Coagulopathic/Bleeding Disorder Education?

BadJoke88

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Hello All,

So a year and a half ago now my then four year old son was diagnosed with Severe Type A Hemophilia. Since then I've been sucking down as much knowledge as I can in regards to hemorrhage control and trauma in patients with bleeding disorders. I've come to find, though, that there isn't much knowledge in easily digestible formats. It's all feature length papers and scholarly articles with an emphasis on hospitals and prolonged treatment. All well and good except one needs to break it down in order to grab what is applicable to the EMS provider.

Being BLS, I'm not sure to what depth this is covered for higher level certifications but it certainly hasn't been touched on very often during the bleeding control classes I've attended. My idea was to possibly get with my Department's Medical Director as well as the Bleeding Disorder Clinic my son goes through and begin forming a short introduction to these disorders leading into treatment considerations. Before I do all that, though, has anyone encountered something similar already available? I know this is a small percentage of the population when compared to other conditions, but there are some specifics that I know I would have liked to have known as an EMT prior to encountering this ailment.

Any thoughts or suggestions?
 

mgr22

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I can't speak to hemophilia specifically, but there is a growing awareness of the need for EMS to occasionally treat rare diseases that may not be covered by local protocols. Here's an article that might help you:
 

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Chronic consequences of the disease aside, acute/emergent bleeding in these patients shouldn't necessarily be treated any differently than someone on, say, coumadin, for BLS EMS purposes.

If someone has hemophilia, statistically, they are likely being treated in a network of specialized treatment centers. So where you take the patient that is bleeding is important if you have the choice. If no such network center is in your area, at least going to a center that has a blood bank/pharmacy sophisticated enough to be able to treat these patients is a consideration.
 

Peak

ED/Prehospital Registered Nurse
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Unfortunately there is little that EMS can do for a patient with factor VIII deficiency beyond things that would be done for ordinary hemorrhage control.

With pediatric hemophilia the biggest thing you can do is prevent bleeding in the first place. Of course kids will always be kids, but risk reduction is worth its weight in gold.

In the setting of an acute bleed factor replacement is essential, and something we are never going to see on an ambulance.

I would seek out which ED/Medical centers actually stock factor VIII replacement. Not every department will be able to administer it and time is crucial, even in bleeds that we don't typically think of in EMS (intraocular, deep muscle bleeds, etc) in addition to more obvious things like CNS and belly bleeds.

Depending on what center your kid is being treated at you might be able to have them do some education. We put together binders to be kept with the patient and do education with the local EMS agencies for our more complex kids, typically our heart kids but we do it for other programs as well.
 

DrParasite

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I did my research paper for psychopathology on hemophilia. the biggest takeaway is prevention is key, and the biggest threat is internal bleeding, not external. EMS can control external bleeding (in theory, I know, stay with me on this), and can identify the need to transport the patient to a facility that has the VIII replacement, as well as will know which facilities can do this. it's the bleeding that we can't see, such as joints (knees are particularly bothersome) and abdominal areas that concern me, because we can't see them.

Unfortunately, there isn't enough of a patient population to put clotting factor on the ambulance (and it would be cost prohibitive, nor can ALS do much. Identifying the patient as being a hemophiliac, trying some basic bleeding control methods, and transporting to a facility that can manage the patient appropriately would be the top three things to convey to to EMS crews.
 

NomadicMedic

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Would hemostatic agents, like quick-clot, have any value in a patient with a clotting factor deficiency? Logic would say no... in my mind, in cases of major bleeding a tourniquet would be the only real method to control that hemorrhage.
 

Peak

ED/Prehospital Registered Nurse
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Would hemostatic agents, like quick-clot, have any value in a patient with a clotting factor deficiency? Logic would say no... in my mind, in cases of major bleeding a tourniquet would be the only real method to control that hemorrhage.
Yes, but with less efficacy than normal. If you are running at 20% of normal factor VIII for example you are still only going to have 20% of normal factor activity. Results will vary based on what kind of hemophilia the patient has (or other clotting inhibition) and where the product promotes clotting in the clotting cascade.

We have found pretty good results with quick-clot and topical TXA, although many patients just don't achieve good hemostasis until they have had factor replacement.
 

Peak

ED/Prehospital Registered Nurse
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Just for clarity we should not delay tourniquets, clamps, sutures/ties, et cetera in the setting of life threatening hemorrhage.
 
OP
BadJoke88

BadJoke88

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Chronic consequences of the disease aside, acute/emergent bleeding in these patients shouldn't necessarily be treated any differently than someone on, say, coumadin, for BLS EMS purposes.

If someone has hemophilia, statistically, they are likely being treated in a network of specialized treatment centers. So where you take the patient that is bleeding is important if you have the choice. If no such network center is in your area, at least going to a center that has a blood bank/pharmacy sophisticated enough to be able to treat these patients is a consideration.
I was thinking of hitting on that subject should I continue down this road. I find it ironic that my Mom is on Coumadin for blood clotting issues while my son is currently on Hemlibra (previously Factor VIII was administered 3x a week, more if there was an active bleed) for the reverse. Might be a topic worth exploring with our clinic.
 
OP
BadJoke88

BadJoke88

Forum Ride Along
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Unfortunately there is little that EMS can do for a patient with factor VIII deficiency beyond things that would be done for ordinary hemorrhage control.

With pediatric hemophilia the biggest thing you can do is prevent bleeding in the first place. Of course kids will always be kids, but risk reduction is worth its weight in gold.

In the setting of an acute bleed factor replacement is essential, and something we are never going to see on an ambulance.

I would seek out which ED/Medical centers actually stock factor VIII replacement. Not every department will be able to administer it and time is crucial, even in bleeds that we don't typically think of in EMS (intraocular, deep muscle bleeds, etc) in addition to more obvious things like CNS and belly bleeds.

Depending on what center your kid is being treated at you might be able to have them do some education. We put together binders to be kept with the patient and do education with the local EMS agencies for our more complex kids, typically our heart kids but we do it for other programs as well.
Oh, I know there's precious little an ambulance crew could do in the field. I was thinking of speaking to how each disorder actually affects the individual, and little tid bits such as why Ibuprofen isn't the best choice and the lasting damage left in a joint bleed. Things like that.
 
OP
BadJoke88

BadJoke88

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I did my research paper for psychopathology on hemophilia. the biggest takeaway is prevention is key, and the biggest threat is internal bleeding, not external. EMS can control external bleeding (in theory, I know, stay with me on this), and can identify the need to transport the patient to a facility that has the VIII replacement, as well as will know which facilities can do this. it's the bleeding that we can't see, such as joints (knees are particularly bothersome) and abdominal areas that concern me, because we can't see them.

Unfortunately, there isn't enough of a patient population to put clotting factor on the ambulance (and it would be cost prohibitive, nor can ALS do much. Identifying the patient as being a hemophiliac, trying some basic bleeding control methods, and transporting to a facility that can manage the patient appropriately would be the top three things to convey to to EMS crews.
The internal bleeding was the issue that surprised me the most, but in hind sight should have been obvious. My son's Hemophilia was diagnosed finally after a severe bleed into his right knee. And you are absolutely spot on with the cost. Factor replacement is ridiculously expensive and the Hemlibra my son is on costs about $18k. Accounting for the shelf life of Factor and the chances of using it in the field, among other considerations, don't justify the cost of stocking it.
 

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