AMERICAN ACADEMY OF DEVELOPMENTAL MEDICINE & DENTISTRY
Our Child Has An Intellectual Disability – Should We Be Concerned About Having A Second Child?
BY H. BARRY WALDMAN, DDS, MPH, PHD, STEVEN P. PERLMAN, DDS, MSCD, DHL (HON), JEFFREY SEIVER, DDS AND RICK RADER, MD, DHL (HON)
HISTORY
Three Generations of Imbeciles Are Enough "So wrote Justice Oliver Wendell Holmes, Jr. in Buck v. Bell, a 1927 (U.S.) Supreme court case upholding a Virginia law that authorized the state to surgically sterilize certain 'mental defectives' without their consent." 1
Carrie Buck was a patient in the Virginia State Colony for Epileptics and Feeble-minded. Upon a finding that she was "…the probable potential parent of socially inadequate offspring, likewise afflicted, that she may be sexually sterilized without detriment to her general health, and that her welfare and that of society will be promoted by her sterilization, the Court upheld her involuntary tubal ligation. The Court infamously justified its decision… It is better for all the world if, instead of waiting to execute degenerate offsprings for crime or to let them starve for their imbecility, society can prevent those who are manifestly unfit from continuing their kind. Buck herself did not learn of her sterilization until decades later — she was told at the time that the operation was an appendectomy… more than 65,000 people were sterilized under such laws, which were enacted in more than 30 states… (State laws permitted) sterilization of individuals deemed unfit to reproduce — most commonly institutionalized persons with mental illness, or even conditions such as epilepsy …" 1
The doctors who sterilized Carrie Buck claimed she was a "feeble-minded" woman whose future offspring posed a threat to society. Her life paints a very different picture. As a youngster, Carrie was fostered by the Dobbs family. As a teenager she gave birth to a child out of wedlock, fathered by a nephew of the Dobbs. After learning of Carrie's pregnancy, the Dobbs petitioned to have her institutionalized, claiming that she was "feeble-minded." 2
MORE RECENTLY
Eugenics is the science of improving a human population by controlled breeding to increase the occurrence of desirable heritable characteristics. Developed largely by Francis Galton as a method of improving the human race, it fell into disfavor only after the perversion of its doctrines by the Nazis. 3
THE DEEP END: The older the age of the pregnant women, the greater the risk of having a child with Down syndrome; At age 25, her risk of having a baby with Down syndrome is 1 in 1,340. By the time she is 45, that risk climbs to 1 in 35.
"Most cases of severe intellectual disability (formerly called mental retardation) are not inherited, but are instead the result of random genetic mutations… The finding should offer reassurances to parents of those with intellectual disability, (who are considering having more children) that the likelihood of passing on the condition is low…" 4 Mutations in a group of genes associated with brain activity frequently cause an intellectual disability.5 While some genes have been identified as causing an intellectual disability (ID), it's unclear what leads to the condition in most people. 4
YES, THERE ARE INHERITED GENETIC CAUSES OF ID
Two of the most common are: Down syndrome, the most common genetic origin of ID, occurs in 1 out of every 700 births. Down syndrome derives in name from John Langdon Down, who first pinpointed the disorder. The condition is caused by an extra chromosome. An error in cell division during prenatal growth results in an extra third chromosome 21. The extra chromosome is called Trisomy 21.
Down syndrome has a unique pattern of symptoms which doctors recognize at birth. The child's eyes usually have an upward slant. There also are white spots on the iris. The child's ears have an unusual shape. The neck is shorter than usual. The shape of the face is full. The profile of the face tends to be flat. The palm of the hand may have a profound crease running cross ways. A person with Down syndrome may not have all of these physical features. Then definitive diagnosis of Down syndrome requires a blood test to reveal abnormal Trisomy 21.6
The older the age of the pregnant women, the greater the risk of having a child with Down syndrome. If the woman becomes pregnant:
• At age 25, her risk of having a baby with Down syndrome is 1 in 1,340. • At age 30, her risk is 1 in 353. • At age 40, her risk is 1 in 85. • At age 45, her risk is 1 in 35. 7
The child with Down syndrome may have problems such as heart defects, respiratory problems and eye defects, and may variously exhibit the following characteristics: auditory and visual impairment, delayed fine- and gross-motor skills, difficulties with thinking and reasoning and applying knowledge in new situations.7 Adults with Down syndrome experience "accelerated aging," meaning that in their 40s and 50s they experience certain conditions that are more commonly seen in elderly adults in the general population; such as dementia.8
Fragile X syndrome is one of the most common forms of inherited ID affecting 1 in 5,000 male births. Usually males are more severely affected by this disorder than females. Most affected males have mild to moderate intellectual disability, while about one-third of affected females are intellectually disabled. They may have attention deficit disorder. About one-third of individuals with fragile X syndrome have features of autism spectrum disorder. Most males and about half of females have characteristic physical features that become more apparent with age; including a long and narrow face, large ears, a prominent jaw and forehead.
NO GUARANTEES: In most cases, intellectual disabilities are the result of random mutations of genes, which could just as well produce marvels of memory or general intelligence.
The impact on particular genes prevents necessary protein material which leads to defects in synaptic transmissions. The syndrome gets its name from the fact that when cellular chromosomes from individuals with the syndrome split during reproduction, they appear to contain a gap or constriction at the end of the long arm of the single X chromosome. 9
But again, "Most cases of severe intellectual disability (formerly called mental retardation) are not inherited, and are instead the result of random genetic mutations. Consultation with a geneticist and your pediatrician may be advisable.
WHAT ABOUT ANOTHER CHILD?
Do you remember the joy when you both learned that you were pregnant? As the months went by, the excitement only increased as the all-important anticipated date of meeting your first child neared. Upon arrival she (or he) was so tiny and beautiful. You lost sleep, took turns caring for those few pounds of joy; trying to figure out how such a tiny squirming little person could require so much care and attention especially since this little package came without instructions or a use by date, guarantee or warranty and could not be returned to the manufacturer. There was the first bath, somehow trying to figuring out how to clip those tiny finger and toe nails that scratched everything, the unbelievable numbers of laundries and the usual up-chucking of milk.
Yes, they were difficult but happy times. Later there were the challenging periods when you learned of your first child's limitations. But there was so much that the new little person could do that expanded the life and joy of your family. Come to think of it, your marriage also did not come with any warrantees and guarantees.
As to a second (or even a third) child, repeated studies indicate in most cases, intellectual disabilities are not inherited, but the result of random mutations of genes that are associated with the brain – which could just as well be mutations which produce marvels of memory or general intelligence.
OVERVIEW
It has been almost a century since U.S. Supreme Court Justice Oliver Wendell Holmes, Jr. in Buck v. Bell, in 1927 wrote that "Three Generations of Imbeciles Are Enough." At the time of his writing, the germ theory of medicine (by Louis Pasteur) and Charles Darwin's book On the Origin of Species were only fifty-plus years old. The first antibiotic (penicillin) was discovered because Alexander Fleming forgot to clean an uncovered petri dish in 1928. In the past, individuals with intellectual and other disabilities were out of sight in large institutions or back rooms of homes. In 1967, there were more than a quarter of a million individuals with ID (then referred to as mental retardation) and other developmen tal disabilities (DD) in state institutions. It was not until the end of the twentieth century that the number of residents with ID/DD decreased by 75%. 10
Today, individuals with intellectual and developmental disabilities are truly members of our society. They live and work in our communities and partake of the many activities that are available to all of us. It's time to forget Justice Oliver Wendell Holmes and his archaic world and plan our family's future; especially when modern research and knowledge provides realistic expectations of the safe and productive setting that we can offer our children.
ABOUT THE AUTHORS:
H. Barry Waldman, DDS, MPH, PhD is a SUNY Distinguished Teaching Professor, Department of General Dentistry, Stony Brook University, NY. E-mail: h.waldman@stonybrook.edu Steven P. Perlman, DDS, MScD, DHL (Hon) is the Global Clinical Director and founder, Special Olympics, Special Smiles and Clinical Professor of Pediatric Dentistry, The Boston University Goldman School of Dental Medicine. Jeffrey Seiver, DDS is Clinical Assistant Professor, Department of General Dentistry, Stony Brook University, NY. Rick Rader, MD, DHL (Hon) is Director of Morton J. Kent Habilitation Center, Orange Grove, Chattanooga, TN; Senior VP Public Policy, American Academy of Developmental Medicine and Dentistry; Adjunct Professor, Human Development, University of Tennessee-Chattanooga. He is Exceptional Parent magazine's Editor in Chief.
References
1. Doerr A. Three generation of imbeciles are enough. Available from: theprivacyreport.com/2009/06/25/three-generations-of- imbeciles-are-enough/UNE 25, 2009. Accessed January 9, 2019. 2. Brosnahan C. Finding Carrie Buck. Available from: stories. americanexperience.org/finding-carrie-buck Accessed January 9, 2019. 3. Google definitions. Available from: google.com Accessed January 9, 2019 4. Hensley S. Often not inherited. Disability Scoop. Available from: disabilityscoop.com/2012/09/27/intellectual-dis- ability-not-inherited/16538 Accessed from: January 9, 2019. 5. Science News. Intellectual disability is frequently caused by non-hereditary genetic problem… Available from: science daily.com/releases/2011/04/110418114005.htm Accessed January 9, 2019. 6. Reynolds T, Zupanick CE, Dombeck M. Genetic Causes of Intellectual Disabilities: Down syndrome. Available from: mentalhelp.net/articles/genetic-causes-of-intellectual-dis- abilities-down-syndrome/ Accessed January 9, 2019. 7. March of Dimes. Down syndrome. Key points. Available at: marchofdimes.org/baby/down- syndrome.aspx?gclid=EAIaIQobChMIlZvCjszh3wIVzZzCh3WQwMLEAAYAiAAEgJu7vD_BwE Accessed January 9, 2019. 8. National Down Syndrome Society. Aging and Down Syndrome Available from: ndss.org/wp-content/uploads/2017/11 Aging-and-Down-Syndrome.pdf Accessed January 9, 2019. 9. National Institute of Health. Guide to understand genetic condi tionsnih.gov/condition. Available from: ghr.nlm/nih.gov/condi- tion/fragile-x-conditions Accessed January 9, 2019. 10. Anderson, LL, Lakin C, Mangan TW, et al. State institutions: thirty years of depopulation and closure. Mental Retardation 36:431443, December 1998.
The American Academy of Developmental Medicine and Dentistry (AADMD) was organized in 2002 to provide a forum for healthcare professionals who provide clinical care to people with neurodevelopmental disorders and intellectual disabilities (ND/ID). The mission of the organization is to improve the quality and assure the parity of healthcare for individuals with neurodevelopmental disorders and intellectual disabilities throughout the lifespan.