Support Your Connective Tissues, Think Rationally Stress Less

It is hard denying adrenaline's power commensal with a Type 'A' personality.

Alpha hats rule!

Probably my drive over the years to be 'a leader of the pack' has been partially responsible for the open heart aorta surgeries I've required.

Today, my increased awareness of the hormone's destructive power is allowing me to replace the automatic flight or fight responses with a more rational response when I'm faced with challenges.

But unfortunately those daily challenges perceived by me to be a direct confrontation - such as another driver cutting me off on the road - still produce that all too familiar, mostly unproductive adrenaline surge.

When challenged, my blood pressure rises and stress increases as cortisol pulses through my veins.  In the car, fingers tighten around the steering wheel.  Words not normally suitable for public use whirl around my amygdala while my frontal cortex wants to slip into hibernation mode.

But with the passing of a few dangerous seconds the icy layer blanketing my cortex's rational  systems thaws and the red quickly drains from my face.

My pulse drops back to the usual forty to fifty beats per minute.

By now the offending car is far out in front of me.

I'm not going to speed after it to show them who is...., who is what? Maybe more quickly prone to push an accelerator pedal down?  Best at petal to the metal?  Come on.

Really, was it super significant that an aggressive driver cut me off?

Yes and No.  And the struggle between the ancient survival portion of my brain and the more modern frontal cortex continues.

But my connective tissue limitations require me to deal with fight or flight response in a way that supports my cardiovascular system rather than further damaging my aorta.

My Type A aspect urges for resolution by dominance while the Beta aspect (Type B) presses for a ' do what benefits you most longterm' ending.

As a human driven by testosterone I struggle with adrenaline and cortisol prompted stress daily.

As a connective tissue disorder (Marfan) challenged person who has experienced first hand the damage these influential hormones can cause, I am finally learning to recognize the first signs of situational generated stress and now learning to initiate rational mitigating responses.

Its not easy, honestly.  It is a work in progress though but I am improving.

Self recognition of my dominant Type A tendencies was a good start.

Today, living with a dissected descending aorta (ascending is now Dacron) requires stress control.  Stress causes sheer pressures to build and aortic inflammation to occur.  I need neither if I want to live.

Research shows that emotional stress can trigger an aortic dissection.

So stress control is extremely important to those of us challenged by Marfan, Loeys-Dietz, Ehlers-Danlos or any other connective tissue disorder or syndrome.

But just when I thought I was doing better lately, a red mustang cut in front of me yesterday boasting a big bumper sticker that read 'If you are reading this you are loosing!!!'.

I always drive no faster than the speed limit.  My driving capability is a privilege I absolutely need, and besides - whoever printed up that bumper sticker couldn't even spell.

Four years ago my license was medically revoked because of my dissection.   Today however it has been restored and I take the privilege of driving very seriously.

Three years of living without a driver's license taught me many things.  Near the top of the list is 'do not get a ticket and jeopardize the driving privilege'.  So I stick to the speed limit.

Many drivers consider the speed limit to be 'grandpa slow'.  So on a daily basis drivers, young and old whiz around me.  Most just whiz by.  Some blow their horns.  A few flip me off.

All the whizzing by does bother me but my rational frontal cortex has learned to be boss.  Amygdala is still active but now understands about consequences and the 'time-out' corner.  Amygdala has learned of downsides of 'the ticket'.

At first this shift from Type A to Type B responses in my life made me feel as though I didn't live up to what evolution had programmed my body to be.  Survival required brute dominance, right?  I mean look at the lions and gorillas and baboons fight for 'boss' rights. I had to be first in line at the traffic light.

Nature selects for Type A, Alpha, 'leader' personalities, correct?  Think about mating rights.

Not necessarily.

Though Type A personalities may 'earn' certain short term mating rights or dominance positions in some species, Type A lifestyles also carry many downsides too.

Alpha personalities normally possess higher stress levels, and as some research shows may harbor even lower intelligence levels.

What does that mean to a connective tissue challenged individual?

Simply put, Type A driven actions may move one to the front of the line but they may not be there long if the adrenaline and cortisol surges damage the cardiovascular system to the point of a dissection or aneurysm failure.

There is a very interesting report entitled "We Can All Relate To Stressed Out Baboons", where Stanford physiologist Robert Sapolsky is reported to suggest that the stress of competition associated with an Alpha personality actually leads to disease and depression.

Sapolsky goes on to theorize that those who can differentiate between real threats (requiring life saving flight or fight responses) and common competitive but non-survival situations may have much lower bodily stress levels.

The implications here are especially important for connective tissue challenged persons.

High levels of stress are more damaging to cardiovascular systems than smoking, according to Sapolsky.

Increased cardiovascular stress is the last thing anyone needs, especially if looking to minimize aortic dissection risks.

Today I'm thinking whoever printed the bumper sticker about 'loosing' may have actually been a Beta personality, one who figured out how to make money off of the Alpha's irrational drive to be the brute and possibility their inability to spell correctly.

I will always struggle with dominance stress.  My long legs propel me to the front of any group walk about.  My amygdala still whispers about being the fastest and best-est.

No longer though do I think the Alpha personality is superior to the rational, calmer, more socially integrated approach.

With the Alpha approach the game is controlled by others so paradoxically we always end up 'loosing'.

Beta responses allow us much more control, especially to the connective tissue challenged population who are genetically predisposition to potential aortic tearing.

And maybe ultimately a more extended shot at mating too.

Take that you Alpha powered red mustang!

You are the one who is really 'loosing'!

Intentional Unintentional Disability Discrimination #Marfan #Aorta #Dissection

Sometimes a gentle reminder is necessary.  The world is full of disadvantaged and 'minority' groups.  Those of us who are disabled often feel, as many other disadvantaged peoples do, that we are all too easily forgotten.

Parked in Not One But Two Handicap Spots! #Discrimination #Disabled

Days can go by filled with and full of positive reinforcement.  Usually most people are so willing to help one with a walker or cane through the door or provide a space at the head of the line.

But just when you take the deep breath of appreciation for those supportive weeks, out of the blue those bad days show up.

The deep breath of feeling a part of 'normal' society is suddenly deflated.  Poof.  Emptied.  Exhaled.  Gone.

Like the good days the bad days seem to come in waves.

This week was one of those times where the notion that I, as a person with physical challenges (chronic dissection and affiliated health issues), was not really a part of the 'real' world.

A pity party perhaps?  Maybe reoccurring PTSD, or subliminal depression? Maybe my self-esteem is too low?  And really, why do I take perceived discrimination so personal?  These are all thoughts that shoot through my mind after a day filled with 'intentional unintentional disability discrimination'.

The phrase 'intentional unintentional disability discrimination'  may sound confusing.  Simply put, it is when someone intentionally, but without overt malice, acts to ignore or avoid the reality of the disabled persons world.

And this week I was reminded that 'intentional unintentional disability discrimination' is all too alive and well in the world.

First and foremost, discrimination is discrimination - intentional or unintentional, blatant or subtle.

There is no excuse for any type of discrimination, at all, ever.  However most of us are willing to give others the benefit of the doubt.

"They really did not mean what they said or did" is the phrase I find myself thinking when confronted with 'intentional unintentional disability discrimination'.

However, persons must accept responsibility for their actions, intentional or unintentional.  When discrimination occurs it occurs, and ignorance is no excuse.

For me the solution lies in sharing peaceful awareness, letting the offending party know I feel I've been discriminated against as a person with limiting physical and mental challenges.

No good can come from aggressive confrontational or angry retorts.

But I've seen great things happen when the 'normal' world acknowledges and accommodates a disabled person's struggles.

So to the person who replied, "My sense is that this is no path for people to trifle with if inexperienced" to my post in a Florida Trail Hiking group inquiring if there were other disabled or physically challenged hikers, I know your comment was unintentional with respect to any discrimination, perceived or otherwise.

Immediately I equated the terms 'trifle' and 'inexperienced' to be discriminatory towards disabled persons though.  Just because one is disabled does not mean they are trifling with the sport of hiking.  Likewise just because someone may be a disabled hiker does not necessarily mean they are an inexperienced hiker either.

Making an instant leap from 'disabled' to 'trifle' or 'disabled' to 'inexperienced' is discriminatory, intentional or not.

I replied and suggested that sometimes persons with disabilities may actually be more aware of safety issues due to daily coping with physical challenges.  The commentor agreed and let the topic pass by saying "maybe disabilities are just matters of individuals limitations to imagine another person's capabilities".  Not too sure what they meant but the reply sounded helpful.

Anyway after thinking on the matter for a day I moved past the thought of 'trifle or inexperienced hiking' as a good description of my focused outdoor physical therapy treks.

But then the Frito Lay truck showed up.

In Florida it is illegal to park not only in handicap spots without a handicap designation but it is also illegal for anyone to park in access isles adjacent the handicap spots.

The disabled license tag on our van helps me tremendously.  The doctors say I should not lift heavy items.  A dissected aorta's tear can worsen under shear stress and carrying groceries to the car can create that stress.  With my Marfan connective tissue challenges debilitating bone and joint subluxation can occur with any step. So I am grateful I can make the trip from checkout to car as short as possible while carrying groceries.

Invariably, I end up doing more than I am supposed to.  It's my nature.  I want to help.  I don't want my wife to do all the lifting either.  I don't heed the advice of my doctor.  So the disabled parking spots keep me in check too.  They help me stay alive.

But when I pulled into the Fort Myers Beach Publix and found the Frito Lay truck parked in not only one disabled spot, but multiple disabled spots, I was puzzled.

Certainly parking in a handicap spot without the handicap designation is illegal under Florida law.  Florida Statutes, Title XXIII, Section 316.1956 states:

"316.1955 Enforcement of parking requirements for persons who have disabilities.—

(1) It is unlawful for any person to stop, stand, or park a vehicle within, or to obstruct, any such specially designated and marked parking space provided in accordance with s. 553.5041, unless the vehicle displays a disabled parking permit issued under s. 316.1958 or s. 320.0848 or a license plate issued under s. 320.084, s. 320.0842, s. 320.0843, or s. 320.0845, and the vehicle is transporting the person to whom the displayed permit is issued."

The use of multiple handicap parking spaces for commercial transactions doubly surprised me because Publix is quite aware of and sensitive to the challenges of the disabled.  In fact I see more persons with physical disabilities employed at Publix than I do most anywhere else in Florida.

Handicap Parking is Meant to Facilitate Access for Disabled Persons, not Commercial Enterprises

Kudos to Publix for recognizing the challenges of disabled persons!

Publix also displays their commitment to handicap accessibility with a large sign next to their front door.

Publix is Keenly Aware of the Importance of Handicap Access and Kudos to Publix for Their Efforts in Disabled Employment!

So I was actually taken aback with the 'intentional unintentional disability discrimination' by both Frito Lay and Publix with respect to Florida law, handicap parking and disabled persons access.

Moreover, these photos were taken after the store had opened.  But the law still applies to handicap parking after hours.  There is no excuse.

Truly I know there is no ill will towards persons with disabilities from either Publix or Frito Lay.  This is just another case of 'intentional unintentional disability discrimination'.

Yet it stings. And it is wrong.

Upon seeing the Frito Lay truck parked in the handicap spots and access isles I felt that same notion of being a second class citizen, one many other 'minorities' feel daily too.

But I only ask that we acknowledge what is real.  Disability discrimination does exist, daily.

And the only way to overcome disability discrimination is through awareness.

So, thank you Publix for all you do to help persons with disabilities.  And thank you Frito Lay too.

This may be an appropriate time to remind all parties that ensuring American with Disabilities Act compliance is an ongoing effort, one we can not afford to forget.

Intentional Unintentional Disability Discrimination is wrong.  We all need to stop ignoring it and work together to make it go away.

We are Overlooked Assets! #Marfan #EDS #LDS

Sometimes we as a nation can't see the trees for the forest.  When people make fun of those with disabilities, that jesting can foster cultural underestimations of talent values.

Connective Tissue Challenged Persons.  We are assets!

In the last week I've been declined for life insurance and jeered at while driving along with the other typical situations someone with a health challenge encounters daily.  Our van has a disability tag and I purposely drive the speed limit.  Other drivers often gun their engines to pass me by, blowing their horns loudly and usually flipping fingers my direction.  This week a man about my age rolled his windows down while flying by and twisted up his hands and face as if he were having a seizure then laughed as he sped on into the traffic ahead.

Today I take it all with a grain of salt assuming these people are probably having a really stressful day, pumped up on coffee and probably dealing with dangerously high blood pressure.  I am very happy with who I am, health challenges and all.  In fact I think my health challenges make me special.

Not everyone I encounter treats me this way.  Many are courteous, notice I am walking with a cane and treat me with kindness.  My doctors and nurses and their staff seem to really care and for this I am grateful. But our alpha nation, in its fast paced bootstrap mentality often treats those of us with pronounced health challenges as a discardable class.

Attitudes of disregard for the disabled can even be found in our governments.  Since my dissection in 2011 I've had my drivers license medically revoked and I've even been handcuffed, threatened and put on the ground by Flagler County Florida sheriff's department, hands on their guns and tasers as I walked to the grocery store one afternoon with my cane, a limping man that was an obvious threat to society.

Inevitably within the business community my resumes are politely returned, with a thank you but not interested response once my dissection issues surface.  Some say don't disclose the issue but I believe in transparency and it is hard to otherwise explain away the hospitalization time gap.

The 'forest' of mis-information and unfounded assumptions surrounding people with disabilities masks an amazing pool of talent though.

Over the past five years I've come to know others living with connective tissue challenges, aneurysms and dissections, scoliosis and pumphead, chairi and other conditions.

They are some of the most ingenious, brilliant, kind, enthusiastic and talented people I have ever known.

They are also surprisingly some of the healthiest persons I know, despite walking a fine line between life and death in many cases.

And they are certainly some of the most shrewdest and analytical persons on the planet.

Our communities, our nation and our world would be so much better off taping into this talent pool instead of pushing us to the side.

When living with a life threatening issue one realizes that time is truly borrowed and holds great value.  We can be better time managers than the most productive wall street executive for we know the value not only of each moment but of each breath.

And in spite of our challenges, many of us are more healthy than most.  Our diets are focused on  non-processed foods while we avoid inflammatory, artery clogging junk.  Our CT scans may reveal a dissection flap but they also reveal clean arterial pathways and healthy organs.

Yes, we may have physical or mental limitations but we've learned to adapt a better way and work around those issues.  Adaptation is a key component of long term evolution and survival.

Our awareness allows us to deal with and address our limitations - giving us an advantage over many of those who like the ostrich with its head in the sand, don't know what their arteries look like or haven't been to the doctor in a long time.

Besides, as someone recently reminded me, all of us are dying.  Those of us with diagnosed health challenges are just more keenly aware of this fact.  We are some say, better prepared than most when an incident occurs and in the meanwhile make the most of our allowed time.

Because of our focused efforts on our health I would suggest that we are a better class of individuals to issue life insurance policies for, rather than being rejected time after time for 'heart conditions' or other generic but unsubstantiated factors.  Yes we've disclosed these limitations on our applications.  They may sound 'un-insurable' at first take. Yet I still contend those of us with health challenges may be a better insurance wish than those in the population who appear healthy but may be walking time bombs themselves.

Over the years I've met some of the greatest artists, poets and well read intellectuals who each battle with serious or debilitating health issues.  There is an amazing pool of knowledge and creativity waiting to be tapped by a society willing to embrace differences.

And we would make the very best of employees.

All we need is a chance.

But the life insurance rejection letters, the returned job applications, the brush-offs, dismissals and cold shoulders, exhaust smoke and fingers still keep coming.

Fortunately, we are a resilient group.  We don't give up easily.

Many of us describe ourselves as 'survivors' or 'warriors', a fitting description for a group who have experienced trauma and difficulties most can not even imagine, and come through to the other side with strong fortitude and unspoken yet perfected resilience.

The world would be so much better off if this pool of talent was brought into the fold instead of being pushed to the side.

We truly are an overlooked worldwide asset.

And so we ask the world to take note of what we can offer.  We ask all the support organizations out there that provide us with wonderful resources on our afflictions to also advocate for our acceptance into mainstream society.

Those willing to take a risk on us will be rewarded far beyond their expectations.

Living a life challenged by connective tissue issues, cardiovascular and skeletal, vision and other maladies is the best training ground for human creativity and enlightenment.

All the world has to do is to ask for our help.

And if they don't then they'll be missing out.

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!

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 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!

Living with an Aortic Dissection & Marfan Syndrome

Living with a dissected aorta, Kevin Songer & granddaughter Cali

I experienced a 'root to foot' aortic dissection on November 30th, 2011.

Though I received a St. Jude's Aortic Valve and Dacron aorta, my descending aorta is still dissected from the graft down to my feet.

Its been just over one year now and I am still alive.  Challenges arise everyday though.

One of my most significant areas of frustration has been the lack of available information concerning  the dissection, my medications, available treatments, steps I can take to improve the condition, 'mysterious pains', 'what-if's' and so much more.

Granted, the National Marfan Foundation (NMF) is a great place to start yet my particular significant issue is the dissection.  NMF provides a good deal of information regarding aortic dissections but does not address many questions I have had over the past thirteen months.

Living in Florida, I've joined the Florida Marfan Support Network on Facebook, and subsequently been blessed with meeting many others encountering similar issues there.

Moreover,I've even began my own Facebook Marfan page!

And I've been wanting to start a weekly Aortic Dissection blog, so getting a jump start on the New Year's resolutions, here goes.  Look for my thoughts of living with a metal valve, Dacron aorta, Marfan and most importantly a dissected descending aorta that could aneuryize any day.

With a wonderful wife, grown children, two teens and grandchildren I want to stay alive as long as possible.

Whether you are experiencing an aortic dissection for the first time or a veteran of the disease, your thoughts and comments are also welcome.