Daily life for one Dorset mother would be an impossible challenge if not for the care and support of her local children’s hospice, Naomi House.
Sally Amos lives in the village of Child Okeford, near Blandford, with her daughter, Ella.
Ella has a rare illness that means she is unlikely to survive into adulthood. Sanfilippo B syndrome (mucopolysaccharidosis IIIB, MPS IIIB) is caused by a deficiency in an enzyme involved in breaking down waste. The condition leads to degeneration of the central nervous system with progressive dementia, often combined with hyperactivity and aggressive behaviour.
Doctors and specialists are unable to say with any certainty how long Ella might expect to live for. Their best guess is that she would survive into her early to mid teens but nobody can offer any guarantees and the condition could worsen at any time without warning. There is no cure available, nor are there any medicines available to manage the symptoms. As it is such a rare condition, (affecting around 1 in 84,000 children) there has not been the same level of research conducted into Sanfilippo B as there has with more common illnesses.
Ella is fed with a gastrostomy tube, allowing food to be fed directly into her stomach.
Ella’s mum, Sally, said:
“I love Ella so very much - she’s my world but there’s no denying that life can be difficult. Ella is now starting to slow down and has become less mobile and has lost her speech."
I have carers that help me with Ella as she now needs one to one care. I come to the hospice with Ella now so that we can enjoy quality time together. Naomi House is so important – I don’t know what I would do without them.
Sally found out about Ella’s condition after medical tests were conducted to investigate Ella’s hearing difficulties. After a series of tests, including an MRI and deep urine tests, Sally was given the crushing news that Ella had Sanfilipo B. It was shortly afterwards that Ella was referred to Naomi House aged two and a half.
“The scariest thing for me is that Ella’s health could suddenly start to deteriorate. But I take comfort from knowing that Naomi House will be there to support me for as long as I need. Accepting that you need the help of a hospice is a big step but I haven’t looked back.
It’s not until you go there that you realise what Naomi House is all about and see the huge number of children, young adults and families that they help. It is an unbelievably wonderful and moving place and that first visit is a memory that will stay with me forever.
“For me, the respite is absolutely invaluable. It is hard for people to comprehend the importance of a good night’s sleep. Ella might sleep for just 30 minutes then wake up again. The only opportunity I get to sleep right through the night is when Ella is being cared for at Naomi House - I just couldn’t cope without it.”
Naomi House and Jacksplace are the only hospices offering a 24/7, 365 days a year service for children, young people and families living in Dorset.