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 40 years.

Some of the symptoms are:

  • Slurring or thick speech
  • Tripping easily
  • Impairment of arm or leg usage
  • Difficulty in projecting speech
  • Twitching in hand and feet
  • Difficulty swallowing
  • 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.

Diagnosing ALS

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)
Spinal tap
X-rays
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.

ALS Clinic

Although there is not yet a cure for ALS, one of the most successful treatment regimens
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.

Educational Links

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.

www.catfishchapter.org

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.

Other links of interest include:
www.alsa.org
www.als.net
www.ninds.nih.gov
www.alsforums.com
www.als-mda.org
www.wfnals.org
www.rideforlife.com

 

 

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