A tummy bug left me with ‘locked-in’ syndrome

Fiona Duffy / 15 July 2019

When Norma Cullen got a sickness bug she never dreamed it would lead to Guillain-Barré syndrome and six months paralysed, on life support and communicating only by blinking. Two years on, her recovery has astounded doctors.



When I came down with a tummy bug in October 2016 I presumed it would be a short-lived bout of sickness and I’d soon be up and about again.

Factfile

Name: Norma Cullen

Symptoms: Muscle weakness and paralysis caused by damage to the peripheral nervous system

Treatment: Critical care, intravenous immunoglobulin (antibody injections), rest to allow damaged nerves to repair themselves enabling muscles and organs to function.

Recovery: Six months on a ventilator then 18 months of rehabilitation and ongoing physiotherapy

Prevalence: One to two people in 100,000 per year

At 66, I was fit and healthy – a keen walker and cyclist. I’d recently taken part in a 10k obstacle race and had even cycled from John O’Groats to Land’s End. I was never ill.

But over four days I deteriorated. Even when the vomiting and diarrhoea eased, I was too weak to get out of bed.

The last thing I remember is a bizarre, ‘fuzzy’ sensation all over my body and hearing my husband, Phil, say he was calling an ambulance.

Next thing I knew someone was urging me to open my eyes. It took all of my strength to momentarily flicker my eyelids – much to the delight of everyone around my bed. I was in the critical care unit of the Royal Cornwall Hospital.

For two months, I’d been sedated and on life support - breathing through a tracheotomy (surgical opening into my trachea). I was also completely paralysed - unable to move a muscle. Kindly nurses assured me that I was in safe hands and should blink once for yes and twice for no. For another three months that was my only way of communicating.

Doctors told me I’d developed a rare condition called Guillain-Barré syndrome - a rapid-onset autoimmune disease causing inflammation of the peripheral nerves which connect the nervous system to the body’s muscles and sensory organs. The cause is unknown but, in most cases, it is triggered by a respiratory or gastro-intestinal infection.

Instead of attacking germs, the immune system mistakenly attacks and damages the nerves.

The enemy within: immune system diseases

Of the different strains of GBS I’d developed the most serious kind – also known as AMSAN (acute motor-sensory axonal neuropathy). As well as damaging the nerves’ fatty or myelin insulation sheath – the condition had also affected the axon, or nerve fibre, itself along which electrical impulses travel.

Guillain-Barré syndrome

As my condition had deteriorated, sending my body into ‘shut-down’, doctors had performed an emergency tracheotomy - inserting a tube directly into my trachea – and putting me onto a ventilator just to keep me alive.

“But you’re going to get better,” Phil kept telling me. Unable to nod, I’d simply blinked once.

Apparently, I’d given them all a scare. Phil explained that I was so poorly at one point that doctors – who carried out blood tests and a lumbar puncture to make the diagnosis - warned it was touch and go that I’d even survive. Even then I could be on a ventilator for the rest of my life.

My daughter, Jo, insisted that the doctors were wrong. “This is my mum you’re talking about,” she’d insisted furiously. “And she’s a fighter.”

For the next eight weeks I’d clung to life – battling six episodes of pneumonia, continual lung infections and soaring and plummeting blood pressure. So responding to the doctor when he called my name was a turning point.

But my recovery was still going to be long and arduous. Not only would I have to wait for the affected myelin sheaths, which surround the nerves, to grow new cells to repair themselves. But I’d also need intensive physiotherapy to strengthen the damaged axons (central fibres) of the nerves.

Every two hours I continued to be rolled from one side to the other to prevent bed sores from developing. Still on life support, I also needed daily physiotherapy to prevent mucus settling in my lungs.

I understand now why they call GBS ‘locked-in syndrome’ – it was like existing in a bubble cut off from the rest of the world. Loved ones and nurses always included me in conversations but being unable to join in was beyond frustrating.

My next vivid memory is of a nurse dressed up as a reindeer cantering in and out of my room on Christmas Day - swishing her tail from side to side. I couldn’t smile or move but hoped she could see in my eyes just how happy she had made me. You can say an awful lot with just your eyes. I know now that they can show pain, fear, and laughter.

Nurses and family devised an alphabet board – pointing to letters until I blinked to make up a key word. But it was time-consuming, tiring and frustrating. Mainly they asked me to blink ‘yes’ or ‘no’ to questions.

Trapped in my own world, I experienced vivid dreams and hallucinations and would wake, confused and disorientated. Even to this day I’m unsure of what was real or imagined from that time. Each time, nurses would gently soothe me, remind me I was in hospital, and assure me I was safe and doing well. I’ll never be able to thank them enough.

New Year’s Day 2017 marked a turning point. When my grown-up son and daughter, and four grandchildren all came to visit, the consultant suggested wheeling my bed and ventilator outside.

I remember staring, astonished, at the bare tree branches. (I’d completely missed autumn). But it was fantastic to feel fresh air on my face. In that moment, I remember thinking: ‘I’ve got to fight this’. I was determined to get back to full health.

My close cycling friends visited regularly and kept my spirits up. I’ll never forget them tiptoeing onto the ward, in their best Lycra, to quietly sing Happy Birthday to me in January 2017. And I still have the knitted Norma cycling doll one of them made with the wish ‘Get better, Norma’ stitched across the jersey.

Over time, with physiotherapy, I managed to move my head a fraction then was able to turn it from side to side and up and down. From there I was able to shrug my shoulders, smile and even open my mouth to laugh silently – increasing my repertoire of communication! With every tiny improvement, doctors and loved ones would encourage me and tell me how well I was doing.

Just after Valentine’s Day, physiotherapists taught me how to use a voice valve by pressing it against my voice box and breathing through it. When Phil visited later that day I uttered my first words in six months telling him in a Dalek sounding voice: “I couldn’t tell you yesterday but I can do it now.  I love you’.

His jaw dropped to the floor. He gawped like a fish, said ‘I love you too’ and rushed over to hug me. It was such a pivotal moment. Tears ran down our faces.

By the end of March, I was well enough to come off the ventilator and was finally able to breathe and speak independently. I was moved to an ordinary ward then spent the following 12 months at the Marie Therese House in Hayle - a rehabilitation unit – where I learned to sit up and stand using a piece of equipment called a ‘sara stedy’.

A full 18 months after first falling ill, I was finally allowed home in March this year.

Carers visit me several times a day to help me wash, dress and perform my daily exercises.

I use a wheelchair to get around and tire easily – particularly when I’m trying to keep up with my grandkids on their bikes - but I’m getting stronger every day. As part of my physiotherapy I’m cycling on a pedal machine now and upping the distances, gears and speed and am about to start a course of hydrotherapy.

Over two months this summer I ‘cycled’ 86 miles – the length of Cornwall - on a fundraising mission for GAIN – the national charity for those affected by Guillain-Barré syndrome, plus the rehabilitation unit and hospital.

I charted my progress on a map and my loved ones were all there to cheer me across the imaginary finish line. I’m a member of Cycling UK and can’t wait to be back out there with my ladies club doing it for real.

It’s a long way off but I’m determined to get there. Over the last two years I’ve learned so much about the body and am in awe of what it can achieve.

The highlight of my recovery was when a nurse from critical care visited and exclaimed “Look at you! It’s great to see you looking so well. You did frighten us; we nearly lost you a few times.”

I felt so proud. Considering I wasn’t even expected to survive my recovery is nothing short of a miracle.

For more information: see GAIN www.gaincharity.org.uk






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