An Update

It has been a while since we have shared an update on our children’s health. Their health has been stable with no major changes …. well ’til last week that was …..

Last Monday evening, 29th April at 18.42pm, Annabelle fell to the ground and had her first Tonic Clonic seizure. Despite everything that I had read and been guided to remain calm, I screamed and panicked. Luckily, Andy came home and leapt into action. It was the scariest thing I had ever seen, Annabelle did not look like Annabelle. She went grey. She made hideous noises and her body was so tight. Young Georgie witnessed the event and was scared by the situation but once everything had calmed down and Annabelle went into recovery we were able to comfort her. We called the paramedics who came and checked her over and were happy with her speedy recovery.

The next day phone calls and emails were made to make Annabelle’s consultants aware of the situation. Annabelle now has a seizure protocol in place and has started medication. She has returned back to her normal self and has not had any more seizures since. We are hoping this will be the case for quite some time.

Let’s update you on our cheeky Robbie …. we noticed last October that he was struggling with his eyesight so had his consultant authorise our local optician to prescribe glasses for him. The optician advised that the glasses have a weak prescription but they will sharpen up the vision he has. Just before Christmas we noticed he was cautious of where he walked and becoming more clumsy. The school had also noticed that he was struggling to read the board, pulling things closer to him and they had also noticed him bumping into things.

We knew that his eyes had deteriorated, but by how much we had no idea. We had a hospital appointment last Tuesday, that confirmed our suspicions but it also surprised us and his consultant. Robbie’s eyesight has deteriorated to a level where he is now registered severely visually impaired (blind). He has received this registration two years ahead of when Annabelle received her registration. This is just one example of the rate that his eyes have changed,  last December Robbie was able to play crazy golf with no problem, but when we went in April he struggled, it broke our hearts watching him trying to play the game he loves.

The beginning part of last week was just a kick in the teeth, and we struggled with the heartache knowing that Battens is taking our children. This week, however, we have been busy planning fundraising events and ways that we an raise awareness. Please keep following our cause and let us know if there is a way that you can help.

image1

 

 

BFF Jurassic Walk

File 06-06-2018, 20 51 23.jpegOn the 2nd of June a team of 21 amazing people took part in the 50km challenge walk of the Jurassic Coast 100 from Lyme Regis to Exmouth.

Starting at 7.45am the team left Lyme Regis Football club and headed though the “lost world” of the Under Cliffs towards Seaton. This stretch was mainly in the shade and the ground was slippery. A few of the team members fell and were injured, yet they carried on. Just before Seaton we were greeted by our first water stop, jelly babies and coke, these were much needed.

After Seaton we headed towards Branscombe. On route to Branscombe we walked through the beautiful village of Beer, no time to stop and have a pint mind you. We carried on over the White Chalk cliffs to Branscombe.

Leaving Branscombe we faced the toughest part of the walk towards Sidmouth, the path takes you from sea level to heights of 150m, over and over again. It was exhausting with every incline and painful with every decline, it took it’s toll on our knees.

Once at Sidmouth we enjoyed the pleasure of flat ground along the seafront to only then be confronted by a steep climb to Peak Hill and then a few rolling hills into the sleepy town of Budleigh Salterton.

One last climb (we heard those words many times) took us over to West Beacon before we passed the resort of sandy Bay, we came to the Geoneedle, marking the end of the Jurassic coast with an easy flat 2km to the finish line. Those last 2km were the longest we have ever walked.

16 out of the team of 21 completed the walk, those who didn’t manage to get to the end gave it their best and were only beaten because of injuries. We had two great support drivers that rallied us along and met us at various points so we can change plasters and stock up on supplies.

Along the way we were stopped many times by members of the public enquiring about Batten Disease. Several gave cash donations on the spot – inspirational.

A great day. A fantastic achievement by the whole team. An awesome shared experience. Great awareness raising and much needed fundraising.

Bath Bomb making at Lush

We have encouraged Annabelle to think of a list of things she would like to do before she turns 18. Ticked off that list so far Annabelle has been to Florida, Disney Land Paris, she has learnt to ski and the next thing on her list is to make a Bath Bomb at Lush.

We approached the Lush store in Southampton and met with a lovely Australian girl called Mallory.  Mallory was fantastic and listened to our story,  planned the Lush experience to be tactile and full of enjoyment for Annabelle with her friends. Mallory organised it to be a gift for us to make memories and to fulfil Annabelles wish.

Friday the 13th, the day of unlucky occurrences? Not for us, the day of the lush party has arrrived. Annabelle chose two friends, her longest, closest friend Siobhan, and her newest supporting friend Ellie. Georgie also had two friends, Katrina and Jessie.  As we were walking towards the Shop the girls could smell the delights that awaited them, we were greeted by Ashleigh and Mallory.

The first activity was to make the bath bomb, the Lush product called “sex bomb”, renamed for the occasion as the Annabelle bomb. It has a fragrance of Jasmine, Ylang Ylang and clary sage with a pretty rose in the middle. The girls took it in turns to mix the ingredients together, and then each made their bombs. Annabelles words were “this is so cool and fun”, she had a smile from ear to ear as she was moulding her bomb.

The second activity, Fun Bar Pictionary. The fun bar is a product that feels like playdough but you can use it as a bubble bar, solid shampoo or soap. The idea of the the game is that someone is told an item, that item is then modelled then the other guests guess what the item is. Today, it was the first time ever, there was a twist, everybody was blind folded, allowing the girls to experience the activity the same way as Annabelle. The girls all managed to guess what was modelled by touch alone and they all said how weird it was not being able to see.

The Third activity, Bubble bar cocktail. Bubble bars are bars/shapes that you crumble into the bath and they change the colour of water, produce a beautiful fragrance and of course they make bubbles. The girls had to choose from a selection of crumbled bubble baths and come up with there own combination to be judged by their colour, smell and bubble quality. The younger girls just grabbed everything and put the crumble into their jugs, the older girls thought long and hard about theirs. Poor Sam had the task of judging. He chose Bubble Pop, a cocktail produced by Ellie and Annabelle’s came second, she named hers “Pink Sunset”.

To finish the experience all the girls got to choose a bath bomb or a bubble bar to take home. The girls loved smelling every bomb or bar they could find, Annabelle chose a fidget spinner bubble bar. All the girls left with a smile on their face, and the car journey home was full of chatter about their bombs and a rather strong fragrance from their party bags.

We would like to say a massive thank you to Mallory, Ashleigh and of course Sam at Lush, Southampton, you made the experience very enjoyable for Annabelle, and you helped us make memories that we will cherish forever. We will be popping in to say Hi very soon.

 

 

 

A blog from a guest – a member of the BFF Bournemouth marathon team

In January this year a group of us decided we wanted to run an event in the Bournemouth Marathon Festival 2017. The Festival was in October and ages away, so wouldn’t be a problem!

We signed up and wanted to run for a charity; but which one? After throwing the usual suspects into the pot, one of the kids asked: “could we run for Annabelle at school as she is blind, and we have done charity days at school for her”.

Perfect we thought, but little did we know… After meeting with Annabelle’s mum, Lisa, for lunch, we learnt about Juvenile Batten Disease and the BDFA. The more we learned, there was no going back – there was only one charity we were doing this for. We were doing this for Annabelle and Robbie, and the whole Hover family.

So training began. For some of us it was about getting back into the habit of running. But for others, we hadn’t run even 1k in a very long time and struggled!

The months went by and we were still running and training. Injuries started to creep up on us, and then all of a sudden, summer was here and plans got in the way. Every time we got together we talked about the Marathon Weekend… but it was still ages away.

Fast forward a few months and the sponsorship was coming in. The running vests and race numbers arrived – we actually have to do this!

Race weekend was here, and it’s safe to say we were all apprehensive.

The weekend started with the kids 1k run. George dragged his dad, Alex, round the course, beaming with pride – he was running for his friend Robbie! Lucy was loving every second in the lime light, waving at everyone.

Next up was the junior 1.5k. Albha, Clodagh, Maisie Rose, Maisie Minchin, and Lily running amazing races. Those twins can fly!

That afternoon it was the turn of the 10k team. To say we were nervous was an understatement! But Alison ran an impressive 46:32 mins; Lynn just over an hour; and Carriann under her 1 hour 30 target – woohoo!

The next day was the half marathon team’s turn – the big one. And how impressive! Sarah, Alex, Mike, and Simon all running amazingly! They all set brilliant times, and were still smiling while doing it. And Mike didn’t need St John’s in the end – he not only survived, but thrived!

Thinking of Annabelle and Robbie saw us all to that finish line, and as a team we are so proud of ourselves.

We all took turns being the BFF support crew across the weekend. We were there cheering each other, shouting encouragement, and getting some great photos. The support from Team BFF was outstanding and helped push us all to the finish line.

This was an amazing weekend that saw us all wear our orange vests with pride! We couldn’t have run for a better charity.

As the last runner crossed the line, we had a lump in our throats. We all did it! And actually all did it well. We may not be able to walk for a few days (and have a few minor injuries between us), but we did it for BFF.

So that was the Bournemouth Marathon Festival 2017. What next? Anyone up for 2018? Maybe the full marathon?…

One year on

This is a short blog on the anniversary of receiving Belle’s CLN3 diagnosis to reflect on what the last year has given us.

After searching for answers we received some about Belle. We were devastated.

We needed to find out if the younger ones were affected. We were distraught.

Choosing to keep Belle’s diagnosis to ourselves until we had the complete picture was one of the hardest things – we needed help, and got this from the few very close friends and family we told, but we knew so many would be there for us. Did we do the right thing? We just weren’t ready to share

Jan 23rd was the worst day of our lives … parents should never have to tell their kids these things …

Coming out to everyone with the news about Belle and Robbie’s diagnosis and likely future – relief, sadness, humility, overwhelming … we are blessed to have the people around us that we have

Anger and frustration – how we feel about no cure, no treatment, lack of structured medical support, lack of answers, slow pace of research & development, bullshit bureaucracy

We have found another family – Batten’s families

We are fighters – Batten Fighters Forever – her body may be but our girl is not giving up so there is no way we are going to just sit here and watch our kids die

We are lovers – we love our kids and will make sure they have the best possible lives and best memories to take with them wherever they go. We survive as parents because of the love they give us and our love of each other.

So at the end of this year … we have a different focus and outlook … we feel a lot more pain but are stronger because of that … thank you to everyone that is there for us

 

Lisa & Andy
19th August 2017

 

 

social++7998786483

Meet Coach David

Robbie has always shown an interest in football and sports – he would always be kicking a ball around the garden. A couple of years ago we searched around for a football club or group that would take two year olds. We found Play Sportz, a club owned and run by David Emes, that teaches all round ball skills which help develop coordination, agility, balance and spatial awareness to children aged from 2 to 5 years old.

The reason why I wanted to write this blog is to say a massive thank you to David and to introduce him to others. He is a devoted husband and dad – he has two boys and speaks very proudly of them. Beyond Play Sportz, he volunteers his time to teach children cricket and supports Wimborne Town Football Club with fundraising.

Right from the initial taster session he established a healthy rapport with Robbie. David is very good with the children. He quickly gained Robbie’s trust, Robbie loves him.

David’s support has gone beyond running the Play Sportz sessions that Robbie attends. He noticed that I was looking upset one week and he knew that something wasn’t right – this was a few weeks after Annabelle’s CLN3 diagnosis – he offered his full support. Time passed and each week he asked how Annabelle was doing, always offering his help. The week after we received Robbie’s diagnosis he knew that something was wrong, he came up to me after the session and asked, I said “We have had the results for Georgie and Robbie”, I didn’t have to say anymore, David just hugged me and tears filled his eyes, he said “No……….Robbie?”. From that moment on David has wanted to help, to show his support and to raise funds.

David organised a Super Hero week at Play Sportz, where all the children dressed up as super heroes and parents gave donations, he shared our JustGiving page and contacted the local newspaper who have since shared our story. David is also joining us on the London Marathon Walk in September, and has raised a lot in sponsorship.

Sadly, last week was our last session at Play Sportz but I think Robbie’s smile has had an lasting effect on David. I know that David’s support to us will continue for years to come. He is warm hearted, very kind and selfless man. He will always be a BFF.

THANK YOU COACH DAVID

File 02-08-2017, 12 41 18

The First Wheel chair

6 weeks ago, whilst we were away in our motor home, Annabelle had a conversation with me.

The day before we had walked along Hastings seafront, it was a windy day but dry. We parked the motor home at one end and off we went, enjoying the delight the seaside has to offer. We looked into shops and looked at the buildings, strolling along enjoying ourselves.

We walked back to the motor home for lunch. While I was preparing lunch the children were playing on the Green, but Annabelle was in the motor home slumped against the window, head on arms, feeling absolutely tired.

Annabelle said “Mummy, yesterday if I had walked one way and you pushed me back, I could have been playing with the others today. I’m fed up with my body aching all the time, fed up with feeling tired and I’m very fed up with not being able to join in the fun”. I was stunned! My Annabelle admitting defeat. She asked for a wheelchair – a very mature decision to make.

This is something that I thought would not happen for a long time, but today we went to be assessed to see if we are allowed a wheelchair or not. To my surprise we came away with one.

Annabelle tested four wheelchairs and she chose the one she wanted. It was her chair after all. She had to feel comfortable and she had to accept it. She chose an Action Junior, in red with flashing front castors and with hand brakes.

Annabelle loves it, and can’t wait until winter so people can see the flashing wheels in the dark.

IMG_0059

 

Silverstone June 2017

Please excuse my vagueness in this blog with regard to motorsport, Andy and the boys are the real fans, myself and the girls just enjoy it.

Robbie spends most of his time racing toy cars around the carpet and running around the garden making car noises, just like his big brother Aiden did at 4years old. The boys love fast cars so you can imagine their delight when we received an invite from Martin Plowman of UltraTek racing to come to the Silverstone 500 meeting.

Martin had heard our story through a lovely lady called Emma, who’s niece goes to the same school as our children. Emma told me that Martin wanted to help raise awareness and he did a video on his facebook page on Batten Disease Awareness day, the 9th june.

The invitation was for us to visit the teams garage, meet Martin and the other drivers, to see the car and then to enjoy the race from the garage. Wow, of course we accepted, the boys jumped with joy and Annabelle was very pleased that she could actually experience a race.

We arrived at Silverstone on Sunday, first port of call was to admire the super cars, they were all lined up looking perfect with their paintwork glistening in the sun. Robbie was amazed, got a little to excited  and was then told off for touching a Lamborghini, oops.

Ben from UltraTek met us with a box of goodies, there was a Team tshirt for each child, and a set of ear defenders. I was a little disappointed that I didn’t get a Tshirt, but the kids looked better in them than I would have so it was ok.

Silverstone is a lovely track, it is the heart of British racing after all. Great circuit for spectating either in the grandstands or the grass banks. The weather was great so we enjoyed a bit of racing.

We went to the garage and met Martin, he greeted us with a huge smile and a hug, that hug said a lot about the man, it was a tight hug, a hug that showed he cared. He was very polite, he lead Annabelle around the car, allowing her to touch it and then he let the children sit in the car. Annabelle said afterwards how much she enjoyed this experience because she has never seen inside a racing car before and has no memory of one and being allowed to touch the steering wheel with all the buttons gave her a good understanding of what one looks like.

Martin introduced us to his parents, who attend every race and we met his beautiful wife, Nicole. Everybody made us feel welcome.

We were introduced to the other drivers that were present at the time, Kelvin Fletcher and Tim Eakin. Kelvin came across quite quiet, but made pleasant conversation and Tim was a hit with the kids. Martin drives car number 53 and Kelvin and Tim drive in car 54, after a bit of banter between the children and the drivers about which car to support we left the garage for lunch.

At lunch Robbie recognised the Ginetta cars from Thruxton. At Thruxton The boys met a young racing driver called Ollie Wilkinson. We could not find Ollie at that point so we arranged to try and meet him later.

We watched the start of the British GT and sat at Copse corner. Annabelle loved the it, she could smell and hear the cars different engine tones.

We went back to the garage to experienced the garage during the race. Kelvin did an amazing job on his sprint in car 54. Martin Plowman took over car 53 and things did not go well, The car had problems and needed repair in the garage and we were asked to leave as it was about to get hectic.

Ideal time for us to head to the Ginetta cars to meet Ollie. Robbie straight away recognised him and greeted him with a cheeky smile. I did not go to Thruxton so it was a pleasure to meet the young man that Robbie has been imitating with his cars for the pass few weeks. I think Ollie has his first number one fan.

We watched the rest of the British GT race from the grandstands and soaked up the atmosphere.

Afterwards we went back to the garage to say thank you and goodbye. Martin gave us another hug, and thanked us for coming. Tim Eakin made Aidens day by giving him his race worn cap, complete with the sweat and smell, Tim said “wash it mum”, there is no way Aiden will let me wash it.

Martin helped us to raise awareness and he made precious memories with our family, and we will be forever grateful and hopefully we made some more BFF’s that day.

 

 

Extreme Tiredness

Why is my daughter asleep at 11am in the morning when she should be at school?

Its not fair, she is 11years old and should be full of energy and full of life but Batten Disease is taking it away from her. Annabelle slept for almost three hours, during that time I checked on her every five minutes, I was full of worry, I even cried at times.

Annabelle does not sleep that well at all, she can have days of which she only has a couple of hours sleep a night and times where she wont sleep at all. Annabelles behaviour reflects this, she has violent outbursts, anxiety attacks and struggles to concentrate.

Today was a day when her body said “enough is enough” and it wanted to sleep.

This is a symptom of Batten Disease, a disease that we are fighting together and we will carry on fighting.

IMG_0046

Batten Disease Awareness Day 2017 – BFF Disco

Friday the 9th June 2017, Batten disease awareness day.

I turned on my phone that morning to see that 132 of my facebook friends had added the awareness frame to their profile picture, it was fantastic to see the support.

The BFF disco which was organised by two very special friends, Rachel and Amanda, and with support from the lovely Sara who let us use the Girl Guiding Hall in Ringwood and with the dj’ing skills of Chris and Simon we had a successful night. The children loved it, we had a superheroes theme for the younger ones and decorated the hall with capes made by Robbie and his friends at pre school and the older children chilled to the music either inside the hall or outside.

Annabelle did a small speech thanking every body then we both danced to Annabelles favourite song, I love my life. I cried whilst dancing with her because we have had years of her saying she hates her life and now she loves it.

We raised £835 at the disco!!!!! Amazing.

We would like to thank everybody who has given us support, it means a great deal to us. We feel your love and we are receiving your prayers.

 

IMG_0038