Conversation #35: Catholic Slacker and Alpha Male (March 20, 2018)

We’re late this week—-but spring is here! We had a wonderful walk on Saturday. Grace is reading The PK Cookbook by Sarah Myhill and Craig Robinson.

Paul has a possible explanation for the cough he’s had for months. He wasn’t satisfied with his doctor’s lack of concern in helping him get rid of his chronic cough, so he took matters into his own hands and signed up for a study, and provided a blood sample for genetic testing. He’s learned that he has the PiMZ variation of the SERPINA1 gene. This gene is responsible for the production of the alpha–1 antitrypsin protein. The more severe PiZZ variation results in levels less than 15% of normal and produces severe disease of the lungs and liver. Paul’s PiMZ variation means he doesn’t isn’t considered to have the full-blown alpha–1 antitrypsin deficiency disease, but instead is a “carrier.” This isn’t all that rare: individuals with the MZ gene make up about 2–5% of the population.

With some genetic disorders, being a “carrier” means you can pass on a disease to your offspring, but you don’t “have” the disease. In the case of the SERPINA1 gene, it’s a bit more complicated than that. The PiMZ variation usually results in alpha–1 antitrypsin protein levels of about 60% of normal. That’s not nearly as dangerous as the PiZZ variation, but it is associated with increased risk of some lung and liver diseases, including chronic bronchitis and bronchiectasis. It also seems to be associated with elevated risk of COPD, when the “carrier” has relatives with COPD, which suggests there are may be as-yet-undiscovered comorbid genetic conditions. And it seems that people with the PiMZ variation suffer more lung damage from infections and pollution exposure than people with the normal gene (probably not coincidentally, after we moved from Saginaw to Pittsfield Township, and Paul started commuting daily on I–94, his exposure to air pollution, especially soot from heavy truck traffic, increased dramatically).

Grace should be tested, too, and our children should be tested. If Grace has the normal PiMM variant of SERPINA1, our children can’t have the severe PiZZ variation, although they have a 50% chance of inheriting Paul’s PiMZ variation. If Grace has PiMZ (which seems unlikely, since the disease is mostly found in people of Northern European and Iberian ancestry), then there is a 25% chance that our children could have the much more dangerous PiZZ variation.

This is complicated—-just for starters, there are more than 120 known mutations of the SERPINA1 gene. But it’s a relief to have at least some possible explanation of why Paul’s been sick. And it suggests a course of action—-finding a doctor who specializes in these issues and who can help treat the symptoms. Paul only received his results a few days ago, so we don’t know all that much about it yet.

Then, Grace responds at length to a prompt from a friend, who asked to hear more about her process of formation in both politics and spiritual life. Grace talks in some detail about her early influences. One was her father, a civil-rights attorney, who turned family dinners into seminar classes in politics and criminal justice. She participated in protests at General Dynamics Electric Boat in Groton, Connecticut, and was a veteran volunteer for several political campaigns by the time she was 18. She also talks about her journey from pro-choice to pro-life, and to the Roman Catholic Church, and how she felt that she discovered a spiritual home with Dorothy Day and the Catholic Worker movement.

A minor correction to Paul’s comment: Bill Clinton’s speech on the steps of Rackham Auditorium in downtown Ann Arbor, Michigan was not on election day 1992 (November 3rd), but on October 19th. That was almost 28 years ago—-yikes!

Paul also mentioned the strange Michigan Democratic Primary of 2008. In that primary, Obama, Richardson, Biden, and Edwards withdrew their name from the primary ballot, which meant that it was not possible for Michigan voters to vote for Obama in the Democratic primary. Write-in votes for Obama were not counted. The candidates were protesting Michigan’s decision to move their their state primaries to January 15th, in an attempt to increase the state’s influence in the nominating process, in violation of the national Democratic Party rules. The date change was pushed through by a Republican senate with all Democrats opposed. Clinton left her name on the ballot, breaking her promise not to campaign or participate in Michigan.

How to Listen

You can find the MP3 file here.

The Podcast feed is here.

The Podcast channel on YouTube is here.

More Information

You can order Saray Myhill’s book, The PK Cookbook, here. The author’s own page is here.

Paul is still chewing over his complicated thoughts and feelings about Ava DuVernay’s movie adaptation of A Wrinkle in Time. You can find his extremely long and detailed review notes in his blog, here. (It may have grown even longer by the time you read it).

Paul’s been reading a number of different sources about alpha–1 antitrypsin deficiency, the PiMZ genotype, and related topics. Here are a few:

• “What is Alpha–1?” from the Alpha–1 Foundation

• “Alpha–1 antitrypsin deficiency” from the Genetic and Rare Diseases Information Center

• “The Diagnosis and Management of Alpha–1 Antitrypsin Deficiency in the Adult” (Clinical Practice Guidelines) in the COPD Foundation’s Journal

• “COPD in individuals with the PiMZ alpha–1 antitrypsin genotype” from European Respiratory Review

• “Bronchiectasis” from Wikipedia

• “Why do some adults with PiMZ α1-antitrypsin develop bronchiectasis?” ERJ Open Research

Grace has some recommended links this week:

• The Aims and Means of the Catholic Worker Movement here

• The War Resisters League here

This Week’s Music

The music is from Paul’s song “War Criminal.” There’s a video here and you can download the song here.