A couple have described the agony of learning that their three children were diagnosed with a rare terminal disease.
Last summer, Noreen and Lester Jessop’s 10-year-old son Daron and four-year-old twin daughters Angela and Aubrey were diagnosed with pantothenate kinase-associated neurodegeneration (PKAN), a neurological condition that causes involuntary muscle spasms and trouble walking.
The crushing news was made even more painful when they were told their children might not live past 10 or 11 years old.
However, Noreen and Lester, of Saint George, Utah, are hopeful that an upcoming clinical drug trial can give their children a second chance at life.
‘Being told that your children won’t live to be 10 years old or having [doctors] tell us to enjoy them while they were alive is the most devastating news a parent can have,’ Noreen told Daily Mail Online.
Daron, left, and his twin sisters Angela and Aubrey, right, were diagnosed with pantothenate kinase-associated neurodegeneration (PKAN), a rare neurological disorder in June 2017
Noreen, left, and Lester, right, said they knew something was wrong when their children were not meeting major developmental milestones, including being able to walk or talk
The family’s ordeal began shortly after Daron was born in 2008.
Infants usually learn to crawl between the ages of six and 10 months, but Daron didn’t start crawling until he was 13 months old.
However, Noreen didn’t know the severity of Daron’s condition. She only considered having him see a physical therapist to help give him an ‘extra boost.’
But his symptoms became more pronounced as he got older.
Noreen was alarmed when Daron was still unable to walk at two years old, especially since most babies are able to move around on their two legs by the time they are 14 and 15 months old.
Being told that you’re children won’t make it to 10 years old… is the most devastating news a parent can have
She and Lester, founders of the construction company Prime Excavating, took Daron to get an MRI scan, which revealed he had iron deposits in his brain.
However, doctors didn’t tell them what this meant for Daron’s future.
‘The doctors said that his brain scans definitely weren’t normal and suggested that the iron may be the reason he’s not able to walk,’ she said. ‘But they never really gave me an answer.’
Noreen would continue to have Daron undergo a series of MRI scans and genetic testing for the next eight years to figure out what was ailing her son.
She desperately tried to detox her son’s brain naturally using herbal tea and a bunch of ‘homeopathic stuff,’ but gave up after nothing seemed to work.
By the time he was four years old, Daron was still crawling around on the floor until his knees bled.
Noreen also noticed that Daron was starting to regress little by little each day – he was losing his balance more often and wasn’t able to catch himself when he fell anymore.
Right after Daron turned five years old in 2013, Noreen gave birth to twin girls Angela and Aubrey.
She and Lester were happy with the two new additions to their family, but it was difficult raising their newborns while Daron’s condition continued to worsen.
It wasn’t uncommon to see him with bruises on his knees or a bandage on his chin where it had split open from him falling.
When the twins turned two years old, Noreen noticed they showed similar patterns to their older brother and were missing major milestones.
Symptoms of PKAN include weakness, involuntary muscle contractions and stiffness in limbs
They had difficulty walking and communicating, but their symptoms weren’t as extreme as Daron’s.
When Daron turned eight years old, his muscles became weaker and he lost the ability to feed himself.
At one point he lost the ability to chew and swallow his food. He currently uses a feeding tube.
When Noreen took Daron and the twins to get MRIs, the scans revealed that they all had iron deposits on their brain. However, they still weren’t diagnosed.
‘It was hard going from doctor’s office to doctor’s office and never getting an answer,’ Noreen said. ‘That was the story of our lives for nine and a half years.’
However, she considered those brain scan images to be a piece of a puzzle. She was going to use that information to figure out what’s ailing her children.
Pantothenate kinase-associated neurodegeneration (PKAN) did come up in some of her research on iron accumulation in the brain, so she wasn’t completely shocked when a geneticist finally diagnosed Daron, Aubrey and Angela with the disorder in June 2017.
‘I was kind of expecting it,’ Noreen said. ‘But to have that confirmation was devastating.’
PKAN is a disease characterized by the deterioration of specific regions in the central nervous system caused by the accumulation of iron in the brain.
Symptoms of the disease, which affects approximately one to three million people worldwide, include speech problems, stiffness in limbs, poor balance, and involuntary muscle contractions.
Noreen and Lester were even more devastated when they found out there was no cure for the disease and that their children might not even live long enough to be teenagers.
‘It was a very tough time,’ she said. ‘Me and my husband would just sob and sob… we were a mess for months.’
Twin sisters Angela and Aubrey (pictured) had difficulty walking and communicating, but their symptoms weren’t as extreme as Daron’s
Upon hearing this devastating news, Noreen wanted to learn more about the disease that is killing her children and whether there was a cure.
She began researching PKAN and came across a group of doctors in Oregon who’ve been investigating this disorder for the past 25 years.
Noreen was overjoyed when she learned that these doctors may have developed a treatment that could possible stop the progression of the degenerative brain disease as it would give her children a second chance at living a long and healthy life.
‘It’s incredible that there’s a solution for this disease in the works,’ Noreen said. ‘It gives my children a better outlook on life, it gives them hope.’
The Oregon team announced in November that they were creating a PKAN drug to ensure sufficient amounts for a clinical trial.
However, this doesn’t come cheap. The Spoonbill Foundation, an organization created with the goal to discover and develop treatments for PKAN, is seeking $2 million to fund the clinical trial.
A GoFundMe page was set up to help raise funding for the trial. As of Tuesday afternoon, more than $18,000 have been raised since the page was created on February 14.