A year living with Complex 1 Deficiency (part 1)

Before September 2015 (and after that terrifying night where she died outside the doors of A&E) we lived in hope that life with Rosie would get easier. We lived in hope that one day a cure might be found, that she would get to experience the same things that every other healthy child experienced. We lived in hope that she could get stronger, learn to manage her condition and spend less time in hospital. We had hope.

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That hope was shattered one day in late September 2015. Rosie was back in hospital. She’d been in intensive care for about 2 weeks already with another common cold. Our Neurologist came in one day and told Iain and I he needed to speak with us, that they’d found some more results from the muscle biopsy taken earlier that year, a mitochondrial disorder called complex 1 deficiency.

A mitochondrial disorder relates to your energy levels. It’s the thing inside every living cell in your body that creates energy for your body to function normally. Rosie’s energy levels were not as they should be.

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A meeting was called by our Neurologist and a number of senior doctors escorted us into a room a short while later. I’d seen it quite a lot in the intensive care unit at Bristol. A number of health professionals gathering before ushering parents off the unit into a private room – I’d never really seen the outcome but had always been intrigued. I saw people go in, I never seemed to be around when it was time for them to depart.

It was our turn. I knew it was bad. I could feel it. The air felt tense and stressed. Iain and I were tired and emotional and I prayed it was just me. I later discovered Iain had felt exactly the same.

We were taken into a small room and invited to sit. With a calm and compassionate voice, our neurologist explained that further testing on Rosie’s muscle biopsy had revealed a secondary life-limiting condition, Complex 1 deficiency.

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All I remember is the words “It’s not good news“, presented in a low, slightly quieter and slower tone than before.

The room fell silent. I physically felt the last remaining strength within me wither away. I thought I was the one dying.

Our hearts wretched into our mouths as we learned about the condition and its degenerative, life-limiting qualities.

It seriously was bad news. An incurable degenerative disorder that effects the ability for the major, energy requiring organs to function. Over time we would have to watch our little girl’s organs fail, right in front of us. Her heart, brain, eyes, liver and kidneys were all mentioned. We were basically being told that our gorgeous Rosie was definitely going to die. She was already on borrowed time and this was it. This is what would end her life.

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I broke down there and then. How on earth would we all cope watching Rosie deteriorate without being able to do anything about it. I couldn’t see my baby go through that. To be the little girl with a love of singing and making everyone around her smile to die in that way. I flung my hands to my face, burst into tears and wailed something about “Rosie not having a shit life”.

We were in the room for about 30 minutes, asking questions, trying to comprehend what  we had just been told before we all dispersed. Iain and I left the room red-eyed, heart broken and empty.

The next few hours were a blur. We seemed to be pulled from pillar to post, meetings to meetings. When we met with with palliative care team to talk end of life and what the options were, where we could go, who we wanted with us, and to start thinking about any special requests, I withdrew completely and went into denial.

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That night when the meetings were done and Rosie was asleep, Iain and I went to the parents room in the intensive care unit and started calling family. Tentatively, we each called our parents in turn. Where do you even begin to tell a Nanna and Grandad and a Granny and Papi that their only grand daughter had been diagnosed with this secondary life-limiting condition that was inevitably going to be a horrible death we’d all have to watch and it would happen pretty soon. Most likely before the end of the year.

The calls continued. Our sisters and brothers – Rosie’s loving Aunties and Uncles. Our own Aunties and Uncles, and our friends. It was serious. It was going to happen soon and we needed to let everyone know right then that time was precious and memories were to be made now. Right now.

My sister, Rachel, dropped everything there and then. Away for the weekend with friends she got herself to the station for the first train the next morning heading south. No plan, no idea where she was going and whatever the cost, she just headed south to Bristol.

I laugh now thinking about it but at the time she must have been in such a panic she couldn’t think straight or process what she needed to do. It took her multiple trains, a ridiculous amount of stops and I’d hate to even think about the money she spent. I think she took the long, scenic route, cross country, east to west and back again before heading south to get to the hospital, but my God was I glad to see her when she arrived several hours later!

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Auntie Rachel cheering up Rosie

She spent the next few days with us, cheering Rosie up, keeping her company when we were called into more meetings. The night she arrived, when we’d settled Rosie to sleep, I drove home (you can’t stay with your child when they’re in intensive care. The hospital has a few beds near to the unit, but since we only live 30 minutes from the hospital we know there are so many more families from much further afield who need the beds far more than we did). Talking through the last couple of painful days, the things we’d heard and being told, I was at breaking point.

The 30 minute drive was almost over. We were just approaching my home town of Clevedon and I remember turning to Rach saying, “There’s nothing left except for an absolute miracle”.

I kid you not, less than a minute later Mike and the Mechanics, ‘All I need is a miracle’ came on the radio. Rachel and I turned to each other and both burst into tears. I have not heard that song for years, there are not many radio stations that play that song and I prayed to God with all I had left in me, that it was a sign that someone, somewhere was listening.

An unbelievable moment I will never forget, and a song I will always hold close for as long as I live as a sign to never give up hope, ever, after what happened next…

Part 2 coming next…

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2 thoughts on “A year living with Complex 1 Deficiency (part 1)

  1. It’s 1.22am. The kids will be awake in less than 4 hours time, but I can’t stop reading your beautiful blog. Rosie is amazing. How can such a little thing be so so strong. And as you you two….I’m just sperches and in awe. You never know what path life has mapped out for you and you have coped better than we ever could.

    Love you x

    Liked by 1 person

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