Sophia’s Story

Sophia Louise Hart came into this world at 22:25 hrs on 22 July 2005. There was no indication during pregnancy that Sophia would be born with a congenital heart defect. I remember saying out loud when she finally came out (posterior), “Thank God she is healthy”. Little did we know. She fussed during the night, constantly waking and wanting to be comforted. Eventually I took her out to the nurse’s area and asked them to watch her for an hour so I could get a little sleep. They kindly agreed. A half hour later I was woken to be told that something was wrong with my cherry pie and that they had put her on oxygen. I had absolutely no idea what all of this meant and the furthest thing from my mind was that there could be something wrong with her heart.

The paediatrician on call told us that she had a murmur and that her condition would either be related to her heart or her chest, however, he was 90% certain it was her heart. I didn’t even know at that moment what kind of condition she had but I was hysterical, tired and sore and just couldn’t believe that this was happening to us. Sophia was sent to another hospital to have an echo which determined later that day that Sophia had Transposition of the Great Arteries along with pulmonary stenosis and a massive VSD. She was born 8 pound 4 oz, so she was a very healthy weight, and this would help her in the coming weeks. We were devastated as any parent would be hearing news like this. Sophia was flown to Sydney that evening where the following morning she underwent a balloon septostomy. She gained a better colour over the coming days and was able to come home with us. Sophia has experienced only a mild cough/cold since. Apart from the funny shade of blue, she is doing very well. Sophia is a month ahead of herself developmentally and has such a beautiful personality. This summer has had some hot days and she doesn’t cope with them very well. She absolutely needs to be kept cool in summer or she does find it difficult to breathe well, just as she needs to be kept warm in the winter to fight off chest infections. Sophia will undergo surgery (Rastelli procedure) when she is about one year old and hopefully that will be the last until she has a tube change some years later. I am petrified of her first operation. I just can’t imagine watching my baby wheeled away in to the unknown, it simply scares me. But, I will deal with it as I have dealt with lots of things over the past six months.

I have asked myself a million times what I did wrong for Sophia to be born with this condition but I didn’t do anything. I could ask myself until I am blue in the face and I will never find the answer, it just is. As much of a shock as it was to be told our baby would have to undergo open heart surgery, we have accepted it and will do whatever it takes to ensure as best we can that Sophia leads a normal, happy, healthy life as will her sister and brother. I have a little soft spot for her though because already she has done everything some doctors thought she probably wouldn’t and I will continue to have faith that she will keep proving them wrong.

Jason and I would like to thank Professor Graham Reynolds from the bottom of our hearts. His compassion, kindness and obvious love for kids is a rarity. Not only does he give Sophia his undivided attention when he sees her, his understanding of what parents are going through is something I have not experienced with anyone else since Sophia’s birth and it has made a huge difference in how we have dealt with her condition. We are very lucky to have him looking after Sophia.

Update from Julie 12 April 2006

Jason and I visited Sophia’s Cardiologist – Dr Stephen Cooper on 6 March for a regular check up. We weren’t expecting him to tell us that it was time to start arranging Sophia’s surgery. We thought she might be fine up until 18 months, however, Dr Cooper felt that she was in excellent health and her anatomy was perfect for the surgery so he didn’t see a reason to wait. We were referred to Prof Richard Chard in Sydney and visited him on 5 April. Prof Chard also felt that the surgery should be done sooner rather than later, therefore Sophia will undergo surgery on 19 June 2006. Originally the date was set for 11 May, however the hospital theatre was not available so was pushed back to June. This is fine with us as it will give us more time to prepare ourselves and for Sophia to get just that little bit bigger and stronger.

We are not sure yet whether Sophia will undergo the Rastelli procedure or the Nikaidoh procedure. The Nikaidoh procedure was suggested to us by Prof Chard. As Sophia’s condition is very rare, this procedure has not been performed very often, particularly in Australia. However, this surgery has superior long term effects for Sophia and if fully successful she may never have to undergo another surgery. The Rastelli procedure would definitely require Sophia to undergo at least two further open heart surgeries to replace conduit tubing. The only draw back for us is that the Nikaidoh procedure can take up to 12 hours to perform. This is a long time on the heart/lung machine. I guess we will be discussing all of this further with Prof Chard as the time for surgery draws closer.

I will update again when she has had her surgery and is fighting fit!

Update from Julie 17 July 2006

Sophia underwent her open heart surgery on 19 June. It was such a harrowing wait, however, five hours after surgery she came out. We could not believe how pink she was. In fact, we didn’t realise how blue she was until she had her surgery. Sophia had a Rastelli procedure. This will mean that in the next 5 to 20 years she will need a conduit tube change, although it will probably be closer to the ten year mark depending on how much she grows and how well her body adjusts to the conduit tubing in her heart. For now we are just relieved that she is with us and already fighting fit after 3 weeks post op.

We would like to thank Dr Richard Chard for his precision work. Dr Chard, you really are a blessing to us. Thank you so much from the bottom of our hearts for taking on our little girl and giving her a better than average chance at life. What you do for these children is nothing short of amazing and we hope for those little lives lost that this life saved makes it worth you doing what you do.

Thank you to the wonderful staff at Westmead hospital, you made this experience bearable and we simply can’t thank you enough.

A huge thank you from us to Ronald MacDonald House. The generosity of the staff and volunteers is nothing short of commendable. We would have found it very difficult to survive financially had it not been for this wonderful organisation.

Thanks to Cecily from Heartkids for visiting us, it was very much appreciated.

And, last but not least, our deepest thanks goes to Prof Graham Reynolds for his post op support and care of Sophia. Sophia developed some fluid on some of her organs and Graham helped us through it. It just doesn’t get better when you have someone like Graham looking after the best interests of your child.

Update from Julie January 2007

Sophia is now 7 months post op and doing beautifully. She has had no sign of rejection of the conduit tubing and pretty much runs around like a chook with its head cut off. The surgery has changed her energy levels like you would not believe and some days I just can’t keep up with her. It is wonderful to see her so full of energy and life. She had a visit with Dr Murphy in November who indicated that she was in excellent health and a pleasant surprise for him. Sophia is now on 6 monthly check-ups. Sophia is a tiny little girl which in this circumstance suits us just fine. The faster she grows the closer the time will come when she outgrows her tube and needs a replacement. I am more than happy for her to remain a skinny weed for the simple fact that she may just get a few more years out of that tube. I am not quite sure why she is so skinny because she eats more than you would think an 18 month old of her size eats. In fact, she is half the size of her 2.5 year old sister and eats far more.

For now as I have said before, we just enjoy life with her and when the time comes for her to have further surgery, we will deal with it then. What I want to say to those parents who are struggling to come to terms with the fact that their child has a heart condition, is that, there is so much hope for our kids. What can be done for CHD now is nothing short of amazing, and most of our kids will go on to lead very normal lives. I guess the more normal a life they can live makes them just that little more special.

Julie – Sophia’s mum

Update from Jason April 2013

Hi, the recent passing of Sophia’s mum, Julie, has prompted me, Sophia’s dad, to update her story, as it has been a few years.

Sophia is now in year 2 at school and doing really well.  She is also now heading into her third season of Minkey hockey – which she loves. Who would have thought 6 years ago that she would be running around the hockey pitch with all the other kids her age and scoring goals! She makes us so proud. It was only last week that her older sister had a cross country carnival and was a little puffed out with about a kilometer to go, so Sophia sprinted off to run the last leg with her – holding her had mind you.  It was really precious.


Sophia at a Hockey carnival last year (2012)

Sophia has also recently started taking guitar lessons, which she has been nagging us about for over a year now.  I think she now wants to be a rock star!

Sophia is still quite little (skinny) for her age, no matter what we feed her. She obviously has a fast metabolism, which would make most of us envious. She still has her annual cardiologist check up, with one due next week.  As she has not shown any negative signs since her last check up, we are not expecting any bad news – but you never know.

Although I have three children, Sophia holds a special place in my heart as she has always been so brave and adventurous and also doesn’t mind showing strangers her “zipper”, which remarkably is so fine now you can’t see it unless you are up close to her. I hope if she needs another surgery this can be done without opening the chest cavity – she is a pretty little girl after all. With advances in modern medicine we are optimistically hopeful.


Sophia with older sister and younger brother

For other parents who are facing the early stages of introducing your curious and outgoing child into their chosen extracurricular activities. Over the last few years we have learnt to let Sophia just be herself and have a go at whatever she wants to try.  This was quite difficult at first, but its not like she has been asking to play full contact football or anything like that, and besides she’s stubborn and persistent like her mother. I think most important is that all kids get the opportunity to try as many things as possible with minimal obstacles – there will be enough of those in life later.


Sophia at her birthday last year (2012)

Anyway, on behalf of Sophia and the family, take care and remember to give generously to Heartkids Australia.

As a side note, Julie, Sophia’s mum, who passed away in March 2013 was an organ donor, and we were chuffed to find out that her heart was given to a child. What a most fitting outcome and a true reflection of Julie’s spirit.

Jason – Sophia’s dad.


 

New South Wales