Meet the Inspirational Family Fighting for a Batten Disease Cure
We speak to The Nicole and Jessica Rich Foundation, who Living North first spoke to in 2017
In July, Gail and Matthew Rich held their annual Sweetheart Ball to raise money and awareness for the little-known Batten Disease, which both of their daughters, Nicole and Jessica, were diagnosed with in 2016. What made this one so important, though, was that it was held in memory of Nicole, who tragically passed away last October.
‘Everything was perfect’ says Gail. ‘The theme was black and gold, it was like a movie set. We had the room draped and there was gold twinkles everywhere because we wanted it to be like stars, like Nicole was in the stars.’
‘The dance centre, where Nicole went when she was able and Jessica goes now, did a dance for us. [They] portrayed Nicole and Jessica’s relationship and their bond. There wasn’t a dry eye in the house. Nicole’s picture was at the back and they did it to I See the Light from Tangled as that was one of Nicole’s favourite songs.’
Nicole’s passing, at just 11 years old, was unexpected. ’We’re so happy with what we got to do with her. She was blessed by the Pope in Rome, she climbed the Spanish Steps, swam with dolphins, she went up the Burj Khalifa in Dubai. She did more than a lot of people do. Even before she passed, we went to the Mediterranean Sea in Turkey and she was swimming with us. That’s what we wanted out of the holiday and we achieved it. We had beautiful memories with her. We wanted many more, but we just have to be grateful that we got to do so many things.
‘Nicole had a wonderful quality of life, nothing stopped her,’ Gail continues. 'She melted everyone’s hearts and everyone loved her instantly, they all just adored her. She had this beautiful aura about her.’
A couple of years ago, the foundation raised £12,000 for the Great North Children’s Hospital, which was used to make Ward 2B more sensory friendly and to provide activities for children with complex needs. The Sweetheart Sensory Trolley in particular has already been a huge benefit to children who are going through treatment.
‘We feel so proud, but it was really bittersweet because we bought it to benefit Nicole but unfortunately she never got to see it. Jessica’s seen it and used it and it just feels lovely that other children are benefitting and that’ll continue forever, and it’s something that Nicole’s done and is part of as she was the inspiration behind it.’
The foundation also worked with The Blaydon Races, which falls on International Batten Disease Awareness Day. ’They put a big announcement out, we got leaflets in all the packs, so four and a half thousand runners all got a leaflet on Batten Disease and our foundation. We’re looking at next year already so it might end up being an annual thing.’
Gail has previously worked with the BDFA through their Peer Befriending scheme, in which she talks to families newly diagnosed with Battens – she plans to continue this once she feels ready to support others again.
‘It is the most horrendous condition and it impacts every single possible part of your life you could think of. I just think I’ve been where they are, and I know how it feels to think that that’s your world ended. It’s nice that something positive can come out of it because I can say to them “look, it doesn’t feel like it but you will be okay. We’re all in it together.”’
As for Jessica, she’s doing really well, despite the unimaginable difficulty of losing her sister. ‘We’re all adjusting to life without Nicole and she’s struggled but she’s been incredibly brave. She’s had to go back into hospital by herself when she’d normally have Nicole and she’d be looking after [her]. It was just heartbreaking, I can’t even put it into words.’
Jessica turned nine in September, and was the youngest person ever to receive the brain surgery that allows her the Battens treatment of a fortnightly four-hour brain infusion of synthetic enzymes. She can ride a scooter, comprehend complex words and is learning Makaton signs – all of which is unheard of for a child of her age with Battens. As a result, she is held up around the world as a shining example for the potential of the treatment.
‘Normally, all Batten Disease ever was was a decline, “what have they lost” or “what can’t they do?” There’s never been: “what are they attaining?”, “what can they do now?” She really is remarkable.
‘We don’t know what the future holds, it’s unknown as she’s the first one paving the way. But my goodness, we’ve so much hope in our hearts that she’ll carry on being as amazing as always.’
Three years ago, the foundation managed to get the treatment moved to Newcastle from Great Ormond Street which Gail describes as ‘one of their proudest moments’. The treatment is currently coming to the end of a five-year access agreement; their next aim is getting it approved by the NHS for long-term use, as well as fighting to change the system in which rare disease medicines are approved. To do so, they’re in the process of working with local MPs and Mayor Kim McGuinness and follow the motto of being ‘pleasantly persistent’.
‘We’re taking it one step at a time. The first thing is to get the treatment over the line and get it approved so we know Jessica’s safe. The next thing is to challenge how the system works, because it doesn’t work, but we have to choose our battles.
‘Everyone knows the treatment works, it’s just a case of money which is just awful. Without the treatment, if they stopped Jessica’s treatment, she would literally deteriorate and die. We’ll never stop fighting until there is a cure, not just for Jessica but for all children.’
