Summary about Disease
Recurrent Respiratory Papillomatosis (RRP) is a rare disease characterized by the growth of benign tumors (papillomas) in the respiratory tract. These growths most commonly occur in the larynx (voice box) but can also affect other areas, including the trachea, bronchi, and even the lungs. The papillomas are caused by the human papillomavirus (HPV), most often types 6 and 11. The "recurrent" aspect of the disease refers to the tendency for the papillomas to grow back after treatment, often requiring multiple procedures to manage the condition. RRP can cause significant airway obstruction, leading to breathing difficulties and voice changes.
Symptoms
Symptoms of RRP vary depending on the location and size of the papillomas, but common signs include:
Hoarseness: A raspy or strained voice is often the first and most noticeable symptom.
Weak cry (in infants): Changes in the sound or strength of an infant's cry.
Difficulty breathing (stridor): A high-pitched whistling sound during breathing, particularly when inhaling.
Chronic cough: A persistent cough that doesn't go away.
Voice changes: Alterations in the pitch, tone, or quality of the voice.
Noisy breathing: Audible sounds during breathing, even without stridor.
Airway obstruction: In severe cases, difficulty breathing or even life-threatening blockage of the airway.
Dysphagia: Difficulty swallowing
Causes
RRP is caused by infection with the human papillomavirus (HPV), primarily types 6 and 11. The virus is typically transmitted from mother to child during vaginal childbirth (juvenile-onset RRP). Adult-onset RRP can be acquired through sexual contact or other means, though the exact mode of transmission is not always clear. Once infected, the virus can remain dormant for a period before causing papilloma growth.
Medicine Used
There is no cure for RRP, and treatment focuses on managing the papillomas and preventing airway obstruction. While surgery to remove the papillomas remains the primary approach, other medications and treatment approaches can be used:
Surgical Removal: The most common treatment involves surgical removal of the papillomas, often using techniques like microlaryngoscopy, laser surgery (KTP, CO2), or microdebriders.
Cidofovir: An antiviral medication that can be injected into the papillomas to slow their growth.
Bevacizumab: An anti-angiogenic medication that may reduce the growth of papillomas by inhibiting blood vessel formation.
Interferon: An immune-modulating drug that has been used in some cases to help control papilloma growth.
MVA-HPV Vaccine: Investigational preventative vaccine to prevent papillomas from recurring.
Is Communicable
Yes, RRP is communicable. In juvenile-onset RRP, the HPV infection is typically transmitted from mother to child during vaginal childbirth. In adult-onset RRP, the transmission routes can be less clear, but sexual contact is considered a potential mode of transmission.
Precautions
Cesarean section: In pregnant women with genital HPV, a Cesarean section may be considered to reduce the risk of transmission to the infant, although this is not always effective.
Vaccination: HPV vaccination can help prevent HPV infection, particularly in sexually active individuals. Although the vaccine does not treat existing RRP, it can help prevent new HPV infections.
Safe sex practices: Practicing safe sex can help prevent the spread of HPV.
Avoidance of sharing personal items: Avoid sharing items that may come into contact with the virus, such as razors or towels.
Regular checkups: Regular medical checkups can help detect and manage the disease early.
How long does an outbreak last?
RRP is a chronic condition, and there isn't a typical "outbreak" duration. Papillomas tend to regrow after treatment. The time between surgical procedures can vary widely, from weeks to months or even years, depending on the individual and the severity of the disease. The course of the illness is highly variable.
How is it diagnosed?
RRP is diagnosed through a combination of:
Laryngoscopy: A procedure where a doctor uses a flexible or rigid scope with a light and camera to visualize the larynx and vocal cords.
Biopsy: If papillomas are seen during laryngoscopy, a small tissue sample (biopsy) is taken and sent to a lab for microscopic examination to confirm the diagnosis and identify the HPV type.
Physical exam and medical history: The doctor will inquire about symptoms and medical history to assess the possibility of RRP.
Timeline of Symptoms
The timeline of symptoms can vary widely:
Juvenile-onset RRP: Symptoms may appear within a few weeks to a few years after birth.
Adult-onset RRP: Symptoms may develop gradually over months or years. The initial symptom is often hoarseness, followed by other symptoms like breathing difficulties and voice changes as the papillomas grow. The recurrence of symptoms after treatment can occur at any time, depending on the individual and the severity of the disease.
Important Considerations
Airway obstruction: RRP can cause life-threatening airway obstruction, so prompt diagnosis and treatment are essential.
Voice quality: The disease and its treatment can affect voice quality, which can impact communication and quality of life.
Psychological impact: RRP can have a significant psychological impact due to the chronic nature of the disease, the need for repeated procedures, and the potential effects on voice and breathing.
Malignant transformation: In rare cases, RRP can progress to squamous cell carcinoma, a type of cancer. Regular monitoring is essential to detect any signs of malignancy.
Multidisciplinary approach: Management of RRP often requires a multidisciplinary approach involving otolaryngologists (ENT doctors), pulmonologists, speech therapists, and other specialists.