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. |