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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 don't receive the oxygen they need.
- Doctors don't 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 don't 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 can't 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 don't 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 you're 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 aren't physical in nature.
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