Thursday, May 30, 2013

Descending Aortic Dissection, Sonogram and video view of a Marfan Syndrome Leg Aorta

Made it through my periodic sonogram check of the dissected descending aorta in my right leg yesterday at the cardiologist office in St. Augustine.

Technology is so advanced these days and what used to be rather dull, uninteresting paper and pencil diagrams or x-rays are now colorful real-time views of body parts.

Take for example, my dissected descending aorta.  The cardiologist wanted to update the visuals of the dissection down through my leg and look at the vein sufficiency at the same time.  He orders these tests run on a regular basis to make sure the aorta is not in active, uncontrollable aneurysm mode, to ensure there are no clots and keep a check on the status of the torn (dissected) intima layer of the large blood vessel.

Several weeks ago he instructed me to have a CT scan completed on my heart section.  The CT scan is a highly accurate test that provides detailed views of the heart, aorta and other body parts he may be interested in viewing.

However the CT scan did not record images of the aorta passing through my lower abdomen or leg.  So the cardiologist decided to use a sonogram test to view the blood vessels in the lower part of my body.  This sonogram test is generally the same test used that we are all familiar with from visits to the obstetrician when someone is pregnant.  The test uses sound waves to create an image of the body's organs, muscle and tissue.  Also referred to as ultrasound and sometimes echogram, the test can document conditions of the body with relative accuracy.

To run the test, the technologist applies a gel across the area to be viewed and holds the sonogram receiver against the body.  Images are recorded according to the receiver position and angle.
Ultrasound/sonogram test used to check the dissected aorta in my right leg

The tests I had run yesterday included gathering images of my veins, the valves within the veins, my arteries and specifically cross-sectional views of my artery depicting the torn inner layer (dissection).

Interestingly, the ultrasound test gives one a clear view of what the body's blood vessels look like.

My dissected aorta should be a hollow vessel with one open interior diameter.  However the torn inner layer is instead flapping about, having created a false channel similar to a sock, inside the aorta's interior.  This false channel is like a dead end pocket for blood to flow into but offering no way out other than back out through the tear hole into which the blood flowed in the first place as shown in the following video.

In my case the false channel takes up almost half of the aorta's interior flow space, limiting blood flow to my leg and foot. 

One of the reasons my cardiologist wanted to look at the aorta was because my right foot recently started to grow - he refers to the term as swelling - and was wondering if a change to the leg blood flow was occurring.

The radiological technician yesterday did congratulate me on having the 'longest dissection' she had ever seen, and I did take the compliment with pride.  After all, if you have to have a medical issue it is always good I suppose to have a rare condition rather than the standard run-of-the-mill malady.

Anyway,  for those of you who have never seen a cross section of a dissected aorta from someone who is still alive, today is your opportunity to see an example in black and white and living color!

Awareness and education are always critically important and I hope if you haven't taken your blood pressure or seen your cardiologist lately this post may encourage you to do so soon.

In the meantime, the web is full of useful information about the heart, the cardiovascular system, Marfan Syndrome and other connective tissue disorders.  Think Heart Health!

Tuesday, May 28, 2013

Warfarin Lifestyle, Aortic Dissection and a St. Jude Device, #Marfan Syndrome

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.

But if I am in the yard, I can expect the red alert will be the result of a garden utensil hiding in the path.  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 teens or a 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 warfarin 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, warfarin and I had a not too happy introduction.

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

Yes, it is true warfarin, 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 warfarin 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 warfarin overdoses.  I am also sure that there are some warfarin taking bright orange people walking around out there too.

But what was really interesting was finding out why warfarin 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.

The name warfarin is actually derived from a combination of the words found in the phrase 'Wisconsin Alumni Research Facility' and the word 'coumadin'.

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 warfarin 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 warfarin 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 warfarin 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.

Saturday, May 25, 2013

Aortic Dissection and Aneurysm Symptoms, Living with Marfan Syndrome

It happens just too often.  Someone arrives in the hospital emergency room with unusual pain.
Med ID tags can save your life
The inexperienced intern diagnoses the issue as an ischemic event such as a TIA or similar.  The patient's vitals present normal all the time he or she lay on the bed, their aorta, the main blood carrying artery flowing down from their heart through their chest and abdomen into their legs, slowing tearing and leaking like a perforated water balloon.  Then suddenly comes the big rupture and fatal release of blood.

I am not a doctor nor am I a medical researcher.  What I am though gives as much credentials as anyone to talk about what symptoms may occur before a major aortic dissection.  My aorta was slowly aneurysing for decades.  Then in November 2011 it ruptured, tearing out the interior wall, know as the intima, from the root and aortic valve up over the ascending arch, rippling into side arteries, down through my chest into my renal arteries, down my iliac arteries into my leg, ending in my right foot.  One great big tear.

Oh yeah, I felt the entire Type A Debakey tear in slow motion, like someone ripping an old cotton sheet from top to bottom.  Today I live with a wonderful metallic St. Jude aortic valve and a Dacron graft for the ascending aorta.  Unfortunately, my descending aorta is still dissected and is being managed my medications.

Last week my wife and I went to the local emergency room after she had a severe attack of kidney stones.  We wanted to make sure there was no myocardial infarction occurring.  Fortunately her heart and cardiovascular system were OK.  Unfortunately for her though, kidney stones are extremely painful.  But as we waited in the emergency room for the results of the CT scan, the conversation turned to me being a dissection survivor.

The emergency room nurse told us the story of a young man who had come into the emergency room the night before, presenting with symptoms of a stroke so they thought, strange pain in his back, down into his legs.  While in the emergency room with tests in progress, the young man was experiencing an aortic dissection yet no one knew.  Before a CT scan could be run and proper interventory action provided, his aorta ruptured and he died.

This scenario happens way to often, even though aortic aneurysms and dissections are not so common when compared to heart attack or stroke.

I am challenged with Marfan Syndrome, struggling with connective tissue disorders and this was an underlying cause of my dissection.  With Marfan, the connective, structural fibrillin protein does not function properly.  Since blood vessels are built with connective tissue, especially along the interior surfaces, defective connective tissue can weaken the entire cardiovascular structure.

Not all of those reading this article will have issues associated with Marfan, but some may and I hope by sharing those symptoms I encountered and even if one fatal dissection or aneurysm is avoided then it has been worth the time to discuss.

Today I am 56 years old.  I did not know about this connective tissue until I was in my early fifties.  However there were many recognizable events in my life that were pointing to connective tissue issues.

Briefly, though there are different types of connective tissue (CT), the main purpose of CT is to hold our body together.  Cell membranes are a type of connective tissue, as well as ligaments, cartridge, muscles and other tissue that is all interconnected with bones and the structure of the body.  Connective tissue gives the inside of our blood vessels the elasticity and strength needed to withstand the constant pumping and flow of blood through our body.

Connective tissue can be weakened through genetic maladies, such as Marfan and other similar disorders like Ehlers Danlos and connective tissue can be also affected by diet and other environmental factors, such as scurvy where a lack of vitamin C impacts collagen function in our body.  Systemic autoimmune deficiencies may also contribute to or be a part of various connective tissue disorders.  Bottom line is the body begins to fall apart when connective tissue disorders prevail.  This is all bad for our health but particularly dangerous when the connective tissue in our cardiovascular system begins to fall apart.

And so when I was young and my long, skinny ankles and wrists would sprain frequently, that was a sign of a connective tissue issue.

As I grew and required braces, the malformation of my palate was another indicator of potential connective tissue problems.

The inguinal and abdominal hernias I experienced as an infant and then subsequently throughout my life, including multiple repair surgeries, were another indicator of serious connective tissues.

These were all signs that my body was literally tearing apart.  But I did not recognize these as imminently dangerous events, rather they were more of a physical inconvenience.

Then as I aged and entered my forties and fifties my blood pressure, which had been normal all my life began to creep up ever so slightly, plateauing out around 140/85.  I thought nothing of 140/85, probably because the blood pressure was my presently reality at the time and I was functioning like any other normal adult.

Today, if I had a blood pressure of 140/85 I'd be headed to the doctors office or cardiologist's as quick as I could get there.

I feel safe today keeping a blood pressure with my weakened aorta of around 115/60 maximum.

For several years before my dissection I would occasionally feel a quick tearing sensation in my neck that lasted about two or three seconds and afterward which I'd have a three or four minute strong headache that would quickly subside.  I believe these events, all of which would occur while I was involved with strenuous work or exercise, were all small tearing events of the intima, or the interior aortic wall.

Today I would recognize the culmination of all these events as the start of a serious medical problem requiring intervention, but I did not understand what was happening during those earlier years.

The night of the big dissection, I'd been adding to, modifying the rooftop garden over our garage, working on the hot roof, bending and squatting for most of the day.  After showering I sat down and the tear began.

Imagine a slow rip of a long cotton sheet, or the unzipping of the camping tent's front door, or the paper covering a Christmas present being torn off by an excited child.  It felt like a tear, a very painful tear starting in my neck, radiating up through my jaw into my neck and down my back int my legs.

Then came the immense headache.

I laid down on the bed thinking the symptoms would subside but they just got worse.  The pain was not going away.  And so I drove myself to the hospital where the rest is history (you can read more of my surgical adventure here).

Fortunately the ER doctor on duty had a CT Scan run immediately.  I still remember the CT tech exclaiming "wow!  look at the size of his aorta!"

So today I check my blood pressure multiple times each day.  I highly recommend dietary and lifestyle changes be made as soon as possible if one's blood pressure is anywhere near 140/80 (but listen to your doctor first and foremost).  I also wear a med ID necklace noting my medications and condition.
Daily blood pressure monitoring is important for those with connective tissue disorders #Marfan

Sometimes the dissection cannot be detected right away using normal vitals.

Read up on connective tissue disorders.  Understand cardiovascular health and take your blood pressure daily.

The life you save may be your own.

Monday, May 20, 2013

Aortic Dissection and Short Term Memory Loss - Mind Mapping Therapy!

What was I going to write about?
Aortic Dissection & Short Term Memory Loss, Mind MappingTherapy

Yes, short term memory problems haunt me like....uhhhh, I forgot.

Long term memory is sharp as ever.  Just ask me about my childhood!

However, short term memory problems are a writer and lawyer's curse!!!  I mean who wants to read an article where thought continuity is lacking.  And who especially wants a lawyer who can't remember what to say!!!

While the doctors had my heart on by-pass for eight or so hours and my entire body temperature lowered to thirty four degrees there were as the neurologist says embolistic events occurring.  I suspect this means small air bubbles making their way into my brain, or other stroke-like happenings, ischemic or otherwise.

The diagnosis is now 'cognitively challenged'.  Thank God I have Marfan Syndrome because according to the South African Marfan Syndrome Organization scientists, Marf's have above average IQ's.  So I will take that extra IQ and mitigate the short term memory loss, hopefully.

However short term memory loss (STML - hey, the brain is like a computer....) is extremely, extremely, extremely frustrating.  I am sick and tired of people blurting out 'well, I told you...", or 'don't you remember that....",  because no, no, no...I do not remember.

And it gets worse when I am under pressure.  Simple things, like ordering one of those very delicious Publix sub sandwiches that make your mouth water so much you have a hard time swallowing all the drool, makes me stutter and stand in front of the deli worker, speechless when she asks what I want to order.  I should have written the order down.  And it gets worse when she raises her strange looking emo eyebrows with anticipation of my answer while those drooling people standing in line behind me began fidgeting.

Drooling is one of my embolistic event results I struggle with anyway.  But my higher than average IQ has cued me in on ways to hide the quick wipe swipe and find a place to dry my hand without being noticed.

Or when I am on the phone with someone from Craigslist who is selling something that Judy and I know would be perfect for the Florida Urban Permaculture Garden and the seller says, "you know where this or that road is, right?"  Seems like the emphasis is always on 'right?'

No!  I don't remember!  And it is OK I don't remember. It is not my fault my brain doesn't work as smoothly as yours.  But I don't say any of these words.  Usually it is a slow smile that emerges, with the stomach ache inside.

So I try to do mental therapy, like writing, hopefully to help improve my STML.  But word recall is extremely difficult for me now.  Even with my memory challenges, I know that I don't know at least half of the words I used to be able to make appear through my fingertips on the computer screen, even before I knew what to type.

Today with my brain engaged in the non-normal path, the thesaurus is such a great help and my solution to mixing up the words, no pun intended.

But one of the most useful tools I have found in my search for my lost short term memory is something called 'mind-mapping'.  Though not new to the world, mind-mapping is a new concept for me, pulling reason out from the foggy swamp of murky word recall and thrusting idea organization into the world once more full of reason and light!  Ha!

I use MindNode on the IPad to help me organize everything from to do lists of complexity, outlines, journals and other jumbles of thoughts, ideas and words.  LifeHacker has a great post on their top five mind-mapping programs, that I found very informative and helpful.

Mind-mapping is really just a list of ideas put on paper or the computer in an organized fashion for recall at a later time.  The reason I like the IPad apps for mind-mapping is because they allow you to easily add to, adjust, change the order of or otherwise organize your ideas.  The outline for a book would be a perfect example of how mind-mapping could assist anyone, but certainly be of great value to the mentally challenged.

One important tip here.  Do not procrastinate.  Never tell yourself you'll add a great idea to the MindMap later.  Add the idea while the thought is fresh and alive.  How many times have I run to get a glass of water or do something really quick only to return to the MindMap just to forget what my great idea was.

I am disabled.  There is no other way around that acknowledgment.  My word recall sucks.  I slur my sentences from time to time, some days being worse than others.  Maybe it is the beta-blockers or the other med myriad pills or something biological in my brain.

But I'll take anything that can help me cope with the short term memory loss.

Mind-mapping is an awesome tool you should check out!

Saturday, May 18, 2013

Connective Tissue Disorders, How Aortic Dissection Happens #Marfan Syndrome

What is a connective tissue disorder and how does it affect persons? Connective tissue is made up of structural proteins, themselves long chains of amino acids. Connective tissue holds everything together in your body. When connective tissue fails body systems begin falling apart.

Think of a concrete block building that over time looses all of its mortar holding the blocks together. What would happen? The structure would collapse. Similarly, think of a beautiful suit of clothes you are wearing. Now imagine all the sewing thread holding the cloth pieces together loosing it's strength and elasticity. Yikes! We'd be standing there in our birthday suit. Finally visualize an airplane flying high in the sky and unfortunately the metal rivets holding the plan's exterior metal panels begin to disintegrate. Think of what would happen if the rivet material was defective, allowing vibrations to break apart the tiny fasteners.

Now envision your body. You have special bioglue, biofasteners, biothreads so to speak throughout your body, holding your organs in place, supporting your bones, keeping your eyes in their sockets and your backbone straight. Otherwise we'd be a blob. But even then blobs need connective tissue to hold their blob together in some form or fashion (think jellyfish).

There are many protein based compounds that form connective tissues and there are many structural systems your body has in place to hold everything together.

Some of us have a issue with one of the very important connective bio-fastener substances in our body, the inadequacy and insufficiency of a special protein call fibrillin. Fibrillin is a very unique protein referred to as a glycoprotein and provides the glue and fastener effect for much of our body.

Some of the very important roles fibrillin plays includes holding together the cellular layers of our blood vessels, the layers of support for our spinal cord, holding up the arch in our feet, keeping our retina attached inside the eyes, facilitating the long term assembly of ligaments, bones and muscles and so much more. Fibrillin is like a bio-super glue matrix.

Sometimes, due to a gene mutation, fibrillin isn't produced in quantities or qualities our body needs for long term sustainability. Generally speaking there are seven different forms of fibrillin. Generally speaking again, the fibrillin associated with many symptoms characterized by the French pediatrician, Dr. Antoine Marfan in the late 1800's, is referred to as fibrillin-1. Diagnosis of Marfan Syndrome is usually completed by geneticists who examine the fibrillin-1 gene. This gene is typically called FBN-1. So when you hear FBN-1 (that pseudo-acronym sounds so unnecessarily complicated to me) then you now know the term refers to a bio-fastener gene. Simply said, FBN-1 problems means lack of bio-fastener/glue in the body.

I like to compare the fibrillin-1 deficiency to a stack of cards and my aorta. This is very easy to see and the example really helped me understand why my aorta tore apart, all the way from my heart to down through my chest and abdomen into my foot.

First imagine a stack of 52 cards and every three cards are held together with a couple thin rubber bands. Then stack all the cards banded in groups of three on top of each other and wrap the first six stacks of three cards with another couple thin rubber bands and repeat for two more stacks of six-three stacks. Then take all the stacks and place neatly on top of each other and wrap them all together with several thin rubber bands. You have a stack of cards bound tightly together but quite flexible at the same time!

This is what a normal aorta or blood vessel, or heart wall, of spinal cord support system or eye tissue layer is like generally speaking. Think of the rubber bands as the fibrillin in your body, holding everything together.

Now in my aorta only a few rubber bands exist and that is why my aorta is unraveling. Think of that same deck of 52 cards with only a few rubber bands holding the entire stack together. Now think of a stress, like a hard physical activity or high blood pressure (hypertension) in the body - or two people each one pulling on a separate end of the card deck. At some point the few rubber bands break and the entire deck of slick cards flies everywhere. Ooops!

Exactly what I though when my aorta tore (ooops! - yeah right...).

Connective tissue disorders (CTDs) like Marfan Syndrome (fibrillin issues), Ehlers-Danlos Syndrome (collagen issues) and several others present themselves in many ways. Most are very painful and the mortality risk can be extremely high. Some manifestations are a genetic mutation and others are autoimmune type responses, similar to diabetes. But they are all part of the same integrated and non-isolatable problem, that of our body literally falling apart.

Unfortunately, CTDs are just now beginning to find a place in the research community, and though there are several excellent specialized clinics such as the Cleveland Clinic, much more work needs to be done. A recent study showed that those clinics with documented experience in treating CTDs have so much more success, particularly when considering mortality frequencies. This is because CTD disorders are complicated and unique, requiring precise medical treatment and prompt recognition. For instance, mortality rates jump within hours of a Type A dissection, as I experienced. If a dissection is not diagnosed immediately, death may soon follow.

But there are also many lifestyle changes we all can make, that though are designed to help persons suffering from CTDs, can also produce longevity and a quality of life for all.

We will be discussing how diet, exercise and spirituality can help all of us beat CTDs and best of all, live a long and happy life regardless of the infirmity we are faced with. Pain is pain and some hurt so badly all the time. But if there is even one small action we can take to ease that pain, we have succeeded in improving our lot, if even just a bit.

The next few blog posts will focus on what we personally can do to intervene with the CTD issue, hopefully making our daily life activities a little or possibly a lot easier to cope with.

Now, at least I hope the mystery of what a connective tissue disorder, such as Marfan, is unraveled and the cards shown.

Friday, May 17, 2013

Is My Aorta Still Dilating? #Marfan #Aorta Dissection

How many times during the day do I ask myself this very question?
Periodic Iodine Dye Contrast Test in the General Electric CT Unit checks out the progression of my aorta's dilation and dissection
Flagler Hospital's GE Lightspeed CT Scan Unit

Everytime I reach to hold an object or lift something, my mind shifts to the wondering and questioning mode. Whenever I strain, holding by breath, even for a second I wonder if the now very thin walled vessel will hold or pop. Looking at my teens and my wife the same question flashes through my mind. What is in store for my aorta?

Even watching the few television episodes I waste my time on, the rupture of an aorta is always lurking somewhere in the background. Such was the case when we were watching the season finale of Revenge and the character Dechlan died of an aorta rupture. Another character, Nolan (explaining through tears to Dechlan's brother Jack) referred to the event as a rupture of the 'aorta artery', leading me to believe the script writers had to include the term 'artery' because most viewers would have never asked if their aorta was dilating or unhealthy, and for that matter even know the aorta was an artery, let alone the largest artery in our body.

So every so often my cardiologist also begins to wonder what is happening in my thoracic cavity (chest) and orders a CT Scan event, complete with iodine for better contrast.

I really like Flagler Hospital in St. Augustine. The stormwater pond is a marvelous example of as to how truly green infrastructure should be designed. Birds from around the world come to Flagler Hospital, not of course for the medical expertise but because the big stormwater pond has plenty of shallow water roosts where the two wings can perch, poop and wait for a fish to swim by. I see roseate spoonbills, all types of herons, the goofy anhingas, ducks galore and more. Moreover, the hospital boasts some of the loveliest living walls around, flowering vines releasing their lovely fragrance that unfortunately has to mix with the exhaust of the laundry vents.

Flagler's staff are always so welcoming and friendly (and cute too!). Most are experts at cracking the lamest jokes in an attempt to turn my dour smirk into a smile.

Last time the inside of my elbows looked like a chickenpox battlefield after the nurse couldn't find the vein. Finally they had to get someone else to try. This time the nurse got the IV attached effortlessly and without any pain, making me very happy and keeping my blood pressure at reasonable levels.
Being a Marf is always a challenge for many reasons. The first reason is always having to explain just what a 'Marf' is, the whole connective tissue disorder and aortic dissection thing. I always know at what point in the story their eyes widen to the size of a raccoon's and the words, 'wow, you are so lucky to be alive!' are uttered.

After determining my kidneys are borderline strong enough to handle the contrast dye they tell me about what the IV's iodine rush will like. One never forgets the warm flush as the iodine flows from the arm into the heart down the aorta into the abdomen area, very similar to the wonderful feeling of finally finding a toilet a couple hours after eating a big plate of chopped, steamed okra. I always manage to hold it in, but notice the retractable bed is covered in plastic causing me pause to think of others not so disciplined.

The 'nitro' pill is like afib in a bottle. Because my pulse usually stays around the low 40s, the CT techs always administer nitroglycerin either before or during the test. This time they used a spray, telling me to lift my tongue before they coated the inside of my mouth with a fine mist that makes your heart stand up, salute and sing the Star Spangled Banner.

GE should use a larger font for the 'Do Not Stare at the Laser' warning sign placed adjacent the unit's laser opening. I found myself staring over and over again at the opening and sign trying to read the text. Then there are those ugly little green and yellow heads with noses as long as Pinocchio's that light up when you are supposed to breath or exhale. It is so much easier to interpret to color of the evil grinning heads than understand what the CT techs are saying over the very crackly and way too loudspeaker.

All the stretching and yoga poses I've practiced paid off nicely, for when the CT Techs placed my arms straight out above my head so I'd fit through the CT unit's orifice, the techs did not have to tie down my wrists and the IV stayed in place. The entire test took about twenty or so minutes and, except for the hospital gown flapping about, went smoothly.

The CT Techs and nurses were speechless when I asked, after the test was complete, if I could take a photo of the CT Unit with my Ipad for the Marfan Blog.

Another CT Scan completed I walked down the hall of the lovely hospital, admiring the beauty of the courtyard's waterfall and stunning planted gardens, finding a place to sit and make sure I was not to dizzy to walk out.

My cardiologist will read the scans and compare to the last set of test results and I've an appointment to discuss the findings with him next week.

In the meanwhile, I'll still be asking myself the question, 'I wonder if my aorta is still dilating'. Such is the life of someone who deals with a connective tissue disorder such as Marfan Syndrome.

Tuesday, May 14, 2013

Marfan Life, Another Iodine CT Scan Today, Flagler Hospital, St Augustine

Flagler Hospital hospital is just twenty minutes north up US 1.  Today I'll be having another Cardiac and Abdominal CT Scan.

So I wrote a haiku for myself.

super hero's cloak
won't stay tied and so so cold,
blue hospital gown

 Those flimsy blue hospital gowns are just plain unstylish.  Being so tall they never fit me right anyway and make one feel, as Miami Herald humor writer says, "more naked than nakeed".

But I always worry how the iodine dye they use to get better contrast on the CT scan, will do to  my kidneys.  Yes, I do probably worry more than others, but that is my nature.  It doesn't help though that Judy's parents have some friends, one of whom just passed away from iodine dye invoked kidney failure. 

Looking, on the bright side, the cardiologist will, after today's test, be able to tell if my descending aortas dissection has stabilized, shrunk or (gasp) grown in size.

I don't like to think about it growing in size because I don't like to think about bad things and I don't really want to go through another surgery so soon.

There is a medication that I read last night about, called N-acetylcysteine and supposed a twenty five cent 500mg tablet can protect the kidneys from potential dye damage.  I am definitely going to ask about this drug today.  There is also a very interesting on the potentially life saving drug in the National Review of Medicine from a couple years ago, which you can access here.

Updates tomorrow.  In the meantime, I hope the Hospital Gown stays tied today!

Monday, May 13, 2013

Cardiovascular Health from Food? Beat Aortic Dissection with Delicious Watermelon

I love to eat good food.  And when you can eat all you want (well almost) and get healthier at the same time, you have found reason to celebrate.

Healthy diet is one area of focus since Marfan Syndrom and aortic dissection came into my life, especially when my cardiovascular system and my dissected aorta is supported.

Food for Healthy Cardiovascular Systems - Watermelon is one of the best!
Florida State University (FSU) researchers say watermelon is one of the best foods for lowering blood pressure, calling the juicy melon a 'functional food'.  Functional foods are those, according to FSU as "... foods scientifically shown to have health-promoting or disease-preventing properties, above and beyond the other intrinsically healthy nutrients they also supply."

In studies conducted by FSU Assistant Professor Arturo Figueroa and Professor Bahram H. Arjmandi, results indicated that a diet including watermelon provided natural defenses against hypertension.  According to the FSU professors, watermelon is "the richest edible natural source of L-citrulline, which is closely related to L-arginine, the amino acid required for the formation of nitric oxide essential to the regulation of vascular tone and healthy blood pressure".

Importantly, the highest concentration of the L-citrulline is in the rind.  This means eating into the green.  I found the rind taste to be excellent, not bitter and I was excited about the possibility of finding a natural support system for my blood pressure issues.

Heart healthy watermelon's L-citrulline is found in the melon's rind.
 As someone who suffers from Marfan Syndrome and a dissected aorta, I need to keep my blood pressure low.

Current medications include the beta-blocker Metoprolol and Losartan among others.  Without meds my blood pressure runs high 140s over 90s.  With meds my BP is 120s over 60s.  With meds and watermelon my BP has steadily hovered around 105-110 over 55.

I love to eat watermelon because it is delicious, especially on a hot Florida afternoon.  But in addition to the L-citrulline, watermelon, according to FSU, in addition to the vascular benefits of citrulline, watermelon provides "abundant vitamin A, B6, C, fiber, potassium and lycopene, a powerful antioxidant. Watermelon may even help to reduce serum glucose levels, according to Arjmandi".

For me, watermelon helps cure to 'go grab something sugary sweet' urge, thereby supporting good kidney function.  The less sugar I consume (I am trying to eliminate all forms of refined sugars from my diet, along with most processed foods) the happier my cardiovascular system and organs, such as liver and kidneys are.

I've even grown watermelons on some of the green roofs we have built!  Of course, Judy is growing watermelon in her backyard Florida permaculture garden.

Of course, with any change in lifestyle you should check first with your doctor.  Yet I am very happy with the positive impact watermelon in my diet has made on my life.

Information concerning the FSU watermelon study can be accessed here.

Sunday, May 12, 2013

Marfan Syndome, Aortic Aneurysm and Every Parent's Worst Nightmare

Every so often Ruairi, our soon to be 16 year old teen son will walk up to me and say, 'Dad, my chest hurts'.

#Marfan Syndrome, Our son, Ruairi's aorta is already beginning to dilate
Those words are every parent's nightmare and though I take beta-blockers to keep adrenalin surges from hitting my heart muscles quickly, the unexpected fright response kicks in.

As a loving mother, Judy my wife has the same reaction.  Sometimes our as our eyes meet, though our facial expressions calm as ever, we share deep, silent anxiety.

Of course we pay close attention to what our teens, Jincy and Ruairi say about any pain.  Aortic aneurysms and Marfan Syndrome issues have taken too many young children who were in or just approaching the prime of their life.

Out of God's grace and the superb medical attention I received in the Emergency Room at Jacksonville's Memorial Hospital the November night of 2011, I survived a complete root to foot, Type A aortic dissection.  I now have a St. Jude mechanical aortic valve and a Dacron graft for an ascending aorta.

One month later my mother has the same surgery and she too has the mechanical valve and Dacron aorta.

Genetics play a controlling role in Marfan Syndrome, connective tissue disorders and aortic dissections.  Unfortunately many people, including medical professionals do not recognize the symptoms or traits of this potentially deadly connective tissue disorder.  However, outreach efforts focused on education the public and medical professionals alike are beginning to appear around the globe today in response to too many untimely connective tissue related deaths.

Living in Flagler County, Florida provides our family with the opportunity to enjoy some of the worlds beaches, renown for surfing, fishing and coquina shell sand and surf.  Marfan Syndrome and aortic aneurysms are not however limited to older people living here in retirement.  Children and youth too are impacted by this devastating condition.  Sadly, several years ago a young twenty four year old local surfer, Tommy Tant passed away in his sleep from an aortic aneurysm in the prime of his life.  Today the Flagler Beach Community celebrates the life of the young surfer with the annual Tommy Tant Surfng Classic event to raise awareness of just how aortic aneurysms and Marfan Syndrome can affect youth.

Additionally, the National Marfan Foundation is a resource for individuals and families faced with the challenges of connective tissue disorders.

Yet young people are still unexpectedly dying, leaving this world at way too young an age.

Today more and more medical institutions are participating in research and providing health care for those afflicted with connective tissue disorders.  Stanford Hospital and Clinic, Boston's Children Hospital, and Cleveland Clinic are just a few of the hospitals with Marfan and connective tissue related disorders specialty units.

Yet just because these medical units are available today, the anxiety of being a parent to children diagnosed with cardiovascular issues doesn't diminish.  Those words, 'my chest hurts', are truly one of every parent's greatest concerns.

I personally know what a severe aortic dissection feels like.  I vividly remember the searing pain and can to this day recall how what it felt like.  Sometimes my experience lulls me into a false sense of security with the teens as I question them when they tell me something hurts.  I think I would recognize a really serious event.  That approach is wrong.  A really serious event would require immediate medical intervention.  And so I always take seriously what the teens are saying about their 'pains'.

Regular medical checkups that include CT scans, echo-cardiograms and other tests to make sure the teen's condition is not worsening are some of the proactive actions we as parents can do.  Of course we all have adopted lifestyle modifications including medications, diet, proper physical activity and blood pressure monitoring.

There are support groups too.  Facebook offers several Marfan Syndrome support groups where one can connect with others facing similar crises.  The support groups are so very valuable a place with respect to learning about available therapies, medications and of course how others deal with connective tissue disorders.  Worldwide, the Marfan community shares knowledge, tragedies, encouragement and success stories.

But heartbreak comes from these support groups too as members pass away unexpectedly, as happened again this week.  Always death is always sad, yet sometimes the lethal aneurysm or myocardial infarction comes at way too young of an age.

If the aneurystic event is severe enough there is not really any time to respond.  I was lucky.  My aneurysm dissected only through the first layer of my aorta's wall, giving me enough time to drive myself to the hospital ER.

Life is full of challenges.  There is plenty of pain and suffering throughout the world, much of it unnecessary.

Understanding the importance of cardiovascular health issues is vitally important, especially to parents of children with connective tissue challenges.

Knowledge is probably one of the most important life saving tools for families coping with Marfan Syndrome.

Those teen words will come.  It is not a matter of if but when.  Develop a relationship with a primary care physician, pediatric cardiologist and hospital that understands connective tissue challenges.  Those words, 'my chest hurts', are truly one of every parent's greatest concerns.  Know what to do when you hear those words.

Life is such a challenge.

Saturday, May 11, 2013

Marfan Syndrome? Aortic Dissection? Say What?

The below photo was taken six months before my aortic dissection.  I'm standing on a wonderful Green Roof at Breaking Ground Contracting in Jacksonville, Florida.
Aortic Dissection occurred six months after this photo by Ryan Ketterman of Ketterman Photography

I never had a clue as to what Marfan Syndrome was or that my connective tissue was slowly unraveling.

All the signs were there yet how was I supposed to know about these critical medical issues?  I had periodic medical checkups like most, but never once did any of my primary care physicians ever suggest I should be tested for a connective tissue disorder.  No one told me that all my episodes with strained or pulled muscles, multiple hernias, buck teeth before braces, and a tall lanky physique were indicators of Marfan Syndrome.

Even when my surgeon finished repairing one of my stomach hernias and told me "you have no muscle structure - eat more meat!" did he mention the possibility of a connective tissue disorder.  I have so much mesh in my body I could be a mighty good air filter.

My BMI was in the normal range.  I worked out.  We used EVOO in cooking and I had a high fiber diet.

Sure there were stresses, but everyone experiences the challenges of living in today's society, supporting a family.

But I though my blood pressure of 135 / 80 was ok.  Big time wrong.

Today I keep my blood pressure around 105 / 55 through diet and medications like Metoprolol and Losartan.  That is because though my ascending aorta is now a Dacron tube, up and over the arch the descending portion of my body's largest blood vessel is still dissected.  The inner layer, the intima layer has pulled apart from the middle and outer aortic layer.  My aorta is now like a very, very thin walled balloon.

Want to learn more about living life with Marfan Syndrome?  Follow the blog here for all the inside scoop, the ups and the downs of live with a dissected aorta and a connective tissue disorder.

Want more information on Marfan Syndrome?  Check out all the resources on the National Marfan Foundation website.

Friday, May 10, 2013

Marfan Syndrome Issues - Descending Dissection Issues

One of the reasons I started blogging about Aortic Dissections and Marfan Sydrome was to share what I've learned and experienced.  Today I wanted to mention about how my right foot is growing while my left foot is staying the same size it always has been.

Marfan Syndrome, Aortic Dissection extends all the way down my descending aorta into my right foot

There have been many times I never knew what was happening to me after the dissection and through out the healing phase.  Even today, I've barely the energy to get through the day, though I make myself by taking baby steps, literally, with each task I am confronted with.  With all my medications, my metabolism and heart rate are so slow most mornings it seems almost overwhelming to even get dressed for the day, much less figure out what new daily challenge was occurring with my body.

Not having answers was so frustrating at first.  Yet over time I came across resources and self-help groups.  So this blog hopefully can serve to help others facing an aortic dissection aftermath or living with Marfan Syndrome, either themselves or someone close, such as a child or loved one.  Both of our teens already have aortas showing signs of dilation.  So come back to the blog often and I'll try and  begin posting frequently about the many issues this connective tissue disorder presents.

But getting back to today's topic, I have come to learn I cannot be surprised too much at what happens to the body when dealing with Marfan Syndrome.  When my aorta dissected, the injury extended down through my abdomen into my renal arteries and down into my right iliac artery all the way to my foot.  Over half of the inside arterial diameter, or lumen diameter, is made up of a dead end false channel. 

Theoretically, the blood flow down through my right leg into my foot should be accordingly decreased due to the reduced available flow space in the artery.   One would expect that with reduced blood flow the muscle tissue and foot size would shrink.  Interestingly, ankle blood pressure readings show that both right and left legs have approximately the same systolic and diastolic pressures.  However my right foot is growing in length.

Of course this doesn't make any sense.  But lots of things about Marfan Syndrome do not make sense.  Yet now I have to wear a size twelve sandal to accommodate my right foot while my left foot remains it's original size ten.

The only answer I can conceivably come up with is the right foot's connective tissue is breaking down causing my foot to elongate.  I have noticed a 'sprained' feeling in my foot and ankle area, one I've felt from time to time though all my life, and I have started wearing an ankle support recently.

So for now living with the mysteries of Marfan Syndrome continue.  I am glad to have the foot because walking helps keep my heart and cardiovascular system function from falling to too much of an insufficient level.

I will update what I find out about this curious result of my aortic dissection and Marfan Syndrome as I come across additional elongated foot information.