Angelina's Story1 October 2020
“Just two years ago, Angelina’s schoolwork showed full pages of neat writing, underlining, answering of questions and problem-solving. Now Angelina is lucky to write a few words or read simple sentences.”
In September 2018, 14-year-old Angelina, was found unconscious in a corridor at her school. Her stepfather was at her side as she woke up. While he didn’t know at the time she was waking from a seizure, he noticed an unusual expression on her face. “It almost looks like she’s smiling when she comes out of a seizure,” explains her mum, Niki.
Niki noticed Angelina seemed tired and was occasionally dropping things and falling over. On a night around two weeks after her seizure at school, Niki watched with horror as Angelina tried to set the table for dinner.
“She was smashing the glasses. All of them. Just dropping them. I called our medical centre and they sent an ambulance. They were concerned it was a stroke.”
It was another week until Angelina had her first witnessed seizure. She was mid-conversation with a terrified Niki who managed to catch Angelina in her arms.
“I thought she was dying,” she remembers.
Angelina was initially diagnosed with a form of epilepsy, however, over the next nine months her seizures worsened. Angelina also appeared to be suffering cognitive decline. “She came home very upset one day because her friends had lost patience with her during a game of UNO. She wasn’t following the cards’ directions and she wasn’t aware when it was her turn.”
After three months of testing, worried doctors gave Angelina and her family devastating news. Angelina was diagnosed with childhood dementia, caused by a rare genetic disorder called Lafora disease. Symptoms include seizures, muscle spasms, difficulty walking, behavioural changes, confusion and cognitive decline. Within just a few years from the onset of symptoms, children typically find it hard to complete daily activities. Most only live for around 10 years from those first symptoms
“It was a complete shock. Angelina was like most other teenagers. She had no signs or symptoms or any abnormalities. She was extremely social, self-motivated, goal-driven, academic and had big dreams for her future. She was in school musicals and attending acting classes and wanted to become a makeup artist and business owner.”
Sadly, Angelina’s condition declined in the year following her diagnosis. At times Angelina started to find it difficult to speak, swallow or walk unassisted. In May 2020 doctors inserted a gastrostomy tube so that she could be fed and given medications safely.
By June 2020, Angelina’s behaviour started to change. This is a common result of progressive dementia. For Angelina, this made her irritable and impacted her mental health. She started to refuse to eat, co-operate with self-care, get out of bed, or take her medications. Angelina’s short and long-term memory and cognitive abilities significantly declined too.
“Just two years ago, Angelina’s schoolwork showed full pages of neat writing, underlining, answering of questions and problem-solving. Now Angelina is lucky to write a few words or read simple sentences. She’s very childlike. She needs words to be broken down and said slowly so that she can understand. She has difficulty initiating things and basic, daily-life decisions, such as what shirt to wear, are a struggle for her.”
Angelina’s body will eventually become tolerant to the medications that give her some respite from seizures and behavioural challenges. She is at high risk of Sudden Unexpected Death in Epilepsy (SUDEP).
“Every day is extremely challenging. The whole family is on high alert all day, every day. We must monitor her constantly,” says Niki.
Niki wants to see more awareness of childhood dementia, more screening for it at birth and more research.
“How common is dementia in old people? It’s upsetting to see a parent like that. To see that happening for a child who has lost all their future dreams is even more devastating," says Niki.
There is currently no cure available for Angelina or the estimated 700,000 children and young people worldwide who suffer from childhood dementia.
In 2021, Niki recorded Just Like a Butterfly, a song dedicated to Angelina. 'This song is about how she bounces back from her seizures and what I wish for her,' says Niki. You can download this beautiful song on Apple Music, iTunes, Spotify, Amazon Music and Youtube.
Please don’t leave childhood dementia to slowly kill more children.
Image 1: Angelina at her Year 6 Formal in 2015. Angelina showed no symptoms of childhood dementia until she was 14. Image 2: Angelina singing on her 13th Birthday in 2017. Angelina loved acting and taking part in school musicals. Today, she is unable to attend school and is suffering significant cognitive decline. Image 3: Angelina in August 2020 at a palliative care hospital for children. Angelina’s seizures can be triggered by light and too much activity, making rest essential. Image 4: Angelina in 2020.