Johns Hopkins Magazine -- April 1999
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APRIL 1999



In the rough and rugged world of orthopedic surgery, Dr. Michael Ain stands out.

H E A L T H    A N D    M E D I C I N E

Aiming High
By Melissa Hendricks
Photos by Mike Ciesielski

If Michael Ain believed in playing the odds, he never would have become a doctor. Rejection letters from more than 20 medical schools--as Ain received--would have convinced most people to change career plans.

But more than anything else, Ain wanted to be a doctor. He was smart and knew he could do the job. Being only 4 feet 3 inches, he staunchly believed, should not affect how his dice were cast.

Fortunately, the odds do not always prevail. Which is why now, on a rainy day in January, Ain slips on a pair of sterile surgical gloves, climbs up onto a step stool, and calls out, "Scalpel."

Proving the naysayers wrong: Ain was rejected by two dozen residency programs on the basis that he wasn't physically up to the job. Today at Hopkins, his surgical card is full. The adjustments have been minor--a stool in the O.R. and specially tailored surgical gowns.
FROM A DEMOGRAPHIC PERSPECTIVE, Ain, 38, is remarkable. A pediatric orthopedic surgeon at Johns Hopkins Hospital, he is one of only a handful of physicians in the country who are dwarfs. He may be the only dwarf in the world who is an orthopedic surgeon, a field that has the reputation of being the rough and rugged medical specialty.

But to Ain, the day-to-day adjustments that enable him to do his job and thus be remarkable are really quite simple. "To be very honest, the only special things I need are a stool in the O.R., and I have special gowns tailored that are shorter."

On this particular day, Ain is straightening and fusing the severely crooked spine of a 10-year-old girl named Stephanie. The girl has congenital scoliosis that is progressively getting more pronounced. Fusion will prevent her spine from curving into a painful deformity. "We're doing an anterior and posterior," explains Ain, meaning that he will access her spine first through her chest and then through her back.

Ain made the first incision at 9 a.m. He now removes Stephanie's right sixth rib, gaining access to the spine. The rib will also provide the implantable material that he'll use to promote the spinal fusion. Reaching through the chest, Ain removes five of Stephanie's disks, the cushiony material between vertebrae, and inserts slices of the rib in their place. He and visiting resident Michael Mann then stitch Stephanie back up.

Ain's hands are muscular. He works with concentration, alternately offering instruction to Mann and punctuating these lessons with banter and bad jokes. At one point, I ask whether the patient will live comfortably without one of her ribs. His eyes twinkling above his surgical mask, Ain replies, "Adam lost a rib, didn't he?"

By noon, the first half of the operation is complete.

What are the odds?
A few years ago, geneticists determined that achondroplasia results from a mutation on the fibroblast growth factor receptor-3 (FGFR3) gene on chromosome 4. "This site seems to be the most mutable in the entire genome," says geneticist and pediatrician Michael Wright, acting clinic director of the Greenberg Center for Skeletal Dysplasia. The mutation is dominant and occurs in about one out of 25,000 births. It is passed from one generation to the next, or may result from a new mutation. In Michael Ain's case, where neither of his parents is an achondroplast, the mutation seems to have occurred anew.

If an achondroplastic dwarf marries a person of average stature, there is a 50 percent chance that their child will be a dwarf. If two achondroplasts marry, there is a 50 percent chance that their child will inherit a single copy of the gene for achondroplasia, and thus be a dwarf. There is a 25 percent chance that their child will be of average stature. And there is a 25 percent chance that their child will inherit two copies of the gene. Such "double-dominant" offspring die in utero or before age one.

"Okay, like we've never been here," says Ain, ordering his O.R. staff to change scrubs and surgical gloves. After putting on a fresh gown and gloves himself, Ain climbs onto his step stool. He and Mann turn Stephanie onto her stomach and begin phase two of the operation. They will fuse Stephanie's spine using small surgical hooks and rods, inserting more slices of the rib between adjoining vertebrae. "The rib helps the fusion because it is bone," explains Ain.

It will be nightfall by the time Ain completes the operation.

MICHAEL AIN TREATS PATIENTS who have a wide variety of orthopedic illnesses and injuries, from fractured tibia to club feet, but he specializes in the orthopedic problems of dwarfism and related disorders. He fuses painfully curved spines, reduces bones that compress the spinal cord, and performs other procedures for complications that can result from these disorders. In medical terms these conditions are called skeletal dysplasia--literally abnormal growth or development of the bones. Skeletal dysplasia is a grab bag of more than 100 different disorders, most of which cause short stature.

The bulk of people with skeletal dysplasia, including Ain, have a form of dwarfism called achondroplasia. Achondroplastic dwarfs have an average size trunk, but their limbs are shorter than average and often their head is enlarged. Many also have bowed legs and swaybacks. On average, they grow to about 4 feet to 4 feet 3 inches. (In contrast, people with dwarfism stemming from an endocrine dysfunction are extremely short-statured but have limbs that are proportional to their trunk size.)

While achondroplasia does not affect intelligence, it can contribute to a host of medical problems ranging from chronic ear infections to potentially fatal compression of the spine. Hopkins is one of the world's leading medical centers for patients with achondroplasia and other forms of skeletal dysplasia. Dwarfs from all over the world visit Hopkins for medical care, which is coordinated through the Greenberg Center for Skeletal Dysplasia, named for philanthropists Alan C. and Kathryn Greenberg.

Healthcare providers from 23 clinical specialties are affiliated with the Greenberg Center. These include medical genetics, genetic counseling, otolaryngology, ophthalmology, neurology, nutrition, pediatric pulmonology, and, of course, Ain's specialty, orthopedic surgery.

Ain is upbeat and friendly and adept at explaining medicine in plain English, traits that endear him to many patients. But he means something special to his patients who are dwarfs, like 26-year-old Heather Davis. "I'm glad he can get beyond the barriers and has dedicated his life to helping people," says Davis, who was Ain's surgical patient two years ago and is now a graduate student at the University of Minnesota.

Marie Bieniek, mother of 5-year-old Andrew, says her son "adores" Dr. Ain. Andrew has traveled with his parents to Hopkins several times to be treated for complications of dwarfism. In September, Ain straightened Andrew's severely bowed legs.

"Some doctors think they can try to make him normal," says Bieniek. "Dr. Ain knows Andrew is normal already. He wants to keep him healthy."

A visitor meeting Ain for the first time almost expects to encounter a saint. So it is somewhat refreshing to find that he is an unpretentious mortal with a Long Island accent.

Being cast as a role model, says Ain, makes him uncomfortable. "I try to downplay it," he says with a shrug.

Ain grew up in Roslyn Heights, Long Island, where his parents still live. His father is a lawyer, and his mother is a travel agent. Neither is a dwarf.

Five-year-old Andrew Bieniek "adores" the upbeat Dr. Ain, according to his mother, Marie. The little boy has traveled to Hopkins several times to be treated for complications of dwarfism; last fall Ain straightened Andrew's severely bowed legs.
"When I was growing up, I didn't have role models who were dwarfs," he says. "My mother and father were good role models, great role models. They were very wonderful, always encouraging. Being short could never be used as an excuse--if I came home and didn't make the basketball team or get an A. They always instilled in me I could do anything I wanted to."

As a child, Ain saw a fair share of doctors and made a trek every year to Johns Hopkins Hospital to be examined by medical geneticist Victor McKusick, who diagnosed Ain's condition. Ain did not require serious medical interventions, aside from having ear ventilation tubes implanted, a procedure prescribed for many achondroplasts. (Having a smaller throat and nasal passages appears to impede ventilation, which raises the risk of middle ear infections.) However, young Michael spent enough time in examining rooms to experience a callous side of doctoring. Some doctors, says Ain, "used to talk in the third person: 'Michael's drainage output this, this, and this.' They'd kind of talk about you."

In school, he experienced the teasing that most "little people" go through. But he has always been outgoing and made friends easily. He and eight childhood friends get together every year for a reunion.

After attending Andover Academy in Massachusetts, Ain went to Brown University, where he decided he wanted to become a doctor. "I thought medicine was a fascinating field, a chance to help people." Having been a patient himself, he felt, would also be an advantage. He was determined to have a better bedside manner than many of the doctors who treated him when he was a child.

At Brown, Ain built the solid academic and extracurricular record that medical schools seek in their applicants. He majored in math, earning a better-than-B average, did research in a physiology lab, and earned good MCAT scores. He also played second base on the varsity baseball team, co-directed the university's Big Brother program, and was an officer in his fraternity. Ain sent out 20 to 30 applications to medical schools all over the country including Hopkins. On the advice of a guidance counselor, he noted in the personal letter each school required that he was a dwarf. "Because I am," Ain says simply. "It shaped me."

At first he thought he had a good chance of getting into medical school. But then his optimism began to fade. During several of his admissions interviews, officials told him he'd have great physical difficulty performing the duties of a physician. When Ain pressed them to explain, they told him he would not be able to reach his patients' bedside. To Ain, the solution seemed obvious. He would use a footstool. Others worried that he wasn't strong enough. Ain, who had been lifting weights and working out regularly, fired back, "I'm stronger than anybody you're interviewing today." He suggested he could match any of them in the weight room. What about gaining the respect of his patients? asked some interviewers. Ain thought that was a lame excuse. His classmates at Andover had chosen him to receive the coveted "End of the Year Award," signifying their respect and admiration. He had proved his leadership skills time and again. "You don't have to be intimidating to be respected," Ain says.

But Ain's fears were confirmed. One by one, the thin envelopes bearing rejection notices arrived, until eventually Ain had received one from every medical school to which he had applied.

Ain was devastated. Although none of the letters mentioned his height, he had no doubt that some or all of the admissions offices believed a dwarf could not or should not become a physician.

"I was scared. I was angry. I was hurt. It was the only time I hit the wall," he says. "It was the only time I felt trapped."

The experience challenged his whole outlook. "I was being denied this for no good reason, for a reason I couldn't do anything about. It goes against everything I was taught. My parents told me I could do anything I wanted as long as I pushed myself. If this was going to happen, then during the first 23 years of my life, this lie was being made."

Ain decided to try again. He returned to Brown the year after he had graduated to try to improve his chances of getting into medical school. He took two advanced science courses, earning two A's with distinction. He continued his research and got his work published. He applied to about 20 medical schools, including some of the same ones he had applied to the first time, again frankly revealing that he was a dwarf.

Again the thin envelopes began arriving. But this time, one envelope was fatter than the others. It was an acceptance from Albany Medical College in upstate New York.

One of the Albany faculty members who interviewed Ain was B. Barry Greenhouse, then an associate professor of anesthesiology. Greenhouse and Ain hit it off. Greenhouse was a baseball fan and was impressed that when Ain played for Brown he had batted against Ron Darling, the Yale player who went on to become a famous Mets pitcher. Moreover, says Greenhouse, "I just felt he was a good student. He impressed me immensely with his intelligence. He was a compassionate, decent young man who would be a credit to the medical profession. If I were ill, I would like him to be my doctor."

Getting a straight answer
When Michael Ain was 19, he underwent a surgical procedure known as an osteotomy to straighten his bowed legs. It involved breaking and resetting the legs. The procedure caused Ain a good deal of pain, and he lay in a body cast for several months.

A wrestling injury prompted Ain's surgery, but doctors recommend the procedure to many achondroplasts with bowed legs to reduce their chances of arthritis.

Now that he is an orthopedic surgeon, Ain suspects that this medical premise may not apply to all achondroplastic dwarfs, about half of whom have the condition. "I think many [achondroplasts] do not need any type of treatment," he says.

The link between bowing and arthritis is based on research involving patients of average stature, Ain notes. Bowing may increase pressure at the knee joint, setting the stage for arthritis. But because achondroplasts have shorter limbs, they may not have the same degree of risk, suggests Ain.

To test this theory, he and biomedical engineer Edmund Chao are creating a biomechanical computer model of bowleggedness. Ain is also conducting clinical studies in achondroplasts older than 40 to see whether the amount of arthritis the patients have correlates with their degree of bowleggedness.

Ain did well at Albany and decided while he was there that he wanted to become a pediatric neurosurgeon. He was an avid woodworker and had always enjoyed working with his hands. And he liked the immediate gratification such surgery provided. Ain applied to 14 residency programs, had nine interviews, and was rejected by every program. He then applied to nine or 10 general surgery residencies, including some "bottom of the barrel programs" to which a candidate with inferior credentials would have been admitted. Again, they turned him down.

The gatekeepers of the residency programs were blunt. "Every place one guy said, 'You can't do it physically' or 'Patients won't respect you,'" recalls Ain.

Ain spent the next year doing a pediatrics residency at the University of California at Irvine. Though he enjoyed working with children, he still yearned to be in the operating room. So Ain wrote to John Hall, a renowned pediatric orthopedic surgeon at Children's Hospital in Boston, who had done surgery to straighten Ain's bowed legs when Ain was 19. When Ain was in medical school, he had done a rotation at Children's under Hall's supervision. In his letter to Hall, Ain asked what his chances would be of getting into an orthopedic surgery residency. Hall was encouraging, and Ain applied to two residencies. He was considering a third, but the chairman at that program told him, "There's no way you're going to get in. You should be quite happy doing pediatrics," Ain recalls.

But the chairman at Albany Medical College did not see it that way. Richard Jacobs, who is now retired, told Ain, "If your record is as good as everybody else's, you'll get in. It doesn't matter whether you're 4 feet 3 inches or 6 feet 3 inches. We will take you." After examining Ain's record, Albany accepted him.

In entering the ranks of orthopedic surgery, Ain was indeed standing certain assumptions on their heads. Of all the specialties, orthopedics has a machismo reputation--not without some justification. It takes a fair amount of strength to push and pull fractured bones back into place. When a patient has dislocated a hip, for instance, an orthopedist has to lift the patient's leg and push against muscle and soft tissue to force the hip back into the socket. The tools of orthopedic surgery are bold and serious affairs. An orthopedic cart parked in the Hopkins operating suite resembles a medieval hardware store: drills, saws, reamers, mallets, hooks, rods, and screws--tools for cutting bone, reducing fractures, drilling holes for screws.

During his five-year residency at Albany, Ain proved he could do the job, says Allen Carl, an associate professor of surgery at Albany who was one of Ain's mentors. "He's just a wonderful person who exudes the positive," notes Carl.

While at Albany, Ain also began dating a nurse named Valerie Frinks, who, at 5 feet 6 inches, is of average stature. They married and now have a 2-year-old daughter, Alexa. Their daughter is a little person, says Ain, and he does not want to discuss her size in any more detail than that. "She is the most delightful, beautiful person," he adds.

At first, Ain thought he might become a hand or joint surgeon. He never considered specializing in bone disorders such as achondroplasia. "It's the last thing in the world I wanted to do because it's like looking in the mirror every day," he says.

That feeling changed one day in the operating room, when Carl mentioned to Ain that he had read a newspaper article about a couple from southern California who had an achondroplastic daughter. The parents were worried about the limitations their child would face. (After all, notes Ain, "Where do you see most little people? On TV, at the circus.") But the father had been encouraged when he met a physician at a party who was an achondroplast. If this man could become a physician, the father reasoned, then his daughter could have many opportunities in her life.

Ain suddenly realized that he was the physician at that party.

An improved outlook
Today, physicians know a lot more about some of the more serious complications of dwarfism than they did a decade ago, when 8 percent of children with achondroplasia died before age 5. Many of these children died suddenly, and the cause was a mystery. But researchers now suspect that undersized bone structure set the stage for many of these deaths, says Hopkins's Michael Wright. The culprit appears to be an opening at the base of the skull, called the foramen magnum, through which the spinal cord connects to the brain. In some achondroplastic babies, the foramen magnum is so narrow that it clamps down on the spinal cord where it meets the brain stem, compromising the nerves that control breathing. In the worst cases, babies stop breathing and die.

Through a surgical technique refined by Hopkins pediatric neurosurgeon Ben Carson, many of these babies are now spared. Carson enlarges the foramen magnum to make more room for the spinal cord. It appears that the improved surgical technique, in addition to better diagnosis of babies at risk for foramen magnum compression, is saving more babies, says Wright. Today the mortality rate for achondroplasts under age 5 is just 1 percent.

Ain and Hopkins neurosurgeon Daniele Rigamonti perform a similar surgical procedure on the lower portion of the spine, where undersized vertebrae can impinge on the spinal cord. This condition affects adult dwarfs more than it does children, causing back pain and in the worst cases preventing mobility.

"So at that point, without sounding corny," says Ain, "I thought, there are a lot of good hand and joint surgeons. If I could have an effect, help people, or be able to sympathize and understand certain issues, then maybe that's why God wanted me to become an orthopedic surgeon. Maybe this is it."

So Ain applied for a fellowship in orthopedic surgery at Hopkins, where director of pediatric orthopedics Paul Sponseller was eager to build a program in skeletal dysplasias. Sponseller had no hesitation about hiring Ain. "We thought Michael would be a good candidate," says Sponseller. "I was reassured to my satisfaction by people in his residency that he was capable of doing surgery."

AIN SEES PATIENTS at the Hopkins orthopedic clinic on Mondays. One recent Monday, at least 30 patients visit the fifth floor clinic in the Outpatient Center. Anything that could possibly go wrong with a bone--breaks, infections, wasting conditions--is seen in the clinic's warren of examining rooms.

Ain's patients include a 7-year-old child with a leg problem known as Perthes avascular necrosis that is dissolving the head of her femur; a chubby 5-year-old named Jason who fractured his tibia and femur in an auto accident several months earlier; a young boy with an arm fracture that is healing nicely; a 2-year-old girl whose cerebral palsy freezes movement in her left leg; and a toddler who was born with club feet that Ain surgically realigned several weeks earlier.

In this age of sophisticated medical technology, an orthopedist's hands are still an important tool. One of Ain's patients in clinic this day is 5-week-old Emma, a pink-cheeked, healthy looking baby. Clasping one of Emma's legs in each of his hands, Ain gently moves them from side to side, sensing through his touch whether Emma's hips are properly aligned. Emma was born with developmental dysplasia of the hips, meaning her legs slip in and out of the hip sockets. She is wearing a special harness over her legs and tummy that is designed to fix her hip joints in place over the next several weeks. The baby's mom asks Ain why her daughter was born with this condition. He replies that for some unknown reason, developmental dysplasia of the hip occurs most commonly among first-born girls who are breech babies, as Emma was. "It's the way God smiled upon her," Ain says.

Ain gently teases nervous children and their parents, and sharpens the edge of his banter when the target is a colleague.

Occasionally, a child will ask him why he is so short, says Ain. His standard reply: "I never grew as fast as everyone else." Most adults do not remark about his height.

"I really have a lot of confidence in him," says Jackie Scott, the mother of one of the patients Ain sees that day. "He is personable and funny and does put you at ease. He doesn't make you feel he's pressed for time and has got to go. That's very important."

One of the last cases of the day is the most complex. Two brothers and their sister have OSMED syndrome, a rare genetic form of skeletal dysplasia reported in only a handful of cases. The children, along with their parents and a sister who does not have the disease, have come from the United Arab Emirates to be seen by a retinue of Hopkins experts.

The children have abnormally weak collagen, which contributes to a slew of medical problems including cleft palate, knobby joints, and a dip in the chest that could pose a danger if it presses on the heart. They are small for their age, though within the normal range.

Even on the craziest days, Ain loves what he does. "I'm able to foster caring for people," he says. "I like the immediate gratification of surgery."
The syndrome is not life threatening, but it predisposes them to premature arthritis and scoliosis. It also impairs hearing. The three children with the syndrome are wearing hearing aids. They have developed their own sign language and gesture to each other while waiting for Ain to examine them. When Ain enters the examining room, they stop gesturing and look at him timidly.

"Hi. Hello," says Ain, looking around as though he doesn't know where to start. "From an orthopedic point of view, what are your concerns?" he asks the children's father.

"Their joints. All. They are painful sometimes. And will they grow?" the children's father replies through an interpreter.

One by one, Ain examines each child, asking gentle questions as he goes. The process is tedious, involving two sets of translations, from sign language into Arabic, and from Arabic into English, and vice versa.

Ain orders an extensive set of X-rays for each child, then tells the family he'll see them in a few hours after the films have been taken.

Back in his office, he shuffles through patient files. "I have a headache," he tells a nurse. "A huge headache." The family with OSMED syndrome has pushed his schedule way behind. In the meantime, there are two more patients with complex cases of skeletal dysplasia waiting to see him. He needs to see X-rays of another patient, but that patient's HMO refuses to cover radiation charges incurred outside its own offices. Frowning, Ain picks up a patient file from his desk and walks into the next examining room.

The next day, Ain apologizes for being irritable. "That's about as cranky as I ever get," he says. Even on the craziest days, says Ain, he loves what he does. "I like being at Hopkins. It's a center of excellence. I like working with the people I work with. I'm able to foster caring for people. I like the immediate gratification of surgery." He has arrived, he says, where he wanted to be.

For a long time Ain saved all the rejection letters he had received from medical schools. Perhaps one day, he thought, he would write back to those schools to tell them that he had defied their odds. Over the years, however, his anger faded. He mellowed and lost track of the letters. It's no longer important to tell people he can do the job. Now he just shows them.

Melissa Hendricks is the magazine's senior science writer.