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January – March 2006

NIH, NHLBI Discuss New IPF Research Network
The National Institutes of Health’s (NIH) National Heart, Lung and Blood Institute
(NHLBI) will soon begin recruiting patients for a new program, the Idiopathic Pulmonary
Fibrosis Clinical Research Network (IPF Network), that promises to change the way IPF
is treated. The IPF Network is a consortium of 11 medical centers across the country that
will serve as sites for new NHLBI-sponsored clinical trials for newly diagnosed patients
with IPF, plus a data coordinating center. Recruitment of patients is expected to begin in
June 2006.

Dr. James Kiley, director of the Division of Lung Diseases at the NHLBI spoke with the
Teresa Geiger, vice president of patient outreach and advocacy for the CPF at the NHLBI
Public Interest Organization (PIO) meeting in Bethesda, Maryland on Jan. 31. The annual
PIO meeting brings together various advocacy organizations representing a variety of
patient groups and allows them to meet with NIH leadership as well as network with
other organizations.

―The bottom line is that we saw the basic science [around IPF] continuing to develop and
we put a push behind it [by establishing the IPF Network],‖ said Dr. Kiley. ―We hope we
can move things along to improve treatment options and patient care. Our goal is to
increase attention on translational IPF research.‖

What makes this clinical research network different from many others in the area of IPF
research, Dr. Kiley says, is that it is independent of pharmaceutical industry involvement.
―The IPF network is not influenced by outside organizations in terms of what clinical
protocols will be done,‖ he says. ―We can stand back and decide what to do and fill in the
science. If needed, the network can do head-to-head drug studies to give the doctors and
patients what they need.‖

Though optimistic about the opportunities for discovery in the network trials, Dr. Kiley
says he is realistic in his expectations. ―We might not provide the magic bullet for IPF
through the network, but we may be able to provide insight into new treatment

The IPF Network, which was formed in 2005, is currently funded through 2009. The
network expects to carry out two to four protocols in the first five years and publish the
results in peer review medical journals, according to Dr. Kiley.

The IPF Network is one of five such lung disease networks funded by the NIH/NHLBI in
which research centers work collaboratively. The others cover Chronic Obstructive
Pulmonary Disease, Acute Respiratory Distress Syndrome and Adult and Pediatric
The clinical sites chosen for the IPF Network are:
• Emory University (Georgia)
• University of Alabama (Alabama)
• Tulane University Medical Center (Louisiana)
• National Jewish Medical & Research Center (Colorado)
• University of California- San Francisco (California)
• David Geffen School of Medicine at UCLA (California)
• University of Washington Chest Clinic (Washington)
• University of Chicago Medical Center (Illinois)
• University of Michigan Health System (Michigan)
• Cornell University Medical Center (New York)
• Vanderbilt University Medical Center (Tennessee)
• Duke University Medical Center (North Carolina); the data collection site
CPF Talks with Mark Steele, M.D.
Duke University Medical Center
Mark P. Steele, M.D.
Associate Professor
Division of Pulmonary, Allergy, and Critical Care Medicine
Duke University Medical Center

How did you become involved in IPF related research and why?
It is an area of the lung I studied in the lab for five years and translated it into clinical
research. In terms of interest, I come from a genetics background.

As a follow up to your recent initial findings, what do you consider the most
convincing evidence on the genetic link to IPF?
The most convincing is the number of families and the consistency that pulmonary
fibrosis is passed through those families. It is moving generation to generation
―autosomal dominant‖ meaning it just takes one copy of the gene for a person to get the
disease. The sheer number of families [with a history of IPF] was surprising. Years ago,
there was little thought at all that this ran in families, mostly because no one asked or
looked for it. Other convincing evidence is that our current genetic data is yielding
linkage to two chromosomes. Our next step is to establish and identify genes in their
regions of linkage on the two chromosomes. We think it will not be a single abnormal
gene that causes both familial and sporadic IPF, it will likely be multiple abnormal genes
that are found to be responsible for IPF. What remains to be determined is how broadly
applicable the genes are in the sporadic [or isolated cases] of IPF.

Of all pulmonary fibrosis cases, what percentage do you believe has a genetic
In overall IPF cases, I would estimate that five to 10 percent are familial [genetic].

Do IPF cases tend to be in any isolated geographic areas of the country?
Most of the families are spread across the U.S. There is some clustering in population
centers on the coasts, but we can’t say [that there are more cases in a given region]. It
could be a referral and ascertainment bias.

How much penetrance in the average family with IPF are you seeing? In other
words, what percentage of individuals in families are developing IPF?
The penetrance we have been seeing in families [in general research], is that there is one
gene and we predict a dominant model. So, half the families or 50 percent are affected. In
the Duke study, we are seeing less than half. It also has to do with who comes into the
study. We don’t have universal participation within the families [every member in the
study] therefore the exact risk can’t be estimated. There are people who carry the gene
who don’t get IPF. One of the risk factors for developing the disease was cigarette

Does IPF seem to increase in number in families in subsequent generations?
There are circumstances that diseases can become more penetrant as they move through a
generation. We are not sure yet on IPF. Most people in the field [of IPF] believe that it is
more prevalent than we previously realized, likely due to previous eras and lack of

Compared to other diseases that have known or unconfirmed genetic links, is
IPF more or less prevalent? Can you provide any comparative examples?
In terms of comparing to those diseases that are similar by way of genetic links, cystic
fibrosis is a common genetic disease. Huntington’s Chorea is less common. Classic
genetic diseases or ―classic mendelian diseases― are characterized by one abnormal gene
that causes the diseases. IPF will be a complex disease in which there are probably
multiple diseases that interact to give you the final disease. Also a gene/environment
interaction and a combination of those factors is important.

For families who seem to be vulnerable to IPF, what would you tell them? More
specifically, should they tell their doctor of their family history?
I think it is important that they share it and that they be aware of their environment. We
are less certain to what those environmental triggers are, like certain dust exposures as
well as exposures to viruses and metal. Cigarette smoke is clearly a factor. I would ask
them to have a medical professional monitor them for the presence of IPF, if they [the
doctors] feel comfortable doing that. A chest CT scan, X-rays and breathing tests can be
performed. If the doctor is not comfortable, [the family members should] ask for a
referral for a pulmonary specialist. Most can screen for the disease. When you have been
diagnosed with IPF, it helps to get to a center of excellence where they concentrate on
diseases such as IPF.

Why is the research on IPF important?
It is a terrible disease and probably the only disease I take care of in pulmonary disease
where we have absolutely no FDA-approved treatment.

There aren’t many diseases where we have no treatment for the underlying causes. We
can only offer supportive care such as pulmonary rehabilitation and oxygen therapy.

Will the research you're doing help lead to treatments? If so, how?
We’re seven years into our research and it is a long, slow process, especially with the
genetic angle. Fundamentally, we don’t understand IPF though we have hypotheses out
there about what we think is the problem. The most common one is one in which there is
believed to be an abnormal wound repair process, disregulated scarring somehow gone
wrong. There are treatment strategies to slow down scar formation. If that hypothesis is
right, you’d think we will find a treatment soon. If another hypothesis, based on an
injured alveolar epithelium [surface] that is not repairing, is accurate, our current
treatments will fail. I think we need to look at the fundamentals. If we can find the genes
that are abnormal, it can help us with current and future treatment options and let us
know if we’re heading in the right direction.

What trends do you see? Where is the excitement in the areas of research? Are
there any emerging therapies in development?
Most of the therapies are going along the road of targeting the fibroblasts, the cells that
make collagen or scar tissue. We’re using a lot of bioengineered molecules (i.e.,
interferon gamma). The pathway is to block collagen formation in most of it where you
will see the activity. Perfenidone is a small molecule that blocks collagen formation.
Another is an area of research that the NIH has put together with the clinical network to
study the disease. It will be important to sustain this network by recruiting adequate
numbers of patients into the NHLBI-sponsored trials, and get additional funding [than to
fund individual research]. It is a major new development in the last year or two.

Is this the foundation for future genetic research?
I think this is the future for complex diseases. In complex diseases, the direction is going
towards genetic research to identify why some people get some disease and some don’t.

In the next two years, and further out, what is on the horizon for IPF in terms of
patients or research?
I see many more clinical trials on the horizon. Patients will probably have more than one
or two trials that they can choose from. They may have four, five or six trials in the
future. The wider you can cast your net and bring more patients in, the more you can
learn. An example is the interferon gamma trial. It was such a large trial that we learned a
lot about the natural history of how the disease behaves. We will learn more of that in the
next couple of years.

As a researcher and scientist, if you could speak with political leaders regarding
the need for education and research dollars for IPF, what would you focus on?
You’re basically asking for money. I don’t think patients should be unnecessarily
penalized for having rare diseases. To say ―everyone gets diabetes, heart disease, etc., we
spend a lot on you‖ but ―if you have IPF, we won’t spend a lot on you.‖ I think that is
very wrong. That concept needs to be pushed aside. When a disease strikes at the prime
of life, there is no therapy, and further more, there is no replacement therapy available,
like dialysis for kidney failure, there should be money spent on it.

IPF is rare, yes. It is a terminal, untreated disease. We need to put efforts toward it.
Education is important, in terms of making people aware that this disease is out there.
The CPF has found that patients have been shown to be misdiagnosed with asthma and
other conditions. Also, we have to find the disease earlier. There has to be an awareness
that it is out there, not only to educate the public, but the physician community as well. A
patient who is not diagnosed three to four years after the disease has started is what we
see way too much of at this point.

What is the current situation and future of lung transplants?
There is much encouragement for IPF patients to pursue lung transplantation.

Realizing we are extending the upper limits for age in transplantation which has been a
limitation for IPF patients since it is a disease affecting most patients 60 plus. Transplant
programs are getting more comfortable with patients throughout their 60s, thus more IPF
patients may be candidates for lung transplantation. Lung transplant survival is slowly
improving. I think they [patients] have an opportunity to help their fellow patients by
participating in clinical trials and I encourage them to participate. The trials don’t work if
we don’t enroll to the target [number of patients needed]. A sacrifice that patients will
make is the risk of getting placebo, but it will advance the field if we do these trials well.

What is the main issue with clinical trials?
It’s taking too long to get patients enrolled in the trials. I would like
to see it done faster. A large cardiology trial will enroll 20,000 patients in six to 12
months. The issue is that IPF is more rare and it is harder to get patients and they are
spread out geographically and many are not close to centers. What do you do with the
patients who live in between and far from [study centers]? Another limitation is when
physicians provide drugs off the label without getting patients into clinical trials.

If physicians are putting patients on novel treatments because the drug is available and
we think it might work that is not the standard of care that we want to hold ourselves to.
We want to drive home to the medical community the message of putting patients in
clinical trials.
Research Update
Actelion, Ltd.: Bosentan (Tracleer) Studies In IPF
Show No Effect On Primary Exercise Improvement Endpoint
Secondary endpoints related to death or disease worsening provide strong rationale for
Phase III mortality/morbidity study in idiopathic pulmonary fibrosis

Actelion, Ltd. recently announced that in the BUILD program with bosentan
(Tracleer(R)) in patients suffering from either idiopathic pulmonary fibrosis (IPF)
(BUILD-1) or pulmonary fibrosis related to systemic sclerosis (BUILD-2), no effect was
shown on the primary endpoint of exercise improvement as measured by the six-minute-
walk test.

In the IPF study BUILD-1, although not statistically significant, positive trends were
observed for pre-defined secondary endpoints, such as the combined incidence of death
or treatment failure at 12 months (36.1 percent in the placebo group versus 22.5 percent
in the bosentan group; p=0.076; 95 percent CL 0.37, 1.05), representing a relative risk
reduction of 38 percent.

Treatment failure (per-protocol) was defined as worsening in Pulmonary Function Tests
(PFTs) or acute decompensation of IPF. Worsening in PFTs was defined as a decrease
from baseline in two of three criteria: decrease from baseline greater than 10 percent in
FVC; decrease from baseline greater than 15 percent in DLco; decrease from baseline
greater than four percent in O2 saturation (blood gas) at rest or increase from baseline
greater than eight mmHg in A-a gradient PO2. These findings provide a strong rationale
for pursuing a mortality-morbidity Phase III study. Accordingly, the company will
initiate discussions with scientific experts and regulatory authorities worldwide on the
design of the Phase III study.

In both studies, the use of bosentan was well-tolerated. The observed safety profile was
consistent with earlier findings in clinical studies that led to the approval of Tracleer(R)
in pulmonary arterial hypertension (PAH).

Full results of the BUILD program are expected to become available through
presentations at upcoming international scientific meetings and publications in peer-
reviewed journals.

Talmadge King, Jr., M.D., professor of medicine at UCSF, San Francisco General
Hospital and principal investigator of the BUILD-1 study, commented: ―I am encouraged
that in this well-designed study in patients with IPF, a severe lung disease with no
effective therapy, clinically relevant trends were observed in important parameters related
to death or disease worsening. This observation should be further investigated in IPF with
a clinical program that focuses on improving patients' long-term outcome.‖

Dr. King concluded: ―From the results of BUILD-1, we might consider that the six-
minute-walk test – selected for the first time as the primary endpoint in pulmonary
fibrosis studies – is not an appropriate endpoint to evaluate the response of this patient
population to treatment.‖

In the study with patients suffering from pulmonary fibrosis related to systemic sclerosis
(BUILD-2), no effect was observed on either primary or secondary endpoints.

Source: Actelion, Ltd.
CPF Joins More than 20 Lung Disease Organizations in Providing
Suggestions to DOT for Oxygen Use in Airline Travel
On Jan. 30, 2006, the CPF joined more than 20 lung disease organizations to provide
suggestions and guidance via comments to the Department of Transportation (DOT)
regarding oxygen use for airline travel. The organizations submitted their combined
comments on behalf of their memberships and individuals with lung and airways disease
who are often reliant on medical oxygen and other respiratory assistive devices.

The organizations’ comments were in response to the Department of Transportation’s
initiation of the Notice of Proposed Rulemaking (NPRM) on Nondiscrimination on the
Basis of Disability in Air Travel – Medical Oxygen and Portable Respiration Assistive
Devices (Docket No. OST 2005-22298).

The group included the Alpha-1 Foundation, National Emphysema COPD Association,
American Association for Respiratory Care, American Autoimmune Related Diseases
Association, as well as the CPF and others. Its recommendations were provided to help
clarify the NPRM from the patient perspective and encourage expedition of final
guidance to people who require supplemental oxygen and other assistive respiratory
devices during air travel.

The CPF supports the consensus of the ATS statement as delivered to the DOT. The
recommendations by the patient groups included:

      No limited coverage of oxygen to carriers operating larger than a 60 seat aircraft,
       but include all air carriers, including smaller airports and rural areas where
       smaller aircrafts are used. The requirement should cover both compressed and
       liquid carry-on systems.

      Support and creation of clear pathways for aircraft manufacturers, the airline
       carriers, and assistive respiratory device manufacturers to accelerate testing while
       containing costs and expediting approval of new and existing equipment;
       streamline the testing requirements for respiratory devices.

      Support of creation and maintenance of a centralized list of approved devices.
       This list should be posted on the FAA website, linked to by each carrier that
       maintains a web presence and available on request at a toll free number that is
       maintained by the FAA. With respect to devices that are in the process of being
       evaluated or have been disallowed for air travel, individuals should have access to
       this information upon request.

      •Regulation of reasonable carrier charges for medical oxygen. Individuals should
       not be charged by carriers when using their own pre-approved equipment; when
       individuals use equipment provided by carriers, fees should be fair, consistent,
       well-published and understood by passengers who need these services. In
       addition, travel initiated and completed with one carrier should carry a single fee
       and not a ―per leg‖ charge for changes and layovers.
Research Update
Advanced Genomics and Proteomics May Improve Diagnosis and
Treatment of IPF
A recent University of Pittsburgh study suggests that standard anti-inflammatory therapy
may not be appropriate for many interstitial lung disease patients.

In an article in the Jan. 15 issue of the American Journal of Respiratory Critical Care
Medicine, University of Pittsburgh researchers report that a serious, life-threatening form
of pulmonary fibrosis, called idiopathic pulmonary fibrosis, lacks all the hallmarks of
inflammation and is probably unnecessarily treated with anti-inflammatory drugs.
Moreover, in a related study, the investigators identified a protein found in excess
amounts in the lung tissue of patients with idiopathic pulmonary fibrosis, which may be a
more appropriate target for therapy.

Interstitial lung disease describes a diverse set of chronic lung conditions that often have
strikingly similar symptoms but different clinical courses. However, all are characterized
by differing degrees of progressive scarring of lung tissue between the air sacs, or the
interstitium. With repeated damage, the interstitium becomes thickened and stiff, or
fibrotic, making it increasingly difficult for the individual to breathe. Some forms of
interstitial lung disease, particularly idiopathic pulmonary fibrosis, which has no known
cause, have a very high death rate due to respiratory failure. Effective treatment,
however, is complicated by the fact that a definitive diagnosis often requires a lung

―Unfortunately, many patients do not receive lung biopsies,‖ explained James Dauber,
M.D., medical director of the University of Pittsburgh's Dorothy P. and Richard P.
Simmons Center for Interstitial Lung Disease, and professor of medicine, Division of
Pulmonary, Allergy and Critical Care Medicine. ―As a result, about one-third of patients
who come to our clinic have previously been misdiagnosed, and many have been treated
with the wrong medications.‖

To improve the diagnosis and treatment of interstitial lung diseases, Naftali Kaminski,
M.D., director of the Simmons Center and associate professor of Pathology and Human
Genetics, Dr. Dauber, and their colleagues decided to test the effectiveness of DNA
chip technology in distinguishing between the gene expression patterns of several types
of interstitial lung diseases. Because it can be
difficult to obtain lung biopsy samples for some types of interstitial lung disease, the
Simmons Center investigators collaborated with researchers in Mexico to obtain samples
for another type of pulmonary fibrosis known as hypersensitivity pneumonitis - a
pneumonia-like inflammation of the lungs caused by the body's immune reaction to small
air-borne particles that is more prevalent in countries such as Mexico where pet birds are
Drs. Dauber, Kaminski and their collaborators obtained lung biopsy samples from 15
patients diagnosed with idiopathic pulmonary fibrosis; 12 patients with hypersensitivity
pneumonitis; and eight patients with a third, less-understood type, known as nonspecific
interstitial pneumonia. The latter is characterized by inflammation and fibrosis that
occurs suddenly and progresses rapidly over a relatively short period of time.

When the investigators analyzed the gene expression patterns of the samples using a
DNA microarray chip containing sequences for approximately 46,000 known gene
clusters–which represent most of the genes in the human genome–the
results were startling. Although all of the patients from whom the samples were taken had
similar X-ray and laboratory test results, their gene expression patterns were radically
different. Indeed, the
investigators found that the hypersensitivity pneumonitis samples showed significantly
increased expression of genes associated with inflammation, immune cell activation and
immune response. In contrast, there was almost no genetic evidence of inflammation in
the idiopathic pulmonary fibrosis samples.

―Our results show that interstitial pulmonary fibrosis and hypersensitivity pneumonitis,
which clinically often look quite similar, are really two vastly different conditions,‖ said
Dr. Kaminski. ―Idiopathic pulmonary fibrosis is characterized by the increased
expression of genes involved in the re-growth of lung tissue. So, it is not really an
inflammatory condition per se. On the other hand, hypersensitivity pneumonitis does
exhibit all of the hallmarks of inflammation, with increased expression of genes that
control T-cell activation and immune responses.‖

Another surprising finding came when the investigators compared these gene expression
patterns to those exhibited by biopsies from the eight patients diagnosed with nonspecific
interstitial pneumonia. Two of the eight cases exhibited interstitial pulmonary fibrosis-
like gene expression patterns, one closely resembled the gene expression pattern of
hypersensitivity pneumonitis, while the other five expression patterns resembled neither.
Thus, the investigators were able to reclassify some of the cases of nonspecific interstitial
pneumonia using this technology.

In an accompanying editorial, Victor J. Thannickal, M.D. of the University of Michigan
and Athol U. Wells, M.D. of the Royal Brompton Hospital in London wrote that although
―further studies with greater numbers of patients are required, these genetic signatures
provide important clues to the observed differences in clinical course, prognosis and
responses to therapy in these two disease processes.‖

If these findings hold up in larger studies–and Dr. Kaminski is strongly convinced that
they will–the diagnosis and management of interstitial lung disease may radically change.
In particular, patients with idiopathic pulmonary fibrosis who are commonly prescribed a
course of corticosteroids or other anti-inflammatory drugs as the first line of treatment,
will not be subjected to such unwarranted and potentially harmful approaches.
―Until now, the treatment of idiopathic pulmonary fibrosis has been primarily focused on
its inflammatory component,‖ said Dr. Kaminski. ―However, our findings indicate that
lung tissue from these patients does not exhibit a typical inflammatory pattern. So, these
patients need to be managed in an entirely new way.‖
Although there is currently no effective treatment for idiopathic pulmonary fibrosis,
results of another study suggest help may soon be on the way. Dr. Kaminski and his
colleagues reported in the Sept. 6 online edition of PLoS Medicine that idiopathic
pulmonary fibrosis lung tissue samples display an ―over-abundance‖ of a protein known
as osteopontin, which other studies have implicated in the growth and progression of
tumors. In further examining the potential role of osteopontin in idiopathic pulmonary
fibrosis, Dr. Kaminski's group found that it directly increases the proliferation and
movement of fibroblasts, cells centrally involved in lung fibrosis.

―Taken together, these findings are very exciting because we now have a basis for
designing drugs that are specifically directed against osteopontin,‖ explained Dr.
Kaminski. ―By manipulating osteopontin levels, we may be able to slow or stop the
course of this deadly disease. In addition, the level of osteopontin may be used as a
diagnostic marker for this disease.‖ He added that his group is currently investigating
whether measuring the expression patterns of osteopontin and other genes can predict
disease progression and outcome.

In addition to Drs. Kaminski and Dauber, other investigators involved in this research
include Kevin Gibson, M.D., Thomas Richards, Ph.D., and Samuel Yousem, M.D., from
the University of Pittsburgh; Moises Selman, M.D., Lourdes Barrera, Msc., and Andrea
Estrada, M.D., of the Instituto Nacional de Enfermedades Respiratorias in Tlalpan,
Mexico; Annie Pardo, Ph.D., of the Facultad de Ciencias, Universidad National
Autonoma de Mexico, Mexico City. This research was supported by a grant from the
National Heart, Lung and Blood Institute, National Institutes of Health and a donation
from the Simmons family.

Source: Simmons Center for the Study of Interstitial Lung Diseases at the University of
Pittsburgh Medical Center
Research Update
New Study Shows N-acetylcysteine (NAC) May Improve
Treatment of IPF
Adding a high-dose antioxidant to standard drug therapy can improve lung function for
patients with a serious respiratory disease called idiopathic pulmonary fibrosis.

The report, which appears in the Nov. 24 issue of The New England Journal of Medicine,
found that patients' lung function improved when high-doses of the enzyme N-
acetylcysteine were added to standard drug treatment. Acetylcysteine is an enzyme that
acts as an antioxidant and is also used to help loosen up mucus.

―Up to now, there are no real effective drugs for idiopathic pulmonary fibrosis (IPF),
which is a disease with a poor prognosis,‖ said lead author Dr. Maurits Demedts, from
the Division of Pneumology at University Hospital Gasthuisberg, Leuven, Belgium.

―Our study showed that adding high oral doses of N-acetylcysteine to the standard [drug]
therapy of prednisone and azathioprine — which by most experts is considered to be
probably of no real effectiveness — significantly slows the rate of deterioration of the
lung function,‖ he said. In their study, Demedts' team randomly assigned 182 patients to
receive 600 milligrams of N-acetylcysteine three times a day or a placebo plus standard
drug therapy.

The researchers found that, compared with placebo, N-acetylcysteine slowed the
deterioration of vital lung capacity by nine percent and diffusing capacity by 24 percent
after one year.

During the trial, nine percent of the patients receiving N-acetylcysteine died, as did 11
percent of the patients receiving placebo, the researchers report.
―We consider these effects of adding N-acetylcysteine to the standard therapy clinically
relevant, because it is accepted from other recent studies that decreases of at least 10
percent for vital capacity and 15 percent for diffusing capacity over a period of six to 12
months are associated with an increased risk of death in IPF,‖ Demedts said.

Demedts believes that this treatment can be effective in boosting IPF treatment. ―High-
dose N-acetylcysteine in addition to standard therapy is a rational treatment option for
patients with IPF,‖ he said.

One expert thinks that claims for increased survival of IPF patients taking N-
acetylcysteine cannot be substantiated based on this study alone. ―They [the researchers]
infer that the effect is sufficient to translate into prolongation of life, but we cannot be
certain of this,‖ said Dr. Norman H. Edelman, chief medical officer at the American Lung

Another expert said the benefits of N-acetylcysteine, if any, can't be shown from this
study since patients were taking other medications, so the effect of N-acetylcysteine was

―Because of the design of the study, we can't know if N-acetylcysteine independently is
beneficial for patients with IPF,‖ said Dr. Gary W. Hunninghake, a professor of internal
medicine and director of the Pulmonary Program in Internal Medicine at the University
of Iowa, and author of an accompanying journal editorial. ―There are two possibilities,‖
Hunninghake said. ―One is that N-acetylcysteine truly helps patients with IPF. The other
possibility is that its main effect was to ameliorate the toxicity of azathioprine,‖ he added.
Although prednisone and azathioprine are commonly used to treat IPF, there is little
evidence that they help patients with the disease, and in fact they may be toxic,
Hunninghake added.

Hunninghake believes that N-acetylcysteine might be potentially useful in treating IPF. ―I
am not dismissing that. But you just can't tell from this study,‖ he said.

As for patients, Hunninghake thinks that taking N-acetylcysteine won't do any harm. ―It
will almost certainly do no harm, and it may be of value,‖ he says. ―We just don't know.‖

Source: Health Daily News & Reuters News Service
Campaign ACT Update

CPF Urges Membership to Meet with Congressional
Representatives in their own Districts

In Sept. 2005, the U.S. House of Representatives passed a special resolution (H. CON.
RES. 178) recognizing the importance of National IPF Awareness Week and formally
calling for a treatment and eventual cure for IPF.

The Senate version of the bill recognizing the work of the CPF and National IPF
Awareness Week - known as S. RES. 236 - was introduced by Senator Norm Coleman
(R-MN) on Sept. 13, 2005.

The CPF continues to urge its members to contact their Senators in Washington, D.C. and
ask them to support S. RES. 236. If your U.S. Senator is not listed as a co-sponsor below,
please contact them today. If they are listed,
let them know that you appreciate their support.

Current co-sponsors of S. RES. 236:
    Sen. Burr (R-NC)
    Sen. Chambliss (R-GA)
    Sen. Isakson (R-GA)
    Sen. Lugar (R-IN)
    Sen. Bayh (D-IN)
    Sen. Bingaman (D-NM)
    Sen. Durbin (D-IL)
    Sen. Snowe (R-ME)

For information on contacting your Senators, visit the CPF ACT Web page at:
Legislation May Affect Oxygen Equipment and Services for IPF

Senate Bill 1932, a provision of the Deficit Reduction Act of 2005, passed earlier this
year raised concerns of patients and groups such as the CPF and the American
Association for Respiratory Care (AARC) that patients could suffer not only financially,
but may experience gaps in service.

However, according to CPF sources, Medicare will continue to pay for any needed
servicing and parts replacements for the oxygen equipment.

Historically, Medicare has provided reimbursement for oxygen, oxygen equipment and
services for patients who are eligible to receive oxygen services in their home. So
patients are, in effect, ―renting‖ their equipment from their device providers because the
provider is being reimbursed a set amount of money per patient each month.

The CPF will keep our members updated as new information on the bill becomes
available. The bill is not scheduled to take affect for three years and it may be amended
before that time.
Coalition for Pulmonary Fibrosis Grants $43,000 Gift
to the University of Chicago to Advance New Approaches to
Understanding IPF
The Coalition for Pulmonary Fibrosis (CPF) recently announced a gift to the University
of Chicago that will go to help further idiopathic pulmonary fibrosis (IPF) research. The
gift has been made in the amount of $43,000 and is the result of funds raised at its
inaugural B.I.G. (Breathing is Glorious!) Ball held in Chicago, Ill. in partnership with the
University of Chicago on Oct. 21, 2005.

As a member of an NIH-designated clinical research network for the study of IPF, the
University of Chicago is using the funds to aid the establishment of a new study of the
―natural history of interstitial lung diseases and IPF,‖ a study that has been approved by
the University’s institutional review board (IRB) to begin enrolling subjects from the
Interstitial Lung Disease Clinic. The study aims to better describe the ―phenotypic‖
expression of interstitial lung diseases, specifically IPF. Following patients over time will
allow investigators to track the natural history of these diseases with special attention
focused on: assessment of quality of life (QOL) questionnaires as predictors of
physiological changes; assessment of response to various therapies; and surrogate
physiological markers of mortality. The study will also assess if blood samples for later
genetic analysis and genomics provide insight into the polymorphisms related to the
etiology and pathology of the lung damage caused by IPF.

Patients will also be screened for participation in new studies that become available. This
information will help identify trends and hopefully lead to a better understanding of the
disease progression, treatment options and outcomes.

―The CPF’s partnership with the University of Chicago clearly advances our mission of
raising awareness about IPF and supporting and funding emerging approaches to
understanding the disease,‖ said Mark A. Shreve, chief executive officer of the CPF.
―Through this fundraising effort we were able to provide an avenue for donors to support
their local medical center, while at the same time supporting the national programs and
services of the CPF. This fundraising model continues to demonstrate that we can fuel
new efforts to help all those affected by IPF.‖

Dr. Imre Noth, assistant professor of medicine, and the University of Chicago have also
established a consortium of investigators from Northwestern University and the
University of Illinois at Chicago to advance efforts to improve standards of care for the
treatment of IPF in the Chicago metropolitan area.
Specifically, support from CPF will be used to recruit a data coordinator and acquire the
software resources to help manage the data collected through the consortium.

―With the help of generous donors and the CPF, the regular and consistent funding of this
project over time will lead to marked improvements in how we prevent, diagnose and
treat IPF,‖ said Dr. Noth.
The CPF and the University of Chicago will be hosting the second annual B.I.G. Ball on
Oct. 21, 2006 at the Renaissance Chicago Hotel with a goal to raise $200,000. The
University of Chicago will continue to use these funds to support new and existing
studies in IPF to determine innovative targets for therapy and assist in earlier diagnosis.
For more information, visit or call the CPF at (888) 222-8541.
Quilting for IPF:
CPF interviewed Ruth Elaine Roy to find out more about her local
fundraising effort

Ruth Elaine Roy, 54, of Cincinnati, Ohio began her quilt fundraising project on Jan. 3,
2006 and two months later she had already raised $330, $1 to $5 at a time. The
entrepreneur and owner of Aunt Ruth’s Scrapbasket in Sharonville, Ohio was motivated
by her husband’s diagnosis with IPF in 2004.

How did you hear about the CPF?
I heard about the Coalition because my husband, Tim, had been diagnosed with
pulmonary fibrosis in 2004. We tried to seek out anyone who had information and we
found it on the internet. I was previously a nurse and used to work as a VP with an HMO.
We were visiting the CPF website, trying to see what other options besides lung
transplant were available.

What was your inspiration for creating the quilt?
When you receive a diagnosis such as IPF, it is very easy to feel that you have lost total
control over the event and your life. I do have control over who or what I will raise
money for. It makes me feel like I am doing something for him. It goes back to mixing
personal with business. As part of my business, my mission statement has in it that we
will be good corporate and community citizens. We are active in community events. We
have done several things, like each month a group meets and sews quilt tops for
Children’s Hospital. To date, we have contributed 740 quilt tops.

With Tim’s diagnosis, we wanted to get our customers involved in doing a quilt for IPF;
it was an easy fit. The quilt is one of a kind. It was created for our Tri-State Shop Hop in
2004. Each of the 12 shops had a specific block and we all had to use the same fabric
line. The yellow block is the Aunt Ruth’s quilt block. Aunt Ruth’s quilt uses traditional
blocks in a contemporary setting. The quilt is Aunt Ruth’s version of how to put all of the
blocks together. Customers know Tim. We have been in business for three years (opened
Oct. 1, 2002). The first year, he was at the shop quite a bit helping me out. We now have
seven part time staff members, so he does not need to be there as much.

When you started your fundraiser, were your customers familiar with IPF?
Before Tim was diagnosed, most [customers] weren’t familiar with IPF – they had no
clue what I was talking about. I have done an awful lot of clinical discussion with people
who are asking questions. I really was not that familiar with IPF, even as a nurse I
vaguely knew about it. I didn’t know how [a person] got it and didn’t know there was no
treatment. I knew there was scarring. I tried to come up with a time when I was taking
care of a patient with that diagnosis. I couldn’t remember them.

When did you leave nursing?
In 1982 I left bedside nursing full time, stayed part time while also working full time at a
local physician-owned HMO. In 1992, I moved to Indianapolis and worked for Anthem
as the director of Medical Management and in 1996, I moved to Nashville and was a vice
president for Medical Management at HealthNet.

How did you find the CPF?
I wasn’t sure how we were going to be connected to the CPF because when somebody
tells you that there is no treatment other than transplant, then [transplant] is where your
energies lie. As the weeks would go by, our focus was on contacting the transplant
programs. When it was all first happening, he wasn’t feeling well and he was on
Prednisone. Mostly, we were skimming websites of anyone who had any info on
anything. We saw that there were educational seminars through the CPF.

When the transplant program at the Cleveland Clinic asked us to find another transplant
program, I started feeling that there had to be something out there to help him live his life
as best he could as long as he could, even without transplant. I went to the CPF site and
saw a seminar being put on at Emory University. On one level, it was fine, because Tim
had never talked with anyone else who had IPF – see them, meet them or cope with the
disease. While we didn’t hear anything that we hadn’t heard before, what really struck
[us] was the lack of funding and the lack of attention to that disease entity. I decided if I
couldn’t do something on a national level, I could do a local story. I could contact local
newspaper folks.

Did you use the CPF fundraising kit?
No – I first contacted my lawyer to see if I was going to stumble into anything that would
do the business any harm. My attorney said I should contact the CPF to make sure they
were aware of my effort and then when the event was over, I should give the funds to the
place it should go to. I like the fundraising kit, though, and I recommend people use it in
their efforts.

Have you begun to see good things happen around your efforts?
There is a sweet story that has happened already. One of our staff said I should talk to
this lady who had come into the store. She wanted to buy $50 worth of raffle tickets. She
read we were doing this in the local paper (The Valley Courier). The Sharonville writer
wrote about it in her column. The lady came over and by the end of the conversation, we
both had tears in our eyes. She was buying raffle tickets for her mom and siblings. The
first anniversary of her father’s death was the next day (he died from IPF). When she saw
it in the newspaper, she said ―this is right‖ and she had to do this.

How have you been able to promote the raffle fundraiser?
Through my newsletter (see, available twice a year via
print and email), and I have promoted it in-store – the quilt is hanging there.

There are signs on it, and we are very verbal about pointing it out. One of my staff also
volunteers at the Gorman Farm, a working farm and living museum about a mile and a
half from our store where a quilting group meets every Wednesday night – they offered
to have the raffle quilt displayed there so that they can sell tickets too.
What are people saying about the quilt? IPF?
They love the quilt. It is much prettier in person than in the picture. Folks always want to
ask me how Tim is. I am still learning how to answer their questions. Folks have the
misconception that this is something you feel bad for a while and then you get better. I
usually say, he is stable, he is no worse. Depending on who it is – I might also say ―with
this disease there really is no better. It is progressive. It won’t let go of you.‖ And then
they often say, ―Oh, I didn’t know that.‖ I tell them that ―diseases get placed in different
categories…acute, chronic and terminal. IPF is terminal.‖ That is where the light bulb
really goes off. People, for the most part, don’t have that understanding. They haven’t
spoken with others who have had IPF.

Two customers have had a close experience [with IPF] with a family member or a person
they work with. I have one customer who has IPF and wears her oxygen into the store
and has been a very big help to me in trying to get my hands around who I am with this.

How much money do you hope to raise?
My initial goal was $500, but I think we are going to do better than that. Our goal is to do
this every year. This isn’t a one-time shot.

When is the raffle itself being held?
We’re selling raffle tickets now, every day. We will keep going until Oct. 13, the date of
the drawing.

Will there be an event around it?
We decided to hold it in October because of IPF Awareness Week.

Are you planning to tell media about it in your area?
Yes, we contacted our local newspaper and they have already done a local story in a
column. It generated much interest in our fundraiser.

What do you hope to achieve with the quilt raffle, other than raising money?
I can keep this going – as long as I have the shop – over several years it will really add

What would you tell other people who are interested in doing a fundraising
project of their own?
Don’t wait too long. Don’t sit and wait for someone to come to you with an idea. Come
up with your own!
Recommended Reading for IPF Patients and Caregivers
The following books offer information on lung disorders, and are available at major
booksellers nationwide:

Breathe Better, Live in Wellness: Winning Your Battle Over Shortness of Breath.
By Jane M. Martin

Healing After Loss: Daily Meditations for Working Through Grief
By Martha Whitmore Hickman

The Breathing Disorders Sourcebook
By F.V. Adams, M.D.

Shortness of Breath: A Guide to Better Living and Breathing
By A.L. Ries, et al

The Lung Transplantation Handbook
By K.A. Coulture

Coping with Prednisone
By E. Zukerman and J.R. Ingelfinger, M.D.

The Official Patient’s Source Book on Idiopathic Pulmonary Fibrosis
By J.N. Parker & P. Parker

Share the Care: How to Organize a Group to Care for Someone Who Is Seriously Ill
By C. Capossela & S. Warnock

Taking Flight - Inspirational Stories of Lung Transplantation
Compiled by Joanne Schum, Authored by lung recipients around the world
Maine Fundraising Event Raises $2,200 for CPF

When Carolyn Krahn’s father, William ―Bill‖ Cousins, was diagnosed with IPF in March
2005, the marketing expert was empowered to do something about it. She soon realized
the treatment options for her 75-year-old father, a retired school teacher, were limited. At
the same time, Krahn of Bethel, Maine, discovered the CPF and was introduced to
resources that helped her father and her to understand and better cope with the disease.

Inspired by IPF patient and CPF member Frank Cabral, who was successfully
transplanted in 2005 and has held fundraising efforts of his own, including an
―Everybody Loves Frank‖ event in 2005 that raised more than $12,000, Carolyn sought
to make a difference for IPF patients like her father. She planned a concert event
featuring a popular local all-male a cappella singing group called ―The Bowdoin College
Meddiebempsters‖ at the local private high school, Gould Academy, where her father
taught for 34 years.

The event, held on Feb. 18, 2006 raised more than $2,200 for the CPF to support efforts
to fight IPF. In a town with only 2,000 residents, Krahn says she was happy with the
results of her efforts.

Cousins was unable to attend the fundraiser held in his honor due to a heart attack he
suffered just 10 days before the event. However, more than 200 people attended despite
the lowest temperatures of the year in Bethel, recording two below zero on Feb. 18.

―I would like to do this again, maybe when the weather is a bit better,‖ said Krahn. ―The
location was terrific and there was lots of interaction with the singers and the audience.
They have lots of respect for my Dad and lots of interest in helping.‖

Krahn has advice for IPF patients and their friends and families who are thinking of
doing a local fundraising event of their own. She recommends the CPF Fundraising Kit, a
free kit that provides simple-to-use guidance for creating any sized event. ―People really
want to know what they can do. As a daughter, [at father’s diagnosis], I kind of felt
helpless. I thought with my background, maybe I could hold an event. But anyone can do
this. Don’t be afraid to ask entertainers and others to volunteer and donate. People are
generous about giving.‖

For more information on how to host a fundraising event in your community, please
contact the CPF at (888) 222-8541 or visit the fundraising section of our Web page at
Saturday, June 3, 2006

University of Minnesota’s Center for Advanced Lung Disease
Will Host the Second Annual Pulmonary Fibrosis/Interstitial Lung Disease Education
Day on Saturday, June 3, 2006 in Minneapolis, Minn.

The University of Minnesota in partnership with the Coalition for Pulmonary Fibrosis
(CPF) is proud to announce the second annual Pulmonary Fibrosis and Interstitial Lung
Disease Education Day. The seminar offers an opportunity for patients and families
living with pulmonary fibrosis to learn more about their disease. There is no charge to
attend, and the event is open to all those interested in learning more about pulmonary
fibrosis or interstitial lung diseases. CMU credit is available for healthcare professionals
interested in attending.

Timothy Whelan, M.D., the U of M’s Interstitial Lung Disease Program director, and
Marshall Hertz, M.D., director of the Center for Advanced Lung Disease will host the
event. The seminar will address:
 • Overview of Pulmonary Fibrosis and Interstitial Lung Disease
 • Clinical Trials and Current Treatments
 • Lung Transplantation
 • Genetics of Pulmonary Fibrosis
 • Oxygen Management
 • Pulmonary Rehabilitation
 • Patient and Family Resources and Support Services

This is a unique opportunity for patients, family members, caregivers — anyone affected
by IPF to learn about the latest in IPF diagnosis, research, and treatment from leading IPF
researchers and pulmonary experts.

For more information, or to register, please contact the Center for Advanced Lung
Disease’s Administrative Director Joslyn Biever at (800) 646-9255.

What:           Pulmonary Fibrosis and Interstitial Lung Disease FREE Education Day
When:          Saturday, June 3, 2006 8:15 a.m. - 3:00 p.m.
Where:         University of Minnesota - McNamara Alumni Center
               200 Oak St. SE
               Minneapolis, MN 55455
2006 Spring IPF Seminars

Sutter Medical Center Pulmonary Rehab Department

The Coalition for Pulmonary Fibrosis (CPF) in partnership with the Pulmonary
Rehabilitation Department of the Sutter Medical Center in Sacramento, Calif. is hosting a
free seminar on Saturday, April 22 for patients and families living with idiopathic
pulmonary fibrosis (IPF). This is a unique opportunity for patients, family members,
caregivers — anyone affected by IPF to learn about the latest in IPF diagnosis, research
and treatment from leading IPF researchers and pulmonary experts.

What:          ―Living with IPF‖ Free Educational Seminar
When:          Saturday, April 22, 2006
               8:45 a.m. – 1:00 p.m.
               Complimentary breakfast and lunch
Where:         Sutter Cancer Center – Sutter General Hospital
               2800 L Street, 1st floor, Classrooms 1,2,3,4
               Sacramento, CA 95816

University of Miami Miller School of Medicine

The Coalition for Pulmonary Fibrosis (CPF) in partnership with the University of Miami
Miller School of Medicine in Miami, Florida is hosting a free seminar on Sunday, April
23 for patients and families living with idiopathic pulmonary fibrosis (IPF).

What:          ―Living with IPF‖ Free Educational Seminar
When:          Sunday, April 23, 2006
               8:45 – 1:00 a.m.
               Continental breakfast included
Where:         The Rosenstiel School of Marine and Atmospheric Science (RSMAS)
               4600 Rickenbacker Causeway (on Virginia Key)
               Miami, FL 33149

For more information, or to register for either of these events, please contact the CPF at
(888) 222-8541 or visit
CPF Names Teresa Geiger Vice President of Patient Outreach and
The Coalition for Pulmonary Fibrosis (CPF) recently named former CPF board member
and Secretary Teresa Geiger (formerly Teresa Barnes) vice president of Patient Outreach
and Advocacy. Geiger previously served as a founding member of the CPF Board of
Directors since the foundation’s inception in 2001.

In her new role, Geiger will be responsible for leading the CPF’s educational and
advocacy programs, including its ―Living with IPF‖ national education series, its national
network of support groups, and leading all public and media relations efforts. Idiopathic
pulmonary fibrosis (IPF) is a progressive and generally fatal disease characterized by
scarring of the lungs and affects more than 83,000 Americans.

―We are happy that Teresa is joining the CPF executive team,‖ said Mark Shreve, chief
executive officer of the CPF. ―She was instrumental in the creation of the CPF almost six
years ago and has been a very active advocate, an instrumental board member and a
positive voice for the IPF community. IPF has hit Teresa’s family hard with the loss of
four family members. It is her personal connection to IPF that gives her the passion to
make even greater things happen for the CPF.‖

Geiger has participated in the CPF’s National IPF Awareness Week, an effort to build
awareness about IPF, for the past three years and has met with more than 50
Congressional leaders about the need for more awareness, education and research
directed toward IPF, a disease that has no effective treatments and no cure.

Geiger lost her father to IPF in 1996, an uncle in 2001, an aunt in 2003 and another uncle
in 2005, all within the same sibling group. The last surviving relative in this sibling group
is currently suffering from IPF. More of Teresa’s family story can be found at

―I am honored to join the CPF team and to expand my role with this incredible
organization,‖ said Geiger. ―I feel so lucky to be able to realize my life’s passion as my

In addition to her position with the CPF, Geiger currently serves on the Board of
Directors of the Lung & Critical Care Research Division at the University of Colorado
Health Sciences Center and provides counsel for the National Emphysema/COPD
Association. Geiger received her journalism degree from the University of North
Carolina at Chapel Hill.
New IPF Support Groups

Charleston, SC
Pulmonary Fibrosis Support Group in partnership with the Medical University of South

Time: First meeting is Thursday, May 4, 2006 from 6:00 p.m. to 9:00 p.m.;
future meetings to be determined by the group.
Location: Institute of Psychiatry on the Medical University of South Carolina campus;
67 President Street. Parking is directly across the street in Lot G.
Contact: For additional information or to RSVP, please contact Ruth Oser at 843-792-
3168 or by email at

Sleepy Hollow, NY
Pulmonary Fibrosis Support Group in conjunction with the American Lung Association
(ALA) Better Breathers Club

Time: First meeting was Tuesday, March 14; please call or email for future meeting
Location: Phelps Memorial Hospital Center Sleepy Hollow – walkway conference
Contact: RSVP is not required, but appreciated by calling Susan DiFabio, RT, at 914-
366-3712 or by email at
CPF Scientific Advisory Board
Paul W. Noble, M.D. - Chairman
Professor of Medicine
Yale University School of Medicine

Harold R. Collard, M.D.
Assistant Clinical Professor of Medicine & Coordinator, Interstitial Lung Disease Program
Division of Pulmonary and Critical Care Medicine, University of California San Francisco

Serpil C. Erzurum, M.D.
Director, Lung Biology Program
Cleveland Clinic Foundation

Adaani Frost, M.D.
Professor of Medicine
Baylor College of Medicine

Marilyn Glassberg, M.D.
Assistant Professor of Medicine
University of Miami/Jackson Memorial Medical Center

Jeffrey Golden, M.D.
Medical Director, Lung Transplantation
Director, Interstitial Lung Disease Clinic, University of California, San Francisco (UCSF)

James E. Loyd, M.D.
Medical Co-Director, Lung Transplant Program, Vanderbilt University Medical Center

Kevin O. Leslie, M.D.
Consultant Pathologist
Mayo Clinic, Scottsdale, AZ

Fernando J. Martinez, M.D.
Director, Lung Volume Reduction Program, Medical Director, Pulmonary Diagnostic Services,
University of Michigan Medical Center

Maria Padilla, M.D.
Director, Advanced Lung Disease Program, Mount Sinai Medical Center, New York, NY

Ganesh Raghu, M.D.
Professor of Medicine, University of Washington, Director, Lung Transplant Program and
Interstitial Lung Disease Program, University of Washington Medical Center, Seattle, WA

Glenn Rosen, M.D.
Director, Interstitial Lung Disease Clinic, Stanford University Medical Center

Cecelia M. Smith, D.O.
Medical Director, Reading Hospital & Medical Center, West Reading, PA

Robert M. Strieter, M.D.
Chief, Division of Pulmonary and Critical Care Medicine, University of California, Los Angeles

CPF Board of Directors
Shirley Becker
Family member of IPF patients

Celeste Belyea, RN, RRT
Editor, The Pulmonary Paper

Paul W. Noble, M.D.
Professor of Medicine
Yale University School of Medicine

Deirdre R. Roney
Family member of eight IPF patients

Marvin I. Schwarz, M.D.
CPF Chairman & The James C.
Campbell Professor of Pulmonary
Medicine & Head, Division of Pulmonary Sciences and Critical Care Medicine, University of
Colorado Health Sciences Center

Gregory Tino, M.D.
Associate Professor of Medicine,
Director, Pulmonary Outpatient Practice, Pulmonary, Allergy and Critical Care Division,
University of Pennsylvania Medical Center
Profile: CPF Scientific Advisory Board Member

Ganesh Raghu, M.D., FCCP, FACP
Professor of Medicine & Lab Medicine (Adjunct)
Division of Pulmonary & Critical Care Medicine
University of Washington, Seattle, WA

Dr. Ganesh Raghu is a leading international authority in interstitial and fibrotic lung
disease. Educated in India, Dr. Raghu completed post-doctoral residencies in Internal and
Chest Medicine in England, U.K. and the State University of New York School of
Medicine at Buffalo, where he also served as a chief medical resident. He then joined the
University of Washington (UW) initially as a senior clinical fellow in Pulmonary and
Critical Care Medicine and subsequently as the senior research fellow in Lung Cell
Biology (Dept of Pathology). Dr. Raghu has been at the University of Washington since
1981 and he is currently a professor of medicine & lab medicine (adjunct) in the
Pulmonary and Critical Care Medicine Department at the University of Washington
Medical Center. He is also the chief of the Chest Clinic, the director of the Interstitial
Lung Disease, Sarcoid and Pulmonary Fibrosis Program and medical director of the Lung
Transplant Program.

Dr. Raghu’s research has continued to break new ground in IPF research since the 1980s
and he pioneered the first study using novel agents for IPF including pirfenidone in IPF
and introduced the concept of antifibrotic treatment for IPF in 1999. His research has
been published in the New England Journal of Medicine, The American Journal of
Respiratory and Critical Care Medicine and The European Respiratory Journal, just to
name a few.

Dr Raghu has served on the editorial boards of top medical journals including THORAX,
CURRENT OPINION and currently serves on the editorial board of the European
Respiratory Journal. He holds leadership roles in the American Thoracic Society and the
American College of Chest Physicians and serves as a steering committee member of the
newly formed IPF Network, created by the NIH to further IPF research and to improve
the standards of care for IPF patients.
Supporting the CPF

The Coalition for Pulmonary Fibrosis relies on the contributions of individuals,
corporations and associations who share our commitment to improving awareness and
education of IPF, and improving the quality of life for patients fighting IPF nationwide.
Through your generous support, the CPF will continue to provide information, resources,
and support to more than 83,000 IPF patients, caregivers and families, and to the
healthcare professionals who treat them.

To contribute by phone using any major credit card, please call the CPF at (888) 222-

Should you wish to make a tax-deductible contribution to the CPF, we encourage you to
send your check or money order to:

       Coalition for Pulmonary Fibrosis
       Suite F, #227
       1659 Branham Lane
       San Jose, CA 95118-5226

Contributions are also accepted online by bank transfer or by using any major credit card
safely and securely through PayPal. The CPF’s PayPal ID
is Contributors can visit our secure PayPal link at, or by visiting

If you have any questions about your contribution to the CPF, or if you would like to
make a restricted donation to advance specific CPF programs or research efforts, please
contact us at (888) 222-8541, or by email at
About the Coalition for Pulmonary Fibrosis
The Coalition for Pulmonary Fibrosis (CPF) is a 501(c)(3) nonprofit organization,
founded in 2001 to further education, patient support and research efforts for interstitial
lung disease, and specifically pulmonary fibrosis. The CPF is governed by the nation’s
leading pulmonologists, individuals affected by pulmonary fibrosis, medical research
professionals and advocacy organizations. With more than 9,500 members nationwide,
the CPF is the largest nonprofit organization in the country dedicated to helping those
with pulmonary fibrosis. The CPF’s nonprofit partners include the Caring Voice
Coalition, the Genetic Alliance, the Mary D. Harris Memorial Foundation, the National
Coalition of Autoimmune Patient Groups, the National Organization for Rare Disorders
(NORD), The Pulmonary Paper, the Second Wind Lung Transplant Association, and
more than 30 leading medical and research centers nationwide. For more information
please visit or call (888) 222-8541.

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