In 2015 Ollie and Amelia Carroll from Poynton, Cheshire were diagnosed with the ultra rare life limiting neurodegenerative CNL2 Batten Disease.

CNL2 Batten Disease robs seemingly healthy children of their abilities one by one until ultimately taking their life.

Life expectancy is between six and twelve years.

Children with CNL2 Batten Disease are born healthy, symptoms of the disease do not appear until around the age of three when speech delay and seizures occur.

For almost two years Ollie and Amelia’s parents, Mike and Lucy fought for access to a treatment which was proving to slow down the progression of this disease.

Eventually Ollie and Amelia were granted access to this ground breaking treatment on the grounds of compassionate use at Great Ormond Street Hospital London, funded by the pharmaceutical company Biomarin.

The treatment which replaces the missing enzymes within the brain of a child with CNL2 Batten Disease consists of a four hour long brain infusion every two weeks.

Heartbreakingly during the fight for treatment Ollie now ten lost his ability to walk and talk. He is now fed via a feeding tube which goes directly into his stomach, devistatingly Ollie is now blind.

At the time of starting treatment Amelia became the youngest child in the UK and one of the youngest in the world to receive enzyme replace treatment for CNL2 Batten Disease.

At the age of three Amelia had brain surgery just months after her big brother Ollie and began her battle against battens.

As Amelia was able to access treatment at such a young age Amelia now seven is still able to walk, talk, attend a mainstream school. She can eat and play like any other child of her age.

To date Amelia is currently four years seizure free, because of this treatment Amelia’s future is now unknown.

After a lengthy battle by Ollie and Amelia’s parents and others including professionals at Great Ormond Street Hospital in London and The Batten Disease Family Association which resulted in Mike and Lucy personally taking legal action against The National Institute of Health and Care Excellence. The treatment for CNL2 Batten Disease became available in September 2019 on the NHS for all children with CNL2 Batten Disease in England under a five year managed Access Agreement.

Sadly although this treatment is slowing down the progression of the disease it does not halt the regression of the loss of sight, due to the drug not being able to cross the blood brain barrier.

Meaning that even with enzyme replacement into the brain children with CNL2 Batten Disease will still go blind.

Our aim now is to make everyday count, provide Ollie and Amelia everything they need to make their lives more comfortable and a little easier, to create memories with them and their siblings and to raise the much needed awareness of this ultra rare disease in the hope of preventing other families facing the same fight that we have had.

We thank everyone who supports us during this journey. Our hearts are broken but whilst our children continue to fight we will be right beside them fighting with them.