How Is Cystic Fibrosis Treated?
There still is no cure for cystic fibrosis (CF), but
treatments have improved greatly in recent years. The goals of CF treatment are
to:
- Prevent and control infections in your
lungs.
- Loosen and remove the thick, sticky mucus from
your lungs.
- Prevent blockages in your intestines.
- Provide adequate nutrition.
Treatment for Lung Problems
The main treatments for lung problems in people with
CF are:
- Antibiotics for infections of the airways
- Chest physical therapy
- Exercise
- Other medications
Antibiotics
Most people with CF have ongoing, low-grade lung
infections. Sometimes, these infections become so serious that you may need to
be hospitalized. Antibiotics are the primary treatment.
You may be given several different types of
antibiotics. The choice of antibiotics depends on:
- The strains of bacteria involved
- How serious your condition is
- Your previous history of antibiotic use
The different types of antibiotics include:
- Oral antibiotics for relatively mild airway
infections.
- Inhaled antibiotics, such as tobramycin
(to-bra-MI-sin). They may be used alone or with oral antibiotics.
- Intravenous antibiotics for severe infections or
when none of the oral antibiotics work.
- Antibiotics, such as azithromycin
(az-ith-roe-MYE-sin), that also reduce inflammation.
Chest physical therapy
Chest physical therapy (CPT) is also called chest
clapping or percussion. It involves pounding your chest and back over and over
again to dislodge the mucus from your lungs so that you can cough up the mucus.
CPT for cystic fibrosis should be done three to four times each day.
CPT is also often referred to as postural drainage.
This involves your sitting or lying on your stomach with your head down while
you do CPT. This allows gravity to help drain the mucus from your lungs.
Because CPT is hard or uncomfortable for some
people, several devices have been developed recently that may help with CPT.
The devices include:
- An electric chest clapper, known as a mechanical
percussor.
- An inflatable therapy vest that uses
high-frequency air waves to force the mucus out of your lungs.
- A "flutter" device, a small hand-held device that
you breathe out through. It causes vibrations that dislodge the mucus.
- A positive expiratory pressure (PEP) mask that
creates vibrations that help break the mucus loose from the airway walls.
Several breathing techniques may also help dislodge
the mucus. These techniques include:
- Forced expiration technique (FET)—forcing
out a couple of breaths or huffs and then doing relaxed breathing
- Active cycle breathing (ACB)—FET with deep
breathing exercises that can loosen the mucus in your lungs and help open your
airways
Exercise
Aerobic exercise helps:
- Loosen the mucus
- Encourage coughing to clear the mucus
- Improve your overall physical condition
If you exercise regularly, you may be able to cut
back on your chest therapy. Check with your doctor before doing this.
Other medications
Anti-inflammatory medications may help reduce the
inflammation in your lungs that is caused by ongoing infections. These
medications include:
- Inhaled or, sometimes, oral steroids. Steroids
are the most effective anti-inflammatory medicines.
- Ibuprofen, a type of nonsteroidal,
anti-inflammatory medicine. It may slow the progress of CF in young children
with mild symptoms.
- Bronchodilators, which are inhaled drugs that
relax the muscles around the airways so that the airways can open up. They
should be taken just before CPT to help clear mucus.
Mucus-thinning drugs reduce the stickiness of mucus
in your airways. They include:
- Human DNase (Dornase Alfa), a drug that loosens
the mucus in your lungs. It may lead to shorter hospital stays.
- Acetylcysteine and saline.
- Hypertonic saline, a solution of very salty
sterile water taken by nebulizer two times a day, can help clear mucus and
improve lung function. Some doctors are now giving it to select patients over 6
years old.
Oxygen Therapy
If the level of oxygen in your blood is too low, you
may need oxygen therapy. Oxygen is usually given through nasal prongs or a
mask.
Lung Transplantation
Surgery to replace one or both of your lungs with
healthy lungs from a human donor may help you. Some of the factors that
determine whether you can undergo
lung transplantation include:
- The type of bacteria in your lungs
- Your age and weight
- The medications you are taking
- Whether you have other medical conditions,
including osteoporosis
- How well your lungs are functioning
Management of Digestive Problems
Nutritional therapy can improve your growth and
development, strength, and exercise tolerance. It may also make you strong
enough to resist some lung infections. Nutritional therapy includes a
well-balanced diet that is rich in calories, fat, and protein.
As part of your nutritional therapy, your doctor
may:
- Prescribe oral pancreatic enzymes to help you
digest fats and proteins and absorb more vitamins. The enzymes should be taken
in capsule form before every meal, including snacks.
- Recommend supplements of vitamins A, D, E, and K
to replace the fat-soluble vitamins that your intestines cannot absorb.
- Recommend that you use a feeding tube, called a
gastrostomy (gas-TROS-to-me) tube or T-tube, to add more calories at night
while you are sleeping. The tube is placed in your stomach. Before you go to
bed each night, you attach a bottle with a nutritional solution to the entrance
of the tube. It feeds you while you sleep.
Other treatments for the digestive problems caused
by CF may include:
- Enemas and mucus-thinning medications to treat
intestinal blockages
- Medicines that reduce stomach acid and help the
oral pancreatic enzymes work better
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