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langerhans cell histiocytosis
Emma Royle's baby boy, Casper, was already being treated for a vicious looking rash on his groin, which came up when he caught chicken pox from his sister, Nora, four, when she and his dad, Nick Southall, 40, rushed him to the hospital, as he was puking up “fluorescent yellow" vomit.
Luckily, the consultant on duty at the Royal Devon and Exeter Hospital was a pediatric oncologist, who recognized the tell-tale signs of Langerhans Cell Histiocytosis (LCH) which develops in about 50 children a year in the U.K., according to the Children's Cancer and Leukemia Group.
Student recruitment manager Emma, 37, of Exeter, Devon, who was told the condition has a high survival rate, said: “It was July 19, 2018, and I'd called the NHS 111 helpline, as Casper had been vomiting for 12 hours straight and I was advised to take him to hospital.
“The consultant took one look at his rash and said, 'I hope I'm wrong, but I think I know what this is.'
“It all started to fall apart from there really, but in a sense, if there hadn't been an oncology specialist working that night, who knows when Casper would have got the diagnosis."
Following a home birth in the family kitchen on February 5, 2018, 10lb 2oz Casper had a great start to life, until the May when he caught chicken pox from his sister and developed an angry rash as well as the usual spots.
“I took him to the doctors for his chicken pox because of his age," said Emma. “When he saw the rash, he said to come back if it didn't clear up in a week alongside the spots."
When the rash refused to budge, the GP said it was eczema and prescribed some steroid cream to help it clear up.
But three weeks later, at the start of July 2018, when the rash spread to his neck and leg, Emma went back to the doctor, who thought it had become infected because Casper was wearing a nappy and prescribed antibiotics.
“I wasn't convinced by the diagnosis," she continued. “Nora has had very light eczema and it seemed completely different to her's, but when a doctor tells you something you take their word for it."
Heading off on their first family holiday to Brittany in France the next day, doctors advised Emma that Casper would be well enough to go.
But just a day in, he “screamed the house down," as his rash became more and more inflamed.
“We tried putting him in the bath to ease the pain, but he was uncontrollable," she said. “You could tell he was in agony, so we took him to the local hospital.
“We were in there for three days, as doctors wanted to ensure he wasn't dehydrated, but the general consensus was that he was fine, although one nurse did suggest we weren't bathing him enough, which was the last thing we needed to be told."
But just a day after they returned home from holiday Casper started vomiting non-stop and Emma and Nick, a campaign manager, took him back to the E.R., leading to his LCH diagnosis.
First doctors took a biopsy from the affected area around his groin to test for LCH – which has no known cause, but sufferers of which have too many histiocyte cells which normally protect the skin, but sometimes move around the body causing damage – and he was transferred to Bristol Royal Hospital for Children, awaiting the results.
“It didn't really click how poorly he was, until we were being transferred for more specialist care," Emma recalled. “Up until then I'd been dazed, but sitting in the back of the ambulance going to Bristol, it started to dawn on me."
Four days after the biopsy it was confirmed that Casper, who was in the high dependency unit, had multi-system LCH, meaning the condition – which some experts describe as 'cancer' while others believe it is not and simply shares some characteristics – affects more than one part of the body.
Unlike most cancers, it can spontaneously resolve itself and has a survival rate of 90 percent, although multi-system LCH can and often does return after treatment.
With Casper's diagnosis confirmed, in August 2018 he began a grueling 12-week course of chemotherapy once a week, to kill the rogue cells which were causing potentially life-threatening lesions in his skin, bone, gut and bone marrow.
“Doctors told us that although there were no tumors, the lesions were effectively zapping Casper of healthy cells that usually help the immune system to function properly," said Emma.
“At first the chemo seemed to be working, as his rash cleared up and he started to seem more like a normal baby, but just when we thought we were over the hill, Casper started deteriorating again."
When his three-month treatment program finished in November 2018, with no signs of improvement, Casper's family and doctors were left wondering where to turn to next.
“We had a choice, we could try a more aggressive round of chemo, or we could try and get a place on a clinical trial – a completely different method of treatment," Emma explained.
Worried that Casper might not have the fight in him for another round of chemotherapy, with the help of their consultant, he was given a clinical trial drug Dabrafenib, a cancer growth blocker, which stops the signals cancer cells use to divide and grow. It also inhibits the production of the BRAF gene, which can mutate and is thought to be largely responsible for LCH.
“The clinical trial requires children to be over one," said Emma. “Casper was only eight months old, so he wasn't eligible, but the hospital was brilliant.
She continued: “The medics approached the drug company, which guaranteed us a year's supply, on compassionate grounds."
Within a week of taking the drugs, which Casper has twice a day, the rash had completely cleared, and blood tests showed the mutated cells were no longer active.
“By Christmas you would never have known anything had been wrong with him, it really was mind-boggling how quickly he recovered," said Emma.
“It was like we finally had our baby boy back and could be a proper family again, just in time for our first Christmas together."
While the family were massively relieved to see Casper so well, Emma's maternity leave was due to finish in January 2019, and she felt his illness had robbed them of the precious time new moms usually spend bonding with their babies.
She said: “It felt like we'd lost our first year with Casper, with endless trips to doctors, hospitals and chemotherapy sessions."
She continued: “Maternity leave should be a precious time for a mother and child to bond, instead we had ours ripped away from us."
Fortunately, Emma had taken out an insurance policy when she gave birth, covering children with serious illness and, when she looked into it, she was entitled to a £20,000 (~$24,000) payout, meaning she could take another eight months off to spend with Casper.
She said: “I'd taken out Vitality cover, while updating my life insurance, after I gave birth to Casper."
She continued: “It only cost £2 more a month and, if I'm honest, at the time I did it for the free cinema tickets!
“But the payout was fantastic and meant I could finally spend some quality time with my son."
Sadly, Casper is not completely out of the woods yet, as the LCH has a habit of returning.
Emma explained: “It's likely it will come back and we can't expect the drugs company to provide free medication indefinitely.
“When it stops, we don't know how quickly the LCH could return and, even if Casper's lucky enough to be on the clinical trial for years, we don't know what side effects it might have later down the line.
“Not having a time frame is hard and, while we've steered clear of any prognosis, the fact is nobody knows what affect this will have on Casper's health as he grows older."
She continued: “We're just grateful for being allowed to make memories and start living like a normal family again – for however long that might be."
To find out more about Vitality life insurance, visit the website here: www.vitality.co.uk/life-insurance/