Frequently Asked Questions about IPF
 
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arrow_purple Does the CPF provide information regarding Pulmonary Fibrosis specialists for initial treatment or second opinions?
arrow_purple What is Idiopathic Pulmonary Fibrosis?
arrow_purple What caused my Pulmonary Fibrosis?
arrow_purple Is Pulmonary Fibrosis the same as Cystic Fibrosis?
arrow_purple What if my doctor called my condition by another name?
arrow_purple How many people are suffering from Pulmonary Fibrosis?
arrow_purple Is Pulmonary Fibrosis genetic?
arrow_purple How does Pulmonary Fibrosis affect a person?
arrow_purple What causes Pulmonary Fibrosis?
arrow_purple Why does the lung scarring take place?
arrow_purple What are the symptoms?
arrow_purple How is Pulmonary Fibrosis diagnosed?
arrow_purple What types of test will my doctor administer to determine if I have Pulmonary Fibrosis?
arrow_purple What may happen as Pulmonary Fibrosis progresses?
arrow_purple How is Pulmonary Fibrosis treated?
arrow_purple Is a lung transplant an option?
arrow_purple What can I do?
arrow_purple Are there any new treatments on the horizon?
arrow_purple Where do I turn if I have questions about these trials or want to be involved in a study?
arrow_purple Once I understand more about Pulmonary Fibrosis, how else can the CPF help
Does the CPF provide information regarding Pulmonary Fibrosis specialists for initial treatment or second opinions?
It’s not surprising that many patients ask about getting a second or third opinion. The CPF can easily connect patients with specialists across the country as well as those closest to where they live. The CPF has relationships with PF specialists nationwide. Some of the best doctors in the world are also part of the CPF’s Board of Directors and Scientific Advisory Board, which are listed at  www.coalitionforpf.org/board-of-directors/.
What is Idiopathic Pulmonary Fibrosis?
Idiopathic pulmonary fibrosis (PF) is a debilitating disease-marked by progressive scarring of the lungs-that gradually interferes with a person’s ability to breathe. Pulmonary Fibrosis belongs to a family of approximately 100 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).
What caused my pulmonary fibrosis?
Sometimes Pulmonary Fibrosis can be linked to a particular cause, such as certain environmental exposures, chemotherapy or radiation therapy, residual infection, or autoimmune diseases such as scleroderma or rheumatoid arthritis. However, in many instances, no known cause can be established. When this is the case, it is called idiopathic pulmonary fibrosis or IPF.
Is Pulmonary Fibrosis 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. Pulmonary Fibrosis is a completely different clinical diagnosis.
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 Pulmonary Fibrosis. This confusion may be due, in part, to similarities between Pulmonary Fibrosis and the other forms of interstitial lung disease. Only since 2001 has Pulmonary Fibrosis been recognized as a distinct clinical disorder, meaning that specific clinical criteria were developed to determine a diagnosis. Your physician combines clinical information derived from a medical evaluation and certain diagnostic tests to diagnose Pulmonary Fibrosis according to these criteria.
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How many people are suffering from Pulmonary Fibrosis?
Approximately 128,000 people in the United States have Pulmonary Fibrosis, 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.
How does Pulmonary Fibrosis affect a person?
Pulmonary Fibrosis 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, Pulmonary Fibrosis is ultimately disabling and can be fatal. The median survival rate is just over three years.
Is Pulmonary Fibrosis genetic?
Pulmonary Fibrosis appears to be genetic in as many as 15% of all Pulmonary Fibrosis cases.  Many scientists believe research involving these families may hold the key to understanding the causes of Pulmonary Fibrosis and may lead to future treatments or a cure. A large familial Pulmonary Fibrosis research study is underway at National Jewish Health to further study the genetic underpinnings of Pulmonary Fibrosis that may be found in families like this. Recent research has identified two genetic mutations that are associated with familial pulmonary fibrosis, and tests for those mutations have recently become available to the public.If you have two or more cases of Pulmonary Fibrosis diagnoses in your family, you may want to get involved.  The CPF and National Jewish Health established the first Genetic Counseling program for families affected by pulmonary fibrosis. For further information on the Familial Pulmonary Fibrosis Genetic Counseling Program, to speak with a genetic counselor, or to learn how your family can participate in this landmark study of Pulmonary Fibrosis, please call 1-800-423-8891, ext. 1022.
What causes Pulmonary Fibrosis?
While the cause of Pulmonary Fibrosis remains a mystery, what researchers do know is that Pulmonary Fibrosis involves changes in the lung’s normal healing process. Pulmonary Fibrosis’ 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 the lungs less able to function and provide the body with the oxygen it needs.
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 Pulmonary Fibrosis. 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 Pulmonary Fibrosis 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.

How is Pulmonary Fibrosis diagnosed?
Because Pulmonary Fibrosis 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 PF is usually a matter of ruling out other diseases. Also, until recently, the medical community had no agreed-upon standards for the diagnosis of Pulmonary Fibrosis. Consequently, other related diseases may have been mistakenly classified as Pulmonary Fibrosis. With new diagnostic standards now in place, the recognition and management of PF should be substantially improved.
How is Pulmonary Fibrosis diagnosed?
Because Pulmonary Fibrosis 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 PF is usually a matter of ruling out other diseases. Also, until recently, the medical community had no agreed-upon standards for the diagnosis of Pulmonary Fibrosis. Consequently, other related diseases may have been mistakenly classified as Pulmonary Fibrosis. With new diagnostic standards now in place, the recognition and management of PF should be substantially improved.

What types of test will my doctor administer to determine if I have Pulmonary Fibrosis?
To diagnose Pulmonary Fibrosis, 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.

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 Pulmonary Fibrosis progresses?
Pulmonary Fibrosis 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 Pulmonary Fibrosis makes it difficult for a person to fight infection
  • Pulmonary Fibrosis 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).
  • Pulmonary Fibrosis 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.
How is Pulmonary Fibrosis treated?
Physicians generally follow standards of care intended to improve symptoms and hopefully slow the progression of the disease. In 2001, a panel of experts sponsored by the American Thoracic Society and the European Respiratory Society recommended that if therapy was given, it should consist of a trial of corticosteroids and an immunosuppressive agent (e.g. azathioprine or cyclophosphamide). Recent evidence has suggested that adding a third therapy called N-acetylcysteine (NAC) to this regimen may be beneficial. It is important to recognize, however, that there are no definitive studies showing that this treatment approach is effective, and there is no consensus regarding the use of this approach in the pulmonary community. For a complete listing of active clinical trials for Pulmonary Fibrosis, please visit www.coalitionforpf.org.Importantly, these therapies can cause side effects – some minor and some more serious. The potential risks and benefits of therapy should be discussed with your physician in detail. Be sure to contact your doctor or nurse if you have any negative reactions to any medications you have been prescribed.In all cases, doctors should consider referring their patients to a clinical trial to gain access to experimental treatments and also refer patients for lung transplantation evaluation.
Is a lung transplant an option?
In some cases, doctors may consider lung transplantation as a life-saving treatment option for Pulmonary Fibrosis. Please visit the Lung Transplantation section of our Web page for comprehensive information about lung transplantation to learn more. This procedure is most often performed in patients under 60 years of age whose Pulmonary Fibrosis has not responded to other treatments, but eligibility criteria vary between medical centers, which is why it is critical to discuss this potential treatment with your doctor as soon as possible.
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What can I do?
If you have been diagnosed with Pulmonary Fibrosis, 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 Pulmonary Fibrosis:

  • 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.
  • Take all prescribed medications as instructed by your doctor.
  • Get your influenza (flu) vaccine every year, and also make sure that your pneumococcal vaccine (Pneumovax) is up to date.
  • 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.
  • 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, while protecting your heart and other vital organs.
  • Consult your physician about enrolling in a pulmonary rehabilitation or respiratory therapy 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 education and exercise to their treatment. Please note you can participate in a respiratory therapy program even if you are currently taking supplemental oxygen.
  • Eat a well-balanced diet to maintain in ideal body weight. 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.
  • Talk about Pulmonary Fibrosis: 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 members of Congress about Pulmonary Fibrosis and join the CPF in its work to increase Congressional attention to the disease.
Are there any new treatments on the horizon?
Researchers are developing and testing a variety of new ways to treat Pulmonary Fibrosis. These approaches target the various steps in the disease process.  Investigational therapies currently in clinical trials include:

  • Antifibrotic or anti-fibrogenic agents to suppress the scarring process
  • Antioxidants (such as N-acetylcysteine-NAC) to prevent damage to lung tissue
  • Endothelin antagonists (such as certain medications for pulmonary arterial hypertension (PAH)
  • Monoclonal antibodies to inhibit “bad” cytokines (protein growth factors, such as TGF-beta, TNF-Alpha, VEGF, 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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Where do I turn if I have questions about these trials or want to be involved in a study?

If you have any questions about experimental treatments or ongoing clinical trials,
ask your doctor, contact the CPF at info@coalitionforpf.org, or click here for an up-to-date listing
of active studies, locations, and study-specific contact information.

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Once I understand more about Pulmonary Fibrosis, how else can the CPF help?

We are a resource for patients and families across the entire continuum of care for their fight with Pulmonary Fibrosis.  We educate patients on all of the possible treatment options and clinical trials available.  We educate patients about lung transplantation as a potential treatment option. We can also refer them to the best transplant centers, specializing in Pulmonary Fibrosis, in the country, and refer prospective transplant candidates to patients who’ve received transplants and can share their experience and offer support.Patients are also educated on the importance of other standards of care associated with PF such as pulmonary rehabilitation, oxygen management, nutrition, and support groups. We are available to discuss end of life issues with patients and their families and can help with virtually any situation that arises during the natural course of their condition.Our goal is to empower patients with information and education so they can regain a sense of control while facing a disease that has seemingly taken away their control. We talk about being active in the search for answers.  The CPF has found many friends in those who care about changing the future of Pulmonary Fibrosis. They gain healing and strength from doing things such as taking part in our national advocacy campaign, holding a fundraiser,  or helping to raise public awareness.

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