Stony Brook Surgeon Dana Telem, MD, has implanted the first diaphragm pacing system for an ALS (amyotrophic lateral sclerosis) patient on Long Island. Famed chef Gerry Hayden, co-owner of the North Fork Table & Inn of Southold received the implanted device on March 8 at Stony Brook University Hospital.
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Called the NeuRx Diaphragm Pacing System (DPS), the device assists the breathing of individuals who develop chronic hypoventilation from ALS (also known as Lou Gehrig's disease), allowing them to breathe for a longer period without the assistance of mechanical ventilators.
Hayden, a three-time James Beard Foundation Award finalist for best chef in the Northeastern U.S. and the only chef from Long Island ever nominated for the award, said he hopes the device will extend his ability to use his diaphragm to breathe on his own by as much as 16 months. He suffers weakness on the right side of his diaphragm muscle, he said. Ten days after the procedure, he was already able to use the device for three to four hours a day; his goal through conditioning is to be able to use it around the clock. Currently, his breathing is assisted by a Bilateral Positive Airway Pressure (BPAP) device while he sleeps.
"I hope that this will prolong my diaphragm so that it can continue to work on its own," he said. "I was very happy I was a candidate for it. If we can get the word out about it, it can help more ALS patients.
"The breathing part of the disease is the worst," he said. "If it can catch more people early [in their disease process] and help them breathe, it can help patients with their longevity and quality of life."
Prior to his diagnosis, Hayden served as executive chef at North Fork Table & Inn, which he co-owns with his wife, pastry chef Claudia Fleming, and Mike and Mary Mraz. After he was diagnosed with ALS in 2011, his fellow chefs rallied to his support, raising $110,000 through a celebrity chef fundraiser in Manhattan last summer.
This fall, Hayden hopes to organize additional events on Long Island to raise awareness and funding for ALS research. He is working with Jeri Woodhouse of Taste of the North Fork in Cutchogue, to create gift packages of sauces to be sold under the name "Hayden's Heroes" to raise money for the ALS Association of Greater New York, Project ALS and Chris Pendergast's annual Ride for Life.
"I can't wake up every morning wondering whether I'm going to die," Hayden said. "I have to wake up every morning wondering how I'm going to live with it [ALS]. And that's a big thing I think patients need to know."
Hayden is being treated at Stony Brook University's Christopher Pendergast ALS Center of Excellence, the only such center in Suffolk County. "I applaud the surgical team and Dr. Telem for their compassion, commitment and perseverance in making this pioneering procedure a reality," said Pendergast, a 20-year ALS patient for whom Stony Brook's center is named. "Stony Brook Hospital's bold decision illustrates forward thinking and the desire to bring cutting-edge medicine to Long Island's residents. With DPS, they now have a weapon that hopefully will prove useful in improving the status of a selective group of ALS patients. It is literally and figuratively a 'sigh of relief.'"
"I did this for my breathing," Hayden said. "I did this for my speaking, because hopefully that diaphragm will continue [to help] the words come out. And the words I need to tell everybody are, 'Get this thing if you can, as soon as you can.'"
Dr. Telem is among the nation's first surgeons to implant the device since the US Food and Drug Administration (FDA) approved it in September 2011. "This procedure demonstrates that when it comes to cutting-edge surgery, Stony Brook is the leader on Long Island," said Alexander B. Dagum, MD, FRCS(C), FACS, Interim Chair of the Department of Surgery and Clinical Professor of Surgery and Orthopaedic Surgery, Stony Brook University School of Medicine.
ALS is a rapidly progressing, incurable and fatal neuromuscular disease characterized by progressive muscle weakness that results in paralysis. As the nerves to the diaphragm muscles fail, patients lose the ability to breathe without ventilator support. Approximately 30,000 people in the United States live with ALS, and more than 5600 new cases are diagnosed each year.
Candidates for the DPS device have chronic hypoventilation as a result of ALS, and have a diaphragm that can be stimulated, Dr. Telem said. The nerves supplying the diaphragm are examined through fluoroscopy, ultrasound or EMG to assess if a patient is a good candidate for the procedure.
"Traditionally, we were only able to assist these types of patients with ventilators, which can be cumbersome and not well tolerated by many patients," Dr. Telem said. The FDA study of the device showed a 16-month improvement in survival for ALS patients from the time they were diagnosed with chronic hypoventilation, she said.
ALS patients also benefit from improved sleep soon after beginning treatment, said Nurcan Gursoy, MD, PhD, a neurologist with the Christopher Pendergast ALS Center of Excellence. "ALS can cause patients to develop sleep apnea and nocturnal ventilation, which increases carbon dioxide levels and reduces their sleep efficiency," she said.
The device is implanted through minimally invasive laparoscopic surgery, said Dr. Telem, who was assisted on the case by Aurora Pryor, MD, FACS, Chief of the General Surgery Division at SBUH.
After the procedure, the patient gradually conditions the diaphragm muscle through a series of physical therapy sessions. The device provides electrical stimulation to the muscle and nerves in the diaphragm. When the muscle is stimulated, it contracts, helping to condition the muscle to improve fatigue resistance during normal exertion.