“Pretty good odds” to prevent the one armed man.

Hi! I hope everyone is well.

My plea for 2023 to sort it’s shit out hasn’t really gone to plan so far, albeit things are beginning to look a bit brighter as we leave February behind (or F***y February as I have affectionately coined it this year).

Just after our last update we received the devastating news that my wonderful Mother-In-Law had been diagnosed with a brain tumour. To say it hit us all like a ton of bricks is the understatement of the century. However, our amazing NHS swooped in (as they frequently have to do in our family) to save the day, and within a fortnight she was out for the count in a 6 hour surgery to remove the unwelcome golf ball which had been growing steadily at the back of her head. It was a whirlwind few weeks, but she is one tough lady! Discharged from hoskipal (‘hospital’ in Squidge Speak) 2 DAYS after surgery, attempted to put up a fight about going in a wheelchair down to the car (I won that one, I am a formidable force when tested) and has been on the rapid road to recovery ever since! Now said golf ball has been removed, lovely M-I-L now acknowledges that she may have been able to feel it all along, but just put it down to her head being a strange shape. Moral to that story: If your head feels knobbly, get it checked.

In all seriousness though, she truly is an incredibly lady who has been feeling unwell for far too long. The tumour has (thankfully!) been found to be benign and she’s now quickstepping around the house, and building her strength back up. Mags, you rock.

In T news, I have reports both medically and domestically.

You will need to ensure you are sitting down for this first bombshell:

T, can now load and work the dishwasher.

See photographic evidence.

Basically, I am on a bit of crusade to make T independent. Or, as independent as possible. More often than not, we all run around after him, which isn’t a bad thing. When you see how hard it is for him to move from his bed to his chair, it is easier (and often quicker) to just do things for him, like move dishes from his room, take him food/drink etc. However, in doing so we are going to limit him in the long run and make him solely dependent on others. So, I am A WOMAN ON A MISSION. One new domestic task whenever possible. This one was a no brainer as it was starting to look like a dirty Ikea in his bedroom (used cups and plates, to be clear. Not the other kind of dirty. Eww.) As you can see from the pictures, he is THRILLED with my new found obsession of interrupting his moody teenage TikTok life. J is 100% the cooler parent at the moment; he talks football and Playstation with T – I make him load the dishwasher and use mouthwash.

In medical news, we have also had some movement there too. We had an appointment with Dr Mordeca, T’s Ataxia specialist. I really really like this man. I always feel like we’ve had a positive experience when we see him. In our most recent visit he was telling us about a new drug which is currently going through FDA approval in the US. Trials have shown that it slows the deterioration of Friedreichs Ataxia, which is BLOODY AMAZING!! This is the first drug of its kind. Hopefully it will come to the UK towards the end of this year. As it stands, it is only for use in people aged 16+, however wonderful Dr Mordeca is pushing for a 12+ trial. SIGN. US. UP.

In’t Science brilliant.

We have also had another appointment with T’s spinal surgeon in Sheffield. We knew T’s Scoliosis had deteriorated. However, we weren’t quite prepared to see this:

The image on the right shows T’s spine in February 2022, the image on the left shows his spine now. His curvature is now over 100, and the Dr feels surgery is the best option for him. As T’s muscles are weakening, if his spine is not straightened, he will effectively become a ‘one armed man’, as he will continually have to use his left arm to stop himself from falling over/out of his chair. T’s stomach is definitely compressed, hence the lack of appetite, but thankfully his lungs and heart seem to be OK at present. That would, however, change if we leave his spine untreated.

The operation itself will last most of the day, with T going down at approximately 8.30am and returning at around 4.30pm. As he is young and his spine is still reasonably flexible, the Dr is confident that the ‘straightening’ aspect of the surgery will happen more or less of its own accord, and then it will be a case of fixing it in place. All sounds relatively straight forward! However. And sadly, it is a big ‘however’. When this surgery is performed, something called ‘Spinal Tracing’ is used, which is basically electrical currents which run through the spinal cord throughout the surgery. If there are any issues with the cord, the tracing picks this up and alerts the surgical team who then make adjustments to try to prevent any damage. Due to T’s Friedreichs Ataxia, his nerve conduction does not work correctly, meaning that it is an almost certainty that the spinal tracing will not work. The Dr was incredibly honest (one of the reasons we like him so much), and stated that if this were an able bodied person, and the tracing did not work, he would refuse to perform the surgery due to the increased level of risk. He described it as ‘operating blind’. However, he has performed this surgery successfully on a number of FA patients.

The odds of a serious complication in a surgery such as this are approximately 1-2%, but the ‘serious complication’ in question could be paralysis from the waist down and loss of bowel, bladder and sexual function.

It took us all a minute to absorb this.

And then T spoke up…

“They seem like pretty good odds to me”

He may disturb my watching of Greys Anatomy by shouting profanities at his Playstation, but man alive, this kid never fails to amaze me.

It certainly made the Dr smile too.

Dr B has made it very clear that the first sign they will have that anything is wrong, will be when T is woken up post surgery, and is asked to move his legs/feet. If he can’t, they will send him for an immediate MRI and will endeavour to go back into surgery, but by that point it may be too late.

He has also, however, made it clear that if it is successful, this will greatly improve T’s quality of life. And prevent him from becoming a one armed man.

Oh, just to complicate things further (because you know, things are never simple with the Riley’s), T will have to go straight to the High Dependency Unit following his surgery, as there could be complications with his breathing. As a result, the surgery cannot be performed between October-April, as the HDU will be full of respiratory patients. T is now on the fast track waiting list, so his operation could be around September of this year, or else it will be April/May time next year. We’ve signed up for cancellation appointments too, so now it’s just a waiting game.

Apologies if you have asked me about the above appointment and I haven’t disclosed all of these details, I find them quite challenging to say out loud. However, that is the beauty of our blog I suppose! And thank you to everyone who has checked in with us, it never goes unnoticed.

So that’s where we are. The good, the knobbly and the wonky. But, we have pretty good odds, so that’s worth smiling about.

I shall leave you with a few pics of some recent family excursions.

Take care,

L and The Riley’s

xxx