|SERIES NO. 53
AMYOTROPHIC LATERAL SCLEROSIS
A Risk to Oregonians?
Amyotrophic lateral sclerosis, also known as Lou Gehrig's disease, is a fatal disorder often overlooked by the public; nonetheless, during 1997 it killed twice as many Oregonians (78) as did sudden infant death syndrome (36), a more widely known disorder. The age-adjusted death rate 1 for this disease has long been higher for Oregon than for the U.S., and during 1993-1996 ranked second highest nationally. 2,3 Death rates are especially high for Oregon 65- to 74-year-olds (Figure 1) (21K GIF). It is unclear why Oregon's ALS death rate rates are higher than the nation's; one possible explanation is that Oregon physicians are more likely to accurately diagnose and report the disorder.
The following briefly describes the nature of amyotrophic lateral sclerosis (ALS) and the demographic characteristics of residents dying from the disease.
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ALS Death Rates, 1993-96 2
Highest (Maine) 1.5
Lowest (D.C.) 0.5
Age-adjusted per 100,000 population.
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WHAT IS ALS?
ALS is a neurological disorder that affects the motor neurons in the brain and spinal cord. It is characterized by neurofilament buildup and diseased nerve fibers that result in a loss of control of an individual's voluntary muscles. As motor neurons die, the muscles weaken and atrophy. Early symptoms of ALS vary with each individual but may include unusually decreased endurance, stiffness and clumsiness, muscle weakness, slurred speech, and difficulty swallowing. Other manifestations include tripping, decreased grip, abnormal fatigue of the arms and/or legs, muscle cramps and twitches and excessive laughing or crying. As the disease progresses, patients gradually lose the use of their hands, arms, legs, and neck muscles, ultimately becoming paralyzed. Speech or swallowing may be lost or at least difficult. However, thinking ability, bladder, bowel, and sexual function, and the senses (sight, hearing, smell, taste, and touch) are unaffected. 4
About half of the people with ALS die within three to five years of diagnosis. About 20 percent live five years or more and 10 percent survive for more than ten years. The usual cause of death is failure of the diaphragm muscles that control breathing. Life can be prolonged with the use of a ventilator but death from infections or other complications may ensue.
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WHO DOES ALS AFFECT?
More than 30,000 Americans have ALS, with an additional 3,000-5,000 diagnosed annually. Although ALS can strike at any age, it usually appears between the ages of 40 and 70. Men and women of all ethnic and racial groups are about equally affected. During 1997, more than 4,000 Americans died of ALS. During 1980-1996, the national age-adjusted death rate for this disease increased by 50 percent, rising from 0.8 per 100,000 population to 1.2. The reason for the increase is unknown, but improved diagnosis and reporting on death certificates may be a factor.
Who Was Lou Gehrig?
Lou Gehrig, considered a great by baseball aficionados, was the New York Yankee's first baseman who benched himself on May 2,1939, ending a streak of 2,130 consecutive games. For months, his game had been in decline. His reflexes were off. He stumbled, fumbled, and struggled to hit or catch the ball. No one understood why, least of all Gehrig himself. A few weeks after Gehrig benched himself, doctors diagnosed his illness as amyotrophic lateral sclerosis. Two years later, on June 2,1941 Gehrig died at the age of 37. The disease that took his life became known to Americans as Lou Gehrig's disease.
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Nearly as many Oregonians died of ALS during 1997 as died of lymphatic leukemia.
WHAT DO THE OREGON DATA SHOW?
Number of deaths
During 1997, a record 78 Oregonians died of ALS, just 15 fewer than the number who died from AIDS. During most of the 1990s, 60 or more Oregonians succumbed annually to ALS with the age-adjusted death rate varying between 1.0-1.6 per 100,000 population, but in 1997 the rate increased to 1.8 per 100,000 population, a record high. 5 However unlike the U.S., no clear upward trend has been seen in ALS age-adjusted death rates since at least 1980.
Nationally, the age-adjusted death rate is half-again as high for men as it is for women (1.2 per 100,000 population vs. 0.8), but the disparity is nowhere near as large among Oregon residents(1.3 vs. 1.1).
The median age at death during 1997 for Oregonians suffering from ALS was 69 years (compared to 77 for all other causes). The highest death rate was recorded among 65- to 74-year-olds. Figure 2 (10k GIF) illustrates the death rates by age and sex.
County of residence
During 1986-1996, Oregon's age-adjusted ALS death rate was 1.4 per 100,000 population. Among the counties with at least five deaths during this time period, six recorded a death rate of least 2.0 (Table 1). Rates less than or equal to 1.0 were recorded in four counties; all were located west of the Cascade Mountains (Figure 3) (15K GIF).
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WHAT CAUSES ALS?
Although first described in 1874, by the French physician J.M. Charcot, the cause of the disease has remained a mystery. A number of hypotheses have been put forth, but in most cases no evidence has been found to support them. Researchers once thought that ALS might be caused by the same virus that causes polio and that exposure to polio would increase the risk of ALS. Another conjecture was that an environmental toxin might cause ALS, but the nearly uniform incidence of ALS worldwide suggests this is not the case. 6 Some physicians have suggested that ALS is an autoimmune disease where antibodies attack and kill the motor neurons. However, aggressive autoimmune therapies have been tried and have failed to alter the course of ALS.
Another hypothesis is that ALS is caused by toxic levels of glutamate in the brain. Glutamate is a protein constituent used by motor neurons to communicate with one another and abnormally high levels of glutamate have been found in the cerebrospinal fluid of some patients with ALS. Treatment with a glutamate-inhibiting drug has shown a modest effect in prolonging life in ALS victims.
A Remarkable Survivor
About 5-10 of every 100 people who get ALS have an inherited form of the disease. Children of people with familial ALS have a 50-50 chance of developing the disease themselves. In 1993, scientists identified a gene that, when defective, is associated with some cases of familial ALS. However, this genetic mutation is found in only about one-fifth of the people with familial ALS.
Stephen W. Hawking, the author of A Brief History of Time, and brilliant British theoretical physicist is one of very few people who have survived for many years with ALS (also known as motor neuron disease in Great Britain). Hawking, now 56, was diagnosed with ALS in 1963 when he was a 21-year-old graduate student at Cambridge University in England. Hawking?s life demonstrates that ALS impairs neither intellect nor sexual function. His work on the origin and nature of the universe has been "ground breaking and revolutionary." 7 Hawking married and subsequently fathered three children after his diagnosis. In 1985, after suffering a windpipe blockage, Hawking had a breathing device surgically implanted in his throat. The surgery resulted in the loss of his voice. He now "speaks" by using a voice synthesizer connected to a computer that he operates by squeezing a switch in his hand. 7
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HOW IS THE DISEASE DIAGNOSED?
Diagnosis of ALS may be difficult. There is no one test or procedure to establish the diagnosis of ALS, especially in early stages. Only through clinical examination and a series of diagnostic tests to rule out other diseases can a diagnosis be established. There are many diseases that exhibit some of the same symptoms as early ALS ? some of which are treatable. The ALS Association recommends that persons diagnosed with ALS seek a second opinion from a neurologist specializing in ALS.
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WHAT IS THE TREATMENT FOR ALS?
There is no cure for ALS. The ALS Association has summarized the situation: "Present treatment of ALS is aimed at symptom relief, prevention of complications, and maintenance of maximum optimal function and optimal quality of life. Most of this, in the later stages, requires nursing management of a patient who is alert but functionally quadriplegic with intact sensory function, bedridden and aware he or she is going to die." The cost is not only physical and emotional, but financial; in the advanced stages, care can cost $200,000 a year. One drug has been approved by the Food and Drug Administration (FDA) for the specific treatment of ALS: Rilutek, an anti-glutamate, appears to prolong the life of persons with ALS by at least a few months. However, unless a cure or prevention can be found, it is projected that 300,000 Americans alive today will die of ALS.
Despite the poor current outlook for ALS patients, researchers are pursuing a variety of avenues to better understand and treat ALS. Recent research at the Johns Hopkins Medical Institutions has shown that mutations appear to cause or contribute to more than half of all non-inherited cases of the disease; if a test can be developed to detect the mutations, then diagnosis and treatment could begin much earlier in the course of the disease. Scientists at Columbia Presbyterian Medical Center have reported finding a human gene that may delay the onset of ALS while a team at Massachusetts General Hospital has found evidence that a key programmed cell death gene may play a role, and that inhibiting activity of the gene could slow the progression of ALS. With the expanding sophistication in the fields of molecular biology, genetics, neurology and pharmacology, the prospect of finding a treatment for ALS continues to improve.
Special thanks to Dr. Wendy Johnston for reviewing this article.
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1. Age-adjusted death rates control for differences in the age structure of the subsets being compared, for example states and counties; therefore, any differences are due to factors other than age. Death rates are adjusted to the 1940 U.S. standard million population.
2. This is the most recent period for which comparative national data by state are available. Unless otherwise stated, all mortality data reported here are for the period 1993-1996 and are from the Center for Disease Control and Prevention's WONDER system. Other death data are from the Health Services?s mortality files. The underlying cause of death is determined by applying a complex set of algorithms to the information provided on a death certificate by the decedent?s physician or other certifying physician. For example, if the immediate cause of death of an ALS patient was pneumonia as a consequence of ALS, the underlying cause of death would be coded to ALS -- not pneumonia.
3. Two other states also tied for second: Washington and Kansas.
4. Much of the information (excluding Oregon data) was drawn from the following Internet sites: http://www.fda.gov/fdac/features/796_als.html, and http://www.ALSA.org/.
5. Because the number of deaths occurring in any one year is not large, rates may vary considerably from year to year due to random statistical variation.
6. Although the prevalence of ALS in Guam and parts of Japan are elevated, the reason is unknown. Ingestion of the cycad nut, a traditional food in Guam, contains a substance capable of killing motor neurons, but not at a level capable of causing the degeneration seen in ALS.
7. Michael White and John Gribbin, authors of Stephen Hawking: A Life in Science.
Oregon Health Sciences University
ALS Association, National Office
Les Turner ALS Foundation
Muscular Dystrophy Association
SW Oregon Support Group
Oregon Self-help Support Systems
503-623-5448 (Dallas Area)
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Ashes to Ashes, or the Worm?s Lament
It costs me never a stab nor squirm
To tread by chance upon a worm.
?Aha, my little dear,? I say,
Your clan will pay me back one day.?
Dorothy Parker 1
Trends | Demographic Characteristics | Oregon vs. the U.S.
Alas, for more and more Oregonians, this is no longer true. We are cheating our vermiform cousins of their rightful due. For the first time, more than half of all Oregonians who died were cremated. During the past several decades, the proportion of decedents who were cremated has increased inexorably. By 1997, and for the first time, just over half of all resident deaths ended in cremation. 2 This article briefly summarizes the demographic characteristics of Oregon residents by type of disposal of remains, information not available from the Center for Health Statistics? Vital Statistics Annual Report , but requested by data users with some frequency.
Cremation has been practiced for millennia; European pottery vessels from the Neolithic period have been found filled with human ashes. 3 Cremation was the preferred burial custom in Europe between 1400 B.C. and 200 A.D., but by the Third Century Christianity had become widely accepted and with it the doctrine that forbade cremation (because of the belief that the body could not be resurrected if it were destroyed). Since the late 1800s, however, cremation has become an increasingly popular option. (In other parts of the world, such as India and Japan, there was no gap in the practice of cremation.) Economic and sanitary considerations are the principal reasons for the increased number of cremations in recent years. 3
Disposal of Remains, Oregon Residents, 1980-97
In less than a generation, the proportion of Oregon decedents who were cremated more than doubled (Figure 1). In 1980, the first year such data were recorded, 23 percent of Oregonians who died were cremated while 65 percent were buried. By 1997, the figures were 50 percent and 39 percent, respectively. During the same time period, internment in a mausoleum became less common while removal of remains (out-of-state) became more common.
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Gender, age, race/ethnicity, years of education and county of residence are all linked to the method of disposal of remains. Table 1 presents the percentages of persons who were buried or cremated.
Gender. In 1980, males were only marginally (4.5 percent) more likely to be cremated than were females. Over time, however, this difference has continued to widen so that by 1997 males were cremated 13 percent more often than were females.
Age. Cremation is the selected option more often by/for middle-aged Oregonians than by/for their younger or older peers. Nearly two-thirds of 45-to 54-year-olds (65 percent) were cremated after death compared to about two-fifths of children ages 14 or less (37 percent) and elderly ages 85 or older (41 percent).
Race/ethnicity. Race/ethnicity, and its concomitant cultural practices, is strongly linked with the chosen method of disposal of remains. Those least likely to choose cremation were Chinese Oregonians (27 percent) while those most likely to do so were Japanese Oregonians (69 percent).
Education. Strongly correlated with the manner of disposal of remains are the years of education of the decedent -- the greater the number of years of education, the more likely the decedent was to be cremated. Among adults 25 or older, just 32 percent of those with no education were cremated compared to 63 percent of those with a post-baccalaureate education, a twofold difference.
Percentage of Decedents Cremated, by County of Residence, Oregon, 1997
County of residence. A striking geographic pattern is apparent across the state with as much as a nine-fold difference in cremation rates between counties (Table 2). Curry County, the county with the highest cremation rate for residents dying in Oregon (72 percent), is the state?s most southwestern county (Figure 2)(22K GIF). Trending northeast from Curry County, a pattern of decreasing likelihood of cremation extends along a southwest-northeast axis ending in Wallowa County, the county with the lowest cremation rate (8 percent). The reason for this pattern is unclear, but the distribution of crematoria may be a factor.
Occupation. In general, white-collar decedents and/or those in analytical/creative occupations were most likely to be cremated while lower education blue-collar workers were least likely. Table 3 lists the occupations in which the decedents were most and least likely to be cremated. 4
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OREGON VS. THE U.S.
Nationally, 21 percent of all decedents were cremated in 1996, a figure less than half that seen for Oregon. 5 (During 1996, 36 percent of Canadian decedents were cremated, but in British Columbia the figure was 65 percent.) Like Oregon, the U.S., too, shows marked geographic patterns in the proportion of decedents cremated. Rates are highest in the western states and lowest in the southern states (except for Florida). In 1996 (the most recent available data), Oregon's cremation rate (48 percent) tied for fifth highest nationally. The states with the highest proportions of cremations were: Hawaii, 58 percent; Alaska, 55 percent; Nevada, 54 percent; Washington, 49 percent; and Montana, 48 percent. The five states with the lowest proportions of cremations were: Mississippi, 4 percent; Alabama, 6 percent; Kentucky, 6 percent; West Virginia, 6 percent; and Oklahoma, 6 percent.
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1. Parker, Dorothy. Thought For A Sunshiny Morning, Scholastic, May 23, 1936.
2. The most recent year available.
3. Infopedia. Funk and Wagnall?s New Encyclopedia. SoftKey International Inc. 1996.
4. Based on all Oregon residents who died during 1996-97 and for which more than 25 deaths were recorded by occupation.
5. The most recent available national and Canadian data are for 1996 from the Internet Cremation Society
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After the Cradle, Before the Grave
Every 20 minutes a couple is married in Oregon.
Every 35 minutes a couple is divorced in Oregon.
-Fetal and Infant Mortality
-Adolescent Suicide Attempts
Every 18 minutes an Oregonian dies.
Every 1.2 hours an Oregonian dies from heart disease.
Every 1.3 hours an Oregonian dies from cancer.
Every 1.4 hours an Oregonian dies from tobacco-linked causes.
Every 3.4 hours an Oregonian dies from cerebrovascular disease.
Every 5.3 hours an Oregonian dies from chronic obstructive pulmonary disease.
Every 6.8 hours an Oregonian dies from unintentional injuries.
Every 11 hours an Oregonian dies from diabetes.
Every 12 hours an Oregonian dies from Alzheimer's disease/dementia.
Every 16 hours an Oregonian commits suicide.
Every 20 hours an Oregonian dies from gunshot wounds.
Every day an Oregonian dies from the natural effects of alcoholism.
Every 3 days an Oregonian is murdered.
Every 4 days an Oregonian dies from AIDS.
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Center for Health Statistics
Oregon Department of Human Resources
800 NE Oregon Street, Suite 225
P.O. Box 14050
Portland, Oregon 97214-0050
Send comments, questions and address changes ATTN CDP&E or phone (503) 731-4354.
Material contained in this publication is in the public domain and may be reproduced without special permission. Please credit Oregon Health Trends, Oregon Health Services.