Felix

Felix and big sister Sophia

I would like to introduce our family and in particular our little miracle man – our son Felix – to the group. Felix is now 22 months old and we have a daughter, Sophia, who was 4 years old in May. We are Aussies, who have recently moved to Tokyo, Japan.

When I was 19 weeks pregnant with Felix and living in Sydney, I was having a routine ultrasound, when suddenly the nurse asked us to go out of the room and wait for our baby to turn as she couldn’t get a good picture of our baby due to the position. We waited and waited, not suspecting anything. Then the head doctor arrived and called us into his office. He said we have found some “problems” with your baby and that he would like to do an amniocentesis to see if it was a chromosomal disorder as he suspected. We felt like the bottom had dropped out of our world. I was totally in shock. This couldn’t be happening?

Without having time to think about anything, I went straight into the procedures room and had the test done without further ado. The doctor explained that he thought that either Felix had some kind of genetic disorder, which would eventually prove fatal OR he had a condition called Dandy Walker Variant, accompanied with some ?irregularities? with the heart. He sent us straight to see a Paediatric Cardiologist, who did a scan and agreed that there were ?irregularities?, but couldn’t precisely confirm what they were. He thought that the left side of Felix’s heart looked small, but couldn’t say more.

We waited for a couple of days before the rushed results of the amnio came through. What an awful 2 days. The results came back that all of his chromosomes were ok. I was sent for an MRI, which confirmed that he had Dandy Walker Variant, which is a cyst in the cerebellum of the brain. At this stage, they weren’t really too worried about the heart. The cyst took centre stage.

Over the next few weeks, we visited many doctors, neurosurgeons, paediatric cardiologists and genetic counsellors and we weren’t hearing very good news from anyone. We were told that the prognosis was poor and that “most” people in our situation would terminate. The doctors kept reminding us that we had another child (a daughter aged 2) and what kind of negative impact a seriously ill little brother could have upon her. We were kind of being rushed along with our decision as if we left our decision for too long, we would have to appear before an ethics committee to explain why we wanted to terminate (if we decided that!)

It was such a cruel situation – being made to act as god to our child. The doctors gave us a huge range of possible outcomes for the Dandy Walker Variant from very severe (never recognising us, being tube fed for his whole life, totally dependent for life etc.) to being completely ?normal? (what or who is normal??). We did our own research over the internet and spoke to a friend who is a neurosurgeon. She dug up all of the information she could on the topic and again, we were left with little hope.

During this time, we had a few counselling sessions with our doula (female birth companion – similar to a mid-wife, but not medically trained as such), who was also a close friend of ours. Denise got us to talk through our thoughts and fears and was an immense support to us all. As there was so much doubt as to the outcomes, we decided to give Felix a chance to prove himself both to us and to our doctors. We decided to not do any more ultrasounds until 36 weeks as we couldn’t bear to go through the weekly rollercoaster ride of emotions associated with scanning, waiting and lots of very, very vague information from doctors.

Needless to say, the pregnancy was fairly stressful from that time onwards and even though I tried to continue as per a non- high-risk pregnancy, it was always in my mind and I felt as if I was falling apart. Even writing this now, over 2 years later, I am still very, very emotional about it.

Sometime in the last 6 weeks or so of my pregnancy, after another “routine” visit with my obstetrician, it was decided that I should go to a different hospital to give birth to my son as they were expecting that he would need heart surgery after birth. This also meant changing doctors. Again, this really scared us as we had come to know and trust our doctor so much over the previous months.

At around 36-38 weeks (the details seem very vague right now!), we went to see our paediatric cardiologist again for him to do the final scan before I would have the baby. What a traumatic visit it was to become. Our doctor did the scan and the news was grim. The left side of Felix’s heart was still much smaller than the right and he suspected (though couldn’t completely confirm) HLHS. He gave us 3 options:

1. Heart Transplant
2. A series of heart reconstruction operations (which would mean our whole family moving to another state in Australia – really a small sacrifice!!)
3. compassionate care (bringing home our baby and waiting for nature to take its course)

With this news on board, we were more upset than ever and I was totally convinced that I wouldn’t even get to hold my baby. I came home from the doctor in a real mess and went into my bedroom. I had just set up Felix’s bassinet in our room next to our bed the previous day and when I saw it sitting there all ready for him, I was just devastated. I really thought that he would never get to come home with us!

(I am sorry that this story is so long? just wanted to give everyone the full picture? I apologise for rambling!)

To cut a very long story short-ish. I went into labour at 40.5 weeks and laboured for about 3 days or so at home before going to hospital. I thought that I was in established labour and that I would be almost fully dilated and ready to go. (I should add here that I was attempting a VBAC ? Vaginal Birth after Caesarean ? as my daughter was born by c-section after 5 days of labour and a failure to progress.) When we arrived at the hospital, I was 1cm dilated and was devastated (again ;-) at this news. We decided to go for another c-section as we didn’t want to put Felix in danger and also because I felt like I couldn’t go on any more with such a long labour. I was prepped and our wonderful baby son Felix was born at 5am by epidural c-section on 6th November 2003.

He was taken from me without me even getting to see him. (I still feel very sad about that to this day!) I could hear him crying as they checked him over and hear the doctor saying that he was big (3.89kg) and “handsome”, but I only glimpsed the tip of his little hand as they wheeled him off to the NICU (Neonatal Intensive Care Unit). My husband went too as we had previously decided to stick to Felix “like glue” after he was born.

After I had finished in recovery, I was sent back to my room and not long after that my husband came BOUNDING in the door saying “he’s ok, he’s ok” and “the doctor wants you to go down and breastfeed him right now”. I couldn’t believe what I was hearing. He hadn’t needed any oxygen, hadn’t needed intubation and he was doing really well AND I could breastfeed. It all seemed too good to be true.

Felix stayed in the Grace Ward at Westmead Children’s Hospital (Sydney, Australia) for 9 days and then we took him home with us. Felix took a long time to put on weight. I was breastfeeding every 2 hours and expressing in between feeds and giving that to him in a bottle. (I thank my lucky stars that I even got to breastfeed him at all!) Eventually, after Christmas 2003 and our ?force feeding? ;-) holiday, our paediatrician was blown away by the enormous amount of weight Felix had gained. He was finally on the right track.

We have had regular paediatrician and paediatric cardiologists appointments and to date he has not had any intervention for either his heart or the Dandy Walker Variant. Apart from initially being slightly delayed with his walking (possibly due to the cyst) and several trips to hospital with bronchiolitis and pneumonia, he has been fantastic.

He was diagnosed with Shones Complex (Disorder) in November 2004, though our doctor has told us not to worry about it too much. We did our own research and then started to worry. I guess that it must be hard for our doctor, but when I ask him what the prognosis is for Felix in the long-term, he says that he really doesn’t know. He may need an operation next year, in 5 years or never. He told us to try to go on with life as normal and not to treat Felix as being different or sick. He got very cranky with me for worrying so much (I told him that it is my job as a mummy to worry!) and said to explain to Felix that his heart is just a bit different ? not that he is a sick boy.

Felix is a wonderful little boy with a great sense of humour and a real love of life. He makes us laugh and he is a real blessing in our lives. I know that we may not be out of the woods as yet, but we just have to love him and nurture him as well as we can and cross our fingers (and our toes!). I just can’t imagine not having him around to light up our days.

I would love to get in touch with other Shones Complex families to try to get some more information on the subject and to be a supportive and understanding member of the group.

Warmest wishes,