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Cystic Fibrosis Care Center


How do I care for an infant with cystic fibrosis? What are the nutritional needs of my toddler? What are the standard tests? What are the treatment options? What does having CF mean to the person's diet, and how do those nutritional needs change over time? What is Pancreatic Enzyme Replacement? How should my child's teacher handle her CF? How I can ensure that my teen takes care of himself? And should a woman with CF think about having children? This section - divided into age groups - will help you answer these and many more questions on living with CF. You'll also find downloadable information from the Cystic Fibrosis foundation for more in-depth explanations of these topics.

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Standard Tests


Lung Function Tests

Lung function tests (or pulmonary function tests - PFTs) let the doctors see how well your lungs are working and assess respiratory muscle strength. Testing is relatively simple, painless and present little or no risk.

Spirometry
Spirometry is the first lung function test done, and measures the air you can move out of your lungs. For spirometry, you breathe into a mouthpiece that's attached to a recording device (spirometer), and the results are printed on a special chart called a spirogram. Spirometry measures a variety of lung functions - you may hear your doctor discussing these:
  • Forced Vital Capacity (FVC) - how much air you exhale forcefully after you've inhaled deeply
  • Forced Expiratory Volume (FEV) - how much air you can exhale with force in one breath - sometimes at one second, (FEV1), two seconds (FEV2) or three seconds (FEV3).
  • Peak Expiratory Flow (PEF) - how quickly you can exhale
  • Maximum Voluntary Ventilation (MVV) - how much air you can breathe in and out during one minute
  • Slow Vital Capacity (SVC) - how much air you can slowly exhale after inhaling deeply
  • Total Lung Capacity (TLC) - how much air is in your lungs after you've inhaled deeply
Respiratory Muscle Strength
Respiratory Muscle Strength are noninvasive tests:
  • Maximal Inspiratory Mouth Pressure (PImax)/Maximal Expiratory Mouth Pressure (PEmax) - after exhaling and inhaling forcefully, patients breath through a flanged mouthpiece while wearing nose clips.
  • Sniff Nasal-Inspiratory Force (SNIF) - A catheter (tube) with a plug on one end is attached to a pressure transducer. The plug is put into one nostril, the other nostril is blocked, and the patient then exhales, closes his/her mouth, and takes a deep sniff.
  • Cough peak flow - while sitting down, the patient breathes in deeply, then coughs into a face mask attached to a peak flowmeter.
Preparing for your Lung Tests
  • While you do not have to fast before lung tests, you should not eat a heavy meal before this test.
  • Wear loose clothing that does not restrict your breathing.
  • Do not eat or drink anything with caffeine (coffees, teas, caffeinated sodas).
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Bronchoscopy

Bronchoscopy is a technique performed by medical professionals to examine a patient's airways, including the larynx (voice box), trachea (main windpipe) and the bronchii (the windpipes within the lungs.) A physician can use bronchoscopy to observe the way these airways move and change shape as the patient breathes in and out. The procedure is usually recorded, and the physician can print pictures to document their findings. The procedure is performed under sedation and light anesthesia given by an anesthesiologist, who monitors the patient continuously throughout the procedure.

The bronchoscopy is performed with a flexible, fiber-optic bronchoscope that is a soft tube no more than ¼" in diameter that incorporates a light source and camera. The scope normally passes through the nose or throat.

The procedure may include a bronchoalveolar lavage (BAL), in which a small amount of saline is injected through the scope to flush out fluid and mucus from the airways so they can be suctioned and tested.

Bronchoscopy is generally performed in the hospital, and the patient may not eat or drink for six hours before the procedure. The patient should not be suffering from a cold at the time of the bronchoscopy. Parents generally accompany a child at the outset of the procedure, while the child remains conscious. After the child is given anesthesia and loses consciousness, parents are generally asked to leave the room. The procedure generally lasts 15 to 20 minutes, followed by an observation period of about two hours before the child can leave. The anesthesiologist may administer a numbing medicine (lidocaine) through the bronchoscope to numb the larynx, trachea and bronchii.

Side effects of the procedure are generally minimal. They may include soreness of the throat, fever, hoarseness, cough or wheezing. To prevent or minimize these problems, the child may receive a dose of anti-inflammatory medication such as dexamethasone or methylprednisolone before the end of the procedure.

Tips for a Successful Bronchoscopy
  • If your child is old enough, explain as much about the procedure as they can understand.
  • Pack some favorite items such as stuffed animals and toys to help your child relax before and after the procedure. Also pack snacks that you and your child can eat after the procedure.
  • Bring the names and amounts of any medications the patient is taking.
  • Alert the physicians to any changes in the patient's medical condition.
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