IPWSO at ESPE "Why wait for obesity to diagnose PWS?"

Dear IPWSO members,

I just wanted to give you an update on our IPWSO awareness booth at the ESPE conference.  Although it is the “European Society for Pediatric Endocrinology”  there are 2,500-3,000 participants from all over the world – and I mean all over the world.  Giorgio and I are both invigorated and exhausted!  We were just crazy busy today with people stopping by our booth for information on PWS.  There were times when even with the two of us, we could not talk to everyone or get them all scanned because there were too many at a time, but they all got one of the packets Giorgio put together.

IPWSO Booth at ESPE Conference, Glasgow

We have extensive packets for those who have no information, packets for those that received information from us before, but were looking for new information, and crisis packets for those dealing with weight and behavior issues.  I also brought a suitcase full (75) of our brand new growth hormone books that we created and printed at PWSA (USA).  Between Giorgio’s 9 cartons and my suitcase full, we only have 3 cartons left and have 2 ½ days to go!  We are looking at ways to stretch the information. 

Giorgio has been able to recruit physicians from three countries where we are not represented to be the IPWSO professional delegate.  We have also made contact with the key people at the two pharmaceutical companies that have the indication on GHT and PWS.  Having the new GHT booklet helped to peak their interest.  One physician from the country of Georgia even personally brought Giorgio blood samples to the conference in order to get the free diagnosis services we provide in collaboration with BIRD.  Another physician from Vietnam stopped to personally thank IPWSO for the free diagnosis services.  They are now getting children with PWS diagnosed in their country.

Giorgio and I make a good team for these conferences.  I handle the medical questions, but Giorgio is really the impressive one to watch in action.  He can greet people in almost any language and have conversations in 6-7 languages.  He also knows the delegates in all of the countries, so can discuss that with them.  He also works very hard to get all of the materials together (I send him the new articles we need to add) and knows the details on how to ship internationally – and of course, is renowned for being frugal!  He knows the conference organizers and they treat us well.  We have a great corner location for our booth.  As always, we are not in the fancy hotels with the doctors, but in a cheap one and Giorgio drove 4 hours to fly out of an airport where the flight was much cheaper. 

Iranian Delegate with Giorgio Fornasier

I am sharing this information so you can appreciate the significance of this important education service we offer, and the collaborative efforts between IPWSO, BIRD, and PWSA (USA).  

Warm regards,

Janalee Heinemann, MSW

Vice President, IPWSO

Director of Research & Medical Affairs

PWSA (USA)

Today I went swimming

I don't often go to the pools; I don't like the smell of chlorine, but all this week I have taken my daughter to the pools and watched as she aqua-jogged length after length.  She really seemed to work hard, so today I said I'd brave the water and come in with her.  She was delighted, of course.  She showed me how to put on a body-belt to help flotation, and off she set with me trailing behind. 

She cheerfully announced that we'd do 30 lengths. I quailed at the thought, but had to appear staunch.  She reached the first length and turned to ask how I was doing.  "Ok", I said, "how about you?"  "Oh," she said, "I'm just fine."  After 10 lengths, she was still in the lead.  After 20 lengths, she announced that we could quite possibly reach our goal and do 10 more, "that'd take us up to 40, then we could try 50". 

What had I done to deserve such a hard taskmaster?  She raced me to each end loudly announcing to all and sundry that I was "close to useless".  On and on we went, alternately jogging with feet on the bottom of the pool and then 'cycling', she called it, when it got deep.  I thought it was more like upright dog-paddling.   I was completely out of my depth, metaphorically and physically; there was my daughter, yes, the one with PWS, beating me hollow, with a cheeky grin on her face, laughing as she reached the wall before I did. 

Now, quite apart from burning 915 calories for herself, she also won, big time.  Two other women, also water-jogging, shared her amusement, and I smiled as she hauled herself out of the water ahead of me.  I was the one left in the slow lane this time. 

Here's another plus:  she's been in a good mood since!

A great little book by Kirsty Reid and Peter SW Davies of the CHildren's Nutrition Research Centre at the University of Queensland, called "Exercise and Phyiscal Activity for Children with PWS" is available from our IPWSO office (cost is postage only).  It talks about activities that will help lose weight, how to maintain interest in exercise and to encourage exercise with pre-schoolers, tye types of games, dancing and music-based activites that can be used effectively and there's a wonderful chart which states the calories lost for each exercise, according to the weight of the person and the time done exercising. 

Take time to check this out.  Contact me if you would like a copy:  secretary@ipwso.org

Linda

Keeping Well

Keeping fit and well, for anyone, is lifetime goal just as it is for people with PWS.  As our children start off in life, their general wellness (quite apart from PWS) is pretty good.  Out of the three of my daughters, the one with PWS was, by far and away, the healthiest of the lot and always seemed to keep good health.  As she ages (now 27), however, all sorts of things start to appear, many of these due to the fact that she is overweight (not having had the benefit of GHT) and being overweight means more stress on not just the vital organs in the body, but feet and ankles, hip joints and just general wellness.

One of IPWSO's medical consultants, Dr Susanne Blichfeldt, has put together a comprehensive document on physical health in PWS.  You can see the full article at our website.   It's full of commonsense, what to look for, what is particularly PWS, and how it affects our sons and daughters.  This is not just for the older person, but a very good guide for the health of our younger children.  It includes eyesight, dental care, the skin, tummy problems, hormone deficiency, joint problems, and a really good checklist for parents and caregivers.

While some issues are obvious, such as overweight, needing glasses, skin-picking, maybe fluid retention in the legs, there are issues that are not so obvious like osteoporosis (with up to 90% above the age of 30 suffering from this), adult onset diabetes (Type II), heart and circulation problems, breathing difficulties, sleep apnoea, sleep problems, constipation.  It's really important to have a good check-list so that both you as a parent or caregiver, know what might be affecting the person with PWS, and for the doctor or specialist to know as well.

Besides that, the more the primary caregiver knows what is possible, the better care we can offer!

 

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!