“Your care team should walk you through how to take your medication,” says Craig. These medications can require some complicated techniques, so don’t be afraid to ask questions during your appointments if something is unclear.
How you administer your medication depends on which one your doctor prescribes.
C1-Inhibitors (C1-INH)
Human C1-esterase inhibitor (Berinert) and conestat alfa (Ruconest) increase the body’s levels of C1-INH, a protein that helps control swelling, inflammation, and vascular permeability. These medications are injected intravenously to deliver C1-INH directly into your bloodstream. The process is tricky, so you need detailed instruction before doing it at home.
First, reconstitute the medication by injecting sterile water into the glass vial containing powdered medicine. Gently swirl the vial on a flat surface until it clears, readying the medicine for infusion. With training from your medical team, you will learn how to prime a syringe and locate a vein in your arm or hand. Once you’ve successfully punctured the vein, perform a slow IV push, manually pressing the plunger to deliver the medication at a steady rate.
Kallikrein Inhibitors
Kallikrein inhibitors like ecallantide (Kalbitor) and sebetralstat (Ekterly) work by blocking kallikrein, an enzyme that causes blood vessels to widen and leak, thereby reducing swelling and inflammation during an HAE attack.
Kalbitor is injected subcutaneously into the abdomen, thigh, or upper arm. Using a large bore needle and syringe, withdraw 1 milliliter (mL) of the liquid medication from the medication vial. Then, switch out the large bore needle for an injection needle on the syringe, clean your chosen injection site with an alcohol swab, and inject the dose until the syringe is completely empty. Repeat this procedure until you’ve dosed the amount of the medication your doctor prescribes. You don’t need to rotate injection sites, but you should separate each injection site by at least 2 inches and avoid giving injections in areas where you’re having an attack.
Ekterly is the first and only oral on-demand HAE medication approved for treating acute attacks in people ages 12 and older. It’s available in a 300 milligram (mg) tablet. At the very first sign of an acute attack, take two tablets by mouth (for a total dose of 600 mg). If your symptoms don’t improve, or if they worsen or return, you can take a second 600 mg dose at least three hours after your first dose, up to a maximum dosage of 1,200 mg in any 24-hour period.
Bradykinin B2 Receptor Agonists
Icatibant (Firazyr) is a bradykinin B2 receptor agonist that stops HAE attacks by blocking bradykinin, the peptide that triggers rapid swelling and abdominal pain.
Firazyr is injected subcutaneously into the abdomen. Take the cap off the prefilled syringe and replace it with the needle from the needle cap. Clean your injection site with an alcohol swab, gently pinch the skin to make a fold, and slowly inject the medication until the syringe is completely empty.
Post-Attack Management
On-demand treatments start working right away to prevent your attack from worsening, says Macginnitie. But depending on your medication, it may take 30 minutes to two hours to start noticing an improvement in symptoms like swelling.
What you should do in the meantime depends on your symptoms. You can return to your normal activities if you feel up to it, but rest or an over-the-counter pain reliever like ibuprofen or acetaminophen can be helpful if you’re uncomfortable, says Macginnitie.
If your symptoms don’t start to improve within the expected timeframe, you may need to take a second dose of your medication. Check the prescription information for your medication to determine when you should take a second dose. If the second dose doesn’t seem to help, contact your doctor or seek emergency medical attention for life-threatening symptoms.
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