Blog 3 - The First Two Years

Mum and Dad dressed me in a hand made frilly dress, a bonnet and booties, wrapped me in a delicate pink shawl and took me home. This was a time of joy but also heartache. My parents had to come to terms with how their baby looked, not knowing whether I would live or die. More questions but no answers...... would I look like this always? Was I intellectually normal? Many many questions swirling, erupting. No one around who had been in their shoes. Loneliness can be cruel.

On top of coming to terms with me, and going through the mourning process of not having the baby they thought they were going to have.....they had to endure what the community had to say about my face and the constant staring. This extended to my brothers too who had to contend with the children's opinions of their little sister.
The good, the bad and the ugly comments.

                                                 Though always a rose in my Nana's heart.

My family's life would never the same. There would be joy, tears, laughter, physical and emotional scars and love.

I was referred to the paediatrician, Dr Grantley Stable in Brisbane. He was the one who diagnosed me. He would have looked through his thick medical textbook until he found a photo of someone that looked something like me. Then he would have read the characteristics and decided if they matched me. If they did, then that was what I had. Today genetic testing gives the diagnosis. Dr Stable decided that I had Crouzon Disease (now called a syndrome). This diagnosis came to be questioned when my first daughter was born.

Crouzon Disease/Syndrome (also known as Craniofacial Dysostosis) was originally described in 1912 by O. Crouzon in Paris in a mother and her daughter. It is caused by a mutation in Chromosome 10 n the FGFR2 (fibroblast growth factor receptor-2) gene or in the FGFR3 gene (known as acanthosis nigricans when a dark discolouration occurs with rough skin in the armpits and groin). This mutation means that the cells that form the skull and face stop growing too early causing small bones. 

I went to see Dr Stable at 3 months, 6 months and 12 months. He was an angel in Mum and Dad's world offering them  kind support. An ear to listen, who they could be honest with. Something they needed emotionally and psychologically. At each visit the usual checks of weight, height, reactions, responses, feeding etc. were done and all were normal. I met all milestones e.g. smiling, crawling and standing up in my cot.

 9 months old

As I started to grow my facial syndrome became more pronounced. I was sent to have x-rays at 12 months. Mum remembers vividly how she had to hold my head still for the x-rays but I was not co-operative. Mum got upset and Dad took over. The radiologist was unhappy that he had to re-explain to Dad what to do. Today the baby would be sedated....

The x-rays showed that my brain was pressing on the skull. An operation had to be performed quickly or mental impairment and eventually death would occur.

But there lay the problem. This was the late 1960s. No one had seen this before so who could help me? I can only imagine how scared my parents felt.

There was also divided opinion on this surgery.

Dr Stable wanted to save my life. He was determined to find a surgeon to help me.
On the other hand the Professors at the University of Queensland said it would be a waste of time so not to do it....let me die....

Reach for the hope of life...or do nothing and allow death to occur....

An easy choice for my parents.

Dr Stable was told to find the surgeon. They were going to take the risk. There really was nothing to lose. This was their baby's  life.  This was love.

Dr Stable was the angel who found the surgeon to help me.

I was referred to Dr Ken Jamieson, also in Brisbane. He was a neurosurgeon who was renown for rebuilding car accident victims' skulls. Dr Jamieson had never encountered craniosynostosis (fusion of the skull bones) before but was prepared to help me. 

Dr Jamieson is the reason I am alive today.

A month later I was in hospital. The operations were done in two stages - one half of the skull at a time.

Dr Jamieson dissected the skull into flaps of bone and implanted plastic between the sections to stop the bone from growing back together. Both operations were to be completed within three weeks but the second operation was postponed for a week as I had an infection. I was in the hospital for 6 weeks, not the three as planned.

Hospitals in the 1960s were not parent friendly like today. There was no staying overnight, strict rules, strict visiting hours, strict nurses. No counselling, no social workers.

Seeing me after my first operation was a shock to my parents. My black eyes and head bandages were too much for them to cope with. My crying when they went home still rings in my mother's heart.

As well as emotionally draining my operations were financially draining as the hospital would not reduce its fees for my parents even though I was staying twice as long as originally planned and they had medical insurance. Strict rules, no compassion.

After my bandages came off the healing began and my development continued. It was after these operations that I walked independently.


The doctors concluded that these operations were a success but that nothing more could be done for me as at that time no facial construction surgery existed.


The future was unknown.

© 2012 by Jenny Woolsey

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