Juvenile-onset recurrent respiratory papillomatosis (JORRP), is an uncommon condition, occurring in approximately 2 per 100,000 children in the United States1 and is diagnosed between birth and adolescence. Approximately 1500 to 2000 new cases occur annually in the United States.3-5 Although rare, JORRP results in substantial childhood morbidity.2
JORRP is of viral etiology caused by HPV 6 and 11 and manifests as recurrent, benign tumors that can occur anywhere along the respiratory tract, but it most commonly involves the laryngeal mucosa, or the area around the vocal chords. The natural history of JORRP is highly variable and unpredictable and can be fatal due to frequent recurrence, fast rate of growth and tendency to recur and spread throughout the respiratory tract (C. S. Derkay & Wiatrak, 2008). The two most common impacts are reduced voice quality (children are hoarse or speak in a whisper) and an airway that can become obstructed by the quick growing tumors. There is no cure for JORRP and for many children management requires surgical treatment every few days to months and use of adjuvant medical therapy
JORRP can have significant physical and emotional suffering for affected patients and their families because of the need for repeated surgical removal of the papillomas from the airway.
To date there is only one study that specifically looks at the impact of JORRP on the quality of life for children and adolescents. Lindman, Lewis, Accort and Wiatrak’s 2005 study the Pediatric Quality of Life Inventory (PedsQL) to compare the health-related quality of life (HRQOL) in children and adolescents, with JORRP with the HRQOLs reported for healthy children and children with other chronic medical conditions and found that children 5 to 18 years of age with JORRP self-reported a significantly (p < .05) worse HRQOL as measured by the PedsQL Total Score, Psychosocial Health score (a combination of Emotional, Social, and School Functioning Scores), and Social Functioning and School Functioning scores than the health control children lower school functioning scores than those for children with other chronic illnesses. (J. P. Lindman et al., 2005).
JORRP is an often overlooked consequence of human papillomavirus (HPV) infection is the transmission of virus from mother to child. Often times the mother is not even aware that she is a carrier for the HPV virus.
Our experience with JORRP
My son has JORRP and was diagnosed at age two. Between his second and fourth birthday he had nineteen surgeries. He was typed as HPV-6 which is usually the most mild strain, but it has proven to be quite aggressive in him. I started by not using any medication on him at all, but after four weeks his airway was the size of a coffee straw. So with a heavy heart we tried cidofovir. After six injections there was little change and the side effects are worrisome enough that I didn’t want to keep him on it. We are now seeing Dr. Hartnick at Mass Eye and Ear Infirmary and using propranalol and avastin to treat the JORRP with promising results.
Does your child have JORRP? Email me! Let’s connect. xo Nora
Web Resources for JORRP and RRP:
Connect to others about JORRP:
Contribute to the body of knowledge:
Clinical Trials:
- Clinical trial studying the treatment of RRP with avastin and the pulsed KTP laser (click here)
for a list of more RRP clinical trials, click here
JORRP related videos:
Refernces Cited
1 W.C. Reeves, S.S. Ruparelia and K.I. Swanson et al., National registry for juvenile-onset recurrent respiratory papillomatosis, Arch Otolaryngol Head Neck Surg129 (2003), pp. 976–982.
2. Armstrong LR, Derkay CS, Reeves WC. Initial results from the National Registry for Juvenile-Onset Recurrent Respiratory Papillomatosis. Arch Otolaryngol Head Neck Surg.1999;125:743-748.
3. Pransy SM, Kang DR. Tumors of the larynx, trachea, and bronchi. In: Bluestone CD, Stool SE, Alper CM, et al, eds. Pediatric Otolaryngology. Vol 2. Philadelphia, Pa: WB Saunders Co; 1996:1402-1414.
4. Gaylis B, Hayden RE. Recurrent respiratory papillomatosis: progression to invasion and malignancy. Am J Otolaryngol. 1991;12:104-112.
 |
|
5. Derkay CS, Malis DJ, Zalzal G, et al. A staging system for assessing severity of disease and response to therapy in recurrent respiratory papillomatosis. Laryngoscope. 1998;108:935-937.
