[elizabeth martin]

It’s all a matter of when you are actually diagnosed and the methods used to make the diagnosis.  My sister was diagnosed a week before she passed away.  For years she had been variously diagnosed as having bronchitis, pneumonia, asthma, etc.  She walked into the hospital and never walked out.  A lung biopsy confirmed IPF.

I was diagnosed 11 years ago after a routine chest XRay showed honeycombing. Subsequent PFTs and then a lung biopsy confirmed IPF.  I am not on oxygen and am able to maintain oxygen levels at 96-97% except when I do strenuous work it will drop to low 90s.

I recently was diagnosed with moderate congestive heart failure which is not surprising since IPF puts a strain on the heart.

I was initially taking Esbriet but the side effects were horrible- diarrhea 8-10 times a day, weight loss, fatigue, low blood pressure.  I am now on Ofev and not sure whether or not I will continue with it due to the side effects, although they do not seem to be as severe yet as those with Esbriet.

In my opinion, many people have lived with IPF for decades until it becomes severe enough to have definitive tests such as a lung biopsy.  That’s why the survival estimates of 3-5 years are not reliable.  Those are based on so many people not being diagnosed until the disease is well progressed.

[elizabeth martin]

I’m convinced that the 3-5 years can be misleading.  For many people, there have been years and years of bouts of shortness of breath, dyspnea, coughing, etc., and the person is diagnosed with pneumonia, the flu, bronchitis, or some other respiratory illness. Many suffer for a decade or more with those symptoms until they actually see a pulmonologist who orders the required testing which confirms IPF. My sister passed within days of her diagnosis of IPF. She walked into the hospital with yet another bout of ‘bronchitis,’ one of many, many rounds of that misdiagnosis over the years.  She was hospitalized, grew steadily worse, and died within a few days of her hospitalization.

My diagnosis was 10 years ago, initially diagnosed by an ER doctor who looked carefully at my x-Rays after a bout of difficulty breathing.  He referred me to a pulmonologist. I had X-rays, CT, and VATs over the next year which confirmed the diagnosis. Because my diagnosis was made when I barely showed honeycombing in my lower lobes, and had few symptoms, I have had little concern about the 3-5 year life expectancy because my disease advanced slowly.  However, within the past six months, there has been a noticeable change, intensified symptoms, so maybe I am now at the 3-5 year threshold.

Only God knows!

[elizabeth martin]

I am 71 yrs old, a retired registered nurse.  I had VATS (Video Assisted Thoracotomy) and a diagnosis of IPF in 2018, not sure why my pulmonologist chose that instead of a bronchoscopy. VATS is a very invasive surgery and very painful as rated by the NIH. The surgeon must go through your ribs to get the lung specimen, this involves damage to the ribs as well as nerve damage. The area around my right breast was so painful that I had to have narcotics to deaden the pain, and I have a high tolerance to pain and hate to take meds. That nerve pain continued for months, resulting in my no longer being able to wear a bra or to have anything snug covering my chest.  The pain where they cut through my ribs is transient, I can live with both it and the nerve pain. But, even though I am a retired registered nurse, and I did due diligence in researching VATS, I was totally unprepared for the pain to be so bad and to continue for so long. I would possibly have been better off having a bronchoscopy, but considering that the honeycombing was at the extreme lower lobe level of both lungs, it probably would not have been nearly as diagnostic as the VATs.

My older sister died at the age of 60 from IPF. There are studies that suggest that some people are more susceptible to IPF who grew up on a farm, which we did.  Another issue is chronic GERD, which we both had/have, and the probability that acids enter the lungs and start the erosive process. So far I am only on meds for GERD and have not had to rely on oxygen yet . I do get winded with exertion and there are times that I feel no oxygen is available but those times are not regular or often. I have an appointment with my pulmonologist next month to discuss the antifibrotic meds.

Best wishes to all, Hugs!

[elizabeth martin]

I, too, have started to have thick plugs of mucous. I usually spend a good bit of time in the morning coughing up the crap. I cannot imagine what it is like for someone who is frail and who does not have the ability to do deep coughing. I have an appointment with my pulmonologist next week and I’m sure she will say that this is the normal progression. It’s still very scary!