Reexamining hemophilia emergency protocols and the need for advocacy

I spent part of a day this week trapped in my condo’s elevator. Since Hurricane Ian hit my town of Punta Gorda, Florida, in September 2022, the elevator has been temperamental, frequently trapping people inside.

With high humidity and the outdoor temperature at 98 F, I was hot inside the elevator. But I’d followed the advice I give my elderly neighbors: “Always have your phone with you when you ride the elevator.” I usually add “and carry water,” but I was only going to retrieve my mail, so I didn’t have any with me.

The elevator had made a loud noise and then stopped. I then called 911, and the fire department freed me in 20 minutes. It was amazing how seriously they take elevator entrapments. A firefighter suggested we contact our elevator company, because he’d just freed a neighbor of mine last month. Yet despite our repeated calls, that company hasn’t found anything wrong with the elevator.

But what if this occasion had been an emergency involving bleeding and complications from my hemophilia B and von Willebrand disease? I’ve learned to self-infuse to avoid an emergency room at all costs, but what if I needed an ambulance and couldn’t administer my medication?

As I was reflecting on these questions, I thought about an organization I’d discovered while attending national hemophilia events: Danny’s Dose.

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Danny’s Dose

The “Danny” of Danny’s Dose was diagnosed with severe hemophilia B in 2011. His family didn’t have a history of hemophilia, so the news prompted confusion and fear.

Danny’s grandmother, Darlene Shelton, worked hard with the other members of Danny’s family to understand his diagnosis and implement safety plans, which included affixing a MedicAlert ID to his car seat and putting together a package of medical orders, medication, and dosing instructions for paramedics and emergency room staff.

Shelton assumed that in the event of an emergency, paramedics would treat her grandson’s hemophilia B with his prescribed clotting factor, according to his doctor’s orders. She was shocked to learn that paramedics weren’t permitted to administer Danny’s life-saving medication, and the nearest emergency room equipped to treat him was two and a half hours away. Determined to address this issue, the family decided to raise awareness about it and contact local lawmakers.

In 2014, Shelton started Danny’s Dose, a 501(c)(3) dedicated to updating emergency protocols for chronic illnesses and rare diseases. Its mission is to raise awareness about the current gaps in emergency treatment for people with special medical needs, advocate for changes to outdated protocols, educate emergency service personnel, and assist affected families with proper planning and protection.

According to Danny’s Dose, “Every day is the right day to go meet your local EMS [emergency medical services] team and connect with your local [emergency department].” Shelton travels the country, speaking and exhibiting at events for people affected by rare diseases. The Danny’s Dose website offers additional tips to help families be prepared.

As Shelton told Rare Disease Advisor:

“Families must go meet with their local EMS service and say, ‘Hey, I live in your area and our family has this condition,’ and ask for a treatment plan. That means everybody is going to get together, you’re going to have meetings, they’re going to contact your specialist, and they’re going to develop a plan of action for when an emergency happens, and know exactly who your doctor is, know exactly the best emergency room to go to.”

Reflecting on my own experiences, I’m reminded of the importance of being prepared and the critical role emergency services play in our lives. Clearly, our systems need to be reliable and our protocols updated to ensure safety and prompt responses for everyone, especially those with special needs. Kudos to Shelton and the folks at Danny’s Dose for accomplishing change and raising awareness.

Note: Hemophilia News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or another qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Hemophilia News Today or its parent company, Bionews, and are intended to spark discussion about issues pertaining to hemophilia.