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Lung volume reduction surgery is not usually recommended by physicians for alpha-1 antitrypsin deficiency (AATD). This is partially due to the risky and rather unbalanced cost-benefit ratio for the patient. However, Ryner Lai, science writer for Rare Disease Advisor, reports that there are studies showing this procedure, along with one-way endobronchial valve treatment, have helped some patients with AATD in the article, The Merits of Lung Volume Reduction in Emphysema Driven by AATD.

Lai references the dire circumstances of end-stage chronic obstructive pulmonary disease (COPD) and the suffering those patients endure. He infers that lung volume reduction surgery might well be in high demand in the future, given the grievous state these patients eventually arrive at in life.

I like how he thinks because I have had my doctor throw around the term emphysema in my case. I’m not even 40 years old, and given the discomfort I am in, I can only imagine what the end will be like. Recently, I was getting a chest X-ray for chest pain, and they found that I already had hyperinflation. This was hard to hear, although not fully unexpected. It was just another reminder that a diagnosis of COPD was becoming more real to me than ever.

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Lai’s article states that the placement of endobronchial valves has been helpful for patients with AATD who dare to have it. It can be risky to have surgery on the lungs, but I am sure many patients with COPD would be willing to take the risk. I think I would, but I needed to research more about why lung volume reduction could help someone with AATD. I found some answers.

Read about comorbidities with AATD

I wanted to be sure I understood the reason emphysema is truly progressive to see if I might want this procedure. However, I have yet to find literature explaining the process emphysema tends to go through that makes it truly progressive. I understand that emphysema scarring is truly irreversible, and inflammation or irritation makes the situation worse. I just didn’t know the “why” behind the progressive nature of the disease and how to identify the underlining factors.

If I had to imagine why this happens, I would say it might be different for each person. For some, it may happen because the cause, ie, smoking, never ceased. That would make sense. I, however, do not and have never smoked. In my case, if AATD is the cause, getting augmentation therapy would be a good idea if that is why emphysema affected me in the first place. And I do receive that, but if it progresses too quickly even with that kind of treatment, 1 would want to look at alternatives.

I think that when told that emphysema is a chronic, progressive disease, I can look at why all I want to. The reasons will never tell me if Lung volume reduction surgery or endobronchial valve treatment will work for me specifically. All that those of us with AATD can do is to look at what the benefits might be and go from there to see what is best.

Everyone is different, and if a person is too far advanced in the disease, surgery might be too risky, similar to the risks of a lung transplant. Each situation needs to be addressed individually.

Read more about therapies for AATD

This is the first time I’ve read about something that is encouraging when it comes to lung volume reduction for patients with AATD. I am so glad that there are so many fascinating discoveries being made for new treatments for us. It is truly a godsend.

To be so bold as to challenge these findings is commendable, too. I, personally, still do not like to think about end-stage COPD. It is just not a pleasant thought. Being that I am already having such symptoms in my 30s, like most AATD patients with COPD, I know it may be very hard to cope later in life.

I am fighting for augmentation therapy right now as a patient with the PiSZ genotype. I am hopeful that I can get this one day, and I hope this encourages someone who is facing treatment options or hardship. We must know the risks and advocate for ourselves well.