Vagus Nerve Stimulation

On looking back through my notes taken at the IPWSO Conference, I want to describe a little further the topic of Vagus Nerve Stimulation and what the study and researchers want to do to take this further.  The reason for the research was to see whether there would be weight loss in PWS, as previously discussed.  This didn't happen, but there were other positive effects.  The slide below summarises:

"Vagus Nerve Stimulation is used in epilepsy and depression.  It is surgically implanted and works for 30 seconds on, and 5 minutes off.  There have been reports of weight loss as a side effect (in other patient groups).
A study was done (in Cambridge, under the guidance of Professor Tony Holland)  for three years from 2009 to 2012 with three participants: Katie, Simon and Jennifer.  They travelled to Cambridge on a regular basis for many different tests and measurements."  One of the participants was able to negotiate the travel by train and bus by herself.

Slide showing where the implant was placed, what it looks like, and a picture of the ward (on right)

: What does the future hold?  The scientists/researchers want to:
"1. Undertake a formal trial involving 12 people with PWS to test:

• Whether VNS reduces the frequency and severity of problem behaviours
• If it results in improvements, how it works;
• Whether any improvement in eating behaviours are secondary to the changes in other behaviours.

2. Outline application to be submitted next week (end of July) for government funding to undertake this research.

3.  Researchers would like to hear from anyone with PWS from age 16 years and over and/or their family if they might be interested in being part of this.  Contact:  Tony Holland  ajh1008@medschl.com.ac.uk "

Message from Yosuke, Self Advocate from Japan

My name is Yosuke.  I am twenty-one years old and I study social welfare at a College of Health and Welfare in a University in Japan.  I have PWS and am fighting against the devil in PWS.

I have magic words for fighting.  I learned them from my best friend’s grandfather:

Makete oboeru sumou kana

It means “Failure teaches success.”

Katte kabutono o wo shimeyo

It means, “Don’t let your guard down even after you’ve won.”

And this,

Naseba naru nasaneba naranu nanigotomo naranu narikeri

It means “You can do anything if you try.”

This one is special to me.

These words help me to stop my overeating.  Whenever I get “I wanna eat!! I wanna eeaatt!!!” I remember these magic words which I have written on a card and I am carrying it with me in my bag.

And when I walk, I am always conscious of the way I walk. It improves the metabolism, and increases perspiration.  I have actually lost weight using such a walking method.  I showed my father the walking method, and he was glad that it worked on him, too.  Therefore, I believe that the other children with PWS who are having the same trouble can get better.  I am walking with consciousness on my way to and from school, at school, and anywhere.

This is how I walk.  I follow the rules which I learned from a walking lesson called “10 important points to remember for beautiful and healthy walking.”

1.       Do not lower your eyes.  (Keep them horizontal.)

2.       Lightly pull up your belly.  (Enhances breasts, raises hips, and tightens waist.)

3.       Raise toes and land on the heel.  (Prevents falling.)

4.       Rub your knees together lightly. (Prevents bow-legs)

5.       Stand and walk with vertical balance.  (For a shapely figure.)

6.       Basically walk with shoulder-width strides.  (For a smart walk.)

7.       Use your sole evenly.  (Prevents cold feet and swelling legs.)

8.       The three points – the shoulder, the waist, and a knee – should be on as straight line.  (Improves stiff shoulders, a back pain, and a knee pain.)

9.       In the image of describing 人or “person” in Chinese character.  (To check balance)

10.   Feel the bases of your legs and arms in around your stomach.  (Makes your legs look long.)

My happiest experience in my life was when my teacher said, “Come to my room and consult me whenever you are being bullied”, when I was being bullied about my body shape at a high school.

The most painful thing that happened in my life was when I was teased, and was looking down and crying, a teacher said, “Don’t sleep in class.”

The hardest thing when on a diet is that there are always worries about when my “devil” might come out.  For example, I worry that even though I know I will gain weight, I might not e able to stop myself from buying food.

Although it is not easy, I always think about food calories and how I can cut them down.  Whenever I am hungry and want to buy food at a supermarket, I have to check labels and make it less than 300 kcals.  I am always thinking about it.  This is the hardest thing.

When you see a person like me, with the same syndrome, and is about to buy extra food, I would like you to say, “You should stop.  If you stop now, you will have a better future.”

Although controlling myself is not easy, it is easier when I have aims and when I am enjoying it, so I would like to think of the ways which I can relax and enjoy.

For example in a Subway Sandwich, there is a service which you can ask for more vegetables with a low calorie sandwich.  I enjoy buying it, together with a calorie zero drink.  Moreover, I enjoy having bony parts of fish (which are called “ara” in Japanese) for dinner so that I can take time in eating them clean with my chopsticks.

Conference Day 4

Always at a conference there are one or two things that really stand out for you, and I'd been waiting for this to happen.  My curiosity had been spiked by a talk to be given by Prof Tony Holland about Vagus Nerve Stimulation, something I'd never heard of before.  What happens is that something similar to a pacemaker is inserted just beneath the skin on the chest.  It is designed to release electric impulses which stimulate the vagus nerve in the brain.  This type of treatment is used for patients with epilepsy and, during trials, it was noticed that patients lost weight.  So a trial was set up for 3 adults with PWS.

Presentation of this talk started with a brief introduction from Prof Holland and then each person with PWS spoke about their experiences, and then the parent spoke about the noticeable differences in behaviours, weight loss, appetite suppression etc.  The results were interesting.  Sadly, there was no weight loss, but there were noticeable changes in behaviours cited by both those with PWS and their families.  There was a very noticeable decrease in anxiety and stress, and also a decrease in the interest in food.  Tests were carried out at the beginning of the trial showing a timed session of "all you can eat" and done again after some months of treatment.  In the second "all you can eat" session, the interest in food, and consumption, was lower, although there was no overall weight loss.

What interested me most was hearing from the very articulate three adults who had undergone this treatment.  Each spoke highly of their increased confidence and lessened stress and all recommended this to anyone with PWS!  One lass said that without this measure of self-esteem, she would never have been able to get through what was the happiest day of her life - her marriage!

What more could you ask!

Conference Day 3

What can I tell you about today?  One of the most interesting issues in PWS which has turned up again and again, is this conference is about "delayed gastric emptying".  In other words, the contents of the stomach,  in the general population, take around about an hour to go through and into the intestines,  but take much longer in PWS, taking one hour 40 minutes.  So what does this mean?  It seems it could explain the gastric reflux that some suffer more than others, and the slower emptying of the stomach can cause a build-up of food which, if a person goes on an eating binge, can contribute to gastric necrosis (where the walls of the stomach are extended so much they thin out and can burst).  Also, the slower emptying can build up in the gut causing chronic constipation which is something  many people with PWS suffer from.  After listening to a few speakers who all mentioned this issue, but in different contexts, it all started to make a lot more sense.

Also very interesting was work done by Dan Driscoll et al on the nutritional phases that our guys go through, from birth to adulthood.  They did some tests with children who all had the same amount of calorific intake with no change and noted that the weight tended to suddenly increase at around 8 yrs of age.  They measured the amount of Ghrelin in the system (an appetite increasing hormone in the stomach) and found it was present in increased amounts which coincided with the sudden weight gain although appetite and calories had not been increased.  The inference is that obesity begins before the appetite increases, ie Ghrelin is probably involved in the increase of body fat, but not the hyperphagia.

I listened to an excellent talk by Jan Forster about insomnia, and her second talk about the need for routine. More of this on the blog later.

There was a blip in the catering front at lunch-time where catering had been done for far fewer than there actually were.  We were milling around outside waiting for sandwiches to come and without fail, everyone commented on how it must feel to have PWS!

Then there was the celebration dinner - held in a huge marquee with dancing and dancers (from a special dancing troup of wonderfully talented dancers with Downs' Syndrome) and everyone enjoying themselves.  Parents I spoke to all said the same thing, how wonderful it is to meet parents from all around the world.  Not only that, there are plenty of opportunities to speak to the professionals as many of them stay longer than just their two day conference and speak at the parents' conference too.  The pictures tell the story...

Conference, Day 2

Fitzwilliam College, Cambridge

Pictures are of the grounds of Fitzwilliam College in Cambridge.  It's one of the newer colleges so the buildings are more modern than the older ones.  The weather, as you can see, is fine and hot.  It has been this way for many days now with temperatures reaching 30C.  Thankfully, two of the main rooms where the conference is being held, have airconditioning...

Entrance to Fitzwilliam College

Day 2 saw the completion of the Scientific Programme and the Caregivers' Programme.  I was interested in reading some of the posters.  There was one from Israel about pre-natal diagnoses of PWS and whether there are vital 'clues' in utero that might indicate the presence of PWS.  These came mainly from various measurements of the foetus when compared with a normal growth rate.  There are differences in growth, head circumference etc and so now it may seem possible that in the near future pre-natal tests can  in the done in the first few weeks of pregnancy.  I learned that since this has been put into practice for Down Syndrome, the percentage of DS births has diminished considerably.  It is surely an ethical dilemma.

A long-term follow=up study has been done on patients previously presenting with psychotic illnesses, looking at the recurrence of any further incidences and at the general health of the individuals since their partiuclar episode.  Of 20 patients, mostly with the UPD (uniparental disomy) diagnosis, only two had experienced a recurring eposide.  Of these two, one was UPD and the other was PWSDel (deletion).  The majority of patients were taking psychotic medicine, and ten out of 18 were taking more than one type. The conclusion was that relatively few people had more than one psychotic illness; that after regaining their health they maintained it, and that medication played an important role in helping maintain this balance.  This was a study done by Prof Tony Holland et al.

Quite a lot of information is now being gathered as to the often adverse affects of anaesthetic on those with PWS.  Having come through whatever operation it was, there are now some worrying anecdotal stories of patients displaying odd and different behaviours.  Talking to other parents, I found other cases of this happening.  The patient can present with hallucinations, confusion, poor sleep, violence, bizzare behaviour and so on.  It is suspected that these are triggered by their response to the anaesthetic.

I was interested in a poster looking at the amount of depression suffered by parents of children with PWS, particularly the mothers. thirty-nine completed the survey, of which 8 were fathers,  The results showed that 36% showed mild to moderate depression and severe depression was not found.  Personally, I found this rather comforting - it must mean that the support, information, and knowledge that is available today is much better and more helpful than it was some 30 years ago when many of us faced the complete unknown.

(Some views around Cambridge)

Conference: Day 1

From our Japanese children

The first day of the conference started with the Scientific presentations and, alongside this was the Caregiver Conference.  I am simply not able to report on everything (but there will be a report on the conference at a later date), so this blog will just be me talking about the things I have heard and seen.

Although the Scientific conference is always the first place you look to for any new developments, my first interest is always to do with how we can best manage and care for those with the syndrome.  Today I sat in on two of the scientific presentations that interested me, and the rest of the day was with the Caregivers.  The presentation "Can Oxytocin Help in Prader-Willi Syndrome" was of interest to me because of the studies being done to see whether oxytocin, in the form of a nasal spray, will help to modify "behavioural temper tantrums, perseveration, obsessive compulsive behaviours, manipulative conduct, sudden aggression and abnormal food seeking".  Thirty-one participants enrolled in the trial conducted at the University of Sydney, Australia.  Eight participants completed the trial with a lower dose; 17 participants completed with a higher dose.  The age ranged between 12 and 30 years and participants were selected because they had difficult or challenging behaviours.

Behaviours were evaluated daily by a primary caregiver:  hyperphagia, temper outbursts, skinpicking, obsessive and compulsive behaviours and under-reaction to pain.  At the end of each phase the carers also completed questionnaires about the individuals' general behaviours, sleep patterns, social skills and emotional liability.

After all the clinical trials were completed, the answer to the question, "Can Oxytocin help in Prader-Willi Syndrome" was, (and I quote) "No."

Apparently there were no differences noted in any of the above behaviours.

I guess this is disappointing since we all hope for something that will help those with PWS to be less ruled by their behaviours and become happier people, fitting into society.

After that, I sat and listened to part of the Caregivers Conference where a lovely young man called Aneil who lives in a residential setting here in the UK spoke with his caregiver about his life.  These are some of thing things he told us:

• Know and understand me.  
• Don't label me, I am individual.  
• Listen to me and advocate for me.  
• Prioritise the things that are important TO me and be realistic about what is important FOR me.

Somehow it always helps the balance of things to actually hear from the people we are always talking about!

.... More tomorrow!

Linda