Someone that my husband works with said that he goes to church with a woman who has CF & wanted to know if I would be willing to speak with her because she is not doing well. Of course I try I said yes. My husband brought home the name/# of this woman & I know her. I called my Mom & best friend from school to see if they knew she had CF & no one knew anything. We all thought it was odd that we didnt know. We live in the country & every family knows the other or at least enough that something like this shouldnt slip past. She also is much older with grandkids, but as we are learning that doesnt mean anything. So I decide to call because I need to know. It turns out she has Excentra (sp?) (similiar to excentric--unusual, unexplained) Pulmonary Fibrosis. Although much of her breathing difficulties are similiar it is not a genetic illness nor due they know the cause & its not very common. The basics is that the lungs turn to leather type consistancy not allowing them to work properly which of course makes it hard to breath. When she was diagnosed 5 years ago the Dr. told her not to expect more then 5 years & during her recent checkup was reminded that in January her 5 years is up. I said, "You cant get an extended warranty? I mean thats what he makes it sound like." She just laughed (as best as she could). She told the Dr. that since they cant do anything to stop the progression that whatever it is it is.....My whole point is that she is a very sweet, sweet lady. She took my number & said she will keep in touch because even tho our illnesses are different, the breathing difficulties are comparable at points. Has anyone ever heard of this?
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Pulmonary Fibrosis
- Thread starter JazzysMom
- Start date
My husbands Grandmother said something to me months ago. For the last 18 months or so she has had major breathing problems .She told me her lungs were like leather and is on breathing treatments and things every 4 hours or less. Didn't think much of it just her elderly age was starting to show she is almost 80 but what you explained sounds just like what she told me except no name for it.
2
2sickkids
Guest
That is what my mother in law died of. It is simalar to the end stages of cf with out the gi problems. I actually think because our family watched someone suffer from that may me why they are so distant from our sons. If you asked your pulm dr about it I am sure they could tell you anything you'd like to know.
My husbands grandfather was diagnosed with pulmonary fibrosis about 7 months ago. He was 88 years old. He passed away this past June, six months after diagnoses. I researched PF after his diagnoses and this is what I found. Hope it helps!
What is Pulmonary Fibrosis?
Pulmonary Fibrosis involves scarring of the lung. Gradually, the air sacs of the lungs become replaced by fibrotic tissue. When the scar forms, the tissue becomes thicker causing an irreversible loss of the tissues ability to transfer oxygen into the bloodstream.
What are the symptoms?
" Shortness of breath, particularly with exertion
" Chronic dry, hacking cough
" Fatigue and weakness
" Discomfort in the chest
" Loss of appetite
" Rapid weight loss
What is the prevalence of Pulmonary Fibrosis?
There are five million people worldwide that are affected by this disease. In the United States there are over 200,000 patients with Pulmonary Fibrosis. As a consequence of misdiagnosis the actual numbers may be significantly higher. Of these more than 40,000 expire annually. This is the same as die from Breast Cancer. Typically, patients are in their forties and fifties when diagnosed. However, diagnoses have ranged from age seven to the eighties. Current research indicates that many infants are afflicted by Pediatric Interstitial Lung Disease. At this time there is limited data on prevalence for this group.
What are the causes?
Traditional theories have postulated that it might be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a growing body of evidence which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of Pulmonary Fibrosis. The most current thinking is that the fibrotic process is a reaction to microscopic injury to the lung. While the exact cause remains unknown, associations have been made with the following:
" Inhaled environmental and occupational pollutants
" Diseases such as Scleroderma, Rheumatoid Arthritis, Lupus and Sarcoidosis
" Certain medications
" Therapeutic radiation
How is it treated?
There are currently no effective treatments or a cure for Pulmonary Fibrosis. The pharmacological agents designed to treat lung scarring are still in the experimental phase while the treatments intended to suppress inflammation have only limited success in reducing the fibrotic progress.
Because the origin and development of the disease is not completely understood, misdiagnosis is common. Varying terminology and lack of standard diagnostic criteria have complicated the gathering of accurate statistics about people with pulmonary fibrosis. Supplemental oxygen improves the quality of life and exercise capacity. Single lung transplant may be considered for some patients. Pulmonary Fibrosis is a very complex disease and the prediction of longevity of patients after diagnosis vary greatly.
There are a number of new trials testing drugs to treat Pulmonary Fibrosis. Contact us at:
Pulmonary Fibrosis Foundation
1332 North Halsted Street Suite 201
Chicago, Illinois 60622
(312) 587-9272 fax (312) 587- 9273 for more information.
Seana
mother to Lauren, 14, no CF- Courtney, 12, with CF- Cameron, 10, no CF
What is Pulmonary Fibrosis?
Pulmonary Fibrosis involves scarring of the lung. Gradually, the air sacs of the lungs become replaced by fibrotic tissue. When the scar forms, the tissue becomes thicker causing an irreversible loss of the tissues ability to transfer oxygen into the bloodstream.
What are the symptoms?
" Shortness of breath, particularly with exertion
" Chronic dry, hacking cough
" Fatigue and weakness
" Discomfort in the chest
" Loss of appetite
" Rapid weight loss
What is the prevalence of Pulmonary Fibrosis?
There are five million people worldwide that are affected by this disease. In the United States there are over 200,000 patients with Pulmonary Fibrosis. As a consequence of misdiagnosis the actual numbers may be significantly higher. Of these more than 40,000 expire annually. This is the same as die from Breast Cancer. Typically, patients are in their forties and fifties when diagnosed. However, diagnoses have ranged from age seven to the eighties. Current research indicates that many infants are afflicted by Pediatric Interstitial Lung Disease. At this time there is limited data on prevalence for this group.
What are the causes?
Traditional theories have postulated that it might be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a growing body of evidence which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of Pulmonary Fibrosis. The most current thinking is that the fibrotic process is a reaction to microscopic injury to the lung. While the exact cause remains unknown, associations have been made with the following:
" Inhaled environmental and occupational pollutants
" Diseases such as Scleroderma, Rheumatoid Arthritis, Lupus and Sarcoidosis
" Certain medications
" Therapeutic radiation
How is it treated?
There are currently no effective treatments or a cure for Pulmonary Fibrosis. The pharmacological agents designed to treat lung scarring are still in the experimental phase while the treatments intended to suppress inflammation have only limited success in reducing the fibrotic progress.
Because the origin and development of the disease is not completely understood, misdiagnosis is common. Varying terminology and lack of standard diagnostic criteria have complicated the gathering of accurate statistics about people with pulmonary fibrosis. Supplemental oxygen improves the quality of life and exercise capacity. Single lung transplant may be considered for some patients. Pulmonary Fibrosis is a very complex disease and the prediction of longevity of patients after diagnosis vary greatly.
There are a number of new trials testing drugs to treat Pulmonary Fibrosis. Contact us at:
Pulmonary Fibrosis Foundation
1332 North Halsted Street Suite 201
Chicago, Illinois 60622
(312) 587-9272 fax (312) 587- 9273 for more information.
Seana
mother to Lauren, 14, no CF- Courtney, 12, with CF- Cameron, 10, no CF