[Doug Jones]

@yoli

The two most common side effects of Esbriet are 1) increased photosensitivity, i.e. more likely to sunburn and 2) gastrointestinal issues including nausea and diarrhea.  Most GI symptoms go away within the the first 3 months as your body adjusts to taking the medicine.  During that time (and afterwards) it is suggested that you take the Esbriet with food which usually reduces symptoms.  Also, your doctor may temporarily reduce the dosage which may help during the introduction phase.  I also try to space out my doses taking them as close to 8 hours apart as possible.  For more information from the manufacturer see https://www.esbriet.com/taking-esbriet/managing-certain-side-effects.html?c=esb-163fb797758&gclid=Cj0KCQjw8IaGBhCHARIsAGIRRYrv9msK4kvrtMjoFP2Jm2vPwXdao1vJ0sKE-5y2BX8nqGC8osZV8icaAisFEALw_wcB&gclsrc=aw.ds

I have been taking Esbriet (pirfenidone) for over 6 years, since it was first approved by the US Food and Drug Administration.  I have been taking the full, normal dose of 801 mg three times per day.  I live in Tucson Arizona where the potential for sunburn is high so I found a hand/face lotion with SPF 15 that I apply daily to my face and arms.  I wear a hat and–if I am going to be out in the sun a long time–a long-sleeved shirt.  I also have long-sleeved beach wear made of comfortable sun-protective cloth that I wear if I go swimming or to the beach.  My fibrosis has continued but at a lower pace and I have been essentially stable for the past 2-3 years.

The alternative medication to Esbriet is OFEV which apparently has similar therapeutic results although it works differently.  There is a a much greater percentage of patients taking OFEV who have GI issues, however.

[Doug Jones]

Hi Kate–this is a good topic, especially as many of us with reduced lung capacity begin to travel and fly again.  I have been researching this for some time and suggest the following authoritative resources:

1)  The Pulmonary Fibrosis Foundation has a an informational section on Oxygen Therapy that discusses traveling with oxygen.  See https://www.pulmonaryfibrosis.org/life-with-pf/oxygen-therapy  PFF also operates an Oxygen Therapy Information Line 844-825-5733 which you can contact.

2)  Most commercial airlines pressurize their cabins with oxygen levels comparable to 6000-8000 ft altitude.   Depending on what altitude you currently live at this could be a drop of about 25% oxygen from approximately 20% at sea level to approximately 15% at 8000 ft.  https://hypoxico.com/altitude-to-oxygen-chart/

3)  The Univ of Az Center on Aging has a brochure titled “Fit to Fly” that discusses flying for individuals with limited lung function.  See  https://www.uofazcenteronaging.com/care-sheet/providers/fit-fly-older-adults-and-air-travel#:~:text=Oxygen%20Pressures%20During%20Air%20Travel,21%25%20found%20at%20sea%20level.

4)  Most Portable Oxygen Concentrators (POCs) are accepted by major airlines for in-flight use.  When you get your POC,  double check the make with the airline(s) you will be flying to confirm.  Also, you should notify the airline when you schedule your flight that you plan to use your POC in flight because most want a document from your doctor certifying your need for oxygen.  Also, because the POC and cannula cannot be stowed in the same way other carry-ons are, you may be seated in a window seat so that in an emergency others don’t trip over your cannula.   All commercial airlines have a special unit that deals with “disabled” passengers.  Look for it on the website or ask the airline customer service agent.  Also, a POC is NOT counted as part of your carry-on luggage as it is a medical device.

5)  Lithium-ion batteries–the most common type of battery for POCs–are allowed as part of your carry-on–at least in the US–but NOT in checked baggage.  See the FAA guidelines at https://www.faa.gov/hazmat/packsafe/more_info/?hazmat=7   Most airlines want you to have sufficient battery capacity to last 150%  of the length of your flight. This may take some pre-flight testing on your part to see how long a battery will last at the rate you will use on the flight.  I assume margin of safety is because of potential delays extending the expected flight time.

Good Luck and Happy Travels.  It sounds more complicated that it is but ….

Doug Jones

[Doug Jones]

Hi Steve–sorry you are having problems with adverse effects from the 2 authorized IPF drugs.  I have been taking pirfenidone (Esbriet) since November 2014 when FDA first authorized its use in the US.  I am fortunate that I have not had any significant side effects.  One of the major medical databases–UpToDate– lists percentage of potential side effects from the manufacturer studies.  About 26% experience fatigue and up to 36% experience nausea.  For many, these effects seem to lessen after about 3 months.  Other suggestions–already noted–are taking a reduced dose and taking with food.  I’ve copied the info below.

You may also want to take a look at some of the medical literature about pirfenidone and nausea which reflects more recent real-world clinical experience. You can search in PubMed  https://pubmed.ncbi.nlm.nih.gov/  for free.   For example,  “Pirfenidone safety and adverse event management in idiopathic pulmonary fibrosis” European Respiratory Review  suggests improvement in adverse effects after 6 months.

Since Ofev and Esbriet are currently the only drugs currently authorized for treatment of IPF in the US, you may have to choose between quality of life and length of life–not an easy choice.  Hope you are able to better tolerate Esbriet soon–or find a regime that works for you.  Best wishes.  Doug

The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified. Central nervous system: Fatigue (26%), headache (22%), dizziness (18%)
Gastrointestinal: Nausea (36%), diarrhea (26%), abdominal pain (24%), dyspepsia (19%), anorexia (13%), vomiting (13%), gastroesophageal reflux disease (11%)
• GI effects: Adverse effects including nausea/vomiting, diarrhea, dyspepsia, gastroesophageal reflux disease, and abdominal pain have been reported; incidence may be reduced by administering with food. The incidence of gastrointestinal events was highest early in the course of treatment (initial 3 months) and decreased over time.>

[Doug Jones]

Just a few comments about POCs and some reliable sources of information:

1)  The Pulmonary Paper organization prepares an annual comparison of oxygen concentrators with detailed specifications from many companies. Latest is 2019.  Included are both continuous flow and pulse flow models as well as models such as the Simply/Go that Charlene mentions which will do both–although not at the same level.  https://www.pulmonarypaper.org/portable-oxygen-concentrators-comparison-chart-2019/

2)  Suggest you visit the Pulmonary Fibrosis Foundation (PFF) website, especially the section that deals with Oxygen Therapy which includes a wealth of reliable information about needs, options, requirements and guidelines  https://www.pulmonaryfibrosis.org/life-with-pf/oxygen-therapy  Also, the PFF has a FREE professional Oxygen Therapy Information Line 844-825-5733 which can provide individualized information.

3)  Be aware that the FLOW RATES on oxygen concentrators are NOT equal to liters/minute.  Normal air is about 80/20 nitrogen/oxygen.  Concentrators remove most of the nitrogen so the output varies from approximately 88%-96% pure oxygen.  If your doctor says you need 5 liters/min of oxygen, a POC operating at 90%  will provide only 4.5 liters/min.

4)  I have a large oxygen concentrator from Inogen–about the size of a carry-on suitcase–that weighs 18 lbs and provides  CONTINUOUS flow up to approximately 4 liters/min which I use for sleeping with my CPAP machine.  I have an Inogen One G3 that provides PULSED FLOW up to approximately 4 liters/min that I use for walking, exercise, etc It is FAA approved. Those were provided on a rental basis through Medicare.  I also purchased a SimplyGo portable oxygen concentrator that I use for travel because it provides up to 2 liters/min CONTINUOUS flow oxygen that I use with my CPAP on trips.  That level may be too low for some but your sleep oxygen requirements may be lower than waking so for me it still works.

5) If you plan to travel (post-COVID 19) there are companies and cruise lines that specialize in helping those of us who need oxygen therapy when we are away from our home resources.

There is much to know about oxygen therapy and I suggest you read the online brochures on the PFF website for more detailed and authoritative information.  Good luck–and join the club.  Doug Jones

[Doug Jones]

Hi Jackie–Sorry to hear about your IPF diagnosis but welcome to the club!  I have had episodes of coughing over my 5 years with this disease.  For me, coughing comes and goes–but you may never have this symptom.  Is your doctor prescribing anything for the GERD?  GERD is apparently common in as many as 80% of pulmonary fibrosis patients.

There are two FDA drugs approved to help manage IPF–Ofev and Esbriet–which apparently have similar outcomes.  If Ofev becomes too challenging to manage, you might want to ask about switching to Esbriet (pirfenidone).  I have been taking it since 2014 without significant adverse effects–but everyone’s reaction may be different.

As a new IPF patient you might find it helpful to find a pulmonary fibrosis support group.  Most groups are meeting via Zoom with COVID-19.  There are both local and national groups.  See https://www.pulmonaryfibrosis.org/life-with-pf/support-groups for more information.

Good Luck and best wishes–Doug

[Doug Jones]

Hi Cindy–I struggle with the same problem. A couple of small tips that help me are the following:

1)  I spray my throat with Chloraseptic sore throat spray beforehand.  It has 1.4% phenol which is a mild anesthetic.  I’m sure there are other brands including generics.

2) I bring a water bottle with me and take regular sips to keep my throat moist.  Sometimes just the act of swallowing changes the muscle tone and relaxes me between efforts.

3) I try to remember my relaxation techniques and think I can only do the best I can.

Doug

[Doug Jones]

Bob and Trixie–Sorry about the “messiness” of my response.  I copied the info from the PFF and it included all the formatting.  the phone number to call  is 844-825-5733 for the PFF Support Line.  Doug

[Doug Jones]

Hi Bob–as Mark mentioned, you will need a prescription from your doctor for oxygen.  I have IPF and need oxygen.  Medical facilities have developed COVID-19 protocols which protect both patients and staff when procedures such as a Pulmonary Function Test ((PFT) and 6 Minute Walk Test (6MWT).  I had a PFT performed in July 2020 and have another one scheduled for January 2021 along with a 6MWT.

For additional professional assistance you may want to contact the Pulmonary Fibrosis Foundation Support Line.

“Call Our Support Line! For those living with pulmonary fibrosis, obtaining the most accurate and current information can be a challenging and frustrating task. In response to these concerns, the Pulmonary Fibrosis Foundation launched the PFF Patient Communication Center (PCC)”. The PCC, a dedicated call center, provides patients, caregivers, and health care providers with the most up-to-date medical information, communicates the availability of support services, and provides information about other essential resources”

[Doug Jones]

1)  Charlene–gentle clarification re: COVID-19 terminology.  COVID-19 is the name given to the disease which is caused by a particular NEW corona virus– “It is likely impossible that you’ve not heard about COVID-19“>, a novel coronavirus”

2) I agree with Paul that there is often too much misinformed commentary/politics on these pages.  Not sure how to deal with that because these are essentially “speak-your-thoughts” blogs.  Using reliable resources such as those from the US government (e.g. CDC, NIH, NLM) or authoritative medical research institutions (e.g. Harvard, Mayo, Cleveland Clinic, UCSF)  Look for citations to the published medical journals and be wary of any small study or undocumented cohort–these are complex issues.  It is useful to keep in mind the analogy of the 6 blind men and the elephant:  Depending on whether you are touching the tail, the trunk, the ear, the leg… you may be “right” but you don’t see the whole elephant.

3) The most recent guidelines for older adults or those with underlying health conditions (e.g. IPF)  recommend to STAY HOME AND AWAY FROM OTHER PEOPLE–not just avoid crowds, etc.

https://www.whitehouse.gov/wp-content/uploads/2020/03/03.16.20_coronavirus-guidance_8.5x11_315PM.pdf

[Doug Jones]

For those interested in the formal recommendations from the American College of Chest Physicians, you may want to check out this article from CHEST–their official, refereed publication from 2018; below is an abstract of the article..

Doug Jones

Treatment of Interstitial Lung Disease Associated Cough:  CHEST Guideline and Expert Panel Report.   Surinder S. Birring, MD; Joanne E. Kavanagh, MBChB; Richard S. Irwin, MD, Master FCCP; Karina A. Keogh, MD, FCCP; Kaiser G. Lim, MD, FCCP; Jay H. Ryu, MD, FCCP BACKGROUND: Chronic cough in interstitial lung disease (ILD) causes significant impairment in quality of life. Effective treatment approaches are needed for cough associated with ILD. METHODS: This systematic review asked: Is there evidence of clinically relevant treatment effects for therapies for cough in ILD? Studies of adults aged > 18 years with a chronic cough $ 8 weeks’ duration were included and assessed for relevance and quality. Based on the systematic review, guideline suggestions were developed and voted on by using CHEST guideline methodology. RESULTS: Eight randomized controlled trials and two case series (10 patients) were included that reported data on patients with idiopathic pulmonary fibrosis, sarcoidosis, and scleroderma-related ILD who received a variety of interventions. Study quality was high in all eight randomized controlled trials. Inhaled corticosteroids were not supported for cough associated with sarcoidosis. Cyclophosphamide and mycophenolate were not supported for solely treating cough associated with scleroderma-associated ILD. A recommendation for thalidomide to treat cough associated with idiopathic pulmonary fibrosis did not pass the panel vote. In view of the paucity of antitussive treatment options for refractory cough in ILD, the guideline panel suggested that the CHEST unexplained chronic cough guideline be followed by considering options such as the neuromodulator gabapentin and speech pathology management. Opiates were also suggested for patients with cough refractory to alternative therapies. CONCLUSIONS: The evidence supporting the management of chronic cough in ILD is limited. This guideline presents suggestions for managing and treating cough on the best available evidence, but future research is clearly needed. CHEST 2018; 154(4):904-917

https://doi.org/10.1016/J.CHEST.2018.06.038

[Doug Jones]

These response are interesting; glad that gabapentin may work for some.  I note that the research study cited from Lancet EXLCLUDED chronic coughers who had a respiratory disease so its effectiveness for IPF patients may not be the same–see excerpt below.  The medical literature shows mixed results for gabapentin in controlling cough–plus a variety of potentially undesirable side effects.  My doctor declined to prescribe this for me.  Doug

“This randomized, double-blind, placebo-controlled trial was undertaken at an outpatient clinic in Australia. Adults with refractory chronic cough (>8 weeks’ duration) without active respiratory disease or infection were randomly assigned to receive gabapentin (maximum tolerable daily dose of 1800 mg) or matching placebo for 10 weeks.

[Doug Jones]

Consumer Reports recently reported on “The Best Flu Treatment for You”  which includes many of the suggestions noted in the previous comments.  However, Elderberry has very limited research support so I would be skeptical of that.  See the full report at https://www.consumerreports.org/flu/the-best-flu-treatment-for-you/
“Elderberry (which comes in liquid, tablet, gummy, and tea form and is said by some to ease congestion; elderberries are shown at top) and the homeopathic product Oscillococcinum, which is marketed to “temporarily relieve flu-like symptoms,” appear to be in high demand—selling out in some stores. But whether it’s a botanical such as elderberry or oregano oil, a vitamin or mineral, or a homeopathic remedy like Oscillococcinum, “there’s no good research on any of them,” says Marvin M. Lipman, M.D., Consumer Reports’ chief medical adviser. 

“The key word here is “good,” notes Lipman, and while small, preliminary studies have shown some promising possibilities for the flu-symptom-reducing effects of elderberry and oregano oil, larger, longer, and more rigorous studies are needed.

[Doug Jones]

Regarding “Off-label” use of a drug, the October 2019 issue of AARP Bulletin (page 36) has the following information and link to determine if a drug is being used “off-label”, that is, for a purpose it was not originally validated to treat.

“At DailyMed.nim.nih.gov   you can check if the drugs you’re currently taking are off-label for your disease or medical condition.  Enter the name of the medication, select the type you’re taking (pill, liquid), then click on Indications and Usages to see if your diagnosis is listed.  If you have any questions, ask your doctor or pharmacist.”

Note:  This applies to US drugs approved the US Food and Drug Administration (FDA).

Doug

[Doug Jones]

I will be attending the PFF Summit and plan to take some informal notes to share with my local Pulmonary Fibrosis Support Group.  Doug

[Doug Jones]

In the US there are two drugs approved to treat, i.e. slow the progression of IPF–Ofev and Esbriet (pirfenidone).  Clinical outcomes suggest that neither is better than the other.  So if you are really have trouble with the side effects of Ofev, considering talking with your doctor about Esbriet.

I have been taking Esbriet over 6 yrs–since it was first approved in the US–and had minimal side effects.  Of course, everyone responds differently.  I do have to be careful in the sun (wear sunblock and long sleeve shirts & hat) as it increases photosensitivity.