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RRP ISA Survey Results


    What is RRP?

    What is RRP?

    RRP (Recurrent Respiratory Papillomatosis) is a disease of the respiratory tract caused by the Human Papilloma Virus (HPV).  It causes tumor-like lesions to grow on the larynx and, in some cases, in the trachea and lungs. They invariably cause voice difficulties, including hoarseness and vocal fatigue. They can occasionally convert into cancer. Left untreated, the lesions may grow, causing suffocation and death.

    The incidence of RRP is spread fairly evenly between children and adults.  The lesions often recur, even after repeated surgical excisions. Infants and young children sometimes have to undergo biweekly surgery just to keep their airway open. Some children have undergone many hundreds of surgeries under general anesthesia. 

    RRP is NOT the same thing as vocal cord polyps or nodules. Nodules are often treated with speech therapy and polyps are easily removed, rarely come back, and do not cause long term voice problems once they are removed. RRP can cause years of hoarseness or worse.

    Few people have ever heard of RRP.  Although the HPV virus that causes Recurrent Respiratory Papillomatosis is widespread (the CDC has estimated that tens of millions of people in the United States are infected with HPV), the prevalence of RRP is very low. It has been estimated that there are 10-25,000 people in the United States with this disease. 

    The only HPV subtypes that cause RRP are HPV 6 and 11, and occasionally 16. No other HPV subtypes are known to cause RRP. Gardasil, a prophylactic HPV vaccine released by Merck in June 2006, is said to prevent uninfected people from contracting these viral subtypes. Dr. Keerti Shah, a respected molecular biologist and HPV/RRP expert at Johns Hopkins Medical Center has publicly asserted that Gardasil will, over time, prevent new cases of RRP.

    It is known that children contract the disease from mothers who have genital HPV (can be genital warts or asymptomatic HPV). Babies are infected as they pass through the birth canal. It is therefore imperative that OB/GYN and pediatric physicians be astute in diagnosing and helping to prevent this disease. Unfortunately, however, an infant's first diagnosis of RRP is all-too-frequently given in the emergency room, since he or she can no longer breathe. The ER visit might have been preventable with proper physician early diagnosis and care.  The epidemiology of adult RRP is less well-understood, but theories have been advanced.

       

    Most cervical cancers and about 25% of head and neck cancers are caused by HPV. It is thought that in both cases, the infection appears to follow sexual contact with an infected partner. RRP cannot be called an STD, however, anymore than cervical cancer can. Any speculation that RRP might be an STD, when viewed in this light, is inappropriate.

    One does not catch RRP from someone else who has it. It does not show up as an opportunistic infection even amongst patients diagnosed with advanced AIDS, and AIDS patients often contract HPV infections in other parts of their bodies. This suggests that most people couldn't contract RRP even if they tried. Genetic factors and impaired immune responses at the cellular level of the respiratory tract appear to play a key role in determining who is susceptible to contracting this disease. 

    However challenging the disease is in itself, the story does not end there. Treatment-induced injuries (these injuries are called iatrogenic injuries) caused by over-aggressive surgery may lead to vocal cord scarring.

     

    The arrows point to multiple papilloma growths on the larynx caused by a viral infection.

    (Permission to reproduce photo courtesy of the University of Pittsburg Voice Center).

    While there is no cure for this disease, there are prevention and treatment strategies, some of which appear to work better than others.  Physicians and patients alike need to be aware of the pros and cons of these various strategies. The single most important thing that you can do is to educate yourself.

    The single most important thing
    that you can do is to educate yourself.

    Society sometimes responds to RRP patients with fear, and schools and employers have been known to discriminate against them. The disease may flare up even after a 20-year hiatus, and the effects of RRP can extend over decades. Clinical depression and a sense of profound hopelessness and isolation are not unusual in patients and their families.

    While the human cost of RRP is devastating, the economic cost is staggering. In the United States, the lifetime cost for RRP can run into hundreds of thousands of dollars. Single mothers are especially hard-hit because they have to choose whether to stay home and care for a child with a life-threatening disease or work several jobs just in order to pay their rent and cover their medical bills.  

    In developing countries, treatment is often marginal to non-existent. Untreated, this disease is as deadly as cervical cancer. To say the disease is a heartbreaker is an understatement.