The ‘lottery’ where patients with cystic fibrosis are losers
PLEA TO MAKE LIFE-EXTENDING DRUG AVAILABLE FOR GRANDSON
A COUPLE whose son died from cystic fibrosis are making a desperate appeal for a life-extending ‘wonder drug’ to be made available to help their grandson who is battling the same cruel condition.
John and Frances Binns are campaigning to end the deadlock between the NHS and Vertex, the makers of the drug Orkambi, which has been shown to improve lung function in people with cystic fibrosis (CF).
The retired couple, who live in Wooldale, near Holmfirth, are guardians for eight-year-old CF sufferer Tristan whose father, Andrew, took his own life in 2016.
Their other son, Adam, had CF and died from the condition in 1999 when he was 25.
John, 70, who was a divisional commander with West Yorkshire Police, says Orkambi could help Tristan live a longer, healthier life.
Only around half of those with the inherited condition live to celebrate their 40th birthday.
“For a long time cystic fibrosis has been a desperately sad and tragic genetic disease with no cure.
“When we lost Adam in 1999 I don’t think there were many options other than a lung transplant.
“Now the gene for the condition has been identified and there has been a lot of work on ‘super drugs’.”
The drug Orkambi, he says has the ability to ‘stop the clock’ in sufferers, allowing them to live longer.
“The fact that the drug Orkambi can be used does save lives and is available in 11 other countries - including Scotland and Eire - is simply obscene and painful beyond belief.
“This is a cystic fibrosis lottery, where one loser is one too many. It cannot and should not be allowed to continue.
“Cystic fibrosis patients, many of them children, are dying as this devil’s poker game is played out. The Orkambi impasse is outrageous and completely unacceptable.”
John and Frances, 68, a retired nurse, had their healthy son Andrew in March 1973, before the birth of premature triplets Francine, Claudine and Adam in February 1974.
Adam, who was diagnosed with CF, was a poorly baby but with the help of a CF expert the couple managed to keep him as fit and healthy as they could.
John recalls: “He was accepted onto the transplant list and we had a
The Orkambi impasse is outrageous and
completely unacceptable
pager (in case a pair of lungs became available). The pager didn’t ring.”
Sadly, Adam suffered from a series of lung infections and died, aged 25, in March 1999.
Their other son, Andrew, a Para Combat Engineer with a ‘bomb disposal’ regiment, had son Tristan in 2010 with his then girlfriend.
Andrew became the sole carer for Tristan but found it hard to care for the poorly child and struggled with