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Remote pulmonary function testing in amyotrophic lateral sclerosis (ALS)

The speciļ¬c objective of this proposal is to validate the practice of remote pulmonary function testing (rPFT) through home measurement of breathing function. The central hypothesis is that guided home assessment of breathing function is a valid method for detecting respiratory distress. This study has the potential to transform the current practice of conducting breathing assessments every three months, resulting in timelier detection and management of respiratory distress. The study has two parts. [CLOSED TO ENROLLMENT] The first part is a self-controlled study which will enroll 40 patients from the ALS clinic. On the day of their clinical visit, study participants will perform both a standard PFT with a respiratory therapist and a simulated rPFT. The primary hypothesis is that there is no difference in the results of PFT and rPFT. The second part is a two-arm randomized intervention in 40 patients with ALS. All subjects will perform weekly home respiratory testing for up to a year, and complete questionnaires at regular intervals during this period. Part 2 also incorporates a nurse health coaching component, with subjects randomized to a group that recieves monthly nurse coaching, and one that does not.

Andrew Geronimo
Wint Nandar - at

18 year(s) or older
This study is NOT accepting healthy volunteers
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Inclusion Criteria:
Possess a diagnosis of amyotrophic lateral sclerosis (ALS)
Be 18 years of age or older.
Have a caregiver available to assist with home PFTs or, in the opinion of the investigator, can perform home PFTs unassisted.
Symptom onset within the last three years.
Have an internet-capable home computer and cellphone.

Exclusion Criteria:
Inability to perform pulmonary function testing with mouthpiece or with mask, as determined by one of the study investigators.
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Hershey, PA ,