Managing a pulmonary fibrosis cough

‍Why do you cough with pulmonary fibrosis

Cough is a natural reflex which protects the lungs from irritants and inhalation of foreign matter/particles. When we cough our vocal cords close together forcefully. Over time and particularly if the cough is persistent, this process can cause the vocal cords to become inflamed and irritated, which can result in further worsening of the cough. This can then lead to production of mucus, which can trigger even more coughing. This vicious cycle can be very difficult to break.

Cough hypersensitivity is a term that refers to patients who are extremely sensitive to external stimuli such a cold air, perfumes, smoke and bleach.

In a recent APF cough survey 57%% of respondents said that their cough affected everyday life, and 100% of responders reported that either exercise, talking or even a change of position could trigger a coughing fit.

Learn about pulmonary fibrosis cough research.

I try not to do things that trigger my cough, which is everything. I'm embarrassed with other people's reactions as they don't understand my condition.

Types of pulmonary fibrosis cough

Coughs are categorised by healthcare professionals as follows:

Acute cough – this lasts less than 3 weeks and is usually due to upper or lower respiratory infections, asthma, or having breathed in irritants such as smoke or chemical fumes.

Sub-acute cough – lasts for 3-8weeks. This type of cough may be due to prolonged recovery phase after an infection, or due to other conditions such as whooping cough or tuberculosis(TB).

Chronic cough – this is a persistent cough which lasts for more than 8 weeks. Chronic cough is a very common symptom when you have been diagnosed with a long-term respiratory condition.

Refractory cough – this is a cough which persists despite ongoing treatment.

With pulmonary fibrosis your cough may be dry and tickly, or it can be productive of mucus. Some people with pulmonary fibrosis will have a combination of both dry and productive cough.

Dry/unproductive cough (or Cough hypersensitivity)– this can be triggered by many different factors such as changes in temperature of the environment, smoky/dusty/polluted atmospheres, aerosol sprays, talking, laughing, exercise or physical activity.

Productive cough – this is a cough which moves phlegm (mucus) from the airways or shifts fluid / food which has gone down the wrong way. It’s our body’s way of protecting the airways so that you can breathe normally again.

I just wish it would stop. The feeling that it is never going to improve is sometimes overwhelming.

Watch 'Clearing the air about pulmonary fibrosis' cough webinar:

Pulmonary fibrosis cough treatment

There are currently no approved or licensed treatments for pulmonary fibrosis cough. Your ILD consultant or nurse can take a detailed history of your cough and consider further investigations which may lead to trialling some treatments. There are also research studies for pulmonary fibrosis cough.

Treatable factors which may impact and contribute to your cough may include acid reflux, postnasal drip (sinus problems), side effects of certain medications, infections, inflammation, other chest conditions. Treatments which may be offered include:

• Proton pump inhibitors (acid reflux)
• GI motility agent (non-acid reflux)
• Inhaled steroid (eosinophilic cough)
• Oramorph (cough hypersensitivity)
• Codeine Phosphate Linctus
• Gabapentin (an anticonvulsant medicine)

If your pulmonary fibrosis cough is mucus productive, your doctor may suggest a mucolytic therapy. These treatments help to ‘break up’ mucus, making it runnier and easier to cough up and clear from the lungs.

Treating persistent cough in pulmonary fibrosis can be a challenge because cough associated with this condition often doesn’t respond to medication. However, there are alternative drug treatment options available that may help manage coughing bouts. These treatments are the same as ones used to treat persistent cough in other conditions and are mostly palliative in nature e.g. they are aimed at relieving the symptoms and do not address the underlying cause of the cough.

It is also important that you speak to your doctor or specialist healthcare team about other possible causes which may be contributing to your cough e.g. acid reflux, postnasal drip (sinus problems), side effects of certain medications, infections, inflammation, other chest conditions. These will need to be treated/managed effectively to minimise their impact.

The pandemic has made life much more difficult as soon as she starts to cough other people assume it’s because of COVID-19.

Top tips: Other ways to manage the pulmonary fibrosis cough

Download PDF: Top Tips on managing a pulmonary fibrosis cough

There are also lifestyle changes and non-drug coping strategies to help manage your cough. 

You may also find some of the following tips helpful to try:

• Sip water at regular intervals throughout the day as this will keep your throat moist reducing dryness and irritation
• Keep well hydrated and aim to drink approximately 1.5 to 2 litres of water daily (NB: unless you are on a fluid restriction regime recommended by your medical team). Avoid caffeine and alcohol which can increase throat dryness
• Practice breathing in gently through your nose – this reduces the irritating effect of cold air on the throat and helps to warm, filter and moisten the air you breathe in
• Try breathing out through pursed lips – this helps channel the air and cushion the vocal cords to reduce further irritation
• Be aware of your posture and try to relax any areas of tension that you notice. Explore different relaxation, mindfulness and distraction techniques to find out which are most helpful for you.‍
• Learn how to breathe gently and rhythmically using your diaphragm
• If going out in cold or windy conditions, try loosely wrapping a light scarf around your mouth and try to breathe in through your nose if you can.
• Try swallowing to ease and reduce tickling and irritation in the throat.
• Try sucking boiled sweets or chewing gum – be aware that medicated lozenges can cause more dryness of the throat. Try non-medicated lozenges such as honey based or menthol free which have a soothing effect.
• Try steam inhalation using a facial steamer or a bowl of water. Always take care when using hot water and gradually build up your tolerance levels if you find it of benefit. Be aware that adding essential oils to the water may be helpful for some but cause more dryness / irritation for others.
• You may find using an air humidifier or purifier helpful.
• Keep a record of your cough triggers and try to avoid/minimise contact with any known triggers such as smoky, dusty, or polluted atmospheres, animal fur, pollen, sprays, cleaning products.
• If you smoke – consult your doctor or NHS website for advice on stopping and the contact details of your local smoking cessation service.‍
• If activity and exercise trigger your cough, pace yourself more and practice breathing through your nose as able. Build up your exercise tolerance gradually.
• Try to keep your home at a comfortable and constant temperature which suits you.
• If your cough is dry, unproductive and persistent a respiratory physiotherapist /speech and language therapist will be able to advise on additional cough suppression techniques which may be helpful in controlling it. Ask for a referral to a cough clinic for this support.
• If your cough produces regular mucus/phlegm which is difficult to manage, it would be helpful for you to learn how to clear this effectively and in a controlled manner. Techniques such as Active Cycle of Breathing can be helpful in achieving this. Ask for referral on to a respiratory physiotherapist who can assess your individual needs and recommend an appropriate self-management plan for airway clearance.‍
• Learn about and get involved in pulmonary fibrosis cough research.

Specialist Cough Clinics:

If you are struggling with your cough despite intervention from your local respiratory team, you can discuss onward referral to a specialist cough clinic for further advice and management.

There are currently 8 specialist cough clinics in the UK which are. These links are for information only. To be referred on to a Specialist Cough Clinic you will need to go through your ILD Consultant. This will be an option if treatment options for your cough have been explored and not been of benefit.

• Kings College Hospital
• ‍Royal Brompton Cough Clinic
• ‍Northumberland Cough Clinic
• ‍Manchester Cough Clinic
• Hull Cough Clinic
• Belfast Cough Clinic
• Leicester Cough Clinic
• ‍Oxford Cough Clinic