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an online version of the magazine Fall 2009
Medical Rounds
Medical Rounds
Pancreatic cancer beware: Schulick, left, and radiation oncologist Joe Herman
Photo by Keith Weller
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Defeat for a Cancer Most Cruel

Could ganging up against pancreatic cancer be changing the game?

 

Patty Gardon, 43, felt good as she scurried about her nursing duties before a colleague stopped her in the hallway. “Are you okay?” her friend asked. “Your face looks yellow.”

Gardon and her friend both served on a ventilator unit in upstate New York. At first they suspected jaundice. But an ultrasound and CT scan soon found something much more ominous—pancreatic cancer, one of the most assuredly fatal tumors known.

With two young kids at home, Gardon reeled at the diagnosis. Then she got into the fight. A surgical team at a nearby hospital went in on February 1, 2007, providing an “all’s well” update halfway through. But hours later they declared defeat. The tumor was too entwined with the critical portal vein, doctors said. One surgical nick could induce a fatal hemorrhage. They were closing her up and withdrawing, with the edict: “It’s in God’s hands now.”

Gardon and her family had great religious faith, but they also placed their faith in second opinions. They found a circuitous route to Johns Hopkins, where surgeon Rich Schulick reviewed her scans and records and then called with a welcome message. “Why don’t you come down to Baltimore and let our multidisciplinary pancreatic group meet with you?” he said.

She and her brother soon joined a group of six other pancreatic cancer patients, all gathered in “a room filled with hope” on the Hopkins medical campus. In a ritual performed every Tuesday, Schulick and 19 other Hopkins specialists reviewed each case. Later in the day, each patient received an authoritative answer. On that February 27, all six learned the exact plan of action proposed by the multidisciplinary team.

First, Schulick said, Gardon needed radiation and chemotherapy to get the tumor down to a manageable size “and to get the tumor to show its true colors.” When Gardon returned to Baltimore in the spring, Schulick et al, studied her new scans. He was pleased. “I think I can get it out,” he told her. Gardon was stunned.

Before surgery, Gardon asked Schulick for “one small favor. If you get it out,” she asked, “could you whisper those four words in my ear?”

In the operating room, Schulick and his team of seven found Gardon’s tissues scarred and toughened from the chemo and radiation treatments. Despite the difficulties, he was able to completely remove the cancer and the surrounding lymph nodes. Before leaving the suite, Schulick bent down to his unconscious patient and whispered: “I got it out.” Even from her anesthesia-induced delirium, Patty Gardon roused. “I love you!”

Now two years post-op, Gardon remains cancer-free. She has returned to work. She’s been busy prepping her son for 7th grade and her daughter for college.

Schulick sees the case as exemplary of a decidedly more aggressive commitment to flood the pancreatic cancer problem with all of the expertise available. Though surgical techniques have changed little in recent years, Schulick says, “we now know how to better select those whom we can help.” He rattles off the wide range of specialists involved in each patient’s case—including medical oncologists, radiation oncologists, immunologists, geneticists, and coordinating patient advocates.

“We live, eat, and breathe the destruction of pancreatic cancer,” he says. “It’s a terrible cancer,” he says, “so unless we throw everything at it, we will never beat it. But if we are going to make progress, this is the model.” Ramsey Flynn


Under Pressure

Less invasive approach leaves infant’s skull in great shape.

 

Under Pressure
Ahn makes two tiny incisions (top) to give access to the sagittal suture. Below: A rounder skull, post surgery.
Illustration by Tim Phelps,
Art As Applied to Medicine

When Kristie Palmer discovered last spring that her newborn daughter’s head was misshaped and would require surgery to correct, she called a children’s hospital near her home in Pittsburgh. They couldn’t see her until the end of May. Then Palmer learned that Hopkins pediatric neurosurgeon Edward Ahn specialized in treating the condition, called craniosynostosis. Afflicting 1 in every 2,000 newborns, it is caused by a premature closing of one or more of the sutures of the bones that make up the skull.

Ahn takes a minimally invasive approach, which would mean less blood loss than in the open procedure and only one or two days in the hospital instead of a longer intensive care stay—as well as potentially better outcomes in the shape of the skull. Palmer called and two days later 3-month-old Addison was being evaluated in Ahn’s office. “Her head was changing so fast that a month seemed a long time to wait,” says Palmer. “And the hospital here didn’t offer the less-invasive operation.”

Palmer learned that had she waited another week or two, Addison might not have been eligible for the minimally invasive procedure. The earlier the surgery after birth, Ahn explains, the better the cosmetic outcome. But neurosurgeons often delay the traditional, open operation until infants are at least six months of age because of anesthesia and bleeding risks. The minimally invasive approach allows them to perform the procedure quickly, usually within one hour, and early, preferably when the infant is less than three months of age. “The sooner you release the restriction that the craniosynostosis is causing,” says Ahn, “the sooner you allow the brain and skull to develop as they normally would.”

That restriction—the skull suture that has prematurely fused—Ahn adds, is commonly the sagittal suture. In his minimally invasive approach, only two tiny incisions are made, allowing pediatric neurosurgeons to see the suture with an endoscopic camera and to remove it without major dissection. “Using the endoscope, we can create a space beneath the skull to remove the suture safely and without a lot of dissection,” says Ahn, noting that studies have shown that only 10 percent of patients undergoing the minimally invasive approach require blood transfusions. “In the open procedure, I would argue that 100 percent of patients receive transfusions.”

Addison was scheduled for her procedure but then another potential obstacle came up: Her preoperative blood screening pointed to a clotting disorder that proved to be Factor VII deficiency. The extremely rare bleeding disorder—1 in 500,000—was confirmed by pediatric hematologist Shirley Reddoch. “We knew that the [bleeding] risk was increased with this identified factor deficiency, and this procedure, even though it was minimally invasive, was not without some bleeding risk of its own,” says Reddoch. “Since we were also pressed by the need to do the surgery for this baby at this time, we had to come up with a plan, quickly.”

Reddoch recommended scheduled infusions of a coagulation product for specific factor replacement—recombinant Factor VIIa (rFVIIa)—beginning just prior to the start of the procedure and ultimately continued for a period of 72 hours, allowing Addison’s blood to clot normally during and after the operation. Addison did receive one red blood cell transfusion following the surgery but no other blood products were necessary. She was also observed closely for a few days following the surgery, but there was no need for further infusion. The plan worked.

“The interesting thing,” says Ahn, “is that with the help of our hematologists we were able to offer minimally invasive cranial surgery on a baby with a clotting disorder. Had she not come to us early, she would have had to undergo the large open procedure, receive a number of blood products, and undergo a lot of blood loss.” Gary Logan

 



Anxious No More

A family-based therapy program shows success in anxiety-prone kids.

 

Anxious No More

Associate professor of psychiatry Golda Ginsburg has devoted her career to studying anxiety in children, and is well aware of the data: Up to 65 percent of children living with an anxious parent meet the criteria for an anxiety disorder themselves. “Knowing that anxiety runs in families,” she says, “I wondered if one could develop preventative measures for these children.”

She and her team at the Hopkins Children’s Center appear to have found a promising approach: a family-based program using cognitive behavioral therapy. In their Child Anxiety Prevention Study (CAPS) of 40 children between 7 and 12, the team members found that as few as eight weekly sessions went a long way toward preventing or minimizing the psychological damage of childhood anxiety. The researchers reported their results in the June issue of the Journal of Consulting and Clinical Psychology.

“We were looking for children of anxious parents who showed signs of anxiety but who had yet to develop a full-blown anxiety disorder,” says Ginsburg. Half the children and their families were enrolled in an eight-week, hour-long session of cognitive behavioral therapy, in which parents learned to identify and change their own anxiety-producing behaviors. In addition, the children learned to recognize and overcome their own problems. The other half of the study group, which served as a control, received no therapy at the time, but were offered therapy a year later.

Within a year, 30 percent of the children in the no-intervention group had developed an anxiety disorder, compared to none who participated in the family-based therapy. Moreover, the children in the therapy group saw a 40 percent reduction in anxiety in the year following therapy. There was no such reduction among children on the waiting list.

Ginsburg says prevention of childhood anxiety is critical because it affects one in five U.S. children, but often goes unrecognized. Delaying diagnosis and treatment can lead to depression, substance abuse, and poor school performance throughout childhood and well into adulthood. She and her team have now launched a larger five-year study involving 100 families. Jennifer Wilkinson


Dizzy, Falling—and Diabetic?

 

A landmark Hopkins study about balance disorders published last spring found that nearly a third of all Americans suffer from vestibular problems that can wreak havoc with balance. Most of these people are over 40, and many of them are unaware of their risks.

This has dramatic implications for an aging population, says lead investigator Yuri Agrawal. Falls, she says, have become a “hot topic” in geriatric medicine. “The average life expectancy after a fall can be on the order of months, so it’s really a catastrophic event in a person’s life.” Just from the falls themselves, Agrawal says, about 13,000 American seniors die every year.

But the three-year survey went deeper. In results published in a May issue of Archives of Internal Medicine, Agrawal and her Hopkins colleagues tapped data from 5,000 men and women over age 40. They found that 85 percent of people over 80 had an imbalance problem—23 times more than people in their 40s.

Agrawal’s group also outed a little-known culprit behind these growing vestibular disorders. To wit: Among the 69 million Americans found to suffer balance problems, a full 16 percent are also diabetic. This is twice the level expected, says Agrawal. Which naturally brings up the question: Why is this disorder picking on diabetics with such a vengeance?

Agrawal suspects the problem stems from diabetes’ known propensity for harming so many of the body’s cellular tissues. A diabetic’s high sugar levels, she explains, damage the fine hair cells in the inner ear that facilitate balance control. They also damage the inner ear’s small blood vessels.

Many vestibular disorders can be addressed with rehabilitation techniques, notes Agrawal, but she also recommends safety measures in the home, such as installing guard rails along stairs or hallways where a fall might occur, making sure rooms are well lit, and removing carpeting in places where people are more prone to trip. Ramsey Flynn


Eye-Opener

How a Hopkins duo mapped uncharted territory to advance the fight against glaucoma.

 

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Eye-Opener
Quigley: tracking change in iris volume to better treat glaucoma.
Photo by Keith Weller

When Harry Quigley set his sights on a better way to fight glaucoma, he knew it was time to think beyond his specialty. The secrets behind the particular form of glaucoma were locked within the eye’s fluid interior, where the aqueous flowed, or failed to flow, amid the ocular structures. Quigley knew he needed a fluids expert.

Enter David Silver, a Johns Hopkins Applied Physics Laboratory scientist with a passion for fluid dynamics. Soon after linking up with Silver, Quigley knew he had found the help he needed. “David not only was willing to give me advice,” Quigley recalls. “He just practically jumped into doing a whole bunch of geometric modeling.”

The condition that intrigued the duo was Angle Closure Glaucoma, or ACG, which blinds 16 million people worldwide. ACG takes its name from the choking of the intraocular canal through which fluid moves from the eye’s inner chamber into a drainage channel. The closure of that canal can trap aqueous within the inner chamber, allowing pressure to build to a point where nerves at the back of the eye suffer permanent damage. Ophthalmologists can make a hole in the iris to prevent pressure build-up, a procedure known as an iridectomy, but have learned that only one in five people with that structural anomaly will actually suffer the effects of ACG.

If Quigley and Silver could untangle the riddle behind why ACG affected some at-risk people and not others, millions might be spared an unnecessary procedure.

Early on, the duo focused on the mechanics of the iris, which contracts and dilates with changing light conditions. During one white board discussion in Quigley’s office at the Wilmer Eye Institute, Silver concluded, “The iris can’t work.”

“What do you mean the iris can’t work?” asked Quigley.

“Well, according to all of our models and formulas, it looks like everyone should have angle closure.” If the iris retained a constant volume as it dilated to allow more light onto the retina inside, Silver explained, the narrow angle at its outer rim would always be choked off in low light conditions, spiking everyone’s intraocular pressure to blinding levels. Then Silver proffered a more basic question: “Is it possible the iris could lose some volume somewhere?”

In the ensuing months, Quigley teamed up with optical engineers at Carl Zeiss to develop an imaging technology that could dynamically track the size of the iris in both light and dark conditions, providing data that allowed him and Silver to gauge the iris’s volume with each shift in light. What they learned could completely change the way scientists think of the iris. “It’s like a sponge,” says Quigley. It can absorb water and expel it, he explains, “and it loses volume rapidly with pupil enlargement.”

The startling thing, says Quigley, is that scientific interest in the iris goes back 4,000 years, but no one ever “knew it lost half its volume when the pupil gets big. So the fun here is that it’s a fundamental property of the human body that we didn’t know before, and it came about as a collaboration between a physical scientist and a clinical scientist.”

The new understanding quickly informed the duo’s attempts to more accurately identify the people who would suffer ACG: The ones really at risk, they found, were those whose iris tissues lacked the ability to adequately contract in volume with the pupil’s dilation.

Along with that breakthrough, Quigley and other researchers had identified further distinctions: ACG would harm more women than men, would most likely emerge after the age of 65, and would more likely afflict Asian populations than those of European or African descent.

Thus armed, Quigley and colleague David Friedman have taken these new ideas out into the world in a major trial now unfolding in China, involving 1,000 older Chinese who meet ACG’s more refined criteria. The outcome could save millions of people from needless iridectomies and focus on those who most need them instead. Ramsey Flynn

 
 
 
 
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