When Monnay Johnson was two-years-old she could walk, talk and play. Her mother Kelly Johnson couldn’t ever have imagined that soon she would start to forget how to do all those things and would need a wheelchair to get around. Monnay suffers from Batten disease, a rare neurodegenerative condition, which causes children to experience dementia. Children eventually lose the ability to walk and speak, experience seizures and develop vision loss. Most children with Monnay’s form of the disease don’t live past their early teenage years. Ms Johnson, a single parent from Hertfordshire, doesn’t know how much time she has left with her daughter.
Monnay has now begun a pioneering but experimental treatment in the US in the hope it will slow down the progression of her illness.
Ms Johnson, 27, told i: “Monnay’s symptoms came out of the blue. I had a healthy pregnancy, she was born healthy and reached all her milestones. “But then when she was four I noticed she was walking funny. She could no longer understand the difference between me asking her to put something up somewhere or down somewhere. “I was pregnant at the time with my youngest daughter and I wondered if Monnay was just acting out at first. “Now she falls a lot and is in a wheelchair. It’s heartbreaking to see her deteriorate but she is still such a happy child. I pray that the treatment will slow it down so that she stays that happy child.”
When she first noticed Monnay’s symptoms in October 2016, Ms Johnson took her to her GP who referred her to a child development centre. There she was diagnosed with dyspraxia, a developmental disorder of the brain in childhood causing difficulty in coordination and movement. Then in February last year, Monnay had an epileptic-like seizure, followed by a further ten over the next week. “She has different types of seizures, where she will black out for a while, or lose muscle function or just zone out,” explained Ms Johnson.
After being referred to Great Ormond Street Children’s Hospital, specialists delivered a devastating diagnosis in May. “They knew it was something neurological and after repeat EEGs to record Monnay’s brain activity and MRI scans they told me she has Batten disease, variant CLN6, which makes her just one of two children in the country with that type,” said Ms Johnson. “It’s very hard for them to tell me too much about her prognosis because it’s so rare, but they told me to expect a rapid decline.” There are 14 different forms of the disease, according to the Batten Disease Support and Research Association. There is no cure for any type and therefore doctors focus on managing symptoms. Monnay has hydrotherapy and physiotherapy.
Ms Johnson was told gene replacement therapy could help her daughter. “We were told she may be eligible to take part in a clinical trial in the states, but we didn’t know for many months if she could get on it. Then in February this year we flew to America for the screening and thankfully she passed. She had to still be able to walk so far and talk for it to be worthwhile them treating her.” ‘She doesn’t seem to have deteriorated further so far. But it’s early days.’ Monnay was given the treatment and Ms Johnson says she has responded to it. “It’s hard to tell – the damage already done is done,” she said. “She doesn’t seem to have deteriorated further so far. But it’s early days.” Monnay still has regular muscle spasms and seizures but these are controlled with medication. Despite her illness, her mother says she is a very happy, smiley little girl.
“She’s still such a joyful child who loves playing with her younger sister Nariyah, as best she can, and she still has a big smile on her face when she’s watching Peppa Pig or eating chocolate cake,” added Ms Johnson. Monnay’s godmother Sereena Morris has launched a GoFundMe page to help Ms Johnson with the costs of flights to the US and accommodation for the checkups she needs every few months for the next two years. “We are lucky that the cost of her care has been covered by the trial but I’m a single mother and I’ve had to give up my carer job to look after Monnay,” said Ms Johnson. “I’m very grateful to The Charlotte and Gwenyth Gray Foundation to Cure Batten Disease for their financial support.”