A Question I Wasn't Expecting!

By M.H.

 

Last week my 11 year old son, who has PWS, asked me a question.  In his usual fashion the question was delivered in a meandering, unnecessarily long-winded way (why use 1 sentence when 5 will do?).  Nonetheless, his point was clear.  He wanted to know if a man who underwent sex reassignment surgery to transition to a woman would still have PWS after the process was complete.

I quickly informed him (reassured him? disappointed him?) that yes, PWS would withstand the process and would remain present.  I enquired why he was asking and he told me he was just wondering.  And then the conversation moved on.

Still, it's an interesting question.

My first instinct (when he was out of earshot of course) was to laugh.  Was this a typical pre-teen assumption that one's parents must be dim?  Had he experienced an Eureka moment in the middle of the night and assumed that his Mom and Dad had somehow missed this "obvious" way of getting PWS out of his life?  Had he mulled it over and  concluded that transitioning to a woman would be a price worth paying?  Of course, he made the obvious mistake of thinking that such a procedure would be a simple choice, rather than a necessary and complicated process required to correct the problems that occur when some individuals' assigned sex at birth does not correspond to the gender with which they identify.

 

Or should I interpret the question as a poignant one, a grasping at straws by a child who has reaching an understanding that he does not like having PWS and is mentally thrashing around in the hope of finding a way of getting rid of it?

Or is it a coming to terms with just how fundamental a part of his being PWS is - even more fundamental than his sex?

Or is it best viewed as an illustration of how little he (and most of us) understand about genetics?  In his head he reasonably connected PWS (something to do with genetics) with sex (something to do with genetics).

One of the oft-reported traits of PWS is repetitive or excessive questioning.  I get that too.  Sometimes I feel that I know what my son is going to ask before he even opens his mouth.  There are days when I feel like an unwilling participant in a bad school play, compelled to listen to one set of lines time and again and to deliver another set in return. 

My son only asked me this question once.  Considering his age and limited understanding of genetics, PWS and gender dysphoria I think it was a pretty good question too.  And I'm glad that sometimes he leaves the script behind.

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

"I don't want to go to school!"

Although going to school for the first time is usually a really happy occasion, as the years pass by, there can be that familiar cry, "I don't want to go to school!"  More often than not, there is an element of bullying going on that makes a child fearful of school.  Katherine Stanley, who has PWS, wrote a book about bullying in schools.  We wrote a blog about that which you can see here 

There are so many issues that our kids with PWS have to deal with at school so it is really important that teachers are able to recognise not just the learning ability of the child (and be able to meet their individual special needs as required), but also the social needs and how to help the child fit in comfortably.

And, having said that, it's not just the classroom teacher who needs to know, but everyone - including the relieving teacher so that they are not caught in the trap that one relief teacher found herself in.  She was just relieving for one day and she asked for a volunteer to collect the class lunches - you are all already ahead of me and yes, you are correct -  the child with PWS shot up their hand and willingly volunteered.  The school lunches only made it as far as the nearest toilet block and all were consumed.  

It is so easy to exclude the child with PWS from outings, camps, and other out-of-school activities for this very reason - that they 'can't be trusted'.  Or, 'there isn't enough staff to cover a one-on-one situation'.  It is a sensitive situation.  Do you agree with the teacher and keep your child home for the day, or do you postpone what you were going to do so that you can accompany your child?  Or do you insist that the school gives as much attention and care to your child as they would to any other child who needed special help?  It's a tough call and I have kept my child home from school on quite a few occasions in the past to avoid unnecessary confrontational situations.  I have to say that we both enjoyed our days out together and probably had more fun.

Quoting from what is written on our website, "Students with PWS are very receptive to learning and are keen to please.  In general they have good reading skills, but poor numerical skills and handwriting can be slow to develop.  They show ability to learn computer skills and often have excellent fine motor skills, for instance, many are particularly clever with jig-saw puzzles, threading beads, and many show an aptitude for fine handiwork including needlework and knitting.  The IQ level generally falls in the just-below-normal category, but often shows “islands of competence”, in other words, they might be equal with their peers in some areas, but need support to reach potential in others.

"Maths teaching needs to be conceptual, practical, and often repeated several times before there is understanding.  Teaching the use of a calculator immediately helps the level of understanding.  Once understanding has occurred, the concepts generally remain.  Like all students, they thrive on praise.

"Throughout integrated primary and secondary education, it is important for the student to have teacher aide time if this is available at their school.  Although not always available for many students with special needs, it should be applied for on all levels.  The need for teacher aides will not decrease as the student progresses.

"Teachers should be as familiar as possible with the characteristics of this syndrome, even including the genetic subtypes, as this will impact on the learning ability of the student and the teaching strategies employed."

This may require some dedicated education on your part to make sure the school really does understand PWS, and may even need you to talk to the classroom to explain why your son or daughter needs the students' understanding and support.  I remember doing this (with my child absent) by telling them a story about a child with diabetes and how although this child looked just the same as anyone else, they had an illness that could kill them if they weren't looked after properly.  Now that growth hormone treatment is much more readily available, children with PWS who benefit from GHT will definitely look just like any other child and it's this that makes it even more important for everyone to understand what it means to have PWS. 

But, they can learn and they can succeed and they can often astound you in their success.  With the right support at school, at home, and from their peer group, school can be a happy and convivial place of learning.

Of course, there may well be many things that go unsolved at school.  Like who really took Sally's school lunch money, and who was it who cut the computer cords, or took someone's prized possession, or carved f*** you into the headmaster's table...

Some things just remain urban myths, don't they?

 

A Perfect Day

by M.H.

Some days confound expectations.  Today was one of them.  My 11 year old son (who has PWS) headed off this morning to spend a day having fun with his grandparents.  He does the same thing every summer with varying degrees of success.  Some years he returns sullen and anxious, but today came home with a broad smile on his face accompanied by his equally happy grandparents.   

There is always the potential for things to go badly wrong on a day like this.  Sometimes the excitement is so overwhelming that upset becomes almost impossible to avoid.  Sometimes my son's (often unspoken) expectations about what will happen are not met leading to tears.  Sometime the stress and worry prove so exhausting that the outing cannot be enjoyed at all.  

But not today...Today, the sun shone!

The pet farm was as he remembered and as he wanted it to be.  The amusement park was fully operational and he could enjoy all his favourite rides.  His snacks and lunch were as he expected and he was satisfied with them.

Today, my son laughed and sang and talked and joked with his grandparents and glowed with happiness.  

Today, my son enjoyed a great deal of independence.  He planned the day and decided where they would go and when.

Today, my son willing embraced physical activity.  Without having told me of his plan, he decided to bring his grandparents on an unscheduled 7 kilometre (!) walk as part of their day out.   I think he was proud that he was able to do so.

Today, there were no signs of hyperphagia or anxiety around food.  My son  arrived home over an hour after his normal dinner time exhibiting no signs of worry about food or anything else.  On days like this it is almost possible to forget about PWS.  It is certainly possible to believe that it need not interfere with happiness or fun.   

Recognising and taking pleasure in perfect days and remembering  that they are possible makes harder days so much easier to tolerate.  

My daughter and PWS

by M.H.
 

Last year when my then 3-year-old daughter was attending a preschool her teacher stopped me one day to report an observation.   My daughter, she reported, was remarkably understanding and relaxed around a particular boy in her class.  This boy had autism.  He was nonverbal, 3 years older than my daughter, much bigger than her, and frequently had meltdowns in class.  In contrast to the other children, who were afraid of him, my daughter would sit beside him.  She would talk to him even though he didn't speak back.  She would bring him PECS cards to help him understand when it was time to transition to his next activity.  And she appeared totally unfazed by his meltdowns.  According to the teacher, the many professionals who worked with this boy had all remarked on how this very small girl seemed to understand him and accept him just as he was.

My daughter's teacher did not know that my son has PWS.  I feel quite sure that my daughter was drawn to this boy because of her experiences with her older brother.  Listening to meltdowns, is, unfortunately, something she has grown up with.   

More recently I found my (now 4-year-old) daughter alone in her bedroom planning an elaborate junk food feast for my son's 90th birthday.  That's right - his 90th!  I asked why and she reminded me that I had once said that when people are 90 they can do whatever they want.  Apparently I had said this when she asked why her Granddad was allowed to eat junk food when she and her brother were not.  Her brother was with her at the time and had sought clarification as to whether people who had PWS were also allowed to do whatever they wanted when they were 90.  I had laughed and confirmed that it did.  Clearly, my daughter remembered this, which was why she concluded it was reasonable to plan a junk food bonanza for his 90th birthday 80 years in advance of the actual occasion.  

PWS has been a part of my daughter's life since she was born.  She understands that it is why her brother sometimes has to go to hospital, why he has to have such a small amount to eat, why he cuts his skin, and why he becomes so very upset so very often.  As a two-year-old she occasionally claimed that she too had PWS and at different times demanded a back brace, a CPAP machine, daily injections and, most importantly, my attention for her (pretend) physio routine.

 Like many parents of a child who has PWS who also have one or more children without the syndrome, I've wondered what the impact of PWS on her life will be.  I've worried that one day she will feel forced to take on the responsibility of advocating for a sibling whose needs may be greater than his parents can handle.  I've worried that she may feel neglected because her parents spend so much time with her brother.  I've worried that she will develop an unhealthy relationship with food having grown up in a house where food is treated so unusually and guarded so closely.  And I've eagerly embraced all reports from adult siblings of people who have PWS who have claimed that their experiences with their sibling were more positive than negative.  

The truth, of course, is that I don't know what the impact on my daughter will be.  Nor can I know how she will evaluate the experience as she grows older.  Despite this, I feel a need to believe that my daughter's experience in a household affected by PWS can be positive and that its (unavoidable) negative consequences can be offset by experiences that will teach her valuable lessons and help her to value and enjoy your own life.  

So, I've made a deliberate choice to interpret her actions and reactions as positives not negatives.

I felt happy when my daughter's preschool teacher told me of her relationship with her classmate who has autism.  I have chosen to believe that her experience with her brother will help her be open to all people and as a result will enrich her life and help her be happy.

I also felt happy when I found my daughter planning her brother's 90th birthday party.  I have chosen to interpret it as a sign of her growing empathy and a growing sense of injustice that her brother cannot enjoy what others can.  I have chosen to believe that these qualities will help her make a contribution to the world and find fulfilment in her life.

In evaluating her experiences of living with a sibling with PWS I also hope that she will follow my lead and focus on the positives more than the negatives.