This Is Louise's Life Story

By Dr. Mignon Moyle (Godmother)

        I first met Louise when she was about 5 years old. A tiny blonde haired waif with incredible power to disrupt! Her story was tragic - Mother dying of cancer, Father who due to his choice of beverage could not look after her, multiple half siblings ; some with jail sentences…and this dreadful disease - Cystinosis.

        She was diagnosed with Cystinosis at the age of one. The usual story which all families who have had exposure to the disease will recognize. The vomiting, thirst, failure to thrive…Her Mother tried hard but she was frequently hospitalized until the diagnosis was made.

        There was a big meeting where the consensus opinion was that although some new treatment (Cysteamine) was being trialed overseas, the prognosis was grim and her social situation was such that any attempt to prolong her life would be unkind. Treatment was for comfort only.

        Her Mother died and she spent her life in transit. Initially with one half brother, then his ex partner, then another. During this time she developed social skills that are not usually highly thought of - she was a wild child!

        Her home life finally reached an untenable position; at about the same time that her kidneys failed and she was sent to Melbourne. There was much flurry as the decision was taken that treatment of her renal failure was required. Attempts were made to place her back with her family but it became obvious that this was not viable.

        She was in hospital for a long time…The recurring theme was the vomiting…At this time she had her first renal transplant which failed. It was also during this time she was taken out during the weekends by two of the nurses - Yvonne Bates and I. On our days off we took her to the zoo, for picnics and to our homes.

         She attended the local special school -Glenallen School, which sent a bus to the hospital to pick her up. Yvonne specialized in getting Lou ready for school in the morning. She , who was never a morning person made this a far from pleasant task as there was only 1 hour for rising, eating, medication, disconnection from dialysis and hygiene. Louise would not be hurried in the mornings! Yvonne had a fine mix of patience understanding and firmness so that success (whilst never totally guaranteed) was frequent!

        She was on peritoneal dialysis and placed into foster care. This placement was a disaster for all concerned - her behaviour was such that she was returned to hospital. (It was discovered later that this was a recurring theme- any change in her place of abode was rewarded with the grossest of behaviour.). Her story was advertised in one of the state newspapers and it was from here that things improved for her.

        Her life pattern was however set in the early years; periods of relative tranquility interspersed with frequent "death attacks". Her ability to create havoc was legendary around the hospital, as was her fighting spirit. (I hasten to add that unfortunately as the behaviour improved, stories from the past were enhanced!)

        She was placed into foster care with her new family in 1988 at age 8. Karlene and Adrian Stephan; 2 loving parents and David and Kyla; 2 young adults. The family was informed to expect her life to be short - the thoughts were that 3 months would be realistic. Despite this they took on the challenge. Louise had finally landed on her feet!

        She was loved, fought for, fought with, and for the first time in her life had some permanency. In the best of Louise tradition things did not go according to plan. There was to be a long transition process between hospital and home. Louise was wildly excited - she had met and bonded with them in less than seconds.

        She could not understand why she was unable to go into her new house straight away - and fixed it in true Louise style. She developed measles and would have been transferred to the Infectious Diseases Hospital but Karlene and Adrian brought her home early. In fact, it was Thanksgiving when Louise went to the Stephans. Maybe there was some portent in the significance of the day.

        Just to encourage the family bonding; Louise was more sick and cranky than usual. Karlene informed her that she was here to stay regardless of behaviour and things slowly settled down. Her favorite support crew, apart from her family, was two hand puppet badgers - Bill and Will (short for Wilhelmina); in whom she confided initially.

        She had ongoing peritoneal dialysis and then another transplant, and then further peritoneal dialysis until finally Haemodialysis was the only option. Her family grew to include several short term foster babies (This child had an uncanny ability to get what she wanted - "More babies please Mum"). . Her family also included a zoo of domestic pets - cats, budgies, quail, rabbits guinea pigs and a pug! Then the ultimate success - two younger siblings - "ex- foster children"; Tamika and then Jacob.

        Louise was probably the only person who felt that hospital food was of a gourmet nature. She would often refuse to eat at home; preferring hospital fare. Even when the same brand of "chicken nuggets" was bought and cooked in the same oil at home it was "yuk". I hasten to add that Karlene is an excellent cook! Her last meal was cooked by the cleaner in the kitchen at the hospital (as all the regular chefs had left) who was trying to meet her requirements for chicken nuggets and gravy in a jug.

        It has been said that Louise's survival often depended upon her intrinsic (and possibly subconscious) ability to draw around her the diverse support group that she needed - and then exploit them beyond what would be considered to be the normal threshold of pain!

        Another of Lou's favorite pastimes was "cookin". She and Karlene or her dialysis sitters would pore over cookbooks for hours. Discussing recipes and deciding what would be good, at home Lou and Karlene would try out the recipes, but Louise much preferred that hospital food! She loved to organize parties and would enjoy all of the planning; often to fall asleep during the actual event! A party was never complete without lolly bags, regardless of the occasion.

        During all of this time the Stephans encouraged Yvonne, I and other people from Louise's past to stay in touch. The initial professional's plan was that Louise's past should gradually recede into the past but the Stephans felt that she should have ongoing contact with people who had had a role in her youth. They kept in contact with various brothers, sisters and of course her birth Father as well.

        Karlene and Adrian actively supported me whilst I undertook my Medical training interstate. I am well aware of the financial impost- at times of Louise's frequent crises I was receiving 3-4 calls a day to keep me updated! Louise continued to enjoy talking to her badgers, but had found a new friend; Lena the white tiger. During every hospital admission these three were unvarying part of the menagerie who accompanied her to hospital and to theatre.

        Her family did their own research and discovered the Cystinosis Foundation. They encouraged the Australian Doctors to trial treatment. They helped the Doctors keep up to date with the work being undertaken overseas. At this stage it was believed that Louise was the only Cystinotic in Australia. Her family sponsored me to attend the Paris conference to discuss her case.

        By now it was obvious that the years without treatment had taken their toll. It was obvious that other things were deteriorating. She lost the ability to read, spent more time asleep (even at night) and began walking less. Thyroid, eye, bone, heart, and immune systems were all affected. Despite this she remained a child who would not do the expected - in anything! Many times she was gravely ill and not expected to survive but she specialized in the unexpected!

        There was the understanding that options were becoming less available. She seemed to have trouble with haemodialysis lines - aneurysms, infections, and clots. This was to be the reason for her death. There was just no where else for a dialysis line to go. Without dialysis she had 10 days. Despite this, it was a standing joke amongst nothing would happen until she had managed to wring every last drop of emotion from her support crew; and wrested every last drop of skill from the medical team. Unfortunately this finally occurred.

        She died at the age of 22. A child who was advertised in the daily news had an attendance of 100 - a tribute to her ability to touch people's hearts.

        It is important to note that at this time of great world stress; Louise had prayers said in mosques, synagogues and churches of many denominations. A poignant reminder of her ability to draw diversity around her was her funeral as a Catholic, in an Anglican (Episcopalian) church conducted by a Uniting Church hospital Chaplain; and attended by people of many denominations and beliefs.

        At the funeral an important ritual was carried out - the gracious bestowing of lolly bags - from Louise to the mourners. The child that gave even without knowing it. (Or, did she???)

        For Louise, the diagnosis of cystinosis lead to her finding her family. As for the Stephans - Louise's parents who are both beautiful people….these wonderful parents are an inspiration…She introduced them to a world they never knew existed.

        Louise has taught me many things (some of which do not have a place in civilized society!) However the lesson best learned is never ever under any circumstances, to accept defeat - despite the odds do not give up.

        As Adrian has recently said many times: "Our warrior has gone."

      Dr. Mignon Moyle