One of biggest challenges treating ALS is breathing difficulties which occur due to the gradual weakening and decline of the respiratory muscles. Researchers nevertheless are working hard to develop treatment strategies to keep these respiratory muscles moving to help people with ALS breathe easier.
One potential strategy called phrenic pacing hopes to keep the diaphragm moving by boosting the stamina of the respiratory muscles through the implantation of a device that regularly stimulates the connecting nerves. The FDA-approved device, called the NeuRX diaphragm pacing system (DPS), is available by prescription for patients experiencing frequent trouble breathing (chronic hypoventilation).
But a group of physicians from Assistance Publique – Hôpitaux de Paris led by respiratory specialist Thomas Similowski MD PhD thinks that the NeuRX DPS could benefit a lot more people with ALS. They suspect the device, introduced at the very first signs of breathing difficulties, might jump start the training of these muscles to keep the diaphragm moving even longer - postponing the need for non-invasive ventilation.
Now, the French team is gearing up to put their treatment strategy to the test in people with ALS. The double-blind randomized clinical trial, called RespiStimALS, is to be conducted at the Pitié-Salpêtrière Hospital in Paris. All participants are to be monitored three times monthly for 2 years. Outcomes include: reductions in respiratory decline (forced vital capacity), improvements in sleep quality and overall survival. 74 people with ALS are expected to participate.
The trial is scheduled to begin in June 2012.
To learn more about the NeuRX DPS and how the device might help people with ALS, read DPS Sleep. To read about the ongoing NeuRX DPS trial in the UK, check out UK Gears Up To Put DPS Through Its Paces. To find out about other strategies to help people with ALS breathe easier, read CK-357, helping pALS live strong?
References
Gonzalez-Bermejo, J., et al. (2011) Diaphragm pacing improves sleep in patients with amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis. doi:10.3109/17482968.2011. 597862 Abstract | Full Text (Subscription Required)
Onders, R.P., et al. (2009) Complete worldwide operative experience in laparoscopic diaphragm pacing: results and differences in spinal cord injured patients and amyotrophic lateral sclerosis patients. Surgical Endoscopy, 23(7): 1433-1440. Abstract | Full Text (Subscription Required)
Further Reading
Ducko, C. (2011) Clinical advances in diaphragm pacing. Innovations: Technologies and Techniques in Cardiothotacic and Vascular Surgery 6(5), 289-297. Abstract | Full Text (Subscription Required)
Marion, D.W. (2011) Diaphragm Pacing. UpToDate. Excerpt | Full Text (Subscription Required)
Patient Resources
Early Stage Amyotrophic Lateral Sclerosis Phrenic Stimulation (RespiStimALS). Contact | ALS TDI | Website
Diaphragm Pacing in Motor Neuron Disease (DiPALS) Study. Contact | ALS TDI | Website