Men and Grief

A very good friend of mine (also with an older daughter with PWS) reminded me of the early days, “back when…” and all the struggles we went through to get recognition for our children, to understand the syndrome, and to cope with the grief it brought with it.  That got me thinking about grieving and how it’s so ongoing with PWS, but as time goes on you tend to pick yourself up and carry on.  That reminded me of an article a father once wrote and submitted for a magazine I was editing at the time, and I thought it deserved a further look.  He wrote:

“I had been looking forward to the birth of our first child.  For months I had been fascinated by her movements inside her mother; at the beginning little fluttering 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 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 months.  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 Prader-Willi Syndrome.  It took me a long time to accept this.  I read all I could about the 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.  I hope so.”

 

Contributed by Linda Thornton

Let's talk about sex

In the past we swept the subject of sex under the carpet.  We knew that women with PWS menstruated infrequently, if at all, and that males were infertile.  Also, in those days, sex hormone therapy for males and females wasn't really an option, so the whole chapter of sexuality was closed for many years.  It was rudely opened in 1999 when the first birth of a healthy baby girl to a woman with PWS was recorded in Sweden.  This was followed in 2001 when a 32 year old woman with the deletion diagnosis was recorded as having given birth to a baby with Angelman’s Syndrome, in Denmark.  The third recorded birth was in New Zealand in 2004 when a baby girl also with Angelman’s syndrome was born to a young woman with a genetic deletion diagnosis.

Sex hormone therapy is now recommended for both males and females with PWS.  This “encourages the development of secondary sexual characteristics and potentially improves bone mineral content and density”

http://books.google.co.nz/books?id=Js81Pr1PmaAC&pg=PA171&lpg=PA171&dq=women+with+PWS+given+birth+to+child&source=bl&ots=WdkaahgMa7&sig=qYzjIz18-NjJaRKkzGGd5nKB8t0&hl=en&ei=kXERTsybAtHPrQeU-oCIBA&sa=X&oi=book_result&ct=result&resnum=4&ved=0CCsQ6AEwAw#v=onepage&q&f=false

It will also induce menstruation and the likelihood of pregnancy.  This means greater observance of lifestyle, good sex education and support, as well as a better understanding of how to cope with the resulting birth.  It is also recommended that males have testosterone therapy, and many youngsters are on growth hormone therapy and may well continue to be on GHT into adulthood.  Combined with sex hormone therapy, could this mean an increase in fertility? What about sex itself?  Marriage, even?   

Back in 1999 I visited a residential home in Seattle where a couple (both with PWS) were engaged to be married.  I asked the young man why he wanted to marry, and his reply has stayed with me ever since…”because I want to see her in the shower”.  I don't know whether they married, or whether they just lived together.   I know of another couple in Australia, she with PWS and he, not, who married.  I don’t know the reason behind that one!  

It used to be thought that marriage was too serious a contract for a person with an intellectual disability to enter into and that a person with PWS would be likely to walk out at the first sign of a melt-down.  Same as it was thought that a person with PWS should not drive a car as they would probably have road-rage.  However, I know of two people who have their driver's licence and have held them for quite some time, content to potter around their local town on their own.

Many of our young people want to experience a close relationship to "have" a boy or girlfriend, and nearly every young woman with PWS that I have met expresses a desire to have babies, to look after babies and to have them to cuddle and hold.  Most have a fine collection of dolls, or soft toys and many are genuinely wonderful with small children and babies.  However, I know from talking with the young woman's family in New Zealand, that there were many difficulties involved, even from the beginning when no one knew she was pregnant until a visit to the doctor in her last trimester brought the surprising news.  Birth was by caesarian section as they did not think the mother would be able to tolerate a natural birth.  The mother was overjoyed to have her own baby, but not so overjoyed when the baby cried and interrupted her dinner time.  Neither did she prove a natural mother able to care for a small baby or answer its constant demands nor did she express great interest or maternal instinct.  


What she did want, however, was for her baby to grow up quickly so they could live and travel together.  The baby has Angelman's syndrome and is cared for by the grandmother and sister while the mother is in residential care.  She does have regular contact with her natural mother, but the daughter's relationship, as one might imagine, is closer with her primary caregivers.


Somewhere along the line there has to be an extra element of responsibility and care from parents and caregivers alike.  There still needs to be educated and intelligent management and support around such important decisions as choosing to share your body or life with another.   There have been many changes in the way we care for and manage the lives of those with PWS, and doubtless there will be further improvements as we understand the needs of those with disabilities.  


At the end of the day, I expect every parent will share the same thought, regardless of whether there is a disability or not; "I just want my son/daughter to be happy", but let's make sure we know what they want, and how we can ensure that happiness and support.

Perspective on Challenging Behaviours

I came across an article on tantrums in children the other day.  It’s on the website: http://www.betterhealth.vic.gov.au/bhcv2/bhcarticles.nsf/pages/Tantrums?open

 

It described tantrums as being “a young child’s way of physically expressing feeling such as anger, frustration, hurt, and being upset.”  

And this has always been the way that I have seen a person with PWS expressing him or herself when all else has failed.  When something goes wrong and she is unable to explain what is happening, or that she doesn’t understand, or I don’t understand, my daughter with PWS will often revert to having what is described as a tantrum, or rage, or outburst.  It is frequent in PWS and is one of the deciding characteristics of the syndrome, and it doesn’t matter how old they might be, when they get to a certain point, this child-like reversion to tantrum behaviour is the only way they can express themselves.

It is important for us as parents or caregivers, teachers or residential providers, to understand that this is basically another form of communication when all else has failed.  The article goes on to say, “A tantrum is a young child’s way of physically expressing feelings such as anger, frustration, hurt, and being upset.”  And this is exactly what is happening to those with PWS.  It is also frustration in not being able to be understood, or to actually understand what is required of them.

In the online article, tantrums are expressed in the young child as “crying, screaming, punching, kicking, foot-stamping, running away, or going limp like a rag doll, throwing or breaking things…”   How very like a person with PWS this is! 

The young child “does not have the cognitive thinking abilities to plan their tantrums or use them to upset parents."  Neither does the person with PWS.  “Tantrums are simply a physical expression of the child’s feeling”.  Again, the same explanation for those with PWS.   The rages that can evolve from something that has triggered them off, are not planned in advance – they just ‘happen’ and often come out of nowhere.  The stressors for children are often “feeling tired, hungry, frustrated, overstimulated, or feeling stressed”.  Again, something we can easily recognise in PWS.

Of course, the major difference is going to be that tantrums are a normal part of child development and that children grow out of this as their communication levels get better and they feel more comfortable in themselves and in the world, whereas in a person with PWS this so often doesn’t happen.  The tantrums continue and are re-labelled ‘challenging behaviours’.  We must realise that just as tantrums in a small child are not ‘bad’ or especially naughty, so they are not in the person with PWS.

Recognising the triggers likely to set off a tantrum is the answer to successfully avoiding the behaviour.  Of course, we are not always so clever that we recognise the triggers each and every day, and neither are we born experts in the psychological management of avoiding difficult behaviours, but if we can start to understand the reasons behind the behaviour, then we are halfway there.  It requires great patience and control, often two things that, by the end of the day, we have totally used up!  This is when you mutter under your breath the prayer for serenity (only I call it the prayer for sanity):

God grant me the serenity

To accept the things I cannot change;

Courage to change the things I can;

And the wisdom to know the difference.

...and pour myself a glass of good wine.

Critical Parenting

There comes a time in parenting when you sometimes start to look at your son or daughter with a critical eye.  Are they shaping up the way you expect them to?  Are they a good reflection of the way you brought them up?  Will they be strong enough not to be weakened by corrupting influences around them?

We all do it, because we all want the best for our children.  Regardless of whether the child has special needs or not.

However, many of us are extremely critical of ourselves and set such high standards that others seem to fall by the wayside, inviting our criticism.   Often we seem to get more critical as we get older—life just isn’t ‘what it used to be!’ 

Having a child with special needs can sometimes make us even more critical, not necessarily outwardly, but inwardly as we compare our child’s “failings” with the next person’s child. 

By being critical we can build up a wall of protection against others—striking first before we are criticised.  There’s nothing wrong with being protective—heaven knows, it’s what we parents are best at… but criticism also has a darker side.  Ron Potter-Efron’s book, Stop The Anger Now (New Harbinger) says that ‘being critical of others:

• Helps me feel superior/dominant
• Convinces me that I’m right and you’re wrong
• Helps me avoid noticing my imperfections and faults
• Keeps me in control by making others appear weak, dumb or bad
• Is similar to what I do to myself.  I’m very critical of myself.
• Keeps me from getting too close.
• Is my way of trying to help, protect or guide others, even if they don’t like it
• Feels good

He says, “too much criticism of others can make you mean-spirited and cynical”.  Cynicism is the enemy of optimism and happiness.  It makes us aware of how easy it is to give in, give up.  Parenting a child, or even an adult, with PWS can often have this reaction.  Tiredness sets in, cynicism takes over, and the thought of another year with all the same battles just seems to be not worth getting up for. 

One of the best resolutions (New Year's or just today's) we could ever make is to be kinder to ourselves.  If we let go some of the expectations that come with ‘critical’ parenting, learn to appreciate and enjoy the smallest of achievements, we can re-charge our batteries and set different goals.  Goals that aren’t bothered by what the next person is doing or achieving; goals that aren’t rooted in materialism, or comparisons. Achievements that are as uncomplicated  as helping your daughter get her balance to slide down a gentle waterfall—just like everyone else.

Potter-Efron suggests, “make a goal for the next 24 hours to notice as many good things about the world and about other people as you can—do this every day for a month”.

It’s good advice for the weary and the cynical!

                                           

Residential Woes

Residential care is one of the most important things we need to think about for our children as they reach adult-hood.  This is something that IPWSO has taken very seriously, and, after two international conferences specifically designed for caregivers, a fantastic resource has been developed, "Best Practice Guidelines for Residential Care".  I wish this resource had been available when my daughter (now 27 yrs) first went into residential care. 

She has been in residential care since she was 18 years old.  At first, I thought she could manage in a small house by herself (with 24 hour staff care), but somehow this didn't work out.  Everything that could go wrong, went wrong.  She wanted to cook for herself (with supervision) and I taught her how to stir-fry food, something that was quick and easy.  But on many occasions she burnt the food and had to cook more (thus depleting the weekly allowance).  On other occasions she blamed staff for taking food from her fridge & cupboards.  She wanted to entertain and cook for friends, family and neighbours; she wanted to to try new foods, new recipes, and to do her own shopping.  After 4 weeks of this, we changed the rules.  Food would be cooked outside her house and brought in daily.  I did this myself for a number of years until staff took over and provided meals cooked off-site. 

Because meals were now controlled, she took to pan-handling in the neighbourhood.  She was extremely opportunistic and found money very easily (staff learned quickly not to leave cars unlocked, handbags unattended) and was blamed for missing money (creating denial melt-downs).  She executed brilliant reasons for needing hand-outs, money, cigarettes, extra food and so on.  One of the best was that she was a "solo mother with 3 children under 5 years old - one with a broken arm, the other who wet the bed every night, and the third who was just staring school.  Their 'father' looked after them during the week, but dumped them on her during the weekend and she had no food to give them."  When this ruse was discovered, "Elder Abuse" had already been called in as she was going around to not only choose the food from the cupboards herself, but cook it at the neighbour's house.

We shifted her to another house (still solo, but with 24 hour care) where there were no shops close by, but the neighbourhood was not great and neighbours were bullies.  She also managed to give her staff the slip and find new avenues of food, money, and, now cigarettes.  Six months later, we found another house with a beautiful little garden, no shops, and on a corner, so only one neighbour.  Again it was staffed night and day, but by now, my daughter really did have the hang of how to play staff off against one another, frighten them, and bully them.  There was a serious complaint laid, police involvement, and a court decision that she was not able to live in the community under the current circumstances.  She was moved to a very secure place where everything was locked down, and where there were a mix of clients who had also been through the courts.  None of these clients had PWS; she was the only one.  For her, to live with other people under such strict rules, was a big wake-up call.  This lasted a year, with a transition farm-house for the last few months.  I was proud of the way she had 'learned her lesson', but not proud of the fact she had put on a lot of weight.  This last home was full of big, strong, men who ate big meals.  She was happy about that! 

During the year she was there, I spent a great deal of time searching for providers for when she got out, who would be prepared to abide by the latest IPWSO Best Practice Guidelines for Residential Care.  I spent a long time combing the Best Practice Guidelines for the best ways they would fit our New Zealand culture.  I changed a little, but not a lot.  After some months of talking to providers, we chose one who seemed very enthusiastic and ready to take on the challenge.  We put an extreme amount of training and support into the staff (much from the IPWSO Best Practice Guidelines) and brought in two clients with PWS (one, being my daughter, and the third is without PW) and staggered their arrival over a period of 2 weeks, and sat back to watch.  The honeymoon period lasted another 2 weeks and these girls had the staff exactly where they wanted them.  Basically, as slaves!  They would sit around all morning in their jamas, ordering cups of coffee, expecting breakfast when they deigned to get up, morning tea, lunch, and so on.  No, they didn't want to go out and exercise; no, they weren't interested in showering, or getting dressed...

Back to the Guidelines and the application of more rules.  Exactly as the Guidelines suggests.  Staff learned to call the tune, put rules in and around everything - if the girls weren't up by a certain time, breakfast would be cleared away; if they didn't walk, swim, exercise, there would be no priviledges.  There followed a couple of weeks 'shake-down' period where staff stood their ground, and finally, the girls got used to the idea that routine would be followed and priviledges earned.  Things are now going much better as staff have quickly learned to cope with the cleverness of two young women who know their way around.  Some staff were worn out quickly, some handled it well.  There have been tough times, as well as the funny side of life. Best of all, they are happy and losing weight!

It was a risk, introducing a new service provider to the world of PWS, but one that has been well worth the taking.  Having staff interested in the syndrome, willing to be helpful, and willing to be forgiving over minor transgressions (particularly the swearing) has been paramount to this home's success.  Things won't always be easy, and the good old learning curve will always be steep, but by following the Best Practice Guidelines right from the start everyone has had a chance work together.



Would you like to holiday in the Dolomites?

Other than the very rewarding work of being on the IPWSO board, is the great pleasure of finding out where in the world we are next meeting!  Our board meets once at our three-yearly IPWSO World Conferences, and, generally, once inbetween.  Although it might sound extravagent, we generally manage to get support with our venue and accommodation, relying on wonderful international friendships.  This year we met in Giorgio's (our CE) home country, Italy, a couple of hours north of Venice at Laggio di Vigo di Cardore, way up in the Dolomites.  While we were here, we learned that this wonderful place is offering family holidays for special groups... and we're included!

Casa di Laggio high above the village



This wonderful old 'holiday house' used to be a sanitorium for those with TB, but is now used as a holiday place for those wanting to experience the great walks, ski-fields, and explore the beautiful mountains. 

This year will see an enormous upgrade of the cassa with complete refurbishing which will encourage groups to visit and stay.  In this regard, IPWSO will be organising family holidays for anyone with PWS and their families.  Can you imagine a more perfect holiday?  And we will be able to offer a very reasonable family rate!  The menu will be designed for those with PWS in mind, there will be activities for children, and time to relax for parents.  Interested?  Watch this space.... More on this later! 

 

A Note from Japan



Description=Yachounomori Garden, Tatebayashi-shi(city) Gunma-ken(Prefecture), Japan [http://www.sibazakura.com/ 群馬県館林市 野鳥の森ガーデン] |Source=[http://www.flickr.com/photos/13910409@N05/3456398565/ Sakura and Moss P)



Wikipedia states: Every year the Japanese Meteorological Agency and the public track the sakura zensen (cherry-blossom front) as it moves northward up the archipelago with the approach of warmer weather via nightly forecasts following the weather segment of news programs. The blossoming begins in Okinawa in January and typically reaches Kyoto and Tokyo at the end of March or the beginning of April. The beautiful blossoms are enduring metaphor for the ephemeral nature of life. Cherry blossom is an omen of good fortune and is also an emblem of love, affection and represents spring.

From Eiko Shoji comes the following message to all our IPWSO family:

"Thank you very much for forwarding messages from the IPWSO members to me. I learned that our friends all over the world were praying for us. Certainly their prayers gave us strength, and were answered.

I'm really grateful to you all being so thoughtful and helpful for us this time. I think Japan will still have to go through a difficult time for some time, but it is a great encouragement to know that our friends are always with us."

Eiko also told us that all their "PWS families" are now accounted for and are safe!  We are so happy to know this and we hope the beauty of the blossom can bring hope for new life for Japan.