The Harsh Realities of Living with a Congenital Heart Defect

 

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Photo credit: Amelia Smith 

Poppy Smith is unlike most other kids her age.

At just four and a half years old, Poppy has undergone five open heart surgeries.

Five.

Almost one for each year of her life.

When I ask her what her favourite colour is, she replies that she likes all of them- all the colours of the rainbow except for one- red.

Perhaps this is because the colour red is associated with blood- a harsh reminder of the congenital heart defect that impacts her everyday life.

Poppy suffers from the heart condition Hypoplastic Left Heart Syndrome (HLHS), a congenital heart disease that is among the most severe forms of heart defects.

Most babies diagnosed with HLHS are extremely ill in the early days of life and need urgent heart surgery to survive.

According to the Royal Children’s Hospital (RCH) cardiology department’s online resources, HLHS occurs when the left side of the heart is very poorly formed and therefore cannot support circulation around the body. The left ventricle and the aorta are both abnormally small or hypoplastic.

“In very basic terms she only has half a functioning heart,” says Amelia Smith, Poppy’s mother.

“The left side of her heart didn’t develop properly when she was growing in the womb.”

Amelia and her husband Matthew were distressed when doctors first learned of Poppy’s condition.

First-time parents are almost always ecstatic during the early stages of pregnancy- picking out colours to paint the nursery and planning comical games for baby showers.

For the Smith family, things were very different.

Five months into the pregnancy and during the second trimester, tragedy struck.

During a routine anatomy scan that lasted an hour and a half, Mr and Ms Smith became anxious when the sonographer could not visualise all four heart chambers.

The next day, they received a call from their general practitioner- there was an abnormality with Poppy’s heart.

“It was horrible- we didn’t know what it meant for our unborn baby. We didn’t know if we would have to terminate the pregnancy or if she was even able to survive.”

“From when we were given the diagnosis to when we got to speak to a foetal cardiologist was weeks. In the meantime, we had the words of an obstetrician stuck in our heads that our baby would have severe developmental delays and would need endless surgeries,” says Ms Smith.

“She would have no quality of life and she would never live a normal childhood, that is, if she even survived birth or the first two surgeries. She painted a very bleak picture for us.”

Amid their anxiety, Ms Smith recalls how relationships with friends and family began to suffer. The severity of Poppy’s condition was not understood and therefore they lost the dependency of their family when they needed it most.

“They refused to acknowledge the severity of what we were facing,” says Ms Smith.

Children who are diagnosed with HLHS must undergo a series of palliative surgeries designed to give them the best quality of life.

The first of these surgeries, the Norwood Operation, was performed when Poppy was only 36 hours old.

She needed urgent surgery if she were to survive.

However, in December 2016, just a month after Poppy’s Fontan surgery, she was readmitted to the RCH, after tests revealed an increased build-up of fluid around her heart.

While other children her age were getting excited about the arrival of Santa Claus and decorating the Christmas tree, Poppy and her family spent the Christmas period in a hospital bed.

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Photo credit: Amelia Smith

The reality for families of children living with HLHS is bleak and appearances are often misleading.

“The oldest survivors with HLHS are only in their 30s. She will eventually need a heart transplant; if a transplant is even an option.”

“For now, she looks like a normal, healthy four-year-old girl, but the grim reality is that she’s a ticking time bomb. We don’t know if she’s going to survive to even be able to have a transplant, and if she does, if it will be in six months’ time or twenty years. It’s all very unknown and we just live cardiac review to cardiac review trying to give her the most normal life possible,” says Ms Smith.

Life has become relatively easier for Poppy and her family since her Fontan surgery last year.

Before the operation, Poppy was always blue in colour and out of breath- both symptoms of the congenital heart condition.

She couldn’t run or play with other kids her age and she would become tired easily.

Now, she doesn’t stop moving from sunup to sundown.

“In the cold, her lips still turn a little blue and she does get a little puffed because her heart works twice as hard, but you would never know just by looking at her if you can’t see her scar,” says Ms Smith.

Poppy also has a mix of three medications that she is required to take every day, including an anti-clotting medication because she has an artificial conduit in her heart.

The medication also comes with its own set of problems, including bone density issues such as osteoporosis and not being able to play contact sports due to bleeding risk.

So just how does a child of Poppy’s age comprehend such a complex disease?

“I have a special heart. I have Hypoplastic Left Heart Syndrome and that’s forever. I’m a HeartKid for life. My heart doesn’t work like other kids’ hearts do. I wish one day they would be able to fix my heart and make it normal,” says Poppy.

HeartKids is a registered Australian health charity that provides essential support for children suffering with HLHS and their families.

Poppy, and kids just like her with ‘special hearts’ are referred to as HeartKids.

Together with the RCH’s Good Friday Appeal, HeartKids has given Poppy and her family invaluable support over the past four and a half years.

“Through HeartKids, I have met many other families with children who have HLHS. Their support inspired me to start a HLHS parents support group online, so that no other parents should feel the way we did when we found out about Poppy’s defect- they don’t have to feel like they are facing it alone,” says Ms Smith.

Both Poppy and her parents would have been lost without the support of the non-government funded charity who rely heavily on the donations of the public and sponsors.

During the most difficult moments of their lives, HeartKids was there to assist the Smith family.

“We wouldn’t have made it this far without the support of HeartKids…they hold morning teas twice a week with support workers there. They can also help with the costs associated with basically living in a hospital, like meal, fuel and grocery vouchers. Plus, the morning teas are a great way to meet other parents who have children with cardiac conditions,” says Ms Smith.

HeartKids works alongside health professionals to provide lifelong support to those with childhood heart defects.

They also advocate for the needs of children impacted by heart disease and provide information and education to families­.

HeartKids states on their website that eight babies are born every day in Australia with a heart defect.

The annual Good Friday Appeal is also an incredibly important time for Poppy and her family.

They are heavily dependent on the services available at the RCH in Melbourne, with many of Poppy’s surgeries and check-ups taking place there.

Fundraising for the hospital is vital, with money raised each year to help support research and provide patients with the most advanced equipment and technology.

In April 2017, the Good Friday Appeal raised an outstanding $17 605 662.

Not only will the investment of this money benefit the Smith family, but also the thousands of other children and their families that are admitted to the hospital throughout the year.

“Without the Good Friday Appeal and HeartKids, the Royal Children’s Hospital wouldn’t be able to provide life-saving treatment and surgery to kids like Poppy. It is the only paediatric hospital in Australia able to do transplants,” says Ms Smith.

The RCH and HeartKids accept donations all year round.

The links can be found below.

Donate now to the Good Friday Appeal

Donate now to HeartKids

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