Friedreich Ataxia
Conditions
Brief summary
This is a prospective interventional study of patients with Friedreich's Ataxia that receive respiratory strength training for a period of 12 weeks with two research visits at the beginning and at the end of the study period. Visits include swallowing evaluation with fiberoptic endoscopic evaluation of swallowing, pulmonary function testing, surface electromyography and patient surveys.
Interventions
OTHERRespiratory Strength Training (RST)
Respiratory Strength training involves inhaling/exhaling against a fix resistant to potentially increase respiratory strength
Sponsors
University of Florida
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
OTHER
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 65 Years
Healthy volunteers
No
Inclusion criteria
* confirmed genetic testing of Friedreich's Ataxia * have an overall DIGEST score of 1 or higher based on FEES screening * are able to perform pulmonary function testing
Exclusion criteria
* have been on antibiotics within 15 days prior to baseline screening. * have been prescribed systemic corticosteroids or neuromuscular blocking agents within 15 days of the baseline screening * have an allergy or contraindication to topical lidocaine or oxymetazoline * have any other concurrent medical condition which, in the opinion of the investigators, would make the subject inappropriate to participate in testing or respiratory exercise
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Force Vital Capacity | Through study completion (4 months) | Forced Vital Capicity will be measured before and after the 12 week intervention |
| Maximal Inspiratory Pressure (MIP) | Through study completion (4 months) | Maximal Inspiratory Pressure (MIP) will be measured before and after the 12 week intervention |
| Maximal Expiratory Pressure (MEP) | Through study completion (4 months) | Maximal Expiratory Pressure (MEP) will be measured before and after the 12 week intervention |
| Sniff Nasal Inspiratory Pressure (SNIP) | Through study completion (4 months) | Sniff Nasal Inspiratory Pressure (SNIP) will be measured before and after the 12 week intervention |
| Swallowing | Through study completion (4 months) | Fiberoptic Endoscopic Evaluation of Swallowing (FEES) will be measured before and after the 12 week intervention. |
| Surface Electromyography (sEMG) | Through study completion (4 months) | sEMG will be placed on the skin to measure muscle activity involved in breathing and swallowing |
| Diaphragm Ultrasound | Through study completion (4 months) | Diaphragm movement will be measured through an ultrasound probe positioned on the skin before and after the 12 week intervention. |
Countries
United States
Outcome results
None listed