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

Sunday Independent (Ireland) - Life - - CONTENTS -

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.”

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