The RRP Foundation has been supporting and networking the RRP community for more than a decade and wants to continue to be responsive to the needs of the RRP community. In this regard we would appreciate any comments or criticisms you may have regarding the RRPF. The best way to let us know what you are thinking is by email to one of the members of the RRPF Board, i.e., Chris Neuberger, Maura Weiner, Susan Woo or Bill Stern, (see addresses listed in the section on “Organizational Information”.)

We continue to seek additional help in preparing, editing and coordinating the publication of the RRP Newsletter. In particular, we are asking for a volunteer to take on the lead role of coordinating and publishing future issues. If you are interested in assisting in any way, please contact Bill Stern (bills@rrpf.org).

We hope you find this newsletter issue to be interesting and helpful.

We are most grateful to all those individuals, medical professionals and corporations who have supported the RRPF. Although it is impossible to publish the names of all who contribute, we extend our sincere thanks to everyone who has supported our efforts. Future donations from individuals, professionals or from the business community will be very much appreciated.
Tax-deductible contributions may be made to:

RRP Foundation
P.O. Box 6643
Lawrenceville, NJ 08648-0643

Do you donate to the United Way through your employer? You can select a "Donor Choice" option, which would allow you to direct a donation to the RRPF as the 501 (c) (3) of your choice. Since the RRP Foundation is a 501(c) (3) foundation, you may specify the RRP Foundation directly by writing in the name and address of the foundation as follows' RRP Foundation, P. O. Box 6643, Lawrenceville, NJ 08648. If you should need to add our Fed. ID number, it is 521798693. Thank you for your support.

Donations accepted online via Pay Pal

From the RRPF home page (www.rrpf.org) or go dirctly to http://www.rrpf.org/donate.htm

Special Acknowledgments

We would like to thank Stressgen Biotechnologies and Medtronic Corp. for their generous support of the

2004 RRP Focus Session

And

We would like to thank Medtronic Foundation for its generous assistance of the RRP Foundation patient support program.

RRPF Officers, Directors & Advisors

Marlene Stern

President

P.O. Box 6643

Lawrenceville, NJ 08648-0643

(609) 530-1443

marlenelin@aol.com

Bill Stern

Treasurer and Director

P.O. Box 6643

Lawrenceville, NJ 08648-0643

(609) 530-1443

bills@rrpf.org

Henry Woo, Esq.

Secretary

Medtronic International Inc.

Suite 1602 16/F., Manulife Plaza

The Lee Gardens, 33 Hysan Ave.

Causeway Bay,

Hong Kong

henry.woo@medtronic.com

Chris Neuberger

Director

13001 Burlingame Ave.

Oklahoma City, OK 73120

(405) 749-8499

cneuberger@eti1.com

Maura Weiner

Director

4900 Fieldwood Court

Fairfax, VA 22030

(703) 691-1922

mauraweiner@serviceimpact.net

Susan Woo

Director
The Manhattan, Flat 33D

33 Tai Tam Road

Hong Kong
(852) 2812 7379

susanleewoo@hotmail.com

[Please see the support info. on page 16 for a complete list of the RRPF regional and state coordinators]

Scientific Advisory Committee

Thomas R. Broker, PhD, University of Alabama at Birmingham Schools of Medicine & Dentistry

Haskins K. Kashima, MD, Johns Hopkins University School of Medicine

Linda Miller, RN, MSN, Children’s Hospital of Philadelphia

Clark Rosen, MD, University of Pittsburgh Voice Center

Robert J. Ruben, MD, Albert Einstein College of Medicine

Keerti V. Shah, MD, DrPH, Johns Hopkins University School of Hygiene and Public Health

Bettie M. Steinberg, PhD, Long Island Jewish Medical Center

Kathleen Sullivan, RN, Children’s Hospital of Boston

Voice Specialist/Advisor

Julie Bowne, M.S., CCC-SLP

RRP Newsletter Editors

Chris Neuberger
Jennifer Woo

Other RRP Newsletter Contributors

Randy Sparkman

Marlene Stern

Bill Stern

RRP Reference Service Editor

David Wunrow

RRPF Fundraising Coordinator

Ed Weiner

(703) 691-1922

eweiner@weinerandassociates.com

RRPF Corresponding Secretary

Christine-Hartman Davis

RRPF Publication and Subscription Policy

The RRPF produces two publications, the RRP Newsletter and the RRP medical reference service. The RRP Newsletter focuses mainly on the human and clinical aspects of recurrent respiratory papillomatosis and in this regard targets a broad readership, including patients/families, attending physicians/nurses, as well as researchers and the general public seeking to stay in touch with RRP from a clinical perspective. The RRP medical reference service serves those in the community seeking a more comprehensive understanding of this disease. Please help us by supporting these publications and other RRP services including patient outreach, support, advocacy and research

Subscription Policy and Suggested Minimum Annual Donations:

RRP Newsletter

Professional/Corporate - $25
Individual - $15

RRP Newsletter plus Medical Reference Service

Professional/Corporate - $40
Individual - $25

[Note: Issues of the RRP Newsletter and Medical Reference Service are available on the website.]

RRP Network News

Our international support network has grown to approximately 700 respiratory papilloma families. Patients range in age from about 2 to 88 years. Domestically, patients are located in 48 states plus the District of Columbia. Outside the U.S. there are currently 33 patients from 14 countries.

Our thanks to all who have taken the time in the past to fill out the RRPF Patient/Therapy Survey. There is now a new conprehensive RRP patient survey available online at http://www.rrpf.org/rrpf/survey. Development of this new survey has been a collaborative effort with Thomas Mingot, Director, Clinical Research and Operations at Stressgen Biotechnologies. We have also invited additional collaboration with the RRP ISA Center, with the goal of evolving the new survey into one that will be adopted as an RRP community standard. So even if you have already completed an older survey, help us to learn more about this disease by taking a little time to complete the new survey. Please make sure to alert us of changed addresses by checking the “new address” box. There is also a box which we ask you to check if you do or do not want your name and address information to be included in the RRPF Patient Directory. We are requesting the information contained in this survey be made available for RRP research. In this regard there is a place in the beginning of the survey to grant permission. Finally, even if you are not able to provide answeres to all the questions, it is important that you get the information you provide entered, by remembering to click on the “submit” button at the end of the survey. If you have more information to enter at a later time, it is easy to provide an update as outlined below,

As our support network has grown, we have become more dependent on the patient questionnaires to maintain our mailing list and keep our database of RRP patient information up to date. If you are updating a previously filled out questionnaire, you need only identify yourself, and answer only those questions where you have new or updated information to provide. This is also the case for the new comprehensive survey, just make sure you specify the patient’s first and last names and their year of birth.

Doctors and nurses treating RRP patients take a few minutes to fill out the practitioner survey form.

You can find the online “patient survey” and “practitioner survey” respectively on the “patient” and “practitioner” page links from the RRPF home page (www.rrpf.org).

……………………………………………………………………

RRP Web/Internet News

by Bill Stern

The Internet is now the most often used information exchange for the RRPF. Our website (www.rrpf.org ) has recently been redone with a totally new look, which we hope will make finding information easier. It contains a wealth of information relevant to patients, families, doctors, nurses and researchers. It includes an online database of RRP practitioners (updated through January 2005). The website has a new Interactive Discussion Forum which allows for the posting of questions, comments and replies to previous postings relevant to RRP. We also have the RRPF Email Listserve.(see below), linked to the home page. As noted above, you can find the new RRPF Patient/Therapy Survey and RRPF Practitioner Survey forms on line, which allow RRP patients and caregivers to easily submit their survey to the foundation. This is a very important aspect of the Foundation in that this information is used in analyzing RRP treatment therapies, experiences, etc. We ask that patients and practitioners update their survey at least once a year.

Also, we maintain an online library of RRP Newsletter and RRP Reference Service issues plus links with many other sites relating to RRP and much more.

If you have some experience/expertise with the WWW and would like to help us improve our website, please contact Bill Stern.

……………………………………………………………………

RRPF Listserve Highlights

by Randy Sparkman

The RRPF-sponsored e-mail distribution list is now five years old. Its 220+ subscribers continue to maintain a lively and useful dialog. There have been over 500 posts over the past six months. Of recent note, there has been much discussion regarding the spread of papilloma to the lungs and whether there is a need for regular bronchoscopies, x-rays or cat scans in all cases.

RRP patients, health care providers and caregivers share technical information and opinions about the various RRP treatments and adjunct therapy. But most importantly, the “listserve” is a vital way for the newly-diagnosed and RRP veterans to know that they are not alone and that RRP can be managed.

Listserve users should be aware that the RRPF e-mail list is vulnerable to the same issues as all on-line services. Concerns among list users about patient privacy led the RRPF leadership to restrict access to the mailing list to only users who have registered with the hosting service, YahooGroups. Even though anyone may register with YahooGroups, limiting access to registered users prevents access from open Internet services like search engines, etc. In any case, subscribers with privacy concerns should not post full names, postal addresses, e-mail addresses, etc.

There have also been recent posts about computer viruses. Users should not forward e-mail attachments to the mailing list and should not open any attachments within messages received from the list. This does not mean that the mailing list increases the risk of receiving a computer virus, it is simply good practice to delete e-mail messages with attachments unless you are absolutely certain of the identity of the sender and the content.

Basic subscription information and complete list archives are available on the Internet/World WideWeb at: http://groups.yahoo.com/group/rrpf. The messages may be generated and received from within your e-mail computer client or can be completely generated and received from the yahoogroups rrpf list web pages. Messages may be received one at a time or in a "daily digest". Anyone within the RRPF community that needs technical assistance with any aspect of the mailing list can send an e-mail to: rsparkman@bellsouth.net

RRP Remission News

!!! A new addition to our growing list of remissions!!!

Nikole, who was born in September, 1990 in Ohio, was diagnosed with RRP at a year old in 1991, at which time her airway was almost completely blocked by the lesions.

She went a couple years with surgeries, about once every 2-3 months but in 1994 she took a dramatic turn for the worse requiring surgeries every week. This is when her doctor in Cincinnati suggested Interferon shots to boost her immune system. She received 2 doses daily for a six month period and continued with weekly surgeries. The Interferon treatment was taking its toll making her sick and weak. At first it didn't appear the treatment was working but then Nikole started to improve after she was taken off the Interferon, as her doctor had indicated would be the case. Her surgeries gradually started to spread

out.

Around 1998-1999 she was clear and only required annual check-ups at the office. Finally in 2002 her doctor told Nikole that she would only need to return if she had symptoms. She has had no problems since and is now a very active 14 year old.

RRP Patient Stats

RRP patient statistics will appear again in the next newsletter issue, as we are just starting to gather additional RRP statistics via a new comprehensive RRP patient survey available online at: http://rrpf.org/rrpf/survey

This new patient questionnaire is intended to enhance our epidemiological knowledge of RRP with expanded surveying of diseae related issues, surgical and non-surgical treatment histories/responses, plus additional questions to assess the scio-economic and public health impact of RRP. There may be a number of questions for which you do not have answers, just answer as many as you can and remember to get the information formally entered into our database by clicking on the submit button at the end of the survey.

Support and Fundraising Activities

[For support of new RRP research initiatives, please see section on “Science and Research Activities”]

Support for RRP patient related travel expenses:

The RRPF has dedicated a limited amount of funds to provide indirect support of some travel expenses to obtain treatment for RRP families truly in need. If you would like more information please contact:

Geni Mesi

5780 Village Way

South Ogden, Utah 84403 (801) 695-0108
e-mail: mesifam@hotmail.com

Fundraising Activity:

Running For RRP – 2^nd Marathon Fundraiser

On April 18, 2005, RRP patient and newsletter editor Jennifer Woo will be running the Boston Marathon for the second time to raise awareness and funds to support RRP research and networking between patients, physicians and scientists. Please see the RRP Newsletter Spring 2004 for more about Jennifer.

She encourages all Boston-area affiliates of the RRPF to contact her at jwoo@fas.harvard.edu if they would be interested in coming out to celebrate this fundraising effort for the RRP Foundation. (More to follow)

RRP Meetings

22^nd International HPV Conference
in
Vancouver, British Columbia, Canada
HPV/RRP Education and Advocacy Forum
2005 May 5-6

The 22^nd International HPV Conference will be held in Vancouver at the Hyatt Regency from 30 April to 6 May 2005. The RRP Foundation will be participating in the HPV/RRP Education and Advocacy Forum which is scheduled to be held on Thursday May 5, with a session devoted strictly to RRP during the afternoon (~ 2-4 PM). The forum will continue on Friday May 6. For more information go to: http://www.hpv2005.org/

If you are interested in attending, please contact Bill Stern at bills@rrpf.org.

……………………………………………………………………

Summary of Fall 2004 RRP Task Force Meeting
Minutes prepared by Craig Derkay, MD
summarized below by Bill Stern

On Monday, September 20, 2004, a meeting of the RRP Task Force took place in conjunction with the annual AAO meeting in New York City. There were 20 members present including Chris Neuberger and Bill Stern representing the RRP Foundation.

A number of RRP research initiatives were discussed as follows:

Dr. Farrek Buchinsky’s RRP Genetics Study - for more info. see “Science and Research Activities” section.

Hsp-E7 proposed Phase III trial – at this time FDA is currently reviewing Stressgen’s proposal. It is anticipated that 25-35 centers in the U.S., Canada and abroad will participate and 130-140 children with severe RRP would be enrolled. (See update in “Science and Research Activities” section)

Merck quadrivalent vaccine (i.e., HPV 6,11,16,18) is in phase III trial showing great promise. Animal data shows antibodies to HPV 6 & 11 in the offspring of vaccinated mothers up to 13 weeks postpartum. If this applies to humans it could be very effective in preventing HPV transmission from mother to child.

Dr. Mark Shikowirtz spoke about the proposed Celebrex study at LIJ. For more information see the 2004 Focus Session highlights that follow, and an update in the “Science and Research Activities” section.

Michael Green distributed articles about two new skin papilloma agents, Artemisinin and Hamlet.

The rest of the meeting focused around two additional issues:

The first topic was Public Health issues regarding the risk in school settings for transmission of papillomas in children, especially those with tracheostomies. As a follow up, the Task Force has prepared a Public Health policy statement that concludes that patients who have RRP, with or without a tracheostomy tube, should be allowed to attend classroom programs, since the risk to both classmates and classroom personnel is viewed as being extremely low.

There was extensive discussion associated with the second topic, which revolved around the safety of cidofovir. Much of the otolaryngology community treating RRP patients have expressed enthusiam about cidofovir’s effectiveness in treating this disease. However, some studies, including one cited by a toxicologist from the FDA, are showing that cidofovir is a potent carcinogen in rats, even at doses comparable to those used for intralesional injections to treat RRP. Until more is undestood about the long-term safety profile of cidofovir in humans, the RRP Task Force is recommending that,

[patients and doctors]

1) Given the promising results reported in pediatric and adult patients, cidofovir should be routinely presented as a treatment option in moderate to severely afflicted RRP patients. i.e.; those patients whose disease is not improving on surgical therapy alone or in conjunction with less potentially morbid adjuvant measures and/or requiring surgical intervention 3 or more times a year. With appropriate consent, cidofovir therapy should be a viable option in patients whose disease severity is resulting in a need for frequent surgery, worsening airway compromise or severely impaired communication or those who otherwise may be considered candidates for tracheostomy.

2) Patients with more mild disease, particularly children, should be discouraged from seeking treatment with cidofovir, until a better understanding of the long-term implications of the use of this drug have been established. With appropriate informed consent, cidofovir could still be utilized on a case-by-case basis, at the discretion of the prescribing physician, for the more mildly affected patient.

[doctors]

3) As with all surgical procedures, informed consent should be obtained and documented in the patient's record. At a minimum, this should include a frank discussion of the nephrotoxic and carcinogenic potential of this drug.

4) Adverse responses, particularly evidence of dysplasia or malignant transformation to squamous cell carcinoma, either locally or remotely, should be reported simultaneously to the FDA (form 3500 or 3500A) and to the RRP Task Force through email communication with its Chairman, Craig Derkay, MD. (derkaycs@chkd.com)

RRP Focus Session 2004 Highlights

On September 18, 2004, in conjunction with the American Academy of Otolaryngology meeting in New York City, the RRP Foundation and the International RRP ISA Center co-sponsored a special meeting dedicated to RRP. It was the fourth RRP focus session since the first one took place in January of 1999 in Charleston, S.C. at the HPV meeting. Generous support for this meeting was provided by Medtronic Corporation and Stressgen Biotechnologies.

There were approximately 80 - 100 attendees, including about 50-70 RRP patients/family members and about 20-30 RRP doctors / researchers. In addition to the many RRP families from the New York City area, some people traveled from more distant locations such as Michigan, Oklahoma and even as far as California.

Bill Stern and Michael Green provided some introductory remarks and discussed some organizational objectives. They were followed by presentations from Thomas Mingot, Dr. Bettie Steinberg, Dr. Mark Shikowitz, Dr. Graciela Andrei (who came all the way from Belguim!), Dr. Nigel Pashley, Dr. Alan Shaw, Dr. Robert Bastian and Dr. Tom Broker. Due to the severe impact of Hurricane Ivan in the Birmingham, Alabama area, Dr. Brian Wiatrak was unable to attend, but did email his presentation, which Dr. Broker was able to cover in part during his talk. After the presentations there was time for some interesting follow-up discussion.

The following is a summary of some of the highlights from the presentations (most in”bullet point” format):

RRP Priorities, Statistics and Perspectives
Bill Stern, RRP Foundation

RRP Foundation priority activities

1) Provide RRP information, RRP physician referral, networking and emotional support primarily via the RRPF website, RRP Newsletter, RRP Reference Service, and RRPF email listserve

2) RRP Epidemiological data:
Data collected via practitioner and patient survey forms

Maintain RRPF practitioner and patient databases

Analyses of patient supplied data and assistance to researchers

New online patient survey and web based database being developed in collaboration with T. Mingot of Stressgen
Coordinate with RRP ISA Center and propose that RRP Task Force endorse and host new RRP patient survey forms.
3) Address some of the major patient/family concerns including:
Proper and early diagnosis of RRP,
Help in coping with RRP,
Help in finding proper RRP treatments – both surgical and adjunct,
Ways to preserve, restore and improve voice quality

Mortality - pulmonary involvement, malignancy
Disease transmission

4)RRP research - collaboration and support

Analysis of patient/parent assessed adjunct therapies for RRP

According to RRPF patient survey results the four leading adjunct therapies for RRP are, I3C/DIM, Interferon, Mumps vaccine (with reported efficacies of between 50% and 60% and Cidofovir (with a reported efficacy of about 80%). An indication that adjunct therapies may be having an impact on reducing surgical procedures for RRP was seen in a diagram showing a reduction over the last 6 years in the percentage of patients requiring 6 or more surgeries / year from about 35% to just over 25%.

RRP research activities with support from and/or involvement by the RRPF

Completed:

Risk factors for JORRP

Mouse study investigating I3C/DIM and BMD

Quality of life studies for JORRP patients

Ongoing/Proposed:

Assist with recruitment for HspE7 RRP trial

Assistance with study of RRP genetic susceptibility

Study of HPV vaccine applicability for RRP

Proposed for future:

Assessing genetic patterns in treatment response

Treating pulmonary RRP

International RRP ISA Center 2004 RRP Focus Session Michael Green, International RRP ISA Center

Organizational Description

The RRP ISA Center was established 1998 with a 9 person board of directors that includes a physician, an R.N., a clinical social worker, a genetics researcher, parents of children with JORRP, adult RRP patients and a representative of third world nations.

The scientific advisers include physicians and RRP researchers.

Some of the organizational goals and activities include:

Educate RRP patients and families so that they can make more informed treatment decisions

Create an empowering and supportive community network for those afflicted with RRP

Improve treatment of RRP and eventually find a cure

Make distributions to RRP-related organizations and to RRP patients and families who cannot afford medically necessary treatment

Sponsor educational forums that will provide treatment and research-related information to physicians and patients

Advocate on behalf of patients whose insurance coverage has denied benefits for RRP, and also with physicians

On April 18, 2005,

Your support for this cause is very much appreciated!

Pledge per mile or a flat amount to: marathon@rrpf.org

or send a check to the
RRP Foundation

Analysis of patient/parent assessed adjunct therapies for RRP

RRP research activities with support from and/or involvement by the RRPF

Organizational Description

On April 18, 2005,

Your support for this cause is very much appreciated!

Pledge per mile or a flat amount to: marathon@rrpf.org

or send a check to the RRP Foundation

Analysis of patient/parent assessed adjunct therapies for RRP

RRP research activities with support from and/or involvement by the RRPF

Organizational Description

or send a check to the
RRP Foundation