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.

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.

Marfan Life, It's 1:41 AM and…We Need More Connective Tissue Awareness

My right leg hurts too bad to sleep.

My connective tissue tears easily and since I am on warfarin, many times I will develop internal bleeding

I can't lie still.  1:42 AM now and the blue iPhone light reflects across the room.

I worry that all the tossing and turning will wake my wife.  But I worry about that almost every night.  I am hoping Marfan Awareness month helps with others understanding crazy connective tissue life.

My leg hurts and I cannot find a good way to lie on the bed.  At least it does not hurt as bad as it did two weeks ago.

All I did step down off a curb a little differently.  I didn't fall.  I just stepped forward in a slightly unusual fashion.  All this pain because of one mis-step.

People wonder why I am hurting so bad.  Heres to hoping Marfan Awareness month helps with others understanding crazy connective tissue life.

Two weeks ago the nurses at the ER could tell something was up.  Perhaps my dark purple upper thigh looking like a beet was a clue.

"I tear", the words automatically roll off my tongue now when I go to the ER.  This is the second ER visit within a year for a major internal bleed.

The nurse's forehead wrinkled as he studied my leg.  I wish my wife, Judy wouldn't have that worried look on her face.

"I tear and I am bleeding out internally.  I've got blood all in my stool", I had said,  partially repeating myself and rubbing my swollen leg.

Yes, I hoped the hospital ER would be able to quickly determine the extent of my internal bleeding, tell me I was not going to die and patch me up and send us on our way.  My wife had a nature photography presentation the next day and I was so proud of her marvelous art.  I didn't want a stupid ER visit to ruin the show.

But that was two weeks ago and the walnut sized hematoma in my upper thigh muscles now felt hard as a rock and hurt like heck.  And the iPhone clock told me it was 2:10 AM.

I still can't find a comfortable position to lie in.  And the light blanket keeps pulling off my feet.

Why did I take my socks off earlier?   I know good and well the sheets will be off my feet sometime during the night.  Best to leave socks on when you are a long Marf.

How come sheets and blankets are most always made for short people?  Marfan life requires sheets with adequate long length.  And its tough too to find long enough pajamas.

My connective tissue obsessed mind swirls with unending strange thoughts and it is 2:20 AM.

"Are you already awake?" Judy asks.  I feel guilty for having woken her up with my tossing and turning and trying to arrange the sheet back over my feet.

Perhaps more Marfan Awareness could help those we sleep with understand just how much a hassle nighttime can be for those of us with connective tissue problems.

"I think I will type on the computer", I whispered and gently swung my pained leg out of bed.

Tramadol was supposed to help.  My primary care physician had taken one look at my purple thigh and said, "Ouch".  I can't take opioids because they constipate me in a really, really bad manner.

"No NSAIDs for you with all that bleeding", Doc had said.  "You have a torn muscle or ligament and we aren't going to go poking inside of you to see what it is.   Last thing you need is more prodding around inside of you.  I suppose it will take about twelve weeks to heal.  I'm giving you a script for Tramadol.  No need to come back unless it gets worse."

Oh God, I wish doctors really understood what a life with weak connective tissue is really all about.  Maybe Marfan Awareness month will help with doctors more accurately putting together pieces of the connective tissue life puzzle, I thought.

The Tramadol really helped with the hematoma pain.   Unfortunately my PT INR subsequently shot up to over seven.  Even more alarming was the fact that my stools had turned to bloody liquid.

But that was two weeks ago and now its 2:35 AM and I can hear my wife breathing as she sleeps once more.

Living with someone who has Marfan syndrome is difficult, I know this.  I wish she did not have this hard health care giver journey to endure.  Even so, I am really really glad she is my life partner.

Judy has unselfishly given the best of her life to help me through all kinds of crazy emergency aorta surgeries, heart infections and the emending day to day connective tissue problems I encounter.  And I do the same for her with her non-Marfan health issues.

But I hope this Marfan awareness month can help her and others like her understand even more so the craziness of connective tissue life.

Awareness is crucial.  It is now 2:45 AM and I hurt with that danged leg pain.  But I don't dare take another Tramadol or any other pain killer for that matter.  Where are the pain management experts who understand the relationships of artificial heart valves, anti-coagulants and pain killers?  I need to find one!

Yes, I know now the literature supports Tramadol as being compatible with PT INR management.  Looking back on my bleeding episode though, I probably should have been instructed to monitor my PT INR carefully with the addition of the pain medication.

Perhaps Marfan awareness month will help with more doctors understanding connective tissue life and pain management.

The day of my ER visit last week, Judy was supposed to have had a photography showing of some of her fabulous nature photography art.  She especially has a magnificent eye for wading bird and wildflowers.  We both love Florida nature so very much.  Our daily walks are therapeutic on both a spiritual and physical level.

"I'm going to postpone my presentation", she told me.

"No!" I replied.  Last thing I ever want as a tearing Marf is to be a burden for anyone.  "I'll be out of the ER in time for you to do your presentation!"

"I just can't", she said and shook her head.  "I can't speak to a group of people not knowing if you are going to live or die," Judy continued.

This was the last thing I wanted to hear.  Why can't people understand that Marfan Life is all about not knowing what is going to tear or break next?  Why do I have to be the one to get in the way of everyone else's lives?

I just wish they'd understand.

Then it hit me.

As I was writing this post.

At 3:04 AM, Wednesday morning EST.

This whole February Marfan Awareness month is not just to help others understand crazy connective life issues.

I am sure Marfan Awareness month will help those primary care doctors and pain management specialists and nurses and ER doctors with understanding their connective tissue challenged patients like myself.

Maybe even the sheet and blanket and pajama manufacturing companies might read this post and start offering a line of extra-long products (affordable ones please).

And there are so many others out there Marfan Awareness month could reach and ultimately improve the lives of us Marfs.

But what hit me just a few moments ago was the idea that Marfan Awareness month efforts should not just be directed at the 'others'.

Yes, others need to understand our connective tissue problems.

But I need Marfan Awareness as much as anyone else.  I need to know there are others whose legs and back and arms hurt at 1:41 AM.

I need to know that my feet are not the only feet protruding out from under too short sheets.

I am not the only one in the ER because I tear so easily and yes, my wife and family do worry about me  much more than a photography presentation.

Marfan awareness is not just about making others understand how they impact us.  Marfan Awareness is also about helping us understand how we fit into a non-Marfan world.

Like the short sheets and floody pajamas and PT INR or pain management, connective tissue life integration can be difficult to facilitate.

I need to stay involved with our local chapters and participate more in support group activities.

Marfan awareness is not just about 'the others', its about 'us Marfs" too.

I am so appreciative of all my supporting family and friends and physicians and the Marfan Foundation.  I hope we all can continue to be more aware of how we 'connect' in this crazy connective tissue life experience.

Here's to more connective tissue awareness for us all!

Connective Tissue Challenges, Learning to Live All Over Again

Someone started a rumor long ago that says you can't teach an old dog new tricks.  I say, "it depends on if they have a connective tissue disorder and how bad that dog wants to live".

Living with a dissected aorta - my descending dissection #Marfan

My present mission is to stay alive at least long enough to see my two teens able to support themselves and to enjoy life with my wife, Judy. That is pretty significant incentive.  I sure hope this is possible but I have to be honest and say that each day I wonder if I am going to be able to make it or not.  There are so many common, every day things that can seemingly push me towards the final edge.  

Living with a dissected root to foot and up into my kidneys, aorta and a HUGE false lumen that floats around inside the main blood vessel, requires me not to strain, lift or otherwise exert myself.  Surprisingly this is much harder than it sounds.  You'd think, OK, just don't lift or strain too much.  Yeah, right.  Open this peanut butter jar for me.

It is not the hour long weight lifting outings or intense home remodeling or green roof construction activities that I've already given up that present those unexpected aneurysm dangers.  Rather, it is the little things in everyday life that I have the most problems with.  Unfortunately I don't find out about just how I am hurting myself until after the fact and my chest starts becoming tight and my aorta begins hurting again.

After living with a Dacron graft and St. Jude valve for two and a half years I am still learning new tricks.  This dog has finally come to realize that there is no going back to the old way of type A living.  Today's life is centered around measured movements, cautious exertions, decreased activity duration and cardio instead of anabolic muscle approaches to exercise.

Tightness, aching and jaw, chest and back hurt are all still fresh memories haunting everyday life.  I never again want to experience the unzipping feeling radiating down my body as the intima wall separated from the other two aortic layers.  Today, I have very similar pain events occur out of the blue, though nowhere near as intense, whenever I put stress on my cardiovascular system.  So I have to learn to avoid cardiovascular stress of any type.

But these 'instigating events' sometimes are activities one would never expect to be potentially responsible for a future dissection or aneurysm.  Simple stuff, like taking the garbage out or moving a piece of furniture (hey dollies were invented for a reason).

Today I know I can no longer jump up from the couch and hoot or holler when a sports game is on television.  In fact, after the last few college football seasons and the Florida Gator's not so stellar performance (but hey, Go Noles!), I do not even turn on the television to watch games.  In fact I do not turn on the television at all.  There goes a bunch of cardiovascular stress out the door.  Now I blog.  New trick.

Today I must watch myself when sneezing or straining on the toilet.  Too many people have kicked the bucket on the loo; even some very famous people.  Thanks to daily organic psyllium fiber all things run much more smoothly now.  Yet the sneezing can't be avoided.  I just hold my chest tight when sneezing though I am sure that clasping my chest tight is not going to hold my pulsating internal supply hose together if the sneeze is big enough.  

Today I can't work on my bike like I used to be able too.  One of my pedals needed replacing and as I tried to loosen the proper bolt with an open ended wrench I felt that old familiar pain returning.  Stop, take a deep breath and think.  I must learn new tricks.  I can not strain like I used to.  My teenage son though can help me and the use of cheater bars and levers can make the job much less stress intensive.

Today I can not jerk on weed eater or lawn mower starter handles.  Upper body rapid upper chest movements while straining are a trigger for dissection according to Dr. Lars Svensson.  But this old dog here has found that today's electric weed eaters and lawnmowers are, especially with their 56V batteries, amazingly capable, lightweight yard machines and best of all they just require a button's push.

No more working on the car, but it is time for the teen son to learn anyhow.

Those darn random firing red-light camera flashes used to send adrenaline surging through my body causing the blood pressure to soar when I was driving.  Now, without a driver license I don't have to worry about keeping my eyes on the road.  No more gripping the wheel so hard the veins on my neck stand out.  No more yelling un-intelligible, sloberish words out the car window and hitting my head on the frame as I offer up evil hand gestures.  Much less stressful to walk or ride a bike.

No more scuba diving into caves.  Sometimes I wonder about the summer before I dissected.  I was diving in a tiny vertical cave shaft well over one hundred feet below the surface with hardly any room to even turn around.  Or those times I would free dive down into the west sink in Promise Sink, swim through the connecting cavern about forty feet down and come back up in the adjoining east spring.  Or those times in Peacock Springs where we'd dive straight down through fifty feet of zero visibility algae blooms to finally break out into a crystal clear basin full of alligators resting on the bottom of the cave opening.  What a rush!  And my aorta held.  Lucky I did not dissect underwater.  Today I lay on the beach and watch flocks of prehistoric pelicans glide seemingly motionless overhead, a much easier cardiovascular activity than equalizing pressure in one's head underwater or becoming tangled up with a dive partner scrambling away from large underwater reptiles.

No more eighty pound bags of cement.  No more forty pound bags of compost.  No more ten pound bags of ice.  Doctor says two pounds max.  Ugh, this is a challenge.

But the cheater bars (a long hollow piece of metal used to gain leverage - not a place to find alcoholic beverages or wild women), levers and teen help works.  The teen's echo shows dilation has started though, so the teen help will be limited and cautiously monitored.  This old dog is learning some new strategy.  Will it be enough to keep me alive?  I think where there is a will there may be a new trick.  

Connective tissue challenges might cause all sorts of hullabaloo but they also present all kinds of opportunities for learning to be creative.

So for all you out there with special challenges, Be Elite.  b31337.  We are the magicians.

Getting Through A Connective Tissue Disorder Day

My embolistic hit brain needs things laid out clearly or panic, vertigo and unending uneasiness set in.  I sit down then, put my head in my hands or lay down under the window and become immobile.  At least I want to.

My Connective Tissue Disorder Life Day-Bloom

Many times the lack of clarity leads me to wander around the room or house through a confusing mental fog.  I need structure and order during my day.  I need an easy to understand roadmap that helps me keep moving forward, something to held avoid the confusion stroke related dyslexia and ADHD brings.

So when I lay awake at night staring at the stars out the window, I see my day and my week and the year in the form of a big circle, like a clock, probably due to looking at clocks now for fifty seven years.

Even fresh ground coffee in the French press does little to clear out the fog.

But pictures so help.

Since I love flowers I thought I would take the 'clock' concept of a day and turn it into a blossom, a bloom, a 'day-bloom'.

I have printed my day-bloom out and attached to the kitchen refrigerator.  The kitchen refrigerator is an important place to me because it is where I usually end up after wandering around wondering.

When I open the stainless door a flood of bright light rolls out of the cold ice box.  I usually stand still for several minutes mesmerized by the potential snacks and cold air enveloping my feet.  Then the thought of sardines in the pantry and the humming sound of the refrigerator compressor kicking in shakes me out of my zombie like state and I shut the door.

Now my day-bloom will be there, right in front of my face to remind me of what I think I need to be doing.

Yesterday while riding in the vehicle with a friend I kept falling forward and couldn't keep my eyes open, probably because events of the last couple months have given me an excuse for not focusing on my day-bloom schedule.  Remembering the doctor has told me that my number one job was to 'stay alive', I am going to try and stick to consistent physical therapy, exercise and rest.

Using pretty pictures is more fun than a bulleted list.

I can't even seem to keep my eyes open to write this post and it is only 10:20 am.  Struggling with the side effects of the meds controlling a barely beating but loudly beating heart when it is behaving is tough.

Now, life seems like  it may contain only a few really important things; like family, friends and Love.

My day-bloom reminds me of this.

Even when I do not have my reading glasses with me.

Because I can see the hearts and the smiling sun.  And one more smile for me.

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.

Connective Tissue Disorders, How Aortic Dissection Happens #Marfan Syndrome

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Posted with Blogsy

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.