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Monday, 11 September 2017

Sepsis Survivors: How My Family Has Been Touched By Sepsis

(c) UK Sepsis Trust

September is Sepsis Awareness Month and Wednesday 13th September is this year's World Sepsis Day. My family has been touched by sepsis a number of times. We know too well how serious sepsis is. There’s an estimated 150,000 cases of sepsis in the UK each year, and around 44,000 deaths. Myself and my relative should be part of that 44,000, but we are fortunate to have survived. I have survived more unscathed than my relative. However, I have pre-existing conditions that predispose me to having sepsis, which means I am more likely to suffer sepsis repeatedly, as I already have. 

(c) UK Sepsis Trust

What is sepsis? Sepsis is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. Despite common misconceptions, sepsis is not the infection itself. Your immune system protects you from many illnesses and infections, but it's also possible for it to go into overdrive in response to an infection, which is what occurs in sepsis. Common signs and symptoms include fever, increased heart rate, increased breathing rate and confusion. Severe sepsis is sepsis causing poor organ function or insufficient blood flow. Insufficient blood flow may be evident by low blood pressure, high blood lactate, or low urine output. Very severe cases of sepsis can lead to septic shock, which is a medical emergency. Septic shock is low blood pressure due to sepsis that does not improve after reasonable amounts of intravenous fluids are given. 

I’m 23 years old and a 12 times sepsis survivor. 12 times, I could’ve been part of the 44,000 who don’t make it. To survive once is great, twice is lucky, to survive three times unusual. To survive 12 times? I don’t even know what to call that. However I can say with certainty, it isn’t luck that’s enabled me to survive a dozen times, it’s good care. Admittedly, my care hasn’t always been perfect, even in the throws of sepsis, but thanks to the expertise of my fantastic specialist teams I have had the care I require to survive and they have meant that I have successfully overcome sepsis 12 times. 

Why have I had sepsis so many times? I’ve had sepsis from various underlying infections. I have a number of complex, life-limiting conditions that mean I have multi-organ failure and a lot of intervention is required to keep me alive, as well as an immune system that doesn’t always recognise or fight infection. I have a permanent central line known as a Hickman Line, which gives constant intravenous access directly into my heart. My nutrition, TPN, is administered through this line over 21 hours a day, and I also receive intravenous fluids and intravenous medications every day. I simply wouldn’t survive without permanent intravenous access, nor the intravenous feeding, fluid and medication I receive each day. However, this line and the high glucose content in my feeds, mean I am prone to infections in the line which can then spread into my bloodstream, overwhelming my body and causing sepsis. This has happened four times, where an infection has settled in my line, spread into my bloodstream and my bodily reaction has escalated to sepsis. These infections have caused me to lose lines, on which my survival depends. I have also had sepsis resulting from an operation, occurring after the surgery to form my Urostomy (Ileal Conduit), where a piece of bowel is cut free and used to form a ‘tube’ for the urine to drain from my kidneys, through the ureters, into this section of bowel and then out onto a bag stuck to my abdomen. My gut harbours much bad bacteria due to Chronic Intestinal Pseudo Obstruction (CIPO) causing Small Intestine Bacterial Overgrowth (SIBO), where bad bacteria overpopulates in the bowel. Due to CIPO, my gut has dilated and stretched so much and the bowel wall become so thin and fragile and this allows this ‘bad’ bacteria to leak through the bowel wall and into my bloodstream. There, it settles in my Hickman Line or other points in my body, known as translocation. The third cause of my sepsis battles, seven of them in total, is my kidneys, known as urosepsis. I have a permanent infection in my kidneys, E.coli, which was caused by gut bug translocation initially and is now unable to be cured or even treated unless the infection threatens my life. You see, I have had so many different antibiotics for the E.coli in my kidneys that have failed to treat it, that it is now almost completely resistant. We have two intravenous antibiotics that we can use, in life-threatening episodes, to calm the infection down, but they do not cure it. Therefore, we have to save them for life-threatening situations. It means I live with symptoms from the kidney infection and the infection is slowly damaging my kidneys, but it has not caused urosepsis since March 2016 - a record for me. Aside from my aforementioned battles with sepsis, I live with four permanent, incurable infections, all of which could cause sepsis at any time. 

I do have some lasting impacts from sepsis, but fortunately these are relatively mild. These after-effects, which may be referred to as "post-sepsis syndrome", which for me are mild compared to the after-effects some suffer, are nonetheless troubling. Sepsis has weakened my already weak body. Each battle with sepsis has left its mark, serving to speed up deterioration and increase the impact of symptoms I already suffer as a result of my conditions and their complications. I have suffered an increase in fatigue, aches and pains, increased weakness and an increase in the symptoms of the Autonomic Neuropathy I already suffer with. Each sepsis bout would leave me more tired, weaker and with increased cognitive decline, albeit mild. I often have described the latter as "I feel as if I'm becoming stupid." I have a brilliant brain and am not afraid to say I'm very intelligent, but each bout with sepsis would serve to batter my short-term memory, increase the 'brain fog' that sometimes plagues me and make things like word recall and remembering names harder; but my long term memory has remained unscathed. Another, somewhat strange effect of sepsis was the sudden onset of pain in my right hip around the metalwork following sepsis in March 2016. I fell out of bed in hospital in July 2014 and broke my hip, necessitating a dynamic hip screw and a plate. Once I recovered from the post-surgical pain, I had little complaints about the metalwork and no pain in my hip. Then, less than 2 years later, I suffered yet another bout of sepsis, which resulted in pain around the metalwork that has never really left. Initially panic ensued that I had developed an infection around the metalwork in my hip, but apart from noticing arthritic changes in the joint and poor healing around the metalwork, there was no reason for my sudden increase in pain - the trigger was the bout of sepsis. However, I'd had multiple bouts of sepsis between the fall that necessitated the operation to insert the metalwork into my right hip, and the onset of pain in that hip in 2016, so why the sepsis in 2016 triggered the pain where the previous bouts hadn't, no one knows. The biggest post-sepsis problem for me is with my immune system. My immune system has become very poor at recognising infections, allowing them to escalate quickly without any attempt from my immune system to fight it. I get seriously ill from the infection running rampant in my body, which suddenly triggers my immune system which jumps into overdrive, causing sepsis and related symptoms and organ dysfunction. It makes it very dangerous for me; I get no early warning signs of an infection, then suddenly I'll become very poorly in a matter of an hour or two. I'll be taken to hospital, at this point I've almost always developed sepsis, and it becomes a battle to stabilise me. Isolating the source of infection can be hard, when you already have infections in many places, and given all my interventions I have a lot of places for infections to 'hide out'. My immune system doesn't recognise them in the early stages, then when the infection takes hold suddenly my immune system jumps into action but goes overboard and causes sepsis. This immune system dysfunction is almost certainly the result of all the infections I've had, the fact I have four incurable infections living in my body and all twelve battles with sepsis. Post-sepsis syndrome is little understood, rarely talked about and to be honest, very few people know about it. Whilst my post-sepsis syndrome may be mild compared to the effects others suffer, it is nonetheless troubling. I already have a life-limiting, degenerative condition, and sepsis just accelerates the decline. It attacks a body that doesn't have the reserves in the first place. 

Despite the post-sepsis effects, I know how fortunate I am that, in each sepsis battle, most of the sepsis-related organ damage and derangement has been reversed by timely treatment including intravenous antibiotics, intravenous fluid and intravenous Paracetamol, sometimes needing vasopressors and sometimes oxygen. I have to live with post-sepsis symptoms and problems, but at least I'm alive. To overcome sepsis twelve times, especially when you already have a weak and struggling body, is nothing short of a miracle. However, I owe my survival to the care of my teams who have ensured that I have got through each infection and each battle with sepsis and can continue on with living my life, which I am so grateful for. Timely and appropriate treatment has meant I'm still here today. 

(c) UK Sepsis Trust
In addition to my battles with sepsis, there is another member of my family whose life has been affected by sepsis; more specifically septicaemia. This family member of mine was 16 when she contracted meningitis and resultant meningococcal septicaemia (often called “blood poisoning”). She was in a coma for a while and had to have both legs amputated below the knee. The septicaemia also caused kidney failure, meaning she went home a double amputee on dialysis at the age of 17. She then had a kidney transplant on Christmas Day 2003. It has not stopped her, however. She is a qualified nurse, emigrated to New Zealand with her husband and is now a very proud (and busy) mum. She’s the most determined person I know and serves as an inspiration to me through my own struggles. In her case, the septicaemia affected blood flow and damaged tissues in her body, which caused the sequelae from the sepsis: the severe disease and gangrene in her lower legs and thus the requirement for her limbs to be amputated below the knee and damage to some other tissues as well as the irreparable damage to her kidneys, causing the need for dialysis and then her kidney transplant. She was also in a coma for a long time as a result. She's lucky to be alive. 

I hope people will become more aware of the signs and symptoms of sepsis. People need to "think sepsis". It's important to recognise the signs. To the unaware, they may miss the signs, especially as the early symptoms of sepsis can be indistinguishable from the symptoms of the original infection itself. It's important to recognise when the symptoms continue to deteriorate and other suspicious symptoms occur that may indicate sepsis. These symptoms include a high temperature, especially if it isn't responding to Paracetamol; elevated heart rate (normal resting heart rate is 60-90 beats per minute); elevated respiratory rate (fast breathing); chills and severe shivering (rigours); reduced (or absent) urine output; confusion and may include other symptoms including vomiting, light and noise sensitivity and severe pain, as well as an often reported feeling of "I'm dying" or an impending sense of doom. Children may have similar symptoms but they have a lower threshold. 

Sepsis kills. Know the signs. Please spread the word this Sepsis Awareness Month and World Sepsis Day.
Thank you.

For more information, head over to the UK Sepsis Trust website (currently being updated but do check back at a later date). Thank you to Dr Ron Daniels BEM, founder and CEO of UK Sepsis Trust, for allowing me to use their images in this blog. 

(c) UK Sepsis Trust

Wednesday, 6 September 2017

Celebrating 1 Year Since Molly's Qualification As My Assistance Dog

One year ago today, Molly qualified as my Assistance Dog. What was almost three years of hard work, culminated in us passing our level 3 assessment. 

I will admit, there were times where I thought Molly would never make it. We struggled at times but it always came down to me; Molly is so in tune with me and after mum's brain tumour diagnosis, surgery and complications, I was in a very dark place mentally which severely impacted upon Molly. She couldn't cope with me being so sad, negative, irritable and anxious and as a result she wasn't performing or behaving in her normal way, almost acting out. She was feeding off my negativity and mood and acting strangely. Sure enough, as my mood lifted and I became more positive, happy and settled, as if by magic Molly was back on top form. She is so very in tune with me. Sometimes I don't realise I'm in a bad mood until Molly acts oddly and I realise that it's me. 

In this year since she qualified, we've done so much. Molly has blossomed. She absolutely adores her job. Walkies has always been one of Molly's favourite things to do, along with training, but these have been far surpassed by her love of working. If her working jacket comes out or she knows we're going somewhere, she is so excited. She loves her job, loves helping me and loves accompanying me to places. She takes everything in her stride, with a smile on her face and a wag of her tail. I love having her beside me, too. We're a great team. I owe Molly so much. 

In the year since qualification, we've been to so many places. From the House of Commons to the Shard, from the London Eye to the Royal College of Physicians, from the Sealife Centre to the Emirates Cable Cars, from BBC Essex to Badminton Horse Trials and more. Molly's come to a variety of my charity events, from meetings for charities including NCPC/Hospice UK meetings to Pseudo Obstruction Research Trust meetings to NICE committee meetings, to national conferences such as RISE4Disability (of which our next one is RISE Maidstone) and the Big Bowel Event, to small group meetings, as well as our visit to the House of Commons for Together for Short Lives. We even took part in the Twilight Colourthon in July to raise money for charity. Molly is a well travelled dog! 

Molly's visited a number of different hospitals where I receive my care, presided over many of my tests including nerve conduction studies, X-rays, blood tests and ultrasounds, she's met a number of my consultants and been photographed to show family members and even appeared in one of my consultant's clinic letters! 

Molly and I have grown over the last year. Our bond is stronger than ever, we enjoy exploring the world and dealing with life's challenges side by side and Molly adores helping me in every way that she can. Whilst she may be qualified, learning never stops and we've continued to learn things as we go about our lives, such as Molly passing the cashier my purse, pressing door entry buttons and turning on the light. 

Molly was also awarded the PDSA Order of Merit in October (but not publicised until January this year) and we did a lot of work to raise awareness of International Assistance Dog Week in August. 

We've achieved an awful lot together. We are a team, a partnership and we face life together. Molly is always there helping me and giving me the strength to keep going even on the most difficult of days. Our love and our bond knows no bounds. A dream team, if you will. 

I just want to thank you all for your support. You are a huge support to Molly and I and on bad days we can revisit your comments for inspiration, and on good days we rejoice in your comments on our posts. 

We hope you will continue to follow our journey for years to come.

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! 

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