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Pulmonary Fibrosis

Online Health Chat with Dr. Joseph Parambil

September 14, 2011

Introduction

Cleveland_Clinic_Host: Idiopathic pulmonary fibrosis (IPF) is a progressive disease isolated to the lungs. It is a type of interstitial lung disease, which is a group of 200 diseases with similar symptoms but different causes. In IPF, lung tissue becomes scarred. The scarring typically starts at the edges of the lungs and progresses toward the center of the lungs, making it more difficult for a person to breathe. Unfortunately, IPF is a disabling disease that can be fatal. Close to 130,000 Americans are currently affected by the disease, and more than 50 percent of those affected lose their lives within four years of diagnosis, with almost all cases eventually ending in death.

The cause of IPF is unknown. In some patients, the disease is genetic (you inherited the disease from your parents). Environmental factors (particularly exposure to certain types of dusts) may also play a role. What is known is that IPF changes the lung's ability to function normally. Typically, mild scarring of the lung tissue occurs first, but over months to years, the normal lung tissue is replaced by more heavily scarred lung tissue, which makes it difficult to breathe and deliver needed oxygen to the body.

Joseph Parambil, MD, is an Associate Staff Member in the Department of Pulmonary, Allergy and Critical Care Medicine. He is also Assistant Professor of Medicine at Cleveland Clinic’s Lerner College of Medicine. Dr. Parambil earned his medical degree from Semmelweis Medical University in Budapest, India. He completed a surgical internship at Frankford Hospital in Philadelphia, PA. He completed an internal medicine residency at Henry Ford Hospital in Detroit, MI, as well as a fellowship in pulmonary and critical care medicine at Mayo Clinic in Rochester, MN. He is certified by the American Board of Internal Medicine with additional specialty certification in pulmonary medicine and critical care medicine. Dr. Parambil has presented a number of times on topics such as disease pathology, connective tissue disorders, and the causes and associations of pulmonary infarctions, among many others. He has also published a number or articles in peer-reviewed journals, as well as having published a number of abstracts and a book chapter on pulmonary hypertension in pregnancy.

To make an appointment with Dr. Parambil, please call 216.444.6503 or 800.223.2273, ext. 4650. You can also visit us online at clevelandclinic.org/respiratory.

Cleveland_Clinic_Host: Welcome to our Online Health Chat with Joseph Parambil, MD. We are thrilled to have him here today for this chat. Let’s begin with some of your questions.


Basics

shaneme123: Can one get pulmonary fibrosis from receiving chemotherapy for Hodgkin’s lymphoma (specifically bleomycin) and then SCUBA diving a year later? I see theories on the Internet but no actual cases.

Dr__Joseph_Parambil: Certainly chemotherapy - especially the use of bleomycin - can be worsened by concomitant chest radiation and has been associated with pulmonary fibrosis. SCUBA diving has little impact on the development of pulmonary fibrosis.

davidfay: Can pulmonary fibrosis (possibly caused by inhaling insulation particles) go away?

Dr__Joseph_Parambil: Pulmonary fibrosis can occasionally occur in the context of inhalation lung injury. The classic example would be asbestos-related pulmonary fibrosis. We always recommend patients remove themselves from further particle exposure in order to prevent continued deterioration of lung function. However, whatever fibrosis has been established remains and does not go away.

davidfay: Is pain common in IPF?

Dr__Joseph_Parambil: Rarely. IPF patients can develop pneumothorax and that can cause pain. If the pain is excruciating and constant, I would recommend seeing your doctor at the earliest possible time.

Dr__Joseph_Parambil: If you would like us to evaluate you or give a second opinion, please call 216.444.7567


Diagnosis and Treatment

Ftrnurse9: My 51-year-old mother was diagnosed with IPF per open biopsy after a sudden onset of SHOB (shortness of breath). From onset of s/s (signs and symptoms) until today it has been 2 1/2 months. The pulmonologist said her TLC (total lung capacity) was 38.7. My questions are: Are there any other treatments besides prednisone, because the doctor said to discontinue it if no improvement by Oct. 17, 2011? What is the normal range for TLC? With a patient that has progressed so quickly in such a short time, what are we looking at prognosis and potential life expectancy? Also, are there any clinical trials that she may qualify for and research that may help her rapidly declining condition? If you could supply me with information that may help us understand the disease and possibly prepare us for what is to come, that would be greatly appreciated.

Dr__Joseph_Parambil: Given the young age of your mother being diagnosed with IPF and the rapidity of progression, the best option here would be an evaluation toward lung transplantation. Cleveland Clinic has the largest lung transplant program in the country with enough expertise in this disease. If desired, we would be more than happy to assess your mother.

healthcare: Lung capacity is 36 percent on my pulmonary function test. Doctor here wants me to go to pulmonary rehab. How can I exercise when I can't breathe? I have shortness of breath, and one doctor thinks it may be pulmonary hypertension. Another says the right side of my heart is enlarged and, therefore, not letting in enough oxygen (something like that); meanwhile, they gave me Revatio (sildenafil) -- made me retain water, legs swelled and was more short of breath. So now they are saying it couldn't be PH because that medication should have worked. None of the sprays like Spiriva® work; they make me shorter of breath. What to do? Help!

Dr__Joseph_Parambil: In patients with IPF, we always recommend pulmonary rehab, as cardiomuscular conditioning improves the sensation of dyspnea (shortness of breath). Pulmonary hypertension is commonly seen in patients with IPF and is usually mild. Unfortunately, most of the available treatments for pulmonary hypertension do not make significant improvements in patients with underlying PF. In your condition, given your lung capacity of 36 percent and the presence of pulmonary hypertension, the best option is being assessed toward lung transplantation.

davidfay: My husband has lots of pain since being diagnosed with pulmonary fibrosis and is still coughing up blood. His diagnosis was given two weeks ago. He was given antibiotics and nebulizer. Is this the right treatment?

Dr__Joseph_Parambil: It is unusual for PF to be associated with significant chest pain. I would recommend having your husband worked up to rule out cardiac, esophageal, and musculoskeletal causes.

The coughing up of blood is concerning, and I would recommend that you see the physician at the earliest possible time to assess this further.

In general, nebulizer medication and bronchodilator medicines have a limited role in the management of IPF.


Medications

ExonicJay: I understand several countries have approved a drug treatment for IPF. For example: pirfenidone - Esbriet® ........ Europe and U.S.(Canada?), Pirespa® ...... Japan, Pirfenex® ..... India, BIBF 1120 .... German pharmaceutical giant Boehringer Ingelheim. Can you comment on the effectiveness of these drugs and when we can expect FDA approval here in the USA? A follow-up question: If the effectiveness of pirfenidone is acceptable, would you recommend patients travel to these countries for treatment?

Dr__Joseph_Parambil: Two studies were done on pirfenidone. One was positive. The second one was equivalent, not negative. Based on these studies, Europe and Canada have approved this drug. The FDA has required us here in the U.S. to do a third trial, of which Cleveland Clinic is a part. It should be starting in the next couple of months.

We would encourage you to come here or find a close center that is participating in the upcoming trial.

mmansour: What are your thought on the drug pirfenidone, and what risk factors are there in taking it versus other treatments such as PANTHER?

Dr__Joseph_Parambil: Pirfenidone has had positive results in several studies as early as 1999. However, the medicine is not yet approved in the U.S., although it is approved in Canada and Europe. Cleveland Clinic is part of a third trial in assessing the efficacy of pirfenidone for IPF. Since we do not have results of the PANTHER trial, it is difficult to say if this combination is better.

mmansour: What are the most serious side effects of pirfenidone versus current treatments?

Dr__Joseph_Parambil: Pirfenidone has been shown to demonstrate a very good safety profile. Side effects that may be seen have included GI discomfort and photosensitivity, but this is rare.

doxiemom: My husband is in the PANTHER trial currently and remains stable. He can get into the final phase of study for pirfenidone, but would need to be off the current study medication (or placebo) for 28 days and is somewhat hesitant since he's doing well. Any suggestions?

Dr__Joseph_Parambil: Since he is doing stable and fairly well on the PANTHER trial, I would recommend continuing on the trial. The initial study of this combination done in Europe was positive, and that is why we are testing this over in the U.S.

E1234: What is the role of corticosteroids in IPF?

Dr__Joseph_Parambil: There is no role for corticosteroids in IPF.


PF and Other Disorders

sheleyjw: As pulmonary fibrosis progresses, can it be an underlying cause of memory loss? Will pulmonary fibrosis increase the heart rate? If so, what rate is within a safe zone?

Dr__Joseph_Parambil: It is unusual for PF to cause memory loss; however, when reduced memory is seen in patients with pulmonary fibrosis, it is because of low oxygen levels. You may want to get tested to see whether you have low oxygen levels at rest or with exertion.

To answer your second question: yes, people with pulmonary fibrosis can have an increase in heart rate because of the increased effort of breathing, increased load on the heart, and deconditioning. Generally, a safe heart rate would be up to 85 percent of predicted for your age. That is determined by the formula 220- your age x 0.85.

lagalbraith: Over the last year, my blood pressure has increased, running 145/80. Previously it always ran 110/80. I have been told this increase could be due to the fibrosis in my lungs, and I am now taking Lisprinol 10 mg. What can be done to determine if fibrosis is the cause of the increased blood pressure?

Dr__Joseph_Parambil: It is very unusual for pulmonary fibrosis to directly cause an increase in blood pressure. However, in patients with PF, anxiety tends to be common, and this can drive up your blood pressure.

to: I have obstructive lung disease and interstitial lung disease and lupus and scleroderma and Sjogren’s syndrome and RA. I have had at least five different diagnoses before they agreed on these two. I have had no treatment except steroids. I would like to know my life expectancy as closely as you can calculate. My doctors won’t even give me an idea, and I have to read about it on the Internet. Thank you.

Dr__Joseph_Parambil: It is impossible to predict or calculate life expectancy based on the above diagnosis. Even in the presence of concrete numbers, giving you an exact number would not yield much. However, since you have lung disease on a background of various autoimmune diseases, outcomes for this are significantly better than what you read on the Internet about pulmonary fibrosis.

E1234: Why does pulmonary infection take place in IPF?

Dr__Joseph_Parambil: In IPF, lung reserve is reduced and local immune regulation also becomes abnormal. Therefore, even a simple viral infection that may have been tolerated by you previously now becomes of significant concern. The only advice is that when you notice symptoms of a respiratory infection, you should contact your local doctor or pulmonologist at the earliest, as quelling this can help prevent progressive deterioration in lung function.


Lung Transplant

doxiemom: What are the criteria for lung transplantation? Must the patient be "severe"?

Dr__Joseph_Parambil: There are no specific criteria, as mild or severe, for consideration for lung transplantation. In the past, the earlier the patient got on the transplant list the greater the likelihood of transplantation.

Today, we have a Lung Allocation Score (LAS) that determines how severe a patient is and accordingly moves him or her up or down the list.

to: If I had a lung transplant, wouldn’t my lupus and scleroderma affect the new lung also?

Dr__Joseph_Parambil: In general, lupus does not come back to the affected lung. However, in scleroderma, beside lung fibrosis, patients suffer from an immotile esophagus. This predisposes patients to chronic aspiration and, as a consequence, increased rates of lung rejection.


General Questions

lagalbraith: +I have pulmonary sarcoidosis - diagnosed in 1995. Over the last 16 years, the fibrosis in my lungs is gradually increasing. I was taking Plaquenil and methotrexate, recently just stopped Plaquenil. What do you find to be the best to prevent increased fibrosis?

Dr__Joseph_Parambil: We do have a chat on sarcoidosis scheduled for next Tuesday, and we will be happy to discuss this next week. Please join us then.

to: what do you think of stem cell transplantation from Dr. Burt in Chicago for my obstructive and interstitial lung disease

Dr__Joseph_Parambil: There is preliminary data on the benefits of stem cell transplantation for diffuse scleroderma. Contacting the University of Chicago or NIH (National Institutes of Health) may not be a bad idea to see if you would be a candidate, especially given the additional diagnosis of SLE, RA and Sjogren's syndrome.

to: I forgot to mention that I have limited scleroderma, so would you still recommend I pursue stem cell?

Dr__Joseph_Parambil: Thus far, stem cell transplantation is being considered for diffuse scleroderma and not limited disease.

doxiemom: Given your answer on viral infections, is it safe for an IPF patient to get a flu shot?

Dr__Joseph_Parambil: Yes, and we recommend annual flu vaccination for all of our patients.

sheleyjw: Is it common for a person with IPF to develop a barrel/protruding stomach?

Dr__Joseph_Parambil: It is very unusual to develop a protruding stomach as a direct consequence of IPF, as the disease is limited to the lung and does not affect other organs.

mmansour: Do you know of any well-recommended doctors in this field in the Philadelphia area?

Dr__Joseph_Parambil: No I don't, but we would be happy to facilitate an appointment for you at Cleveland Clinic based on your convenience.

lagalbraith: I have Pulmonary aspergilloma and cough up small amounts of blood < 2 tblsp. They have treated it with voriconazole, and the blood has decreased to almost nothing. Is there a way to know for sure it is gone?

Dr__Joseph_Parambil: This is a chronic pulmonary infection and has no relationship to pulmonary fibrosis. However, to ensure the aspergilloma is responding to treatments, following up with your doctor with CT scans may help.


Closing

Cleveland_Clinic_Host: I'm sorry to say that our time with Joseph Parambil, MD, is now over. Thank you again, for taking the time to answer our questions about pulmonary fibrosis.

Dr__Joseph_Parambil: Thank you and I will be back for the Sarcoidosis chat with Dr. Culver on September 27th at 1 p.m. Eastern Time.


More Information

Cleveland_Clinic_Host: To make an appointment with Dr. Parambil, please call 216.444.6503 or 800.223.2273, ext. 4650. You can also visit us online at clevelandclinic.org/respiratory.

A remote second opinion may also be requested from Cleveland Clinic through the secure eCleveland Clinic MyConsult Web site. To request a remote second opinion, visit www.eclevelandclinic.org/myConsult.

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This chat occurred on 9/14/2011

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