AMERICAN ACADEMY OF DEVELOPMENTAL MEDICINE & DENTISTRY

BY H. BARRY WALDMAN DDS, MPH, PHD, RICK RADER, MD, FAAIDD, DHL (HON) AND STEVEN P. PERLMAN, DDS, MSCD, DHL (HON)

"One of history's most transformative human rights movements began on a steamy July afternoon 50 years ago in front of fewer than 100 spectators. Today 5 million Special Olympic athletes train year-round in 50 states and 170 countries." – Jack McCallum, Washington Post 1

Fifty years ago, one of us (HBW) had graduated from dental school ten years earlier; was then working on his PhD dissertation trying to prove that one of the obligations of the dental profession was to provide oral health services for individuals with special health care needs. (He's still at it!) The second of us (RR) was selling Belgium waffles at a stand during the 1968 World's Fair in San Antonio Texas. The third of us (SPP) had just graduated from high school and was about to enter dental school. And you (or your parents) were doing what?

WHAT IS SPECIAL OLYMPICS?

In June 1962, Eunice Kennedy Shriver started a day camp called Camp Shriver for children with intellectual disabilities at her home in Potomac, Maryland. She started this camp because of her concern about children with intellectual disabilities having very little opportunity to participate in athletic events. Using Camp Shriver as an example, Eunice Kennedy Shriver, who was head of the Joseph P. Kennedy, Jr. Foundation and a member of President John F. Kennedy's Panel on Mental Retardation, promoted the concept of involvement in physical activity and other opportunities for people with intellectual disabilities. Camp Shriver became an annual event, and the Kennedy Foundation (of which Eunice became director in 1957) gave grants to universities, recreation departments and community centers to hold similar camps. Special Olympics is a global inclusion movement using sport, health, education and leadership programs every day around the world to end discrimination against, and empower people with intellectual disabilities.Founded in 1968, and celebrating its 50th Anniversary this year, the Special Olympics movement has grown to more than 5 million athletes and unified partners in more than 170 countries. With the support of more than 1 million coaches and volunteers, Special Olympics delivers 32 Olympic-type sports and over 108,000 games and competitions throughout the year. 2

Disability is part of the human condition. Almost everyone will be temporarily or permanently impaired at some point in life, and those who survive to old age will experience increasing difficulties in functioning. Most extended families have a member with a disability, and many non-disabled people take responsibility for supporting and caring for their relatives and friends with disabilities. Every epoch has faced the moral and political issues of how best to include and support people with disabilities. This issue will become more acute as societies change and more people live to an old age.

VICTORIOUS AND BRAVE: Eunice Kennedy Shriver presents Adonis Brown, 18, of Baltimore, MD with a gold medal after he won the mile run at the International Special Olympics in Los Angeles, CA, August 17, 1972 .

Responses to disability have changed since 1970, prompted by self-organization of people with disabilities and by the growing tendency to see disability as a human rights issue. Historically, people with disabilities had largely been provided through solutions that segregated them, such as residential institutions and special schools. Policy has now shifted towards community and educational inclusion, medically focused solutions have given way to more interactive approaches recognizing that people are disabled by environmental factors as well as by their bodies.

Across the world, people with disabilities experience poorer health outcomes, achieve limited economic participation, and have higher rates of poverty than people without disabilities. This is partly because people with disabilities experience barriers in accessing services that many of us have taken for granted, including health, education, employment and transportation, as well as information. These difficulties are exacerbated in less advanced countries and in locales in the U.S. 3

IT'S THE 1960S AND 1970S

"Intellectual disability refers to a group of disorders characterized by a limited mental capacity and difficulty with adaptive behaviors such as managing money, schedules and routines, or social interactions. Intellectual disability originates before the age of 18…" 4

"Developmental disability is a severe, long term disability that can affect cognitive ability, physical functioning, or both. These disabilities appear before age 22 and are likely to be lifelong. The term 'developmental disability' encompasses intellectual disability but also includes physical disabilities…" 4

• "Historically, people with intellectual disabilities did not live as long as others and were at increased risk for health problems. Children often died because their conditions could not be diagnosed. It was common for people with intellectual disabilities to be institutionalized and healthcare was either nonexistent, ineffective, or harmful.

• Until the 1960s, screening methods to test newborns for many developmental disabilities were not yet available.

• For example, in the mid-1970s, more than 1,000 U.S. children each year acquired an intellectual disability shortly after birth because of hypothyroidism – the body's failure to produce sufficient amounts of thyroid hormone, essential for normal brain development. Although the hormone could be supplied artificially, the condition typically went undiagnosed until after permanent brain damage had occurred.

• A large study funded by NIH in the early 1970s showed that hypothyroidism could be easily detected, and treated within two weeks after birth, before any brain damage resulted. Soon, every state required thyroid hormone screening. Each year in the United States, roughly 1,000 cases of intellectual disability due to insufficient thyroid hormone are prevented.

• In the 1970s, Haemophilus Influenzae Type B (Hib), a bacterial disease that causes meningitis, was the leading cause of acquired intellectual disability. No means existed to prevent infection from Hib, which most often struck children from 6 months to 2 years old. On average, 1 in 10 infected children died from Hib meningitis, 1 in 3 became deaf, and 1 in 3 was left with an intellectual disability. Researchers at the National Institutes of Health (NIH) developed a vaccine for Hib. Their work has virtually eliminated Hib meningitis from the developed world.

• Intellectual disability also can be acquired from environmental exposure. It was not known in the early 1970s that exposure to even small amounts of lead in the environment could have an adverse effect on the developing brain. At the time, more than 10 million children had blood lead levels high enough to affect their cognitive functioning. NIH-funded research linking elevated lead levels to lower intelligence test scores led to federal laws banning lead as an ingredient in paint and as an additive in gasoline, which reduced the chances that children would be exposed to this toxic metal." 4

IT'S 2020 AND BEYOND

Approximately 6.5 million people in the United States have an intellectual disability. Approximately 1-3 percent of the global population has an intellectual disability – as many as 200 million people. Intellectual disability is significantly more common in lowincome countries – 16.4 in every 1,000 people. 5

The evaluation and classification of intellectual disability is a complex issue. There are three major criteria for a person to be designated as intellectually disabled: significant limitations in intellectual functioning, significant limitations in adaptive behavior and onset before the age of 18.

• "The IQ test is a major tool in measuring intellectual functioning, which is the mental capacity for learning, reasoning, problem solving, and so on. A test score below or around 70 – or as high as 75 – indicates a limitation in intellectual functioning.

• Conceptual skills—language and literacy; money, time and number concepts and self-direction.

• Social skills – interpersonal skills, social responsibility, selfesteem, gullibility, naïveté (i.e., wariness), social problem solving, and the ability to follow rules, obey laws, and avoid being victimized

• Practical skills – activities of daily living (personal care), occupational skills, healthcare, travel/transportation, schedules/routines, safety, use of money, use of the telephone." 6 The National Institutes of Health is supporting the development of new technologies for newborn screening. The goals are to develop fast, reliable, and cost-effective means to screen newborns and to expand the number of conditions these tests can assess. Such screening makes it possible to begin early intervention treatment when chances for success are greatest.

• "Research into the causes and early diagnosis of intellectual disabilities is a priority of the NIH-sponsored Eunice Kennedy Shriver Intellectual and Developmental Disabilities Research Centers. Researchers affiliated with these centers conduct studies to better understand the causes of such disorders and to pursue new avenues for treatment.

• Health disparities in survival and access to care are another priority for NIH research. People from disadvantaged backgrounds are less likely to receive screening services, diagnostic evaluations, or treatment interventions. Future studies will seek to identify factors that contribute to these disparities and develop new approaches that ensure equal access to early screening, therapeutic services, and treatment…

• Fragile X syndrome affects one in 2,500 births, resulting in intellectual disability, sleep problems, attention deficit disorder, aggression, and compulsive behavior. NIH-funded scientists working with mice having the same genetic mutation found in Fragile X syndrome learned that the mice have increased activity in the metabotropic glutamate receptor (mGluR), which sits atop brain cells. Researchers hope that drugs that block the mGluR receptor might one day be used to lessen the disorder's effects in humans…

• Hypoxic ischemic encephalopathy – loss of blood or oxygen to an infant's brain during birth - will lead to brain damage or death. Researchers supported by the NIH discovered that lowering a baby's body temperature in the first six hours of life could help prevent that disability…

• Duchenne muscular dystrophy occurs in about 1 in every 3,500 males. It is caused by an inherited mutation in a gene that produces dystrophin, a protein required to make muscles function. Symptoms include muscle weakness and difficulty walking and talking. Death usually occurs by age 20. In dogs with a canine form of Duchenne muscular dystrophy, researchers used DNAlike molecules called morpholinos to cover up the genetic error that causes the disease. The treatment restored functioning to the skeletal muscles, but was unable to prevent deterioration of the animals' hearts. Researchers are now seeking more effective ways to deliver the treatment to the heart…" 4

Another experimental approach by Mendell and Rodino-Klapse and approved by the Food and Drug Administration for further study employs a micro-dystrophin version to make functional muscle protein. 7

Eunice Kennedy Shriver started a day camp called Camp Shriver in 1962; which was followed in July 1968 by the first Special Olympics meeting. How could anyone have imaged that 50 years later, 5 million Special Olympic athletes would train year-round in 50 states and 170 countries? As to future... •

ABOUT THE AUTHORS: H. Barry Waldman, DDS, MPH, PhD is a SUNY Distinguished Teaching Professor, Department of General Dentistry, School of Dental Medicine, Stony Brook University, NY. Rick Rader, MD, FAAIDD, DHL (hon) is Director of Habilitation Center, Orange Grove Center, Chattanooga, TN. He is Exceptional Parent Magazine's Editor in Chief. Steven P. Perlman, DDS, MScD, DHL (Hon) is Global Clinical Director, Special Olympics, Special Smiles Clinical Professor of Pediatric Dentistry, The Boston University Goldman School of Dental Medicine.

References

1. Bottom Lines. Newsday 2018; July 2:A31. 2. Special Olympics to "Light Up" the Globe to Celebrate Inclusion \Revolution Available from: specialolympics.org/Press/2018/Light_Up_the_Globe_to_Celebrate_ Inclusion_Revolution.aspx Accessed July 22, 2018. 3. World Health Organization and World Bank. World Report on Disability. WHO Library Cataloguingin-Publication data. Available from: who.int/disabilities/world_report2011/en Accessed July 21, 2018. 4. NIH Research Portfolio on line reporting tool. Yesterday, today and tomorrow. Available from: report.nih.gov/nihfactsheets/ViewFactSheet.aspx?csid=100 Accessed July 22, 2018/ 5. Google.com Number of individuals with intellectual disabilities. Available from: google.com/search?q=number+of+intellectual+disabilities&oq=Number+intellectual+ddi sabilities&aqs=chrome.1.69i57j0l2.146182j0j4&sourceid=chrome&ie=UTF-8 Accessed July 22, 2018. 6. American Association on Intellectual and Developmental Disabilities. Frequently asked questions on intellectual disabilities Available from: aaidd.org/intellectual-disability/definition/faqs-on-intellectual- disability#.W1TSO1WnEnQ Accessed July 22, 2018. 7. Feuerstein A. With tantalizing early results, Sarepta's gene therapy for Duchenne raises hope for 'real change". Boston Globe, 2018, June 19:10,12.