It is a term that encompasses many different conditions that cause the accumulation of scar tissue in the lungs.
This buildup of scar tissue, which makes your lungs stiff, is called fibrosis.
Pulmonary fibrosis is a type of interstitial lung disease (ILD). Interstitial means that the disease affects the interstitium, the lace-like network of tissue that supports the air sacs in the lungs.
Some types of pulmonary fibrosis have an identifiable cause. But for many styles, no definite reason can be found.
In PID, there may be scarring in the lungs or inflammation in the lungs. Some PIDs mainly cause scarring; some mostly cause inflammation. But there is often a combination of these processes. Depending on which of these processes is dominant, can you determine what type of treatment you can have?
Treatment and outlook for the different types of pulmonary fibrosis vary considerably, so if you are unsure about your diagnosis, check with your doctor or nurse for the exact name of your lung condition.
Types of pulmonary fibrosis
All types of pulmonary fibrosis are rare. Although we don’t always know what causes pulmonary fibrosis, we do know that it is not a form of cancer or cystic fibrosis, and it is not contagious.
This information covers the most frequently seen:
- Idiopathic pulmonary fibrosis or IPF.
- Hypersensitivity pneumonitis, formerly called extrinsic allergic alveolitis.
- Pneumoconiosis knew as occupational interstitial lung disease.
- Pulmonary fibrosis is associated with connective tissue and autoimmune diseases.
- Drug-induced pulmonary fibrosis.
Sarcoidosis is a relatively common type of disease that generally affects the lungs. It can sometimes cause pulmonary fibrosis, but we don’t know why. Cystic fibrosis is not a type of ILD or pulmonary fibrosis.
How does pulmonary fibrosis affect your breathing?
Pulmonary fibrosis scars the lungs and reduces the efficiency of breathing.
Scarring makes your lungs stiffer and less elastic, making them less able to move and take in oxygen from the air you breathe.
Each time you inhale, you breathe through your nose or mouth, down your throat, and into your windpipe. Your windpipe divides into two smaller air tubes called bronchi, which go to your lungs. Air passes through the bronchi, which divide again and again into thousands of smaller airways called bronchioles.
Bronchioles have many tiny air sacs called alveoli. Inside the air sacs, oxygen moves through paper-thin walls to capillaries (tiny blood vessels) and into your blood. The air sacs also collect waste gas and carbon dioxide from your blood, ready for you to breathe out.
If you have pulmonary fibrosis, scarring affects the lung air sacs. The air sacs are supported by the interstitium, a web of tissue, a bit like lace. Scarring fills the spaces between and around the air sacs and limits the amount of oxygen that enters the blood.
As healing increases, your lungs are less able to expand to allow you to take deep breaths, and the oxygen level in your blood may begin to drop. Breathing can seem like more challenging work, and you may feel out of breath from daily activities like walking.
In a small number of cases of pulmonary fibrosis, it is possible to identify a specific cause.
Some causes are:
- Being exposed to certain types of dust, including wood, metal, or asbestos dust.
- Being exposed to allergens, such as bird feathers or mold.
- A side effect of a medicine.
Some types of pulmonary fibrosis occur when you have another condition, such as rheumatoid arthritis or scleroderma.
In very few cases, two or more family members can develop pulmonary fibrosis. But current research suggests that the genetics of familial interstitial lung disease are complex, and there is no clear inherited predisposition to develop pulmonary fibrosis.
In most types of pulmonary fibrosis, no specific cause can be found. One of the most common fibrosis forms is idiopathic pulmonary fibrosis (IPF). The word “idiopathic” means that there is no known cause.
Symptoms of pulmonary fibrosis
Different types of pulmonary fibrosis have similar symptoms, which is one of the reasons you need to get tested and speak with a specialist to find out exactly which type you have.
Many people notice the first symptom of shortness of breath when straining, such as climbing a hill or climbing stairs. But you may feel constantly short of breath, not just when moving.
Various forms of pulmonary fibrosis usually occur after the age of 60, so you might think that you are running out of breath because you are not as young as you were before. This breathlessness will get worse over time if it is not treated.
Your shortness of breath can also be affected by other long-term conditions, such as chronic obstructive pulmonary disease (COPD), heart disease, and overweight.
A cough that doesn’t go away and you feel exhausted are two other symptoms of pulmonary fibrosis. Some people with pulmonary fibrosis may also have a fever, lose weight, or experience muscle and joint pain.
You may not think that a lung condition could affect the fingers and toes, but it is often a hallmark sign of pulmonary fibrosis. You may notice that:
- Your nails feel too soft or like they are coming loose.
- The shape of your nails changes.
- The tips of the fingers or toes bulge out.
This condition can also occur with other lung conditions due to heart or liver disease.
If you have symptoms that could be due to pulmonary fibrosis, your GP will refer you to a consultant who specializes in lung diseases.
It is essential to identify the cause of your symptoms, if possible, and what type of pulmonary fibrosis you have, as it will influence your treatment.
The doctor will ask you questions about your medical and work history, listen to your chest, and order some blood tests to rule out possible known causes.
Additional tests used to diagnose pulmonary fibrosis are described below; you may not need to have all of them. Your advisor will evaluate you when they see you.
X-rays and scans
You will have a chest X-ray. If your doctor thinks you might have pulmonary fibrosis, you will be followed by a computed tomography (CT) scan, which uses x-rays to get a detailed picture of your lungs. The scan results can be evident for some types of pulmonary fibrosis.
On a CT scan, idiopathic pulmonary fibrosis (IPF) often appears as a distinctive lung pattern. You may hear your doctor call this lung “honeycomb,” as the picture shows many empty pockets or bubbles where more solid lung tissue appears typically.
These are also known as lung function tests. There are different types, but most involve breathing into a machine through a mouthpiece or tube. The equipment measures how well you can live in or out.
The results can tell your doctor how much your lungs and breathing capacity have been affected. If it is found that you have pulmonary fibrosis, these tests will be used to monitor your condition.
You can also take a walking assessment. This usually involves walking for a few minutes to see what happens when you exercise, including changes in the level of oxygen in your blood. This is measured with pulse oximetry, using a sensor placed on the finger or the earlobe.
Looking inside your lungs
Your doctor may need to look inside the lungs and possibly remove some cells or tissue for testing to confirm the diagnosis. The samples are examined under a microscope by a pathologist, a skilled doctor, in laboratory tests.
Different procedures can be used to obtain some lung tissue:
Flexible bronchoscopy involves inserting a narrow tube through the nose or mouth into the lungs. A local anesthetic will be applied to your nose and throat, and often a sedative injection will be given into your vein for your comfort.
The tube has a camera on the end so the doctor can see inside your lungs. The doctor may flush some water through the line to collect and collect the cells for testing.
Your doctor can also remove tissue samples at the same time. These are called biopsies and are painless. This is usually an outpatient or day procedure.
Video-assisted thoracoscopy or TAPV involves surgery under general anesthesia to obtain a more significant portion of lung tissue. A surgeon makes keyhole-shaped incisions in the chest for a video-assisted surgical telescope to enter and remove tissue samples from the lungs.
It has higher risks than a bronchoscopy. It is done only if your specialist believes that more tissue is needed to make a diagnosis and treatment plan. Your doctor will discuss the risks with you. You will stay in the hospital for a few days for this test.
The identification and diagnosis of pulmonary fibrosis is a joint effort of your team of specialists, including several physicians who are experts in lung conditions, surgery, X-rays and scans, and laboratory tests.
The nurse practitioner is an integral part of this team and a good source of information and support for you. The team will gather the findings from all of your tests to give you a diagnosis.
Treatment for pulmonary fibrosis
There are treatments for most different types of pulmonary fibrosis, and your treatment will depend on the specific form of pulmonary fibrosis you have.
You will also be offered treatment for specific symptoms, such as a cough, and specific therapies for the disease.
Your doctor may treat problems that make it worse for a cough, such as heartburn or a stuffy nose.
Tell your doctor if you have GERD symptoms, such as heartburn or a sour taste in the back of your mouth. There is some evidence that this can worsen inflammation and fibrosis and make coughing worse.
Feeling out of breath can have a severe effect on your everyday life. It can also be a terrifying experience. Pulmonary rehabilitation is necessary to help you cope with shortness of breath. If you smoke, stopping is essential.
Pulmonary rehabilitation, sometimes called RP, includes:
- A physical exercise program designed for people with lung diseases and tailored for you.
- Tips and information on taking care of your body and lungs, how to manage your condition, and coping with the feeling of shortness of breath.
RP is led by a qualified respiratory professional, usually a physical therapist. Nurses, occupational therapists, psychologists, and dietitians can also participate.
RP is designed to support you, reassure you, and help you improve your condition. You may find that you can walk farther, feel less breathless, and generally feel more positive.
If your condition worsens, the oxygen level in your blood may drop, making you feel short of breath. Your doctor will recommend you for an oxygen therapy evaluation if this happens.
You may be given a portable oxygen cylinder to use when you walk around or have an oxygen concentrator installed in your home. The concentrator draws normal air from the room and enriches it with oxygen before inhaling it. The machine is connected to pipes throughout the house, so it is not confined to one room.
Some people with pulmonary fibrosis need supplemental oxygen when they are moving and sometimes later when they are resting.
Your oxygen prescription will be tailored to meet your individual needs. And you will be informed on how to adjust the oxygen flow according to your physical activity.
Having a lung transplant may be an option for very few people if pulmonary fibrosis progresses and is not controlled by treatment.
Whether you can be considered for a lung transplant depends on factors that influence the likelihood of a successful outcome, such as your general health, other medical conditions, and your body mass index.
There is no age limit, but it is unusual to accept people over 65. Unfortunately, there are not enough donor lungs available to meet the demand.
If you want to explore this option and your doctor thinks it might be appropriate, you will be referred to a transplant unit.
Your doctor, nurse, or physical therapist should also talk to you about palliative care or symptom management or refer you to a palliative care specialist. This care is designed to improve the quality of your life and that of your loved ones.
It includes managing pain and other symptoms, which may be related, such as shortness of breath, fatigue, and anxiety.
Pulmonary fibrosis tends to be progressive, and you may become increasingly short of breath. If you have trouble breathing, your doctor may prescribe a sedative or morphine even when you are resting.
You may know them as pain relievers or sleeping pills, but these medications can also help with symptoms of shortness of breath as pulmonary fibrosis progresses. You may also need oxygen all the time.
Ways to help your breathing
If you have pulmonary fibrosis, you may tend to breathe very fast and shallow, like gasping.
You can use techniques and positions to help you control and slow down your breathing. You can also use them to avoid getting out of breath when you exert yourself and to help yourself recover when you are out of breath.
Talk to your respiratory physical therapist or nurse to help you figure out what is best for you. Try the different breathing techniques to find what helps you and practice those that help you.
Our suggestions are based on what physical therapists find, as little research has been done on people living with pulmonary fibrosis.
Use these positions to practice your breath control or to catch your breath.
Sitting upright in a firm chair: Sit upright in a strong chair. If your chair does not have arms, rest your arms on your thighs. Let your wrists and hands relax.
Support tilted backward or to the sides: keep your feet apart, about a foot or 30 cm from the wall. Relax your hands at your sides. If you prefer, rest your hands or thumbs on the waistband or belt loops or the shoulder strap of your bag.
Side-lying with pillows: Lie on your side with pillows under your head and shoulders. Make sure the top pillow supports your head and neck. Slightly bend your knees, hips, and upper leg.
Breathing control using your diaphragm
Controlling your breath means breathing gently, using minimal effort. It will help you when you are short of breath or feel anxious. This type of breathing is commonly used in yoga.
Use this breath control in combination with any of the techniques below.
You use your diaphragm, a large umbrella of muscle below your lungs, at the bottom of your rib cage. It contracts when you breathe, pulling the lungs down, stretching, and expanding.
Getting used to this helps to practice when you are sitting, relaxed, and without difficulty breathing. You can then use it when you are out of breath or reduce your shortness of breath when straining.
Get into a comfortable position, with your arms supported. Let your shoulders and body be relaxed and loose.
Place one hand on your chest and the other on your abdomen (tummy)
Close your eyes to help you relax and focus on your breathing
Inhale slowly through your nose, with your mouth closed. If you are relaxed, the air will reach a low level in your lungs. You will feel your abdomen rise; it will move towards your hand. The hand on your chest won’t move much at all
Breathe through your mouth, like a sigh or with your lips pursed. Your abdomen will drop gently. Imagine all the tension in your body when you let the air out
Try to use as little effort as possible and make your breathing slow, relaxed, and smooth. With each exhale, try to feel more comfortable and calm. Try to breathe slower.