FAMILY APPEAL FOR DRUG TO GIVE MAEVE A FUTURE
Lixnaw family appeal to Govt to fund PKU drug Kuvan
A YOUNG Lixnaw girl is struggling with a life-limiting condition that could ultimately cause her brain damage while the State rejects calls to fund a drug representing her only chance at a normal future.
Sixth class pupil Maeve Quilter – who is just shy of her 12th birthday – was born with the metabolic condition Phenylketonuria or PKU, as it is commonly known. Like Cystic Fibrosis it is a condition that affects the Irish to a greater degree than other ethnicities; and is caused when both parents carry a recessive gene. In this case the gene inhibits the sufferer from breaking down the protein phenylalanine - found commonly in our foods and of no threat whatsoever to the vast majority of people.
But for those, like Maeve, with PKU the inability to break down the protein and the resultant build up of it in the brain to toxic levels can lead to brain damage, seizures and behavioural problems among other deeply distressing health prospects.
There is a drug that could give Maeve and her many fellow sufferers (roughly one in every 4,500 are born with PKU or a milder variant in this country) a chance at some quality of life.
Irish-manufactured Kuvan is the only treatment available for people with PKU but it was recently rejected as a candidate for funding by the National Centre for Pharmacoeconomics (NCPE) to the despair of families.
Now they’re ramping up a campaign to lobby the Government, with a briefing taking place in Leinster House on Thursday.
“Kuvan would take away a lot of the side effects and we’re told that Maeve could be a candidate for the drug,” Maeve’s mother Siobhán told The Kerryman.
Maeve’s life is already heavily circumscribed by PKU as she has had to adhere since birth to a diet of incredible rigidity.
“She’s a fantastic girl and we’re so proud of her. You wouldn’t know she has to live with a serious condition from looking at her and she enjoys a very active life but we have to go through everything she eats and drinks with a fine comb,” Siobhán explained.
“Maeve takes seven ‘exchanges’ a day of natural proteins which we measure and weigh with special scoops that have to be absolutely accurate. This is to prevent her from ingesting phenylalanine which she can’t break down and which is found in so many foods and drinks.
“Maeve also has to take a synthetic drink every day, and we also get synthetic foods and drinks on prescription from the chemists, such as bread, crackers, rice and pasta.
“Because of the condition you’re always worried about when you have to travel somewhere as you can’t simply grab a sandwich or drink. We know what products and brands Maeve can take, but even then manufacturers change the ingredients slightly every now and again so you have to constantly read labels.”
Maeve was born in the US – where Siobhán is from – moving to Lixnaw at an early age. But when the family returned recently for a five week trip, Maeve’s special food and drink required two separate suitcases.
“Ironically if we had stayed in the US Maeve might now be on Kuvan as it is available to PKU sufferers there. The restrictions are so tough on Maeve but we’re so lucky that she is so wise about it. She knows she just can’t have these foods and drinks.
“Our big worry is that Maeve would lose cognitive function without Kuvan, which is the only thing that could give her hope of a chance at some quality of life. That’s why we’re calling on all our TDs and senators to attend Thursday’s briefing and support our calls to fund this vital drug,” Siobhán said.