LaMay Axton of Menifee, California, remembers every detail of Sept. 23, 2016, her own birthday, when life changed forever for her granddaughter, Cambria "Cami" Tate, 2 1/2 years old. LaMay, who is Cami's caregiver, says the child originally had just another cold, before things took a more terrifying turn. Cami's fever was 103 degrees and "her body seemed to be super-rigid and she couldn't cough up secretions."
After a hasty trip to the doctor, Cami was sent home with a bronchitis diagnosis: "I think God told me to sit down and watch Cami that day," LaMay told HealthCentral in a telephone interview. She obeyed without hesitation. "Later, her little legs gave out from under her, and she looked like a marionette doll."
LaMay rushed Cami to a San Diego military hospital, where the child went into respiratory failure and was without oxygen for 33 minutes, as her grandmother prayed: "God, please don't take her." Doctors "brought her back" but Cami was paralyzed from the neck down and in a medically-induced coma for three months.
Now at age 4½, Cami can't walk without assistance because of her flaccid right leg, trunk — her pectoral muscles won't return to normal — and her right arm. She goes to physical and occupational therapy for help with movement and for her scoliosis, for which she'll have surgery in a year or two. She also has gastrointestinal reflux disease due to gastroparesis, or delayed gastric emptying.
The inquisitive and precocious Cami was ultimately diagnosed with acute flaccid myelitis (AFM) — a very rare but serious neurological condition that affects gray matter in the spinal cord, weakening muscles and reflexes. It's often compared with polio, and last year, 223 cases were reported in the United States. Experts estimate AFM affects fewer than one in a million children here every year, with the average age being 4 years old. According to the Centers for Disease Control and Prevention (CDC), there have been increases in AFM every two years since 2014, with 149 confirmed in 2016, but only 35 cases in 2017.
How to understand AFM
To learn more about AFM, HealthCentral spoke by phone to Benjamin Greenberg, M.D., M.H.S., a member of the AFM task force at the CDC. He is vice chair of translational research and the Cain Denius Scholar in mobility disorders at The University of Texas Southwestern Medical Center, in the departments of neurology and neurotherapeutics and of pediatrics.
Dr. Greenberg explains that viruses probably cause AFM, usually arising between July or August and November, when enteroviruses like this circulate.
Enteroviruses cause various infectious illnesses that can present with coughing, sneezing, and fever, but if they infect the central nervous system, serious illness can result, as is suspected in the case of AFM.
All viruses are seasonal, he explains. "Viruses that are able to survive outside the human body are easier to spread, and their seasonality is dictated by their chemical properties.”
“Think about flu — we don't have the same influenza virus every year, and different enteroviruses circulate during different years,” he says. “This one [the virus associated with AFM], enterovirus D68 (EV-D68), is more prevalent every other year."
"It's important to understand that AFM is a form of transverse myelitis, or inflammation of the spinal cord," says Dr. Greenberg. "We suspect the virus for AFM has been around a long time, knowing also that viruses mutate, and this one did."
Experts agree that there's no consensus on why one child is at higher risk for AFM while another is not, and they have no data to predict long-term consequences. They do agree that if a parent sees any sudden weakness of limbs, they should seek medical care immediately.
Help for AFM on the horizon
Dr. Greenberg knows we haven't seen the last of AFM, and he’s working on a grant to build infrastructure to manage data on the impending 2020 outbreak.
"We want to be able to track these cases in real time," he says. "We need staffing, with coordinators to make contact with all the families as cases are being reported. Then we will get them enrolled in a prospective study, collect laboratory samples, and do assessments on a regular basis,” he says
He sees room for improvement in several key areas.
"After the acute event, when children are left with significant weakness or paralysis, function can be recovered with intensive rehabilitation over a prolonged period of time and years," Dr. Greenberg says. "They may not return to 100-percent normal, but they can achieve normal function even with isolated deficits. We need two things. One is for insurance companies to approve rehabilitation on an ongoing basis. It makes no sense to put a cap on the time allowed for this."
The second need is to stop the spread of incorrect folklore about AFM that even some clinicians perpetuate, he says.
"They say that whatever improvement you have after a year is all you will get, and that is factually incorrect. You must keep doing the hard work of rehabilitation, looking ahead to when the child is going to be 20 or 40 years old,” says Dr. Greenberg.
He also supports more research investigation into variants or sub-types of AFM to determine which therapies, such as anti-inflammatories, may work best for certain patients. And he's advocating for stem-cell-based trials to restore muscle contractions when a person becomes paralyzed from AFM.
Previous research has focused on approaches for “normal” infectious diseases, and even environmental agents are on the "possible" list as being a cause of AFM, the CDC has said.
What families should know and do abut AFM
"My colleagues and I are awake at night, thinking and trying to make sure this doesn't become the next polio," he says.
Meanwhile, Dr. Greenberg makes himself available via email or phone call, 24/7, to healthcare providers worldwide who suspect a person has AFM, often suggesting a transfer to a referral center or large hospital with more experience dealing with this often-mystifying condition.
"Families have a right to simply request a transfer to another facility," he says. "Doctors recognize when a patient might be better served somewhere else."
As a member of the Transverse Myelitis Association Board of Directors, Dr. Greenberg urges anyone, especially parents of children with AFM, to use the resources of the association, which include a helpline, support group network, resource library, and an active group of Hope Ambassadors who will speak with parents. Start there to find other families who have "been there, done that," he says, and "get linked up" to people in your city or state.
That's a recommendation seconded by LaMay Axton, who urges parents to "be a voice for your child, and to understand the signs and symptoms. You may have to be forceful when you interact with healthcare professionals, since you know your child — and when something is wrong with them — better than anyone else."
LaMay has taken her advocacy around AFM to discussions with officials and researchers at both the CDC and National Institutes of Health, and she’ll continue to work with them this year. Along with other parents and doctors, she has criticized the CDC's tardiness in tackling AFM and not welcoming useful data from families affected by the disease.
"Now the national effort is moving in the right direction, and I'm so impressed with the outreach from the California Department of Health," she says. "They've set an example and have brought such value to the community across the board."
Here's the department's resource page, with links to other valuable information, along with names, emails, and phone numbers.
Where social media fills gaps about AFM
Social media efforts have connected parents of kids with AFM to resources when national associations, medical institutions, and other networks have fallen short.
In her piece “Social Media in the Age in of the New Polio,” published in the New England Journal of Medicine, Riley Bove, M.D., assistant professor of neurology at the University of California, San Francisco, and a parent of a child with AFM, describes social media’s role in helping parents get access to and leverage critical information. Social moderators have become, in essence, physician assistants, disseminating important information to parents in the group.
“They not only facilitate member entry, social cohesion, and dissemination of information about external resources (e.g., clinical trials or CDC case reporting),” Bove explains in the article, “but also seek pathways through which knowledge can flow back to medical professionals.”
As an example, she mentions a partnership with AFM specialists that developed as a Facebook group, where parents‘ working groups are led by healthcare providers. “Moderators have supported several initiatives to create ‘working knowledge’ through data repositories, using data from consenting members only, that will expand AFM case ascertainment and data generation,” she explains.
With any illness, know what you’re dealing with
Information is what any parent with AFM is after, whether it be through a support group, medical center, or social media network.
"Always remember, more knowledge is power," LaMay says.
In terms of prevention, the CDC reminds everyone to observe vigilant and correct hand washing — just as you would during cold and flu season —keep vaccinations current, and use insect repellent to help prevent mosquito bites because mosquitos could carry and spread the viruses that may cause AFM.
HealthCentral extra: Here's a YouTube video of LaMay discussing AFM.