Starting his evening shift, Dr. James Lindsay heard about a mysterious case of an infant who wouldn’t breathe on his own. The baby had been diagnosed with a respiratory infection and put on a ventilator three days earlier. But as the infection cleared and doctors removed the breathing support, the baby wouldn’t breathe on his own. The baby was weak and lethargic, hardly moving at all.

Then one look at the baby’s face jogged Lindsay’s memory.

“His face was absolutely expressionless,” he said. “There was absolutely no tone in his face. You couldn’t say he was sad or anything.”

Lindsay recalled a trip to Hawaii 20 years earlier, when he toured a pediatric ICU at the Kapi’olani Children’s Hospital in Honolulu. His colleague pointed out a child with infant botulism that had the same tell-tale, expressionless look on his face.

That memory helped Lindsay diagnose and treat Josiah Ferguson, a 6-month old infant from Redmond, currently at Randall Children’s Hospital at Legacy Emanuel in Portland with a rare case of infant botulism. After a month in the intensive care unit, including three weeks on a ventilator, the child was released from the ICU Thursday. Doctors expect him to make a full recovery.

Not food poisoning

Infant botulism affects fewer than 100 babies in the U.S. each year. It develops somewhat differently than the botulism that affects older children or adults. In those cases, botulism spores germinate outside of the body, such as in improperly canned food, producing a toxin that is then consumed causing illness.

Infant botulism occurs when babies ingest the botulism spores, which germinate in their immature colons eventually producing the toxin internally. The toxin then migrates to where nerve endings meet muscle cells, blocking the nerve cell’s signals. The muscles become weak or paralyzed, usually starting with the neck and working downward. (See graphic inside.)

Researchers still aren’t 100 percent clear on why the spores can germinate in infant colons, but not in older individuals. Nor are they sure how most babies are ingesting the spores.

About 10 percent of honey contains botulism spores, which is why experts advise against feeding honey to children under the age of 1. But the spores can also be found in dust and dirt, and in most cases, doctors cannot determine the source when an infant is sick.

Josiah’s ordeal started on a weekend in late June, when the entire Ferguson family — parents Shari and Matthew, and two older children — came down with a cold. Josiah picked up a case of pink eye and developed infections in both ears.

By that Monday, the infant stopped nursing and the next day he wouldn’t lift his head up anymore. Shari took him to the doctor on Wednesday, who diagnosed the ear infections. But later that day, Josiah just didn’t look right.

“He had a weird noise coming from his chest, and he was grayish-blue,” Shari recalls. A friend drove them to St. Charles Redmond just in the nick of time.

As they arrived, Josiah stopped breathing.

“If it had been a minute longer,” she said, “it would have been a much different story.”

The emergency department doctors stabilized the infant, put him on a ventilator and sent him by an Airlink Critical Care Transport helicopter to the hospital in Portland. There doctors confirmed that Josiah had a respiratory infection.

“That’s the main thing they were thinking about first,” Shari said. “It really threw everybody off.”

The infection cleared over the next two days, but when doctors tried to remove him from the ventilator, they couldn’t get him to breathe on his own.

Lindsay first saw Josiah after midnight on Saturday. He immediately suspected infant botulism and called the Infant Botulism Treatment and Prevention Program part of the California Department of Public Health. The program developed and distributes the only antidote to the toxin that causes botulism in infants.

The doctors in California urged Lindsay not to wait for laboratory tests to confirm the diagnosis. The antidote, known as BabyBIG, works best when given within the first three days, and still has benefits on days four to seven. After a week, however, it adds little. Josiah’s symptoms fit the condition. “I decided to pull the trigger,” Lindsay said.

There were consequences if he were proved wrong. The drug costs $45,300 per treatment, and once the medication leaves the facility, it cannot be returned. Because it is manufactured and distributed by a public health organization, rather than a drug company, the program requires a hospital administrator to sign a purchase agreement pledging to wire the payment within five business days.

Now well into the early morning hours, Lindsay had to wake the hospital’s administrator to sign the paperwork, arrange for the medicine to be released from the warehouse, flown to Portland and delivered by medical courier to the hospital. By noon the next day, he had the vial in his hands.

“I keep on saying, ‘If you need a man to move mountains, you call that guy,’” Shari said.

New treatment

As recently as a decade ago, Josiah’s case might have gone much differently. Although the vast majority of botulism cases occur in infants, until recently there was no antidote to the toxin available for them. The military had developed an antitoxin for adults in 1978, and California public health officials wanted to test that in infants. But just as they received approval, Iraq invaded Kuwait, and the military redirected its supply to the Persian Gulf, where they feared the botulism toxin could be used as a biological weapon.

By that time, however, many individuals in the California Department of Health had been immunized with an experimental botulism vaccine, and they volunteered to donate their plasma so the immune globulin that counteracts the toxin could be used to make a botulism treatment for infants. The product was licensed as BabyBIG by the Food and Drug Administration in 2003.

“BabyBIG, it’s the greatest stuff on earth,” said Dr. John Chunn, a pediatriacian with Central Oregon Pediatric Associates, who has treated about a dozen cases of infant botulism over his nearly 40 years of practice in Bend. “Before the evolution of BabyBIG, most of them would be in the hospital for two months.”

Eventually, the body clears the toxin even without an antidote, but that process can take months. And the longer a child remains on a ventilator, the greater the risk for complications.

In clinical trials of BabyBIG, which included cases treated at St. Charles Bend in the late 1990s, the treatment cut hospitalization time from an average of 5.7 weeks to 2.6 weeks, with similar reductions in ICU time, ventilator use and complications. The savings in hospital costs, Chunn said, more than offsets the high price of the drug.

Josiah is covered by the Oregon Health Plan, and the Fergusons expect OHP will pay for the treatment.

“We actually have no idea,” Shari admitted. “We didn’t ask.”

They had little choice. The botulinum toxin has a paralytic effect, which is one of the reasons it’s used to relax facial wrinkles in the cosmetic product Botox. By the time, Josiah received his treatment, he had ceased almost all movement. He has made slow but steady progress ever since.

Before they can take him home, Josiah will have to relearn how to eat on his own, how to swallow without aspirating his food, even how to hold his head up again. He is undergoing regular physical therapy and is still getting most of his calories through a nasal tube.

“You go from, he’s not moving his eyes to look from side to side, to the next day when he can move his eyes and slightly move his fingers,” Shari said. “It’s been gradual but every day, a little bit more.”

She is convinced the worst is behind them and they are looking forward to going home.

Lindsay credits the emergency room doctors in Redmond for stabilizing the infant and the Airlink team for successfully transporting the child in such a fragile state. Without their work, he wouldn’t even have had a chance to make his diagnosis.

“That’s quite a feat,” he said. “There’s a lot of places that don’t have that capability.”