Guilt

How can I talk to you about guilt?   It's not an easy subject and I feel awkward talking about it, but sometimes when I stop and analyse my feelings, they seem to be conditioned by guilt.

When my daughter was born, I grieved - we all did - for the daughter we were expecting and didn't get.  Time goes by and the grief calms down.  It never disappears entirely because there are hundreds upon hundreds of little things that other children do that remind you your own child can't; but it abates.  It becomes replaced with pride in what your child can do, rather than what they can't.  Suddenly, you have this amazing opportunity to see life from another perspective and you stop searching for those rose-tinted spectacles that made life the way you wanted to see it, and you clean the lenses of your old glasses, and suddenly get a whole new view.

Most of the time it's rewarding, it gives you courage to go on, it fills your heart with love for your child who tries so darn hard to do everything in life that they can.  Then there are times when your 'life spectacles' cloud over and it takes time to get back the right perspective.  The knock-backs are still there, but can be seen differently and learned from. Yet, even the smallest of knock-backs can still hurt.

"I visited my niece yesterday, Mum.  It was lovely, and she is so sweet.  I played with her on the floor, but she didn't smile at me, just at her own mother.  And the clothes I bought for her, well, they don't want them.  They've got enough clothes."

And immediately I feel hurt on her behalf.  I know how she has shopped in all the second-hand places for these little clothes.  I know how she has bargained for them and I know how carefully she has washed them all, hung them out to dry, folded them and taken them as gifts for her niece.  

"Mum, I hope you don't mind, but I have been thinking about not visiting Grandma much any more.  (Grandma is in a retirement home.  She is frail and forgetful.)  I just don't think she knows me, really.  She tells me she does things like play bowls, and I know she doesn't.  And she never says goodbye to me."

And immediately I feel hurt again.  I know how she plans her visits to Grandma, how she takes her little things that she thinks she might like.  I, too, wish Grandma was not like this.  I feel guilty that my daughter is not loved enough.  I feel guilty that I am not loving her enough to prevent even the slightest knock-back.

So, I asked, "Did you mind when your sister said not to bring any more clothes because they have plenty?  And do you mind not going to see Grandma, now that she is old and forgetful?"

And here's when I know my feelings of guilt are totally unfounded -

"No, not at all.  I know they've got heaps of clothes!  They're already spilling out of the cupboard!  And no, I don't mind about Grandma - I don't want to upset her."

Why can't I have babies?

This was a question my daughter used to ask me when she was around 10 -13 years old.  It's heartbreaking to break the truth, but parents will often hear this sad refrain and wonder how to answer.

Here are my thoughts:
It's true that females with PWS are unlikely to regularly menstruate unless they have sex hormone therapy and this is now a recommended treatment because it helps strengthen the bones, help prevent kyphosis (forward curvature of the spine) and helps make the person feel more like their peers;  more 'normal'.  Many girls will still menstruate, even lightly and irregularly, and if sexually active, do run a risk of pregnancy, but a large percentage of our PWS population will not have sex hormone treatment, will not menstruate, may never be sexually active and still desperately want their own babies.

Girls with PWS love babies - so do the boys, but the girls have a strong nurturing streak as often seen with their attraction to anyone's baby!  Many also have large collections of dolls and act out their fantasies.  This may extend to fantasies about boyfriends, and I mean really strong fantasies that they insist on being true!  This calls for patience and understanding.  If it gets more unrealistic and even confrontational, then the collaboration of parents with professionals, school teachers, counsellors, and the wider family (particularly people whom the child admires and sees as an 'expert') to help unravel the fantasy even to the point of explaining, for example, that "although, because of Prader-Willi Syndrome, your body works a little differently from others, so that you may never have children - and many, many women in the world don't, or can't - but your sister (cousin, friend, etc) may still let you help care for her baby.  You may well become an aunty to lots of children!"  If the child hears the same story from all, then they generally accept that what they are hearing is correct.  When the child is ready to understand more about their condition, you can either do this slowly, bit by bit, yourself, or seek professional help.


The collaboration of parents with professionals helps legitimise the situation of PWS;  the same story from counsellor (or school) and parents, is often more easily accepted as being ‘ok'. This acceptance will play a major role in if, how, when and where this human sexuality is expressed.  I personally think there is a need for honest, uncomplicated sex education that is two-way.  In other words, listening to the child/teen/adult's concerns and questions, as well as giving the educational side of things.  Softening the blow is always good, but remember that people with PWS are not dumb, they are quite astute and frequently need to hear the full, detailed story!

Deep water.  Yes, often it is, and I know I've written about this in a previous blog and I've been through the whole "I want to have sex" drama with my own daughter, but before I proceed, I just want to reiterate that this is a personal issue for each and every person, and for each and every family.

My daughter decided a couple of years ago when she was in her mid-twenties, that she and her long-standing boyfriend (and that's another story... I mean, she insists he's her boyfriend, yet she hardly ever bothers to see him unless there's a present in the offing) were going to have sex.  This was nothing to do with babies that I could see, it was all about sex.  Great, I thought.  Just what I need, and in spite of the numerous blocks I tried to put in the way (visit to the doctor for sex health education, visit to the sex clinic, sex talk from me, sex talk from her residential manager), she was determined.  To cut a long story short, we (her parents, and her residential staff) agreed it would be ok.  So armed with a box of condoms ("will this be enough, Mum?") she and her boyfriend had sex.

When I next spoke to her, I asked her how it all went (well... you know our kids, they're always willing to share!).  "Did you sleep together?" I asked, and in all innocence she replied, "No, he went home!"  I tried again, "Did you and .. make love?"  Her reply, "What's that?"  It was at that point, I remembered her concrete thinking.  "Did you have sex?"  I asked.  "Oh!  Yes," she said, "twice, but the second time it wouldn't work."

It was all I could do to contain myself.

"We're not going to do it any more, we'll wait til we get married," was her final comment on the subject.

It led me to think about all of this.  Somehow, all she was wanting to do was to be like the next person.  She was acting out a fantasy.  A curious situation, I admit, but she was behaving like a teenager wanting to explore her own sexuality.  She was well out of her teens, but her knowledge about sex was still exploratory and, as far as she was concerned, needed to be tested.

As I said before, everyone's different and I've heard a lot of stories from parents about this subject, but I think the hardest part is the very first time you hear, "Why can't I have babies?"

A kitchen with no food in it...

By MH

When we were young and beautiful (kind of) my husband and I acquired a site in the city where we lived.  We then embarked on an 18-month collaborative adventure with a team of architects, engineers and builders with the aim of creating the house that would become our home.  Shortly after becoming pregnant with my first child (who was later diagnosed with PWS), my husband and I moved into this new house.  

As is the norm where we live, the kitchen was central to the design and intended as the primary communal living space.  Just as the kitchen was designed to be central to the house, food was envisaged as central to the kitchen. So, a large open larder along one wall was intended to provide both easy access to food and a means of displaying food.  

Obviously, we would planned our home differently had we known that our son was going to be born with PWS!

For nearly 10 years since our son's birth we have (largely unquestioningly) accepted the vision that inspired the original design.  The kitchen is occupied more than any other room.  We have used the open larder for food storage.  We have left a bowl of fruit permanently on the windowsill.  We have entered the house through the back door, which leads into the kitchen.   While we have had to exercise some caution (e.g. we don't leave our son in the kitchen on his own) we have largely used the house as we would have had our son not received a diagnosis of PWS.  

Over the recent holidays I finally began to change things by removing all food from the kitchen.  Where did I put it?  Fortunately, the architects had included a small utility room near to the kitchen.  Designed to hold ugly but useful items it has become our new food storage area.  It's isn't large enough to accommodate the fridge and isn't ideal for other reasons, but it works reasonably well.

The open larder and erstwhile food storage spaces in the kitchen are also more than capable of accommodating what has been taken from the utility room.  So there are hammers and screwdrivers in place of cereal, and weighing scales and a first aid kit in the place of tinned food.  It looks ugly, and probably bears no resemblance to what the architect had in mind, but it works better for us.

Did I enjoy reorganising my kitchen?  Not particularly, it just felt like another chore.  However, in the course of my morning's work I reached two conclusions:

1. My family has been far too willing to conform to existing norms and infrastructures that clearly don't suit our lives.  Just as the cultural norm of a food-filled kitchen acting as the focal point of a home does not have to be endured, there are also many other norms and practices that we can and probably should resist. 
2. I have completely accepted PWS. I don't resent having to have a kitchen with no food in it.  I don't resent the fact that my life is (in many ways) different to the life I dreamed of as I planned my house.  I don't feel sorry for my son, my other family members or myself because of his diagnosis. Our life is different to what we expected and PWS is one big reason why, but that's fine.

Incidentally, around the time of my kitchen reorganisation I explained the concept of New Year's Resolutions to my son.  When he appeared not to understand my initial explanation I asked him if there was anything he didn't like about his life in 2013 that he would like to change in 2014.  He was very clear that he didn't want to change anything.  While this could be interpreted as an absence of creative thinking on his part, or an unhealthy obsession with sameness and routine, personally I choose to interpret it as a sign that he's happy with his life too! 

Happy New Year!

I want to go home now

Once upon a time I never thought I'd hear those words without assuming it meant back home to our house.  They were often said, and most frequently when everyone else was enjoying themselves on a long-planned outing, usually to the beach.

"I want to go home", my daughter would say.  "I'm too hot" or "I'm too tired", or anything that seemed designed to interrupt an otherwise pleasant day.  And so it was that outings became truncated, or even avoided and often my husband would take the older siblings on their own holiday - tramping, or skiing, or out to the beach - while I would stay behind with our youngest, thus avoiding having holidays cut short, or "ruined" (according to her sisters).

Separate holidays in our household became the norm, with me taking the youngest off somewhere for a couple of days special time together (often dropping in on other parents who had a child with PWS), and the older ones having some Dad-bonding time.  It worked rather well - no arguments, no fighting, no locking food away and losing the keys - in fact it's something we still do years later (yes, and we still lose the keys...).  We have the celebratory holidays together: Christmas, Easter, birthdays,  but I've noticed that the youngest daughter (now 29) really struggles with anything over 5 days.  Why?  Well, I think it's because she now has her own home, with her own things around her, her own cat, and staff who genuinely like being with her and don't mind repetitive conversations, lengthy window-shopping excursions, or just hanging out with her.  For sure they have 'off' days when arguments become heated and rules are broken, but she would still prefer to live her own life, visiting our home when she wants to.

It's such a relief to know this is her choice and it's something she is completely happy with.  It's been the best residential option so far and, as she has matured, it seems to have become happier.  The staff are very open with her, and with us, but I no longer jump at the ring of the telephone thinking it will be some crisis I have to deal with.  Best of all, she's lost 10kg this year.  She enjoyed her Christmas, was happy to see her small nephew and niece, was happy with her presents (and the promise of a holiday later in the year), but after 5 days when she said, "I want to go home now", I knew it was not to ruin things, but merely a choice she could happily make.

Skepticism and Ignorance

Blog by MH

I have a confession to make, and this might be seen as a long moan about PWS and food, but....  my son (who has PWS and is 9) is thin.  In fact, he is not just thin, he is very thin.  The descriptors scrawny, slight and spindly all seem appropriate.  And that's with his back brace on.   With it off he looks even more fragile.  Most of the time I think my son looks like he needs a good meal.  If asked, no doubt he would agree!

I say this neither as a boast nor to suggest that my family has somehow mastered weight management in PWS.  My son, after all, is only 9, and I do not assume that his weight will always stay like this.  But, right now, he bears no resemblance to the overweight children with PWS whose images I was introduced to when he was diagnosed.

One might think that my son's weight (low, but appropriate for his height) would be a cause for celebration.  And, certainly, I am happy that he is not carrying excess weight.  But I have concluded that being thin with PWS also causes problems.

 

For a start, it's very hard to convince non-professionals to take hyperphagia, the threat of obesity or even stomach necrosis seriously when the only person with PWS they have ever encountered appears as my son does.  I routinely encounter extreme skepticism when I explain that my son must have less food than others, cannot have any junk food whatsoever, and cannot be granted unrestricted access to food lest he binge.  When, on rare occasions, we eat in restaurants and I begin by removing half the food from my son's child's portion I fully expect to encounter raised eyebrows.  More worryingly, I suspect that even some family members don't fully believe my assertions about the need for such a strict food regime, despite my having repeated the message at every available opportunity.

In addition, and ironically, the manifestations of hyperphagia that are visible are often attributed to the restrictive diet imposed by Mom and Dad, rather than seen as evidence of hyperphagia itself.  So, when my son scrapes his fork off his plate 30 times after every meal to ensure that he hasn't missed anything or demands an extra pea because one has escaped from his plate, this is interpreted (along with his skinny stature) as evidence that he isn't getting enough to eat.  

Not being believed doesn't particularly bother me on a personal level ...I know enough to know that managing my son's food is the right thing to do.  But it bothers me because if people don't accept the reality of hyperphagia and the need for calorie control then I can't allow them to mind my son.  So, for the most part, he can't attend groups or parties or stay with family members without me or his father shadowing him all the time.  It bothers me too, because if I am not believed in relation to these issues regarding my son's care then I might not be believed in relation to other issues regarding his care either. 

Whereas, in my experience, non-professionals find it hard to believe in hyperphagia and the threat of obesity, I also fear that many professionals can't see beyond them.  I recall one such doctor cheerfully welcoming my son into his clinic by saying "I don't even need to ask how you are, I can see just by looking at you".  Excuse me! What about his skin-picking, perseveration, anxiety attacks, sleep apnoea, low muscle tone and scoliosis?  Do none of these matter as long as he is thin?

The reduction of PWS to one element bothers me too because I need professionals to support my son in relation to all his needs.  If hyperphagia and obesity are seen as the key problems associated with PWS and these appear under control, then that offers a rationale for service providers to start denying services.  

The saying, "a little knowledge is a dangerous thing" seems very apt to me when it comes to many of the professionals my son meets.  No knowledge at all, coupled with skepticism towards my explanation of PWS, on the part of non-professionals is equally worrisome.  However, I have concluded that the biggest problem is neither skepticism nor ignorance of the range of PWS issues, but an unwillingness to learn that might be - just might - due to plain old laziness.  The truth, of course, is that PWS is complex and if people with PWS and their families are to be really supported, then we need both professionals and non-professionals to accept this and, ideally, educate themselves about PWS.  Or, if that is too much to ask, at least believe the stories we tell about PWS and how to manage it.

Sorry, but I did warn you that this was a moan!