Monday, August 26, 2013

Fasting, Cardiovascular Issues and Marfan Syndrome

Quite excited about the four day get back to nature fast I am on. 

Kevin Songer's Marfan Life Challenges
Follow my diet journal here on the blog under the diet tab.  Trying to stick to juiced or raw vegetables and fruits.  After Day One so far so good.

Even with all the meds I felt no additional dizziness, blood pressure or blood sugar issues.  In fact yesterday afternoon I had so much more energy that I spent additional time doing my physical therapy in the pool. 

My blood pressure had dropped about ten points both systolic and diastolic  - and this is OK for me because of my dissected descending aorta.

I will do an in-depth post towards the end of the week, but so far so good!

Wednesday, August 21, 2013

Life Tips, Aortic Dissection & Marfan Syndrome

There is a new 'page' tab on our blog beginning this week, one labeled "Life Tips".
We want to add a new tip daily, sorted into a variety of categories.  Hopefully these Life Tips will help make your life easier, especially if you are challenged with cardiovascular dissection, aneurysms or a connective tissue disorder such as Marfan Syndrome.

Please feel free to submit your one-liner tips you would like to share, and let me know if you wish to have your contact info included.  If you wish to remain anonymous, please indicate your desire to do so.

Here are the first four Marf Life Tips from this week!  Hope they help.
  • LIFE:  Every morning, first thing to say is,  "my number one job today is to stay alive".   Dead, you are no good to anyone.
  • LIFE:  Progress is to me measured incrementally, in small advances.  Over time tiny accomplishments add up to great significance.  Do not expect leaps and bounds forward.  Take one baby step at a time.
  • MEDICATIONS:  Most all of  my prescription medications come from Winn-Dixie Pharmacy.  They have a $4 generic plan that saves me hundreds (potentially thousands) of dollars per month.  Wal-Mart has a similar plan.  Because at this time I do not have insurance, the Winn-Dixie $4 generic prescription plan is a life-saver.  Note:  Your doctor must write the script in one month supply increments to qualify for the $4 generic plan.
  •  SUPPORT:  Join a support group.  Facebook and social networks offer networking to real solutions and other people just like yourself.  This is so important.

Tuesday, August 13, 2013

The Pain House; Chronic Illnesses and Family Life with Marfan Syndrome

As a young child I pledged to myself I'd never talk about how sick I might be.  Listening to older people discuss their ailments was revolting and I'd never be a part of such conversations for many reasons.  Life was not supposed to be about pain.

We are going to start having fun once more - no more 'pain house' #Marfan
Fast forward to the late 1990s.

Judy and I were great outdoor adventure parents to our kids.  Hiking in the deepest of Florida's swamps, our kids stepped on the tail of six foot timber rattlesnakes, ran from charging boars and hunkered down in the canoe as ten foot alligators sailed overhead.  We made sure their home-school science classes were true hands on experiences.

Yes, flying alligators and the story is worth briefly telling.  One day, Judy, I and Jincy and Ruairi were canoeing up in the wild, wild St. Marks National Wildlife Refuge near Live Oak Island, Florida.  Ruairi was two and a half years old, Jincy four at the time.  Wakulla Beach was our launch site and after paddling out in Apalachee Bay for a bit we made our way up Goose Creek several miles.

Goose Creek is pure wilderness with high alluvial banks of sand and oyster shells along the shores, some as high as ten or so feet above the surface of the brackish water. Mullet swim up the creek to the big freshwater headwaters spring.  Some days the spring is filled with millions of mullet, a haven for the big hungry gators living in the adjacent swamp.  With the mullet come the mullet fishermen.

We heard the loud airboat way before the driver saw us and Judy turned to look at me as if to say 'he's going awfully fast'.  Jincy and Ruairi had their life jackets on as always.  Judy and I paddled as hard as we could to bring our canoe up against the bank under a high sand ridge where, unbeknownst to us, a very large alligator was sunning.

If you've ever heard an airboat you know how loud and startling they can be.

As the fisherman spun around the bend in the creek, barely forty feet from us the commotion startled not only us but the big gator just above our heads.

It happened fast but the memories are so clear.  One second the airboat to our left was churning water, the next second a long torpedo shaped creature jumped off the bank above us, sailing inches over our children's heads, splashing into the water adjacent the canoe.

The airboat kept going, the operator not even noticing us.  The alligator swam off, scared to death.  Jincy and Ruairi sat still, knowing their Mom and Dad were always an adventure a minute and maybe this flying alligator was not really that much out of the ordinary.  Fortunately we made it back to the safety of Wakulla Beach later that day.

But something since has changed.

No longer are we the adventuresome parents we used to be.  Sure time has passed.  Jincy and Ruairi are in their late teens today.

Sitting on the lanai yesterday, Judy and I were discussing our chronic illnesses, my dissection and Marfan issues and her IC problems, and she looked over to me.

'This is a pain house', she said.

Her comment threw me for a loop.  And now I fear I've become what I pledged never to be.

Tonight I wondered how dismal, bleak and gloomy the on-going conversation about pain and chronic illnesses must be for our children, Jincy and Ruairi.

What am I doing to our kids?  What are Judy and I doing to ourselves by allowing third and fourth parties, the party of chronic Marfan pain and the party of chronic IC, to be in our life all the time?

Tonight I could not sleep much thinking about our house and how it had become a 'pain house'.

So I am making a new pledge, one this time I am going to try and keep.  Our house is going to be known as a 'loving,  fun and safe home', not a 'pain house'.

For starters I am not going to speak about my chronic pain or debilitation inside the house anymore.  If I want to talk in a gloomy manner then I will go outside to do it.

For me this will be a challenge because I will now have to think about something new to talk about at dinner, in the living room, in the bedroom and on the phone.

I am sure there will be other steps to take along the way but I was different before and I can be different again.

This is not to invalidate our pain.  I acknowledge our very difficult challenges.  Leaving our children with an example of how to make the best of any situation would be a good way to ultimately be remembered.

Wow!  Marfan life is a challenge.  Being a smart parent is even more challenging.

Monday, August 12, 2013

Soap and Floss Can Save An Aorta or Your Life

Things we take for granted, like personal hygiene, can literally save your heart and aorta from inflammation, dissection and help you avoid an Infective Endocarditis (IE) incident such as the one I ended up with.
Personal Hygiene and Marfan Syndrome - very important for heart health
Inflammation can cause vascular damage in anyone, but especially in those of us with connective tissue disorders like Marfan Syndrome.

Skin infections can quickly reach the heart through a cut or scrape.  Once the cardiovascular system becomes infected and inflamed, this condition can produce deadly results.

Infective endocarditis is especially dangerous for those with prosthetic heart devices such as the mechanical aortic valve in my heart and the Dacron graft across my ascending aorta.

One of the first things I remember after waking up from my first open heart surgery was throwing up all over my freshly sutured and sealed chest incisions.  Barfing never felt so good I thought as I lay in the hospital cardiac ICU bed.  Volumes of fluid erupted out of my mouth and across my chest and I did not care.  It felt so good to empty my stomach of all the slosh.

I vaguely remember Judy shouting to the ICU nurses who practically ignored her calls for help.  She ended up cleaning the vomit herself for the most part.  My vision whirled for a second then I passed back into the land of post-operative deep sleep.

Ten days or so later I made it home (to our now Palm Coast home) with my new bionic heart parts and met our home health care nurse, Ted Rhodes, RN who always says RN stands for 'writes notes'.

Within a matter of days Ted worriedly sent Judy and I back to doctor's office in Jacksonville.  My chest was turning a bright red above the heart incision.  The nurse practitioner pressed hard down on my chest sore now swelling in size, prescribed an oral antibiotic and sent me home with instructions to come back if the boo boo worsened.

Have you ever had a Physician's Assistant take a scalpel to your chest without painkiller?  It hurts!  But the PA felt like he had to get the infection out immediately.  So he pushed me back on the table and cut.  Judy was gasping in the corner of the room at the site of her husband (me) being manhandled on the exam table, cut and sliced without anesthesia.  Puss and blood of course was everywhere.

To make a long story short, the entire episode of madness lasted for over four months from a reopening of my sternum, pressure washing the green goop from around my heart and Dacron graft, installation of a picc line for daily IV treatment with some of the nastiest antifungal and antibacterial drugs I've ever had.  There were wound vacs taped to my chest and heparin hypos everywhere. I still worry each time I run a slight temperature.

Bless Judy for the many times she was sprayed with the stuff trying to purge a line or add a bag of solution at one am.  I believe she still suffers collateral damage even today from exposure to the IV liquids.

An array of doctors and lab techs poked, pulled, prodded and took so many samples but they never figured out what the green slime around my heart was.  Everything was negative with respect to bacteria.  Infectious disease doctors finally blamed an unknown fungus. 

I know what it was.  It was an infection and it was trying to eat me.

So I am here to say, pay attention to personal hygiene, use lots of soap and floss.

Even the American Heart Association and other health groups have been emphasizing personal skin and mouth care lately.  As the use of antibiotics has been backed away from as a favorite for many procedures, to the contrary cleanliness has remained at the top of every list for preventing IE.

In fact some medical literature says personal hygiene is very, very important.

Legs, ankles and feet are more difficult to reach and scrub during a shower.  But pay attention to all the appendages.  Clean between toes and under nails.  Use a back scrubber and reach behind those ears.

Our bodies usually have staph and strepto populations growing across our skin.  But a cut or scrape can allow those germs to enter our bloodstream and reach our heart.  The bad guys are especially attracted to prosthetic implanted devices like mechanical valves or Dacron grafts.

Let me tell you from experience, you do not want infective endocarditis.  Besides the high mortality rates associated with IE, recovery will take a year or so out of your life.

An aorta free from inflammation caused by infection may also be less prone to dissect or develop an aneurysm.

So grab the soap and scrub and scrub and scrub.

No more going to bed at the end of a long, hard day without a shower and scrub. Never again.  Brush and floss too.

Your heart and your partner will thank you.

Saturday, August 10, 2013

Marfan Pain. One Simple Cost Effective Solution.

Connective tissue disorders (CTDs) such as Marfan Syndrome, Ehlers Danos Syndrome and others all have one thing in common.  Pain.

Rice Sock Heating Therapy works as well if not better than any heating pad.

Many times the pain is chronic and intense.  Other times the pain may be moderate. But in most cases, torn and ripped connective tissue hurts bad.

Pain killers are certainly one answer, but in my search for sustainable health I would prefer pain relief with less of an impact on my kidneys and liver than pharmaceuticals.

So that is why I am so enthralled with Judy's rice sock, a cost-effective simple pain relief therapy.
Rice Socks are simple and cost effective - all you need is rice and a sock
Over the past week I've experienced really terrible hurt in my ankles, wrists and right shoulder, not to mention a trip to the emergency room with a huge kidney stone(s).  Tonight the cumulative pain had me running for the bathtub and hot water.

Heat helps me deal with the pain.  Heat also moderates inflammation, a very important benefit since inflammation of any type in my body is not good for my compromised cardiovascular system.

A helpful hint here is that if something ever turns into doubling over, unbearable pain and even narcotics don't begin to touch the agony (like my experience tonight), then find the hot!  A scalding bath is first choice for me followed by a heating pad or the rice sock.

Judy's rice sock is so handy because you don't need a tub and a rice sock is less expensive than a heating pad.  Moreover, sometimes when pain strikes in the middle of the night and stores are closed, most people have both rice and a sock (that is all you need).
Rice socks can be wrapped over any painful muscle or joint.

The key to making a great rice sock is finding a thick, long white cotton sock.  I prefer an unused sock but an old sock can work if it has been washed in wonderful scented detergent such as Tide Ocean Mist (beats the smell of WalMart new).

Fill said sock with rice leaving enough room to tie a knot in the sock top.  Any rice will work.  Jasmine or a long grain rice will produce an especially comfortable rice sock.  Once the sock is tied you have one of the most awesome pain relief therapies ever invented.

Though my wife Judy introduced me to the rice sock there are numerous DIY sites across the web.  Google 'Rice Sock' and check out all the amazing testimonies.  I've read of people using the rice sock for pregnancy pain or even to place over the face at night as a warm eye pillow.

With rice sock complete, head to the microwave.  Some people prefer to warm their rice sock in the oven but I like the microwave because it is so quick.  Just don't try and heat the rice sock in a pan of boiling water or you may end up with a huge mushy mess.

I heat our rice sock for two minutes on medium heat.  The rice sock will be HOT when the two minutes are up so be careful to pick up the improvised heating pad by the knot end.  Place the hot sock carefully where your pain is located.  Heat lasts for about twenty minutes or so.

Relief for me is almost immediate.  They are totally reusable too.  Just keep the rice sock dry.

Funny how it is not until my mid-fifties before I find out all these neat and handy life help hints.  Anyway I am so glad Judy made us one.  Hope you find the rice sock helpful for your pain and be sure to pass along the suggestion!

Whoops.  There goes the microwave bell.  Mine is hot.  I think I'll make another so we can have two.  Or maybe three.

Coumadin Lifestyle, Bruising and Bleeding with Marfan Syndrome

Coumadin, is just a dreaded anti-clotting drug that just needs to be understood (yeah, right!).

Bruising and Bleeding with Coumadin
Even though I've been taking Coumadin for twenty one months now there are times when life's normal activities appear to be doable, but wishful illusion quickly fades away with bruises, bleeding and orange skin.

The other day Ruairi and I replaced spark plugs and wires in the car.  Leaning over the motor I braced myself with my right arm, something I'd done many times before during my pre-Coumadin life era.

Three days later I still have a large and quite painful arm bruise, many other small bruises and lots of nicks and cuts scabbing over.  So predictably, I have to periodically remind myself, 'therapeutic Coumadin life has its own set of requirements that must be adhered to or there will be painful consequences'.

I think I can do something I used to be able to do.  Nope.  Not anymore.

So score another for the great drug that keeps me alive, keeps blood clots from forming around my St. Jude mechanical heart valve but also is responsible for all the nasty little
side effects.

And it helps me to go back and re-read this post about Coumadin from time to time because I quickly forget what the drug actually does to keep me alive and of her side affects.  So here is my Coumadin post...


It happened again.  Out of nowhere comes a slow steady dripping, bright red blotches Jackson Pollock style across the open book page, table or as it was with yesterday's event, all over the shiny white bumper of the Lincoln.  The unmistakeable metallic scent of fresh blood fills my nose but as usual there is no pain, no twinge of a prick or sting of a slicing cut.  The ever increasing amount of afternoon sun brilliant, lipstick red blood splatter now has my explicit attention.

Warfarin and St. Jude Aortic Valve #Marfan
After being on warfarin, also known as Coumadin, Jantoven, Marevan, Lawarin and other brand names, for going on a couple years I now know and am familiar with the whole drippy routine.

First task is to find the source of the flowing blood.  Usually the best places to look are the toes, feet, fingers or head, depending upon where one is in the house or yard at the time the red splots start appearing.  Chances are if I am in the kitchen then the blood will be forehead presenting and a sharp cornered cabinet door probably open.  Yet kitchens too are notoriously awful places for the feet if the teens are in a hurry sweeping up glass shards of an accidentally dropped ceramic mug.

However if I am in the yard, I can expect the red alert will be the result of a garden utensil hiding in the grassy paths. 

Blood in the bathroom is primarily indicative of a head wound from the shower door frame and so forth and so on.

The key to this entire blood thinner/anti-coagulant lifestyle is to find the cut or poke quickly, before you loose a quart of life's thirst quencher.  So yesterday when the red blotches started covering the rear bumper of the Lincoln I headed for the bathroom sink, mirror, hydrogen peroxide and band aids. 

A big mirror will usually tell you right away about the blood's origin without an unnerving shriek the teens or my spouse usually emit as I walk by covered in red.

Laughingly I can honestly say, life on anti-coagulants is not really as bad as it sounds.  When I first started taking Coumadin I heard all kind of negative or bad comments like 'OMG!" and 'I can't believe you have to be on that horrible drug the rest of your life!'.  Other words of encouragement included statements like 'my mother's skin turned bright orange!' or 'my Uncle bled out before' and (really heard this one) 'Shit!  That stuff is rat poison!'.  Even a TEDx talk I watched about aortic aneurysms decried the blood anti-coagulant I was taking.

Understandably, Coumadin and I had a not too happy introduction.

But perception can be quickly changed once one acquires hands on experience.

Yes, it is true Coumadin, the anti-coagulant I was and still am taking, is used as rat poison.  However the more I researched this plant-based miracle drug, the less uncomfortable I became.

Don't get me wrong.  I  wish I never had to take any of the many medications I have to take.  But in reality the Coumadin scare was mostly hype.

Warfarin allows my St Jude device to function without clotting

Again, please don't get me wrong.  I am sure some people have bled and coded out from the drug's use, but a quick meal of fresh garden collard greens or broccoli should stop any bleeding as vitamin K is the antidote for Coumadin overdoses.  I am also sure that there are some Coumadin taking bright orange people walking around out there too.

But what was really interesting was finding out why Coumadin is used as a rat poison and the history behind the drug's discovery!

Back at the beginning of the twentieth century there were incidences of cattle dying after being castrated or dehorned, bleeding to death, their bodies unable to develop and implement the normal clotting and healing process.

Researchers at the University of Wisconsin, including Karl Link and clinics suspected that there was something in the cattle's diet was responsible for their inability to clot when wounded and bleeding.

Studies showed that there was a compound in the fermented clover the bulls were ingesting which interfered with prothrombin, the substance responsible for clotting action.  Further tests isolated a coumarin compound called dicoumarol.  Interestingly, though the clover produced the basic coumarins it was the fungi responsible for the moldy clover that actually converted the coumarins into the dicoumarol.

Without a medical use apparent, scientists and industry looked at and began using the dicoumarols as rodent poison.  When the rats ate the drug they bled out.  The substance interferes with vitamin K metabolism and as we know, vitamin K is essential for our blood clotting process.

One of the first human applications was Dwight Eisenhower when he was provided the drug after a heart attack in 1955.

Coumadin's other name, Warfarin, is actually derived from a combination of the words found in the phrase 'Wisconsin Alumni Research Facility' and the word 'coumadin'.

Coumadin or Warfarin is important to me and many others because the anti-clotting effects allow for foreign objects in our body to function with reduced risk for the potential of blood clot formation.  For me this means warfarin allows my St. Jude aortic valve to open and close for many years without sticking due to clot formation (that could be fatal).  Disconcertingly though, data shows that Warfarin use has its risks and mortality due to hemorrhaging does occur.

Yet to date my bleeding has been manageable.

I do wear a bike helmet when cycling and always take a cautious approach when participating in activities with potential for serious trauma, carrying a variety of tape, band aids and antibiotic ointments in my backpack.  Backpack and band aids go where I go, be it for a short walk or trip to the store or even over to a relative's house.

Checking my PT/INR is easy.  INR stands for 'international normal ratio' and PT is 'prothrombin time' and both are used to calculate the body's clotting time efficiency.  Usually I will ask my daughter or wife to take me to the neighborhood medical laboratory, about a mile away for my once a month PT/INR test.  The lab sends the results to my primary care doctor who reviews the results and then either adjusts my dosage or says 'all looks good' and I wait until next month for another test.

I take on average about 5 mg Coumadin each day.

And I've learned to type on the IPad with a band aid or two on my fingers, though this feat is not an easy one to become accustomed to.

Rest assured, I am not super excited about being on anti-coagulants for the rest of my life.  Yet Coumadin is not as bad as I first imagined.

I am super happy the obnoxiously loud St. Jude aortic valve in my chest is still working as designed, and appreciative that Coumadin helps keep the device from clogging up with fibrous clot material.

And the frequent red splots don't surprise me now as much as they once did.  Thank goodness for fermented cover.

Wednesday, August 7, 2013

Anonther Dissection or Kidney Stones? Marfan Syndrome Life

Surviving a large kidney stone's exit from the body can be particularly painful, very similar to a dissection but in a different location. 

Ouch!  Its a kidney stone and not another dissection!
My first experience with a kidney stone happened yesterday while we were visiting my parents in Tallahassee, about a three and one half hour drive from our home in Palm Coast.  Tallahassee is lovely anytime of the year and Granny and Grandaddy's house brings back so many memories to the two teens.

We left Palm Coast early Sunday morning, drove through Jacksonville and arrived in Tallahassee mid-day, making the requisite bathroom stops along the way.   Ironically as kids we'd need to stop all along the way during summer vacation trips.  As our bladders grew to adult sizes those wee-wee pullovers seemed to vanish.  However to our dismay they'd return with a fury once we hit the 'big Five O'.   We began to think of interstate rest stops as oasis on the asphalt ribbon's horizon, shimmering mirage vapors calling to us to pull over.  Thoughts of the previously unimaginable began to appear, frightening hallucinations of adult wee-wee pullovers morphing into adult wee-wee pullups.

Delightfully though, our hotel suite was comfortable and they proprietors even served shelled walnuts and raisins as part of each morning's breakfast.

Fatigue had been my constant nemesis the past several weeks and so the evening of our arrival I gave the blame for my exhaustion and desire to curl up in a fetal position to my dissected descending aorta and weak heart.  I quickly remembered my latest bout with chronic fatigue in South Florida where my headed started spinning and I laid down on the concrete, thinking a new chapter in the whole scheme of things was about to occur.

Every thing seemed OK for a moment as I laid on the bed and I pulled the additional, extra small hotel pillow into my abdomen area.  A good night's rest and the fatigue will go away, I told myself.

Monday morning arrived and the fatigue remained and now accompanied by sharp, constant pain in my lower left gut.  Metamucil was back at home and the characteristically delicious travel food like, pizza, chips and snackaroos had been my weakened resolve's choice along the way to Tallahassee.

Judy always packs water, nuts, dried fruit and those really good for you foods before we leave on a trip.  Funny though how scrumptious colorfully packaged food looks stacked neatly on the gasoline station shelves.

The pain seemed to resolve with the morning's activities and the family spent a wonderful day on the grounds of Walkulla Springs, a Florida State Park.   The once crystal clear waters now stained brown with fingerprints of adjacent housing developments and shopping centers are still a great place for an afternoon swim.

Our two teens, Jincy and Ruairi, love to swim.  Their already dilated aortas probably aren't up to jumping off the high-dive platform into the mysterious waters below but what can you tell a teen, (especially a 'Marf Teen')?  In spite of their limitations, their passion for life is as unquenchable as the river basin's thirst for cool cold spring waters. 
Ruairi, 15 after a jump off the high dive into the deep spring
Jincy, 17 and her cousin Suzie going off the top platform into alligator infested waters below
Hotdogs and jalapeno covered cheesey corn chips beckoned from behind the lodge's quaint little diner and Grandaddy even talked me into a double chocolate mint ice cream cone.

Later, back in the hotel room, we were all beat, dog-tired and ready for a shower and bed.  Judy reached over to kiss me goodnight.  Uncharacteristically I pulled the sheet up over my head and told her to leave me alone.  The chronic fatigue beast had returned.  Lethargy was swallowing me up and all I wanted to do was tighten up into the fetal curl, hidden from the world under hotel sheets.

Sharp pain cut through my lower stomach as morning light streamed between the heavy dark curtains. 

'Absolutely no more junk food for me on this trip', I told myself as I had a zillion times before, 'especially those yummy jalapenos'.

'Ohhhh!' I grabbed by stomach and practically ran out of the room, down the hall to the bathroom downstairs by the hotel's fitness center.

Hotel bathrooms are usually always too small.  They are especially too small when they don't have a vent fan.  I always scope out a nearby hallway bathroom when staying at a motel.  The ones near the lobby always have an exhaust vent fan and are usually much more spacious and with insulated walls where those in the adjacent rooms won't raise their eyebrows in surprise.

Ten minutes later and back on the hotel bed the searing pain I was back in the fetal curl.  Pain was not going away.  This was not a throbbing pain but a deep, continued hurt.  I wondered if my left iliac artery was now dissecting.

Herein lies the horrible curse of living with a connective tissue disorder.  You never know what is happening deep in your body.  The sharp pains can be a strained muscle or they may be your aorta unraveling, death moments away.  Flip a coin.

The constant fear of imminent death gives way to a level of acceptance for some, but the adrenalin is always flowing despite beta-blockers and other medications flowing in the bloodstream.  Comparing notes in a support group is one of the only reliably available tickets to sanity.

I headed back to the bathroom.

Five minutes later and with a toilet full of bright red blood and cramping pain I knew it was time to go head out to the emergency room.  For a brief instant I thought of calling 911 but the hospital was only three miles away and if this was a big dissection or aneurysm I really wanted to be in control of my last few moments.

Judy didn't hesitate when I told her I wanted to go to the ER.  The teens would finish packing for the return trip to Palm Coast and we'd have Grandaddy pick them up.

When Ruairi asked me on the way out if I was OK, time for me froze.  If I told him I was scared that another major dissection was underway, his worry and stress levels would skyrocket.  But neither could I lie and tell him everything was OK.  

After considering all possible answers I blurted out, 'Going as a preventive measure, son'.  Children with Marfan parents have it just as bad as parents with Marfan children when it comes to worry about the other.

Fortunately there were few in the emergency room waiting room and after I explained about my descending dissection, aortic valve and Dacron graft, the nurse quickly hooked me up to the ECG unit and found me a room.

Despite the best accommodations, going to the ER while on vacation is not my first choice of activities.

But I was totally impressed with the professionalism of Tallahassee Memorial Hospital staff, their courtesy and manner with which they conducted their emergency room operations.  One of the first tests doctors want to run on me when I have an incident is a CT Scan with contrast dye.  They usually don't even ask me about the potential for contrast dye damage to my kidneys.  I was amazed when Dr. Draper asked me about my ability to withstand contrast dye.

Renal failure had hit me during previous surgeries, so I asked if he could administer a prophylactic medicine before the dye.  Without skipping a beat he told me he understood and would try the CT scan without the IV contrast dye at first.  The dye would be only used if the radiologist could not read the first non-dye scan.  Instead of IV dye I drank several large cups of a bland tasting watery substance.

First he rolled me over and took a stool sample to rule out blood in my colon.  You have graduated with flying colors with a degree in humility after having to raise your butt high in the air wearing nothing but a floppy hospital gown so the medical professionals can inspect goods along the various body ports of entry.  Pride is a word no longer relevant.  Anyway, when you are in that much pain you don't care what is being done to you as long as the hurt goes away.

As usual, when terrible pain hits, the words 'aneurysm' and 'dissection' roll around and around my mind.  I can't tame this fear.  Despair experience of the unknown hurt from my previous dissection always stays fresh on my heart. 

I don't run to the ER every time a new hurt appears.  If that were the case I'd be in the hospital every day.

But when a deep chronic pain sets in, one obviously not going away and one associated with lots of blood, I know it is OK to be proactive, cautious and seek out medical help.  So what if someone else labels me as hypochondriac.  Staying alive can be a tricky job sometimes.

Arriving in the CT scan room my bladder felt like an over filled water balloon.  After the CT scan I ran to the toilet, my hospital gown flying high in the air behind me.

Relief can be an unappreciated word until one is in the position of finally finding an available loo when when carrying around more sprayable liquid than a fire truck.

But this time it was a mixture of blood clots and bright red blood.  'Oh oh', I thought.  Could my renal arteries, already dissected be pumping blood directly into my kidneys?

I knew the aspirin and Coumadin in my body didn't help the situation any, and in fact had grabbed a handful of fresh spinach leaves before leaving the motel room fro the ER, hoping they'd help with clotting.

'Doc, I am bleeding badly', I said, pitifully upon return to the ER

'That may be a good sign' he said.  'Blood in the urine and the CT scan tells me your problem is not aortic in nature.  You have a really big kidney stone!

'Beam me up, Scotty' was all I thought.  No longer on the edge of death, I was instantly transported back into the realm of the living.  Such is the life of someone with a connective tissue disorder like Marfan Syndrome.

I am so thankful to the staff at Tallahassee Memorial Hospital, the doctors and my family. 

Not only is living a breath away hard on me, it is just as terrifying for my family, especially the teens and my wife and parents.

The stone must have cut its way out during my CT scan wee-wee episode, landing in the comode with all the blood clots.

Discharged and feeling so much more alive, I walked out of the hospital to the car.  Judy drove me to my parent's house where the family who had been expecting an extended stay in the hospital were so surprised when we walked in.

Now I know what the symptoms of a kidney stone include.  Next time I have lots of blood, excruciating pain, fatigue and lethargy I will think kidney stone.  But I will also still probably think aneurysm, or another dissection, or ..........

Such is life with Marfan Syndrome.