Maurice Gueret on Simon Harris
Ava Barry has to endure hundreds of seizures every year. Unfortunately, most modern medicines fail to control the episodes. Her worried parents tell Joy Orpen they believe a cannabis oil, not available here, may hold the key
Vera Twomey and Paul Barry are absolutely terrified that their gorgeous little girl will die if she doesn’t get a particular drug that has not yet been approved in Ireland. This hard-working couple from Aghabullogue, in Co Cork, have four beautiful children aged six and under. Ava, who is the eldest at six years old, is followed by Sophia, Michael, and baby Elvera May. Having such young children is difficult enough, but unfortunately these parents also have to contend with the fact that Ava has a chronic, life-threatening illness. They believe her only hope rests with a medication that has not yet been approved in this country for patients like her. So Vera and Paul fear that Ava will die, simply because she cannot get access to the drug.
Vera and Paul, a tractor mechanic, come from the same local area in Co Cork. Seven years ago, they married and have built a comfortable home adjoining Vera’s mother’s farmhouse. They were absolutely thrilled when baby Ava arrived safe and sound following a difficult labour, which culminated in a Caesarean section delivery. Fortunately, things soon reverted to normal.
However, four months later, baby Ava had a massive seizure. Her frantic parents called the emergency services who rushed the infant to Cork University Hospital (CUH) by ambulance, where she was seen by a paediatric neurologist. As some form of epilepsy was suspected, blood tests were done. In the meantime, the seizures continued. Paul says there is no discernible pattern to them. Ava can have several in succession, and then none for several days.
In time, Vera and Paul were given a diagnosis of Dravet syndrome (DS). They learned that this rare condition, also known as severe myoclonic epilepsy of infancy (SMEI), meant Ava was unlikely to ever walk or talk. Vera says she was hysterical when she got the diagnosis, but she became determined to do everything in her power to help her precious daughter.
Over time, doctors tried various kinds of medication in efforts to control the seizures, but with little lasting success. “It’s said that Dravet’s breaks through all types of medication,” Vera explains. “When she has a seizure, she goes stiff, then her arms and legs twitch and she can have uncontrollable muscle spasms and jerks. She also has what are known as ‘absences’ when she is not really present, and ‘drop seizures’ when her legs suddenly turn to jelly; these can cause very serious injuries. It all very frightening and worrying.”
In the meantime, she and Paul worked so diligently they even got Ava walking when she was two years old, in spite of all the predictions to the contrary. But, one day not long after, this sweet little girl had five consecutive seizures. Vera says the doctors had no option but to try a different medication. Unfortunately, Ava had such a bad reaction, she was unconscious for five days. “Following the coma, she lost the ability to walk,” says Vera. “It took us another year to get her back on her feet; but she did it; we are so proud of her.”
Apart from the seizures, DS causes a range of other difficulties, including insomnia, delayed learning, speech, balance and gait issues, low immunity and acute sensitivity to various things including light and heat. “A bath could trigger a seizure if it was too hot, or too cold.” explains Vera. “Her personal freedom is constantly compromised because she can react so badly to so many different things. She has to be watched like a hawk all the time.”
At 5am on the morning of this interview, Ava had a particularly severe seizure. It left her exhausted, tearful and fractious. She’d had two episodes the day before, and 18 the previous Sunday. “It was a desperate day,” says her mother tearfully, “and that doesn’t even include the absences.”
Tears are quite a common feature in Vera’s life these days. With three younger, delightfully boisterous children to contend with, on top of Ava’s incredibly serious and highly demanding medical condition, she’s reached breaking point. This, in spite of the fact that she has huge support from her immensely capable and loving husband Paul, and from her wonderful mother. “When Ava has a seizure, we have to wait for one minute to see if it passes on its own, before giving her medication,” says Vera. “If Michael or Sophia are with her when she has one, they automatically start counting to 60. They are only three and four years old. This is no way for such young children to be living.” Vera says she used to get home help for one hour a day from the HSE, but unfortunately for this unbelievably stressed family, even that came to an end, and Vera says people don’t understand just how difficult the situation can be. “They’ll say, ‘It’s just epilepsy’. Well, epilepsy is bad enough, but we have the added problems of seizures being triggered for no apparent reason.”
Paul says Ava is rushed to hospital, on average, once a week. So he and Vera have nothing but praise for the paramedics who come from Macroom, to rush little Ava to Cork. “They really, really care about her,” Paul volunteers.
Vera says the doctors at CUH have tried all of the drugs that might be likely to lessen Ava’s seizures, but without any obvious, lasting success. “Some weeks back, they told us that there were none left to try. So they want to try combinations of those drugs.”
‘But you have to ask, what if Ava has 22 seizures next week, and because of them, she slips into a coma?’
Vera Twomey and her daughter, Ava
Vera believes Ava’s best hope lies in CBD, an oil derived from the cannabis plant. She says this has been made available to patients in certain States in America and in other countries. She says some of the results have been outstanding. “One little girl was having up to 200 seizures a day, and, after a period of treatment on CBD oil, they were reduced to three a month.” Vera says that in some cases, the seizures were eliminated altogether. But Ava’s chances of getting CBD in Ireland are nil right now, because it is nowhere near being approved by the authorities for cases like hers. And so Vera has begun a petition to put pressure on those very same authorities about the current situation. “I’m canvassing everyone I can think of,” she says.
“The doctors know CBD works. But my daughter can’t have it, and I’m not allowed to bring it into the country. We are always trying to be really positive, but you have to ask, what if Ava has 22 seizures next week, and because of them she slips into a coma and dies? Just before Christmas she went into cardiac arrest. I’m so, so terribly frightened for my beautiful little girl.”