Welcome to RRP Africa
RRP Africa is a not-for-profit task group committed to spreading awareness around Recurrent Respiratory Papillomatosis (RRP) in Africa. It was started by paediatric Ear, Nose and Throat (ENT) surgeons in Cape Town, but is intended for anyone interested in advocating for this rare airway disease in children. Currently RRP has no cure, but is easily preventable.
Through advocacy, research, collaboration and information sharing, we aim to improve the diagnosis and reporting of RRP. In doing so, we hope that this will have a synergistic effect on optimising the treatment and care of RRP patients in Africa to avoid recurrent airway obstruction in these children, which can be lethal.
What is RRP?
Recurrent Respiratory Papillomatosis (RRP) is a rare disease that is characterised by the growth of tumours in the respiratory tract caused by the human papilloma virus (HPV).
Although the tumours primarily occur in the larynx on and around the vocal cords, these growths may spread downward and affect the trachea, bronchi, and sometimes the lungs. When this happens, it can affect the patient’s breathing and urgent surgery is needed to relieve the airway obstruction by removing the warty growths.
RRP is sometimes referred to as laryngeal papillomatosis, tracheal papillomatosis, and glottal papillomatosis. In the past, it was thought that RRP developed mainly in children (Juvenile Onset RRP, or JORRP), but adults can also develop the disease (Adult Onset RRP, or AORRP). It is still not known why some people develop this disease, while others do not. What predisposes children to this condition still needs to be studied. Sadly, RRP is a diagnosis for life.
What are the symptoms of RRP?
The most common symptom of RRP is an altered voice quality. A voice that is persistently hoarse, weak, low in pitch, breathy or strained, is typical of RRP, and depending on how the growths affect the vocal cords, this is variable. Sufferers might also experience difficulty in speaking, and loss of voice. Affected children may also experience difficulty speaking, and loss of voice. As the disease progresses, the airway can become blocked by bulky papillomas, and can be associated with shortness of breath.
Young children may have a weak cry, swallowing difficulties, chronic cough, and stridor (noisy breathing). Inspiratory stridor is indicated by a high-pitched whistle or snore as a child strains during inhalation, usually when they sleep. Adults may also experience breathing difficulties, especially during exercise or on exertion.
In places with poor access to health care, children may present with progressive obstruction after having a hoarse voice for a while. They may even be misdiagnosed as having asthma or recurrent croup. It is thought that in resource-poor settings, children may die from airway obstruction without ever being diagnosed, and almost certainly, that the true burden of the disease is under appreciated.
How does RRP affect one’s quality of life?
Treatment for RRP has not changed for more than half a century. The standard treatment option is to debulk the airway through repetitive surgical removal of the papillomas that obstruct the lumen. Due to the nature of the disease, these surgeries are repetitive and can be traumatic, resulting in permanent damage to the vocal cords and remaining airway. Further research has gone into the use of adjunct, non-FDA approved medication, which is often inaccessible and expensive in poorly resourced settings. Furthermore, some therapies cannot be stopped, as the papillomas recur, which means that patients need to remain on the medication for long periods of time.
In many developing countries, the standard treatment option is often a tracheotomy due to a lack of resources by the family or medical community. To understand the full impact of this disease, we will be sharing some patient stories in the next few months.
Why is addressing RRP important?
RRP is often a confusing disease to diagnose and as a result, it is either misdiagnosed or goes untreated. This problem is exacerbated in developing countries that have poor medical resources and an even poorer repository of patient data. This makes it almost impossible to quantify the extent of the disease in so many low- to middle-income (LMIC) countries.
As with all conditions, early and appropriate treatment is always preferable in order to stabilise disease progression, and ultimately a) increase the rates of remission, b) reduce the need for surgeries, and c) improve patient comfort. Ensuring that effective drugs for adjuvant therapy are available throughout Africa will greatly help with this.
Most importantly, and perhaps the real solution in preventing the next generation of RRP sufferers, is ensuring that HPV vaccination is widely adopted throughout Africa. This will be the most effect way to help prevent the disease.
How can you help?
Pledge your support today!
RRP Africa is working towards facilitating greater support for RRP patients and the clinicians managing them; research into non-surgical treatments for RRP; and, ultimately, disease prevention across the continent. We hope that, one day, RRP will be a thing of the past. HPV vaccination with Gardasil 4 or 9, which covers the appropriate virus strains (type 6 and 11), is available, but not currently accessible to many LMIC countries across the world, and this should be addressed. To help us work towards a global solution of preventing the next generation of RRP sufferers, you can pledge your support. By adding your name to our list of supporters to promote universal HPV vaccination with Gardasil 4 or 9, you will be adding your voice to our cause. It’s a simple act, but a powerful one.