Ellie M O’Neills Story: Pulmonary Atresia

Northern Ireland

“Hello, my name is Ellie Maureen O’Neill. I was born at 8.38am on a Tuesday near the end of October 2006 by emergency section as my heart rate had slowed down. I spent my first two days in the neonatal unit in Craigavon Area Hospital. When I was six weeks old, the doctors found out I was sick and rushed me down to Clark Clinic in The Royal Belfast Hospital for Sick Children.

I am a wee miracle, as my Ductus Arteriosus stayed open for those six weeks and kept me alive. Usually it closes a few days after birth. My Mummy and Daddy met with Dr Frank Casey the Consultant Cardiologist. He explained that I was a very sick wee girl and had complex congenital heart disease. I have Pulmonary Atresia, which means that I have no Pulmonary Artery, so blood from the right side of my heart cannot go to my lungs to pick up oxygen. This means that I go blue a lot, like a smurf!!

I also have a double-inlet single ventricle and major aortopulmonary collateral arteries (MAPCAs) . Normally, the heart has a right and left ventricle. The right ventricle normally pumps blue blood (without oxygen) out of the heart through the pulmonary artery to the lungs for oxygen and the left ventricle normally pumps red blood (with oxygen) through the aorta out of the heart to the body. Double Inlet Left Ventricle is one of the so-called Single Ventricle heart defects, as there is effectively only one pumping chamber in the heart.

Operation Number One - BT SHUNT:
I went to Clark Clinic on Friday 1st December 2006. The doctors gave me prostaglandins to keep my Ductus open until they were ready to operate on me. I had my first operation on Wednesday 6th December 2006. It was called a Blalock-Taussig shunt. Mr Gladstone was the surgeon. He was a very nice man with big, long fingers! He is very good at his job and I am glad he operated on me. I was away for about five hours and Mummy and Daddy were very worried about me.

After the operation I went to Paediatric Intensive Care to recover. I was so small in the cot and I was wired up to lots of machines. Around 9pm that night my oxygen levels began to drop. They were sitting at 80/90% and the doctors were very happy, but then they dropped down to the 40′s and 50′s. My shunt was blocked by a clot and I was dying. Dr Sands, another cardiologist spent hours working on me and told Mummy and Daddy to expect the worst. He went home after 2am and I was stable for the time being. I went back to the operating theatre the next morning to get the clot unblocked. This time it worked great. Mr Gladstone was very upset that it hadn’t worked first time round and could not understand how I made it through the night. I was in PICU for five days and then went back to Clark Clinic and finally got home on the 20th December. I was in great form and had a big scar to show everybody (thoracotomy).

Operation Number Two - The Glenn Operation:
I went back to Clark Clinic on Wednesday 4th July 2007 for a cardiac catheterisation. I was in for three days and it was to make sure I was ready for the second stage of my surgery, the Bidirectional Cavopulmonary Shunt or Glenn Operation.

The Glenn operation is open heart surgery and I underwent it on the 17th January 2008. This operation diverts all the blood coming back from the upper body through the Superior Venacava. Now, all of the blood coming from my upper body is diverted to the lungs so more pink blood is coming back from the lungs to the left side of my heart. They also removed my old shunt. The operation took five hours and I was home after 6 days, which amazed the doctors and the nurses.

Operation Number Three - The Fontan Operation:
Operation number three is the Fontan Operation. I will need to get this when I am three or four. I will have to get it sooner than later as I am very tall for my age and this puts extra pressure on my heart. It is the last of the three planned operations and will make me nice and pink!!

The Future:
Ten years ago, there was little hope for children like me. Recent advances in medicine have enabled me to live a much more fulfilling life. I will be on anti-coagulants for the rest of my life (I am on aspirin now and will be on warfarin later). This stops a potentially fatal clot, but unfortunately long-term use has its side-effects too.

To look at me I look like any other child of my age. People comment on my blond curly hair and my height. They don’t see my lips going purple and my body of scars. I often forget my physical capabilities and tire easily. I love to play with other children and do not realise that this scares the wits out of my mummy and daddy. A simple blow to the chest when playing can be very dangerous for me although it doesn’t stop me enjoying life to the full.

The doctors say I will need a heart transplant in my late teens. I try not to think that far ahead and take inspiration from other CHD patients who have done amazing things with their lives. ”

Wrote by Sinead O’Neill

Sinead O’Neill has kindly given CHD-UK permission to use the photograph on this page.