The brave little girl in constant agony from Butterfly Syndrome
And the indomitable mum who nurses her round the clock and has raised £4.5million to find a cure
WE WANT you to nominate women for our Inspirational Women of the Year. The awards, in association with L.K. Bennett, will raise funds for Young Minds, the UK’s leading charity committed to improving the wellbeing and mental health of the young. It is one of eight UK charities that Heads Together, a campaign co-ordinated by the Royal Foundation of the Duke and Duchess of Cambridge and Prince Harry, has brought together. Today, TESSA CUNNINGHAM tells the story of another nominee . . .
Sharmila COlliNS dreams that one morning she will be able to run into her daughter’s room, cover her face in kisses and squeeze her tight in a huge bear hug.
instead, every day starts in the cruellest way imaginable, with Sharmila having to inflict pain on her beloved daughter. Such is the agony of simply getting up and dressed that Sohana, 15, has to be fed painkillers to help her cope.
Sohana — a smiley, bubbly girl — suffers from a rare but particularly horrible condition, recessive dystrophic epidermolysis bullosa, or EB for short. it’s a genetic illness in which the skin blisters and peels off at the slightest brush or knock. it leaves sufferers such as Sohana — of whom there are 8,000 in Britain — covered in red raw wounds and huge blisters.
Their skin is so delicate, they are often called ‘butterfly children’. The pretty name is a world away from the excruciating pain of what sufferers endure every single day. The inside of Sohana’s mouth, her oesophagus and her gut are affected, too. Food has to be liquidised and mealtimes are an agonisingly slow process.
‘ Food should be one of life’s pleasures, but Sohana hates mealtimes because it can take her 90 minutes to eat a simple snack,’ explains Sharmila. ‘She has blisters in her mouth which make it extremely hard for her to eat anything solid.
‘Watching her nibble at a piece of toast is heartbreaking. She so wants to enjoy tearing into the bread, but she can’t.’
Sohana’s eyes are prone to developing agonisingly painful blisters, which can leave her temporarily blinded.
‘it’s like Sohana has been flayed or horribly burned — but it never heals, it never goes away,’ says Sharmila, 48, a vet. ‘her skin is so fragile that i have never been able to give her a big squeeze and she has never been able to touch my face. She has to wear gloves all the time to protect her skin.
‘i have to dress her wounds morning and evening. it’s excruciating for her.’
all Sharmila wants is for Sohana to have a normal life, free of pain like her sisters Jacinda, 13, and tenyear-old twins akhaila and Zuleikha, who are all mercifully unaffected by the gene.
instead, Sohana is denied the normal rough and tumble of family life they take for granted.
‘ it breaks my heart to watch the girls running in the garden and see Sohana shuffling behind, every step agony,’ says Sharmila, who lives in North london with her husband, James, 47, a barrister. ‘ When they share a pizza, all Sohana can do is watch. The crust is too hard for her mouth.
‘and she has never felt the sand between her toes or walked barefoot on the grass. When her sisters rush off to play tennis or go swimming, she has to stay at home.
‘as she’s got older it’s become increasingly embarrassing. People stare at her which, of course, she loathes. The first time it happened she was just two. it was in a toy shop and this little girl started pointing at an ugly open wound on her face.
‘We had always told Sohana that she was gorgeous, so it was a horrible shock for her. Yet, like so many children who suffer appalling health problems, she has a fantastically positive spirit which puts us adults to shame.’
While her sisters are a merry and protective band for Sohana, their very presence simply highlights how limited and painful her life is.
‘We were determined to have more children so that Sohana would enjoy as normal a family life as possible,’ says Sharmila. ‘The girls adore each other. Sohana and Jacinda are particularly close. They call each other “dude”. it’s so sweet.
‘But i also feel incredibly guilty because every day her sisters hold up a terrible mirror — showing Sohana all the things she can’t do.’
Worst of all is the ordeal every morning and evening when Sharmila has to gently lubricate her daughter’s eyelids, check and re-dress every one of her wounds and prick the numerous blisters which otherwise will only keep on getting bigger. The process can take up to two hours.
‘There can’t be anything worse for a mother than having to cause your child pain; and i have to do it twice, every single day of my daughter’s life,’ says Sharmila. ‘When Sohana was born, the shock of discovering she had this condition was horrific. She was just a day old when the skin started peeling off her feet and around the little name tag the midwife had put on her ankle.’ Sohana was examined by a dermatologist at Great Ormond Street hospital in london and a biopsy confirmed the worst. a week later, the shellshocked couple were sent home with a booklet on EB and the advice from doctors to wrap their daughter in cotton wool.
‘We were so terrified of hurting her that we did that almost literally,’ recalls Sharmila. ‘When we carried her, it was on a pillow. We sprinkled cornflour in her tiny baby clothes so they wouldn’t rub her. We dreaded the milestones that most parents look forward to.
‘We couldn’t let her crawl because her knees were too fragile. and she was three before i finally trusted her enough to walk without holding my hand to stop her falling.’
But even worse for Sharmila was knowing that there was no treatment or cure. ‘Everything we were told and had read stressed that EB children have very short, painful lives,’ says Sharmila. ‘it was impossibly cruel.’
and it was news this redoubtable mother simply refused to accept.
‘We were warned that Sohana would live in constant pain and almost certainly die in her early 20s. Children with EB are at exceptionally high risk of developing malignant skin cancer,’ says Sharmila.
‘Effectively, it’s a skin problem with
a death sentence attached. I looked at my beautiful little girl and knew I had to fight for her — and for other children like her.’
When Sharmila started to investigate the condition, she discovered there was no government funding for experts hunting for a cure. In fact, just £400,000 a year of private money was being channelled into research.
‘Scientific progress meant there was real hope of safe, effective treatment and possibly a cure — but only if money was poured into research,’ she says. ‘ These children are doomed to such short lives that we had to act quickly.’
The result is a charity, Sohana Research Fund, devoted solely to raising money for research. Launched in 2010, the Fund has already brought in more than £4.5 million through a mixture of charity dinners, concerts and sponsored events. It’s a staggering amount for one woman with zero experience of fundraising.
‘I started in the obvious place — the school playground,’ says Sharmila. ‘We had always tried to make light of Sohana’s condition. So much so that many people assumed she just suffered from severe eczema. And we have always encouraged her to be positive. So, when people see her happy, smiling face they don’t realise the trauma that goes on behind the scenes.
‘however, when I explained what she and other children with eB suffer every day, parents flocked to help.’
It undoubtedly helped that one of the dads at Sohana’s school is actor Damian Lewis, star of homeland and Wolf hall.
he is now a patron of the charity. But it’s Sharmila who has kept up the drive, endlessly coming up with new ways to raise money.
‘I could either sit at home dreaming of winning the Lottery or I could go out and make it happen,’ she says. ‘There was no choice.’
It’s hard to imagine how this extraordinary woman manages to juggle everything. But somehow she does.
her latest idea is to encourage families to invent games to play with water balloons. They then text the word ‘POP’ to 70300 to donate £3 to the Fund.
‘Teachers at one school lined up and let kids waterbomb them for 50p a go,’ says Sharmila. ‘It’s about having fun while raising money.’
The Fund has already paid for a trial in which ten children — including Sohana — were given infusions of donated bone marrow cells to repair the damage to their skin caused by the lack of a protein that holds the skin together. It proved hugely successful.
‘One three-year- old girl’s mum thought the trial finally helped her daughter walk,’ says Sharmila. ‘The treatment made the wounds heal better. For the first time, too, Sohana’s skin began to improve.’
Buoyed by this success, Sharmila is now determined to find a cure. Scientists are working on gene therapy treatments, with the aim of altering sufferers’ genes so that they start working properly, producing the missing protein.
‘We don’t have years to play with,’ says Sharmila. ‘There are thousands of children living every day in agony, their lives shortened because of this condition.
‘With the collective generosity of ordinary people, we can give them a pain-free future.’
Sharmila is far too modest to admit it, but her devotion to her daughter’s cause is transforming the treatment and outlook for thousands of children with eB.
She has proved how a mother’s love can shine a much-needed spotlight on a terrible disease.
‘The future really is looking a lot brighter for patients,’ says Professor John McGrath, head of the genetic skin disease group at King’s College London. ‘ What Sharmila has done is nothing short of amazing.’
Few would disagree.