How Is Cystic Fibrosis Treated?
Cystic fibrosis (CF) has no cure. However,
treatments have greatly improved in recent years. The goals of CF treatment are
to:
- Prevent and control lung infections
- Loosen and remove thick, sticky mucus from the
lungs
- Prevent or treat blockages in the intestines
- Provide enough nutrition
- Prevent dehydration (a condition in which the
body doesn't have enough fluids)
Depending on how severe the disease is, you or your
child may be treated in a hospital.
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 team of
nurses, physical therapists, dietitians, and social workers. CF specialists
often are located at major medical centers.
Treatment for Lung Problems
The main treatments for lung problems in people who
have CF are chest physical therapy (CPT), exercise, and medicines.
Chest Physical Therapy
CPT also is called chest clapping or percussion. It
involves pounding your chest and back over and over with your 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 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.
- 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 breathe out
through. It 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 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.
Exercise
Aerobic exercise that makes you breathe harder helps
loosen the mucus in your airways so you can cough it up. Exercise also helps
improve your overall physical condition.
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.
If you exercise regularly, you may be able to cut
back on your CPT. However, you should check with your doctor before doing
this.
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
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.
Oral antibiotics often are used to treat mild lung
infections. Inhaled antibiotics may be used to prevent or control infections
caused by the bacteria mucoid Pseudomonas. For severe or hard-to-treat
infections, you may be given antibiotics through a tube inserted into a vein.
This type of treatment may require you to stay in the hospital.
Anti-inflammatory medicines can help reduce swelling
in your airways that's caused by ongoing infections. These medicines may be
inhaled or oral.
Bronchodilator medicines help open the airways by
relaxing the muscles around them. These medicines are inhaled and often are
taken just before CPT to help clear out mucus. You also may take
bronchodilators before inhaling other medicines into your lungs.
Your doctor may prescribe mucus thinners to reduce
the stickiness of your mucus and to loosen it up. These medicines can help
clear out mucus, improve lung function, and prevent worsening lung
symptoms.
Treatments for Advanced Lung Disease
If you have advanced lung disease and the level of
oxygen in your blood is low, you may need oxygen therapy. Oxygen usually is
given through nasal prongs or a mask.
If other treatments haven't worked,
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.
Treatment for Digestive Problems
CF can cause a number of digestive problems,
including poor growth and development, bulky stools, intestinal gas, a swollen
belly, severe constipation, and pain or discomfort.
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 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 include:
- Oral pancreatic enzymes to help you digest fats
and proteins and absorb more vitamins.
- Supplements of vitamins A, D, E, and K to replace
the fat-soluble vitamins that your intestines can't absorb.
- High-calorie shakes to provide you with
additional nutrients.
- A high-salt diet or salt supplements that you
take before doing vigorous exercise.
- 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, it may be placed directly into your stomach through
a surgically made hole. Before you go to bed each night, you will 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. 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.
Treatments for Cystic Fibrosis Complications
A common complication of CF is diabetes. The type of
diabetes that people who have CF develop often requires different treatment
than other types of diabetes.
Another common complication is the bone-thinning
disorder osteoporosis. Your doctor may prescribe medicines that prevent your
bones from losing their density. |