After the Conference

Some thoughts on the 9th IPWSO Conference, Building Global Community

from Marguerite Hughes, Vice President, IPWSO

It is hard to know where to begin with describing the 9th IPWSO Conference, hosted by FPWR Canada, which has recently concluded!

FPWR Canada rose with style to the huge logistical challenge that was involved in organising a parents' program, a scientific program, a caregivers' program, scientific workshops, a people with PWS program, and a host of other meetings and events all across the course of a single week. The smooth running of this complex event is a great testament to their energy, skill and high standards.

The atmosphere at the conference was as impressive as the logistics with a genuine warmness and spirit of cooperation evident throughout the week.  From conversations in lifts and corridors, to mealtime encounters and formal presentations, everyone seemed eager to help build the "global community" described in the conference's title.

As at every IPWSO conference the opportunities for parents to interact with professionals and vice versa stood out.  So too did the many interactions between people from different countries and cultures who educated each other on how they manage PWS and the challenges they face. Everywhere I looked I saw business cards being exchanged and suggestions being made as to how one person or association could help another.

The gala, which was effortlessly hosted by Keegan Johnson, included an array of special moments including well-deserved awards for Dr. Suzanne Cassidy, who has worked tirelessly as IPWSO president for the past 6 years; and Dr. Glenn Berall, for his outstanding work on behalf of people with PWS in Canada .  The eloquent, humorous and truly inspirational speech by Megan Michie, who has PWS, received the loudest applause of the night, although the beautiful singing by Giorgio Fornasier and Monika Fuhrmann came a close second. 

 

Jim and Joan Gardner at the IPWSO Booth

Outside of the gala there were many inspiring and touching moments too.

The speech by Dr. Tom Hughes, President and CEO of Zafgen, which he made on the day after Zafgen announced it was ceasing the development of beloranib in Prader-Willi syndrome, was surprisingly moving.  Dr. Hughes promised that Zafgen would share the information it had collected to help others engaged in drug development and was warmly applauded by the large crowd as he left the PWS stage.

The presentation by Loisel and Marlen from Cuba in which they spoke with passion about how, with help from IPWSO and others, they have worked to bring the PWS community in Cuba together was one that will stay in my memory for a long time to come.  The standing ovation they received was as warm an ovation as I have ever heard.  Later in the week at the IPWSO General Assembly the same couple successfully bid that Cuba would host the 10th IPWSO conference in Havana in 2019.

Overall, it was a wonderful conference made possible and successful only by the willingness of individuals and organisations to work hard and cooperate in the interests of the global PWS community.  Congratulations to all involved!  

At the end of the Conference, IPWSO held its General Assembly where the new board was inducted.  We are sorry to say farewell to those members who have worked tirelessly for the past 6 years, but excited to introduce our new Board to you all.

 

 Back:  James O'Brien (Australia), Hubert Soyer (Treasurer, Germany) Mariona Nadal (Spain) Georgina Loughnan (AUstralia

Front:  June-Anne Gold (USA) Tony Holland (President, UK), Marilyn Dumont-Driscoll (USA), Marguerite Hughes (Vice President,Ireland) Amalia Balart (Chile)

How can I trust you, now that I know your ways?

Trust is, I think, one of the most difficult things to, well, to trust.  It's hard enough in the real world, let alone in the magical world of PWS.  Or even one's other children, come to that.

There are plenty of lovely sayings about how we should trust our instincts, trust each other, trust our partners etc.  But if that trust is broken, it is one of the most difficult things to repair.  As far as trusting our kids with PWS, well, we tend to trust with our fingers secretly crossed behind our backs; we hope for the best, we pray we've made the right decision ... and I remember when I trusted her (after plenty of persuasion from her) to go down the road to the library on her own.  She persuaded me that she would go to the library and only the library and would get books out and walk back home.  It wasn't far and she didn't have to cross road and besides, it was good exercise and she had to carry the books home.  For the first couple of times she did exactly as she promised and the trust between mother and daughter built up accordingly.  The following week, however, she was becoming a little late with returning home and I thought I would walk down the road to meet her.  I ended up walking the the whole way to the library.  I walked in; couldn't see her diligently searching for books, but I did find her curled up in a bean bag happily reading, with a large bag of sweets at her side.  Admittedly she was sharing these with a cluster of small children sitting at her feet, but a good many had been consumed by her already.  I suspect the money came from my handbag, something I no longer ever leave around.  It gets locked in the pantry, or locked in the car when she comes home these days.

After talking severely to her about the disappointment of having the trust broken, I decided it would be cruel not to give her a second chance, so when she asked a few weeks later if she could take the dog for a short walk down the road I was rather surprised to hear the phone ringing about 5 minutes later.  It was the neighbour from over the road who asked if I knew my daughter was "collecting for the church".

Another lecture from me about trust, right and wrong, annoying the neighbours and whatever else I could think of to hammer home the message.

When will I ever learn?  Obviously it is going to take a while.  There have been plenty of incidents since then: she tested the other side of the street, "collecting for Girl Guides".  And then as the years went by, she grew more cunning and her latest scam (what else can I call it?) is to take back things that I have bought her to the shop and ask for the money back.  The only way I can overcome this is to mark anything new with a highlighter as she is just so quick to convince the shop-owner "Mum bought the wrong thing..."

There is a huge amount of advice out there about trusting your children, cutting them some slack, to stop putting high expectations on them (which apparently leads to their becoming stressed), and to assume the best and trust your child and believe in their inherent goodness. Well, I'm going out on a limb here and saying, don't trust your child; it can just be too much for them.  Too much temptation; too much freedom; and way too much slack.

You know, I think that this is one of the most difficult things to deal with because we long to trust with all our hearts, but we know full well that to do so is setting the person with PWS up for failure.  Big time.  They can't help doing what they do best; survive.  Their whole being is driven by the need to find food, find money, find whatever it is in their lives that they have decided they must have in order to survive, and if I just open the door and say "go for it; I trust you", I am doing my daughter the biggest disservice in the world.  I know she won't thank me for it - ever - but I can't trust her, and I won't trust her.  It's sad, but she's proven that she can't tolerate the stress of being trusted, or being caught out, so it's up to me to do something.  The boot is clearly on the other foot - it is I who must be trusted by her, not to let her down, and not the other way around.

So, you're quitting for the third time? How's that going?

Look, I'll call this at the outset; this blog isn't for the faint-hearted or very young.  This is  the warning on the outside of the cigarette packet, ok?

So, today she's home for the weekend and is quitting smoking, for the third time.  This time she has gum, patches, pills, an oral spray, and peppermints.  Oh, and other little cinnamon-tasting sweety things that I discovered as well.  It's a rigmarole and according to the paper-work that has come with her, I have to keep track of all of this along with every other pill she is taking.  (It is my resolve that she is pill-free by the end of the year - there, I've put it in writing - so we shall see.)  Her staff are very supportive and a few of them have also given up smoking and managed to stay off the demon tobacco. However, there are those who haven't, and, well, there's the rest of the world's smokers out there as well.

It all started many years ago at one of her residential placements where staff all smoked.  They said it helped keep them calm.  She thought that was a great idea and since she needed to also keep calm, she managed to either bludge cigarettes or they were given to her quite freely.  Or she stole them, or picked up other people's butts, or stole money to buy them.  She was totally hooked.  I, on the other hand, along with the rest of the family, could not believe she had started smoking, let alone how she was acquiring them.  I remember following her down our garden path when she was home once, camera in hand, to see for myself if she was actually smoking and whether she was drawing back the smoke or just puffing.  It was the complete draw-back, probably with a few smoke-rings thrown in for good measure, but I didn't wait to see.  I clicked the camera and made for safety.  Too late.  Furious with me, she took the camera when I wasn't looking and ripped out the sim-card.  That was quite a few years ago now.

At the time, I spoke with several friends, with staff, and with other parents who were in the same boat.  I went with the advice of letting her have 5 cigarettes a day - God knows why, but I did.  That lasted all of a week because she was clever enough to take out all the tobacco of the tailor-made cigarettes and, using cigarette papers, rolled herself seven.  I know.  Clever and cunning,  and more fool me.  Over the years things went from bad to worse and naturally the cost of cigarettes went up each time the Government prepared its annual budget.

I'd heard of Champix, a drug that has great success in getting people off the habit.  Now, the good thing is that you can get Champix on prescription which the Government subsidises.  After the birth of a few much longed-for nieces and nephew, she decided with great persuasion from us, that she would give up in time for Christmas.  For the first time.  And, to her very great credit, she did. Credit also to her supportive (new) staff.  It was difficult, because the drug did seem to increase her hot-headed behaviours, but she remained cigarette-free for many months, so much so that we all took it for granted that that was the end of it.  Success!  But sadly, I have to report that it wasn't the end of it and within the following year she had taken up smoking again.  Stress, she said.

Alarmed, I said I would help pay for another round of Champix (you only get one 'free' round of this per 12 months) and I did.  It wasn't cheap, but for the six-week term it took, I gladly paid as she was keen to try again for the second time.  Her residential staff were once again supportive and particularly great with the increased behavioural outbursts.  Once more, we were smoke-free for another 6 months before once again she picked up the habit.  Stress, she said.

After hiding it from me for a few months she is trying once again to give up.  This time she's on another drug, Zyban.  I've looked up all the side-effects and I'm not that happy.  It has to be taken along with the patches and all the other paraphernalia (as per above paragraph 1).  She's broken the programme twice already.  Stress, she said.  "And I can't sleep, so I've got this other pill for sleeping."  I'm not happy.  But this weekend she's determined to carry on with the programme and I'm doing my level best to be positive for her sake.  It's particularly difficult as for some reason 2016 seems to be an unhappy year as far as people shuffling off their mortal coils, and that disturbs her greatly.

After she'd failed the Champix programme for the second time, a PW expert told me she'd always be a smoker and it simply wouldn't work.  I was in tears after that.  It's rough being a parent and trying to do your best for your child, no matter what age they are.  There's a huge and very complex issue of co-dependency (look it up, it's worth a read... and don't blame yourself), wrapped around the core of guilt that somehow makes you feel you were, and are, responsible for this person from the very outset and until the end of time. 

One side of the coin I recently flipped goes all-out for good healthy living and an attempt at avoiding all the cancer-related illnesses that nicotine can bring, plus the cost of the habit when you're living on a sickness-benefit; the other side of the coin brings up "choice", that hysterically fashionable word that government agencies are wont to use in some vague attempt to look pro-active and on top of the situation.  They just don't get it, and never will.  That's why guardianship is so important when our kids become adults - it at least gives you (the parent) some sort of control over what they can and can't do when it becomes (as it so often does with PWS) life-threatening.  Sometimes it feels about as powerful as a wet bus-ticket, but sometimes it's all we've got.

You see, I know because it's happened before, that if I say, ok, your choice, she will start smoking, and there will be no limit.  Seven cigarettes a day will never be enough.  There will be a huge drive for more and that means finding the money to pay for them.  When you live on a Sickness Benefit as she does, and you have to pay for your doctor, chemist, personal stuff, and the cat which includes veterinary and cattery fees, then feeding the habit will mean everything else will lose out.  That means debt and theft, and we'll end up in court. Again.  Stress just doesn't cut it, not to mention the eventuating illnesses.

I reckon I'm fairly easy-going and if the world isn't going to stop turning, I'll generally choose to 'go with the flow', but when you step on my toes/cross the boundary/break one of my life-principles, or try to force my bottom-line to go even lower, then you'll meet a brick wall.  It's really hard maintaining that brick wall, I can tell you.  Some days it's nigh on impossible, but No-Smoking is one of my brick walls and I am determined and steadfastly refusing to let it be broken.

If you can possibly avoid your son or daughter taking up smoking, then please, at all costs, do so.  The other side of the coin of choice is everything I've just told you about.

Introducing The Lodger

(with thanks to our contributor, Emma Walsh)

The Lodger’s (that’s our son with PWS) cousin aged 5 has an amazing understanding and tolerance of PWS. She’s grown up with PWS in her life and knows no difference. She's The Lodger’s best friend and already a member of the secret food police.  

She comes running into the room,

“Auntie, we have a problem! The Lodger is saying he’s hungry and he’s just finished his snack, what are we going to do?”

I went in to talk to The Lodger, with my concerned niece by my side. I found him lying on the couch cushions on the floor, pretending to be asleep. He woke up, smiled and signed ‘eat’. He was playing. It was breakfast time. I explained to his very caring cousin, that he wasn’t really hungry, he was just using his hands to tell her he was hungry in the game. She smiled and the game resumed…

The Lodger has been using sign language since he was about 14 months old. As we live in Ireland, we use Lamh but it's not very different from Makaton (or other simple sign programs).  Its simple signs help him communicate his needs. THe majority of the signs are very obvious and so it's very easy to learn and understand.  Signing has eased The Lodger's frustrations.  He's been able to tell us he's thirsty, tired, or wants to read books, all by using his hands.  We started simply with 'eat' 'drink' and 'bed' which moved quickly to 'NO BED' when he learned to shake his head!

 

In the peak of The Lodgers sign usage, (before words) we probably had 50 to 60 signs that we used regularly - from activities like ‘drive car, walk, swim’, to animals like ‘cat, dog, rabbit, horse’, to feelings such as ‘happy, sad’ and words such as ‘more, finished and help’. 

The problem being an adult brainwashed into doing 50 or 60 different signs is that in normal grown up interactions the signs slip in and people start to wonder if you are going mad.

 At three years old now, The Lodger is starting to put two to three words together but still uses signs to get his point across, usually to emphasise the same point as above: ‘No bed!"

The lodger started Playschool last September and although he did have a few words, he was able to match them with a sign which was understood by his preschool assistant and teachers. Signing has really helped him with his independence. It’s been wonderful to see him dropping signs as he’s mastered the word and he loves the celebrations of learning new words!

When we were first told about signing, it was daunting. We weren't sure how quickly we’d pick it up or if it would be effective, never mind trying to teach a child without words. I can honestly say, it’s part of our day to day lives now- even though The Lodger can speak many words now, I’ll often see my husband sign ‘drink?’ while the Lodger responds with actual words. I’m guilty of not dropping the signs myself and often use signs and word combinations with The Lodger - and others!

As we introduced new signs for the Lodger to learn, they became part of our daily conversations with everyone! The sign for happy is two thumbs up and I still sign 'happy' in response to questions of how I am!  Between that and carrying invisible pigs (you can read about that story on our blog) people must really wonder what goes on in our house.

Signing is not just for children with additional needs. I’ve encouraged friends with typical babies to use signing too, it’s easy to learn and a great tool to help your child be able to communicate with you!  It's a fantastic bridge for the often frustrating communication gap before speaking comes naturally. Simple signing has really helped communication with The Lodger. The only problem nowadays is understanding his words when he refuses to sign to really make us work! I think we're learning as much as The Lodger these days. Two thumbs up to that!

You can read more about The Lodger here 

Carpe Diem - Seize the Day!

Yesterday I went to see my daughter - she lives in residential care about an hour away.  My plan was to take her out for morning coffee and just spend some time with her after what had been a dreadful Easter.  Why? I hear you ask...

...well, she caught a tummy bug, something she never really has and if she does, then it's normally in the form of diarrhoea  (I know... I wasn't going to tell you all this, but you did ask).  This time, however, her stomach became distended and very hard.  And we all know what this could mean; with the lack of vomiting reflex, the stomach could burst and the results could be lethal.  I didn't know what to do and, naturally enough, it was on the one day when everything was closed and we live way out in the country.  It was very lucky that she vomited.  Twice.  Look, I'm not going to get into details, suffice to say that it took hours to clean up and both her father and I also got the bug.  Happy Easter.

But I wanted to tell you about yesterday.  I'd phoned to make sure she was ready (and hinted at the fact that last time I saw her house it was extremely untidy), and I arrived on time to be greeted with a very tidy house and my daughter with what can only be described as 'raspberry red' hair.  She was wearing a very pink cardigan, so the red hair could not easily be dismissed.  I did a double take - much to her amusement, of course.  As we sat down in a lovely little cafe with the sunshine gleaming on the raspberry-red head of hair, I thought how pretty she looked and how happy she was, sitting there smiling at me.  I asked her how things were going and she told me she'd got herself a new job (a paper run, which is such a great way to exercise, plus get a small financial reimbursement), then she said she'd stopped eating bread, and was really becoming a vegetarian and hadn't eaten meat for two weeks, plus had taken dairy out of her menu, replacing it with soy and other proteins like tofu.  She said, with a big grin on her face, that her staff were really great about this and she was loving the change in menu.

I was really taken aback - on many levels.  My skeptic self said, "wonder how long this will last", and, "bet she's really enjoying finding out what else she can eat..." while my proud-mother self said, "for heavens' sake, just enjoy the moment." 

So I told her how proud I was of her decisions, how well she was looking, how much I loved the raspberry-red hair, and praised her for looking out for a new job, particularly one which meant more exercise.  I told her how healthy she looked - and she certainly did.   We often leave our small dog with her when we go away for a few days and there's nothing she loves more than to take small dog with her on the paper run, and there are several parks nearby where she can let small dog off the leash to roam (which means more walking for her, too).

I know that things will change and that there will be issues to deal with and all of that, and by writing this I'm probably tempting fate very seriously, but for once I thought - just seize the day!  Enjoy the moment!  Be proud and happy!  Carpe Diem, indeed.