Category Archives: Have a Heart

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

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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.