Seven years ago, a big gust of wind off the Wyoming plains sent a piece of plywood flying, hitting the back of Spencer Straub’s skull and knocking him out. When the 23-year-old came to, he was no longer in the family lumberyard in Cheyenne, where he still works, but in the emergency room (ER). And what happened there added insult to injury.
Even though the ER staff knew Straub had mild hemophilia B, they didn’t give him factor right away. When his mother finally convinced the staff to contact his hemophilia treatment center (HTC) in Denver, the ER doctor told the HTC nurse about the calf muscle bleed the plywood also caused but not the injury to Straub’s head and neck. The resulting treatment delay, says Straub, could have caused a fatal intracranial hemorrhage.
“Some doctors have the mindset, ‘You’re not going to come into my office and tell me how to do my job and how to treat you,’” says Straub, now 30, married and father of a three-year-old son. “You just have to be the best advocate you can be.”
Like Straub, many patients with bleeding disorders and their families find trips to the ER an exercise in frustration. Hospitals may not stock the factor they need or have staff who know how to administer it. Physicians and nurses may not be familiar with bleeding disorders and therefore fail to act appropriately, with potentially deadly consequences. Further, ER staff may not trust patients and parents to know what’s best for themselves.
Fortunately, HTC staff are reaching out to ERs to educate them about bleeding disorders and their treatment. And there's plenty individuals and families can do to help ensure that a trip to the ER goes well.
Educating ER Staff
Joanna A. Davis, MD, medical director of the pediatric clinic at the University of Miami’s HTC, is one doctor who's taking action. The fact that ER staff members aren’t familiar with bleeding disorders isn't necessarily their fault, she says. Medical and nursing school curricula don't cover bleeding disorders in depth. Even the staff at some large ERs may not see people with bleeding disorders that often. Finally, the needs of patients with bleeding disorders may not seem as urgent as other cases.
"When you're in an ER setting, you may have someone with a gunshot wound, someone with a heart attack," says Davis. A patient complaining of a knee bleed may seem like a lower priority compared to such cases, she says. In the educational sessions Davis offers, she emphasizes that injuries in people with bleeding disorders should be a triage priority. With funding from Novo Nordisk, Davis gives hour-long presentations to ER staff on hemophilia signs, symptoms and treatment. A similar series, funded by Octapharma Plasma, focuses on von Willebrand disease.
Medical staff may not understand that the lack of dramatic outward signs like huge swollen joints doesn’t mean nothing’s happening internally, says Davis. "In the early stages, emergency room staff may not see anything,” she says. But the lack of visible signs of a bad bleed can be deceiving.
As a result, ER staff may ask patients to wait or do CT scans, X-rays or other diagnostic procedures first. Both strategies are mistakes. "You can lose someone that way," says Davis. "I tell ER staff, ‘Treat first, then do everything else later.’" She also warns against invasive procedures, such as withdrawing blood from joint bleeds, until patients receive factor.
Davis also urges ERs to allow patients to bring their own factor, in its original packaging and with documentation from their HTC, so they can be sure they get exactly what they need. Because factor is so expensive, she also encourages ER staff to use the whole dose rather than discarding leftovers.
Becoming an Advocate
As a patient, there are things you can do to make a trip to the ER successful, says Jennifer H. LaFranco, RN, BSN, vice president of clinical programs at the Mary M. Gooley Hemophilia Center in Rochester, New York. For one, contact your HTC as you head to the ER, so HTC staff are in the loop and can call ahead about your diagnosis and treatment needs. Then grab a "go bag” you’ve prepared for yourself or your child (See sidebar, “Your Personal Go-bag.”). Even more important: Familiarize yourself with your diagnosis, brand of factor, dose and frequency. That way you can be an advocate for yourself or your child, says LaFranco.
LaFranco’s trainings for local ER staff emphasize the need to listen to patients. Patients and parents know what works for them, she says, but ER staff don't always view them as the resources they are. "Don't be afraid to speak your mind," says LaFranco. If there are any questions or problems, call your HTC for back-up, she says.
Learning to advocate for her child was a struggle for stay-at-home mom Jennifer Ruklic, 36. She lives outside Calgary in Alberta, Canada, with her husband Willie and two children—Cassie, 8, and Carter, 4. Carter, who has severe hemophilia A, spent his toddler years in and out of the ER. Sometimes the triage nurse would ask the family to wait.
“Growing up as a child, I was always told, ‘Doctors know best; just do what they say,’” says Jennifer. “It was hard to break out of that.” Then Jennifer realized that not doing so would mean her son wasn’t getting the care he needed. The last time Carter visited the ER—for a head bleed after bumping into another child playing tag in November 2011—Jennifer was calm but assertive as she successfully persuaded ER staff to treat him right away.
Parents who are agitated or angry can prompt ER staff to respond in kind, says Jennifer. “You can’t shout at someone and get results,” she says. “It’s hard when it’s your child, and you want to make sure they’re safe. But it helps nobody when you’re frantic.”
Establishing a good relationship with the ER is also helpful. Jennifer counts herself lucky that Carter’s hemophilia clinic and the ER are both at Alberta Children’s Hospital, so that ER staff has access to his full medical records. Patients and parents who aren’t so lucky may want to visit ER staff before an emergency strikes to introduce themselves and prevent potential frustration in the future.
Jennifer also rewards good behavior. After a positive ER experience a few years ago, she delivered a thank-you note and chocolates to ER staff. “Two and a half years later I still get hugs from that ER nurse,” says Jennifer.
- Read MASAC Document #175, “Guidelines for Emergency Department Management of Individuals with Hemophilia.”
- Read MASAC Recommendation #210, “MASAC Recommendations Concerning Products Licensed for the Treatment of Hemophilia and Other Bleeding Disorders.”
- “Head Bleeds and Hemophilia,” HemAware, 2011.
- Contact HANDI, NHF’s information resource center at 800.42.HANDI or firstname.lastname@example.org for an NHF “readiness card.” Fill it out and keep it with you at all times so that you’ll be prepared for an ER visit. Also request the “Family Emergency Kit Checklist,” available in English and Spanish. This brochure contains important information and checklists to help families prepare for emergencies.