Crue Bucknell is a tremendous seven-year-old. He’s cherished by his friends at college, adored by his brothers and a giant fan of rainbows and enjoying tiggie. He’s additionally dwelling with an extremely uncommon illness that can trigger him to lose all the talents he has gained in his quick life.
Most kids with Crue’s uncommon illness don’t make it previous their teenagers.
Crue’s illness is known as Sanfilippo Syndrome Sort A. It’s similar to Alzheimer’s and dementia however in kids. It’s most likely some of the devastating ailments we’ve ever heard of which is why we needed to share Crue’s very courageous journey thus far.
Whereas Sanfilippo Syndrome Sort A could be very very uncommon, it does occur. Advocate in your youngster in case you really feel one thing isn’t proper and, if you’ll be able to, please present your assist by donating to Crue’s Combat for a Remedy.
Crue’s Story
Kayla Bucknell, Crue’s mum, shares updates on Crue and their quest for a Remedy for Crue on Instagram and likewise by way of Love What Issues.
Crue was born in 2017. The primary signal that one thing is likely to be amiss with their darling second son was his fixed ear infections. Crue by no means confirmed indicators of ache however he battled on and off with ear infections for the primary yr of his life and had tubes when he was 18 months outdated.
As a toddler, Crue didn’t appear too taken with books or strolling, which Kayla handed off as ‘second youngster syndrome’. Crue didn’t stroll till he was 16 months outdated (which isn’t thought of a delay) and he had speech delays however the household assumed Crue was doing issues in his personal time. When Crue started preschool he struggled with studying and developed a stutter.
Subsequent got here the unexplained diarrhea that didn’t accompany some other signs akin to fever or sickness. Medical doctors urged he was constipated and did a MiraLAX cleanout. This didn’t clear up the problems and on one other examination, docs found Crue’s liver appeared bigger than regular.
An ultrasound confirmed that Crue’s liver was bigger than regular, even bigger than the common grown grownup. Kayla knew one thing was mistaken. They received a referral to the pediatric gastroenterologist after which a genetic specialist who lastly had a solution for the Bucknell household.
On 13 October 2022, a day Kayla refers to as D-Day, she obtained the information that their son had Sanfilippo Syndrome Sort A or MPSIIIA.
We had been informed to not Google Sanfilippo till we met with the physician the following day, any mother or father would by no means take that recommendation. I felt bodily in poor health, with tears that didn’t cease for days. We had been damaged.
Sanfilippo NEVER confirmed up in any of my analysis or 1000’s of google searches. This was not on our radar. This was not one thing our youngster may have.”
What’s Sanfilippo Syndrome?
Sanfilippo Syndrome is a quickly, degenerative illness that assaults the mind and physique. It’s brought on by a single genetic defect which causes the physique to lack a crucial enzyme. Due to the dearth of enzymes, the physique is unable to interrupt down heparan sulphate, a pure mobile waste.
As a result of kids can not break this down, their brains develop into full of poisonous ranges that can result in disordered sleep, lack of speech, cognitive decline, cardiac points, seizures, lack of mobility, lack of ability to eat by mouth and finally, dying.
“Crue will lose all the talents he has gained in his quick life, endure seizures and motion problems, and finally die in his teenagers.
You see 5 years of your youngster’s life. You consider his first t-ball recreation, his first day of kindergarten, his first basket scored, his first solo on the Christmas live performance, his first date, and a future partner. The dream of them changing into mother and father at some point.
A household of three boys you think about them rising up collectively. Kade, Crue, and Kai are the very best of buddies. They race by way of the home, wrestling, and throwing any ball they will get their fingers on.
Not solely will we lose a toddler, however they can even lose their finest pal, their brother.”
Youngsters with MPS III often seem wholesome at start, however developmental delay is often evident by age two to 5. Psychological and motor improvement peaks by three to 6 years of age, after which an mental decline often happens.
Different signs that Kayla observed in Crue are:
- Persistent ear infections
- Speech delays
- Persistent diarrhea
- Stutter
- Studying delays
- Delayed strolling
- Enlarged liver
Since Crue’s analysis, the Bucknell household has continued to boost consciousness within the hopes of a treatment throughout Crue’s lifetime.
“We wait, we hope, we pray, and we struggle for somebody, someplace on the market that can care sufficient to assist fund medical trials and save these kids. If cash was no object, would we discover one thing to assist their high quality of life.”
Crue, now seven years outdated, is somewhat champion who dressed up as a brown bear for Halloween and had a Toy Story-themed birthday. His speech, cognitive abilities and mobility are, for probably the most half, robust however the likelihood of studying any new abilities is nearly inconceivable.
Nonetheless, Crue and his household rejoice him on daily basis.
“We rejoice his love for all times, his household, love for the outside and he can’t wait to go deer looking! We rejoice his love for books, UNO, asking all of the questions and ensuring he is aware of who the Packers are enjoying always.
His smiles are infectious, his politeness is real and if he trusts you he’ll love you ceaselessly.
Keep robust candy boy the world is best due to you.”
To know your youngster won’t make it previous their teenagers is completely heartwrenching. We ship our assist to the Bucknells and hope for a Remedy for Crue. You’ll be able to donate to Crue’s mission right here.
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