Facilitating home-based treatment of hereditary angioedema Jonathan A. Bernstein, M.D.,1 Marc Riedl, M.D.,2 Lisa Zacek, R.N.,3 and Ralph S. Shapiro, M.D.3 ABSTRACT Hereditary angioedema (HAE) is a rare disorder causing periodic attacks of nonpruritic swelling, for which highly effective subcutaneous and intravenous therapies are available. The need to seek ongoing medical attention for HAE attacks at clinics and hospitals adds to the already considerable burden of the disease. Recent international consensus treatment guidelines have emphasized home-based therapy as a preferred managed strategy whenever possible. Here, we review various strategies for facilitating home-based treatment with injectable HAE medications (plasma-derived C1 esterase inhibitor [C1-INH], ecallantide, icatibant, and recombinant C1-INH). Medical literature relating to home-based treatment of HAE is reviewed and strategies for implementing home-based therapy are presented. Home-based treatment of HAE has been shown to reduce the time to initiation of treatment, reduce the duration and severity of attacks, and improve patients’ quality of life. Several options are available to facilitate home treatment of HAE. Medical staff in a primary care setting can be educated in the care of HAE patients and can teach the technique of parenteral drug administration. Home care agencies and specialty pharmacies are present in most communities and specialize in patient education. Infusion centers are skilled at working with patients with chronic diseases who perform extensive self-care. HAE comprehensive care clinics provide expert diagnosis and disease management and may become the patient’s primary source of HAE care. Home-based therapy of HAE has been shown to be safe and clinically advantageous. Various strategies are available for equipping HAE patients to administer their treatments outside of a medical facility. (Allergy Asthma Proc 36:92–99, 2015; doi: 10.2500/aap.2015.36.3820)



ereditary angioedema (HAE) is a rare autosomal dominant disorder affecting approximately 1/50,000 persons. HAE causes periodic attacks of nonpruritic, nonpitting edema and swelling, usually of the skin or gastrointestinal tract but potentially affecting the airway and other organ systems.1 Attacks of HAE typically begin in the first or second decade of life, with a mean age of onset of 11 years, and persist throughout life.2 The two most common types of HAE are associated with a deficiency in functional C1 esterase inhibitor (C1-INH) activity (C1-INH-HAE). Type I results from mutations that cause a reduced quantity of functional C1-INH to be secreted, whereas type II results from mutations that cause C1-INH to be secreted in normal amounts but in a nonfunctional form.1 These two types are clinically indistinguishable.1 Over recent years, a third and more uncommon type of HAE has




University of Cincinnati Medical Center, Cincinnati, Ohio, 2University of California, San Diego, La Jolla, California, and 3Midwest Immunology Clinic, Plymouth, Minnesota J. A. Bernstein is a consultant, speaker, and has received grant and research support from CSL Behring, Dyax, Shire, Pharming, and ViroPharma. M. Riedl is a consultant for and/or has received grant funds from CSL Behring, Dyax, Shire, ViroPharma, Pharming, Biocryst, and ISIS and is a paid speaker for CSL Behring, Dyax, and Shire. L. Zacek is a paid speaker for CSL Behring and Shire. R. S. Shapiro is a consultant for CSL Behring, Baxter, Shire, and ViroPharma and a paid speaker for Shire This work was funded by CSL Behring Address correspondence to Jonathan A. Bernstein, M.D., University of Cincinnati College of Medicine, 3255 Eden Avenue, ML number 563, Suite 350, Cincinnati, OH 45267-0563 E-mail address: [email protected] Published online December 30, 2014 Copyright © 2015, OceanSide Publications, Inc., U.S.A.




been identified, characterized by normal C1-INH function; in some patients this variant is associated with mutations in the coagulation factor XII gene but has an unknown cause in the majority of cases.3,4 Hereafter, all mention of “HAE” is specific to C1-INH-HAE unless otherwise stated. The frequency of HAE attacks is highly variable, ranging from one or fewer per year to one or more per week.5 Attacks affecting the gastrointestinal tract or airway may require hospitalization; attacks affecting the upper airway typically progress over several hours but may progress quickly and lead to fatal asphyxiation.6,7 Most HAE attacks occur in the skin, usually of the extremities but also of the face, trunk, or genitals. However, many other organs may be affected.2,5 Attacks at any site may be acutely painful, but abdominal attacks, in particular, are often associated with severe pain, sometimes accompanied by nausea, vomiting, and diarrhea.2,8 Although laryngeal swelling is rare, occurring in less than 1% of HAE attacks, as many as 50% of patients with HAE report having experienced at least one such attack.2 Laryngeal attacks were a frequent cause of death among HAE patients before the availability of effective treatment and remain a major cause of mortality in undiagnosed patients.7,9 Although most attacks occur spontaneously, potential triggers include trauma; medical, surgical, or dental procedures; psychological stress; infection; hormonal changes; or intake of estrogen-containing drugs or angiotensin-converting enzyme inhibitors.10 –12 HAE can cause substantial physical and emotional distress, lim-

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itations on professional or educational achievement, financial hardship, and poor quality of life.5,13,14 Patients live with the fear of intolerable pain, airway closure, and passing the disease to their children.15 Over recent years, a number of effective treatments have been developed specifically for the treatment of HAE, including plasma-derived C1-INH (pdC1-INH), ecallantide, icatibant, and recombinant human C1-INH (rhC1-INH). All of these medications are administered by intravenous (i.v.) infusion or subcutaneous injection, but the option of administration outside of a healthcare facility exists for each of them (Table 1). Current clinical guidelines unanimously recommend that patients be trained in home administration and have a supply of medication with them at all times.16 –21 A summary of guidelines from the HAE International Home Therapy Consensus report is presented in Table 2. A recent HAE patient survey conducted in Spain, Germany, and Denmark found that almost all patients (91%) were equipped with medication at home to treat attacks and 66% of respondents had self-treated their most recent attack.22 The rate of home treatment appears to be much lower in the United States. A survey of 172 HAE-treating physicians in the United States conducted from October 2009 to February 2010 indicated that approximately 12% of HAE attacks were being treated in a home setting. A follow-up to this survey conducted in 2013 found that the proportion of HAE patients treated at home had risen but only to 33%.23 Although it is recognized that home administration of i.v. or subcutaneous medications may not be appropriate for every patient, it seems likely that many patients would benefit from the implementation of such treatment plans. A recent survey and expert panel found that the primary perceived barrier to home treatment of HAE is the difficulty of administering the drugs. However, patients or guardians of all ages and a wide range of educational backgrounds have been successfully taught to self-administer HAE drugs and usually retain these skills over time even if they do not use them with great frequency.24 One potential barrier to wider use of home treatment of HAE may be lack of familiarity among HAE-treating physicians with the logistics of training and following patients who are self-treating with injectable medications. It is beyond the scope, and is not the intent, of this article to compare the risks and benefits of any particular HAE treatments or provide comparative clinical information to assist in making treatment decisions. This article is intended for healthcare providers who desire to facilitate home treatment of HAE with the new injectable medications and are looking for guidance and training options to help accomplish this goal. The term “home treatment,” as used throughout this article, refers to the administration of pdC1-INH, ecal-


lantide, icatibant, or rhC1-INH outside of a medical facility, either by the patient alone or with assistance from a caregiver or trained medical individual. BENEFITS OF HOME TREATMENT Home treatment of HAE has grown in response to recognized drawbacks of treatment in a healthcare facility, which include delays in initiating treatment after the onset of an attack; the inconvenience of traveling to a healthcare facility, which may cause patients to forgo treatment; and prolonged waiting times in emergency departments, which may result in progression of the attack, more severe symptoms, and the need for more doses of medication.25,26 Moreover, not all facilities routinely stock agents that are specific for the treatment of HAE, many do not have extended hours, and not all providers are familiar with HAE. Providers who are not familiar with the disease may administer inappropriate and ineffective treatment (such as antihistamines or corticosteroids) or be reluctant to treat early in the course of an attack. Home treatment of HAE is modeled on home therapy programs for patients with immunodeficiency, hemophilia, and other chronic conditions and has been used successfully for more than 30 years.27–30 Home administration of on-demand treatment results in a shorter time from the onset of attack to the initiation of treatment, shorter attack duration, and less severe attacks.30,36,38,39,44 Home administration of prophylactic treatment of HAE results in reduced frequency of attacks compared with previous treatment, fewer days spent in a hospital, and fewer days missed from school or work.31,36,39 Patients using home treatment for HAE report improved quality of life and better functioning in work, educational, and social settings.30 –32,37,38 Numerous studies have demonstrated that home administration of HAE medications is safe, with a low rate of adverse events.30 –32,36 –39





PATIENT SELECTION Given the considerable burden of seeking care for HAE attacks from a healthcare provider and the improved outcomes associated with home administration of HAE treatments, all patients with HAE should be considered for home therapy.18,19,25 A survey of healthcare providers and reports of clinical experience have found that most patients learn self-administration of HAE drugs quite readily, often in only a few hours, and that they retain the skills over long-term use.33,40 In a recent survey of home therapy nurses, almost three-quarters of respondents reported that less than 10% of their patients decide against home administration of HAE medications after being trained.34 Certain individual personal factors should be considered when evaluating patients for suitability for training in home therapy of HAE (see also Table 3):

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C1-INH Intravenous Berinert: - On-demand treatment - Short-term prophylaxis (Europe only) Cinryze: - Prophylaxis - On-demand treatment (Europe only) Yes

Berinert (CSL Behring) Cinryze (ViroPharma)


Kallikrein inhibitor Subcutaneous On-demand treatment

Kalbitor (Dyax)


Bygum et al.37 N ⫽ 9 pts; follow-up ranging from 27–72 months; improved QOL and no serious complications Tourangeau et al.38 N ⫽ 21 pts treating at home for 8 weeks; home injection more efficacious and equally safe compared with clinic treatment Kreuz et al.39 N ⫽ 20 pediatric pts; no side effects or injection site complications Shapiro40 N ⫽ 10 pts; no serious AEs or administration issues, other than 1 instance where the site of swelling interfered with i.v. placement Instructional videos (Berinert) http:// www.berinert.com/professional/dosingadministration/self-administrationtraining.aspx Path to Independence program (Cinryze): http://www.cinryze.com/hae-patientindependence.aspx

Levi et al.36 N ⫽ 31 pts; mean of 21 injections per pt over 3.8 years; technical failure rate ⫽ 1.8%; no serious AEs

Yes, with assistance of a healthcare professionalb Kuhlen et al.42 Data collected as part of manufacturersponsored home infusion service; N ⫽ 158 pts and 1,177 nursing visits; 9 reports of hypersensitivity reactions

Farkas et al.45 N ⫽ 2 pts; 65 attacks; No safety issues reported and patient satisfaction was high

Boccon-Gibod and Bouillet43 N ⫽ 15 pts; 55 attacks; no serious AEs; injection site reactions in 48/55 attacks Maurer et al.44 N ⫽ 136 pts; 233 self-treated attacks; early treatment of attack (⬍1 hour) significantly reduced attack duration and time to resolution; AEs not discussed


C1-INH Intravenous On-demand treatment (except laryngeal attacks)

Bradykinin receptor agonist Subcutaneous On-demand treatment


Ruconest (Pharming Group, NV)

Kalbitor Home Infusion Services: http:// www.kalbitor.com/ patient/patientsupport/kalbitorhome-infusionservices.html

Recombinant C1-INH

Firazyr (Shire)


Training video: http:// www.firazyr.com/usingfirazyr

AE ⫽ adverse event; C1-INH ⫽ C1 esterase inhibitor; i.v. ⫽ intravenous; HAE ⫽ hereditary angioedema; QOL ⫽ quality of life. a Cinryze approved for prophylaxis only in the United States b Healthcare professional assistance required because of risk of anaphylaxis.41 The manufacturer offers home infusion services.

Manufacturer-sponsored assistance with home administration:

Published data on home treatment

Approval for homebased administration

Trade Name (manufacturer) Mechanism of action Route of administration Indication(s)

Plasma-Derived C1-INH

Table 1 Overview of HAE treatments





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Table 2 Recommendations for home treatment of hereditary angioedema Inclusions • Home therapy training should be inclusive. • Extremes of age or lack of an infusion partner are not necessarily contraindications. • Prophylaxis, if required, should be optimized while home therapy training is ongoing. Attack treatment • Attacks should be treated as early as possible. • Dose of C1-INH concentrate should be individualized. • Prophylactic C1-INH concentrate regimens may be implemented for certain patients. Site of attack • Attacks at all sites may be treated at home. • In the case of laryngeal edema, urgent transfer to hospital is recommended after treatment. Counseling/consent • Physician and patient should take joint and individual responsibility for the safe and appropriate use of home therapy. Training • Should take place in a center experienced in HAE management. • Should offer ongoing support and refresher training.


C1-INH ⫽ C1 esterase inhibitor; HAE ⫽ hereditary angioedema. Source: Adapted from reference 25.



Table 3 Criteria for patient selection for home treatment of hereditary angioedema

• Proven C1-INH deficiency. • The patient’s use of and compliance with prescribed prophylactic therapy should be optimal. • In order to maintain required infusion skills, the patient should normally require infusion of C1-INH at least every 3 months. • The patient must be motivated to comply with the home therapy program and all its implications and be willing to be responsible for administering home therapy. Written consent confirming this must be obtained before the program is commenced. • When applicable, the patient must be counseled regarding the risk of transmissible infections from a blood product. The patient should demonstrate an understanding of this and provide written informed consent to receive therapy. • The patient should have a partner willing to attend the home therapy program who will be present when therapy is required. • Written confirmation of support for home therapy must be established with the patient’s general practitioner, including emergency support or an agreed pathway of emergency care. • The patient must have access to a telephone when administering therapy. • Patients planning to use C1-INH must have good venous access. • For C1-INH users, the patient must agree to call for an ambulance if self-cannulation is unsuccessful when concentrate is required.



C1-INH ⫽ C1 esterase inhibitor. Source: Adapted from reference 46.

Is the patient mentally and physically capable of learning drug self-administration? Is the patient reliable (e.g., does the patient arrive on time for medical appointments or call to postpone or cancel when necessary)? Is the patient eager to learn and willing to invest the necessary time in training? Does the patient have adequate veins for administration of an i.v. drug?

Will the patient require the assistance of a caregiver because of age or disability? Certain characteristics of the disease should also be considered when evaluating patients for training in home therapy of HAE. Patients with frequent attacks are the most obvious candidates. There is no specific frequency of attacks that makes home therapy appropriate for an individual patient, although a rough guideline is that patients who have two or more attacks

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Table 4 Essential topics when training patients in home administration of C1-INH concentrate • • • • •

Background information on the disease and treatment possibilities. Which HAE attacks require treatment and when to initiate treatment. Storage, reconstitution and administration of C1-INH concentrate through venipuncture with a butterfly needle. Hygiene and how to work properly with needles and syringes. Documentation of each C1-INH concentrate replacement and swelling attack in a diary with registration of adverse effects. • Management of potential adverse events. • Instruction in handling a potential allergic response with epinephrine (Epipen). • An appropriate back-up plan if initial treatment is not successful, including guidance on when to seek emergency treatment at a local hospital or medical facility. • Advice as to when to consult a physician if the attack symptoms are atypical or accompanied by fever. C1-INH ⫽ C1 esterase inhibitor; HAE ⫽ hereditary angioedema. Source: Adapted from references 37 and 47.

per month will get the greatest benefit. Highly motivated patients with less frequent attacks may also do well on home therapy. The frequency of attacks also may help determine whether the patient should be using on-demand or prophylactic therapy, although again, there is no specific frequency that would signify that one or the other is indicated. Ideally, the patient should be administering the HAE medication often enough that she/he retains proficiency in the technique. If drug administration is not performed routinely, the patient may benefit from occasional review of infusion or injection technique. The site of HAE attacks should also be considered, because patients whose hands are often affected may have difficulty administering an injectable medication. The patient’s living situation also should be evaluated before initiating training for home treatment of HAE. Patients without easy access to an emergency department or clinic because of geographic distance or limited access to transportation, or who travel frequently, may benefit from learning selfadministration of an HAE medication. If concerns exist, a nurse may conduct a home visit to determine the safety and cleanliness of the home and confirm that there is an orderly family environment. The patient and family should be interviewed to identify a family member who can participate in the training and serve as a backup for administering treatment. A communication system should be in place so that if problems occur during administration, the patient can contact the healthcare team promptly.


OPTIONS FOR FACILITATING HOME TREATMENT Using Current Staff Recruiting current staff members to train patients in home treatment of HAE is an appealing option, be-



cause it ensures continuity of care and is convenient for the patient. The staff member who is to conduct the training must become highly knowledgeable about HAE and not just about the administration of i.v. and subcutaneous drugs. Therefore, staff training may be necessary before patient training can begin. In addition, staff should have ongoing access to an HAE expert for consultation and troubleshooting. Once staff members are competent in discussing HAE and drug administration, training the patient (and/or a caregiver) can usually be accomplished in two to three sessions depending on the patient’s aptitude and comfort with needles and, if i.v. administration is required (pdC1-INH or rhC1-INH), the quality of the patient’s veins. Table 4 provides a list of topics which should be considered when training patients for home administration of injectable HAE medications. In the first session, the staff member should confirm that the patient understands HAE and its treatment, teach the patient how to mix the drug when required, demonstrate the technique of i.v. or subcutaneous drug administration, and allow the patient to carry out the administration on his/ her own. Subsequent sessions should be devoted to continued practice of drug administration in front of the staff and answering questions that arise. It is highly recommended that another capable individual, such as a family member or caregiver, undergo training at the same time as the patient. Particular attention should be paid to management of HAE attacks affecting the airway. Patients should be trained to recognize the early symptoms of laryngeal attacks, to start treatment promptly if they occur, and to seek or call for emergency help if there is any sign of lack of response or worsening symptoms after administration of an HAE agent. Patients should be instructed to bring their reserve supply of medication with them to the hospital.




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Partnering With a Home Care Agency or Specialty Pharmacy Home care agencies and specialty pharmacies can be found in most communities and may be a valuable resource for training patients in home treatment of HAE. Staff members at these providers are practiced in educating patients about disease states, most can teach subcutaneous drug administration, and many can teach i.v. self-administration. Home care agencies can provide follow-up with home visits, during which it may be easier to include family members in the educational process. Before patients are referred to a home care agency or specialty pharmacy, it is important to confirm that there is staff available that is knowledgeable about HAE and that has a comprehensive understanding of HAE-specific medications (particularly of the associated techniques and risks of administration). Ecallantide is not recommended to be administered without the assistance of a healthcare professional because of the risk of anaphylaxis41; however, the manufacturer offers home infusion nursing services to assist with home administration (see Table 1). Partnering With a Local Infusion Center The staff at infusion centers routinely work with patients with immunodeficiencies, hemophilia, and other conditions that require home administration of drugs and specialize in educating patients and teaching self-care. An infusion center staffed by healthcare providers who are familiar with and experienced in the management of HAE may be able to serve as the primary point of care for patients with this condition.





Partnering With an HAE Comprehensive Care Clinic There are comprehensive care clinics specializing in the diagnosis and management of HAE. They are widely used in Europe and Canada, where this model was developed in response to the recognition that most physicians have little experience with this rare disorder, that patients often suffer unnecessarily as a consequence, and that the management of HAE is highly specialized, complex, and requires a multidisciplinary approach.16 A comprehensive care clinic is expected to track patient outcomes as well as develop and meet standards of care for patients with HAE.16 Clinical guidelines now call for all patients with HAE to have a consultation with an expert in the disease.16 –20,35 The goals of such recommendations are to provide the proper diagnostic evaluation, to develop an individualized, comprehensive management plan for each patient, and to advance basic and clinical research efforts intended to improve the clinical care of patients with HAE. In addition, many HAE centers provide robust HAE educational resources for patients and families

and clinical training for other healthcare providers. The staff generally includes a nurse specialist who educates patients on the disease state and trains them in home administration of HAE-specific medications. Key to the success of comprehensive care clinics is the collection of outcomes data that can be used as feedback and that permits these clinics to continuously improve the quality of care provided. National HAE registries for both clinical and research purposes are frequently maintained at these centralized specialty centers. The United States Hereditary Angioedema Association Angioedema Center at the University of California, San Diego is the first center in the United States focused on comprehensive clinical care, research, and education in HAE. Patients who live within easy traveling distance of a comprehensive care clinic may use it as their primary source of HAE care. However, patients may choose to travel regionally or nationally to such a center for an initial work-up and, possibly, for periodic follow-up evaluations. Such circumstances require a team-based approach to the management plan: the patient may have local healthcare providers consult and collaborate with the comprehensive care center to ensure local accessibility and attention, as the management plan requires. In addition, there is increasing interest among centers in the use of “telemedicine,” with advanced communication technology making remote ongoing consultative care possible. Although regulatory, legal, and reimbursement issues currently limit this approach, future progress in this area may extend the reach and presence of comprehensive care centers. Comprehensive care clinics generally encourage patients to attempt home care because of the demonstrated improvements in clinical outcomes.


Patient Training Materials Print or electronic patient education materials can be highly useful in the management of HAE and are available from several sources, including the United States Hereditary Angioedema Association (www.haea. org), a patient advocacy and educational organization. Instructional materials, online videos and person training are offered by a number of drug manufacturers (see Table 1). Written instructions on dosage and administration, preferably multilingual, should be provided for all patients who are given medication to be used at home. These materials may supplement but should not be expected to replace individualized training by a skilled instructor. Follow-Up With Patients Treating HAE at Home On-going communication between healthcare providers and patients is essential to the success of home treatment of HAE, especially given the expected de-

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crease in direct contact between patient and physician. Ideally, after the initiation of a management plan, patients should follow up with their provider after each attack and report on their treatment experience. Home healthcare providers may be helpful in facilitating this communication regarding the relative success or failure of a given treatment plan for each individual patient. The nursing staff should check in with the patient periodically by telephone to assess the treatment program and help patients with problems encountered and to schedule follow-up appointments. International HAE experts recommend routine follow-up patient visits once every three months initially, then once every six months as the patient becomes more comfortable with home administration.24 Frequently, treatment plans need to be adjusted or changed based on various dynamic factors (patient work or home situation, drug efficacy or side effects, variability in HAE symptoms, etc.). Each patient should have a backup plan in case an attack does not respond to the primary therapy, and the plan should be reviewed periodically to aid retention. All drug products distributed to patients should be tracked so that they can be retrieved in the event of a product recall. Finally, other healthcare providers the patient sees, including dentists and obstetricians/gynecologists, should be made aware of the patient’s HAE and the steps that should be taken in the event of an attack.












15. 16.


ACKNOWLEDGMENTS Editorial assistance was provided by Churchill Communications (Maplewood, NJ), funded by CSL Behring.




Zuraw BL. Clinical practice. Hereditary angioedema. N Engl J Med. 359:1027–1036, 2008. Bork K, Meng G, Staubach P, and Hardt J. Hereditary angioedema: New findings concerning symptoms, affected organs, and course. Am J Med 119:267–274, 2006.

Cicardi M, Aberer W, Banerji A, et al., HAWK under the patronage of EAACI (European Academy of Allergy and Clinical Immunology). Classification, diagnosis, and approach to treatment for angioedema: Consensus report from the Hereditary Angioedema International Working Group. Allergy 69:602– 616, 2014. Zuraw BL, Bork K, Binkley KE, et al. Hereditary angioedema with normal C1 inhibitor function: Consensus of an international expert panel. Allergy Asthma Proc 33:S145–S156, 2012. Caballero T, Aygo¨ren-Pu¨rsu¨n E, Bygum A, et al. The humanistic burden of hereditary angioedema: Results from the Burden of Illness Study in Europe. Allergy Asthma Proc 35:47–53, 2014. Zilberberg MD, Nathanson BH, Jacobsen T, and Tillotson G. Descriptive epidemiology of hereditary angioedema hospitalizations in the United States, 2004 –2007. Allergy Asthma Proc 32:248 –254, 2011. Bork K, Hardt J, Schicketanz KH, and Ressel N. Clinical studies of sudden upper airway obstruction in patients with hereditary angioedema due to C1 esterase inhibitor deficiency. Arch Intern Med 163:1229 –1235, 2003. Bork K, Staubach P, Eckardt AJ, Hardt J. Symptoms, course, and complications of abdominal attacks in hereditary angioedema due to C1 inhibitor deficiency. Am J Gastroenterol 101:619 – 627, 2006. Bork K, Hardt J, and Witzke G. Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. J Allergy Clin Immunol 130:692– 697, 2012. Zotter Z, Csuka D, Szabo E, et al. The influence of trigger factors on hereditary angioedema due to C1-inhibitor deficiency. Orphanet J Rare Dis 28;9:44, 2014. Morcavallo PS, Leonida A, Rossi G, et al. Hereditary angioedema in oral surgery: Overview of the clinical picture and report of a case. J Oral Maxillofac Surg 68:2307–2311, 2010. Bork K, Hardt J, Staubach-Renz P, and Witzke G. Risk of laryngeal edema and facial swellings after tooth extraction in patients with hereditary angioedema with and without prophylaxis with C1 inhibitor concentrate: A retrospective study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 112:58 – 64, 2011. Lumry WR, Castaldo AJ, Vernon MK, et al. The humanistic burden of hereditary angioedema: Impact on health-related quality of life, productivity, and depression. Allergy Asthma Proc 31:407– 414, 2010. Gomide MA, Toledo E, Valle SO, et al. Hereditary angioedema: Quality of life in Brazilian patients. Clinics (Sao Paulo) 68:81– 83, 2013. Huang SW. Results of an on-line survey of patients with hereditary angioedema. Allergy Asthma Proc 25:127–131, 2004. Bowen T, Cicardi M, Farkas H, et al. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma Clin Immunol 6:24, 2010. Caballero T, Baeza ML, Caban˜as R, et al., Spanish Study Group on Bradykinin-Induced Angioedema (SGBA). Consensus statement on the diagnosis, management, and treatment of angioedema mediated by bradykinin. Part II. Treatment, follow-up, and special situations. J Investig Allergol Clin Immunol 21:422– 441; quiz 442– 443, 2011. Cicardi M, Bork K, Caballero T, et al. Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: Consensus report of an International Working Group. Allergy 67:147–157, 2011. Craig T, Aygo¨ren-Pu¨rsu¨n E, Bork K, et al. WAO guideline for the management of hereditary angioedema. World Allergy Organ J 5:182–199, 2012. Gompels MM, Lock RJ, Abinun M, et al. C1 inhibitor deficiency: Consensus document. Clin Exp Immunol 139:379 –394, 2005.



CONCLUSIONS A growing body of evidence demonstrates that home-based treatment of HAE offers improved outcomes, including shorter time to initiation of treatment, reduced duration of attacks, reduced severity of attacks, and improved quality of life. Various avenues are available to facilitate patients’ transition to home therapy. With proper training, most motivated patients are fully capable of learning to self-administer HAE medications on their own or with the help of a family member. Giving patients the opportunity to self-manage their disease without traveling to a medical facility can add significant independence and control to the lives of HAE patients and reduce the burden of this chronic illness.







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Facilitating home-based treatment of hereditary angioedema.

Hereditary angioedema (HAE) is a rare disorder causing periodic attacks of nonpruritic swelling, for which highly effective subcutaneous and intraveno...
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