Volume 8, Issue 1
The most
important thing
to remember
from the
“Fighting Fit,
Fighting Fat”
workshops is that
energy intake
(what we eat)
should equal
energy output
(exercise).
If we don’t
exercise off the
equivalent
amount we have
eaten, IT WILL
BE STORED AS
FAT.
And that’s
regardless of
what it is we eat.
The body will
store all excess
March 23, 1998
From the Editor
This year is shaping up to be one of our
busiest. We‟ve just had one of the best
Summer Camps ever (see Cindy‟s
report); a wonderful last-minute grant
from the Variety Club allowed us to
underwrite much of the costs; we had a
positive, pro-active Annual General
Meeting; another grant from the T.G.
Macarthy Trust allowed us to bring
Professor Peter Davies over from
Brisbane to run the “Fighting Fit,
Fighting Fat” workshops again - in
Wellington, Nelson, Christchurch and
Dunedin.
Our PWS group home in Auckland has
now been purchased (by Spectrum
Care) and staff recruitment is under
way. We are looking at June for our
opening date. The house is a 3
bedroom place with room for a fourth
bedroom. We will be holding an open
day later this month.
The International PWS Conference is
in May and both Cindy Adams-Vining
and Jo Gibbins have done some serious
fund-raising towards the costs. It is
Energy intake = energy output !!
always a mammoth task for a small
organisation like ours to attend such a
large conference (by PW standards) so
far away and I am truly delighted that
Jo and Cindy have shown such
enthusiasm and initiative in getting
themselves there. They will represent
New Zealand (Cindy will be taking
over from me as NZ Delegate) and also
join forces with Australia in a joint bid
to host the next international PWS
Conference in Sydney in the year 2001.
Wish us luck!
food as fat.
Remember that,
and you’re
halfway to
winning the
battle.
Inside this edition:
Letter to the Editor
Challenging Behaviour Seminar
Genetic Testing for PWS
Life from the Rural Side
Obsessive Compulsive Disorder
Xenical the wonder ‘fat’ drug
Easter at the Kellerman’s
Skin-picking in PWS
p.3
p.4
p.5
p.6
p.6
p.7
p.8
1
The Variety Club Bash
From the Mainland
Memories of Auntie Di
p.10
p.11
p.12
Pickwick Papers
Editor‟s response:
As much as I would like to be able to wave a wand and
create residential homes throughout New Zealand, I‟m
simply unable to. The Prader-Willi Syndrome Association is
primarily parent and family support group, run by parents,
and not seen by the authorities as a „service provider‟. The
only exception to this is North Health with whom we has a
contract to provide residential accommodation in the
Northern region.
(The second home you refer to in your letter, by the way, is
run and owned by the IHC, not the PWS Association.)
This contract with North Health came about by good luck as
well as a little good management.
Two years ago the PWS Association put in submissions to
all RHA‟s asking for specialised PWS residential care to be
recognised and provided.
None of the RHA‟s, except Auckland, even replied.
A this time, North Health was lucky to have been given a
small pocket of extra funding for disability services.
Also at this time we held our Australasian Conference in
Auckland and a couple of the senior executives from the
Disability Support Service attended. They must have liked
what they saw, for shortly afterwards they offered us a
contract to provide residential services in Auckland region.
It has taken two years of negotiations to get to where we are
today. It has taken several visits to canvass all other services
providers in the area before deciding on Spectrum care to
provide the daily management of the home.
We are still a couple of months away from actually opening
the doors.
Unfortunately, it has not happened like this in other regions.
I understand your anxiety to have a home in your own area,
and I would point out that several phone conversations as
well as face-to-face meetings have taken place; letters and
proposals have been put forward to the Southern RHA, all to
no avail. But we have not given up hope and we are still
looking for ways around SRHA‟s refusal to appoint us as a
service provider. However, it all takes so long for the wheels
to turn, and several factors have to all fit into place before the
wheel turns another cog.
It also needs pro-active parents who are prepared to spend
some voluntary time making it all happen. (Needs
Assessments, meetings, talking with other parents, etc)
The greatest sticking point to getting residential care, is
getting the right Needs Assessment done. For some reason,
this can take a long time. Often, people with PWS are seen
as being quite „able‟ and therefore are not a high priority for
residential care. Thus the request for the Accommodation
Supplement can be put on the back burner for several years.
This has happened over and over again, and it would seem that
unless you can prove that your life, lives of your family, or the
person with PWS, are in danger, you are just not likely to be
heard.
Establishing a specialised home for PWS is not simple. First,
find your people. Then get your Needs Assessment done, then
fight the battle to get the Accommodation Supplement; then
find an approved Service Provider. If only it were true, as you
put it, that we are “uniquely placed to take the initiative in
Christchurch”. There are many more groups such as ours who
also have urgent needs and we are not unique in our
requirement for assistance.
All I can do in the meantime, is to assure you that Christchurch
is not forgotten, nor is the rest of the country, and I can also
assure you that your Association is continuously fighting to
keep the matter of residential care before each and every RHA
in the country. Should you wish to see a copy of the Proposal
for Residential Accommodation, this can be obtained from this
office, or from the South Island Coordinator (photocopying +
postage costs). This is a subject near and dear to each of our
hearts and one which we discuss regularly at our Summer
Camp meetings and other meetings throughout the country.
Each and every member of this Association needs to be proactive, both individually and together as a body. By attending
meetings, Summer Camps, AGM‟s and staying in touch,
everyone benefits in the end.
Linda Thornton
Editor
2
Pickwick Papers
TAUTOKO SERVICES
CHALLENGING BEHAVIOUR SEMINAR**
on
14th May 1998
9.30am - 3.30pm
at
Lindale Lodge
Conference Centre
Paraparaumu
Tautoko Services provides a community based service to people with intellectual disabilities
and challenging behaviours. Tautoko Services has been operative successfully across the
Central health Region over the past 18 months. This is an opportunity for you to hear at first
hand about the range of work we have been doing and to participate in discussion around the
issues that people whose behaviour challenges raise for families and services.
What is Challenging Behaviour?
Behaviour which frequently interferes with, restricts or prevents access to everyday routines,
settings, activities and relationships and consequently poses a challenge to providers and
families in developing effective responses, supports or services.
Registration forms can be obtained from: PWSA office or direct from Tautoko Services,
Suite 6, 99 Mana Esplanade, Paremata, Wellington.
Cost: $60.00 (Waged) or $35.00 (unwaged) - morning & afternoon tea, and lunch provided.
Registrations close 1 May. You must register on the appropriate registration form.
3
Pickwick Papers
Genetic Testing for PWS
Although we still do not know what causes PWS, the means
whereby we can test for the syndrome are becoming more and
more scientifically exact.
There are three ways that the deletion on the long arm of
chromosome 15 (giving the characteristics of PWS) can
happen:
1.
a deletion on the paternal chromosome 15
2
a mutation on the paternal chromosome 15
3.
maternal disomy (both chromosome 15s from the
mother and none from the father)
None of the above are the fault of either mother or father.
What has happened is that the forming embryo has failed to
pick up this important piece of genetic material from the
paternal side.
The usual way of testing for this deletion until now, used to be
chromosome analysis which would return a high percentage of
deletions, but not all.
The newer molecular tests can identify the presence of a
deletion (FISH: fluorescence in situ hybridization) or
uniparental disomy 15 (PCR: polymerase chain reaction), in
99% of cases.
Uniparental disomy 15 accounts for about 30% PWS diagnoses
- for the highest degree of accuracy blood samples from the
affected person and both parents are usually required.
Methylation analysis This type of test also clearly determines
whether the father's chromosome material is missing, is
accurate in virtually all cases, and is commonly used in all
States in Australia and is available in New Zealand.
The recommendation by most geneticists for those who have
had chromosome analysis in the past - regardless of results - to
have the newer, more accurate testing done, is suggested both
for management and for family planning purposes. Also since
there is a small chance that some causes can recur in a family,
it is wise to know the type of deletion so that genetic
counselling can be undergone for other members of the family.
In some cases, prenatal diagnosis is available.
RECURRENCE Just what is the risk of recurrence? In
August 1996 The Gathered View reported the findings of Dr
Robert Nicholls and colleagues concerning “rare cases of PWS
(about 2% or less,of all cases) caused by an imprinting
mutation or an apparently intact paternal chromosome 15,
rather than a deletion or uniparental disomy.” Concern
expressed by parents that there might be a transmitted form of
PWS, elicited the following response from Dr Nicholls: “Any
4
family who has their child diagnosed with an imprinting
mutation should seek professional advice from their
genetic counselor and pediatrician, and if these are
unfamiliar with the latest research, they should be
referred to a centre that works with imprinting mutations
and the latest research ...
“With imprinting mutations, about half the patients
(families) have a very small deletion in the region we
refer to as the imprinting centre (IC). The other half of
the patients have no deletion, although this has just been
referred to in passing in the literature. The imprinting
centre regulates the setting of imprints in the male and
female germline, and it controls this for all imprinted
genes in chromosome 15q11-q13. Thus PWS imprinting
mutation patients, with either the tiny deletion or no
deletion, have inactive paternally inherited imprinted
genes. The half of these patients with a tiny deletion
(referred to as a microdeletion) differ from the typical
deletion group because the latter patients have a very
large deletion that includes not only imprinted genes, but
the Angelman syndrome gene and nonimprinted genes.
For those families with an imprinting mutation which has
a tiny deletion, we can offer „at risk‟ testing and when
necessary, prenatal testing”. Dr Nicholls also notes that
some of these mutations are not inherited but “arise as
new mutations in the father‟s germline.” He stresses that
mutations are “no one‟s fault”; their cause is unknown.
Although the statistics for an inherited form of PWS are
very low indeed, and there are only a very few reported
cases, it pays to be aware that with the more and more
advanced scientific analysis and discoveries in the
genetic world, the very unlikely chance of a sibling being
a carrier of PWS is something to consider. The bottom
line for genetic testing is a recommendation to confirm
the diagnosis of PWS through genetic testing and for
genetic counselling to find out whether your family
carries an increased risk of recurrence.
It goes without saying that those in the group where
chromosome analysis returned a negative test result, but
clinically the features and characteristics of PWS are
obviously presented, then if the family of the person
feels that their son/daughter indeed has PWS and that
their needs are best met under the „label‟ of PWS, they
should stay where the services meet the needs. §
Pickwick Papers
Life from
the Rural Side...
February
And we head into the hottest month of the year with Masterton
recording 11 consecutive days with temperatures over 30C.
The apples, having been ruined by hail in November, are now
actually cooking on the branches. Some of them have sunburn
marks so badly they have gone black, like huge blisters. This
is incredible!
Sheep are allowed into the orchard as there is no longer any
grass - what there is is burnt brown and so crisp it turns to dust
under foot. Horse and donkey follow suit - but strictly
supervised and severely time-limited!
Towards the end of February we start strip-picking the Cox‟s
Orange apples for juice. What a heart-breaking job. More to
come.
Since the juice apples can wait a few weeks, we store the bins
in sheds. Due to memory-loss from heat exposure, I forget that
the donkey and horse are lying in wait for this very moment.
Hey! Freshly picked apples, stored in very large bins, just for
us! Can you imagine the sight that greeted me next morning? I
swear they took a bite out of every apple in the top few layers...
there were pieces from here to eternity and great gobs of
satisfied chewings. I hosed the entire place down, picked out
the scraps, hosed the boxes of apples and sent the horse &
donkey waddling out into Barren-land.
During my trip to Nelson for the BOT meeting, I meet up
with Yvonne and visit with Francie their place in the
country! Remarkable similarities with our place, except
they have about a million more chooks than we do. I was
fascinated by the soft grey chooks (Aracanas) that actually
lay green eggs! Well, the shells are green. So desperate
was I to add to my collection, Yvonne offered to bring a
pair up on the ferry when coming to the camp! No greater
friend... Not only that, but she offered to bring Francie up
with her. So, loaded with a box of two chooks, two kids,
and Aunt, Yvonne headed up on the ferry for Kiwi Ranch.
My admiration was boundless.
March
The drought has still not been broken sufficiently to ensure
any autumn growth. The real farmers are becoming very
anxious. To drive over the Rimutaka hills into Wellington
and to go from such a brown landscape into a lush and
verdant green one, is almost too much. A distance of only
half-an-hour from barren to lush. As we head into autumn
and winter, I wonder how we will fare, trying to work with
Mother Nature. The forecasters promise us another disaster
with La Nina....
We‟ll just have to wait and see. At least by then I should
have plenty of green eggs!
A Plea to Watch What we Say
On going back through some old newsletters I came across this article which, once again, repeats the old message - using
appropriate language. It deserves repeating
At the close of the Seattle Conference (July 1995) Hope Mays, parent and executive Director of the Georgia chapter, and
disability advocate, asked to address the PWSA Board of Directors to express her concern about the language many people
use in talking about individuals with Prader-Willi syndrome. She cited instances throughout the conference in which various
speakers, PWSA officials, and others referred to “PWs,” “PW people”, and even “Praders”, or spoke of adults with the
syndrome as “kids” or “youngsters”. The language we choose in speaking about our son/daughter/patient/client with PWS
should focus on the person, not the disability; and the terms we use should be appropriate to the age of the person. It takes
only one more word to say “my son/daughter with PWS” instead of “my PW child”. But what a difference it can make in
how we think about that person! If we are to win respect and appropriate services for people with Prader-Willi syndrome, we
must first show our respect by using proper language. As Dr Elisabeth Dykens, the Yale psychologist, succinctly puts it:
“People are more than their syndromes.” The right language keeps us reminded of that.§
5
Pickwick Papers
Phenomenology of Obsessive-Compulsive Disorder in
Persons with Prader-Willi Syndrome.
OCD.
Third, symptoms were compared across a subset of 43
adults with PWS, matched on age and sex to 43 nonretarded adults with OCD. The PWS and OCD groups
showed comparable levels of symptom severity, including
distress and adaptive impairment. Although the PWS and
OCD groups had more areas of symptom similarity than
difference, the PWS group was more likely to hoard and to
need to tell or ask. The OCD groups showed more religions
obsessions and checking compulsions.
Thus, remarkably high rates of OCD were found in both
children and adults with PWS. Although further work is
needed, these data suggest a possible gene locus for OCD in
the region of chromosome 15 involvement in PWS. These
data also have treatment implications in PWS. As OCD
likely involves a disturbance in serotonin, persons with
PWS may similarly show reduced brain serotonin function.
Serotonin re-uptake inhibitors are thus likely to be of
particular help in reducing these symptoms. Findings also
underscore the need for treatment that goes beyond dietary
management and that targets relations between family stress
and a wide range of compulsive behaviours.§
This article was part of the research presented at the 1995
American PWS Conference’s Scientific Day in Seattle. It was
researched by Elisabeth M. Dykens, PhD, and James F.
Leckman, MD, Yale University Child Study Centre; and
Suzanne B. Cassidy, MD, Case Western Reserve University
and Centre for Human Genetics.
While food preoccupations are a hallmark of PWS, the
disorder features repetitive thoughts and behaviours outside
the food arena. Persistent skin-picking is often seen, as are a
host of recurrent, intrusive thoughts and behaviours consistent
with OCD. Increased risks of OCD are suggested and this
research evaluated this risk in three ways.
First, the range, scope and severity of non-food obsessivecompulsive symptoms were identified in 91 subjects with
PWS, ages 5 to 47 years. Prominent symptoms included
hoarding, ordering and arranging, concerns with symmetry
and exactness, rewriting, excessive grooming, and the need to
tell, show or ask. These symptoms were not correlated with
maternal obsessive-compulsive features, but were related to
increased familial stress.
Second, these symptoms were compared with well-established
clinical criteria for OCD. A full 60 percent of the sample met
criteria for OCD. An additional 35 percent showed key
symptoms of OCD, but did not fully meet diagnostic criteria.
Children were just as likely as adults to be classified as having
Xenical the Wonder “fat”Drug
Some of you may already have heard or read about Xenical
which is being touted as the wonder drug for the cure of
obesity and may want to know how this would affect those
with PWS.
Xenical is already available in Europe and our Health
Ministry‟s approval is expected to be gazetted this month.
What the drug does is to inhibit pancreatic and gastric lipase,
so that instead of the fat being absorbed into the body, up to
one-third of it actually passes straight through the gut.
Sounds good? Sounds wonderful!!
But what is currently not being publicised is the „end result‟
and I‟m not referring to the weight loss. What physically
happens is that the stools become extremely loose, extremely
smelly, and can happen at inappropriate times. Put this
together with trying to treat an already obese person whose
cleanliness habits are physically often impossible, and you
have bought one huge problem.
Some time ago last year, the Association was approached by
the distributors of Xenical wanting to know whether we would
support the use of this drug. I stated that we were not in a
position to do this until trials had been established that it was
particularly suitable for Prader-Willi syndrome. I also think it
is an individual „parent decision‟ or at least a decision that
has to be made together with a doctor who is fully cognisant
with the syndrome. Personally speaking it is not something
that I would try either for myself or my daughter. I would
also be concerned that the idea of being „cured‟ might lead
a person into thinking that no matter how much fatty foods
they consumed, just popping a pill would stop them from
becoming fat. Now, I‟m no dietician, but it would concern
me to think that if this were the case, wouldn‟t the person
actually be starving themselves? If they ate only fatty foods
which were being quickly flushed through the system by
fair means or foul (and in this case, the use of Xenical
would suggest foul), then there would be absolutely no
benefit from the foods eaten.
I would instead prefer to promote the idea of healthy eating,
keeping fatty foods out of the diet, and plenty of exercise.
Exactly the same message we promote through our
“Fighting Fit, Fighting Fat” workshops.
Fight on, friends!
Linda Thornton
6
Pickwick Papers
Easter at the Kellerman’s
(Teresa is the mother of Karie, 23 going on 6, with PraderWilli Syndrome; John, 20 going on 8, with Foetal Alcohol
Syndrome; and Christopher, 17 going on 25, gifted but
otherwise "normal," with no valid excuses for his behavior
problems; and Scarlett O'Hairy, a buff cocker spaniel who has
symptoms of both F.A.S. and PWS.)
Karie and John are eating boiled eggs, Karie's without
the yolks. I'm trying to remind them of their table
manners. "Johnny, when you chew with your mouth
open, it looks pretty gross, so please try to keep your
mouth closed." I turn my back, and Johnny goes
AAAAAAAGGGGGGGHHHHHHHHH!!!!!!" and
laughs uncontrollably. Sigh. The Ritalin hasn't
kicked in yet, so it will take another 20 minutes for
him to "sober up." The people who thought up the
idea of sustained-release Ritalin must have been
parents. Too bad it doesn't work. Thank God the
regular kind does. Nineteen more minutes.
It's Saturday evening, the night before Easter. I just picked up
Karie after stopping for cash at the bank. Karie and I do the
grocery shopping for Easter dinner. Normally I wouldn't take
Karie to the store with me, it's a difficult place for someone
who is always in starvation mode. And a difficult place for
everyone else who happens to be in the store at the same time.
But my car had been in the shop for repairs, and this was the
first chance I had to shop in three days.
Chris, my not-so-typical teenager, asks me for $20... please.
It's his allowance, so I take a twenty out of my purse and hand
it to him. He's a good kid, a teenage brat, but a good kid.
Karie is home for an overnight visit to allow her group home
staff to be home with family. I am coloring 3 dozen eggs, all
by myself, as none of the children want to do it this year.
That's okay with me, I get to color them ALL. When I was
young, I never got to dye more than 3 or 4 each year, being one
of 10 children. I do every egg a different color, no two the
same. Some are rainbow striped. I even do plaid and polkadots eggs. Good therapy. I think about my childhood, how I
was surrounded by brothers, and how it was good preparation
for special needs parenting. Having these three kids is
equivalent to having at least seven "typical" kids. Studies show
that it takes seven adults working 8-hour shifts to effectively
care for a child with Prader-Willi Syndrome... I'm serious.
Having two children that need constant supervision can be a
little draining. I don't know how some adoptive families with
more special needs kids handle the stress. I really don't. I
consider myself lucky that I was able to stop after adopting
two.
Some parents are addicted to kids and couldn't stop taking them
in if they wanted to.
Karie and I share a bed for the night. She sleeps all night
without waking up, but has two nightmares (that she doesn't
remember the next day) and numerous fits of arm and leg
muscle twitching. No wonder she gets so tired during the day
time.
Easter morning. No jelly beans in the house this Easter. (Chris
and I ate them all last week... really.) That's okay, though,
because Johnny would have a reaction to them, not because of
the sugar, but due to the artificial colorings and flavorings.
And Karie is on a special diet because of her disability (only
900 calories a day, poor kid). She is always looking for ways
to get food, or to get money to get food.
7
Easter dinner is nice, even if I practically had to cook
the meal two different ways, since Karie needs 50%
fewer calories and John needs 50% more. We eat
regular ham, but Karie gets 2.5 ounces of Buddig
sliced 98% fat-free ham. It had taken me awhile to
clear all the paper off the dining room table, but it
was worth it, so we could all sit down to eat together.
Made the meal seem almost normal. Almost.
"Johnny, eat with your mouth shut." "Mom! Karie
has her hand in your plate!" "Karie, put that piece of
ham back on my plate." "I was going to give it to
Scarlett, Mom, really."
"Johnny, cover your mouth when you sneeze."
"Mom! Karie's picking her nose!" "Karie, go get a
Kleenex." "Where's the desert?" "We're not having
desert today, honey." "Aawwwwwwwwwwww!"
"Mom! Karie's picking her hand and it‟s bleeding!"
I'm glad we didn't invite anyone over. Chris asks to be
excused. He certainly finished eating quickly.
Karie is sleepy after dinner and goes to lie down in
my bedroom while I clean up the kitchen, which is
right next to my bedroom, so I can listen for trouble.
What kind of trouble? You never know with Karie. I
was careful to have removed any food from my room,
had hidden my stash of chocolate bars in Chris's
room, and had all edibles locked up. (Yes, I have a
lock on the pantry and an alarm on the refrigerator.) I
check on her after about 10 minutes, and see her
rustling under the comforter. I had forgotten that my
purse was in my room, and she had gotten into my
wallet. I do a body search and find two twenties
hidden someplace only female prisoners would think
to hide something. She also had stuffed a couple of
twenties into the battery compartment of the
mechanical frog toy that she bought with her. That
frog's "body cavity" hiding place must have given her
the idea for the other hiding place. (contd over)
Maybe she got the idea from her favorite doll, Hungry
Helen. This is a beautiful little baby doll, with "real"
hair and eyes that blink, and the doll eats little plastic
Pickwick Papers
Skin Picking in Prader-Willi Syndrome
I was recently asked whether I had any advice for care-givers
about skin-picking. The short answer is that I know nothing that
will ‘cure’ it. But I promised to look up some old articles. This
one, by Jeanne Hanchett, MD., of the Rehabilitation Institute of
Pittsburgh describes skin picking and other forms of self-trauma,
but has been edited to include only skin-picking. The previous
article on Obsessive Compulsive Disorder points to skin-picking
as possibly being an OCD. - Ed.
About 80 percent of persons with PWS pick their skin. This
behaviour is one of the hallmarks of this syndrome and is helpful
in establishing the diagnosis. In most persons this is mild and
intermittent, occurring when there is a minor skin lesion such as a
mosquito bite or slight abrasion. However, some have severe and
persistent skin picking which goes on for years.
There has been no uniformly helpful treatment. here at TRI we
have had some success with using Play-Doh or Silly Putty to
“keep the fingers busy”. This works for short periods of time, ie. a
few weeks or a few months, but does not cause complete
resolution of picking. It works best in persons who are aware of
their skin picking, those who admit that they pick their skin and
ask if there is something they can do to keep themselves from
picking. We sometimes offer a choice of Play-Doh or Silly Putty
and even offer a choice of colours and suggest that they
keep it with them at all times, particularly in bed at night.
If there is a concern that small pieces of this may drop off
and stain furniture or carpeting, it can be enclosed in a
small plastic bag which seals. [a Koosh ball might also
have the same effect. Ed.]
We have tried Periactin, Prozac, and topical skin creams,
none of which has been helpful. One thing which we have
found definitely not to be helpful is to tell the person that
s/he should not pick or to say “stop picking your skin.”
In some cases this even results in more picking. The less
attention the better. We do, however, suggest that hands
be washed whenever we see blood on fingers or hands.
In summary, there is no uniformly successful way to deal
with self-traumatising behaviour in PWS. Too much
attention on the part of parents seems to be counterproductive. A matter-of-fact approach of keeping one‟s
hands clean seems to work the best for skin picking.§
Easter at the Kellerman’s ... contd
French fries when you stick them in her mouth. (They go into her
body cavity, which has a little door on the back for French fry
retrieval.) After eating each French fry, Hungry Helen says,
"Mmm, these French fries taste delicious." Seems like torture to
me, for one who can never eat French fries, but Karie loves her
doll, loves feeding her those French fries. A case of living
vicariously through another, I guess.
Before bedtime, I take Karie back to her group home, do another
body search there, and find a post card order form (in her panties)
that she had filled out to order panty hose. I have no idea what
she wants to do with panty hose, I can't even get her to wear a
dress. She says the postcard HAS to be mailed TODAY! She is
frantic that it might not get in the mail in time. I look at the
postcard and ask her where it says it has to be mailed by today,
thinking that Easter would be a strange date for a mailing
deadline. She points to the words "Mail Today." Sigh.
I'm exhausted. When I finally get to bed, I sleep fitfully, having
nightmares of not having enough time to get everything done, and
having to be in three places at the same time. I wake up to realize
I have only two days to do three sets of taxes. And then I only
have two days to get everything ready for the annual PWSAA
meeting. I think about the card I got that said "May all your
8
dreams come true." I've gotta start having different
dreams.
At 8:30 a.m., I get a call from Karie's day program that she
had in her possession an envelope containing three
twenties. She was going to send away for "sea monkeys."
I'm not sure if they were intended to be pets or snacks.
And I have NO idea where she hid THOSE twenties.
Meanwhile back to my taxes. Where's my chocolate?
Sigh. Where‟d Easter go to?
Teresa Kellerman
Tucson, USA
Pickwick Papers
Family Camp at Kiwi Ranch
Saturday morning
saw us all flying
through the air on
various foxes, these
were as much fun to
watch as they were
to be on. The sight of
someone flying
backwards through
the air, 50 feet off
the ground riding a
unicycle is almost
indescribable - you
really had to be
there. Well done
Yvonne for being
the first to attempt
this one.
Next year we
thought we’d have a
Kite competition...
This year‟s camp was a great success,
and I think the best we have had yet.
The weather was great, the venue set in
lovely rolling countryside. Lots of neat
fun things to do, and of course - good
company.
If you didn‟t get to the camp this year
I suggest you start planning now for
next year‟s. Guaranteed to be an event
not to miss! Lynn and Russ at Kiwi
Ranch, Upper Hutt took good care of
us, fed us well and made sure we had
everything we needed, nothing was a
problem.
Everyone arrived at different times on
the Friday night, this gave us the
opportunity to say hello and catch up
since we last met. Our children of
course wanted to get into the pool and
spa pool as soon as possible so this was
arranged and Rees became the official
‘Pool key holder‟ for the weekend. A
very important job.
Saturday after morning tea was horse
riding, these horses are very gentle and
obviously much loved at Kiwi Ranch.
Those who wanted to were able to
have several rides around the fields and
the lake before feeding and turning the
horses out to the paddock.
In the afternoon parents were invited to
attend the „Fighting Fit-Fighting Fat‟
workshop presented by Peter Davies.
This was an excellent forum, the
relaxed, friendly atmosphere
encouraged lots of discussion
afterwards which gave everyone the
opportunity to ask questions and share
information that will be helpful in
managing our children‟s diet and
exercise needs.
We were fortunate to have to have 5
very enthusiastic care-givers, Lucy,
Caroline, Bridget, Lisa and Grant..
While the workshop was on they took
everyone else on a bush walk,
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swimming and a very creative session
of hat making to be worn at the hat
party that night. I must admit they all
seemed to have a very busy time of it,
judging by all the coloured paper, pom
-poms, glitter, stickers, glue, string and
cellotape that was scattered around the
room. All this had created some
excellent hats.
When this was cleared away it was into
our swimmers and outside to the
waterslide.
It is amazing how much fun can be had
on a slope with a long strip of plastic
and lots of soap suds. Young and old
had hours of slippery fun on this
(though I will say, it pays to have a bit
of extra padding on your bum for this).
Dinner was going to be a BBQ but
with a fire ban on we weren‟t able to
do this, it was a very hot evening
however so I don‟t think anyone
minded being out of the sun.
In the evening everyone assembled in
the hall wearing the most impressive
and varied array of hats I have ever
seen and proceeded to boogie all night
to the disco music. There were flashing
coloured lights, strobe lights, a smoke
machine and of course lots of great
music. The line dancing was great fun
and most managed to do it really well.
This was a brilliant evening, it was just
great to see everyone enjoying
themselves so much and boy, did we
dance !!!!! I think Heather could win
a dance marathon if she wanted to.
Needless to say, everyone slept very
well that night.
On Sunday morning the AGM was
held. Those not at the meeting went
swimming, water-sliding, walking or
playing outside on the play equipment.
After the meeting everyone gathered at
the lake where we went rowing and
Pickwick Papers
The Variety Club Bash
The PWS Association was very fortunate to be benefactors
Entertainers. A wonderful breakfast was provided to 200-
of the Variety Club who gave a grant to help fund our
300 people. The only disappointing part of the morning was
annual family camp ( which was a great success, high on
that it started to rain and the event was outdoors. However,
the list of “places to have been in „98”).
there has been a severe drought here this summer so nobody
I was invited to attend the Variety Club Breakfast Bash as
seemed to mind too much.
they travelled through Blenheim to accept the cheque on
So thank you to the variety club for the money and the rain.
behalf of the PWSA. If you ever get the chance to go
along to one of these events when they are passing through
Cindy Adams-Vining
your town I thoroughly recommend it. It certainly is a lot
of fun.
There were dozens of amazing vehicles from classic cars
to vans, trucks and buses all painted and decorated by their
sponsors. This in itself was worth seeing and then there
were all the celebrities.
My daughters Hannah and Cloe came along with me,
which is just as well otherwise I would not have known
who the “stars” were (not being a very avid TV watcher).
There were many celebrities doing some pretty wacky
things like trying to ride a dyslexic bicycle. I did
recognize faces of comedians and TV presenters, sports
stars and the girls were particularly delighted to meet
Fiona (ex „What Now”) and the McDonalds‟ Young
Family Summer Camp contd..
canoeing or simply sat on the edge and talked or
fed the ducks .This was so very pleasant and
relaxing.
After lunch it was time to say thank you and
good-bye to everyone as we all went our separate
ways. Hopefully to meet again next year, if not
before.
I would like to say a big Thank You to
the Variety Club for their generous grant which
allowed us to have the many extras at this year‟s
camp: Disco, crafts, horse riding. heated pool,
spa pool and care-givers.
Cindy Adams-Vining
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Pickwick Papers
IPWSO
CONFERENCE,
VENICE
MAY 21-24
We are delighted
that both Jo and
Cindy are able to go
to Venice as our
New Zealand
representatives this
year.
Both have worked
extremely hard and
raised their own
funds for much of
this trip. The
Association has
been in a position
this year to also
help with some of
the financial costs.
Attending an
international
conference is an
experience I would
love all of you to
have at some stage.
Being able to put
From the Mainland ...
I cannot believe how fast the year is
going.
It was great to catch up with some of
you at the family camp, although
numbers at the camp were low this
year, a fairly large percentage were
from the South Island - that‟s great!!!
I met up with others were at the
“Fighting Fit - Fighting Fat” workshop
in Dunedin. It is very encouraging to
have so many carers and extended
family attending our workshops, to
know that information is getting
through to the people who have majors
roles in our children‟s lives.
We were very fortunate to have Dr
Peter Davies back in New Zealand to
hold these workshops again. We ran
four of these in as four different
locations is almost as many days. All
of them went very extremely well and
feedback has been very positive.
I wrote to every member in the South
Island about the Dunedin workshop
and the family camp which I then tried
to follow up with a phone call, if you
did not receive either, please let me
know. Addresses change and I am sure
names to faces, hear
the latest research
on PWS, and be part
of a community all
with the interest of
PWS at heart, is a
great thrill. We
wish them a safe
journey, lots of
learning, and time
to see a little of
Italy’s most
beautiful city...
Look forward to
hearing all about it
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my list needs updating.
I am pleased to be able to report that
due to our endeavours to promote
PWS and our Association in the South
Island, contacts with service providers
etc has produced some excellent
communication networks. We now
have good contacts with most of these
organizations through-out the South
Island. Of course much of this is
success is due to your own efforts as
parents and PWSA members and I
thank for your support in this.
Next month Jo Gibbins (from Hawera)
and I will be representing NZ at the
International PWS Conference in Italy.
At this conference we will be
supporting Australia in a bid to hold
the next IPWSO Conference in Sydney
in 2001. It would be great if this were
to happen, I will be encouraging
everyone to plan ahead for it.
Cindy Adams-Vining
Pickwick Papers
Memories of my Auntie Di
[At Di Stuck’s funeral, her niece, Elizabeth spoke about her Aunt. I asked if we could share her
thoughts and memories as seen through the eyes of a niece.]
Auntie Di was dearly loved by all of her eight nieces. She was a
patient, positive Aunt that I hardly ever heard complain. For most of
my childhood, Christmas meant holidays in Hamilton, spending time
with my Grandparents, cousins and my Auntie Di.
Luckily for us nieces, Di had some interests that all of us could take
part in. For example Di loved her puzzles. I was always amazed at
how she could complete a thousand piece puzzle without looking at the
picture on the box once.
Another interest that Di enjoyed was her love for animals.
Throughout my holidays in Hamilton I remember her owning animals
ranging from a praying mantis, budgies and cats. Recently I was
glad to find out that Di and I shared the same love and interest for
gambling. We both enjoyed scratching those instant Kiwis while she
was in hospital last December.
One of my fondest memories of time spent with my Auntie Di was in
Grandma’s pool in Hamilton. Di helped all of us cousins from an
early age graduate from inflatable arm floats to swimming a width or
two on our own. Lisa, Cara, Tash, Cindy, Anna, Meridith, Caroline
and I would not be so confident in the water if it was not for Diane’s
patience and coaching.
Up until the age of about 13, I firmly believed that my Auntie Di was
an Olympic Gold Medallist. I remember her showing me the Gold
medal she had won at the Olympics. It was not until I grew older and
taller than Di that I realised she was special and that her Gold Medal
triumphs and her enormous proud collection of various soft toys was
thanks to the IHC community in which she lived.
Di helped me to appreciate special people like her. I know I was
privileged to have Di as my Aunt. Not many nieces can walk down
the path of acceptance; enrichment and discovery of the important
things in life that Di led me down.
Auntie Di and I shared many little important moments as all Aunts
and nieces do. I could try to tell you about them all. Unfortunately
no amount of words could express the impact that one special lady
like Auntie Di can have on someone. Di was in her own unique way a
very loving individual. I am sure you would all agree with me in
saying that she was indeed our own special Lady Di. I love and miss
you Di, Goodbye.
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P O Box 143
Masterton
New Zealand
Pickwick Papers
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