Mitigating Hemophilia's Impact on Quality of Life


Many challenges are present in the setting of hemophilia regarding patient quality of life; a panel of experts review just how severe hemophilia’s impact is and how that impact might be mitigated.

Peter L. Salgo, MD: Once the diagnosis is made, or once it’s clear this is a real problem, what is the impact on patients living with this disease? Is there a lot going on?

Robert F. Sidonio Jr, MD: The patients often feel like they’re left out of normal activities in childhood. In the south, it’s playing football with their friends and participating in aggressive contact sports. Those are things that we have to obviously have some restrictions on because I saw a kid yesterday, and he had developed an intracranial hemorrhage from heading the soccer ball. His mom won’t let him go anywhere near a soccer ball these days, even though he’s 19-years-old now. Obviously, we just have to be protective. We don’t know when they’re going to develop those bleeding events. Groups like the National Hemophilia Foundation have listed what are considered safe activities.

Obviously, not being able to participate and do some of these things can lead to anxiety issues and some depression, and we definitely see that in studies of children and adults. You’re living in constant fear of having a bleeding event that could affect your life, and so you can imagine how that would change the trajectory of their lives.

Peter L. Salgo, MD: To that mom who said no heading soccer balls: right on, good idea.

Tim Boonstra, RPh: Sorry to interrupt.

Peter L. Salgo, MD: No, go.

Tim Boonstra, RPh: But it can be as simple as adult patients wanting to mow the lawn.

Robert F. Sidonio Jr, MD: Exactly.

Tim Boonstra, RPh: Or do some simple things with their kids.

Robert F. Sidonio Jr, MD: Driving the car. If you develop joint bleeds in your elbow, you’re not able to drive the car or use a stick shift—just activities of daily living. Yes, you’re right, that’s a good point.

Peter L. Salgo, MD: What are the medications you need to avoid in hemophilia? Let’s get back down to some basic pharmacology here. What other cautions do you give to folks? Who wants to start us off on that?

Tim Boonstra, RPh: Often, when we look at our medical record, we see an allergy to NSAIDs [nonsteroidal anti-inflammatory drugs] and to aspirin. It’s not a true allergy. It’s just something that hemophilia patients shouldn’t take.

Robert F. Sidonio Jr, MD: It’s there for you guys to notice.

Tim Boonstra, RPh: Exactly. It can cause GI [gastrointestinal] bleeding possibly, so we certainly wouldn’t want that in a patient with hemophilia.

Peter L. Salgo, MD: I see allergies on the chart all the time. I’m allergic to morphine. What happens? My breathing slows down and I itch.

Robert F. Sidonio Jr, MD: We put that as an alert, but it’s a good way for people to notice it. You put it in under allergies.

Peter L. Salgo, MD: You put it in the allergy section; they get the wrist band.

Robert F. Sidonio Jr, MD: Yes, exactly.

Peter L. Salgo, MD: Don’t go there.

Robert F. Sidonio Jr, MD: Do not give Advil.

Peter L. Salgo, MD: If you want patients to live as normal a life as possible and you want to get to the goals of therapy, what do pharmacists contribute to this? How do you help?

Lacey Chapman, RPh: Well, instead of NSAIDs, you could recommend Tylenol. Tylenol is fine in a hemophilia patient.

Peter L. Salgo, MD: My liver transplant people would disagree.

Robert F. Sidonio Jr, MD: In moderation, right.

Lacey Chapman, RPh: Or, just ice and rest for an injury, resting the joint. We have ice packs that we give patients that aren’t super cold, ones that you can put right on the joint. They might not need an NSAID. Wearing the medical bracelet for hemophilia might be a good idea because then doctors will know not to give them aspirin and NSAIDs; or, in case they get in an emergency or an injury, people know that they have hemophilia.

Tim Boonstra, RPh: But it’s important to know, too, as our hemophilia population is aging, that there are going to be patients who are going to be developing cardiac disease. Aspirin might be part of their treatment. That’s when you want to consult your hematologist.

Robert F. Sidonio Jr, MD: And your cardiologist.

Peter L. Salgo, MD: But that’s a real Hobson’s choice, right?

Robert F. Sidonio Jr, MD: Yes.

Tim Boonstra, RPh: Yes.

Peter L. Salgo, MD: I like that Hobson. Here we have a life-saving medication—life-saving in terms of aspirin and heart disease—and yet typically, it’s possibly life-threatening medication at the same time. How do you balance that out? What would you say?

Lacey Chapman, RPh: Well, I don’t have any patients who are on aspirin.

Peter L. Salgo, MD: How old is your oldest patient?

Lacey Chapman, RPh: We do have some patients in their 50s, but that brings up the HIV concern from the 80s. A lot of our older patients have HIV or hepatitis, so we don’t really see any patients well into their 70s or anything like that. We will in the future, hopefully.

Robert F. Sidonio Jr, MD: Yes, we will now.

Lacey Chapman, RPh: But I don’t have any patients who are really that old. I do have some people who are on Celebrex [celecoxib]. Some of the NSAIDs that are newer are less targeted towards clotting.

Tim Boonstra, RPh: We just recently had a patient who had 3 vessel stents placed, and so he is on antiplatelet therapy.

Peter L. Salgo, MD: He needs antiplatelet drugs.

Tim Boonstra, RPh: Exactly.

Peter L. Salgo, MD: This is a non-trivial issue.

Robert F. Sidonio Jr, MD: Yes.

Tim Boonstra, RPh: So, he was a challenging patient to get stabilized.

Peter L. Salgo, MD: What about physical therapy here? My view of physical therapy is that it may provoke joint use and may in fact provoke some bleeding, no?

Robert F. Sidonio Jr, MD: It depends on how aggressive the physical therapy is, and they have some modifications in what they do. Thankfully we have most of them in our treatment centers, so we can advise the outpatient physical therapist. But particularly for the ones who are on prophylaxis, we shift their prophylactic dose to physical therapy sessions. More and more as you go to these meetings, you see that it’s important to increase the muscle mass and make sure that the joints are stable because you’re protecting those joints with good exercise. We talked about how they’re already at risk for osteoporosis and decreased bone mineral density. And so, it’s important that we increase their physical activity. But they choose activities like swimming and should be a little bit careful about jogging and other exercises that are potentially harmful to the joints. But we know that there’s a good balance there, obviously trying to stay in shape and working out. We have a lot of our young guys who want to bench press as much as they can, and we always say, “Well, if you can do it in 20 reps, that’s an appropriate amount of weight to do. To stay healthy, you don’t need to look like Arnold Schwarzenegger.”

Peter L. Salgo, MD: If I’m a dentist and I hear somebody is coming to my practice with hemophilia, I might run for the hills. And yet, they need dental care.

Lacey Chapman, RPh: Some of them do run for the hills.

Tim Boonstra, RPh: They do.

Robert F. Sidonio Jr, MD: Yes.

Peter L. Salgo, MD: How do you manage that? What do you do?

Lacey Chapman, RPh: A lot of times the HTC, the Hemophilia Treatment Center, will have a dentist associated with it that is familiar with hemophilia patients. But some hemophilia patients do struggle to find a dentist who will treat them because like you said, they don’t want to deal with it. It’s too complicated.

Tim Boonstra, RPh: We’re fortunate to work at the University of Minnesota, where there is a dental school. A lot of our patients do go there, so they are familiar with working with hemophilia patients. But there can be some proactive work done, such that they may have Amicar [aminocaproic acid] available, which we’ll talk about shortly. That can prevent some of the bleeding issues.

Robert F. Sidonio Jr, MD: The key thing for us is the communication. We have our own dental hygienist at our hospital who does hundreds of dental procedures a year. We always feel like that person may move on, and now they have that expertise to go out and teach their colleagues. But just like anything else, we come up with very strict bleeding plans of what to do and what not to do with those patients. They don’t let the patient say, “Oh, don’t worry about it, I’ll probably be fine.” They get a very direct plan of what has to happen before you want to do that procedure.

Peter L. Salgo, MD: I have heard through the grapevine, the medical grapevine, that people with hemophilia often have very poor dental situations, if you will. I don’t know what else to say. Their teeth aren’t great. Is that part of the disease or part of the lack of treatment?

Robert F. Sidonio Jr, MD: I think it’s a part of the lack of access to care. In the United States, dental access is a big problem, and then you take something that could potentially lead to significant bleeding and it gets worse. We actually conducted a study at our center, and we looked at simple things. How often do you brush your teeth a day? We were alarmed at how often people brush their teeth. Actually, the majority of people brush their teeth once a day, I kept thinking, Do you brush them in the morning or the afternoon? I don’t even know which one is better. But that was pretty alarming to us, and the only predictor of poor dental health was whether the parents had poor dental health as well. There’s probably some bacteria issue as well, but it comes down to simple things. We actually started handing toothbrushes out at our clinic and saying, brush your teeth twice a day. Just simple message like that. Sometimes it’s very simple.

Peter L. Salgo, MD: I’m afraid because I’m going to bleed from my gums. Do you get that?

Robert F. Sidonio Jr, MD: Yes. Well, they’d say that, but then they don’t perform that good dental care, and they get horrible gingivitis and they’re oozing. Then they have to go and have 6 teeth removed at one time, which is a costly and potentially morbid procedure.

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