If your body is a house, think of your nerves as wires. Much like the wires running to your television, your nerves are covered with a critical layer of insulation that protect them — and your body — from danger.
The insulation, known as myelin, is a collection of lipid, fats and proteins that considerably increases the speed of nerve signals. Damage — or demyelination — short-circuits the central nervous system.
|Pediatric Neurologist Jayne Ness works with a patient in the Center for Pediatric Onset Demyelinating Disease clinic. Ness is the director of the clinic — the only one of its kind in the Southeast.
Demyelination may be a one-time event involving the spinal cord (transverse myelitis), or nerves to the eyes (optic neuritis) or multiple regions within the brain (acute disseminated encephalomyelitis). However, multiple bouts of demyelination can occur, raising the possibility of Multiple Sclerosis (MS).
UAB’s Center for Pediatric Onset of Demyelinating Disease (CPODD) is the only clinic in the Southeast dedicated to treating children with these conditions. Funding for the center began in 2006 with a five-year grant from the National Multiple Sclerosis Society, which declared the group one of six Centers of Excellence and created the network of Pediatric Multiple Sclerosis Centers in the United States.
“Our goal is to provide comprehensive, multidisciplinary care for children with MS and other demyelinating disorders,” says Jayne Ness, M.D., Ph.D, the pediatric neurologist who directs the clinic. “The six centers work as a collaborative group. We hope that working together will help us resolve some questions about MS.”
The centers are working to accomplish three things:
- Improve evaluation and management strategies to enhance diagnosis and care of children with MS and other related disorders.
- Develop resources for families, health-care professionals and the public.
- Collect data that will enable large-scale research initiatives.
Data collection is crucial to the enterprise given the rarity of the diseases. For example, Ness says, MS affects approximately 400,000 people in the United States. It is estimated that 2 to 5 percent of those affected had symptoms before age 18. The number is closer to 2 or 3 percent in the South, she says.
CPODD has evaluated more than 150 patients from 12 states in the Southeast since 2006. Patients, who must be age 18 or younger, may be referred by physicians or make a direct appointment.
Patients receive a comprehensive evaluation that in-cludes a detailed medical exam, cognitive testing and psychological evaluation. Treatment recommendations are tailored to address medical needs plus rehabilitative, psychosocial and educational issues.
“We’re available for consultation and have a support system in place for families,” Ness says.
“There’s much anxiety, and many of our patients are depressed or fatigued. We really try to provide multidisciplinary and comprehensive care to the child and the whole family because everyone is affected by the diagnosis.”
When to treat
Demyelinating diseases are diagnosed by careful review of the patient’s history, laboratory testing and MRIs of the spine and brain. Sometimes repeated exams and testing are required to determine if a child with an initial demyelinating event will develop MS.
Early recognition and treatment can limit risk of future attacks and neurologic disability. Though pediatric MS patients and their families always are relieved to hear that MS is not fatal, there always is disappointment that MS will require lifelong therapy.
Current treatments are available only as injections into the skin, muscle or vein. Determining whether or not treatment is necessary is the most difficult part of the decision-making process. Some children experience multiple demyelinating episodes without developing MS.
“That’s the conundrum: When do you treat?” Ness says. “With kids you just have to be so careful because there are things that look like MS but are not. There’s probably a difference in MS risk for kids who are pre-pubertal versus kids who are post-pubertal. It also may be that there are infectious triggers or autoimmune triggers, and examinations of pediatric MS patients who are closer to the onset of disease might offer understanding into causes for both kids and adults.
“That’s why we hope this collaboration will help us resolve some of the many questions we have about MS.”
The CPODD team includes clinical faculty from Pediatric Neurology, Adult Neuro-Immunology, Neuro-Psychology, Child Psychiatry, Neuro-Ophthalmology, Pediatric Urology, Pediatric and Neuro-Radiology, Pediatric Rehabilitation and Physical and Occupational Therapy.
Parents can ask their pediatrician for a referral to the clinic for an evaluation. Call 996-7633, e-mail firstname.lastname@example.org for more information or visit www.uab.edu/cpodd.
The other Centers for Excellence are UCSF Regional Pediatric MS Center in San Francisco; Partners Pediatric MS Center at the Massachusetts General Hospital for Children in Boston; Mayo Clinic Pediatric MS Center in Rochester, Minn.; Pediatric MS Center of the Jacobs Neurological Institute in Buffalo, N.Y.; and the National Pediatric MS Center at Stony Brook University Hospital in Stony Brook, N.Y.