What is HAE? HAE Attack Info. | Ruconest® (C1 esterase inhibitor [recombinant])

Hereditary angioedema, also known as HAE, is a rare genetic condition that affects the blood vessels and organs and can cause severe swelling in different parts of your body.

HAE can affect both adults and children, with symptoms getting worse after puberty.
HAE is an inherited disease but can occur even without a family history of the disease.

HAE is a rare disease generally classified into three types

Hereditary Angioedema Type I

Patients have low levels of C1 inhibitor (C1-INH), a protein that helps to control swelling.
HAE Type I is the most common, making up 80-85% of HAE cases.

Hereditary Angioedema Type II

Patients have normal-or-above normal levels of C1 inhibitor (C1-INH), but this protein does not function properly.
HAE Type II makes up 15-20% of HAE cases.

HAE-nl-C1INH
(formerly known as Type III)

Patients have normal functioning levels of C1-INH proteins, but there is still swelling.
New information suggests HAE normal is higher than previously thought (ranging between 19-23%) which would impact the prevalence of Type I and Type II.

Hereditary angioedema occurs because of a genetic mutation of the C1-INH protein, causing your body to be out of balance.

Without normal and functional amounts of C1-INH, the body generates too much of a protein peptide called bradykinin. Bradykinin causes excessive fluid to leak through the walls of body tissues, which leads to inflammation and episodes of swelling.

If one of your parents has HAE, you have a 50% chance of having it too. Yet while HAE is an inherited disease, it can occur without a family history of the disease.

1 in 50,000 people is affected by hereditary angioedema

When C1-INH levels are too low or C1-INH is not working properly, fluid can leak from the blood vessels into the body and cause swelling.

Picture of a hand experiencing a swell. It shows a normal hand and version where the hand and fingers are swollen.

If you’re suffering from HAE, there is an organization working diligently to make sure you know you’re not alone

HAE advocacy groups

The US Hereditary Angioedema Association (HAEA) is a nonprofit patient advocacy and research organization dedicated to serving people with angioedema and their families. They provide a wide variety of services and resources that support the HAE community.

WHAT IS RUCONEST?

RUCONEST® (C1 esterase inhibitor [recombinant]) is an injectable prescription medicine that is used to treat swelling and/or painful attacks due to hereditary angioedema (HAE) in adult and adolescent patients over the age of 12.

IMPORTANT SAFETY INFORMATION

Do not use RUCONEST if you have experienced life-threatening immediate hypersensitivity reactions, including anaphylaxis, to RUCONEST or to any other C1 esterase inhibitor (C1-INH) product. Call your healthcare professional or the emergency department right away if you experience: wheezing, difficulty breathing, chest tightness, turning blue (look at lips and gums), fast heartbeat, swelling, faintness, rash or hives. A serious side effect of life-threatening hypersensitivity (anaphylaxis) was reported in a clinical study.

Do not use RUCONEST if you have a known or suspected allergy to rabbits or rabbit-derived products. Tell your healthcare provider about all your medical conditions, including if you have a known allergy to rabbits. Before starting RUCONEST, tell your healthcare provider about any other medications you are taking, as some medications, such as birth control pills and certain androgens, can increase risk of clotting problems. Blood clots have occurred in patients receiving plasma-derived C1-INH product. Also tell your healthcare provider if you are pregnant, breastfeeding, or planning to do so.

The most common side effects patients experienced during clinical studies include headache, nausea and diarrhea. These are not all the possible side effects of RUCONEST. Tell your healthcare provider about any side effect that bothers you or does not go away. You can also report negative side effects to Pharming Medical Affairs at (800) 930-5221, or contact the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

If symptoms persist, a second dose of RUCONEST may be taken at your recommended dose. Do not take more than two doses within 24 hours.

For more information, please see the full Prescribing Information including the Patient Product Information.