Behaviour in PWS - where? What? Why?

by Linda Thornton

In .52 of a second, Google furnished me with a choice of 158,000 articles on behaviour in Prader-Willi syndrome.  It is overwhelming and if I were a new parent, I would not know where to start.  In fact, I probably would yell at Google, close the page and burst into tears.  There are scholarly articles, there are profiles of behaviours, advice on managing behaviours of pre-schoolers, toddlers, pre-teens, teens, adult and so on.  After 20 pages of Google and in amongst all the PWS articles,  suddenly I find a page on growing marijuana and by this stage, I’m thinking “what a good idea!”

Personally, I think that finding a PWS Association either online or in reality, is the best possible thing to do.  To be able to talk to someone face to face, or on the phone, or even just an email, will result in a calmer, easier-to-understand answer than trying to assimilate information from 158,000 different articles.  That’s just frightening.

The best possible thing you can learn about behaviour and PWS is that children and adults are all different.  They are people first, and just happen to have Prader-Willi syndrome.  Knowing your child, what motivates them, what they love and who they love, what scares them, what makes them anxious, and so on, is the first step towards understanding behaviour.  When you stop and think about it, everything we do, from getting up in the morning to going to bed at night, invokes behaviour of some kind or another.  Our behaviour sends messages to others in many different ways – no matter whether we have a disability or not.  It’s how we interact.  

I’ve watched the behaviour of my 32-year-old over the years and can read her like a book.  But I can also do this with my other children as well, the only difference is that my 32-year-old has more pages in her book.  That’s life.  Whether her behaviour is challenging, whether she’s testing whatever system that’s been put in place, or whether it's her every-day behaviour, for me the most interesting thing is how she interacts with other people.  I used to worry that she would ‘snap’ if someone said or did the wrong thing – and by this I mean a stranger, not someone she is familiar with because the more familiar she is with someone, the more likely she is to let loose! 

The other day, for example, I listened to her on the phone to her bank.  She was polite, very clear in her request to transfer funds and knew exactly what she was talking about.  She quickly and efficiently changed one automatic payment into another.  No problem at all.

I’ve heard her ring and make a doctor’s appointment; phone the library and request a particular book; she manages a visit to the Vet with her cat perfectly well and asks all the right questions and makes sure she knows the answers.  In an emergency (her concept of one) she will get herself down to the Emergency Department at the local hospital (with a staff person) and wait until she can be seen.  She knows when she is ill (see previous blog on gastroenteritis).  By being able to do these things she avoids becoming anxious, frustrated, and angry.  It has become so clear to me over the years that by first teaching her about making good choices, she is able to do so much more for herself.

Sometimes, in the middle of a catastrophic outburst I tend to forget everything I’ve just said above and feel as though I’m clinging to the life-raft for all I’m worth.  Just getting through to the other side of the meltdown is all I’m hoping for at this stage!  I hate it when these happen because I know how terrible it is both for her, and for the person she is focussed on.

I began to wonder what sort of chemical imbalance might be in place when these meltdowns got under way.  So I asked Tony Holland about this and about his research with vagus nerve stimulation.  He responded,

"Our work on vagus nerve stimulation suggested that people with PWS essentially have a a low threshold for such outbursts and also impaired emotional control - in other words people with PWS are easily triggered and once it starts such an outburst more easily builds up and leads to loss of control when compared to people without PWS. This appears to be improved by vagus nerve stimulation. The vagus nerve is part of the autonomic nervous system of the body that manages our response to threat - what we think vagus nerve stimulation is doing is normalising that response. The other rather different issue we are beginning to look at are two chemical (neurotransmitter) systems in the brain - GABA and glutamate - the former is inhibitory and the latter, excitatory. It may be that an imbalance of these two systems is important - a new study we are starting uses brain imaging to explore this hypothesis"

So, although I know that there is much behavioural research going on with Oxytocin* (Google: 1.10 seconds, 115,000 articles) and the Vagus nerve stimulation** (Google:  .72 of a second, 60,600 articles), I can’t help but think that deep down it pays to really understand your child and what makes them tick.  You may be surprised.

 (If you would like help with behaviour issues and would like to talk to our specialists about this, please email us)

*Maithé Tauber Oxytocin research

**Tony Holland Vagus Nerve Stimulation

The Awfulness of Meltdowns

The other day my darling (adult) daughter had a huge meltdown.  She lives in residential care so I wasn't there to witness it first-hand, but I had the full works via phone.

It shakes me to the core whenever this happens and I just never really get used to it.  Things can go along so well for such a long time, then suddenly, something jumps out of line and no power on earth can stop the mountains being moved.  She phones me in order to play me off against the staff.  But the more I stand my ground and don't give in to the desperate cry for more food, a cigarette, or whatever, the worse it gets.  Whatever started this all will fall by the wayside and I become the focus of this powerful deluge of verbal abuse.  Apparently I simply don't love her, I don't understand and never have, I'm the worst possible parent, I'm useless and *%@^* so on.

 It hurts.

I feel useless, guilty, anxious, frightened, alarmed, and often really scared.  I just don't know what is going to happen next.  Sometimes, when the situation really escalates and she becomes a danger to herself or to others, the police are the only ones whose presence can calm her down.  I guess that's the authority hierarchy thing.

I shouldn't feel all these things because I know what happens in a meltdown; I know the pre-cursors to it, I know how one should handle these things, and I know they will blow over.  But I am unable to take off my parent 'hat' and disassociate myself from what is happening to my daughter.  I know for a fact that she is feeling "guilty, anxious, frightened, alarmed, and scared".  I know she is unsure of what is going to happen next, and I know that she tries to take control of the situation, but can't.

Having PWS must be one of the greatest stressors imaginable, not just for families, but for those who were born without that tiny little piece of the 15th chromosome.  So unfair.  Because if we don't take control of this situation, if we give in to that 'just one extra potato, or whatever' then we are promoting a life of constant battle.  As parents, we need to trust those who have studied PW and know what to do in these situations.  As parents, this is quite hard to do.

This picture of parents of a child with autism can be exactly how parents of children with PWS might feel as well.

Our IPWSO Famcare committee is about to start a study of de-escalation strategies or how to handle a major meltdown.  We want input from other parents, so if you are willing to share (we don't need your name or that of your son or daughter), we would love to hear from you.  Write to me separately and I will answer.

IPWSO is lucky to have such a strong team of clinical specialists who will answer our calls for help.  We are lucky to have a strong team of committed parents willing to share their knowledge and understand what others are going through.   So I would like to gather together what we as parents can teach others about what happens before, during, and after a meltdown and what we have learned to do that might help alleviate this, or sometimes even actually avoid the whole disaster.  When looking through the web for more information, I came across this from the Autism association.  I thought it was pretty much word-for-word something we might use as well!

Linda Thornton
Communication Coordinator, IPWSO

Your Christmas Shopping is now Complete

I wonder how many of you have a daughter or even maybe a son with PWS who enjoys Christmas shopping so much that unless it is done by mid-August, the world as we know it, will cease to exist?

I don't know what it is - maybe it's genetic? - but it's been like this forever.  Well, ever since the concept of Santa Claus was rudely demolished by the older sisters.  This terrible news seemed to force the issue for youngest daughter (the one with PW) who took it upon her small shoulders to become utterly and completely responsible for the purchase, wrapping, and distribution of Christmas presents.  So anxious did she become that pretty soon the idea of shopping one week before Christmas became unmanageable, and it quickly became one month before, then two, then three, and now if shopping is not complete by mid-August, as I said before, the world as we know it will collapse and crumble.

Nothing has changed, as far as she is concerned, and her own shopping is, as you would expect, now complete.

So, having wrapped that lot up, she advanced on me with her brochure of Avon products, a pen, and a notebook so that I could make some detailed choices.  Nothing, it would seem, would prevent her from accomplishing the result she wanted - to sell me as many Avon products as possible, so that she could benefit from the kickback that being an Avon representative would give her.  Did I mention that she was a self-styled Avon rep?  Earlier this year, she answered an advertisement in the little local paper and signed herself up; along with her ever-patient caregiver who has been delegated a 'silent supervisor' who handles the money and oversees the distribution and collection of brochures.

Next, she advanced on her rather frail grandmother complete with Avon brochure, pen and list of people that Grandma would probably want to give presents to.  Thankfully, daughter was extremely patient and gentle and the feedback I had was that she was very thoughtful and had some excellent ideas.  For which I am eternally grateful!

When she was very little (daughter, that is, not Grandma) she would also take it upon herself on Christmas Day to distribute all the gifts to everyone.  Not just hers, everyone's.  As you might imagine, this also included the exact time when this should be done.  Which meant that to avoid a fully impacted disaster, we had to learn patience and give in to this ritual.  It was not appreciated by Older Sisters.  However, as she grew older and slightly more wiser, she has allowed this ritual to be changed to her distribution of gifts into designated piles which people are allowed to open at their leisure.  Phew!  Disaster avoided.

However, the early Christmas shopping I doubt will ever be changed and as she comes from a family whose best skill is shopping-at-the-last-minute, she will always be the odd one out... unless she manages to convert everyone to the joys of brochure shopping with Avon!

It's always about the food...

Here it is, Sunday early afternoon, and I hear, "Is someone coming down for lunch, or are we just not eating today?"  It's my daughter, the one with PWS.  I know I won't be able to finish this blog until she's had lunch... so...

Later:   This week has been a little tiring insomuch as food has been even higher on the list of priorities.  We've had a wedding and a baptism to attend and, of course, this is always around food in one way or another.  I remember when she was very little, before she was diagnosed, we went to Church one Sunday, and I took her up to Communion whereupon she thrust out her hand to the Vicar and demanded to be fed, "I want bread!  I want bread!".  Unfortunately, at the same time, she upset the chalice of red wine all down his white cassock.  I didn't know quite where to put myself and quickly carried her down the aisle to our seat, with her demanding very loudly all the way back, "I want bread! I want bread!"  I don't think it went down all that well with either the Vicar or the congregation.

Trying to avoid food issues is a huge struggle for most of us: parents, caregivers, family or friends - whoever the person with PWS is with, and it is quite possibly the most tyrannical issue to deal with.  Society demands that food is a natural part of socialising.  As our sons/daughters get older, they know this and adapt their tactics accordingly... they know that in a crowd of people, parents are less likely to refuse them food as the thought of a socially embarrassing outburst is not what they want.  My daughter, particularly.  What's more, she's extremely adept at manoeuvering her way out of sight, and picking her times when I'm deep in conversation. I find social events a huge trial and would rather stay home than face the test!

Last week as we were struggling to reach 35 lengths (walking) up and down the pool, the chief topic of conversation was, yes, food.  It's all about what she wants me to buy, whether I have this, or that on the grocery list, when I'm going to get it, what looks as though it's about to run out etc.  Whenever she comes shopping with me, and this is something I do try to avoid if at all possible, I notice that she always puts two of whatever into the shopping trolly.  Two packets of this, two packets of that.  I always take one out, saying we won't starve and the car won't break down, so we can always come back.  But she seems to harbour this inate fear of running out of food and won't stop her habit of storing.  She also has a cat which is here with us at the moment - that cat has such a backup of food that I swear we could feed all feral cats within several miles.

I asked her the other day whether she felt full after her meal.  She said she did.  She knows the difference between feeling hungry and feeling full, or even just feeling ok.  She can even hold off and wait for a meal if she's busy doing something else.  Once all the food has been cleared away and cupboards locked, she doesn't food-seek or ask for food until she judges it time for her meals.  But she is a fully-fledged opportunist and should any opportunity for accessing food arise, she will take it.  So, it's always a situation of being 'on guard', locks secured, guests warned, and situations scanned for any weak spots. 

I know it will never go away and I know I will never hear the truth about what happened to the packet of biscuits left out on the bench, and I know there will still be slip-ups with who's got the key, and I realise I will never get the better of the social situations either, but it's amazing how quickly you learn to to use those eyes in the back of your head, practice your own sleight of hand, and to lie, yes, outright bareface lie, about things to do with food.