Emma Jackson wrote her story to be included in the myheart booklet which was launched in November, 2013.
I never liked sports when I was younger and was never any good. I remember having strange missed beats, tiredness and slight dizziness but thought this was normal. It was pure chance that a doctor noticed a heart ‘murmur’ prior to knee surgery when I was 13 in April 2001 – something not even noted on my discharge sheet! However, Mum used to be a nurse so she dragged me along to the GP to get it checked out. A few months of tests followed, visits to the local hospital in Bedford, then a transfer to Papworth for lots more tests. I was diagnosed with hypertrophic cardiomyopathy (HCM) – an enlargement of the heart muscle, especially the septum, the dividing wall between the two sides of the heart. I can’t really remember how I felt when diagnosed, except it felt strange because I had never had symptoms. It seemed unreal, and didn’t really sink in that HCM could cause sudden cardiac death.
The problems caused by HCM are twofold. The enlarged tissue cells are disorganised and so do not carry the electrical signals well in the heart, causing atrial fibrillation, arrhythmia or ventricular fibrillation which can lead to sudden death; but then the enlarged muscle can also cause obstruction to the outflow tract, causing inefficient pumping of blood. Because HCM is inherited, my family were advised to be screened but we were not offered genetic testing. My consultant recommended I had an implantable cardioverter defibrillator (ICD) to record any unusual heart rhythms and shock my heart back to normal if it detected a dangerous rhythm. This stunned me and I remember sitting in the car with my mum before the appointment, psyching myself up to accept the medication prior to surgery; but there is no cure to HCM so I had no option.
On 25th October 2001, aged 14, I had my first ICD fitted. As I was young my consultant ensured I had a full anaesthetic and was completely unconscious for the operation. I know they can do this surgery under local anaesthetic but there was no way that was happening! I was back at school 2 weeks later. It was strange feeling a box under my skin but eventually I barely noticed it. The scar hypertrophied – where the scar becomes thick and red and takes a long time to become flat and pale – but the placement on my shoulder means it is covered by tops most of the time.
I knew my ICD was my safety net. I worried about it going off, but if it did then the shock would be better than what could have happened! I carried on as normal, did GCSEs, A-Levels, and went to university. The heart thing was something I could choose to tell people or not. I felt much more in control and overall barely thought about my heart or the ICD. My friends were interested to hear about it and my house mates were fully informed. I didn’t keep anything from them.
My first real ‘incident’ was at 8.25pm on 24th November 2008, aged 20. I felt dizzy walking upstairs and sat down on a chair waiting for it to pass. I came round on the floor under the chair with my whole body tingling from electricity. My house mate called an ambulance and Whitechapel Hospital kept me in for 4 days because I had suffered an episode of ventricular fibrillation (VF) with my heartbeat fl uttering at 329 BPM.
Luckily my ICD shock stabilised my rhythm, but it was frustrating being in a different hospital with unfamiliar doctors inexperienced with HCM who wouldn’t allow me home although I felt fine. Frequently I had to explain my condition to the doctors and nurses, but the cardiologist who said “We have to keep you here because if this has happened once, it can happen again quite soon” was right!
There were 5 more episodes over the following 2 months, mostly at home. I became experienced in recognising the symptoms and getting to my sofa or bed so I had a soft landing! It did upset me once when it happened at work, in the middle of a busy shop, because it felt so out of control. It was a nuisance, I didn’t want to spend any more time in hospital but on reflection would think “Thank f**k I’m still here!” My consultant changed my medication to nadalolol, but ensuing bad headaches meant I had to go onto a more ‘hardcore’ anti-arrhythmia drug called amiodarone. Good for stopping arrhythmias and with more tolerable side effects, I have been on amiodarone ever since.
Another quiet ‘cardiac period’ followed and my ICD was changed in February 2010 after 8 years of faithful service. I had used it a lot and the battery was getting low but I was grateful to be able to schedule my surgery around important university work. Having the ICD changed was a walk in the park compared to having one implanted and 2 weeks later I was travelling around Norway. After having so many shocks and then being stable for a year I appreciated it was a necessity! I was in the first myheart group back in 2002 and returned after being absent for a while to again benefit from sharing thoughts and feelings about the effect HCM has on my life, with others in a similar position to me.
September 2010 brought more breathlessness and exhaustion. An echocardiogram identified the enlarged muscle was causing an obstruction. For the first time (aged 23) I personally made the decision to have major treatment, taking my consultant’s advice that open heart surgery called a myectomy might give me a new lease of life. I decided to get this surgery out of the way whilst I was young so that it wouldn’t interfere with my career, family and future life. The surgery on 4th October 2012 was a blur but I do remember feeling really hopeful and wasn’t nervous until I left my mum and brother at the entrance to the theatre – by which point it was a bit late to change my mind. Open heart surgery is not an option to be taken lightly. It’s not easy and it’s not fun. They removed part of my septum, replacing my damaged heart valve with a mechanical one and I am now on warfarin for life to thin my blood and prevent rejection of the mechanical valve. A myectomy is an extreme treatment for HCM and many people can cope without the need for surgical intervention, but for me it was the right choice. I was fit enough for the CRY Parliamentary Reception a month later.
Since then I have felt a great difference. It is brilliant no longer spending the whole weekend recovering from work. Cardiac rehab (with 9 elderly men) helped me rebuild my fitness. Since being diagnosed my mum, aunts and their children have had genetic testing and 2 mutated genes were identified. Four family members identified with HCM are being treated. We have found this very helpful but I do understand that others do not always want to know. I would worry about passing the genes on if I had children, but hope that genetic testing and screening would help protect them. My mum always describes herself as one of the lucky mums. I can’t imagine how difficult it must be for those who are diagnosed as a result of a close family member dying.
Although I know how difficult it is to come to terms with a diagnosis when you are young, it does begin to shape who you eventually become. CRY provides both screening and support to those who are diagnosed, something I wasn’t offered through the NHS. They offer help to all young people affected and it is not dependent on where you live or which NHS Trust you are under.
When Alison started CRY in 1995, many consultants told her that she should not be starting a screening programme to identify young people at risk, because young people couldn’t cope with a diagnosis. As with me, most young people have no symptoms prior to their diagnosis. If Mum had not ensured my murmur was checked, things could have been very different for us all.