NeuRx Diaphram Pacing System (DPS) Use in Amyotrophic Lateral Sclerosis (ALS)

Multi-Center, Concurrent-Controlled, On-Label Study of the NeuRx Diaphragm Pacing System (DPS) in Participants With Amyotrophic Lateral Sclerosis (ALS)

The purpose of this research study is to collect more information about the use, safety, and effectiveness of the NeuRx DPS® in ALS patients.

Characterize the safety and benefit of on-label Humanitarian Device Exemption (HDE) use of DPS in patients with ALS who initiated noninvasive ventilation (NIV).

Inclusion Criteria:

• Age 21 or older

• Familial or sporadic ALS diagnosed as possible, laboratory-supported probable, probable, or definite according to the World Federation of Neurology El Escorial criteria

• Bilateral phrenic nerve function clinically acceptable as demonstrated by bilateral diaphragm movement with fluoroscopic sniff test or with phrenic nerve conduction studies

• Chronic hypoventilation was documented by at least one of the following:

  • MIP (respiratory muscle strength) less than 60 cmH2O, or
  • Forced Vital Capacity (FVC) less than 50% predicted, or
  • PaCO2 greater than or equal to 45 mmHg, or
  • Nocturnal SaO2 (arterial oxygen saturation) less than or equal to 88% for at least five continuous minutes

• Initiation of non-invasive ventilation at or before time of enrollment

• Suitable surgical candidate to receive diaphragm pacing stimulation

• Negative pregnancy test in female participants of childbearing potential (treatment group)

• Informed consent from patient or designated representative

Exclusion Criteria:

• FVC less than 45% predicted within 10 days prior to surgery.
• Participation in simultaneous therapeutic clinical trial which could affect the safety or benefit outcomes of this study.