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.

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.

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!

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.

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.

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.

Marfan Syndrome, Physical Characteristics in the Family

The photo here shows my two wonderful teenage children, one in college already and the other active in High School.  According to the last echo-cardiogram both of their aortas are already beginning to dilate.

Marfan Syndrome physical characteristics

As a child I too was tall and lanky, with long fingers, long arms and legs and a thin-skinny build.  Though these physical characteristics are not always indicative of Marfan Syndrome, they may be.

I am glad that we now know of the connective tissue issues our family members experience.  Knowledge is important.  Understanding and tracking health issues associated with Marfan Syndrome can save lives.  Whereas I never know I had connective tissue problems until  my aorta dissected, we know the kids have these issues and we can address them before acute problems arise.

Some of the symptoms I regularly experienced as a child included; sprained wrists and ankles, collapsed arches, pulled back muscles, multiple hernia surgeries, stomach hernias and then finally a completely dissected and aneurysed aorta.

The National Marfan Foundation website is an excellent place to learn more about this health issue.

Education about the medical issues associated with Marfan is critically important.  Know what resources are available to help you or others who may suffer from potentially life threatening health problems arising from Marfan related problems.

I'll be posting more soon about my unexpected aortic dissection and how I've coped and what I hope to do, as my primary care physician says is my 'full time business' - to stay alive.

Marfan Dissection and My St Jude Aortic Valve

Along with the Dacron Graft I received a St. Jude valva.  Below is a photo of the specific model that keeps me alive.  The surgeon, Dr. Bates told me later he could have used a pig valve but since I was younger at the time he used the metal valve.  Supposedly it is rated to last ten thousand years.

My St. Jude aortic valve and Dacron Graft!