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| | What is Idiopathic Pulmonary Fibrosis? |
| Idiopathic pulmonary fibrosis (IPF) is a debilitating disease-marked by progressive scarring of the lungs-that gradually interferes with a person's ability to breathe. IPF belongs to a family of approximately 200 related diseases, called interstitial lung diseases (ILDs), that have similar characteristics and can result in scarring. The lung scarring, a condition typical of these disorders, is referred to as pulmonary fibrosis (PF).
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| | What caused my pulmonary fibrosis? |
| Sometimes PF can be linked to a particular cause, such as exposure to metal dust, wood dust, gases, or fumes; chemotherapy or radiation therapy; residual infection; or a connective tissue disease, such as systemic lupus erythematosus or rheumatoid arthritis. In the majority of PF cases, however, no known cause can be established. When pulmonary fibrosis has no known cause, it is called "idiopathic pulmonary fibrosis" or "IPF." The word "idiopathic" means "of unknown cause."
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| | Is IPF the same as Cystic Fibrosis? |
| No. Cystic Fibrosis is a genetic disease that causes the body to produce abnormally thick, sticky mucus, due to the faulty transport of sodium and chloride (salt) within cells lining organs such as the lungs and pancreas, to their outer surfaces. It primarily affects children. IPF is a completely different clinical diagnosis.
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| | What if my doctor called my condition by another name? |
| Your doctor may have referred to your condition by one of a number of names that are sometimes used to describe IPF. This confusion may be due, in part, to similarities between IPF and the other ILDs. Only recently has IPF been recognized as a distinct clinical disorder.
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| | How many people are suffering from IPF? |
| Approximately 128,000 people in the United States have IPF, and prevalence is on the rise with an estimated 48,000 new cases developing each year. Prevalence has increased 156% since 2000, when it was thought that only 50,000 were affected.
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| | Who does IPF affect? |
| Your doctor may have referred to your condition by one of a number of names that are sometimes used to describe IPF. This confusion may be due, in part, to similarities between IPF and the other ILDs. Only recently has IPF been recognized as a distinct clinical disorder.
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| | How does IPF affect a person? |
| IPF hinders a person's ability to take in oxygen. It causes shortness of breath and is usually associated with a persistent dry cough. The disease progresses over time, leading to an increase in lung scarring and a worsening of symptoms. Unfortunately, IPF is ultimately disabling and can be fatal. The median survival rate is just over three years.
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| | Is IPF genetic? |
| IPF appears to be genetic in as many as 10% of all IPF cases. There are thousands of families in the U.S. who have had two or more family members diagnosed with the disease. Many scientists believe research involving these families may hold the key to understanding the causes of IPF and may lead to future treatments or a cure. A large familial IPF research study is underway at Duke University. If you have two or more cases of IPF diagnoses in your family, you may want to get involved. Contact Duke University for more information on the Familial Pulmonary Fibrosis research program by visiting www.fpf.duke.edu or calling 877-587-4411. The CPF and National Jewish Medical and Research Center established the first Genetic Counseling program for families affected by pulmonary fibrosis. For further information on the Familial Pulmonary Fibrosis Genetic Counseling Program or to speak with a genetic counselor, call 1-800-423-8891, ext. 1022.
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| | What causes IPF? |
| While the cause of IPF remains a mystery, what researchers do know is that IPF involves changes in the lung's normal healing process. IPF's chronic cycle of injury leads to an exaggerated or uncontrolled healing response that, over time, produces fibrous scar tissue. This scarring, in turn, causes the lung's tiny air sacs, called alveoli, to thicken and stiffen-rendering them less able to function and provide the body with the oxygen it needs.
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| | Why does the lung scarring take place? |
| Exactly what sets this abnormal tissue-repair process in motion is unclear. The body's own immune response appears to play a major role. Researchers are investigating a number of potential risk factors that may make a person more likely to develop IPF. These risks may include:
- Certain occupational exposures (e.g. metal or wood dust, pesticides)
- Cigarette smoking
- Use of anti-depressant medications
- Asbestos exposure
- Viral infection
- A family history of pulmonary fibrosis
- Acid reflux Disease or GERD
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| | What are the symptoms? |
| Symptoms of IPF usually have a gradual onset and may include:
- Shortness of breath during or after physical activity
- Spasmodic, dry cough
- Weight loss
- Fatigue
Patients may also develop a buildup of tissue in their fingertips. This condition is called clubbing.
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| | How is IPF diagnosed? |
| Because IPF has symptoms and an underlying pattern of scarring similar to those of other lung disorders, it can be difficult to diagnose. In fact, in most cases, identifying IPF is usually a matter of ruling out other diseases. Also, until recently, the medical community had no agreed-upon standards for the diagnosis of IPF. Consequently, other related diseases may have been mistakenly classified as IPF. With new diagnostic standards now in place, the recognition and management of IPF should be substantially improved. What types of test will my doctor administer to determine if I have IPF?To diagnose IPF, your doctor will take a complete medical history and perform a thorough physical examination. During this exam, the doctor will use a stethoscope to listen to your chest to try to determine if your lungs produce any abnormal sounds when you breathe. He or she may then order one or more of the following diagnostic tests or procedures.
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|  | | Diagnosis | Description | Purpose | | Chest Imaging | Use of radiologic machines to take, pictures (x-ray or CT scan) of the lungs | To view lung structures look for scar tissue, and assess patterns of scarring | Pulmonary
function test | A test using a device with a mouthpiece to measure a patient's breathing capacity | To measure the degree of impairment in lung function | | Arterial blood test | A measurement of oxygen levels in blood taken from an artery | To determine how well the lungs are performing vital gas exchange | | Exercise test | A test in which the patient is monitored while using a treadmill or stationary bicycle | To measure how well the lungs and heart respond to physical activity | | Bronchoalveolar lavage (BAL) | A "lung-washing" procedure conducted through a flexible tube (bronchoscope) inserted into the airways through the nose or mouth; fluid (salt water) is injected into the lungs and then removed for inspection | To examine cells and look for signs of inflammation in the lungs | | Lung biopsy | A procedure in which a tissue sample is obtained through a bronchoscope (see BAL, above) or by means of a small surgical incision between the ribs (open-lung biopsy)
| To obtain a sample of lung tissue for direct examination | |  |
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| | What may happen as IPF progresses? |
| IPF affects each person differently and progresses at varying rates. Generally, the patient's respiratory symptoms become worse over time. Activities (such as walking or climbing stairs) become more difficult. In addition:
- The patient may require supplemental oxygen
- Advanced IPF makes it difficult for a person to fight infection
- IPF causes a lack of oxygen in the blood. This condition (called hypoxemia) puts a strain on the heart and on the blood vessels in the lungs, and may lead to high blood pressure in the lunds (pulmonary hypertention).
- IPF has also been associated with these potentially life-threatening conditions: heart attack, respiratory failure, stroke, blood clot in the lungs (pulmonary embolism), lung infection, and lung cancer.
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| | How is IPF treated? |
| Current treatments are intended to improve symptoms and slow disease progression. As yet, there is no cure for IPF. A panel of experts sponsored by the American Thoracic Society and the European Respiratory Society has developed guidelines for the treatment of IPF. This panel recommends the following as standard IPF therapy: - An anti-inflammatory corticosteroid (e.g prednisone) used in combination with:
- A drug to suppress the body's immune response (azathioprine) This treatment approach is effective in only a small number of cases and tends to work best when started early in the course of the disease. These drugs can also cause side effects-some minor and some more serious. Be sure to contact your doctor or nurse if you have any negative reactions to your medications.
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| | Is a lung transplant an option? |
| In some cases, doctors may consider lung transplantation. Please visit the Lung Transplantation section of our Web page for comprehensive information about lung transplantation for IPF. This procedure is most often performed in patients under 60 years of age whose IPF has not responded to other treatments.
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| | What can I do? |
| If you have been diagnosed with IPF, there are a number of things you can do to take part in your own treatment and help yourself stay healthy. Caregivers may also be interested in the following information, to assist a family member diagnosed with IPF. - Join the CPF! Complete our registration form to stay abreast of the latest information and resources available to the IPF community by clicking here - Take all prescribed medications as instructed by your doctor. - If you smoke, it is very important that you stop as soon as possible. Ask your doctor or nurse about smoking cessation programs and products that can help. - Eat a well-balanced diet. This helps support your body and keeps up your strength. Discuss any special nutritional concerns with your doctor or nurse. - Consider eating smaller, more frequent meals during the course of your day. Many patients find it easier to breathe when their stomach isn't completely full. - Try some moderate exercise, such as walking or riding a stationary bicycle. If you're already exercising, keep up your regular workout. This helps you maintain strength and lung function. Talk to your doctor before starting a new exercise program. Those who need to can generally use oxygen during this kind of activity. - Consider enrolling in a pulmonary rehabilitation program to help increase your strength, learn breathing techniques, and expand your social support network. Ask your doctor or nurse for more details. Many patients report improved breathing and quality of life after adding respiratory therapy to their treatment. - Your doctor may have prescribed supplemental oxygen, which can provide your body with the oxygen it needs but your lungs can no longer supply. Some patients fear that they will become addicted to oxygen, but this is not true. Supplemental oxygen can help you feel less breathless and more energetic. - Join an IPF support group; the CPF has helped to create more than 40 IPF support groups across the country. If there is not yet an IPF support group in your area, join a lung disease support group or other similar group. It's a great way to get the emotional support you need. Visit the CPF website page with the latest support group listings at: http://www.coalitionforpf.org/cpf_support.php. If you would like to start one of your own, contact the CPF for your free copy of the "IPF Support Group Coordinator's Kit". Patients just like you have helped create IPF support groups in their communities. - Talk about IPF: Get the word out to raise awareness about the disease. Tell your friends, colleagues and local newspapers about your experience - your story can make a difference. Speak and write to legislative members about IPF and join CPF in its work to increase Congressional attention to the disease. - Call your doctor or nurse with any questions about your condition or its treatment. If you notice anything unusual about how you are feeling or how your medicines are working, call your doctor right away. | | |
| | Are there any new treatments on the horizon? |
| Researchers are developing and testing a variety of new ways to treat IPF. These approaches target the various steps in the disease process. Therapies under investigation include: - Antifibrotic or antifibrogenic agents (such as Pierfenidoneand certain blood-pressure-lowering medications) to suppress the scarring process
- Antioxidants (such as N-acetylcysteine) to prevent damage to lung tissue
- Endothelin antagonists (such as Bosentan)
- Monoclonal antibodies to inhibit "bad" cytokines (protein growth factors, such as TGF-beta, TNF-Alpha, or CTGF, that activate inflammation) A complete list of all clinical trials and research in the U.S. is available by click here.
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