Are you considering augmentation therapy? Experts agree that Alphas with emphysema should get it. However, they disagree about whether it’s the right approach for people with very mild or very severe lung disease.

Download Considering Augmentation Therapy – Is It Right for You as a PDF.

Some research studies have shown that people who fall in the middle range of severity get the most benefit from augmentation therapy. Why? With mild lung disease, you might not notice any improvement. If you have severe lung disease, your lung function declines very slowly. Again, it’s not easy to see any improvement.

Some members of the healthcare community use these findings to suggest that only people with moderate lung disease should receive augmentation therapy. But, augmentation therapy “puts the brakes” on lung damage. Why wouldn’t you begin therapy as soon as you know you have lung disease due to Alpha-1?

We don’t know which Alphas will get lung disease. So, we could argue that all Alphas should get augmentation therapy to keep their lungs as healthy as possible. And if it were safe, easy, inexpensive, proven to be effective and available in unlimited supply, most Alphas would probably get it. But that’s not the case.

Here’s a shortlist of things to do before you start augmentation therapy:

#1. Get an IgA deficiency test.

Immunoglobulin A (IgA) is a protein that fights infection. If you have inherited this condition, you may have severe allergic-type reactions to plasma products.

#2. Get Hepatitis A and B vaccines.

These vaccines reduce your risk of liver injury. You’ll get a series of three shots over a six-month period.

#3. Learn about augmentation side effects.

The vast majority of people who get this therapy have no side effects. The most common side effect is feeling drained or having flu-like symptoms. These effects last up to 24 hours after having an infusion. Slowing the rate of infusion can reduce or remove the symptoms.

Other possible side effects include:

  • Hives
  • Itching
  • Tightness in the chest
  • Shortness of breath (dyspnea)
  • Wheezing

Note: Taking an antihistamine like Benadryl before an infusion can get rid of these side effects.

In clinical trials, all four of the augmentation therapy products currently available showed similar rates of side effects. Switching to a different brand of augmentation therapy can get rid of persistent side effects. However, if you have severe systemic reactions to a certain brand, your doctor should monitor you closely while you try a new brand.

#4. Find the dose that’s right for you.

The FDA-approved dose for all four available augmentation products is 60 mg/kg of body weight given once a week by intravenous (IV) infusion. So, if you weighed 165 lbs (or 75 kg), your recommended dose would be 4,500 mg per week.

Some doctors think they should monitor your alpha-1 antitrypsin (AAT) levels and adjust your dose based on these levels. We don’t recommend this dosing approach at the present time.

That’s because AAT levels found in your lungs after long-term augmentation therapy tend to be much more stable and consistent than the levels found in your blood. Adjusting your dose based on blood levels may not have the desired effect on your lungs.

The FDA’s recommended doses promote a consistent protective level of AAT within your lungs. They won’t stop the progression of lung disease entirely. But so far, no study has found a dose that can do that. So, we suggest sticking with the FDA-approved dose of 60 mg/kg of body weight once a week.

If you have trouble getting to your infusion center or your doctor’s office, your doctor may adjust your dosing schedule to every two weeks. They may also suggest an extra dose if travel plans affect your infusion schedule. But, evidence shows that weekly dosing is the most effective.

If you have questions, we have answers!

For more in-depth information on this topic, please visit the Big Fat Reference Guide (BFRG). If you are enrolled in AlphaNet’s Subscriber Portal, you can access the BFRG here.

Download Considering Augmentation Therapy – Is It Right for You as a PDF.