Airway Intervention Registry (AIR): Recurrent Respiratory Papillomatosis (RRP)

Study Overview
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Brief Summary
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Recurrent Respiratory Papillomatosis (RRP) causes wart-like growths in the airway which can make it difficult to breathe, speak and carry out normal everyday activities. It is a rare condition affecting all ages, but is more common and aggressive in children than in adults, affecting 4 in every 100,000 children. There is no known cure for RRP, but symptoms are checked through regular hospital visits, with multiple therapies or procedures under general anaesthetic needed to remove or shrink the growths which can grow back quickly. The problem is that nobody knows which therapies or procedures work best. Aim: To identify which RRP treatments currently used in National Health Service (NHS) hospitals within the United Kingdom (UK) are the most effective and safest in the short- and long-term. It will also identify which patients respond best to specific treatments, and those who are at higher risk of experiencing a complication after treatment. Method: Collect information from usual patient care and quality of life questionnaire responses in a secure online database. Participation in this study requires patient/parent/guardian consent. This observational study does not require patients to undergo any additional intervention as part of the research.

Detailed Description
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Primary aim: The overall goal is to improve the care of patients with Recurrent Respiratory Papillomatosis (RRP) - and the investigators aim to do this by determining the most effective and safe RRP treatments currently being used in patients in National Health Service (NHS) hospitals within the United Kingdom (UK) (information which is currently lacking). By determining the most effective treatments of RRP, the investigators will be able to increase the time interval between surgical interventions to maintain symptomatic control, reduce overall number of RRP interventions, severity and spread of papillomas in the airway, hospital visits, medications and ultimately improve quality of life in those suffering from RRP. By also capturing peri- and post-procedural details the investigators will be able to determine the relative safety of treatments and identify those which slow the progression of disease. From the data collected the investigators intend to identify patient subgroups (based on patient characteristics such as age, gender, human papillomavirus (HPV) type, location of papillomas, RRP severity and spread, comorbidities) who respond better to specific treatments, and also identify patient risk factors which contribute to the complication outcomes (such as tracheostomy). Secondary aims: build an evidence base of the different RRP treatments used across the UK which will help to formulate hypotheses for future research in RRP and improve quality of life for RRP patients; inform National Institute for Health and Care Excellence (NICE) interventional procedure guidance on radiofrequency cold ablation (IPG434,2012), which is currently under special arrangements due to lack of safety and efficacy evidence; identify common symptoms or signs associated with RRP disease profile, to aid future diagnosis of RRP; determine the geographical spread of RRP patients across the UK, to inform effective use of future NHS resources and inform the Department of Health strategy in its quadrivalent HPV vaccination programme (protecting against four types of HPV including types 6 and 11 commonly associated with RRP) currently offered to 12-13 year old girls within the NHS childhood vaccination programme; inform future development of national clinical guidance on management of RRP to ensure that everyone receives the best care based on best available current knowledge; determine impact of COVID on RRP patients including changes to RRP management, RRP symptoms. Objectives: The investigators will make use of an existing secure online database platform (the Airway Intervention Registry, AIR - developed and hosted by The Newcastle upon Tyne Hospitals NHS Foundation Trust, NUTH) and its associated infrastructure and develop it to capture additional observational outcomes from standard clinical practice and quality of life questionnaires from RRP patients. All RRP patients (any age) receiving treatment in any UK NHS hospital will be eligible for inclusion. Data collection will be open for 53 months with no minimum follow-up required for patients. Due to its recurrent nature (requiring regular hospital visits to maintain an open airway), following this patient population will allow the investigators to determine both the short- and long-term relative safety and efficacy of RRP treatments used in the UK. Consent will be required (from patients/parent/guardian) before data are entered into the online database.

Official Title
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Airway Intervention Registry (AIR) Extension: Recurrent Respiratory Papillomatosis

Conditions
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Recurrent Respiratory Papillomatosis, Human Papilloma Virus

Intervention / Treatment
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* Procedure: Microdebrider
* Procedure: Cold-steel surgery (forceps)
* Procedure: Carbon dioxide laser
* Procedure: Potassium titanyl phosphate (KTP) laser
* Procedure: Pulsed dye laser
* Procedure: Radiofrequency ablation
* Procedure: Monopolar suction diathermy
* Procedure: Plasma coagulation
* Procedure: Other
* Other: Adjuvant therapies


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: patients of any age (no restrictions) diagnosed with (newly diagnosed or existing) and receiving treatment for respiratory papillomatosis informed assent/consent of data participation provided by patient/parent/guardian Exclusion Criteria: patients participating in other studies
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Intervention/Treatment |
| --- |
|Procedure: Microdebrider|Removal of lesions via microdebrider|
|Procedure: Cold-steel surgery (forceps)|Removal of lesions via forceps|
|Procedure: Carbon dioxide laser|Removal of lesions via carbon dioxide laser|
|Procedure: Potassium titanyl phosphate (KTP) laser|Removal of lesions via KTP laser|
|Procedure: Pulsed dye laser|Removal of lesions via pulsed dye laser|
|Procedure: Radiofrequency ablation|Removal of lesions via radiofrequency ablation|
|Procedure: Monopolar suction diathermy|Removal of lesions via monopolar suction diathermy|
|Procedure: Plasma coagulation|Removal of lesions via plasma coagulation|
|Procedure: Other|Removal of lesions via other methods|
|Other: Adjuvant therapies|All adjuvant therapies|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Time interval between surgical interventions | Time between surgical interventions to maintain symptomatic control | Over study duration (53 months) | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| RRP surgical intervention - nature | Nature of intervention | Over study duration (53 months) | 
| RRP surgical intervention - timing | Timing of intervention | Over study duration (53 months) | 
| RRP adjuvant therapy - nature | Nature of intervention | Over study duration (53 months) | 
| RRP adjuvant therapy - timing | Timing of intervention | Over study duration (53 months) | 
| Histology results | Including HPV type | Over study duration (53 months) | 
| Severity of papillomas | Measured via Derkay severity score (higher score corresponding with higher severity; clinical score range 0-11, anatomical score range 0-75, summed total score range 0-86) | Over study duration (53 months) | 
| Number of hospital visits/attendances | Inpatient, Accident & emergency, Outpatient | Over study duration (53 months) | 
| Voice assessment (paediatric) | Measured via paediatric voice handicap index (higher score corresponding with higher voice handicap, range 0-99) | Over study duration (53 months) | 
| Voice assessment (adult) | Measured via voice handicap index (higher score corresponding with higher voice handicap, range 0-120) | Over study duration (53 months) | 
| Short-term complications | Complications arising in-hospital | During hospital admission for RRP intervention (length of hospital stay can vary between patients e.g. from 0-100 days) | 
| Post-operative discharge location - planned | Planned discharge location (decided pre-operation) | During hospital admission for RRP intervention (length of hospital stay can vary between patients e.g. from 0-100 days) | 
| Post-operative discharge location - actual | Actual discharge location (occurring post-operation) | During hospital admission for RRP intervention (length of hospital stay can vary between patients e.g. from 0-100 days) | 
| Long-term complications | Unexpected hospital, Accident & emergency (A&E), General Practitioner (GP) attendances due to RRP symptoms. | Over study duration (53 months) | 
| Impact of COVID | Test results, antibody results, symptoms, changes to management, | From March 2020 onwards (30 months) | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Recurrent respiratory papillomatosis