Greetings everyone. Sorry again for the delay for the newsletter but I have had little time to write it. Please send me anything that you want or think should be included in the newsletter since this newsletter is for your benefit and not my ego.

 

The issue has an international flavour with a report on the International CHARGE Syndrome Conference in Miami and the DBi world conference in Slovakia.

 

Family Day is in a couple of weeks (18th September) and both Jeremy Kirk (medical advisor to the group) and Gail Deuce from Sense (specialist multi sensory impaired teacher) are attending. This is an opportunity to ask questions in a relaxed and informal environment.

 

I am slowly getting your email addresses, sometimes by circuitous routes. Please if you do not receive the newsletter by email and have an email address contact me – especially those whose email addresses have changed. I also have quite a number of non functioning email addresses.

 

By not giving me an email address means you could be missing out on information about the group, research etc since sometimes I have news/info or requests that by their nature are time critical.

 

Simon

 

 

 

NEWS

 

Family Day

 

This is being held again at the Royal School for the Deaf in Cheadle Hume on Sunday 18th September at 10.00am. For those not able to arrive there without a journey remember that the group will pay for overnight accommodation 10 minutes away from the school. I reiterate that this year we have both Jeremy Kirk and Gail Deuce attending. Contact me for details.

 

Update – Jeremy Kirk

 

Jeremy now has funding to establish a clinical screening service for CHD7 (CHARGE gene) in Birmingham. It is hoped that this will be a national service. Those parents who previously gave him blood samples of themselves and their children need to give retrospective consent for testing. Please contact me if you were one of these so that information sheets and consent forms can be sent to you.

 

With the research testing I recall Jeremy advised me that out of 26 samples 14 tested positive for the mutation in the CHD7 gene.

 

New Groups

 

There is a new family group that has been set up in Belgium. Its website is www.asblchargevzw.be and the contact email address is plehanceassocharge@yahoo.fr

 

There is also a forum in Germany that can be found at

http://www.charge-syndrom.de.vu

 

 

 

PEN PORTRAITS

 

There are no pen portraits because none have been sent to me. I NEED them so write about your child, grandchild or if you were born with CHARGE write about yourself. However I have received a story from Jack Murray-Bell who is 7. He writes many of these stories and I believe that these are some of the few times he stops talking!

 

The Blue Planet  - Jack Murray-Bell (7)

 

One afternoon I went in the rocket. I went up a ladder. I went in a control room. I sat down. I pressed a button. The rocket blasted off into space.

 

I was space sick because the rocket went very fast.  I saw lots of planets out of the window. I saw a good planet so I pressed the button to land.


 

I got out of the rocket and I was on the moon, there was an alien with 7 eyes. I didn’t see him because I was looking for scary things and air the alien could see me. He was frightened so he jumped off the hill.

 

         

 

International CHARGE Syndrome Conference, Miami – Simon Howard

 

Flo and I decided that we would take the girls to the conference in Miami at the end of July. This is a bi-annual conference that has been held on 5 previous occasions and this year there were over 500 people in attendance. Once we had raided Jessica’s savings for the flights the enormity of taking 2 girls aged 5 (with CHARGE) and 3 on a 9-10 hour flight and then face the joy that is US customs began to dawn.

 

Having nearly missed our flight due to an accident (not us) the journey went smoothly – shorter than expected flight and through US customs in 5 minutes! We arrived at the conference hotel (if any of you know Miami Beach when you walk past any hotel pool there you walk out on the beach) 3 days early to accommodate the children adjusting to jet lag. Very soon we recognised people from Australia and the States that we have had email contact with. During the course of the next couple of days more families (and some professionals) arrived some we knew many we didn’t. As Meg Hefner, geneticist with CHARGE Syndrome Foundation said when she looked out of the window from their meeting “the pool was full of ours” ie CHARGE families.

 

I should point out at this stage that both Flo and I are active on the CHARGE listserve (see below) and have over the past few years got to know other parents, some young adults with CHARGE and professionals throughout the world via the internet. In addition as part of what I do for the UK group I have made contact with other CHARGE groups across the world.

 

As you can imagine the hotel was full of children with CHARGE and therefore it was fantastic that as a parent you were not made conscious of your child’s idiosyncrasies or have to explain them – mind you it’s scary when a 6 year old child with CHARGE makes a beeline across the child pool for your nipple ring!

 

In the same vein there was no ‘us and them’ with the professionals attending the conference. CHARGE Syndrome is still a little known syndrome, although awareness is increasing and for example Sense now consider the main cause of congenital deaf blindness in the UK is CHARGE. As a result it is better to describe those professionals who have an interest as having a passion. Everyone was very open and accessible and much useful discussion happened at the pool bar. Over the duration of the conference I had such important discussions as the quality of American chocolate (David Brown), the problems of being a busy soccer mum (Dr Kim Blake), where the queues were at Animal Kingdom (Dr Jeremy Kirk) whilst Jim Thelin (audiologist) and I demonstrated Aikido wrist throws to Flo and Nancy Hartshone during the poster sessions to the bemusement of other conferencees. There are some others I cannot disclose!

 

Enough light heartedness.

 

 Who presented at the conference? One of the key presentations was by Connie van Ravenswaaij on the gene discovery and this was later followed by a question and answer session involving her, another colleague, Baylor Institute (a US research establishment) and Genedex (a company offering gene testing services).

 

What can I add to what you’ve read here before? The Dutch testing originally was very wide including children who perhaps had only a couple of elements of CHARGE and it is clear that those children who tested positive for the gene mutation were already clinically diagnosed.

 

Connie again made it very clear that the gene mutation is de novo ie the parents are not carriers and that it is a genetic aberration in the child. As a result this is of no significance to a sibling.

 

To show how variable someone can be affected there are twins with CHARGE whose symptoms are very different.

 

One very positive point that came out of these presentations is the fact that Connie, Baylor and Genedex are looking to assist each other rather than keep secrets.

 

The other keynote speaker was a 21 year old Australian woman with CHARGE Belinda Arnell whose presentation was very funny without belittling her problems.  In addition to this there was a question and answer session with a


panel of young adults with CHARGE. I did not attend this session but got the impression that the parents who attended raved about how wonderful the panel were. I also understand that problems and issues that the panel members have in life (some quite considerable) were glossed over. It is interesting to highlight here comments made by Joff McGill in the paragraphs on ‘Range of abilities’ and ‘Young people’ in his piece on the Dbi conference below.

 

Outside of these there were probably too many presentations and on several occasions there was a choice of more than 2 to attend. Flo and I only once went to one together that was David Brown’s presentation ‘Emotional Health as a Key to Successful Education and Development’. It is the third time I have heard David speak and I have also read a couple of articles written by him. All of these have revolved around a theme and this is not a criticism, because every time I hear or read David I learn or realise something new.  On this occasion it emphasised the need to minimise the stress in our children’s lives. This stress relates to their sensory input be it too much or too little. To use a quote from his article in the American Journal of Medical Genetics “Time spent trying to reduce stress levels, and trying to give the children acceptable strategies for doing this for themselves, must be one of the most precious gifts we can offer them, and one of the biggest favours we can do ourselves as educators, therapists, and family members.”

 

 

 

There were specific sessions for siblings and grandparents. Although my daughter Elly is only 3 I can see how her sister Jessica’s needs have impacted on her. It is important for parents to take into account how CHARGE (or any other disability for that matter) affects siblings. At the bottom of the article I have set out some sibling support details.

 

Kim Blake’s presentation on osteoporosis clashed with Jeremy Kirk’s on growth – not the best scheduling so they decided to do a double act and present 2 one hour presentations in one hour!  Again Kim’s presentation emphasised the problem that our children have (based on her recent study). She explained that calcium intake in preadolescent years is a key factor in the development of peak bone mass and what the risk factors were in CHARGE – delayed/absent puberty, poor diet (low calcium and vitamin D intake) and inactivity. It’s not difficult for us to think why these factors are present in our children.

 

The solution? Keep our children active and ensure sufficient calcium and vitamin D. Use supplements and oily fish. Also ensure that your child sees an endocrinologist. This is where Jeremy comes in!

 

His presentation was 2 fold relating to growth and puberty. Apparently growth for us occurs in 3 stages and for children with CHARGE there are potentially problems in all of these be it nutrition, growth hormone or sex hormones. He also pointed out that if the gene CHD7 is expressed in the pituitary gland then this is likely to be the cause of shortness (however there is little data).

 

Again puberty raised its head not just in short stature but also in the lack of it in children with CHARGE. It should be assumed that any child with CHARGE is going to have a problem with puberty.

 

Although Jeremy went through the testing and issues of growth hormone deficiency and its rectification, likewise puberty we arrive back at the issue of osteoporosis potentially in young adulthood – so growth and in particular the going through of puberty are so important.

 

On to a presentation I didn’t attend but Flo thought hit the spot. Tim Hartshone spoke about Mistaking courage for denial: family resilience after the birth of a child with CHARGE Syndrome. As many of you know Tim is a parent of a teenager with CHARGE as well as being a psychologist. This examined ones responses to your child, its situation and the way he/she is sometimes viewed by professionals. You’re damned if you’re strong and you’re damned if you are weak ie if unhappy and discouraged you need help or if happy and optimistic you are in denial. Tim also spoke how roles can change in relationships. As a side issue he commented that neither he nor Nancy do the housework. This appears to be not unusual (as Flo and I are trying to find time to send the kids to relatives so that we can try to get some order back into our house let alone the 4 years of paperwork that needs proper filing!).

 

2 years ago the theme of the conference related to behaviour. This one seemed to have a communication theme.

 

Given that Jessica doesn’t have feeding issues Flo and I did not go to Sara Rosenfeld Johnson’s presentations – Feeding & Speaking and Sensory deficits and how they impact on feeding and Speech. I know that these were very


popular and she also spent a lot of time testing children at the conference. From her printed material I can say that feeding is very important to speech


since the muscles used are the same. She went through types of food to use to progress feeding and also how to use different straws both in diameter and shape – those novelty straws do have a use!

 

The communication theme continued through such presentations as Reading behaviour as communication (by Sharon Barrey-Grassick and Tim Hartshone)  ‘acknowledging behaviour as communication can be an effective way of preventing challenging behaviours from emerging’ and ‘as parents…. we must learn to read the behaviour and respond accordingly’; The role of visual processing in seeing, learning, relationships and communication (by Kim Lauger and Ann Gloyn; Range of effective modes of communication (case studies); How to enhance communication and learning in children with CHARGE Syndrome ( Dr’s Jan Dijk and Arno de Kort).

 

For new families attending there was a multi person presentation called CHARGE 101. From this I should highlight Meg Hefner on ‘Characteristic and Diagnostic Criteria’ that ‘no feature in CHARGE is sacrosanct. In other words any given feature can be absent or present and, if present, maybe mild, moderate or severe.’

 

You will have to forgive me but I do not have space to explain Jacques Souriau’s - An ecological approach to development; Jan van Dijk (as above) he has a CD rom available or George Williams’ - The neural crest in CHARGE Syndrome despite being fascinating to either Flo or I. There were also quite a number of other presentations.

 

What has the UK group got out of me going to the conference? Although I have sought contact with other groups it makes a huge difference to actually put faces to names. As Meg Hefner said (paraphrased) ‘when you have met someone you get a feel for them and you realise whether a comment made by them is serious or in jest’.  Following the conference I have been able to exchange/seek information. Those with emails will know I have sought participation in a worldwide study in charge. It will enable me to obtain information for you that otherwise is unavailable. Previously these links have recently enabled people to take part in an osteoporosis study (see report on Kim Blake).

 

Mind you David Brown in his closing speech did comment that people would only hear Miami and think holiday and not conference. So what are you to know? 

 

 

I have copies of a number of the presentations from the conference. If anybody is interested in them please contact me, but please be patient. I will forward them when I have some spare time.

 

CHARGE Listserve - CHARGE@yahoogroups.com (worldwide discussion forum for parents, professionals with an interest, people with CHARGE etc)

 

Group for siblings of children with CHARGE  (moderated by a young adult with CHARGE) - http://groups.yahoo.com/group/CHARGESibs

 

Sibs Helpline (Mon-Thurs 10-4) tel 01535 645453

Sibs, Meadowfield, Oxenhope, West Yorkshire, B22 9JD

Website: www.sibs.org.uk

 

SibNet@yahoogroups.com  (this appears to be for older siblings)       

 

SibKids@yahoogroups.com (this appears to be for younger siblings ie under 18

 

Sibling Support Project  - www.thearc.org/siblingsupport

 

www.sibspace.org

 

 

 

6th Dbi World Conference in Slovakia – Joff McGill (head of Voluntary Services, Sense)

 

The beginning of August saw 400 people descend on Presov in the East of Slovakia for a wonderful Deafblind International World Conference. The range of people there - deafblind people, families, professionals - meant the networking opportunities were endless and there were always interesting people to share experiences with, learn from and to generate new ideas.

 

Of particular interest was the fact that the programme included 6 hours worth of sessions on CHARGE.  Around 28 people attended all six hours of


this 'stream' including our very own Carol Thomas.  The participants ranged from those who had significant experience of working with people with CHARGE and others who wanted basic information about the condition.

 


Perhaps the most interesting thing to come out of it was a set of 'competencies' needed for a team of professionals working with a person with CHARGE.  This came out of a talk Carol Thomas gave about what she expects from professionals and an exercise about what are the knowledge, skills, qualities and attitudes that a perfect professional needs.  What was interesting about the list was that those skills and qualities the group decided were core (knowledge of the individual, knowledge of and sensitivity to the person's communication, belief in the development potential of the individual, an active listener to all involved in support to the individual, a good observer, empathy, knowledge of child development, time and patience etc) was that they were universal – the sort of skills and attributes we could expect of anyone.  Of course within a team of professionals providing support specific skills and knowledge are needed, but all the team need to have these core 'competencies'.  It is hoped that the Deafblind International CHARGE network may develop these competencies and point budding CHARGE professionals in the direction of learning opportunities so they can develop them.

 

Other key themes emerged from the 'stream':

 

Multi-sensory impairment

 

It was recognised throughout the stream the importance of understanding that people with CHARGE are truly multi-sensory impaired and the impact this has on how they make sense of the world around them and the strategies they develop to balance the sensory input they get. Reference was made to several of the articles in the American Journal of Medical Genetics.

 

Networks

 

Participants in the stream drew the networks they have with people with CHARGE, their families, the professionals that work with them, and organisations.  We concluded that for people with CHARGE to fulfil their potential these connections/networks need to:

 

·                Have a clear purpose

·                Be open to new knowledge

·                Have clearly defined roles and responsibilities (it is hard to keep networks going sometimes)

·                Recognise the impact of the internet but not to forget that access is not universal

·                Share experiences and inspire each other

·                Maximise international links

 

Ulla Holmqvist-Ang from Sweden helped us in this section.

 

Range of abilities

 

We reflected on the fact that children with CHARGE have a huge variety of abilities and disabilities so we must never underestimate them, but also recognise that it is hard for some families to always see images or hear stories of the CHARGE 'high' achievers.  We were told this point was made at the recent Miami conference as well. 

 

Interactions

 

With the help of Jacques Souriau we recognised the importance of interactions between parents and child, between children, between children and others around them.

 

To encourage interactions we need to:

 

·                Sustain activities - allowing the child to dip in and out at their own level

·                Ensure activities are dynamic and allow inspiration

·                Provide environments that are strong, secure, but not over protective

·                Allow exploration and risk taking

·                Encourage and recognise communication at all levels.

 

Allow children to find their space

 

Corinne Blouin from France and her colleagues helped us see the importance of allowing children to find their own 'space' and position where they are comfortable and can give their attention - they even physically demonstrated some positions CHARGE children use - I for one will not forget the sight of three French presenters wrapped around table legs.  Corinne also reminded us of the importance of touch for children to feel the emotions of others.

 


Young People

 

We watched a video of young adults with CHARGE getting together for the first time in the UK and exploring their hopes and fears for the future. We reflected that as children get older and more adults are identified with


CHARGE we need to focus on young people and young adults as well as children; not just to focus on their health issues but most importantly their hopes, fears and dreams.

 

People said they enjoyed the stream - at the end everyone was asked to write down on postcards some of the things they were going to take home and do differently as a result of the stream.  These were posted to participants so when they got home they would have a reminder of all the things they said they would do when they got home.  Ideas people came up with as a result of the CHARGE stream included: try and establish CHARGE network in Estonia, join the DBi network listserv/e-group, read articles on CHARGE, discuss core skills and how to incorporate them into staff training, establish a newsletter in Denmark about CHARGE, share the resources with others and tell fellow professionals about the various networks, re establish links with David Brown.

 

If just a few of these are followed up then the six hour focus on CHARGE will have been very worthwhile.

 

             


 

RESEARCH

 

There is a worldwide study about to start of children with CHARGE syndrome from the ages 9 through to 21. Little is known about the impact of CHARGE on adolescent development.  The study will be looking at issues related to puberty, behaviour, adaptive behaviour, and executive function, as well as other medical issues that may develop during adolescence. This is being conducted in the States, Canada, Australia and here.

 

If you are interested contact me although it maybe that the participants have already been selected.

 

             

 


 

FUNDRAISING

 

Message from Flo. She and Alexis (newish mum of boy with CHARGE) have had a mad idea of organising a CHARGE group for the Hydroactive 5K women’s challenge on 3rd September 2006 in Hyde Park (you may recall that Flo ran this last year to raise money for the group, this year she ran for Sense and at some point next year she is intending to run a 10K for the group). There are also runs in Birmingham and Liverpool. Any takers for the 5K women’s challenge? It can be run, walked or push buggy/wheelchair etc.

 

Sponsor forms

 

If anyone is looking to raise funds for the CFSG Carol has sponsor forms available.

 

 


 

FEEDBACK

 


As ever please give me any comments – positive or negative - about the newsletter.  I can only improve it if you give me feedback.

 

 


EMAIL ADDRESSES

 

Please see my introduction.


 

 

 

CONTACT DETAILS – Please note additions/changes below

 

CHARGE Family Support Group Website

http://www.widerworld.co.uk/charge

 

(Please note that until the end of September the website is not available. The family that host the website are moving and until they are in their new home at the end of the month the website is not accessible.

 


Carol Thomas (Chair)

Telephone: 01484 844202 (note new number)

Email: cajthomas@btinternet.com


 

Simon Howard and Flo Njeru


Telephone: 020 8265 3604             

Emails:         si_howard@hotmail.com or flonjeru@hotmail.com


(note if you are not using a hyperlink for my email it is si_howard@hotmail.com – the underscore cannot be seen on the link)

 

Any problems with my email please use Flo’s or my work callingtonestates@yahoo.co.uk

 


Yvonne Arnold (Committee)

Telephone: 01708 764533

Email: eveian@btinternet.com


 

In Scotland Elaine Murray- Bell is available as a point of contact.

Telephone: 01387 250284.

Email address is:              Jmurraybel@aol.com

 

In Wales Heather Jones is available as a point of contact.


Telephone: 01267 233960

Email:          howardsway@btinternet.com

 

The views expressed in this newsletter are not necessarily those held by the CHARGE Family Support Group.