Patient Counseling
We recommend all patients, in case of acute attack, if they cannot self-infuse at home, to go promptly to a first aid or activate the emergency medical service preventing the attack from reaching its maximum swelling.
You should be accompanied by a relative as anyone who is aware of your condition as a rare disease patient so that he/she can assist you and communicate with caregivers in case you were not able to be fully self-sufficient.
Invite health care professionals to visit this site and especially this page.
We recommend all patients to take with them the concentrated plasma C1 Berinert-P or the Firazir (Icatibant / acetate) depending on the instructions received from their doctors.
If you need any help invite the health staff to contact the emergency number.
Information for health professionals in an emergency
Angioedema deficiency of C1-inhibitor (C1-INH) is a disease caused by the deficiency in the serum of affected, the inhibitor of the first portion of the complement system. It can be caused by a genetic defect (hereditary angioedema) or by an increased consumption (acquired angioedema).
IT HAPPENS WITH THE DISEASE:
Angioedema for subcutaneous edema
Abdominal colic type for gastrointestinal mucosal edema (acute abdomen)
Dyspnea with signs of asphyxia due to laryngeal edema. Particularly frightening is the glottis edema that can lead to death by asphyxiation. Symptoms last 1-3 days – is not allergic disease
For laryngeal edema and severe abdominal colic administer:
The patient usually has availability of one or more packages of the life-saving medicine in the type and dosage indicated to him by the medical practitioner who follows him in his course of treatment. If you have further questions, please visit the association or the doctor available in an emergency.
the protein deficient 1000-2000 through customized units of C-1 Berinert-P, produced by CSL Behring GmbH, or the synthetic product Firazyr® (Icatibant acetate), an inhibitor of bradykinin; administered with pre-filled syringe (30 mg) by subcutaneous injection in the abdominal area; The drug is marketed in Italy by: Shire Italian spa.
The patient usually is in possession of drugs.
In the absence of these drugs, use fresh frozen plasma.
Cortisone ANTIHISTAMINES AND ARE INEFFECTIVE
In case of attack of the face, oral cavity and pharynx, neck or severe abdominal attack, it is recommended that the staff of the emergency room triage officer to be assigned to the patient:
YELLOW CODE
If there is even one sign of laryngeal involvement (dysphonia, stridor, retraction of the jugular, dyspnea, desaturation) assign:
RED CODE
(Cicardi et al. Intern Emerg Med 2014; 9: 85-92.)
The patient usually has availability of one or more packages of the life-saving medicine in the type and dosage indicated to him by the medical practitioner who follows him in his course of treatment. If you have further questions, please visit the association or the doctor available in an emergency.