Most people focus on the physical challenges of living with a rare disease like pulmonary hypertension (PH). However, conversations about mental and emotional health don’t get enough airtime, in my opinion. If you’re reading this and thinking that mental health issues like anxiety and depression aren’t intertwined with rare and chronic diseases, think again. In addition to physical symptoms, chronic illnesses affect our emotional well-being. The daily grind of juggling tasks, medications, doctor appointments, and more can feel like an insurmountable mountain. For those of us with PH, the stre ..read more

MACC1, a gene that’s been linked to overgrowth of cancer cells, is more active in the lungs of people with pulmonary arterial hypertension (PAH) than in healthy individuals, a study out of China found, suggesting it could be used as a diagnostic biomarker for the disease. Researchers also observed that knocking down MACC1 to reduce its activity in lab-grown smooth muscle cells — cells that wrap around blood vessels to control blood flow — made them grow less, move slower, and become more prone to death. It’s thought that smooth muscle cells act up early in PAH, so “these findings suggest that ..read more

Low levels of circular RNA Alstrom syndrome protein 1, or circALMS1, may be associated with poor outcomes with pulmonary hypertension (PH), a study suggests. This may be because low circALMS1 levels promote higher numbers and the migration of pulmonary microvascular endothelial cells (PMECs), and reduce the cells’ death. PMECs line the walls of small blood vessels and may factor in lung arteries’ lesions in PH. This may make circALMS1 a promising diagnostic and prognostic indicator in PH, say researchers in “CircALMS1 Alleviates Pulmonary Microvascular Endothelial Cell Dysfunction in Pulmonar ..read more

Nestled in my cozy swing chair on the patio, I’m soaking up the brisk wind that playfully tosses my hair. I’m listening to the sweet melody of my wind chimes, a thoughtful gift from my husband, Manny. Sunlight dances through the miniature stained-glass hot-air balloon atop the chimes. The color and light are like a beacon. The sun’s warmth and the gentle breeze announce spring’s arrival, a welcomed season of renewal. Now that the rainy season is over here in Southern California, I’m thankful for the sun. Warmer temperatures bring the promise of more days spent at my sanctuary, the beach, wher ..read more

Despite more women being affected by pulmonary hypertension (PH), men with the cardiovascular disease had worse right ventricular function and lower odds of transplant-free survival relative to their female counterparts, a study found. In a large group of patients with various types of PH, sex differences in function of the right ventricle (RV) were most notable where pulmonary vascular resistance (PVR), an indicator of resistance to blood flow in the vessels of the lungs, was not severely elevated. The study, “Male Sex Is Associated With Worse Right Ventricular Function and Survival in Pulmo ..read more

Most people with pulmonary arterial hypertension (PAH) due to connective tissue disease (CTD) who are using Opsumit (macitenan) do so as part of a combination therapy, according to real-world evidence from two U.S. drug registry studies. Its safety and effectiveness, in terms of clinical outcomes, for these patients were comparable to people with idiopathic (of unknown cause) or hereditary PAH, more common disease types. “We show that macitentan [Opsumit] is used in newly diagnosed and prevalent patients with CTD-PAH … as part of a combination therapy regimen in the majority of patients,” the ..read more

When living with a chronic illness, countless situations arise that require one to prioritize health above all else. When I was growing up and my friends and peers were expanding their horizons, I often decided to take a back seat to focus on self-preservation. These sacrifices became even more common when I attended college. I’d stay back while my friends went to Colorado for a concert, knowing the altitude would be hard on my lungs. When they spent a month traveling in Europe, a trip my doctors never would’ve approved, I remained on campus and studied. When weekend parties involved drinking ..read more

Winrevair (sotatercept-csrk), the under-the-skin injectable treatment recently approved in the U.S. for adults with pulmonary arterial hypertension (PAH), is expected to be available for dispensing at CVS and Accredo pharmacies by late April, Merck reports. Winrevair’s distribution network will continue to be evaluated after its commercial launch, a Merck spokesperson said in a written Q&A with Pulmonary Hypertension News. Winrevair binds to and traps activins, proteins that trigger the growth of the endothelial cells that line blood vessels. In PAH, this cell layer keeps growing, narrowi ..read more

Having pulmonary hypertension (PH) worsens the likelihood of potentially life-threatening cardiac and respiratory complications in people with human immunodeficiency virus (HIV) infection, a U.S. database study reported. Its findings “can inform clinical practice and help healthcare providers make more informed decisions regarding the screening, diagnosis, and management of PH in patients with HIV,” the researchers wrote. The study, “In-hospital outcomes of pulmonary hypertension in HIV patients: A population based cohort study,” was published in the International Journal of Cardiology. Recom ..read more

Inhibikase Therapeutics is meeting with the U.S. Food and Drug Administration (FDA) April 5 to discuss the potential of its experimental formulation of imatinib, dubbed IkT-001Pro, for pulmonary arterial hypertension (PAH). “Following our … discussion with the FDA related to the path to approval for IkT-001Pro in up to 11 blood and stomach cancers in January, we requested an additional FDA meeting with the Division of Cardiology and Nephrology to discuss Pro as a treatment for Pulmonary Arterial Hypertension,” Milton Werner, PhD, president and CEO of Inhibikase, said in a company press releas ..read more