Implant Helps ALS Patients Breathe
Carol Lasaro, a 66-year-old mother of two, plus one son-in-law, and grandmother of four, has a few things in common with Superman: a problem, an operation and an implanted device.
OK, maybe not with the original Superman, the comic book character, but with Christopher Reeve, the late actor who played the "Man of Steel" in four Superman movies. After a severe neck injury, which left him paralyzed, Reeve became the second person in the world to have a new device implanted to help him breathe.
Lasaro was the first to have the same device implanted at the University of Chicago Medicine.
The device, known as the NeuRx Diaphragm Pacing System (DPS), was developed by Synapse Medical to help people who lack the neuromuscular tools to breathe on their own. Reeve needed surgery following an injury he suffered when a bad fall during an equestrian competition damaged his spinal cord. The injury disrupted the connections between his brain, where the signal to breathe originates, and his diaphragm, a large, upper-abdominal muscle that pulls air into and out of the lungs.
Lasaro, of Griffith, Ind., has a similar problem but a very different cause. She was recently diagnosed with amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease), a progressive, incurable and eventually fatal neuromuscular disease. ALS damages motor neurons, which tell muscles to contract, including those that control breathing.
Her nerve troubles began in 2010, when she lost some control over one foot after a lower back operation. This made it difficult to walk. Six months later, "my good foot also started flopping," she said. Over time her legs got weaker, then her arms, and she had increasing trouble just breathing.
By the fall of 2011, she said, she could barely hobble from the bedroom to the living room and back before she had to sit down, rest and catch her breath. She also had trouble sleeping and eating.
In late November, she came to the University of Chicago in search of a better understanding of her health problems. On November 27, her doctor, Kourosh Rezania, MD, associate professor of neurology at the University of Chicago Medicine and a neuromuscular-disease specialist, delivered some bad news. As Lasaro had suspected, she had ALS, for which there is no cure and no effective treatment.
But Rezania identified Lasaro as a patient who could benefit from a diaphragm pacing system. He and the multidisciplinary DPS team -- neurologists, pulmonologists and surgeons who evaluate ALS patients on a case-by-case basis -- recommended a DPS implantation and scheduled the procedure for December 18.
The operation, developed by Raymond Onders, MD, of University Hospitals of Cleveland, "is becoming a potentially important part of ALS care but it is not yet standard of care," said Rezania. "The efficacy of this device should be proven by studies on large numbers of ALS patients," he said. "Further clinical studies are underway to investigate the impact of DPS on the survival and quality of life of ALS patients."
To implant a DPS, surgeons insert a tiny camera and miniature surgical tools through a few half-inch incisions in the abdomen. Through these keyholes, they attach four small electrodes to the diaphragm and implant a fifth electrode just under the skin. All five are connected by wires that are attached to a small external battery-powered pulse generator, about the size of a cell phone. The generator sends electrical signals to the electrodes, which tell the diaphragm to contract.
"This is not a difficult operation for an experienced minimally invasive abdominal surgeon," said Vivek Prachand, MD, associate professor of surgery at the University of Chicago, who implanted Lasaro's DPS. It is often done at the same time as placing a feeding tube, to help the patient get adequate nutrition -- as was done in this case.
After the operation, her doctors adjusted the timing to match her normal breathing pattern, usually around 12 to 15 breaths per minute.
The operation is done on an inpatient basis, but the patients can generally be discharged the next day, Rezania said. Because this was the first such procedure at the medical center, Onders traveled from Cleveland to observe the operation. It went smoothly and the medical center was immediately granted "certified-center" status, one of about 45 such programs in the United States. The team did a second case that afternoon.
Onders initially developed the device to help quadriplegics, such as Reeve, breathe without constant reliance on a ventilator. The Food and Drug Administration (FDA) approved the device for that purpose in 2008. In September 2011, the FDA, under the Humanitarian Device Exemption Program, extended its approval to ALS patients with inadequate breathing. Studies show it can help "extend their lives without a ventilator by 16 months," according to Onders.
"It doesn't change the course of the ALS," Prachand said, "but does improve quality of life."
"It's helping me," Lasaro said, one month after her procedure. "I can inhale a lot better, take deeper breaths. My disease is getting worse. But I'm breathing a lot better. And I'm sleeping much better. It was a good decision."
Lasaro comes back to the medical center every six weeks for follow-up visits. She's looking forward to the spring, when her husband and her son-in-law are planning to install a chair lift in the house and build a ramp over the front stairs, making it easier for her to get in and out of the house.
She also cites a benefit not previously described in DPS studies: "I can sigh now, deeply and whenever I feel like it."