Some people with neuromuscular diseases are experiencing grandparenthood, retirement and other welcome hallmarks of a long life. But the golden years may also bring some not-so-pleasant surprises.
The future isn't what it used to be.
A few decades ago, whenever a child was found to have muscular dystrophy, spinal muscular atrophy or another neuromuscular disorder, doctors predicted a grim future — perhaps one that would end in childhood. While some neuromuscular disorders continue to be fatal in a few years, others aren't so bleak. Many who received those dire prognoses as kids 30, 40 or 50 years ago are still here. For this, they can thank dramatic changes in health care and assistive technology, and an increased life expectancy for Americans at large.
People over 40 with neuromuscular diseases can celebrate the greater longevity and better quality of life these changes have afforded. But what their doctors didn't know decades ago was that middle age often brings a new set of medical and functional challenges to those with long-term mobility disorders.
Like everyone else in the middle and later years, people aging with neuromuscular disease face predictable health risks, such as heart disease, osteoporosis, diabetes, arthritis, hypertension, and loss of acuity in sight and hearing.
But for those with early-onset mobility impairments, these changes may come much earlier in life. And, because of long-term effects of a disabling condition, the changes will be met with what one researcher calls "a narrow margin of health," that is, less resiliency for accommodating to the change.
A relatively new area of rehabilitation research is confirming what some 40-, 50- and 60-year-olds with neuromuscular diseases have discovered for themselves: Aging can be very tough on people with disabilities. As the T-shirt slogan says: "Old age is not for sissies."
Studies show that people aging with a motor disability often experience a cluster of interrelated medical conditions that may include pulmonary problems such as asthma or respiratory decline, diabetes, heart disorders, severe osteoporosis, and increased muscle fatigue, weakness and pain. Depression and dramatic functional decline — further loss of physical ability — also can occur with age.
People with physical disabilities have much higher rates of these conditions than do people of the same age without disabilities. Researchers have also found that these losses and changes may occur 20 to 25 years earlier in life for those who've been living with a disability for several years.
If several of these "secondary conditions" occur together, a person may need to restructure work hours or retire early. This cluster of late-life effects may mean a loss of independence or a drastic change in activities.
Only a few of the studies focus on aging with muscular dystrophy and other disorders covered by MDA. Other studies have explored aging with postpolio syndrome, stroke, spinal cord injury, rheumatoid arthritis, cerebral palsy and disability in general. But experts acknowledge that many of the findings in those inquiries are likely to apply to MDA-covered disorders as well.
As this field of inquiry continues to shed light on the aging process, here are some highlights.
Bryan Kemp, director of the Rehabilitation Research and Training Center on Aging with a Disability at Rancho Los Amigos Medical Center in Downey, Calif., put the dilemma in perspective.
Speaking at an American Society on Aging conference earlier this year, he said that, while life expectancy for people with long-term disabilities has improved drastically over the last few decades, "The increased life expectancy isn't accompanied by necessarily good health or good quality of life."
Much current research on midlife and late-life change started with identification of the postpolio syndrome. In this condition, some people with childhood polio develop new neurologic symptoms 20 or more years after their earlier conditions have stabilized.
Kemp says, "We think we've found there's probably a late-life syndrome attached to any other impairment as well. For any given impairment, there's a significant midlife and later onset of problems that we didn't know about 15 or 20 years ago, which have a significant impact on life."
A five-year aging with disability study conducted by Rancho Los Amigos revealed details about the aging process in people over 45 with polio, rheumatoid arthritis and stroke. When the health history of those surveyed was compared to that of people of the same age in the general population, the subjects with disabling conditions reported much higher frequencies of 15 out of 17 medical conditions usually associated with aging.
For many conditions, the rate was twice as high or more, up to 10 times as high. The conditions include osteoarthritis, vision problems, digestive disorders, diabetes, thyroid disorders, asthma, emphysema, high blood pressure and stroke.
|"Will our health allow us to keep doing what we want to do whether it's work or travel or play or what?"|
These secondary conditions are more advanced in those with disabilities than in those without disabilities of the same age because they began much earlier in the disabled group. Researchers call this phenomenon accelerated or fast-track aging.
Margaret L. Campbell, the study's primary investigator, says the term accelerated aging means that "the timing of onset of these age-related conditions is significantly earlier, placing people out of sync with their peers and out of sync with their stage in the life cycle. So you get people having to leave the work force at very young ages, which is not only inconvenient, it can also have really serious financial ramifications. Or being able to maintain their role as a parent, for example, becomes very difficult to do because of this accelerated aging."
Campbell, now a rehabilitation program specialist at the National Institute on Disability and Rehabilitation Research in Washington, says the causes of accelerated aging are complex. Each disabling disorder puts people at risk for different secondary conditions. The reasons for accelerated aging "vary by the nature of the underlying impairment, sedentary condition and lifestyle," she says.
Campbell identifies four categories of reasons why an individual might experience accelerated aging, all of which could apply to people with long-term neuromuscular disorders:
Overuse can be seen in people who experience stress injuries from many years of operating a manual wheelchair. Misuse also includes "doing more than they have the residual capacity to do," such as walking long after it's comfortable or advisable and with resulting gait problems. These behaviors can lead to more rapid onset of osteoporosis, scoliosis, lower back pain, hip pain and other effects, Campbell says.
Underuse is a risk for those who are sedentary, such as long-term wheelchair users. If there's no effort to maintain joint flexibility or use muscle capacity, however limited, the resulting poor conditioning can lead to earlier chronic pain and contractures. Campbell adds that a sedentary life "gives rise, of course, to functional problems but also to obesity, and obesity leads to diabetes and heart problems."
Environmental and attitudinal barriers may limit a person's access to preventive health services and to social and professional opportunities. Campbell says attitudes on the part of society at large, health care providers and people with disabilities themselves can stand in the way of a person getting needed technical assistance and health care.
An example is people with neuromuscular disorders who "have internalized the stigma against wheelchair use and the strong bias in our society for walking." Their continued walking may lead to overuse of weakened muscles and its complications.
The fourth factor in accelerated aging is conditions related to the original neuromuscular disorder — for example, heart problems in Becker or limb-girdle dystrophy; respiratory decline in SMA and other disorders; or dangers of surgery in myotonic dystrophy (MMD).
Though complications of neuromuscular disease may be unavoidable, maintaining good health where possible and monitoring of secondary conditions can minimize or delay their effects. A lifetime of good health habits and careful protection of functional abilities seems to pay off in later years.
Poor health care
Longer life expectancy makes good health care even more important for adults with neuromuscular diseases. In an age of managed care, this can be difficult. But experts in aging with disability stress that becoming a strong self-advocate in the health care system is essential.
June Isaacson Kailes, a health and wellness consultant, also spoke at the recent American Society on Aging conference. She said, "The question is not, are we going to live, but how well will we live?" Kailes, who has cerebral palsy, notes that when she began to experience some functional changes, her primary care doctors dismissed her concerns with "the reflex response: 'You're just getting older, it's the natural course of events."
But Kailes found that others with disabilities were seeing similar changes — loss of energy, more difficulty walking, respiratory decline and others — for which they were unprepared. Such losses could mean a drastic decrease in independence to people who already had disabilities.
"For a lot of us who were told our disabilities were quite static, losing any part of our independence is a violation of our expectations," Kailes said.
When people in the prime of life lose vital abilities, she says, "often the differences are really quite significant. I've observed a lot of people struggling with these aging issues give up everything except work. I see people come home and collapse, sleep the weekends, and go to work again on Monday because that's what they value most. It's hard to see, it's hard to watch."
Among the concerns of people facing the aging challenges, she lists: "Will our health allow us to keep doing what we want to do, whether it's work or travel or play or what? We worry about maintaining employment, relationships and health insurance. We also worry about being able to afford the technology we're going to need over time."
Exercise and diet
|Remaining active and getting moderate exercise can have beneficial health effects.|
Good diet and exercise may help people aging with a disability preserve maximum functioning and postpone secondary conditions.
R. Ted Abresch, director of research at the Research and Training Center in Neuromuscular Disease at the University of California at Davis, is studying the impact of exercise and diet on 1,200 people with "more slowly progressive muscular dystrophies," such as limb-girdle, facioscapulohumeral and myotonic dystrophies. The study isn't finished, but Abresch says anecdotal results imply that "moderate exercise certainly is not deleterious. It seems to be beneficial."
People with neuromuscular disorders are "deconditioned," he says. Earlier research showed that when they're given moderate strengthening and endurance exercise programs fitting their capabilities some beneficial results appear.
Further studies will help determine at what point physical exertion becomes damaging and to what point it helps." What we're trying to understand is, what is the role of exercise?" Abresch says. "Some of the secondary problems are definitely, of course, due to the progression of the disease. But we're trying to understand what secondary problems are really due to lack of activity because of lifestyle and physical activity changes."
Researchers expect their efforts will support the hypothesis that moderate exercise — physical therapy, swimming, gardening, adapted sports, walking if possible — helps to relieve symptoms such as pain and fatigue, and may help improve cardiovascular health.
Getting ready for getting older
At this stage, findings on aging with disability serve primarily to alert people with neuromuscular disorders to possible future developments in their health. While researchers continue to learn more about the impact of aging on specific disabilities, and advocates work for changes in public health policy, Campbell has a few suggestions for individuals anticipating the future.
"As we become more entrenched in a consumer-driven health care system, i.e., managed care, persons with long-term muscular dystrophy need to become better advocates," she says. "That is as critical to their health and well-being as anything else."
She also emphasizes the importance of "lifestyle modification," which includes "functional adaptations, ways of doing things that conserve energy and that modify your lifestyle." These techniques could include taking frequent rest breaks, and negotiating duties with family members and co-workers. Another modification is use of assistive technology, including wheelchairs, scooters and adapted computers, to minimize stress on weakened muscles.
Maintaining function requires a careful balance between using physical abilities to keep them going, and not overusing them so they deteriorate earlier than necessary. Modifying your home for maximum comfort and accessibility is part of this process.
Energy-saving techniques can be discussed with your doctor and physical and occupational therapists. MDA support groups can be another resource to help anticipate changes and find out how others have handled them.
For a more detailed report on the aging with a disability study, see "Secondary Health Conditions Among Middle-Aged Individuals with Chronic Physical Disabilities: Implications for Unmet Needs for Services," by Margaret L. Campbell, Debra Sheets and Patricia S. Strong, in Assistive Technology, Vol. 11, No. 2.
A new National Center on Physical Activity and Disability at the University of Illinois at Chicago offers guidelines on exercise and daily activities; go to www.ncpad.org or call (800) 900-8086.