Category Archives: Marfan syndrome

Please help us get a new van.

In 2008, I got my first motorized wheelchair, and we were blessed with an opportunity to buy a twice used 2000 Chevrolet Express 3500 wheelchair van, which was first a prison van and then a medical taxi (I call it our “Paddy Wagon,” since the expression came from stereotypical Irish cops collecting groups of stereotypical Irish drunks in police vans).
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The van has served us well for almost 9 years as our primary vehicle.  “We’ve” had to put some money into it to keep it going, but when it all adds up, it was less than we’d have paid even for a regular van in payments, much less for a handicapped van that can fit our family.

Me and kids at Roper

Me and my kids in 2013, after my aortic graft surgery

Our economic situation simple: we make a little less than enough to get by in modern America.  Unless I should obtain the time and inspiration to write a best-selling book, we strike the lottery or get a really good  investment, we’ll never make much more than we do now.
So when a major expense arises, we need help.
Every few months, something malfunctions in the van.
We had to purchase a second vehicle, using up the small amount of room we had in our budget to add another monthly payment, so we only have to use the van when we need the power chair and so we have a backup when it fails.  The very day we went to pick up the “new” car, the lift stopped working.  When my abdominal aneurysm ruptures or requires surgery, if I survive, I will most likely lose my ability to walk completely.  In the meantime, I need to be able to keep strain off my aorta to delay that surgery as long as possible.
It won’t be long before our eldest daughter has to use a scooter or power chair–technically she already should because she subluxes her ankles every time she walks very far, but we can’t get insurance to pay for one.
As communities, Muslims, Mormons and Evangelicals seem to be very good at rallying around their members.  We Catholics, as a community, need to show the same generosity with ourselves as we do with strangers, to provide the “safety net” that keeps people from falling completely into destitution.  On an individual basis, we have many wonderful Catholic friends who have helped us more than we can ever thank them for.  We know someone out there can afford to help us.
We’re hoping to get a used, 2015 or 2016 Ford Transit Wagon XLT 350, medium height, for around $25,000.  I figure we can modify it ourselves for around $3000-5000.
So accounting for fundraising fees, taxes, etc., we’re trying to raise about $30,000-35000 just for that, though if a generous benefactor wants to help with about $80000 in other expenses we expect to face in the near future, we’d be very grateful.  If someone out there reading this happens to own a car dealership or know a car dealer (I recently heard a rumor that a major Catholic donor in our state owns a dealership), I’m going to be bold and ask if, in the name of Our Lord, you could please donate a van directly?
Please share this post. Please share the link to our fundraiser.  Most of all, please pray that God opens people’s hearts to share, and that He profoundly blesses all those who have helped us.

Please click here to donate.

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.