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Searching for a cure

5 min read
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Addison Johnson will forever remember her fifth wedding anniversary – but not for reasons she could ever have imagined.

It was the day that she and her husband, Ryan, learned that the couple’s 8-month-old triplet daughters had the same terminal illness as their 3-year-old son.

“I thought, ‘No way,'” Addison said during a recent telephone interview during a trip to the pharmacy. “It was very hard knowing they all have it. We are the only family in the entire world where all of their children are affected.”

The youngsters were born with axtaxia-telangiectasia, a rare, genetic disease that attacks early in childhood and progressively affects coordination. A-T predisposes children to fatal cancers and severely compromises their immune system. Children usually are unable to walk by the age of 10, and they rarely survive their 20s.

But the Johnsons are not letting the grim numbers or their children’s myriad health issues get them down.

“We kind of ignore it in a sense,” Addison said. “We don’t focus on the illness as much as we do on who they are.”

And the couple also are doing their part to promote – and fund – research in the search for a cure.

On Saturday, they will hold their fourth major fundraiser, Johnson Angels, at the New Stanton Volunteer Fire Hall. Proceeds will benefit the A-T Children’s Project, which is predominantly funded by the grassroots efforts of families and friends of children with A-T. On Monday, the family, along with the children’s grandparents, will travel to Boston, where Ayden, now 8, and his sisters, Alivia, Payton and Riley, now 5, will participate in a weeklong A-T study.

The family resides in Greensburg, but has strong ties to Washington County. Ryan is a 1997 graduate of McGuffey High School, and his family still lives in the area.

“It’s been devastating,” said Ryan’s sister, Tina Johnson-Hines of Washington. “More research needs to be done so they can treat more of the symptoms. A lot of the children with A-T suffer from immune deficiencies. It’s like ALS in children. The muscles are not getting the signal from the brain.”

The disease is caused by mutated copies of the A-T gene, of which both parents must be carriers. Addison was told the chances of both she and Ryan carrying the gene, then meeting and marrying was one in 2.5 million.

When Ayden was born, Addison said he was on track developmentally. However, that all started to change when Addison’s mother noticed that something was “a little off” when Ayden started to walk. He was 14 months old.

“He looked like a drunken sailor in every sense of the word. He was very wobbly,” said Addison, noting that since Ayden was the couple’s only child, they really didn’t suspect anything was wrong.

By the time Ayden was 18 months old, he wasn’t getting any better, so the Johnsons took him to see a neurologist. After extensive testing and consulting with various specialists, the Johnsons were told Ayden did not have A-T.

So, they bought a house and started to try for a second child.

In the meantime, Ayden’s test results were sent to Ronald Reagan UCLA Medical Center, and just a few months into her pregnancy with the triplets, Addison learned that Ayden was misdiagnosed and that, yes, he does have A-T.

“I fought with them. I said, ‘You told me he doesn’t have it,'” Addison said.

The Johnsons decided not to have the girls tested for A-T in utero, but when the triplets started to display symptoms similar to Ayden’s, the Johnsons knew they had to do something. They weren’t sure if the girls’ developmental issues were related to their premature birth – they were nine weeks early – or A-T.

“Ayden couldn’t tolerate tummy time, and I noticed that with Alivia,” Addison said. “In the back of my mind, I thought they might have it, too.”

The girls were 8 months old. Two weeks after they were tested, Addison’s worst fears were confirmed.

“They haven’t asked or said anything that they’re different, and we answer questions as we go,” Addison said. “Ayden was about 4 when he said, ‘What’s A-T, mom?’ I said, ‘It’s something that makes you extra special.'”

Ayden is a second-grader and is mainstreamed, and his school has been very accommodating. There is a place in his classroom where he can nap if he needs to, and extra snacks are always on hand. He recently earned his yellow stripe in Tae Kwon Do.

The girls did not sit up on their own until they were 1 year old, and Payton and Riley were 22 months old when they started to walk.

But Payton now uses a wheelchair because of her dual diagnosis of cerebral palsy and A-T, and on April 11, 2014, Alivia was diagnosed with stage 3 T-cell lymphoma. She has been in remission since December, but she uses a wheelchair as well. All of the girls attend preschool.

“Thank goodness Alivia is so young,” Addison said. “She lost all of her hair, and when I would be blow-drying her sister’s hair, Alivia would say, ‘Blow-dry my hair.’ Then she’d laugh and say, ‘I don’t have any hair.’

“We are so blessed,” Addison continued. “We have four of the most beautiful kids.”

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