Practice Pearl 3: Complications and Pain Management With Hemophilia


A review of the complications of self-administration and pain management.


  • Practice Pearl 3: Procedure for Transitioning and Patient Education
  • Practice Pearl 3: Reviewing the Dispensing Process

Luigi Brunetti, PharmD, MPH: In terms of complications, specifically with outpatient care, I know there is a push, if we can, we'd like patients to receive their treatments at home, right? But what are some of the complications that you can encounter when that occurs in that transition period?

Giles Slocum, PharmD: So taking into consideration transitioning into a skilled care facility or rehabilitation, is that the aim of this?

Luigi Brunetti, PharmD, MPH: We can start with that, or even just at home.

Giles Slocum, PharmD: Sure, fair. I wanted to touch on the skilled care and rehabilitation because that's an area where we run into issues at our institution. They either don't carry factor VIII or don't have the comfort level to provide it. That's something that can delay discharge, or having to keep these patients coming to bigger academic medical centers where we do have skilled care units that are attached to a pharmacy that can dispense it.

So that's one complication that we can see where we may need to get creative with ways to get them home. And then the other complication we touched on is acquiring that factor VIII to get home so that we don't ahve that bounce back to the ED [emergency department] just for a dose of factor VIII, or those types of complications.

Robert F. Sidonio, Jr, MD: Yes, it's really trying to make that transition. It's like any other chronic disease. The last thing the family wants to do is return back to the emergency department after they've been in the hospital for a week. Nobody has that goal. And so really, it is about having good follow-up, make sure they know. And we have our nurses often call. We have an email setup so we can see pictures of wounds and different things, because we want to track this. If somebody calls and says, "I got this and it looks a little puffy," it's really hard to manage that over the phone, and I don't really want them to go to the emergency department every single time.

We often have them send pictures and I think that helps. But really make sure that the HTC [hemophilia treatment center], or whoever is taking care of them, has constant communication as soon as they get home. Because oftentimes they say, "Well, that factor VIII never got delivered and it's been 2 days." And you think, "Why didn't anybody call me?" And so it's really making sure all those things happen: the prior authorizations, the nursing care, especially in pharmacy. If it's over a holiday weekend it can be quite challenging to deliver factor VIII. And so make sure all of that is coordinated well, because the last thing you want to do is to have it returned to the clinic, and make sure we write enough factor VIII to cover events in which maybe they have a little bit more bleeding. They may need a few doses because, again, this always happens on a late afternoon before the weekend and so it can be challenging.

Giles Slocum, PharmD: And then it might be a little bit out of the scope, but taking into consideration pain management, this is also something we see with our patient population. I know it's not directly with the factor VIII component, but with the disease state as a whole. Unfortunately, we may get potential bounce-backs for pain management and things like that. It is something also to keep under consideration when you're planning that discharge.

Robert F. Sidonio, Jr, MD: Helping with all those issues outside of bleeding, we sometimes get so focused on just bleeding and we forget that they might need FMLA [the Family and Medical Leave Act provisions]. They might need someone to call their work and say, "Hey, they really need to be home for a week," and write letters for them to help get back into the situation. And they may need physical therapy. That may or may not be close by. And they may have issues with transportation, which could be challenging. You can write a script for a pain medicine, but they may not be able to go get it. And so all these things really take a lot of good coordinated care, and thankfully we have great nurses and social workers and patient managers who do the lion's share of the work.

Luigi Brunetti, PharmD, MPH: Speaking of pain management, you made me think of something in terms of the transition, inpatient more so. We're in flu season now in New Jersey, and there's an automatic fire for the flu shot. Everyone gets the flu shot. The importance of understanding which routes of administration are appropriate and not appropriate in the inpatient setting is perhaps something that we need to inform the patient on and make sure that the caregivers in the inpatient setting contact the HTC for guidance if they're not familiar with that.

Robert F. Sidonio, Jr, MD: Vaccines are a challenge because of the route. If they’re giving a dose of prophylaxis, then giving an intramuscular injection is going to be fine. We just had a patient who was discharged from the hospital on Hemlibra, he had his line removed, and of course everyone was asking, “Can he get the flu vaccine?” I said, “Oh, you definitely should get that.”

So we haven’t seen any issues if they’re on Hemlibra about getting an intramuscular injection. Certainly, there’s a risk, but I think the risk is pretty minimal. And when you’re talking about flu prevention, that’s a big deal for a patient who already has a chronic disorder. And so, it becomes more challenging if they’re not giving factor VIII prophylaxis and whether it’s as good in the subcutaneous route, and that’s where it’s very challenging for children.

Luigi Brunetti, PharmD, MPH: Sure. And there’s just one last item in this segment of our discussion. Regarding that transition from getting the infusion or getting the treatment in a facility versus being in the outpatient setting, in your HTC, how many sessions do you provide at the HTC before you feel comfortable for the patient to self-administer at home?

Robert F. Sidonio, Jr, MD: On the label they don’t want children younger than 6 or 7 to administer it by themselves. And that makes sense. I think over time with practice, these kids are pretty good at it. We have some 6-year-olds starting IVs [intravenous drips] and giving infusions. So certainly, they can do a subcutaneous injection.

But oftentimes what we do is we write the prescription. It may take a little while to get approval. And then as soon as it gets delivered to their house, we tell them to come back to the clinic, and we want to give the first injection. We make them watch us. We have some practice pads for them to work on. And then we bring them back for the subsequent week. Because you mentioned before, there’s a loading phase in which you get 3 mg/kg per dose for every 7 days for 4 weeks.

And so oftentimes we bring them in, we give the first 1. We make them watch the second and the third. To be honest, after seeing them watching 1, seeing them do 1 and maybe another, they feel pretty good. And if they can’t come in, we often will send a nurse out to watch them give it to make sure they’re administering it correctly. People pick up on this very quickly. It’s like insulin injections. They don’t watch many; you don’t get to watch a bunch of them before it’s done, but you pick it up pretty quickly.

And so as long as they’re engaged early on and they’re paying attention, knowing that they’re going to have to do this. And we often tell the children, “If you want to do this, you could do this yourself.” And it makes them feel a little bit more in control. They don’t feel as anxious about giving the injections. There’s a little bit of leeway on teaching that as well. But bringing them in, it’s an opportunity to educate them about bleeding events, to not ignore bleeding events.

Because a big concern that we’ve had recently is that people are doing relatively well, they don’t call us about some of the bleeding events that they did before, and then that could lead to pretty catastrophic bleeding events. Even though they’re rare, we want them to still call us and field these questions with us. We do that at our center, where we come in and teach them, and sometimes our pharmacist will be involved but mostly it’s the nurse doing it at our center. I don’t know if they do that similarly at your institution, where they’re teaching them in clinic.

Giles Slocum, PharmD: I believe so. I think that’s how we do it. We have a great pharmacy technician who is skilled, not just the prior authorization but with the demo kits and things like that, just to give a plug to her and the great job she does.

Robert F. Sidonio, Jr, MD: Yes, that’s really important. These patients pick it up really quickly. They’re pretty good at this. You have to remember a lot of them have been doing IV infusions for years. So subcutaneous injections are nothing to them.

Luigi Brunetti, PharmD, MPH: Correct. Going from IV to subcutaneous injections, I don’t want to say is simple, but it’s an easier transition than going to an IV infusion.

Robert F. Sidonio, Jr, MD: You can imagine a mother starting an IV in a 5-year-old.

Luigi Brunetti, PharmD, MPH: Correct.

Robert F. Sidonio, Jr, MD: Or accessing a port-a-cath under sterile conditions, they can definitely do a subcutaneous injection.

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