Uses of Adagen

The drug adagen is a man-made form of an enzyme called adenosine deaminase (ADA). On the market today there is a lot of information about adagen drug products, but it is still incomplete. To better understand what adagen drug is, what is the use of adagen drug, let's learn more in the article below.

Pegademase bovine (Adagen) is a man-made form of an enzyme, also known as adenosine deaminase (ADA). ADA is very important in the body helping to prevent the accumulation of certain proteins that are harmful to white blood cells that help your body fight infections.
Adagen is used to replace ADA in people with severe combined immunodeficiency disease (SCID).
Adagen may also be used for purposes not listed in this medication guide.
Drug form and content of adagen drug: 250 UI/Ml injection solution and 1.5mL vial.
Pharmacodynamics and mechanism of action of the drug adagen: Adagen has the effect of converting adenosine (toxic) to inosine (less toxic) by deamination. It also converts 2'-deoxyadenosine to 2'-deoxyinosine through this reduction process.
In the absence of adenosine deaminase enzyme in your body, the purine adenosine substrates as well as 2'-deoxyadenosine and their metabolites are toxic to lymphocytes, causing lymphocyte death. immune system decline.
Adagen Pharmacokinetics: Absorption Following an intramuscular dose, the drug is rapidly absorbed and peak plasma adenosine deaminase concentrations persist for the next 2 to 3 days.
Distribution When pegademase is administered weekly at a dose of 15 IU/kg, plasma adenosine deaminase activity ranges from 20 to 25 μmol/hour/mL.
Metabolism Not reported.
Elimination The elimination half-life of adenosine deaminase in plasma will range from 3 days to more than 6 days.

2.1. Interactions with other drugs Adagen is structurally similar to elapegademase-lvlr and should not be used together due to possible interactions. Vidarabine is a substrate of both the enzyme adenosine deaminase ADA as well as pentostatin (2-deoxy co formycin), a potent ADA inhibitor. Therefore, the activity of these drugs can be significantly altered if used in combination.
2.2. Interactions with food The use of drugs with food, alcohol or tobacco can all cause interactions.
2.3. Drug incompatibilities Do not dilute or mix pegademase with any other drug before use.
Drug interactions may change the ability of the drug to work or increase the effect of side effects. This document does not cover all possible drug interactions. Make a list of all the medicines you are taking (including prescription, nonprescription and dietary supplements) and show them to your doctor or pharmacist.

Adagen is used to treat adenosine deaminase enzyme deficiency in people with severe combined immunodeficiency, increases the number of blood cells, thereby helping to prevent and block infectious agents and eliminate agents that induce lymphocyte death.
The use of bovine pegademase is recommended for infants or children of all ages.

Non-adenosine deaminase-associated immunodeficiency such as AIDS. Bone marrow failure. Bone marrow transplant. Hypersensitivity to adenosine deaminase, elapegademase-lvlr, or to any of the other ingredients in the product

Get emergency medical help if you have any of these signs of an allergic reaction: hives ; shortness of breath; swelling of your face, lips, tongue, or throat.
Call your doctor right away if you have:
Easy bruising, unusual bleeding (nose, mouth, vagina or rectum) and purple or red spots under your skin; Pale or yellow skin, dark urine, fever and confusion or weakness Signs of infection - fever, chills, sore throat, mouth sores, flu symptoms, and skin sores or swelling. Common side effects may include:
Headache Redness or itching where the injection was given. This again is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects.

You should not use Adagen if you are allergic to it, or if you have:
Severe thrombocytopenia (low levels of platelets in your blood). To make sure Adagen is safe for you, tell your doctor if you have: Easy bruising or bleeding. FDA pregnancy category C. It is not known whether Adagen will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant while using Adagen.
It is not known whether Adagen passes into breast milk or if it could harm a nursing baby. Talk to your doctor if you are breastfeeding.
All instructions on your medicine label should be followed. Your doctor may sometimes change your dose to make sure you get the best results. Do not take this medicine in larger or smaller amounts or for longer than recommended.
Adagen injected into the muscle. You will likely be taught how to use the injection at home. Do not self-inject this medication if you do not understand how to inject and properly dispose of used needles and syringes.
Adagen is usually injected every 7 days. Follow your doctor's medication instructions carefully.
Use disposable needles and syringes only once. Follow any state or local laws regarding the disposal of used needles and syringes. Use a puncture-proof "sharps" container (ask your pharmacist where to get one and how to throw it away). Please keep this container out of reach of children and pets.
Each single-use vial (bottle) of this medicine will be for one use only. Throw away immediately after one use, even if there is still some medicine in it after injecting your dose.
Do not use Adagen if it has changed color or has particles in it. Call your pharmacist to get a new medicine.
While using Adagen your blood will need to be checked regularly.
It may take up to 6 months before your immune system improves and you are less likely to get infections. Continue using the medicine as directed and tell your doctor if your symptoms do not improve.
Call your doctor if your symptoms do not improve after the first few weeks of treatment.
Store in the refrigerator, do not freeze. The medicine must be thrown away if it has become frozen.

7.1. General Notes The maintenance dose to improve immune function should ensure that the following goals are achieved:
Maintain adenosine deaminase activity in the range of 15 to 35 μmol/hr/mL. Decrease in erythrocyte deoxyadenosine triphosphate (dATP) content by approximately ≤ 0.005 to 0.015 μmol/mL in red blood cells or 1% in total adenin nucleotide (ATP + dATP) content in red blood cells (this normal ATP level will be required to be measured first). when injected). Dosage adjustment may be necessary when there is evidence of antibody formation, tolerance, and restoration of adenosine deaminase ADA activity.
Use with caution in patients with thrombocytopenia.
Note that only a single dose of medicine should be used for each potion and discard the remaining unused medicine.
Store the medicine at a cold temperature between 2 and 8 degrees C (or 36 to 46 degrees F), do not store at room temperature or temperatures that cause freezing of the medicine.
When the patient's erythrocyte deoxyadenosine triphosphate (dATP) level falls below the therapeutic target, dATP level monitoring continues at 2 to 4 times/year for the first year of treatment. , then you can reduce the frequency of testing to 2 to 3 times/year.
After treatment with Adagen for 3 to 9 months, plasma adenosine deaminase ADA activity should be checked twice monthly, then may be lowered to once per month for 18 to 24 months of treatment. next treatment. For patients treated for 2 years, ADA activity levels should be checked every 2-4 months. Monitoring may be more frequent if treatment is interrupted or the patient is showing signs of increased clearance.
The increase in drug clearance may be related to the appearance of antibodies, which directly inhibit both endogenous ADA as well as Adagen, when the patient is indicated to be treated with an intramuscular dose of 20 IU/kg , used twice a week, no adverse events have been reported at this dose level. The concentration of ADA in the plasma will gradually be restored, the patient will be returned to treatment with a dose of 20UI / kg per week after 4 months.
Adagen must be used continuously and for life, do not arbitrarily stop treatment without permission from the doctor. Regular treatment interruptions weaken the body's immune system, increasing the risk of serious infections.
The degree of improvement of the immune system can vary according to the patient's condition, so each patient will have appropriate care requirements.
For patients taking medication at home, carefully consult all accompanying instructions for use and consult with qualified medical personnel. Always make sure that the solution product is clear and colorless, should be checked visually before use, do not use if the solution contains particles or is discolored.
The body's immune system will usually improve slowly after a certain period of time, within 2 to 6 months or at the latest, there should be a clear effect by the end of the first year of treatment.
7.2. Note to pregnant women should only be used during pregnancy when absolutely necessary.
7.3. Cautions for Lactation: There are not enough studies to determine the possible risk of the drug to the nursing infant. Therefore, it is necessary to weigh the potential benefits against the risks for the baby before deciding to use the drug.