Coalition for Pulmonary Fibrosis Breathing Is Glorious

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					                                                                                                  2   Fundraising Update

                                                                                                      Research and Clinical
                                                                                                  7   Trials Update

                                                                                                 15   Campaign ACT Update


                                          OCTOBER – DECEMBER 2006
                                                                                                 18   IPF Support Groups




                 Action Alert                       The Quarterly Publication of the Coalition for Pulmonary Fibrosis



Coalition for Pulmonary Fibrosis B.I.G. (Breathing Is Glorious!) Ball
Raises More Than $250,000 for IPF
Event provides foundation for new education and research efforts to fight IPF

The CPF’s second annual B.I.G. (Breathing Is Glorious!)
fundraising event, held in Chicago, Ill. on Oct. 21, 2006,
raised more than $250,000! The gala was hosted at
the Renaissance Chicago Hotel in beautiful downtown
Chicago, and was attended by more than 350 supporters.

A percentage of event proceeds will be directed back to
the University of Chicago to improve the quality of care              “The generosity and support of all
provided to IPF patients in the metropolitan Chicago
area, and fund research to identify new approaches to             those involved in the B.I.G. Ball reinforce
treating the disease currently underway at the clinic.
The funds will also be used to further the education,
                                                                     our longstanding belief that finding a
awareness, and research efforts of the CPF.                        treatment and cure for IPF is only going
“This year’s event was incredibly successful,” said Mark               to happen by working together.”
 Shreve, chief executive officer of the CPF. “We are
 grateful to all of those who supported the event, and            – Mark Shreve, chief executive officer of the CPF
 we’re especially grateful to once again join forces
                                         Continued on next page




            “Your last issue [of the Action Alert newsletter] was very powerful.
        It was filled with information that I really desperately needed about new
             developments, drug trials and patient stories. Thank you CPF!”
                                               – Tom Coen, Seattle, WA
Fundraising Update

     Continued from previous page

     with the University of Chicago to form a successful
     partnership. The generosity and support of all those
     involved in the B.I.G. Ball reinforce our longstanding
     belief that finding a treatment and cure for IPF is only
     going to happen by working together.”

     Illinois Senator Richard Durbin served as honorary
     chairman for the event. The CPF also presented its 2006
     national awards at the B.I.G. event. Trey Schwab, former
     Marquette University assistant basketball coach and
     lung transplant survivor, was the recipient of the Frank
     Cabral Humanitarian Award, recognizing his efforts
     to significantly improve awareness of IPF through his
     work as an advocate and transplant coordinator at the
     University of Wisconsin, Madison. Talmadge E. King, Jr.,      Mark Shreve (CEO of the CPF), Imre Noth, M.D. (University of Chicago),
     M.D., the Constance B. Wofsy Distinguished Professor          and Skip Garcia, M.D. (University of Chicago)
     and vice chairman of the Department of Medicine at the
     University of California, San Francisco (UCSF), and chief
     of medical services at San Francisco General Hospital,
     was awarded the Marvin I. Schwarz Research Award,
     recognizing his commitment to improving the quality of
     life for those living with IPF through his compassionate
     patient care, education and support, while leading
     research efforts to find a cure for IPF. In recognition
     of his career’s work, Dr. King was awarded a $15,000
     gift to accelerate research efforts underway at UCSF in
     pulmonary fibrosis.

     Jack Hurley, an IPF patient from Chicago also gave a
     heart-felt presentation on his experiences living with IPF.
     Jack received a life-saving lung transplant in June 2005.

     Guests at the gala enjoyed an evening of fine dining,
     live and silent auctions, music and entertainment. The
                                                                   Recent lung transplant recipient Jack Hurley shares his experience
     signature item of the live auction was a private, guided      fighting IPF with B.I.G. Ball guests
     tour by Jay Leno of his world-class car collection, which
     auctioned for $26,000. Ski trip packages to Breckenridge,
     Colo., fine art, custom made jewelry, and private yacht
     cruises on Lake Michigan also highlighted the auction.
     Overall, more than 100 items were auctioned off, raising
     more than $90,000.

     The event provided a fun and elegant atmosphere for
     guests to mingle with their Chicago friends and share
     their commitment to furthering this important cause.
     At the same time, they celebrated the event’s huge
     success and the hope it gives to thousands of patients
     around the country suffering from IPF and the physicians
     who treat them. To learn more about the CPF’s future
     fundraising activities, please visit www.coalitionforpf.org
     or call (888) 222-8541.                                       B.I.G. Ball Committee members Elizabeth Masterson and Deborah Beggan
                                                                   with B.I.G. Ball co-chair Debbie Roney
 2
                                            “Right now, the only hope for survival for
                                           end-stage IPF patients is a lung transplant.
                                            The more people we can help to receive
                                        transplants, the better off we’re all going to be.”
                                                                      – Trey Schwab




Organ Donation Champion Honored                                                 “I was 37 years old when I was
                                                                                diagnosed,” says Schwab. “I had never
Trey Schwab receives Frank Cabral Humanitarian Award                            heard of IPF before, but when you’re
By Kathy Schultz, UW Health                                                     told you’re going downhill quickly and
                                                                                have maybe six months to live, you do
Culminating his two-year struggle      connections to the media to get          your research.”
with idiopathic pulmonary fibrosis     his message out. “I wanted to do
(IPF), Trey Schwab, who received       whatever I could to promote organ        “Right now, the only hope for survival
a double-lung transplant in 2004,      donation,” says Schwab.                  for end-stage IPF patients is a lung
was essentially dead for 40 minutes                                             transplant, it is the end of the line for
following complications from the       “Trey bravely fought a very public       them,” adds Schwab. “The more people
operation. Surviving by what his       fight with IPF and shared his story      we can help to receive transplants, the
surgeon called “a miracle,” Schwab     in such a heartfelt manner that          better off we’re all going to be.”
paused to take a look at his second    it inspired patients around the
chance at life, walking away from a    country,” says Mark Shreve, CEO          “I’m very honored to receive this
promising coaching career, leaving     of the Coalition for Pulmonary           award,” says Schwab. “It means a lot
his position as assistant basketball   Fibrosis. “He is an inspiration for so   to me that the Coalition thinks that
coach at Marquette to dedicate his     many patients; he exemplifies the        much of the work I’ve tried to do to
life to improving organ donation.      character and integrity for which the    promote organ donation. Until we can
Schwab, who now works at the           award is named.”                         discover a better treatment – drugs or
UW Health Organ Procurement                                                     some other way to help these people
Organization, was honored for his      After his transplant, Schwab             who are suffering from this awful
work at the Second Annual B.I.G.       returned to his team at Marquette        disease – many of them will need a
(Breathing Is Glorious!) Ball.         and served one more year as their        lung transplant to live.”
                                       assistant coach, but soon realized he
The award is named for Frank Cabral,   could help a lot more people if his
who also suffered from IPF and         work became official. Schwab joined
was successfully transplanted, and     the UW Health OPO in 2005 and
has dedicated his life’s work to the   works tirelessly traveling the state
disease.                               educating hospital staff, arranging
                                       public events and sharing his story
Originally, Schwab used his highly     to encourage people to take action
visible coaching position and          and share their intent to be a donor.


                                                                                                                         3
    The CPF is deeply grateful for the support of the
    following sponsors, contributors and auction item
    contributors that made the B.I.G. (Breathing Is
    Glorious!) Ball such an incredible success, raising
    more than $250,000!




4
Event Underwriters                    Mr. & Mrs. James Herring         Bernard & Darlene Wiczer                   Kate & Company/Kate Gleason
James & Sally Giancola                Marlene Huebner                  David & Sheri Wisner                       Kathleen & Lucy Keifer
Kristy Mau                            Fr. Tom Hurley                   Sarah Wood-Prince                          Kenosha Steak House
Rick Simon                            Jack & Eileen Hurley             Patricia Wray                              Killian Advertising
Erin & John Shipka                    Gia Interlandi                                                              The Krage Family
Peter & Marijane Thornton             Michael & Ruthie Jawgiel         Auction Item Contributors                  Mavis & Jay Leno
                                      Marilyn Johnson                  The Art Institute of Chicago/              Lettuce Entertain You
Contributors                          Holly Johnson-Carr                    Harriet Stratis                       Lone Star Sports
Julie & Sam Abushalback               Bob & Christy Kallay             The B Spot/Rashida B                       The LoSasso Family
Gina Anderson                         Amy & Joseph Kartheiser          Bahnoff Sport                              Michael Lucero
Frances Apostolides                   Hyun & Ed Kim                    George Balbach                             The Masterson Family
Malgorzata Bak                        Suzanne & Ken Kush               Beautiful Feet Chicago/Michael Chin,       Max’s Screen Machine
George & Vicki Balbach                Paul & Leslie Lehner                  M.D./Charlotte Covello, M.D.          Kristy Mau
Mr. & Mrs. Rudy Bartolomiu            Noah & Mel Levy                  Deborah & Tom Beggan                       Michael Anthony Salon & Day Spa
Wendy Baum                            Karen & Gerald Lorenz            Blackbird                                  Milwaukee Brewers
Charles Beck                          Suzanne & Scott LoSasso          Bovis Lend Lease/Alex Weiner               Musical Magic/Roseanne Locricchio
Shirley & Dr. Jerry Becker            John & Jane LoSasso              Bravo Restaurants, Inc.                    The North Face
Tom & Deborah Beggan                  Gina & John Marafino             Breckenridge Outfitters                    The Renaissance Chicago Hotel
Phillip Berger                        Arlene & John Matthews           Breckenridge Ski Resort                    The Roney Family
Felice Bernstein & Peter Tannenwald   Kristy Mau                       Bubbles Academy                            The Roney Apostolides Family
Belinda & Alec Bliss                  Daniel & Joan McCarthy           Lisa Centner                               Schaefer Smith
Jeffrey Blumenthal                    Larry McKay                      Helen Charizonko                           The Second City
Nancy Borders                         Keith Moore                      Chicago Cosmetic Surgery and               Erin & John Shipka
Mary Kay Bottorff                     Charles Mountford                     Dermatology/Carolyn Jacob             Shoreline Sightseeing/Chip Collopy
Kathleen & Thomas Brent               Dr. Andrew & Susan Muskovitz     Chicago Mercantile Exchange                Smart Destinations/Go Chicago Card
Andrew & Gail Brown                   Jessica & Frederick Nielson      Chicago Teacher, Inc./Belinda Bliss        Some Like It Raw
Susan Burden                          Victor & Terri Pantaleo          Clay Cottage Studio                        Southwest Airlines
John Cadarette                        Audrey Perrott                   Chantecaille Beaute                        Steppenwolf Theatre
Pepi Camerlingo & Daniel Arnett       Mary Jane Phares                 Chicago Bulls                              Stuart-Rodgers Photography
Jack Carriglio                        Jeff Pilgrim & Jill Ladendorf    The Chopping Block                         Tabula Tua
Thomas Carris                         Kristin & Paul Poister           A Creative Side/Jennifer Holleran          Deane Tank, Jr.
Teresa & Ray Carso                    Judith Price                     Alex Cue                                   Suzanne Toon
Helen & Ray Carucci                   Brogan Ptacin                    Roxanne Davis                              Toujours Deux Salon
Cynthia & Bob Chase                   Barbara Pulcini                  Daylight Donuts                            Traci Toutant
Sarah Clarkson & Andrew Pflaum        Heidi Randhava                   Phyllis DiPiero                            WGN-TV/Micah Materre
Kim & Jeff Cohen                      Dan & Georgia Roach              Rosemary & Carey Dowdle                    The Wang Family
Betty Cohout                          Matthew Robins & Claudia Rose    Downstairs at Eric’s                       Johanna Wright
Gwen & John Connery                   Jennifer Roche & John Svolos     eliana lily/Elizabeth Floersheimer         Teri Yarbrow
Candace Corr                          Deirdre Roney                    Fantasy Kingdom                            Yogaview
Andrew & Janet Cory                   Charles Roney                    Scott Floersheimer/The Park Grill          Zanie’s Comedy Club
Thomas Cousins                        John & Kathy Rudy                Nino Gabasvili                             Natalia Zauadouych
Karen Curtin                          Steve & April Rusing             Gabrielle Becker Designs
Tracy Dale                            Amy & William Ryan               Teresa & Walt Geiger
Carl Dalke                            Debra Schnebel                   Gene & Georgetti Italian Steak House
Leslie Davis                          David Schroeder                  Sally & James Giancola
John & Roxanne Davis                  Victoria & Scott Schutte         Gold Coast Plastic Surgery/George
Tom & Nancy DePoy                     Lois Scott                            Moynihan, M.D.
Robert Todd DeYoung                   Julie & Dan Semenak              Hackney’s
Edward DiPiero                        Barry Shkolnik                   Haute PR/Stacey Roney
Phyllis DiPiero                       Rick Simon                       Lydia and Daniel “Fitch” Henry
Eileen Dordeck & Chris Landgraff      Karla Snead                      The House of Blues
Brack Duker                           Laura Stamp                      Iarca Gallery
Roger & Betty Eger                    Maureen Stimming                 Interiors on the Move/Jennifer E. Harris
Amy & Thadford Felton                 Jenelle & Dan Sullivan           Jake
Jan & Michael Ferrari                 Susan Takacs                     JanSport
Tracy Flynn                           Robert Tenges                    Peggy Kasinski
Rick & Tami Frain                     Margaret Thornton
Harvey Friedman                       Kathy Tisdahl & Patrick Hughes




                                                                                         You!
Mignon Fulton                         Suzanne & Jeffrey Toon
JP & Khrista Gallagher                Charles Toon




                                                                                  h an k
Ian & Diane Gallaher                  Sarah Tornes
Susan Gemeund                         Janet Ulrey




                                                                                 T
Steven Gilford                        Trudy & John Vanderbeck
Patricia Gillerand, DDS               Jo Dee Walsh
Kelly & Bryan Goossen                 Monique & Jay Walters
Susan Harmon                          Daria & Ernie Wang
Jill Heise                            Patrick & Lauren Ward
Rick & Linda Hemwall                  Sophia Weiss

                                                                                                                                                  5
                                                                                              reflected on the opportunity of serving
  CPF Concludes Successful National IPF                                                       as a National IPF Awareness Week
  Awareness Week In Washington, D.C.                                                          delegate on Capitol Hill in Washington,
                                                                                              D.C. in September 2006, “I feel as if I
                                                                                              have just planted several small, young
  Contingent of IPF patients, family members and healthcare                                   trees on Capitol Hill, and over the next
  professionals on Capitol Hill to represent the IPF community                                many years they will grow into a nice
                                                                                              big canopy of protection, comfort and
                                                                                              love for the generations of IPF patients
                                                                                              and their families to come.”

                                                                                              The CPF group also met with leaders
                                                                                              of the National Institutes of Health
                                                                                              (NIH)/National Heart, Lung, and Blood
                                                                                              Institute (NHLBI) to learn about their
                                                                                              efforts in IPF-specific research and
                                                                                              new research efforts and clinical trials
      2006 IPF Awareness Week Advocates on Capitol Hill                                       underway to find treatments and a cure
                                                                                              for the disease. The NIH plays a critical
  The CPF recently announced results               perspective on legislation important       role in addressing health problems and
  from its fourth annual National IPF              to its 11,000 members across the           provides primary research funding for
  Awareness Week (Sept. 25 – Oct. 1).              country. Meetings with Members and         many diseases, including IPF. The CPF
  A delegation of CPF representatives              staff of the U.S. House Energy and         will be assisting the NIH in recruiting
  from across the country helped                   Commerce Subcommittee on Health            patients to participate in these trials,
  mark National IPF Awareness Week                 included Rep. Gene Green (TX), Rep.        which are slated to begin enrollment in
  by traveling to Washington, D.C. to              Mike Rogers (MI), Rep. Mary Bono (CA),     January 2007, according to the Agency.
  educate Members of Congress about                and Rep. Ralph M. Hall (TX). Meetings
  idiopathic pulmonary fibrosis (IPF)              with the U.S. Senate Health, Labor,        On Sept. 26 the U.S. House of
  and the need for improved research               and Pensions Committee included Sen.       Representatives passed a bill that would
  funding, and to encourage support for            Mike Enzi (WY), chairman, Sen. Richard     reauthorize the NIH for the first time
  important IPF-related legislation.               Burr (NC), Sen. Hillary Rodham Clinton     in 13 years and increase the program’s
                                                   (NY), and Sen. Orrin Hatch (UT).           total budget by five percent annually
  “This was our most productive                                                               through 2009. The IPF community is
  IPF Awareness Week to date with                  CPF representatives encouraged             further served by the NIH through this
  nearly 20 patients and patient                   lawmakers to support specific IPF-         legislation, as reports language offered
  advocates on Capitol Hill to meet                related legislation currently before       by the House Energy and Commerce
  with lawmakers and to deliver a                  Congress that can help improve             Committee included specific references
  singular message that more needs                 quality of life for patients, including;   to IPF. The reports language in Section
  to be done to treat and cure IPF,”               The Ending the Medicare Disability         5 of the bill states “…the Committee
  said Mark Shreve, chief executive                Waiting Period Act, which would            is interested in learning more about
  officer of the CPF. “There is not                waive the two-year waiting period          the research activities with respect
  a more effective way to educate                  for terminally ill patients to receive     to diseases such as…[idiopathic
  Members of Congress than by having               important benefits, and the Home           pulmonary fibrosis]…”
  them hear directly from their                    Oxygen Patient Protection Act, which
  constituents who are impacted by                 would protect patients’ interests with     This language serves as an important
  their decisions. We carried with us              respect to oxygen use and equipment        reference for implementing the NIH
  the voices of more than 128,000                  management. The meetings were              legislation, which now awaits Senate
  people across this country currently             effective in continuing to establish the   approval. The IPF-specific reference is
  fighting IPF.”                                   CPF and its members as the definitive      supported by the CPF’s advocacy efforts
                                                   advocacy organization and resource         and could bring greater attention to the
  The CPF met with nearly 50 staff                 for IPF information, and Congress          need to advance IPF-related research.
  and leadership of key congressional              welcomed the team and its message.
  committees responsible for                                                                  For additional information about
  authorizing the National Institutes              “I am full of energy for this cause,”      National IPF Awareness Week 2006
  of Health (NIH), and with oversight              said Bill Rhodes, CPF member and IPF       activities or the CPF’s Campaign ACT
6 of Medicare and Medicaid to provide              patient from Penngrove, Calif., as he      program, visit www.coalitionforpf.org.
                                                                                                            Research Update


Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease
to Explore New Approaches to Understanding and Treating Idiopathic
Pulmonary Fibrosis as Part of $12.8 Million NIH Grant
The Dorothy P. and Richard P.             disease and to design better drugs.      Genomics Center that aims to use
Simmons Center for Interstitial Lung      “The numbers tell us that only 50        the Human Genome information
Disease in the Division of Pulmonary      percent of patients survive three        to understand lung disease. “This
and Critical Care Medicine at the         years,” said Dr. Gibson, associate       project should allow us to identify
University of Pittsburgh School of        professor of medicine. “In reality,      what genes are active in a specific
Medicine announced that it has            some of them survive much longer,        patient during disease progression
received $6.4 million to explore          while others do very badly, we           and in the future to tailor a
new approaches to understand and          currently have no way to tell. This      therapy.”
treat idiopathic pulmonary fibrosis       study should allow us to predict how
(IPF). The research dollars are a part    the patient will do and to try and       Prabir Ray, PhD, associate professor
of a larger $12.8 million National        guide their options.”                    of medicine has shown that certain
Institutes of Health grant that                                                    growth factors can protect the
establishes Children’s Hospital of        One of the most devastating              lung against the development of
Pittsburgh and the University of          manifestations of IPF is the acute       fibrosis in mice. He will further
Pittsburgh School of Medicine as          exacerbation of the disease that         study the mechanisms of these
a Specialized Center for Clinically       leads to significant morbidity and       protective effects and will examine
Oriented Research in Pediatric and        mortality. The cause and course of       whether specific therapies could be
Adult Pulmonology. With this funding,     these exacerbations is not completely    developed. Dr. Kaminski said that
Simmons Center researchers will seek      understood. The Simmons Center           Dr. Ray’s experiments are critical
to improve treatment and diagnosis        study, which will monitor the            because they may help develop
of patients with IPF, a devastating       changes in the expression of genes in    approaches not only to halt fibrosis,
form of interstitial lung disease         the blood of patients over a period      but maybe even to reverse it.
affecting more than 128,000               of five years should allow a better
Americans, and characterized by           understanding of this phenomenon,        The Dorothy P. and Richard P.
progressive scarring of the lung tissue   according to Dr. Kaminski.               Simmons Center for Interstitial Lung
without any recognizable reason.                                                   Disease was established five years
                                          Steven R. Duncan, M.D., associate        ago through a generous donation
Investigators from the Simmons            professor of medicine and a co-          by the Simmons family. More than
Center for Interstitial Lung Disease      investigator has discovered that         1,000 patients with interstitial
and the Division of Pulmonary,            many patients with IPF have              lung disease are being followed
Allergy and Critical Care Medicine        exaggerated immune response. This        in the center; around 350 are
at the University of Pittsburgh           new observation may suggest that         patients with idiopathic pulmonary
School of Medicine will focus most        not all patients will respond to         fibrosis. In addition to providing
of their attention on designing           the same therapy and may suggest         patients access to multiple research
better methods for diagnosis and          that some patients will respond          protocols, the center provides
treatment of IPF. Naftali Kaminski,       to specific drugs that modify the        personal care by physicians who
M.D., associate professor of medicine,    immune system.                           are experts in interstitial lung
pathology and human genetics, and                                                  diseases, information and education
director of the Simmons Center,           “It is now accepted that you can get     programs and an active support
and Kevin Gibson, M.D., will follow       lung fibrosis by many mechanisms,        group (for more information about
patients to identify changes in           but we still try to treat all patients   Simmons Center please visit http://
their genes that predict disease          the same way, and often times, we        simmonscenterild.upmc.com/).
progression. These changes may            fail,” said Dr. Kaminski, who is also    Source: UPMC Press Release 10/2/2006:
help to understand what causes the        the director of the Lung Translational   Content edited for space
                                                                                                                           7
    Johns Hopkins Hosts IPF Patient Education Day in Baltimore
     Johns Hopkins University Division of Pulmonary & Critical Care Medicine held an IPF patient education day on Oct. 14,
     2006. The event was co-sponsored by the CPF, and covered a wide variety of issues that concern IPF patients including
     diet and nutrition (Dechen Surkhang, RD), social services and support available to patients with IPF (Chih Garbus,
     LCSW) and common sleep disorders (Loretta Colvin, RN, CRNP). Three physicians involved with IPF patient care dis-
     cussed the nature of IPF (Sonye Danoff, M.D.), the ‘alphabet soup’ of tests that patients undergo (Albert Polito, M.D.)
     and the tricks to traveling with oxygen (Maureen Horton, M.D.). More than 50 patients and family members attended.
    “We’re pleased to support Dr. Danoff and her team at Hopkins by sponsoring this event,” said Mark Shreve, CEO of the
     CPF. “I know the patients are grateful to the event speakers for volunteering their time to improve education and
     awareness of IPF in the Baltimore area.”


     “Many thanks to the CPF for helping to make this
     important IPF patient education and support day
        a meaningful experience for all involved.”
                – Sonye Danoff, M.D., Johns Hopkins University

                                                                                     Johns Hopkins team on IPF Patient Education Day



    CPF Research Manuscript Accepted for Publication in Respiratory Medicine
    The CPF is pleased to announce            Colorado Health Sciences Center),        suggest there is a substantial need for
    that a summary manuscript from            Gregory Tino, M.D. (University of        improved patient education regarding
    its Basic Research Questionnaire,         Pennsylvania Medical Center), Paul       the diagnosis and management of
    considered one of the largest             Noble, M.D. (Duke University Medical     pulmonary fibrosis.
    databases of IPF information              Center), Mark A. Shreve (CPF), and
    in the country, was accepted              Maureen Michaels and Bruce Carlson       Overall more than 2,000 patients and
    for publication in Respiratory            (Michaels Opinion Research, Inc.).       families have completed the
    Medicine, an internationally-                                                      Basic Research Questionnaire. It is
    renowned, peer-reviewed journal           The manuscript focuses on the            managed by Michaels Opinion Research,
    devoted to the rapid publication          issues of patient education and          Inc., an independent research firm with
    of clinically-relevant respiratory        resources. A survey of 52 defined-       expertise in healthcare issues. Funding
    medicine research. This manuscript        choice and open-ended questions          for this program is provided through
    represents the first large-scale          regarding the diagnosis and              grants from the DuBrul Family Fund
    survey of patient experiences and         management of pulmonary fibrosis         and from Helen and Michael Galvin in
    perceptions regarding the diagnosis       was delivered. Two-thirds of the         memory of their family members who
    and treatment of pulmonary fibrosis       total 1,448 respondents reported         have passed away from IPF.
    published to date.                        a clear lack of information and
                                              resources on pulmonary fibrosis at       The manuscript will be available on
    The paper, titled “Patient                the time of diagnosis. Less than         the CPF Web page upon publication in
    Experiences with Pulmonary                half of respondents reported they        Respiratory Medicine, expected at the
    Fibrosis,” was authored by Harold         felt well-informed about treatment       end of 2006. To learn more, please visit
    Collard, M.D. (University of              options, the role of supplemental        www.coalitionforpf.org.
    California, San Francisco), Marvin        oxygen, pulmonary rehabilitation
    Schwarz, M.D. (University of              and transplantation. These results
8
InterMune Presents Preclinical Data Showing Additive Anti-Fibrotic
Combination Effects of Actimmune® and Pirfenidone
InterMune recently presented              potential anti-fibrotic mechanisms       gene induction to varying degrees,
preclinical study results involving its   of our two Phase III compounds,”         while combination treatment
two Phase III drug candidates for         stated Lawrence M. Blatt, Ph.D.,         resulted in additive suppression
idiopathic pulmonary fibrosis (IPF),      chief scientific officer of InterMune.   of all TGF-beta-induced genes.
Actimmune® (interferon gamma-1b)          “The data provides insight into the      The results of the present studies
and pirfenidone, at CHEST 2006, the       anti-fibrotic activity of Actimmune®     also confirmed that pirfenidone
annual conference of the American         and pirfenidone and suggests that        blocks TGF-beta-induced activation
College of Chest Physicians in Salt       co-administration of these two           of p38-gramma MAP kinase and
Lake City, Utah.                          compounds may lead to benefits in        subsequent collagen synthesis. Taken
                                          patients with IPF.”                      in total, these studies suggest that
The in vitro studies of Actimmune®                                                 the co-administration of Actimmune®
and pirfenidone show each inhibit         In vitro studies conducted by            and pirfenidone does not appear
fibrotic pathways and, when used          InterMune researchers involved           to inhibit the individual activity
in combination, show an additive          human fibroblast cells pretreated        of each compound and that the
suppressive effect, indicating            with pirfenidone and Actimmune®          two compounds may work through
the two compounds may offer               that were then stimulated with           complementary pathways.
complementary approaches to the           transforming growth factor beta
treatment of IPF.                         (TGF-beta). TGF-beta has been            Both Actimmune® and pirfenidone
                                          shown to be an important mediator        are currently in Phase III clinical
“IPF is a disease characterized by        of fibroblast proliferation and          trials as a potential treatment for
fibrosis or scarring of the lungs,        collagen production by downstream        IPF. To learn more about either
and our in vitro studies further          gene activation. Actimmune® and          clinical trial, visit the Research
expand our understanding of the           pirfenidone inhibit TGF-beta-induced     section of the CPF’s Web site.


Hometown Friend Fights Against Evil Disease for Evel Friend
Evel Knievel battles IPF as his family, friends raise money and awareness
When Bill Rundle first heard his friend   Rundle founded an event several years ago to recognize Evel for his
Evel Knievel suffered from idiopathic     contributions to the stunt and entertainment world called “Evel Knievel
pulmonary fibrosis (IPF), he thought      Days,” which has been an annual event for years. This year, Rundle and the
there must be a treatment and maybe       Knievel family contributed a portion of the event’s proceeds to fund IPF
even a cure for the disease. “I was       research.
amazed that there is no cure,” said
Rundle, Knievel’s longtime family         The event attracted 10,000 people its first year and hosted more than 80,000
friend and fellow Butte, Montana          in 2006, with people coming from 43 states in the U.S. and representing
native. “You’d think there’d be some      13 countries. It featured daredevils from all parts of the world, many who
kind of treatment for it. I’ve been       idolized Knievel as children. Evel’s son, Robbie Knievel, continues in the
around Evel my whole life. He is like     family tradition performing motor cycle stunts and showcasing his talent for
a father to me.”                          the death defying as well.

Rundle said he realized IPF was taking    The irony, Rundle says, is that Evel has survived incredible feats and stunts,
a toll on his friend as he watched        yet he is helpless against IPF. “[I see] the effect that the disease has had
him struggle to breathe and use           on Evel,” said Rundle, as he recalled an event where he could see the toll IPF
supplemental oxygen. “He was used         was taking on his friend. “I introduced him in Daytona at his last personal
to Florida and here in Montana at         appearance. We had to help him off stage and I choked back tears. This
5,400 feet, he was having a tough         disease is taking him away. As many things as he has been through – he is
time.”                                    obviously a tough man – but here is a disease [he must fight] that no one can
                                          help him.”                                                                     9
                                          American Thoracic Society Appoints CPF’s
                                          Teresa Geiger to Key Advocacy Committee
                                          The CPF is pleased to announce         The ATS Public Advisory Roundtable
                                          that Teresa Geiger, the CPF’s vice     serves as a bridge between
                                          president for Patient Outreach &       the American Thoracic Society
                                          Advocacy, has been appointed to        and organizations representing
                                          the Public Advisory Roundtable         individuals affected by lung
                                          of the American Thoracic Society       disease and breathing disorders
                                          (ATS), the world’s leading             by stimulating collaboration in
                                          professional organization for          research, education, patient care and
                                          pulmonary, critical care and sleep     advocacy. The committees’ current
                                                                                 members include organizations such
         “We’re proud of her              physicians.
                                                                                 as the Pulmonary Hypertension
         and her willingness              “Teresa brings the unique              Association, the Alpha-1 Foundation,
                                          perspective to this committee of       the ARDS Foundation and the
        to participate on this            being able to offer her insights not   National Sleep Foundation.
       important committee                only through her work with the CPF,
                                          but also because of her personal       This represents the second
           and to help direct             experience of losing five family       partnership effort between the ATS
                                                                                 and the CPF. In early 2006, the two
           the advocacy and               members to idiopathic pulmonary
                                                                                 organizations also announced the
                                          fibrosis,” said CPF CEO Mark Shreve.
           patient education              “We’re proud of her and her            establishment of a jointly-funded
                                          willingness to participate on this     research award in pulmonary fibrosis
         efforts of the ATS in            important committee to help direct     in the amount of $100,000. The
          the coming years.”              the advocacy and patient education     award will be given to a U.S.-based
                                          efforts of the ATS in the coming       investigator working on translational
     – Mark Shreve, CEO of the CPF        years.”                                studies in pulmonary fibrosis. The
                                                                                 recipient is expected to be named at
                                                                                 the end of the year.




     About the American Thoracic Society (ATS)

     The ATS is a non-profit, international, professional and scientific
     society for respiratory and critical care medicine. The ATS is
     committed 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. To learn more, visit
     www.thoracic.org or call (212) 315-8600.

10
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                                                                                                                            Please use these cards to refer




o I’d like to become a CPF member                                                                                              o I’d like to become a CPF member
o I’m already a member of the CPF and would                                                                                    o I’m already a member of the CPF and would
  like to update my contact information                                                                                          like to update my contact information
Name:                                                                                                                          Name:
Address:                                                                                                                       Address:


City:                State:     Zip Code:                                                                                      City:                State:        Zip Code:
Phone Number:                                                                                                                  Phone Number:
Email:                                                                                                                         Email:
Age: o Under 21    o 21-35    o 36-50   o 51-65   o 66+                                                                        Age: o Under 21    o 21-35    o 36-50     o 51-65   o 66+
I am a: o IPF Patient                                                                                                          I am a: o IPF Patient
        o IPF Family Member                                                                                                            o IPF Family Member
        o Healthcare Professional                                                                                                      o Healthcare Professional




o I’d like to become a CPF member                                                                                             o I’d like to become a CPF member

o I’m already a member of the CPF and would                                                                                   o I’m already a member of the CPF and would
  like to update my contact information                                                                                         like to update my contact information
Name:                                                                                                                         Name:
Address:                                                                                                                      Address:


City:                State:     Zip Code:                                                                                     City:                State:      Zip Code:
                                                                T he CPF is look ing for new mem ber s!




Phone Number:                                                                                                                 Phone Number:
Email:                                                                                                                        Email:
Age: o Under 21    o 21-35    o 36-50   o 51-65   o 66+                                                                       Age: o Under 21     o 21-35    o 36-50    o 51-65    o 66+
I am a: o IPF Patient                                                                                                         I am a: o IPF Patient
        o IPF Family Member                                                                                                           o IPF Family Member
        o Healthcare Professional                                                                                                     o Healthcare Professional
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Coalition for Pulmonary Fibrosis                                                            Coalition for Pulmonary Fibrosis
Suite F, #227                                                                               Suite F, #227
1659 Branham Lane                                                                           1659 Branham Lane
San Jose, CA 95118-5226                                                                     San Jose, CA 95118-5226
                                    $ 0.24                                                                                       $ 0.24
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                                     Needed                                                                                      Needed
Coalition for Pulmonary Fibrosis                                                            Coalition for Pulmonary Fibrosis
Suite F, #227                                                                               Suite F, #227
1659 Branham Lane                                                                           1659 Branham Lane
San Jose, CA 95118-5226                                                                     San Jose, CA 95118-5226
CPF, IPF Expert Share Story With WCBS Radio
In New York
Radio host, Al Meredith, asked “What is IPF?”

Dr. Maria Padilla, associate professor of medicine and director of the
advanced lung disease program at Mount Sinai Medical Center, leaned closer
into the microphone and explained that IPF is a debilitating lung disease           WCBS Production
that has no FDA-approved treatments and no cure. Her voice growing more             Manager Raises Awareness
passionate with each word, Dr. Padilla understands the disease all too well.        of the Disease That
She has spent her entire medical career trying to stop it.
                                                                                    Claimed Her Father’s Life
Padilla, and CPF Vice President Teresa Geiger were interviewed by WCBS-FM
and JACK-FM radio stations in New York City on Oct. 5.                              When Maria Martello called
                                                                                    the CPF to ask for help with
“There is a revolution in IPF,” Dr. Padilla said. “We are learning a little about   her father, who was a patient
 its course and about the complications, but we still don’t know the natural        in the intensive care unit of a
 history. We are looking at the pathogenesis to try to understand the disease
                                                                                    New York hospital, she quickly
 and to apply novel approaches.”
                                                                                    learned that her family was
Dr. Padilla told WCBS much is happening in the IPF community. There are             not alone. They share much
many exciting clinical trials in process and a viable, potentially life-saving      with other families in their
treatment could be on the horizon.                                                  fight against IPF including the
                                                                                    shock of dealing with a disease
Al Meredith first heard about the CPF and idiopathic pulmonary fibrosis (IPF)       that is more devastating than
through production manager Maria Martello, whose father was suffering
                                                                                    its name “pulmonary fibrosis”
from IPF and later died. He said it was her compelling and emotional first-
hand account of the issues her father and her family faced with the disease,        implies.
including the hard-hitting realities of IPF, that inspired him to help raise
awareness of IPF through his program.                                               Though her father has since
                                                                                    passed away, Maria has risen
                                                                                    above her families’ grief to help
                                                                                    other patients with IPF and
                                                                                    to help spread the word about
                                                                                    the disease that claimed her
                                                                                    father’s life.

                                                                                    “The story of IPF needs to be
                                                                                    told,” said Martello. “We have
                                                                                    to start now to educate people
                                                                                    about the deadly disease and to
                                                                                    let them know that the CPF is
                                                                                    out there to help families and
                                                                                    patients who are dealing with
                                                                                    it. I am so grateful I was able
                                                                                    to do something to help raise
                                                                                    awareness of IPF. It has become
                                                                                    part of the healing process for
                                                                                    me to know that I might be
Al Meredith of WCBS interviewing Dr. Maria Padilla
                                                                                    able to help others.”           11
     Lung Patients See a New Era of Transplants                                        The number of lung transplants has
     By Denise Grady, The New York Times                                               risen to 1,405 in 2005, 248 more
     Sept. 24, 2006                                                                    than the year before. Fewer people
                                                                                       are dying on the waiting list: 360 in
     A quiet revolution in the world of        Recent changes have revitalized         2005, down from 488 in 2004.
     lung transplants is saving the lives      lung transplantation. Starting in       Lungs have always been “the bad
     of people who, just two years ago,        May 2005, new rules nationwide put      stepchild” of organ transplants
     would have died on the waiting list.      patients who needed transplants         - harder to get, harder to transplant,
     In the past 16 months, waits have         most at the top of the list - people    more prone to rejection and
     shortened, lists have shrunk, and         who would soon die without a            complications than other organs,
     the number of lung transplants has        transplant, but who had a good          said Dr. Scott Palmer, the medical
     gone up. Further improvements are         chance of surviving after one.          director of Duke University’s lung
     expected this year.                       Previously, lungs went to whoever       transplant program. Lung transplants
                                               had been waiting longest, even if       were not consistently successful
     The changes have all but erased the       another patient needed them more.       until the mid-1980’s, lagging far
     need for transplants from live donors     The waiting time was often two          behind those of kidneys, livers and
     - desperate, last-ditch operations        years or more, so there was little      hearts. From the start, lungs have
     requiring two donors per patient,         hope for people with lung diseases      been offered first to whoever had
     usually relatives and friends who risk    that came on suddenly or progressed     spent the most time on the waiting
     major surgery in hopes of rescuing a      rapidly.                                list, in the donor’s geographic
     loved one whose time is running out.                                              region.
     “It’s almost as if it’s a whole new       Another major change is that more
     day for lung transplantation,” said       lungs from cadavers have become         Changes in the system came about
     Dr. Cynthia Herrington, a surgeon at      available, for two reasons: more        partly because of a 1998 federal
     the University of Minnesota Medical       people are becoming organ donors,       regulation requiring that all organ
     Center, Fairview, in Minneapolis. “It’s   and doctors have figured out ways to    transplants go to patients with the
     amazing.”                                 salvage lungs that previously would     greatest medical need. The intention
                                               have been considered unusable. The      was to even out waiting times
     Nationwide, it is too soon to tell        new methods use drugs, respirator       around the country and decrease
     what the impact of the transplant         settings and other techniques to        deaths on the waiting list. Changes
     changes will be.                          prevent damage to the lungs and         have been gradual.
                                               keep their tiny air sacs open in
     “Are we actually improving overall        brain-dead patients.
     survival?” asked Dr. Selim Arcasoy,                                                   Recent changes
     the medical program director for          In the past, lungs could be retrieved
     lung transplantation at New York-         from only about 15 percent of organ      have revitalized lung
     Presbyterian Hospital/Columbia            donors, but at some centers the
     University. “Or are we transplanting      rates have risen to 40 percent. Dr.
                                                                                           transplantation.
     sicker people who don’t last as           Herrington said that in Minnesota,       Starting in May 2005,
     long?”                                    the number of lungs retrieved went
                                               to 97 from 25 in a single year.          new rules nationwide
     Transplants are given to people           “Good organs 5 or 10 years ago
                                                                                          put patients who
     whose lungs fail because of               were probably being buried because
     emphysema, cystic fibrosis or other,      doctors did not know how to               needed transplants
     less common diseases. Since demand        save them,” said Dr. Kenneth R.
     exceeds supply, patients must join        McCurry, director of heart and lung        most at the top of
     regional waiting lists that are part of   transplantation at the University of            the list.
     a national network.                       Pittsburgh.

12
For lungs, figuring out how to          recipients quickly, which was a huge     said, many people thought the old
measure medical need and rank           improvement, because once an organ       system gave an unfair advantage to
patients with different diseases        donor is brain dead, organs start to     emphysema patients.
took time. “Our concern was that        deteriorate. The lungs are especially
if we used just severity of illness,    fragile.                                 On the waiting list, 5 to 10 percent
we might waste a lot of lungs on                                                 with emphysema died each year,
patients who were so sick they were     In the past, transplant coordinators     compared with 30 to 40 percent
unlikely to survive anyway,” said       might have spent hours calling           among those with cystic fibrosis or
Dr. Thomas Egan, a cardiothoracic       hospitals, only to hear again and        pulmonary fibrosis. “It was an ethical
surgeon at the University of North      again that the patient at the top of     dilemma,” Dr. Patterson said, adding
Carolina, Chapel Hill, who led a        the lung list was not sick enough        that some doctors were troubled
UNOS panel that spent several           for a transplant. Meanwhile the          to see so many transplants go to
years developing new rules for          clock would be ticking; patients         people with emphysema, which is
lung allocation. The panel studied      would have been found who needed         caused by smoking, whereas “others
medical records to figure out which     the heart, kidneys and liver; and        have disease they didn’t produce.”
patients were most or least likely      surgeons would be standing by,           Now, at most centers, more patients
to survive after a transplant, and      ready to remove them. Doctors say        with pulmonary fibrosis are getting
worked that into the scoring system.    some lungs were probably wasted          transplants.
As a result, lungs are now the only     because recipients simply could not
organs with transplant rules that       be found fast enough. “Placement is      Dr. Jonathan B. Orens, medical
consider the recipient’s survival       easier now,” Dr. Egan said. “It takes    director of the lung transplant
odds.                                   four or five calls. It used to be 16.”   program at Johns Hopkins, said that
                                        The new system has also changed          in the past year, more than half the
Almost immediately, the new system      the types of patients who receive        28 recipients there were people who,
cut the waiting list in half. Because   the most transplants. Before, a          under the old system, would have died
waiting time no longer mattered,        majority had emphysema, a lung           on the waiting list. Dr. Orens said he
people who had been listed early in     disease nearly always brought on by      and his colleagues had just performed
their illness just to hold a place in   smoking. They received transplants       a preliminary analysis of the
line dropped in rank or were deleted    because the disease moves slowly         nationwide data on the first patients
(unless they needed a transplant        and they could wait, outlasting          treated under the new system, and
right away) but could rejoin the list   patients - often younger ones - with     found that so far, one-year survival
later if they became sicker.            other lung diseases.                     rates appeared to have dropped, to
Overnight, some patients who had                                                 about 70 percent, from about 80
waited for years to reach the top of    “People with pulmonary fibrosis          percent over all.
the list suddenly found themselves      or pulmonary hypertension can be         Slightly lower survival rates under the
at the bottom, or even crossed          diagnosed and go downhill very,          new system, he said, “may be the best
off. Nobody was grandfathered in.       very rapidly,” said Dr. G. Alexander     we can do with lung transplants when
“We tried our best to educate and       Patterson, a surgeon at Washington       patients are this sick.”
communicate, but many felt they         University in St. Louis, which has
had been cheated,” Dr. McCurry          one of the country’s largest lung        The rates may still represent a
said. But at his center in Pittsburgh   transplant programs, with about 55       net benefit, he said, “compared to
there were no deaths among those        to 60 adult patients and 25 to 30        shortening the lives of patients who
who lost their places in line, he       children a year. Pulmonary fibrosis      did not quite need the transplant.”
said, adding that many still received   causes extensive lung scarring,          As more data comes in, the rules may
transplants.                            and its cause is often unknown.          need to be adjusted, Dr. Orens said.
                                        Patients can die within a year of        “We’re trying to capture just the right
Those who remained on the list          the diagnosis. But patients with         patients at just the right time.”
needed transplants soon. As a           emphysema can often live for a
result, it became much easier to find   long time. As a result, Dr. Patterson    Source: The New York Times
                                                                                                                          13
                                             Transplant Recipient Honored
                                             By John Carney, Shelbyville Times-Gazette (Shelbyville, Tenn.)
                                             Story edited for space by CPF staff

 American Lung Association
 Launches National Influenza
 Education Initiative

 Influenza immunization rates fall far
 short every year, even though health
 experts recommend more than 200
 million people in the U.S. receive an
 annual influenza vaccination. The
 American Lung Association recently
 launched Faces of Influenza, a
                                             Ralph Nollenberger at Webb School where   Headmaster Albert Cauz, Teresa Geiger of the
 multi-year national public awareness                                                  Coalition for Pulmonary Fibrosis, Ralph Nol-
                                             he taught mathematics.
 initiative to help Americans recognize                                                lenberger, and assistant headmaster Stan Rupley.
 the importance of an annual influenza                                                 (T-G Photo by John I. Carney)
 immunization as a preventative
 measure to protect themselves and           The Webb School in Bell Buckle, Tenn., a private prep school, made a
 their families every year.                  contribution to the Coalition for Pulmonary Fibrosis in honor of mathematics
                                             instructor Ralph Nollenberger, who suffered from idiopathic pulmonary fibrosis
 As part of the campaign, the Lung           or IPF. He received a transplant in February 2005 at Vanderbilt University
 Association is offering its Flu Clinic      Medical Center.
 Locator as a public service. By visiting
 www.facesofinfluenza.org and typing         “We are so happy that we can honor Ralph, while at the same time help
 in their 5-digit zip code at the Web        provide much-needed funds that can fuel research that may someday lead to
 page, site visitors can receive a list of   life-saving treatments or a cure,” said Webb headmaster Albert Cauz in a news
 immunization clinics in their area. Site    release.
 visitors may also schedule reminders
 and sign up to receive seasonal             “It is the research and advances in medicine that saved my life,” said
 influenza news. The Flu Clinic Locator      Nollenberger. “It is my goal now to give back to the people who made the
 will remain active as long as public        difference for me. This modest gift can make a difference for so many others
 flu immunization clinics are offered.       who suffer from IPF.”
 Consumers and health care providers
 can also visit the site to find more        IPF is a mysterious disease which causes scarring of lung tissue. Its cause is
 information about influenza and the         unknown, and it eventually becomes so severe that the lungs are prevented
 importance of immunization.                 from functioning. There is nothing that can be done short of a transplant to
                                             remove the failing lungs.
 The initiative also includes educational
 materials as well as the national           Nollenberger was first diagnosed with IPF in 2001, when he had to get a
 distribution of new television and          physical exam in order to be licensed to drive a school bus. He’d had spots on
 radio public service announcements          chest X-rays prior to that, but they had been attributed to smoking.
 directed at target groups recommended       Today, Nollenberger hikes, canoes and uses a push lawnmower. Because his
 for influenza immunization.                 immune system is suppressed by drugs which prevent rejection of the new
                                             lungs, he must take certain precautions, but he is living an otherwise-active
 Source: American Lung Association           life that he would have thought impossible a few years ago.

14
                                                                                                   Campaign ACT Update


IPF Patient Talks to Members of Congress,                                            can help patients like me to overcome
Asks for Waiver of Mandatory Medicare                                                at least the financial burden of this
                                                                                     disease.”
24-Month Waiting Period
                                                                                     Darcy changed her major from nursing
Elizabeth Darcy shares her story with Members of Congress                            and continues to attend classes as
                                                                                     long as she’s physically able to be
Elizabeth Darcy lives in an RV in her     thereby granting patients like Darcy       there. It is her status as a student
28-year-old daughter’s drive way. She     the ability to afford and receive the      that is keeping her afloat right now,
suffers from idiopathic pulmonary         vital care that they need.                 she says, both emotionally and
fibrosis (IPF) and is a candidate for a                                              financially. Until she receives any
lung transplant, though her wait for      In spite of her own issues, Darcy          benefits, she is living off of student
a transplant may not be as long as        still recognized the need to travel        loans.
her wait for much needed disability       with other patients and advocates
benefits.                                 recently to Washington, D.C. for IPF       The Ending the Medicare Disability
                                          Awareness Week to try and help. She        Waiting Period Act of 2005 would
Darcy, like many other patients           joined the CPF and other patient           grant more immediate Medicare access
who suffer from IPF, is desperate,        advocates to share her story with          to the terminally ill and disabled who
financially strapped and dying. At        Members of the United States House         currently have to wait as long as two
the same time, remarkably, the            and Senate.                                years before collecting these benefits.
58-year-old is working with the CPF                                                  For someone living with IPF, which
to make a difference for others. Her      Darcy, of Charlotte, N.C., was             has a median survival rate of three
health status and her age have made       diagnosed with IPF in 2005 and was         years, the waiting period
her a “poster child” for The Ending       forced to quit her job as a nursing        is too long and for
the Medicare Disability Waiting           assistant and her work as a nursing        those without
Period Act of 2005. Currently,            student.                                   resources, simply
though deemed permanently                                                            cruel. This
disabled and thus eligible for            Darcy brought the message home to          legislation will be
Medicare coverage, she must wait 24       each of the Members and staffers           re-introduced
months for her Medicare coverage to       she met with on Capitol Hill, some         early next
become effective, per the existing        from her own state, including Rep.         session. Please
Medicare coverage guidelines. The         Virginia Foxx. “I think that walking       contact your
Waiting Period Act was introduced         into the offices with my oxygen            Members of
so patients like Darcy, who may           tank gave the Members a face and           Congress to
not live long enough to receive           name to place with the request             voice your
their Medicare benefits, would be         for congressional support of much          support of
exempt from current guidelines            needed legislation,” said Darcy. “This     the Act.
and provided immediate coverage,          is not just a bill, it is something that



  The Ending the Medicare Disability Waiting Period Act of 2005 would grant more
  immediate Medicare access to the terminally ill and disabled who currently have to wait as
  long as two years before collecting these benefits. For someone living with IPF, which has
  a median survival rate of three years, the waiting period is too long and for those without
  other resources, simply unacceptable. This legislation will be re-introduced early in the 2007
  Congress. Please contact your Members of Congress to voice your support of the Act.
                                                                                                                           15
     Inspector General Says Medicare Still Pays Too Much for
     Home Oxygen Equipment Despite New Policy
     By John Reichard, CQ HealthBeat Editor

     A new policy under the budget            Limiting Medicare coverage of rental payments to 13 months, a step urged in
     savings law enacted in February          the Bush administration’s fiscal 2007 budget proposal released last February,
     ending a rental-only policy in the       would save Medicare and its beneficiaries about $3.2 billion over five years,
     Medicare benefit for home oxygen         according to the Inspector General’s office.
     equipment should be revised further,
     according to a new report by the         The study also found that concentrators and portable oxygen equipment that
     HHS Office of the Inspector General.     patients receive under the benefit require minimal servicing and maintenance.

     The law (PL 109-171) limits Medicare     In addition to reducing the rental coverage period, the report recommends
     coverage of rental payments for          that Medicare should work with Congress to determine the necessity for and
     home oxygen equipment to 36              frequency of non-routine maintenance. Suppliers perform routine maintenance
     months of continuous use, after          without payment from Medicare.
     which suppliers must transfer title
     of the equipment to the Medicare         Medicare also should determine if a new payment methodology is appropriate
     beneficiary. But even with that          for portable oxygen systems, the report added. Payment for the contents of
     change, Medicare will pay far more       the portable cylinders is now bundled into the Medicare payment covering
     over the 36 months for rentals           rental costs. Once Medicare stops covering rental payments after 36 months,
     than the purchase price of oxygen        it will pay only for the contents of the portable cylinders, but the current
     concentrators, stationary devices        payment rate for contents may not be adequate, the report said.
     that are a key component of
     home oxygen equipment and that           Source: CQ HealthBeat News [article edited by CPF staff]
     concentrate the oxygen in room air
     before it is inhaled by the patient.
     Patients also receive portable
     oxygen equipment under the
     Medicare benefit.

     The report said Medicare will
     allow payment of $7,215 for rental
     payments while concentrators cost
     just $587 on average to buy. The
     report added that beneficiaries also
     incur $1,443 in co-insurance costs
     over the three-year rental period.

     Nearly all Medicare beneficiaries
     who rent home oxygen equipment
     rent concentrators, the report
     said. In 2004, concentrator rentals
     accounted for 84 percent, or $2.3
     billion of all Medicare spending
     for home oxygen equipment and
     supplies, the report added.

16
  L
                                  Transplanted IPF Patient Plans for His Future, the
                                  Future of IPF Research
                                  With each breath, Charlie Cox remembers how difficult it was to breathe
                                  with his IPF-damaged lungs. Having survived the fear, pain and coming to
                                  terms with an early death, Charlie Cox now celebrates his new lease on life.
                                  Each day he remembers the fear of facing death from a terminal illness,
                                  and pledges to help the Coalition for Pulmonary Fibrosis (CPF) find a cure.

  Legacy Leaders                  Cox received a lung transplant two years ago, just days after being placed
 are special supporters who       on the waiting list at the University of California, Los Angeles (UCLA) and
                                  after eight months of testing and years of fighting the disease with little
  remember the Coalition          hope of extending his life.
  for Pulmonary Fibrosis in
                                  His journey was helped by the resources offered by the CPF. “I couldn’t
 the creation of their wills or   believe there was finally a resource that provided information about various
                                  drug studies and which experts were doing them, as well as a resource that
 through other estate gifts,      offered support and research information to patients like me,” said Cox.
 thereby securing the future
                                  A PERSONAL COMMITMENT
of our mission of assisting all   Although Cox is living comfortably with his new lung, he and his wife feel
                                  very emotionally linked to the IPF community. They continue to join the
those affected by idiopathic      monthly support group that he looked to for support so that he can assist
   pulmonary fibrosis (IPF)       others in coming to terms with their disease. His sense of the need is
                                  summed up in these words, “people are really hurting out there.”
through education, support,
                                  “When you go to the CPF, and to support groups, you know you aren’t in
   advocacy, and research         this by yourself,” he said. “You know where you can go for help. There
funding. To learn more, visit     are people to help you - people trying to come up with solutions.” Cox
                                  encourages other patients and family members to seek help from a support
     the CPF website at           group.

  www.coalitionforpf.org or       More importantly, Cox and his wife have made a commitment to the CPF’s
 contact Mishka Michon at         Legacy Leader program. As a Legacy Leader, Cox feels he is doing what he
                                  can to change the future for other patients. This simple act, for Cox, is a
(888) 222-8541, extension 701.    way of taking action that will make a difference.

                                  Although the first nine months after the transplant were difficult, Cox says
                                  his life is nearly back to normal. He now plays golf with his sons, a sport
                                  he had given up due to his illness. That simple pleasure is something for
                                  which he is very grateful.




                                                                                                                 17
     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.

     Sarasota, FL
     Sarasota Pulmonary Fibrosis Support Group
     In partnership with Lung Associates of Sarasota

     Time:     Every other month on the second Monday from 3:00-4:30 p.m. beginning Nov. 13, 2006
     Location: HealthSouth RidgeLake Hospital, 6150 Edgelake Drive, Sarasota
     Contact: For more information or to register, please contact Karen Erceg at (941) 366-5864 ext. 830
                  or by email at kerceg@lung-associates.com

     Wichita, KS
     In Partnership with the Wichita Clinic

     Time:     Quarterly
     Location: Health Strategies, 551 N. Hillside, Wichita, Kansas
     Contact: Susan Picotte at (316) 689-9521 or by email at PicottSG@wichitaclinic.com

     Charleston, SC
     In partnership with the Medical University of South Carolina

     Time:     Next meeting is “IPF Patient Education Day”, Friday, March 23, 2007
     Location: MUSC campus (venue to be announced)
     Contact: For more information, please contact Ruth Oser at (843) 792-3168 or by e-mail at oserrk@musc.edu




     We need your help!

     Encourage your friends, family – even your doctor and respiratory
     therapist – to join the CPF – it’s free!

     Contribute to the CPF’s efforts through a tax-deductible gift.
     Raise awareness in your community about IPF - ask us how by emailing
     Teresa Geiger at tgeiger@coalitionforpf.org or call (888) 222-8541.
     Access our website at www.coalitionforpf.org for patient information and
     resources to learn about how you can be an advocate for all IPF patients
     and contribute to this important work.
18
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, Division Chief, Pulmonary,
                                                                                                         Profile: CPF Scientific
Allergy and Critical Care Medicine
Duke University Medical Center
                                                                                                         Advisory Board Member
Deirdre R. Roney
Family member of eight IPF patients

Marvin I. Schwarz, M.D.
CPF Chairman & The James C.
                                                                                                         Maria Padilla, M.D.
Campbell Professor of Pulmonary Medicine
University of Colorado Health Sciences Center
                                                                       Associate professor of medicine and director of the Advanced Lung
Gregory Tino, M.D.
Associate Professor of Medicine,                                       Disease Program, Mount Sinai Medical Center
Director, Pulmonary Outpatient Practice, Pulmonary, Allergy and
Critical Care Division, University of Pennsylvania Medical Center

                                                                       Dr. Padilla’s clinical interests center on the pathogenesis and treatment
                                                                       of diseases such as idiopathic pulmonary fibrosis/interstitial lung
CPF Scientific Advisory                                                diseases, sarcoidosis, pulmonary hypertension, cystic fibrosis and
                                                                       collagen vascular-associated lung diseases. Her research focuses on
Board                                                                  models of pulmonary fibrosis and emphysema.
Paul W. Noble, M.D. – Chairman
Professor of Medicine, Division Chief, Pulmonary,
Allergy and Critical Care Medicine
Duke University Medical Center
                                                                       Dr. Padilla started the lung transplantation program at Mount Sinai
Harold R. Collard, M.D.                                                Hospital and served as its medical director until recently. She is an
Assistant Clinical Professor of Medicine & Coordinator, Interstitial
Lung Disease Program Division of Pulmonary and Critical Care
                                                                       active member of the faculty, engaged in the practice of medicine,
Medicine, University of California San Francisco                       teaching and clinical research. Her work has been published in several
Serpil C. Erzurum, M.D.
Director, Lung Biology Program
                                                                       journals. She is a fellow in various pulmonary and transplantation
Cleveland Clinic Foundation                                            societies and serves on the advisory board of WASOG (World Association
Adaani Frost, M.D.
Professor of Medicine
                                                                       of Sarcoidosis and other Granulomatous Diseases), Sarcoidosis Research
Baylor College of Medicine                                             Foundation, Scleroderma Foundation and on the editorial board of the
Marilyn Glassberg, M.D.
Associate Professor of Medicine
                                                                       journal Sarcoidosis, Vasculitis and Diffuse Lung Diseases. She has been the
University of Miami/Jackson Memorial Medical Center                    recipient of awards in teaching and in the practice of medicine. She has
Jeffrey Golden, M.D.
Director, Lung Transplant Program
                                                                       repeatedly been named to the “best doctors” lists in the U.S.
Director, Interstitial Lung Disease Clinic,
University of California, San Francisco (UCSF)

James E. Loyd, M.D.
                                                                       Dr. Padilla graduated with a BS in biology from the City College of
Medical Co-Director, Lung Transplant Program,                          New York and pursued her medical studies at the Mount Sinai School
Vanderbilt University Medical Center

Kevin O. Leslie, M.D.
                                                                       of Medicine. Her pulmonary training and research experience were also
Consultant Pathologist                                                 completed at Mount Sinai Medical Center.
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




                                                                                                                                                   19
Supporting the CPF                                                                                             Don’t forget!
The Coalition for Pulmonary                       Should you wish to make a tax-                      Year-end is the time to make
Fibrosis relies on the contributions              deductible contribution to the CPF,                 charitable gifts to accelerate
of individuals, corporations                      we encourage you to send your check
                                                                                                        our efforts to find a cure
and associations who share our                    or money order to:
commitment to improving awareness                                                                       for IPF. Gifts received by
and education of IPF, and improving                   Coalition for Pulmonary Fibrosis                midnight December 31, 2006
the quality of life for patients                      Suite F, #227                                     can reduce your taxable
fighting IPF nationwide. Through                      1659 Branham Lane
your generous support, the CPF will                   San Jose, CA 95118-5226
                                                                                                          income for the year.
continue to provide information,
resources and support to more than                Contributions are also accepted online by bank transfer or by using any major
128,000 IPF patients, caregivers                  credit card safely and securely through PayPal. The CPF’s PayPal ID is
and families, and to the healthcare               info@coalitionforpf.org. Contributors can visit our secure PayPal link at
professionals who treat them.                     www.coalitionforpf.org/AboutUs/contribute, or by visiting www.paypal.com.

To contribute by phone using any                  If you have any questions about your contribution to the CPF, or if you would
major credit card, please call the                like to make a restricted donation to advance specific CPF programs or research
CPF at (888) 222-8541.                            efforts, please contact CPF Executive Vice President of Development Mishka
                                                  Michon at (888) 222-8541, or by email at mmichon@coalitionforpf.org.


About the Coalition for Pulmonary Fibrosis
The Coalition for Pulmonary Fibrosis (CPF) is a   fibrosis, medical research professionals and    the Mary D. Harris Memorial Foundation, the
501(c)(3) nonprofit organization, founded in      advocacy organizations. With more than          National Coalition of Autoimmune Patient
2001 to accelerate research efforts leading to    10,000 members nationwide, the CPF is the       Groups, the National Organization for Rare
a cure for pulmonary fibrosis, while educat-      largest nonprofit organization in the United    Disorders (NORD), The Pulmonary Paper,
ing, supporting, and advocating for the           States dedicated to advocating for those with   Second Wind Lung Transplant Association,
community of patients, families, and medical      pulmonary fibrosis. The CPF’s nonprofit part-   and more than 35 leading medical and
professionals fighting this disease. The CPF is   ners include the American Thoracic Society,     research centers nationwide. For more
governed by the nation’s leading pulmonolo-       the Anne Harroun Landgraf Foundation, the       information please visit www.coalitionforpf.
gists, individuals affected by pulmonary          Caring Voice Coalition, the Genetic Alliance,   org or call (888) 222-8541.



                  Coalition for Pulmonary Fibrosis                                                                     NONPROFIT ORGANIZATION
                  Suite F, #227                                                                                           U.S. POSTAGE PAID
                                                                                                                         SAN FRANCISCO, CA
                  1659 Branham Lane
                                                                                                                           PERMIT NO. 925
                  San Jose, CA 95118-5226