What Is Idiopathic Pulmonary Fibrosis?
Pulmonary (PULL-mun-ary) fibrosis is a condition in
which tissue deep in your lungs becomes thick and stiff, or scarred, over time.
The development of the scarred tissue is called fibrosis.
As the lung tissue becomes thicker, your lungs lose
their ability to move oxygen into your bloodstream, and your brain and other
organs dont receive the oxygen they need. (See the section on
How the Lungs Work for more
information.)
In some cases, doctors can find out what is causing
the fibrosis. But in most cases, they cant find a cause. They call these
cases idiopathic pulmonary fibrosis (IPF).
IPF is a serious condition. About 200,000 Americans
have it. About 50,000 new cases are diagnosed each year. IPF affects mostly
people 50 to 75 years of age.
IPF varies from person to person. In some people,
the lung tissue quickly becomes thick and stiff. In others, the process is much
slower, and in some people, the condition stays the same for years.
There is no cure for IPF yet. Many people live only
about 3 to 5 years after diagnosis. The most common cause of death related to
IPF is respiratory failure. Other causes include:
Some people may be more likely to develop IPF
because of their genes (the basic units of heredity). If more than one member
of your family has IPF, the condition is called familial idiopathic pulmonary
fibrosis.
Today, scientists are beginning to understand more
about what causes IPF, and they can diagnose it more quickly. They also are
studying several medicines that may slow the progress of the disease. These
efforts should lead to longer and better quality lives for people with IPF.
How the Lungs Work
The air that you breathe in through your nose or
mouth travels down through your windpipe (trachea) into two tubes in your lungs
called the bronchial tubes, or airways.
The airways are shaped like an upside-down tree with
many branches. The trachea is the trunk. It splits into two bronchial tubes, or
bronchi. Thinner tubes called bronchioles branch out from the bronchi.
The bronchioles end in tiny air sacs called alveoli.
These air sacs have very thin walls, and small blood vessels called capillaries
run through them. There are about 300 million alveoli in a normal lung.
Scientists say that if the air sacs were opened up and placed next to one
another, they would cover a tennis court.
When the air that you've just breathed in reaches
these air sacs, the oxygen in the air passes through the air sac walls into the
blood in the capillaries. The blood then flows into larger veins, which carry
it to the heart. Your heart then pumps the oxygen-rich blood to all your
bodys organs. These organs cant function without an ongoing supply
of oxygen.
In idiopathic pulmonary fibrosis, scarring begins in
the air sac walls and the spaces around them. It makes the walls of the air
sacs thicker. As a result, oxygen cant move from the air sacs into the
bloodstream.
Other Names for Idiopathic Pulmonary Fibrosis
- Idiopathic diffuse interstitial pulmonary
fibrosis
- Pulmonary fibrosis of unknown cause
- Pulmonary fibrosis
- Cryptogenic fibrosing alveolitis
- Usual interstitial pneumonitis
- Diffuse fibrosing alveolitis
What Causes Idiopathic Pulmonary Fibrosis?
Doctors dont know what causes idiopathic
pulmonary fibrosis (IPF). They think that something inside or outside of the
lungs attacks the lungs again and again over time. These attacks injure the
lungs and cause scarring in the tissue inside and between the air sacs. This
makes it harder for oxygen to pass through the air sac walls into the
bloodstream.
Doctors think the following things may increase your
chances of developing IPF:
- Cigarette smoking
- Viral infections, including Epstein Barr virus
(which causes mononucleosis), influenza A virus, hepatitis C virus, HIV-a
virus, and herpes virus 6
- Exposure to environmental pollutants, including
inorganic dust (silica and hard metal dusts), organic dust (bacteria and animal
proteins), and gases and fumes
- Use of certain medicines, including:
- Nitrofurantoin and sulfasalazine, which are
antibiotics used mostly to treat urinary tract infections, ulcerative colitis,
and rheumatoid arthritis
- Medicines to keep your heartbeat regular (for
example, amiodarone or propranolol)
- Chemotherapy or cancer medicines, such as
methotrexate, cyclophosphamide, and bleomycin
Your genes may also play a role in the development
of IPF. Some families have at least two members with IPF.
Scientists have found that 9 out of 10 people with
IPF also have gastroesophageal reflux disease (GERD). As a result, some
scientists think that some people who have GERD may breathe in tiny drops of
acid from their stomachs on a regular basis. The acid may then injure the lungs
and lead to IPF. More research must be done to confirm this.
What Are the Signs and Symptoms of Idiopathic
Pulmonary Fibrosis?
The signs and symptoms of idiopathic pulmonary
fibrosis (IPF) develop over time. They may not even begin to appear until the
disease has done serious damage to your lungs. Once they do appear, they are
likely to become worse over time.
The most common signs and symptoms are:
- Shortness of breath. This is usually the main
symptom. At first, you may be short of breath only during exercise. Over time,
you are likely to feel breathless even at rest.
- A dry, hacking cough that doesnt get better
when you treat it with over-the-counter cough medicines. Over time, you may
have repeated bouts of coughing that you can't control.
Other signs and symptoms that you may develop over
time include:
- Rapid, shallow breathing
- Gradual, unintended weight loss
- Fatigue (tiredness) or malaise (a general feeling
of being unwell)
- Aching muscles and joints
- Enlargement of the ends of the fingers or toes,
which is called clubbing
IPF may lead to other medical conditions, including:
collapsed lung, lung infections, blood clots in the lungs, and lung cancer.
As your condition gets worse, you may develop other
potentially life-threatening conditions, including respiratory failure,
pulmonary
arterial hypertension, and
heart
failure.
How Is Idiopathic Pulmonary Fibrosis
Diagnosed?
Idiopathic pulmonary fibrosis (IPF) produces the
same kind of scarring and symptoms as some other lung diseases. This makes it
hard to diagnose. Seeking medical help, preferably from a pulmonologist (a
doctor who specializes in lung problems), as soon as you have symptoms is
important.
To diagnose IPF, your doctor will ask about your
medical history, perform a physical exam, and do several tests to rule out
other causes of your symptoms and learn how badly your lungs are damaged.
Medical History
A complete medical history is important. Your doctor
will ask about:
- Your age
- Your history of smoking
- Things in the air at your job or elsewhere that
could irritate your lungs
- Your hobbies
- Your history of legal and illegal drug use
- Other medical conditions that you have that can
trigger an immune system response
- Your family's medical history
- How long you've had symptoms
Diagnostic Tests
There is no single test for diagnosing IPF. Your
doctor will probably conduct several different tests, including the
following.
Chest X Ray
A chest x ray takes a picture of your heart and
lungs. It can show shadows that suggest scar tissue. Many people with IPF have
normal chest x rays at the time theyre diagnosed.
High-Resolution Computerized Tomography
High-resolution computerized tomography (HRCT) scan
is an x ray that provides sharper and more detailed images than a regular chest
x ray. It can show scar tissue and how much lung damage you have. This test can
help your doctor spot IPF at an early stage or rule it out. It also can help
your doctor decide how likely you are to respond to treatment.
Lung Function Tests
Your doctor will do a breathing test called
spirometry to find out how much lung damage you have. A spirometer is a device
that measures how much air you can blow out of your lungs after taking a deep
breath. It also measures how fast you can breathe the air out. If there is a
lot of scarring in your lungs, you will not be able to move a normal amount of
air out of them.
Pulse Oximetry
Your doctor attaches a small device to your finger
or earlobe to see how much oxygen is in your bloodstream.
Arterial Blood Gas Test
The doctor takes a small amount of blood from an
artery in your arm, usually in your wrist. Its then checked for oxygen
and carbon dioxide levels. This test is a more accurate way to check the level
of oxygen in your bloodstream. Your blood can also be tested to see whether you
have an infection that may be causing your symptoms.
Skin Test for Tuberculosis
Your doctor injects a small amount of a substance
that reacts to tuberculosis (TB) under the top layer of skin on one of your
arms. If you have a positive reaction, a small hard lump will develop at the
injection site 48 to 72 hours after the test. This test is done to rule out
TB.
Exercise Testing
Exercise testing is used to find out how well your
lungs move oxygen and carbon dioxide in and out of your bloodstream when
youre active. During this test, you walk or pedal on an exercise machine
for a few minutes. Electrodes attached to your chest and an
EKG
(electrocardiogram) machine show your heart rate, a blood pressure cuff
monitors your blood pressure, and a pulse oximeter attached to a finger or ear
lobe shows how much oxygen is in your blood.
Sometimes doctors place a catheter in an artery in
one of your arms to draw samples of your blood that will provide a more
accurate measure of the oxygen and carbon dioxide in it. They may also ask you
to breathe into a tube that separates the air you breathe out from room air and
provides a precise measure of the amount of oxygen youve taken in and
carbon dioxide you're breathing out.
Lung Biopsy
Looking at samples of tissue from several places in
your lungs under a microscope is the best way for your doctor to diagnose IPF.
A lung biopsy can help your doctor rule out other causes of your condition,
such as
sarcoidosis,
cancer, or infection. It can also show your doctor how far your condition has
advanced. Doctors use several different procedures to obtain samples of your
lung tissue:
- Bronchoscopy. Your doctor inserts a flexible,
rubber tube with a tiny light and camera on the end through your nose or mouth
into your lungs to look at your airways. He or she then inserts a forceps
through the tube to collect tissue to examine. This procedure is usually done
under local anesthesia on an outpatient basis.
- Bronchoalveolar lavage. During bronchoscopy, your
doctor may inject a small amount of salt water (saline) through the
bronchoscope into your lungs. This fluid washes the lungs and helps bring up
cells from the area around the air sacs for examination under a
microscope.
- Video-assisted thoracoscopy. This is the
procedure that doctors use most to obtain lung tissue. Your doctor inserts a
small, lighted tube with a camera (endoscope) into your chest through small
incisions between your ribs. The endoscope provides a video image of the lungs
and allows your doctor to collect samples of tissue. This procedure must be
done in the hospital, under general anesthesia.
- Thoracotomy. Your doctor removes a few small
pieces of lung tissue through an incision in the chest wall between your ribs.
Thoracotomy is done in the hospital under general anesthesia.
How Is Idiopathic Pulmonary Fibrosis Treated?
The goals of treating idiopathic pulmonary fibrosis
(IPF) are to prevent more lung scarring, relieve your symptoms, maintain your
ability to be active and get around, keep you healthy, and improve your quality
of life.
Treatment cant remove scarring that has
already happened. As a result, diagnosing and treating IPF as early as
possible, before a lot of scarring has taken place, is very important.
Treatment is usually based on your age, medical
history, any medical problems you may have, and how much the IPF has
advanced.
Treatments can include:
- Medicines to reduce inflammation (swelling) in
your lungs and/or prevent more scarring
- Oxygen therapy
- Pulmonary rehabilitation
- Lung transplantation
Medicines
The main treatment for IPF is medicine to reduce
inflammation. Many doctors also add a medicine to suppress your body's immune
system. These treatments can prevent further scarring and increase survival
time in some people, but they dont work for everyone.
Prednisone
The anti-inflammatory medicine that most doctors
prescribe is high-dose prednisone, a corticosteroid. You usually take
prednisone by mouth every day. Sometimes your doctor may give it to you through
a needle or tube inserted into a vein in your arm for several days. After that,
you usually take it by mouth.
Because prednisone can cause serious side effects,
your doctor may prescribe it for only 3 to 6 months at first. Then, if it works
for you, your doctor may reduce the dose over time and keep you on it for a
longer time.
Most people who take high-dose prednisone for a long
time can have side effects, including difficulty sleeping at night (insomnia),
weight gain, acne, and irritability. Using prednisone for a long time also can
lead to other conditions, including:
- High
blood pressure.
- Hyperglycemia (high blood sugar).
- Cataracts (a cloudy area in the lens in your
eyes).
- Glaucoma (a serious eye condition that can lead
to blindness).
- Anxiety or depression.
- Osteoporosis (thinning of the skin and
bones).
- Adrenal gland insufficiency (a condition in which
the adrenal glands dont produce enough of certain hormones). This
condition should be treated by an endocrinologista doctor who specializes
in the diagnosis and treatment of the adrenal glands.
Prednisone also can cause conditions such as
diabetes and glaucoma to get worse.
Other Medicines
Many doctors prescribe a second medicine with
prednisone.
- Azathioprine (Imuran®) is a
medicine that affects your immune system. Most patients take it by mouth every
day. Because it can cause serious side effects, your doctor may prescribe it
with prednisone for only 3 to 6 months. Then, if you dont have serious
side effects, and the drug combination seems to help you, your doctor may keep
you on it long term. The most common side effects of azathioprine include:
- Nausea, vomiting, diarrhea, and fever and
chills
- Anemia and low platelet and white blood cell
counts
- Liver problems
- Pancreatitis or lymphoma (rarely)
- Cyclophosphamide (Cytoxan®) is
another immune system suppressant that doctors use to treat IPF. They usually
add it to low doses of prednisone for patients who are getting worse while
taking prednisone alone. Many patients who can't take prednisone take
cyclophosphamide alone.
- Most people take
cyclophosphamide by mouth every day. Some IPF patients receive it for 3 to 5
days through a needle that is inserted in a vein in the arm. After that, they
take it by mouth every day. You usually start on a low dose thats
increased over time. It may take 3 to 6 months before you see any benefits from
cyclophosphamide.
- The most common side effect of
cyclophosphamide is a decrease in the number of blood cells that you have. This
increases your chances for infection. Your doctor may order blood tests before,
during, and after your treatment to see how your blood cells are affected by
the drug. Other side effects can include:
- Infertility in both men and women.
- Nausea, diarrhea, and fatigue
(tiredness).
- Hair loss.
- Bladder irritation. Some people who have
taken cyclophosphamide for more than 2 years have developed bladder cancer. If
you take cyclophosphamide, you should drink at least 8 glasses of water a day.
Your doctor should test your urine at least monthly.
Other medicines that may help people with IPF
include the following:
- Influenza and pneumonia vaccinations may help
prevent infection and keep you healthy.
- Cough medicines or oral codeine may relieve
coughing.
- Vitamin D, calcium, and a bone-building drug may
help prevent bone loss if you are taking prednisone or another
corticosteroid.
- Anti-reflux therapy may help control
gastroesophageal reflux disease.
New Medicines Being Studied
Researchers like those in the
NHLBI's Idiopathic Pulmonary Fibrosis Network are studying new
treatments for IPF. Some are looking at medicines that may reduce inflammation
and/or prevent or reduce scarring in IPF. These include medicines that are used
to treat other conditions.
N-acetylcysteine. N-acetylcysteine
is an antioxidant thats used mostly to thin mucus in patients with other
lung conditions. A recent study showed that adding it to prednisone and
azathioprine helped prevent further damage to the lungs of people with IPF.
Interferon gamma-1b. Interferon
gamma-1b is a manmade version of a substance that your body normally produces
to help fight infections. The drug is usually injected under your skin three
times a week. The most common side effects include fever, headache, muscle
soreness, fatigue, and chills.
Etanercept. Etanercept is a
medicine thats used to reduce signs and symptoms of active arthritis or
rheumatoid arthritis, such as joint swelling, pain, tiredness, and morning
stiffness. Etanercept is also used to slow the progress of arthritis. The most
common side effects are chills, cough, fever, sneezing, and sore throat.
Bosentan. Bosentan is a medicine
developed to reduce high blood pressure in the blood vessels of the lungs
(pulmonary arterial hypertension). You usually take this drug by mouth once or
twice a day. The most common side effects include headaches, flushing, leg
swelling, dizziness and fatigue, and liver problems. If you take this medicine,
your doctor should test your blood often to monitor how your liver is
working.
Imatinib. Imatinib is a medicine
developed to treat a type of leukemia. The drug is usually given by mouth once
a day. The most common side effects include fluid retention (swelling), nausea,
vomiting, diarrhea, muscle cramping, and liver problems. If you take this
medicine, your doctor should do frequent blood tests.
Sildenafil. Sildenafil is a
medicine that is used to treat pulmonary hypertension and erectile dysfunction.
It usually has few side effects.
Pirfenidone. Pirfenidone is a
medicine that may decrease scarring. The drug is usually taken by mouth three
times a day. The most common side effects include a rash and sun sensitivity,
nausea, vomiting, loss of appetite, drowsiness, and fatigue.
Colchicine. Colchicine is a
medicine that has been used to prevent or treat attacks of gout. It may slow
scarring in IPF. You usually take it by mouth once or twice a day. The side
effects are generally less serious than those from prednisone. They may include
nausea, vomiting, stomach pain, and diarrhea.
Methotrexate. Methotrexate is an
immune system suppressant thats used to treat some cancers and autoimmune
diseases. Most people take it once a week by mouth or injection. People with
IPF usually take it for at least 4 to 6 months to see if it works. You may have
side effects, especially if you take it at high doses.
The most serious possible side effect is liver
damage. Your doctor should monitor how your liver is working every month that
youre on this drug. If you take methotrexate for more than 2 years, you
may want to have a liver biopsy to make sure your liver hasnt been
damaged and that you can continue to take the medicine.
Other side effects may include nausea, mouth sores,
skin rash, a decrease in infection-fighting white blood cells (requiring
regular blood tests to check white cell levels), and an allergic reaction in
the lungs that goes away when you stop taking the drug (this is very rare).
Your doctor should also monitor your white blood
cells each month while youre on methotrexate. You can often reduce your
chances of having bad side effects from methotrexate by taking folic acid.
Penicillamine. Penicillamine is
used to treat rheumatoid arthritis and to prevent kidney stones. Its
available in pill or capsule form. Several small studies have shown that
penicillamine can improve lung function in some IPF patients, but more research
is needed.
Penicillamine can cause serious side effects. The
most common include nausea, vomiting, diarrhea, dyspepsia, and anorexia.
Cyclosporine. Cyclosporine is
another immune system suppressant. Its used mostly to prevent rejection
of kidney, liver, and heart transplants. Some doctors think it may help people
with IPF reduce their dose of corticosteroids while waiting for a lung
transplant. More research is needed.
Oxygen Therapy
When the amount of oxygen in your blood gets low,
you may need oxygen therapy to help reduce your shortness of breath and let you
be more active. Oxygen is usually given through nasal prongs or a mask. At
first, you may need it only during exercise and sleep. As your condition gets
worse, you may need it all the time.
Pulmonary Rehabilitation
Pulmonary rehabilitation is now the standard of care
for people with ongoing lung disease. It usually involves treatment by a team
of specialists in a specialized clinic. The goal is to teach you how to manage
your condition and function at your best.
Services usually include:
- Physical conditioning training
- Breathing exercises and retraining, so that it
takes less energy for you to breathe
- Anxiety, stress, and depression management
- Nutritional counseling
- Support groups
Lung Transplantation
Early referral for surgery to replace one of your
lungs with a healthy lung from a human donor is usually recommended if you:
- Are younger than 65
- Have no other medical problems
- Are not being helped by medicines
Single lung transplantation can improve your quality
of life and help you live longer.
Complications can include rejection by the body of
the transplanted lung and infection. You may have to take medicines for life to
reduce the chances that your body will reject the transplanted lung.
Because the supply of donor lungs is limited, asking
for an evaluation for a transplant as soon as possible is important.
Living With Idiopathic Pulmonary Fibrosis
There is no cure for idiopathic pulmonary fibrosis
(IPF) yet. Understanding that your symptoms may get worse over time is
important. As your symptoms get worse, you may not be able to do many of the
things that you did before you had IPF.
If youre still smoking, the most important
thing you can do is stop. Ask your doctor or nurse about smoking cessation
programs and products that can help. Avoid secondhand smoke as well. Ask family
members and friends not to smoke in front of you or in your home, car, or
workplace.
Ongoing medical care is important. Treatment by a
pulmonologist who specializes in IPF is usually recommended. These specialists
are usually located at major medical centers.
Treatment may relieve your symptoms and even slow or
stop the fibrosis (scarring). Following your treatment plan is very important.
You should:
- Take your medicines as your doctor
prescribes
- Make any changes in diet or exercise that your
doctor recommends
- Keep all of your appointments with your
doctor
- Enroll in pulmonary rehabilitation
Being as active as you can is important to both your
physical and mental health. It can help you maintain your strength and lung
function and reduce stress. Try moderate exercise, such as walking or riding a
stationary bike. Ask your doctor about using oxygen while you do this.
As your condition advances, use a wheelchair or
motorized scooter, or stay busy with activities that arent physical in
nature.
As your condition worsens, you may need oxygen full
time. Some people with IPF carry portable oxygen when they go out.
You should also follow a healthy diet. Eating
smaller, more frequent meals may relieve stomach fullness, which can make it
difficult to breathe. If you need help with your diet, ask your doctor to
arrange for a dietitian to work with you.
Getting plenty of rest can increase your energy and
help you deal with the stress of living with a serious disease like IPF.
Maintain a positive attitude. Practicing relaxation
techniques may help you do this. It may also help you avoid excessive oxygen
intake caused by tension or overworked muscles.
Join a support group to help you adjust to living
with IPF. Talk to others who have the same symptoms so you can see how they're
coping. To find a local support group, talk to your doctor or check your
telephone directory.
Avoid situations that can make your symptoms worse,
such as traveling by air or living at or traveling to high altitudes where the
air is thin and the amount of oxygen in the air is low.
Key Points
- Pulmonary fibrosis is a condition in which tissue
deep in your lungs becomes thick and stiff, or scarred, over time. The
development of the scarred tissue is called fibrosis. As the tissue becomes
thicker, your lungs lose their ability to move oxygen into your bloodstream,
and your brain and other organs dont receive the oxygen they need.
- Doctors dont know what causes most cases of
pulmonary fibrosis. They call these cases idiopathic pulmonary fibrosis
(IPF).
- IPF varies from person to person. In some people,
the lung tissue quickly becomes thick and stiff. In others, the process is much
slower, and in some people, the condition stays the same for years.
- At present, there is no cure for IPF. Many people
live only 3 to 5 years after diagnosis.
- Doctors dont know what causes IPF. They
think that something inside or outside of the lungs attacks the lungs again and
again over time. These attacks injure the lungs and cause scarring in the
tissue inside and between the air sacs.
- Some things that may increase your chances of
developing IPF include: cigarette smoking, viral infections, exposure to
certain environmental pollutants, use of certain drugs, and having
gastroesophageal reflux disease.
- Your genes (the basic units of heredity) may also
play a role in the development of IPF. Some families have at least two members
with IPF.
- The most common symptoms of IPF are shortness of
breath and a dry, hacking cough.
- Other symptoms that you may develop over time
include rapid, shallow breathing; gradual, unintended weight loss; fatigue
(tiredness) or malaise (a general feeling of being unwell); aching muscles and
joints; and enlargement of the fingers or toes, which is called clubbing.
- IPF may lead to other serious medical conditions,
including a collapsed lung, lung infections, blood clots in the lungs, and lung
cancer.
- As your condition gets worse, you may develop
other potentially life-threatening conditions, including respiratory failure,
pulmonary
arterial hypertension, and
heart
failure.
- To diagnose IPF, your doctor will do a detailed
medical history, a physical exam, and several diagnostic tests, including a
chest x ray, high-resolution computerized tomography, lung function tests,
pulse oximetry, arterial blood gas tests, a skin test for tuberculosis, and
exercise testing.
- Looking at samples of tissue from several places
in your lungs under a microscope is the best way for your doctor to diagnose
IPF. Video-assisted thoracoscopy is the procedure that doctors use most to
collect the tissue. Your doctor inserts a small, lighted tube with a camera
(endoscope) into your chest through small incisions between your ribs. The
endoscope provides a video image of the lungs and allows your doctor to collect
tissue samples. This procedure must be done in the hospital under general
anesthesia.
- Treatment cant remove scarring that has
already happened. Therefore, diagnosing and treating IPF as early as possible
(before a lot of scarring has occurred) is very important.
- Recommended treatments include medicines to
reduce inflammation (swelling) in your lungs and/or prevent more scarring,
oxygen therapy, pulmonary rehabilitation, and lung transplantation. Pulmonary
rehabilitation is now the standard of care for people with ongoing lung
disease.
- The main treatment for IPF is medicine, such as
prednisone, to reduce inflammation. Many doctors also add a drug to suppress
your body's immune system. These treatments can prevent further scarring and
increase survival time in some people, but they dont work for
everyone.
- Other medicines that may help people with IPF
include flu and pneumonia vaccinations; cough medicines or oral codeine;
vitamin D, calcium, and a bone-building drug; anti-reflux therapy; and
antioxidants.
- If youre still smoking, the most important
thing you can do is stop. Avoid secondhand smoke as well.
- Ongoing medical care is important. Treatment by a
pulmonologist who specializes in IPF is usually recommended.
- Taking your medicines as your doctor prescribes,
making any changes in diet or exercise that your doctor recommends, keeping all
of your appointments with your doctor, and enrolling in pulmonary
rehabilitation are important.
- Other things you can do for both your physical
and mental health include being as active as you can, following a healthy diet,
getting plenty of rest, joining a support group, and, as your condition
advances, using a wheelchair or motorized scooter and staying busy with
activities that arent physical in nature.
Links to Other Information About Idiopathic
Pulmonary Fibrosis
NHLBI Resources
Non-NHLBI Resources
Clinical Trials
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