More Research, Advocacy Can Improve PAH Disparities

Pulmonary arterial hypertension (PAH) is a rare disease without a cure that’s often not diagnosed until it’s in advanced stages. When you’re diagnosed with a condition such as PAH, it’s important to receive the best care possible. But certain groups may run into obstacles known as health disparities. Health disparities are preventable differences in the way you are cared for due to your race, socioeconomic group, or other factors that could leave you at a disadvantage. 

PAH gets worse over time, and there’s a high risk of death. Early treatment can help people live longer with this form of pulmonary hypertension (PH). That’s why early detection, better therapies, and aggressive treatments are critical. But disparities in treatment remain a challenge.

What Disparities Are Unique to People With PAH?

Social determinants of health like your race, where you live, and how much money you have can influence your health with any condition. But in PAH, the disease itself can put you at a disadvantage.

You may think symptoms like fatigue and shortness of breath are just a normal part of aging. By the time you get to a doctor about these symptoms, the disease could be advanced, says Charles P. Vega, MD, a clinical professor of family medicine at University of California, Irvine. 

One in five people with PAH had symptoms for more than 2 years before getting a diagnosis. 

A person with shortness of breath may be diagnosed with asthma but may also have PAH that goes undiagnosed. Or their symptoms may be misdiagnosed completely, says Stephen C. Mathai, MD, an associate professor with The Johns Hopkins Pulmonary Hypertension Program.

Another disparity unique to PAH is the significantly high cost of frequent follow-up care and medications. 

What Factors Cause Health Disparities in PAH Care?

These factors can create more disparities if you have PAH:

Sex. If you were assigned female at birth, you’re more likely to get PAH than if you were assigned male at birth. You’re more likely to survive longer, but you’ll respond to treatment differently than men.

PAH causes high blood pressure in the pulmonary artery, which carries blood from the heart to the lungs. In women, one heart chamber works better than it does in men, which improves women’s prognosis. 

Race and ethnicity. If you’re Black, you’re more likely to have PAH risk factors like systemic sclerosis, HIV, sickle cell disease, or liver disease. 

Black people are more likely to have connective tissue disease-associated PAH. Hispanic people get congenital heart disease-associated PAH more often. 

Black and Non-Hispanic white people are more likely to get PAH-specific medications than Hispanic people.

Another study showed a difference in the way people of different sexes and races responded to a specific treatment. In testing responses to endothelin receptor antagonists, a type of therapy for PAH, women and white people were shown to respond more significantly to the treatment than men or Black people. 

Location and environment. Where you live can also put you at a disadvantage. More than 91% of PH specialty centers are in urban areas. More people die from PH in rural areas than urban regions, another study found.

“PAH specialists are distributed unevenly across the U.S., with many regions having few specialists if any,” says Mei-Sing Ong, PhD, an assistant professor at Harvard Pilgrim Health Care. 

Other research found that living in more polluted areas can make PAH worse.

Socioeconomic status. Research links low socioeconomic status — your education, job, and wealth — with worse PAH outcomes. 

The COVID-19 pandemic may have made care worse for people with PAH who have low socioeconomic status. The pandemic’s economic effects had a higher impact on those in this group. And while lockdowns expanded telehealth, not everyone could use it if they lacked internet service or video-enabled devices. 

Bias and discrimination. There’s not much research on discrimination faced by those who have PAH, or how PAH disproportionately affects people with disabilities, older people, prisoners, and members of the LGBTQ+ community.

Researchers want more studies on how discrimination affects the prevalence of PAH, time to diagnosis, and overall outcomes. 

Bias also exists amongst medical professionals.  In order to improve PAH care, providers must work to remove their implicit biases, or the stereotypes that unconsciously exist in their minds, from their practice. These biases about people, lower-quality facilities, and unattractive reimbursements can keep PAH specialists from working in areas where more disparities exist. Bias has also been linked to poor communication between doctors and minorities needing care. 

What Solutions Can Improve PAH Disparities?

These are a few solutions that can ease disparities:

Accessible, expert care. PH Care Centers (PHCCs), which house PAH specialists, are expanding. But a lot of people have a hard time getting to them, Vega says.

“Unfortunately, those centers become centers of privilege,” Vega says. “They’re not geared towards health equity.”

The Pulmonary Hypertension Association (PHA) is looking to expand PHCCs in areas where they don’t exist, says Elizabeth Joseloff, PhD, vice president of quality care and research at the PHA.

Stronger research. Because it’s a rare disease, there aren’t as many people that can participate in trials, Mathai says.

Most people in PAH research are women. Study participants tend to be  older and more obese, which represents people with PAH on the whole. The percentage of people who aren’t white has gone up thanks to targeted recruiting. 

Researchers want more underrepresented groups of people to be involved in trials consistently. They want more trials on PAH with all types of PAH represented, as well as people with a broader range of disease progression. For example, research has looked into how genetics may make people in different groups more prone to other lung disorders, but not PAH.

One thing helping research on PAH and disparities is the Pulmonary Hypertension Association Registry (PHAR) — a database on people getting care at PHCCs. It’s helped to increase research on PAH and see how disparities affect outcomes, Mathai says.

More research will lead to better therapies. Both have increased in recent decades, but this area has room to grow, Mathai says.

Affordable treatments. Mathai says PAH drugs can cost anywhere from $50,000 to $250,000 a year. If you have PAH, you may need to be on multiple therapies, which can raise costs. You also may need frequent follow-up visits and tests, which are an economic burden, Mathai says. 

Insurance can improve care access and outcomes regardless of race, one study found.

If more PAH medications are available in generic form, that could also lower costs.

Better education. Mathai says frontline providers need to know more about PAH so they can diagnose people faster and avoid misdiagnosing them. Doctors who are unsure about symptoms need to refer people to a specialist, Mathai says.

Raising awareness. Advocacy groups continue to get the word out about PAH. Pulmonary Hypertension Awareness Month and World Pulmonary Hypertension Day aim to educate others about this condition. 

These have boosted awareness of PH, but not PAH specifically. They also haven’t seemed to shorten the time it takes to get an accurate diagnosis, so awareness must continue, Mathai says.

“It’s not something that’s going to turn around in 6 months or a year,” Vega says. “But if we keep doing it … there’s signs for a brighter future ahead.”