Ideopathic Pulmonary Fibrosis

[mtorresm]

My husband has been diagnosed by the diagnostic clinic at the medical center in houston with IPF. He still has not had an open lung biopsy. The doctor told him he had 2-4 years to live. How can he be so certain of this diagnosis without an open lung biopsy?

[National Jewish]

Idiopathic pulmonary fibrosis (IPF) is swelling that leads to scarring of the lungs in the area between the air sacs and the blood vessels.  The amount of information required to make the diagnosis of IPF can range from a simple to a more extensive evaluation.  The simple evaluation includes a history and examine combined with a chest x-ray.  The more extensive evaluation could include breathing tests, a blood test to check the oxygen and carbon dioxide levels, an exercise test, bronchoscopy and open lung biopsy.  The amount of evaluation required to make the diagnosis will be dictated by how certain the diagnosis needs to be and how much discomfort and risk your husband wants to undergo.

A pulmonologist at a university medical center is most likely to have the greatest experience in evaluating people with IPF.  This would be the best specialist to know if an open lung biopsy would be needed to be certain of your husband’s diagnosis.  Please read our Interstitial Lung Disease MedFact at http://www.nationaljewish.org/medfacts/interstitial.html for more information.

Given the poor prognosis of this disease, it is important that the diagnosis be established with certainty. A biopsy is recommended to confirm the diagnosis; unless the chest films are clearly showing IPF and no other treatable diagnosis might be considered

[CINDY4]

I am sorry to hear about your husband's recent diagnosis.  Things like that are a shock to hear.  As I am sure you were told IPF is a serious disease with no cure, but you can treat it to slow progression.  There are many drugs like steroids, Actimmune, and Interferon.  IPF is fairly rare and you should be under the care of a dr who specializes in the disease.  Most pulmonologists are not that familar with it, and you need to find one that is.  The Houston area should have some good pulmonologist, you need to ask specifically for one that has dealt with IPF.  Pulmonary rehab is also an important part of IPF, that way you learn to use what you have.  My daughter has another disease that was thought to be IPF at one time.  She does have areas of fibrosis but they are secondary to another disease.  A friend of mines mother was diagnosed with IPF without a biopsy, from the CT and pulmonary function tests that included diffusion capacity.  This determines the lungs ability to exchange gases, and may be what caused your dr to give you such a short time span.  I am on a support group for lung disease and there are several with IPF on it.  Some only have around 20-30 capacity, but have already lived longer than what this dr told you.  Also there is the option of transplant, if you meet age and other medical requirements.  There are some new treatments on the horizan that are supposed to actually be able to repair the fibrosis(which was thought to be irrepairable).  So there is some hope, you just have to really research and know what is available.  Best of luck.

Cindy