A variety show called Little Hands, Big Hearts is held every year to help fundraise for charities which in some way support the patients and families of Southampton Children's Hospital. However, with the COVID-19 pandemic, this year will be very different.
With the help of some friends and family, Emma - a nurse on Ocean Ward - has produced a virtual choir performance in its place. This special edition will raise money for Naomi House, Jacksplace and Ocean Ward in memory of one of our patients, Emily.
Read Emily’s story below.
When Emily was born she initially seemed very healthy, but after smiling for the first time at five weeks old she failed to meet any other milestones. Her parents, Adam and Amy, knew something was very wrong.
At seven months old, she had her first terrifying seizure and was later referred to the paediatric neurology department at Southampton Children’s Hospital. Emily started having regular seizures and painful muscle spasms and, following a series of tests, she was diagnosed with Rett Syndrome through whole exome sequencing by the Wessex Genetics team in 2017.
Rett syndrome is a rare genetic disorder that affects brain development, resulting in severe mental and physical disability. It is estimated to affect about 1 in 12,000 girls born each year and is seen only rarely in boys.
Shortly after diagnosis, Emily started choking on milk so had to have an a nasogastric (NG) feeding tube passed through her nose and down into her stomach. She was later referred to our paediatric gastroenterology specialists to have a percutaneous endoscopic gastrostomy (PEG) tube surgically implanted directly into her stomach to provide her with all her nutrition. Unfortunately Emily didn’t tolerate the anaesthetic and had to go to PICU to recover.
She was soon at home enjoying life again and, although her epilepsy gradually got worse, she started attending mainstream preschool. She was always happy and content despite what she was going through. She was learning to use an eye gaze device to enable her to communicate which was loaned to her by Rett UK but as she was doing so well her family knew she would benefit from having her own device.
Emma is one of our nurses on Ocean Ward and has been friends with Amy, Emily’s mum, since they studied paediatric nursing together. Emma put together the first variety show of Little Hands, Big Heart and raised a huge amount of money that enabled the family to buy Emily's very own eye gaze device. This gave Emily a voice which she used to be very cheeky and often to make jokes!
Sadly in November 2018, Emily's condition hugely deteriorated. Her epilepsy became very difficult to control and she spent a lot of time in Salisbury District Hospital.
She stopped smiling, rarely communicated and became very withdrawn. In February 2019 Emily had a seizure lasting over two hours, during which she aspirated and developed pneumonia.
Her condition worsened and the decision was made to intubate her. The Southampton Oxford Retrieval Team (SORT) transferred her to PICU at Southampton Children’s Hospital for a rest.
Emily had some more tests done which showed that she was in a very rare type of status epilepticus called myoclonicus. The neurology team started some new treatment but unfortunately Emily's central brain was so damaged that she couldn't survive without a ventilator. The staff supported Amy and Adam to make the decision to take her to the local hospice, Naomi House, for end of life care.
Amy said: “Naomi House was one of Emily's happy places, she spent many happy times there so it felt right that was where she should go.
“Before Emily was transferred to Naomi House the Southampton PICU staff went above and beyond to care for our sparkly princess. They read to her, did her hair and dressed her (Emily was very particular about how she looked!) and helped us to make memories.
“Even when we had to leave her to prepare our boys for what was ahead Emily was treated like a queen by the nurses”.
The family spent five days making memories with Emily at Naomi House, supported by their wonderful staff. Emily died peacefully snuggled between her mummy and daddy on Thursday, 21 March aged three and a half. She left such an impression on so many people and is missed terribly by everyone who loved her.
This is not the first heartbreak Amy and Adam have suffered as they very sadly lost their second son, Alex, shortly after he was born.
Shortly after Emily passed away, Amy discovered that she was expecting a baby, and in December 2019 Elizabeth arrived. Amy said “We always talk about Emily to Elizabeth, she is so like her beautiful big sister”. Elizabeth thankfully does not have Rett Syndrome.
Amy is very thankful to the team that cared for their daughter adding: “The care Emily received from before her diagnosis, right through to the SORT team taking her to Naomi House for end of life care, was amazing and Adam and I are forever grateful to SCH”.
Posted on Friday 26 June 2020