Curt and Shonda Schilling became interested in the battle against ALS nine years ago when they met ALS patient Dick Bergeron through the Philadelphia Phillies. As part of its generous support of the work of ALSA's Greater Philadelphia Chapter, the Phillies organization each year introduces new players to ALS patients and family members. Curt and Shonda have been deeply involved ever since and were inspired to name their first child Gehrig after baseball great Lou Gehrig, who died as a result of ALS.
Curt Schilling pitches for ALS
By Mike Falcon, Spotlight Health
With medical adviser Stephen A. Shoop, M.D.
When it comes to helping those with ALS, Curt Schilling really is an All Star.
July 8, 2002 - When Curt Schilling takes the mound as the starting pitcher in Tuesday night’s All-Star Game, he’ll have the American League locked in his sights. But last year’s World Series co-MVP admits having an even bigger victory on his mind — finding a cure for amyotrophic lateral sclerosis (ALS).
“It’s about as devastating a disease as you can imagine,” says Schilling, who leads the league in victories and strikeouts. “It’s relentless, ruthless, and devastates entire families as one member perishes slowly. There is nothing I wouldn’t do to raise awareness and the resources for desperately needed research and care.”
ALS is no stranger to baseball.
It’s also known as Lou Gehrig disease, named after the New York Yankees first baseman whose “iron man” Hall of Fame career was cut short by the degenerative nerve disease. Gehrig played in 2,130 consecutive games before ALS stopped him, a mark eclipsed only by Baltimore Orioles shortstop Cal Ripken Jr.
And Schilling is no stranger to ALS.
He and wife Shonda have been involved in the fight against ALS for more than a decade. Shonda serves on the board of directors of ALS Association’s (ALSA) Greater Philadelphia and Arizona Chapters and advocates for ALS patients with members of Congress.
Although it’s a family effort, it’s not due to a family member. “I first met a man who had ALS when I was pitching for the Phillies,” says Schilling. “I couldn’t believe what he was going through. No cure!? I thought there had to be some way to help.”
That man with ALS played a part in Schilling’s drive to the pennant. Before last season he and Schilling made a pact. “He was having a lot of trouble because he was at the stage in ALS when he was becoming ventilator-dependent. He wrote that if I promised to win the World Series and the Cy Young Award he would hang in there for the entire season. I agreed and the night before the final game he wrote, ‘I kept my end of the bargain and now it’s your turn to keep your end of the promise.’”
During the All Star game Schilling will likely make a direct pitch about ALS to the fans and television audience. Earlier in the season, Schilling, ALSA, and Major League Baseball teamed up for a homerun-hitting pledge drive called Covering All the Bases.
Now Schilling and ALSA are going nationwide with a program called Curt’s Pitch for ALS. Schilling will donate $100for each strikeout he pitches and $1000 for each victory to both the Greater Philadelphia and Arizona ALSA chapters. Last season Schilling’s contributions combined with pledges from fans exceeded $50,000. This year donors can pitch in and dedicate their pledges to any local ALSA chapter.
ALS is a progressive neurodegenerative disease that attacks nerve cells in the brain and the spinal cord called motor neurons. Motor neurons transmit commands from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. As the motor neurons deteriorate the information is distorted. When they die, the brain can no longer initiate and control muscle movement.
“Basically, your body limbs and muscles stop listening to your brain,” observes Schilling. “Your muscles atrophy and you cannot make them work, but all the time you are still fully aware and can feel everything — from the way your child hugs you to your dog’s tongue licking your hand.”
There are two common forms of ALS. Sporadic ALS has no known cause and comprises about 90% of the roughly 30,000 USA cases. The familial inherited form is seen five to 10% of the time. One in five familial cases is due to a known genetic defect on a specific chromosome.
One rare form is generally confined to areas near Guam, and may be associated with environmental toxins and/or radioactive fallout: incidence is 50-150 times greater than for other populations.
Gulf War veterans have also incurred “an incredibly high incidences of ALS,” notes Schilling.
Similar environmental concerns voiced by local residents prompted CDC investigations in the area surrounding Kelly Air Force Base in Texas earlier this year. A study cited in the CDC report showed a two-fold increase in death due to motor neuron disease — a broader classification, of which ALS makes up the majority of cases — among commercial pilots. Exposure to jet fuel has been suspected as a contributing factor.
Symptoms
ALS symptoms often begin as very slight and subtle changes, and are frequently overlooked by both patients and physicians alike, especially since a family doctor is likely to see just one case of ALS during their entire career.
“Early misdiagnosis or no diagnosis is common,” says Schilling.
Symptoms may include one or all of the following:
Muscle weakness and impairment in the hands, arms, or legs.
Difficulty speaking, swallowing, or breathing normally.
Twitching and cramping of muscles, especially in the hands and feet.
Shortness of breath.
Symptoms and their initial severity can vary widely. Functional deterioration often begins in the hands and feet. Tripping over things, difficulty walking, or household tasks like dressing and buttoning clothes is common. The effects then usually move towards the body’s core, affecting speech, swallowing, and breathing.
ALS progression can also vary. Usual survival time is three to five years, although many live up to a decade after symptoms appear. But progressive muscle weakness and paralysis is almost universal.
Oddly, ALS has gone into symptomatic remission or halted in its progression in a small number of cases, although the reasons behind these events are not understood.
All of this combines for a generally dark prognosis. “A lot of doctors who have diagnosed someone with ALS just tell their patient to get their affairs in order,” says Schilling, “because there’s nothing they can do.”
There is hope, however.
The drug Ritulek can slow the progression of the disease and modestly prolong life in some cases. It is the first drug to offer hope that a medicine might lead to an eventual cure.
“I think that we need some scientific breakthrough,” says Dr. Lucie Bruijn, ALSA medical director. She speculates that this might come from research into the more rare form of familial ALS.
By any account, there is much work to be done “and we need dedicated great researchers who can stick with the job,” says Bruijn.
“To fund that research we need awareness to create an educated public that cares,” says Schilling. “It’s not only money we need — we need car dealers to donate vans and people who will volunteer for a few hours to drive ALS patients to the doctor or just help them.”
“What I really want is to strike ALS out,” concludes Schilling. “Permanently.”