This is my amazing 4 year old nephew, Jeffrey. He is a very brave little boy, who has unfortunately been one of a very small number of children diagnosed with the rare CLN2 Battens Disease. We were given the horrific news on Thursday the 6th May 2021. About 5 children per year in the UK are diagnosed and there are about 30-50 estimated affected children in the UK.
This is an incurable, life limiting neurodegenerative disorder, that first presented itself as seizures about 18 months ago. The disease will mean that Jeffrey will soon start to lose his sight, speech, control of his body, ability to chew and eat, cause dementia, he will need a feeding tube fitted and it will ultimately take him from us. Children diagnosed have a life expectancy of 6 - 12 years of age and generally at age 6 require full time care.
Not only is this physically and emotionally crippling, but it also comes with a hefty financial impact too.
Jeffrey is going to be travelling from Accrington, Lancashire to Great Ormond Street Hospital (GOSH) next week for an MRI scan to decide where a shunt will be fitted in his brain. Then every 2 weeks for the rest of his life he will need to travel to GOSH for an injection, into the shunt, to hopefully slow down the awful effects of the disease. Unfortunately this will not put a stop to the progression of Battens.
Thankfully the operation itself has recently been made available on our amazing NHS! However Jeffrey will also soon need various specialist equipment, aides and adaptations in all aspects of his life, to help him sit up, bathe, eat, sleep, walk, travel etc.
His family (Sheryl, Andy and Jeff) will also soon have to move, most likely into a bungalow, to facilitate their new housing needs that his care will require. They currently live in a small 2 bed terrace house they rent that is accessed by stairs, there are also 2 sets of stairs inside and this simply isn’t going to be practical and they need to move before the end of the year. Any new home they are lucky to find will then need to be altered (door widening, wet room, installing ramps, providing suitable floor surfaces etc). The finances we have to get together in the next month or 2 to simply get him to and from his appointments is frightening, never mind what is coming. It will cost about £200-£250 each return trip to London alone.
Along with all the looming massive changes in their lives, my sister and her partner Andy, probably have 6-12 months to maximise what limited time they have left with Jeffrey to make some special memories. We would also like to make his 5th birthday (in July) extra special as it may be his last where he is mobile!
We will be forever grateful for anyone who can spare whatever money they can to help them! Funds are needed immediately and during the coming years! They will be used to help the family with travel costs, bills (given the time out of work for appointments and when one of his parents stops working to care for him), go towards uncovered costs of aides, equipment, generally giving Jefffrey a better quality of life, making some special memories, housing and ultimately funeral expenses for when we lose our Jeffrey. There may be some help from the local authority in terms of grants, but they are unlikely to cover everything, or the cost of their regular needs as the disease progresses.
Please also follow Jeffrey’s journey and help us raise awareness of this horrific disease. Hopefully one day there will be more advanced treatment available that will give children a better quality of life or even be a cure.
Thank you for reading and any help you are able to donate, Louise (Jeffreys Aunty)
Further information can be found with the Batten Disease Family Association (BDFA)