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

Hereditary angioedema, also known as HAE, is a genetic condition that can cause severe swelling in different parts of your body.

Hereditary angioedema (HAE) affects about 1 in 50,000 people.
HAE is an inherited disease but can occur even without a family history of the disease.

HAE is a rare disease generally classified into 3 types

TYPE I

Characterized by low levels (below 35% of normal) and low inhibitory function of the C1 esterase inhibitor (C1-INH)
Approximately 85% of HAE C1-INH cases

TYPE II

Characterized by normal or elevated levels of C1-INH, but the protein does not function properly
Approximately 15% of HAE C1-INH cases

HAE with normal C1-INH levels
(HAE-nl-C1INH)

Formerly known as type III HAE
Most cases are due to unknown cause
There are currently 6 genetic mutations identified in patients with HAE-nI-C1INH: FXII, plasminogen, angiopoietin-1, KNG1, MYOF, and HS3ST6

FXII=coagulation factor 12; HS3ST6=heparan sulfate 3-O-sulfotransferase 6; KNG1=kininogen-1; MYOF=myoferlin

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.

HAE attacks can occur anywhere on the body, and vary in length and severity. Therefore, it is important to treat HAE at the first sign of a swell.

Because all swells are not visible, it is important to learn to recognize the early signs that a swell may be coming. These differ from person to person and may include:

HAE attack symptom: fatigue

Fatigue

HAE attack symptom: rash

Rash

HAE attack symptom: stomach upset

Stomach upset

HAE attack symptom: headache

Headache

HAE attack symptom: stomach cramps

Difficulty swallowing and breathing

HAE attack symptom: numbness or tingling

Numbness or tingling

HAE attack symptom: muscle and joint pain

Muscle and joint pain

RUCONEST, a C1-inhibitor, replaces the missing or nonworking C1-INH to stop an HAE swell before it fully develops
US HAEA Medical Advisory Board 2020 Guidelines state early treatment is important to prevent swell progression, no matter how severe. After self-administered treatment, you should seek medical care if the attack involves the airway.

Even though swells may occur without a clear trigger, it's important to recognize some common triggers that can lead to an attack, such as:

Anxiety
Stress
Minor injuries
Surgical or dental procedures
Certain medications (e.g., estrogen-containing birth control medications)
Infections
Hormonal changes, such as those occurring during pregnancy or menstruation

In mose HAE-affected patents, defined trigger factors precede HAE swells. Food can be a relevant factor in triggering angioedema attacks or swells.

Suspected foods include:

Fish
Shrimp
Chicken
Bread
Milk
Cheese
Tree Nuts
Citrus Fruits
Apple
Strawberry
Pineapple
Banana
Kiwi
Tomato
Garlic
Onion
Leek
Green Salad
Chili
Alcohol

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

The US Hereditary Angioedema Association (HAEA) is a nonprofit patient advocacy organization dedicated to serving people with angioedema. They provide patients and families with a support network and a wide range of services.

Preparing for Your Visit

Prepare for a visit with your healthcare provider with this helpful discussion guide.

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Why RUCONEST?

RUCONEST is a plasma-free C1-INH proven to help treat HAE attacks.

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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.