Feeding the force

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Quinns has finally made it back to school. He’ll only be in for short periods of time a few days a week building up gradually as the weeks go on. His class were absolutely delighted to see him back and even now he’s akin to a celebrity as they scream and shout his name every time they see him. 

Quinns is smiling wearing his school uniform with white collar and red sweatshirt. He has a white tube coming out from his nose which is stuck onto his cheek with a piece of tape with colourful planets on it.

Initially the big question they asked was about the tube stuck to his face. One big change from Quinns being unwell and the most noticeable is the nasogastric (NG) tube that goes into his nose and down to his tummy. After various issues with his tummy and lungs he’s currently nil by mouth so he gets all food and medications down his tube.

Community nursing staff are supporting the school in this new method of feeding. They have been in and had a chat with the children in his class to explain it all, answered numerous questions and heard random tube related stories from the children.

Four times a day the NG tube is connected to a feed pump (affectionately known as ‘Darth Feeder’) which delivers a carefully calculated quantity of special feed. It’s a very efficient way for him to receive nutrition and he’s looking really healthy for it. Mealtimes are no longer long and drawn out as it would take him a while to chew and swallow the quantity he needed for sufficient calories.

The majority of the time it works fine. The biggest problem is when he manages to pull the tube out on a Saturday evening. We need to take him on a 30 minute drive to our local Children’s Ward for a replacement. All going well it’s a straightforward process but sometimes there are difficulties that result in an x-ray to check the position. 

At home, we miss the social aspects of eating. Before Quinns went into hospital we would always sit round the dining table as a family. Eating was a huge part of our lives. Quinns would have his eye gaze computer in front of him and we would chat while we ate. He picked out words on his device to tell us he was following our conversation or demand pudding or tv when he had had enough. 

We would often go out to eat on nights I couldn’t be bothered cooking or out for breakfast as a treat on a weekend morning. It doesn’t seem fair to do either now that Quinns can’t join us in eating. We have realised how often we mention food in casual conversations.

As well as being less visible in the community he also misses out on a big part of his social life. Often when we went out we found him making friends with people at other tables while we were eating. They would make themselves known to us at the end of a meal telling us he had been smiling at them.

We’re hopeful that as he gets stronger he might get back to eating orally because he previously loved food. Especially cake and custard! While we wait and if he doesn’t we will find ways to adapt to the new eating situation and find ways to ensure his place in the community. For now we are embracing the brilliantly patterned tube tapes we’ve found on Etsy. Everyone is excited to see what he’s got on each day whether rockets, aliens or helicopters!

Routine brain surgery

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When Quinns ended up in hospital last year caught up in a dystonic (choreic) storm nothing was working to break him out of it. We had exhausted all our strategies at home and everything the doctors tried also failed. He ended up heavily sedated to control the movements and on a ventilator to keep him breathing. He had no quality of life. The only option left to try was described as ‘routine brain surgery’.

Photo of Quinns smiling. On his t-shirt down the left sleeve reads the word 'Recharge'.

After being flown to the Evelina hospital in London he was fully assessed, and with his new diagnosis of GNA01, was deemed an appropriate candidate for a Deep Brain Stimulator (DBS). Although far from routine for children, DBS has been used for adults with movement disorders like Parkinson’s for a number of years. 

Usually candidates spend about a year preparing for the device to be fitted. They undergo assessments to monitor involuntary movements so that changes can be tracked after the DBS is fitted. For children the surgery is carried out under general anaesthetic. A week or so after surgery the DBS device is turned on. Changes to the electrical pulses are then made slowly with frequent trips to hospital.

In Quinns’ situation speed was of the essence. There was no time for any preparation. Two small electrodes were implanted into his brain sending electrical impulses to the part of his brain that controls his movements. The device was turned on immediately with the simple aim to break the dystonic crisis and get him out of intensive care. 

Thankfully it worked. His movements lessened allowing the levels of sedation to come down and finally he made it off the ventilator. But what does having a Deep Brain Stimulator mean for him now?

To keep the device working well we need to remember to charge it every day. The electrodes are connected to and powered by a small battery pack which is implanted in his tummy. We hold a charging device to the battery pack in his tummy for about 15 minutes. It is a straightforward process that has quickly become part of his evening routine.

Like someone with a pacemaker he will need to alert airport security to the presence of his device when he travels and he will need to have any MRI scans in a designated DBS centre. He should also avoid activities that risk dislodging the probes or damaging the wires.

He will need to make regular trips to the Evelina in London for check ups and for any adjustments to be made to the device. The DBS should last about 25 years and will hopefully help prevent any future storms.

While he has been recovering, as well as his body being a bit stiller, we have also seen slight differences in his hand function compared to before the surgery. Although the initial aim was to rescue him from the dystonic crisis it is hoped that further improvements can be made to his movements by tweaking the electrical pulses. 

This far from routine brain surgery has not only saved Quinns’ life but it may also improve his quality of life.

Home at last

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After five ambulance rides, two air ambulance flights and four admissions across three hospitals (Forth Valley Royal Hospital, RHCYP Edinburgh, the Evelina London and then Edinburgh again) Quinns finally rang the bell to signal his discharge from hospital 171 days after admission.

Quinns in his wheelchair reaches up to help pull the cord his mum is holding to ring the bell to signal the end of his long stay in hospital.

Unfortunately he was only home for three nights before being admitted to Forth Valley Hospital for another six nights.

Thankfully, a full six months after his initial admission, Quinns is now home again where hopefully he will stay and fully recover from his adventures.

Big Sister and Quinns smile directly at the camera.

Thanks again to everyone who helped and supported us along the way.

Flight GNA01

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Today is Rare Disease Awareness day which seems like an appropriate time to share a bit more about the journey we’ve been on with Quinns these last five months. 

Big Sister and Quinns smile together looking up towards a tv above the hospital bed Quinns is sitting atop in his comfy chair.

In September last year Quinns ended up in hospital experiencing a dystonic crisis. Dystonia or involuntary movements are common in Cerebral Palsy. He underwent a whole raft of tests in an attempt to establish the trigger for the crisis. The only way to control the movements, which were by this point causing him physical harm, was to heavily sedate him. With so many sedation drugs on board Quinns eventually needed help to breathe and was put on a ventilator. 

The initial cause of the crisis was never established but in the process we discovered that his initial diagnosis was wrong. Rather than Cerebral Palsy, one of the most common childhood disabilities, Quinns actually has a very rare genetic neurodevelopmental condition called GNA01. 

GNA01 was only discovered in 2013 and as such there are currently only about two to three hundred people with this particular genetic mutation in the world. It presents in one of two ways either as a movement disorder which looks very like cerebral palsy or as seizures. Sometimes people have both but in Quinns’ case it is simply the movement disorder. 

It is obviously a shock to have an entirely new diagnosis after seven years. There’s a lot for us to get our heads around but what does it really change?

Cerebral palsy and the movement disorder caused by GNA01 are very similar. They both result in a disability requiring Quinns to use a wheelchair to get around and an eye gaze computer to communicate. He would have needed all the same equipment and support regardless of the name of the condition. The only thing that really changes is the narrative around his birth. His condition isn’t caused by brain damage but by a change in his genetic coding. There was absolutely nothing we or anyone else could have done when he was born to make his condition any different. 

As in Quinns’ case the condition usually presents when a child has an extreme episode of dystonia or hyperkinetic movement which spirals out of control and leads to a long period in intensive care. This is known as a ‘Storm’. If we had known earlier that Quinns had GNA01 and that such a Storm was possible perhaps we could have been more prepared? But on reflection there is very little that I would have done differently for him in the years leading up to the Storm. His life was fun, social and fulfilling and we did everything possible to ensure that for his future.

Although cerebral palsy was the best explanation we were given for Quinns’ difficulties it never fully explained his condition. We now have a condition and a list of symptoms that describe Quinns’ difficulties with movement more exactly. Unfortunately we can’t rule out the possibility of future Storms but hopefully we will be better equipped to avoid them or at the very least deal with them when they arise. Our decisions moving forward can be based on knowing more about the condition we’re dealing with and hopefully more and more research will be carried out on this relatively new discovery. 

Although we’ve yet to connect with them, Quinns is now part of a new small community of people with GNA01. There is a tribe of people who present almost exactly the same as him. One thing that struck me when I watched a video about some of the other children with GNA01 was how often their smiles are mentioned. 

Quinns has been through a lot these last few months but he’s come out the other side the same smiley happy boy we all know and love. He attracts the attention of many passers by as he sits in his chair atop his hospital bed giggling away while he watches Thunderbirds for the 100th time. Big Sister is of course still his most favourite person.

I set up this blog a short while after Quinns was originally diagnosed with CP. The aim was to share our frustrations and struggles that we faced as a family simply because Quinns was a wheelchair user. Those frustrations and struggles remain the same. Accessibility and societal barriers are still a big problem for him. We still need access to Changing Places toilets to allow him to get out and about and accessible play parks are even more necessary now as he so deserves some fun after all he’s been through. 

Hopefully once he gets out of hospital we’ll get back to sharing our life experiences again. In the meantime we want to raise awareness of GNA01 for the two hundred or so people living with the condition as part of the 300 million people living in the Rare Disease Community. 

You can find out more about GNA01 from the Bow Foundation website and watch the video Who is GNA01?  

Thank you

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Over the last few months we have experienced some of the very best of the NHS. While sadly other family members have not due to increasing pressure and broken systems.

I am looking at the camera while Quinns cuddles into my shoulder. He has an oxygen mask near his mouth, a blanket round his shoulders, shaved head and very long eye lashes.

Quinns has been very unwell since the end of September. I don’t think my brain has processed quite how unwell yet. Thankfully his care has been great and he is on his way to being home. I’m not quite ready to share the many complexities of what has been happening but hopefully as he continues to recover and rehabilitate my ability to articulate will return.

He has been helped and is continuing to be cared for by so many people it’s difficult to name them all but here goes.

This New Year we are very grateful to the doctors, nurses, physiotherapists, dieticians, speech & language therapists, occupational therapists, psychologists, radiographers, surgeons, anaesthetists, pharmacists, phlebotomists, play specialists, hospital teachers, cleaners, caterers, administrators, specialists, porters, blanket gifters, volunteers and entertainers at Forth Valley Royal Hospital in Larbert, the Royal Hospital for Children and Young People in Edinburgh and the Evelina Hospital in London.

Special thanks to the ScotSTAR retrieval team, paramedics and air ambulance paramedics who got us safely to where Quinns needed to be. I have some amazing stories to share of our journeys!

Also as a family we couldn’t have managed without Kindred, Ronald McDonald House, the Edinburgh Hospital Children’s Charity, the Evelina Hospital School, Beads of Courage and of course all the support, love and kindness from our family and friends.

And finally thanks to the listeners, the pyjama enthusiasts, the ones who were always there, the task givers, the shoulders to cry on, the banner maker, the card makers, the hamper givers, the jokers, the organisers, the sorters, the providers, the bead supplier, the chair deliverers, the video makers, the new friends, the creatives, the tea makers, the custom pillow makers, the custom support makers, the messagers, the massager, the visitors, the cake sender, the flower senders, the chocolate and sweet suppliers, the dinner providers, the Disney enthusiasts and the music providers.

Thank you.

Spontaneous combustion

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We were lucky enough to manage a week away during the summer holidays. The beautiful Schoolhouse at Fenwick in Northumberland, just a short car journey away from home, provided a brilliant base for our adventures with absolutely everything we needed. I planned our days based on the experiences of a couple of friends so we could relax and enjoy some time away.

Quinns smiles his biggest smile with a large aeroplane behind him on tarmac.

Although I am a bit of a planner anyway it does get a little frustrating not to be able to be a bit more spontaneous. Earlier in the holiday I discovered when trying to book tickets for an exhibition on a Saturday evening for the Sunday that I couldn’t get a carers ticket because they were only available to those phoning during working hours Monday to Friday. Another unnecessary obstruction on top of everything else for disabled people who need support.

Anyway when we were driving to our holiday I spotted a sign for the National Museum of Flight. A few people had mentioned it to me because of Quinns’ love of planes but I hadn’t done my research nor did I intend to while on holiday. The planning extended to let’s just go on the way home and see what we find. I booked the tickets including a carers ticket online the night before.

As you can imagine for a display that includes aeroplane hangars the site is large. There are disabled parking spaces at each of the buildings and wheelchairs for use by anyone who needs one. As well as large thankfully the site is also very flat and the pathways tarmac which made it easy to get from one building to another so once we were parked up we were ready to explore.

The grounds were full of planes for us to discover. Quinns was immediately very excited to see so many up close. Although we took him on a few flights when he was younger we haven’t quite managed to bring ourselves to again now he’s bigger.

Disappointment came before we even made it to the suggested start of the exhibitions. We couldn’t help but have a closer look at the first plane on route only to realise we could board to see the inside. I stayed with Quinns on the ground while everyone else disappeared up the steps. To be honest I’m not sure he was too bothered about the inside but it wasn’t a good feeling that he was excluded. 

At the desk we were given times when each of the planes would be open to explore. Of course I couldn’t help but ask if any were accessible to wheelchair users. A very confused man simply told me no. I was disappointed but not surprised given the difficulties wheelchair users have with flying as described in this recent Guardian article. 

Rather than miss out exploring the inside of Concorde with Dad and Big Sister I decided to take copious numbers of photographs to share with Quinns afterwards. Again he was probably more interested in the large screens showing videos of the red arrows in action or Concorde in flight but I couldn’t help wondering why there isn’t more effort to make at least one interior available for wheelchair users who might be interested?

After lunch we went to explore the Fantastic Flight hangar. I was seriously impressed. There were loads of interactive exhibits for children and adults to explore and learn about flight. Quinns was absolutely fascinated by the balloon that filled with air and rose up. Even better, it was operated by a button that he could reach and press himself! He also spent quite a lot of time trying out the controls for operating different parts of the plane. Although he had a good go at all the different levers his favourite was the one that switched the lights on. For someone who often has to sit passively at such places this was amazing!

Quinns pushes a button to make a hot air balloon rise while Big Sister looks on.
Quinns operates four stick controls which lead up to a metal demonstration plane while Big Sister looks on.
Quinns handles the stick controller of a flight simulator.

He was also able to have a good go at the flight simulator but given that his favourite activity was letting the plane crash I think I might leave it a while before he starts flying lessons! 

So our little bit of spontaneity paid off on this occasion. The museum redeemed itself from the immediate disappointment of inaccessibility with interactive installations that Quinns could actually enjoy. However there are no excuses on a site that large not to have a Changing Places toilet which would have made our day even better! 

A week away

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We had a brilliant few days away thanks to the beautiful and very accessible Schoolhouse Fenwick. It had everything we needed as a base for our adventures. 😀

Fabulous friends gave us recommendations for what to do in Northumberland based on their own experiences so we had lots of fun without the fuss of inaccessibility!

First up was the Heatherslaw light railway which went from Heatherslaw to Etal where we explored the castle. The train had a couple of wheelchair accessible carriages and it was great to see them both being used. The castle, which is mainly ruin, wasn’t too bad for getting Quinns around in his wheelchair.

Quinns loved the train ride of course, although he learned quickly to cover his ears when the whistle blew!

Quinns is always asking to go swimming so when we heard Berwick Leisure pool had recently been refurbished we knew we had to try it out. Sure enough it has a brilliant Changing Places toilet with access from reception straight through to pool side so no need to drip through public areas while wet! 😀

It also has a Pool Pod Lift (https://tinyurl.com/4yk6x495 ) for accessing the pool. Although the submersible wheelchair didn’t have quite the right support we made it work (he was small enough for his head to be supported by the back of the chair.)

We had to rescue him on the way in as the chair kept going down until he was under the water so he sat on my knee on the way back up for safety.

Beach wheelchairs are a brilliant idea for inclusion!! It’s really difficult to move a wheelchair across sand so it’s great to see more and more beaches with these specifically designed wheelchairs.

We had a fabulous albeit very windy day on Beadnell Beach where Quinns could easily access the whole beach and sea where he was close to Big Sister who thinks she’s a fish! 😆

Our holiday was complete with a sunset boat trip around the Farne Islands. It was amazing getting so close to the wildlife particularly the seals.

Quinns absolutely loved being on the boat! We phoned ahead to check the best time as it needed to be high tide for level access.

Thanks to Serenity Boats for being so helpful, friendly and knowledgeable, it made a wonderful last night for us. 😀

No holiday story of ours is complete without a toilet tale. We managed the week despite a real lack of Changing Places toilets by careful planning and a camp bed in the back of the transit van we took to transport all Quinns’ equipment!

It was brilliant to find a Changing Places toilet in Tesco in Berwick. And one so impressively decorated! Tesco seem to be leading the way on providing these toilets but it would be great if other supermarkets followed suit. 🤞🏻

A boring trip to the zoo

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Quinns asked to go to the zoo to see the giraffes. Not a big ask for his seventh birthday but there were two big problems. As far as I was aware there is no Changing Places toilet at the zoo. Generally we like to go places where we are certain they have the facilities we need which is absolutely fine as a strategy. But if we don’t ever go to places that don’t have them there’s never any pressure to install them. So we decided to take the risk and make sure to ask about the facilities raising a little awareness while we were at it.

The second issue is our car is no longer good for travelling any distance. We’re currently awaiting delivery of our WAV (wheelchair accessible vehicle). Although we ordered it a couple of months ago we will be lucky if it arrives before the end of the year.

After researching public transport we planned our day to take the Citylink bus. Before paying for our four seats I was asked if we needed a wheelchair space. We did and I booked it for both directions at no extra cost. Simple.

So far so good but I will admit I was intrigued about how they would get Quinns’ wheelchair on a coach! I was also extremely nervous about whether the day would run smoothly or not because there were so many variables and given our past experiences it seemed like a fairly ambitious plan.

When the big day arrived, I told the bus driver I had booked the wheelchair space. We had to wait till everyone else boarded because she ‘would need to do all the shenanigans with the steps’. The shenanigans turned out to be a quick button press which turned the steps into a lift to take Quinns and his wheelchair into the coach where they were secured in the front seat.

Quinns being lowered to the ground in his wheelchair on a lift attached to a coach.

He had the best view out of the front of the bus the whole way to the zoo where we repeated the process of getting him off to an amazed group of onlookers.

Quinns smiling as he sits in his wheelchair at the front of the coach

On arrival at the zoo the first thing to do was find out about the facilities. After my usual conversation about how accessible toilets are not sufficient for our needs I found out there is a Changing Place toilet in the planning just not opened yet. We were offered the use of the medical room and given the number to call for access.

We had been before so were well aware of the giant hill the zoo is built on. I was impressed with the accessibility map we were given which pointed out the steps and showed us where the steepest slopes were allowing us to plan our route. We had the option to use the accessibility vehicle to get to the top of the hill but in our wisdom we chose to make our own way there. Something I think we regretted a little when it drove past us as we struggled up a steep incline.

However between the three of us we made it. We even managed to time our visit to the giraffes when they had been brought inside to eat so we had a perfect view. Some of the enclosures on the way up made it really difficult for Quinns to see but there were also plenty of lower or glass viewing points for him so he didn’t miss out on too much.

Quinns & Big Sister have a chat while looking through glass screens at the penguins in their enclosure

Once we had seen all the animals we had a quick lunch. Then all too soon, and without having a chance to try out the accessible roundabout in one of the play parks, it was time to head out to catch the coach home. Which was as uneventful as the first time.

Despite my concerns and readiness for some sort of fight or difficulty the day was pretty peaceful. Thankfully our risk with going somewhere without a Changing Place Toilet paid off on this occasion but it would have been so much easier to know it was available for us. Hopefully the plan for one will soon be a reality.

All in all the day was a success and Quinns went to bed that night a very tired but also very happy little boy.

Quinns and Big Sister pose smiling beside a #GiraffeAboutTown at the entrance to Edinburgh zoo

To boldy go…

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Close up of Quinns' tired but happy face. He holds a red curly straw up in his right hand.

With Covid restrictions and several bouts of illness this winter it’s been a while since Quinns has made it to a party. I’m tired from having recently had Covid but I’ve also been watching and reading a lot that’s made me want to speak out more about our experiences so here’s a little glimpse at the emotions that went into one birthday party at a trampoline park.

I’m grateful he’s got friends and has been been invited to the party. I’m thankful that restrictions have lifted and he’s well enough to go. I’m hopeful it’ll go well knowing I have friends ready for action.

I’m delighted at the squeals of excitement that ‘Quinns is here‘. I’m proud of Big Sister supporting her brother on the trampoline. She enjoyed the responsibility and loved chatting to all his friends. I’m relieved it’s not me up there scaring them all away!

I’m sad, angry and disappointed but accepting that I had to change him on a toilet floor. No-one should have to but it’s our reality without a Changing Place toilet. I’m exhilarated by freeing a red emergency cord and adorning it with a Euan’s Guide card.

I’m happy there’s a lift to get him to the party room. Quinns enjoyed the ride while I’m relieved we didn’t get stuck! I’m disappointed there’s not enough space or flexibility for his wheelchair to fit under the table allowing him a space at the table.

I’m happy to see his excited face when he’s given tomato ketchup with his meal. Even happier to see his full on excitement when the amazing birthday cake is brought out and we all sing happy birthday to his friend.

I love that he dipped into his party bag with another friend before proudly holding his red curly straw all the way home.

Thanks for a brilliant party!

“To boldly go where all others have gone before” #ThenBarbaraMetAlan

Thunderstruck

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Learning to understand what an emerging AAC user is trying to communicate can be challenging but recently a couple of things have clicked into place for us.

An eye gaze computer grid with symbols and words for vehicles and the words 'remote, plane, helicopter, fire engine' in the speech bar.

After watching a trailer for Back to the Outback, an animated comedy adventure set in Australia, Quinns clearly told us he wanted to watch it by going to his animals board on the eye gaze and choosing the words ‘snake’, ‘spider’ and ‘koala bear’; three of the main characters from the film. Whenever we asked what he wanted to do he would repeat the same three animals followed by ‘remote’ until we finally had time to sit down and watch it. 

Although he loved it, giggling his way through the entire movie, the next time we asked him what he wanted to do he said ‘plane’, ‘helicopter’, ‘fire engine’ followed by ‘remote’. It took us a while but we eventually worked out that he was referring to an already favourite film of his, Planes: Fire and Rescue, a spin off from the classic Pixar film Cars based on fire planes and helicopters rather than cars and trucks.

This is a brilliant step in his ability to communicate what he wants to watch without us having to programme all the films that ever existed into the eye gaze! 

Quickly following this discovery, one evening at the dinner table Quinns interrupted our conversation with an obvious request for music. Dad usually puts some on when he sits down at the table but on this occasion he hadn’t. Quinns chose ‘song’ and ‘piano’ on the eye gaze before looking expectantly at Dad. A playlist was chosen on the basis of what Quinns had just been talking about ‘rain’, ‘clouds’, ‘weather’ (there may have been a film request in there but we didn’t manage to work it out if there was!) First up was Thunderstruck by AC/DC.

With music now in the background our dinner time conversation resumed until suddenly Quinns got VERY excited! His entire body was in such motion he could barely keep still enough to choose the words he wanted to say. He managed to choose “plane, helicopter, helicopter…” before looking around us all hoping that one of us would realise what he was saying. But we all just looked at each other knowing there was something but no idea what so he repeated ‘helicopter’ a couple more times before changing board to say ‘song’.

It still took us a while but suddenly it clicked and we realised the AC/DC song that was playing had been used in Quinns’ favourite film, Planes: Fire and Rescue or as he likes to call it ‘plane, helicopter, fire engine’.

So now we know Quinns can identify films and their songs and tell us about it just as long as we can guess from his clues. Anyone for a game of AAC charades?!