RRP Foundation Pulmonary Papillomatosis Research Initiative (PPRI) 2018 Grant – RFP

The Recurrent Respiratory Papillomatosis Foundation is continuing its PPRI by issuing a request for proposals (RFP) from qualified investigators interested in advancing understanding and treatment of pulmonary RRP.  For application details see:  http://rrpf.org/RRP_research/PPRI_RFP2018.pdf 

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In memory of Jeremy Spock

I am very saddened to report that on September 9, 2018, RRP patient Jeremy Spock passed away at the tragically young age of 35.  He was first diagnosed at 8 months old. I first met Jeremy when he was about 9 years old and was amazed to learn that this happy kid, who I played a bit of soccer with, had already endured over 100 surgeries for RRP. Over his short life, Jeremy had over 350 surgeries, but it was about 5 years ago that the RRP progressed to his lungs and transformed to carcinoma. Recently, Jeremy was part of an immunotherapy trial, which unfortunately did not work for him.
Throughout the many years that Jeremy battled RRP his parents Susan and Bob Spock were incredible advocates in seeking the best available treatments.
Although I believe that I only met Jeremy personally on two occasions, I felt so much closer to him.  Jeremy will be missed by so many of those he touched in life.

 

Bill

 

The Spock family has requested that for those who wish to make a donation in memory of Jeremy, please donate to the RRP Foundation at: rrpf.org/donate

Donations will be dedicated to pulmonary RRP research

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RRP & Avastin Online Survey for Patients/Parents

RRP Community- Anonymous Survey to Patients

 

The recurrent respiratory papillomatosis (RRP) task force is currently working to develop an expert consensus statement for the use of systemic bevacizumab (Avastin) in the treatment of RRP. We are in the process of identifying all practitioners who have experience using intravenous bevacizumab for this purpose.

If you or someone you know has been treated with intravenous bevacizumab we invite you to complete following 1-minute survey asking for the name and location of the hospital where treatment occurred. We are not requesting any personal or patient information. In no way will your personal information be linked to any email, survey, or study:

Link to Anonymous survey: RRP + Avastin Treating Hospital Information

Or copy and paste url into browserhttps://stanforduniversity.qualtrics.com/jfe/form/SV_aaa22uB5G5o443H

This is a completely anonymous survey. We cannot link your name or information to any answers you submit in this survey. There is also no way for us to identify you based on the information provided about the institution.

If you have any questions, please contact Kim McClellan at themcclellanfamily@comcast.net

 

Sincerely,

 

Douglas R. Sidell, MD

Stanford University

Department of Otolaryngology, Head and Neck Surgery

Division of Pediatric Otolaryngology

 

Background:

 

Although anecdotal evidence suggests bevacizumab to serve as a disease-suppressant rather than a cure for patients with severe RRP, it has shown great promise in dramatically reducing papilloma burden in the majority of patients with severe disease who undergo infusion. Despite this promise, many key facets of treatment remain undetermined: 1) optimal dosing concentration, 2) treatment interval and duration, 3) appropriate follow-up laboratory and/or imaging studies, and 4) outcome measures. Developing a registry for ongoing data collection is critical for understanding outcomes including biopsy results, symptom profile, pulmonary sequelae, complications, response to treatment, relapse and response after retreatment. In addition, by establishing consensus and collecting data through this process, practitioners can justify the use of systemic bevacizumab for severe RRP, as this medication is often not available at many major medical centers who provide ongoing treatment patients with severe disease.

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RRP & Avastin Online Survey for Practitioners

Dear Colleague,

On behalf of the Recurrent Respiratory Papillomatosis Taskforce, I am inviting you to participate in the development of an expert consensus statement regarding the use of systemic bevacizumab (Avastin) in the treatment of recurrent respiratory papillomatosis (RRP).

Although anecdotal evidence suggests bevacizumab to serve as a disease-suppressant rather than a cure for patients with severe RRP, it has shown great promise in dramatically reducing papilloma burden in the majority of patients with severe disease who undergo infusion. Despite this promise, many key facets of treatment remain undetermined: 1) optimal dosing concentration, 2) treatment interval and duration, 3) appropriate follow-up laboratory and/or imaging studies, and 4) outcome measures. Developing a registry for ongoing data collection is critical for understanding outcomes including biopsy results, symptom profile, pulmonary sequelae, complications, response to treatment, relapse and response after retreatment. In addition, by establishing consensus and collecting data through this process, practitioners can justify the use of systemic bevacizumab for severe RRP, as this medication is often not available at many major medical centers who provide ongoing treatment patients with severe disease.

Our first goal is to identify all physicians who have treated adults or children with RRP using intravenous bevacizumab. We request that you please complete this short (3 minute) questionnaire by clicking this link:

IV Avastin for RRP Survey

If you have not personally used systemic bevacizumab but know of someone who has we would be grateful if you could forward this on to them.

Sincerely,

Douglas R. Sidell, MD
Simon Best, MD
Karen Zur, MD
Craig Derkay, MD
Anna Messner, MD
Karthik Balakrishnan, MD

Follow this link to take the online IV Avastin for RRP Survey:
Take the Survey

Or copy and paste the URL below into your internet browser:

https://stanforduniversity.qualtrics.com/jfe/form/SV_2gydR7Ei5xtkwMR?Q_DL=cU4PAVrDJX0GcD3_2gydR7Ei5xtkwMR_MLRP_d76bQTbssMBXr3n&Q_CHL=email

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In Memory of Karen Barnes Medley

It is with much sadness that we report that 36 year old Karen Barnes Medley has tragically lost her battle with pulmonary RRP and lung cancer.

[Excerpted from a “GoFundMe” page for Karen]
Karen was diagnosed with RRP as a child. Throughout her childhood, she had countless surgeries on her vocal cords and had been in remission since the age of 10. Recently she went to the doctor as she had a faint, lingering cough that just wouldn’t quit. Her world was turned upside down when she found out the RRP came back, went into her lungs and converted into cancer.

She fought the disease fiercely and leaves behind her husband and two beautiful children.

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Highlights of 2018 Spring RRP Meetings – National Harbor, Washington, DC

On 2018 April 19 representatives from the RRP Foundation attended and participated in the RRP Task Force meeting. On April 20, as part of the larger 2018 Spring Combined Otolaryngologic Societies Meeting (COSM), representatives of the RRPF also attended an RRP panel discussion, which included presentations from some of the leading RRP researchers. The following links provide some detailed highlights of these meetings:

http://rrpf.org/meetings/RRP_TaskForceSpring2018Meeting_HighlightsReport.pdf

http://rrpf.org/meetings/RRPPanelDiscussionCOSM2018.pdf

We were very encouraged by some of the research/clinical topics presented/discussed.

If you have any questions or feedback, please post to the RRPF email listserve or to the RRP Foundation Facebook page.

 

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In Memory of Margarita Kleinman

We are very saddened to learn of the death of 39 year old Margarita (Margie) Kleinman, who lost her battle with pulmonary RRP earlier this month (April 2018). Margie was diagnosed with RRP at the age of three and according to her father had frequent surgeries during her childhood, although he didn’t know how many or at what point the papillomas migrated to her lungs. It was in recent years that the pulmonary papillomas transformed to lung cancer. Marlene and I speak for the entire RRP Foundation in extending our sincere condolences to Margie’s family, particularly her daughter and son who are now without their mother. – Bill Stern

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HPV Awareness Day March 4, 2018

The International Papilloma Virus Society along with a number of HPV partner organizations have designated March 4, 2018 as HPV Awareness  Day.  It is hoped by creating greater awareness of the HPV along with ways to prevent and treat it, we may be able eliminate HPV and RRP for future generations.

You can find more information at the HPV Awareness Day website:

http://www.givelovenothpv.org/

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RRP Task Force Meeting in Chicago on May 19, 2016

On May 19 The RRP Task Force, headed by Dr. Craig Derkay held their Spring 2016 meeting in Chicago. Representing the RRP Foundation at this meeting, is long time RRP patient and advocate, Kim McClellan.

Highlights of the meeting compiled by Kim McClellan:

RRP Task Force Meeting Minutes
Hyatt Regency Hotel
Chicago, IL
May 19, 2016

Members Present: Craig Derkay, Ben Anthony, Harlan Muntz, Simon Best, Elissa Meites, Kim McClellan (RRPF), Seth Pransky, Jeffery Simons, Libby Smith, Matt Brigger, David Tunkel, Marc Thorne, Chris Hartnick, Henry Milczak, Shazia Peer, Yang Xiao, Terri Giordano, Adam Klein, Scott Schraff, Farrel Buchinsky, Stephen Conley, Fred Dikkers, Pamela Mudd, Bill Stern-RRPF (via Skype)

Dr. Derkay opened the meeting with information regarding a few RRP focused lectures during COSM. RRP lecture on HPV immunology treatment/polarization, Zeitels on minimally invasive airway procedures as they related to RRP, a lecture on Difficult Airways, as well as a lecture (ASP) on the Efficacy of High Dose Avastin as it relates to RRP.
➢ June 2016, the RRPF was made aware of a presentation at COSM by Dr. Zur, of CHOP, regarding Avastin, used in intravenous infusions, in a pediatric case of pulmonary RRP/tracheal RRP with success.

Upcoming RRP events and RRP Grants:
a. International HPV Eurogen meeting in Salzburg, Austria
b. Core grant, $19,976 (approved by ASPP board):
Prosser, Georgia Regents University, “Characterizing Xenograt Models of Recurrent Respiratory Papillomatosis”
c. ASPO Survey approved: Goodstein, Nemours, “HPV Vaccination Attitudes and Current Practices”

Vaccine Update:

o Gardasil 9: approved 2015, will be replacing the prior Gardasil vaccine. G9 appears to be better tolerated than previous Gardasil. You do not need to administer G9 if the patient has already had the prior vaccine. Can switch to 9 if already in a Gardasil series. CDC current guidelines on age is: Female-9-26, Male-9-21.
Immunogenicity and Safety of a 9-valent HPV vaccine
• —Van Damme P, Pediatrics, 2015;136;e28-e39
• —3066 women 16-26 given 9-valent vaccine
• —>99% seroconverted for each vaccine HPV type
• —Responses in girls and boys were non-inferior to those of these young women
• —Persistence of response demonstrated 2.5-3yrs after dose 3
• —Generally well-tolerated with mild injection site AE’s
• —Conclude: Data support guidelines adopted by ACIP
• —Comment: Encouraging safety and immunogenicity data

o VGX-3100:
John Hopkins, Simon Best, ASPO grant award. VGX-3100 includes plasmids targeting the E6 and E7 proteins of HPV types 16 and 18. Intramuscular injection of the plasmid DNA immunotherapy is followed by electroporation using the CELLECTRA® delivery device. In a phase II trial, VGX-3100 induced regression of precancerous cervical disease and cleared HPV infection with robust T cell responses. http://www.cancerresearch.org/news-publications/our-blog/september-2015/new-study-hpv-vaccine-holds-promise-in-treating-cervical-cancer
• How this relates to RRP: There are DNA vaccines that target the HPV E6 and E7 proteins which are also the key proteins for HPV6/11. If they are successful in producing a therapeutic response with the HPV 16/18, extension to HPV 6/11 should be straightforward. With HPV 16/18 being more oncogenic and related to cervical cancer, it represents a much larger market than the RRP community, hence the initial focus on those HPV types.

o ADXS11:
Mt. Sinai; ADXS11-001 immunotherapy using a live Listeria monocytogenes bioengineered to secrete a HPV-16-E7 fusion protein targeting HPV transformed cells. Currently focusing on Tonsil and Tongue Base HPV. (How it relates to RRP, see above.)

Cidofovir:
Mention was made on how European colleagues could access:
Samir Parikh
CEO
Nexcape Pharmaceuticals Ltd.
15 Martinfield Business Centre
Welwyn Garden City
AL7 1HG
United Kingdom
Phone: +44 20 3667 3070 / Fax: +44- 20 3163 0707

Pegasys:
Pegasys is a new alpha-interferon which seems to be better tolerated. For assistance in administering to pediatric RRP patients, contact Kathleen Schwarz, MD, Pediatric Hepatologist at Hopkins, 410-955-8769

Celebrex:
Dr. Bettie Steinberg will be presenting the findings of this study in Salzburg. General result is that it worked no better than the placebo. Several comments were made to the quality of this study and how it was formed and implemented. The word “ideal” when referencing the study design was used.

Misc:
A. Genetics Study: Dr. Buchinsky reported on interim results regarding 611 patients. Still analyzing HLA data. Preliminary data on his study showed age at DX greatest predictor of clinical course. Has tissue and HPV sub-typing on 230 children and 60 adults. (To be noted, there were at least two dissentions on his results based on prior patient data in those present and their cohorts. This result also goes against RRPF data showing type as the strongest indicator of disease process.)

B. “Three Peaks”, Dr. Dikkus. Dr. Dikkus then described his study recognizing “Three Peaks”, a study using all white Europeans. He showed three peaks for JORRP. Seven years of age, thirty-four years of age and sixty-two to sixty-four years of age. Mentioned this correlated with a Costa Rican study of 20K oral HPV cases. Seemed to be some disagreement into whether this actually matched what others saw in their group. Dikkus study is published in “Clinical Otolaryngology.”

C. Emory Tissue Bank: Adam Klein’s tissue bank is up and running collecting blood, saliva and tissue (mostly in adults with RRP, but open to all). Goal will be to coordinate collection of relevant demographics with CDC study in children with RRP. Currently with 49 adult patients in the tissue bank. Additional pediatric tissue banks created at San Diego, Mass Eye and Ear as well as CDC.
• San Diego: Growing papilloma for in vitro for drug testing
• Boston: Growing RRP in epithelial cells and coordinating with Dr. Schlegel in DC to try and create an immortalized cell line.

D. Dr. Pransky, researcher, is doing in-vitro sampling/testing with possible therapeutic drugs on tissues grown in the lab from patients. Currently phase two and will be requesting some funding from the RRPF.

How the tissue banks relate to RRP: having an extensive collection of RRP patient’s tumor tissues should help researchers with new drug testing. They can do full genomic sequencing on the tumors to see if they can find genetic alterations that might respond to specific treatments.

CDC Study:
A representative from the CDC was in attendance (Elissa Meites, Atlanta). Currently there are 18 pediatric centers in the U.S. enrolled with 93 patients in the study. JORRP cases only. No mention of this including any patients that were JORRP and are now adults. Centers must have IEB approval and sub-award agreements to participate. Started study in 2015 and looking to type specific DNA/Antibodies. Boston Children’s and University of Tennessee latest to come on board.
EVMS 2
Children’s Hospital Cincinnati 16
University of Kansas 3
Children’s National Research Institute 5
University Alabama 8
Indiana 2
Emory 4
Johns Hopkins 23
Lurie Childrens 14
Nationwide Childrens 4
University Maryland 3
University of Kansas 3
University of Minnesota 1
Children’s Hospital of Philadelphia 2
Utah Childrens 3

Additional centers interested in participating should contact Dr. Derkay and Laura Stone, RN for assistance in getting started. craig.derkay@chkd.org stonelj@evms.edu

Additional Gardasil 9:

MUSC-Twenty patients with retroactive Gardisil vaccine showed varied response rates. Some increased intervals between procedures by three months, this was not controlled. Based upon this “study”, males showed greatest response.
http://www.ncbi.nlm.nih.gov/pubmed/25619468

Dr. Dikkers of the Netherlands, introduced us to a recently available study he was part of regarding Immunological response to quadrivalent HPV vaccine in treatment of recurrent respiratory papillomatosis. http://www.ncbi.nlm.nih.gov/pubmed/27188508 This has no clinical response data, only measured antibody response.

Should Gardasil (or the upcoming 9-valent vaccine) be initiated earlier than at age 9 to our RRP patients? The group agreed that this would be appropriate. Data gathering at individual sites where this is pursued is encouraged for use in the future in a potential retrospective study. Suggested that this may be delivered at the time of surgical interventions to encourage third party payers to cover the cost.

Ideas for RRP Task Force Research Initiatives:

• HPV sub typing: Matt Brigger: Systematic review completed. Difficult to control for age in the meta-analysis. Type 11 appears to be more aggressive in terms of need for tracheotomy, adjuvant therapy, distal spread. Heated debate among the Task Force regarding which factor (early age of onset vs. HPV type) is most predictive of aggressive course in children. (RRPF data indicates HPV type as indicator, but for a given HPV type early age onset may also be a factor.)

• A decision was made to pursue a publication based upon the systematic review including a Task Force recommendation to sub-type all pediatric RRP patients.

• After the RRP Task Force meeting, Dr. Derkay met with Dr. Jack Krouse, Editor of OTO-HNS (white journal) and secured a commitment to publish an Invited Commentary on this topic to be authored by Drs. Brigger, Dikkers, Buchinsky, Hartnick and Derkay.

New Business:

A please for Open Access of RRP research data was made-specifics of how this may be accomplished in the future to come.

Please be generous and contribute to the ASPO RRP Research Fund to continue funding of pilot projects. To date we have raised only $67K towards our goal of $100K. ASPO Board will only issue awards every other year until we can reach our goal. You may contribute to the ASPO “Sustaining the Future” campaign and designate your contribution to the Micah RRP Research Fund. Checks should be sent to:

ASPO RRP Research Fund
c/o Bruce Maddern, ASPO Treasurer
10475 Centurion Pkwy N
Suite 302
Jacksonville, FL 32256
drmaddern@gmail.com

(this is a merged document of advocate minutes and Task Force minutes)

Craig Derkay, MD, FACS, FAAP
Professor and Vice-Chairman
Department of Otolaryngology Head Neck Surgery
Eastern Virginia Medical School
Children’s Hospital of the King’s Daughters
Norfolk, VA 23507
craig.derkay@chkd.org (o)757-668-9853 (c) 757-439-5752

Kim McClellan, RRPF Advocate and Task Force Meeting Representative
Chicago, IL
themcclellanfamily@comcast.net

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Jenn Woo, President of the RRP Foundation, has passed away

Dear RRP Community,

It is with deep sadness and sorrow that I am letting you know that Jenn
Woo, President of the RRPF, has passed away from complications
associated with pulmonary RRP and lung cancer. Over the last few years
despite having to battle this most challenging form of RRP, she
completed her medical degree at Georgetown and became a practicing
physician. More details will be forthcoming.

She touched the lives of so many of us and will be sorely missed.

With much sympathy to her family and friends,
Bill

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