Thursday, August 9, 2018

The Disease That Always Killed: By GEORGE H. WHIPPLE, M.D., as told to J.D.RATCHLIFF


Courtesy: Medical Miracles, from Readers Digest 1981. November.
From the frontiers of science and the far horizons of personal courage, these stories of medical triumphs and miracles will reaffirm your faith in the awesome powers of the human spirit. Dramatic victories and human triumphs.
Selected and edited by the editors of Readers Digest
It was a pure horror of a disease.  It started innocently enough.  Victims noted that everyday chores tired them more than usual.  Soon they were finding difficulty rising from a chair.  Their skin took on a waxy, yellowish cast; tongues became flaming red and sore.  Some sufferers became addle-headed; a few went on to paralysis.
Pernicious anemia was a leisurely killer.  It might take two to five years to claim its victims.  But, eventually, all died.  When they finally expired, blood counts were often down from a normal five millions red cells per cubic millimeter of blood to a watery 500,000—not enough to carry the needed amount of oxygen around the body.  Slowly, tissues and organs were asphyxiated.
Physicians fought futile battles against this doom.  A mysterious poison was killing some thought the red cells off.  Others made a better guess; that the bone marrow, which manufactures red cells, had, for unknown reasons, forgotten how to produce the trillion new cells needed each day.  Still others believed that the spleen played some role in the disease and that its surgical removal would help.  Such patients did well for a while, and then began slipping again.  Whatever the treatment, the end result was always the same: death.
The conquest of this disease was, I believe, one of the first instances in medical history in which a universally fatal illness was converted into a minor inconvenience.  Today, tens of thousands of people live normally with pernicious anemia, scarcely aware that they have it.  Here is the story of how this came about.
Both my grandfather and father had been country doctors in New Hampshire, and I was tempted to follow in their footsteps.  But, while studying at Johns Hopkins, I became interested in pathology; the study of disease itself.  Blood in general, and the anemias in particular, fascinated me almost from the start.  I studied the anemia, which accompanies hookworm disease, and observed tropical anemia in Panama in 1907 while the Panama Canal was under construction.  Opportunity to get at the problem in a concentrated way came in 1914, when I was invited to San Francisco to head the new Hooper Foundation, the research arm of the University of California’s Medical School.
The anemia, of course, is a wide spectrum of diseases, and even in those days most of them were curable.  But pernicious anemia was different.  The normal lifespan of a red blood cell is 120 days.  As each dies, it must be replaced—ten million are needed each second.  In pernicious anemia the new cells were not produced in adequate number.  It seemed obvious to me that the only source of building material for red cells was food, and I blocked out a study along these lines.
The first step was to produce artificial anemia in dogs.  Form neck artery I intermittently withdrew blood until the level of hemoglobin—the vital red coloring matter in blood cells—was 40 to 50 percent below normal.  [The procedure, incidentally, did not seem to bother the animals at all.]  Then various diets were tried to see which, if any, hastened production of new red cells.
At this point a compact, blond young woman appeared in my lab and demanded a job.  German-born Frieda Robscheit—later she would Americanize her name to Robbins—was absolutely determined.  ‘I am going to work with you, whether you like it or not,” she said.  I hired her, and she turned out to be one of the finest collaborators any researcher can ever had.
Our first problem was to devise a basal diet for our dogs, one that would provide essential nourishment but would be the poorest possible for building blood.  Then we would supplement this diet with various foods to see which would hasten production of red cells.
Months, years, passed.  Thousands of red-cells counts were made as the work crawled methodically along.  Scores of foods were tried as blood builders—milk, eggs, lettuce, Naturally, we also tried a variety of meats.  A slaughterhouse near the lab furnished whatever we required in this line; spleen, pancreas, bone marrow, brains, sweetbreads.  And in time an extraordinary performer emerged.  It was liver in as little as two weeks liver restored low-red-cell dog blood to normal.  We found it difficult to believe that any food could act with such speed and efficiency.
But our work was by no means completed.  If liver was to be the hero in conquering anemia, the fact had to be nailed solidly down—which meant trials on many more dogs, with time of recovery exactly measured.
In 1921, I moved to Rochester, New York, to become dean of a medical school then being started.  As soon as the new laboratory was ready, Frieda Robbins came east by train, accompanied by our kennel.  Finally, in 1925, we felt confident enough to report: “Liver feeding remains the most potent factor for the sustained production of hemoglobin and red cells.”  Now, somebody else had to extend our findings to treatment of pernicious anemia in human.
Dr. George R. Minot of Hayward was one of the very finest in Boston medicine.  Tall, rail-thin, fastidious, reserved, he was totally dedicated to his patients.  When he heard of our work on dogs he determined to try liver on his pernicious anemia cases.
Not at all well himself—he suffered from diabetes—he enlisted the help of a younger colleague, dr. William P.murphy, then at Peter Brigham Hospital.  The two almost literally stuffed their patients with all the liver they could get down protesting gullets.  Sometimes Murphy mixed ground raw liver with orange juice and poured this in.  Patients in a coma got ground liver via stomach tube.
The response was almost beyond belief.  Within a few days, patients looked better, felt well, and their red-cell counts were climbing.  May be these people weren’t doomed after all!
On May 4, 1926, Minot, in his flat New England voice, read a paper before his peers at the annual meeting of the Association of American Physicians.  He reported that he and Bill Murphy had stuffed 45 patients with liver.  Some had had blood counts lower than on million, now all but one had near normal counts.  That one was an elderly lady who so despised liver that she said she preferred death to eating it.  Most of the other patients were up and about—no longer breathless, no longer weary.  Most were back at normal occupations.  Minot’s report was acclaimed by a standing ovation.
Ata hospital in Boston next morning, doctors were preparing to give an elderly man a transfusion—the only thing that had kept him barely alive for several years.  “Why not just let me die?” the weary sufferer asked.  A young doctor burst in.  “Did you see the morning paper?” he asked.  A news report had summarized Minot’s paper.  Liver diet was started immediately, and within a week the patient had new life in his limbs.
Such events were soon being enacted all over the world.  A year later, Minot and Murphy reported on 105 patients.  There had been three deaths: one in an auto accident, one from cerebral thrombosis, one from unknown causes—but none from pernicious anemia.
In 1934, Minot, Murphy and I received identical cablegrams from Stockholm.  We had been accorded medicine’s highest honor, the Nobel Prize.
A way had been found to rescue the doomed—but the story was still by no means complete.  Even at outset it was clear that it wasn’t liver that was working such wonders, but some elusive X substance in liver.  The problem was to get rid of useless components and concentrate on the substance.  Dr. Edwin J. Cohn, the gifted Howard chemist, took this job upon himself.  Soon he had an extract, one tablespoon of which was the equivalent of half a pound of liver.  Next came an injectable liver extract 400,000 times as potent as the mother stuff; a short every two to four weeks was sufficient to keep blood normal.
The search for the X substance came to an end at last in 1948, with the cornering of Vitamin B12.  An invisibly small amount of it—a millionth of a gram a day—was sufficient to control a disease once totally deadly.
Pernicious anemia, which had probably plagued man from the beginning, at last could be rendered harmless.

New Hope for Retarded Children: By SARA D. STUTZ


Courtesy: Medical Miracles, from Readers Digest 1981. November.
From the frontiers of science and the far horizons of personal courage, these stories of medical triumphs and miracles will reaffirm your faith in the awesome powers of the human spirit. Dramatic victories and human triumphs.
Selected and edited by the editors of Readers Digest
Jordy is mongoloid.  When he was born, the pediatrician suggested that his parents put him in an institution.  Fortunately for Jordy, his parents ignored the doctor’s advice and took him home.  Within a few months they enrolled him in the Infant Development Program of the Exceptional Children’s Foundation in Los Angeles.  At three, he’s functioning so well that he has been accepted in a pre-school for normal children.
Linda, normal at birth, suffered massive brain injury in an auto accident when she was sent to Lanterman State Hospital in Pomona, California, where her parents expected her to be a crib case for the rest of her life.  But now, through special sensory motor training, Linda is learning to walk and talk again.  She’ll never return to normal, but she’ll soon function in a manner her family can manage at home.
At nine months, Christy was hospitalized with malnutrition and other evidences of parental neglect.  She was unresponsive and slow for her age.  Now, enrolled in the developmentally delayed infant education project at the Nisonger center in Columbus, Ohio, she is, at 13 months, feeding herself, crawling, and trying to talk.  It looks as if Christy is going to catch up.

Jordy, Linda and Christy aren’t miracle babies.  They are typical of the youngsters being served by infant intervention programs, a new and highly promising concept in education.  “With early intervention, many developmentally delayed children may be entered in regular classes of helped so that their disabilities require less extensive special services,” said James J. Gallagher, former associate commissioner of education for the handicapped, at HEW.
There are an estimated 2.2 million retarded persons in the United States. Dr. GeorgeTharjan, professor of physiology at ULCA, testifying before President’s Committee on Mental Retardation, estimated that as many as 50 percent might have been classes as “normal,” had they had the benefit of early training.  Not only could they be leading more satisfying lives, but also society could be spared the expense of their lifetime institutional care.  The cost of such care for a person from age six can be $300,000 to $1.5 million.
Babies learn from experience.  If they can take in what’s happening around them, and if their surroundings contain an average amount of stimulation, they develop to their full potential.  But, if their ability to absorb their environment is limited, they don’t get the experiences they need for mental development.
“Any infant suffers is his original capacity to inquire, to seek, to explore, is stifled.  Sterility of the early childhood environment, especially the absence of daily conversational exchange with the mother and others in contact with the infant, seems to impose a permanent limitation on intelligence,” noted John W. Kidd, former president of the Council of Exceptional Children.
When a profoundly retarded infant is put in a crib and given only the necessary custodial care, as was common practice until recently, he merely lies there, explains Clara Lee Edgar, the physiologist who developed a training program a lanterman State Hospital.  He has no way of making anything happens.  He cannot learn anything.
But if that same child is taken out of the crib and strapped t a scooter board on wheels with his toes hanging down on the floor, he can, with the slightest amount of wiggling, make the board move.  He seems to say to himself, “Hey, I can go somewhere.”  In subsequent periods on the board he learns to scoot across the room.  Eventually, he begins to hold his head up while doing it and even use his arms and hands to guide him.  He’s having experiences that will increase his intelligence.
Now, with the new intervention programs, which have sprung up in the past 15 years, babies with developmental delays, are being helped to have the experiences they need to make mental and physical progress.  Most programs are open to any developmentally delayed baby—a preemie, the baby having difficulty relating to people, the child of overanxious parents, the slow walker—not just children with known physical of mental impairment.
Babies enter programs through a verity of channels.  Some, usually low-birth-weight preemies or babies who have experienced unusual difficulties at birth, become part of a program while in the newborn nursery.  Many are referred to programs by their pediatricians or public-health nurses because of obvious medical conditions such as Down’s syndrome [mongolism], hydrocephaly [enlargement of the brain because of an abnormal drainage of cerebral fluid], microcephaly [abnormally small skull] of spina bifida [open spine].
Babies who have developmental problems evident at brith may cry all the time or they may be very ‘good.’  They might not cry or fuss for attention for a verity of reasons.  Without being neglectful, a mother would leave such a child in the crib all day except for feeding and changing him.  Yet this is the baby who most needs an environment that provides a maximum of social and sensory experience.
Directors of infant programs usually request mothers to bring their babies once a week to a center where special equipment is available, and where trained personnel can show them how to teach their babies.  Group activities are offered when babies are old enough to work on the self-help and language skills necessary for entry into pre-school.
At the Early Childhood Intervention Center in Dayton, Ohio, I followed a group of seven Mongoloid youngsters, one and-a-half to three years old, through a morning’s activities that would be almost unbelievable to the person conditioned the think of Down’s Syndrome as a totally incapacitation handicap.  After a period of free play with specially chosen toys to improve coordination, the children sang songs that helped them to identify their own names.  Then they divided up, one group going to draw with crayons and play simple ball games while the other had a lesson in identifying colors and matching shapes.  At snack time, all the children fed themselves.  In half-hour discussion periods, the mothers were told what the children would be learning next and how to reinforce it at home.
Rural areas, as well as cities, can have such special services.  In 1969, the Office of Special Education and Rehabilitation, part of the Department of Education, provided fund to develop a model rural program in Portave, Wisconsin.  “We expected the first to build a special school and bring in children for classes,” said David Shearer, director of the project.  “But we soon rejected that.  The area we’re responsible forcovers 3600 square miles of farms and villages.  Since youngsters with prblems may live 100 miles apart, we use ‘home trainers’ instead.”
The home trainers—women who either have had instruction in special education or are paraprofessionals—come once a week for an hour-and-a-half lesson.  They show parents how to conduct similar lessons the other days of the week, and leave any equipment that is needed.  Results?  The average child in the Portage project gained 13 months in an eight-month period.
Lanterman State Hospital at Pomona, California, is showing that there is no level at which children are ‘hopeless.’  Severely retarded youngsters—ones who are often crib cases for life—are trained so well that they can often return to their families.
Therapists are talking severely retarded children through the developmental stages that the normal child experiences.  For a verity of reasons, the retarded youngster cannot effectively use his body to deal with the world around him.  Research has shown that by improving his balance and other sensory motor skills the child can be helped toward more normal behavior.  I watched the most advanced group go to the dining room for lunch, where one bright-eyed little girl carefully set the table and served the rolls to her classmates.  It was hard to imagine that she had been a crib case.
Will the community be ready to accept these children?  The teachers and parents I talked with said yes, if the public is given adequate information about developmental problems.  I heartily agree.
My youngest child, Eric, is afflicted with Down’s syndrome.  He has been a much-loved member of the family ever since he was born.  Friends and acquaintances with which we have openly discussed his condition are interested in his development and are rooting for him in a truly heartwarming way.
Several years ago, when Eric was hot quite three, I took him on one of our routine trips to the supermarket.  I held his hands as he walked into the store for the first time.  As we passed through the turnstile, we were startled by the sound of loud applause.  The checkers were clapping for him and his small chest swelled with pride.
At that moment thought of what might have been crossed my mind.  Even with early stimulation and training, Eric is slower than the ‘average’ mongoloid child.   He could easily be a hopeless, unreasoning hospital patient instead of a lively happy little boy embarking now on a program of special public education.  Where he was born and the kind of advice we were given have made that much difference in his life.

The Miracle of Muscle: By J.D.RATCLIFF


Courtesy: Medical Miracles, from Readers Digest 1981. November.
From the frontiers of science and the far horizons of personal courage, these stories of medical triumphs and miracles will reaffirm your faith in the awesome powers of the human spirit. Dramatic victories and human triumphs.
Selected and edited by the editors of Readers Digest
Events that take place when a dog wags its tail, a baby toddles across the floor or you scratch your nose with your forefinger dwarf in complexity the workings of a hydrogen bomb.  All are examples of muscular contraction—so commonplace we pay it no heed, yet so mysterious that it has baffled the most gifted scientists.
More than half of the human body is muscle—“the most remarkable stuff in nature’s curiosity shop,” as one scientist had said.  From birth to death, muscles play a critical role in everything we do.  They propel us into the world in the first place—when the womb suddenly empties itself.  They provide nearly all our internal heat.  They push food along the digestive tract, suck air into lungs, and squeeze tears from lachrymal glands.  And finis is written for fails and us when the heart muscle, after beating two and half billion times in a 70-year life span, flatters.
We speak of “muscles of iron.”  Yet the working or contractile element in muscle is a soft jelly.  How this jelly contracts to lift 1000 times its own weight is one of the supreme miracles of the universe.  An elaborate series of chemical and electrical events, which would require hours of days to duplicate in the laboratory, occurs almost instantaneously when a muscle contract—the twitch of an eyelids, for example.
There are three types of muscle in the human body.  One is the striated muscle, which looks like a sheaf of hair-sized filaments.  These are the muscles of motion—that propel us when we walk, that lift a forkful of food, that nod our heads.  Next come the “smooth” muscles.  These control such involuntary actions as the churning of intestines during digestion, the dilation of the pupil.  A third type is found in the heart.  In structure, it is midway between the other two.  All types of muscle are startlingly efficient machines for converting chemical energy [food] into mechanical energy [work]. 
Hundreds of books and scientific papers have been written on muscles—but none explains fully the process by which muscles contract, how you wiggle a tow.  “It is essential that we understand these puzzles,” said Dr. Albert Szent-Gyorgyi, Nobel Prize winner and scientific director of the National Faundation for Cancer Research.  “In one was or another, failure of muscles to contract porperly accounts for the vast majority of deaths—from heart failure, high blood pressure and other diseases.”
It is the riddles such as these that Dr. Szent-Gyorgyi and his co-workers went years seeking to solve.  Fiber by fiber and molecule by molecule they have taken muscles apart, and then fitted them together again trying to discover the mechanics of muscular action.
Research suggests that muscle is never thoroughly relaxed.  Because it is partially tensed—something like a taut spring—it is ready for almost instantaneous action when an electrical message from the brain orders it to contract.
Twp proteins, Dr. Szent-Gyorgye has found, are mainly responsible for the contraction—actin and myosin.  Alone, neither is contractile.  But when an electrical impulse from the brain orders the batting of an eye, or the wrinkling of a nose, actin and myosin combine to form actomyosin, which is contractile.
In a sense of actomyosin is the muscular ‘engine.’  Its fuel is a remarkable chemical substance, adenosine triphosphate—ATP for short.  ATP is a submicroscopic bombshell of energy.  Actomyosin fibers contract violently on contact with it.  At death, ATP disintegrates rapidly and muscles become hard, inelastic.  This is rigor mortis.
To demonstrate the critical importance of ATP, Dr. Szent Gyorgyi stored rabbit muscles, which had been washed free of the chemical, in freezers for periods up to a year.  Taken out, thawed and touched with ATP, the hard, brittle muscles spring to life; once again they show the elasticity they had when they propelled a rabbit in its hopping gait.
Creating ‘living’ tissue in the laboratory has been something of a scientific will-o-the-wisp.  But muscle researchers have come close to it.  Dr. Szent-Gyorgyi mixed jelly-like actin with jelly-like myosin.  Then, with the aid of a tiny glass nozzle, he spun this material into gossamer filaments.  Watching through a microscope, he added a droplet of ATP to the fluid surrounding the filament.  There was violent contraction!  He had created artificially perhaps the most fundamental of all life processes—muscular contraction.  “It was, “he says, “the most exciting moment of my life.”
Where are such experiments leading?  They may open a new frontier of attack on some of mankind’s greatest ills.  There is no logical reason why the human heart should beat two billions times during a lifetime, the suddenly fail.  Almost nothing is known about the cruel crippling of muscular dystrophy; or why muscles in blood-vessel walls should tighten to produce the misery of hypertension; of why the uterine muscles of many women become crumply contracted each month to cause painful distress.  Once the mechanics of muscular action are thoroughly understood, we may be at the beginning of a new biology, a new medicine.
Meanwhile, there is a great deal all of us can do to keep our muscles functioning well.  First, they must be promptly fed.  Generally speaking, the average diet includes all the protein needed for muscle repair, and all the carbohydrate required for muscle fuel.  But muscles can starve through lack of exercise—witness hospital patients who eat perfectly balanced meals and get out of the bed too weak to walk.  Reason: muscles are nourished by thousands of miles of hairline capillaries, which transport food and carry off wastes.  In the sedentary adult, large numbers of these capillaries are collapsed, out of business, nearly all the time.  Exercise alone can open them up and provide better muscle nutrition.
A number of studies have shown the beneficial results of exercise on the heart muscle.  A study in London bus drivers, for example, showed that drivers, who sat all day, had far more heart trouble than conductors, who were constantly on the move.  Similar checks have shown office workers more prone to heart disease than postal workers.
Often, muscles become unduly fatigued when required to work at too fast a rate.  One housewife rushes at her chores and is worn out by noon, while her more leisurely sister accomplishes just as much and finishes the day still fresh.  A series of treadmill experiments tells why.  In one, subjects were paced at 140 steps a minute.  Gradually speed was increased to 280.  At the doubled rate, oxygen requirements of muscles increased eightfold supplying such demands are fatiguing in it.  All work and exercise should be paced to get the most of our muscles.
Like all other body organs and tissues, muscles must have rest.  Millions of people sleep the traditional eight hours, and then get up exhausted.  The most likely explanation: one set of muscles has been cramped, tensed all night—wearing out the rest of the body.  The best way to avoid this is to become acquainted with your own muscles.
Lie quietly in bed, legs straight, and arms at the side.  Contract one set of muscles at a time, and consciously relax them.  Start with the feet and work upward.  In a matter of minutes, real relaxation can be achieved—leading to more restful sleep.
Overburdened or weakened muscles sometimes require additional support.  This is particularly true of the back muscles, whose chief function is to hold the body erect.  Often, low-back pain can be traced to weakness of these muscles. Every physician has his favorite set of exercises to provide new strength.  But until exercises are well under way, extra support is sometimes necessary.  A strong extra-wide belt is useful for this purpose.
It is best, of course, not to wait until muscles are weakened before giving them the care and consideration they deserve.  For, to a great degree, we are what our muscles make us—sick or well, vigorous or droopy, alive or dead.