“Being a parent, all we want to do is protect our children and tell them everything will be OK. I can only watch this disease slowly take my little boy away from me.”
This is the harsh reality faced by Naomi as her four-year-old son, Rupert Wright, has severe mucopolysaccharidosis type II, more commonly known as Hunter syndrome.
A multisystemic disease, touching just one in 100,000 male births, Rupert is affected in almost every way imaginable, from his growth and physical appearance, brain and intellectual ability, eyes and ears to his windpipe and nasal passage, heart, liver, spleen and bowel, and bones, joints and skin.
He is at major risk if he ever needs general anaesthetic and his care is extremely complex. He will need more and more as he gets older and the condition continues to take hold. There is no cure, but Rupert does receive weekly enzyme replacement therapy, delivered at home in Suffolk’s Walsham-le-Willows by a specialist nurse.
Naomi will never forget receiving Rupert’s diagnosis. She said: “It was by far the worst day. I was given Rupert’s diagnosis over the phone by his consultant at the Norfolk & Norwich University Hospital on 13 October, 2016 and from that moment my life changed. We were asked to go in the next day for a meeting to discuss what would happen next. I remember sitting in a room with no windows and a, very aptly, box of tissues on the table. Rupert's wonderful consultant spoke kindly to us and did his best in every possible way, but still had to talk about the harsh reality that was about to start a new path for all of us. Lots of things were discussed, mainly that Rupert would be under the care of Great Ormond Street Hospital. We were also told the life expectancy of children with the severe form of Hunter syndrome [is 10 to 20 years] and, very simply, to just enjoy every moment with him.”
Naomi, 32, who works for her parents’ office cleaning business when she is not caring for Rupert, and Rupert’s sister, Alice, six, who goes to Gislingham CE Primary School, are helped to do that by East Anglia’s Children’s Hospices (EACH).
“We receive a lot of care and support from EACH,” Naomi explained. “Rupert receives short break care. We also attend a support group for children under five and Alice goes to the Sibz groups in the holidays. I receive counselling, too.
“They’re all beneficial for different reasons and are greatly needed. We very much appreciate everything EACH does for Rupert.
“EACH makes a huge difference and, honestly, I wouldn't want to be without the care and support from everyone who helps.”
Naomi describes Rupert, who goes to nursery at Riverwalk School in Bury St Edmunds, as “a very loving and cheeky character”. He likes being outside and enjoys tractors and motorbikes.
EACH is currently in the process of moving to its newly built hospice, The Nook in Framingham Earl from its previous Norfolk hospice in Quidenham. The Nook will transform children’s palliative care in Norfolk.
“My most treasured EACH memory was when Rupert got excited about going to Quidenham,” said Naomi. “He realised where we were when we turned down the drive, got very excited and couldn’t wait to go in and start playing, making himself at home. This was a massive achievement as he was very uncertain at first and it’d be quite difficult leaving him. Jo, his play worker, had worked very hard to understand Rupert's needs, the way he communicates, what he likes and doesn't like, what upsets him, etc.
“There will always be something very special about Quidenham to me, but the nook is really exciting. We’ll really benefit from the nook, as will all families at Quidenham. It’ll be an amazing place for the children and their families, and the staff. It’ll make caring for the children more efficient and having the space will be amazing. I’m most excited about the hydrotherapy pool, as this will be a great help for Rupert as his joints get stiffer. The amount of effort that's going into it really is amazing.”