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.