What if we viewed lived experience as an asset? As an asset of professional, economic,…
#HereForThe49000 – A Blog for Together for Short Lives
It’s no secret if you know me that I have a life-limiting/life-threatening form of a condition known as Ehlers-Danlos Syndrome. Sadly, though some people with the condition can lead a normal (or relatively normal) life, for me it has caused multiple organ/multi-system failure (intestinal and bladder failure), Autonomic Neuropathy, Chronic Intestinal Pseudo Obstruction, Kyphoscoliosis, Osteoporosis, Hyperthyroidism and Sleep Apnoea, amongst other things, left me wheelchair bound but forced to spend a lot of time in bed, being fed into my bloodstream via a line directly into my heart (the intravenous feeding is known as TPN) and with a tube continuously draining my stomach and two stoma bags bypassing my failed organs. I am cared for by ITU nurses (and carers overnight) and my care is very complex and involved, and both my conditions and my care put many restrictions on my life. However that is not all I am as a person, I am more than just my conditions and the limits they impose on my life. I am a young person with wants, needs, hopes and dreams, I am a 22 year old who loves horses, my amazing assistance dog-in-training Molly, photography, writing, making videos, editing photos/graphic design and my beloved charity work.
I became ill when I was 14, after a lifetime of problems that were never explained nor connected. I became a wheelchair user, in excruciating pain and with a body that was slowly failing me – I’d battled for years to stay active and to live my life, but in January of 2008 I couldn’t carry on anymore, and things escalated from there. After much disbelief from medical professionals, I was diagnosed with EDS in October 2008, 4 days after my 15th birthday. Within a year I couldn’t eat or drink and was being fed through a tube, initially an NG tube (up the nose and down into the stomach), and then a PEG (straight through the abdomen into the stomach). Shortly after my bladder stopped working and I started intermittent self catheterisation. My gut was beginning to fail, and it didn’t move, so food, fluid and air didn’t move through my gut much, if at all, causing numerous symptoms including abdominal pain, severe bloating, nausea and vomiting and such severe constipation that no amount of medication helped – despite taking 5 times the recommended dose of some of the drugs. My bladder was (and still is) continuously infected. My autonomic nervous system doesn’t work and so all the automatic processes in my body don’t function normally, such as blood pressure, breathing, heart rate, digestion, temperature regulation etc.
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| Me in May 2011 |
By May 2011 my gut had failed completely, I was absorbing nothing at all, and so, after my new hospice nurse (who had only come on board weeks before) brought my case to the urgent attention of my consultant and the nutrition doctor he worked with, they immediately admitted me to hospital and put me on TPN, which is intravenous (into the bloodstream) nutrition – I weighed under 7 stone, dangerously underweight for someone of 6 ft 1. I was literally days away from things being too late, I was so malnourished and my body could’ve easily rejected the nutrition as I’d gone so long without any. I was skeletal. We’d even tried feeding directly into the bowel, but with no motility (movement) of my gut and no absorption, it was useless. A week later, having (very fortunately) tolerated the nutrition, I had a central line placed, called a Hickman line. This line enters my body on my chest, is tunnelled under the skin until it reaches my neck, where it enters the jugular vein, and then extends down into the central vein leading to the heart; the tip of this line sits just inside the heart itself. This comes with very grave risks, including potentially fatal infections in the lines which can lead to sepsis (also known as septicaemia or bloodstream infections), blood clots, and liver failure – the fat in the feeds damages the liver and can make it fail. I came home after 5 weeks, my mum having been trained to do the highly specialised aseptic (sterile) technique necessary to use the line – any bugs that get into the line can spread straight into the bloodstream, so the line must be accessed under strictly aseptic (sterile) conditions to prevent infections – so she could set up and take down my TPN feeds, and later administer IV medications and IV antibiotics when this was needed a year later. I stopped being able to have any medications via the tube into my stomach as they were useless with no absorption, I have to have ones that go IV into my line, or ones that dissolve under the tongue or in the gums but I struggle with these, or patch form, or if absolutely necessary via injection (I was once injecting 6 times per day), but I suffered infections and even pressure sores as a result of injections so this is not a viable long-term medication route. The tube into my stomach is now used solely for draining my stomach of the built-up acid and the bile that travels backwards up from my bowel into my stomach; without draining this tube I would be vomiting a lot.
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| My Ileostomy and PEG |
9 months later, in February 2012, I had an Ileostomy formed, whereby the end of the small bowel is brought out onto the surface of the abdomen and a bag worn to collect the waste. This helped, but I was still in and out of hospital on a weekly or fortnightly basis with pseudo-obstructions, whereby the bowel shuts down, giving all the symptoms of a bowel obstruction in the absence of a physical blockage. The intermittent self catheterisation I used to empty my bladder, also failed, and so in 2013 I had a permanent catheter called a Suprapubic Catheter was placed through my lower abdomen and into my bladder, but this caused nothing but problems, so in October 2014 I had a huge operation to completely remodel my urinary system. I had a section of small bowel cut free and used to form a tube. The tubes that come from the kidney were attached to one end of this ‘tube’, and the other end of the bowel ‘tube’ (called an Ileal Conduit) is brought out onto the surface of the abdomen (on the left hand side of my abdomen, whereas my Ileostomy is on the right) and a bag worn over this to collect the urine. I suffered a lot of complications, including a line infection which meant I lost my line – it literally is my lifeline, so I had to have a temporary central line put in to cover me while I was without my permanent line, and my wound (I was cut down the middle of my abdomen from above my belly button to my bikini line) split open and got infected. I was really poorly, but it was worth it as I couldn’t have continued like I was.
Lots has happened in between this; my spinal curvature has dramatically worsened, my spine is effectively crumbling, my bones are extremely thin (Osteoporosis), I have an overactive thyroid (Hyperthyroidism), I fell out of bed in hospital in July 2014 and badly broke my hip, which is now held together by a lot of metal screws and a plate, I’ve had a few line infections, my PEG (tube into my stomach) has a severe, antibiotic-resistant, treatment-resistant infection which we cannot get rid of and never will (but removing my PEG is not an option, so it’s something I must live with), I get repeated kidney infections and have had multiple bouts of sepsis from this (urosepsis) and so much more. Life is extremely challenging, and things can go downhill so quickly. My deterioration has been rapid over the last 7 years and 10 months of being ill. It’s almost hard to comprehend all the things that have happened in that time. It’s been an uphill struggle, and it’s pushed us to the edge, but we’ve always managed to fight back.
As I said earlier, I am much more than my conditions and everything they’ve put me through. For so long before palliative care my healthcare focused on my illness and trying to fix things that are, realistically, unfixable. I felt nothing more than a list of diagnoses and a bucket-load of symptoms. Then palliative care came on board. Suddenly I was a person, not a condition. Suddenly what I thought, what I wanted, who I am, my interests and hobbies and my goals for the future were central to my care; not just my conditions and symptoms. The hospice and palliative care professionals focused my quality of life, not trying to fix these incurable problems. I felt like a person, and every aspect of my life (and my mum’s and sister’s) was considered. I have a fantastic relationship with both of my hospice nurses. My first hospice nurse and I have a special bond though, and she’s the first health professional I fully trusted after some horrendous treatment in the early years of my illness.
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| My hospice nurse, Bev, and I |
My palliative care journey started when I was literally starving to death. The J’s Hospice stepped in, after a friend contacted Bev, Director of Nursing, and Bev immediately set to work – within a fortnight she was fighting to keep me alive, getting me admitted to The Royal London to start TPN, and has fought for me ever since, as does my new hospice nurse who recently came on board. I would not be here without them, nor would I have any quality of life. The hospice helped us work out an effective routine of pain relief and antisickness, and Bev was instrumental in getting a lot of medications prescribed, appointments arranged and even my surgeries done. I would not be alive without palliative care – people have a common misconception that palliative/hospice care is only about dying, but actually it’s about living well, quality of life and symptom control as well as having a good, comfortable death. I owe my life, and my quality of life, and all the things I’ve achieved, to palliative care.
We fought my illness completely alone until palliative care stepped in, navigating the complex and exhausting world of healthcare and different services by ourselves, we had no one to turn to, and no knowledge of what was out there – how can you fight for services and support when you don’t know that anything exists? It’s a scary world. Illnesses are very isolating, and you feel like you’re walking alone in the dark. It wasn’t until palliative care came on board that the light was turned on, and we realised we weren’t alone. Help was there. We just couldn’t see it.
My illness broke up our family; my parents split up when I became ill (which was for the best) and we all had to adjust to a new life. I had to give up all the things I enjoyed, adjust to a whole new medicalised, painful, difficult, exhausting, emotional, upsetting and traumatic world; mum gave up her life to save mine and keep me alive and at home, learning various specialist medical procedures necessary to achieve this, and to adjust to the fact that she will more than likely outlive me; and my sister had to adjust to the possibility of losing her sister, get used to having hardly any quality time with her mum, the loss of her dad from the household (and the country for that matter) and to the medical world she was thrust into as a result of my illness. It’s not been easy. I’ve nearly lost my life a few times now, and no one can underestimate the impact this has on a family and the individual themselves. Then when the bombshell dropped that my lifespan will be limited, and my condition life-threatening, it’s terrifying. You go through a grieving process. But we went through this largely alone; with an uncommon condition and an extreme form of it, people don’t understand. Prior to diagnosis, with a lot of disbelief and people insisting it was all in my head, it was hard for people around us to understand what was going on. And then I got the diagnosis of a condition that no one’s heard of, so the understanding doesn’t improve – you just have a name to throw at them, not that they will understand what it means. People couldn’t understand that I wasn’t going to get better and people kept saying “but you look well” as if you can’t possibly be ill if you look ok. I don’t blame them, but it further isolates you. Illnesses do break up families, and drive huge wedges between friends and relatives. It’s not easy on either part – they don’t understand, so hold things against you, or when you cancel more than one get together they stop inviting or including you, or they don’t understand that some days are better than others and accuse you of exaggerating/making things up, or they don’t make the effort to come to you when you can’t get to them; and you isolate yourself to avoid misunderstanding, hurtful comments (even if not meant to be hurtful) or to avoid the exhaustion caused by the effort of socialising. Illness is isolating, and it robs you of so much.
Palliative care recognises all the emotions, problems and struggles of life with a life-limiting/life-threatening/terminal condition, for the individual and their family, and supports you through them. Every aspect of your life is considered, from health needs, to emotional wellbeing, to the financial situation, to caregiving, to social wellbeing, to spiritual needs, right to bereavement support. It truly is holistic care. Palliative care doesn’t focus on the prognosis, it focuses on quality of life regardless of how long a patient has left to live. They help you to live while you’re alive, and then, when your time comes, to have a good death.
I achieved a good quality of life thanks to palliative care – most people would think I have no quality and wouldn’t be satisfied with my life, but I am. I do everything I can, squeeze everything I can out of life, within the restrictions my conditions and my care needs impose on me. It’s all about making the most of what you have. Through the hospice, I found a way to channel my energy and make myself feel useful. Through the hospice, I was put in touch with a charity called Together for Short Lives; TfSL invited me to work with them, my first project with them was to speak at their event in Parliament. If you knew me, and how excruciatingly shy I was, and how I hated being centre of attention, you’d have think you were imagining it. However yes, I agreed to take up the challenge and duly set about working on my speech. I spoke in Parliament on the 19th November 2013 – I haven’t looked back since, speaking at the Department of Health, speaking at and attending other events, appearing in videos and recordings, even on the radio, writing numerous pieces and blog posts, amassing 145,000 views on my personal blog, and making a difference in many ways – it gives my life meaning and purpose. I have worked with a number of charities, including the National Council for Palliative Care, Dying Matters, Council for Disabled Children, Essex Coalition for Disabled People, National Voices, Think Local Act Personal, Scope, Hospice UK, Transition Information Network, plus the Children and Young People’s Health Outcomes Forum. I work closely with the following charities as: Young Avenger for Together for Short Lives, the first Global Youth Ambassador for the International Children’s Palliative Care Network, Trustee of the Pseudo Obstruction Research Trust, Ambassador for Dreams Come True and Community Champion and End The Awkward campaign blogger for Scope. I owe this to Together for Short Lives, as none of this would be happening had I not been given so many wonderful opportunities by Together for Short Lives – I look forward to all the projects now and in the future, with all of my charities but especially with TfSL.
I do my best to use my knowledge and experiences constructively to benefit others, and I hope I have made my mark and made a difference. This is what palliative care has given me: quality of life, getting my symptoms under control so I can live a little, walk my assistance dog in training Molly, go out when I can, but also to go on and help others, write a successful blog and work with many different charities. Palliative care has given me hope too; hope that there is a life for me to live, a way to achieve, and a place for me in this world, because prior to palliative care I only existed. Palliative care has both saved and enriched my life. I am still supported by The J’s Hospice, a local young adult hospice.
I am writing this blog as part of Together for Short Lives’ #HereForThe49000. Together for Short Lives is the children’s hospice and palliative care charity for the UK, supporting both patients and their families and professionals, commissioners and service providers. There are 49,000 children and young people with life-limiting conditions in the UK, and Together for Short Lives works to support and improve the lives of and services for each and every one of them. The charity tirelessly campaigns, to raise funds for their own work but also to disperse it to all the children’s hospices, helping to change and improve services, work with commissioners and service providers and they provide a helpline to support families. They also harness the power, knowledge and experiences of patients and parents and involve them in campaigns and projects. I work with TfSL as one of their Young Avengers, the group of young people working with Together for Short Lives, including as part of their Transition Taskforce. The charity always needs more support and more funds to reach more families, make a bigger difference and improve the care, support and services for all the children and young people with life-limiting conditions (LLCs) and their families.
Together for Short Lives are #HereForThe49000 – are you?
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Your blog is at once heartbreaking and inspiring. And the fact that the only comment on here so far is from a spambot seems sadly indicative of your silent isolation. I heard you on Radio One and was moved to find out more. I hope your candidness has been rewarding for you
Have you heard of Professor Graham she's doing lots of research into eds as we speak and there is a big talk going on this year in the states for public to attend. There is also a french researcher involved also. I bet your mum has been stressed, awesome news on your MBE u deserve it!
loads of people have mis-diagnosed eds due to lack of information and lack of medical knowledge sad to say, after 9 years we were diagnosed only coz a Doctor had eds and linked everything together now after 9 years of in and out of hospital various consultants we are in "The System" and banging our heads against a brick wall. Blood pressure is a big problem also. Glad youn found the strength not to give up or give in and that you had people fighter your corner!
Message from a fighting the system parent arrrrh lol 🙂