What is alpha-1-proteinase inhibitor-injection, and how does it work (mechanism of action)?
Alpha-1 proteinase inhibitor
(Prolastin-C) is a prescription medicine used to treat alpha-1 antitrypsin (AAT)
deficiency. AAT deficiency is a genetic disorder in which abnormally low levels
of the protein AAT allow certain enzymes to attack healthy tissues, especially
the lungs. Over time, significant lung damage occurs, and patients develop serious
lung diseases like emphysema.
Alpha-1 proteinase inhibitor contains AAT donated by human subjects. AAT is
derived from the plasma, the fluid part of the blood, of healthy human donors.
Prolastin-C is a new formulation of Prolastin, the original alpha-1 proteinase
inhibitor that has been available since 1988. Prolastin-C is a more purified and
concentrated formulation that can be infused over a shorter period of time. When
administered at the recommended rate, Prolastin-C can be infused in
approximately 15 minutes. Additionally, unlike Prolastin, Prolastin-C does not
contain prions, the infectious agent that causes mad cow disease or
Creutzfeldt-Jakob disease. However, as alpha-1 proteinase inhibitor is derived
from human plasma, it carries the potential risk of transmitting other
infectious agents like viruses.
There is no cure for AAT deficiency but alpha-1 proteinase inhibitor can help
patients manage their condition. Alpha-1 proteinase inhibitor works in the lower
respiratory tract where it inhibits neutrophil elastase (NE), the enzyme that
damages lung tissue. In clinical trials, alpha-1 proteinase inhibitor therapy
increased the plasma levels of AAT. The effects of augmentation therapy on lung
symptoms and disease progression have yet to be proven in well-controlled
clinical trials.
What brand names are available for alpha-1-proteinase inhibitor-injection?
Prolastin-C
Is alpha-1-proteinase inhibitor-injection available as a generic drug?
No
Do I need a prescription for alpha-1-proteinase inhibitor-injection?
Yes
What are the side effects of alpha-1-proteinase inhibitor?
The most common side effects of alpha-1 proteinase inhibitor are:
- chills,
- malaise,
- headache,
- rash,
- hot flush, and
- pruritus.
- increase in enzymes used to monitor liver function,
- shortness of breath,
- serious hypersensitivity-type reactions or anaphylaxis, and
- rapid heartbeat.
What is the dosage for alpha-1-proteinase inhibitor?
The recommended dose of alpha-1 proteinase inhibitor
is 60 mg/kg administered into the vein (intravenously) once weekly.
Alpha-1 proteinase inhibitor should be administered at a rate of up to 0.08
milliliters for each kilogram per minute. On average, each infusion takes about
15 minutes.
Patients, family members, or caregivers can be trained to administer
alpha1-proteinase inhibitor at home.
Which drugs or supplements interact with alpha-1-proteinase inhibitor?
Information on drug-drug interaction is
not found in the medical literature and is not provided by the manufacturer.
Is alpha-1-proteinase inhibitor safe to take if I’m pregnant or breastfeeding?
Alpha-1 proteinase inhibitor has not been
adequately evaluated in pregnant women. Due to the lack of conclusive safety
data, alpha-1 proteinase inhibitor should be used in pregnancy only if the
potential benefit justifies the potential risk to the fetus. Alpha-1 proteinase
inhibitor is classified as FDA pregnancy risk category C.
It is not known if alpha-1 proteinase
inhibitor is excreted in breast milk. Because many drugs enter human milk and
can potentially cause harm to the nursing infant, alpha-1 proteinase inhibitor
should be used cautiously in nursing mothers.
What else should I know about alpha-1-proteinase inhibitor?
What preparations of alpha-1-proteinase inhibitor are available?
Alpha-1 proteinase inhibitor is available in a
kit containing a single-use vial of 1000 mg alpha-1 proteinase inhibitor as a
lyophilized powder, one 20 ml vial of sterile water for dilution, a transfer
needle, and a filter needle.
How should I keep alpha-1-proteinase inhibitor stored?
Alpha-1 proteinase inhibitor should be stored below
25 C (77 F). This medicine does not require refrigeration and should not be
stored in the freezer. After mixing, the solution can be kept at room
temperature for administration within 3 hours.