Andrew Buddemeyer was pedaling as hard as he could on astationary bicycle at Washington University’s Center for AdvancedMedicine.
He had a clip holding his nose shut, a tube shoved into hismouth, a blood pressure cuff squeezing his upper right arm and ahalf dozen or so wires running from his chest to echocardiogram andelectrocardiogram machines.
W. Todd Cade, assistant professor of physical therapy atWashington University, urged on Buddemeyer: “Ten more seconds …eight more seconds, Andrew … come on, five more seconds … keepgoing.”
The idea was to push Buddemeyer, 21, of Tampa, Fla., toexhaustion to see whether three months of regular aerobic exercisehad strengthened his heart and increased the efficiency of musclesthroughout his body. Both are severely weakened by a geneticdisorder called Barth syndrome.
Cade received a $40,000 grant from the Barth SyndromeFoundation to do the same test on three other young men withthe condition as part of a pilot study. he hopes to get anothergrant to do a larger study with more participants and more weighttraining.
“And because exercise affects the mitochondria (cellular powerplants), the study can give insight into other conditions such asaging, diabetes and other inherited cardiomyopathies and heartfailure, so potentially a lot of people beyond Barth syndrome willbenefit,” Cade said.
When he reached exhaustion, 5 minutes and 50 seconds into hisride, Buddemeyer quickly got off the bike and lay down on a nearbygurney so a technician could get the echocardiogram andelectrocardiogram readings.
His mother, Leslie Buddemeyer, also of Tampa, was standing inthe doorway to the room watching. she noted how her son had gainedabout five pounds in the past few months and that his arms, legsand even his torso looked bigger, more muscular.
Until recently, patients such as Buddemeyer were told not toexert themselves.
That thinking has begun to change among researchers like Cade,who believe that people with heart conditions like Barth syndromecan benefit from exercise.
Buddemeyer was diagnosed seven years ago with the geneticcondition. it leaves muscles throughout his body unable to processoxygen efficiently and compromises his immune system leaving himvulnerable to bacterial infections. Cardiomyopathy, a conditionthat weakens and inflames the heart, is also usually found in boyswith Barth syndrome.
“Parents are often afraid of exercise, because their childrenare susceptible to an irregular heart beat, so they tend torestrict their activity levels,” Cade said.
Buddemeyer’s test results showed marked improvements in severalareas.
The maximum amount of time and pedal resistance he was able toendure had increased by 17 percent. The muscles in his legs wereable to extract 50 percent more oxygen for energy. and a crudemeasure of his lactic acid production had decreased by 8 percent.That’s good because lactic acid levels drop when oxygen levels risein muscles. his HDL numbers — the good cholesterol — had increasedand so had his quality of life as determined by the Minnesota Living with Heart Failure Questionnaire.
The questionnaire assesses the affects of frequent symptoms,such as shortness of breath, fatigue, peripheral edema anddifficulty sleeping, on daily life.
Buddemeyer was a senior at Florida State University in November2009 when he began feeling unusually weak and short of breath. Hethought he had a bad cold or maybe even pneumonia, so he drovehimself to the hospital.
He had a stroke while there and found out he was in heartfailure. Doctors prescribed medicine to keep his heart musclestrong and implanted an internal defibrillator to keep it fromgoing into arrhythmia. A pink scar marks the spot on his chestwhere it lies beneath his skin.
Buddemeyer was selected to take part in the exercise study whileat a national Barth syndrome convention in July. Cade tested him atthat time to determine his baseline levels then developed athree-month exercise program.
It consisted of cardio training on a stationary bicycle and anelliptical trainer for 45 to 50 minutes, three times a week underthe supervision of medical experts in Tampa. he also did lightweight-lifting.
Before the program, Buddemeyer said he was doing a littleexercise but nothing regular or vigorous.
“When I started I could only do about five straight minutes ofrigorous exercise,” he said. “By the end I could do the full 45 or50 minutes without stopping.”
He knew before the most recent test that it would provebeneficial.
Buddemeyer plans to continue exercising at a cardiac rehabcenter in Tampa, but at a higher intensity.
Shortly after the most recent test, he saw his cardiologist atTampa General Hospital, said Leslie Buddemeyer.
“They were so impressed because they never see such a dramaticimprovement,” she said. “He was being considered for a hearttransplant list. now he’s not.”