Come and meet Luke...



Luke with Boots



I first met Luke a year ago when I was in South Africa giving some talks and workshops.  We stayed with Luke's family in St Lucia and spent a few wonderful days with them all.  Luke and his brother captured our hearts and soon Nick became "uncle Nick".  Luke's family have been through some tough times, but they are all incredibly resiliant and courageous.  Let me tell you a little about Luke:

Luke is five-and-a-half years old and attends a pre-school in Durban in a class of sixteen.  The school has been wonderful with food security and sticking to a routine - they take time to explain changes to Luke and have made it their policy to always tell the children that if something (an outing or activity) does not happen because of rain etc, they will do something else.  This has meant that Luke is extremely happy and secure.  He does speech therapy, occupational therapy and horse-riding twice a week.

His mother, Janet, says, "He has just started to become more aware of food and tell me he is hungry more often, but I can still keep to our time frames for food.  He has an incredibly memory about everything!!  You dare not say something about what you are going to do, buy, or say, as he will make certain that you do!  He has a wonderful sense of humour, he idolizes Matthew (his older brother) and always says he wants to be just like him."

Matthew with Boots

Let me tell you a little now about Matthew... in Janet's words:

"Brian and I are parents to two wonderful boys, Matthew (13) and Luke (5).  Throughout my pregnancy with Luke we had prayed for a normal birth and normal child, no issues.  We did not ask for an "Einstein" or a "Sports Star" just a normal baby.  You may ask why the focus on this - well Matthew was born with a congenital heart defect and honestly, we had been through the mill with him.  At the time of Luke's birth, Matthew had already had 5 open-heart surgeries, we spent weeks outside of ICUs and Matthew has consequential health and learning issues.

"Dealing with two boys with medical as well as educational issues has been difficult to say the least, but we press forward.  Matthew is an amazing older brother and has immense empathy and understanding of Luke, perhaps because of his own health issues.  He is wonderful with Luke, a real friend and brother to him.

"We are constantly questioning and researching how we can give our boys what they need in the future.  Of huge concern is the lack of PWS-focused residentail care for our children in South Africa, because, let's face it, we will not be around forever and we already know how difficult times can be when we do not have the support we sometimes need.  Brian and I are looking at facilities (and if necessary we will develop one ourselves) and are trusting that we will be able to do something before Luke requires it.

"One thing we are sure of is that we would not be where we are with our boys today without our faith and trust in God to see us through.  Even despite this, at times it has been difficult."

Matthew still faces more surgery and has recently been through another battery of tests and is on oxygen.  Personally, I have taken great strength from this wonderful family and it was such a pleasure and joy to get to know them during our short stay in South Africa.  Janet has allowed me to tell you a little of their story...

...so, she joined WSPA



Thanks to http://www.northrup.org/photos/moon-bear/



As we pulled up to the supermarket carpark (and parked as far away as possible... for extra exercise), the WSPA (World Society for Protection of Animals) posters were plain to see.  There was a sorry-looking bear with a ring through his nose, and a very sad little donkey with liquid eyes just imploring you to do something.  A stronger magnet for someone with PWS you could not hope to choose.  Immediately my daughter went straight for the posters and engaged a rather patient young man in conversation:

"What's wrong with those bears?  Who did that to that donkey?  How can people be allowed to do that?  What do you want me to do to help?"

The conversation became seriously involved with how Moon Bears (named for the moon-like crescent on their chests) are captured, their claws and teeth ripped out, and used for bear-baiting.  They are also killed for their gall-bladders. Although it has been illegal since 1989, it still goes on.   WSPA rescues the bears by intercepting bear-baiting fights, and also by offering better incentives for money earning (raising crops, selling produce etc), and then puts the poor clawless, toothless animals into a special reserve where sometimes a claw or two might grow back, but the teeth don't, so they live on soft foods and fruit.  Francie absorbed all of this, at the same time telling me she was going to join up and she would make sure her budget included the $3.60 (NZD) a week necessary.  This link is a video of what happens - warning, it's very graphic, but it does show the refuge with happy bears at the end.

http://video.google.com/videoplay?docid=7013650950411952306#

Of course, my mind was racing back and forth between the interest she would get through joining WSPA and the fact that her miniscule budget barely allows her to feed her cat and buy some personal items like shampoo, not to mention tobacco (that's just another story).  The nice young man, eager to make a convert, said she could get out of it at any stage, or postpone payments if needed.  Of course, Francie was metaphorically captivated and eager to plant her signature on the contract. 

It always interests me how our kids with PWS see the world - they instinctively know who is worse of than themselves, they have a clear idea of their own values, they have a desire to help, to nurture (and to feed) those who can't help themselves.  This was such an occasion.  It will give her an interest, and a kind of ownership, an authority, if you like, over something that she can do for something for someone else.

On the way home in the car she said (absolutely true to form):  "Well, at least my $15 will go towards some fruit for the bears!" 

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!

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)

Men and Grief

A very good friend of mine (also with an older daughter with PWS) reminded me of the early days, “back when…” and all the struggles we went through to get recognition for our children, to understand the syndrome, and to cope with the grief it brought with it.  That got me thinking about grieving and how it’s so ongoing with PWS, but as time goes on you tend to pick yourself up and carry on.  That reminded me of an article a father once wrote and submitted for a magazine I was editing at the time, and I thought it deserved a further look.  He wrote:

“I had been looking forward to the birth of our first child.  For months I had been fascinated by her movements inside her mother; at the beginning little fluttering and then as the months passed, stronger, more vigorous movements.  It was exactly what one would expect of a strong, healthy baby.

“I was present at my daughter’s birth, an experience that defied words.  All stories and films that had the birth of a child in them made much of the initial birth cry.  I was absolutely unprepared for our experience: she arrived without a sound.  I have a clear picture of the doctor holding her up, her little mouth shaped like a cry, but there was no sound.  I am unable to describe what came over me at that moment.  Words like “shock, disbelief” do not even hint at what I felt.

“My overriding reaction was what I can only call resignation; whatever would be, would be, and I had no power whatever to change any of it.  I was devoid of feeling.  That stayed with me for months.  The only thing that broke through my lack of emotion in those early days was a kind of subdued elation when our little girl would actually swallow milk from an eye-dropper which was the only way we could feed her.  Eventually our efforts weren’t enough and she had to be tube fed at hospital.

“At work, I was an automaton.  I have little recall as to whether I was happy or sad, warm or cold; I had no appetite.  I used to enjoy a drink.  I never tasted the stuff from this time for months afterwards.

“As the hospital, testing continued to determine what the condition might be; I became aware how differently my partner was responding.  She saw all tests as a challenge.  I was indifferent to the various tests and as each eliminated this or that possibility, she saw all these as major victories.  The diagnosis at this stage was ‘benign hypotonia’.  I was conscious that I was unsupportive.  My partner’s hopes were constantly undermined by my scepticism, actively negative, and yet I could not break out of the “what will be, will be” mindset.  While my wife wanted to talk about progress and development and testing, I just wanted to be left to myself.  I had closed off – little outside myself held any interest.  I ‘escaped’ into Wilber Smith.

“A friend was a good listener, but I could only share at a superficial level.  Talking was not helping as the overriding feature of ‘all this talk will not change anything’ undermined any chance of healing.  

“The second stage was marked by my waking one day and noticing the sun was shining.  I felt warm for the first time in months.  This time pin-pointed another stage: anger.  Wild mood swings, optimism (that our little girl would be ‘normal’ that she would outgrow this strange condition), followed by depression caused by the uncertainty of what the condition would mean long-term.  I cried more now – most nights.  Sleep was full of curious dreams and I would wake up exhausted.  But there was less of ‘what will be, will be’.  By now our little girl was drawing me more and more into her life.  I felt I was able to share more of my partner’s hopes.  The future seemed much less fixed.

“Eventually our daughter’s strange condition was diagnosed as Prader-Willi Syndrome.  It took me a long time to accept this.  I read all I could about the syndrome, the information coming from my partner’s research; a book here, a paper there, an article somewhere else.  After a time, I noticed a shift in what I was feeling.  The crippling resignation had given way to a hope that what was now did not have to be in the future.  My wife was already planning counter-measures for our daughter to run against the classic symptoms – extra help for learning, exercises to firm up the little muscles, games to keep her stimulated.

“Sometimes it seems a long time ago; more often, it feels like yesterday.  A kind of emotional evenness came when she was five and a wholeness at seven.  The anniversary is still hard, but my partner eases me through it each year.

“Yes, we men do cope with grief differently.  Education should enable us to cope better.  I hope so.”

 

Contributed by Linda Thornton

Let's talk about sex

In the past we swept the subject of sex under the carpet.  We knew that women with PWS menstruated infrequently, if at all, and that males were infertile.  Also, in those days, sex hormone therapy for males and females wasn't really an option, so the whole chapter of sexuality was closed for many years.  It was rudely opened in 1999 when the first birth of a healthy baby girl to a woman with PWS was recorded in Sweden.  This was followed in 2001 when a 32 year old woman with the deletion diagnosis was recorded as having given birth to a baby with Angelman’s Syndrome, in Denmark.  The third recorded birth was in New Zealand in 2004 when a baby girl also with Angelman’s syndrome was born to a young woman with a genetic deletion diagnosis.

Sex hormone therapy is now recommended for both males and females with PWS.  This “encourages the development of secondary sexual characteristics and potentially improves bone mineral content and density”

http://books.google.co.nz/books?id=Js81Pr1PmaAC&pg=PA171&lpg=PA171&dq=women+with+PWS+given+birth+to+child&source=bl&ots=WdkaahgMa7&sig=qYzjIz18-NjJaRKkzGGd5nKB8t0&hl=en&ei=kXERTsybAtHPrQeU-oCIBA&sa=X&oi=book_result&ct=result&resnum=4&ved=0CCsQ6AEwAw#v=onepage&q&f=false

It will also induce menstruation and the likelihood of pregnancy.  This means greater observance of lifestyle, good sex education and support, as well as a better understanding of how to cope with the resulting birth.  It is also recommended that males have testosterone therapy, and many youngsters are on growth hormone therapy and may well continue to be on GHT into adulthood.  Combined with sex hormone therapy, could this mean an increase in fertility? What about sex itself?  Marriage, even?   

Back in 1999 I visited a residential home in Seattle where a couple (both with PWS) were engaged to be married.  I asked the young man why he wanted to marry, and his reply has stayed with me ever since…”because I want to see her in the shower”.  I don't know whether they married, or whether they just lived together.   I know of another couple in Australia, she with PWS and he, not, who married.  I don’t know the reason behind that one!  

It used to be thought that marriage was too serious a contract for a person with an intellectual disability to enter into and that a person with PWS would be likely to walk out at the first sign of a melt-down.  Same as it was thought that a person with PWS should not drive a car as they would probably have road-rage.  However, I know of two people who have their driver's licence and have held them for quite some time, content to potter around their local town on their own.

Many of our young people want to experience a close relationship to "have" a boy or girlfriend, and nearly every young woman with PWS that I have met expresses a desire to have babies, to look after babies and to have them to cuddle and hold.  Most have a fine collection of dolls, or soft toys and many are genuinely wonderful with small children and babies.  However, I know from talking with the young woman's family in New Zealand, that there were many difficulties involved, even from the beginning when no one knew she was pregnant until a visit to the doctor in her last trimester brought the surprising news.  Birth was by caesarian section as they did not think the mother would be able to tolerate a natural birth.  The mother was overjoyed to have her own baby, but not so overjoyed when the baby cried and interrupted her dinner time.  Neither did she prove a natural mother able to care for a small baby or answer its constant demands nor did she express great interest or maternal instinct.  


What she did want, however, was for her baby to grow up quickly so they could live and travel together.  The baby has Angelman's syndrome and is cared for by the grandmother and sister while the mother is in residential care.  She does have regular contact with her natural mother, but the daughter's relationship, as one might imagine, is closer with her primary caregivers.


Somewhere along the line there has to be an extra element of responsibility and care from parents and caregivers alike.  There still needs to be educated and intelligent management and support around such important decisions as choosing to share your body or life with another.   There have been many changes in the way we care for and manage the lives of those with PWS, and doubtless there will be further improvements as we understand the needs of those with disabilities.  


At the end of the day, I expect every parent will share the same thought, regardless of whether there is a disability or not; "I just want my son/daughter to be happy", but let's make sure we know what they want, and how we can ensure that happiness and support.

Perspective on Challenging Behaviours

I came across an article on tantrums in children the other day.  It’s on the website: http://www.betterhealth.vic.gov.au/bhcv2/bhcarticles.nsf/pages/Tantrums?open

 

It described tantrums as being “a young child’s way of physically expressing feeling such as anger, frustration, hurt, and being upset.”  

And this has always been the way that I have seen a person with PWS expressing him or herself when all else has failed.  When something goes wrong and she is unable to explain what is happening, or that she doesn’t understand, or I don’t understand, my daughter with PWS will often revert to having what is described as a tantrum, or rage, or outburst.  It is frequent in PWS and is one of the deciding characteristics of the syndrome, and it doesn’t matter how old they might be, when they get to a certain point, this child-like reversion to tantrum behaviour is the only way they can express themselves.

It is important for us as parents or caregivers, teachers or residential providers, to understand that this is basically another form of communication when all else has failed.  The article goes on to say, “A tantrum is a young child’s way of physically expressing feelings such as anger, frustration, hurt, and being upset.”  And this is exactly what is happening to those with PWS.  It is also frustration in not being able to be understood, or to actually understand what is required of them.

In the online article, tantrums are expressed in the young child as “crying, screaming, punching, kicking, foot-stamping, running away, or going limp like a rag doll, throwing or breaking things…”   How very like a person with PWS this is! 

The young child “does not have the cognitive thinking abilities to plan their tantrums or use them to upset parents."  Neither does the person with PWS.  “Tantrums are simply a physical expression of the child’s feeling”.  Again, the same explanation for those with PWS.   The rages that can evolve from something that has triggered them off, are not planned in advance – they just ‘happen’ and often come out of nowhere.  The stressors for children are often “feeling tired, hungry, frustrated, overstimulated, or feeling stressed”.  Again, something we can easily recognise in PWS.

Of course, the major difference is going to be that tantrums are a normal part of child development and that children grow out of this as their communication levels get better and they feel more comfortable in themselves and in the world, whereas in a person with PWS this so often doesn’t happen.  The tantrums continue and are re-labelled ‘challenging behaviours’.  We must realise that just as tantrums in a small child are not ‘bad’ or especially naughty, so they are not in the person with PWS.

Recognising the triggers likely to set off a tantrum is the answer to successfully avoiding the behaviour.  Of course, we are not always so clever that we recognise the triggers each and every day, and neither are we born experts in the psychological management of avoiding difficult behaviours, but if we can start to understand the reasons behind the behaviour, then we are halfway there.  It requires great patience and control, often two things that, by the end of the day, we have totally used up!  This is when you mutter under your breath the prayer for serenity (only I call it the prayer for sanity):

God grant me the serenity

To accept the things I cannot change;

Courage to change the things I can;

And the wisdom to know the difference.

...and pour myself a glass of good wine.

Critical Parenting

There comes a time in parenting when you sometimes start to look at your son or daughter with a critical eye.  Are they shaping up the way you expect them to?  Are they a good reflection of the way you brought them up?  Will they be strong enough not to be weakened by corrupting influences around them?

We all do it, because we all want the best for our children.  Regardless of whether the child has special needs or not.

However, many of us are extremely critical of ourselves and set such high standards that others seem to fall by the wayside, inviting our criticism.   Often we seem to get more critical as we get older—life just isn’t ‘what it used to be!’ 

Having a child with special needs can sometimes make us even more critical, not necessarily outwardly, but inwardly as we compare our child’s “failings” with the next person’s child. 

By being critical we can build up a wall of protection against others—striking first before we are criticised.  There’s nothing wrong with being protective—heaven knows, it’s what we parents are best at… but criticism also has a darker side.  Ron Potter-Efron’s book, Stop The Anger Now (New Harbinger) says that ‘being critical of others:

• Helps me feel superior/dominant
• Convinces me that I’m right and you’re wrong
• Helps me avoid noticing my imperfections and faults
• Keeps me in control by making others appear weak, dumb or bad
• Is similar to what I do to myself.  I’m very critical of myself.
• Keeps me from getting too close.
• Is my way of trying to help, protect or guide others, even if they don’t like it
• Feels good

He says, “too much criticism of others can make you mean-spirited and cynical”.  Cynicism is the enemy of optimism and happiness.  It makes us aware of how easy it is to give in, give up.  Parenting a child, or even an adult, with PWS can often have this reaction.  Tiredness sets in, cynicism takes over, and the thought of another year with all the same battles just seems to be not worth getting up for. 

One of the best resolutions (New Year's or just today's) we could ever make is to be kinder to ourselves.  If we let go some of the expectations that come with ‘critical’ parenting, learn to appreciate and enjoy the smallest of achievements, we can re-charge our batteries and set different goals.  Goals that aren’t bothered by what the next person is doing or achieving; goals that aren’t rooted in materialism, or comparisons. Achievements that are as uncomplicated  as helping your daughter get her balance to slide down a gentle waterfall—just like everyone else.

Potter-Efron suggests, “make a goal for the next 24 hours to notice as many good things about the world and about other people as you can—do this every day for a month”.

It’s good advice for the weary and the cynical!

                                           

Residential Woes

Residential care is one of the most important things we need to think about for our children as they reach adult-hood.  This is something that IPWSO has taken very seriously, and, after two international conferences specifically designed for caregivers, a fantastic resource has been developed, "Best Practice Guidelines for Residential Care".  I wish this resource had been available when my daughter (now 27 yrs) first went into residential care. 

She has been in residential care since she was 18 years old.  At first, I thought she could manage in a small house by herself (with 24 hour staff care), but somehow this didn't work out.  Everything that could go wrong, went wrong.  She wanted to cook for herself (with supervision) and I taught her how to stir-fry food, something that was quick and easy.  But on many occasions she burnt the food and had to cook more (thus depleting the weekly allowance).  On other occasions she blamed staff for taking food from her fridge & cupboards.  She wanted to entertain and cook for friends, family and neighbours; she wanted to to try new foods, new recipes, and to do her own shopping.  After 4 weeks of this, we changed the rules.  Food would be cooked outside her house and brought in daily.  I did this myself for a number of years until staff took over and provided meals cooked off-site. 

Because meals were now controlled, she took to pan-handling in the neighbourhood.  She was extremely opportunistic and found money very easily (staff learned quickly not to leave cars unlocked, handbags unattended) and was blamed for missing money (creating denial melt-downs).  She executed brilliant reasons for needing hand-outs, money, cigarettes, extra food and so on.  One of the best was that she was a "solo mother with 3 children under 5 years old - one with a broken arm, the other who wet the bed every night, and the third who was just staring school.  Their 'father' looked after them during the week, but dumped them on her during the weekend and she had no food to give them."  When this ruse was discovered, "Elder Abuse" had already been called in as she was going around to not only choose the food from the cupboards herself, but cook it at the neighbour's house.

We shifted her to another house (still solo, but with 24 hour care) where there were no shops close by, but the neighbourhood was not great and neighbours were bullies.  She also managed to give her staff the slip and find new avenues of food, money, and, now cigarettes.  Six months later, we found another house with a beautiful little garden, no shops, and on a corner, so only one neighbour.  Again it was staffed night and day, but by now, my daughter really did have the hang of how to play staff off against one another, frighten them, and bully them.  There was a serious complaint laid, police involvement, and a court decision that she was not able to live in the community under the current circumstances.  She was moved to a very secure place where everything was locked down, and where there were a mix of clients who had also been through the courts.  None of these clients had PWS; she was the only one.  For her, to live with other people under such strict rules, was a big wake-up call.  This lasted a year, with a transition farm-house for the last few months.  I was proud of the way she had 'learned her lesson', but not proud of the fact she had put on a lot of weight.  This last home was full of big, strong, men who ate big meals.  She was happy about that! 

During the year she was there, I spent a great deal of time searching for providers for when she got out, who would be prepared to abide by the latest IPWSO Best Practice Guidelines for Residential Care.  I spent a long time combing the Best Practice Guidelines for the best ways they would fit our New Zealand culture.  I changed a little, but not a lot.  After some months of talking to providers, we chose one who seemed very enthusiastic and ready to take on the challenge.  We put an extreme amount of training and support into the staff (much from the IPWSO Best Practice Guidelines) and brought in two clients with PWS (one, being my daughter, and the third is without PW) and staggered their arrival over a period of 2 weeks, and sat back to watch.  The honeymoon period lasted another 2 weeks and these girls had the staff exactly where they wanted them.  Basically, as slaves!  They would sit around all morning in their jamas, ordering cups of coffee, expecting breakfast when they deigned to get up, morning tea, lunch, and so on.  No, they didn't want to go out and exercise; no, they weren't interested in showering, or getting dressed...

Back to the Guidelines and the application of more rules.  Exactly as the Guidelines suggests.  Staff learned to call the tune, put rules in and around everything - if the girls weren't up by a certain time, breakfast would be cleared away; if they didn't walk, swim, exercise, there would be no priviledges.  There followed a couple of weeks 'shake-down' period where staff stood their ground, and finally, the girls got used to the idea that routine would be followed and priviledges earned.  Things are now going much better as staff have quickly learned to cope with the cleverness of two young women who know their way around.  Some staff were worn out quickly, some handled it well.  There have been tough times, as well as the funny side of life. Best of all, they are happy and losing weight!

It was a risk, introducing a new service provider to the world of PWS, but one that has been well worth the taking.  Having staff interested in the syndrome, willing to be helpful, and willing to be forgiving over minor transgressions (particularly the swearing) has been paramount to this home's success.  Things won't always be easy, and the good old learning curve will always be steep, but by following the Best Practice Guidelines right from the start everyone has had a chance work together.



Would you like to holiday in the Dolomites?

Other than the very rewarding work of being on the IPWSO board, is the great pleasure of finding out where in the world we are next meeting!  Our board meets once at our three-yearly IPWSO World Conferences, and, generally, once inbetween.  Although it might sound extravagent, we generally manage to get support with our venue and accommodation, relying on wonderful international friendships.  This year we met in Giorgio's (our CE) home country, Italy, a couple of hours north of Venice at Laggio di Vigo di Cardore, way up in the Dolomites.  While we were here, we learned that this wonderful place is offering family holidays for special groups... and we're included!

Casa di Laggio high above the village



This wonderful old 'holiday house' used to be a sanitorium for those with TB, but is now used as a holiday place for those wanting to experience the great walks, ski-fields, and explore the beautiful mountains. 

This year will see an enormous upgrade of the cassa with complete refurbishing which will encourage groups to visit and stay.  In this regard, IPWSO will be organising family holidays for anyone with PWS and their families.  Can you imagine a more perfect holiday?  And we will be able to offer a very reasonable family rate!  The menu will be designed for those with PWS in mind, there will be activities for children, and time to relax for parents.  Interested?  Watch this space.... More on this later! 

 

A Note from Japan



Description=Yachounomori Garden, Tatebayashi-shi(city) Gunma-ken(Prefecture), Japan [http://www.sibazakura.com/ 群馬県館林市 野鳥の森ガーデン] |Source=[http://www.flickr.com/photos/13910409@N05/3456398565/ Sakura and Moss P)



Wikipedia states: Every year the Japanese Meteorological Agency and the public track the sakura zensen (cherry-blossom front) as it moves northward up the archipelago with the approach of warmer weather via nightly forecasts following the weather segment of news programs. The blossoming begins in Okinawa in January and typically reaches Kyoto and Tokyo at the end of March or the beginning of April. The beautiful blossoms are enduring metaphor for the ephemeral nature of life. Cherry blossom is an omen of good fortune and is also an emblem of love, affection and represents spring.

From Eiko Shoji comes the following message to all our IPWSO family:

"Thank you very much for forwarding messages from the IPWSO members to me. I learned that our friends all over the world were praying for us. Certainly their prayers gave us strength, and were answered.

I'm really grateful to you all being so thoughtful and helpful for us this time. I think Japan will still have to go through a difficult time for some time, but it is a great encouragement to know that our friends are always with us."

Eiko also told us that all their "PWS families" are now accounted for and are safe!  We are so happy to know this and we hope the beauty of the blossom can bring hope for new life for Japan.