It’s been a while since we’ve added any blogs to the website, mainly because of the hustle and bustle of life but particularly due to changes and challenges with the health of Annabelle and Robbie. I’ve decided to provide this update because we have experienced a significant deterioration of Annabelle’s condition and want to share this to advise and help others.

Sleepy modes, anxiety and depression

Since the end of the summer holidays Annabelle has experienced a few of her sleepy periods. These are times when she seems to want to do nothing but sleep – which we often compare this to a car going into “limp home” mode – where she will be very quiet, often non-responsive, and needs encouragement to eat, drink and do the basics. These have lasted between a few days and several weeks. This resulted in missing some school in the first half of the Autumn term. When she was at school she was increasingly experiencing frustration and anxiety.

Annabelle is in the last year of secondary school (year 11), a time when students are making final preparations for exams and planning their post 16 journey – sixth form, college, apprenticeships. A year ago we knew that Annabelle would likely struggle to sit her GCSE exams and that we would be looking at specialist education setting for post 16. We started the processes to seek adjustments for her exams. We visited a specialist school in January which Annabelle very much liked and she decided is where she wants to go from year 12 and ideally start 1 day a week in year 11 to access some of the support and subjects that her current school cannot offer. The process was started with the Specialist Educational Needs team at the council, which is always subject to budgets, panel approvals, etc. When school restarted in September, Annabelle had received no answers about where she was going to be post 16 and was experiencing the almost constant discussions at school about exam preparations, mock exams, tests and assessments – she didn’t know whether she could sit exams or be assessed through other methods and did not know if she could go to the specialist school that she had chosen – this all created a tremendous amount of anxiety.

Anxiety, along with tiredness, triggers a deterioration in Annabelle’s health and well-being. She experiences rapid mood changes and depression. We are fortunately to have support from Dorset’s ID CAMHS unit which has helped with coping methods and ways to communicate feelings. Earlier this year they prescribed medication (Diazepam) for Annabelle, to be used in situations where she was experiencing a high level of anxiety where coping techniques and anything we say or do simply doesn’t help. This medicine rapidly calms and suppresses the anxiety, often making Annabelle fall asleep.

Delirious and psychotic

In October we had an enjoyable break in our motorhome on the Devon coast over the half term holiday week. Annabelle was a bit sleepy but otherwise enjoyed time on the campsite, a few visits to places nearby and swimming with Robbie and Georgie. After the half term she went back to school but was sent home as she was falling asleep in class. She remained at home for the next few weeks suffering her sleepy episodes. She was much better in the third week of November and managed three straight days at school without problem, just before our first overnight visit to Naomi’s House and Jack’s Place.

Due to COVID restrictions we have had a patient wait since initially contacting Naomi’s House in 2019 to be able to make our first visit. The first visit always consists of a family stay over in order that care plans can be established and parents are to hand in case a child does not settle. Both Robbie and Annabelle had their own rooms, with staff on hand and monitoring to ensure their well-being at all times. There were plenty of activities and play facilities. Georgie and us parents stayed in separate accommodation and enjoyed the respite. Annabelle enjoyed chatting with the staff about everything she loves and she enjoyed making Christmas decorations and other crafts. She shared that she enjoyed being at Naomi’s House and Jack’s Place because she knew that she would be supported if something happened to her while she is staying there. We headed home on the Sunday morning and Annabelle was very upbeat, was very chatty and talked a lot about the meal she was going to make at school the next day.

Annabelle’s chattiness turned into constant talking, sometimes with us and often with imaginary friends. This went on for 48 hours before she finally crashed and fell asleep. When she awoke, she was again in a different world to the rest of us. Talking to other people. Sometimes she was very happy speaking to members of her favourite pop group (The Vamps), singing and dancing but sometimes she was very scared. The ID CAMHS doctor made a home visit on the Thursday and witnessed this. He was as concerned about this as we were and immediately organised blood tests to rule out infections and prescribed Lorazapam to Annabelle to help her settle.

The following week things worsened and Annabelle became much moodier, scared and violent. The medical support has now prescribed anti-psychotic medication (Olanzapine) to help control the mood changes and sedate her.

Many times in the last weeks I’ve thought (hoped) that this is just a mode that Annabelle will snap out of at any moment, similar to her sleepy periods that have lasted a few days or weeks and then back to normal. I’ve looked for reasons, for what has triggered this, for things to blame ….. the lack of progress and answers about schooling & exams and the unnecessary stress that has caused ….. how safe Annabelle felt at Naomi’s house and has this allowed her to stop fighting knowing she will be looked after

It’s now clear that this is the next phase of Annabelle’s fight with Battens. The new normal that we will need to manage.

I can try to imagine, but only Annabelle knows what it feels like to be experiencing what is happening in her head and the effects of the cocktail of medicines she now takes.

What’s next

The approval for the specialist school has just come through and Annabelle can start 1 day a week from mid January. I suspect the transition from mainstream to the specialist setting will now need to speed up.

Our priority is to help Annabelle to be as happy as possible and give her the best possible care.

Lisa

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.

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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.

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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

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

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.

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

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!

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.