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 postural drainage and percussion (PD & P), uses gravity and percussion (clapping on the chest and/or back) to loosen the thick, sticky mucus in the lungs so it can be removed by coughing. Unclogging the airways is key to keeping lungs healthy.
PD & P is easy to do. For a child with CF, PD & P can be done by anyone, such as parents, siblings and even friends. It can also be done by the physical therapists, respiratory therapists or nurses during care center visits or in the hospital.
People with CF sometimes use other types of treatments, such as inhaled bronchodilators and antibiotics to keep their lungs healthy. If ordered, bronchodilators should be taken before PD & P to open the airways, and inhaled antibiotics should be taken after PD & P so that the medicine gets deep into the lungs to better fight off infections. Your care team will help you figure out a routine that will work best for you or your child
Learning more about the respiratory system and its relationship to other organs in the body can help you to understand why PD & P treatments are effective.
The goal of PD & P is to clear mucus from each of the five lobes of the lungs by moving mucus into the larger airways so that it can be coughed out. The right lung is composed of three lobes: the upper lobe, the middle lobe and the lower lobe. The left lung is made up of only two lobes: the upper lobe and the lower lobe.
The lobes are divided into smaller sections called segments. The upper lobes on the left and right sides are each made up of three segments: top (apical), back (posterior) and front (anterior).
Segments of lobes are made up of a network of airways, air sacs and blood vessels. These sacs allow for the exchange of oxygen and carbon dioxide between the blood and air. During PD & P, it is these segments that are being drained.
PD & P involves a combination of techniques, including multiple positions to drain the lungs, percussion, vibration, deep breathing and coughing.
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.
Postural drainage uses gravity to help move mucus from the lungs up to the throat. 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 CF care team may tailor these positions to your or your child's needs.
Percussion or clapping 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 percussion also 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 or sting.
Special attention must be taken to not clap over the spine, breastbone, stomach and lower ribs or back to prevent injury to the spleen on the left, the liver on the right and the kidneys in the lower back.
Different devices may be used in place of the traditional cupped palm method for percussion. Ask your doctor or therapist for which one may work best for you.
Vibration gently shakes the mucus into the larger airways. The caregiver places a hand firmly 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 also may 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 respiratory or physical therapist on your CF care team can
help you learn more about this type of breathing.
Generally, each treatment session can last for 20 to 40 minutes. PD & P 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 PD & P and the number of times of day it is done may need to be increased if the person is more congested or getting sick. Your CF doctor or therapist will help you know what positions, how often and how long PD & P should be done.
Both the person with CF and the caregiver should be comfortable during PD & P. 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 PD & P 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 table on which the person with CF lies should be at a comfortable height for the caregiver.
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 PD & P. Talk to your doctor or therapist at your CF care center about equipment for PD & P.
Many families find it helpful to use pillows, sofa cushions, bundles of newspapers under pillows for support, cribs with adjustable mattress heights/tilts, foam wedges or bean bag chairs while doing PD & P. Infants can be positioned with or without pillows in the caregiver's lap.
To enhance the quality of the time you spend doing PD & P, do one of the following:
The following diagrams describe the positions for PD & P. In the diagrams, shaded areas show where the chest should be percussed or clapped.
Pillows may be used for added comfort. If the person tires easily, the order of the positions can be varied, but all areas of the chest should be percussed or clapped.
Please remember to percuss and vibrate only over the ribs. Avoid percussing and vibrating over the spine, breastbone, stomach and lower ribs or back to prevent trauma to the spleen on the left, the liver on the right and the kidneys in the lower back. Do not percuss or vibrate on bare skin.
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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