Thanks to Teamwork — and a lot of free pens — physicians and surgeons are treating pulmonary embolism faster and more effectively
By Beth Saulnier
When Joanna Cis's right leg started to ache, she didn't take it too seriously. Being seven months pregnant with her first child, the thirty-four-year-old dismissed it as another discomfort of the third trimester, as inevitable as it was unpleasant. "I thought the baby was pushing on a vein or something, and it would go away with time," she recalls, speaking in the lilting accent of her native Poland. "So I didn't do anything about it." For two weeks, the pain persisted. Then one Sunday last fall, she suffered a severe ache on the left side of her chest. And her leg still hurt — so badly that she couldn't stop rapping on her thigh, as though to work out a muscle cramp.
Neither she nor her husband got much sleep that night. But it wasn't until she rose to take a shower the next morning that they really got worried. "In ten minutes my calf doubled in size, and it got darker and darker," she says. "We were shocked and scared." As her husband, Marcin, recalls: "The leg actually swelled up in front of my eyes. In two hours, it was the size of mine."
Joanna and Marcin, who run a construction business together, hurried from their home in Greenpoint, Brooklyn, to her ob/gyn in Queens — who immediately sent her to the emergency department at Flushing Medical Center. Scans found that she had a large blood clot in her left lung, known as a pulmonary embolism (PE). She also had a deep-vein thrombosis (DVT) — in her case, a large blood clot in her right groin area — which could break free at any moment, traveling to the heart and lungs and causing yet more emboli and possibly death. Within two hours she was in an ambulance headed for NYP/Weill Cornell, lights flashing and siren blaring. "From Queens to Manhattan took fifteen minutes, with traffic and everything," Marcin remembers. "It was the easiest commute ever."
Marcin can make light of their high-speed adventure now, because everything worked out: both mother and child survived a condition that is too often deadly or debilitating. According to the CDC, accurate figures on DVT/PE are hard to come by. Each year an estimated 300,000 to 600,000 Americans suffer them; in people over eighty, the rate may be as high as one in 100. The outcomes are often grim: 60,000 to 100,000 Americans die of the condition annually, with 10 to 30 percent of patients perishing within a month of diagnosis. In a quarter of cases, the first and only symptom is death. "The scary thing about PE is that it can happen to anybody at any age," says Akhilesh Sista, MD, assistant professor of radiology in the Division of Interventional Radiology. "It could be a twenty-year-old woman who's on birth control, has a genetic propensity toward clotting, and develops a big DVT that goes to her heart and lungs."
Besides genetics, age, and use of oral contraceptives, other risk factors for PE include cancer, pregnancy, smoking, obesity, and recent surgery. Another common cause is prolonged immobility — whether due to bed rest during an illness or from sitting in the same position while traveling. In 2011, tennis great Serena Williams suffered a PE after flying from New York to L.A. with a foot injury that limited her mobility; while covering the invasion of Iraq in 2003, an NBC reporter died from a PE after hours crouched inside an Army tank. "PE is one of the more sinister and insidious diseases," Sista says. "It's on every list, but there's nothing specific about the presentation. It may be that somebody collapses — but that could be stroke, arrhythmia, all sorts of things. The presentation can range from not feeling quite right to being dead on arrival. We may find not find out it was a PE until the postmortem."
But for the past two years, NYP/Weill Cornell has led an effort to improve treatment for pulmonary embolism, through a combination of teamwork and a specialty best known for minimally invasive image-guided interventions. In early 2012, Sista and his interventional radiology (IR) colleagues were asked to consult on a neurosurgery case. The man, in his mid-thirties, had post-surgical bleeding, low blood pressure, and severe shortness of breath. "We ordered a CT scan and saw a huge clot in his pulmonary artery," Sista recalls. "We realized that he needed more than a blood thinner if he was going to survive."
"The scary thing about PE is that it can happen to anybody at any age."
The ICU doctor knew that the IR team was skilled in treating clots in deep veins. Could they do the same in the lungs? Sista, who'd performed such a procedure several times in fellowship, was willing to try. In the IR suite, he entered through a vein in the patient's neck, navigating to the lungs and using a device to clean out the clot. "Within five minutes, he was able to support his own blood pressure," says Sista. "We thought this was a powerful case — and that it was something we needed to tell the hospital about, because it wasn't being done. We also realized that there were a lot of cases in the community that weren't getting recognized and treated quickly enough."
Dr. Akhilesh Sista
Those insights led to the formation of the Pulmonary Embolism Advanced Care (PEAC) team. The group is led by faculty from the specialties that the disease touches: pulmonology (Oren Friedman, MD, assistant professor of medicine), cardiology (James Horowitz, MD, assistant professor of medicine), cardiothoracic surgery (Arash Salemi, MD '97, associate professor of cardiothoracic surgery), and interventional radiology (Sista and David Madoff, MD, professor of radiology). "PE is a multidisciplinary disease," Horowitz says. "But when someone comes in really sick, the standard ED or medicine person often can't get all the players to see them fast enough before something bad happens, and they could die. So we've created this multidisciplinary approach."
The most dire cases are known as "massive PEs," having a mortality rate between 20 and 50 percent. In "submassive" cases, between 3 and 10 percent of patients perish. So while the condition is challenging to diagnose, the stakes are high — and even though most patients survive, quality of life can be much diminished. "It's a stress-inducing disease for doctors," says Friedman. "We believe many patients die while physicians decide whether to do more. Physicians are sometimes so worried that they'll make the situation worse by escalating treatment that they end up not considering more advanced treatments. A team approach not only allows the combination of experience from different perspectives, but it also leverages our expertise to recommend the best treatment for our patients. And sometimes it's not just about immediate survival. Aggressive therapy up front can make the difference between having your patient back to their baseline in six months or being unable to walk half a block before stopping to catch their breath."
It wasn't until the mid-twentieth century that patients with major PEs even stood a chance, thanks to the blood thinner heparin, improved imaging, and modern surgical techniques. Currently, the standard therapy is to give heparin to help resolve the clot; in urgent cases, surgeons may perform an open procedure called an embolectomy. "For the sickest patients and the most hemodynamically unstable, the safest place is in the operating suite with careful anesthesia and cardiopulmonary support," says Salemi, noting that surgery remains the gold standard for certain types of PEs.Patients who have life-threatening PEs but aren't surgical candidates can get an intravenous dose of the clot-busting drug tPA. FDA approved in 1996, it has saved many stroke patients, whom interventional radiologists treat by delivering it directly to a brain clot. But doctors only give it intravenously when there's no other option. "It breaks up the clot — but the drug also goes everywhere else," Sista notes. "You have a high risk of bleeding in bad places, including the brain, and there's real mortality associated with that." In short, with the best options — heparin or surgery — dating to the Johnson Administration, PE treatment was ripe for improvement. "You could make a list of a hundred diseases," observes Horowitz, "and I challenge you to find another where the treatment hasn't changed in fifty years."
Horowitz can claim credit for the PEAC team's low-tech secret weapon. It's a ballpoint pen — or, rather, hundreds of them, bearing the pager number 1-CLOT. Designed to spread the word to staff throughout the hospital, the humble pieces of lime-green plastic fill a void left when giveaways from drug reps were disallowed. "There are no more free pens," Friedman says with a chuckle, "so ours are everywhere." In a system that Madoff compares to a tumor board, team members meet monthly to review cases and tweak treatment protocols. They've now had about 150 consults and more than two dozen interventions as well as a number of outside hospital transfers. And they've given talks promoting the team to colleagues from internal medicine, the ED, anesthesiology, and more. Research projects — including a study chronicling the outcomes of all cases treated through the 1-CLOT system — are in the works, and team members have shared their approach with other hospitals, including Hospital for Special Surgery, Brooklyn Hospital, and NYPH Lower Manhattan.
Dr. David Madoff
"In the normal way of dealing with PE, the patient is treated arbitrarily based on whoever sees them," Horowitz observes. "We now have access to all this expertise with one phone call. Timing is crucial, and getting all the appropriate studies up front and making decisions fast is absolutely necessary."
At NYP/Weill Cornell, the team effort is complemented by another unorthodox approach: using IR techniques not only to physically remove clots but to administer targeted doses of tPA. Known as catheter-directed lysis, the technique avoids the bleeding risks of intravenous tPA while concentrating the drug where it's needed. Its successful use in more than two dozen cases positions Weill Cornell as a leader in PE treatment. "This procedure is not being done frequently in the country or the world," Sista says. "People have been treating PEs endovascularly for a couple of decades, but it hasn't gained much traction." Sista aims to change that, beginning with a randomized trial for submassive PEs that will compare the efficacy of catheter-directed lysis versus heparin alone. "You want a treatment that gets rid of the clot in a minimally invasive manner," he says. "Catheter-directed work may hit the sweet spot. We have the potential to minimize bleeding but maximize the therapeutic benefit."Among those successful cases is that of Mary Deberry, a sixty-five-year-old elementary school paraprofessional from Crown Heights, Brooklyn. A breast cancer survivor, Deberry was undergoing treatment for lung cancer when she found she could barely breathe. "She thought she was going to die," Sista says. "And she was possibly en route to that." A CT scan found a massive PE in her right lung — the only one she has, the other lung having been removed. "Ms. Deberry's case was complex and unique, since she only had a single lung," says Madoff. "To our knowledge, catheter-directed lysis had never been used in this setting before." After the treatment, he recalls, "She was literally crying tears of joy, because we'd saved her life." Deberry says that although she hasn';t been able to resume work, she's making a steady recovery. "Right now, I don't have any problems breathing or anything," she says. "I know I'm a walking miracle. Through God and the doctors, I'm here."
"In the normal way of dealing with PE, the patient is treated arbitrarily based on whoever sees them. Now we have access to all this expertise with one phone call."
PEAC team members involved in Deberry's case have written it up to highlight how catheter-directed lysis can save a fragile patient — a massive embolism in one's only lung being an exigent circumstance. They've also submitted the case of Joanna Cis, whose treatment affected two patients: it wasn't just her life in the balance, but her unborn child's as well.
When Joanna came in that Monday, the team leapt into action. She was evaluated and given heparin, with initially positive results. "Tuesday and Wednesday I felt better," she remembers. "I was doing my hair and makeup, and they thought I was going to be good." Then came Thursday morning. "It hit me again," she says. "The pain was back in the left side of my chest. In an hour, it went from a one to a ten." With her case becoming increasingly serious, the various specialists examined her and consulted with each other. "There were about thirty doctors," Marcin recalls. "There was a line outside the door." As Joanna underwent catheter-directed lysis on the clot in her lungs, an ob/gyn was on hand in case fetal distress necessitated a C-section and a speedy transfer to the neonatal ICU. After tPA was dripped to her lung embolus for twenty-four hours, the team repeated the procedure on the clot in her groin area. When it was all over, she says, "There was no more pain. I was so relieved."
But there were still some pitfalls on her path to motherhood. Joanna developed preeclampsia, a serious pregnancy complication. At eight months of gestation, to protect her life and the baby's, doctors induced labor. On New Year's Day, in an otherwise normal vaginal birth, she delivered a son whom she and Marcin named Kacper. "He's a beautiful boy," she says. "Very calm, very quiet. So great."
While Joanna will have to self-administer injections of an anticoagulant drug every twelve hours for the foreseeable future — a process she admits is painful and unpleasant — she knows that her outcome could have been far worse. "I'm happy I'm alive," she says. "So if I have to give myself those shots, that's OK." When asked about the care she received from the PE team and other medical staff, both she and her husband are effusive in their praise. "All the doctors and nurses were like my friends," Joanna says. "They took care of me so well — just outstanding. I never had any second thoughts." Adds Marcin: "With her, there was no book they could refer to — but they did everything right."
This story first appeared in Weill Cornell Medicine, Vol. 13, No. 2.