A 10-month-old baby who has a rare condition that turns the body ‘into stone’ could be given a second chance but her parents are in a race against time.
Lexi Robins from Hemel Hempstead was diagnosed with Fibrodysplasia Ossificans Progressiva (FOP) – a genetic condition that gradually replaces connective tissues, tendons, ligaments and muscles with extra skeletal bone.
It’s thought to affect just one in two million people, although a recent study by the International FOP Association, published in August 2021, suggests the prevalence could be higher at one in one million.
Over time, a person with FOP essentially becomes a human statue – described as a healthy mind locked inside a frozen body.
Once the bone has developed and movement is lost, there is currently no way to reverse it.
Lex’s mum told Herts Live she hopes a parliament debate on the disease, due to take place next Monday, could be the “start of a new journey”.
No funding is currently available on the NHS and, although there are currently a limited number of individuals working at Oxford University in search of a cure, this costs around £125,000 a year.
It’s one of the most debilitating conditions affecting children in their early years and, on Monday at 4.30pm, a number of MPs will debate FOP in Parliament Hall.
There are three main calls for action during the debate: to increase research funding, to transform standards of patient care and to increase awareness and transform diagnosis.
Alex Robins, Lexi’s mum, launched a petition to secure Monday’s debate and gathered over 100,000 signatures in just eight days.
She says the support she’s received from friends, family and complete strangers, as well as famous faces like John Terry, Jacqueline Jossa and Patrick Bamford, has been overwhelming and hopes the MP debate will be a step in the right direction.
People from across the country have also been writing to their own MPs asking them to take part in the debate.
“I think it’s going to be the start of a new journey,” said Alex, 29, who is also mum to three-year-old Ronnie.
“I think it’ll go into further discussions, and how can you get higher up than MPs in the House of Commons? The fact we’re there, talking to the right people, we’ve got heads turning.”
Most people with FOP are immobile by the age of 30 with an average life expectancy of 40, according to research.
Lexi’s story has been shared across the globe and reached countries as far flung as Brazil, Australia and India.
As she gets older, there are more and more precautions that Alex and her husband Dave, 39, need to take to keep her safe.
“Lexi is extremely happy,” added Alex, “she’s extremely laughable and she’s at the stage now where she just wants to get up and play which obviously to us is the most worrying.
“She’s trying to crawl and walk and it’s great to see her develop but at the same time that’s going to come with a whole other host of worries for us.
“When she bangs herself, when she bangs a muscle, every single time there’s a chance that the FOP will flare up, especially if it’s on the joints.
“When it flares up, it calcifies into bone and will paralyse that joint. She could literally be walking all over, hit her knee and, a couple of days later, wake up and that leg will never work again. It’ll be paralysed.
“That’s the pressure and the worry we now have.”
At present, fundraising money for research is generated through parents of children with FOP, or those with FOP themselves.
Alex and Dave, with the help of the wider community, have so far raised over £100,000 for FOP Friends, a charity founded in 2012 by Chris Bedford-Gay whose son Oliver, 10, was born with the condition.
Alex’s main aim is getting better treatment – and ultimately a cure – before Lexi’s body begins turning to stone.
Lexi’s parents are in a real race against time.
“Everyone tells me it’s a marathon, not a sprint – but for me it’s a sprint,” said Alex.
“The safer we keep Lexi and the sooner she gets a cure or some treatment to reduce the bone growth, then that means she can have a lot more of a normal life than what it looks like at the moment.”
FOP Friends has over 50 patients with FOP known to them, and 14 of these are children under 18. There could be as many as 70 people living with it in the UK.
The short-term aim of the debate is to try and secure regular corporate funding while the long-term strategy is to receive NHS backing, and better funding will hopefully have a snowball effect.
If researchers crack FOP it could help increase knowledge of more common and related conditions, and Alex hopes this is what Monday’s debate will help achieve.
“It’s the tip of the iceberg,” said Alex.
“There are thousands of people that are suffering with a rare disease, this [FOP] is unfortunately one of the most severe out there.
“If we find a cure for this, we also help other conditions like osteoporosis and rare childhood cancers.
“If you get involved in this, you’re actually going to help so much more than just people with FOP.”
Lexi’s local Conservative MP, Sir Mike Penning, said the Parliamentary Petitions Committee has responded in record time and hopes the debate will raise awareness of FOP.
It comes as over 50 per cent of those affected in the UK have received an initial incorrect diagnosis, which can often lead to harmful treatments that can hasten the progression of the disease, thus causing further unnecessary harm.
“As Alex and Dave’s local MP I was moved by the plight of Lexi’s story and inspired by the work of FOP Friends to fund vital research to find a cure,” he said.
“Research into FOP could have wider implications for common conditions such as osteoporosis, heart disease, chronic anaemia and joint replacement, which are a huge cost to the NHS”.
Alex and Dave both have full time jobs which, alongside looking after their two children and fighting for an FOP cure, can be exhausting and overwhelming.
The support from their friends, family and complete strangers has helped them pull through though, showing them hope under some of the most trying of circumstances.
“It’s actually been overwhelming,” added Alex, “especially when you’re having a bad day, because we do get them.
“Last week, I had a really bad week and it comes in waves. It just takes one message from someone, and most of the time it’s someone that you don’t know, and they don’t even know you’re having a bad day.
“It really does make a difference so we cannot thank people enough and we really do appreciate it.
“It doesn’t fade, and people really do follow Lexi’s journey and hopefully it’ll be a positive journey and we’ll cure something that didn’t have a cure before.”
Chris Bedford-Gay, founder of FOP Friends, added: “FOP Friends was set up as a charity following the diagnosis of our own child, Oliver. We never dreamt we would be debating in Parliament ways to change the lives of our small but strong FOP community and if we are lucky, the lives of many others living with rare diseases.
“If we can instigate real, meaningful change and support at governmental level it would be fantastic. Many might ask why rare?
“There are 3.5m people living with a rare disease in the UK. Alone we are rare, together we are strong”.
The parliamentary debate will be held on Monday, December 6 at 4.30pm. Find more information here.