The Evidence for Symptomatic Treatments in ALS
The Evidence for Symptomatic Treatments in ALS
Although beneficial, NIV is not without problems, as the masks can cause issues with claustrophobia, discomfort and act as a barrier to communication. NIV is not tolerated well in many patients with bulbar dysfunction. These issues have driven interest in alternative strategies to support failing respiratory muscles. Diaphragmatic pacing is an exciting new development. The diaphragm muscle is stimulated with surgically implanted electrodes through a pacemaker in the abdominal wall. Some residual diaphragmatic function appears necessary and general anaesthesia is required, which can be risky; careful patient selection appears crucial.
In the USA, the technique is available as a funded treatment, but effectiveness is yet to be established in RCTs. An initial pilot study of 16 patients reported qualitative improvements in diaphragmatic movement, thicker diaphragm muscles and nonsignificant trends towards a slower decline of FVC (−2.38%/month pretreatment and −1.34%/month posttreatment). Another study reported improvements in sleep efficiency measured with polysomnography. In contrast, the authors of another small series of eight ALS patients reported that insufficient tidal volumes could be achieved; no benefits in vital capacity, sleep or survival were found and their patients required NIV. Diaphragmatic pacing is now being systematically evaluated in a number of prospective studies.
Diaphragm Pacing
Although beneficial, NIV is not without problems, as the masks can cause issues with claustrophobia, discomfort and act as a barrier to communication. NIV is not tolerated well in many patients with bulbar dysfunction. These issues have driven interest in alternative strategies to support failing respiratory muscles. Diaphragmatic pacing is an exciting new development. The diaphragm muscle is stimulated with surgically implanted electrodes through a pacemaker in the abdominal wall. Some residual diaphragmatic function appears necessary and general anaesthesia is required, which can be risky; careful patient selection appears crucial.
In the USA, the technique is available as a funded treatment, but effectiveness is yet to be established in RCTs. An initial pilot study of 16 patients reported qualitative improvements in diaphragmatic movement, thicker diaphragm muscles and nonsignificant trends towards a slower decline of FVC (−2.38%/month pretreatment and −1.34%/month posttreatment). Another study reported improvements in sleep efficiency measured with polysomnography. In contrast, the authors of another small series of eight ALS patients reported that insufficient tidal volumes could be achieved; no benefits in vital capacity, sleep or survival were found and their patients required NIV. Diaphragmatic pacing is now being systematically evaluated in a number of prospective studies.
