It began with a trip to give blood – and ended with a ‘death sentence’.
As a fit and active 50-year-old, Esther Davidson loved long bike rides and walking in the hills. Thankful for her good health, blood donation was one way to show her gratitude.
But for the mother-of-two from Edinburgh, life changed dramatically during one routine session, when the standard safety tests flagged up a potential problem with her liver.
Further investigations led to a devastating diagnosis: a liver disease called primary biliary cholangitis.
‘I’d never heard of it,’ says Esther, now 67 and a retired special needs teacher. ‘And I found it really hard to believe there was anything wrong with me because I felt so fit and healthy.
‘But suddenly I was told I could no longer donate blood and was referred to a liver specialist who warned – incorrectly, as it turned out – that I would live only another five to ten years without a transplant.
Esther Davidson, of Edinburgh, was 50 when she was given a devastating diagnosis following a routine blood donation. She was told she had an autoimmune liver disease called primary biliary cholangitis. ‘I’d never heard of it,’ says Esther, now 67 and a retired special needs teacher (pictured with her beloved cockapoo Suzy)
‘In a matter of days, I’d gone from feeling in perfect health to being given a death sentence.’
Esther soon discovered that there were thousands more people like her across the UK suffering with primary biliary cholangitis.
Today, having redoubled her efforts to stay healthy, Esther is still well – having staved off that transplant op until November 2018.
However, many patients are not so fortunate. Some live for as little as five or six years, especially if diagnosis is late and the liver is already damaged.
Primary biliary cholangitis is just one of a number of autoimmune liver diseases that strike people in the prime of life with little warning.
While each disease is subtly different, all are linked by the fact that, for reasons that are not yet known, the immune system turns rogue and attacks the body’s organs. And experts fear the number of people affected is on the rise.
Last week, Robert Colvile wrote movingly in The Mail on Sunday about losing his wife Andrea, the mother to their two young sons, to autoimmune hepatitis. She was just 40.
Launching our Christmas campaign to help better fund research into these cruel illnesses, Robert, who is director of the think-tank the Centre for Policy Studies, says: ‘We desperately need new treatments. Nothing has really changed since the 1950s, even as other areas of medicine have surged ahead. Andrea should still be here to watch over our two boys, Edward and Alexander, at Christmas.
Last week, Robert Colvile wrote movingly in The Mail on Sunday about losing his wife Andrea, the mother to their two young sons, to autoimmune hepatitis. She was just 40. (He is pictured with his son Edward, and late wife)
‘The greatest gift I can give her memory is to ask people to support this research so there will be fewer families, like us, with an empty seat at the Christmas table.’
An estimated 22,000 Britons currently have primary biliary cholangitis and a further 10,000 have autoimmune hepatitis.
Thousands more suffer similar but even rarer diseases.
The cash target of £200,000 will be used to fund at least one full-time scientist to investigate the exact causes of these diseases, in the hope of finding lifesaving new therapies.
Illnesses that slip through the net
In the minds of many people, liver disease is synonymous with alcohol and obesity.
Yet in those with autoimmune liver diseases, problems emerge when the immune system – which is meant to protect the body against illness – goes haywire and attacks the organ. This leads to irreversible scarring, or cirrhosis, which eventually causes the liver to fail.
The illnesses can go hand in hand with other conditions that involve the immune system, such as type 1 diabetes and multiple sclerosis. But many sufferers are otherwise in perfect health.
Worse still, they are wrongly labelled as problem drinkers.
‘I used to enjoy the occasional tipple but I barely drank,’ says Esther. ‘Yet I’d see on people’s faces that’s what they thought was to blame for my illness.
In the minds of many people, liver disease is synonymous with alcohol and obesity. Yet in those with autoimmune liver diseases, problems emerge when the immune system – which is meant to protect the body against illness – goes haywire and attacks the organ. This leads to irreversible scarring, or cirrhosis, which eventually causes the liver to fail. (File image of a diseased liver)
‘A nurse at hospital even asked me how much I drank when she heard I had a liver disease. She should have known better.’
By the time most sufferers are diagnosed, about a third already have significant cirrhosis. In the early stages, the only symptom many patients experience is fatigue – and patients often don’t seek medical help. If they do visit a doctor, their symptoms may be dismissed as simply a virus.
That was the case when mother-of-two Monira Khanom fell ill in early 2015.
Her GP advised her to rest, but when her nausea and vomiting continued for several weeks, Monira became gravely concerned. ‘This time the GP said it was a stomach bug and gave me antibiotics,’ says the 39-year-old from Telford. ‘But nothing worked. I couldn’t keep any food down and was losing weight rapidly.’
After three months of trips back and forth to the GP and, eventually, a liver specialist, Monira was given devastating news. She had autoimmune hepatitis and desperately needed a transplant.
‘I was told I was being put on the super-urgent waiting list for a donor liver,’ says Monira.
‘In other words, I had only a few hours to live without one.
‘My life was at risk of being cut short and I wouldn’t be around to care for my children.’
Luckily, a suitable donor was found and Monira made a good recovery. But doctors still have no idea what triggered her condition in the first place.
Without a transplant, treatment includes immune-system and inflammation-dampening steroids. But these must be taken for life, and have side effects including diarrhoea, abdominal pain and skin reactions. In some patients, the drug can even make the liver cirrhosis worse.
‘As my liver started to shut down, it affected my blood sugar levels so much that I would get really confused,’ says Esther.
‘Once, someone found me just sitting on a path not knowing where I was or what time it was. I also suffered terrible cramps in my arms and legs. I used to love baking but could no longer hold a mixing spoon long enough to make a cake.’
The liver is actually one of the most versatile organs in the body, capable of regenerating itself when injured or damaged. But the scarring caused by cirrhosis eventually overpowers its ability to heal.
At that point, the liver can spiral into rapid failure, often leaving patients with just days or weeks to live.
The only option at that point is a transplant, which cures the condition. But the number of patients needing livers, due to all types of liver disease, is more than double the number available.
In the year to March 2019, official figures show there were 432 sick patients in the UK desperate for a transplant – yet fewer than 200 operations a year are carried out. And the waiting list is growing – it rose by 20 per cent last year.
Even those lucky enough to get one must wait an average of five months, during which time their health can deteriorate rapidly.
The chances of long-term survival are slim in those for whom there is no matching donor.
Experts warn that both main types of autoimmune liver disease, primary biliary cholangitis and autoimmune hepatitis, are on the increase. No one is sure why, although common viral infections and environmental toxins have been suggested as potential triggers.
Women are far more at risk, due to the differences in the immune systems between the sexes.
Professor David Jones, professor of liver immunology at Newcastle University, says: ‘Female immune systems switch off when they are pregnant to make sure they don’t attack the growing foetus, which is essentially a foreign body. This may mean women are more susceptible to certain immune system malfunctions.’
Itching drives some to suicide
Jaundice – the yellowing of the eyes and skin – is a main symptom of liver disease. But in many patients that shows up only when the condition is fairly advanced.
Before that comes a symptom that sounds innocuous but can in fact be so crippling it’s been known to drive people to suicide: itching.
Scientists think it happens because the liver is unable to process bile and, as a result, bile salts accumulate in the skin and other organs, causing extreme irritation.
‘Some don’t get the itch at all,’ says Colette Thain, chief executive of the PBC Foundation, a charity she helped set up in the 1990s to support patients with primary biliary cholangitis.
‘But others have taken their own lives because it has been so bad. It’s every part of your body – not just from head to toe but even the inside of your eyes.’
I used to enjoy the occasional tipple but I barely drank… yet I’d see on people’s faces that’s what they thought was to blame for my illness.
For several decades, there have been few major developments in the treatment of autoimmune liver diseases. But a new stem-cell trial at Birmingham University could lead to a radical new treatment.
The therapy involves injecting a certain type of stem cell – called mesenchymal stromal cells and taken from babies’ umbilical cords – into patients’ bloodstreams to see if they can reduce inflammation, restore normal liver function and eradicate the need for a transplant.
Stem cells are the building blocks of human tissue with the capacity to turn into any type of specialist cell, such as skin, muscle or nerve.
These particular type of cells in the ground-breaking trial are known to be especially good at repairing damaged tissue, stimulating the growth of healthy new cells and easing inflammation.
Scientists hope this could make them ideal for tackling severe damage to the liver.
It is early days – the trial is recruiting just 56 UK patients with primary biliary cholangitis and autoimmune hepatitis – but researchers have high hopes that it could bring a breakthrough.
Esther survived… with the help of a dog
After her initial shock at her grim prognosis, Esther took matters into her own hands.
Her illness had been caught early and she was determined to avoid a liver transplant for as long as possible – and defy the doctor who gave her just a few years to live.
She says: ‘I kept working for as long as I could, regularly went to the gym and ate healthily.
‘I was determined to stay as well as possible for as long as possible, rather than just rely on having a transplant.’
And it worked – for 16 years.
But in November 2018, Esther finally underwent a transplant after her liver began to fail for good. Within months, she was back out on her walks – this time accompanied by a new companion, her beloved black cockapoo Suzy.
‘I feel fantastic and we walk miles every day,’ she says.
‘The standard of care I received all the way through from the NHS has been outstanding. I’ve been given another chance at life.’
You can donate to the Medical Research Foundation Fund here: https://www.justgiving.com/fundraising/andrea-colvile