Treatment

Unfortunately, Cystic Fibrosis is not curable at this time. However, a great amount of time, energy, and money is being directed towards finding new improved ways of treating CF and of finally finding a cure. At this time physicians are only able to treat the symptoms and complications of the disease. The treatment of CF depends upon the stage of the disease and which organs are involved. Many pharmacological agents and therapeutic interventions are available to improve most people with Cystic Fibrosis. These interventions help CF patients to lead normal and productive lives.

Cystic Fibrosis used to be known as the children’s disease because a person with CF never survived to be an adult. However, with increasing knowledge about the disease and with more effective treatments, the median survival age increased from 8 years to the 30’s.

Pharmacologic Agents:

• Mucolytic agents: Used to break down the large amount of thick tenacious mucus associated with the CF
  
  
  1. Acetylcysteine (Mucomyst)
  2. rhDNase(Pulmozyme)
  3. Sodium bicarbonate (2% solution).

• Sympathomimetics: Used when CF patients are having bronchial spasms.
  
  
  1. Albuterol (Ventolin)
  2. Salmeterol (Serevent)
  3. Pirbuterol (Maxair)

• Parasympatholytics: Used to offset bronchial smooth muscle constriction.
  
  
  1. Ipratropium bromide (Atrovent)
  2. Glycopyrrolate (Robinul)

• Expectorants: Used when water alone is not sufficient to facilitate expectoration.

1. Guaifenesin (Robitussin)

• Antibiotics: Used To prevent or combat secondary respiratory tract infections.
  
  
  1. Tobramycin for inhalation (TOBI)
  2. Gentamicin (Garamycin)



• Enzymes: Used to aid food digestion
  
  
  1. Pancrease
     
     
• Sodium Chloride: Patients with CF are encouraged to replace body salts either by heavily salting their food or in pill form.

• Vitamins & Minerals: CF patients have vitamin A, D, E, and K deficiencies; therefore, supplemental multivitamins and mineral are very important.

• Supplemental Oxygen: Hypoxemia is associated with CF, therefore, O₂ is often required. The hypoxemia that develops in CF is caused by the pulmonary shunting associated with the disorder.

Therapeutic INTERVENTIONS

• Postural drainage and clapping (also called chest physical therapy [CPT]). This therapy requires vigorous percussion on the back and chest to dislodge the thick mucus from the lungs.

• Flutter Valve: For use in the removal of mucus from the lungs of patients with Cystic Fibrosis, Chronic Bronchitis, Bronchiectasis. When a patient exhales through the FLUTTER, the steel ball inside is momentarily pushed up, but the weight of the ball brings it back down. This action transmits vibrations to the airways and loosens mucus.
  
  

• The Vest ™

• Intrapulmonary percussive ventilation (IPV): The Intrapulmonary Percussionator is a positive pressure device that provides bursts of gas under pressure at rates of over two hundred cycles per minute. These bursts transmit aerosolized medication deep into the airways and provide internal chest physical therapy.

 

• View a powerpoint presentation by Karen Conyers, BSRT, RRT on Airway Clearance

OTHER WAYS TO MOBILIZE BRONCHIAL SECRETIONS:

• Incentive Spirometry (IS)
• Small Volume Nebulizer (SVN) aerosol therapy

 



• Positive Expiratory Pressure (PEP)
• Suctioning
• Bronchoscopy
• Deep breathing and Coughing
• Ultrasonic Nebulization
• Increased Fluid intake (6-10 glasses of water a day)