What does a great day look like?!

Here's what a great day looks like in Sharon's household - her son Derek has had a really tough year and been in and out of hospital many times, the most recent time being only two weeks ago, but is now on new medication and is feeling great.  So great, in fact, that he scored second best in the Special Olympics golf tournament, skills division, out of a field of around 40 golfers... and won a silver medal!!

Sharon says it was not only a great day for Derek, but for her whole family!

Stories like this just bring a surge of "that's what it's all about" to one's heart, don't they?  We all love it when our kids, young and old, achieve something that once we would never have thought they could.  It's magic just to see them beat the odds and come up smiling.  Derek has had a tough year, but he's really come up smiling this time!

Working in Partnership

Dear Prader Willi Associations around the World,

My name is Rob Pleticha and I am the Online Communities Manager at EURORDIS.

We hope all is well with you and your colleagues.  An opportunity has come up, and we'd like to get your thoughts on it and to share the following information with any of your colleagues who might be interested.

EURORDIS is working in partnership with the National Organization for Rare Disorders, NORD, to establish global online patient communities.  The aim of these communities is to help affected individuals and families understand their conditions, connect with others, and access helpful tools and resources.  Ultimately, we hope these communities also will help link patients and families with researchers and clinicians who have expertise in their diseases.  A tool has been developed to provide translation to and from five different languages to help break down barriers that might otherwise exist.

We are currently in the early stages of setting up a community for PWS, and we would be delighted if your group was interested in partnering with NORD, EURORDIS, IPWSO, PWS USA, PWS Romania, PWS India, and PWS New Zealand.

The vision is for the online communities to be monitored by patient advocates, patient organization representatives, and experts from within the specific disease community.  This format will create a trusted safe space that can facilitate community building and information-sharing. 

We recently launched 11 communities, which you can visit at rarediseasecommunities.org.  We hope to launch 3 or 4 more in the next several months and, ultimately, many more.  Is this something that might be of interest to you?  And, if so, would you be available to hear more about it sometime soon?

Rob

Robert Pleticha, Online Patient Communities Manager

 EURORDIS

Plateforme Maladies Rares
96, rue Didot
75014 Paris
France Tel: +33 (1) 56.53.52.69
Fax: +33 (1) 56.53.52.15
robert.pleticha@eurordis.org

Snippets from our Website

Residential Providers' Resource

One of the most interesting things IPWSO has done over the past three years is the development of our Porfessional Providers and Caregivers' Advisory Board. This was the brainchild of Pam Eisen (our past President) and Norbert Hödebeck-Stuntebeck and Hubert Soyer.  Both Norbert and Hubert are involved with the development and management of residential homes in Germany: Norbert is Co-chair of the advisory board, along with Mary K. Ziccardi (USA) and Hubert is also on the IPWSO Board.

The production of Standards of Care & Best Practice Guidelines is a valuable resource for all residential providers, managerial and hands-on.  This manual starts from the beginning - what are the residential options/what should a house look like and provide, through to the hands-on skills required to manage the characteristics of the syndrome.  It also covers psychiatric behaviours, psychological information, dietary and physical exercise, and is the "Bible" of providers, carers, and stacked full of information for parents and teachers as well.  It's available by contacting us through our website www.ipwso.org.

New Board Members
We are delighted to welcome our new Board members: Georgina Loughnan from Australia, Anil Choubey from India,  Jose Luis Garcia from Chile, and Hubert Soyer from Germany.   IPWSO holds board elections at each 3-yearly international conference and posts are held for 2 three-year periods.  Most of our work is done via email and satelite link-up from all corners of the earth.  We meet face-to-face at our international conferences and, finance permitting, once inbetween times.

PWS Conferences
Three PWS conferences coming up!  Japan - November this year; Australia - March 2012, and UK in July 2013.  Take a look at what is on offer.  Specialised conferences dedicated to the needs of those with PWS and those who care for them, are the life-line to successful living.  Without the expertise and knowledge of those who spend their lives studying PWS, parents, caregivers, teachers, and all those who come into contact with those with the syndrome, we would be hard-pressed to know how to manage the situations with which we are faced. We are lucky in the PWS world to have many extremely knowledgable experts who have got to know not just the syndrome, but the person behind it, and his or her family.  It's this integration between professional and parent that is invaluable!

It's not easy, raising a child with PWS, managing in the classroom, finding the right residential support, and the more we can encourage parents and experts to share experiences, the better off our children will be.  

Knowledge is sustainable, and PWS is unique!

School Bullying

What is it in the human psyche that allows us to bully?  It seems to be a need to be more powerful than the other person; it seems to be a way of survival for some people.  Kids do it to make themselves look tough and to gain popularity.  Most grow out of it, but plenty don't; the bullying just becomes more subtle and cunning.  We've all experienced bullying in one form or another, and perhaps some of us have done the bullying ourselves.  It's not great to be at the rough end of a bully and worse when you can't stick up for yourself; even worse if you aren't part of a crowd, but are a loner for one reason or another.

Often our kids become loners, not through any fault of their own, but because their peers outgrow them and move on.  The knowledge gap becomes wider.  Being the one left behind often means a vulnerability to bullying.  This can take several forms from snide, or harsh comments, laughter, mockery, pushing and shoving.  But it can also take the form of being persuaded to do things for the fun of the protagonist.  Taunting can make our children very quickly enraged, creating nothing but amusement for the others, and often leaving our kids to take the blame.

Our children often take the blame for things going missing - particularly in the classroom - and once it is known that not only food, but anything else, can be blamed on our kids, they will carry this burden for as long as they are there.  Nothing easier to automatically blame our kids for anything that's gone missing!  Trouble is, that it often is something that has taken their fancy, and the trouble with that is that our kids are strongly into denial ('it wasn't me!!!').  

If bullying is something your child is having to cope with; get help.  If it's at school, make time to talk to the teachers, make sure they understand what is happening, what it means to have PWS and how they can help and support your child.  Teach your child not to respond, but to walk away from the bully (easier said than done, I know), and to tell a teacher they trust.  Help them understand that it isn't telling tales, but that often the only way to get a bully to stop is to get help from an adult.

I've seen bullying happen in all sorts of ways, and watched my daughter having to deal with it herself. It usually takes place at the swimming pool when having PWS can attract all sorts of unkind comments and stares.  I've seen her front up to the bully and tell them she "can't help being the way I am - I've got Prader-Willi Syndrome".  It doesn't necessarily stop the taunts, but stops the bully in their tracks for a few moments!  I've heard her tell people to stop staring at her, and I've heard her use a few choice swear words as well.  It doesn't help, of course, and often requires quick intervention before she goes any further. 

I think bullying has become more subtle, more hurtful, than ever before particularly cyber-bullying through texts, social media like Facebook and Twitter, and email.  It's a problem for parents and schools world-wide and it seems as soon as we think we've started to stamp it out, it flares up in another guise.  Parents are their children's best advocates, and for those with PWS, we are the lifelong advocates.  Let's stand up to bullying!

It's not about the egg...

The other day my daughter, who was home with us for a few days, decided she would be very helpful and get her own breakfast.  She decided on a boiled egg and toast.  Instead of waiting for me to be in the kitchen with her, she thought she would do it anyway because after all, she was 27 and knew-how-to-boil-an-egg for-heavens'-sake.  We have gas hobs, one which is quite large and three smaller ones.  She lit the larger one and put on a small saucepan to boil the water.  As she was doing this, the flames which were high up around the small pot, singed her sleeve and burned her wrist.  Not badly, but enough to make her yelp.

When I arrived in the kitchen and saw what had happened, I naturally told her in no uncertain terms that she knew she was NOT to cook anything, that she KNEW she was not to touch the gas hob, or to even be in the kitchen without me.  She exclaimed that she "knew how to boil an egg and that anyone would know how to do such a simple thing."

"It's not about the egg", I said.  "It's about everything else - it's about thinking about what you are doing, it's about thinking of the consequences, it's about safety, it's about knowing exactly what you are doing!"

"Well", she said, "I didn't know the flames were going to burn my jersey, did I?" 

Which is exactly what it's all about - it's about the dysfunctional versus the functional.  It's about the "islands of intelligence" that our kids all have, but the lack of connecting bridges.  And it's this, that makes it even harder for them... so many of our adult children are quite capable of doing things for themselves, and resent being told what to do.  But so many of them, with their concrete thinking patterns, think only about the "egg" and not about what might happen if something goes wrong.

We have to out-think them all the time, be one step ahead, imagine all the possibilities, particularly all the things that might go wrong.  All the time, every day.  They can't do it themselves.

The Therapy of Love

Daniele and "his" children

By Giorgio Fornasier  (Italy)

                                                                                          

My son Daniele was born 32 years ago and we were lucky that the pediatrician who saw him first knew about PWS and made a clinical diagnosis when he was 8 months old.

After he finished his studies at a professional school, he worked successfully in a couple of factories for over 11 years and was happy to live a life that looked normal to him. Two years ago, all of a sudden something changed dramatically and we think someone at work offended him saying he was a disabled boy without a future. He probably opened his eyes on a different reality and realized he was nearly 30 years old, most of his schoolmates were driving a car, had their own apartment and were married with children… He had nothing instead and no hope or expectations to have it in the future!

He fell into a depression, left his job and kept crying. He tried to find a way out and thought life was easier in South America where we went together and where some “friends” told him he could get as many girls as he wanted and he could get an easy life. He started speaking Spanish and changed his name with a Spanish one and got angry with anybody calling him Daniele. He also phoned travel agencies trying to get an air ticket to go to Paraguay and drove us a bit crazy.

Facing this crisis, we felt lost and desperate and took him to a specialized Hospital in Milano. For the first time in his life he had to take psychotropic drugs to overcome his paranoiac obsession and after a few months he forgot about his Spanish identity and was Daniele again.  But he was no more the lovely, cheerful and caring boy we knew and we had to accept this change and a new reality.

To worsen the situation ten months ago his brother Redi, who is only one year older, became father of a beautiful boy whose name is Alessio. You can imagine how happy we were, but Daniele wasn’t and strongly refused to be called as a uncle and did not recognize him as his nephew. Psychologists at the Hospital said he was jealous, but this was not the case. Our children with PWS are not stupid, they have a simple but clear and precise logic that we “normal people” often do not realize. To recognize Alessio as his nephew was the same as admitting his defeat and to be different with no hope to be a father himself too.

I took Daniele with me to Brazil last February to offer my wife a period of relief, as I was leading a group of 40 tourists to visit  the South of this Country where a large population of Italian origin lives. At the end of our program we visited Bairro da Juventude, an Institute run by an Italian Priest, Father Vincenzo Lumetta, who takes care of over 1,500 children who live in the poor and degraded outskirts of a town in Santa Caterina State.

 

Children who have not enough to eat or have not enough clothes and often suffer any kind of violence. Children who can have a future only in this house where they can find love, food, clean clothes and especially a good professional education, so they can get a job when they finish the internal school. As I am personally involved in helping this mission to support these children with individual sponsoring of Italian families that we call “distance adoption”, I always take people I lead on tours there, as they usually adopt many children and provide the money they need to be properly followed and continue their studies.

I was too busy to follow my group of 40 persons and I did not realize that my son Daniele chose a boy and a girl, took a form and filled it completely with all data to become their “father”. While I was in the office together with Father Vincenzo and had just made my annual donation to the Institute, the door opened and a shiny Daniele came in holding a boy and a girl by hand. He was excited, but at the same time he was afraid of my reaction, as he did something important without my permission. He first looked at me and then said: “Father Vincenzo, I adopted these two children!”. Father Vincenzo who knew about Daniele’s crisis smiled and said: “They are yours! Your father just paid one year fee for both.”

I will never forget the gratitude and happiness in my son’s eyes. He gave me a long strong hug and then introduced me to my new grandchildren João Pedro and Ana Alice. Then Daniele asked me to give him my mobile phone. He phoned my wife first and said: “Mom, I made you Grandma twice!”, then he phoned his brother and proudly said: “Now you’re uncle too!” and then he said the key magic words that made me cry and understand the nightmare was over: “How is my nephew Alessio?”.

A few days later I brought a new Daniele back home, the happy smiling boy we knew, more mature and more careful now. He was no more a child, but the responsible father of João Pedro and Ana Alice. Since then our life has changed and our son Daniele is a wonderful uncle to Alessio and went back to work as a volunteer guard at the local Municipality. He always talks about his children and proudly shows their pictures to everybody he meets, as all “normal” parents do. He writes to them quite often and they answer to him and have exchange of drawings they make, hoping they will be connected soon by Skype, so they can talk directly and see each other on webcam.

Once again our son with PWS is surprising us. He found the way out alone and the perfect solution to his problems. He did not know the causes, but he realized he could not have biological children in his life, so he found the easiest way to get his own family anyway. His life has changed completely and everything is finalized to his international family, so he is taking care to save or earn the money he needs to pay the annual fees, he often writes emails or letters to them and get organized to send gifts or wishes for their birthdays or for Christmas.  We plan a trip to Brazil with Daniele every year to see how our grandchildren are doing and this is really the best therapy we can offer our child and ourselves too… a therapy of love.

If anyone wants more information on Father Vincenzo’s program, they can contact Giorgio at g.fornas@alice.it.   Giorgio is the past president of IPWSO, the international organization for PWS, and is currently the Director of Programs for IPWSO. 

Smarter than Jack....

... is the name of a series of books that my daughter likes to read.  It's more of a catch-phrase in our household for just how clever those with Prader-Willi Syndrome can be.  She now lives in residential care with another young woman with PWS who is also smarter than Jack.  They've known each other since they were very young and have grown up together (well, as much as you can be with the geographical distances); sometimes liking each other, other times, well, just not.  It's the way of PW.  Especially when you're both smarter than Jack.

Locks are part and parcel of managing this syndrome.  Locks on food cupboards, pantries, refrigerators, and, if possible, on handbags and wallets.  This is because  the dysfunctional hypothalamus (two words I've learned to spell and pronounce with monotonous regularity)  does not have an 'on/off' regulator switch for the appetite.  Which means - bottom line - those with the syndrome can eat themselves to death, quite literally, if not managed on a 24/7 basis.  So, there are locked cupboards in our house, along with a locked fridge.  Siblings always resent this, and who can blame them.  It's unnatural, prison-like, embarrassing, and, well, just too different to be normal.  But for the one with PWS, it's seen as a relief.  It's a unpassable boundary.  It's safe.  There is no need to fret about food being such a temptation.  And, besides that, it's a total protection for what is seen as "my" food.  Sibs can't get at it, outsiders can't steal it, it's quite safe, and of course, if you're in residential care, staff can't help themselves to it either.

But, if you have PWS, and you're smarter than Jack, it stands to reason that locks are a temporary barrier, a challenge if you like, and let's see just who's smarter than whom.  My daughter and her friend live in residential care.  The locks are the best keypad variety with many, many combinations.  My daughter saw this as an 'end of story' limit.  Her friend saw it as a challenge.  For days and days she watched carefully as the staff punched in the code to open the kitchen.  Even though staff turned their backs and obscured her vision, she still watched and learned.  She counted the number of punches: four.  She noticed the "C" button was used first, and realised that staff then only used the top three numbers.  Still keeping quiet about her observations, she waited for an opportunity to test out her theory.  She punched the C button, then tried several options for the top line.  One of them worked.  She was ecstatic.

Friend made an executive decision.  She would not tell my daughter just yet as she would be "too over-excited".  She would wait until the weekend.  Quite often temporary staff were on over weekends and, because there was not necessarily a schedule, staff wouldn't be on their cases time-wise.  Careful time-watching gave the girls the chance they needed.  The code was cracked.  The kitchen was entered.  Cupboards were quickly opened.  Disappointment reigned as there was only "healthy" food :-(

There were water-crackers, though, and noodles.  Butter and jam was sneaked and a packet of noodles smuggled out of the kitchen.  Door was relocked and girls retired to my daughter's bedroom.  Guess what?  They decided they wouldn't eat raw noodles - good grief no!  They had a much better idea.  Back to the kitchen, out with a small stainless steel bowl.  This they filled with enough water to cover the noodles, and placed the bowl on top of the heater.  Friend decided to leave the bedroom "as staff would want to know what we were doing", and with instructions to call her the minute the noodles were soft, my daughter was left to preside over the cooking.  Apparently it took 15 minutes before the noodles were considered edible, but the surreptitious feast was made all the more delicious by the knowledge they'd been smarter than Jack.

Of course, it wasn't to last.  Although not caught at the feast, the friend was snapped early on Monday morning, standing in the kitchen deciding what to take next.  Discretion was not the better part of valour, and so the story of the locks came to a sad ending.  For the time being, I daresay.


(For dietary needs in PWS, click here)