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An Insatiable Hunger: This documentary follows the lives of four children who are living with Prader-Willi Syndrome, a condition known to cause an insatiable appetite.
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00:02Imagine a world where all food is kept under lock and key.
00:07Where people would eat themselves to death if not controlled.
00:12And for whom obesity is written in their genes.
00:16A world where the constant desire for food overwhelms every other sense.
00:22This is the world of Prada-Willi syndrome.
00:25A rare and incurable genetic disorder best known for its insatiable appetite.
00:31People with the condition stare down the barrel of morbid obesity and an early death.
00:39So why is the only drug which can help being withheld from the vast majority of the 150 New Zealanders
00:45with Prada-Willi?
00:48Four families from around New Zealand are allowing us to follow their lives over a period of a year.
00:57These are ordinary people under extraordinary pressure from an insatiable hunger.
01:13Food?
01:15No, I didn't say food neither.
01:18Eleven-year-old Northland boy Darcy Harris can't stop thinking about food.
01:23Ice what?
01:23Ice cream?
01:24No, I didn't even say ice cream.
01:26Yeah, stuff it out?
01:27No.
01:28Darcy was born with Prada-Willi syndrome or PWS.
01:31You're not listening.
01:33The condition has many effects, but the most obvious is that he is always hungry.
01:38His mother Kathleen must watch him like a hawk.
01:42Get out of the fridge, I can see you in the fridge.
01:44Leave that here, leave it.
01:46Get out or you'll go to bed with no tea.
01:49Do you think you do bad things?
01:52What do you do?
01:56Steal food.
01:58Pardon?
01:58Steal food.
01:59Steal food.
02:02Darcy's desire to obtain food at any cost means all of the Harris family's food is
02:08kept under lock and key.
02:11When they forget, Darcy will always manage to sneak food.
02:19There have been several times where Darcy's been into the freezer, helped himself to a
02:25whole two litre of ice cream, raw mints, raw sausages.
02:29He'll just eat himself to death.
02:31A little race Darcy, just slow down.
02:34New Zealand doctors first began diagnosing Prada-Willi syndrome in the late 1980s when genetic
02:40testing became possible.
02:41The disorder affects just one in 20,000 people.
02:46As you can see this is her length.
02:49Auckland specialist Dr Paul Hoffman is a leading authority on PWS.
02:55These are children and teenagers and adults who always feel hungry and if you can imagine
03:01always feeling hungry and never feeling full.
03:03you have an overwhelming desire to eat food, you have this insatiable appetite which doesn't
03:10go away.
03:12Deep within our brain there is a small gland with an important job.
03:17The hypothalamus controls body functions like appetite and temperature.
03:22And it is faulty in people with PWS.
03:25Instead of releasing hormones telling Darcy he's full, it always tells him he's starving,
03:31no matter how much he eats.
03:33They have no ability to stop eating.
03:37It's not their fault.
03:38It's not something they can control.
03:39Lady, his hand's quicker than the eye.
03:43If you're not watching your aid feed, you can guarantee you would have stolen it right
03:47from under you.
03:49When you feel hungry, what does your body feel like?
03:52What's your tummy telling you?
03:55Start eating.
03:56Pardon?
03:57Start eating.
04:00You need to eat all the time.
04:05All the time.
04:09So what's your tummy doing now then?
04:13Waiting.
04:14It's waiting.
04:17This will be a big year for Darcy.
04:19He's struggling to cope at school, and the extra burden he places on his teachers could
04:25mean his days at mainstream schooling are numbered.
04:3222-year-old Francie Thornton has PWS.
04:36She's determined to be independent, and this part-time job is an important step along the
04:41way.
04:42But even here, she's accompanied by a caregiver who helps her control her compulsion to eat.
04:49Francie lives in sheltered housing, and is supervised 24 hours a day by our roster of caregivers.
04:57I'm quite an independent.
04:59I can do a lot of things on my own.
05:02Do housework on my own.
05:0590% of the time I'm HP.
05:07The percentage of that is I am sometimes safe.
05:14But yeah, that's life.
05:18Come along Santa.
05:20Christmas marks an important milestone for Francie.
05:24She's determined to make the year ahead one in which she loses weight.
05:28If she can keep away from temptation.
05:31We all raise our glasses to Francie.
05:34Francie!
05:36Who is in the kitchen?
05:39If Francie finds food here or at her own place that she's not allowed to have, that
05:48isn't part of her diet.
05:49She finds it and eats it.
05:51Whose fault is that?
05:53It's not her fault.
05:54Hanging around the kitchen.
05:55And cutting up the rest of the meat for the dog.
05:59Francie's father Nick supports her desire to live an independent life, despite the weight
06:04gain which has accompanied the new freedom.
06:06It has worried me, but at the end of the day we're more happy that she is a happier person.
06:13So having a few kilos on, if that's the price you pay for her being happy rather than forcing
06:20her to be thin, then we accept that for what it is.
06:24I want to stop.
06:25But without careful management, people with PWS become morbidly obese and risk an early
06:31death.
06:32Francie must make big changes in the year ahead.
06:35If she's to have a chance at long life.
06:38A new me.
06:39We're going to get rid of the old you and have a new one.
06:42Yeah.
06:43How are we going to do that?
06:45I want to get down to about your size.
06:50Yeah.
06:50Your eyes.
06:51Yeah.
06:53People with PWS spend their lives fighting weight gain.
06:58But in a cruel irony, they are born incredibly small and struggle to thrive.
07:04When Francie was born in 1984, the condition was not well known in New Zealand.
07:10Linda only found out about PWS from an article in a 1987 Australian Woman's Weekly.
07:16So I took the magazine along to the professor of paediatrics in Wellington at the time, and
07:25he looked at me as though I was the one that needed diagnosing.
07:29In fact, I got a diagnosis from him as an overanxious mother.
07:33What have we got cooking in the pot?
07:35Um, eggs.
07:37Nowadays, Prada-Willi syndrome is picked up much earlier.
07:40Even before birth, there can be signs that all is not well.
07:44No, get out of the fridge.
07:46Definitely different from the previous pregnancies.
07:49He was growing okay, but didn't notice a hell of a lot of movement during pregnancy.
07:55He was born in Anganay Hospital by C-section.
07:59So it was quite a shock to see a very small three-pound baby.
08:04A very lump.
08:05He wasn't moving at all.
08:07I couldn't bond with him.
08:09He just didn't feel like mine at all.
08:12Baby Darcy's limpness, known as hypertonia or low muscle tone,
08:17is common to children born with PWS.
08:21The defining characteristics of Prada-Willi syndrome in the first two years
08:25are really those of a child who has low tone, who feeds poorly,
08:31generally fails to thrive.
08:33They do not achieve the developmental milestones.
08:36They sit late, they walk late, they talk late.
08:42I thought that there was some drug or some operation that could fix things, you know.
08:48Oh, it's all right, we can fix this, you know, but there isn't.
08:53What are you doing in there? Get out of the fridge.
08:55Although most physical signs of the condition are obvious from birth,
08:59the insatiable appetite which defines PWS doesn't appear until after year three.
09:05Get out of the fridge.
09:06For Darcy, the hunger arrived on his fourth birthday.
09:10He was crawling everywhere quite slowly,
09:14and all of a sudden he just stood up and walked to the table
09:17and helped himself to the table.
09:20Maybe.
09:21Even though he was this small, skinny child,
09:24from that day on you can guarantee he's started to put on weight.
09:29They'll eat a range of foods that you and I would consider abhorrent.
09:33Butter, sometimes with the paper on,
09:36will include foraging and rubbish bins.
09:37It just reflects the degree of hunger that they have.
09:47But hunger is only half of the problem.
09:51Despite Darcy's strict diet, at 65 kilos he is much heavier than most children his height.
09:59This is because his metabolism is much slower than that of other children.
10:04It's just another challenge placed in his way by Prada-Willi syndrome.
10:09Though compelled to eat more than his friends,
10:12Darcy burns calories at a much slower rate.
10:15Our metabolic rate, the way we burn energy when we're just resting there,
10:19is to a large degree determined by the amount of muscle we have.
10:23So if we have less muscle, we actually utilise or burn less energy.
10:28So these kids not only are eating more, they're actually burning less,
10:32and so they're gaining even more weight.
10:35Darcy's situation may seem hopeless, but there is a light at the end of the tunnel,
10:40a drug which could counteract many of his problems.
10:43So why is this potentially life-saving treatment being withheld from Darcy?
10:56Amelia Reid has Prada-Willi syndrome, a rare and incurable genetic disorder.
11:02At 18 months, she hasn't yet developed the insatiable hunger associated with the syndrome.
11:10She's much smaller than other children her age and is struggling to grow at all.
11:15Amelia's favourite place in this world is here at daycare.
11:19She just loves watching the kids.
11:22She's often unable to play with them like a normal child,
11:27but the caregivers here are great.
11:29They actually take her to where the action's happening and play with her,
11:34and she's never a dull moment for her here.
11:38Amelia was born at the same time as Latham, and she should be close to his size.
11:44Her muscle and bone growth have been held back by her condition.
11:49While the other toddlers run and play, Amelia needs leg braces and support simply to stand.
11:55We've had some people say that we're lucky that she's so easy-going,
11:59but just the stress of knowing what the future holds, or potentially holds for Amelia,
12:07scares the living bejeebus out of us.
12:10In six months' time, the Reid family will receive news that will determine Amelia's future.
12:16For now, they must wait, and take heart from the progress that another child with PWS is making.
12:24When he's playing, it's hard to believe that nearly three-year-old Cameron has Prada-Willi syndrome.
12:30But watch closely, and you can spot the signs.
12:36There is something different in the way he speaks.
12:39Mummy, I'll jump high.
12:41You jump high, you're doing great.
12:43And he is less coordinated than other kids his age.
12:47Oh, crash!
12:48But as his mother Karen watches from the sidelines,
12:51Cameron fights against the limitations he was born with.
12:55Oh, did you fall over?
12:56Oh, crikey, Cameron.
13:00Cameron is lacking the reflexes to save himself if he's going to fall,
13:05so he won't put his hands down.
13:06He loses his balance.
13:08So he becomes a cropper quite regularly,
13:11and he falls over and often just lands flat on the ground.
13:22Did you bang your head?
13:24Did you?
13:25We try not to wrap Cameron in cotton wool,
13:28because we think that if we do,
13:29we are going to deprive him of a lot of experiences that he needs,
13:33and the stimulation and the fun.
13:35You're okay.
13:36But it's taken more than just fierce determination
13:38to get Cameron growing to near-normal physical development.
13:44Cameron is part of a new generation of PWS kids.
13:48He's one of the few New Zealanders with Prada Willy
13:51receiving growth hormone therapy.
13:57Regular growth hormone injections help his body grow stronger
14:01and his bones grow larger.
14:04It's not a cure, but it's a significant boost.
14:09Kick, kick, kick.
14:11After ten months of treatment,
14:13Cameron's almost three-year-old muscles have grown,
14:16and he's now able to do many of the things other kids take for granted.
14:21These children often just transform.
14:24They get up and walk.
14:26Their parents describe quite dramatic changes in the quality of their lives.
14:31It doesn't change other aspects of the PWS.
14:35Unfortunately, it doesn't change the cognitive problems they have,
14:39but it makes a big difference to these children
14:42and a big difference to their lives.
14:43It makes them leaner.
14:44It makes them healthier.
14:46But all miracles come at a cost,
14:49and growth hormone has an expensive price tag.
14:52A full course for a child of Cameron's size
14:55costs well over $1,000 per month.
15:00Cameron's parents are paying a high price
15:03to give their son a chance at a normal life.
15:06We're privately funding for Cameron.
15:08At the moment, he's on half a dose.
15:11And as you can see, he's just got truckloads of energy.
15:15So even half a dose is a hundred times better than no dose.
15:20Because before he was on the growth hormone,
15:23he was just basically limp, really.
15:26He just didn't have any strength at all.
15:27It's expensive, but absolutely worth everything.
15:31In New Zealand, growth hormone has only been a prescribed treatment
15:35for PWS since 2006.
15:39Government drug purchasing agency, Pharmac,
15:42has extremely tight criteria for funding the treatment.
15:47Pharmac will only consider funding for PWS children
15:50after they turn two,
15:51and only if they fall well under normal growth measures.
15:58In six months' time, Amelia will turn two
16:01and be assessed for funded treatment.
16:04Her physical development has been closely monitored since birth,
16:07and every trip to the hospital is full of tension.
16:13Amelia's grown just 17 centimetres since she was born
16:16and is currently well within the Pharmac criteria for funding.
16:20She's actually getting further away from the normal height range.
16:24It's a cruel irony that Amelia must now stay small
16:27in order to receive the life-changing treatment.
16:30Before our financial security,
16:32we're hoping that she doesn't grow and flourish for now
16:35until she's on the drug.
16:38So we feel, or I feel really guilty
16:41about hoping that she doesn't grow big and strong at all for now.
16:46At least Amelia has hope of someday getting the miracle drug.
16:52Growth hormone therapy was not available when Francie Thornton was growing up,
16:56and under Pharmac criteria, the treatment is only funded
17:00until a girl reaches the physical maturity of an average 12-year-old.
17:05Boys qualify until they physically mature to the age of an average 14-year-old.
17:11This opens the door for 11-year-old Darcie Harris.
17:16Overseas tests have shown the treatment promises profound improvement for a boy of his age.
17:22But Darcie's too tall to qualify for funding under the current Pharmac criteria.
17:29Darcie's parents are unable to afford the crippling cost of up to $40,000 per year
17:34for treatment for a boy of his size.
17:38I might need to get a good grip on what the hell are they doing.
17:43For me, this is my kid's life. This is Darcie's life.
17:47Give it to him.
17:50It's going to help him in so many ways.
17:55Darcie wants nothing more than to fit in,
17:57but he's constantly frustrated by his condition.
18:01And in the months ahead, he will boil over,
18:04leaving the school with a difficult decision.
18:14Northland boy Darcie Harris just wants to be like any other 11-year-old.
18:19But the challenges of Prada-Willi Syndrome
18:22make Darcie's everyday life quite unlike that of other kids his age.
18:27For a start, Darcie is never alone.
18:31Without our cameras with him,
18:33Darcie wouldn't be trusted to walk to school unaccompanied
18:36because his body is constantly telling him to hunt out food.
18:44Because PWS also affects intellectual development,
18:48Darcie reads at several years below his age
18:50and prefers to play with younger children.
18:53His emotional development is also affected.
18:59Hikurangi School has taken special steps to cope with Darcie's unique needs.
19:04All food, including other children's lunches, is locked away.
19:08This helps to foil Darcie's natural urge to forage.
19:11The boys have been told time and time again to lock their food in that cupboard.
19:16But there's times where the boys just can't be bothered.
19:19And he goes through their bags to see who the lazy ones are and who will eat their lunch.
19:26I've seen him sitting out here when there's been an apple core sitting in the garden.
19:31And he'll sit there like this and if it's behind him he'll see it.
19:35And then he'll just slowly, without anybody sort of noticing, reach down, pull it up and then carry on eating
19:42it like it was his apple.
19:47To help deal with Darcie's hunger, the school has two 40-minute lunch breaks per day.
19:53But even a three-hour wait for food can be too long.
20:00It must be hard for him to be in here and to know there's food just locked in that cupboard
20:07and trying to focus on doing things like spelling and reading and writing.
20:15Desperate to fit in with normal classroom activities,
20:18Darcie tries to avoid the special attention his condition often calls for.
20:24And though he tries to be the best boy in room 11, he often slips up.
20:32He has frequent outbursts and temper tantrums when his desire for food is not met.
20:39Or his routines are disturbed.
20:42Get up and put your stuff away.
20:45Put your stuff away, Darcie.
20:55He has been known to bite, swear and hit teachers and other children.
21:01Fuck you.
21:04That's naughty. Don't do that.
21:06Darcie, don't.
21:07Darcie!
21:08But Darcie's behaviour, like his appetite, is something he cannot fully control.
21:14The same complex brain structure known as the hypothalamus, which controls appetite,
21:20is also responsible for regulating our behaviour.
21:23In adolescence, the major issues for Padawili syndrome revolve around personality problems.
21:32They start in childhood, but they become progressively more stubborn.
21:36Temper tantrums and anger, mood alterations, depression, are also a major feature.
21:44Darcie's growing physicality makes him a handful for school staff.
21:49He is capable of throwing the chairs around the classroom.
21:52He will upend tables.
21:55He will verbally abuse features.
21:59We have occasionally had to restrain him physically and wait until he calms down, which can be a while.
22:10To help manage this behaviour, Darcie has been given his very own teacher aide, Fred.
22:15Darcie, Darcie Smith?
22:18He likes his routines.
22:19If there's any changes, you need to let him know beforehand.
22:22It's like reading a book.
22:26If you've read a book continuously and you know how the story goes,
22:30and then somebody comes along and rips out the middle of a page,
22:32well, it just throws the whole book all out of whack.
22:35So if you do that with Darcie, it's going to throw his whole day all out of whack.
22:39You know, the consequences can be not good.
22:48The two have formed a close bond that helps Darcie get through the long school day.
22:54Try it again. Don't worry about that.
22:56With his hunger, it's always there all the time.
22:59When you come in in the morning, the first question he asks you is,
23:02what did you have for tea last night?
23:04Food is always on his mind.
23:06But if you keep him busy and give him an activity
23:09so that you can actually take his thinking away from food, he's all good.
23:13Good shot!
23:17The transition from adolescence to independence is one which those with Prada Willy never fully make.
23:25Francie Thornton lives in her own flat, but she is never alone.
23:29Her compulsion to seek food means she must live under constant supervision.
23:34Is that ginger beer you're putting in there, Francie?
23:37Yeah.
23:37It'll taste like ginger beer.
23:40It'll probably taste like ginger.
23:42She is on a strict 1,000 calorie daily diet, around half of what most people eat,
23:48and her caregivers keep all food under lock and key.
23:53She must also keep to a regular fitness plan,
23:56but carrying extra weight makes most exercise difficult and uncomfortable.
24:04I love swimming because you're in the water and the water's holding up your weight basically.
24:10Like, you're floating and it's really nice. I mean, I find it quite easy.
24:16But swimming also exposes her body and feelings to public ridicule.
24:22One day I was at the pool and these kids told me that I was a big 12.
24:28That's a real put off for me because I know I'm big and I don't need to be told that
24:34I'm as big as a whale.
24:37I try and do 40 lengths each time I go swimming and that is one whole kilometre.
24:45Swimming for 60 minutes will burn around 250 calories, or a quarter of her daily food intake.
24:51But while our cameras were with Francie, she managed just 10 minutes of sustained activity.
25:02For Francie, losing weight means much more than just looking better.
25:07Heart disease, diabetes, high blood pressure and cholesterol are just some of the illnesses related to obesity.
25:15And despite her strict diet and exercise regime, Francie still struggles with her weight.
25:21Hilary, has the weight gone up since last time?
25:24Have a look here. Are you worried about that?
25:26Yeah.
25:26Yes, it has.
25:28It was 1-1-5.
25:301-23 now.
25:35I'm not expecting anything.
25:37What's going wrong?
25:39Oh, Francie, this is bad.
25:41I know.
25:41It is bad.
25:42What's going wrong?
25:42Very bad.
25:43What's going wrong?
25:44I've got no idea.
25:46No idea what's over there.
25:48The computer says you're morbidly obese.
25:51Has anybody been giving you extra food?
25:53No.
25:55No, honestly.
25:56And where do I bump into you, Francie?
25:59In town.
26:00Yeah, we're in town.
26:02I've seen you once at the supermarket.
26:04Here we go.
26:07Francie's weight gain keeps me awake at night.
26:09You know, you wake up in the middle of the night and you think,
26:12well, where is this food coming from?
26:14How is this weight gain?
26:16Where is she slipping through the net?
26:19I just say, me is me and I've got to cope with it.
26:27And mum's got to sort of accept the way how I am too.
26:35Four months later, Francie comes clean and admits she's been buying snacks.
26:41Now she's being moved into a new house, away from the lure of the corner dairy.
26:46Yeah.
26:47It was temptation.
26:49And on a hot day I would sometimes go down and get a nice spot for lollies.
26:55And lollies are not good.
26:57Things went wrong, but I'm happy that I've moved.
27:03And because it's going to be a new start to everything.
27:07It may be a new start, but it's still the old Francie trying to make it.
27:12There is a petrol station down in the corner.
27:15But there isn't really anything that would tempt me to buy.
27:20Apart from my favourite, which is green leaves.
27:24But they're not the most fattening thing you could buy.
27:27Because they're baked, they're not fried.
27:31There are still no appetite issues for almost three-year-old Cameron O'Reilly.
27:36With the help of growth hormone, his physical development is coming on in leaps and bounds.
27:41But before he is five, Cameron is expected to develop the insatiable appetite and behavioural problems typical of PWS.
27:50I don't want to hold it.
27:51His father, Rody, is determined that Cameron won't be defined by the condition.
27:57When he was born, one of the things they said about children with Prada Willie is that they can't climb.
28:02And we've got a climbing frame out the back.
28:05And he's up there doing his best, climbing right to the top.
28:08And that's inspiring, and that's humbling, and that's just fantastic.
28:13Happy birthday to you.
28:17Cameron's third birthday party brings mixed emotions to the family.
28:22It may be the last time he's allowed free access to sweets and party food.
28:28While the growth hormone therapy helps with many of the physical conditions of the syndrome,
28:32it will not fix the part of Cameron's brain which tells him his body is starving.
28:38In the back of our minds all the time there's a real sense of anxiety about when the eating disorder
28:44will kick in.
28:45We try to tell ourselves daily that it won't kick in.
28:49But it's one of our greatest anxieties and fears is that he'll just start to want to eat and eat
28:56and eat and eat,
28:56and we'll watch our beautiful son be destroyed by this disorder.
29:01Halfway through the year, and there have already been huge challenges for all our families.
29:07But the changes ahead will push some to their limits.
29:15The year has brought big changes for 11-year-old Darcy Harris at school and at home.
29:21He's had a few things happen this year, but just recently it's been his grandfather has been in hospital quite
29:29sick.
29:29And it's obviously taken quite a toll on Darcy.
29:34His stress levels have definitely increased and therefore we've had some behaviour problems, I suppose.
29:46At school, teacher aide Fred is no longer with Darcy.
29:50A Ministry of Education decision to cut back Darcy's teacher aid allowance to just three hours a day has meant
29:57that Fred had to move on.
30:03Darcy is now unsupervised for large chunks of each school day.
30:09I've dropped him off at school, he's taken off through the back field and gone into a shop.
30:15He shoplifted a quantity of food, then went and locked himself in the public toilets and chowed down on as
30:24much as he could.
30:27So, and all of this happened before 9 o'clock in the morning.
30:32He's also taken a razor to school and he's cut his arms and his fingers.
30:38He was probably stressed, not getting his feelings out, me neglecting him because I've been spending so much time at
30:45the hospital.
30:47Sometimes I think that maybe I'm not giving him enough attention at the time.
30:56I don't have problems with him when he has a teacher aide in here.
31:01It's when he doesn't have somebody with him that's when problems occur because I can't keep an eye on him
31:07all the time.
31:09And he's very clever at sussing up the situation in the classroom and then thinking,
31:16OK, I can get away with this, I can get away with this and he'll disappear from classroom.
31:21And when he finds the opportunity to stray, it's no surprise when Darcy's PWS draws him directly to food.
31:30We had an incident not so long ago in the hall kitchen and we don't know how long he may
31:35have been doing this,
31:36but our hall kitchen's always been open and it was just something that we didn't think of.
31:41One day the boys just happened to be walking past the kitchen and saw him in the fridge
31:46and there's stacks of food in there and he had raw sausages in his hand
31:51and he knew he was in trouble so he just went straight to the toilet, locked himself in the toilet.
31:56and was in the toilets munching down on him.
32:01And that presents a whole new problem of how do you get him out of the toilets which he's locked
32:07and how do you get them off him then and the whole emotional problems for him.
32:15Darcy struggles to cope with changes in routine and the stress that he's feeling is making things worse.
32:22Today he has an air infection, which means he can't put his head under water, but he's having trouble understanding
32:29why.
32:30Mum said that you can go for a swim, but you can't put your head under the water.
32:34But you can still swim along the side, alright? Alright?
32:38Once he gets upset, he can't just stop and think about what's happening and then defuse it.
32:46Most of the time he has to actually go through the whole full-on tantrum before he settles down.
32:54Darcy, if you don't tell me you're not going to put your head under water, I won't be able to
32:57let you go for a swim.
32:58I want to wet my...
33:01You want to wet your head? You can't!
33:05When I first took on this position, I thought, yeah, we can do this.
33:09You know, mainstream's for him. We can work through it.
33:12But I've just seen all the things he's had to miss out on.
33:16I can honestly say there's not a heck of a lot of learning going on without his teacher aide there.
33:22If he had somebody full-time, then maybe it'll be different.
33:27Ms Tito's whole class is waiting to swim, and she needs to supervise them, leaving Darcy out in the cold.
33:36The cavalry arrives in the form of new teacher aide, Joe, whose three hours' work today will begin by defusing
33:44yet another of Darcy's tantrums.
33:47When Fred was here as his teacher aide, there was a lot of violence. He would hit and kick and
33:54that sort of thing.
33:55He's been physical with the new teacher aide a couple of times, and that's the tricky thing about him being
34:01in the mainstream school as well,
34:03is seeing him do things that other students would be asked to leave the school or we'd have a meeting
34:09with parents.
34:10Then why has the Ministry of Education insisted on cutting back on Darcy's teacher aide hours?
34:16Let's go, let's go, let's go.
34:19There seems to be a conception that as he gets older, the level of support can be lessened because there's
34:29this belief that he becomes more higher functioning as a result of his being at school.
34:35Not true.
34:37Not true.
34:37His needs increase, he's going through puberty, he's got hormones to deal with, and he doesn't have the ability to
34:48deal with that.
34:51Every time Darcy has one of these episodes, it presents me with that real hard choice.
34:58Darcy has his behaviour got to the stage where it is now impinging on the ability for other children to
35:07learn and their right to learn within the classroom.
35:12At last, Darcy gets into the pool for the final five minutes of the class swim, taking care not to
35:20wet his head.
35:22For a child without Prader-Willi syndrome, any one of Darcy's behaviours would have led me to say that this
35:32child needs to be suspended.
35:36I certainly don't want to suspend him, but if by suspending him we get the support he needs, then maybe
35:48we have to do that.
35:52If Darcy can make it through the school year without being suspended, his battle will not be over, and it
36:00can only be fought by those who love him.
36:02It's just a job and I have to do it, because no one else is going to do it.
36:09No one else is going to fight for Darcy, but me.
36:18There has been a significant victory in the O'Reilly family's struggle with Prader-Willi syndrome.
36:24Pharmac has agreed to fund Cameron for a full course of growth hormone therapy.
36:29Strangely, not under the Prader-Willi criteria, but through a loophole.
36:34As it turned out, he is growth hormone deficient, so Pharmac are now paying for the full treatment, and he'll
36:41have that treatment until he stops growing at around 16 years of age.
36:47For Karen and I, the psychological burden has lifted hugely, because funding it for the rest of his life was
36:53just going to cripple us financially, which had a huge impact on just how we looked at our future really.
36:58And just having this funded for the next 13 years has given us a chance to breathe and to have
37:04hope and joy, and it's just lifted a huge burden off us.
37:08Now receiving a full dose of the miracle drug, Cameron is going from strength to strength.
37:14It seems that every day, every week, every month, he surprises us with just how well he's doing.
37:19It's really awesome, actually, because we used to feel quite hopeless about his future.
37:24Now he brings us a huge amount of joy that we didn't really think he would.
37:27I can do it.
37:28Yeah, hop up the ladder and try again.
37:30We were told he'd never be independent from us.
37:33You know, who says he won't be independent from us, and who says he can't grow up and go to
37:39university and go flatting or whatever he wants?
37:45Kids overseas are doing it, so he can.
37:49Be a plumber, they do quite well.
37:51Plumbers, yeah.
37:52Can you have one soon? Have one soon, okay?
37:55Growth hormone will help Cameron build a strong, lean body, but it won't have any effect on the insatiable appetite
38:02that is expected to develop any day.
38:05Well, of course, every time we find him in the cupboard, I get paranoid that he's started food-seeking, but
38:11in fact Jasmine's normally with him, and they're really just making a mess.
38:16But it is the monkey that sits on our shoulder, isn't it?
38:19It's a cloud that we try and keep at bay, really, and we hope beyond hope that it won't actually
38:25be in Cameron's future.
38:30For now, anything seems possible.
38:33While growth hormone has no direct effect on Cameron's intellectual development, he's recently been assessed as reading at a level
38:40higher than his age.
38:42So that's really exciting for us because it indicates that even though his speech quality is not great, everything inside
38:49his head seems to be going on normally.
38:53And, yeah, so I think the sky's the limit if the brain is functioning properly.
38:59When he gets his mind set on something, he really just sticks to it, and he's determined, and that's just
39:07fantastic because certainly determination is what makes a difference in life.
39:13And he's full of determination, and when he gets an idea in his mind, he won't let it go.
39:18And that's just more than we could have ever hoped for, really.
39:22Eh, Camo?
39:23Yeah.
39:25Not everyone's year has been as good.
39:28OK, it's got nothing to do with you, and I'm asking you nicely.
39:33Francie has strayed into dangerous territory.
39:38I don't want to go then. No, I'm not talking about that.
39:44Francie started the year determined to lose weight. Just how successful she's been is about to become clear.
39:52Cool. That's great, Francie. You've lost a kilo. You were 124 and now you're 123.
39:59OK?
39:59At 123kg, Francie's weight is the same as it was 12 months ago, despite plenty of regular exercise and supposed
40:08strict dieting.
40:10Francie is difficult to cope with in lots of ways.
40:16It's harder than anybody else to be in calories, and it's hard to stop eating.
40:22I just got to concentrate and getting on top of the exercising and make sure that I do exercise every
40:27day.
40:29She worked really, really, really hard. She did a lot of hard-out work, but she thought because she was
40:36exercising,
40:38she could therefore eat more. You know, the weight did go on.
40:42So what does it leave us after a page? Mum.
40:45But food is not Francie's only addiction now. Her compulsion to have something in her mouth has meant a major
40:52development in her lifestyle.
40:54Smelly things. They stop them.
40:572nd of January, we've got them up till.
41:00OK.
41:01Just like food, Francie's cigarettes are tightly controlled, just three a day, which is more than Linda would like.
41:09Far be it from me to say that the people who work with her can't smoke or aren't allowed to
41:15smoke.
41:15Many staff do smoke, and Francie sees this as part and parcel of being an adult and growing up.
41:22So why can't I smoke as well? It just got beyond me, actually.
41:26This year, things piled up and piled up, and there are some things you just have to let go, and
41:32that cigarette smoking was one of them.
41:35Each time I light a cigarette, it's like dropping a dollar coin down the drain.
41:39But it does stop me from wanting to eat more.
41:43It keeps me calm. It keeps me in a good mood.
41:49And next year, I definitely want to give it up, because it's cutting down on my bills.
41:54It's a disappointment, but all things considered, I don't suppose lung cancer's going to kill her.
42:03OK, it's got nothing to do with you, and I'm asking you nicely, please go away.
42:09Breaking long-term habits is something Francie must try to do every day.
42:14But in a new environment, she has found this a struggle.
42:18Her welcome to the neighbourhood was short-lived.
42:21Francie's compulsion to eat at any cost soon drove her to steal from her neighbour.
42:28I don't want to go there. No, I'm not talking about that.
42:32That's just in the past now. That's been dealt with.
42:36So, yeah, I just hope not to get into trouble with the place again.
42:40A year of living independently has had its ups and downs, but Francie is still committed to her long-term
42:46goal to lose weight.
42:49Definitely, I am going to still work on that goal, and hopefully, if I ever see you guys again one
42:59day, I will be down to the size of my mum.
43:04Do you want to put any in there? Do you have some room, do you think, or not?
43:06The whole weight thing aside, she is a much, much happier person.
43:12She feels more independent.
43:15She loves her life.
43:17So Francie, as a person, has really, really matured.
43:22I really, really love her.
43:25So this is a very new trick. She's only been doing it for a few weeks.
43:30The last few months have changed Amelia's life forever.
43:34Soon after her second birthday, she began a full course of Pharmac-funded growth hormone therapy.
43:41Now Derek and Debbie can look forward to seeing their daughter Bloom.
43:46We noticed a difference within 24 hours.
43:50Her energy levels were just huge, just jumped immensely.
43:55She couldn't crawl at that stage, she would just collapse.
43:57She managed to do that, and within 10 days of starting, she was actually crawling.
44:03I'm coming to get you.
44:04It's just wonderful to see her, how she should have been when they should have started the drugs earlier.
44:09So I think that Pharmac have cost us about a year of happiness.
44:14And with other families out there that don't have it, it's longer for them.
44:19In Utopia, where money was no constraint and resources were no constraint,
44:24I think there is a very good argument to be made for all PWS, adults and children,
44:29to be given a trial of growth hormone.
44:33Despite extensive international evidence that growth hormone benefits all people with Prada Willie,
44:40Pharmac funds just 8 out of 22 children of an age eligible to receive it.
44:46If the government is prepared to give children growth hormone therapy just to make them taller,
44:52then they should give it to our kids because it saves their lives.
44:56And for them to say no, Darcy won't be able to have growth hormone, I think that sucks.
45:05Maybe I should send Darcy to their house and they can look after him and see how they like it.
45:10This is his life we're talking about.
45:24I think that we're gonna have awesome people that want to have a great day.
45:26In the third day, one year, they have a great way to grow.
45:30And they've got three days to grow.
45:31They have a great day.
45:31In the third day, two years, they have an incredible future.
45:35And you've got to grow.
45:36And you can grow.
45:39Yeah, yeah, yeah.
46:08Whoo-hoo!
46:10Good boy, Grandpa!
46:13I'm sorry.
46:26Thank you, Fancy.
46:38This program was made with funding from New Zealand on air.
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