How Is Idiopathic Pulmonary Fibrosis Treated?
Idiopathic pulmonary fibrosis (IPF) is treated with medicines, oxygen therapy, pulmonary rehabilitation (rehab), and lung transplant.
The goals of treating 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 can't 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 usually is based on your age, medical history, any medical problems you may have, and how much the IPF has advanced.
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 don't 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. However, 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 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 on an eye's lens).
- 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 don't produce enough of certain hormones). This condition should be treated by an endocrinologist. This is a doctor who specializes in diagnosing and treating a number of endocrine problems, including adrenal gland problems.
Prednisone also can cause conditions such as diabetes and glaucoma to get worse.
Other Medicines
Many doctors prescribe a second medicine with prednisone.
Azathioprine. This medicine 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 don't 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 (feeling sick to your stomach), vomiting, diarrhea, and fever and chills
- Anemia (a low red blood cell count) and low platelet and white blood cell counts
- Liver problems
- Pancreatitis or lymphoma (rarely)
Cyclophosphamide. This 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 get it for 3 to 5 days through a needle that's inserted into a vein in the arm. After that, they take it by mouth every day. You usually start on a low dose that's 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 recommend blood tests before, during, and after your treatment to see how this medicine affects your blood cells.
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 who have IPF include the following:
- Flu and pneumonia vaccines may help prevent infection and keep you healthy.
- Cough medicines or oral codeine may relieve coughing.
- Vitamin D, calcium, and a bone-building medicine may help prevent bone loss if you're taking prednisone or another corticosteroid.
- Anti-reflux therapy may help control gastroesophageal reflux disease.
New Medicines Being Studied
Researchers, like those in the Idiopathic Pulmonary Fibrosis Network, are studying new treatments for IPF. With the support and guidance of the National Heart, Lung, and Blood Institute, the researchers who are part of this network continue to look for new IPF treatments and therapies.
Some of these researchers are looking at medicines that may reduce inflammation and/or prevent or reduce scarring in IPF.
Oxygen Therapy
When the amount of oxygen in your blood gets low, you may need oxygen therapy. Oxygen therapy can help reduce shortness of breath and allow you to be more active.
Oxygen usually is 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 rehab is now the standard of care for people who have ongoing lung disease. Rehab 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 Transplant
Early referral for a lung transplant may be recommended if you:
- Are younger than 65
- Have no other medical problems
- Are not being helped by medicines
Pulmonary Fibrosis - Single lung transplant 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 the rest of your 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.
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