The Boston Globe from Boston, Massachusetts • 29

THE BOSTON GLOBE MONDAY, SEPTEMBER 18, 1995 29 Marfan syndrome becomes manageable Bogart's son recounts a tough act to follow low. It's a very, very safe operation," said Dr. Nicholous Kouchoukos, a surgeon at Washington University. But with such patients living longer now, there's a greater risk of subsequent problems in other parts of the aorta, requiring repeated surgery. Sometimes it's necessary to replace most or all of the aorta.

The hardest segment to repair is the descending aorta in the chest along the backbone, with its many tributaries that supply the spinal cord and internal organs. "In the early 1970s, we weren't tackling these aneurysms, because we weren't getting people through the operations," said Dr. Anthony D. Whittemore, chief of vascular surgery at Brigham and Women's Hospital. But since then, surgeons have devised safer techniques, like cooling the spinal cord to reduce its need for Medical Institutions in Baltimore, and renowned geneticist Victor McKusick developed a several-pronged strategy to combat Marfan.

It included: Using ultrasound and other imaging techniques to monitor the width of the aorta every year. Putting patients on beta-blocking drugs to reduce the force of the heartbeat, thus slowing the aorta's enlargement. Warning patients to curb strenuous exercise that causes the heart to pump blood more forcefully into the weakened aorta. Doing surgery to replace a section of the aorta usually where it exits the heart when it has dilated dangerously. Much credit for the results goes to surgeons' long struggles to improve the results of the daunting operations on the aorta.

Although Marfan patients aren't alone in having aortic aneurysms or dissections, their weak tissues make aortic repairs particularly challenging. Surgeons have said operating on By Michael Blowen GLOBE STAFF When Humphrey Bogart uttered his trademark line, "Here's looking at you, kid," he wasn't referring to pAn his son. In fact, Bogart didn't R6VI6W spend that much time looking at any kids, especially his own. The essence of "Bogart: In Search of My Father" by Stephen Humphrey Bogart is the classic battle between a father's identity and the son's fight for independence. In this case, of course, the struggle is magnified by the father's success and the son's failures.

For years, Stephen Humphrey Bogart failed at just about everything he touched. He was booted out of prep schools and colleges. He abused drugs. His first marriage ended in divorce. His life was a mess and it didn't help, according to Bogart, to have a mother who always insisted that he accept his father's legacy instead of fighting it.

In this compelling mixture of autobiography and film history, Stephen Humphrey Bogart emerges as a man as tough and clear-headed and, in his own way, as talented as his father. And he couldn't have done it on a rougher playing field. The son of Bogie and Lauren Ba-call seemed to have it all. He was raised in a wealthy family in a neighborhood with the Crosby kids, Lana Turner and Sammy Cahn. Frank Sinatra was a frequent guest along with such notables as director John Huston and Swifty Lazar.

It sounds like great fun unless you're seeing it from a knee-high perspective where all the words are, literally and figuratively, going over your head. Maybe it's not so much fun when you want a father to be a father. Humphrey Bogart died when Stephen was 8 years old. The timing was particularly cruel. Just as the boy was becoming old enough to participate in his father's life, his father was dead.

And, over the years, as Bogie's image grew from movie star to cultural icon, Stephen Humphrey Bogart's self-esteem seems to have shrunk in direct proportion to his father's burgeoning fame. For decades, Stephen Bogart resented his heritage. Named after the character Bogie played in "To Have and Have Not" and raised amid the celebrated world of Hollywood in its heyday, he struggled to find his own identity. Whether he was producing segments for ESPN or having dinner in a restaurant, people would HMARFAN Continued from Page 25 apart the layers of the aorta's wall. It can can be instantly fatal, but with quick treatment many patients survive.

those who died without knowing they had the syndrome wferje Olympic volleyball star Flo Hy-irtan in 1986 and college basketball standout Chris Patton a decade ear-liejJ Some specialistss think Abraham Lincoln's gaunt, gangly frame suggests he was affected. A proposal to- test some of Lincoln's tissues to seeif he had the genetic mutation is qri hold. tlven today, a century after the French pediatrician Antoine Marfan Grsl described a patient who may have had the condition, the syndrome often goes undetected. Lifespans sharply increased I -That's disturbing, because a study this year showed that early diagnosis, improved preventive measures and treatment have prolonged life; expectancy dramatically. These advances have tranformed Marfan syndrome from a largely untreatable killer into a usually manageable condition.

"There's been a 25 percent increase in expected survival in a little over two decades, and there aren't many conditions you can say that about," Dr. Richard Devereux, a New York Hospital cardiologist, not- -ed last month at a National Marfan Foundation conference in St. Louis. He was discussing a study he co-authored with researchers at the University of Connecticut Health Center and elsewhere, published last January in the American Journal of Prior to the late 1960s, even if syndrome was detected, little could be done to prevent the devastating heart and aorta complications. As recently as 1972, male Marfan patients lived only to an average of about 40, and women to about 50.

"Today, the new study of 417 Marfan patients found, the projected average survival has risen to nearly 70 years. Better treatment is the biggest factor, but Devereux said physicians are also now recognizing less deadly forms of the syndrome in some people, who live longer even without treatment. Mutated gene found The study outcome "has been a real morale-booster for all of us," said Devereux, who also is chairman of the advisory board of the Marfan Foundation. Research into the syndrome has intensified since 1990, when scien- tists discovered that a mutated gene on chromosome 15 causes Marfan. The gene normally makes a protein called fibrillin, which forms hairlike structures called microfibrils that give ligaments, tendons, artery walls and other tissues strength and resil-j ience.

When the gene contains a change or mutation and about 80 different mutations have been found "There's been a 25 percent increase in expected survival in a little over two decades in Marfan patients, and there aren't many conditions you can say that about." DR. RICHARD DEVEREUX Neiv York cardiologist a Marfan aorta can be like trying to handle wet tissue paper. In their quest for improvements, surgeons have developed ingenious cylindrical grafts of synthetic materials, found ways to reinforce the sutures so they don't tear the tissue, and have gotten better at protecting the heart and other organs from damage while the aorta is out of commission during surgery. "We have a lot more time now to stop the heart and work meticulously "said Dr. Lars Svensson, a cardiovascular surgeon at the Lahey-Hit-chock Clinic in Burlington.

Svensson, who heads a Marfan syndrome clinic at Lahey-Hitchcock, said he has operated on about 30 Marfan patients with no surgical deaths. Not all those with Marfan syndrome will need surgery. But in most, a portion of the aorta will enlarge enough at some point that doctors will recommend surgery to replacing the initial segment, including the aortic valve, before it ruptures. "The risk of this operation is very Vi STEPHEN HUMPHREY BOGART BOGART uo In Search of My Father By Stephen Humphrey Bogart, Dutton, -325 illustrated, $24.95 point and stare. Even though he was out of earshot he could sense the words as clearly as if they'd beefj amplified by Bose "He's HumV phrey Bogart's son." He felt freakish reflected in the mirror of his father's celebrity.

For years his mother implored, some might say badgered, her son to take pride in his father's accomplishments. Instead of resenting his father's glorious heritage, she insisted, he should be honored by carrying on his father's name. He saw it differently. In one of the book's revealing anecdotes, the author recalls afeage 7 going to lunch with his father at Romanoffs, the famous Hollywood hangout where Bogie had his own table. This was a sort of coming out party for Stephen where the father could show off the son and the youngster could bask in the glory of his famous father.

By the time.1t was over, the kid was bored and the father angry. Dad would rather talk shop and, as the son puts it, he'd have rather been climbing trees with Diane Linkletter. While Humphrey Bogart held most groups particularly phony Hollywood types and most politicians in contempt, he adored writers. After finishing "Bogart: In Search of My Father," you can't help but think that he would have adored his son the author. One final note: Stephen Bogart gives credit to Gary Provost, who assisted him with the manuscript.

Provost has since died and the book is dedicated to both Stephen Bogart's father and Provost. about time those croakers are made to pay for goofing up. Too bad it doesn't happen to more of them. The world would be a lot safer place for all of us. According to Larry McShane of the Associat ed Press, a physician in New York City mistook a patient's dialysis catheter for a feeding tube and ordered two quarts of feeding solution pumped into the woman's stomach.

Four days later, she died. The doctor was convicted and sentenced to weekends in jail. Yes, I said weekends. During the week, he is permitted to see patients. An even more frightening case is that of a doctor who was barred from practicing surgery at a Utica, N.Y., hospital but continued to treat patients in his home.

This doctor was accused of operating on patients without using an anesthetic and sewing the wrong parts of a woman's anatomy together after a Caesarean section. The state Department of Health ordered an emergency revocation of the doctor's license, but the Board of Regents decided to suspend his license for three months instead! Several weeks later, the New York paramedics were called to this doctor's home. They found a dead woman with a breathing tube inserted in her stomach instead of her lungs. Did the law catch up with him? No. They were too late.

The neighbors said they saw him and his family packing their ear. They left I'd bet anything he is practicing medicine in another city. FED UP IN N.Y. Hair-raising? You'd better believe it. Does anyone out there have any answers? I ilon't.

Patient lifespans up sharply, says Dr. Richard Devereux. so far the microfibrils are defective and poorly organized, causing tissue weaknesses throughout the body. In addition to having long arms and legs and sometimes heart problems, people with Marfan may have loose and extremely flexible joints. Many have vision problems.

Curvature of the spine, or scoliosis, is common and can be disabling. The chest bones may be protruding or sunken. "I have it bad," said Harriet George, 63, of Thomaston, Maine, who is severely hunched over with scoliosis. On average, 50 percent of the offspring of an affected parent have Marfan syndrome. But four of George's six children have the syndrome and suffer from scoliosis as she does.

One son, William George, suffered a aortic dissection last December. Three of her affected offspring also have a child with Marfan syndrome. "We've had a lot of rotten luck," she said. Vision problems common Because eyes have a lot of connective tissue, nearsightedness is very common, and often the lens of the eye is displaced because the ligaments that secure it are weak. Carmella Kolman, an artist who lives near New Haven, doesn't have the gangly look of a classic Marfan, but her eyes were severely affected as a child.

Her father, a judge in Chicago, became blind when young and died at 34 of an aortic rupture. Kolman had cataracts that worsened from childhood on. They were removed when she was 22. When Marfan syndrome is diagnosed in children, they may be given drugs to slow enlargement of the aorta. Some infants are born with a severe form that is inevitably fatal.

Adults with Marfan syndrome frequently have a heart murmur from a prolapsed, or floppy, mitral valve, a condition also common in people without the syndrome. The aortic valve alsocan malfunction when the aorta dilates markedly. The turning point in treatment came in the late 1970s, when Dr. Reed Pyeritz, then at Johns Hopkins cally sealed greenhouse, or even carry out research by monitoring data -live, as it is gathered by the hundreds of electronic sensors that continually monitor conditions inside. Adshead is hoping that as her class progresses, students eventually will be "interacting with the principal investigators at Biosphere 2 in such a way that they are actually talking science with the researchers, as opposed to saying, 'So, what's it like to be a One thing that should help the Biosphere 2 project work smoothly is an innovative piece of computer software developed by scientists at Northwestern University to help students in several places collaborate on a research project The software, called CoVis for collaborative visualization, enables hundreds of students to enter their notes, data, theories and comments into a common online "notebook." "We're piloting a radically transformed science curriculum," said Roy Pea, one of the developers of CoVis and dean of Northwestern's school of education and social policy.

With it, he said, students "develop their science understanding by practicing science as it is practiced by scientists." Teachers involved in the project say it seems to be working. "I've seen kids who I know have been turned off by school in general become excited about learning for the first time in years," said Larry Geni, a teacher at Evanston Township High School in Illinois. oxygen and adding blood clotting factors to prevent excessive bleeding. Today, said Whittemore, the risk of death in operations on the descending aorta has dropped to 10 percent or less. And the risk that the patient will suffer paralysis of the legs has fallen from 20 percent to 5 percent, he said.

Repeated problems For Ellen Ford, these advances didn't come a bit too soon. After surviving her 1984 dissection, she needed more surgery in 1988 when her graft developed a leak. Then, last December, her descending aorta tore while she was loading groceries into her car. In a grueling 8-hour operation, Whittemore at the Brigham replaced the dissected section with a synthetic graft. Ford's recovery was slow, and one of her vocal cords was paralyzed during the surgery.

But therapy has helped her regain her voice, and she has returned to her doctoral program in clinical psychology at Antioch-New England College in New Hampshire. "I feel like I'm not ready to die, and my resources are coming back," she said recently. Moreoever, she had an opportunity to make a difference for someone else. While recovering from her last operation, she was tended by a visiting nurse who one day said, "You know, I have a friend who looks just like you" but who knew nothing about Marfan syndrome. "You've got to talk to her! You've got to tell her!" Ford recalls responding.

The nurse did, and set up an appointment with a cardiologist who found that the woman not only had Marfan syndrome, but an aneurysm on the verge of rupture. After surgery, the woman is doing well, says Ford, who declares, "We saved her life!" agency's Jet Propulsion Laboratory and project manager for Red Rover. "We've had enormously good reactions from students of all different types," he said. Powell said students from South Central, an "oasis for gangs and drugs, do just as well as the top science students selected from a science class in Utah." The experience they gain, Powell hopes, will enable some of them to become leaders of the space program in the future. "The challenges the students are facing are exactly what the real project managers are facing," he said.

Milton. 5-8 p.m. To register: 696-8810. THURSDAY, SEPTEMBER 21 "Modern Asthma Medications: What Is Best for Yor?" Forum sponsored by the Asthma and Allergy Found. p.m.

Mass. Respiratory Hosp 2001 Washington Braintree. Register: 1-800-610-7400. Send notices to: Bulletin Board, Health Science, The Boston Globe, Box 2378, Boston, MA 02107-2378. Priority is given to items of widest general interest, and publication is dependent on space availability.

Before facing scalpel, get out magic marker Dear Ann Landers: After reading the horror stories in your column about mistakes made in the operating room, I had to write, Doctors and nurses are only hu- For the students, it's real-life experience eryone else. Un- tmmMmmmtm From preceding page 'chance to put in practice." New projects are constantly add- ing to the range of opportunities for students to participate in far-flung research. This year a pilot project wip enable students in four schools Including Cape Elizabeth High II School in Maine to link up with sci-. eritists at the Biosphere 2 project in Arizona to learn about ecology, global climate change and the cycles of "water and carbon through Earth's atmosphere, oceans and land. "I think it will have more of a sense of reality for my students, because of being able to communicate with other students of their age, as well as adult scientists," said Gail Adshead, who teaches a course in environmental science to Cape Eliza-Tjeth ninth graders.

She is one of the teachers from around the coun-try selected to participate in four one-month projects with Biosphere 2 this year. Next year, Biosphere 2 scientists hope to expand the project to dozens more schools. But they don't want to expand too fast, because part of the concept is to be able to create an ongoing electronic dialogue between the students and the scientists. "We want to build a community of people," said Josh Tosteson, who is developing the Biosphere 2 curriculum. "We want to make sure that's preserved as we expand." Eventually, students will be able to propose experiments for scientists 'working inside the 3'4-acre hermeti Similarly, the Planetary Society's "Red Rover" program, in which middle-school students build Mars rovers and simulated Martian environments, has served to intrigue many young people who otherwise might never have thought about getting in-.

volved in a scientific project. One of the most successful sites in the project is the Challenger Boys and Girls clubs in the blighted South Central area of Los Angeles. Several thousand kids participated in the project there over the summer, said George Powell, a 'spacecraft systems engineer at the space MONDAY, SEPTEMBER 18 Prostate Health Lecture. 7 p.m. Milton meeting room, 92 Highland Milton.

To register: 696-8810. WEDNESDAY, SEPTEMBER 20 "Helping Families Take Control" Lecture by Dr. Paul Raia, Alzheimer's Assoc.of Eastern Mass. p.m. Hearthstone Alzheimer Care.at New Horizons, 400 Hemenway Marlborough.

Free. Info: 508-481-8808. Prostate Caneer Screening. For men over 50. Milton Hostpital, medical office bulling 100 Highland fortunately, some of their mistakes end up in the mortuary.

A few years ago, I needed to have surgery on my right foot. I took a black magic marker to the hospital and wrote on my left leg from my mid-calf down, "No! Wrong foot! Don't cut anything! Look on the oilier leg!" On the right leg, I wrote, "Yes! This is the right one. This foot is the one to operate on." Some people will insist it is the surgeon's responsibility to do things properly, but I figure it doesn't hurt to give the person with the knife a little guidance. PENNI IN MOORE, OKLA. Thanks for your testimony.

It brought back an old memory from the late '60s. Dr. John Merrill, a distinguished kidney specialist at Harvard Medical School, told how startled he was early in his career at Peter Bent Brigham Hospital in Boston, when a surgical patient was un-draped. The attending physicians saw in bold, block letters over the right kidney a message printed in bright red lipstick. It said, "Do not cut here.

This kidney is fine. Take out the other one." I thought that story was funny at the time, but now, many years later, I see the wisdom of it Read on for a letter that starts out in a reassuring way but ends up scary: Dear Ann Landers: At last, a doctor who "made a mistake" is going to jail. I say it's NMNlflRMIMM.