Hi my name is Alex, I’m 45 years old and I live in Australia. I suffer from CIPO and GASTROPARESIS, and a few other co morbidities attached to those conditions. I first became ill when I was 11, and it all started with a swelling in my groin, a low-grade temperature and fatigue. I generally just felt unwell and my GP organised for me to see a gastroenterologist, as he suspected I was suffering from something more than just a bug.
The themes of Rare Disease Day 2021 – rare is many, rare is strong, rare is proud.
This is our story representing these themes.
Pseudo Obstruction (CIPO) is a rare and potentially debilitating bowel motility disease where the complications can be life threatening. PORT (Pseudo Obstruction Research Trust) is a small charity that was founded in 2006. It was founded in the memory of a beautiful little girl named Emily; whose life was cruelly taken by complications relating to this disease. Amidst this awful loss two friends, Emily’s mum Sonia and friend Sue founded PORT. They wanted to create a legacy for Emily, to fund research to improve the lives of others with the condition, and to support people with the disease so sufferers and their families wouldn’t have to go through the journey alone. Their initial determination to do something positive lives on in PORT today. PORT has a flourishing Facebook group. Although the disease is rare, many come together as a community, supporting one another and offering invaluable advice from lived experience. The closed Facebook support group really is an example of rare is many at its finest. People come together, of all different ages, parents with babies suffering from the condition, to adult children whose parents suffer with the disease, as well as many seeking personal advice too. From a small group that started in Essex, PORT now offers support to those all over the UK, and internationally too. It’s a forum to support each other through the difficult times, but also to celebrate the good.
PORT has also demonstrated time and time again rare is strong. The initial aim to fund research into this rare disease, still remains at the heart of the charity. Sonia and Sue showed incredible strength when they established PORT, and that continues today. Despite our families going through huge amounts, on a daily basis, the fundraising efforts have been and continue to be phenomenal. Right now, one of our trustees who has a son, Charlie, with the disease, has been intensively training for the London Marathon 2021. He had barely run before, but his determination to accomplish this challenge is nothing short of inspiring. His drive to complete the task has been matched by his fundraising efforts and the support of his wider friends and family, who know Charlie and what he and the family have to endure with the disease day in day out.
Rare is strong is lived out on a daily basis by all the adults and children with this disease. Getting up in the morning, and going about one’s day, whilst having to contend with severe pain, sickness and the countless medical procedures and appliances such as intravenous feeding, stoma bags and tubes, all demonstrate rare is strong. Not many people have to train to be nurses to receive their nutrition, or administer nutrition to their loved ones, or to be vigilant for the signs of sepsis due to having a central line into their bloodstream, but this is daily life for many sufferers of CIPO. And at the helm of PORT is the Chair Sue Stewart, who works tirelessly, with compassion and strength, to keep PORT going – rare is strong!
PORT might be a small charity run solely by volunteers, but the impact it has made and continues to make is undeniable. Over £750,000 has gone towards research. This is research that would not have gone ahead if PORT did not exist. This is just a glimpse into all the work that goes on behind the scenes for the research to happen. All the links and relationships that have been forged and all the research that has been scrutinised before being funded. For this we at PORT are extremely proud. We are proud of all that PORT has achieved, all the people that have been given hope, advice and information right through their journeys, from before their diagnosis right through to living with the disease. We are extremely proud of the amazing legacy that has been created in Emily’s memory, and all those that have gained strength through PORT.
PORT really epitomise rare is many, rare is strong and rare is proud and the following testimonials from members substantiate this too.
‘This charity has been an invaluable support platform, providing not only incredible contributions to research and treatments, but also providing sufferers and families with a voice.’
‘One of the biggest things for me is PORT has given me hope that one day a cure will be found, it gives me comfort that I’m not alone in this battle and it’s given me support and friendship when I’m feeling alone and isolated.’
‘PORT’s efforts provide hope that people living with these conditions might have a better future.’
‘Sharing stories, advice and well wishes with others is invaluable when you have a family member with a rare and debilitating illness.’
Isla is 9 years old and was born with a rare condition that has only been partly diagnosed. Her younger years were mostly spent in hospital, with her longest stay being 9 months. She has intestinal failure which is the umbrella of the diagnosis, which includes gastroparesis, pseudo obstruction, myopathic bowel disease. Due to this she is unable to eat or drink any food, as to put it simply her stomach is paralysed.
Ellie-Mai was born in 2011 at 42 weeks. She is my third child and I had an unremarkable pregnancy and straightforward labour. As soon as she was born I knew there was a problem. She was screaming, as if in pain, and would not latch on. The midwife insisted it was because of a quick labour. I was not convinced!
Sadie made a dramatic entrance into the world on the 10th October 2015 and has pretty much turned our world upside down ever since. We knew pretty early on that Sadie had trouble feeding, it was always a really stressful experience, she would scream in pain and go floppy and a horrible shade of grey pretty soon after it. My son had reflux and a milk allergy and so the medical professionals put a lot of her symptoms down to that.
Ollie (the happiest lad alive) was born on 28th August, after a 30 something hour labour, via vacuum due to low heart rate, and other labour complications. During prenatal scans we were made aware that he had a condition known as hydronephrosis and would be required to stay in hospital for a few days for monitoring, however it should be of minimal concern. Little did we or the prenatal doctors know, he had another on-going issue……
My name is Ruby Skye and I am 13 years old. I had symptoms of a motility problem from before birth as they noticed on scans that my bladder was very big. When I was born I spent a few weeks in intensive care but was sent home, and they thought the main problem was my ‘megabladder’ which didn’t empty so I was sent home with a catheter.
During Feeding Tube Awareness Week, we posted awareness posts on our social media accounts-Instagram, Facebook & Twitter. For many with Chronic Intestinal Pseudo Obstruction feeding tubes are one aspect of managing their condition. There are however many other reasons why people may need a feeding tube. We hope through the posting and sharing of our posts we helped to spread knowledge around this issue, and especially how people with CIPO are affected.
PORT has sent it’s newsletter, PORT DIGEST, out to members and is available online.
The first edition of PORT Digest is now finished and has been uploaded to our website. The newsletter contains lots of information, so please do have a read. There are articles about our current research projects and future projects in the pipeline too. Two members stories are also featured, and these share what it is like to live with CIPO. There are also articles about fundraising activities organised by and for PORT. The final page contains all the contact details and information about the charity as well as some useful tips.