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Unresolved Respiratory Symptoms? Get The Right Tests And Care

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Sep. 9—(StatePoint) Each year, 500 to 1,000 people in the United States are diagnosed with pulmonary arterial hypertension (PAH), a rare lung disease that can be fatal if left untreated.

To encourage earlier diagnosis of PAH, the American Lung Association, with support from Johnson & Johnson, is sharing information about the disease, its symptoms, and the importance of testing and proper treatment. Here's what the Lung Association wants you to know:

What is PAH? PAH is a rare, progressive lung disease caused when the tiny arteries in your lungs become thickened and narrowed. This blocks blood flow through your lungs, causing the blood pressure in your lungs to rise. The heart has to work harder to pump blood through the narrowed arteries. Over time, your heart has difficulty effectively pumping blood through your body.

What are the symptoms of PAH? In the early stages, you may not notice symptoms. As PAH progresses, common symptoms include shortness of breath, especially with activities like walking up the stairs or long distances, fatigue, edema (swelling of the feet, legs and eventually the abdomen and neck), dizziness and fainting spells, chest pain, heart palpitations, and lips and fingers turning blue. Contact your healthcare provider if you're experiencing these or any worrisome symptoms.

Who is most at risk? While anyone can get PAH, those most at risk are women ages 30-60, and Black and Hispanic women.

Why is PAH difficult to diagnose? Because symptoms of PAH are similar to other diseases, the average time it takes to get a diagnosis is about 3 years. This delay can be frustrating for patients and their families, and can result in poorer health outcomes. Unfortunately, health disparities exist due to a wide-range of factors, like lack of access to healthcare, health insurance coverage, transportation to specialists, and health literacy, and can exacerbate these delays. Increasing awareness of this rare disease can help patients get a quicker diagnosis so they can get the treatment they need.

Elva V., a mom and nurse, has been living with PAH since she was 23. She shares that getting the correct diagnosis and treatment plan has been a "game changer," helping her "feel well enough to do the things I enjoy and live a good life with my daughters."

How can the path to diagnosis be shortened? Your provider may order several tests to help with diagnosis:

—A simple blood test, the BNP test (also called B-type natriuretic peptide test) or NT-proBNP test, can help determine if your heart is working harder than it should, a sign that you may have PAH. Getting this test done early can help speed the path to diagnosis.

—Electrocardiogram to show the electrical activity of your heart.

—Echocardiogram to check the size and condition of your heart.

—Lung function tests.

—A right heart catheterization to confirm PAH, once your provider suspects it.

How is PAH treated? PAH-specific medications come in multiple forms: oral, inhaled and subcutaneous. Some allow blood to flow more easily through the arteries of your lungs. Others improve heart and lung function. PAH medications help slow how quickly your disease worsens. For optimal health outcomes when dealing with a rare disease like PAH, it's best to see a specialist.

"When seen by a specialist, you'll receive the latest treatment and resources to help you live your best life," says Dr. Michael Cuttica, a pulmonary hypertension specialist.

That is certainly the case for Lindsay T., who has been living with PAH since her early twenties and works with her doctor to manage her care. "My BNP has been within a normal range since I alerted my specialist that my symptoms were getting worse and he adjusted my medications," she says.

If you have unresolved respiratory symptoms that are not improving with your current treatment, learn more about PAH at Lung.Org/pah.

A PAH diagnosis doesn't have to be delayed. With the right tests and care from a specialist, you can get on the path to the treatment you need.

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Novel Blood Test Helps Evaluate Severity In Pulmonary Arterial Hypertension, A Rare Lung Disease

Researchers at the National Institutes of Health have found that a novel blood test can be used to easily evaluate disease severity in patients with pulmonary arterial hypertension (PAH) and predict survivability. PAH is a rare, life-threatening condition that causes unexplained high blood pressure in the lungs. In early clinical studies, the researchers showed the test to significantly improve upon conventional tests, some of which use invasive tools.

The new blood test measures DNA fragments shed by damaged cells. Researchers found that these fragments, called cell-free DNA, were elevated in the blood of patients with PAH and increase with disease severity. If future studies confirm the findings, this first-of-its-kind blood test for PAH patients could allow doctors to intervene faster to prevent or delay progression of the disease and possibly save lives. Cell-free DNA is a relatively new analytical technique that is growing in its potential medical uses, which include the early detection of heart- and lung-transplant rejection as well as early detection of cancer.

The study was funded by the National Heart, Lung, and Blood Institute (NHLBI) and the NIH Clinical Center, both part of NIH. The findings will appear online in the journal Circulation, a publication of the American Heart Association.

PAH is a rare form of pulmonary hypertension that can cause difficulty breathing, chest pain, and fatigue. The disease, whose exact cause is unknown, is estimated to affect less than 50,000 people in the United States, according to the NIH's Genetic and Rare Diseases Information Center. It is characterized by progressive narrowing and blockage of the small pulmonary arteries of the lungs, strain on the right side of the heart, and eventual death from heart failure. The damage to the lung in severe cases can require lung transplantation. Patients with PAH have a high death rate, and the condition mostly affects women. Despite treatment advances, it currently has no cure.

Current tests used to monitor PAH severity rely on established risk prediction scores based on clinical symptoms and on the use of an invasive catheter to measure pressure in the lungs. Doctors sometimes use echocardiography, or heart imaging, to measure pressures in the heart as an indirect measurement of lung pressure, but these tests tend to lack reliability and sensitivity.

"Researchers have been actively searching for novel, less-invasive approaches to evaluate PAH severity, disease progression, and response to therapy for more than a decade. These cell-free DNA analyses represent progress toward that goal," said study co-author Michael A. Solomon, M.D., M.B.A., who is part of the NHLBI Cardiovascular Branch and co-director of the NIH Clinical Center Pulmonary Arterial Hypertension Section.

Sean Agbor-Enoh, M.D., Ph.D., study co-author and chief of the NHLBI's Laboratory of Applied Precision Omics, agreed. "Here we're proposing a one-time test where you collect a vial of blood from a patient and use that to predict survival. We're very encouraged by the early results."

In the current study, the research team analyzed cell-free DNA from blood samples taken from 209 adult patients, predominately women, diagnosed with PAH at two large U.S. Medical centers. The researchers compared the results to cell-free DNA measured from a control group of 48 healthy volunteers without PAH at the NIH Clinical Center.

They found that cell-free DNA was elevated in patients with PAH, and also found that cell-free DNA concentrations increased in proportion to the severity of the disease. Patients with the highest level of cell-free DNA had a 3.8 times greater risk of either death or a need for lung transplantation compared to those with the lowest level of cell-free DNA, the researchers said.

Further analyses of cell-free DNA samples revealed that multiple tissue types -- including the heart, blood vessels, fat tissue, and inflammatory cells circulating in the blood -- were affected by PAH. The new blood test will allow researchers to better pinpoint the specific tissues involved in the PAH disease process. This knowledge may lead to new drug interventions for PAH, whose current treatment options may slow but not halt or reverse disease progression.

In addition to funding from the Intramural Research Program of the NHLBI, this research is supported by the NIH Clinical Center Research Award for Staff Clinicians Program, the NIH Distinguished Scholar Award, the Lasker Clinical Research Scholars Program, and the Intramural Research Programs of the NIH Clinical Center.

Dietary Changes May Treat Pulmonary Hypertension

Blood vessels in the lungs aren't like the others in the body. This difference becomes clear in pulmonary hypertension, in which only the lungs' blood vessels stiffen progressively, leading to chronic lung disease, heart failure and death. The underlying reasons for this organ-specific vessel stiffening remained a mystery until University of Pittsburgh researcher Stephen Chan and colleagues made a surprising discovery about these blood vessel cells in patients with pulmonary hypertension -- they're hungry.

Chan, Vitalant Chair in Vascular Medicine and Professor of Medicine in the Division of Cardiology at the University of Pittsburgh, and his team collaborated with the team of Thomas Bertero at the Université Côte d'Azur in France. They found that hypertensive pulmonary blood vessel cells have a voracious appetite for two amino acids, glutamine and serine, and -- as happens with any unbalanced diet -- there are consequences. This metabolism of glutamine and serine is a key driver of pulmonary hypertension disease progression.

Amino acids are the building blocks of proteins, which help build cellular structures, carry out biological functions, and regulate tissue and organ function. As hypertensive pulmonary blood vessels metabolize glutamine and serine, they create two new amino acids, called proline and glycine. Proline and glycine are the primary building blocks of collagen protein, which makes up 30% of our body's total protein and provides a structural framework for our skin, muscles, bones and connective tissues. The appetite for glutamine and serine and the resulting elevated levels of proline and glycine in hypertensive pulmonary blood vessel cells drive the overproduction of collagen, which leads to vessel stiffening and impaired function -- the hallmark feature of pulmonary hypertension.

Using rodent models for the disease, the researchers saw that drugs that limit cellular uptake of glutamine and serine deprived hypertensive pulmonary blood vessels of their craving. In turn, the lack of cellular glutamine and serine metabolism halted the excess production of collagen building blocks and collagen production. Knowing amino acids are most often absorbed through our diets, the team also discovered that reducing the dietary intake of glutamine- and serine-rich foods helped reduce collagen overproduction.

"For the first time, we have a dietary maneuver that may serve as an effective therapy for the disease," says Chan, who also directs the Vascular Medicine Institute and Center for Pulmonary Vascular Biology and Medicine at the University of Pittsburgh School of Medicine and UPMC.

For patients with pulmonary hypertension, avoiding foods rich in serine and glutamine, or eating foods with these amino acids depleted, might bolster the effectiveness of current medications. "It opens up a new way that we could treat this disease, because now -- instead of just relying on medications and transplantation -- there are possibly effective lifestyle interventions," says Chan.

Chan's team also harnessed the characteristic appetite of these cells to create a new diagnostic test for pulmonary hypertension using positron emission tomography (PET) scan technology and a glutamine imaging tracer. The imaging tracer acts like a GPS monitor to track where glutamine goes in the body. As a result, cells hungry for the amino acid light up on the PET scan, and the intensity of that light shows how ravenous cells are for glutamine and where those cells are in the body. This screening will enable earlier disease diagnosis and implementation of lifestyle and pharmacological interventions and allow doctors to check the efficacy of medications in slowing disease progression.

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