Rarity
Dear Reader,
We are delighted to share our twelfth issue of Rarity Life magazine with you, this edition feels especially poignant as we launched #RarityLife on #RareDiseaseDay2022. Over the last three years we have had the opportunity to speak to some wonderful individuals and organisations, and it is always both an honour and a privilege to be entrusted to write and share all of the stories that we have featured.
Rarity Life is an online publication that offers those affected by rare disease, disability and cancer the opportunity to create content that is truly inclusive. A platform to unify our collective experience and to celebrate and share our differences.
The experiences of children, young people and those with rare cancers are shared throughout this edition; from the stories of three truly remarkable young women, Sianna, Linseigh and Taylah to the commitment to the neuroendocrine cancer community that has shaped the life of charity founder and CEO Catherine. The personal stories shared by two incredibly strong and driven families, the Adelekans and the Stones, are testament to the power that individuals can have to truly bring about change and shine a light on their rare disease community.
As ever we’re so grateful to everyone who contributes their time, effort and stories to this magazine and to Same but Different, together Rarity Life truly shares your stories with the world.
Happy reading,
Same but Different
MEET THE TEAM
Ilmarie Braun
Ilmarie Braun Claire Li Gareth Jones
Ceridwen Hughes
conversation with Catherine Bouvier, CEO & Co-Founder of Neuroendocrine Cancer
with Bev Walley, Cheshire Community Nurse for Hope House Children’s Hospices
RARE Tales
Sleeping Beauty - Awakened Radiance
Our beautiful sleeping beauty Sianna was diagnosed with cancer at the age of just five years old, it was the love of her family that sustained her through her gruelling treatment, and it is love which will guide her through the unknowns of her adulthood. We are once again turning to a much-loved childhood tale, Sleeping Beauty, to reflect an unseen and rare interpretation of the ways in which the power of love can overcome obstacles and bring hope and healing.
£169.00
UK Sizes 6-16
Photography by Ceridwen Hughes
Dress:
Tulle Bandeau Tiered Midi Dress
View online Dress: Model’s own Purchased from The Dress Studio
Photograph by Ceridwen Hughes
A ways Dancing
In conversation with
Sianna Lyle Davies
“When I was five years old I was diagnosed with a Wilms tumour, which meant I had cancer in my right kidney. They had to operate to remove it, and so I’ve only got one kidney left which is on my left side. I finished my treatment just before Christmas of 2012 when I was six and a half years old.”
Sianna is eighteen years old now, and when you meet her what is immediately striking, beyond her obvious beauty, is her vitality. She has gorgeous, glossy long dark hair, and the fresh glowing skin and bright eyes that we typically ascribe to someone who is the ‘the picture of good health.’ However, having undergone the desperately necessary, but gruelling, cancer operations and treatments at such a young age the situation is a little more complicated than it might outwardly appear.
When reflecting on her cancer journey, she explains that “A lot of my memories are quite misty, but I really remember quite vividly going into the operating theatre room and the nurse telling me to count down from 10, when they gave me
the anaesthetic, I can remember that so clearly.” While the period during which she underwent first an operation, and then both chemotherapy and radiotherapy, remains largely hazy some of the ways in which her treatment impacted her, and a few other memories have stayed with her. “After my treatment, my hair fell out and I had to wear headbands. I was conscious then that I was different to the others. My mum let my hair fall out naturally, and to grow back in without shaving it all off which I’m glad about, as I always had a little of my own hair left.”
Another clear memory she has is how when, in the week that it was her turn to have her primary school’s mascot - a bear, the children would take turns to look after, she brought the bear with her to her first radiology appointment at Clatterbridge Cancer Centre. Once there first, the bear, then Sianna, were gently placed on the bed in turn to be shown how the radiotherapy machine would work. Not quite the normal, or typical experience, for either a primary school mascot or a five year old girl.
There are some more, special memories that she holds close still, both from her day to day life, with the kindness and care she was shown by her primary school community being one that stands out, but also from her time in hospital. “I had an obsession with the High School Musical films, and
one of my mum’s friends had given me the soundtracks. I remember I was dancing in my little room and I didn’t realise at first but the nurses were watching me from the window and smiling. I felt shy and kind of awkward though so I quickly got back into bed.” Indeed, not only were the times spent watching countless Disney films, including Sleeping Beauty, curled up with her mum in a hospital bed some of the happiest times in that difficult period of her life, but they have gone on to form an integral part of who she is today, and underscore some of the dreams and hopes she has for her future.
The weekend after Sianna was diagnosed with cancer. All photographs courtesy of The Davies Family 11 www.samebutdifferentcic.org.uk
Sianna loves the performing arts, from theatre, musical theatre through to dance, she dreams of a future which involves her passion for acting, singing, storytelling and movement. She began her first steps into this world by attending her local Stagecoach Performing Arts school, and she credits the confidence and joy she feels on stage to the support and guidance of the teachers she met there. “Stagecoach really helped me, they helped to build my confidence in acting, in singing and in dancing. I know it sounds like a cliche, but they really have helped me to learn, and to feel confident in what I can do - if you had asked me three years ago to sing a solo on stage I wouldn’t have done it! I just don’t think I would be where I am today if it wasn’t for them.” Over the years she has had the opportunity to take part in a number of stage productions, some of which have seen her tread the boards at the award-winning Chester Storyhouse Theatre. She was delighted to be cast as the wonderfully malicious Miss Gulch - who famously turns into
Sianna and the school mascot being shown how the radiotherapy machine works at The Clatterbridge Cancer Centre
the Wicked Witch of the West, in a recent production the Wizard of Oz. Because, as she explains “I loved that I got to play that kind of mean role, and it also meant that I got to be many of the different ensemble characters too, taking part in the big dance numbers which was brilliant.” She is particularly proud that, during the ‘West End to Broadway’ show that was performed as part of the 30 years of Stagecoach Chester and Castaway Theatre celebrations, she got to take part in many of the different numbers chosen, from Copacabana, Legally Blonde through to Oliver! But whilst it might have looked effortless to the audience, the reality is dancing for hours in exuberant, fast paced numbers was a step up for Sianna.
“After my treatment ended I had to learn how to walk properly again. I remember I had to have these little plastic splints that went from the top of my calf to underneath my foot, and I’d wanted pink but they didn’t have any (AFOs, ‘Ankle Foot Orthoses,’ are plastic splints which are made to keep feet and ankles in a good
position for standing and walking). There’s still an impact on my feet, and I can’t actually lift all my toes up properly. For example I can’t really tap, which is really annoying as I’d absolutely love to be able to tap dance. Learning how to drive was quite tricky, because having my foot on the clutch all the time during driving lessons was such a strain on me, but although it was hard I learnt how to drive a manual car.” Sianna is due to take her A-Levels this summer, and over the past months she has been busy revising for her mocks, the last of which she’d sat just a few days before we spoke. She was the last candidate to leave the exam hall, as she is given extra time and short breaks throughout the exam which she needs because, as with her feet, the strain of prolonged periods of writing is hard on her hands. This is because one of the chemotherapy drugs, a drug called vincristine, that she was given caused peripheral neuropathy, impacting both her feet and her hands.
But perhaps one of the biggest ways in which her life-saving cancer treatment continues to shape her life is in the unknowns that it brings and the ways in which it might yet shape her future. The section on the long-term effects of cancer treatment on the Children with Cancer UK website notes that ‘now that so many children are surviving cancer, there is a large population of childhood cancer survivors and these effects are becoming more apparent…The risk of adverse effects varies from child to child, depending on the treatments used, as well as the age of the child at the time of treatment. Possible effects include infertility.’ After her treatment had ended Sianna was still seen on a regular basis at Alder Hey Children’s Hospital for regular scans and tests.
Typically she attended these appointments with her mum, but when she was thirteen her dad took her, and it is this appointment more than any other that is seared into her memory. “It was the first appointment I went to with just my dad, and they told me that I might potentially struggle to have a baby and that they could offer me egg freezing if I wanted that. They also explained that if I was to carry a baby I may not be able to carry it for the full nine months, which did really, really scare me. They then went on to mention,
quite casually, that there was a possibility of me experiencing an early menopause.”
When Sianna was first diagnosed with cancer she was of course far too young to have had any of the difficult conversations that have since been needed. But during our call it becomes clear that the way and time in which her medical team decided to initially broach these subjects was neither fully appropriate, nor was it handled with the delicacy it would have warranted. Whilst we are talking her mum, Katina, pops in to say hello, and what she shared felt important to include here. “We were always very sensitive to, and mindful that the conversations the doctors had with Sianna should take place at the right time, and that she was not given information that she was not cognitively or emotionally ready for. I was very protective of giving her that space, of not having conversations that might put fears, and the wrong narratives into her head, and of using words and language that might then be selffulfilling or self-defining, too soon. So, for this to be shared so bluntly the one time I’d not gone to an appointment was hard.” And it is still hard, this unknown, and this worry, that she must now carry. Sianna
Sianna in front of her Head Girl photo
and her mum were watching a film the other night, and she described how parts of it made her feel a bit sad, and worried. “Even now it does hurt a bit. Yesterday, I was watching Bridget Jones’s Baby with Mum and we ended up talking about me, and my situation. I just really hope I can have a baby, but there is also the fear of not being able to carry it full term, of it being premature. I do worry, and feel quite anxious about it all.”
Now that she is an adult her ongoing care has been transferred to the local Women’s Hospital, and she is being supported by the fertility service to investigate and plan for her reproductive future. Her parents made sure that they were both at her first transition appointment there last year, and had collected her from school, which meant that she arrived in full school uniform. She laughs as she remembers how it might have looked to those waiting, because “I got so many looks, people were looking me up and down in my school uniform with my mum and dad by my side, and we realised that everyone was probably thinking that I was pregnant and of course I’m thinking ‘my god, this is not what it looks like, it’s like the total opposite,
I’m here to see if I will ever be able to get pregnant!” But Sianna is able, despite the uncertainties that she faces, to view her cancer journey pragmatically too, explaining that “I am kind of happy it happened to me then, because I feel like in many ways I just didn’t know what I was actually going through.”
Although there is no doubt that some of the conversations that Sianna has had to have, and will continue to have, are hard, she has generally been met by love and support from those around her. “I do know I’m quite different from my friends in some ways, especially when it comes to drinking alcohol. I have to be so much more aware of what I’m doing, and how much I drink because I’ve obviously only got one kidney, and I have to take care of it. But my friends are really supportive, they know that this has happened to me, and understand that I need to be more careful in some ways. Especially those of my friends who were in primary school with me, they know what’s happened, and although I don’t want people to give me sympathy, the support from them really means so much.” She continues, reflecting on how in many ways her cancer journey has also shaped their family life in an unexpectedly positive way, creating a loving
and strong family bond between the four of them. She explains that it was far from easy on them at the time, especially perhaps for her younger brother. “I remember when my dad had to rush me into hospital on Christmas Eve, I think I might have had a water infection or something, and they really needed to check me over, but what I remember most was my younger brother Zach who was only two years old not really understanding where we were going, and being sad that we were suddenly leaving.”
She still feels sad when she thinks of how it must have felt for him, not really knowing or understanding why their family was suddenly forced to spend so much time apart, with Sianna and her mum in the hospital and him at home with his dad. But, she says, “I think that now we have a better relationship for it. I think
it really kind of brought us together in a way, because we have to be there for each other.” In the years that followed her treatment Sianna visited Lapland with her Dad, a trip that gave them valuable time together, and the four of them visited Disney Land Paris, creating special family memories together after years of upheaval. It is trips like these, which were gifted to them by the charity ‘When You Wish Upon a Star’ and the Round Table’s Children’s Wish Charity, that are truly life changing in these situations, enabling families space and time together to heal.
With only a few short months left before her school years are behind her, Sianna is busy planning and dreaming about what comes next. She is going to take a gap year and hopes to go to Drama school the following year. Her gap year is important, as many of the most reputable drama schools want their students to have had some life experience before they allow them to enrol. Alongside her previous stage experiences, she has appeared in a short film, and as the cover model of this edition of Rarity Life magazine can now also include modelling experience to her list of achievements. She is also proud to represent her school as Head Girl, an accolade not many achieve, and certainly within her school, it is one more typically accomplished by those with dreams of Oxbridge rather than the performing arts. But it is an experience, or opportunity really, that she has been offered for the coming year about which she is most excited. Beaming when she tells me that her drama coach at the North West End Acting School has offered her “a year’s scholarship during my year out. He has agreed to let me attend the weekly adult professional acting classes, which is just incredible.” It is wonderful opportunities like these, opportunities that she has worked so hard for, that will help to ensure that her future is a bright one. A future filled with the sheer joy of dancing; in an empty room, with friends or on the stage, but always dancing.
What is a Wilms’ tumour?
A Wilm’s tumour is typically described as a type of kidney cancer. A Wilms’ tumour is a embryonal tumour, this means that the tumour develops from immature cells which, instead of developing into the kidneys, remain undeveloped in a child’s kidney after birth. They then start to grow, forming a jumbled and uncontrolled mass of small immature cells, in the main Wilms’ tumours usually only affects one kidney (unilateral). Currently, they account for around 5% of all childhood cancers in the UK, and in keeping with other embryonal tumour types Wilms’ tumour is most commonly diagnosed in children between 1-3 years of age.
For more detailed information visit the NHS website, linked here:
NHS - Wilms Tumour
To find out more about some of the charities which supported Sianna visit the links below:
www.whenyouwishuponastar.org.uk www.rtcw.org
www.childrenwithcancer.org.uk
www.younglivesvscancer.org.uk
To find out more about Stagecoach and the North West End Acting School visit:
www.stagecoach.co.uk
www.northwestendactingschool.co.uk
Sianna on her 18th birthday
In conversation with Bethan Adelekan
Photograph by Richard Boll
“Ravi was so brave when he was going down to A&E, we were in the taxi and I remember asking him ‘Daddy’s told you what’s happened, right?’ and he replied ‘yeah, he said that there’s something growing in my brain that shouldn’t be there, and it needs to come out.’”
Ravi Adelekan was just six years old in October 2021 when not only his life and his dreams, but also the lives and dreams of his family, were changed forever when he was diagnosed with a benign brain tumour that was located in his brain stem. The next year was a whirlwind of emergency hospital admissions, a hugely traumatic and difficult operation, a slow recovery and then the new reality of a body that needed to be helped to heal and flourish once again. Ravi needed more surgeries, rehabilitation in the form of a range of weekly therapies, and a seemingly constant schedule of medical appointments. What it means to live through such a harrowing year is a story worth telling, but the story of what came next is an utterly compelling one, and the one we want to share. A story not only of hope, but of truly trying to find the proverbial ‘silver lining’ in every situation, no matter how difficult it might be.
Bethan and Gbenga met when they were both working in the music industry. She was running a talent booking agency for jazz musicians, and he worked in music PR. When they decided to get married they moved to Brighton as they felt it would be a great place to eventually start a family. Married life was busy, with Bethan commuting to London and Gbenga working and playing in the highly successful band, Metronomy. Although they had made a handful of friends in Brighton it was the arrival of their first baby, Ravi, that really saw the family set down roots. “Our community 100% started as soon as we had Ravi. We went to an NCT class and the friends we met are still our core group. Our community is very much based in a small physical area, and where we live, there are so many creative people doing amazing, inspiring things. In particular, there are a lot of incredibly strong, inspiring women, which I just absolutely vibe off. Getting to hang out with them - and our community here - is one of the reasons why I think I would struggle to ever move away.”
Maya was born three years later, and Bethan describes those early years as a family of four as being wildly fun. However, she is, she explains, also something of a contradiction in how she likes to structure their family life. “ I’m incredibly fixated on
routines and processes, but I also can’t bear the daily churn, I need variety, I need different things to be happening, I need the craziness. I need all the wild things that we’re up to, because otherwise life gets too boring.” She continued, “we’re a very creative family. We love having music on and we also love watching sports together. We both believe strongly in treating children not as uninformed kids, but in having a very honest, open relationship with them. So we’ve always spoken to our kids like adults, within reason of course, and I think that that’s why we’ve ended up with Ravi, who’s like a forty-year old man at times, and Maya, who’s basically ready to take over a company at only six years old!”
It is surely in large part because Ravi has always been wise beyond his years that he has continued to dream big, instead of letting the, at times harsh, realities of a life lived with the after-effects of both a brain tumour, and the
Photograph by Photo Phoebe
Photograph by Nigel Buckner
operations and treatments that were needed, to deter him. Bethan explains that it was not always an easy path, and not one that Ravi navigated without struggle. The surgery he needed to remove the tumour was incredibly hard on him, and due to its location the surgeons were not able to remove the whole tumour, which means that he lives with continued symptoms as well as some permanent nerve damage. The weeks spent in hospital were difficult for them all, but they were unprepared for how hard it would be once Ravi was discharged. “There’s not enough secondary support in place, and there is also no plan for continued care.”
Bethan continues; “You’ve got a kid coming home after brain surgery who can barely walk, and all of a sudden you’re on your own and we weren’t able to access any rehab care when we came out.” After being discharged the family expected that Ravi would access the therapies and support he needed through the different community teams, but because brain tumours are so complex he needed a lot of support and input from different specialists. Bethan soon discovered that “there’s no one person coordinating that path to rehab. Ravi was having issues eating so we needed to see a dietitian. He needed psychological help, for which you’d need a clinical psychologist. He also needed an occupational therapist and a physiotherapist and we were simply told that the community occupational therapy and physio will be in touch soon. Great. But then we got a letter through confirming that he was on the waiting list, and that the wait was at least six months…”
Looking back on those first difficult months Bethan reflects that “we are so fortunate that Gbenga and I are both the kind of people who are able to go out and find things out, who will research things and reach out and connect with people. Some people don’t have that capacity in them, or they don’t even have the time or the energy so it shouldn’t be that way, but I am a fixer, I’m the kind of person who thinks ‘how are we going to solve this.’ So we managed to access
a lot of our support through people we knew.” Eventually the network that they built up around Ravi would go onto to become some of the brilliant team that appeared alongside him on Britain’s Got Talent as ‘Ravi’s Dream Team Choir.’
In the months after his surgery Ravi had to learn to live with the changes to his body and face, which he found especially hard as the facial paralysis he’d suffered left him with a ‘wonky’ smile he struggled to accept. A once sporty kid who dreamt of being a footballer he also had to learn how to walk again, and to accept that his balance and coordination would always be impacted to a certain degree. Accepting such huge changes is hard, and Bethan can still remember the day Ravi’s struggles were laid bare. She explains that “I’m not very good at dealing with feelings of disappointment or hurt, I don’t like feeling those feelings at all and so I protect myself by always pivoting to a positive angle so that I don’t fixate on that feeling of hurt or disappointment. One day we were watching a Newcastle United match and they were losing badly, so I tried to point out some of the good football they had played to Ravi as a way of ‘finding the silver lining’ because I wanted him to see that there are always positives to be had. But he threw me under the bus by asking what the silver lining was to his brain tumour.”
Although surprised by Ravi’s question Bethan replied, and it was this reply that would once again change their lives forever. “I told him that he had a voice now, and that people would want to hear more about his experiences. I told him that ‘you have a story that other people don’t, so they’re going to want to hear it, which means you can do whatever you want with it’.” At the time Ravi was still very angry about the surgery, and he blamed it for making his symptoms worse.
She continued, explaining how when she had talked to Ravi about what he wanted he’d said that what he really wanted most was to stop another child having to go through the same surgery. “So we talked about the fact that it is medical
research that is needed to find better treatments, and that requires money, so maybe we should start to do some fundraising” and although she initially had something like a cake sale in mind, Ravi replied that actually, what he wanted to do was to release a charity single!
The whole family love to sing, and Bethan thinks that the idea Ravi had for the charity single and the song he chose came about because of that. “At bath time we generally just listened to The Greatest Showman soundtrack, and the kids and I would sing incredibly loudly - and probably drive our neighbours up the wall, but it was definitely a release valve for us all to be able to just throw ourselves into singing.” She continues, “and unfortunately, I’m not very good at not taking things to the nth degree, I get very carried away with everything. My brain just kind of goes into overdrive and I get really excited from a creative point of view, so everything just totally snowballed.”
Once again the brilliantly supportive and creative community around the Adelekan family kicked in, and over the coming months the ‘Ravi’s Dream’ campaign came to life, with a website, an ambitious fundraising target and an utterly wonderful charity single that marked the first anniversary of Ravi’s brain surgery. The video for Ravi & Friends’ cover version of ‘A Million Dreams’ from The Greatest Showman featured Ravi, as well as Coldplay, Bastille, Damon Albarn, Paloma Faith, Mary Berry, Björn Ulvaeus (ABBA) and Heather Watson amongst others. When the family discussed how best to channel the funds they hoped to raise Ravi had chosen two charities “because they both sent care packages which were the only things he received where Ravi felt like somebody actually cared about him. He was having all these medical appointments that didn’t feel about him personally,
Photograph by Richard Boll
because it’s just the thing that’s happening to his body, whereas the care packages were supporting him as an individual, and so that’s why he chose them.” So this impressive cast of internationally acclaimed musicians and celebrities gathered in Ravi’s name to raise funds for The Brain Tumour Charity and brainstrust.
In the months that followed the momentum around the Ravi’s Dream campaign continued to build. From hosting star studded events at Soho House and The House of Commons, a charity football match, a fashion show through to TV appearances and winning gold at the More Radio Child of Sussex Awards, Ravi was eventually named by the Daily Mirror as the Pride of Britain Child of Courage in the summer of 2023. Ravi’s fame is however something that his parents have struggled with for a number of reasons, the main one Bethan explains is “the fact that he’s had so many amazing experiences that have come about because of this tumour doesn’t always sit well with me.” They can also see that they need to balance this conflict that they feel against the positive impact that these
experiences have had on their son. “Ravi’s face surgeries were really tricky times for him, and this has all given him some confidence that he didn’t used to have. He didn’t want to look in the mirror, he thought his smile looked silly. He just absolutely hated what had happened to his face, and was incredibly upset about it. All of the recognition has made him feel like it’s okay to look the way he looks, and to have the challenges he has.”
The success of the Ravi’s Dream campaign can at times feel like a bit of a mixed blessing for Bethan and Gbenga, who have to try to balance their own family life with the commitment they feel towards reaching the stated fundraising targets, and making the most of the unexpected and wonderful opportunities Ravi is offered. “We need to keep coming up with ideas, and keep giving so much of our time and energy to all these projects. Often, although we’ve given absolutely everything, we’ve only moved the needle maybe a tiny bit on our fundraising target. So then you can’t help but wonder if the outcome was worth everything that you have had to put into it. At times the fundraisers have really taken over our lives, and it has just become too much pressure so what we’re trying to we do now is to focus on raising awareness more.”
It was this recognition that led to the decision for Ravi, and the newly formed Dream Team Choir around him, to audition on Britain’s Got Talent (BGT) in the summer of 2024.
The team at BGT had reached out to Bethan and Gbenga after seeing Ravi’s charity single, but they had explained to Ravi that although he, like so many kids, had always dreamt of going on BGT one day they didn’t want him to take part. They felt strongly that too often “these programs are about manipulating people and their stories for ratings.” Yet when BGT
got in touch again the next year Ravi explained to his parents that actually he really, really wanted to take part. So Bethan agreed to talk to the producers at the very least, and to use this conversation as a way to explain their reservations. “I told them exactly what my concerns were; firstly, we shouldn’t be putting a kid with a brain tumour in a competition up against other people. Especially as Ravi’s vocal chords were affected by his facial palsy, so singing actually isn’t that easy for him. They explained that they didn’t want Ravi because he’s the best singer, but because they loved his story, and wanted to give him a platform to share it. They suggested we think of it as a one-off opportunity to perform live on the London Palladium and to share his story with the millions of viewers that watch.”
After much discussion the family had not only agreed to go for it, they’d come up with the novel idea of forming a choir around Ravi made up “of all these different people who had helped his journey, and so we ended up with this amazing group of people.” From medical professionals through to other children whose lives had been upended by a brain tumour, Ravi’s Dream Team Choir was only ever meant to be a way for Ravi to share the crazy and unique opportunity to appear on BGT with the community around him. Bethan is almost rueful when she explains that “I was convinced that we would not go through based on the conversations that I’d had with the producer about it being a once in a lifetime opportunity. So when we got the Golden Buzzer I was so utterly shocked, it wasn’t supposed to happen.”
The experience for the other kids with brain tumours, who were part of the choir along with their
families, was so overwhelmingly positive that Bethan and Gbenga knew that they had to embrace the crazy whirlwind that followed, but they were rightfully wary as the last thing they wanted was for anyone to experience any negativity which they feared might come once they reached the semi-finals. “We’re so proud of how we did, but I am so grateful that we did not get through to the final! The public outpouring of support at the audition was so positive, but then when we got to the semi-final all of a sudden it became a real competition. And that’s where the not so nice elements started to come out, and we saw comments like ‘these kids can’t even sing.’ So even though it was an amazing experience performing in the semi-final on the stage, and it really was utterly brilliant, I am just so, so glad that we didn’t get through to the final.”
When I ask Bethan what comes next she sighs, and it’s clear that she is wrestling with how to best explain the conflicting emotions that she feels. “So here we are, two years after we actually started fundraising, with what sometimes feels
like this huge weight still on our shoulders to do more. So we have to keep reminding ourselves that actually it is all having an impact, even though it can feel like we’re being pulled along by it at times. There was so much ‘noise’ between the audition and the semi-final, a real buzzing positivity, and it felt there was such a huge possibility in it all. Then straight afterwards there was just absolutely nothing. Everyone was asking what was next, but I was just burned out. I was so exhausted with it all, and it had been such an emotional roller coaster so I knew that we just needed to take some time out for a bit.”
One of the things that Bethan and Gbenga are most proud of is the experiences that Ravi has been able to share with his local friends, as well as other children with brain
tumours that he has met over the years. “For me, one of the most important things that’s come out of it all is that we were able to offer experiences to these kids that really felt like unique and amazing opportunities. When it was over most of the kids were really sad, not because BGT was over, but because they weren’t going to get to hang out on a Sunday anymore. For them it was a brilliant way to spend time with other kids with a brain tumour, and not in a hospital setting but out in the real world doing fun, normal things. It was a really safe space for them to make friends with other kids who share the same reality of living with a tumour.”
This joy is tempered with the knowledge that for some of those children these precious experiences were to be some of their last. Nothing can diminish
the huge loss of a child, but Bethan and Gbenga are comforted, if only a little, by knowing that Jasmine who was one of the models in their 2023 Fashion Show, absolutely loved having her moment to truly shine in the spotlight.
Today Ravi is doing well, and his tumour has recently shown signs of shrinking which is incredible. His symptoms, if not hugely improved, remain at least the same. He has ataxia, a hearing loss in his left ear, he can’t see out of his left eye following complications post-surgery and he has a hand tremor and general left sided weakness, but as Bethan notes, it has been three years since his surgery and he’s used to his new normal now. There is no doubt, however, that these symptoms “will stop him from being able to do certain things. He knows he’ll never be able to do a job where
you need to be able to do anything with fine motor skills for example. This is one of the reasons why I’m passionate about making sure there are more opportunities given to him than the ones that are taken away by the sheer physicality of the effects his tumour.”
Bethan and Gbenga are grateful for the truly incredible opportunities and experiences that Ravi has had, but they also do not want them to in any way continue to shape or even burden him if that’s not what he wants or needs. “We don’t want Ravi to feel that this is his identity, we’ve worked so hard to make sure that he doesn’t feel labelled as ‘the brain tumour kid’. Especially now that his tumour’s started shrinking, because it feels like there is a real possibility that within a few years’ time he they may not even be having regular medical appointments to check the tumour. Then we need to work out how we make sure that the experience and legacy that he’s created has only added to his opportunities rather than leading him on a path that he feels that he has to stay on. He’s already done so much, and given so much back, and maybe he will just want to forget that he ever had a brain tumour.”
So whilst Ravi is still busy doing lots of different things, for now they are mostly just things which are just related to him, to his interests, and to the secret dreams he has for his future. Bethan and Gbenga are taking some time, and will wait to see what comes next, knowing that when the time is right they will once again take a leap into the unknown. “It’s scary sometimes, but I think if you overthink it, that’s where the problems come in. I often say that ‘people can only say no,’ and that’s how I’ve always tried to live life. To try things, to not be scared of trying new or hard things. Over the years lots of our ideas haven’t come off, but they’ve always given us something to concentrate on as a family, a positive focus to guide us through the difficult times.”
About Brain Tumours
Ravi had a benign tumour in his brain stem, but what is a brain tumour? The NHS website describes it as ‘a growth of cells in the brain that multiplies in an abnormal, uncontrollable way.’ There are two main types of brain tumours, the first are non-cancerous, or benign brain tumours, and the second are cancerous, or malignant brain tumours. Tumours are also graded, the grading is based on two factors, ‘how fast they grow, and how likely they are to grow back after treatment.’ Benign tumours are graded a grade 1 or 2, with malignant tumours graded as 3 or 4.
However, if a benign tumour is located in a key area of the brain, or in a location that is difficult to reach, as was the case for Ravi, they can still have a profound impact. Indeed, the ‘symptoms of a brain tumour vary depending on the exact part of the brain affected.’
For more information visit the NHS here:
Learn more here
Information and support:
For additional information and support, or to find out more about the two brain tumour charities that Ravi is fundraising see below:
For information on The Brain Tumour Charity click here:
Visit The Brain Tumour Charity’s website
To find out more about brainstrust click here:
Visit the brainstrust website
Photograph by Yan Krukau, Pexels
Futures Bright
Nurturing Children’s Mental Health
“In the last three years, the likelihood of young people having a mental health problem has increased by 50%. Our Good Childhood Report 2022 shows that children’s happiness continues to decline. Now, five children in a classroom of 30 are likely to have a mental health problem.”
The Children’s Society’s Good Childhood Report 1
Ensuring good mental health is essential, allowing us to both cope and thrive in daily life. Today, we know much more about mental health, and support is more available and accepted in society. For children the need for good mental health is paramount,
significantly impacting their overall well-being and development. In a world where they are already trying to figure out where they fit in, plus dealing with hormonal changes, peer influences, and their perceptions of the world around them, mental health is a critical concern.
According to NHS England, one in five children and young people aged eight to twenty-five had a probable mental disorder in 2023. This alarming statistic was well described by the Children’s Commissioner Dame Rachel de Souza in her ‘Children’s mental health services 202223’ report which was published in March 2023. “Since I came into post in 2021, I have spoken to a million children and young people across the country. The vast majority of children are happy and feel supported by their family, school and wider community. However, there is a growing group of children who are struggling with their mental health. This generation of children has experienced uniquely
1 www.childrenssociety.org.uk/good-childhood
The Benefits of Good Mental Health
Emotional Well-being:
Promoting a healthy mental state in children helps boost their selfesteem, making them feel confident and capable. It also fosters resilience, enabling them to cope with stress and recover from setbacks effectively. These skills are essential for navigating life’s challenges and contribute to a strong, positive outlook.
Social Development:
Good mental health is the cornerstone of healthy relationships. Children with positive mental health are better equipped to form and maintain relationships with peers and adults. They are also more empathetic, as they can understand and share the feelings of others, nurturing compassion and a sense of community.
Academic Performance: Mental well-being significantly impacts academic performance. Mentally healthy children can focus and concentrate better in school, leading to improved learning and achievements, and they are also more motivated to engage in school activities and pursue their interests, enhancing their educational experience and future opportunities.
uncertain and challenging times. Some have spent some of their most formative years isolated and indoors, fearful they or their loved ones may catch a deadly virus. They are constantly bombarded by negative news, of wars and climate catastrophes. An increasing number are exposed to the harmful impact of social media, cyberbullying, and online exploitation.” These struggles truly highlight the urgency of addressing emerging mental health issues early on, as timely intervention and continuous support can help to prevent more severe problems in later life, underscoring the importance of proactive mental health care for children.
Investing in children’s mental health benefits not only the individual child, but society as a
whole. In today’s fastpaced and often stressful environment, it is a shared responsibility that requires the combined efforts of parents, educators, and the community. By promoting and supporting good mental health in children, we can help them develop into well-adjusted, resilient and successful adults.
Below are some practical tips to consider that can help to support children’s mental health. By implementing these strategies, we can support the mental health of children, helping them to thrive both now and in the future.
Open Communication: Encourage your child to express their feelings and thoughts, and create a safe space where they feel heard and understood without judgment.
Routine and Structure:
Children thrive on routines, it can be hard but where possible try to provide consistent schedules for meals, homework, play, and bedtime to help foster a sense of security.
Healthy Lifestyle: Ensure your child gets regular exercise, balanced nutrition, and adequate sleep because physical health significantly impacts mental well-being.
Positive Reinforcement:
Praise and reward positive behaviour, acknowledging their individual efforts and achievements can help boost self-esteem and confidence.
Teach Coping Skills: Help children develop healthy coping mechanisms for stress, such as deep breathing, mindfulness, or engaging in a hobby they enjoy.
Model Healthy Behaviour: Children learn by observing, so try to demonstrate healthy ways to manage stress, resolve conflicts, and practice self-care in ways that are meaningful for you.
Limit Screen Time: Encourage activities that don’t involve screens, like outdoor play, reading, or arts and crafts, to help nurture creativity and physical activity.
Stay Involved: Engage in your child’s life by participating in their activities, attending school events, and showing interest in their hobbies and friendships.
Encourage Social Connections: Support your child in building strong, positive relationships with their peers, wider family, and community members where possible.
Seek Professional Help: If your child shows signs of anxiety, depression, or other mental health issues, don’t hesitate to seek help from a mental health professional.
Of course remember, as well, every child is unique, so it’s important to tailor these tips to fit your child’s individual needs and personality, and do not hesitate to seek advice from professionals or your GP if you need to.
The long-term impact of good mental health in childhood can extend into adulthood, and creating a sense of balance and well-being during childhood influences overall life satisfaction, relationships, and career success. Ensuring a good standard of mental health in children is a crucial step towards a healthier, more compassionate world.
Photograph by Michael Morse, Pexels
www.mind.org.uk
www.hubofhope.co.uk
www.youngminds.org.uk
www.happymaps.co.uk
www.nhs.uk/every-mind-matters
www.youthaccess.org.uk
This
Year I’ve Decided NOT TO SHRINK
Written
and Contributed by
Actress, Writer & Immersive Producer Linseigh Green
This year, I’ve decided not to shrink. My most radical act is not existing differently but being okay with it. Celebrating it, even. It drives people mad.
Ever since I was small, I’ve felt strange. There was an inherent disconnect; an inherent sense of isolation and inability to properly toe the straight lines necessary to fit in. I processed things differently, my motor skills were poor, I couldn’t eat more than small amounts at once, and I suffered from an occasional stabbing pain in my stomach. At birth, I’d survived necrotizing enterocolitis (NEC), a rare and often fatal gastrointestinal disease that occurs in neonates. I’m alive because part of my colon was surgically removed. I was never going to be ‘normal.’
When I was sixteen, I began to be hospitalized with regular traumatic NECrelated episodes. I couldn’t access care due to a lack of research; in fact, a whole Gastroenterology department refused to see
me. I was worsening with age and wondered if I was going to die. On a quest to feel understood, from the age of nineteen, I’ve led the first known project on longterm NEC outcomes, raised awareness in speeches around the world, met with US Congress, run educational series at hospitals, helped 200+ patient-families tell their stories, and more. It turns out, I’m lucky. Some NEC survivors do pass away as teenagers. Some suffer from severe brain damage or rely on feeding tubes. I know a sweet, lonely boy who is practically growing up in a Paediatric Intensive Care Unit (PICU). We’ve been forgotten by those who saved us.
As a rare patient, my future is uncertain, but one thing is definite: I am okay with myself. I love my strangeness and give myself grace.
Photograph by Sylwia Wozniak @snowwhitephotography
Years later, I hosted the first known gathering of adult NEC survivors. Everyone said they’d always felt strange; everyone said they’d always struggled to connect. I was a Neonatal Intensive Care Unit (NICU) graduate with a uniquely wired brain. Perhaps if I’d known that earlier, I would’ve given myself grace.
I attended a private Catholic high school that regularly makes headlines for racist incidents. Between the ages of 14 to 17 I spent my time sitting on therapists’ couches and eating lunch in the library’s toilet, where I could conveniently flush away my shame. Students enjoyed stoning me, whispering the n-word in my ear, and reminding me that I was weird.
By the end of first term, I began to hate myself, too. I only felt safe and powerful when I:
• Had voice lessons.
• Performed in musicals.
• Got to wear my own clothes.
In these contexts I got to decide who I was: talented, stylish, and fleetingly untouchable. Out-of-uniform days were my chance to externalize what I felt internally. My authentic self-expression formed a kind of armor that lifted my chin and gave me a sense of conviction. I’d walk down the hall in swinging dresses and pumps that my French teacher said would give me bad knees.
And when I performed, I was good. Even those who didn’t like me couldn’t help but acknowledge it. It was somehow harder to feel like a loser when I felt like a glittering star.
I decided my revenge would be to grow up well. I would become an actress and be FABULOUS, and then everyone would know they were wrong about me. I moved to New York, where I didn’t become an actress, but a quiet academic who was reevaluating her place in society.
I was 18 when I realized being me wasn’t a bad thing. Because the only way to belong in New York is to be yourself. For the first time, I flourished in all my dimensions.
Even as I began inexplicably collapsing.
Even as, a year into a Cambridge Creative Writing degree, words stopped making sense.
Lectures sounded like a foreign language and my memory was moth-eaten.
I had what my neurologist suspected was a seizure. An MRI revealed a benign brain tumor, but nothing explaining my symptoms. My whole life, I’d said, “At least I still have my mind.” I never considered that, too, might someday fail me. What you should know about me is that I’ve always been stubborn. I’ve always fought, and I’ve always been a bit delusional. So I did
everything I could to push forward, writing in the rare pockets of clarity. The only time text processed easily was when I was acting out a script. Otherwise, reading was so challenging, I couldn’t enter bookstores without crying.
Still, I showed myself grace. Tried to appreciate snatches of light when I was quietly scared.
I was 23 when I realized I could love myself. It was the morning I could feel the first needles of sensation after being hospitalized with paralysis on the right side of my body. I rushed from Bed G to a Cambridge formal, accidentally accessorizing my cocktail dress with a hospital wristband. I suffered through three courses and a breadbasket across from a man who showered me with condescension. And for the first time, instead of internalising that negativity, my brain smacked his words down like Serena Williams. For the rest of that summer, my psyche automatically rejected and filtered anything that depleted my worth. I had been through so much that the unkindness of others felt so inconsequential, my brain did not have the capacity to file it away. So into the bin it went.
That did not mean that I was perfectly secure in myself. As I went on dates and chased midnight fireworks and danced until sunrise in a
physicist’s backyard, a phantom hand twisted my arm, leg, and face flogging them with needles. I was on fire. Drowning in ants. If I’m stuck like this forever, I thought, I’ll go mad. I wanted to claw out of my body and scream.
For a few days, I avoided going out in public, because my arm and leg had begun writhing uncontrollably, as if I were possessed. I cried in bed and watched The Hunchback of Notre Dame, thinking, you know what? I get this guy. Because the sicker and more divergent I became, the harder it was to make a case for my humanity.
But because I am me, I still went on and did a piece of physical
theatre, which summoned an uncomfortable awareness of the lack of control I had over my body. A year later, I walked out of the hospital at 5am with a new speech impediment, eyes that shut randomly, and an inability to look at faces and paragraphs. Again, because I am me, I went onstage the next day as Faust in a digital adaptation that was filled with the very stimuli that riddled my brain with ellipses. I couldn’t string a sentence together offstage, but onstage I spoke perfectly.
I still didn’t know why my brain only functioned when I was acting, nor did I know
why, a year later, I still couldn’t feel half my face or hold a pencil or understand my lectures. I’d been promised a neurology follow-up after the paralysis episode, but when I was finally able to read my discharge papers, they said: ‘Patient is just stressed; she says so herself. No further action will be taken; patient says she is happy with this. Patient will seek psychiatric help in her own country; patient says she is happy with this.’ When you can’t read, people have an easier time promising care they don’t intend to give.
By the time I performed as Faust, I’d been fighting for answers for over a year in the UK and for two years in the US. I was struggling through an Immersive Storytelling MA at Royal Holloway (while waiting for my Cambridge marks) that the course leader had convinced me to remain in. The following month I graduated from Cambridge with Distinction. Don’t ask me how a girl with cognitive dysfunction pulled that off but it became my new benchmark of possibility. In other words: It meant nothing was impossible.
Two weeks later, I was finally diagnosed with functional neurological disorder (FND), a signaling condition in which the brain’s hardware is fine, but the software isn’t. I was given a therapist in lieu of treatment. I sent my diagnosis to Royal Holloway, but was denied extenuating circumstances and disability support. Instead my program’s preferred response was to blame me; ‘poor work ethic,’ ‘lazy,’ ‘excuses,’ or even ‘you’ll never get a job’ but it simply wasn’t my fault, nor true. I once again somehow managed to graduate, except I was too angry to attend the ceremony. I bought myself a cupcake and demolished it in anger, and cried and walked and slept in anger for months.
After spending my entire youth preparing for the launchpad from education to the Real World, I realized that girls with broken brains don’t fly. This was not who I wanted to be when I grew up.
In January 2023, I was at my sickest. My neurologist had suggested experimenting with theatre as a form of therapy. So I did. I’d give critically acclaimed performances and then leave the stage door with a
walking stick, looking like a corpse. I had no idea where I was going. It was the only thing I had. By summertime, I had an agent and had booked the UK & Ireland tour of the West End hit The Drifter’s Girl, where I covered the leading role of Faye Treadwell as well as her daughter, Tina. I asked my doctor if it was possible for someone like me, who could barely ride the tube safely, to go on a weekly tour of thirty cities, unsupported. I was told there was no precedent, so try it out and report back.
I didn’t just survive; I put on stunning, highly functional performances with only a couple hours’ notice. My debut was executed without tech or dress rehearsal, prop familiarization, or a full run through of the show beyond practicing alone in my bedroom. I’d been taught the music the evening before. And I carried a two-show day in front of 2,000 people, once again shifting my benchmark of possibility.
It wasn’t a fairytale. I dealt with harassment, loneliness. A superior gave me a form declaring me “unfit” to be in a musical, demanding invasive medical history that was irrelevant to my job. On my last day, they repeated, “You know you can’t bring that walking stick onstage.” I replied that “I never have.” But if I did… so what? An agency once asked: “As a disabled person, can you really do eight shows a week? If people knew you were disabled, they wouldn’t have hired you.”
The fact is, the musical theatre industry, which thoroughly believes in green girls and skating anthropomorphic trains, isn’t ready for disabled talent. Which is a shame, because we make fantastically nuanced storytellers. And we need art because it heals. Because I had spent so
Ever
since I was small, I’ve felt strange. There was an inherent disconnect; an inherent sense of isolation and inability to properly toe the straight lines necessary to fit in.
Photograph courtesy of Linseigh Green
much time immersed in my craft, I returned from my tour stronger than ever.
This year, I’ve decided not to shrink. I’ve gone from feeling like a pathetic ‘thing,’ a spectacle with a cane and the butt of rude jokes, to boldly sporting neon, glittering, glowin-the-dark sticks like a dandy or flâneuse. I coordinate my outfits around them and have learned to appropriate the gaze that has historically stigmatized the existence of disabled young women. The first time I carried my clear stick with mesmerizing black swirls into the theatre, my colleagues passed it around, studying it in awe. My costar looked me in the eye and said “good for you.”
I’ve discovered expressions of joy and power can coexist with disability. My next project is aimed at helping disabled girls like me rediscover their power through fashion and celebration. I’ll also be collaborating with NYU on incorporating mobility aids in their 2025 fashion show. Twice, theatre and fashion have saved me. Twice, they’ve been pathways to self-determination. In the most unconventional way, I grew up to become my childhood dream. Being okay with yourself feels like stepping into a white New York February without a coat.
It’s your disabled body sparkling in a spotlight before thousands.
As a rare patient, my future is uncertain, but one thing is definite: I am okay with myself. I love my strangeness and give myself grace.
About Necrotizing enterocolitis (NEC):
Necrotizing enterocolitis (NEC) is an intestinal disease that affects premature or very low birth weight infants.[4][1] Symptoms may include poor feeding, bloating, decreased activity, blood in the stool, vomiting of bile, multi-organ failure, and potentially death.
About Functional neurological disorder (FND):
Functional neurological disorder (FND) describes a problem with how the brain receives and sends information to the rest of the body.
NEC
FND charities:
What We’re Into
In this edition we are doing something that we’ve not done before: reviewing a book, a podcast series and a documentary that all feature the indomitable Kris Hallenga, founder of the charity Coppafeel!
When Kris was diagnosed with breast cancer at twenty-three years old, her life changed instantly, setting her on a path she could never have imagined.
To find out more about Coppafeel! click here:
Coppafeel!’s website
Book Review:
Glittering
a Turd: How Surviving the Unsurvivable Taught Me to Live
By Kris Hallenga, published by Unbound
I’d had this book on my ‘must-read’ list since it was released in 2021, when I saw in May 2024 that Kris had passed away I was sad that I’d not read her book while she was still alive. Kris was clear that this book was not going to be ‘just another cancer memoir,’ but that instead it was to be ‘a handbook for living life to the fullest,’ and I worried that her death would colour my reading of it. But although there were undoubtedly moments when I felt sad knowing that Kris was no longer out there glittering her turd, I absolutely loved reading all about the many different ways in which she did glitter said turd throughout her remarkable life. She was a true force of nature, and her warmth, humour, determination and unbeatable optimism shine through the pages of the book. Glittering a Turd is a brilliant read: honest, uplifting, and yet pragmatic, wry, and reflective and it absolutely achieves her key aim; ‘to understand that what is important is how and why we live.’
You can buy this book from all the usual places.
Podcast Review:
Glittering a Turd – Hosted by Kris Hallenga, an Audioboom Studio podcast
In August 2022 the preview to a brand-new podcast series hosted by Kris Hallenga was released, teasing all the exciting plans that she had for her Glittering a Turd podcast. The series ran for 24 episodes, each one featuring a special guest as well as sharing some listener stories. The aim was simple, to ask both the guests and listeners to share some of the ‘turds’ that life had thrown at them, and how they had found a way to glitter them.
In listening to the series it became clear that she was a warm, engaging and often funny interviewer, and her conversations with her podcast guests often feel as though you are listening to some friends catching up. Episode 1 features Giovanna Fletcher, which very much sets the bar for the brilliant guests that follow, from well-known presenters like Dermot O’Leary and Fearne Cotton through to campaigners, sportspeople and musicians. My personal favourite was episode 7, which features Sophie Morgan, whose book Driving Forwards we reviewed back in Issue 3 of Rarity Life magazine.
You can find the series here:
Listen on Audioboom
Also available on all the usual streaming platforms.
Documentary Review:
Living Every Second: The Kris Hallenga Story
Directed by Neil Bonner, Produced by October Films Limited, broadcast by BBC TWO
To watch it click here:
Watch on iPlayer
I watched this documentary after reading Glittering a Turd and listening to a number of the Glittering a Turd podcast episodes, and there can be no denying that that will have influenced both the way, and possibly also the extent in which I was impacted by seeing Kris’s story unfold.
Kris had an undeniable presence. Although there is something almost luminous and delicate about her features and her slight frame, the minute she begins to talk her honesty, her drive and her relentless energy become evident, and you can’t help but be swept headfirst into her story.
It isn’t always easy seeing the harsh realities of cancer, especially in the final months of Kris’s life, but life isn’t always easy, and to pretend otherwise would be doing her a huge disservice. She spent so much of her time and energy trying to share the lessons she learnt through receiving a terminal cancer diagnosis when still so young; that what matters most of all is how and why we live in the days that we are given.
Photographs courtesy of Costello Kids
COSTELLO
“After we got married we wanted children, but it just wasn’t to be and so we underwent eight years of fertility treatment at Manchester Hospital.”
The charity Costello kids, which is based in the UK but has a global reach, is best described as ‘a parent-to-parent support group that provides information and support about Costello syndrome to parents and health providers around the world.’ As with so many rare disease charities the story behind Costello kids is a deeply personal one, it is the story of Colin and Cath Stone, and their incredible, and much longed for daughter Helaina.
After going through years of fertility treatment the Stones were excited to discover that Cath was expecting, but as Colin explains the pregnancy was difficult from the offset. “Cath had a very complicated pregnancy, because things looked wrong from the beginning, but nobody quite knew at the time what the issue was. The baby was
In conversation with Colin Stone
initially misdiagnosed with arthrogryposis (a rare condition in which describes congenital joint contractures) because their limbs were in unusual positions. Because it was such a fraught situation different options were discussed, and because it wasn’t thought that the baby would survive birth a late termination was suggested. But we’d done eight years of fertility treatment to have this baby and so we decided ‘let’s just see how things roll.’”
Their baby, who they called Helaina, was born and the doctors were initially relieved to note that she did not have arthrogryposis after all, however there were still complications.
Helaina couldn’t feed, and at two weeks old her heart developed severe arrhythmia.
Eventually, after eight weeks in the Neonatal unit she was allowed to go home with her parents, but as Colin explains, things were far from easy. “She wouldn’t feed, so feeding was a major nightmare, and she couldn’t sleep. She was awake and crying most of the night, just sleeping fitfully, waking, sleeping fitfully… and not eating. When she did eat, she kept throwing up constantly.”
Because of the issues discovered during the pregnancy and her difficulties since birth Halaina had a multidisciplinary medical team around her, and quite incredibly her geneticist had an idea about what might be underlying all the issues she was experiencing. “Costello syndrome was originally reported by Dr Costello, a paediatrician from New Zealand,
in 1971, followed by a more detailed paper in 1977. But then it was pretty much forgotten about until the early 1990s. Helaina was born in 1994 and so you have this rare syndrome that nobody really knew much about, if anything, because there were hardly any publications, but her geneticist knew about it, and was able to diagnose her with Costello Syndrome at six months old.”
Despite now having a diagnosis for their daughter they soon realised that there was scant information, advice or indeed support that their doctors could offer them, as so little was really known about the syndrome - indeed Helaina was only the 23rd person worldwide to have received this diagnosis. But, Colin explains, they are both resourceful people and so they decided to do whatever they could to learn more. “I worked at Manchester Metropolitan University, so I took myself off to the medical library and the librarian got me all the publications that were available at the time. Unfortunately, our paediatrician didn’t think that the publications were suitable for parents and had opted not to share them with us.”
After reading everything they could find the Stones felt relieved to finally feel as if they “now knew a bit more about Helaina. She had always had chronic feeding issues and we saw that it was one of the main features of the syndrome. Today the majority off
individuals with the syndrome will have surgery to have a PEG tube fitted (a percutaneous endoscopic gastrostomy (PEG) is a feeding tube that allows enables someone to receive nutrition directly to the stomach). At the time we tried an NG tube, very unsuccessfully, so I found a solution whereby I stuck a knife into a bottle teat and just poured the milk down her throat. I ended up making up my own formula based around unsweetened condensed milk mixed with regular baby formula to try and get more calories into her, so she might gain some weight.”
Reflecting on those early days
Colin describes how “we were in a somewhat difficult situation, and we knew very little, so we just struggled along.” Determined to help their daughter as much as they could they decided to try to find and connect with other families, and so Colin thought he might try and search online. Anyone reading that sentence today would likely not give this decision a second thought, but of course in 1995 using the internet was still relatively new, and there was certainly not the wealth of knowledge that there is today, either about how to find or indeed share information. However, he had a background in IT and was also able to use the infrastructure and resources at the university.
“I thought ‘I wonder what would happen if we set up a website’. And, despite only limited internet access and technology at the time, I managed to set up a very basic site, which I called Costello Kids. We went live in January 1996 and within three months were contacted by a family in Birmingham, Alabama.” This first contact would prove to be an incredibly important one, because after chatting online over the following weeks Colin explains how he and the American Mum (Tammy) decided that “there’s the two of us families now, so let’s set up a support group.” Over the next few years the
support group slowly grew, with some of the first families joining from Australia and France, and today there are families living in 50 different countries around the world who have registered their membership with the charity!
Costello Kids had decided to host their first global conference in Alabama in Easter 1999, and Colin and Cath were really looking forward to finally meeting all the families they’d been so central in bringing together, but it was not to be. “Unfortunately, Helaina got cancer. She was diagnosed with cancer six weeks before we were due to go over to America. When we she got the diagnosis, it was once again a situation where there was no real information for us.” However, not only had Colin read through everything he could find about Costello Syndrome years earlier, but he had also added scans of the publications to the charity website and so had everything to hand. He explained that when they discovered that she would need emergency surgery, he told her medical team that we “have all these publications on our website, and they may help. Indeed, having them to review was a huge help to the surgeons, anaesthetists and everybody else looking after her, and they turned out to effectively help save Helaina’s life.”
It is a perhaps overused phrase, but knowledge really is power, and the knowledge that the Costello Syndrome Community has been able to gather, share and explore over the last few decades truly highlights how it is often the community immediately affected by an ultra-rare condition that drives forward research. Colin describes how “we didn’t know at the time that Costello Syndrome was actually the part of something much, much, much bigger. Understanding it more became, for me and for Cath, an obsession at the time.” He continues and describes how several of the parents shared this drive to truly understand their children’s condition and felt that “there was more to it than met the eye. There’s a lot of crossovers with both Cardiofaciocutaneous syndrome (CFC) and Noonan syndrome, with physical features and other health related issues, and people are often misdiagnosed with having Costello instead of CFC or Noonan’s and vice versa.”
Although it was not yet clear if the syndromes were linked a lot of research was being done, and teams around the world were trying to identify the gene for Costello Syndrome. Despite being an incredibly rare syndrome, it became apparent that “a number of children with Costello Syndrome had also been diagnosed with cancer, and it was a mystery why there was such a high incidence of cancer within this really, really, really rare syndrome. The statistics indicated that between 15 to 17 percent of children
were diagnosed so it was a very high risk. The cancer diagnosis most received was for a childhood cancer called Rhabdomyosarcoma, which is also a very rare cancer itself at the best of times.”
In 2005 Professor Aoki, as part of a team in Japan, identified the gene for Costello syndrome, and Colin describes how “they identified that it was on the HRAS gene, so that explains the cancer because HRAS is a wellknown cancer gene.” It is worth noting that the HRAS gene belongs to a class of genes known as oncogenes, which if mutated, have the potential to cause normal cells to become cancerous. The HRAS gene is in the Ras family of oncogenes, which proved to be important in the broader framework that Costello Syndrome is part of. “So, we have a genetic disorder sat on a cancer gene which had never been heard of before. But we saw that what Professor Aoki found was that Costello Syndrome was, in fact, related to CFC syndrome and it wasn’t just something that looked similar but was a unique syndrome. More surprisingly, unbelievably almost, a huge new picture emerged that Costello Syndrome was the rarest of a group of conditions, which include Noonan Syndrome and Neurofibromatosis type 1 (NF1) and that in fact, at that time, there were nine related disorders.”
That these separate conditions were in fact related enabled them to be viewed together, as the RASopathies. Colin continues to explain “Costello Syndrome might only affect 1 in 390,000 births, and Noonan Syndrome and Neurofibromatosis type 1 affect around 1 in 2500 births respectively, but together Rasopathies are the largest group of developmental disorders that nobody’s ever
heard about! So whilst they are individually still very rare disorders, viewed together, they’re huge.” This knowledge changed everything, and as the different rare disease communities connected and began to work together as their understanding of the individual syndromes only continued to grow.
Colin began to work closely with Lisa Schoyer and other parents, explaining how they
were all “slightly obsessed, to a point of really being insanely obsessed, with developing a much, much better understanding of the syndromes, as well as exploring new treatments and therapeutics.” In 2007 the Costello Syndrome, CFC and Noonan Syndrome support groups worked with Lisa to build a parentled nonprofit which would help cement the vital link between the family support organisations and researchers. Today, the RASopathies Network continues to work on their central mission; ‘to advance research to improve the quality of life for RASopathy families by bringing together families, clinicians and scientists,’ supported by a global network of advisory boards which includes Colin and Costello Kids.
Back in 1994 when Helaina was diagnosed with Costello Syndrome the doctors had very little knowledge about the condition, and they felt that her overall prognosis was poor, telling the Stones that “we don’t think she’ll walk, we don’t think she’ll talk, and she probably won’t achieve much in her life.” But, as Colin proudly shares, she has proved them wrong time and time again, and has achieved so much in her life. “She was awarded the gold Duke of Edinburgh award in 2018, and she also was awarded a gold blue Peter badge because of her bravery and determination. Her biggest love is dancing, and she dances both ballroom and Latin, competing in the additional needs
competitions in Blackpool and Withenshaw Manchester. Having a disability has not stopped her from achieving and enjoying her best life.”
For families who receive a new diagnosis today the picture is a more hopeful one, because whilst the syndrome is complex, the depth of knowledge around the typical presentations and complications of the condition, and how best to treat and support those living with Costello syndrome has grown exponentially. That is in large part due to the commitment and determination of parent’s like Colin and Cath to both better understand and advocate for their rare children.
When Colin created the website for Costello Kids they were, he thinks, “one of the first rare disorder groups officially online” and, despite the fact that Costello Syndrome remains incredibly rare, the Costello Kids website estimates that there are between 1000 to 1500 people living with it worldwide. Although often separated by hundreds of miles, their community is a very close and supportive one. “We’ve talked to families across the world, we might need to use Google Translate but we’ve been able to put families in countries around the world in touch each other. Families that have been alone, and have been isolated, and through Costello kids we try and end that isolation. There might be nobody else within hundreds of miles to where you live, but we’re a global community, and you’re not alone.”
About Costello Syndrome
Costello Syndrome was first discovered, and subsequently described in the Australian Paediatric Journal, by a paediatrician in New Zealand called Jack Costello in 1977.
The RASopothies Network website describes how ‘Costello syndrome affects multiple organ systems. Its typical presentation is characterized by diffuse hypotonia and severe feeding difficulties in infancy; short stature; developmental delay or intellectual
disability; characteristic facial features; curly or sparse, fine hair; loose, soft skin with deep palmar and plantar creases; papillomata of the face and perianal region; joint laxity with ulnar deviation of the wrists and fingers; tight Achilles tendons; and cardiac involvement (hypertrophic cardiomyopathy [HCM], congenital heart defect, and arrhythmia). Postnatal cerebellar overgrowth can result in Chiari I malformation with associated hydrocephalus or syringomyelia.
An approximately 15% lifetime risk for malignant tumors includes rhabdomyosarcoma and neuroblastoma in young children and transitional cell carcinoma of the bladder in adolescents and young adults.’
As with many rare conditions the understanding of the syndrome has continued to grow over the years and today the NHS website lists Costello Syndrome under RASopathies, noting that ‘RASopathies are a clinically defined group of conditions caused by changes in genes
involved in the Ras/MAPK pathway. These conditions include Noonan, LEOPARD, Costello and cardio-faciocutaneous (CFC) syndromes, neurofibromatosis type 1 (NF1) and Legius syndrome.’
Information collated from the following websites:
For more information and support visit:
www.costellokids.com
www.rasopathiesnet.org
www.costellosyndromeusa.org
Versions
The different of Me
In conversation with Taylah Miranda, Ambassador for the Children’s Cancer Foundation
When I speak to Taylah, a beautiful and vibrant 24-year-old childhood cancer survivor, it is early in the morning in Australia and midevening for me. Even though it is still early in Melbourne, Taylah is wide awake and energetic, but then as she notes, her usual time to wake is at 5:30 am as she likes to get some exercise in before starting her day. Today she is a vision of glowing good health. However, appearances don’t always tell the
full story because in her relatively short life she has twice been diagnosed with, and treated for, Acute Lymphoblastic Leukaemia. The first time was in 2014 when she was just 14-years-old and she was 19 when she was diagnosed for the second time, just as the global covid pandemic hit and much of the world went into lockdown.
“I was in year nine when I started feeling a bit unwell, I was getting headaches and feeling dizzy, and I remember how that Remembrance Day in November I simply couldn’t stand up in the school assembly which was so unlike me as I was very sporty, very active. My mum took me to the doctors, but they said I was just
depressed, and probably under too much pressure from school and just sent me home. But my mum knew that something was wrong and got me a blood test, and although it looked relatively normal my doctor said if I got a temperature to go to the hospital.” Taylah continues, recounting how her mum did indeed take her into hospital, from where she was referred to the Children’s Cancer Centre. Initially, they too planned just to discharge but her mum, trusting her mother’s instinct insisted they do more tests, arguing that “if there’s no clear answers from the examinations and tests that you’ve done then you need to be able to tell me that it’s definitely not something awful.”
Devastatingly the results of a subsequent bone marrow biopsy found leukaemia, it was still in such early stages that “it wasn’t even in my blood, it was very preliminary and was just in the bone marrow. But still, that day changed my life. My mum came home and told me the news, and then we went straight into the hospital that night where I had surgery to get a porta-cath fitted” (a medical device which is inserted into the chest and used to draw blood and give treatments). The next few years were hard on Taylah, as the prescribed treatment protocol for leukaemia is necessarily aggressive. Immediately she was started on an intensive chemotherapy regimen to
All photographs courtesy of Taylah Miranda & the Children’s Cancer Foundation
‘try to get rid of the cancer,’ after which she explained the treatment entered a “consolidation phase that lasted around a year and a half and involved a weekly, regular chemotherapy schedule which was then finally followed by a maintenance phase.”
There can be no doubt that undergoing cancer treatment is gruelling, both physically and emotionally, and to do so throughout your teenage years as you stand on the cusp of adulthood is not to be downplayed. Looking back on those often difficult times Taylah reflects that “this was all going on for me from year 9 through to year 12 which was my final year of school, and so school really became my escape in that time. It was the one place where I felt that I could still be ‘normal,’ it gave me space to still be me, whereas at home and the hospital it felt like it was a totally different version of myself.” The side effects of cancer treatment are hard to hide, from vomiting and nausea through to visible hair loss, in many ways, there is nothing private about cancer.
“When you lose your hair it’s really hard, because it’s part of you, your identity, and what you look like is also kind of who you see yourself as. So, I felt I just became a totally different person within a few weeks. I didn’t mind people knowing what was going on with me, but I was upset that people couldn’t just view me as me anymore. Of course at the same time, I didn’t view
myself as me, it was like I was split into two and I now had this whole new life that nobody else at my school had, it was almost like a double life.”
At 14 years old we are all still learning, and growing, and don’t always know how to deal with the difficulties that life can bring, and it was no different for Taylah, her friends and the wider school community around her. Even as adults we don’t always know how best to support someone going through cancer, and it becomes harder still when your priority is not the cancer patient themselves, but your child who is their friend. She recalls that “a lot of my friend’s parents were, I think, afraid to send their
kids into a hospital to see a child who’s really sick. Now I’m an adult I can understand that it might feel tricky for them put their child into that super emotional situation with another child, but it meant that it was only really one friend who visited me in hospital, and that was in large part because her mum is a doctor.”
“I had a really big breakdown with a lot of my friends maybe six months into treatment because I felt like they did not want to ask me anything about what was going on, I think understandably perhaps because
they were scared, and they were worried that it would upset me but I felt like it was just being ignored and not acknowledged at all.” As Taylah found, although it is hard to do, being honest about what you need and how you feel is worth it, because “after that chat, it was better, and my friendship group was able to be there for me, and they were still my friends.” However, she still felt a disconnect, because despite being supportive they could not truly understand about this whole other life she was having to live outside of school, the life of someone with cancer, and so they could not always truly ‘empathise or support’ her. Now she can see that in some ways this was also a good thing for her because their lives were still the same and it meant with them “I could still be in that same life I had before, I could still be just me when I was with them.” With them she could still be the version of herself that she knew best, and could take refuge in.
Taylah was so inspired by the doctors and the staff at the hospital that she had met over the course of her treatment that she had decided she wanted to be a doctor. After graduating from High School she enrolled onto Bachelor of Biomedical Science degree at Monash University, but having done her first year she realised that “I was super burnt out from all my treatment, and from working so hard at school. So, I took a year off and went travelling overseas, which
is probably the best thing I ever did, especially as it was just before the covid pandemic.” It was whilst she was in the UK that she found a lump in her breast, the GP she saw referred her to St Thomas’s Hospital in London where despite her medical history the doctor she saw didn’t even examine her.
“He reassured me that it was ‘fine’ and was likely ‘just going to be a fibrous growth or something.’ He suggested I keep travelling and not to worry, so I did keep travelling for four more weeks but it was growing and I was getting more concerned so I flew home.”
“They did a biopsy on it, and it was my leukaemia. It was just so unexpected. At that point I simply could not even fathom that I could relapse, it was just not something I had really thought about before. For a blood cancer to come back there (in the breast) and to not come back systemically through my bone marrow was just such an unknown, indeed there’s no protocol for that. It just doesn’t happen. When they did a bone marrow biopsy there was nothing in there, it was just all really weird, and I was immediately thrown back into that world.” This time would prove to be incredibly difficult for Taylah and her family, and the blows just kept coming.
Two months after her relapse her beloved stepdad died of a sudden cardiac arrest, and just two months after that the pandemic struck and the shell-shocked and grieving family were locked down at
home, without the support of the community around them. This also meant that, as she was now 19 years old, she had to attend all of her medical appointments alone without her mum by her side.
This time she only had treatment for a year, and she had finished in January 2021 she felt well enough to return to university part-time for her second year, and then full time for her third year in order to complete her degree. After graduation, and despite having been given the ‘all clear’ she had “a little bit of a scare, I did a blood test and they thought that maybe that cancer was coming back because there was a cell that looked suspicious.” It proved not to be, but it led to Taylah having a conversation with her mum about her younger brother who was struggling at that time with his mental health and school. “She told me that he’d confided in her that his biggest fear was that I would die. And that breaks my heart still as it wasn’t that long ago.”
Taylah’s siblings were also older the second time she became ill, and unlike the first time when they were too young to “grasp the gravity of what was happening that time, they definitely knew what was going on.” But even still, she hadn’t realised how much they all continue to worry about her. She continues, explaining sombrely that “when you are first diagnosed with leukaemia the odds are at around 80% survival, but when you relapse it’s more like less than 50% of people who survive long term.”
Four years on from treatment Taylah needs regular blood tests and continues to see her oncologist too. Once a year she undergoes a battery of tests to check on her overall health, including an echocardiogram because “all the chemotherapy treatments could have damaged my heart. I have spirometry tests for my lung function, thyroid tests because I had radiation the second time I had cancer, and I have bone density tests amongst other things, just to make sure my body’s functioning.”
Despite the very real impact the cancer had, and will always have on her overall health she is thriving.
“It’s only now, in this last year really, that I feel pretty normal again because for the years during my treatment, and even in between finishing my first treatment and starting my second I still didn’t feel like I was fully recovered. I wasn’t ready to go back to do sport for example, and for years I still had my ‘steroid face.’”
“But, now I am in remission I’m just living my life and trying to give back to the community. I fundraise for
the hospital I was treated at, and most importantly, through my role as an ambassador I want to try and make people’s journeys through cancer a little bit better, that’s my main passion now.” Driven by her desire to give back to the settings and organisations that saved her life Taylah began working with the Children’s Cancer Foundation as one of their ambassadors.
“I’ve always been a good public speaker, and able to get my message across, so if I can I’m happy to use my story to help others.”
One of the issues that she is most passionate about is the impact on fertility that cancer
treatments can have, and the right individuals should have to try to protect and preserve their ability to have a family in the future should they wish to do so.
“When I was first diagnosed, I didn’t have the choice at 14 to choose what to do, so my mum just made the decision. Obviously, at the time it was the right decision to put me into treatment straight away, rather than delaying for weeks and trying to harvest eggs to be frozen. At that time you couldn’t just take a slice of your ovary and freeze it, the technology simply wasn’t there, instead you’d have to go through weeks and weeks of injecting yourself, and then going through the surgery to get your eggs and we just didn’t have a month, as my leukaemia would also just keep growing.”
By the second time she underwent treatment she was able to make the decision to undergo the treatment needed to harvest and freeze some of her eggs.
In December 2024 to address these challenges, and to ensure that children can thrive after cancer, the Children’s Cancer Foundation announced funding into a groundbreaking national fertility preservation service, and it is projects, research and initiatives like this about which Taylah is most passionate. Explaining in the self-deprecating manner in which she has spoken of all her accomplishments, “If I can come along and say a few words about why I think it’s important, and if people listen because of things that I’ve been through, then I’m honoured to be able to do so.”
About Acute Lymphoblastic Leukaemia
Acute lymphoblastic leukaemia, also known as ALL, is a rare type of cancer which is most commonly found in children, often before the age of four years old, but it can also affect older children and teens. ALL affects the blood and bone marrow, and usually develops quickly and needs to be treated straight away.
The prognosis will depend on the type of acute lymphoblastic leukaemia diagnosed, as well as the age and general health of the child.
Information taken from the NHS website, linked here:
NHS - What is acute lymphoblastic leukaemia?
To find out more about the Children’s Cancer Foundation Australia click here:
Australia click here: Visit the Children’s Cancer Foundation Australia’s website
Photograph by Jonathan Leonardo, Unsplash
Introduction to Cruising
Sailing has been a way of travelling and indeed a way of life, for thousands of years. However, for many of us today sailing is thought of as a luxury, and cruises especially are often looked forward to as ‘the trip of a lifetme.’ When we think of cruises we think of luxury, elegance and the romance of bygone eras.
Many cruises provide highclass restaurants, bars, and entertainment that draw people in while they travel from port to port, visiting new destinations. Some ships are more party-oriented, some are formal and others more relaxed, but there will be a cruise suitable for your own tastes, needs and experiences. Be aware that some cruises will depart and arrive at destinations that may involve you needing to fly to or from them.
Most cruise lines will offer accessible holidays, but it is worth noting that there will be a limit to how many passengers with additional needs will be allowed onboard at any one time. Each ship will have different mobility options and restrictions, and it is advisable to check that it will be suitable for you and your family. If you have additional needs, we always recommend contacting the cruise lines directly to ensure your cruise is perfect for you.
Set Sail on Unforgettable Voyages Adventures
Destinations
There are a wide range of cruise options that will cover destinations globally, not to mention the length of cruises, from weekends to months at a time will vary. There will be a choice that is perfect for you. We have picked our top 5 dream cruise locations and which are, it seems, some of the most popular cruise destinations.
Alaska
Alaska is famed for its awe-inspiring natural beauty, with towering glaciers, abundant wildlife, and breathtaking landscapes. Accessible cruises offer a unique opportunity to explore this majestic region from the comfort of the ship. Passengers can enjoy views of glaciers and fjords. You can even, if you are lucky, sometimes catch sight of whales, bears, and eagles. A great destination for nature lovers.
The Caribbean
The Caribbean is renowned for its warm climate, crystalclear waters, and idyllic beaches. Cruises here allow guests to enjoy the beauty and culture of various idyllic islands. Many ports offer accessible shore excursions, including beach access, cultural tours, and snorkelling adventures. The laid-back atmosphere and stunning scenery make the Caribbean a top choice for a relaxing and enjoyable cruise.
Mediterranean
Rich in history and culture, the Mediterranean offers a diverse range of experiences, from ancient ruins to vibrant modern cities. You can explore historic landmarks, beautiful coastlines, and charming villages. Ports like Barcelona, Rome, and Athens are equipped with accessibility features, making it easier for guests with disabilities to enjoy the sights and sounds of this captivating region.
Norwegian Fjords
The Norwegian Fjords are known for their epic natural beauty, with deep blue waters, dramatic cliffs, and lush greenery. Cruises here are popular and provide an excellent way to experience this unique landscape. Passengers can enjoy breathtaking views, visit picturesque villages, and explore Norway’s rich cultural heritage.
Northern Europe
This region includes destinations like the Baltic Sea and Scandinavia, offering a blend of history, culture, and natural beauty. You can visit historic cities like Stockholm, Copenhagen and Helsinki. These cruises often feature accessible shore excursions, including visits to palaces, museums, and scenic coastal areas. The combination of vibrant cities and stunning landscapes makes Northern Europe a fascinating destination for a cruise.
A cruise ship in Nordfjord - one of the Norwegian fjords. Photograph
by Steinar Engeland, Unsplash
The Disney “Wish” cruise ship at Port Canaveral, Florida, January 2024.
Photograph by Stephen Mease, Unsplash
Top Accessible Cruise Lines
Holland America Line: Known for its excellent accessibility features and it’s dedication to providing an accessible cruising experience for guests with disabilities. They offer wheelchairaccessible staterooms, ramps and lifts for boarding, accessible dining venues, and specialised shore excursions. Entertainment options include assistive listening devices and accessible seating. Service animals are welcome, and medical staff are available for assistance. Guests are encouraged to contact the Guest Accessibility Department and submit a Special Requirements Information (SRI) Form in advance to ensure proper accommodations.
Visit the website
Royal Caribbean International: Offers some of the largest and most accessible ships, with wide staterooms, accessible public areas, and entertainment venues. The largest cruise ship in the world is the Royal Caribbean’s “Icon of the Seas” which can accommodate up to 7,600 passengers. They offer a wide range of accessible activities for guests with disabilities, including adaptive sports and fitness programs, accessible shore excursions, and entertainment options with assistive listening devices and sign language interpreters upon request. The pools and whirlpools feature lifts or sloped entry, and the dining venues are wheelchair-friendly with accessible seating and menus available in large print or Braille.
Visit the website
Disney Cruises: Perfect for adults and children alike, featuring the Disney magic we all love and incorporating it into a fun cruise, filled with all your favourite Disney characters. Disney Cruise Line offers accessible staterooms with roll-in showers and grab bars and welcomes service animals. Guests can bring mobility equipment or request special items like shower stools. Entertainment includes accessible seating, assistive listening devices, and sign language interpreters for shows. Activities include adaptive sports, accessible shore excursions, and wheelchair-friendly dining venues with large print or Braille menus available. Pre-cruise planning with a Special Services form ensures all accommodations are in place.
Visit the website
Things to Do
Cruises offer flexibility and plenty of options to keep you entertained during your time on board. There are kids clubs to keep the little ones and teens entertained, not to mention the onboard spas and activity programs for the adults, there will be something you can enjoy.
Accessible Shore Excursions
Cruise lines often partner with local tour operators to provide accessible shore excursions. These excursions are designed to accommodate guests with mobility challenges, offering accessible transportation, ramps, and guides trained to assist passengers with disabilities. Examples include accessible beach outings, city tours with wheelchairfriendly routes, and visits to historical sites with ramps and elevators.
Wheelchair-Friendly Pools and Whirlpools
Many modern cruise ships have pools and whirlpools with lifts or ramps, allowing guests using wheelchairs to easily access the water. These facilities often include features like zero-entry pools, which have gently sloping sides, and accessible changing rooms with handrails and grab bars. A perfect way to cool off at sea.
Adaptive Sports and Fitness
Cruise ships offer a range of adaptive sports and fitness activities tailored for guests, including guests with disabilities. These activities can include wheelchair basketball, seated volleyball, and other adaptive sports that promote physical fitness and social interaction. Some ships also have accessible fitness centres with equipment designed for guests with mobility challenges.
Entertainment and Shows
The entertainment on a cruise is world-class and will include a whole host of choices for your tastes. Cruise lines provide accessible seating and assistive listening devices for shows, theatres, and other entertainment venues. This ensures that guests with hearing or visual impairments can fully enjoy the onboard entertainment. Some ships also offer sign language interpreters for select performances.
Onboard Dining
Cruise ship dining is an experience and offers a variety of choices, from elegant multi-course meals in main dining rooms to speciality restaurants featuring diverse cuisines like Italian, French, and Japanese. Buffet-style dining provides casual, flexible options, while casual venues like pizzerias and burger joints cater to quick bites. All options ensure all dietary needs are met. Bars and lounges offer drinks and light snacks with live music and themed decor, making dining on a cruise ship a delightful and varied experience for all guests. Dining onboard is an adventure in itself.
Photograph by Reynier Carl, Unsplash
Meet the Professional
In conversation with Bev Walley, Cheshire Community Nurse for Hope House Children’s Hospices
“Our purpose is to reach all the local children with a life-limiting or lifethreatening condition from the point of diagnosis, so that we can offer them our help as soon as possible. Our experience has shown that the majority of families we consult say, ‘We wish we’d come to you earlier.”
For many people the mere thought that their child might need palliative care is a deeply distressing one, perhaps in large part because there is a widespread belief that palliative care is always associated with dying. This is not the case, however, and palliative care can also describe the care and support offered to a child throughout their life to improve the quality of their lives. Palliative care aims to prevent and relieve physical, social, and emotional distress. While it does not only refer to care provided in the latter stages of life, it also encompasses this. This misconception can
The exterior of Hope House, photography courtesy of Hope House Tŷ Gobaith
sometimes mean that when a child is first offered a referral to their local children’s hospice their family might instinctively refuse it, because as Bev explains, “Some families think that I’ve been referred to a hospice, so therefore my child’s going to die.”
“There’s not a great understanding initially about what palliative care means with a lot of our families, that it’s
about creating happy memories, and offering respite, support and the other support services that we can offer families.” Part of the misperception around the role of children’s hospices might stem from the fact that they are still relatively new. It was only in November 1982 that Sister Frances Dominica opened the doors to
Glossary
Chronic: Describes a condition or illness that cannot be cured, but which is not progressive or lifethreatening
Palliative Care (children):
Describes the care offered to a child which aims to meet all their care needs. Palliative care can, or might be, offered from the point of diagnosis, throughout the child’s life, to their death, and does not only refer to care provided in the latter stages of life. Primarily focused on the enhancement of quality of life for the child or young person it might include pain management, management of symptoms, as well all the meeting any regular medical and personal care needs they might have.
Prognosis: Describes the expected or likely trajectory of an illness or medical condition
Life-limiting: Describes conditions or illnesses for which there is no known cure, and from which the child will (eventually) die.
Life-threatening: Generally describes any illness that will due to lack of effective treatments, severity or a progressive nature, put the child’s life in danger. Also refers to treatments where a cure might be possible, but in this case has not been successful (for example cancer)
Respite Care: In this context used to describe the temporary care of a child in hospice, offered to provide both the child and their regular care givers a break
Helen House, which is widely recognised as being the first children’s hospice, not only in the UK, but in the whole world. It was ‘the first building to be conceived, funded, designed and built entirely with the needs of the families of children with life-limiting conditions in mind.’ 1 Today there are 54 children’s hospices across the UK, with the demand for them growing over the years, as the number of children living with life-limiting conditions has also grown.
As part of our regular ‘Meet the Professional’ series we knew we wanted to focus on the vital work that is done within the framework of children’s hospices, as the role which they can play in the lives of those who access their services cannot be understated. When we approached the wonderful team at Hope House Children’s Hospices, they suggested we feature their
newly created Cheshire Community Nurse role, as it was created in large part to help improve the understanding of the support hospices can offer. “Having community nurses who are based in hospitals, and in the different clinics, will mean that they’re more visible. This means they will be more able to let families know about the range of support that’s available to them, and to encourage them to come and have a look around, to see what we do.”
“A typical day for me is looking at the visits I’ve got booked in for the day, and I’ll contact the families that are due for visits. Then I will look at the new referrals and make an initial assessment to see if the children fulfil the criteria for a hospice referral. There’s a significant number of children in the community who don’t quite meet the criteria, so if I feel that they are likely to be able to access a hospice I ring
1 Taken from the introduction to Hospices and palliative care for children: converging stories, linked here: www.academic.oup.com/bmb/article/130/1/81/5435787
A child’s bedroom, photography courtesy of Hope House Tŷ Gobaith
the families up and suggest I come out for a chat. I usually plan one or two visits into my diary because for most of those initial appointments I’m usually at the house for an hour and a half, or even two hours. It’s not a quick visit which is why I don’t do very many visits a day.”
During an initial visit Bev carefully explains “who I am, where I work, what I do, and the reason for my visit. I will gently establish what their awareness is of their child’s condition. Then of course we talk about all the different support that Hope House can offer.”
Hope House Children’s Hospices run two hospices; Hope House in Oswestry which opened in October 1995 and was the tenth children’s hospice in the world, and Tŷ Gobaith. Situated just outside Conwy in North Wales. The setting supports families from North Wales and is able to offer support to those whose first language is Welsh. Today the charity supports ‘more than 750 local families who are either caring for a terminally ill child, or whose child has died.’ Over the years the services that they provide have developed to reflect the changing needs of children and families.
Bev explains how important it is that families understand that “our support is tailor made to the families, and once they’ve been through panel and they’ve been accepted at the hospice the support for every family is different.” In order to
Ellison having fun with sensory play, photography courtesy of Hope House Tŷ Gobaith
access hospice support a family will need to go through the referral process. If the family she’s visiting are interested in receiving hospice support, then she explains both the referral and the panel process to them. Each family is asked to give permission for Hope House to contact their child’s medical team to request any relevant medical information be shared with them, this information is then collated and discussed at a meeting of professionals. The ‘panel’ is made up of the hospice medical officer, senior nurses and social work staff who decide whether a referral is accepted.’ Once a decision has been made the family can decide what support they feel ready to access.
“Some of our families, especially those who have been referred from the neonatal unit, don’t want respite support because they don’t yet really understand fully what their child’s needs are going to be. Very often parents are being quite brave, saying ‘it’s okay, we’ll manage.’ I’ll explain that there are other services that we offer. Some families might just want my support in the community and might not want to come to Hope House initially, but once they’ve been accepted by the panel, I’ll explain to them that they can have my support as a starting point, and I’ll often work with some of them for a few months. Then I might try to arrange to meet at the hospice, just to have a coffee. Then the next time I might suggest arranging for our family support worker to meet them and show them around, and they might stay
Hope House Therapy Pool, photography courtesy of Hope House Tŷ Gobaith
for an hour. They may then decide to try our weekly Sunshine support group. There they might get to know other families whose children also have complex needs. If, once they’ve been home for a couple of weeks or even months, it sometimes becomes clear they can’t manage twentyfour-hour care, seven days a week then they might decide to stay overnight with their baby to see what respite feels like.”
The hesitation to access hospice support is occasionally shared by families with older children too. “Sometimes I’ll go out and do a visit and have a chat about what the hospice can offer. Some families – particularly those with teens and young adults who have not previously been involved in a hospice – will say, ‘Actually, no, it’s not for me.’ However, I always leave it very open ended and explain that they can reach out to us at any time.” In the cases where a family does not want hospice care, or where they do not currently meet the criteria to be offered care, then Bev will still work with the family to identify any other ways in which they can be supported at home and in the community. For example, she might look at a “referral to the local social service Children with Disabilities for a child in need of assessment (which is different to a child in need of protection assessment) as it might well be that they are entitled to some social care.”
The view from Ty Gobaith, photography courtesy of Hope House Tŷ Gobaith
For the large part once a family visit the hospices, they are both reassured and impressed by what they experience. Although Bev carries a picture book with her that highlights some of the key areas in the hospice seeing it for yourself is different. “When people come and have a look around the services and facilities here, they truly are amazed at how wonderful it is.” Bev pauses and adds “and I’m not biased because I work here, it is truly a wonderful place. The feeling of love and comfort that you get when you walk through the door is so important. Everybody is so welcoming and friendly, and the families really do appreciate that. Many of them can’t wait to come back, and many of our children and young people look forward to coming as they really enjoy their time here. They can swim, access the sensory room, and the play team arrange a lot of fun and engaging activities. The child is supported by the care team, and families can do as much or as little of the care as they want to whilst here. Once the families have visited, I will call them to ask how it was, and they always reply that it was wonderful.”
The aim of hospice respite care is simple, to give families a much-needed break from their daily caring routine. Families can choose to stay on-site in the family accommodation, or to go home, and many families use the breaks as a chance to get away themselves. As well as respite care for the child the wider family can access Hope House, and not only the parents, but also siblings, grandparents, aunts, uncles and close friends can visit or stay at any time whilst their loved one is there. In addition, Hope House offers crisis care, providing a safe place for the children that they support if needed. They also offer the vital care needed before, during, and after the death of a child in the beautifully and thoughtfully appointed Snowflake suite. Hope House offers counselling as well as bereavement counselling, the death of a child is hugely difficult and emotionally challenging, and not all counsellors will have a breadth of experience that includes such a loss, but at Hope House the counsellors do.
Before joining Hope House, Bev had already worked within community paediatric nursing in a number of roles, including as the matron for the Children’s Community Nursing Team in Cheshire. “I’ve got lots of experience with children with significantly complex medical needs. We supported the children and their families for many years, both at home and in the community. We have also offered end-oflife care over many years, but more on a voluntary basis, rather than as part of a commissioned service. I was the palliative care nurse over in Crewe for quite a long time which significantly developed my interest in families whose children have had a palliative diagnosis.” So, when she saw the Hope House role, created as “part of a two-year pilot project to look at service provision in Cheshire for the children and young people that have a palliative diagnosis,” she felt it represented an exciting opportunity to be able to use her experience and her skills to help shape and deliver a better route into palliative care.
Over the years the team at Hope House saw time and again that for so many of the new families the referral for hospice support had been made far too late, and it is this that they are most determined to change. One of the key areas of Bev’s role is to help share the message that children’s hospices are not only for end-of-life care, but that they offer a wide range of services which support children and families throughout their lifetimes, as well as during the latter stages of their lives if needed. As Bev had already worked within Cheshire for so long, she was able to utilise these connections to introduce her role with Hope House, and to work on coordinating a more uniform approach to the signposting and/or referrals of families who might benefit from hospice support at the earliest opportunity. “By the time I meet a lot of the families now they are in crisis, they get to the point where they’re burnt out, or they can’t cope. I am finding a lot of families are like that, and that’s why when they can start coming to the hospice for support, they are so grateful, because they never, ever thought anybody was going to give them the help that they get here. It has the potential to be lifechanging for people and we want them to reach us before they break.”
About Children’s Hospices
As with many areas of healthcare there will be differences in both the language that is used, and also the provision and nature of support that is available within a national, and also local, context. In short, when we refer to a children’s hospice we are describing ones based in the UK, and it might be that such settings operate differently in other countries.
Myles loves ‘army crawling’ in the soft play room, photography courtesy of Hope House Tŷ Gobaith
There are currently 54 children’s hospices in the UK, the majority of hospices are established as charities and are run independently. In the main they rely on charitable donations to operate, but they do also receive some statutory funding from the NHS and central Government.
Children’s hospices provide support to local children with life-limiting or life-threatening conditions, and although the
services that each hospice offer may well be different, most will offer support to their families at the hospice, at home and in the community. Children’s hospice care is life-long supportive care for children with life-limiting conditions, and not just end of life care which is often what it is assumed to be.
For more information visit the charity Together for Short Lives here:
Visit the website
About Hope House
Hope House and Ty Gobaith hospices support seriously ill children in Shropshire, Cheshire, Powys and North Wales.
Find out more:
Photograph by Meruyert Gonullu, Pexels
into Fun
Winter often finds us hibernating in our cosy, warm homes, but spring heralds a new beginning. With the weather warming up and sunny days on the horizon, it’s the perfect time to explore the outdoors again. Engaging in outdoor activities with our children not only helps us to reconnect and to have fun together, also promotes physical and mental well-being. Here are some options for everyone to enjoy regardless of abilities:
• Accessible Parks and Playgrounds: Look for local parks that offer inclusive play areas, ensuring all children can join in the fun.
• Nature Walks: Explore nature trails and look for those that are wheelchair accessible if required. Many National Trust properties and local parks have paths suitable for children with disabilities.
• Sports and Physical Activities: Activities like swimming, horseback riding (including equine therapy), and adaptive sports such as boccia or wheelchair basketball can be great for physical fitness and social interaction.
Outdoor Activities for Kids of all Abilities
• Sensory-Friendly Activities: Engage in sensory-friendly activities like blowing bubbles, playing with sensory baskets, or creating nature collages. These activities can help children with sensory processing issues.
• Fruit Picking: Visiting a pickyour-own farm can be a fun and rewarding experience. It allows children to be active and enjoy the outdoors while picking fresh fruit.
• Arts and Crafts: Outdoor arts and crafts, such as sidewalk chalk drawing or creating nature collages, can be both creative and enjoyable. There are so many ideas for being outdoors and crafting, a quick search online can help you find something suitable for everyone.
• Community Events: Look for local events and activities organised by disability charities and organisations. They often have special days out and activities tailored for children with disabilities.
• Local Youth Groups: Groups such as Girl Guides and Scouts are inclusive and a great way to involve children. Activities are varied, and the social side of youth groups can positively impact mental health and foster growing independence.
Spring is the perfect time to get outside, explore, and make lasting memories with your children. Enjoy the fresh air, the warmer weather, and the endless opportunities for fun and connection!
Photograph by Leo Rivas, Unsplash
Empowering & Supporting the
Neuroendocrine Cancer Community
In conversation with Catherine Bouvier, CEO & Co-Founder of Neuroendocrine Cancer UK
photographs courtesy of Neuroendocrine Cancer UK & Catherine Bouvier
“The incidence of neuroendocrine cancer has risen 371% in the past 25 years.”
Catherine Bouvier is the Co-Founder and CEO of the charity Neuroendocrine Cancer UK. Their vision, “Of a world in which people know how to recognise, diagnose, treat, care for, and ultimately cure patients with neuroendocrine cancer,” has been at the heart of her life’s work. Twentyodd years ago whilst working as a nurse, she found herself in an office next to Professor Martyn Caplin, a gastroenterologist who had begun to see increasing numbers of patients with neuroendocrine cancers in his gastroenterology clinic. “He started to bring together different consultants to set up a clinic, which included an endocrinologist, a radiologist and an oncologist, and he asked whether I would come into that clinic as the nurse, that’s really where it all started. We didn’t have a dedicated clinic for this disease prior to that in the UK.”
The clinic grew rapidly, and year after year more patients were referred and Catherine found herself desperately trying to support an active caseload of over seven hundred patients. One of the biggest challenges they faced
was that the knowledge and information about what neuroendocrine cancer is, was not widely available at the time. “There was a lot of concern within the patient community about information and access to expertise and about what was really wrong with them. There was real confusion about the illness, and there were many unanswered questions around this disease, its origins, pathology and treatment pathways.”
Neuroendocrine cancer, also medically referred to as neuroendocrine neoplasm (NENs), is an umbrella term which encompasses a group of cancers that all originate in the neuroendocrine cells. These cells are vital in sending and receiving signals from
hormones to keep the body working properly. When cancer develops, it disrupts this process. “We refer to neuroendocrine cancer, and that reflects the aim to have it recognised as the cancer type that it is. There are still assumptions and misconceptions within the medical field and that can have a very detrimental impact on those diagnosed. Within the classification of the disease there is a clear split between neuroendocrine tumours (NETS) and neuroendocrine carcinomas (NECs).”
NETs will usually be found to be grade 1 or 2 during the biopsy, which means they will have a slow to moderate growth pattern. Whereas NECs typically are a grade 3, which means they will likely grow more rapidly. Previously tumours which were slow
to grow or did not grow at all were classified or described as being ‘benign’ and ‘non-cancerous,’ and those which grew more quickly were described as ‘malignant’ and ‘cancerous.’ However, as a paper on the MD Anderson Cancer Center website explains, ‘these terms did not always accurately reflect whether the tumour would metastasize and behave aggressively or not, so they have been abandoned. Today, almost all neuroendocrine tumours are considered malignant. There are just differences in how aggressive they are. Some spread easily. Others do not. But we consider all of them cancer.’ (10 things to know about neuroendocrine tumours by C Demarco).
Neuroendocrine cancer is often very challenging to diagnose. This is in large part because of the ‘silent’ nature of the growth of some neuroendocrine tumours, and because the symptoms can be similar to other more common conditions such as irritable bowel syndrome, menopause, asthma and/ or anxiety and therefore misdiagnosed. After our interview, Catherine shared some research that illustrated that ‘on average a patient will visit their GP eleven times before diagnosis’, and that ‘it takes an average of four and a half years for someone to be diagnosed
with neuroendocrine cancer from the onset of their first symptoms’ (see Basauroy et al. BMC Cancer 2018; 18(1):1122. Delays and routes to diagnosis of neuroendocrine tumours). These delays can have a devastating effect, and research has shown that ‘more than 50% of people will have advanced stage neuroendocrine cancer (stage 3 & 4) at the time of diagnosis’ (Genus et al. British Journal of Cancer, 2019. Impact of neuroendocrine morphology on cancer outcomes and stage at diagnosis: a UK nationwide cohort study 2013-2015). A grading system is used in the diagnosis of cancer, and although this varies depending on the type of cancer, it generally follows a scale from 1 to 4, with 1 being the least aggressive and 4 being the most aggressive.
The majority of those diagnosed will have neuroendocrine tumours, and as such they will often experience a very delayed diagnosis and once diagnosed are often initially misdiagnosed. “We have so much evidence around the different challenges in the route to diagnosis, broadly speaking these are twofold. The first is that as the person who’s got all these fairly general symptoms you don’t automatically assume that you’ve got cancer, and push for this to be investigated by your doctor because it could always be something else. I think our understanding of cancer
is that ‘I’ll know if I’ve got it because I’ll bleed from something, or I’ll have some other more obvious sign’ but cancer doesn’t really work like that. Secondly, even when you do go, your symptoms could potentially be diagnosed as something else. For example, some people are told ‘You’ve got adult asthma’ because of some of the wheezing that you might get associated with the disease if it’s in the lungs. Or ‘you’ve got anxiety’ or issues related to menopause or gastrointestinal issues and you know, it could be any of those. Honestly, it’s more likely to be one of those, but
the issue is that if a patient is continually returning with those same symptoms that cannot be definitively diagnosed – then there has to be a point where rare or less common diseases are considered.”
Diagnosing patients earlier is life-changing because there are treatments that can improve prognosis as well. Catherine uses an example of a patient they supported to illustrate this simply; “One patient was diagnosed with breast cancer, but in fact, that was a breast metastasis from her lung neuroendocrine cancer. She was treated
for breast cancer without them even picking up the lung neuroendocrine cancer, and so she not only underwent the completely wrong treatment, it also added an even bigger delay to her receiving the correct diagnosis.”
A smaller number of people will have neuroendocrine carcinomas or NECs, but overall, for this cohort, the diagnosis can be easier and made more quickly.
“The disease more obviously follows the pathway of a more common cancer, and so they will tend to be diagnosed because they might have some of those more red flag symptoms, i.e. pain, unexplained weight loss etc. This group of patients have a more challenging prognosis, a more aggressive type of cancer.”
For both NETs and NECs the treatment they are offered needs to deal with both the source of the primary cancer, as well as any secondary sites and the different and often debilitating symptoms and side effects they can present with.
“Across all cancer types, surgery is an optimal first-line therapy, because if you can get rid of the cancer then that has the best outcomes. However, because a number of our patients, up to 50% of them, will also have metastatic disease
surgery is often only an option for potentially removing their primary sites or for debulking the sites where there’s a lot of tumour. This is done for symptomology reasons, and to try and improve the symptoms for the neuroendocrine tumour group. Typically, chemotherapy and radiotherapy are not our first-line therapy, except maybe for some lung and some pancreatic patients. There are other treatments that we utilise, for example, molecular radiotherapy is quite a big treatment within our community, along with other more targeted therapies and treatments.”
In part, it can be this difference in the standard treatment protocols that leads to some confusion around the disease, to people feeling like they or their loved one don’t really have cancer as they are not necessarily receiving the treatments we associate so strongly with cancer. This ambiguity also tends to be linked to the two different diagnoses.
“Again, splitting out those two groups, many neuroendocrine cancer patients will not look obviously unwell. Many will definitely feel incredibly unwell depending on the symptomology, the pathology of the tumour, and the stage or grade. They might have to cope with dietary restrictions, symptoms
from over expression of circulating hormones, bowel changes, pain and fatigue. All of that will make them feel ‘more like a cancer patient.’ But there are a lot of people that will say ‘I don’t look like I’m a cancer patient’ and they are not always believed to feel as poorly as they do because those obvious visual signs of cancer are not there.”
It was this very mixed, confusing and often unnecessarily fraught experience that Catherine saw time and again in the first few years whilst working in and helping to grow the neuroendocrine cancer clinic set up by Professor Caplin. As is often the way, the need of the community far outstripped what the clinic could offer, so a small cohort of patients set up a small support group. “I’d go and see them, have a chat and just try anything I could do to help. It soon got to a point where they realised their remit needed to be wider and I agreed that we needed something a little bit more concrete for the patient community to access if they couldn’t get in to see or speak to their team. So, we started by writing some patient information leaflets on which we provided my phone number, we got so many calls that I asked a couple of patients to help answer them, to offer peer-to-peer support.”
A fundraiser hosted by a well-known snooker player, Paul Hunter, who
had been diagnosed with neuroendocrine tumours and who passed away in 2006, followed, after which they were able to set up a website. At the same time Catherine was seeing a huge increase in patients at the unit, many of whom had been misdiagnosed, given little to no information, or not offered the correct
treatments. “The majority of patients spend 90% of their time at home, they don’t spend it in the hospital, and who is helping them there? They are alone at home with no one to give them any answers, and it was so frustrating. I saw patients not receiving any treatment, or being wrong information, or being told time and time again ‘we can’t help you’ and so I took a leap of faith and left the clinic to try and grow this small charity,
which was associated with a specific hospital clinic, into a national organisation.”
Today Neuroendocrine Cancer UK is a wellestablished, national charity that is doing incredible work within the field. However, they receive no government funding, NHS funding or grants from the larger cancer charities. It is this that worries Catherine the most; “One of the scariest
things for me is funding our national helpline service, the amount of calls and the complexity of the calls that we’re now getting is almost unmanageable for us. There is such a lack of access for patients to their hospital teams, who are themselves overrun for so many different reasons, and we bridge that gap. But, trying to find funds to employ people is really hard and there needs to be some collaboration, we
need to be supported by the local UK government if we’re providing NHS services, which we are. The huge rise in the incidences of neuroendocrine cancer means that it is less likely to be considered ‘rare,’ and the central funding made available for research, for treatment and for support needs to reflect that, because the needs of the neuroendocrine cancer community must become a priority.”
Neuroendocrine cancer: Information collated from the Neuroendocrine Cancer UK website and the NHS website, the main resources are linked below.
Neuroendocrine cancer, also known as neuroendocrine neoplasm or NENs, is an umbrella term which encompasses a group of cancers that all originate in the neuroendocrine cells.
Neuroendocrine cancer encompasses a group of cancers that originate from neuroendocrine cells. These cells are vital in sending and receiving signals from hormones to keep the
body working properly. When cancer develops, it disrupts this process.
There are two primary types of NENs, NETs (neuroendocrine tumours) and NECs (or Carcinoids). NETs are characterised as ‘well-differentiated‘ tumours which usually have a gradual to moderate growth pattern. NECs (neuroendocrine carcinomas) are described as ‘poorly differentiated‘ and tend to grow more rapidly.
6,000 people every year are diagnosed with neuroendocrine cancer, that’s 1 person every 90 minutes (Reference: White et al. 2022;23: 100510 The Lancet Regional Health – Europe. Incidence and survival of neuroendocrine neoplasia in
England 1995−2018: A retrospective, populationbased study).
For more information visit:
Neuroendocrine Cancer UK
Cancer Research UKNeuroendocrine cancers
For information and support visit:
Neuroendocrine Cancer UK
Photograph courtesy of Amanda Riddle
Choosing a
Outlook
In conversation with Amanda Riddle
“Sometimes I’ll complain to my husband, and I’ll say something like ‘Well the doctors didn’t tell me about this side effect of the surgery or treatment did they?’ and he will reply ‘No they just told you they were going to save your life.’”
What really stood out when talking to Amanda is how positive she was, even when speaking to Amanda is how positive she generally is. Even when she reflects on some of the impossibly hard parts of her recent neuroendocrine cancer diagnosis, she can balance what has happened to her with gratitude that she is here today to talk about it.
Life for her has always been busy, raising two children who are now both in their twenties, and setting up and running her own successful learning consultancy business, which suited her perfectly. “I like a busy life, I’m very proactive, I love to get involved in things, and I love challenges. Keeping everything rolling is what I love doing best. I always believed that I was fit and healthy. I’ve never had any medical situations in
the past, I didn’t take any medication and really, I previously believed that I was going to live ‘til I was way over a hundred! I never had any aches or pains and never considered anything like this could possibly affect me in the way that it did.”
In August 2023 she started to experience a pain in her chest, which initially only really hurt when she breathed in deeply, so assuming it was ‘probably nothing’ and that she was ‘fine’ she carried on as normal. However, her sister-in-law, on hearing about the symptoms she was experiencing which by now included ‘a bit of backache’ as well, was worried and suggested that she suggested she ought to get it checked out. As it was the August bank holiday weekend she told her that she should go to A&E, a suggestion Amanda refused until her sister-in-law told her she was going to drive her there herself. “I really was going just to shut her up more than anything else, but also because I thought that perhaps it could be something. I thought maybe I’m having a heart attack so
I’ll go just to be on the safe side.”
Although he initially shared her concerns about going to A&E on a bank holiday weekend when they would doubtless be hugely busy, her husband insisted on going with her. “I drove, parked the car, and really, I felt no different at all, I just had this bit of pain. We walked from the car park to A&E which is not very far at all, maybe a ten minute walk but by the time I got there I couldn’t even breathe. I couldn’t talk, I was gasping for my breath. I don’t know what happened in that ten minutes but by this point I thought I was dying. I really, truly thought that.”
Over the next few hours, she underwent several tests, including an ECG, blood tests, chest X-rays and an MRI but they all came back clear. After seeing a few different consultants it was suggested she go home and see how she felt over the weekend, but she refused, and trusted her instincts, “By this point, I knew something was wrong.” What happened
over the following hours and days is a story that will be familiar to anyone who has had to wait in a hugely over-stretched A&E department. Impossibly long waits on hard plastic chairs in a freezing corridor until she was eventually placed in an assessment ward where, after reviewing the scans and test results again, it was decided that she probably had a chest infection so she was started on a course of antibiotics.
However, as part of their preparations to eventually discharge her Amanda was sent for another CT scan, and as the radiologist’s report differed from the initial report it was decided to keep her in for twenty-four hours just to be sure. The only bed they could find was on the infectious diseases ward, the prospect of which naturally deeply unsettled her. It was, however, to prove to be the turning point, as the consultant was, she explains, ‘absolutely amazing.’ “In the middle of the night, I had another incident where I couldn’t breathe, and when I saw the consultant he said ‘I don’t think this is a chest infection so I’m going to send you for more tests.’”
Initially, it was believed she likely had pneumonia and so needed to be kept in to be monitored, but then she experienced a return of episodes of actute diarrhoea, something she had been experiencing since the beginning of August but which had been investigated and no obvious cause found. When she told the consultant it seemed to cause him some concern, and more tests were run because as he said; “Something is not right, and I can’t put my finger on it.”
After a battery of additional tests, Amanda was eventually discharged home with a follow up inpatient appointment at another hospital scheduled to undergo an endobronchial ultrasound (an EBUS is a medical procedure used to diagnose different types of lung disorders, including inflammation, infections, or cancer.) Her consultant had explained “One of the CT scans has shown that you’ve got a mark on your right lung, and that’s what the second radiologist had seen. We think it’s actually from the pneumonia, but I want to just double check that” so she was not overly concerned. The test itself is a fairly invasive one, as a lung sample is taken, and when she underwent it a few weeks later she was advised that she would most likely feel unwell for several days afterwards.
Photograph courtesy of Amanda Riddle
When five or six days after the procedure she still felt desperately unwell she began to worry, and after advice from a consultant, she returned to the hospital. Whilst there she underwent another CT scan, and it was when the doctor came in to discuss the results with her that Amanda’s life was turned upside down. “She said ‘so you came a few weeks ago to have the biopsy, has anybody given you the results?’ I explained that they hadn’t. ‘She said I can give you the results. I just wasn’t sure if you knew or not.’ Then she sat there, and she just kept holding and stroking my hand before she finally said ‘So, just to let you know that the results have come from back from the biopsy and you have cancer.’”
Although both Amanda and her husband, who had accompanied her, were incredibly shocked by this news she recalls trying desperately to ‘keep it together,’ asking “What do you mean I have cancer? What does that actually mean? She said that I had lung cancer and I remember saying ‘But I’ve never smoked in my life’ because at that point I was so uneducated and I believed you could only get cancer in your lungs by smoking.”
The doctor explained to them that the cancer Amanda had been diagnosed with was called a neuroendocrine cancer, which is quite a rare cancer, and asked if they had any questions. “I asked her, ‘So am I going to die’? And then I cried. I was told that I’d be referred to a consultant who would give us more information and I was discharged. We came out from the hospital, and we just sat there, we were just absolutely dumbfounded, we had no idea what on earth was going on, what this meant or what was going to happen really.”
Amanda pauses, takes a deep breath and continues; “After that moment, one of the hardest things I’ve ever had to do was telling my children and my family. I don’t think I’ll ever forget that experience, seeing how upsetting the news is to people around you. The people that you love most in the world. It was so hard watching their heartache and I was trying as best as I could to say the right words and to remain positive, even when I was crying on the inside.”
Incredibly, according to information shared by the charity Neuroendocrine Cancer UK, the average time globally for a diagnosis of neuroendocrine cancer to be made is four
and a half years. One of the biggest challenges in diagnosing neuroendocrine tumours is that they can be present and cause a wide and diverse range of symptoms that could also be explained by other factors, or there can be situations where the patient is symptomless. It is unsurprising that such a delay can of course significantly impact both the prognosis and treatment outcomes for a patient. Despite the relative speed with which Amanda received her diagnosis, the
year which followed was far from easy on her.
Over the coming weeks a dizzying array of different scans and tests were conducted, when reflecting on that time she explains, “It was a real minefield emotionally, going from being well to suddenly going through this process, it was huge.”
The first consultant that they saw advised them that she would likely need
to have chemotherapy or radiotherapy, and because she had neuroendocrine cancer they didn’t feel they could operate. However, she was already scheduled to see another consultant, this time a surgeon, who advised the opposite. “We’re going to do surgery on your lung, we’ve looked at where the lump is and it’s spread to the lymph nodes.
Photograph courtesy of Amanda Riddle
We’re going to do surgery because the kind of cancer you’ve got doesn’t respond well to chemotherapy or radiotherapy, so the only choice we’ve got is to remove it.” The operation was booked for November and the family was advised
that she would likely need to have one third, or possibly two thirds at most, of her lung, removed.
Before the operation, Amanda felt worried that they might have to remove her whole lung but was reassured repeatedly that this was simply not going
to happen. On waking up post-operation, however, she discovered that it had, indeed, needed to happen. The impact this had on her in terms of both her recovery and health moving forward was of course huge. After a week spent recovering in hospital and some rudimentary physiotherapy, she was discharged home. At her follow-up appointment in December, she was given the results of the biopsy,
which had confirmed: “It definitely was neuroendocrine cancer, and because the whole of the lung had been removed, there was no fear of any other cancer kind following on from it, as the affected lymph nodes had been removed too.”
The following weeks and months were far from easy on her, she was readmitted a few times over Christmas with breathing problems and also contracted an infection in the womb. At an appointment in January, she was told they’d had a few areas ‘light up’ on her CT scan. “When I asked him What do you mean? What is a light up? Because I don’t understand. He explained that ‘a light up, put simply, are areas in your body in which your cells are not behaving normally.’ However, he went on to say that at a multidisciplinary meeting, they’d all agreed that as the light up was all around my chest area they believed that it was because of the operation that I’d had, it was the cells replenishing and so fairly normal, there was nothing to worry about.” So Amanda went home and focused on trying to recover and to get as fit and healthy as she could.
By February she was still struggling. “I didn’t feel fit to work, I was sleeping all day. I couldn’t breathe properly. I was lying up in bed and I just couldn’t do anything without being exhausted. I couldn’t dress myself, I couldn’t wash myself, I couldn’t walk very far. I’d gone from being a healthy, fit, mobile person to being really unwell, but I told myself I’d had this huge surgery and it would take time.”
Photograph by Drew Hays, Unsplash
At another routine scan and follow up Amanda was told that this time there had been light ups in her back and her right abdominal area. What followed echoed her experiences in the early weeks of her diagnosis, with a battery of tests, scans and appointments with different specialists until in May she was told that she had another tumour, this time in the abdominal area. Devastatingly a review of the original scans taken back in August the previous year showed that there were indeed two lumps, one in her lung and one in her abdomen, but because she was not experiencing any issues associated with it, it had been missed.
The weeks that followed were an utter rollercoaster of emotions, of highs and lows, caused in large part by the often conflicting information that they received following various tests and during often confusing and contradictory appointments with different teams. At one point she was referred to gynaecology as it was felt the tumour was not perhaps ‘neuroendocrine but just a lump.’ At the same time, she was advised by the thoracic team that “I’d got cancer in the lining of my ribs, in between my ribs and round my organs. I asked what that meant and he said ‘There’s absolutely nothing we can do, as we can’t operate.’” Just a week after this incredibly distressing news Amanda received a phone call to advise her
that, after another review, they thought that it was likely not cancer after all, but the after-effects of the surgery still. Any relief she might have felt was shortlived, as the rollercoaster simply did not stop.
The gynaecology team advised her just a few weeks later that “As it is not neuroendocrine it’s likely to be inside of your ovary, so we need to talk to you about what the possible options are for surgery. The first thing will be that we will diagnose where the lump is, if it is in the ovary it would be advisable to have a full hysterectomy, but if it isn’t inside of the ovary then it could be floating and we could just cut it out.” Before they could operate some more results were returned which confirmed that the tumour was in fact a neuroendocrine after all, and as such was more likely to be in my bowel. If that were the case, it opened up the possibility of Amanda losing part of her bowel, perhaps resulting in a stoma. It was decided to go ahead with the planned surgery but this time to diagnose and confirm what and where the tumour is, and if possible, to remove it at the same time.
In early October, she found herself having to prepare for another surgery without knowing for sure what the outcome would be. This time however the news was more positive, as the tumour was located on the outside of the ovary not inside, they only needed to remove it and the tube. A
few weeks after the surgery her neuroendocrine specialist explained that as the tumour had been removed the ‘light ups’ had been dealt with she would be discharged. When Amanda asked about the light ups found in her back/ chest he explained that as this was not an endocrine or cancer, but rather her body trying to repair postsurgery to remove her lung, she would be monitored by the thoracic team instead.
It is early December when we talk, and given the incredibly fraught year Amanda has had it might be hard to understand why in the introduction it is her positivity that is mentioned. Despite the harrowing story she has shared she has been upbeat throughout, showing an understanding of the untenable position in which the NHS currently operates, with services stretched beyond breaking point. When this is pointed out she explains simply “While it all sounds like a very grim story I try to never let anything get to me. I go to bed being grateful for my life. I get up in the morning being appreciative that I’ve made it until the morning and knowing what a wonderful day it’s going to be. I’ve always lived life to the fullest and been appreciative of everything. We need to understand that we’re all on a journey, and this is now my journey. I’ve gained lots of learnings from what’s happened to me, I’ve understood more about myself, I found more of me.”
About Neuroendocrine cancer
Neuroendocrine cancer, also known as neuroendocrine neoplasm or NENs, is an umbrella term which encompasses a group of cancers which all originate in the neuroendocrine cells. The neuroendocrine system is the system in our body which produces hormones, and although very rare neuroendocrine cancer can affect anyone, because we all have a neuroendocrine system.
There are two primary types of NENs, NETs (neuroendocrine tumours) and NECs (or Carcinoids). NETs are characterised as ‘welldifferentiated‘ tumours which usually have a gradual to moderate growth pattern. NECs (neuroendocrine carcinomas) are described as ‘poorly differentiated‘ and tend to grow more rapidly.
Only around 4,000 people are diagnosed with a neuroendocrine tumour each year in the UK.
Information collated from the NHS website and from the Neuroendocrine Cancer UK website, main resources linked here:
Neuroendocrine Cancer UK
Cancer Research UKNeuroendocrine cancers
For information and support for NETs visit: www.neuroendocrinecancer.org.uk www.netrf.org
For information and support for lung cancers visit: www.roycastle.org
Photograph courtesy of Amanda Riddle
5 Questions with
Craig Graham Owner and Head Coach at Alternative Movement
Please share a little background about why you set up Alternative Movement?
Alternative Movement came about when I discovered a hidden issue, and that was the lack of inclusivity within the fitness industry. After being a regular PT
All photographs supplied by Craig Graham & the Alternative Movement
(personal trainer) for 10 years I began my journey working with individuals with disabilities, and after many conversations quickly learned that prior to training with myself their experience within a gym setting had not always been a positive one. This was largely due to the
lack of knowledge that the trainers had, and the issues around accessibility that they experienced in the gym. After this I made it my mission to not only help those who needed access to appropriate training, but to also educate and inspire others to have the tools and knowledge to support
individuals with a disability on their training journey.
What are your top five tips that you typically share with someone who is nervous about getting started on an exercise and fitness journey, either for themselves or their loved one?
• Train with a friend - find someone who will go with you to the gym. Having support is super important, and a training buddy would do just that.
• Look for classes that you can attend. A lot of functional fitness spaces and group training facilities are now becoming more open to working with adaptive athletes, so should have a better understanding about how to adapt and scale workouts to work for you. Also having a supportive community that you find in a group training facility is worth its weight in gold, from helping to keep you accountable in your training efforts through to developing lifelong friendships.
• Look online - There are a lot more resources for adaptive athletes online around training now, and also don’t be afraid to reach out to someone with a disability that already goes to the gym. You’ll find that they are more often than not very happy to help, and to give you some advice.
• Ease into it - don’t feel you need to go in 100% to start with, start off slowly. Maybe once or twice a week initially, and then you can build from there. If you just improve by 1% each day it will still compound over the course of the year, helping you to achieve amazing results.
• Just get started‘never leave that till tomorrow which you can do today!’ Taking the first few steps can often be scary, but the longer you wait the harder it will become.
What is the change you would most like to see, or hope for, within the world of adaptive functional fitness over the next 10 years?
I would really like to see many more mainstream gyms become more inclusive, and for their staff to be provided with additional training, and the opportunity to work with people with disabilities over the next 10 years. I think a lot of trainers are initially apprehensive about working with individuals who might have different or additional needs and adaptions to consider then they’ve encountered before, this is largely due to their lack of understanding and knowledge around the subject.
Which achievement are you most proud of?
The biggest achievement I am most proud of is Wodaptive, a UK based competition that I cocreated with Nigel Barber and Ben Wadham. The aim was to enable adaptive athletes and able-bodied athletes to compete together in an inclusive competition. We are now on our 4th competition, and it is only growing. By having the ability to compete as a
pair we hope to give adaptive athletes the confidence to take that, often daunting, first step into competitive sports.
What do you do for yourself to switch off and recharge?
Spending time with family and friends is super important to me, so I try to switch off from work when I can so I can spend quality time with them. Also becoming a dad in the last year has been a big eye-opener about what’s really most important in life. Although, that said, when your hobby is actually also your career the gym can sometimes be a little hard to avoid!
www.wodaptive.co.uk
Rare conditions are individually rare but collectively common, with over 3.5 million people in the UK affected. There are around 7000 rare conditions, with new conditions identified regularly through scientific progress.
To mark Rare Disease Day 2025, Genetic Alliance UK is publishing ‘More than you can imagine: an anthology of rare experiences’ which captures individual experiences through creative pieces and demonstrates the impact of rare conditions on people’s sense of self, relationships, and wellbeing. The submissions pull on themes such as the impact on mental health, the experience of ‘fighting’ for care and feelings of isolation. They also capture the value of support and community.
Challenges in obtaining a diagnosis, barriers to accessing care and treatment, poorly coordinated care, as well as challenges with employment, education and local government support can all impact a person’s ability to manage their daily life.
The UK Rare Diseases Framework, published in 2021, sought to address many of these issues.The existing Framework has laid the foundations, and since its publication, new opportunities have emerged.
Illustration by Matilda Tumim - taken from More than you can imagine: an anthology of rare experiences, Genetic Alliance UK
Genetic Alliance UK’s report, ‘More than you can imagine: opportunities to improve the lives of those with rare conditions’ explores the progress that has been made under the existing Framework and identifies where gaps remain. The report calls for the Governments of the UK to renew their commitment to rare conditions and refresh
the Framework for 2026 and beyond.
The anthology and report will be launched at the parliamentary receptions taking place in Westminster, Holyrood and the Senedd around Rare Disease Day.
For further information, please visit geneticalliance.org.uk/ rdd25
ADVENTURES AFLOAT
www.disneycruise.disney.go.com www.hollandamerica.com/en/gb www.royalcaribbean.com
ALWAYS DANCING
www.childrenwithcancer.org.uk www.digital.nhs.uk www.northwestendactingschool. co.uk www.rtcw.org www.stagecoach.co.uk www.whenyouwishuponastar.org.uk www.younglivesvscancer.org.uk
BRIGHT FUTURES
www.childrenscommissioner.gov.uk www.childrenssociety.org.uk www.happymaps.co.uk www.hubofhope.co.uk www.mind.org.uk www.nhs.uk www.youngminds.org.uk www.youthaccess.org.uk
CHOOSING A POSITIVE OUTLOOK
www.cancerresearchuk.org www.netrf.org www.neuroendocrinecancer.org.uk www.roycastle.org
COSTELLO KIDS
www.costellokids.com www.costellosyndromeusa.org www.genomicseducation.hee.nhs.uk www.rasopathiesnet.org
EMPOWERING AND SUPPORTING PEOPLE AFFECTED BY NEUROENDOCRINE CANCER
www.neuroendocrinecancer.org.uk www.cancerresearchuk.org
LIVING RAVI’S DREAMS
www.brainstrust.org.uk www.nhs.uk www.ravisdream.com www.thebraintumourcharity.org www.youtube.com
MEET THE PROFESSIONAL
www.academic.oup.com www.hopehouse.org.uk www.togetherforshortlives.org.uk
RARETALES: SLEEPING BEAUTY
- AWAKENED RADIANCE www.dress-studio.co.uk www.karenmillen.com www.next.co.uk
THE DIFFERENT VERSIONS OF ME
www.childrenscancerfoundation.com.au www.nhs.uk
THIS YEAR I’VE DECIDED NOT TO SHRINK www.fndaction.org.uk www.fndhope.org www.necsociety.org www.necuk.org.uk www.pequenosgrandesguerreiros.org/en www.titusstrong.org
WHAT WE’RE INTO www.audioboom.com www.bbc.co.uk/iplayer www.coppafeel.org
5 QUESTIONS WITH CRAIG GRAHAM www.altmovement.co.uk www.wodaptive.co.uk
Supported by:
Connect with meaning and honesty
We produce powerful films, strong imagery & compelling narrative; creating a visual storytelling experience that allows you to connect more closely with your audience.
We work with organisations, agencies and publications that love to make an impact. We can join as a creative partner and help shape the creative direction into an impactful photography campaign or film, or we can work to your brief.
Helping you connect to your audience with integrity.
Creativity. Collaboration. Empowerment.
Find out more by visiting: www.sbdcreative.co.uk