A new twist in my journey: Familial idiopathic pulmonary fibrosis

I also have familial PF, as does one brother and a cousin. . And our father and uncle died of it a while back. While there’s no doubt it must be familial, we have yet to find the genetic link. For the sake of our children and grandchildren I hope research will uncover the identifying gene, and eventually find a cure. Keep us all posted on any progress!

Thank you for this post. Two of my siblings who are now deceased had ipf and I was diagnosed 2 years ago. I do not have an official diagnosis of it being familial, but that certainly looks like what it must be.

I was diagnosed with idiopathic pulmonary fibrosis by an incidental CT scan last July. I did not go to a pulmonologist until January of this year. She explained that I was early in the disease and my pulmonary function test showed mild impairment. A enhanced CT scan verified that I did have scarring but it did not show any honeycombing or severe scarring. I immediately started Pirifinadone in February. I have had one really bad sunburn which was my fault because I did not put on sunblock. Other than severe itching Ion and off I have had very few side effects.
My older brother died of Idiopathic pulmonary fibrosis six years ago. His was caught later in the of the disease and died at the age of 76. He did not take either of the approved medications because of the possible side effects. He live three years after diagnosis and was put on oxygen within six months. As far as my extended family can tell he was the first in my family to have this disease. The Dr. suspects a familial connection. I am not convinced of this however. We were raised in a block that had a grain elevator, flour mill, and a cast iron foundry that burned coal without pollution controls. We used a clothesline as a dryer and my mother often had to wash our clothes on a bad pollution day. We had air pollution from both plants daily for the 1st 22 years of my life. I also used a CPap machine for 10 years that was recalled because the foam insulation broke down and was put into the flow of the machine for who knows how many years. I am part of a class action suit against the manufacturer of the C pap. My brother worked in several industries that used harsh chemicals and I suspect this could have been the cause of his illness. He also worked for 30 years making tarps, tents, and boat covers. The dust and fibers from that type of work could have been a contributing factor for his disease.

Now for some good news, I had a pulmonary function test in July that came back completely normal. My doctor was astounded by that result she said she never had a patient get better. As we know the two medications that are approved just slow down progression. I know that all of us with this dreadful disease face an uncertain future but I feel blessed that I caught it early, was put on medication asap, that the only signs I am exhibiting now are abnormal scans and the crackling in my chest that my doctor hears when she listens to my lungs. My blood oxygen is usually 96 to 98 even after exercise. I feel hopeful about the many medications that are in trials right now that show promise of slowing the disease down slower than the medication that I am on. There are some that show the possibility of reversing some of the scarring. I appreciate pulmonary fibrosisnews.org about the disease we face and to share with each other our personal stories with others battling this disease. Live one day at a time and feel blessed for each day we have as we adapt to our journeys with pulmonary fibrosis.

My mother had COPD(perhaps misdiagnosed) and my sister died of Pulmonary Fibrosis caused by hypersensitivity Pneumonitis. That is also my diagnosis. I also have wondered if it is familial though I have not been told that it is. My sister and I just assumed we had inherited a weakness that made us more susceptible to lung disease because we both had pneumonia frequently before we learned about PF.

As always I enjoy reading your columns , however this one about familia IPF is very interesting to me. I’ve had it for the last 13 yrs , my mother and younger sister died of it and I have 2 other siblings that have it. I appreciate all the information you can share with us .

There is definitely a familial link to this disease in some cases. My cousin had it and passed away 2 years ago from an unrelated cause. His mother had a similar lung disease and my father was found to have a lung issues that was found during an autopsy after his death at the age of 77. My cousins mother and my father were brother and sister and there youngest brother died of lung disease at 58 which was attributed to lung cancer and other lung complications which could have been PF. Too much for coincidence.
I too am fortunate to have been diagnosed early. I can still swim for an hour at a time 3 to 4 times a week,albeit not at an olympic pace,and my last two check ups have not shown any negative progress(kind of an oxymoron) in the disease, PTL. For those who are struggling with the thought of going on Esbriet or OFEV 150 or 100mg due to the side effects all iI can say is do it. I have days when I cannot stray too far from a bathroom and if I have to travel or have a multi day event I go off the meds for that time without a problem. These meds are the real deal and hopefully they can slow the IPF progression enough to give us some time until some of these new meds get approved.
Many words to say that yes it is definitely familial in some cases and if you have access to Esbriet or OFEV take it and be flexible with your lifestyle to work around the side effects. God bless you all and hang in there.

When my mother passed at age 51, we were told she had IPF (diagnosed less than a month before her death). A few years later, my younger brother was diagnosed with IPF...and he passed at age 45. Then, 4 years passed and one of my other brothers was diagnosed with IPF. He also passed about 4 years after diagnosis, at age 51. Drs. then began to open up to the idea that iPF has a genetic component. But there's more. I too, was diagnosed with IPF at age 63. Went on OFEV and experienced all the fun side effects. I was finally accepted into the Lung Transplant list... and about 5 years following my diagnosis, I "qualified" and was fortunate to be listed... In early 2021, I went through the BLT. Definitely extended my life. Not perfect, but I'm thankful!