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Saturday, 12 August 2017

International Assistance Dog Week 2017 - Lucy & Molly

So, last week (6th-12th August) was International Assistance Dog Week. IADW is an awareness week educating others about Assistance Dogs and what they do and celebrates how they transform lives. 

Molly and I have done all we can to raise awareness this IADW, through daily videos, a blog and media appearances. We've told our story and shared different aspects of our lives through our videos which were posted on our website and on Molly's Facebook Blog, I wrote a blog for the Huffington Post and we appeared on BBC Essex on the Ronnie Barbour Show and in the local Echo newspaper. 

We hope you have enjoyed it if you've followed us, but if not, here's a recap with all of our endeavours from this IADW. 

Click here to go "Dogs That Open Doors", my Huffington Post blog. 

Click here to go to our Echo Newspaper appearance. 

Our BBC Essex Appearance:

Our International Assistance Dog Week Videos:

Video #1 - Our Story & Assistance Dogs

Video #2 - Molly's Training

Video #3 - Molly's Tasks

Video #4 - A Day In Our Lives

Video #5 - Molly "on duty"

Video #6 - Molly "off duty"

Video #7 - Lucy & Molly's Awards & Honours

Echo News Article:

Monday, 31 July 2017

Charlie Gard: A Case With No Winners

Charlie Gard. A little boy whose care has polarised the world. A little boy with a severe condition called infantile encephalomyopathic Mitochondrial DNA Depletion Syndrome. He sadly passed away last week. His doctors for months had wanted to remove his ventilation and allow him to "die with dignity". Conversely, his parents wanted to take Charlie to America for one last chance at saving his life with an experimental treatment which hadn't been used in his form of Mitochondrial DNA Depletion before. I can understand both sides of the story; life is precious and we all want to think we've done everything for our loved ones to give them a chance, even when the odds are stacked against them. Charlie's devoted parents, Chris and Connie had raised £1.3million to take him to the US to exhaust the final chance of saving Charlie's life. Great Ormond Street Hospital (GOSH) did not believe the medication would have had a chance of working and that it would have prolonged his suffering, that he shouldn't be taken abroad for the treatment and his ventilation should be stopped to allow him to pass away, in receipt of palliative care to keep him comfortable. 

After a long-fought battle in the courts, between Charlie’s devoted parents and with Great Ormond Street Hospital, Chris and Connie accepted that the treatment would no longer work and agreed to let Charlie pass away, but sadly their wish to move Charlie home to spend some time with him before letting him go could not be fulfilled. They were outraged, but in some cases with such complex and high-level needs, children cannot always be moved home to pass away. In the end, Charlie was moved to a hospice and had a compassionate extubation (where his ventilator was switched off and the breathing tube removed) and was allowed to pass away, aged 11 months old, just a week before his 1st birthday. I have the utmost respect for Chris and Connie for their valiant fight for Charlie’s right to life, and to one last chance at saving his life and gaining some quality of life, no matter how slim those chances were. I also applaud Great Ormond Street Hospital and their staff for their courage to care for Charlie despite the deplorable actions of the public in relation to this case which included being harassed in the street, targeted and even death threats, which is completely unacceptable. The teams were doing their utmost for Charlie, ensuring he was comfortable, pain-free and that Chris and Connie could spend quality time with Charlie, no matter the outcome of the legal case. I do not believe it should’ve gone to the courts, but I understand why it did when Charlie’s parents could not reach an agreement with the teams at Great Ormond Street Hospital, a sad situation in which there never would be any ‘winner’. 

Every person has the right to life, including those of us with especially complex and life-limiting conditions. To take the position that Charlie’s condition inherently precluded him from any quality of life is incorrect. That said, Charlie’s tests did indicate that he was severely brain damaged and the team caring for Charlie and experts they brought in had the view that tests showed that, even if the treatment was to be successful, the brain damage could not be reversed. 

There’s no right answer in this case, no winner and the case has polarised the world and tested ethics to the limit. I wanted to share my story in relation to the Charlie Gard case to demonstrate some of the key points featured in Charlie’s short, but poignant life. 

I have followed the Charlie Gard case like many others through the press and the Charlie Gard #CharliesFight Facebook page. It has truly tested my own thoughts on life and death, because I saw the case for both sides of the argument. The right to life and parental rights, to the argument about quality of life and dying with dignity. It has made me think about my own situation and rethink things, in a way. I have felt torn about Charlie’s situation, because both sides feature arguments that are felt within my own condition and my own life.  

I am a 23 year old young adult with a life-limiting condition. I've been in receipt of palliative care for six years. I'm kept alive by the nutritional equivalent of life support, Total Parenteral Nutrition (TPN), which provides all my nutrition and fluid via a Hickman Line into my heart, because my gut cannot digest or absorb nutrition or fluid properly. Like Charlie couldn’t survive without ventilation, I wouldn't survive without TPN. TPN, whilst sustaining my life, comes with life-threatening complications including infections in the central lines through which the nutrition is administered into the heart, leading to bloodstream infections and sepsis (also called septicaemia), as well as blood clots and liver problems, amongst other complications. Also, the central lines required to deliver the TPN do not last forever - I myself have had eight lines in six years - and the lines do damage veins, meaning that over time access for lines can become problematic - and without central access, we can't have the TPN and thus we won't survive. TPN has kept me alive for over six years. It is the reason I am here today. I'm also dependent upon a tube draining my stomach, an Ileostomy and Urostomy. I undergo painful procedures and operations, including just last week to insert the third new Hickman Line in as many months. I suffer a tremendous amount as a result of my conditions and complications - I may even have a form of Mitochondrial Disease, albeit very different to Charlie's - and as a result of the interventions I require to manage them. Pain is a part of my life, I require opioid painkillers (Fentanyl), I'm hooked up to intravenous drips at least 21 hours every day, I suffer pain from the tube in my stomach which is infected, the Urostomy that bypasses my bladder has meant that the e.coli infection in my kidneys has flourished leading to kidney problems, my digestive system is constantly painful where it cannot get rid of the acid and bile it produces and I'm wheelchair dependent, forced to spend most of my time in bed and cared for at home by intensive care nurses. However, to me, my life is still worth living, despite all I go through. My condition is progressive and will shorten my lifespan and my quality of life restricted, but to me, I'm content with the life I have. There will come a time, however, where I have to sign that Do Not Attempt CardioPulmonary Resuscitation (DNACPR) form, which I'm having to contemplate, and there may come a time where we end up withdrawing TPN and allowing nature to take its course. We also have had to put things in place including my Preferred Priorities of Care (PPC) and my Lasting Power of Attorney (LPA). I have to face the fact that my life is finite and I have had to value quality of life over quantity of life. 

I’ve known I was life-limited since I was 17. I've had to do my end of life planning, which I am currently updating. 6 years ago when I first did my end of life planning, I didn't make any advanced directive or sign a Do Not Attempt CardioPulmonary Resuscitation (DNACPR, also called a DNR of DNAR), just ensured my wishes for where I want to be cared for in life and also at the end of my life were documented. I felt strongly in 2011 that I wanted to be resuscitated. I felt very much that I wanted to live for as long as possible, almost at any cost. In the 6 years since then an awful lot has happened and an awful lot has changed. 6 years ago doing that end of life planning, I didn't feel like I was life-limited. I was certain I'd far exceed any prognosis. I'd be the one to defied the odds. That I would live beyond young adulthood, into middle age, or even beyond. In my mind's eye, I didn't see my life being short. As such, talking about my wishes back then was easy, especially as we only did my Preferred Priorities of Care and Preferred Place of Death, not any advance decisions or refusals of treatments or interventions. In the last year, there's been an enormous change in my condition. The decline is evident. I feel like I'm on a slippery slope, I can see the decline in my conditions, my abilities, my energy and my pain and other symptoms. I can feel that my life will be cut short, my my mind's eye acknowledges that time is not something I will have in abundance and I won't live a long life and far exceed the prognoses I may be given. I was told I'd be lucky to get 5 years in 2012; I'm fortunate to have made it to this point. My hospice nurse and I have had some frank conversations, I know I need to get everything in order. I hope I have a life ahead of me for now, that I will keep going for as long as possible, but I have to prepare for my death. I also have had to accept the likelihood that my death will not be a slow and gradual process where I have time to prepare and slowly drift out of the world, I've been told that it's likely an infection causing sepsis that'll claim my life and that will be sudden. I have to prepare now, whilst I still can, for the occasion when my life will end. I'm having to do a lot of soul searching, talking to myself and asking what my true wishes are. I don't want to die, I'm terrified of dying because I don't want the amazing life I have now to end. However, I cannot live in denial. I have to face the fact that resuscitating me is probably a futile prolongation of suffering. My body, if it did survive a cardiac arrest, would likely come out the other side with a restricted quality of life and severe problems and repercussions. Do I want to live without quality of life? No. The answer is no. I want to live my life so long as I have quality of life - I've always been of the opinion of quality over quantity. 

The conversations I've had with my hospice nurse and my deep thinking, usually at night (as it is in writing this), has truly made me reconsider what I really want and what decisions I need to make about my treatments and care. I really should sign a DNACPR, purely as efforts would almost certainly be futile and if successful, the chances of having any quality of life if I survived is slim. However, I just don't feel able to sign that paperwork. It feels like I'm giving up on myself. I need to truly think about my chances, what my wishes are, what I define as quality of life and whether I would want suffering to be prolonged when I would have no quality of life? I need to seriously consider and face up to the DNACPR and/or an Advanced Directive. For example, my lungs are weakening. If I were to be intubated, it's not likely I would be able to be extubated. I need to make these decisions for myself and not leave my family with horrendous, heart wrenching decisions if I were unable to make decisions for myself. I am in the process of doing my Lasting Power of Attorney too, so that my mum can make decisions of my behalf if I lacked capacity. I've had accept a lot of painful truths and think about what is in my best interests, what is acceptable in terms of quality of life and make decisions to ensure my suffering isn't needlessly prolonged, that my wishes are respected and my family are not left with difficult decisions that they cannot consult me about. I need to accept the truth and to make every preparation I can.

The difference between myself and Charlie is that I am an adult and I can speak for myself and make preparations for future care. I can express my pain or my wishes. I can make decisions about my life. Charlie couldn’t. Charlie’s parents had to fight for what they felt was right for their son; a chance at life, no matter how slim the odds. I also understand the perspective of the medical professionals looking after Charlie at the world-renowned Great Ormond Street Hospital (GOSH). They felt, in Charlie’s best interests, it was kindest to turn off the life support on which he depended, as they feel he was suffering and that there was no meaningful chance of any improvement in his condition. Who is right? I for one don’t know. 

I have been appalled at the hate and negativity directed at Great Ormond Street Hospital, however. I understand the feelings on both sides of the ‘debate’ over what the right answer was in Charlie’s case. However, I also know for some, standing back and being objective when it’s so difficult, emotional and it’s your loved one who you’re being told should be allowed to pass away, is almost impossible. You’re too emotionally invested. It’s just a shame that a decision couldn’t be reached between Charlie’s family and the teams at Great Ormond Street and that it had to go to the courts. I understand the wish for Charlie to have one last chance at life, I think about myself and how I’d feel if I was told that my TPN would be withdrawn - a death sentence - and how my mum and sister and extended family would feel in that situation. Could they be objective? I am not sure. However, the difference is, my mum and I have had long conversations and she knows my wishes intimately and what I would and wouldn’t want. Charlie couldn’t communicate whether he was suffering or what he’d want to happen. All his family could go on was what they felt were in his best interests. All Great Ormond Street could go on was the scans and test results and the fact that the treatment had never been used in Charlie’s condition as well as the fact he had brain damage that they felt couldn’t be reversed, therefore they believed it was in Charlie’s best interests to extubate him and allow him to pass away naturally. What was in Charlie’s best interests? We will never have a concrete answer to that, as it relied on the opinions of those close to and involved in Charlie’s life and his care. Since an agreeable decision couldn’t be reached, it was one opinion versus another. We will never know what the right answer was. I do believe that the parents’ right to decide the treatment of their child is important and considering they’d raised the money to take him to America for treatment, the feeling of “why couldn’t they have one last chance?”. I also understand that Charlie was possibly suffering and that the kindest thing to do would be to let him go. I felt very torn by his case. 

One of my friends lost her daughter at Great Ormond Street in the same ITU after having to make a similar decision to Chris and Connie. She had to make the decision to turn off D’s life support. D had a life-limiting condition and had been supported by the hospice since she was 6 months old and by the palliative care team since she was 7. Her family had been forced to prepare, make decisions and accept that D’s life would be short. In the end, D’s mum desperately wanted to get her home to pass away, after she developed septic shock and there was no way she could ever overcome it. The requirement for this to be possible would be to extubate her, put her in an ambulance and get her home - but the likelihood would be that D would die in the ambulance on the way home. The same with the hospice. It just wasn’t an option. D’s mum decided to turn off her life support, surrounded by her mum and siblings, in the ITU. D’s mum said that, at the end, all that mattered was that D knew she was loved and was surrounded by her beloved family. They were together as the life support was switched off and D passed away. It was a tough decision for D’s mum to make, but at the end of the day, it wasn’t the location that mattered but that D was surrounded by the people she loved. She wrote a blog about this and I wanted to share the link with you, as I feel it is compulsory reading for those on both sides of the Charlie Gard case debate. Click here to read it. 

My message at the end of this that in cases like these, there’s no winners. The treatment of Great Ormond Street and its staff was not acceptable. I understand it is a very emotive situation and we all wanted what was best for little Charlie, but that does not excuse the behaviour that occurred. The most important thing in all of this was Charlie and his family. 

It's easy to imagine what we'd do in a certain situation, but it's different if you truly live it. I can only imagine the pain and heartache Chris, Connie and family have gone through and are continuing to suffer following the loss of their beautiful, precious little Charlie. My thoughts are with them. However my thoughts are also with the healthcare professionals who have looked after Charlie who've had much negative press. I extend my gratitude as a human being to those professionals for the care they provided Charlie; they, too, will feel the loss of Charlie as they do every patient they lose. 

Sleep tight Charlie. May you rest peacefully and watch over your mummy and daddy. They love you very much. 

Charlie Gard photos (c) of Chris Gard and Connie Yates. 

Saturday, 15 July 2017

My Stay At The Neuromuscular Complex Care Centre

Many of you will know, in November 2016 it was confirmed that I have some form of neuromuscular disease by the fantastic team at the National Hospital for Neurology and Neurosurgery (NHNN) and would be embarking on a long process to try and find a diagnosis. Dr Ros Quinlivan doubts my Ehlers-Danlos Syndrome diagnosis, rather than having ligament laxity, it is my weak (and some undeveloped) muscles that cause my joints to be lax. The neuromuscular disease is far more likely to cause the wide ranging, degenerative complications than EDS is, as everyone in my team have for a long time felt that EDS just didn't 'fit'. It could be possible to have both EDS and a neuromuscular disease, but it's unlikely - that'd involve winning the genetic lottery twice, I'm not sure I'm that lucky (or unlucky). We have a long diagnostic process to plough through, it is very likely I have a rare or even unique genetic condition; especially since nothing runs in my family. 

Since November when I saw Dr Quinlivan, I've been waiting for an admission for testing; this admission occurred this past week, Monday 10th - Friday 14th July. Whilst no diagnosis has been reached - it'll be a long process - the week has been productive. I thought I'd give a recap/update of the admission for those who are interested.

I had bloods taken for the 100,000 Genomes Project, to try and find the diagnosis. 
I also had some metabolic bloods too. 

I had some lung function tests, which showed my respiratory muscles are weak and my lung capacity is low and the force at which I can expel air is poor. I'm breathing in my upper chest and air isn't reaching the bases of my lungs. My cough is weak too. I've recently (pre-admission) been started on twice daily saline nebulisers to loosen up the secretions that build up whilst I am asleep, which combined with my weak cough are hard to clear. I've been given a Nippy Lung Volume Recruitment bag and mask to help the air reach the bases of my lungs. Here is a picture. Basically, I press the mask above my face, someone squeezes the bag as I take a breath in, then we air stack, which means I take little breaths as they squeeze the bag to force more air into my lungs to get enough air to reach the bases. I then remove the mask and breathe out or cough. This is to help air reach the bases of my lungs, forcing air in as I cannot naturally breathe in enough to fill my lungs, and I can then use that to mobilise secretions and/or to produce a better, more effective cough. That, combined with the twice daily nebuliser, should prevent chest infections by giving me a better clearance of my chest. I've also got to keep recording my cough volume to ensure things aren't deteriorating. 

I had a sleep study which showed my blood carbon dioxide level (PaCO2) does rise in the night but fortunately no big oxygen desaturation and the CO2 rise is not alarming at this point. I don't meet the requirement for needing non-invasive ventilation (thank goodness), so we can just keep an eye on things for now. 

I was reviewed by Dr Robin Lachmann, who said no metabolic diseases stood out but that a Mitochondrial Disease was likely. 

I'm already under Professor Qasim Aziz and have been since 2010 but they wanted me to see Dr Anton Emmanuel, the neurogastroenterologist based at UCL hospitals. He didn't have much to add, other than to restrict my fluid intake as it's making the situation worse with my gut. It'll be hard as I feel desperately thirsty 24/7 but if it'll help things then I will do it, as it's worth a try. He didn't have anything else to add, but he did educate his students on my "end stage Intestinal Failure". He was lovely and I'm glad to have met him. 

I saw the physiotherapist who, in addition to the respiratory interventions, gave me some resistance bands to try and maintain my arm strength or at least slow its decline. We won't build muscle but maintaining the strength I have now is the ultimate goal. She was really lovely, and did pick up on the Hypermobility in my joints. 

My heart is okay, my ECG is not too bad and echocardiogram shows nothing of much concern, which is fantastic.

Sadly, despite the team there trying hard, my muscle biopsy couldn't be organised whilst I was inpatient. They did their best. I just hope it won't be too long before we can organise it, as it will potentially lead to a diagnosis or if not, narrow down the possibilities. So the muscle biopsy will be a waiting game, but the Genomes bloods have been sent off so they'll be sequencing it and looking at that, but that's a very long process - like finding a single spelling mistake in a library full of books. It could take years, so that won't be a quick process.

I feel very positive. We've got management strategies in place for my respiratory problems, I've had some tests, things are moving forwards and hopefully the muscle biopsy will be organised soon. The stay was not bad, the Neuromuscular Complex Care Centre (NMCCC) staff are fantastic and despite some teething problems with TPN and things the admission went smoothly. I even managed to leave the ward twice to get out with mum, which was nice, a luxury I’m not afforded in most admissions as it takes so much time and manpower to get me out of bed and ready and to time in between IVs and everything, most wards can't allow me or facilitate me leaving but this admission we went out twice in the evening to escape the ward for a bit. I've met some lovely people too. So it was definitely a positive admission, and was step one on the road to a (hopeful) diagnosis. 

I am now home and reunited with my gorgeous companion and Assistance Dog Molly. 

Lucy x

Wednesday, 5 April 2017

Facing Facts; Still A Life Worth Living

On Tuesday 4th April, my hospice nurse Nicky and I sat down and talked the nitty gritty. We talked about how I am, what I've been up to, what's troubling me - the same as always. Then the conversation got serious. Nothing was said that I didn't already know, but boy did it hit me like a tonne of bricks. It's just confirmed they what I've been trying to pretend to myself isn't happening, is actually happening. I can't ignore it.

I've been putting on this persona for those around me, but also for myself. I lied. To you, to myself. Watered-down facets of the truth I have revealed to some, but by and large, most people think I'm in the prime of my (poorly) life. In terms of my charity work, I'm in the best place. I have wonderful family and friends, a great support network, I've got Molly my Assistance Dog and companion who is the light of my life and I have my purpose in life - which is to make a difference. What could be better? Lots of projects and opportunities, a great group of people around me, my wonderful dog Molly and a life that I enjoy living. Except, this is not the best time of my life in a health perspective. And I've been fooling you all to think otherwise. 

You all know I have life-limiting conditions - that's conditions which will, or could, shorten and prematurely end your life, often considerably so. We've known this for six years, that my life would be shortened. Initially there was hope I could live into my thirties or even forties, provided no additional complications set in - but, inevitably, they did. That was 2011; I was 17. In 2012, there was the revelation that I would be lucky to get five years. Well, I have reached that prediction. I’m now 23. That's an achievement. The sad fact is, however, that my visions of far exceeding those five years is unlikely. Sure I could have a year, two or three years, or more - but I could, and very well might not. 

In my minds eye, I've never accepted that I would have a short life - stupid when I was given only a five year prognosis. I just never 'saw' my life panning out that way, despite the timeframe given by my consultants. I always thought I'd have plenty of time. I've confounded so many expectations over the years, why couldn't I do so again? I'm not one to follow set paths, my life likes to freehand and follow its own unique path. My subtitle should definitely be "she does things in her own way". When have I ever followed the set path that medical professionals predict? So why should I resign myself to their five year prognosis? So I didn't. I carried on living thinking I had plenty of time, whilst in the back of my mind burying any fears about the prognosis. "Fake it until you make it" - boy have I been faking it! I just refused to accept it. 

However, over the last year, I've had to cope with rapid deterioration. My body, sadly, is giving up. It is struggling, it's weary, it's failing more and more and doesn't have the strength to withstand what it once could. Time is not on my side (though I will damn well live as long as is physically possible). So, Tuesday I had 'The talk'. I've breezed the 'life-limiting' talk, I've aced the 'you need to plan your end of life' talk, I've faced the 'you might not survive this' talk or the 'you can't have that because you won't survive' talk - but the conversation Tuesday was hard. Why? Because it made me confront the truth. 

My body is weak. It is tired. It is struggling. It will not be able to take much more - but we don't know how much, or how little, that is. We're at the end of the road in terms of my conditions and their complications, all we can do is cross our fingers and hope my body holds on and keeps going. I have four incurable infections, infections that do not respond to even the strongest intravenous antibiotics. For my pyelonephritis (kidney infection), we have two intravenous antibiotics left that work - but by work, I mean they dampen down the infection, they cannot cure it. These two antibiotics can only be given in hospital, but more to the point they can only be used when I develop sepsis (septicaemia), because if I become completely resistant to those two, when I next develop urosepsis, I will lose my life to it. So I can't treat the infection unless it's a life-or-death emergency - but the fact is, with my body as weak as it is, if I develop sepsis again, there's a high probability I won't survive irrespective of the antibiotics. The other infections I have, in my PEG and bladder, these are completely unresponsive to any treatments but they 'usually' don't make me systemically poorly. However, these are also taking a heavy toll on my body. 

Not only the infections, but my condition generally is not good. You see smiley, happy, bubbly Lucy at events or when you meet me, but please know, it's an act. I have fooled you. That persona belies the situation inside my body. My muscles are being eaten away at a rapid rate. My lungs are struggling more. My spine is a mess but the pain is not responsive to the forms of pain relief available because neuropathic pain requires different treatment and none of those treatments come in intravenous form. So, my back is extremely painful, I am never comfortable and no amount of opioids (in my case fentanyl) will change that. My back is always painful, but it's the worst it's been - and it's never usually painful if I am in bed. So to have my back agonisingly painful 24-7 is unusual. The neuromuscular disease (muscle-wasting/mitochondrial disease) is progressing at a rapid rate. It is eating away my muscles, including my respiratory muscles, which are getting weaker. It’s affecting the sensation in my body too. Things I once found easy are now impossible. It now takes two people to wash, dress and hoist me. Where once I could do things with relative ease, these are now a distant memory. A year ago I stopped being able to transfer from bed to wheelchair and back - an ability I had been fighting to keep for 8 years. So this last year has been a rapid decline, a scary, upsetting, frustrating decline. 

Six months ago, I would get up, walk Molly, get back into bed and then work solidly until bedtime. Gone are those days. These days I'm lucky to get one productive day out of four or five - a fact that is extremely frustrating for myself who is used to being busy and working solidly for hours at a time. I didn't have endless energy, but a good nights sleep would leave me mentally with a spring in my step so to speak. I was energised and ready to work continuously, and work hard. Now, some days refreshing Facebook and watching TV is all I can manage - and that's not 'me'. 

I've had to accept so much throughout my life. I've been on a never-ending grieving cycle since I was 11 when I started losing abilities, but grief is now intensified - grief that my limited time will be much more limited than I myself had envisaged and imagined and pictured in my minds eye. 

By no means am I giving up. Far from it! Nor will I give in. I have a whole life that I will fight to keep, my work is the best it's ever been, I've got so many wonderful opportunities and projects, I have wonderful family and friends and Molly of course, I have so much I want to do and things I want to achieve - this girl isn’t going down without a fight. I will fight to the death - literally and metaphorically. I've got so much to live for.

For now, I will be fighting to keep the life I have, to continue my work and to complete all the projects and things I want to do. I want to enjoy however long I have left and work hard to make my life the way I want it. I may have been reminded that we're at the end of the road with my conditions and complications - but there's "life in the 'ol bird yet". This is not the end of my story. All I can hope is that my body can keep going but I have to be mindful it might not. So, although I have for 4 years grasped life and done the things I wanted where possible, to just keep on as I am. Continue my work, enjoying life, doing things with my family and with Molly and living life to the fullest extent possible. 

There’s lots of things I want to do, but there’s also lots of things I need to do. As always, I am driven my my need, desire and ability to make a difference - however much harder it becomes to do that, I shall keep on doing it for as long as possible. I have lots of projects, lots of roles and lots of ideas and plans in the making. 

So please live life whilst you still can. Live life to the full, make the most of every day and cherish everything you have instead of focusing on what you don’t have or wishing for what someone else has. Life is what you make it. I hope I can not only inspire people to truly live and appreciate all the good, but to plan for all eventualities, including making a will, appointing a Lasting Power of Attorney who could make decisions for you should you ever lack capacity and making your wishes known (and yes, that does mean organ donation - why take your organs with you when multiple people’s lives could be saved or enhanced by them?). Yes, I know, we’re British and we don’t like thinking or talking about death, but, newsflash, life has a terminal prognosis if you hadn’t noticed. So get planning! Don’t wait until it’s too late.

Life is for living - and boy have I lived. I have a restricted life but I work within my limitations, push the boundaries and I never let myself be overcome by self doubt. I embrace everything life has to offer. I don’t think I’ve done too bad - an MBE at 22 and being presented that honour by HRH the Prince of Wales, other awards, a whole voluntary career culminating in seven permanent charity positions, being an advocate and Ambassador especially within the realms of palliative care for children and young people in the UK and across the globe, speaking out and helping to improve the transition period, raising awareness of charities and of issues that affect people like myself, a successful blog (over 275,000 views), improving my public speaking leading to paid jobs (starting as someone who had never given a speech before, and then in my maiden speech spoke at a reception in Parliament in front of over 100 eminent professionals, MPs and others), training my own Assistance Dog with help from Dog Assistance in Disability (Dog A.I.D.) and raising awareness of the charity, being invited to become a HuffPost blogger albeit only one blog has gone out (so far), appearing on TV and radio, writing for a variety of places, recently being appointed Project Advisor on a medical research project - and more, with many more plans, ideas and goals yet to fulfil. I have a great support network, wonderful friends and family and I dedicate myself to something that not only keeps me going but creates positive change for other people. I always felt the need to make a difference, to make my life count and to use my experiences constructively to benefit others, and I like to think I have done that. It’s my purpose in life. 

So please, if you have an illness or any adversity, I hope this shows you that you can achieve. You can contribute. You are important, wanted and worthy. Always strive to be the best version of yourself. I hope my story shows that there’s life beyond illness. 

If you haven’t - cherish what you have, appreciate all the things, and people, you have in your life, savour every day and please, plan for tomorrow - because tomorrow is never guaranteed. Life is for living - so live! 

Tuesday, 28 February 2017

Rare Disease Day 2017 - Do You Care About Rare?

According to Rare Disease UK, rare diseases affect 1 in 17 of us at some point in our lifetime, which translates to 7% of the population being affected by a rare disease during their life. This equates to 3.5 million people in the UK, or 30 million across Europe. With as many as 6,000-8,000 rare diseases, it's high time doctors accepted that 'rare' is more common than we think. The individuals with these conditions can be as few as a handful or even just one person, or affect as many as 20-30,000 people. 80% of rare diseases have a genetic component and 75% of rare diseases affect children. Rare diseases can be chronic and even life-threatening and life-limiting, and include conditions right from super-rare conditions like Field's Condition, only known to affect and named after Catherine and Kirstie Fields, ranging to conditions such as Fibrodysplasia Ossificans Progressiva (FOP), with 700 cases reported worldwide, as well as more prevalent conditions such as Cystic Fibrosis, affecting approximately 8,000 people in the U.K. 

So that's the statistics; rare diseases aren't as rare as you think! However, when you break the conditions down, rare diseases can affect tens of thousands, or just one or two people. 

I have a rare disease. You all know I was diagnosed with Hypermobile Ehlers-Danlos Syndrome when I was 15, and EDS Hypermobility is not a rare condition. Other forms of EDS, like Vascular, Kyphoscoliotic and Arthrochalasic EDS are. So Hypermobile EDS didn't make me rare, despite the misconception in the EDS community about it being so. Still, I have the zebra pattern stuff that is the mascot for rare disease, and has been adopted as the Ehlers-Danlos mascot. The saying goes in medical school "When you hear hoofbeats, think horses, not zebras."
Doctors are taught to think of common conditions (horses) rather than rare conditions (zebras), they are taught to think of the simplest and most common causes and conditions, rather than matching symptoms to rare diseases. Also, zebras stripes are never the same, much like our fingerprint, and this is said to represent how no two EDS patients are the same, just like a zebras stripe pattern is unique to them. 

However, my Ehlers-Danlos Syndrome diagnosis was questioned last year. I could not have EDS at all, or I have EDS as well as another condition, which explains the severity, complexity and progressive nature of my condition, and why it affects so many organs and systems. The neuromuscular specialist told me I likely have some rare genetic neuromuscular (muscle-wasting) disorder, or a rare form of Mitochondrial Disease. Finally everything made sense, why I was born with health problems, I was born with a dislocated hip, had severe silent reflux, a floppy airway that would stop me breathing if I was laid flat and severe constipation. I had problems throughout my childhood, many problems, septic arthritis from the undiagnosed dislocated hip, flat feet, constipation, tummy pain, weakness of my muscles, Hypermobile joints, joint deformities, eye problems and more; but no one connected the dots, I saw different people for different problems and no one thought it was odd. I started physiotherapy age 11, and my physio couldn't understand how I was very physically active, horse riding most days, working at the yard mucking out, sweeping, carrying heavy objects like hay nets, hay bales, feed bags and water buckets, grooming the horses, doing lots of walking, pushing wheelbarrows and the actual riding itself. I was doing this almost every day. Yet, my muscles were weak, some completely undeveloped and they couldn't support my joints at all. It made sense why I was falling a lot from my legs giving way and struggling to write, to walk, I couldn't run and why I was struggling to keep up with my active life, needing more and more help. I struggled to write, I had to go up and down stairs on my bottom, I couldn't get up off the floor, and I needed help to wash my hair. Not only were my muscles weak, but despite religiously doing my physiotherapy exercises, my muscles were getting weaker and not stronger. So despite how much I was doing and how hard I worked on my physio, my muscles were getting weaker still. It didn't make sense and my physiotherapist regularly took my mum aside and said he knew something was wrong, but he didn't know what.

In January 2008, age 14, I became wheelchair dependent. I got my diagnosis of EDS a few days after my 15th birthday in the October. I continued to deteriorate though. I became bed bound in 2009. My gut failed and I started tube feeds in August 2009, was diagnosed with Chronic Intestinal Pseudo Obstruction and POTS in 2010, started having to catheterise to empty my bladder in 2010, went onto TPN (intravenous nutrition) in May 2011 after nearly dying, having a heart attack from my body digesting my heart muscle and my bone marrow starting to fail.  In 2012 I had my Ileostomy formed, in 2013 I had a permanent catheter (suprapubic catheter) placed, in 2014 I had a Urostomy (Ileal Conduit) formed and as such no longer had the suprapubic. I now have a collection of secondary conditions and complications: complete failure of my digestive system (type 3 Intestinal Failure), neurogenic bladder failure, severe problems with my Autonomic Nervous System (Autonomic Neuropathy and POTS), as well as problems with my bones (Osteoporosis and early-onset arthritis), spine (Kyphoscoliosis, spinal degeneration and others), lungs (Restrictive Lung Disease and gas exchange abnormalities), metabolic and endocrine problems, immune system dysfunction and problems with my kidneys. My muscles are progressively atrophying too, they're just wasting away.

Knowing why I am the way I am and finally understanding why Ehlers-Danlos Syndrome didn't cover all my symptoms and complications or the progressive nature of my condition, has been a huge relief. And the new 'diagnosis' of sorts has been agreed by all my specialists to make sense. They had their concerns but just referred to my condition as complex or atypical EDS. Now, it all makes sense, but it took this fresh, expert pair of eyes to comb through my life and see what others had missed all these years. I am 23 now, and it's taken all that time to know what's wrong, albeit I don't yet have a diagnosis, we know what type of condition we're dealing with. I will be undergoing a whole barrage of tests, including a muscle biopsy, as well as an inpatient stay, and I will be recruited onto the 100,000 Genomes Project to try and find the cause of my condition should the other tests not find the cause of my condition. I am desperate to know because I want my sister to be tested; I don't want her future children to end up like me. 

I am life-limited, my conditions will shorten my life expectancy considerably, but I try to make the most of every day. I am grateful for all I have, and that I live in a country with a free-at-the-point-of-use health service. I am very lucky. I am kept alive artificially by my TPN (feeding into the bloodstream) and the fluid and medication that gets pumped directly into my heart. I have expert interventions, a venting gastrostomy, an Ileostomy and a Urostomy. I have a care package, albeit far from perfect, that enables me to live at home, and I have a large team of specialists who fight to keep me alive and to give me the best quality of life that I can have. 

Knowing I have a rare disease, and that the condition that has become a part of my identity, I might not have at all, has been a confusing new direction in my life. EDS is so ingrained in who I am, it's almost like a badge of honour, but now I might not have it at all, and knowing, even if I do have EDS, it is not the cause of the majority of my complications, has taken a lot to get my head around. However, it's a relief to know why I am the way I am. I still get to wear my zebra gear, but it represents a different condition now. My rare genetic or Mitochondrial Disease. 

Today is rare disease day - do you care about rare? Please show your support if you can. Rare is everywhere, we just don't realise. 1 in 17 of us will experience a rare disease at some point in our lives; it can happen to you. So please show you care about rare, and support all the people affected by a rare condition like me.

Thank you.

Monday, 13 February 2017

For The 49,000 - House of Commons Reception for Together for Short Lives

On Tuesday 7th February 2017, I attended my third event at the House of Commons for Together for Short Lives, with the theme of #forthe49000. The event was hosted by Stuart Andrew MP. A fantastic day was had by us all. I attended along with my Assistance Dog Molly, my mum, my nurse and my Uncle Ray who drove us. We had a very enjoyable day, got plenty of photos, and I got to see lots of friends and people I know through my work and the charities I am involved with, as well as to meet two other Young Avengers (young ambassadors) who I know through working with them on projects, but had not met in-person until Tuesday. 

This year's theme was the launch of the All Party Parliamentary Group for Children who need Palliative Care, as well as to propose seven key areas for the Government to invest in children's palliative care. The event was well attended, with parents of children with life-limiting conditions, young adults with life-limiting conditions and hospice and palliative care professionals being joined by over 50 MPs and Peers.

Barbara Gelb OBE, CEO of Together for Short Lives, proposed the following calls that she asked of the Budget to invest in children's palliative care:
  • Adequately fund children's palliative care to meet demand.
  • Giving young people dignity and respect - bridging the cliff edge in care between children's and adult services.
  • Supporting Managed Clinical Networks (MCNs).
  • Ensuring nursing care today and tomorrow. 
  • Preventing family breakdown.
  • Lifting the baby benefit bar.
  • Care and compassion in bereavement.
(c) Together for Short Lives
At the event, there was a display showing the amount of equipment needed by many under threes with life-limiting conditions, including supportive car seat, specialist buggy, oxygen, ventilator, gastrostomy supplies and feed, medication, suction, spare supplies and more. These children desperately need an adapted vehicle in order that they can leave the house, but the baby mobility benefit bar means they cannot claim the mobility component of Disability Living Allowance until the child is three. This leaves families trapped at home as they cannot claim the DLA mobility component that would allow them to fund a specially adapted vehicle to accommodate the child's needs and equipment. Some of these children will not even live long enough to claim the benefit. It needs to be lifted that young children with complex, intensive, specialist needs can receive Mobility DLA and fund a specially adapted vehicle to allow them to leave the house, enabling them to attend appointments and meetings, but also have quality of life for the whole family, enabling them to leave the house for outings and days out. It's vital the baby mobility benefit bar be lifted - please support the campaign. Other areas covered included improved funding for and access to short breaks, vital to give carers a break from caring, to allow them to rest and recharge their batteries, and to enable family time with other children or as a couple without the child's care needs to worry about or work around, as well as improving the ‘patchy’ care and the postcode lottery when it comes to the funding of children’s palliative care and related services. 

Parent Rachel Thompson shared her story, as her son, Frank, has Late Infantile Batten Disease, a rare, incurable, neurodegenerative condition, robbing Frank of his mobility, his speech, his sight and his ability to swallow and eat safely. He is immobile, suffers from dementia, is fed through a tube and needs assistance with breathing - his care is 24/7, 365 days of the year. He has a life expectancy of between 6 and 11 years old. Frank and his family are supported by a children's hospice, where care is for the whole family - support for Frank and his parents and his siblings. Frank's story epitomises what palliative care is; looking after Frank and the specialist, complex nursing needs Frank has, quality of life and quality of care, preventing unnecessary hospital admissions and the ability to step-down from hospital to the hospice, support in accessing equipment and services, support with fighting for care packages, care around the clock, someone they can call upon at any time for advice or support, the desperate need for - and benefit of - respite/short breaks for the parents, care for the whole family from diagnosis and which will extend to bereavement care when the time comes, quality end of life care when needed - a whole-family approach to care. Rachel's story was very emotional and shows the importance of the work of children's hospices and paediatric palliative care, and of the work of Together for Short Lives in representing the 49,000 children and young people with life-shortening conditions in the U.K. 

It is an honour to be a part of Together for Short Lives, who I have been working with for almost four years now. Parliament holds a very special place in my heart because it is there that my charity work all started, when I gave a speech for Together for Short Lives - my first speech (ever) and the first bit of charity work I did - on the transition period between children’s and adult services. I was one of the original Young Avengers and became part of their then-newly formed Transition Taskforce. It was wonderful that I got to meet two fellow Young Avengers (young ambassadors), Junior and Leah. Junior and I have crossed paths so many times with our work, both through Together for Short Lives and the Open University Sexuality Alliance (OU SA), but we had never met face-to-face before. 

Molly was a little star at the event - well, a big star. She was the Belle of the ball, getting lots of fuss, in fact she was more in-demand than I was! She gained many friends and admirers at Parliament. She did me so proud, and her tail did not stop wagging the whole way through the event. She certainly was a big attraction, and just lapped up being centre of attention. In true Molly style, she thought every round of applause was for her! She was such a good girl doing exactly as I asked, and was very taken with Junior and Leah, doing paws-up on their wheelchairs so they could say hello. 

All in all, it was a fantastic event, it was great to catch up with the people I have met along the journey with my work, highly influential people, and those who have had a big impact upon my work, and this time, for them to be able to meet Molly too. I am so fortunate to have been invited to Parliament for the third time and to have so many wonderful experiences. 

Also, Baroness Campbell of Surbiton (Jane Campbell), a crossbencher, popped into the event. I only briefly spoke to her, and she gave me some words of advice that will ring in my ears in future. She told me not to let professionals dictate campaigns, decisions and how things are run because they don't have experience; we, as disabled people, must be the ones steering campaigns and speaking up. I will always remember this - because she is right, it is us as disabled people who must speak up for ourselves and campaign for our needs and rights. If you don't know about Jane, I suggest you read her story on her website. She's an amazing woman who was instrumental in the campaigns that led to the creation of the Disability Discrimination Act 1995, and has campaigned, run and managed charities and organisations including being a Commissioner in the now-defunct Disability Rights Commission. She is currently a crossbencher in the House of Lords. She is an inspiration to me in many ways, not because she's disabled, but because she used everything she has to advocate, create change, campaign and make a difference. She is a role model to me, she shows me that anything is possible if you work hard, and shows how powerful our words and actions are if we use them to the fullest extent. So to be able to speak to her, albeit briefly, and to have her words forever in my mind, was a massive boost. I will go forwards with renewed determination. 

Friday, 20 January 2017

Lucy & Molly - BBC Essex Interview (Dave Monks Show) - 19-01-17

Here's the video from our interview on the Dave Monks Show on BBC Essex. 

Thursday, 19 January 2017

Molly Awarded the 'Animal OBE', the PDSA Order of Merit

In October 2016, on a sunny, day, we had a very important visitor. Jan McLoughlin, Director General at the People’s Dispensary for Sick Animals (PDSA) came round to present Molly with a very prestigious honour. In the same year as I received an MBE in the New Years Honours, Molly was awarded the animal equivalent, the PDSA Order of Merit. She became only the fourth dog to receive the award. It recognises her outstanding devotion to me, as my companion and as my Assistance Dog. Below is the video of the presentation, and click here to go to Molly's award story on the PDSA website. 

Molly has truly changed my life. She came to us as an eight week old puppy, and transformed my life, from one spent in bed except for when I attended hospital appointments, to getting out and about and having a life again. Once we got Molly, I was getting out to walk her every day. Not only that, people who were previously too scared to talk to me were striking up conversations with me about Molly. I was also gaining in confidence, too, and so would start conversations with fellow dog walkers. My life was completely changed, from one spent trapped in the four walls of my room nearly all of the time, to getting out every day, and having a life again. I was taking Molly to dog training classes every week too, meeting new people and having fun, for the first time in a long time.

The confidence Molly gave me led directly to me accepting to speak at a reception in Parliament for Together for Short Lives. Despite Molly not being there with me, where I had been getting out, meeting people and rapidly gaining in confidence as a result, it meant that I had enough confidence to give the first speech I’d ever given, in Parliament, to around 100 people comprising MPs, charity representatives and eminent hospice and palliative care professionals. It was a huge success, and I have not looked back since. I currently hold 7 positions within charities, have written blogs, articles and forewords, have given a number of speeches at events and meetings, appeared in and presented charity videos and appeared on TV and radio. My life has been taken a direction I could never have foreseen or ever imagined.  My charity work, which happened as a result of the confidence Molly gave me, led to me receiving an MBE in the 2016 New Years Honours for services to Young People with Disabilities. I would not be Lucy Watts MBE if it weren’t for her.

However, Molly had an even more extraordinary destiny than to just be my pet. When she was ten months old, Molly was assessed and then began training with Dog Assistance in Disability (Dog A.I.D.) to become my Assistance Dog. Dog A.I.D. help disabled people train their pet dog to become their Assistance Dog, by pairing them with a volunteer trainer who helps them to get their dog to the required standard and to pass the three levels of training. Molly and I thrived on the training, I loved the fact I was training her myself with help from our wonderful trainer Midge, and things were going so well. Molly and I won Friends for Life at Crufts 2014, being voted winners by the public, and Molly was awarded a Dogs Today Medal.

Despite lots of setbacks and time off with my health, Molly and I passed the Level Three assessment and gained Full Assistance Dog Status on the 6th September 2016, so Molly can now come everywhere with me and assist me, and we have our ID booklet and Molly has her posh working jacket. Molly makes such a difference to my life, giving me confidence and allowing me to be less dependent on those around me, and I love having her beside me as I go about my life. She can completely undress me, including my hat, scarf, gloves, coat, jacket, top, trousers, underwear and socks. She fetches the post, picks up dropped items, fetches named items, passes notes between me and my mum or nurses, fetches help, loads and unloads the washing machine, closes doors and will pull my blanket off my lap if I get hot, amongst other things. Molly also alerts me three to four hours before my temperature spikes, giving me a vital warning that I will become poorly, since I don’t get signs or symptoms of an infection until I have developed or am scarily close to developing sepsis so the warning gives me time to act. She wasn’t trained to do this, it is something she does naturally.

I love having her beside me every day, making me less dependent. She is a welcome guest at my charity events and meetings, people love having her there, and for me I feel less like a burden. If I get hot, Molly can take my coat or jacket off, or pull my blanket off my lap, without asking someone to help me. If I drop something she will automatically pick it up, saving me from asking my nurse to pick things up time and time again, leading me to constantly apologise for dropping it. For Molly, all of this is a wonderful, fun game, she loves doing it, so I don’t feel bad when I drop something for the twentieth time, or I suddenly get hot and want to take my jacket off. She makes me feel less of a burden.

Molly changed my life, and is the light of my life. She was the only reason I kept going when mum was diagnosed with a brain tumour and suffered a stroke after the operation, she is my focus to keep going when I am poorly or in hospital, and when I am feeling down, she is there bringing comfort, company and I can stroke her and she will even lick the tears off my face when I cry. Walking her every day gives my day structure, and I love it so much. She makes me smile and laugh, and she keeps me going. Now, she’s not just my dog, but my four legged carer. Molly also has her own blog, with over 3,700 followers. She brings light and laughter not only into my life, but theirs, too, through sharing her adventures and antics with her followers. You can follow Molly’s Facebook blog here - Molly, Dog With A Blog

Molly is my life, and she is a dog in a million. I am so pleased that the PDSA have recognised just how extraordinary she is, and that they think her worthy of an award such as the PDSA Order of Merit. To be only the fourth dog to have received the award, it just shows how special she really is. 


The PDSA runs a world-renowned animal honours programme, which included the animal version of the Victoria Cross, the Dickin Medal, for military animals, alongside awards recognising non-military animals for their outstanding devotion to duty and/or their owner. Molly has become one of an elite group of animals recognised by the system.


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