Like many parents, Bernard and Carlene had never heard of a rare disease called acute
flaccid myelitis (AFM) before the summer of 2016, when their 2-year-old daughter, Maipele,
started exhibiting some strange symptoms. Maipele, whose name is Polynesian for
“sweetheart,” had had a runny nose on May 21, which grew into a cough, followed a few
days later by a severe asthma attack. By the time Maipele reached a local emergency
department near the family’s home in Camarillo, she was in respiratory distress. After
doctors stabilized her, Maipele was airlifted to a hospital in Santa Barbara. A few days
later, back at home, Maipele “woke up as happy as can be,” recalls her father. She ran
through the house, laughing. It was then her parents noticed something odd: Maipele’s right arm seemed to dangle by
her side. They asked her to lift her arm. She couldn’t. “Her arm was like a dead weight,” says Carlene. “She wasn’t in
pain, but she could not move her arm at all.”
Viral Mystery
A rare, polio-like illness called acute flaccid myelitis appeared on the national radar
in 2014 and may be on track to return this summer and fall. Here is one family’s
story, and a look at CHLA’s innovative, multidisciplinary approach.
By Candace Pearson
Innovative surgery
AFM is a polio-like illness that scientists suspect has a viral origin. In its worst-case scenario, the disease can leave its
targets partially or totally paralyzed in one or more limbs. CHLA takes a multidisciplinary approach to AFM, bringing
together experts from Infectious Diseases, Neurology, Rehabilitation Medicine, Pulmonology, Surgery and more.
Though there is no effective medical treatment for the disorder yet, CHLA plastic surgeon Mitchel Seruya, MD, has
developed a workaround, refining a novel nerve-transfer surgery—borrowing nerves from the ribs and diaphragm in a
kind of “electrical bypass”—to replace the deadened nerves in the affected extremity. CHLA is the only place in the
western U.S. to offer this surgery for AFM. “Too often these kids and their families are being told that nothing can be
done about AFM except give it more time,” says Seruya, director of CHLA’s Brachial Plexus and Peripheral Nerve
Center in the Division of Plastic and Maxillofacial Surgery, and assistant professor of Clinical Surgery at the Keck
School of Medicine of the University of Southern California (USC). “We are a flagship for this type of management for
AFM.” But before Seruya and Maipele would meet, Maipele’s parents began an odyssey in summer 2016 to discover
what was wrong with their youngest daughter. Her illness struck at a busy time for the young family. Carlene was eight
months pregnant with their third child, while Bernard, Petty Officer - First Class, at the Naval Base Ventura County Air
Operations at Point Mugu, was in advanced training. An air traffic controller, he is also assistant radar branch chief.
The worried parents returned to the local emergency room, where doctors diagnosed a dislocated shoulder, which can
cause limb weakness. Next, the family saw their pediatrician, who started Maipele on a course of physical therapy.
An unexpected diagnosis
By mid-June, with Maipele no better, their pediatrician referred the family to Children’s Hospital Los Angeles.
By then, Carlene had given birth to their son, Elijah. She stayed home to care for him and their oldest daughter,
Rochelle, then 7, while Bernard and Maipele made the hour-and-a-half trip from Camarillo to CHLA. Finally, the mystery
had a name—acute flaccid myelitis. It hit hard. “We had so many questions,” says Carlene. AFM, marked by sudden
onset of limb weakness or paralysis, first appeared on the national radar when 120 cases were reported the summer
and fall of 2014. Two years later, in 2016, 149 people were confirmed with the disease. In the off years of 2015 and
2017, cases went down. So far, no one—not scientists, not clinicians, not the Centers for Disease Control and
Prevention (CDC)—understands why. The prime suspect is a viral infection, as a number of viruses can cause AFM
symptoms. The CDC reports that the 2014 emergence of AFM coincided with a nationwide outbreak of enterovirus D68
(EV-D68), associated with severe respiratory illness. Now officials are concerned that if AFM’s two-year cycle holds
true, the number of cases may rise again in 2018. The illness primarily affects children.
An uncommon condition
CHLA neurologist Wendy Mitchell, MD, cautions parents to keep in mind that AFM is extremely rare. “Fewer than one in
1 million people are affected,” she notes. But it is something to be aware of, she adds, “especially since we don’t yet
know why it seems to increase every two years.” AFM targets the gray matter of the spinal cord, Mitchell explains,
“where motor neurons reside, and so it affects movement and muscle control.” If children haven’t recovered within
three months after diagnosis, they typically face persistent paralysis, says Seruya. Doctors have yet to identify any
factors that point to one child having a better recovery than another. “It’s a tough thing for families,” he adds, “because
we have so few answers.”