There are a number of challenges when you have a chronic illness. I think these are common to many conditions. Questions about your identity, dealing with uncertainty, denial vs acceptance, hope vs despair, sharing vs secrecy and isolation vs connection. As human beings we are social creatures, but living with a rare disease can make you feel isolated. You feel that no one understands and you are the only one going through it. Add to this not being able to talk to people going through the same experience, and it increases the feelings of being shut off from the world.
I didn’t fully appreciate my voice until I lost it. Meeting friends in public places means that no one can hear you talking. Asking for things in shops is a battle to be heard. Most jobs require talking. My job requires quite a lot, so losing my voice was life changing. To be frequently told you sound rough, asked if you are sick, or be given suggestions to improve your voice is also wearing… If only honey and lemon would improve things.
I was diagnosed with recurrent respiratory papillomatosis (RRP) by an ENT surgeon after having had a hoarse voice for three months which I had put down to a virus or my asthma medications. I have to date had 15 surgeries in three years and they may never stop. That is the nature of a condition that has ‘recurrent’ in its title.
What is RRP?
RRP is a condition where non-cancerous tumours caused by the human papilloma virus (HPV) grow in the respiratory tract. The most commonly affected area is the larynx (voice box). The disease can also spread into the trachea and restrict or block the airway. In rare cases it can invade the lungs, become cancerous and be fatal. There are two forms: juvenile and adult onset. RRP is estimated to affect 4 in 100,000 as children and 1.8 in 100,000 adults. The disease varies in severity with some achieving remission for periods, whilst others endure hundreds of procedures over the years. The impact of voice loss, breathing difficulties, repeated procedures in addition to the unpredictable nature mean this disease comes with a huge physical and emotional burden for patients and their families.
Repeated surgery is the main treatment and several different medicines have been developed, but are not universally effective. There are also side effects from repeated surgeries, such as scar tissue, which even if the disease goes into remission can still cause problems with your voice and breathing.
The importance of information, support and advocacy
When I was diagnosed I took to the internet to try to understand what the disease was, and what it would likely mean. There is a lot of information about some illnesses but I initially struggled to find much information about those living with RRP. I eventually found a wonderfully supportive Facebook group for people and families who deal with RRP. People who understand the ups and downs of this disease and support each other through these times. This has been invaluable for feeling less alone, and in finding out information about treatments and new research. In turn this has supported me in advocating for myself.
As a rare disease there has been limited research but we are beginning to benefit from innovations in new cancer treatments, and a small group of doctors who have an interest in making our lives better. I am lucky to have a medical team who are prepared to investigate, listen and seek others’ input and advice. Developments such as the HPV vaccine can potentially eliminate RRP, in addition to other diseases caused by HPV. The hope is that research will bring about new treatments as well as a cure. In the UK a database has been developed for RRP, funded by the National Institute for Health Research, to share information on patient experience, research and information. This will hopefully help others in the future to receive the best possible care through sharing practices and latest research. I have participated in the 100,000 Genomes Project which is an exciting new area with huge potential. There is now real hope that there will be a medical treatment for this disease rather than the ordeal of a lifetime of repeated surgeries.
I still hope to return to a voice that is more than a whisper but writing allows me to ‘talk’ when I can’t be heard.
You can read more about Kay’s experiences by visiting her blog ‘Voice Loss Diaries’