Tuesday, April 25, 2017

Who Is The Best Cardiologist? You Know Who.

You know who is your own best cardiologist.
Who is the Best Cardiologist? (You Know Who)

Now hear me out as to why I believe so.

First of all there aren't many cardiologists in the world who have the number of hours under their belt dealing with dissection and aorta surgery as You Know Who.

This week my local cardiothoracic surgeon who will tend to me one day when (not if) my false lumen  blocks off blood flow to my vital organs, said "in a typical year we see about as many dissection patients as there are fingers on my hand."

As a side note I'm betting I might out live him.  We'll see.

When he first started seeing me as a patient I think he said something like, 'you aren't supposed to be alive'.

This week he asked if I'd type up a support group curriculum.

I've been through a lot of cardiologists.

They are all really, really smart.

They are smart about what they are familiar with.

So when a good friend posted the other day about recommendations for an expert cardiologist who understands connective tissue challenges on top of dissection, she inspired me to write about You Know Who.

'I'd travel anywhere in the U.S,' she suggested.

I know the feeling.

Lets see.  To begin with my cardiologists were those who I thought might shed some light on what just happened to me after I dissected.

Maybe they could tell me how I could heal.  But they did not.

Maybe they could tell me how long I'd live.  But they would not.

Perhaps they'd prescribe the right pills.  But after years of trial and error it was You Know Who who begun to figure out how I respond to various meds.

Perhaps they'd know when to operate again on my descending dissection.  You look pretty stable they'd say.  Let's discuss that next year (fine with me).

Maybe they can help me resolve my PVCs, PACs and occasional bigeminy.  Lets do a two week halter monitoring session that ends up telling us not a whole lot.

Maybe they could do this or that or perhaps I am expecting just too much from my cardiologists.

Its scary to think that maybe they really don't know what the hell to do with someone who is ripped up into the neck and down into kidneys and legs.

Except prescribe Amlodipine, Metoprolol, Losartan, Aspirin, Coumadin and statin pills.  Oh yeah, don't forget the annual dose of abdominal and thoracic CT radiation.

Sorry you have traumatic stress disorder.  Can't do much for that.  See your primary care doctor.

Actually all of my cardiologists have helped me along my dissection life journey but in a way I'd never expect.

No they weren't my go-to Guardian Angels.  They weren't the ones with the dissection life answers I'd been seeking.

But they were my teachers.

My cardiologists have taught me to learn everything I could about aorta health, aneurysms and dissection.

They taught me to look for answers.  Answers found not from them, but to look to You Know Who.

Yes, those who deal with dissections and aneurysms and stents and mechanical valves ten or twelve hours a day are a great resource.  Yes they are dispensable to our survival.  But they never had the answers I was seeking.

Ended up You Know Who had the answers instead.

There are lots of You Know Who's in my world, and most of them share a commonality with me.  They are survivors.

Instead of spending ten or twelve hours a day observing and repairing aortic aneurysms and dissections, the You Know Who's live twenty four seven with the same shit I live with.

You become an expert after years of working ten or twelve hours a day with dissection patients.

You are an expert's expert if you live with a dissection for just a short time.

Really now, I just couldn't find a cardiologist who could give me the answers I could 'buy'.

Lets see.  Except for You Know Who, who could really relate to:


  • hearing the emergency room CT tech loudly holler 'Oh My God!' while laying on the sliding platform that keeps whispering 'Breath', 'Hold Your Breath', 'Breath'.
  • listening to the surgeon on call tell you about slim chances
  • vomiting all over your sewn up chest as you wake from an aorta replacement excursion
  • knowing something was bad wrong a week later as fever rose and chest swelled bright red
  • having the thoracic nurse practitioner frown but shake her head and send home with antibiotics
  • going back a couple days later with puss building in my chest just to have the P.A. slice open the swollen chest with a scalpel but without pain killers just to quickly relieve pressure
  • enduring a second open heart to clean out a green fungus covering aorta and more
  • having the doctor say 'renal failure' and something about a 25% E.F. heart output.
  • hoping the infectious disease doctor knows what he is doing with long term IV antibiotics and antifungals 
  • watching PICC lines installed into arm with tube threaded up near heart, more than once
  • learning how to thread hypodermic into PICC line with one hand and then watching Vancomycin spray out of the IV bag coating your wife care-giver
  • wanting to strangle home health care nurse who plasters so much latex tape over wound vac on chest and then pulls every hair out of chest when changing
  • wondering why memory doesn't work like it used to with all the statins and other meds
  • listening to your neurologist talk about embolistic events and strokes
  • stumbling across the phrase 'pumphead' then reading how the machine saving lives causes strokes
  • losing driver's license when someone thinks driving is not smart for a survivor
  • navigating life as a pedestrian for years as I appeal drivers license medical revocation
  • being cuffed and thrown to the ground, threatened with tazing and guns as I walked to the grocery store simply because I apparently looked like a criminal
  • hearing cardiologist after cardiologist say 'sorry, can't support you driving' and the cardiologist's receptionist say 'we really didn't think you were going to make it'....
  • and that's just the tip of the iceberg.
So I don't really blame cardiologists for not having answers.

And today I don't look to my cardiologists or my cardiothoracic surgeons for answers.

Today I look to You Know Who for answers.  Yep, me.

I did finally find some doctors in Miami at Cleveland Clinic who helped me regain my drivers license.  And if I need to have a planned aorta surgery I'd have them do it.

But for the day to day life stuff, I just don't have the energy or time to regularly make the drive across the everglades to see them.

And so I've come to realize that the best cardiologists are those in my neighborhood who at first I'd shaken my head about.

Yes, they are the ones who said 'you aren't supposed to be alive'.

Now they listen to me.

Because I've learned from You Know Who.

I've learned as much as I could from the 'school of experience (or hard knocks as some might say) and from others who are You Know Who's too.

Like when I finally figured out that if my heart rate drops into the mid thirties and I start getting cold then cutting back on metoprolol from 200 mg per day to 100 mg per day helps bring pulse back up to the mid 50's.    And after discussing with my cardiologist in detail and explaining to him what I wanted to do by adjusting my beta-blocker dosage, he agrees.

Now I'm in control.  Fast pulse of heart flutters?  Take more beta-blockers.  Pulse dropping too slow? Cut back on beta-blockers.

Same principle with warfarin and statins for other cause and effects.

Of course never try adjusting meds without consulting with your appropriate doctor.

But once I explained what and why and they agreed, I now had a seriously comforting level of control.

Control in a life of not-knowing what is going to happen is important.  Friggin crucial.

After five years plus of first hand experience living dissection life and input from hundreds of other You Know Who's who are living the same life and learning similar dissection hacks (thank you all who live with dissections and share your experiences), I finally am finding answers.

And I don't have to travel far across the state or nation to find a cardiologist who really understands.

Because I am guessing there aren't but a handful of cardiologists who are dissection survivors themselves.

And honestly, it takes a dissection survivor to really know what the questions are, much less the real answers.

I love you cardiologists.  I love you care-givers.  You both are so very special.

But a torn vessel that holds life precious blood safely in a holy channel can only be understood by another dissection survivor.

So my cardiologist today has an office less than a mile from our townhouse.  And he is so fascinated now with connective tissue dissection news that I have overload him with and interested him in that I really feel comfortable with him now.  My aorta-centric passion has been contagious.

He listens today.  And asks questions.

If I suggest something he considers it from a perspective I know what I am talking about - from an educated patient perspective.

He then frames the situation with his medical training.

And I come away satisfied I probably have the answers I was looking for.  I came up with them and my doctor fine tuned them.

The best cardiologist is really not far away.

Actually in my own home.

Yep, the best cardiologist for me is You Know Who.

We always must be our own best advocate.

Educate yourself, tap into the marvelous support available from others who are dissection survivors around the world.

Inquire, learn and share.

Only you really know what needs to be done.

And its time to share.  You have the answers.  Others need them too.




Friday, April 14, 2017

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'!







Tuesday, March 14, 2017

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.




Wednesday, February 22, 2017

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.







Sunday, February 12, 2017

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





Monday, December 19, 2016

Mr. Marfan Man, Do Not Pass Go, Do Not Collect $200, Go Directly To...

Each day is another opportunity to move across life's board game.  More likely than not I end up drawing the Go Directly To Jail card with each roll of the dice.
Marfan Syndrome slows me down but doesn't stop life from happening. #Dissection life

Of course in my Marfan dictated life the 'Go Directly To Jail' card bespeaks of oft unexpected maladies.

Physically and mentally there are so many challenges slowing our daily struggle towards the Park Place destination.

Constant setbacks are so frustrating, especially when I have adopted a healthy vegan and sometimes pescataria diet.

And I walk daily, do yoga, stretch, practice deep breathing, spirituality and all the 'right' things my body and brain need.

All the while I see young and old others drinking colas, eating fried fast foods and even doing nicotine seemingly unfazed health-wise.

So what did I do to deserve this?

Nothing, of course.

Its just I am challenged by a connective tissue disorder.

And living with a dissected aorta.

The healthy diet and faithful gratefulness and exercise does pay off I believe.

But this Mr. Marfan Man can seem to be making progress when that nasty little card is flipped over.

Go Directly To Jail can translate into a seriously pulled leg or thigh muscle, one where I honestly cannot even walk one step without feeling like I am going to fall to the floor.

Or the card can mean a huge hematoma, one the size of a grapefruit across my upper back, black and blue.  Purple too.  The hematoma disappear over time after the dark bruise slowly runs down my back or sides.

The Jail Card can wake me up in the middle of the night shouting in tempo to my mechanical valve, refusing to let me fall asleep for hours, manifesting itself in bad PTSD episodes.

Probably the most appropriate action the Monopoly board game maker could do would be to rename the 'Go To Jail' card 'Go To The ER'.

'Go Directly To The ER, Do Not Pass GO, Do Not Collect $200' fits the bill perfectly, especially for those strange, indescribable pains shooting through my chest, head, arms,  or legs.  The card drawn may also mean an episode of bad vertigo or the seeming inability to breath.

However after five years of living with dissection and the Marfan diagnosis I've begun to realize that its actually good to expect the 'Go Directly To Jail' card each day.  Its not that the Jail Card is good.  It is however important not to be caught off guard so frequently.

That way I'm not so surprised.  Its a little easier to deal with the frequent physics and mental setbacks.

You see now I know my setbacks are results of nothing wrong I've done, they are just the random design fluke that defines who I am.

I am special and different than most.

Thats bad and good.  Bad because I have to suffer, most of the time with unexpected painful and debilitating physical injuries and limitations.  Good because I am aware of this potential for physical injuries and that I am part of a broader group of people who want to spread awareness of 'connective tissue issue' life.  With knowledge we can overcome.

Being aware I have an advantage over others for long term survival, despite.

I know each day I have to be on my best survival game.

Living a Marfan aware life has rewarded me some decent benefits; such as lower, life extending blood pressure, healthy eating nutritional benefits and the positive impacts of my spirituality and gentle physical exercise, and focused health care.

But those Go Directly To Jail cards still keep popping up.

But now they don't freak me out quite as bad.  Because I expect them.  And I know I probably will make it through this next challenge.

I am grateful to The Marfan Foundation and the support groups across Facebook.  The people behind these efforts deserve kudos.

In the meantime I am quite over being surprised by the Go Directly To Jail, I mean 'Go Directly To The ER' card.  Its in the deck and if I get it I get it and will deal with it.  If I don't know how to deal with the challenge there is someone in the Marfan community who really can help.

Looks like there may just be more 'Get Out Of Jail Free' cards in the stack now.

Happy Holidays!






Saturday, October 15, 2016

Aortic Dissection, Connective Tissue Issues; Coping With All the Information

I've usually way too many apps open on my phone and am surprised to see how much faster my iPhone runs when I close them all except for the one presently in use.
Corkscrew Swamp hiking for Aortic Health
"Be here now."

More relevant to me than the app analogy is an image of paper file folders scattered across a desktop, flung open, stacks of typed or handwritten pages lying everywhere.  A jumbled up mess of a lot of information is not only confusing but disheartening too.

"Peace be still."

Life with a dissected aorta and Marfan Syndrome (the connective tissue disorder in part responsible for my torn aorta) and with chronic kidney disease from multiple open heart surgeries is a challenge not only on the physical limitation front but also because of the massive amounts of health information I must process daily.

Will this particular food raise your INR or drop the INR and cause a clot?

What about the bleeding an activity might cause if I get bumped or scraped?

What will I be doing when its time to take my beta-blocker that makes me want to fall asleep?

How long do dissectees usually survive?

Daily the questions fill the desktop of my mind like pages from the scattered, jumbled files or too many open apps.

My solution lately is to imagine taking a break and neatly filing all the paperwork and files back into the file cabinet in my back pocket.  Except for the one file I am using here and now.

Sometimes I switch to the app analogy and close all the open apps in my mind except for the one I need now.

So if I am driving then all the thoughts of medications, things I need to do, people I need to stay in touch with, my yoga and swimming I have not done for the day, my blog which I have not touched in a year - well all those thoughts disappear and my focus is only on the road and those cars around me.

Which is the way it should be.

Peace be still.  My blood pressure falls back to where it should be.

The people I am with take notice that I am more engaged presently.

And when I practice this mode of information management my chronic depression from living with  these challenging physical conditions begins to subside.

Be here now.

Close the files.  Close the apps.

Try telling yourself "Close the files. Close the apps" next time you are overwhelmed with a barrage of  information, thoughts and ideas running rampant.

Pease be still.

And then I can more easily deal with my "new reality" of living with a torn aorta.

When, in fact the "new reality" I've been reminding myself daily of is not really a "new reality".

Sure my aorta was not torn before my dissection but it was going to happen.  I just didn't know it.

Now, today I know I live with a pre-disposition (and a torn aorta) to connective tissue tears and all the cardiovascular and muscular problems associated with Marfan.

Understanding my dissection life is not a new, strange and unknown life for me is important.

I've always lived with the potential for cardiovascular problems, I just did not know it.  But today I understand.

The difference today is I have all the folders and information now about these chronic health problems whereas before I did not.  I am still the same person physically today yet I now know.

And all this new knowledge is what causes much of my anxiety.

I am overwhelmed and depressed until I remember....

Close the files.  Close the apps.

Peace be still.

Be here now.

And its all ok.

My back pocket file cabinet is especially important when I am writing this blog, or laying down to sleep or working on my art or doing yoga or preparing food or doing chores, you see I close out all the other apps, especially those files of mortality or other unpleasantries and focus on the task at hand.  Life is much easier when the winds of a thousand pages are not constantly buffeting my curly thin hair.

So when the dermatologist's office called yesterday morning and told me the mole they removed from my leg biopsied positive for melanoma, all the files flew out of the cabinet back onto the desktop of my life once more.

For a while I did the whole 'search the internet for answers on how to put the files back to the way they were before the phone call thing'.

Then I realized the melanoma had been there before yesterday, probably long before yesterday and the reality was similar to when I learned about my Marfan Dx.  I now had information I hadn't had before.

So I quickly filed the scattered papers and folders and put them back into the filing cabinet and closed out all those extra apps.

Instead of fretting about the 'M' word Dx we went to Corkscrew Swamp and watched the sun go down and the almost full moon rise.

And I enjoyed my evening.

The dermatologist office has a great MOH surgeon and they are scheduling a surgery to remove the affected skin area.  I'll open the 'mole' file as I have to just like I do with the 'dissection' file.

But I will also keep them closed when in not in use.

Scattered pages, even if they are full of important information, are useless when in an out of focused jumble.

So close out your excess files and put them away.

Be here now.

Life is really a privilege and I so enjoy focusing on each breath, each moment and each day.

Peace, be still.