PROJECT ABSTRACT
The goal of the proposed project is to provide Ms. Brianna Kiefer with training opportunities at Purdue and
Columbia University from Drs. Jessica Huber, Michelle Troche, and Kristine Marceau – experts in speech
science, respiration, cough physiology, and multilevel modeling. To achieve this goal, Ms. Kiefer will conduct a
study of expiratory muscle strength training (EMST) in people with Amyotrophic Lateral Sclerosis (ALS).
Progressive muscular atrophy is a detrimental consequence of motor neuronal death associated with ALS.
Atrophy of the respiratory musculature is particularly harmful since speech and cough production require ample
and coordinated respiratory support. Due to respiratory musculature atrophy, people with ALS are unable to
generate sufficient subglottal pressures to support speech. People with ALS are also unable to generate strong
expiratory forces to effectively expel foreign materials from the airway when they cough. Consequently, people
with ALS often rely on alternative communication devices and alternative methods of nutrition. Losing the
abilities to speak and eat independently significantly reduces quality of life for people with ALS. Recent
investigations have found that implementing moderate intensity strength training paradigms in the early stages
of ALS is safe and effective. EMST is a commonly used paradigm that strengthens the respiratory musculature
to improve speech and cough production. While EMST has a large body of literature to support its use for
people with other neurodegenerative diseases, only one study has examined the effectiveness of EMST for
people with ALS. The proposed project aims to build upon Plowman and colleagues (2016) findings to address
three broad aims. Specific aim 1 will define how the underlying respiratory support patterns mediating
speech and cough production are altered by ALS. We suspect that individuals with ALS will utilize less
efficient and effective respiratory support patterns during speech and cough production relative to age- and
sex-matched controls. Specific Aim 2 will determine how moderate intensity EMST modifies the
effectiveness and efficiency of speech and cough production. In a multiple baseline, delayed-treatment
design, people with ALS will undergo a six-week moderate intensity EMST protocol. We suspect that EMST
will improve the effectiveness and efficiency of speech and cough production. And finally, Specific Aim 3 will
identify how EMST affects the psychosocial states of people with ALS. We suspect that improved speech
and cough production will positively impact the psychosocial states of people with ALS. Together, results from
the proposed study will provide much needed information to guide clinical practice in the treatment of
dysarthria and dystussia associated with ALS. Through conducting this study and engaging with her mentors in
a variety of activities, Ms. Kiefer will greatly expand her research skills. Specifically, she will gain experience
with ALS, reflexive cough production, respiratory function, multilevel models, and clinical trial management.