How the lungs work
The lungs are made up of a system of hollow tubes ("airways") that start at the windpipe ("trachea"). You can feel this in your neck. These tubes divide and get smaller toward the lung edges. The tubes end in air sacs ("alveoli"), which is where the oxygen you breathe in is transferred to the bloodstream to be transported around the body.
The lining of the airways continuously produce mucus that keeps the airways moist. If dust or bacteria are breathed in they get stuck in the mucus, which is then cleared from the lungs and can be swallowed. This is one of the normal, continuous processes that keeps the lungs clean and free of infection.
Cystic fibrosis and the lungs
Cystic fibrosis (CF) is a genetic disorder caused by a fault in the gene that controls the movement of salt and water in and out of cells in the body. In CF too much water passes into the cells and turns the body's secretions, which normally act as a lubricant, into thick mucus. The mucus clogs up some of the body's tubes, ducts and passage ways. This hinders the function of certain organs, such as the lungs and digestive system.
There are many factors that make it harder for CF patients to clear the mucus from their lungs and this make them prone to chest infections:
- The mucus is abnormally thick and sticky.
- The normal clearance process of dirt and bacteria does not work properly which causes bacteria to build up and block the smaller airways making them more vulnerable to infection.
- Infection leads to inflammation/swelling of the airway tubes.
There are ways of assessing to see if your baby has secretions on their chest or a chest infection. This assessment checklist will help you and advises what action to take.
Download the full size CF mucus assessment flowchart.
Physiotherapy and CF
Chest physiotherapy is designed to help your baby clear any mucus from their lungs, which helps to prevent infections. Find out how to assess if your child has secretions or a chest infection.
A member of the physiotherapy team will teach you airway clearance techniques to help move secretions. We will advise you about how to incorporate these techniques into your family’s daily routine. There are many techniques that can be used, and each intervention will be assessed and introduced for your child as appropriate.
For babies and pre-school age children, physiotherapy treatment usually begins with intermittent percussion (clapping) in various positions aiming to move the mucus, and the baby may cough. Daily exercise is also strongly encouraged at all ages, including babies.
There is evidence to show that CF-related changes begin early in a baby’s life. Therefore, we advise that parents or carers carry out physiotherapy sessions daily with their child and increase the frequency of sessions if the child has a cough or a cold.
The frequency of physiotherapy is individually determined for each patient meaning that some children may require less/or more physiotherapy than others. Not all children will use the same techniques. It is essential that physiotherapy and exercise become part of daily life and can be used as special time with your child. It is hoped positive experiences as a baby or toddler will help co-operation with treatment in later years as the child will learn that physiotherapy and exercise play an important role for a person with CF.
Also, by doing the physiotherapy regularly with your child, you will build up your assessment skills and learn what their chest is like "normally". This should allow you to detect when something is not right and know when to increase the frequency of treatment and/or seek medical advice.
Why do I need to exercise?
Exercise is very important for people with CF as it helps keep your lungs working well, improves muscle strength and keeps your joints flexible.
- Helps to clear your chest
- Helps to maintain lung function
- Keeps bones strong and healthy
- Improves appetite
- Improves posture
- Increases fitness levels
What counts as exercise?
- Walking the dog
- PE lessons and playtime at school
- Walking to school instead of getting the bus
- Wii Fit / Xbox Kinnect
Ideally, the exercise you choose should be enough to increase your heart rate, make you breathe harder and get a bit sweaty.
What do we do on Rose Ward?
If you are admitted to Rose Ward, you will get the chance to do regular exercise. There is a therapy assistant who runs the sessions and can advise you on what exercises to do in hospital and at home.
Some of the choices you may be given:
- Wii Sports / Fit
- Dance mat
- Xbox / Kinnect
- Yoga / pilates
- Gym ball
Why can't I mix with other children?
As with all other activities and care on the ward, we try to reduce any possibility of passing bugs between CF patients (ie cross-infection). For this reason we carry out individual exercise sessions for children with CF.
Some, but not all, people with CF need to monitor their blood sugar levels as it is possible to develop CF-related diabetes. These blood sugar levels may change during exercise.
If your blood sugar levels become too low when you are exercising, you may feel dizzy and unwell. Therefore, make sure you have a sugary snack or drink with you.
Safety precautions when exercising
Avoid dehydration: when you exercise a lot of sweat and salt from your body is lost. This can cause you to become dehydrated and feel thirsty. After exercise it's essential to drink plenty of fluids and to eat a salty snack i.e. crisps (and Creon if you take it ).
Eat well: exercise increases the body's energy needs. Children with CF already have high requirements. It is important to eat enough calories to replace the calories that are lost through exercise. The dietitian can help you work this out if you are unsure. Also, make sure you eat regular meals so that you have the energy to exercise.
Airway clearance: exercise may help to loosen up the sticky secretions in the lungs. Therefore, you may find you cough during or after exercise. If you have any other problems during exercise, such as pain or leaking wee, please tell your physiotherapist, as they may be able to help.
Inhalers: some children with CF need to take inhalers before, during and after exercise if they get wheezy. Please ask the physiotherapist what is appropriate for you and, if necessary, keep your inhaler with you during the session.
Physiotherapy techniques for CF patients
Physio techniques for CF patients include modified gravity assisted positioning, percussion, infant PEP and physical activity.
Modified gravity assisted positioning
Both lungs have different areas within them, called lobes. Postural drainage places the baby into positions where it is thought that gravity will work to help move mucus into the larger airways, helping them cough and clear phlegm, while improving airflow to different areas.
- Don’t do physiotherapy immediately after a feed, this can lead to reflux or your baby being sick
- Don’t tip your baby’s head down lower than their hips as this can lead to reflux or sickness
- Place your baby on a pillow on your lap to do treatment with their head supported
Percussion is used to help move lung secretions. It is usually performed with your child in modified postural drainage positions.
To perform percussion a cupped hand or a number of fingers is used to clap the chest firmly and rhythmically. Always use a layer of clothing or a towel to cushion the skin from the percussion. This is usually performed in 30-second intervals. Treatments are usually 15 minutes and comprise of five minutes each in three positions.
This technique uses a soft face mask that is placed on the baby’s face and gives a small amount of back pressure to the lungs when the baby breathes out. This helps to open up the airways and clear any mucus.
If the physiotherapist feels this is the most appropriate treatment for your baby, you will be shown exactly how to give the treatment and clean the mask. We often combine this treatment with bouncing on a gym ball once your child is a little older and has head control.
Physical activity and exercise are vital for your child’s health and wellbeing and is an important part of your child’s care, alongside airway clearance, to keep their lungs healthy. As well as being good for your baby’s lungs, it is also important for bones, muscles and development. It is a good idea for the whole family to adopt a more active lifestyle where possible.
Even a tiny baby can "exercise". By moving the baby around in different positions, stretching their arms and bouncing with your baby on your lap (once they have good head control) you can ensure that air gets to all the different parts of the lungs. Examples of exercise for young babies include infant massage, baby gym, music classes, swimming (following routine inoculations), and yoga.
As your baby becomes a toddler, you can develop other exercises to help keep interest in treatment, and your physiotherapist will be able to advise you with ideas of what activities will be most beneficial.
There are many other physiotherapy techniques which you may come across as your child grows older. These include blowing games, PEP, autogenic drainage (including assisted autogenic drainage), Flutter® and Acapella®. These techniques will be introduced, if and when appropriate, to your child following continued assessment from a trained physiotherapist. It is important to see the physiotherapists regularly because there are such a variety of options for physiotherapy, and each child’s needs are individual.