Tests interval

[Donald Salzberg MD]

Hi Chris:

So sorry you have this lovely disease. I also initially presented (at age 63) with what I thought was an asthmatic attack (never had that before). No cigs. Mild PF was seen on XRay and HDCT confirmed it. I wanted to have a lung biopsy done as at first I was going to be treated for HP (hypersensitivity pneumonitis)(Prednisone). My biopsy proved IPF and have been on OFEV. Prednisone would have been the inappropriate therapy for me. I get PFTs every 6 months (4 months if it got worse)!!  In 4.5 years I’ve had a total of 2 CT scans as it doesn’t add as much as the PFTs do. Are you on any anti-fibrotics? From everything I’ve read, the patients who truly have IPF will benefit the most with early diagnosis and treated with APPROVED antifibrotics (OFEV or Esbriet)!! I’ve been relatively stable for 5 years but I’m b excited about the new trial of inhaled Esbriet that’s in early Phase 2!!  Less side effects. Also read aspirin potentially helps reduce fibrosis (if you can tolerate it)!!
Don S

[Karen Martin]

Inhaled Esbriet?  Really?!  I hadn’t heard of that.  I am using the inhaled version of TyvasoDPI for PAH, which is much less hassle than the nebulizer form.  Lots less waste, too.  Guess I need to do some research on the Esbriet.  Thanks for bringing this up.

[Mary Frances]

My mother Emily was diagnosed in 2018 with IPF. We had here on bp meds and started her on 10 mg of prednisone. It worked wonders. Then they wanted her to try Tyveso and Ofev. She cannot tolerate the Ofev and stopped. I read Ofev takes 6- 12 weeks to really get inside of her body. At the 6 week mark they added Tyveso and inhaler 4 times a day. She used that for 10 days (Ofev 6weeks Tyveso 10 days) When the Tyveso was added she was extremely ill with upset stomach. She then had a bp reading of 200/101. We were at the ER twice. They were not familiar with these medications did not know how to treat her and sent her home after being bp way down. Now she is not in anything but Almodapine 2.5 mg a day. Her numbers are good and she uses 4 liters of oxygen at night and 8 during the day. They weaned her off the prednisone. Now she has no energy at all. She has headache’s on the right side of her head and her stomach has been hurting. We were told to call hospice. I am upset b/c as I posted before the prednisone and her bp meds had her able to have quality of life. We have one more appointment to hopefully have a CT to see if her disease has progressed so badly or did the over dose of the Ofev and Tyveso make her so sick. Any thoughts would be greatly appreciated. My prayers are with all of you!

[Adele Friedman]

He may be trying to assess how much, if any, your fibrosis progresses in six months. That’s something that could be valid initially, but HRCTs are not routinely done at specific time intervals indefinitely. It is pretty routine to have PFTs and walk tests every six months, maybe more often in some cases. CTs and HRCTs are noninvasive, but involve a fair amount of radiation compared to other “scanning” procedures, so it’s generally wise to do them only when there’s a solid clinical indication. For example, dramatic worsening of condition.

[Stephen B. Strum, MD]

I concur with Adele.

Stephen

[Stephen B. Strum, MD]

Testing of patients for any illness is dependent on the gravity of the illness at baseline, the history the patient relates to the MD, to follow-up of a new treatment after baseline testing.

Per your story, I would think that something as noninvasive as pulmonary function tests (PFTs) and a six-minute walk test (6MWT) would be a so-called “no-brainer” to repeat in 4 months, no less 6 months.  Call me an old old fashioned MD but that’s OK with me.

The holy Grail of all of medicine and biology to boot is….

Status begets Strategy (SBS)

If you do not know the patient’s status then everyone is flying blind.  Maybe I am just a dumb old physician that never learned anything after 60 years of cancer medicine, but that’s how I see it.

[Adele Friedman]

Thank you, Dr. Strum. I’m glad I was on track.

[anonymous]

all very helpful, thank you!

[Karen Martin]

I can understand not wanting to repeat too often tests that expose a person to radiation unnecessarily.  Still, I would think that the doctor should discuss the plan with the patient.  I have felt as if perhaps it’s just a matter of knowing this will not get any better, so why bother?!  Not the best feeling and I have struggled with it.  I have an appointment with my doc this coming week and will be asking for more  information about his approach to future testing and what I need to be on alert for as time passes.

[anonymous]

This is a serious problem, but there IS hope. I am hoping these new drugs being researched can stop, and even reverse the scarring (that has been achieved in mice, so it is apparently biologically possible). Also, as a last resort for some patients, a donor lung can also be an option.

My doc told me “there is research on fibrosis” I googled that and he is right.  Of course, some of us might not make it long enough to benefit from that research, as we could get hit by a bus, fall off our bike and get a head injury, have a heart attack, get abducted by a UFO, or anything else!  Personally, I am going to try to avoid those other issues and keep faith that this substantial ongoing research will yield very good treatments. I counted something like 20 drugs being studied!  One anti-fibrotic (BI1015550) worked so well in Phase 2 trials (almost completely halted progression in most participants and was well tolerated) that the FDA gave it “breakthrough designation” and Phase 3 trial ends at the end of next year.  I dont know what the timeline is after that even IF it works as well in Ph3, to when it hits the market, but that, and all the other research and drugs being tested is certainly a reason for hope that the status quo can only get better.

 

 

[Margaret]

CTs are done more frequently if there is a reason they are needed. My doctor is monitoring several slowly growing pulmonary nodules so a CT is done every 3 to 6 months. My last one showed returning infiltrates before I had any worsening symptoms so I went back on oral prednisone for a while.

Mary Frances – My PF is radiation induced (breast cancer) and steroids are the treatment for it as they are for PF caused by hypersensitivity. They are used for IPF exacerbations but don’t stop that type of fibrosis. High dose prednisone causes serious side effects including high blood sugar, osteoporosis, weight gain, glaucoma, high blood pressure and ulcers. Hopefully I will be able to get back on inhaled corticosteroids soon since those have fewer side effects.

OFEV is approved for RIPF if steroids stop working but I haven’t heard of anyone in this situation having success with it. That might just be because progressive RIPF is pretty rare.

[Stephen B. Strum, MD]

Margaret and Mary Francis,

I am pasting part of your post below in order to answer in context.

Mary Frances – My PF is radiation induced (breast cancer) and steroids are the treatment for it as they are for PF caused by hypersensitivity. They are used for IPF exacerbations but don’t stop that type of fibrosis. High dose prednisone causes serious side effects including high blood sugar, osteoporosis, weight gain, glaucoma, high blood pressure and ulcers. Hopefully I will be able to get back on inhaled corticosteroids soon since those have fewer side effects.

ss: I do not know the level of expertise of forum members to search for leads relating to articles that are pertinent to their individual situation.  I just did a PubMed search on “radiation-induced” pulmonary fibrosis and have 110 publications from the peer-reviewed literature. These span publication years from 2023 to 1966.  I am pasting some titles in below.

2023:
<p class=”p1″>Rimner, A.; Moore, Z.R.; Lobaugh, S.; et al. Randomized, phase II, placebo-controlled trial of nintedanib for the treatment of radiation pneumonitis.</p>
<p class=”p1″>Prasanna, P.G.S.; Aryankalayil, M.; Citrin, D.E.; et al. Radiation-induced pulmonary fibrosis: roles of therapy-induced senescence and microRNAs.</p>
2022:
<p class=”p1″>Wang, Z.; Chen, J.; Su, L.; et al. Downregulation of miR-761 ameliorates radiation-induced pulmonary fibrosis by regulating PGC-1alpha</p>
<p class=”p1″>Wang, P.; Yan, Z.; Zhou, P.K.; et al. The Promising Therapeutic Approaches for Radiation-Induced Pulmonary Fibrosis: Targeting Radiation-Induced Mesenchymal Transition of Alveolar Type II Epithelial Cells.</p>
<p class=”p1″>Targeting USP11 may alleviate radiation-induced pulmonary fibrosis by regulating endothelium tight junction</p>
<p class=”p1″>u, X.; Li, T.; Yao, Q. The Effect of Ophiopogonin C in Ameliorating Radiation-Induced Pulmonary Fibrosis in C57BL/6 Mice: An Update Study.</p>
2021:
<p class=”p1″>Mesenchymal stromal cells can repair radiation-induced pulmonary fibrosis via a DKK-1-mediated Wnt/beta-catenin pathway</p>
<p class=”p1″>Nam<span class=”s1″>✸</span>, J.K.; Kim, A.R.; Choi, S.H.; et al. Pharmacologic Inhibition of HIF-1alpha Attenuates Radiation-Induced Pulmonary Fibrosis in a Preclinical Image Guided Radiation Therapy.</p>
<p class=”p1″>Targeting senescence-like fibroblasts radiosensitizes non-small cell lung cancer and reduces radiation-induced pulmonary fibrosis</p>
<p class=”p1″>Luo, M.; Chen, L.; Zheng, J.; et al. Mitigation of radiation-induced pulmonary fibrosis by small-molecule dye IR-780</p>
<p class=”p1″>Elkiki, S.M.; Mansour, H.H.; Anis, L.M.; et al. Evaluation of aromatase inhibitor on radiation induced pulmonary fibrosis via TGF- beta/Smad 3 and TGF- beta/PDGF pathways in rats.</p>
2020:
<p class=”p1″>Park, S.H.; Kim, J.Y.; Kim, J.M.; et al. PM014 attenuates radiation-induced pulmonary fibrosis via regulating NF-kB and TGF-b1/NOX4 pathways.</p>
<p class=”p1″>Li, W.; Lu, L.; Liu, B.; et al. Effects of phycocyanin on pulmonary and gut microbiota in a radiation-induced pulmonary fibrosis model.</p>
<p class=”p1″>Kim, H.; Park, S.H.; Han, S.Y.; et al. LXA(4)-FPR2 signaling regulates radiation-induced pulmonary fibrosis via crosstalk with TGF-beta/Smad signaling.</p>
<p class=”p1″>Johnson, S.; Shaikh, S.B.; Muneesa, F.; et al. Radiation induced apoptosis and pulmonary fibrosis: curcumin an effective intervention?</p>
<p class=”p1″>Guo, K.; Chen, J.; Chen, Z.; et al. Triptolide alleviates radiation-induced pulmonary fibrosis via inhibiting IKKbeta stimulated LOX production</p>
I have also found an average of 10 articles on treatment of interstitial lung disease (ILD) with agents such as carnosine, carnitine, Gossypol, DMSO, zinc and assortment of other agents.

I have found a total of 55 articles on T. chebula, the key herb in Haritaki. In T. chebula are two major tannins and two major polyphenols.

In my opinion, having done a fair share of cancer research involving innovative therapies, I do not see why IPF/ILD/RIPF/etc should be any different.  The agents are out there waiting to be studied.  It seems to me that the incentive for many academic institutions comes from the funding by big pharmaceutical companies.  The very same companies have little interest in natural products unless they can alter them and patent them and sell that for massively inflated prices (especially in the US).

And we do not need thousand patients in various arms of the study.  A pilot study involving 20-30 patients will often disclose highly active agents.  We don’t need statistics to talk about a 50% increase in survival vs. controls if that survival amounts to six weeks.

Lastly, I speak as a cancer patient with a rare disease, as rare if not rarer than IPF. The drug that put me in a complete remission (CR) came from my pushing my docs to “allow” me to use this med after I was tredding water x 2.5 years and having a lousy quality of life.

 

 

OFEV is approved for RIPF if steroids stop working but I haven’t heard of anyone in this situation having success with it. That might just be because progressive RIPF is pretty rare.

[Stephen B. Strum, MD]

This reply is written with hope that patients will work together to improve the lot of all patients.  Fundamental principles in medicine have been forgotten.  Examples are

1. The requirement to obtain BASELINE studies before initiating a new treatment.

2. The need to have every patient’s medications & supplements checked for drug-supplement interactions. See http://www.medscape.com/druginfo/druginterchecker?src=google

3. The need for repeat studies of whatever baseline markers the physician-patient team are employing to monitor the success or failure of treatment.

I am consulting with a man with IPF who was started on pirfenidone in Sept 22 and his baseline PFTs and six-minute walk test (6MWT) were done 4 months earlier. It is now April 2023 (7 months later) and this patient informs me he is not doing well and yet no follow-up PFTs and 6MWT have been done.

This is not the way medicine is supposed to be practiced.  Perhaps the patient is a cause in this and has declined follow-up visits. I have reviewed all medical records from the patient portal and there is no suggestion of this but the patient portal fails in that it does not include consultation, office notes, hospital admission and discharge summary and other records revealing cognition.  This is why I refer to the portal as the dummied-down patient version (DDPV) of the medical record.  I will obtain the official medical records on this man but first I am waiting to see if he at least complies by calling and firmly asking  to have his pulmonary function tests (PFTs) repeated.

Lastly, with a disease such as IPF where the two leading treatments have only shown stabilization of disease as the best outcome, you may conclude why bother to do interval testing.  Well, it’s the obligation of the physician to seek other treatments that may be of value especially if the patient is showing decline or worsening in their quality of life.  The approach a good MD (medical detective) takes is “seek and ye shall find.”

 

[Gavin John Powers]

I’ve ordered some haritaki online. I’m dearly hoping it makes some kind of difference before it’s too late.

[Margaret]

Dr. Strum – Thank you for your reply. I have a math/science background and am comfortable reading scientific papers even though I occasionally have to look up the definition of a medical term. While limited lung fibrosis from radiation is not unheard of the progressive “recall” RIPF is rare (for breast cancer 3/1000 radiation patients.) Not surprisingly it is more common in lung cancer patients but they often don’t have the survival expectation of bc /Hodgkins patients. There are fewer than 10 of us who post on this website and besides me one poster on breastcancer.org whose fibrosis stayed within her portal.

My pulmonologist is part of an 8 doctor ILD team who generally treats the autoimmune, hypersensitive and other miscellaneous patients. He always tells me that mouse lungs are not human lungs but listens to ideas I have read about. He has prescribed high dose inhaled corticosteroids to help control my severe cough (lung distortion from fibrosis exposes nerves to air.) I suggested this after reading about proposed clinical trials in Mexico and China which never happened due to lack of enrollment that postulated inhaled steroids would prevent progression. Hopefully they will work for this and at any rate my cough is much improved. US clinical trials for ILD generally exclude cancer patients and cancer trials exclude ILD patients so it’s a catch-22 situation.

Once again, thanks for taking time out of your busy schedule to give us your thoughts.

[Adele Friedman]

Thank you, Dr. Strum, good info. And thanks to everyone participating on this thread. Of course, if there are nodules in the lungs that seem suspicious, one may have to have CTs more often.

[Brenda]

I hope you all are pushing through this disease. It is exhausting. I’m replying to test intervals. I was suspected of having IPF in 2020 by my family doctor after having shortness of breath. My lungs showed up a little behind my heart which was being tested.  With Covid and delayed pulmonary testing, I officially was diagnosed July 2021. I have a team of 6 doctors. I have a CT scan and the breathing and the 6 minute walk every 3 months. I go in for blood once a month and have a urine test about every 2 months. I have now developed a terrible sinus infection with thrush in the nose. I’ve been seeing the ENT for 2 months every 2 weeks. I had 3 biopsies of my left nostril (nothing major) and then a culture test that I don’t have results for, yet. My ability to make all my appointments is getting to be a lot. I’m blessed to have a son that usually takes me. My grandkids have also pitched in. The walk to the pulmonary area is too long for me. I am on Ofev, SMZ/TMP DS (antibiotic) and Mycophenolic. My night oxygen is 2 and 3 or 4 when I’m up and about. My cough is also bad. I’m on a cough syrup with codeine. It has helped a lot. The good news is I still clean my house and do some grocery shopping. I have to rest but I feel better about myself doing things. Soon, I’ll be out in the flower gardens. Take care all.

[Steve D.]

Question – Does anyone know of a single location that lists and regularly updates the status of all clinical trials taking place for IPF? Using Google provides way to many false positives and dated information. This is certainly a service I would subscribe to if available (assuming it’s reasonably priced)!!  Any inputs will be appreciated!!

[Lee]

Rather than wait on pulmonary tests, during the interim periods you can track your own PFTs (with the exception of DLCO which requires a $100,000 machine!). I use a $30 digital spirometer called a Peak Flow Meter that I purchased on Amazon. It measures FEV1 and PEF which are two indicators of lung volume and function.
I tend to battle IPF rather aggressively, and I do daily strenuous exercise and walking plus daily breathing exercises to improve my lung function. About every two weeks I conduct my own PFT and record the date in a log.
Over the last year I have recorded a 50% improvement in lung function that my physician was shocked by considering my diagnosis of “end stage” IPF. My own PFT log gives me more real-time feedback on what is working.

[anonymous]

Lee that sounds awesome. In all my “research” it seems while fibrosis decides for itself who stays mild for years and who progresses. It really varies. But lot of patient stories that includes what you doing, it really seems to make a life quality difference and I would think longevity too. Also, there’s lot of research going on into PF. My pulmo told me that and he was right. So, for me being a very mild stage, hoping for even one of these new treatments to work well and come onto the market. If not, there’s transplant if it gets to that point. Those are good reasons to make good choices like what you doing and in my case I protect my lungs with N95 masks when around ANY dust etc, hepa filters running in the house, and am taking lung health supplements (which may or may not help, but more likely to help than not I think).

[Kim Morrison]

My Dr. orders a CT Scan every year along with pulmonary function test. Also labs to make sure the medication is not affecting my liver.

[Gina Myhill-Jones]

Chris… could you perchance have had Covid? Even mild cases are showing some fibrosis. They don’t know yet if it will self resolve, but there’s hope it might.
Other tests to identify the culprit can be really helpful. Pulmonary fibrosis happens for about 200 reasons and not all are as bleak a prognosis as IPF.
Wishing you all the best as you navigate your investigations, here’s hoping for a simple and manageable finding!
Ps… patience is always the tough part of awaiting a solid diagnosis ! Everyone here can speak to that! We’re all in this together !

[anonymous]

Dont think I had covid before this, and was vaxxed literally from day 1. But I did get covid last January, thankfully was given paxlovid and it was mild.  Always for decades had an issue with dry cough periods (sometimes lasting months) which they called reactive airway disease which is kind of like a light athsma. Then last year started with the SOB, diaphragm felt ‘tite’, came out of nowhere and I didnt think much of it “out of shape, or maybe a little fat”, until my GP sent me for xray and PF was seen on that, then confirmed on CT, and PFT was showing slightly low DLCO. All blood work for the known causes that can be shown that way were negative.  But I would jump for joy if this were “not a bad PF”.  Seems like the only way to know is to wait.   🙁

[Donna]

Hi,

I was originally diagnosed PF Early Stage 5 years ago. I have had COVID twice the second time last year and had Pneumonia caught COVID for the  second time caused more lung damage. I had the hardest time getting over Pneumonia then the Cough came. I switched Pulmonary Dr.’s like starting everything all over again not easy to do. He was so much more thorough and detailed than the first Dr. So glad I did. It is a very busy place hard to get appointments. I have been on Esbriet not a good experience after that decided not to do OFEV. Dr. was OK for now since I was pretty stable my last appointment. Hopefully stays that way.