There are 5 U.S. Meals and Drug Administration (FDA)–permitted on-demand medicines for HAE. All are meant to be self-administered (or administered by a father or mother or caregiver) so you may get the remedy into your system as rapidly as wanted.
“Your care group ought to stroll you thru tips on how to take your remedy,” says Craig. These medicines can require some sophisticated strategies, so don’t be afraid to ask questions throughout your appointments if one thing is unclear.
The way you administer your remedy will depend on which one your physician prescribes.
C1-Inhibitors (C1-INH)
Human C1-esterase inhibitor (Berinert) and conestat alfa (Ruconest) improve the physique’s ranges of C1-INH, a protein that helps management swelling, irritation, and vascular permeability.
These medicines are injected intravenously to ship C1-INH straight into your bloodstream. The method is difficult, so that you want detailed instruction earlier than doing it at dwelling.
First, reconstitute the remedy by injecting sterile water into the glass vial containing powdered medication. Gently swirl the vial on a flat floor till it clears, readying the drugs for infusion. With coaching out of your medical group, you’ll discover ways to prime a syringe and find a vein in your arm or hand. As soon as you’ve got efficiently punctured the vein, carry out a sluggish IV push, manually urgent the plunger to ship the remedy at a gentle charge.
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 decreasing swelling and irritation throughout an HAE assault.
Kalbitor is injected subcutaneously into the stomach, thigh, or higher arm. Utilizing a big bore needle and syringe, withdraw 1 milliliter (mL) of the liquid remedy from the remedy vial. Then, swap out the big bore needle for an injection needle on the syringe, clear your chosen injection web site with an alcohol swab, and inject the dose till the syringe is totally empty. Repeat this process till you’ve dosed the quantity of the remedy your physician prescribes. You don’t have to rotate injection websites, however you need to separate every injection web site by a minimum of 2 inches and keep away from giving injections in areas the place you’re having an assault.
Ekterly is the primary and solely oral on-demand HAE remedy permitted for treating acute assaults in folks ages 12 and older. It’s out there in a 300 milligram (mg) pill. On the very first signal of an acute assault, take two tablets by mouth (for a complete dose of 600 mg). In case your signs don’t enhance, or in the event that they worsen or return, you possibly can take a second 600 mg dose a minimum of three hours after your first dose, as much as a most dosage of 1,200 mg in any 24-hour interval.
Bradykinin B2 Receptor Agonists
Icatibant (Firazyr) is a bradykinin B2 receptor agonist that stops HAE assaults by blocking bradykinin, the peptide that triggers speedy swelling and stomach ache.
Firazyr is injected subcutaneously into the stomach. Take the cap off the prefilled syringe and exchange it with the needle from the needle cap. Clear your injection web site with an alcohol swab, gently pinch the pores and skin to make a fold, and slowly inject the remedy till the syringe is totally empty.
Put up-Assault Administration
On-demand remedies begin working immediately to stop your assault from worsening, says Macginnitie. However relying in your remedy, it might take half-hour to 2 hours to begin noticing an enchancment in signs like swelling.
What you need to do within the meantime will depend on your signs. You possibly can return to your regular actions in the event you really feel as much as it, however relaxation or an over-the-counter ache reliever like ibuprofen or acetaminophen might be useful in the event you’re uncomfortable, says Macginnitie.
In case your signs don’t begin to enhance inside the anticipated timeframe, you might have to take a second dose of your remedy.
Test the prescription info on your remedy to find out when you need to take a second dose. If the second dose doesn’t appear to assist, contact your physician or search emergency medical consideration for life-threatening signs.
