Growing Up with Crouzon Syndrome

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God Sent to Me an Angel

Praise be to God

Jenny's Inspirational Sayings

Jenny's Inspirational Sayings II

The Secret of Success

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in Australia in the 1960s-80s

My Mother's Pregnancy and Birth:

I was born in 1967. My mother was a home maker and my father was a plumber. My mother was a high school teacher before having children. I have two older brothers born 2 years apart from each other. Both of my brothers are normal looking.

My mother conceived me after being off the pill for a month. Whilst pregnant with me my parents were camping and were told that there was a camper who had just left who had Rubella, so Mum went to the local hospital to have an injection. A few weeks later she had some spotting so rested and it stopped. Her obstetrician told her that it was probably due to her retroverted uterus coming forward. My mother had an uneventful pregnancy after that. I was induced two weeks after my due date and my mother recalls that the midwife said that the membrane was tough to break. Delivery was relatively normal apart from mum needing stitches.

Baby:

After I was born Mum saw me briefly and I was taken away. My father was rung at work and told to come to the hospital as soon as possible as there was a problem with me. Upon arriving at the hospital Dad was told that his baby daughter had a craniofacial deformity.

It was a deep shock to my parents. They were devastated when they saw me. Dad says that I had very prominent 'froggy' eyes, a receded upper jaw and no soft spot. I also had longer fingers and feet then the average baby. This was a big shock because in those days there were no ultrasounds, no other babies in the family like it and no reason to suspect that anything would be wrong with me.

Later on a resident of the town told Mum that many years before there had been another family where two of the children were both born with the same or similar condition. Both of these children had died.

It was even harder on my mother as she had a cold sore and was not allowed to hold me or feed me. I also had reflux and the doctor wanted to keep me in hospital until it had settled down.

Mum was isolated to a private ward with expectant mothers away from the babies and away from me. I was put in a nursery with a number of babies who were up for adoption. Mum could only look at me through the nursery glass. Mum found this hard to deal with and it added to her emotionality and feelings of loneliness and frustration. There was no one at the hospital she could talk to about her feelings. She got so frustrated with the hospital that she went home and left me in the hospital until the doctors said I could go home.

A couple days after I was born my parents received another shock as the doctors told them that they thought that I could see. It had not occurred to them that I might be blind.

After a few weeks in hospital I was allowed to go home. They dressed me in a pretty dress, a bonnet and booties and rolled me in a shawl. Mum found that when I was wrapped in a shawl with only the back of my head showing I looked like a normal baby. She found that this was a good way of disguising me in public when she didn't feel she could cope with people's stares.

This may seem wrong but the society of the times was very unaccepting of anyone who was different. Anyone who was different was usually put into homes or kept inside out of sight.

My parents fought this societal attitude. They did not listen to the advice "hide her under the bed" made by one callous lady. They decided that they wanted me to be an independent and happy member of society. It was very hard on them, especially on my mother. My mother still cannot talk about what she went through when I was a baby and growing up.

First Surgery at 13 Months:

In my first year of life my parents were sent to a pediatrician Dr Grantley Stable, who monitored my progress. He was the one who diagnosed me as having 'Crouzon Disease'. Growing up I did not cope well with the 'Disease' part of the label. I never thought I had a disease because I wasn't contagious and I didn't think anyone else could catch it from me like Scarlet Fever or Chicken Pox. I didn't particularly like the name or being associated with it. But I must note that it is now called a 'Syndrome' which sits better with me.

I met my milestones and Dr Stable saw me every 3 months. At one year I had an x-ray which showed that my skull was fully fused and that the brain was pressing on the skull. There were no craniofacial surgeons at that time so the pediatrician found a neurosurgeon who repaired road accident victims. His name was Dr Ken Jamieson. Dr Jamieson had never encountered craniosynostosis before but was prepared to help me. Dr Stable had every faith that I would develop once the craniosynostosis was dealt with but there were others in the field at the time who disagreed with him. Some of the Professors at the local university felt that the operation would not be worthwhile. Thank goodness they were wrong.

At 13 months I underwent two surgeries, one half of my 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. I was to have the surgeries 3 weeks apart but due to infection, it was extended to 6 weeks between. This surgery was expensive and was very traumatic on mum and dad as I had severe swelling of my eyes and face. My parents remember that I would cry when they would leave me which was very difficult on them as well.

The surgery was successful and my parents noticed a large improvement in my development afterwards. It was after these operations that I walked independently.

After these operations Mum and Dad were told that nothing more could be done for me.

Childhood:

Mum and Dad loved me and took me everywhere with them. Family and friends were very supportive and local people who were used to seeing us around took no notice. My parents had close friends who had a daughter with water on the brain (hydrocephalus) so they took their families out together until the little girl slipped into a coma and then died.

When I was little I went to Sunday School which I liked. I then started Brownies and went right through the Girl Guide movement because I loved it so much. I also learnt how to play tennis. I went to the local Kindergarten that my mum and nana were teachers at and then the local primary school. My mother started back teaching at the primary school when I was in Year 2. In school I always tried my best and enjoyed being with my friends. I didn't have any friends living close by but went to birthday parties and to other children's houses to play sometimes.

I was teased daily at primary school. I was often called 'goggly eyes' and 'froggy'. They really hurt but I was fortunate that I had good friends who stuck by me. I would often come home with skin missing from children pushing me over and my brothers would come home with skin off them from being in fights with the kids who teased me. Mostly boys that I remember. My brothers would also stand up for me when we went on family holidays.

My cousins would come over often and it was fun to play with them. In later years they have told me that as children they didn't notice that there was anything wrong with me. I was just Jenny.

During this time my parents realised that I had above average intelligence but they found that society thought because of the way I looked that I was mentally retarded. This was an additional prejudice they had to battle.

I had very restricted eye movements so would tilt my chin to look up or open my eyes wide to see. I had glasses to wear but didn't like wearing them. I am about 4 years old in the photos below.

Mum and Dad found that it was the people who did not know me that caused the most hurt. Some incidents that Mum recalls are:

* A woman walking past us near the Fire Station stared at us even after she went past.

* Mum took us to see the puppet show 'The Magic Pudding' when I was about 3-4 years old and the photographer refused to take our family photo.

Pioneer Craniofacial Surgery:

In the 1970s my parents were sent to an oral surgeon and a plastic surgeon to see if they could help me. At about that time the Australian Craniofacial Clinic opened in Adelaide and my parents were thinking about whether they would take me there. Then there came news in 1976 that a clinic was opening at the Mater Children's Hospital where we live. My parents were surprised and went to see Dr Tony Emmett, the plastic and reconstructive surgeon. Dr Emmett had spent two years in Paris learning reconstructive techniques from Dr Paul Tessier who had pioneered craniofacial surgery. Dr Emmett told them that I could have an operation to correct and rebuild my facial features. Over the ensuing months, I went to see various specialists and had x-rays and photos taken.

My parents were told by my eye specialist Dr Paul Spiro that I should not have the surgery as it was too great of a risk to my eyesight but my parents weighed everything up and decided to go with it hoping for the best. I remember going to see Dr Emmett and him explaining on a human skull what he was going to do to me. I also remember him telling me that he would have to do a plaster mould of my face and I would have to have straws up my nose to breathe through. I panicked over this but thank goodness didn't have to have that done. I was told that the doctors went to the local university to practice the operation beforehand.

So in 1977, when I was 9 years old, I was the second patient operated on at the Mater and the first with Crouzon's. In a 10 hour operation the surgeons advanced my top jaw 1.5 cm to approximate with my lower jaw and held it there with rib bone blocks. They also moved my eye sockets forward and held them forward with a bar of bone above and blocks of bone below. Hip and rib were used. The bone was crafted to shape and fixed in position by stainless steel wire. My jaws were wired together with toothcaps and splints and I had a tracheotomy.

I had complications from the surgery. My left eye was more swollen and prominent then my right afterwards which the surgeons thought may have been due to a misplaced block of bone in the eye socket but a CAT scan showed that this was not so. I had damage to my optic nerve in this eye which progressed and low vision resulted. Muscular stretching around that eye also was put down to having happened in the operation. With these complications my parents questioned themselves as to whether they had made the right decision.

I have vivid memories of the operation. I remember being in ICU and not being able to talk or see. I remember listening to my Dad's transistor radio and the songs of the day are ingrained in my mind, particularly 'Leo' and 'Video Killed the Radio Star'. I remember my Dad reading Magpie Island by Colin Thiele to me. I remember how painful having physio on my chest was after having ribs removed. I remember not being able to walk when I got back to the ward and had to re-learn. Back in the ward I remember eating my food processed into a soup and not being able to have the party food at my 10th birthday party in the hospital. I remember going to the hospital school which I thought was great fun. I remember having the splints taken off my teeth (which I still have) which was not a very nice experience. I remember not having any hair as it was shaved off and the nurses trying to make me wear one of their beanies which I didn't want to wear. I remember Mum bought me some beautiful scarves to wear. I then hid my scar from everyone.

Life After Surgery:

After my operation I did not want to go back to Brownies or school. My mother bought me a wig to wear and I wore it everywhere. I would not take it off until the light was off in my room. It did not look real and I was teased at school for wearing it. Here is a photo of me at school after the operation, still swollen and wearing my wig. My mother had to force me to give it up when my hair was long enough not to need it.

I looked good for about a year after the operation. After that my face began growing back to how it was. My mid-face did not grow so my lower jaw became prominent again and my eyes became bulgy (photos below were taken in 1978 one year after the operation, then in 1980 three years after). This was devastating.

Being a Teenager:

My parents were told that I would have to wait until I was 18 before any more surgery could be done as it would be a waste of time. At about 17-18 a girl's face stops growing. I went through those image-conscious, self-esteem forming years with a face which was not very well accepted. I was teased daily at high school and often found it tough going. Names like 'Frankenstein' and 'Elephant Man' still ring in my ears. I was extremely shy around strangers but an extrovert around people I knew. I was a high achiever, always trying to prove myself. I participated in many activities from school musicals to public speaking, creative writing and spelling competitions. I won many awards, my most treasured being my Queens Guide Award which was the highest award in the Girl Guide movement at that time. I also became Honoured Queen which is the girl leader in Jobs Daughters. I found comfort in the local church and held onto a faith in God to help me through those hard times. I tried hard to be liked by the boys but never had a boyfriend. I found dancing at high school horrible as I would usually be the last one to be picked which really hurt. Below is a photo taken of me in my mid-teens dressed as a clown showing the true me.

I had braces when I was 13 and then again when I was 17. I cried when I was told that I had to have them again and I knew how ugly they made me look and how painful they were. But of course I had no choice, I had to have them.

Final Operation at 18:

In 1985 when I was 18 (the professional photo above on the right, was taken just before my final operation), I had my final surgery. In this operation my upper jaw was moved forward and my lower jaw was cut and moved backward. They were then wired together for about two months. This put my jaws in line once again. More rib bone was then grafted onto the eye sockets under my eyes to increase the depth of the sockets. This was to make the eyes look less prominent.

I asked to be in the adult ward which was important to me at the time. I remember being extremely nervous before the operation and I have very vivid memories of ICU and the recovery. When I went to ICU my eyes were swollen shut and my jaws were wired together so I could not talk. I was on strong painkillers and they made me hallucinate. I kept seeing demons and could not get away from them and I could not tell anyone. It was horrific. I had severe swelling again and lost some more eyesight. The swelling lasted for months. When I got out of ICU my jaws were still wired together and were for a few months. I drank nothing but soups and high energy drinks. I ended up with a face full of acne and became quite thin. I remember having Christmas lunch all blended up, it was yummy but not the same.

When I went out with my friends I had to carry wire cutters incase I vomited or choked. Luckily I did not need to use them. I tried to keep doing what I normally did with my friends and activities. I went on an international Girl Guide camp as a first aider and took my blender with me. It was hard as the blender decided not to work but was thankfully able to be fixed.

After my splints were taken off I have another vivid memory. This one was earth-moving for me. I went into the city and was walking down the mall. And even though I was still swollen, for the first time in my life that I could remember, no one looked at me or teased me. A huge weight rose from my shoulders and I felt like dancing and screaming. I finally felt accepted by the world. I finally felt 'normal'. It had taken 18 years but I had finally reached the summit and the feeling was amazing. I will never forget it. The photo taken below was taken 10 months after the operation.

Post Script: The other associated conditions that I have with my Crouzon's are big hands and feet, long fingers and toes, gaps between my big and second toes, mild hearing loss, small nasal passages with some sleep apnea, a high-arched palate, missing teeth, a lisp, very little sight in my left eye, muscle damage around my left eye, glaucoma, haemanopia (I see out of the right hand side of my right eye) and short-sightedness.