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    • #21790
      Mark Koziol
      Keymaster

      I recently wrote a column for Pulmonary Fibrosis News and was afforded the opportunity to interview a pulmonologist with the Cleveland Clinic. The column is titled; Q&A With a Pulmonologist: What IPF Patients Want to Know. Dr. Brian Southern is a celebrated researcher in the pulmonary fibrosis world. Since there is a word restriction for columns, the entire interview was not included in my latest column, however, I have included it in its entirety as a forum topic below. I encourage you to read Dr. Southern’s bio as well; he is a patient doctor who values the rapport he creates with his patients.

      Here are his responses, verbatim to the questions I asked in our interview:

      You have many job duties at the clinic. How do you juggle work and home life? Sometimes it is difficult. I am always juggling family, clinical responsibilities, and research. Often I cannot put 100% effort into all three and one must suffer. I try to prioritize family above all.

      How do the diagnostic procedures differ between PF and IPF? Pulmonary fibrosis can have many causes. Our main mission is to determine whether the patient has IPF or one of the other causes of pulmonary fibrosis. This has big implications for prognosis, planning, and treatment. If we suspect an autoimmune problem that is causing the fibrosis, we may do a series of blood tests to try to make a diagnosis. If we suspect hypersensitivity pneumonitis or infection we may perform a bronchoscopy. A diagnosis of IPF can often be made by identifying certain features on the CT scan and excluding the other major causes of pulmonary fibrosis. Occasionally we recommend a lung biopsy for a definitive diagnosis.

      Can you explain the pathobiology of IPF in layman’s terms? The current understanding is that people who develop IPF have an intrinsic “genetic” defect in the way their lung epithelial cells respond to injury. Epithelial cells line the air sacs in the lungs and frequently experience “micro injuries” from things like exposure to smoke, dust, viruses, or acid reflux. In most people there is an efficient repair program that occurs on a daily basis to maintain the lining of the lung, one that we are not even aware of. In people with IPF, this program sends some incorrect signals which result in formation of a tiny scar, instead of normal repair. Over time these small scars start to accumulate and stiffen the lung. This further activates the scar producing cells, fibroblasts, to try and repair the damage, but this actually creates a vicious cycle. As the lung gets stiffer, the fibroblasts make more scarring, which makes the lung even stiffer and less able to function normally. This is how progression of fibrosis is thought to occur in IPF.

      In your May 2018 research article; what primary care physicians need to know? Why is it so difficult for some PCP’s to identify a specific ILD such as IPF? These doctors have all went through accredited institutions and received formal training but when faced with a patient who exhibits these symptoms they fail the patient either by a failure to diagnose or misdiagnose, resulting in delayed treatment. How can the medical community rectify this situation? Unfortunately, this continues to be a problem we encounter. It is mostly because of the way the health care system is set up in this country. Most primary care physicians and pulmonologists in the community are really good at treating common pulmonary illnesses like COPD or asthma. They are sometimes seeing 20-25 patients per day and have to try to make a diagnosis and treatment plan, often within a span of 15 minutes. Common things being common, a lot of pulmonary fibrosis patients are misdiagnosed with COPD or asthma. Only when the patient or doctor realizes that they are not responding to treatment do they seek the advice of a specialist in pulmonary fibrosis. One of our missions is to help educate and make it easier for community physicians to refer patients to pulmonary fibrosis specialists much earlier.

      How can the medical community help the doctor working in rural Kansas or Appalachia identify symptoms of ILD and specifically IPF? Adding to this I am fortunate to live near excellent hospitals who are actively involved with the research of IPF and pulmonary diseases. I co-moderate a forum and feel badly for some members after hearing of their experiences. I feel sort of guilty of the experience I had and am having at the clinic compared to many of the forum members. Many of our physicians give talks in rural communities on how to suspect pulmonary fibrosis and refer patients earlier to a specialist. A lot of this work is also being supported by the pharmaceutical companies. Educating physicians in rural areas on how to suspect pulmonary fibrosis and refer to a specialist early is key.

      In single lung transplant patients, why is it necessary for persons be removed from OFEV or Esbriet while they are taking anti-rejection drugs and other meds to facilitate their survival? There is still debate about this in the IPF community. There have been multiple fairly large studies showing that continuing anti-fibrotics after double lung transplant does not result in worse outcomes. Theoretically, both pirfenidone and nintedanib may impair post-operative wound healing or cause problems with the engrafted lung. Nintedanib, because of its effect on vascular endothelial growth factor (VEGF), may also increase perioperative bleeding risk. For these reasons, some transplant physicians are hesitant to continue anti-fibrotics after lung transplant.

      What is the biggest obstacle in finding a cure for the fibrosis? Where is most of the research geared to; preventative or reversal of fibrosis? I think one the biggest obstacles is that we do not have reliable animal models in which to test therapeutics. There have been numerous successful studies using the bleomycin model of mouse fibrosis, which is the most commonly used model, but these have not translated into effective drugs. There are currently efforts underway to create animal models that more accurately represent human pulmonary fibrosis, and some of the preliminary work is very exciting. I think research in pulmonary fibrosis spans the spectrum. Many researchers are working on the initiation phase of fibrosis, a number of researchers including myself are working on ways to halt the progression of the disease, and there are quite a few groups trying to develop a therapy that reverses fibrosis.

      Are you currently working on a research publication the PF/IPF can get excited about? Our lab is currently working on several avenues of research. We have identified several critical molecules that are involved in how fibroblasts in the IPF lung sense lung stiffness and react to that. We believe if these molecules can be targeted safely and effectively, this would halt the progression of fibrosis.

      To our valued forum readers: What are your thoughts on this interview? I am curious to hear!

    • #23125
      Sara Tanner
      Participant

      Thank you so much!! IPF is new to us &  this interview answered and/or clarified many of our concerns & questions.

    • #23126
      Mark Koziol
      Keymaster

      Thank you for being the first to respond. Dr. Southern is an amazing doctor and researcher who has devoted his life to treat pulmonary disease patients and find a cure for pf. Take care, Mark

    • #23130
      Denny
      Participant

      Brian Southern is my Cleveland Clinic doctor. He diagnosed my usual interstitial pneumonitis by the scarring pattern on the CT and his panel was unanimous about NOT having a biopsy which would have ended my passion of playing hockey. I really like him.

    • #23131
      Mark Koziol
      Keymaster

      Thanks Denny, he is easy to like and down to earth which I feel is a great characteristic for a physician. I am also at the clinic but now I see only the transplant team of pulmonologists. Thanks, Mark

    • #23516
      Miltont
      Participant

      @mark-koziol

      Well done, Mark. I woould like to add some explanation of the scarring problem and also an alert to a new drug to slow progress. I have been studying and treating scarring since 2005 with 100%. What happens and why exercise has to be treated cautiously is this. When the body sustains an injury, it takes some Collagen – the most abundant protein in the body – out of either its liquid or plastic form and makes microscopic solid fibres and weaves a mat through the injury site. This holds it together while it heals. The Collagen fibres have a tensile strength of about 2000 times that of steel, so are effectively inelastic. When you exercise and stretch the normal tissue, the Collagen will not move and so further tearing of normal tissue occurs and the scar length increases. This is what causes athletes to retire with chronic muscle tearing.

      On the second note there is a new drug soon to go on a World trial. Volunteers are being sought at the moment. It has so far only been tested on less than 150 IPF sufferers. Reportedly it had zero side effects and was better at slowing the progress than the exising drugs. I was asked to volunteer for the trial but because I had very recent bladder cancer I was rejected. Bugger.

    • #23537
      Dave Sabatelli
      Participant

      Thank you good interview, I am sharing with my family.  I am approaching my one year anniversary of being diagnosed with IPF  also one year on  Esbriet. I need to learn everything I can about IPF .

      Thanks

      Dave

    • #23541
      Mark Koziol
      Keymaster

      Hello Dave, I appreciate your feedback. Dr. Southern is an extraordinary doctor and researcher. He is pursuing his passion in life.  Peruse the the topics a little each day and I think you will find suggestions and information you will find helpful. Take care, Mark.

    • #23542
      George Manolakis
      Participant

      Mark,

      Thank you for an excellent interview. For the first time I have a logical explanation of how IPF develops. It took slightly more than a year to diagnose the cause for shortness of breath by my cardiologist and PCP altho I was diagnosed with AFIB and had ablation during that period. Fortunately, my scarring appears more in the center of the lungs and has not had much, if any, progression over the 2 years since diagnosis. My PFTs are good. I would be open to being included in a clinical study if being a active 85 is not a disqualifier.

    • #23543
      Mark Koziol
      Keymaster

      Hello George, thank you. I am always happy when a forum member can get valuable information from a topic. I’m glad you have remained stable. I am not sure if there are age limitations on clinical trials. If you need help searching for a trial page, please get back to me. Thanks Mark

    • #23544
      Mark Koziol
      Keymaster

      Hello Miltont, thank you. I appreciate the information you have supplied. If you find out the name of the trial please notify the forum. I apologize for being late to respond to your post. Take care and stay active, Mark.

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