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.