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Home > Drugs > Hereditary angioedema agents > Ruconest > Ruconest: 7 things you should know
Hereditary angioedema agents
https://themeditary.com/patient-tips/ruconest-657.html

Ruconest: 7 things you should know

Drug Detail:Ruconest (C1 esterase inhibitor recombinant injection (conestat alfa))

Drug Class: Hereditary angioedema agents

Contents
Uses Warnings Before Taking Dosage Side effects Interactions

1. How it works

  • Ruconest is a brand (trade) name for a man-made form of a protein called C1 esterase inhibitor, that naturally occurs in our plasma. Ruconest may be used to treat attacks of hereditary angioedema (HAE) in adults and children. HAE is a rare genetic condition that occurs in people with low levels of certain proteins (C1-INH and C4) in their bodies.
  • The C1 esterase inhibitor protein helps regulate several cascade systems within our body, such as the complement system, the intrinsic coagulation system and the coagulation cascade (the process that causes our blood to clot), and the fibrinolytic system (the process that removes and degrades clots after blood vessels are repaired). It also helps control bradykinin production (bradykinin is a protein fragment that increases the movement of fluid through blood vessel walls, resulting in swelling and inflammation).
  • People with hereditary angioedema (HAE) have low levels of naturally occurring C1-INH in their blood. During an attack, these levels of C1-INH become so low that angioedema (severe swelling in the limbs, face, intestinal tract, and airway) occurs. Ruconest adds working C1-INH, which brings levels of C1 back up and closer to normal, reducing bradykinin production, decreasing the permeability of blood vessels, and reducing swelling.
  • Ruconest belongs to the class of medicines known as C1 esterase inhibitors. It may also be called a hereditary angioedema agent

2. Upsides

  • May be used to treat acute attacks of hereditary angioedema (HAE) attacks in adults and adolescents. Effectiveness has not been established in HAE patients with laryngeal attacks.
  • Reduces swelling.
  • The recommended dosage is 50 units per kg body weight by slow intravenous injection over approximately 5 minutes, with a maximum total dosage of 4200U. Do not exceed 4200U per dose use and if attack symptoms persist, no more than 2 doses should be administered within 24 hours.
  • Bodyweight should be used to calculate the dosage for people weighing less than 84kg, but for people weighing more than 84kg, use 2 vials (4200U).
  • Available in a 2100U vial for reconstitution with 14mL of sterile water for injection.

3. Downsides

If you are between the ages of 18 and 60, take no other medication or have no other medical conditions, side effects you are more likely to experience include:

  • Headache, nausea, and diarrhea were the most common side effects reported with Ruconest in adults. In adolescents, abdominal pain, headache, and oropharyngeal pain were most commonly reported.
  • Not suitable for people with a history of allergy to rabbits or rabbit-derived products.
  • Not suitable for people who have developed life-threatening, immediate hypersensitivity reactions, including anaphylaxis, to Ruconest or other C1 esterase inhibitors.
  • Severe hypersensitivity reactions (severe allergies) have been reported and epinephrine should be available for immediate treatment if a reaction occurs. Signs of a hypersensitivity reaction include hives, rash, fast heartbeat, difficulty breathing (wheezing), chest tightness, feeling light-headed or faint, and swelling of your face, lips, tongue, or throat that occur during or soon after the administration of Ruconest. Note that hypersensitivity reactions may have similar symptoms to HAE attacks. If a hypersensitivity reaction occurs, discontinue Ruconest and administer appropriate treatment, such as epinephrine, if necessary.
  • Serious thromboembolic events (blood clots) have been reported with C1 esterase inhibitor products, such as Ruconest, The risk is greater in those with an indwelling catheter, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives, certain androgens, morbid obesity, or immobility. Weigh up the benefits of using Ruconest in these people versus the risk of an event.
  • May not be as effective at treating facial and laryngeal (around the larynx) attacks compared to abdominal or limb attacks.
  • Only for intravenous use. Has not been approved for self-administration, although appropriately trained patients may self-administer upon recognition of an HAE attack. Water for injection is not included in the Ruconest package.
  • Because Ruconest is made from human blood there is a risk of transmitting infectious agents, such as viruses, and theoretically, the Creutzfeldt-Jakob disease vector.
  • Requires reconstitution before self-administration. The dosage of Ruconest is based on body weight, so it is individualized for each person, except for people over 84kg for whom the dosage is 2 vials (4200U).
  • There is also the potential for immunogenicity and antibody formation.
  • Not to be used for the prevention of HAE. Other medications, such as Haegarda, do this.
  • There are no adequate, well-controlled studies regarding the use of Ruconest in pregnant women. Animal studies could not exclude an effect on embryo-fetal development. Only use Ruconest if needed. It is not known if Ruconest is excreted into human milk.
  • No generic is available.

Note: In general, seniors or children, people with certain medical conditions (such as liver or kidney problems, heart disease, diabetes, seizures) or people who take other medications are more at risk of developing a wider range of side effects. View complete list of side effects

4. Tips

  • Ruconest is usually administered by a trained healthcare professional. If you want to learn how to self-administer Ruconest at the time of an HAE attack, ask your doctor or nurse to give you a demonstration and provide you with the appropriate equipment. Do not attempt to self-administer Ruconest without the appropriate training and approval from your health professional.
  • You should tell your healthcare professional if you have any signs of an allergic reaction during your Ruconest injection or soon after. Signs of an allergic reaction may include hives, rash, fast heartbeat, difficulty breathing (wheezing), chest tightness, feeling light-headed or faint, and swelling of your face, lips, tongue, or throat.
  • Report any signs of a blood clot, such as swelling, pain, discoloration, a feeling of warmth, in an arm or leg, shortness of breath, chest pain or discomfort that worsens on deep breathing, numbness or weakness on one side of the body.
  • Because Ruconest is a blood product, it does carry a risk for transmission of infectious agents such as viruses, but this is unlikely because the risk of transmitting disease has been reduced by carefully selecting blood donors, testing donors for infections, and inactivating viruses during the manufacturing process.
  • Talk to your healthcare provider about the risk of taking this drug during pregnancy or during breastfeeding especially if you are considering trying to become pregnant or breastfeeding. Tell your doctor if you inadvertently become pregnant. There is limited information about the use of Ruconest in pregnant women.

5. Response and effectiveness

  • The average time to relieve HAE symptoms with Ruconest is 90 minutes (95% CI range 61 minutes to 150 minutes). Some substudies indicate a larger treatment effect in men than women.
  • Among patients who achieved relief within 4 hours, 27% of patients in the placebo group had a relapse of their symptoms compared with 3% in the Ruconest group.
  • 42% of patients in the placebo group required rescue medication compared with 11% in the Ruconest group.
  • Only 50% of patients with facial HAE attacks achieved relief with Ruconest 50U/kg compared with 88% with abdominal attacks and 85% with peripheral attacks.

6. Interactions

Medicines that interact with Ruconest may either decrease its effect, affect how long it works, increase side effects, or have less of an effect when taken with Ruconest. An interaction between two medications does not always mean that you must stop taking one of the medications; however, sometimes it does. Speak to your doctor about how drug interactions should be managed.

Common medications that may interact with Ruconest include:

  • anti-cancer medications such as carfilzomib
  • danazol
  • epoetins
  • evening primrose oil
  • hormones, such as estrogen, estradiol, nandrolone, or testosterone
  • lenalidomide
  • raloxifene
  • streptokinase
  • tamoxifen
  • thalidomide
  • thrombolytics such as alteplase, anistreplase, or urokinase.

Note that this list is not all-inclusive and includes only common medications that may interact with Ruconest. You should refer to the prescribing information for Ruconest for a complete list of interactions.

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