I am suffering with idiopathic pulmonary fibrosis, a terminal lung disease.
 There are experimental drugs being tested to treat this condition. 
One that holds promise is Perfenidone on which, I believe, a trial is
now being concluded in Japan.  It has also seen some usage in Mexico. 
Another new drug is Actimmune (Interferon Gamma) made by InterMune,
Inc.  I have been taking it for 17 months with questionable results.
      InterMune has obtained the rights to test Perfenidone in the US
but I fear it could take two years or more -- which I may not have --
for FDA approval.
     I am willing to travel anywhere in the world to try promising
drugs.  If you can find available sources or clinical trials, I would
like you to get this information for me.

Hello Joe250,
 
I've been able to find a variety of clinical trials with involving
several different treatments.  The phase II trials in Japan for
pirfenidone is indeed closed.  You can read the full abstract on the
results of the study, but you'll need to register via
http://www.medscape.com.  It takes a very short time but it's proven
very worthwhile if you do very much medical research.  The title of
the article is "Highlights From the 98th International Conference of
the American Thoracic Society, May 17-22, 2002, Atlanta, Georgia."
http://www.medscape.com/viewarticle/436464_3
 
"Patients in the treatment group showed more stabilization and
improvement in total lung capacity (9.7% vs 1.1%), vital capacity
(12.1% vs 6.1%), and minimal exercise O2 saturation (19.7% vs 12.1%)
than patients in the placebo group. However, what prompted the DSMB to
halt the study was the incidence of acute exacerbations (5 of 35
patients in the placebo group vs 1 of 74 patients receiving
pirfenidone).Two patients in the placebo group died compared with none
in the treatment group. These are promising data and hopefully will
lay the foundation for a larger phase 3 study."
 
{Azuma A. A placebo control and double blind phase II clinical study
of pirfenidone in patients with idiopathic pulmonary fibrosis in
Japan. Program and abstracts of The 98th International Conference of
the American Thoracic Society, May 17-22, 2002; Atlanta, Georgia.
Abstract 729.}
 
To locate a clinical trial, I logged on to the National Institute of
Health and entered "pirfenidone."
http://clinicalstudies.info.nih.gov/cgi/detail.cgi?A_97-HG-0085.html
or
http://www.clinicaltrials.gov/ct/gui/show/NCT00001596?order=48
or
http://www.nlm.nih.gov/cgi/medlineplus/leavemedplus.pl?theURL=http%3A%2F%2Fclinicaltrials%2Egov%2Fct%2Fgui%2Faction%2FFindCondition%3Fui%3DD011658%26recruiting%3Dtrue
 
Title: Therapeutic Clinical Trial of Oral Pirfenidone for Pulmonary
Fibrosis
Number: 97-HG-0085 
Contacts: 
Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793 

Electronic Mail:prpl@mail.cc.nih.gov
 
From http://www.medscape.com again, title of abstract:  Usual
Interstitial Pneumonia, Page 15-17
http://www.medscape.com/viewarticle/410887_1 
 
"Agents that inhibit fibrosis in vitro or in animal models and are
worthy of study in future clinical trials include captopril;
platelet-activating factor receptor antagonists, inhibitors of
leukocyte integrins, cytokines, or proteases; keratinocyte growth
factor; relaxin; and lovastatin."

For additional information, please check this page at the Coalition
for Pulmonary Fibrosis:
http://www.nlm.nih.gov/cgi/medlineplus/leavemedplus.pl?theURL=http%3A%2F%2Fwww%2Ecoalitionforpf%2Eorg%2Fpatient%2Fipfresearch%2Easp

For the Vanderbilt study and from their website:
http://medicine.mc.vanderbilt.edu/divisions/allergy/clinical_trials.cfm
 
"The major focus of the VUCCC has been trials of agents and techniques
with potential value in the diagnosis or treatment of sepsis and the
acute respiratory distress syndrome. More recently, the Center has
broadened its scope to include asthma, primary and secondary pulmonary
hypertension, interstitial pulmonary fibrosis and pulmonary diseases
associated with bone marrow transplantation.

The resources available within the VUCCC include scientific study
design, protocol development, clinical center selection and training,
data collection, data analysis, clinical coordination, study drug
distribution and tracking, study monitoring, report generation,
manuscript preparation, interactions with the Food and Drug
Administration and coordination of investigators meetings for
multi-center trials. A local web site is maintained to facilitate
study coordination. The VUCCC has participated in all aspects of Phase
I, II and III trials but specializes in Phase IB through IIB type
investigations."

For more information please contact:

Gordon R. Bernard, M.D.
Professor of Medicine
Associate Director, Division of Allergy, Pulmonary and Critical Care
Medicine
Division of Allergy, Pulmonary and Critical Care Medicine
T-1208 MCN
Vanderbilt University Medical Center
Nashville TN 37232-2650
Office: (615) 343-0077
Fax: (615) 343-4479

Additionally, here is another trial studying the efficacy of
interferon gamma-1b.
 
http://www.upmc.edu/aaarc/clintrials.htm
 
"The University of Pittsburgh is conducting a study for IPF patients
entitled A Randomized, Double-Blind, Placebo-Controlled, Phase III
Study and Efficacy of Subcutaneous Recombinant Interferon gamma-1b
(IFN-y 1b) in Patients with Idiopathic Pulmonary Fibrosis under the
direction of William J. Calhoun, MD."

For more information on becoming a  participant in this study, please
contact Jeannine at  412-647-0778.

There is also some data pointing toward the treatment of IPF with
retinoic acid, so I've located these studies:

http://www.clinicaltrials.gov/ct/gui/show/NCT00000621?order=40

retinoic acid
 
Location  : California- La Jolla  
Institution  : University of California, San Diego  
Principal Investigator  : Ramsdell, Joe W.  
Function  : Clinical Center  
Contact Information : Arlene Fulton - Study Coordinator 
200 West Arbor Drive, Room 9-260
San Diego, California 92103-8223
Phone:  (619) 543-7736
FAX  :  (619) 543-7742
eMail :  ayoffe@ucsd.edu  
       

--------------------------------------------------------------------------------
Location  : California- Los Angeles  
Institution  : University of California, Los Angeles  
Principal Investigator  : Roth, Michael D.  
Function  : Clinical Center  
Contact Information : Grace Ibrahim - Research Assistant 
10833 Le Conte Ave, CHS 32-170
Los Angeles, California 90095-1721
Phone:  (310) 206-0396
FAX  :  (310) 267-2581
eMail :  gibrahim@mednet.ucla.edu  
       

--------------------------------------------------------------------------------
Location  : Massachusetts- Boston  
Institution  : Boston University  
Principal Investigator  : O'Connor, George  
Function  : Clinical Center  
Contact Information : Paula Goncalves, RN - Study Coordinator 
715 Albany Street, Room R304
Boston, Massachusetts 02118
Phone:  (617) 638-4860
FAX  :  (617) 638-6114
eMail :  pgoncalves@bupula.bu.edu  
       

--------------------------------------------------------------------------------
Location  : Minnesota- Minneapolis  
Institution  : University of Minnesota  
Principal Investigator  : Connett, John E.  
Function  : Coordinating Center  
       

--------------------------------------------------------------------------------
Location  : New York- New York  
Institution  : Columbia University  
Principal Investigator  : Schluger, Neil W.  
Function  : Clinical Center  
Contact Information : Patricia Jellen, MSN, RN - Clinic Coordinator 
161 Fort Washington Ave
New York, New York 10032
Phone:  (212) 305-1158
FAX  :  (212) 305-0412
eMail :  jellenp@nyp.org  
       

--------------------------------------------------------------------------------
http://www.hopkins-lungs.org/pul/jhu/research/trials.shtml Location  :
Pennsylvania- Pittsburgh
Institution  : University of Pittsburgh  
Principal Investigator  : Sciurba, Frank  
Function  : Clinical Center  
Contact Information : Christopher Stewart - Assistant Study
Coordinator
3471 Fifth Ave, 1117 Kaufman Bldg
Pittsburgh, Pennsylvania 15213
Phone:  (412) 692-4020
FAX  :  (412) 958-2470
eMail :  stewartc2@msx.upmc.edu  
 

--------------------------------------------------------------------------------

Johns Hopkins Clinical Trials:
http://www.hopkins-lungs.org/pul/jhu/research/trials.shtml

Information on n-acetylcysteine (there are several promising trials in
Europe)
http://nhscrd.york.ac.uk/online/dare/990510.htm     

Additional Links for IPF Resources:

http://www.lef.org/protocols/abstracts/abstr-046b.html

http://cchs-dl.slis.ua.edu/clinical/pulmonology/environmentallungdiseases/interstitial/ipf.html

I hope these clinical trials are what you're looking for, but if any
of the information is unclear, please notify me in the clarification
area and I will be more than happy to assist you further.

Best regards,
V

Search Criteria:
pirfenidone (Deskar)
n-acetylcysteine
ipf/idiopathic pulmonary fibrosis+clinical trials

---

Clarification of Answer by voila-ga on 15 Sep 2002 11:20 PDT

Here is also another trial being conducted at Long Island Jewish with
interleukin-10 in conjunction with Schering-Plough.

Treatment Trial, IL-10:
We will soon be enrolling patients into a multi-center, randomized,
placebo-controlled trial of Tenovil (rHuIL-10) in patients with
Idiopathic Pulmonary Fibrosis and NSIP. This trial is sponsored by
Schering-Plough Research Institute and all study visits would be
provided free to patients who qualifying for the study.
 
Other Treatment Trials Available for Idiopathic Pulmonary Fibrosis
(IPF):
In general, the response to our currently available therapy for
idiopathic pulmonary fibrosis (IPF) is much lower than we would like.
Because of this we are aggressively pursuing new approaches for this
disease. We anticipate another protocol of a novel inhaled therapy
available to newly diagnosed, untreated patients with IPF within the
next 6 months.
http://www.nationaljewish.org/a4.html

Joe250, 

I'm on this project until you kick me off.  If I've included any
studies that you've already considered and aren't interested in
further, please let me know.  I'm committed to finding you additional
studies that meet your health needs.  As you know, there are specific
requirements to participate in these trials and I'm not sure if you
match *every* criteria, but I can surely tell you what's out there.  I
will continue to look this weekend and as long as needed.

Regards,
V

---

Clarification of Answer by voila-ga on 15 Sep 2002 11:43 PDT

Oops, sorry, that's National Jewish in Denver.  There's another one at
Long Island Jewish I'm check on as well.  Here is the contact
information in Denver:

Interstitial Lung Disease Program Staff
National Jewish Medical and Research Center
1400 Jackson St. F107
Denver, Colorado 80403
(303) 398 1621 Phone
(800) 423 8891 x 1621 Phone
(303) 270 2240 Fax 

Marvin I. Schwarz, M.D.
Principal Investigator

Kevin K. Brown, M.D.
Director

As well, here is some interesting data from Japan using serum CoQ10: 
"Effects of coenzyme Q10 administration on pulmonary function and
exercise performance in patients with chronic lung diseases.

Fujimoto S, Kurihara N, Hirata K, Takeda T
First Department of Internal Medicine, Osaka City University Medical
School.
Clin Investig 1993;71(8 Suppl):S162-6

Serum coenzyme Q10 (CoQ10) levels were measured at rest and during
incremental exercise in 21 patients with chronic obstructive pulmonary
disease (COPD) and 9 patients with idiopathic pulmonary fibrosis
(IPF). The mean serum CoQ10 levels at rest in patients with COPD and
IPF were 0.56 +/- 0.20 and 0.45 +/- 0.16 microgram/ml, respectively.
In both groups these levels were decreased compared with those of
healthy subjects. In the patients with COPD, CoQ10 levels were
significantly correlated with body weight, however, there was no
correlation between CoQ10 levels and ventilatory function, PaO2,
VO2/kg at rest, or maximal VO2. In eight of nine patients whose PaO2
at rest was lower than 75 torr, serum CoQ10 levels were lower than 0.5
microgram/ml. We studied the effects of the oral administration of
CoQ10 at 90 mg/day for 8 weeks on pulmonary function and exercise
performance in eight patients with COPD. Serum CoQ10 levels were
significantly elevated in association with an improvement in hypoxemia
at rest, whereas pulmonary function was unaltered. Oxygen consumption
during exercise was not changed, whereas PaO2 was significantly
improved, and heart rate was significantly decreased compared with the
results obtained at an identical workload at baseline. Furthermore,
lactate production was suppressed during the anaerobic exercise stage
after CoQ10 administration, and exercise performance tended to
increase. These data suggested that CoQ10 has favorable effects on
muscular energy metabolism in patients with chronic lung diseases who
have hypoxemia at rest and/or during exercise."

There are also trials being conducted at UCSF with the use of the drug
called "suramin (relaxin)."
Pulmonary & Critical Care Clinical Activities:
http://pulmonary.ucsf.edu/activities/fibrosis.html 

I will continue looking for studies involving pirfenidone but the one
in Bethesda seems to be a lock at the moment.

---

Request for Answer Clarification by joe250-ga on 26 Sep 2002 11:51 PDT

Dear Voila,

Thank you for your complete report on pulmonary fibrosis.  There is no
need to research lung transplants because they are not available to
persons over age 65.

I have been on Actimmune (Interferon gamma) for 18 months with
negative results.  It appears that Pirfenidone is the only promising
drug available at this time.  A trial has just concluded in Japan with
very good results.  The Shionogi Company in Osaka, Japan, conducted
the trial.  I am attempting to contact them to see if they will
release some Pirfenidone to me.  Perhaps you can find a source
somewhere else in the world, in case Shionogi does not come through.

Thanks for your help.


Joe

---

Clarification of Answer by voila-ga on 29 Sep 2002 20:13 PDT

Hi again, Joe,

Thanks for the kind rating but I'm still on the case and check new
sources several times a week.  I'm glad you gleaned the Shionogi
address from the information presented.   I thought contacting them
might be an option.  Pirfenidone was listed as test article "S-7701"
in the Shionogi trials.  I am also checking under "orphan drug
status."

As pirfenidone seems to be the most promising treatment, I will just
focus on those trials.  Since posting my answer, NIH listed another
trial for pirfenidone -- this one for radiation-induced fibrosis.

Another area of geographic research is the UK and posting a note to
these folks might be an idea:
UCL Centre for Respiratory Reseach - London
http://www.ucl.ac.uk/medicine/respiratory-research/research/ 
 
Additional information on artificial lungs:
http://unisci.com/stories/20022/0520023.htm 

Also found this Canadian company selling pirfenidone for research
purposes.   It may just be a matter of "following the pirfenidone
trail" if doesn't cross any ethical boundaries.  Worth a shot?
http://www.moravek.com/CatalogFiles/Pirfenidone.htm 

I also would like to send inquiries to the top hospitals specializing
in respiratory disease on your behalf, probably starting with National
Jewish.    I'll keep you posted of any progress.

Talk to you soon,
V

---

Clarification of Answer by voila-ga on 06 Oct 2002 21:27 PDT

Hi again, Joe

Found this company in Toluca, Mexico and another researcher (thanks
THX!) helped me locate the contact information.  If you have a
Spanish-speaking interpreter, it might be worth a phone call.

Signa 
Av. Industria Automotriz S/N OF, Esq. Alfaro Nobel Zona Industrial 
50140 Toluca, Edo. de México 
México
Tel: (7) 226-1100

http://www.acic.com/07_partners/partners.html

I am very pleased with Voila's thoroughness and diligence.

Joe250

Hello joe250-ga,

An alternative (which you might have considered already) is a lung
transplant.
I found the 'new lungs' website ( http://www.newlungs.com )
The author Roger Stevens is an IPF patient lucky enough to receive a
double-lung transplant.

He goes into extraordinary detail of the process (
http://www.newlungs.com/intro.html )

"How Do I Get one?'
( http://www.newlungs.com/howdoige.html )

"What is it like?"
( http://www.newlungs.com/whatsitl.html )

"Where do I get one?"
( http://www.newlungs.com/wheredoi.html )

"What does it cost?"
( http://www.newlungs.com/whatdoes.html )


"What are the dangers?"
( http://www.newlungs.com/whataret.html )

"What is the Success Rate and what does it Mean?"
( http://www.newlungs.com/whatisth.html )

"How difficult is Recovery?"
( http://www.newlungs.com/howdiffi.html )

"Can I live without One?"
( http://www.newlungs.com/canilive.html )

"Can I live long Enough to Get One?"
( http://www.newlungs.com/canilivl.html )

"How Long can I Live after I get One?"
( http://www.newlungs.com/howlongc.html )

"What Kind of Long Term Problems will I Face?"
( http://www.newlungs.com/whatkind.html )

His site is full of information on this subject with various links to
relevant external sites, a wealth of knowledge from the patients
perspective.

kind regards
lot-ga

I took a shot in dark and tried UT Southwestern since the original
manufacturer of perfenidone was a Dallas company by the name of
Marnac, Inc. and found this story: 
http://216.239.33.100/search?q=cache:jeodemJLn5IC:www.swmed.edu/home_pages/publish/magazine/pulmonary.html+%22carlos+girod%22+%22UT+southwestern%22&hl=en&ie=UTF-8

"Those therapies include interferon-gamma, pirfenidone and
interferon-beta. All are being used in clinical trials at UT
Southwestern, the only medical center in the country participating in
all three trials. The reason for that may be the reputation of
Weissler's team of specialists. Despite pulmonary fibrosis being a
relatively unusual disease, they diagnose about one new case each
week."

If the study is continuing, I couldn't locate on it on their clinical
trials page.  http://www.swmed.edu/home_pages/medill/clinical


Also, I've read that the Seattle trials are also closed but here are
Dr. Raghu's findings at least.
Pirfenidone Study, G. Raghu, M.D., Seattle, WA 
http://ajrccm.atsjournals.org/cgi/content/full/159/4/1061 

That's all for me today.  I'll check on you tomorrow and please excuse
all my typos.  I'm runnin' on no sleep today.  ;-)
V

p.s.  Thanks so much lot-ga for covering the lung transplant issue. 
It's great to have some backup on this one.