The Many Faces of Moebius Syndrome!

"Gavin's Story"

n The Beginning Gavin John Fouche, was born at five to twelve on the night of the 19th September 1977. I was actually supposed to be born a month later on my brother's birthday . This was indeed an historical event; to date it has been the only time in my life that I have actually arrived early for anything. Yes, I was a premature baby, but that, I'm sure you'll learn as you read further was the least of my problems. I was born to Justin Henry Fouche - an all round good guy who would jump off his white horse and slay a dragon for anyone in need, and his wife Alvera Theresa Fouche - a woman who had seen the rough side of life but kept on smiling and bringing love and joy to others. I was to be their third and final child. My predecessors, Vanessa and Julian Fouche were at home waiting patiently. Vanessa, a typical ten year old girl was hoping for a sister. While Julian, a mischievous yet lovable seven year old was hoping for a dog. A sudden silence had fallen over the delivery room. Just a few moments before my birth the doctor and my dad were cracking jokes and speaking of the sport. The nurses wrapped the newest arrival of the human race in a blanket and briefly showed him to my mom before whisking me away. It was then that she knew that somehing was wrong. Dr. Des Stumpff, the family doctor and chief advisor to my family on all medical matters stood in the nursery of the then highly thought of Jan S. Marais hospital. A look of concern appeared on his face as he looked at the new arrival. Out of all the children he had ever delivered he knew this one was different. The baby's features were indistinct as if he had been taken from his mother's womb while he was still forming. He noticed that the tiny fingers of each hand were different to his own. The right hand only consisted of four fingers of which the last two were joined and at first glance looked like one finger. The fingers on the left hand were all there - all five - but the fingers looked weak, shrivelled and unable to move. He had never seen anything like this in his entire medical career and it puzzled both him and his assisting colleagues. Doctors, Scientists and Journalists alike love labelling things and people. I suppose it brings a sense of order to the world around them. But in my case it would not be so easy to fit me into a category. No-one knew what to classify me as or what the cause of this deformity was. It was suspected that this was a case of severe mental retardation and that I would be a vegetable. After checking me for any hidden clues which might lead to a diagnosis I was placed into my mother's arms for the briefest of moments and then led away to the nearest incubator. Dr. Stumpff meanwhile, explained to my parents that he thought that I was not really considered to be premature but rather dismature. This meant that parts of my body such as my face were still in the process of forming even after I had been taken from my mother's womb. It dawned on my parents that I was definitely the family "ugly duckling" and the chances were good that I possibly would not make it to become a swan. The next day a Paediatrician, a Dr. Schraader was informed of my case and came to take some X - rays. It was discovered that muscles were missing in various parts of my body but mainly in my upper arms. The chances of me doing anything except breathing became very minimal. Dr. Schraader informed my parents that the hospital did not have the necessary facilities to care for me and that he had considered two courses of action my parents could take. The first was to transfer me to Tygerberg hospital where I could be given the proper care that I needed. The second (a more sinister option in my opinion) was to - in the light of all my defects and deformities - have me commited straight-away to Alexandria, an institution The 'good doctor' even assured my parents that he could have all the necessary documents drawn up so that I could be transferred that very day. (It sounds like this guy sold encyclopaedias before he entered the medical profession, doesn't it?) I can honestly say that my parents never thought twice (although they might have regretted it in times of trouble) about having me transferred to Tygerberg where hope still lay. The first step had been taken in the direction of giving me a better chance, but a long and difficult road still lay ahead.. ******************************************************************* Of Moebius And Medicine Looking back on my babyhood I think I was probably the only child in South Africa to have a Geneticist, a Paediatrician, a Radiologist, two Orthopaedic surgeons, (One for ach foot??) an eye surgeon, a Plastic surgeon (eat your heart out Michael Jackson) a Speech Therapist, a Physio Therapist and an Occupational Therapist all looking out for me. The day after my birth I was transferred to Tygerberg Hospital, where a number of medical specialists were called in to study my case. I was by now a whole thirty-two centimetres long and weighed two-thousand-four-hundred and ninety grams. I was tube fed because I was unable to suck, and by now it had been established that my face was paralysed. Doctors speculated about my condition and thought I had Golden Hare Syndrome (which is something similar to what I have) but the notion was dismissed because I had no heart defects, which is an important characteristic of the syndrome. On the home-front my father was coming to see me just about everyday but my mother had a lot of misconceptions about me. She had never actually looked at me before and believed -from all the things doctors had told her- that my face was a gaping black hole and that I had no limbs whatsoever! Visits to me from my godmother encouraged her to take my sister and my brother to see me. On arriving she was greeted by a nurse with a little me in her arms. One of my feet were sticking out from underneath the blanket and she saw that I was actually not that bad. She came to see me regularly after that and fed me and bathed me and did all the other things mothers usually do with babies. I was released from Tygerberg on the 9th of October 1977, weighing two-thousand-seven-hundred and thirty grams. I still had to come back for further testing, (these people even tested my belly-button!) But for the moment I was allowed to go home to 51 High Trees, Eversdal, Durbanville. A few months later while I was undergoing treatment at Tygerberg, Professor Byton, one of my limitless cohorts of physicians, asked my mother if she would mind if they used my particular case in an upcoming conference where a group of doctors - some from Britain and some from the United States - would discuss birth defects. My mother agreed and was allowed to attend this gathering with me. (Of course I had no idea what was going on at the time) To start off with slides were shown of babies - one was a pair of Siamese twins - and the others also had some missing body parts which put them in the running to star in this little medical freak-show. At one point a slide was shown of me (which come to think of it probably made me look even worse. I am not a very photogenic person) and all the doctors sort of went into a huddle and discussed me. Some doctors agreed that I showed all the sign of having Foetal Alcohol Syndrome. It was also suggested that passed through my mother's system and entered mine. My mother didn't and still doesn't drink, so the first suggestion was ruled out. One of the doctors said, that with all the defects that I had the body should have aborted me before my birth, and the reason it didn't was because I could have been lying in a different position to what I should have been. (In which rubbish bin did that guy find his degree?) Long live my mother's uterus! The lecturer concluded that in medical terms they would refer to it as a 'living abortion' (Not exactly the nicest thing I've ever been called!) As the months went by I grew stronger. When I arrived home for the first time, my mother had to exercise my arms because they were stiff and stuck behind my back. I had regular appointments with Physios and Speech therapists, where I was submitted to all types of strange treatment. Rolling on a gigantic red Physio ball is one of these strange 'tortures' I can vividly recall. I remember my Physio, Margaret Wyndham, holding on to my ankles and rolling the ball with me helplessly stuck to it like a dead ant to a soccer ball. It made me really seasick! (Which probably explains why I don't like all those hair-raising rides at funfairs) Among these ghastly daily rituals were countless arm and leg exercises. Thanks to all this exercise from my Physios and my mom I could eventually lift my head, move my arms and sail on my bottom. (I couldn't walk until I was three) Operations were being planned to increase the use of my fingers and Orthopaedic boots (doots - as I then called them) were being made because I was rather club-footed. I also could speak and could say my first constructive sentences (Well, almost) at the tender age of three which included such useful verbal communications as: 'No Nessa!' (I would be saying that very sentence to my sister for the next eighteen years!) My first operation took place when I was just six weeks old. I had a twisted bowel hernia and it had to be removed. An eye operation was soon to follow. I was a bit squint. With all these operations and the ones that lay in the future, no wonder I had a fear of men in white coats! My mother first consulted Dr. Deon Burger, an eye surgeon about a previously performed eye op when I was 5 years old. He had studied in the States and was familiar with me before we even met. (Remember: I was to doctors what Brad Pitt is to teenagers) During our visit my mother explained the nature of my birth and the mystery of me. He out-rightly surprised her when he said that he had heard about someone like me before. He took a medical book from a shelf and opened it to a page which stated what I was, the very same riddle which, both South African doctors and many of their overseas counterparts had considered insolvable. The answer hit us like a ton of bricks: Moebius syndrome. He gave my mother a copy of the text regarding the syndrome. According to the book, previous studies into Moebius syndrome patients had led to discoveries of deformities of the tongue, chest, neck, ears, eyelids unable to close, webbed digits and, in some cases, mental retardation. I am lucky to possess only two of these characteristics. (lack of facial muscles and 'webbed digits') Finally everyone knew what I was, even if the exact cause of the disorder was, and still is, unknown. A few months later I underwent my first hand operation. The operation was performed by Dr. Pretorious and his team. These operations were designed to give me better use of my hands and fingers. After the first few I was able to open and close my left hand and later could grasp objects. My first finger on the hand actually adopted the likeness and functions of a thumb These operations later became so routine that I was actually more scared of a dentist holding a cotton-swob than a doctor wielding a scalpel! My final operation occurred when I was seven, my second finger on my right hand was removed and my hand was deepened so that I could grip as well with the right as I could with the left. Along with all these ominous operations came strange things that I had to wear and do, such as splints (they were mainly designed to space my fingers apart but often dug into the webbing between my fingers. It hurt like mad!) And really weird looking pairs of shoes for my feet. One was a pair of shoes that looked like boots crossed-between a pair of high-heels, except that the heel was in the middle. I wore that after a leg operation where both my feet were in Plaster-paris. From my birth I suppose you can say my life was literally riddled with operations and other minor procedures, that I later considered myself to be somewhat of an expert in being a patient. Amateur Aneathatists used to think they could coax me into wearing the gas mask by saying things such as: 'This is just like fighter pilots wear!' or 'You're going to have a nice dream and wake up feeling very fresh.' (Usually I would wake up and ask if anybody had taken down the registration number of the double-decker London red bus that had hit me - because that what I felt like) They were right about the dream part though, I now have a vague idea of what John Lennon and Jimi Hendrix felt like when they were tripping on life-threatening drugs like LSD. As the years wore on I'd often play a bit of a game with the Anethitists - When they applied the mask to my face I wold hold my breath for as long as I could. Now, unbeknown to me at that stage, I have very narrow airways... I could never quite work out why all the doctors in the operating theatre went into flat panic after I "stopped breathing." But it certainly was a lot of fun to see their reaction....! For a boy whose destiny was foretold by physicians to be retarded and a vegetable - meaning that I would not be able to ever walk, talk or even swallow, I was doing pretty darn well! With the help of Physio's, doctors, and the rest of my little 'entourage' who were all bent on seeing me succeed, I had broken a barrier, and proved that the expected had become the unexpected. A lesson which I continue to teach people - I think anyway.

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