Bowler strikes at childhood disease
July 31, 2000
By Mike Falcon
Unlike most professional athletes, marathon bowler Thomas Becker is not driven by fame or fortune. Until he heard about spinal muscular atrophy (SMA) - a devastating neuromuscular disease that is the leading genetically-linked cause of death in infants under two - he had never thought about rolling 16-pound bowling balls for a world-record consecutive 30 hours and 48 minutes.
But until there's a cure for SMA, Becker may bowl forever. "I have to," he says. "It's the only way I know to bring attention to this tragedy."
Up to one in 6,000 babies is born every year with SMA, although estimates vary widely. One in 40-80 people carry the recessive gene that causes it. When two people with the gene decide to have children, there's a 25% chance their baby will develop SMA.
The baffling disease destroys motor neurons that transmit impulses from the brain to otherwise-healthy muscles. Due to lack of stimulation, the muscles atrophy, leaving the victims - most often babies and children - unable to walk, stand, or even sit up. They eventually die, usually from respiratory complications.
Despite the relatively common occurrence of the
gene that causes it, SMA is not well-understood. "It's
Becker has teamed up with AMF Bowling, Inc. to take his marathon work on the road. The AMF/FightSMA.com Tour kicked off in May and concludes in late August. When it's over, Becker will have bowled in all 50 states in 100 days in an attempt to raise $1 million for FightSMA.com and Andrew's Buddies, SMA advocacy groups and research sponsors.
Marathon bowlingBecker's introduction to SMA - and the genesis of his marathon bowling career - occurred in late 1998, when he happened to glance at a TV news show detailing eight-month old Allie Brenner's fight with the disease.
"Her dad was raffling off a Super Bowl ticket to raise money," recalls Becker, "and I had a tough time just watching. His little baby girl was dying. It really bothered me that nobody seemed to know about SMA."
After visiting Allie in the hospital, Becker came up with the notion that "a world record would make people pay attention." But what kind of record can a decorated veteran, father of two, and former bowling alley manager hope to break?
Having been around bowling all his life, Becker had vaguely heard of marathon bowling, but had never thought seriously about it. But after consulting the Guinness Book of World Records, he decided go after the most demanding of the several records in the category. After all, he thought, how tough could it be?
Very tough, it turns out. After one day and 41 minutes of continuous bowling, Becker was wheeled into a hospital, unable to stand, both feet covered in raw blisters, hands bleeding, suffering from dehydration and exhaustion. But he had his world record.
Six weeks later, Allie died.
The little girl's demise touched Becker deeply and prompted him to make himself a promise. He would not stop bowling until SMA could be stopped.
His next new marathon record of 31 hours and 48 minutes - knocking down more than 30,000 pins and bowling 328 strikes during 221 total games - was featured on The Today Show and in USA TODAY. The national coverage began to attract serious sponsors with equally serious commitments, including AMF.
The subsequent tour has turned Becker into the world's only professional marathon bowler. But infinitely more important, it has promoted new awareness of the dreadful disease.
Parents are usually the first to notice SMA
symptoms. Failure to develop age-appropriate motor functions - such as
sitting, standing, or walking unassisted - are key. Poor tongue and
chewing control can also be clues.
SMA is usually classified by when the disease's effects are first noticed - which has strong correlations to both the eventual effects of MSA and predicted survival outcomes.
Type I: Werdnig-Hoffman disease - Onset of symptoms usually occurs anywhere from the last trimester of pregnancy to eight months of age. The most severe form of SMA, it affects chewing, swallowing, respiratory muscles, and the arms and legs. Type I SMA victims can never sit up without help, and respiratory complications almost always end their life before age two.
Type II - Usual symptomatic onset is observed at 6-18 months of age. These babies and infants usually have fewer and less severe symptoms. They can usually sit up by themselves, but will never be able to stand or walk without support. Some children with SMA Type II have a relatively static course and remain free of life-threatening complications such as pneumonia.
Type III: Kugelberg-Welander disease - Symptoms usually appear after 18 months. Many of these children may appear "normal" until they are 5-10 or even older and may remain ambulatory for decades after the onset of symptoms. Other patients exhibit slowly progressive symptoms resulting in loss of independent walking. Respiratory complications are uncommon and swallowing problems are rarely encountered.
Because there is no cure for SMA, treatment concentrates on preventing complications and extending and improving the patient's quality of life. For Type I infants, this may mean feeding tubes and respirators. For children with Type II SMA, ventilator masks and motorized wheelchairs are the rule. For older Type IIIs, braces and physical therapy are usual.
New research developments provide hope,
however. Much of it is based on the work of French researcher Dr. Judith
Melki, who in 1995 "uncovered" the two nearly mirror forms of
the survivor motor neuron gene: SMN-1 and SMN-2, also known as SMN-T and
Actual Internet Story at http://www.usatoday.com/life/health/doctor/lhdoc204.htm