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What is Pulmonary Fibrosis?

Pulmonary = Of the Lungs
Fibrosis = Scarring
So Pulmonary Fibrosis means scarring of the lungs.

Why is this a problem?
The lungs transfer oxygen molecules into the bloodstream, we all need oxygen.
When lungs are scarred they don't transfer as much oxygen.

In many forms of Pulmonary Fibrosis scarring spreads meaning even less oxygen gets into the blood.

Result: we breathe more to get more oxygen in. This strains the lung muscles.

And why do people get scarred lungs:-
Many causes, including the use of drugs, radiation treatment, allergens, dust etc.
Many cases - no obvious reason - this is called Idiopathic

A guide to Pulmonary Fibrosis

This short guide is intended for people and carers encountering the term Pulmonary Fibrosis for the first time. It has been compiled by Malcolm Weallans, a person who was diagnosed with Pulmonary Fibrosis diagnosed at the age of 59. He has learnt a lot about the illness through talking to a wide range of people with a particular interest in the subject and many people who have never heard of the illness. Malcolm has been a patient contributor to the NICE (National Institute for Clinical Excellence) guideline for the diagnosis and treatment of Idiopathic Pulmonary Fibrosis(IPF), the NICE quality standards for IPF, a NICE technical appraisal of a drug for the treatment of IPF, the NHS England Clinical Reference Group for Specialist Respiratory, and the British Lung Foundation advisory panel for IPF. Malcolm has also been inovolved with people through various social networking groups and as one of the founders of Pulmonary Fibrosis Trust, the first national charity in the United Kingdom which aims to help people who are coming to terms with the diagnosis and help people cope with some of the financial challenges of living with the illness.



What exactly is Pulmonary Fibrosis? And what is IPF?

To understand what Pulmonary Fibrosis is will involve learning about the structure and purpose of the lungs.

The lungs are one of the vital organs of the body. They are responsible for breathing in air, extracting oxygen molecules from the air, transferring the oxygen into the bloodstream, extracting the carbon dioxide from the blood stream, and exhaling unused gases into the air. The structure which allows this to happen is very complex, but as most of you know we have two lungs. When we breathe in from the nose or the mouth air is transferred by a complex system of tubes which get steadily smaller until they reach small little airsacs which are called alveoli. There are millions of these alveoli in each lung and it comes as a surprise when people learn that the surface area of all the alveoli in both lungs is approximately half the size of a tennis court.

One of the best diagrams which explained this, showed an inverted tree with two main branches and many sub-branches getting smaller and smaller until they have small buds on the end.

The gaps between the alveoli are much smaller than the size of a human hair and are interlaced with very minute capillaries. The material between the alveoli and the capillaries is called the interstitium. One way of thinking about this is to think of a sponge. There are lots of very small pockets separated by very thin layers.

Most lung problems are caused by blockages of the tubes. This is commonly referred to as COPD (Chronic Obstructive Pulmonary Disorder) often also referred to as emphysema. Temporary blockage of the tubes is called Asthma. Other illness which affect the transfer of oxygen into the blood or the transfer of the use gases from the blood is called an Interstitial Lung Disorder (ILD). Pulmonary Fibrosis is one form of ILD.

With Pulmonary Fibrosis the interstitium shows signs of scar tissue. Many of you will have injured yourself in the past and normally the scarring disappears over time. However with Pulmonary Fibrosis the scarring of the interstitium does not disappear, and can even spread. Just as nobody knows how to stop scars forming when we cut ourself, nobody knows how to treat the scarring in the lungs.

Some of you will be wondering why we have mentioned IPF but I haven't really explained what IPF is. The “I” in IPF stands for Idiopathic. This is a medical way of saying they don't have any idea why a person is suffering from Pulmonary Fibrosis.

What are the symptoms of Pulmonary Fibrosis?

The most common symptoms of Pulmonary Fibrosis are that the person is breathless, sometimes only on exercise. Sometimes there are tell-tale signs in the nails and the ends of the fingers, something called "clubbing" where the nails are bent. Sometimes there will be a crackling noise that can normally only be heard through a stethoscope and is only normally detectable by a well-trained ear. The presence of just one of these symptoms can be indicative of other illnesses which makes Pulmonary Fibrosis and other Interstitial Lung Disorders very difficult to diagnose and treat. Many people also report that they have a persistent cough or spells of coughing.

How is Pulmonary Fibrosis Diagnosed?

The diagnosis process for Pulmonary Fibrosis normally starts by a person visiting a General Practitioner complaining that they are constantly breathless or become breathless when they exercise, or when they do basic household tasks. Some people don't have any symptoms but some of the signs of Pulmonary Fibrosis become visible when the person is being examined for some completely unrelated reason.

Both the NICE guideline for IPF and the more general NHS England service specification specify that diagnosis should only be made by a specialist centre where this is a suitably qualified Multi-Disciplinary Team(MDT) with experts in pulmonary radiology, pulmonary pathology, and clinicians who see more than the occasional person with an interstitial lung disease. Patients have worked hard to make sure that this is the case because far too many people are being misdiagnosed by local GPs and hospitals with resultant incorrect treatment and increased alarm for people. The multidisciplinary team will use a high resolution CT scan, the results of blood and many other tests, and the clinical history of the person to help them form a consensus diagnosis. If they are unclear they might want to have other tests, often including checks on the heart, bronchial lavage, or even a surgical biopsy. Only when they are completely satisfied with a consensus diagnosis should they tell the person. And at that point the person and carers (if the person wishes) will be told the diagnosis and given verbal and written information on what the illness entails and future courses of action.

If a cause is known the clinician should tell the person what has caused the scarring of the lungs. There are many possibilities. Some drugs have been known to cause Pulmonary Fibrosis in some people. Exposure to certain types of animal, mould or dusts are also known to cause Pulmonary Fibrosis. Pulmonary Fibrosis has also been strongly linked to various connective tissue illnesses including Rheumatoid Arthritis. In about 5% of cases there is a link between members of the same family and not always between people who are blood related. With a large number of people the clinical team can find no reason behind the Pulmonary Fibrosis and people are therefore diagnosed as having Idiopathic Pulmonary Fibrosis. (Idiopathic is a medical way of saying "we don't know why".

And even if the diagnosis says that a person has IPF there are still more differences. Pulmonary Fibrosis is a term that clinicians use to describe the illness. But there are two distinct radiological patterns that have been seen with Pulmonary Fibrosis. These are referred to as Usual Interstitial Pneumonitis(UIP) and Non-Specific Interstitial Pneumonitis (NSIP). Some clinicians have been known to use these terms when telling people what is wrong with them. UIP is much more common than NSIP and much of the research has been conducted only on people with UIP. A person can have UIP or NSIP and it is still not IPF if there is reason to believe it has been caused by other things.

Lung Function Tests (LFTs)

Part of the diagnosis process is often to perform Lung Function Tests. These are a set of tests that are performed to try to give the Multi-Disciplinary Team (MDT) an idea of how far the fibrosis has spread though the lungs and what effect that has had on the volume of the lungs, and the ability to transfer oxygen into the blood stream. Some of these tests are performed in a sealed glass cabinet so that they can detect changes in air pressure and from that calculate the effective volume of the lungs. Other tests are performed on the same equipment but with the door open, and a third set of tests may also be performed on a different set of equipment all of which is linked up to a computer system which is operated by a pulmonary physiologist. These tests are often repeated at regular intervals during your treatment so that the clinician can see how fast the condition is developing and apply the right sort of treatment at the right time.

The results of lung function tests can help to indicate how far the illness has progressed. There are particular measures that the clinicians use to help them. They normally look at these by comparison with predicted values based on height, weight, and age and quote the values as a percentage of the predicted value.

The main values that seem to be significant are FVC (forced vital capacity), FEV1 (forced expiratory volume) and TLCO (transfer factor for carbon monoxide). These do get lower as the disease progresses but there does not seem to be any correlation between these values and life expectancy.

Treatment for Pulmonary Fibrosis and IPF

Until quite recently treatment for Pulmonary Fibrosis was confined to treating the underlying cause. However doctors had no evidence of how to treat Idiopathic Pulmonary Fibrosis so they tried using the treatments that seemed to work for Pulmonary Fibrosis where a cause had been identified. This was very hit and miss and was not based on any industry accepted evidence.


Pharmacological treatments

The only approved treatment for IPF is a new drug called Pirfenidone. This drug has only been generally available since 2013 but was being used in clinical trials for a few years before. This drug can slow down the progression of IPF but will not always do so. There are also significant side effects. For that reason the drug can only be prescribed by specialist centres where there is a suitably qualified Multi Disciplinary Team and after this team is convinced that the diagnosis is IPF. No tests have been performed on people with other forms of Pulmonary Fibrosis so it has yet to be licensed in those areas.

Many specialists have found that the use of Steroids has helped some people with Pulmonary Fibrosis. Again there are significant side effects especially when used at high dosage levels and or long term. Clinical Trials of using high dose steroids to treat IPF have shown that this treatment is harmful and therefore the use of long-term high-dose steroids is not recommended by the British Thoracic Society. However short term use is still used where the specialists think that there is inflammation. Steroids are also used for the treatment of other forms of Pulmonary Fibrosis, especially where there is another underlying condition.

Another drug that has been used in the past is azathioprine. This is more often used in conjunction with steroids as it is thought to counter some of the side effects of the steroids. In tests with people with IPF there was no evidence of the effectiveness of this drug.

By far the most common treatment for IPF has been N-acetyl cysteine. This drug is used when the person is coughing up mucus and helps to thin the mucus making it easier to cough up. There is no evidence to support the use of this drug as most clinical trials do not look at the severity of the cough when assessing evidence.

The use of steroids, azathioprine, and N-acetyl cysteine was quite common and was widely though to be a successful treatment until 2012 when a large trial of this in people with IPF showed some very negative results.

Clinical Trials
There are many other drugs being tested in clinical trials to see if they can impact any of the symptoms of pulmonary fibrosis. Most of these are in the Idiopathic Pulmonary Fibrosis arena as that is where there is a perceived greater return on investment for the pharmaceutical companies. Generally research in the area of pulmonary fibrosis is directed at the underlying cause of the fibrosis.


Other treatments

Supplementary Oxygen
Many clinicians and respiratory nurses believe that providing additional oxygen helps people who are suffering with pulmonary fibrosis as it can increase the amount of oxygen being provided to the lungs and therefore helps to counter the breathlessness. There are two types of supplemtary oxygen, Ambulatory, and Long-Term. How the medical profession decide on what levels of supplementary oxygen are required is determined by measuring blood oxygen levels. This is done using a pulse oximeter, a small probe which is placed on the end of a finger which measures the pulse rate and the percentage of oxygen in the blood. Alternative, but more painful, tests involve taking blood samples from the earlobe or the wrist. If the blood oxygen levels at rest show that there is less than 90% of oxygen in the blood then long-term oxygen therapy will be recommended. By repeating the tests with the person having supplementary oxygen at different levels it is possible to determine what level of long-term oxygen is required.

Many people require additional oxygen when they exercise so all people are also tested to see what levels of ambulatory oxygen are appropriate.

It should also be noted that any form of oxygen therapy should only be used where are are no naked flames, and should certainly not be used whilst smoking or near smokers. This is because the combination of oxygen and flames of any sort could cause significant amounts of damage to all concerned.

Long Term Oxygen Therapy
Long term oxygen therapy is provided by the use of Oxygen Concentrators. These are machines about the size of a small fridge which use compression techniques to extract higher levels of oxygen. Patients are often told to use this for a certain number of hours daily and/or overnight. Oxygen cylinders are also provided as backup in case of power outages. Patients should also notify their electricity supplier that they are on long term oxygen therapy so that they will be a priority for reconnection, and to receive assistance with electricity bills.

Whilst every effort will be taken to ensure that oxygen concentrators are made available to all there are some places where they cannot be used for health and safety reasons. These include difficulties when the person lives in an upstairs flat.

Ambulatory Oxygen
So that people can move around and continue to exercise people may often be prescribed ambulatory oxygen. This can be in addition to long term oxygen therapy. This relies on portable oxygen equipment, which can be provided by portable cylinders or by concentrators which extract oxygen from the air. How much oxygen people need varies according to the progression of the disease and the types of exercise. Various methods are used to estimate the required levels and the testing process might include a six-minute walk test where people walk as far as they can in 6 minutes and their blood oxygen levels are monitored during this time. If the levels drop significantly then oxygen therapy will be provided. For ambulatory purposes the oxygen is normally provided through a nasal cannula. It has recently come to light that nasal cannulae are only effective at level of 6lpm and below, otherwise a mask is recommended.

The types of ambulatory oxygen provided vary from portable oxygen concentrators to portable cylinders, and some systems use a refillable container which has to be topped up from a storage tank.

Portable Oxygen concentrators vary in weight and flow rates. For low flow rates (2 and 3lpm) there is a wide choice of equipment, some of which is much lighter than portable oxygen cylinders, but for higher flow rates the equipment is heavier and more likely to be transportable rather than portable. Most portable concentrators can be operated from a cigarette lighter in a car and all of them can be operated from batteries. But of course the higher flow rates rerquire heavier batteries.

Oxygen cylinders come in two types, compressed oxygen and liquid oxygen solutions. Liquid oxygen cylinders are lighter but require a back up storage tank so that they can be refilled. Whilst oxygen cylinders are available for higher flow rates than portable concentrators but are often restricted in cylinder duration at higher flows.

Liquid oxygen cylinders are normally only provided for people living on ground level as the storage tanks are very heavy and are normally kept outside the house.



Pulmonary Rehabilitation

Most medical professionals are in favour of all lung people taking part in Pulmonary Rehabilitation. The implementation and availability of this varies according to which part of the country people live in. There is no evidence that Pulmonary Rehabilitation will lengthen a person's life but it is generally believed that people who do undergo pulmonary rehab will get benefit from it. The primary benefit being that they will be better able to cope with some of the side effects of their illness, which in the case of Pulmonary Fibrosis means that the people can cope with their breathlessness and know how to control their cough. There has been little research into what Pulmonary Rehabilitation is appropriate for pulmonary fibrosis people and work is underway to start defining this.

Lung Transplant
A few people who suffer from Pulmonary Fibrosis may be suitable for lung transplantation. This is not available to all people and is further restricted by the acute shortage of suitable lungs for transplant. Whilst a lung transplant has been very successful for many people they still need to take anti-rejection medications and undergo many regular tests for the rest of their lives. It was described by one person as “swapping one illness for another”. Not all lung transplants are successful and many people die waiting for a transplant.

Living with Pulmonary Fibrosis
Links

Living with pulmonary fibrosis challenges people on a daily basis but with a little care and planning it is possible to continue with a fairly normal lifestyle.

Being diagnosed with a long term condition does take some getting used to. Don’t rush into things or make sudden changes in lifestyle.

Taking care of yourself

There are few dos and don’ts as far as Pulmonary Fibrosis is concerned. But if you are a current smoker it does make sense to stop if you can. There is no proof that smoking causes pulmonary fibrosis and there is no proof that it doesn’t. It just seems fairly obvious that adding fumes of any sort is not conducive to healthy lungs.

Similarly take precautions to protect your lungs from pollutants and even aerosols. Some people have reported difficulty with air freshening sprays; others with diesel and petrol fumes; and some have reported difficulty with animals and birds.



Dietary hints

Many people have experienced problems with weight. In some cases people have put on weight, whilst others have lost weight. It is a well-known side effect of steroids for people to gain weight, and others people have experienced problems with weight loss.

It is important that you should eat as healthily as possible and a well-balanced diet is recommended. However if you are losing weight without trying you might find that eating small meals and supplementing this with snacks between meals might help. And if eating unhealthy foods stimulates your appetite it is better that than not eating enough.



Exercise

Any exercise will help. Some of the people who have survived longer report that they exercise regularly. Fit muscles don’t need as much oxygen to function. It doesn’t matter that you get breathless doing it. In fact getting breathless two or three times a day will help to keep your breathing muscles fit.



Monitoring your health

It is very important that you should know your body and be alert for changes. Knowing your weight is important, as is looking for changes in appearance.

If you suffer from blood pressure problems keep a close watch on it. It can really help.

And know as much as you can about the lungs. Think about how you breathe. It is important that you breathe from the diaphragm and not from the shoulders. Breathing from the diaphragm gets more air into the lower lungs.

As a test lie down somewhere and place one hand on your chest and one on your stomach. As you breathe in your stomach should rise and when you breathe out your stomach should lower.

Little helpful tips

A long towelling dressing gown can be quite useful to put on when you get out of the bath or shower. It means you don’t need to rub yourself dry and that saves energy.

Take your time washing and dressing if possible. Washing and dressing involve a lot of bending which places additional strain on your lungs. Where possible try to spread the task over a longer period of time. Even try sitting down as much as possible while washing or dressing or asking your occupational therapist for a perching stool or shower board.

Try to make trips upstairs as efficient as possible. If you need something from upstairs try to think if there are other things that you might need later in the day. Just going up and downstairs can require a big effort.



Holidays

Holidays can be very rewarding. Try to do what you want. You may need to do it more slowly. If you are flying it would be worth doing a fit to fly test first. That will tell you if you need supplementary oxygen whilst on the plane as the oxygen content on a plane is equivalent to being at 8,000 feet above sea level and the air is a bit thinner.

If you are on oxygen planning ahead is important. Your oxygen supplier will be able to arrange for a supply of oxygen at your holiday location if you can give them enough notice. At least 3 weeks is recommended. There may be a charge for this payable locally.

It is also important to let the airline know if you need oxygen on the plane. Not all airlines provide this and many of them charge extra for the facility. Some even have restrictions on how many people they have on a plane with supplementary oxygen requirements. Again telling them when you book the flight is recommended and make sure they have enough notice.

One of the challenges of going out of the country with supplementary oxygen is that the oxygen equipment is owned by the oxygen companies and they are not willing for you to take it out of the country. They can arrange for you to have a supply at your destination. If that is the case you may be able to get help from the Pulmonary Fibrosis Trust who offer an oxygen away from home service which can provide a portable oxygen concentrator for the duration of your holiday. Flow rates for portable oxygen do present challenges so talking to the trust a few weeks prior to needing the facility is strongly advised.

Tracie's Reflections

You look at me and see my face, my body, my hair, and you feel my warm embrace;
what you don't see, behind my smile and my laughter is the stress and the pain of this life ever after.

It's worriesome to know my breathing is off, my lungs are becoming crinkled and my heart is enlarged;

not to mention my brain has taken a beating, to the point of not remembering only this mornings meeting.

I watch people look at me when I park in the disabled, I believe I know how they think it is wrong, they judge me because, yeah I look still quite strong. But inside I'm falling apart at the seams, my life is barely recognisable to me, I used to, I used to, I used to, I used to be free...

Free from the oxygen tank I must carry in order to work and to walk around the town, or to sit on a bench when my O2 is down.

It helps, yes it does and I'm grateful for it. But damn, what happened to the five to six miles I used to walk? Around the block now and my breathing is altered, if a hill presents itself then I know I'll be slaughtered.

So, you people who look at me and think I look well, I am well, in deed as well as I can be, but that's not the best, and I guess right now it's not the worse. I may not be carrying a stick or my leg in a cast, my arms might be able to function as normal. But my lungs, oh my lungs they just won't work right, and now I'm exhausted by seven each night.

No partying for me, no Zumba class either, just me in bed with a cup of tea, some biscuits or chocolate to help me forget that "Bertie" is my oxygen cylinder and not a new lover;

He's the one who keeps me alive throughout the night, not a man with strong arms wrapping around me, a cannula gushing oxygen up my nose.

His isn't the pleasure I used to receive, but for now he is mine and I have learned to accept him with ease.





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