Updated PAH Treatment Guidelines Released by CHEST - Pulmonary Hypertension News

Posted: 13 Feb 2019 12:00 AM PST

An updated guideline with new recommendations for the treatment of patients with pulmonary arterial hypertension (PAH) was released by the American College of Chest Physicians (CHEST).

The free guide, titled "Therapy for Pulmonary Arterial Hypertension in Adults 2018: Update of the CHEST Guideline and Expert Panel Report," was published in the journal Chest.

PAH is a progressive lung disorder caused by increased pressure in the lung blood vessels. Available treatment options for the disease help alleviate the pressure, reduce inflammation, and improve blood flow.

Therapeutic guidelines help clinicians make informed decisions about the appropriate treatment for patients with PAH. The latest update is the fourth version of the guidelines and contains 78 recommendations for the proper use of therapeutics.

The previous version of the guidelines was published in 2014. For the most recent update, a team of clinicians — made up of pulmonologists and chronic disease experts — reviewed literature published after 2014 to identify studies discussing PAH treatment options and management.

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Findings were scored using a grading system, and the study panel recommended new guidelines based on these outcomes. Some recommendations that were not graded but had a high consensus (consensus-based statements) among the panel members were also considered in this update.

Furthermore, the expert panel also developed a guidance tool for clinicians (an algorithm) that can be used for the design and management of treatment regimens. The algorithm can also be used by readers to navigate the document to find specific detailed information.

"New recommendations and ungraded consensus-based statements were developed in this update based on new studies that were published since the 2014 guidelines," Deborah Jo Levine, MD, co-chair of the CHEST Pulmonary Arterial Hypertension Guidelines Committee, said in a press release. "In addition, an evidence-based and consensus-driven treatment algorithm was created to guide the clinician through an organized approach to management."

The panel included two new recommendations on the use of combination therapy to improve the patient's six-minute walk distance – a functional ability test that evaluates the walking distance covered by the patient within six minutes on a flat, hard surface. The two recommendations are:

In addition, two palliative care-related ungraded consensus-based statements were also part of the new guidelines. These are:

the inclusion of palliative care services in the management of PAH patients.
patient participation in exercise activity under expert supervision as part of their care regimen.

The panel concluded their guidelines by calling attention to the fact that "optimal use of new treatment options requires prompt evaluation at an expert center, utilization of current evidence-based guidelines, and collaborative care using sound clinical judgment."

Arkansas woman's diagnosis of lung illness sparks new support group - Arkansas Online

Posted: 08 Jul 2019 12:00 AM PDT

The bad news came during a heart catheterization test.

Thekla (pronounced TEK-lah) McGinley, 52, had been sick for months, getting out of breath when she walked uphill or jogged, sometimes feeling as though there were a belt around her chest. During a series of hospitalizations, the catheterization was just one test among many.

"[In the testing room], all of a sudden it's just quiet; you could hear a pin drop," she recalled in a recent interview.

"And they roll me out into the hallway, and a nurse comes out -- who had been behind the glass wall with the computers and everything -- and she took my hand and she said, 'I am so, so sorry.'"

The Bryant resident was diagnosed with pulmonary arterial hypertension. The illness is one of five types of pulmonary hypertension, a serious condition of increased blood pressure in the lungs.

When McGinley was diagnosed in 2013, some pulmonary hypertension patients were thought to have just a few years to live. But on that day, she says she told her nurse, "Don't cry for me. This is not the end of me."

McGinley is the founder of central Arkansas' first support group for people with pulmonary hypertension. The diagnosis is rare. Between 10,000 and 20,000 people in the United States are estimated to be living with its variants, according to the American Thoracic Society.

She started the group two years ago after frustrating experiences visiting health care providers who weren't familiar with the condition or its treatment. Between four and 10 patients, as well as their caregivers and family members, now attend its regular meetings.

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Although patients sometimes are too sick to show up and the group has experienced a few deaths, McGinley said she "make[s] sure there's a lot of laughter."

"You finally meet someone who's fought the same things you did," McGinley said. "Who's waited to die, who's waited for treatment, who doesn't know if what they're doing is right, who doesn't understand why their family doesn't get it."

On average, it takes about three years to be diagnosed with pulmonary hypertension, which is often linked to an underlying condition such as heart disease or a clotting disorder, said CHI St. Vincent cardiologist Dr. Muhammad Waqas.

That's in part because many of its symptoms, such as fatigue and shortness of breath, could have multiple causes. There also aren't many specialists in the "orphan" condition.

While patients await a diagnosis, Waqas said, pulmonary hypertension is remodeling the lungs' circulatory system to an abnormal structure -- one that can't be rolled back.

So support groups, such as McGinley's, can be critical for patients grappling with a long-term health problem.

"They share the ideas of the very important point of how to live with this condition," he said. "This is a chronic, debilitating condition which, over time, we know can kill patients."

"When they can connect with people with similar disease, it makes it easier for them to deal with."

At an April meeting, more than a dozen patients and caregivers from the central Arkansas group sat at long tables in a sunny room at U.S. Pizza Co. in North Little Rock. A few wore nasal cannulas to deliver oxygen from portable tanks. McGinley sported zebra ears, a print she said symbolizes pulmonary hypertension.

Group members said the gatherings bolster their spirits as they fight an illness few people have heard of, and which can be invisible to observers. Friends and family confuse it with ordinary high blood pressure, they said.

Patsy Fleniken, 77, said her friends tell her she looks good and ask if she's feeling better. They don't see when she's too weak to go for a walk, or can't attend an event if she isn't able to park her car close enough to the door.

"It's not going to get better," said Fleniken, who lives in Russellville. "[With this group], you know the saying misery loves company? It is amazing to be able to get around with people who understand and know what's going on."

Tammy Gaddy, a single mother from Hazen, went to the meeting with her teenage daughter after being diagnosed during a hospitalization in February.

"It's humbling, very humbling," the 46-year-old said of her diagnosis. "It's been an eye-opener."

"I plan to get more involved with my condition. ... I wanted to make sure that other people are aware of it," she said.

There also are support groups in Batesville and Springdale, according to the Pulmonary Hypertension Association website.

Several in the central Arkansas group said they now see Waqas after seeking therapy at clinics in Texas or Tennessee.

Some of those people were traveling hours for treatment, the doctor said. He wants to provide "local care" for a condition he says is gaining a higher profile, especially as people with heart and lung disease live longer.

"You diagnose anything more if you suspect it more. Now that we're suspecting it more, we're seeing it a lot more," he said.

He said the prognosis for patients with pulmonary hypertension is improving, citing the development of 14-15 medication options and the approval of a fully implantable drug pump.

McGinley said she's hopeful that one of those treatments will slow her illness.

She's invited legislators and officials to join the group in August to learn more about her condition, and several sent their aides to the same meeting last year. In 2017 Gov. Asa Hutchinson signed a proclamation declaring November pulmonary hypertension month.

Despite having a setback about every six months, McGinley said she's determined to support people who share her illness.

"My disease is progressing, but I'm not stopping. I will never stop," she said. "No one needs to go through what I've been through, trying to get help and trying to get treatment for this disease."

Metro on 07/08/2019

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