Living with pulmonary atresia with intact ventricular septum

OHS at two days old (1980) and three years old (1983)

So here I go – finally putting pen to paper after all these years. I have recently been inspired by so many people who I have read about just lately after learning about The Somerville Foundation. Who knew there were more like me in the world? I feel like I’ve found long-lost, virtual siblings. Lockdown and coronavirus have brought me something that I never knew existed, and I only stumbled upon it after trying to call hospitals to see if I was one of the ‘vulnerable’ ones. These people, teamed with my fast-approaching 40th birthday, has inspired me to tell my ‘story’ which, to be honest, I never thought of as a story at all.

A quiet matter

My heart condition has always been a quiet matter. Never really discussed with friends and only very close family members understanding the technicalities of it and remembering the seriousness of it. It has always been one of those things that has been mentioned, but never really at the forefront of my mind. It was something I had, along with two 10-inch scars and various puncture wounds on my body, but never something I ever really cared about too much. Whether this was a fault of mine or my parents’ way of handling things I will never know, but it always remained a quiet matter. My parents were told that I may never walk, would spend most of my life in a wheelchair and would quite possibly have learning difficulties. Their advice for this, back in the 80s, was to go home and have another baby. My brother was born 21 months later, not because of the advice, but because my parents wanted me to live as normal a life as possible – which included having a sibling.

My parents never took photos of me in hospital like I’ve seen so many families do over the years. I understand this completely. Why would you want to capture the most terrifying time of your life on camera? But for me and for now, out of interest, I would have liked to have seen what they had to go through and what I looked like at that time. My mum tells a story of when she first took me out in the pram and a passer-by looked in to coo at the newborn and said, “Well, she doesn’t look ill,” and I think that was just it. I was lucky. I didn’t have the paper-thin skin and tiny, skinny limbs or the dusky blue colour around my eyes and lips. As far as people saw, I was a bonny baby and therefore I was fixed and able to carry on with life as normal.

Competitive sport

To me, this is why it was probably a well-kept secret amongst just my nearest and dearest. So, I carried this tradition through my childhood – a happy and very healthy one, I might add. I was only ever aware of my condition on a few occasions. School was sometimes an issue. P.E. during primary school was my biggest bugbear. I was always top notch at sports and particularly competitive sport. I was a good sprinter and won flat races with ease. Changing in a classroom full of girls and boys, though, sometimes proved tricky. Boys in particular would notice the huge scar down the middle of my chest, and I would get called a few names like, “Zippy.” It all changed when I hit secondary school though, and my condition was hidden once again. Unfortunately this wasn’t so with P.E., when my hospital decided that I could no longer do competitive sport. So, my sprinting days were done, and they’d hardly begun. I remember this day like it was yesterday. I had a new lady doctor and her words were, “No more competitive sport.” I remember my parents looking at each other and nodding. I rolled my eyes and huffed and puffed about it and got rather upset. “What does she know? She doesn’t know my body!” But nonetheless my protests fell on deaf ears and that was the end of it. I still took part in cross country, but I vividly remember my sports teacher shouting, “Purcell, that’s enough, stand out!” I look back now and think they were only doing what was best for me. I liked to push myself physically to see what I could do, but to allow me to carry on would have been like having blood on their hands if I’d suddenly collapsed and died!

My mum enrolled me into ballet classes when I was three and thank God she did. Dancing was my absolute love through my childhood, particularly ballet, and what better discipline to have to learn about how far you can push and train your body without a massive cardio strain. I loved it and was good at it.

A little bit different

Another time through my childhood that I vividly remember that made me very aware of my condition was an evening round my grandparents’ home. Every Tuesday my dad would take my brother and me to see them while he shaved my grandad. He’d had a terrible stroke some years previous and my dad would go round in the week to help him out as he was very immobile, and my grandmother was his main carer. This evening, she had two elderly lady friends around. As my dad and grandad left the room my nan asked me if I would show the ladies my scars. To be fair, I was more than happy to do so as no one had ever seemed interested in my little uniqueness before, and my nan seemed to be so very proud to be showing me off. I look back now and the memory of it isn’t bad or good, it’s just there, and comes and goes like all my memories related to my condition. I am neither afraid nor happy to have them. They just stay in my memory to remind me every now and then that I am just a little bit different.

Great Ormond Street Hospital

My early memories of being in Great Ormond Street Hospital are happy ones. My memories don’t include any pain or sadness at all. I remember a couple of the nurses’ faces and I remember where one of my beds was, along with a couple of the other patients’ beds. I remember playing with guinea pigs and actually feeling rather special. One girl that was being treated was considerably older than me and she used to let me play with her ‘Cindy’ toys. My parents became friendly with her parents and even after she sadly passed away, they remained friends for some years. Although back in the 80s there was no particular support put in place for parents, there was a sense of CHD community. Over the first three years my parents made friends or were put in touch with other parents who were going through similar experiences. One couple lived very close to us and ironically, we moved to the same village when I was 18. They had a son who died when he was three of several complex defects. The two mums had become very close through the experience and I remember him being a friend I could play with and always loving his furry brown slippers. I remember the day the mums got together and he wasn’t there anymore. I didn’t understand at all. I’m happy to say though that the families remain friends to this day.

Strangely, the only time I ever remember experiencing any pain was when I got home from the OH surgery when I was three. I remember being very sore with my new scar down my chest and my parents having to put ‘special tape’ on it to heal. I always wanted to pull it off myself though and I can still feel the soreness and smell the medication on the tape. My trips back to hospital were generally lovely. I enjoyed going back to GOSH and was always made to feel special and like I’d achieved so much without really having done a thing. My recovery was really all down to the expertise of the specialists and fantastic care and parenting; however, I took all the credit. For the majority of my life my heart condition had always been something that was affecting my loved ones, and all I had to do was just keep on being me.

The beginning of anxiety

That is until 1992, when ironically the said new lady doctor decided that a catheterization test was well overdue and decided to have me in for a little look around my heart before I started secondary school in the autumn. I wasn’t at all phased by this as my experience of hospital was always very nice. At 12 years old I felt very grown up and was more than happy to take control of the situation and told my parents I was fine and let them go to wherever they were staying for the night. I was put under general anaesthetic the very next morning and the procedure took place successfully to everyone’s knowledge – that is until I came round… I will never forget the pain I had when I sat up. The sharpest of pains penetrating from the top of my back through to the front of my chest. I remember telling my parents and that was it. I collapsed into my dad’s arms. This is all I remember until I came round again with curtains around me and a team of doctors and nurses pressing buttons on monitors and connecting drains into my left side. During the procedure of putting the camera up through my groin to my heart, a blood vessel had been burst which bled into my lung, causing it to collapse. From what was meant to be a routine procedure and a three-day stay in hospital, turned out to be a week.

This must have been a very impressionable time for me, as this was where my anxieties of going back to hospital appointments began. It also proved to leave some anxieties around my immediate family too. My brother struggled to cope with the ‘not knowing what was happening’, and some years later told us that he thought I’d died – a very horrible ordeal for a 10-year-old boy.

After several days recovering at home, I developed what we now know to be paralysis and aura migraines. At the time it was a scary experience as the underlying thought was that I may be experiencing a stroke, but it was confirmed to be migraines. I have suffered with migraines ever since I can remember – excruciating headaches and bouts of sickness – but this was on another level. In later years I have often questioned if they are related to my heart defects, but I have always been told that it’s unlikely. It is something that niggles away at me, however.

Pregnancy and CHD

Since then my CHD has, touch wood, never affected me at all, apart from the anxiety of going to hospital – completely unnecessarily because I have always had positive yearly visits. I chose to ignore completely my condition all through my teens and most of my twenties. In fact, if I’m honest, I had very little respect for my condition and regrettably sometimes took it for granted that I was healthy and normal. That was until I met my husband in 2008. I opened up straight away about my heart condition, which was hugely unusual for me around new people. I became pregnant in 2009 which kickstarted a whole new respect for my healthy heart. It was always advised that a pregnancy should be planned so each stage could be monitored carefully and kept under complete control. However this wasn’t meant to be and luckily I sailed through pregnancy, with numerous scans, plans and fetal scans back at GOSH. It was lovely to return after being discharged from there at the age of 18 and put under the care of the Heart Hospital in London.

I was induced two weeks early, as the baby started to look too small on the late scans I was having, and it was found that the blood going through my placenta was minimal. My baby girl was born on Valentine’s Day with no complications for me at all. I refused the epidural they wanted me to have in early labour and actually enjoyed most of the whole experience. My daughter was in SCBU for 10 days due to her very small size and small abdomen caused by the breakdown of the placenta, but otherwise I took home a very healthy, all be it tiny, baby girl. It was without a doubt my CHD that saved my baby, as I was being so well looked after (being classed as ‘high risk’) and having so many scans throughout, to detect her size. We would have most definitely lost her without this extra care. I am a great believer in fate and know that my parents went through their trauma with me to save our trauma years later.

A child with CHD can lead a normal life with brilliant medical expertise, a caring family and a lot of love

After this I married my husband and went on to have another child, where again I had absolutely no complications at all. Since having my children I have a new-found respect of how lucky I am and what my parents went through – and still go through today – worrying about me and if or when things will ever change with my heart health. Particularly after reading some other heart stories I do feel well and truly blessed. I am a success story – even though I had never even considered it before. I am proof to those parents who are now in the same situation as my parents were almost 40 years ago, that a child with CHD can lead a normal life with brilliant medical expertise, a caring family and a lot of love.

Sarah Richmond