ACTION ALERT
JANUARY – MARCH 2007
Coalition for Pulmonary Fibrosis and American Thoracic Society Award $100,000 Pulmonary Fibrosis Grant to Johns Hopkins Researcher
CPF and ATS Jointly Fund Two-Year Study for Pulmonary Fibrosis
The Coalition for Pulmonary Fibrosis (CPF) today announced a jointly funded $100,000, twoyear research grant with the American Thoracic Society (ATS), one of the world’s leading professional organizations for pulmonary, critical care and sleep physicians. The grant will go to help find the causes of pulmonary fibrosis, which may lead to treatments and a cure for the deadly disease that claims 40,000 lives each year. The American Thoracic Society/Coalition for Pulmonary Fibrosis Partnership Research Award in Pulmonary Fibrosis was awarded to Sonye K. Danoff, M.D., Ph.D., assistant professor of medicine of the School of Medicine, Johns Hopkins University, for research in pulmonary fibrosis. Entitled ―VEGF: Marker or mediator of lung injury in pulmonary fibrosis?,‖ Dr. Danoff’s research project will test the hypothesis that locally elevated levels of vascular endothelial growth factor (VEGF) in the lungs of patients with autoimmune pulmonary fibrosis contribute to disease progression. ―It is truly an honor to have received this award,‖ said Dr. Danoff. ―My most heartfelt thanks go to CPF for supporting this research. Too little is known about pulmonary fibrosis – a disease which kills far too many. The ATS/CPF research grant will give me the opportunity to explore how angiogenic factors contribute to the development of pulmonary fibrosis. My hope is that this understanding will translate into new therapeutic strategies for our patients.‖ The ATS/CPF grant was established in 2006 and was open to U.S.-based investigators working on translational studies in pulmonary fibrosis. Dr. Danoff’s project was chosen by the ATS Scientific Advisory Committee through a rigorous peer-review application process. ―We are pleased that we can partner with the ATS to advance important IPF-related research that will help us to better understand this devastating disease,‖ said Marvin I. Schwarz, M.D., chairman of the CPF and the James C. Campbell Professor of Medicine at the University of Colorado Health Sciences Center in Denver. ―Dr. Danoff’s project may help to identify a critical marker that may play a role in the development of IPF.‖ ―This award is incredibly important to uncovering novel insights into the mechanism of disease pathogenesis in pulmonary fibrosis,‖ said Thomas R. Martin, M.D., past president of ATS and chair of the ATS Research Program. ―Our partnership with the CPF allows us to fund new and emerging research that might not otherwise receive the funding it needs and the priority it deserves.‖ The CPF and ATS intend to continue this important partnership by establishing a second $100,000, two-year grant in 2007 to advance pulmonary fibrosis research efforts in the
United States. Details will be announced during the American Thoracic Society’s annual meeting in San Francisco in May 2007.
ATS Research Program Serves as ―Bridge for Young Investigators‖
Over the last five years, the ATS Research Program has grown tremendously, providing nearly $4 million in awards to 27 young researchers investigating a wide spectrum of lung diseases ranging from asthma and COPD to pulmonary fibrosis and alpha-1 antitryopsin. Not only have these awardees produced some of the latest science in these fields, but their participation in the Research Program has led to publications and to other funding. ―Since its inception in 2002, the program has advanced pulmonary, critical care and sleep medicine by providing individuals at the beginning of their careers with opportunities to conduct independent research, improve their skills in the lab and gather the data necessary to secure other grants,‖ said Thomas R. Martin, M.D., former ATS president and chair of the Scientific Advisory Committee, which will announce the new awardees soon. The program’s success, he adds, is evidenced by its growth – this year, it will award $1.4 million to select investigators – and the accomplishments of past awardees.
Source: American Thoracic Society
About the American Thoracic Society (ATS)
The American Thoracic Society (ATS) is a non-profit, international, professional and scientific society for respiratory, critical care and sleep medicine. The ATS is committed globally to the prevention and treatment of respiratory disease through research, education, patient care and advocacy. The long- range goal of the ATS is to decrease morbidity and mortality from respiratory disorders and life threatening acute illnesses in people of all ages. In keeping with these goals, the American Thoracic Society interacts with both national and international organizations which have similar goals. For more information please vis it www.thoracic.org.
Congressman Norwood, an IPF Patient and Advocate Declines Further Hospital Treatment
U.S. Rep. Charlie Norwood, DDS (R-10) has declined further treatment at Georgetown University Medical Center in Washington, DC and will return to his Augusta home for hospice care. Dr. Norwood decided to forego further hospital treatment after battling non-small cell lung cancer (NSCLC) since mid-November. He was initially treated with chemotherapy at Inova Fairfax and Inova Mount Vernon Hospitals, both in the Virginia suburbs outside Washington. He was transferred recently to Georgetown University Medical Center in Washington where he was evaluated for Cyberknife laser surgery as a means of combating the NSCLC tumor located in his liver, but has declined the procedure. The Norwood family thanks all who have helped through Congressman Norwood’s extensive
health battles since 1998 stemming from Idiopathic Pulmonary Fibrosis. They request continued prayers for Charlie and his family.
Source: Rep. Norwood’s Congressional Office
As this newsletter went to print, the CPF learned that its dear friend and staunch IPF advocate, Rep. Charlie Norwood of Georgia, had been admitted to hos pice care in his Augusta, Georgia home. Rep. Norwood received a lung transplant for IPF in 2004. The CPF and its membe rship are deeply grateful to Congressman Norwood for his willingness to discuss his condition publicly and raise awareness of this dreaded disease. He also introduced legislation recognizing IPF for the first time in Congress, and has been a source of inspiration and s upport for patients and families around the country. We ask that the IPF community keep Rep. Norwood and his family in their thoughts and prayers.
CPF and University of Chicago Researcher Featured on Nationally Syndicated Radio Health Journal Program
The Coalition for Pulmonary Fibrosis (CPF) was featured on the nationally syndicated ―Radio Health Journal‖ radio show in an interview that aired nationwide between Nov. 26 and Dec. 2 on more than 400 radio stations. The CPF’s Teresa Geiger, vice president of Patient Outreach & Advocacy, and Imre Noth, M.D., assistant professor of medicine at the University of Chicago were interviewed by Reed Pence. Their 10-minute interview is the second of three interviews included in the 28-minute program. In the interview, Geiger and Dr. Noth shared pertinent information on the devastating and deadly lung disease that affects 128,000 Americans, with an incidence and prevalence increase of more than 150 percent in the last five years. To listen to the Radio Health Journal online, please visit the CPF Web site at www.coalitionforpf.org.
Editor’s Note: Like many other clinical hypotheses about potential causes of IPF,
environmental exposure by first responders and workers at the World Trade Center (WTC) site is one researchers are looking at with a careful eye. While a link between the environmental exposures suffered by WTC responders and IPF is possible, it has not been conclusively proven. The CPF includes the following stories as a matter of human interest, since there are several stories in the media discussing the potential connection between 9/11 responders and IPF.
Suit Links Rare Lung Disease to 9/11
A $20 million wrongful death suit has been filed against New York City by the wife of a utility repairman who died of a rare lung disease. The suit contends Mark DeBiase, 41, contracted pulmonary fibrosis as a result of his work at Ground Zero to restore essential cell phone service after Sept. 11, 2001, The New York Post reports. Legal papers filed by lawyer Andrew Carboy accuse the city of failing to provide DeBiase with protective gear to keep him from breathing toxic air and dust while toiling for weeks at Ground Zero and the Fresh Kills landfill. A Coast Guard veteran, DeBaise left behind a wife, Jean Marie, and three sons aged 12, 9 and 8. Two other first responders, Detective James Zadroga and Police
Officer Cesar Borja, also died of pulmonary fibrosis. The DeBaise case is one of the first wrongful death suits filed by a World Trade Center responder that specifically links the rare lung disease to Ground Zero exposure.
Source: UPI
The CPF was saddened to learn that forme r New York City police office r Cesar A. Borja, a 9/11 responder at ground zero, s uccumbed to IPF on Tuesday, Jan. 23, according to multiple news reports. It is of special note that his son, Ceasar Borja, was personally invited to attend the State of the Union address on Capitol Hill by Senator Hillary Rodham Clinton. Borja did so even though he had been notified only hours earlier of his fathe r’s passing, which demonstrates his commitme nt to bringing attention to the cause. IPF patients nationwide are grateful to Mr. Borja for his dis play of courage in light of his family’s loss, which has raised aware ness of the desperate need for improved understanding and aware ness of IPF.
Officer Who Epitomized Ills of Ground Zero Workers Dies
By Sewell Chan Published: January 24, 2007 in The New York Times
A former New York City police officer died of a lung disease last night, hours before his son attended the State of the Union address to draw attention to the plight of 9/11 rescue workers like him who became ill after they were exposed to toxic dust at ground zero. The police officer, Cesar A. Borja, 52, died around 6:15 p.m. at Mount Sinai Medical Center, where he was enrolled in a monitoring and treatment program for ground zero workers, said Lauren Woods, a hospital spokeswoman. Officer Borja died of pulmonary fibrosis, a type of chronic lung disorder that involves scarring of the tissue between the air sacs. Officer Borja had been in intensive care and had been accepted as a potential candidate for a lung transplant, but his critical condition, complicated by infection, precluded him being listed to receive a lung, said his physician, Dr. Maria L. Padilla. A Congressional official briefed on the officer’s case, who spoke on condition of anonymity because of federal health privacy rules, said that federal officials had approved government financing for virtually all of Officer Borja’s care — an acknowledgment that his condition was linked to work at ground zero. Officer Borja’s 21-year-old son, Ceasar, had been invited to attend President Bush’s address as a guest of Senator Hillary Rodham Clinton. On Monday at ground zero and again yesterday in Washington, the young man stood next to Mrs. Clinton and discussed the need for federal financing for treatment of 9/11 workers. ―It is really painful for me to be here, so close to where my father contracted this disease,‖ Ceasar Borja said in New York. He said he was trying to stay strong for his mother and two younger siblings and added, ―9/11 did not end that day.‖
The son was at a dinner at Bullfeathers, a Capitol Hill restaurant, when he received a phone call notifying him of his father’s death, according to an official briefed on the situation. Four Congressional aides were at the dinner, along with several other guests invited to attend the presidential address in order to draw attention to 9/11 responders. Those at the dinner tried to comfort Ceasar Borja, the official said, and he decided to go through with his plan to attend the address. Officials warned last month that money for two major monitor ing and treatment programs — one run by Mount Sinai and the other by the city’s Fire Department — would run out in a matter of months. Representative Vito J. Fossella, the only Republican House member from New York City, has urged White House officials to support the workers, but so far he has evidently not met with success. President Bush did not mention the 9/11 workers in his address last night.
Running 50 Miles for My Dad and the CPF
By David S. Houston
Shortness of breath stopped my dad from going to karate. Shortness of breath stopped my dad from walking on the treadmill. Increasing shortness of breath and the fear of ―pneumonia‖ forced my dad to finally seek medical help. His shortness of breath continued even after April 2005 when he underwent heart bypass surgery. Though the surgeon noted ―something‖ on his lungs, they decided not to worry about it until later. As dad recovered from the heart surgery, we decided that our family would run/walk the 2006 Heart Run 5k together to celebrate our dad surviving heart disease. It was again, the unrelenting shortness of breath that kept dad from running or walking with us in April of that year. Finally, the root of the shortness of breath was revealed. A referral to a pulmonary specialist resulted in a diagnosis of idiopathic pulmonary fibrosis or IPF, a lung disorder which slowly steals away its victim’s ability to breathe. There is no known cause and no cure. Equally troubling is that the median survival rate for IPF patients is less than three years. The more I learned about IPF and how it would affect our family, the more sense of hopelessness crept into my thoughts. Our father is sick and there is nothing we can do to help him. Unlike many medical conditions, IPF has no cure and until very recently, there were no uniform diagnostic standards for IPF. Even now there is no FDA-approved treatment for IPF. Researching IPF did not provide a great deal of hope. In my search, however, I found the Coalition for Pulmonary Fibrosis (CPF), which is leading the charge to further education, patient support and research efforts for pulmonary fibrosis. With that discovery I also found a way to not only honor my father during his struggle, but to help other families who have been impacted or will be impacted by this horrific condition. I am determined to raise money and raise awareness with their help. On April 14, 2007, I will be running the American River 50 Mile Endurance Run (www.run100s.com/AR50). This run will be 50 miles for my father and for others suffering from IPF. Together we can fight the shortness of breath that is taking the lives of so many. Oh, and by the way, the shortness of breath may stop my dad from doing many of the physical activities he wants to do, but it will never stop his spirit and his fight against this deadly disease.
To support David’s effort, which will directly benefit the CPF, visit his Web site at www.firstgiving.com/leehouston.
UCSF and Seton Medical Center Initiate Study of Pulmonary Rehabilitation and its Impact on IPF
The University of California - San Francisco (UCSF) and Seton Medical Center (Daly City, Calif.) are currently conducting a study investigating the role of pulmonary rehabilitation in patients with pulmonary fibrosis. The goal of the study is to determine the impact of short-term pulmonary rehabilitation on dyspnea (shortness of breath), exercise capacity, pulmonary function and health-related quality of life in patients with fibrotic interstitial lung disease. Although studies have shown important benefits to pulmonary rehabilitation in patients with chronic obstructive pulmonary disease (emphysema and chronic bronchitis), its role in patients with pulmonary fibrosis remains unclear. Researchers are actively seeking 50 patients for participation. Patients must be referred by their treating physician for pulmonary rehabilitation and be able to travel to Seton Medical Center. Patients interested in participating or seeking more information should contact Cyrus Shariat at 916-397-1331 or email cyrus.shariat@ucsf.edu, or Chris Garvey at 650-991-6776 or email chrisgarvey@dochs.org.
Shionogi Reports Encouraging Results of Phase III Clinical Trial of S-7701 (pirfenidone) for IPF Treatment
Japanese pharmaceutical company Shionogi & Co., Ltd. announced that it has achieved the primary objectives of Phase III clinical trials for the idiopathic pulmonary fibrosis treatment S7701 (generic name: pirfenidone), which the Company is developing in Japan under a license from U.S.-based Marnac, Inc. and KDL, Inc. of Tokyo. In the Phase III clinical trials for this drug with vital capacity (VC) change (from before commencement of treatment to 52 weeks after commencing treatment) as the primary endpoint, both high and low doses of the drug (600mgl per day, three times a day and 400mgl per day, three times a day, respectively), significantly inhibited worsening of the condition compared with a placebo. While continuing to conduct further analysis and study, Shionogi plans to expedite the application process based on these clinical results with the intention of submitting a new drug application (NDA) within the current fiscal year. InterMune (Brisbane, Calif.) is also studying the use of pirfenidone for the treatment of patients with idiopathic pulmonary fibrosis in its Phase III ―CAPACITY‖ clinical trials. InterMune commented on the Shionogi clinical trial in a regulatory filing with the Securities & Exchange Commission (SEC) that ―while we are encouraged by Shionogi’s announcement, we believe that the Shionogi findings are informative but not necessarily predictive of a potential outcome of InterMune’s CAPACITY trials. There are key differences between the Shionogi and InterMune clinical trials, including differing patient populations, dosing regimens and length of treatment. There is no assurance that the results of the Shionogi and InterMune clinical trials will be similar.‖ InterMune has worldwide rights, excluding Japan, Korea and Taiwan, to develop and
commercialize pirfenidone for all fibrotic diseases.
Source: Shionogi Press Release 12/22/06 & InterMune SEC Filing 12/22/06. Content edited for space.
Proteins May Predict Lung Transplant Rejection
Using the latest in high tech tools, researchers have identified three proteins that were highly predictive of chronic lung rejection up to 20 months before the rejection occurred. ―Lung transplant patients have the highest mortality rate of organ recipients, about 45 percent over five years,‖ said lead investigator and pulmonologist Chris Wendt. ―Currently, there is no reliable way to predict which transplants will fail. When signs of chronic rejection appear, it is usually too late to reverse it. If doctors can predict which patients are beginning to reject the transplanted organ, they could try to head it off.‖ The study, ―Proteomic biomarkers of chronic lung allograft rejection,‖ was carried out by Wendt, Tereza Cervenka, Madelaine Haddican, Yan Zhang and Gary Nelsestuen, of the University of Minnesota. The researchers used the power of computers and new, sophisticated methods of analysis to find the proteins that form a ―biosignature‖ or ―biomarker‖ of organ rejection from among the thousands of proteins that exist in the lung. DISEASE DISTURBS PROTEIN FUNCTION Patients who receive a new lung may suffer bouts of acute or chronic rejection. Acute rejection often responds to therapy. Chronic rejection, which results in scarring of the lung’s airways following inflammation, is irreversible. In addition, often by the time doctors make the diagnosis, the disease is already fairly advanced, says Wendt. In addition to early identification, the researchers hope to eventually open the door to developing a preventative treatment and also gain insight into the physiological mechanisms of lung rejection. The study used 411 lavage samples obtained from 137 lung transplant recipients from 1993 to 1996. Because this study looked back at patients treated years ago, the researchers could look for differences between patients who subsequently suffered chronic lung rejection and those who did not. The researchers combined proteomics (the study of proteins), the latest in mass spectrometry technology and the best analytical methods from the field of bioinformatics (the use of computers and statistics to analyze and find patterns in scads of data). They hypothesized that proteins would change as chronic lung rejection developed and that the new technology would make it possible to find these patterns from among the thousands of proteins at work in the lungs. An earlier study found that HNP was three times more likely to be elevated among those who later suffered chronic rejection. Later analyses identified 265 proteins that were upregulated in lung rejection up to 20 months before it happened. With yet more research, they found the following became elevated with chronic rejection:
• matrix metalloprotein-9 (66 times more likely to suffer rejection) • proteinase 3 (nine times more likely) ―Preliminary evidence suggests these biomarkers will be an early sign of lung transplant rejection,‖ the authors wrote. The research is continuing to determine which protein combinations are the best predictors and whether some biomarkers may be better than others at different points of the disease’s development. ―The information could offer inroads to new therapies,‖ said Wendt. ―These findings could also be used to understand the physiological mechanisms that lead to lung rejection.‖ Her team has developed two mouse models to determine whether these proteins play a role in the development of chronic lung rejection or whether they are a byproduct of the disease.
Source: American Physiological Society
GAO Says Challenges Remain in Getting New Drugs to Market
Despite increased spending on drug research, the creation of new medicines has slowed, according to a recent report by the Government Accountability Office (GAO). Complex science, risk-averse business practices, confusing regulatory requirements and profitminded patenting have all impeded drug creation, the report concludes. The report, which used data on pharmaceutical expenditures and Food and Drug Administration (FDA) drug reviews, found that even though the pharmaceutical industry reported a 147 percent increase in spending on new drug research and development from 1993 to 2004, there was not a corresponding increase in new medications. The report found that the creation of innovative drugs has become stagnant. After consulting with experts, the report offered several broad reasons for the decline in drug development: • The difficulty of translating scientific breakthroughs into effective drugs has led to more failures of experimental drugs in clinical trails, which in turn leads to higher development costs. • In the past 10 years, the pharmaceutical industry has focused on creating drugs with high returns on investment, focusing on blockbuster drugs, which reap huge profits, rather than more experimental drugs or those that treat rare conditions. • Confusing and burdensome FDA regulatory standards have made pharmaceutical companies reluctant to invest in riskier and more experimental drugs. In an analysis accompanying the report, the panel of health experts recommended a number of changes to encourage the creation of new medicines, including the creation of an expedited review process for drugs treating neglected diseases. ―The GAO report confirms that the journey from laboratory to medicine cabinet has become more challenging, risky and expensive than ever before,‖ said Ken Johnson, senior vice president of the
Pharmaceutical Research and Manufacturers of America (PhRMA), in a press release.
Source: Congressional Quarterly. Content edited for space.
AARP to Start Notifying Its Members How Individual Lawmakers Vote on Key Bills
The American Association of Retired Persons (AARP) said in a letter to Congress that for the first time it will track roll call votes on key legislation and report the results to its 38 million members. ―The American people need and deserve to know where their representatives and senators stand on the issues, and AARP will deliver that knowledge on the issues of health care and financial security,‖ AARP Chief Executive Officer William Novelli wrote in the letter. AARP said the first vote it will report to its members in this way will be on H.R. 4, which would require the Department of Health and Human Services secretary to negotiate lower prices for drugs covered by the Medicare prescription drug benefit.
Source: CQ HealthBeat News. Content edited for space.
New Congress Weighs in On Health Legislation
Lowering the Costs of Medications for Medicare Patients:
The House approved legislation requiring the government to negotiate with drug companies to lower the prices of medicines for Medicare patients. Despite a veto threat from the President, democrats used their majority status to push through another of House Speaker Nancy Pelosi’s priorities for the first 100 hours of the new Congress. The vote was 255-170. The idea behind the bill is using the sheer size of the Medicare program to generate steeper discounts than private insurance plans can muster. While a majority of seniors are expressing satisfaction with the program, surveys also indicate that they overwhelmingly want the government to have the power to negotiate drug prices. A survey of seniors for the Kaiser Family Foundation showed that about 81 percent of seniors want to let the government use its buying power to negotiate drug prices, including 67 percent who said they strongly favor such negotiations. The issue is expected to have a tougher time in the Senate. However, Sen. Max Baucus (D-MT), gave supporters of the measure a lift on Thursday when he said the total prohibition on government negotiations for Medicare beneficiaries should be eliminated.
Source: AP
Expansion of Federal Spending on Embryonic Stem Cell Research:
The House again gave strong support to a bill to expand federal spending on embryonic stem cell research, ignoring a renewed threat by President Bush to veto the measure.
The bill (H.R. 3) passed Jan. 11 by a vote of 253 to 174 — still well short of the two-thirds majority that would be required to override the president. But supporters say they are gaining momentum. Under the bill, federal grants could be used for research on embryos donated by invitro fertility clinics, as long as they were not created for scientific purposes and would otherwise be discarded. Bush vetoed an identical bill last summer, and the House fell 51 votes short of an override. House supporters have picked up 18 votes since then, most, but not all, from democratic freshmen. The Senate is expected soon to pass either the House bill or similar legislation, possibly with a veto-proof majority.
Source: CQ Weekly
IMPORTANT BILLS TO WATCH
Ending the Medicare Disability Waiting Period Act of 2007
H.R. 154: Rep. Gene Green (TX) introduced into the new 110th Congress ―Ending the Medicare Disability Waiting Period Act of 2007.‖ If passed in both Houses of Congress, the legislation would amend title II of the Social Security Act to phase out the 24-month waiting period for disabled individuals to become eligible for Medicare benefits and to eliminate the waiting period for individuals with life-threatening conditions.
Look for the Reintroduction of Bills from the 109 th Congress in the New 110th Congress
Important IPF-specific legis lation:
H. CON. RES. 178: Recognizing the need to pursue research into the causes, a treatment, and an eventual cure for idiopathic pulmonary fibrosis, supporting the goals and ideals of National Idiopathic Pulmonary Fibrosis Awareness Week, and for other purposes. Sponsor: Rep. Charlie Norwood [GA-9] (introduced 6/14/2005), Cosponsors (50) Committees: House Energy and Commerce; Senate Health, Education, Labor, and Pensions Latest Major Action: Passed the House of Representatives 401-0 in October 2005. S. RES. 236: A resolution recognizing the need to pursue research into the causes, a treatment, and an eventual cure for idiopathic pulmonary fibrosis, supporting the goals and ideals of National Idiopathic Pulmonary Fibrosis Awareness Week, and for other purposes. Sponsor: Sen. Norm Coleman [MN] (introduced 9/13/2005), Cosponsors (10) Committees: Senate Health, Education, Labor, and Pensions Latest Major Action: 5/25/2006 Passed/agreed to in Senate. Status: Resolution agreed to in Senate without amendment and with a preamble by Unanimous Consent. CPF Note: The CPF expects H. CON. RES. 178 and S. RES. 236 to be re-introduced (using new resolution numbers) early this year with the help of Sen. Coleman and Rep. Norwood.
Home Oxygen Patient Protection Act of 2006
The most recent progress of the bill in the 109 th Congress was:
H.R. 5513: To amend part B of title XVIII of the Social Security Act to restore the Medicare treatment of ownership of oxygen equipment to that in effect before enactment of the Deficit Reduction Act of 2005. Sponsor: Rep. John J.H. ―Joe‖ Schwarz [MI-7] (introduced 5/25/2006), Cosponsors (84) Committees: House Energy and Commerce; House Ways and Means Latest Major Action: 6/5/2006 Referred to House subcommittee. Status: Referred to the Subcommittee on Health. S. 3814: A bill to amend part B of title XVIII of the Social Security Act to restore the Medicare treatment of ownership of oxygen equipment to that in effect before enactment of the Deficit Reduction Act of 2005. Sponsor: Sen. Pat Roberts [KS] (introduced 8/3/2006), Cosponsors (7) Committees: Senate Finance Latest Major Action: 8/3/2006 Referred to Senate committee. Status: Read twice and referred to the Committee on Finance.
Source: CQ.com
Together, we can make sure the voice of the IPF community is heard. We encourage you to write emails and letters to your Members of Congress to let them know you support the CPF’s efforts and the items listed above.
For those CPF members who have legislative experience and connections on Capitol Hill, we’d like to hear from you. Call Teresa Geiger at (888) 222-8451, ext. 702 or email tgeiger@coalitionforpf.org. For additional information about the CPF’s advocacy activities or the CPF’s Campaign ACT program, visit www.coalitionforpf.org.
Jodi Howe, RN Discusses Importance of Pulmonary Rehabilitation for IPF Patients
Interview with Jodi Howe, RN, Pulmonary Rehabilitation at El Camino Hospital, Mountain View, Calif.
Jodi Howe helped establish the first support group for IPF patients in the San Francisco Bay Area in 2001 with the help of the CPF. She continues to be a wonderful resource for not only the CPF, but for the patients and families she helps on a daily basis. We recently sat down with Jodi to discuss the role of pulmonary rehabilitation in IPF. Q: WHAT IS PULMONARY REHABILITATION (PR)? Pulmonary rehabilitation is a program that helps patients become physically stronger and can help them breathe better, as a result. In addition, pulmonary rehabilitation can help the patient learn to breathe correctly by learning ―tips‖ such as pursed-lip breathing which can help them cope with more physically demanding life tasks and activities. Q: WHAT CAN PATIENTS EXPECT WHEN THEY GO TO PR? Although PR is not a proven therapy for patients with IPF, I find that patients generally increase
their strength and endurance so that day to day activities are easier for them and their quality of life improves. They will feel more knowledgeable about how their body processes oxygen and have a better understanding of what to do when they get sick or have an infection (we teach infection control, as well). Q: DOES A PATIENT NEED A REFERRAL FOR PR? A referral is needed by a doctor. Patients must qualify for PR. Q: HOW DOES A DOCTOR DETERMINE IF A PATIENT IS WELL ENOUGH TO DO PR? Their doctor can make that determination and we do an intake interview, assessment and sixminute walk test. Even severe patients will benefit from PR, but it would be great to get them when they are not severe so they can learn early. Q: WHAT IF I HAVE BEEN DIAGNOSED AND HAVEN’T BEEN TO PR? Be proactive and ask your doctor if PR is right for you. Not all IPF patients get referred to pulmonary rehabilitation. Yet, it is one of the potential ways to improve a patient’s quality of life. We always want patients to understand the treatment options available to them, and for many IPF patients, pulmonary rehabilitation is helpful. Q: WILL I FEEL BETTER IF I GO TO PR? PR has benefits that help many IPF patients. We’ve seen it. When we teach pursed-lip breathing (PLB) – physiologically it is different than for patients with COPD, but it does help IPF patients breathe better. And, we have seen improvement in oxygen saturation when IPF patients use PLB. Q: IS PR A COMMON COMPLEMENTARY ―TREATMENT‖ FOR IPF? Many pulmonologists consider PR to be important for pulmonary fibrosis especially after a patient begins using supplemental oxygen. However, PR as a ―treatment‖ for IPF is unproven but many doctors feel it is beneficial to IPF patients. Q: ARE ALL OF THE PATIENTS IN A GIVEN PR CLASS IPF PATIENTS? At our center, about one in seven is an IPF patient. We are seeing more, I just wish we could have enough IPF patients that we could have a PR class just for them. There is a difference in the disease and the process of the disease and it would be ideal if IPF patients can go to PR with other IPF patients. However, the benefits of PR are believed by many pulmonologists to be substantial. Q: DOES INSURANCE COVER PR SERVICES? In our area, most insurances do cover PR, if the patient qualifies. Insurance coverage can be a major issue in some parts of the country as PR remains an unproven therapy for IPF. Q: WHEN A PATIENT FINISHES THEIR PR PROGRAM, DO THEY STOP PHYSICAL AND BREATHING EXERCISES? Once the program is done, we hope we have trained them to exercise on their own. Some PR centers offer a supervised exercise maintenance program at a cost to the patient. Q: HOW DO YOU FEEL ABOUT THE NEW STUDY AT THE UNIVERSITY OF CALIFORNIA, SAN FRANCISCO AND SETON MEDICAL CENTER, WHICH IS LOOKING AT FUNCTIONAL IMPROVEMENTS AND QUALITY OF LIFE MEASURES OF PR SPECIFICALLY FOR THE IPF PATIENT? I am so excited about this study. I believe it will help demonstrate what I already believe – that PR is key to a better quality of life for patients with IPF.
Q: ARE PEOPLE EVER TOO SICK TO GO TO PR? A doctor can make the determination if PR is right for a patient and if they are physically strong enough to go through it. We exercise patients here and sometimes they cannot do the physical exercise component. We give them a great education and tools to use, but the bread and butter is the exercise. If they are not well enough to exercise, PR can be difficult for them. CPF Note: The American Thoracic Society Public Advisory Roundtable, of which the CPF is a member, recently contributed comments to the Centers for Medicare and Medicaid’s national coverage analysis for pulmonary rehabilitation (CAG-00356N) and urged CMS to provide support for important pulmonary rehabilitation services.
―Thank you again, CPF, for all of your support. Your organization has been a wealth of knowledge during the progression of this disease.‖
– Julie Crouse, Family Member of IPF Patient
CPF Supporters Surpass ―Virtual‖ Butterfly Ball Fundraising Goal by Raising More than $160,000
The CPF’s extraordinarily successful research funding campaign, ―Virtual‖ Butterfly Ball, was generously supported by two unique efforts in the fall. Many donors stayed in the comfort of their homes, toasted to the event’s success, and sent their support to the CPF, while another small group of individuals attended a private gathering in Malibu, Calif. The ―virtual‖ event, promoted almost exclusively through a mail campaign, raised three times the amount of money for research than event coordinators expected. ―We were hoping the campaign would attract $50,000 which would go toward funding the CPF’s partnered grant program with the American Thoracic Society (ATS),‖ said Mishka Michon, CPF executive vice president of Development. ―Donor response was so outstanding that we realized a total of $165,000!‖ The extraordinary success of this event will allow the CPF and ATS to make a second, fully funded two-year grant award in 2007. The first grant with ATS was funded in 2006 and recently awarded to researcher Sonye Danoff, M.D., Ph.D., assistant professor of medicine of the School of Medicine, Johns Hopkins University, for research in pulmonary fibr osis. Entitled ―VEGF: Marker or mediator of lung injury in pulmonary fibrosis?,‖ Dr. Danoff’s research project will test the hypothesis that locally elevated levels of vascular endothelial growth factor (VEGF) in the lungs of patients with autoimmune pulmonary fibrosis contribute to disease progression. Concurrently, the funds will allow the CPF to maintain and grow its existing programs and services for patients and families around the country. In addition to the special ―Virtual‖ Butterfly Ball mailing to CPF constituents, the CPF’s Malibu gathering gave donors an opportunity to hear directly from: Harold Collard, M.D., assistant professor of medicine & coordinator, Interstitial Lung Disease Program at the University of
California, San Francisco and member of the CPF’s Scientific Advisory Board, as well as CPF Board member Deirdre Roney, who has lost nine family members to IPF, and Mishka Michon. Many of the ―Virtual‖ Butterfly Ball donors joined the CPF in 2005 for the inaugural B.I.G. (Breathing Is Glorious!) Butterfly Ball, which raised more than $190,000 for the CPF and was a key event in establishing the CPF’s presence in Southern California. ―The CPF is especially grateful to 2005 Butterfly Ball donors who continued their support in 2006,‖ said Michon. ―It is well understood that new research is fundamental to finding answers to the mystery of IPF and to saving lives. The donors who supported this special ―virtual‖ event have made an important contribution to search for answers. The answers to IPF are out there, and we will continue to ask for support until we find them.‖
The CPF would like to express its deepest appreciation to all ―Virtual‖ Butterfly Ball donors. Both the CPF and ATS want to especially thank these key donors for their generous support of the partnership grant:
• John Cadarette & Deirdre Roney • Steven duBrul • Bill Guthy & Victoria Jackson • Yvonne Lacko • Ozzie & Wendy Silna • Dave & Diane Steffy • Bennet van de Bunt & Laura Fox • Venable LLP
Each and every donor, at every level, is investing in the progress toward a cure. Your support helps the CPF continue its mission. To learn more about the CPF’s research efforts and how you can help, please contact Mishka Michon at (888) 222-8541 or email mmichon@coalitionforpf.org.
IPF Affects Everyone in the Family: Through the Eyes of a Child
It is true that IPF patients are adults and on average, are 65-years-old. There are many patients in their 30s, 40s, and 50s as well. As our members know all too well, it is not only t hese adults who are affected. Their children, nieces, nephews, grandchildren and great grandchildren suffer through the experience of seeing a loved one fight this devastating disease, as well. At the successful ―Virtual‖ Butterfly Ball dinner, CPF Board member and event coordinator Deirdre Roney’s son, 15-year-old Colin Cadarette, surprised his mother with a heart-felt speech that brought the audience, and his mom, to tears. The CPF would like to share an excerpt from his speech with you: Mom, it’s funny…seeing you here tonight looking lovely and incredibly excited by how well your event is going. It’s funny because I know how much pain and hard work was put into this day. I can’t imagine how hard it must be to constantly have to re-live all of your worst moments and use them as fuel for your inner fire. Fuel for your constant war against a relatively unknown disease
with no cure in sight. Remember that you are fighting not only in memory of your lost family, but also for your existing one. You are fighting for Camille [Colin’s sister] and me, and for everyone else who is young and hopes to live without fear of this disease. Now before you all get back to this wonderful event, I just want to say I have my own donation to give. Mom, I’ve been secretly working as a night janitor for one of those theaters that shows the midnight showing of Rocky Horror Picture Show…so, here is my contribution…with a little help from Ben and Laura, Bill and Dad. Together we are donating $45,000. I hope it makes this amazing night just that much better. I love you. Colin
CPF Fundraiser, Advocate Featured in National Women’s Magazine
CPF volunteer fundraiser and advocate Debbie Roney is featured in the February/March 2007 issue of Complete Woman, a national women’s magazine. Roney co-chaired the CPF’s highly successful inaugural B.I.G. (Breathing Is Glorious!) Ball event in the fall of 2005 in Chicago, raising $75,000. The 2006 B.I.G. Ball event, for which Roney served as sole chairperson, was even more successful by raising $250,000 for IPF research and patient services. The article, entitled ―Party with a Purpose‖ and written by Tracy Porpora, appears on pages 66-67 of the magazine. It is available on newsstands until March 27.
Donors are ―Bricks in the Wall‖ Says CPF Founding Partner and Supporter
When Jeff Harris lost his wife, Mary, to IPF in 1998, he decided to honor her legacy by establishing the Mary D. Harris Memorial Foundation to help find a cure for the disease that claimed her life. The foundation was one of the first partners and contributors to the CPF in 2002, and remains a valued partner today. Harris knew that there were probably thousands of other people out there who had experienced the difficulties and loss of people they loved to IPF, b ut he still felt all alone. ―I didn’t know a thing about this disease before it hit me,‖ said Harris. ―It hit me, my wife died, and it set off a trigger in me. I was sort of like the character in the movie Network – I was mad as hell and I wasn’t going to take it any more.‖ ―Someone who has been touched by the disease often has a tendency to feel like
they’re alone and no one knows what they’re experiencing,‖ he said. They are not alone anymore, he says, since the CPF was established in 2001. ―As t he CPF has grown and developed, many are realizing that there is power in numbers.‖ For example, the CPF’s advocacy efforts in Washington, D.C. have been effective, because the CPF brings patients and families directly to Washington to tell their story to Members of Congress. For the past four years, dozens of patients have traveled to Capitol Hill, representing the IPF community, putting a human face on the disease and becoming empowered by the experience. And the number of CPF members continues to climb. The membership has now grown to over 12,000. Because of the support of people like Harris and other donors, the CPF has accomplished an incredible amount in a very short time. ―We’ve come so far,‖ said CPF CEO Mark Shreve. ―We are now actively fund ing research and provid ing much needed patient support through our patient events, support groups and educational tools. Our staff and I are available directly to patients to answer their questions and concerns, supply information on emerging research and to provide referrals for them to centers of excellence. Information on IPF is also available to patients and professionals 24/7 through our web site. None of this would be possible without the continued support of our contributors and partners.‖ Harris, who received the Frank Cabral Humanitarian Award in 2005 in recognition of his efforts on behalf of IPF, is humble about his involve ment as an early supporter and donor of the CPF. ―Contributing to [the CPF] as an individual is empowering,‖ said Harris. ―It is a cause that is tangible, that you can point to and truly say you’re doing something in trying to make a difference. [Some people feel] their donation doesn’t seem to have any meaning. But regardless of the amount of money and other resources one ca n donate, it is empowering on an individual basis…like bricks build walls and walls build houses and houses build villages…it is a stepping stone process where every brick in the wall really does make a difference.‖
―The [―Living with IPF‖] seminar at the University of California, San Francisco (UCSF) brought to light for me the unique challenges and obstacles people face with pulmonary fibrosis. I was very impressed by [the CPF’s] dedication as well as the various speakers and patients alike, who are increasing public awareness for pulmonary fibrosis. The CPF is leading the way to improve the detection, diagnosis and treatment to increase quality of life, and the ongoing research effort which will eventually lead to a cure.‖
– John E. Bess III, American Lung
Association of California
CPF Announces Spring Patient Seminar in Partnership with Medical University of South Carolina
What: ―Living with IPF‖ Free Educational Seminar When: Friday, March 23, 2007
11:30 a.m. – 6:00 p.m. Complimentary lunch and snacks will be provided
Where: Medical University of South Carolina
Storm Eye Institute Auditorium 167 Ashley Ave. 8th Floor Auditorium Charleston, SC 29425 RSVP to the CPF at (888) 222-8541 or via email at tgeiger@coalitionforpf.org. Oxygen will be available at the event. Please let us know if you’ll require oxygen services when you RSVP. We apologize that we cannot return all phone calls and emails because of the volume of RSVPs we expect to receive. Please consider your voicemail or email to be confirmation of your attendance. The CPF recently set the date for an upcoming “Living with IPF” patient seminar in New York City on Saturday, April 14 in partnership with Mount Sinai Medical Center. Further details will soon be available on the CPF Web site.
The American Thoracic Society Public Advisory Roundtable is pleased to invite you to our first annual forum
Breathing Better with the ATS
to be held on Saturday, May 19, 2007 from 1:00 to 4:00 p.m. at the Renaissance Parc 55 Hotel in San Francisco, Calif. You’ll have an opportunity to hear from patients and experts in the pulmonary community and network with them afterwards. The gathering will be held in conjunction with the ATS International Conference. Hors d’oeuvres will be provided at the meeting. Please RSVP by email to par@thoracic.org no later than March 30, 2007. If you have any questions contact Karen Belgiovine (212) 315-8640.
New Support Groups Established
The CPF currently sponsors more than 40 support groups around the country. Attendance is open
to patients, their families, caregivers, and anyone interested in sharing and learning about interstitial lung diseases, including pulmonary fibrosis and idiopathic pulmonary fibrosis. The focus of our support groups is to provide patients and their loved ones with essential educational resources and support, while providing an opportunity to network and share experiences with other patients living with interstitial lung diseases.
Tualatin, OR
Pulmonary Fibrosis/Transplant Support Group In partnership with Meridian Park Hospital
Time: First Thursday of every month, 3:00 p.m. Location: Community Education Building, Room 104 (next to Meridian Park Hospital in
Tualatin) 19300 SW 65th Ave. Tualatin, OR 97062
Contact: For more information or to register:
Gary Hinton at (503) 521-8725 or email garhin@msn.com Sandy Metheany at (503) 829-5253 or email smetheany@hotmail.com Anne Richards at (503) 692-2548 or email aericha@lhs.org
Frankfort, KY
Pulmonary Fibrosis of Kentucky
Time: February 24 and March 24, 2:00 p.m. Location: Paul Sawyier Public Library, Boardroom of
the Frankfort Library 305 Wapping St. Frankfort, KY 40601
Contact: For more information, please call (502) 803-7533 or email pfofkentucky@yahoo.com
CPF to Attend National Institutes of Health, Public Interest Organization Meeting
The CPF’s Teresa Geiger will be attending the upcoming National Institutes of Health Public Interest Organization (NIH/PIO) meeting in Bethesda, Maryland, with representatives of many national lung disease organizations. The February meeting will bring together leaders of the NIH and the PIOs to allow the open exchange of ideas for making communications between the federal government and patient organizations stronger and more productive. The meeting provides an opportunity for the CPF to discuss the needs of IPF patients in an intimate forum directly with decision makers who can impact the way the disease is
treated with regards to research, education, awareness and funding.
Profile: CPF Scientific Advisory Board Chairman Paul W. Noble, M.D.
Dr. Noble is a professor of medicine at Duke University School of Medicine and Division Chief of the Interstitial Lung Disease Program at Duke University Medical Center. Prior to his work at Duke, he was a professor of medicine at Yale University School of Medicine and director of the Interstitial Lung Disease Program at Yale-New Haven Hospital. Dr. Noble graduated from New York University School of Medicine in 1984. He did his internal medicine and chief residency at the University of California, San Francisco Hospitals. He received his pulmonary and critical care training at the University of Colorado and National Jewish Medical Centers in Denver, Colorado. He was an assistant professor of medicine and established the Interstitial Lung Disease Clinic in 1992. He moved to Yale University in 1997. Dr. Noble has an active clinical practice in ILD and directs a research laboratory funded by the National Institutes of Health with the goal of understanding the pathogenesis of lung fibrosis and finding new therapies. Research from his laboratory has been published in journals such as Science, Nature Medicine, the Journal of Clinical Investigation and the Journal of Experimental Medicine. He has also co-authored clinical studies that have been published in the New England Journal of Medicine.
CPF Board of Directors
Marvin I. Schwarz, M.D. - Chairman University of Colorado Health Sciences Center Shirley Becker Family member of IPF patients Celeste Belyea, RN, RRT Editor, The Pulmonary Paper Paul W. Noble, M.D. Duke University Medical Center Deirdre R. Roney Family member of nine IPF patients Gregory Tino, M.D. University of Pennsylvania Medical Center
CPF Scientific Advisory Board
Paul W. Noble, M.D. – Chairman Duke University Medical Center Harold R. Collard, M.D. University of California, San Francisco Serpil C. Erzurum, M.D. Cleveland Clinic Foundation Adaani Frost, M.D. Baylor College of Medicine Marilyn Glassberg, M.D. University of Miami/Jackson Memorial Medical Center Jeffrey Golden, M.D. University of California, San Francisco Kevin O. Leslie, M.D. Mayo Clinic, Scottsdale, AZ James E. Loyd, M.D. Vanderbilt University Medical Center Fernando J. Martinez, M.D. University of Michigan Medical Center Maria Padilla, M.D. Mount Sinai Medical Center, New York, NY Ganesh Raghu, M.D. University of Washington Medical Center, Seattle, WA Glenn Rosen, M.D. Stanford University Medical Center Cecelia M. Smith, D.O. Reading Hospital & Medical Center, West Reading, PA
Supporting the CPF
The Coalition for Pulmonary Fibrosis (CPF) 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 128,000 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 info@coalitionforpf.org. Contributors can visit our secure PayPal link at www.coalitionforpf.org/AboutUs/contribute. 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 email info@coalitionforpf.org. About the Coalition for Pulmonary Fibrosis The Coalition for Pulmonary Fibrosis (CPF) is a 501(c)(3) nonprofit organization, founded in 2001 to accelerate research efforts leading to a cure for pulmonary fibrosis, while educating, supporting, and advocating for the community of patients, families, and medical professionals fighting this disease. The CPF is governed by the nation’s leading pulmonologists, individuals affected by pulmonary fibrosis, medical research professionals and advocacy organizations. With more than 11,000 members nationwide, the CPF is the largest nonprofit organization in the United States dedicated to advocating for those with pulmonary fibrosis. The CPF’s nonprofit partners include the American Thoracic Society, the Anne Harroun Landgraf Foundation, 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, Second Wind Lung Transplant Association, and more than 35 leading medical and research centers nationwide. For more information please visit www.coalitionforpf.org or call (888) 222-8541.