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Why is physiotherapy important?

People with PCD need to do regular chest physiotherapy usually twice daily or more frequently if suffering from an infection.  Each patient will follow a routine using techniques that are tailored to their needs by a physiotherapist.

Normally mucus traps dust and bacteria in the lungs which is then swept out by normal cilia. However, in PCD mucus stays in the lung because the cilia do not work properly. In a normal person, the cilia in the lungs act like an escalator moving mucus up and out of the lungs where it can be expelled naturally by swallowing or coughing. In PCD, the escalator movement does not happen effectively or at all and therefore physiotherapy is needed to get rid of this mucus.

It is important to break the cycle of sputum getting trapped which causes infection and inflammation. This in turn causes airway damage, which then leads to more sputum being trapped. Physiotherapy can help enormously to break this cycle and prevent irreversible lung damage. It is important to keep the chest clear of mucus to reduce the risk of infection. Coughing is not enough; sputum needs help to move up by various physio techniques.

Plugging of the airways occurs if sputum accumulates in the lungs and this can reduce airflow preventing air from getting into or out of some alveoli (the tiny air sacs that take up the oxygen you breath in at the end of the bronchial tubes) and may lead to them collapsing.  Physio ultimately helps by delaying lung damage, reducing wheeze, reducing the rattle of cough, and helps prevent infection and inflammation.

Physiotherapy English National Standards of care for children with Primary Ciliary Dyskinesia (2018) – ACPRC Guidelines – Link

Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient – Joint BTS/ACPRC Guidelines – Link

Physiotherapy equipment

Various devices can be used to increase the efficacy of physiotherapy and can also help with compliance if there is reluctance to do it. Oscillatory and positive expiratory pressure (PEP) devices such as the Pari PEP or Astra PEP help mobilise and move secretions. This positive pressure on breathing out helps to ‘splint’ the airways open, enabling more air to get in and out of the lungs, therefore increasing the efficiency of mucus clearance.

The Acapella Choice and Flutter are oscillatory PEP devices which provide both resistance and vibration as you exhale. It may be necessary to hold the cheeks to make sure that the vibration is in the lungs and not the cheeks. An Acapella can be used in the postural drainage position or the seated position. The Flutter is easiest used in a seated position.

These vibrations affect all the branches of the airways, and like “shaking apples from the branches of a tree”, the mucus is loosened. Whilst this is happening, the changes in air pressure that makes the steel ball move are both keeping small airways open and allowing air to move throughout the lungs. As a result of this movement, mucus is moved out from where it was previously stuck, into the larger airways and to where it can be coughed out. The Acapella has a similar effect but uses a seesaw mechanism to cause the vibration instead of a ball.

PEP devices (positive expiratory pressure) help keep the airways open by blowing against resistance. It therefore makes it easier to cough up the mucus. Ask your physiotherapist if you can try any adjuncts. Please note it is important that a physiotherapist assesses which device or technique may be most appropriate for you and teaches you how to use it.

Button & Button (2013) compared the forces produced by various physio techniques and devices to turning a ketchup bottle upside down and shaking it. The more you do to move and agitate mucus it changes from being sticky and viscous to becoming thinner and able to move more easily.

Your physiotherapist

Your physiotherapist will work with you to work out the most effective way to do your physio – which may vary depending on your age and personal circumstances.  It is important to have regular reviews with your physiotherapist to check that your physiotherapy is effective, fits with your current lifestyle and is keeping you well.

Younger Children

Parents/carers will be taught the most appropriate daily treatments by a physiotherapist.  Doing daily physiotherapy sessions with children is important but can be a difficult task at times depending on the age of the child.

  • Tickling! Lots of laughter and giggles – they sound like huffs and do a similar job.
  • Games. Playing hide and seek with a toy car/doll/hand puppet whilst the child is upside down on the wedge doing chest physio postural drainage. The hand puppet can give physio instructions, sometimes in funny voices, daft instructions or hiding under clothes.
  • Remember that laughing is a bit like huffing, and if the child associates these physio sessions with fun, they are more likely to be co-operative next time. As they get older a funny video/TV programme may help.
  • Blowing anything: bubbles or cotton wool off a hand or kazoos.
  • Encourage playing of wind instruments to develop strength and control of respiratory muscles and increase lung volume. Singing, in choirs, on car journeys, in the bath.
  • Exercise – Swimming is especially good as it encourages deep breathing, breath control and loosens secretions. Encourage any exercise that is enjoyed. Both adults and children will be more inclined to do something they enjoy: a team sport encourages commitment and a regular date in the diary. Gym helps to keep the rib cage and back strong, mobile and healthy.

Older Children and Adults

For older patients, your physiotherapist may teach you the Active Cycle of Breathing Technique (ACBT). ACBT is a breathing technique that can be used in conjunction with postural drainage, percussion and vibrations during treatment sessions, but it can also be used on its own to help clear mucus. It is an ideal technique to learn to allow older children to start doing treatments more independently.

  • Breathing Control: This is normal gentle breathing using the lower chest, with relaxation of the upper chest and shoulders. It helps you to relax between the deep breathing and huffing.
  • Deep breathing: These are slow deep breaths in followed by relaxed breaths out. Three or four deep breaths are enough.
  • Huffing: This is a medium sized breath in, followed by a fast breath out through an open mouth, using the muscles of the chest and stomach to force the breath out. This will move secretions along the airways to a point where you can cough them up.
  • Coughing: This should follow two or three huffs, or a deep breath in. Do not cough unless secretions are ready to be cleared.