ã 2002 Elisabeth Thomas-Matej, posted with permission.
Summary: Elisabeth Thomas-Matej is a freelance medical writer who lives in the San Francisco Bay Area. In this article, she reveals the correct diagnosis and official cause of death for acclaimed actor Michael Dunn, explores how his condition influenced his life and death, and overturns misconceptions about his physical abilities. She also discusses treatment options during Dunn’s time and presents a first-hand account describing a modern course of treatment.
Michael Dunn was born Gary Neil Miller to Jewell Miller (née Hilly) and Fred Miller, on October 20, 1934. His press kit biography from 1964 says he took his stage name to differentiate himself from another actor named Gary Miller. He died in his sleep and was found the morning of August 30, 1973. This article is dedicated to his memory.
What’s in a Diagnosis?
A Medical Biography of Michael Dunn
Actor Michael Dunn (1934-1973) had an average head on a tiny body with barrel chest and short, angular limbs, limped badly with a cane, and wheezed. He stood 3' 10" and weighed about 78 pounds (117 cm, 35 kg). He smoked1 and is described as a heavy drinker; and his internal organs are said to have been too large for his chest—a condition that reportedly was predicted to result in premature death, before age 40.2 He died two months before his 39th birthday. His obituary in The New York Times states that the cause of death was not disclosed.3
Articles on paper, microfilm, and the Internet describe a genius who triumphed over the laws of physics and time. He is said to have played baseball and football in childhood “like a normal sized kid.”4 He is said to have been a concert pianist at age 15—although that is the age at which he supposedly entered the University of Michigan as a full-time academic student (not a music student).5 After college graduation, he reportedly “found employment as a sports reporter, a hotel detective, and a missionary.”6 He also “mastered judo.”7 A print biography asserts: “Despite almost constant pain…he never gave into his suffering…He taught himself to drive a car, ice skate, swim, fly a plane, and skydive.”8 Some sources say he had to give up piano in childhood, because of crippling elbow problems; others say he “overcame” all pain and limitations. At least two sources deny that he was a dwarf, although disproportionate short stature is the definition of dwarfism.
Dunn is widely alleged to have suffered from achondroplasia,9 or “progressive achondroplasia.”7 The term "achondroplasia” formerly was used to describe almost all types of disproportionate short stature. However, more than a hundred different types of dwarfism are recognized at present and are classified as skeletal dysplasias—that is, growth disorders that affect the entire skeleton.10
Achondroplasia now refers to disproportionate short stature (dwarfism) with a normal length torso and very short limbs. Features are apparent at birth and include an average or large head with a prominent forehead and chin but flat nasal bridge. Thighs and upper arms are especially short, hands and feet are broad and stubby, and the normal length torso tends toward swayback. Breathing passages may be obstructed in infancy, and pinched nerves in the spine can cause problems in later life; but intelligence and life expectancy usually are normal.11 The gene for achondroplasia was found in 1994 and is called fibroblast growth factor receptor 3 (FGFR3).12,13,14
Dust bowl diagnosis
This description clearly does not fit Dunn. Photos and films show a barrel-chested, lean little man with arms and legs that are almost gangly compared to his extremely short, sharply swaybacked torso. His head is about average size, but the cranial vault is low with a sloping, not bulging forehead and somewhat prominent chin. His face looks taut, especially around the mouth. He appears to have no neck.
His long hands seem skewed at the wrist, with spidery fingers and thumbs that lie close to the palms. His elbows don’t straighten out, his wrists and fingers barely do so. He often stands braced in a crouch. His feet look too long for his legs with ankles that roll inward (pronation). On film, at age 31, his gait is a stiff, lopsided waddle characteristic of severe hip joint disorder with uneven leg length. A high-pitched wheeze is audible with laughter.
Dunn did not wear glasses on camera but reportedly was extremely nearsighted and suffered repeated corneal abrasions from wearing hard contact lenses—the only type available at the time. The same source reports he had scoliosis, an abnormal sideways curve of the spine. He apparently sought a consultation for hip replacement, late in his short life. This was a new procedure in the late 1960’s, with a very long recovery. But he was told he was medically too fragile to survive the surgery.15
Internet rumor says Dunn appeared normal at birth. But his Times obituary says: “He did not develop normally, with both hips dislocated, which made walking a constant pain throughout his life…By the time he was 4, he knew he would be a dwarf, and when he was 5 the disease was diagnosed. It was a rare form of nonhereditary dwarfism believed to be caused by a chemical imbalance during gestation.”3
Dunn’s press package biography, dated August, 1964, says he had congenital, progressive chondrodystrophy—a nonspecific diagnosis.16 The designation of congenital suggests some clues may have been evident at birth, at least to an astute observer; yet, the diagnosis reportedly was not made till age five.
However, the reports are subject to interpretation. Dunn is quoted in TV Guide: “ ‘By the time I was four,’ Michael says, ‘I realized I was a dwarf.’ ”7 That statement suggests age four was the first time Dunn grasped his situation—not the first time a doctor diagnosed disordered growth. The disparity also could be explained by circumstances: The biography says Dunn was born in Shattuck, Oklahoma in 1934, and that the family moved to Dearborn, Michigan, when he was four. No doctor practicing in the “dust bowl” of Oklahoma in 1934 could claim expertise in skeletal dysplasias—which the renown Steven E. Kopits, MD, identified as a subspecialty of orthopedics, three decades later. According to Little People of America, only five clinics specialize in skeletal dysplasias now, in the U.S. It seems likely that Dunn’s parents would have pursued a correct diagnosis after moving East, at a big city hospital in Detroit, Cleveland, or Chicago.
Dunn sometimes is said to have suffered from the same disease as French artist Henri de Toulouse-Lautrec (1864-1901) and the German-American electrical engineering wizard Charles Proteus Steinmetz (1865-1923).2 What happens when we look to them for clues?
Toulouse-Lautrec was about a foot taller than Dunn. His height is often cited as 1.5 m (150 cm) or 4.5 feet (4' 6")—but 1.5 m is 4.9 feet (4' 11"). Since the French invented the metric system and adopted it in 1799 (“for all the people, for all time”), his metric height probably is accurate.17 He usually is described as having a normal body with very short legs, but photos show short arms and legs. The shoulders droop and accentuate a long neck. His head looks too large but invariably is obscured by a hat and beard. His face is unusual, with downward-sloping eyes, a big nose, very full lips, and a receding chin. Toulouse-Lautrec suffered two leg breaks from two minor falls in adolescence—at ages 13½ (left femur) and 14¾ (right femur), when he should already have attained 90% of his adult height.18,19 At least one of the fractures took a year to heal.20 He is theorized to have had an extremely rare skeletal dysplasia, pyknodysostosis (also spelled pycnodysostosis), which includes dense, brittle bones that heal slowly. He died before his 37th birthday, allegedly from severe alcoholism and syphilis.21
A photo of Steinmetz leaning on a table, with an oblique camera angle, is posted at the German Corner website.22
So much for the parallels between Dunn and these other short men of fame. A 1966 interview with Dunn in a news magazine for elementary school students provides a clue about how this rumor may have started: “He believes he could play Toulouse-Lautrec, the artist, and Charles Proteus Steinmetz, the scientist, as well as jesters and fools in Shakespearean plays.”25
Then, what is progressive chondrodystrophy? Dunn is quoted in Life magazine: “I've always lived with constant pain, so that wasn't a factor in whether I made a life for myself or not. I could have copped out, lived with my parents and pulled the dwarf bit.”9 What causes the constant pain? Can anything be done for it? Were Dunn’s suffering and early death inevitable?
Progressive chondrodystrophy (“worsening cartilage bad-nourishment”) is a descriptive term no longer in use. Skeletal dysplasias encompass a dizzying array of syndromes with overlapping symptoms and a plethora of synonyms. Syndromes are renamed and reclassified as more is learned through microbiology and genetics research. But for most syndromes, classification is largely based on x-ray findings (appearance) during childhood.
The most accurate diagnosis available for Dunn is spondyloepiphyseal dysplasia (SED), type unclassified—literally, “spine, ends-of-long-bones bad formation.”26 This is also merely a descriptive phrase, but more is known: SED encompasses a group of skeletal dysplasias caused by a spectrum of closely related genetic mutations—only some of which have been identified precisely. These mutations occur by accident during conception—a piece of bad luck. They disrupt the production of hyaline cartilage. Hyaline cartilage caps the vertebrae and the long bones (bones of the spinal column and limbs) and normally creates perfectly smooth bearing surfaces for each joint.
A precise diagnosis of known subtypes of SED must be made by x-ray in childhood, before the bones have finished growing, and before joints are distorted by advancing arthritis. Dunn apparently found expert care only after puberty, when important clues to his particular subtype of SED no longer showed up on x-ray.
Since the exact subtype of Dunn’s SED is not known, its incidence cannot be stated. However, SED congenita (SEDc) occurs in about one in 100,000 live births as a spontaneous new mutation. This makes it much less common than achondroplasia—one in 26,000 to one in 40,000 births.27 SED affects males and females in equal numbers. The defect has a 50% chance of being passed on, if the affected person has children.
Defective cartilage at the ends of the bones inhibits the growth plates, or epiphyses (singular: epiphysis). The epiphysis is the area at the end of a bone that forms the joint—and it is the area where most lengthwise growth takes place. Normally, bones grow longer through a remodeling process: As a child grows, the most interior portion of the joint cartilage is converted into bone, and new cartilage is formed on the surface to maintain smooth joints. The old joint margins (edges) are resorbed, so that the overall shape of the joint is preserved as growth continues.
SED disrupts this process and stunts lengthwise growth. But the bones continue to grow in width; and the head, pelvis, hands and feet also grow to more normal size. The vertebrae wind up squat, often with deformities—so the spine is extremely short (platyspondyly). Spinal deformities may include abnormal curves in any direction, which can cause chest wall deformities.
Adults with SED have adult bone density and muscle mass and are much heavier than lean children of the same height. A lean six-year-old boy of 3' 10" is about 44 pounds, not 78 pounds (20 kg, not 35 kg at 117 cm).28 Dunn’s metabolic requirements—his need for food, water and air—would have been closer to the needs of a sedentary fifth-grade child, not a first grader.
SED produces fragile cartilage that is highly vulnerable to damage through normal use. This leads to early, disabling osteoarthritis, especially in joints that are poorly aligned, such as in knock knee. Poorly aligned joints wear badly, just as loose bearings do. The effect is even worse when the bearing material itself—cartilage—is defective.
Osteoarthritis is classified as a non-inflammatory type of arthritis. However, the disease does cause inflammation in the lining of affected joints (the synovium), shredding and erosion of cartilage, and bone cysts and spurs. Television commercials for pain relievers refer to “the minor aches and pains of arthritis,” whereas “the aches and pains of minor arthritis” would be more accurate.
Pfizer, Inc. and Pharmacia Corporation (subsidiary of Monsanto, parent company of G. D. Searle & Co.) pitch their arthritis pain reliever Celebrex with TV images of laughing grandmothers bounding through the surf. But, as of February, 2001, the FDA had issued two untitled letters and one Warning Letter for false advertising, including concerns about comparative efficacy and drug interactions.29
Celebrex is a brand name for celecoxib, a non-steroidal anti-inflammatory drug (NSAID). NSAIDs in general allow modest improvements in necessary daily activities—climbing into and out of cars, managing some stairs, fastening buttons and tying shoelaces, perhaps using a normal toilet without an elevated seat or handrails.
Osteoarthritis destroys entire joints, slowly, with chronic pain and loss of function. A severely arthritic joint may become so distorted with bone spurs that it actually undergoes spontaneous fusion, after many years—which at least relieves pain by preventing movement. But this is only the final stage of a protracted, grindingly painful process that causes tense, tiring days and restless nights with aching and muscle spasm.
People affected with SED often have eye problems, most commonly nearsightedness (myopia). Detached retina is also a common complication. The reason is the relation between cartilage and vitreous humor—the clear gel that fills the eyeball and gives it shape and firmness. Both tissues are made with collagen, a connective tissue; and some types of defective collagen produce vitreous with uneven viscosity. A liquefied area of the abnormal vitreous can exert traction on a portion of the retina, the sensory lining at the back of the eye.30 If the retina pulls loose, emergency surgery is necessary to prevent permanent blindness.
The report of Dunn suffering from dry eye is not necessarily related to his SED. Many people cannot tolerate hard contact lenses; and city dwellers are vulnerable to airborne grit and chemical irritants. Cigarette smoke is another strong irritant, and Dunn apparently spent a great deal of time in smoke-filled bars.1
Bad hips—a pivotal problem
The report of Dunn’s congenital, bilateral hip dislocation is also consistent with a diagnosis of SED and presents a grim picture. Congenital hip dislocation is the most severe form of congenital hip dysplasia (CHD), now usually called developmental dysplasia of the hip (DDH). Most babies with this condition do not have a whole-skeleton dysplasia; but many children with a skeletal dysplasia do have bad hip joints.
Normal adult pelvis and hip joints
Pelvic x-ray of normal hip (left) and congenital high
dislocation (right) with narrow underdeveloped femur.
A baby born with severe DDH has underdeveloped hip sockets that are too shallow and allow the thigh bone—the femur—to slip out. If caught at birth, the condition is now treated aggressively, to prevent lifelong problems. The orthopedist manipulates the femur back into the hip socket and keeps it there with splinting, casting, or surgery if necessary. Splinting and casting impose proper contact between the two parts of the joint—the femoral head (the “ball” at the top of the femur) and the shallow hip socket. If successful, these measures will result in formation of a rounder femoral head and a deeper socket.
Untreated, a dislocated femoral head will remain out of its socket. When the child starts to walk, the femur slips upward till the head comes to rest against the side of the pelvis—where there is no cartilage. If the child still goes untreated, the pressure of walking eventually will form a shallow “false socket” on the side of the pelvis. The cartilage capping the femoral head thus will grind against plain bone, and will wear out quickly. With no cupped socket to guide its growth, the femoral head loses its sphericity and grows into a bizarre mushroom shape.
At the same time, the muscles, nerves and ligaments grow to accommodate the foreshortened “telescoped” femur. Corrective surgery becomes difficult or impossible after early childhood, because those tissues cannot be lengthened.
The mechanics of such a hip are inefficient, because lack of a true socket means lack of a fulcrum for the lever arm created by the femur. This deficit creates weakness in the stance position, balancing on one leg. The patient is forced to tip the torso over the affected hip with each step—a walking pattern called the Trendelenberg gait. If both hips are affected, the patient is forced into a splay-footed waddle, like Michael Dunn’s. Severe hip joint pain produces the same gait.
The odd mechanics lead to hip joint contracture—the inability to straighten out the joint. Contractures in both hips cause swayback (lumbar lordosis), which is a compensatory posture that permits the head and shoulders to remain erect, while the hips are bent. In this posture, the knees must also bend. The affected person therefore tends to stand in a partial crouch.
In a person with normal cartilage and uncorrected congenital hip dislocation, disabling hip joint arthritis often arrives by the late teens or early twenties, with bone grinding against bone. Dunn’s hips had fragile cartilage that probably wore out in childhood, making walking a misery. His shortened muscles, nerves, and ligaments would have presented a great technical challenge for any surgeon attempting hip replacement, if he had been healthy enough to tolerate the procedure.
Hip replacement had become available by the time of his death in 1973—but the implants came in only two or three standard sizes for normal adults. A compassionate, intrepid surgeon might have gone out on a limb, to help a desperate human dwarf. If he enjoyed good rapport with his implant manufacturing sales representative, he could sometimes “get the rep to swipe a couple of hips from the dog lab,”31 as one surgeon put it. (Dogs are used for experimental implantation of orthopedic components, which must be manufactured in the correct doggie size.) But very small components made for very small humans did not start appearing on the market till the early 1980’s. They were developed partly in response to the needs of small Asians and patients with DDH, whose hip joints tend to be very small. Today, many Little People are fitted with custom implants designed by computer-assisted drawing (CAD).
Pain and immobility
Dunn surely must have needed drugs, merely to take the edge off the pain and survive the daily grind. The NSAID ibuprofen (Rufen, later Advil, Motrin) became available in 1967. As discussed above, NSAIDs are not especially effective for advanced arthritis, anyway, even when only one joint is involved. Further, chronic use of ibuprofen can lead to intestinal bleeding, which can be fatal. There were aspirin and paracetemol for mild to moderate pain;32 and for severe pain, there were addictive prescription painkillers such as codeine, pentazocine, amidone (methadone) and laudanum, which was tincture of morphine in an alcohol base.
The section on arthritis in a 1957 home health book begins with orotund authority: “All arthritic joint conditions may, for convenience, be divided into two classes: those whose causes are known, and those whose causes are not known.” After making this dubious distinction, the authors lump together pain relief modalities for various arthritic conditions. They recommend aspirin, application of heat or cold for bursitis—along with “a shoulder cap made of wool material or felt” for shoulder bursitis and “exposure to the rays of the hot sun” for chronic cases—and use of a cane for a painful knee. Under the heading on rheumatoid arthritis, they state: “In the relief of arthritic pain, amidone is superior to morphine, which was formerly used.”33 But a retired orthopedist remarks today: “As young doctors, we had it drummed into us never to prescribe morphine or other narcotics for arthritic pain.”34
There was no Americans with Disabilities Act (ADA), no provision of disabled parking placards, no wheelchair access, no electric scooters. Al Thieme, president and founder of Amigo Mobility International made his prototype sit-down scooter for his young wife with MS, in 1968, and struggled for years to sell the Amigo through medical supply shops. “They laughed at me,” he says, “they said no one would want a thing like that.”35 A manual wheelchair would have been useless, for a man with Dunn’s tiny dimensions and arthritic arms, fingers and spine. A heavy electric wheelchair would have been the only recourse—almost useless for navigating city streets without curb ramps, and an ordeal to take on plane trips. “A power chair,” says Thieme, “was thought to be for someone who couldn’t even hold their head up. You were supposed to struggle along however you could, until you just couldn’t function anymore—then resign yourself to being a shut-in.”
What about the report of Dunn’s organs being too large for his body? This description actually implies the reverse situation: His ribcage was too small for his organs. SED apparently prevented his ribcage from keeping pace with the normal growth of his lungs and heart, which had to support life in a 78-pound person. Despite Dunn’s barrel-chested appearance—partially an artifact of severe swayback—his chest capacity likely was smaller than that of the average fifth-grade boy, who at a lean 78 pounds would stand nearly a foot taller than he did (at about 57" or 145 cm).28 The result of an inadequate ribcage is called chest constriction, thoracic constriction (constriction of the thorax), or thoracic insufficiency syndrome.
Chest constriction in a growing child prevents the lungs from completing their growth—restrictive lung disease. Permanently crowded lungs cannot inflate fully or draw in enough oxygen.
In compensation, the blood vessels running through the lungs constrict—pulmonary vasoconstriction. Normally, this is a local compensatory mechanism that shunts blood to healthier areas of the lung, where inflation is better and more oxygen is available. But the generalized vasoconstriction in restrictive lung disease creates increased resistance throughout the lungs. The heart’s right ventricle has to pump harder, in order to get the blood through the narrowed vessels and back to the left side of the heart, for delivery to the body. The result is high blood pressure within the lungs—pulmonary hypertension—and a chronic strain on the right ventricle, a disorder called cor pulmonale.
The heart is a muscle, and the right ventricle does grow thicker from pumping harder. But it also stretches out—dilates—under the volume overload, as it receives more blood than it can readily pump out to the lungs. So it progressively loses muscle tone and strength. Dilatation that advances far enough may damage the tricuspid valve, allowing a backwash from the right ventricle up into the right atrium.
Early in the course of the disease, the patient may suffer from fatigue and shortness of breath with exertion. Over time, chronic cor pulmonale leads to gradual failure of the right ventricle. The patient may wake at night feeling short of breath, and may have headaches or chest pain from lack of oxygen. As the right ventricle becomes weaker, blood backs up into the right atrium and back down through the veins, causing a rise in venous blood pressure. Fluid accumulates in the tissues (edema), especially in the feet and legs, and in the abdominal cavity (ascites). Blood vessels in the liver become congested with deoxygenated venous blood, causing liver dysfunction, and the patient may appear jaundiced. (continued)
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