Saturday 14 May 2011

SIDS: Virginia: Infant botulism a cause?

 May 6, 2011 : Prue Salasky
Five-month-old Mya Williams looks alert and curious as she sits on her mother's knee for a medical checkup. She gurgles uncertainly, uncertain whether to laugh or cry, as pediatric neurologist Ralph Northam taps her knees. "She doesn't have her reflexes back yet," he says, "They'll come back in time."

"She's getting stronger every day," responds her mother Lavista Williams, without undue concern. Just weeks earlier, Williams refused to take a physician's word when he assured her that her daughter was fine.

Williams' insistence on seeking appropriate care swiftly most likely saved her daughter's life.

Later that same day, Mya was diagnosed with infant botulism, a rarely occurring reaction to a toxin that attacks the nerves and muscles and develops rapidly over 24 hours. "She was weak as a kitten," says Northam, who treats about one case every two years. He speculates that some cases of Sudden Infant Death Syndrome in her age range may well be caused by infant botulism. They're still not sure how Mya contracted it. Most often, in infants, it's from ingesting honey, but Mya has only had formula and juice. The most likely culprit is from dust caused by construction in the Williams' neighborhood. Eventually, a stool culture should reveal the cause.

It was on a Thursday night that Williams noticed her daughter's cry sounded different. By the next morning, even though Mya took her formula fine, her cry was "really, really weak. Her arms were floppy, her head fell back. She couldn't roll on her side." Alarmed at her daughter's condition, Williams, who lives in Hampton and works in Newport News, called an ambulance to take her to a local emergency room. The doctor there ordered X-rays and blood work which revealed nothing. "She's OK, take her home," he advised. There was no pediatrician on staff in the emergency room.

Williams ignored the directive to go home, called ahead to the children's hospital and made the 40-minute drive to its emergency room. "Everything was immediately fast-paced," she says. "They took her vitals and ran three tests on her. By then her legs, which had been strong, stopped moving. Her cry was a whisper and her breathing was raspy." When the two doctors who treated her saw how weak she was, they called in a pediatric neurologist. Of the five on staff, one is always on call.

Northam responded and ran a nerve conductivity test and a spinal tap, both of which came back fine. At that point he narrowed the diagnosis to one of two things: infant botulism or Guillain-Barre syndrome (pronounced geeyon-baray). The latter, a peripheral neuropathy that occurs after an infection, usually affects children age 2 or older, but it has symptoms almost identical to botulism.

The final tip-off for the neurologist was that in addition to her saliva pooling from her difficulty swallowing, Mya had been constipated for a couple of days. "Her muscles were not getting the message from her nerves," he says. He admitted her to the intensive care unit where she stayed for 17 days, 12 of those on a ventilator.

Northam, who is also a Virginia state senator, waves a picture of Mya on the ventilator taken by her grandmother. "I'm taking this to show to the home-canning folks who come to Richmond who don't want to follow our laws," he says. "I'm going to talk to them about botulism, how it's serious and costly."

Then he turns back to Mya to check her eyes. "They're a little sleepy," Northam says. "It should take her about three or four more weeks to get back to her baseline."

Williams nods acceptingly. "They were really honest from the beginning," she says. "They told me it would be between two and six weeks before she made a turnaround."

Mya's recovery time was speeded up significantly — from several months to a few weeks — by the administration of a single dose of botulism immune globulin, better known as "BabyBIG," an antidote that had to be ordered from California. Administered by IV in a couple of hours, the single dose cost $45,300. "You may call her Mya, but I call her Mercedes," says Northam joking.

The cost actually evens out, he says, if you consider the cost of being in intensive care for three to four months, which would be the alternative. Slowly, her body started coming around, first the head and neck, then arms and legs and therapists started muscle work with her while she was still on the ventilator. Mya will continue physical therapy twice a week until she regains full strength. She isn't expected to have any lasting effects from her sudden, dramatic illness.

"It was just frightening to see her in that paralyzed state so quickly," says her grandmother Brenda Henderson. "It's so nice to hear her cry now. In ICU, you could see the tears but nothing was coming out."
It was the sound of Mya's cry that initially convinced Williams to keep pursuing medical help for her daughter. "It just wasn't a normal baby cry," she says.

What is infant botulism?Clostridium botulinum is found in dirt and dust; honey is a known source of the bacteria spores that cause botulism. It typically affects infants aged 3 weeks to 6 months, and up to 1 year. Once an infant ingests the spores, the bacteria multiply and produce a toxin that interferes with the interaction between muscles and nerves. Children younger than 1 year old should never eat honey or products made with honey. Symptoms occur from three to 30 days after ingestion.
Symptoms: Weak cry, poor feeding, muscle weakness, breathing problems, constipation.

In adults: Wound botulism occurs when bacteria infect a wound; food-borne botulism from improperly canned foods.

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