It’s been 18 years since I was diagnosed with long QT syndrome and had my very first permanent pacemaker implanted; and 25 years since my father found me unresponsive on the bathroom floor, before resuscitating me – I later found out that 95% of females who suffer their first major ‘event’ do not survive. My father’s quick thinking had literally saved my life. Prior to this, for years on-and-off, I had suffered with what appeared to be random panic attacks. To finally receive a diagnosis was a huge weight lifted after seven years of not knowing.
Now aged 43 and having completed my family, it’s my turn to walk in parental shoes and experience the worry that comes with it. I knew before my two girls were born that there was a possibility of them inheriting my condition. “There’s a 50% chance that any of your children could have this condition,” is what I was told. They were bang on. Grace was three and Jessica was five months old when bloods were taken for genetic testing and sent to a specialist clinic in Italy, along with blood from myself and my parents. I knew it would be a long time until we would receive the results and they only came almost two and a half years later. No amount of preparing for the results can prepare you for how you feel when you hear them. I guess I knew all along that one of them would have long QT. I think a mother’s intuition kicks in and you just know.
One day a lady phoned from the genetics unit at Taunton, who asked if I would be ok to receive the results over the phone. She said that both my parents had tested negative – BIG relief there! Then I said, “one of my girls has it, don’t they? It’s Jessica, isn’t it?”, to which she said, “how on earth did you know?”. The call ended after a bit more chat, none of which I can remember as by this point I’d zoned out, but I do remember putting the phone down, looking at Jessica, picking her up, holding her really tightly and sobbing like a dam had burst.
The feeling I had after the call was an intense mixture of relief to finally know so that I could process things and move forward, but this was over-shadowed by a huge cloud of guilt, knowing that it was because of me she had this condition. All these years later, the guilt is still there, but much less so, knowing that she has never known anything else and is unbelievably happy with life – this does get easier to deal with. If I picture the alternative – not having my girls – that feeling would be far worse. The call told me several things. My parents were NOT responsible for me having long QT and Grace, my oldest daughter, was in the clear. It also meant that I had become the start of a chain that could pass the condition to future generations, starting with Jessica. This is a very difficult thing to comprehend. My mum constantly tells me, “Rebekah, for-warned is forarmed!” She always picks me up when I’m down.
A series of appointments monitored Jessica’s heart at Yeovil Hospital and then Southampton General, where Jessica received the best possible care. ECG’s aplenty, 24-hour heart monitors, along with having a Reveal device implanted into her chest. At the age of three, she had her first one put in, then when the battery ran low at the age of six, she had another one implanted. The Reveal device allowed Jessica’s heart to be monitored for abnormal rhythms and would record anything untoward for the hospital to assess. At night, she had a device in her room to link her directly to the hospital in case anything sinister occurred whilst she was sleeping. Every six months a full download was done via this device straight to the hospital for assessment. After the second Reveal device, Jessica was implanted with her first permanent pacemaker. As she hit puberty (the time when I had started to show symptoms), Jessica was fully protected. Alongside her new ‘friend’, she takes beta-blockers twice a day to ensure she has full protection from an ‘event’ ever occurring the same way it had done with me. The day she finally got her pacemaker was one of huge relief, knowing I had fought for so long to make her as safe as possible.
Jessica amazes me at every turn! She’s never let the devices stop her and leads such a well-rounded life, enjoying sports and playing out with her friends and is a normal ‘nightmare’ pre-teen girl who clearly loves life. Despite cannulas, needles, post-op sickness, stitches and sore wounds, she has coped with hospital life like an absolute trooper, but she’s never known anything else, so she just accepts what ‘is’ and gets on with it. I am beyond proud of how she’s dealt with it all and she never complains. I’d like to think it’s because of how positive we’ve been as a family, never speaking of it negatively.
It’s not easy being the reason your daughter has to go through life with semi-regular battery changes and constant medication, but I wouldn’t be without her. The guilt and I have come to terms with each other now and life is good, knowing she is safer. Jessica has just celebrated turning 12 and is loving life more than ever. What more could I ask for?
Should anyone be going through anything similar and want to contact myself, or Jessica for that matter, please go through CRY who can point you in my direction.