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.

Oxygen

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.

Medications

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.

Oxygen

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.