Currently, the bulbar-onset pacer table is updated to show the bulbar pacer group median now between thirty-one and twenty-eight months. While the datasets referenced by Noah Lechtzin, MD, MPH in their August 2006 journal paper can be historical controls, their associated studies did not exclude best patients; such datasets need 'handicapping' for comparison to the groups in the DiPALSs' study. Researchers responsible for those datasets might identify a 'handicapped' median after excluding the best twenty-five percent of the study patients . . . undoubtedly the DiPALS study would increase in 'rank' were the table amended accordingly because the historical 'handicapped' medians are all but certainly much lower than their full count median. As is it bests about one-third of the historical datasets whereas the uncontrolled studies practically best all of the historical datasets. If the best 25% pacing Amyotrophic Lateral Sclerosis patients were removed from the Bulbar table, its 'handicapped' median would fall to approximately the Turner 2010 median, implying little if any efficacy.



Bulbar onset table . . . :

**



Historical table of datasets plausibly usable as historical control . . . :

**



Some words of caution on FVC

A patient and facebook member indicated they desisted using the device because their FVC declined to 20% . . . There are two well known FVC levels associated with the pacer decision . . . one threshold is FVC less than 85% because some involvement of respiratory neurology is prerequisite for pacer implant. Another threshold is FVC less than 45% - a different concern at this level - the concern is whether the patient's surgical risk is too high. The device may be effective even when FVC has dropped to 20%. . . successful implantation was achieved in patient with only 26% FVC. Diaphragm(s) either adequately respond to the stimulation or they do not. If one of the two diaphragm muscles is 100% healthy, the pacer ought to be able to cause at least 20% FVC to occur but not much more than 25%. The exploratory component of the implant surgery is definitive. The responsiveness of the diaphragms to the surgeon's stimulation is charted; if there is sufficient response and no complications are encountered the implantation can go ahead as the pacer will be able to provide adequate stimulation. How much of the full potential value of the pacer will a patient realizes depends on how close to the optimal moment the implant is made and the rate the patient's diaphragm muscles are regressing.



My understanding is any injury possibly related to the pacer is subject to the reporting requirements of the United States Food and Drug Administration's "Manufacturer And User Device Experience" (US FDA MAUDE service). As of Christmas, 2017 there were nineteen reports involving about fifteen patients; thirteen resolved but there were two 'unexpected' deaths - neither were tied to deficiencies of the device.

One patient apparently died because the device was not activated or became deactived somehow . . . the patient might be a spinal cord injury patient . . . the device was sent for independent appraisal rather than to the manufacturer . . . their independent appraiser's report indicated the device was 100% operational.

The other patient incurred a pulmonary embulism the day after successful pacer implantation . . . the patient received an Amyotrophic Lateral Sclerosis prognosis but declined 'CAVA' filter and died from the embulism - the investigation report does not indicate problems with the pacer implant. Plausibly, the patient injured their leg on return home (I HAVE NO INSIDE INFORMATION ON EITHER OF THESE CASES). There is no way I can master the extensive literature on haematology, however I could find no papers deeply addressing blood health and Amyotrophic Lateral Sclerosis; the primary concern in Amyotrophic Lateral Sclerosis has always been clot prevention.

To the best of my knowledge and belief, neither patient was member of any amyotrophic lateral sclerosis social media web sites. The following URL provides access to the MAUDE database:



MAUDE - Manufacturer and User Facility Device Experience

{add "synapse biomedical" to the manufacturer field, set the 'start date' to any date before the millennium turned, set the 'end date' to any date later than your "today" and set the number of reports per page to twenty-five. 'Click' [search].}



Having a trach does not necessarily disqualify one from the pacer.



URLs for Some Important Publications:



. . . 2017 . . .

Completed FDA feasibility trial of surgically placed temporary diaphragm pacing electrodes: A promising option to prevent and treat respiratory failure

Completed FDA feasibility trial of surgically placed temporary diaphragm pacing electrodes: A promising option to prevent and treat respiratory failure - ScienceDirect



December, 2015 Courtesy of ALS Worldwide: Dr. Onders explains DPS in ALS

ALS WORLDWIDE: Diaphragm Pacing Stimulation System - YouTube



September, 2015 The Lancet Neurology, DiPALS Writing Committee & Study Group Collaborators, (n=74)

Safety and efficacy of diaphragm pacing in patients with respiratory insufficiency due to amyotrophic lateral sclerosis (DiPALS): a multicentre, open-label, randomised controlled trial.

Safety and efficacy of diaphragm pacing in patients with respiratory insufficiency due to amyotrophic lateral sclerosis (DiPALS): a multicentre, op... - PubMed - NCBI



March, 2015, AJS Volume 209, Issue 3' University Hospitals's Raymond Onders, (n=53)

Identification of unexpected respiratory abnormalities in patients with amyotrophic lateral sclerosis through electromyographic analysis using intramuscular electrodes implanted for therapeutic diaphragmatic pacing

Identification of unexpected respiratory abnormalities in patients with amyotrophic lateral sclerosis through electromyographic analysis using intramuscular electrodes implanted for therapeutic diaphragmatic pacing - ScienceDirect



March, 2014 (n=16) Onders, et alia

Final analysis of the pilot trial of diaphragm pacing in amyotrophic lateral sclerosis with long-term follow-up: diaphragm pacing positively affects diaphragm respiration.

https://www.ncbi.nlm.nih.gov/pubmed/24439161



September, 2012, (UMich: Gruis, MD, MS & Johns Hopkins: Lechtzin, MD, MHS)

RESPIRATORY THERAPIES FOR AMYOTROPHIC LATERAL SCLEROSIS: A PRIMER

https://deepblue.lib.umich.edu/bitst...=1&isAllowed=y

https://www.ncbi.nlm.nih.gov/pubmed/22907221



September 28, 2011 US FDA HUD/HDE Approval



March 2009, American Journal of Surgery, Onders, et alia (n=51)

Amyotrophic lateral sclerosis: the Midwestern surgical experience with the diaphragm pacing stimulation system shows that general anesthesia can be safely performed

https://www.sciencedirect.com/scienc...02961008008003



August, 2006, Johns Hopkins's Noah Lechtzin, MD, MPH

Respiratory effects of amyotrophic lateral sclerosis: problems and solutions.

https://www.ncbi.nlm.nih.gov/pubmed/16867198



January 2006, Muscle Nerve, Schmidt . . . Lechtzin (n=95)

Pulmonary predictors of survival in amyotrophic lateral sclerosis: use in clinical trial design.

https://www.ncbi.nlm.nih.gov/pubmed/16258948



March 2002, Lechtzin, Rothstein

Amyotrophic lateral sclerosis: evaluation and treatment of respiratory impairment.

https://www.ncbi.nlm.nih.gov/pubmed/12061943



February, 2001, N Engl J Med, Lechtzin et alia

A Fatal Complication of Noninvasive Ventilation

http://www.nejm.org/doi/full/10.1056...00102153440718



February, 1995, Tohoku Journal of Experimental Medicine, Handa I, et alia (n=1)

A clinical trial of therapeutic electrical stimulation for amyotrophic lateral sclerosis.

https://www.ncbi.nlm.nih.gov/pubmed/7597693



February, 1993, Chest, Schiffman PL, Belsh JM (n=36)

Pulmonary function at diagnosis of amyotrophic lateral sclerosis. Rate of deterioration.

https://www.ncbi.nlm.nih.gov/pubmed/8432145



Since 1973 - current status: UNK

William Kibbie: ALSisnotfatal.com