Vol.1 No.2 An RRP Support group Publication 1992 Summer

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From the Editor

Our first issue of the RRP Newsletter was created after our daughter, Lindsay, was diagnosed with Juvenile Laryngeal Papillomatosis(JLP). Its purpose was to seek out others in similar situations so as to share our experiences. The continued expansion of our RRP support network remains a major theme of this publication. In this second issue and subsequent ones, we plan to broaden the focus beyond our own experiences to help provide information about RRP. This will include a section covering activities the Support group has been involved in, case histories of individual patients, RRP developments (i.e. treatments and research), plus any additional information that might help further understanding and/or awareness with regard to this disease. Some other ideas are to include a question and answer section and to occasionally include an up to date bibliography of relevant publications. Please respond with any additional information, questions or comments which you have or would like to share with others via future newsletters. Your feedback will be very helpful in improving this publication.

Bill Stern

Please reply to:

Bill and Marlene Stern
50 Wesleyan Drive
Hamilton Square, NJ. 08690

Home Phone: 609-890-0502

Bill at Work: 609-452-6545
e-mail (internet):
wfs@gfdl.gov

Marlene at Work: 609-258-2751
e-mail (bitnet):
mstern@lindsay.princeton.edu

Activities

Aug. 15-16 Craft Boutique at Marlene and Bill Stern’s house (20%-40% of sales donated to the RRP support group)

Sep. 13-17 Exhibit booth at the National Convention of the American Academy of Otolaryngologists - Head and Neck Surgeons. In Washington, DC.

Oct. 10 Dinner Dance at Neil’s New Yorker Restaurant in Denville, NJ (Donation $30, papilloma kids are free)

Oct. 25 Children's concert at the Arts Council of Princeton, Princeton, NJ

Support Network News

Our national support network has grown to approximately 20 laryngeal papilloma families with whom we have made contact and a few more who we know of but have not been able to contact as yet. So far there are families from 11 states, with the current ages of patients ranging from 9 months to 35 years (11 females and 9 males). Average frequency of surgeries for those who have had this disease for at least a decade ranges from about once a year to nearly twenty per year.

We have received questionnaires from about half of the families. A slightly revised questionnaire has been enclosed for those of you who haven't replied or those who would like to update their original responses. Please take a few minutes to fill them out and return them to Marlene and Bill Stern. Our ultimate objective is to have a national registry of all patients with RRP. With rough estimates of from 2000 to 4000 patients nationwide, we still have a long way to go, so if you know of any others with this disease let them know that this network exists and how to become part of it.

RRP Patient Update

In each issue, with the permission of the family, we hope to be able to present a case history of an individual patient with recurrent respiratory papillomatosis.

Linda and Richard Daudelin gave us permission to write about their son Ricky for this issue.

Ricky Daudelin was diagnosed with RRP in May of 1990 at the age of 16 months. Initially, CO2 Laser surgery was performed at approximately a six week interval, at the Strong Memorial Hospital in Rochester, NY, by Dr. A. Hengerer. The criteria they used to determine when surgery was necessary was when Ricky’s breathing would start to become distressed. Over the following 15 months the disease became progressively more aggressive requiring surgeries every four weeks then three weeks, two weeks and finally every week. The disease had spread into Ricky’s trachea and his breathing in general had become worse. In order to control the growths and improve his breathing a tracheotomy was performed in August 1991. A special diverted tracheotomy was implemented which separates the larynx from the trachea. It is hoped that this would help to prevent further spread of the disease into the trachea, although it does not allow Ricky to speak. In this regard, Ricky has learned sign language and communicates quite well. By providing a new airway, the tracheotomy allowed Ricky to go until early November at which time the standard laser surgery was performed and he was "loaded" with papilloma. Ricky’s case had also been referred to Long Island Jewish Hospital (LIJ) as a candidate for Photo Dynamic Therapy (PDT). Dr. A. Abramson performed the PDT on Ricky in November shortly after the standard laser procedure. Light sensitivity (sunlight and fluorescent) is a side effect of this procedure, in Ricky’s case it lasted about 8 weeks. His next procedure was back at Strong in February of 1992. Although the re-growth of papillomas was less aggressive than before the PDT, a significant amount was found and another PDT followed at LIJ shortly. (This time the light sensitivity lasted somewhat longer, through the end of April.) In the months since the last PDT, Linda and Richard feel that overall Ricky has improved. He has just recently (July 92) returned to Strong for another procedure and although a significant amount of re-growth of papillomas has occurred (but not below the position of the tracheotomy), it has been five months since he was operated on last.

In just a bit over two years RRP has put Ricky and his parents through more stress than many people have to deal with in a lifetime. But Linda and Richard Daudelin say that Ricky is a happy and active child who has a terrific spirit, and his spirit is what keeps them optimistic about the future.

RRP Research News

Any new research developments or studies that we become aware of will be included in our newsletters. In this issue we feature the on-going human papilloma virus (HPV) research by the husband and wife team of Dr. Thomas Broker and Dr. Louise Chow. Dr. Broker also serves as research advisor to the RRP support group. The following article was written by Dr. Broker for this newsletter.

The molecular and cellular oncology laboratory of Tom Broker and Louise Chow at the University of Rochester School of Medicine Biochemistry Department and Cancer Center, has been investigating the fundamental nature of human papillomaviruses since 1980, focusing on their genetic organization, expression and regulation, on their productive infection program in human epithelium and on the mechanisms of pathogenesis in genital and oral warts, dysplasias and carcinomas. The graduate students and post-doctoral and medical fellow in the research group have succeeded, for the first time with any papillomavirus, in reproducing virus particles of HPV type-11 (the primary virus associated with juvenile laryngeal papillomatosis) in stratified, differentiating epithelial cell cultures. In addition, infected cells present in biopsies of laryngeal papillomas and genital condylomas can be grown in similar, differentiating culture conditions with the maintenance of viral gene expression. The next objectives are to expand the population of infected cells from patients, determine the events responsible for rapid growth of papillomas, then use this knowledge to develop therapeutic drugs and treatment protocols for suppression of the virus during wound healing after surgery. Attainment of these goals should allow customization of treatment protocols for individual patients, as well as a much more comprehensive understanding of the viral infection cycle.

Beginning November 1, 1992, Thomas R. Broker, PhD and Louise T. Chow, PhD, will be Professors of Biochemistry and Oncology, in the Biochemistry Department of The University of Alabama - Birmingham School of Medicine and Dentistry, Birmingham, Alabama 35294-0005.

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The support group for Recurrent Respiratory Papillomatosis is pleased to publish this issue of the RRP Newsletter. We hope that this and future issues will serve to enhance the support network among laryngeal papilloma families and also help to provide accurate medical and scientific information about this disease.

Although this newsletter is not intended as a solicitation of donations, we would be grateful for any help in offsetting postage and printing costs. Thanks to all those who have contributed.

Write:

RRP Foundation
c/o Bill Stern
50 Wesleyan Drive
Hamilton, NJ 08690.