VCU Adult Cystic Fibrosis Program

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 reduce symptoms and improve quality of life by:
  • Preventing and controling infections in your lungs.
  • Loosening and removing the thick, sticky mucus from your lungs.
  • Preventing blockages in your intestines.
  • Providing adequate nutrition.

Below you will find information on the following CF Treatments:

 

Small Molecule Therapies

Recently small molecule therapies have become available for a small subset of the patient population with CF in which a drug acts at basic defect in CF and improves hydration of secretions resulting in substantial improvement of quality of life. More research is ongoing to investigate these drugs for a large group of CF patients.

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Treatment for Lung Problems

  • The main treatments for lung problems in people with CF are:
  • Antibiotics for infections of the airways
  • Chest Physical Therapy (CPT)
  • Exercise
  • Other medications

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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. Sometimes it may be possible to receive intra-venous (IV) antibiotics at home.

  • 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), Aztreonam (AZ-treo-nam) and Colistin (co- LIS-tin). They may be used alone or with oral antibiotics.
  • Intravenous antibiotics for severe infections or when none of the oral antibiotics work.
  • Azithromycin (az-ith-roe-MYE-sin) that reduces inflammation.

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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, especially in asthma.
  • 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.
  • Some of the other inhaled medications can cause some patients to experience breathing problems. Bronchodilators should be taken prior to other inhaled agents to minimize the chance of breathing difficulties.
  • 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.
  • Hypertonic Saline
    A solution of very salty sterile water taken by nebulizer two times a day can help clear mucus and improve lung function.

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Chest Physical Therapy (CPT)

Chest Physical Therapy 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 sitting or lying on your stomach 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) devise that creates back pressure that helps hold airways open, making it easier to clear mucus 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.
  • Autogenic Drainage
    A breathing and cough control technique that can be very effective as a secretion clearance. It does require instruction and practice to perfect the technique

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Exercise

  • Aerobic exercise and strength training help:
  • Loosen the mucus
  • Encourage coughing to clear the mucus
  • Improve your overall physical condition
  • Improve lung function

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 (cannula) or a mask.

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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, 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 (gas-TROS-to-me) tube or G-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.
  • High calorie nutritional supplements like Ensure.
  • 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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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

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