What is Pulmonary Fibrosis?
The word “pulmonary” means lung and the word “fibrosis” means scar tissue— similar to scars that you may have on your skin from an old injury or surgery. So, in its simplest sense, pulmonary fibrosis (PF) means scarring in the lungs. Over time, the scar tissue can destroy the normal lung and make it hard for oxygen to get into your blood. Low oxygen levels (and the stiff scar tissue itself) can cause you to feel short of breath, particularly when walking and exercising. Pulmonary fibrosis isn’t just one disease. It is a family of more than 200 different lung diseases that all look very much alike. The PF family of lung diseases falls into an even larger group of diseases called the interstitial lung diseases (also known as ILD), which includes all of the diseases that have inflammation and/or scarring in the lung. Some interstitial lung diseases don’t include scar tissue. When an interstitial lung disease does include scar tissue in the lung, we call it pulmonary fibrosis.
IPF is a form of interstitial lung disease, primarily involving the interstitium (the tissue and space around the air sacs of the lungs), and not directly affecting the airways or blood vessels. There are many other kinds of interstitial lung disease that can also cause inflammation and/or fibrosis, and these are treated differently. It is important to work with your doctor to determine if you have IPF or another form of interstitial lung disease.
The cause of pulmonary fibrosis is not completely understood. No one knows what causes idiopathic pulmonary fibrosis or why some people get it. “Idiopathic” means of unknown cause. Common risk factors for IPF include:
- Genetics (family history): Up to 20% of people with IPF have another family member with an interstitial lung disease. If more than one member of your family has IPF, the disease is called familial pulmonary fibrosis.
- Cigarette smoking: Approximately 75% of people with IPF are current or previous cigarette smokers.
- Acid reflux (Gastro-oesophageal reflux disease (GORD)): Approximately 75% of people with IPF have symptoms of acid reflux or heartburn.
- Male sex: Approximately 75% of patients with IPF are male.
- Age: Almost all patients with IPF are over the age of 50 years
Breathlessness (also known as shortness of breath or dyspnea): Usually the breathlessness of IPF first appears during exercise. Breathlessness can affect day-to-day activities such as showering, climbing stairs, getting dressed and eating. As scarring in the lungs gets worse, breathlessness may prevent all activities.
Chronic cough: About 85% of people with IPF have a chronic cough that has lasts longer than 8 weeks. This is often a dry cough, but some people may also cough up sputum or phlegm.
Other symptoms may include:
- chest pain or tightness
- unexplained weight loss
- loss of appetite
- tiredness and loss of energy
- Getting out of breath
- Cough (that does not go away)
- Doctor may hear crackling in your lungs (sounds like velcro pulling apart).
- Finger clubbing of the nails.
Environmental Causes of PF
Typically called hypersensitivity pneumonitis (HP) or chronic hypersensitivity pneumonitis. HP occurs when the lungs react with inflammation and scarring after breathing in mold spores, bacteria, animal proteins (especially from indoor or caged birds), or other known triggers. No one is certain why some people are so susceptible to developing HP and others are not.
Pulmonary Fibrosis and Autoimmune Diseases
AUTOIMMUNE DISEASES are also called connective tissue diseases, collagen vascular diseases, or rheumatologic diseases. “Auto” means self and “immune” refers to your immune system. So if you have an autoimmune disease affecting your lungs, it means that your body’s immune system is attacking your lungs. Examples of autoimmune diseases that can cause PF include
- Rheumatoid arthritis.
- Scleroderma (also called systemic sclerosis).
- Sjögren’s syndrome.
- Polymyositis, dermatomyositis, and antisynthetase syndrome.
WHAT IS IDIOPATHIC PULMONARY FIBROSIS?
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.
What is Mental Wellbeing?
Mental wellbeing is a term used to describe how you are feeling and how you cope with daily life. This includes how you feel about living with your lung condition.
If you have good mental wellbeing you are more likely to be taking good care of yourself and managing your treatment well. You will feel positive about life and want to be a part of things that are happening around you.
If you are experiencing poor mental wellbeing you may find that you are less interested in doing things you once enjoyed. You may feel more anxious and tense and find it harder to cope with daily life and the stress of your condition.
Living with a lung condition
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.
How can I look after my mental wellbeing?
Looking after your mental wellbeing is a personal process and it is important to find something that works for you. There are lots of different approaches:
Living an active life when you have a lung condition can help to manage your symptoms and keep you feeling well. The more active you are, the easier you will find daily activities, despite your breathing difficulties. It is important to find an activity that matches your level of breathlessness. You could find something to build into your daily routine, such as gardening, walking up and down the stairs or getting off the bus one stop early and walking home. Or you could try something more structured like keep-fit classes, running or strength exercises.
Remember that experiencing some breathlessness during activities is normal and not a sign of disease flare-up. You could also look to join a pulmonary rehabilitation course. This is a highly effective structured programme of exercise and information classes that are specific for people with lung conditions to help you stay active.
Mindfulness means focussing on the present moment. It teaches that an emotion or feeling is our normal reaction to something, but that you can respond to this feeling in different ways. It helps you understand that some thoughts and feelings might be positive and some negative. But that neither of these reactions changes the reality.
Research has shown the benefits of mindfulness for people with lung conditions, particularly to help relieve the stress that could be caused by breathlessness. Experiencing some breathlessness during activities is normal in people with lung conditions and accepting this can help you feel better about your condition and about life in general.
You can take steps to help yourself, such as learning what affects your mood – what improves it and what makes you feel worse? This may help you to prevent feelings of being unable to cope. You might also want to build things into your life that you find enjoyable, such as learning something new, a phone call with a friend or taking more time for yourself to relax. This might be particularly helpful if you feel that you cannot do the things that you used to. Instead, you could find something enjoyable that you can do now.
It is important to regularly ‘check in’ with yourself. Notice how you are feeling and whether you have felt better or worse recently. If you begin to feel like you are not coping well, then discuss this with your healthcare professional early on before your mental or physical symptoms become worse.
“It’s okay not being well. It’s normal to feel defeated or like you can’t stand to do anything else. It’s important to remember that this is not a problem and you can find support to help you manage this, just as you would do if you experience bad physical symptoms. The other thing that helped me a lot is to remind myself that I am not less for having the condition. I am not less of a person and I don’t deserve less than others just because of my condition. Remembering this really helped me manage my mental wellbeing in times when I felt low.” David
What mental health conditions might I experience?
Approximately one in four of us will experience a mental health condition at some point in our lives. Here we take a look at three conditions that might be more common for people with lung conditions.
Many experiences linked to your lung condition could lead to anxiety. These could include:
- being hospitalised if you have a chest infection or your symptoms flare up
- being frequently or constantly breathless
- losing your job or missing interactions with friends and family, or
- feeling that your symptoms are out of control.
If this feeling of worry continues for a long time or overwhelms you, these are symptoms of anxiety which you can get help for.
Things to look out for:
- Racing thoughts
- Difficulty concentrating
- Feelings of panic, dread or doom
- Problems with sleep
- Feeling like you want to escape
- Heavy and fast breathing linked to your mood
- Sweating or hot flushes
- Palpitations and trembling
Living with a lung condition can feel like hard work. You may feel angry or disheartened at times, particularly if your condition stops you taking part in your usual activities. If this feeling lasts for weeks and affects your daily life or is accompanied by strong feeling of joylessness or feeling really down, this could be a sign of depression. Depression is not the same as just feeling low for a few days – it is a serious mental health condition and one you can get help for.
Things to look out for:
- Feeling helpless or hopeless
- Losing interest in daily activities
- Sleep changes-either more or less
- Appetite changes-eating more or less
- Lack of energy and concentration
- Anger, irritability towards yourself or others
- Feelings of low self-esteem and guilt
- Thoughts about death or suicide
Post-traumatic stress disorder (PTSD)
As a person living with a lung condition, you may experience traumatic events that have a lasting effect on your mental health. One example is if you require time on an intensive care unit in hospital or if you require mechanical assistance to breathe. This can be a traumatic experience and some people are left struggling to cope. It can lead to a condition called post-traumatic stress disorder. This condition can cause severe disruption to your daily life as the stress you felt at the time of the event continues to affect you. This is a serious mental health condition and one you can get help for. If you notice the signs or symptoms of any mental health conditions, it is important to seek help from your healthcare provider as soon as possible.
Things to look out for:
- Flashbacks to the trauma you experienced so you feel that it is happening to you again
- Severe stress at reminders of the trauma
- Sweating, nausea or trembling when you remember your experience
Treatments for mental health conditions
Everyone is different and will have different needs that your healthcare provider will discuss with you. These are some of the treatments for mental health conditions for people with lung disease.
- Multidisciplinary pulmonary rehabilitation. This has been found to be a very effective option for improving your mental and physical health if you have a lung condition.
- Talking therapies with a counsellor or psychotherapist. This could include cognitive behavioural therapy (CBT), exposure therapies – which help overcome specific fears as well as help with depression and PTSD – or trauma therapy.
- Medication such as antidepressants or anxiolytics. Doctors may suggest this as a treatment option and will work with you to find the right dosage to limit side-effects.
You may be offered a combination of these treatments and your healthcare team will work to find the best solution for you.
Where can I find support?
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.
Caring for someone can be stressful and this could impact your mental wellbeing. This is very normal, and it is important for you to look after your own mental health. If you notice the signs or symptoms of a mental health condition in yourself, it is important to seek help as soon as possible. Your health matters and you will be able to provide better care and support if your own health is well looked after.
If you are caring for someone with a lung condition and you want to be able to support their mental wellbeing, it can be hard to know how best to do this. Everyone’s experience of mental wellbeing or mental health conditions will be unique and personal to them. Having open discussions about what can trigger negative moods, what the person finds helpful and what support they would like from you can be a good place to start.
If you notice a change in their mood and you are concerned, ask how the person is feeling, if they feel they are coping and what kind of support they may need. You may not be able to help them directly, but you can point them in the right direction for professional help and let them know you are there for them. Often listening is more important than giving advice. You could also offer to accompany them to appointments if they are worried about discussing their condition with a healthcare professional.