Staying upright with a neuromuscular disease
Staying upright with a neuromuscular disease
Some people have nightmares about falling off cliffs. Brad Williams has nightmares about falling — period.
“Whenever I’m walking, falling down is always the major thought on my mind,” says Williams, 39, of Alexandria, Va. “It has to be on my mind a lot for me to be dreaming about it.”
Williams has Miyoshi distal myopathy, a slowly progressive form of muscular dystrophy that primarily affects the extremities. He hosts an MDA Internet chat under the nickname “dysf,” and notes that other chat participants also have reported falling nightmares.
“It’s like a flying-falling dream, except it’s just about falling down.”
For many people with neuromuscular diseases, falling is a common waking nightmare. Although most falls are merely frustrating — resulting in cuts, bruises and embarrassment — some are far more serious, breaking bones, causing head injuries and sometimes putting an end to walking.
What causes falling? More important, what “causes” stability? Are there ways to protect against injury? When is it time to go to a wheelchair? This article addresses those questions by culling the expertise of physicians and physical therapists, and the hard-knocks wisdom of people who fall.
Causes of falls
Among the causes of falling for those with weak muscles, environmental hazards play a big part, of course: ice, snow, uneven ground, wind, crowds, gravel, toddlers, dogs and cats, even well-meaning friends.
“It’s when somebody pats you on the back to say hi that you go down,” wryly observes Jon Nitz, 47, of Tucson, Ariz., who has spinal muscular atrophy (SMA) type 3.
Whatever’s happening around you, your muscle problems are the underlying cause of falls. Weakness in leg muscles can lead to foot drop and tripping, or the inability to straighten your knee on uneven ground. Arm and neck weakness can cause balance problems.
Myotonia, or lack of muscle relaxation, which occurs at times in amyotrophic lateral sclerosis (ALS), myotonic dystrophy, myotonia congenita and paramyotonia congenita, makes muscles hard to control. Muscle cramps or too-tired muscles can drop a person in mid-stride.
Contractures, lack of flexibility and poor range of motion — common to many neuromuscular disorders — make it tougher to stay upright. Difficulties with proprioception — your awareness of your posture, movement and equilibrium — can make it hard to properly place the foot while taking a step.
Body habits are another big cause of falls, says Wendy King, adjunct professor of neurology at Ohio State University in Columbus and a physical therapist in the MDA clinic there.
“Often, people just make a move too suddenly,” she explains. “They’re doing something they’ve always done, like working in the kitchen, and they turn quickly to grab something out of a drawer, and down they go.”
Concentration is critical, agrees Todd Allen, 38, of Chicago. Allen has Kennedy’s disease (spinal-bulbar muscular atrophy), which is similar to ALS but with a slower progression.
“My subconscious still believes my body works on autopilot. If I’m concentrating, I can walk across a freshly plowed field, but if I’m distracted by talking to a friend, I can easily trip on a crack in the sidewalk,” Allen says.
Moving too quickly after prolonged sitting can cause a crash. For example, in January, Brad Williams stood up after a session at his home computer. His muscles were stiff and unready and his knee buckled, resulting in a severely broken leg.
The mind plays a part as well. In spite of progressive weakness from his SMA, Nitz stood all day in his job as an auto repair facility manager by leaning against the counter.
“Psychologically, you need that wall or counter to hang onto,” he says. “Take it away and psychologically you think you’re going to fall. You kind of cause yourself to lose your balance.”
Assistance and resistance
Walking is a valuable activity for people with neuromuscular diseases who are still ambulatory. Being on your feet enhances bone growth and strength, helps prevent osteoporosis, improves muscle tone and makes it easier to interact professionally and socially.
But when falling is an issue, walking has the potential to do more harm than good. The trick is to safely maximize the benefits of walking while minimizing the dangers of falling. That’s what assistive devices are for.
Braces, canes, crutches, walkers, scooters and wheelchairs can be a big help — if you use them.
“Pride goeth before a fall,” King quotes. “For many people, it’s worth the risk of a fall not to use a device. The most common problem is not that people refuse any device, but that they use a single-point cane when they need much more than that.”
People may resist using a device because they think it looks funny, feels funny or makes them feel they’re giving up the fight to walk independently.
But the bottom line, doctors and therapists say, is safety.
“Don’t give up hope, don’t stop fighting the disease, but be smart about it!” says Anne Clark, research physical therapist at the Eleanor and Lou Gehrig MDA/ALS Center at Columbia Presbyterian Medical Center in New York. “If you’re falling all over the place, do you continue to fight [the disease]? Absolutely! But you need to stay safe, too.”
Persistence doesn’t mean you should continue to use a device that doesn’t seem to help. MDA offers annual PT and OT evaluations, which may include an assessment of walking ability, as part of its clinic program.
“Each individual is different. There is some trial and error involved,” says Carol Marulic, a physical therapist and manager of therapeutic and diagnostic services for University Medical Center in Tucson. For example, if your leg braces or AFOs make it harder to walk, then go back and work with the therapist on another solution, rather than giving up on assistive devices entirely.
In the same vein, resist the urge to buy a decorative cane at a craft fair or a used walker at a yard sale. Not only might you be wasting money because the device won’t be effective for your situation, but you may be putting yourself in danger.
Devices need to be professionally fitted, and some materials are stronger than others. Wooden canes, for example, can have dangerous stress fractures that cause them to fail when you need them most.
If you’re falling frequently — once a week in different situations — or if you’re severely limiting your activities out of fear of falling, it’s time to consider a wheelchair or scooter. Therapists often recommend using a chair or scooter to get to an activity, then walking once you get there. This not only is safer, but it helps conserve energy and ultimately may keep you walking longer.
Children tend to fall more frequently than adults but sustain fewer fractures.
“Children definitely will fall more because they’re less careful, but they’re more agile and able to pop back up. We encourage them to be active,” Marulic says. “When considering a wheelchair, the questions I always ask parents are: How often are they falling, how far can they walk safely and what is the school saying?”
Safety is key — but so is sensitivity to the affected person’s feelings about using a wheelchair or scooter. Marulic recalls a teenager with facioscapulohumeral muscular dystrophy who had needed a wheelchair for quite a while but absolutely refused to get one.
Marulic and the girl’s parents both thought she was in denial, “but I wasn’t listening to her as closely as I should have been,” Marulic confesses. “One day she told me in tears, ‘Just let me graduate out of the chair.’ She just wanted to walk down the aisle at graduation. You really have to listen to the patient and what is going on in their lives.”
Making simple home modifications and planning ahead are as useful in safe walking as are assistive devices.
Eliminate throw rugs and toys scattered on the floor. Even out variations in floor height or add handholds. Make sure furniture arms are sturdy enough to provide support when standing up. Replace a squishy deep pile carpet with a thinner, firmer one. Installing a new or used stair lift can make it safer, and easier, to go from one floor to another.
“I always ask adults, ‘Is there a rug or steps that you always trip over?’ It’s amazing how often they say yes,” says Petra Kaufmann, assistant professor of neurology, and associate director of the Pediatric MDA Neuromuscular Center at Columbia University in New York.
“They think they’ll take care of it one day but not this week. I always emphasize the importance of taking care of it right away, and recommend that they get a home evaluation.”
Professional home evaluations, offered by occupational or physical therapists, look at ways to make your house more user-friendly, both now and in the future.
Sometimes private insurance will authorize an in-home evaluation. You can do your own informal evaluation of your environment, too. Anticipate a fall, King advises.
“Look at your common pathways or places where you stand, like the kitchen, and do a ‘360.’ Ask yourself, ‘If I fell in this direction what would I hit? How about this direction?’ Then remove sharp corners or whatever.”
Children need more teaching and supervision than adults to learn caution, Kaufmann says. “It’s more of a challenge to keep them safe because they want to do the same things as everybody else,” she notes.
Review situations in which your child may need help — the playground, stairs in school or carrying books — and work together to create alternate strategies the child can live with.
Rest and sleep are crucial to fall prevention, says Patrick Griffin, 52, of Washington, Kan. Griffin’s Kennedy’s disease has given him many opportunities to “inspect the ground up close and personal.”
A former Army medic and mountain climber, Griffin now has to “listen to my body and rest before it becomes absolutely necessary,” even if it’s only a brief sit after walking across a parking lot.
He now allows himself to be helped and uses handicapped parking spaces.
“I’ve accepted that being seen as a ‘wimp’ or ‘lazy’ is preferable to falling and sustaining a concussion, or overtiring myself so that I need three days of intensive rest afterward,” Griffin says.
Falling down and standing up
Falling hurts. As muscles weaken, it becomes more and more difficult to avoid injury.
One way is to fight the instinct to put out your hands to catch yourself, which can lead to arm or wrist fractures. As unnatural as it sounds, when you’re about to fall, “relax,” Williams advises. “You’re going to fall anyway and if you’re not rigid, it makes it less likely you’ll injure yourself.”
Allen agrees. “I’ve never been severely hurt when I simply let myself go down, curling up or rolling to take the impact in the fleshier parts of my body, such as the butt and shoulders.”
Allen finds in-line skating protective gear, such as wrist guards and knee and elbow pads, a practical way to avoid injury when romping with his dogs.
Judo taught Griffin how to fall as a youngster and he finds the technique useful now. Whenever possible, roll with the fall instead of landing flat, he advises.
Tuck in your chin so your head doesn’t smash into the ground, he adds. When falling forward, extend an arm in a curve and tuck your head into it. Use the arm as a curve upon which to roll.
Often the worst part of a fall is getting back up again.
“It really helps to not get back up too quickly,” Allen says. “It helps to rest a bit and then think through exactly how to get back up.”
Williams has an armchair with a lift in it that raises him from sitting to standing position. More than once after a fall he’s made his way over to the footrest of the lift chair and used it as a hoist to get himself back up.
“That’s not what it’s designed for, but it works great,” he says.
Some people simply lack the strength to get back up by themselves. King tells horror stories of people who have lain for hours before help arrived, sometimes soiling themselves.
Get used to carrying a cell phone or emergency assistance pager at all times, she says, and don’t feel self-conscious about calling 911 for help. If you ask that the rescuers not to use their sirens, they usually won’t.
In the same vein, when he was walking, Nitz kept his front door unlocked so that rescuers could get inside if needed.
King also advises building up a network of people who expect to see or hear from you, including friends, neighbors and even the mail carrier. “If they don’t hear from you at a certain time, they can check on you.”
Wearing a heavy belt makes it easier for someone to hoist you to your feet, Griffin says. He recalls a time when he fell in front of the post office, was unable to get back up and leaned against his car to rest.
Two “little old ladies” stopped to help. He tried to wave them off but they said, “Nonsense, we’re retired nurses,” and took positions on each side of him.
Bending their knees, they grabbed the back of his belt with one hand and his shoulders with the other, then stood and raised his 190 pounds back onto his feet.
Use it or lose it
People with neuromuscular diseases sometimes find themselves caught in a double bind: They’re walking less because they’re falling, and they’re falling more because they’re not walking.
Unused muscles rapidly deteriorate. Gently exercising and stretching your muscles every day makes falls less likely.
“Exercise is not just for strengthening, but for balance, coordination and flexibility,” says Stanley J. Myers, professor of clinical rehabilitation medicine, Columbia University College of Physicians and Surgeons, New York, and attending physiatrist at New York Presbyterian Hospital.
He adds, “Physical therapy is structured exercise.”
Both adults and children should learn the exercises from a physical therapist, then faithfully perform them at home, Kaufmann emphasizes.
“School PT is not enough. We want it ongoing, at least twice a week.”
Beyond PT, gentle physical exercise also is valuable, if done properly.
Allen notes that, “In the past I’ve tended to be somewhat irregular with my exercise. I’ve gotten too excited about my progress and pushed too hard and fast, leading to an injury. Then I’d greatly curtail exercise while hurt.
“Or I’d not push enough and get bored with the process and miss too many sessions in a row. Then I’d get injured when trying to resume at the same pace where I left off.”
He gets the best results, he says, “when I manage to be very regular and consistent.”
Falling and disease progression
Do more falls mean that you’re getting weaker? Maybe yes, maybe no, Kaufmann says.
Progressive weakness is the hallmark of almost every neuromuscular disease. “If nothing else changes in the environment, yes, [falling] could mean the disease is progressing,” Kaufmann says. “But it’s important to be evaluated to see if there is anything else that could be causing it.”
Other treatable possible causes of falls include side effects from medication, other medical conditions (such as diabetes) or bad contractures. Lack of gentle daily exercise, including range of motion and stretching, can make the body stiffer and more unresponsive. Changes such as a new house, new work environment or new season can cause increased falls.
And bad fashion sense also plays a part. “I’m amazed when people with gait problems come in wearing flip-flops,” Kaufmann says.
The big fall
Nitz was told he would be using a wheelchair by the year 1995. He set a goal of walking until 2000 and actually walked one year past that.
But when an exuberant 4-year-old pushed against the back of his knees in January, causing him to crash and break his leg in several places, “I knew this was it. It finally got me,” he says.
He’s been adjusting to life in a wheelchair ever since.
By contrast, Williams, who broke his leg standing up from his computer desk and spent several weeks in the hospital, has been able to use a walker in physical therapy, and believes he one day will be independently ambulatory around his home and office.
What’s the difference? When someone is nearing the point of needing a wheelchair, a fall can put a sudden end to walking, Myers explains.
But don’t let the big fall make you give up, urges Williams. “When you break your leg, it’s easy to say, ‘Well, I won’t walk anymore.’ But medically that may not necessarily be true. Don’t give up, try physical therapy, see what you can get back.”
And if you find that a wheelchair is going to be your legs, don’t give up then either, Griffin says. Although he’s still walking, he relies more and more on his wheelchair for mobility.
“My wheelchair has reopened many places to me. I can travel much faster. It’s fun to go to a store and ‘fly’ down the aisles. No longer do I constantly have to look for my next place to rest.
“I can park blocks away and still get to a restaurant. Museums are accessible to me again. I can get fresh air and exercise, visit zoos and get back to nature.
“And wheelies are fun!”
An assistive device with heart
Elaine Gentry, 58, of Coatesville, Pa., is still ambulatory despite severe Charcot-Marie-Tooth disease (CMT), thanks to her trained service dog, Dhuey.
Before she got the black standard poodle three years ago, Gentry was falling several times a week and spending lots of time in the emergency room.
Dhuey helps Gentry in many ways. He senses when she starts to fall and becomes an anchor so she can pull or push against his harness, sometimes even leaning against her for support.
At curbs and stairs, he provides critical stability and help in pulling her up. He once helped her get back up after a fall that otherwise would have left her outside on the ground all night.
“At first I thought I might fall on Dhuey, and we’ve done a ‘little two-step dance’ every once in a while,” she admits. “But Dhuey knows how to adjust and even has saved me from taking a full-fledged fall on the ground.”
Dhuey, also known as Angel Boy, can turn lights on and off, fetch the portable phone, help take off Gentry’s jacket, and pick up things from the floor, down to a dime or a credit card. And unlike a brace or walker, he loves to cuddle.
“My very special milestone regarding ‘balance’ was the day four months after Dhuey and I graduated from Canine Partners for Life,” recalls Gentry. “Dhuey and I went to the ocean and walked down the beach at the water’s edge. This was something I could not do for 15 years.
“I cried for joy that day!”
For more information about Canine Partners for Life in Cochranville, Pa., call (610) 869-4902 or visit www.k94life.org.
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