New York author Philip Carlo first started showing the symptoms that eventually would be diagnosed as ALS in February 2005.
“I was coming back from doing an interview of Richard the Ice Man at Trenton [N.J.] State Prison,” recalls the 58-year-old Carlo, who has published five books of true and fictional crime and has a sixth one (on the Mafia) under way.
“I was in Penn Station, and my left foot was flopping on the marble floor. I was in excellent physical condition, and I thought I had some problem attributable to running too much.”
For the dedicated cyclist and runner, ALS was “a very hard pill to swallow,” he says, and he soon started searching “outside of the box” for help.
Carlo’s search led him to Raymond Onders, a general surgeon at University Hospitals of Cleveland who’s dedicated to developing an alternative to existing methods of assisted ventilation.
If it contracts, you breathe
|Augie Nieto, whose ALS was diagnosed in 2005, says he can now breathe while lying flat.
Rather than pumping air into the lungs, which is how ventilators work, Onders has pursued a different strategy to assist breathing: stimulating the diaphragm, the flat, pancake-like muscle that sits just under the lungs and moves up and down to allow the lungs to fill and empty. (When you take a breath, the diaphragm contracts and moves down. When you exhale, it relaxes and moves up.) The use of this device in ALS is somewhat controversial.
A few years ago, Onders began surgically implanting electrodes in the diaphragm muscles of patients with spinal cord injuries who couldn’t breathe on their own. When the electrodes are stimulated through wires outside the skin, the diaphragm contracts. The strength and rate of the contractions can be regulated to suit individual needs.
“If you look at recovering arm function, it’s complex,” Onders says. “But with the diaphragm, if it contracts, you breathe.”
He soon began implanting diaphragm stimulators (also called pacers) in people with ALS who were losing respiratory function. “Patients with ALS develop nighttime sleep dysfunction,” he says. “The diaphragm works differently in the daytime and in the nighttime. There are [centers] in the brainstem that control night breathing, and that part of the brainstem, which controls the diaphragm, is affected in ALS.
“BiPAP [bilevel positive airway pressure] will help patients when they don’t breathe at night, but when you stimulate the diaphragm, that’s natural breathing.” People don’t notice anything different from normal breathing, he says.
Carlo got his diaphragm stimulator in Cleveland in March. “Overall, I highly recommend it,” he says. “I find it helps me sleep better and has put more power in my cough. I can actually cough now, where before I had this little kitten cough.”
Decision was a ‘no-brainer’
For Augie Nieto of Corona del Mar., Calif., a leader in the fitness industry, ALS became a reality in March 2005. The 49-year-old entrepreneur and his wife, Lynne, have since become co-chairpersons of MDA’s ALS Division and helped launch MDA’s Augie’s Quest, an ALS research initiative.
In May, Nieto went to see Onders in Cleveland and get a diaphragm stimulator. “Every time I make a decision, I think about what the risk is to my quality of life and measure it with the possible benefit,” Nieto says. The diaphragm stimulator decision, he says, was a “no-brainer.”
“There are so many charlatans out there preying on ALS victims, whether it’s inserting stem cells in China or some other exotic treatment,” Nieto says. “This is for me the best investment I’ve ever made.” (His insurance plan didn’t cover the procedure.)
Nieto says he now can lie flat on his back and breathe without difficulty, something he couldn’t do before getting the stimulator. However, he says he continues to use his BiPAP device during the night because he believes it helps his lungs stay well inflated and not become brittle.
“Augie was one of the first patients to have his diaphragm electromyogram [record of muscle activity] serially followed,” Onders says. “There has been significant improvement in Augie’s diaphragm EMG.”
Study remains open
Onders says he thinks diaphragm stimulation works in at least two ways: It causes the person to breathe at a rate and depth that’s comfortable, and it also appears to condition the diaphragm, keeping it from deteriorating and perhaps actually strengthening it.
Onders believes the device can extend survival and delay ventilator use in ALS. “They may get an extra year or so not on a ventilator,” Onders says. “But they can also use their device along with a volume ventilator or BiPAP.”
For now, if you have ALS and want a diaphragm pacer in the United States, the Food and Drug Administration requires you to be part of Onders’ study. It’s open to people who meet the criteria in Cleveland, Baltimore and Stanford, Calif., with an anticipated additional seven centers to open soon.
As of mid-July, Onders’ team in Cleveland had implanted pacers in 26 people with ALS and scheduled another 25 implants.
Some insurance plans cover the procedure, which has received “category B” approval from the FDA. Medicare considers category B devices, which are deemed essentially safe but still under investigation, “eligible for coverage,” but all the agency’s criteria must be met.
For those without third-party coverage, the cost of the procedure is about $5,000.
For details of Onders’ study, contact MaryJo Elmo at (216) 844-8594 or email@example.com.
A possible biomarker for clinical trials
In addition to its possible therapeutic effects, Onders is enthusiastic about using the diaphragm pacer as a “biomarker” for ALS clinical trials.
With its ability to detect natural diaphragmatic muscle contraction when the electrodes are not stimulated, it could provide a reliable indicator of whether a therapy that’s supposed to stabilize or improve muscle strength is working, he says.