ACTION ALERT 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 strategies.‖ 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 Asthma. 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 link? 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 smoking. 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 diagnosis. 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 biopsy. ―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 microarray 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 common. 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 Association. 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 masked. ―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: http://capwiz.com/coalitionforpf/dbq/officials/. Legislation May Affect Oxygen Equipment and Services for IPF Patients 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 www.coalitionforpf.org 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 www.auntruthsscrapbasket.com, 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 up. 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 http://www.coalitionforpf.org/AboutUs/contribute. SAVE THE DATE 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 Campus 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 www.coalitionforpf.org. CPF Names Teresa Geiger Vice President of Patient Outreach and Advocacy 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 http://www.coalitionforpf.org/AboutUs/pressroom/pressroom.asp. ―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 career.‖ 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 Carolina 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 email@example.com. 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 dates. Location: Phelps Memorial Hospital Center Sleepy Hollow – walkway conference room. Contact: RSVP is not required, but appreciated by calling Susan DiFabio, RT, at 914- 366-3712 or by email at firstname.lastname@example.org. 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 (UCLA) 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- 8541. 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 email@example.com. Contributors can visit our secure PayPal link at http://www.coalitionforpf.org/AboutUs/contribute, or by visiting www.paypal.com. 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 firstname.lastname@example.org. 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 www.coalitionforpf.org or call (888) 222-8541.