Muscular Dystrophy Association
Encyclopedia
The Muscular Dystrophy Association (MDA) is an American organization which combats muscular dystrophy
and diseases of the nervous system
and muscular system
in general by funding research, providing medical and community services, and educating health professionals and the general public. The organisation was founded in 1950 as the Muscular Dystrophy Associations of America, renamed to its present name in the 1970s.
Many celebrities assist the organization, including Jerry Lewis
, the late Ed McMahon
, Tom Bergeron
, Jann Carl
, Ace Young
, Cynthia Garrett, Billy Gilman
, Norm Crosby
, Alison Sweeney
, Maureen McGovern
, Don Francisco
, John Ondrasik, and many more. Several notable celebrities with Muscular Disorders make personal appeals on behalf of this association, including Todd Taylor
. MDA's national headquarters are in Tucson, Arizona
.
, who has supported the MDA, serving as its national chairman since its inception in 1950. In 2008, the annual Labor Day Telethon raised a record USD
$65,031,393. In 2005, the MDA made the unprecedented decision to pledge $1 million of the Telethon's money raised to Hurricane Katrina disaster relief
, making the donation specifically to the Salvation Army
(though the Telethon also urged viewers to give to the American Red Cross
). Originally broadcast for up to 21½ hours from 1966 to 2010, the event will be cut back to six hours in 2011.
The 2011 edition of the telethon was originally announced to have been Lewis' last as host, with him continuing his role as MDA's National Chairman; however, on August 3, 2011, the MDA announced that Lewis resigned as host and chairman, due to circumstances not revealed.
Lewis' support has been so ironclad over the years that children and adults assisted by MDA are referred to as Jerry's Kids. Each year (sometimes for multiple-year stretches), one of these children is chosen to be the MDA's "National Goodwill Ambassador", which, until the 1980s, were referred to as "poster children
". The MDA's first poster child was Jolene Kay Worley, in 1955; she lived through 1993. The most well-known of these was Mattie Stepanek
, who was National Goodwill Ambassador from 2002 until his 2004 death; he was notable for his Heartsongs series of poetry books.
Every summer, for one week, hundreds of children that have been diagnosed with Muscular Dystrophy are able to attend a camp designated for only them. There is a one counselor to one camper ratio and the entire week the children, ages 6-17, are paired with an adult volunteer. They get to participate in fun activities and games and stay overnight from Sunday to Saturday. The camps are set up regionally and are different weeks throughout the months of June and July. The entire camp staff are volunteer members and are required to interview and apply with good recommendations. The cost of the camp for the campers, the children diagnosed with Muscular Dystrophy, is covered by the many fundraisers the MDA organization do each year.
The MDA and Jerry Lewis have been criticized by disability rights
activists for their tendency to paint disabled people as, these advocates say, "pitiable
victims who want and need nothing more than a big charity to take care of or cure them." Critics argue that focusing the public's attention on medical cures
to "normalize" disabled people fails to address issues like providing accessible buildings and transportation
, and employment opportunities and other civil rights
for the disabled. The Telethon has recently been targeted by disability activists who engage in street protests in front of the Telethon's location during its broadcast.
It also targets the following:
It also targets muscle diseases due to deficiencies in carnitine
and the following enzyme
s:
summary released in February 2004:
Charity Navigator
, which is the largest independent evaluator of charities, gives MDA two out of four stars for organizational efficiency and fiscal health. A two-star rating by Charity Navigator
indicates that the charity "Needs Improvement", and that the charity "Meets or nearly meets industry standards but underperforms most charities in its Cause". The primary reason that the MDA receives this relatively low rating is that 22.7% of its income is used for Administrative and Fund-raising expenses.
Muscular dystrophy
Muscular dystrophy is a group of muscle diseases that weaken the musculoskeletal system and hamper locomotion. Muscular dystrophies are characterized by progressive skeletal muscle weakness, defects in muscle proteins, and the death of muscle cells and tissue.In the 1860s, descriptions of boys who...
and diseases of the nervous system
Nervous system
The nervous system is an organ system containing a network of specialized cells called neurons that coordinate the actions of an animal and transmit signals between different parts of its body. In most animals the nervous system consists of two parts, central and peripheral. The central nervous...
and muscular system
Muscular system
The muscular system is the anatomical system of a species that allows it to move. The muscular system in vertebrates is controlled through the nervous system, although some muscles can be completely autonomous.- Muscles :...
in general by funding research, providing medical and community services, and educating health professionals and the general public. The organisation was founded in 1950 as the Muscular Dystrophy Associations of America, renamed to its present name in the 1970s.
Many celebrities assist the organization, including Jerry Lewis
Jerry Lewis
Jerry Lewis is an American comedian, actor, singer, film producer, screenwriter and film director. He is best known for his slapstick humor in film, television, stage and radio. He was originally paired up with Dean Martin in 1946, forming the famed comedy team of Martin and Lewis...
, the late Ed McMahon
Ed McMahon
Edward Peter "Ed" McMahon, Jr. was an American comedian, game show host and announcer. He is most famous for his work on television as Johnny Carson's sidekick and announcer on The Tonight Show from 1962 to 1992. He also hosted the original version of the talent show Star Search from 1983 to 1995...
, Tom Bergeron
Tom Bergeron
Tom Bergeron is an American television personality and game show host, best known as the host of the ABC reality series Dancing with the Stars and host of America's Funniest Home Videos . He was also host of Hollywood Squares and a fill-in host for Who Wants to Be a Millionaire...
, Jann Carl
Jann Carl
Jann Carl is a Los Angeles-based journalist and, from 1995 to 2008, correspondent for Entertainment Tonight. She has also appeared on Candid Camera and America's Next Top Model. She has co-hosted the Jerry Lewis MDA Telethon for 18 years...
, Ace Young
Ace Young
Brett Asa "Ace" Young is an American singer, songwriter and actor. He came to national recognition upon appearing on the fifth season of the reality television talent show, American Idol, although he was eliminated from the competition on April 19, 2006, finishing in seventh place.-Early...
, Cynthia Garrett, Billy Gilman
Billy Gilman
William Wendell "Billy" Gilman III is an American country music artist. In 2000, at the age of 12, he debuted with the single "One Voice," a Top 20 hit on the Billboard country music charts and became the youngest singer to a Top 40 hit on the country music charts...
, Norm Crosby
Norm Crosby
Norm Crosby is an American comedian sometimes associated with the Borscht Belt who often appeared on television in the 1970s. He is best known for his use of malapropisms and is often called The Master of Malaprop. He was born in Boston.-Career:Crosby went solo as a standup comedian, adopting a...
, Alison Sweeney
Alison Sweeney
Alison Sweeney is an American dramatic actress and reality show host. Sweeney is best known for her portrayal of Samantha "Sami" Gene Brady on NBC's long running Days of our Lives, a role she has played under contract with the show since January 6, 1993...
, Maureen McGovern
Maureen McGovern
Maureen Therese McGovern is an American singer and Broadway actress, well known for her premier renditions of the Oscar winning songs "The Morning After" from the 1972 film The Poseidon Adventure, and "We May Never Love Like This Again" from The Towering Inferno in 1974.-Early life:McGovern was...
, Don Francisco
Don Francisco (television host)
Mario Luis Kreutzberger Blumenfeld , better known by his stage name, Don Francisco, is a Chilean television personality, and a popular television personality on the Univision network reaching Spanish-speaking viewers in the United States...
, John Ondrasik, and many more. Several notable celebrities with Muscular Disorders make personal appeals on behalf of this association, including Todd Taylor
Todd Taylor (banjo player)
Todd Taylor is an American five string banjo player. He held the Guinness Book of World Records title for "world's fastest banjo". In a demonstration of speed play he played "Dueling Banjos" by himself January 31, 2007 in Palm Bay, Florida at an event for Guinness Book of World records. Listed in...
. MDA's national headquarters are in Tucson, Arizona
Tucson, Arizona
Tucson is a city in and the county seat of Pima County, Arizona, United States. The city is located 118 miles southeast of Phoenix and 60 miles north of the U.S.-Mexico border. The 2010 United States Census puts the city's population at 520,116 with a metropolitan area population at 1,020,200...
.
Overview
MDA is most famous for its long-running nationwide telethon it holds on Labor Day each year. Debuted in 1966, it was previously hosted by actor and comedian Jerry LewisJerry Lewis
Jerry Lewis is an American comedian, actor, singer, film producer, screenwriter and film director. He is best known for his slapstick humor in film, television, stage and radio. He was originally paired up with Dean Martin in 1946, forming the famed comedy team of Martin and Lewis...
, who has supported the MDA, serving as its national chairman since its inception in 1950. In 2008, the annual Labor Day Telethon raised a record USD
United States dollar
The United States dollar , also referred to as the American dollar, is the official currency of the United States of America. It is divided into 100 smaller units called cents or pennies....
$65,031,393. In 2005, the MDA made the unprecedented decision to pledge $1 million of the Telethon's money raised to Hurricane Katrina disaster relief
Hurricane Katrina disaster relief
The disaster recovery response to Hurricane Katrina included federal government agencies suchas the Federal Emergency Management Agency , state and local-level agencies, federal and National Guard soldiers, non-governmental organizations, charities, and private individuals...
, making the donation specifically to the Salvation Army
Salvation Army
The Salvation Army is a Protestant Christian church known for its thrift stores and charity work. It is an international movement that currently works in over a hundred countries....
(though the Telethon also urged viewers to give to the American Red Cross
American Red Cross
The American Red Cross , also known as the American National Red Cross, is a volunteer-led, humanitarian organization that provides emergency assistance, disaster relief and education inside the United States. It is the designated U.S...
). Originally broadcast for up to 21½ hours from 1966 to 2010, the event will be cut back to six hours in 2011.
The 2011 edition of the telethon was originally announced to have been Lewis' last as host, with him continuing his role as MDA's National Chairman; however, on August 3, 2011, the MDA announced that Lewis resigned as host and chairman, due to circumstances not revealed.
Lewis' support has been so ironclad over the years that children and adults assisted by MDA are referred to as Jerry's Kids. Each year (sometimes for multiple-year stretches), one of these children is chosen to be the MDA's "National Goodwill Ambassador", which, until the 1980s, were referred to as "poster children
Poster child
A poster child is a child afflicted by some disease or deformity whose picture is used on posters or other media as part of a campaign to raise money or enlist volunteers for a cause or organization...
". The MDA's first poster child was Jolene Kay Worley, in 1955; she lived through 1993. The most well-known of these was Mattie Stepanek
Mattie Stepanek
Matthew Joseph Thaddeus Stepanek , known as Mattie Stepanek, was an American poet, who had six books of poetry and one book of essays all reach The New York Times bestsellers list...
, who was National Goodwill Ambassador from 2002 until his 2004 death; he was notable for his Heartsongs series of poetry books.
Every summer, for one week, hundreds of children that have been diagnosed with Muscular Dystrophy are able to attend a camp designated for only them. There is a one counselor to one camper ratio and the entire week the children, ages 6-17, are paired with an adult volunteer. They get to participate in fun activities and games and stay overnight from Sunday to Saturday. The camps are set up regionally and are different weeks throughout the months of June and July. The entire camp staff are volunteer members and are required to interview and apply with good recommendations. The cost of the camp for the campers, the children diagnosed with Muscular Dystrophy, is covered by the many fundraisers the MDA organization do each year.
The MDA and Jerry Lewis have been criticized by disability rights
Disability rights movement
The disability rights movement is the movement to secure equal opportunities and equal rights for people with disabilities. The specific goals and demands of the movement are: accessibility and safety in transportation, architecture, and the physical environment, equal opportunities in independent...
activists for their tendency to paint disabled people as, these advocates say, "pitiable
Pity
Pity originally means feeling for others, particularly feelings of sadness or sorrow, and was once used in a comparable sense to the more modern words "sympathy" and "empathy"...
victims who want and need nothing more than a big charity to take care of or cure them." Critics argue that focusing the public's attention on medical cures
Medical model of disability
The medical model of disability is a sociopolitical model by which illness or disability, being the result of a physical condition, and which is intrinsic to the individual , may reduce the individual's quality of life, and causes clear disadvantages to the individual.It is today specifically...
to "normalize" disabled people fails to address issues like providing accessible buildings and transportation
Universal design
Universal design refers to broad-spectrum ideas meant to produce buildings, products and environments that are inherently accessible to both people without disabilities and people with disabilities....
, and employment opportunities and other civil rights
Inclusion
Inclusion may refer to:- Metallurgy :*Inclusion , a type of metal casting defect*Inclusions in Aluminium Alloys, solid particles in liquid aluminium alloy- Social inclusion of persons :...
for the disabled. The Telethon has recently been targeted by disability activists who engage in street protests in front of the Telethon's location during its broadcast.
Diseases targeted by MDA
The MDA targets the following muscular dystrophy diseases:- Duchenne muscular dystrophyDuchenne muscular dystrophyDuchenne muscular dystrophy is a recessive X-linked form of muscular dystrophy, which results in muscle degeneration, difficulty walking, breathing, and death. The incidence is 1 in 3,000 boys. Females and males are affected, though females are rarely affected and are more often carriers...
- Becker's muscular dystrophyBecker's muscular dystrophyBecker muscular dystrophy is an X-linked recessive inherited disorder characterized by slowly progressive muscle weakness of the legs and pelvis....
- Emery-Dreifuss muscular dystrophyEmery-Dreifuss muscular dystrophyEmery–Dreifuss muscular dystrophy is a condition that chiefly affects muscles used for movement and heart muscle.It is named after Alan Eglin H. Emery and Fritz E. Dreifuss.-Presentation:...
- Limb-girdle muscular dystrophyLimb-girdle muscular dystrophyLimb-girdle muscular dystrophy or Erb's muscular dystrophy is an autosomal class of muscular dystrophy that is similar but distinct from Duchenne muscular dystrophy and Becker's muscular dystrophy...
- Amyotrophic lateral sclerosisAmyotrophic lateral sclerosisAmyotrophic lateral sclerosis , also referred to as Lou Gehrig's disease, is a form of motor neuron disease caused by the degeneration of upper and lower neurons, located in the ventral horn of the spinal cord and the cortical neurons that provide their efferent input...
- Congenital muscular dystrophyCongenital muscular dystrophyCongenital muscular dystrophy is the term used to describe muscular dystrophy that is present at birth. CMD describes a number of autosomal recessive diseases of muscle weakness and possible joint deformities, present at birth and slowly progressing...
It also targets the following:
- Infantile spinal muscular atrophy
- Juvenile, Intermediate, and Adult spinal muscular atrophySpinal muscular atrophySpinal Muscular Atrophy is a neuromuscular disease characterized by degeneration of motor neurons, resulting in progressive muscular atrophy and weakness. The clinical spectrum of SMA ranges from early infant death to normal adult life with only mild weakness...
- Spinal bulbar muscular atrophyKennedy diseaseKennedy's disease or X-linked Spinal and Bulbar Muscular Atrophy or Spinobulbar Muscular Atrophy or X-Linked Bulbo-Spinal Atrophy is an X-linked recessive, slow progressing, neurodegenerative disease associated with mutation of the androgen receptor...
- DermatomyositisDermatomyositisDermatomyositis is a connective-tissue disease related to polymyositis and Bramaticosis that is characterized by inflammation of the muscles and the skin.- Causes :...
- PolymyositisPolymyositisPolymyositis is a type of chronic inflammation of the muscles related to dermatomyositis and inclusion body myositis.-Signs and symptoms:...
- Inclusion body myositisInclusion body myositisInclusion body myositis is an inflammatory muscle disease, characterized by slowly progressive weakness and wasting of both distal and proximal muscles, most apparent in the muscles of the arms and legs...
- Myasthenia gravisMyasthenia gravisMyasthenia gravis is an autoimmune neuromuscular disease leading to fluctuating muscle weakness and fatiguability...
- Lambert-Eaton myasthenic syndromeLambert-Eaton myasthenic syndromeLambert–Eaton myasthenic syndrome is a rare autoimmune disorder that is characterised by muscle weakness of the limbs...
- Congenital myasthenic syndromeCongenital myasthenic syndromeCongenital myasthenic syndrome is an inherited neuromuscular disorder caused by defects of several types at the neuromuscular junction...
- Hyperthyroid myopathy
- Hypothyroid myopathy
- Charcot-Marie-Tooth diseaseCharcot-Marie-Tooth diseaseCharcot–Marie–Tooth disease- , known also as Morbus Charcot-Marie-Tooth, Charcot-Marie-Tooth neuropathy, hereditary motor and sensory neuropathy , hereditary sensorimotor neuropathy , or peroneal muscular atrophy, is an inherited disorder of nerves that takes different forms...
- Friedreich's ataxiaFriedreich's ataxiaFriedreich's ataxia is an inherited disease that causes progressive damage to the nervous system, resulting in symptoms ranging from gait disturbance to speech problems; it can also lead to heart disease and diabetes....
- Dejerine-Sottas disease
- Myotonia congenitaMyotonia congenitaCongenital myotonia is a genetic, neuromuscular channelopathy that affects skeletal muscles . It is congenital, meaning that it is present from birth. Amongst other problems, it causes delayed relaxation of the muscles and rigidity...
, both Thomsen's and Becker's Disease - Paramyotonia congenitaParamyotonia congenitaParamyotonia Congenita , also known as Paramyotonia congenita of von Eulenburg or Eulenburg disease, is a rare congenital autosomal dominant neuromuscular disorder characterized by “paradoxical” myotonia...
- Central core diseaseCentral core diseaseCentral core disease , also known as central core myopathy, is an autosomal dominant congenital myopathy . It was first described by Shy and Magee in 1956...
- Nemaline myopathyNemaline myopathyNemaline myopathy is a congenital, hereditary neuromuscular disorder that causes muscle weakness, generally nonprogressive, of varying severity....
- Myotubular myopathy (Centronuclear myopathy)
- Periodic paralysisPeriodic paralysisPeriodic paralysis is a group of rare genetic diseases that lead to weakness or paralysis from common triggers such as cold, heat, high carbohydrate meals, not eating, stress or excitement and physical activity of any kind...
, both Hypokalemic and Hyperkalemic - Mitochondrial myopathyMitochondrial myopathyMitochondrial myopathy is a type of myopathy associated with mitochondrial disease. On biopsy, the muscle tissue of patients with this disease usually demonstrate "ragged red" muscle fibers...
, a mitochondrial diseaseMitochondrial diseaseMitochondrial diseases are a group of disorders caused by dysfunctional mitochondria, the organelles that are the "powerhouses" of the cell. Mitochondria are found in every cell of the human body except red blood cells... - Facioscapulohumeral muscular dystrophyFacioscapulohumeral muscular dystrophyFacioscapulohumeral muscular dystrophy , which is also known as Landouzy-Dejerine, is a usually autosomal dominant inherited form of muscular dystrophy that initially affects the skeletal muscles of the face , scapula and upper arms...
, FSHD
It also targets muscle diseases due to deficiencies in carnitine
Carnitine
Carnitine is a quaternary ammonium compound biosynthesized from the amino acids lysine and methionine. In living cells, it is required for the transport of fatty acids from the cytosol into the mitochondria during the breakdown of lipids for the generation of metabolic energy. It is widely...
and the following enzyme
Enzyme
Enzymes are proteins that catalyze chemical reactions. In enzymatic reactions, the molecules at the beginning of the process, called substrates, are converted into different molecules, called products. Almost all chemical reactions in a biological cell need enzymes in order to occur at rates...
s:
- PhosphorylasePhosphorylasePhosphorylases are enzymes that catalyze the addition of a phosphate group from an inorganic phosphate to an acceptor.They include allosteric enzymes that catalyze the production of glucose-1-phosphate from a glucan such as glycogen, starch or maltodextrin. Phosphorylase is also a common name used...
- Acid Maltase (Pompe's disease)
- PhosphofructokinasePhosphofructokinasePhosphofructokinase-1 is the most important regulatory enzyme of glycolysis. It is an allosteric enzyme made of 4 subunits and controlled by many activators and inhibitors...
- Debrancher enzyme (also known as Amylo-1,6-glucosidase); a glycogen storage diseaseGlycogen storage diseaseGlycogen storage disease is the result of defects in the processing of glycogen synthesis or breakdown within muscles, liver, and other cell types. GSD has two classes of cause: genetic and acquired. Genetic GSD is caused by any inborn error of metabolism involved in these processes...
also known as Forbes disease - Carnitine palmityl transferase
- Phosphoglycerate kinasePhosphoglycerate kinasePhosphoglycerate kinase is a transferase enzyme used in the seventh step of glycolysis. It transfers a phosphate group from 1,3-bisphosphoglycerate to ADP, forming ATP and 3-Phosphoglycerate....
- Phosphoglycerate mutasePhosphoglycerate mutase-Overview:Phosphoglycerate mutase is an enzyme that catalyzes step 8 of glycolysis. It catalyzes the internal transfer of a phosphate group from C-3 to C-2 which results in the conversion of 3-phosphoglycerate to 2-phosphoglycerate through a 2,3-bisphosphoglycerate intermediate.This enzyme is...
- Lactate dehydrogenaseLactate dehydrogenaseLactate dehydrogenase is an enzyme present in a wide variety of organisms, including plants and animals.Lactate dehydrogenases exist in four distinct enzyme classes. Two of them are cytochrome c-dependent enzymes, each acting on either D-lactate or L-lactate...
- Myoadenylate deaminase
Better Business Bureau and Charity assessment of MDA
According to a Better Business BureauBetter Business Bureau
The Better Business Bureau , founded in 1912, is a corporation consisting of several private business franchises of local BBB organizations based in the United States and Canada, which work through their parent corporation, the Council of Better Business Bureaus .The Better Business Bureau, through...
summary released in February 2004:
- The MDA oversees a network of 230 hospital-affiliated clinics providing diagnosis and treatment
- In 2003, 4500 children and young adults, between the ages of 6 - 21, attended week-long summer camps sponsored by the MDA
- Research and clinical trials on treatments for Lou Gehrig's diseaseAmyotrophic lateral sclerosisAmyotrophic lateral sclerosis , also referred to as Lou Gehrig's disease, is a form of motor neuron disease caused by the degeneration of upper and lower neurons, located in the ventral horn of the spinal cord and the cortical neurons that provide their efferent input...
are conducted in 30 MDA/ALS centers - The MDA has a paid staff of 1353 people
- Of the $166.5 million donated because of fund-raising activities (mostly its annual telethon), 17% of that was spent on the fund-raising activities themselves.
Charity Navigator
Charity Navigator
Charity Navigator is an independent, non-profit organization that evaluates American charities. Its stated goal is "to advance a more efficient and responsive philanthropic marketplace by evaluating the financial health of America's largest charities."-About:...
, which is the largest independent evaluator of charities, gives MDA two out of four stars for organizational efficiency and fiscal health. A two-star rating by Charity Navigator
Charity Navigator
Charity Navigator is an independent, non-profit organization that evaluates American charities. Its stated goal is "to advance a more efficient and responsive philanthropic marketplace by evaluating the financial health of America's largest charities."-About:...
indicates that the charity "Needs Improvement", and that the charity "Meets or nearly meets industry standards but underperforms most charities in its Cause". The primary reason that the MDA receives this relatively low rating is that 22.7% of its income is used for Administrative and Fund-raising expenses.
External links and sources
- Muscular Dystrophy Association's website
- Muscular Dystrophy Association's/ALS Division website
- MDA Spanish website
- MDA Puerto Rico website
- Wise Giving Alliance report on MDA from the Better Business Bureau
- The Kids Are All Right, a documentary film critical of the MDA and Jerry Lewis
- Muscular Dystrophy Association's website in Greece