Lucinda’s story: ‘We have had it for four generations’
Lucinda McNerney (45), has had a pacemaker since she was 18 to offset the effect of a congenital heart defect she was born with called Long QT syndrome.
The syndrome, which also affects two of her three children, can lead to sudden heart failure and death, sometimes in adults unaware they have the condition.
“We didn’t know the inherited nature of it about 27 years ago, so there was not much known about it,” recalled Lucinda, who was diagnosed after suffering an “event” from which she recovered. “I just got on with my life, went to college.”
For some with LQT, and this includes Lucinda’s family, LQT is particularly dangerous as it lurks in the background without any symptoms showing, until the condition is triggered with potentially fatal consequences. The trigger can be anything from heavy exercise to medications, and can affect all age groups.
Lucinda is anxious to make the world as normal and safe as possible for her two children with LQT. This involves having a defibrillator installed in the home in Cork and listening carefully to medical advice. She is the patient representative on the Sudden Adult Death council, which is run along with the Irish Heart Foundation.
Her eldest child, Oisin, has the gene, and he is being treated with beta blockers. For him, it meant that he had to give up playing competitive sports with friends.
“He [Oisin] could play a game of hurling,” said Lucinda, who is from Cork, “but the training would be so intense with your peers. Would a 10 or 11-year old stop because he felt faint? That would be the worry. It wasn’t worth the risk.”
Lucinda’s youngest child, Dervla (7), was diagnosed with LQT syndrome shortly after her birth. “She was born in Cork and brought by ambulance to Crumlin, where she was put on beta blockers,” said Lucinda. It meant that Lucinda, her mother (63), and two of her three children, plus some of her siblings had LQT.
Lucinda heard about research using Crispr technology to tackle LQT being undertaken jointly by Dr Terry Prendiville, consultant paediatric cardiologist at Our Lady’s Hospital for Sick Children, Crumlin, and Prof Timothy O’Brien, based at the Remedi centre at NUI Galway. The scientists were looking for families with LQT that were willing to donate skin samples for research with the goal of correcting the heart cells that have the genetic defect that causes the syndrome, at some point in the future.
Lucinda said it was an emotional day for the entire family.
“We have had it for four generations and we have lived with it, but it would be amazing to think that you wouldn’t have that worry for the next generation,” said Lucinda. “My brother is also a gene carrier and he has two kids, one affected and one unaffected so we are going to bring them all up – again.
“We want to encourage as many people as we can to participate in it.”
Three generations of the McNerney family have suffered with a congenital heart defect called ■ Long QT syndrome: Lucinda McNerney, Lucinda’s daughter Aoife McNerney and Marian Cassidy (Lucinda’s mother)