Robert C. Frere, M.D.
Medical Director of ALS Clinic
What is ALS
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease
that affects nerve cells in the brain and the spinal cord. Officially, the name
comes from these Greek words:
“a” for without
“myo” for muscle
“trophic” for nourishment
“lateral” for side (of the spinal cord)
“sclerosis” for hardening or scarring
Essentially, it means that the muscles have lost their nourishment, and have
atrophied, or become smaller. The nerves that nourish the muscles are located
on the sides of the spinal cord, and the diseased part of the spinal cord develops
hardened tissue where healthy nerves should be.
Often referred to as “Lou Gehrig’s Disease” after the New
York Yankee hall-of-fame baseball player who was diagnosed with ALS in the 1930s,
this degenerative disease damages motor neurons in the brain and spinal cord.
The neuromuscular system enables our bodies to move. All normal everyday activities
such as lifting, walking, running, breathing, turning, swallowing, etc., are
all controlled by the neuromuscular system.
Symptoms of the Disease
At first, ALS symptoms may be so slight that they are overlooked. The rate
of progression also varies from person to person, as does the length of the disease.
The mean survival time for ALS is three to five years, although many people live
ten or more years. The most notable person living with ALS is renowned physicist
Stephen Hawking. Diagnosed at age 21, he has been living with the disease for
Some of the symptoms are:
Slurring or thick speech
Impairment of arm or leg usage
Difficulty in projecting speech
Twitching in hand and feet
Shortness of breath
The mind is not affected by the ALS. Individuals are able to think as clearly
as normal, although communication can be difficult, since the disease affects
breathing and the muscles controlling speech and arm movements. Also, the senses
of hearing, taste, touch, smell and sight are not affected.
A series of diagnostic tests must be completed to fully establish an ALS diagnosis,
as there is no one test to fully determine. A comprehensive battery of tests
normally include the following:
Electromyography (EMG) and nerve conduction velocity (NCV)
Blood and urine samples
Myelogram of cervical spine
Muscle and/or nerve biopsy
Full Neurological work-up
How is ALS Treated?
To date, no cure has been found. However, the first drug, riluzole, has been
approved by the Food and Drug Administration for treatment. Clinical trials show
that riluzole prolongs survival for several months, and offers hope that the
progression of ALS may one day be slowed by new medications.
Physicians can prescribe medications to assist with several of the symptoms,
including easing muscle cramps, reducing excess saliva, reducing fatigue, and
other prescriptions to help with depression, sleep and constipation.
Physical and speech therapy can also aid patients’ independence, while
low aerobic exercise such as walking, stationary bicycling and swimming can strengthen
muscles not currently affected.
Although there is not yet a cure for ALS, one of the most successful treatment
is a comprehensive clinic specifically designed for ALS patients and their families.
Fortunately, now such a clinic is operational in eastern North Carolina. The
ALS Clinic is a working partnership between East Carolina Neurology, Pitt County
Memorial Hospital, Duke University Medical Center, and the Jim "Catfish" Hunter
Chapter of the ALS Association. The clinic is held one day each month at the
Regional Rehabilitation Outpatient Center of Pitt County Memorial Hospital. It
is staffed by neurologists, Dr. Robert Frere of East Carolina Neurology and Dr.
Richard Bedlack of Duke University Medical Center. Additionally, ancillary staff
from Pitt County Memorial Hospital including physical therapists, occupational
therapists, social workers, dieticians, respiratory therapists, and others complement
the clinic staff. For more information or to schedule an appointment at the Clinic
please call East Carolina Neurology (252) 752-4848.
There are many internet resources available for more detailed education on
ALS. Among those are our local chapter, named after Jim “Catfish” Hunter,
a Hertford, NC native and former World Series Champion and Hall of Famer who
died from the disease in 1999.
This chapter offers many services including respite care grants, transportation
grants, three durable medical equipment loan closets and a series of ALS specific
support groups throughout North Carolina.