Life as a GUCH goes on

Nearly 30 years post Fontan surgery and still life as a GUCH goes on!! I was born in 1973 and diagnosed with Tricuspid Atresia with ASD, VSD and right pulmonary artery stenosis at 3 months old. My mum was insistent

Written by Wendy Knowles

I was born in 1973 and diagnosed with Tricuspid Atresia with ASD, VSD and right pulmonary artery stenosis at 3 months old.  My mum was insistent shortly after my birth that I was cyanosed and would not feed  too well. I was discharged from hospital post birth with what was described as ‘a heart murmur’.  As the months went by my breathing became more and more affected and I struggled to feed with constant chest infections and obviously my blue tinge remained.  My mum would persistently inform the doctors and health visitor of her concerns, but this was not followed up further. Eventually I was rushed into hospital by ambulance with a chest infection and later transferred to the University Hospital of Wales paediatric cardiology team where after numerous tests my parents were called to the cardiac surgeon and consultants office to be told there was nothing they could do for me, described my condition in basic terms and informed my parents to ‘take me home and love me’ and I was quite simply ‘going to die’.  My parents were 19 years old at the time and astounded, devastated and angry at this news.  For months my mum poured over medical texts in the public reference library trying to understand my condition in more depth and clarify if there was more that could be done in relation to my treatment plan.  In those days the information available on congenital heart disease was limited so the best my mum could do was look up the medical meanings of tricuspid and atresia separately and put the two together to understand my diagnoses in better detail.  In 1975 the Cardiology team at the University hospital of wales informed my parents that they could consider the Waterstun shunt which would prove significantly high risk for me, but there was a chance that it could prolong my life, being only palliative in nature was emphasised to my parents during their decision making proces.  After much thought they decided to take the gamble as they described me as being extremely sick at this time.  I was underweight, not developing physically and so listless I couldn’t even crawl, never mind the option of learning to walk.  The waterstun shunt went ahead in the July of 1975 and my parents were told as I was wheeled to theatre ‘don’t be surprised if she doesn’t come out of theatre alive’.  Well survive I did…. a few days later I was bouncing up and down in the cot, with pink skin instead of blue!!

My early childhood was categorised by a healthy period until the age of about 5-6 when I began to deteriorate.  At the age of 2 I was also diagnosed with a congenitally dislocated right hip.  Another issue overlooked at birth and only discovered when I began learning to walk, this has also affected my spine and caused scoliosis.  I remember beginning school a year later than my peers but I loved it and I enjoyed reading books and drawing. I became increasingly lethargic, tired and unable to participate in most of the activities my peers were engaged in, but I would still sit on the sidelines in the cold to be a part of the action in a minimal way or be carried on my best friends back when I was too tired or breathless to walk anymore…Where there was a will, there was a way and that is pretty much how I have lived my life!!   At around the age of 7 we were informed that I may have to travel to France to undergo the then ‘pioneering fontan operation’.  Later we were told that the operation could be performed in the UK by Professor Yacoub and that my care from then on would be transferred from the University Hospital of Wales to Consultant Cardiologist Professor Jane Somerville at the National Heart hospital, London.

My brother and I in the summer of 1982, pre my fontan op.

In July 1982 we met Professor Somerville for the first time and it was like someone had finally flicked on the switch to ‘hope’.  We were told that there was something that could be done for me and in the October I was admitted for a long batch of tests and of course to undergo the pioneering fontan operation.  On the 2nd November 1982 I was wheeled to theatre unknowing of the amazing life that I was to have post operation.  I was aware however of the hope that was to lie ahead in that my life would improve on a physical level.  The kids from FAME song ‘Starmaker’ was number 1 in the charts at the time and shortly post op the film E.T was released….I still love both of these to this day as they remain symbolic to me of the life that my fontan has given! In terms of my fontan structure, this was described as a modified fontan as Professor Yacoub created 2 fontan connections.  One from my right atrium to right pulmonary artery at the site of my previous waterstun shunt and pulmonary artery stenosis and the other from my right atrium to the right ventricle (Bjork modification).  I also had a repair to my ASD.  My recovery was a challenge and I had several admissions to hospital in the year that followed, but I never looked back!  I felt alive, like a ‘real child’ one who could walk, run and not become so breathless.  I learnt to ride my brother’s bike and play on his roller skates (even with the dislocated hip which has never been repaired due to the priority of my CHD over the years).  Even at 8 years old I guess you could say that subconsciously I had made the decision to respect the gift of life I had been given and wow was I going to make the most of it!!  I spent my last 2 years of primary school in a school for children with physical disabilities.  Although academically the schools provision was limited, I learnt a great deal about other aspects of growing up and life with a health issue/ disability.  I made friends with children who sadly passed on and some I am still close to today and have helped to get me through some of the toughest times in my life.  In terms of transition to comprehensive school, I travelled nearly 50 miles each day to attend a neighbouring local authority comprehensive which catered for children with health problems/ difficulties.  No local comprehensive in the area I lived would accept responsibility due to my health issues, but this particular one did and my mother was determined for me to have an academic education and achieve my maximum potential, even if it did mean having to travel nearly 50 miles daily to obtain this education. and so I did.

My secondary school years were quite lonely ones for me at times.  I never knew anyone with a single ventricle heart condition or who had undergone a fontan and worried about what the future would hold for me.  I did experience some verbal bullying, which at the time I did not realise, but I had chosen to internalise my feelings on this matter simply to increase my own determination and aid my success.  I did however sustain a few friendships, some I still maintain to this day.  I particularly liked Art as a subject and would use this as a therapeutic process to express my feelings.  I also flourished academically (except in maths) and at 15 I was informed that it would be highly unlikely I could ever have children of my own due to the risks that pregnancy would pose due to my CHD.  At this time, I also discovered the subject Sociology and instantly knew this was the career path I wanted to follow and sought to become a social worker, not giving a second thought to not being able to have children of my own one day .This perspective still resonates with me to this day as being advised not to have children again at 18 and 27 years of age was one limitation I was not prepared to gamble with.   I proceeded to do well in my GCSE studies, however around this time there was discussion of fontan conversion surgery as my right atrium- right pulmonary artery fontan connection had begun to narrow.  After completing my GCSE’s and whilst studying for my A levels I was referred back to Professor Yacoub to discuss conversion surgery.  In August 1992 nearly 10 years post my fontan I and some of my family met with Professor Yacoub for the first time in 10 years.  My brother was 12 at the time and insisted that he wanted to attend the appointment too in order to meet the man who saved his sisters life.  Professor Yacoub’s advice at this time was to ‘go and enjoy my life’ that I did!!  I proceeded to university and obtained a 2.1 honours degree in Sociology and Education in 1997.  In between my first and second year at university I underwent a balloon dilatation to my narrowed fontan connection which made me feel considerably better afterwards.

I began my career as an unqualified social worker uneventfully until I begun getting fast heart rhythms.  My care at this time was overseen by the Royal Brompton hospital and having promptly been informed that the rhythm upset was a result of the fontan I thought no more of this.  However, as my career progressed and I completed my social work diploma to become fully qualified, I began to tire easily, have raging headaches and sickness, the arrhythmia persisted with increased breathlessness.  In September 2006, I was informed that I was probably indicating symptoms of deterioration.  It was then that I was told of the change in thinking re: TCPC conversion surgery/ transplant.  In January 2008 I was informed that TCPC conversion surgery would be an unlikely option as would transplant in my future. There were further discussions regarding an ablation procedure of which I underwent in May 2008.  I was left with right diaphragmatic palsy diagnosed as a complication 2 months post procedure and by August the atrial tachycardia had returned.  My dosage of Metoprolol beta blocker was increased, but when I attended clinic one day in April 2010 in tachycardia I was told that I would need to be admitted in a few days time to undergo a cardioversion.  When I arrived in Paul wood Grown Up Congenital Heart (GUCH) ward at the Brompton Hospital 4 days later my heart had returned to normal rhythm.  I was then offered an ablation for the 2nd time to which I promptly refused, I did not wish to pursue this option again as my diaphragm had only recently healed and so I requested further exploration be made with respect to the TCPC conversion operation.  The Brompton would not proceed with this option, but agreed to refer me to both Southampton GUCH unit and Queen Elizabeth Hospital, Birmingham GUCH unit for me to have suitable discussions with them regarding TCPC conversion surgery.  It was at this time that I decided to transfer my care to the Queen Elizabeth Hospital GUCH unit, Birmingham as a permanent measure.  I had decided that if I was going to require frequent hospital admissions from there on, it would be a more accessible GUCH unit for my family and friends to visit me and more convenient and comfortable for me to travel to for clinic appointments.  I also knew Dr Thorne from when she was senior registrar to Professor Somerville at the Brompton Hospital many years before so I felt safe and confident in her care as a specialist GUCH consultant.  I cannot say it was easy for me to transfer my care from the Brompton hospital as I felt like a part of me as a GUCH had gone.  I had met many GUCH friends there over the years and some I have lost, not to mention that GUCH as a concept really began there with Professor Somerville as it’s pioneer.  However, with careful consideration and discussion with Professor Somerville, I felt it in my own best interests to be cared for in a GUCH unit closer to home and more easily accessible.  In October 2010 my official transfer was complete and I attended my first appointment with Dr Thorne in Birmingham.  Again a whole batch of tests were undertaken and in April last year I was told that the conversion fontan maybe feasible based on a catheter to determine PVR and pressures.  In terms of complexities indicative of conversion they determined these as follows:  Right pulmonary vein compression from my enlarged right atrium, the right atrium- right ventricle connection is contributing limited forward flow, narrowing in the right pulmonary artery- right atrium connection at the previous stenosed site and the remaining tachycardia.  The catheter performed in July last year confirmed adequate pressures and left ventricular function.  I was referred to Consultant Cardiac surgeon Mr Barron based at Birmingham Children’s hospital and had a consultation on the 9th December 2011.  Following this consultation I weighed up all the evidence/ research I could find on the conversion fontan, spoke to a few other GUCH’s who had undergone this process and obviously expressed  to both Dr Thorne and Mr Barron the view that I wished to meet with both Professor Yacoub and Professor Somerville before making my final decision.

In the last 10 months I have soul searched, reflected on my past, all that I have achieved, all that I am and all that I still wish to be.  I finally made the decision in February that I was not prepared to undertake the risk of the TCPC conversion operation and rush to such high risk surgery with unknown outcomes.  I have therefore chosen to retain the amazing unique fontan that Professor Yacoub undertook nearly 30 years ago.  I have achieved so much in my life; I have travelled and visited many countries including Egypt and New York.  In Egypt I climbed inside the largest pyramid and visited the University of Cairo where Professor Yacoub began his medical career all those years ago.  As I stood at the university gates, I reflected on the life that this wonderful man had given me.  I adore music and love going to concerts.  I am a bit of a rock fan to be honest and my favourites are Pink Floyd, Ozzy Osbourne, Metallica and Fleetwood Mac.  The last concert I attended was at the O2 arena last May to see Roger Waters perform The Wall album in its entirety.  I cried from beginning to end.  If there is one album that has got me through all the toughest times in my life it has to be this one.  I remember my mum and dad playing it on vinyl for the first time when I was 5 years old and I became instantly addicted to Pink Floyd’s music after hearing it.  When I went into hospital for my fontan I was this tiny 8 year old child who would sit on the hospital bed playing rock music through a pair of huge earphones that were highly popular and fashionable in the 1980’s. It got me through!!……

I have 4 lovely brothers and 1 sister whom I have witnessed studying at college, university, obtain employment, grow and mature and have children of their own.  The day my eldest of 4 brother’s obtained his place at Medical school to study Medicine was one of the proudest moments in my life.  I cried buckets of happiness!!  I have watched my beautiful niece and 3 nephews grow and develop and want to be alive for as long as possible for them.  I have an amazing mum who has been my rock over the years and is always there for me and made the biggest decision of her life in both 1975 and 1982 signing the surgical consent forms to help keep me alive, well and safe.  I am so proud of all that my mum has achieved, including a 2.1 Bsc Honours degree which she obtained in 2009.  On her graduation day, I cried bucket loads again!!!  I also have a supportive dad, stepdad and friends.  I am currently able to continue working part time as a Social Worker (3 days per week) and when I can no longer maintain this, I aim to complete a Masters degree part time in Criminology.  My next big goal for the forthcoming year is to travel to the Grand Canyon and on the 2nd November I and my family shall be celebrating the 30th anniversary of my fontan.  We haven’t quite decided how we are going to mark this occasion yet, but have considered travelling to London to see a show on the West End and visiting the Tate Gallery.

When it came right down to the final decision of fontan conversion surgery I just simply could not gamble with losing all that is precious in my life.  I do not know what the future holds aside from the fact that I will continue to deteriorate, but I know I have made the correct decision for me and my family.  I have had a wonderful life so far and my mottos are ‘life goes on’ and ‘on with the show’.  On bad days I sometimes need to repeat these to myself constantly……

To all CHD parents and children/ young people just beginning this lifetime journey, I would say, always believe in hope and I offer the following tips:

  • Where there is life there is always hope, but I also consider that without hope, there is no life so it can work both ways.
  • Support your child in everything they do and encourage them to find their own limits, growing independence and live to their maximum potential.
  • Maintain all hopes, goals and dreams as anything is possible once you have identified and accepted your limitations in life with a CHD!
  • Obtain as much information / knowledge as possible about your child’s condition and if you feel it would be of benefit read research/ evidence as well as the information provided by your child’s Consultant.  It provides a wider understanding of the issues/ questions you may later have and can aid in any decision making processes.  Also when your child is of an age and understanding to grapple with their CHD it is good to encourage them to do the same and to always ask questions, no matter how trivial they may seem.  Asking the Consultant Cardiologist to draw a diagram of the anatomy and physiology of your child’s heart is also highly useful.
  • Talk, seek support and communicate with other CHD’s and parents, always remembering that no two people’s cardiac anatomy/ physiology or coping mechanisms will be the same, but experiences can be shared and there will likely be some common ground.
  • Spread the awareness that CHD is lifelong and not something you acquire all too often I have been asked the question how long have you had your heart condition. My response is always. umm. pre birth, but 3 months old before I was officially diagnosed!!
  • Involve extended family members/ friends and the child’s siblings as often as possible and where appropriate in your child’s treatment plan.  Many occasions my brother’s have had to explain my condition if I am unwell or assist me if I needed them to over the years.  Sometimes they have nagged me to slow down or comment when I am cyanosed.  It also helps siblings to feel more involved and have a greater understanding of the situation and feel that they are important too.  
  • Most importantly.maintain a sense of humour and positive attitude!!!  Even in the darkest of times I know that most of us GUCH’s and our parents and family members are able to demonstrate a wicked sense of humour and see the positive in every negative it’s a coping mechanism most GUCH’s I have met have acquired and adapted over many years of hospital stays, surgery, tests, bad news, etc, etc throughout their lives.the list is endless!

Well that is all from me!  I will leave you with a photo of my mum and I at the top of the Rockefeller building in New York on my 35th Birthday in 2008!!  Best wishes to all. The CHD journey never ends but hope prevails!! ; )  I think of how lucky I am to still be here every single day and remember all the friends with CHD I have lost over the years’ My heart breaks for them and their families, but still it also beats on and reminds me that this is what they would want it to do so that I can continue living my life to the full..The gratitude and respect I also have for Professor Sir Magdi Yacoub and Professor Jane Somerville is immeasurable and I will never forget the life that they gave me on that cold November day in 1982.

Mum and I in Egypt – October 2006
My brother and I in the summer of 1982, pre my fontan op.

Isla’s Story

When my husband and I went to my anomaly scan at 21 weeks I was so excited to see how much our baby had grown since our earlier scan and this being my 2nd baby, I knew how much baby’s grew and how amazing this scan was!

Read More »

Finlay’s Story

This is Finlee’s story as told by his Grandparents

Finlee Thomas Grimshaw (DOB : 18.09.2009) – aged 2 years and, what an eventful two years it has been!

This is our story of Finlee living with Pulmonary Atresia with Ventricular Septal Defect (VSD).

Read More »

Jade’s story

It all started on the 25th May 1995, Jade was 9 days late but she was here and she was beautiful. When the doctor came round when she was 5 days old I had no concerns, he checked her all over and then he listened to her heart. This is the start of our journey……

Read More »