Exercising with a Muscle Disease

Spring • April — June 2009 quest.mda.org Ex ercising with a Muscle Disease I don’t exercise. If God had wanted xercise: Love it. Hate it. Gotta do it. On me to bend over, he would have put Ethe following pages, Quest looks at Table of Contents: diamonds on the floor. the many sides of exercise for people with Warning: Read Me First! (What to watch ~ Joan Rivers muscle diseases: out for when excercising)...... 2 • What does the research tell us? Exercise Q&A (the latest research Physical fitness is not only one of • Which types of exercises are OK and and medical advice)...... 3 the most important keys to a healthy which aren’t, and how can you tell the body, it is the basis of dynamic and difference? Glossary of Exercise Terms...... 5 creative intellectual activity. • What are other people doing — or not Recommended Exercises ~ John Fitzgerald Kennedy doing — and what are the results? in Muscle Disease (chart)...... 6 • And of course, the ultimate question: Is Profiles of people who If it weren’t for the fact that the TV it worth it? exercise...... 7, 8, 9, 10, 11, 12 set and the refrigerator are so far On the next page you’ll see a box of apart, some of us wouldn’t get any warnings — please be sure to start there. Effects of Exercise on Different Muscle exercise at all. ~ Joey Adams Much of the conventional wisdom about Diseases (chart)...... 13 exercising isn’t true for people with muscle What Kind of Exercises Lack of activity destroys the good diseases, and doing it wrong may cause Can Be Done By …?...... 15 condition of every human being, irreversible muscle damage. Exercises for Caregivers...... 18 while movement and methodical As you read through the exercise articles physical exercise save it and in the magazine (see the table of contents), How Do You Feel About Exercise?...... 20 preserve it. ~ Plato one thing is clear: Each body is unique, and Exercising Will Over Despair...... 22 its owner knows it best. Please filter all this I really don’t think I need buns of information through your own wisdom and Adapted P.E. for Kids...... 23 steel. I’d be happy with buns of experience, and please share it with your Quest Tech: cinnamon. ~ Ellen DeGeneres physicians, therapists and trainers. “Wearable technology” for exercise...... 24 And if you want to share your exercise As the Wheel Turns: experiences with others, please visit Quest Effect of sports on power chairs...... 27 online (quest.mda.org) and post a message on the Feedback page. Because one thing is Kid Quest: definitely true about exercise: It’s more fun Are power chair sports exercise?...... 30 when it’s shared! From Where I Sit: Am I Disabled or Aren’t I Disabled?...... 33

1 Quest 2009, No. 2 ©2009 MDA Warning: Read Me First! What to watch out for when exercising verybody agrees that exercise is a good thing — but only if undertaken safely. EHere are some warning signs that say STOP. When muscle fibers are undergoing damage, they usually — but not always — announce it pretty quickly, with cramping and pain. But sometimes muscle destruction doesn’t make itself known until enough damaged muscle fibers have released a pig- mented protein called myoglobin into the blood and eventually into the kidneys and urine. Dark, cola-colored urine hours after exercising indicates this kind of muscle destruction.

Signs of muscle damage or impending muscle damage are: • cramping in muscles (probably related to insufficient energy supply for muscles) • pain in muscles • weakness of exercised muscles • dark urine that looks like cola, following exercise (seek medical care immediately if this occurs)

Many muscle diseases affect the heart muscle, impairing its ability to pump blood fast enough to keep up with the demands of strenuous exercise. Others can cause an irregular heartbeat or one that’s too fast or too slow, and strenuous exercise can exacerbate these abnormalities.

Signs of cardiac stress are: • shortness of breath • chest pain • very elevated heart rate (150 to 160 beats per minute) with even moderate exercise • weakness • nausea • sweating • a gurgling sound in the chest with breathing (seek medical care immediately if these symptoms occur)

It can’t be stated enough: Always consult a physician before undertaking a new exercise program.

Q: What is exercise? Why do it? of bone density and mood elevation. A: Exercise is muscle exertion (use), Because exercise requires energy requiring the expenditure of energy. It (calorie burning), it offsets the intake can be very roughly divided into two of dietary calories and may help keep major categories: aerobic and resis- weight in check. tance (sometimes called “strengthening”). Aerobic exercise helps maintain Q: What does or doesn’t the overall fitness, especially cardiac and medical literature tell us about respiratory health. If muscles are exercise and neuromuscular intact and functioning, then resis- disease? tance, or strengthening, exercise A: Unfortunately, there isn’t a lot Q helps increase muscle mass and the known about exercise in neuromus- force muscles can generate. However, cular disease, but some studies have some muscle diseases severely limit been conducted that shed some light the ability of muscles to increase on the subject. mass or force because they limit the “We know so little,” says Ted regenerative capacity of muscle tis- Abresch, director of research at sue. Additional benefits of exercise are the Research & Training Center maintenance of flexibility, preservation for Neuromuscular Disease at the University of California at Davis. (The MDA Neuromuscular Disease Clinic at UC Davis is part of the MDA Clinical Research Network that supports studies in Duchenne muscular dystrophy.) Abresch says some forms of exercise are beneficial in neuromuscular disease, but unfortunately, it’s usually not prescribed unless patients specifically ask. Studies of exercise, he says, have largely been “messy” and hard to interpret, because often there aren’t adequate control groups (groups assigned not to exercise), and because people with different diseases are often studied together. At his center, he says, “We’re looking at what people are actually doing, and we’re looking at wearable technologies to see how much they’re walking or sitting, so we can get a baseline A stationary bike offers excellent aerobic exercise. Elastic band stretching is a good resistance exer- and then see if an exercise prescrip- cise. tion makes a difference.” (A pedom-

3 Quest 2009, No. 2 ©2009 MDA Ted Abresch, who directs the Research & Training Center for Neuromuscular Disease at UC Davis, says studies of exercise in this area have been hard to interpret. Photo: Emi Manning, UC Davis

eter, which measures how many steps are, however, some general precau- a person takes, is an example of a tions to take. wearable technology. For more on Many neuromuscular diseases this subject, see page 24.) affect the heart muscle, and that adds Abresch says the only thing he an extra requirement for caution. can say with certainty is that adults Cardiac abnormalities figure promi- with slowly progressive muscular nently in many muscular dystrophies, dystrophies (such as limb-girdle, particularly Duchenne, Becker, facioscapulohumeral and myotonic myotonic and Emery-Dreifuss MDs, dystrophies) showed improvements as well as in Friedreich’s ataxia. with moderate exercise. “The one pro- If someone has a cardiomyopathy gram we did that seemed to work well (heart-muscle degeneration), the was a walking program, where we put pumping action of the heart is pedometers on people and asked them impaired, and the muscle won’t to walk 25 percent more over time respond to exercise the way a than they did initially. They reduced healthy heart would. It may thicken weight, said they felt better and did (hypertrophy) in an attempt to meet better on quality-of-life testing.” the increased workload of exercise, resulting in diminished blood-holding Q: Can exercise hurt me? capacity. Or it may dilate, causing the A: If you have a neuromuscular dis- heart to become floppy and relatively ease (and even if you don’t), some ineffective as a pump. types of exercise can hurt you. It’s When cardiomyopathy or other always a good idea to consult with types of heart disease render the your physician before starting an heart’s pumping action insufficient exercise program and, ideally, to in supplying the body’s cells with the work with a physical therapist who understands your disorder. There ... continued page 7

active exercise: exercise that a person doesn’t extend completely and suddenly does using his or her own strength (see elliptical trainer: stationary exercise device passive exercise) that simulates walking or running with- aerobic exercise: exercise that involves or out causing the joints to be subjected to improves oxygen utilization by requir- much force ing the heart and lungs to work harder. exercise: muscle exertion (use) involving Aerobic exercises involve low to mod- expenditure of energy erately intense activities performed for fitness: the ability of the circulatory and extended periods of time. Examples respiratory systems to supply nutrients to are walking, running, swimming and skeletal muscles during sustained energy cycling. expenditure (exercise) and the ability of anaerobic exercise: exercise that does not muscles to respond involve or improve oxygen utilization; forced vital capacity (FVC): the amount of weight lifting is an example air one is able to exhale after inhaling as cardiomyopathy: degeneration of the heart completely as possible muscle, such as occurs in some muscle interval training: repetitions of exercise inter- diseases rupted by periods of rest or low activity conditioning: training to perform better isometric muscle contraction: a muscle con- for a long period of time; usually done traction in which the muscle fibers remain through aerobic exercise, such as walk- approximately the same length even ing, swimming, etc. though force is being exerted; an example coordination: the ability to integrate muscle is pressing the hands against a wall with- movements to perform specific functions, out extending the arms such as walking, running or manipulation maximal: full-out; exercising maximally of small objects; eye-hand coordination is means exercising as hard and as fast as the ability to integrate what one sees with one possibly can subsequent muscle action myoglobinuria: the presence in the urine of concentric muscle contraction: a muscle myoglobin, which is released by damaged contraction in which the muscle fibers muscle fibers; myoglobin turns the urine shorten as they contract; flexing the brownish or cola-colored and can damage arm at the elbow is an example the kidneys; its presence indicates severe, contraction: what muscles do when they’re acute muscle damage active; refers to protein filaments actin oxygen consumption: the amount of oxygen and myosin sliding over each other taken in during exercise or at rest; can contracture: permanent shortening of a be determined directly by measuring muscle or tendon, resulting in a per- oxygen intake and carbon dioxide exhala- manent “freezing” of a joint in a certain tion through a metabolic mask or can be position; occurs when muscle weakness approximated indirectly by measuring or spasticity prevents normal range of heart rate motion over a long period of time passive exercise: exercise that a person does eccentric muscle contraction: a muscle con- without any exertion; for instance, having traction in which the muscle fibers are someone else move one’s limbs in range- pulled apart (lengthened) even though of-motion exercises (technically speak- they’re being activated to contract; for ing, not really exercise, although it gives example, eccentric contractions occur some of the benefits of exercise such as when straightening the elbow gradually improving circulation, movement, comfort while holding a weight, so that the arm and flexibility)

5 Quest 2009, No. 2 ©2009 MDA physiatrist: a physician who specializes rhabdomyolysis: the rapid breakdown of in maximizing functional abilities and skeletal muscle tissue quality of life for people with physical submaximal: exercise that’s performed at impairments; the specialty is called less than maximum effort; sometimes physical medicine and rehabilitation defined as exercise that is not too stren- physical therapist: health care professional uous to prevent talking, or exercise in who helps individuals develop, maintain which the heart rate does not go above and/or restore maximum movement 65-70 percent of maximum (maximum and functional ability = 220 minus age) range-of-motion exercise: exercise that tachycardia: abnormally rapid heart rate involves putting a joint through its nor- trainer: a fitness professional who devel- mal range of motion (as far as it can go ops and implements an individualized in any direction); can be done actively approach to physical fitness, generally or passively working one-on-one with a client; train- resistance exercise: a form of exercise in ers generally do not have any medical which each effort is performed against background a specific opposing force generated treadmill: piece of indoor sporting equip- by resistance (for example, resistance ment used to allow for the motions of to being lifted, pushed, squeezed, running or walking while staying in one stretched or bent); sometimes used place interchangeably with strength training

Recommended Exercises in Muscle Disease Exercise purpose Examples Notes

improve endurance walking, running, • recommended for most people with neuromuscular diseases and conditioning swimming, cycling who choose to adopt an exercise regimen, with intensity and duration depending on ability and cardiac function • walking or running downhill not recommended • not to be done to exhaustion

build strength weight lifting, working against • lifting light weights and working against light resistance and muscle mass resistance with equipment such are usually OK for most people with neuromuscular disease, as stretchable bands unless in acute phase of an inflammatory muscle disease; stop at pain and before exhaustion

increase agility, wheelchair sports, some • OK for most people with neuromuscular disease coordination and balance video games, balance ball, horseback riding

maintain or active or passive range-of-motion • OK for most people with neuromuscular disease increase flexibility exercises; gentle stretching

n September 2008, Mary Gallo, 38, learned Iabout a local pulmonary rehabilitation gym called Oxygym. She contacted her pulmonolo- gist, who wrote a prescription for Oxygym’s restrictive lung program. “Oxygym is the first place I’ve ever attend- ed that focuses on both breathing and physical therapy. I found a workout program tailored to my medical needs,” says the Lindenhurst, N.Y., resident, who has congenital muscular dystrophy (CMD) with severe kyphoscoliosis and restrictive lung disease. Gallo attends Oxygym for 90 minutes, three times a week. Each visit begins with a check-in, where her blood pressure and blood oxygen levels are checked. Gallo works her arms by using a hand cycle, resistance band, and 3-pound weights. She then spends 15 minutes on the treadmill, after which her blood pressure and blood oxygenation levels are checked again. Range-of-motion and light resistance exercise can be done in the water. After the treadmill, she usually has a nutri- tional energy drink. blood supply they need, the person is days a week. Those in the usual-care In addition to the leg press and leg curl said to be in “heart failure.” group continued their usual medi- machines, she does step-ups on a low The traditional view of exercise cal therapy and were encouraged to aerobic-training step, while holding on to a for people with heart failure has been be active, but they were not given a bar. She hopes to advance to a stair-climbing that it should be undertaken with specific exercise program. machine. great caution, if at all. But at a meet- It’s imperative for anyone with a The last stop is the breathing room, where ing of the American Heart Association disease that affects the heart to con- she does group exercises to strengthen the in November, 2008 investigators sult a cardiologist before beginning muscles of the chest, back, neck and stomach. from Duke University and Thomas an exercise program. Chances are, if In just two months at Oxygym, she’s Jefferson University reported results your skeletal muscles are up to the doubled the volume in her lungs, notices more of a long-term, large-scale study of task, some degree of exercise can be muscle mass in her arms and finds walkways heart failure patients assigned to an undertaken. and ramps easier to tackle. exercise or a usual-care group. The Then there’s the critical issue of “Since I’ve been attending Oxygym, I feel study found that exercise was safe for whether or not exercise can weaken physically and mentally better, and I’m happier study participants with heart failure and damage muscles already made when I’m working out.” and lowered their risk of hospitaliza- fragile by a dystrophy. This is a par- tion or death. ticular concern in the dystrophies in The study included 2,331 patients which the muscle-fiber membrane is from 82 study sites who were fol- compromised. These are Duchenne, lowed for an average of two-and- Becker and the sarcoglycan- a-half years. Those in the exercise deficient forms of limb-girdle MDs. group started with three 30-minute In these diseases, a type of muscle sessions per week on a stationary contraction called eccentric can bicycle or treadmill. After 18 sessions, seriously damage already weakened they transitioned to 40 minutes five muscle-fiber membranes and ulti- Mary Gallo

leven-year-old Jerry Huang is a happy mately destroy muscle tissue. with medication. When the disease is Ecamper these days, and his new exercise An eccentric contraction is what no longer active, recreational aerobic program is the reason. happens when a muscle is used in a exercise, such as biking and swim- Two years ago Jerry, who has congenital braking action. When extending your ming, and progressive resistance muscular dystrophy, had tendon surgery to arm slowly while holding a weight, exercise, can be helpful in maintaining relieve severe contractures in his legs. He your muscles act as a brake to pre- strength and cardiovascular health. also began a physical training regimen that vent your arm from flopping down Recently, studies have shown that, wasn’t much fun. “They had him doing things suddenly. Or when walking down in some muscle diseases where the like throwing and catching balls,” said his a steep hill, your leg muscles use fiber membrane is disrupted (such as mother Tina. “It was very tiring for him, and eccentric contractions to keep you Duchenne and type 2D limb-girdle after 10 minutes he was ready to quit.” from falling forward. This type of muscular dystrophies), signals that Enter a new physical therapist at Jerry’s muscle activity is best avoided. normally tell blood vessels to open grade school in Los Angeles. She started him In inflammatory muscle dis- (dilate) during exercise don’t occur. on a schedule of swimming exercises and eases like polymyositis and derma- The signals aren’t sent because a yoga, and got him both a stander and walker. tomyositis, active exercise during molecule called nitric oxide synthase, He now exercises and stretches twice a week an acute flareup of the disease can which starts the process, can’t anchor in PE class for an hour at a time. exacerbate the inflammation and itself to the membrane. The implica- His mom says the change in her son has make things worse. Range-of-motion tions of this problem aren’t yet clear. been incredible. “When he’s in the water, he (ROM) exercise with someone However, researchers believe this can stand, he can walk, he can jump — all assisting or doing all the work (pas- mechanism may explain some of the things he couldn’t do before. It has been so sive ROM) is fine and may help significant fatigue some patients feel good for him.” keep joints flexible. Exercise should with even mild exercise. Jerry also doesn’t have to wear his full- progress in duration and intensity In diseases of the motor neurons, length braces during the day anymore, just as the disease comes under control such as spinal muscular atrophy at night. “Now I can get out of my wheelchair (SMA) and amyotrophic lateral and stand, and I can walk about 30 feet. And sclerosis (ALS), there’s a theoreti- my mom doesn’t have to come with me to cal concern that too much exercise the bathroom anymore,” he says. can hasten the loss of these muscle- The change has been as much emotional controlling nerve cells. These cells, as physical. His mom says when Jerry was the theory goes, not only are com- younger, he was quiet and withdrawn. Now, promised by the disease, but they with his newfound physical independence, may be overburdened by trying to and with mentoring from an aide at school, compensate for neighboring cells that he’s become more outgoing and social, and have been lost. he’s an honor student. Studies in mice with an ALS-like disease have shown that mice that ran regularly on a treadmill at a low speed survived longer than average, but mice that ran regularly on a treadmill at a high speed survived a shorter time than average. The researchers found the mice did best when exercise periods were interspersed with rest. However, they acknowledge that they Weight machines can be adjusted to light resis- Jerry Huang tance. don’t know whether these findings

nce a week, working mother Beth Bax Oof Altadena, Calif., works out at 24 Hour Fitness, where she’s been a member since the If a patient pushes beyond the early 1990s. limits of fatigue or pain, acute An environmental engineer, Bax, 39, received muscle breakdown can occur, spill- a diagnosis of Friedreich’s ataxia in 1999. ing muscle proteins like myoglobin Every other week she works with a personal into the bloodstream and potentially trainer at the gym. “I originally signed up for damaging the kidneys. sessions because I hadn’t exercised for over two “Trainers like to say ‘no gain years, and I needed someone to show me what without pain,’ says Alfred Slonim, a exercises to do and make me accountable for pediatric endocrinologist at Columbia showing up,” says Bax, whose neurologist sup- University, “but in metabolic muscle ports her exercise routine. disease it’s ‘no gain with pain.’” She notes that for some people, a physical On the other hand, Slonim says, therapist might be a better alternative than a “When you turn a person into a personal trainer. couch potato or a wheelchair- Water generally makes exercising easier and “They’re better accustomed to people with reduces stress on the joints. dependent patient, their muscle sta- disabilities and knowing their limits,” she says. tus deteriorates from disuse.” “My trainer sometimes tries exercises with me apply to humans with ALS or other He recommends light aerobic that don’t work out.” motor neuron diseases. conditioning exercise, such as walk- Bax’s visits, which usually last 90 minutes, To add to the uncertainty, stud- ing on a treadmill, or using a sta- start with a 15-minute warm-up on the gym’s ies in mice with an SMA-like disease tionary bicycle, under the watchful stationary recumbent bike. Then it’s over to found regular running resulted in eye of a physician or knowledgeable arm, leg, and back exercises with the chest, leg better motor function, a longer life physical therapist. Light resistance and calf press machines and the lat pulldown. span, and more surviving motor neu- training, using weights of no more Depending on the machine, the weight is usually rons compared to mice that didn’t than 5 or 10 pounds, is OK for some, 40-50 pounds, though heavier for leg lifts. And participate in the training. But again, he says, after they’ve done some then there’s the 100 sit-ups. extrapolation to humans is risky. aerobic exercise. It doesn’t stop there. Bax rides her TerraTrike Perhaps the most dramatic recumbent tricycle around her neighborhood for instances of damage resulting from an hour twice a week. She even takes it along certain types of exercise are those on family vacations so she can ride with her that can occur in the metabolic mus- husband, Eric, and their two children, Natalie, 7, cle diseases, such as Pompe disease and Sarah, 4. (acid maltase deficiency), McArdle “I recommend a recumbent trike if you can disease (myophosphorylase deficien- sit in a seat and be stable,” she says. “It’s a great cy), carnitine palmityl transferase feeling to be going downhill super fast, and it’s (CPT) deficiency, and others in this nice to do something on my own that I don’t category, including diseases involving need help with. the mitochondria, where much of a “Exercise is proven to get you in a better cell’s energy is produced. mood, and I don’t know anyone who can’t use In these disorders, a biochemical that in life.” defect is almost invariably associated At the UC Davis center, respiratory and other with exercise intolerance, and normal functions are monitored during treadmill and exercise is impossible because of other forms of exercise. Photo: Emi Manning, UC Davis extreme fatigue, muscle pain and/or cramps.

Beth Bax

ost athletes devote themselves to one Q: Does proper exercise reduce good for people with neuromuscular Msport. But Ryan Levinson of San Diego pain? disease, it’s probably moving in the isn’t most athletes. A: “Proper” exercise sometimes can water. The buoyancy of water makes Levinson, who has facioscapulohumeral reduce certain kinds of pain. For exercise easier and reduces stress muscular dystrophy (FSHD) and has weakness in instance, if pain is due to stiffness on the joints. However, actual swim- his chest and leg muscles, excels personally and in the joints or prolonged sitting or ming, especially for those with car- professionally at cycling, surfing, sailing, paddle- lying in one position, gentle stretch- diac abnormalities, can be risky, and boarding, kiteboarding, scuba diving, kayaking ing or range-of-motion exercises can should be approved by a physician. and the triathlon. relieve it. However, severe muscle Also, cold water can make myoto- He builds up strength and skill in the pool, cramps or pain, either immediately nia (inability to relax muscles) much ocean, bay, beach, gym, and on the road. Aside or a day or two later, are an indica- worse, especially in the condition from practicing his sports regularly, Levinson tion that the exercise may be doing known as paramyotonia congenital focuses on stretching all muscle groups, core some damage, and it should be (PMC). “If swimming, particularly strength and balance training, functional strength either stopped or reduced in duration in cold water, PMC patients are at training with a combination of exercise physio or intensity. increased risk of an attack [of myo- balls, dumbbells, ropes hooked to cable weights, tonia], and in the water, this can be aerobics and swimming. Q: Is there one kind of exercise life-threatening,” says MDA grantee Levinson no longer runs because weakness that’s good for anyone with a Louis Ptacek, a neurologist and in the muscles that give his knees and hips sta- neuromuscular disease? muscle physiologist at the University bility puts him at risk for injury, but his exercise A: If there’s any kind of exercise that of California San Francisco. “No one program is designed to optimize his strength and comes close to being universally should swim alone, but particularly aerobic fitness for his target events. not PMC patients.” Working with a registered dietician, he found that a healthy diet has had a positive impact on Q: Will exercise cause me to his health and athletic performance. lose weight? His diet mainly consists of lean protein, veg- A: Sometimes — or it may at least etables, whole grains and raw nuts. He avoids offset some of the weight gain that processed foods and concentrated simple sug- usually accompanies a sedentary ars, completely eliminating trans fat. Water is his lifestyle. Aerobic exercise is what’s beverage of choice during the day, and before usually recommended for weight loss going to bed and after waking up, he drinks a or maintenance, since it directly burns glass of whey protein, which is a mixture of calories. However, recent research whey concentrate, whey isolate and amino acids. has suggested that resistance exer- Levinson says he has found that good food cise, which builds muscle (at least in helps his body compensate for some exercise some people), also can be beneficial. stress. “Without good food, your athletic per- Muscle is where most fuel is metabo- formance can actually decrease because your lized for energy, so if there is more body’s ability to recover will be impacted.” muscle, theoretically, there is more fuel burned instead of stored as fat. A program at UC Davis in which people with slowly progressive muscular dystrophies were given pedometers and asked to increase their

Not all exercises require fancy equipment. walking time by 25 percent resulted in

Ryan Levinson

or six years, teenager Dani Anderson of FNewbury Park, Calif., trained regularly in exercise is to see whether you can the martial arts discipline of tae kwon do. talk while doing it. If you can’t talk, Her dedication eventually earned her a cov- says Ted Abresch of the UC Davis eted black belt rating. Center, you’re doing something too An integral part of reaching that level of fast or for too long. accomplishment was exercise, day in and Submaximal aerobic exercises day out. include pool therapy, walking or Anderson, now 22, is even more a stand- bicycling on a flat surface. out in her moves because she has Charcot- Marie-Tooth disease and uses a wheelchair Q: How hard should a person for mobility. with neuromuscular disease “My tae kwon do school created a expect to work at resistance special curriculum for me,” she explains. exercise? “We came upon ways for me to do kicks, A: As above, it depends on disease punches, blocks, etc., from my wheelchair, type and severity and the individual equivalent to those the rest of the class was patient’s condition. In general, one doing. When the rest of the class was doing When using free weights, be careful not to exceed should consider only light resistance 5-10 pounds. push-ups, I would repetitively lift a weighted exercise. Lifting more than 5 or 10 stick.” weight loss, “feeling better,” and scor- pounds or resisting more than 5 Anderson is no longer a full-time martial ing better on quality-of-life measures. to 10 pounds (for instance, using artist because she’s attending school and a stretchable band or a moveable working full time, but she still exercises Q: How hard should a person device you push against) is likely regularly. with neuromuscular disease to cause muscle-fiber destruction. “I exercise at home by doing movements expect to work at aerobic In most neuromuscular diseases, similar to those I did in tae kwon do. My exercise? muscle fibers are difficult or impos- physical therapist supports the exercise A: This, of course, depends on the sible to regenerate. program completely, but she also encour- disease in question and its severity, the Abresch says studies at UC ages me to swim regularly,” she says. “Now age and condition of the person, and Davis found high-resistance exer- I swim at the gym. I do laps as well as walk their cardiac and respiratory status. cise wasn’t any more beneficial in the pool. Along with tae kwon do move- But as a general rule, physicians than moderate-resistance exercise ments, I expect these exercises to maintain recommend that patients with neu- in patients with slowly progressive the muscle mass I already have, and build romuscular diseases do submaximal muscular dystrophies. strength.” aerobic exercise, which means activity that maintains the heart rate at about Q: How do rest and recovery fit 65 percent of its maximum. into an exercise strategy? The maximum heart rate can be A: At UC Davis, they use a “one- calculated by subtracting one’s age rep max” strategy, says director from 220. Multiply that number by Ted Abresch. “That means they lift 0.65 to get 65 percent. So, for exam- a weight, then we give them 15 ple, the submaximal heart rate for a minutes of rest, and then they get a person who is 30 years old is: weight that’s a little heavier.” Patients 220 - 30 = 190; 190 x 0.65 = approxi- only go on to the next weight if the mately 123 beats per minute. previous weight was easy to lift. Another way of assessing aerobic They’re told to stop when the weight

Dani Anderson

ick Johnson, Waltham, Mass., has been is no longer easily lifted. Na big proponent of working out since he Resting at intervals so that heart was a teenager, including throughout the 26 rate doesn’t go above 65 percent of years since his diagnosis of Friedreich’s ataxia maximum is a general recommenda- (FA). tion for aerobic exercise. Now 45, Johnson, a mechanical engineer, There’s an exception to most still is working out, but has cut back on the rules, however. In periodic paraly- types and frequency of his routines. “There’s sis, it’s during a rest following exer- no question that FA has taken its toll on my cise that patients are most likely to muscles,” he says. “But I still find value in experience an attack of paralysis. exercising.” In hyperkalemic periodic paraly- These days he mainly uses machines like sis, attacks can come on within Life Fitness that utilize resistance training. seconds or minutes or even up to an Johnson focuses on eight types of resis- hour after ceasing vigorous exercise, tance training that exercise different muscle says neurologist Louis Ptacek. For groups in his arms and legs. He goes for instance, sitting in a chair after doing eight repetitions per type. Exercising once or Passive range-of-motion exercise means another housework can make someone too person manipulates the patient’s joints. twice a week, he relies on an attendant for weak to get up from the chair 30 help when at his health club, and his wife’s minutes later. Q: In the absence of clear assistance when exercising at home. In hypokalemic periodic paraly- answers, what should a person In addition to resistance training, Johnson sis, the attacks are more likely to who wants to exercise do? spends 30 minutes on a handcycle, and occa- come on the morning following the A: In most cases, people can do mod- sionally tosses and catches a medicine ball. exercise. erate-intensity aerobic training. Some “Exercise helps me in two ways,” he says. People can sometimes ward off can do moderate-intensity resistance “First, I feel it slows the rate of progression an attack of paralysis by continuing training in muscles that can still work of my disease. Second, it helps me with my their activity and slowing down very against gravity (for instance, muscles mind-to-body connection. It helps me main- gradually or by “walking off” the that can extend the leg from a seated tain that, and it can help reduce the physical weakness early in an attack. position with the knee bent). pain in my body.” People always should check with Both his physician and physical therapist Q: What about people who can’t their neuromuscular disease special- approve of his exercise regime, as long as he exercise in the usual sense? ist and, if they have a disease that does it in moderation and with assistance. Can a daily power chair roll involves the heart, with a cardiologist, “My advice to anyone with a disability con- through the mall or the before beginning an exercise program. templating exercise is ‘just do it!’ Be respon- adrenaline rush of video People also need to know how to sible, but try to keep pushing your comfort games contribute to fitness? recognize the warning signs that tell zone. Try not to get frustrated if your body A: It’s not clear that this type of exer- them they’ve gone too far. See “What isn’t doing everything you wish it would.” cise can improve fitness. However, it to watch out for,” page 2. q may help restore energy and reduce fatigue, as well as reduce depression and, if done in a group, offset social isolation.

Nick Johnson

Muscular Dystrophies These are degenerative muscle dis- • Muscle fibers can be damaged by strenuous exercise, and the dam- Duchenne, Becker, congenital, eases, meaning the normal ability of aged sustained may be permanent. distal, Emery-Dreifuss, muscle to regenerate and repair dam- • In DMD, BMD and the sarcoglycan-deficient LGMDs, exercise involv- facioscapulohumeral, age is limited, resulting in a net loss of ing eccentric (lengthening) contractions is particularly damaging. limb-girdle, myotonic, muscle tissue over time. • If the heart’s pumping ability or rhythm is affected by the disease, oculopharyngeal Some (BMD, DMD and some LGMD sudden, strenuous exercise could trigger an acute heart problem, forms) involve fragile membranes around respiratory problem or even death. A cardiologist should be consulted skeletal and/or cardiac muscle fibers. before undertaking an exercise program. Many muscular dystrophies (DMD, Warning signs of an acute cardiac problem are chest pain, shortness BMD, some LGMD, EDMD, myotonic of breath, nausea, sweating without strenuous exercise, a feeling of MD) involve cardiac muscle deteriora- fullness or pressure in the chest, or a gurgling sound durinig breath- tion or abnormal heart rhythms. ing (a sign that fluid may be backing up into the lungs).

Motor Neuron Diseases Nerve cells in the central nervous • Theoretically, remaining nerve cells can become overburdened if amyotrophic lateral sclerosis, system (spinal cord and brain) that too much is required of them with exercise. spinal muscular atrophy control muscle movement are lost, (all types), spinal-bulbar leaving muscles “orphaned” and muscular atrophy undernourished.

Inflammatory Myopathies Muscle fibers are attacked by the • Exercise should vary with disease activity. During severe disease dermatomyositis, immune system, causing inflamma- activity, when strength is very poor, assisted or passive range-of- polymyositis tion and tissue destruction. motion exercise can be undertaken. During periods of mild to moderate disease activity, when muscles can work against gravity, light aerobic exercise, isometric strengthening, and active range-of-motion exercise are recommended. When the disease is inactive (in remis- sion), recreational aerobic and progressive resistance exercise are OK.

Diseases of the Communication between nerve fibers • Exercise can be undertaken when the disease is under good con- Neuromuscular Junction and muscle fibers is compromised, trol. In periods of acute disease exacerbation, or when the disease myasthenia gravis in most cases by a mistaken attack is not well controlled, exercise may increase weakness. on the neuromuscular junction by the • Exhaustion, lasting joint or muscle pain, or shortness of breath immune system. means the exercise is too strenuous. • If on prednisone, mild exercise can help offset side effects, such as weight gain, bone loss, high blood pressure, high blood sugar, depression and anxiety.

Peripheral Nerve Diseases In CMT, and DSS, nerve fibers and/ • Exercise is good for the heart and lungs but will not overcome Charcot-Marie-Tooth disease, or the insulation around them are muscle wasting, which is the result of damage to nerve fibers and Dejerine-Sottas disease, abnormal, compromising communi- loss of input from the nervous system. cation to muscle fibers. • Low-impact exercise, such as swimming or biking, or yoga, is better than high-impact exercise

Friedreich’s ataxia In FA, an abnormal cellular distribu- • Moderate exercise may help slow the loss of motor skills in FA and tion of iron leads to diminished cel- help offset weight gain. lular energy production, with damage • Cardiac precautions apply. No exercise should be undertaken with- to the heart and nerves out consulting a cardiologist.

13 Quest 2009, No. 2 ©2009 MDA Metabolic Muscle Diseases Processing of fuel (carbohydrates or • Muscle fibers may break down in an attempt to meet the energy acid maltase deficiency, fats) in muscle fibers is inadequate, requirements of exercise. carnitine deficiency, carnitine leading to subnormal energy produc- • Do not engage in aerobic exercise that causes the heart rate to palmityl transferase deficiency, tion and/or a toxic buildup of fuel. exceed 65 percent of maximum (220 minus age, multiplied by debrancher enzyme deficiency, 0.65). lactate dehydrogenase deficiency, • Light resistance exercise, such as lifting no more than 5 to 10 mitochondrial myopathy, myo- pounds, can help build muscle mass. adenylate deaminase deficiency, phosphofructokinase deficiency, • Warning signs to stop are pain, muscle tightness or cramps, phosphoglycerate kinase defi- exhaustion or cola-colored urine. Seek medical help if urine turns ciency, phosphoglycerate mutase dark. deficiency, • If the heart is involved, cardiac precautions apply. phosphorylase deficiency

Ion Channel Diseases Channels in muscle fibers that allow • People with periodic paralysis benefit from good fitness. However, paramyotonia congenita, the passage of sodium or calcium vigorous exercise followed by rest can precipitate a bout of weakness periodic paralysis are abnormal. or paralysis. (In hyperkalemic PP, attacks generally occur within an (hypokalemic & hyperkalemic) hour of exercising. In hypokalemic PP, they’re more likely to occur the morning after exercise.) • Low-intensity exercise is best. Some people find they can ward off weakness by cooling down slowly. • In paramyotonia congenita, muscle cooling can increase myotonia (inability to relax muscles) and transition into weakness and paralysis. • If swimming, particularly in cold water, paramyotonia congenita patients are at increased risk of an attack and of drowning.

Centronuclear Myopathies Muscle fibers show centrally placed Because these conditions are not progressive and don’t have spe- myotubular myopathy, nuclei, in contrast to their normal cific defects in the muscle fiber membrane that lead to its fragility, autosomal centronuclear position, which is around the periph- exercise is likely safe if the myopathy is not too severe. Check wih myopathies ery (edge) of each fiber. Mutations in your doctor before beginning an exercise regimen. There are no genes for the muscle proteins myo- data on exercise for children with severe centronuclear myopathies, tubularin, dynamin 2, MYF6, amphi- such as the X-linked, myotubularin-related type. physin 2, and probably others result in weakness, the precise cause of which remains uncertain. Outcomes range from severe weakness affecting all muscles, including those involved in breathing, to weakness so mild it leads only to poor athletic performance.

… a middle-aged, overweight per- … an elementary-school child with son with myasthenia gravis who is Duchenne muscular dystrophy who able to walk? uses a wheelchair occasionally? The child with DMD who is beginning to Unfortunately, even when MG is under need a wheelchair for distances, such as control, as it usually is these days thanks at the mall, is beginning to experience to effective medications, lack of endur- the effects of deteriorating muscle ance when exercising and excess weight fibers. The fibers are degenerating gain as a side effect of medication and because they lack dystrophin, which inactivity remain problematic. helps keep them intact, and so they’re People should not exercise to exhaus- excessively fragile. tion but may undertake submaximal Doctors disagree about exercise aerobic exercise, such as walking, in DMD, but most don’t feel a child swimming or using a stationary should become a couch potato. Doing bicycle. It’s also OK to try some no exercise at all leads to weight gain, mild resistance exercise, using depression and stiff joints, and may light weights (no more than 5 even contribute to muscle atrophy. to 10 pounds) or stretchable Children with DMD should bands. Several short periods of avoid exercise that involves exercise are better than one “eccentric” contractions. This type long one. of exercise can severely damage muscle fibers that may not be able to regenerate. Eccentric muscle contractions occur when muscles are used as brakes, such as when walking down a steep hill or slowly lowering a weight. Weight lifting is an example of an activity that requires a lot of eccentric contractions. After being cleared by a cardiologist, a child with DMD may undertake submaximal aerobic exercise, such as walking or swimming, while keeping an eye out for warning signs of overexertion. (See “What to watch out for,” page 2.) Children with DMD always can do passive or active range-of- motion exercise, and physical therapists often recommend stretching of the heel cords to prevent stiffening of the ankle joints.

15 Quest 2009, No. 2 ©2009 MDA … a child with type 2 spinal mus- … a 25-year-old with Charcot- cular atrophy who uses a power Marie-Tooth who can walk with the chair full time? use of ankle-foot orthoses? The child with SMA is in a somewhat dif- CMT is a disease of the peripheral nerves, ferent situation from a child with DMD, which are made up of bundles of nerve even though they both may use wheel- fibers that connect the spinal cord with chairs. the muscles. It causes loss of motor In SMA, which is primarily a disease function, particularly in the forearms and of the muscle-controlling nerve cells hands and in the lower legs and feet, and (motor neurons) in the spinal cord, there often some loss of sensation, especially isn’t as much concern about direct dam- in those areas. age to muscles as there is in DMD. If the Exercise may not change strength, child is comfortable doing a particular dexterity or sensation in the affected exercise, the exercise is probably all right. areas, because the primary problem is There’s a theoretical concern that faulty maintenance of the nerve fibers exercising too hard or too long with SMA themselves or the coating that sur- might cause stressed motor neurons to rounds them. This type of defect does wear out faster than they might oth- not respond to exercise. Exercise may erwise, but that hasn’t been proven. help if some component of the weakness In general, submaximal aerobic is due to disuse. exercise, light resistance exercise However, as with any condition in and range-of-motion exercise are which mobility is impaired, it’s important fine. to keep putting the affected joints through their range of motion. And it’s important for cardiovascular health, psychological health and prevention of obesity to keep moving in general. Anything someone with CMT can do without causing pain or increased weakness is probably fine, although checking with a physician about any specific situation is always advisable.

©2009 MDA 16 Quest 2009, No. 2 … a 10-year-old child with … a 20-year-old male college dermatomyositis whose disease student with McArdle disease? has been well controlled by McArdle disease, which is caused by the medication for several months, lack of an enzyme that normally breaks but who still tires easily with any down glycogen (animal starch) to generate exertion? energy in muscle cells, poses some unique Children with DM (and their parents) face challenges with respect to exercise. a complicated set of problems. In this In McArdle disease, unlike most other disease, the person’s immune system neuromuscular disorders, what one can mistakenly sets up a severe inflam- do and what one should do are very dif- matory reaction that affects the blood ferent things. Moderate exercise, such as vessels, muscle fibers, joints, skin and brisk walking, may be achievable but may sometimes other organs. Chronic fever is cause severe muscle injury even if the pain not uncommon, and some patients feel or cramping it causes are tolerable. Weight lethargic much of the time. lifting or picking up a heavy object of any The goal should be to preserve as kind can cause more injury than the initial much normal growth and development as pain might indicate. possible (which requires exercise), stave In this condition, even mild cramping or off excess weight gain from immobility burning or aching pain in the muscles can and drug side effects, help joints stay flex- indicate muscle breakdown. ible and not exacerbate the inflammatory It’s tempting for a young man who may process (which over-exercising can do). look fit and strong to engage in strenuous During severe disease activity, when workouts or sports, but if that young man muscles are too weak to work against has McArdle disease, he should never push gravity, a child with DM should do past pain. very little exercise other than pas- After exercising moderately for about 10 sive or assisted range of motion. minutes, the typical McArdle patient is able When the disease has been to tolerate an aerobic activity much more brought under control to the extent easily. This “second wind” is believed to that muscles can work against gravity, occur because fuel for energy production children can engage in active range of in the form of glucose (sugar) has arrived motion; light aerobic exercise, at the muscles through the bloodstream. (such as walking or swim- The “second wind” phenomenon has ming); and mild strengthen- led to the idea of boosting energy by ing exercise, such as using a ingesting sugar right before exercising, stretchable therapeutic band. such as a soda or a candy bar. Research confirms that this approach does help with exercise tolerance and injury prevention. However, it also can cause serious weight gain, so its use is limited. Exercise generally should be limited to submaximal aerobic workouts, as long as they’re comfortable, and perhaps light resistance exercise, lifting no more than 5 to 10 pounds and stopping when pain begins. q

17 Quest 2009, No. 2 ©2009 MDA And the beauty is, “whatever you can do will help,” says physical therapist Jean Hill, [formerly] with the Kessenich Family MDA/ALS Center at the University of Miami. “A little here and there adds up.”

Bad habits

Most adults have bad body habits, like poor posture or only moving in a few routine patterns. The result of these habits is that muscles only stretch within a limited range of motion that works OK for everyday life, but doesn’t stand up to stress. “Injuries occur when you put your body into positions it’s not used to,” explains Lee Burkett, emeritus professor in the Department of Exercise and Wellness at Arizona State University in Tempe. “When your muscles are supple, you can put your body into more positions than you could before.” Beyond flexibility, leg and hip strength is vitally important for lifting, and strong abdominal and buttocks muscles stabilize the lower back region.

Opportune moments

There are dozens of opportunities during Caregiver Exercises the day to fit in quick, gentle exercise. Simple ways to resist injury You can perform the exercises listed below basically anytime you have a spare by Christina Medvescek 30 seconds. If you pair an activity with an exercise — for example, always doing t just takes one little wrong move, like Hoyer lift or another person) significantly partial squats while waiting for your toast Ileaning over to tie a loved one’s shoelace, reduce the risk of injury and always to pop or hamstring stretches during TV for something to pop in the lower back and should be used. But unless you’re also commercials — you’ll improve without put you out of commission. For a caregiv- taking care of your body, an injury may even realizing it. er, this kind of injury can be disastrous. be just one wrong move away. If you already have painful back, neck “Loads of people have had to give up Muscles need flexibility and strength or shoulder muscles, check with your caregiving due to injury,” says Brenda to resist injury — and stretching and doctor before undertaking any of the Shaeffer, [formerly] a physical therapist strengthening exercises are the way to exercises below. at the MDA/ALS Center at Johns Hopkins achieve those goals. University in Baltimore. “Then they have We’re not talking about an hour-long Stretch like a cat to find someone else to provide care, or trip to the gym, but exercises that fit in some cases find a nursing home.” neatly into odd moments of the day, like Stretch to the edge of comfort but not Good body mechanics (like lifting with when you’re watching TV or sitting at a beyond, or you may trigger a stretch the legs, not the back) and assists (like a traffic light. reflex that actually contracts the muscle

and defeats your efforts. Pain isn’t the Get strong Shoulders and arms: Take a weight point, nor is distance. Luxuriate in a The rule of thumb for strengthening with- like a shoe or soup can and lift it straight stretch, the way a cat does. out weights is to tighten your muscles to up in front and to the side, from your Hold stretches about 15 to 30 sec- their maximum, hold for a count of 10, waist all the way over your head. onds, repeating two or three times. Never relax for a count of 10 and do 10 repeti- Hold arms straight out to the sides bounce to get a greater stretch. tions. Be sure you’re stable, either by at shoulder height, then bring one arm Lower back stretch: Tight hamstring taking a wide stance or by holding on to across in front of your chest, keeping the muscles can cause back pain, says John a secure object. elbow straight. Go just to the point where Klune, physical therapist at the MDA/ALS Legs and hips: Whenever possible, your arm starts to bend, then back. Center at the University of Arizona Health climb stairs instead of using an elevator. Force your shoulder blades together Sciences Center in Tucson. In addition, try partial squats. Hold on to in the back, then relax, then push them Stretch your hamstrings by sitting in a stable object like a countertop. Keep together again. Do shoulder shrugs that a chair with your feet up on a footrest or your back straight and slowly lower as if bring your shoulders up toward your coffee table. Keeping your knees straight, you’re about to sit down. Go down as far ears, as if you’re saying, “I don’t know.” lean forward until you feel an easy, not as comfortable, then back up. Neck: Place your palm in the middle painful, stretch. For greater effect, use only one leg at of your forehead and gently press, resist- Shoulder stretch: Scratch your back. time. You also can work on leg strength ing the push by holding your head still. First point your elbow in the air and each time you get up from a desk or Repeat on each side and the back of your scratch the top of your spine. Next, curl table, by rising from the chair using only head. each arm around the front of your neck one leg. Hold on to the table for stability These are just a few of the many exer- and scratch over your opposite shoul- but don’t pull yourself up. Alternate legs cises that will help keep your muscles der. Finally, hold your elbow down by for an equal workout. supple and strong. For more information, your side and twist your hand back up Lower back: Here’s an exercise Betsy Curless, [former] physical thera- between your shoulder blades. nobody will even know you’re doing. pist at the MDA clinic at the Via Christi Chest stretch: Place your hands at Whenever you think of it, squeeze your Medical Center in Wichita, Kan., recom- shoulder height on either side of a door- buttocks or your abdominal muscles as mends these books: Treat Your Own jamb, then step into the doorway so your tight as you can and hold. “You can’t Back and Treat Your Own Neck, both by arms slightly wing out behind you. Feel overdo it,” says Klune. Robin McKenzie (Orthopedic Physical the stretch through the middle of your A San Diego State University study Therapy Products). q chest. of abdominal exercises found that they Neck stretch: Lower your ear to your should be done frequently rather than for This article originally ran in the MDA/ALS shoulder on either side. intense periods. Five minutes a day can Newsletter, Oct. 2003. Updates have been make a difference if done regularly. added for experts who were quoted.

“There’s no adrenalin rush” Feel“It’s a huge pick-me-up” About Exercise? “I’d rather be doing other things” “I must exercise regularly to keep my strength”

A s muscle weakness progresses, people give different answers

by Bethany Broadwell n this age of obsession about body image, romuscular disease are dealing with similar Iwhen someone asks me if I want a piece challenges regarding loss. of strawberry pie for dessert, I’m apt to People’s degree of mobility and their decline. “No thanks. I need to maintain my past experiences significantly impact how girlish figure.” they feel about exercise. Some choose not My playful reply is designed to conceal to work out. Others remain motivated and the fact that I’m actually assessing my abil- find ways to modify their routines to accom- ity, as a 40-pound skinny mini with spinal modate their weakness. muscular atrophy type 2 (SMA2), to eat the The following perspectives about pie. exercise from people with neuromuscular Do I have the strength to cut the berries disease may help you decide the extent of and the crust? Will I have the oomph it takes workout activity that fits your lifestyle. to raise my fork to my mouth? Will I have the musculature to chew and swallow the Time to stop dessert? Simply feeding myself and eating has become an exercise workout for me. Sue Charlip, 48, of It is an awkward predicament to be in St. Petersburg, Fla., when everyone around me seems to be received a diagnosis consumed with tight abs, low-carb diets and of myasthenia gravis intense activities like spinning or step aero- (MG) in January 1997. bics. I’ve never really had the strength or She describes how stamina for any significant exercise. Instead, the progression of the I try to preserve as much of my strength as disease has gradu- Sue Charlip possible, call on assistance when necessary ally decreased her and realize that plenty of others with neu- motivation for exercise, because continual

©2009 MDA 20 Quest 2009, No. 2 exertion causes her extreme physical weak- tunities help her hold onto the thrill of mov- stronger.” ness and fatigue. ing, and make it a little easier to listen when Tyler Roope, 11, of Burbank, Calif., “I was a big walker. I used to walk in others share their excitement and interest received a diagnosis of SMA2 at 18 months the neighborhood, walk the mall,” Charlip about fitness. old. Although he doesn’t always like to take recalls. “I just walked and never gave it Even so, she admits, “There is a tinge time for stretching exercises, physical edu- much thought until MG hit and walking of jealousy, longing and sense of loss when cation is his favorite class in school. became difficult.” I hear about people doing things that are Says Tyler, “I like baseball best. I want to She says she enjoyed the exercise beyond my physical abilities.” manage the Cincinnati Reds someday. When because it was easy. It didn’t take any spe- we play, my friends hit for me and I run the cial talent or equipment. Plus, walking didn’t Keeping going bases in my chair. I like to play kickball at aggravate her asthma. school in P.E. I pick someone to kick for me Repetitive motion exhausts her now, Greg Halamicek, 59, of Lancaster, Calif., and I run the bases. When I am in the field, so Charlip uses a motorized scooter when knows he needs to keep exercising to if the ball hits me, it’s an out.” she needs to cover long distances or avoid maintain his weight and body function, as standing for long periods of time. his type 1 Charcot-Marie-Tooth disease pro- Of mind and muscles “I just try to accept my limitations,” she gresses. He has modified his workouts and concludes. “I was sick for two years, at made swimming the primary focus of his For some who are very competitive or who least, before I got diagnosed; so in one way, routine, using an indoor pool near his home deeply feel the psychological insult of neuro- I’m glad to know what is wrong with me and three or four times a week. muscular disease, losing the ability to exer- know how to take care of myself. It’s just The limited extent to which he is able to cise can be hard to bear, says Carl Tishler, a frustrating, depressing and sad that I had to exercise, Halamicek says, gives him a “huge clinical psychologist and professor at Ohio give up the little bit of physical activity I was pick-me-up” both emotionally and physically. State University. able to do and enjoyed.” Joey Wells, But, he says, there are other ways of Tina Baughman, 49, of Pacific Palisades, 21, of Shadow “staying in shape” when moving the body is Calif., says living Hills, Calif., no longer feasible. with adult-onset became moti- Exercising the mind can “strengthen the limb-girdle mus- vated to exercise psyche and thus help the individual feel at cular dystrophy two years ago ease,” explains Tishler. As possibilities, he (LGMD) has after a hospital suggests learning a new language, solv- completely taken stay depleted his ing crossword puzzles, playing computer away her desire strength. Wells games, following the stock market, reading Joey Wells Tina Baughman to exercise. The has myotubular or volunteering. reasons are myopathy and uses a power wheelchair. For those who have some degree of multiple: “I can’t stand up off the floor. I His workout routine includes arm cycling, remaining mobility, Tishler urges: Make the don’t see any positive results. I would prefer stretching, stepping, kicking and weight most of the capability you do have. It might to spend my time doing other things.” training. not help your muscles, but it might help In her younger years, Baughman found it Wells explains, “When I was younger, I your mind. rewarding and fun pumping weights, devel- had more strength to do what I needed. So, “Any exercise — no matter how brief or oping her muscle strength, playing sports I could get away without exercising. Now with little stress (weight) — will improve the and working against her own personal best. I need to exercise regularly to keep what psyche of the individual with MD.” q Her experience is different now. “There’s no strength I have.” adrenalin rush. It’s like asking a competitive Nikki Young, 34, of Clarksville, Md., finds Bethany Broadwell is a freelance writer downhill snow skier to be happy spending fitness and healthy living empowering as she based in Traverse City, Mich. Her strength is time walking through the snow … for me, struggles to deal with facioscapulohumeral wrestling with words. that’s not going to happen.” muscular dystrophy. Through exercise, diet With assistance, Baughman has suc- and physical therapy, she’s lost more than cessfully managed such adventure activities 56 pounds and regained some indepen- as tandem skydiving, High Sierra backpack- dence. She advises, “I know that even with ing on horseback, indoor rock climbing and a neuromuscular disease, we can have posi- flying a single-engine aircraft. These oppor- tive influences on our bodies and keep them

Exercising Will Over Despair

by Michael P. Murphy

s a kid, I couldn’t play — much less had ter. I was only a kid, but I knew doing these shoulders started telling me, “Hey, you don’t Aany interest in — sports, so I wrongly Pumps saved my life, once a month. have the stamina of a 20 year-old anymore. assumed that exercise just wasn’t neces- This was my first inkling that some form Let’s get some stretches going here!” sary. I was a brainiac and a science fiction of physical activity was important to good Two: I was diagnosed with type 1 dia- nerd, so I figured that exercise was just for health. I’ve continued doing Pumps all my betes. I had suffered an ungodly summer: the jocks, right? The only physical exercise life, even though my stomach illnesses have three months of weight loss (40 pounds), I ever did on a regular basis were what I long since subsided. I have my home health constant hunger, constant thirst, constant called Pumps. assistants pump each leg for five minutes peeing, impotence and an out-of-control To this day, we’ve never figured out why (about 300 reps each) every morning before temper. Then, once a blood test revealed I had so many stomach ailments during my I go to work. Pumps help with circulation a blood sugar level of 540, I faced another childhood. Once a month, almost like clock- and also keep some limberness in my con- few months of too much insulin, resulting in work, I would be down for two or three days tracted limbs. These stretches eventually dangerously low blood sugars. with nausea, vomiting, chills and sensitivity expanded to range-of-motion exercises with The diagnosis of diabetes knocked me to light. Even in the midst of all this misery, my arms and have now become such a nor- on my butt, in every sense. I felt so lousy I had a strange need for movement. I feared, mal part of my daily routine that if I should that all I wanted to do was stay in bed where I sensed, I was convinced that if I just lay miss a day or two, I feel as if I’ve aged a I was most comfortable, where I could work, still, my body would shut down. My mom, hundred years. read, listen to music, watch movies and surf God bless her, would hold my ankles and In 2006, two things happened that fur- the Internet. The idea of just staying put was pump my legs back and forth, 24 hours a ther proved the need to keep physically fit. seductive, but I never let it stop my Pumps. day, sometimes in her sleep, until I felt bet- One: I turned 40, and my back, neck, and Once my metabolism settled down and I

©2009 MDA 22 Quest 2009, No. 2 could feel some strength returning, I forced but it has certainly helped with my morale. forces that attempt to slow us down. q myself to get into my wheelchair and stay The body and brain must work together to up a couple hours longer than I wanted to. keep the machine in good working order. Michael P. Murphy, 42, has spinal mus- Every day, I made myself keep up with my Whichever side is lagging, the other side cular atrophy and lives with his family in e-mail correspondence, my novel research, pushes to get up and go. Oconomowoc, Wis. He has written two the screenplay I was writing — building the Having a disability means that many science fiction novels, To Rule in Hell and time a little bit each afternoon — because of us can never truly test the limits of our Data Streets, and a thriller, Innocence Kills: I feared turning into a decadent blob, like endurance. We don’t have a whole lot of A Paul Murdock Mystery, available at autho- a skinny Jabba the Hutt. It wasn’t easy. endurance to test! At that low point in my rhouse.com, Amazon.com and bookstores. Sometimes, all that kept me from staying in life two years ago, I was tempted to forget He’s hard at work on his latest novel, Suka: bed was the fact that our new high-definition about the rest of the world, to be a specta- A Paul Murdock Mystery. TV was two rooms away in the living room. tor rather than a participant. By dragging But it worked! I got back to moving, exercis- my exhausted body and mind out of bed, I ing my will over my despair. achieved a major victory over both middle I knew then, and I know now, that I need age and this new disease. to keep moving, mentally and physically. Sometimes, just to keep moving IS the I’m fortunate that my current low-carb diet race. We might never score the winning means that I only have to take one unit of touchdown, pass military training, or break insulin a day, but my daily exercise regi- the tape at the end of a marathon, but we men helps with the quick metabolizing of have to savor every victory we can grab. that insulin. I don’t know if pushing myself And we win. Big time. We win through our has been all that useful for me physically, determination to triumph over all those

Getting Creative with Adapted P.E. acob Engers, a ninth-grader from to fulfill his P.E. requirement. The school rest of the class, such as when they’re JBaltimore, switched from a 504 plan to agreed and an adaptive program was learning the rules of various games or an IEP once he qualified to receive adapt- designed by the head of the adapted P.E. completing written assignments. When ed physical education (P.E.) services. program for Baltimore County Schools. he can’t participate due to physical limita- In most areas, schools require all stu- The Wii is a video game console that tions, he goes to an office near the gym dents to complete a P.E. requirement. For interacts with a wireless remote controller where he uses the Wii for the 90-minute this reason, adapted P.E., which can be that users strap to their arm. The remote period. provided by a physical education teacher is activated by physical movements or by Jacob plays different or special education teacher, isn’t consid- pressing buttons. The console typically sports on the Wii, includ- ered a “related service,” and occupational connects to a television set. ing tennis and baseball. and physical therapy can’t be used as To play a Wii sports game, the user Oftentimes, another substitutes. straps on the remote and watches the TV student comes in to Jacob, 14, has Duchenne muscular screen for the virtual serve or pitch. The play against him and dystrophy and uses a power wheelchair user then mimics the action of swinging a keep him challenged. full time. His mom, Cathy Engers, says racket or a bat as if hitting the virtual ball. While using the Wii, Jacob did very well with a 504 Plan The console interprets the movement of Jacob is hooked up throughout elementary and middle the remote and the TV screen shows if the to a heart monitor, school. The only difference between the virtual ball has been hit, and, if so, where and his heart rate 504 Plan and his new IEP is the adaptive it went. The Wii can be used by people of gets elevated, says P.E. program, she says. all skill levels; as they improve, the skill his mom, show- Because the state allows some flexibil- level automatically increases. ing “he’s getting ity with the adapted P.E. curriculum, Cathy Jacob still attends a general P.E. class. something out of proposed that Jacob use the Nintendo Wii He participates in some activities with the it.” q

Tracking Physical Activity and Energy Expenditure Muscle-disease researchers say ‘wearable technologies’ may help in the quest to gain strength, lose weight

by Alyssa Quintero hen people exercise, they typically to determine the effect of exercise or other Wwant to see results on the scale and therapies on people with muscle diseases. Note: People with muscle diseases in how they feel. Now there’s another place always should consult their physicians to check for results. What researchers and MDA clinic team, particularly physi- “Wearable technologies,” ranging from want to know cal and occupational therapists, before basic step-counting pedometers to high- beginning any exercise or weight loss tech “BodyBuggs,” can give users con- At the NIDRR Rehabilitation Research program. crete and immediate feedback about their & Training Center in Neuromuscular physical activity — and in turn may help Diseases at the University of California at Low-tech devices such as step-counting pedom- them achieve their exercise and weight- Davis, researchers are evaluating the role eters can help people with slowly progressive loss goals. that wearable technologies play in moni- muscle diseases track their steps and gradually increase physical activity levels. Some of these devices currently are toring and improving the health of people being used by neuromuscular researchers with muscle diseases.

©2009 MDA 24 Quest 2009, No. 2 you can watch and do every day, so that’s improve strength, participants should walk one that we think would be very effec- more, which will be precisely recorded by tive,” Abresch notes. “I would think that SAM, explains Abresch. for most people who can walk, it would He adds, “We like the step monitor and be helpful. But with our population, you the pedometer because they’re something always should ask your physician if it’s OK that people understand. As long as people or not.” are ambulatory, using them makes sense.” In addition to simple pedometers, UC Davis researchers have conducted stud- That’s an IDEEA ies using the StepWatch Activity Monitor (SAM), a small pager-like device that’s Accelerometers, which record how much strapped onto the ankle and measures and energy (measured in calories) is used records minute-by-minute step activity (or during physical activity, also are useful gait cycles). This device is not available to devices, says Abresch. the general public. The MiniSun company, located in SAM is programmed via computer to Fresno, Calif., has designed a computer- Some researchers are evaluating the daily activities of people with muscle diseases by having them wear define the length of time for data collection like device called the Intelligent Device for portable devices. and to make adjustments for different gait Energy Expenditure and Activity (IDEEA), styles. It can be connected to a computer which analyzes body motion, measures Because they aren’t as active, people to upload data for further analysis, includ- physical activity, monitors behavior pat- with neuromuscular diseases are more ing frequency, duration and intensity of terns and estimates energy expenditure on susceptible than the general population physical activity. a 24-hour basis. to “metabolic syndrome,” says R. Ted In a 2005 study, researchers investi- Researcher Ming Sun (president of Abresch, director of research at the facility. gated activity and heart rate patterns in MiniSun) has worked with the UC Davis Metabolic syndrome is a weight-related ambulatory boys with Duchenne muscular team to investigate new wearable tech- condition strongly linked to the develop- dystrophy (DMD) for three days as they nologies, and developed IDEEA as a way ment of heart disease and type 2 diabetes. went about their normal daily routine. When to determine energy consumption during Abresch says wearable monitoring compared to boys without DMD, the study all daily activities, not just prescribed devices provide researchers with insight participants had significantly more inactive intervals of exercise. into the kinds of physical activity being minutes. When they were active, they took Now used extensively in research labs, done by people with muscle diseases, significantly fewer steps and spent fewer IDEEA has five motion sensors, or accel- when the activity is done, the duration of minutes at moderate and high step rates. erometers, that can identify more than each activity and energy expenditure. “The goal is to use the SAM to 40 types of physical activity and provide For example, UC Davis researchers describe the type and level of activities detailed information on the duration, fre- gave a pedometer to 15 people with slowly that a person is doing in a normal day,” quency and intensity of that activity. progressive muscular dystrophies (such Abresch explains. “You can tell how much The information can be uploaded to a as limb-girdle and facioscapulohumeral time a person spends sitting, how much computer and the MiniSun Web site will muscular dystrophies). Participants time a person is walking, how much time give a detailed analysis of physical activity tracked their steps for a week and then they’re running and the length of time and energy expenditure components in were instructed to increase their steps they’re walking continuously.” any given period of time. by 25 percent. Study participants were Abresch hopes further research will The device takes into account a per- encouraged to look at the pedometer every determine if the SAM can be used both son’s height, weight, age, gender and day for six months and aim to increase to modify behavior in people with muscle physical ability level when estimating their step counts. diseases and to gauge the effect of differ- energy consumption. Overall, the increased exercise had ent therapies on activity levels. “The good thing is that it’s very accu- beneficial effects on quality-of-life mea- For example, SAM is being used to rate and does a good job of measuring sures, Abresch says. The participants felt measure outcomes in both the PTC124 energy balance,” Abresch says. “It’s a very better about themselves, and some experi- clinical trial for boys with DMD and interesting device.” enced slight changes in weight. Insmed’s Iplex trial in adults with myotonic Sun’s research team recently complet- “It’s a simple, easy intervention that muscular dystrophy. If the treatments ed the new wireless IDEEA3 system that

25 Quest 2009, No. 2 ©2009 MDA menu planning system. When someone is in a wheelchair or has limited activity, “it can be easy to overeat,” says Vaughan. BodyBugg users report that the device makes them more aware of food calorie values, allowing them to better monitor calories and keep weight under control. The armband with digital display costs $348.95, which includes online food logging with customized meal plans, a six-month subscription to the Web-based burned and consumed 24 hours a day. It program and a phone session with a records the level, intensity and duration of BodyBugg coach. Consumers must fill out all physical activities throughout the day. an extensive health history and food pro- Jolyn Vaughan, a BodyBugg coach file questionnaire before using the device. in Phoenix, Ariz., notes, “It’s addicting. After the initial subscription expires, People like to see how many calories they users must renew in order to main- burn. It’s immediate feedback, and that’s tain access to the data collected by the what keeps people going.” BodyBugg and online program. Renewals The wireless BodyBugg armband range from $14.95 per month to $99 per contains four physiological sensors that year. (For more information, call (800) monitors and records accumulate data about motion and physi- 656-2739, or visit www.bodybugg.com.) cardiac functions, including heart rate cal exertion, body heat, core temperature And remember: Before using a and electrocardiogram; electromyogra- and amount of sweat on the skin. The user BodyBugg, pedometer or any wearable phy (measuring the electrical activity of then uploads the information to an online technology, always check with your physi- muscles); leg, trunk and head motions; program (PC and Mac compatible). cian. q and ground-impact force. Tailored to the user’s age, gender, “Combined with these variables, IDEEA height, weight and physical ability, the becomes ideal for simultaneous measure- BodyBugg program calculates, minute by ments of cardiac stress, energy expendi- minute, activity levels and calories burned. ture, function and activity/exercise levels,” “You don’t necessarily have to go to Sun says. a gym to do it,” Vaughan says. “People get their physical activity in so many Burning calories ways, like cleaning their house, or running with the ‘Bugg’ around and playing with their children, and they’re surprised at how many minutes Although IDEEA’s extremely high cost of physical activity they get just by doing makes it appropriate mainly for research, their normal routine.” there is an accelerometer option that’s (Unfortunately, the device isn’t water- available for consumers — the BodyBugg. proof and can’t be used during pool exer- Manufactured by Apex Fitness, located cises, a preferred activity for many people in Westlake Village, Calif., the BodyBugg is with muscle disease.) a small portable device that attaches to a Because the key to weight loss is burn- user’s arm via an armband. Used for calo- ing more calories than are taken in each rie management, it’s been featured on the day, the Web-based program tracks calo- NBC TV weight-loss show “The Biggest ries burned, calories consumed (based on Loser.” the user’s daily food logs) and the differ- The BodyBugg program helps users ence between the two figures. The online track and adjust the number of calories program also offers food options and a

Wear and Tear on the Chair C are and feeding of the sports machine by Bill Norman

ower wheelchair users are a Pgrowing presence in the world of sports. Players (and parents) need to be aware of the unique stresses that power chair sports exert on the chair’s framework and machinery. Common power chair sports include: Power soccer. Two four-player teams square off against each other, typically on a basketball court. Players use their chairs (equipped with an add-on plastic or metal foot guard) to The sport of extreme racing has wheelchair pilots powering at high speed through the great outdoors. kick an oversized ball toward the goal. They also use their chairs to intersect All of these sports are high-speed time to time, but sacrifice everyday or deflect an opponent’s kick. and chairs get a real workout. In reliability, as there’s long-term harm Power hockey. Similar to power particular, wheelchair motors, control- to constantly running power chair soccer except players slap a puck lers and batteries can be subjected to components at very high amperage (usually a plastic 2”- or 3”-ball) with stresses their manufacturers may not and excessive heat,” says Smith. “I’ve lightweight hockey sticks. Sticks may have intended. The metal structure of seen serious European players with be used by hand or taped to the wheel- the chair also can be bent, twisted and custom-modified, unlimited electronics chair and the chair’s power provides broken. go through a controller and/or a set of the “swing” energy. motors every few games by eliminat- Dog agility competition. Dog Heat is Enemy No. 1 ing thermal protection -- it makes for a owners race alongside as their spe- very dangerous, expensive sport.” cially trained dogs navigate an outdoor Mark Smith, creator and operator of Adam Elix, program coordinator obstacle course, striving for fastest the Web site wheelchairjunkie.com, for Far West Wheelchair Sports in time and accuracy. says the main issue in power soccer San Jose, Calif., (www.fwws.org) a Rural racing. Also sometimes is “thermal foldback,” where the con- nonprofit organization that promotes called extreme racing, this sport entails stant acceleration demands and turning and organizes wheelchair sports, says competitors – usually equipped with resistance run very high amperage power chairs can be modified to a cer- protective clothing – striving for fastest through the controller, overheating the tain extent for sports. time when negotiating multi-mile race chair. “We have a mechanic who can courses that can include dirt, mud, “Power chairs have been custom adjust the gear ranges — gear one for water and other obstacles. modified for individual users from street cruising, gear four for athletes.

27 Quest 2009, No. 2 ©2009 MDA We’ve done a couple of things to deal 6-pole motors rather than 4-pole. in three group sizes: 22, 24 and 27, with heat problems. One parent [of Motors have magnetic poles, just like with the larger numbers signifying a soccer player] is a tech guy who simple magnets, but their poles are larger external dimensions. Group 22 hooked up a personal fan to blow on creating by winding magnetic wire in is standard for wheelchairs; group 24 the battery compartment. We’ve also bundles. Motors with fewer poles spin is utilized to power heavier chairs; 27 used ice packs on top of the batteries, faster (1,800 revolutions per minute, is best for frequent chair use (as in and we’ve tried blowing [canned] com- in the case of four poles) and have sports) and traveling long distances pressed air on them. It comes out of to work harder. Six-pole motors spin over rough terrain. Larger batteries the can very cold.” slower (1,200 rpms), but they have also often have the greatest power, more torque (the twisting force that reflected in their ampere (“amp”) hour C omponent factors produces rotation) and can propel a rating. Before attempting to move chair at greater speeds. The 6-pole ver- up in battery group size, chair users Power chairs intended for rigorous sion also runs quieter. must ensure there’s adequate space sports use can benefit from utilizing John Cross, president of ASI in the battery compartment and that specific types of equipment: Technologies, (www.asidrives.com) their other electrical gear, such as the Belt drives rather than direct drives. which designs and manufactures drive chair’s controller, is compatible with a A belt-drive chair, in conjunction with systems for wheelchairs, says another boost in power. wheelchair motors that have a brake, advantage of 6-pole motors is that “a Mike Serhan, product manager can be abruptly switched into reverse lower mechanical gear ratio could be and vice president of sales for Drive gear from full-speed forward, says Dick used with the higher pole motor.” Medical Design and Engineering, Roberts, customer service representa- However, apart from specialized (www.drivemedical.com) said gel cell tive for 21st Century Scientific, (www. applications like sports, Cross said, batteries cost more and usually don’t wheelchairs.com) an Idaho firm that “The added complexity and cost of have the range of wet cell batteries, manufactures the Bounder brand of 6-pole vs. 4-pole is usually not justified but they do provide more torque. Some power wheelchair. He notes, “If you try in a wheelchair motor.” wet cell batteries can leak or spill acid; that with a direct drive, you’ll have parts Bigger batteries are better. gel cells are sealed (as are some wet flying everywhere.” Wheelchair batteries usually come cells), and that can be important in

Power soccer wheelchairs often have structural and mechanical modifications.

inches) offer a smoother ride and more S hopping considerations flotation (less tendency for the tires to mire down on wet or muddy terrain). Abledata, which provides information Serhan also suggested that flat-free about assistive technology products to wheelchair tires may be good for out- the National Institute on Disability and back cruising, while pneumatic tires Rehabilitation Research (part of the are preferable on the court. U.S. Department of Education), also has some guidance about power chair Heading for the woods drive systems. Power wheelchairs have either rear- John Mryczko is president of Extreme wheel, front-wheel or mid-wheel drive. Chairing (extremechairing.com) and the The drive type has a significant effect Power Wheelchair Racing Association, on maneuverability and handling. based in the Chicago area. Both orga- Rear-wheel drive is better for nizations organize outback wheelchair outdoors travel, said Drive Medical’s treks and races. Extreme Chairing, Serhan, while mid-wheel drive is better a 501(c)3 nonprofit group, aims to on a court (soccer or hockey) setting. expand the popularity, corporate sup- “But mid-wheel drive is a little squirre- In power hockey, players sometimes tape their sticks port and geographic range of events ly if you’re going to be outdoors,” he to the wheelchair frame. for a sport that is just getting off the said. ground. The Abledata website, www.abledata. sports where impacts and steep-terrain “We’re pretty much using everyday com, contains an exhaustive list of can sometimes knock batteries (along power chairs in the events right now, wheelchair manufacturers and distribu- with the wheelchair) onto their sides. but competitors usually have their tors; a wheelchair-related organizations Shock absorbers. Most wheelchairs chairs’ controllers reprogrammed so resources list; and information about don’t have them. Roberts said optional they can get more speed. When you approved wheelchair feature standards nitrogen-filled shock absorbers sur- first get up to 10 miles an hour, it approved by the American National rounded by coil springs greatly reduce seems really fast, but after a few miles Standards Institute in cooperation with the impacts felt by a wheelchair user it just seems normal. Sometimes I even the Rehabilitation Engineering Society when traveling over rough terrain. wonder if I’m slowing down when I’m of North America. q Digital controls rather than ana- not,” Mryczko said. He hopes they’ll log. “The read-outs on your controller succeed in boosting the speed to screen look the same with both types, 20-plus mph. but digital is more precise. You get Some specialized all-terrain power more joystick sensitivity and ability to chairs have four-wheel drive; others make a fine distinction between drive have literally replaced wheels with speeds,” Roberts said. smaller versions of the metal treads TIG welds for frames. The welds used on tracked vehicles. that hold metal wheelchair structural In that regard, the Wheelchair members together can be subjected Site (www.thewheelchairsite.com), to front and sideways wrenching and an independent consumers’ guide to twisting forces strong enough to break wheelchairs, scooters and accessories, them apart during intense sports says of all-terrain wheelchairs, “Sure, action. Tungsten insert gas (TIG) welds the most rugged models will travel are stronger than those produced by over just about anything, but they drive common arc welding. like tanks!” The site suggests renting Different tires for different surfac- an all-terrain before buying, and notes es. Roberts said low-pressure wheel- that many resorts throughout North chair tires that are taller (14 inches vs. America offer all-terrain rentals to 12 inches) and wider (6 inches vs. 4 vacationing guests.

Are Power Chair Sports Really Exercise?

by Kathy Wechsler

any kids have tried power soccer or wheelchair should be able to play, says stress on the heart, says J.P. Barfield, assis- Mpower hockey at MDA summer camp Greg Carter, who co-directs the MDA/ALS tant professor of fitness and wellness at and loved it. Center at the University of Washington Tennessee Tech University in Cookeville. Everyone agrees that these sports Medical Center in Seattle. “One of the ways that we quantify physi- designed for power wheelchair users are Both sports are played on a regulation- cal activity is by examining or documenting a lot of fun — but are they also exercise? size basketball court by two teams of four. that increase in heart rate,” says Barfield, Absolutely, say the experts. And playing Power soccer players use foot guard attach- who participated in studies in which power once a year may not be enough time to reap ments to kick a 13-inch ball into a net, while soccer players’ heart rates were measured all the benefits power chair sports have to power hockey players hit a small plastic ball before and after a game. Heart rates offer. with hockey sticks, which sometimes are increased during games to meet the players’ mounted on their chairs. energy demand. What’s the game? Aerobic activity (which increases heart A heart-y workout rate) may reduce the risk of heart disease, Power soccer and hockey are similar high blood pressure and obesity. It also can to the traditional versions of the sports. While playing power soccer or hockey isn’t stimulate metabolism, burn calories, and Players are males and females of all ages. a “locomotor” activity, such as walking, run- improve mood and appetite. Any child who can safely operate a power ning or swimming, it still puts an aerobic Because the heart is stressed during

©2009 MDA 30 Quest 2009, No. 2 power chair sports, Barfield recommends “Everyone, even the that children with diseases that affect the adults in chairs, are tired heart (such as Duchenne and Becker mus- once they’re done — it’s cular dystrophies and Friedreich’s ataxia) just great to see them be first visit their doctors. able to experience that release of exhaustion Keep it moving from a game well played,” says Dena. “I played a lot Power sport players may be sitting in their of team sports and really chairs, but they’re still getting exercise. didn’t think he’d ever have “You’re balancing yourself in your chair, that feeling of ‘ahh,’ totally and as you’re moving, you’re shifting your tired from a great game.” body weight continuously to try to move where you’re pointing your chair,” Barfield Facing page: Lauren Taylor takes control of the ball and speeds down the court. Putting driving Above:Power soccer allows Joseph Miller, left, to make friends of all ages. says. “In muscular dystrophy, we see skills to the test that kids are ‘exercising’ while playing in a children have are limited,” he says. “But motorized chair just because of the other Power chair sports also can improve coor- power soccer is certainly one outlet for movements that are occurring.” dination because the body learns to process individuals with muscular dystrophy to be No matter their ability level, children try information faster, says Greg Carter. able to interact with all sorts of individuals.” to get the most possible movement out of Because players must zoom up and Not only is this good for social skills but their bodies during play. down the court without running into other it also encourages independence, he notes. For example, 12-year-old Lauren C. players, practicing their coordination and Peer connections make children more Taylor of Lewisville, Texas, used to have driving skills is a must, says Dominic likely to want to stay active so they can be her hockey stick taped to her wheelchair, Russo, president of the United States Power independent and keep seeing their friends. because her congenital muscular dystrophy Soccer Association (USPSA) in Carmel, Ind. Like all athletics, power chair sports affects her arms. Eventually, she decided to Each Saturday, Joseph’s team spends teach life skills such as self-confidence, try holding and swinging the stick instead three hours running drills to sharpen its discipline, teamwork, sportsmanship, of attaching it, because her teammates held wheelchair maneuvering skills and preparing independence and strategic options, and swung their hockey sticks. By chal- both physically and mentally for power soc- says Russo, whose two children, Natalie, lenging herself to keep up with her peers, cer plays. During drills, team members race 20, and J.C., 18, both with SMA2, play Lauren gained some physical benefits. in and out of cones, then stop suddenly and power soccer for the Ball State University “I use my arm strength to hit the ball, so drive backwards through them. Dena has Cardinals. my left arm has gotten a little stronger since noticed these practices paying off. Lauren Taylor admits she was a little I started,” she says. “My left fingers are a “I thought he was a great driver before; intimidated when she began playing hock- little more flexible than my right, consider- now he can blow your mind,” she says, ey at her local YMCA. She was the young- ing that’s the arm I play with.” noting Joseph excels at driving full speed est player and the only girl. “Sometimes she’ll move her legs like backward. “He loves to show off his driving “At first I was the shyest one there,” she’s running,” says her mother, Faith. for people.” says Lauren. “Then once I became friends “It’s just overall — she’s yelling, so she’s Power hockey adds an extra element of with everyone, I became one of the most exercising her lungs, and moving that stick eye-hand coordination for players who hold outgoing and outspoken.” exercises her arms and her shoulders.” their sticks. Adds her mother, “I’d say confidence The sport also seems to help with Lauren’s is the biggest thing that I see from her, endurance. Kickin’ it with friends because every time she makes a goal, or Dena Miller of Haverhill, Mass., consid- outplays somebody, she just lights up.” ers power soccer to be a great workout for Barfield says the chance to socialize is Faith says there’s a friendship and her 6-year-old son, Joseph, who has type 2 one of the greatest benefits of power chair camaraderie that comes with being on a spinal muscular atrophy (SMA2). When he sports. team and cheering for one another. She started playing, he’d need a nap after prac- “There aren’t many activities that are thinks it’s good for Lauren to learn to play tice, but now he stays awake and seems to designed for people with neuromuscular along with others, and to experience win- have more stamina throughout the day. disorders, so the social opportunities that ning and defeat.

31 Quest 2009, No. 2 ©2009 MDA Power sports help level the playing field, giving children with Preventing Injury disabilities common interests with able-bodied kids. henever there are power wheel- University of Washington Medical Center “I can’t tell you the number of Wchairs traveling at high speeds and in Seattle. times I’ve heard a player say they’ve maneuvering in close quarters, there’s “We’ve had a number of serious inju- always gone to soccer, basketball, bound to be a fender-bender or two. ries over the years in power wheelchair softball games to cheer on their Lauren Taylor, who has congenital sports,” he says, describing a game in brother or sister and now their muscular dystrophy, has had her share. which a player ran over the ball with his brother, sister, mom, dad are here to She’s broken some footrests (her own power wheelchair and it flipped over on cheer them on,” says Russo. and another player’s) and a few hockey top of him, causing serious facial lacera- Players aren’t the only ones sticks. tions (but no head injuries, fortunately). getting benefits from power chair Power soccer players have a foot Kids need to be securely strapped into sports, says Russo. Parents enjoy guard that goes around the front of the their wheelchairs and have plenty of head, being around other parents with chair but not so in power hockey, says neck, and spinal support, Carter empha- similar situations to swap stories, Lauren’s mom, Faith. “They’re just out sized. He suggests buying these supports advice and support. there, and when they crash into each from the wheelchair vendor. “Power soccer has been so great other, it’s very possible to get a leg hurt, Without wearing chest straps and for Joseph’s dad and me, because or whatever. I haven’t seen it happen too seatbelts, there’s significant risk of falling a few years ago we didn’t think this often, fortunately.” from the chair, particularly with the sud- possible,” Dena says. “I can finally Power wheelchair sports must be den stopping and starting in a game, he say I’m a soccer mom, and that’s carefully supervised, says Greg Carter, warns. Helmets, although not required, priceless to me.” q co-director of the MDA/ALS Center at the are probably a good idea, he adds.

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A m I Disabled or Aren’t I Disabled? by Eugène Etsebeth

hile I lay on a plinth, a Swiss doctor just before the team flew to Holland, Wgestured that I should lift up my left when I received my team kit with our leg. He bear-hugged my leg and resisted national emblem, the Springbok, embla- its elevation with his full body weight. He zoned on my tracksuit. I had achieved the grunted as he fought my movement. ultimate — to represent South Africa at He wasn’t the only person prodding an international sporting event. and inspecting me. A team of doctors, A day before my classification exam with biokineticists and physiotherapists sur- all the examiners, I was starting to feel part rounded me. It was like the United Nations of the disabled community. The opening of medics. Besides the Swiss doctor, there ceremony had over 30 countries participat- was a lady from Israel, a young athletic ing. We walked on to the famous Alkmaar Austrian and two Americans. cheese market with our country’s flags They were tallying up points of my waving in front of hundreds of onlookers. strength on a clipboard. And things weren’t I was finally making peace with my body. going too well — it was turning out that I I displayed my imperfections proudly — was too strong! along with the other athletes. I felt a sense It wasn’t suddenly apparent, but there of belonging. My body was OK. I didn’t need was a growing unease and stillness in the to conceal my imperfections. room, and knowing glances were passed between the medical tribunal. I was about Excommunicated to be eliminated — not only from the “World Championships,” but also from a The Swiss doctor placed my leg down man-made categorization that determines alongside the other. His perspiration who is “disabled” and who is not. matched my own. The medical team finished their examination by informing me that I A difficult irony do not fit into any particular locomotor category. I had too much muscle to fall into a I had trained hard for the equivalent of category to take part in the championships. the World Paralympic Championships Luckily for me, in the spirit of the competi- (called the Open European Paralympic tion, I was allowed to compete even though Championships), held under the auspices I didn’t fall into the classifications defined by of the International Paralympic Committee the IPC, the international cycling body. (IPC). My training buildup had been intense. But this irony did not sit well with me. At night I would sweat on my stationary You see, muscle is actually my biggest bike as I cycled more than two hours. On problem. It is in a constant state of atro- weekends and early mornings, I was on the phy. I get weaker by the day. It is a slow cycle track practicing starts, sprints and weakening. I have facioscapulohumeral keeping my top-end speed at over 26 miles muscular dystrophy (FSHD), or in cycling per hour on the cliff-like embankment. jargon, a slow puncture. Eugène Etsebeth My proudest moment was at the airport This hereditary condition can vary by

33 Quest 2009, No. 2 ©2009 MDA degrees. Dale, my older brother, can walk (PGD). This is a process whereby geneti- only with the help of orthotics. My situ- cists implant only embryos unaffected ation is that I can still walk, albeit with a by my faulty gene. We are to return to slight drop foot. Push-ups are out of the England to re-attempt this process. question, along with sit-ups. I use trick For me, this is the real world cham- movements to get myself out of bed in the pionships. The prize is the ability to give morning. But to the untrained eye I look my child — or, hopefully, twins — a almost perfectly normal. fresh start. To avoid all the nettles and With a jacket, loose-fitting shirt and barbs that can sting the soul of some- trousers, you don’t see my winged scapu- body who looks different. My soon-to-be lae, hunched shoulders or atrophied stom- role as a dad supersedes all concerns or ach and chest muscles. Casual onlookers worry about where I fit in. I will carve out also don’t notice that I kick the lip of raised my own sense of belonging. tiles, or feel unsteady on uneven surfaces. Cycling still runs in my blood. On any Nevertheless, the damage had been given morning you’ll find me readying done by the Paralympic organization. I my bike for a ride. I clip my shoes into had been excommunicated. My fears of my pedals and ride, oblivious to what my where I fit in society had been amplified body looks like, oblivious to the deteriora- onto a world stage — am I or aren’t I tion of my muscles. disabled? Which family do I belong to? It Rather, I revel in the clicking of my was like I was banished from the disabled gears and the familiar tapping cadence of family,yet I didn’t neatly fit in with the my pedal strokes. I cycle into the distance able-bodied folk. I was alone in the middle knowing full well that I am harnessing the somewhere — in limbo. power of my body and storing these sensa- I can’t lay the blame of this schizophren- tions deep into my memory banks. ic toggling purely on the medical examiners. I know I will ride on, long after the With cock-crowing regularity I position pedaling stops. q myself as fully healthy. At work I don’t trum- pet my genetic flaws. I don’t gush about my deterioration to new friends. I wear a cloak of normality, but underneath, the muscular truth lurks. One day in the near future, when my orthotic arrives to assist me to walk or a Segway is called upon for transport, I will finally land with my rightful family. For now, I am mentally dangling.

Finding my fit

Since being “out-classified” I have adopted a more philosophical approach to my Etsebeth and his wife, Monique, live in body’s deterioration. No man-made system Johannesburg, South Africa, where he is can pigeonhole my physique or my life. a senior business analyst for a software I have climbed Mount Kilimanjaro, cycle- development company. His extensive trav- MDA’s website is constantly toured through Europe, gotten married and els include spending a year in the U.S. in updated with the latest information achieved success at work. the mid-1990s. Says Etsebeth, “I come about the diseases in its program. My latest mission — and my wife’s — from a family that has been hit hard by mda.org • (800) 572-1717 is to have a healthy child. We already have muscular dystrophy. We all love adven- had one attempted in vitro fertilization cycle ture and travel. We are a close-knit family, ©2009, Muscular Dystrophy using preimplantation genetic diagnosis and that makes all the difference.” Association Inc.