A young Scots girl suffering from a ‘ Alzheimer's style’ illness has been left non-verbal as her harrowing condition continues to progress.
Sophia Scott, 11, was diagnosed with Sanfilippo Syndrome in 2015 after parents Darren, 43 and Amanda, 45, noticed she was having developmental issues.
The rare genetic condition, also known as Mucopolysaccharidosis type III (MPS III), causes fatal brain damage and is a type of childhood dementia.
Sadly the average life expectancy for children suffering from the disease is between 10 to 20-years-old, but most don't survive their teenage years.
Sophia's dad Darren, from Glasgow, has opened up about the heartbreaking reality of the condition and said watching his daughter suffer is 'shattering'.
Darren said his daughter's diagnosis came "like a bolt of lightening" as Sophia, who was previously fit and healthy, began to have difficulties with her speech and reading.
A referral to speech therapy from the youngster's nursery, eventually led to the tests which diagnosed her terminal illness.
Speaking to the Daily Record, Darren said: "This has destroyed all of us. It turned our life upside down.
"Not a day, not a minute, goes by that it doesn't flash through your head the sadness of it.
"We want to give Sophia the best life possible, even if that is just things like taking Sophia on once in a lifetime trips.
"But it is hard trying to squeeze as much as possible into such a short time. It is obviously a very difficult and upsetting life."
Darren and Sophia's mum Amanda have some very difficult medial decisions to make for their daughter's future and have to choose whether or not she will undergo potentially life-threatening surgery.
As her condition has progressed, Sophia's spine has curved which is now impacting her internal organs.
Surgeons would like to do a major operation to fuse metal rods into her back, but the procedure does not come without its risks.
The youngster previously had spinal surgery but the emotional impact of the decision was extremely difficult for the family.
Darren added: "It is a horrendous experience to have to live with and be a part of, especially with some of the decisions we have had to make recently.
"When we went through the surgery with her a few years ago, it took everything out of us.
"These are decisions you don't want to be thinking about for your child - never mind your child with a lower life expectancy than other children.
Sophia is also now struggling to keep weight on and will most likely need another operation to insert a feeding tube.
Despite the heartbreaking reality of the family's situation, Darren and Amanda focus on short term goals so they can give Sophia a happy and positive life.
Darren said: "Watching Sophie become non-verbal and watching the condition take away all the things she has learnt is hard.
"But she is an absolute bundle of energy and joy. She is very bubbly and has crazy blonde hair with big blue eyes and an infectious smile.
"The two things she loves the most are adventures and people.
"We won't give up on her. There is no cure yet, but there is research going on. Awareness is the only hope we have."
For the past three years Darren and Amanda have launched a birthday fundraiser for Sophia with some cash going towards making memories and the rest being donated to medical research and charity.
This year money will go towards 'fighting the incurable and progressive illness' as a donation will also be made to Sophia's fantastic school Kelburn Park Primary.
Donations to the fundraiser can be made by clicking here.
What is Sanfilippo Syndrome?
Sanfilippo Syndrome is a rare genetic metabolism disorder which leads to serious problems in the brain and nervous system.
The condition often goes undetected until early childhood as most don't have symptoms until they are between two and six-years-old.
Early symptoms of the condition include recurring ear or sinus infections, speech and development delays, persistent nasal congestion, sleep disturbance, hearing loss and diareah.
Appearance can also be affected with some children having coarse facial features, thick eyebrows and a large head.
Behavioural issues are also linked to the condition with some children being hyperactive, impulsive and difficult to cooperate with. Symptoms are similar to that of people with autism.
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