Eating our way through the holidays...

So... we are coming up to Christmas, or Hanukka, or just holiday time, and our kids are either off school for weeks, or coming home for the holidays and we experience that panicky feeling of "how can I make this go right!" 

Janalee Heinemann has written some great Holiday Tips which I thought deserved repeating here...  In fact, I would go so far as to suggest you print this out and hand it to all members of the family well in advance.

The holidays are typically a food fest in many countries – and can be a time of stress for our PWS families.  With good planning, it is possible to make it a happy holiday for all. 

Christmas, Hanukkah, Lunar New Year, etc.  

·        If you will be with relatives, carefully plan ahead of time and communicate the importance of food control with all involved. Make sure all attending know the “rules of engagement” and agree to cooperate.

·        See that someone at all times is clearly in charge of your child with PWS.  Clearly define when you are “changing guards”.  As Dr Linda Gourash states, “When everyone is in charge – no one is in charge.”  

·        If your child is old enough, rehearse the “rules” before the special  day and come to a mutual agreement on what your child will be allowed to eat.  You can barter, i.e. “Do you want a little extra turkey and dressing, or do you want a piece of pie as your special treat?”

·        It is okay to request that Grandma and other relatives tuck away tempting items during your visit and to discreetly check with you prior to offering your child a treat. 

·        Make sure you know what everyone is bringing, so there are no surprises on what the choices will be. 

·        Grandpa and Grandma, or aunt and uncle may want to bring a special gift toy to compensate for the food they have to deny your child. 

·        Go over with the hostess or your family on how to contain the accessibility of food.  See to it that where your child is sitting there will not be a lot of bowls of food, rolls, or condiments nearby (many people do not consider how many calories our children can consume with the extras – sugar, butter, catsup, etc.) 

·        After eating, when people are just visiting, see to it that if the food cannot all be put away, someone is responsible for guarding it. 

·        Your child must have the security of knowing you will be strong in your commitment to keep them protected from food – in spite of themselves.  Giving in, even once, means several battles ahead.  I know you get tired of hearing it, but consistency is the key. 

Of course, each family must judge their own situation based on their child’s food drive and their own regulations on treats.  Some families are raising their children to never have any sweets – no exceptions.  Others (like ours) just go by calories and the weight of the child, trying to keep the diet less in quantity yet similar to others in variety.  Often, the most important thing is to prevent food sneaking or food demands.   There is a large variance in the food drive of children with PWS.  Some will ask or beg for more food, but make no significant attempts to sneak food.  On the other hand, some will go to great extremes to get food, and are incredibly clever at doing so. 

HOLIDAY WARNING        

The holidays have an extra risk factor for our older children and adults with PWS.  In the USA, four individuals with PWS were reported to have died of gastric rupture and necrosis. Furthermore, 4 additional individuals were suspected to have gastric dilatation and perforation, but without autopsy evidence. Some of these were over the holidays or special events and due to a food bingeing episode that led to necrosis (deadening of the tissue) of the stomach wall and a perforation (tear) in the stomach.  In most of the deaths, the person with PWS was relatively slim, so there was no great concern about weight gain.  Keep in mind that a person with PWS who is slim still does not have total food control.  When one also has many opportunities for food ingestion, the lack of feeling full, the high pain threshold, and a weak vomiting reflex – then one has the potential of filling the stomach dangerously full.  Because there are many food bingeing episodes of our children and adults with PWS, most not having such disastrous results, we think there are probably other factors that play into this life-threatening situation that we are currently researching.  One hypothesis is that due to prior food binges, and stomach muscle weakness, certain areas of the stomach wall become thinner putting this area at risk.  

Please see that this holiday season, the safety and security that your child deserves is provided. 

A small step for the world - a big step for Macedonia

Norbert Hodebeck-Stuntebeck

In July 2010 Valentina Kostoska – a mother of a 12 year old girl with PWS – send out an email to IPWSO which read “We need help for our daughter Emanuela with PWS! - and we have no other source to ask than you.” Her health situation had come to a critical point and also her behavioural problems overburdened the family.

Giorgio Fornasier – as the Executive Director of IPWSO – sent an email with the question “Is there someone in Europe who can help this family?  Can you help?” to Norbert Hödebeck-Stuntebeck, Chairman of the Professional Providers and Caregivers Board of IPWSO.

Norbert works for the Diakonische Stiftung Wittekindshof,  an institution in Germany for people with intellectual disabilities with special places for people with PWS (children and adults). When the directors of this institution were informed about the situation they gave a very positive signal for being interested to develop support for the family.

After the first contacts between the family and Norbert, and finding financial support, Emanuela and her parents were invited to stay for a week in the group home for children with PWS in Gronau, a facility of the Diakonische Stiftung Wittekindshof in Germany.

The idea of this first week was like a “test” - to find out:

- if it could be helpful for Emanuela to stay for a longer time (half a year) in the group home

- if the family gets the feeling that it could be helpful for Emanuela and

- if it could be acceptable for the family (Emanuela had never been without her mother before)

- if the others with PWS in the group home could accept the situation

- and how it would work with the language barrier (Emanuela didn´t speak any German at this time)  

In November 2010 Emanuela, her mother and Deci  – an engaged medicine student, and friend of the family in Macedonia – stayed for a week in Gronau. Emanuela in the group home, her mother and Deci in a hotel in the city. During this week Emanuela had some very positive experiences in the group and participated in the school. Her mother and Deci used the time for to learn more about the syndrome from both the staff of the residential home and Norbert, mostly about behaviour management, nutrition management and all-day care.

At the end of the week there was a meeting with all participants (Emanuela, mother, staff, leader of Gronau, Norbert) to decide what can be the next step. All agreed that the week was very successful and also Emanuela was interested to come back. So the decision was:

1. Emauela should come for 6 months to the group home with the main goals of a positive development of her health situation and her social behaviour.
2. There must be a development of a support system in Macedonia, so that after the 6 month the support for Emanuela carries on and also for other people with PWS in Macedonia.

And so, the idea of a PWS Network in Macedonia was born!

The main question after this decision was: how to find the money for the 6 month treatment in the group home in Germany. And how to develop a PWS network in Macedonia.

Six month later there was success with the funding;  50% of the needed money came from the health system of Macedonia and the other 50% were given by the Diakonische Stiftung Wittekindshof as a sponsoring of this project. So there could be the offer to Emanuela to come to Gronau in June 2011 for 6 month.

During the time after her first stay in Gronau (November 2010) up to June 2011, when Emanuela was back with her family, two very positive things happened:

-          Emanuela lost nearly 10 kg and

-          her behaviour problems decreased.

-         

The explanation for this by the family was that they tried to work in the way they had been taught by the staff at the group home in Gronau. This development gave everyone the confidence that the project would be successful.

During the time from June 2011 to December 2011 when Emanuela stayed in Gronau, the family visited her twice and were in phone contact sometimes every week.

Once a week or when necessary, there was a translator so that Emanuela or the staff could explain things Emanuela seemed not to understand.  But it was really interesting how fast she started to understand the routines, the schedules and the language. At the end she was able to speak the most common things in German and understand most of the things people told her

.

Other important results of the 6 month are:

–        the positive development of her social behaviour

–        the increased acceptance of her role in everyday life (in the group home and in school)

–        the decreasing of behaviour problems (crisis situations)

–        her competence and knowledge about nutrition (“What can I eat, what not”)

–        and she lost another 10kg during this time

At the end Emanuela was asked if she wanted to stay longer in the group home in Gronau or to go home and her answer was very clear: “I want to go home  -  but I like you too”.

Also the professionals who took care of her over the six month made the same decision: “she learned so much and now it is time to go back into the family and implement these new things there – because in Macedonia is her family and her future”.

At the same time Emanuela was in Gronau the activities to build up a PWS network in Macedonia were going on. Norbert come in contact with Dr. Elena Shukerova-Angelova (Pediatrican and Endocrinologist)  and Dr. Aleksandar Sajkovski (Pediatrican and President of the Pediatric Association of Macedonia). Together they developed the first step, a lecture by  Norbert about the main characteristics of PWS during the Pediatric congress of Macedonia in October 2011 in Ohrid.

This gave an opportunity to  more than 600 pediatricians all over Macedonia to learn about this rare disorder and discuss the concept of having a first conference solely for PWS in Macedonia.

30 November 2011

IPWSO is so proud to have people like Norbert, and institutions like the Diakonische Stiftung Wittekindshof who able to carry out such great international cooperation between families and organisations worldwide on behalf of people in the world who have Prader-Willi Syndrome.

What if we all lived 'til we were 100?

Yesterday I went to the funeral of my second cousin.  She was 100 years old, plus 3 days.  It's pretty rare these days to make a century, let alone to die quietly of old age.  Although statistics tell us that we are living longer, we seem to be dying less of old age and more of debilitating and terminal illnesses.  Our Western lifestyles are quite different from the days when my cousin, Mary, was young.  By comparison, we are pampered, live cushy lifestyles, expect and demand more, some earn ridiculous amounts of money and become immensely rich, some lose everything in life's lottery and are condemned to live in poverty.

And some of us have children with Prader-Willi Syndrome.

One hundred years ago, there was no such thing as "Prader-Willi Syndrome".  It may have existed, but had yet to be described and doctors Prader, Willi, and Labhart had yet to make their entrance on the world's scientific stage. Autism was only described as a particular condition in 1943, and 28 years after Mary was born, penicillin was discovered.  Transport was usually by horse and carriage, certainly for Mary, radio and hand-written letters were the means of communication.  Nothing was as instant as it can be today.  Every household chore was labour-intensive, food was plain and wholesome, visitors didn't just drop in, they stayed sometimes for weeks.

Child mortality in Mary's day was common.  The vast majority of children born with what we now call "PWS" would have died, just faded away through lack of nutrition.  Any who did survive would later die from obesity related illnesses.  We are lucky today that our knowledge over the last 100 years has become very informed and through medical science and research we understand complicated disorders such as PWS.  Even if it takes us another 100 years, our work is to continue to push the boundaries, to advocate for our children and adults with PWS, to break the barriers... together.  We must keep sharing our knowledge, working together, parents, caregivers, teachers, and the medical profession; for scientists and researchers to share their learning so that we can all work from the same page.

One of the best ways of doing this is through our PWS conferences, and by sharing what we have learned both in theory and practice.  Sweden, Denmark and Norway PW Associations are about to celebrate their 25th anniversary, IPWSO will celebrate its 8th international conference in 2013; and the world will celebrate the Emerald Anniversary of 55 years since the naming of Prader-Willi Syndrome. Fifty-five years ago, Mary was 45 years old.  She had raised 5 children and was matriach on a large sheep station.  She'd lived through two world wars, known hardship, sadness, and great happiness. 

We each have our stories to tell, and although Mary was unaware she would live for a full century, what an amazing time she has lived through!  And how lucky we all are that during her lifetime, doctors Prader, Willi, and Labhard also lived. 

Money, money, money, it's a rich man's world!

Oh, thank Goodness!  All my money woes are solved - this week I have been given $1.5 million from a TNT Director, and $2.5million from the Blackberry Internet Promotional Draw (actually they had to remind me twice about picking this up), then there was this lovely chap who wrote to say that this firm had the best money-lending rate I'd ever hope to find.  Last week was the same, Sani Mohammed wrote to me from (wait for it) the G20 meeting held in France recently to say that they had nothing better to do than hold a ballot for lucky beneficiaries and this time I'd won $1.2 million.  On the very same day, as luck would have it, the United Nations of International Oversight Services emailed me to say I had a compensation payment of $9,000 (but they wrote in words alongside that it was actually nine thousand, five hundred American dollars).

I'm so happy about this, and, although I'm really sorry for Miss Elena Kojawa's terrible loss of her father in the Ivory Coast, I'm utterly overjoyed to think that she has thought carefully about the 8.5 million dollars her father left in a bank deposit and is prepared to offer me 15%.  A local bank has emailed me to say I have a tax return of $173.50 to pick up, but since it's such a small amount, it's hardly worthwhile bothering about, is it, really?

Now... I know this is all a huge joke and it amuses me no end to receive these spam emails, but the truth of the matter is that in these uncertain economic times, the hardest hit are the charitable organisations all around the world who are desperately trying to continue their work with very limited budgets.  Understandably there are many of us in the same boat, and IPWSO is just as hard-pressed as every other charity to find an income source. 

The difficulty for IPWSO is that it is a global charity, it is not operating in just one country, but world-wide.  That means applying for funding is even more difficult as most funders prefer to see their money spent for the benefit of their own country.  IPWSO has to rely heavily on donations from people like you and me; and from our member countries.   This month we are going to celebrate our Emerald Anniversary - it is 55 years since PWS was first 'discovered' by Professors Prader, Willi, and Labhart.  We are using the symbol of a bridge, with candles to help light the way.  Each time we receive a donation another candle will be lit.  We are looking forward to presenting you with this creative concept for fundraising, and we are hoping that, unlike our Spam friends above, you really will be able to help us cross that bridge!

Training the puppy

Recently we got a new pup and she was everything a pup should be: fun, pretty, cuddly, and very naughty.  She was into everything and anything, she chewed anything, she'd drag things around, and, of course, she'd pee.  Anywhere, everywhere and any time.  Just like any pup.  Eventually once she got to know the system, and once we got to know her better, she started to settle down. 

Just a few weeks ago, I decided to hire a personal puppy trainer.  Kind of like a puppy whisperer...this wonderful woman came out to our place and for three hours she trained both me and the pup.  Both of us were trained to respond to good behaviour, and both of us were rewarded, the pup with something she really liked (in this instance liver treats) and me with the knowledge that the pup was learning to respond to me and respond to commands that would help both her and our family live together in harmony.

The essence of the puppy training was to reward good behaviour and ignore the bad.  Each time the pup leapt up onto the trainer, she would turn aside and ignore.  Once the pup stopped, she was rewarded with a 'key' word (in this case it was simply the word "yes!!"), and given a small treat.  Same with learning to come when called, to sit, lie down, and to walk nicely on the lead.   Later, my trainer told me, we could cut out the treats and just reward with a kind word and petting.  Within 3 hours, the trainer had both the pup and me following a very successful "ignore, redirect, and praise" routine.

On the other hand, elsewhere this week I also saw a young horse being trained to walk quietly and behave without constantly dancing around on its hind legs.  The person doing the training spent much time jerking the horse's head down and flapping a plastic bag in its face.  After watching this for an hour, with no change in behaviour in the horse, I said to my friend, "where's the reward for the horse's good behaviour?"  She said, "well, it doesn't get jerked around!"  Hmmm.... I thought.  I wonder if the horse knows that's the reward?

I bet you're beginning to see where this is going? 

And you'd be right if you said it sounded a lot like working with people with an intellectual disability.  Or maybe you said it sounded like raising your own child.  You'd be right on both counts.  It's such a simple and successful method: ignore the unwanted behaviour, redirect to the preferred behaviour, and immediately reward the new 'good' behaviour.  It's so simply that it almost gets lost in the mountains of information on behaviour management that's available these days.  Of course, there is a huge amount of really good stuff about behaviour that you can learn and apply, and many experts have added to the excellent resources available, but the core to it all, the base on which to build a good relationship of care, trust and understanding, is this simple premise - reward good behaviour whenever you see it, wherever you are. 

Ignored and Forgotten?

This week I have been staying with my mother-in-law helping her come to terms with a particularly debilitating and progressive illness.  It's been my job to coordinate help and care from the agencies - both public and private - and to tie it all together in a way that will help and support her.  It has been a salient reminder first of our own frailty as we face the aging process, and secondly the similarities in the way we want the best care for our elderly and for our young adults with PWS.

Although there are some similarities in the care process, there are also yawning gaps in both groups.  First is the cost of care for the elderly to be cared for in their own homes; over and above what the government can offer, it is expensive without a doubt.  But getting the best care is what we want, and although there are many free services of help health-wise; having someone to live in the house, caring, assisting, cooking meals, running errands, going on outings and making life enjoyable and dignified, needs to be recognised financially if it comes outside the realms of social welfare.  The other option is to live in a sheltered residential environment, or retirement village. 

Our young adults with PWS are just as vulnerable and, quite often, medically frail, as the elderly.  We also want to make their lives enjoyable and to give them dignity and a reason to get up in the mornings.  In most of the Western world, residential placement for those with intellectual and physical disabilities is provided by the government.  Some are really wonderful with kind and caring staff.  Others leave something to be desired.  I expect it is the same with different retirement homes.  The vast difference is, of course, that as non tax-payers, our young adults can't be expected to pick up the tab for more personalised one-on-one care.  And agencies are not able to provide more than their budget allows.

Caring for the vulnerable, be they elderly, or disabled, is not a job that many people choose.  In fact, it takes a very special kind of person to do this, someone who cares for, respects, and really understands the person they care for.

My question is this:  Do we value our elderly and disabled enough to be able to give them the choices in life that they deserve, or are they still an ignored, forgotten part of our society?

A little Ned Kelly?

Any Australian worth his or her salt will know the story of Ned Kelly, the Irish Australian bushranger who was outlawed with a huge bounty on his head.  He was the one who wore the 'tin can' on his head as protection, as his image, and his disguise.  He was the one who never gave up, who fought the system with strength and daring.  He is an Australian icon, indeed.

So, let me introduce you to another young Australian who, like his namesake, will battle the system and never give up, and will always have a band of loyal followers ... meet Tobias Arnold Ned Kelly, born on February 27th, 2011 in Canberra.  Anxious to arrive in the world, he made it 6 weeks early.  Diagnosis came  5 weeks later when a chromosomal study was done straight after his birth. 

His parents, his brother Clancy, and sister, Grace, live four hours away from Canberra in southern New South Wales.  His mother, Maria, is a photographer (as you can clearly see!). 

Tobias spent 6 weeks in hospital, he was fed with a nasal gastric tube for several months, although now at 7 months, he is tolerating solids pretty well.  A sleep study is going to be done so that he can start on Growth Hormone Treatment.  Maria says, "Tobias is an extremely placid baby and we are enjoying his brief giggles and smiles, now that he's a little older.

"His sister, Grace, and brother, Clancy, dote on him daily and he recognises their voices and presence with his gorgeous eyes."

Maria is keen to link up with parents in Australia, and around the world.  Networking and sharing stories is one of the most powerful supports you can offer another parent, so I hope you will contact her via our Facebook pages.

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)