Recurrent respiratory papillomatosis (RRP) remains a devastating disease for pediatric patients. Each time we diagnose a new case of RRP we are reminded of our limitations in battling a disease that has no known cure, that has an unpredictable course, and that carries a risk of morbidity. Answering probing questions from family members about disease transmission requires sensitivity, as the knowledge that it is a vertically transmitted disease often evokes much guilt in parents.
Explore this issue:October 2006
The lack of a cure for this disease cannot be attributed to lack of research efforts. An infectious etiology for RRP was first proven in 1923 when Ullman injected homogenized papillomata from a child’s larynx into his own forearm and developed papillomatous lesions at the site three months later. Clearly, Dr. Ullman was operating prior to the creation of Institutional Review Boards! Numerous investigators have since sought to find a cure for this virally transmitted disease. Countless treatment modalities have been proposed based on promising preliminary results, only to find that the purported benefit was more likely a reflection of disease variability than efficacy of the drug. I recall the wisdom of Professor Bruce Benjamin, who told me during my fellowship that cures for RRP come and go like passing trends. His wisdom has prevailed.
Vaccines: A Great Leap Forward
The FDA approval of Gardasil in June of this year is the most exciting news for otolaryngologists who treat patients with RRP. This quadrivalent vaccine combines empty virus-like particles with the L1 major capsid protein of HPV-6, -11, -16, and 18 to induce a robust host-antibody response. The Phase II and Phase III trials have demonstrated remarkable safety and efficacy in preventing HPV-related cervical dysplasia and carcinoima in situ from the viral subtypes of interest. While the impetus for creating the vaccine has been to prevent anogenital HPV-related disease, the potential for preventing RRP, which is often considered an orphan disease, is clearly our windfall.