Aisha Te Kani was born with a malformed heart sitting outside her chest. She is the only adult in New Zealand - and likely the world - with a rare heart condition called Ectopia Cordis.

She sits on the living room sofa, her fragile, blue-tipped fingers wound together.

Lost in her thoughts, her shirt moves like magic; the gentle ripple across her chest like a breath of wind. Except it's not.

Aisha Te Kani's heart sits outside her chest, which is what is causing the movement. It's the size of an orange; she can cup it in her hand while it beats.

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Born prematurely by caesarean section in Tauranga Hospital on February 11, 1980, Te Kani wasn't expected to live.

It wasn't possible to put her heart back in her chest and a skin graft was performed so that she could be comfortably nursed by her mum, Moana.

The graft, taken from her leg, was only expected to be temporary but 39 years later, it's the only heart surgery she's had.

Her condition, Ectopia Cordis - the heart outside the rib cage - is extremely rare, cause unknown.

What makes her case tougher, says Tauranga Hospital consultant cardiologist Dr Graeme Porter, is that she also has congenital heart disease and surgery is too risky.

Ectopia Cordis - the heart outside the rib cage - is extremely rare, cause unknown. Photo / George Novak
Ectopia Cordis - the heart outside the rib cage - is extremely rare, cause unknown. Photo / George Novak

Matapihi resident Te Kani is not only unique in New Zealand with her condition; she's most likely the only one in the world.

Porter, who runs the Adult Congenital Heart Disease Clinic in Tauranga supervising around 300 patients, has known Te Kani for about 14 years. He knows it's extremely unlikely he will ever see another adult patient with her condition again.

The complications are not just that her heart sits outside her chest but the fact she has a complex abnormality known as 'Tetralogy of Fallot'. Left untreated, the condition is rarely survivable into adulthood.

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"I'm not aware of anyone else in the world with that combination who's now an adult in their 30s. It's very likely that Aisha is the only person in the world with her particular physical attributes."

"I'm not aware of anyone else in the world with that combination who's now an adult in their 30s. It's very likely that Aisha is the only person in the world with her particular physical attributes."

Originally under the care of Greenlane Hospital in Auckland, it's taken a team of cardiologists to make the decision not to attempt heart surgery, he says.

Baby Aisha Te Kani with her skin graft over her heart. Photo / supplied
Baby Aisha Te Kani with her skin graft over her heart. Photo / supplied

"We didn't think we could get her heart back into her chest and, because of how things have changed over time (functionally), we also don't think an operation now would make things better. It would just be very, risky."

Despite the bleakness of her situation, Te Kani is plucky and treats every day like a gift.

Her close-cropped hair is a lush black. It's trimmed neatly around a delicate, perfectly-formed face. Outwardly, her smile shows good health, but her lips and fingertips are blue due to lack of oxygen in her bloodstream.

She is friendly, with a sense of humour and an admirable acceptance of her uniqueness.

"No one has really told me how long my life will be," she says from the lounge of her parents home, where a print hangs on the wall with the word: 'Hope'.

She speaks pragmatically about the complicated facts of her life.

"They never really expected me to survive at all, so I guess they just wait and see."

Te Kani speaks pragmatically about the complicated facts of her life. Photo / George Novak
Te Kani speaks pragmatically about the complicated facts of her life. Photo / George Novak

Of Christian faith, God has been her anchor in a journey that's best described as up and down.

Her pastor, Tamati Cameron at C3 Church Tauranga calls her "incredible".

"She's a person of wisdom and insight, and she's strong and courageous."

Some saw her as a "curse" when she was born and, when she went to school, she had to wear a fibreglass shield strapped around her chest.

She also has club feet and has to wear built-up shoes. As a child, she was in an unwelcome spotlight everywhere she went.

She didn't walk until she was nearly 5. Her right foot is two sizes bigger than her left and she walks on tip-toes when she hasn't got her shoes on. Certain terrain means that she has to be carried.

Efforts were made to straighten her feet when she was 12 and 13, putting her in hospital for lengthy periods and in a wheelchair for a year.

Aisha Te Kani in 1990, age 10. Photo / Glenn Jeffrey
Aisha Te Kani in 1990, age 10. Photo / Glenn Jeffrey

Then she had a growth spurt and her feet went back to how they had been.

Her mother sees her club feet as a blessing. "If my feet move too fast, then it would put pressure on my heart," Te Kani explains.

She is grateful for the steadfast support of her protective family but at times has felt guilty.

Late last year, she apologised to her mother for "what I cost you".

"The magnitude of being an adult and seeing the impact my birth had on my family ... I'd come to grips with what that meant for them."

Her mother responded by shaking her head: "Don't you ever think that about yourself," she scolded kindly.

Te Kani muses: "Even when I think I'm fully healed and good and accepting of who I am, there are still things that come up. I really have to think: 'How do I walk through it in a way that's going to be helpful for me?'"

This is especially the case as she nears 40.

She had relatively few health issues growing up but now that she's getting older, she's had two abscesses on the brain and irregular heartbeats.

One of the long-term consequences of untreated cyanotic heart disease is abscesses on the brain and Te Kani is now on a continuous course of antibiotics to try to prevent it from happening again.

She lives at home with parents Moana and George, partly because of previous health scares. After the first brain abscess in 2006, she was advised it'd be too risky to ever have children.

She cried at the magnitude of understanding, for the first time, the life-long implications of her illness.

Te Kani had few health issues growing up but now that she's getting older, she's had two abscesses on the brain and irregular heartbeats. Photo / George Novak
Te Kani had few health issues growing up but now that she's getting older, she's had two abscesses on the brain and irregular heartbeats. Photo / George Novak

She is single, saying she's never let herself be interested in "that side of life".

Growing up, she had the same aspirations and desires as her peers but lacked the confidence and support in her peer group to believe any of it was possible for her.

"Then as an adult, I've weighed up the cost of me and what it means to be with someone like me. It's a combination of me overthinking things and me thinking my situation is worse than it is because all my friends are like: 'Nah, there will be a man there'," she laughs.

Life has never been straightforward and she's endearingly honest.

She recalls the shame of being wrongly accused of stealing an apple in a fruit shop at 7. The store owner was suspicious of a moving bulge under her clothing and to prove her innocence, made her pull up her shirt.

Then at 10, she recalls a "defining moment" of going into the bathroom and contemplated taking her own life.

"I didn't want to have to deal with the reactions of people anymore and the way that I felt about myself. Those feelings were so big."

She falls silent.

What stopped her was an image of her youngest brother, Josh, then 5.

"I loved him as soon as he arrived. He was perfect. The biggest thing for me was that there was nothing physically wrong with him."

Love has lifted her up time-and-time again.

Porter wary of treatment

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21 years' experience as a cardiologist, Dr Porter has always been wary fellow doctors don't treat Te Kani as a "spectacle or unusual".

"There have probably 30 to 40 people in the Bay who come to my clinic with repaired Tetralogy of Fallot, so that's still not that common, but that's very common compared to having your heart outside your chest," he notes.

"She's a wonderful young woman and her body has sort of adjusted so that she can live life with what - on paper - you'd say is not survivable but that's what she's been doing."

"Her body has sort of adjusted so that she can live life with what - on paper - you'd say is not survivable but that's what she's been doing."

There is no clear explanation for Te Kani's rare heart condition. Photo / George Novak
There is no clear explanation for Te Kani's rare heart condition. Photo / George Novak

Porter says it's unusual for cardiologists to pinpoint a cause of her condition because a lot of complex things happen when organs form and cells divide, migrate and differentiate.

Her club feet are likely linked to her heart abnormality.

"Genetics and active research are trying to sort all of this out but at this point in time for most congenital heart disease, there isn't a simple explanation."

Her condition has meant countless poking and probing from medics and that's something her three brothers find uncomfortable.

"'Sis, I hate it when they do that to you'," her middle brother has told her. She's had to reason: 'I know that you don't like it but this is part of my life'."

A growing journey

OVERCOMING

obstacles has defined her character as she's moved from the darkness into the light.

She's bolder now. When she was young, her parents did the speaking for her. "It's been a growing journey."

Her brother, Dillon sees his sister as an inspiration as well as a "miracle".

"Not much gets in the way of Aisha and her aspirations in life. She's had a hard journey but it's been a journey of discovery as well."

She got her driver's licence eight years ago which opened up "all sorts of freedoms" for her, he says. "She's not been one to sit idle at home."

Not much gets in the way of Te Kani and her aspirations in life. Photo / George Novak
Not much gets in the way of Te Kani and her aspirations in life. Photo / George Novak

A qualified counsellor, she works part-time as the operations coordinator for the charitable trust, Live For More, which runs an eight-week programme for troubled young men aged 17-24.

In week seven, the theme is "perseverance" and Te Kani shares her story.

At the end of her talk, the males whisper the same thing: 'Am I allowed to see it?"

"I show people my heart if they ask me because I know they will never see it again in their lifetime."

"I show people my heart if they ask me because I know they will never see it again in their lifetime. They just buzz out. 'Bro, I touched it!' They're like kids in a candy store," she quips.

Strangers who've heard her story approach her and thank her. But if she's hugged too hard she can pass out.

Every day is a blessing.

"My family, my friends, the connections that I have in the community. These are the things that make me who I am," she says.

"Without these things I would just be like everyone else, and I don't know if I want that. I want to be authentically me."

SIDE BAR
# What is Tetralogy of Fallot?
A form of cyanotic, congenital heart disease. Where the communications are between the cardiac chambers, there's a passage of unoxygenated blood from the venous system that gets shunted into the arterial system.
Blood has to go through the lungs, and when you lose that filtering (like Aisha Te Kani), bugs that get into the bloodstream can end up in the systemic organs and with the low oxygen environment they can grow.

# Public speaking
Te Kani hopes to do more public speaking. To watch her TEDxTauranga talk, go to: https://www.youtube.com/watch?v=kITBem3HK5c The next round of talks is on June 22. See www.tedxtauranga.com


Where to get help?
Lifeline: 0800 543 354 (available 24/7)
Suicide Crisis Helpline: 0508 828 865 (0508 TAUTOKO) (available 24/7)
Youthline: 0800 376 633, Kidsline: 0800 543 754 (available 24/7)
Whatsup: 0800 942 8787 (1pm to 11pm)
Depression helpline: 0800 111 757 (available 24/7)
Rainbow Youth: 09 376 4155
Samaritans 0800 726 666
If it is an emergency and you feel like you or someone else is at risk, call 111.