This post is for all those who have been prescribed and approved for augmentation therapy.
You probably already know that the basic goal of augmentation therapy is to increase the level of alpha-1 antitrypsin More (Alpha-1 antitrypsin) protein and to restore the balance between the alpha-1 antitrypsin More and mediators of inflammation including A powerful enzyme released from white blood cells to fight infection, but it can attack normal tissues (especially the lungs) if not tightly controlled by alpha-1 antitrypsin. Source: Genetics Home Reference. More. The therapy cannot restore lost lung function – nor is it considered a cure. The hope is that by replacing the deficient protein, the progression of lung destruction will be slowed or arrested. In addition, there is evidence that augmentation therapy can reduce the frequency and severity of exacerbations.
Because augmentation therapy is considered preventative rather than curative; that is to say, it puts the brakes on lung destruction rather than reversing it, it seems logical to begin therapy as soon as possible after Alpha-1 lung disease has been detected. If your doctor has prescribed augmentation therapy for you, and you have been approved, do not put it off… get going on it ASAP!
The approved dose is 60 mg/kg of body weight administered once weekly via intravenous infusion, on an ongoing basis for each of the currently available augmentation products.
Some things to know about augmentation therapy:
- You do not need to monitor or re-test your antitrypsin levels – The levels of antitrypsin found in the lungs following prolonged augmentation therapy tend to be much more stable and consistent than the levels found in the blood. Adjusting dosages based on blood tests will not give a consistent protective level in the lungs.
- Dosing should be once a week unless there is a significant reason not to (e.g. logistical problems that would make weekly dosing impossible). Scientists have found that antitrypsin spikes on infusion day and then quickly drops off over the next few days, so the only way to stay covered is to keep to a once a week schedule.
- Don’t throw any away! – Your doctor will prescribe your dose based on your weight. But that number may not match the amount in the bottles… All products are available in a vial size of approximately 1,000 mg active alpha-1 antitrypsin More per vial. (Prolastin and Aralast also are available in a 500 mg vial size as well.) If you weighed 150 lbs (or 68 kg), your dose would be 4,080 mg… that would mean either rounding down to 4 bottles or having extra after you received your dose. Instead of throwing that extra away, you’d want to use the entire vial for your infusion. Always round up!
Hopefullythis is already usual practice everywhere, but you may want to check with your nurse or person giving you your infusions to be sure.
Dr. Ken Chapman, Vancouver Education Day 2018 – Alpha-1 Canada