Recurrent Respiratory Papillomatosis

RRPF Officers, Directors & Advisors

Marlene Stern

President

P.O. Box 6643

Lawrenceville, NJ 08648-0643

(609) 530-1443

marlenelin@aol.com

Bill Stern

Director and V. President

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 and Treasurer

12505 Cobblestone Pkwy.

Oklahoma City, OK 73142

(405) 603-8850

cneuberger@eti1.com

Maura Burke Weiner

Director

4900 Fieldwood Court

Fairfax, VA 22030

(703) 691-1922

maura.weiner@jurymatters.com

Susan Woo

Director
Hanking Court, Flat 9B
43 Cloudview Rd.
Northpoint
Hong Kong, SAR
(852) 2812 7379

susanleewoo@hotmail.com

Scientific Advisory Committee

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

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

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

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 Patient Support Assistance

Jennifer Woo
Geni Mesi
Lindsay Stern

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 over 850 respiratory papilloma families. Patients range in age from about 2 to 92 years. Domestically, patients are located in 48 states plus the District of Columbia. Outside the U.S. there are currently 0ver 70 patients from over 30 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 comprehensive RRP patient survey available online at http://www.rrpf.org/rrpf/survey. So even if you have already completed a 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.

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 providing updated information, you need only identify yourself, and answer only those questions where you have new information to provide. For the online survey, just make sure you specify the patient’s first and last names and their year of birth.

Doctors and nurses treating RRP patients, please take a few minutes to fill out the online 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).

We ask that patients and practitioners update their survey at least once a year.

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RRPF Listserve Update
by Randy Sparkman

The RRPF "listserve" continues as a valuable resource for the RRP community. As of December, 2007, the electronic mailing list has over 500 subscribers that include RRP patients, families, caregivers, researchers and healthcare professionals.

Over the past year there are have been many threads about diagnosis, treatment methods and risk trade-offs. Most importantly, the listserve is a community of care. It is a place where those of us with long-term RRP and the newly-diagnosed can share experiences and enjoy the support of others who understand the RRP experience.

The RRPF has sponsored an electronic mailing list since its inception. The list was hosted on Yahoo Groups in 1999. Yahoo's search function is included within the site. The increased number of subscribers along with the multi-year archive of threads now provides an extensive history of experience that makes the list even more useful to the community.

On average, there are now about one hundred posts per month. The posts may be received into your electronic mail inbox one at a time or can be received as a daily digest of all posts received that day. As with all forms of Internet communication, users should be careful with personal information. Posts to the RRPF website are not linked by search engines such as Google. Access to the mailing list requires registration as a Yahoo user and approval of the mailing list "moderator", currently the RRPF director. Despite these safeguards, authorized members have the ability to copy and redistribute mailing list information. So, again, be thoughtful as you post medical and personal information.

To subscribe to the list simply access: http://health.groups.yahoo.com/group/rrpf/ from your Internet browser. Those who need technical assistance with the RRPF listserve can send an e-mail to jubrising@gmail.com for one-on-one assistance.

RRP Patient Survey Stats

Please complete or update the comprehensive RRP patient survey available online at: http://www.rrpf.org/rrpf/survey

NOTE: If you have received Gardasil vaccinations whether by standard protocol or in any other manner, please indicate this on your survey via the “other” entry category.

Very preliminary statistics may be viewed at:

http://www.rrpf.org/rrpf/survey/update/admin/

user = “rrpf”
password = “Foundation” (case sensitive)

(Caution: These are “raw” stats and in some cases may not make sense.)

Patient Support

[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. We are doing this by providing small grants to two charity travel organizations, i.e., Miracle Flights for Kids and Angel Flights. If you would like more information please contact:

Geni Mesi

(801) 358-9351
e-mail: mesifam@hotmail.com

Fundraising Activities

Please come to the Sixth Annual
RRP Foundation Team USA

HOCKEY NIGHT

At the VERIZON Center
Washington, D.C.

Saturday, February 2, 2007

Questions? Contact Ed and Maura Weiner maura.weiner@jurymatters.com

100% of proceeds from this fundraising event go to the RRP Foundation. Everyone wins!

RRP Meetings

RRP Focus Session 2007 Highlights

DVD of the Focus Session will be available early in 2008!

The RRP Focus Session is an event that is often convened in conjunction with the American Academy of Otolaryngology-Head and Neck Surgery (AAO) annual convention. The following is a summary of the proceedings of the 2007 meeting, held on September 14, 2007, in Washington DC.

There were about 45-55 attendees including RRP patients, parents, RRP doctors and researchers. Highlights of this year’s meeting included updates from the RRP Foundation, the RRP Task Force, and the RRP ISA Center; updates on HPV vaccines; the heterologous effects of the MMR vaccine; Celebrex therapy for RRP; an in-office laser treatment protocol for RRP; a PDL procedure for treating RRP; and a therapeutic vaccine for canine papillomavirus. The presentations were informative and well received, and the meeting and the dinner that followed provided fertile ground for discussion and interaction among members of the RRP community, many of whom had traveled from across the country to attend.

The following summaries are presented as highlights only. Details are provided in the PowerPoint on the web at: http://www.rrpf.org/meetings/RRP_focus_2007/RRP_Focus2007Program.htm

We strongly encourage you to refer to the Powerpoints for more specific information on the topics outlined below.

I. RRP Foundation Priorities and Perspectives (Bill Stern)

Opening remarks from Bill Stern

RRP Priorities, Awareness, Epidemiology, Research Support

Ongoing and past supported projects

Major patient/family concerns

Diagnosis issues, coping with RRP, treatments (surgical and adjunct), voice (preservation, restoration/improvement), mortality (pulmonary involvement, malignancy), disease transmission

RRPF List-serve Overview - forum for exchange of information, ideas, opinions and emotions related to RRP

Currently ~500 members consisting of patients, parents, practitioners, researchers

Collecting RRP Patient Data: provide informational support for RRP families and practitioners, improve understanding o RRP epidemiology, availability of database for RRP research studies, Web-based survey linked to MySQL, coordinate with RRP ISA Center

Pulmonary RRP: greatest risk of mortality from RRP, approximately 6% of RRP patients, remains virtually untreatable, propose to establish referral centers to coordinate experimental treatments and clinical research

II. International RRP ISA Center Update (Michael Green)

Who We Are, What We Do

Policy Board - up to 9 individuals

Scientific Advisory Panel

Research efforts

Gardasil -

VLP-based vaccine, near-total immunity against HPV 6,11,16,18

Merck used reproductive tract data to generalize regarding therapeutic efficacy elsewhere

Dr. Ian Frazer - currently using VLP treatment RRP vaccine in Brisbane, China, similar to Gardasil but without Al adjuvant

AIDS Data: AIDS patients get all kinds of opportunistic HPV infections except RRP à assumption that reproductive data map to respiratory system is “highly questionable”

Want to educate more broadly on 20/20, Oprah, 60 Minutes about HPV being not just a female disorder, there is RRP involved too, it is not just cervical cancer and genital warts

III. RRP Task Force Update (Craig Derkay, MD)

Meets twice a year, in conjunction with AAO and COSM

No further funding from CDC to continue registry

Formulated statement on public health infection concerns for children with RRP

Tackling statement on HPV typing

Post-licensing suggestions for RRP vaccine trials

vaccinate cohort of children currently in remission

begin surveillance study of new onset RRP

attempt therapeutic trial

establish anti HPV 6 and 11 antibody levels in cohort of actively treated RRP patients to determine who might benefit from therapeutic administration of vaccine

Overview of Gerein et al (2006) - patients with RRP are able to have healthy children regardless of stage of disease. Pregnancy has a negative impact on disease course, worse with HPV 11.

Toxicity issues with Cidofovir: potent carcinogen in rats (Annals 2005)

Should be routinely presented as a treatment option in moderately to severely afflicted RRP patients, viable option in pt’s whose disease severity is resulting in a need for frequent surgery/worsening airway compromise/impaired communication

Role for HPV testing

Clearly, in the pediatric airway, HPV 11 = high risk

Review of several ongoing and recently published studies (Maloney, Buchinsky, Gerein, Reidy, Wiatrak)

HPV sub typing: Linear array kit, Digene HPV Test, AMPLICOR HPV test

Celebrex study - supported by Task Force

IV. RRP Genetics (Farrel Buchinsky, MD)

RRP genetics study enrollment tripled since LA 2005, discovered transmission disequilibrium in 2 candidate genes

Cause of RRP: HPV 6 and 11, necessary but not sufficient

Many exposed, only a few get the disease

Genetic susceptibility: higher prevalence in relations, HLA DRB1 0301 and DQB 0201 disproportionately present in RRP, rabbit papillomas data, HIV/AIDS, malaria, mortality by infectious disease in adoptees more associated with biologic parents than seen for cardiovascular and cancer

Overview of data collection process

Regulatory process impediment to most would-be collaborators

Which gene or genes - candidate gene or genomic scan approach

Candidates: MHC, innate immunity, known cell biology interactions, other diseases

Transmission disequilibrium test

V. Pulse Dye Laser for treating RRP (Matt Brigger, MD)

585 nm PDL in children with RRP, all children will be treated, no placebo

Safety of PDL has been established

Sites: Boston, San Diego, Birmingham, Cincinnati

The problem: mucosal disruption, scarring potential à do not disrupt opposing mucosal surfaces

Basic CO2 laser physics: 10,600 nm in a continuous beam, primarily absorbed by H2O, heated to steam, mucosal disruption

1980’s: Parrish and Anderson develop 585 nm PDL based on selective photothermolysis, destroys vessels within the lesion, destroying epithelium

Vascular core: prime target for selective photothermolysis

Destruction of papillomas vascular supply results in involution with mucosal preservation, allowing more complete debulking, potentially better voice outcomes

Initial, pediatric and adult data presented

Hartnick 2007 - no episodes of vocal scarring or web formation, trend toward increased intervals

Method: general anesthesia, microlaryngeal suspension, debulk exophytic lesions as needed, use fiber through long cannula or suction

Cleaner ablation of papillomas

Where to go from here: feasibility and safety shown, objective outcomes are lacking - does a more complete excision result in less procedures? Are voice outcomes truly better?

Randomized controlled trial with clear objective outcomes

Multi-center efforts needed

Objectives: determine if PDL can increase time interval, improved voice outcomes

PDL represents potential advantage by allowing more complete debridement; no objective outcomes; need more data to determine role in routine practice t