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.

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“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.