It’s a dark week in West Kerry. The grey sky echoes the mood that eerily saturates a small country village just outside Castlemaine. It’s called Keel.
Tonight in Keel no one smiles. I stop at a local shop to ask for directions to the Heffernan home. My voice is quiet as I query where I am to go. The reply is almost inaudible. We both know the polite chatter that normally ensues when two strangers come in contact was unnecessary.
A small boy is lying in a coffin three minutes up the road in the home he shared with his parents and his sister, Saoirse, before she passed away three years ago.
Liam is the second child Tony and Mary Heffernan have lost to a neurological disease -- Rare Infant Batten Disease -- a rotten, disgusting disease that robs the lives of young children. It’s incurable.
Saoirse was five when she passed away in 2011. Liam was also five when he died in his parents’ arms on Sunday morning.
My husband John and I finally find the house moments after leaving the shop. It isn’t hard. There are dozens of cars lined on either side of the country road. We walk up their steep driveway with heavy hearts and little or no words between us.
Out of respect to Liam’s parents, grandparents and extended family I will stop there, but seeing beautiful, peaceful Liam lying in his coffin in his own home just screams injustice, unfairness and outright sadness.
Liam and Saoirse were too young to die. I’ve no doubt it was unbearable losing one child to this horrible disease but two babies, their only two children, is undeniably horrific.
I first met Tony in 2010 while working as a full time reporter with the Irish Voice. Tony had come to New York with Saoirse and his wife Mary to see if there was a small possibility he could save his children’s lives. There were trials for those with Batten Disease, and the Heffernans hoped and prayed every day that their children would be accepted, that it would prolong their lives and ultimately save their children.
I instantly connected with Tony. He is that sort of guy. He oozes passion for what he believes in, and anyone who came in contact with this Kerry man knew his one and only aim in life was to try and save his children’s lives.
As he fought with every bone and feeling in his body he soon learned he wasn’t alone. There are other families in Ireland also affected by Batten Disease. He became a support to them, and before long the Heffernans were getting to know hundreds of families around the globe in their situation.
They all had one thing in common -- they were all ultimately going to lose their child with Batten Disease. No one outside of this could truly understand what was ahead of them.
The Kerryman’s Association of New York, spearheaded by Billy O’Sullivan, decided it was time to step in and help this family. I being a fellow Kerry woman got on board and a group of us supported by local businesses, and the wonderful Irish and Irish American community of New York and beyond, raised a substantial amount of funds that went directly into helping the Heffernans fight to save their children’s lives.
Little did I think back in New York in 2010 that both little angles would grow their wings so soon -- a phrase that Tony and Mary use to describe the death of their beautiful children.
The Heffernans founded the all-Ireland Batten Disease charity, Bee for Battens, in 2010 to support sufferers, fund research and participation in medical treatment trials.
Mary and Tony’s world began to tilt off course back in January 2009. Their first born, Saoirse, began having seizures. Doctors put it down to epilepsy. All the symptoms pointed in that direction.
However, Saoirse's seizures became more aggressive, finally resulting in her suffering up to 200 a day. In March of that year Saoirse was referred to Temple Street Hospital in Dublin. Endless tests still revealed epilepsy.
The Heffernans were satisfied with the diagnosis and Saoirse began a course of treatments to help her little body deal with her seizures. The following August she was called for an MRI in Dublin.
The results came back a few weeks later. The news wasn’t good.
The Heffernans were asked to bring Saoirse to Beaumont Hospital, an academic teaching hospital in Dublin city, to meet with a group of Irish neurologists who were visiting that day.
"They said they wanted a fresh pair of eyes to look at Saoirse. I never expected the outcome," Tony Heffernan told me during an interview back in 2010.
Overhearing the whispers between doctors, the word Batten Disease was thrown around. Tony wasn’t familiar with the term so he Googled it.
His heart began to rip from his chest slowly as he read what was before him. “Batten Disease is a rare neurological disorder that begins in childhood…. It is always fatal.”
His worst fears were later confirmed. His daughter was going to die. Not long after that their son Liam was also diagnosed.
Both Mary and Tony couldn’t accept this. During his research, Tony happened across a Batten Disease trial in Weill Medical College of Cornell University in New York that gave him hope for his two children. In 2004 a clinical trial was launched at Cornell to study a gene therapy method for treatment of the signs and symptoms of Batten Disease. An experimental drug works by delivering a gene transfer to the brain.
In 2008 the procedure was deemed safe. Both Saoirse and Liam were registered for the trial.
When it came to Saoirse’s turn she was too sick to participate. Liam did travel to Cornell Hospital in May three years ago, where he underwent eight hours of brain surgery as part of a medical trial, the only hope the Heffernans had of extending his life. It did for a short time.
In the three years that followed Liam lost his sight, his ability to swallow and multiple seizures became part of his everyday existence. Over the past few months he deteriorated rapidly, resulting in him being admitted to Kerry General Hospital two weeks ago.
Last Wednesday Liam was brought home to die. Tomorrow Liam’s little coffin will be placed beside his sister’s in Keel Cemetery, a few yards from the family home.
Tony and Mary have fought tirelessly to find a treatment in an effort to save their children and hundreds of children around the world affected by Batten Disease. They have been successful in planning to develop Ireland’s first National Respite Center for Rare Disease on a 10-acre site in Tralee, Co. Kerry.
Construction is set to begin next year with the hope of having it up and running by 2016. They have been lobbying the government for funds and asking the public to help out whatever way they can.
Donations can be made to the Saoirse Foundation, AIB Tralee, Co Kerry Account No: 85130187; Sort Code: 93-62-19; IE13AIBK93621985130187; Swift Code: AIBKIE2D
Postal donations can be made to: Bee for Battens, the Saoirse Foundation, Castldrum, Castlemaine, Co. Kerry, Ireland.
You can also donate by sending old mobile phones free of charge to the Bee for Battens Appeal, Freepost, PO BOX 7958, Dublin 11.
More details available on www.beeforbattens.org.