A heart too big for his body

At 34 weeks pregnant, Charlotte was told her baby’s heart wasn’t developing properly. Consultants at Great Ormond Street Hospital (GOSH) diagnosed her baby, Joe, with Ebstein’s anomaly, a congenital heart defect affecting the flow of blood to the lungs. Here, Charlotte describes how Joe had emergency surgery twice at GOSH.

A diagnosis

“Joe’s movements were fine until I was 32 weeks pregnant. I had scans to check he was gaining weight and they all looked okay. Later, a scan at Norfolk and Norwich Hospital revealed Joe had an enlarged heart. Within a few days, we were taken to GOSH for an appointment with a specialist cardiac consultant.

“The consultant told us that Joe had a really rare condition called Ebstein's anomaly and that his was the most severe case he had ever seen. So many questions and emotions rushed through me and I felt helpless and desperate. We listened carefully to the consultant but it was weeks before the news sunk in; by the third trimester you feel confident that everything is okay.”

Joe’s arrival

“Joe was born after 41 weeks in Norwich. I only saw him for a few seconds before he was taken to the Neonatal Intensive Care Unit (NICU). Seeing him later, covered in all those machines and wires broke my heart.”

Joe was taken to GOSH using the Children's Acute Transport Service (CATS), a specialised service designed to make intensive care rapidly available to critically ill children. Charlotte travelled to London where Joe was being treated at GOSH.

Joe's treatment

“Joe was on medication but he was deteriorating. The Cardiac Intensive Care Unit (CICU) team didn't know what could be done as the usual procedure was too risky and Joe’s arteries were not big enough. It was decided that a catheter would be inserted to direct the blood flow past his deformed heart valves and into the lungs.

“We held a private baptism at Joe’s bedside beforehand. The staff nurse joined us together with a chaplain and few other staff members. It felt incredibly special and spiritual despite the CICU background. The chapel was our place of peace; we felt incredibly close to our faith.

“Joe had the surgery when he was five weeks old, but took many turns for the worse afterwards. He needed blood transfusions and a ventilator to help him breathe normally. After a week, Joe started looking healthier and began to stabilise. We were moved up to Bear Ward, which was amazing as we had our own room, meaning I could sleep in the same place as Joe for the first time.”

For a few months, Joe lived at home where a community nurse visited weekly to take observations. Soon, his oxygen levels began to fall considerably meaning that the duct in his heart could be closing. He had to go back to GOSH.

Returning to GOSH

An echocardiogram (a non-invasive, high frequency ultrasound scan of the heart, also called an echo) revealed a restricted flow through the stent Joe had fitted in September, which was intended to improve the blood supply to his lungs and be his life support. Charlotte and her husband were told not to take Joe home and offered parent accommodation across the road from the hospital.

“We received a call from our cardiac nurse specialist. She said they needed Joe on Bear Ward overnight and that they had made space for him. The team wanted him inside the hospital, despite Weston House, the parent accommodation at GOSH, only being a stone’s throw away. Thank the Lord they did, it was their gut instinct and judgement that saved Joe’s life.”

The following day, Joe had an emergency CT scan.

Joe (photo by Kate Sanderson)

Waiting for surgery

“Joe’s consultant, Dr Giardini, told us in shock that the scan results showed Joe had a clot or tissue obstructing his stent. He was put on a heparin IV (a blood thinner used to prevent blood clots) and monitored every 15 minutes."

At 2am that morning, a nurse doing Joe’s observations noticed that his oxygen saturation was dangerously low. The Cardiac team were called in the middle of the night to meet and decide if Joe could have the surgery (known as a Glenn procedure) he desperately needed in order to help his heart pump oxygenated blood to his body.

Charlotte said: “They needed to be sure and accurate as this was an unexpected complication and Joe could have a further deterioration at any time.”

“We were desperate when the time came to take him to theatre, but Joe was awake and even smiled and gurgled before they put him to sleep for the surgery.

“My husband and I waited for news silently for hours. When the call came we ran to CICU to see him. He was asleep, fighting for his life. We watched him for three days.”

After a few days on CICU, Joe was moved back to Bear Ward, where the family had stayed on their first visit to GOSH. Charlotte said: “It was surreal as we had been there before and we were worried to leave the intense safety of CICU. We were on Bear Ward for three weeks but there were still challenges. Joe had a reflux problem, changes in medication and feeding problems after a feeding tube came out and we had to encourage him to breastfeed again.”

Family balance

“My focus was Joe, but while he slept in hospital I’d spend time with our toddler, Oli, in the playroom nearby. Oli couldn’t bear to see his brother in the cot and understandably didn’t want to be near the bedside. My husband spent a lot of time with Oli when I was with Joe, and the hospital staff were great too.

“Day by day, Joe got stronger and consultants were happy he was stable. Gradually, lines and wires were removed. The feeling of being able to walk Joe around the ward and then out of the hospital was overwhelming.

“Joe is doing incredibly well now. All of our praying, wishing, belief and encouragement has kept the positive energy and really helped.

“Thanks to GOSH, Joe can now play with Oli. We’ll keep fighting and loving and taking each day as a gift. We had the best Christmas ever as we had our beautiful boys together. We will be indebted to GOSH and the Cardiac team forever.”

My advice to others

“Don't be afraid to ask the hospital for support. Go to the chapel, go and speak to a psychologist, ask the doctors to explain everything to you. It will make you stronger for your child.

“If someone says they can do something to make your life easier, make the most of it because it helped us so much. Most importantly, have faith and hope and be positive – everyone at GOSH has your child’s best interests at heart.

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