Chloe Harris on getting a reveal device

My name is Chloe and in April 2006 I lost my half-sister to ARVC (arrhythmogenic right ventricular cardiomyopathy). Like so many other ordinary families, our loss was our first introduction to the reality that so many young people lose their lives to this type of condition that so regularly goes undetected.

In last nine years I have travelled once or twice to my hospital from my home in Kent to have tests carried out and repeated to monitor me for the same condition. In January 2015 they detected a change from the year before, and this –combined with a few unexplained fainting episodes in the October of 2014 – prompted the staff to want to fit me with a “reveal device”. [The device is used to record your heart rate and rhythm if you have an unexplained fainting episode, dizziness or palpitations.] I had the reveal device fitted at the start of April.

I struggled to find anyone to talk to about how I felt about having this done, after all, the doctors can only tell you the medical side, and anything you find on the internet reads pretty much the same thing. This was really frustrating considering I was reassured that it was a very common thing for people to have fitted. This is what prompted me to contact CRY and write this. The medical ins-and-outs of the procedure was not the information that I needed. Medically it’s so simple my consultant wouldn’t even call it an operation, which is fine, but he wasn’t the one having it done. Facing the procedure was the first time (nine years down the line) that I had to face the fact that my and my family’s journey with ARVC might not have stopped at the loss of my sister.

Leading up to my admission to hospital I went through the full range of emotions – I am very lucky to be so well supported by my family. I was exceptionally worried and nervous almost all of the time, more about the future and what this might uncover than the device-fitting itself.

On the day of my procedure I travelled by car, as they advise that after you’ve had the device fitted you do not use public transport to get home again. I was admitted to the short stay ward, changed into a lovely hospital gown and had an ECG and blood pressure taken. I waited to meet the consultant doing the procedure and one of the theatre nurses who would be looking after me. I had a cannula fitted (in case of an emergency, and to give antibiotics to protect me against a very small risk of infection). I chose not to be sedated and had the procedure just under local anaesthetic.

I walked with the nurse into one of the catheter labs; just under an hour later I was wheeled back to the ward. The fitting takes a very short time; the rest of the time was taken up by the staff’s preparation and the careful stitching they did to reduce the size of the scar as much as possible. After the stitches they scanned the device to make sure it was working. Back on the ward my family were waiting for me. I was given my other half to the device so I can track my symptoms (if I get any) and my patient card which has the device’s serial number on it; I have to keep both of these things with me at all times so they stay in my handbag. After two hours I was allowed home after being given all of my paperwork; I had to eat, drink, walk around and go to the toilet.

After a week I went to the GP to have my stitches taken out – which was a big relief for me as I was very worried about catching them. I had to have a week off from work and now I can do everything that I could do before.

It may seem strange but I was really concerned about being able to feel the device. Yes I can feel it when I move or lay on my front etc., but I am getting used to it being there and it isn’t anywhere near as pronounced as I feared it might be. No-one can see it and the consultant made sure that it was very low in placement in case I became concerned or self-conscious about it, even with the very small scar I will be left with.

For anyone facing having this device fitted I would suggest the following; make sure you have someone to talk to, the emotions this stirred up for me were huge. Write down your questions to ask the doctor, and if you are not happy with the answer ask your question again and ask for clear answers. Ladies, get a comfortable non-wired bra for the first few weeks just in case. Find something to focus on to distract yourself if you aren’t very comfortable or happy in hospital environments; it really will help, especially if you opt not to be sedated like I did. In my head I repeatedly sung (Baz Luhrmann’s “Wear Sunscreen” to be precise), it may seem strange but it will give you something to focus on and will take you to a different place, it kept me calm anyway; find something you know will work for you.

I hope that this might help someone with the non-medical information I needed but could not find.

I wish you enough sun to keep your attitude bright.

I wish you enough rain to appreciate the sun more.

I wish you enough happiness to keep your spirit alive.

I wish you enough pain so that the smallest joys in life appear much bigger.

I wish you enough gain to satisfy your wanting.

I wish you enough loss to appreciate all that you possess.

I wish enough “Hello”s to get you through the final “Good-bye”…

Lucy Challis

Lucy Challis wrote her story to be included in the myheart booklet which was launched in November, 2013.

Lucy 1On Sunday 29th January 2006, my older brother Stuart, age 25, collapsed and died playing football. I was 23 and my other brother was 27. Stuart was the fittest of the three of us. He had no symptoms. His post-mortem, confirmed by the CRY Centre for Cardiac Pathology, identified arrhythmogenic right ventricular cardiomyopathy (ARVC). Our GP explained the genetic implications and the importance of screening. I think the shock of Stuart’s death meant that we didn’t really take in what being screened meant – it was just another hurdle to overcome. On a Monday in April, my parents, brother and I had ECGs and echocardiograms. The doctor asked if anyone had experienced possible symptoms and when I mentioned I had fainted on a horse at 15, the look on his face got me thinking. By the time he had studied the results of the tests, I’d come to expect the answer – he thought I had ARVC too. In comparison to Stuart’s death, I almost felt like this was nothing – at least I was still alive.

Two days later I was referred to The Heart Hospital and took my overnight bag, I was so convinced I would be admitted. A specialist nurse questioned me prior to retesting, which included an exercise test. Suspicions of ARVC triggered an MRI scan, a 24-hour Holter monitor and the offer of genetic testing, with a 50% chance of finding the gene. The diagnosis was confirmed, with the MRI indicating mild left-ventricular involvement and I was started on medication to control my irregular heart rhythm and offered an implantable cardioverter defibrillator (ICD), which I immediately agreed to. There was just no question of me putting my family through the possible heartbreak of losing another child. I’m a practical kind of girl and like to just get on with things. I was lucky not to be hugely sporty so didn’t have to make many life changes; I was still permitted to continue horse riding and only exclude mad cross-country gallops! The hardest thing was dealing with other people – some wanted to wrap me up in cotton wool and not let me lift a finger, others found it difficult to accept I was actually suffering from a potentially life-threatening condition as I had no outward signs.

My ICD was fitted in May 2006 and I think Mum was more nervous than me! I don’t tend to worry about these things, so the day just kind of happened. I was nil-by-mouth from midnight, so frustratingly I could not eat or drink and needed to occupy myself until 4pm because I was last on the list! I hadn’t wanted to see an ICD before it was put in, but a rep from the ICD company was chatting to me in the lab prior to surgery and offered me a demonstration model to show the children when she learnt I was a teacher. She put it in my hand there and then – not exactly the ideal time just before I was about to have one put in me! My ICD was implanted under sedative and local anaesthetic and within half an hour of returning to the ward I was starving and demanding food. My left shoulder, arm and side were so stiff and sore my husband had to cut everything up and feed me, but otherwise I felt fine. It was odd knowing there was something metal inside me that I couldn’t feel unless I moved. After a relatively comfortable night, I was allowed home.

Having to take 10 days off work was disappointing but I needed stronger painkillers than I was given. You don’t realise what muscles you use until you can’t use them. Standing, sitting up and sitting down, were challenging. I couldn’t lie on my side, so had 2 weeks being propped up on cushions to help me sleep. My wound needed to be kept dry for 1 week and I couldn’t lift my arm above my head for 6 weeks. I found it difficult to wash my hair one handed! I’m a primary school teacher, so had to be careful lifting heavy piles of books, writing on the board and teaching PE and Games for a while after returning to work. I have always felt comfortable talking about my condition and was open with my class about my ICD. I wanted them to know what was going on and that it was fine to question me. The best bit was when they asked if they had to plug me in to recharge me! Being around kids is great because they soon forget about it all and just treat you like they always have. They looked after me and carried my things around for as long as I could get away with it!

Genetic testing on Stuart’s tissue showed one gene each from Mum and Dad combined to be responsible – a million to one chance. Mum, Dad and my brother carry one gene each, unlike me and Stuart who had both. They are regularly checked and take medication as a precaution. Learning the whole family were affected was a shock, but at least we know. Some relatives have had ECGs which appear normal. Some have chosen not to be tested which I struggle to understand, but this option is always available. None have had genetic testing. After months of upheaval our family settled into a more routine existence as we learnt to live life without Stuart. Memories became happy rather than sad and 8 years later his name is mentioned without tears, on most occasions.

The first time my ICD fired was August 2007 when I was horse riding – thankfully I didn’t fall off! I was retested and an ‘inappropriate shock’ was confirmed – it shouldn’t have fired. My medication was increased and once I got over the shock (literally!) it was a funny story to tell! In March 2008, my ICD fired again. This time checks confirmed it had fired appropriately; my heart reached 220 beats per minute and my ICD charged and fired within 7 seconds. It was odd thinking that without my ICD I could have been in serious difficulty, but I felt it had done what it was meant to do – save my life. Neither shock was pleasant, but within 2 minutes of each I felt so well it was almost unbelievable it had gone off at all. I was slightly cautious for the next couple of weeks, feeling conscious of everything my heart was doing, but that worry faded and I just got on with things. Most annoying was not being able to drive for six months but my lovely friend who lives close by chauffeured me for those months which was an absolute godsend. Weekends were OK as other people were around, but my six months spanned the summer holidays and there’s nothing more frustrating than being deprived of your freedom and becoming dependent on others.

My ICD was replaced in September 2011 and there have been no further episodes. My family and husband, thankfully, don’t treat me with kid gloves, although I’m sure they worry every time I go horse riding or on school residential activity weeks. My recent pregnancy put my heart under more strain but I had brilliant care and my 12 week check up indicated my heart seems to have coped well.

ARVC is a genetic condition and I have 2 faulty genes, so realise my children could be affected. However, this has not put me off having them as they can have gene testing to remove any doubts. Not having to make huge lifestyle changes has helped; if I had been severely affected I could have felt differently, but having our little boy in our life is certainly the best thing that’s ever happened to us.

I understand why some think young people can’t cope with being diagnosed, but my experience from the last 8 years has taught me that children have terrific reserves of resilience, adaptability and acceptance and it is important for young people to have the chance of a diagnosis. If Stuart had been screened and diagnosed with ARVC then it might have stopped him playing competitive football, meant adjustments to his social life and possibly an ICD, but he would probably still be alive. Some might find having a heart condition difficult to accept and struggle to cope but others will acknowledge their limitations and move on. For those who think young people can’t cope, I ask this – is it better to know and live, or not know and die?

  • Lucy 1

Rob Jackson

Living with arrhythmogenic right ventricular cardiomyopathy (ARVC)

I am a 31 year old Sergeant in the Army Air Corps and until recently was a Lynx Helicopter pilot and aircraft commander operating in Northern Ireland. I joined the Army at 18 and have spent the past 12 years living an active, healthy lifestyle as a soldier.

I passed the Army Pilots Course in 2001 and have enjoyed the challenge of flying the Lynx MK7 and MK9 Helicopter. I have served in Germany, Bosnia, Canada, Poland, Cyprus and Northern Ireland.

I am a keen sportsman and have represented my Corps at rugby and windsurfing. I cycled approx 10 miles a day to and from work and considered myself as one of the fittest amongst my work colleagues.

It was Saturday 26th September 2006. I’d been out for a run with my dog Amber in the morning and was heading out for a few beers with the boys that evening. On our return from the club in the car, I found myself sweating and out of breath, it was about 2am. I got home and remember my partner giving me grief for the state I was in. I could feel my heart racing and decided to call an ambulance as I was struggling to remain conscious.

The paramedics arrived in what seemed like minutes and diagnosed me as being in VT (ventricular tachycardia), with a sustained heart rate of 240 bpm. I was rushed to Antrim hospital and was defibrillated back into normal sinus rhythm.

I spent a week in hospital connected to an ECG and showed no further symptoms. I then had an ECHO scan on my heart and exercise stress test on a tread mill and still showed no symptoms of VT. My consultant, Dr Tom Trouton, discharged me with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC) and I was to return at a later date for an angiogram and EP studies. These were both carried out and I was still un-diagnosed. The next step was for me to have a cardiac MRI scan which I would have to wait for 4 months. Luckily the Army paid for my MRI privately at St Georges, which confirmed that I had ARVC.

I was shocked, I considered myself as a healthy and fit guy, with a promising future as a pilot and until that night in September, I’d had no symptoms at all.

On 19th January 2007 I was admitted to the Royal Victoria hospital in Belfast to have my implantable cardioverter defibrillator (ICD) fitted, which will protect me from abnormally fast heart rhythms. I was discharged the same day and am currently on 2 weeks sick leave to recover.

I am slowly coming to terms with the fact I’ll never fly again, or do all the things I’ve enjoyed as a soldier. I am lucky though, I very nearly didn’t make it. I’ve had a lot of help from my friends and family and the Army have re-employed me as an instructor.

I’m already planning a fund raising sponsored walk for the summer to help support CRY. I think their national screening programme is invaluable as most people with my condition are diagnosed when its too late.

David Hillson

Living with suspected arrhythmogenic right ventricular cardiomyopathy (ARVC)

In February 2008 I was out running with a friend. It was bitterly cold and I had not run over the Christmas period so this run was my attempt to ‘get back into it’!

I had always been fairly healthy, played football all my life (and rugby at University) but at the age of 29 I had kind of fallen out of fitness, so a run once a week was my small way of keeping fit.

Anyway, after 20 minutes of the run I felt dizzy and before I knew it, collapsed. After coming round, within minutes I passed out again, so my friend rang for an ambulance. It arrived within 10 minutes and I felt a little embarrassed by what I felt was a bit of a fuss.

On entering the Ambulance they took an ECG and my heart was beating at around 150bpm. They were a little worried as I had stopped exercising about 15 minutes before. Within minutes I was finding it hard to catch my breath and was rushed to hospital.

What I did not know at the time was that my heart had gone into ventricular tachycardia and was accelerating into a life threatening arrhythmia. On arriving at the hospital I was taken through A&E and ‘shocked’ (defibrillated) whilst still awake, to correct the arrhythmia. My heart had apparently almost reached 300bpm.

After the initial overnight observation and resulting stay in the hospital I was transferred to St Thomas’ Hospital in London for further tests. Its funny to reflect on this time, as the night I collapsed was the night before I was due to have a joint birthday party with a friend – all I was preoccupied about was whether I would be OK to go to that!

After a MRI, EP studies and consultation between the cardiologists they advised I have an implantable cardioverter defibrillator (ICD) fitted as they could not rule out the risk that I could be in for further arrhythmias. The doctors feel that I may have the condition known as ARVC and the ICD would act as the ultimate safety net. On receiving the news I was shattered. Though it had been inferred all week it was to be the most likely outcome, it still shocked me as I felt like my life had been altered forever.

So I had my ICD fitted the day after my 30th birthday. Once over my first few weeks of recuperating life pretty much got back to normal. Bike riding and lots of walking were my attempts back to ‘normal’ life. I have since qualified as a teacher and begun teaching in September 2008.

All seemed well until I received my first ‘shock’ from my internal ‘safety net’! I was playing 5 a side football – so was really pushing my heart to its limits perhaps.

It is odd that I have never had a single heart complaint in my life but suddenly this year it seems there is a real problem. However, it is comforting to find out (from sites like this, or being in hospital itself) that there are others out there who have had similar experiences – and like I try to think when reflecting on this: at least I am alive and typing this!