[nurse_k]

Hi all!

I'm actually an OB nurse but I'm coming to you all with a topic/question that is very close to my heart. I am wanting any personal experiences, knowledge, etc about patients with pulmonary fibrosis. Two years ago my grandfather was diagnosed with pulmonary fibrosis and given 6 months to live, which turned out to be accurate. Two years before that his brother died of PF. Today, one of his sisters, my great aunt, was diagnosed with PF. Keep in mind, I am young, all of these people have been diagnosed in their early 60's. I am feeling a little doomed here. From the research I have done, 3-6 out of 100,000 people are diagnosed with PF. Now 3 of those 100,000 are in my family. Does anyone know anything about familial PF or where I can get more info? Any universities/hospitals currently doing research? Any current studies my family could participate in?

Any help would be very much appreciated. This seems to be a diagnosis that is somewhat rare.

[Angie O'Plasty, RN]

As you know, we cannot give medical advice here on Allnurses, but here is a website that might give you more information:
http://www.pulmonaryfibrosis.org/ipf.htm

[nurse_k]

Hello! I am a huge fan of this website and I am very aware that no one can give medical advice. That's not what I am seeking. I am just seeking more information. Pulmonary Fibrosis is a very under-researched disease and I am just inquiring if anyone has ever seen any familial tendencies because at this time it is not hereditary. Thanks!

[NRSKarenRN]

My pearls' of wisdom:

End Stage Pulmonary Fibrosis - allnurses.com Nursing for Nurses

[rn/writer]

You might try Googling to find out which universities and medical schools are known for their pulmonary research. Using the Internet is a great way to do the legwork of narrowing down resources.

[nurse_k]

Thanks so much but most of these websites are no longer working. I have done quite a bit of research myself on PF, I am just specifically curious about anyone who has seen or done research involving any familial tendencies in PF. My family was involved in a study with Duke University but the study ran out of funding and was cancelled. I am just trying to reach out and find anyone who knows of any current studies or up to date info on this subject. It is so hard to come by. Thanks again, I appreciate you taking time to reply!

[NRSKarenRN]

I just updated all the links in thread End Stage Pulmonary Fibrosis----take a second look.

Onset of illness age 40-60; with long term survival 3-6 years post diagnosis; history of cigarette smoking shorter side.

When look at familial pulmonary illnesses, think: did they all smoke? work in same industry? live in same part town near industries/lots air pollution?

People today are freer today to relocate, change work environment, avoid smoking and get checkups for early detection. Knowing this illness is "in your jeans" discuss with your PCP when you should have screening exam by Pulmonologist and try follow healthy lifestyle.

Under Physician Info: http://www.coalitionforpf.org/Healthcare/treatment.asp

For an updated listing of clinical trials underway in the United States, as well as other active IPF clinical research programs, please visit http://www.coalitionforpf.org/IPFResearch/default.asp

NIH also has familial study: Pulmonary Fibrosis

Best Wishes to you and family.

[llg]

I don't have any real knowledge to add, but wanted to wish you and your family well. My mother died of Pulmonary Fibrosis -- diagnosed at age 73, 2 years after a bout of pneumonia that may have triggered it. She died less than a year after diagnosis.

The lack of knowledge about this disease was very frustrating for me and my family. What was most frustrating was that we could see that she was dying quickly, but every health care worker seemed to be denying it. They kept delaying giving her the treatments and supports she needed until 3 or 4 weeks after she needed it. We kept saying, "She needed this last month," with each step up in the support they would "allow" her to have -- more oxygen, morphine, hospice services, etc. She and our family could see the rapid progression of the disease, but the professionals all seemed to be in some sort of denial. (and money was not an issue) I wanted to slap them.

[nurse_k]

I am so sorry for your loss. It does seem like there is a major misunderstanding and a lack of knowledge regarding this disease and the toll it takes on its victims. I've already watched two of my family members suffer with it and now we have one more. One of the times when my grandfather was in the hospital he was on 12 liters of oxygen at home and the RT at the hospital refused to put him on more than 8 liters because technically, no one is "supposed" to be on more than 8 liters because of the CO2 buildup. It took us fighting with them and a call from his pulmonologist to finally get them to put him on what he needed. I wish the medical community was more aware of this disease because it is becoming more and more prevalent. I am just becoming more scared, for my mother's health in particular, because I thought when we had 2 maybe it was just a coincidence, but 3? Scary.

[Nurse Laurie]

Hi,
I too have watched my lovd ones suffer from PF. My grandmother died the week after diagnosis at the age of 65. The following year, my Mom was dx. ( age 45) and died in 2 years. The following year her younger sister was dx. ( age 45) and died within 2 years. That was 1997. My great uncle, my GM's brother was dx. 2 years ago and died this past Dec. 07.
I understand your fear all too well. I recently had an abnormal chest CT after a bad bronchitis. I live in fear every day.
Many doctors still believe there is no familial tendency. How can that be. I will be happy to share any info. I come across.
Good Luck to you and be well!
Laurie