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Lungs and airways

Thanks to members of the Adult OA/TOF Working Group for researching and writing this content.

Those born with congenital respiratory conditions have never had ‘normal’ airways.

Most born with OA/TOF had an abnormal connection between the trachea and oesophagus removed at birth, and many will also have tracheomalacia and/or bronchomalacia (floppy upper airways).

Even those born with pure OA (without TOF) may still have a degree of airway floppiness. This means that some may not realise that symptoms they have always had are abnormal and may be helped by medical treatment and/or physiotherapy.

"This is a very comprehensive and informative resource."

Caerwyn Roberts

Clinical Specialist Physiotherapist,
Pulmonary Rehabilitation Services,
Betsi Cadwaladr University Health Board

Healthy lungs and airways should be able to:

  • Breathe with ease and no effort.
  • Breathe quietly and without wheeze.
  • Breathing is pain-free.
  • Breathe easily at rest and mild exertion, recovers quickly from being breathless at exercise.
  • Don’t produce any phlegm/mucus/sputum unless infected.
  • Don’t cough daily.

Healthy airways and lungs should be able to do the activities below without breathlessness, painful breathing or coughing:

  • Sit still or lie down
  • Bend over
  • Talk
  • Sleep
  • Do housework
  • Get washed and dressed
  • Walk around the house
  • Walk outside on flat ground
  • Climb a single flight of stairs without pausing.

Normal lung function

Blood oxygen levels (with a pulse oximeter) should be 95-100% (can be lower in older adults and at high altitude).

Normal peak flow test scores and how to do the test (breathing out as hard and fast as you can into a device called a peak flow meter) can be found here. If it is below expected, it is a sign of airway narrowing, as can happen in asthma. However, a normal peak flow isn’t a guarantee your airways are healthy.

Spirometry may be done at your GP or hospital. It is a more complicated version of a peak flow test, where you will be asked to breathe out as fast as you can for as long as you can.

It measures both how well air comes in and out of your lungs, and how much air your lungs can hold (lung volume).

It measures your FVC (forced vital capacity- how much air you can blow out after your deepest breath) and FEV1 (forced expiratory volume in 1 second- how much air you can blow out in 1 second).

Normal results are 80% or more of what is expected for your age, sex, ethnicity and weight. When the two measurements are divided (FEV1/FVC) a ratio of 70% or more is normal,

Breathing techniques (for all respiratory issues)

Healthy lungs use the diaphragm (a muscle at the bottom of the lungs) to move the lungs, replacing old air with new air full of oxygen. With chronic airway problems, the lungs become less good at moving air in and out, trapping stale air in the lungs. Regular breathing exercises can help the lungs get rid of stale air, increase oxygen levels and strengthen the diaphragm. (1)
  • Pursed lip breathing:
    breathing in through your nose, then out through pursed lips keeps airways open longer and reduces the number of breaths needed. More air is able to flow in and out of the lungs so you can be more active.
  • Diaphragmatic breathing: breathe in through your nose, expanding your stomach/abdomen then out through your nose, so the abdomen empties. You’ll need to relax your neck and shoulders too.
  • Keeping cool when it’s hot or when you are unwell:
    A small handheld fan near the face or air conditioning can be useful in alleviating breathlessness. This can have a calming effect and help you relax.

Regular exercise (for all respiratory conditions)

Regular exercise (within your tolerance zone and capabilities) improves your ability to make use of oxygen during exertion, as well as fitness and endurance. This has been shown to reduce dyspnoea (breathlessness and difficulty breathing (pain or effort) and tiredness, as well as the number of flares people with lung disease have. (2)Exercise helps with lung function, heart health, muscle strength and posture. Exercise can help control blood sugar levels, maintain bone health, improve mood , general fitness and overall health and well-being. People with lung disease should aim to do a range of physical activities on a regular basis to get the most health benefits. Both airway clearance and exercise are important parts of bronchiectasis physiotherapy.Exercise can help loosen mucus in the lungs and make airway clearance techniques quicker and easier. Your bronchiectasis physiotherapist will help you to find ways of exercising that are best for you and advise you on how exercise can help with airway clearance. (3)

Music (for all respiratory conditions)

Playing a musical instrument such as a harmonica or recorder can teach new breathing control techniques to strengthen breathing muscles.

Singing can also help the strength of your voice and improve breathing muscles and if in a group it can also improve mental wellbeing.

Airway clearance breathing exercises (for clearing secretions)

Secretions can build up, bacteria can stagnate in the mucus and lead to infection. Infections cause swelling and can lead to further mucus production. Recurrent infections can lead to lung damage.

People with excess secretions need to cough up secretions/mucus every day/several times a day (and the mucus may be thick and hard to cough up). It may also feel like breathing through fluid and you may hear breathing noises to suggest your airways are full of mucus (like the ‘snap crackle and pop of Rice Crispies adverts!). You may also have to ‘clear your throat’ on a regular basis (this may not always be from the airways and lungs as problems in the nose and sinus can also cause this and these don’t need these exercises, so see your doctor/a chest specialist for formal investigations and diagnosis if not already diagnosed with airway and lung problems before doing these exercises).

Active cycle of breathing technique

This is a series of exercises that help loosen secretions from the lungs, improve the amount of air getting into and out of the lungs and makes coughing more effective at clearing out secretions. It needs to be taught by a physiotherapist first, to make sure you are doing it correctly, as if you do it wrong, it may worsen rather than help. 

It has three parts:

Breathing control

  • Breathe in and out gently through your nose if you can. If you cannot, breathe through your mouth instead.
  • If you breathe out through your mouth, it’s best to use ‘pursed lips breathing’ (see above).
  • Try to let go of any tension in your body with each breath out and keep shoulders relaxed.
  • Gradually slow your breathing.

Closing your eyes can help relaxation and focus on your breathing.

Deep breathing exercises

These are focussed on breathing in deeply, allowing you to loosen secretions in the lungs and airways.

  • Relax your chest and shoulders.
  • Take a long, slow and deep breath in, ideally through your nose.
  • At the end of the breath in, hold the air in your lungs for 2-3 seconds before breathing out.
  • Breathe out gently and relaxed, like a sigh. Don’t force the air out.
  • Repeat 3 – 5 times. If you begin to feel dizzy or lightheaded, stop and return to breathing control instead.
  • Placing your hands on your ribcage can help do this.

Huffing
(forced expiration technique)

  • Take a normal breath in, then breathe out firmly, like you are steaming up a mirror. This helps move secretions in the lower airways.

  • Then take a deep breath in, open your mouth wide and breath out hard and fast. This can move secretions in your upper airways.

  • Only do one or two of each without a break as this can cause airway irritation and chest tightness.

Positive Expiratory Pressure devices

These devices are prescribed for people with chronic lung disease and those with difficulty clearing secretions from their airways.

The aim of the device is to make you breathe out against resistance, which helps air to get behind the mucus in the airways, move the mucus from the airway walls and hold airways open for longer.  Usage can result in an improvement of lung function, as well as improving clearance of secretions.

These need to be prescribed by a physiotherapist – the patient is first assessed by respiratory physiotherapist, trained to carry our Active breathing Technique – then mask fitting and correct valve size assessment.

Common ones prescribed or available in the UK include:

Directed coughing techniques

Some people with airway disease are not able to cough effectively and thus can’t clear secretions from their airway well. There are different techniques to improve the effectiveness of your cough, and they can be used with the other exercises, techniques and devices listed here.
  • Deep cough- sit upright at the edge of the bed or chair and take 2 normal breaths in through the nose and out through pursed lips. Next, take a deep breath in and hold it for 3 seconds. Lastly, use your stomach muscles to force air out of your lungs in a deep cough. This should move the mucus in your lungs and airways so they are easier to cough out. You can repeat this as needed, but should give yourself several normal breaths before doing it again, and not tire yourself out or irritate your airways by doing it too much. (4)
  • Huff coughing (see above)
  • Splinting- when coughing is painful or difficult, it can help to hold a pillow up to your chest, or a large soft toy, squeezing it close to your chest when you cough. This can help the cough to be more effective and less uncomfortable- many of us have discovered this through trial and error in any case.

Postural drainage

This used to be recommended and taught by physiotherapists to aid drainage of secretions from certain particularly affected areas of the lungs and airways. It consists of lying and sitting in certain positions to drain secretions from the airways using gravity and some born with OA/TOF may have been taught this in the past. It is not appropriate in those born with OA/TOF due to the aggravating reflux and aspiration in some of the positions. It is also now not recommended in other conditions like cystic fibrosis for the same reason.

Percussion and drainage

This consists of very fast chest clapping with cupped hand/rhythmic squeezes to chest wall as you breathe out. A physiotherapist will teach you how to carry out this technique safely. It is useful when you are tired, or symptoms have flared. It should not be done if you are on treatments to thin the blood/prevent clotting or have osteoporosis.

Moist air/steam

Moist air (like a hot shower) and steam inhalation can moisten airways and sticky secretions and make them easier to cough up. However, caution should be used to avoid burns.

Medical management of secretions

  1. Carbocisteine and acetylcysteine are tablet medications prescribable by GPs that thin the thickness (viscosity) of secretions
  2. Nebulised saline can also be prescribed by your gp or respiratory specialist to help loosen secretions. A nebuliser is an electric machine that sprays a fine mist of salt water (in this case) into the airways. (5)
  3. Reflux and nose and sinus problems can aggravate or cause secretions (post-nasal drip. Sinus drainage from chronic sinusitis). See Adult OA/TOF management handbook for more information about these conditions and their treatment. (6)
  4. Steroid inhalers may be prescribed by your doctor to reduce airway inflammation which can also lead to secretions. These need to be used regularly for good effect and can take up to 2 weeks to show an effect. Salbutamol (blue) inhalers are not usually useful in those born with OA/TOF and can aggravate airway issues by relaxing our floppy trachea’s muscles further. It is important to use inhalers using the correct technique, preferably with a spacer device or mask to help the powder reach the trachea, bronchus and smaller airways. Usually, in those born with OA/TOF, it needs to get to the trachea and bronchus, not the smaller airways like in asthma, so some of the inhaler types may not be suitable here – please discuss with your doctor.

Understanding correct techniques to get the best from inhaled treatments – aerochamber and facemask.

Positioning to ease breathlessness

Wherever possible try to remain calm and relaxed, keeping cool – a small handheld fan near the face or air conditioning can be useful in alleviating breathlessness.

For those who are breathless for a longer period (e.g. not just after exercise) Some positions can make it easier to fill your lungs with air when you breathe in. With all positions, try to relax the hands, wrists, shoulders, neck, and jaw as much as possible and maintain good posture. (7,8Sit upright in a chair, as this position fixes the shoulders in place and this enables the breathing muscles to be most efficient and eases breathlessness.

Try leaning forward whilst sitting to ease breathlessness. Leaning forward may also improve the movement of your diaphragm, which is the main muscle of breathing.

Try leaning forward onto pillows with legs placed apart.

Sit or stand in a forward lean position with arms resting on a sturdy chair or a windowsill (Only for those patients for whom forward lean sitting or standing is effective).

Lean against a wall with legs slightly apart.

Bibliography

  1. Association AL. Breathing Exercises [Internet]. [cited 2024 Mar 24]. Available from: https://www.lung.org/lung-health-diseases/wellness/breathing-exercises
  2. Lee AL, Hill CJ, Cecins N, Jenkins S, McDonald CF, Burge AT, et al. The short and long term effects of exercise training in non-cystic fibrosis bronchiectasis–a randomised controlled trial. Respir Res. 2014 Apr 15;15(1):44.
  3. Cystic Fibrosis Physiotherapy [Internet]. [cited 2024 Apr 14]. https://www.cysticfibrosis.org.uk/what-is-cystic-fibrosis/cystic-fibrosis-care/physiotherapy
  4. Fink JB. Forced expiratory technique, directed cough, and autogenic drainage. Respir Care. 2007 Sep;52(9):1210–21; discussion 1221-1223.
  5. BNF: British National Formulary – NICE [Internet]. NICE; [cited 2021 Aug 2]. Available from: https://bnf.nice.org.uk/
  6. Adult OA/TOF Management Handbook – TOFS | OA/TOF Support [Internet]. [cited 2024 Apr 14]. Available from: https://tofs.org.uk/oa-tof-information/professionals/adult-oa-tof-management-handbook
  7. How can I manage my breathlessness? | Asthma + Lung UK [Internet]. 2024 [cited 2024 Mar 24]. Available from: https://www.asthmaandlung.org.uk/symptoms-tests-treatments/symptoms/breathlessness/how-can-i-manage-my-breathlessness
  8. Bott J, Blumenthal S, Buxton M, Ellum S, Falconer C, Garrod R, et al. Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient. Thorax. 2009 May 1;64(Suppl 1):i1–52.