They said she would not last a year... now she’s 18

ERIKA Cowie’s 18th birthday cannot come soon enough.

Crossed-legged in an armchair, occasionally glancing at her phone, the teenager is animatedly running through the minutiae of the upcoming celebration in Cardiff. Chattering at a mile a minute, she goes over the itinerary she has memorised by now; down to sleeping arrangements, where she and her posse will grab a bite before duly hitting the town on Saturday.

But her obvious excitement and haste to finally turn 18 are not born of youthful impatience.

She was never meant to survive her 14th birthday, let alone reach adulthood.

And while her heart could still give out at any given time, Erika, who sufferers from idiopathic pulmonary arterial hypertension, refuses to let life pass her by.

“Why can’t my life be like anyone else’s?” asks the 17-year-old defiantly. “It can be. I just have to adapt. It could happen at any moment so I’m getting on with it. I’m going to Cardiff for my birthday - that’s happening.”

Throughout, her mother Rachel listens silently, occasionally nodding; only her clipped expression betrays her apprehension at the prospect of her daughter’s overnight stay. Keen not to spoil Erika’s fun, she soon puts on a smile, playing along. “I’ll be there, hiding behind lampposts,” she quips. “Joking is how we deal with everything,” explains the 47-year-old from Rodbourne. “We could burst into tears anytime, and we do, but you have to pick yourself back up and laugh. It’s not remotely funny but you have no choice. You can’t spend your life worrying.”

Erika was just 12 when she first complained she “couldn’t keep up” in PE classes. Rachel took her daughter’s moans with a pinch a salt. Erika was never one for group sports at the best of times and she initially dismissed her complaints as a cunning ploy to get out of lessons.

She eventually relented and signed her daughter off PE. But Erika continued to complain of shortness of breath and dizziness.

They consulted a doctor who suggested she may have asthma. But the hypothesis seemed more and more unlikely as time went by and Erika grew uncharacteristically lethargic.

Then, on the eve of her 13th birthday in July 2011, the teenager collapsed at a friend’s house. She came round only to fall unconscious again a few moments later.

Things came to a head when Erika returned home from school so exhausted a few weeks later that she sat on the floor and dozed off on the spot. Her skin had turned a blueish hue. She was rushed to A&E.

“It was just mad,” recalls Erika. “I had never even had a nosebleed, I was rarely sick. It was just from zero to sixty. I never left the hospital after that.”

She was taken to Bristol Children’s Hospital before being blue-lighted to Great Ormond Street where she underwent a battery of tests, including a right-heart catheterization, to measure the pressures in her heart and lungs. Rachel and Erika’s dad went through the motions, keen not to alarm their daughter. A few days after her transfer to London, they were ushered into a room by consultants and told Erika suffered from idiopathic pulmonary arterial hypertension, an extremely rare, life-threatening disease which prevents the heart from pumping blood through pulmonary arteries adequately. There was no cure. Erika’s heart would likely fail before her next birthday.

“They said she would not last a year,” whispers Rachel, choking back tears. “I remember ringing my mum and saying, ‘There’s nothing they can do. It’s fatal.’ It was heart-breaking.”

She kept the prognosis from Erika. Until just a few weeks ago, the 17-year-old still had no idea how close to death she once came.

And yet against all odds, her child celebrated her 14th birthday and despite some setbacks, thrived, confounding consultants and her own devoted parents.

“For a long time I didn’t think we’d be here today, not a chance,” says Rachel, her eyes firmly on her daughter. “For me it was about little goals, those milestones I could get her to. She had been going on about prom since the age of 11 so I wanted to get her to prom.”

“She has managed all of it,” adds the self-employed home help. “But you can’t help worrying. I still walk into her room every morning to make sure she’s still breathing. She could deteriorate at any moment. It will be her heart that gives up the ghost.”

Partly because of her condition but also due to treatment, Erika will never be able to have children.

While IPAH is incurable it can be stabilised to some degree with the right drugs. Erika initially received treatment through a nebuliser. When the course of medication stopped bringing her relief, she was fitted with a Hickman line and pump, which releases her medicine directly into her bloodstream 24 hours a day.

But the drugs have had very little impact in recent months, Erika explains. And the condition has only gnawed back more ground, taking its toll on her weakening body. Although Erika is resolved to live as normally as possible – and even recently took up a sales assistant job at the Outlet Centre eight hours a week – she cannot stand more than 30 minutes without going through excruciating pain, her breath grows more shallow every day and the niggling pressure on her chest more difficult to cope with. Climbing up the few stairs to her room has become such a challenge that Rachel is currently converting part of the ground floor of their home into a new accessible bedroom for Erika.

“It’s a constant battle,” Erika concedes, only the slightest hint of frustration piercing through her voice. “It’s getting worse. I can definitely feel it.

“I have my good days and my bad days. A lot of people are shocked that I’m getting on with it. But there is nothing else I can do.”

The fiercely independent teen has found welcome solace and friendship in Amelia Brown, her Rainbow Trust support worker. The children’s charity provides practical and emotional help to young patients with life-threatening or terminal illnesses and their families.

“I don’t really like asking for support,” points out Erika. “But with Amelia it’s different. We talk about anything, boys, parties. It’s not a reminder that I’m ill. She understands me, and I know I can go to her about anything. I can say things to her that I can’t say to my family or friends.”

Erika, Rachel and their family have raised nearly £850 for The Rainbow Trust this year.

Despite her daughter’s unflagging optimism and confidence in the future, the severity of her condition and her scant life prospects are never far from Rachel’s mind. As it stands, Erika is not expected to reach her twenties.

“It’s very vague,” sighs Rachel. “Last time we were told she would not turn 19 or 20. But then again, they gave her a year when she was diagnosed.

“But you always wonder: ‘Will she make it to Christmas?’, ‘Will she make it to her birthday?’ Then there are days when you think you can’t live like this, you have to enjoy the bits in between.”

“Which we do,” chips in Erika with a grin.

Rachel nods: “If she goes, whether it’s quick or slow, we will deal with it then. It will be awful and we will crumble, we know that, but I won’t spend every day dwelling on it. You can’t live your life thinking, ‘Could it be today?’”

Erika cuts in cheerfully: “I’m here, aren’t I? All I want is to make sure I’ve got a good life. I’d rather die doing something that I enjoy than waste time.”

Factfile

• Rainbow Trust Children’s Charity provides palliative care and practical and emotional support to children with life-threatening or terminal illnesses and their families.

• The charity relies almost entirely on voluntary donations and helps more than 2,000 families a year throughout England.

• In Swindon demand for Rainbow Trust family support workers far exceeds the charity’s current capacity.

• In a bid to raise much-needed funds and help families in need across Swindon and beyond, Rainbow Trust recently launched a campaign, A Day with Amelia, highlighting the vital work of the family support team to make life just a little bit easier for families caring for a seriously ill child.

• To make a donation call 01372 220083, text RAINBOW to 70111 to make a £3 donation or visit rainbowtrust.org.uk/amelia