The conventional wisdom is to put retarded children in an institution. The conventional wisdom is wrong.

October 1977By Comments

The traits of Down’s syndrome include mental retardation, short stature, and stubby limbs, but it was the slanted eyes and a Mongolian-like fold of the upper eyelid that caught the fancy of a nineteenth-century English doctor.

Langdon Down, familiar with the then new work of Charles Darwin on the evolution of species, described the condition that came to bear his name in a paper titled “Ethnic Classifications of Idiots.” In it he concluded that the mongoloid represented a genetic regression to the supposedly more primitive Mongolian race. He was wrong, but some people accepted his theory, and in 1924, F. G. Crookshank took Down’s theory one step further; in a book titled The Mongol in Our Midst he wrote that the mongoloid was not a throwback to a primitive Oriental human being, but rather to the orangutan. Even today the straight flexion crease found on the palms of many mongoloids is known in medical parlance as the simian line, in reference to a similar crease found on the palms of apes.

Jennie is more like a monkey. Just now as I was typing this, she scampered into the study, put her hands on her hips and said “Hi, Da!” She crinkled her half-moon eyes and gave me a bright, Brushfield-spotted smile (Brushfield spots, white places in the irises or mongoloids, represents defects in the eye-coloring layer). As she was whirling to leave, I gave her a kiss on the head. She smiled and brushed it off. It’s not that she doesn’t like to be kissed. She loves to be kissed, but she brushes all kisses off. It’s what her brothers and sisters do when Jennie kisses them, and Jennie’s picked it up.

Jennie’s eight, and she is mentally and physically retarded. Full grown, she’ll stand no more than five feet. Her mental age now is about four, but it may increase to that of a child of seven or eight. She is perceptive in many ways. She can recognize the way to the store or to Mona’s house (where she stays before and after school), and her memory is excellent. She never forgets a meal and can remember which toys and books belong to her. She’s also capable of holding a grudge when she feels she’s been slighted, and she’s perfectly normal in her ability as the youngest child to tattle on her brothers and sisters if they’ve mistreated her (which is rare).

Food is a big item in her day. The first thing she does in the morning after her mother’s hug is say “Seer-ul.” Once, when she kept gaining weight despite our efforts to restrict her food intake, we became suspicious that she was eating too much at school. My wife, Mimi, and I spoke to Jennie’s teacher, and were told proudly, “Oh now, Jennie—she’s one of my good eaters. She’ll even clean off the others’ plates.” That, we tried tactfully to explain, was just the problem.

The thing they teach you about birth defects during medical training is that when one is present, look for others. A child with a clubfoot may have a cleft palate as well; one with congenital cataracts may also have a heart murmur. In Jennie’s case, she has duodenal atresia, a condition that often occurs with mongolism. The first part of her small intestine was a blind, obstructed pouch rather than a clear passageway from stomach to colon. From birth, she vomited all her nourishment. Strange that nature should accompany its imperfection with a self-destruct mechanism, and yet, maybe not so strange either.

Although it was months before Jennie’s mongoloid condition would be confirmed, the diagnosis of her bowel obstruction was made two days after she was born. Our pediatrician recommended immediate surgery, and by nine o’clock that night she was in the operating room. The bypass was successful, and soon Jennie was greedily taking milk.

A week after she left the hospital, we moved from Sacramento to Madison, Wisconsin, where for a year I did research in medical genetics. We discovered that John Opitz, a well-known medical geneticist, had recently moved to Madison. He saw Jennie and confirmed the diagnosis of mongolism. Opitz held Jennie in his arms as he counseled us. “She sleeps a lot, doesn’t she?” he asked. My wife nodded. Opitz smiled. “Mongoloids are such good babies.” He didn’t seem to want to stop holding Jennie, but holding her was difficult. Floppiness is one of the features of a newborn mongoloid. It’s due to an underdeveloped nervous system, and Jennie had it in full measure. She’d slip right out of your lap if you didn’t hold her closely. “You know she won’t live as long as your other children,” Opitz cautioned, “but love her and keep her with you.” The average age of death of institutionalized mongoloids is about 30 to 35 years. While I was doing medical work at a state school in South Texas I encountered several mongoloids in their late sixties and early seventies in good health. Recent studies show that mongoloids who survive past age five have a good chance of living to adulthood.

We were grateful for Opitz’ advice, because we’d been told earlier to put Jennie in an institution. It was a temptation, since I myself had been taught in medical school to recommend institutionalization for all retarded children. “You can’t do anything for the child,” the pediatrics professor had said, “so do something for the parents.” In fact, you can do a lot for a mongoloid child, and for children who are retarded from other causes. You start with love and do most of the same things you would do in raising a normal child. We have found that the fears we had about a handicapped child not fitting into the family were unwarranted.

Jennie is the hub of our family. Her brothers and sister accept her as a special person but not as a sluggish retardate. When Roger, the oldest and most serious of our children, was eight, I asked him to write down what he thought of Jennie, who was then a year and a half. Here is what he wrote: “She was born in 1969. I didn’t see her until about a month after she was born. The first thing she learned to do was clap her hands. Second she learned to say ‘bye.’ It was not until 1970 that she learned to pucker up with you did. Boy! That sure made our family happy! Jennie is mentally retarded. Yet, I like her just the way she is. She has not learned to walk, but I think she wants to very much. And learn a lot more things, too. She learns very fast for a mentally retarded child.”

Jennie rides her “ri-tickle,” plays on her swing set, loves hide-and-seek, and can do a fair job of watering the yard. She “reads,” cleans up after herself, and watches TV. She is not a victim of “mongolian idiocy”—a term that still appears in medical texts and one that is not only offensive but also wrong. In the first place, the mongoloid is not a throwback. The eyes of a person with Down’s syndrome are slanted and partially covered by a skin fold that is suggestive of the eye fold in a person of Oriental extraction. However, in the Oriental the fold of skin extends from the bridge of the nose all the way across the eye. Indeed, it partially covers the upper eyelid. The eye fold of a mongoloid child is much shorter, starting at the bridge of the nose and ending just above the corner of the eye. It resembles a small pinch of skin that didn’t get tucked in, and it often disappears by the time the child is an adolescent. The notion of “idiocy” is misleading, too. A few people with mongolism are able to learn to read and write. And most are not so severely retarded to merit being called idiot. The modern practice is not to use words like “idiot,” “imbecile,” or “moron” when referring to retarded persons. The more specific and less offensive terms are “profound,” “moderate,” or “mild” retardation.

The cause of mongolism is a genetic accident. A normal fertilized egg of a human contains 46 chromosomes; a mongoloid begins with one extra. Within each chromosome there are thousands of genes containing the hereditary material DNA. Even one defective gene can cause abnormal traits in an individual, so you can imagine the problems an entire extra chromosome in each cell might cause. It isn’t understood exactly how the additional hereditary material produces the features of mongolism. We do know that the extra chromosome is a number 21 chromosome, a relatively obscure-looking structure when compared in a microscope’s field with the rest of the cell’s chromosomes. Chromosomes occur in pairs, and the extra 21 added to the cell’s two normal 21s creates a trio; hence, the genetic name for mongolism is trisomy 21.

How is mongolism transmitted? In most cases a genetic mix-up occurs in either the egg or the sperm, which brings one extra chromosome to the new person. This extra chromosome is more likely to be present in the egg than the sperm. A woman is born with all the eggs she will ever have, and these continuously undergo division in her ovaries during her reproductive years. The more years in which the eggs have to divide, the greater the likelihood of a genetic accident occurring. Thus, from 30 on, the chances of a woman having a mongoloid doubles every 5 years. A pregnant woman 45 or older runs a 1 in 65 risk of having a mongoloid baby, compared to a 1 in 1500 risk is she becomes pregnant before age 30. By contrast, the father’s age has no bearing on whether a pregnancy will result in a mongoloid child. A man’s sperm cells at the time of ejaculation are no more than six weeks old, and much younger if the man ejaculates often.

The chromosome defect responsible for mongolism was discovered by Jerome LeJeune and his coworkers in Paris in 1959, and only since then have doctors been able to distinguish with certainty between mongolism and cretinism, a similar-looking defect of the newborn. Cretinism results from a failure of the thyroid gland, and the condition can be cured by giving the infant thyroid hormone. Quacks continue to take advantage of this onetime confusion, and now and then I read of a “miracle way” to cure mongolism by giving the child thyroid hormone.

Jennie fits into the moderate range of retardation. Like many persons with Down’s syndrome, she is trainable but educable. She can learn a few reading and writing skills, but she will never be literate. For the record, she walked at age two, said her first word (bye!) at eight months, and didn’t begin using simple sentences until she was six. Her social skills and ability to mimic have always outstripped her verbal achievements. One morning mimi hiccupped just as she went in to awaken Jennie. It made a loud HO-WHUP sound. Jennie didn’t seem to pay any attention, but the next morning when Mimi went in to get her, she woke up and said, “HO-WHUP!” Then she grinned. She will say, “I do it!” and leap in front of you to carry the groceries, set the table, pour the tea. She can make a monster face and run through the house frightening people or become a snake and slither across the floor nipping at your feet. About the only thing she lags on is dressing herself.

She’s also not the cleanest of children. To keep her from wetting the sheets, her mother dresses her in double diapers. The faint smell of ammonia hangs in the air of her room. It doesn’t bother Jennie, but it has bothered others. When Jennie was three, we put her in a preschool nursery. Before long we began getting hints that she wasn’t “fitting in.” Mimi would leave for her job about 7 a.m., and I’d take Jennie by the preschool on my way to work about an hour or so later. In that time Jennie might have a bathroom accident, get syrup in her long brown hair, or decide to draw on herself with Crayolas. Because mongoloids are prone to colds, she often had a runny nose—and the unladylike habit of wiping it on her dress. The head of the school finally wrote to us saying that it wasn’t much fun to pick up a child who had food in her hair and a dirty face and often needed a bath. We took Jennie out of the school.

When Jennie was five she started going to public school. Her world blossomed. She rides the bus and is in a class with others like herself. Her teachers have taken the businesslike attitude that Jennie is a student who is capable of learning. They’ve taught her to count to ten (sometimes she makes it to twenty) and to recite her body parts. She got an identification bracelet at school and learned her name and address. One day she came home and said, “Commere!” After we had all gathered around, she took a crayon and sheet of paper and very laboriously wrote out the letters: J-E-N-N-I-E. Then she put her hands on her hips and beamed.

We had wanted to keep one of her school group pictures, but Jennie didn’t cooperate. She wore the picture out. Jennie would proudly name each person in it over and over, starting with her teacher and tracing her fingers to the faces of each of her classmates. She recited the names for us, and, when no one listened anymore, she would sit down and name them for herself. She took the picture to bed with her and would find it first thing next morning. She carried it everywhere. Within a week the faces were blurred, and Jennie’s litany turned into a puzzled recital as she searched in vain for the missing persons.

TV is special to her. She likes ballgames, enjoys clapping and cheering with the fans, and she gets irritated if we try to change the channel when she’s watching football or baseball. Her favorite program is Emergency. “Woo-woo,” she calls it, circling her hand above her head to imitate the flashing red light. I don’t know how much she understands of the program, but she cries during the commercials.

Little things are what you remember about her. The way she can drop off to sleep suddenly as if she were shot, and the way she marches through the house on a cold morning trailing her sheet behind her like a queen on the way to her coronation. She’s not all sweetness. She can be grouchy in the morning; often she comes out of her room looking like the wrath. She can be stubborn, bossy, sulky. She even has her own profanity, which she resorts to use on special occasions: “Cock-ka-me!” We discourage her from saying it. Usually, though, Jennie is happy. She enjoys the little things—undressing to take a bath, eating watermelon, going for drives in the car, being left alone to explore the refrigerator. Most of all, she loves people. She likes to be around them, tickle them, talk to them, laugh with them. Give her a chance, and she’ll take off after the other kids and play with them for hours and hours just like she is one of them. Which, of course, she is.

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