It’s the little things…..(and the big things)

By Helen Simmonds

2018 was a big year for us in terms of Rett and all that entails.  A huge year in fact.  We had to make decisions that no parent wants to or indeed (importantly I think!) feels medically knowledgeable enough or equipped to make and plan for scenarios that frankly scared the life out of us.  And as a result of those decisions we watched our then 10, going on 11 year old daughter, Lauren endure three significant surgeries in the space of 11 months.  Three very separate operations, but all very much interconnected.  

In September 2017 we heard those dreaded words from our spinal consultant that despite efforts to slow progress of her Scoliosis, Lauren was now in much need of surgery.  She has two curves, much like an ‘s’ shape for a spine and the lower curve was then measuring 78 degrees, a big jump from our previous six-monthly review. His chosen weapon of choice was Shilla Growing Rods which would be fixed in position to her pelvis to anchor and stop any pelvis rotation as a result of surgery and allow her natural growth as she was still so young, and it needed to happen quickly, ideally within 3 months.  

As any parent who attends Scoliosis clinics will know, spinal consultants can be a breed of their own and ours is right up there with lack of any social graces and empathy! Breaking this news to Lauren and I was about as matter of fact as you can get but inside I was reeling and not really hearing the words properly.  I did somehow manage to say, fighting back the tears that my biggest concern for it was Lauren’s recent, very problematic increase in reflux which in turn was causing a sharp increase in epileptic seizure activity, as a reflux ‘attack’ would trigger one. It was happening daily by then, sometimes multiple times a day. The thought of her laying flat in recovery from spinal surgery and then a big, physical tonic clonic seizure happening due to pain of reflux was too much to consider. So the spinal consultant wrote to our Gastro consultant asking him to expedite plans to sort that as a matter of urgency. After much liaising (mostly on my part) between both consultants we finally had our plan – Lauren would have Fundoplication surgery to sort the reflux, and hopefully reduce seizure risk. Then ideally six months recovery, but certainly a minimum of three before Scoliosis surgery and when healed and recovered, we’d change her existing Gastrostomy feeding tube in to her stomach to a Mic-Key Button (a small port that sits flush to the skin and goes through to her stomach which we could attach and detach a wider feeding tube to) allowing us to start Lauren on a blended diet (feeding her blended, real food as opposed to a fortified milk based diet), hopefully improving the reflux threat further and also help us increase her weight.  Weight has always been an issue for Lauren in that she struggles to ever put it on and maintain it and suffering with severe reflux and increasing pressure of scoliosis on her stomach and internal organs, it wasn’t helping the situation any.  

We had no choice but to embark on the surgery road ahead.  

So in December ‘17, a week before Christmas, Lauren had Fundoplication surgery. It was successful, though a much bigger surgery and recovery than we anticipated and were led to believe. It wasn’t a straight forward recovery either resulting in another hospital admission just a few weeks later but after 6-8 weeks and things were settling we could start to see the benefit and reduction in the awful reflux attacks. Some respite for her, finally, though felt short lived for us as no sooner was recovery happening, our appointment letter for spinal surgery pre-op dropped through the letter box. Most of our consultants are based out the John Radcliffe Hospital in Oxford, over an hours drive away for us, so with Scoliosis surgery looming we made our way up the M40 to pre-op.  

I can’t sugar coat it, pre-op was a gruelling day. We were at hospital for 8 hrs and met with our consultant, his surgical second in command, the anaesthetist (who explained explicitly the risks involved), the physio and OT connected to spinal team, nurse and doctor from spinal team, the ward manager and play support manager (to help us manage Lauren’s high anxiety levels – also a huge seizure trigger risk), then she had blood tests, photos and x rays taken. Her Scoliosis was now measuring 86 degrees.  We went home numb and mentally drained and my husband and I barely spoke to each other for a few days, a bit shell shocked. We were told the bloods taken would be valid for three months so knew surgery date itself would be imminent and were planning ahead thinking it’d be towards end of school summer term which would have helped me a lot in terms of absence from work and not missing too much school as majority of recovery in holidays. Before we knew it, the surgery letter arrived, and it was scheduled for just 2 weeks later on 3rd May.  Both my husband and I planned to be there for the surgery and her hospital stay so mad panic ensued getting things organised from work cover, pet cover, B&B close to hospital for my husband to stay in, and not to mention care for our then 9 year old son who was struggling with idea of us disappearing out of his life for a week or so. We were also working with school and SALT to help us prepare Lauren herself, to help her try to manage her anxiety a little easier, using her eyegaze to build pages to help her in her recovery, and also talking to her in an appropriate way about what was to come. Having been through a big surgery just months before and knowing (in general terms) what she had to have done this time, her reaction was heartbreaking and her response so absolute. That she was scared, she was frightened, she didn’t like it, didn’t want to.  And about Rett itself – it’s not fair.  Not, not, not , not fair.  They were her exact words on her device.  And she’s right, it’s not bloody fair.  

By the day of surgery, Lauren’s spine measured 106 degrees, and she was bent so drastically that her lowest rib and hip bones were almost touching on one side. The anguish and worry that a huge operation such as spinal surgery puts on parents/families is immense, and the 9 hours she was away from us in theatre and recovery was the longest 9 hours of our lives, and the whole thing worthy of a blog in its own right, but after 10 days in hospital we returned home and embarked on recovery.  Lauren was amazing and coped admirably with everything thrown her way.  And making up for his severe lack of empathy, charisma and charm, our consultant did do an amazing job and Lauren healed beautifully.  She went from 106 to 56 degrees and literally grew 3 inches overnight! 

Two operations down and one (minor by comparison) to go, we finally felt able to come up for air and take stock of the stresses they’d bought. That summer, my sister surprised me with concert tickets to see the Foo Fighters in London. I was ready for a night out – my first for what felt like a decade and some loud rock music on a warm summers evening, with a beer in my hand and in a stadium surrounded by adult company very much ticked that ‘escape’ box!  Whilst waiting for the Foos to come on, my sister and I sat people watching, mulling over life and chatting about all sorts.  But inevitability (as it often does somehow) conversation steered back towards events of the months leading up to that point. We talked about how tricky life can be at times and not just with the immediate things going on then but actually throughout our entire Rett life.  At that point we’d had 9 years of juggling, compromising, adjusting, accepting, not accepting, arguing, fighting, worrying, learning and being on the biggest emotional roller coaster with nowhere near enough sleep under our belts! And we spoke of how sometimes it’s the little things, not the huge great big whoppers that can either make or break your day. The little things that seem to serve as a sometimes painful reminder (in case you’d dared forget) that your life isn’t, despite your best efforts, as ‘normal’ or smooth as most other people’s. 

As an example, my sister and I, along with our siblings grew up with Radio 2 as the soundtrack behind our lives.  It was on in our kitchen at home from 5.30am when our Mum got up through to 11pm when she went to bed. Every day, every month, every year.  And I talked about how I’d love to be able to have the radio on in our kitchen as I cook, or to jolly us along in the morning and help us be out of the door on time. She looked puzzled, and asked ‘well why don’t you then?’ Put simply, because we can’t.  Just a step too far for Lauren in terms of a distraction which really, really bothers her and pushes her anxiety levels up to point she wouldn’t be able to eat or drink or breathe properly.

Another is the envy I’ve recently (and shamefully I know!) developed of others who have the luxury of just grabbing a coat, their keys and a (small) bag and simply getting in their car and driving off.  They have no idea!  We are lucky in that we have a wheelchair accessible vehicle (WAV) and it allows us freedom we would otherwise struggle to have. But, those that have them know, it’s not quick to load up and go, and can be a killer on the knees.  Or head, as in a rush you smack in to a chunk of metal bolted to the floor of car or forget your child’s wheelchair has handle bars which don’t give at all as you then sit up forgetting they’re there.  

Those kind of little things!

In October 2018, we made our way back to Oxford again for Lauren’s final surgery of that year, to change an existing PEG tube to a Button.  As I said before, certainly minor by comparison and sometimes even done as a day patient, but due to health complexities it was another admission for us and overnight stay and another General Anaesthetic, something Lauren struggles to recover from. All went as smoothly as possible however, and the benefit being that we’ve been able to introduce a blended diet to her regime which has made a world of difference for the better – also worthy of its own blog.  

A year on, we can look back on what was without doubt one of the toughest years we’ve ever had and done more ‘hospital’ than lots of people have in a lifetime.  We can breathe a sigh of relief that we’ve come out the other side…..for now (though we do know another spinal surgery is likely to be on the cards unfortunately). Rett lives are tough and sometimes they can really push you to your absolute limits, both for the person suffering and for their families. We do somehow stumble through it though and I think that is a strength most of us don’t often feel or even realise we have.