An Update on Annabelle’s Health – Batten Disease Awareness Day

Annabelle is finding life more challenging as her mental health is deteriorating. She has episodes of extreme tiredness and they are becoming more frequent. We can see a pattern of events that lead up to one. Annabelle has high levels of anxiety caused by stresses of daily life. They can push the anxiety up to a point where her body can not cope so she goes into a sleepy episode. During these episodes Annabelle will sleep a lot, her dementia symptom’s are apparent and she does not know which day it is, whether she has eaten or not, and will have no memory of events. She will have periods of uncontrollable crying brought on by fear and she will also have hallucination – these can be very scary for her and for us. These episodes can last from a couple of days to a couple of weeks and after each one she returns back to her normal self but we are very much aware that one day these episodes will be here to stay and she will not return to herself.

Her mobility is getting slower and her aches and pains are increasingly forcing her to use the wheel chair around school and visiting shops. She loves her swimming and she can swim independently, giving her the freedom she craves for.

Annabelle has remained seizure free since her first and only one back in 2019, and remains on the medication.

Annabelle remains in main stream school and has plans to sit her GCSE’s next summer but there will be options for her to get recognised for her education if she cant sit them. We reduced her curriculum last year so she will not sit as many subjects as her peers. We are considering her sixth form options and this will most likely be in a special needs school setting for the flexibility of education and accessibility around the buildings as well as access to after school clubs. 

Annabelle will not let these new challenges get in her way. She loves beading and making jewellery and she has now set up a Facebook page to sell her makes. With the support of the Amber Trust she is having weekly singing lessons which is perfect for her love of music. Annabelle is still passionate about girl guiding and she is now a Ranger, loving the badge work and socialising with the other members. We cannot forget her love for The Vamps of whom we will be seeing in September at Bournemouth, fingers crossed COVID will allow.

Update on Robbie’s Health – Batten Disease Awareness Day 2021

Robbie’s eyesight has deteriorated fast and now, at the age of 8, he has very little useful sight left. He only has peripheral vision. Robbie relies on his cane for navigation, reads braille and uses devices in the kitchen such the talking scales and drink level indicator.

We have noticed a massive change in his behaviours. He has become very hyperactive and struggles to concentrate, he is like a coiled spring. He can also have sudden aggressive outbursts that don’t seem to be triggered by anything. Robbie’s levels of anxiety are increasing and he has developed worries of things that didn’t bother him in the past. One of these is loud noise, especially in busy places, he holds my hand tighter, sometimes at school he wears ear defenders to cut out classroom noise.

In February this year Robbie had is first tonic-clonic seizure whilst at school. He was in the playground and luckily his TA was there to catch him. He was taken to hospital via ambulance as his recovery was slow. Five weeks later he had his second seizure. This time he was at home in his bedroom. It took hold whilst he was sitting on his bed and it made him fall backwards off his bed. This seizure lasted just under 5 minutes. His recovery was slow and it left him very scared, it took a while for him to recognise us. 

We made the decision to start medication to manage/ prevent seizures. As with but all medications there is a list of possible complications and with epilepsy medication, the patient can suffer with behaviour changes. With Robbie’s behaviour already at a level that we are struggling to control we asked for the choice of medication to take this into consideration.

Robbie has started taking Lamotrigine. Each week the doses increase until it reaches his final dose. On the second week of the medication Robbie had his third seizure whilst he was asleep. We were alerted by the sleep monitoring system and we were able to be by his side quickly.

Since that third seizure, Robbie has not had a tonic-clonic seizure but he has had what appears to be focal seizures. He will complain of being really hot, then cold, he will go clammy, pale and will feel very sick. These have lasted for about a minute with recovery within five to ten minutes.

Despite all these new challenge’s that Robbie is facing he does not let them get in the way of living a happy life. He continues to ride his bike in the garden and play football at home. He now has started drumming lessons with the support from The Amber Trust. He practises most days and he has a natural rhythm. He has lots of good friends who adapt their games so he can join in and now he is making new friends at Cubs.

Robbie Playing the drums

BFF Charity … the first 2 years

We learned in the first few years of our family journey just how hard it is to face the challenges of dealing with the disease and needs of the children. While there is support in the Batten Family community and a small number of charitable organisations that provide help, there are limitations and gaps.

In April 2019 we registered Batten Fighters Forever as a charity and in the first 2 years have raised around £25,000 in funds that has enabled grant awards to help families with equipment and services they can’t get support elsewhere.

Here’s a short video reflecting on the first 2 years of BFF as a charity

Learning Braille

Sight is quite often the first ability to be lost by children with Juvenile Batten Disease. Progression onto learning braille is a natural step in the journey to cope with this. The challenge this presents seems to be related to the age at which functional sight is lost.

By the time Annabelle lost her sight she had a very good reading capability. She picked up braille very quickly. To her credit she was very determined and worked hard to effectively learn a new language alongside her normal school learning.

Robbie has lost his functional sight at a much earlier age so he is learning braille in parallel to reading and spelling abilities. This appears to be a greater challenge but he is making great progress with the fantastic support of his school and VI resources.

See the videos below showing progression from Sept 2020 to March 2021

September 2020 – Robbie learning braille
March 2021 – Robbie learning braille contractions

My Garden

Blogger Belle here, as you know I have Batten disease, so most people with a disease like me get to make a wish.  I decided to make my wish of a garden just for me with things that I love to do. My nurse at Julias House was going to put my wish to Make A wish when this stupid Corona Virus struck and they are not accepting new wishes.

My dad being the expert at DIY and being at home all this time decided he would try and help me out and grant some of my wish by creating me a sensory garden, somewhere for me to chill. He has built me a raised bed with herbs because I like cooking, also he built me a swing seat that hangs from a pergola. I also asked for a water feature, wind chimes and lots of Lavender. Dad did an amazing job and I’m so happy.

I’m hoping that after this corona virus has passed and make A wish are accepting wishes again I can apply for the rest of my wish. The rest of my wish is really special to me because I want to play with my younger siblings and Robbie will also be able to use the garden after I cant. I really really want an in ground trampoline and a basket swing. I love to jump on my trampoline but I now struggle to get on it and I’m to big for our swings set, Well, we have had them for 11years!!!.

I hope you enjoy your summer, I know I will sat in my sensory garden.

My new bed

Written by Annabelle

Today my parents got me a new bed just like a hospital one.

It goes up and down, also you can lift your legs and sit up at the same time. It took a while for my parents to get home and then they had to set it up because my mum had to unscrew my old bed to clear the space for this new bed. 

Mum had some help from my siblings to carry the old planks of wood from the old bed which made the job get done faster. Then dad and Aiden carried the new bed upstairs and set it up in my bedroom. 

Mum taught me how to lift my legs up and down, I can sit up and down, she also showed me all the buttons on the remote. It was very funny when mum was showing me because I had no idea what she was doing. It was very cool and amazing.

I really like this new bed and it will also make it easier for my mum because when i’m poorly as she has to lift me up to a sitting position and get me up but this bed will do that job for her. Thanks for reading

Do you like my Vamps posters??

Robbies Wish: Meet Santa in Lapland

Thanks to Make A Wish, Robbie got to see Santa before his eyesight totally goes.

Such a wonderful adventure searching for Santa and experiencing reindeer and husky rides, tobogganing, visiting the post office, Wendy Wood’s workshop and playing with the elves along the way.

Please watch the video to get a flavour of the fantastic time we had – creating lasting memories for the family.

Thanks to everyone at Make A Wish and Canterbury Travel.

P.S. Look out for us on Channel 5 on 18th Dec!

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.