Recurrent Respiratory Papillomatosis: “While We Breathe, We Hope”

Many people know that human papillomavirus (HPV) causes genital warts and certain types of cancer (e.g., cervical, anal, and throat), but they may not know about another condition caused by HPV. Known as recurrent respiratory papillomatosis, or RRP, this rare condition is characterized by the growth of benign (noncancerous) tumors, or papillomas, around the vocal cords as a result of HPV infection. The papillomas cause voice changes and, in some cases, breathing difficulties. People affected by RRP can experience a series of symptoms, including hoarse or raspy voice, noise when breathing, choking, shortness of breath, difficulty swallowing, and snoring. In some cases, affected individuals may experience repeated bouts of pneumonia, and affected infants may be diagnosed with “failure to thrive,” meaning they do not grow at a reasonable rate compared with other infants of the same age.

Because there is currently no cure for RRP, affected individuals often require surgery to remove the papillomas, particularly if they interfere with breathing. Some individuals require frequent surgeries, and in the most severe cases, they may require a tracheostomy. During a tracheostomy, an opening is made in the throat to insert a tube in the windpipe, enabling breathing through the opening rather than through the nose and mouth. This can be a long-term solution for a small number of people with RRP, and because air exchange occurs without passing the vocal cords, it can interfere with speaking.

RRP can affect children and adults, known as juvenile- or adult-onset RRP, respectively. It’s estimated that about 20,000 people worldwide are currently affected by RRP. Because of the relatively small number of affected individuals, families dealing with this condition were often isolated. So, in the early 1990s, two families came together to create the Recurrent Respiratory Papillomatosis Foundation (RRPF). Recently, the Vaccine Education Center team had the opportunity to speak with Kim McClellan, President of RRPF. Keep reading to find out more about RRP and RRPF.

RRPF – “A voice for those with RRP”

When RRPF was formed, there was no centralized location for information, support or research initiatives. Bill and Marlene Stern realized this when their 2-year-old daughter was diagnosed with RRP, and they started trying to learn all they could about the condition. They met Henry and Susan Woo, another couple whose daughter was affected by RRP, and together, they founded the Recurrent Respiratory Papillomatosis Foundation to not only centralize information and support other families, but also to work with researchers and clinicians studying and treating RRP. Dr. Craig Derkay and his RRP Task Force worked with the Stern and Woo families to create RRPF and bring awareness of and research to the issue of RRP. As described by Kim, with the development of RRPF, “the community began to have a voice.”

RRPF – “Working to transform lives”

When asked about the ongoing efforts of RRPF, Kim indicated that RRPF is working “to transform the lives of patients with RRP” through various areas of focus:

• Improve care for patients with RRP
• Advocate for a non-surgical paradigm shift
• Convene clinicians and researchers
• Catalyze new research
• Strengthen RRP community

Kim offered examples of each of these efforts. Let’s take a closer look.

RRPF – Improving care for patients with RRP

Because of the small number of patients globally, RRPF works to provide information to those newly diagnosed as well as to offer support. For example, RRPF has used social media to form support groups that are open to patients, and their caregivers, around the globe. They are also currently working on a patient toolkit that will be available digitally as well as in hard copy. It will launch in the fall of 2024. Their website also helps patients find a doctor in their area who treats RRP since not all clinicians are familiar with the most common clinical approaches, disease progression, and latest treatment options for this rare condition.

RRPF – Advocating for a non-surgical paradigm shift

As described, surgical interventions to remove papillomas or improve breathing have been common approaches in the treatment of RRP. However, new tools are becoming available. First, HPV vaccination has been shown to decrease the occurrence of RRP, so prevention is an important opportunity, especially since we don’t know why some people develop RRP and others do not. Second, antiviral medications have been shown to decrease the ability of the virus to replicate. Finally, monoclonal antibodies have shown some promise. Monoclonal antibody treatments, like bevacizumab, target infected cells. RRPF is currently working on projects to help patients access bevacizumab. They have also partnered with biotech and major pharmaceutical companies to bring the patient voice to potential treatments and industry decisions regarding RRP interventions and research studies.

RRPF – Convening clinicians and researchers

RRPF also works to ensure that healthcare providers are equipped to recognize RRP and that regulators understand patient experiences. For example, in the fall of 2022, RRPF held a listening session for regulators at the Food and Drug Administration (FDA) to share survey data as well as patient stories, such as Eden’s. They have also been supported by the Chan Zuckerberg Initiative’s (CZI’s) “Rare As One Project,” allowing them to host RRP roundtables, including one coming in 2024. 

RRPF – Catalyzing new research

RRPF has assisted with clinical trial recruitment and published clinical trial experiences from the patient perspective. The RRPF website also offers a place for people diagnosed with RRP to add their name to a global patient registry, called the Coordination of Rare Diseases at Sanford, or CoRDS. The RRPF/CoRDS registry gathers this information to support research into a better understanding of the disease. It is free to join, and patients can remove their information at any time. The team at RRPF has also collected data on numerous pulmonary RRP patients to help develop a tissue bank as well as treatment guidelines and screening rubrics. Pulmonary RRP occurs when the virus moves beyond an infection of the throat and upper airways to the lungs. This can lead to the development of nodules in the lungs; persistent cough, including production of blood; recurrent pneumonia and, rarely, cancer. The RRPF website includes information about pulmonary RRP, a rare complication of an already rare condition.

RRPF – Strengthening the RRP community

With support from CZI, RRPF is currently working to develop a patient advisory council. This group will serve as regional contacts for the RRPF, help facilitate local fundraising events, and be speakers or interview contacts, if needed. This group will also serve as a resource for patients and the community.

RRPF – “While We Breathe, We Hope”

By working together, patients, families, clinicians, researchers, and others who care about RRP continue to work toward ending the burden of this condition caused by HPV. As they declare, “While we breathe, we hope.” Find out more about RRP and those working to eliminate it through the efforts of the Recurrent Respiratory Papillomatosis Foundation:

• Follow RRPF on Facebook.
• Sign up for their e-newsletter.
• Donate to support their mission.
• Donate your talents or story by emailing RRPF at info@rrpf.org.
• Check out patient stories, clinical trial opportunities, and frequently asked questions on the RRPF website.
• Read Kim’s interview featured in Katie Couric Media.

If you or someone you know has been affected by RRP and you are not already familiar with RRPF, get in touch with them today! They can answer your questions, provide you with resources, and offer support as you navigate this life-altering condition.