11/ Times are changing...

The date for the replacement pacemaker was set and couldn't arrive soon enough. My consultant had explained how the current device was causing the right and left ventricles to ignore each other and beat how they wished. The result of this being that there was no synchronicity of their contractions, often resulting in both ventricles contacting at the same time and rendering my heart function very poor and inefficient. His plan? Well that was simple, a different type of pacemaker, a re-synchronising device that would listen to what was happening with the left ventricle and ensure never again should the two ventricles wrestle for dominance. It would require disconnection of one of the pacemaker leads currently used and insertion of another two to facilitate proper function. The unused lead would just lay inside me redundant as the surgery to remove leads can be problematic and at times dangerous. This cardio resynchronisation therapy pacemaker (CRTP) sounded just the ticket. I'd struggled for nearly a year with the original device, I was just thankful to be shot of it.

The surgery passed smoothly, much to my amazement, and I was readily discharged following fine tuning of the device. I quickly had a sense of relief that the device had eased a lot of the problems I had been encountering. I gradually became stronger and more able to return to more normal activities. I still suffered quite badly at times with breathlessness but the cardiologist had said much of this was down to the now very poor condition of my tricuspid valve separating my right atria and right ventricle. More and more blood was being pushed away from my lungs as the valve gradually got progressively worse. The consultant had said it would need to be addressed and that he would be having consultations with his surgical colleagues about the possibility of needing to surgically replace the valve, most likely with a mechanical valve. We would discuss the outcome at my next outpatient appointment with him in a years time.

The year came to pass and I was at best just ticking by. I was much better with the new device but the simple acts of day to day life, work and family would render me exhausted by the end of each day and my resolve was challenged practically every day. To have to repeatedly get up, struggle physically to dry after showering and putting socks on, to get dressed and then go to work for the day before coming home to start my Dad role was a daily chore of having words with myself that today was not the day I said "Enough". Stubbornness has always been my best trait to ensure I drive on even when my body is screaming at me to stop. The day I give in is the day the disease wins. I won't allow that to happen, not yet that's for sure. I want my children to see their Dad isn't a quitter and that you can't just cave in when life throws challenges at you.

I arrived at the hospital for my outpatient appointment, keen to find out what the results of these year long consultant discussions held. Would they advise they felt a new valve would be beneficial? If not, what would the alternative be? I was called to the office by a doctor I didn't recognise. Please no. Please don't let this be another example of a consultation when I'm meeting a registrar who doesn't even know what ARVC is and I need to sit there again and explain once again all of my history. That had happened plenty of times before and it was just frustrating. I would leave from those appointments none the wiser about my condition or the future plan, all it achieved was to provide a doctor with a free CPD session at my expense. I was ushered in and sat down.

"Hello Nick I'm your interim doctor, until we have appointed a permanent replacement for your consultant"

"Wait, sorry. Where is my consultant? He's left?" I quizzed the young doctor.

"Weren't you informed? Didn't you receive a letter about your consultant's early retirement?" the doctor asked, looking somewhat embarrassed.

"No! I wasn't" I responded abruptly out of pure frustration and a bit of anger. I recall feeling tired from the travel to the hospital and the frustrating thought of having to go through all of my medical history again was evidently written all over my face.

"I'm aware that you are here today to hear the conclusions of your consultant's discussions on how to proceed with your tricuspid issue" the doctor said.

Thank goodness, he knows, he unlike so many before him, has read my notes and has the answers for me. I suddenly felt very relieved.

"I am yes, I've been waiting for today for the past year to know where we go from here"

The doctor held up a thin cardboard folder and allowed it to fall open. With any of my files this would have caused a potential landslide of paper to fall out all over his desk. However not on this occasion, the folder was empty.

"I know you were expecting answers today and I wish I were able to give them to you. However I'm afraid any consultations your consultant had must have been corridor conversations because there is absolutely nothing in your files. I'm very sorry and can only apologise" his empathy was evident.

I can't write the thoughts that passed through my head at that moment as they are unprintable, but to surmise I was extremely disappointed and felt totally ignored, as though I just didn't matter. To his credit the doctor was very nice and listened to everything I had to say. He proved over time to be good to his word and promised to act on my concerns about my current health and how I felt the device still wasn't working as well as I felt it possibly could, to offer me more freedom and comfort. Over the coming months he proved to be very helpful, getting me on a treadmill to tailor adjust my device settings himself exactly to my body's response to exercise and exertion. He really did help me where I felt before I had been brushed aside at times as being too tricky to sort. I desperately hoped he would take on the consultant role permanently but alas it wasn't to be, he had accepted a consultant role at another hospital closer to his family. He promised to do a full proper handover to the replacement consultant who was due to start in a couple of months. He didn't let me down and after a coupe of months I had an appointment to meet my new consultant.

The day came to meet the new consultant and I sat in the waiting room eager to meet him. I was called into the consultation office and was greeted by, a young registrar.

"Please could you tell me a bit more about your.............A....R....V....C" he asked as he checked the papers in front of him to ensure he had the letters of the disease in the right order. For crying out loud! I've had a belly full of this now!

"With all due respect, I was under the impression I was meant to be meeting my new consultant today. We need to discuss what the plan is to deal with my tricuspid valve regurgitation as it's getting very debilitating now"

"He is consulting with another patient next door at the moment, but you can discuss with me"

"Do you know what the plan is? What has been proposed? Are we talking about a replacement valve and if so does that mean sternotomy again or is there a possibility of it being feasible via keyhole? "

A blank expression stared back at me.

"I'll just go and see if the consultant is free!"

I sat alone in the office feeling incredulous that once again I was getting brushed aside, the case that is too troublesome, the one who doesn't just need a stent or a bypass, the one who has a complicated disease that morphs over time and is hard to treat. Maybe if they continue to ignore me I might go away I thought, problem solved.

The door opened and the consultant walked in and introduced himself.

"You have a very complex history Nick, the surgery you had was very unusual" he went on to say.

"I'm unsure whether it is wise or not to look to replace the valve as the right side of your heart shows a very advanced stage of disease. It might prove very problematic to successfully replace the valve given the amount of cardiomyopathy you have"

"But the valve is really causing me problems. I'm really struggling to be able to do many day to day things the way things are now. If the valve can't be replaced then where does that leave me with future options?" I enquired.

"I suggest I talk to some of my colleagues and see what we collectively think. I suggest we make you a follow up appointment for two years from now and then we can see how much the disease has progressed and it will give us an indication of the rate of decline" he suggested.

"Enough" I thought. In that statement he clarified exactly what I had hypothesised. Ignore my case for two years to see how bad I get? "Fudge that!" I thought, the road had come to an end right there, all trust had evaporated.

I returned home to inform my wife as to the day's events. No interim tests ordered, not even an echo or a stress test, just the usual pacemaker device check ups, just go away for two years. "I'm not feeling this at all love" I said, "I need to get a second opinion. I don't accept that this is the end of the road for me. If they won't take my disease seriously then I will go to someone who will".

I've always been keen to understand my disease and my prognosis and research had taught me in the very advanced end stages of the disease the only treatment option available, if you are lucky enough to qualify, is heart transplantation. I felt with the last few years rate of decline that a transplant may well be my only hope in the next decade or so. I researched where best to go for support for ARVC that could also offer the best option of support for transplant should that time come. All my research pointed me to the Royal Brompton hospital which has a plethora of top cardiologists, many of whom also specialise in ARVC. Better still the Trust also incorporates the Harefield Hospital, the centre held in high regard globally as one of the top transplant centres. If these guys can't help me then nobody can I thought. I approached my supportive GP who agreed to refer me for consultation to obtain a second opinion.

I was told my first day's consultation at the Brompton in London would be a whole day's experience. They would perform many tests including, echo, ECG, exercise stress test, blood, urine, holter all before seeing the Consultant in the afternoon. I was seriously impressed. It was more tests in one day than I'd had in the previous three years combined. All tests were performed swiftly and very professionally. The afternoon came and I was called in to meet my new consultant.

My wife and I entered the small office and were greeted by the consultant himself as well as three other doctors, all huddled in the corner of the small room.

"Do you mind if the doctors are present for your consultation Nick?" the consultant enquired.

"No, not at all, no problem" I replied.

"It's very rare to come across a case quite like yours, especially given your treatment history" the consultant explained.

Oh, I see, I thought, all gather round to look at the wierdo! The consultant and I painstakingly went over the past two and a half decades of my life in minute detail, him pausing often to ask me "why did they choose to do that?!", to which I would reply each time "I don't know, the cardiologist insisted it was the best option for me", pleading my innocence.

"Basically I was wanting to know your opinion on my best option moving forward." I requested of the consultant.

"My last consultant thought there was something to be achieved by replacing the tricuspid valve. What is your feeling regards that approach?"

"I think that would be a disaster" he responded without a shadow of a doubt or hesitation. "Your heart is very weak and misshapen on the right side and even if you could fit a valve, it would likely add more resistance to the circulatory system making your condition worse. Further, I can't think of a single surgeon in the country that would undertake an operation of that magnitude on you. It is not a feasible proposition at all". I really appreciated his boldness and certainty.

"Your disease is so advanced now there is nothing more to be achieved with further surgery on your heart. I also have severe concerns that you don't have an implantable cardioverter defibrillator (ICD) to shock you if you were to have a sudden cardiac arrest (SCA). You are at serious risk of a SCA and if you were to hit the floor, I for one don't fancy your chances of getting up again". My face must have been a picture.

"I will make a referral to my colleagues at the Harefield for assessment, I feel your only option now is heart transplant".

I felt his words form the fist that proceeded to punch me hard in my stomach, the hardest punch I'd ever felt. Transplant had been something that had popped in and out of my head from time to time, but I was so convinced that was still a long way off in the future, if ever. Yet here I was, now, with an extremely experienced, well respected consultant sat before me and he uttered the words, they felt so concrete and unquestionable. Hearing it out loud all of a sudden made it all feel very scary. I looked to my wife as I tried my hardest to keep the tears in my eyes, desperate not to blink and cause one to break free and roll down my face. I needed to stay strong, I couldn't let her see me struggle with this.

We were still in a state of shock as I drove us home down the motorway, discussing all the events of the day and starting to work, as we always do, on our plan to tackle this next hurdle. I turned to my wife nervously laughing and said;

"I might be 41 but I really want a grown up to make this decision for me!"