Monthly Archives: February 2011

Have a Heart: Me at Various Ages

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A Marfan American Spiritual

The leaky tricuspid’s connected to the right ventricle
The right ventricle’s connected  to the leaky pulmonary valve
The leaky pulmonary valve’s connected to the pulmonary artery

So hear the words of the Lord!

The pulmonary artery’s connected to the lu-ungs
The lungs get the asthma and pneumonia.
The lungs are connected to the pulmonary veins

So hear the words of the Lord!

Them bones them bones are gonna dislocate!
Them bones them bones are gonna dislocate!
Them bones them bones are gonna dislocate!
So hear the words of the Lord!

The pulmonary vein’s connected to the left atrium!
The atrium’s connected to the mitral valve!
The prolapsin mitral valve’s connected to the enlarged left ventricle

So hear the words of the Lord!

Left ventricle’s connected to the St. Jude valve
The St. Jude’s connected to the dacron tube
The dacron tube’s connected to the aortic arch

So hear the words of the Lord!

Them bones them bones they gonna dislocate!
Them bones them bones they gonna dislocate!
Them bones them bones they gonna dislocate!

So hear the words of the Lord!

The aortic arch connected to the tortuous carotid
The carotid’s connected to the left cranial artery
The cranial artery’s got an aneurysm.

So hear the words of the Lord!

The cranial artery’s connected peri-something vein
The perisomething vein is streching too; it’s insane
The artery and vein are pressing on my brain

So hear the words of the Lord!

Them swollen blood vessels gonna pinch a nerve
Them swollen blood vessels gonna pinch a nerve
Them swollen blood vessels gonna pinch a nerve

And that’s called a pin stroke

One more time!

That aortic arch connected to the thoracic aorta
Thoracic aorta’s got an aneurysm
Thoracic aneurysm’s pressing on my spine

So hear the words of the Lord:

Them bulging blood vessels gonna burst one day
Them bulging blood vessels gonna burst one day
Them bulging blood vessels gonna burst one day

You’d better obey the Word of the Lord!

Cool story about a 10 year old boy with Marfan syndrome (originally published 5/9/9)

Great, good, OK, not bad in your case, bad, evil: A Crash Course in Catholic Ethical Thinking

In 2000 years of studying human behavior in the light of the Bible and the Natural Law, and drawing from some of the greatest philosophical minds in history, the Catholic Church has developed some rather nuanced teachings about morality.

Sometimes, when issues get discussed, people have a hard time distinguishing between discussion of principles and application of those principles to individuals. Some people point out that a basic difference between how Catholics think and how Americans think has to do with our different concepts of law. Catholic Canon Law is based upon Roman law. In Catholic law, things have to be written out very precisely (though it’s interesting that the 1917 _Code_ was the first ever codification of Canon Law). No law is “absolute” in the sense that there isn’t an “exception.” Catholic teaching has worked out a very good system of how to understand and apply what, for lack of a better term, we might call “exceptions.”

For some people, these nuanced, complicated systems have given rise to a word: Jesuitical–referring to the process of thinking like a Jesuit, a synonym for casuistry.

In Roman law, the law had to be precisely worded. If a person was brought up on charges of violating the law, the judge would see if the individual’s case applied to the law as worded. If the individual’s case did not apply, that individual was innocent. However, in the Anglo-Saxon common law tradition, if an exception can be found that the wording of the law does not apply for, or if a contradiction can be found between different laws, then that law itself has to be thrown out.

A couple generations ago, American Jesuits and the young Catholics they educated apparently decided they didn’t want to be Jesuitical anymore. Instead, they decided to start applying Anglo-Saxon methods to Catholic teachings, and if they could find an exceptional case where the law seemed unjust, then not only was that exception OK, but the Church teaching itself had to be thrown out (a key example of this is contraception).

Furthermore, in a move to emphasize feelings over Truth, the kinds of distinctions I’m going to talk about became uncomfortable to people because the new kind of Jesuitical thinking didn’t want to hurt anyone’s feelings. It was not enough to acknowledge that a particular exceptional case took away the personal sinfulness of an otherwise evil action, but you could no longer call that action “evil” because it might hurt someone’s feelings.

This mentality, for a few generations, was limited mostly to “progressive” Catholics. One of the things that distinguished “progressives” on the one hand from “conservatives” and “traditionalists” on the other was this mentality: those on the “Left” wanted to emphasize this knew form of interpreting Church teachings, while those on the metaphorical “Right” of the Church reacted against it often to the point of an overly strict absolutism.

In my own case, my rejection of the inherent flaws in the “progressive” movement led me to embrace the more conservative viewpoint. I always hailed conservatives for not compromising their principles, yet, in recent years, certain rifts have begun forming among conservatives, often on these very questions. With the rise of the Internet, and lay apologetics, more Catholics are thinking seriously about day-to-day moral questions we might have previously just dismissed.

When waterboarding became an issue, it became a hot button–not just because of voting, but because it got to fairly every day situations. If “torture” is intrinsically evil, what does that say about soldiers who are otherwise faithful Catholics? What does it say about police who may use “torture” of some sort in interrogation? What does it say about parents using corporal punishment?

If “lying” is always and in every case evil, what does that say about cops? Spies? Parents? Politicians?

Personally, I think part of the problem is that, since _Veritatis Splendor_, there has been a tendency to emphasize “intrinsic evil,” and that intrinsic evil itself has become a political term. The “Right” likes it because they use it to select certain intrinsic evils as what Catholic Answers called “non negotiables” for voting. The “Left” likes it because a) it takes attention away from personal sin and b) “intrinsic evil” can be used to apply a sort of Lutheran approach to Catholic moral teaching: “See! Lying is intrinsically evil! Abortion is intrinsically evil! Bad work conditions are intrinsically evil! See? They’re all evil!”

No, that’s not how it works. Intrinsic evil has nothing to do with the degree of evil involved in an act, but merely that the act may never be good.

One of the most helpful distinctions I ever learned is suggested by early 20th century theologian Karl Adam in his book _Spirit of Catholicism_: theological truth versus psychological truth. He uses this distinction for various complex issues, and anticipates many “Vatican II teachings”. What Adam calls “theological truths” are concrete, objective theological and moral principles. They’re absolute. However, our souls are limited. We cannot live in a world of absolute, so “psychological truth” is the subjective perspective of the individual and how and whether the theological truth applies.

For example, various Church documents of the passed emphasized that a Jew cannot be saved qua being Jewish: in other words, Judaism as a religion has no power to save. This is theological truth. In principle, no one who is literally outside the Church can be saved (extra ecclesia nulla sancta). Psychological truth, however, is that people have various levels of ignorance, and may not understand, or be able to understand, the truth of the Catholic faith. A person may be a sincere Jew and sincerely think the Catholic Church is wrong but try to follow God as best as possible. On the psychological level, this person is following the religion he thinks is most true, most good, and has made a conscious decision to be a Jew–because his knowledge of the Church is imperfect (if he has chosen Judaism because he *knows* the Catholic faith and rejects it, that’s another story). So, psychologically, he’s good.

Now, I’m about as Traditionalist as one can be this side of the Society of St. Pius X (or, really, this side of the Greek Orthodox Church, because that’s the direction I’d go if I were to go schismatic). Yet I am also a huge admirer of C. S. Lewis and T. S. Eliot. I hope both men are in Heaven. I would love to meet them there. In Lewis’s case, I believe his deep set issues about growing up Protestant in Northern Ireland constitute a kind of invincible ignorance where Catholicism is concerned–though I’ve also heard rumors that Lewis, who had a Catholic view of sacraments, received his final sacraments from a Catholic priest. In Eliot’s case, Russell Kirk says Eliot’s rejection of the Church was purely cultural, part of his embracing all things English, and Eliot himself recognized the contradiction. Again, I hope that Eliot had some kind of death bed conversion, but as far as what is publicly known about him, he consciously rejected the Catholic faith for no other reason than personal choice.

All right, so what does this say about issues? Again, we tend to confuse issues with people, and vice versa. If we admire or love someone, we want to overlook that person’s faults. “My husband’s a cop! How dare you suggest that [lying/shooting weapons at people/whatever] is wrong!” “I love John Paul II! How dare you suggest it’s wrong to kiss the Koran or let pagans have sacrifices in Catholic churches!” It’s that feelings thing again. Or it may be the opposite: “You think that it’s OK for cops to lie. You’re a BAD Catholic!” or “John Paul II kissed the Koran! He’s a heretic!” These are both unacceptable extremes that cross from looking at the act objectively to judging the person who commits the act (either judging them to be “bad” or judging them to be “good”).

Jesus says, “Judge not lest ye be judged.” He doesn’t say which way. I love it when I hear someone say, “My son is living with his girlfriend, but we’re not supposed to judge. And, after all, he’s a *good* man. He still goes to church, and he volunteers, and . . . . ” Judging works both ways, as Jane Austen teaches us in _Pride and Prejudice_.

Anyway, all that said, when the Church looks at a particular action, She takes three things into consideration: the act itself, the intent, and the end. All three must be good for an act to be fully good. If an act is good, it may be merely virtuous, or it may be heroically virtuous–going above and beyond the call of duty.

Our Lord establishes the concept of heroic virtue numerous times in the Gospel. For example, in the incident of the rich young man, the man asks what he must do to be saved, and Jesus tells him to keep the Commandments. The man says he does. Jesus replies, “If you wish to be PERFECT, then sell all you have and give it to the poor.” In the Sermon on the Mount, Jesus says that “Unless your righteousness exceeds that of the Scribes and the Pharisees, you shall not enter the kingdom of Heaven.” And he talks about people who will be “greatest” and “least” in the Kingdom–this implies varying degrees of sanctity.

So, what does all this mean? Most people ought to know the difference between mortal and venial sin (though Vatican II parlance likes to say “grave” sin instead of mortal). They are kind of like mirror opposites of virtues and heroic virtues. Mortal sins completely sever the divine life in us. Venial sins, as Mark Shea says, are like “gateway drugs” to mortal sin. Virtues are good acts, but they require no special grace. They are normally in accordance with the Natural Law as commonly understood: boy scout type morality. We all know it’s a good thing to help an old lady across the street if you can. That’s a virtue. If you help thousands of old ladies across the street for no earthly reward, or if you help an old lady across a particularly dangerous street, in spite of fear, that’s heroic virtue.

I like to use the example of St. Gianna Baretta Molla. People are canonized as capital-S Saints because they exemplify heroic virtue–martyrs exemplify it par excellence.

One of the “gray areas” that comes in when we’re dealing with the psychological truth versus theological truth is what’s called “double effect.” My friend Jennifer Fitz recently wrote the best description I’ve ever read of Double Effect. See here:

http://jenniferfitz.wordpress.com/2011/02/23/the-trouble-with-double-effect/

There is a popular misconception that the Catholic Church sometimes allows killing. The Church, technically, never allows killing. While the death penalty is a stretch, every case of “justifiable killing” involves double effect. I recently watched a rerun of _Bones_ where the victim had brittle bone disease, and the killer technically killed him in self-defense. The two men were having a fight–the victim initiated the fight–and the “killer” (who went to prison not for the killing but for covering it up) pushed him in self-defense. The pusher was unaware of the genetic disorder, and when the “victim” fell back after being pushed in self-defense, his head shattered. That’s a *great* example of double effect.

Even in war, the Church’s teaching on Just War is extremely nuanced, compared to how it’s usually treated. Not only must the cause be just (and that’s one whole can of worms), but the way the war is waged must be just. There are weapons and methods of warfare which are intrinsically evil, and one of the Church’s teachings is that, even if the War is just because an assailant is invading another country, once the war has started, both sides have a right to self-defense. In other words, the Nazis may be evil, but the individual Nazi soldiers still retain their human rights. Any legitimate method of warfare must give both sides a fair shake at self-defense or escape.

Double effect means that you’re trying to do something good, or neutral, and something bad happens. If you don’t want the bad thing to happen, then the act is not a sin. However, even if double effect is at work, as soon as you will it, it’s a sin.

As Jen Fitz puts it, you can draw a nice warm bath for your enemy in the hopes that the relaxing bath might make him nicer to you–that would actually be very virtuous to do. However, if you set up the bath so your enemy will slip, or drown or be scalded, or if you even *WISH* for one of those things to happen while your enemy is in the bath, that’s a mortal sin.

If double effect is in play, and the action you’re trying to do is morally neutral, and you don’t intended the bad effect, it’s neutral. If double effect is in play, and the action is virtuous (such as doing something nice for an enemy), that’s virtuous.

In Gianna Molla’s case, many people think she refused an abortion to save her child. This is not exactly the case. The Church teaches that it’s OK for a pregnant woman to have a medical procedure to save her own life so long as the baby is not directly killed. For example, in the very tricky situation of tubal ligation, the baby is killed by double effect: the baby is in the tubes instead of the uterus; the tubes are damaged and need to be removed. The fact that the baby is removed with them is an unintended consequence. However, if the doctor did something to kill the baby *first*, that would be morally evil.

St. Gianna chose not just to reject evil but to reject a procedure where double effect came into play. That is what we call “heroic virtue.” It’s sometimes what people call “scruples” (and then accuse most of the Saints of “scruples). It’s choosing the option that most absolutely conforms to the Gospels and the examples of the saints, the option that is the absolute most virtuous, even if it costs material benefit or one’s own life.

A similar “gray area” or modifier of action is culpability. Like the non-Christian who really doesn’t know Christ or the Church, there are lots of reasons why a person might not make a fully informed decision to sin.

In order for a sin to be mortal, the person must fully choose to engage in the act.

If someone holds a gun to your head and says, “Renounce Jesus,” and you say, “I believe in one God, . . ..,” that’s heroic virtue. If a person holds a gun to your head and says, “Renounce Jesus,” and you say something like, “Whatever you say,” you’re not really desiring to renounce Jesus, but you’re justifiably scared for your life. So it’s not mortally sinful. It may not even be venially sinful, but it never hurts to confess something like that.

On the sin side, lack of culpability may reduce mortal sin to venial or even take away all guilt. On the flip side, if a person overcomes a lack of culpability and does the virtuous thing anyway, that becomes heroic virtue.

So, a person may not be culpable because of coercion, or because of ignorance (i.e., a woman who doesn’t know birth control pills cause abortions isn’t guilty of abortion), or because of psychological disorder or even physiological disorder (i.e., a person in extreme pain does something bad because of the pain). There are lots of reasons people may not be culpable, and it’s important to know those reasons. However, the mentality I addressed at the beginning of this piece suggests that the exceptions should be treated as normative.

Even in the case of contraception, John Paul II taught that couples who use artificial birth control because of extreme health circumstances or financial circumstances may not be fully culpable for their actions–and the greater guilt lies with society for not providing them with the support they need.

That is not to say contraception is “OK”, but merely that the sin involved is not as severe for a couple in extreme circumstances. The Church can have compassion for the person in the extreme without saying “this teaching no longer applies because there is this one extreme case, and the rest of you can go on and do whatever you want.”

When someone’s holding a gun to your head, either literally or metaphorically, and you do something intrinsically evil, you’re not a sinner. If the Nazis are at your door, and you lie out of fear, that’s not heroic virtue, but it’s also not a sin.

Again, intrinsic evil just means that the act is always evil and can never be virtuous in and of itself. It does not mean that it’s as bad as something else or better than something else.

So:

1. Means, motive and end are all good? Act is virtuous.

2. Means, motive and end are good, circumstances are extreme? Act is heroically virtuous.

3. Means are neutral or good. Intentions are good. There are multiple ends, at least one bad and one good? This is “double effect”.

4. Means are bad. Intentions are good. Ends are bad. Circumstances are extreme or person is ignorant? The act is wrong, but culpability is reduced, possibly to nothing.

5. Means are bad. Intentions are bad. Ends are bad. Person is ignorant or not acting in total freedom; and/or matter is not grave? Venial sin.

6. Means are bad. Intentions are bad. Ends are bad. Person acts in full knowledge and freedom. Matter is grave. Mortal sin.

It is really crucial to be clear on these distinctions. To say “X is intrinsically evil” is not the same thing as saying “Y is a sinner for doing X” or even to say that “X is always mortally sinful”. It’s just to say that the using X as a means puts the action under categories 4-6 above.

Fantastic testimony of a man whose wife’s a Marfan

Andrew Sullivan relays a very moving, anonymous testimony from a man whose wife has Marfan syndrome.

I’m glad he doesn’t name which “Specialist” at Hopkins tried to force his wife to have an abortion.

I’m already disgusted enough they’re doing ESCR, but this is a case where remote material cooperation is justified, since they’re the only game in town.

I wasn’t 100% sure where he was going till the end.

I’m also glad that Georgetown came out the good guys in this one.

The tough decision was not at Johns Hopkins, where we ignored (foolishly ignored, you could say) this cold research professional with his certitude that pregnancy = death.  The tough decision was at Georgetown, in the few minutes while we were waiting for the MRI results to confirm the aortic dissection and set the wild night of surgeries into motion.  My wife and I discussed what to do if there were complications during the C-Section and it came down to saving her or saving our newborn son.
 
Our son is now 16, and my wife is fine. So is our daughter, age 21.

New Marfan Syndrome Diagnostic Criteria

The official diagnostic criteria for Marfan syndrome (officially known as the Ghent Nosology) have been changed yet again, but this system seems to be a bit streamlined from the previous versions.

Now, if there are any physicians reading this: DIAGNOSTIC CRITERIA ARE NOT THE SAME AS “SYMPTOMS”!!!

One of the perpetual problems is doctors looking at the *diagnostic criteria*, and then looking at some particular problem and saying, “Marfan doesn’t cause that.”  Well, the point of the diagnostic criteria are the things that are more likely to occur in Marfan.  The old Ghent Nosology was based upon a “Major” and “minor” criteria.

What they used to call “minor criteria” are not called “Systematic Features”.  Some of the old ones have been eliminated.

The new Systematic Features:

1.  Wrist AND thumb sign.  These are poplularly misinterpreted.  Officially, the “thumb sign” is that, with the hand in a fist, the person’s thumb can stick out past the palm of the hand.  The “Wrist Sign” is that, with the fingers wrapped around the arm bones of the wrist (not the joint), the thumb can cover the fingernail of the fifth finger. (3 points)
2. Wrist OR thumb sign (1 point)
3.  Pectus Carinatum (2)
4. Pectus excavatum or chest asymmetry (1)
5. Hindfoot Deformity (2)
6. Plain Flat Foot (2)
7. Pneumothorax (2)
8. Dural Ectasia (2)
9. Protrusio Acetubulae.  Hip ball is too far into the socket. (2)
10.  Increased armspan and leg length and decreased torso–in other words, what Marfan originally described (1)
11. thoracolumbar kyphosis or scoliosis (1)
12. Reduced elbow extension (1)
13. 3 of 5 facial features (1)
15. Skin Striae-stretch marks not caused by the usual reasons (1)
16. Myopia (1)
17. Mitral valve prolapse (1)

According to the new criteria, you need a “systematic score” of at least 7.
For conditions I’m diagnosed with according to the descriptions they give, I’ve got an 8.  Jury’s still out on dural ectasia, or whether I”ve ever actually had a pneumothorax, so those would bring it up to 12.

The other thing that matters is the “Z-Score” on the aorta.  For children, it should be a Z-Score of >= 3, or for adults, >=2. 

The criteria are now based upon how to distinguish Marfan from related disorders.  The new system puts the weight of diagnosis on the aorta and ectopia lentis, with or without family history or positive FBN1 gene test.

Now, the key diagnostic criteria are basically:

Aortic root dilation (with the above Z-Scores) OR dissection
Ectopia Lentis (dislocation of subluxation of the lens)
A positive FBN1 test
A “Systematic Score” (above) of 7 or higher.

A “Systematic Score” of 7 or higher indicates a connective tissue disorder in general, and requires a differential diagnostic to rule out Sprintzen Goldberg, vascular Ehlers-Danlos or Loeys-Dietz.

A family history of Marfan (MFS) and EITHER Ectopia Lentis OR aortic root dilation is sufficient for diagnosis.
A family history of MFS and a System Score of 7–with the above disorders ruled out–is sufficient for diagnosis.

Without Family History:
Aortic Root AND Ectopia Lentis — Definitely Marfan
Aortic Root AND any FBN1 mutation — Definitely Marfan
Ectopia Lentis AND an FBN1 mutation that is known to cause aortic dilation — Definitely Marfan 
OR
Aortic Root, with a System Score of 7 and related disorders ruled out

“You look like you’ve been clawed by a lion” (originally published 6/4/09

Here are some pointers from an article on what to do about stretch marks/stria:

Soothe the skin. There are a number of creams and oils containing various ingredients such as alpha hydroxy acid, cocoa butter, vitamin E and plant extracts that may help get rid of stretch marks. While their efficacy may vary from person to person, and the products are sometimes costly, their use is probably not harmful. Check with your doctor to see if they may be worth trying in your situation.
Medicate. Your doctor may prescribe a topical retinoid cream to help remove stretch marks. Sometimes topical steroids may be used in combination with other creams or ointments.
See a dermatologist. Skin treatments such as laser therapy, microdermabrasion and chemical peels can help, especially if the stretch marks are relatively new.