H1N1 hits children, teens with neuromuscular disorders hard

From USA Today:

Despite his cerebral palsy, Derek Collette (pictured) never lagged very far behind.
He rode the school bus with other special-needs children, hustled to class on crutches and got decent grades for a child with a learning disability, if not on par with those of an average 13-year-old.

Then, in May, the first wave of swine flu hit Forest Grove Middle School in Worcester, Mass.

It swept Derek under. Doctors say the H1N1 influenza virus somehow inflamed the nerves in his spine, crippling him.

Derek's plight may be extreme, but it isn't unique. He is one of thousands of children and adolescents with neuromuscular disorders, asthma and other conditions who are suffering consequences of H1N1 that will linger long after the 2009/2010 swine flu pandemic ends.

Now Derek can no longer go to school. He uses a wheelchair and is in chronic pain, his legs locked and contorted, his toes curled in an endless muscle spasm. He can barely tolerate shoes, much less his mother's struggles to wrestle the 180-pounder from his wheelchair into the car.

"My muscles get so, so tight," Derek says. "Really hard squeezing. I can only bend down so far. It's excruciating to me."

Says his mother, Christina Collette: "It's a terrible thing. I hate to get him up in the morning, because it hurts him so badly."

On the other hand, she says, "we're lucky he's alive."

The 21st century's first flu pandemic will be remembered for popping up unexpectedly, like a spring storm, just as the previous flu season was drawing to a close.

It's also memorable for being far more transmissible and much less lethal than the H5N1 bird flu that cropped up in 1996 and has killed half of the 500 people worldwide who have been infected. In contrast, swine flu has infected millions, though the number of cases has dropped sharply since mid-November.

As of Jan. 16, an estimated 57 million people have developed swine flu in the USA, and about 11,000 have died, according to the latest calculations by the Centers for Disease Control and Prevention. But what may be most striking about swine flu is the toll it has taken on children.

All told, CDC says, roughly 19 million children up to the age of 18 were infected by the virus. An estimated 82,000 were hospitalized, two to three times the number admitted in a typical flu year, says Lyn Finelli, head of the CDC's swine flu surveillance and response team.

The childhood death toll is high too, Finelli says, with two to three times as many confirmed swine flu deaths as in a typical flu year, 324 vs 74. But, because only a fraction of those who contract the flu are tested for it, the actual death toll among children is likely even greater, an estimated 1,250, according to CDC's latest tally.

There's another puzzle, she says: In some young people, including Derek, H1N1 damages the central nervous system far more often than other forms of flu.

CDC statistics bear that out, Finelli says. During a typical flu season, one of every five childhood flu deaths occur in children with a neuromuscular disorder. "This year, it's almost one of every two," she says.

"We've been looking at the cases closely, each one of them, to see if there are underlying commonalities to explain them," Finelli says. "We're not seeing any patterns, except that we're seeing an awful lot of kids with neuromuscular disorders."

No one knows exactly why flu has such a devastating effect on children with neuromuscular disorders. But the evidence has been mounting since late 2005, when Ron Keren and Susan Coffin of the Children's Hospital of Philadelphia and their colleagues reported that, even in typical flu years, children with these ailments were six times more likely than other children to develop severe complications from flu.

Edwin Travathan, director of CDC's National Center on Birth Defects and Developmental Disabilities, says that people with conditions that affect movement and other motor skills "take a harder hit" from influenza because they become so weak that they may lose their ability to cough and clear their lungs.

People with cerebral palsy, like Derek, are typically born with impairments in regions of the brain that control movement and sometimes language or cognition. Their muscles contract in painful spasms, he says. Muscular dystrophy, a genetic condition that leads to a gradual breakdown of muscle, is much less common, but these children also are at high risk, Travathan says.

Based on that evidence — and early reports of the effect of swine flu on children — the CDC's Advisory Committee on Immunization Practices recommended in June that the agency place children high on the H1N1 vaccine priority list. At the time, no swine vaccine was available, and none would be until the fall, when vaccine makers began slowly churning out their supplies.

In June, for the first time in half a century, the Muscular Dystrophy Association canceled its summer camps, fearing fatal outbreaks of flu. Kimm Echkoff of Birmingham, Ala., says her daughter Demi, 17, who has muscular dystrophy, was "heartbroken" at the news. Then, she says, when vaccine began trickling in in the fall, "I couldn't find it." Demi and her sister Ainsley, 5, both contracted H1N1. Demi landed in the hospital, twice. Her lung collapsed. It took five weeks for her to recover, Echkoff says.

State vaccine coordinators, charged with supplying vaccine to those most in need, found themselves juggling competing demands. Health officials caring for high-risk children often found themselves waiting in line.

Rick Rader, director of the Morton J. Kent Habilitation Center in Chattanooga, a day program for children and adults, says his team requested 1,100 doses of vaccine for his direct-care staff and clients. They got 200 "and no promise that more would be forthcoming," he says.

He contacted Tennessee's vaccine coordinator, Kelly Moore, and asked whom he should vaccinate — the health workers that care for clients or the clients who were vulnerable to infection?

"I asked, 'How much do you need?' " Moore says. "As soon as we realized folks with neurodevelopmental disorders were having problems (with flu), we were getting vaccine to them as soon as possible."

Vaccine came too late for Derek Collette (pictured), who apparently caught the virus in May at school. His first flu test was negative, and the pediatrician sent him home.

Two days later, his fever rising and his breath coming in gasps, his mother called an ambulance, which took him to the University of Massachusetts Memorial Hospital in Worcester. Doctors kept Derek overnight, gave him Tamiflu and sent him home. A week later, the family learned the results of Derek's second flu test, which was positive.

At first he seemed better, but then the downward spiral began. "He was catatonic," she says. "You could talk to him and he would stare as if he didn't know where he was. I couldn't touch his skin — it would hurt. He would sleep all day and all night."

By the end of June, Collette took her son to the emergency room at the university hospital and said she wouldn't bring him home until he was better. After a week, the virus relented. Derek was transferred to Franciscan Children's Hospital in Boston for rehabilitation, where he stayed for a month. Christina has spent so much time caring for her son that she was asked to resign from her job.

Her husband has been struggling to support the family, including Derek's sister Catherine, 5, and pay thousands in medical bills on a policeman's salary. Only fundraisers enabled the family to buy a wheelchair. Someday, Collette says, she hopes the Derek Collette Fund, established at the Webster First Federal Credit Union, will collect enough money to enable the family to buy a wheelchair van so she no longer has to wrestle Derek from his chair in the car to leave the house. Meanwhile, his medical bills mount.

"They already cost probably hundreds of thousands of dollars," says Jeffrey Forman, Franciscan's director of rehabilitation medicine, who has taken a personal interest in Derek's case.

For months, he has been researching options that might help Derek. It came down to only one. On Jan. 29, surgeons at Tufts Medical Center in Boston implanted a pump in Derek's abdomen that slowly infuses a drug called baclofen directly into his spine. The drug blocks signals from his brain that force his muscles into painful contractions. Forman hopes the drug will relax Derek's muscles and relieve his pain. If that doesn't work, Forman says, it might be necessary to add morphine.

Despite Derek's discomfort, Collette says, the operation has given Derek hope. "I can't believe how great he's been," she says. "He's working so hard. He said to my husband the other day, 'I'm going to work really hard and get out of here by my birthday.' " Derek's birthday is April 2, about two weeks before the first anniversary of the outbreak.