Self-infusion of Berinert for HAE Seen in Study to Be Safe, Effective, Reduces ER Visits

Ana Pena, PhD avatar

by Ana Pena, PhD |

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HAE with normal C1 activity

Self-administration of Berinert was found to be safe and effective and reduces the need for emergency room visits during acute attacks in patients with hereditary angioedema type 1, according to researchers.

Their study, “Safety, effectiveness, and impact on quality of life of self-administration with plasma-derived nanofiltered C1 inhibitor (Berinert) in patients with hereditary angioedema: the SABHA study,” was published in the Orphanet Journal of Rare Diseases.

Patients with types 1 and 2 hereditary angioedema have a deficiency in the protein C1 esterase inhibitor and experience recurrent attacks that cause swelling of multiple body sites. These attacks can last for days and can be life-threatening.

Berinert is one of the most-used medications to treat angioedema attacks, but it has an important practical limitation, which is the need for intravenous administration.

Taking the medicine usually requires admission to a hospital or clinic, which can delay time to treatment and can worsen the outcome of patients.

As a result, home-based and self-treatment with Berinert is recommended to help accelerate the start of treatment, preventing further progression and severity of the attack.

Berinert, manufactured by CSL Behring and first licensed in Germany in 1979, has been approved since 2011 for self-infusion, which is feasible after training by healthcare providers.

The purpose of the study was to address the safety, tolerability, and effects on patients’ quality of life of Berinert self-infusion in a “real-world” setting. 

The SABHA observational study used data from patients referred to the Luigi Sacco University Hospital in Milan, Italy.

Twenty patients with hereditary angioedema type 1 were enrolled. All patients attended two sessions of training at the hospital, consisting of theoretical and practical lessons teaching them and their caregivers how to do their own infusion.

During a follow-up period of one year, attacks were recorded and patients were asked to fill out surveys evaluating the attacks, their satisfaction with the treatment, and their quality of life.

In total, 189 attacks were recorded with an annual median rate of four attacks per patient. Patients waited a median of two hours before self-administering Berinert, and the attacks stopped after a median of six hours.

Most attacks were in the abdomen (39%) and in peripheric body sites (22%). The majority (81%) resulted in mild to moderate symptoms.

Patients preferred to use their arm (88%) to infuse Berinert, followed by the hand (11%) and the forearm (1%). 

Importantly, nearly all attacks (92%) were treated by the patient or their caregiver, avoiding trips to the hospital ER.

Only 8% of the attacks required an emergency room visit and assistance from a healthcare professional. The figures contrasted greatly with the time before enrollment in the study, when patients always went to the hospital to receive their infusions.

Self-infusion was safe with no serious adverse events reported. Mild to moderate symptoms occurred in one-third of the infusions, including swelling, reddening, burning, and pain. Also, the number of attacks with side effects decreased over time, from 37% to 13%.

Of note, patients’ satisfaction with the treatment increased over time, becoming significant three to six months after the study started. Although quality of life did not change significantly, it also showed a trend toward improvement throughout the study period.

“Self-administration of [Berinert] is safe and effective, increases patients’ confidence in the treatment and shows a trend in improving the quality of life,” researchers wrote in the study.