Toss out the words “chronic thromboembolic pulmonary hypertension” at your next get-together with friends and you’re likely to get a collection of blank stares. But Terrie Karp, who has the condition, can cut through the mystery with a few words.
The unwanted efficiency is the result of a genetic mutation – Factor V Leiden clotting disorder – that Karp, 45, inherited from her father. The condition predisposes Karp to developing blood clots that have for more than a decade required regular treatment with anticoagulant medications. Over time, however, the medications failed to dissolve some of the clots, even as Karp’s body produced new ones. Clots collected in the blood vessels of Karp’s lungs, causing them to scar and narrow. This, in turn, led to pulmonary hypertension, or high blood pressure that strains the right side of the heart. The dangerous combination goes by chronic thromboembolic pulmonary hypertension, or CTEPH.
Pulmonary house cleaning
It was this condition that led Karp to UCH for a procedure in December with another tongue-twisting name: pulmonary thromboendarterectomy. The surgery, performed for the first time at UCH in several years, involved opening Karp’s chest, entering the pulmonary artery of each lung and painstakingly scraping the blood vessel walls clean of clotted build-up.
A couple of weeks after the procedure by a multidisciplinary team that included surgeons Jay Pal and Christopher Scott – both of whom had trained for it during separate stints at Duke University Medical Center – Karp was discharged from UCH and returned to Grand Junction to recover.
The procedure required Karp to invest a good deal of trust in her providers at UCH. “We were clear with Terrie that hers would be our first case here,” said Scott. However, he and Pal also reassured her that a team that included experts from pulmonology, hematology, radiology and nursing had reviewed her case for months before determining surgery was the best course of treatment.
More daunting, the procedure required stopping blood flow to her entire body for a total of about 50 minutes while Pal and Scott did arterial cleaning. More on Karp’s trip to the netherworld later. She makes clear that, whatever the risks, giving the thumbs up to surgery was easy when she considered her alternatives.
Simply put, CTEPH had laid waste to her life. In 2007, Karp was working happily as a fourth-grade teacher on a Navajo reservation in Gallup, New Mexico when the first overt signs of CTEPH appeared: a swollen leg and back pain that were the result of a blood clot. She spent her Christmas vacation in the hospital and later flew to Albuquerque, where providers threaded a catheter into her leg to suck out a clot the size of an eraser at the end of a pencil. She left the hospital with the clot cleared. With the help of a blood thinner, she resumed normal activities.
But Karp’s body continued its clot-making efficiency, and the toll increased. In 2010 she had a new teaching job in Lawrence, Kansas when clots in her leg and arm felled her again. Treated with blood thinners, she recovered, but the next year, during a Christmas break visit to her parents’ home near Elko, Nevada, Karp suffered a sudden painful attack that turned out to be a blood clot in the lung. The following summer, she was back in Elko, watching President Obama accept his party’s nomination for a second term when a violent headache struck. She called 911 and was airlifted to the University of Utah, where she was treated for a stroke caused by a blood clot in her brain. She was just 39 years old and her life was in shambles.
“The disease derailed my teaching career,” Karp said. It took four arduous years of recovery, including treatment to control epileptic seizures, to land another teaching position, this one at a school serving the Navajo Nation in Yatahey, New Mexico. However, her hopes for a successful return to the classroom were sabotaged by multiple clots in both lungs. She missed the first four days of class and required oxygen around the clock after she returned. She struggled through fatigue, shortness of breath and pain to finish the year, but the school didn’t renew her contract because she’d had too many absences.
Following this disappointment, Karp moved to Grand Junction to live with a friend. She says her medical providers there told her that there was little they could do for her other than attempt to manage her disease with blood thinners. But one of them referred her to UCH pulmonologist Dr. James Maloney for the evaluation that eventually led to the pulmonary thromboendarterectomy.
Maloney said the multidisciplinary team’s consideration of Karp’s case included reviewing her extensive medical records and an echocardiogram. A pulmonary angiogram that yielded images of the blood vessels on the right side of her heart helped the team quantify the severity of her pulmonary hypertension and decide on medications to manage the condition. The imaging also revealed Karp’s “high clot burden,” which made it unlikely that medications alone could restore her quality of life, Maloney explained.
“One of Terrie’s goals was to get back to teaching, and it’s something she simply couldn’t do, not only because of her oxygen levels, but because she had frequent hospitalizations related to her CTEPH,” he said. “We knew that she would have not only a shorter life but also that the quality of life she had was not consistent with what she viewed as acceptable.”
Pal and Scott traveled in October to UC San Diego Health, which developed the pulmonary thromboendarterectomy procedure. Colleagues from San Diego later journeyed to UCH to support Pal and Scott during Karp’s surgery.
The razor’s edge
The procedure begins with the surgeons opening the sternum, reaching the pulmonary artery, and using a sharp knife to delicately scrape away and suction out clotted debris pressed into the wall lining. Success or failure balances on a razor’s edge.
“The most important thing is to identify exactly where the clot begins and ends because it is embedded in the walls of the artery,” Pal said. “If we take too little of the clot out, we haven’t really helped the patient. If you take too much, you risk rupturing the artery, which is universally fatal.”
Then there’s the matter of eliminating blood flow and movement from the artery. That requires lowering the patient’s body temperature to about 65 degrees Fahrenheit and stopping the heart, creating a state Scott described as “functionally dead.”
At that temperature, the body requires little oxygen to remain functional, Pal said. “It’s analogous to when a kid falls in a frozen lake in Michigan,” he said. “The body gets so cold that the heart stops. An hour later they warm up and they’re fine because their body was so cold it didn’t matter that they had no blood flow.”
The removal of the blood clots – first on one lung artery, then the other – takes only about 50 minutes total, but the entire procedure takes many hours. That’s because after about 20 minutes, the super-chilled body begins to warm up, requiring the work to stop so that the process of gradual cooling can begin again in preparation for another round of clot removal.
For her part, Karp said she was concerned about “losing her intelligence” if the procedure went wrong, but was also hoping to experience and remember a near-death experience.
“I wanted to see what it’s like on the other side,” she said. No such luck: the procedure was a blank. When she regained consciousness she was intubated and so upset that she was restrained that she threatened to inform the Southern Poverty Law Center that she’d been subjected to inhumane practices.
“I knew the restraints would happen, but I was in delirium,” Karp recalled, chuckling.
She recovered her faculties in a day or so and began a recovery that included a brief bout of atrial fibrillation and pain from implanted drains she described as “garden hoses.”
Karp said she appreciated the careful explanations Pal and Scott gave her of the procedure and its risks, but she had little trouble making her decision.
“I said, ‘Let’s do it,’” she recalled. “I was ready to get my life back. I was ready to start teaching. I was ready to be able to take a shower without needing oxygen, to got to Walmart and shop without getting short of breath.”
Arteries cleared, on with life
With the wreckage of the old clots removed from her lungs and her pulmonary hypertension relieved, Karp now has reason for optimism. She’s set her sights on returning to teaching, first in a substitute role, as soon as possible. Her body will continue to form clots, but Maloney said she has a good chance of controlling them with consistent anticoagulant treatment.
“She has a very good prognosis,” he said. “She can be more physically active, which increases her cardiac output and helps to scrub clots away.”
She’s the first of what the multidisciplinary team hopes will be a steadily growing list of CTEPH patients to receive the procedure. Maloney estimates that about 40 people in Colorado each year could benefit from the surgery. The team, led by Dr. Todd Bull, medical director of UCH’s Comprehensive Lung and Breathing Program, now meets regularly to review cases and select the most appropriate candidates.
Scott said it is important in building the program to increase awareness of CTEPH among the public and community providers. He noted that ventilation-perfusion (VQ) scans, which measure air flow and blood flow in the lungs, are relatively inexpensive yet reliable early screens for the disease and thus could help to spur referrals.
Whatever the number of cases, the team’s goal is not only to repair the physical damage caused by CTEPH but to restore hope for the future, Maloney said.
“That’s always a motivator for anything we do: not only to extend life with a certain therapy, but to improve the quality of life,” he said.