The human papillomavirus, or HPV, has been a well-recognized risk factor for developing several types of cancers. Initially, it was a known precursor to cancers of the cervix, with certain sub-types of the virus predisposing women to HPV lesions (genital warts) and formation of cancerous growths.
In more recent years, the association of HPV and head and neck cancers, specifically those of the tonsil, larynx (voice box), tongue, and sinus in otherwise healthy under 65-year-old non-smokers and non-drinkers has led to HPV-related head and neck cancers becoming known as ‘baby-boomer’ cancers.
A much lesser known consequence of HPV disease is that the transmission from a pregnant mother who has HPV in the genital tract can lead to HPV disease in her unborn child. Transmission of the virus, even in the absence of visible genital warts, can lead to a rare, yet devastating, condition in children, known as recurrent respiratory papillomatosis, or RRP. These children typically develop symptoms of hoarseness, raspy voice, chronic cough, and severe breathing problems during the toddler years. As the disease is quite rare and not readily identified, duration between symptoms and diagnosis can be several months to over a year. Often by the time the child is diagnosed, he or she is in respiratory distress due to lesions blocking the air passages.
Treatment is usually frequent surgeries involving removal of the HPV lesions, which look similar to genital warts. As the disease is caused by the virus, one surgical treatment is not curative, and children require multiple surgeries throughout childhood, ranging from 2 to 25 per year. Multiple adjuvant therapies have been successful, including injection of anti-viral medications to the lesions and systemic therapies. To date, while many of these children will undergo spontaneous disease remission in adolescence, RRP has no cure.
Caroline Wilcox, now an elementary school teacher, is all too familiar with RRP. She began developing problems with her breathing before her first birthday, and was not diagnosed until four months after her symptoms of hoarseness and gasping for breath began. As with many patients with a new diagnosis of RRP, her first surgery was performed as an emergency, when her airway became severely compromised by lesions blocking her breathing. “The doctor told my mom that my breathing passage was the size of a pencil lead. If I had gotten a cold, I would have suffocated,” said Wilcox. What followed was a childhood of multiple surgeries to remove the lesions: “We lost count after 100 [surgeries],” Wilcox recalls. ” I had surgery every six weeks to six months for most of my childhood.” Needless to say, this affected her family life growing up. Her mother stayed at home caring for her daughter during her early childhood, and would stay at a local hotel near the hospital for several days after each surgery. The financial and emotional burdens were substantial, and Wilcox’s mother developed cervical cancer before the age of 30. Wilcox is currently disease-free, but continues to have some issues with her voice and with her breathing.
While RRP remains a rare disease of childhood, it is one with high morbidity, and is life-altering for a child and his or her family. An under-recognized benefit of the HPV vaccine may be eventual eradication of this disease. Prior to use of the HPV vaccine, a pregnant woman with HPV had up to a 1 in 250 chance of her child developing RRP. Current trials are underway to identify the incidence of HPV-acquired RRP in the setting of use of the HPV vaccine in early adolescence. Dr. Craig Derkay, Professor of Pediatric Otolaryngology at the Eastern Virginia Medical School and Chair of the RRP Task Force, is optimistic. “Frequency [of RRP] is dropping precipitously with uptake of the HPV vaccine.”
A recent Australian study published in the Journal of Infectious Diseases in January 2018 found a significant annual decline of new diagnoses of RRP between 2012 and 2016, after a widespread initiative of administering the HPV vaccine to females ages 12-26 years. Rates of RRP declined from 0.16 cases per 100,000 in 2012 to 0.07 cases per 100,000 in 2016. None of the children of vaccinated mothers developed RRP. While this was a small population, the findings that the HPV vaccine appeared to be effective in blocking maternal transmission of HPV to an unborn fetus is promising, but it must be given prior to sexual activity. Derkay, who was not involved in the study, states that a woman “needs to get vaccinated before she becomes sexually active” in order to prevent risk of transmission of the virus to the unborn fetus. “If she has an actively shedding genital lesion, getting the vaccine during pregnancy would be of no value to the fetus.”
The debate on use of the HPV vaccine in teenagers remains, especially regarding whether or not the vaccine should be mandatory. Concerns about vaccine-related injuries and the notion that administration of the vaccine will lead to sexual promiscuity have been the most notable issues from those objecting to vaccine use. Lesser known benefits of the vaccine, including reduced risk of head and neck cancers, and marked reduction of RRP should be included in discussion of the vaccine’s benefits.