Assignment three …
Today’s assignment: write the post that was on your mind when you decided to start a blog.
Actually more tricky than it looks. Or at least for me. My very first blog was started over at Blogspot just before I underwent a pulmonary valve replacement surgery a few years ago. The point was to document what I went through: my thoughts and fears, what happened in hospital before and after, what I could do and what I couldn’t do. I did it too. At least for a time. The ‘prequel posts’ were done. Even the “Hurrah – I survived!” post-op post. And then it dwindled. I wanted (and this makes me feel ashamed) someone who was undergoing the same procedure as me, perhaps with the same condition that I have, to read it and go “Oh – well maybe it won’t be as scary as I think … I wonder how my progress will differ from his …”.
I talked briefly in a previous post about the condition I have. Tetralogy of Fallot – a congenital heart defect. For starters, I hate the term ‘defect’. Yep. I’m aware that the way my heart was assembled is not the way that yours was. I’m aware that its a serious condition (survival rate for untreated tetralogy of Fallot is approximately 75% after the first year of life, 60% by four years, 30% by ten years, and 5% by forty years). I understand all of that. I just don’t consider it a defect. Perhaps I should explain why.
First of all – what is tetralogy of Fallot (TOF)? As I said, its a congenital heart defect involving four anatomical abnormalities of the heart. It helps if you remember your biology lessons about the human heart …
- Pulmonary Infundibular Stenosis – A narrowing of the right ventricular outflow tract (usually at or just below the pulmonary valve) caused by overgrowth of the heart muscle wall.
- Overriding aorta – The aorta is situated above the ventricular septal defect and connected to both the right and the left ventricles.
- Ventricular septal defect (VSD) – A hole between the two ventricles of the heart.
- Right ventricular hypertrophy – The right ventricle is more muscular than normal, causing a characteristic boot-shaped (coeur-en-sabot) appearance as seen by chest X-ray.
How much the aorta overrides can vary a lot between patients, as can the size of the VSD, as can the level of right ventricular hypertrophy. I just went to look at the copies of my notes sent to me following hospital visits to see if any of these are clarified for my personal case but for some reason I can’t find them. I have another check-up in March this year (must remember the date!) so I’ll ask some more details then, as well as a copy of the subsequent notes to be sent to me.
TOF is also a cyanotic defect, in that unoxygenated blood from the right ventricle flows into the aorta preferentially because of the obstructed outflow tract into the lungs. This means less blood has the opportunity to be oxygenated in the lungs. However, I wasn’t considered a ‘blue baby’. According to my mother, the condition was caught by a locum GP in a regular post-natal check-up. Following that, I underwent my first open heart surgery when 18 months old, performed by Professor Sir Magdi Yacoub (he was awarded the Order of Merit in the 2014 New Year Honours, I have just discovered). Apparently he unblocked the valve that was blocked (which one, I currently don’t actually know) by poking it with his little finger. Although I’m sure it was more complicated than that, it makes for a pretty cool story. I remained under the care of Dr Rosemary Radley-Smith, a paediatric cardiologist at Harefield Hospital for many years.
As the youngest for four high-spirited boys I was never going to be wrapped in cotton wool. In fact, my parents made the decision that if I was to live a foreshortened life then it had better be a bloody good one and not one spent staring wistfully out of the window (as it happened, as I got older I often preferred to stay in with a book than go outside, but as a young child I spent a great amount of time outside; running, jumping, climbing trees …). Nor was I to be treated any differently than anybody else – at school or at home. I knew the limits. No contact sport. If I break a bone then immediate antibiotics. Take care of teeth. (Bacteria in the bloodstream = bad.)
There were yearly visits to Harefield to track my progress. Then once every two years … I mentioned that I wasn’t a blue baby (low blood oxygenation without cyanosis is known as “pink tet”). I also didn’t suffer from hypercyanotic spells (also known as tet spells) nor digital clubbing, dyspnea or polycythemia. One area of concern was my growth … but not in the way expected. Children with TOF are not expected to grow or have ‘normal’ physical development. Perhaps I should point out right now that I’m 6’4″ (193cm) and have always been a *cough* healthy *cough* weight. Yep – it turns out that I grew too fast for their liking and they were concerned that my heart wouldn’t keep pace. Fair enough. But as it stands, not too much to worry about.
I’m not entirely sure how, but for several years I missed my check-up appointments. Granted, for 4 of these years I was living in France (well, ish), but from the age of 16 until 20 I don’t remember going to Harefield. Anyway, I returned from France and was living in a houseshare with another guy (not like that). Then one winter I contracted the ‘flu and lost consciousness on the bathroom floor. Not great, but all things considered not a big worry. Then the next winter I contracted it again and passed out. This time, however, I was standing at the kitchen sink and went over onto a flagstoned floor. I don’t remember how long I was out for, but it was longer than a few seconds. Stupidly, I took myself off to bed and didn’t tell anybody what had happened (despite the fact that my brother worked not more than a 2 minute walk from where I was living). I woke up after several hours and made an appointment with my doctor, where I mentioned (oh-so casually) that I hadn’t had a heart check-up for some years. He said he’d sort it.
That was … possibly 2005.
I went back to Harefield for a check-up and the consultant I saw (a really lovely guy who has now retired, but I’m afraid that I don’t remember his name) made it clear that I would need surgery again to replace my pulmonary valve. I didn’t realise it at the time, but 90% of patients with total repair as infants develop a progressively leaky pulmonary valve later in adulthood and therefore require surgery. I had no idea that this would be part of my future. I thought it was a one-and-done kind of deal. Shame on me for not educating myself on my own condition.
A few more years of tests and ‘watching’ and then the decision was made that the surgery was to be booked in when the surgeon could do it.
I started a new job in the July of 2008. 11 months later I was back in hospital (this time the Royal Brompton in central London) having my second round of open-heart surgery. And that is a post in itself … But everything went smoothly, and I have a pig valve in place of my regular old worn out pulmonary valve. The best part was not having to take any blood-thinners due to not having a mechanical valve. The surgeon preferred to use organic valves and I have no objection to pigs so we agreed that if he could then he would, but there was no guarantee as it all depended on what he found upon slicing me open.
Actually, the best part was coming home and then spending 3 months in France before going back to work.
And since 2009? No problems. Not that I ever had any. Regular check-ups, this time at the Brompton under the auspices of Professor Michael Gatzoulis (although I’ve never met him), and another valve replacement in about 30 years (by which time it is hoped that it’ll be keyhole surgery and not open-heart).
There is an Australian cricketer, Beau Casson, who is also a TOF patient. An article appeared in 2008 that mentioned it and that he had undergone open-heart surgery three times before he was 25. The opening paragraph sums up my feelings about this … defect:
Beau Casson is uncomfortable with this story. He fears it will convey him as different, a victim, a man somehow worthy of your sympathy. So, before we begin, he asks this: judge him for his spin-bowling, not his medical history. View him as you would any cricketer.
I don’t want sympathy. I am not my medical history.
I guess that’s what I wanted to say.