Closer Look
His rare disease runs through generations. So he built a community around it
Photo illustration: Casey Shenery for STAT
By the time Jack Johnson was 7, he'd already seen many family members struggle with Fabry disease, a rare condition that was beginning to affect him, too. It causes the fatty lipid Gb3 to build up in the body, causing gastrointestinal issues, kidney failure, stroke, chronic pain, and dangerous heart abnormalities. Growing up in Bakersfield, Calif., where his job on the family farm was to cool down the pigs in scorching weather, he could sympathize because he couldn't sweat, either.
Now a husband, small farmer, and father to two sons, he leads the online the Fabry Support & Information Group. "The disease affects everybody differently in many regards and people have to be able to make their own decisions," he told STAT's Isabella Cueto. "We just try to give them the information that they need to be able to do that." Read more.
insurance
New White House guidelines take aim at sticker shock in health care
New guidelines issued by the Biden administration seek to take the "surprise" out of surprise billing in health care, going beyond 2020 restrictions and their loopholes while also taking aim at short-term insurance policies that often fail to cover essential treatment. Also on the hit list are high-interest credit cards and payment plans specifically marketed to help patients cover medical debt. Another target: facility fees, surprise bills tacked on on by hospitals when they provide services in an outpatient location.
The White House intends to force health plans and providers to share information about these fees, with the goal of making them more transparent and less of a shock to patients who might not be informed about the fees ahead of time or even be familiar with the concept. Whether that will translate into savings for patients is another question, STAT's Annalisa Merelli reports, reminding us that hospital price transparency has had limited impact. Read more.
emergency medicine
Pre-hospital dosing of emergency medications for kids falls short of guidelines, study says
In emergencies, children may need medication on their way to the hospital. A new study published today in Pediatrics found dosing of 10 common medications varied widely, often deviating from national guidelines that are determined by weight. Based on its review of more than 990,000 electronic medical records from roughly 2,000 U.S. emergency medical services, the researchers conclude that these drugs were given correctly only 42.6 out of 100 times.
In most cases, the differences were underdoses, particularly for sedating medications such as benzodiazepines and morphine, likely from concern about overdoses. But underdosing can be dangerous for children having seizures or asthma attacks. The authors also note that estimating weight when it isn't known can be an obstacle to getting the dose right. Citing other work in addition to their own, they say "there may be even greater opportunities for improving dosing of pediatric medications in prehospital compared with in-hospital acute care settings."
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