Living with hypertrophic cardiomyopathy
Jack, our six year old son – usually referred to as Jack Wright – left for school as usual on the morning of Tuesday 20th June 2006. He was arguing with his eleven year old sister Abigail and Mum Tracey on the way out of the front door, with his hair sticking up despite attempts to calm it with water before leaving. He even noticed the first rose on a bush at the front of the house and commented how beautiful it was, before heading off to the car, game boy in hand. Life couldn’t have felt more normal.
That morning he’d got up, had his breakfast and came upstairs to get ready for school munching a piece of chocolate he had found, which his Mum had left on the arm of the sofa the night before. This I think had started the argument with Abigail, as there was only one piece and Jack had eaten it without a thought of sharing – typical six year old boy!
I was working at home that day. The Sunday before, I had completed the British Heart Foundation London to Brighton Charity Cycle Ride and thought it appropriate for my body to have a rest for a few days after. My being at home was to be a blessing with all that followed.
At 11:30am I got a call from Tracey who was very upset, saying that she had received a call from Hampton Lucy Primary School and that Jack was being taken to hospital in an air ambulance. At this time there were no more details available – all the school had told her was that Jack had collapsed and that they were taking him to Warwick Accident and Emergency. This was later changed to Walsgrave, Coventry.
Tracey had been working in Leamington Spa that morning. She had gone straight to Warwick only to be told that the plans had changed and that it had been decided that Jack would be taken to Coventry – she made her way there on her own. Looking back I don’t know how she managed it, as she was extremely distressed.
We have visited A&E with Abigail numerous times, so at first I was relatively calm and thought that the school were being over protective in having Jack air lifted to hospital. I expected him to have broken something falling off the play area, as he collapsed at 11:14am – the last minute of school break time. I locked our house and left keys with our neighbours so they could let our elderly golden retriever out – explaining what was happening, still fairly calm at this time.
With the news that Jack was still at the school I decided to try to see him before they left for the hospital. From our home in Kineton to Coventry, I was going to virtually pass the school door and thought seeing him would both reassure Jack and ease my concern. On arrival at the school, I ran to the rear of the buildings where the air ambulance was parked on the sports field. One of school assistants showed me where to go – she was crying. Only when I saw the helicopter did the reality and seriousness of the situation hit me.
The crew were fabulous, encouraging me to talk to Jack and let him know I was there. Jack lay motionless, very pale with a grey tinge to his skin. He looked so small, so frail and most importantly to me he didn’t show any sign of moving or responding to my voice. It was clear he was very ill and my thoughts turned to his need to be in hospital quickly. I asked that they go as quickly as possible to Walsgrave and that I would meet him there. As the helicopter took off, I was on the phone to Tracey explaining that I had seen him and contacted Tracey’s parents, as it was obvious Tracey was going to need their support.
The police took me to Coventry. The prospect of driving after the shock must have been apparent and their kindness was much appreciated. For the first time, I got an understanding of how slow people react to the sound of a siren – all the way, all I could think of was “get out of the way!”
Tracey was waiting for Jack at the Walsgrave. She commented on how scruffy he looked as he emerged from the ambulance – his shirt grubby and too small (last clean one in the drawer that day), trousers a little too long and shoes needing a good clean, as did his finger nails. Its funny the things you notice at a time like that, but it made her feel pleased that he had obviously had a chance to play and have some fun. We were later told that he had been having a good time and was running around the playground with his friends, when he just stopped and put his hands against a wall for support before collapsing – falling to the floor, right in front of his best friend. Some children went for help, as Jack “would not wake up” and from that point started the amazing effort to bring him back to us.
By the time I arrived at the hospital Jack was surrounded by medical staff. He was being manually ventilated and at one point I counted 22 people attending him. At this stage nobody knew why he had collapsed so everyone was involved in the diagnosis.
After a chest x-ray, it became apparent that Jack’s heart was enlarged and discussions ensued about what should happen next. Time seemed to stand still but when I looked at my watch hours had passed seemingly unnoticed. It is strange, but we have come to realise, with great surprise, how true this is in hospitals when you would expect time to drag!
The decision was taken to contact Glenfield Hospital, Leicester, as they have extensive experience with children with heart problems. A retrieval team was requested and preparations were made to transfer Jack.
Upon arrival, the retrieval team busied themselves examining Jack. Pete Berry introduced himself, to Tracey and I, as the Team Intensivist and said he would like to talk to us somewhere quieter. A room was provided and Pete explained what had happened to Jack. His heart had become enlarged due to a condition called hypertrophic obstructive cardioyomyopathy or as it is knows in medical circles HOCM. The walls of the heart become too thick, affecting the electrical conductivity required to allow the heart to pump properly. The result was an arrhythmia or irregular beat culminating in a heart attack. Pete praised the efforts of the people at Jack’s school, stating that most children do not survive such an attack and added that their swift actions in administering CPR, along with the fantastic response of the air ambulance had undoubtedly saved Jack’s life.
He went on to say that Jack was still very poorly and that his chances of survival were very poor and that we should prepare ourselves for the worst. Pete also warned us of a possible hypoxic brain injury due to Jack’s heart having stopped – this passed us by largely at this stage but was to play a much bigger part later in Jack’s condition.
All I remember of this in detail was Tracey saying “he can’t die”.
I signed his transfer papers after Pete warned us of all the risks attached to moving him – there was no choice, he needed the care at Glenfield and there was no option of him staying at Walsgrave.
The journey to Glenfield was very distressing, as Tracey and I contemplated the loss of our little boy. The same boy who only hours earlier had made us laugh and made us cross with his mischievous behaviour.
On arrival at the Glenfield we searched frantically looking for the paediatric intensive care unit (PICU). Pete and the team greeted us with reassuring smiles that all had gone well with the transfer and Jack was stable. Huge relief, several hours had passed and other than the ventilation, Jack was coping on his own with no further issues with his heart. The ventilator, we were told at the Walsgrave, was supporting his breathing rather than breathing for him to alleviate any further strain on his heart.
Pete wanted to talk to us again along with a cardiology consultant – we feared the worse naturally. A more thorough explanation of Jack’s condition this time with conversation about the likely cause – genetic or virus induced. Tracey, Abigail and I were to be screened immediately to rule out problems for us. Amazingly the consultant started talking about Jack’s future and that when Jack has children they would need to be screened as the condition was likely to be passed on. At this point Tracey commented her surprise and asked him to clarify that he was now saying that Jack had a future – only a few hours earlier we were looking at a life for our family that may not have included Jack! Now we were offered signs of hope. This was to be the beginning of the emotional roller coaster – highs and lows of proportions I cannot describe.
He spent eight days in intensive care, with 24 hour care from nurses dedicated to him, working 12 hour shifts (and how dedicated they were). They spent most of their time sat at the end of his bed monitoring him and the machines he was hooked up to; writing down, seemingly continuously, the details they gave as well as being bombarded with hundreds of questions from us and our family. They were great too in caring for and keeping us amused, entertaining our daughter and finding ingenious ways to hang the ever increasing number of cards that kept coming over and around his bed. They even put up with his mum sleeping in a chair at night by his bed, until one of them very kindly moved her to a little room with a fold out bed so at least they got her out from under their feet, whilst I tried to sleep in a room a short distance away.
He looked so lovely and peaceful except for all the wires and tubes surrounding him. He also looked so out of place – the other patients he had for company were very poorly babies. We wondered whether we were luckier – we’d had Jack in our lives for over six years, whereas for these other parents it was hours, days or months. We had been privileged to watch him grow and turn into such an amazing little person – or was that worse? We were not sure.
The day after his cardiac arrest, Tracey, Abigail and I were screened for this genetic defect and given the all clear. We also met with the surgeon who could fit an implantable cardioverter defibrillator (ICD) and pacemaker on the next day to monitor and control Jack’s heart rate. If his heart went into a life threatening irregular arrhythmia again and failed to function, the ICD would shock it back into sync again. However, this did not happen as the surgeon was not available and provisions would be made for Monday.
We were also told that they would be weaning Jack off the medication that was keeping him sedated and paralysed to see how he would react. We were told we were looking for him to wake up and pull at the tubes up his nose. We waited for this all day, still reading Horrid Henry stories, audio books, songs, singing etc, just looking for any sign. It came just before eight that evening. I had left the ward only minutes before, leaving Tracey with one of our friends. Jack was listening to songs on an iPod, only seconds into Barry White’s “My First, My Last, My Everything” – a song sung many times in the car (Tracey’s choice not mine) – it came. Not the ‘appropriate response’ we had wanted, but the sort we didn’t want. Jack’s eyes disappeared into the top of his head, his back arched so severely that it lifted him out of bed, doctors and nurses were there in seconds and once again he was sedated. A CT scan was arranged and within twenty minutes he was being taken down to assess what damage there was to his brain. We waited for what seemed to be an age to be told that they could not see any obvious signs but would need to do an EEG to find out if his seizure was epileptic, or a one off and a more detailed MRI that would give us a better idea of what we were dealing with. The EEG showed no signs of seizures which was good news. We just had to get through the weekend.
We kept our vigil at his bedside, with numerous visitors, watching and listening to the endless bleeps and alarms of the monitors. The lows – Jack’s heart rate going sky high and very low, the monitor even flashed 0 one morning, it’s alarm sounded very loudly and staff came from every where. One nurse (she was the nurse who had waited way past the end of her shift for the CT scan results, sometimes even hardened staff can be affected) even flung herself onto his bed ready to give CPR. We just watched dumbfounded, then Jack’s heart kicked back in seconds later and back to normal and probably not for the first time.
Jack was transferred from the security of PICU to a room on a general ward, but with no dedicated nurse, just a very paranoid and over anxious Mum. They were still lovely and just as kind, it was just hard to get used to. It was here we were given the most unwelcome and grave news, the results of Jack’s MRI were not just localised to one area of his brain as originally thought, but we were told it was global and that once again his outlook was very poor indeed and we should prepare ourselves once again. We’ve been there before and he got through it, we just hoped he would do it again. We were told we would need to be transferred to the Leicester Royal Infirmary (LRI) where they have a Neurological Rehabilitation ward. That was a whole different ball game.
It was actually a ward with children and babies with anything from epilepsy, asthma, reflux, viruses and all manner of things. Again we met more dedicated wonderful staff, with tales of hope of a couple of other children who had made recoveries of varying degrees. We knew that Jack was not a run of the mill case for them and here shortfalls, inefficiencies and incompetences were all too familiar and were highlighted. We know that the NHS has budget constraints and our own private health cover was not going to be of any use as they don’t have paediatric rehabilitation facilities. The NHS is adequate as far as they were concerned, but had he been 18 they may have been of some use. How wrong they were. Despite best efforts of therapy staff, Jack was having major problems, primarily due to not being able to position him and support his body that had no control over itself – except for the stiffness or ‘tone’ as it is called, that distorted his body daily, leaving problems that can no longer be put right, no matter how many pillows, rolled up sheets and towels. We even used his teddies to jam by the side of his head for support and to put in his hands to avoid his nails digging into the skin and to try to relieve the fists that his hands were permanently making as they had nothing else.
The most frustrating thing of all was that we did not even have a wheel chair to move him around, just to be able to get him off his bed and sitting up (7 months on, Jack still can’t sit up at 90 degrees, despite hours of physio). The summer of 2006 was one of the hottest we’ve had for a long time and I think for Jack’s 10 weeks stay in hospital we were able to take him out for short times for the last month of his stay in a ‘day chair’, that took 3 people to steer. It was not designed for outside use – the nurses and occupational therapy (OT) staff had to turn a blind eye as it was not really suitable, but we had nothing else and I don’t think they wanted to question an already angry mother.
During Jack’s last 2 weeks in Leicester, quite by chance after nagging, chasing and then quite by mistake through a conversation with a therapist at the Children’s Trust in Tadworth, Tracey got the number of the one person who has probably gone out of his way, bucked and played the system, to get an old unused chair that met Jack’s immediate need to at last be sat up at last not slumped and be able to go outside easily especially by his sister. Incidentally, he worked for the very department that Tracey had asked the OT team to get in touch with almost two months ago to sort a chair in our own area of Warwickshire!!! Some system.
One thing that became apparent, early in July, was that Jack was not getting access to the right facilities that they seem to have in Birmingham and Oxford. We were even given the possibility of looking at moving Jack to another hospital where facilities were better and they were used to seeing lots of children like Jack. We could not even get Jack’s consultant’s stand-in to speak to one of the consultants there, despite his secretary freeing up time, they just kept telling us that he was in the best possible place. Every week something would happen or not happen that would remind us that this was not the case and again Tracey would hound the consultant and his registrar. They did finally concede that things should have been better and they should have some basic equipment and that this would be rectified, too late for Jack but hopefully someone else’s child would benefit.
The only glimmer on our horizon, was the ray of hope our consultant had told us of on our first day at the LRI – The Children’s Trust in Tadworth, Surrey, where they have a rehabilitation centre for children with acquired brain injuries (ABI). This was our goal and we would at least try to move heaven and earth to get him there. Again we came up against the bureaucratic red tape of the NHS and the frustration of inadequacies and the inefficiencies of things not being done because someone is on holiday for a couple of weeks, whilst our little boy was in great discomfort and distress
The NHS is an amazing organisation – when he needed it to save his life it was un-faulting, everything possible was done and seemingly no expense spared. It has its faults too – the frustration of not having simple basic equipment to aid Jack’s recuperation, which in the end has caused other complications, short sighted and unnecessarily as we kept pointing out, but that is another story in itself.
Jack did eventually get to the Children’s Trust, in record time according to his consultant. I am convinced it was just to get rid of his Mum and her constant nagging and at the time of writing that is where he is today, making the slow but good progress that we could only have hoped for. He has started coming home at weekends and spent over a week at home for Christmas. We are getting there, Tracey says it’s like having a new born and just getting the household up and ready takes all morning.
We have met so many amazing people here, not just the staff but the children too and their families. It has become our second home and we have realised something that we never thought we would say about our situation, we are lucky – ours is not the saddest tale on the ward but one of hope and we are so lucky that we still have Jack.
It is without doubt that we owe so much to the love and dedication and abilities of the staff and a parent at his school (an ex-nurse who had turned up early to collect her own child!), the Air Ambulance crew who were available and able to reach him so quickly and do what was necessary (we were amazed to find out that they are a charity and receive no support from the government) and the hundreds of skilled medical and rehabilitation staff that have met and cared for our little beautiful boy.
Now it is up to Jack to prove the professionals wrong, he always was a stubborn little chap so hopefully this will stand him in good stead. No matter how long is takes, we have time, we also have the love and support of our family and friends, but best of all we still have Jack and his wonderful big sister Abigail, who has been an amazing tower or strength and help to us. She has kept us going and reminded us that we still needed to function as a family. Some days are better than others, some days it’s hard to get out of bed, but now Jack gives us smiles and with them lots of hope of what could still happen.