Protocol for the Use of the Diaphragm Pacing System in ALS Patients (HUD) - FDA Approved
This study is to be used as a treatment compassionate use of a HUD to treat patients with ALS. The study design consists of a pre-operative clinic visit where the patients will receive a complete history and physical exam including evaluation of the diaphragm which is routine standard of care with this patient population. The consent process will also take place during this visit. Standard guidelines for pre-operative testing will be followed. The HDE patient instruction manual and product labeling will be reviewed (these are attached). The only difference in these documents is that DP will only be used for conditioning because the patient is not on a ventilator. Post-implantation, the patients will be followed by the PI and co-investigators. They will continue to be followed by their primary physicians who have managed them since diagnosis. Along with the PI, the diaphragm pacing team will establish a conditioning regimen for the patient with follow-up as needed. No follow-up data will be collected for purposes of this study. The only data to be collected will be for device accountability.
Patients who are 18 years of age or older with chronic hypoventilation due to the effects of ALS, who are clinically stable. TO be defined as having chronic hypoventilation, the patient must meet one of the four criteria:
1. FVC less than 50% of predicted
2. MIP less than -60cm H2O
3. PaCO2 greater than or equal to 45mmHg
4. SaO2 less than 88% for 5 consecutive minutes during sleep.
Also, both right and left phrenic nerves must be at least partially working to be considered for the pacing system.