This week is PWSA UK Awareness Week. The Prader-Willi Syndrome Association UK want to spread the word about the rare chromosome disorder. They want more people to know about the syndrome, in an effort to allow children and adults with PWS to be understood and accepted, and to make more people across the country aware of what the condition means for PWS people and their families, friends, and carers. Palms
People often talk about being 'overwhelmed' with emotion. For most, it’s just hyperbole. But if you know anyone with Prader-Willi Syndrome, you’ll be aware it’s not just an expression; it’s a reality.
Emotional meltdowns are a common PWS characteristic, and they are hard to watch, hard to explain, and hard to deal with. Imagine what they are like to feel.
Recently, the subject of emotions came up at a talk on Alzheimer’s I attended, where the speaker used a description vividly applicable not only to dementia but, it seemed to me, to PWS too.
What I learned was this: apparently the one part of the brain that remains unaffected by any form of dementia is the limbic cortex. It’s the most primitive part of our mind - sometimes referred to as our ‘lizard’ brain - and it's home to our base emotions. The speaker explained how sometimes Alzheimer’s can attack the parts of the brain that control our reasoning, our sense of what’s appropriate, our understanding of how to act towards others, whilst the lizard brain is still firing on all cylinders. And this is why elderly dementia patients can be overcome by their emotions - becoming angry, upset, terrified, and even violent.
The speaker used their hand to describe what happens. Imagine your palm is your lizard brain, and this is where all the big, overwhelming emotions are: love, anger, sadness, fear, joy. Now imagine your fingers and thumb are the parts of your brain that keep your emotions in check. Fold them down on your hand, slowly, to make a fist. This is you, keeping your emotions in check, as you often need to do in life. This is you taking the conscious decision not to punch that mouthy work colleague in the gob. This is you, ‘gathering’ yourself when making that Oscar speech, and not sobbing and snotting everywhere, mentioning no names, Gwyneth Paltrow. Now spring those fingers back up, and pretend you can’t fold them down. This is what it is like for many people with dementia: they’re palms open, primal feelings to the fore.
Sound familiar? PWS people are DEFINITELY palms pushed forward, fingers locked straight kind of guys. And those of us who look after them have to fold and clench like it’s going out of fashion because letting our own primal reaction loose is just about the worst thing we can do. When someone with PWS is desperately sad, it won’t help if you let your sadness flood out to; you’ll run out of Kleenex for a start. If someone with PWS is shouting and screaming with anger or frustration, responding by letting your inner Hulk out is only going to escalate things. And you’ll never be able to wear those purple shorts again. (Oh, actually, I forgot, there is one occasion when it’s OK to go primal, and that’s when our palms out people are overcome with love. It’s fine to let your lizard brain loose and reciprocate overhugging, and over-I love you-ing. This is the exception that proves the rule).
So hold up those hands, all you PWS parents and carers. And clench those fingers down, to practice keeping your emotions in check, because someone’s got to.
High five me. Keep palm and carry on. It’s kind of like the Black Power salute. I might get a special glove.
This week is PWSA UK Awareness Week. The Prader-Willi Syndrome Association UK want to spread the word about the rare chromosome disorder. They want more people to know about the syndrome, in an effort to allow children and adults with PWS to be understood and accepted, and to make more people across the country aware of what the condition means for PWS people and their families, friends, and carers. Eatiquette It’s Awareness Week and I’ve been trying to share some knowledge about Prader-Willi Syndrome. The basics - for the vast majority of people with the condition - begin and end with food.
So I’ve come up with some etiquette advice about food. I'm calling it eatiquette.* Here it is...
Keep food out of sight:Don’t snack in front of people with PWS unless you know it’s their snack time, too. If you’re having a party, maybe the food could be kept in the kitchen, behind a closed door, rather than out on the table from the start. Never leave the PWS person alone with food. If it’s your responsibility to supervise them, watch them closely; they can be unexpectedly sneaky. If it’s not your responsibility, give the minder peace of mind by doing something really simple: move the bloody food.
Keep food out of mind: Don’t talk about that lovely curry you had last night, what you had for breakfast or how you could ‘murder some chips’. You’re not ‘famished’, you’re just a bit peckish. People with PWS are obsessed with food because they ARE properly,physically, constantly hungry. If they bring the subject up, answer any of their questions, and try to move the conversation on to something else.
Communicate: Ask parents or carers in advance about the person's meal times, menus, and portions.
Beconsiderate: You wouldn’t open a beer under the nose of an alcoholic. You wouldn’t plonk a bowl of peanuts down on the table in front of someone with a nut allergy. A whispered “Is she allowed this?” when brandishing a plate with forbidden food does not count as being considerate. Check with mum or dad first, out of earshot of the person with PWS (and be aware that their earshot can put a long distance sniper to shame).
Think of alternatives:If you’ve got tortillas and dips, get a low fat huomous and some cucumber sticks. If you’re making a birthday cake, have a low calorie Weight Watchers’ cake slice on the side. There are such things as no-sugar jelly and light ice cream. Really. Think ahead. Ask.
Be on time: If you’re having a meal with a PWS family, don’t be late. Or if you are, send a message as soon as you know. And be prepared to grovel when you get there. Trust me, it really is just easier not to be late.
Do NOT say:“Oh, one little piece of cake won’t hurt,” “They’re not even overweight,” “I don’t see what the problem is,” “They’ll grow out of it,” “They’ve only got Prader-Willi a little bit, haven’t they?”. (This last one, in particular, might result in me giving you a “little bit” of a slap).
I don’t want to be dictatorial about it. (Yes, I know that sounds hypocritical when I’ve finished that list with a threat of violence). I appreciate it’s hard for someone outside the PWS family to understand. But being thoughtful or thoughtless when it comes to food can be the difference between a day being utterly joyful or utterly awful.
The simplest and the best thing you can do is to say this: “Tell me what I can do to make it easier.” And then I’ll hug you.
Song is Spearhead - Food For The Masses *This blog post was originally entitled Foodiquette, until my friend Hannah pointed out that it should be Eatiquette. I bow to her greater punnage.
