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02-02-04 Roundtable Part 3

02-02-04 Roundtable Part 3

2004

A collection of Pulitzer Prize-winning articles by & Thomas M. Burton

in the category of Explanatory Reporting Deadly Discrepancy: New Light on Aortic Aneurysms

THE JOURNAL. A few years ago, we set out to make Wall Street Journal coverage of health—both as an industry and as a personal concern of our readers—as authoritative, vital and engaging as our coverage of and technology. We’ve been pleased to see this additional coverage valued by readers, and we’re honored to see some of the best of it recognized with journalism’s highest award.

Karen Elliott House Publisher, THE WALL STREET JOURNAL. Deadly Discrepancy: New Light on Aortic Aneurysms

A collection of Pulitzer Prize-winning articles by

Kevin Helliker Thomas M. Burton

in the category of Explanatory Reporting Cover designed by Joanna Tobias, Reference Services Dept. THE WALL STREET JOURNAL.

2004 Pulitzer Prize for Explanatory Reporting Deadly Discrepancy: New Light on Aortic Aneurysms by Kevin Helliker and Thomas M.Burton

Battling the Bulge: Test for Aneuryms Might Save a Lot of , Some Say by Thomas M. Burton January 13, 2003 ...... 6 Fears Mount Over Dangers of Pumping Iron by Kevin Helliker March 13, 2003 ...... 8 A Time Bomb Near My by Kevin Helliker April 22, 2003 ...... 10 How An Autopsy Could Save Your by Kevin Helliker June 3, 2003 ...... 13 A Death Sentence You Can Avoid by Kevin Helliker June 10, 2003 ...... 15 Denying Death No More by Kevin Helliker October 21, 2003 ...... 17 Knowledge Gap: Medical Ignorance Contributes to Toll from Aortic Illness by Kevin Helliker and Thomas M. Burton November 4, 2003 ...... 19 Health: Which Test Should You Get? by Kevin Helliker November 21, 2003 ...... 23 Health Journal: A Mother’s Mission: When You Just Know the Doctors Are Wrong by Kevin Helliker December 23, 2003 ...... 24 The Surgery Your Shouldn’t Perform by Thomas M. Burton December 30, 2003 ...... 25 Paul E. Steiger Managing Editor THE WALL STREET JOURNAL. , New York 10281 212.416.2327 Fax: 212.416.2720 !

February 1, 2004

The Pulitzer Prize Board 709 Journalism 2950 Broadway New York, NY 10027

To the Judges, Explanatory Reporting:

While the media focus on AIDS and certain cancers, an even more common killer is at large. Most doctors don’t recognize its symptoms. Most patients don’t know they are at risk. And most of the 25,000 to 40,000 American deaths it will cause this year may be preventable. The killer? The aortic aneurysm, a bulge in the body’s largest artery. When an aneurysm rup- tures, it is almost always fatal. Conventional medical wisdom holds that aneurysms are rare and that little can be done to prevent them. But as Wall Street Journal reporters Kevin Helliker and Thomas M. Burton explain in this explosive, year-long investigation, the conventional wisdom is wrong on both counts – with deadly results. Messrs. Helliker and Burton uncovered the surprising prevalence of aneurysms, which in fact claim more lives than AIDS each year and perhaps as many lives as breast and prostate cancers. They explain how a simple screening process, using often inexpensive body scans, can easily detect aneurysms, which can then be repaired before they do any harm. And they expose in disturbing, precise detail why these simple steps haven’t been taken or disseminated by the medical community, which remains largely uneducated about aortic disease. As the legendary heart surgeon DeBakey, inventor of aortic aneurysm-replacement surgery, told the reporters, “No physician can diagnose a condition he never thinks about.” Messrs. Helliker and Burton lay bare the deadly discrepancy between available medical knowledge about aortic aneurysms on the one hand and the ignorance of many front-line physicians on the other. They show how thousands of deaths could be prevented. And their work already has saved lives. While most people might associate aortic disease with the death this year of actor John Ritter, the Journal reporters came to this important story in a more personal way. Mr. Helliker, the Journal’s 43-year-old Chicago Bureau Chief and a triathlete, was diagnosed with an aortic aneurysm himself, discovered by chance during a CT scan. Mr. Helliker, who helps oversee the Journal’s health coverage, had never encountered in his extensive perusal of medical literature the fact that aneurysms could be detected – and surgically removed – before they burst. A simple question struck Mr. Helliker as well as Mr. Burton: If new-technology scans made possible the discovery of Mr. Helliker’s aortic aneurysm, why wasn’t the medical profession seeking to find this killer in others? The pursuit of that question led to more alarming ones. In the past two decades, Messrs. Helliker and Burton learned, medical science has mastered the ability to detect and repair aneurysms in the aorta. Scientists have also made tremendous progress in identifying people at high risk for aortic aneurysms. Yet in reviewing the medical literature, Messrs. Helliker and Burton realized that the rate of misdiagnosis for rupturing aortas hadn’t declined – and neither had the death rate. How could that be? Finding experts who could shed light on that question wasn’t easy. No medical specialty is devoted to the aorta. There are no patient advocacy groups, nor is there any patient literature. What was even more difficult, the nation’s few aortic specialists were reluctant to go on the record with their criticism that the medical profession has itself failed, and that thousands are dying because of medical ignorance. Finding patients presented another challenge. The two reporters spent months researching medical literature, combing through obituaries and interviewing survivors, ultimately turning up dozens of cases of medical mishandlings of aortic aneurysms. The result is a body of work so authoritative that many physicians have contacted Messrs. Helliker and Burton seeking aortic advice. Their work persuaded federal death-statistic experts to concede that the official number of aortic deaths – about 18,000 a year – was 40% too low, while aortic experts believe the actual number may be more than twice as high. Their work was cited in a December article in the Journal of the American Medical Association. “I’ve found better information in The Wall Street Journal than in the medical literature,” said Peter Luttrip, a radiologist and aortic-aneurysm survivor. The impact of these stories has been overwhelming. Many readers learned from the coverage something no doctor ever told them – that they belonged to high-risk groups for aortic aneurysms. Numerous readers took the Journal articles to their doctors to demand screens, and several found aneurysms as a result. Medical societies are using the articles to seek Medicare funding for aneurysm screening. The cause has been taken up by former Sen. Bob Dole, who cites the Journal in speeches asserting that medical ignorance is killing people with aortic disease. Ivan Lubash of Sudberry, Mass., was one of many readers who learned of an aneurysm thanks to the coverage. As he wrote to the reporters: “I believe my reading the article and following up on it has saved my life.” The Wall Street Journal is proud to nominate this groundbreaking work by Kevin Helliker and Tom Burton for the Pulitzer Prize for Explanatory Reporting.

Sincerely,

Paul E. Steiger 6 Reprinted from THE WALL STREET JOURNAL JANUARY 13, 2003

Doctors almost never inquire about a Battling the Bulge family history of aneurysms, even though they have a strong familial link. They How It Works Te st for Aneurysms draw minuscule research funding. And Aneurysms arise when a while vast medical industries have grown thinning, weakening section up to detect ills such as prostate cancer of an artery wall balloons Might Save a Lot and diabetes—with doctors routinely out to twice the artery’s ordering tests and insurers routinely normal diameter. The Of Lives, Some Say paying—doctors hardly ever suggest that most commonly deadly a well patient take a simple test that of these occur in the could detect countless repairable aortic aorta, the big vessel Artery Flaw Is Fixable if Found aneurysms. It costs as little as $40 at stretching from the And Often a Killer if Not, some centers. heart to the lower “These deaths are basically pre- abdomen. But Lacks a Constituency ventable if people just got themselves screened,” says M. David Tilson, who has Aorta treated and researched aneurysms for more than a quarter-century and now ‘These Deaths Are Preventable’ holds an endowed chair in surgery at Columbia University. “Aneurysm disease By THOMAS M. BURTON is one of the most neglected diseases in Jo-Anne Coe took every medical test American history.” Kidney recommended by doctors and was deter- Why this anomaly? An important rea- son is that aneurysms produce no large Normal Aneurysm mined to stay healthy. At 69 years old, aorta she was working as an aide to former group of patients motivated to raise Sen. Bob Dole while remodeling a Vir- awareness and funding. Most people with ginia farmhouse. an aneurysm never have symptoms and But on Sept. 27, while shopping for don’t know about it. If it bursts, they’re kitchen cabinets, Ms. Coe felt an intense usually dead or disabled. In a third pain in her back and went to an emer- scenario, where the aneurysm is found gency room. Unlike chest pain, back pain and surgically repaired, patients aren’t A normal aorta is between 1.6 and 2.8 often isn’t regarded as urgent, so she likely to become activists. Most are essen- centimeters wide. If a spot gets as wide waited 90 minutes to see a doctor. After tially cured, unlike the many people who as 5.5 centimeters, the risk of rupture finding that blood was pouring into her live for years fighting cancer, AIDS or may be high enough to call for surgery. body from a leaking aneurysm—a bal- heart disease. looned section of a blood vessel—alarmed A rare exception is Bill Maples, who the screening but also monitoring of doctors rushed her launched a support group and Web site aneurysms found, and surgery for some into surgery. Dur- out of his after having an aneurysm of them. To Rodney White, a surgery pro- ing the operation, found and fixed. “We have no funding fessor at UCLA, “It’s a social dilemma on her torn aorta, whatsoever,” says Dr. Maples, a retired because you can’t afford to screen every- she died. college biology professor in Carrollton, body. But the argument for screening is The popular im- Ga. stronger now [and] a lot of professional pression is that an- A different obstacle prevents screening groups are advocates for screening.” eurysms are like tests from becoming common and cov- Aneurysms arise when a thinning, lightning: striking ered by insurance. Many insurers take weakening section of an artery wall bal- rarely, suddenly their cue from Medicare, which doesn’t loons out. Such spots are considered and unpredicta- cover aneurysm screening. Now, how- aneurysms when they reach twice the bly. In fact, the ever, a debate is stirring in medicine over artery’s normal diameter. The deadliest most lethal aneu- occur in the aorta, the big vessel stretch- whether some groups of people with no rysms, those on ing from the heart to the abdomen. symptoms should be screened for the aorta, develop They usually produce no symptoms. K. Craig Kent slowly, are often aneurysms. K. Craig Kent, chief of vas- The majority never burst. But when they easy to diagnose cular surgery at New York Presbyterian do, the patient dies about 90% of the time, with an inexpensive ultrasound test, and Hospital-Cornell, did an economic often never reaching a hospital. By con- can usually be treated. analysis concluding that ultrasound trast, when a large aortic aneurysm is But most are never diagnosed, with the screening for abdominal aneurysms found and operated on, the survival rate result that bursting aneurysms in the would be more economical, in terms of is typically 95% or better, depending on abdomen and chest kill an estimated life-years saved, than mammography. In the hospital and where on the aorta the 18,000 Americans a year—more than an article last August in the journal aneurysm is. AIDS or brain cancer, and four times as Surgery, Dr. Kent recommended that all Most deaths from aortic aneurysms many as cervical cancer. Based partly on men over 60 be offered a quick ultrasound involve the abdomen, where they can be estimates from doctors, deaths from all exam of the stomach. He also urges the detected by the inexpensive, and very types of aneurysms, including cerebral, test for all women over 60 who have a accurate, ultrasound test. The rest of equal prostate cancer’s toll and approach family history of aneurysms. aortic-aneurysm deaths—an estimated that of . 2,500 a year in the U.S.—occur where the ‘Social Dilemma’ vessel runs through the chest. Spotting Neglected Disease What such a policy might add to the these usually requires a CT scan, which For all this, there is no national effort country’s surging medical bill is can cost as much as $800. to find aneurysms before they rupture. unknown. The cost would include not only Cerebral aneurysms present a more Reprinted from THE WALL STREET JOURNAL JANUARY 13, 2003 7

complicated case, because detecting them has tripled to about 200,000 a year. The costs more and it’s not so clear which surge appears partly to reflect the greatly ones need surgery. But these, too, can be Little-Known Killer increased use of CTs, MRIs and the like, deadly. They kill roughly 14,000 Ameri- Aneurysms are comparable to some high-profile to check for tumors or other conditions. cans a year, estimates Gary Steinberg, killers, though not to the biggest, such as heart Relatively few scans are done simply to chief of neurosurgery at Stanford Univer- disease, stroke and lung cancer. Estimated hunt for aneurysms. sity Medical Center. annual deaths in U.S. Would insurers pay if tests to detect The aneurysm toll actually may be Breast cancer aneurysms were done to screen symptom- much higher. In the absence of autopsies, coroners tend to attribute sudden deaths 41,000 less patients, rather than to diagnose to cardiac failure. Each year in the U.S., Aneurysms* symptoms in individual cases? So far, about 450,000 sudden deaths, most of 32,000 insurers haven’t faced the issue. When them unautopsied, are ascribed to car- Prostate cancer asked, some point to the added costs that diac events. Dr. Kent says it’s likely a 31,500 would result from monitoring and sur- substantial portion actually are due to AIDS gery, and say they haven’t been con- burst aortic aneurysms. 14,500 vinced that screening would be broadly High blood pressure raises the risk, Brain and nervous-system cancer effective. both that an aneurysm will develop and 13,100 Some doctors also are reluctant to that it will someday burst. Yet while doc- endorse widespread testing. Robert tors routinely check blood pressure, and Cervical cancer 4,400 Zwolak, a vascular surgeon at Dartmouth warn about heart risk, they rarely Medical School, says, “We think we can mention aneurysms. *Includes abdominal, thoracic and cerebral identify a risk group for whom aortic Meanwhile, doctors and fitness experts Note: Aneurysms and AIDS figures are for 2000; aneurysm screening is appropriate, but are increasingly preaching the benefits of others are 2001. we need more substantiation.” weightlifting, including for the elderly. Sources: National Center for Health Statistics; American But “heavy weightlifting and heavy Cancer Society; interviews with physicians A huge British study provided some straining could worsen aneurysms,” says last fall. After following 61,000 men aged Christopher K. Zarins, chief of vascular Screening for aneurysms on the aorta 65 to 74 for an average of four years, it surgery at Medical would be simpler, because these appear found a 42% drop in risk of death from ab- Center. He suggests that people with in more distinct patterns. For instance, dominal aortic aneurysm among those aneurysms use only light weights. 80% occur in men, and the odds rise with who had been screened. Their aneurysm Institutes of Health will hypertension, smoking and arterioscle- death risk was 1.9 per 1,000, vs. 3.3 in the spend $2.77 billion for research on AIDS rosis. others. “Screening can significantly this year, along with $732 million on In addition, when this type of aneur- reduce mortality rates associated with breast cancer and $408.3 million on pro- ysm is spotted, it’s easier to know if abdominal aortic aneurysms,” concluded state cancer. The amount for abdominal surgery is needed, because the risk of the study, published Nov. 17 in The aneurysms is just over $6 million. rupture increases with size. Normal Lancet, the British medical journal. Some doctors say cerebral aneurysms, aortas range between 1.6 and 2.8 centime- A large new study is being organized in particular, warrant more study. It is ters wide. Doctors say any sections wider in the U.S. by the medical schools of difficult to know which ones are likely to than four centimeters generally need to burst, and it takes a $1,500 magnetic-reso- Dartmouth, the University of Pennsyl- be watched closely. Many are stable, but vania and the . nance or CT scan to find them. Despite when they start growing, alarms go off. If Initially it will measure the prevalence of the uncertainty, experts recommend the a spot gets as wide as 5.5 centimeters, the aneurysms; a later phase will check for a exams in a number of cases, such as risk of rupture may be high enough to call mortality benefit from screening. persistent severe headaches and vision for surgery. The death rate in surgery problems. isn’t negligible but is much lower than “There is reasonable emerging evi- Devastated that from burst aneurysms. dence suggesting that it’s reasonable to screen men over 60” for abdominal aortic Lois Porteous might have benefited. U.S. surgeons repair about 50,000 ab- aneurysm, “particularly if they have a Suffering from severe headaches and a dominal aneurysms each year, typically loss of peripheral vision on one side, she replacing the puffed-out area with a history of smoking, and anyone with a was given headache medicine but no plastic or fabric tube. Some now use a first-degree relative with an aneurysm,” scan. Last Jan. 30, the 58-year-old in less-invasive procedure that threads a says Jack L. Cronenwett, a study orga- Zebulon, N.C., collapsed in her kitchen tubular device called a stent-graft into the nizer and chief of vascular surgery at after an aneurysm behind her eye burst. bubble. Dartmouth. That would have included She survived but needs 24-hour care. “It Over two decades, the number of intact Ms. Coe in Virginia: Her mother, too, had just devastated her,” says a son, Michael. aortic aneurysms diagnosed in the U.S. an aortic aneurysm. 8 Reprinted from THE WALL STREET JOURNAL MARCH 13, 2003

surgeon, John Elefteriades of the Yale Fears Mount University School of Medicine, that people over 40 years old bench-press no more than half their body weight. Equally important is Over Dangers breathing regularly during exercise to minimize spikes in blood pressure. Of Pumping Iron Aneurysms aren’t the only concern for heavy-weight lifters. Vascular experts say it can induce stroke, as well as dissection, Weight Lifting Craze Comes in which the inner lining of the aortic artery separates from the outer walls. Under Scrutiny by Doctors Heavy-weight lifting can spike blood pressure to dangerous heights. In max- Concerned About Health Risks imum-effort lifting, which pits a partici- pant against the most weight he can hoist By KEVIN HELLIKER one time, studies have shown that blood pressure rises to as high as 370/360 from S A FITNESS TRAINER and health a resting rate of 130/80. Conventional blood- fanatic, Michael Logan knew that pressure monitors can’t even measure A weight lifting could strengthen his levels above 300. “At that level, nobody bones and protect his heart. would be surprised if you had a stroke,” What he didn’t know was that it could be says Franz Messerli, a hypertension spec- lethal. Mr. Logan had a bulge in his ialist at the Ochsner Clinic Foundation in primary artery, the aorta. Knowledge of New Orleans. that bulge, or aneurysm, would have John Robertson witnessed just such an Getty Images prompted doctors to allow only light-weight event one day when he was lifting weights Overload: lifting. But like the vast majority of people as a medical student. Lifting beside Dr. Heavy-weight lifting can trigger with aneurysms, Mr. Logan didn’t know he Robertson was a fellow medical student had one. who suddenly keeled over backward. A strokes and aneurysms. So he continued heavy-weight lifting— vessel in his brain had ruptured. He was until an aortic aneurysm killed him last rushed to the hospital, and survived. June at age 46. “It’s very surprising that “During the time that you’re lifting, the Dissection typically occurs in older adults, something he did for his health might pressure on the artery wall is intense,” or those who have a family history or who have hurt him,” says Mike Logan, the says Dr. Robertson, chief of thoracic and suffer from a syndrome called Marfan’s dis- late Chicago trainer’s son. cardiovascular surgery at St. John’s ease. Yet Dr. Elefteriades has treated two In a nation obsessed with looks and fit- Health Center in Santa Monica, Calif. young dissection victims who had none of ness, weight lifting is the latest workout Doctors have long suspected that the the traditional risk factors but who were craze. Recent studies have shown that steep blood-pressure spikes arising from heavy-weight lifters. Similarly, a study lifting can lower blood pressure, combat heavy-weight lifting could trigger rup- conducted at Los Angeles County Harbor- diabetes and strengthen bones. Bookstore tures of already weakened vessels. Now, UCLA Medical Center profiled four men shelves are teeming with new fitness suspicion is growing that such lifting can (aged 22, 34, 37 and 57) who entered the tomes touting weight lifting. Over the damage healthy vessels. Yale’s Dr. Eleft- emergency room suffering dissection—all three years ended in 2001, participation in eriades has shown in a lab experiment heavy-weight lifters. Steriod use may weight lifting in the U.S. has risen 12%— that intense pressure can induce dissec- increase the risk. while aerobic exercise declined 2%, ac- tion, often requiring emergency open- One option for anyone over 60 or with a cording to American Sports Data Inc. heart surgery. family history of aneurysms or dissection Now, however, a small but growing number of researchers are raising con- cerns about the safety of lifting heavy weights. Such lifting can trigger strokes Pumping Iron Safely and aneurysms, and perhaps even cause The safest way to lift weights may also be the easiest. a highly fatal arterial disease called dis- section, believe doctors at prominent Go Light Breathe Rest health centers such as Start out with a weight The key to keeping down After 15 repetitions, take a School of Medicine and the Stanford that seems ridiculously your blood pressure is 30-second break. By your University Medical Center. easy. Lift and bring down regular breathing: Exhale fourth set of 15, you might be Aneurysms alone kill 32,000 Americans in a smooth, unhurried as you lift, inhale as you surprised at how heavy that a year, making them as big a killer as motion. return the weight. weight has begun to feel. prostate cancer, and a more common kil- ler than brain cancer or AIDS. Especially vulnerable to aneurysm and other arte- rial conditions are senior citizens—a inh a group that has been urged to take advan- l e tage of the bone-strengthening effects of e x ha weight lifting. 2 lbs. le Aneurysm experts express little con- cern about moderate to light-weight lifting. Some define light as an amount Two 20-minute sessions a week (or even one) of this style of lifting can add muscle tone, that can be lifted 60 times, in four sets of lower your blood pressure and strengthen your bones. 15. A leading aneurysm research and Reprinted from THE WALL STREET JOURNAL MARCH 13, 2003 9

is to get scanned before starting a lifting program. Most aneurysms and dissec- tions can be detected by CT scans. Also, an inexpensive ultrasound test can detect the abdominal aortic aneurysm, which ranks as the nation’s 13th-leading cause of death. The latest trend in resistance training is called slow lifting, in which the partici- pant takes 10 seconds to raise a light to moderate amount of weight and 10 sec- onds to lower it. Proponents tout it as a safer and more effective alternative to both regular lifting and aerobic exercise. But the authors of two books on slow lifting concede they haven’t measured its blood-pressure spikes, which is arguably the most crucial safety issue. The two au- thors, Adam Zickerman and Michael Eades, say that slow lifting produces smaller spikes than regular lifting. Other doctors and fitness experts dis- agree. They say that one cause of blood- pressure spikes during weight lifting is the contraction of the affected muscle. During slow lifting, a muscle may be con- tracted for more than 60 seconds com- pared with two or three seconds in reg- ular speed lifting. For anyone concerned about stroke, aneurysm or dissection, or for the vast number of Americans with uncontrolled hypertension, “I would not recommend slow lifting,” says Wayne Westcott, a slow-lifting proponent who is director of research at the South Shore YMCA in Quincy, Mass. 10 Reprinted from THE WALL STREET JOURNAL APRIL 22, 2003

ESSAY A Time Bomb Near My Heart

Discovering you have an aortic aneurysm could save your life; But first you have to figure out what to do with the information.

By KEVIN HELLIKER but aneurysms aren’t rare or exotic. The pared with a normal size of 2.6. It meant abdominal aortic aneurysm alone is the he had an aneurysm, and in just this N SEPTEMBER I learned I have nation’s 13th-leading cause of death. way, aortic-aneurysm diagnosis has trip- an aneurysm in my aortic artery Combined, aortic and cerebral aneur- led over the past two decades. just above the heart. This stunned ysms kill 32,000 Americans a year, more Tough Answer me, in part because I felt great. At than the toll from prostate cancer, AIDS Like most of us found to harbor these 43, I was thin and fit, and ate well. or brain cancer. Moreover, aneurysm ex- symptomless killers, the Harvard Health NotI a drink since age 22, or a since perts consider the number a gross under- Letter patient asked what he could do to 28. Only two weeks before getting this statement. That’s because aneurysms keep his aneurysm from growing. In aor- diagnosis I set a personal record in the strike quickly and usually in people mid- Olympic-distance triathlon (two hours, 28 tic aneurysms—the most common kind— dle-aged and older, who rarely get autop- risk of rupture increases with size. The minutes), finishing ahead of most com- sied. Found dead on the floor, these peo- petitors half my age. editor of the Harvard Health Letter, Tho- ple are called “cardiac event” victims and mas Lee, answered that there is no pro- What surprised me most, however, was carted off to the morgue. The actual num- the diagnosis itself. I hadn’t realized a ven way to keep aneurysms from grow- ber of aneurysm deaths could be twice ing. But he advised the patient not to living person could actually have an an- the official tally. smoke and to get regular scans and to eurysm. I thought they struck out of the So if aneurysms are so common, why monitor the status of his aneurysm. If it blue, quicker than you could say , don’t we hear more about them? Why grows to 5.5 centimeters, Dr. Lee said, and with lethal force. Everybody I’d ever don’t we screen for them? Why don’t surgery would be recommended. “But heard about having an aneurysm was warnings about hypertension mention you don’t want to get surgery unless you dead. aneurysms as a possible consequence, absolutely have to,” Dr. Lee said, warn- Others I’ve talked to since then have along with heart attack and stroke? Why ing that mortality rates from the surgery had the same impression: An aneurysm don’t the warning labels on cigarettes range from 4% to 8%. is an arterial bulge that you find out ab- note the association between smoking out when it kills you. A few people, upon and aneurysms? hearing about mine, have argued that I The medical community’s silence on The surgery is serious. couldn’t technically have an aneurysm, aneurysms isn’t anything sinister. In the because that would mean I was dead. battle for medical attention and dollars, During the most crucial Eager (and afraid) to discover what this this condition—despite its prevalence— point, the patient is clini- diagnosis meant, I searched for never gained priority status for under- and articles, and in doing so standable reasons: Seemingly healthy cally dead. came to see why aneurysms are so greatly one minute, aneurysm victims are dead feared and so little understood. If you the . No suffering, no victims’ sup- In other words: There’s nothing you want to know how to prevent or manage port group demanding action, no exorbi- can do to keep it from growing. But if it heart disease, cancer, diabetes or AIDS, tant medical costs that could be reduced grows, be afraid. Be very afraid. the popular press is teeming with - with new treatments or technology. No After six months of researching the mation. So is the Internet, with official great incentive—empathetic, political or implications of my own condition, I’ve medical Web sites devoted to diseases financial—to prevent these deaths. come to the conclusion that those of us much less common than aneurysms. But But starting about 20 years ago, a a search for information on aneurysms with known aneurysms should feel grate- change occurred. Increased use of ultra- tends to yield a very particular kind of ful. Very grateful. An accident of tech- sound combined with new-technology im- article: the obituary. nology has identified for us a problem no ages from CT scans and magnetic reso- doctor ever sought to find or warn about. Feeling Ambushed nance imaging machines started turning Even the risk of death associated with the As someone who had paid close atten- up the aneurysms that no doctor had ever aortic-aneurysm removal procedure is a tion to matters of health, I felt ambushed. sought to find. The Feb. 1 Harvard Health godsend compared with the estimated This wasn’t a disease or condition I’d Letter featured a typical case—a patient 90% mortality rate when an aortic aneu- ever known to look out for. I understood asking what it meant that his gallstone rysm ruptures or dissects (dissection be- that seemingly healthy people die every search accidentally turned up a section of ing the shearing off of an artery’s inner day of ailments I’d never heard about, aorta 3.8 centimeters in diameter, com- lining from the outer wall). You wouldn’t Reprinted from THE WALL STREET JOURNAL APRIL 22, 2003 11

and that overall I’d proved to be four years How Patients’ Prospects Differ younger than my actual age, I hardly heard him. I walked out feeling as if I’d 1 Long-term survival rates by treatment status for aneurysm patients , from time of diagnosis received a death sentence.

Elective surgery False Hopes Medical therapy2 Within hours I conjured up two hopes. 100% Emergency surgery First, that the diagnosis wasn’t as serious as Dr. Hecht had suggested. That week- end, my girlfriend and I were having din- 80 ner with her aunt and uncle, both promi- nent radiologists. My girlfriend broached the subject lightly, saying she was cer- 60 tain I was overreacting to some recent medical news. As I offered the details, however, her aunt and uncle grew visibly 40 concerned. They advised me to take this news immediately to my own doctor, back in Chicago. An awkward silence 20 hung over the dinner table. My second hope: that the scan was

0 wrong. CT scans are prone to finding abnormalities that turn out to be nothing 1 Year 2 Years 3 Years 4 Years 5 Years —false positives. But it turns out that an- 1For patients with thoracic and thoracoabdominal aneurysms of the aorta. 2Medical therapy refers to care without an operation. A variety of specific medications were used to control blood pressure and discourage aneurysm growth. eurysms on a scan are much less ambig- In general, these patients either had milder disease than the operated patients, or were too old or frail to undergo surgery. Source: John Elefteriades uous than, say, tumors. Aneurysms grow slowly and show up well on screens. know it from the vast majority of doctors lifestyle, the center promises to tell you Indeed, if the roughly 16,000 Americans you might consult about aortic aneu- your “biological age,” versus your chron- who made up last year’s official aortic- rysms, but being diagnosed with one ological age. aneurysm death count had been screened, means that your chance of surviving it is One of the procedures is a CT scan, virtually all of their bulges would have very good. meant to examine the coronary arteries. been found. Probably most of those The popular perception that aneurysms A buildup of calcium or plaque in these aneurysms would have met the standard are as unpredictable as lightning persists arteries, which feed blood into the heart, for preventive surgery, and more than largely because doctors offer no warnings is a warning sign of heart disease. I 92% of those undergoing surgery would to known high-risk groups. People whose climbed into the electron-beam tomog- have survived. The bulk of those 16,000 first-degree relatives died of aneurysms raphy machine—an advanced CT scanner would still be alive—but the medical are at higher risk but generally aren’t —thinking I’d ace this test, and I did. system would be creaking under the cost being screened. Hypertension literature “Ninety-nine percent of subjects of your of screening millions of people to find teems with warnings about stroke and age and gender have a higher calcium those aneurysms. heart attack, but rarely mentions aneu- score, and 0% have a lower score,” said the In early October, I visited a hospital in rysms. Aneurysm experts say screening computerized summary. Chicago hoping to prove the CT scan for abdominal aortic aneurysms—the But there was a problem elsewhere. The wrong. But a finer technological eye—the most common kind—ought to be routine scan happened to catch a good glimpse of magnetic resonance-angiogram with for men over 65, especially smokers. A ve- my aortic artery, which ascends from the contrast—looked inside me and confirmed ry high-risk group of people are those heart, then curves downward through the the existence of the aneurysm, though at with Marfan’s syndrome, a genetic dis- stomach to the legs, supplying blood reduced dimensions. The MRA showed order whose victims often have weak or throughout the body. At a point just above that my problem was 4.1 centimeters in diseased arterial tissue. the heart, this artery was enlarged. “It’s diameter, not 4.6. That felt like a big step Still, a significant percentage of aneu- generous,” said Harvey Hecht, the center’s back from the edge. rysms strike people outside any known cardiologist. It sounded like a good thing. It isn’t easy to find doctors who have a risk group. I’ve yet to lose any first-deg- But it wasn’t. In most locations in most deep understanding of aneurysms. When ree relatives (parents or siblings); my people, Dr. Hecht explained, the aorta my younger brother asked his primary- grandfathers died of cancer, and my measures 2.6 centimeters in diameter. care physician whether my diagnosis grandmothers lived past 90. My blood The scan showed mine, at this point just meant he ought to be screened (it does), pressure is fine, I haven’t smoked in 15 above the heart, to be 4.6 centimeters. the man looked at my brother’s neck and years and I don’t have Marfan’s. My diet is When I asked what this meant, Dr. Hecht said no—that he wasn’t at risk because his low in animal fats and high in fruits and mentioned the A-word. Aneurysm. Dr. veins weren’t visible. No expert I consulted vegetables, and I exercise an hour a day or Hecht told me that if mine grew to five or knows of any association between vein more. 5.5 centimeters, surgery would be recom- visibility and aneurysms. mended to remove it. Then he gave me What a primary-care physician typi- Smugness and Fear the most serious look I’ve ever received cally would do is refer such an inquiry to In fact, it was with an attitude of smug- from a physician. “You do not want to a specialist. Had my coronary arteries ness that I entered the Princeton Long- have that surgery,” he said. shown a worrisome amount of calcium, evity Center one morning last September. At the end of the day, I had a consulta- for instance, my internist might have ref- I went as a journalist, intrigued by this tion with Dr. David Fein, chief executive erred me to a cardiologist. But until re- new clinic’s proposition that not everyone of the Princeton Center. He’s a cently there has been no vascular equiva- born the same year is the same age. Af- brilliant doctor with a reassuring man- lent of the cardiologist, no specialist de- ter a daylong battery of exams and deep ner. But when he told me that afternoon voted to the care of your arteries and analysis of your family history, diet and that this diagnosis didn’t call for panic, veins. Only now is such a specialty devel- 12 Reprinted from THE WALL STREET JOURNAL APRIL 22, 2003

oping—the vascular-medicine specialist, patients have died of ruptures at ages as wreck than have to have that surgery,” and they number only about 300. That’s young as 11. recalls Mr. Butler, a retired software about 15,000 fewer than the number of What Now? executive in Orange County, Calif. U.S. podiatrists devoted to the care of This tendency of the medical profes- your feet. But the experts don’t have the informa- tion you really want—how to keep this sion to warn more loudly about aneurysm Not a Primary Concern bubble from growing and bursting. They surgery than about the ruptures or dissec- So research, monitoring and treatment agree that smoking, heavy weight lifting, tions it prevents may date back to when of aneurysms has fallen to that category contact sports and ultraendurance ae- the surgery, in its infancy, had a mor- of physician charged with the grim duty robic events are proscribed (no more tri- tality rate of nearly 50%. But today, at the of treating them when they rupture or athlons for me). Intense aerobic exercise highest-volume centers—such as New dissect—the surgeon. Yet the aneurysm is discouraged until a treadmill test is York’s Mount Sinai Hospital, ’s belongs exclusively to no surgical group. taken to make sure your blood pressure Medical Center and the hospitals An aortic aneurysm in the stomach is the doesn’t spike unacceptably (mine rose attached to Stanford and Yale universi- vascular surgeon’s business; in the chest, only to 165/75). Hypertension is such a ties—the rate of survival without compli- it’s the cardiac surgeon’s. The brain, mean- concern that some experts recommend cations is better than 95%. In the large while, is the province of neurologists and beta blockers even for patients whose majority of cases, surviving surgery sig- neurosurgeons. blood pressure is normal, but other ex- nals the end of the patient’s aneurysm This division of responsibility accord- perts disagree. All of these recommenda- troubles. “It was really no big deal,” says ing to location in the body means that an- tions and proscriptions are based more on Kristin Fast, a 33-year-old Southern Cali- eurysms aren’t a primary concern for intelligent assumptions than on evidence. fornia mother of two who went home four most of the surgeons who treat them. But The bottom line is that no doctor knows days after Stanford’s Dr. Miller removed there are a few who have made aneurysms how to keep aneurysms from growing. The her aneurysm in February. their bread and butter. Finding them isn’t best information on growth rate and risk of Mr. Butler, another patient of Dr. Mil- easy, although aneurysm patients share rupture comes from John Elefteriades, a ler’s, says he recovered more quickly some valuable tips on a Web site professor of cardiothoracic surgery at Yale from aortic-replacement surgery than he (www.westga.edu/wmaples/aneurysm.html) University Medical Center, whose data run by Bill Maples, a retired college biolo- show that aortic aneurysms of the chest did from a coronary-bypass and valve- gist in Georgia. grow about one-tenth of a centimeter a replacement procedure four years earlier. Before talking to some cardiac surg- year. His and other experts’ data show that Avoiding an Emergency eons devoted almost exclusively to tho- the risk of rupture or dissection exceeds the As a result, some surgeons who per- racic aneurysms, I heard various doctors risk of surgery when an aneurysm in the form these operations are quicker to dis- express the idea that the discovery of an ascending aorta reaches 5.5 centimeters. miss the concerns other doctors have ab- aneurysm as small as mine could turn out To account for various body sizes, D. Craig out the procedure. During a consultation to be disadvantageous. Some doctors Miller, a professor of cardiovascular doubt that all aneurysms grow, and sug- surgery at Stanford University Medical with Dr. Miller, I noted that if a hose in my gest that I might spend years taking tests Center, deems an aneurysm to be ripe for car were found to contain a bubble, and if a and feeling nervous about a condition removal when it is twice the normal size at mechanic said that the bubble might last that would never really threaten me. But that particular location in that particular 50,000 miles or burst 10 miles down the from the tiny scattering of physicians person. road, I would order the hose replaced, who have made aneurysm research and If my 4.1-centimeter aneurysm grew at whatever the cost. Peace of mind. treatment their mission, I did not hear a rate of one-tenth a centimeter a year, it “That’s a good analogy,” Dr. Miller this kind of talk. Their data, much of it wouldn’t reach the surgical standard for said, “except make it a King Air.” too new to have spread beyond their cir- more than a decade. Or maybe it will “What?” cle, suggest that aortic aneurysms virtu- never reach that standard. Or maybe it “Make it an airplane. Then the impli- ally always grow, and that the bigger will reach that standard and grow beyond cations are similar.” they get the greater the risk of rupture or it without ever rupturing or dissecting. Some might call that scaremongering. dissection. The unanimous view of these Maybe I should leave it alone. But it’s hard for patients who feel healthy experts is that the discovery of any an- The surgery, after all, is serious. It is a and who are experiencing absolutely no eurysm is fortunate. form of open-heart surgery that is called symptoms to willingly undergo major Another thing the experts tell you is aneurysmectomy or aortic-replacement surgery. When those patients later expe- that the chest pains that commenced the surgery. It takes about eight hours. Dur- rience what aneurysm experts call cata- instant you got the diagnosis almost cer- ing the most crucial point—a period strophic events—dissection or rupture—it tainly are imaginary, because aneurysms lasting as long as 45 minutes—the patient is cardiac surgeons such as Drs. Miller and typically aren’t symptomatic until they is clinically dead: No pulse. No breath. Elefteriades who are roused from sleep and rupture or dissect, and then the pain is No brain waves. His head is packed in intense. If you have a thoracic aneurysm, ice, his body temperature lowered to ab- rushed to emergency rooms. “The survival they will tell you that you may have been out 65 degrees. This is when the surgeon rate at that point is awful,” says Dr. Miller. born with it, may have inherited it and clips off the diseased section of artery In rare cases, rupture and dissection may have passed it on. A conversation and replaces it with a piece of Dacron occur in aneurysms of my current size. To with Dianna M Milewicz, the director of hose. me, the danger of rupture or dissection is medical genetics at the University of The eyes of nonsurgeons tend to widen scariest of all. I’m not required to wait until Texas Health Sciences Center/Houston, at any mention of this procedure. When my aneurysm reaches the surgical stan- convinced me that my son should under- Jim Butler learned he had an aortic an- dard of 5.5 centimeters to get on the oper- go periodic screenings, starting now at eurysm, his cardiologist at the time told ating table, and I don’t intend to. I’m age 9. First-degree relatives of aneurysm him, “You’d rather get in a motorcyle doing it at 5. Reprinted from THE WALL STREET JOURNAL JUNE 3, 2003 13

How an Autopsy Could Save Your Life

New Research Boosts Value Autopsies can provide a range of valuable Of Procedure, but Families health-related information. Rarely Choose to Request One Left, forensic pathologist By KEVIN HELLIKER Michael Baden. FTER HER 29-YEAR-OLD son dropped dead, Jeanann Ward re- A fused to have him autopsied. She couldn’t bear the thought. Four years later, that decision haunts her. Whatever struck down the seemingly healthy young man may be lurking in other family members, and an autopsy could have provided a warning. “Big mistake,” she says of her decision. One of the most important medical ex- ams you can obtain these days involves

somebody else’s body—that of a parent, Courtesy of HBO’s “Autopsy” sibling, child or even an elderly grand- report showed that autopsies overturned pain, and the corpse is sewn up so well parent. As genetics are found to play a the official cause of death in U.S. hospi- that open-casket viewing remains an deeper role in a wider range of conditions tals in as many as 23% of cases in the option. than previously imagined—everything year 2000. A person who dies in an auto- from aneuryisms to Alzheimer’s dis- Taking a motive accident, for instance, may be ease—your long-term survival could found during autopsy to have had a can- In the case of Russell Ward, his moth- depend on knowing a family member’s cerous tumor of the sort that runs in er’s aversion to an autopsy prompted the cause of death. families. medical examiner’s office The most reliable source of that infor- Most often, it can provide important merely to conduct a toxicology test, which mation is the autopsy. But while the value additional details about the precise cause ruled out drugs. The office then took a of the procedure is climbing, its frequency of death, allowing surviving relatives to guess and cited heart disease as the cause get critical medical of death for the big, young investment screening tests and banker, who until the moment of his death Most likely, no hospital or governmental make appropriate life- had seemed robustly healthy. style changes. After consulting with medical special- official will perform an autopsy on your Doctors, for example, ists, Mrs. Ward, a nurse by training, came loved one. So it may be necessary to hire often cite simply “heart to suspect her son suffered not from heart disease” or a “cardiac disease but from a syndrome called Mar- a private pathologist. event” as a cause of fan’s whose victims are prone to thoracic death. But new evidence aortic aneurysms. Such aneurysms have is emerging almost daily showing that it’s enormous familial implications. An art- is plummeting. Once performed on more extremely important to know whether an icle to be published this summer in the than half of American corpses, autopsies immediate relative died of a heart attack, journal Circulation profiles a Midwestern today are reserved mostly for cases pulmonary embolism or thoracic aortic family whose members young and old involving suspected foul play. Most hospi- aneurysm—three distinct conditions that have been decimated by thoracic aortic tals have discontinued the once-common are increasingly understood to bear a aneurysms. procedure in an effort to cut costs. And strong genetic component. New tests such as CT scans can find doctors, reluctant to have their diagnoses If the cause of death was a heart at- these arterial bulges before they burst. second-guessed, don’t often encourage it. tack, the surviving offspring and siblings Aneurysm experts recommend that all Consequently, fewer than 10% of U.S. should watch their diet and cholesterol, parents, siblings and children of thoracic- corpses these days are autopsied. and perhaps get their coronary arteries aortic-aneurysm victims get tested, re- Yet, it’s increasingly easy to order up scanned. If it was a pulmonary embolism gardless of age. “If we’d had an autopsy, an autopsy from numerous private com- (a blood clot in the lung), they should be we’d know for sure whether other family panies that perform them. The cost is screened for a susceptibility to that and members need to be screened for this,” substantial—usually between $1,000 and perhaps placed on blood thinners. If it says Mrs. Ward, of Randolph, N.J. $3,000—and insurance won’t cover it. But was a thoracic aortic aneurysm (a bulge (Autopsies can be performed on em- many medical experts believe it’s money in the body’s largest artery), then a CT balmed and buried corpses, but the cost is well spent. “You need to know what your scan or echocardiogram ought to be used exorbitant and the results less precise.) relatives died of,” says Catherine DeAn- to examine their aortas. gelis, editor in chief of the Journal of the To survivors of the just-deceased, an Reluctant Doctors American Medical Association. autopsy sounds gruesome. And it is, with Of those patients determined in autop- Even when the cause of death seems the chest, abdomen and skull opened for sies to have been misdiagnosed by their apparent, an autopsy can provide critical purposes of removing and examining the doctors, as many as 8% may have been information for survivors. It can uncover heart, lungs, liver, brain and other org- harmed by the medical treatment they an erroneous diagnosis. A recent federal ans. But the patient, of course, feels no were receiving, according to a recent fed- 14 Reprinted from THE WALL STREET JOURNAL JUNE 3, 2003

loved one. So it may be necessary to hire a Navigating an Autopsy private pathologist. A home—if it is local rather than part of a national chain Why they’re important and where to get them such as Service Corp. International —should n Always get an autopsy when a relative who seems healthy dies suddenly – regardless of their age. be able to offer a referral. Sometimes, pathologists on staff at the county morgue or Share the results with your primary care physician. at a local hospital will perform autopsies n If the death occurred in the hospital, request that the institution’s own pathologists perform the after hours, perhaps at the funeral home. autopsy – for free. Argue that the hospital has a moral and intellectual obligation to determine Some cities boast private companies definitively why its patient has died. such as Chicago Area Autopsy Service, a n If the hospital refuses to do it for free, ask how much the procedure would cost. 12-year-old firm that performs about 1,300 autopsies a year at a basic cost of about Then check with the county morgue or seek a pathologist referral from a funeral home. $950 each (with extra fees added for testing n If you have reason to believe that your relative received poor care in the hospital for paternity, toxicology, exposure or nursing home, seek an autopsy from a pathologist who doesn’t work for that facility. and such). In Los Angeles, 1-800-AUTOPSY n If litigation seems likely, the need may arise for a second autopsy. So consider burying rather than performs about 700 procedures a year at cremating the corpse. about $2,000 each, often on bodies flown in from elsewhere. n Request not only the preliminary report citing cause of death but the full report Problems can arise, however, when rela- that typically emerges a month or so later. This more-detailed analysis sometimes tells of incipient tives don’t agree on the need for an diseases or conditions that the deceased never knew he had. autopsy. When his mother died, Dwight K. Oxley, a pathologist in Wichita, Kan., wanted an autopsy to determine whether eral government report. Little wonder All rights to a body belong to the next of she had Alzheimer’s, a condition with a strong that doctors don’t encourage autopsies. kin except when the local medical exam- genetic link. But the procedure wasn’t “The low rate of autopsies has gener- iner demands an investigation—in which performed because his brother was ated a collective sense of cockiness in the case autopsies are performed courtesy of opposed. medical community about how accurate taxpayers. Such investigations are rela- Getting Families to Agree our diagnoses are,” says Richard Dever- tively rare, however, even in big cities with eux, professor of medicine at Weil Cornell large medical-examiner offices. In New “If the next of kin can’t agree on it, Medical College in New York. For pa- York City, the office of the medical exam- then we don’t do it,” says Zack Qualls, tients’ families, he adds, “Having an iner autopsies only about 5,000 of the 75,000 vice president of Chicago Area Autopsy autopsy is always a good idea.” or so who die there each year. Service. Hospitals used to perform autopsies Public medical examiners generally The solution for families might be to routinely on patients who died within conduct autopsies in two types of cases: discuss the rational arguments in favor of their corridors, in part because doing so when foul play is suspected, and when autopsies before death strikes. “The trou- was necessary to the retention of their people below a certain, unpublicized age ble is that you’re presented with the deci- accreditation. But that requirement has die of unknown causes. (“We don’t want sion about an autopsy at a time when been diminished or abolished, and now murderers to know that dead people over you’re overwhelmed with grief and not few hospitals will perform them. One the age of X get less of a look at,” says exception is teaching facilities such as Edmund Donoghue, chief medical exam- thinking straight,” says Dr. Donoghue, Chicago’s Northwestern Memorial Hos- iner for Chicago and Cook County, Ill.) the Cook County medical examiner. “Ma- pital, which performs about 10 free-of- Most likely, no hospital or governmental ny people who think they don’t want an charge autopsies a month. official will perform an autopsy on your autopsy later regret it.” Reprinted from THE WALL STREET JOURNAL JUNE 10, 2003 15

A Death Sentence You Can Avoid A Simple, Seldom-used Test So while radio advertisements boast that preventive CT and Ascending Thoracic Can Detect Lethal Aneurysms; MRI scans can identify early- stage cancer and heart disease, Who Should get Screened another argument for getting a preventive scan may be to By KEVIN HELLIKER detect aneurysms. Though scans Normal Aortic often fail to find cancer or heart Aorta Aneurysms HE DEATH of John Rosnow last disease—or register false posi- summer illustrates one of modern tives for those conditions—they T medicine’s most preventable fail- are brilliant at finding asympto- ings. A retired plant manager in Clinton- matic aneurysms. “There’s no ville, Wis., Mr. Rosnow collapsed after a ambiguity with a CT scan in daily exercise regimen that included rid- Diaphram terms of aneurysms,” says ing his bike seven miles. Until then, “he Michael Dake, chief of cardio- was a fitness fanatic,” says his widow, Joan Rosnow. vascular and interventional radiology at Stanford University Yet the death of Mr. Rosnow, 70, was Abdominal hardly inevitable. The aortic aneurysm Hospital. that killed him had been growing for Moreover, the value of a scan years, not days. Had he spent $40 to $150 for aneurysms is longer lasting on a noninvasive ultrasound test, his arte- than that for other diseases such rial bulge almost certainly would have as cancer. Aneurysms generally been found. Then he could have under- take years to develop, compared gone an insured surgical procedure that with certain cancers that can Yale University School of Medicine is 90% successful at repairing aneurysms. grow almost overnight. Adults at Aneurysms—bulges in weakened risk of aneurysms could undergo Aneurysms—weakened arteries that can artery walls that are almost always fatal scans as infrequently as every bulge to the size of a soda can and rupture when ruptured—may be the most pre- five years. are one of the top killers in the U.S., but doc- ventable common killer that doctors rare- tors rarely watch for them. Should you be Who Is at Risk ly warn about. They account for more screened? See the next page. deaths in the U.S. than brain cancer or Research into aneurysm AIDS. And by all accounts, aneurysm deaths has identified several deaths are underreported because sud- groups at risk. All men over 50 who visited a geneticist and cardiologist, am- den, unexplained deaths are often simply smoke—or who once smoked for years— ong other doctors. A sales representative labeled “cardiac events.” Yet despite the should consider getting screened for for a large pharmaceutical company, Mr. growing accuracy of screening technol- abdominal aortic aneurysms, aneurysm McGinley had extraordinary access to ogy, the medical community has never experts say. All men over 65, even medical experts. But nobody told him that made much effort to detect aneurysms, healthy nonsmokers, should get such a his siblings—including a sister who also and most general practitioners never test, according to a recent study has a heart murmer—should be screened. advise patients to get tested. published in the British medical journal That may be slowly starting to change. “It’s absolutely crucial that close rela- A federal health panel is considering whe- Lancet. People under 50 who should be tives get screened,” says Carl Vaughan, ther to recommend routine screening for screened include heavy smokers, those assistant professor of medicine at Weill abdominal aortic aneurysms, one com- with family histories and those with Cornell Medical College in New York, who mon form. And health insurers—which recurring sharp pain in the head, has studied the family connection. have rarely paid for aneurysm screenings stomach or back. Most aneurysm victims are middle —are starting to do so for patients with a Perhaps the biggest high-risk group aged or older, creating a false impression family history of the condition. consists of the relatives of aneurysm among doctors that the condition never The key to surviving an aneurysm is victims. Research showing that people strikes the young. When the 19-year-old knowing about it before it ruptures. About with aneurysms tend to share a genetic son of a middle-age aortic-aneurysm vic- 90% of ruptured aneurysms result in weakness in the walls of their arteries is tim began experiencing chest pains, he death. But about 90% of those fatalities relatively new but profound. At least 20% could be prevented if the arterial bulge was twice turned away from the emer- of aneurysm victims have a first-degree gency room on the mistaken belief that were detected in advance. relative—a parent, sibling or child—with The advent of CT, MRI and ultrasound he was too young to have an aneurysm. an aneurysm. Sufferers of a familial technology has produced an estimated He died, and other young members of his condition called Marfan’s are especially 200% increase in the diagnosis of aortic extended family have been found to have aneurysms—the most common variety. at early risk of the condition. thoracic aortic aneurysms, says Dianna But widespread awareness of this But nearly all these discoveries have oc- Milewicz, director of medical genetics at research has failed to penetrate the curred in patients who were being tested the University of Texas Health Sciences medical community, in part because few for something else. A radiologist scrutini- Center. zing film for gall stones can’t help noti- specialists focus specifically on veins and cing if an aorta, typically the diameter of arteries. After a scan taken to investigate What to Request a garden hose, measures as large as a a heart murmur found an aortic an- Anyone obtaining a preventive scan soda can. eurysm near his heart, Paul McGinley should tell the radiologist or technician 16 Reprinted from THE WALL STREET JOURNAL JUNE 10, 2003

Who Should Be Screened for Last month, Kevin Napolitano, a 40- Aneurysms? year-old Maine father and construction manager, fell over dead from an ■ Men over 65 should get their abdomi- unknown cerebral aneurysm. Dr. Batjer nal aortas checked out. says the chances are “very good” Mr. Napolitano would be alive and well if he ■ Men over 50 who smoke should do so. had had a scan. ■ Men and women over 50 whose parent Patients found to harbor aneurysms should demand to see a specialist, al- had an abdominal aortic aneurysm should get checked out. though this isn’t as simple as it sounds. Which specialist to visit depends on the ■ All first-degree relatives—siblings, location of the bulge. Cardiologists and parents, children—of people with thoracic cardiac surgeons follow aortic aortic aneurysms. aneurysms in the chest, while vascular surgeons treat those that appear else- ■ Anyone with two or more family mem- where—except in the head, where bers who had cerebral aneurysms, or neurologists and neurosurgeons take who died suddenly of unknown causes. over. Within these specialties, it is important to find individual doctors ■ People with connective tissue with deep aneurysm experience; many diseases such as Marfan’s Syndrome, cardiologists, for example, know little Polysystic Kidney Disease, Ehler Danlos about the condition. Syndrome. Sometimes aneurysms rupture before reaching the recommended size for sur- studying it to look out for aortic aneu- gical repair. But even in such cases, vic- rysms, because attention tends to focus tims who have had scans and are aware on organs rather than the artery sup- of their condition face better odds. That plying blood to all of them. is because emergency-room doctors For smaller and harder-to-see cerebral misdiagnose rupturing aneurysms about aneurysms, it is best to skip the CT scan 30% of the time, studies show, often res- and get an MRI. Cerebral aneurysms are ulting in death. By contrast, the patient less predictable than aortic, whose who enters the emergency room chance of rupture grows with size. But knowing he or she has an aneurysm can the value of cerebral scans for identifying go straight to surgery. the need for surgery is great and grow- One reason doctors have paid so little ing, says Hunt Batjer, chairman of neuro- attention to aneurysms is that virtually surgery at Chicago’s Northwestern Mem- nobody is sick with them. They are orial Hospital. asymptomatic, and then they kill. Reprinted from THE WALL STREET JOURNAL OCTOBER 21, 2003 17

ESSAY Denying Death No More When you know you have a potentially fatal condition, how does it change the way you live?

are enabling doctors to identify the By KEVIN HELLIKER genes that predispose us to certain cancers. Similarly, until the recent HE ACCIDENTAL dis- advent of CT and MRI scans, aortic covery last year of an aneurysms (which don’t show up aneurysm in my aortic well on X-rays) remained unde- artery was fortuitous. tected until they ruptured or This is what doctors tell dissected, which is an often-fatal Tme, anyway, and I mostly believe tear in the inner lining of the arte- it. Knowledge of the aneurysm rial wall. But now, scores of scans means I can keep an eye on it and taken to look for gallstones or coro- have it surgically removed before nary calcium are accidentally it reaches a size where rupture is a catching something more serious serious danger. — asymptomatic aortic aneurysms. But knowledge of its existence also makes me hypersensitive to every twinge in my chest and A Prophecy Fulfilled leaves me constantly wondering: In theory, such knowledge can Am I about to die? This unending enable us to act pre-emptively. But state of alert could wind up killing 2,500 years before the invention of me. The biggest danger for any- the CT scan, Sophocles wrote about one with an aortic aneurysm is how such pre-emptive action can high blood pressure — and my backfire. His drama Oedipus Rex blood pressure started climbing illustrated vividly the danger of the instant I learned about this receiving knowledge of one’s fate. aneurysm. Researchers have learned a lot Told by an oracle that he is doomed in recent years about the role of to slay his father and marry his various factors — genes, gender, mother, Oedipus flees the kingdom medication — in the development of his parents — unaware that they of all sorts of illnesses. More adopted him. Arriving then in the people than ever know they’re at STARK EVIDENCE: This MRI image shows the land of his biological parents, Oedi- higher risk for cancer, or heart author’s aneurysm. The aorta is the lighter pus unintentionally fulfills the disease, or diabetes. But what object shaped like a question mark. The awful prophecy. about the risk of knowledge? aneurysm is the wider section of the aorta Like the oracle Oedipus re- on the left side of the question mark. ceived, CT scans of asymptomatic aneurysms in some cases have set From Triathlete to Code Blue in motion tragic chains of events. Discovering that your largest artery this aneurysm means I should be even Some people, consumed by this perhaps contains a bulge that any moment could more careful about my health, it has had fatal flaw near their heart, opt for serious burst and kill you changes the way you the opposite effect. I no longer impose surgery — and die from the operation. think about yourself. News of my upon myself a Draconian diet. I’ve stop- They’ve, in effect, killed themselves by aneurysm slayed the self-perception I’d ped taking vitamins inexplicably, gained seeking to eliminate an aneurysm that had — that of a superhealthy 43-year-old a little weight and resumed my old love might never have burst. triathlete. Suddenly, I was a Code Blue affair with caffeine, which I’d given up As someone whose aorta is about 1.5 just waiting to happen. A view of one’s years ago in an effort to keep down my centimeters too large to be normal and a self as unhealthy can become self-ful- blood pressure (which I now suppress centimeter short of the current surgical filling, many doctors say. with pills). I exercise more vigorously standard, I’ve come to accept that the Stark Evidence: This MRI image than some aneurysm specialists believe I only certainty about aneurysms is dan- shows the author’s aneurysm. The aorta should. ger. You’re in danger if you act. You’re The point is, I’m now less bent on is the lighter object shaped like a ques- in danger if you don’t. tion mark. The aneurysm is the wider reaching old age than on enjoying my So you begin by trying to forget about section of the aorta on the left side of the present age. As a friend of mine said it. This is what my swimming pal does. question mark. upon hearing about the aneurysm: “If In part, that’s because knowledge can this can happen to you, we should all eat He is a cardiac surgeon who happens to change behavior for the worse. Although whatever we want.” have an aortic aneurysm, and when I The importance of knowledge as a first learned about mine he was very factor in medical outcomes is large, reassuring. He shrugged it off, said he MR. HELLIKER IS CHIEF OF THE WALL STREET growing — and little understood. Ad- and I just have big aortas, that we’d JOURNAL’S CHICAGO BUREAU. vances in medical genetics, for instance, monitor them and have surgery if they 18 Reprinted from THE WALL STREET JOURNAL OCTOBER 21, 2003

got bigger. No big deal. It gave me triathlons is the illusion you get of invin- tremendous comfort to see that his cibility. Following the Sept. 11 terrorist aneurysm didn’t worry him. Then one attacks, I wasn’t the only triathlete to day at the pool he told me that a sudden gulp hard at the revelation that some swoon had sent him recently to the emer- extremely accomplished triathletes failed gency room. “I thought I was dissecting,” to escape the top floors of the Twin he said. It turned out to be nothing, but Towers, our irrational assumption being the episode had the disappointing effect that completing an Ironman race of revealing my hero to be only human. prepares you for anything. It also confirmed what I already knew: Yet that gulp was nothing compared You can “forget about it” only so much. with the news of my aneurysm. Last After learning about my aneurysm I April when I wrote in these pages of the started experiencing a sensation of tight- shock of learning about this condition (A ness in the left side of my chest. I Time Bomb Near My Heart), I received assumed this was psychosomatic, hundreds of sympathetic letters. But the because I’d never noticed it before, and one I remember best was from a reader also because the physicians I consulted who said, “I have bad news for you and shrugged it off, saying this happened to all the other health nuts. One of these everyone who got diagnosed with an days you are going to die.” That, it seems asymptomatic aneurysm. But it was clear to me, is the message of my aneurysm, to me that they didn’t really know and there’s no escaping it. whether an enlarged aorta might be painful, and it also occurred to me that Betrayal and Freedom swollen body parts are usually sore. Then I interviewed an aortic expert — a chief But to live daily with the knowledge of of cardiovascular surgery at a top acad- death is not all bad. emic hospital — who said he is convinced It has shown me that some of what I small aneurysms do cause pain. Now did in the name of prevention was actu- every sensation in my chest feels ally a form of cowardice, of not facing my dangerous. mortality. I’d made a kind of unconscious pact that if I scored a perfect grade on those tests that ask how well you take Dreaming of John Ritter care of yourself, I’d live a very long time. A few days after the death from aortic Then along came the aneurysm, and I felt dissection last month of Hollywood star betrayed — and freed. With the pact John Ritter, I had a dream that I was broken, I no longer had to be perfect. And sharing a house with two women who amid the constant threat of death, I no were only my roommates. The dream longer the point of it. Eating only puzzled me until I remembered that this “good fats” does indeed improve your was the role that John Ritter had played chances against heart disease, but it in his most famous sitcom, “Three’s guarantees you nothing. Avoiding butter Company.” Suddenly the dreadful is no discipline if what you’re really meaning was clear: I had become John avoiding is the truth. It’s true that Ritter. Becoming John Ritter is now the staying healthy and fit improves the fear of everyone with a known aortic quality of life regardless of longevity, but aneurysm. And so, I can’t forget. The aneurysm I believe I went too far: Prevention was a won’t let me. It is a constant reminder religion for me until the aneurysm put a that I am going to die, preventing me spotlight on its substantial limitations. from engaging in the daily denial of In the past year I’ve been reminded of death that psychiatrists say shores us up. my childhood fascination with the Sioux, This is why some people demand surgery and specifically their cultural distaste for the instant their aneurysm is diagnosed, fear of death. When death was imminent, no matter how small it is, no matter how their custom wasn’t to scream or run. unlikely it is to kill them; they will do They sang. Let’s face it: To let fear of anything to allow them to deny death death rule you is cowardly. I can’t escape once again. death, but I can show it — with a pat of In my case I suspect that denial was butter or harder swim than is recom- stronger than average, because of the mended for me now — that it no longer triathlons I completed. The allure of has me . Reprinted from THE WALL STREET JOURNAL NOVEMBER 4, 2003 19

A common misconception among phy- replacement surgery in the 1950s and, at Knowledge Gap sicians is that aortic disease is rare, 95, still on staff at Methodist Hospital of when in fact it kills an estimated 25,000 Houston. Medical Ignorance Americans a year. That is a larger toll Medical News than that of AIDS and most kinds of can- cer. Another misconception is that little This isn’t doctors have Contributes to Toll can be done about aortic aneurysms. The been slow to absorb news of medical ad- reality is that improvements in diag- vances. Hungarian physician Ignac Semmelweis discovered in 1847 that nostic-scanning methods introduced since From Aortic Illness merely by washing their hands, physi- the 1980s, and greater experience with cians could avoid spreading infection. But aortic surgery, have vastly enhanced the medical leaders resisted his teachings, Many Doctors Don’t Realize ability to detect and repair aneurysms. and hand-washing didn’t gain wide Behind these misconceptions is an ano- acceptance for years. In the 1840s, rudi- Aneurysms Are Treatable; maly in the medical profession’s struc- mentary communication slowed the ture: There is no medical specialty dev- A Paucity of Specialists spread of knowledge. Today, many physi- oted to treating or educating others about cians complain they are so swamped by the aorta. information—from journals, drug and ‘I Could Have Saved Him’ Aortic disease “falls squarely between device companies, and continuing-educa- about four different specialties,” says tion courses—that they can’t absorb all of By KEVIN HELLIKER Eric Isselbacher, a cardiologist at Har- the latest news. And THOMAS M. BURTON vard Medical School. “There’s an educa- In the case of aortic disease, this tion gap among physicians about aortic problem is heightened by the lack of After aortic aneurysms struck her ex- disease, and this gap isn’t small. It’s blood-vessel specialists dedicated to husband and his brother, Debra Mc- huge.” getting out the word about aneurysms. Millan learned that the often-fatal condi- The depth of medical unfamiliarity Every other significant body part—brain, tion can be hereditary. So last autumn, with this illness became clear in Sep- heart, lungs, bone and so on—boasts its when pain suddenly ripped through the tember, following the sudden death of own specialty association. Fifteen-thou- chest of her 19-year-old son, she rushed Hollywood star John Ritter, 54. He suf- sand podiatrists in the U.S. focus on feet. him to an emergency room in Omaha, fered an aortic dissection, a tear in the The aorta is the River Nile of blood Neb., and told the doctor about the weakened wall of an aneurysm. Although vessels. It rises from the heart nearly to family’s medical history. dissection kills quickly by disrupting the neck, then descends through the chest But she says the ER doctor at Nebras- blood flow to major organs, the aneurysm ka Methodist Hospital dismissed her talk and abdomen, carrying blood for every that typically causes this event takes organ and limb. But only about 300 non- of aneurysms, which are bulges that can years to grow, during which time it can lead to lethal rup- surgical doctors in the U.S. specialize in be detected and removed. Yet in media tures in the aorta, blood vessels. Cardiologists are respon- interviews after Mr. Ritter’s death, doc- the body’s largest sible for the cardiovascular system, and after doctor described dissection as artery. Instead, the typically are very knowledgeable about doctor diagnosed rare and undetectable. the tiny coronary arteries that channel Ms. McMillan’s son, Such comments disheartened aortic ex- blood from the aorta back into the heart. Tyler Kahle, with a perts. “I’ve been distressed by the series But their training leaves many of them in minor lung infec- of physicians getting on the screen and the dark about aortic disease. tion and sent him calling this kind of death unpreventable,” A small corps of aortic experts from home, she says. In says Dianna Milewicz, director of medical various specialties are beginning to focus following days, the genetics at the University of Texas more attention on aneurysms. But there young man and his Health Sciences Center in Houston. “The is a long way to go, especially in medical mother visited their message should have been that John schools, where the experts say aortic family physician Ritter’s children should be screened for problems typically receive inadequate and returned to Tyler Kahle this,” adds John Elefteriades, chief of study. Nebraska Metho- cardiovascular surgery at Yale-New During eight years as a medical stu- dist. During each visit, she asked whether Haven Medical Center. It hasn’t been dent and resident at West Virginia her son’s continuing chest pain could be disclosed whether Mr. Ritter ever was University, obstetrician Devin Ciliberti connected to the family history of aneur- checked for aortic problems. says he rarely heard any mention of ysms. Each doctor answered no—but Despite the diagnostic and surgical aortic illness. “If it ever came up, it was without ever examining Mr. Kahle’s breakthroughs, recent academic studies like, ‘This goes at the bottom of your aorta, she says. suggest that there has been little or no list’” of possible diagnoses, says the Eight days after his symptoms began, improvement in a longstanding misdiag- physician, who finished his residency in the young man died of a ruptured aorta. nosis rate of about 35% for aortic dissec- 2001. In particular, research suggesting Ms. McMillan has filed a malpractice suit tions, compared with about 5% for heart that pregnancy heightens the risk of against Nebraska Methodist in state court attack. Even with rupturing abdominal dissection never came up, Dr. Ciliberti in Douglas County, Neb. The hospital has aortic aneurysms—a variety that tends to adds. denied any liability, saying it provided strike a highly identifiable group, men This all became relevant when 25-year- adequate care. over 60 with a history of smoking and art- old Julie Neal Lee came to The Women’s The Kahle case illustrates a deadly dis- eriosclerosis—studies have found a misdi- Hospital of Greensboro, N.C., last Nov- crepancy between the available medical agnosis rate of about 30%. The in-hospital ember. She was 37 weeks pregnant and in knowledge about aortic aneurysms and mortality rate from aortic dissection ha- extreme distress, but clearly not in labor, the ignorance of many front-line phy- sn’t declined in decades. Dr. Ciliberti says. He says he ordered sicians. The discrepancy contributes to “No physician can diagnose a condition tests for kidney stones, a pregnancy- what may be thousands of unnecessary he never thinks about,” observes Michael related high-blood-pressure condition deaths each year. DeBakey, an inventor of aortic aneurysm- called pre-eclampsia and anything else he 20 Reprinted from THE WALL STREET JOURNAL NOVEMBER 4, 2003

could think of. All proved negative. Hours stretch a vessel normally the diameter of years ago, his cardiologist in Las Vegas passed, and the young woman was a garden hose to that of a soda can—was called a private meeting with Mr. Kehe’s frantic with pain. captured by a comment a century ago by wife. “He took my hands in his hands, Finally, Dr. Ciliberti ordered a comput- medical pioneer William Osler: “There is looked me in the eyes and said there was erized-tomography, or CT, scan of Ms. no condition more conducive to clinical no hope—that Donald should tell his loved Lee. “Even then, I wasn’t thinking about humility than aneurysm of the aorta.” ones goodbye,” says Rowena Kehe. After aortic dissection,” he says. The scan, Today, this shouldn’t be true. Aortic a friend pointed Mr. Kehe, then 69, taken more than seven hours after she aneurysms don’t show up well on X-rays. toward Cedars-Sinai Medical Center in arrived, showed an aortic dissection. Dr. But the advent of high-tech scans—such Los Angeles, Sharo Raissi, that hospital’s Ciliberti performed an emergency as CT; abdominal ultrasound; magnetic- top cardiovascular surgeon, removed the Caesarean, saving the baby. But Ms. Lee resonance imaging, or MRI; and echo- aneurysm. A few months later, instead of died after aortic-repair surgery by cardiogram—have made aneurysms rela- telling his family goodbye, Mr. Kehe another doctor. tively easy to catch. (The scans cost from treated them to a Hawaiian vacation. Mr. Her parents, Harold and Robin Lee, $40 to $2,000, depending on the aneu- Kehe, now 71, is alive and well today. say they blame Dr. Ciliberti for failing to diagnose the problem sooner, but they haven’t gone to court. Dr. Ciliberti says, “I don’t think a quicker diagnosis would A Medical Orphan Arch Ascending aneurysm have saved her, but I don’t know for No medical sure.” He attended Ms. Lee’s wake and specialty is Root Thoracic funeral and says he has spent much of devoted to aneurysm the past year learning about aortic treating the dissection. Women’s Hospital declines to body’s largest comment. artery. Driving Progress Specialists drive most medical prog- ress, educating generalists and promo- Heart ting prevention. Twenty years ago, few Americans had heard of prostate cancer, Abdominal but urologists have spurred screening and Aorta aneurysm awareness campaigns, and U.S. deaths from that disease fell 21% between 1990 to Normal aorta Diseased aorta 2000. The aorta rises from the Aneurysms can develop With no comparable campaign, unfa- heart, then descends anywhere along the vessel. miliarity with aneurysms prevails in through the chest and Those in the abdomen tend to many emergency rooms and physicians’ abdomen, carrying blood strike men over 60; those in offices. Michael Giusti, 44, entered the for every organ and limb. the chest can strike anyone. ER at Methodist Medical Center in Source: Yale University School of Medicine

Peoria, Ill., one night in June 1998, com- Street Journal. Mikhaela Reid/The Wall Illustration by plaining of chest pain and asking whether his aorta should be scanned, says his rysm’s location.) Medical geneticists Classic Symptom wife, Kathy Schwindenhammer, who have identified high-risk groups in whom In some aneurysm cases, the knowl- accompanied him. For 13 years, he had the condition ought to be suspected. And edge gap is especially clear because been undergoing scans to monitor an with experience, surgeons have improved multiple cardiac doctors miss danger aortic aneurysm that previously hadn’t to roughly 90% the success rate of re- signs. Daniel Slaughter, a 37-year-old caused any symptoms and only now was placing damaged sections of aorta with father of four, entered Methodist Hospital approaching a dangerous size, she says. Dacron hose. of Indianapolis in May 2001, experiencing In the ER, two residents picked up a text- One obstacle to disseminating inform- chest pain radiating into his neck. That is book and began flipping pages before ation on the aorta is corporate profit. a typical symptom of aortic dissection. He concurring with the primary physician on Medical-device and drug companies, was bleeding into the sac around the duty that a scan wasn’t needed, she says. which are playing an increasingly large heart, a common consequence of aortic In fact, aortic experts say that any per- role in shaping continuing-education dissection. And an echocardiogram found son with an aneurysm who experiences seminars, tend to focus on products they that his aorta was 50% larger than significant chest pain ought to have a sell, such as coronary stents, which are normal, according to a hospital report. scan done. But the doctors at Methodist used to prop open clogged coronary ar- Yet the cardiologist who signed the Medical diagnosed Mr. Giusti with a teries. Industry hasn’t developed a echocardiogram report noted in it that pulled chest muscle and sent him home, comparable product for repairing aneu- the heart and aorta looked normal. A his wife says. He died there that day of rysms that is inexpensive or effective second cardiologist and a cardiac surgeon an aortic dissection. enough to replace most surgery. For never looked at the echocardiogram, The hospital has denied any negli- cardiologists trying to keep up with their according to subsequent written state- gence. But in 2002 it agreed to pay Ms. field, “pharmaceutical and device devel- ments they made in administrative Schwindenhammer $850,000 to settle a opment for the coronary arteries is where proceedings. A week after entering suit she had filed, Illinois state-court the money and glamour are,” says Methodist, Mr. Slaughter died. After records show. Harvard’s Dr. Isselbacher. learning in the autopsy room that the Twenty years ago, diagnosing aneu- Some heart doctors don’t even realize cause of death was aortic dissection, the rysms was extremely difficult, and that action can be taken. When an ec- cardiac surgeon called Mr. Slaughter’s surgery to repair the condition had a high hocardiogram—a scan of the heart and widow. “He said, ‘This probably won’t mortality rate. The fatalism that surrounding vessels—found a large aneu- help you now, but I could have saved surrounded the ailment—which can rysm in the chest of Donald Kehe four him,’” says Paige Slaughter. She has Reprinted from THE WALL STREET JOURNAL NOVEMBER 4, 2003 21

named the hospital and three doctors in a proceeding that Indiana requires before the filing of a malpractice suit. Methodist Missing the Signs Hospital denies any negligence. The high death rate from aortic disease is partly due to physicians’ unfamiliarity Dr. Elefteriades, the top aortic surgeon with the symptoms, the groups that face high risks, and available diagnostic and at Yale-New Haven, and Craig Miller, his surgical procedures. counterpart at Stanford Medical Center, say they are each asked about twice a Julie Neal Lee, 25, Sandy Morris, 13, month by lawyers for plaintiffs and died November died July 1998, defendants to review cases alleging 2002, Greensboro, Columbus, Ohio: malpractice related to aortic disease. In N.C.: Pregnancy is When her parents only about half are the doctors’ or hospi- a huge risk factor took her to the ER tals’ actions legally defensible, say the for women with with severe chest doctors, who are paid for their opinions aortic disease. But pain, they knew but typically don’t testify in court. Both her obstetrician enough to request a physicians say that doctors’ performance says his medical high-tech scan. But in heart-attack cases they review is training barely the hospital didn’t defensible far more often. touched on the scan her aorta; four Banding Together condition. hours later, she died. At some hospitals, including Massachu- setts General in Boston, cardiologists, surgeons and other physicians are ban- Eric Eshleman, 28, Daniel Slaughter, 37, ding together to form aortic centers that died September died May 2001, draw on a range of specialties. In June, 2000, : He Indianapolis: He the American Heart Association entered the ER with entered the hospital published in its journal, Circulation, an sudden back pain. with symptoms of article on heredity and aortic aneurysms. His lanky dissection and a Still, AHA President Augustus Grant appearance classic says, “I don’t think aortic dissection is signaled he might consequence: blood analyzed with the frequency it should be” have Marfan’s, a in the sac around his at cardiac conferences. condition that heart. Yet he went Aortic disease generally strikes two makes the aorta undiagnosed for a types of victims. The first are men typi- prone to dissect. week. fied by James Whitehead, a professor who at 67 had a long history of smoking and high blood pres- sure. This August, he experienced sudden, severe back pain. His wife, Britt uled an MRI scan but for sudden, intense pressure in his chest, Eshleman, says it was the first time she morning, about eight hours after Sandy radiating into his jaw. At Washington had ever seen him cry. Neglecting to scan had arrived at 11 p.m. She didn’t live that Regional Medical Center in Fayetteville, his aorta, the hospital prescribed long. “Why don’t they do something, Ark., he tested negative for a heart attack painkillers and sent him home, his wife Daddy?” were the last words Andrew but remained stricken by pain so intense says. Seven days later, he died of an Morris says he heard his daughter speak. that morphine failed to numb it, his aortic dissection. The county autopsy Children’s Hospital has settled a family says. Eight hours after his arrival, report describes him as “marfanoid malpractice suit filed by the parents in doctors did the CT scan that revealed he appearing.” state court in Columbus on terms that had an aortic dissection, and by then, it Ms. Eshleman has sued Northside for weren’t disclosed. The hospital declines was too late, his family says. He died malpractice in state court in Fulton to discuss the case. In 2000, Children’s before reaching the operating room. County, Ga., alleging that based on her Hospital and the Washington Regional declines to husband’s body type, among other Medical Center, which share faculty, comment. factors, the hospital should have tested opened a Cardiovascular Connective The second type of aneurysm victims more aggressively for aortic dissection. Tissue Disorders Clinic. That unit serves are young, fit people cursed with a The hospital says the suit “is without patients with Marfan’s and others who genetic predisposition for aortic prob- merit.” have a genetic predisposition to develop lems. Most people in this category don’t The seriousness of aortic dissection is aortic disease. know they have an aneurysm, although lost on many doctors. Sandy Morris, 13, family history can provide a clue. arrived in July 1998 in the emergency Extreme Pain So can body type. Aortic experts say room at Ohio’s Columbus Children’s Aortic dissection is one of few condi- that especially tall, lanky people entering tions that causes pain so severe it often an emergency room suffering sudden and Hospital, complaining of intense chest isn’t relieved by morphine, experts say. intense chest or back pain ought to be pains. Her parents knew their daughter Even so, after doctors rule out heart considered possible aneurysm victims. had Marfan’s, and they say they knew People with strikingly long limbs may the pain might indicate an aortic dissec- attack, they sometimes neglect to test have Marfan’s syndrome, a connective- tion. They even knew enough to request patients experiencing this level of chest tissue disorder, and Marfan’s sufferers an echocardiogram. But doctors failed to or back pain for aortic problems. statistically have a much-greater-than- do one, testing Sandy instead for heart Christopher Cole, 39, a manufacturing average risk of dissection. attack, the Morrises say. That test came executive in Elyria, Ohio, once broke his Eric Eshleman, 28 years old, 6-foot-8 back negative, because Sandy was having leg in six places in an amateur motor- and 190 pounds, entered Atlanta’s North- an aortic dissection, the parents say. cycle race. His foot ended up pointing side Hospital in September 2000 suffering Court records show that doctors sched- backward, he says. On another occasion, 22 Reprinted from THE WALL STREET JOURNAL NOVEMBER 4, 2003

the South native was hit by that went on to huge success. Yet aneurysms. An article scheduled to shrapnel while serving in that country’s Diane Sixsmith, one of the physicians appear in the winter issue of the Annals military in the 1980s. The pain from his charged by New York state medical of Emergency Medicine describes the aortic dissection 14 months ago was far authorities with investigating the case, failure of three sets of medical personnel worse than from either of those injuries, concluded no negligence occurred. Mr. in Omaha to scan the aorta of Mr. Kahle, he says. “When my heart would beat, it Larson had complained only of flu-like the 19-year-old whose mother rushed him felt as if my skin was tearing,” he says. symptoms, and it would have been a huge to the emergency room and told doctors But it took doctors an alarmingly long leap to guess that he had a disintegrating about the family’s medical history. time to conclude that anything was wrong aorta, says Dr. Sixsmith, chairman of “Scanning him very likely would have with his aorta. When he arrived at Elyria emergency medicine at New York saved his life,” says Dr. Milewicz, the Memorial Hospital, near Cleveland, in Hospital Medical Center and a University of Texas genetics expert who August 2002, doctors and nurses ran leader in efforts to educate physicians co-wrote the journal article. various tests, but not a scan that would about aortic disease. In August 2001, about a year before Tyler’s death, his uncle, Tom Kahle, had have shown the dissection, he says. Mr. Pregnant Patient Cole stayed overnight at the hospital, and entered St. Luke’s Hospital in Cedar Ra- the next morning a cardiologist told him Many aortic dissections and ruptures pids, Iowa, complaining of chest pain. He they couldn’t find anything wrong and he involve aneurysms that doctors spot but told doctors about his family’s history of could go home. Mr. Cole did, but his pain fail to treat. An echocardiogram picked aneurysm, relatives say. But the hospital grew worse. It took two more visits to the up Lori Irving’s aortic aneurysm in 1998, discharged him without scanning his ER the next day before doctors finally her mother, Patty Irving, says. But her aorta, the relatives add. Two days later, gave him a CT scan. When that showed a cardiologist, who was employed by Kaiser Tom Kahle, 37, died of an aortic dissec- dissection, he was flown immediately by Permanente, said nothing about it, the tion. His family has filed a negligence helicopter to the Cleveland Clinic. Lars G. mother adds. The younger Ms. Irving, a suit against St. Luke’s in state court in Svensson, the clinic’s chief aortic psychology professor at Washington State Linn County, Iowa. The hospital has surgeon, performed successful emer- University in Vancouver, Wash., was denied any liability. gency surgery. then 35. In mid-2000, she became preg- Terry Kahle, Tom’s brother and Dr. Svensson says Mr. Cole probably nant. Aortic experts say that any woman Tyler’s father, survived a dissection in wouldn’t have survived more than of child-bearing age who has an 1998. After attending Tyler’s funeral in another two hours without it. The surgeon aneurysm should be warned that preg- Omaha last year, Terry Kahle returned to estimates that every second or third nancy severely compounds the dangers. his home in Atlanta with his older son, aneurysm case he gets was originally “We’d never have gotten pregnant if we’d Marcus, 23. Almost immediately, Marcus misdiagnosed. An Elyria Hospital known about the risk factor,” says Mike started complaining of chest pains. “I spokesman declines to comment. Morgan, Lori’s husband. figured it was the power of suggestion, Aortic dissection and rupture are fatal When intense chest pain sent Ms. but I wasn’t taking any chances,” the far more often than heart attack. As a Irving to the emergency room at South- father says. result, some doctors are aggressive about west Medical Center, a Kaiser Perma- Rushing his son to the emergency testing for aortic disease. When Howard nente hospital in Vancouver, during the room at St. Joseph’s Hospital in Atlanta, Carney entered St. Luke’s Hospital in last month of her pregnancy in April of Mr. Kahle says he requested a scan of the Kansas City, Mo., last year, complaining 2001, she had no way of knowing the young man’s aorta—only to be told that of sudden, intense chest pain, Dr. Lance cause. Doctors didn’t take an echocardio- aortic disease didn’t strike people that Waldo immediately ordered a CT scan gram, her mother says. They diagnosed young. Mr. Kahle, an auto technician that showed an aortic dissection. Mr. the 38-year-old patient with indigestion who says he had never stood up to a Carney, 36, underwent emergency and sent her home, her mother says. doctor before, did so then. surgery and today is fine. “I’m paid to be That same day, Lori Irving and the “There were tears in my eyes,” he a pessimist,” says Dr. Waldo. unborn baby died. says. “I said, ‘Listen, I just buried my 19- Not every case of aortic rupture or Kaiser declines to comment, citing a year-old son last week, and I buried my dissection can be diagnosed. Composer settlement and confidentiality agreement brother last year—both of them aortic Jonathan Larson died of an aortic dissec- with Lori Irving’s husband. aneurysms. We’re not leaving here until tion in 1996 after two New York City you scan my son.’” hospitals misdiagnosed him. The 35-year- Father and Son After getting scanned, Marcus Kahle old’s death drew widespread attention Some physicians hope that the story of underwent emergency surgery to repair because it came after the final dress Tyler Kahle’s family could help educate an aortic aneurysm. Today, he is alive rehearsal of his show “Rent,” the rock the profession about the dangers of and well in Atlanta. Reprinted from THE WALL STREET JOURNAL NOVEMBER 21, 2003 23

Health Which Test Should You Get? Mike Agliolo/Photo Researchers, Inc.

By KEVIN HELLIKER diogram. The not-so-great news is that Fortunately, my internist knew this, and these scans aren’t perfect at determining he ordered up a third test — an MRI. This With all the CT scans, “echo” scans size — and size is paramount when it differed from the original CT in that it and MRIs, it isn’t always easy figuring comes to aortic aneurysms. Worse yet, involved 90 minutes of image-taking, out which test is best — as our reporter human error can render any of these instead of just a snapshot. In addition, I found out the hard way. scans useless. had contrast, or dye, inserted into my veins “What this underscores is that as a — a factor that bolsters reliability. SERIES OF SCANS taken last autumn consumer, you’re building a case,” says The MRI confirmed the presence of an Aof my aortic artery concluded that I Michael Dake, chief of interventional radi- aneurysm but at 4.1 centimeters. A subse- faced either grave danger — or none at ology at Stanford University School of quent echocardiogram (this time adminis- all. Aneurysm! Warning! A whopper! Medicine. Building a solid case may re- tered by a pro) validated this finding, as concluded the first test I took, a CT scan. quire more than one scan and more than did a second MRI. Chill out — there’s no aneurysm, one radiologist. So I’m proof that newfangled scans can concluded the second scan, an echocardio- The first scan I received was just a detect a condition that not so long ago was gram. snapshot of my heart, taken to search for undetectable, and that is more common You’re both wrong, said the third, an calcium in the coronary arteries. It found than most doctors realize, killing more MRI with contrast: There IS an aneurysm none of that, but caught a glimpse of the Americans each year than does AIDs and — but it’s not a whopper. nearby aortic artery — and what a most kinds of cancer. For me, the drill now This debate would have been fasci- whopper it appeared to be: 4.6 centime- is to keep an eye on the aneurysm through nating to witness if the artery in question ters, or nearly two centimeters too large. twice-annual scans, looking in particular hadn’t belonged to me. Or if the artery Meaning: I had an aneurysm nearly large for growth. If the bulge reaches a certain had been a minor vessel — say, the one enough to be in danger of bursting. Yikes! size — say, five centimeters — then the that carries blood to my little toe. But this Except my internist wasn’t convinced. risk of rupture will be greater than the risk was my aorta — carrier of blood to every To make sure, he ordered up a second of open-chest surgery to repair the organ in the body — meaning that this scan, this one an echocardiogram. When aneurysm. battle of the scans bore life-and-death this test came back negative — No But can I rest assured that it isn’t five implications. Watching the scans bounce aneurysm at all! — I did a little dance. centimeters now? Often, people who under- my prognosis from grim to great and Prematurely, as it turned out. Echocar- go aneurysm-repair surgery are told after- back, I was bewildered. diogram, it seems, is highly operator ward that the bulge was much larger than This experience wasn’t unusual. The dependent. In the right hands, it can be any scan had shown. But Stanford’s Dr. great news is that aortic aneurysms — an more accurate than CT or MRI, and it Dake is skeptical about some of these tales. often-fatal condition that never showed up has the added advantage of emitting no Quoting a former radiological mentor, he well on X-ray — are captured easily on radiation. In the wrong hands, however, says, “There’s no such crude diagnostic in- new scans such as CT, MRI and echocar- it’s worthless. strument as the surgeon’s hand and eye.” 24 Reprinted from THE WALL STREET JOURNAL DECEMBER 23, 2003

Health Journal/By Kevin Helliker 7 A Mother’s Mission: When You Just Know the Doctors Are Wrong CHILD BORN WITH A RARE ail- Michael was diagnosed as “hypotonic.” Ament can only hope for a mother as This meant her now four-year-old had low persistent as Karen Murray. The instant muscle tone. Since it shed no light on why her son was born she noticed he seemed he had low muscle tone, the diagnosis different. Her doctors shrugged, saying was meaningless but benign. Why not he was fine. But something was wrong, stop worrying? She heard this from her and it took her five years to get the husband, her doctors, her friends. answer. Only her own mother believed her. It’s a condition five times more com- Once, in the pediatrician’s office, Ms. mon than cystic fibrosis and similarly Murray’s mother asked whether exces- fatal. Whereas doctors offer pregnant sive height could indicate trouble. The women screening for multiple conditions pediatrician’s response: “Worry if your such as CF, this one gets almost no atten- child doesn’t grow.” tion, for the scariest of reasons: The The breakthrough came on Michael’s average doctor knows next to nothing fifth birthday. His mother bought him an about it. Apple computer, and with it came a CD Karen Murray and her son Michael, This wouldn’t matter if the condition called Family Doctor. Instead of sleeping who has Marfan’s Syndrome. were untreatable. But early diagnosis of that night, Ms. Murray sat in his dark this condition boosts life expectancy to room, scrolling down a list of unusual dis- nearly 70 years from about 40. Indeed, orders. In its classic presentation, Marfan’s is this condition is significantly more treat- When she came to Marfan’s Syndrome, a series of symptomatic non-life-threat- able than many ailments doctors regu- the description floored her. “I can still ening conditions that collectively point to larly seek to find. But most doctors aren’t recite it: A connective-tissue disorder. a treatable fatal problem that will exhibit looking for this one, and don’t recognize Fingers contracted backwards at birth. no symptoms until too late. Marfan’s af- it when they see it. Taller than their peers. Arms and legs flicts about 200,000 Americans, compared Ms. Murray’s son had a litany of puz- disproportionately long. Indentation in with about 30,000 with cystic fibrosis. But zling symptoms. Newborns’ hands us- chest bone. Weak lung tissue.” fewer than half of Marfanoid Americans ually are balled into fists. But his hands Then came this news: The aorta, the know they have it, estimates Reed Pyer- were open — his fingers bent backward. body’s largest artery, can grow weak, en- itz, chief of medical genetics at the He had a dislocated hip. Why, she asked. large and rupture, causing sudden death. hospital of the University of Pennsyl- It means nothing, the doctors said. Just That morning Ms. Murray took Michael vania. Many of these cases won’t be diag- keep him in a harness for a while. to Mount Sinai Hospital. After measuring nosed until the victims die young and His name was Michael and she wanted his arms and legs, doctors agreed he had suddenly of an aortic weakness that could him to be perfect. But Ms. Murray, 35 at Marfanoid characteristics. They have been repaired. the time, hadn’t risen to the top ranks of suggested that he return in three weeks In part this failure to diagnose reflects Inc. by ignoring imperfec- for an echocardiogram of his aorta. Ms. the absence of a simple screening test. tions, and she wasn’t about to start with Murray demanded it now. “I was crying But when a fashion executive on a home her child. hysterically,” she says. computer makes a diagnosis that New “He was floppy,” she says. As a tod- When the doctors emerged from the York City’s finest pediatric professionals dler, he couldn’t sit straight in a high scanning room, “they were white as a missed, clearly physician cluelessness is chair. His limbs and fingers were extra- ghost,” she says. They told her she had part of the problem too. That cluelessness ordinarily long and bendable, as if made been right to seek an immediate scan. extends beyond Marfan’s to all aortic of rubber. His chest was deeply indented. Michael’s aorta was twice normal size. disease, which kills more Americans each He was dramatically taller than other He was immediately prohibited from year than do AIDS and most kinds of can- children. Yet unlike his father, her broad- engaging in strenuous activity. He was er. Many of those deaths are attributable shouldered husband, Michael was thin, also placed on beta blockers to lower his to a failure to diagnose. even flimsy. Also, his lungs became in- blood pressure and reduce strain on his Which diseases get medical attention fected every time he caught a cold. aorta. is partly a matter of fashion. Marfan’s A college-educated New Yorker with The treatments seem to be working, has never been fashionable. But Ms. deep professional and personal resources, since the artery hasn’t grown in seven Murray, president of menswear for Liz Ms. Murray sought out and visited the years. In most Marfan’s patients the Claiborne, is changing that. Each year in city’s top pediatric specialists. She took enlarged section of aorta eventually the company’s designer showroom, she Michael to see an orthopedist, an ortho- needs to be replaced with synthetic hose, hosts a star-studded fund-raiser for the pedic surgeon, an occupational therapist, but in children the hope is to forestall that National Marfan Foundation, each event a joint-disease specialist, even a neuro- very serious surgery until adulthood, says raising more attention and money than surgeon. Not to mention his regular Harry Dietz, a geneticist at Johns Hop- the last. For more information, contact doctor, “the best pediatrician at Mount kins University Hospital in Baltimore who the foundation at 1-800-8-Marfan, or Sinai,” says Ms. Murray. now is Michael’s doctor. www.Marfan.org. Reprinted from THE WALL STREET JOURNAL DECEMBER 30, 2003 25

The Surgery Your Doctor Shouldn’t Perform

Despite this gap, general surgeons dure has gained steam in recent years Vascular Procedure Carries perform about 30% of the 60,000 abdom- because of the advent of new imaging Greater Risks When Done inal aortic aneurysm surgeries each year tests such as ultrasound and CT that can in the U.S. While generalists also do a glimpse the arteries and foresee events By General Surgeons variety of other surgeries, research in such as ruptured aneurysms, which once those areas hasn’t suggested such a were considered unpredictable forces of By THOMAS M. BURTON disparity of results between the general- nature. ists and the specialists. With abdominal Many general surgeons, of course, are HOUSANDS OF AMERICANS are aortic aneurysm surgery, the public is highly competent, and some have done at risk of dying needlessly each largely unaware of the differing success extensive aneurysm repair operations, Tyear because general surgeons rates. gaining significant expertise in the pro- are being allowed to perform an espe- Vascular surgeons argue that the cess. Dr. Veith and others say such sur- cially precarious type of surgery. The reason they have met opposition from the geons should be eligible to qualify for a surgery in question involves replacing a ABMS board is cronyism. A change in full vascular certificate if one is created. weakened and enlarged section of aorta, policy could take hundreds of millions of But studies show that many general the body’s largest and most vital artery, dollars a year from the pockets of gen- surgeons who do vascular operations with synthetic tubing. eral surgeons who exert considerable only periodically, and with inferior re- A growing body of medical literature influence inside the specialties board. sults, continue to do them. (Cost isn’t an suggests that only highly trained issue because most aortic-aneurysm vascular surgeons should, in the repairs are done on Medicare majority of cases, be allowed to patients, and Medicare sets the same perform the surgery. Because it Is Your Surgeon Qualified? rate for all surgeons.) Physicians on requires the surgeon to close down a Questions you should ask a doctor before both sides of the issue concede that section of the aorta—akin to getting an aneurysm the patients of general surgeons replacing a fuel hose in a plane at operation: generally aren’t aware of the studies 30,000 feet—it has a relatively high n Are you a full-time associating better outcomes with mortality rate. vascular surgeon? vascular surgeons. But despite a growing cry by vas- You’re looking for a Certainly Daisy Storry wasn’t. cular specialists to limit general “yes” answer here. After an ultrasound test discovered surgeons’ ability to perform the ab- an aneurysm in the abdominal por- dominal aortic aneurysm surgeries, n How many aneurysm tion of her aorta, she went to a gen- no such potentially life-saving operations do you do eral surgeon in Brainerd, Minn., for restrictions are planned in the short- every year? elective repair in December 2001. term. At least 15 is desirable. Medical records show that the 74- Vascular specialists want the year-old woman’s blood pressure American Board of Medical Special- n How many major plummeted during surgery. Her rela- ties, an influential professional vascular operations do tives, who say the surgeon told them group, to make vascular surgery a you do annually? that her prognosis was poor and that separate specialty, with its own cer- Over 50 is reassuring. PictureQuest he had done everything he could, tificate of expertise. Doctors say this insisted she be helicoptered to a would lead most hospitals to decide Minneapolis hospital 130 miles away. that only vascular surgeons should The Medical Specialties board “is an There, records show, doctors found “a perform nonemergency vascular proce- old-boys’ club in which professional self- huge amount of blood present” and dures, just as only neurosurgeons can do interest, not patient care, [is] the over- “blood poured out in a very large quan- surgery on brain aneurysms, and only riding consideration,” says Frank J. tity” from veins and arteries near where cardiac surgeons can operate on the Veith, vice chairman of surgery at Albert the Dacron tubing was sewed in. Mrs. heart. Einstein College of Medicine in New Storry died. The issue has been heating up in York, and himself a vascular surgeon. Her son, Robert Storry, says the fam- recent months. Last year, a committee “Better results are achieved by those ily had questioned the doctor prior to sur- that has significant representation from who specialize in vascular surgery.” gery whether he could handle it and had the ABMS rejected a request by the The American Board of Medical been told the operation would be “a piece vascular surgeons to create the new Specialties, one of medicine’s least- of cake.” specialty, a decision the vascular sur- known and most-powerful organizations, There is no guarantee Mrs. Storry geons later appealed. Letters and memo- declines to comment on the vascular- would have lived had a vascular surgeon randa from both sides have been pouring surgery issue. performed the procedure. But “obviously, into the ABMS offices in Evanston, Ill. A change in policy, of course, would we would have gone to a vascular sur- The overall mortality rate from also be a business windfall to the vas- geon had we known the difference,” says abdominal aortic aneurysm surgeries cular surgeons, but they insist the moti- Mr. Storry, whose family has filed suit. averages about 5%. But when general vation is successful medical outcomes, The surgeon, James J. Dehen, didn’t surgeons perform the surgery, the not profit. respond to calls for comment. Dr. mortality rate is 76% higher than when Abdominal aortic aneurysm surgery, Dehen’s attorney, Barbara Zurek, said: vascular surgeons do it, according to a most common in men over 60, is per- “Having an elective aneurysm repair recent /Johns formed to stop a swelling within the carries a statistical risk of mortality in Hopkins study of 3,912 cases. Other aorta that, if unaddressed, can eventu- the range of 5%, and Dr. Dehen’s statis- studies have reached similar findings. ally cause it to rupture fatally. The proce- tics are better than that.” 26 Reprinted from THE WALL STREET JOURNAL DECEMBER 30, 2003

Speaking about the broader debate, David H. Nahrwold, president of the ABMS, declined to discuss the vascular surgeons’ campaign. But he says that, generally speaking, one downside of limiting certain medical therapies to specialists is doctors’ availability for patients. Leading vascular surgeons argue that there are enough vascular specialists to do the necessary operations. Currently, there are about 2,100 vascular surgeons in the U.S. Moreover, they say, if vas- cular surgery becomes an independent specialty, it could lure more medical resi- dents into the field. The findings of the University of Mich- igan/Johns Hopkins study have been supported by other evidence. The Wall Street Journal looked at Pennsylvania’s uniquely extensive database of medical records compiled by its Health Care Cost Containment Council which produces public reports on the quality of medical care. The Journal worked with Michael Pine & Associates which helps Pennsyl- vania evaluate its hospitals. The re- search took into account the medical severity of each case. The finding: In 5,128 abdominal aortic aneurysm operations in Pennsylvania over three years, general surgeons had a 73% higher mortality rate than did vascular surgeons. Dow Jones Roster of PULITZER PRIZE-WINNING JOURNALISM From The Wall Street Journal

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