Motor Speech Disorders 127Case Study 6-6: A 38- Year- Old Woman With
Amyotrophic Lateral Sclerosis
The early signs of disease were barely perceptible. Barbara was simply too busy taking care of
her two children, working part- time, and dealing with her failing marriage to focus on them. The
first indications Barbara could not ignore were the muscle cramping, weakness, and trembling in
her hands. Even her children commented on her shaking hands during mealtime. But it was the
chronic fatigue that took her to the doctor, and after a series of tests, she gave Barbara the bad news:
Barbara had Lou Gehrig’s disease— ALS.
Barbara met with a medical social worker and enrolled in an ALS support group. She learned
that ALS occurs less frequently in women than in men, and she was relatively young to have it.
ALS is a rare disease affecting about 1 in 100,000 persons. The hard real ity was that she would
not survive. According to the social worker, the average survival rate was about 3 years, although
some patients lived for 20 years or more. There were many theories about the cause or causes of the
disease, but no one knew what was causing Barbara’s muscles to waste away.
Over several months, the disease progressively impaired Barbara’s walking, dressing, and other
activities of daily living. The occupational and physical therapists helped to slow the effects of the
muscular degeneration and found ways to compensate for the functions she was gradually losing.
Barbara worked hard, but her increasing fatigue and the nausea caused by her strong medi cations
limited her participation in occupational and physical therapies.
As the disease progressed, Barbara’s speech began to degenerate. At first, a few consonants
were slurred, beginning with /t/ and /d/; others soon followed. Barbara began to drool and con-
stantly had to wipe her mouth. Her speech slowed and became monotonous, with no changes in
pitch. Her voice became weak, raspy, and nasal. She could not create the air pressure necessary
for intelligible speech due to the weak muscles and air leaking through her nose. Before Barbara
completely lost her voice, she voiced all speech sounds, even the voiceless ones. This was an auto-
matic compensatory be hav ior to increase loudness, but it also reduced her intelligibility. Together,
Barbara and the speech- language pathologist did all they could to stave off the speech degenera-
tion, but over several months it progressed relentlessly.
ALS is a devastating disease, not only physically but also psychologically. At first, when she
began to lose her speech, Barbara was intelligible. She could express her fears, anxiety, hopes, and
resolve to defeat this terrible disease. She could tell her children that all would be well, discuss with
her husband their care should she die, and participate in her church. Early in the course of the
disease, speech served power ful psychological functions. But during the middle and final stages
of the disease, she lost the ability to speak at a time when she most needed to reach out to others.
The social worker and members of the support group told Barbara’s family that she was likely
experiencing preparatory grief— she was preparing to be separated from all the things and persons
she loved. And although her marriage had been in trou ble before the onset of the disease, when
she lost the ability to speak, all hope of reconciliation was lost. In the final stages of the disease,
Barbara was even removed from the comforts of her home and placed in a nursing home for the
care she required.
The social worker and members of the support group understood the stages of grieving
Barbara would experience to achieve ultimate ac cep tance of the inevitable. At first, she denied that
something was seriously wrong and postponed going to the doctor. When the test results appeared,
she said that they referred to someone else. At times during the early stages, Barbara returned to
the comfort of denial; this was easy, because there were periods when she felt relatively normal.
Soon, however, denial gave way to anger and bargaining, marking Barbara’s frustration with this
insidious and pernicious disease.