Growing Up Looking Different in Australia in the 1960s-80s
Jenny's Mother's Pregnancy and Birth:
Jenny was born in the 1960s. Her mother was a home maker and her
father, a plumber. Jenny's mother had previously taught Commercial subjects at the local high school teacher before having
children. Jenny had two older brothers, both of
whom were not born with her syndrome.
Jenny's mother conceived her after being off the pill for a month. Whilst pregnant with Jenny her parents were camping and were told there was a camper who had just left who had Rubella, so Jenny's mother 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. Jenny's mother had an uneventful pregnancy after that and Jenny was induced two weeks after the due date. Her mother recalls that the midwife said that the membrane was tough to break. Delivery was relatively normal apart from Jenny's mother needing stitches.
After Jenny was born her mother saw her briefly and she was taken away. Her father was rung at work and told to come to the hospital as soon as possible as there was a problem with Jenny. Upon arriving at the hospital her father was told that his baby daughter had a 'craniofacial deformity'.
It was a deep shock to Jenny's parents. They were devastated when they saw her. Jenny's father described her as having very prominent 'froggy' eyes, a receded upper jaw and no soft spot. She 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 her.
Later on a resident of the town told Jenny's mother 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 Jenny's mother as she had developed a cold sore and was not allowed to hold Jenny or feed her. Jenny also had reflux and the doctor wanted to keep her in hospital until it had settled down.
Jenny's mother was isolated to a private ward with expectant mothers away from the babies and away from Jenny. Jenny was put in a nursery with a number of babies who were up for adoption. Jenny's mother could only look at her through the nursery glass. Her mother 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 Jenny in the hospital until the doctors said Jenny could go home.
A couple days after Jenny was born her parents received another shock as the doctors told them that they thought that she could see. It had not occurred to them that their baby might be blind.
After a few weeks in hospital Jenny was allowed to go home. They dressed her in a pretty dress, a bonnet and booties and rolled her in a shawl. Jenny's mother found that when Jenny was wrapped in a shawl with only the back of her head showing she looked like a normal baby. She found that this was a good way of disguising Jenny 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 institutions or kept inside out of sight.
Jenny's 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 Jenny to be an independent and happy member of society. It was very hard on them, especially on Jenny's mother (Jenny says her mother still cannot talk about what she went through when Jenny was a baby and growing up.)
First Surgery at 13 Months:
In Jenny's first year of life her parents were sent to a pediatrician Dr Grantley Stable, who monitored Jenny's progress. He was the one who diagnosed her as having 'Crouzon Disease'. Growing up Jenny did not cope well with the 'Disease' part of the label. She never thought she had a disease because she wasn't contagious and she didn't think anyone else could catch it from her like Scarlet Fever or Chicken Pox. Jenny didn't particularly like the name or being associated with it. (It is now called a 'Syndrome' which sits better with her.)
Jenny met her milestones and Dr Stable saw her every 3
months. At one year he did an x-ray which showed that Jenny's skull was fully fused
and that the brain was pressing on the skull. Jenny's parents had noticed a decline in her milestones and she was becoming quite agitated. 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 encountered craniosynostosis before and was happy to help Jenny. Dr Stable had
every faith that Jenny 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
At 13 months Jenny underwent two surgeries, one half of her 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. Jenny 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 Jenny's parents, as she had severe swelling of her eyes and face. Jenny's parents remember that she would cry when they would leave her which was very difficult on them as well.
The surgery was successful and her parents noticed a large improvement in Jenny's development afterwards. It was after these operations that she walked independently.
Jenny's parents were then given the news that nothing more could be done for me.
Jenny's parents loved her and took her everywhere with them. Family and friends were very supportive and local people who were used to seeing her around took no notice. Her 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 Jenny was little she went to Sunday School which she liked. She then started Brownies and went right through the Girl Guide movement because she loved it so much. Jenny also learnt how to play tennis. She attended the local Kindergarten that her mother and nana were teachers at and then the local primary school. Jenny's mother started back teaching at the primary school when Jenny was in Year 2. In school Jenny always tried her best and enjoyed being with her friends. She played with the neighbourhood kids, went to birthday parties and to other children's houses to play.
At primary school, Jenny was teased daily. She was often called 'goggly eyes' and 'froggy'. They really hurt but she was fortunate that she had good friends who stuck by her. Jenny would often come home with skin missing from children pushing her over and her brothers would come home with skin off them from being in fights with the kids who teased their sister. Mostly boys that Jenny remembers. Her brothers would also stand up for her when they went on family holidays.
Jenny's cousins would come over often and it was fun to play with them. In later years they have told Jenny that as children they didn't notice that there was anything wrong with her. She was just Jenny.
During this time Jenny's parents realised that she had above average intelligence but they found that society thought because of the way she looked that she was intellectually impaired. This was an additional prejudice they had to battle.
Jenny had very restricted eye movements so would tilt her chin to look up or she would open her eyes wide to see. She had glasses to wear but didn't like wearing them. Jenny is about 4 years old in the photos below.
Jenny's parents found that it was the people who did not know her that caused the most hurt. Some incidents that her mother recalls are:
* A woman walking past them near the Fire Station stared at them even after she went past.
* Her taking the three children to see the puppet show 'The Magic Pudding' when Jenny was about 3-4 years old and the photographer refused to take their family photo.
Pioneer Craniofacial Surgery:
Jenny's parents were told by her eye specialist Dr Paul Spiro that she should not have the surgery as it was too great of a risk to her eyesight but Jenny's parents weighed everything up and decided to go with the surgery hoping for the best. Jenny remembers going to see Dr Emmett and him explaining on a real human skull what he was going to do to her. She also remembers him telling her that he would have to do a plaster mould of her skull and face and she would have straws up her nose to breathe through. She panicked over this but it didn't eventuate. Jenny was told that the doctors went to the local university to practice the operation beforehand.
So in 1977, when she was 9 years old, Jenny was the second patient operated on at the Mater and the first with Crouzons. In a 10 hour operation the surgeons advanced her top jaw 1.5 cm to approximate with her lower jaw and held it there with rib bone blocks. They also moved her 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. Her jaws were wired together with toothcaps and splints, and she had a tracheotomy to breathe.
There were complications from the surgery. Jenny's left eye was more swollen and prominent then her right afterwards which the surgeons thought may have been due to a misplaced block of bone in the eye socket but a CT scan showed that this was not so. Jenny was taken back into theatre for further examination. As well as the physical swelling, the optic nerve had become damaged from pressure and swelling, resulting in permanent very low vision in that eye. Muscular stretching around that eye also was put down to having happened in the operation. With these complications Jenny's parents questioned themselves as to whether they had made the right decision.
Jenny has vivid memories of the operation. She remembers being in ICU and not being able to talk or see. She remembesr listening to her dad's transistor radio and the songs of the day are ingrained in her mind, particularly 'Leo' and 'Video Killed the Radio Star'. She remembers her dad reading Magpie Island by Colin Thiele to her. She remembers how painful having physio on her chest was after having ribs removed. She remembers not being able to walk when she got back to the ward and had to re-learn. Back in the ward she also remembers eating food processed into a soup and not being able to have the party food at her 10th ward birthday party in the hospital. She remembers going to the hospital school which she thought was great fun. She remembers having the splints taken off her teeth under light sedation - it was a horrible experience. She remembers not having any hair as it was shaved off and the nurses trying to make her wear one of their beanies which she didn't want to wear. She remembers her mother buying some beautiful scarves to wear. She then hid her ear--to-ear scar from everyone.
Life After Surgery:
After Jenny's operation she did not want to go back to Brownies or school. Her mother bought her a wig to wear and she wore it everywhere. She would not take it off until the light was off in her room. It did not look real and Jenny was teased at school for wearing it. Below is a photo of Jenny at school after the operation, still swollen and wearing her wig. Jenny's mother had to force her to give it up over the Christmas holidays when her hair was long enough not to need it.
Jenny looked good, 'normal' you could say, for about a year after the operation. After that her face began growing back to how it was. Her mid-face did not grow so her lower jaw became prominent again and her eyes became bulgy (photos below were taken in 1978 one year after the operation, then in 1980 three years after). This was devastating to Jenny - she was back to being different again.
Being a Teenager:
Jenny's parents were told that she would have to wait until Jenny
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. Jenny went through those image-conscious,
self-esteem forming years with a face which was not very well accepted. Just like primary school, in high school she was
teased daily and often found it tough going. Names like
'Frankenstein' and 'Elephant Man' still ring in her ears. Jenny was extremely shy
around strangers but an extrovert around people she knew. She was a high achiever,
always trying to prove herself. Jenny participated in many activities from school
musicals to public speaking, creative writing and spelling competitions. She won
many awards, her most treasured being the Queens Guide Award which was the highest
award in the Girl Guide movement at that time. Jenny also became Honoured Queen
which is the girl leader in Jobs Daughters. She found comfort in the local church
and held onto a faith in God to help her through those hard times. She tried hard
to be liked by the boys but never had a boyfriend. She found dancing at high
school horrible as she would usually be the last one to be picked which really
hurt. Below is a photo taken of Jenny in my mid-teens dressed as a clown showing
the true her. (Jenny collected clowns and loved that they had a white face which disguised who they really were. She also loved acting and drama as she could be another person just for a little while)
Jenny had braces on her teeth when she was 13 and then again when I was
17. She cried when she was told that she would be having them again. One reason was the pain, but another was she thought they made her look uglier. But even though she tried to resist she had no choice.
Final Operation at 18:
In 1985 when Jenny was 18 (the professional photo above on the right, was taken just before her final operation), she underwent her final surgery. In this operation Jenny's upper jaw was moved forward and her lower jaw was cut and moved backward. They were then wired together for about two months. This put Jenny's jaws in line once again. More rib bone was then grafted onto the eye sockets under her eyes to increase the depth of the sockets. This was to make the eyes look less prominent.
Jenny asked to be in the adult ward which was important to her at the time. She remembers being extremely nervous before the operation and after she had her sedation, the lady beside her consistently asking her about what she was having done. All she wanted to do was go to sleep and wake up on the other side. Jenny also has very vivid memories of ICU and the recovery. When she was in ICU her eyes were swollen shut and her jaws were wired together so she could not talk or see. She was on strong painkillers which made her hallucinate. Jenny kept seeing demons and could not get away from them. She could not open her eyes or tell anyone - it was like hell. Jenny experienced severe swelling again and lost some more eyesight, due to optic nerve damage. The swelling lasted for months. When Jenny left ICU her jaws were still wired together and were for a few months. She drank nothing but soups and high energy drinks. She ended up with a face full of acne and became quite thin. Jenny remembers having Christmas lunch all blended up, it was yummy but not the same.
When Jenny went out with her friends she had to carry wire cutters in case she vomited or choked. Luckily she did not need to use them. She tried to keep doing what she normally did with her friends and activities. She even went on an international Girl Guide camp as a first aider and took her blender with her. It was hard as the blender decided not to work but was thankfully able to be fixed.
After Jenny's teeth splints were taken off she recalls another vivid memory. This one was earth-moving for her. She went into the city and was walking down the Queen Street mall, something she had done many times before. And even though she was still swollen, for the first time in her life that she could remember, no one looked at her or made comments about her face. A huge weight rose from Jenny's shoulders and she felt like dancing and screaming. Jenny finally felt accepted by the world. She finally felt 'normal'. It had taken 18 years but she had finally reached the summit and the feeling was amazing. Jenny says she will never forget it. The photo taken below was taken 10 months after the operation.