Alpha1-Proteinase Inhibitor: Uses, Benefits & Alpha-1 Antitrypsin Therapy

Summary of Key Points

Alpha1-Proteinase Inhibitor is a medication used to treat conditions associated with Alpha-1 Antitrypsin Deficiency, such as emphysema and chronic obstructive pulmonary disease (COPD). It works by replacing a missing protein that helps protect the lungs from damage. This medication is typically administered through an intravenous (IV) infusion. Always consult your healthcare provider before starting or adjusting your Alpha-1 Antitrypsin therapy.

Names of Medication

US Brand Names: Prolastin-C, Aralast NP, Zemaira, Glassia

Generic Name: Alpha1-Proteinase Inhibitor (Human)

The generic name refers to the active ingredient, while the brand name is assigned by the manufacturer. The generic name is listed in parentheses.

Pharmacologic Category

Alpha1-Proteinase Inhibitor

Protease Inhibitors

Dosing

Alpha1-Proteinase Inhibitor is used to treat various conditions related to Alpha-1 Antitrypsin Deficiency. The dosage may vary depending on the specific condition being treated. Always consult your healthcare provider before adjusting your dose or starting treatment. You can also schedule a telemedicine appointment with one of our providers for personalized advice.

Alpha-1 Antitrypsin Deficiency

Dosage: 60 mg/kg once weekly

Frequency: Once a week

Length of Treatment: Ongoing, as directed by your healthcare provider

Chronic Obstructive Pulmonary Disease (COPD)

Dosage: 60 mg/kg once weekly

Frequency: Once a week

Length of Treatment: Ongoing, based on your doctor’s recommendation

Emphysema

Dosage: 60 mg/kg once weekly

Frequency: Once a week

Length of Treatment: Long-term treatment, as prescribed by your healthcare provider

Liver Disease Related to Alpha-1 Antitrypsin Deficiency

Dosage: 60 mg/kg once weekly

Frequency: Once a week

Length of Treatment: Ongoing, under the supervision of your healthcare provider

Bronchiectasis

Dosage: 60 mg/kg once weekly

Frequency: Once a week

Length of Treatment: Long-term, as advised by your doctor

Asthma

Dosage: 60 mg/kg once weekly

Frequency: Once a week

Length of Treatment: Ongoing, depending on your healthcare provider’s recommendation

Pulmonary Fibrosis

Dosage: 60 mg/kg once weekly

Frequency: Once a week

Length of Treatment: Long-term, as directed by your healthcare provider

Cystic Fibrosis

Dosage: 60 mg/kg once weekly

Frequency: Once a week

Length of Treatment: Ongoing, under the supervision of your doctor

Interstitial Lung Disease

Dosage: 60 mg/kg once weekly

Frequency: Once a week

Length of Treatment: Long-term, as prescribed by your healthcare provider

Dosage Forms and Strengths

Injection, solution: 1000 mg/50 mL (Prolastin-C)

Injection, solution: 1000 mg/50 mL (Aralast NP)

Injection, solution: 1000 mg/50 mL (Zemaira)

Injection, solution: 1000 mg/50 mL (Glassia)

Administration Instructions

Alpha1-Proteinase Inhibitor is administered as an intravenous (IV) infusion, typically once a week. The infusion is usually performed in a healthcare setting, but some patients may be trained to administer it at home. Follow your healthcare provider’s instructions carefully. If you miss a dose, contact your doctor or schedule a telemedicine appointment to discuss the next steps.

Adverse Reactions and Side Effects

Like all medications, Alpha1-Proteinase Inhibitor may cause side effects. Not everyone will experience them, but it is important to be aware of potential reactions. If you experience severe side effects, contact your healthcare provider immediately or schedule a telemedicine appointment with one of our providers.

Common Side Effects (1-10% of patients):

1. Headache: Mild to moderate headaches may occur after the infusion.
2. Dizziness: Some patients report feeling lightheaded or dizzy.
3. Fatigue: You may feel more tired than usual after treatment.

Less Common Side Effects (Less than 1% of patients):

1. Fever: A low-grade fever may occur after the infusion.
2. Chills: Some patients experience chills during or after the infusion.
3. Rash: A skin rash may develop, which could indicate an allergic reaction.

Rare but Serious Side Effects:

1. Allergic Reactions: Severe allergic reactions, including difficulty breathing, swelling of the face or throat, and hives, may occur. Seek immediate medical attention if this happens.
2. Blood Clots: In rare cases, blood clots may form, leading to pain, swelling, or redness in the limbs.

Contraindications

A contraindication is a specific situation or condition in which a medication should not be used because it may be harmful. If you have any of the following conditions, you should not take Alpha1-Proteinase Inhibitor or should discuss these risks with your healthcare provider.

1. Known hypersensitivity to Alpha1-Proteinase Inhibitor: If you have had an allergic reaction to Alpha1-Proteinase Inhibitor or any of its components, you should not take this medication. Allergic reactions can range from mild to severe, including anaphylaxis, which can be life-threatening.
2. IgA deficiency with antibodies against IgA: Patients with IgA deficiency who have developed antibodies against IgA are at risk of severe allergic reactions, including anaphylactic shock, when taking Alpha1-Proteinase Inhibitor. This medication contains IgA, which can trigger these reactions in susceptible individuals.
3. Severe liver disease: In patients with advanced liver disease, the metabolism and clearance of Alpha1-Proteinase Inhibitor may be impaired, leading to potential complications. Discuss with your healthcare provider if you have liver disease before starting this medication.

Drug to Drug Interactions

Alpha1-Proteinase Inhibitor may interact with other medications, potentially altering its effectiveness or increasing the risk of side effects. Inform your healthcare provider of all medications you are taking, including prescription drugs, over-the-counter medications, and supplements. Some known interactions include:

1. Immunosuppressants: Medications like azathioprine (Imuran) or cyclosporine (Neoral, Sandimmune) may interact with Alpha1-Proteinase Inhibitor, potentially affecting your immune system. This could increase the risk of infections or reduce the effectiveness of either medication.
2. Blood thinners: Drugs like warfarin (Coumadin) or heparin may increase the risk of bleeding when taken with Alpha1-Proteinase Inhibitor. Your healthcare provider may need to adjust your dose or monitor your blood clotting levels more closely.
3. ACE inhibitors: Medications such as lisinopril (Zestril, Prinivil) or enalapril (Vasotec) may increase the risk of allergic reactions when taken with Alpha1-Proteinase Inhibitor. Be sure to inform your healthcare provider if you are on these medications.

Alpha1-Proteinase Inhibitor in Pregnancy

Is it safe to take Alpha1-Proteinase Inhibitor during pregnancy?

There is limited information regarding the safety of Alpha1-Proteinase Inhibitor use during pregnancy. While animal studies have not shown harm to the fetus, there is a lack of comprehensive human studies. If you are pregnant or planning to become pregnant, it is essential to discuss the potential risks and benefits of this medication with your healthcare provider. Together, you can determine whether the benefits of Alpha1-Proteinase Inhibitor therapy outweigh any potential risks to you and your baby.

Alpha1-Proteinase Inhibitor while Breastfeeding

Is it safe to take Alpha1-Proteinase Inhibitor while breastfeeding?

It is not yet clear whether Alpha1-Proteinase Inhibitor passes into breast milk. Given the possibility of serious side effects in nursing infants, it is important to consult your healthcare provider about the risks and benefits of continuing this medication while breastfeeding. Your provider may suggest alternative treatments or recommend monitoring your baby for any potential adverse effects related to Alpha-1 Antitrypsin therapy.

Estimated Cost of Alpha1-Proteinase Inhibitor

The cost of Alpha1-Proteinase Inhibitor can vary depending on your location, pharmacy, and insurance coverage. Without insurance, the estimated cost for a 30-day supply, using a GoodRX coupon, ranges from $7,000 to $10,000. Be sure to check with your pharmacy and explore financial assistance or patient support programs that may help cover the cost of Alpha-1 Antitrypsin replacement therapy if needed.

Possible Alternatives to Alpha1-Proteinase Inhibitor

There are alternative treatments and lifestyle modifications that may help manage conditions treated with Alpha1-Proteinase Inhibitor. However, it is crucial to discuss any changes to your treatment plan with your healthcare provider before making adjustments. Our telemedicine providers are available to help you explore these options.

Alpha-1 Antitrypsin Deficiency

In addition to Alpha1-Proteinase Inhibitor therapy, lifestyle changes such as quitting smoking and avoiding lung irritants can help slow the progression of the disease. In more advanced cases, lung or liver transplantation may be considered for patients with Alpha-1 Antitrypsin deficiency.

Chronic Obstructive Pulmonary Disease (COPD)

Common treatments for COPD include bronchodilators, inhaled corticosteroids, and pulmonary rehabilitation. Quitting smoking and maintaining a healthy lifestyle can also significantly improve symptoms and overall quality of life. Alpha1-Proteinase Inhibitor may be recommended for COPD patients with Alpha-1 Antitrypsin deficiency.

Emphysema

Management of emphysema often involves the use of inhalers, oxygen therapy, and pulmonary rehabilitation. Avoiding smoking and environmental pollutants can also help reduce symptoms. Alpha1-Proteinase Inhibitor may be part of the treatment plan for patients with Alpha-1 Antitrypsin deficiency.

Liver Disease

For patients with liver disease related to Alpha-1 Antitrypsin deficiency, lifestyle changes such as avoiding alcohol and maintaining a healthy diet can be beneficial. In severe cases, liver transplantation may be necessary.

Bronchiectasis

Treatment options for bronchiectasis include antibiotics, bronchodilators, and chest physiotherapy to help clear mucus. Staying active and maintaining good hydration can also assist in managing symptoms.

Asthma

Asthma is commonly treated with inhaled corticosteroids, bronchodilators, and by avoiding known triggers. Lifestyle changes, such as regular exercise and weight management, can also improve asthma control.

Pulmonary Fibrosis

Management of pulmonary fibrosis often includes antifibrotic medications, oxygen therapy, and pulmonary rehabilitation. Quitting smoking and avoiding lung irritants are important lifestyle changes that can help manage this condition.

Cystic Fibrosis

Treatment for cystic fibrosis typically involves airway clearance techniques, inhaled medications, and enzyme supplements. Maintaining a high-calorie diet and staying physically active can also help manage symptoms.

Interstitial Lung Disease

Common treatments for interstitial lung disease include corticosteroids, immunosuppressants, and oxygen therapy. Lifestyle changes, such as quitting smoking and avoiding lung irritants, can also help slow the progression of the disease.

Alpha-1 Antitrypsin Deficiency-related Liver Disease

In addition to Alpha1-Proteinase Inhibitor therapy, lifestyle changes such as avoiding alcohol and maintaining a healthy diet are crucial. In severe cases, liver transplantation may be considered for patients with Alpha-1 Antitrypsin deficiency-related liver disease.

Recent Updates on Alpha1-Proteinase Inhibitor Research

Research on Alpha1-Proteinase Inhibitor continues to advance, with recent studies focusing on optimizing dosing regimens and improving patient outcomes. One area of ongoing research is the development of more efficient delivery methods, such as inhaled formulations, which could make Alpha-1 Antitrypsin replacement therapy more convenient for patients. Additionally, studies are exploring the potential benefits of early intervention in patients with Alpha-1 Antitrypsin deficiency to prevent the progression of lung and liver disease.

Another promising area of research is gene therapy, which aims to correct the underlying genetic defect in Alpha-1 Antitrypsin deficiency. While still in the experimental stages, gene therapy could offer a long-term solution by restoring normal levels of Alpha-1 Antitrypsin in the body.

As new research emerges, it is important to stay informed and discuss any potential changes to your treatment plan with your healthcare provider. Our telemedicine providers are available to help you navigate these updates and ensure you are receiving the most up-to-date care for your condition.

James Kingsley

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