Our Therapies

ZEMAIRA® Infusion Therapy

autoimmune condition affecting the eyes and surrounding tissues

About ZEMAIRA® Infusion Therapy

ZEMAIRA® (Alpha1-Proteinase Inhibitor, Human) is made from human plasma and replaces a protein your body is not making enough of. That protein, alpha-1 antitrypsin (also called alpha1-proteinase inhibitor, or AAT), shields delicate lung tissue from neutrophil elastase, an enzyme white blood cells release during normal immune activity. When AAT levels are very low, elastase slowly breaks down the elastic tissue in the lungs, and that is what produces emphysema in alpha-1 antitrypsin deficiency.

Weekly infusions raise and maintain the amount of AAT circulating in your blood and lining your lungs. This is called augmentation therapy, and it helps to be clear about what it is meant to do. It replaces the missing protein to help protect the lung tissue you still have. It does not reverse damage that has already happened, and it is not a treatment for how you feel day to day.

Dosing and administration

60 mg/kg of body weight, given intravenously once every week
Supplied as a lyophilized powder in 1,000 mg single-use vials, reconstituted with the sterile water provided just before your infusion
Infused at roughly 0.08 mL per kg per minute, which works out to about 15 minutes for most adults
Continued at the same weekly interval long term, since protection depends on keeping AAT levels steady rather than on reaching a finish line

Two things are confirmed before the first infusion. ZEMAIRA® should not be given to people with selective IgA deficiency who have antibodies against IgA, or to anyone with a history of severe reaction to an alpha1-proteinase inhibitor product. And because ZEMAIRA® is made from human plasma, hepatitis A and hepatitis B vaccination is recommended for patients receiving it.

In the RAPID clinical trial program, patients receiving weekly alpha1-proteinase inhibitor therapy lost lung density more slowly than those receiving placebo. That remains the clearest evidence that augmentation therapy slows the structural progression of emphysema in alpha-1 antitrypsin deficiency.

Condition treated by this therapy

ZEMAIRA® is FDA approved for a single indication:

Chronic augmentation and maintenance therapy in adults with emphysema due to severe congenital deficiency of alpha1-proteinase inhibitor, known as alpha-1 antitrypsin deficiency or AATD

A few limits on that indication are worth stating plainly, because they decide whether a referral can be filled:

Adults only. Safety and effectiveness in children have not been established.
Emphysema has to be clinically evident. Augmentation therapy is not indicated for people who carry a deficient genotype but have no lung disease.
The effect on pulmonary exacerbations and on the rate at which emphysema progresses has not been established in the labeling, so the benefit is framed around protecting lung tissue rather than relieving symptoms.
ZEMAIRA® does not treat the liver disease that some people with AATD develop. Liver injury in AATD comes from abnormal protein building up inside liver cells, not from a shortage in the bloodstream, so replacing the circulating protein does not address it.

ZEMAIRA®, PROLASTIN-C®, GLASSIA®, and ARALAST NP are separate products. They share a mechanism but differ in formulation, vial size, reconstitution, and infusion rate, so moving between them should be prescriber-directed rather than assumed.

ZEMAIRA® Price

The cost of prescription medications can vary depending on your insurance coverage. If you're concerned about the price of your medication:

Contact your insurance provider to understand your coverage
Ask your doctor or pharmacist about lower-cost alternatives
Check with the drug manufacturer about financial assistance programs
Explore patient assistance options if you're uninsured or underinsured

Remember, many resources exist to help patients access necessary medications. Don't hesitate to ask for help if you're struggling with medication costs. No patient should go without necessary treatment due to financial limitations. If you have questions about financial assistance opportunities, contact a member of our Violet Health team.

What to Expect During ZEMAIRA® Infusion Therapy

ZEMAIRA® is a prescription medicine used to treat adults with emphysema caused by severe alpha-1 antitrypsin deficiency. It was approved by the FDA in 2003, and at Violet Health we're proud to offer it in our spa-like infusion center, a comfortable alternative to traditional hospital settings.

Here is what a typical ZEMAIRA® visit looks like:

Your weight is confirmed, since the dose is calculated from it
The powder is reconstituted at the time of your appointment and given through a standard peripheral IV, no port or central line needed
The infusion itself is short, usually around 15 minutes, and most visits run under an hour from check-in to walking back out
No premedication is required, and most patients drive themselves to and from treatment
Your care team watches for signs of a reaction during the infusion and for a short period afterward, and the rate can be slowed or paused if you feel unwell

The rhythm is the real adjustment. ZEMAIRA® is a weekly commitment with no maintenance phase that stretches the interval out, so we work around your schedule and can usually hold the same day and time each week. Consistency matters here more than on most therapies, because AAT levels fall between doses and the protection depends on keeping them topped up.

ZEMAIRA® Side Effects

ZEMAIRA® is generally well tolerated, and the most commonly reported side effects in clinical studies were mild. They can include:

Headache, the most frequently reported reaction
Upper respiratory infection, sinusitis, or sore throat
Cough or bronchitis
Infusion site reactions such as redness, swelling, or discomfort
Dizziness or lightheadedness
Tiredness, fever, or a flu-like feeling after an infusion

Respiratory symptoms are common in this group of patients to begin with, so tell your care team about any change in cough, sputum, or breathlessness rather than assuming it is the medication or assuming it is not.

Get emergency help right away if you experience signs of a severe allergic reaction:

Trouble breathing or wheezing
Swelling of the face, throat, or tongue
Hives or a rash spreading over your body
Chest tightness or chest pain
Fainting, a racing heartbeat, or a sudden drop in blood pressure

Important Safety Information

Who should not receive ZEMAIRA®

People with selective IgA deficiency who have antibodies against IgA, because of the risk of a severe allergic reaction
Anyone with a history of severe hypersensitivity to an alpha1-proteinase inhibitor product or to any component of ZEMAIRA®

Risks to be aware of

Hypersensitivity and anaphylaxis can occur, including in patients who have tolerated previous infusions. Treatment is stopped immediately if a severe reaction happens, and emergency medication is kept on hand in the center.
Because ZEMAIRA® is made from human plasma, it carries a theoretical risk of transmitting infectious agents, including viruses and, in theory, the Creutzfeldt-Jakob disease agent. Donor screening, plasma testing, and viral inactivation steps during manufacturing make this risk very low, and no transmission has been attributed to the product. Hepatitis A and hepatitis B vaccination is recommended.

A few contrasts with other therapies offered here are worth noting. ZEMAIRA® has no boxed warning. There is no dose-reduction ladder, since the dose is fixed at 60 mg/kg and a difficult infusion is managed by slowing the rate rather than lowering the amount. And there is no contraception requirement, though ZEMAIRA® has not been studied in pregnancy or breastfeeding, so tell your care team if you are pregnant, planning a pregnancy, or nursing.

Augmentation therapy protects lung tissue, but it does not replace the rest of your care. Smoking cessation remains the single most important thing a patient with AATD can do, and vaccinations, pulmonary rehabilitation, and inhaled therapies continue alongside weekly infusions.

What Your Referral Should Include

Eligibility for augmentation therapy turns on documentation more than on clinical impression, so a complete referral moves scheduling along considerably. Please include:

Serum alpha-1 antitrypsin level with the date and the reporting lab
Genotype or phenotype results naming the variant, for example PI*ZZ, PI*SZ, or a null allele, with the lab report attached
Evidence of clinically evident emphysema: pulmonary function testing with FEV1 and DLCO, and chest CT findings if available
Smoking status, and whether cessation support is in place for current smokers
IgA level, or confirmation of known IgA deficiency and anti-IgA antibody status, since this is a contraindication rather than a caution
Prior alpha1-proteinase inhibitor therapy, which product, and any reaction history
Hepatitis A and hepatitis B vaccination status, plus influenza and pneumococcal vaccination history
Current weight, since the dose is weight-based and recalculated as weight changes
Venous access notes, including any history of difficult sticks
Liver involvement, including transaminases and any known AATD-related liver disease
Supplemental oxygen use, pulmonary rehabilitation participation, and recent exacerbation history
Confirmation that ZEMAIRA® specifically is the intended product rather than another alpha1-proteinase inhibitor, and whether a payer preference applies

ZEMAIRA® Infusion Therapy at Violet Health

Your comfort during ZEMAIRA® infusion therapy at Violet Health is our priority. Our patients enjoy walk-up parking, complimentary entertainment and snacks, and a team of highly trained providers during each treatment. Plus, Violet Health's evening and weekend hours and convenient city and rural locations make it easier to keep a weekly appointment that fits around work and family.

Violet Health Infusion Centers provides infusion therapy for patients with rare, chronic, or complex conditions in a comfortable, patient-centered atmosphere.

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