Category Archives: Have a Heart

“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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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.

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)

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

I most feel like a parent when the kids are sick

Most of my fondest memories of my parents come from when I was sick-sick, or in the hospital, or the doctor’s office.

I mean, Dad did that “typical father” stuff, like teaching me Shakespeare at 8 and preparing me for the SAT at 9.

Mom and I had great conversations in the car going to school or running errands. And she used to buy writing journals back in PA, and we would sit at Presque Isle State Park or just in our house and write, and sometimes if I was lucky, she’d share her poems with me.

Yet the first thing that comes to mind of my childhood in general, and certainly with my parents, was doctors’ offices, tests, hospitals, or just in bed with a cold or flu. After all, that was the time when it was just me with Mom and/or Dad: nothing and no one else in the way.

I have a collection of “Mary Pictures.” I keep it on my phone, my laptop, and all the desktops. I made a video of it. One of my favorite pictures was taken at my nephew’s birthday when we were engaged, and I was having chestpain, and Mary was still in the “this is all new to me, so I’m gonna panic every time he has pain” stage. I was laying there on the sofa having chest pain.

It’s something that, prior to Mary, I did quite often, but it was always one of my deep emotional pains that my body kept me from participating in so much of the “basics” of life, and I spent so much time alone in the other room. So Mary was sitting there next to me on the couch, holding my hand, and someone took a picture, and I love that picture.

So, as a husband and a father, I feel like it’s my chance to “give back” when someone’s sick. Sadly, especially in the past couple years, I’m not much use to Mary when she’s sick, especially if I’m sick at the same time. But she also might not even know about certain underlying health conditions she has if we weren’t so aware of those matters because of me.

Her POTS, mitral valve prolapse and afibrillation would still be “just laziness” or “just fatigue” if she didn’t have me to confirm, “Yeah, that pain you’re having sounds cardiological. Go to the hospital.” Ironically, just a month before she went to the hospital and found out about her heart condition 2 years ago, I had been laying my head on her chest and thinking how nice it was to hear a “normal” heart beat. I felt like I cursed her.

Allie picked up very quickly on what a bonding experience the doctor’s office is, especially since I presented it to her that way.

She had her first echocardiogram (discounting one when she was a newborn) a month or so before Mary’s first sonogram with Gianna. They let her sit in on the sonogram since she’d already had an echo (for those who don’t know; it’s the same machine; just pointed in a different spot), and she already knew how to behave at a doctor’s office. After watching her sister’s sonograms, it also made her more comfortable with her echo.

She often accompanies me when I take her siblings to the doctor or the hospital and, with one exception, she’s always been a big help, and she’s always impressed the doctors.

Then there’s Gianna, the little nurse. She’s not as adamant about her life plans as she used to be, but we still tell her she’d be a great nurse if that’s what she chooses to do.

I will always remember Allie’s first major stomach bug when she was a one year old, which was I think the first time I took her to the pediatrician for a “sick visit.” I will always remember having to give the pediatrician a detailed description of what was in her diaper–never in a million years thought I’d be able to tackle that task. When she was born, the nurse laughed at me for wearing a mask and gloves while learning how to change a diaper (of course, in addition to the whole “grossed out” thing, I was also extremely paranoid at the time about my valve and infection).

I’ve always said my real “parental boot camp” came when Allie was a little over a year old. We all slept in the same room, and we kept the baby gate up in case she woke up. She knew how to walk, and she had just learned how to take off her own diaper. I woke up to discover that she had already woken up, taken off her diaper, and made a big mess with what was inside it. I had to clean the bed, the bedclothes, the carpet and the kid.

I was also used to being able to call the cavalry in such situations. I called Mary’s aunt. I called her parents. No one answered. When I finally got in touch with someone, it was her sister: “I can’t come over; I’m at the hospital because Mom’s had a heart attack.–But dad says not to tell Mary at school.” So now I had to clean all that stuff up on my own without Allie getting into further trouble *and* get her dressed so we could go to the hospital and find out what was happening with my mother in law.

Somehow, I did it, and I felt like I’d “arrived.”

Then Gianna and Joe came along with their severe food sensitivities. . . . Another permanent memory is the time when Gianna had some kind of accident on Allie’s favorite doll, “Gillian”–which she had received from my sister the previous Christmas. Mary was like, “The doll is ruined. Throw it out!” Allie was crying. I put it in the bathroom, cleaned up the rest of the mess, threw stuff in the wash, then went back to the bathroom and used shampoo, baking soda, Oxy-Clean and elbow grease till Gillian looked like a bright new doll.

Then there was her first stomach bug as a preschooler. I was working my job in Springfield at the time, and I had to go in on Saturday morning. They’d said it was OK to bring your kids on those Saturdays, and Allie had been looking forward to it.

So Friday night, woke up in the middle of the night, throwing up. I slept on the couch; she slept on the recliner. Mary and the other two at the time stayed upstairs. Next day, she went with me to work and mostly hid under my desk. Late in the morning, she was hungry, so I got her some crackers and pretzels from the vending machine, but I insisted she try ginger ale. “I’ve already drank water,” she said. “When did you do that?” I asked. “I got up in the middle of the night and got myself a glass of water, and I held it down OK.” That’s Allie.

Three eye surgeries for Allie. A broken arm for Gianna. Another broken arm for Joe. The common cold, influenza, bronchitis, allergies, etc. Gianna’s gastroenterological tests. Allie’s various physicians. Helping Allie through her “Marfan milestones”: her first knee dislocation, her first chest pain, . . . And the thing she has that I don’t have, that worries her mother and me to death, her pectus excavatum. Every year, they test it and tell us it’s “fine” (meaning they don’t want to operate yet). Yet when you put your arms around the kid, you can feel how frail her little ribs are. She doesn’t like being hugged because it hurts. Sometimes, I lay my hand on her chest, and I feel that indentation so deep I can put my fist through it, and I wonder how there’s any room in her chest at all, and I just want to cry for her.

Then there was the time Mary called me in a panic. Allie had said, matter of factly, as she so often does these things, that she couldn’t see anything in her right eye. I came rushing home, fearing retinal detachment. I did some informal tests. I called her cardiologist and her ophthalmologist. I ruled out retinal detachment, but I got her an emergency visit to the ophthalmologist, and he said the zonules on her right lens were so loose that, while the lens wasn’t dislocated, it wasn’t focusing at all anymore, so it was completely obscuring her vision. It took three surgeries and two years to get that lens out of the way, and by then it was almost too late.

I have a problem where Allie’s care is concerned: I’m the first to want to get whatever the best things for her I can get. Technology has improved a lot in the last 20 years, and I want her to have the opportunities I didn’t for care that didn’t exist then. However, I also know what it’s like to be a “guinea pig,” and I know what it’s like to suffer side effects and complications that are sometimes worse than the problem. And I know what the stress of all this stuff is like, and how all the different things going inside the Marfan body come together to drive one crazy.

Being a Marfan is like being a patient on “House”. They give you a drug for your heart, and the drug, in conjunction with the Marfan, screws up your intestines and lungs. They give you a physical therapy method for your hips, and it makes your knees dislocate. They give you at therapy for your knees, and it makes your back hurt. They give you a therapy for your back, and it makes your hips hurt. . . .

I’m very cautious about giving her cures that may be worse than disease, or putting her through therapies that might give her undue stress, because, on the one hand, I know exactly what she’s going through. On the other, unlike her father, the kid never complains . She only complains when she’s about ready for the hospital, and if she’s not feeling well, the only way she ever shows it is by lashing out.

We used to have a regular thing. We get into the habit and then somehow get out of it. It’s called, “If a kid’s being exceptionally crabby, give ’em some Tylenol.” Allie has headaches all the time, because of her vision, and everything else, and she doesn’t stop to say, “Hey, Mom and Dad. I have a headache.” She just gets crabby and uncooperative. When we’re smart, and we think, “Maybe she has a headache,” we offer her a Tylenol, she accepts it, and after a little while, she’s sweet and cooperative again.

Over the three years we lived in Columbia, I got to know several of the ER nurses, between my own visits, and the fact that I was always the designated parent for taking the kids. And nurses shift duties and hospitals. So, one time we have a nurse in triage who’s the room nurse the next time. Or we’re at a different hospital, and a nurse says, “Don’t I know you from somewhere! Oh, yeah! You’re the Marfan dad!” Or a nurse will say, “How’s Gianna’s arm?” “How’s Allie?”

I bring all this up because Joe fell this evening. It was pretty scary. We had gotten home from running errands. I was getting the grocery bags together. Mary and the kids ran in the house because the kids had to go to the bathroom (they sit all day and go hours on end between potty breaks; as soon as we leave the house, though, they need to go every 10 minutes). For some reason, Joe and Clara decided to run outside and play “Scooby Doo” in the dark. From inside the car, I heard them come running out, saw them run towards the front yard, and was just about to yell at them to go back in the house or get over to the car when
I saw and heard Joe take a bad fall.
He was crying. “Are you all right?” “No!” I came running over, and he was already on his feet. He toddled himself into the house and laid down on the futon. While everyone crowded around him, I unloaded the groceries. Then I knelt down beside him, cleaned his wounds, put on band aids, stroked his head, and talked to him gently. At first, all he said was that his arm hurt all over–and it was the arm he broke last year.

It didn’t look broken, and after talking to him about it, I got him to explain that his hand and wrist hurt, and he had fallen on his hand. I put ice on it for a while, and Mary took him to the ER, and she said they were great, and they said it’s probably not even a sprain but just a bruise.

But there was something special in that time. As terrifying as it was, it was also a special bonding moment for me, to kneel there in the den and tend to my little guy, stroking his head and talking to him. English 101 textbooks like to include an essay that E. B. White wrote about taking his son to the cabin that he used to go to with his father, and how the time seemed to blend and invert.

Something like that happens when I’m tending to my kids in their infirmities, especially the most drastic ones. It’s like I channel my mother. As I comfort my own child, I find myself a child again, being comforted by my Mom.

On “Doing Fine,” “Being OK” and “Feeling Better.”

No, I’m not.

Never have.

Never will.

And “never will” doesn’t mean I don’t believe God may give me a miracle. He has given me many miracles, which is why I’m still alive today. However, I consider everything a miracle. I consider my aortic dissection itself a miracle.

Thank You, Father.

Forgive my ingratitude.

But as for “doing fine”? “Being OK”? “Feeling Better”?

Those are abstract concepts that have more to do with the questioner’s peace of mind than the respondant’s situation.

People want you to tell them you’re “fine” so *they* can stop worrying.

Problem is: the problems are here. Some of them have always been here.

I took a gamble. 14.5 years ago, I had a surgery that I didn’t really want to go through with. I had prayed for many years for God to miraculously intervene and prove His power before the doctors could do anything. I worried that, if I went through with the surgery, I might be denying God’s power to heal me. I just wanted to go to Heaven, though I also knew that I wasn’t spiritually ready yet, but I had to keep up appearances.

After the surgery, they told me I’d “be fine.” They told me I could stop living the way I’d always done and start exercising, and enjoying life a bit.

Actually, after the surgery, the surgeon told my parents that, now that the aorta had been grafted once, I could have a dissection at any time. I had already known this from my reading and paying attention at all those NMF conferences my parents took me to, and it was one of the reasons I didn’t want the surgery.

However, I bought the life of “You’re fine.” I got an apartment on campus for my senior year. I began walking for an hour a day. I really was enjoying it.

Then I developed some tearing around the stitches of my valve, and was back to sedentary.

Then those tears scarred over, and I sought out equilibrium. Still doctors, and my parents, encouraged me to walk, to try things, to not use the wheelchair, etc.

I met Mary.

We had kids.

In those early years of our marriage and parenthood, I was the healthiest I’ve ever been. I still had chest pain and stuff, but everything was remarkably stable, and I had more stamina than I had ever had previously or since.

I even made it to full time employment, and Mary got to be a stay at home mom for over a year.

I pushed myself too hard. Icarus flew too close to the sun.

At first, even before that full time job, I started having really bad headaches, with numbness in my face and legs. There are neurological problems associated with Marfan syndrome, so I went up to Hopkins to see a neurologist and a spine expert. They said my problems were not consistent with dural ectasia, and my tests did not show any evidence of it.

As time went on, and the headaches and spells became more acute, I realized they were TIAs–transitory ischemic attacks. I tried to get checked for those, and had a brain CT which showed nothing. I was told “It’s just stress. Get better sleep.” Eventually, I found out that TIAs which recur daily like I had them were more indicative of a brain aneurysm, but I also knew brain aneurysms were hard to detect. My cardiologist agreed and suggested we start medicating as if I had one.

Throughout this process, in 2006, I also developed a new aneurysm in my thoracic aorta.

When an angiogram in 2009 finally found the brain aneurysm, it also found a tortuous and redundant carotid artery and a “venous ectasia” (kind of like an aneurysm, only in a vein and not an artery). The more I researched these, I figured out my TIAs were actually caused when my BP was high, and blood was trying to force itself through the maze of my right carotid artery (like water through a hose with a kink in it).

And, after about 5 years of being relatively healthy, I had declined a bit more quickly than I’d ever anticipated.

Our prayer had always been for me to find full time work, so Mary could stay home with the kids and we could hire some kind of assistant, whether someone to assist with the housekeeping,the kids, our household management, or our medical concerns.

I worked and worked at various part time jobs to try and make ends meet. I overworked myself. I lost jobs either because of my health or working too many jobs at once or both.

We constantly struggled. We accumulated debt. We accumulated debt because I wasn’t strong enough, especially in a townhouse, to take care of the kids and the housekeeping and cooking and work all these jobs. So we ate out far too much. We accumulated debt because, while our income was sufficient to pay the monthly bills (including any eating out), life is more than monthly bills. Cars needed repaired. Medical bills needed paid. Relatives wanted us to visit. Job interviews would present themselves. Car taxes.

Then I would not get enough classes, or I’d lose a job, or whatever, and our income would drop. Each of the last 3 years, I’ve ended up without any classes in January, and the tax return money–intended to get ahead on our debts–ended up going towards living expenses.

But we kept trusting things would work out. I finally had an interview for a potential full time job. They loved me. They told me I’d start in October. We found a fantastic handicapped one story ranch to rent and moved so I’d be ready for the job. The job didn’t materialize. They froze the position.

So here we were, in a new home that solved a lot of our problems in itself, yet we had several others to deal with.

Financial security had been in our grasp, and we lost it. That was OK, we said, because my current jobs were bringing in enough money. Then I didn’t get assigned as many classes because of restructuring. So I applied for two more adjunct jobs and got them, just before Christmas, one of them on campus.

Then my aorta dissected.

So, how am I doing?
1) My aorta is stabilized, but that doesn’t mean I’m fine. It means I have to do everything I can to avoid stress and avoid raising my blood pressure so my aorta doesn’t dissect again. It means I’m going to be on heavy blood pressure medicines and pain killers. Now, instead of 8 diagnosed potential causes of sudden death, I now have 11.

2) I am giving up driving. That is going to considerably cripple my family, but we’ll get rid of our second car, which will reduce our monthly bills by over $500.

3) I am going to appreciate life a bit more and stop putting off important stuff. I’m going to stop hoping for something better and make do. I’m going to trust God to help us with the bills.

4) I’ve always needed help with the kids, but hopefully, if people don’t buy into “I’m OK,” they’ll be willing to give it. This drastic change in the situation has us considering Catholic school–if we can get some kind of charitable scholarship. Otherwise, I’m going to need volunteer help with the kids, at least a few hours a day. In the meantime, the grandparents are helping out.

5) After we find some ways to seriously cut corners, I’m going to try to keep teaching a minimum number of classes, and I’m going to start applying SSDI. In the meantime, I’m going to apply for South Carolina’s Working Disabled Medicaid program, since my dissection qualifies me for both programs.

6) Sometime in the next 3 years, I will need a replacement of my entire aorta. It may be in 6 months; it may be in 3 years; it may be next week.

7) I’m happy and content and at peace with God and my family. Other than my parental concerns about money and my children’s education and care, I have no worries.

Have a Heart: Unsung Heroes, then and now

Bill Feinstein wasn’t famous.  But he was one of those big heroes within the Marfan community.  He was a regular poster on Marfan List, and a valuable resource.  When I first “met” him online several years ago, he had already had several aortic graft surgeries, and had small dissections in the remainder of his natural aorta.  Bill had been through it all, and was in a situation where further heart/aortic surgery could result in almost guaranteed death or paralysis.  Bill passed away on Sept. 14, 2008, during surgery.

My parents and I knew Wendy Weiss back in the 1980s.  Here’s an article written about her in 1986.   She’s still kicking.  I went to an NMF conference with my parents in 1986 or 87 and heard Wendy speak: “I get so sick of having to get up off the operating table and tell the doctors what to do!”

Marshall Weiner passed away many years ago–sorry to say  I can’t remember when.  I met him at the above conference, where he gave me a free hat.  He was in charge of selling merchandise at a table.  They had buttons with a sign saying, “FREE Take one.”  I assumed that applied to everything on the table, so I grabbed a National Marfan Foundation baseball cap and walked off with it.  My parents scolded me, but Marshall laughed and said the sign was confusing and let me keep it.

Have a Heart: Actor Vincent Schiavelli

Vincent Schiavelli was not only one of our most noted “celebrity Marfans,” but in the Marfan community he’s known for his generosity in reaching out to other Marfans, particularly children, and giving them his time and attention.

May he rest in peace.

This should be the Marfan theme song.

When I was a kid, I suggested that the theme song for kids with genetic disorders should be “Three Blind Mice.”

Seriously, though, Rachmaninoff’s “Rhapsody on the a Theme by Paganini” (Same theme on which Andrew Lloyd Webber’s _Variations_ is based) should be the Marfan theme song: a Marfan writing a variation on a work by another Marfan.

Have a Heart: NMF Statement to SSA, updated

Back in the mid-1990s, the Marfan community was excited that Marfan syndrome finally made it into the Social Security Administration’s official list of disabling conditions.

However, the listing was for untreated aortic dissection. In other words, your aorta dissects, and you’re eligible for Supplemental Security Income (SSI) or Social Security disability for the day or so before you die or have it operated on. OK, there are some people who have dissections that they have to live with for indefinite periods of time, for various reasons.

However, the point is that it’s a very limiting criteria. This is a written statement to the SSA, dated, November 28, 2007, by one Dr. Josephine Grima, representing the National Marfan Foundation, asking the SSA to take into account the systematic effects of Marfan.

The following two sentences sum up my life:

Each major “incident” translates into non-productive years, disrupted or discarded careers, lost wages and assets, and an emotional and psychological disconnect and alienation from society. Couple this with the specter of early death and the resulting angst and pervasive depression that ensues further
debilitates the patient.

I most firmly believe that the worth of a person has nothing to do with how much money he or she can make, or with utilitarian contributions to society. When it comes to my personal life and spiritual life, I am quite happy. “You’re rich in the economy of grace,” as a friend recently said to me.

However, when it comes to survival in this cutthroat world, it’s a different story. I’ve worked hard all my life to always come just short, always not having quite as many qualifications as other candidates for scholarships, awards and jobs, always getting high praise for my intelligence and work ethic but never quite able to advance beyond a basic level job when I do have work, and always getting the riot act when my disability invariably gets in the way of my work.

A year ago, I began working on the _Hide Me In Your Wounds_ CD in the hopes of taking my career into my own hands. Self-marketing has proven challenging as a I have to work so many hours as an adjunct college instructor to make ends meet, and I thought I could have success with the “guerilla”/”buzz” marketing approach through my blog and Facebook.

Please download Hide Me In Your Wounds today.  The more copies I can sell through direct marketing, the more money and time I can spend on advertising, publicity and working on getting some of my other works published.

Have a Heart: Paganini Could do amazing things with the violin because he was a Marfan