Treatment for  Cystic Fibrosis

An Overview of Cystic Fibrosis Treatment 

Although there is  still no cure for cystic fibrosis (CF), treatment has  improved greatly in recent years.
 
The goal of cystic fibrosis  treatment is 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 Cystic Fibrosis  Lung Problems

Common  treatments for lung problems seen in cystic fibrosis may include:

• Antibiotics for infections of the airways
• Chest physical therapy
• Exercise
• Other methods. 
• Other treatments for lung problems may include: 
• Oxygen
• Lung transplantation. 

Antibiotics
Most people with cystic fibrosis  have ongoing, low-grade lung infections, which  may require hospitalization.  Antibiotics are the primary treatment for cystic fibrosis. 
 
If you have cystic fibrosis, you  may be given several different types of antibiotics. The type of antibiotic your doctor recommends will depend  on:

• The strains of bacteria involved
• How serious your condition is
• Your previous history of antibiotic use.
• The different types of antibiotics used as cystic fibrosis treatments  include:
• Oral antibiotics for relatively mild airway infections
• Inhaled antibiotics, such as tobramycin, which  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 (Zithromax®), that also reduce inflammation.

Chest Physical Therapy 

Chest physical therapy as treatment for cystic fibrosis  is also called chest clapping or percussion. Chest physical therapy  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. Chest physical therapy  for cystic fibrosis should be done three to four times each day.
 
Chest physical therapy is also often referred to as postural drainage because it requires you to be  sitting or lying on your stomach with your head down while you do chest physical therapy.  This allows gravity to help drain the mucus from your lungs.
 
Chest physical therapy may be hard or uncomfortable for some people, which is why  several helpful devices have been developed.  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, which is  a small hand-held device through which  you breathe out.  It causes vibrations that dislodge the mucus.
• A positive expiratory pressure (PEP) mask that creates vibrations. This helps to  break the mucus loose from the airway walls.

Several breathing techniques may also help dislodge the mucus. These techniques include:

Forced expiration technique (FET), which is forcing  out a couple of breaths or huffs and then doing relaxed breathing?

Active cycle breathing (ACB), which is FET  with deep breathing exercises that can loosen the mucus in your lungs and help open your airways.
 
Exercise
Aerobic exercise may help to: 

• 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 physical therapy. However, you should check  with your doctor before using exercise as a cystic fibrosis treatment. 

Other Medications 

Anti-inflammatory medications may help reduce the inflammation in your lungs that is caused by ongoing infections. Medications  include:

• Inhaled or, sometimes, oral steroids. Steroids are the most effective anti-inflammatory medicines.
• Ibuprofen, a type of non-steroidal, anti-inflammatory medicine. It may slow the progress of cystic fibrosis  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 chest physical therapy  to help clear mucus.
• Mucus-thinning drugs, which  reduce the stickiness of mucus in your airways.

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

Lung transplantation requires surgery to replace one or both of your lungs with healthy lungs from a human donor. 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.

Nutritional Treatment for Cystic Fibrosis 

• Nutritional therapy as a cystic fibrosis treatment  can improve a person's: 
• Growth  and development 
• Strength  

             .           Exercise  tolerance.
 
Nutritional therapy  may also make you strong enough to resist some lung infections. Nutritional therapy includes a well-balanced, high-calorie diet that is low in fat and high in 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 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 and it  feeds you while you sleep.
  

Other treatment for digestive problems may include enemas  and mucus-thinning medications to treat intestinal blockages. Your doctor may also recommend medicines  that reduce stomach acid and help the oral pancreatic enzymes work better. 

Notes: Dr. M. Kristine Schlossberg

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EditText of this page (last edited February 27, 2010)