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A cruel fatal illness that recently deprived a four-year-old Devon boy’s ability to speak and much of his ability to move will also deprive him of sight unless his parents can fund treatment not available on the NHS.

Elliott Jackson, of Bovey Tracey, was diagnosed with a rare genetic disorder, Batten’s disease, similar to dementia, six months ago. Unfortunately, there is no cure.

Previously, it was believed that he suffered from severe autism, as the symptoms are similar to those of the disease. It is estimated that around 100 to 150 people in the UK are living with the disease.

Symptoms include increasing visual impairment leading to blindness, severe seizures, decline in speech, language and swallowing, deterioration of fine and gross motor skills which leads to loss of mobility and difficulty sleeping .

Elliott Jackson was diagnosed with Batten disease

Since last Christmas Elliott is now unable to speak and has lost much of his ability to walk. However, since receiving a treatment called Brineura infusions in his brain at Great Ormond Street Hospital in London, he has regained some mobility.

Ongoing global trials are pioneering the use of Brineura in the eyes to prevent sight degeneration in children.

The Great Ormond Street Hospital is able to offer the treatment, but is unable to fund it as it is currently not available on the NHS.

Elliott’s parents, Rob and Claire – who also have another daughter Faye, six, who does not have the disease – are working on a collaborative fundraiser with other parents across the UK who have children also in need of treatment.

The joint fundraiser has so far raised over £ 100,000, of which the own fundraiser has generated almost £ 38,000.

But since the cost of the full treatment is unknown, more donations are needed to try and give Elliott the best quality of life for as long as possible.

This Friday April 9 at 4:30 p.m. Rob will be participating in a 24 hour Batten Royale live game event to raise funds.

He said: “On October 21, 2020, we received the devastating news that Elliott was suffering from CLN2 disease, also known as late childhood Batten disease.

“This disease is incredibly rare with around five to six children diagnosed in the UK each year. Batten disease is comparable to dementia, but primarily affects children.

“Most children with CLN2 disease do not live beyond 10 years of age and rarely see their teens, losing all ability to communicate, see, walk and generally take care of themselves. There is no cure.

Elliott Jackson with his father Rob
Elliott Jackson with his father Rob

“While Batten’s disease has changed the course of our lives, it also provides an answer to why Elliott was not developing like other children his age.

“This explained his inability to learn to speak; leaving him with less than a handful of words to rely on. This explained his uncontrolled seizures which began a little over a year ago. His mobility deteriorates and he falls often, every day.

“Although Elliott is four years old, he is much younger developmentally.

“Fortunately, we are able to get Elliott a new treatment – unique to CLN2 disease – that will dramatically prolong his life by slowing or, hopefully, preventing even his brain from degenerating further.

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“It requires us to go to Great Ormond Street Hospital every fortnight so that Elliott can receive an infusion of Brineura – an enzyme or protein that he cannot produce – directly into his brain.

“Treatment is not a cure, and will not allow him to ‘catch up’ or live normally. He will continue to have complex needs, however, this will allow us to provide him with the quality of life he deserves.

“Although this treatment is, quite literally, a lifeline, it unfortunately does not prevent the loss of sight, which is an inevitable part of CLN2 disease.

“Elliott will go blind, maybe in just a few years. We will not accept that, on top of everything he has to go through. We have huge hopes in the ongoing global trials that are pioneering the use of Brineura in the eyes to prevent degeneration of children’s eyesight.

Elliott Jackson with his parents and sister
Elliott Jackson with his parents and sister

Brineura in the eyes has been approved for compassionate use in Great Ormond Street.

The Jackson’s and other families in the Batten Disease community will provide much of the money needed for the Batten Disease Family Association (BDFA) which will then fund the hospital to carry out the treatment. It is also understood that the Great Ormond Street Hospital will also be able to cover some of the costs involved.

Surplus funds will be used by BDFA for the benefit of children and their families affected by Batten disease.

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The BDFA specifies that: “For the moment, the costs of this trial are provisional and are not yet known with precision. The duration of the trial will be 18 months. There will be hospital costs associated with the intravitreal injections which will include operating room time and general anesthesia costs as well as program coordination.

“Our target is now £ 200,000 and we are on track to get there.”

There are two ways to donate; tune in to Rob’s 24 hour live game stream by by clicking here, or to donate to the JustGiving page please Click here

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