Hereditary Angioedema

What is Hereditary Angioedema (HAE)

Hereditary angioedema (HAE) is a rare disease that causes unpredictable attacks of swelling that typically occur in the face, throat, hands, feet, stomach (abdomen) and genitals. People with hereditary angioedema (HAE) experience recurrent episodes, or attacks, of swelling in the skin and/or mucous membranes. Depending on the severity of the disease, some people will have frequent attacks, while others may have few throughout the year.1  Physical exertion, stress, and trauma are commonly reported triggers of an HAE attack.2

What are the symptoms of HAE?

Hereditary Angioedema (HAE) attacks are unpredictable. Some people will have one or two swelling attacks a year, while others may have one every one or two weeks. Many people have their first attack in childhood; attacks typically worsen after puberty and persist through adulthood.3

Prodromal symptoms can commonly precede Hereditary Angioedema (HAE) attacks. Swelling occurs within the skin and/or mucous membranes and can be extremely painful. Symptoms are usually worse during the first 24 hours and can last up to a week.3 Depending on the area of the body affected, people may not be able to leave the house—to go to work or to school for example—which can have a significant impact on their lives.4

HAE symptoms most often affect three areas of the body—skin, gastrointestinal (GI) tract, and upper airway.5

What is the diagnosis and treatment of HAE?

HAE Diagnosis
The rarity of HAE, diversity of its affected population, and the overlap in symptoms with other more common diseases, such as allergic angioedema and appendicitis, make diagnosing HAE challenging and an HAE diagnosis may be delayed. Skin symptoms are often misdiagnosed as allergic angioedema. Abdominal symptoms can be misdiagnosed as appendicitis. Awareness of HAE has increased in recent years; however, patients continue to experience misdiagnoses.6 Understanding and recording the patterns of your symptoms will help your doctor make a proper diagnosis. Tell your doctor about any family history of HAE.

HAE Treatment
Guidelines recommend that all HAE attacks are considered for on-demand treatment and that any attack affecting or potentially affecting the upper airway is treated. On-demand therapy, or acute treatment, is medicine used to treat the symptoms of an HAE attack.7

Patients should be evaluated at every doctor visit (at least once a year) for the need for long-term prophylactic treatment that reduces the likelihood of attacks in severely symptomatic HAE patients. You and your doctor should discuss whether long-term preventative treatment is right for you.

Short-term prophylactic treatment is for patients who know they will encounter a trigger and want to reduce the chance of an attack, such as a dental procedure or menstruation.

It is important to work with your doctor to develop a treatment plan that works for your individual needs.

[1] 2010 Lumry – The humanistic burden of HAE impact on HRQOL, productivity, and depressionREFERENCEvf1.15
[2] Zotter Z, Csuka D, Szabó E, et al. The influence of trigger factors on hereditary angioedema due to C1-inhibitor deficiency. Orphanet J Rare Dis. 2014;9:44.
[3] Lumry WR. Overview of epidemiology, pathophysiology, and disease progression in hereditary angioedema. Am J Manag Care. 2013;19(7 Suppl):s103-110.
[4] Lumry WR. Hereditary Angioedema: The Economics of Treatment of an Orphan Disease. Front Med (Lausanne). 2018 Feb 16;5:22.
[5] Genetic and Rare Diseases Information Center. Hereditary angioedema. National Institutes of Health.
[6] Zanichelli A, Longhurst HJ, Maurer M, et al. Misdiagnosis trends in patients with hereditary angioedema from the real-world clinical.
[7] 2018 Maurer – The international WAO_EAACI guideline for the management of hereditary angioedema – the 2017 revision and updateREFERENCEvf1.14

The contents of this site are not intended for the purpose of disease diagnosis or as a substitute for information that is provided to you by your physician. You should always discuss your medical condition and any questions you have with your doctor.

Learn more about the program support for Hereditary Angioedema offered through KabiCare by selecting the support title.

There are several ways to enroll in the programs provided by KabiCare. Your healthcare provider can enroll you online in the KabiCare Patient Support Program or by fax. Patients can opt-in for training or clinical support and note any communication preferences that the KabiCare Case Managers should be aware of.

Once enrolled in the program a Case Manager can help coordinate delivery to your home and can provide information related to your insurance coverage.

You will receive a phone call from a Case Manager to review your benefits and discuss other financial assistance programs you may be eligible to receive.

Based on the information we obtain during the benefits investigation there may be a need for a prior authorization (PA) from your insurance company. PA means the insurance company needs to approve a medication before it will cover all or some portion. If a PA is required, Case Managers will provide information regarding the applicable PA requirements.

Should a claim or PA be denied, a Case Manager will provide information regarding the appeal process, including information regarding the documentation needed to contest the denial.

Based upon the amount of a patient’s out-of-pocket expense, KabiCare will provide additional savings towards the cost of the co-pay, depending on eligibility requirements and program rules. Eligibility criteria apply. Patients are not eligible for commercial co-pay assistance support if the prescription is eligible to reimbursed, in whole or in party, by any federal or state health care program.

The Bridge Program provides a limited supply of medication to eligible (commercially insured) patients whose prescription dispense may be subject to delay. For eligible patients, the Case Manager will coordinate directly with the pharmacy to secure dispensing of the product directly to the patient’s residence or healthcare provider’s office. Eligibility criteria apply. Patients are not eligible for commercial Bridge Program support if the prescription is eligible to be reimbursed, in whole or in part, by any federal or state healthcare program.

Once the benefits investigation is complete, the Case Manager will determine the specialty pharmacy options available based on a patient’s health plan coverage.

As a KabiCare patient, inside the welcome kit you will find important information you need to increase your awareness about the KabiCare Patient Support Program and resources. A welcome kit will be provided to you or your caregiver once enrollment is complete.

Once patients opt-in to the program, KabiCare nurses are available to help you make sense of your disease, treatment and medication as prescribed by your healthcare provider. Nurse support provided by KabiCare is not meant to replace discussions with a healthcare provider regarding a patient’s care and treatment.

KabiCare’s clinical team offers support in a number of ways: including providing disease state education, reviewing frequently asked questions associated with therapy, and providing in home injection training. Nurse support provided by KabiCare is not meant to replace discussions with a healthcare provider regarding a patient’s care and treatment.

Self-Administration Training

Icatibant Injection requires training under the guidance of a healthcare professional. Please call 1-833-KABICARE (1-833-522-4227) to request a self-administration training kit.