Lily Gauthier-Chapman has been a patient at Royal Brompton since she was born in 2001. Her mother, Leah, explains: “I gave birth at Epsom Hospital and everything seemed fine, but Leah had problems feeding, and then became quite breathless and blue.”
Tests showed Lily had a cardiac problem so, at around 12 hours old, she was rushed to Royal Brompton, where she was diagnosed with pulmonary atresia with intact ventricular septum (PA/IVS).
The condition means Lily’s pulmonary valve, which allows blood to be carried from the heart to the lungs, is blocked, resulting in a low level of oxygen in the blood.
“It was a very scary time, but I was relieved that her condition had a name,” says Leah. “I’d never heard of congenital heart disease before. When she was diagnosed, the doctor drew a diagram and explained the differences in Lily’s heart structure and what he was going to do to alleviate her symptoms. He gave me all the information I needed and made me aware of some of the challenges ahead.”
Lily had surgery at just a couple of days old to create a hole where the pulmonary valve should have been and to implant a shunt (surgical connection) between her aorta and pulmonary artery. This was a temporary measure until she was nine, as by then her heart had grown, so the shunt was closed and she had a stent (a wire mesh tube) inserted into her right pulmonary artery to keep the blood flowing. The procedure involved inserting a catheter (a long, flexible tube) through her groin and up to her heart. She had another stent inserted in 2014 at just 13 years of age, and her next operation will be a valve replacement, which should take place in the next year or two.
“Lily is now 15, nearly six feet tall, and has led a relatively normal life,” says Leah. “She finds cardiovascular exercise a bit of a struggle and prefers not to take part in competitive sports or cross country running, but she enjoys swimming and can join in with her friends at most things. She does get out of breath sometimes, but says she feels OK. We’ve had a few calls from the school over the years when she’s felt faint and had to sit quietly, but nothing serious.”
When children with congenital heart disease reach 16, they move over to the adult CHD service, and Royal Brompton runs a unique nurse-led transition service to help make the move as smooth as possible.
“Lily has already had two meetings with the transition nurse,” says Leah. “Up until now I’ve been managing her health, but now it is important that she begins to manage the condition herself. At the moment I have to remind her to take her aspirin (which helps prevent blood clots) but she’s soon going to have to start taking much more responsibility.”
Lily adds: “The meeting with the transition nurse was really useful. We discussed what was different with my heart and talked about blood sugar levels and breathlessness, and how I should always let someone know if I’m not feeling well. She also talked about the dangers of alcohol, smoking and drugs. And she helped me focus on what I need to remember, like taking my aspirin!
“I feel ready to move to the adult services, and I’m pleased that I’m staying at the Royal Brompton, as I feel really safe there and I know they give me the best care possible. I’m glad I don’t have to move hospitals, and it’s good to know that the doctors who operated on me as a child are still involved in my care.”
(Image right: Lily (top) today and aged three with her mum Leah)