News

Low blood levels of a protein called brain-derived neurotrophic factor (BDNF) are associated with right heart failure in people with pulmonary arterial hypertension (PAH), a study reports.

Experiments in mouse models of PH, however, suggest that the low BDNF levels are a consequence, and not the cause, of right heart failure.

The study, “Decreased plasma levels of the brain-derived neurotrophic factor correlate with right heart congestion in pulmonary arterial hypertension,” was published in the journal ERJ Open Research. read more

The world is full of opinions, personalities, and attitudes. On my first day of nursing school, our dean told us to treat others as we’d treat our family or loved ones. How would we want nurses to treat them? This stuck with me, as I’ve tried to deal with others respectfully, no matter our differences.

If you know someone with a rare disease, you may want to help, but how? Think about how you’d like to be treated if the script were flipped.

Friends and family are essential to a patient’s support team. But they can have trouble fully understanding the day-to-day experience of living with a rare disease — in my case, pulmonary hypertension (PH). Relatives and friends mean well, but they’re outsiders, and they can unintentionally add to our burden with what they say and do. read more

It’s officially spring! Flowers bloom, rain falls, and allergies flare as we begin to emerge from our collective hibernation. Before formally springing forward, I took a vacation to Florida where I spent a week visiting different amusement parks, and my comfort level with asking for and using disability accommodations reached a new frontier.

The trip served as a maiden voyage for me — and not because I’d never been to Disney World. Rather, I wondered if visiting a large, crowded amusement park seven years after my pulmonary hypertension (PH) diagnosis would be a positive experience. read more

Second in a series. Read part one.

According to the Online Etymology Dictionary, “emergency” is derived from the Latin word “emergere,” which means to come forth, to rise up and out. That is what our brave emergency responders do. They rise up, emerge, and help patients and caregivers through situations they cannot handle on their own.

Last week, fire service paramedic Michelle Roche explained what to expect when first responders arrive at a scene.

Now we hear from Linda Wrigley, 50, a registered nurse, whom I interviewed via email. I asked Linda to share her experience as an emergency room (ER) nurse and offer advice for when patients and caregivers arrive at the emergency department. read more

A noninvasive measure of heart and blood vessel function called the TAPSE/PASP ratio could help prioritize people with pulmonary arterial hypertension (PAH) who may benefit most from a lung transplant, according to a new study.

“We demonstrated that the echocardiographic index of TAPSE/PASP … was lower in patients who had undergone [lung transplant] or died during the follow-up period, and was significantly associated with prognosis after referral [for transplant],” the researchers wrote in the study, “The ratio of TAPSE to PASP predicts prognosis in lung transplant candidates with pulmonary arterial hypertension,” which was published in Scientific Reports. read more

Researchers have identified a significant risk factor that affects the prognosis of people with chronic thromboembolic pulmonary hypertension (CTEPH) in a large 26-year study.

They found that elevated left ventricular filling pressure (LVFP), a measure of left heart function, was common in patients and predicted worse outcomes. Pulmonary hypertension (PH), or high blood pressure in the pulmonary arteries — those that supply the lungs — is typically associated with right heart malfunction. read more

Referral to a specialized chronic thromboembolic pulmonary hypertension (CTEPH) center for follow-up treatment after a pulmonary embolism (PE) — a blood clot that blocks an artery in the lungs — is more likely if patients live closer to the center and have access to a primary healthcare provider, a U.S. study found.

Patients not referred generally were older and had a higher rate of coexisting disorders. But neither sex, race, ethnicity, nor household income appeared to have an effect on whether or not patients were referred to CTEPH centers for further treatment. read more

A rare disease like pulmonary hypertension (PH) is challenging enough to manage. Add other coexisting conditions and it gets even more complicated.

I have several coexisting conditions, but my stage 3 chronic kidney disease (CKD) is the most difficult to manage alongside my PH. I must be organized and proactive​​ in responding to each condition.

In CKD, the kidneys are damaged and can’t filter blood properly. It is classified in five stages, depending on the severity of decline in kidney function. At each stage, taking steps to slow down kidney damage is essential. read more

Four genes associated with the metabolism of copper in the body have been identified as potential diagnostic biomarkers of pulmonary arterial hypertension (PAH), a new study reports.

Researchers used computer software tools to look for specific genes tied to copper metabolism that may serve as less-invasive markers for diagnosing PAH, by testing bloodwork.

“The results of this study may have significant implications for the development of new diagnostic biomarkers and actionable targets to expand treatment options for PAH patients,” the researchers wrote. read more

First in a series.

The rare disease community has an abundance of compliant patients and proficient caregivers. We do our best to avoid serious medical emergencies but are wise enough to know that we must also prepare for them.

Important healthcare information and prescription lists are diligently maintained and kept close at hand. To-go bags are packed and ready with the essentials needed for unexpected hospital stays. Many of us pray, meditate, journal, and even seek counseling to help ready our minds for worst-case scenarios. read more