Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, and other organs, and how to treat and live with this chronic disease.
CF is a rare genetic disease found in about 30,000 people in the U.S. If you have CF or are considering testing for it, knowing about the role of genetics in CF can help you make informed decisions about your health care.
If you or your child has just been diagnosed with cystic fibrosis, or your doctor has recommended testing for CF, you may have many questions.
Diagnosing CF is a multistep process. A complete diagnostic evaluation should include a newborn screening, a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center.
Raising a child with cystic fibrosis can bring up many questions because CF affects many aspects of your child’s life. Here you’ll find resources to help you manage your child’s daily needs and find the best possible CF care.
Living with cystic fibrosis comes with many challenges, including medical, social, and financial. By learning more about how you can manage your disease every day, you can ultimately help find a balance between your busy lifestyle and your CF care.
People with CF are living longer, healthier lives than ever before. As an adult with CF, you may reach key milestones you might not have considered. Planning for these life events requires careful thought as you make decisions that may impact your life.
People with cystic fibrosis are living longer and more fulfilling lives, thanks in part to specialized CF care and a range of treatment options.
Cystic Fibrosis Foundation-accredited care centers provide expert care and specialized disease management to people living with cystic fibrosis.
We provide funding for and accredit more than 120 care centers and 53 affiliate programs nationwide. The high quality of specialized care available throughout the care center network has led to the improved length and quality of life for people with CF.
The Cystic Fibrosis Foundation provides standard care guidelines based on the latest research, medical evidence, and consultation with experts on best practices.
As a clinician, you’re critical in helping people with CF maintain their quality of life. We’re committed to helping you partner with patients and their families by providing resources you can use to improve and continue to provide high-quality care.
As part of the Cystic Fibrosis Foundation's mission to help improve the lives of people living with cystic fibrosis, the PSDC initiative taps the CF community to inform key efforts to support the management of daily care.
Your cystic fibrosis care team includes a group of CF health care professionals who partner with you to provide specialized, comprehensive CF care.
Many cystic fibrosis patients and families face complicated issues related to getting the care they need. But CF Foundation Compass makes sure that no one has to do it alone.
For many people with cystic fibrosis, dealing with insurance is as much a part of living with the disease as nebulizers and vests. Many people with CF and their families face issues related to getting the care they need, but no one has to do it alone.
The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.
The CF Foundation offers a number of resources for learning about clinical trials and treatments that are being developed to improve the treatment of cystic fibrosis.
Our understanding of CF continues to evolve as scientists study what causes the disease and how it affects the body. These insights drive the development of new and better treatments and bring us one step closer to a cure.
Researchers, supported by the CF Foundation, have made tremendous advances to improve the health and quality of life of people with CF. We are committed to providing the tools and resources you need to continuously build upon this work.
Chest physical therapy (CPT or Chest PT) is an airway clearance technique (ACT) to drain the lungs, and may include percussion (clapping), vibration, deep breathing, and huffing or coughing.
With chest physical therapy (CPT), the person gets in different positions to use gravity to drain mucus (postural drainage) from the five lobes of the lungs. Each position is designed so that a major part of the lung is facing downward. When combined
with percussion, it may be known as postural drainage and percussion (PD&P). This is where a caregiver or partner can clap and or vibrate the person’s chest to further dislodge and move the mucus to the larger airways where it can be coughed or huffed
out of the body.
CPT is easy to do. For a child with cystic fibrosis, CPT can be done by anyone, including parents, siblings, and friends. It can also be done by physical therapists, respiratory therapists, or nurses during care center visits or in the hospital.
With postural drainage, the person lies or sits in various positions so the part of the lung to be drained is as high as possible. That part of the lung is then drained using percussion, vibration, and gravity. Your care team may tailor these positions
to your or your child’s needs.
When the person with CF is in one of the positions, the caregiver can clap on the person’s chest wall. This is usually done for three to five minutes and is sometimes followed by vibration over the same area for approximately 15 seconds (or during five
exhalations). The person is then encouraged to
cough or huff forcefully to get the mucus out of the lungs.
Clapping (percussion) by the caregiver on the chest wall over the part of the lung to be drained helps move the mucus into the larger airways. The hand is cupped as if to hold water but with the palm facing down (as shown in the figure below). The cupped
hand curves to the chest wall and traps a cushion of air to soften the clapping.
Percussion is done forcefully and with a steady beat. Each beat should have a hollow sound. Most of the movement is in the wrist with the arm relaxed, making percussion less tiring to do. If the hand is cupped properly, percussion should not be painful
Special attention must be taken to not clap over the:
Different devices may be used in place of the traditional cupped palm method for percussion. Ask your CF doctor or respiratory therapist to recommend one that may work best for you.
Vibration is a technique that gently shakes the mucus so it can move into the larger airways. The caregiver places a firm hand on the chest wall over the part of the lung being drained and tenses the muscles of the arm and shoulder to create a fine shaking
motion. Then, the caregiver applies a light pressure over the area being vibrated. (The caregiver may also place one hand over the other, then press the top and bottom hand into each other to vibrate.)
Vibration is done with the flattened hand, not the cupped hand (see the figure below). Exhalation should be as slow and as complete as possible.
Deep breathing moves the loosened mucus and may lead to coughing. Breathing with the diaphragm (belly breathing or lower chest breathing) is used to help the person take deeper breaths and get the air into the lower lungs. The belly moves outward when
the person breathes in and sinks in when he or she breathes out. Your CF respiratory or physical therapist can help you learn more about this type of breathing.
Generally, each treatment session can last between 20 to 40 minutes. CPT is best done before meals or one-and-a-half to two hours after eating, to decrease the chance of vomiting. Early morning and bedtimes are usually recommended. The length of CPT and
the number of times a day it is done may need to be increased if the person is more congested or getting sick. Your CF doctor or respiratory therapist can recommend what positions, how often and how long CPT should be done.
Both the person with CF and the caregiver should be comfortable during CPT. Before starting, the person should remove tight clothing, jewelry, buttons, and zippers around the neck, chest and waist. Light, soft clothing, such as a T-shirt, may be worn.
Do not do CPT on bare skin. The caregiver should remove rings and other bulky jewelry, such as watches or bracelets. Keep a supply of tissues or a place to cough out the mucus nearby.
The caregiver should not lean forward when doing percussion, but should remain in an upright position to protect his or her back. The surface that the person with CF lies on should be at a comfortable height for the caregiver.
Sue Smith does CPT on her 4-year-old son, Zack, to help clear his lungs of mucus.
Many families find it helpful to use pillows, sofa cushions, or bundles of newspapers under pillows for support, as well as cribs with adjustable mattress heights/tilts, foam wedges, or bean bag chairs while doing CPT. Infants can be positioned with or
without pillows in the caregiver’s lap.
Equipment such as drainage tables, electrical and nonelectrical palm percussors, and vibrators may be helpful. These can be purchased from medical equipment stores. Older children and adults may find percussors useful when doing their own CPT. Talk to
your doctor or respiratory therapist at your CF care center about equipment for CPT.
Although insurance providers often do not cover equipment of this kind, check with your insurance provider for your specific plan’s benefits. Sometimes, companies that make
this equipment offer assistance programs. Contact the company (or visit the company's website) for details.
You also can contact CF Foundation Compass, a personalized service that can help you with insurance, financial, legal and other issues. Call Compass directly at:844-COMPASS (844-266-7277) Monday
– Friday, 9 a.m. – 7 p.m. ETcompass@cff.org
To make CPT more fun, consider one of the following:
"I firmly believe that I am better off for focusing on chest PT done manually by my dad ... It was a more personal connection. I wasn't sitting alone for an hour a day with a vest on. I know it's time consuming, but I think there's a value to it."
-- Chris Kvam, adult with CF
The following diagrams describe the positions for CPT. In the diagrams, shaded areas show where the chest should be clapped or vibrated. As a reminder:
Self-Percussion -- Upper Lobes
Your child should sit upright and reach across his or her chest to clap on front of chest over the muscular area between the collarbone and the top of the shoulder blade. Repeat on the opposite site. Your child can also clap his or her own upper back if able to reach it.
Upper Front Chest -- Upper Lobes
Have your child sit upright. Clap on both sides of the upper front chest over the muscular area between the collarbone and the top of the shoulder blade.
Upper Back Chest -- Upper Lobes
Have your child sit up and lean forward on a pillow over the back of a sofa or soft chair at a 30-degree angle. Stand or sit behind your child and clap both sides of the upper back. Take care not to clap on your child's backbone.
Upper Front Chest -- Upper Lobes
Have your child lie on his or her back with arms to sides. Stand behind your child's head. Clap both sides of your child's chest between the collarbone and nipple.
Left Side Front Chest
Have your child lie with left side up and raise his or her left arm overhead. Clap over the lower ribs just below the nipple area on the front side of left chest. Do not clap on your child's stomach.
Right Side Front Chest
Have your child lie with right side up and raise his or her right arm overhead. Clap over the lower chest just below the nipple area on the front side of right chest. Do not clap your child's lower ribcage.
Lower Back Chest -- Lower Lobes
Have your child lie on his or her stomach. Clap both sides at the bottom of his or her chest just above the bottom edge of the ribcage. Do not clap the lower ribcage or over the backbone.
Left Lower Side Back Chest -- Lower Lobe
Have your child lie with left side up and roll toward you a quarter turn so you can reach your child's back. Clap on the lower left side of his or her chest just above the bottom edge of the ribcage.
Right Lower Side Back -- Lower Lobe
Have your child lie with right side up and roll toward you a quarter turn so you can reach your child's back. Clap on the lower right side of his or her chest just above the bottom edge of the ribcage.
*Children shown without shirts to better demonstrate the PD&P technique in illustrations. Images are from the CF Family Education Program.
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