Easing Gridlock in Hypertensive Lungs

Picture of highway overtop of lungs

Pulmonary vascular disease program at MUSC Health offers a multidisciplinary health care team, clinical trials and patient support groups

by Celia Spell

Languishing lungs loom large for people with pulmonary hypertension as their breathing moves from a gliding subconscious act to a rattling daily struggle.

Designed to swell with air and expel carbon dioxide, the lungs are the largest organ in the body. And their partnership with the heart keeps oxygen moving throughout — both at rest and during exercise. There are multiple forms of pulmonary hypertension, but what binds these types together is high blood pressure in the arteries of the lungs as well as the heart. As the many and closely clustered blood vessels in the lungs begin to narrow, blood flow slows, which decreases the amount of oxygenated blood circulating throughout the body and can act as a gridlocked highway.

People with pulmonary hypertension often experience tightness in the chest and difficulty breathing, during exercise at first and then eventually even at rest. They find it difficult to perform everyday tasks like walking down the driveway to take the trash out. If left untreated, pulmonary hypertension can eventually lead to heart failure.

The lungs are uniquely and beautifully crafted, according to Rahul Argula, M.D., MBBS, MPH, an associate professor and program director of the MUSC Health Pulmonary Vascular Disease program. “No other organ in the body performs a job like the lungs do,” he said. “In patients with certain forms of pulmonary hypertension, the blood vessels in their lungs can become so narrow that blood can’t reach an entire portion of the lungs. And when that happens, it’s like trying to rev up the engine of a car – you just can’t because it’s too weak.”

When someone is unable to shift into a higher gear like they’re supposed to, they can’t tolerate any amount of exercise. Healthy lungs can handle increased blood flow up to five times higher during intense exercise as a way to circulate more oxygen throughout the body, but people with pulmonary hypertension often can’t even climb the stairs or walk across a parking lot without becoming out of breath. Their car stalls out when they need it the most.

The Road to Diagnosis

Physicians are taught a simple mantra when it comes to diagnosing patients: When you hear hoofbeats, think horses, not zebras. Think of the most common or likely diagnosis rather than looking for something unusual or exotic. But with rare diseases like pulmonary hypertension, seeing zebras leads to the correct diagnosis, which is why the National Organization for Rare Disease chose the striped quadrupeds as their mascot.

To be categorized as rare, a disease needs to affect fewer than 200,000 people in the United States or one person per 2,000 people in the European Union. Argula points to rarity and the lack of familiarity with the disease as often the roots behind its misdiagnosis. Since shortness of breath is such a common complaint of patients with a variety of lung diseases, health care providers can sometimes misdiagnose pulmonary hypertension as asthma or chronic obstructive pulmonary disease (COPD) since they appear more frequently.

People with pulmonary hypertension often see multiple doctors over the course of multiple years before they receive an accurate diagnosis. Denise Sese, M.D., is an assistant professor in Pulmonary and Critical Care medicine at MUSC as well and works with Argula in the MUSC Health Pulmonary Vascular Disease program. She says it takes these patients an average of two and a half years before they learn that their breathing issues are due to high blood pressure in their lungs.

And while pulmonary hypertension is a lifelong disease, Sese and Argula say that it’s very possible to live a full life with it and that drastic quality-of-life improvements can start once an accurate diagnosis is made. But those two and a half years can often lead to irreversible damage, so it’s important that patients find health care teams with the necessary experience to help.

Argula describes pulmonary hypertension as a very complex disease whose treatment and diagnosis can incorporate pulmonologists as well as cardiologists. Ryan Tedford, M.D., the chief of heart failure and medical director of cardiac transplantation at MUSC, works with Argula and Sese and is often involved in diagnosing patients with unexplained shortness of breath. He finds this type of teamwork important for patients.

Pulmonary hypertension may be a result of genetic mutations, medication use, connective tissue disease, heart disease, chronic lung disease or from unidentifiable causes. And while there are officially five different groups of patients with pulmonary hypertension, Tedford places them in two overarching categories: those with left heart disease and those with precapillary pulmonary hypertension, which primarily affects the lungs.

Tedford focuses on patients who have pulmonary hypertension due to left heart disease, while Argula sees patients with precapillary pulmonary hypertension.

Argula says that while there are different types of pulmonary hypertension, they all translate to poor quality of life without treatment. "Because of that we take a very organized approach to their diagnosis and treatment," he said. "Oftentimes, such diagnostic and treatment resources are not available at smaller community hospitals but are only available at academic health centers like MUSC."

The Comprehensive Care Center On-Ramp

An interdisciplinary team like the staff at the MUSC Health Pulmonary Vascular Disease program is helpful for both pulmonary hypertension patients and their providers according to Argula. “It’s our job to figure out where the problem is coming from,” he said. “And it takes a very experienced team of radiologists, cardiologists and pulmonary hypertension specialists to piece all these details together and provide an accurate diagnosis.”

And it’s that accurate diagnosis that is the treatment cornerstone for these patients and for Argula. Without it, he can’t properly help his patients. He can’t improve their quality of life.

To expand the treatment options for pulmonary hypertension patients in the state of South Carolina, Argula and his team sought and successfully received an official Pulmonary Hypertension Comprehensive Care Center (PHCC) designation from the Pulmonary Hypertension Association (PHA) in late 2019.

To receive this designation, MUSC designed a team of providers that includes Argula, Sese and Tedford as well as Kristine Ritenour, the nurse coordinator for the program, and Ashley Warden, CCRC, the pulmonary vascular center’s clinical research program manager.

“Our accreditation reassures our patients that when they come here, they are going to get state-of-the-art therapy and the most evidence-based medical practices in the field,” Argula said. “More than anything else, they know that they have access to a very experienced group of providers familiar with diagnosing, treating and taking care of people with this disease.”

As the nurse coordinator for the program, Ritenour works alongside each patient throughout their treatment, which could include continuous IV therapies and subcutaneous infusion systems. Since some treatments require a constant infusion of medication, Ritenour teaches patients how to use their pump or troubleshoot any other treatment issues they may have. She is also the first person Argula and Sese reach out to when asking if a new treatment option will suit a particular patient’s lifestyle or goals. She is integral to each patient’s individual care plans and knows which therapies might fit well.

The MUSC program underwent a rigorous accreditation process to become one of only 50 accredited programs in the country and the only one in the state. Members of the PHA, the largest and oldest pulmonary hypertension group in the world, inspected the facilities at MUSC before awarding the PHCC designation. They met with every member of the pulmonary hypertension health care team, including physicians, pharmacists, nurses and coordinators, and conducted interviews with current patients to evaluate the program. It will be evaluated further every 3 years to renew the designation.

Ritenour says this designation opened the door for more referrals and gave patients with pulmonary hypertension a place to go. By adding Sese to the team, the MUSC providers can now see twice as many patients as before, and with the recent addition of a pharmacist to the outpatient program, provider access for MUSC’s patients has only continued to grow.

Members of the pulmonary vascular disease program had one goal in mind when creating the center: offering compassionate care to their patients. “We want them to feel like someone understands them, understands their disease and understands what it means to be living with pulmonary hypertension,” said Argula.

Cars in traffic 
“In patients with certain forms of pulmonary hypertension, the blood vessels in their lungs can become so narrow that blood can’t reach an entire portion of the lungs," said Argula. "And when that happens, it’s like trying to rev up the engine of a car – you just can’t because it’s too weak.”

Paving the Way with Patient Education

Researchers in recent years have started taking their patients’ emotional needs into consideration when assessing the effectiveness of a new therapy, which is particularly important with pulmonary hypertension patients. Sese points to energy level as a good example. A side effect of pulmonary hypertension is often fatigue and feelings of low energy, so a successful treatment is one that provides more energy to the patient. The perception of a patient’s own treatment often determines its success over that of reported numbers or tests.

“Pulmonary hypertension physicians are studying everything,” she said. “They’re looking at medications. They’re looking for ways to increase their patients’ life expectancy — what clinical factors might make things better or worse — but no one’s telling a patient how to live their life.” Sese sees less value in extending a patient’s life without also improving it, so she emphasizes prioritizing the comfort levels of patients.

Studies have shown that pulmonary hypertension patients tend to score lower on quality-of-life surveys than those with more terminal illnesses like cancer. A sense of hopelessness often surrounds them, which is one of the reasons Sese is so passionate about patient education and activation — or the knowledge, skills and confidence to manage one’s own health. These emotional needs can sometimes be overlooked by a health care team.

“By the time many of these patients reach our clinic, it feels almost as if we’ve been set up to become their saviors,” Sese said. “They’ve been seeing physicians for years without a correct diagnosis, and they’ve been told they’re going to die, that there is no hope and that nothing can be done. But that’s just not true. There are new treatment options every day, and improved diagnostic capabilities. There’s this center of excellence with all its ancillary support and subspecialties. We’re here to help.”

Clinical Trials Provide Additional Avenues

A major component of the recent Pulmonary Hypertension Comprehensive Care Center designation is the program’s ability to offer clinical trials, which provide cutting-edge treatment options to patients. It can take years for a new medication or therapy to reach the market, but patients in clinical trials as part of the pulmonary vascular disease program can access new drugs years before they’re widely available, and without having to pay. When a medication does eventually reach the market, they can then switch from the clinical trial version to the commercially approved version.

As the clinical research program manager, Warden meets with the program team weekly to discuss which clinical trials might offer the right solutions to either incoming patients or recently seen patients. Once enrolled, patients also have access to research coordinators to help them navigate their journey through the trial.

Warden says increased access to providers is another positive side effect of enrolling in a trial. “We see them in the clinical trial space a lot more frequently than on the clinical side alone,” she said. “If an issue does pop up, we can immediately relay that to the nurse coordinator or the providers directly.”

In research trials, patients also receive lab work or tests more frequently, which can help their health care team recognize changes quickly and tweak their treatment.

Argula says the care center team often works with industry partners and the National Institutes of Health alike to develop new strategies for patients. With so few approved treatment avenues, he is focused on having a portfolio of promising clinical trials to offer this group of patients. Most patients are excited to try a new therapy even if some trials prove unsuccessful.

“These treatments may help, but even if they don’t, most of our patients also appreciate being a part of something that collectively advances the science and could help others like them,” he said. 

Warden has found that research teams and clinical teams are often kept separate at other programs, while at the MUSC Health Pulmonary Vascular Disease program, they work in tandem. “There’s a lot of good communication between both sides,” she said. “Which allows us to really make a difference. It’s about the communication and the way we all work together.”

In addition to funded clinical trial treatment options, MUSC also offers support groups for pulmonary hypertension patients so that they can learn from each other and hear about others’ experiences with the disease. Ritenour worked with the PHA to organize a local group in Charleston, South Carolina. Before the pandemic, patients could meet in person, but she says they have since shifted to a virtual platform to keep patients connected.

It's these long-term connections and relationships that fulfill Ritenour and the rest of the team. “I meet these patients at their lowest of lows,” she said. “It might be in the ICU or right after they’ve been told they have a chronic disease that’s not curable. But once we get them started on the right therapy and we find the combination that works best for them, we can give them some of their life back.” 

She highlights the importance of the interdisciplinary team as well. “There’s a lot of hard work and teamwork that goes into treating these patients,” she said. “But forming these relationships and helping them improve is really rewarding.”

Sese added, “The disease becomes livable because of all the progress we’ve made in recognizing pulmonary hypertension as well as providing treatment.”

With recent advancements in both diagnostics and therapy options, people can live for years with pulmonary hypertension, even decades. And with the help of a multidisciplinary health care team, they can find which solution works best for them and hopefully breathe a little easier.