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    FAQ

    In this section, patients, researchers and health care professionals will find frequently asked questions (FAQs) pertaining to RRP. 


    What is RRP?

    1. What is RRP?
    2. How is RRP diagnosed?

    Demographics

    1. Who gets RRP?
    2. Where can I find demographic data on RRP?

    Epidemiology

    1. Is RRP contagious?
    2. I might have genital warts. Is there a risk to my unborn baby?
    3. Is RRP a Sexually Transmitted Disease (STD)?
    4. Can RRP patients transmit HPV to uninfected sexual partners?
    5. I am expectant and I have genital warts. My OB-GYN refuses to do a C-section, describing the risk of RRP as impossibly low. What should I do?

    Living With RRP

    1. What course does RRP generally take?
    2. Should I read the research literature?
    3. Why does RRP recur?
    4. What can I do if I feel discouraged and depressed?
    5. How can RRP patients and their families get in touch with one another?
    6. Is there anything that is known to make RRP worse?
    7. Does it help to humidify and/or filter the air in my home?
    8. I have bumps on the back of the tongue and visible bumps in the throat. Does RRP start out with these symptoms?
    9. Is HPV typing important?
    10. How do I find out what type of HPV I have?
    11. What is high risk HPV?
    12. How do patients pay for all these surgeries?
    13. Where can I find information about travel assistance?
    14. What Is Informed Consent?
    15. What triggers RRP initially?
    16. Is it OK to give blood if you have RRP?
    17. I have RRP and smoked for about 10 years. Does that have an impact on the recurrence?
    18. Does a Tracheostomy Carry Special Pulmonary Risks for RRP Patients?
    19. What is the cost of RRP?

    What are RRP's Treatment Strategies?

    1. Is there a cure?
    2. Should surgery be aggressive or conservative?
    3. Should I be concerned about anesthesia during RRP surgery?
    4. What should I look for or avoid in choosing a RRP physician?
    5. What about speech therapy?
    6. How long does it take to heal after surgery?
    7. How do I Know If I Have RRP?
    8. How do I find a physician to treat RRP?
    9. Is Cidofovir Risky?
    10. What About the Cruciferous Vegetable Protocol?
    11. What About Alternative Therapies?
    12. How important is video Stroboscopy?

    Advocacy

    1. Why is Advocacy an integral part of RRP ISA’s [Information, Support and Advocacy] name?
    2. What is RRP position on Gardasil (Merck’s new preventative HPV vaccine)?
    3. What can I do if my insurance won’t pay for the treatment that my physician has recommended?

    RRP ISA?

    1. What does RRP ISA mean?

    Other

    1. What does the larynx look like? Anatomy? Physiology?

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    What is RRP?

    1.) What is RRP?

     

    Large larynx with arrows

    The arrows point to multiple papilloma growths on the larynx caused by a viral infection. Permission to reproduce photo courtesy of the University of Pittsburgh Voice Center.

    RRP (Recurrent Respiratory Papillomatosis) is a disease of the respiratory tract caused by the Human Papilloma Virus (HPV). HPV 16 has been found in 1-2% of RRP patients, but the two most common HPV subtypes are HPV 6 and 11, the same viral subsets that cause genital warts. In a small subset (prevalence is approximately 10-50 people out of a million) of susceptible individuals, HPV causes tumor like lesions to grow on the larynx and, in some cases, in the trachea and lungs. Left untreated, these tumors can cause suffocation and death. The lesions tend to recur, even after repeated surgical excisions. Some individuals--even young children--have undergone hundreds of surgeries.

    For some other photographs of vocal cord pathology, including of adult and juvenile Recurrent Respiratory Papillomatosis, click on the Airway Atlas. These photos show the extent to which the airway may be compromised.

     

    2.) How is RRP diagnosed?

    By observation and finally by biopsy, Observation MUST be in a physician's office or in the operating room, where the papillomas often present in a recognizable form. Note, however, that a biopsy (at least initially) is necessary to confirm the diagnosis.

     

    Demographics

    1.) Who gets RRP?

    The incidence of Recurrent Respiratory Papillomatosis is spread fairly evenly between children and adults. Physicians who treat Recurrent Respiratory Papillomatosis estimate that between .005-.01% of people in the United States have this disease.  The low estimate is 10 patients in a million (Steinberg). Other estimates range between 5,000-25,000 people. The Center for Disease Control (CDC) had for several years maintained a registry of patients of children (up to age 17) with Recurrent Respiratory Papillomatosis, but it is now defunct. The CDC has never created a similar registry for adult RRP patients.

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    2.) Where can I find demographic data on RRP?

    No one knows the exact demographics of RRP case distribution. Our organization is trying to make a difference in that regard. See RRP ISA Survey Results for data on several hundred RRP patients.

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    Epidemiology

     1.) Is RRP contagious?

     Not casually, and not even through intimate contact. You cannot catch RRP from another person who has it. There are a number of letters from RRP physicians and researchers corroborating this statement. Physicians who work in major treatment centers for Recurrent Respiratory Papillomatosis agree that most people are not susceptible to catching the HPV virus in their respiratory tract. It simply doesn't get a foothold, nor can it.  Children do not catch Recurrent Respiratory Papillomatosis by playing with a child who has it or by sharing utensils. Siblings do not catch it from their brother or sister. Spouses who are sexually intimate do not contract it from one another. If people living with advanced AIDS do not catch Recurrent Respiratory Papillomatosis, we may feel confident that it is not contagious in any normal sense, casual or intimate. As it stands, few AIDS treatment centers have ever even heard of RRP.

    Having said all this, two exceptions must be made: (1) Neonates who pass through the birth canal of a mother infected with genital warts can become infected with Recurrent Respiratory Papillomatosis. [A CDC study showed that the incidence is very low, however, and most neonates of infected mothers will not be infected.] There is also evidence that an infant doesn't have to pass through the birth canal to become infected, but that this transmission takes place in the uterus and is either hematogenous or transplacental (Abramson). (2) Also, some physicians and researchers (Broker, Kashima, Steinberg) acknowledge that some adults may risk infection from engaging in oral-genital sex with a person who has genital HPV. The subset of adults who are thought to be able to catch RRP in this way must be very low, otherwise, there would be many tens of millions of people in the United States with Recurrent Respiratory Papillomatosis.  It is thought that a very small subset of sexually active adults simply do not have sufficient locally-mediated immunity to fight off this disease if they are exposed to HPV during oral sex. 

    Two cautions are therefore in order:  Mothers with genital HPV should inform themselves concerning the risks and may want to opt for a C-section in order to minimize risk that their baby might be infected with Recurrent Respiratory Papillomatosis, bearing in mind that this may not be preventative. Expectant mothers should consider being tested for genital HPV (the DNA test is, unfortunately, not very reliable). At the very least, they should have regular pap smears.  The question of risk has been addressed in a paper called "Risk Factors for Juvenile Onset Recurrent Respiratory Papillomatosis," by  K. Shah, W. Stern, F. Shah, D. Bishai and H. Kashima, published in the Pediatric Infectious Disease Journal (1998).  (2) While the vast majority of people cannot catch RRP from unprotected oral sex  with a person who has genital HPV, it may be risky for some people. These individuals may wish to consider using a protective latex barrier when having oral sex with any partner. (Note that in most instances, genital HPV infections cannot be ruled out without extremely sophisticated laboratory tests that are out-of-reach for most people, and even then false negatives can occur.

    Note that new cases of RRP may now be prevented if early vaccination occurs in the mother, prior to her getting HPV. The HPV subtypes that cause RRP are mostly HPV 6 and 11, and rarely HPV 16. Merck’s HPV vaccine Gardasil, which was released in June 2006, is expected to prevent uninfected people from contracting HPV 6, 11, 16 and 18. Dr. Keerti Shah, a molecular biologist and HPV expert at Johns Hopkins has publicly asserted that Gardasil will, over time, prevent new cases of RRP.

    Finally, you should know that HPV presence (6, 11 and even 16) is mostly subclinical and that pap smears and genital HPV testing mostly "light up" in the presence of clinically active disease. Subclinical infections are, however, a significant vector for JOORP, and while testing is helpful, it often misses the subclinical form. For this reason, we say that a negative finding does not rule out subclinical infection.

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    2.) I might have genital warts. Is there a risk to my unborn baby?

    Yes. Such a risk does exist. See the FAQ "Is RRP Contagious.”

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    3.) Is RRP a Sexually Transmitted Disease (STD)?

    It is universally acknowledged in the HPV/RRP research and treatment communities that juvenile onset RRP arises following a susceptible baby’s passage through the birth canal of a mother who is infected with HPV 6, 11 or 16 (Shah). Since these subtypes do not grow elsewhere on the skin, and since these HPV subtypes don’t come down from the sky, there are two possibilities that might explain how the larynx of an adult could become infected: (1) Adult onset RRP arose either from a latent juvenile-onset infection that was quiescent and somehow later became activated; or (2) It arose in a susceptible host as a result of oral sexual contact with an infected partner or through oral contact with an object that had touched the genitalia of an infected person. This is what has been propounded by HPV/RRP experts such as Drs. Bettie Steinberg, Keerti Shah, Haskins Kashima, Tom Broker and others. No other epidemiological possibilities have been propounded.

    Thus we have it: RRP did not come down from the sky. It arises from contact of some sort with an area of the body (i.e., the anogenital region) affected by these viral subtypes. So is RRP a STD? The answer is that it's an STD only if most cervical cancer and 25% of all head and neck cancer (e.g., cancer of the tonsils), both of which are caused by the same HPV subtypes, could also be called STDs. If your doctor doesn’t consider them to be STDs, and we’re pretty sure s/he won’t, then it is both meaningless and misleading to call RRP a STD. This isn’t because the virus that causes RRP and the majority of cervical cancer isn’t sexually transmitted. It is. But to suggest that RRP is a STD carries certain connotations. If the implicit connotations to the term STD do not apply in the case of cervical and tonsilar cancer, they do not apply to RRP.

    Let’s look at the connotations that are generally attached to the term STD. Anyone can catch syphilis . Anyone can catch herpes. Anyone can catch genital HPV. All of these are universally acknowledged as STDs. RRP, however, could not be contracted by most newborns or adults even if they tried. Genetically susceptibility seems to play a critical role.

    Consider the fact that tens of millions of people have genital HPV. According to one RRP researcher who looked at the sexual practices of adult RRP patients and compared them with people who do not have RRP, almost everyone who is sexually active engages in oral sex. Yet the incidence of RRP is only about ten people out of a million (Steinberg). Consider the fact that there is an epidemic of anogenital warts amongst people living with AIDS, and these individual also engage in oral sex. Yet RRP is so rare amongst this population that most AIDS treatment centers have never heard of it.

    For this reason—because RRP falls so far outside the usual connotations of the term—RRP can no more be considered an STD than cervical cancer.

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    4.) Can RRP patients transmit HPV to uninfected sexual partners?

    Answer by:

    Bettie M. Steinberg, Ph.D.
    Chief, Division of Otolaryngology Research LIJ Medical Center
    Associate Director and Chief Scientific Officer Institute for Medical Research at North Shore - LIJ

    As most of you know, RRP is caused by one or more of the human papillomaviruses (HPVs). This is a large family of related viruses (more than 150 and still being discovered) that cause papillomas or warts on skin or mucous membranes. About 30 types infect mucous membranes like the mouth, throat or genital tract. Only two types usually cause RRP, types 6 and 11. These are the same types that cause "bumpy" (the scientific term is "exophytic" ) genital warts or condylomas in women, while many of the other HPV types (including HPV 16 that can rarely lead to cervical cancer) cause flat condylomas that are not easily seen. For some unknown reason, HPVs usually cause flat warts on genital tissues of men, so they are usually not detected. In the throats of both men and women (and kids), HPV 6/11 can cause papillomas than can range from rather flat "carpet" types to large "grape-like clusters" that can block the airway.

    It is important when you talk about these diseases to make it clear to people that HPV is the virus, while RRP and genital condylomas are diseases caused by the virus, since the virus can be present without any disease (latent infection). The skin-type HPVs are present in everyone without any symptoms. Genital HPVs cause obvious disease in about 20% of women at some time (it usually goes away after a few months or years) but infect nearly 100% of adult women and men at some time with no symptoms, and usually remain as latent or silent infections. HPVs are present in the throat of at least 5-10% of the population with no symptoms. About 3 people out of 100,000 get RRP. You can see by the numbers that most HPV infections don't cause disease. It apparently takes just the right combination of many different genes, maybe in combination with some other trigger like another virus infection or throat irritation, to be likely to develop RRP.

    Now to the whole question of transmission. Lets start with genital tract HPV infections, and initial infection of the airway. In the genital tract, HPVs are transmitted from one person to another by sexual contact, so genital warts are classified as STDs. This does not mean that sexual contact is the only way they are transmitted, just the usual way. Since the virus is shed from the surface of genital warts in reasonably large numbers, any type of physical contact can transmit the virus. Most doctors and scientists believe that the most likely way for a baby to get an HPV infection in the airway is when genital tract HPV is transferred from the mother to baby during birth, although there is some evidence that it can occasionally occur before birth. Since many genital tract infections are flat warts that are not visible, most mothers don't know they have the infection and you cannot go back later to look for it, since the active infection usually goes away with time . So, this data is based on strong epidemiology studies that show a link. We don't know how HPV infection in adult onset RRP is acquired. One possibility is transmission from the genital tract of the sexual partner by oral sex. Another possibility is that the virus has been present in the throat since birth, but something now "triggered" it to become active. Either way, no blame should be put on anyone. Since most people carry this virus and have active genital tract disease at some time or another without knowing it, exposure is very common.

    Transmission from someone who has RRP is a completely different question. We know that the disease is not generally contagious, because it is very rare to see two cases in the same family where contact is frequent. We also know that many RRP papillomas don't produce any virus at all, and the others don't make very much. Very different from genital infections, and probably reflects biochemical differences in the two types of tissues. From the virus' point of view, infecting the throat is a loser - little or no production of new virus. For now we have to go with the knowledge we have to determine whether there is any real risk of infecting someone else:

    1. No evidence that RRP is contagious within a family

    2. Very little HPV made by papillomas in the throat

    3. Many people already have HPV in their throat, with no symptoms - not right combination of genes?

    4. Nearly everyone who has been sexually active has already had a genital tract HPV infection.

    Therefore, the chance that you will transmit HPV to someone's throat by kissing and they will get RRP is essentially zero. Transmitting it to the genital tract of a partner by oral sex is probably close to zero, and the partner is most probably already carrying the virus. Bottom line - I would not worry about it unless your partner is heavily immunosuppressed due to drugs for something like an organ transplant. In that case, you might not want to take the risk, but even then, your partner probably already has a genital tract infection and people who are immunosuppressed shed more HPV from genital warts, so your partner [ed., who has no RRP] would be more contagious than you are. . . ." Bettie Steinberg, PhD)

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    5.) I am expectant and I have genital warts. My OB-GYN refuses to do a C-section, describing the risk of RRP as impossibly low. What should I do?

    Dr. Keerti Shah, who is an HPV/RRP researcher at Johns Hopkins, writes: 

    "... We have estimated that the risk of transmission of JORRP from a condylomatous [ed. note: has warts] mother to an infant may be 1-3%, and could be as high as 8% for first-born children of teenage mothers (Bishai et al., unpublished data). .." Dr. Shah also published on the risk issue in Shah KV, Kashima H. "Prevention of juvenile-onset recurrent respiratory papillomas," in Curr Opinion Otolaryngol Head Neck Surg 1997;5:107-11.

    Another paper by Dr. Shah calculates that only approximately one in 400 exposed babies will have the disease. He writes: “There are risks of c-section deliveries. . . . Therefore there is no cut and dried answer to the question ‘Should a woman with genital HPV infection deliver by c-section?’ Each woman must discuss things with her doctor and then decide." 

    The difference between one in 400 and 3 in one hundred (3%) is significant. The difference between one in 400 and 8 in 100 (8%) is even higher. From Dr. Shah’s presentation, it can be inferred that this difference is based on the presence of genital warts (condylomas). 

    If you decide to remain with your current physician, you might want to ask him or her to write you a notarized letter indicating that if your child ever develops RRP, she will assume full liability and will pay for all medical costs associated with this disease in perpetuity.

    RRP ISA has received permission to post the following response by Tom Broker, PhD. Dr. Broker is the president of the International Papillomavirus Society. Like Drs. Steinberg and Shah, he specializes in HPV/RRP. Dr. Broker writes:

    "I share your considerable concern in this case. Obviously I am not able to dispense medical advice. But I do make a distinction between a “history of genital warts” without current symptoms, and an active, particularly florid case. Virus load and dose of exposure do matter with many infections. Absent other qualifiers, C-section seems more advisable and, not the least, because of the breech position. I have a sense that birthing difficulties including post-natal intubation are other, real risks. I do agree that some infections could well initiate in utero (as a small number of C-section babies do go on to RRP), but why run the added risk? In any event, one needs to hope that the immune system of the baby is well matched to reject the HPV. I would add that breast feeding would be prudent.

    "I agree whole-heartedly with you that the OB/Gyn seems very short-sighted or misinformed about the risks and potential consequences.”

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    Living with RRP

    1.) What course does RRP generally take?

    The disease usually first attacks the larynx. In adults, the initial symptom is hoarseness. In young children, there may also be stridor. [If you have a child who has difficulty breathing when sleeping, see a physician right away. One footnote: A number of obstetricians and  pediatricians have misdiagnosed this disease. Physicians treating young children need to educate themselves about Recurrent Respiratory Papillomatosis. Failure to properly diagnose this disease could lead to obstruction of the airway.]

    In most persons, the disease will stay localized to the larynx. It can also infect the trachea. In most instances, when it does this, it does so as a result of some significant tracheal trauma which causes epithelial changes that facilitate Recurrent Respiratory Papillomatosis growth in the trachea. It is important to avoid tracheotomy procedures in RRP patients since this seems to lead to growth of papillomas in the trachea.

    Some patients can require surgery every couple of weeks just to keep their airway open. Others might require surgery once a year or less. After several years, there may be a period of remission--or not. Remissions sometimes last for years. RRP behavior in this regard is still not well-understood. The variables that determine who goes into remission and why, or how aggressive the disease will be, are still largely unknown.

    If the disease enters the trachea, there is increased danger that it will enter the lungs. If it enters the lungs, the disease is even more difficult to treat and control. When is it localized to the larynx, quality of life may be severely compromised. When if affects the lungs, it is life-threatening.

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    2.) Should I read the research literature?

    Absolutely, but you shouldn't believe everything you read, especially abstracts. Researchers sometimes fail to maintain accurate data and/or overstate their case. 

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    3.) Why does RRP recur?

    Many cases of RRP do not recur. It's a once-only event, and it never comes back. But for many other patients, RRP tends to recur even after repeated surgery. Some individuals--even young children--have undergone hundreds of surgeries, though most do not. People whose disease has been dormant for twenty years have been reported to suddenly have a flare-up for no apparent reason. The exact reasons why Recurrent Respiratory Papillomatosis recurs when it does are unknown. Hormonal cycles (high estrogen in pregnancy, for example) may play a role for some people, but it is unlikely that this explains the whole picture. More research is needed.

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    4.) What can I do if I feel discouraged and depressed?

    Take heart. The larynx is very resilient. Patients have had several surgeries in which they were very hoarse afterwards, only to find that on the next surgery, the hoarseness suddenly resolves.  

    It is normal to feel grief and discouragement after going through what seems like an endless cycle of surgeries. It doesn't matter whether you're the patient or the family. As the years pass, many people become clinically depressed. 

    Depression is also often masked and manifests in many other ways besides "the blues." Because depression frequently does not appear as "the blues," it is easy to slip into denial, and the underlying problem is ignored. It continues to manifest, however, in sleep and eating disorders, in problems with concentration and attention, or in irritability and in low energy. The problem here is that it also affects the immune system and other bodily functions. This in turn deepens the depression. Most of this happens unconsciously but a vicious cycle continues to operate just below the surface. 

    RRP can swallow people. If you believe that you might be suffering from depression, you should find a good counselor or therapist with whom to talk things over. You may not choose to use medication, but at least make an effort to find a professional with whom you feel comfortable and in whom you have confidence. The important thing is to give yourself a chance to break out of the depressive cycle. We all need a little help from time to time.  Don't let pride or a sense of shame stand in the way. People have an ability to respond to life's problems in different ways. Remember that despair and cynicism aren't inevitable byproducts of sustained adversity. Judicious use of antidepressants is sometimes the fastest and most effective treatment strategy.  They do carry side-effects, however, and it usually takes a few weeks or months of trial and error before an optimum solution is found that is uniquely suited to the individual patient.

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    5.) How can RRP patients and their families get in touch with one another?

    Families and patients who are already isolated by the physically debilitating nature of RRP are sometimes marginalized within their own communities. It is essential that people who have been touched by this cruel disease find strategies that build a sense of empowerment/self-esteem and neutralize the sense of isolation. RRP ISA has several message boards in its archives and we also encourage patients and professionals to join the RRP Support Tribe. This is a powerful interactive environment through which patients, families and health care professionals may communicate with one another.

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    6.) Is there anything that is known to make RRP worse?

    Radiation treatment is known to sometime cause Recurrent Respiratory Papillomatosis to become malignant. 

    Our database also shows that about 30% of all RRP patients stated that voice abuse has caused an recurrence/exacerbation and that about 20% of all patients stated that irritant fumes caused recurrence/exacerbation. The "voice abuse effect" is well-known to many doctors, but the "irritant fume effect" is not.

    See http://foodconsumer.org/7777/8888/M_edicare_54/021603382008_
    Formaldehyde_and_Cancer_Questions_and_Answers.shtml.

    Some physicians and researchers (Rosen, et al) have stated that RRP is exquisitely sensitive to chemical exposure events [includes irritant fumes], often moving the disease from quiescent to acute. Some RRP researchers (Steinberg) have elaborated, testifying that aldehydes and any number of other irritants can exacerbate RRP, including formaldehyde off-gassing from carpets and other building supplies, paints, etc. It can also include smoke from fires. The chief of the University of Washington Otolaryngology Department (Weymuller) said that while it is impossible to develop a comprehensive list of ALL the fumes that may be irritating, the best indicator of what is noxious are those fumes which irritate a patient on a repeated basis. Several physicians (Cox) have hypothesized that irritant fumes appear to disturb the delicate homeostasis of the already-infected tissues. This is known to happen with other viruses such as herpes, where a disturbance in homeostasis can cause the virus to flare up.

    Pregnancy and times of great hormonal change (puberty) can sometimes, but not always, cause latent RRP to flare up.

    Patients are urged to be careful about using systemic corticosteroids like prednisone. Some physicians and researchers (Steinberg) have expressed concern about the possibility they might exacerbate the underlying viral disease, stating that they should only be used to relieve severe and unremitting inflammation and their use should be time-limited.

    Other researchers (Zeligs) have expressed strong concerns about pesticides. Pesticides often behave very much the way estrogen behaves, and because estrogen metabolism seems to be important in Recurrent Respiratory Papillomatosis, they may have deleterious effects on the course of the disease process.

    GERD (acid reflux) even at subclinical levels is known to exacerbate RRP (Koufman).  Additionally, in treating GERD, patients should be careful about using Cimetidine/Tagamet since it has been reported that this drug causes an INCREASE in 16 hydroxyestrone levels, which is what adjunctive treatments such as I3C and DIM are trying to reduce.

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    7.) Does it help to humidify and/or filter the air in my home?

    It is known (Steinberg, Rosen) that RRP is very sensitive to irritant fumes. Having one or more air cleaners (non-ionizing) makes sense. Your environment should not be too dry or too moist, and a humidifier and a good humidity gauge can help in this regard. This can be a room model, as many patients have, or one servicing a larger area.

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    8.) I have bumps on the back of the tongue and visible bumps in the throat. Does RRP start out with these symptoms?

    We have not heard of anyone presenting with RRP who had these symptoms. There are visible bumps (called papilla) on the back of your that are supposed to be there.

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    9.) Is HPV typing important?

    Dr. Bettie Steinberg believes this need for this is vastly overrated. Dr. Tom Broker disagrees. There is no consensus on this question. HPV 11 and 16 tend to be much more aggressive than HPV 6 (Derkay, Wiatrak). The incidence of malignant conversion, which we must remind readers is quite rare in any event, also seems to be higher with these types. It is particularly concerning in the case of RRP that is caused by HPV 16. HPV 16 shows up in from 1-2% of RRP samples, so we are talking about small numbers, and only a portion of those go on to convert. It seems fairly obvious that one might with to subject those cases that are known to have a higher incidence of malignant conversion to more frequent monitoring.

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    10.) How do I find out what type of HPV I have?

    You need to have the tissue tested. Most pathology departments are only equipped to determine “risk factor,” which means “high risk” (16/18) or low risk (6/11). Many patients believe they have both 6 and 11 but the test is really saying it’s one or the other or both. In order to find out exactly what subtype you have, another test needs to be done, and most pathology departments cannot run this test.

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    11.) What is high risk HPV?

    In order to find out the HPV type that your son has, a sample of the papilloma would have to be tested. Generally, RRP patients have HPV types 6 & 11, and more rarely type 16 (1-2%). Type 16 is usually very rare. Although 18 is mentioned in some of the literature, no HPV researcher that RRP ISA talked to has ever seen type 18 RRP.

    Usually, children exhibit a more aggressive disease & adult on-set patients exhibit non-aggressive disease, but this is not always the case. While I do not have a definite definition of aggression, some view aggressive cases as requiring 4 or more surgeries per year.

    Tom Broker, PhD presented data at the last RRP meeting confirming that HPV 11 tends to be more aggressive than 6.

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    12.) How do patients pay for all these surgeries?

    Our database shows that this can be a problem for some patients, especially those who lack insurance. RRP ISA endeavors to addres some of this unmet need and to provide ample support and advocacy for those patients who require it.

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    13.) Where can I find information about travel assistance?

    There is material on the Resource page that addresses this question.

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    14.) What Is Informed Consent?

    It means that a patient or guardian is FULLY informed regarding risks/benefits before consenting to treatment. Patients need to sign off on the Consent for Treatment (Informed Consent) form prior to surgical procedures. 

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    15.) What triggers RRP initially?

    Answer by:

    Bettie M. Steinberg, Ph.D.

    Chief, Division of Otolaryngology Research LIJ Medical Center

    Associate Director and Chief Scientific Officer Institute for Medical Research at North Shore - LIJ

    “To all of you who wonder what triggered your RRP to start with, doctors and scientists who work with this disease cannot say for any individual patient. However, there are several things we do know:

    1. Many people carry HPV in their throat without any disease. We call this virus latency. At least 5% of the people have latent virus, while 10 people in a million have RRP. Big difference! Obviously, most of the time the virus causes no problem. 

    2. Latent papillomavirus can be activated by irritation or trauma. In studies we have done in a model system, even minor irritation (like the UV exposure you get from one afternoon at the beach to give you a "pink" skin, or gently rubbing the skin with an emery board daily) can activate skin-specific papillomavirus. We think the same thing can happen in the throat. So - exposure to harsh fumes, bad coughing, a virus infection like the flu, or anything else [ed. note: other irritants] can probably activate the virus. Now the question is: why don't 5% of the people have RRP, since we all have things happen occasionally (like getting a cold) that could activate HPV.

    3. We suspect, although we haven't proven it yet, that most people's immune system clears any activated infection as soon as it happens. For some reason, people with RRP are not able to have a good immune response to HPV even though they respond to most every other infection. This is one of the problems we are studying right now. Hopefully, we will get answers that will allow us to develop ways to stimulate an effective response. Our long term goal is to stop this disease, even though it will put me out of business! I have given 20 years of my life to studying RRP, and I would love to have nothing more to work on. 

    “Meanwhile, my advice to all of you is to keep your spirits up, talk to each other and hope and believe that the future will be bright.”

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    16.) Is it OK to give blood if you have RRP?

    The virus that causes genital HPV and RRP reside in the tissues of the specific bodily areas that have been infected by the virus, but it does NOT show up in the blood or the lymphatic tissue. Nor does it circulate in the body as free virus. If you are taking a systemic drug like interferon for your RRP, that very well may pose problems for a blood donor recipient, and people taking this drug should not give blood while they are being treated. NO ONE taking Accutane (isotretinoin) should give blood. DIM could be expected to have a negligible effect.

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    17.) I have RRP and smoked for about 10 years. Does that have an impact on the recurrence?

    Yes! If you are still smoking, stop!

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    18.) Does a Tracheostomy Carry Special Pulmonary Risks for RRP Patients?

    A tracheostomy changes the cells in the trachea, significantly increasing the chance of further RRP-events. While there is widespread agreement amongst RRP experts that tracheostomies pose a risk, they are sometimes necessary to preserve the airway. Yes, they are to be avoided. No, they are not the kiss of death. We know of no DATA showing that a tracheostomy induces the SPREAD of papilloma down to the lungs. It doesn't change the cells in the lungs. What it does is change the cells in the lining of the trachea. This raises the chance of RRP appearing in the trachea. Period. It is possible that bronchial and lung involvement could arise from some sort of "downward spread" from the trachea, but we know of no study suggesting that it takes place through that mechanism. Risk of seeding can be mitigated by careful and attentive surgical techniques. 

    While there is widespread agreement amongst RRP experts that tracheostomies pose a risk, if they are necessary to preserve the airway, they are necessary. We have known a number of RRP patients with tracheostomies (our data shows that 11% of 349 respondents report having been given a tracheostomy) with no lung involvement. The data does not suggest that most of those people went on to have lung involvement.

    Yes, tracheostomies are to be avoided. No, they are not the kiss of death.

    We know of no DATA showing that a tracheostomy induces the SPREAD of papilloma down to the lungs. It doesn't change the cells in the lungs. What it does is change the cells in the lining of the trachea. This raises the chance of RRP appearing in the trachea. Period.

    It is possible that bronchial and lung involvement could arise from some sort of "downward spread" from the trachea, but we know of no study confirming that it takes place through that mechanism as opposed to "seeding." Risk of seeding can be mitigated by careful and attentive surgical techniques.

    Informed consent is good but fear is not. We'd be happy to chat with you by phone if you write us using the contact form. 

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    19) What is the cost of RRP?

    Assumptions and Conclusions:

    Let us assume that the "average adult-onset patient" in the United States has a mild to moderate disease process requiring 40 surgeries over the course of his or her working career, and let us also assume that the average cost of surgery is somewhere around $7,000 [allowing for inflation, that is a VERY low estimate]. The total surgical cost will therefore be around $280,000 ($7,000 X 40).

    Then there's the lost time from work, etc., figured at 40 surgeries X 10+ days per surgical event=400+ days of work. Many surgeries will require more, few will require less, so this time loss expense could easily top $60,000 (includes benefits), depending on the patient's income level, and that does not factor in the inflation index over time. It also fails to include time loss that may result from long-term or temporary disability.

    Then there are the non-surgical interventions (adjunctive treatments, speech therapy, psychotherapy, antidepressants, ancillary medical treatment, etc.) which may exceed $60,000 over the course of a lifetime.

    Thus it is that an average per adult patient cost of $400,000 might be incurred. This figure may be conservative.

    Now, it is estimated that there are between 5,000 to 25,000 RRP cases in the United States. If there are 7,500 adults with Recurrent Respiratory Papillomatosis (a very conservative estimate), this suggests a total cost of about $3 billion ($400,000 X 7,500), assuming that every adult patient got what he or she needed in the way of care. [Note: Not everyone gets this, of course, but this estimate is probably still valid when one allows for cost sharing.]

    Assuming that there are another 7,500 cases of juvenile-onset RRP, which usually requires many more surgeries and which often persist into adulthood, this $4 billion figure is probably quite low.

    If that is true, the total lifetime cost of 15,000 RRP cases (adult and children combined) may exceed $6 billion (=3 + 3 billion).

    We can't even begin to imagine how this disease impacts healthcare providers, patients and families in third world countries.

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    Treatment Strategies

    1.) Is there a cure?

    There is no cure for this disease. There are treatment strategies, some of which appear to work better than others.  The primary author of this website, speaking for RRP ISA, believes that one of the most promising treatments--which as yet hasn't been studied systematically except in a handful of patients--uses BOTH Gardasil and artemisinin, as explained here.  Physicians and patients alike need to be aware of the pros and cons of all the various treatment strategies. As stated elsewhere on our website, the single most important thing that you can do if your family has been touched by RRP is to educate yourself.

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    2.) Should surgery be aggressive or conservative?

    During the first few surgeries, some physicians (Morrison) think that an aggressive approach makes sense. The goal is to excise as much of the infected tissue as possible, while leaving the laryngeal function intact. The intent is to get rid of the virus once and for all, if at all possible. But once the disease has recurred several times, it is likely that the viral DNA has already infected much of the surrounding healthy tissue, including (Abramson) the trachea (RRP normally doesn't express in the trachea, but these researchers claim to have found that it is invariably present. It is therefore impossible to talk about excising all infected tissue. Virtually every cell in the area is a potential "virus factory" even if it appears healthy.  At this point, a conservative surgical strategy makes more sense.

    Aggressive use of the laser is very tempting to some physicians. This was especially true in the early 80s, when it was the latest surgical toy and many physicians regarded it as something that, with enough powr, might cure RRP. Both patients and physicians, however, need to be mindful that one of the cardinal rules of medicine is "Do No Harm." An overly-aggressive approach in no way retards the underlying disease process and it can easily burn and scar healthy tissue.  Many surgeons havebacked away from the laser for this reason, but it has its place in treating RRP. It is usually better in certain areas of the vocal cords than the microdebrider. Most physicians avoid operating on both sides of the anterior commissure (the place where the vocal cords meet) at the same time because it can result in scar formation (webbing). 

    Surgeons who are extremely aggressive have no greater rate of success than those who are more gentle and conservative. On the contrary, aggressive surgery leads to more wound-healing. Since RRP tends to grow faster during periods of wound-healing (Broker), an aggressive approach can paradoxically lead to greater RRP growth than more conservative surgery.

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    3.) Should I be concerned about anesthesia during RRP surgery?

    RRP surgery requires close coordination between surgeon and anesthesiologist. Patients are encouraged to talk with their anesthesiologist and ask questions.

    There are three distinct approaches to anesthesia for Recurrent Respiratory Papillomatosis. (1) Through a "venturi jet-ventilation device," the anesthesiologist blows air into the patient's lung at periodic intervals. This device does not block the airway with an inflatable balloon. The advantage is that the surgeon has an unobstructed view of the airway and the larynx. The disadvantage is that if the vapor plume is inadvertently sucked into the lungs, the lungs can be "seeded" with papilloma virus. This, obviously, is to be avoided. (2) The anesthesiologist inflates an endotracheal cuff (balloon) in the airway through which an endotracheal tube extends. The patient breaths and exhales through the tube. The advantage is that the balloon will block the vapor plume from ever getting to the lungs. The disadvantage is (a) that the balloon may bruise the airway, which might inadvertently facilitate the growth of more papillomas; and (b) the balloon sometimes can get in the way and obstruct access to the sub-glottic area. (3) The anesthesiologist uses neither the venturi-jet nor the "cuff."  Instead, the surgeon works on the patient in-between period of breathing. This is called the apneic (or modified apneic) technique. These periods can be separated by intervals of several minutes, or longer. The advantage is the same as in the jet ventilation method. Also, it is not as likely to result in the inadvertent blowing of the vapor plume into the patient's lungs. And additionally, there is less risk that there will be too little or too much air blown into the lungs via the jet ventilation technique. The disadvantages are that (a) the vapor plume is not blocked by a endotracheal balloon and could conceivably still enter the patient's lungs, though there is significantly less risk that with venturi jet-ventilation; (b) because the patient is not breathing for several minutes at a time, if blood oxygen levels are not monitored properly, there is the possibility of increased stress to the heart. Modern techniques for monitoring carbon dioxide in the blood make this modified apneic approach relatively safe for experience anesthesiologists.

    There is no agreement amongst physicians on which approach is best.There are claims and counterclaims. 

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    4.) What should I look for or avoid in choosing a RRP physician?

    RRP surgery requires close coordination between surgeon and anesthesiologist. Patients are encouraged to talk with their anesthesiologist and ask questions.

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    5.) What about speech therapy?

    RRP patients with voice problems often carry a lot of tension in their necks and in the muscles used to control the vocal cords. This is because they learned to compensate so that they might be better understood. A voice therapist can help the patient learn to relax some of these chronic, unconscious tensions, which may make it easier to talk, especially if damage has occurred from over-aggressive surgery. But voice therapists can’t reverse scarring nor can they do anything that will significantly reduce the chances of the RRP recurring. Evaluation of vocal cord function is important and in this regard, video-stroboscopy equipment is essential.

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    6.) How long does it take to heal after surgery?

    Healing time is variable, but anything longer than 3-4 weeks may point to the possibility of a greater-than-average surgically-induced trauma.  That said, the larynx is very resilient, so do not get discouraged. While some doctors encourage their patients to talk immediately after surgery, other physicians think that it is unwise for patients to try to talk too soon. If, after 7-10 days, it's still hard to talk without sounding raspy and effortful, then patients should consider the possibility of NOT to try to talk. Surgery is always hard, and afterwards, one needs to treat one's vocal cords with respect and lots of TLC. Any patient who fails to respect the feedback that his or her vocal cords are giving is asking for trouble.

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    7.) How Do I Know If I Have RRP?

    Papillomas come in all shapes and sizes. An experienced physician can often make an excellent preliminary diagnosis with an indirect laryngoscopy (light and mirror) but a definitive diagnosis requires a biopsy. It is also possible to subject the excised tissue to further tests in order to determine the genetic HPV subtype.

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    8.) How do I find a physician to treat RRP?

    The question we get from patients has to do with finding a doctor to treat their RRP. Our survey shows this is a very big deal for patients. It is one thing for a support group to refer patients to doctors who have declared an "interest" in RRP  (RRP ISA absolutely does NOT make this its criteria for referral), but that's a very different standard from competence in treating RRP. By asking patients to rate their doctors, RRP ISA is, for the first time, collecting customer satisfaction information that can be used to make referrals. On balance, we consider this of potentially major benefit to the RRP community. There really is no intrusiveness, since personal responses (1) are confidential and (2) since patients can always choose to opt out from the rating process (one of the choices is that no rating be assigned).

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    9.) Is Cidofovir Risky?

    There is much that we do not know about regarding cidofovir's long-term side-effects. It does cause cancer in some rodents and Gilead, which makes Vistide, lists it as highly carcinogenic based on those studies. Should we therefore regard this as a problem? No one knows, but a lot of cautious voices have been sounded, since cidofovir usually has to be given multiple times for it to work. The RRP Taskforce indicated that baseline recurrence rates should be well-known and only patients requiring 3 or more surgeries a year, and who have not been able to lower their recurrence rate through other treatments, should be given cidofovir.

    There are other first line treatment strategies that are potentially less risky. Phytosorb-DIM work on a number of RRP patients, sometimes reducing the need for surgery from every few weeks to every few years or even less.

    There are new drugs against RRP/HPV just around the corner. Our "job" as patients should be to try our best to stay out of trouble until these new drugs are released. We have reason to be hopeful that medicine will make a quantum leap in the very near future in terms of treating this disease.

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    10.) What About the “Cruciferous Vegetable Protocol?” 

    This treatment approach was instituted about 12 years ago at Long Island Jewish Medical Center. Patients were told to juice up to a pound of cabbage a day in hopes of getting clinically efficacious amounts of Indole-3-Carbinol (same mechanism of action as DIM, as explained in the Treatment Strategies Page). Unfortunately, only a small percentage of cabbage contains the necessary I3C levels to produce clinical effects, and there is no easy way to tell which head of cabbage does and which doesn’t. Eating cruciferous vegetables is good in that they provide sulforathanes and other beneficial compounds, but to use them for I3C is inappropriate since pharmaceutical grade I3C and DIM are readily available. Long Island Jewish Medical Center stopped its cruciferous vegetable protocol years ago. 

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    11.) What About Alternative Therapies?

    When a diagnosis of Recurrent Respiratory Papillomatosis is made, some very predictable things occur. There's usually an extended period of confliucting emotions, including attitudes that cycle between shock, denial, bargaining, anger and grief. 

    People usually also go through a period where they look for an "alternative" treatment strategy. There is a common misconception that taking something to boost the serum immune system (blood/lymph) will help RRP. It is important to know that most RRP researchers currently believe that RRP results not from a failure in the serum immune system but from a failure at the level of the local immune system (immunity of the respiratory tissues themselves). Taking echinacea, which boosts the serum immune system, probably will not help RRP. 

    Alternative therapies range the gamut, but who can argue with good nutrition, a clean environment (exposure to pesticides and chemicals/radiation are known to increase the chance of malignant conversions), a healthy mind and a healthy body? Stress is known to be bad for many diseases. There are “alternative therapies” that work well to reduce stress, including acupuncture, yoga, meditation, skilled chiropractic interventions, tai chi, etc. One should not expect these approaches to cure RRP, but they may be helpful in “toning and healing” the “energy body,” which in turn—like getting good sleep and good nutrition—may help the body heal itself.

    For more, see Novel Therapies.

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