Original subtitles

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in 1814 a baby girl was born in a tiny

Scottish village

she was named Margaret after her mother

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her father worked long hard days cutting

trees in the forests of Aberdeen sure

when she grew up she married her

sweetheart in a dress that despite her

tall figure failed to obscure eight

months of pregnancy over the next 18

years Margaret gave birth to seven

children

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and when her youngest James was two she

was admitted to the Aberdeen lunatic

asylum

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when James was 9 his dear sister Jane

set off on a sailing ship to a place

they called New Zealand she said it was

on the other side of the world

as soon as James came of age he followed

his big sister to that faraway place was

he running away from the specter of his

mother's madness we'll never know but he

took with him an inheritance deep within

the fiber of his being

James passed the inheritance to his son

William who passed it on to Mavis my

grandmother

my name is Bridget for generations

Huntington's disease has decimated my

family hammington disease has been

described as the worst disease known to

man Huntington Rob's you of who you are

involuntary movement which is called

Korea it's actually a word that comes

from the Greek word meaning a dance and

we get the modern word choreography from

the worst thing is watching somebody's

personality melt away

Huntington patients are really trapped

within their own bodies this also an

increased incidence of suicide it is a

slowly progressive disease that plays

out over over a couple of decades we

don't have many treatments for

Huntington's disease and there's no cure

for ending tenses at the present time

but even worse than that is that at the

time that you might be dying of your

illness is the recognition that your

children might also follow the same

course will my generation witnessed the

yielding of this affliction or will it

continue unabated this is the story of

me and my family facing up to an

invisible enemy that threatens

everything we love I was too young to

know my grandmother Mavis very well but

my mum would tell me about her she would

take herself to town and go and buy that

and she would fall over on the street an

alcoholic the chemists shop she went to

a lot and he was very he would take her

and get home so she ended up in hospital

and I'll never ever ever forget going

and then finding her in this ward and

here she was she must have been on

valium and she was just all the traces

of the personality had gone and she was

too stoked up to the eyeballs just

sitting sort of in retrospect we should

have done more for him I didn't really

realise that that state what it was but

she may have had Parkinson's and

mum was shocked she was told that it was

hereditary the odds were 50/50 that she

had inherited it and if she had she may

have already passed it on to any number

of her three children

Mavis died after 15 years of mental and

physical deterioration at the age of 62

mum told me to have the chance to say

goodbye it breaks my always knew about

the risk of Huntington's disease because

of my parents attitude that they

shouldn't hide anything from their three

children and we grew up in a frenzy of

adventure with a family motto of trying

to fit in as much as we could to

everyday meanwhile scientists had been

hard at work and in 1993 the gene for

Huntington's disease was found after ten

years of searching near the top of

chromosome 4 the following year mum took

advantage of the newly developed

predictive tests which could tell her if

she had inherited the HD gene it doesn't

skip a generation so if she didn't have

it then me and my brothers couldn't have

it when jude was tested positive for the

disease I was just crying my eyes out

was a tragedy to know that this disease

could be possibly passed on to you

children

genes are made up of four chemicals that

are represented by the letters a C G and

T what the scientists had found was

remarkably simple on the top of

chromosome four the parts are spelled C

AG everyone has this gene but people who

have Huntington's disease have too many

repeats of CAG in theirs that's what the

genetic test tells you how many CAG

repeats you have mum has 43 particularly

over 39 people would tend to develop

Huntington disease anyone who has an

expanded gene has a 50% chance of

passing on that gene anytime they have a

kid so it's exactly like tossing a coin

if you could actually get that

surgically implanted here really makes

me need to carry it around and free up

your arms after some years of living in

the UK where my brothers Nick and

Warwick were also living I settled in

Sydney and by 2003 I found myself in

love and unexpectedly pregnant I'd been

so sure I would find out my gene status

before bringing children into the world

but Geoff and I found termination wasn't

an option after our beautiful boy was

born I quickly had the test I was

thinking I'd be let off the hook that

they would say you don't have

Huntington's and I'd go around cheering

and everything would be lovely

but it wasn't I too would dance the

Richard dance

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what normally

tragically around 10 percent of people

with Huntington's are children but the

average age for adult onset is between

35 and 45 and generally the earlier you

get it the faster it progresses

luckily mum didn't start developing

symptoms until her mid-50s she'd always

been a great talker and words started to

fail her

this little plasma symptoms crept up on

her I found my test results had left me

feeling guilty for my child's future I

thought if I came to understand the

disease more I could show him I wasn't

afraid of it as an HD family we had been

inextricably linked to scientists

studying the disease my mum had been

friends with the visionary

neuroscientist Professor Paul for many

years he's the director of the center

for brain research in Auckland where

they study Huntington's brains

she had knows something about this meter

she was always the person who it felt

bit of having met so what is really

interesting about Huntington's disease

is that it causes cells to die in their

millions and the area called the basal

ganglia it sits right in the center of

the brain in the normal brain you can

see the basal ganglia via this the

normal brain whereas man in his brain

they are striking down as a result the

ventricle becomes massive along with

professor fall scientists all around the

world had been working passionately on

understanding Huntington's for decades

their progress was gathering momentum

the cerebral cortex at enormous sheet of

tissue around the surface of the brain

which actually separates humans from

other primates this is one of the most

evolved parts of the human brain also

becomes dysfunctional but the more we

look the more we find to understand what

they had discovered we have to go

deep down into the fiber of our beings

to see our bodies a million times

magnified when molecular machines

replicate the mutant Huntington's DNA

unaware of the mistake in the recipe

it's a bad gene which makes a bad

protein and it does a lot of bad things

and that's a scary thing you know and

some diseases you're lucky and it might

be you know one bad protein maybe like

kills a blood cell or they're really

difficult thing with Huntington's

disease and the Huntington protein if

there's a lot of bad things

proteins hasta together so they become

kind of sticky like glue so they form

these very small balls inside the nerve

cells inside the brain is a bit like

pouring cement inside a brain cell which

calls the brain cells to die this gene

is expressed everywhere in all the cells

from the body and we might get effects

in all the cells in the body so it's

it's it's more than a disease of the

brain I would say it's estimated around

600,000 people worldwide have

Huntington's disease symptoms are varied

in their severity and onset for each

person it helps to break it down into

four stages

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that's because I've anyway the initial

symptoms are quite subtle mum started

getting the movements late at night and

she'd sit there with her leg or her arm

jerking around but she'd always hold

them down the person may become offer

familiar with strangers they may lose

the ability to understand other people's

emotions I'm happy impossible and the

idea the the steps so I know what to do

what what to what to acknowledge and the

hardest part probably the irrationality

of it people might do bizarre stuff like

start buying things that they would have

never spent money on before in and go

through a lot of money and also being

unable to work so families end up often

in a very poor financial situation there

should never be nothing you can do for

someone with Huntington's disease it

bothers me that I think around the world

patients and families are still left

with that impression they go to the

doctor's office and they're told well

that's a fatal disease there's there's

nothing I can really do for you I was

always told as a medical student we

don't make new brain cells and then the

Huntington's brain mercy we found that

this layer of stem cells was in fact not

only there

but we saw it multiply the more advanced

the disease the more new brain cells the

brain was trying to make from these stem

cells in other words it was trying to

appear itself by studies that we've

ended on the normal brain we found that

every person can make new brain cells it

was revolutionary

we made this discovery that if we took

the mice and increase their levels of

metal and physical activity we found

that led to a dramatic delay and onset

of these symptoms of Huntington's

disease in the mice the idea that you

can stay more mentally and physically

active and building a brain reserve but

together with a good diet and other

things that if that are healthy for the

body and therefore healthy for the brain

mum exercised took supplements used

brain training and alternative therapies

she read and know everything that was

going on about the disease she would see

all the challenges and the ways you

could overcome the disease we're taking

a different attitude to it and that

we're trying all sorts of things if

there's possibly this'll work we'll give

it a go we believe that cumulatively

they

to slow down the progression of disease

so you really live the way with finding

finding ways to beat Huntington's the

one thing that every every every HD

person will say is that they are

convinced without question that exercise

is helpful aerobic daily great couldn't

it have been that lying around like a

slob was the thing they found helped did

it have to be exercise I'd avoided it

reasonably well for 20 years and now it

was my lifeline the problem is you have

to get going quite fast to get brain

regeneration and I wasn't going that

fast

Charles Sabine a former TV journalist is

now a spokesperson for people with

Huntington's disease

he's got the gene too as I talked to him

I sensed that he lived with the same

knowing that I did the same background

hammer of dread the important point is

environmental enrichment what you don't

want to do is sit in a room so it's not

to have any stress the one good thing is

about the freakin disease is that grass

is good for you it's dope really

cannabis can avoid receptors good for

you

sex is probably good yeah I mean I just

say that as logically as because it's

environmental enrichment it's exercise

that's getting your heart rate up

depending on who's on top

speaking of which Jeff and I wanted to

have another child

lucky for us those hard-working

scientists had come up with a way to

have children free of the disease in

early 2006 we booked in for IVF our

chance of success was around 35% in

addition to normal IVF we had to go

through a process called

pre-implantation genetic diagnosis we

had 22 eggs collected 5 days later only

four had grown to the size they needed

to be for testing it turned out that

three of the four had the Huntington's

gene so we had one embryo without HD one

chance we were extremely lucky and the

embryo resulted in a pregnancy and a

gorgeous baby boy was born who has 0%

chance of having Huntington's disease so

if you don't have the gene then you

can't pass it on that's right and that's

why with our second son he he won't

develop Huntington's and he won't pass

it on and your first son you don't know

have you discussed with your partner

about when he should know the genetic

counselors strongly advised that

children aren't tested for Huntington's

and so it's after he's 18 he can decide

for himself whether or not he wants to

be tested so I don't think it is

something that he needs to know before

he's an adult

we didn't have available the technology

that Bridget and Jeff have used if PGD

had been available and had we known at

the time we were conceiving our children

would we have done that absolutely we've

come along once mum's brain deteriorated

further she would visit us and do crazy

things like wander around naked seeing

things that weren't there and I would

watch her horrified walking about like a

living nightmare

I would imagine myself that way in my

future and I would imagine my son with

it and go back to being horrified for

her if my son does have the gene I worry

that I won't be there to look after him

a friend of my mum has been looking

after her son for the last 19 years you

remember Judy don't show he's been on

the ticket for research she's now on the

pureed food so he sort of had those few

symptoms of 27 and I've got him on the

medication for hallucinating we don't

know what he says sometimes it's

absolutely yeah I mean it well that's

what it is that just just definitely

crates you can only do so much to hell

you know you you kind of powerless

really as a mother you think I can

always look after much

take them we can't but she just can't go

in and you've got to keep on doing what

you can do

I am inspired by her by the way she's

she's fought to to try to keep the

symptoms at bay

I was probably it more inspired a few

years ago and just lately she's been

deteriorating quite a lot one of the

symptoms of Huntington's is losing

social inhibition we went to a cafe once

and she got too hot so she took both her

tops off and she was sitting there in a

bra people with Huntington's disease can

develop psychiatric problems the most

common is depression the other area

would be psychosis believing in things

that are not true and also

hallucinations as seeing or hearing

things that are not perceivable by other

people she was starting to think that

dad was really out to get her and then

after a while she'd been there she

started to think that you were out to

get her and then finally she started to

worry about me being at together she

thought that I was trying to kill her

and it was until we got to a drug called

seroquel that we got it got on to a drug

which she could live on as soon as she

took those pills within 24 hours she was

back to normal just about everybody who

meets her and comments to me says look

she's trying she's doing so well but she

does look like she's drunk one thing

they say about the career you know the

movement disorder is that it actually

bothers other people much more than it

does the person with E because he's

horrible to see it's very scary my

brother's gone really bad right now

she can't keep her League still I'm

trying to

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the connections between the deep

movement centers and the frontal lobe

the big bit at the front of the brain

that's unique to humans and gives us our

personality but also a sense of self and

so the connections between those two

areas are affected early in Huntington's

which is probably why we get this

combination of involuntary movements but

also loss of insight and particularly

loss of insight into those movements I

can't really have a conversation with

mum the responses are you know they are

quite simple but sometimes she can be so

lucid so all of a sudden you can have a

conversation about something you live

for those moments

night undersell got him may have had

instantly

and because my brain she's still going

for a walk it's best she can she's still

going to the gym we were looking at a

trial on a drug called Demi bond it's

the big hope we have he has to tell him

to do everything you know to get dressed

which clothes to me and she follows him

about and yet still he just loves it and

you'll do anything for you family

divorces make sure she she thinks she's

beating it and it's the drugs you know

in her mind she's getting better I don't

have the heart to tell her otherwise

speaking left

it continues to amaze me how courageous

and resilient families are with

Huntington disease for some people this

will bring up the beast and family and

other families the gene tears families

apart you cannot go through the course

of Huntington's disease alone and in

fact I don't think that that a couple

can really go through the course of

Huntington's disease alone just the two

of them in fact I feel as a physician I

can't really care for a patient or a

couple with Huntington's disease by

myself I think it is done best by a team

by a community by a family people's

relationships change from being husband

and wife it's very different to being

carer in patient it's a totally

different relationship the thing that

motivates me is going back and looking

at photos of her particularly when we

were courting and when were engaged

somewhere a married he's so proud of how

gorgeous you he was so proud he go

who would ever deny she isn't worth

looking after for the rest of her life

such a stunning person it's easy knowing

the times we had then to say wrong okay

it's my turn my turn to look after brain

just doesn't compute those instructions

and I think she's aware because to me

things like yeah you're lucky you're

from a strong family some people have

got nobody else and if I don't help them

there is nobody either their families

are dead or they've designed them

because they've created mayhem or

they've got no money or they've drunk

themselves into oblivion I had a client

who was very very unwell in he had a

phone bill and he couldn't pay it and he

was living in a house and had this huge

lemon tree out the bag

like we didn't see if I've got this

great recipe for a lemon cordial that

was my grandmother's so I started making

it and I've sell it so that I can pay

the bills for people buy them things

that they need Jeannette also takes

people to the hydrotherapy pool

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they of his balls she chokes sometimes

she sees a psychologist in she's on

medication six boys donor to me today I

think these three vectors Tendo

Karen was only diagnosed last year and

she does have quite a few

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other than not don't move

but we've certainly had people that taxi

drivers have thought they're drunk and

looking

when I was a child my mother became a

founding member of the local

Huntington's Disease Association and did

her part to raise awareness of the

disease in the eighties she got a book

about living with Huntington's disease

from dr. Edmund Chu for me it was the

first time I'd seen the words

Huntington's disease associated with the

word living rather than the word death

this man dr. chu became a point of light

in my teenage brain advocacy and

Huntington's disease has mostly been

family led when the singer Woody Guthrie

died of it in 1967 his wife Marjorie

campaigned to raise awareness of it a

year later

Milton Wexler set up the hereditary

disease foundation after his wife was

diagnosed with the disease they had two

children Nancy went on to be

instrumental in the discovery of the

gene and Alice became a historian I'd

always wanted to know more about where

the name Huntington had come from Ellis

told me about the town of East Hampton

in the US where in the 19th century

there were many families with the

disease in 1806 there was in the

newspaper a little article about a woman

who had disappeared one morning her

husband had come home she was working at

home she was ironing clothes and her

husband had gone to bed leaving her

there working late at night but when he

got up in the morning she wasn't there

it has become our duty to publish the

following melancholy circumstance which

took place at East Hampton a few weeks

since captain David hedges returned to

his home in the evening and found mrs.

hedges ironing clothes and apparently in

good health even the doctors journals it

had this woman whose name was Phoebe

hedges had called for the doctor to come

to see her she had been to prescribe

various calming

after considerable searchin enquiry her

footsteps were traced from the house

through fields of grain to the shore and

there is every reason to believe she has

precipitated herself into the surf which

washes to South Shore

mrs. hedges is about 40 years of age and

was much esteemed by her neighbors this

extraordinary step is attributed to her

extreme threat of a disorder called st.

Vitus dance with which she began to be

affected and which her mother now has to

a great degree

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transyl arrangements of their clothing

it appears she had for some time

contemplated her melancholy and sixty

years later in the same town a doctor

the son of the doctor who treated Phoebe

hedges was visiting patients with his

twelve-year-old son we suddenly came

upon two women mother and daughter both

bowing twisting grimacing the young

boy's name was George Huntington he is

an interesting guy because for one thing

he was only 21 I think when he wrote his

famous paper describing the hereditary

pattern other people had written about

it they knew it was hereditary but not

the specific way that it was inherited

his father and his grandfather brought

physicians in East Hampton so they had

been there for almost 75 years and they

had as their neighbors families with

this disease so they had seen the

different generations and George

Huntington always said without his

father and his grandfather he could

never have written the paper that he

wrote subsequently of course the disease

was named after him around the same time

over the other side of the Atlantic and

Scotland my great-great-great

grandmother was decaying in the Aberdeen

lunatic asylum 29th of April 1856 named

Margaret per day 42 years there was at

present marking the disorder or

unnatural elevation of spirits violent

temper and a disposition much the

reverse of that natural to her she has

been violent towards her husband at

times she has also several delusions of

witchcraft and others such as that

people are doing her harm

casting ill upon her but latterly her

talk has been mostly about money making

on the 12th of February 1857 the doctor

noted this case has remained stationary

since admission she is noisy excited and

incoherent when addressed five months

later increasing emaciation and ability

of body have become associated with

acute attack of bronchitis which has

confined her to her bed for about a

fortnight and which seems to be

threatening a fatal termination nine

days later she was dead

her son James would have been just three

at her funeral he would have had no

memory of her when as an adult he

emigrated to New Zealand well my face is

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burns I am bringing you he married

another immigrant when he was 33 an

English girl called Mary and had four

children he was 46 when he accidentally

drowned crossing the Manawatu River

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his son William lived long enough to

develop the disease however and in fact

be diagnosed by a doctor out from

Scotland around 1950 who recognized

Huntington's disease but for some reason

the rest of the family was never told I

can't imagine what it would have been

like to bear such a burden alone I am

bringing you

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skiing had always been the way our

family came back together even when we

lived far apart but this time in 2009

our family was struggling with the

fallout from predictive testing four

years earlier my eldest brother Nick had

taken the test but chose to keep his

positive result very private just two

close friends and family meanwhile my

other brother Warwick prepared to take

his predictive tests this strong family

was creaking at its foundations a hell

of a legacy to leave your kids to have

to deal with the odds are still 50/50

although it's tempting isn't it to think

that it's gonna fall the right way this

time well in our family we'd all wanted

to be tested

it wasn't something everyone at risk of

Huntington's wanted to do by far the

majority of people are not having

predictive testing but those who want

the predictive gene test really wanted

when I was working for NBC in the last

days of the war in Bosnia I was taken

captive by some Russia Hadean guerrillas

and who would had executed some people

the day before so we were by the wall

where they'd executed these people and

one of them held a grenade to my head at

one point which about is is a scarier

moment as you can imagine but that was

not as scary as taking that test for HD

for someone who already knows about what

Huntington's disease is to go through

that test which is so going to be so

completely and utterly determining so

this is Meccan fate I've got written by

Ella Switzer about her family there's

something at the front of it everyone

real or imagined deserves the open

destiny of life the open destiny of life

that's what got me that's what I feel

like it's been taken away because I did

the predictive tests did you think I was

absolutely no way I'm gonna have that

was he when I was a kid my mum didn't

know what she was so it needed both of

you - yeah and that would be really

unlucky right

yeah well good luck thing you always

start thinking about luck don't you do

finally start thinking about casinos and

like red and black lettering coins and

stuff because I thought my father had it

yeah my brother had it

someone's got to not have it by a lot

you know it's like surely that's right

because I was the first one to be tested

so I thought I realized when my brother

got tested and he also had it I was

pissed off I I thought I'm supposed to

be the fall guy

and so my my second brother the third of

our siblings is gonna have his test soon

and we're all just going oh god where it

took the test and he too tested positive

three out of three bad luck it's

devastating tonigh that we passed on

this Hanuman's gene

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my heart cracks there'll be no one from

our generation left functioning my

partner Jeff will be the only person

left standing

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were can I talked about other things

about everything else but Huntington's a

few days later I called my other brother

Nick in the UK it surprised me a bit and

you're never ever pause to think about

have I have been hard done by it you

know because there just simply isn't any

point it was Carson's time forty years

ago

you know we need to plan to be about to

support one another and we're gonna make

sure the boys are taken care of and

we've got to think about how what we

want to put ourselves and those that

will be caring for us through there's

the big question about how much of the

HD experience you want to have - you

know I you know that I'm not interested

in these middle or late stages of them

no no plans to go there that sounds

McCart doesn't it but I mean this it's

probably a fair like I'd go of the Swiss

appointment-plus I've also had the most

astonishing time it's hard I'm a bit

touched to life to be honest I'm really

attached to it so attached to it yeah

you have to give it up at some point

right

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I hear about someone who might be able

to help me a real expert on living with

this disease all of us

because most of us passed away between

40 in 48 I'm saying my sister passed

away and did these suicide in the car

but would they think you clean up I'm

thinking about doing more my sister dead

cuz I thought at least my son wouldn't

have to wash me today I perfect I had

myself

I thought maybe not because I fail to be

selfish to go thing I've got two

brothers Emily

we all have had the genetic test and

we're all going to get hurt Ethan's oh

yeah I'd like some advice on how you

manage that it's a bit of a I can cope

with a bit of humor

I suppose of maybe they should be I

think in my face in my church asleep it

and I've fine

virtually a I feel occasion made my

movements on this book I think of the

effect of drew the victim but all good

man

and kept it a secret

I try with him

I don't handle the idea of of imagining

your future with Huntington's is that

you think oh no I'm going to be an idiot

I'm going to lose everything that I've

worked so hard to be you know as as

you're losing all these things that are

important to you conscious of it I don't

know how you deal with that

I mean debate it is the healthy way to

deal with it

the problem right now is that whenever I

do anything silly I think that it's

Huntington's coming on I'm really almost

smack bang in the middle of the the time

when you're supposed to get it he can

tell me that maybe I don't have any

symptoms but I'm still stuck with the

fact that I'm gonna get it sometime

I should be exercising a lot more but

I've really turned a corner with it you

know and now I'm running and swimming

and doing yoga Nick my older brother

makes it sounds so intense and he's so

intense about it he does the Sault Ste

Marie says this is a discipline for the

rest of our lives if we want to keep

ourselves off the feeding troubies

deeply dramatic and also deeply freaks

me up Bridget I'm going to give you a

letter of the alphabet and I would like

you to generate as many words as you can

potato lion tiger hamster name these

pictures for me kangaroo we now realize

that people could develop thinking

difficulties up to 15 years before they

develop movement problems so we've

tested your thinking ability and your

movement today and both of them are

clear so that that's good news there

many circumstances where different

family members in the same family have

the same CAG repeats counts but these

two have different ages of onset

that was it then I'd watch for early

symptoms at least I wouldn't be like my

great-grandfather William and

grandmother Mavis as their symptoms came

on they would have had no idea what they

were when great-grandfather William was

finally diagnosed and he would have

found himself living in a society

inhospitable to hereditary disease in

the late part of the 19th century early

20th century this movement called

eugenics became very popular to bring

about quote-unquote better breeding to

improve the human race there were many

advocates of compulsory sterilization

particularly in mental institutions

there was a biologist named Charles

Davenport who was the leading eugenics

scientist in the United States and the

conclusion that he drew was people with

Huntington's should not have children

other people advocated that people at

risk for Huntington's ought to get

sterilized one of the main ways that

eugenics has influenced Huntington's

families by creating the idea that of

course you shouldn't have children for

generations upon generations the disease

has been associated with evil bad

families you know witchcraft so

inevitably there has been a shame and a

stigma about the disease which has

profoundly affected the status of the

people who have it for me personally

when I started to learn about this

history I got much more understanding of

my mother I had been very angry at her

why didn't you tell us this this is

really important for financing me to

know about she had grown up during that

era of eugenics in the 1930s and in fact

had studied biology so I thought she had

good reasons for keeping it a secret the

misdiagnosis tends to tended to happen

because people you know general

practitioners and you know and and

doctors didn't see the disease because

it was hidden so they didn't recognize

it so people who did have it or just

died

as being lunatics and two stuck in an

asylum or even more recently diagnosed

as having no schizophrenia when I tried

to learn about Huntington disease in

South Africa or Africa in the late 70s

it was almost nothing written in fact

most of my mentors told me it didn't

exist we win then went on to visit every

single mental hospital in the country

and identified hundreds of patients with

Huntington disease it has been massively

massively hugely underestimated in its

prevalence because of all of this in

some parts of the world even today just

as they's discrimination with people

with mental illness hammington disease

is hidden up until this point people

have been ashamed about having this

disease many families still don't talk

about it and if they do talk about it it

doesn't go well they might have seen

that the grandfather had Parkinson's or

somebody committed suicide and then they

just said they had some psychiatric

illness my family like including my

mother and or my brother and his family

do not say the H word I remember was

very open when she tested positive

she put a notice on the staff notice

board telling everybody she tested

positive funny because disease these

diseases are just like diabetes and

cancer

these are unfortunate but they don't

carry any other value judgment they

don't say who you are as a person they

say you're just unfortunate

communication brings awareness and that

process dissolves fear the patient's the

families who are open about it who

engage themselves in the larger

community who who do not marginalize the

person who's suffering with the disease

amazing beautiful wonderful things

happen

[Music]

June of 2011 Judy was diagnosed with

rectal cancer as well because it's a new

her ballot also means that she is

incontinent her feces because she

doesn't understand necessarily what's

going on she might take everything off

and and just go to the toilets wherever

because she's not conscious of doing

that she's probably just gonna die of

cancer which is except that it's really

tricky because dad's trying to deal with

her of course I mean I don't mean to

sound clever I know you know you can

sound but do you know I wish that my

father there could have been something

that could have taken him yeah before

the last year

the cancer grew and blocked mums bowel

so if she didn't have a colostomy

operation she would die

as a family we'd previously discussed

that we would let the cancer take her

but when it came to the moment of truth

we couldn't let it go for your father

the hardest thing is that he really

thought they had slowed your mum's

deterioration and they've done

everything that they could and I think

that's really difficult for him to now

see that things haven't been stopped

[Music]

you are having a bit of trouble with the

carers how does that been more recently

and when did you now see your

grandchildren what their names ones you

saw most recently James hello just come

and see how you're getting on it's a

nice thing to say George that's a really

nice thing to do today it used to be

lovely when we went out four cups of

coffee into the pool and things didn't

no it's great

yeah yeah it was great what are you

watching on TV me what's this program a

bit you ble is it a music program Jeff

and I and the boys had moved to

Wellington from Sydney to be closer to

family

why are you out a bit because I think

basically I really should be with

another group of people should I call

you to everyone else's watch me you like

me out here it's the most beautiful day

I think it was seen to make ourselves

decent then one night I was saying good

night to mom I stared in her eyes and

she stared back at me with eyes filled

with love I soaked it up we've connected

she blinked and told me she hated me I

guess it is sad for us for our

relationship in a way cuz it's um

that's very little to her because she

can't react emotionally to me like she

used to physically like she used to

symptomatically from the cancer point of

view I don't think there's been much of

a change but certainly from Huntington's

she has deteriorated that's a big job

isn't it

horrible don't worry hey let's just

worry about having breakfast

watchin here we go that's a met guys huh

yeah hey hi

thank you how you doing today really

awful and what do you like to the show

do you like the carries giving you a

shower

why can you tell me what you think mom

is thinking you mean can I look at her

and tell tell what she's thinking it's a

pretty big vacuum there and how old are

you 20 and then you married yes are you

married

you constantly do what you can to keep

her in the best of health to keep her

from pain

[Music]

I don't know where it goes from here see

this things like eating you still eat

him 1 2 3

where did it hurt

further down the track it depends I mean

you might find that the cancer leaves it

behind don't have to worry about that

sort of thing but she is at times in a

lot of pain and I can only assume that's

from the cancer

[Music]

since coming to live in New Zealand I've

been I've found me confronted by mum

situation and really really honestly

been thinking she'd be better off not

living

and I just kept anticipating that she'd

be kind of dying at some point for the

cats are all from the hunting tips

but having spoken to Jeanette it seems

that people can go on for for years and

years in the state that mums in maybe

mum being able to see her grandchildren

play on the lawn is the equivalent of

doing something incredibly marvelous

been away what she's doing it's kind of

what Buddhist monks achieve

thoughtlessness they they call it

mindfulness they doing

as a youngster I always believed that

mature would come along form um I guess

then I was hopeful that a cure would

come along that would that would fix me

the genes been around to twenty years

and initially people thought there was

going to be a cure the next year or the

year after I think I didn't know what to

believe about the likelihood of a cure

the dimi bond trials had been

inconclusive I went to find out for

myself I got to meet a hero of mine the

scientific legend Michael Hayden I've

got good news or bad news for you you

might have more time than you expect so

you know you might have more time or

compos mentis I suspect we're gonna find

ways to modify our reality in one form

or another who knows what it's gonna

take

and the key thing I think we should

recognize there

and I think there's a light at the end

of this time was this your mum or dad

mom so your disease is going to be

different tomorrow there was a time that

polio was a fatal illness or that AIDS

we entered all these diseases have been

overcome Huntington's is different but

we're making massive inroads and no

communities come together in ways that

are incredible so I'm quite hopeful I'm

hopeful not that we'll cure this disease

but that we'll have a way to change the

course of this illness in your lifetime

scientists around the world have now

come together in a community that's

really profound sharing cooperating

collaborating in ways unprecedented

amongst scientists I think that

Huntington's disease is sometimes

regarded as a as kind of a model disease

for perhaps both Alzheimer's and

Parkinson's the level of cells there's a

lot of commonalities between what's

going wrong in a Huntington's brain and

what might be going wrong in an

Alzheimer's or Parkinson's brain hunting

this is uniquely special because of the

fact that you can genetically predict it

before people have the disease so

Huntington's is is is uniquely

positioned to offer the testing ground

for therapies that could have influence

for all sorts of diseases my brother

Nick has had his brain scanned 2 or 3

times a year for the past five years as

part of the effort to track disease

progression but as I just get the email

Nick will you come and I'm doing it you

just see the words HD scan research I

don't even read them now to apply yes

what dates today was an MRI for 90

minutes and then a PET scan as well in

the afternoon so and then so and then

they wanted to do more tests so we did

more we did more cognition tests the

track HD scans and researchers and

researching and checkups was all about

trying to see changes in the brain that

they think are happening 10 or 15 years

before you become outwardly symptomatic

there has been profound progress in

terms of understanding mechanisms we

understand what are some of the causes

of the neuronal loss the neuronal injury

that occurs in

disease we use a fruit fly model we can

insert the human gene into the fruit fly

and this allows us to look at genetic

modifiers so we can understand how these

genes might be normally trying to

protect the cells from death and then

hopefully if we can find these genes

that are helpful we might be able to

boost them for example this is a concept

that I call environment medics these

would be drugs which mimic or enhance

the beneficial effects of this

environmental stimulation we have

generated a transgenic sheep model of

Huntington disease we're now looking to

see if we can inject into the brain and

antisense or an interference paradigm

which could turn the gene down or turn

that gene off

I trust the science you know it's not a

simple process and it takes time so you

know that's the thing that people don't

really want to hear every step forward

seems to be them discovering that HTT is

far more complicated than they thought

yes if you summarize the last four or

five years that's pretty much been it

we're on the brink of a of a major sea

change in this fall sorts of reasons the

best stuff that's gonna happen which is

going to which is really gonna bring

people out of the shadows is the kind of

critical chance they're gonna say okay

if it's actually something out there

that can make a difference then I've got

it sooner rather than later one of these

clinical trials will show a intervention

that significantly slows down this

disease and hopefully eventually stops

the disease in its tracks this is a

pretty nasty disease that's complicated

and I think what's quite you're gonna

see is that there's gonna be a lot of

drugs for Huntington's disease and so

you might have a drug that you know is

for the brain and you might have a drug

for the muscle deterioration and you may

have a drug

for depression you'll be treated when

you are still well but not yet ill but

still carry the mutation and others

you'd be so-called pre-symptomatic I've

always said that HD is the most curable

incurable brain disease I would say that

if there is ever a time to be hopeful

for the future this is it I think there

is a great deal of hope there's a lot of

work being done but it's unlikely to be

fast science takes a long time I think

people with Huntington's are coping

really well and I think we do really

well here with the sort of support and

the and the things that we can offer

them as for me I'm scared to be

optimistic

I watched my mum wait for 20 years for a

treatment and I have no idea where this

leaves my brothers in me so I'm slowly

I've been off running for about four

months back running now just started

about two weeks ago so yeah probably

here the exercise just has to come first

doesn't it so it's the only thing we've

got

[Music]

[Music]

no we're being married 50 years in a

couple of months time sure I threaten no

nice thing see

she went out in the mouth for a medicine

or something well you're off to the rest

time but I don't mean it

[Music]

I can see that dad doesn't want to let

mom go he wants to keep her here he's

not religious so here is the only place

there is so he wants to keep her with

him as long as he can give her a chance

good fantastic

fantastic keep going keep going oh these

are these the start again I live with

the knowledge that my beautiful brothers

will most likely lose their minds and

die slowly trapped an unresponsive

body's unless they choose another way

out and Jeff and my sons will most

likely have to watch me do the same but

despite all this somehow on this journey

I've discovered that I'm stronger than I

thought I've looked the enemy directly

in its yellow eyes and told it to beware

if it wants to take us all on then fine

good luck to it I know for sure it still

hasn't seen the full strength of my

family

[Music]

there's a secret that I would never

whispered never to

[Music]

to secret that is written never touched

by light of B

extremists

gift for you and given

[Music]

well my face is wet today

not tears but oceans will

I am bringing you a gift from

[Music]

you

you