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Diaphragm Pacing System Developed by Synapse Biomedical Continues Efficacy Trials

Synapse Biomedical is hard at work to discover how its diaphragm pacing system (DPS) can help people living with ALS. Used by the beloved Superman actor Christopher Reeve, the system was initially created to liberate people with spinal cord injuries from their mechanical ventilators for several hours per day. For many, the device allows those with spinal cord injuries to completely disconnect from their ventilators, as it works by bypassing the injured spinal cord and directly stimulates and contracts the diaphragm muscle to allow breathing.

For people living with ALS, the diaphragm pacing system, which must be surgically implanted using minimally invasive techniques is being investigated as a potential therapy to support the diaphragm muscle with electrical stimulation. Presently, scientists are working to definitively confirm whether this device will maintain the condition of the diaphragm (breathing muscle), have any adverse effects and identify what subset of people living with ALS will benefit.

Diaphragm Pacing System Shows Mixed Results

The U.S. Food and Drug Administration (FDA) previously approved the diaphragm pacing system (DPS) for use in individuals with spinal cord injuries in 2008. In 2011, the FDA approved the DPS under the Humanitarian Device Exemption (HDE) program for people living with ALS and chronic hypoventilation (reduced ability to breathe). The FDA’s approval was based on clinical trial results that were published on FDA’s website.

Results from several recent international studies have shown varying results. In September 2015, the Data Safety and Monitoring Board (DSMB) for the NEALS-affiliated randomized controlled trial (RCT) of DPS in ALS study being conducted in the United States issued a recommendation that new enrollment in that study be suspended. At the same time, the DSMB recommended that patients already participating in the study continue utilize their diaphragm pacing system according to the study’s protocols.

These DSMB recommendations were made after consideration of published data from DiPALS, a randomized diaphragm pacing trial in Great Britain, as well as press reports regarding a French study utilizing much different inclusion (patient participation) criteria than the U.S. study. Information from both of the studies conducted outside the U.S. suggested potential harm to patients who received diaphragm pacing.

The DSMB also reviewed data from an ongoing, open-label study in the U.S., the Humanitarian Device Exemption Post-Approval Study of NeuRx Diaphragm Pacing System for ALS, which found longer survival in paced patients than was observed in the British study. An interim data analysis of the U.S. Post Approval Study did not show the risk to patients found in the European trials. The investigators are continuing to enroll new patients.

Much of these data discrepancies may be attributed to differences in the European and U.S study designs.

Prioritizing Standard of Care

So what does this mean for people with ALS? Lisa Wolfe, M.D., Associate Professor of Medicine and Neurology at the Feinberg School of Medicine at Northwestern University, says that people with ALS need to prioritize and make sure that they receive what we currently consider to be the “standard of care.”

“They should be getting non-invasive ventilation, and they should be getting it as early as possible,” explained Dr. Wolfe. “They should be getting adequate nutrition including a gastrostomy tube, and they should have access to riluzole. Those are the things that we know make the biggest difference, and what we frequently have seen is that those basic tools for which there is no argument about, for which the AAN guidelines are very clear, we still frequently see patients who are not being given access to those standards of care. So, if a family is really interested in doing the best thing, number one: make sure you're getting standard of care.”

The ALS Association Certified Centers and Clinics are a good resource for people with ALS not only to receive access to the current standard of care, but to also access a team of compassionate multidisciplinary specialists who can address a broad range of health and quality of life concerns.

“We think that patients with ALS should look at new and upcoming treatments, but they should do it in a way in which they can participate in formal research so that what happens to them gets recorded and helps us to better understand the science of the disease,” advised Dr. Wolfe. Then, lastly, what we would say is that this is clearly not a therapy for everyone and that patients should not just be looking at ‘should I do diaphragm pacing?’ They should be working with their physician to make sure that this is a therapy that is good, right and appropriate for them because it certainly is not for everyone.”

More Information

Find more information on ALS clinical research:

Learn more about participating in a clinical trial:

View the FDA’s 2011 Summary of Safety and Probable Benefit:

Read the Medpage Today article on DPS in ALS trial:

For more information on diaphragm pacing, please visit:

Respiratory Decisions in ALS is a series of three educational videos that aids in the process of understanding respiratory changes that occur, evaluating treatment options and making important decisions about respiratory care that can be accessed here:

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