Idiopathic pulmonary fibrosis (IPF)
What is IPF? | What causes IPF? | Symptoms of IPF | Diagnosis of IPF | Treatment for IPF | Future Research | Personal stories: Faces of IPF | Additional Resources for IPF |
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What is idiopathic pulmonary fibrosis (IPF)?
Idiopathic pulmonary fibrosis (IPF) is a disease of scarring in the lungs. The scars in the lungs cause the lungs to become stiff, making it hard to breathe. Over time, the scarring becomes so thick that the lungs cannot take in oxygen.
Idiopathic pulmonary fibrosis is a type of interstitial lung diseases. Interstitial lung disease is a term for a large group of diseases that cause inflammation (swelling) and scarring of the lungs (fibrosis). The swelling and scarring can make it difficult to breathe and to absorb oxygen. Interstitial lung disease is also called pulmonary fibrosis.
There are many different kinds of interstitial lung disease, or pulmonary fibrosis. It is very important to find out what is the cause of your pulmonary fibrosis, because different kinds of pulmonary fibrosis are treated differently. When a cause of pulmonary fibrosis can not be found, the disease is called idiopathic pulmonary fibrosis.
The cause of idiopathic 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.
What causes idiopathic pulmonary fibrosis (IPF)?
Although doctors and researchers do not understand the true cause of IPF, they know that the disease is linked to a number of risk factors including:
- genetic factors may play a role in causing IPF. If more than one member of your family has IPF, the disease is called familial pulmonary fibrosis.
- smoking tobacco, especially if you have IPF in your genes.
- Acid reflux, also known as gastro–esophageal reflux disease (or GERD) that occurs when acid from your stomach backs up into your throat.
- viral or bacterial lung infection – researchers have found certain viruses in the lungs of people with IPF. These viruses include Epstein-Barr and hepatitis C.
What are the symptoms of idiopathic pulmonary fibrosis (IPF)?
The two main symptoms are:
- 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 any activity.
- dry hacking cough, usually lasting longer than 8 weeks
Other symptoms may (or may not) include:
- chest pain or tightness
- unexplained weight loss
- loss of appetite
- tiredness and loss of energy
- change of finger shape called "clubbing"
- cyanosis (blue lips, nail beds)
How is idiopathic pulmonary fibrosis (IPF) diagnosed?
Idiopathic pulmonary fibrosis (IPF) can be hard to diagnose. Many lung diseases can cause breathlessness and cough. You may have to see more than one specialists, including a respirologist, to help with the diagnosis. Your doctor will be determining if you have pulmonary fibrosis and trying to figure out if there is any cause for the pulmonary fibrosis or if it is idiopathic (IPF).
- Your doctors may ask you questions about your home and work environments, your smoking history and your medical history. This will help them learn if there were any environmental, occupational, genetic, or other factors that could be linked to the development of pulmonary fibrosis.
- Your doctor may do a physical examination, by examining your skin, fingernails and joints and listening to your chest with a stethoscope.
You may also need to do some tests. Although no single test can diagnose IPF, your doctor may recommend a number of different tests including:
- spirometry test or Pulmonary Function Test (PFT) are simple breathing tests to measure how much air you can blow in and out of your lungs.
- chest X-ray
- blood tests to see if you have an infection, or how much oxygen is in your blood.
- six-minute walk test to measure the amount of oxygen in your blood at rest and with physical activity.
- high resolution computerized tomography (or CT scan) to see if there is scarring in your lungs.
- bronchoscopy and bronchoalveolar lavage – a test that involves inserting a small flexible tube, called bronchoscope, into your windpipe to examining the breathing tubes from inside. The lavage is done by njecting a small amount of fluid into lungs, that is then withdrawn and analyzed.
- surgical lung biopsy is a test that involves a small incision in the side of the chest, which allows removal and examination of lung tissue. This test is performed by a surgeon under general anesthetic.
Treatment for Idiopathic Pulmonary Fibrosis
Unfortunately, there is no cure and no effective treatment for pulmonary fibrosis. Currently, there are no medical procedures or medicines that can remove the scarring from the lungs. Treatments are used to lessen the symptoms of cough and breathlessness.
If there is something in your environment that is a known cause of pulmonary fibrosis – removing it is your first priority.
Some medicines may lessen the symptoms of IPF, such as cough and shortness of breath. If you have other diseases such as gastro-esophageal reflux (GERD), your doctor may prescribe medications for them.
Other treatments include supplemental oxygen, pulmonary rehabilitation, lung transplant and/or referral to a clinical trial.
It is very important to work with your doctor to find out what the best treatment is for you.
There are things you can do to help you manage your IPF:
Work with your health-care team to manage and understand your IPF
It is important to work with your family doctor or respirologist (lung specialist) to find the best treatment for you. Not all medications will work for everyone. Your doctor knows your medical history and will be able to determine if medication is an option for you.
Here are some things to talk to your health-care team about:
- Prepare a written list of the questions you want to ask. Put the most important questions at the top of your list.
- Take notes as the doctor is answering your questions. If you don't understand the doctor's answers, ask for more details.
- Show the doctor a written list of all your symptoms. Be honest.
- Bring along all your medications (including herbal remedies).
- If you're not sure you're taking your medications correctly, ask your doctor to review it with you.
- Bring a friend or relative to your appointment. He/she can help you remember details and also take notes for you.
- Ask your doctor about programs in your community that help people with IPF and other lung diseases. Ask about joining a pulmonary rehabilitation program.
Your health-care team can help you learn more about IPF and how to cope with it. People on your IPF health-care team may include:
- doctor(s), you may have a family doctor and a respirologist (lung specialist)
- a nurse
- a certified respiratory educator, a health professional with special training in lung disease
- a pharmacist
- a respiratory therapist, nurse, or cardiopulmonary physiotherapist who leads your pulmonary rehabilitation program
- a certified respiratory educator from the Lung Association's free helpline (call 1-866-717-2673)
Make changes to your lifestyle to stay healthy, and fight germs to prevent infections
- Quitting smoking is the number one thing you can do to help slow down the progression of any lung disease.
- Get a flu shot each year.
- Ask your doctor about getting a pneumonia shot.
- Keep away from people with chest infections and colds.
- Wash your hands properly.
- Stay as fit as you can.
The scarring in the lungs can prevent oxygen from being absorbed into your body
Low oxygen levels can make you more short of breath and tired. If your blood oxygen levels are too low, your doctor may prescribe supplemental oxygen (home oxygen). This helps to reduce breathlessness, helping you be more active. Some people with IPF may need oxygen therapy all the time, while others may only need it during sleep and exercise.
If your doctor has prescribed supplemental oxygen, it is important to use it as prescribed. Many patients fear that they will become “addicted” to supplemental oxygen. Supplemental oxygen is not addictive and your body needs a proper amount to maintain normal body functions.
People who take supplemental oxygen must continue taking their other medications.
Not everyone who has IPF needs to be on oxygen. Generally, oxygen therapy can help only people with very low blood-oxygen levels (hypoxemia). To find out if you need supplemental oxygen, speak to your doctor or certified respiratory educator.
Take IPF medications to prevent additional scaring and lessen symptoms
There are no medications that cure IPF or decrease the amount of scarring in the lungs. Some medications help slow the progression of pulmonary fibrosis. Most therapies for IPF require further study.
It is very difficult to find medications that will work for everyone with IPF. Many of the medications for IPF have side effects, so it is important to talk to your doctor about the possible risks. If there is no improvement with medication, you need to work with your doctor about other options including lung transplant, or ask about clinical research trials.
Please note that according to the most recent Guidelines for Diagnosis and Management of IPF, the majority of patients with IPF should NOT be treated with the medications listed below, either alone or in combination. Some of these treatments may be reasonable for a minority of patients with IPF.
As with any medicine for any condition, you should talk to your doctor about different treatment options and ask about medication side effects.
Corticosteroid pills (for example, Prednisone ©) can reduce the swelling in your lungs by suppressing your immune system. They are only successful in a small number of patients and can have various side effects.
Some side effects of corticosteroids include: thinning and bruising of the skin, bloating, weight gain, emotional changes, sugar intolerance that can worsen or cause diabetes, problems with blood pressure control, and peptic ulcers. Since corticosteroids suppress your immune system, you will be more susceptible to infections.
Your doctor may sometimes give you corticosteroid if you are having an acute exacerbation, or a flare-up of your IPF symptoms.
Several research studies of pirfenidone suggest that this drug has a modest benefit in slowing the progression of IPF. It has been approved by Health Canada for the treatment of mild to moderate IPF.
Azathioprine is a medication that is used to treat autoimmune disease (such as rheumatoid arthritis) or help prevent organ rejection after transplantation by suppressing the immune system.
Cyclophosphamide is an anti-cancer drug sometimes used to treat pulmonary fibrosis. It may also be given alone, either as a pill, or through an IV.
N-Acetylcysteine (oral or aerosolized)
N-acetcysteine (NAC) an antioxidant. There is a large clinical trial looking at NAC as treatment for IPF in the US. N-acetylcysteine is available over the counter at many pharmacies and nutritional stores.
Join a pulmonary rehabilitation program
Pulmonary rehabilitation is an exercise and educational program designed just for patients with lung disease. Pulmonary rehabilitation can help in teaching patients with IPF how to breathe more efficiently and to perform their activities of daily living with less breathlessness.
Because most pulmonary rehabilitation programs are designed for people with COPD (emphysema and chronic bronchitis), some programs would not accept patients with IPF, although majority of the programs in Canada accept and welcome IPF patients.
Read more about pulmonary rehabilitation and find a program near you.
Talk to your doctor about the option of a lung transplant
Lung transplant is not right for everyone, but it may be a treatment option for some people.
If a person qualifies for a lung transplant, they are put on a wait list for organ donation. The wait can be long and the surgery can be risky. People who get transplants must take several anti-rejection medicines (immune-suppressants) for the rest of their lives.
Lung transplants may not be right for everyone with IPF. It is important to work with your doctor to see if lung transplants may be an option for you.
Future Research and Clinical Trials
Research for effective drugs for IPF is on-going. Although there are no current effective medical therapies, researchers are looking for new treatments for idiopathic pulmonary fibrosis (IPF) by conducting various clinical trials in Canada and around the world. While the long term goal of IPF research is to prevent and cure the disease, current studies also attempt to slow disease progression, and to increase the life expectancy of patients with IPF.
For information on how to set up an IPF education and support group, visit the Canadian Pulmonary Fibrosis Foundation website where you can download a toolkit.
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