Evie was a happy and extremely sociable, friendly girl who loved life – she was a participant, not a spectator.
Evie had a progressive neurological disorder known as Labrune syndrome (leukoencephalopathy). The condition caused a gradual deterioration in her health and sadly, in June 2020, she passed away at the age of just 12. Her dad, Simon, has bravely shared the last few days of her life.
“Evie’s health had slowly been deteriorating for the last few years as Labrune Syndrome began to take hold. More recently during lockdown, she had a few instances of sickness. We put this down to her diet.
"At about mid-afternoon on the 16th June, after a 'normal morning', she started crying when we were outside. I mistakenly thought she was too hot so we moved into the shade and then onto her bed so she could rest. Soon after, she started to be sick. We were concerned at this point, but as she often went through periods of sickness, we were just keeping an eye on her. Her condition deteriorated rapidly and we noticed that her eyes were looking in different directions and a rash developed on her body just before she was about to be sick. It was then we dialled 999. They came and assessed her and left it down to us if we wanted them to take her in to the Queen Alexandra Hospital. She then vomited again whilst they were there and they decided she should go in.
"As we were about to leave the house we gave her emergency meds to settle her down. Very soon on reaching QA things took a drastic turn for the worse. She stopped breathing and staff gave her CPR, eventually under sedation putting a tube down her throat to help her breathe. The CAT scan showed enlarged ventricles, so we were transferred to the Paediatric Intensive Care Unit (PICU) in Southampton. Two drains were inserted to help alleviate the pressure on her brain that was caused by the cysts which had been forming in her brain due to the Labrune’s syndrome.
In my mind, we lost her that night, as she never regained the ability to hold our hands or smile at us which, to those of you who knew her, was a big part of who she was.
"After an MRI scan, we (Caroline, Isobel and myself), were taken to a room with lots of professional people in to be given the bad news. The damage that had been caused in this recent episode was catastrophic, meaning her ability to breathe for herself would probably not return and she would, as a result, have more brain damage than previously. She was stable but very poorly and the decision was left in our hands; to wait and see what happened, or to withdraw life support. During this time, PICU staff were brilliant in all aspects. Attentive to our needs and did everything to accommodate us, as much as they could with respect to Covid.
As a family we made the incredibly difficult decision to withdraw life support. This was not a life that Evie would want. She was always at the centre of things and loved to be involved in everything. She was a participant in life – not a spectator.
"We had decided to donate Evie’s organs, so on Tuesday morning, we said our final goodbyes to Evie in the theatre anteroom, so that as soon as she passed away, they could take her into theatre to start the process. The ventilator was removed and we spent our final few minutes together.
'We left Evie in theatre and made the journey to Naomi House where we were welcomed with a hot lunch, none of us wanted to eat, but it was just what we needed.
"The hospital brought Evie to Naomi House after the operation had finished. We were thankful to Naomi House for allowing us to stay in the room across the corridor - we were able to go and spend time with her at any time we wanted to. We could just go and sit with her or spend time together as a family and begin to think about what happens next.
As always, Naomi House staff were very understanding leaving us time to grieve as a family unit, but were on hand if needed.
"They were able to provide us with some resources regarding funerals, readings, poems etc which was really useful. It’s not the sort of thing you know anything about.
"When we were ready, they made the arrangements with the funeral director to come and collect Evie. We then made our way home – something which we were dreading.
"We had a cremation for Evie and were allowed 30 people at the ceremony, which was lucky, as the previous week it was only 18 (due to Covid 19). This meant all of the family could attend, however, Evie was so popular, we would probably have had nearer 100. People were waiting to see the funeral cortege near Park Gate School with bubbles and windmills, there were lots of people outside the crematorium, including therapists and staff from St Francis School. Also for those who could not be there, it was broadcast online.
"Three months on and it has not got much easier to accept, but she has left us with lots of happy memories of good times."
Naomi House & Jacksplace are there for families when they just need a break, knowing that their children are safe and having their own special holiday. We are there when families need someone to talk to, when life can seem just too difficult. And we are there when that last day comes.
In 2017, Simon shared their family story with us...
Evie lives with her dad, Simon, mum, Caroline and 12 year old sister, Isobel. Due to the complexity of her progressive neurological disorder, Labrune Syndrome (leukoencephalopathy), it took doctors a long time to be able to give a diagnosis, but it has now been established that Evie’s condition is caused by a faulty gene. Evie is unable to walk or talk and is fed by tube into her stomach.
Simon told us:
“It's good to have a diagnosis, but it actually doesn’t change anything. We, as parents, deal with whatever comes our way. Evie’s condition presents similarly to cerebral palsy, but it is progressive and she will likely deteriorate.
“When she started school, she could walk, talk and feed herself. She had splints on her limbs to support her feet, ankles and wrists. As time went on we stopped using the splints because we couldn’t physically get her feet in them. Now she is unable to walk. She used to feed herself with finger foods like pasta, but she has lost the ability to do that. She lost the manual dexterity to be able to pick the food up and get it in her mouth. In addition, she had no concept of how much she should put in her mouth and she could choke.
“Sometimes when things change I wonder if she is just having a bad day or if this is the next step in her deterioration. She can do very little for herself and her verbal communication is virtually non-existent. This time last year she could talk and chatter away, but now we get maybe one word a day if we are lucky. On a positive note, Evie is an extremely sociable, friendly girl who loves life."
“Evie absolutely loves Naomi House. When we left her for the first time we thought, what have we done?! Then we realised she's in a place where there are people who are even better placed to look after her than us! She has so much fun there.
We say we’re going to Naomi House to see her friends and there's a big smile on her face. We say we’re taking swimming stuff, and there would be another beaming smile on her face.
"There are lots of accessible things to do there and she’s got constant one to one attention, which she loves. We know that Evie is going to be looked after well, if we didn’t think that, we wouldn’t let her go. I love going to pick her up because there is always a big smile on her face and you know she's had a good time.
"She has a good understanding of most things going on around her. She doesn’t think she’s different from the other children, which is lovely. As long as she is involved with the other people and doesn’t get left on her own, then she is ok.
Evie is full of beans and everything is a laugh. She has such a positive attitude and she doesn’t moan - she is a little trooper.
“As things deteriorate, you think what is going to be next? We can see the effect it has on people who haven’t seen her for a while and it makes us realise that yes, she is getting worse, quickly. We try to shut off that side of things and think right, let’s deal with what we’ve got, otherwise we wouldn’t get through the day. You try not to think long-term about how it's all going to pan out.
“When Evie stays at Naomi House it just gives us a welcome rest where we can do things with Isobel and be like a ‘normal’ family for a bit. We know she is in a place where she will be looked after by professionals who are there for her 24/7 and don’t have to go off to do the cooking, don’t have to worry about what’s in for tea, and don’t have to worry about another sibling. It eases things for a few days and we can even make a few plans to go out as a couple. It gives us a chance to wind down and ‘breathe’ for a bit. Sometimes we just need a break for a couple of days to re-charge the batteries.
Knowing that we’ve always got that buffer and a bit of chill time really helps, it’s our salvation. There is always help for the whole family.
“We live day by day. We try not to think too much about the future - we deal with Evie’s situation on a day to day basis.
"Without Naomi House and the dedication of the staff, we would not get an opportunity to spend some quality time with our other daughter, Isobel, and do activities which other families take for granted."
Thank you so much to Simon and his family for sharing their family story with us, and for bravely sharing her last few days with us.
