It is so easy to take our time on this planet for granted. We get so caught up in our day-to-day lives that we seem to think there’s always tomorrow.
Sadly, for Queensland siblings, Lachlan and Hayley Webb, who suffer from an extremely rare and incurable genetic brain disorder called Fatal Familial Insomnia (FFI), their time together may be cut short sooner than they had hoped.
Lachlan Webb, who has a one-year-old son, is currently in a race against time as he battles ‘triggered’ FFI and prays he will be able to celebrate Christmas with his family.
FFI is a genetic condition passed down from generation to generation. It is as terrible as it sounds with victims being unable to get proper sleep.
Once the disease triggers, the body starts mass-producing mutated proteins which attack the sleep cortex of the brain, making REM sleep a physical impossibility. Without proper sleep the body can’t regenerate resulting in insomnia, loss of motor skills, memory loss, dementia and ultimately – death,” Hayley explains on a GoFundMe account.
The tight-knit siblings were diagnosed with the brain disorder in 2015. And although it is unknown what, how, or when it could be activated, Hayley told 7news.com.au, that they had put the diagnosis in the back of their minds until this year when Lachlan began to display symptoms.
“This year hit us like a big punch in the face and we realizsd that time isn’t guaranteed. And that is frankly f****** terrifying. That’s really scary,” Hayley said.
“While we’ve always known there was a risk of the disease ‘triggering’ at some point, never in a million nightmares did we think it would strike so early.”
A family curse
The Webb family has been battling FFI for generations. In the 1970s, one of their uncles passed away in his early 20s from unknown causes.
As Hayley, a former 9 News reporter told 7news.com.au, “At the time, my family had no idea what was happening to him.”
However, years later, in 1994, when their maternal grandmother passed away at age 69 in similar circumstances the family discovered they carried a gene causing FFI.
Then, six months after being diagnosed as ‘FFI-triggered,’ the Webb siblings lost their mum, Narelle, to the unforgiving disease in 2012. She was 61 years old. The family also lost an aunty aged 42 and another uncle at 59 years of age.
Both Hayley and Lachlan were diagnosed with the incurable condition but were not displaying symptoms.
Lachlan’s heartbreaking diagnosis
In April of this year, the worst-case scenario occurred. Lachlan was diagnosed as ‘triggered’ for FFI the day after his son, Morrison’s, first birthday.
Before the diagnosis, Lachlan’s symptoms included “memory fog, dizziness, weight loss, and night wakings.”
He had just had a baby and was under a lot of stress at work. He had things going on in his life that could explain these symptoms away.”
But Lachlan said he suspected “something was wrong” in the months leading up to “FFI taking hold.”
Lachlan is only 36 years old. Since his diagnosis, he and his now-wife Claire, were married and Lachlan is doing as much as he can to soak up the days with his family.
He is now wheelchair-bound, experiencing loss of eyesight, physical co-ordination, confusion, memory loss and is losing his ability to speak.”
Despite all the challenges, Hayley says her brother “Has surprised us all with his resilience.”
“Lach has weeks, maybe months, at best.”
While the family, unfortunately, cannot be sure about time frames with this disease, Hayley writes on the GoFundMe page she’s created for her brother and his family that
Based on the disease’s rapid progression in our other family members; Lach has weeks, maybe months, at best.”
“There’s nothing we could do to stop it.”
With three and a half-year-old boy and a second one only a few weeks away, Hayley feels overwhelmed at the thought of losing her “darling brother and lifelong best friend” while also knowing FFI will one day come for her as well.
In a video diary, she says, “You know when you get fuzzy-brained when you’re really exhausted? I think, ‘Crap! Is that a symptom?’ Subconsciously, you can’t help but be scared.”
Thankfully, Lachlan and Hayley are not alone in this tragedy. Aside from their loving and supportive partners, they have their dad and stepmum, who also help take care of Lach as his disease progresses. Hayley has also set up a GoFundMe account to help cover the costs.
“While there’s no amount of money that will ever ease this nightmare, by helping to alleviate some of the financial pressures it will mean we can focus on what’s important right now, which is spending quality-time together as a family.”
Their page has reached $285,000 of their $300,000 goal, which will be used to provide Lachlan with “the best healthcare” and “support for his family financially during this period of loss of income.”
What is FFI?
According to the Cleveland Clinic, Fatal Familial Insomnia (FFI) affects approximately 50 to 70 families worldwide.
The National Organisation for Rare Disorders explains that this rare, incurable, unpreventable neurodegenerative genetic disease triggers the body to mutate proteins, also known as prions, which we all have as part of our normal biology.
As the mutated prions start mass-producing, they accumulate and attack the thalamus, otherwise known as the sleep cortex, killing brain cells and leading to a sponge-like pattern.
The disorder is caused by a single dominant gene mutation; therefore, only one parent must have it to be passed on to their children, which is how Lachlan and Hayley inherited it from their grandmother.
FFI doesn’t prevent the person from sleeping, but it does keep them from getting to the restorative stages of sleep.
In Lachlan’s case, Hayley says her brother “still goes to bed every night. He still falls asleep – albeit for short periods of time. The problem isn’t so much him falling asleep, as it is reaching the restorative stages of sleep.”
“The family curse stops here!”
Both Hayley and Lachlan have had their children via IVF in order to ensure the disease isn’t passed down to their children.
We knew there was no cure…no prevention…there was nothing we could do to stop it. So, we focused on the things we could do, that is raise awareness, raise money for research and do IVF, so we never pass it down to our children and the buck would stop with us,” Hayley said.
You can follow Lachlan’s brave battle and support the Webb family through GoFundMe.
