Family in need of a heart of gold

AMY Miller is an elfin-faced child who loves to chatter, dance and paint. She skips down the passage of her

home to fetch toys from her bedroom – but expecting her to walk further than a few metres is

difficult and her mother Natasha has to carry her.

Amy, who will be four in September, has a congenital heart defect (CHD) that makes the normal rough and tumble of childhood impossible. She has less energy than other children her age. Her parents, Natasha and

Michael, have to keep a watchful eye for telltale signs of blueness, which indicate that her oxygen

levels are not optimal. Born with a poorly developed heart, she has had three operations since birth to assist an underdeveloped left ventricle to do its job. But in a few years, she will have to face the only thing that will

save her life – a heart transplant. CHDs affect one in 100 babies, yet many parents know nothing about them until a child is diagnosed. Natasha would like more parents to be aware of CHDs because early detection and treatment offer the best outcome. She believes that pulse oximetry screening (a test to determine the amount of oxygen in the blood and the pulse rate) should be compulsory for newborns. She and Michael have joined the Hudson Initiative, which was started in Johannesburg by Andrea Slater

whose son, Hudson, died of CHD at four months. They are also lobbying for a 20-week foetalassessment scan to check the development of babies’ hearts. “Many people have no idea about CHD and it is only when symptoms present days or weeks after the baby is born that it is picked up,” says Natasha.

“We believe this simple test should be routine so that heart abnormalities can be detected and

treatment started soon after birth.” Before her 20-week scan,

Natasha had no clue that her baby had a heart problem. The scan showed the left side of the heart to

be smaller than the right, but further detail could not be seen. As the baby grew bigger, the heart’s malformation could be seen more clearly and at 32 weeks, a narrowing of the aorta was

detected. Doctors thought it would be correctable after the birth and at 34 weeks the family headed to

Johannesburg so the baby could be treated by a specialist neonatal

cardiology team after delivery. Detailed scans showed a narrowing of the aorta and stenosed (narrowed) mitral valve. The left ventricle was underdeveloped.

Then medical aid issues cropped up, meaning the baby would have to be treated in Cape Town’s Christiaan Barnard Hospital. Because of her advanced pregnancy, no airline would carry Natasha. “We drove from

Johannesburg to Cape Town, with nowhere to stay. We knew we had to get there fast.”

Natasha says it turned out to be a good move as the care they

received at the hospital was superb. Amy was delivered by C-Section at 38 weeks. “She weighed 2.8kg, she was beautiful – but she looked blue. She was whisked away to ICU and she hung in there. It seemed that

every time we saw a doctor, the news was worse. They could not say whether she would survive.”

Amy was diagnosed with Hypoplastic Left Heart Syndrome. At six days, she underwent a nine-hour

operation. After 45 days in hospital, the family was given the go-ahead to take her home. “We had a good few months as a normal family. Amy progressed within her limitations and we had to check her oxygen levels by way of a monitor on her finger or toe. But in January 2011, when she was four months old, we

needed to go back to Cape Town as blood flow to her lower extremities was impaired.”

Amy had a nine-hour operation and she recovered well. She developed better muscle tone and had more energy. Because of her physical limitations, her cognitive skills and vocabulary surged ahead. She started talking at eight months but crawled at a year. Soon after her first birthday, Natasha and Michael noticed an

ominous blueness. Doctors were reluctant to operate until she was about two, but she lacked energy, indicating a shortage of oxygen. After her second birthday, and a five-hour operation in Cape Town, her colour was better and she had more energy. A second baby, Layla, was born – with a hole in the heart. However, this closed and she is now free of heart problems. Just before Amy’s third birthday, Natasha and Michael noticed “whole-body breathing”, as though she were struggling for air. Her colour was poor and the family raced to Cape Town. Heart failure was diagnosed. Her echocardiogram showed cardiac shunts were working but the heart was under strain and was nearly double its size. She responded to anti-failure treatment and the family was sent home to manage her with medication. After an assessment in February this year, the news was grim. Pulmonary arteries were not growing and the right ventricle was not strong enough for the next operation. The only option would be a transplant. “It was terrible news,” says Natasha. “I was angry and scared.” She and Michael know they have no option but to accept this. Amy could go into heart failure again at any moment and the hope is to get her to 10 years before it becomes essential. “It’s a waiting game for us as it is an inevitable step for Amy. Surgically no more can be done for her.” Her parents, and the extended family, want her to enjoy to her childhood. Amy is aware of her limitations. She tells her parents when her nails are blue and says when she is tired. She takes medication daily as part of life. “So, for the next few years, we have to try to make her life as normal and as happy as possible,” says Natasha. www.heartkids.co.za