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. Most of the chest problems in adults and children are caused by non-acid airway reflux and aspiration.
Professor Alyn Morice
Head Cardiorespiratory Studies, Hull York Medical School, University of Hull
Healthy lungs and airways:
Caerwyn Roberts
Clinical Specialist Physiotherapist, Pulmonary Rehabilitation Services, Betsi Cadwaladr University Health Board
Healthy airways and lungs should be able to do the activities below without breathlessness, painful breathing or coughing:
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. It is good to measure and record your peak flow daily in order to know what is normal for you.
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,
To find the most effective treatment for you/your child is it important to work with your respiratory physiotherapist.
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)
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)
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.
Secretions can build up, bacteria can stagnate/get stuck 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, sometimes several times a day. This may be difficult as the mucus can have a thick, sticky consistency and be hard to cough and clear. It may also feel like breathing through fluid, you may hear breathing noises to suggest your airways are full of mucus (like the ‘snap crackle and pop of Rice Crispies adverts!).
Excessive secretions, frequent throat clearing and chronic cough can be due to irritation of the vagal nerve triggered by oesophageal dysmotility (abnormal muscle movement due to oesophageal atresia). Mucus in the throat may not be from the airways; it may be draining from the nose and/or sinuses. These symptoms will not respond to breathing exercises – you should see your doctor/chest specialist for formal investigations and diagnosis.
If wet cough persists for more than two weeks
or
If the cough is more persistent
or
If coughing regularly overnight on more than two nights a week
or
Coughing regularly with exercise.
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:
Closing your eyes can help relaxation and focus on your breathing.
These are focussed on breathing in deeply, allowing you to loosen secretions in the lungs and airways.
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.
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:
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.
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.
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 (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.
Ask your medical team about management of secretions prescribable by GP/respiratory physicians.
Saline and nebulisers should only be used under medical and physiotherapy guidance.
Understanding correct techniques to get the best from inhaled treatments – aerochamber and facemask.
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. (7,8) Sit 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.
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