“Prednisone, Azathioprine and N-acetyl crysteine”, Clinical Trial for Bosentan,

[Guest guest]

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 negative

Pathology: 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 IPF

Consider 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

[Guest guest]

I was just put on prednisone and Imuran (azathioprine) and have been on them for a couple of weeks and I really don't feel any better yet at all. Guess it will take some time. I wish I had more info for you. I have to go to UAB (University of Alabama in Birmingham) in June to discuss what you have in your post most likely. I would like a chance at lung transplant, myself, but don't even know if I would qualify until I go for the visit. I wish you all the best. Carovlinkmillionare wrote: 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 CaliforniaCaroASTHMA 1976,OSTEOARTHRITIS 2002, COPD 02/06, IPF 08/07, UIP 01/08, RHEUMATOID ARTHRITIS 03/08Mississippi __________________________________________________Do

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After my experience I would never do Pred or Az again. The side effects are really bad. There is NO proven treatment for IPF. I don't know the difference in IPF and UIP.Be sure to ask a LOT of questions.. What does your DR. think it is going to do ? is it a proven corse of action?  Oh I HATE THIS DISEASE. The side effects are not worth it. ( my opinion ) I wish you all the best. Gods Blessings. Love and Prayers, PeggyIPF  2004,  Florida"Worry looks around, Sorry looks back,  Faith looks up." 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

[Guest guest]

I hate this disease, too. I don't really have any idea what they are going to do. The only thing I know for sure they told me ahead of time they are going to do are PFTs and ABGs, which I just had done in December, but they "want to do their own," so I have to have them repeated. I have never even done a 6-minute walk to see how much my SATS drop. I know they do rapidly cuz I get short of breath so quickly when walking long distances (Like within the first 5 minutes or so). My pulmo also said there is no treatment for UIP and it is the worst type of PF there is, being the most fatal (heck they are all fatal, I just got lucky enough to get the fastest one!! LOL!!). The only thing he told me the prednisone and imuran would do would be to counteract the rheumatoid arthritis which is "killing" my lungs and maybe slow it down a bit, but at the same time it will decrease my immune system making me more susceptible to colds, etc., and it

COULD POSSIBLY drastically drop my white count and shut down my liver. Then I had to say, "ok, I will give it a try." Still can't believe the words came out of my mouth to go ahead, BUT I guess I would rather try anything than just do nothing and wait for the inevitable. Caro Peggy wrote: After my experience I would never do Pred or Az again. The side effects are really bad. There is NO proven

treatment for IPF. I don't know the difference in IPF and UIP. Be sure to ask a LOT of questions.. What does your DR. think it is going to do ? is it a proven corse of action? Oh I HATE THIS DISEASE. The side effects are not worth it. ( my opinion ) I wish you all the best. Gods Blessings. Love and Prayers, Peggy IPF 2004, Florida "Worry looks around, Sorry looks back, Faith

looks up." 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 CaroASTHMA 1976,OSTEOARTHRITIS 2002, COPD 02/06, IPF 08/07, UIP 01/08, RHEUMATOID ARTHRITIS 03/08Mississippi __________________________________________________Do You

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Dilwala,

I did the drug cocktail. I could not bear to "do nothing". I have used whatever drugs they have wanted me to and tried everything. I did have positive testing for Connective Tissue diseases, where yours are negative.

Prednisone is a horrible drug, yet is a miracle drug for lungs. It reduces inflamation and helps to slow it, so (at least for me) helped prohibit the scarring. It has really awful side effects....sleeplessness, shakiness, tears, fat and more fat, too much energy, moon face, anger, moodiness....some people experience joint pain and aching muscles.

You should go through the evaluation for transplant. You don't have to make a commitment to transplant, you aren't close to that stage. But, you can be in a readiness mode, so that when it is time you will be ready. Also, you glean so much information from an evaluation.

The fact that you do not have honeycombing yet, is a positive. The fact that you have really low diffusion is a negative. What was your diffusion reading?

Keep all your records and get copies of everything. Manage your disease and care. In the meantime, enjoy life....it is wonderful!

Hugs, Joyce D.Pulmonary Fibrosis 1997 Bronchiectasis 2004 Pulmonary Hypertension 2008 Mixed Connective Tissue Disease (Lupus, RA, Sjogren's, etc) Rejected for Transplant 2006 .....I will not forget you. Behold, I have engraved you on the palm of my hands. Isaiah 49: 15-16 >> 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 negative> > Pathology: 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 IPF> Consider 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>