What to Do When Initial On-Demand Treatment for Hereditary C1 Inhibitor Deficiency Has Not Provided Relief
This protocol covers the next clinical step when a patient with hereditary C1 inhibitor deficiency has received first-line on-demand therapy during an acute attack but did not achieve the expected onset of symptom relief within the required timeframe.
Previous Treatment — Failure Condition
Initial on-demand therapy — administered as early as possible at attack onset using agents such as ecallantide, icatibant, plasma-derived C1 inhibitor (Berinert), or recombinant human C1 inhibitor (Ruconest) — was given. The treatment goal of onset of relief from angioedema swelling within 30 to 120 minutes was not achieved, triggering escalation to this next protocol step.
This Protocol — Partial Overview
The next step involves repeat dosing of specific on-demand agents during the same attack, within defined limits. The protocol also addresses management of respiratory distress that is not improving. The complete agent selection, dosing structure, and escalation pathway are in the full protocol.
References
- Repeat treatment during a single attack has been approved for icatibant (maximum of 3 doses within a 24-hour period at intervals of at least 6 hours) as well as ecallantide and rhC1INH (both with maximum of 2 doses within a 24-hour period).
- Elective intubation should be considered for any patient with signs of respiratory distress who is not improving after treatment.
DOI: 10.1016/j.jaip.2020.08.046
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