Carter Lantz, who was diagnosed with hemophilia A at age 2, learned to self-infuse factor at a hemophilia camp when he was around 7 years old. With the support of his parents, he did well with his treatment in childhood.
But when he got to middle school and started juggling schoolwork and sports, time management became an issue. “I’d miss an infusion just because I wasn’t thinking about it,” admits Lantz, now 19 and a sophomore at Ball State University. “And then I’d throw my shoulder out playing tennis or something. Just about every season there was a wrench in the plan.”
Lantz is far from alone. Even the most well-intentioned people with hemophilia can struggle to stick with their prophylactic factor replacement. Just around half of nurses at hemophilia treatment centers (HTC) who were surveyed in 2010 reported that most of their hemophilia type A and B patients met the definition for adherence (defined as taking prescribed doses 80% and 78% of the time, respectively). The survey suggests that adherence was highest among those younger than age 2 and lowest among those ages 18 to 24.
Staying on top of treatment regimens can prevent devastating consequences. Adherence is associated with fewer bleeding events—overall and in the joints—and a reduction in joint disease. What’s more, it’s linked to fewer sick days, decreased pain and a greater quality of life.
“The goal is to prevent bleeding and the complications that come from it, and it’s hard to do that if patients aren’t infusing consistently,” says Jennifer Maahs, RN, PNP, MSN, a pediatric nurse practitioner at the Indiana Hemophilia and Thrombosis Center in Indianapolis.
Ruth Mulvany, PT, DPT, MS, a physical therapy consultant for the University of Tennessee Health Science Center in Memphis, agrees. “We see a world of difference in people with bleeding disorders who are compliant, compared with those who are not,” she says.
Getting Organized
Many diseases require regular self-care and treatment, but hemophilia can be especially challenging. Patients or parents of patients need to know how to infuse factor, store it properly, discard medical waste and document infusions, while staying in close contact with members of their care teams.
Treatment logs are the foundation to proper care. Patients can scribble notes on a piece of paper or calendar or use apps for smartphones and tablets that record bleeding episodes and treatments. Technology not only makes such tracking easier, but it can also provide calendar reminders and phone alerts.
“Most people are tech-savvy and have a cellphone with smart technology,” Maahs says, “or they may use an iPad for school.”
Lantz relies on an app called MicroHealth. “It reminds me to infuse and once I do, I click a button that says I infused,” he says.
The logs are shared with your HTC and are important for developing the best treatment plan for yourself or your child. Your insurance company may also require them.
Empowering Young Patients
While it can be tempting to manage a child’s care, turning aspects of it over to them is key to nurturing lifelong adherence habits. “At our center, we have a transition program that starts really early,” Maahs says. “Every year there are new goals that help the parents back away and help the child take on more responsibility.”
First kids learn how and when to infuse factor, often learning how to self-infuse at hemophilia camp. “Then the next year, you start teaching the child how to mix factor and so on,” Maahs says.
Patients and families do best when they dedicate a particular place in the house for infusions, Mulvany says. It also helps to create a supply box for children.
“Put syringes on one side and alcohol on another and make it very visual,” Maahs says. “Your child needs to be able to just glance at it to see if it’s time to order supplies. The last thing you want is go to infuse and not have something you need. You can’t just go to a drugstore and ask to buy a syringe.”
Barriers to Adherence
Trouble can emerge when patients reach adolescence. “That’s a period of time that we know it’s going to be a real challenge to keep people on the right track,” Mulvany says. “We hope by then that we’ve built up a good relationship of trust and have done a good job educating them so that compliance will continue.”
Ironically, proper treatment can be a victim of its own success. “Patients have to see the importance of it to them, and it can get complicated when you have somebody who’s doing very well, because they forget what it’s like to have problems,” Maahs says. “Then maybe they slack off on their treatment a bit because the last time they missed a dose, nothing happened. But it’s the consistency that’s important.”
The overarching aim, experts say, is for people with hemophilia—children or adults—to appreciate the seriousness of their disease and the benefits of sticking with their treatment regimen. “You want them to feel that ‘by doing this, I’m helping myself’ versus ‘I have to do it because I have hemophilia,’” Maahs says.
Going to hemophilia camp was the eye-opener for Lantz. “My first time at camp, I thought doing my medication three times a week was a lot,” he says. “But there were kids who were infusing twice a day. I realized there are kids who have it a lot harder than I do. But we’re all in the same boat.”
Fortunately, aside from a few missed infusions in high school, Lantz has managed his condition well. “It’s fortunate that we live in the time that we do and that we can get the care that we need,” he says.
How to Be a Savvy Patient
You or your child relies on medication to avoid bleeds and long-term joint damage. Here’s how to make the most of these prescriptions:
- Get to know your medications. Keep a master list of your medications that includes the name, dosage and reason you are taking them. Read the medications’ package inserts, then file them in a place that you can access later.
- Avoid running out. If your prescriptions are filled locally, call several days in advance of when you need them, in case the pharmacy has to order your medication. Some pharmacies can set up automatic refills and alert you when the medication is available. If you use a mail-order company, set up delivery for at least two weeks in advance to allow for shipping glitches.
- Get familiar with your insurance plan. Check your plan’s formulary—a list of brand-name and generic drugs that are preferred by your insurance company. Make sure the formulary includes your medications. If it doesn’t, you may need prior authorization.
How to Get the Most Out of an HTC Visit
Developing solid communication skills with providers can help them better manage your or your child’s bleeding disorder. Follow these tips to get the most out of appointments with your hemophilia treatment center (HTC) healthcare team.
Before Your Appointment
Know why you are going for a visit to the HTC. It may be for your annual comprehensive visit, for a midyear checkup, for evaluation of a bleed or for a follow-up after a bleeding episode. For an annual visit:
- During the year, keep a running list of questions you have for your healthcare team. Clinic visits can be hectic and you will see a lot of providers. Remember to bring the list with you!
- Track bleeding episodes using an infusion log, treatment calendar or app. This information is key for your healthcare providers so they can assess your treatment plan and make changes if necessary.
- If you or your child has specific issues, write down when the problems occur, or if there is something that precipitates a problem. For example, do you or your child typically have bleeds on one particular day of the week, or following a specific activity?
- Keep track of any school or work days missed due to your bleeding disorder.
- If this is your first HTC clinic visit, make a list of your family members and note the following:
- Who has been diagnosed with a bleeding disorder (such as hemophilia or von Willebrand disease)? How are they related to your child?
- Are there family members with similar bleeding symptoms who have not received a diagnosis? What is their relationship to your child?
Bring with You to the Appointment
- A health history since your last visit. Bring any pictures you have taken documenting bleeding episodes, and don’t forget your list of questions.
- Infusion logs for yourself or your child.
- Names of prescription and nonprescription medications you or your child takes. Include vitamins, herbal remedies and pain medicines.
- Names of medications and foods to which you or your child is allergic.
- Medical records, including X-ray films, test results or past doctor’s records. If you have been seen at another HTC, bring a copy of the records from those past visits.
- A notepad to jot down information.
- If possible, bring a trusted family member or friend with you to the visit. This extra person will help you remember what was said, and he or she may ask questions you didn’t think to ask.