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Family living with Haw River Syndrome needs help

September 29, 2018

BURLINGTON, N.C. (AP) — Michael Worth loved roller skating when he was little.

So for his 13th birthday last month, his mother, Kelly Davis, had a private party at a skating rink so he could watch his sisters and friends go around.

“He loves skating,” she said. “Everything that’s going to make him move, he wants it.”

Worth can’t skate anymore. He has a rare genetic condition called Haw River Syndrome. It is a neurological condition seen in several generations of one local family which damages nerve tissue leading to the loss of control of body movements, seizures, dementia and is eventually fatal. It’s similar or related to other diseases like dentatorubral-pallidoluysian atrophy, or DRPLA, which is mostly seen in Japan, or Huntington’s disease. There is no cure, so Worth’s doctors treat his symptoms.

He must be a pretty good patient because he is a really good-natured kid.

“He’s a happy kid — he’ll be happy for no reason sometimes,” Davis said. “When he cries, you know something is wrong.”

Even a wonky monitor that started beeping when his oxygen content reached 100, which is good, rather than when it dropped dangerously into the low 70s became something to laugh about when his mother leaned over his bedside and said “beep” close to his ear each time, making him smile until the beep itself became funny to him.

“I’ve got to make the best life for him that I can,” she said. “As long as he’s happy and smiling, I’m smiling.”

The disease mostly strikes between the ages of 15 and 30, according to the Journal of the American Medical Associaton. Worth’s doctor told Davis he hadn’t seen anyone’s symptoms start as early as Worth’s.

“Michael started having seizures when he was 5,” Davis said.

It always happened to him at school, never at home. After a while they realized it happened when he went outside into the sunlight.

“That’s how we figured out he was sensitive to the light,” she said.

It took three years to get the genetic testing and diagnosis at UNC Hospitals, Davis said. It came from his father’s side and wasn’t something anyone warned Davis about.

“I didn’t know about the disease in the family,” Davis said. “His dad didn’t know too much about it. They didn’t talk about it.”

Worth has an aunt now in a health care facility with the disease at a more advanced stage. Davis said it’s too hard to visit.

“That’s what Michael’s going to be like, and I don’t want to see it,” Davis said.

For a while things were mostly fine. Worth was an active kid, getting into everything, playing outside, riding his bike, eating junk food. As the disease progressed, his movements became limited and he used a walker and sometimes a wheelchair. Davis has a picture of him in his sunglasses and walker getting a certificate for completing fifth grade at Andrews Elementary School.

The seizures were still bad, though, sometimes lasting a half hour. Last September he had an allergic reaction to an anti-seizure medication and got very sick. He stopped talking, walking and eating. He slept all day and night. Davis told his doctors to take him off the medication. He started waking up, but ended up on a feeding tube and still couldn’t talk.

“Before that he would have a seizure and pop right back up,” Davis said.

When he started talking again, Davis said she was so happy, she didn’t even mind that he was cussing sometimes, at least at first.

“I was so happy he said something, it didn’t matter,” she said.

Worth can speak a little now, saying his sisters’ names — there are four of them — or “yeah” or “Friday,” a movie he likes a lot, but can’t use sentences much. Still he gets his point across, Davis said, slouching down when he wants to lie down or kicking his pillow when he wants to get out of his chair

He also ended up in the hospital eight times since January. All of this put Davis’ career as a certified nurse’s assistant on pause.

“I went from having it all together and taking care of my kids to nothing,” Davis said.

Davis doesn’t have too many people to care for Worth when she’s not around. There are a couple of nurses Worth likes and she trusts. Family members help out, but few feel confident about what to do if he has a seizure, so taking care of her son has become her full-time job.

There is not much money. She gets some help to care for Worth, Medicaid for most of his medical bills, but not all, and disability, which leaves about $100 or so after rent for a family of six.

Different churches, Hospice and others help — a lot, but there is always more need. Davis has lost her car, and the expenses just pile up.

“I don’t know all the people that help me, but I’m thankful for the people that help me.”

Patty Brothers, a board member with the Alamance Regional Medical Center’s Charitable Foundation, set up a GoFundMe page to help Davis pull together some living expenses for a few months at gofundme.com/fundmichael18.

“It’s been a long road — I wish he would get stable enough that I could go back to work,” Davis said. “If we can keep him out of the hospital, that’s the main thing.”

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Information from: Times-News, http://www.thetimesnews.com

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