Pulmonary Fibrosis NITreatments for Pulmonary Fibrosis.

Learn MoreTreatments for Pulmonary Fibrosis.

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.

Learn MoreTreatments for Pulmonary Fibrosis.

Medications

Drug therapy for pulmonary fibrosis is often specific for the type of PF a patient has. Many medications exist and are available in NI

Oxygen

Oxygen is is normally prescribed by the Specialist Respiratory Nurse as required

Lifestyle changes

It is imperative that individuals cease to smoke and structure a healthy diet to ensure their lungs are as efficient as possible

Rehabilitation

Pulmonary rehabilitation (PR) is a structured exercise program designed for people living with chronic lung diseases like pulmonary fibrosis

In DepthFind out more detail.

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.

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.

Idiopathic Pulmonary Fibrosis (IPF) is a scarring disease of the lungs of unknown cause. To make a diagnosis of IPF, your doctor will perform a thorough history to try to identify potential exposures or other diseases that might lead to scarring of the lung.

If diagnosed, over time scarring can worsen making it hard to take a deep breath. The lungs then cannot take in enough oxygen to oxygenate the blood. IPF is a form of interstitial lung disease, primarily involving the interstitium or the tissue and space around the air sacs of the lungs, and not directly affecting the airways or blood vessels.

There are various kinds of interstitial lung disease that can also be caused by inflammation and/or fibrosis. These types of IPF are treated a bit differently. It is important to work with your health care provider to determine if you have IPF or another form of interstitial lung disease. If a plausible cause is found, then you do not have IPF. The scarring pattern of IPF is technically called usual interstitial pneumonia (UIP). Your doctor will use detailed X-rays of your lungs called high-resolution computed tomography (HRCT) and sometimes a lung biopsy to look for this pattern. A diagnosis of IPF requires that your doctor cannot find a cause and the presence of a pattern of UIP on either HRCT or a surgical lung biopsy sample.

You may be more likely to experience poor mental wellbeing or a mental health condition if you are living with a lung condition. This might be because: you find it harder to do the same activities that you did before you became ill you worry that you will not be able to breathe you feel frustrated if you now need regular medical treatment and more support from others, or you worry about the future with your lung condition.

This could then have an impact on how you view your role at work, interactions with your family and friends, and your self-esteem. Having a mental health condition may also affect your ability to cope with your lung condition.

This might mean: feeling anxious about being out of breath, so you avoid exercise being depressed and not feeling able to take your medication, or avoiding social situations and feeling isolated. This creates a negative circle of events; if your symptoms are not well-managed, it could then make you feel worse.

Having issues with your mental health is very common and nothing to be ashamed of. It is good to be able to speak openly about it. If you feel like you are not coping, discuss this with your healthcare provider who will be able to talk through your options for finding support. In addition to this, you could also try the following. Find a support group for people living with your condition. This could be local to you, or an online community. Many people find it beneficial to talk to others who have similar experiences. Talk to your family and friends. Although they may not be living through the same experience, you can help them to understand what it is like for you.

You could discuss with them the ways in which they can help you and explain to them the times when you need your own space. They may be able to help you notice changes in your mood and if you need further support. “One thing I learned from my time spent networking with people from other patient organisations is the useful phrase – it is okay not to be okay. You have to allow yourself not to be okay at certain times in your life. It’s so difficult to be strong and cheerful and happy and okay all the time. You have to allow yourself to be in a bad mood. I have bad days sometimes – I don’t see anyone and they are days just for me. I cover myself in a blanket and stay home. I cry, I shout or I sleep if I have to. I allow myself not to do anything and then I wake up the next day and feel refreshed – it’s a new day and I almost feel reborn, I can be positive again and I feel like new.”

Elena Connect with patient organisations working to support people with your condition. You may find that becoming actively involved with these organisations gives you communication with a range of people with similar goals to you and also keeps your mind active as you work towards these shared goals.

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Learn MoreManaging Breathlessness.

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Breathing Habits

Unhelpful breathing habits will make you feel more out of breath. Breathing using all your lungs rather than the top can help.

Breathing Control

Breathing control means breathing gently, using the least effort. It will help when you’re short of breath or feeling anxious. The technique below is sometimes taught in yoga.

Pursed Lips

Pursed-lips breathing can be used at any time to help you control your breathing. Breathe in gently through your nose, then purse your lips as though you’re going to blow out a candle.

Recovery

Stand leaning backwards or sideways against a wall. Have your feet slightly apart, about one foot or 30cms away from the wall. Relax your hands down by your sides.

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