Advice for Newly Diagnosed Patients

[Sandra Seabrooks Bush Connor]

After my diagnosis and not really knowing very much about IPF, I looked to the internet.  The first thing I read was 3-5 years lifespan is the norm.  That scared the heck out of me.  So first of all, don’t even think about that because we are all different and many, many have lived way beyond that.  I’m in my third year and feel better about it all than the first year I was diagnosed.  Back then, I panicked at every little thing that felt different.  I made it very upsetting for my husband and family.  My doctor prescribed Xanax and that helped calm me down.  I have had no need for it in a long, long time because I am use to this disease.  I do my exercises each day and if I am having a bad day, I skip them.  But, I do go back to them the next day.  Keeping your body strong is key.  Keeping a positive attitude, getting dressed each day, and walking helps a lot toward feeling better.  And lastly, I f you’re on prednisone, stay far away from chocolate!  LOL ?

[Charlene Marshall]

This is such good advice Sandra, thank you so much for sharing. I also was guilty of turning to the internet for information, and the prognosis / lifespan expectancy of 3-5 years was terrifying for me. It would be so amazing if folks who have been living with PF/IPF well beyond the 3-5 years (I know there are lots out there, I’ve “run into” many of them via Facebook conversations) posted here. I think it would reassure people newly diagnosed that the life expectancy is merely just a number. The exercises you refer to, are those pulmonary rehab exercises prescribed by your pulmnologist? I have some exercises from a transplant physiotherapist that I do, but I also try to incorporate other exercises that I can tolerate, although they haven’t been approved by my team. It just feels good to be active and as you say, keeping your body strong is so important. Thank you for sharing!

[gil]

Good advice above.  The only thing I’ll add is take care of the legal paperwork that specifies your wishes at the end of the life cycle to include what is to be done once we pass away.  I did all this so that my family will not have to make difficult choices  such as what type of treatments I want and don’t want,  what to do with my body, how assets are to be divided, etc…  Doing this isn’t an indication that one has lost hope, one should do this simply because it makes it easier on everyone. Tomorrow morning we  might get into an accident which takes us to the end of life cycle, it would help everyone if we have taken care of all the legal stuff and made our wishes known.

The 3-5 years expectancy was  scary, the doctor said I had three good years and then a rapid decline.  It was the rapid decline that hit me the hardest. That was about 7 years ago and I am still walking and I am not taking any medication.  The three to five year span is based on an average, some people will last many years.  Also, there is science and so there is hope.

 

[Charlene Marshall]

Hi Everyone,

Hi Gil,

I really like how you mentioned that taking care of all the legal paperwork isn’t an indication of one ‘giving up’ or giving up hope, and instead, it is more a matter of practicality because as you say, anyone’s life could abruptly end unfortunately. I really should look into doing this. A friend of mine who is now widowed mentioned that her and I should do a will together, since she doesn’t have one done up either. This is a daunting task for me, being only 30 but I agree with you in the sense that I really don’t want my family to have to deal with all of this. And, death is inevitable, we are all eventually going to die (PF or not) so as difficult as it is to talk about, this is important.

I’m also so glad that you shared that it has been 7 years since your diagnosis because it really highlights that the 3-5 year life expectancy really is just a number and an average for people, but does not apply to everyone. If you are comfortable, can you tell me (the forum) a bit more about the rapid decline you referenced? What did that look like for you, was it more frequent periods of shortness of breath or physical lung function decline? I hope it is okay that I asked you, it is something I’d also like to know.

Thanks,
Charlene

[Nanzy]

I was diagnosed about a year ago just after retiring at age 58.  In some ways I consider myself fortunate in that I was familiar with the disease because my mother had died six years previously at age 70 due to complications of cardiac arrest and pulmonary fibrosis.  Unfortunately she was treated for COPD rather than the pulmonary fibrosis.  I had the dry constant cough, sounding just like my mom had years before her death.  A diagnosis of pulmonary edema sent me to a cardiologist who game me a clean bill of Heath heart-wise.  I insisted on  a referral to a  pulmonologist who did a CAT Scan and an outpatient lung biopsy – both were inconclusive for IPF.  A lung biopsy was performed and the IPF was confirmed.  My family and I feel that my mom has saved my life due to an early diagnosis.

I have been on OFEV since the diagnosis and have not shown a decline based on the six minute walks.  I don’t need oxygen right now, but I found that the heat and humidity in Louisiana make it more difficult to breathe.

My pulmonologist has referred me for the initial lung transplant evaluation.  I pray the OFEV continues to work in keeping me ‘stable’ so that a transplant is years away!  Except for the occasional shortness of breath and cough, I would never know I have IPF.

Has anyone else been prescribed OFEV?  Has it helped?

 

 

[Charlene Marshall]

Hi Nanzy,

Thank you so much for sharing your advice and joining in on this topic with us!

Although I am awfully sorry to hear about your Mom, especially the treatment of COPD instead of the PF, it gave me goosebumps to read about how you feel that your Mom saved your life by being aware of the disease for an early diagnosis for you. Have they linked the disease as hereditary for you then, calling it familial PF? I’m trying to learn more about that, because it was so rare for me to get it (there is no history of any type of lung disease in my family) and I want to try and see if there is a link out of fear that others in my family could get it. I’m not sure of the criteria that would quality PF to be considered familial, whether it is on one side (ie. paternal or maternal) or whether there needs to be a certain number of family members, generations diagnosed. I’m hoping to learn more about this at some point.

I am also so glad to read that you insisted on the referral to a pulmonologist, I think advocating for our own health needs is so important. That is probably one of the pieces of advice I’d add to the list for those newly diagnosed: don’t be afraid to advocate for your needs if you feel like you’re not being heard. This is hard to do in the medical world, but it is also so important.

I pray that the OFEV continues to work for you as well, and that transplantation surgery is still a ways out for you too. Thanks again for writing Nanzy!

Charlene

[Michelle Merry]

My Mom started OFEV last week. We were all very worried about the side effects but thankfully that has not been an issue yet.  Still in early days.  Mom thinks that her secretions are drying up a bit.  I know it will take time to see if the OFEV stabilizes her.  Fingers crossed.

[Charlene Marshall]

Hi Michelle,

Thank you so much for sharing about your Mom’s experience thus far with OFEV. I will continue to keep my fingers crossed that she doesn’t experience any nasty side effects. If I may ask, how long did your Mom have PF before being put on OFEV?

Charlene

[Charlene Marshall]

Thank you so much for your contributions to this topic so far everyone! I really hope patients who are newly diagnosed come across it and find comfort in everything you’ve shared 🙂

[gil]

Charlene,

“If you are comfortable, can you tell me (the forum) a bit more about the rapid decline you referenced? What did that look like for you, was it more frequent periods of shortness of breath or physical lung function decline? I hope it is okay that I asked you, it is something I’d also like to know”

The “rapid” decline is what the doctor predicted after three years, I don’t believe I am at this point. Yes, 2016 and 2017 I experienced not being able to do things I used to do but the decline isn’t rapid. Scans show my lungs are worse than a year ago but as  doctor tells me, I don’t yet show the expected symptoms.  I am lucky.

[Charlene Marshall]

Wow Gil, thank you so much for sharing. I am glad you’re lucky in the sense that you haven’t declined as rapidly as the Doctor predicted. Did they say this decline would just happen suddenly, or at random? Or did they say it would be triggered by something, like a cold or flu? I am pretty terrified of catching the flu at the moment, just out of fear that it will cause an exacerbation and then a significant drop in my lung function. I am hyper-vigilent about washing my hands on a regular basis, and I pay particularly close attention if I feel even anything coming on, because I always understood that a cold or flu could cause a rapid decline in those with IPF. I wonder if this is similar to what your Doctor was referencing, or something totally different?

Talk with you soon,
Charlene

[Rick Weldon]

My final diagnosis was in November of 2017. I have questions rather than advice. I am on 2 LPM resting and 8 LPM while active. I am thinking of going on Esbriet and wondering about the side efffects and such. Any opinions?

[Charlene Marshall]

Hi Rick,

Thanks so much for sharing a bit about your journey and diagnosis with IPF. Did it take you awhile to get diagnosed? I’ve read both in literature and heard from many other patients, that a final diagnosis of PF takes an average of 16 months even when symptoms are regularly present and a patient suspects something is wrong. Mine took about 13 months, but that was from my very first episode of shortness of breath all the way up to needing oxygen during my 6 minute-walk-test. All in all, I think I’m pretty lucky to have had it only take that long because IPF was not on the radar of any physician I saw, since I was so young (28) at the time of my diagnosis.

Your questions are always welcome here! In terms of Esbriet, I was on it for awhile and I didn’t have major side effects but there were some unpleasant ones, like GI-type stuff and just a really sour stomach that nothing seemed to resolve. I could eat and drink, it wasn’t anything like that, but I just  had a constantly upset stomach that was unpleasant, particularly if I was out somewhere public or at work. Do you have a choice between Esbriet and OFEV? Just curious…

Charlene

[Linda Smith]

I was diagnosed,shortly after OFEV was approved. I had finally found a doctor who did not tell me I had asthma or COPD as decades of others had. He diagnosed me within minutes of my first visit. (Have the other pulmonary doctors not heard of IPF?)

Once he had done all the preliminaries to confirm his diagnosis he gave me two years to live. Then he put me on OFEV. I’m sure I’m the first of his patients to take the drug.  It was, perhaps, a ‘what the heck’ decision on his part.

I’ve had a little trouble with side effects from time to time, but this is the outcome.

My lungs are clear. The IPF has reverssed itself.

When I first learned this I was puzzled. Had I been misdiagnosed?

The OFEV people call once a month to see how I’m doing. A couple of months ago I described my improvement and asked if other people were having this result.

The caller said, “yes”, without details or numbers.

I suggest that anyone suffering from IPF try OFEV. It may be an actual wonder drug. It certainly has been in my case.

[Charlene Marshall]

Hi Linda,

I am glad you found a doctor who listened to you and didn’t insist you “just” had asthma. This was my experience initially as well because I was so young, which was incredibly frustrating. It wasn’t until they checked my pulse ox that they realized something was really wrong, and they shipped me to the ER right away.

I also am on OFEV,  and my doctor was quick to prescribe it when he realized how rapidly my lungs were deteriorating.  Did you experience any side effects when you first started it or as you continued to be on it? I read that you mentioned you’ve had a little trouble with side effects, but was this when you first started the medication?

I am so hopeful to hear your story, and how this drug has helped you Linda. Thank you for sharing 🙂

Do you still consider yourself having a diagnosis of IPF, meaning there is still fibrosis in your lungs? I just wanted to clarify what you meant by it reversing itself. Needless to say, I am so glad your symptoms have subsided. I wish you all the best!

Warm regards,
Charlene.

[Rick Weldon]

Charlene    Once we started looking at lungs, it took about a year. We spent about 2 years looking for heart issues. I had 2 angiograms about 2 years apart and seeing no change, they sent me to a pulmonary specialist.  Once there, I did a bronchoscopy, but the results were inconclusive. We then did the full lung biopsy and  determined it to be IPF. I am now trying to decide if I am going to try Esbriet. I have joined a fitness/rehab clinic that provides the O2 needed and it does seem to be making a difference. My doctor has only suggested the Esbriet but I will discuss why at my next appt. The end of February.

[gil]

Charlene,

“I wonder if this is similar to what your Doctor was referencing, or something totally different?”

I don’t know.  The doctor said “rapid decline” but I did not ask, I think I may have been in shock or something. Next time I will ask him.

I know what you mean about the flu,  I believe I recently had the flu but it may have been my allergies or even IPF symptoms…..  I am going to ask my doctor if there is a way to tell the difference, I suspect that regardless of what triggers my symptoms the “cure” is the same, rest and drink plenty of juice and water.

Nancy, Rick,

You both had it rough, Good luck with the medication and hopefully science will make advances in time to help us, especially all of you who are young enough to avoid the Senior Citizen title.

 

[Charlene Marshall]

Hi Gil,

It’s nice to hear from you!

Yes, I am not sure what my doctor meant about “rapid decline”, although I suspect it is after looking at my PFT results and seeing a decline in those. They told me when I was diagnosed that I might not see a difference in years of living with this disease, or that everything could change due to a decline in my lungs very quickly. Unfortunately it was the latter that happened for me.

When you ask your specialist about noting the difference between flu or IPF symptoms, can you let me know what he or she says? I suspect one of the biggest indicators of difference between these two would be the presence of a fever or not. I had one when I think I had the flu, so that is why I think it is more the flu than anything.

Hope you are doing well and thanks again for sharing!

Cheers,
Charlene.

[gil]

This is more advice related to pneumonia but I think it applies to pulmonary fibrosis too.

Research show brushing one’s teeth and mouth care will inhibit bacteria from infecting lungs, in one study pneumonia was a problem and oral care, including teaching one how to correctly brush teeth, got rid of the problem and improved the patients’ quality of life.

https://www.blogs.va.gov/VAntage/45224/routeva-road-trip-preventing-disease-through-better-oral-care/

 

[Charlene Marshall]

Hi Gil,

Thanks for sharing the post about oral healthcare! Somehow I missed this, and I am so glad to have read about it. So important to know that oral healthcare can inhibit germs from travelling to the lungs: an easy thing we can do to help ourselves. I always like reading those things!

 

Thanks again 🙂
Charlene.

[Charlene Marshall]

Hi Rick,

Thanks so much for sharing! Did you have any suspicion that it was your lungs, or did you feel more strongly that it might be your heart? I never really connected the symptom of shortness of breath to my heart (although I don’t know why I didn’t, looking back in hindsight I should have) so I felt a bit frustrated that they were initially focusing so much on my heart at first. What was the biopsy like for you? I know this is one of the most invasive, but concrete methods of diagnosing IPF but I have heard lots of people talk about not wanting to go that route out of fear that it would make their breathing troubles worse.

Good for you for joining the fitness/rehab clinic, is it one at a local gym or is it a program through a hospital/treatment center? Always curious from those who do pulmonary rehab programs, as I really felt like mine made a difference for not only my ability to breathe better but for my overall body strength. I’m hoping to do another program in the spring actually.

Goodluck with your upcoming appointment!
Charlene.