Pulmonary Fibrosis Treatment

There is no cure for PF and there are currently no procedures or medications that can remove the scarring from the lungs. Treatments are used to slow progression of the lung scarring, and may not necessarily lessen the symptoms of cough and breathlessness. Pirfenidone (Esbriet) and Nintedanib (OFEV) have both been shown to slow the progression of PF; however, some patients cannot take these medications due to their side effects. These medications are not approved for use in other interstitial lung diseases.

Some additional medications can be used to improve the symptoms of IPF, such as shortness of breath and cough. This includes treatment of Gastro-oesophageal reflux disease (GORD) with anti-acid therapy and treatment of shortness of breath with opioids (a class of medications that includes morphine). Other treatments include supplemental oxygen, pulmonary rehabilitation (exercise training, education and support for people with chronic lung disease), and lung transplant. 

Self Help Measures

  • Eat smaller amounts more often
  • Maintain a healthy weight
  • Raise your bed pillows to be more elevated whilst sleeping or resting.
  • Avoid food and drink that increases anti-acid
  • Talk to your GP about prescribed medication that can help ease symptom

Lifestyle changes

Pulmonary Fibrosis is an irreversible, progressive disease. So it is important to learn good coping skills and educate yourself about the disease as much as possible. 

  • Quitting smoking is the most important thing you can do to help slow down the progression of any lung disease.
  • Get a flu vaccination each year.
  • Ask your GP about your one off pneumonia vaccination.
  • Keep away from people with chest infections and colds.
  • Wash your hands properly and often.
  • Stay as fit as you can.


The scarring in the lungs can prevent oxygen from being absorbed into your body. Low oxygen levels can make you feel short of breath and tired. If your blood oxygen levels are too low, your doctor may prescribe supplemental oxygen (home oxygen). This helps to reduce breathlessness and can help you be more active. Some people with IPF may need oxygen therapy all the time, while others may only need it during exercise or sleep.


There are no medications that cure PF or decrease the amount of scarring in the lungs. Some medications help slow the progression of pulmonary fibrosis.
It is very difficult to find medications that will work for everyone with PF. Medications for PF have side effects in some patients, so it is important to talk to your doctor about the possible risks.

Pulmonary rehabilitation

Pulmonary rehabilitation is an exercise and educational program designed just for patients with lung disease. Pulmonary rehabilitation can help in teaching patients with IPF how to breathe more efficiently and to perform their activities of daily living with less breathlessness.

Lung transplant

Lung transplant is not right for everyone, but it may be a treatment option for some people. It is important to work with your doctor to see if lung transplant is an option for you.
If a person qualifies for a lung transplant, they are put on a waitlist for organ donation. The wait can be long and the surgery can be risky. People who get transplants must take several anti-rejection medications (immune-suppressants) for the rest of their lives.


Most people diagnosed with ILD experience breathlessness.

Not all homes or properties are suitable to have deliveries of liquid oxygen. Liquid oxygen has to be kept in a safe area, approved by your provider and deliveries require good access to this area.

This is usually first noticed during exercise and may be relieved by using oxygen. Not all breathlessness is relieved by oxygen, so it is important to be formally assessed for oxygen before using it (see also section on breathlessness).

You may be offered ambulatory oxygen if after an assessment it is found that your oxygen levels drop while you are performing activities. Activities such as walking, going up the stairs, and doing some routine activities can be improved by using ambulatory oxygen. Your doctor or healthcare practitioner can perform a simple walking test to see whether ambulatory oxygen might help you.

Ambulatory oxygen is provided in the form of cylinders for walking outside the home. It is up to you whether you use ambulatory oxygen; some people prefer to slow down and stop and start in preference to wearing and carrying oxygen cylinders. It is however, important to remain mobile and active. Your muscles still need to be exercised otherwise they will lose strength and your exercise capacity will drop significantly if you do not continue moving around. We know that it is unpleasant to get breathless, but you should not avoid doing things because you get breathless. It is important to remember that everyone who exercises to their maximum get breathless. Even elite athletes get breathless! If you find that you are avoiding doing things because you are getting breathless then maybe oxygen might help you.

Sometimes ambulatory oxygen is needed to help perform activities of daily living such as washing and dressing and moving from room to room. For ambulatory oxygen inside the home, you would use your oxygen concentrator.

Sometimes your doctor might suggest that you need oxygen for a minimum of 16 hours a day. Oxygen in this instance is being recommended to help maintain the health and function of your organs including your heart. It is not being recommended to relieve breathlessness and it is important that you use oxygen in this instance. You may have 2 different prescriptions of oxygen; one for resting, sitting and sleeping and one for ambulating or moving around.

There are a variety of oxygen delivery devices including nasal cannula and masks, all of which need to be assessed on you and you may have different oxygen prescriptions for each device. Always check with your local healthcare team if you are unsure of how you should use your oxygen.

Oxygen concentrator

This is a small machine that is delivered to your home. It is powered by electricity which takes in air and removes the other gases allowing only oxygen to be filtered out to the patient through either nasal cannula or a mask.

It is capable of running 24 hours a day 7 days per week and is regularly serviced by the oxygen contractor. You will be reimbursed for the electricity used. You will also be given a large back-up cylinder for use only in an emergency in case there is an electrical power cut.

Oxygen cylinders

These are small lightweight cylinders used for ambulating outside the home. They may be fitted with an oxygen conserver that will deliver a pulse dose of oxygen only when you breathe in.
This allows the cylinder to last longer. Sometimes your oxygen assessment unit will recommend that you use the oxygen from a cylinder without a conserver if they feel you need continuous oxygen.

You may be given several cylinders at a time for ambulating outside the home and instructed to telephone the oxygen company once they are empty and you need replacement full cylinders.
You will be supplied with a back pack to carry your oxygen in your back, but some oxygen companies will supply a trolley for transporting your oxygen cylinder.

You might prefer to use your own trolley to transport your oxygen.

In some areas the oxygen companies are supplying transportable portable oxygen concentrators. Sometimes these units do not supply an adequate flow rate for ambulation for people with pulmonary fibrosis, but your healthcare practitioner will assess you and be able to tell you what is suitable and available for you.

Liquid Oxygen

If you are using a lot of ambulatory oxygen at higher flow rates then your oxygen assessor may recommend that you use liquid oxygen. Liquid oxygen is usually delivered at regular intervals in the form of a large reservoir unit and a smaller domiciliary device that you fill yourself from the reservoir unit. Your oxygen supplier will show you how to use this.

You will need to store your reservoir tank on the ground floor or in a garage. You are given 1 or 2 ambulatory cylinders that you can fill yourself before you go out. They usually last longer than regular cylinders, although the oxygen will ‘leak’ from the ambulatory canisters if not used on each occasion.

It is important to fill the liquid oxygen canisters up just before you need them. You will be shown by the oxygen supplier how to ‘decant’ liquid oxygen from the reservoir tank into your ambulatory device.

There are special storage instructions associated with the storage and use of liquid oxygen which you will need to consider. If you have any concerns about using liquid oxygen; please contact your healthcare practitioner or your oxygen supplier. Liquid oxygen is useful in that the ambulatory cylinders can provide up to 15 litres/min. Obviously the higher the flow rate that you use the shorter time it will last, but generally speaking it will last longer than a cylinder of oxygen.

Remember please be careful

All oxygen is flammable. Please do not smoke using oxygen and keep away from naked flames. Store your oxygen in a safe place.

Holidaying with pulmonary fibrosis

Everyone loves a holiday, but when you’re living with pulmonary fibrosis, it can take more preparation than it used to. If you need oxygen, it’s worth thinking through how this will affect your holiday.

Planning ahead

Holidays are to be enjoyed. Focusing on what you CAN do and being realistic about what is manageable can help set realistic expectations. Being prepared – planning ahead and finding out as much as you can about your trip can help ease anxiety before you go. 

Here are our five tips for planning your holiday:

1. Think through the journey

Wherever you plan to go, consider how long a journey you can manage, whether you need to move around, how you will manage connections and whether you can get assistance with luggage and transfers. If you’re planning to go by plane, you’ll also want to consider whether you can manage the flight. There’s more information about this below.

2. Consider your destination

Will the altitude or weather affect your breathing? Are there a lot of hills where you are going? Find out as much as you can about the accommodation, what facilities are available, accessibility and how flexible they can be to meet your particular needs. You’ll also want to make sure you have a supply of oxygen if you need it. Read more about this below.

3. Get expert help

Consider travel agents specialising in accessible or disabled travel and seek advice from disability forums such as www.disabilityholidaysguide.comwww.disabledholidayinfo.org.ukwww.ageuk.org.uk and www.disabledinfo.co.uk. If you need advice that’s tailored to you, talk to your doctor or nurse well ahead of your planned trip.

4. Ask others like you

Talk to members of your local Support Group who may have experiences and tips to share. There’s a story below about a couple’s experience on a cruise.

5. Save your documents

Print out and take with you (or save on your phone) copies or photos of any relevant medical information: hospital letters, list of medications, details of your oxygen prescription, your GP details, EHIC and insurance details – just in case you need healthcare while you are away. And make sure you take enough medication (and spares!). You may want to take a standby course of antibiotics.


It is important to get full travel insurance for a holiday overseas. Competitive travel insurance can be difficult to find when living with pulmonary fibrosis. There are specialist companies that provide insurance for disabled and older people. Shopping around and using the phone rather than just buying online might result in more success. 

Many people use the Action for Pulmonary Fibrosis facebook or twitteraccounts to find out recent deals found by other families. The British Lung Foundation can provide useful advice. 

European Health Insurance Card

Until the end of 2020, the European Health Insurance Card (EHIC) is free for UK and EU citizens and entitles you to all medically-necessary treatment – but if local residents have to pay a fee, so do UK visitors. 

From 2021, the agreement will move to individual arrangements with European countries and might mean you need to pay for treatment in full. You can find out more about health care abroad here 

The EHIC is not insurance and will not cover repatriation or private medical care, so you should still take out full insurance for you and anyone you travel with. You must declare all relevant medical information. You may also want to consider the level of cancellation cover, should you be too ill to travel.


If you need oxygen at home when at rest, you will need to arrange a supply at your destination. Always make sure you have details of your oxygen prescription with you.

If you’re holidaying abroad, provision of oxygen varies but you’ll probably need to hire it.

If your holiday is in the UK, you can contact your home oxygen supplier to arrange for your usual equipment to be provided. The supplier will need to know your destination address, a contact number and dates. You may need different equipment from usual – so it’s worth discussing your plans with your medical team.

Try not to leave it to the last minute to arrange your oxygen. It can take several weeks to get everything in place.

Fit to fly?

Air cabins are pressurised to the equivalent of 6,500-8,000ft altitude, and the level of available oxygen is reduced. This means that your breathing and heart rates increase to maintain oxygen delivery. If you have PF you may experience increased breathlessness, discomfort and a blueness to fingertips and lips.

There are risks to flying with a lung condition. You should discuss the flight with your medical team. They may suggest a hypoxic challenge test during which:

  • you breathe a mix of gases which simulates cabin oxygen levels
  • oxygen saturations are measured to determine whether you would need inflight oxygen is needed
  • the test tells you whether you can maintain oxygen levels under flight conditions, but it does not tell you whether you are ‘fit’ to fly. 

If inflight oxygen is recommended, check the airline’s policy and find out what paperwork they need. Most airlines will be able to advise on what special assistance is available. 

On the day of travel, if you are unwell or have an unstable medical condition, you should avoid flying.

For more about flying with oxygen visit the European Lung Foundationwww.europeanlung.org  and the European Federation of Allergy and Airways Disease

Oxygen suppliers in the UK

There are four companies in England that provide home oxygen services for the NHS. Each covers a certain geographical area. Your oxygen treatment clinic will organise your oxygen supply from one of the suppliers below:

Air Liquide covers London (0808 143 9991) and South West of England (0808 143 9999)

Baywater Healthcare covers North West England, Yorkshire and the Humber, West Midlands and Wales (0800 373 580)

British Oxygen Company (BOC) covers the East of England, East Midlands and Northern Ireland (0800 136 603)

Dolby Vivisol covers the South East of England, North East of England (0800 917 9840) and Scotland (0800 833 531)

Hiring oxygen overseas

Provision of oxygen abroad varies but usually needs to be hired. Your local oxygen supplier or oxygen service can advise. Don’t leave it to the last minute to arrange oxygen as it can take several weeks to get everything in place.

Healthcare in Spain has information about getting oxygen therapy in Spain. You can also visit private oxygen suppliers Oxygen worldwide and Omega oxygen.

Ian and Dorothy’s story

Ian and Dorothy, members of Newcastle PF Support Group, have undertaken a number of cruises, mostly from Southampton. They normally use a local travel agent to make the trip as stress free as possible and prefer to travel with the same cruise company.

Just remember everything is optional. Try to be flexible and enjoy the experience!

‘When booking, we would normally ask for a cabin near to a lift in the middle of the ship. On arrival at the departure hall we head to the Medical Assistance area. I require a wheelchair owing to the fact that you would normally have to use an air bridge to enter the ship, and the staff guide you through the booking-in process and also through security so there is no stress or drama about getting on board.

‘Excursions can be very tiring so we have found it is better to have a rest day between excursions, and check the tour brochure to see how strenuous they are.

‘Dining areas are often quite large, so you can request a table near the doors which does not require such a long walk from the lifts and restaurant entrance – this should be mentioned on your booking form.

‘Just remember everything is optional. Try to be flexible and enjoy the experience!’


Breathlessness can be a frightening and distressing symptom of pulmonary fibrosis. You can learn how to minimise, cope with and manage this symptom – and make a big difference to your quality of life.

The causes of breathlessness

We feel breathless when our muscles sense that they are not getting the oxygen they need. So they send a message via the nervous system to our brain, which tells us to breathe faster and work harder to get more oxygen.

There are many things that can cause breathlessness and it’s a good idea to have your breathlessness assessed by your medical team to make sure that there is no other medical cause. You might have a breathing test, a chest x-ray, blood test or have your oxygen levels checked.

Managing breathlessness

There are a number of things you can do manage your breathlessness:

Don’t overdo it!

It’s likely that you will experience breathlessness when you are physically active. With pulmonary fibrosis, the scar tissue in the lungs slows down oxygen entering the blood.

But if you stop what you are doing, you will notice that your breathing will slow down and you will recover quite quickly. You do, therefore, have some control over your breathlessness.

You will learn very quickly which activities you can manage. This will mean that over time your choice of activities may change. For example, you may still want to spend time walking your dog but may have to delegate cutting the six-foot hedge to someone else!

You can explore the amount you can manage – and increase it – by taking part in a pulmonary rehabilitation program.

Learn to slow down

If you start out slowly, you’ll be able to sense your breathlessness developing, a bit like a wave building up as it travels into the shore. Learning to sense the wave building up gives you a chance to remain in control. If you stop at this point and allow your breathing to settle, then you can start up again as soon as you feel comfortable.

If you feel in control of your breathlessness you are less likely to panic. Many people with pulmonary fibrosis learn to pace themselves by stopping and starting.


Help may also be at hand in the form of additional oxygen. Many people use oxygen for many years, just like any other treatment. Your healthcare practitioner will be regularly assessing you to see whether you might benefit from oxygen. 

Walking aids

A rollator supports your body weight as you walk. There are several different models; you might want to try one with a seat so you can have a rest if needed, or with a basket so you can carry oxygen. You could also experiment with a supermarket trolley when shopping to see if this helps you.

Some people also find carrying a stick provides the support they need, and helps them to feel less uncomfortable about going slowly when they are out and about.


Drugs can help reduce the sensation of breathlessness. Some of these drugs include small doses of morphine, which can also have side-effects of constipation. Some drugs work by reducing anxiety brought on by breathlessness. Talk to your medical team about drugs that might help.

Using relaxation

It’s not just physical triggers that can bring on breathlessness. When people experience a nervous or anxious situation, they often remember feeling breathless or being unable to speak.

Relaxation is useful for relieving anxiety and managing breathlessness, so try to find what relaxes you. It might be listening to a relaxation tape or app, music, or reading a book. It might just be spending time with your family and friends.

Distraction is also a useful tool to help people relax and forget about their breathlessness. Getting absorbed in something that you enjoy is a good way of switching off.

Fresh air

Opening a window and having cool air circulating can help breathlessness. Likewise, a fan can help to move air and feel refreshing around the face. There is some evidence that using a handheld fan can help reduce the unpleasant sensation of breathlessness.

Breathing exercises

There are also breathing techniques to help you use your lungs more efficiently and to feel in control of your breathing.

Breathing Techniques 

When you’re feeling breathless or anxious, there are breathing techniques to help you feel more in control and more comfortable. 

Breathing control

Breathing control is useful when you’re short of breath or feeling anxious or panicked. It helps you make the best use of your diaphragm, your main breathing muscle. 

Your diaphragm contracts when you breathe, so pulling the lungs down, stretching and expanding them. It relaxes back – into a dome position – when you breathe out, reducing the amount of air.

How to practise

The best time to practise is when you are relaxed and not out of breath, and can sit comfortably: 

  1. Get into a comfortable position, with your arms supported and your shoulders relaxed. Put one hand on your chest and the other on your abdomen. Close your eyes to help you relax and focus on your breathing.
  2. Slowly breathe in through your nose, with your mouth closed. If you’re relaxed, the air will reach low in your lungs. Your abdomen will move out against your hand. 
  3. Observe your breathing. If your breathing is controlled, the hand on your chest will hardly move. Breathe out through your mouth. Your abdomen will fall gently. Imagine all the tension in your body leaving as you let the air out. Use as little effort as possible and make your breaths slow, relaxed and smooth. 
  4. With every breath out, try to feel more relaxed and calm. Gradually try to breathe more slowly When fully in control of your breathing, your out-breath should be longer than your in-breath. There should be a natural pause at the end of your out breath. 

Feeling out of control 

For those times when you are too breathless or anxious to manage breathing control, simply:

  • Gently fan yourself
  • Lean forward
  • Focus on longer out-breaths

Pursed-lips breathing

Use pursed-lips breathing to control your breathing:

  1. Breathe in gently through your nose, then purse your lips as though you’re going to blow out a candle. 
  2. Blow out with your lips in this pursed position. Blow out only for as long as is comfortable – don’t force your lungs to empty any more than feels natural. 


Blow-as-you-go helps make tasks and activities easier – especially those that make you breathless. You can use it with pursed-lips breathing. 

Breathe in before you do the part of the task that involves making an effort. Then breathe out while you’re making the effort. 

For example, when standing up, breathe in before you stand up, and then blow out as you stand up. Try using pursed lips as you blow out. 

Paced breathing 

Paced breathing is useful when you are active, for example, walking or climbing stairs. You pace your steps to your breathing. You can use it at the same time as pursed-lips breathing and blow-as-you-go. Count to yourself as you walk or move. 

For example, breathe in for one step and then take either one or two steps as you breathe out. Take more steps as you breathe in or as you breathe out, if that feels better for you. Try different combinations to find what works best. 

Start singing!

Some patients have found singing to be beneficial, although it doesn’t suit everyone. A number of support groups have invited singing coaches to their sessions and found this very useful. 

You can also talk to your medical team about finding a pulmonary rehabilitationcourse – a programme of exercise and advice for people with lung conditions. Your pulmonary rehab course will include advice with managing your breathing to help with daily activities. 

Managing breathlessness when bending

You may experience breathlessness and discomfort when bending if you have pulmonary fibrosis. This is a common problem, as the action of bending squashes and restricts the muscles of breathing so they don’t work as effectively, making you feel more breathless. 

If you are carrying additional weight especially around your abdomen, the breathlessness is likely to feel worse.

Here are some handy tips for you to try:

Position yourself well

Avoid bending from the waist and bring the task up towards your waist if you can.

If you need to work at below waist level e.g. accessing a low cupboard, washing machine, dishwasher or whilst gardening, try using a low stool or chair and sit to do the task instead of bending.

If standing, ease into a crouch position, keep your back straight and bend from your knees. 

Use a litter picking stick to pick up the post, fill the washing machine etc.

Use a secure support if there is one available. This will allow you to keep your chest upright and shoulders back enabling you to breathe more comfortably. The support will also give you something to push against when returning to the upright position.

Avoid holding your breath when bending and use the ‘blow as you go’ technique. Try to breathe out on the effort part of the movement i.e. when bending forward  


Try to reduce how often you bend over by adapting the way you do an activity and/or using appropriate aids and equipment where able. 

Ask yourself:
‘Do I really need to do this?
‘Could someone else do this for me?’
‘Is there something else I would rather be using my time, energy and breathing to do?’


It is important to pace your activities and take adequate rest periods in between. 

Try breaking tasks up into manageable sections. Rest in a comfortable position in between and use relaxed breathing control to settle your breathing before you resume activity again 

Do the task differently 

See what gadgets and equipment are available that will make the job easier to do. e.g. helping hand grabbers, long handled gardening tools, using slip on shoes as opposed to lace ups.

A final word….

Remember none of the above will make your breathlessness go away.

It will take time and practice to feel comfortable and confident doing a task in a different way to how you have been used to doing it before. 

However, adapting and modifying the way you do a task may make it a little easier to manage, leaving you with more energy to do activities that you most enjoy.