My name is Lucy, I’m 20 years old and I have a life limiting condition. You probably won’t have heard of it, it’s called Ehlers-Danlos Syndrome (EDS). It’s considered rare, but is becoming more prevalent as awareness and understanding of EDS improves. I have the most common type, however I have very rare, very severe complications. It means I am very bendy, my joints dislocate easily and frequently, I live in chronic pain, my muscles are weak, my autonomic nervous system doesn’t work and my gut and bladder have failed. As you can see, I am in a wheelchair. What you can’t see is the line in my chest that goes into my heart, the tube that goes into my stomach, the stoma bag on my abdomen and the catheter going into my bladder. You won’t see the medication and nutrition being pumped into my heart, or the body that struggles to do even basic things. You can’t see the pain, the fatigue, my racing heartbeat or low blood pressure. You will see me, with a smile on my face, hiding all the horrific things going on inside my ailing body.
I now have a fantastic team of consultants, surgeons, district nurses, hospice nurses and carers but it wasn’t plain sailing to get to this point. When I first became sick and disabled, aged 14, I was told, repeatedly by some medical services that my illness was just laziness or a school phobia – for the record I loved school, as I love learning. I started to question myself; was I really ill? Were they right that I was lazy and useless? It took 10 months before I was diagnosed. I had a poorly understood condition that had been misunderstood by the health professionals that I had encountered. I was relieved. Finally I had a name for what was wrong with me. Finally I could let myself off the hook. I wasn’t making it up. I didn’t have a school phobia. I wasn’t fat, lazy and useless. I had EDS.
Despite my illness with the support of The Children’s Support Services, I managed to complete my GCSEs in 2010 at the same time as my former peers, doing the exams laid in bed with the help of a scribe and the use of a laptop. I achieved an A star, three As, two Bs, one C and two distinctions. I was elated. I only received seven hours a week of tuition so I had to be self-motivated and push myself hard.. I started college studying the sciences at A level, despite only being able to attend part time and only being able to sit up for an hour per day. I was really enjoying it and was aiming for a career in medicine, determined not to let my body stop me from achieving my dream, but I only managed a term and a half before I became too ill. I hoped to go back once my health stabilised but sadly I never made it as I was too poorly.
Between the age of sixteen and seventeen, depending on individual services, I was transferred into adult services. By then I was completely bed bound, my gut was failing and I was self catheterising my bladder to empty it. Within six months I was down to only seven stone and was literally starving to death. I was put on TPN – intravenous nutrition – and having a stoma bag was starting to be discussed. I was put on morphine for my pain and the local young adult hospice were now involved. The legacy of some of the poor treatment I received when I first became ill has stayed with me – these scars run deep. If something goes wrong or my condition deteriorates, I immediately blame myself. I am frightened my consultants will turn against me or not believe me. I have a deep rooted fear of physiotherapists who gave me such a low opinion of myself. After being told I was fat and lazy for so long, that is often how I see myself.
I’m fortunate my team now all understand my condition and all work to improve my quality of life. We know my life will be limited, and so the focus is on quality. I am in receipt of palliative and hospice care. I am supported by two young adult hospices; The J’s Hospice who care for 18 to 40 year olds all over Essex, and Jacksplace at Naomi House, a three hour journey away in Winchester, for inpatient respite. My hospice nurse helps coordinate my care, though most of it is down to my mum. I don’t have the energy to keep phoning person after person, fighting to get the treatment I need and passing information from one professional to another. In reality, it is all down to my mum. She phones person after person, arranges blood tests and appointments and sorts out all of my medication, one of which has to be couriered down from London every few months, and when I’m on intravenous antibiotics, she drives to the hospital to pick up the drugs and picks up the equipment from the district nurses. My mum does my TPN and medication every day, she administers IVs and takes blood from my hickman line, gives injections, dresses wounds, can catheterise me, mixes and prepares medication, and so much more; she can do things many nurses can’t do. It’s a huge responsibility, she is the one keeping me alive every day. She does all the fighting and the chasing. She liaises between consultants and departments. She sorts out my prescription and collects medication. She deals with my personal budget and pays my carers. She’s my mum, nurse, carer, PA and best friend. It’s all down to her. She’s given up her life to keep me alive, not having a holiday or break in 6 years. My team now are very grateful for all that she does, as am I. However at times her contribution to my life is totally overlooked. There have been occasions when she has been treated like a neurotic mother or has not been allowed to be with me when decisions are being made – even though I want her with me. Professionals often forget that mothers are the experts in their children. They live with them, see them every day, bring them up and take care of them. They know their child better than anyone else. Doctors may have the qualifications and the knowledge of the human body, but parents are the experts in their children. They need to be listened to, included and respected. That includes when their child becomes an adult, especially if the patient can’t speak for themselves. Their child, no matter how old, will always be their baby.
All throughout my illness, and my life in general, the one thing I’ve always wanted is to be listened to. That is extremely important. I have very little control in my life, being dependent on others for almost everything. The only bit of control I have is my opinion and my ability to make decisions. I value this greatly. However, I am not in control over whether people listen to my opinion. My opinion or decision is only in my control if people listen to it and take it on board. It’s hard for some people to understand why I get so upset if someone makes a decision on my behalf without my consent and prior knowledge, but when it’s the only thing I have control over, you can understand why. Healthy people make decisions all the time and everyone has their own opinion. But when you’re sick and/or disabled, suddenly some people think it’s ok to make decisions for you, as if, somehow, being sick or disabled makes your opinion worthless or unnecessary. Others do it to be kind, but it still can be upsetting. I am perfectly capable of making my own decisions, and so I should be involved in all decisions that concern me or affect my life. My decision, no matter what it may be, should be supported. Be it what clothes I want to wear, where I want to go or even down to things like whether I want to be kept alive by machines or if I choose not to have life-prolonging treatment. However hard or important the decision, the individual should be listened to – even if you don’t agree with what they’re saying.
My opinion has not always been valued and I’ve had times where people have not listened at all. For a long time my paediatric occupational therapist insisted I didn’t need an education, I should just physically go into school. I wanted to do my GCSEs and was doing well thanks to the tutoring from Children’s Support Services and as I said I completed 9 GCSEs in 2010, from my bed, at the same time as my peers. I started planning for college long before I needed to, knowing I’d need a lot of support and we needed to leave enough time to organise it. We held many meetings, but at every meeting, my occupational therapist shot me down, saying I didn’t need to go to college, and when I protested, said that I should only do one A level. I stood firm and went to college to study the three sciences despite what she had said, but unfortunately I became too unwell to continue. I was often not listened to about my symptoms or my condition as, according to my paediatric professionals there was nothing wrong with me. I once went for a swallow test at my local hospital but I can’t stand up so the test would have to be modified so I could do it. Yet when I got to the room the consultant wouldn’t believe I couldn’t stand up. He made me show him how I can’t stand up. Being listened to is so important. Our views should be at the forefront of any treatment. We are all individuals and want different things out of life. So what suits me won’t always suit another young adult. After all, we want to live our lives just like any healthy person, and healthy people don’t always want the same thing as the next person. And remember, we are the ones who live inside our bodies. We know how our bodies work, what is normal for us and our pain is what we say it is. I’ve been accused of exaggerating my pain in the past and being treated like a drug seeker because people didn’t believe I was in as much pain as I said I was. If we say it hurts, it hurts. There are the minute minority that are drug seekers, but that doesn’t mean every person in pain is a drug abuser. If we say something’s not right, it’s because something is not right. We know our bodies inside and out. We are in pain, and pain is a horrible sensation and is so debilitating. Imagine being in pain and nobody giving you anything for 12 hours because they deem you a drug seeker. You wouldn’t wish that on anybody.
The jump into adult services – and it is a big jump – was hard for me. There is very little support specifically for young adults. We are no longer children in the medical and social definition, we are young adults, but adult services aren’t equipped to deal with the wants and needs of young adults. Being ill at fourteen meant I started out on children’s wards. Then I turned sixteen and my transition into adult services began. On one hospital admission I was on a children’s ward where my mum could stay with me during the day and throughout the night and would make decisions on my behalf. In my next admission I found myself on an adult ward. I was in a bay with five dementia patients, totally reliant on the overstretched nurses who didn’t have the time to hold my hand and talk to me; they barely had the time to give me my medication or change my bed. They never had the time to shower me or do my hair; my mum used to spend visiting time doing these. I felt vulnerable and scared stuck in bed unsure of how to deal with the dementia patients. These defenceless and confused ladies misused the call bell system resulting in the nurses ignoring the majority of the buzzing, but that meant my needs were also ignored. I needed their help with everything; even going to the toilet. My consultants expected me to make decisions by myself, and my mum was only allowed in at visiting time which meant she wasn’t there when the doctors did their rounds anyway. I was suddenly in charge of everything.. I spent five lonely weeks on this ward.
What do I want to see changed? I want the NHS to recognise that young adults are distinct from both paediatrics and adults, we are a subgroup of adults who are no longer children but are not mature adults yet either. We want to be helped to live our lives, to gain some independence and to allow us to do things other people our age do. I want to see more individualised care, we aren’t a one-size-fits-all group. We’re unique individuals, with different wants, needs, hopes and dreams. I want the wants, needs and views of the child or young adult to be at the forefront of healthcare. Our bodies and our lives are our own and we want to be helped to live the life we dream of. I want patients to be listened to when they say they’re in pain and their pain managed effectively to improve their quality of life. And finally I want to say that doctors need to be more aware of rare diseases when treating a patient. Just because you haven’t heard of a condition or it’s not well understood doesn’t mean that the person is making it up. It means that you need to listen to your patient even more, as we know more about our conditions, especially with respect to living with the condition. Patients and parents have a lot of knowledge about themselves, their condition and what life is like with that condition and I wish health professionals listened more to their patients and the parents. They know more than you realise. They just might hold the key.