Often misunderstood and mistaken for other diseases, hormone-secreting tumours can wreak havoc on your life.
ALLEN Wilson, 50, had complained of general malaise, anxiety, and night and day sweats to his doctor, who insisted he was perfectly healthy, despite him perspiring profusely on a cool day in Ireland.
In 2006, his symptoms worsened and he was prescribed beta-blockers for high blood pressure.
Looking back, Wilson now knows he had been suffering from pheochromocytoma (pheo for short) symptoms for more than 12 years.
A second opinion finally concurred and he was admitted to the Royal Victoria Hospital in October that year.
Interestingly, after conducting his own research on the Internet in 2003, he had asked his physician whether it could be pheo. The doctor had laughed it off and said the disease was so rare, it almost didn’t exist, and dismissed him as being overly anxious.
As it turned out, he had three tumours, one on his adrenal gland, and two smaller tumours on the lower lobe of the left lung.
Wilson underwent surgery to remove the tumours in March 2007. It took a team of general surgeons and another team of vascular surgeons 11 hours to remove a very aggressive 12cm tumour.
The surgery was almost aborted on three occasions due to the aggressive adrenal response from the tumour, but thankfully, it was successful.
Two months later, the smaller tumours in his lung were removed. However, he was told his “cancer” had metastasised and that he would be dead before Christmas.
He spent the next 24 hours making peace with his Maker and family, only to be told that pheo didn’t progress the same way as other cancers. He was later informed that he was tumour-free and would make a complete recovery.
Then in October 2008, a 3cm liver tumour was discovered right next to his inferior vena cava (a major vein leading from the lower body to the heart). He was started on alpha blockade again, and scheduled for a major liver section in late January 2009.
Wilson contacted the NIH (National Institute of Health) in Bethesda, United States, to inquire about the possibility of (then) experimental radio frequency ablation (RFA) to treat his tumour.
He headed to the NIH, and in May 2009, underwent an embolisation of the artery feeding the tumour prior to the actual RFA.
He has been tumour-free to this day. His current diagnosis is “sporadic metastatic paraganglioma”, and goes for regular check-ups to monitor hormonal developments.
His unfortunate experience with pheo spurred him to hook up with Amy Pitzer, Matthew Capogreco and Laura Becktel, leading to the setting up of Pheo Para Troopers (para being short for parangliomas), a US-based non-profit, patient-run organisation.
None of them knew each other before this. Wilson, 50, is chairman of a few small low carbon chemical companies in Belfast, Ireland; Becktel, 41, was a software developer (who lost the battle and passed away in 2011); Pitzer, an automotive engineer in her 40s; and Capogreco, an educator, also in his 40s.
“We realised that we understood patient needs best and could promote appropriate patient care, research and best treatment,” says Wilson, now president of Pheo Para Troopers, in an e-mail interview.
Formed after the second pheo/para patient conference in Parsippany, New Jersey, in 2008, the organisation aims to help facilitate patient communication and supply important information such as links to special events, studies and current research developments.
In the long term, hopefully, the collecting and analysing of data from patients will contribute towards finding a cure for pheo and paraganglioma.
Currently, there are about 500 members (and growing steadily), and more than 800 Facebook members, stretching across different continents, with the majority from the United States, and various other countries such as Japan, Australia, India, Ireland, Britain and Malaysia.
Pheo Para Troopers aspires to reach out to more and more countries, and globalise the awareness of this condition.
“We are well on our way to achieving this via local physicians and language translations of our newsletter. There’s the website, the drop zone newsletter published in three languages and a Facebook support page (https://www.facebook.com/groups/pheoparasupportgroup/),” Wilson adds.
The organisation operates online, and its raison d’être is advocacy and information-sharing.
“This is a rare disease that is badly and disjointedly treated throughout the world. Our objectives are to spread knowledge of the disease and offer patients a forum and resource guidance on best medical practice and diagnosis.
“It’s surprising how many physicians approach treatment badly, especially in less developed areas of the world. Even in first world countries, some still practice kidney removal or open abdominal surgery, which is no longer necessary in the majority of cases,” he says.
Pheo Para Troopers also hopes to provide a directory of experienced physicians worldwide, thereby ensuring that patients can identify the most qualified and best physicians with good knowledge of best practice.
“We are in the final stages of creating a common database of patients for better data research. The intention is to recruit international research centres into this one database to look for statistically significant trends within the variants of this disease.
“Most researchers don’t have access to enough patients to achieve statistical significance in their work, so hopefully, this facility will empower and enable better, faster and more co-operative international research and treatments,” he adds.
“What’s important is that you should always take an active interest in your own treatment. This is a complex condition, so wherever possible, only allow experts with demonstrable experience to treat you,” concludes Wilson.
While there is no known cure or prevention for paraganglioma and pheo yet, hopefully, with the ongoing dialogue and medical research, progress will someday make this a disorder that can be conquered beyond invasive surgery.
Other avenues of information support include the Pheo Para Alliance, a physician-oriented organisation (http://www.pheo-para-alliance.org/), and the Pheochromocytoma Support Board on Facebook.