Family Weekend - September 2013

Family Weekend –September 2013

Saturday Morning

The Family Weekend 2013 began on the Saturday with families registering with our newest trustee Antonia East and then mingling whilst having some refreshments. There were the usual information stands and Lisa Massingham and I also had a stall set up selling the Sturge Weber branded goods, including the new additions of calendars and pens.  Pam and Alan Stevens displayed the Christmas cards and were pleased that so many families purchased them and took some away to sell later. Paul Callaghan very kindly arranged for one of our posters to be enlarged for Jenny to put a display with a collecting tin in the hotel reception area. Unfortunately Rebecca and Paul were unable to attend the Weekend as Matilda was poorly. The children started the day being uncharacteristically shy but soon came out of their shells and began playing with each other which was lovely to see.

Saturday Afternoon

Following a hot buffet lunch, the young adults  attended the Changing Faces workshop run by Shirley and Michelle, while the younger ones were looked after in the crèche where the Warwick Toy Library had very kindly loaned us some toys for Saturday afternoon.  As you will have read in the last newsletter we were fortunate to receive a grant for around £10,000 from BBC Children in Need to pay toward the children’s activities. Clare Cannock came from them and Jenny took her to see the children in the crèche. She was impressed how Safehands were entertaining them and she then spent some time in the Changing Faces workshop with the older children. She later spoke to the children and parents to ask their opinion of the Weekend. She was pleased with the all the activities we had arranged for the children and especially the trip to Cadbury World on the Sunday. Unfortunately Elizabeth, from Contact a Family, was unable to attend because her son had an accident. Ruth Card, who is a local representative, kindly stepped into the breach and explained to the parents about the work of Contact a Family.  After Ruth’s talk and the workshop had ended people went off to their hotel rooms to get ready for the evening meal.
We had very good feedback from two of the teenagers who attended the Changing Faces workshop and they asked if they can do it again next time. Many thanks to Christine Bleasedale who helped enormously with organising the workshop.

Saturday Evening

Before we all had our evening meal the children were entertained by the clown, Mr Joe Ridgely. It was especially lovely to see two little girls, Yasmin and Poppy, looking so well after their TPO surgery three months earlier. They are an inspiration to us all.
During the evening Chantelle Roberts and her two lovely girls Megan and Teigan sold raffle tickets for all of the prizes that were kindly donated, for which we are very grateful.  Poor Chantelle didn’t even get time to eat as she was so busy selling tickets! Megan and Teigan were brilliant giving up their time to sell the tickets too and we are very grateful to them. They raised a total of £458.  There was also a separate raffle for the beautiful hand carved artisan rocking horse which was made especially by Ingrid Grimes’ Dad, Mike.  A massive thank you goes to all involved in the raffle. The horse raised £175.

Part of the evening also included an auction of a Sturge Weber polo shirt which was signed by the cast of Coronation Street, an enormous thank you to Marie Cavalier for organising the T shirt for us to auction.  After stiff competition in the bidding, Wayne Prewett won it for his wife Kathryn who was extremely pleased. This raised £56. During the evening Lisa handed out questions to each table for a quiz based on nursery rhymes and a prize was given to the table with the most correct answers.

Most of you will know that Lisa Massingham carried the Olympic Torch in 2012 and she brought it along to the Family Weekend.  It went down very well as lots of people had their photographs taken with it.

Sunday

Sunday was business day, after all of the children were settled in the crèche or on the coaches to Cadbury World everybody filtered into the conference room to listen to the speakers.

Dr. Colin Flanagan

Our first speaker was Dr Colin Flanagan, Lead in Oral Health from Northern Devon Health NHS Trust, who covered Dental Care relating to SW.  As well as covering general dental health, Dr Flanagan discussed possible complications with Sturge Weber syndrome. He discussed how some drugs can cause decay and showed pictures of a port wine stain in the mouth. The main challenge during dental treatment is the risk of bleeding due to the port wine stain.  Dr Flanagan discussed how good dental hygiene is essential in preventing any decay.  The challenge of getting children to brush their teeth was also discussed and some ideas were put forward about how to encourage brushing. Dr Flanagan recommended Oranurse toothpaste which is unflavoured for those who do not tolerate mint flavoured toothpaste and a Collis Curve Toothbrush which brushes more than one tooth surface at a time.  You can find more information here: http://www.colliscurve.co.uk

Dr. Krishna Das

Our second speaker was Dr Krishna Das who spoke about seizure mimics.  Dr Das gave a figure of 1/4 to 1/3 of episodes being misdiagnosed as seizures. Dr Das showed slides of different people and we had to decide if they were having an epileptic seizure. Many of the people shown did not have epilepsy but were displaying seizure like behaviour. It was explained that there are many seizure mimics that can be confused with epilepsy and that it is only epilepsy if there are recurrent seizures.
Some seizure mimics are: Pallid Attacks – where the breath is held, the most common stimulus is a painful event. The child turns pale (as opposed to blue) and loses consciousness with little if any crying. The EEG is also normal, and there is no post ictal phase, nor incontinence. The child is usually alert within a minute or so.
Reflex Anoxic Seizures (RAS) are a form of fainting encountered mainly, but not exclusively, in young children. Reflex anoxic seizures are not epileptic seizures. This is usually a consequence of a reduction in cerebral perfusion by oxygenated blood. It can be a result of either a sudden reduction in the blood flow to the brain, a drop in the oxygen content of the blood supplying the brain, or a combination of the two. The STARS website has more information http://www.stars.org.uk/ .
Dr Das went on to explain that there are 40+ seizure types and some unclassified ones and so careful monitoring is needed to get a correct diagnosis as well as the usual tests. Dr Das suggested looking on You Tube for seizure mimics.

Tracey White

The next speaker was Tracey White, the Nurse Specialist in Emergency Medication Management from GOSH.  Many of our members already know Tracey from their visits to GOSH .
Tracey talked about how it is important to treat seizures effectively in order to stop any progression. A prolonged seizure is considered to be anything over five minutes. There are different types of rescue medication which can be used, Buccal Midazolam, Rectal Diazepam and IV Lorezapam.  Diazapam can be administered either rectally or intravenously.  If a patient is non responsive intravenous phenobarbitone or phenytoin infusions may be used. Information about Buccal Midazolam and how best to administer it, can be found on the following website: www.buccolam.co.uk
Clobazam is used to break a cycle of clusters of seizures of short duration, usually in a short course of three or so days. Patients must then be weaned off Clobazam.
Tracey then handed out two versions of the A&E guide which Sturge Weber UK asked the team at Great Ormond Street Hospital to produce for our members.  Tracey asked for people’s preference for either the small or the larger version and the consensus was for the larger version. The guide is being looked at again by the ethics committee who will decide if the guide can be adapted for use by those patients who do not attend Great Ormond Street.  We hope to have a decision for you all soon.
Shortly after the Family Weekend it was announced that Tracey is leaving her position at Great Ormond Street and moving to another hospital.  She will be sorely missed by our members and we wish her well in her new position.

Varsha Siyani

The last speaker before lunch was Varsha Siyani – Speech and Language Therapist Great Ormond Street Hospital NHS Foundation Trust.  Varsha talked about the role of the speech and language therapist and language and communication difficulties in Sturge Weber syndrome.  Varsha also explained that speech therapy is not only stammering therapy,  which is probably  what most people now think it involves and that they see wide range of children and adults with wide ranging needs. They are, in fact, part of a multi disciplinary team who take time to listen to parents’ concerns.
Assessment is done via formal, informal and parental questionnaires.These results are looked at in the context of learning, e.g. SLI, behaviour. There is a liaison with local services and outcomes are fed back and an appropriate therapy is agreed upon. The patient is then monitored over time to ensure progress is maintained.
We broke for lunch and families collected those children that had remained in the Hotel in the crèche.

Maria Papadopolous

After lunch the first speaker was Maria Papadopolous, who some of you may know from Moorfield’s Eye Hospital.  Maria talked about glaucoma and discussed how having a haemangioma around the eye area can cause high pressure and the eye to expand. There is around a 50% incidence of glaucoma
There is a need for lifelong monitoring with glaucoma and it is important to monitor vision.  Pressure in children’s eyes can be unreliable so more factors need to be looked at.  The goal is to preserve a life time of vision.  Pressure can be controlled with medication, but where medication fails to control pressure, surgery is often necessary with multiple operations being a possibility.

Dr. Samira Syed

Our final speaker was Dr Syed, who again, those of you who attend GOSH will already be familiar with.  Dr Syed briefly covered SWS in general and also mentioned the discovery of a genetic mutation that occurs before birth which is said to be the cause of Sturge Weber syndrome (SWS) and port-wine stain birthmarks.
Dr Syed talked about the multiplex laser which is effective for resistant port wine stains.   This laser has two wavelengths - a high-powered pulse dye laser, and a 1064nm long-pulse Nd:Yag laser.  Both wavelengths are ideal for treating vascular-related lesions.  Rapamycin cream is said to help prevent the recurrence of the port wine stain after laser treatment.
If the drug is used in conjunction with pulsed dye laser treatments on port-wine stains it might inhibit the body’s natural response to injury.
If blistering and scabbing appears after laser treatment, Bactroban, an antibiotic cream is recommended. The use of sun block is important to prevent hyper pigmentation caused by exposure to sunlight.
Samira finally handed out a questionnaire with the aim of assessing how useful the Sturge Weber booklet had been to families and to find out if they felt any other information should be included. The information was needed for a poster presentation she was making on the SWS booklet for a meeting in Melbourne in April 2014.
After the speakers we held our A G M to change the logo and Constitution
We are very grateful to Greg Williams, who once again was on hand to help setting up the Powerpoint equipment and made sure that everything worked smoothly during the day.

Sunday Evening

Sunday evening was the night for the children to wear their fancy dress and they all looked very cute.  Lisa had organised some games which included pass the parcel.  She was assisted by Alex Buchanan who was in charge of the music.  By ‘luck’ every child managed to be the last one to unwrap a present so they all went away happy.    Lisa then spent some time playing games with the children who thoroughly enjoyed themselves.  Everybody had some good food and spent time getting to know each other as the children ran around playing with each other.

Monday

Monday morning was the day of the balloon release to launch the Charities’ Awareness Day, 1st November which the charity had set up along with members from other countries.  The balloons arrived and Colin Buchanan took on the task of handing them out to the excited children (and the parents seemed quite excited too).  Everyone was trying to guess who was inside the Mascot Bear costume (thank you Carl!!) All of the balloons had tags attached and the one which went furthest was to receive a prize.   It was a lovely bright day and we all assembled outside for a photo shoot before the balloons were released.  Most of the balloons went straight up into the sky but one or two became stuck in a tree!  Hopefully the wind released them later that day.  Everybody had great fun trying to keep an eye on their individual balloon.
The winner was the lovely Poppy whose balloon reached Northern Germany.  Poppy received a voucher for Toys R Us and the sender, from Germany, received €20.
The committee would like to thank everybody who attended the weekend and provided useful feedback, there would not be a charity without you or your support. We were pleased that so many families attended and that so many stayed on the Sunday night as this was the first time the Weekend had been extended to include Sunday night since the Pioneer Centre in 2005. Last but not least we would thank the speakers for giving up their time freely to come and give their presentations and answer questions.

Lynn Buchanan