Category Archives: Marfan syndrome

People understand “cancer”

(John drafted this but never finished it.  His lifelong struggle was getting people to understand that his condition was as terminal as cancer.  It was very hard for me, as his wife, to see that people did not understand this, how delicate his precious earthly life was and every time they beheld my darling husband, they were beholding a wondrous miracle of God.  Humankind cannot bear very much reality.)

They understand “heart disease.”
They understand “diabetes.”
At least as well as they “understand” anything about someone else’s sufferings.
I’ll never “get better.”  I’ll never “recover.”  I have good times and I have bad times, but my “good times” usually lead directly into my “bad times” because my body can’t take it.
I have  a dissected aorta.  If such things can be quantified, articles on pain often mention aortic dissection as the worst possible pain a person can have (though it can also be asymptomatic).
I have 2 aortic grafts, including an artificial valve.
I have a 4.9 cm aneurysm in my gut that will, if it doesn’t burst first, have to be operated on once it hits 6 cm.  Right now, my left kidney and left leg are getting their blood supply. from scar tissue.  The most likely best outcome of my next surgery will be that I lose just one leg or kidney.
In fall 2008, I spent 2 months coughing and taking antibiotics for “pneumonia” and was told I had a nodule on my left lung that looked like a possible tumor, too small to biopsy or operate on yet.  After my dissection in 2011, I found out it wasn’t a nodule or a tumor–it was scar tissue from a lung collapse that wasn’t properly treated–the two months of “pneumonia.”  I spent two months thinking I had some horrible contagious disease and then 2 years after that thinking I had early stage lung cancer (attributed to my frequent CT radiation) because an urgicare doctor didn’t know that hearing no air in my lung (his words) meant it was collapsed and not pneumonia.
My natural lenses sit at the bottoms of my eyes, attached by a few zonules.  If those ever tear completely, my lenses will pose a risk to my retina and have to be removed.  As it is, if I turn or move my head too quickly, or lay flat, my lenses float up, disrupt my vision, disrupt the pressure in my eyes, and give me a headache.
I have, for simplicity’s sake, a brain aneurysm.  It’s in a vein, so they say it’s nonoperable and won’t burst.  But it’s right in one of my motor cortexes, and when it acts up it causes everything from slurred speech and forgetting how to sign my name to migraine to loss of motor control.
I have a tortuous carotid artery.
I *used* to have mitral valve prolapse, but that has apparently been healed by the Lord.
I have a paralyzed vocal cord, the cost of my last aortic graft surgery, which not only limits my speech but my swallowing and breathing.
I have gastroparesis, and eating too much or eating the wrong foods causes unbearable cramping, nausea and occasionally vomiting.
That’s not getting into my chronic joint problems and the side effects of my medicines.

“Hey! I know her!”

The Aiken Community Playhouse Youth Wing is putting on Kate Hamill‘s award winning, 2016, off-Broadway adaptation of Jane Austen’s Sense and SensibilityFrom their website, the showings are:

February 16, 17, 23, 24 at 7:30 . February 18 at 3 pm
School show: Feb. 22 at 10 am. Interested schools should contact the Box Office at 803-648-1438

Now, it should be noted in the interest of both journalistic ethics and apparently federal law, my daughter is in the play and I got to see the dress rehearsal for free because of that.
That said, I really did think it was fantastic, and I strongly recommend it.  I considered trying to do the whole “Snarly Judge who everyone loves to despise” thing, as Mr. Lunt puts it,  and try the whole reverse psychology thing, but I really did like it too much to say anything bad.
One of the marks of professionalism in a performance is the ability to go with the flow when things go wrong, and these kids did great.  I got so wrapped up in it I kept forgetting two things: a) that it was a dress rehearsal (in fact, we previously had free tickets to ACP’s adult dress rehearsal of Beauty and the Beast, and that was so good I forgot it was a dress rehearsal) and b) my daughter was in it.  I barely recognized her.
A basic rule of performance is the ability to go with the flow if something goes wrong.
I kept telling my daughter about William Shatner’s “big break” as Christopher Plummer’s understudy in a Toronto production of Henry V.  On the night when the critics wrote their reviews, Plummer was ill and Shatner took his place.  He kept forgetting his lines, but his pauses to remember them were so dramatically effective the critics loved him.
So these kids were consummate professionals: I noticed a couple times where one of them would stammer, and it fit so well with the story it was either really good acting because they stammered intentionally or really good acting because they actually stammered with good timing.
The only other mistake I noticed was a feedback problem near the ending, and it happened when a character was in angst, so again, it was impressive that the young actors just kept going with the scene.
There were some interesting choices for the incidental music, some of which was at times a bit too anachronistic, but it was emotionally effective nonetheless, and had me listening to the Piano Guyseponymous album on repeat for two hours after the show.
Between the superb acting, the effective dramatization of Austen’s classic, and the music, with the added impact of my daughter’s debut (almost 11 years to the day after her unofficial stage debut as a volunteer audience participant in a dinner theater), I spent the next hour after the show in pure Joy/Sennsucht.

The play was so good that I’d go back even if my daughter wasn’t in it.

As for my daughter, Alexandra Hathaway, in the role of Mrs. Dashwood, she has a tendency to be a bit too fast in her delivery, but in this case it was like the opposite of William Shatner’s King Henry.  They all had to speak quickly to deliver the extensive dialogue, and she came off very effectively as a frazzled and desperate widow.  Like Estelle Getty in Golden Girls, she was one of the younger members of the cast, but a little make-up added to her Marfanoid physique, and her eldest sibling “Little Mother”/babysitting skills made her extremely convincing as a 40 year old mother.

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Alexandra “Mrs. Dashwood,” standing (Photo Credit: Christina Cleveland, Aiken Standard)

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Photo Credit: Me.

10 years ago, she couldn’t see. Now, she has lens implants that give her vision like her mother’s. 10 years ago, her aorta “enlarged.” It stopped growing and even shrank a bit, such that her cardiologist calls her the “miracle girl.” She is in constant pain from ankles that sublux all the time, yet she’s enduring the pain to do this. Whether this is the first step in an illustrious career in theater or valuable experience that will help her in a future path, we are impressed at what she has accomplished, and she deserves accolades and lots of ticket sales, and Aiken Community Playhouse deserves a lot of ticket sales for giving her this well-earned opportunity.

Update on the vehicle issue

Updated 12/11/2018:  John died suddenly after a seizure on October 11, 2018, in the middle of Hurricane Michael.  We are devastated at the loss of our husband and father.  We were never able to get him the new van he so desperately needed, and I ask his intercession to Almighty God for all those who are in need of one.   If you wish to help me (Mary) and the children, the fundraiser link for our living expenses is here:  https://www.givesendgo.com/GHRS#.W8QNumXt9jQ

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Letter to the Postulators for the OFM, Regarding Bl. John Duns Scotusk

To the Office of Postulation for the Causes of Saints,
Order of Friars Minor
Dear Brothers,

First, as a Secular Carmelite, I would like to congratulate you on the canonizations of Sts. Louis and Zelie Martin, who have significance to both our Orders. I don’t know how these proceedings work, but I would like to report a potential miracle for the cause of Bl. John Duns Scotus.

I have had a lifelong battle with the Marfan syndrome, a genetic disorder of the connective tissues. I grew up with a dilated aortic root which went aneurysmal and was replaced-with a St. Jude” valve in June 1996, when I was 19 years old (http://www.discovery.org/a/514). Approximately 10 years later, my descending thoracic aorta began dilating. In October 2008, I suffered a spontaneous pneumothorax. That same year, I was diagnosed with a tortuous carotid artery and a brain aneurysm (it’s complicated, but does not need surgery although it causes some neurological deficits). On January 1, 2011, at approximately 1 AM, I suffered an aortic dissection from the middle of my aortic arch to iliac arteries. The blood flow to my right leg was cut off for 24 hours. The only surgery they did at the time was a “femoral-femoral” bypass.

I have a wife and four children, and, though I have always waivered between accepting my cross and praying for healing, I prayed in early 2011 for guidance on which Blessed to pray to for healing, that he or she might be canonized. I have a BA in philosophy, and I have long been fascinated with the figure of Bl. Scotus, particularly given his defense of the Immaculate Conception. In my own speculations, I had considered Bl. Scotus or one of several Carmelite venerables and beati, or a few others, and after praying about it, I felt Our Lord wanted me to devote my prayers for healing to Bl. Scotus.

Surgery on the descending aorta is a high-risk procedure for anyone. For someone with Marfan syndrome who has already had a previous aortic graft and a dissection, it is especially risky: putting various mortality studies and statistics together told me that I’d have a less than 10% chance of surviving the surgery and not having permanent organ damage or paralysis. I have read many stories of people with Marfan syndrome undergoing post-dissection aorta repairs and ending up in comas, having their lungs fill up with fluid, etc.

I was told in May 2012 that I wouldn’t survive the summer without surgery, but I wanted to wait for the right doctor. I prayed about it. In December 2012, I made my final profession as a secular Carmelite and then found the name of a highly ranked vascular surgeon, Dr. John “Jeb” Hallett at Roper-St. Francis Hospital (a Bon Secours hospital that merged with a Protestant nonprofit hospital) in Charleston, SC, 3 hours from where I live. Dr. Hallett and the then-head of cardiothoracic surgery at Roper, Dr. David Peterseim, performed the surgery on March 27, 2013. On March 20, they performed a bypass of my left subclavian artery to my left carotid to prevent a stroke. I was permitted to come home for my daughter’s Confirmation. Then I went back for the main surgery. Though it had been scheduled for that day for months, the doctors told my family that the widest part of my aorta was larger than 6 cm, and that it was so weak that I should have already had a fatal dissection and likely would have if the surgery had been a few days later.

The surgery went relatively smoothly, but there were complications. I ended up in the hospital for 3 months, spending 3 weeks anaesthetized. I had to have surgery to repair a tear in my thoracic duct, a drainage tube for a chyle leak, a trachyostomy, and insertion of a J-peg feeding tube. I was fed intravenously and by feeding tube for 2 months. My stomach was paralyzed, though some of its function has returned. The only long-term complication was a paralyzed vocal cord. Every day until I was well on the road to recovery, my wife posted a prayer to Bl. John Duns Scotus on her and my Facebook pages, asking people to pray for his intercession.

Almost every doctor I’ve talked to has said my survival and recovery are miraculous. Few surgeons would have put the care and dedication into my survival that the people at Roper did, and most patients in my situation would have had their respirators and feeding tubes pulled. I know as postulators you know what does and does not count as miraculous, but I’ve heard of cases being used in canonizations that were far more medically explicable than my own. While it’s easy to say, “You just had the right doctors,” even finding those doctors at the right time was an answer to prayer.
I hope my story will help to get the champion of the Immaculata the canonization he well deserves.

Scars and Self-image

As I approach the 19th anniversary of my first heart surgery, meaning that I’ve had my artificial valve now for half my life(!), the “me” I think of when I approach a mirror is still the “me” without the scars.   Now, the original “zipper” is less noticeable both from age and from the others that now surround it.  Every now and then, one strikes me.  A few weeks ago, I suddenly “saw”/noticed how big and dark the scar in my side from two years ago is-the one from the drainage tube and the chyle surgery, which healed wrong so Dr. Peterseim cut it back open in the CVICU–I can still feel the sensation of his fingers in my side–removed the infected tissue and partially stitched it back up, but it had to heal with mainly biodegradable gauze.  Every 2 days for my last month and a half in the hospital, a wound care nurse would come in and change my bandages.
They give my wife, the schoolteacher, a crash course in wound care and showed her how to do what a specialized nurse had been doing–but it saved us getting a home care nurse.
There was a lot to reflect on in that wound alone.  Then there was my  feeding tube hole, which required a slightly *different* kind of cleaning and dressing, and my trach hole–all three requiring slightly different treatments, and different healing times. . . .
Tonight, I looked in the mirror, and somehow the scar from my carotid-subclavian bypass, which prepared the way for my arch-abdominal aorta repair, struck my attention just now and inspired this post.  The bypass is kind of cool in and of itself–you can see my pulse in the tube that runs up my collar bone to my neck–but right under it is the scar, slightly curved, more like a fossilized centipede or a lightning bolt in a movie.
I can make an effort to see the beauty and the meaning in my scars–thankfully, my wife doesn’t need an effort to do it–and it’s much easier with the ones from two years ago–but still, after 19 years, I still expect to see my unscarred “self,” and I’m different.   And maybe it’s different when one chooses it, but I don’t understand how anyone can voluntarily do that–whether it’s piercing, or tattoos, plastic surgery or whatever–to make a permanent change that will be there, not just for the rest of this earthly life but possibly in the Resurrection.
While Catechism paragraph 2297 comes into play in these matters, I’m not really thinking in terms of morality here.  I’m more musing on the emotional experience, but that is  seen with a view to the resurrection of the body.  Presumably, the resurrected body will be free of worldly defects, though some of the Saints suggest those “defects” would instead be glorified.

Rare Disease Day #RDD2015 

February seems to be the month of choice for everything-awareness, including Marfan syndrome.  I usually do a whole series of blog posts (or re-posts) in February but didn’t this year.  Meanwhile, I learned February 28 is also #RareDiseaseDay, and there’s a Twitter thing for rare disease awareness.  Click on either of the category tags for this post to see a list of my posts related to #Marfansyndrome or what used to be called the “Have a Heart for Marfan” campaign.

Rare Disease Day #RDD2015 

February seems to be the month of choice for everything-awareness, including Marfan syndrome.  I usually do a whole series of blog posts (or re-posts) in February but didn’t this year.  Meanwhile, I learned February 28 is also #RareDiseaseDay, and there’s a Twitter thing for rare disease awareness.  Click on either of the category tags for this post to see a list of my posts related to #Marfansyndrome or what used to be called the “Have a Heart for Marfan” campaign.

A funny thing happened on the way to Confession . . .

I had made a point of trying to get my family to Confession in the midst of other Saturday plans.
Then I was in bed with chest pain, so we were running late but got there around 4:45. The Confessional was dark; no sign of a priest. I noticed my wife hadn’t followed us in, and we were leaving anyway, when I found her standing by the van, the rear gate still open. There was oily fluid all over the ground, and she showed me how the fluid streamed out when she opened and closed the wheelchair lift:
Hydraulic fluid, leaking out of the pistons.
Great.
We’d “just” had them replaced, four years ago. Seems like just yesterday but forever. That was right after the second engine (for us, third for it) in our handicapped adapted 2000 Chevy Express 3500. Turns out, 4 years is about as long as those pistons last. Now I know, but while we have started budgeting for repairs, we just put a bunch of money into “regular” repairs, and she’s had to pay cash for some graduate courses. We were just starting to work on tightening the budget a bit more to save for emergencies and hopefully a down payment on a house in a year or two. We’d like to finally live in a house big enough for 4 kids sometime before they’re adults.

So, here we are, looking at another repair that could reach into the thousands. We tried Modest Needs 4 or 5 years ago, but that’s “All or Nothing.” This time, we went with GoFundMe, which takes a fee but sends the money as it’s donated.

Since Saturday night when I set it up, we’ve already raised about $470. In the morning, I will take it to a repair place and get a more exact estimate. Please pray, donate or at least share the link.
my GoFundMe Campaign

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“We deserve our punishment”

I know a lot of people  who suffer from chronic pain.  Most of my Marfan friends are non-Catholics, and I observe how very differently they approach the question.  Often, “Offer it up” has become such a cliche that it loses meaning.  Even Jesus cried out from the cross, and sometimes that’s what we have to do, but we must always remember to keep focused on the goal.  I constantly have to remind myself of these things:

1.  “Though He was in the form of God, Jesus did not deem equality with God something to be grasped at, . . .”

2.  “We deserve our punishment, but this Man has done no wrong.”

3.  “In my own body, I fill up what is still lacking in the sufferings of Christ.”

4.  Mother Angelica once asked, “Why me, Lord?”  She got a response: “Why Me?”  She never asked again.

5.  A single mortal sin merits eternal suffering.  The worst we can bear here is nothing compared to that.  Imagine enduring *anything* forever.  My mom’s all-time favorite homily was, “You think it’s hot here?!”

C. S. Lewis once responded to someone who said, “It’s hot as Hell,” with “How would you know?”  When I was in CVICU last year, thinking I was dead and in Gell, everything seemed unendurable because ?I thought it was forever.  I was hot (high grade fever and screwed up post op metabolism).  I was thirsty (living off a feeding tube and npo).  I was in pain.  Most of all, I was *bored.*  I couldn’t move or speak.  I was strapped in a bed with tubes all over my body.

The only way to survive such a situation without despair is the Lord’s grace.  The Voice kept telling me to stop waive ring and make a choice.  It kept telling me it was over: I was in Hell or destined for it, that Jesus would never forgive me.  Yet, I thought of Faust, and I prayed, and I used the seemingly endless monotony to pray.  In particular, I thought about “70 times 7 times,” though I confused it as “70×70” and couldn’t remember if I was supposed to ask or grant it, so I kept naming people in my prayers and asking their forgiveness while offering mine.  I prayed the Pater repeatedly, the Publican’s Prayer and St Dismas’s prayer, over and over, 24/7, for at least 2 or 3 days.  My recovery began.

Are you being Saved?

The person or persons who write “Coffee With Jesus,” the popular webcomic, hit another one out of the ballpark (much like Casting Crowns, they’re pretty Catholic in their thought, even though they insist they’re non-denominational).

I “got saved” almost every week in sixth grade at “Chapel” at Thomas Sumter. Almost every week, some speaker or Christian rock group or something would come with often truly inspiring and sometimes superficial cheesy, “testimonies” (or performances, as the case may be) and finish by saying, “Now, I want you all to bow your heads and give your life to Jesus,” and they would always have us recite the same words in unison, and my thought was always, “And these people would take issue with liturgical prayer. . . .”

Me, circa sixth grade

Almost every week, that is, except the two times my dad, the school’s first Catholic teacher, had his turn (each week a different faculty member would plan the program for Chapel). The first time, he had Fr. Anthony Rigoli, OMI, come, and the second time, he had a panel of students, myself included, speak about treating each other with love and respect (and speaking against bullying). This was inspired by one of his students unwittingly writing a paper about me. She wrote of this thin boy in sixth grade with glasses who was always being picked on and never seemed to notice when everyone laughed at him in the halls (I did), knocked his books out of his hands (I thought I was just clumsy), etc., and yet always seemed happy.

Interestingly, our headmaster once made the same comment.  He passed me in the hall and said, “That’s what I admire about you, John: you’re always smiling.”

I was honored that Mr. Owens, known for his very strict personality, took the time to say that–though at the time I was actually squinting.   This gets to any interesting side note about body language, Asperger syndrome and Marfan syndrome, since a) I have a hard time understanding other people’s expressions, and b) people have a hard time understanding mine.  Even after 14 years, Mary can’t read most of my expressions since they rarely indicate emotion and usually indicate some sort of pain, eye strain, trying to see, etc., though I do try to make a point of smiling.

Anyway, it’s interesting to me how people constantly want to engage in “institutional reform” of things that are just human nature.  As Joe Sobran put it regarding attempts to legislate against “hate,” “some people are just jerks.”  The Reformation supposedly started about “sale of indulgences” and yet many Protestant denominations require their members to tithe.   They criticize Catholics who seem to live a superficial religion but aren’t “Christians,” and yet so many Protestants seem to live the same way.

Salvation is always a process.  The greatest Saints refused to say they had achieved spiritual perfection, even if they had.  The only times in the Bible when Jesus makes definitive statements about people’s salvation are a) to Dismas on the Cross and b) to Zacchaeus at his house, when Zacchaeus promises to give just about everything away (and even then He doesn’t say “You are saved,” just “salvation has come to this house,” which is still an indication of process).

“Work out your salvation in fear and trembling” (Philippians 2:12).

Have a Heart for Marfan Month

You can’t know what it meant to me to see this description ca. 1995 when I first read this.  It is exactly what I used to feel/hear all the time.

<blockquote>“I’ve got a good deal to say,” our prisoner said slowly. “I want to tell you gentlemen all about it.”
“Hadn’t you better reserve that for your trial?” asked the inspector.
“I may never be tried,” he answered. “You needn’t look startled. It isn’t suicide I am thinking of. Are you a doctor?” He turned his fierce dark eyes upon me as he asked this last question.
“Yes, I am,” I answered.
“Then put your hand here,” he said, with a smile, motioning with his manacled wrists towards his chest.
I did so; and became at once conscious of an extraordinary throbbing and commotion which was going on inside. The walls of his chest seemed to thrill and quiver as a frail building would do inside when some powerful engine was at work. In the silence of the room I could hear a dull humming and buzzing noise which proceeded from the same source.
“Why,” I cried, “you have an aortic aneurism!”</blockquote>
–Sir Arthur Conan Doyle, _A Study in Scarlet_, Chapter 6 (emphasis added)

Being Where Terri Was: Part 1–How I Got There

Since late May, I have debated in my mind about what or whether to write about my experiences in April. Like most such autobiographical topics, I’ve fretted about such quibbles as whether to write it here first or save for some formal publication opportunity, whether to go with a fiction or nonfiction approach, how much to share, etc. So I’ve ended up squandering the time I’ve been given to share it by hindering myself, and I decided I’d better just post it here and trust God’s will.

So, here goes: on March 27, as my few regular readers are aware, I had my aorta repaired from the middle of the arch to the middle of the abdomen. I knew going in that it was a risky procedure. I’d spent 2 years reading medical abstracts, studying statistics, talking to surgeons, emailing surgeons, etc., not to mention having spent my entire life *up* to that point studying for the inevitable. For an otherwise “healthy” person, surgery on the descending aorta carries, depending on whose stats you read, something like a 40% chance of mortality within the first two years, and I forget the exact numbers I started with, but when I added them all up, I came up with a 90% chance of some kind of “permanent complication,” be it death, paralysis and/or organ damage. The likelihood of those complications occurring, and the need for surgery ASAP, increased if one was a) A Marfan (check), b) had previous surgery on the aortic root (check), c) had already suffered dissection (check) and/or d) had an aneurysm greater than 6 cm in diameter (check).

Since my dissection in January 2011, I’d been debating whether and where to have surgery. My longtime cardiologist agreed with me that my odds weren’t good and it was better, if I did anything at all, to find someone relatively local who was competent enough rather than do it long-distance. i had already ruled out Johns Hopkins, Emory and UNC, and while I’d been seeing a local surgeon who was competent and confident enough to do it, he needed at least one more team member and told Tme that he literally asked every cardiothoracic and vascular surgeon in the Augusta area, with no one willing to “touch me.” The last time I saw him was in May 2012, when he told me that, recommended I see his mentor at UNC, and told me I wouldn’t live through the end of the summer without surgery (this was based upon CT scans done in November of 2011 and January 2012).

After ruling out UNC, I put the whole thing in God’s Hands. I happened to see a trailer on my Facebook feed about a new movie of _Les Miserables_. I thought, “Oh, not another one,” thinking it was going to be another adaptation of the novel. Then I clicked play, and heard Anne Hathaway’s amazing performance of “I Dreamed a Dream,” and I was ecstatic: THEY MADE A MOVIE OF THE MUSICAL?! I ran to my wife and kids. I called my parents and my in-laws. My wife said, “That’s it: no doctors, no surgery, no activity. Total bed rest for six months. I want you to live long enough to see that movie!” That was the “goal,” and in the mean time we got to go on a Make-a-Wish trip to Florida, and I was able to make my definitive promises as a Secular Carmelite. At my Community’s Day of Recollection, after making my Promises, last December, one of my friends not only knew what I was talking about about when I explained about the surgical options I was considering (and trying to get someone to do, since I was hoping to find someone willing to do a stent operation rather than “traditional” open surgery) but knew one of the top vascular surgeons in the country and had another friend who’d had an iliac aneurysm repaired by that surgeon–I believe he was at Shands in Florida. So he immediately got his cell phone out and called the surgeon’s registration nurse, with whom I spoke for a few minutes that Saturday morning. She gave me a few more names. I started researching.

In early January, having done all that and seen _Les Miserables_ twice, I started looking these names up. I stumbled on the _US News_ list of top surgeons and, since their database of “top vascular surgeons” was nowhere near as long as other categories, I just started scrolling through it after having specifically searched for the other names. Of course, most of the people on the list were from NYC, Boston, Philadelphia, etc. Surprising, few of the names I’d been given were on the list. The one doctor, Dr. Svensson, I believe, at Cleveland Clinic was named by _US News_ as the top cardiac surgeon in the country. I contacted his office and explained how the only reason I was going to travel that far for surgery was for stents because I knew I couldn’t physically handle the travel or financially or emotionally handle the consequences of being stuck 10 hours from home should complications arise. She said, “No way, no how would we ever do stents on a Marfan.” I thanked her for her time.

So, after knocking off everyone else from my list, I finally hit someone within my 3-hour ideal radius that wasn’t Emory: Dr. Jeb Hallett at Roper St. Francis Health Care in Charleston. Plus, he had a little star indicating that he was in the “top 10%.” I Googled his name, and I found this video:

I called his office and scheduled an appointment. When I saw him, it was the most amazing medical appointment I’d ever had. I can honestly say that in all my experience with doctors, now as a patient and a parent, and having known some very excellent physicians, I have never known a doctor quite like Dr. Hallett. He knew everyone whose names I mentioned either as previous surgeons or as surgeons I’d considered. He even agreed with my reasons for ruling out some of the others. He spent a good 2 hours with us. He pulled the CT up on the computer and went over it with us. When his colleague, whose name I can never quite spell correctly for some reason, came for the cardiothoracic consult, Dr. Hallett stayed in the room. They talked right there with us. Our two eldest daughters were with us, and rather than insisting that they leave the room, as we feared, he included them in the conversation.
Afterwards, Mary asked me to talk to the girls about how they felt about it, as Gianna seemed a bit taken aback (as Mary and I both were) by seeing my aorta on the computer screen, and how shockingly huge it was. I said, “What do you girls think?”
Allie said she wanted them to do her surgery when the time comes, and said, “I think it’s a miracle!”
I later told Dr. Hallett on the phone what Allie said, and she said, “She may be right.”

"I am eternally grateful to God for both of these men."  -- my wife

“I am eternally grateful to God for both of these men.” — my wife

ACLU Suing Catholic Hospital

Doctor tries to “force his opinion” regarding abortion on patient. Patient complains. ACLU sues Catholic hospital. Sounds predictable, right?

Not this time.

This time, they’re suing on behalf of the *doctor*.

You see, if a patient goes to a doctor or pharmacy, even one that’s openly Catholic, and demands contraception or abortion, then it’s “The doctor/hospital doesn’t have the right to force their moral views on the patient.”

However, if a patient goes to a Catholic facility expecting it will follow Catholic moral teachings, then it’s “the patient doesn’t have the right to force her moral views on the doctor”

If you want to put your own blood pressure at risk, you can see the typical hate-filled account and commentary at “Reproductive Health Reality Check” (aka, “Reproductive Poisoning Delusion Check”).

What makes this case hit close to home, and the exact kind of situation this blog was created for, is that the patient in question was suspected of having Marfan syndrome. And much like the cases of so many people who’ve been advised to abort their babies for eugenicist purposes only to find out later the babies didn’t have the genetic disorder in question, the woman doesn’t even have Marfan.

So much for “pro-choice.” If a person with same sex attraction disorder wants therapy for that problem, New Jersey’s “Catholic” “Republican” governor has made it a crime to provide that person with such therapy. Now, the ACLU is trying to say that it’s illegal for those of us who put our moral views first in making medical decisions to seek out providers who agree with us.

The unnamed woman had an unspecified “family history” and was sent to the cardiologist by her Ob/Gyn because she got pregnant. If she had been going for an evaluation for school sports, we know darn well she’d be told, “there’s very little risk, go for it,” even though if you go by the pre-1990s statistics, sports are far more dangerous than childbirth (given the mortality rate for untreated women is much higher). If a person *were* diagnosed with Marfan, and chose to play sports anyway, that would be considered “courageous,” but a woman who chooses life is considered “foolish” and “throwing her life away for a blob of tissue” (better than throwing her life away for a blob of rubber).

At least one of the articles thankfully specifies “severe cases may be fatal,” but a “severe case of Marfan syndrome” would have been obvious before she was pregnant, especially if she had a family history and knew to look out for it. Media are about as accurate in reporting on Marfan syndrome as they are about reporting on Catholicism, and the reports on this case illustrate both areas of gaping ignorance. Typically, “Marfan syndrome” is referred to as synonymous with “aortic root aneurysm,” and while that, in conjunction with ectopia lentis, has become the distinguishing characteristic from other connective tissue disorders, if she truly had a “severe case,” with a family history, other signs would have manifested themselves. If she did not have any existing aortic enlargement, there would have been no more risk from childbirth than any other strenuous activity she’d likely engage in.

As for the Catholic hospital side, commentbox feminazis (noting that the definition of “feminazi” is “a person who uses feminism as an excuse to ensure there are as many abortions as possible”) are making all sorts of false claims about “women’s health care,” saying that Catholic hospitals don’t treat ectopic pregnancy, give “emergency contraception,” etc. Treating an ectopic pregnancy is not the same thing as an abortion; the death of the child is a matter of double effect, and in many cases the child is already dead. The Church allows for necessary medical care which may endanger the baby, so long as there is not a direct abortion. It’s why St. Gianna Molla demonstrated heroic virtue; she went above and beyond the call of duty, opting not to have life saving medical care the Church would have permitted. Similarly, while the question of contraception in the case of rape is a matter of debate in Catholic circles, most Catholic ethical guidelines state that “emergency contraception” is permissible within 24 hours of a rape, so long as conception has not yet occurred.

I have never understood, “Don’t get pregnant, or have an abortion, because your child might me killed by your medical treatment,” any more than I’ve ever understood, “Kill your child now so you don’t have to watch him or her die later.”

Also, she went to a cardiologist because she was pregnant and had a family history. This could be taken either way, but anybody with a modicum of experience knows that’s one of the first things the “experts” say about Marfan syndrome: that it can be fatal for pregnant women (I’m not sure what the statistics are, but again, best I can tell it’s no more dangerous than any other strenuous activity one engages in while trying to actually have a “life”).

I’m sure that this woman heard this “advice” already and specifically went to a Catholic hospital to avoid being pressured into an abortion.

Want to go to a doctor for advice on Natural Family Planning? That’s illegal now, because according to the reasoning of the the ACLU, the likes of Chris Christie and the Obama Administration, since contraception is legal, that makes NFP illegal. If it’s illegal to provide “gay conversion therapy” or to provide a 100% pro-life medical practice to people who want it, then should Weight Watchers be illegal? How about vaccinations, regardless of your reason for objecting? “Don’t force your religious views on your doctor.” Don’t want to benefit from embryonic stem cell research, fetal tissue research, etc.? “You can’t put your religious views ahead of your health care.” What about “alternative medicine”? How many of those people who insist on polluting their bodies with birth control pills yet won’t eat at McDonald’s or take antibiotics would like it if people suddenly started suing them and saying, “McDonald’s is legal, so you *must* eat there”?

The hypocrisy of the ACLU and the “pro-choice” euphemism is that liberty is a two-way street. Even if we take a bare modicum standard of “liberty,” setting aside Natural Law, medical ethics, etc., a free market needs to operate both ways.

Part 2: Reflections on The Memorial of St. Wenceslas

I realized that yesterday was the feast of St. Wenceslaus. When my Dad played for daily Mass, the last three days in September were an opportunity to break out some Christmas music: “Good King Wenceslaus,” and then the angel songs for Michaelmas and the Guardian Angels. It’s become a tradition for one of us to call the other on September 28 and for us to sing it together. In 2011 and 2012, I didn’t quite have the energy to sing the duet, but we tried. This year, it didn’t happen. So, here it is:

Good King Wenceslas; click for a Youtube of the Irish Rovers rendition (happened to be my first hit on YouTube, and since my Dad likes them, it fit)

The hardest part of this last 6 months for me (Thursday having been the sixth mensiversary of my surgery–another day that went by in a blur) has been my inability to sing. Not only can I not carry a tune, but I can barely sustain a sentence speaking. I’ve already explained in my previous post why I opted not to get surgery, and even if I got it, I wouldn’t be able to sing.

I cry almost daily about it. I first mentioned it the day I “got my voice back” after my “temporary injection.” I was watching the 2004 _Phantom_ movie with the kids and couldn’t help but burst out with “Angel of Music,” only to croak like Carlotta in “Poor Fool He Makes Me Laugh.” I keep dreaming that suddenly I try and, even though I still can’t talk, I can sing like I did before.

Once in Fifth Grade, my friend’s father followed me to the car leaned down when my Mom rolled down the window, touched me on the shoulder, and said, “I know what happiness is! Teach this kid a new song!”

The late Laurie Beechman (1953-1998), Broadway’s longest-running Grizabella. Click here for an amateur recording of one of her performances. Everything comes to a halt when the audience applauds.

When I was in high school, and my great ambition was to return to St. Jude (now closed) as a teacher or principal, another friend, Jeff, my future best man, would joke that “twenty years from now” (which is now), I’d still be walking down the hall singing Andrew Lloyd Webber shows (all parts, all the way through), come into teacher’s lounge, and Mr. Z would still be sitting there, saying, “John, shut up!” Or the time his dad was preparing a sample interim for a demonstration of how to write them in the “new” gradebook software, and wrote, “John Hathaway is a terrible student. He’s in my Trig/Pre-Calc class. He sings in the halls, falls asleep in class, tells jokes, and has a 110 average.”

Side story: the latest I ever went into the pool was in mid-October (I think the 15th), when their family came over for dinner, and Jeff convinced me to go swimming. The next day, at lunch, a girl who graduated the year before sat at our table. We were talking about swimming the night before, and she said, “You were at his house?” (Our parents were friends through church and Cursillo). “Yeah, his mom and my mom are friends,” he said. “And my dad and his dad,” I replied. “And my dad and him,” Jeff retorted, referring to the amount of time his dad I and would spend talking about computers. A few years later, during my parents’ annual Christmas party, which had a particularly big guest list that year, Jeff went to get a regular cup from the cabinet instead of a disposable. His sister scolded him and said that was impolite: “That’s for family.” “But I’m like family, aren’t I, John?” he replied, and I validated. When Mary met Jeff, he asked her, “Do you like Barry Manilow?” She said, “I don’t know yet.” He said, “Well, you’re going to have to.”

Here Comes the Night (no link)

_Evita_ got me through the Clinton years, and Eva’s poignant prayer at the end of “Waltz for Eva and Che” has always been a catharsis for me: “Oh, what I’d give for a hundred years, but the physical interferes–every day more, O my creator! What is the good of the strongest heart in a body that’s falling apart? . . .”

Back in VA, when I’d see a sign for Dumfries, or Mary would talk about her friend who used to live in Carlisle, PA, or just being on the VRE or Metro, would evoke “Skimbleshanks.”

“They were sleeping all the while I was busy at Carlisle Where I met the stationmaster with elation! They might see me at Dumfries if I summoned the police If there was anything they ought to know about.”

Allie (who, after her most recent growth spurt and personality growth, is starting more to fit her full name, Alexandra) has always preferred Provolone. One time, I bought her some when we had gone to Wal-Mart for one specific reason, and she said, in the car, “Well, are you gonna sing it?” Whenever we’d pick some up at the grocery store, or go to Subway, I’d sing the verse from the Italian mouse in “There Are No Cats in America” from _An American Tail_:

“The Times were harrd in Sic-cily; we hada no provolonay! The Don, he wa-as a tabby wi-ith a taste for my brother Tony! When Mama went to pleada for him, the Don said he would see her. We found her Rosary on the ground. Poor Mama Mia! BUT–“

During our first two years here, when I was doing my gardening, I would see my sunflowers, think of “Like a sunflower, I yearn to turn my face to the dawn,” and start singing “Memory,” or just doing labor which always leads to “Look Down” or “It’s a Hard Knock Life,” and thus everything that follows. I’d sing “Mungojerrie and Rumpleteazer” when I was in a joking mood about Josef & Clara’s mischief and bickering.

In the ICU and rehab, I kept playing songs in my head, like Peter Cetera’s “Glory of Love” or the Four Seasons’ “Working My Way Back to You.”

Now, I just keep thinking of the best song from _Love Never Dies_: “Till I Hear You Sing”

Love Never Dies

Give Kids The World: Ice Cream for Breakfast

Give Kids the World Village is having a special fundraiser/awareness campaign called “Ice Cream for Breakfast.”

Ice Cream For Breakfast is a grass-roots awareness and fundraising campaign inspired by one of the more unique traditions of our thousands of visiting wish families. At Give Kids The World, families are treated to complimentary, all-you-can-eat ice cream in the Village’s Ice Cream Palace from morning until night- even for breakfast!

We hope you will help support the Village by setting a goal, creating a mini-campaign online and asking your friends and family to join you in your efforts. Once you meet your goal we encourage you to host your own ice cream social or just meet your supporters for a scoop at your neighborhood ice cream parlor.

You can read more and either make a donation or start a mini-campaign by clicking here.
Give Kids the World Village is an amazing entity: an all-inclusive, self-contained resort for “Wish families” in Orlando, FL. The resort itself has enough to fill a week, especially for families with disabilities. While we were there last November, we encountered families who ended up spending most of their vacations in the hospital, although we were all blessed with relatively stable help. Families are supposed to come through sponsoring “Wish” programs like Make a Wish, though I’ve read of at least one family that raised their own funds independently. It’s a “once in a lifetime” vacation, and a particular child can only be sponsored once, but families with multiple children with severe illnesses have been known to come back for other children.
In any case, the wish child and immediate family are always welcome to return and visit the resort for “day use,” play miniature golf, ride the carousel, etc., and eat at the restaurants (below) for a nominal fee. The child gets a “star” in the Castle of Miracles:

Allie's Star is somewhere in this "constellation"

Allie’s Star is somewhere in this “constellation”


Many of the volunteers we encountered were relatives of past wish children. They say that the staff is like over 90% volunteers, and they ranged from teenagers to college students to working adults to retirees.
The Make a Wish process is a lot simpler than one might think. The child no longer has to be terminally ill: just a diagnosis of a condition that may likely cause death before age 18. Indeed, I saw on my Facebook news feed the other day an article from Make a Wish about how “Wish children” tend to have better prognoses. We applied for Allie, got a letter from our primary care physician, and within 2 weeks got a letter that she was approved. This was about a year ago. Two local volunteers met with us, talked in detail with me and with Allie about her wishes, her interests, etc., and with me about our family’s overall situation. I explained that while it was Allie’s “wish”, the urgency pertained to my health, and they put us on a fast track to get in for November.
I hadn’t blogged much about it because I’d intended, and still intend, to write a book about our experience, based around selections from the literally thousands of digital photos we took.
Anyway, a week at GKTW includes 3 days at Disney World, 1 day at Sea World and 2 days at Universal/Islands of Adventure. It includes all the Make-a-Wish amenities (like cutting in line and free professional photos), plus things that are specific to GKTW: every morning and evening at the resort has an “event.” The family gets a food card for 3 meals a day at one of the restaurants (the Ginger Bread House, all you can eat buffet, sponsored by Perkins, a sandwich shop sponsored by Boston Market, or an “Express breakfast” at the Ice Cream Palace). Then there’s all you can eat Ice cream from early in the morning till like 11 at night, and the possibility of having pizza delivered to the villa: an option we used one evening just for the sake of trying it, but they feed you so much you don’t need to.
In addition to the “cutting in line” and free photos at the amusement parks, GKTW has characters representing the various parks almost every morning. There is some sort of party almost every evening, and the family gets copies of all photos taken by GKTW photographers, along with options in the going away package to get different albums, posters, etc. We have a nice poster hanging on our wall of our family with “Belle.”
"Tale as old as time . . ."

“Tale as old as time . . .”


The founder of GKTW, is Henri Landwirth, arguably one of the most amazing “unsung heroes” of the world. A Holocaust survivor, Landwirth got his US citizenship and was immediately drafted to the Korean War, later using the GI Bill to get a degree in hotel management. He worked his way up in the hotel field and got into the hotel business in Florida right when NASA and Disney World were getting started. He also made friends with some of the astronauts. Eventually, he started a number of charities, including an astronaut scholarship, a charity to provide clothes for homeless and abused children, and, of course, Give Kids the World. He had been involved with Make-a-Wish since the seventies, serving as one of the main providers of hotels for wish families. When he learned that a little girl passed away before her arrangements could be made, he started GKTW to expedite travel arrangements. Over the years, it grew from free hotel rooms to the villas to the full resort it is today.
We made ample use of the unlimited ice cream, stopping every day right before we left for the parks to get shakes and/or sundaes “to go,” and having “ice cream for breakfast” on our second to last day. However, we didn’t many pictures there.
Our last meal at the Ice Cream Palace

Our last meal at the Ice Cream Palace

Measuring One’s Life in Coffee Spoons: About Spoon Theory

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The “Spoon Theory” is an increasingly popular way for people with chronic health problems to explain how we live our lives.  With no apparent reference to T. S. Eliot intended, it was developed by Christine Miserandino of ButYouDon’tLookSick.com to answer the question, posited by her best friend, of what it was like to have Lupus.  Here is the article in which she tells the story.
She was asked by her best friend to explain what it’s like for her to have lupus–a question she found puzzling given that her friend had been with her through the diagnosis, etc.–until she realized her friend meant experientially. So, after a pause for thought, she grabbed every spoon she could reach. She handed the stack of spoons to her friend and said, “You have lupus.”
She then went on to say that the spoons represented the ability to “get things done.” Most people think of themselves as having limitless “spoons,” but people with chronic ailments are keenly aware of their “spoons”: sometimes there may be more; sometimes there may be less, but when the spoons for the day run out, you’re done. Sometimes, you have to “save up” spoons for a big event or in case of illness. Sometimes, a particularly bad day creates a deficit. Then she went on to explain how it’s not just “jump out of bed and get ready for work”: getting out of bed alone is a huge achievement that costs a spoon or two, then getting breakfast, taking medicine, taking a shower, etc.–what we now refer to in my house as ADL’s–Activities of Daily Living–the key goal in my recent stint in Rehab following my surgery on my descending aorta.
Reading Miserando’s account pretty much describes my experience living with Marfan syndrome, except that for me it’s been a lifelong thing. Even many others with Marfan do not have as severe a manifestation as I do: they were diagnosed as adults and have a lot of problems they’ve encountered in adulthood but weren’t severely effected as children the way I was. Then there’s my aortic root replacement in 1996 which temporarily gave the illusion of “health” (it’s amazing how many health care professionals I’ve encountered in the past few months who are confused by the fact that I’ve had *two* surgeries for aortic aneurysms and/or don’t understand what an “aortic root” is). That was quite a difference from my recent surgery, after which, experientially, I really feel a lot *worse* than I did before it.
People say things like, “Glad to know you’re doing better,” and it’s hard to know what to say. Even the first time around, I was technically “doing better” and just aware of the long term risks I’ve since lived through. This time, I’m not only facing the risks to my remaining “natural” aorta, the two grafts themselves, my valves, and my cerebral aneurysms, but on a day to day basis, I feel worse than I did even in the two years between the dissection and the surgery.
How do you explain that to people who are so optimistic after praying so hard?
When people ask, “How are you doing?” I say, “Let’s put it this way: I’m here.”
My left shoulder was dislocated or frozen or something–I have a week and a half till I see an orthopedic surgeon to find out exactly how badly–but it’s hurt me constantly since April, in a degree that no joint has hurt me before. My left rib cage still hasn’t healed; it constantly hurt and is still swollen.
I have no voice beyond a whisper and occasionally sounding like I’m hoarse, due to a paralyzed vocal cord. A temporary injection that was supposed to last 3 months lasted about a month and a half. It gave me enough sound to hold a conversation, but I still couldn’t sing or speak loudly, and my voice used to be one of my biggest assets (I was a teacher; it was literally my livelihood). Every other person I’ve “talked to” who has Marfan syndrome, had vocal cords paralyzed, and had the repair surgery, said it gave minimal benefit at best. The only person whose seen improvement was a fellow who had his surgery a month before I did, is a paraplegic because of it, and had his vocal cord come back “miraculously” without intervention. So I decided against surgery. Even with surgery, I’m never going to be able to sing or engage in public speaking. The main advantage I’d get is the ability to speak on the phone, for which I don’t have enough “spoons,” anyway.
That’s not getting into the tachycardia, the distinct pain that I know is my aorta–both the throbbing in my remaining arch and abdominal aorta, as well as the “stitch pain” around my grafts–I’m 90% sure there will be at least a few millimeters of growth in both the next time I have a CT scan.
I was very grateful to my attending physician and my physical therapist in Rehab. They actually listened to me, and did their own research on Marfan syndrome. My physical therapist told me that what physical therapists use is the “Borg Rating of Perceived Exertion”, a scale from 6-20, where 6 is “No exertion at all,” and 20 is “Maximal exertion.” 10 is “Light,” and that’s where he told me to stop. It’s a subjective scalle that emphasizes the patient’s perception, but it was nice to have a professional telling me what I already knew, versus pushing me past my limits. In fact, the physical and occupational therapists repeatedly told me that it was nice to have a patient they had to tell to *stop*, since I was so eager to get through my exercises (so I could get home to my family). I saw this in some of the other patients, who would sit there and cuss out their therapists over minimal activities while I was tearing through and saying, “What’s next?”
Most of the time, my sessions would get cut short, and I’d be sent back to bed because my vitals were too high–again, they knew as I already knew that a pulse of 100 is too high for a Marfan, but in ICU they couldn’t get it lower than that.
So I exceeded all the goals they set for me in time for my pre-arranged, insurance-mandated discharge date. Then for the first month, I needed extensive help from my wife and kids for my ADLs. I’ve gotten a bit more independent, but I still have a lot less “spoons” than before, and it’s not likely I’ll get them back.
The thing people don’t understand about a condition like mine is that it doesn’t get better. Even ignoring the aorta, there will always be new problems (like my shoulder) to come along. That doesn’t mean I’m pessimistic or “giving up,” but I just face the facts: it’s what I mean by “36 with a life expectancy of 20.” As I noted earlier, “I’m here.”

Hug a Marf Day!

Apparently, today is “Hug a Marf Day”! If you know someone with Marfan syndrome, give that person a hug!



Hopkins proves link between connective tissue disorders and allergies

This is very big, and very interesting, especially in regards to our family history. A Johns Hopkins study has found that transforming growth factor-beta (TGF-beta), the growth hormone that’s the direct cause of Loeys-Dietz syndrome (LDS) and a partial cause of Marfan syndrome, has been proven to influence immune system development, as well. This explains the link between Marfan/LDS and chronic allergies, as well as a common genetic root to allergies, connective tissue disorders, and autoimmune diseases-varying based upon other genetic factors, environment, etc. (for instance, Marfan and LDS are caused by different genes that influence TGF-beta). While the article doesn’t mention Ehlers-Danlos syndrome (EDS), it is also found to be associated with allergies and food allergies.
The study involved patients with chronic allergies who both did and did not have comorbid Marfan or LDS. All were found to have a defect in one or more of the genes responsible for TGF-beta, though other genes were involved in manifestations of specific conditions.
One of the take homes: Hopkins is now looking into whether the Losartan family of drugs, which have proven effective in Marfan and LDS–may also be effective in treatment of allergies. It also gives credence to anecdotal evidence that EDS sufferers find improvement in their overall symptoms by taking Zyrtec and Zantac.

Facing Death . . . over, and over, and over (originally published 10/17/09)

I’m not talking about some science fiction story here.

Most adults have probably had some “brush with death” in their life by the time they reach their 30s, whether it’s a diagnosis–or possible diagnosis–of a life threatening illness, an accident, or whatever.  Even just contemplating the death of someone we know puts us in touch with our own mortality.

There are several approaches to the idea of impending death:

  • “Eat, drink and be merry, for tomorrow we die” — or anything to that effect
  • Making sure your loved ones know you care
  • Getting very spiritual very fast
  • Despair
  • Working hard to get something accomplished.

What few people have to deal with is the constant awareness that, not only are they mortal, but there’s a relatively high probability they could die today.  And don’t tell me, “You could be hit by a bus.”  I get sick of hearing that response (or words to that effect).

If a person says, “Oh, no!  What if I get hit by a bus?” all the tiem, that’s generally considered being paranoid and/or phobic.

It is not paranoid and/or phobic for a Marfan, especially a post-operative Marfan with a St. Jude valve, a daily dose of Warfarin (aka Rat Poison), a brain aneurysm, and a thoracic aortic aneurysm to think, “What if I die today?”  Especially when one hears of middle-aged Marfans whose aortas dissect simply from the strain of coughing.

Today, I learned of a 16 year old girl who died of Marfan syndrome on October 9.  Her name was Madison Beaudroux.  She told her sister, “I feel like I’m going to pass out.”  She did, and those were her last words.

Every day, there’s some point where I feel like I’m going to pass out, and I often think at those moments, “What if I’m dying?”

Sometimes, I just get in so much pain that I don’t quite “pass out” or fall asleep, but I just kind of hunch over and close my eyes and stay perfectly still.  I think, “What if I die like this?” or, more precisely, “If I had just died, would anyone have noticed?”

Every day, I consider each of those above options to some degree or another.  Usually one or another predominates the others, depending upon my mood, circumstances, etc. 

Every time I get up to do something mildly strenuous, I stop and think, “What if this is the strain that pushes me over the edge?  Will this be worth it?”

What of my duties to this family God has given me?  Is it better to push myself to the limits for them and die or to hold back and be there for them?   Would I not be of more use to them as a saint in Heaven than as a cripple here on earth?
What of my duties to this body God has given me?  Is it merely a mere “coil” to be “shuffled off”?  Is it essentially a burden to be relieved from or a treasure to be protected? How to walk that line?

What of the sins I commit in thought and deed and ommission because of the strain my constant pain and fatigue put on my conscience?  Are the pain and fatigue merely the devil pressuring me to sin?  Will God show me mercy if I can’t get to Confession in time to once again confess the same bad habits and mindsets I fall back into over and over?  What if I’m not detached enough?  What if I’m just excusing myself? 

Have mercy on us, and on the whole world.

Have a Heart: Blues Singer Robert Johnson

Robert Johnson, a legendary blues singer who died before his time, is another of the most credible “historical Marfan” diagnoses.

According to this site, he had long arms, legs and figures; hyperflexibility; lazy eye and “cataract”; and died a sudden death in a manner often described with aortic dissection. 

Another link

Wikipedia