Pulmonary arterial hypertension (PAH) is a rare medical condition that’s difficult to diagnose and gets worse with time. To get ahead of this condition, getting a timely diagnosis with specialized care and PAH-specific medications is key.
But for most people, getting a proper diagnosis and referral to a pulmonary hypertension care clinic can take up to a year or more. This delay can be far greater for people of color, especially Black and Hispanic people. And this can result in worse outcomes for minorities with PAH.
In fact, health databases on PAH show that Black women are more likely to die across all ages and genders compared to other groups of people.
Why is that? Research shows there are several structural barriers in society that cause this. Here’s a look at how pinpointing and addressing PAH care gaps can improve access to treatments and long-term health for minorities.
Delays in Diagnosing Pulmonary Arterial Hypertension
For Nicole Creech, 57, doctors took almost 3 years to figure out she had PAH.
“It [PAH] kind of played peek-a-boo with me,” says Creech.
Over the course of three years, the Lexington, Kentucky native’s PAH symptoms, such as shortness of breath, came and went many times. This led to several trips to the emergency room, and even a brief stay at the hospital with specialists checking in on her. But nothing came of it.
Research shows that on average, the time from when your PAH symptoms start to your diagnosis can range anywhere from 2 to 4 years.
After another bout of severe symptoms, Creech was finally diagnosed with PAH in 2008.
“It felt like I had a chicken bone hanging from my ribcage right in the middle. I didn’t know if I had eaten something bad or swallowed something sharp. I just couldn’t figure out what was wrong, I could barely walk from the living room to the refrigerator,” Creech recalls.
During her diagnosis, Creech spent 3 weeks in the hospital. A team of lung and heart specialists placed a catheter inside a vein in her chest to supply PAH-specific medication daily. Fifteen years on, access to quality care has largely improved how PAH affects her quality of life.
For this, she credits several things including her diagnosis of sickle cell anemia when she was 6 months old.
“I’m a minority but I’ve had good health care when it comes to PAH,” says Creech. “I think the thing that worked for me was the fact that I’ve always been very familiar with the health care system. I have been able to foster relationships with my doctors and my nurses, and I’m educated. I have taken an interest in my illness and tried to learn as much about it as I possibly could so that I can communicate effectively with my caregivers or my health care providers.”
But Creech, who works in PAH advocacy and is involved in support groups, says this isn’t true for everybody.
Health Disparities in Pulmonary Arterial Hypertension Care
According to Vinicio de Jesus Perez, MD, an associate professor of medicine who specializes in pulmonary and critical care at Stanford University, health disparities and delays in getting a proper diagnosis for PAH among minorities stem from several different non-medical social factors.
Experts often refer to these as “social determinants of health,” and they can play a powerful role in influencing your overall quality of life. That’s because PAH is a complex medical condition that’s hard to spot, and you’ll need specific medications and in-person doctor visits to keep it in check.
Social determinants of health include:
Geographic location. Where you live might dictate the quality of health care you’re able to get.
“We like to see our patients every 3 months to get an assessment of how they’re responding to therapy, whether they need to have an adjustment in the dosage, [or] whether they’re having any complications,” Perez says.
For this, ideally, you’ll need to visit specialized pulmonary hypertension care centers to work with a team of specialists. Currently, there are only about 80 centers across 32 states in the U.S.
Most centers are located in large cities or academic hospital institutions. If you don’t live close to one of these centers or if you live in a rural location, it might make it difficult to get timely routine care due to a lack of transport or time.
Racism and implicit bias. There’s evidence to point out that race-based bias or misconceptions can affect how health care workers treat or diagnose people of color, especially Black and Hispanic people.
In fact, researchers have found that doctors are twice as likely to downplay a Black person’s pain symptoms compared to all other racial groups. They’re also more likely to assume that Black and Hispanic patients misuse substances than white people.
Such false beliefs can lead to “implicit bias” – a form of automatic and unintentional viewpoint that can affect a doctor’s judgments, decisions, and behaviors in their treatment approach. Because of this, Black and Hispanic people are more likely to get low-quality health care compared to other racial groups.
Socioeconomic status. Your income level, job, and education, among other things, can impact your care. Being in a low income bracket can affect your ability to access quality health care, afford medications, and seek support, especially through a life-changing diagnosis like PAH.
Distrust in medical experts. “For African American communities, one of the major issues we encounter is that there’s a mistrust in the medical system. There’s the sense that when it comes to medicine that they are being experimented upon. I think historically, there are a lot of reasons for that such as the Tuskegee Experiment,” Perez says.
To counter that and bridge the gap, Perez says health care providers need to work on “establishing trust.”
“They will need a special kind of education for physicians and health care practitioners to be able to connect with that patient population.”
Lack of knowledge or training about PAH among doctors. There are many types (or groups) of pulmonary hypertension. PAH is one of five. You develop it when the arteries in your lung become thick and stiff, which makes it harder for your heart to pump blood through it.
According to Perez, when you develop PAH symptoms, they may look like lung-related breathing problems or heart failure in certain cases.
“The priority for the physician is to identify which type of pulmonary hypertension the patient suffers from. Because each of those has a different treatment approach,” says Perez.
If you start on medications that are not meant for PAH, prescribed off-label, or before you’re diagnosed by a pulmonologist (lung doctor) or cardiologist (heart doctor) who specializes in PAH, it could make your condition worse.
“It can lead to [issues] related to drug-to-drug interactions that may put the patient at risk for complications,” says Perez.
Lack of health insurance coverage. With or without health insurance, PAH treatment can be extremely expensive. Even more so if you have to pay out of pocket. In fact, on average, monthly medical costs for PAH treatments can range from $2,000 to $10,000.
Even if you have good coverage through your insurance, additional costs like copay, deductibles, office visits, and premium fees can add up.
Your ability to afford medications through health insurance coverage or lack thereof can greatly impact your family’s financial status and make it difficult to stick with the treatment plan in the long run.
Perez says lack of proper coverage could also mean you might not be able to get more effective therapies for PAH such as infusion therapies.
“There are generic [medications] available. But they’re still quite expensive, so a patient that does not have insurance is significantly limited in terms of what they can access,” Perez says.
You can also get partial to full coverage from government-based health insurance like Medicaid, Medicare, and insurance-linked with veterans for military service.
Immigration status and language barriers. Among minority populations, those who don’t speak English and are undocumented, especially if they also happen to live in rural areas, may have a hard time establishing or accessing any type of care, let alone having or using health insurance.
Without medical translators coupled with a lack of medical education or difficult medical terms used in a different language, the health outcome for PAH may be worse.
Strategies to Improve Access to PAH Care
In the 1980s, studies show the average survival rate for PAH was about 2.8 years. But today, with early diagnoses along with advancements in treatments, this number has significantly improved. About 65% of people with PAH have a 5-year survival rate after diagnosis.
As the access to proper care and early diagnosis is key, here are some ways to improve PAH-related health care for people of color:
Improve medical education. “I think the low-hanging fruit is education,” says Perez. Because the condition is both rare and hard to spot, to manage it, people with PAH have to make big lifestyle changes and understand the condition well. For this to happen, there needs to be a multi-pronged approach toward PAH care.
Including PAH-trained nurses, social workers, and case managers in the care team can be key. They can help educate people with PAH about:
- Ways to minimize falls during symptoms like shortness of breath, dizziness, or fatigue.
- How to use oxygen therapy and make lifestyle changes through diet or exercise to stay healthy.
- Help with resources to cut back or quick smoking and drinking alcoholic beverages.
- In-home care for people who have mobility issues due to PAH.
- How to check on their living situations to help modify them to better fit their new lifestyle with PAH.
Perez also suggests meeting people where they are. This means doing education outreach about PAH in certain geographical areas where people with low socioeconomic status tend to live. Community outreach can be done through local schools, religious organizations, and regional PAH advocacy groups.
Include PAH training for primary care doctors. Most general physicians don’t have training or specialized expertise in PAH and the medications required to treat it. To improve access to PAH care, it’s important for doctors to collaborate with PAH specialists like pulmonologists and cardiologists. This can help speed up referrals and ensure early diagnosis and treatment.
Telemedicine. While this is relatively new, telemedicine has become a useful tool, especially for those who live in rural areas and lack means of reliable transport. Telehealth through video or phone call meetings can help people continue PAH care in some cases.
Of course, it has its limitations, especially because PAH requires certain physical tests like electrocardiogram and 6-mile walk tests. It’s useful as an alternative option if necessary. PAH specialists will need to collaborate with regional doctors or health care providers to practice telemedicine.
Perez notes telemedicine may be especially hard if the person is very sick or lacks the basic skills necessary for internet use.
“We can still talk with the patient and get a sense of where they are symptomatic. I can make some decisions in regard to their care, but many patients may not have access to adequately stable Internet. They may not have the technical know-how to use the apps and the software. That limits our capacity to be able to deliver the adequate level of care,” Perez says.
Improve minority representation in studies. Clinical studies and patient registries in general have had low racial and ethnic minority representation in their data sets. Proper representation could help fill the gaps. To do so, questionnaires, forms, and studies should also be available in multiple languages as well as be accessible to those with disabilities.Studies should also include questions relevant to minorities to more effectively gather relevant information.
Promote support groups. A support group can be a useful resource to share advice and suggestions to cope with and manage PAH better.
It could also give you and your family members an outlet to share your experience, build community, and fill the gap between medical treatments and emotional support as you navigate PAH care. If you’re unable to go in person or find one near you, you can always connect virtually through online forums or attend video and audio meetings.
“When you try to explain [PAH] to other people, they don’t understand what it is. You tend to feel like your days are numbered or limited and that’s just not true,” says Creech.
“If you can get around other people that share the same symptoms as you and share the same diagnosis, and you see how other people manage and how they’re living … it does wonders.”