Rebecca

Alarm bells started ringing on Monday when she was sick over breakfast. Sue and Nick took her to the doctor’s surgery just round the corner but it was closed due to it being a bank holiday. They rang the emergency number and were told to take Rebecca to the nearest out-of-hours medical centre. Speed was of the essence but pushing the wheelchair from the cottage, situated near the quayside, up the extremely steep hill to where the car was parked proved hard going. Rebecca didn’t complain. She just looked forlorn and languid.

Her condition suggested there was a need for urgency, although Sue and Nick hoped that the necessary medication would have the desired effect. But during the journey to the medical centre she started to turn blue and was going in and out of consciousness. All four adults were trying to keep her awake.

“I knew there was something really wrong,” Sue admitted. “She wasn’t really responding to us. We were chatting to her, she was cold and it was scary. When you are with other people you try to be calm and composed, but if Jennie and David hadn’t been there I don’t know what I would have done. Inwardly, I was beside myself with worry, but you don’t show your emotions and you don’t want other people to panic. I was concerned that if Rebecca saw us panicking she would have realised something was wrong.”

On arrival, Nick scooped Rebecca up and rushed into the centre with her. Staff took one look at her and immediately telephoned 999. “The receptionist and nurse saw there was a dire problem, and went running off for an oxygen machine,” said Nick. “They administered oxygen and she did recover a little, but their concern was obvious.”

Within minutes an ambulance arrived and Rebecca and her mum were ushered into the back and were told they were being blue-lighted to the hospital in Truro. Nick, Jennie and David followed, as best they could, by car. It was a nerve-wracking journey. Rebecca was being treated as the ambulance raced along the busy roads, severely congested by bank holiday traffic, while mum comforted her little girl, at the same time trying to be oblivious to the speeds they were travelling.

Sue continued: “The siren was blurring, they were asking me all sorts of questions and Rebecca was getting agitated because obviously she was struggling to breathe, and as she was struggling she was becoming more worked up. Her arms and legs were flailing. It was an absolute nightmare. I am not the best of travellers so I was feeling ill myself roaring along in the back of an ambulance at goodness knows what speeds. I was worried about Rebecca but also worried about being sick in the ambulance when I was not even the patient!

“It was a great relief when we got to the hospital, although I had no idea where Nick, Jennie and David where, how far they were behind us or anything. We were rushed through to the A&E and a whole team of people started working on Rebecca. She hadn’t lost consciousness, but wasn’t far off it, and they were firing questions at me. I was trying to answer as concisely as possible, but we were still very new ourselves to what was happening with Rebecca and, of course, they hadn’t heard of Leigh Syndrome. Then they asked me to leave the room - it didn’t make much difference because Rebecca wouldn’t have known if I was there or not - but no-one knew her and I just felt helpless. I wasn’t able to do anything for her. It was totally out of my hands. I was the one with the information and I was hoping I was giving them the right information. But they kept coming out asking me other questions and I would just say ‘ring Alder Hey, they have her notes,’ and eventually they did. When Nick finally arrived he tried to answer questions but they put Rebecca on a ventilator because they thought it was the best thing they could do.”

Rebecca was taken to the hospital’s intensive care unit and a doctor contacted Alder Hey to become more familiar with her background. A decision was taken to evaluate the situation over 24 hours. One logistical problem was that Truro had only two paediatric intensive care beds so there was talk of transferring her to Bristol - the nearest specialist children’s hospital.

Sue and Nick were hoping for any sign of improvement, however slight, in their daughter’s condition and it came during the night when staff told them they were taking Rebecca off the ventilator and sedating her. It was a bit of good news as they tried to settle in a side ward and get some much-needed sleep. But the respite proved all too brief.

“About 10.00am the next morning, after being off the vent for some hours, she was sitting on my knee and we were reading a story and she started to become very lethargic,” stated Sue. “We had just seen the Registrar who had checked her chest and listened to her heart and told us that she would be taken to the high dependency unit. But a few minutes later she was just like a rag doll in my arms. We don’t know whether her heart had stopped, but she was taken out of my arms and they started working on her straight away. They threw us out of the room and I don’t know how long it was before we went back in, but she was back on the ventilator and fully sedated.”

For the parents it was like being back on the rollercoaster, up one minute, down the next. Nick alluded to that when the latest setback occurred. “I remember the nurse came and told us that she was sitting up and asking, as she always did, for food. We were encouraged by that and we went to see her, but suddenly it all changed and nursing staff were dashing round and working hard to steady her oxygen saturations which had gone down to a dangerously low level. It was so traumatic and standing there watching it going on was mind blowing. After she was stabilised I left the room for a few minutes. I had to. I just completely and utterly dissolved because the thought of what might happen was too much to take. They admitted to us after stabilising her that they thought they were not going to be successful.”

But within a few hours came a dramatic and unexpected twist in the crisis. Alder Hey’s ICU department, having discussed Rebecca’s condition with their Truro counterparts, agreed to send a special plane to air-lift her back home. The news was met with tremendous relief by her parents, who later discovered there was one shock drawback in the arrangements - there was only room for one of them on the flight back to Speke Airport (now Liverpool John Lennon International). After a brief chat it was decided that would be Sue, although she was almost traumatised by the thought of flying at several thousand feet in a small aircraft.

“Once again, I felt sheer panic,” Sue admitted. “We would be leaving Nick to drive home, which he was more than capable of doing, but Rebecca being flown home in a little plane, being looked after by medics, was a little too much to take in.