Extended Pause

10 weeks. No dialysis. We’re not supposed to have made it this far. If you’d had plotted a graph with RD’s blood chemistry results as they had charted previously, the odds were stacked against him.

Our little underdog.


Not only is he still here, with us, he’s living. Of course there are changes, my eyes are open. Fluid balances being tinkered with and then tinkered again. For our sanity, I needed to know- is RD showing some kind of radioactive gunk, superhuman strength against blood toxicity that could fell a large horse or, actually has it somehow plateaued? We’d agreed no more invasive treatment, and that included blood tests, but here I was going back on my word.

And here’s why: this stasis is stifling. The worry that sits like a stone in my throat all day, everyday is heavy and exhausting. You know that worry as a parent all the time; a sickness bug, a temperature that lasts longer than you expect. How helpless it can make you feel. How you wonder when it will get better so life can continue as it was before. How you worry about too much or not enough medicine. 

It’s that but endless. Or actually there will be an end but you don’t want it. Or maybe you do want it, but you worry how that sounds. Those niggles that eventually all you’ll be left with is memories of this time and how annoyed you are that its filled with worry. But you can’t make the two mutually exclusive.

I’m worried that in this timeframe I’m not working, not providing. I’m worried that grief will rob me of my ability to function as I was before ever again.

I worry about worrying. About not worrying. About driving myself insane. About driving others insane.

So I needed to know. We needed to know.

And as ever RD has pushed the barriers of the understanding of his disease. His levels have plateaued or increased marginally. There quite possibly is some radioactive gunk afoot. The prediction now, akin to licking your finger and holding it in the air, is that RD will have several more weeks, even possibly months as he is.

Things we’d put as impossible: returning to school, going on another holiday, planning a 6th birthday party are returned to us. Returned with a caveat, but there nonetheless.

Here’s the big one: he’s not expected to die suddenly anymore. 

And whilst that’s smack your arse and kiss your grandma amazing, there’s also a terrifying worry about how he will deteriorate. I can only hope for what we’re seeing so far.

RD. 

The difficult stuff; he’s tolerating less and less feed. And less and less fluid. But it’s not because he’s vomiting lots. It’s affecting his blood pressure so our determinants are him going more floppy, tired, irritable and cold hands and feet. Hard to distinguish from his overall decline. Because he’s generally all of those things a bit more now anyway.

The good stuff; his zest to live and love is unaffected. He is gloriously naive to what is happening. (Well, apart from his erythropoietin injections and he’s made his feelings perfectly clear about those). He tries and tries to carry on, he’ll just take more cuddles and more naps. And we are happy to oblige, indulge and breathe him in.

First, for memories. Second, to try and absorb some of that radioactive gunk superhuman stuff so we can all keep going.


DD.

Well, she tried to cut off his head with a toy chainsaw.

Broken Sweetness

Caring for someone you love when they are ill is like sustaining a hundred impacts a day. Something going wrong. A night of pain. Their fear. It is your honour- and your only plan- to absorb those blows, so your broken sweetness doesn’t have to.

Caitlin Moran, Times Magazine, 23rd July 2016

Apologetically I’m being asked if I’m okay, how I’m doing? I say apologetically as I know it seems a futile question. But those little reaches, the fingers of trying to understand, are so warmly received. Apologetically I can only muster a futile response. Turn it into a status report of RD that day. 

But this is how it is. It’s raw. It’s everyday. It’s mundane.

With every good day RD gives us, I smile with him, love with him, try to remain in that moment. But a voice tells me that I am failing him. That he is fighting but I have handed over that fight.

With every good day I worry that I’m doing enough to make these days count. Is there enough for him to experience a lifetime in these weeks? His cognitive disabilities mean his demands are refined and relatively easy, but I wonder whether he wants for more but cannot ask for it.


With every signal of change in RD I wonder where the balance will tip and falter. Whether we’ll enter a phase of more bad days than good. He’s currently not tolerating his feeds, and screaming in pain until we have reduced them to half. We’ve have spent his lifetime tinkering with his calories and his gastric wibbles, building him back from periods of intolerance for six years. But now there’s no going back. So entering a phase of calorie counting and measuring feels mundane and everyday for us, and yet the future phases aren’t.

He’s sleeping more. He’s a shade of Churlish Green (Farrow and Ball TM) but without a hint of churlishness. He’s losing weight and muscle mass. But because of that he loves to be held. So I take him in my arms and try to memorise the weight, joints and smell of him before it becomes a memory.

And DD. She watches wide eyed and silently. She makes heroic efforts to gain attention, which essentially involves being a massive d*ck. But she asks me if I’m okay when she hears my voice crackle with tears even when she can’t see I’m crying. She ever so quietly slips away and returns with her favourite toy for RD to cuddle in response to his agonising cries. 

I find myself wanting to retreat from others and yet also desperate to keep them close. I want to write about this and yet don’t want to be a car crash for a good rubberneck. I want to go and get inappropriately shitfaced. I want to wake up and none of this to be true. I want to run up a hill and punch a fucking cloud.

I want to worry less that others are struggling to deal with this. Because I’m not dealing with this. I’m in a stilted reality, aware that my very universe is going to fall out of my arse. And I’ll just be stood there shaking and sh*tting and crying. I’ve just had years of keeping a lid on my fears over RD’s mortality. He felt like he came on loan. And no, not from heaven. Or God. But to show me what is true and pure in life. So for now I don’t cry much, but soon I know I won’t not cry much.

So how am I? I look the same from the outside. Got a nice new jumper. Wearing lipstick. Maybe going for the odd run. Still whispering FFS under my breath what feels endlessly at DD. Taking a billion photos and videos of RD on my phone. If you saw the facade, I probably look like a cut out and keep mid thirties mum. But my insides feel like jelly that’s been shook up and gone into that pile of bits.

Bit of a roller coaster then…

How to Exist

How to Exist: 

Get up, even though you’re not entirely sure if you’ve been asleep. Actually, you must have slept as those nightmares that leave you screaming and sweaty require sleep.

And then just be. Keep busy. Shut it down. Focus on the present.

I’m not hysterical. In fact it’s unfunny how unmoved I may currently appear.

But I’ve made myself a promise: I won’t force emotion. I will just go for a ride on whatever appears. And so far that is the cart with three wheels but painted beige: wonky and meh.

It appears that even though there is an inevitability to the decision we’ve come to, there is no predictability.

It could be sudden. Imagine that? I have. Finding him in his bed, the phonecall from school. Yes, school, because he loves it, and the veritable King like status he now holds means he gets all the things he loves. Then odd thoughts: out in the park, a cafe, in the car. Having to try and explain that no, no, don’t call an ambulance.

It could be slow. Imagine that? We have medicines for nausea, spasms, seizures, increased secretions.


So we wait and watch. Hold him tight but let him be free to enjoy every day as he wants.

But here’s what helps us exist, RD is unsurprisingly surprising everyone. Bar the increasing weariness and muscle fatigue, his itching is under control. He’s still engaging, giggling, pulling at my hair. I can see that he’s getting paler, his eyes puffier and darker, but his mind still races with the million impulses to find all the toys and press all the buttons.

Also, the kindness of others. Visits, messages, post. Thankyou.

Grief is stilted. Part of me still waits for this all to be A Mistake. The wrong NHS number, an inaccurate measurement of blood toxins. So I guess denial plays a strong hand too.

The cart is on a slow trundle to a cliff edge in the fog.

Hello?

So, we’ve decided

We think it’s best

After looking at all the risks

There’s no way to write those texts. To make those calls. Dig deep into the energy reserves to speak of the unthinkable.

It’s fair to say that 2016 has been one massive kick to the balls. Another baby loss. Then David Bowie. And frigging Victoria Wood, I mean, come on man. 

Whilst the majority of the nation voted to leave last week, our medical team were also coming to a controversial conclusion. But the resemblance in the situation ends there. What use is political posturing in the face of real, actual grief?

But before that conclusion, we had a glorious week on holiday. It wasn’t a cruise, but I can only describe the underlying reason for that last minute break felt like seasickness. There were little toes dipped in sand and sea, relaxing in the dappled sunlight through a forest canopy. A slow clock and our child’s desires first and foremost. 
And then the weekend, where between my brilliant, generous friends and the charity Make a Dream, we treated RD and DD to their first Big Weekend. Parties galore. Watching RD enthralled by a live brass band, surrounded by his very own band of brothers renders me speechless and wordless.


A week to last a lifetime. Which now it will need to.

That’s probably the best way I can put it. Awkward and unreal. Renal replacement therapy is failing. I won’t put all the reasoning here, but here are some facts:

RD will no longer be cannulated; pricked and bruised repeatedly for his battered veins only to reject it within a matter of hours. No more repeat repeat repeat blood tests. No more hospitalisations. 

For the next howeverlongitmightbe our goal is to spend each day as it comes but with the minimum intrusion and discomfort. Just full of love and everything he loves.

RD isn’t unconscious. Far from it, in spite of his blood toxicity he rallies and giggles as he has always done. The increasing itch and muscle fatigue just peeking out in between.

He’s not covered in wires. There are no machines to stop. He is surrounded purely by as many cuddles as he will tolerate and desire.

We are fortunate to have this time. For all RD to know and remember is love. For DD to feel just as present in that. His life has been forged and etched by only him, and now this stage will too. 

The pain is like I’ve swallowed a burning hot drawing pin that never cools. I’ve had to discuss things no parent should. I have had to imagine handing an ambulance driver a form that says ‘Do Not Resuscitate’ because I know I’m likely to scream, or quietly beg repeatedly to save him. 

I have a bag of medicines that remain sealed until The Time.

Grief is on hold, cornered, ready to pounce. Now is about the here and now.

(Photo credit Rebecca Lupton. Laughter credit DD and her knickers)

Quiet Storms

Sometimes crises aren’t loud or newsworthy.

They’re quiet and insidious. An intake of breath, an apology.

My heart pounding in my ears louder than the VTech Toot Toot toy that RD presses over and over. Louder than his peels of laughter at the power of his fingers on a button.

A simple lifting of a vest. A snake of redness etching up from a dialysis line. 

It’s only been two weeks since those conversations. Three weeks since this new line has been fitted. Everyone in the room knows this is not good. I feel the hot burn of vomit in my throat. Try to shush the panic, remember, no assumptions, question, question.

We sit and wait. There will be no dialysis today. We will not be going home today. RD is oblivious, on glorious form. No temperature, no signs of illness. No dramatic ambulance ride. I’m not sure how to phone wolf and tell him; only four days away from hand in at the end of his first year of his PhD. Three days away from Father’s Day his son’s life is being quietly threatened.

I channel my five year old self and phone my parents first, test out sharing the news. Quell the panic until we know what this really means. The surgeon arrives, confirms that the only way to fix this is to take out the line. Slight crumple, deep breath. But offers a little hope: RD does have another vessel if we wish to try again. 

It feels like a life vest made from barbed wire for now, given that RD has had two dialysis lines placed in ten weeks, eight weeks of dialysis, two serious infections and one undiagnosed virus with serious anaphylaxis. That’s not good odds. 

I just know I’m getting taken to the room with the bile yellow walls and the box of thin, inadequate tissues. I phone wolf, hear his breath leave him. “He’s got another line infection” enough to transfer the jolt of pain through him.

The last few days are too raw still. Too many conversations to unpick. The option of fight or flight has hit wolf and I differently. We look at each other wanting to come together across this gulf.

I can see that the recommendation of the doctors may not be too pessimistic. That we are hitting too many hurdles. Putting him through the pain of dialysis: the insertion of another line, the management of it when actually it is a law unto itself in RD’s body, awaiting what feels like the inevitable infection- how is that in his best interest? Yes dialysis itself has transformed his health and he’s so brightened and alive on it, but the means to dialyse are not working.

And then, what if no infection, or it lasts long enough to get to transplant: the intense immunosuppressant therapy required. He’s now classed as severely allergic to two antibiotics and one antihistamine. He has a history of gut shutdown, a known side effect to these drugs, and if we’ve taken that vessel for dialysis we have lost access for intravenous feeding.

I have screamed in pain in RD’s bed imagining him no longer being with us, but I wrangle with what his needs are. Do we decide to fight and let go when there is no choice, or do we know we’re on a path to no choice that is filled with risk and pain and wonder whether it’s worth walking down? RD only knows what is happening to him in the moment. In spite of his cognitive disabilities I have fought for him, but now because of his cognitive disabilities I wonder who I’m fighting for: more time for us or him.

Non miracles aren’t newsworthy. 

Wolf wants to try again. It’s in his gut. And I love him for it. It’s an impossible fight to have between us because there are no winners.

For now we’re going away on holiday next week. We’re having photographs taken. A charity is organising a party for Rufus with a live orchestra for our own little maestro. A chance for us all to love and celebrate him. The hospice has been engaged for more than respite.

And then we will meet again with the medical team around us, to try and find a way to navigate this. Push or pull. This unchartered territory has been invigorating but now it feels impossible.

The Bags are Packed but The Shelves are Full

A pair of shoes for the day. Maybe two- closed toe and sandals. A dressy shoe in case you get to go out somewhere nice in the evening.

A capsule wardrobe carefully put together with a key colour and some accessories that can dress something up or down.

Matching undies and maybe a nice nightie. It is holiday after all.

Certainly not: 3 cardigans, spot cream, knickers you last wore whilst doing burpees in a muddy field and no pyjamas. 

But then my bag was packed by a flustered husband who was trying his best. Who had last seen his son unresponsive as an antibiotic anaphylaxis bloomed across his porcelain white skin. After carrying his 3 year old daughter on his shoulders to find the car I’d abandoned. I had had to call 999 from the side of the road. I’d dialled as I’d  watched RD seize in my rear view mirror.

But that was a few weeks ago now. On the weekend that was British Summer Time. At 10.22pm I watched as RD was put to sleep in my arms, his septic dialysis line apparently almost fell out in theatre. It should do, it had only been there for 5 weeks.

There had been some signals of an undercurrent of a burgeoning conversation.

In A & E, as I held the oxygen close to RD’s face whilst keeping up the toddler conversation with DD as she ate “her favourite sandwich ever” on the floor in resus, there was a brief snippet.

“The line has to come out”

“Okay, and then what?”

“Well, then maybe nothing”

I’m guessing that I didn’t respond with a ‘smeyes’. Sorry Tyra.

“Oh no, I mean we see how he does whilst we clear the sepsis”.

And then, a few days later, in a stuffy hospital cubicle. We discussed how serious the episode had been, and how it could have been a lot worse.

So yet again, what next?

Well, perhaps nothing. 

Nothing. The end. Making him comfortable. Weighing up the risks and benefits. 

I was firm that no, this was not the end. The line had not been placed well, it was always at risk. We had to try again. Dialysis had been transformative. I was already burdening the guilt of the infection. I could take on more if I was wrong. 

I am a mother. Wolf is a father. ‘What is best for us as a family’ is that our children have the best opportunity to feel loved and thrive. Both of us would lie on the metaphorical train tracks to save our children if needed. But we are not foolish. Or naive. Our eyes are wide open to the bumpy road that awaits us. Well, perhaps a little foolishly hopeful. Because if not, then what?

You put that conversation on a shelf in your brain is what. And continue.

After 2 weeks of intravenous antibiotics, RD wasn’t coping without dialysis. The line was going back in. We decolonised him- human sheep dip. We prepped a pain relief plan post surgery given he’d struggled so much the time before, we spent time with the surgeon discussing what line and best placement.

All went well. We felt vindicated. RD came home full of beans after another cycle.

24 hours later he looked a little pale. And hot; Over 38 degrees. Which when you have a central access line is immediate hospital admission.

Here again. This time I’ve spent the morning not showering in case I miss ward rounds. I’ve lounged in my pyjamas with the mist of grossness that can only be achieved from sleeping on a waterproof mattress. So, what next?

I get ushered along the corridor in my pyjamas, a hospital Walk Of Shame. To a small room with shit sofas and school toilet roll tissues. Been there, done that. No good comes from these rooms with their walls that could talk of broken hearts and dreams. But in a jolly shade of bile yellow.

Next. Next is:

a) this is a random virus. Treat it and the allergic reaction to the antibiotics. Continue on dialysis.

b) the lab grows cultures that we can blast with antibiotics, we hope.

c) the lab grows a bug that can only go with removing the line. But then what? Then nothing. That’s the end of the line and the end of the line. No more access. 

So how long with him then?

We don’t know.

Days? Weeks? Months? 

Weeks. 

Fuck.

We have 24 hours to wait. 24 endless hours. I grip tightly to case a.

Monday morning, negative cultures. The line is safe. I feel like I could fly and vomit at the same time. A clever friend of mine tells me to put it on the shelf in my brain. All this talk of mortality. But my shelf is full. Over stuffed. 

When RD is well, I would very nearly hoik out my kidney with a butter knife myself and give it to him. When I watch him suffer, scared and unable to understand any coping mechanisms, I worry. I worry about making the right decisions. I can only lurch from event to event, hoping that I’m doing the right thing by him at each turn. We are his voice, but sometimes I can’t understand him.

The best tonic I had this weekend was from my 5 year old niece. Bright and interested, she asked pertinent questions about her poorly cousin. In a gloriously fresh view on life and death she helped tidy the shelves.

I’m going to embrace my inner five year old: question things simply and succinctly. Stop making assumptions. And see the possibilities in everything. Including my 5 year old son with the strength of an ox but as fragile as eggshell.


And if you are on holiday, and not having to wrangle with Ryanair over a refund, please please soak up every glorious second. But you can fuck off with your tan.

Find Your Tribe

Before you, I assumed This Wouldn’t Happen To Me.

Before you, I assumed that if it did, I would be prepared. I would know what to expect when I was expecting.

Before you, I assumed I wouldn’t cope.

After you, I assumed that we were one blood test away from closure. One blood test that would make the connection.

After you, I assumed we’d never find our place. I searched and searched, I denied and denied.

After you, I assumed my life would be full of more worries than happiness.

 “When you assume, you make an ass out of you and me”

 

But here you are, my little blonde pixie.

Rufus-49.jpg

Here’s what I know;

You haven’t caught up. You have no recognisable words. You have legs like twiglets that don’t support you well.

That one blood test took 4 years of many many tests, and it has taught us nothing more than we had already learned so far.

That one box is never enough on any form. Nor is the answer to “what’s his diagnosis?”. Or less politely “what’s wrong with him?”. That’s unlikely to get a favourable response.

More of you doesn’t work than does.

Here’s what I’ve learned;

The true definition of quality of life. You try with your whole. When you love, you love with all of you.

You have enriched us all. Not just us as a family of four, but wider family and friends.

That every goal you achieve, no matter the scale, is enough. You are enough.

That you are a pure soul with the heart of an early 90s raver: there are never enough flashing lights or tunes.

That some days are harder than I could have ever have assumed. But not because of you, but because I have to hold you down when you’re scared. I have to let people prick and poke at you. I have to quietly shush in your ear so that I don’t cry. When I see people look at you and not see what I’ve learned.

That I have a fight in me I never knew was there. How to truly advocate for another.

That sometimes there are more questions than answers. That life doesn’t always get worse even when the chips are down.

That we are not alone. We weren’t alone when Undiagnosed. We are still as supported in a community now we have some answers as we were when we had so few.

We found our tribe. Unconnected by symptoms and level of ability. Connected by a feeling of otherness, of feeling and finding our way through the unknown.

SWAN UK is more than a support group, they have a Big Ambition:

  • They want to ensure every one of the 6000 families that have a child born with a Syndrome Without a Name every year, have the support they need, when they need it.
  • They want recognition that being Undiagnosed is not always a temporary stage.
  • They want every child and young person with a Syndrome Without a Name to receive high quality coordinated care and support both in hospital and at home, regardless of diagnosis.

That Big Ambition needs financial support. As our fourth annual Undiagnosed Children’s Day approaches, please help us support it by donating any amount to:

https://www.justgiving.com/swanuk/

Text: SWAN11 (amount) to 70070

 

And if you are a family needing that support:

 

info@undiagnosed.org.uk

undiagnosed.org.uk

undiagnosed.org.uk/blog

020 7704 3141