OLD AGE AT ELEVEN
A boy’s strange story
“He was the saddest child I've ever seen," his doctor said. He was brave, too, and because of this medical science may yet learn how to prevent other children from dying of old age
MACLEAN’S ASSOCIATE EDITOR
WATSONVILLE, CALIFORNIA, is a Pacific coast community of 14,000 people which lies about 100 miles south of San Francisco. Straddling the rich and fertile Pajaro Valley, it has long been known as “the frozen food locker” of the United States. The black earth spews forth everything in abundance—grapes, artichokes, almonds, apples, broccoli, green beans and berries of all kinds. It was in this lush setting that there recently unfolded one of the most bizarre medical case histories of modern times. It reached its tragic climax on February 6. 1962, with the death of a young local inhabitant, eleven-year-old Arthur “Tito" Balidoy, the son of a vegetable-packing plant employee.
Arthur Balidoy died of a rare disease known as progeria, which is the latin term meaning “premature old age." By some unknown error of nature, the aging process — or some phenomenon similar to it — takes place in the body of the progeriac with whirlwind speed. At eleven. Arthur died of “old age." He resembled a wizened old man: his skin was rough and wrinkled: only a few hairs remained on his head: the veins of his scalp protruded. His real teeth had long been replaced by false ones. Because of stiffness of the joints, he had walked with difficulty, like a stooped and aged man. His voice had been high and squeaky. He had suffered from arteriosclerosis— abnormal thickening and hardening of the arteries—a disease associated with aging. On two occasions. Arthur had had heart attacks. At the famous Stanford Medical Centre at Palo Alto. California, where Arthur was frequently a patient.
doctors stated that the immediate cause of his death was arrhymthia, a breakdown in heart rhythm that prevents the flow of blood.
Arthur Balidoy's spirit, like his body, aged prematurely. "At nine he was like a grumpy old man,” recalls his mother, a soft-spoken woman in her mid-thirties, with a Madonna-like face. He complained about the noise of younger children at play. He preferred adult pastimes, such as checkers and cards. He talked, a great deal, about God and religion. “He had an unusual sense of responsibility," says his mother. "Toward the end, he was handling the family budget, telling me how much I could spend on each item." Mrs. Balidoy never got used to the tragic and baffling ailment which plagued her son during most of his lifetime. She recalled the moment, at 6 p.m. on Tuesday, February 6, when he died in her arms. "It was an old man I cuddled but he was still my little boy," she said.
Mrs. Balidoy's bafflement is shared by medical scientists throughout the world who have been puzzling over the riddle of progeria since 1886. That was the year a Scottish physician. Dr. J. Hutchison, published in the Edinburgh Medical Journal the first recorded account of the disease. A survey of the international medical literature reveals that, since then, some thirty-four cases of progeria have been discovered in the United States. United Kingdom, France, Italy, Spain. Germany, The Netherlands. Poland and Egypt. I have read the medical reports of most of these cases and they show a remarkable similarity.
The family of th~ progeriac is completely unprepared for the tragedy which lies ahead. There is no unusual history of family illness. The other children in the family are healthy. The mother's pregnancy and d'livey are uneventful The progeriac child is born normal in every resp'ct He teethes, walks and talks on schedule. However, usually between the first and second year of life, the first disquieting symptoms appear. The mother takes him to the doctor because he's not growing or gaining weight as quickly as he should: furthermore, his hair is beginning to fall out. The doctor prescribes special diets, gives injections and attempts other types of therapy—but they don't work.
THE QUICK MARCH TO SENILITY . . .
From then on, the disease proceeds inexorably on its course to the end. The patient's size remains fixed at about the normal level for a six-year-old. The hair continues to thin out. leaving nothing but a w hispy fuzz on the head. Eyebrows and eyelashes vanish. Fingernails become brittle and dry and start to atrophy. Subcutaneous fat diminishes, gi\ing the skin an old. wizened appearance. The voice
becomes squeaky and piping. Because the face shrinks in size, the head appears to be abnormally large. The jaw recedes and the nose becomes beaklike. Décalcification of bones makes the arms and legs thin and spindly. At the age of five, arteriosclerosis sets in, making movements of hands, arms and legs difficult. Death, from heart disease, usually occurs between the ages of ten and twelve, although one Dutch patient survived until he was twenty-seven. After examining photographs of a dozen progeriacs, one might conclude that almost any two are identical twins, so strikingly similar is their appearance. Dr. Nathan B. Talbot of the Harvard Medical School, describes them as “a weird and unique picture of immaturity upon which has descended the blight of premature senility.”
...AND HOW A CHILD FACED IT
What causes progeria? This question is of intense interest to medical scientists because the answer might shed considerable light on the mysterious process of aging. But we are still far from finding a satisfactory explanation of the disease. There are almost as many opinions concerning the causes of progeria as there have been cases. Some years ago, a French investigator suggested that hereditary syphilis could be a factor. A Dutch doctor, W. A. Manschai concluded that progeria was “a form of pituitary dwarfism” while two French pediatricians, Drs. Varrot and Pironneau, stated that the condition resulted from malfunctioning of the adrenal glands. In the opinion of Dr. Ira M. Rosenthal of the University of Illinois, progeria was due to “an inborn error of metabolism,” while Dr. A. Mostafa of Cairo University, is convinced that the disease is a familial, inherited condition — a view which is rejected by most medical men. These divergent opinions lend weight to the statement by Drs. J. Thomson and F. O. Forfar of the Dundee Royal Infirmary that “we are no closer to understanding progeria than was Dr. H. Gilford.” Gilford was the doctor who gave progeria its name after publishing two complete accounts of the disease in a British medical journal sixty-five years ago.
The brief life and death of Arthur Balidoy of Watsonville. California, may provide new clues to the origin of progeria. During his final stays at the Stanford Medical Centre he uncomplainingly allowed himself to be studied. Dozens of urine, blood and breath samples were taken. He was injected with radioactive substances. An intelligent youngster, he knew what was happening. “The doctors can't help me but perhaps they'll be able to help somebody else." he told his mother, shortly before he died.
It was a life whose beginning held no hint of the CONTINUED ON PAGE 40
CONTINUED ON PAGE 40
OLD AGE AT ELEVEN
continued from page 12
Arthur Balidoy was a cheerful, bright and normal child — for the first 18 months of his life
prolonged nightmare which lay ahead. As a young girl his mother, of Spanish descent, moved from New Mexico to Watsonville. Here, she met and married Leandro Balidoy, a Philippino who worked in a vegetable-packing plant. “C ould the mixed marriage have been responsible for Arthur's condition?" she wondered aloud, as she recounted her family's history for me. The couple settled in a modest white frame house on Marchant Street, not far from the Pajaro River. Arthur was their second child. The other three— Danny, thirteen; Delia, eight; and Victor, two—are in good health.
The complete normalcy of Arthur's early life is noted in medical file No. 5-39-04 at the Stanford Medical Centre. Entries record that his parents were of normal weight, height and health. There was no family history of diabetes, TB or diseases of the heart or glands. The mother experienced a full nine-month pregnancy; delivery w'as without complications. Arthur was breast-fed for four months and then switched to formula, containing a vitamin supplement. He was a bright, cheerful child and the cause of no anxiety until he was one-and-ahalf years old. "He was underweight and even feeding him extra food didn't help," recalls his mother. She took him to a doctor—the first of many steps she w'as to take in seeking help for her child. The doctor suspected anemia and gave him iron shots. When they didn't help, she took him to another physician who administered injections of pituitary hormones. On her owm. she fed the child tonics and nostrums recommended by friends. She took Arthur to a succession of doctors in California—in Watsonville, El Centro and San Francisco. Once, she heard there was a skilled child specialist in Phoenix, Arizona, so she made the long trek there. In hope and sorrow she took her child to a faith healer. He laid his hands on the boy's head, and exhorted God to make him grow. Not only did the prayer go unanswered but. between the ages of two and four, the youngster gradually began to undergo disquieting changes in his appearance. These arc noted in the medical history at the Stanford Centre, which Arthur first visited in 1955:
"The patient is four years old. He’s a strange-looking boy with a birdlike face whose eyes give the appearance of relative enlargement because of the small underdeveloped lower jaw' . . . Skin shows areas of increased pigmentation . . . Hair is sparse . . . dentition, poor."
As Mrs. Balidoy recalls it. the Stanford doctors told her that the boy was a dwarf who would never grow up and would be dead by twelve. "1 found it so hard to accept that 1 kept on hoping for a miracle." she says. In the face of facts, it was a faith difficult to maintain. By the time Arthur was five, his hair was turning gray and lines appeared in his face. Veins protruded on his scalp, and. because ot
stiffness in his joints, he walked in a peculiar manner.
When Arthur became six, there were new trials to be faced. As a preschool youngster, it had been possible to protect him by keeping him at home. People in the neighborhood had become accustomed to his gradually changing appearance but he obviously shocked strangers w'ho saw him for the first time. Mrs. Balidoy recalls clearly the first time she took Arthur downtown in Watsonville. “When we came home we both cried. Everybody stared at us. Arthur was now' old enough to know that he was different from other children—some kind of a freak.” Another crisis was reached when the time came to enrol Arthur at Linscott public school. Henry Muceus, now the principal of Mac Quiddy School where Arthur spent the last year of his life in fifth grade, recalls the interview'. One of the teachers present subtly suggested that Arthur should attend the school for the handicapped. Heartbroken, Mrs. Balidoy took him to the special school where intelligence tests were administered. He passed with flying colors and returned to the Linscott school w here he was to distinguish himself as a scholar in the first four grades.
What was life like for the small boy who looked like a wizened old man? As he became older, he became more self-conscious about his gnomelike appearance. He was never to forget that he was different from other children. He had to go to a certain local store where special shoes were ordered for him from San Francisco. For health reasons he was not allowed to attend football games in the chill autumn weather. "I wish I were like other kids.” he complained. "Why do I have to be like this?” He puzzled over his illness. He would sneak into his mother's bedroom and stare at himself. for several minutes at a time, in her gilt-framed hand mirror. When he heard his mother coming, he w'ould hastily put the mirror aside. In his cousin's home he spent much of the time poring over a large medical textbook, as if he were trying to unravel the mystery of his malady. He was obviously hurt by the attention he attracted when he went to Sunday morning mass. “I don’t want to go any more," he told his mother. She took him to sec the parish priest. Father Meyer, who blessed him. and encouraged him to keep up his church at-
tendance. Arthur followed the priest's advice because he had a considerable interest in religion. He studied his Bible carefully and tried to engage his mother in religious discussions. At one point he chided her. "Gosh. Ma, you know practically nothing about religion.”
During the last few years he tired easily and had to restrict his activities. He continued to walk to and from the library to get books, but now' he punctuated the trips by sitting on steps to rest along the way. He would go to movies, once or twice a week, but this, too, was a great effort. “I get so tired," he said. “1 have nothing to live for. I wish I were dead.” His pastimes soon were limited to passive ones: watching TV. reading, playing cards and checkers, keeping the family budget.
In September of 1961, he entered the fifth grade of the Mac Quiddy Public School. His teacher was Dorothy Uren, a handsome, forthright woman who had been w'orking with children for thirty years. She sat him in the front row of the class where his view wouldn't be blocked by children who were giants by comparison. He was a serious student, with a particular aptitude for reading. As the autumn w'ore on Arthur's health grew' worse and he was frequently absent. "Wc would sometimes talk about him when he w'as away,” says Miss Uren. “The children showed a sensitive understanding of his predicament. One girl said, ‘Arthur must feel very sad inside. We must help make him happy.' " In the schoolyard, when some of the younger children would tease him w'ith epithets like “dwarf” and “midget.” his own classmates came to his rescue. One day in December, w'hen Arthur's health and spirits w'ere particularly low'. Mrs. Balidoy summoned Father Meyer who heard the boy’s confession. After the priest left. Arthur said, “1 don’t want to die yet. I want to sec Victor w'alk.”
There w'ere worse days ahead. Early on the morning of New Year's Day, 1962, Mrs. Balidoy found her son in pain and having difficulty with his breathing. She summoned Dr. O. Schmida, a young pediatrician whose office is located in Aptos, about ten miles away. Doctors at Stanford had familiarized Schmida with the nature of Arthur's illness. “I was dead tired after New Year's Eve," says Schmida, “but I quickly got dressed and sped to the Balidoy home. Arthur had suffered a heart attack and his life hung in the balance. I took him to the Watsonville hospital and. the next day, moved him to the Stanford Medical Centre.” He w'as to stay there for the next four-and-a-half weeks.
Arthur had been a patient at Stanford. from time to time, since he was four years old. Entries in his hospital file indicate that his disease symptoms had progressed rapidly since his last admission, a year earlier. It was noted that at eleven years of age his weight was only thirty-three pounds. The veins on his neck were distended; the muscles in his legs and arms were wasted and atrophied, limiting the movement ot his joints. But his intelligence was unaffected. The entry ends with. “The patient did not deny his illness and recognized reality.”
At Stanford. Arthur was treated for congestive heart failure and was the
object of an exhaustive series of investigations which might help unravel the riddle of progeria. “He was the saddest little boy I have ever seen,” recalls Dr. Norman Kretchmer, head of the department of pediatrics. “He had good reason to be sad." He rarely talked. "He had withdrawn from society,” says Kretchmer.
Now' began a long and seemingly endless series of tests, requiring the taking of various samples from his body and the administration of injections. Arthur was co-operative, although he was highly sensitive to the pain of the needles. "Dr. Bob Greenberg (Kretchmer's assistant) and 1 both flinched when it came time to give him the needles,” recalls Kretchmer. "The boy had suffered enough; we didn't want to add to his discomfort.”
Jeanne Bryan, a pretty girl from Flint. Michigan, w'as Arthur's favorite nurse. When the boy’s heart condition had improved somewhat, Dr. Kretchmer instructed Miss Bryan to devote herself to making him happy. Miss Bryan was in the pediatric ward through choice. A member of a large family herself, she derived deep satisfaction from ministering to children. “At first, Arthur's appearance horrified me,” Miss Bryan told me. “I couldn't even look at him. But after two weeks, he looked no different to me than any other patient.”
Miss Bryan applied all her skill, experience and energy to her new assignment. -She played cards and checkers with Arthur, helped him build model planes, watched TV with him. At times, she spent whole days wheeling him around the hospital grounds. He especially loved watching the fountains in the courtyard. He was fond of visiting the vending-machine room v here he could buy soft drinks and popsicles. Sometimes Miss Bryan pushed his chair into the offices of her friends, Nancy Lucas and Beth Sunshine, medical secretaries. “Sometimes Arthur would offer me a popsicle as a token of friendship.” recalls Miss Lucas, “but we would seldom say very much.” Even with Miss Bryan. whom he loved, he exchanged few words. “Arthur kept his feelings and emotions to himself.” she says.
According to the staff who tended him. these private feelings were deep and painful. His health was declining; he was depressed: and he ate poorly. He knew' that many patients and visitors were shocked by his grotesque appearance. He was weary of the endless procession of doctors who came to his room—Number C-375— to peer at him. Noise bothered him. He began declining Miss Bryan's invitations to play checkers or tour the hospital grounds. Dr. Ketchmcr couldn’t tempt him out of his gloom with a new model airplane. Mostly, he just wanted to lie in bed. concealed by a beige, patterned curtain. He now resigned himself to an early death, perhaps even welcomed the event. His father urged Arthur to get well and come home to a big birthday party. Arthur replied quietly, “I'm not going to get well. There will be no more birthdays."
On February 2. Arthur was discharged from the Stanford Medical Centre. “We couldn't help him and we wanted him to enjoy the solace and comfort of his home once again
before he died,” says Dr. Kretchmer, who estimated that Arthur had about a month to live. He was wrong. Time was to run out on the boy in five days.
Mrs. Balidoy vividly recalls those last poignant days. Fatigued, Arthur spent most of his time in bed, occar sionally wandering into the living room to sit on the striped sofa and watch TV. He forbade his mother to carry him. “He didn't want me to feel sorry for him." she said. He continued to spend long periods gazing at himself in his mother’s mirror. He frequently complained to his mother about the other children: "Please tell them not to be so wild and noisy," he said. "1 need my sleep." He insisted on being taken to Castroville, ten miles away, to visit Adalia Isidro, one of his mother's friends of whom he was very fond. He didn't object to being carried to and from the car.
His mother sensed a difference in his behavior early on February 6—the last day of his life. Arthur seemed to be acutely aware of the passage of time. He listened to the clock ticking away the seconds; stared at the calendar. He repeatedly gazed at the brightly colored reprint of Christ which hung on the living-room wall beside the sofa. In the afternoon he begged his mother to go to the store and buy him an orange soft drink. “But hurry!” he said. That was the day, too, that his little brother Victor took his first faltering steps by himself.
That evening, the Balidoy family had supper at the usual time, 5.30 p.m. Mrs. Balidoy customarily spent an hour, after eating, speaking to relatives and friends on the telephone. On this day, Arthur made a strange request. “Don't talk on the phone tonight.” he said. "After supper let's sit on the sofa and talk." Mrs. Balidoy agreed, and started to wash the dishes in the kitchen, occasionally looking out at the hlack sky and the pouring rain. She heard a cry and rushed to Arthur's bedroom where she found him dying. “I talked to him but he never heard me,” she said. Beside the boy, on the bed. lay the gilt-framed mirror.
Dr. Schmida was summoned and pronounced the boy dead. His body was immediately removed to the Stanford Medical Centre for an autopsy. It will probably take two years for Stanford's scientists to complete their investigation of the materials provided by the Balidoy case. Hopes are high for at least a partial breakthrough in our understanding of progeria. "Some of my friends criticized me for allowing the autopsy,” says Mrs. Balidoy. “1 don't agree with them. Arthur didn't have a chance. Maybe some other kid will. I know that’s the way Arthur felt. He had so much sense."
It was still raining the next day when Arthur was buried in the Valley Catholic Cemetery. After the funeral Father Meyer came over to comfort the bereaved mother. "It’s a blessing that his suffering is over," he said. Mrs. Balidoy later explained to me. "1 didn't feel that way. In spite of everything. I could only see Arthur as my iittle boy. He was sweet: he was likable. It's like Dr. Kretchmer told me when Arthur left the hospital for the last time. 'I never liked a boy so much!' " ★