Stone family in TV spotlight for Text Santa

A family from Stone are in the TV spotlight to help this year’s ITV Text Santa appeal to raise money for the Donna Louise Children’s Hospice.

The Trentham hospice, which looks after children and their families from Staffordshire and Cheshire, is set to benefit from some of the funds raised through the appeal thanks to its partnership with Together for Short Lives, one of this year’s six national Text Santa charities.

Ian and Amanda Harrison, who live on the Aston Lodge estate, have been accessing the hospice’s care and support since their six-year-old son James was diagnosed with the extremely rare and life-limiting Infantile Batten Disease in 2010. In fact, James is one of only three children in the whole of the UK with the condition.

Their story – and the importance of the Donna Louise Children’s Hospice to their family – will be shown on ITV’s This Morning on Tuesday 9th December. A film crew filmed the family both at the hospice and at their home.

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Amanda said: “We really would be in a mess without the help of The Donna Louise. During our stay I can be a mother to all three of my children (James and five-year-old Ruby and Isabelle, three) and not a nurse. I have the time and the energy to play with them. I leave the hospice a new woman. This respite is key to staying upbeat and positive. We’re not sure what the future holds for James but we do know it would be a lot harder without the care and support of The Donna Louise Children’s Hospice.”

I spoke to Amanda today, but nothing I could write would come close to what Amanda wrote herself for the hospice below about James’s condition, how her family has coped and the vital role played by the Donna Louise Children’s Hospice… 

James was born perfectly healthy in March 2008 and met all of his important milestones. By 18 months he was walking (as long as he held our finger) and he could say the odd word – dadda, ozza (Oscar the cat). He was into everything – taking the coals out of the fire, pinching my lunch, pulling the cat’s tail and helping us decorate by putting his full arm into the paint tin! It was all done with the cutest smile! I was pregnant with my second child and life was good.

In September 2009 the seizures started. Not full blown how you would imagine, but short, quick jerks that floored him. He had 40 in a few hours and he was admitted to hospital. The tests and the medication started …

My daughter Ruby was born two months later in November 2009. By this time it was clear that something was seriously wrong with James. He had stopped crawling, walking, standing and was struggling to sit. He was a different boy – like something had been switched off.

During Christmas of that year James’s seizures changed so I contacted the hospital and we were given a glimmer of hope – that it could just be epilepsy that needs controlling. We felt so relieved and hopeful.

We were then called into the hospital on the 9th of January 2010 to meet with James’s consultant. We thought it was to discuss the new type of seizures. It was a Saturday and we were to go to Outpatients. I hated going to Outpatients as it was so very, very busy. However, when we arrived it was empty… two nurses were waiting for us… alarm bells started to ring.

When we were called in to see the consultant we were told that he had a diagnosis… James had Infantile Batten Disease. No treatment. No cure. James would not live beyond five years old. He was 22 months at the time.

I don’t have the words to describe how hearing that felt. They just don’t exist. Devastated, heartbroken, shocked, crushed just don’t come close. Infantile Batten Disease is a neurological degenerative disease. James is basically missing one of the enzymes which clears the rubbish from his brain cells. Therefore, the cells are dying as they fill up with this rubbish.

The sheet we were handed said that “over time affected children suffer mental impairment, worsening seizures and progressive loss of sight and motor skills. Eventually, children with Batten Disease become blind, bedridden, and demented. It is always fatal”.

It was hard to take this all in. Through our crying we heard the Consultant asking for Ruby’s date of birth. He then told us that the condition was genetic. My husband and I are both carriers, therefore Ruby had a 25% chance of also being affected – she was seven weeks old.

We had her tested as soon as we could. It took a week for the results to come back. Thankfully she is unaffected – the relief is also indescribable.

We then had two choices – to let Batten Disease ruin our family or dust ourselves down and live. It was not a path we ever expected to go down but we owed it to James and Ruby to give them both the best possible life we could, however long or short that maybe for James. We have a saying: ‘Don’t count the days, make the days count’ and that’s just what we decided to do.

Bravely or stupidly we decided to have another baby. This baby also had a 25% chance of being affected. So we rolled the dice again. Fortunately,testing is available during early pregnancy and we were told our baby was unaffected and Isabel was born in September 2011. So family life took a life of its own.

The Donna Louise plays a vital role for our family. It’s a place I can go for a complete rest as James is so well cared for. We go to stay as a family.

The girls love it. There I meet other families and we become ‘normal’ less isolated. I can chat to other mums about feeding pumps, suction machines, seizures and it’s our norm. To mothers without Special Needs Children there is a look of horror on their faces!

We would be in a mess without the help of The Donna Louise. During our stay I can be a mother to all three of my children and not a nurse. I have the time and energy to play with them. I leave the hospice a new woman. This respite is key to staying upbeat and positive.

James is now 6 and he likes to prove Doctors wrong. Yes he is poorly – he’s severely visually impaired, immobile, tube fed as he’s unable to swallow, he can’t speak but he loves life. He goes to school where he has friends. He loves hydrotherapy and the sensory room. He smiles to show us he’s happy. He has started opening his eyes wide to communicate with us and if we’re really lucky we get a laugh or giggle.

He loves cuddles and when you’re rough with him, wiggling his legs or arms. He loves his sisters and they are so good with him.  They are so caring and thoughtful. And yes it’s hard, it’s 24/7 – it’s exhausting. It’s the hardest job I’ve ever done and I was Deputy Head of an inner city school before but I love every minute I have with James.

We are not sure what the future holds for James but we do know it would be a lot harder without the care and support of The Donna Louise Children’s Hospice.

This year’s Text Santa beneficiary charities are Together for Short Lives, Teenage Cancer Trust, Guide Dogs, WellChild, Marie Curie Cancer Care and The Alzheimer’s Society. The Text Santa live appeal show is on Friday 19th December on ITV1, presented by Phillip Schofield, Christine Bleakley, Alesha Dixon, Paddy McGuinness and Ant & Dec. Click HERE to donate now.