Isaac Fighting Batten Disease - The Big Build

This is my beautiful nephew Isaac who is 9 years old. At the age of 5, after several years of tests, Isaac was diagnosed with Batten Disease, a very rare neurodegenerative disease, which is terminal. Since this diagnosis Isaac has lost the ability to walk, talk and has lost his sight. He suffers from seizures and has been fitted with a mic-key button as he struggles to take on proper nutrition and hydration. Another cruel symptom of this disease is Dystonia. These painful muscle spasms affect Isaac every day and his doctors are currently trialing different medications to try to give him some relief. This is all on top of his fortnightly visits to hospital for his enzyme replacement therapy given via a catheter in the brain. This treatment isn’t a cure but rather slows the progression of the disease down and allows Isaac to be with us for longer. Life expectancy for children with Batten Disease without treatment is around 12 years old.

Adaptations were made to Isaac’s home 2.5 years ago and at the time the council decided that the living room would eventually become Isaac’s bedroom. This means that the family will loose their living space and have no physio space as a small sofa will have to be placed in that area. Anything bigger will mean Isaac won’t be able to join them at the dining table as his chair won’t fit. In reality he won’t be able to access much of his home aside from his bedroom. Not only this but when Isaac is showered he will have to be wheeled through the house to what would be his bedroom before then getting dressed. We all feel that this isn’t right. Isaac may not be aware but any teenage boy or girl would want their privacy and dignity and Isaac deserves the same care and respect.

Isaac is now 33kg! Which is amazing but that does mean that it won’t be long before my sister and brother in law are unable to carry him upstairs to his current bedroom which means that Isaac’s bedroom needing to be downstairs is fast approaching.

Rather than the family, including Isaac’s 12 year old Sister Eva, loosing their living space, we want to convert the garage into Isaac’s bedroom and extend it by 6ft. This means that he will have the space in his room for his wheelchair so that he can be moved from there into his bed. The current wet room will be connected to this new bedroom, meaning that he will get complete privacy, something that all children are entitled to. This also ensures that the current downstairs areas can stay as they are and the whole family can continue to spend time together. They have already had so many changes, challenges and upheaval, that this one thing could make such a massive difference to them and Isaac’s quality of life.

Aimee and Adrian have connected with a lovely builder called Steve, he has many years experience, is a caring family man & a foster carer too. Him and his team at Garrett Builders would be able to give Isaac the space that he needs but to be able to do that we need to find around £20,000. Unfortunately, they just don’t have access to this kind of money.

We understand that times are tough for everyone right now so we appreciate any and all donations. If you are unable to donate, even just sharing this fundraiser helps so much. Since his diagnosis our whole family has been determined to raise awareness of this rare but cruel disease in the hopes that children can be diagnosed earlier, started on treatment and with us for longer.

Thank you for reading and being apart of Isaac’s journey.

Liane - Isaac’s Auntie x