News

physical activity

A three-month, unsupervised health intervention based on a commercial activity tracker and motivational texts significantly increased daily step counts and improved quality of life in adults with pulmonary arterial hypertension (PAH), according to data from a single-center trial in the U.S.

Since physical activity has been shown to improve clinical outcomes in people with pulmonary hypertension, the findings highlight the potential benefits of this inexpensive and safe intervention in these patients. read more

birthday, survival, nurses

I still remember sitting on the couch with my mom on the eve of my 15th birthday. We were talking about how old I was getting, and after a brief pause, she said softly, “Every year for you is a miracle, you know.”

I don’t remember how I responded, but I do recall every detail of that moment because at that age, it was the closest my mom had ever come to talking openly with me about my prognosis. She said it not as a warning, but with great awe and tenderness.

Growing up, my parents raised me with bravery amid my pulmonary hypertension diagnosis. My mother was never eager to talk about statistics. She refused to believe they applied to me. And so moments like this, when we were upfront about my circumstance, did not happen frequently. read more

rest, dating, PTSD, grief, workplace, fear, interview, less is more, unique, covid-19

Do you find yourself scrolling through emails or multitasking when you sit down to rest? If you can’t seem to stop working or focusing on tasks, you are not alone. Our society has a troubling dilemma with overworking.

Recently, I experienced an epiphany of sorts, and I’m now learning to rest fully and unplug on some days. Well, maybe not an entire day yet, but at least a few hours. It’s a start.

Those of us in chronic disease communities often refer to the “spoon theory to help others relate to our challenges with fatigue. The spoon theory is a metaphor to explain our finite daily energy, which we refer to as “spoons.” Each activity requires a number of spoons that can only be replaced by resting. read more

PAPP monitoring system

A new metric for assessing cardiac health, called pulmonary artery proportional pulse pressure or PAPP, could help to identify people at high risk of hospitalization or death due to pulmonary hypertension or heart failure, allowing for more timely and effective treatment, a study reported.

Researchers at the University of Virginia (UVA), who developed PAPP, called it a “simple measure” that could be useful in identifying at-risk patients, so doctors might “tailor more aggressive treatments” for them, such as a heart monitor implant, according to a university press release. read more

Eurordis opened a campaign, called Rare 2030 Action, that is seeking to establish a European action plan for rare diseases to ensure that none of the 30 million people in Europe living with rare diseases are left behind by the start of a new decade.

As part of the campaign, rare disease patients and their caregivers, family members, and others are being invited use an online form to tell Ursula von der Leyen, president of the European Commission, why Europe needs to act now to ensure a better future for them by 2030. read more

nurse, bully, pets, community, personality, double-lung transplant, limitations, marriage, transplant, wise, phantom ph, gaming, weather, identity, gifts, laughter

Nurses are remarkable people. My respect for them has grown significantly throughout my son’s pulmonary hypertension (PH) and transplant journey.

Their job requires important skills, intelligence, patience, persuasiveness, compassion, energy, emotional control, a strong stomach — the list is endless. And I can’t think of a more suitable person to fulfill these qualifications than a mother who has experience caring for a PH child.

Carrie Walls is one of those remarkable people pursuing nursing, all while caring for a child battling PH and several other rare diseases. In an email interview, Carrie discussed both experiences and how she manages them. read more

atypical PAH

Treatment with a single medication (monotherapy) may be sufficient to help manage people with “atypical” pulmonary arterial hypertension (PAH), according to an analysis from four clinical centers in Germany.

One-year survival of adults diagnosed with “atypical” PAH — those older than 65 and with multiple risk factors for heart disease — did not differ for patients on monotherapy compared with patients taking dual or triple combination therapies.

The study, “Monotherapy in patients with pulmonary arterial hypertension at four German PH centres,” was published in the journal BMC Pulmonary Medicine. read more

LIQ861 for PAH

The U.S. Food and Drug Administration (FDA) has accepted an updated application asking that the dry-powder inhalation therapy LIQ861 (treprostinil) be approved to treat pulmonary arterial hypertension (PAH).

Liquidia Corporation, the therapy’s developer, first requested approval in April 2020, but the FDA asked for more data on LIQ861 in a complete response letter issued in November.

Specifically, the regulatory agency requested more information and clarification of the medicine’s chemistry, manufacturing, and controls. Additional data also had to include further evidence of the safety of the portable device used to deliver LIQ861. No further clinical trial data or information regarding the therapy’s pharmacology or toxicology were required. read more

Ahead of this year’s Rare Disease Week on Capitol Hill, held virtually July 14–22, the EveryLife Foundation will award grants to top advocates of rare disease organizations who participate in the week’s pre-events.

The top 50 point-earners will be eligible to win $1,000 to $5,000in  grants, totaling up to $100,000, the foundation announced in a release.

The pre-event Meet and Greet Session, to be held June 8 and 9, brings together regional and state-wide rare disease advocates to interact in virtual games and prize sessions. Registration is available through this link. read more

PH probability and pregnancy

Pregnant women with high pressure in the pulmonary artery of the heart, but who are not considered at risk of pulmonary hypertension (PH), are likely to also be at lower risk of other major cardiac problems, researchers reported.

In this study, the level of PH probability was judged based on an echocardiogram — a non-invasive and common test for PH that uses sound waves to show how the heart is working. Its findings suggest this test could help to identify those at higher PH risk, who may require further invasive testing and specialized medical care. read more

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