hands of a surgeon, he slices through redundancy and mishmash to create a readable piece.
PROLOGUE
There was only one way to say it. Walter P. Maughn was an odd child. At birth he seemed fairly normal with dark straight hair, an unusually big head and a puffy, swollen face that bore more than a passing resemblance to a prize fighter on the losing end of ten rounds. The only thing that bordered on peculiar was his lack of the usual baby fat. Walter was definitely skinny, but otherwise a fairly nondescript baby with all the appropriate body parts.
As he matured, it was obvious he was going to be tall, in contrast to his parents, who were both compact and downright short. People speculated, in jest of course, as to Walter’s true lineage and it did not go unnoticed that the postman and milkman at the time, were both taller.
With his long gangly legs and arms, Walter seemed just slightly out of kilter. He wobbled and swayed when he walked, like the tires of an automobile with its front-end out of alignment. Like soaring birds, Walter’s arm span was enormous and appeared too long for his torso, eventually he developed a paunch, a beer belly, though Walter drank no beer.
Throughout his adolescence Walter was a loner. Not because this was his preference, but simply, he had trouble making friends. The neighborhood kids found him weird at best. He was not very bright or athletic and he had no interest in sports, not even as a spectator. For that matter, Walter wasn’t interested in much of anything. With no real hobbies or goals, he was a dull kid. To the delight of his tormentors, the neighborhood kids, he also had the bizarre habit of carrying on long conversations with himself. Thus, it was to no one’s surprise, that Walter grew up to be a social recluse.
As his age-group rode tumultuous waves of puberty, Walter seemed unaffected. Yes, he liked girls. They certainly were not as mean as boys, and their bodies were different and even interesting, but to explore this contrast was not a primary driving force in his life. Walter did not go on his first date till long after he graduated from high school, and then he dated only sporadically.
Walter graduated from high school near the bottom of his class, and there was never any question about him continuing his education. At age nineteen when all of his male acquaintances were preparing for Mormon Church missions, Walter thought about a mission as well, but he had difficulty memorizing, making it impossible for him to learn the missionary lesson plans. But the major obstacle that prevented Walter from being a missionary was his bishop never called him to go. This did not upset Walter very much. Deep down, he suspected he really wasn’t missionary material either.
Following high school he went to work for Quality Bakery as a janitor. Eventually, they let him pack boxes of bread and pastry for the delivery trucks and then after a few years, they made him an apprentice baker. In that position, he worked long hours. Walter would arrive at work at 3:00 a.m. and not leave till the trucks were loaded at four p.m. The tedious and seemingly unending hours did not bother Walter, after all, he had very little else to do.
It was at the bakery he met Kate. For a short time, Kate was a very bright ember in his dull existence. She worked as a clerk in the retail section of the bakery and was hired primarily due to the efforts of her mother, who was also employed by the bakery as a dough mixer. To be kind, Kate was just plain. Actually, she was fairly bright, but because of her conspicuous lack of physical appeal and skills, Kate appeared to be slow and dull. In reality, however, Kate was just homely, not dense.
She learned to make change efficiently and on occasion she even seemed to be an asset to the company. It was rumored some customers would buy extra bread because they felt sorry for Kate, a kind of alms for the ugly.
Walter and Kate had a brief courtship, no more than two months, then both decided they couldn’t do any better, so they proceeded to get married. Since they were of the Mormon faith, it was to be a Temple marriage and the wedding date would be within the month. Like all Mormon faithful, Walter and Kate were sealed together for time and eternity, which gave them very little working margin for error in the event they had made a mistake.
Kate never used any birth control. Not that she was unaware such devices existed, but she was simply too reticent, too shy, to seek out birth control. For a couple of years she didn’t think too much about it, but eventually she wondered why she never got pregnant. They hadn’t made love that much, but over those two years she and Walter had managed it several times.
Ultimately, Kate checked out some fertility books from the library and with the voracity of one studying her own medical problem, she learned about ovulation, sperm production and how to time intercourse. But even armed with these new weapons, Kate still didn’t get pregnant. By now, she desperately wanted a baby. Subconsciously, Kate felt a baby would compensate for Walter’s shallow contribution to their marriage. It became her obsession.
After repeatedly insisting, she eventually convinced Walter to go to her family doctor to be checked to be sure he was fertile. The doctor charged them ninety dollars, then promptly referred Walter to a urologist. The urologist did a semen analysis, which showed no sperm, not a solitary one. After performing a physical exam, the urologist commented to Walter that he had small testicles, as well as unusually long arms and legs. Following the physical exam, the urologist ordered two blood tests, a serum Follicle Stimulating Hormone and Luteinizing Hormone and as a final exam, performed a painless procedure called a buccal smear.
The reason for the tests, the doctor explained, was that he suspected Walter might have a congenital condition called Klinefelter’s syndrome. The FSH and LH were messenger hormones produced in the pituitary gland and were rather conspicuous indicators of testicular function. If the testicular factory had shut down, the pituitary gland would promptly secrete more and more of these hormones, trying to coax the testicles to bring their production up to normal. By measuring these circulating hormones in the blood, one could tell if the problem was primarily testicular or pituitary. The FSH and LH would be high in testicular failure.
The buccal smear, however, was the definitive test. It was designed to tell them if Walter had Klinefelter’s Syndrome. With Klinefelter’s Syndrome, the male has an extra X-chromosome. Normally, the male has 22 somatic chromosomes with X-X pairing, and an X-Y combination for the sex chromosome, making a total of 46 chromosomes. With Klinefelter’s, there is an extra X chromosome, leaving the patient with an XXY karyotype for the sex chromosomes. Ordinarily, the chromosomes come together as pairs in meiosis, a process designed to reduce the number of chromosomes by half in the germ cells. After pairing, the chromosomes eventually separate again and the cell divides. In Klinefelter’s Syndrome, the set of X, sex chromosomes, do not separate. Consequently, one germ cell gets XX paired sex chromosomes (instead of the normal single X) and the other germ cell gets none. When the XX germ cell, the egg, is fertilized by the Y germ cell, the sperm, then the resultant embryo is a male phenotype with XXY for his sex chromosomes. The genetic term for this failure to separate is non-disjunction.
The buccal smear was obtained by scraping the inside wall of Walter’s mouth with a tongue blade and placing the scrapings on a glass slide to be stained and processed later. If there are at least two X chromosomes, one can see Barr bodies in the cells. Barr bodies are distinctive clumps of nuclear material which appear at the periphery of the nucleus near the nuclear membrane and can be found in almost any cell in the body, including the mouth. As a consequence, females have Barr bodies in their cells normally (because their complement of sex chromosomes is XX), but men do not (sex chromosomes are XY), unless they have the rare genetic disease of Klinefelter’s Syndrome (sex chromosomes XXY).
Walter’s buccal smear came back positive for Barr bodies and the urologist seemed satisfied. He explained to Walter about Klinefelter’s Syndrome and emphasized that Walter would never be able to have children. Perhaps, the urologist suggested, he and Kate should consider options like adoption
