Huntington’s Disease – Marjorie Thomas

Welcome along, it’s lovely to see you all – such a lot of good faces here tonight, and it’s lovely. I’ve been press ganged into welcoming our speaker tonight and it gives me great pleasure to introduce to you Marjorie Thomas. She’s going to talk to you about living a life with, and about, Huntington’s Disease. So she’s a local lady, having been here, gone away, come back as a lot of us do, and I think her story will interest you all, so welcome, and we look forward to hearing your story.

[Applause]

Thank you. By 1907 all of my grandparents were in New Zealand. Both my grandmothers were born here, one in Lawrence where her father was a gold miner; the other in Wairoa where her father had been given land in return for serving in the army. And just as aside, he was the only European ever wounded in Hawke’s Bay in fighting the Hauhau. He got shot in the knee at Petane, and after six months they sent him to Sydney for treatment.

One grandfather came here with his family in 1878 and they settled in the Maheno area south of Oamaru, where the family had an agricultural contracting business, and they finally ended up owning the local flour mill. It’s now a national Historic Places Trust [property]. The other grandfather came here to escape his large family in New South Wales, and that was around 1907. His grandfather had got free passage to Australia as a convict. He was found guilty of burglariously entering licensed premises after hours, [chuckles] and that was a life sentence. So, which of them brought the HD [Huntington’s Disease] gene with them?

My father, Ray Thomas, was the oldest of five. When he left school he did a range of jobs – farming, road building, and in 1931 after attending a herd testing course run at Massey Agricultural College, he got a job as a herd tester in Taranaki. A friend persuaded him, some years later, that he was getting a bit boring and he should go back and do something different. So they travelled to Invercargill where they got work on the Milford Sound road programme. They were responsible for putting in the road from the Homer Tunnel down to the Sound; he was there for about three years in total. Photography was a hobby of his; I’ve got a most amusing photo album at home. He specialised in taking photographs of the vehicles he owned, never mind the background, so it’s, you know, ‘Chevy’, or whatever it was.

My mother on the other hand was the youngest of three; she had two older brothers. When she was fifteen her father was killed – a car ran over him as he was [coughing] biking home from work. She left school soon after, and got a job as a shorthand typist with New Zealand Railways, based at the Wellington Railway Station. She took the opportunity to travel with her girlfriends when possible. Her photo album shows they travelled to many tourist spots in New Zealand, from Rotorua right down to eventually, Milford Sound. That was a weekend trip. They met there, and I believe it was because of their mutual interest in photography. So here is a photo that I worked out from the photo albums was taken by Mum on that weekend trip. It’s one of the first colour photos; colour photography came about in the early 1930s, and this would’ve been taken about 1939, and it’s really a very clever photo … when I first saw it I always thought it was black and white. It’s not. So, that is where they met, and clicked.

World War Two broke out. Dad became unwell and he was required to leave in 1940. On his way back to Wairoa he stopped in Lower Hutt to meet Mary. In 1941 they were married. They moved to Rawene in North Auckland, where Dad had found a herd testing job. Life there went well. Isobel, my eldest sister was born in 1942. Dad found that Mum, although an active person used to outdoor sports, was unaware of cooking and housekeeping duties. [Chuckles] He helped her learn. The local rural community was very supportive, and he had nothing but praise for the health scheme running in the Hokianga at the time; it was a bit of a trend setter.

Then in 1943 he was conscripted and assigned to help safeguard the North Auckland beaches from possible invaders … that is, putting barbed wire on them. Mum returned to her mother in Lower Hutt, and she was pregnant. Anne was born there. Dad was due to go overseas but luckily, or unluckily, Dad was ill again … what is now recognised as H-bug. [Hospital-bug, or staphylococcal infection] He was put on medical leave; they didn’t know what to do with him. They didn’t have antibiotics – they just literally didn’t know. He had the most horrible scars; they used to open him up and scrap the muck off the rib cage, and then close him up again. He used to enjoy making our stomachs churn with that story. [Chuckles] He also said waking up after the operation was really horrible because all he wanted to do was vomit; so he tried all ways of avoiding the vomiting. He said none of them worked. So, there he was. Finally the army thought, ‘Nah, it’s not worth keeping this guy on’, so they discharged him on medical grounds, and that was in 1944. What to do? Wife, two kids, no job. He took the train to Wairoa to talk with his mother about taking on the family dairy farm. His youngest brother had got in first – his three younger brothers were all serving overseas. [Children’s voices in background]

On the return trip to Wellington the train stopped for refreshments in Hastings; I didn’t know they did that then, I always thought it was Napier. He saw a real estate firm across the road, got up, went in and within ten minutes had organised the mortgage for a house at £800, put in my mother’s name because women live longer than men, [chuckles] and went off down to Wellington. As an aside, I can’t see that happening today, can you? [Chuckles] We don’t have passenger trains any more. He got back to Lower Hutt and told Mum they were going to live in Hastings. “Huh?” That wasn’t a tourist spot she’d been to. “Pack up, we’ll go up by train.” On arrival the real estate agent was waiting at the station and drove them to their new home. I still live there.

What to do? Find work. Mr Mardon of Pernel Orchard gave Dad a job. And later on when one of his brothers returned from overseas, he and Dad joined a local rehab [rehabilitation] carpentry scheme. They built a lot of the State houses in Hastings and Napier.

Meanwhile I was born in early ‘45 for those of you wondering – I am seventy-six. Mum had post-natal depression; three babies in under four years. Gran Clark was a great help. They joined a Friendly Society … a Lodge. They made friends, socialised, visiting friends to play cards – euchre, canasta, samba. In summer there were Sunday trips to the beaches. New Zealand Railway Buses would be lined up at the bus station in Karamu Road North … remember it? And the destination – so you chose: Haumoana, Te Awanga, Waimārama – that was our favourite. We’d be there for the day and come home pinker than the Qtol [lotion] that covered us. [Chuckles] Those days there weren’t many trees out at Waimārama. Peeling started on Thursday, and on Sunday we repeated the process again. [Chuckles] We were all blondes … fair haired; oh, maybe Alison had bright red hair – she really burned. So that was what happened during the summer.

Mum joined the Kindergarten Mother’s Club and she would go off to the meetings on her bike at night. I remember going to the free kindergarten in the Methodist Hall in Hastings Street. The bus would pick me up at the front gate, and we would get off at the bus stand in Russell Street by Westerman’s, and one of the teachers would be waiting, and form us into a line to walk down to the hall. At the end of the session we’d be lined up, and reverse the process. Funny – they’re going to trial an ‘On Demand’ bus service again. See? Everything keeps getting reinvented.

Alison was born in ‘47. I remember being sent to Gran Thomas’s farm in Marumaru. Uncle Jim milked cows. The toilet [was] a long drop. Gran Clark came up to help Mum with the baby, and then about three months later, home again.

Isobel and Anne were caught by a neighbour one day, holding my head in the letterbox and then closing the lid down. [Chuckles] Oops! Kids do these things. [Coughing] A more memorable event was when Mum took Alison to town in her push chair and got distracted. Us [We] older girls were so envious when Alison was returned home by the police, in a police car! [Chuckles] None of us had had that happen.

Mum was becoming erratic; she would take off on her bike whenever it suited her. Our neighbours kept an eye on us because things were going wrong, and Dad was staying out on the job site. And four girls in the house … mother gone … what would we do without our neighbours? One time she went to Napier at three in the morning – I hope she had a light on her bike.

Dad had started his carpentry business; their relationship being under stress, he started staying out on the job sites Monday to Thursday. On Friday nights he’d take us to the pictures – the Embassy Theatre, he wouldn’t go to the other two. I remember the newsreels showing the Korean War – that was how we got our news in those days.

Financially they were doing well; by the early fifties we had an electric stove and a fridge; a phone was essential for Dad’s business; do you remember party lines? [General assent] “Working” [chuckles] … “Working?” “Engaged”; and we all knew everybody’s rings.

Gran Clark would take two of us for the holiday breaks to give Mum relief; we would go down on the railcar. In summer we’d be sent to the swimming pool and library daily. It was fun, and we got to know our cousins.

In the mid-fifties Mum went to Auckland to see a neurologist; Huntington’s Chorea, or pre-senile dementia was the old name. Now Mum was going to go to Wellington – Porirua Hospital – for treatment, staying occasionally with her mother. Very difficult. I was at Intermediate – I’d just started. How was Dad to cope? The Lodge came to his assistance; the family offered to take two of us; our neighbour, Gwenny, my saviour of the letterbox, took the other two. Wow! Dad could keep working.

After three months he had had enough … “Many thanks” … he wanted his girls back home. We were the team of five. He couldn’t get help from Social Welfare because he was married – funny that – so us [we] girls were responsible for housework, lawns, gardening, meals; Anne was a great cook.

Dad worked in an area over half an hour from town, Raukawa and Argyll East. I asked him once how he managed with the driving on top of the work. He said it was his sanity time – he could think without interruptions. While at Intermediate I would take the banking to school in my bag, and go down in the lunch hour to pay it in – great way to learn about finance. Bank hours were ten to three; no-one ever asked why I left in the lunch break, I just got on my bike and went. Similarly, with health issues – if we needed a doctor, we went on our own. I had to go to the orthopaedic clinic at the hospital for four to five years, every three months, to get a new surgical corset made. The teachers just … “Okay, well she’s off.” “Oh, she’s back again.”

Dad found creative ways to look after us in the school holidays. Alison was easy, she just settled in with Gwenny’s pair. He persuaded the rest of us that it was an exciting adventure to go out and help mothers with their young children – and we got paid. [Chuckles]

In late ‘58 Mum was – again, hearsay – on a drug trial on one of her stays in Porirua. Her friends were happy with her progress, and suggested she go home and spend Christmas with her family. Wasn’t that nice of them?

Dad got the call on Christmas Eve, during tea; no, he couldn’t drop everything to come and get her. He was told he would need someone to help him with her. He said it was impossible, but he was told she had to be discharged; as a voluntary patient they had no right to keep her. He agreed to come [coughing] on Boxing Day. I was it, the nominated helper. The [coughing] trip home seemed interminable in his Morris Commercial truck – not much room on those bench seats, and Mum had to sit in the middle because when we tried the other way, she was opening the door to get out and run away, so that wasn’t feasible.

There was no more of the drug; the improvements were wearing off. Within a week chaos ensued. Mum was the first streaker I ever heard of. [Chuckles] She took off down the street naked, but we never talked of it. We kept everything at home, I t was never talked of outside of home. The minister couldn’t help; the police came and took her to the hospital. The court committed her to Porirua after less than ten days home. Another neighbour used to take us … this was afterwards … two at a time to see Mum, in a one day drive; so we’d drive down, see Mum and drive back.

Gradually, over time, we all declined her well-meaning offers. I was fourteen when I chose not to see her. Isobel was the last to see Mum; Dad had been ordered by his doctor to have a break – he was fairly stressed. He went fishing, which was unheard of – I didn’t even know he had a fishing rod.

Porirua Hospital rang and said Mum was dying. Isobel went and was with her when she died in February ‘64. Her family, aunts and cousins, were at the service in Wellington. One of them followed us back to Hastings … [knocks on table] on the door, said, “I’ve come to apologise. We should’ve done more to help you. We knew about the family illness.” Dad said, “Too late”, and shut the door in their face[s].

Later on Anne suggested that Dad take up a hobby, roses. He did – not just the initial six bushes, but at one time over seven hundred rose bushes in the garden. We became a destination for bus tours; who [the visitors] would walk around the garden and take photos and so on. It was eye catching; [cough] I still have two of the original six bushes. He joined the local Rose Society and worked hard to get the Frimley Rose Garden established, helping with the selection of new varieties for some years.

Dad insisted we stay at school and leave only if there was a qualification to be gained. Anne went to Otago and got a Diploma of Home Science – she couldn’t do a degree because she hadn’t done maths at school – then she went on to Teachers’ College. Isobel went to the Memorial Hospital as a trainee radiographer; later, after a break, returning to train as a nurse. Later on in life, she also qualified as a primary school teacher. Would you believe she was dyslexic? I followed my interest in science and trained as a maths and science teacher, but wasn’t any good at it so did other things; while Alison qualified as a radiographer and that was of great value when she and her husband were in Mt Isa. In fact her husband later said that it was because she was a radiographer that he got the job.

As we turned eighteen, Dad told us about Huntington’s Disease. All we knew was that Mum was sick; didn’t know what was wrong, so when we were eighteen we got told. It was called Huntington’s Chorea then. Now it’s called HD for short – I read up about it; it made sense to me. He also made it clear that if or when we were interested in marriage, we had to tell our prospective partners about it. He didn’t want anyone else blindsided like he had been.

In ‘64 Anne and I were both at Teachers’ College in Auckland, and we went to see the neurologist who had seen Mum. We wanted to discuss what HD would mean for us. He confirmed what I had read. “Get on with your lives, marry young, have children if you want. By the time they have children, there will be a cure.” Well, we are still waiting for that, but there are some promising research results; and bonus, he never charged us.

Anne married first, then me. And finally when Dad had both his unmarried girls engaged at the same time, he talked them into a joint, double wedding. The wedding rehearsal was hilarious, as Minister Bill Whimsey had never officiated at a double wedding before. At one point, when it looked like the guys were going to have two wives and vice versa, [chuckles] he quickly realised that wouldn’t work and reverted to separate vows. Problem solved.

So, what is HD? George Huntington’s family was a [were] GPs [general practitioners] in East Hampton, New York, who had told his son about the local families afflicted by ‘the disorder’. George wrote it up for his medical studies. The Greek word ‘chorea’ means dancing, which refers to the involuntary movements. It is inherited; an autosomal dominant gene. If you get the gene, you get HD; if you don’t, you don’t, and you can’t pass it on. It is an equal opportunity gene, being gender neutral. It doesn’t matter which parent has the gene, if they pass it on, the child gets it.

DNA was first described in 1953. Professor Arthur Veale returned from post-graduate study overseas to set up the first School of Human Genetics at Otago University. Dad started corresponding with him, and he started investigating Mum’s family. I met him once and he wanted a saliva sample. Have you ever tried to spit into a test tube? [Chuckles] It took ages. It never came to anything.

Alison’s husband, Jerry, took on this family tree; it was one of his things, he did his own family as well as ours. [Coughing] And it was determined that Grandfather Clark and his ancestors carried the HD gene and brought it to New Zealand. I never knew this, but every New Zealander is entitled to write to the Department of Internal Affairs, once a year, to get a copy of some document of importance. And being in Brisbane it was a bit hard for him to get this, so he asked me to get the inquest into my grandfather’s brother. And I thought, “Ooh, this is a bit weird, but okay.” Anyway, it was one of the family in Maheno. So I got the report back, and I read it and burst out laughing, and went, “That’s wacky!” The family member had spent time in the mental hospital just north of Dunedin, but when he was well, he was back home with the family. He never married, and his older brother had him come for tea every night at the farm. So – he didn’t turn up for tea on this wet, windy night, so brother took the lantern and went out to look for him. And the inquest was into the cause of death. He accidentally drowned while trying to hang himself. [Chuckles] That just tickled my funny bone.

So, more on HD – the HD gene is one of the oldest in the human genome; it’s also one of the largest. The protein made by the HD gene is one of the biggest molecules made. About five years ago an [coughing] x-ray picture of the normal molecule was published, and this will help with the development of a drug, because once you know what the shape is, then you can organise the thing to go into it, like putting a key into a lock.

The Huntington’s molecule is found in every cell in the body. It mainly appears to affect and destroy the basal ganglia in the brain; however, the researchers are finding that other areas of the brain are also affected. The HD gene mutations repeat [a] sequence of three nuclear tides: cytosine-adenine-guanine, or CAG for short, we talk about CAG repeats – CAG codes for the amino acid glutamine. Genes that have a CAG repeat are known as polyglutamine disorders. It’s been describe as if the gene has a stutter. This is shared with other neurological conditions; their repeats maybe different. The more repeats over thirty-five a person has, the more likely they are to get HD. And we’re toying with this … the more you have, possibly the earlier the age of onset. It is possible for children to be diagnosed with juvenile HD, and that would be so horrible.

So with the stutter CAG present, the shape of the molecule change[s]. So if you have a molecule that was that shape, [demonstrates] when you’ve got to put more in because the DNA has instructed that more of that section be made, you end up with it being that shape, and it doesn’t function very well. So those who have ten to twenty-six repeats have the normal HD gene and can’t get HD.

Genetic modifiers are where one gene affects the function of another gene. At the 2018 conference, a report was given about research into why people with the same number of CAG had varying ages of onset. It was found that those who had the FAN gene found on Chromosome 15 had more stable CAG repeats. It was suggested the researchers check out the HD sheep from South Australia. New Zealand would not allow the genetically modified sheep to be kept in New Zealand; Australia agreed to have them, and they were based in South Australia. They found there was very little variability in the CAG repeats – it seemed to remain very stable at seventy-three, both the original sheep and their offspring. Maybe they fluked it, but who knows? However, they were urged to check out the impact of the FAN gene on the sheep as a future research topic, by those of us at the conference. If we could succeed in pushing out the age of onset by ten or twenty years, it may not be such an emotionally and economically devastating disease. After all, not many families have a parent with HD while they’re preschoolers, like we had.

When Professor Faull came to Auckland University on his return to New Zealand in ‘78, he continued with his research on the basal ganglia. One of the questions he [coughing] queried from families, was my question, “Did my parent have HD?” This lead to the establishment in ‘94 of the Neurological Foundation Human Brain Bank at Auckland University Centre for Brain Research. That’s a bit of a mouthful. Tissue samples are used for research here and overseas, and to confirm diagnoses. One finding in 2007 followed up on a US [United States] cancer research study’s unexpected finding when doing post-mortem studies on patients who had had radiotherapy; their brains showed unexpected [new cells?] This lead to a major breakthrough, and an overturning of the idea that you only get one lot of brain cells – you’re born with them and that’s it. Not true. Richard Faull described them finding the ‘motorway of neurogenesis’ in the brain tissues held by the Brain Bank. This shows that new neurons are being formed all the time. This is the idea of neuroplasticity, which is why stroke victims can recover if they get medical help as soon as possible. HD is an ideal gene disorder for investigation and has lead to insights into other neurological disorders.

In New Zealand we have three HD Associations, Auckland, Wellington and Christchurch. They employ social workers [and] nurse advisors; our advisor in Hawke’s Bay is Jocelyn Pack who joined us in 2017. We meet regularly as a group to share and support each other, and I’m very pleased to see some people here tonight from that group.

I’ve attended all the New Zealand HD conferences since the mid-eighties, plus the Melbourne one in 2011. I signed up for HD Buzz – it’s a great internet site which has up to date information on topics of interest. This one is ‘Vaccines and HD’ – very topical this year – written by scientists for non scientists, so it’s in basic language.

In Melbourne one of the research items was about the prevalence of HD. It’s so hard to know; traditionally it was quoted at five to six per hundred thousand. For this conference Scotland research showed that there, it was twenty two to twenty six per hundred thousand, which is quite a change. The last conference was in Auckland in 2018, and our guest speaker from England was Charles Sabine. He was telling us about the Venezuela situation, Lake Maracaibo, where the incident rate is one in ten. Currently in New Zealand we are quoting a rate of one in ten thousand. So it’s one of these … is it? When do you say a person has HD? When they’re diagnosed? When they learn they carry the gene?

The Huntington’s collaborative research group announced the discovery of the gene in 1993, by a team led by Nancy Webster doing research in Venezuela. Now we had a test. Alice Webster, her sister, later wrote a book, ‘The Woman who Walked into the Sea’, about HD. Their mother had HD; their father was a prominent psychotherapist in Hollywood so was able to tap into some very notable people, and worked from ‘68 onwards to raise awareness of, and funds for, research into HD. Later in an interview, Nancy was asked if she had been tested; her reply, “Why? I don’t have children.”

Soon after, at an HD conference in Wellington, one of the men who had been tested spoke of the process – over six months long. He had to see the social worker, the psychiatrist, the doctor, and finally, if you pass all the tests they will allow your blood to be taken for testing. His reaction on hearing he didn’t have the HD gene? “Bugger!” [Chuckles] “Now I’ll have to think about superannuation.”

In [At] Christmas ‘93 Alison and family came for a visit home from Brisbane. She was worried about HD and wanted all of us tested. She had the test: positive. She left her job. Then my sons indicated they were thinking of marriage. I had the test: negative. Isobel had the test in ‘94: positive. She lost her job. Anne refused to be tested but lost her job. Later she had a positive result. All three had HD; all presented differently … movement issues, cognitive problems and depression. To an outsider it would’ve been hard to tell, in the early stages, that they had the same disease. I told all my cousins [about] the family illness; one cousin had the gene test: negative. We celebrated. Soon after, she was diagnosed with early onset Alzheimers.

A sad thing happened with Anne; her HD was very strange. She took against me and decided that I had been a successful solo parent, and she just decided to cut ties with me, and so there were some sad consequences to that. She also convinced her family that I gave her HD by talking about it. This is the muddled thinking that can go on.

In New Zealand today – this is a really strange finding – a couple with a positive HD test can have one free IVF round. As a bonus these couples are providing valuable data about fertility to the researchers as they do not have fertility problems, so get a better result from IVF – they have a greater chance of success.

I visited Alison in 2005 when her husband told me she was very ill and had been hospitalised. Sudoku had just come out, and I was hooked – it’s great to do when sitting waiting. Alison asked what I was doing; her speech was very hard to understand due to the muscle problems. Her daughter was there, also doing Sudoku. We told her, and then she went, “No! No!” And she was right … I had got it wrong. [Chuckles] The ability to think remains while the body deteriorates. After diagnosis, usually when in the mid-forties, it can take fifteen or more years before the patient dies. Dad often said, looking back, Mum had it when they married.

In August ‘15 my niece sent an email: “Mum”, Isobel, “is not too well.” Then she sent another, “When you come, can you bring ..?” Thank heaven for the internet; I booked the ticket, I went with the items and was with them until she died … took about for four and a half days. [I] asked questions as to what she wanted. One issue was brain donation. They decided this was what she would’ve wanted, and it was organised before she died, so that once she died, the process could start. Maybe we will get some answers to help the next generation from this gift.

Now all three are dead … Alison in 2007, aged sixty; Anne in 2011, aged sixty-seven; Isobel in 2015, aged seventy-three. Dad used to say, “This is a bastard, mongrel disease.” He didn’t believe in swearing in front of women. Now it’s over for them; it’s over for my children, two of whom are here tonight, who really don’t remember my sisters except from when they were healthy. But it is not over for my nieces and nephews. Huntington’s is a family problem … a family disease not an individual one, as it affects every member of the family whether they actually get the bad gene or not. Our community helped raise us; our neighbours were fantastic. So, thank you neighbours, and thank you for listening. Any questions?

[Applause]

I’ve got to say first, Marjorie, that you said you’re a failed teacher; well you’ve certainly switched the board in your older years.

Oh, thank you.

Question: With deciding to have children, can those pregnancies in early stages be tested for the gene?

Marjorie: Well, it’s a can of worms. If the parents have chosen not to be tested, – if they then [microphone interference] have to end the pregnancy because of the gene being present, that parent has been given the knowledge that they didn’t want. And it’s a real ethical dilemma. We fought quite hard in the Association not to have insurance companies use that information, or demand … but if you have a test they can legally ask for the result of the test, and then of course that changes totally the premiums the insurance company might ask of you. It’s a fraught area. When my husband and I decided we would get insurance, life insurance, to protect our kids in case one of us dies, I went to the GP in the town we were living in. And he was filling out the form and he said, “What did your mother die of?” And I said HC … Huntington’s Chorea, in those days. And he looked at me, and he said, “Nonsense”, and so I got it at the ordinary price. [Laughter] So there are people out there. How did he know that I didn’t carry the gene? It was weird. But I benefitted.

Question: What actually does a person die of? Is it a heart failure, or ..?

Marjorie: Well basically the body shuts down. As I say, the only one I was there with when they were dying was Isobel. We sat in her in her room in the rest home, and the staff member came in. And she was never fed or given water. And that was from the Monday; she died at three o’clock on the Thursday afternoon. There was [were] no demands being made on her body, and just basically, it just wears out. It just can’t operate any more. So yes …

And one thing some of you may have realised is that there is a very high risk of suicide in Huntington’s families, and a few years ago a young man … and he was only in his early forties … got the diagnosis. And he lived in Wellington; and he thought about it, and went out and killed himself rather than put his family through it. So I think he may not’ve had good counselling. And I feel very sad about that. I’ve always wondered.

Question: Historically, if I’m right, Huntington’s was first diagnosed in the 1880s, 1890s?

Marjorie: It was first described by George Huntington.

Continued: Because my great grandmother died of Huntington’s in 1922. We are obviously the lucky branch and have lost the gene. Looking at the wider family, what would the cause of death be listed [as] on previous generation death certificates? Is it easy to track?

Marjorie: Mum’s one had bronchopneumonia‒Huntington’s. It varies going back … I didn’t bother looking at the death certificates. Jerry got them all when he was doing the family history [coughing] so they would be in there, but he didn’t record it in the story he wrote about it. But it is quite fascinating … as part of the work on the family histories, this is one of the Lowney family which is a branch … they married into the Clark family. And this one starts back in 1878. They sent Jerry this as part of the family tree. Ooh! Amazing! So there’s a lot of work with the genealogy people, and [if] any family member approaches me I just give them Jerry’s email address and say, “Get hold of him.” [Chuckles]

And there’s a photo of Mum and Dad on the day they got married; it’s not a very good photo. He didn’t know what he was getting into, and it’s what happens when you marry someone from a totally different community – there’s no-one around to whisper in your ear. [Chuckle] With my grandfather who got knocked off his bike and run over, I am very strongly of the opinion he had HD, and I feel for the car driver because if he was wobbling his way along on his bike, I think he fell in front of the car rather than the car running him over. Does that make sense?

Question: Yes – you were talking about brain donation; both my father and my brother left their bodies to science. My father left his body to the Medical School in Dunedin; my brother … 2019 they left his body to Auckland University Medical School or whatever – the whole body, kind of thing, so I guess they would’ve looked at his brain as well, I don’t know. So you can sort of specify that you want the … or can the family allow the brain to be donated?

Marjorie: There is a slight problem in Hawke’s Bay in that we don’t have a pathologist; and so we’ve been unable to organise donations from this area because of a lack of a pathologist. But Isobel was in Dunedin and the Otago Medical School was just down the road, so they could organise the procedure and get the brain up to [the] Brain Bank in Auckland. If they get it there fast enough, there are certain tests and procedures they can do. And I was quite fascinated – I’ve done a tour of the Brain centre, and they can get living cells from the brain after death on receipt, and use that to start cell lines. You know, you don’t realise half of what they get up to.

But if you think the Brain Bank sounds fun … when you actually go in there it’s a room that’s lined with chest freezers, [chuckles] and that’s the Brain Bank. But they’ve been doing some stunning work, and some of the donations go back twenty, thirty years; that was how they were able to find out about the neurogenesis. But we’ve had an interesting tale here in Hawke’s Bay, and I don’t think they’d mind me telling it, but the oldest person diagnosed with HD in Hawke’s Bay is eighty-two. And she was in our group; she went into hospital for a little minor procedure and came out with a diagnosis of HD … took about another ten years to die, so that’s okay. The family organised brain donation – we did have a pathologist then. And one day at the support group her son came along and said, “At last Mum’s doing what she always wanted to do.” “Huh?” “Yeah, they’re sending samples of brain off to Switzerland and so on” [laughter] … she always wanted to travel.” [Laughter] So I thought that was a nice one.

Marjorie, that’s just been fantastic, thank you – I feel I’ve been in a science lecture. And a just wonderful, wonderful family story from day one, and with your lovely links to Milford Sound, Oamaru and Maheno – we know these places so it’s just fantastic; thanks from us.

[Applause]

Marjorie, thank you so much for the effort you put into this. And I believe you do give talks, and I’m so grateful that someone does that because the panic in families must be just terrible.