TWO youngsters from Portsmouth have appeared in a music video campaigning for new medicine to help them in their fight against cystic fibrosis.
Emma Franks, eight, and Ivy Weir, five, are some of the stars of a music video released by cystic fibrosis campaigners hoping to get the Orkambi drug on the NHS.
The song, Breathe With Me, is performed by indie singer/songwriter Betsa and highlights the daily battle of people with cystic fibrosis.
Its release comes ahead of a crucial meeting next week which could see progress made on making the Orkambi drug, manufactured by US-based Vertex, available to NHS patients.
Ivy’s mum Gemma, from Paulsgrove, says that the music video is a brilliant way of raising awareness of what people with cystic fibrosis are going through.
She explained: ‘I think the song is great – it’s well-written and should help people to understand what it is like to live with cystic fibrosis.
‘Ivy absolutely loves it, she’s got a little cameo in the music video; my husband and I think it’s lovely to see out little girl involved in something like this.’
Gemma says that living with cystic fibrosis means that Ivy has matured far beyond her actual age.
The family even jokes about how the youngster is ‘five going on 15’ – but she says that shouldn’t be the case.
‘Ivy fully understands that what she is doing will help to fight for a cure.
‘She knows what’s going on and what’s at stake, and is so mature about it all.
'But that’s because she has had to fight to take control of cystic fibrosis, and it’s not fair for a child to be put through that.’
Vertex has been previously accused of trying to profit from terminal illness, with the National Institute for Health and Care Excellence (Nice) saying it would cost £104,000 per patient each year – but Vertex insists it is ‘committed to working with all parties’ to make the drug available in this country.
In a letter sent to health secretary Matt Hancock, Dr. Sarah Wollaston MP wrote: ‘Patients are being denied access whilst the arguments rage about the returns on Vertex’s investments.
‘Nice’s appraisal processes and NHS England’s understandable responsibility for managing a finite budget and the needs of all patients.
‘We would like to see interim access agreed whilst the wider issues are further debated, as has happened elsewhere.’