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What is Recurrent Respiratory Papillomatosis?


Recurrent Respiratory Papillomatosis ("RRP") is a rare disease (there are perhaps 20,000 active cases in the U.S.) that is characterized by the growth of tumors (papillomas) in the respiratory tract caused by the Human Papilloma Virus ("HPV").




Where do the symptoms of RRP primarily occur?


Although they primarily occur in the larynx on and around the vocal cords, these growths may spread downward and affect the trachea, bronchi and occasionally the lungs. It is sometimes referred to as Laryngeal Papillomatosis and in the past was often called Juvenile Laryngeal Papillomatosis because it was thought to primarily affect only children.




What are some aspects of the disease?


A distinguishing aspect of this disease is the tendency for the papillomas to recur after surgical procedures to remove them. Hence, the "recurrent" part of the name. The tumors or growths can be wart-like, often have a cauliflower-like appearance, and are either pedunculated (attached only by a slim stalk), or sessile (closely adhering to mucosa).




Who gets RRP? What is JRRP?


RRP occurs in both children and adults. In children, Juvenile Recurrent Respiratory Papillomatosis ("JRRP") is almost always diagnosed by age ten and usually before the age of five, showing no sexual preference. Statistics indicate that first-born children delivered vaginally to young mothers (under the age of 20) with active condyloma (genital warts) during pregnancy, are at greatest risk. However, even among this group, the disease is still rare.




What are the odds my child has JRRP?


The estimates indicate an incidence among children of about 4.3 per 100,000 and among adults of about 1.8 per 100,000.




What are the risk factors for JRRP?


There is considerable evidence that JRRP in children results from a vertical transmission of HPV from mother to child. Virology studies have substantiated the link between genital condylomas (warts) and JRRP. HPV types 6 and 11, which are responsible for 80-90% of the condylomas, are responsible for nearly 100% of JRRP. In a study using data collected by the RRP Foundation, it was found that the number of JRRP patients born via cesarean section was less than 25% of the statistically expected number based on national norms, suggesting that a cesarean birth might play a preventative role for JRRP. Furthermore, as previously noted, it was found that mothers under the age of 20, who have condyloma during pregnancy and who deliver their first born child vaginally, appear to be at greatest risk of infecting their newborn. In adults, RRP is also caused by infection with HPV-6 and HPV-11. However, there does not appear to be a statistically significant relationship with birth factors as is seen in JRRP cases. This probably indicates that for adult onset RRP, the infection is not likely acquired at birth and there is speculation that for many adults it may be sexually transmitted.




Why does JRRP or RRP occur?


Beyond the previously noted risk factors regarding the transmission of HPV, it is still not very well understood why only certain children present with JRRP. It is estimated that approximately 5% of the U.S. population may have HPV in their respiratory tract, but less than 1 in 1000 of those infected ever develop RRP.




What are the symptoms of JRRP?


The most common symptom of JRRP is a voice that is persistently hoarse, weak, low in pitch, breathy, or strained. Often dysphonia (difficulty in speaking) or aphonia (loss of voice) can occur as well. Tumor mass and location (how the growths interfere with normal vocal cord function) may explain the degree of voice quality defects. For lesions that form near the vocal folds, hoarseness can occur very quickly with small lesions.




What happens as the disease progresses?


As the disease progresses, shortness of breath can occur as the airway becomes blocked by bulky lesions. Although this is more common in children, in some situations, RRP can cause breathing difficulties in adults, especially during exercise. Young children often present with a weak cry, chronic cough, swallowing difficulties and stridor (a high-pitched, whistling sound heard while taking a breath). Inspiratory stridor is noted by this noisy breathing or snoring as a child strains during inhalation when sleeping. This is indicative of an upper respiratory obstruction and warrants immediate attention by an otolaryngologist.




Do JRRP or RRP Symptoms progress?


JRRP or RRP related symptoms may develop gradually over months or even years in mild cases, but in very aggressive situations symptoms may emerge in a matter of days.




How is JRRP or RRP diagnosed?


JRRP or RRP is typically diagnosed by an ear, nose and throat ("ENT") physician performing an examination of the larynx. Some physicians may start with a mirror examination, which uses a mirror placed in the back of the throat reflecting light down the throat and onto the vocal folds. More typically, a doctor or a trained speech-language pathologist diagnoses JRRP or RRP via an indirect laryngoscopy in the ENT office.




How is the exam performed?


This involves the placement of a flexible fiberoptic camera through the nose to further visualize the vocal folds in the throat or the use of a straight, rigid camera placed through the mouth that shines down the throat onto the vocal folds. In addition to allowing the ENT to view the larynx, a video recording can also be obtained with these instruments. Some otolaryngologists or speech pathologists may use a videostroboscopy to obtain an even more detailed look. However, to make an absolutely definitive diagnosis of JRRP or RRP, a direct laryngoscopy (usually in conjunction with surgical removal of papilloma growths) must be performed in an operating room ("OR") with the patient under general anesthesia at which time a biopsy is taken and tested for HPV. In some cases, the direct laryngoscopy is the only option; usually this involves young children in distress where instrumenting the airway outside of the operating room might be hazardous. It is most desirable to diagnosis JRRP or RRP before a surgical procedure so as to facilitate family awareness/expectations and so the anesthesiologist, surgeon and OR nurses will be properly prepared.




Is JRRP or RRP misdiagnosed?


Pediatricians who are unfamiliar with this disease often misdiagnose JRRP or RRP. Many times, shortness of breath and stridor are mistakenly assumed to be the result of asthma or croup. The consequences of these errors may be serious as papillomas are at least partially obstructing the airway to cause these symptoms and should be removed immediately to prevent the risk of asphyxiation.




If my child has been diagnosed with JRRP, how do I explain my child’s needs to his/her teachers?


Under the Health Insurance Portability and Accountability Act of 1996 ("HIPPA") there are no legal need to divulge details.




Will my child outgrow JRRP in adolescence?


It is certainly possible, although sometimes RRP continues but reduces in severity and frequency.




How often do we need to see our ENT?


You should speak with your doctor and develop a plan together.




Is this my fault?


No!




Will my child have a voice post-operation?


Yes.




Will my child always make gurgling noises?


No.




Will my child need to be suctioned very frequently (e.g., every 5 minutes)?


No.




Do I have to carry portable suction with me at all times?


Yes, this will likely be recommended by your ENT.




Can I go outside with my child and can they go to school?


Yes. Absolutely.





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