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HWN Suggests

Hemophilia: For the Love of Bleeding

If you, or the patient or the parents suspect a bleed, immediately infuse factor replacement regardless if they took their home prophylaxis dose or not. You treat based on suspicion of a possible bleed, NOT the confirmation of one...

Regardless of their severity level, or if they took their home prophylaxis, in the ED the full dose repletion for:

Factor 8: 50 U/kg. Each U/kg = an increase of factor by 2%.

Factor 9: 100 U/kg .Each U/kg = an increase of factor by 1%.

If you want to give half dose repletion, for the minor bleeds, just give half that dose. Remember, it is always better to overtreat than undertreat.

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Featured

 Managing Hemophilia in the ED: All Bleeding Stops Eventually

In hemophilia A, every 1 U/kg of factor VIII replacement increases the factor activity by 2%.2 For example, 25U/kg of factor VIII replacement will lead to a 50% correction and should be used in cases of mild to moderate bleeding. For severe bleeding in a patient with hemophilia A, give 50U/kg of factor replacement to achieve 100% correction.

 Therapeutics: Hemophilia Management in the ED

Patients are generally knowledgeable about their disease and emergency physicians should feel comfortable starting treatment based on patient-reported symptoms and bleeding prior to initiating a diagnostic workup. Early consultation with the patient’s treating hematologist is essential, but should not delay treatment with factor replacement in severe or life-threatening hemorrhage

Articles of Interest

Bleeding and Hemophilia in the Pediatric ED

Having a patient with hemophilia who is bleeding can be a frightening thing, but just remember a couple of basics: 1.Use the factor that the patient or family has. It will save time and may be the only factor that works for the patient with inhibitors. Due to cost, use the closest vial size to the recommended dose and use the whole vial. Do not waste the factor. 2.Ask the parents questions. They usually know A LOT about their child’s illness and can give you a good amount of information. 3.Remember to aim for 100% correction for severe bleeds. That means 50 U/kg of factor VIII and 100 U/kg of factor IX.

Emergency treatment of bleeding in patients with hemophilia

Major bleeds: major trauma, the airway, CNS, GI hemorrhage, bleeding in the chest, the retroperitoneum, epistaxis, and the eyes or orbits. Raise factor to 100%. Minor bleeds: muscles, joints, oral mucosa, and hematuria.. Raise factor to 50%.

Hemophilia: What’s so Bloody Funny?

1st ask the patient what kind of factor they are on and if they have their factor with them (Always try and use what the patient is on as a first choice)

Management of Hemophilia Patients in the Emergency Department

Factor replacement is the first intervention and it should be started empirically, prior to any further diagnostic testing.

Peds - Hemophilia in the ED

I think that we would all agree that “bleeding” as the chief complaint catches your attention in the Peds ED (or any ED for that matter).