My love for you is everything More than I ever expected More than I can ever describe though still I try
My love for you is sorrow For the boy who ran everywhere and now stumbles For the boy who approached the world with open arms and a wide grin For the boy of my memories who is slipping away
My love for you is sunshine A thousand bright fragments of light dancing through my heart A glow that banishes darkness to the corners of my mind
My love for you is strength To make the impossible decisions To keep walking this tear-washed path When I have wanted to run far and fast in the other direction
My love for you is regret For all the things I have left undone For all the times I should have fought harder
My love for you is ferocious The impulse to rain never-ending kisses on your brow A mamma bear protecting her forever cub A tempest to keep a cruel world away
My love for you is serenity The comfort of a blanket The eye of the storm
Don’t worry, I haven’t completely lost the plot. The obvious answer is…only when they tell you it’s ok. (Or they are doing a comedy routine.)
At the moment I’m in a show based on one of C J Sansom’s books. Anyone who has read the series will know that his main character, Shardlake, is disabled. At the read-through, I knew straight away that one of the scenes would make me feel uncomfortable. Shardlake is made fun of because of his disability and most of the cast are meant to join in the laughter.
I know, acting!
Thankfully the actor playing Shardlake was lovely in his first rehearsal. He told us all to go for it, and not to feel uncomfortable. He’s a bit of a Tudor fanatic and explained that people used to believe that disability could be a sign of inner evil.
The show has got me thinking about how much has changed since then. But at the same time, how little has changed.
People with disabilities are no longer hidden away from society but yet access to many buildings or areas can still be limited. Independent travel – already an issue – is becoming more difficult with the proposed loss of station ticket offices. Developers still get away without considering all access requirements in some new build projects. I love that the script writer put a reference into the play that there is ‘no welcome for the lame’ even now. Even aside from availability of disabled parking, some people feel entitled to use the limited spaces themselves – ‘it’s after 7pm, anyone disabled won’t be out at this time’ etc (yes, you do indeed get these sort of comments.)
Disabilities are more widely represented on TV now – thank you, Strictly Come Dancing and CBeebies! – but equal access to acting roles has a way to go. Where a disabled character is featured it is often a stereotype of someone ‘overcoming’ their problems, or being seen as an object of pity, rather than a positive representation of a character going about their normal life. (Another kudos point for this production is their Access programme, meaning that a wide range of people with different abilities have been included.)
There are many other examples I could mention – you don’t have to look hard to find them. But what is truly distressing are the attitudes that lead to them. In some cases it’s ignorance or lack of awareness leading to the problems not even being considered. But scrape the surface and there are much more sinister attitudes out there.
Some people may not find Rosie Jones funny, for instance. That’s fine. But what’s not fine is to laugh at the way she looks or sounds due to her cerebral palsy. People will casually fling out insults such as spaz or retard – ‘just a joke!’ – without stopping to consider what those words actually mean, and how hurtful they can be to those who they were originally used to describe.
There also seems to be a hierarchy of ‘acceptable’ disabilities. Heaven forbid that you raise a child like Pudding who will never be a ‘useful’ member of society. As a family we’ve had to develop thicker skins, but when someone stares or laughs at Pudding we do see, and it does hurt. And on occasions where my writing or our story has ended up more in the public sphere, there will inevitably be some troll who wants to tell me that my gorgeous boy should have been allowed to die at birth.
I would love to see a day when disability is no longer considered to be a tragedy or a dirty word. I just don’t think it’s going to happen in my lifetime.
It feels weird to say that on this blog, and also slightly wrong. Like I shouldn’t be.
When my son was diagnosed with a life-limiting condition back in July 2015, I was thrown into a maelstrom of emotions. Most of all, I couldn’t imagine ever laughing again, let alone feeling the deep contentment that I’ve settled into lately. But I guess that’s the thing about emotions – they don’t hang around forever.
Now, I have so much to be happy about: I have a lovely house and garden; strong family support around me; T is growing into an interesting and studious young man who frequently makes me laugh out loud with his quirky take on life; I’ve been able to get into more acting again; meditation has changed my outlook quite a lot; and going on HRT has also made a huge difference in eliminating some of the anxiety that I’d come to think of as normal for me. After two years of writing a gratitude diary every night I certainly don’t struggle to find three things to put down each day.
And then there’s Pudding…
When we got his diagnosis I was already struggling to deal with his whirlwind antics on a day to day basis. And now came the punch punch punch that MPS brings. Heart problems, general anesthetics, weekly trips to Manchester for enzyme treatments, issues with airways, joints…. Through all this there was the knowledge that he might be one of the unlucky ones, one of the ones with progressive Hunter Syndrome who will lose all his hard-won skills and die in his teenage years. That was confirmed in October 2015.
Then we were onto the next roller coaster of a clinical trial – the shaky hopes as we were hauled to the top and plunging despair as the reality of his decline kicked in, the impossible decisions that we were forced into making.
Now, all the hope has gone. He no longer has any treatment, only symptom management. We know that the inevitable will come to pass. Short of a miracle happening we will continue to lose him bit by inexorable bit. Anyone looking in from the outside might expect our situation to be much bleaker than the times I have described above. But with the hope gone we’ve also lost the deep lows. With the loss of Pudding’s abilities we’ve also lost the challenging behaviour. We’ve not necessarily stepped off the roller-coaster – I know there will be further ups and downs ahead – but most days we coast along fairly happily. Back then, I saw the future as a constant bleak decline, but in reality progress is more step-like with a sudden change followed by months of stability where we can settle into peace. After many years of saying I need to learn from him, to live in the moment, I’m pretty much there. And we have many beautiful moments.
Of course, it’s not all sweetness and light. Even Pollyanna had her down days. Ironically, reading the posts that I’ve linked to above I’ve just been crying again, but they’re tears for the me that was writing then. The me who was having to deal with all that. There are days that I cry for the future – a conversation with a doctor at Martin House triggered a vision of saying our final goodbyes to my baby. And there are other days when I cry for the now – when Pudding is unhappy and I don’t know why and can’t fix it, no matter how much I want to. Those times will undoubtedly become more common as we get further on.
One week ago a family said goodbye to their boy for the last time. I only met Thomas once or twice but that didn’t stop his death hitting me hard.
When I heard that he had been admitted to intensive care in early December I was hoping so much that he would pull through. I sobbed for days when he could no longer carry on. And on the day of his funeral I picked Pudding up from school and held him extra tight for as long as he would let me.
From the moment a parent hears about the diagnosis of mucopolysaccharidosis, the death of your child becomes a painful reality. It may depend on many factors – treatments, bone marrow transplant, early diagnosis – and may not come to pass for many years yet. But the knowledge of the possible hovers there anyway.
When I started making contacts with other MPS families after the first few months of shellshock it was a welcome relief from the terrible isolation of hearing that diagnosis. Finally a chance to meet with others who just got it. Who didn’t need anything explaining and wouldn’t give you that look of panicked sympathy before awkwardly changing the subject.
Joining groups on social media provided answers to many questions that I had, but also showed the side of MPS that was harder to deal with. The older kids that were declining, and parents who were already mourning their losses. It was cushioned though, with the hope that maybe that wouldn’t be the outcome for Pudding.
The years passed and so did more children. Each one is heartbreaking, but still muffled a little by differences. ‘He’s much older than Pudding’. ‘She had a different type of MPS’. A drowning man will clutch at a straw…
Tucker. Matthew. Jack. Jamie. Names I knew but not well. Death and grief then marched on through families who were not just acquaintances any more, but had become friends. Ethan. Zack. Each one gets harder to hear about. Each time you wonder who is next.
Pudding turns 11 next month and Thomas wasn’t much older. They were both in the ‘chaos’ phase of MPS2 when they were tested for entry onto a clinical trial only a few months apart. (Thomas didn’t get in. Pudding did, though the benefits were sadly short-lived for him.)
I met his mum at a conference. We probably couldn’t be any more different in terms of background, experience, age, appearance and she’ll probably laugh at me for admitting that I found her rather intimidating to start with. But MPS has a tendency to dissolve away those barriers and I got to know an amazing person on Facebook, on the phone, and very occasionally in person – Norfolk is annoyingly far away from Yorkshire. Over the past couple of years, we’ve both seen our boys become quieter. We’ve both waited for our child to wake up from general anesthetic after a gastrostomy. We’ve both tried to balance the needs of our MPS child and their siblings. She’s not had an easy ride of it – let’s face it, MPS doesn’t give many people an easy ride – but she’s always been there to offer advice and support for others. Even after her own loss she continues to be generous in that way, letting me blurt out my emotions on here.
Because the emotions are inevitably there. Picturing him in hospital, knowing the discussions that took place with family and consultants, hearing about arrangements with the hospice… Knowing all that, it is impossible not to imagine us in the same position and wonder how long it will be.
On the day of Thomas’ funeral I so wanted to be there. To hug his mum. To cry for him, and for Pudding and for all the other children that MPS is stealing away. It just wasn’t feasible to travel that far, but I hope she knows that my heart was there with her.
I was nervous about writing this post. I knew that anything I wrote about Thomas would inevitably be more about the future that I know hovers over Pudding. And I knew that I wanted to find the right balance between expressing that and honouring Thomas’ life and loss.
So the best thing is to leave the final words to his mum. As she wrote on Facebook last week: ‘My darling boy I will always love you. You will always be with me and thank you for everything you have taught me.’
Dedicated also to the memory of Andrew who died the same week and was so supportive of my blog. xx
Warning: contains plot spoilers for the film Arrival
For a couple of years now we’ve had a family tradition – every Friday once Pudding is in bed, we settle down to watch a film together, taking it in turns to make the choice.
A few weeks ago we had a visitor from another MPS family staying with us. Evan is travelling the world on a fellowship, finding out how a diagnosis of mucopolysaccharidosis affects families like ours and learning from clinicians and policy makers who are trying to make a difference.
Although I was a little nervous about having a complete stranger to visit, he slotted right in to our lives – playing chess with T, discussing music with Hubby and having some beautiful moments with Pudding. He wanted to be treated just like one of the family, so of course we gave him the choice of film that week. This was a responsibiliy he took very seriously, considering what we’d seen previously and what would suit us all. A film about aliens arriving on earth and the difficulties of communicating with them seemed perfect, so we sat down to enjoy Arrival.
The opening scenes were a bit of a reality-check for us all. The protagonist, a linguistics professor, separates from her husband and watches as her 12 year old daughter dies from an incurable condition. A little hard-hitting when we’re facing the same situation ourselves. (Not the separation, don’t worry!)
Our guest was mortified and immediately suggested that we could watch something else, but we figured that it was just a back story and wouldn’t feature again. We were wrong. Towards the end, visions of her daughter return and it becomes apparent that contact with the aliens has enabled her to experience time differently. She is actually seeing visions of the future and now faces the choice of whether to go ahead, knowing what will happen. Evan groaned that he couldn’t have picked a worse film for us. But I don’t think that’s true.
The choice faced in the film has stayed with me over the weeks since. What would I do….?
I’ve written in a post previously that if I’d known Pudding’s diagnosis during pregnancy, I might instead have chosen to have an abortion. That I’d rather spare us all the heartache and loss that his MPS will mean. How could I bring a child into the world only to also give him suffering? How could I do that to us and other family members?
The point of the film though was that she could see the loss and the heartache that was coming. And that was almost too unbearable to contemplate. But she could experience all of the rest of it too. The love. The joy. The wonder. And the chance of those was too much to turn down.
And I’ve been torn. What would I do…?
When it comes down to it, there could only be one answer. I’d choose the tears. I’d choose the moments of contentment. The joy. The smiles. The curly-headed whirlwind. And the snuggly boy who purrs warm breath on my shoulder. I’d choose heartache that only comes from love. I’d choose life.
‘Which one?’ you may ask. I suppose many could apply to me. Irritating? Infantile? Idle? Introverted? No, I’m talking about inspirational.
Last month I did a talk to donors for Action Medical Research. They’d asked me because Pudding and I had already appeared in a video for them to raise money for research into Hunter Syndrome. I have done talks before but it was still nerve-wracking. Not just because it meant travelling to London when I’d not been on public transport at all since the beginning of the pandemic, but also because a lot had changed since the last talk I’d done. Pudding’s condition has got worse and I know we’re heading towards the final years, so any subjects that I was touching on were more about reality than potential future. I just wanted to get through the talk without crying myself.
I got through it and although I made other people cry, I held it together myself. Afterwards, various people came up to me and told me that I was brave and inspirational. Now I’ve never been very good at accepting compliments and I’ve written about this before. I don’t think I’m brave – I’m just doing what I have no choice about. And believe me, I get up some mornings and feel full of resentment that I have to get medication ready, access Pudding’s g-tube, clean the stoma, change another pad and that this will happen every day without let-up until the end. (And then I cuddle him and all the grumpiness and anger dissolves.)
As for inspirational? The word is sometimes flung around without any true appreciation of what it means. ‘To inspire – to fill someone with the urge to do or feel something’. A lot has been written about the pitfalls of inspiration porn – where disabled people get labelled as inspirational simply for going about their daily lives. I don’t think I’m any more deserving of the word myself and I’m sure many of my fellow bloggers would say the same about themselves. Yet, we do write for a reason, and yes, perhaps we do want to be inspirational.
To inspire other parents struggling with a diagnosis to feel less alone.
To inspire you to make a donation to help research into rare disease or other charities.
To inspire you to think of wheelchair users or those with visual impairments before you park on the pavement.
To inspire you to reach out for help with mental health issues.
To make your workplace more accessible.
To remember that you never know what struggles other people are going through and to treat them with the same kindness that you would want yourself.
So…if my words have ever inspired you to do any of the above, then perhaps…you can use any adjective you want. (I’m still more comfortable with irritating and introverted though!)
Last month I booked a burial plot for my ten year old son.
That’s a sentence I never expected to have to write when I first got pregnant. Even when he was diagnosed with a life-limiting condition and I was dealing with all the emotional fall-out, I still didn’t really believe it. That is, I did believe it but I don’t think you can ever truly grasp the reality until you’re closer to it. Let’s face it, when you’re watching a cheeky 3 year old making another bid for escape from the play-park, it’s hard to think of those details.
We may still have years yet before we need it, but we’re definitely closer than we were. Seven years down the line and my chunky active toddler is now a ten year old in a six year old’s body, with the joints and mobility of a much older adult.
Ever since Pudding was diagnosed with MPS (Hunter Syndrome) I’ve always faced this journey we’re living by wanting to know as much as I can about what’s coming. And death is no exception.
And yet, it’s been hard to contemplate the realities of death and all that it seems to entail. A black hearse with flowers in the shape of his name, just like the one in a TV programme that bulldozed Hubby and I when we were watching it. Regimented rows in the cemetery with bunches of flowers and fading teddies. I didn’t want any of that.
I’ve always fancied a green burial myself and when I started looking into that for Pudding I came across the Natural Death Handbook. It was a revelation to me. Not just full of advice about natural burial grounds and finding a funeral director, it’s also got personal stories of how people have done the whole thing themselves. Stories that have made me cry, yes, but also smile and laugh and say ‘Oh yes that’s how I want things to be!’ Stories that take away some of the fear.
We don’t have to have a sombre ceremony in a crematorium. We don’t have to have a hearse. We can hire a hall and decorate his coffin with ribbons and drive to the graveside ourselves. Or not. I don’t know the details yet. But having had a fairly unconventional wedding, it’s comforting that we can do an unconventional funeral too. Our way.
I was apprehensive when I visited the burial ground for the first time. Would it make me sad? Would it live up to my expectations? But when I walked down the little lane in February there were birds singing and bulbs starting to come up. Pushing the gate open and taking it all in, the first thought that popped into my head was of Pudding in his cheekier days peeking out around a tree. And I knew it was the right place for him.
I just hope it will be a while yet before this new reality kicks in.
When Pudding was first diagnosed with Hunter Syndrome (MPS) at three years old his future was laid out before us.
He would continue to gain skills for a few years, albeit slowly because of his learning disability. Then his ability to learn would plateau, with everything staying stable for a while before the learning curve started to fall in the other direction. Skills and abilities would be gradually lost until his body couldn’t take it any more and he would pass away.
It was all so very unreal at first.
In that first summer, when the sun was shining and I was surrounded by people enjoying their normal lives it just didn’t seem possible that I would some day have to say goodbye to my gorgeous boy. Watching him pull every book off the bookshelf in turn just to look at one page then discard it, or chasing after him when he made yet another bid for freedom from the playground, it didn’t make sense. Even when I was holding him down for another needle, or getting the results from the latest tests on his heart or airways (tests that already showed deterioration that I was previously unaware of) I still half-expected someone somewhere to tell me there’d been a mistake. The blood test had been checked again and everything was fine: he didn’t have MPS after all. A pipe-dream, of course. That call never came.
I’m not sure I can identify when his skills started to plateau. I do remember the joy of any small win. Watching him jump for the first time, hearing a new word spoken, seeing him respond to a simple command – these were all causes for celebration. Yet how can you truly tell when the little gains stop? It’s not something you can easily quantify. All you can do is suddenly realise that it’s been a long time since any new skill has been ticked off on the inevitable questionnaires.
Even then, as other parents saw me going from 0-60 to run after him yet again, I would joke that for a child with a life-limiting condition he was ridiculously healthy. Yeah, I know, inappropriate humour becomes a bit of a survival mechanism.
Now though… Now there’s no denying that we’re on that downward curve. Pudding no longer runs anywhere – he has to be persuaded to move from his chair. He still enjoys some food, but it’s taking him longer and longer to eat and his (much-delayed) gastrostomy is becoming more necessary. He is on medication for his bowels, for reflux and for joint pain and stiffness. Less mobility means more circulation problems and chilblains recently became an issue. Then just before Christmas, my poor boy started getting periods of gelastic seizures – bouts of laughing or crying for no true emotional reason. (And before you ask, the periods of laughing aren’t cute. After a few hours, they sound hollow and rather creepy.) Whereas before he would greet the whole world with open arms and a big grin, now we have to work hard to get even a brief smile.
As the medical issues stack up, all I can do is look out for the next lot of symptoms and hope that we can keep him as safe and happy as possible.
They say the lobster in the pot doesn’t know what’s coming, doesn’t notice that the water is getting hotter. Unfortunately, I’m not a lobster.
It’s an inevitable question once your child is diagnosed with a life-limiting condition. It’s a question I’ve asked our consultant and one that others have asked me.
How long do we have?
How long before we know for certain? How long before we will see the benefits of treatment? How long before he loses his speech? How long before he can no longer walk? How long before he needs peg feeding? How long before it becomes too much for his heart and airways? How long do we have? How long? How long…
The answer to the ultimate ‘how long’ is elastic. It was cut brutally short at diagnosis but stretched again when enzyme treatment started. When it was confirmed that Pudding had the worst possible outcome (a complete gene deletion) that elastic contracted again. And then it eased out again once he got onto the clinical trial.
Our boys all seem to react to Hunter Syndrome so differently – some are plagued with chest infections, ear infections, hernia and so on from the get go. Others like Pudding seem to escape much of the bodily effects and present mostly with development delay. So when it comes to asking ‘how long’, there is no easy answer that the doctors can give.
But now having stopped the trial and no longer having treatment, we’re back to that pitifully short length of elastic that we were left with when we first had his diagnosis confirmed.
How long do we have before he will no longer reach out to hold my hand? How long before that infectious and increasingly rare smile disappears for ever? How long will I still be able to hold his solid little body to mine? How long before I plan a funeral? How long…
One of the hardest things I’ve ever had to do on this MPS journey was just before the final diagnosis. Hubby (who I’d kept in the dark about all my late night googling) suddenly asked me, ‘So what’s the worst case scenario?’ and I had to tell him that we might lose our little curly-haired livewire as early as ten years old. That’s not likely to be the case now. At least I hope not: he’s already nine and a half. Most estimates of life expectancy before enzyme treatment became the norm were around 14 years.
And yet, I continue to count our blessings. It may seem strange, this insistence of mine on our good fortune, but compared to some other families I know of, we are lucky. Although Pudding is declining, although his condition is life-limiting, we are not yet in the life-threatening stage. I don’t go to sleep worrying that I’ll lose my child to a seizure in the night, or have another acute illness hanging over us.
How long do we have? It could still be years. It could be quicker than we expect.
Maybe some day we will be sat in another clinical room and the consultant will shake his head and say, ‘I’m sorry, you only have…’ Maybe if I had a crystal ball I could check and see how long that will be.
But when it comes down to it, I’m not sure I want to know.
Some places are just special. Martin House Hospice is definitely one of those.
You feel it as you walk in. The welcome smile, the rainbow of pictures, the jars of cakes – it all feels like one big warm hug.
Of course we haven’t actually been there for a while. We were due for a visit at the end of March 2020, but then…well, I think you all know what happened. But Martin House has still been there for us. There have been parents’ meetings on Zoom complete with a delivery of cakes, activities sent through for siblings. And most importantly, there has been somebody at the end of the phone when Pudding has given us a worry (which he always seems to do on a weekend when it’s much harder to reach our normal hospital staff!)
Many charities have struggled over the past year and a half as normal fundraising events have been cancelled and donors have been faced with different priorities. So when my aunt let me have a beautiful quilt that she had sewn herself and asked me to use it to raise some funds, I knew exactly who I wanted to benefit.
This lovely purple hexagon quilt could be yours. To be in with a chance, follow the link below and donate at least £4.
I’ve chosen to do a ‘hundred square’ fundraiser, so everyone who donates has a fairly good chance! Well, better than a standard raffle anyway…
Just click- here – to make your donation and don’t forget to choose the option that will allow me to contact you or otherwise I won’t be able to let you know if you’ve won!
A Hunter's Life 