Oscar Jealous died aged just 10 from the little-known Batten disease. But what is the disease and what are its symptoms?
Oscar’s family described it as “the most horrible disease known”. S’exprimant plus tôt ce mois-ci quelques jours avant sa mort, ses parents Alex et Dani, ont déclaré que la maladie de Batten avait été “si cruelle” envers leur famille.
Although it took the life of Oscar, of Great Barr, it’s an extremely rare condition that luckily doesn’t affect many people. But for those unlucky enough to develop it, the prognosis is grim.
Read more: Pride of Birmingham ‘Superhero’ winner Oscar Jealous tragedy dies aged 10
Oscar was diagnosed with the disease during the first lockdown in March 2020. His family announced the tragic news this weekend (June 19) that he had died last Thursday June 16.
What is Batten disease?
It is a neurodegenerative disease that is often described as a childhood form of dementia. Elle est causée par de faibles niveaux d’une enzyme appelée «tripeptidyl peptidase 1», ce qui signifie que des substances grasses s’accumulent dans le cerveau, le système nerveux et les yeux.
Batten disease usually begins in childhood and is extremely rare. There are only around 25-40 children living with the condition in England. Children with the disease often appear healthy and are developing normally before they begin to show symptoms.
What are its symptoms?
Common symptoms include loss of powers to speak, see, and swallow. Epilepsy and memory loss are also observed.
Other early flags of latte disease include autism and ADHD, but not all children get it. As the disease progresses, there are also learning difficulties, clumsiness and involuntary movements.
Is there a treatment or cure?
Il n’y a actuellement pas de traitement ou de traitements à l’étendue à la vie pour la maladie de Batten, uniquement un soulagement des symptômes et des soins de soutien. Any children who develop it will have a very short life, with most only living between the ages of eight and 14.
Some who develop it later in childhood will live into early adulthood, and some may even survive into their twenties or thirties. For adults diagnosed, the outlook is better, with milder symptoms, and it may not affect life expectancy.
La mère d’Oscar Jealous, Dani, a déclaré que le moment où la famille a appris à l’hôpital pour enfants de Great Ormond Street que leur fils avait la maladie était le pire jour de leur vie. She said: “It’s a neurological disorder that affects the brain – childhood dementia is what they told us.
“That day, knowing these things were going to happen, our world ended. Or it felt like our world had ended. It was overwhelming.
“At the end, I asked them the question: ‘Is he going to die from this?’ and they said “yes”.
“Then the next question was, ‘How long? Bearing in mind that Oscar was eight at the time. They said he could make it to his teens. “
Is it genetic?
Unfortunately yes. Any family whose child is diagnosed will also need to have their other children tested. There is a one in four chance that siblings will get it.
S’exprimant le 2 juin avant la Journée internationale de sensibilisation à la maladie de Batten, Alex Jealous a posté sur Instagram : “La maladie de Batten est véritablement la maladie la plus horrible connue !! Nous aimions passer des journées en famille au parc, playing games, playing football and eating a lot of ice cream. Battens was very cruel to the idea of Oscar taking all of his skills away in just two short years of being diagnosed.”
More details on Battens disease can be found here.