‘My Christmas miracle of life’
One woman’s story of courage and triumph against adversity
No one does Christmas quite like my mum, Debby. She looks forward to it all year round, buying pretty snow globes and baubles on her travels to decorate her picturesque home in Beaconsfield, Buckinghamshire, which she has named Christmas Cottage.
Festooned with mistletoe and dotted with flickering candles, she has a way of transforming the everyday into magic.
But last Christmas, even Mum’s efforts couldn’t rescue me from the despair that had gripped me. Two days before, I had been discharged from St Bartholomew’s Hospital in London, where I was treated for pneumonia. As I watched my family excitedly opening their presents, I was overcome with a profound sadness at the realisation that it may well be my last Christmas.
I have a genetic, degenerative disease called cystic fibrosis, which causes the body to produce a thick mucus that affects the lungs and digestive system. Those who have it eventually die from lung collapse or liver failure and, back when I was growing up, the average life expectancy was just 40, although thankfully it is now higher. Last Christmas, I was about to turn 40 and was gravely ill. Confined to a wheelchair and with very low lung function, the months ahead felt like a ticking time bomb.
Usually, I cook the Christmas dinner, but last year, I was too frail to even do that, so I watched as my mum and sister took over. That evening, Mum and I snuggled up on the sofa, watched Home Alone and tried to press pause on my life.
REACHING MILESTONES
Growing up, Mum spoke candidly to me about my condition and explained as best as she could that I was ill and would always need to go to hospital. Mum is a resourceful, tough woman. She’s had to be.
My father left my mum when she was pregnant with me. She raised me on her own until she met a new partner, when I was five, and went on to have my siblings, Sebastian, now 35, Maddie, 32, and Daisy, 29.
‘I know I’m going to die young,’ I told Mum when I was nine.
Having to face their own mortality is something no child should be forced to do. When I was 11, I wrote my first will, saying I wished to be cremated, I wanted to be wearing my favourite trainers and that I absolutely had to have my treasured blanket with me.
As I grew up, my wishes changed, but the sharpened feeling of my own mortality was ever-present.
When I reached 15, the birthday that doctors had told Mum I would never see, I blew out the candles on my cake and we celebrated.
There is nothing like knowing you are likely to die young to make you devour as many experiences as you can squeeze from life – and
I did; travelling, partying and getting married at 18. The marriage soon fell apart, but Mum was always on hand with love and hugs when I needed them.
It frustrated me when I saw people obsessing over money or sweating over the small stuff. My philosophy was: enjoy life and all its incredible opportunities. If you’re not happy with something, change it.
It was this attitude that made me celebrate my 30th with a ‘double your sell-by date’ birthday party. I dressed up, had all my friends round and, at the end of the night, Mum and I danced in each other’s arms to Bette Midler’s Wind Beneath My Wings.
But life was catching up with me.
I was repeatedly admitted to hospital with lung infections such as pneumonia.
Living independently in a flat near Mum, I had to inject myself with a cocktail of medication and
‘Mum was always on hand with love and hugs’
undergo daily physiotherapy to clear my lungs, a painstaking routine which could take up to nine hours. St Barts Hospital became like my second home. While most people my age were carving out careers and beginning families, most of my life was spent confined to a hospital bed.
I’d suffered two miscarriages in the past, so as well as the fear of passing on the cystic fibrosis gene, I knew in all likelihood that I had fertility issues, too.
I never allowed myself to wallow in ‘why me?’ and tried to adopt Mum’s pragmatic attitude, but there were times when darkness slipped between the cracks.
One time in hospital, I remember seeing cystic fibrosis sufferers in the final stages of their battle with the disease, covered in tubes, too weak to be fed or hallucinating from too much morphine.
DRUG BREAKTHROUGH
The enormity of what I was facing swallowed me whole. I had no wish to die confined to a hospital bed and in prolonged pain. At 33, I said to Mum, ‘If I have no quality of life when I turn 40, I would like to go to a Dignitas clinic and end my life. Will you help me?’
Four simple words with seemingly endless moral, ethical and legal implications. Typically, Mum wasn’t shocked. She knew my request wasn’t the ramblings of an over-emotional or frightened young mind. It was a calm, measured and thought-out question by someone whose daily life has been dominated by managing a dreadful disease.
‘Yes,’ she replied. ‘If that time comes, I will help.’
She told me that she would do anything within her power to help end my suffering if it had become intolerable. To me, it was simple.
Why could I not choose a peaceful death over a painful, undignified one?
We didn’t dwell on that conversation further, but her vow to help me bolstered me through the turbulent decade ahead.
As my health became frailer, it seemed the cruellest of paradoxes that I continued to look so healthy. I saw the raised eyebrows when I parked my car, complete with blue badge, in the disabled bay. I wanted to scream at the injustice of a disease that slowly robs sufferers of the ability to breathe and yet leaves them looking so vital.
By last Christmas, my lung function was down to 30% and I needed a wheelchair to get about. I celebrated my 40th in a subdued way in January this year, knowing I was living on borrowed time.
Over the years, there has often been talk of new drug treatments, so I’ve tried not to get my hopes up.
And when my consultant told me they had put my name forward to be included on a new drugs treatment called Trikafta, I agreed, then promptly put it to the back of my mind.
Besides, I had other things to worry about. When Covid-19 swept the country, I resigned myself to the worst. I was already in and out of hospital with plummeting lung function, so I told Mum: ‘If I contract Covid-19, I don’t want to go on a ventilator and cling to life.’ As always, she accepted my wishes.
Then, two weeks into lockdown, I got the news – I was one of 120 patients granted early access to the drug by the manufacturer, Vertex, under its ‘compassionate-use’ scheme. The drug had been licensed by the NHS and was to be released in the UK under the name
Kaftrio. The medicine had already been dubbed the ‘holy grail’ by both patients and doctors, and so it was with great trepidation, and huge amounts of excitement, that my family gathered in my back garden one sunny Saturday morning to see me take the pill. The results were almost instantaneous.
I took it at 10.30am and by 4.30pm the cough I have lived with all my life had eased. Within two weeks, my lung function had shot up from 30 to 60%, I gained 10lbs, and I no longer felt breathless and exhausted. I also noticed the salty skin that characterises cystic fibrosis sufferers had disappeared.
Mum held me in her arms, almost overwhelmed with emotion. I felt, well, like I still feel now, almost unable to articulate the joy. Four decades of pain and suffering lifted from my shoulders.
I continued to grow in strength, and the effects weren’t just physical.
‘You’re laughing all the time,’ Mum told me. She was right. All my life, I’d suppressed laughter as it took up too much lung function, now I was laughing at everything.
As the lockdown eased, I took my first tentative steps into a new world. My biggest ambition had always been to walk, surf and swim, and this summer, I did just that. In July, I went on a road trip with a friend to Croyde, Devon, and I did things that would have been simply unimaginable four months before.
I walked up a big hill, swam in bracing cold water, surfed and laughed round a campfire. It was incredible.
The trip was followed by others to the south west coast, where the beauty of the countryside was breathtaking. I realised how much life was there for the taking and the sense of adventure I experienced as I took to the road was dizzying.
A NEW LEASE OF LIFE
Today, nine months on from swallowing that very first pill, things have plateaued. It’s not the golden bullet that means I’m now robustly healthy, but Trikafta has given me the ability to fight back and, more importantly, plan for a future that I never expected to have.
In the new year, I’d like to apply to do a degree to become a doula, to provide support for women during pregnancy and birth. Coming so close to death has reaffirmed to me how important birth is. Also, knowing I may never have my own children has given me a deep respect for the importance of childbirth. I remain acutely aware of the fragility of life and how important it is to live every day as if might be your last. What better way to spend your day than helping to bring babies into the world?
Before then, though, there is Christmas to enjoy, and what a difference 12 months makes! I entered 2020 preparing to die, I end it with a new lease of life.
This Christmas, I will spend it with my beautiful family at Mum’s house, cooking Christmas lunch with all the trimmings, toasting our health, grateful to be at the centre of a loving family. Mum is planning on decorating not one, but three trees! It doesn’t matter what presents I unwrap because I’ve already been given the best gift of all: my health. As the country prepares itself for an uncertain festive season, I hope people will draw strength and inspiration from my story because it proves that whatever dark days lie ahead, there will always be hope.