Rewriting a new chapter in the struggle with ALS


Author Philip Carlo is trying an experimental device to help him battle Lou Gehrig's disease

Tuesday, September 04, 2007
By ANDREA BOYARSKY
ADVANCE STAFF WRITER


STATEN ISLAND, N.Y. -- Philip Carlo has seen the darker side of life. He grew up in Brooklyn, living among the Mafia. He's gotten up close and personal with serial killers. And he spends his time recreating extraordinarily violent situations on paper.

But it's nothing compared to the killer the 58-year-old author faces now.



In October 2005, Carlo was diagnosed with Amyotrophic Lateral Sclerosis (ALS) popularly known as Lou Gehrig's disease. A progressive disease, it attacks motor neurons, causing the brain to lose the ability to control muscle movement, leading to muscle weakness, paralysis and death. There is no cure and no definite cause.

"I have interviewed some of the most notorious and dreaded killers of modern time and never batted an eye; I was never frightened," said Carlo, author of "The Ice Man: Confessions of a Mafia Contract Killer," (St. Martin's Press) about Mafia hit man Richard Kuklinski.

"I always felt like a scientist looking through a microscope and studying strange microcosms ... and now I'm being stalked by the worst killer of modern times."

About 5,600 people in the United States are diagnosed with ALS each year, according to the Calabasas Hills, Calif.-based ALS Association. Most people diagnosed are between the ages of 40 and 70, with the average age at 55. But people in their 20s and 30s have been affected as well. Only around 10 percent of cases are hereditary.

MYSTERY DISEASE

ALS could be attributed to an excess of the neurotransmitter glutamate, explained Dr. Stephen Scelsa, director of the Neuromuscular Division and ALS Center at Beth Israel Medical Center in Manhattan. Or, possibly an accumulation of ubiquitinated proteins.

"But we really don't know," Dr. Scelsa said. "At some point the motor neurons just start shutting off or start dying. It never gets better," he said. "Usually, it declines at a steady rate."

ALS symptoms can begin in the arm, Dr. Scelsa explained, with grip and dexterity problems. The disease also can present itself in the leg with ankle weakness and increased tripping. Or it may start with the speech and swallowing muscles, causing slowly progressive slurred speech.

Around 50 percent of people with ALS die within three years, while less than 5 percent can live for 20, Dr. Scelsa said. The cause of death usually stems from the weakening of the respiratory muscles, which leads to breathing difficulties.

Prior to his ALS diagnosis, Carlo ran five miles a day. He started showing symptoms of the disease in February 2005, with a slight limp in his left leg that became progressively worse. Carlo initially thought it stemmed from his running.


Today, the author is wheelchair-bound, unable to stand up long enough to brush his teeth. He falls a lot. But he continues to write, since the disease usually does not affect the mind.

"It's like a slow, flat tire," Carlo said in a phone interview from his Manhattan home. "Little by little you turn into a skeletal form of what you once were and there's nothing you or anyone else can do about it."

TREATING ALS

The only FDA-approved treatment for ALS is the drug Rilutek, which may protect nerve cells from too much glutamate. Dr. Scelsa said that in the last 10 years, more research has been conducted and he will be participating in a trial looking at an antibiotic for Lyme Disease that may increase glutamate metabolism.

People with ALS also can go on a ventilator, which helps them breathe and can increase their life span. However, many choose not to, said Dr. Raymond Onders, because it decreases their quality of life.

More than two years ago, Dr. Onders, a Cleveland, Ohio, surgeon, implanted the NeuRx Pacer into his first ALS patient. The device assists with breathing, delays the need for a ventilator and can prolong a patient's life up to 24 months, he said, quick to note that it's not a cure. The device is manufactured by Ohio-based Synapse Biomedical, which Dr. Onders founded.

Carlo received the NeuRx Pacer in April as the first patient in an FDA-approved 100-patient trial being conducted at 10 sites across the country.

During minimally invasive laparoscopic surgery, electrodes are implanted on the diaphragm to stimulate and strengthen the muscles. The electrodes are connected through the skin to an external device programmed for the best diaphragm movement for the patient. The NeuRx Pacer was first used to help spinal cord patients, including the late Christopher Reeve, with breathing problems.

"We're always hopeful [for ALS patients]," said Dr. Onders, director of minimally invasive surgery and associate professor of surgery at University Hospital Case Medical Center in Cleveland. "You never know when anyone can find the cure."

The device is best for people functioning at a 50 to 85 percent breathing capacity, Dr. Onders said. Carlo's breathing is now around 45. So far, around 70 pacers have been implanted for both ALS and spinal cord patients. Those who receive the pacer are asked to use it five times a day for 30 minutes each time.

Carlo said he turns on the pacer whenever he feels fatigued, and keeps it on at night to help him sleep better.

The author currently is putting the final touches on a novel about the head of a Mafia crime family in New York he describes as a combination of "Goodfellas," "Honor Thy Father" and "The Sopranos." The head of the family cooperated with Carlo for the book, which he hopes to have completed by October or November.

"It's like every day, I write and it's hard," Carlo said. "I'm slowing. It's hard to let my mind go because I have this disease."

But, he continues to have hope that a cure will be found. "To be so personally compromised like this, to me, is devastating. But you got to learn to live with it."

Andrea Boyarsky is a features reporter for the Advance. She may be reached at boyarsky@siadvance.com.

http://www.silive.com/living/advance...690.xml&coll=1