Call for bet­ter aware­ness of rare Neuro-En­docrine Tu­mours

Neu­roen­docrine tu­mours (NETs) are still widely un­known but rel­a­tively com­mon

The Irish Times - Tuesday - Health - - Health Cancer - Sylvia Thomp­son

It was the type of can­cer that killed Ap­ple co-founder Steve Jobs in 2011 and Amer­i­can soul singer Aretha Franklin in 2018.

It’s the can­cer that RTÉ jour­nal­ist, Tom­mie Gor­man has been liv­ing with for more than 20 years, yet neu­roen­docrine tu­mours (NETs) are still not widely known can­cers.

Of­ten mis­di­ag­nosed or not di­ag­nosed at all for sev­eral years, these rare can­cers are of­ten called the quiet can­cers.

A small group of NET pa­tients are now keen to speak out about their con­di­tion so that doc­tors be­gin to con­sider that com­mon symp­toms such as se­vere in­di­ges­tion and heart­burn and di­ar­rhoea might be some­thing more com­plex.

An­drea Martin (59) suf­fered episodes of ex­treme in­di­ges­tion for sev­eral years be­fore she re­alised she had a neu­roen­docrine tu­mour.

“I had gas­tric pain, on­go­ing in­di­ges­tion, heart­burn and nau­sea. I had a gas­troscopy [in­ter­nal in­ves­ti­ga­tion of the stom­ach] and an ul­tra­sound but noth­ing was de­tected,” she ex­plains.

One evening fol­low­ing an acute bout of pain, her sis­ter in­sisted on bring­ing her to the A&E depart­ment of St Vincent’s Univer­sity Hos­pi­tal.

“I had a CT scan which iden­ti­fied a seven cen­time­tre tu­mour on the tail of my pan­creas. I was very lucky be­cause the sur­geon on duty was an ex­pert in NETs and he had a sense that my can­cer was dif­fer­ent from the usual pan­cre­atic tu­mours. He or­gan­ised more tests and a biopsy which confirmed that I had a slow-grow­ing NET,” she ex­plains.

Three months later in Au­gust, 2015, Martin had surgery to re­move the tu­mour. “I pre­pared well for the surgery. I got fit­ter. I ate well, slept well and took a lot of rest. In fact, I felt so well, I con­sid­ered not hav­ing the surgery – which I later re­alised was a panic reaction,” says Martin.

She says that her post-op­er­a­tive re­cov­ery was good and she was back work­ing in her job as a me­dia lawyer in Oc­to­ber, 2015. “I did Tai Chi for a while af­ter­wards and then eased my­self back into Pi­lates. I have to watch my in­take of al­co­hol and sugar be­cause part of my pan­creas was re­moved but other than that, I’m fine,” she says.

Martin says that while there is a risk of the can­cer com­ing back, she feels con­fi­dent it will be treat­able. “I’m in the sys­tem now. I’m checked ev­ery year and if it re-oc­curs it will be picked up quickly and there is a range of treat­ments.”

Mark McDon­nell (53) went for a med­i­cal check-up be­fore start­ing a new job in 2002. That check up re­vealed that his liver wasn’t work­ing prop­erly and he was told to ex­er­cise more and eat and drink more healthily.

Eight years later, he went to the doc­tor with a pain in his toe and men­tioned his liver con­di­tion. An ul­tra­sound re­vealed sev­eral tu­mours in his liver. His on­col­o­gist rec­om­mended a “wait and see” ap­proach but sent him to NET spe­cial­ist, Dr Der­mot O’Toole in St Vincent’s Univer­sity Hos­pi­tal for a sec­ond opin­ion.

A NETs di­ag­no­sis was made and McDon­nell had over 65 per cent of his liver re­moved in two dif­fer­ent op­er­a­tions, eight weeks apart.

“The pri­mary NET was dis­cov­ered in my small bowel and I was put on so­mato­statin ana­logues [hor­mone in­hibit­ing drugs] for one year. I couldn’t tol­er­ate the med­i­ca­tion so I came off them and haven’t take med­i­ca­tion since,” ex­plains McDon­nell.

“I went through all the men­tal an­guish about hav­ing a can­cer and think­ing that I would die. Our chil­dren were teenagers at the time. Since then, I have a scan ev­ery year and I’m in­formed about my con­di­tion and I be­lieve the doc­tors have a lot of am­mu­ni­tion to treat it if it comes back,” he says.

Both Martin and McDon­nell are part of a NET pa­tient net­work which of­fers sup­port to those who are di­ag­nosed with these slow-grow­ing tu­mours and raises aware­ness of the rare and lit­tle-known type of can­cer which usu­ally orig­i­nates in the gas­troin­testi­nal tract. “It helps to talk to peo­ple on the same jour­ney as your­self,” says McDon­nell.

The main mes­sage of the NET pa­tient net­work is to en­cour­age GPs who sus­pect some­thing dif­fer­ent to send their pa­tients to a spe­cial­ist in neu­roen­docrine tu­mours. About 2,000 peo­ple in Ire­land have NETs with be­tween 120-200 newly di­ag­nosed each year. Dr Rachel Crow­ley is a con­sul­tant en­docri­nol­o­gist at St Vincent’s Univer­sity Hos­pi­tal in Dublin which was des­ig­nated as the Cen­tre of Ex­cel­lence for NETs in 2017. She says that the big­gest is­sue about NETs is that they are ex­tremely dif­fi­cult to di­ag­nose partly be­cause the symp­toms can seem so sim­i­lar to more com­mon con­di­tions such as Crohn’s dis­ease. “The ze­bra is the sym­bol for NETs be­cause in med­i­cal school, doc­tors are trained to con­sider the most com­mon ill­nesses first. We’re told “if you hear hooves, think horses” whereas we want GPs and con­sul­tants to also think that com­mon symp­toms such as se­vere in­di­ges­tion and nau­sea in a small num­ber of pa­tients can be due to a slow-grow­ing neu­roen­docrine tu­mour so we say “if you hear hooves, think ze­bras”.

The other dif­fi­culty with di­ag­no­sis of NETs, ac­cord­ing to Dr Crow­ley is that scopes [in­ter­nal cam­eras] and ul­tra­sound

I’m in the sys­tem now. I’m checked ev­ery year and if it re-oc­curs it will be picked up quickly and there is a range of treat­ments

scans rarely de­tect this type of slow-grow­ing tu­mour.

In­stead, a CT scan or a gal­lium PET scan is re­quired to iden­tify NETs. Up to now, pa­tients have been sent abroad for gal­lium PET scans, but St Vincent’s Univer­sity Hos­pi­tal will have the equip­ment in­stalled in Spring 2019.

Surgery is the first op­tion for those di­ag­nosed with a NET. Pa­tients with hor­mone-se­cret­ing ver­sion of NETs will then be given hor­mone-in­hibit­ing med­i­ca­tion to take for the rest of their lives. A small num­ber of pa­tients are sent abroad for a very spe­cific type of molec­u­lar ther­apy called pep­tide re­cep­tor ra­dionu­clide ther­apy (PRRT).

The RTE jour­nal­ist, Tom­mie Gor­man was in­stru­men­tal in set­ting up the NETs pa­tient net­work. He has spo­ken pub­li­cally about how he trav­elled twice yearly for 19 years to Upp­sala in Swe­den for spe­cial­ist treat­ment. “I’ve been told that I was the first per­son to get the E112 card [for the HSE Treat­ment Abroad Scheme],” says Gor­man.

In 2002, Tom­mie Gor­man made the doc­u­men­tary Europe, Can­cer and Me which chron­i­cled this treat­ment. “I was di­ag­nosed in 1994 and at that time, the life ex­pectancy for only 50 per cent of pa­tients was five years. Af­ter that it was in the lap of the Gods but the Gods have been kind to me.”

Since the NETs spe­cial­ist cen­tre opened in St Vincent’s Univer­sity Hos­pi­tal, Gor­man has be­come a pa­tient there. “Things have im­proved dra­mat­i­cally for Ir­ish pa­tients with NETs. This is not a be­nign dis­ease and we have lost some young and mid­dle aged peo­ple in the net­work. But we are now build­ing an Ir­ish ver­sion of the Swedish model. The di­a­logue we have with con­sul­tants, the HSE and pa­tients is more akin to a meitheal or co-op­er­a­tive.”

Tom O’ Donoghue, Mark McDon­nell, An­drea Martin and Michael Fal­lon of NET Pa­tient Net­work. Be­low: Aretha Franklin who died this year

Newspapers in English

Newspapers from Ireland

© PressReader. All rights reserved.