Diagnosis

Well, what did I have?  I was certainly very jaundiced, and had ascites.  I couldn't see my toes, because of all the fluid I was carrying!  Surely it must have been Hepatitis, Leukaemia... the tests went on....and on....and on.... until the Doctors didn't know what they could test next.  I was lucky that the hospital had a Mother's room, so that my Mother and baby sister were often able to come and stay in the hospital.  My Grandparents also drove all the way down from Auckland to visit, as it was quite lonely and scary being away from home and school.  The nurses and doctors were lovely, but I started developing an antipathy to needles at this stage.  One morning I was promised that there would be no need to take blood that morning, so I wheelchaired myself down to visit the lady in the hospital shop, only to have the Ward page me, as they did need my blood after all.  The bone marrow puncture was a memory that will live long, as there was no local anaesthetic or any pain relief and the agony was so intense they had to postpone it until my later operation.  On the whole though I was told that I was a happy and cheerful patient, and I certainly had many cards and visitors.  I was rapidly dying so emergency measures were called for.  My lovely Pediatrician, Dr. Leo Buchanen, did an exploratory laparotomy, and surprise, discovered a totally cirrhosed liver.  He had earlier tested for Wilson's Disease with the serum copper and serum zinc tests of the blood and urine, and was waiting for the results to be sent down from Auckland Hospital.  He had come across Wilson's Disease before, when he had worked in the United Kingdom, and included these tests as part of the total package of tests he was doing.  The results for these tests came back at about the same time as he did the laparotomy, and they were positive for Wilson's Disease.  He then immediately arranged for a slit-lamp examination of my eyes, and saw most impressive Kayser-Fleischer rings.  This was 1974.  Liver Transplants were not available, and the drug of choice was Penicillamine.  One of the most exciting memories is of finally being able to see my toes, once they had got rid of my ascites.  I remember ringing my Mother in great glee from the hall telephone, saying this.  I was only about 3 stone now, the same weight as my sister who was five years younger, and 10 inches shorter.

Bethli and Teddy, Ward 2, Taranaki Base Hospital, November 1974.

Treatment

I was started very very slowly on Penicillamine.  A 12 year old patient in Australia had also been recently diagnosed, after a much longer period in hospital, and was started quickly on Penicillamine, couldn't tolerate the dosage and died.  I was fortunate that they knew this, and learned to start me on a very gradual and slowly increasing dosage.  I have always felt that if it wasn't for that 12 year old, I wouldn't have made it either, and I have always felt that I owed that family part of my life.

It took a long time to get better.  Major milestones included being allowed out of hospital for the first time, and then eventually I was allowed home.  At the end of that year my Father was shifted to Lower Hutt.  I was put under the care of Dr. Anyon.  He was a lovely doctor, who had never heard of Wilson's Disease, and my Mother had to bring him up to speed very quickly on my condition.

I did not have a very active year in 1975.  I remember spending a lot of time in bed or at home, and not getting to class very often.  I was in Standard Two, and I know that most of the year I spent reading.  I continued with my piano lessons.  One of the times that I got to school was unfortunate, as the class was moving from one classroom down to another and we all had to carry our own desks.  I was made to as well, even though I was still recovering.  I fell badly and got a bump the size of a goose egg on my forehead, and beautiful bruised big black eyes that lasted for over 9 months.  One highlight of the year was a day trip to Kapiti Island, and another was a holiday at Punga Cove in the Marlborough Sounds.

In 1976 we shifted to Gisborne, where we stayed for two years.  I was feeling very well by this stage.  I won the Standard Three Speech Contest at Te Hapara Primary, and my Mother, Kylie and I went pony riding at a place called Jingle Bells nearly every Sunday.  My pony Bluebell had a mind of her own, especially when she had foaled, and I recall her taking off down the road at a gallop to the paddock where her foal was, with me hanging on tightly, with no control at all against her Mother's instinct!  This was also the year that I got glasses for shortsightedness for the first time.  My friend Robyn had a Raleigh 20 bike, and I had an older bike, and we zoomed around our street, and up and down the open ditches all the time.  Summer days in their family's Para pool were also a lot of fun.  Our cousins lived in Gisborne too, and we spent time with them, and their pet lamb Tobomory (the Wombles were very popular then) and our pet lamb Friskie.  Fluffy and Friskie didn't get on that well, and Friskie also had an eye on my garden, eating all the plants I particularly wanted to grow and leaving the beetroot (that I didn't like) alone!

In 1977 Her Majesty The Queen visited, and I (along with thousands of other school children) lined Childers Park to see her.  I was most fortunate in that she stopped and chatted for half a minute.  My Doctor in Gisborne was Dr. Frankish.  I still had to have very regular blood tests, from Darcy at the hospital, who drew a cat on my hand every time, and Dr. Frankish also did a liver biopsy under General Anaesthetic to see how the liver was going.  I still had some lung consolidation, and had regular sessions with the Physio who vigorously pummeled my back while I was on a slant table, and I had to cough and cough.  We practiced this at home on an ironing board too!

In 1978 we moved to Melbourne, Australia.  While there I was under the care of Dr. Arnold Smith and Prof. David Danks.  I was treated at the Royal Children's Hospital (Flemington, Melbourne), where they were doing research into Wilson's Disease, and requested some tissue and blood in order to grow cultures.  I was also able to see a slide of my liver biopsy from Gisborne.  My sister was rigorously tested to see if she also had Wilson's Disease, and fortunately she is clear (although we don't know if she is a carrier).

We were fortunate that our parents believed that travel was educational.  We had an old station wagon, and managed to see much of Australia.  Three of us slept in the back and Kylie slept across the front seat and under the steering wheel.  We went as far as the Flinders Ranges and Port Augusta in South Australia, Hobart in Tasmania and Dysart and Rockhampton in Queensland, as well as many places in between.  Stopping for days or a week at a time sometimes.  Towards the end of 1979 we returned to Auckland, New Zealand.

I became an out-patient at Auckland Hospital under the care of Dr. Alistair Scott.  He moved to North Shore Hospital in Auckland, and then subsequently established a private practice on the North Shore.  I was seeing him until he retired in October 1999.

During this period of time I was heavily reliant on my Mother to monitor Wilson's Disease and keep a track of what was happening.  As I was diagnosed at age 8, I grew up looking to her for answers and expecting her to be the one to talk to the Doctors.  I took my Penicillamine tablets, and did all the required tests, but I didn't talk about Wilson's Disease, or like to tell others about it.  I had found it uncomfortable when I was diagnosed to suddenly become a "disease" to many people.  

Because of the public nature of my Father's job, there was a large population base in New Zealand that had heard of me.  When I was with my Mother and she was talking to people I didn't know, it was disconcerting to always be "the little sick girl", and not an individual in my own right.  For this reason I tried to ignore it as much as possible.  I didn't want it to limit me, and what I hoped to achieve in my life.  I already had overheard Dr. Buchanen say that I shouldn't have children, when I was still in hospital after being diagnosed, and I had made up my mind to make my life one that I decided to live, and not one that was dictated to me by Wilson's Disease.  

I started looking for a successful career path from an early age.  Initially my ambition was to be a Nurse, then I decided on becoming a Heart Surgeon (fortunately I gave that idea away, as I have always had a slight hand tremor, and I don't think that would be a good combination with a scalpel!).  By the age of 14 I knew I wanted to go to Auckland University and do a Bachelor of Arts, and then go to Victoria University of Wellington and do the post-graduate Diploma of Librarianship and become a professional Library and Information Manager.  

I had also started to learn the German language while at school in Australia.  I was very keen to save money and travel to Europe.  I started a job in October 1982 as a part-time cleaner at my high school.  I continued this until I completed 7th Form and my Bursary and Scholarship exams, working through the summer until University started.  In March 1985 I started University and also worked part-time in the General Library at the University.  

I was put onto Zinc instead of Penicillamine for this year.  I had awful cramping pains with the Zinc, which would last 3-4 hours, if I didn't get the timing exactly right.  I seemed to need to eat at exactly 18 minutes after taking Zinc in order to avoid the agonising cramps.  These caused me to roll on the floor and be utterly useless until they wore off.  At the end of that year Dr. Alistair Scott did a liver biopsy and bone marrow test, to see how the Zinc had worked.  Unfortunately my liver was not de-coppered, and he put me straight back on the Penicillamine.

In 1986 I had a heavy study load doing 8 papers.  I was also suffering from a very enlarged spleen, and a gastroscopy showed that I had huge oesophegal varices, which were in danger of rupturing.  My spleen was sticking out like I was 9 months pregnant, and I couldn't eat much anymore.  Little snacks were all I had room for.  I had no energy, and was very very pale.  I was still working part-time, and with my study I had no energy or desire to do anything except survive the year.  I was asked to have my spleen removed during my exam times, but after talking to the University Registrar, I was told that if I was unable to sit an exam while in theater I would get a compassionate pass, but not in any other circumstances.  I asked the Doctors if it could be delayed until after I had sat my 8 papers, and they agreed (but not happily) and we all just crossed our fingers that I would last till then.

Splenectomy

I had my spleen removed in the first week of January 1987.  It measured 15 inches by 9 inches by 7 inches, and the cut went from the lower right of my abdomen to the upper left around under my left arm on my back.  The surgeon was Prof. James Shaw, and he did the same operation on my faithful Teddy.  He also shunted the splenic vein to the left renal vein.  [The fact that he didn't cut it short, as was the usual practice, meant that the New Zealand Liver Transplant Unit was very grateful 13 years later, that they were able to attach the donor liver portal vein to it].  Until Prof. Shaw operated in 1987, they were not sure what solution regarding the varices would be possible.  A shunt was what they preferred, but the other option was having to return to the hospital every three months to get the varices nipped.  I was delighted to find out, when in Intensive Care, that I had been shunted successfully!

I got home a lot lighter than when I went into hospital.  Once again, I was excited to be able to see my toes.  This time though, I found it very difficult to get out of bed.  As my side muscles and front muscles had been cut, moving was difficult for a long long time.  At the end of February, willpower and the strength of my friends Eileen, Emma and Kathy got me through enrolment at the University.  They literally carried me up the stairs to the Student Union building so I could enrol.  I was doing six stage III papers this year, and I did find it hard to do this, work for 15 hours a week in the library and recuperate from the surgery, but I made it.  Hardly anyone at University (whether work or lecturers) knew that I had had this operation.

Maintenance Programme