Writing styles

I came across a card written by Francie the other day and wondered why do people with PWS all write the same?  Has anyone else noticed this?  And then I wondered whether all people with disabilities write similarly, and that led me to wondering why.  It took Francie a long time to learn to write - in any style - and she had to have a special triangular grip thing on her pencil so that she could hold it better.  Francie has never had GHT, so I don't know whether that would make a difference as far as the writing style goes, but it's never really changed over the years.

Would anyone like to hazard a guess, or offer an opinion?

Social Adaptations

by Linda Thornton 

In thinking about social adaptations when dealing with PWS, two things come to mind:  How easy life would be if the issues around food weren't there, and second is what part the pressure of success plays.

Let's look at "success".  What does it mean to succeed?  The dictionary says it means to be good at something, to increase your fame, social position or financial gains.  The pressure put upon us these days to do just this is enormous.  We pass it on to our children in the form of 'expectations'.  These then become goals and the pressure is reapplied… and therein lies the crunch – success, or failure.

We all do it to a greater or lesser extent.  After all, we want to succeed and we want our children to succeed.  Then, along comes the child who fails the very first expectation – that he or she will be 'normal'.  And our world crashes.  We are faced with a dilemma of enormous proportions:  suddenly our idea of success seems to have become a failure; we start blaming ourselves, blaming the doctors, blaming anything that comes to mind as we look for reasons why we have failed to produce perfection.

And after a while we start to look at perfection and success in a completely different light.  We have to.  In fact, we are forced to, if we want to be able to cope.  Goals have to become quite different.

There are, to my thinking, two new mindsets here: 

The first is that we recognise that many of the goals we set for our children are in actual fact, goals that we are setting for ourselves.  We want our children to succeed because it reflects well on us – it makes our parenting skills look good.  It makes our family look good, it makes you and me look good.

The second is that we learn to figure out what it is that our kids with PW need to survive in this world, and make their goals our goals.  Not the other way around.

The second one is the hardest, and will take the longest time.  We have to learn to look at priorities differently (is it a priority that Johnny learns to crawl, walk, talk at the same time as the neighbour's child, or is it ok if he takes a little longer and does it in his own time?  Is it a priority that Susie learns to play the piano because her sister does, or is it ok if she plays with her dolls instead?)

We must learn to take the pressure off ourselves.  If Johnny can't get Growth Hormone Treatment from the minute he is born; if Susie isn't showing any signs of improvement on another treatment, we panic.  Our child won't achieve, won't be as good as the next one.  And so we reapply our own goals.  Sometimes it's just not going to happen and we have to accept that not all of our kids will get the treatment  that we want them to have.  They won't all be slim and beautiful.  They won't all compete in the Olympics.  They won't all achieve high scholastic marks; get a job; get married; have kids of their own.   And they won't ever, ever, not have Prader-Willi Syndrome.

What they will need, however, and what we can give them, are some excellent role models that they can look up to all their lives.  And guess who they're going to be?  Yes – their parents!  What you model to your children will be what they will model in their socialising.  And it's my guess that if we role model our expectations higher than our child can meet, setting them up to always striving to reach that unattainable goal, then we’re only buying into disaster.  If we role model our expectations to the level of the child, then we are buying back into that elusive 'success' model. 

So, for example, we don't role model that that the family car is the sole method of transport, but we model to meet the child's needs… and walk.  Or teach them to ride a bike, or use a tricycle, or let them push the pram.

• We don't role model the fast-food outlet as the way we always get food; but we role model an enjoyable lifestyle by cooking healthy meals for all the family.
• We don't role model that money comes out of a hole in the wall; but that pocket money can be earned in return for help around the house.
• We role model that life in the slow lane can be fun!  Getting into the car and getting settled can take forever… but started earlier, taken slowly and at the child's pace can help avoid the build-up of stress – yours and his.

So let's look at the part food plays in this equation…

What does it mean to you to 'socialise'?  Well, it probably means to get together with friends and along with good food and wine, and good conversation, to enjoy yourselves with lots of laughter and companionship.

Or it might mean to go out to a meeting or gathering somewhere new and have a pot-luck dinner and make new friends.

Or it might mean getting together after a sports event, over a few jugs of beer and a quick take-away meal and going over the game in fine detail.

It might just mean bumping into someone in the supermarket you haven't seen for a while and going for a cup of coffee and a bite to eat to catch up with each other.

Whatever it might mean to us, socialising always seems to have one common denominator: food.

It's how the world goes round.  Or is it?

We gather at our conferences from the four corners of the earth; from different countries and different cultures.  But the one thing we have in common is our children.  And the one thing they have in common is that they are not like the rest of us.  They cannot simply sit down and share a meal, or go to birthday parties, or picnics, or even to Grandma's, without a lot of preparation and planning beforehand.

Again – lots of expectations, lots of pressure, and once again we have to think very carefully what that means to us… and what it means for them.  Life around food is never going to be normal for our kids with PWS and sure as eggs are eggs, it's going to change our whole perspective around food as well!

When Francie, my daughter, was born some 26 years ago  there was very little knowledge about PWS.  I learned about it from a women's magazine which had an article about a young man in Australia who couldn't stop eating.  When I took this knowledge along to the paediatrician, it was (predicably) discounted.  After all, I was "only a mother", how would I know how to diagnose?  However, we did have a blood test done, but in those days there was no FISH, no DNA diagnosis, no methylation testing, so the diagnosis came back as normal.  (She's maternal disomy.) But as I continued to struggle for a diagnosis, I decided to assume it could be this weird thing called PWS and to watch very carefully for clues.

My first clue came at a birthday party when Francie was about 3 yrs old.  She was the last person to leave the table, and had to be taken away kicking and screaming.  My next clue came when she developed a passion for church and particularly going to receive Communion.  She would stomp up to the railing saying loudly "I want bread!"  My final clue came in hindsight when I realised the first word she pretty much ever learned was "morningtea".

Armed only with knowledge from the Women's Weekly magazine, I sought help via 'snail mail' – no email in those days – from the States and England, and soon confirmed in my own mind that Prader-Willi Syndrome was what we were dealing with.

Socialisation became a problem, I didn't know how to cope with this child who seemed determined to eat everyone out of house and home.  So for quite some time, I stayed away from events that included food; I dreaded Easter and Christmas, and loathed birthday parties.  

It took a while for me to realise that what I actually dreaded was having to tell people that there was something wrong with my daughter.  At that time, it was one of my biggest adjustments.  Nowadays with all the information in the world, I can do this very easily.  I have a bunch of pamphlets at home which I hand out on appropriate occasions.  I can talk about PW in my sleep.   Each time Francie makes a new friend; each time we have to go out somewhere that includes food; each time she is invited to join in… along go the pamphlets.

I have to say, though, that as she has grown older, she has become very plausible – well, ok, "manipulative" probably is a better word – and if I don't get in first, she can outwit anyone. 

She is a very smart kid.  I wish that I could harness that smartness into something productive.  She already has.  Recently we have moved from the country to the edge of a small town – a really small town – where everyone knows everyone else and what they had for breakfast.

A while ago, when Francie was much younger, I was accosted by a neighbour I hadn't yet met.  "Your daughter has been over to my place 'collecting for the church'" she said.  "What??" I erupted – "she's been what?"  And the story came out that Francie had decided that collecting for the church could be quite a plausible way of getting money.  Fortunately the neighbour had second thoughts and told Francie she didn't give to anything without an envelope stating what it was for.  Quick as a flash, Francie said "haven't you got an envelope inside that you could use?  $2.00 would be fine!"

What Francie saw as an excellent source of funding, the neighbour quite rightly saw as daylight robbery, and I, well I'm afraid I saw the whole thing as hilarious.  More so, since the neighbour obviously had the measure of Francie, even though she had no idea who she was, or what she had.  I invited the neighbour over for a cup of coffee and a brochure the next day so as I could explain.

The concept of socialising a person with PWS, is going to revolve for all of their lives, around food.   And probably in spite of our best intentions, and no matter how quick we think we are, there will come times when your child is going to get the better of you.  And get the better of their teachers, their siblings, their grandparents, and neighbours.

It pays to try to be one step ahead.  Preferably, nine steps ahead.   It pays to wise up people ahead of time, and it pays to start early.  I should have been over to the neighbour before it had time to happen.  But I'm shy.  I don't like to introduce myself with a lecture on PWS; and I'm always hopeful the worst isn't going to happen.  Fat chance.

Giving people the right message at the right time is essential.  It's something I'm still not good at.  But by the same token, it's something that other people aren't good at hearing, either…

Francie recently had a boyfriend.  She went to his school leavers' ball.  She looked lovely and I was really proud of her and happy for her.  A few days earlier, I wised up his teacher who was going to be there with his group of Special Needs Leavers.  I told him as best as I could about PWS and asked him to keep an eye on what she ate around supper time.  I explained how difficult it was to manage her diet and how important it was.  Since this was the school that Francie would be going to in a matter of a few more months, I didn't go full out on PWS, figuring there would be time for that later. 

Francie had the time of her life.  The boyfriend, so handsome in his tux, presented her with a corsage, and danced with her all night long.  At 10.30pm when I went to pick her up, there they were, arms around each other in the middle of the dance floor.  Francie was practically asleep and must have rested heavy on his shoulder, poor boy.  I smiled happily over at the teacher who beamed back at me and said he'd kept an eye on her, and all was well.  She'd eaten (and he listed the food): 2 sausage rolls, 2 sandwiches, some crisps, 2 or 3 fizzy drinks, and a plate of icecream and jelly.

My fault.  Wrong message.  I'd told him to keep an eye out for her, and he had done just that.

It is more difficult as she grows older to control her food intake.  There are many more situations available to her that include food – from school lunches to supermarkets where they now have cooking demonstrations and food samples freely available.  Every social event includes food and it's difficult to persuade the rest of the world that a social event without food can be just as enjoyable; or if food is a requisite, then carrot sticks and fruit platters can be quite attractive!

And then there's the added pressure of not embarrassing them in public.  Now that she's 26, it's very important to her to be able to be a little more independent and not to have her Mum constantly in tow.

When she was 16, she decided that she was going to join our little local library.  I thought that was a wonderful idea.  A walk down to the library on Saturday mornings, a good choice of books, and to walk carrying them back was a fine idea. 

At first she told me it would cost $5 to join.  I didn't fall for that one.  We compromised.  I said she should walk down and join up and if it cost any money, to ring me and I'd come down.  She seemed fine with that.  Just before she left the house, I frisked her for any extra money (and food, despite locked cupboards).  Nothing.  So off she went.  Then the alarm bells sounded.

Feeling cross with myself for even entertaining the idea that she was cheating on me, I gave into my fears and drove down to the library.  There I saw her, sitting up in the mezzanine area with books on her lap and watching two little girls playing.  She was totally absorbed and didn't see me.  I was just about to chide myself for being so suspicious, and sneak away, when I thought no, I'll just let her know I'm here and see if there was a joining fee after all.  So I jauntily walked up the stairs and waved to her.  She looked at me as though she had turned into a block of concrete.  She fixed me with a horrified look and there beside her, I saw a large paper bag of sweeties.

Words failed me.  I stalked over to her and prodded her to her feet.  I grabbed the almost empty bag of sweets and marched her downstairs and out to the car.  I bit my lip and said nothing til we got home.  I sent her to her room and when I had calmed down sufficiently, I went and asked her what she thought she had been doing and where did she get the money from. 

She replied that she asked someone for money and they gave it to her.  My heart sank.  "You know what this means??"  I hissed at her, "You can never ever go out of this house now unless someone I trust goes with you.  You have destroyed my trust in you."

Then I asked "why, Francie, why did you do it?" and she answered, "Because I wanted to know what it was like.  Everyone else I know has pocket-money on Saturdays and goes to town to buy sweets."

And I thought to myself – how could I punish that?  How can I make a criminal case out of what is so normal? 

And I think that is probably the worst thing about PW.  It makes such normal things to you and me, so abnormal for our kids.  It sidelines them; it makes them different; it points them out.

Over and over again, we as parents have to be ready for this.  We have to judge for ourselves what is normal for us may not be normal for our kids.  We have to learn to say "no you can't" when to your child's sister or brother, to the rest of the world, the message is "yes, you can".   

And when we live in a society where food is all around us, where everything we do seems to have to include eating and that 'eating' seems to have to be junk food, it gets more and more difficult.  We try to teach our child with PW that junk food is bad, that we don't need it, and they see every other child often their own sisters and brothers, enjoying those forbidden fruits.

It's not easy – we all know that.  But the one bit of advice I will leave you with is this:  take some of the pressure off yourselves…take advantage of all the help that there is… keep on educating everyone who plays a part in your child's life… and keep on smiling!

Season's Greetings

Isn't it amazing how the end of the year sneaks up on you before you have time to say Prader-Willi Syndrome!  And isn't it lovely that no matter how weary you feel, no matter how apprehensive you might feel about the coming festivities, your son, or daughter (with PWS) still feels excited, year after year, at the promise that something good will be coming their way? 

My daughter, now 26, buys and sends more Christmas cards than anyone I have ever known.  She also buys more presents for more people than I care to count.  She starts in August - one of life's 'planners'.  Although you might think that she gets more joy from giving than receiving, she keeps a careful tally of who has not reciprocated...

This year her cat, who is currently boarding with us, has joined the festive season of gift-giving.  Over the past twelve days, the cat has brought us gifts.  Usually in the small hours of the morning.  He loudly proclaims his generous bounty, calling for us to come and see.  He keeps this up for a while until the calls tail off in disgust with our apparent disinterest.  In the morning, we are greeting with her gift - usually a baby rabbit.  There have been three of these so far; the poor little things, not a scratch upon them, but stiff and cold.  I give them an appropriate burial.  Last night, there was the usual calling out to come and see his latest gift, and again, no answer from those whom he wished to impress, so in the morning I took care as I walked barefoot down the hallway, looking for the small offering.  Nothing to be seen.  Hmm, I thought, I wonder what that would be about then?  I didn't have long to wait; a small brown mouse with a long twitching tail and little black eyes was regarding me silently.  Alive!  A living gift!  Unhappily, I couldn't accept this little gift either, and managed to throw a towel over the mouse and take him outdoors to release.

It is hot and humid with squalls of rain here, a far cry from the storms of Europe, but wherever you are, and whatever you are doing over this season, we all hope you stay safe and well, have a lovely time with your families, and that 2011 brings you peace and joy.

PS.  (Dec 23rd)    This morning's gift was a little yellow bird...
PPS:  (Dec 28th)  This morning was a live mouse...

Congratulations South Africa PWSA!

Rika and family (with Linda Thornton)

The Willowton Group, marketers of edible oil products, decided earlier this year to donate 4 million Rand (USD590,362, or E420,719) to 40 deserving charities throughout South Africa to commemorate its 40^th anniversity.  Three ceremonies will be held this month at which the selected charities will receive their cheques, the Prader-Willi Syndrome Association of South Africa, being one of the lucky forty.  This extremely generous donation was done by the company which is enjoying substantial growth in its industry, so as  “not to forget the impoverished communities across the country”, said director Ali Akbar Moosa and chairman Abdul Razak Moosa.

President of the South African PWS Organisation, Rika du Plooy, and her colleagues, are “over the moon” with the donation, saying that this will certainly help to put PWS on the map in South Africa, and they will be promoting awareness of the syndrome in the hope to identify the many families where there is a child, or adult, yet undiagnosed and needing their help and support.

Congratulations, South Africa, from all at IPWSO!!

Can the satiety mechanism in PWS be controlled?

Kate McAllister and colleagues at the Cambridge University, UK, have been involved in researching the brain's ability to appreciate satiety.  This video clip  http://www.youtube.com/watch?v=ofgjtGra3I8  shows an interesting perspective on looking at how the on-off switch for fullness, particularly in Prader-Willi Syndrome, might be regulated.  Much research has been done on looking at the genetics of obesity, and the genetics of PWS could well hold the key to discovering why the desire to eat over-rides any control mechanism. 

Please have a look at this YouTube clip on research being done by Cambridge University on monitoring the brain's ability to appreciate satiety. If they can implement the conclusion they are suggesting, it is very exciting news.  To contact Kate directly: km511@medschl.cam.ac.uk

New Video "Parental Stress When Your Child has a Developmental Disorder"

A collaboration of the PWS Association (USA), the Angelman Syndrome Foundation Inc, and the International Rett Syndrome Foundation as a genetic partnership, has developed a videotape of Dr Paige Powell's presentation on "Parental Stress when your Child has a Developmental Disorder: Helpful TIps and Coping Strategies.  The link to this is:

http://www.bioedonline.org//workshops/evening-genetics.cfm

Any family with a child whose behaviour is challenging, will find this video of coping stragegies and helpful tips, extremely useful.  So often we forget how important it is as the primary caregiver to look after ourselves first.

Further information on Rett Syndrome & Angelman Syndrome can be found through these links:

Rett Syndrome:

http://www.rettsyndrome.org/

Angelman Syndrome

http://www.angelman.org/

Greetings from Cuba!

Hola, and greetings to everyone from Cuba where we have just held our first family group meeting!

Over the past years we have struggled to get information about Prader-Willi Syndrome, and it wasn't until I was able to attend the PWS International Meeting earlier this year in Taipei, that the idea of starting our own Cuban Family Group  become a reality.  It was so exciting in Taipei to be with other professionals  who all had such a great interest in understanding the syndrome and to meet so many parents who also had children with PWS.  Just knowing you are not alone in the world is heart-warming.  Learning that there is so much research being carried out by scientists, and hearing about the management programs for care-givers and parents, is so important.

So, together with strong support from IPWSO, we started to work on having our very first PWS meeting in Cuba.  We were aiming for September, which was only four months after the Taipei conference.  We did not aim to have a great number of people, just the few parents along with doctors, and psychologists.  On September 22 - 24th, we met for the first time!  It was a great experience, sharing with other families.  We concentrated on the topics of food, behavior and the management of PWS. This was such an important meeting for us because it was our first step.  We called the meeting "Day by Day", so that we can concentrate on the best steps forward for our families in Cuba.

We would like to say thank you to Caritas Ciego de Avila, the PWSA of USA, and the Association Madrilena S. Prader-Willi, for their unconditional help.

Loisel Bello, MD