RE: Re: Perfinedone application made to the FDA

[Guest guest]

Bruce,

thank you so much - I truly appreciate you sharing all this information that must have taken you so much research time.

I guess I just don't know enough about my own DX - UIP caused by autoimmune connective tissue, etc. and that is only a good guess

by several doctors. I just get excited when it sounds like some promising treatment is on the horizon.

Thanks again you are a treasure.

Dorothy Reinecke Fayetteville, GA

66 - DX VATS 7/05 - UIP 7/05

From: Breathe-Support [mailto:Breathe-Support ] On Behalf Of BruceSent: Thursday, November 05, 2009 10:22 AMTo: Breathe-Support Subject: Re: Perfinedone application made to the FDA

DorothyIt could take from months to forever as there is certainly no certaintyit will be approved. While I believe there's a good chance it will be,the facts are that the clinical trials ultimately showed little if anybenefit. While there was some advantage on one of the trials and not onthe other at the 36 week mark or so, the other trial had none. And bothtrials were consistent in showing no difference between the drug and theplacebo at 72 weeks in either function or death rate. In addition thedrug did have a wide range of side effects. I'm sorry that it didn'tturn out to be more than it did. I'm sure some doctors will widelypromote it's use, especially those who have received stipends forspeaking and consulting from Intermune and who also promoted Actimmune.The doctors in other areas, including those in my area, have expressedno interest in prescribing Pirfenidone.If it's approved, whether to take it or not will be an individualdecision. I just hope those with treatable conditions like NSIP and HPand Connective Tissue Disease caused PF don't forego other meds for thisone. Also, understand the test group was a very narrow demographic. Itwas mostly male, mostly older, all IPF (UIP with no known cause), andall very early stage.As much as we all want a treatment, we just need to be cautious. Thisapplication submission is running about 10 months behind Intermune'sprojection. Only time will tell how long the FDA will take or what theultimate outcome will be.The good news is that there are many many more trials underway for otherdrugs. So hope continues. Meanwhile there are things we all can continueto do to take care of ourselves the best possible, depending on ourindividual circumstances.>> Does anyone know how long it takes for approval - are we lookingat months, or years?>  Dorothy Reinecke>> UIP-7/05>>>> ________________________________> To: Breathe-Support > Sent: Thu, November 5, 2009 9:37:47 AM> Subject: Perfinedone application made to the FDA>> Â> I don't know if it's new news, but it is to me. Intermune hasapplied for a new drug approval for Perfinedone as a treatment forpulmonary fibrosis. I was alerted to that by the coalitionforpf.org I'll check with my pulmo guys and see what that means, ifanything. Some time ago I asked them about the drug, having heardit was in testing, and didn't get much of a rise out of anybody, so Idunno.>> Bill C. IPF 10/08> N.E. PA>

[Guest guest]

Dorothy,

Just curious...have you had a work up by a rheumatologist? At Duke or elsewhere? That is an avenue to pursue since treating the autoimmune disease often helps to control the fibrosis.

Just a thought. How are you doing these days? I was thinking of you while I was in Chattanooga and hoping you were ok.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

To: Breathe-Support Sent: Thu, November 5, 2009 7:47:27 PMSubject: RE: Re: Perfinedone application made to the FDA

Bruce,

thank you so much - I truly appreciate you sharing all this information that must have taken you so much research time.

I guess I just don't know enough about my own DX - UIP caused by autoimmune connective tissue, etc. and that is only a good guess

by several doctors. I just get excited when it sounds like some promising treatment is on the horizon.

Thanks again you are a treasure.

Dorothy Reinecke Fayetteville, GA

66 - DX VATS 7/05 - UIP 7/05

From: Breathe-Support@ yahoogroups. com [mailto:Breathe- Support@yahoogro ups.com] On Behalf Of BruceSent: Thursday, November 05, 2009 10:22 AMTo: Breathe-Support@ yahoogroups. comSubject: Re: Perfinedone application made to the FDA

DorothyIt could take from months to forever as there is certainly no certaintyit will be approved. While I believe there's a good chance it will be,the facts are that the clinical trials ultimately showed little if anybenefit. While there was some advantage on one of the trials and not onthe other at the 36 week mark or so, the other trial had none. And bothtrials were consistent in showing no difference between the drug and theplacebo at 72 weeks in either function or death rate. In addition thedrug did have a wide range of side effects. I'm sorry that it didn'tturn out to be more than it did. I'm sure some doctors will widelypromote it's use, especially those who have received stipends forspeaking and consulting from Intermune and who also promoted Actimmune.The doctors in other areas, including those in my area, have expressedno interest in prescribing Pirfenidone.If it's approved,

whether to take it or not will be an individualdecision. I just hope those with treatable conditions like NSIP and HPand Connective Tissue Disease caused PF don't forego other meds for thisone. Also, understand the test group was a very narrow demographic. Itwas mostly male, mostly older, all IPF (UIP with no known cause), andall very early stage.As much as we all want a treatment, we just need to be cautious. Thisapplication submission is running about 10 months behind Intermune'sprojection. Only time will tell how long the FDA will take or what theultimate outcome will be.The good news is that there are many many more trials underway for otherdrugs. So hope continues. Meanwhile there are things we all can continueto do to take care of ourselves the best possible, depending on ourindividual circumstances.>> Does anyone know how long it takes for approval - are we lookingat months, or years?>  Dorothy Reinecke>> UIP-7/05>>>> ____________ _________ _________ __> To: Breathe-Support@ yahoogroups. com> Sent: Thu, November 5, 2009 9:37:47 AM> Subject: Perfinedone application made to the FDA>> Â> I don't know if it's new news, but it is to me. Intermune hasapplied for a new drug approval for Perfinedone as a treatment forpulmonary fibrosis. I was alerted to that by the

coalitionforpf.org I'll check with my pulmo guys and see what that means, ifanything. Some time ago I asked them about the drug, having heardit was in testing, and didn't get much of a rise out of anybody, so Idunno.>> Bill C. IPF 10/08> N.E. PA>

[Guest guest]

Beth,

I thought about all of you last weekend in Chattanooga - I just could not make it this time -

To answer your question:

I have had several work up by rheumatologists both in Atlanta in 7/05; then again at Duke and most recently in April back in Atlanta. No RA. My sed rate see saws up and down continually. The problem seems to be the doctors are sure there is an autoimmune disease, but they cannot get a label for it.

The current diagnosis at the Duke ILD Clinic is "diffuse parenchymal lung disease most consistent with a connective tissue disease related to fibrotic lung disease" which I think equals "we don't know"

I have had tons of tests for rather strong muscle pain that began in December of 2008. My upper arms, thighs, feet /bottom of my feet and hands ache/pain most of the time. Trying to reach back to slip on a jacket or secure a bra is just awful. But again no cause found and I must say Duke has done more lab tests than I can count. We have tried increasing Prednisone up to 30 mg/day but it makes no difference. Also stopped the Cellcept for 2 months because this started a month after I started the Cellcept but that made no difference either. This is all terribly uncomfortable but so far bearable with 3/4 Advil several times a day, and of course still on Cellcept 1500/mg twice a day.

I don't mean to sound like a chronic complainer - I am grateful that the past six months I seem to be holding my own. I don't feel like my pulmonary function is down from August. I am able to get out and work with customers and show property - I do make them aware that I do not go up or down stairs :-) and so far that works fine. The only negative that I observe is that I am more tired all the time. This is probably a lot more information than you wanted, but it is all I know about me - and I am happy for any advice/input you may have to offer. There just isn't anybody to talk to or compare notes with except all you wonderful people in the "Air Family" of ours.

Take care, and thanks for asking

Dorothy Reinecke Fayetteville, GA

66 - DX VATS 7/05 - UIP 7/05

From: Breathe-Support [mailto:Breathe-Support ] On Behalf Of BethSent: Thursday, November 05, 2009 8:04 PMTo: Breathe-Support Subject: Re: Re: Perfinedone application made to the FDA

Dorothy,

Just curious...have you had a work up by a rheumatologist? At Duke or elsewhere? That is an avenue to pursue since treating the autoimmune disease often helps to control the fibrosis.

Just a thought. How are you doing these days? I was thinking of you while I was in Chattanooga and hoping you were ok.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

From: Dorothy Reinecke <atlantadorothy (AT) att (DOT) net>To: Breathe-Support Sent: Thu, November 5, 2009 7:47:27 PMSubject: RE: Re: Perfinedone application made to the FDA

Bruce,

thank you so much - I truly appreciate you sharing all this information that must have taken you so much research time.

I guess I just don't know enough about my own DX - UIP caused by autoimmune connective tissue, etc. and that is only a good guess

by several doctors. I just get excited when it sounds like some promising treatment is on the horizon.

Thanks again you are a treasure.

Dorothy Reinecke Fayetteville, GA

66 - DX VATS 7/05 - UIP 7/05

From: Breathe-Support@ yahoogroups. com [mailto:Breathe- Support@yahoogro ups.com] On Behalf Of BruceSent: Thursday, November 05, 2009 10:22 AMTo: Breathe-Support@ yahoogroups. comSubject: Re: Perfinedone application made to the FDA

DorothyIt could take from months to forever as there is certainly no certaintyit will be approved. While I believe there's a good chance it will be,the facts are that the clinical trials ultimately showed little if anybenefit. While there was some advantage on one of the trials and not onthe other at the 36 week mark or so, the other trial had none. And bothtrials were consistent in showing no difference between the drug and theplacebo at 72 weeks in either function or death rate. In addition thedrug did have a wide range of side effects. I'm sorry that it didn'tturn out to be more than it did. I'm sure some doctors will widelypromote it's use, especially those who have received stipends forspeaking and consulting from Intermune and who also promoted Actimmune.The doctors in other areas, including those in my area, have expressedno interest in prescribing Pirfenidone.If it's approved, whether to take it or not will be an individualdecision. I just hope those with treatable conditions like NSIP and HPand Connective Tissue Disease caused PF don't forego other meds for thisone. Also, understand the test group was a very narrow demographic. Itwas mostly male, mostly older, all IPF (UIP with no known cause), andall very early stage.As much as we all want a treatment, we just need to be cautious. Thisapplication submission is running about 10 months behind Intermune'sprojection. Only time will tell how long the FDA will take or what theultimate outcome will be.The good news is that there are many many more trials underway for otherdrugs. So hope continues. Meanwhile there are things we all can continueto do to take care of ourselves the best possible, depending on ourindividual circumstances.>> Does anyone know how long it takes for approval - are we lookingat months, or years?>  Dorothy Reinecke>> UIP-7/05>>>> ____________ _________ _________ __> To: Breathe-Support@ yahoogroups. com> Sent: Thu, November 5, 2009 9:37:47 AM> Subject: Perfinedone application made to the FDA>> Â> I don't know if it's new news, but it is to me. Intermune hasapplied for a new drug approval for Perfinedone as a treatment forpulmonary fibrosis. I was alerted to that by the coalitionforpf.org I'll check with my pulmo guys and see what that means, ifanything. Some time ago I asked them about the drug, having heardit was in testing, and didn't get much of a rise out of anybody, so Idunno.>> Bill C. IPF 10/08> N.E. PA>

[Guest guest]

Thanks so much bruce, I will keep going to duke every three months and keep testing.One clarification to my not - I wrote the diagnosis from my visit to duke in may. But the diagnosis from my biopsy pathology report is UIP. When I went to mass general in august 'o5 I brought my slides their pathology also concluded UIP. So what do you understand - has duke changed the diagnosis or are they just not saying UIP? Do you think I should as for clarification when I go back to duke?Many thanks for your inputDorothy Sent via BlackBerry by AT&TDate: Fri, 06 Nov 2009 03:42:44 -0000To: <Breathe-Support >Subject: Re: Perfinedone application made to the FDA DorothyYour situation isn't that odd or strange, believe it or not. I wouldrecommend continued rheumatological labs and exams based on what yousay, probably as frequently as every three months. In fact, I'm in asomewhat similar situation and my rheumatologist insists on every twomonths, has made referrals for more opinions, and I may end up going toDuke. Now, one would say, why more labs. Because what might today beclassified as Undifferentiated Connective Tissue Disease can thenmanifest itself. Why hasn't it so far? Perhaps because its often delayedor perhaps because the meds you've been on have kept it from doing so.The meds may have treated the connective tissue disease even though noone yet knows which one you have.The muscle pains certainly sound like a Myositis such as Dermatomyositisor Polymyositis so based on that you might find a muscle biopsyrecommended next.The main thing I'd say is that the possibility of an underlyingconnective tissue disease could still be in your favor as to delayingthe progression of the fibrosis. Perhaps it will become more obviousover time and be treatable. Or, perhaps you've already been treating it.It's worth continuing to chase it. It took several years for Beth'sto be to the point they could diagnose it confidently. There's verystrong argument that I might have one but no one has been able todiagnose it yet. My rheumatologist has suggested many different biopsiesat one time or another but I'm willing to wait (kidney biopsy, musclebiopsy, lymph node biopsy, skin biopsy, lip biopsy, another lungbiopsy.....lol). I do believe that at some point one of the labs thatjumps back and forth may just take off upward or some additional symptomthat will clear things up might emerge.So, I think the following for myself personally:1-As long as it doesn't manifest itself, it's at least somewhat undercontrol so I won't worry too much.2-However, I will continue to monitor and see doctors so that if andwhen it does manifest itself, it will be discovered and if any treatmentis appropriate, I can get it.I also see you as having one distinct advantage over me. You have an ILDbut it is not identified as UIP and based on the treatments may well beone of the others that is treatable and is quite commonly found to havean underlying connective tissue disease. In my case, mine is determinedfirmly to be UIP and so that probability lessened.Don't give up. Time may be here to repeat a major workup as you have newmore detectable symptoms.> >> > Does anyone know how long it takes for approval - are we looking> at months, or years?> >  Dorothy Reinecke> >> > UIP-7/05> >> >> >> >________________________________> > From: " Paradis3@ . " Paradis3@> > To: Breathe-Support@ yahoogroups.> <mailto:Breathe-Support%40yahoogroups.com> com> > Sent: Thu, November 5, 2009 9:37:47 AM> > Subject: Perfinedone application made to the FDA> >> > Â> > I don't know if it's new news, but it is to me. Intermune has> applied for a new drug approval for Perfinedone as a treatment for> pulmonary fibrosis. I was alerted to that by the coalitionforpf.> org I'll check with my pulmo guys and see what that means, if> anything. Some time ago I asked them about the drug, having heard> it was in testing, and didn't get much of a rise out of anybody, so I> dunno.> >> > Bill C. IPF 10/08> > N.E. PA> >>

[Guest guest]

Dorothy,

My memory is not terrific so forgive me in advance. Have your slides been looked at by a Duke pathologist? I thought they had but I could be thinking of someone else. If they haven't I would strongly recommend that being done.

Bruce gave you lots of good info so I won't repeat it here. Let me know if there's anything I can do for you Dorothy and let's make sure we get together again when you're up here in December.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

To: Breathe-Support Sent: Thu, November 5, 2009 11:11:45 PMSubject: Re: Re: Perfinedone application made to the FDA

Thanks so much bruce,I will keep going to duke every three months and keep testing.One clarification to my not - I wrote the diagnosis from my visit to duke in may. But the diagnosis from my biopsy pathology report is UIP. When I went to mass general in august 'o5 I brought my slides their pathology also concluded UIP. So what do you understand - has duke changed the diagnosis or are they just not saying UIP? Do you think I should as for clarification when I go back to duke?Many thanks for your inputDorothy

Sent via BlackBerry by AT & T

From: "Bruce" <brucemoreland@ gmail.com>

Date: Fri, 06 Nov 2009 03:42:44 -0000

To: <Breathe-Support@ yahoogroups. com>

Subject: Re: Perfinedone application made to the FDA

DorothyYour situation isn't that odd or strange, believe it or not. I wouldrecommend continued rheumatological labs and exams based on what yousay, probably as frequently as every three months. In fact, I'm in asomewhat similar situation and my rheumatologist insists on every twomonths, has made referrals for more opinions, and I may end up going toDuke. Now, one would say, why more labs. Because what might today beclassified as Undifferentiated Connective Tissue Disease can thenmanifest itself. Why hasn't it so far? Perhaps because its often delayedor perhaps because the meds you've been on have kept it from doing so.The meds may have treated the connective tissue disease even though noone yet knows which one you have.The muscle pains certainly sound like a Myositis such as Dermatomyositisor Polymyositis so based on that you might find a muscle biopsyrecommended next.The main

thing I'd say is that the possibility of an underlyingconnective tissue disease could still be in your favor as to delayingthe progression of the fibrosis. Perhaps it will become more obviousover time and be treatable. Or, perhaps you've already been treating it.It's worth continuing to chase it. It took several years for Beth'sto be to the point they could diagnose it confidently. There's verystrong argument that I might have one but no one has been able todiagnose it yet. My rheumatologist has suggested many different biopsiesat one time or another but I'm willing to wait (kidney biopsy, musclebiopsy, lymph node biopsy, skin biopsy, lip biopsy, another lungbiopsy.....lol) . I do believe that at some point one of the labs thatjumps back and forth may just take off upward or some additional symptomthat will clear things up might emerge.So, I think the following for myself personally:1-As

long as it doesn't manifest itself, it's at least somewhat undercontrol so I won't worry too much.2-However, I will continue to monitor and see doctors so that if andwhen it does manifest itself, it will be discovered and if any treatmentis appropriate, I can get it.I also see you as having one distinct advantage over me. You have an ILDbut it is not identified as UIP and based on the treatments may well beone of the others that is treatable and is quite commonly found to havean underlying connective tissue disease. In my case, mine is determinedfirmly to be UIP and so that probability lessened.Don't give up. Time may be here to repeat a major workup as you have newmore detectable symptoms.> >> > Does anyone know how long it takes for approval - are we looking> at months, or years?> >  Dorothy Reinecke> >> >

UIP-7/05> >> >> >> >____________ _________ _________ __> > From: "Paradis3@ ." Paradis3@> > To: Breathe-Support@ yahoogroups.> <mailto:Breathe- Support%40yahoog roups.com> com> > Sent: Thu, November 5, 2009 9:37:47 AM> > Subject: Perfinedone application made to the FDA> >> > Â> > I don't know if it's new news, but it is to me. Intermune has> applied for a new drug approval for Perfinedone as a treatment for> pulmonary fibrosis. I was alerted to that by the coalitionforpf.> org I'll check with my pulmo guys and see what that means, if> anything. Some time ago I asked them about the drug, having heard> it was in testing, and didn't get much of a rise out of anybody, so I> dunno.> >> > Bill C. IPF 10/08> > N.E. PA>

>>

[Guest guest]

Many thanks Bruce - Dorothy Reinecke

To: Breathe-Support Sent: Fri, November 6, 2009 2:27:09 AMSubject: Re: Perfinedone application made to the FDA

DorothyBy what you quoted, Duke isn't saying one way or another....just sayingILD. Now that's just not being specific, not necessarily changing orconfirming.> > >> > > Does anyone know how long it takes for approval - are welooking> > at months, or years?> > >  Dorothy Reinecke> > >> > > UIP-7/05> > >> > >> > >> > >___________ _________ _________ ___> > > From: "Paradis3@ ." Paradis3@> > > To:

Breathe-Support@ yahoogroups.> > <mailto:Breathe- Support%40yahoog roups.com> com> > > Sent: Thu, November 5, 2009 9:37:47 AM> > > Subject: Perfinedone application made to the FDA> > >> > > Â> > > I don't know if it's new news, but it is to me. Intermune has> > applied for a new drug approval for Perfinedone as a treatment for> > pulmonary fibrosis. I was alerted to that by the coalitionforpf.> > org I'll check with my pulmo guys and see what that means, if> > anything. Some time ago I asked them about the drug, havingheard> > it was in testing, and didn't get much of a rise out of anybody, soI> > dunno.> > >> > > Bill C. IPF 10/08> > > N.E. PA> > >> >>

[Guest guest]

Beth,

No - Dr. Noble never asked for the slides. On my first appointment he asked for all the data from 18 months at Mass General, labs, CTs, etc. but not the slides from the biopsy. There is no doubt from the CT scans past and current that there is plenty of fibrosis,honeycombing, etc. and enlarging pulmonary artery but not enough yet to qualify as full blown PH. I did have a right heart cath which also confirmed 'mild' PH, but not requiring meds yet. Knowing Dr. Noble as long as I do now, I think he is not so interested in a label as trying to address the condition & slow progression. Does this make sense. to you? I may just ask if he would like me to bring the slides - at this point it couldn't hurt, right?

I would love to plan to meet when I come to Durham for my Dec. 17 appointment. We are flying in on the 16th and staying at the Residence Inn this time.

We have always stayed at Millennium since Aug. 07 - just decided on a change of scenery :-).

Dorothy Reinecke

From: Breathe-Support [mailto:Breathe-Support ] On Behalf Of BethSent: Friday, November 06, 2009 7:51 AMTo: Breathe-Support Subject: Re: Re: Perfinedone application made to the FDA

Dorothy,

My memory is not terrific so forgive me in advance. Have your slides been looked at by a Duke pathologist? I thought they had but I could be thinking of someone else. If they haven't I would strongly recommend that being done.

Bruce gave you lots of good info so I won't repeat it here. Let me know if there's anything I can do for you Dorothy and let's make sure we get together again when you're up here in December.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

From: "atlantadorothy (AT) att (DOT) net" <atlantadorothy (AT) att (DOT) net>To: Breathe-Support Sent: Thu, November 5, 2009 11:11:45 PMSubject: Re: Re: Perfinedone application made to the FDA

Thanks so much bruce,I will keep going to duke every three months and keep testing.One clarification to my not - I wrote the diagnosis from my visit to duke in may. But the diagnosis from my biopsy pathology report is UIP. When I went to mass general in august 'o5 I brought my slides their pathology also concluded UIP. So what do you understand - has duke changed the diagnosis or are they just not saying UIP? Do you think I should as for clarification when I go back to duke?Many thanks for your inputDorothy

Sent via BlackBerry by AT & T

From: "Bruce" <brucemoreland@ gmail.com>

Date: Fri, 06 Nov 2009 03:42:44 -0000

To: <Breathe-Support@ yahoogroups. com>

Subject: Re: Perfinedone application made to the FDA

DorothyYour situation isn't that odd or strange, believe it or not. I wouldrecommend continued rheumatological labs and exams based on what yousay, probably as frequently as every three months. In fact, I'm in asomewhat similar situation and my rheumatologist insists on every twomonths, has made referrals for more opinions, and I may end up going toDuke. Now, one would say, why more labs. Because what might today beclassified as Undifferentiated Connective Tissue Disease can thenmanifest itself. Why hasn't it so far? Perhaps because its often delayedor perhaps because the meds you've been on have kept it from doing so.The meds may have treated the connective tissue disease even though noone yet knows which one you have.The muscle pains certainly sound like a Myositis such as Dermatomyositisor Polymyositis so based on that you might find a muscle biopsyrecommended next.The main thing I'd say is that the possibility of an underlyingconnective tissue disease could still be in your favor as to delayingthe progression of the fibrosis. Perhaps it will become more obviousover time and be treatable. Or, perhaps you've already been treating it.It's worth continuing to chase it. It took several years for Beth'sto be to the point they could diagnose it confidently. There's verystrong argument that I might have one but no one has been able todiagnose it yet. My rheumatologist has suggested many different biopsiesat one time or another but I'm willing to wait (kidney biopsy, musclebiopsy, lymph node biopsy, skin biopsy, lip biopsy, another lungbiopsy.....lol) . I do believe that at some point one of the labs thatjumps back and forth may just take off upward or some additional symptomthat will clear things up might emerge.So, I think the following for myself personally:1-As long as it doesn't manifest itself, it's at least somewhat undercontrol so I won't worry too much.2-However, I will continue to monitor and see doctors so that if andwhen it does manifest itself, it will be discovered and if any treatmentis appropriate, I can get it.I also see you as having one distinct advantage over me. You have an ILDbut it is not identified as UIP and based on the treatments may well beone of the others that is treatable and is quite commonly found to havean underlying connective tissue disease. In my case, mine is determinedfirmly to be UIP and so that probability lessened.Don't give up. Time may be here to repeat a major workup as you have newmore detectable symptoms.> >> > Does anyone know how long it takes for approval - are we looking> at months, or years?> >  Dorothy Reinecke> >> > UIP-7/05> >> >> >> >____________ _________ _________ __> > From: "Paradis3@ ." Paradis3@> > To: Breathe-Support@ yahoogroups.> <mailto:Breathe- Support%40yahoog roups.com> com> > Sent: Thu, November 5, 2009 9:37:47 AM> > Subject: Perfinedone application made to the FDA> >> > Â> > I don't know if it's new news, but it is to me. Intermune has> applied for a new drug approval for Perfinedone as a treatment for> pulmonary fibrosis. I was alerted to that by the coalitionforpf.> org I'll check with my pulmo guys and see what that means, if> anything. Some time ago I asked them about the drug, having heard> it was in testing, and didn't get much of a rise out of anybody, so I> dunno.> >> > Bill C. IPF 10/08> > N.E. PA> >>

[Guest guest]

Dorothy:

I am new to the group and have little knowledge of your case. 

Having said that, I will offer a few comments for whatever you think they are

worth.

IPF is extremely difficult to diagnose as it gets confused with

other diseases such as lupus.  (The book on interstitial lung diseases is a few

inches thick – there are many variants of them.)  Most drug trials

require a lung biopsy as proof of IPF.  Even then, the slides are difficult to

diagnose.  It takes special training to identify the presence of IPF.  In my

case my slides first went to the pathology department of my local hospital and

then to the Mayo Clinic where one of the best doctors in the county gave a

second opinion.  From there my slides went to Dr. Brown at the Jewish

Medical Center in Denver for a third opinion.  Do you recall the case a few

years ago when a man with a rare form of TB was arrested for flying around the

world?  The CDC in Atlanta sent his data to Dr. Brown for analysis to determine

the nature of that man’s TB.  Jewish Medical is considered by many to be

the best pulmonary center in the world.

Dr. Brown confirmed that I have IPF and recommended that I

volunteer for the experimental trial of Bosentan (Tracleer) and I did.  Since I

had sarcoidosis forty years ago my slides were sent to Switzerland for review

by the drug manufacturer.  They accepted me as one of the 600 participants in

the Phase III study.

Bosentan (Tracleer) was approved in the US a number of years ago

for use in treating PH.  In the intervening years it was discovered that PH

patients who also had IPF appeared to be benefiting from the drug.  A study was

run and in the Phase II trial it was seen that patients who were in the early

stages of IPF and having but “slight honeycombing” showed a 68%

slowdown in the progression of their IPF.  This led to the Phase III study of

600 patients with slight honeycombing.

This drug costs $30,000 per year.  If your insurance will pay

for it or you can afford it yourself, your pulmonologist could possibly

prescribe it to treat your PH.  That way you would also have the possibility

that the drug would slow down the progression of your IPF.

Just a suggestion for you to consider.

Jack Mc sdale

From:

Breathe-Support [mailto:Breathe-Support ] On

Behalf Of Dorothy Reinecke

Sent: Friday, November 06, 2009 7:20 AM

To: Breathe-Support

Subject: RE: Re: Perfinedone application made to the

FDA

Beth,

No - Dr. Noble never asked for the slides. On my first

appointment he asked for all the data from 18 months at Mass General, labs,

CTs, etc. but not the slides from the biopsy. There is no doubt from the

CT scans past and current that there is plenty of fibrosis,honeycombing,

etc. and enlarging pulmonary artery but not enough yet to qualify as full

blown PH. I did have a right heart cath which also confirmed 'mild' PH,

but not requiring meds yet. Knowing Dr. Noble as long as I do now, I think he

is not so interested in a label as trying to address the condition & slow

progression. Does this make sense. to you? I may just ask if he

would like me to bring the slides - at this point it couldn't hurt, right?

I would love to plan to meet when I come to Durham for my Dec. 17

appointment. We are flying in on the 16th and staying at the Residence

Inn this time.

We have always stayed at Millennium since Aug. 07 - just decided on

a change of scenery :-).

Dorothy Reinecke

From: Breathe-Support [mailto:Breathe-Support ]

On Behalf Of Beth

Sent: Friday, November 06, 2009 7:51 AM

To: Breathe-Support

Subject: Re: Re: Perfinedone application made to the

FDA

Dorothy,

My

memory is not terrific so forgive me in advance. Have your slides been looked

at by a Duke pathologist? I thought they had but I could

be thinking of someone else. If they haven't I would strongly recommend

that being done.

Bruce

gave you lots of good info so I won't repeat it here. Let me know if

there's anything I can do for you Dorothy and let's make sure we get together

again when you're up here in December.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

From:

" atlantadorothy@... "

To: Breathe-Support

Sent: Thu, November 5, 2009 11:11:45 PM

Subject: Re: Re: Perfinedone application made to the

FDA

Thanks so much bruce,

I will keep going to duke every three months and keep testing.

One clarification to my not - I wrote the diagnosis from my visit to duke in

may. But the diagnosis from my biopsy pathology report is UIP. When I went to

mass general in august 'o5 I brought my slides their pathology also concluded

UIP.

So what do you understand - has duke changed the diagnosis or are they just not

saying UIP?

Do you think I should as for clarification when I go back to duke?

Many thanks for your input

Dorothy

Sent via BlackBerry by AT & T

From: " Bruce " <brucemoreland@

gmail.com>

Date: Fri, 06 Nov 2009 03:42:44 -0000

To: <Breathe-Support@ yahoogroups. com>

Subject: Re: Perfinedone

application made to the FDA

Dorothy

Your situation isn't that odd or strange, believe it or not. I would

recommend continued rheumatological labs and exams based on what you

say, probably as frequently as every three months. In fact, I'm in a

somewhat similar situation and my rheumatologist insists on every two

months, has made referrals for more opinions, and I may end up going to

Duke. Now, one would say, why more labs. Because what might today be

classified as Undifferentiated Connective Tissue Disease can then

manifest itself. Why hasn't it so far? Perhaps because its often delayed

or perhaps because the meds you've been on have kept it from doing so.

The meds may have treated the connective tissue disease even though no

one yet knows which one you have.

The muscle pains certainly sound like a Myositis such as Dermatomyositis

or Polymyositis so based on that you might find a muscle biopsy

recommended next.

The main thing I'd say is that the possibility of an underlying

connective tissue disease could still be in your favor as to delaying

the progression of the fibrosis. Perhaps it will become more obvious

over time and be treatable. Or, perhaps you've already been treating it.

It's worth continuing to chase it. It took several years for Beth's

to be to the point they could diagnose it confidently. There's very

strong argument that I might have one but no one has been able to

diagnose it yet. My rheumatologist has suggested many different biopsies

at one time or another but I'm willing to wait (kidney biopsy, muscle

biopsy, lymph node biopsy, skin biopsy, lip biopsy, another lung

biopsy.....lol) . I do believe that at some point one of the labs that

jumps back and forth may just take off upward or some additional symptom

that will clear things up might emerge.

So, I think the following for myself personally:

1-As long as it doesn't manifest itself, it's at least somewhat under

control so I won't worry too much.

2-However, I will continue to monitor and see doctors so that if and

when it does manifest itself, it will be discovered and if any treatment

is appropriate, I can get it.

I also see you as having one distinct advantage over me. You have an ILD

but it is not identified as UIP and based on the treatments may well be

one of the others that is treatable and is quite commonly found to have

an underlying connective tissue disease. In my case, mine is determined

firmly to be UIP and so that probability lessened.

Don't give up. Time may be here to repeat a major workup as you have new

more detectable symptoms.

> >

> > Does anyone know how long it takes for approval - are we looking

> at months, or years?

> > Â Dorothy Reinecke

> >

> > UIP-7/05

> >

> >

> >

> >____________ _________ _________ __

> > From: " Paradis3@ . " Paradis3@

> > To: Breathe-Support@ yahoogroups.

> <mailto:Breathe- Support%40yahoog roups.com> com

> > Sent: Thu, November 5, 2009 9:37:47 AM

> > Subject: Perfinedone application made to the FDA

> >

> > Â

> > I don't know if it's new news, but it is to me. Intermune has

> applied for a new drug approval for Perfinedone as a treatment for

> pulmonary fibrosis. I was alerted to that by the coalitionforpf.

> org I'll check with my pulmo guys and see what that means, if

> anything. Some time ago I asked them about the drug, having heard

> it was in testing, and didn't get much of a rise out of anybody, so I

> dunno.

> >

> > Bill C. IPF 10/08

> > N.E. PA

> >

>

[Guest guest]

I think he is not so interested in a label as trying to address the condition & slow progression

Dorothy

yes this makes a lot of sense to me

Pink Joyce R (IPF 3/06) IFA 5/09 Pennsylvania

Donate Life Listed 1/09 Inactive 4/09

www.transplantfund.org---

Subject: RE: Re: Perfinedone application made to the FDATo: Breathe-Support Date: Friday, November 6, 2009, 9:20 AM

Beth,

No - Dr. Noble never asked for the slides. On my first appointment he asked for all the data from 18 months at Mass General, labs, CTs, etc. but not the slides from the biopsy. There is no doubt from the CT scans past and current that there is plenty of fibrosis,honeycombi ng, etc. and enlarging pulmonary artery but not enough yet to qualify as full blown PH. I did have a right heart cath which also confirmed 'mild' PH, but not requiring meds yet. Knowing Dr. Noble as long as I do now, I think he is not so interested in a label as trying to address the condition & slow progression. Does this make sense. to you? I may just ask if he would like me to bring the slides - at this point it couldn't hurt, right?

I would love to plan to meet when I come to Durham for my Dec. 17 appointment. We are flying in on the 16th and staying at the Residence Inn this time.

We have always stayed at Millennium since Aug. 07 - just decided on a change of scenery :-).

Dorothy Reinecke

From: Breathe-Support@ yahoogroups. com [mailto:Breathe- Support@yahoogro ups.com] On Behalf Of BethSent: Friday, November 06, 2009 7:51 AMTo: Breathe-Support@ yahoogroups. comSubject: Re: Re: Perfinedone application made to the FDA

Dorothy,

My memory is not terrific so forgive me in advance. Have your slides been looked at by a Duke pathologist? I thought they had but I could be thinking of someone else. If they haven't I would strongly recommend that being done.

Bruce gave you lots of good info so I won't repeat it here. Let me know if there's anything I can do for you Dorothy and let's make sure we get together again when you're up here in December.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

From: "atlantadorothy@ att.net" <atlantadorothy@ att.net>To: Breathe-Support@ yahoogroups. comSent: Thu, November 5, 2009 11:11:45 PMSubject: Re: Re: Perfinedone application made to the FDA

Thanks so much bruce,I will keep going to duke every three months and keep testing.One clarification to my not - I wrote the diagnosis from my visit to duke in may. But the diagnosis from my biopsy pathology report is UIP. When I went to mass general in august 'o5 I brought my slides their pathology also concluded UIP. So what do you understand - has duke changed the diagnosis or are they just not saying UIP? Do you think I should as for clarification when I go back to duke?Many thanks for your inputDorothy

Sent via BlackBerry by AT & T

From: "Bruce" <brucemoreland@ gmail.com>

Date: Fri, 06 Nov 2009 03:42:44 -0000

To: <Breathe-Support@ yahoogroups. com>

Subject: Re: Perfinedone application made to the FDA

DorothyYour situation isn't that odd or strange, believe it or not. I wouldrecommend continued rheumatological labs and exams based on what yousay, probably as frequently as every three months. In fact, I'm in asomewhat similar situation and my rheumatologist insists on every twomonths, has made referrals for more opinions, and I may end up going toDuke. Now, one would say, why more labs. Because what might today beclassified as Undifferentiated Connective Tissue Disease can thenmanifest itself. Why hasn't it so far? Perhaps because its often delayedor perhaps because the meds you've been on have kept it from doing so.The meds may have treated the connective tissue disease even though noone yet knows which one you have.The muscle pains certainly sound like a Myositis such as Dermatomyositisor Polymyositis so based on that you might find a muscle biopsyrecommended next.The main

thing I'd say is that the possibility of an underlyingconnective tissue disease could still be in your favor as to delayingthe progression of the fibrosis. Perhaps it will become more obviousover time and be treatable. Or, perhaps you've already been treating it.It's worth continuing to chase it. It took several years for Beth'sto be to the point they could diagnose it confidently. There's verystrong argument that I might have one but no one has been able todiagnose it yet. My rheumatologist has suggested many different biopsiesat one time or another but I'm willing to wait (kidney biopsy, musclebiopsy, lymph node biopsy, skin biopsy, lip biopsy, another lungbiopsy.....lol) . I do believe that at some point one of the labs thatjumps back and forth may just take off upward or some additional symptomthat will clear things up might emerge.So, I think the following for myself personally:1-As

long as it doesn't manifest itself, it's at least somewhat undercontrol so I won't worry too much.2-However, I will continue to monitor and see doctors so that if andwhen it does manifest itself, it will be discovered and if any treatmentis appropriate, I can get it.I also see you as having one distinct advantage over me. You have an ILDbut it is not identified as UIP and based on the treatments may well beone of the others that is treatable and is quite commonly found to havean underlying connective tissue disease. In my case, mine is determinedfirmly to be UIP and so that probability lessened.Don't give up. Time may be here to repeat a major workup as you have newmore detectable symptoms.> >> > Does anyone know how long it takes

for approval - are we looking> at months, or years?> >  Dorothy Reinecke> >> > UIP-7/05> >> >> >> >____________ _________ _________ __> > From: "Paradis3@ ." Paradis3@> > To: Breathe-Support@ yahoogroups.> <mailto:Breathe- Support%40yahoog roups.com> com> > Sent: Thu, November 5, 2009 9:37:47 AM> > Subject: Perfinedone application made to the FDA> >> > Â> > I don't know if it's new news, but it is to me. Intermune has> applied for a new drug approval for Perfinedone as a treatment for> pulmonary fibrosis. I was alerted to that by the coalitionforpf.> org I'll check with my pulmo guys and see what that means, if> anything. Some time ago I asked them about the drug, having heard> it was in testing, and didn't get much of a rise out

of anybody, so I> dunno.> >> > Bill C. IPF 10/08> > N.E. PA> >>

[Guest guest]

Jack,

Thanks so much - I will certainly ask the question next month when I see the doctor. So far my PH number has been a few points below what insurance will cover. I guess I need to get worse before i can get better

:-(. Seems to be the case in so many of our situations.

Appreciate you taking the time to share the information. Dorothy Reinecke

Fayetteville, GA

To: Breathe-Support Sent: Fri, November 6, 2009 11:53:03 AMSubject: RE: Re: Perfinedone application made to the FDA

Dorothy:

I am new to the group and have little knowledge of your case. Having said that, I will offer a few comments for whatever you think they are worth.

IPF is extremely difficult to diagnose as it gets confused with other diseases such as lupus. (The book on interstitial lung diseases is a few inches thick – there are many variants of them.) Most drug trials require a lung biopsy as proof of IPF. Even then, the slides are difficult to diagnose. It takes special training to identify the presence of IPF. In my case my slides first went to the pathology department of my local hospital and then to the Mayo Clinic where one of the best doctors in the county gave a second opinion. From there my slides went to Dr. Brown at the Jewish Medical Center in Denver for a third opinion. Do you recall the case a few years ago when a man with a rare form of TB was arrested for flying around the world? The CDC in Atlanta sent his data to Dr. Brown for analysis to determine the nature of that man’s

TB. Jewish Medical is considered by many to be the best pulmonary center in the world.

Dr. Brown confirmed that I have IPF and recommended that I volunteer for the experimental trial of Bosentan (Tracleer) and I did. Since I had sarcoidosis forty years ago my slides were sent to Switzerland for review by the drug manufacturer. They accepted me as one of the 600 participants in the Phase III study.

Bosentan (Tracleer) was approved in the US a number of years ago for use in treating PH. In the intervening years it was discovered that PH patients who also had IPF appeared to be benefiting from the drug. A study was run and in the Phase II trial it was seen that patients who were in the early stages of IPF and having but “slight honeycombing†showed a 68% slowdown in the progression of their IPF. This led to the Phase III study of 600 patients with slight honeycombing.

This drug costs $30,000 per year. If your insurance will pay for it or you can afford it yourself, your pulmonologist could possibly prescribe it to treat your PH. That way you would also have the possibility that the drug would slow down the progression of your IPF.

Just a suggestion for you to consider.

Jack Mc sdale

From: Breathe-Support@ yahoogroups. com [mailto:Breathe- Support@yahoogro ups.com] On Behalf Of Dorothy ReineckeSent: Friday, November 06, 2009 7:20 AMTo: Breathe-Support@ yahoogroups. comSubject: RE: Re: Perfinedone application made to the FDA

Beth,

No - Dr. Noble never asked for the slides.. On my first appointment he asked for all the data from 18 months at Mass General, labs, CTs, etc. but not the slides from the biopsy. There is no doubt from the CT scans past and current that there is plenty of fibrosis,honeycombi ng, etc. and enlarging pulmonary artery but not enough yet to qualify as full blown PH. I did have a right heart cath which also confirmed 'mild' PH, but not requiring meds yet. Knowing Dr. Noble as long as I do now, I think he is not so interested in a label as trying to address the condition & slow progression. Does this make sense. to you? I may just ask if he would like me to bring the slides - at this point it couldn't hurt, right?

I would love to plan to meet when I come to Durham for my Dec. 17 appointment. We are flying in on the 16th and staying at the Residence Inn this time.

We have always stayed at Millennium since Aug. 07 - just decided on a change of scenery :-).

Dorothy Reinecke

From: Breathe-Support@ yahoogroups. com [mailto:Breathe- Support@yahoogro ups.com] On Behalf Of BethSent: Friday, November 06, 2009 7:51 AMTo: Breathe-Support@ yahoogroups. comSubject: Re: Re: Perfinedone application made to the FDA

Dorothy,

My memory is not terrific so forgive me in advance. Have your slides been looked at by a Duke pathologist? I thought they had but I could be thinking of someone else. If they haven't I would strongly recommend that being done.

Bruce gave you lots of good info so I won't repeat it here. Let me know if there's anything I can do for you Dorothy and let's make sure we get together again when you're up here in December.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

From: "atlantadorothy@ att.net" <atlantadorothy@ att.net>To: Breathe-Support@ yahoogroups. comSent: Thu, November 5, 2009 11:11:45 PMSubject: Re: Re: Perfinedone application made to the FDA

Thanks so much bruce,I will keep going to duke every three months and keep testing.One clarification to my not - I wrote the diagnosis from my visit to duke in may. But the diagnosis from my biopsy pathology report is UIP. When I went to mass general in august 'o5 I brought my slides their pathology also concluded UIP. So what do you understand - has duke changed the diagnosis or are they just not saying UIP? Do you think I should as for clarification when I go back to duke?Many thanks for your inputDorothy

Sent via BlackBerry by AT & T

From: "Bruce" <brucemoreland@ gmail.com>

Date: Fri, 06 Nov 2009 03:42:44 -0000

To: <Breathe-Support@ yahoogroups. com>

Subject: Re: Perfinedone application made to the FDA

DorothyYour situation isn't that odd or strange, believe it or not. I wouldrecommend continued rheumatological labs and exams based on what yousay, probably as frequently as every three months. In fact, I'm in asomewhat similar situation and my rheumatologist insists on every twomonths, has made referrals for more opinions, and I may end up going toDuke. Now, one would say, why more labs. Because what might today beclassified as Undifferentiated Connective Tissue Disease can thenmanifest itself. Why hasn't it so far? Perhaps because its often delayedor perhaps because the meds you've been on have kept it from doing so.The meds may have treated the connective tissue disease even though noone yet knows which one you have.The muscle pains certainly sound like a Myositis such as Dermatomyositisor Polymyositis so based on that you might find a muscle biopsyrecommended next.The main

thing I'd say is that the possibility of an underlyingconnective tissue disease could still be in your favor as to delayingthe progression of the fibrosis. Perhaps it will become more obviousover time and be treatable. Or, perhaps you've already been treating it.It's worth continuing to chase it. It took several years for Beth'sto be to the point they could diagnose it confidently. There's verystrong argument that I might have one but no one has been able todiagnose it yet. My rheumatologist has suggested many different biopsiesat one time or another but I'm willing to wait (kidney biopsy, musclebiopsy, lymph node biopsy, skin biopsy, lip biopsy, another lungbiopsy.....lol) . I do believe that at some point one of the labs thatjumps back and forth may just take off upward or some additional symptomthat will clear things up might emerge.So, I think the following for myself personally:1-As

long as it doesn't manifest itself, it's at least somewhat undercontrol so I won't worry too much.2-However, I will continue to monitor and see doctors so that if andwhen it does manifest itself, it will be discovered and if any treatmentis appropriate, I can get it.I also see you as having one distinct advantage over me. You have an ILDbut it is not identified as UIP and based on the treatments may well beone of the others that is treatable and is quite commonly found to havean underlying connective tissue disease. In my case, mine is determinedfirmly to be UIP and so that probability lessened.Don't give up. Time may be here to repeat a major workup as you have newmore detectable symptoms.> >> > Does anyone know how long it takes for approval - are we looking> at months, or years?> >  Dorothy Reinecke> >> >

UIP-7/05> >> >> >> >____________ _________ _________ __> > From: "Paradis3@ ." Paradis3@> > To: Breathe-Support@ yahoogroups.> <mailto:Breathe- Support%40yahoog roups.com> com> > Sent: Thu, November 5, 2009 9:37:47 AM> > Subject: Perfinedone application made to the FDA> >> > Â> > I don't know if it's new news, but it is to me. Intermune has> applied for a new drug approval for Perfinedone as a treatment for> pulmonary fibrosis. I was alerted to that by the coalitionforpf.> org I'll check with my pulmo guys and see what that means, if> anything. Some time ago I asked them about the drug, having heard> it was in testing, and didn't get much of a rise out of anybody, so I> dunno.> >> > Bill C. IPF 10/08> > N.E. PA>

>>

[Guest guest]

Pink,

Thanks - makes sense to me and it makes me feel good believing in Dr. Noble Dorothy Reinecke

To: Breathe-Support@...Sent: Fri, November 6, 2009 2:41:27 PMSubject: RE: Re: Perfinedone application made to the FDA

I think he is not so interested in a label as trying to address the condition & slow progression

Dorothy

yes this makes a lot of sense to me

Pink Joyce R (IPF 3/06) IFA 5/09 Pennsylvania

Donate Life Listed 1/09 Inactive 4/09

www.transplantfund. org

From: Dorothy Reinecke <atlantadorothy@ att.net>Subject: RE: Re: Perfinedone application made to the FDATo: Breathe-Support@ yahoogroups. comDate: Friday, November 6, 2009, 9:20 AM

Beth,

No - Dr. Noble never asked for the slides. On my first appointment he asked for all the data from 18 months at Mass General, labs, CTs, etc. but not the slides from the biopsy. There is no doubt from the CT scans past and current that there is plenty of fibrosis,honeycombi ng, etc. and enlarging pulmonary artery but not enough yet to qualify as full blown PH. I did have a right heart cath which also confirmed 'mild' PH, but not requiring meds yet. Knowing Dr. Noble as long as I do now, I think he is not so interested in a label as trying to address the condition & slow progression. Does this make sense. to you? I may just ask if he would like me to bring the slides - at this point it couldn't hurt, right?

I would love to plan to meet when I come to Durham for my Dec. 17 appointment. We are flying in on the 16th and staying at the Residence Inn this time.

We have always stayed at Millennium since Aug. 07 - just decided on a change of scenery :-).

Dorothy Reinecke

From: Breathe-Support@ yahoogroups. com [mailto:Breathe- Support@yahoogro ups..com] On Behalf Of BethSent: Friday, November 06, 2009 7:51 AMTo: Breathe-Support@ yahoogroups. comSubject: Re: Re: Perfinedone application made to the FDA

Dorothy,

My memory is not terrific so forgive me in advance. Have your slides been looked at by a Duke pathologist? I thought they had but I could be thinking of someone else. If they haven't I would strongly recommend that being done.

Bruce gave you lots of good info so I won't repeat it here. Let me know if there's anything I can do for you Dorothy and let's make sure we get together again when you're up here in December.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

From: "atlantadorothy@ att.net" <atlantadorothy@ att.net>To: Breathe-Support@ yahoogroups. comSent: Thu, November 5, 2009 11:11:45 PMSubject: Re: Re: Perfinedone application made to the FDA

Thanks so much bruce,I will keep going to duke every three months and keep testing.One clarification to my not - I wrote the diagnosis from my visit to duke in may. But the diagnosis from my biopsy pathology report is UIP. When I went to mass general in august 'o5 I brought my slides their pathology also concluded UIP. So what do you understand - has duke changed the diagnosis or are they just not saying UIP? Do you think I should as for clarification when I go back to duke?Many thanks for your inputDorothy

Sent via BlackBerry by AT & T

From: "Bruce" <brucemoreland@ gmail.com>

Date: Fri, 06 Nov 2009 03:42:44 -0000

To: <Breathe-Support@ yahoogroups. com>

Subject: Re: Perfinedone application made to the FDA

DorothyYour situation isn't that odd or strange, believe it or not.. I wouldrecommend continued rheumatological labs and exams based on what yousay, probably as frequently as every three months. In fact, I'm in asomewhat similar situation and my rheumatologist insists on every twomonths, has made referrals for more opinions, and I may end up going toDuke. Now, one would say, why more labs. Because what might today beclassified as Undifferentiated Connective Tissue Disease can thenmanifest itself. Why hasn't it so far? Perhaps because its often delayedor perhaps because the meds you've been on have kept it from doing so.The meds may have treated the connective tissue disease even though noone yet knows which one you have.The muscle pains certainly sound like a Myositis such as Dermatomyositisor Polymyositis so based on that you might find a muscle biopsyrecommended next.The main

thing I'd say is that the possibility of an underlyingconnective tissue disease could still be in your favor as to delayingthe progression of the fibrosis. Perhaps it will become more obviousover time and be treatable. Or, perhaps you've already been treating it.It's worth continuing to chase it. It took several years for Beth'sto be to the point they could diagnose it confidently. There's verystrong argument that I might have one but no one has been able todiagnose it yet. My rheumatologist has suggested many different biopsiesat one time or another but I'm willing to wait (kidney biopsy, musclebiopsy, lymph node biopsy, skin biopsy, lip biopsy, another lungbiopsy.....lol) . I do believe that at some point one of the labs thatjumps back and forth may just take off upward or some additional symptomthat will clear things up might emerge.So, I think the following for myself personally:1-As

long as it doesn't manifest itself, it's at least somewhat undercontrol so I won't worry too much.2-However, I will continue to monitor and see doctors so that if andwhen it does manifest itself, it will be discovered and if any treatmentis appropriate, I can get it.I also see you as having one distinct advantage over me. You have an ILDbut it is not identified as UIP and based on the treatments may well beone of the others that is treatable and is quite commonly found to havean underlying connective tissue disease. In my case, mine is determinedfirmly to be UIP and so that probability lessened.Don't give up. Time may be here to repeat a major workup as you have newmore detectable symptoms.> >> > Does anyone know how long it takes for approval - are we looking> at months, or years?> >  Dorothy Reinecke> >> > UIP-7/05> >> >> >> >____________ _________ _________ __> > From: "Paradis3@ ."

Paradis3@> > To: Breathe-Support@ yahoogroups.> <mailto:Breathe- Support%40yahoog roups.com> com> > Sent: Thu, November 5, 2009 9:37:47 AM> > Subject: Perfinedone application made to the FDA> >> > Â> > I don't know if it's new news, but it is to me. Intermune has> applied for a new drug approval for Perfinedone as a treatment for> pulmonary fibrosis. I was alerted to that by the coalitionforpf.> org I'll check with my pulmo guys and see what that means, if> anything. Some time ago I asked them about the drug, having heard> it was in testing, and didn't get much of a rise out of anybody, so I> dunno.> >> > Bill C. IPF 10/08> > N.E. PA> >>

[Guest guest]

dorothy

having confidence in your doctors is another important factor in your care

Pink Joyce R (IPF 3/06) IFA 5/09 Pennsylvania

Donate Life Listed 1/09 Inactive 4/09

www.transplantfund.org---

Subject: Re: Re: Perfinedone application made to the FDATo: Breathe-Support Date: Friday, November 6, 2009, 3:11 PM

Pink,

Thanks - makes sense to me and it makes me feel good believing in Dr. Noble Dorothy Reinecke

From: Joyce T Rosenberg <pinkrockybeach@ yahoo.com>To: Breathe-Support@ yahoogroups. .comSent: Fri, November 6, 2009 2:41:27 PMSubject: RE: Re: Perfinedone application made to the FDA

I think he is not so interested in a label as trying to address the condition & slow progression

Dorothy

yes this makes a lot of sense to me

Pink Joyce R (IPF 3/06) IFA 5/09 Pennsylvania

Donate Life Listed 1/09 Inactive 4/09

www.transplantfund. org

From: Dorothy Reinecke <atlantadorothy@ att.net>Subject: RE: Re: Perfinedone application made to the FDATo: Breathe-Support@ yahoogroups. comDate: Friday, November 6, 2009, 9:20 AM

Beth,

No - Dr. Noble never asked for the slides. On my first appointment he asked for all the data from 18 months at Mass General, labs, CTs, etc. but not the slides from the biopsy. There is no doubt from the CT scans past and current that there is plenty of fibrosis,honeycombi ng, etc. and enlarging pulmonary artery but not enough yet to qualify as full blown PH. I did have a right heart cath which also confirmed 'mild' PH, but not requiring meds yet. Knowing Dr. Noble as long as I do now, I think he is not so interested in a label as trying to address the condition & slow progression. Does this make sense. to you? I may just ask if he would like me to bring the slides - at this point it couldn't hurt, right?

I would love to plan to meet when I come to Durham for my Dec. 17 appointment. We are flying in on the 16th and staying at the Residence Inn this time.

We have always stayed at Millennium since Aug. 07 - just decided on a change of scenery :-).

Dorothy Reinecke

From: Breathe-Support@ yahoogroups. com [mailto:Breathe- Support@yahoogro ups..com] On Behalf Of BethSent: Friday, November 06, 2009 7:51 AMTo: Breathe-Support@ yahoogroups. comSubject: Re: Re: Perfinedone application made to the FDA

Dorothy,

My memory is not terrific so forgive me in advance. Have your slides been looked at by a Duke pathologist? I thought they had but I could be thinking of someone else. If they haven't I would strongly recommend that being done.

Bruce gave you lots of good info so I won't repeat it here. Let me know if there's anything I can do for you Dorothy and let's make sure we get together again when you're up here in December.

Beth

Moderator

Fibrotic NSIP 06/06 Dermatomyositis 11/08

From: "atlantadorothy@ att.net" <atlantadorothy@ att.net>To: Breathe-Support@ yahoogroups. comSent: Thu, November 5, 2009 11:11:45 PMSubject: Re: Re: Perfinedone application made to the FDA

Thanks so much bruce,I will keep going to duke every three months and keep testing.One clarification to my not - I wrote the diagnosis from my visit to duke in may. But the diagnosis from my biopsy pathology report is UIP. When I went to mass general in august 'o5 I brought my slides their pathology also concluded UIP. So what do you understand - has duke changed the diagnosis or are they just not saying UIP? Do you think I should as for clarification when I go back to duke?Many thanks for your inputDorothy

Sent via BlackBerry by AT & T

From: "Bruce" <brucemoreland@ gmail.com>

Date: Fri, 06 Nov 2009 03:42:44 -0000

To: <Breathe-Support@ yahoogroups. com>

Subject: Re: Perfinedone application made to the FDA

DorothyYour situation isn't that odd or strange, believe it or not.. I wouldrecommend continued rheumatological labs and exams based on what yousay, probably as frequently as every three months. In fact, I'm in asomewhat similar situation and my rheumatologist insists on every twomonths, has made referrals for more opinions, and I may end up going toDuke. Now, one would say, why more labs. Because what might today beclassified as Undifferentiated Connective Tissue Disease can thenmanifest itself. Why hasn't it so far? Perhaps because its often delayedor perhaps because the meds you've been on have kept it from doing so.The meds may have treated the connective tissue disease even though noone yet knows which one you have.The muscle pains certainly sound like a Myositis such as Dermatomyositisor Polymyositis so based on that you might find a muscle biopsyrecommended next.The main

thing I'd say is that the possibility of an underlyingconnective tissue disease could still be in your favor as to delayingthe progression of the fibrosis. Perhaps it will become more obviousover time and be treatable. Or, perhaps you've already been treating it.It's worth continuing to chase it. It took several years for Beth'sto be to the point they could diagnose it confidently. There's verystrong argument that I might have one but no one has been able todiagnose it yet. My rheumatologist has suggested many different biopsiesat one time or another but I'm willing to wait (kidney biopsy, musclebiopsy, lymph node biopsy, skin biopsy, lip biopsy, another lungbiopsy.....lol) . I do believe that at some point one of the labs thatjumps back and forth may just take off upward or some additional symptomthat will clear things up might emerge.So, I think the following for myself personally:1-As

long as it doesn't manifest itself, it's at least somewhat undercontrol so I won't worry too much.2-However, I will continue to monitor and see doctors so that if andwhen it does manifest itself, it will be discovered and if any treatmentis appropriate, I can get it.I also see you as having one distinct advantage over me. You have an ILDbut it is not identified as UIP and based on the treatments may well beone of the others that is treatable and is quite commonly found to havean underlying connective tissue disease. In my case, mine is determinedfirmly to be UIP and so that probability lessened.Don't give up. Time may be here to repeat a major workup as you have newmore detectable symptoms.> >> > Does anyone know how long it takes for approval - are we looking> at months, or years?> >  Dorothy Reinecke> >> > UIP-7/05> >> >> >> >____________ _________ _________ __> > From: "Paradis3@ ."

Paradis3@> > To: Breathe-Support@ yahoogroups.> <mailto:Breathe- Support%40yahoog roups.com> com> > Sent: Thu, November 5, 2009 9:37:47 AM> > Subject: Perfinedone application made to the FDA> >> > Â> > I don't know if it's new news, but it is to me. Intermune has> applied for a new drug approval for Perfinedone as a treatment for> pulmonary fibrosis. I was alerted to that by the coalitionforpf.> org I'll check with my pulmo guys and see what that means, if> anything. Some time ago I asked them about the drug, having heard> it was in testing, and didn't get much of a rise out of anybody, so I> dunno.> >> > Bill C. IPF 10/08> > N.E. PA> >>