Online Health Chat with Dr. Jeffrey Chapman
September 22, 2010
Cleveland_Clinic_Host: Idiopathic Pulmonary Fibrosis is a chronic, fatal lung disease that causes progressive lung dysfunction leading to shortness of breath and ultimately death. The disease literally robs patients of the ability to breath and develops without an identifiable cause. Close to 130,000 Americans are currently affected by the disease and over 50% of those affected lose their lives within four years of diagnosis, with almost all cases eventually ending in death. Idiopathic Pulmonary Fibrosis is devastating disease and you can become better educated on it by joining this free online web chat. Dr. Chapman, a Cleveland Clinic Staff Physician, will be answering your general questions concerning the disease, as well as provide you with tips on how to avoid being a victim of Idiopathic Pulmonary Fibrosis.
Jeffrey Chapman, MD, is a Staff Physician in the Respiratory Institute and the Transplant Institute. His specialty interests include interstitial lung disease, IPF, lung transplantation and advanced lung disease. Dr. Chapman earned his medical degree at Washington University School of Medicine in St. Louis, Missouri and completed his residency in internal medicine at New York Presbyterian Hospital.
He is an American College of Chest Physicians Fellow and a member of the American Thoracic Society. Dr. Chapman is also a Diplomate in Critical Care Medicine, Pulmonary Disease and Internal Medicine through the American Board of Internal Medicine, as well as a Diplomate with the National Board of Medical Examiners. He has published a number of articles in peer-reviewed journals on topics such as transplantation, sleep-related breathing disorders and lung disease, among many others. Dr. Chapman also received a ‘Best Doctors Award’ from 2005 to the present.
To make an appointment with Jeffrey Chapman or any of the other specialists in our Respiratory Institute at Cleveland Clinic, please call 216.444.6503 or call toll-free at 866.223.5864. You can also visit us online at www.clevelandclinic.org/pulmonary.
Cleveland_Clinic_Host: Welcome to our Online Health Chat with Dr. Jeffrey Chapman. We are happy to have him here today for our chat on Pulmonary Fibrosis. Let’s begin with the questions.
Pulmonary Fibrosis Overview
Wendy133: What is the difference between UIP and IPF??
Speaker_-_Dr__Jeffrey_Chapman: This is a good question that I even get from medical students. IPF (Idiopathic Pulmonary Fibrosis) is a clinical diagnosis of UIP (usual interstitial pneumonia) in the lungs without another cause.
UIP can occur in the lungs with systemic diseases such as rheumatoid arthritis, scleroderma or polymyositis. In these cases the patient does not have IPF but has scleroderma with secondary UIP. We think IPF and secondary UIP may have different causes and thus respond differently to experimental treatments or treatments for rheumatoid arthritis etc.
rgalvin64: I received a call today saying I have pulmonary fibrosis. I went for a chest x-ray for pneumonia. I have been sick for 10 days. Now I have an apt tomorrow and I am scared to death...am I dying in 4 years?
Speaker_-_Dr__Jeffrey_Chapman: Idiopathic pulmonary fibrosis is fortunately rare and you may not have it. However you need to see a physician who is comfortable and experienced with these patients.
kass: The life expectancy of people with IPF appears to be longer than what the doctors initial advise--3 yrs from being diagnosed--what does Cleveland Clinic estimate as the life expectancy after diagnosis?
Speaker_-_Dr__Jeffrey_Chapman: It is highly variable. On average 50% of patients will pass by 4 years from a firm diagnosis. My rule of thumb is that for patients with a firm diagnosis, not needing much supplemental oxygen with exertion, 50% will be unchanged in one year, 40% will have a decline and 10% will suffer severe decline and not survive. I have had patients not survive one year and others who stabilize for as many ~8 years.
PAC: What do you consider end stage IPF?
Speaker_-_Dr__Jeffrey_Chapman: I consider it based upon exercise tolerance and quality of life. Breathing test can vary and one person’s severe numbers may allow another to have a good quality of life.
I am very concerned about limited survival in those that are worsening quickly, significant supplemental oxygen at rest, or are too dyspneic (difficulty breathing) to move comfortably from their house.
basketlady: We already lost our loved one to this disease in 2009. I am concerned about the cause of the IPF and if it could be something that will run in the family. Should we be asking for autopsies when our PF patients pass away?
Speaker_-_Dr__Jeffrey_Chapman: IPF can run in families, 1-5% of IPF patients have a first degree relative with IPF.
I recommend all adult first degree relatives have a physical examination and if there are symptoms of cough or dyspnea to have a regular CXR and breathing tests. If the examination or either of these tests are abnormal I recommend a CT scan. Fortunately most of the time it is not IPF.
Nivlag1: I was diagnosed thru vats biopsy w IPF/UIP in May 07. I went to National Jewish for confirmation. My pulmonologist says there nothing to do but a lung transplant. I am overweight so the transplant group will not see me. My doctor does nothing because he says there is nothing to do. He checks my breathing tests every 6 months but that is all. He says I need to lose weight in order to have transplant or die. I am on oxygen 24/7 since Jan 09. Should I see another doctor or is he correct? He says I am too young for respiratory therapy which he considers for old people. If I get a bad cold or cough he puts me on antibiotics and prednisone for a little while.
Speaker_-_Dr__Jeffrey_Chapman: The physicians at National Jewish are correct that for UIP/IPF no treatment has been shown to be beneficial. I do not recommend treatment outside of a clinical trial. Several trials are now enrolling at Cleveland Clinic and elsewhere. IPFnet.org lists a selected group of centers with trials open.
I always recommend pulmonary rehabilitation to all of my patients and encourage you to go.
Weight is a very common problem for IPF patients prior to transplant. I recommend exercise at pulmonary rehabilitation and dietary counseling. Good luck and you can do it.
Jimjoejohn: Can Hydrochlorthiazide contribute to the development of interstitial lung disease? What are the best treatments for this lung disease and is today's seminar the same as interstitial lung disease. Thank you. This is very close to us as my sister is living with it right now.
Speaker_-_Dr__Jeffrey_Chapman: Hydrochlorothiazide has been reported to cause interstitial lung disease. In patients whose development of interstitial lung disease is temporally associated with hydrochlorothiazide I recommend stopping the drug and treating the hypertension with one of the many alternatives. Oral corticosteroids can be considered for patients who are acutely worsening and early in her course of hydrochlorothiazide associated interstitial lung disease. I do not recommend oral corticosteroids for persons who are stable, very slowly declining, or many years out from their hydrochlorothiazide exposure.Please note that this reaction is extremely rare, but important to consider, and that idiopathic tumor he fibrosis is much more common.
Larildsen: My father is 82 and was diagnosed with IPF 2 years ago. His father was diagnosed with IPF at Stanford U. Med center and died at age 84 or so. My grandfather was an alcoholic; my father is not (except to coca-cola!) and has been in good health getting regular exercise (golf and tennis). He did have a quintuple heart bypass 10 years ago but had no heart damage b/c they caught it very early (he has a great dr in Washington DC). He has just started on O2 for exercise only. 1st question: what can we expect the progression of the disease to look like from the time you start on some O2, understanding that e/o is different and you don't know his history?
Speaker_-_Dr__Jeffrey_Chapman: Thank you for the question. Disease progression with idiopathic pulmonary fibrosis is highly variable. And one half of the patient's we see a stair step decline with months to years of stable exercise tolerance punctuated by periods of increased cough and worsening dyspnea. The other half of the patients experience slowly declining lung function over a period of several years. Although it is very unpredictable, a patient who requires supplemental oxygen only with exertion may have several years of good quality of life.
Exercise and weight management are essential.
SPT: What are the tips to avoid IPF if grandfather and father have been diagnosed???? (both smokers – but father quit before he was 30) and my sibling and I have never smoked (but lived in air polluted cities….)
Speaker_-_Dr__Jeffrey_Chapman: At this time we'll without knowing the cause of idiopathic pulmonary fibrosis we cannot give sure fire ways to avoid idiopathic pulmonary fibrosis except to recommend good lung hygiene. When I mean by good lung hygiene is avoiding exposure to airborne particles and chemicals. Abstaining from smoking his essential as it is limiting any exposure to airborne particles. The general recommendation to avoid airborne particles comes from epidemiologic studies which demonstrate the risk of idiopathic pulmonary fibrosis is increased twofold in persons who have occupations with high dust exposures. Although it doubles the risk, the absolute risk is still low, needing in persons who have no dust exposure in the annual incidence is 40 cases per 100,000 persons, but persons with dust exposure at the increased risk is 80 per 100,000 persons per year.
Managing Symptoms of Pulmonary Fibrosis
Leannabella: One of the most annoying symptoms of pulmonary fibrosis is the cough. What are your suggestions for helping to alleviate this symptom?
Speaker_-_Dr__Jeffrey_Chapman: You are correct. The cough is terrible and almost always impact quality of life. I recommend sugar free menthol drops for frequent use. Stick to sugar free only to avoid cavities.
I also recommend Hycodan which contains a morphine-like substance and can cause somnolence (sleepiness) and nausea. I recommend the first few doses be taken when you are not going to be operating heavy machinery. If nausea develops I recommend using half dose (1/2 half teaspoon). Hycodan should be taken as needed.
For ever present cough Tussionex Pennkinetic twice daily can be used but is also a morphine like substance.
Nivlag1: Regarding cough: most describe PF with a dry cough. Mine is full of mucus. Sticky white mucus that is so hard to cough up. Especially in the morning and late at night. Any suggestions to help with the mucus?
Speaker_-_Dr__Jeffrey_Chapman: Some do have mucus. I recommend Mucinex 1200 mg twice daily. In addition in some patients I do use low dose prednisone for the cough with mucus but am explicit that the prednisone will not help the pulmonary fibrosis to these patients.
kass: I have been diagnosed with UIP 2 years ago this month. I have not developed a cough as of yet. When does the cough normally appear?
Speaker_-_Dr__Jeffrey_Chapman: A few people will not have cough. We do not know if this indicates a better prognosis. Lack of cough always makes me reconsider the diagnosis of IPF and that it may be something else, but it still can be IPF.
sheleyjw: Will pulmonary fibrosis cause/worsen acid reflux?
Speaker_-_Dr__Jeffrey_Chapman: Yes in many people pulmonary fibrosis will worsen acid reflux which may in turn worsen pulmonary fibrosis. All patients with acid reflux symptoms should be on proton pump inhibitors. Whether asymptomatic patients should be on them is not known.
Viennamom: Is there a link between sleep apnea and pulmonary fibrosis?
Speaker_-_Dr__Jeffrey_Chapman: Sleep apnea does not cause pulmonary fibrosis, but emerging research shows people with pulmonary fibrosis are at increased risk of sleep apnea compared to people with sleep apnea.
davegreg: Hello, I have been DX with PF caused by chronic hypersensitivity pneumonitis and Pulmonary Langerhans cell histiocytosis by Vats Biopsy that was sent to Dr. Leslie at the Mayo Clinic in AZ April, 2010. The ILD was first discovered by a HRCT done 10/08. My Dr.'s say I am early in the disease and my PFT's last done in 7/10 are somewhat still good, FVC 93%, FEV1 91%, FEV1/FVC 79%. PFT in 7/09 my DCLO was 89% and now as of 7/10 it is 76%. It was done at same center, on same machine, and same tech. My question is, my primary thinks it is advancing from what he can hear in my left lung and my DCLO change and says that even though my HRCT's done at the same times show no progression per the radiologist. Why would it not show up on the HRCT? My oximeter that I carry also is showing some decline when I walk. My O2 now goes down to 92-93 when before it never went below 97. I know I am not as bad as most but still scared. I am only 44, male, but I need to lose about 50 pounds. I am on to 02 yet or meds, but can see it coming. What is your opinion?
Speaker_-_Dr__Jeffrey_Chapman: Hi, You have had a very good evaluation by a very good pathologist. Essential to lessen the risk of progression is to stop smoking if you have not already done so and remove exposure from any antigens thought to be causing the hypersensitivity pneumonitis.
Assessing progression can be difficult and CT images are least sensitive. Best is assessing change in pulmonary function tests and exercise capability.
From your description you may be having slight decline. The goal is to balance the potential benefits of treating your hypersensitivity pneumonitis with medications versus observation. I recommend speaking with your local specialist or being seen at a center with ILD experience such as one of the IPF net sites. www.ipfnet.org
Sgrmy43: Is there any way to tell when that "over the cliff" time is coming? Is it like coming down with a cold?<
Speaker_-_Dr__Jeffrey_Chapman: Unfortunately we do not have a way to predict future decline. It is usually heralded by an increase in cough and sputum and worsening dyspnea on exertion. However, the symptoms sometimes may represent just a cold and the person will experience a temporary decline in exercise tolerance which resolves after the infection is healed. In all of my patients with idiopathic pulmonary fibrosis who developed worsening cough, sputum and exercise intolerance, I treat with an antibiotic in the hopes that there is a bacterial infection we can resolve and abate any lung damage.
Medications & Other Treatment Options
hanahelena: Do you have an update on the medication pirfenidone (Esbriet)?
Speaker_-_Dr__Jeffrey_Chapman: I understand it is being reviewed for approval in Europe and have no insight there. If approved in Europe I suspect another US trial (5-7 years until approval) is needed. I do not recommend travel to Japan to obtain the medication unless you live there.
nana42: I take NAC (N-Acetyl-Cysteine, the acetylated version of the sulfur amino acid, l-Cysteine) 600 mg 3 x a day, I really don't know if it is helping. what is your opinion?
Speaker_-_Dr__Jeffrey_Chapman: I do not know either and am part of the IPFnet.org to investigate this antioxidant. If you are stable I recommend continuing it, but if you are declining or having side effects, then I recommend stopping.
basketlady: Way too many doctors, nurses and even respiratory therapists are not up to date on this disease and treatment. Oxygen is a huge problem. What is being done to educate the medical teams??? Even some pulmonologists don't seem to understand. Our loved one was on prednisone for 2 years with declining pulmonary function numbers the whole time. She almost died from complications. It took a new doctor to stop the steroids and thankfully she lived 4 more years.
Speaker_-_Dr__Jeffrey_Chapman: I am glad she was able to stop the steroids and live be with you. A little is the fault of the ILD research in the distant past showing some patients may respond to steroids. When these patients were reevaluated, those that responded did not have IPF and were incorrectly diagnosed.
We now work harder at establishing a firm diagnosis before therapy is considered. Currently we teach that when a diagnosis of IPF is secure, no therapy has yet shown proven benefit.
Heggar: Are there any promising drugs in the pipeline?
Speaker_-_Dr__Jeffrey_Chapman: Currently there are a number of studies evaluating therapies which have either proven beneficial in animal models of pulmonary fibrosis or smaller trials of humans with idiopathic pulmonary fibrosis. 2 obtain more information about currently enrolling trials I suggest going to either www.ipfnet.org or www.clinicaltrials.gov. At Cleveland Clinic we are currently investigating combination therapy of azathioprine, prednisone, and N-acetylcysteine compared with N-acetylcysteine compared with placebo. We also evaluating warfarin compared with placebo and macitentan compared with placebo and ambrisentan compared with placebo.
Ellieiv: I have been diagnosed with pulmonary fibrosis for over a year. I had a bad pneumonia in Jan. Tx. with steroids Bad side effects from steroids. My pulmonary function tests were improved by June 2010. The last 2 weeks I have experienced acute shortness of breath, chest pain, joint pain, weight loss and fatigue. Internal medicine wanted to start steroids, but I have decided to wait until I see pulmonary doctor. I have never smoked or been around 2nd hand smoke. My lungs on CT scan look like I smoked for 30 years. I am 60. Do you think I should try steroids again?
Speaker_-_Dr__Jeffrey_Chapman: Although steroids have not proven beneficial in long-term therapy for idiopathic pulmonary fibrosis, their use in patients who are acutely worsening or having severe cough or increased sputum production is frequent and may be beneficial. I typically recommend those with acute worsening take a course of antibiotics and a brief course of prednisone to attenuate any inflammation that is present independent of the baseline idiopathic pulmonary fibrosis.+
Chino: My father has pulmonary fibrosis. He is on a low dose of steroids and uses oxygen when he sleeps and sporadically throughout the day while at home. He is still somewhat active but is extremely limited in what he can do physically. Do you know of any other treatments that may be beneficial? We have heard about Serrakor NK which can only be purchased online through Biomediclabs and Pivotal Health. It is a combination enzyme treatment that cleanses the body of toxins, which will cleanse the lungs as well. Our concern is that the use of this treatment may cause clots to be released, and ultimately lead to bigger problems. I have heard of Serrapeptase, a drug that is available by prescription in Germany. It is a single enzyme and may not be as strong as Serrakor NK. No clinical trials are available to show its results. Have you ever heard of this drug, and do you think this is something that he should pursue, if he can obtain it?
Speaker_-_Dr__Jeffrey_Chapman: I am sorry to hear about your father.At this time we do not have treatments which are known to be beneficial at reducing the progression of idiopathic pulmonary fibrosis. I have heard of these drugs and your are correct that no clinical trials are available to support their use. In the least it is concerning that these substances are being marketed as beneficial without data, and potentially alarming that the suppliers are using inaccurate information and anecdotal evidence to promote a substance they are not planning on studying rigorously.
kwood: I have the pulmonary fibrosis of Hermansky-Pudlak Syndrome. Although my lung function remains stable and okay, I know that I will eventually need a lung transplant. As far as my research has shown, there is not a lung transplant center within several hundred miles of where I live (in Kansas). If you were seeking a place to have a lung transplant, what questions would you ask of the transplant program? What criteria should I use when looking for a place to go? (besides the cooperation of my medical insurance?)
Speaker_-_Dr__Jeffrey_Chapman: Ideally it is best to go to a center with at a volume of at least 40 transplants per year. Each center sets its own geographic criteria. Some centers require one to wait very close to the center and stay close for several months after transplant. We require listed patients to be within 1000 nautical miles of Cleveland and to travel back to Cleveland every 3-6 weeks post transplant for the first year. One year after transplant patients may transition their care to a local physician.
kwood: What are the benefits/downsides to having a single vs. double lung transplant for someone with pulmonary fibrosis?
Speaker_-_Dr__Jeffrey_Chapman: This is one of the most discussed issues of lung transplantation for IPF. Each center is different, and there are general guidelines but each choice is patient specific.
Understanding that, for patients less than age 60 we usually offer bilateral grafts. For those above the age of 65 and without severe secondary pulmonary hypertension we offer single grafts. 60-65 years is a zone where more research is needed and the 5 year survival being similar.
JBov: Is transplant still the best solution?
Speaker_-_Dr__Jeffrey_Chapman: For very select patients it is an option but it is not for everyone with the risks and aggressive nature with frequent testing before and after transplant.
Many patients discuss the process with us and decide it is not for them. I stress it is a decision which each person must make. There is no right or wrong choice, only the patient’s choice.
olivia911: My mother has pulmonary fibrosis and is a patient at Cleveland right now being evaluated for transplant. News seems to be getting worse and worse. I would like to know how bad it has to be for the emergency transplant to be used. It was something that was explained to us by the transplant case worker, but it was not specified what was required and we are very scared?
Speaker_-_Dr__Jeffrey_Chapman: We frequently discuss patients like her and try to always do what is best for her. We never transplant an organ with issues that are known to lessen survival but do try to use as many organs as possible knowing the native lungs are not going to last much longer.
I am concerned less about the donor organs and more about how her body will react to the new medications and organ.
LEI-28: Hello, I want to know about pulmonary fibrosis and the chances of healing with research and treatment. My father has had this disease for two years. What is his chance to receive a transplant? We live in Brazil and we do not have resources here. Please help us.
Speaker_-_Dr__Jeffrey_Chapman: Hello, if by pulmonary fibrosis you mean idiopathic pulmonary fibrosis, we have no treatments to improve or stabilize lung function at this time. Experimental agents are being investigated in the hopes of stabilizing lung function.
I do not know of transplant opportunities in South America. Chile may be setting up a program. We do have a handful of patients from outside the US as transplant candidates but the travel and commitment are significant.
Kdelacour: When do you recommend a patient be evaluated for a lung transplant? PFT test results?
Speaker_-_Dr__Jeffrey_Chapman: Soon after initial diagnosis of patient should be informed about the prognosis of idiopathic pulmonary fibrosis and that transplant may be an option in a highly selected cases. I encourage referral to a lung transplant center either shortly after diagnosis, in order to establish a baseline and begin the remediation of any pretransplant issues before patients are extremely ill. Certainly any patient using any amount of supplemental oxygen showed discussed lung transplantation with her physician and if they are considering it in any way, the patient should contact a lung transplant center.
Mysticalnurse: The latest diagnosis I have is called "Lady Windermere Syndrome", or MAC. I just turned 69, but I have longevity in my family of 90 to 103 years. Would they take that into consideration for a lung transplant? I have now been told that I have mycobacterium aurum infection. Will treating this stop the fibrosis?
Speaker_-_Dr__Jeffrey_Chapman: Your situation sounds a little bit more complex than the typical patient with idiopathic pulmonary fibrosis."Lady Windermere Syndrome", or bronchiectasis, with mycobacterium colonization can lead to fibrosis and an of itself or can exist in conjunction with idiopathic pulmonary fibrosis.
Treating the mycobacterial infection may reduce the progression of the fibrosis if you're fibrosis is only from the infection and not from concomitant idiopathic pulmonary fibrosis. This important decision is usually made on a case by case basis and I recommend you see a physician who was experienced treating both of these difficult diseases.
Lung transplantation is possible for a select group of patients above 65 years of age. However Cleveland Clinic, and most other transplant centers, prefer single lung transplantation for these individuals and mycobacterium disease almost always requires bilateral lung transplantation. Patient's older than 65 with mycobacterial infection are not routinely transplanted. However, each case is assessed individually and I recommend you contact your nearest lung transplant center.
kwood: Are you aware of any resources that might have basic information about the lung transplant process in Spanish?
Speaker_-_Dr__Jeffrey_Chapman: The following sites should be helpful for you.
Research & Clinical Trials
LEI-28: How is the research area? Is there a forecast for positive results? And how long?
Speaker_-_Dr__Jeffrey_Chapman: The research is active but right now we do not know the starting cause, limiting our efforts. By positive results, I hope in 5 years we will have some therapy to slow progression and by 10 years to have significant or complete stabilization. I am very hesitant to think we can improve scar (fibrosis) or the lung function with scar.
Chino: Are there any trials going on in the Chicago area for treatment of pulmonary fibrosis?
Speaker_-_Dr__Jeffrey_Chapman: IPFnet https://www.ipfnet.org/ lists clinical trials being conducted for pulmonary fibrosis. You may conduct a search for information regarding clinical trials that are being conducted in the U.S. and other countries at http://www.clinicaltrials.gov/.
Nivlag1: I was told if you are in a clinical trial they will not consider you for transplant. Is that true?
Speaker_-_Dr__Jeffrey_Chapman: Fortunately, that is not correct. Although many trials for idiopathic pulmonary fibrosis exclude enrolling patients who are on a transplant list, all trials that I am aware of allow patient's who are currently enrolled in a trial to be evaluated, listed, and receive a lung transplant. Many of my patients who have been in clinical trials have received lung transplant while in the trial or shortly thereafter.
Chino:Do you know what treatment Jerry Lewis is on? He seems to be doing great.
Speaker_-_Dr__Jeffrey_Chapman: He is an interesting fellow in more ways than one! I have no direct knowledge or personal relationship with Jerry Lewis, and am glad he is doing well.
Nivlag1: Do you think different parts of the country are better for PF patients. i.e., living down south better than going thru winters in the north?
Speaker_-_Dr__Jeffrey_Chapman: At this point we have no data on whether climate impacts the possible progression of idiopathic pulmonary fibrosis. A recent study did note that death from idiopathic pulmonary fibrosis are more frequent during the winter in all parts of the country. My recommendation is not to avoid winter, but to avoid gatherings or exposure to persons who may have a viral infection.
Leannabella:IPF vs PF. PF has a known underlying cause and better life span. Approximately what percentage of patients truly have IPF?
Speaker_-_Dr__Jeffrey_Chapman: That is a good question about our confusing nomenclature. Idiopathic pulmonary fibrosis is a disease of unknown cause which causes progressive lung fibrosis. Pulmonary fibrosis is scar in the lung and can result from any number of illnesses such as one-time event like infection, premature birth, or inhalational injury. In addition pulmonary fibrosis can result from ongoing lung injury secondary to other diseases such as scleroderma, polymyositis, rheumatoid arthritis, and exposure to agents leading to inflammation in the lung.
Leannabella: I was diagnosed in 2003 and an ANA blood test was done and came back negative. My PCP just did another one and it came back slightly positive 1..80 tiger speckled. Do you know what that could mean?
Speaker_-_Dr__Jeffrey_Chapman: A positive ANA blood in the correct clinical setting can indicate a connective tissue disease leading to inflammation and fibrosis in the lung. This type of lung injury is treated differently than idiopathic pulmonary fibrosis. However, many patients have a positive ANA, especially at relatively low levels such as yours, and it does not change the diagnosis of idiopathic pulmonary fibrosis since it is an isolated finding and not indicative of systemic connective tissue disease.
Larildsen: Can you give me some guidelines about how an oximeter works. My dad wears one when he's walking and his numbers are all over the place...anywhere from 80-94 regardless of whether he's going up or down small rises in his neighborhood. Should we be doing something differently?
Speaker_-_Dr__Jeffrey_Chapman: An oximeter displays the amount of hemoglobin that has oxygen bound to it in your blood as well as your heart rate. I recommend patient's use supplemental oxygen if there oximetry is less than 88%. If you're father's saturation is above 88% at rest he does not need supplemental oxygen at rest. Since he appears to desaturate with exertion I recommend using supplemental oxygen when he exerts himself recognizing the oxygen will improve his exercise tolerance and quality of life.
Leannabella: What is your opinion regarding NAC?
Speaker_-_Dr__Jeffrey_Chapman: NAC N-acetylcysteine is currently an investigational drug being evaluated in an ongoing clinical trial that I am participating in. A number of centers are also performing this trial and the list can be found at http://www.ipfnet.org/. I do not recommend my patients with idiopathic pulmonary fibrosis take N-acetylcysteine since it is unproven. However, in patients who choose to take this medication I recommend using the same substance we are using and the trial which is PharmaNAC.
Hkirkwood: I know this is an incredibly big and broad question, but from where you sit, looking at the big picture for a moment, what are the biggest challenges holding us back from better treatments for PF? Is there something about the nature of the fibrosis process that makes it a particularly tough nut to crack, or is it more issues to do with funding and attention? What can we, as patients, do to most support the scientific community trying to help us?
Speaker_-_Dr__Jeffrey_Chapman: This is an excellent question. The biggest challenge is that we do not know the cause or causes of idiopathic pulmonary fibrosis, thus we do not know which systems in the body to inhibit or augment. While we used to think that idiopathic pulmonary fibrosis was an inflammatory response to either the bodies on tissue or something inhaled, recent data points us in a much different direction. We are learning that normally the lung is a dynamic organ which has to replace injured or older cells with fresh healthy cells, while maintaining normal lung structure and function. Idiopathic pulmonary fibrosis seems to represent an ending result when normal healing and injury repair goes awry. As we understand more about the normal pathway and the possible ways it can break down we understand where to target new therapies.
Cleveland_Clinic_Host: I'm sorry to say that our time with Dr. Jeffrey Chapman is now over. Thank you again Dr. Chapman for taking the time to answer our questions about Pulmonary Fibrosis.
Speaker_-_Dr__Jeffrey_Chapman: Thank you for all of your questions.
Leannabella: Thank you so much for your time. This was very informative.
basketlady: Thank you for answering questions.
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