[Jonathan Poland]

Hi Margo, It is quite a shock when you discover that a loved one has a serious disease. I’m sorry you are suffering through that shock. But this is a long-term illness and you and your husband will get through it.

Here are random thoughts:

I’ve been on OFEV for over 6 months. I get sick enough with it that I couldn’t imagine eating beef jerky or other snacks. However, the high protein in it might help with some of the drug side effects. Eventually, your husband will need to start a low sodium diet because salt holds water around the lungs that makes breathing harder than it would be otherwise. He’ll know when that time comes.

If you are concerned about oxygen (O2), ask your doctor for a Pulmonary Function Test (PFT) and a 6-minute walk test to determine if he needs supplemental O2. And tell your doctor that he wants to fly. They probably will be doing these tests routinely to monitor disease progress anyhow.

Buy a pulse-oximeter to measure his O2 at home. These are inexpensive and readily available. (Here is a suggestion: https://tinyurl.com/2yacqy4z) Your SPO2 number on a pulse oximeter should stay above 90%. If it goes below 88%, most doctors will prescribe supplemental O2, and if it stays below 88% for any length of time you need to visit the Emergency Room. Measure it several times during the day until you get comfortable with where his levels are, and then continue to monitor at least once a day.

The FAA limits the size and number of batteries that you can carry on a plane – even for medical aids. That limits the amount of time that you can travel. (I can fly from LA to Hawaii, but not from Boston to Hawaii…) Being in Hawaii should not be a problem; even in the mountains as they are just not that high.

The other smoker in the house needs to stop for his own health, not to mention your husbands, AND your health! When the doctor prescribes oxygen for your husband, then the smoking absolutely has to quit! No candles or other open flames either. And your husband will have to stop cooking over open flames (like a BBQ or gas stove).

If his doctor hasn’t suggested it yet, he needs to get in an exercise program that focusses on core strength and heart health. A medically supervised program called “Cardio-Pulmonary Rehabilitation” is absolutely the best choice and should be covered by Medicare or his health insurance when prescribed by a doctor. In my humble opinion, this will do more for his health than even OFEV.

I’m guessing he might be a little overweight based on his diet preferences. The less you weigh, the easier it is to breathe. OFEV causes weight loss for most, sometimes severe. I’ve lost 20 lbs in the last 6 months solely due to taking OFEV. But if he needs to loose a lot: consider going on a diet as a life extending (saving) measure.

OFEV’s manufacturer has a support program that will be helpful to you and your husband. It is called “Open Doors” and you can find about it here: https://www.ofev.com/support/open-doors. It has been very helpful for me and many others.

Keep the questions and dialog coming on this forum. Getting support from others who are farther on in the journey is critical. Also, look for a local support group if you live in a larger metropolitan area. (Open Doors can help you with that…)

All the best,

— Jon

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[Jonathan Poland]

Please be aware that the Inogen G5 and all other pulse-type concentrators are NOT recommended for sleeping! When you sleep your breathing gets shallow enough that the concentrator will stop sending you O2. Then you get hypoxic without knowing it. You need a continuous-flow concentrator for overnight use – and they don’t run on batteries.

Relating to the original question, I can’t give an answer for the UK. But in the US, many campgrounds offer electric hookups that can power a continuous-flow concentrator. That is a reasonable compromise for me – except for the noise of the concentrator running all night.

– jon

[Jonathan Poland]

I have been on OFEV for over 7 months now (2x 150mg). When I started, I followed the manufacturer’s recommendation to get at least 20 gm of protein w/ each pill. To do that, I was taking the pill with a protein shake that (unfortunately) was dairy based. And I’m mildly lactose intolerant. I discovered that the protein shake was causing me as much trouble as the OFEV! As it happened, I needed an unrelated medical procedure at the end of month 3 and decided to take a 10 day holiday from OFEV. That gave my gut time to heal before I restarted OFEV. When I restarted, I made a few diet changes, partly at the recommendation of a nurse with the Boehringer Ingelheim “Open Doors” program. I now take protein + carbs with the OFEV. Specifically: at breakfast, 2 pieces of toast, and 1 egg, and a non-dairy protein shake, and in the evening, another non-dairy protein shake plus a banana.

Taking OFEV without the 20 gm of protein is a bad idea. Also having the carbs has really helped me. My “bad OFEV” days are down to less than 1 per 2 weeks. During the first 3 months, I was losing a pound per week. On my current diet, I’m only losing a pound per month. I’m OK with that.

Another downside with OFEV for me is that I can’t tolerate onions – they are guaranteed to give me diarrhea. I have to be very careful about the onions – especially dining at restaurants. Everyone is different, but look to make those kind of changes with your diet too.

The non-dairy protein shake I drink is called “Evolve” and can be found on Amazon (at least in the US).

– jon

[Jonathan Poland]

Since my progressive interstitial lung disease (pILD) was almost certainly caused or exacerbated by long-term machine oil exposure (on a submarine and Navy shipyard), I would suggest that any aspiration of any oil into the lungs is a BAD idea!

Further, since the mixture of oil vapor, in any quantity, with concentrated O2 is explosive, I would suggest that what you propose might cause a loud-rapid end to your lung problems.

IMHO the term “essential oil” is a misnomer. The only “essential” oils are the ones that your body produces on its own.

[Jonathan Poland]

Heart arrhythmia is extremely common.  But research has shown that persons with lung scarring (i.e. IPF) are much more likely to experience rhythm issues – and more severely – than the general population.  I suspect that OFEV is not the culprit, but an innocent bystander while your heart suffers from lungs that are stiff and works harder than it should.  Said another way:  every PF patient should be aware that arrhythmia is a potential side-effect of the disease.

Disclaimer:  I am NOT a doctor, but I do know how to read medical research…

[Jonathan Poland]

When full-body CT scans were popular several years ago I ask my internist (and friend) if he would recommend that I have one.  He replied by showing me a medical journal article about how they cause more angst and worry than help because we all have internal problems that don’t need treating.  For example, my lung CT also shows a benign tumor on one kidney and a stress fracture on my spine – neither are cause for alarm.

In your case: the radiologist’s wording is very technical and not in simple English.  In simple English it says that you likely have scarring in your lungs (“pulmonary fibrosis” is Latin for “scarring on the lungs”).  It also says that it is NOT ideopathic pulmonary fibrosis (IPF) – because there is no “honeycombing”.  Also there is no bronchiectasis which indicates that the scarring has not (yet) caused your lungs to stretch your bronchia.

“FVC” is the computed full volume of your lungs, and yours are larger than most people your age.  I would like to know 3 other numbers from your PFT that are used in diagnosing respiratory diseases:  FEV1, FEV1/FVC and DLCO.  Those 3 are much more important than FVC.

You will hear or read this many times on this forum: there are over 200 lung diseases.  Not all of them are fatal or even require intervention.  IPF is probably the most common on this forum, but too many people confuse the more generic term “pulmonary fibrosis” with the nasty variant IPF.

IPF used to have a 3-5 year average lifespan after diagnosis.  It doesn’t anymore because there are drugs available now that slow (but not reverse) IPF progress.  Unfortunately, Dr. Internet has not caught up with the new research results!

If I were you, I would not worry about this again unless you develop shortness of breath, chronic coughing, or other respiratory symptoms.  That would be the time for a new CT and a “differential” diagnosis to see if things have gotten worse.

– jon

Disclaimer:  I am not a radiologist or pulmonologist.  Heck, I am not a doctor of any sort.  I know what I know from dealing with my own disease and lots of research.

ps Did your pulmonologist ask you if you had ever been exposed to asbestos?

pps It could be a very long time until you need that 2nd CT.  By that time you might have changed doctors and hospitals; so get a copy of your current CT ASAP so that your future doctor will have something to compare.

[Jonathan Poland]

I am tracking FVC % rather than actual volume because there is a normal decrease over time.  DCLO is more important to me than FVC anyhow.  The actual FVC volume decrease since the beginning of my journey is roughly 8 oz (i.e. one cup of air).

Incidentally, the increase starting around 1/1/2020 is when I was in pulmonary rehab.  I credit it with my recovery of lung volume in 2020.  Even though I do cardio exercise 4-5 times a week since leaving rehab, the disease is now catching up again.

Jon Poland

[Jonathan Poland]

Hi Bernard,

I’m sorry to hear that you are having these problems and are also having difficulty getting answers from your professional staff.  (I wonder if that is universal?  I’m in the states and have a similar difficulty getting answers…)

Here in the US, most medical professionals would not recommend getting the flu and pneumonia shots at the same time because it is such a shock to the system.  We also get 2 pneumonia shots spaced 12 months apart.  Is that the same in the UK?

I have a similar coughing issue as you and have found that drinking plenty of fluids helps.  I usually drink 10-12 8oz (250ml) glasses a day – including one right before bed and one immediately after waking.  I think that would be especially important for you since you have the diarrhea side effect of Ofev.

Every pulmonologist is different in the methods they practice.  I get a pulmonary function test (PFT) every 6 months.  My most recent was just this Tuesday.  While my FVC % did drop the doctor stated that it was well in the range of the test’s accuracy and not to worry.  He also suggested that I would do better to concentrate on the actual FVC volume rather than the %.  i.e. 3.02l vs 61%  That’s because the volumes are more accurate then the % which can vary by machine type and location.  Anyhow, my doctor wouldn’t be concerned about a 7% drop unless it consistently got lower after several PFT’s.

Regular aerobic exercise at the level you can tolerate is a great way to increase your FVC.  Here in the states many hospitals offer “pulmonary rehabilitation” that are primarily structured exercise programs designed to teach you how to safely exercise to improve lung function.  It has been the single most important thing I have done to cope with my disease.

Also, my employer allowed me to switch to part-time in December.  I’m 66 and feel that working is good for my mental well being, but I could no longer handle the grind of full-time.  I’m sure that making the decision to stop working will be difficult for you too.  But at some point it becomes a question of income vs quality of life.  I have decided that the day I start Ofev will be my last day working!

Good luck.  You have found a friendly group in this forum that is more than willing to share your struggles with your disease.

– jon

ps Be sure to let us know the results of your next PFT.

[Jonathan Poland]

@dmwargas

Hi Donna,

I feel your frustration!  I find very few doctors who are willing to spend time explaining everything at a patient’s level of understanding.  And I’m not a doctor so take my comments for what they are worth.  But your feelings of uncertainty, etc. are common for nearly everyone who has this news thrust on them for the first time.

But let’s start with the good news:

– No tumors or signs of lung cancer that is often associated with smoking!  That should be a big relief.

– The airflow obstruction is MILD.  I interpret that to mean that your doctor is watching for COPD but that it hasn’t become severe enough to require treatment (yet).

– I am curious if he said more about the “mild scattered scarring”.  That could mean lots of things.  Did he describe the scarring pattern with terms such as “crazy paving”, “ground glass”,  “honey-combing”, or other odd terms?  They describe how the scarring looks on a CT scan.   Lack of those terms suggests that he is not concerned about any of the worse types of fibrosis.  It also means that whatever you “might” have is very early on.

– I’m also curious if he performed a Pulmonary Function Test (PFT) where you sit in a box or in some other way get your breathing capacity measured.  (If you did get a PFT we can help you understand the numbers.)  But in any case, the fact that he refers to “obstruction” and not “restriction” (2 very different things to a doctor) also points to COPD and not other more serious diseases.  As bad as COPD is: I would rather have that than what I’ve got.  COPD is manageable.

– He doesn’t want to see you again for 6 months.  Be glad for that.  If you were sick, there would have been more tests, trial medications, more tests, frequent visits, etc. trying to figure things out.  Doctors just can’t look at a CT scan and make a diagnosis.  The CT is just one tool to help.

– He did not use the word “fibrosis” which is Latin for scarring.  But to a doctor, it also means progressing and concerning.

I would guess that overall this is a pretty good outcome.

DO NOT LOOK AT THE INTERNET.  There is a lot of misinformation and conclusions that do not take into account modern advances in diagnosis and treatments.

DO FOLLOW this forum, and if you want, the Pulmonary Fibrosis Foundation.  But remember, your doctor is not willing to call what you have Pulmonary Fibrosis and you shouldn’t either.  Again, I would be very hopeful if I were you.

– jon

ps.  My GP forgot to tell me not to look at the Internet.  My wife and I went through a lot of needless stress before my pulmonologist straightened us out.

[Jonathan Poland]

Don and Mark,

I’m replying to a comment from several months ago… Finger pulse oximeters are NOT ACCURATE IN SUNLIGHT. The sensors in them get overloaded. If you exercise inside and get a lower reading when doing the same exercise outside,  then your finger oximeter is likely giving you a low reading when outside. They are also not accurate for persons with darker skin.   Don’t depend on them too heavily.

[Jonathan Poland]

Hi Mark,

My pulmonary fibrosis (PF) is still relative mild, as measured by pulmonary function tests (PFT).  And I have significantly improved my PFT results after starting daily cardio exercise in pulmonary rehab.  Also shortly after finishing pulmonary rehab, I read the book “Breath” by James Nestor where different breathing methods are thoroughly discussed.  I can’t say for certain, but I believe that both cardio exercise and breathing technique have improved my PFT’s and my well-being.  The best thing I ever did was start pulmonary rehab early and I am extremely grateful to the physician’s assistant who wrote the order!

I tried pursed-lip breathing during rehab.  It is simple enough to do while exercising and consciously making an effort.  But I didn’t really feel any benefit.  And it is not something I can do without making a conscious effort, such as when I’m laying in bed falling asleep.  I exercise and sleep with oxygen where nose breathing is essential.  To inhale through the nose and exhale from the mouth requires mental effort.  If I’m sick, congested, or coughing I simply can’t make the pursed-lip breathing effort.

I now practice Buteko-style breathing during exercise, but also throughout the day.  (Also, I’m now a 100% nose-breather – even when congested.)  I make an effort to take slow-deliberate breaths with prolonged pauses between inhale, exhale, inhale, etc.  I try to make the most of every breath.

Now to be technical for a moment:  PF is a “restrictive” lung disease while COPD, bronchiechtasis, etc. are “obstructive” diseases.  The difference is extremely significant.  What is good for one, may not be for the other.  Buteko Breathing Method has been shown to be beneficial for obstructive lung disease.  I’m not aware that any study has shown benefits for restrictive diseases.  Your mileage my vary.

– jon

[Jonathan Poland]

Wow Prati, I’m sure it is scary when you experience that kind of pain.  I hope it never happens when you are driving or need focus.

I learned in Pulmonary Rehab that various chest pains can occur with pulmonary fibrosis (PF), and are usually related to a full stomach putting pressure on the diaphragm and lungs.  But there can be other causes related to PF.  One of the most common is from the coughing that often occurs with PF.  And I’m not suggesting that gastro issues are your cause.  The scarring of the lungs from PF makes them stiffer than normal, and that makes other organs have a hard time.

Chest pain has been one of my biggest frustrations.  I had a hard time convincing my GP (and myself) that the chest pain and shortness of breath was not heart related.  That delayed my PF diagnosis for at least a year.  By the time I actually saw a pulmonologist, he dismissed the chest pain as coronary too.

We finally found the cause, and it was coronary, but not due to a blockage, rather an irregular heartbeat.  It didn’t show on an EKG because I never had an episode while I was getting an EKG.  It was finally diagnosed with a “Holter Test”, where you wear a portable EKG for 1 to 7 days and push a button when you feel “funny”.  And BTW, irregular heartbeats are a common problem with PF patients.

There are so many causes for internal pain that you need to be persistent with your doctors.  It may be PF related, or may not.  There are a lot of organs near those lower ribs that could cause you trouble. All of them need to be considered.

– jon

[Jonathan Poland]

@golfpi

Hi Pete,

I assume you are in the United States because you mentioned Medicare.  In the US, you need a doctor’s prescription to purchase or rent any oxygen system, including a portable concentrator.  Your doctor won’t specify a particular brand or model, just how much O2 she wants you to have and when.  The doctor’s office probably has a preferred local supplier that they will pass the prescription directly to.  Ask your doctor for a paper script if you plan on buying or temporarily renting a unit.

Medicare does cover a portable concentrator if that’s what the doctor prescribes.  You can read a lot about the rules by searching Google so I will just give highlights:  your supplier will be required to provide you service for 5 years, you will pay a (reasonable) monthly rental fee, they will choose the brand/model you get, they will probably provide you with a backup tank of O2 to help you past a power failure.  As far as I’m aware, Medicare will NOT assist in the purchase of your own device.

If you choose to purchase your own please consider these 2 things:  1) You get what you pay for  and 2) you really need to get a FDA approved model.  If you plan to travel by air, it also MUST be FAA approved.  (As with everything you can find FAA approved lists on the Internet.)

I would NEVER, EVER, EVER, buy a Chinese O2 concentrator!  They are NOT FDA approved (even if they say they are).  You welfare and (perhaps) your life depends on them producing what they promised for a long time.  How will you get service if there is a problem?  Also, they are NOT FAA approved so you can’t take them on an airplane, even as luggage.

There are plenty of US based sellers whose websites will help you decide which model is best for you.  Try searching for “best portable oxygen concentrators for 2020”.

Charlene’s model is the only portable I know that can produce continuous flow which is important if you sleep with it.  All others are strictly pulsed and are not recommended for sleeping.  I have an Inogen G3 that I sometimes sleep with.  It beeps loudly if my breathing gets too shallow.  Usually I wake up.  It is also noisy.  Between the noise and the pulse of O2 shooting into my nose, I have a hard time going to sleep, or back to sleep.

Things to consider:

• Will it produce the amount of O2 that the doctor wants you to have?
• How heavy is it?  Even 5 lbs can become a burden to carry after a long time.
• How noisy is it?
• How long will the batteries last at the doctor prescribed setting?  How hard is it to change batteries if you need to?
• Can it run off of your car power?  Can it charge off of you car?
• How will you get it serviced if something goes wrong?  (And something will go wrong eventually:  it is a mechanical device, after all.)

I hope this helps with your decision,

jon

[Jonathan Poland]

Is it the Ofev or actually the IPF?  My hair is thinning but I’m NOT on Ofev.  The thinning has been going on for about the same amount of time that I had other IPF symptoms.  Or it could be male pattern baldness – my maternal grandfather had thinning hair; but he also had IPF symptoms long before IPF was defined as a disease…

jon

[Jonathan Poland]

Hi Susan (and everyone else).  I have PF and use O2 for sleeping and exercise.  I am also an engineer, and it is that role that I’d like to explain the differences between home use and portable oxygen concentrators.

Don’t worry so much about the actual amount of oxygen they are producing, but the amount they are delivering into your body.  The portable is more efficient so it doesn’t need to have as much going out the machine.

Home use concentrators measure true flow of O2 and deliver it continuously (just like in the hospital), even when you are not breathing in.  Depending on how quickly and deeply you breath, a lot of that O2 is wasted back into the air.  (When I exercise, I estimate that 75% is wasted & way more than than when I sleep.)

Portable units are way more efficient because they don’t sent out a continuous flow.   That would make them too heavy to carry and would also drain the battery too quickly.  Rather, they have a sensor that detects when you inhale and shoots the O2 into your nose so that little is wasted.  This is why most portables are called “pulsed concentrators“.

So it is true that your portable unit is not producing the same amount of oxygen,  but it is also very likely that it is delivering an equivalent amount into your lungs; depending on how quickly and deeply you breathe.  The factory designed the portable so that a 2 setting gets the same amount of oxygen into the lungs of a typical patient as a home unit set at 2 lpm.

I am not a doctor.  But I’m sure your doctor would want to see you right away if she understood that you feel like you aren’t getting enough oxygen when you are using your portable.

– jon

[Jonathan Poland]

I think it is fairly common for those with Idiopathic Pulmonary Fibrosis (IPF) to need O2 at night even in early stages.

I was diagnosed with Usual Interstitial Pneumonia (UIP) about a year ago.  I have not been officially diagnosed with IPF yet because the scarring on the lungs does not display a “classic” IPF pattern yet and an invasive biopsy carries too much risk.  (IPF is one of several forms of UIP.)

I have 3 symptoms: cough, breathlessness from exercise, and sometimes waking up gasping for air.  The last one earned me an overnight O2 study.  My saturation when I’m asleep is in the low 80’s.  Now I sleep and exercise with  2l O2 and feel considerably better.  I had thought my morning headaches were from allergies – now I understand that they too were from low O2.

I use a Fitbit Versa to monitor my sleep.  All modern Fitbits monitor O2 during sleep and display a “relative variability”.  Mine occasionally goes into the warning zone:  that turns out to be a problem.

I have also developed an irregular heartbeat that is annoying but, according to my cardiologist, is not dangerous.  While I was not aware of it, and don’t hear it mentioned often, an irregular heartbeat is common for those with UIP or IPF.  My cardiologist believes that this is the cause of my problem; the UIP has damaged my heart’s electrical system.

I notice that the heartbeat rhythm problem is worse on days after I get a poor report on my Fitbit, and it usually takes several days for the heart to feel better.

My advise, based only on my own personal experience is:  If you have any reason to suspect low oxygen at night, get a sleep study.  And if your doctor recommends oxygen at night, use it – you almost certainly are preventing other organ damage.

[Jonathan Poland]

Is it safe to assume that your pulmonologist is older (and perhaps not up on modern understanding of rared lung diseases)?  There was a time when “idiopathic” was a catchall term for all unknown lung scarring.  (That’s actually the correct way – I still haven’t figured out when IPF morphed into a specific disease.)  Now, usual interstitial pneumonia (UIP) or non-specific interstitial pneumonia (NSIP) is used for scarring of unknown origin…

Anyhow, your PFT is extremely healthy.  FEV/FVC indicates how well you can exhale the air in your lungs.  I think it is safe to assume that it is lower than 100% because your lung capacity is above normal, and not because you have an airway obstruction (i.e. COPD).  Also, a FEV/FVC of well over 100% is an indicator of pulmonary fibrosis (PF) where the scarring restricts the lung volume but does not have exit issues.  This suggests that whatever is going on does not interfere with your lung function.  DLCO indicates how well your lungs can absorb oxygen into the blood stream.  Hence a low number is a major indicator of problems. Mine is in the low 50’s & I’m jealous of your number!

If I were you, I would seek a second opinion if you feel the need to get reassured that you are still healthy.

– jon

Disclaimer – I am not a doctor or medical professional.  Please don’t base your diagnosis or treatment plan on my comments.