CredibleMeds for members with Long QT

Members with long QT syndrome may like to consider registering with CredibleMeds to be kept up to date with any changes to the QT drugs to avoid list https://crediblemeds.org/everyone/.

We have recently received the following message through CredibleMeds:

On January 14th, AZCERT added the anticancer drug Glasdegib (Daurismo) to the Possible Risk of torsades de pointes (TdP) list because we have found evidence of QT prolongation but, at this time, no convincing evidence of TdP.

On February 17th, the following drugs were added to the lists:

Lofexidine (Lucemyra, a drug for treatment of opiate withdrawal) was added to the Possible Risk of TdP list because we have found evidence of QT prolongation but no convincing evidence, at this time, of TdP.

Hydroquinidine (an antiarrhythmic drug) has been added to the Known Risk of TdP list because we have found evidence of TdP cases reported with its use.

Xylometazoline (a decongestant nasal spray with many brand names) has been added to the list of drugs that patients with congenital long QT syndrome should avoid if possible.

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Emmerson Hird

On Wednesday 6th June 2012 my 17 year old nephew Emmerson Hird survived a cardiac arrest.

Emmerson had been playing upstairs on his X-box with his girlfriend Micha. As he went to get up he fell down onto her. At first she thought he was messing about but quickly realised something was wrong and shouted for Emmerson’s brother. Emmerson had now stopped breathing and when his brother phoned for an ambulance he was told to make sure that Emmerson was on the floor and carry out CPR.

Whilst performing CPR he shouted for help. Three neighbours heard him shouting, “He’s dead” and ran inside to help. One checked his pulse whilst another continued CPR until the rapid response arrived.

Micha and Emmerson’s brother were in total shock.

Two police cars and an ambulance arrived at the house. Emmerson’s mum Lesley who had been on her way to a hospital appointment quickly returned home in bits whilst his dad came running in.

At 1:50pm Lesley called me, Emmerson’s auntie Linda, in hysterics. At first I couldn’t understand what she was saying, but when I did, I couldn’t believe what I was hearing. When I arrived at the house Emmerson’s dad and brother were outside crying and his mum, girlfriend, neighbour and police were inside the house. They had been working on Emmerson for almost an hour. They managed to get him breathing then rushed him to Sunderland Royal Hospital, working on him all the way there.

He was taken straight to the intensive cardiac care unit where they put him in an induced coma and packed him in ice to bring his temperature down. Two days later they started to warm him back up and wake him up. We were then told that he had gone into his own coma. He was moved into the cardiac care unit where he stayed for around two weeks. He was then moved to the Freeman Hospital in Newcastle where he had an implantable cardioverter defibrillator (ICD) fitted.

Emmerson came out of the coma about three weeks later. When he came round he was swearing and couldn’t stand being touched or having anything on him. We were told this was due to his sensory glands and he was moved later moved to the neuro ward.

He has 20% vision, memory loss and has been diagnosed with long QT syndrome. He is having physiotherapy, occupational therapy and speech and language therapy. He has now been in hospital for 5 months and he is currently at Walkergate Park in Newcastle where he is getting the best treatment and help possible and is doing well.

We have been told his recovery period will be around two years. Everyone has been through the mill but we thank God that he didn’t take Emmerson from us.

 

Jessica Goddard

Living with long QT syndrome

Jessica Jane Goddard was born on the 27th July 2007. She was 3 years old when in May 2010 we received a phone call that would change her life forever…

My name is Rebekah Goddard and I am Jessica’s mother. I was diagnosed with long QT syndrome in July 2001 at the age of twenty-five, after a series of massive seizures that starting occurring just after my eighteenth birthday. It was my father who effectively saved my life on the day of that first episode. When he found me, I wasn’t breathing and didn’t have a pulse. It was his quick actions and medical training from his RAF days that effectively saved my life. To this day my dad struggles to discuss what he remembers about the day he found me.

As a mother, I cannot even begin to imagine what must have gone through my parents’ minds finding their daughter in such a way. Then later, discovering that the first initial seizure with long QT syndrome has such low survival rates… No parents should have to worry about burying their own child. This is now a worry that I have to deal with on a daily basis with my own child.

I married my husband Ian, two months after getting my own diagnosis of long QT, and having a pacemaker fitted. A couple of years later, we discussed the possibility of having children, and that’s when I hit a stumbling block. At the time, there seemed to be very little information about pregnancy and birth, as well as the possible effects of my condition on my unborn child. So after gathering information to the best of our abilities and a lot of discussion, we decided that with a 50/50 chance of me passing on my condition, we should go ahead and have children.

Our first child, Grace Elizabeth Goddard was born by scheduled cesarean section at Yeovil District Hospital on 27th August 2004.

To say the operating theatre was packed would be a massive understatement! It seems the world and his wife had appeared out of the woodwork to witness Grace’s birth. It was reassuring that so many people were there to help if required. Paediatric nurses, cardiologists, anaesthetists, medical students…

Ten minutes after her birth she experienced her very first ECG!

Rebekah with Grace and Jessica

Aftercare was quickly arranged by the hospital for out-patients appointments to monitor Grace as she grew.

So far, so good.

Two years and eleven months later, the whole process was repeated for Grace’s younger sister, Jessica.

When Jessica was five months old, both Jess, Grace and myself had bloods taken for genetic testing, which we were told could take quite some time. Yet the idea of finally knowing one way or another would mean that we could accept whatever the outcome, deal with it, and finally live our lives to the fullest.

As my mum always tells me, “fore-warned is fore-armed.”

It really did take a long time for the test results.

But now we know.

I took the call from the geneticist’s office completely out of the blue, on a Thursday afternoon. I remember it vividly.

I was asked if I wanted to wait to get the results until my next appointment at the hospital, however, I knew we had waited long enough.

It was funny, as when I was asked what my thoughts on what the test results would be, I instantly said, ‘one has it, one hasn’t.’ The lady was shocked to say the least. I knew then that it was Jessica that had it and I told her as much. She was utterly dumbfounded. I don’t know how I knew, I just did… Mother’s intuition?

I put the phone down and sat for what felt like an eternity, but all too soon it hit me. I sobbed… A lot.

I felt relief for Grace, knowing she was free of it all, but mostly I felt guilt. Knowing it was because of me that Jess now had a difficult journey ahead of her. Not knowing where we go from here.

It turns out not only were the genetic tests positive, she has now had three or four ‘dodgy’ ECGs, showing long QT.

After spending the night in hospital on a telemetry set for monitoring her heart whilst on medication, she is now taking 13mg of propranolol twice a day; easier said than done with a three year old…

So here we are today.

She at least she has some protection now, although whether she will require a pacemaker later on down the line is still under discussion.

She also has something that I never did. Someone who truly understands… Right by her side, every step of the way. No matter how uncertain she will be feeling at times, I will always be there for her. I can hand on heart say that I know how she is feeling. Been there. Done that. Got the t-shirt!

Her story is ongoing, as is her life. But one thing we are certain of is we are not going to wrap her up in cotton wool. Life is to be lived, and I want to her to experience it to the fullest.

Rebekah Goddard

Living with long QT syndrome

Even at the age of nine the signs were there, but no one realised what they meant. I recall going home from school near to tears as I had, had a ‘panic attack’ in the school assembly hall…. yet again. My heart pounded and I felt sick as well as cold sweats and on top of that my head was spinning and it was all I could do to stop myself from collapsing. The whole school was watching and imagine just how embarrassing it would be if I fainted in front of them all. The fifteen minutes between then and break time seemed to be an eternity, until I could take it no more. It got to the point where I begged to be taken to the doctors. But all he said was count to ten and breathe into a paper bag. I resorted to asking the teachers to let me stand on the end of the row so I wasn’t in amongst everyone. I felt safer there, despite the fact I knew they all thought I was attention seeking. Being on the end of the row eased my panic a little, but the attacks still haunted me and not always during assemblies.

At thirteen I went to the upper school where the attacks continued, but I grew so used to them that they became a normality. During my A-levels I developed a passion for photography, which I studied in every minute of my spare time. I eventually sourced some equipment and set up my own dark room in my bedroom.

I finished developing late one night and put the chemicals back into their screw-top containers, washed out the trays in the bath and clambered into bed. It wasn’t long before I drifted off into a deep sleep. The alarm went off and I got out of bed, then everything went hazy. People were talking about me, but I wasn’t there and what was that loud whirring noise. My head was pounding like someone had clubbed me with a baseball bat. I opened my eyes, and everything was blurry without my glasses. Where was I? Why wasn’t I in my own bed? Where was my mum? I wanted my mum.

My mum appeared and when I asked her what I was doing here, she had no answers. Had I had an accident, or been in a car crash? Why couldn’t she tell me what I was doing in hospital? The nurses were none the wiser either.

My parents were used to me being noisy in the mornings, but for some reason on that particular morning a loud bang had made my dad paranoid and caused him to get up to check what I was up to. After no answer to his calls he opened the bathroom door and found me in a heap on the floor. He dragged me onto the landing. I had no pulse and I wasn’t breathing. It was then that my dad saved my life. He had been part of a mountain rescue team back in the fifties when he was in the RAF, and everything he had learnt came flooding back. He gave me mouth-to-mouth and called for my mum to dial 999. She couldn’t even remember where we lived when asked by the operator!

By 11.30am the doctors had completed a series of blood and urine tests which had shown no abnormalities, and so decided, to the disgust of my parents, to let me go home. They put my ‘event’ down to inhalation of photographic chemicals and an adverse reaction. As we were leaving the hospital my dad suffered angina pain, but fighting off the nurses he announced that he was taking his daughter home! What a cool dad!

Eight years on and I had spent the night at a friend’s house. The alarm had gone off and once again everything went hazy… I woke up face down on the floor, then I realised I had wet myself and all over my friend’s carpet. That’s all I could think about until I tried to get up, but just collapsed. My body felt like a dead weight. I dragged myself across the floor and pulled myself up the door before collapsing onto the landing. My friend heard the bang and ran up stairs to find me sobbing and desperately trying to talk, but I couldn’t utter a word, my speech was very, very slurred.

They took me into a different hospital this time, where after being kept in for a week they concluded that I had epilepsy and that it had only just manifested itself. They could not give a proper diagnosis unless I had a secondary seizure. They didn’t think that the event eight years previously and this event were related, but I had my suspicions. The worst thing of all was that they took away my driving licence. I became quite depressed about this, where I live, you can’t do much without a car.

Eight months on and the same thing happened. I woke up in hospital, was violently ill before I passed out and woke up the next day on an upstairs ward. My husband had found me having a massive seizure in bed just two minutes before he was about to leave for work. What if he hadn’t found me and he had left on time, instead of being late like he was? It doesn’t bear thinking about. That seizure lasted an hour and a half before they managed to get me off to hospital. One of the ambulance men remembered me from eight years previous (he remembered the massive hole I had kicked in his door!). He said he had never seen anyone else suffer a seizure for so long, and that’s why he had remembered me.

I started the epilepsy tablets soon after I woke on the ward. I then settled down in my bed to watch the tennis. All of a sudden my heart started to pound so hard I thought I would bounce off the bed. A middle school panic attack only a hundred times worse. I managed to press the buzzer before I passed out. I woke up in a side room and because of that I knew it was serious. I was astounded when they announced that I had a heart problem and that I had to be rushed down to x-ray for a temporary pacing wire to be placed into my heart. They did an ECHO of my heart and then wheeled me down. I was conscious throughout the procedure. Had I not been so shocked I would have been scared stupid, but I found myself becoming intrigued by the whole thing. At the end of it I came out with a large box attached to my arm. My pacing box. I spent the next two weeks on the cardiac care unit surrounded by grannies and middle-aged men with angina and heart problems. I should have had an award for the longest stay on the ward. They monitored me night and day and even tried turning the pacing box down on the odd occasion, but that ended in disaster as I kept collapsing on them. They put a twenty-four hour tape on me and took a reading of my heart and the doctor concluded that I was in need of a permanent pacing box of my own. That Thursday was pacing day in the hospital, and if I was good, I could go home on Friday evening. My heart was beating on it’s own at between 30 – 35 beats per minute as opposed to 60 plus beats.

Nearly a year on and after a little tweaking here and there that pacing box has become my best friend. I can still feel it thumping me now and again and I thought I would never get used to it, but I wouldn’t ever consider not having it. The lump where it lies is annoying still but it’s great for freaking people out! And I tell you what though; it’s a good conversation starter!

Best of all, I made my wedding day, despite everything. I couldn’t understand why so many people cried so much. I guess it hit them more than me that I shouldn’t have made it that far. I never saw my dad cry in my whole life, until he cried openly in front of 100 people. I couldn’t have made it as far as I have without him.

Antony Eames

Tony Eames wrote his story to be included in the myheart booklet which was launched in November, 2013.

Tony

I had my first episode of fainting at 4 years old. From then on I passed out regularly. Sometimes I passed out several times a day. During this time I was not allowed to do any school sport or go out in the playground.

I was never invited to other children’s parties as parents were so concerned that I might pass out. I even had to be escorted to and from school and around the school so that I was never alone in case I fainted.

These attacks continued on a very regular basis until it was finally suggested that I had an ECG and was diagnosed with long QT syndrome, a rare heart condition that can cause sudden death in the young, at the age of 12.

For eight years my family doctor had treated my fainting attacks as epilepsy and panic attacks. It was suggested that I be sent to a psychiatric institution, something my parents refused to allow. No-one had tested my heart and all the time I was at risk of a sudden fatal attack. I don’t understand why it took so long for someone to think it might be a cardiac problem and not mental at all.

After my diagnosis I was put on medication to regulate my heart rhythm. At the age of 18, when in my final year of A levels, I became ill again and required a pacemaker implant to back up my medication. My family and I were devastated to be told I needed an implant as I was now enjoying my teenage years after a traumatic childhood.

Soon after the implant I left the security of home for University life in Newcastle. However I decided from the start to be open to my new friends about my heart condition and pacemaker. This gave me confidence, which was seriously lacking during my childhood.

The implant has enabled me to do far more strenuous exercise. Table tennis, jogging and visits to the gym are regular activities. It is fantastic to be free to participate in so many things that for so long I could not even think about trying. Last year I tested my own confidence and achieved a major personal goal by abseiling down from the Tyne Bridge for charity. This really did test my heart and my parent’s nerves!

Through CRY, my story may help further publicise awareness to the health professions and the public of why young children may be dying suddenly and the simple tests that are available. After hundreds of fainting episodes, a pacemaker fitted and taking daily medication I regard myself as one of the lucky ones and intend to live life to the full.

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Louise Dee

Living with long QT syndrome

Hi, my name is Louise.

It all started in 1990 when I was 8 yrs old. I woke up hearing an alarm clock and realised it was my mother’s for work, so I got out of bed and went to turn the alarm off. When I went into her bedroom I turned the alarm off and tried waking my mother up but she did not wake up, so I then went into my grandparents room to tell them my mother wouldn’t wake up.

After that I didn’t see my mum ever again – she had passed away at the age of 30.

As I got older, I was more curious about what happened with my mum because my family was saying my mum just died in her sleep and I was confused because I thought “How can you just die in your sleep?”

So time passed. Then on August 7th 2000 I got a phone call saying my brother Daniel died in the shower – he was 23. Apparently he had just got out of bed and gone into the shower and collapsed and died. I didn’t know what to think – I was in shock…

At this point I moved back to England with my dad, as we had been living in Australia.

So after another horrific thing in my life had happened, it was scaring me because all my close loved-ones were dying for no reason! So I decided to go to the doctors and explain to them what had happened to my close family. These two deaths had happened in Australia so my mother and brother had not been registered at my doctors in the UK. The doctor was concerned and he sorted me out an appointment to go and see Dr Bowes at Northern General in Sheffield.

I had the ECGs done, the treadmills done and also the standing and sitting down thing to check my heart, but still came up as OK. But I also go dizzy a lot – like when you stand up really quick and you can go dizzy at times – but I go dizzy while I am washing the pots or something! So Dr Bowes decided to put me in for the electrophysiological testing where you are sedated and have a needle in your groin and they thread a thin tube through your vein up into your heart and then they experiment with different drugs to get your heartbeat going fast and slow….

As soon as I came out of theatre Dr Bowes came up to me and said, “Well we see you do have long QT syndrome and you will definitely need an ICD implanted ASAP.”

I was crying my eyes out, thinking “My life is over now!” Also feeling sorry for myself “Why me, why me…?” Simple things like going on fast rides and playing sports is going to restrict me a little bit and I also have two children so they have a 50/50 chance of having this condition.

So 6 months later I had this ICD (implantable cardioverter defibrillator) implanted and now I think if I didn’t have it I would be scared for my life. I feel very secure even though nothing is 100% guaranteed – but it helps hugely.

Now my children are undergoing treatment, but so far their tests have come back OK. I also had a blood test to see if they can find this faulty gene and if they do it will be easy to diagnose my children and other family members for this condition. But up to now I’m just enjoying life with my children and hope for the best that my children won’t have this condition.

Lucy Patey

Living With long QT syndrome

I have always been a fit and active person, participating in many sporting activities – two of which were hockey and horse riding, family passions that I inherited. During my teenage years I competed at top level both on horseback and on the hockey field.

Looking back, I was given so many warning signals – dizzy spells, heart palpitations and severe tiredness – all of which I ignored and simply put down to exam stress or a tiring day on the sports field!

In 2004, I joined P&O Cruises and became a member of Youth Crew looking after children, a fantastic opportunity but a job which is known for its long working hours and solid 5/6 month contracts. During the summer of 2005 I had a series of blackouts or what were also thought to have been panic attacks on-board the ship. My colleagues, along with myself, put this down to working and playing too hard, a poor diet and the tough summer holiday season taking its toll. After a couple of days rest, I seemed to be better.

It wasn’t until Boxing Day 2005 that another collapse occurred, leading to a massive seizure. The ship’s medical staff referred me to a hospital at the ship’s next port (Nassau in the Bahamas) where, after 48 hours, I was flown back home to London. Luckily for me, I had some good friends on board who managed to pack some personal belongings together for me to take home.

After the New Year, I was sent as an inpatient to St Thomas’ Hospital, London, where specialists examined my situation. I will always remember that week, it was my 23rd birthday! Neurologists initially believed that I had epilepsy, however, testing for this came back negative.

I was obviously very happy and very ready to go home, but the neurologist advised me that a cardiologist wanted a quick word with me. On his arrival, I was told to get back into bed and the cardiologist explained that something had been detected on a routine ECG. He couldn’t let me go home and needed to keep a close eye on me for a further 72 hours as “you could walk out of the front doors of the hospital and drop dead.” I was so shocked, I was speechless!

He explained I had something called long QT syndrome which was the cause of all my blackouts and fainting. I was put on a beta-blocker and told to report any further episodes. After a further two, much more serious collapses, the decision was made to give me an implantable cardioverter defibrillator (ICD) which was implanted in the right side of my chest in June 2006. Due to all of this I was no longer allowed to continue my career at sea. Naturally I was devastated.

The surgery for the implant seemed to go well with nothing more than the usual discomfort. However, as time went by I began to experience an increasing amount of pain in my implant site. Stabbing pains would shoot through my chest like bolts of lightning, making me double over with the pain. After months trying unsuccessfully to resolve the pain, the decision was made to re-locate the ICD to the left side of my chest. This was done in January 2008.

The relocation went well but my recovery period seemed to last forever and the pain from the procedure was immense!! I really wasn’t myself and was constantly feeling run down, tired, lethargic and if going out and about would need constant breaks to rest. My legs would seem to collapse underneath me and coupled with this my whole chest was throbbing in pain from the past procedures. I now have severe keloid scarring and my GP has also diagnosed me with M.E (myalgic encephalopathy).

My GP and cardiologists have concluded that my body is unable to withstand invasive surgery of any kind. Tissues and nerves that have been interrupted from past procedures are unable to heal and thus, this is the cause of my discomfort. The pain in my implant site again became so severe that in August 2008 I had my ICD completely removed to try to reduce pain. However, I now am anxious that as I have long QT, am at risk of sudden death and feel that I should really have an ICD for that “just in case” moment!

With regards to the keloid scarring, plastic surgeons have decided to operate and perform radiotherapy which, given time, could greatly improve my situation. I am now 25 and feel as though I am living the life of an elderly person!

It seems that my long QT has arrived with more than just a little baggage and as a result my lifestyle has hugely altered.

Editor’s note: Lucy’s experiences of extreme pain in her ICD site and keloid scarring are not necessarily typical of people diagnosed with long QT. Long QT is a complex condition and the symptoms and side effects can vary according to the type of gene involved, sex, age and length of the QT interval.

Jonny Goode

Living with long QT syndrome

I love Sport! Any kind of sport and ever since I remember I have spent most of my spare time either hitting a ball, pedalling a bike or running around somewhere.

It came as a great shock to me when I was diagnosed with long QT syndrome at the age of 23.

The way I discovered I had the syndrome was by complete chance. I had been out surfing in Cornwall and that evening had really bad chest pains. At the time I thought I was having some kind of heart attack as the pain was so severe. On returning home I got checked out and was told that it was pericarditis. At the follow up appointment I had an ECG and it came back as abnormal, from there I saw a number of doctors until I saw a specialist in long QT syndrome. A blood test was taken and almost a year later it was confirmed that I did have the syndrome.

I was told that I would have to take a beta-blocker each day and my specialist then reeled off a long list of potentially dangerous things for my syndrome. The one that stuck in my head was not swimming in the sea; this was interesting as at the time I was working as a surf instructor and beach lifeguard!

To be completely honest and without wanting to sound arrogant or complacent I haven’t changed much since I was diagnosed with the syndrome. I have a faith in God and believe that he has my number and when my time is up, I will get to run around in heaven. Life is too short to worry. My family is my life. I have a two year old daughter called Poppy and my wife Miranda is pregnant with our second baby due in October.

I look after myself and so don’t drink alcohol or caffeine. I exercise everyday and love pushing my body to the limit. One of my favourite moments is when I finish a race or event and I get to present Poppy with my medal.

In April I ran my third London Marathon, I ran for CRY and feel compelled to help people understand that despite having long QT syndrome it doesn’t have to stop you enjoying life and competing in events whatever sport you enjoy.

This August I will be competing in my third London Triathlon hoping to get in under 2hrs 20mins.

I have completed charity bike rides from John O’Groats to Lands End, Calais to Nice and in a few weeks I am cycling from Nice to Naples. I coach cricket, football and tennis to young people in North Devon and love the way sport can teach valuable life skills and bring the best out in people.

Long QT syndrome doesn’t have to stop you doing what you love. For me it makes me realise who I am and it makes me feel alive.

Hayley Brown

Living with long QT syndrome

I was 19 years old and had just failed my second driving test when my first and only attack happened. I ran through the garden and into the house where my mum was waiting nervously. I told her that I had failed and then went on to tell her I didn’t feel very well, but I collapsed and lost consciousness so never got to finish my sentence. 

I remember waking up in the kitchen and hearing my mum on the phone to someone. I had lost control of my bodily functions and because I had fallen on my left side, my arm was tingling.

The next thing I remember is my dad arriving and I could hear him shouting my name as he ran down the garden path. I was rushed to hospital and had several tests done but I was allowed home that night.

Initially the doctors thought I was epileptic and I spent months seeing neurologists. Its fair to say that this was an extremely frightening time for me and my family. However, the results of numerous scans was that my brain was fine – but my ECG from when I was originally taken in didn’t look right.

I was then referred to John Radcliffe hospital and was admitted for the day for several tests, including ECGs, adrenaline tests, scans and the dreaded treadmill test! They diagnosed me with long QT that day and I’d like to think that I took the news relatively well. I was told that my condition is hereditary and that I had had it since birth. I was put on beta-blockers and cautioned to not over exert my body. I have since had genetic counselling to see where the condition came from, but it came back as inconclusive.

Since then I have had no further attacks. I maintain a healthy lifestyle and I am a keen kick boxer. I climbed Snowden for charity last year and I was determined that long QT wouldn’t stop me reaching the top of that mountain!

I was terrified when I was diagnosed that life would never be the same again and I hope my story will help anyone out there who feels that way at the moment. Overcoming this diagnosis has been one of the hardest but most rewarding things I have ever done and I am living proof that this doesn’t have to rule your life. You take your tablets, take life one step at a time and make the best of the hand you’ve been dealt and it’s not as bad as you think.