Alpha-1 Proteinase Inhibitor Human

Alpha-1 proteinase inhibitor, or alpha-1 inhibitor, is sometimes used to treat emphysema.

Overview

US Brand Name

  • Aralast
  • Zema
  • Aralast NP
  • Prolastin
  • Prolastin-C
  • Glassia

Descriptions

Alpha-1 proteinase inhibitor injection (alpha 1-PI) can be used in the treatment of some kinds of emphysema, a lung disease. Emphysema caused by a lack of alpha 1-antitrypsin (AAT) protein can be successfully treated with alpha in the body. This medicine provides the protein if your body can’t make enough of it.

Only take alpha 1-PI under your physician’s care and supervision.

Condition(s) treated

  • Emphysema

Type of medicine

  • alpha1-antitrypsin (AAT)

Side Effects

When taking this or other medication, you are likely to experience side effects. Not all side effects require medical attention, and you may not experience any known side effects.

However, it’s good to be prepared for the eventuality. Check with a qualified professional if any of the following occur prior to taking alpha 1-PI:

Most common side effects:

  • Nasal congestion
  • Difficulty with breathing
  • Bloody or cloudy urine
  • Loss of voice
  • Chills
  • Unusual tiredness or weakness
  • Ear congestion
  • Difficult, burning, or painful urination
  • Sneezing
  • Runny nose
  • Headache
  • Lower back or side pain
  • Body aches or pain
  • Cough
  • Sore throat
  • Fever
  • Bladder pain
  • Frequent urge to urinate

Rarer

  • Muscle aches and pains
  • Sweating
  • Unusual weight gain or loss
  • Joint pain
  • Cough producing mucus
  • Chest pain
  • Dizziness
  • Loss of appetite
  • Bloating or swelling of the hands, arms, face, lower legs, or feet
  • Tingling of the hands or feet
  • Diarrhea
  • Tightness in the chest
  • Feeling of discomfort or illness
  • Slow or fast heartbeat
  • Trouble sleeping
  • Feeling faint or lightheadedness
  • Pounding in the ears
  • Nausea
  • Shivering
  • Nervousness
  • Noisy breathing
  • Blurred vision
  • Vomiting
  • Flushing or redness of the skin, especially on the face and neck
  • Difficult or labored breathing
  • Rapid weight gain

Chance of occurrence not sufficiently tracked

  • Confusion
  • Difficulty with swallowing
  • Faintness when rising from a lying or sitting position
  • Fast, pounding, or irregular heartbeat or pulse
  • Hives or skin rash
  • Itching, puffiness or swelling of the face, eyes, lips or tongue

You may experience other symptoms that don’t require medical attention. These effects often stop once your body is used to the medication. Your doctor can tell you how to prevent them. If these less serious symptoms happen to you, consider consulting your doctor, especially if they persist.

More common

  • Muscle or bone pain
  • Sudden sweating
  • Pain or tenderness around the eyes and cheekbones
  • Feeling of warmth
  • Itching skin
  • Redness of the face, neck, arms, and occasionally, upper chest

Rare

  • Fever
  • Swelling of the joints
  • Change in taste
  • Back pain
  • Bloating
  • Redness of the skin
  • Changes in vision
  • Sleepiness or unusual drowsiness
  • Hives or welts
  • Loss of taste
  • Pain

You may get a feeling of drowsiness, tiredness, dullness or sluggishness. These side effects, like the more serious ones, don’t occur in every patient.

Call your doctor for information on how to handle any side effects from alpha 1-PI. Call 1-800-FDA-1088 to report them to the FDA.

Dosage

This product comes in these dosage forms:

  • Powder for Solution
  • Solution

Clinical studies have shown this drug is believed to be safe for alpha1-PI deficiency. As such, it’s administered weekly at a time suggested by your doctor, and the dosage averages 60 mg/kg of body weight

Zemaira, a popular brand that sells the drug, comes in single-use vials with about approximately 1,000 mg of active A1-PI. This dosage consists of a powder that you can reconstitute using 20 mL of a special water called Sterile Water for Injection. You won’t typically have to worry about this, since a trained nurse or health care technician will give you the injection in a clinical setting such as a hospital. This is a typical injection, with the needle placed directly in a vein.

This medicine is given through a needle placed in one of your veins. Your doctor does this weekly and on the same schedule every week.

In some circumstances, you may be asked to learn to give yourself the treatment, which can save time and expense. However, don’t commit to the injections if you don’t feel qualified or feel unable to do so for other reasons. It’s vital that you understand all instructions before attempting to give yourself your weekly dosage.

Alpha-1 PI is dispensed with an informational pamphlet. If you follow the instructions to the letter, you will experience far fewer adverse effects. As always, this is a good opportunity to ask your healthcare provider for additional information.

Whether you give yourself the shot or go to a clinic, carefully record the details of each shot to be used in case of a medical emergency. Include the lot number on the dosage, date and time of the injection and any strange reactions.

Interactions

Drug Interactions

There is always a chance that certain medicines used together will cause unwanted interactions. Unfortunately, these interactions can vary from person to person. Make sure your doctor has a record of all the prescription and over-the-counter meds you are on while using alpha-1 PI.

The mixture of drugs can result in strong symptoms that impair your health, sometimes permanently. By keeping your doctor informed, you allow her to be prepared in case of a medical emergency. To start, she may change the timing or dosage of your prescriptions, if any, to avoid conflicting with your alpha-1 P1 schedule. The good thing is that the weekly dosage provides a lot of flexibility.

Keep a list of all prescription or nonprescription medication taken on an as-needed basis, in case you need it later.

Other Interactions

Certain medicines should be avoided while taking certain medicines. Your doctor can answer specific concerns about any unwanted interactions. Smoking tobacco products and consuming alcohol while taking alpha-1 PI is not advisable. Ask your healthcare professional whether there are any major concerns about eating certain foods while using this medicine.

Warnings

When you decide to use a medicine, you usually get an opportunity to weigh up the pros and cons. However, with a life-threatening condition like emphysema, you often have little real choice if you want to get better and your treatment options are limited.

Nevertheless, discuss the good and bad impacts on your health with your doctor. For alpha-1 PI, consider these warnings:

Allergies

If you have had allergic reactions to alpha-1 PI or other medication, it’s important to provide a detailed listing of other allergies too. So, note or jot down allergies to animals, foods, preservations and other food-related products. For non-prescription food products, read the ingredients carefully, especially after introducing changes to your body, such as taking alpha-1 PI for emphysema.

Pediatric

At this time, there isn’t enough information available regarding giving alpha-1 PI to children for the treatment of emphysema. Therefore, the impacts of alpha-1 proteinase inhibitor is not known for pediatric patients. Consequently, it's not known if the drug works on children nor whether such treatment would be effective for children.

Geriatric

Similarly, there isn’t enough information available regarding giving alpha-1 PI to geriatric patients for the treatment of emphysema. Therefore, the impacts of alpha-1 proteinase inhibitor is not known for geriatric patients. Consequently, there isn’t a reliable determinant for alpha-1 PI treatment specific to the elderly.

Breastfeeding

While breastfeeding, it’s important to know how a medication can impact your baby. Unfortunately, the literature available provides no comprehensive study or results for the impact of alpha-1 PI on the mother or child in this example.

Other Medical Problems

If you have certain medical conditions, they can impact the efficacy of alpha -1 PI on your emphysema. Emphasize the following in discussions with your doctor:

  • IgA deficiency with antibodies against IgA

Do not be use alpha-P1 if you have this condition.

Your doctor may be able to propose an alternative therapy for your emphysema or change your IgA deficiency regime.

It's very important that your doctor checks you closely while you are receiving this medicine. By making gradual adjustments, he can make sure you are at the least possible risk.

Caution

Alpha-1 PI can cause allergic reactions, including anaphylaxis. Anaphylaxis requires immediate medical attention. Call your doctor at the first sign of the following if you are taking other medicines with alpha-1:

  • Itching
  • Rash
  • Dizziness, fainting
  • Trouble swallowing
  • Swollen face, hands and extremities

Alpha 1-PI is derived from the processing of human blood. Sometimes, using human blood products leads to the unintentional transmission of various viruses to patients who require them.

Fortunately, this risk has decreased with the advent of screening and testing donors prior to accepting their plasma. When donors are tested, the results indicate whether they are eligible to donate. If a human donor has harmful viruses, they can be passed on to you.

Although this risk isn’t significant, ask a health care professional about it if you have concerns. Your doctor can give you a hepatitis B vaccine before giving you the alpha-1 PI injection.

Storage

Alpha-1 PI dosages, such as in the brand Zemaira, come in single use vials containing the required amount of active A1-PI printed on the label.

If you are taking the medicine at home, store it up to 25 degrees Celsius, or 77 degrees Fahrenheit. Zemaira is stable before its expiration date, which should be included on the label. Freezing the drug can compromise the vial and make that dose unsafe to use.

Summary

Alpha-1 antitrypsin deficiency (AATD) is treated successfully with alpha-1 PI therapies. It has no impact on the underlying disorder, which is genetic. In the disorder, protease inhibitor (AAT) is produced by your liver, but doesn’t properly secrete in a serum your body can absorb.

Alpha-1 PI imitates naturally occurring AAT circulating in your body, and it protects normal tissue from damage caused by and untreated by neutrophil proteolytic enzymes. Interference with the weekly dosage can cause adverse effects.

The protective activity of alpha-1 PI, emulating the body’s AAT can leave you vulnerable to lung damage and, eventually, emphysema. Your body will also revert to pooling natural AAT in hepatic cells, which can lead to liver damage.

In general, this can be a life-saving drug if used with proper care and diligence.

Resources
Last Reviewed:
December 10, 2017
Last Updated:
December 22, 2017
Content Source: