Question 1

is pulmonary fibrosis terminal?

Accepted Answer

Pulm Fibrosis: Hi, Pulmonary fibrosis can develop slowly or quickly. There is no cure. Many people with the disease live only about three to five years after diagnosis. The only treatment is lung transplant.

Question 2

is pulmonary fibrosis inherited?

Accepted Answer

Pulmonary fibrosis: While I have not seen it commonly there are some families with a history of pulmonary fibrosis. Most of my office patients and previous fibrosis patients I have come across are the only ones in their family with fibrosis. I have one patient in my practice with several family members with progressive and ultimately fatal fibrosis. She has been evaluated at duke university with a genetic workup.

Question 3

what is pulmonary fibrosis like?

Accepted Answer

Scarring: Idiopathic pulmonary fibrosis is an inflammatory process affecting lung parenchyma and resulting in loss of lung storage and resulting in low diffusin capacity and restrictive lung disease. Cardiovascular effect inckude pulmonary hypertension and cor pulmonale. The disease is slowly progressive and unresponsive to all current therapy. Lifespan can be in months(hamman-rich)  to a few years.

Question 4

can pulmonary fibrosis be cured?

Accepted Answer

No and Yes: At this time the only curative intervention for pulmonary fibrosis is lung transplantation.  There are no medications that have been proven to cure the disease.  Some medicines may help (such as steroids, n-acetylcysteine and pirfenidone) slow the disease however.

Question 5

how is pulmonary fibrosis treated?

Accepted Answer

Options: Pulmonary fibrosis is treated with supportive care and oxygen.  There are no drugs that have been proven to help at this time although a drug called pirfenidone has been studies and shows progress (it decreases progressive scarring of the lungs).  There are also a number of drug trials ongoing in the US and elsewhere to try and find something that works.

Question 6

what to expect in pulmonary fibrosis?

Accepted Answer

Slow progression...: Pulmonary fibrosis is a slowly progressive disease. The patient gradually, usually over years, becomes increasingly short of breath and has increasing difficulty functioning. The patient has less oxygen in their blood over this time period and will eventually require supplemental oxygen. At end-stage, consideration for a lung transplant can be entertained, depending on many other factors.

Question 7

looking for info on pulmonary fibrosis?

Accepted Answer

Websites to start: Here are some links to useful places to start:

http://www.Ncbi.Nlm.Nih.Gov/pubmedhealth/pmh0001134/

http://www.Mayoclinic.Com/health/pulmonary-fibrosis/ds00927/

http://www.Pulmonaryfibrosis.Org/about

best of luck!

Question 8

what are symptoms of pulmonary fibrosis?

Accepted Answer

Shortness of breath: Shortness of breath and nonproductive cough are the most common symptoms for this rare disease.   Older age eg over 50 is usually required ..... Presuming you are talking about ipf.

Question 9

what is the cause of pulmonary fibrosis?

Accepted Answer

SILICA,ASBESTOS,BIRD: Fibrosis in the lungs has many causes
1inhalation of silica dust, asbestos, grain dust, bird and animal droppings
2 radiation therapy for breast cancer
3medications like chemotherapeutic drugs and amiodarone, methotrexate, nitrofurantoin
4 secondary effect of other diseases likeinterstitial lung disease
5 idiopathic when no cause can be found
6 sarcoidosis and wegnors granulomata
7 connective tissue di.

Question 10

what are the tests for pulmonary fibrosis?

Accepted Answer

Several: A high resolution ct scan of the thorax can give valuable information. Blood tests looking for underlying autoimmune diseases are usually done. Many times a lung biopsy may be required for accurate diagnosis.

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