Another day, another LD dude in difficulties.
Today it’s Dude David, who has Prader-Willi Syndrome.
PWS is caused by the loss, or failure to function, of a tiny region of Chromosome 15, so it is sometimes known as a ‘microdeletion disorder’. The relevant genetic region may be minuscule, but the effects of it being unable to work are HUGE, literally and metaphorically. The missing or non-working genes code for controlling the production of a hormone called ghrelin, which tells people when their stomachs are empty and they need to eat. People with PWS vastly overproduce ghrelin. By a miserable twist of ironic metabolic fate, they are also unusually sensitive to another ‘hungry hormone’, insulin, and are resistant to the ‘had enough’ hormone, leptin. They never feel full and are always hungry. Really, really hungry. Stomach-gnawingly, raid-the-fridge, where’s-my-dinner hungry, even if they have just eaten a large meal.
Given the chance, people with PWS can literally eat themselves to death. Some become so enormously obese that their health collapses and their body gives up. Others who have got unexpected access to food have eaten so much that their stomachs have burst, and they have died of organ trauma and internal bleeding. Given money, they will buy food in massively excessive quantities and consume it all at once. They will eat discarded food from bins, still-frozen food straight from the freezer, and non-food items, because of their overwhelming need to still that ever-present, unquenchable hunger.
Families of people with PWS learn to live with locked food cupboards, chained and padlocked fridges and freezers, even locked food-waste bins. They learn ways to distract the person with PWS from seeking food, and from indulging in the skin-picking and self harm that many people with PWS use as a diversion. They know that love is … cooking rigidly calorie-controlled meals. They eat their own meals to a schedule and forgo snacks – except in secret – to avert melt-downs in the affected person. They manage pocket money to ensure that spending opportunities do not include food items. They make sure that the person with PWS follows a rigorous exercise programme (PWS also lowers muscle-tone, lean body-mass and metabolism, so not only do affected people feel hungrier, they also burn off anything they eat at about half the rate of a typical person). They give the daily growth hormone injections that can help the person with PWS build up lean, rather than fat, tissue. Family members deal with the intellectual differences that come with PWS, learn how to answer fifty-times-repeated questions (‘perseveration’) and to cope with difficult behaviour kindly and patiently. Families set inviolably firm boundaries, but with gentleness.
Then their dudes grow up….
Dude David wasn’t keen on moving into a place of his own when he turned eighteen. He wanted to stay at home with Mum, but over the following two years or so, his care manager persuaded him that his own supported flat would be a good place. The flat was beautiful. Dude David got to decide how to decorate it and to fit it out with all the things he wanted. It was perfect.
The support package, however, was a disaster. Staff didn’t manage demands due to Dude David’s hyperphagia (wish to over-eat) well, so he rapidly gained a great deal of weight. Their failure to understand and respond properly to his needs led to a marked deterioration in Dude David’s behaviour. After only ten months, social services decided the flat wasn’t appropriate after all and took it back. Neither of the alternatives offered was tailored to Dude David’s needs: local residential provision was not geared up for PWS, and all the specialist provision was miles from friends, family and familiar support. Dude David’s mother rightly insisted that his emotional needs be given priority after all the upheaval and upset, so for now Dude David is in the local residential home, while his gorgeous, barely-used furnishings languish in storage.
Two years on from his original move into the flat, there is ‘hope’ that a specialist supplier will be creating a properly-tailored service for Dude David ‘at some point in the future’, but no word as yet on firm undertakings or timelines.
Sigh. And sob.
It was precisely because I have heard so many of these doomy-gloomy unhappy stories that I wanted my contribution to #JusticeforLB to be something positive, and so adopted Day 28 of #107days of Action for ‘Drops of Brilliance’. I hoped that enough stories might come in to make a little book of examples of everyday good practice – maybe even 107 examples. Some fabulous stories came in – of a teacher who noticed the achievements rather than the shortfalls, of support staff who achieved the Never-before-attempted – and On Ice – by refusing to admit the word “can’t” into their vocabulary.
But so far, there have been fewer than half a dozen contributions. I’m loath to believe that I, by myself, can find more sad stories than all the rest of you, amongst yourselves, can find inspiringly brilliant ones. So I’m challenging you to cap the following post-within-a-post story, with even better ones of your own.
You can leave your own Drops of Brilliance as comments on this blog or add them to the comments at #107days’ Day 28 here. And your starter-for-ten Drop of Brilliance iiiiiiis:
The Taxi-Lady Comes Up With A Belter.
Grenouille has to wear a medical device that attaches to the body and delivers continuous life-supporting medication, twenty-four hours a day, seven days a week, fifty-two weeks a year. In order to avoid site infections and skin breakdown, the device has to be moved around every three days. G’s taxi escort noticed that on some days, G seemed to be finding it uncomfortable, and realised that the car’s seatbelt was pressing into the repositioned device. Now she checks with G every morning as to where the device is today, and makes sure that G gets to sit on the side of the car where the seatbelt won’t touch it.
Simple. Thoughtful. Kind. Brilliant.
Go on – top that.
Meanwhile, I’m working on my patch for LB’s Justice Quilt.