Idiopathic pulmonary fibrosis is by far the most common version of pulmonary fibrosis. The term idiopathic means that there is not a known cause of the disease, and most of the time, doctors do not know what caused a case of pulmonary fibrosis. Idiopathic pulmonary fibrosis and pulmonary fibrosis with a known cause are treated the same way - with medication, oxygen therapy, pulmonary rehabilitation, and possibly a lung transplant.
Pulmonary Fibrosis Treatment
1 AnswerNumerous studies of pulmonary rehabilitation have documented improvement in symptoms, maximum oxygen consumption, and quality-of-life measures. A decrease in the number of hospitalizations has also been shown in patients who participate in pulmonary rehabilitation programs.
Benefits do vary among individuals, however, and consistent participation in an exercise regimen is necessary to maintain improvements. In addition, it has not been shown that pulmonary rehabilitation produces any change in pulmonary functions tests (PFTs) or overall oxygen requirements for individuals.
Potential benefits include:
- Maximization of strength, endurance, and flexibility
- Decreased symptoms of breathlessness
- Decreased effort for the activities of daily living
- Increased energy to partake in hobbies and social/family activities
Currently there is no cure for pulmonary fibrosis. Research is ongoing to find both a cure and more effective treatments for the condition. For now, your best option is to receive treatment, which may include medications, oxygen therapy, pulmonary rehabilitation, and a lung transplant.
2 AnswersDr. Anthony L. Komaroff, MD , Internal Medicine, answeredPulmonary fibrosis is a condition in which there is significant or widespread scarring in the lungs.
The disease has many causes or triggers. These include medications, immune diseases and exposure to inhaled toxins such as asbestosis. Often, no trigger is found.
As in other parts of the body, scar tissue is usually permanent. There is currently no way to treat, cure or slow the progression of pulmonary fibrosis.
Therapy can be used to reduce symptoms, such as breathlessness or coughing. Avoiding all pulmonary toxins (such as air pollution) and not smoking are essential.
Treating pulmonary fibrosis may include:
- Pulmonary rehabilitation (exercise, chest physical therapy, and education)
- Immune suppression and anti-oxidant therapy (including the drugs azathioprine, glucocorticoids, and acetylcysteine)
- Lung transplantation
Research is looking to discover therapies that can slow or reverse lung scarring. One recent study found that sildenafil (as found in Viagra and Revatio) improved symptoms in some people with pulmonary fibrosis. Therefore, it's worth asking your doctor about clinical trials of promising experimental treatments. You may be able to enroll in a study in your area.
Life expectancy with pulmonary fibrosis varies. Minor amounts of scarring in the lung may have little or no impact on lifespan. People with more severe or progressive disease may have a 5-year survival as low as 20%. However, a number of factors go into these estimates and doctors are not very accurate in predicting life expectancy with this condition.
Oximetry tests can help doctors diagnose pulmonary fibrosis, as well as monitor the progression of the disease. Oximetry tests measure how much oxygen is circulating in your bloodstream. The test is simple and painless - a small device is clipped onto your finger or earlobe, and the device measures your oxygen amounts. Oximetry can help track the course of the pulmonary fibrosis as accurately as a chest x-ray, if not more.
There are no alternative treatments available for pulmonary fibrosis. Professional medical treatment is the only option to relieve symptoms, slow the progression of the disease, improve your quality of life, and help you live longer. If you have pulmonary fibrosis, talk to your doctor about your treatment options.
Pulmonary fibrosis is considered a rare disease. A 2006 study reported that about 128,000 people in the United States have pulmonary fibrosis, which is about 1 in every 2400 Americans. However, some estimates indicate that as many as 200,000 Americans may have the disease (about 1 in 1550). Causing over 40,000 deaths each year, pulmonary fibrosis kills as many Americans every year as does breast cancer.
Medications treat pulmonary fibrosis by reducing inflammation and suppressing your immune system, which helps prevent the disease from damaging your lungs more and can help you live longer. When your lungs are inflamed by the disease, the immune system responds by building scar tissue, which is what damages your lung function. Prednisone is an anti-inflammatory drug called a corticosteroid that doctors often prescribe to treat the disease. Sometimes doctors will prescribe an immune system suppressant such as azathioprine or cyclophosphamide that will keep your immune system from forming scar tissue. Medications aren't effective treatments for everyone who has pulmonary fibrosis.
Following your doctor-prescribed treatment plan and making lifestyle changes are important to managing your pulmonary fibrosis and improving your quality of life. Aside from taking medications and following other recommendations made by your doctor, you should enroll in pulmonary rehabilitation. The program will teach you different ways to manage your pulmonary fibrosis with programs for diet, exercise, efficient breathing, education, counseling, and emotional support. Your doctors and program directors will encourage you to:
- Quit smoking and avoid secondhand smoke.
- Be active. Physical activity will keep your strength up, manage stress, and maintain lung function.
- Eat a healthy diet that includes whole grains, fruits, vegetables, lean meats, poultry, fish, and beans. Consumption of foods high in cholesterol, saturated fat, trans fat, salt, and added sugar should be kept at a minimum.
- Sleep more to increase energy and manage your stress.
- Be positive.
If you know of situations such as air travel and high altitudes that will aggravate your pulmonary fibrosis, avoid them. Talk to your doctor and rehabilitation specialists for other ways to manage your pulmonary fibrosis.
People who have pulmonary fibrosis often have to make lifestyle changes, and if you are close to someone who has the disease, you may have to adjust your own lifestyle as well. Tobacco smoke is very unhealthy for anyone with a lung disease, so not only does your loved one need to quit smoking, but you also need to avoid smoking around your loved one. If you prepare food for someone with pulmonary fibrosis, consult a dietitian for healthy diet guidelines. The dietitian may suggest a diet high in fruits, vegetables, and whole grains and low in cholesterol, saturated fat, and trans fat. Keep in mind as well that stress aggravates pulmonary fibrosis and that exercise and rest are important to your loved one's health. If you have questions, ask your doctor.