with a medical
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Treatment**Specialists Involved**
team of nurses, physical therapists, dietitians, and social workers. CF
specialists often are located at major medical centers.
The United States also has more than 100 CF
Care Centers. These centers have teams of doctors, nurses, dietitians,
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Treatment**Specialists Involved**
respiratory therapists, physical therapists, and social workers who have
special training related to CF care. Most CF Care Centers have pediatric and
adult programs or clinics.
For more information about CF Care Centers,
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Treatment**Specialists Involved**
go to the Cystic Fibrosis Foundation's Care Center Network
Web page.
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Treatment**Treatment for Lung Problems**
The main treatments for lung problems in
people who have CF are chest physical therapy (CPT), exercise, and medicines.
Your doctor also may recommend apulmonary
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Treatment**Treatment for Lung Problems**
rehabilitation (PR) program.
Chest Physical Therapy
CPT also is called chest clapping or
percussion. It involves pounding your chest and back over and over with your
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Treatment**Treatment for Lung Problems**
hands or a device to loosen the mucus from your lungs so that you can cough it
up.
You might sit down or lie on your stomach
with your head down while you do CPT. Gravity and force help drain the mucus
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Treatment**Treatment for Lung Problems**
from your lungs.
Some people find CPT hard or uncomfortable
to do. Several devices have been developed that may help with CPT, such as:
An electric chest clapper, known as a
mechanical percussor.
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Treatment**Treatment for Lung Problems**
An inflatable therapy vest that uses
high-frequency airwaves to force the mucus that's deep in your lungs toward
your upper airways so you can cough it up.
A small, handheld device that you exhale
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Treatment**Treatment for Lung Problems**
through. The device causes vibrations that dislodge the mucus.
A mask that creates vibrations that help
break the mucus loose from your airway walls.
Breathing techniques also may help dislodge
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Treatment**Treatment for Lung Problems**
mucus so you can cough it up. These techniques include forcing out a couple of
short breaths or deeper breaths and then doing relaxed breathing. This may help
loosen the mucus in your lungs and open your airways.
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Treatment**Exercise**
Aerobic exercise that makes you breathe
harder can help loosen the mucus in your airways so you can cough it up.
Exercise also helps improve your overall physical condition.
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Treatment**Exercise**
However, CF causes your sweat to become
very salty. As a result, your body loses large amounts of salt when you sweat.
Thus, your doctor may recommend a high-salt diet or salt supplements to
maintain the balance of minerals in your blood.
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Treatment**Exercise**
If you exercise regularly, you may be able
to cut back on your CPT. However, you should check with your doctor first.
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Treatment**Medicines**
If you have CF, you doctor may prescribe
antibiotics, anti-inflammatory medicines, bronchodilators, or mucus-thinning
medicines. These medicines help treat or prevent lung infections, reduce
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Treatment**Medicines**
swelling, open up the airways, and thin mucus.
Antibiotics are the main treatment to
prevent or treat lung infections. Your doctor may prescribe oral, inhaled, or
intravenous (IV) antibiotics.
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Treatment**Medicines**
Oral antibiotics often are used to treat
mild lung infections. Inhaled antibiotics may be used to prevent or control
infections caused by the bacteria mucoidPseudomonas. For severe or
hard-to-treat infections, you may be given antibiotics through an IV tube (a
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Treatment**Medicines**
tube inserted into a vein). This type of treatment may require you to stay in a
hospital.
Anti-inflammatory medicines can help reduce
swelling in your airways due to ongoing infections. These medicines may be
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Treatment**Medicines**
inhaled or oral.
Bronchodilators help open the airways by
relaxing the muscles around them. These medicines are inhaled. They're often
taken just before CPT to help clear mucus out of your airways. You also may
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Treatment**Medicines**
take bronchodilators before inhaling other medicines into your lungs.
Your doctor may prescribe mucus-thinning
medicines to reduce the stickiness of your mucus and loosen it up. These
medicines can help clear out mucus, improve lung function, and prevent
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Treatment**Medicines**
worsening lung symptoms.
**Treatments for Advanced Lung Disease**
If you have advanced lung disease, you may
need oxygen
therapy. Oxygen usually is given through nasal prongs or a mask.
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Treatment**Medicines**
If other treatments haven't worked, a lung
transplant may be an option if you have severe lung disease. A lung
transplant is surgery to remove a person's diseased lung and replace it with a
healthy lung from a deceased donor.
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Treatment**Pulmonary Rehabilitation**
Your doctor may recommend PR as part of
your treatment plan. PR is a broad program that helps improve the well-being of
people who have chronic (ongoing) breathing problems.
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Treatment**Pulmonary Rehabilitation**
PR doesn't replace medical therapy.
Instead, it's used with medical therapy and may include:
-- Exercise training
-- Nutritional counseling
-- Education on your lung disease or condition
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Treatment**Pulmonary Rehabilitation**
and how to manage it
-- Energy-conserving techniques
-- Breathing strategies
-- Psychological counseling and/or group
support
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Treatment**Pulmonary Rehabilitation**
PR has many benefits. It can improve your
ability to function and your quality of life. The program also may help relieve
your breathing problems. Even if you have advanced lung disease, you can still
benefit from PR.
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Treatment**Pulmonary Rehabilitation**
For more information, go to the Health
Topics Pulmonary
Rehabilitation article.
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Treatment**Treatment for Digestive Problems**
CF can cause many digestive problems, such
as bulky stools, intestinal gas, a swollen belly, severe constipation, and pain
or discomfort. Digestive problems also can lead to poor growth and development
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Treatment**Treatment for Digestive Problems**
in children.
Nutritional therapy can improve your
strength and ability to stay active. It also can improve growth and development
in children. Nutritional therapy also may make you strong enough to resist some
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Treatment**Treatment for Digestive Problems**
lung infections. A nutritionist can help you create a nutritional plan that
meets your needs.
In addition to having a well-balanced diet
that's rich in calories, fat, and protein, your nutritional therapy may
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Treatment**Treatment for Digestive Problems**
include:
Oral pancreatic enzymes to help you digest
fats and proteins and absorb more vitamins.
Supplements of vitamins A, D, E, and K to
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Treatment**Treatment for Digestive Problems**
replace the fat-soluble vitamins that your intestines can't absorb.
High-calorie shakes to provide you with
extra nutrients.
A high-salt diet or salt supplements that
you take before exercising.
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Treatment**Treatment for Digestive Problems**
A feeding tube to give you more calories at
night while you're sleeping. The tube may be threaded through your nose and
throat and into your stomach. Or, the tube may be placed directly into your
stomach through a surgically made hole. Before you go to bed each night, you'll
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Treatment**Treatment for Digestive Problems**
attach a bag with a nutritional solution to the entrance of the tube. It will
feed you while you sleep.
Other treatments for digestive problems may
include enemas and mucus-thinning medicines to treat intestinal blockages.
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Treatment**Treatment for Digestive Problems**
Sometimes surgery is needed to remove an intestinal blockage.
Your doctor also may prescribe medicines to
reduce your stomach acid and help oral pancreatic enzymes work better.
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Treatment**Treatments for Cystic Fibrosis
Complications**
A common complication of CF is diabetes
. The type of diabetes associated with CF often requires different
treatment than other types of diabetes.
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Treatment**Treatments for Cystic Fibrosis
Another common CF complication is the bone-thinning
disorder osteoporosis. Your doctor may prescribe medicines that prevent your
bones from losing their density.
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