RRP ISA Survey Results

    (Important Disclaimer)


    2005 (Los Angeles)


    The following is a summary of material that was shared at “RRP Focus Session: Thinking Outside the Box” event in Los Angeles on September 24, 2005.  

    This year’s event was produced (planned, facilitated and paid for) by the International RRP ISA Center.  It was supported by grants from Medtronic and Stressgen Technologies. The next RRP Focus Session will be produced by the RRPF.  

    About 45-55 RRP patients and their families attended, along with about 10-15 physicians and researchers in addition to  those who were speakers.   Four lay people presented and the other 8 speakers (counting Mark Shikowitz who was inserted into the agenda at the meeting) were physicians or PhD level researchers.  

    The meeting was very well-received and stimulated a great deal of discussion at the dinner afterwards in honor of the speakers.   

    Much ground was covered.  The following summaries are presented as highlights only . Details are provided in the PowerPoint and MP3 files.  The presentation sequence below is different from that shown in the agenda.  

    Readers are strongly encouraged to listen to the MP3 recording and consult the PowerPoints for more information.   

     1) Michael Green, MSW, LICSW       Intl RRP ISA Center's President & Executive Director

            Photo      PowerPoint        MP3 

    Michael acknowledged with gratitude the receipt of two grants from Medtronic and Stressgen Biotechnologies that helped make this event possible. He told the story of how RRP ISA came to be and he explained how ISA is an acronym for Information, Support and Advocacy.  He reviewed RRP ISA’s future plans and past activities/accomplishments, which amongst other items have included: 

    A)   Awarding of research grants to two institutions, with others in the review stage. In addition to the past grants, RRP ISA announced it would offer up to $100,000 in research grants over the next year.  

    B)   A new website that is in development.

    C)   A new message board that was just opened.

    D)   A new informational brochure on RRP that doctors can give to their patients .

     E)  RRP ISA’s presentation at the International Papillomavirus Society’s 2005 convention in Vancouver B.C. last May.  Michael presented a synopsis of data from that presentation, entitled “RRP Patient and Family Data Trends Report.)

     (2) Kathy Blankenship, EdM       Intl RRP ISA Center's Research Director and Board Secretary

            Photo      PowerPoint        MP3

    Kathy provided a very emotional and moving story on her personal experience, which included a cancer diagnosis and the removal of her larynx. Kathy shared her perspective on: 

    A)  The effect her RRP has had on her children and how our children help us to realize what is truly important in life. 

    B)   The importance of providing information, support and advocacy to patients and their families; and the importance of emotional support, health care referrals, case management and patient-friendly websites. 

    C)   Her experience with discrimination by health care providers. 

    D)   Patients Beware/Caveat Emptor: The expectations RRP patients have of their physicians to use good judgment,  provide best care possible, and be knowledgeable about RRP and current treatments. 

    E)  The need for a patient to do their own research and educate themselves about RRP and its treatment. 

    F)   The importance of maintaining a sense of humor despite having a life-threatening illness. 

    In conclusion, Kathy noted that despite the removal of her vocal cords, with the help of RRP ISA, she has learned that she very much still HAS a voice.

      (3) Bill Stern     RRPF Director    

    “RRP Foundation Priorities and Perspectives 2005”

          Photo    PowerPoint     MP3

    The following outline summary was provided by the RRPF:

    Provide RRP Information; RRP Physician Referral; Networking, Emotional and other Support

    RRP Newsletter (http://www.rrpf.org/newletters/RRP_Newsletter_Spring05.shtml)
    RRPF Listserve (http://health.groups.yahoo.com/group/rrpf/)  – Provides a forum for exchange of information, ideas, opinions and emotions related to RRP. Currently ~350 members consisting of RRP patients, parents, practitioners and researchers (at least 5% are RRP professionals). 

    Identify and Address Major patient/family concerns, i.e.,

    Diagnosis issues
    Coping with RRP
    Treatments - surgical and adjunct
    Voice - preservation, restoration/improvement
    Mortality - pulmonary involvement, malignancy
    Disease transmission 

    RRP Awareness
    Are Pediatricians sufficiently aware of RRP?
    Coordinate with other HPV organizations to promote greater public (and political) awareness of RRP and HPV 

    RRP Epidemiology
    RRPF practitioner and patient databases (currently >750 patients)

    RRP Research
    RRP Foundation encourages and supports promising research related to RRP.  Some of the studies that have received support from the RRPF involve: RRP immunology; Quality of life of RRP patients; Familial genetics and RRP.  Furthermore, the RRPF is interested in additional RRP research including:  therapeutic role for HPV vaccines, studies to better assess mechanisms and understanding of various adjunct treatments and new approaches to the treatment of pulmonary RRP.

     (4) Lotta Gustafsson, Msc, PhD,  “Treatment of Papillomas with Human Alpha-Lactalbumin-Oleic Acid Complex (HAMLET),”  principle investigator for June 24, 2004 New England Journal of Medicine article on HAMLET, Lund University, Sweden.     

          Photo       PowerPoint        MP3 (Dr. Gustafsson hasn't released it for distribution)

    HAMLET is a complex from human milk cells. It can be produced under laboratory conditions but it is extremely expensive and slow to make. It works via programmed cell death (apoptosis) and HAMLET kills different cells via different mechanisms.  Encouraging results were presented from a double-blind study.  HAMLET is applied topically for skin papillomas.  Future studies will consider RRP and lesions on the cervix.  Safety studies are needed before the study can progress. 

     (5) Craig Derkay, M.D.- “Update on RRP Research 2005,” Professor and Vice-Chairman. Eastern Virginia Medical School, Chairman of RRP Task Force, President of American Society Pediatric Otolaryngology (ASPO).

          Photo     PowerPoint      MP3

    Adjunctive therapy update highlights:


    Dr. Derkay reported on studies by Dr. Clark Rosen, Peak Woo and others. There was a wide variability in reported response to cidofovir, ranging from increased dysplasia in a single patient to reports of remission in others.  Cidofovir doesn’t work on everyone and it is not a silver bullet. 

    Concerns have been expressed in the RRP Taskforce about cidofovir’s potential to cause cancer down the road.  It was decided that although cidofovir may be an appropriate treatment to recommend to patients with moderate to severe disease (defined as more than 3 surgical intervention per year), it should only be given with FULL INFORMED CONSENT regarding the carcinogenic potential of the drug and is discouraged for patients needing 3 or less surgeries a year. 

    The 2005 RRP Task Force recommendations on cidofovir are available through this website.


    Cuba Study-169 patients – 85 kids/84 adults.  Frequency of relapse declined. Germans have a multi-center trial underway.  [Ed. Note: Listen to MP3 and see PowerPoints. Type of interferon, dose, schedule, etc. wasn’t elucidated. Data presented--as in the cidofovir reports--can only appreciated by asking meaningful questions about the study context and design.]

    Photo Dynamic Therapy (PDT)  

    Review of study at Long Island Jewish Hospital.  Conclusion: PDT is not an optimal treatment strategy.  

    HspE7 Vaccine

    Proposed phase III trial expected in early 2006.  


    Proposed multi-center trial. [Mark Shikowitz, M.D., Long Island Jewish Medical Center, provided a brief summary that was interjected into the middle of Dr. Pransky’s presentation. The Celebrex dose has been lowered to 400 mg/day due to the cardiac risks.  LIJ believes it to be safe at the lower dose.  Several patients are enrolled, and LIJ is actively recruiting patients for this study. Dr. Shikowitz’s PowerPoint presentation elucidated what is believed to be the mechanism of action of Celebrex on RRP.  You will find his MP3 in the middle of Dr. Pransky’s MP3.  


    A)   Glaxo Smith Kline HPV Vaccine – This vaccine is a prophylactic vaccine which protects new  HPV 16/18 infection.   

    B)    Merck Vaccine –– Phase III ongoing.  This prophylactic vaccine against HPV 6, 11, 16 and 18 prevents HPV infection in humans.  It is expected this vaccine will be on the market very soon.   

    There is evidence that the vaccine not only prevents the recipient of the vaccine from acquiring HPV, but the offspring may also acquire the immunity (rodent studies).

    We don’t know if these prophylactic vaccines might exert a therapeutic influence, since they impact the humoral immunity, not cell-mediated immunity that is thought to be deficient in RRP. However, it still may be possible that this vaccine if used intra-lessionally might stimulate the cellular immunity, in a manner similar to the mumps vaccine.  This is a speculation that will need to be tested after it is FDA approved.


    A)   Review of several reference articles (see slides)

    B)    RRP kids have a lower quality of life

    C)    RRP cell biology and risk factors relating to DNA typing.

    D)   GERD exacerbates RRP.

     (6) Arturo Avila Chavez, M.D., “RRP in Developing Countries,”  International RRP ISA Center board member and associate professor, National Institute of Respiratory Diseases, Mexico City, Mexico. 

          Photo      PowerPoint     MP3

    There are an estimated 1,500 – 2,000 RRP patients in Mexico. The main problems in treatment are cultural, medical and economic.  Voice conditions are not treated seriously and are typically ignored until the patient cannot talk or breath properly.  Due to the economic issues of health care in Mexico, different levels of medical attention are provided.  There are very few centers for specialized medical care.  Typically a doctor will have 80-120 patient visits per day or 10 surgeries per day.  There is a 6-8 month wait for surgery.  Some patients are forced to wait until complete respiratory distress.  Most RRP patients are handled in the public hospitals.  Most doctors don’t have specialized training for handling RRP.  Equipment is lacking.  A potential solution is to utilize the International RRP ISA Center to try to help this situation.  

     (7) Richard  Schlegel, M.D. PhD, “Developing Research into Effects of Atemisinin on HPV.”  Professor and Chair, Department of Pathology, Georgetown University Medical School

          Photo     PowerPoint     MP3

    Dr. Schlegel is working under a Bill and Melinda Gates Foundation grant to study HPV.  In this presentation, he explained his research in using artemisinin to treat RRP. Artemisinin is a Chinese herb used to treat malaria.  It reacts with red blood cells by superoxidizing their iron, which gives birth to free radicals that subsequently kill the malarial parasite.  HPV-infected cells appear to be highly sensitive to artemisinin.  Dr. Schlegel is doing research with dogs that have been artificially infected with RRP in hopes that he can use artemisinin to cure their disease. Artemisinin is not an anti viral. Replace with:  For purposes of the dog study, the artemisinin was administered topically.  Future studies are planned.

     (8) Jennifer Woo, Senior Thesis on RRP,  Harvard University

         Photo     MP3

    Jennifer is a senior at Harvard majoring in Medical Anthropology.  The thesis is entitled. "A VOICE OF THEIR OWN: The Social Experience of Illness within the RRP Community." The thesis preparation has included three months of travel interviewing RRP patients, families, researchers and others. The interviews showed the following trends: 

    A)   The voice is part of an individual’s identity and when compromised, it adversely affects a person’s sense of identity. 

    B)    The need to redefine the definition of disability 

    C)    The need to reduce the stigma of RRP 

    D)   The lingering sense of parental guilt (“How to cope?” and “Disease transmission” being the big ones) 

    E)    Frustration of practitioners 

    F)     Politics of research – and the hostile political environment 

    The thesis will be published in April and excerpts will be in the RRP Foundation newsletter, the International RRP ISA Center website and relevant peer reviewed publications.

     (9) Gregory McKee, PhD,  Stressgen Biotechnologies’ Chief Executive Officer 

          Photo   PowerPoint         MP3

    Dr. McKee presented the story of HspE7 and touched on the conclusions of its phase II study. He noted that Stressgen has clinical proof of the concept and is now working on the manufacturing process.  The 2nd generation of HspE7 looks more promising for RRP.  HspE7 is designed to be a heat shock protein therapeutic vaccine.  That’s a very loopy name for most lay people but Dr. McKee offered a very informative PowerPoint that explained how HspE7 is thought to work. Phase III HspE7 trials are scheduled for early 2006 and will be a controlled pediatric study.

      (10) Farrel Buchinsky, M.D.  “Genetic Susceptibility to RRP: Report on Individual and Family Genetics Research Project”  Pediatric Otolaryngologist, Allegheny General Hospital in Pittsburgh, PA. 

             Photo    PowerPoint    MP3

    Dr. Buchinsky presented his ongoing study and acknowledged that it was supported in part by the International RRP ISA Center.  Huge numbers of the population are exposed to HPV 6/11, but only a few develop RRP.  Genetics is thought to play a role in determining who does and who does not develop RRP.  Dr. Buchinsky’s PowerPoint summarized the genetic theory underlying this study. At the time of the presentation, the study has 82 enrollees and their parent (s).  This study requires a sample of an individual’s saliva as well as that of both parents (or one parent if both aren’t possible). In the event parents aren’t’ available, a sample from a sibling would be beneficial as well.  This study requires a very large data sample.  It is easy and free.  Please consider participating. For more information, see http://www.centerforgenomicsciences.org/RRPGenetic.

    Note: RRP ISA is very grateful to Dr. Buchinsky and Allegheny-Singer for transposing the Focus Session audio tapes to MP3. 

     (11) Seth Pransky, M.D. “Current Issues in the Management of Pediatric RRP,” Otolaryngology Dept., San Diego Children’s Hospital

     Photo     PowerPoint    MP3

     RRP is a variable disease and each patient needs to be approached differently.  It is very important to discern and filter out misinformation.   Surgery involves the debulking of lesions via laser, forceps or microdebrider.  A conservative approach is essential as is protection of the anterior commissure.  Presence of HPV 11 appears to be a predictor of more severe disease than HPV 6.     The microdebrider induces less trauma but has less access to the ventricle and subglottis.  The pulse dye laser does not seems to destroy underlying tissue.  It is not good for bulky lesions.   

    Adjunct Therapies: Reviewed quickly due to coverage earlier in the presentation.  Dr. Mark Shikowitz of Long Island Jewish Medical Center was invited to speak on Celebrex (see the middle part of Dr. Pransky’s MP3 and also see Dr. Shikowitz’s PowerPoint which is embedded in Dr. Pransky’s PowerPoint.

    Cidofovir has a variable success rate based on 7 years of history. This treatment should be reserved for severe cases.   

    Dr. Pransky reminded us that there are some very promising therapies that will be available not too far down the road.  Patients need to choose their interventions with care so as not to inadvertently injure their larynx in the interim.