Some Good Days Some Bad Days but they are all Marfan Days

Some days are better than others.  This principle applies to all but may be especially relevant to those of us with chronic illnesses.

#IknowMarfan Become Involved! February is Marfan Awareness Month  www.marfan.org 

Others may see my smile, glossy nature photos on social media, and long walks as a sign that all is good.

But  I know the real truth.  Yes some days are better than others.  But every day is a Marfan day.

Most days I try and portray an upbeat attitude, usually answering a 'how are you?' question with 'everyday is a good day' answer.

And much of the time my positive answers and attitude convince even myself that connective tissue disorder life is not really that bad after all.  Especially on those days that are better than others.

Unfortunately, as I've said before, even the better days are still Marfan days.  This truth applies to all other connective tissue syndromes too; Loeys-Dietz (LDS), Ehlers Danlos (EDS) and so many others.

Even the best connective tissue disorder days are still connective tissue disorder days.

Amazingly a good Marfan day can instantly change and become a day some days are better than.  A wrong step, a bump against the door frame, a twist of the neck in the wrong direction, and even a hard sneeze can turn a 'better than other day' into the start of a painful, hurting week.

#IKnowMarfan Coumadin plays into Marfan days for many.  Here is my arm today - internal bleeding and hematoma 

Unless the torn tendon or ligament bleeds enough for a huge hematoma to form, or an arm or leg subluxes to where crutches are needed just to move, most others never even know how bad those with chronic connective tissue challenges may hurt.

Many of us look really fit, trim and healthy and we often hear the stinging compliment, "you look so good - so how can you be sick?!"

But even on those good days when we glow, smile and laugh the chronic monster of painful potential hovers just over our heads, ready to pounce at the first unexpected chance.

The concept of 'I may look somewhat ok' and 'this may be a better day than others but expect the unexpected' can really wear us down.

A Marfan day is a challenge, even if it may be one of the best of the better than other days.

Marfan can manifest in a number of ways including scoliosis, retinal detachment, muscle and joint disfunction and as in my case, mouth and teeth malformation to where I required braces, multiple hernias and worse - aortic dissection.

Today I live with a dissected descending aorta.  My ascending arch and aortic valve are mechanical and Dacron but my descending aorta is torn and blocked by about 70%.  Unfortunately I was unaware  of my connective tissue challenges until the night I dissected.  Had I known I could have potentially avoided my traumatic emergency surgery and subsequent surgery for graft infection.

Awareness is critically important.  And so we must share.  The Marfan Foundation provides educational and support resources concerning aortic dissection and other connective tissue challenges. To learn more about dissected aortas be sure to read the helpful information shared through the John Ritter Foundation website.

February is Marfan Awareness month.  So be sure to check out the #IknowMarfan hashtag across social media platforms and read more about Marfan Syndrome here.

Finally, many times a Marfan day will include more than just one connective tissue challenge.

For me the Post Traumatic Stress Disorder (PTSD) from two open heart aortic surgeries always looms just below the surface of daily activities.  Many days my upbeat approach to life masks the PTSD, other days something small or seemingly insignificant may trigger a rush of fear, dread and worry .  Some days are better than others but all Marfan days take their toll.

Most people in the world don't go through life aware their aorta is dilating every day, expanding and enlarging to the point where like a ballon, it may burst.  Living with the dread of imminent aorta replacement surgery is really stressful.  Even on the best of days the dread is hard to cope with.  But the aorta surgery worry is real and a part of most Marfan Days just as scoliosis, retinal detachment and other health challenges too manifest themselves most Marfan days.

Yes those of us challenged with Marfan Syndrome and other connective tissue disorders try mostly to 'get on with life' and 'keep on keeping on' or 'hanging in there'.  We try.

And many days that is just what we do and we are good at masking or hiding the hurt.

Then there are those less than better Marfan days where all the challenges just seem piled up way too high for us to deal with.  We want to let out a big sigh but our chest hurts way too much to let the sigh out.

No one understands so it seems.  Some days I feel like we are up the creek without a paddle, all alone, all by ourselves.

Social media support groups really do help but we long for an understanding touch, a personal hug from someone who does more than feel sorry from us.  We need someone who understands; really understands.

This is why becoming involved with The Marfan Foundation with local, but also national and worldwide efforts is so very important.

Each one of has so much to share and teach and receive from others.

We've been through those Marfan days.  We live them each and every day of our lives.

We've 'been there done that'.  Some of us have 'been there done that' for years now.

Whether it be retinal, muscular, cardiovascular or skeletal we all have some words of understanding to offer others.

Read through The Marfan Foundation's Get Involved website.  Become involved.  We have so much to share.  We need each other. Its all about awareness and sharing.

#IKnowMarfan

Aortic Dissection and Gratitude

Its taken a lot of generations of my ancestors to produce me. Last night I was thinking of how much I appreciate all their unspoken-of struggles over the centuries and millennia .
I appreciate how many endured dissections and aneurysms probably just like I did (dissection in our family is genetic - my mom had the same aorta replacement as I), and they adventured on. Since aorta replacement has only been around for the past thirty years of so - many of my ancestors endured and survived without medical repair.
I'm sure many times some of my ancestors wondered what was happening to them, not having community support as we do today. Many got up in the morning, went to work and despite the challenges of a weakened cardiovascular system, did what they could do, despite limited knowledge of their condition and limited medical treatment availability.
The challenges our ancestors faced were enormous. Everyday they had to completely create commerce, food, shelter, protection and family. Many did not have even a small portion of the medical care, shelter, transportation or luxury available to me.
Yet our ancestors all had one thing in common and that was they were "Survivors".
And they adventured on long enough to pass those survivor genes on to us.
Today I am grateful for all their struggles, challenges and perseverance to make sure we too could be survivors.
Today, I am Grateful to them.

Connective Tissue Life Doors and a World Market Pottery Mug

Dissection Life has opened and closed many doors in my life.  One maxim I've found to be unchangeable is 'There Will Always Be Change".

Ritual simplification to celebrate life's changes.  Buy a mug and bowl from World Market.

One particular project I've been working on is simplification.  Indeed, before I was diagnosed with a connective tissue disorder and the big dissection I had accumulated quite a bit in life.  Too much to take care of.  Way too much to take care of.  Of course, I was usually stressed about all the worldly possessions I was carrying around in my 'life backpack'.

When I began the simplification process several years ago a sense of new found freedom immediately swept over me.  This was good.  Stress causes inflammation.  That is bad.  Inflammation can chip away at our aorta until it finally tears.

Last thing I needed was anything chipping away at my genetically pre-disposed to tearing Marfan aorta.

Lifestyle simplification is a disposal of both things and also stress.

Ultimately my goal is to own only one hundred objects.  However even then one hundred objects can be a lot to take care of.

So with each new door of change I encounter on this #DissectionLife journey, there exists an opportunity to simplify even further.  Simplification during change also dulls the emotional pain sometimes associated with big life changes because the act of simplifying affords a level of distraction from the possibly negative change event to the positive results from simplification.

That last sentence was a mouthful and probably could use some simplification itself.

I am really proud of Ruairi's summa cum laude standing upon high school graduation.  He is now off on his own life's journey after Saturday graduation and a successful Sunday drive to the University of North Florida.

He even was awarded a Tommy Tant scholarship as one of his many scholarships.  Tommy Tant Memorial Classic is a surfing event each year in Flagler Beach, Florida to remember Tommy Tant who passed of an aortic aneurysm.

Another door.  The house is not any more quiet, I just know now he is not coming home each afternoon after school or basketball.

For me, embracing each new life change with a celebratory act helps afford validity to the particular change.  So yesterday I went to World Market and bought a new pottery bowl and mug that will become my kitchen utensils for eating.

With no children in the house I am hoping we find the sink less full of dishes.  Now, with my one bowl and one mug, I intend to keep them washed and on the shelf after each use, and out of the sink.

So two of my one hundred personal items are made up of a dark blue World Market pottery bowl and matching mug.  Ruairi has left the house.  One door has closed and another door opened.

Nothing earth shattering but another couple steps towards the Zen I find in simplification. And that is good for my existing medically managed dissection.

One thing #Dissectionlife has taught me though is all those little steps add up.

And anointing each change with a separate act of celebratory simplification makes the journey easier and more interesting.

You can find out more about my Project 100 here.

How Strong Is Your Aorta? Even Dissected the Aorta is Tough and Durable. The Plywood Matrix.

For a couple years after those two open heart surgeries night time would bring with it a Pandora's Box full of demons I created in my mind.

Aortic Dissection.  Check out my existing intima flap!  Both sides are patent but the false lumen is a dead end.

Recently I put a lock on that box.  Once in a while, in an evening's moment of self pity I'll still unlatch the box and peek back inside.  And then regret hits me for days.

Laying in bed at night the fear of dying would consume me.

Each new twinge of pain or hurt was a prelude to, as Fred Sanford used to say on his TV program Sanford & Sons, "Oh, this is the big one".

Unlike Sanford & Sons, my fears weren't funny at all.

Getting all my 'loose ends' tied up and affairs in order helped, but whenever I opened that wicked box again demons flew in my face.

The years of fear that my aorta was going to 'pop' paralyzed me, mostly when I lay down to sleep in the evening.  During the day I kept myself so occupied that time never arose where I could concentrate on my ever imagined mortality as I could after quietly sliding under the sheets.

Looking back on the night when I dissected and drove myself to the ER, I was not afraid then - even when the doctor told me what was going on and of my chances.

Laurence Gonzales in his book, Surviving Survival, suggests that the real challenge to our sanity comes not during the traumatic ordeal but afterwards when our demons gather around.

I grew to dread sunset for that was when my demons would gather to discuss my future.

Then one day I met this interesting doctor.  We moved to Fort Myers from Palm Coast so I could avoid the cooler winters.  My Raynauds issues prevented me from participating in many activities when the temperature dropped below 60F.

My new primary care physician came with multiple recommendations from friends.  I liked him instantly.   He listened to my thoughts and discussed my ideas as valid rather than quacking dismissing them.

Blood pressure maintenance was critically important when managing an aortic dissection long term he reiterated.  And he praised me for maintaining a 'low risk' range of 105/60 with  a pulse of 60 beats per minute.

'Doc' as I'll refer to him, told me a blood pressure of 105/60 carried with it almost no risk for causing cardiovascular damage.  He repeated the 'almost no risk' over and over.  This has stuck in my mind.

"But Doc!" I'd exclaim. "My aorta is peeling apart!"  It could blow at any moment.

"Yes it could with a high blood pressure.  I have patients with systolic over 200 and diastolic approaching 150.  In their cases, yes.  The aorta could rupture."  He shook his head. "But your aorta is still strong.  And your blood pressure is perfect for long term management."

"What do you mean my aorta is still strong?" I asked, puzzled.  "I feel like my aorta is much like a thin, over filled balloon ready to burst!"

"That is what I hear from my other dissection patients," he replied.

"Listen, let me use an example.  Your aorta, Kevin, is built like a sheet of plywood.  Think of layer upon layer of wood glued together.  Now like plywood may do if it gets wet, your aorta has had the inner layer separate.  Plywood does this often but still retains much of its original strength for a very long time".

He continued.  "We know you have a connective tissue challenge so your aorta and body parts may have a tendency to separate.  This is aggravated especially when you have high blood pressure.  However when you remove most of the stress from the layered plywood or layered aorta, the remaining layers can hold up for a very long time.  You may well live a normal life span."

I could relate to his analogy.  There are plywood boats I've seen warped and separating but still floating.  Plywood used to cover windows many times stays in place for years.  It is easy to imagine the difficulty of trying to pull a separated layer of plywood apart from the remaining wood panel.

"Hmmm" I muttered.  "So even though I have a seriously dissected aorta, the remaining layers are still quite strong'" I said.

"Yes, very strong.  Now aneurysms do happen and aortas do rupture, but not 'normally' with proper blood pressure control.  Keep your blood pressure down, avoid straining of any type, eat healthy and exercise."

"Wow, Doc."

"You are going to live a long time I suspect.  Anything else we need to talk about?"  Doc shrugged and opened the examining room door to usher me out.  "See you in six months or sooner if you need to come in."

The battery of annual CT scans and echocardiograms I have seem to prove Doc right so far.  My dissection/ aneurysm is stable, not much change so far after four years.

Now I am not an unrealistic dreamer.  I do recognize the seriousness of my condition, after all my aorta is dissected from the ascending Dacron graft down into my kidneys and iliac arteries.

But for some reason the idea of a tough matrix like plywood, even though it is separated, puts my mind at ease, at least to the point of where I don't feel anymore like I have to invite the demons each night to come and discuss my future.

Perhaps it was Doc's almost caviler attitude about not being too concerned with the chances of an immediate aorta rupture.  Perhaps it was because I could relate to just how long warped plywood could last.

Definitely it was a paradigm shift from the thin over filled balloon to a low pressure tough matrix vision of my heart and main blood vessel that convinced me to snap the lock shut on that box of taunting demons.

I believe there is truth in what Doc says.

And because I am convinced that my aorta is a separated but still quite strong I am not going to burst or pop any given moment, I have been able to go to sleep with less worry.  Maybe I will, maybe I won't burst in all reality.  But if believing in the strength of plywood keeps me from opening that wicked box at night, then I will keep on believing.

Blood pressure control is very important.  And with proper blood pressure control my layered blood vessels may really stay put.

Plywood matrix means strength.  And our aortas are quite strong.

Finally, lying in bed the other night I realized that our friends, family and even dissection and aneurysm forums on social media are like a strong matrix too.  We all help hold each other together.  We are the glue and layers of a very strong community.

I like the idea of strength in matrixes, even if there is a misaligned layer here or there.

How strong is my aorta?  Plywood tough!

Aorta Dissectes Unexpectedly, My Near Death Experience

Unexpectedly on November 29, 2011 my aorta dissected all the way from the valve in my heart up over the arch, down through my abdomen into my renal arteries, following my right iliac artery through my pelvic area into my leg and ending in my foot.  One great big ripping tear.

Home alone that evening with no one to turn to except for our dog, Lily, I had no idea what was happening as the initial acute momentous pain began to change into a deep chronic hurt after about ten minutes.  Walking into the master bedroom I sat down on the edge of the bed, slowly lowering myself back across the top quilt, hoping I was imagining the whole episode, not accepting the fact that control of my body was no longer mine, nor recognizing I had finally hurtled into a really big brick wall.

There was little doubt in my mind something bad was happening inside my chest.  My back felt as though it was being plummeted with a heavy wooden baseball bat, the bottom of my left jaw badly ached, I was dizzy and experiencing an intense headache.

For a brief moment I smiled, exhaling out an ever so slight chuckle, watching the slow spinning ceiling fan seemingly pull me into what I was imaging to be a long, dark vortex.   Day-dream like hypnotic trances are sometimes difficult to shake.  Traveling down into the swirl almost seemed like a relief, an opportunity to explore something different.  But my will to live has always been strong.  I set up and holding onto the edge of the bed and then the walls, moved out of the bedroom, down the hall into the kitchen where the Honda keys were hanging on the wall key bracket.

Four miles away stood the Memorial Hospital complex on the corner of Beach Blvd and University Drive.  Taking the first step out the door was becoming more difficult with each tick of the wall clock and I knew if I stayed at home Judy would probably find me cold lying stretched across the bed the next day.  I reached for the Honda keys.

Flipping on the back porch light, I stepped out and filled Lily's water and dog food bowl, telling her I'd be back soon.

The back route through the neighborhood was uneventful. Winter's stars twinkled brilliantly high in the sky.  Cool night air flowing through the half rolled down drivers door window temporarily renewed my labored breathing, lifting my spirit.  Praying for God to save me, I suspected a heart attack though could not reason how or why.

The trip to Memorial only took five precious minutes yet during those five ticks around the clock's face much of my adult life flashed back through my thoughts.

In my haste to fly through life I was truly neglecting my body's real needs.  In my rush to become the American success story I ended up deceiving myself into believing all the limited diet and intense exercise efforts were good for my body when in fact I was actually destroying it.

A twenty five pound dumbbell had for the longest time sat by the back door.  Each time I came into or left the house I'd pump the iron over my head believing the anaerobic muscle building would provide long term benefits.  After a bout with colon cancer in my early fifties I'd implemented a high fiber diet, daily partaking of the garden fresh hummus I'd make every day or so.

We cooked solely with olive oil and I believed the virgin liquid was going to be a major reason for my personal long term cardiovascular health.

Hard physical labor was no stranger to our household.  Judy was always working in the backyard vegetable garden.  I had just finished a marvelous green roof where I literally carried two tons of planting media up stairs, on my back, to the roof.  I looked good and felt even better.  Florida Trend magazine had just published an article on the trend setting Breaking Ground Contracting living roof.

Yet other issues were silently eating away at my body.

Physical, mental and emotional stress fed hypertension.  Each uptick in systolic or diastolic compromised bit by bit those hidden flaws in my Marfan body's arterial elasticity and cardiovascular structure.

But now, pulling into the Memorial Hospital's quiet parking lot, the truth shown brighter than the eerily buzzing tall evening halogen lights.  All the hurtling forward at breath-taking speeds through time and space to make a living, raise a family and get things done had really only shortened my life.

Unfortunately I pulled the Honda into the parking garage located at the far end of the health campus from the emergency room.  Always know where you should park with respect to the location of your local hospital's emergency room.  Trial runs in the car are a good idea before you find yourself in need to really seek emergency services.

I never figured out why 911 and an ambulance ride was not my first choice.  Perhaps my decision to drive was a subconscious decision consistent with stubborn attitude of doing everything myself.  Family 911 drills, without of course actually calling 911, are also probably a good idea.

The walk to the emergency room was slow and painful.  So many times I wanted to sit down and rest yet I kept walking, knowing that as soon as I sat down the end would come.  Turning another building corner the bright neon ER lights loomed just ahead and as I approached the automatic sliding doors a wave of relief flowed around and over me.

The nurse stationed behind the desk later told my wife I appeared white as a sheet, and barely speaking, clasping my chest, telling them I suspected a heart attack.  Memorial Hospital's staff on call that night included a young surgeon, Dr. Nathan Bates and his team and a CT scan quickly told them about my acute root to foot aortic dissection.

Hospital staff called Judy and began prepping me for surgical replacement of the aortic valve with a St. Jude mechanical device and then removal of the dissected ascending aortic arch and replacement with a Dacron graft.

During the eight hour surgery my heart was put on bypass and body temperature lowered to thirty four degrees fahrenheit.  Any doubts of life after death vanished once the operation was complete and deemed a success.  As I lay practically lifeless on the operating table my spirit, in very tangible form and awareness floated in the air above the doctors and nurses.  Cloaked in what I can only describe as immense love and comfort, the thoracic surgeon's procedure below was clearly visible.  I wasn't carrying anything with me that I'd accumulated in life and I wasn't even concerned much too much about anything I'd left behind.  I was very focused on the present moment and the pervasive loving warmth filling my new body.

Yet I only had a disconnected interest in the operation on my human body.  I was more curious of my new body, a spiritual body but one that was very real.  Immediately I knew that I'd be returning to the prostrate human form laying on the table.  Floating in the room my spiritual body kept bumping into the ceiling and floating back down towards my human form until I could move back away once more.  Hovering above the surgical team a brief wave of disappointment filled my thoughts.  Continuing the afterlife journey was not an option for me.  If I was supposed to die then I would have been able to move through the ceiling and on along my journey.

The warmth of eternal love held me throughout the entire procedure and in the end stood in stark contrast to waking up in the recovery room only to throw up volumes of fluid all over my chest.  Barfing never felt so good.

Subsequent to the initial operation my Dacron graft became infected with some type of unidentifiable fungus and I experienced holes in my chest, wound vacs taped to my chest, months of a pick line and daily IV antibiotic and antifungal sessions, additional surgeries to remove infections from around my heart, a permanently unstable sternum and an inoperable dissected descending aorta reaching into my renal and iliac arteries down to my foot.

We think we are boss of our cardiovascular system and for that matter most all other aspects of our physical body.  I know I did.  We imagine we can tell the body to do this and do that and to regenerate each time we are dietarily or lifestyle abusive.  We wrongly assume complete control over our body and think we know all things about how our bodies function when in fact only our sensory processes gives us a limited window of knowledge into our human functioning.

Our arteries may clog completely while we are unaware of the damage until an ischemic event.  Cancer may inflict many of our organs before we know of its' presence.  Alzheimer disease may establish a foothold in our brains many years prior to us exhibiting the first symptom of dementia.  On the grand scale of body knowledge, most of us really know very little.  'Out of sight, out of mind' has become our officially unrecognized health status motto.

All the diet and exercise improvements I had implemented over the previous few years were noble attempts to heal.  But the were too little and way too late.  I really wish I this blog had been available to me fifteen or twenty years ago.  Had I listened to my own words I plant to share here over time then maybe some of the pain could have been avoided.

Ted Rhodes, RN, was my home health care nurse and the person who saved my life by realizing the throbbing red knot on my chest was a serious infection and sent me to the surgeon's office.  Ted, though I'll never know if he seriously believed I'd heal or was just trying to be encouraging, one day told me that I'd be at the beach with my one more after healing progressed.  His much needed encouragement that day was one of the reasons I began to believe in the possibility of healing.

Today my hope is that by freely sharing the experiences I encountered, I might inspire others to embark on a an adventure of cardiovascular health.  Otherwise preventable chronic illnesses can turn an enjoyable middle or elder age into absolute misery.  Integrating a new life adventure, good food and peaceful spirituality can transform pain into supple, youthful bliss.   Follow my progress here and feel free to share your experiences too.  Life can be way too short.  Cardiovascular health is so important.

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!