In August, what happened was....

All around the world there are families needing our support.  Emails come in continuously - not always with cries for help, but quite often, so I'm going to outline some of the issues we helped with internationally in August.   For instance...

Qatar: A mother with an 11 year old son from Doha contacted us asking for support in maintaining her son’s dietary needs and physical activities.  While her son had been diagnosed at birth, he was unable to have GHT due to a sleep apnoea problem. This mother was asking for information about any centres run specifically for PWS where her son could get some help.  Our office in Italy sent all the relevant packets of information, both in Arabic and in English, including DVDs.  Of course, even though we are a big family of many countries (98 to date), there is not always immediate help at hand, so it was with some hope that we contacted Germany and asked our professionals there at the Witteskindhof centre  whether they might be able to help.

You may remember reading our blog  which told of a young woman with PWS from Macedonia who came to stay for a few weeks at Witteskindhof centre with her family.  While she was able to integrate socially and make new friends, at the same time both she and her family learned more about PWS and what kind of support is needed around this syndrome.

Not long after our Qatar request, we were rewarded with an answer outlining the conditions for acceptance.  We have passed this onto our contact in Doha and are hopeful that an excellent outcome will be reached.

Philippines: Not long after this, our CE, Giorgio Fornasier, had a visit from our parent delegate in the Philippines who has been a very active member in our large group.  Currently she is working for a Dutch company and travelling to the Netherlands every few months.  She was immediately put in contact with Dr Leopold Curfs there, and so another strong strand of information, support and help has been forged.

China: An enquiry from China – and, wonderfully, we have Chinese translated information that was able to be posted out immediately, and our parent contact in China alerted to this new family.

Mexico:  In Mexico, a very successful weekend workshop on PWS was held with topics covering paediatrics, growth hormone, behaviour and so on.  Our Scientific Consultant, Dr Moris Angulo from the USA, was one of their major speakers.  Mexico is making very good progress, especially with such expert help!

Peru:  It was sad to receive an email from Peru from a mother who had lost her 3 year old daughter who had PWS, and who was scared to become pregnant again, not knowing the risks involved of carrying a second child with PWS.  We have good Spanish-speaking medical contacts who were able to talk to this mother in her own language.

Chile: We welcomed a new Parent Delegate from Chile, Amarlia Barlart, who speaks excellent English as well, and who is eager to help families in her country.

New Zealand: We welcomed a new Caregiver Delegate from New Zealand, Julie-Anne Quinney and look forward to meeting her at the 8^th International PWS Conference next year.

Our Professional Providers Board:  Talking about our professional caregivers, they have e-office hours where you can contact them and talk through any issues or problems, or just talk about PWS with another country – it’s a great resource and we hope to build up quite a talk-fest.  You can find information on our website under the “Carers” tab.  During their last month's office hours, they spoke with caregivers from USA and Spain.  You don't even have to have a problem to talk!  They just love to know you're 'out there'!

Fundraising:  Finally – we are still struggling to revive our grant from Pfizer which allows us to do our daily work.  We know it is difficult to find funding for everyone all around the world, but we hope we will never be in a position to close our doors!  You can help, of course – our “Light a Candle” project is still alive and well, and we received a donation just the other day; our “e-gallery” project where you can buy wonderful and unusual art-work for a small donation, is still waiting for you!

Membership dues: And, if we receive all our membership dues, this will also help us in our work.  Outstanding dues still remain from:

Greece

Poland

Venezuela

Don’t forget, you need to be a fully subscribed member country in order to have a vote at the General Assembly next year where we look forward to another wonderful conference, full of more wonderful people!  (Well, we are, aren't we?)

Men & Grief

While we've been on the topic of grief recently, I was reminded of an article about men and grief that  I once asked for to go into our newsletter many years ago now.  I wanted to know why some fathers grieved differently from mothers and why men grieved differently from women.  Bear in mind this was a while ago, and I'm quite sure that, generationally, things have shifted significantly.  But if they haven't, for you, then maybe this might help.  You will also notice that in this story diagnosis had been late, as it still can be in many countries and there was no help with feeding.  This family really struggled.

"I had been looking forward to the birth of our first child. For months I had been fascinated by her movements insider her mother; at the beginning little flutterings 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 all 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 moths. 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 PWS. It took me a long time to accept this. I read all I could about this 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."

After the cat had died...

After my daughter had to make a heart-wrenching decision to have her cat of 10 years put to sleep, she showed some interesting behaviours and some that I'd not seen from her before.  It was difficult enough to handle the behaviours before the event (and I am eternally grateful to her residential staff who supported her, at times, courageously, and also very lovingly, through the entire procedure).  Her anxiety levels had risen considerably once she knew the cat was terminally ill.  Predictably (for her), she became angry, tearful, and violent (bashing a hole in a door, while crying uncontrollably).  All of us tried to talk her through the whole affair, saying that she was doing the cat a kindness, was brave in making the right decision, the cat will never recover, and so on.  Of course, all of you reading this will know that this was rather like water off a duck's back, because all that mattered was the actual moment she was in at the time.  There's no reasoning with someone (with PWS) when they are in such an emotional state.

But somehow we needed to keep pushing forward the idea that what she was doing, was the very best thing.  I apprised the vet beforehand, and told him that as far as my daughter was concerned, he was the "cat specialist" and she would trust and believe him.  I had good knowledge that this vet was not only an excellent diagnostician, but also incredibly kind and gentle.  He asked how much my daughter would understand and comprehend (everything) and conducted the whole procedure in a humane, thoughtful, and utterly gentle manner.  My daughter conducted herself with poise and grace throughout the whole time (and I think that her favourite TV channels, TV vets and Animal Rescue, probably played a part here too).

And now we come to afterwards.

She phoned me immediately from her caregiver's phone (she lives some way from me and I was unable to drive, having had a recent hip operation, otherwise I would have been there), and told me what had happened.  Both of us were very tearful.  And then she said she thought she would like an ice-cream.

She was allowed that (and, stupidly, I didn't stipulate what kind.  So she had one that probably had the calorific value of two meals combined).  Later that evening, she asked her caregiver if she could go to McDonalds for a coffee and muffin.  The caregiver said that she wasn't able to do this.  So there was a medium-sized melt-down.  The caregiver left the room until my daughter calmed down a little.  What she did next was to gather up 10 of her tiny incense cones, go outside onto the drive-way, sit down and put them in a ring around her and lit them.  Each cone represented an animal in her (and our) lives that had died.  She spoke to each of them individually, and collectively, telling them stories, reminiscing, and introducing them to each other.  This lasted an hour.  The caregiver, not wanting to interrupt her, could only finally entice her inside with the temptation of a plate of jelly.

The day after (she'd arisen at 2am to play music), she was calm, collected, and spoke easily about her cat and... how she's going to get a new one very soon.  After she's finished grieving...in about a week, or so, she thought...

***

This blog is a bit longer than usual and the general story finishes here, but if you want to read further, I was so interested in this behaviour that I wanted to find out more.  So what I did was to google behaviours in the intellectually disabled group.  There have been lots of papers written, but I didn't find much anecdotal evidence which is always much more interesting to parents.  I also wondered whether, because our sons and daughters with PWS are living longer, and well past their previous life expectancy, this was an area that we may need to look at for future reference.

Professionally, there was a lot of stuff like:" Individuals with intellectual disabilities as such may have limited understanding of causality.   There also may be cognitive reactions.  In the initial phases of grief, people may be in shock – disbelieving or denying, unable to process information.  Throughout the grieving process, individuals may find their concentration or span of attention is impaired.  They may process information less effectively.  It is not unusual for health professionals or others to be asked the same question or to provide certain information repeatedly."  (Of course, we must understand here, that the subject of the grief  in this instance is not just a cat, but someone close to that person.)

This kind of information, to me, seemed pretty  normal for anyone in a state of grief.  This article went on to say that it wouldn't be unusual for "people with intellectual disabilities to experience sleep disorders", or engage in "risky behaviours such as substance abuse.  They may be angry, lashing out, or seem withdrawn."  They may experience "impaired judgement" and theses changes may be profound.

Some other articles described stages of process for dealing with grief, like, preparing the person, instructing caregivers, modelling emotional support etc.  All of which is necessary, and it's good to know that such work has been done on this kind of preparation already.  Wanting to know more, I asked Professor Tony Holland (Professor of Psychiatry, Cambridge University, and President of the UK PWSA) what he knew.  He led me to Professor Sheila Hollins' work. A Professor of Psychiatry and Disability, she has a son with an intellectual disability; Professor Hollins is also a Peer of the British Realm and as such, one would think has some influence.  Professor Hollins has written some excellent books about loss and grief, mainly in pictures, for people with disability which you can see here "Books Beyond Words" 

To his knowledge, Professor Holland did not know of "anything specifically written about grief in people with PWS, but I could imagine that loss could have other implications that lead to high anxiety - eg, it's a change in routine", and he also advised that it is best to try to explore the mental experiences so as to understand what "might have started as grief, has now moved onto something more."

I still wanted to know more so I turned to my e-group of older parents with older kids with PWS  (PWS_Older@yahoogroups.com  if you're interested).  We've had this group since our kids were in their early teens and shared a lot of 'stuff'.  I was wanting to know about responses to grief in anecdotal terms.  And this is what I was told:

"My Dad passed a couple of years ago and a friend was killed in a house fire 7 yrs ago. My son handled all that amazingly well. But let one of his chickens die or be killed by a predator and he goes off the deep end. He screams and hollers for hours…..then he has to bury it in the back yard. We have our own little pet cemetery….."

"My son is very spiritual, as am I.  He attends church and loves being an acolyte, a server and in the choir.  He loves the ritual and pageantry.  We have had many discussions about God, and such.  Sometimes he's so very deep.  He grieved for our pets and his granddad in a strong, but deep way.  He also seeks solace in food, but then again, don't many of us?"

"A friend who was also a home help, had given our daughter a kitten for Christmas.  Some years later, this friend died.  She had an open coffin and viewing before the serviced.  We explained to our daughter as best we could, and gave her a choice to go and view.  The cat had to go as well.  So off we went, four of us, my husband, daughter, me, and the cat.  No one questioned the cat being there, and in a way she was an ice-breaker for everyone.  They loved seeing the cat there, and how much she had grown since kitten-hood. "  My daughter looked at our friend in her coffin, talked to her, and then we said a prayer.

She has coped with death in the family really well,  and the death of animals as well, since we live on a farm.  But pet dogs, or cats, or lambs - she just has to replace them straight away and is constantly looking for one til it is replaced."

"Yes, my daughter felt grief, but handled the reality very, very quickly.  She was very strong in that area.  She could face life 'square on' but felt very deeply for her animals.  Replacing them seemed to be the answer for her as she then enjoyed them young again and it took her mind into a new experience.  It could be that she was able to cut off after a certain point in her grief pattern."

"I knew of someone who was 'engaged' for a long time to a lady (with PWS).  They hoped to marry.  They lived in close proximity, possibly in the same house, I don't know.  However, she died.  I asked the provider how it had affected him and how he was doing.  She said that it didn't affect him at all and that he was just living as if nothing had happened.

"Another lady died recently, she'd lived with three other ladies for at least 12 years.  She died on a Friday.  The other ladies were concerned about whether the group would be going garage-saleing the next day as previously planned.

"With my daughter, there's no emotion, but only questions about the person's/animal's whereabouts... for years!"

"When his paternal grandmother passed away a few years ago he handled it really quite well. His main upset was that Dad no longer had a mother or father. A few months later when it was Mothers' Day he didn't want to see me at all - he just wanted to take Dad out for lunch because he didn't have his mother."

While we're on the subject of death - how about the after-life?  My daughter had an experience in her new home whereby some chimes began tinkling for no reason, and a small pot of handcream leapt off her table across the room.  To cut a long story short, the caregivers had a priest come in to bless the house, but my daughter asked that the priest allow her friend (who had died a few years ago, and who my daughter thought it was) to continue to visit.  And, apparently he does - the chimes ring, and my daughter holds a conversation with her friend.  In answer to this, one of my e-friends said this:

"My son has frequently been visited by family members who have passed. He mentioned my Mom a few times and his other Grandmother from his Dad's side. But mainly it's a young cousin of ours, she has visited many times and my son would tell me about what she was doing, what they talk about and about heaven. Sometimes I hear him laughing in his room and later when I ask what was funny he'll tell me that he was talking to his young cousin. Recently a mom on facebook who just lost a child (non pws) mentioned that her child with PWS is the only one in the family who is visited by the one who passed. I believe our kids know and see things we are unable to."

This is only a small sample from six mothers, and you can see that grief in PWS may run the whole gamut of expression, just as it does in the normal world, but perhaps there is a link to understanding grief that our guys have that can help us understand them even better.  I just wanted to write about this in case there were other parents wondering the same thing.  I'd be happy to hear any comments.  Quite possibly, it's the behaviour before the event that is more challenging than afterwards, but we need to be aware of both sides of the coin.

Linda


References: http://www.ddhospicepalliativecare.org/forum/ciwid.pdf
http://bma.org.uk/about-the-bma/who-we-are/biography-sheila-hollins

Mum, I've baked you a cake!

I recently had an operation that has required some time recuperating.  My daughter's response was to bake me a cake.  She lives some hours away from us in a house which is fully staffed 1:1. She's the only one in "her" house and she absolutely loves her new life.   Many people think she is very lucky, but luck had nothing at all to do with it.  Sadly she was not able to curb her behaviours to allow harmonious living with others and she had to go through some very tough times because of it.


So...the cake was duly delivered and later that night as I lay in bed thinking, it occurred to me how much she has grown and developed over the past couple of years.  She's now 28 and her move into this new home has been a revelation - to her, and to us.  For the first time in quite a while, she is happy.  She is losing weight, has a couple of small part-time jobs (supported) and the new care-provider agency seems to 'get' what PW is all about.  Why else, I asked myself, would they have let her bake a cake?


Whenever she wanted to bake when she lived at home, I would inwardly cringe.  My thought was that if I 'gave her an inch, she would take a mile' and I didn't have the energy to cope with that, so baking was something she longed to do while I longed for any excuse for it not to happen.  I knew I was denying her the one thing in the world she wanted to really do - cook! 


Once, for a 'significant' birthday she saved up her money to buy me a cake which she had got decorated in bold letters "Happy Birthday Mum" in lurid blue icing.  She didn't ask for a piece of that cake, and neither did she ask for a piece of her latest cake.  Her great joy was in choosing what she wanted to bake, cooking it, and giving it away, although on any given day of the week otherwise, she is one of the most opportunistic "acquirers" of food I have ever known.  And the most ingenious.  But when she's cooking from the heart (an expression bandied around on every cooking show these days) she has this spirit of generosity and nurturing that you seldom see elsewhere.  Except, I think, in PWS.


So many of our guys with PW have this wonderful spirit of generosity.  It's odd, when you think that the chief characteristic of PWS is always said to be this unrelenting desire to eat - yet, sharing food is something they genuinely love to do.  Francie has done it since she was very little.  It's one of the most disarming traits of the syndrome.


And yes, this latest cake was very nice with a layer of yummy dates at the bottom.  She made it from a packet, but included the dates as she thought I might like it better.  And, I did.

New weight-loss drug: Qsymia

The latest weight-loss drug to be approved by the FDA is called Qsymia (pronounced kew-sum-EE-ah).  It has been prescribed for overweight people, so naturally the world of PWS will take an interest in this. To keep you updated with this from a PW perspective, our Vice-President, Janalee Heinemann, has written this review (below).  You can also find information here: http://www.bu.edu/today/2012/will-qsymia-make-you-thin.  It should be noted that if people are planning to try a drug, it's most useful to do so as part of a controlled trial.

Weight Loss Drug Approved by FDA

By:  Janalee Heinemann, MSW

PWSA (USA) Director of Research & Medical Affairs

IPWSO Vice-President

As many of you are probably aware, there is a new weight loss drug, Qsymia, just approved by the FDA.  The weight loss was more with this drug than with two others recently reviewed by the FDA and one approved (Lorcaserin/Belviq) that was not nearly as remarkable regarding weight loss.  Qsymia is a combination of two older drugs that have long been known to help with weight loss:  phentermine (the safer half of the old fen-phen drug that was banned) and topirimate – a drug that was studied back in April 2000 under a PWSA (USA) grant, “Open-Label Pilot Study of Topirimate in Adults with Prader-Willi Syndrome.” Topirimate is an anticonvulsant drug that makes people feel more satiated after eating, which is why we sponsored the study by Nathan A. Shapira, M.D., Ph.D.  The study showed that Topirimate did not significantly change the calories consumed, Body Mass Index, or decreased self-reported appetite in PWS. In addition, there were no significant changes in compulsions. Surprisingly though, Topirimate treatment resulted in a clinically significant improvement in the self-injury (i.e., skin-picking) that is characteristic of PWS.
The researchers of Qsymia state that it targets multiple brain signals that drive people to overeat.  We cannot say at this time if it will be effective with PWS.  Please know that we will keep you informed of any new outcomes on this and other obesity drugs that might be helpful with PWS.  Currently, I have been working informally with two pharmaceutical companies who are working on potential products that might impact on PWS, but as with all pharmaceutical companies, there are always strict agreements of confidentiality.  We are also working with FPWR on financially supporting the Best Idea Grants post the hyperphagia conference with 2012 One Small Step funds.  We never forget that the #1 deadly enemy of PWS is the appetite.

For those outside "Prader-Willi Land"

After posting last week about our new project, FamCare, I read a post in one of the PWS listservs which I want to share with you.  It describes a parent's feeling of utter joy in finding a place for their adult son.  I know - we all do - that not everyone has this opportunity, and this is why IPWSO has developed its FamCare support; to help families along this rocky road, hopefully to a productive and happy ending.


"After four very difficult years, with many, many ups and downs, our son is finally part of a community where he is just like everyone else, and where the staff "gets" the strengths and needs of folks with PWS.   He has joined some great young gentlemen  and he is over the moon with joy. The young men have graciously welcomed him, and are helping him settle in. Finally, I am feeling the relief that my son will be able to live a healthy, happy and productive life. I feel like we won the lottery!


I wanted to share this with you all, as you are the only folks who will truly understand our joy after so much struggle. No one understands the day to day life of living with PWS like us families. While we love our children and they bring us great joy, they present so many unusual issues and cause incredible stress, that those outside of "Prader-Willi Land" will never understand. For the first time in a long time, I am at peace and can rest."

IPWSO's new FamCare (Family Care) project

IPWSO is exited to tell you about a new venture it is undertaking.  Called "FamCare", it is designed to find ways of supporting families around the world who are caring for their adult child with PWS at home.  We know that there are many of you doing this and looking for answers to questions, or just for someone else to say, "we know what you're going through".

Georgina Loughnan, from Sydney, Australia, who is an IPWSO Board member, is heading up this new project.  She has worked over the past 21 years as a clinician with adults and adolescents with PWS.  She has seen families continually work hard to provide and access the best care possible for their adult children with PWS, and recognises the difficulty that this can pose.  She says:

"As children develop into adolescents and adults, families continue to struggle to find the most appropriate setting for them.  It is these areas that need to be addressed as soon as possible for the adult child with PWS, to ensure that every environment they spent time in is as appropriate to the needs of someone with PWS as possible.

"Finding a school that will step up to the challenges demanded by someone with PWS is a difficult task.  The school years can be most rewarding and above all, secure for a child with PWS if the service providers fully understand the syndrome and the specific needs it brings.  On leaving school, the adult child with PWS faces a whole new lifestyle, one that inevitably offers many opportunities for great independence and high hopes.  However, people with PWS can never survive well with independence, especially in travel or situations that provide even the hint of access to food.

"All environments into which adults with PWS venture need to be appropriately managed to prevent the development of overweight and difficult behaviour.  Where an adult child with PWS remains living at home with the family, it will always be up to the family members and any other people supporting the adult with PWS to make provisions for the best possible environment."

FamCare's aim is to support families who need help, inspiration, new ideas, and just plain common-sense answers to the every-day problems that arise in caring for and managing the situations that arise when living with an adult with PWS at home. 

Watch this space... there will be more!

Reflecting on unfairness...

After writing last week about the importance of forward-planning for our sons or daughters with PWS -  something that will stand them in good stead as they enter their older years - I was rudely reminded how fragile this whole thing can be.  A newspaper article written about Spain's financial difficulties included the story of two families, each with a young boy with PWS whose lives were being well-managed thanks to the availability of Growth Hormone Treatment.  However, because of the fiscal difficulties in their country, access to this treatment had been completely restricted and no longer available to them.  It is a reminder how carefully built networks can so easily be blown away in a puff of wind.

It is so disheartening to read things like this knowing what a struggle it has been - and still is - around the world to convince medical authorities of the importance that Growth Hormone plays in the well-being of those with PWS.  I know what a struggle it was in my country, New Zealand, to convince the powers-that-be to make GHT available to children with PWS, and we had to fight for our rights up to the highest parliamentary level.    It is not only disheartening for parents, but also for the medical professionals who have researched and proven the benefits of this treatment for their patients, only to have it snatched away.

Some might say, at least the boys had the treatment for a few years and this should stand them in good stead, but experience has proved that when GH is stopped, there is a reversal in the muscle tone and, for some, in other areas of development, and this can be quite a major turnaround.  For these Spanish boys, there is a huge injustice in this decision.  There is, in fact, a huge injustice in most countries where GH is not freely available, and where medical treatment, support, and even understanding of the syndrome is not to be found.

IPWSO is planning to develop a series of forums on its website, for the medical professionals, one for the caregivers, and one for parents.  The parents forum - which will have a translation button - is a place where all parents can meet and share experiences.  Most of all, this can be a place where parents can ask questions  which can be directed to professionals who may be able to shed light on a problem.  Sometimes there are no answers, but other parents may have had similar experiences that they can share which may help.

It is important to know we are not alone in the world.

What comes next?

Looking through some papers that have recently made it to my desk, I was reminded by this one just how difficult it is in looking forward and anticipating the needs that our young children will encounter in their lives.  Entitled "Complex Care Needs - PWS" the author of this paper makes the following reflection:

When thinking about multidisciplinary care, the scenario often involves elderly patients with a multitude of chronic diseases. In focusing on a completely different age-group, it was amazing to discover the amount of forward thinking that goes into the care of those with congenital disorders and complex care needs. 

The author, an Australian medical student in her final year, was following the case study of a very young boy.  She noted that his parents had actively engaged with a team of professionals for their son, namely: a paediatrician, GP, endocrinologist, dietician, speech pathologist, physiotherapist and occupational therapist.  The author goes on to point out that:

"Not only do goals and requirements of care constantly change with increasing age and ongoing development of the child, but parents and practitioners are constantly having to forward think about contingencies for any potentially arising complications in addition to putting into place plans for what will need to happen when the parents themselves pass on and their offspring will still require care."

And I agree with her.  There is a huge amount of forward planning that has to go into a child with any disability, let alone PWS, in considering the future quality of life that this child might have.  I would go further and say that all of this planning relies on the ability of the parent/s to be able to work through a quagmire of red tape and bureaucracy in order to get results.  How very difficult it is for any parent to find the right agencies who will provide support,  help and medication for their son or daughter.  And that's just in our Western countries where, somewhere along the line, help can be found.

How much more difficult is it for parents in countries where this help is not forthcoming, or it is too difficult even to find the start of that long and winding road.    IPWSO is very aware of this and has its own large network of professionals in over 90 countries around the world which may be the start point a parent needs.

If you need help - start here.  Email your questions to us at IPWSO  We will do our utmost to help you find answers.

Our Guardian Angels in Israel

Urith Boger talking about PWS.  On the screen is her son, Doran, and his caregiver

Some of the Guardian Angels

On May 13, 2012, our PWS Association in Israel held a charity event in honor of all the caregivers who work in our three different programs.   A charity event is the major means by which we raise money.  We have a tradition of producing an evening whereby a part of it is a presentation of our activity and the other half is a nice musical program. Usually half of the money we raise through such an event is by tickets and half or sometimes even more is by donations from people who cannot attend the event itself.  It is very important to us to meet our circle of friends face-to-face, because it is almost the only way to let the public know a little bit more about the syndrome and the treatment of those who have it.

As mentioned, this year the topic of the event was the experience of the dedicated and loving caregivers of "out of home residences".  We named them "Guardian Angels" because we know very well that they play a crucial role in keeping our children alive, and of saving  the lives of their families as well. 

One of the young men, thanking his caregivers

Each program made a short movie and one of the staff came to speak on the stage. Two of the residents spoke as well.  It was very educating to learn a little about how the care givers view their work, their protégés and their mission. And above all what they learn from being with and working alongside people having PWS. As one of them said so nicely: " It makes me a better parent to my own child". 

(contributed by Urith Boger, PWS Association, Israel)

Dr Larry Genstil with a young man from one of his programs

 

Painting, by Urith Boger, of her son, Doran (on the right) with his caregiver

The folk-singers who gave the concert

3rd International PWS Caregivers' Conference

Talk with the hands!

Held this time in the inspirational surroundings of Wildbad Kreuth, the conference was host to 17 countries around the world where residential caregiving for those with PWS has been established.  The format of these conferences is based on lectures and workshops, with the workshops spanning two days.  It's here, in the smaller groups, where procedure and policy is hammered out - what works best for those with PWS?  What are the differences between countries?  How can staff best implement changes?  What are the newest ideas from everyone?  What is working well, and what needs reviewing? 

This all takes time, and our workshop leaders work really hard making sure they get everyone's views and incorporating ideas and concepts into guideline format for the rest of the world.  Already there are Guidelines for Best Practice available to anyone interested, and this time the focus of the meeting was to work out how to train the trainers; what information was needed, how best to portray the information, and how to implement it. 


About to start kinaesthetic workshop

One of the new workshops this time focussed on sensory integration with an extra plus of having a kinaesthetic workshop. 

"Our workshop was on sensory issues in PWS, and unlike the workgroups in the past, we really did emphasize the caregivers personal experience with a variety of sensory stimuli including  touch, vibration, taste, chewing gum, breathing, relaxation  and kinesthetics.  This whole conference was about experiencing the moment within the collegial atmosphere of the workgroups as well as enjoying  the wonderful sights and sounds of Bavaria in the company of friends outside the workgroups."



These youngsters were true professionals!

 "The outstanding cultural program was most memorable, showcasing the band, the songs and the dancing of the village children in their ethnic dress.  An enjoyable and  productive time was had by all!"

Friends, good food, and fine beer!

"When I think of our time in Germany, I immediately feel the great feelings of being with such wonderful people with whom I have been sharing conferences for many years.  I look forward to the conferences for all the information, the learning, the sharing, but mostly for the people we see year-in/year-out.  It's the people with whom I have regular contact all through the year, and the relationships that have formed, are true friendships that I treasure."



PWS in Cuba

Everything started 3 years ago when a doctor from Cuba contacted IPWSO through his brother in law who lives in Sweden. He wrote to us saying he needed help to confirm the clinical diagnosis made to his 4 year old daugther, as this was not possible to be done in Cuba. The methylation test confirmed the diagnosis and that his daughter had UPD. The doctor immediately asked for information and educational material in Spanish, which we provided, and expressed the wish to participate to a PWS Scientific Conference somewhere in Europe to learn more.


IPWSO invited him to join the International PWS Conference in Taiwan in May 2010 instead and covered all his travel costs, while Cuban authorities and especially the Ministry of Health of his country helped him a lot to get the passport and the permission to leave Cuba. As soon as he returned home, so enthusiastic and full of information, he organised a 1st workshop for professionals and parents in his own town, hosted in a room of Caritas, as this Catholic organisation is very active in assisting disabled people throughout the country.

The young doctor, and his wife, who is a psychologist specialising in teaching children with disabilities, have agreed to represent IPWSO, and so Cuba became a member of our International Organisation. They asked Caritas to help them find cases throughout the country, spreading the voice to their branches connected with the various parish churches. In a short time they found 15 patients and especially many doctors and professionals interested to know more about this syndrome.

Once Caritas offered to host a National Workshop open to professionals and parents and to help families financially to join La Habana, we were contacted again for help to find professional speakers in the Spanish language from Latin America and Europe. The key speakers accepted the invitation with enthusiasm and covered their own travel expenses. We owe much to them: Dr. Moris Angulo (El Salvador-U.S.A.), Dr. Maria Del Valle Torrado, Dr. Hugo Serdloff, Karina Abraldes (Argentina), Irune Achutegui (Spain-Italy), Dr. Fanny Cortes (Chile). They covered all important aspects of the syndrome such as genetics, endocrinology, pediatrics, psychology and psychiatrics.

Local parents appreciated also the presence of parents coming from other Spanish speaking countries representing IPWSO network: Luis Barrios and Mayra Urizar from Guatemala, Julia Bonelly from Dominican Republic, Fernando Briones and Mariona from Spain and Giorgio Fornasier from Italy. All scientific speakers modified their own presentations to be understood by parents and this was highly appreciated by Cuban professionals too, because they learned how to approach parents with scientific and medical issues in an easier way. The priority was given to parents, to their questions and especially sharing experiences. During breaks and meals local doctors had the opportunity to talk about scientific details with foreign speakers directly.

The 2nd Workshop on Prader Willi Syndrome in Cuba was held from 27 until 29 April 2012 at the Sacaerdotal House in the Capital La Habana, under the precious umbrella and organisation of Caritas Cuba. Everyone slept at the same house where the Conference was held and had meals at its restaurant, so there was plenty of time to stay together till late at night. The meeting room had equipment for video presentation and became more a sort of a family sitting room, than a Congress.

Looking at the audience, you could not distinguish who were parents and who were professionals. They all participated by heart, laughed and cried together. It was amazing to realise that 3 years ago there was almost no knowledge about PWS in Cuba and now there were 63 people attending the workshop: 6 professionals from abroad, 32 professionals from Cuba, 6 parents from abroad, 15 parents from Cuba, 4 volunteers of Caritas Cuba

The picture shows the smiling group that participated to the Workshop and among them there were parents who were visiting La Habana for the first time and they did not know each other.

It was touching to meet the oldest patient with PWS in Cuba, a 42 years old woman who was diagnosed by Dr. Prader himself when visiting Cuba years ago. Everyone cried listening to the mother of Manolito, a 19 years old boy who died some months ago. Manolito’s mother came to honour the memory of her son with her presence and participated in the discussion and sharing. At the end she told everybody: “Don't miss the unique and precious opportunity you had and what you learned at this fantastic meeting. Work hard and let other Cuban children with PWS live!”


(contributed by Giorgio Fornasier, CE of IPWSO)

International Caregivers Conference

Very soon, in May this year from 7th - 9th, the third International Caregivers Conference will be held in Germany.  This is held in partnership with two residential providers in Germany: Regens-Wagner Absberg, and the Diakonische Stiftung Wittekindshof.

Pam Eisen, opening the first conference in Herne, Germany

This conference is specifically for professional caregivers and attended by countries from around the world.  This was the brainchild of Pam Eisen, IPWSO's past President, and as such has provided the world with the new International Best Practice Guidelines for Residential Care for Prader-Willi Syndrome.  (Copies of this are available here from IPWSO.)    All participants attend workshops of their choice and contribute their own knowledge, ideas, concepts, and country's models of care, which then get translated into draft guidelines.  Each workshop has a facilitator, a scribe, and a translator.  At the end of each day, time is taken to write up all the notes which are tacked up on large boards and presented at the "market place" where everyone gathers to hear what has taken place.  These draft guidelines are then thoroughly discussed and tested by the PPCB before becoming recommended as international best practices.  In effect, they are contributed by each and every caregiver from all corners of the world - what more could you ask for!

Topics included range from the understanding of the syndrome and its characteristics, nutrition, weight, exercise, psychological make-up, psychiatric care, communication, social skills and empathy, what makes  a good residential home run well, the transition into and out from the education system, to, more recently a topic for this year's meeting, how to develop a PWS curricula for staff.


Because these guidelines are something IPWSO wants to be able to endorse internationally, there are, understandably, very different cultures contributing some very different ideas and it is always interesting and exciting to learn how these cultures can be absorbed into our international network of care.  And, not to be forgotten, are families supporting adult children at home.  Their needs are just as great as those in residential homes and must be acknowledged and woven into the whole concept of care.

The 1st and 2nd conferences were held here in Herne, Germany

Care must  be holistic and inclusive.  It needs to acknowledge that home life has a series of transitions to overcome - from home into school, from school into the workforce, and also into the community.   IPWSO and the PPCB strive to support all our families, our residential providers, schools and educationalists, as well as medical professionals throughout the world.

One of the workshops' diagram of "Anatomy of a crisis"

This is not a small task, and the PPCB is completely dedicated to helping provide professional support and help through its combined international knowledge.  This year's conference is in Wildbad Kreuth, about an hour's drive from Munich.  Speakers are from all corners of the world, there are two days of workshops (and 'work' is the operative word!), and the best opportunity professional caregivers will ever have to work with some of the world's experts in this specialised topic.

If you are a professional caregiver and would like to attend the conference, please go here for registration information.   Likewise, if you are a parent and have a son or daughter in residential care and think it would be beneficial for someone to attend the conference, please let them know.

As usual, we have great expectations from this unique gathering, all in the name of Prader-Willi Syndrome!

Onwards and upwards!!

Listen

This is a personal post, nothing to do with IPWSO, yet everything to do with us all.  These past months have been, for me and my family, difficult.  We have been trying to get our daughter back into the residential system and nearly everything has contrived to go against us.  The more that things didn't work out, the larger the conspiracy seemed to become and the slower the wheels seemed to grind.  Of course, it is not a conspiracy, it's just the way things happen in real life.  Or don't happen.  And in the world of disability, wheels grind even slower.

I think now that we are close to suceeding.  But I say that, knowing full well, that as the saying goes, "there's many a slip twixt cup and lip" and plenty of time for things to go wrong.  I don't think one can ever say, "hooray, we've worked that one out - finished, completed!"  Because in the world of PWS life is not a stroll across the meadows.  Rather it is fraught with things to trip us up, therefore the way we live is to prepare for the worst.  It takes some of the joy of just "living" out of life. But that is the way of it and we all do it,  and in 97 countries who are members of IPWSO, I know there are parents going through exactly the same sort of thing.  Night and day.

Sometimes I feel as though I've truly had enough.  And it's no help to remember 'there are others worse off than yourself'; right at that moment, it's the most unhelpful advice there is.  I feel like the little dog, condemned to death, who just needed a hug:   Have a look at her and you will know what I mean.  Just a hug, that's all. (http://www.godvine.com/Meet-the-Scared-Dog-That-Only-Wanted-a-Hug-1087.html)

Sometimes I feel great joy in seeing how others can overcome disability and show the most perfect ability.  Like Kyle Coleman, who has autism and is speech-mute, but can sing like an angel.  He has perfect pitch.  Just look and listen to this young man.  This is what his mum says about him: and you can hear him sing his trademark song, "Just Listen", but a better rendition is on his own website where you can hear him sing, 'Lady D'Abanville' and 'Sunny Afternoon'   Isn't it wonderful that someone just listened?

I've always said it's about listening - if you don't really, really listen, then you don't hear what the person is saying, particularly if that person has a disability.  The first thing that is different is the way they communicate.  If you don't listen, you will never, ever hear.

And, even for those of us who can communicate, talk, write, speak to many, and seem completely confident in this world - there are times when they need to be listened to as well.  We're not that strong.