Re: “Prednisone, Azathioprine and N-acetyl crysteine�, Clinical Trial for Bosentan,

[Guest guest]

Dilwala,

One thing in particular would make in inclined to give the Prednisone, Azathioprine, and NAC. You were told you have "alot" of inflammation. Did your biopsy report actually say that? If it did and there is considerable inflammation the nurse is correct in telling you that there is nothing that will make short work of the inflammation quite like the prednisone. Part of the disease process that we have is that the inflammation eventually scars and becomes fibrotic. If you can interrupt the inflammatory process you can prevent further fibrosis.

Normally UIP or IPF do not respond well to prednisone. If you do indeed have alot of inflammation, though the prednisone might be worth a shot.

Good luck whatever you decide.

Beth

Age 48 Fibrotic NSIP 06/06

Change everything. Love and Forgive

“Prednisone, Azathioprine and N-acetyl crysteineâ€, Clinical Trial for Bosentan,

Hope this finds the Air Family in good spirits.I visited UCSF in late March. Below are the excerpts from their panel after they reviewed my recent CT scan (taken on 3/21/2008) and pathology of the lung tissue (taken on 2/12/08).PFT: "studies are significant for mild restrictive lung disease with a severely reduced diffusion capacity". There is no evidence of obstructive lung disease.Radiology: CAT scan was significant for patchy, peripheral, primarily subpleural fibrosis which effects all levels of the lung. There was no honeycombing. It was also notable for focal areas of ground glass opacities peripherally which are consistent with organizing pneumonia. This is most consistent with fibrotic NSIP or less likely, hypersensitivity pneumonitis. Laboratory: rheumatoid factor is negative; angiotensin converting enzyme negative; ANCA negative; ANA negativePathology: The

biopsy consisted of two samples; right middle lobe and right lower lobe. The right middle lobe sample was significant for patchy fibrosis which was not airway centered with scant fibroblastic foci and chronic interstitial inflammation. There was also macrophage accumulation around the respiratory bronchioles consistent with smoker's disease. The right lower lobe demonstrated contrast dense fibrosis with many more fibroblastic foci adjacent to areas of near normal lung. There was also some emphysema. Taken together, these findings are most consistent with UIP pattern.Assessment: The biopsy results are most consistent with UIP pattern. Given that there is little to support this patient has a rheumatological disease, the most likely clinical diagnosis for this pattern is idiopathic pulmonary fibrosis. This patient is on the younger side for the age that we typically see with this

diagnosis. Also, there are some subtle features of his biopsy that are not classic for IPF; however, this may merely represent the effect of smoking. Plan/Options: Enroll in a clinical trial (I was asked to enroll in a trial named BUILD 3; a trial of bosentan in patients with biopsy proven IPF).Be treated with best guess therapy (prednisone, azathioprine and n-acetyl crysteine)Opt for no therapy as there are no proven effective therapies for IPFConsider referral for lung transplantation. The visit with the doctor was short. There was simply not enough time as there were other patients. Same was the case when the doctor called me after the panel discussed my case. However, I received a call from the nurse who spent over an hour on the phone and did her best to answer my questions. She stated that I don't have a lot of fibrosis but I do have a lot of inflammation. Her

suggestion was to get on prednisone as "there is nothing better than prednisone when it comes to reducing inflammation" . She also stated that I should not be concerned about the side affects. Side affects are directly related to the prolonged usage and higher strength. HowamIDoing: I still have the phantom pain after biopsy (done on 2/12/08). Pain is somewhat tolerable (i.e, one Vicodin/day, instead of three) but it remains.My 7th grade All-Net team made it to the National competition in Los Angeles. We lost the semi-final and took 4th place. The boys made me proud.It is ~6 hours drive to Southern California; I drove both ways with ease. The tournament schedule was hectic but I remained energetic. I also felt `easier to breath' when I was there, mainly in Anaheim. I noticed the difference as soon as I stepped out of the car in my driveway. It was absolutely astonishing. I

don't' think it has anything to do with the sea level. They are about the same. I do live in the `foothills' but the height is not that significant. Something in that `air' which allowed me to breathe effortlessly. I will inquire from my Pulmo when I see him on 4/22. Does any one know why?Baseball season is underway. We are 2-3. Early part of the season is mainly for learning so we're on track. This is big field so boys are still adjusting.I don't do much except motivate and give instructions. I cannot throw the ball and/or hit the ball. It is mainly due to the affects of the biopsy.WhereamIGoing: So they (panel at UCSF) provide me with four options:Do Nothing: I don't like my chances if I do nothing.Clinical Trial: I would rather be treated than be in Placebo group for 18-24 months.Lung Transplant: Unless they tell me that there is no other choice I would like

to stay away form highly invasive medical procedures.Thus I'm leaning towards the recommended drug cocktail. Please provide your input, pros/cons, your experience with these drugs and/or any info that you would like to share otherwise. I will see my Pulmo on 4/22 to start the treatment. Thanks, and God bless all,Dilwala, UIP 2/28 Southern California