43
5.5 Special Considerations With
the Use of Berinert
®
Efficacy
5.51 Self-administration With Berinert
®
A label expansion for self-administration of Berinert
®
has
been approved by European health authorities and by the
Food and Drug Administration in 2011. Self-administration
of Berinert
®
at home can be a suitable alternative to
enable treatment of acute attacks at an earlier stage,
thereby reducing their severity and improving the patient’s
quality of life. As summarized earlier (see chapter 3.43),
international consensus guidelines recommend that self-
infusion programs with C1-INH should be offered to
patients.
68
Eventually, the treating physician will carry the
decision on the use of self-administration with Berinert
®
for
an individual patient. It is his responsibility to ensure that
appropriate training is provided and the use is reviewed
at regular intervals (see chapter 9.2
Summary of Product
Characteristics
).
In a partly retrospective, partly prospective study in
22 patients with severe HAE, intraindividual comparisons of
efficacy, safety, and quality of life with self-administration
of 500 to 1,000 IU Berinert
®
vs prior prophylactic danazol
treatment were made. A significant decrease of median
number of attacks per year and a significant improvement
in quality of life were reported after patients had switched
to self-administration with Berinert
®
.
89
A retrospective observational study in 20 pediatric patients
revealed a significant reduction of mean number of days
hospitalized after patients had switched from physician-
based therapy to self-administration with 500 or 1,000 IU
Berinert
®
(3.8 vs 0.11 days/year). Also, median time to
initial symptom relief for all types of attacks was reduced
from 60 to 40 minutes during self-administration. Self-
administration with Berinert
®
had an excellent safety profile
with no obvious disadvantages compared to physician-
based therapy.
90
5.52 Pre-procedure Prevention With Berinert
®
In 2013, European health authorities approved an extended
use of Berinert
®
for pre-procedure prevention (short-term
prophylaxis) of acute angioedema attacks. Pre-procedure
prevention with Berinert
®
can help avoid acute attacks of
HAE in patients undergoing medical, dental or surgical
procedures.
The risk of potentially fatal peri-procedural HAE attacks has
been described in numerous case reports and studies.
91-94
Of
particular concern, dental manipulations have been linked
with tongue and laryngeal swelling, which in turnmay result
in potentially life-threatening upper airway obstruction.
As summarized in chapter 3.42, recent international
consensus guidelines recommend short-term prophylaxis
(pre-procedure prevention) with C1-INH concentrate (10
to 20 IU/kg body weight or 1,000 IU) 1 to 6 hours before
major procedures or intubation.
11,71
The preventive effect of pre-procedure HAE prophylaxis
with Berinert
®
has been demonstrated in several case series
studies
95-97
and retrospective survey studies.
98,99
In a retrospective cohort study in 171 patients with HAE, the
rate of facial swelling and/or laryngeal edema was higher
for 577 tooth extractions without prophylaxis (21.5%) than
for 128 tooth extractions with prophylaxis (12.5%).
45
The
study examined the effect of no prophylaxis vs prophylaxis
with 500 or 1,000 IU Berinert
®
on the development of peri-
procedural edema, and revealed a graded dose-response
relationship with the difference between 1,000 IU Berinert
®
and no prophylaxis being statistically significant (p<0.05).
