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CF A to Z

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ABPA (Allergic bronchopulmonary aspergillosis)

This is an allergy to the mold Aspergillus fumigatus, often found in the lungs of CF patients. The symptoms of ABPA include increased respiratory symptoms like cough, shortness of breath, increased sputum production and decreased lung function. Diagnosis is made by a blood test and/or skin prick test. Treatment may consist of oral steroids and oral antifungal medication.


A hand-held device that uses vibration and positive expiratory pressure (PEP) to dislodge mucus in the lungs (airway clearance). Patients exhale through the device, which transmits vibrations down into the lungs where mucus is shaken loose from airways. The mucus is then moved toward large airways by huffing or cough maneuvers. These devices are usually used as part of a daily airway clearance regimen.

Airway Clearance

Process of dislodging mucus so that it can be more easily coughed out of the lungs. Typically performed as part of a daily regimen, different methods and devices can be used depending on patient age and preference.


A medication that is prescribed by your doctor to treat an infection. Depending on the specific medication and type of infection, these can be given either orally (by mouth), by inhalation (using a nebulizer), or intravenously (through an IV).


A class of antibiotics that is used to treat an infection caused by specific bacteria, such as Pseudomonas aeruginosa. Some examples are TOBI® (inhaled tobramycin), tobramycin, amikacin and gentamicin.


When the airways into the lungs are blocked, typically by a mucus plug, tiny air sacs called alveoli are unable to fill with air and perform their function of exchanging oxygen and carbon dioxide. The result is atelectasis, a collapse of lung tissue.

Bronchus (pl. Bronchi)

Large airway within the lung. The two primary divisions of the trachea that lead respectively into the right and the left lung are called mainstem bronchi.


Widening of airways in the lung.


An inhaled medication that causes expansion of the bronchial air passages, helping to clear bronchial secretions and keep the respiratory airways open. Also used to treat wheezing or cough that is caused by the constriction of air passages in the lungs.




A procedure used to directly visualize the airways within the lungs and to obtain mucus or tissue samples to be used for diagnosis and/or treatment. The patient is sedated, and a long, thin, flexible fiber optic tube (bronchoscope) is advanced through the vocal cords and into the trachea and bronchi (branches) of the lungs. The physician usually uses a camera and video screen to visualize the lung’s airways and manipulate the scope.

Burkholderia cepacia complex

The term Burkholderia cepacia (B. cepacia) complex describes a family of bacteria that are very similar. There are 9 family members that have slightly different DNA (called a genomovar) and have different names, for example B. multivorans and B. cinocepacia. Members of this family of bacteria can cause chronic bacterial infection of the lungs in patients with CF.


A single-celled organism that can cause disease in exposed patients when it enters the body, either through the mouth and nose (by breathing, eating, or drinking), or the skin. In many cases, an antibiotic can be prescribed to help the body either eradicate or reduce the infection caused by the organism. Bacteria are directly or indirectly involved in the lung disease and damage that ultimately is responsible for increased respiratory symptoms in the vast majority of CF patients.

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