The Equality Act 2010 states that and must make reasonable adjustments to ensure that employees with physical or mental
health conditions are not substantially disadvantaged. This also applies to people with PF. So, talk to your employer. You
might also want to discuss your situation with your family to discuss your options. You can also talk to the Benefits
Department if you need to finish work. Please see our section on benefits below.

While some rarer forms of PF run in families (Familiar Pulmonary Fibrosis), it is not yet known whether IPF and other
forms of PF are hereditary.
Dr Helen Parfrey has been involved into research at the Royal Papworth Hospital and says:
“Importantly studying families with PF has led to the identification of mutations in specific genes that increase the risk of
developing IPF, however, it remains to be determined how these risks interact with other factors such as smoking,
occupational exposures or viral infections, to cause pulmonary Fibrosis”.

ILD and PF are not the same thing. PF is one type of ILD.

The Interstitium is the network of tissue around the air sacks in your lungs, where oxygen and other gasses enter and leave
the lungs.
ILD’s affect this network of tissue. There are many types of interstitial lung dieses. Some ILD’s cause lung scarring
(fibrosis), such as IPF and asbestosis. Other forms of ILD’s cause inflammation (The body’s reaction to injury) but not
lung scarring.
Some ILD’s cause both inflammation and lung scarring, including sarcoidosis and hypersensitivity pneumonitis
The distinction is important because scarring (fibrosis) is permanent and may get worse, whereas inflammation can
improve with treatment.

• Asbestosis
• COVID 19 related PF
• Drug Induced PF
• Hypersensitivity PF
• Idiopathic NSIP
• IPF
• Pneumoconiosis
• Rheumatoid Arthritis ILD
• Systemic Sclerosis
• Sarcoidosis
• Silicosis

No. There is currently no cure for PF. However, there is a lot of ongoing research, which is leading to improvements in
treatment and care for patients.

This can be very difficult to predict as it depends on several factors.
It can depend on what form of PF you have. It depends on whether you have IPF or another condition, your general health,
and your age.
The severity of PF at diagnosis will also make a difference. The earlier you are diagnosed the longer you will survive, and
your medical team can make sure you have the right treatments at the right time.

A sudden worsening of PF is called an exacerbation, it can occur at any time and is associated with a poorer outcome.

Both are lung diseases which can make it difficult to breath, but they are not the same. COPD is a chronic obstructive
pulmonary disease. With COPD the airways tubes are swollen and narrow and produce excessive mucus, leading to a
chronic productive cough. Most COPD cases are caused by smoking. There about 1.2 million people living with COPD in
the UK.
PF is a group of lung conditions which cause scarring in the lungs. The cause of scarring depends on your PF condition.
There are around 70,000 people with PF in the UK.

Antifibrotic drugs are used to slow down the progression of fibrosis in the lungs.
For all forms of PF except IPF, these drugs are prescribed by your consultant on diagnosis.
IPF patients are only prescribed these drugs when their lung function has dropped to below 80%. The drugs are also not
prescribed to patients with IPF who have a lung function of 50% or less.

This is high blood pressure in arteries which supply the lungs. Pulmonary hypertension can be caused by PF and can be
very dangerous.

Smoking is a risk factor for PF patients, you should stop immediately.

Studies have shown that people who have smoked in the past are more likely to PF than people who have never smoked.
Cigarette smoke can make your lungs inflamed and increase mucus production. It can make your PF worse and increases
your chances of getting lung cancer, COPD and heart disease.

There are no formal recognised stages of PF. Every person’s lung disease is different. But it is known that your PF is likely
to progress over time.

PF and IPF are progressive diseases, which means that scarring in your lungs will get worse.
Anti-fibrotic drugs, exercise, oxygen therapy and a good diet can all help to prolong your life.

Sex is an important part of life for many people, but you may worry that it will make your symptoms worse. Because PF
can cause breathlessness and leave you short of breath, it may affect your sex life. But just like any other physical activity,
you can use your breathing control techniques, to help your breathing return to normal.

As well as managing your PF properly, there are several things you can do to improve your sleep:
Ensure your head is higher than your body. Use pillows or a wedge to keep you there as this opens your airways. Sleeping
with your head raised may reduce acid reflux, which can affect your sleep.
Keep a regular sleep schedule.
Avoid caffeine and alcohol at least 6 hours before bed.
A light rather than a heavy meal before bed.
Have a hot bath, a bath 90 minutes before bed helps.
A quiet and tidy bedroom will help you sleep.
Reduce blue screen time two hours before bed.
Learn relaxation exercises.
Many people with PF get sleep Apnoea. Symptoms include snoring, pauses in breathing when asleep, struggling to breath
and tossing and turning. If you think you are suffering from Sleep Apnoea talk to your GP for help.

It is important that pulmonary fibrosis patients are vaccinated as they tend to suffer from respiratory infections. This would
mean a yearly influenza Jab and a Pneumococcal vaccination every 5 years. Also, it would be important to get vaccinated
against COVID-19 and to record dates/times of jabs and to have a “booster” vaccine in a timely manner.

Telemedicine is a relatively new approach to healthcare, which involves the use of technology by healthcare professionals
which includes telephone and video consultations with a nurse or doctor. To be able to attend a telemedicine consultation
it is necessary to have a computer or either a smartphone, tablet, or a phone. Some of the advantages of telemedicine are it
provides services to patients without requiring face to face contact, which is helpful in terms of safety both to patients and
hospital staff, especially in terms of COVID-19 and it is also helpful to patients living in remote areas. Furthermore, it can
be of benefit as there is no need for travel to attend hospital appointments and this consequently saves time. Another
advantage for some patients is in the use of remote monitoring of pulmonary fibrosis through an app on a smartphone
which links with the healthcare provider and keeps track of your health. The app can be very useful in recording both objective information such as FVC levels and subjective information on how you are feeling. Such apps are very useful in
helping patients self-manage their condition.
A disadvantage of telemedicine is that with a telephone consultation without video, body language and facial expressions
cannot be captured. Also, it will not be able to see any physical changes to your appearance such as lip color. A further
disadvantage is that it is hard to include others such as caregivers in the telephone consultation.

Pulmonary function tests are very important because they measure how well the lungs are doing. There are a variety of
tests to measure different aspects about the health of the lungs. After a diagnosis of pulmonary fibrosis, the function of
pulmonary function tests is to keep track of the progression of the condition. This rate of progression varies from person
to person, so the pulmonary function tests enable the healthcare team monitor and best manage the condition. The key
tests are known as spirometry. This for example requires breathing in fully and then breathing out as hard as possible, it
requires a lot of effort, however, they provide very accurate results on the lung condition.
The following spirometry tests provide the main measurements of lung condition.
FVC (Forced Vital Capacity) – This is the total amount of air that you can manage to breathe out from your lungs, after
breathing in as much as possible. It is important to keep a track on your FVC levels as it is both helpful to you and your
healthcare team in monitoring your condition. As the disease worsens FVC levels tend to reduce, which means that the
scarring of the lungs has deteriorated causing more difficulty for expansion as you breathe in.
FEV1 (Forced Expiratory Volume in 1 Second) – This means the amount of air you can breathe out of your lungs within
one second, it measures blockages or narrowing of the airways. In situations where you have co-morbidities such as COPD,
this test can be very useful.
DLCO (Diffusing Capacity of the lungs for carbon monoxide) – This is used to see how well the air-sacs (alveoli) inside
the lungs are performing. It refers to where the oxygen in the air sacs moves from your lungs into the bloodstream, and the
waste gas (carbon dioxide) moves from your blood into the lungs.
When pulmonary fibrosis worsens, the scarring of the air sacs gets thicker making it more difficult for oxygen and carbon
dioxide to move across into the bloodstream. Make sure to ask a member of your healthcare team to explain to you what
the results mean.

The six- minute walk test (6MWT) is a relatively straight forward test that is widely used to assess the functional exercise capacity of pulmonary fibrosis patients. During the test, a patient is asked to walk as far as possible over six minutes on a hard flat surface. Over the period of the six minutes both heart rates and oxygen levels are monitored usually with a pulse oximeter. It provides objective measurements of oxygen saturation, for example if oxygen levels show a level of less than 88% it maybe an indication of a need for oxygen and a worsening of your condition.

This treatment may be offered to you to help you breathe, especially if the level of oxygen in your blood is low when you
are resting, or in situations where your oxygen levels drop too much when you are active. You may need to use oxygen
only when you are doing physical activity or you may be prescribed oxygen to use all the time, it depends on how advanced
your condition is.

Prepare questions in advance to ask at the appointment
• Have someone with you, take notes if necessary
• Ensure you fully understand what is said to you. If you do not understand, ask GP/consultant for clarification
and to restate it in non-medical terms
• Ask about how your pulmonary fibrosis is likely to progress
• Who to contact in relation to post appointment i.e.: exacerbation/queries?
• What should I do in the event of an emergency? i.e.: Acute exacerbation
Note: An exacerbation of pulmonary fibrosis means a somewhat worsening of the condition by recurrent respiratory
infections and an acute exacerbation means a serious sudden deterioration of the condition. It is essential to get
urgent attention for an acute exacerbation.
Aim to provide as much information as possible to your healthcare team, such as any previous surgeries or treatments,
food/drug allergies, health conditions that run in your family and any lifestyle changes/habits that may be relevant to
your health condition.

It is very important for pulmonary fibrosis patients to exercise daily so as to maintain a level of fitness to keep muscles in
good condition. This helps in preventing the body becoming deconditioned. Should you become deconditioned, exercise
can be helpful to you. If you are going for walks. start with what you can do and gradually build up the number of steps
walked. Set goals for yourself and aim to adhere to them. Exercise can reduce fatigue and breathing exercises can help in
the reduction of anxiety. Start with walking the number of steps you can manage, and gradually increase the amount when
you feel up to it. When walking outdoors ensure you are protected against adverse weather conditions, it may be preferable
at times to walk in an indoor setting. You may be able to take part in a pulmonary rehabilitation course to help maintain
your fitness.

Pulmonary rehabilitation is beneficial to pulmonary fibrosis patients both from a physical and psychological point of view.
It is very important to be referred to pulmonary rehabilitation as soon as possible after being diagnosed with pulmonary
fibrosis. Your consultant could write a letter of referral to help you avail of such a program. It usually involves attending
at least two exercise sessions (it may be an on-line training) on a weekly basis, it consists of aerobic and resistance training
and exercises to improve balance/flexibility. For example, it is recommended to do 150 minutes per week of moderate
intensive exercise to get maximum benefit. Ideally the pulmonary rehabilitation exercises should be specifically for
pulmonary fibrosis patients to meet their needs. It is very important to continue to exercise because pulmonary fibrosis
patients can lose the benefits of pulmonary rehabilitation after six months.
To exercise safely avoid strenuous exercise if you are feeling unwell. If on oxygen, use it as prescribed. It is important to
pace yourself and allow recovery time between exercises. Exercise training is safe and effective, it has demonstrated
improvements in breathlessness, exercise capacity and Quality of Life. (QoL) Furthermore, it helps with maintaining a
healthy weight, circulation, muscle strength and endurance.
The keys benefits of pulmonary rehabilitation are
• It improves physical strength
• It also helps to improve symptoms such as fatigue and breathlessness
• Assists participants in learning to exercise safely
• Helps to reduce amount of infections
• Provides an opportunity to meet and interact with others in similar situations
• It is tailored to suit your respiratory condition
• It helps to increase quality of life
• Tends to reduce tension and anxiety
• It helps educate participants in how best to look after their condition

In addition to exercise, pulmonary rehabilitation Singing for Lung Health can be another method of helping you to live
better with pulmonary fibrosis, both from a physical and psychological point of view.
The main benefits of Singing for Lung Health are.
• It improves breathing control and distracts from breathlessness
Improves social participation and reduces feelings of isolation
• It provides tools for self-management of posture, breath, and anxiety
• It has a high uptake and retention
• Participants find it fun and an enjoyable form of activity
• It is a high value intervention and the delivery of it is low cost.
Singing for Lung Health (SLH) program focus on the learning techniques around breathing control and posture that are
considered necessary to sing effectively. Singing helps to strengthen the muscles that the human body uses to breathe. It
means that you can learn to breathe more deeply and slower than before. As a result of the strengthening of these muscles,
it helps to control your breathing. It also helps to strengthen your voice.
The program involves “warm up” sessions prior to singing, the focus is on a “diaphragmatic” breathing pattern which is
seen as helpful. It involves facilitating outward movement of the abdomen while reducing upper ribcage movement during
inhalation. (This represents the movement of the surrounding air through the airways and into the alveoli of the lungs,)
Generally, it means introducing a range of vocal sounds to warm up the voice through a range of different vocal qualities,
such as; range, rhythm and pitch. Appropriate songs are chosen (“fit for purpose,”) so that all participants can join in. The
longer the duration of the program tends to result in added benefit. Likewise, participants who continue to practice the
techniques at home find it to be an additional value.
Singing for Lung Health (SLH) is an additional tool you can use to help you live better with pulmonary fibrosis. It is not
a replacement for pulmonary rehabilitation, which has a very important part to play in helping you with your condition.
Nevertheless, it has the potential to lead to improvements in physical and psychological health, social well-being, and
overall quality of life. Participants feedback on SLH perceive it to have mental and physical benefits, improved well-being,
how to breathe properly, improved posture, also social benefits, and the desire for such group activities to be continued.

Linked with singing, there are also merits in using a musical instrument such as a “pulmonica,” (pulmonica.com) which
builds lung strength, loosens congestion, and helps to relax both mind and body. Always check with your healthcare team
first before commencing any of the activities mentioned above.

Psychological support can take different forms as P.F. patient needs vary. For example, breathlessness is common to most
P.F. patients and such sensations tends to lead to anxiety, low mood, negative thoughts and in some cases anger. There is
a need therefore to provide both physical and psychological support to cope effectively with such symptoms and other
related health issues. Having a multidisciplinary and an interdisciplinary team (dieticians, radiographers, clinical scientists,
GPs and other clinicians) play an important part in meeting such needs. Unfortunately, such patient centered approaches are not sufficiently available throughout Europe at present. If you are struggling to get help through your GP, please talk
to one of the Directors of the NTPFSG to find out how they can help.

There are 5 ways to improve our mental health and well-being; 19
• Connect with others in whichever way is possible.
• Be Active – Find ways to be active daily.
• Take Notice – Become more aware of what is going on around you.
• Keep Learning – It is good for your mental health.
• Give – Be kind and caring to others.
Remember, “it is okay to be not okay”, if you feel that you are not coping well it would be important to have a discussion
with a relevant healthcare professional before your mental or physical symptoms worsen.
There are other ways to look after your well-being, for example, mindfulness can quite helpful. It is a kind of meditation
which is widely practiced in medicine, mindfulness has proven mental health benefits. Put simply, it is a process of learning
how to find a focal point for calming the mind and becoming aware of one’s thoughts, sensations, and breath. It means
focusing on the present moment. Although some do not find it easy at first, many find that it is a help to them.

The term mental wellbeing describes how you are feeling and coping with daily life and how you feel living with P.F.
Good mental health means that you are more likely to be taking good care of yourself and managing your treatment well.
On the other hand, if you are experiencing poor mental wellbeing, you may be less interested in the things you previously
enjoyed and you may feel more anxious, tense, have difficulty coping and the stress of living with P.F. The challenges of
living with P.F. can be quite difficult, so there is a need to look after our self-care and our mental well- being. (It can also
be challenging for carers, so the suggestions outlined below may also apply to them.) That means making time to look
after yourself with a routine of healthy habits and practices. The way to do that is to start with what you are already doing
and build on it. One of the most important points to bear in mind is to be kind to yourself. Rather than thinking negative,
think realistically about what you can do. Set achievable goals, they can be short and manageable. (For example, going for
a 10-minute walk, 3 times daily.) Avoid using the word “try” when goalsetting. Make time to relax, connect with others,
engage in doable hobbies and interests, place some structure and routine into your daily life and ask for help if needed.
How we think matters, because it can influence our feelings, actions and our views of ourselves and others. Where possible
avoid thinking traps that cause you to jump to conclusions. Such negative shortcut ways of thinking tend to make you feel
anxious, fearful, or reluctant to try something new. In situations where your thoughts or worries are affecting your quality
of life, or you are finding it difficult to cope, then it would be important to seek help from a relevant healthcare professional.
It is worth bearing in mind that if you have access to a specialist respiratory (ILD) nurse, he or she can be helpful to contact concerning health and wellbeing issues. Sometimes talking to someone, such as a friend or family member can be very
helpful.
It is helpful to tune into your emotions to help understand how you are feeling at the time and to determine the best ways
of responding. Where the emotions are positive, aim to hold on to them longer as this will help in distracting from any
negative emotions. For example, it could be an event that made you happy, by thinking and reflecting on it, helps to enjoy
such moments longer. Living with P.F. can be an emotional rollercoaster, so it is important to have good relationships to
benefit your mental health and well-being. Aim to connect with other people, even for short periods as this helps to boost
your mood. By investing in kindness and attention in your relationships with others, this will tend to be reciprocated,
resulting in a win-win situation. It takes resilience to cope with the challenges of living with P.F, however, from a
psychological point of view it is helpful to think realistically and optimistically and to show empathy, listen actively and
learn from fellow P.F. patients/carers and to maintain a sense of humor as much as possible.

Air quality is important to anyone living with a respiratory condition such as pulmonary fibrosis. The reasons why are
outlined below.
• Exposure to air pollution can reduce a year off the average European’s life.
• Air pollution causes about 500,000 premature deaths per year.
• Air pollution can be as dangerous as passive smoking.
• Short term high air pollution levels can worsen symptoms such as coughing, wheezing and shortness of breath.
• It can result in an increase in the number of hospital visits.
• Increased risk of dying from high levels of air pollution.
To reduce risks from air pollution, check the local air quality or sign up to a pollution alert service, there are also apps
available to download to your smart phone. Avoid exercising outdoors when the air pollution levels are high, arrange to
see your GP should your respiratory symptoms persist or worsen.
Indoor air pollution can cause difficulties for pulmonary fibrosis patients (especially with severe P.F,) as they tend to spend
more time indoors, so it is necessary to reduce this risk as much as possible. The main sources of such pollution are outlined
below and ways by which to reduce them.
• Smoking – Do not smoke and avoid passive smoking
• Irritants – Avoid using any possible irritants indoors such as oil-based paints/turpentine, use water based products
instead.Moulds – Ventilate well, (air your home 5 to 10 minutes several times daily,) remove mould stains and use
dehumidifiers.
• Chemicals in the home – Take care in the use of detergents, air fresheners, cleaning agents, perfumes and
deodorants, as they release chemicals into the air. Always ensure to ventilate after their use.
• Masks – Where appropriate wear a mask (use a type of mask suited to the situation) to afford you some
protection against air pollution.

For conditions like Idiopathic Pulmonary Fibrosis (IPF) the only curative treatment existing today is a lung transplant. All other treatments are only aimed at slowing down the progression of the disease.
To qualify for a lung transplant there are a lot of different things to consider. Both in respect to medical and biological issues, but also other things like donor availability. In an ideal world everybody in need of a lung transplant should receive one, but unfortunately this is not the case. One of the main reasons for this is the lack of organ donors, therefore all countries have a waiting list for lung transplant recipients.
Patients in Northern Ireland who are put forward for lung transplantation attend Freemans Hospital, Newcastle, in England for the procedure, a team from Newcastle initially meet with the patients in Northern Ireland.
As there is an international basic principle stating that each country should cover the need for organs for its own population,
and the fact is that there is a common shortage of donors, many countries have formed alliances for collaboration in transplantations. Two of these alliances are Eurotransplant, covering eight European countries; Austria, Belgium,
Netherlands, Luxemburg, (BE-NE-LUX), Germany, Hungary, Croatia and Slovenia and Scandiatransplant covering Norway, Sweden, Denmark, Finland, Iceland and Estonia. Other major European countries like France, Spain, UK and others are fending for themselves, covering their respective populations.
You can either receive a single-side (SLT) or a double-sided lung transplant (DLT), many European countries today offer a single side lung transplant, but some also provide a double-sided lung transplant. The SLT has often fewer short term side effects, but the DLT has a better overall survival and long-term outcome.
The best way of increasing the availability of donor organs is by promoting organ donation to people around you, increasing the number of organ donors will shorten the waiting lists and be beneficial for all. In some countries like the UK there are age restrictions (65 years of age), for lung transplants, which are based somewhat
on biological factors. It would be important to check with the country concerned who would be performing the transplant, to find out what criteria is applied.