News

Oxygen therapy may help relieve lung pressure in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are about to undergo balloon pulmonary angioplasty, a procedure in which balloons are used to open blood vessels that have been chronically narrowed or blocked, according to a new study.

Researchers found a decrease in mean pulmonary arterial pressure (PAP) occurred independently of treatment with vasodilators, meaning oxygen therapy could be helpful to maximize the safety of angioplasty. read more

People with pulmonary hypertension (PH) who start treatment with opioids to manage pain after undergoing surgery are at high risk of continuing to take these addictive medications for longer than required, new research suggests.

Indeed, hypertension was one of eight specifically identified “top risk factors” for “new persistent post-surgical opioid use,” according to researchers, who said intervention measures could be key for prevention.

“Our findings identify which patients would most benefit from effective preoperative consultation and should be followed especially carefully after surgery,” Gia Pittet, PhD, a researcher at the University of California, Los Angeles (UCLA), and the study’s lead author, said in a press release. read more

As I prepare for a small diagnostic procedure this week, I’m noticing that my anxiety has skyrocketed. This is happening for two reasons.

The first is that I have a full memory bank of physically uncomfortable and painful procedures that have made me quite sensitive. My years with illness have allowed me to develop a high pain tolerance, but they’ve also triggered my flight response. The idea of anything invasive makes me want to run in the other direction.

The other reason for my anxiety is what this procedure might reveal. The unknown means that anything is possible, and as someone who has been rare for most of her life, my brain has become excellent at developing each and every “what if” — even those that are unlikely or downright absurd. read more

Living with pulmonary hypertension (PH) is a constant challenge. If I’m not educating myself, I’m educating my family and friends, and at times, even my medical team. Yes, the members of my medical team are experts in medicine, but I am the expert of my own body.

Recently, I started a new treatment my neurologist ordered to help clear up brain fog and problems with concentrating, both lingering effects of having COVID-19. Unfortunately, after a month, I didn’t see any improvement. I started tasks, but wouldn’t finish them. I felt like a hamster running on a wheel and suffered from poor appetite, an irregular heartbeat, and increased fatigue. read more

The U.S. Food and Drug Administration (FDA) has cleared Tenax Therapeutics to begin clinical testing of its new oral formulation of imatinib in people with pulmonary arterial hypertension (PAH).

The company is planning to conduct a small pharmacological study this year — and then launch a Phase 3 clinical trial by June 2022 to test the therapy’s efficacy.

“Tenax is thrilled we can now advance our imatinib PAH program towards pivotal testing,” Christopher Giordano, Tenax’s CEO, said in a press release. “We remain on track to be the first company to commence a Phase 3 trial of a new formulation of imatinib in patients with PAH.” read more

I’ve always considered myself an “on” person, somebody who says yes to the boss or co-worker, friend or relative, thing or event asking for my attention and energy. I’m like a faucet running all the time, never slowing down to make time for myself.

Being on all the time fills my schedule to the brim, yet I have never felt like that pace sapped my energy. The faucet would flow and I would attend to most of what needed to be done, both professionally and personally, and wake up and repeat that cycle the next day. Then one spring day in 2016, I found myself in urgent care. Nearly two weeks later, I received a diagnosis of pulmonary hypertension (PH). read more

The Rare Disease Diversity Coalition (RDDC) awarded $600,000 in grants to ease the disparities faced by rare disease patients of color.

These Impact Rare Disease Solution grants will go five RDDC steering committee working groups, which aim to identify problems for rare disease communities and advocate for solutions.

The five RDDC groups are:

• Delays in diagnosis and treatment working group
• Research and clinical trials working group
• Provider education and engagement working group
• Patient and caregiver working group
• Government regulation, legislation, and policy working group

According to the RDDC, people of color with rare diseases face disproportionate challenges in accessing quality care, and are underrepresented in research and clinical trials. read more

Have you ever met someone and instantly felt like you’ve known them forever? I’ve experienced a rather twisted version of this.

In a previous column, I described pulmonary hypertension (PH) as a toxic person rather than a disease. PH had such an immediate impact on my family that by the end of the first year of my son Cullen’s diagnosis, it felt like it had been a part of our lives for much longer.

But something just happened that has me viewing my relationship with PH differently: I turned 50. read more

Infusion with Ferinject (ferric carboxymaltose) safely treated people with pulmonary arterial hypertension (PAH) and iron deficiency, easing disease symptoms, improving exercise capacity, and lowering their number of hospital visits over a period of 18 months, a long-term study reported.

Ferinject, as this treatment for iron deficiency anemia is known in Europe, is available in the U.S. under the brand name Injectafer. It is approved for people who fail to respond sufficiently to oral iron supplements, or are unable to take such supplements. read more

Targeting a protein called p110a, a part of the PI3K family of enzymes, may be useful for preventing or even reversing pulmonary hypertension, research done in cells and rodent models showed.

“Targeted inhibition of [p110a] offers a disease-modifying treatment approach, which is readily accessible by small molecule inhibitors and warrants further evaluation in clinical trials,” the researchers wrote.

Results of the study “Disrupted PI3K subunit p110α signaling protects against pulmonary hypertension and reverses established disease in rodents,” were published in The Journal of Clinical Investigation. read more