Respiratory

What is respiratory physiotherapy in the community?

Children and young people with complex disabilities suffer from a high incidence of respiratory problems. Poor lung function and recurrent chest infections can lead to serious illness and lengthy hospital admissions that impact upon their quality of life and their families.

Respiratory physio helps children and young people to clear secretions and keep the lungs full of air and as healthy as possible.

The physiotherapist will work closely with family, carers and other professionals to ensure the treatment program is specific to meet the child/young persons needs and their current goals.

Our aim is to:

• Reduce attendances to A&E
• Prevent unnecessary hospital admissions
• Reduce length of hospital stay
• Minimise presentations to GP practices for chest infection wherever possible
• Help keep the family together at home
• Increase family and carer confidence in management of the CYP’s respiratory health
• Increase attendance at school and involvement in the community.

Who may benefit from seeing a respiratory physiotherapist?

There are many groups of children with the following conditions who will benefit from specific respiratory physiotherapy input.

• Recurrent chest infections requiring repeated antibiotics
• Long term ventilation
• Upper airway obstruction
• Poor swallow, gastro oesophageal reflux and/or excessive oral secretions
• Skeletal deformities such as scoliosis
• Chronic lung conditions such as CF, Bronchiectasis, Primary Ciliary Dyskinesia
• Other conditions that cause respiratory compromise such as severe global developmental delay, neuromuscular and neurological conditions, brain injury, spinal cord injury, chronic cardiopulmonary disorders.

Please see below for more information on some of these conditions

Who don't we see?

• Stable chest conditions with no acute change/ compliance issues
• Patients without a respiratory condition or respiratory compromise

Cystic Fibrosis (CF)

CF is one of the conditions we help children and parents to manage.

The Cystic Fibrosis Trust website has a lots of amazing information on it to look through.

Here is a list of easy read leaflets on respiratory physiotherapy for people with CF.

Or for a more detailed document, here is their Standards of Care and Good Clinical Practice for the Physiotherapy Management of CF.

Other conditions we see

Bronchiectasis

Bronchiectasis is a long-term condition that affects the airways in the lungs. It’s caused by damage and scarring which make the airways wider and thicker than usual. Most often this damage is from an infection. The reason for the damage is unknown in about 4 in every 10 people with bronchiectasis. Bronchiectasis can happen in part of a lung or a whole lung. It can affect one or both lungs.

Lungs contain many airways that branch from their windpipe like a tree. These airways contain glands that produce a small amount of mucus. Mucus helps trap dust and germs. Tiny hairs called cilia move the mucus up and out of the lungs. In children with bronchiectasis, the airways in their lungs can’t clear themselves properly. Mucus builds up and the lungs can become infected with germs.

The most common sign of bronchiectasis is coughing every day. This cough will not go away, even when you don’t seem to have a cold or another infection. The cough might sound wet and bring up green or yellow mucus. It might improve with a course of antibiotics but will quickly return.

Primary Ciliary Dyskinesia

Primary ciliary dyskinesia (PCD) is a rare, inherited, condition that affects several organs and gets worse over time.

The airways inside our lungs produce mucus to help trap dust and germs. The mucus is then cleared by tiny hairs called cilia. The cilia move continually to clear mucus away from the lungs, nose, and ears. In children with PCD, the cilia don’t work properly. This leads to a build-up of mucus causing inflammation and infection in the airways, sinuses and ears.

PCD is usually diagnosed at birth as they have trouble breathing and may have needed oxygen treatment for days or weeks after they were born. Usually, babies will recover quickly from breathing problems. However, they will often have a runny nose or rattly wet cough every day from the early days or weeks of life.

Severe Global Development Delay

SGDD is an umbrella term capturing a population of children with severe intellectual disability and severe motor impairment. This may be as a result of infection, illness, trauma, genetic or metabolic disorders. Many children falling into this category do not have a definitive diagnosis. Children with SGDD are usually dependant upon others for all of there activities of daily life.

Due to multiple factors they can suffer from a high incidence of respiratory problems. These factors include:

• Aspiration (breathing in secretions, feed, reflux, vomit or foreign bodies into the lungs) - this introduces microorganisms into normally sterile airways increasing risk of infection
• Ineffective cough and diminished sensitivity to cough - this leads to reduced effectiveness of clearing the lower airway secretions
• Altered muscle tone which can have secondary complications on joints, muscles and posture and affect the effectiveness of the lungs.
• Nutrition - Malnutrition can lead to muscle wasting, weakness and reduced lung function and on the other hand obesity (which can be common in down’s syndrome) can make breathing harder.

Neuromuscular Conditions

Neuromuscular conditions such as Duchenne Muscular Dystrophy can compromise the respiratory system primarily due to respiratory muscle weakness which makes it harder to take deep breaths and to cough effectively to clear secretions.

Cerebral Palsy

Children with cerebral palsy (CP) can be at risk of respiratory complications, particularly those with more severe CP such as level 5 on the GMFCS scale who may come under the SGDD umbrella (see above), and those needing two antibiotic courses per year for their chest.

Please have a look at this poster which includes more risk factors: [insert one of Mels CP respiratory risk factors poster]

How do we assess how you're doing?

We have a few different outcome measures we can use to assess how your childs chest is affecting their function and if their management remains appropriate. Click on each heading to find out more:

1. Patient reported outcomes

What you and your child tells us, including in our questionnaires, is key to helping us monitor how your childs lungs are doing.

One commonly used questionnaire for those with CF is the such as the Cystic Fibrosis Questionnaire Revised (CFQ-R)

2. Spirometry

Spirometry is a common, and relatively easy, test used to measure pulmonary function (how well the lungs are working).

It involves a person breathing into a small device, called a spirometer, to measure the speed and the volume of air that is moving in and out of the lungs.

The test can be used in a few different ways but most commonly your child will be asked to breathe in as much as they can, then breathe out into the mouthpiece of the spirometer as quickly as they can..

Other variations on spirometry are also used including the Lung Clearance Index (LCI) which involves the multiple-breath washout (MBW) test. This is simply breathing normally into a device and the Impulse Oscillometry System (IOS) and is easy to perform with children.

3. Sputum samples

Children and young people with respiratory conditions tend to get frequent respiratory infections, sometimes caused by bacteria or fungi.

A sputum (mucus) sample helps us detect and identify these bacteria or fungi so we can prescribe the most effective antibiotics. We look at amount, weight, colour, and ease of expectoration (coughing up the mucus) too.

4. Exacerbations

We also look at how many exacerbations (acute worsening) your child's had and how often they have them

5. CT and MRI imaging

CT scans can help detect your child's lung disease progression although they are not repeated too often due to radiation.

MRI is a radiation-free alternative but has its limitations clinically.

6. Exercise testing

This is recommended at least annually for CF patients to help us monitor how your child and your childs lungs are doing. Some examples include:

• Modified shuttle walk test
• Incremental step tests

What treatment options are there?

We have summarised 4 common treatment recommendations here. Please speak to your physiotherapist about them before trying them.

1. Physical activity and exercise
Physical activity and exercise should be part of the routine management for people with CF and other lung conditions, at any age.

It can move high volumes of air, preserve the elasticity of the lung walls and therefore promotes the ability to clear mucus.

Please speak with your physiotherapist who can create your child an individually tailored exercise program. It is likely to include a combination of fun physical activity that they enjoy with friends and family, aerobic training, resistance/strength training and some balance/coordination practice.

Some studies have shown physical activity and exercise to improve:

• Aerobic fitness
• Exercise capacity
• Pulmonary (lung) function
• Clearing mucus from lungs
• Maintaining blood sugar levels
• Bone mineral density (reducing risk of bone fractures)
• Quality of life

2. Airway clearance techniques
Here are just a few examples of ways to help your child clear sputum (phlegm) for the lungs. Your physiotherapist should go through this with you and your child before you try yourself.

Active Cycle of Breathing Technique (ACBT)
ACBT combines different breathing techniques that help clear mucus from the lungs in three phases.
Click on the drop down menus below or check out this leaflet on ACBT for more information.

Phase 1. Breathing control

Breathing control helps relax the airways. Your child should breathe in through their nose and out through their mouth with very little effort. They should use normal, gentle breathing while relaxing the upper chest and shoulders.

A good way to do this is to place one hand on your stomach as you breathe - your stomach should go out as you breathe in. By using the pursed lip technique when breathing out (pursing your lips like you are kissing someone), you create back pressure in the airways that stents the airway open longer. Repeat breathing control for six breaths before moving to chest expansion exercises.

Phase 2. Chest expansion exercises

This second phase helps your child to get air behind the mucus. They should breathe in deeply. Hold for 3 seconds if they are able. Then they breathe out without forcing the air out.

Phase 3. Huffing

Also called forced expiration technique, huffing is like trying to steam up a window. Try asking your child to huff into a round 'huffing tube' that you would of been advised on, to move mucus up to the larger airways, so they can cough it up easier with less effort.

PEP
PEP stands for positive expiratory pressure and helps to open up your airways and get air behind the mucus to help it move higher up in your airway. Once it gets into the back of your throat, you can huff and cough the mucus out. It is usually given in one of two ways:

Oscillating PEP
An Oscillating PEP device is similar to standard PEP but as you breathe out and get the resistance or back pressure in your airways, it also vibrates your airways. These vibrations can help loosen the mucus away from the airway walls, allowing it to be cleared from your lungs. Different devices are available and your physiotherapist will help to decide which one is best for you.

3. Medications & inhalation therapy

Medicine to benefit the lungs can be taken by nebuliser (inhaled into the lungs), orally or intravenously (into the veins). Some common examples include:

• Bronchodilator drugs open the airways by relaxing the surrounding muscles, relieving tightness and shortness of breath. Bronchodilators may also be used just before a physiotherapy session to open the airways and help with clearance.
• Antibiotics treat or control persistent infection.
• Steroids reduce inflammation in the airways
• Mucolytics such as DNase and hypertonic saline to break down mucus, making it easier to clear from the lungs.
• Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies - to correct the malfunctioning protein made by the CFTR gene in people with CF.

Please see this leaflet on inhaled therapies for more information

4. Oxygen therapy

Children and young people may need supplementary oxygen when acutely unwell. This can be given via a tube in the nose or a mask over the nose/mouth.

Long term supplementary oxygen in children is less common although is needed in those with severely effected lungs.

In children, long term supplementary oxygen is not usually required although some children may need it to help improve school attendance or sleep.

Treatment for babies and toddlers

Take a look at the CF Trusts guide for respiratory physio for babies and young children with CF.

It includes information on exercise and airway clearance such as percussion and baby PEP.

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