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.

A Marfan Easter In The Children's Memorial Garden

Marfan Syndrome Family Easter in the Children's Memorial Garden

Easter morning was wet, cool and drizzly.  We tried the sunrise service at Flagler Beach but the sight of the bright, easter-egg colored panties under the dresses of the ladies singing church songs and shaking tambourines, being blown up over their heads by the thirty mile and hour ocean winds scooting across the stage in the park, was not what I was looking for.  Give me a hot, black cup of coffee instead.

Easter Sunrise Service, Downtown Flagler Beach

So we went and had breakfast on the pier across the street, overlooking the Atlantic Ocean.  I love the big pond, a metaphor of eternity to me, much like the sky.

Flagler Beach and the Big Salty Pond

Sometimes I wonder if eternity flies by as fast as time here as we know it flies by.  Seems like I was just starting college last month, but it has been forty years.  I still haven't really figured out any of the answers.

The last year and a half has been a challenge in many ways for our family.  I wake every day to the same and new, different challenges with Marfan Syndrome problems, but bottom-line grateful I awoke.  Because I have finally realized life is so precious and tenuous I offer up a prayer of thanks and beseeching every night before I fall asleep.  If the night is to be my last I want to go out in a thankful mood.

Judy has been fighting her auto-immune inflammation and hopefully may have found some relief with an older acupuncturist here in Palm Coast.  Jincy and Ruairi forge ahead with school and social activities, and with all the competition in school I worry about their dilated aortas.  On an aside note, I do not understand why the State of Florida and Governor Rick Scott have upped the SAT and ACT score requirements form high to nearly impossible for Bright Futures Scholarships, without an exception for children also battling a disability challenge.  Jincy has over a 4.5 GPA and Ruairi a 4.7 GPA (A=4.0) because of they study so hard and do so much extra credit work.  Teens that work that hard, with grades that good all the while battling disabilities should have access to the state's lottery money supposedly dedicated to education.

Jincy and Ruairi have their own Marfan Syndrome Challenges, but they forge ahead, unstoppable-like

Jincy has a really good deal at the University of South Florida in Tampa which she has accepted, and I am grateful for her hard work.

Seems like the two teens time in high school just started yesterday, too.  And the grandchildren teens (son and daughter of Judy's second oldest daughter who recently had a serious brain aneurysm) Dylan and Dharma have been here six months in May.  Yes, time flies.

A year and a half has also passed since baby Heidi (Judy's daughter Leslie's baby girl) died.  Leslie and her husband still struggle with so much grief.  Shortly thereafter I hung a set of wind chimes on a beautiful scrub live oak in the Children's Memorial Garden overlooking the intracoastal waterway.  The memorial garden is full of wind chimes placed there by others in a child's memory.

Children's Memorial Garden Overlooking Florida's Intracoastal Waterway, Live Oak Nestled

The wind along the intracoastal is not as strong and brisk as the ocean front wind but still steady enough to keep all of the beautiful sounding wind chimes singing most of the time.  If I was a spirit child, I would go to that salty riverside place just to listen to the jingles and bells and wind softly blow.  As an old man, I also like to go there to talk to angels woven throughout the misty salt air.

But not only are there beautiful melodies sounding of a child's choir in the air, the garden is full of beautiful colors and textures, art and nature combined together in love.

Heidi's New Wind Chime is tied next to her Frog Chime in the Memorial Garden

Heidi's wind chime was showing signs of wear from the breezes and I wanted to get the four teens out of the house and into nature for their daily vitamin D dose.  I'd found a really cool dragonfly chine with just one dangle and figured it was just the chime to attach next to Heidi's original chime.

The four teens and I talked about thankfulness, remembrance, love, kindness, memories and a bunch of other esoteric topics I can't remember now, on the way to the intracoastal.  We also talked about baby Heidi and her parents and her brother.  I reached up and held the two silver hearts hanging on my medical ID necklace, hearts from my Mom.

The four teens, Dylan (Grandson 16), Marfan teens Ruairi & Ruairi (16 & almost 18) and Dharma  (Granddaughter 14)

One thing I don't want flying time to do is allow us to forget.

The teens and I enjoyed out time in the children's memorial garden.  We listened to the chimes.  They stopped and read all of the little memorial stones, pavers and bricks placed throughout the special nature walk.  They even reached up to dab a corner of their eyes when they though no one was looking.

Heidi has two wind chimes now.  Her frog wind chime is still there.  And someone else has placed a big green tree frog on an adjacent limb to look after Heidi's smaller frog chime.

New Big Florida Green Tree Watching Over Heidi's Smaller Green Frog Chime, Down By The Riverside 

And there is a beautiful red star in the next tree over.

Stars in the Scrub Live Oaks

As we left I looked down to my right.  Lovely easter eggs for the children here.

I think, despite my challenged cognitive state and memory, I think I will come back here more often.  There is much to learn from a special place full of native plants, overlooking the edge of an eternal ocean and full of children's twinkling and chiming voices, especially for an old man like me.

Easter Eggs for the Children in The Memorial Garden down by the Intracoastal Waters

Living with a Dissected Aorta and Marfan Syndrome, March 2014 Update

Wow, time flies.  It is March 2014 already.  Here are a few updates on my dissection challenge:

One question for my PCP will be - why does my right foot continue to grow while my left foot is shrinking?

• I have all my Medicare paperwork now and the coverage will kick in on May 1, 2014 - just a little over 7 weeks away.  First time I will have had any insurance since my dissection hospitalization!!!  The two year Medicare waiting period is almost over!  I survived (so far)!
• With Medicare in hand I will be scheduling a visit to the cardiologist for my annual CT scan and echocardiograms to see if my aorta is either continuing to aneuryze or is staying the same diameter.  Of course, I am very anxious about these updates and will post the news as soon as I find out.
• I have been having my INR checked once every month, paying for it out of pocket at our local lab.  I am taking 5mg warfarin daily and last time I checked the INR was 2.2.  Fresh garden greens make up a large part of my diet so I can lower or increase INR with the amount of Vitamin K rich greens I consume.  This is easier than adjusting tablet dosage - break one in half, skip a day, yada yada.  My PCP comes up with such complicated formulas.  I've yet to tell her that I am just doing 5mg daily.  This is enough of a challenge for me as it is with my additional memory struggles.
• I do have a list of questions/comments for my PCP, who wants me to come in soon for my annual checkup, and they are:
• My right ankle/foot feels like it is perpetually about to collapse.  It is hard to walk without my forearm crutch.  It hurts.  I am sure there is not a lot to be done about this and I think it is just part of living with a Connective Tissue Disorder (CTD), but I will tell her about it.
• My right wrist is the same way.
• My sternum is still unstable, even after two years since the surgery. I clicks open and closed when I move, much like cabinet doors opening and closing.  Quite painful.
• My right foot continues to grow and my left foot shrinks. I would think this has something to do with a blood flow imbalance.  My right illiac artery is dissected (thoracic aorta is dissected down into my right leg), so it seems to me that the blood flow to the right leg would be impeded and the right foot would shrink, but ???.  
• I will ask her what to do about the chronic pain.  I hurt daily, usually a 7 to 8 pain level while sometimes reaching a 10 for long stretches.  Do Tens units work?  How do I deal with daily chronic pain?
• My left eye has ongoing bouts with a dark, sideways closing curtain.  Is my retina detaching?
• AFIB and VTACH come and go.  Sometimes my pulse will jump, out of the blue - but especially when weather fronts come through - from 50 to 150.  That throws me to the floor, literally and scares me to death.
• I will mention the LOUD clicking my aortic valve makes but I am totally cool with living with a valve that makes noise, so long as it keeps me alive.
• I have chronic fatigue and find myself blacking out or falling asleep right in the middle of the day sometimes.  My diet is wholesome - I do not eat processed foods usually -just fresh meat, fish, garden veggies, some fruits, no grains, no sugar, low salt (sometimes - I love salt).  I exercise - ride bike and walk and do yoga for an hour each morning.  But I think with a 50ish pulse and a heart output capacity of 20-25% that fatigue is to be expected.
• And other questions as they pop into my mind.
• Our teens have genetic testing and their annual echocardiograms in April.  I constantly worry about the teens, especially when their chests hurt or they feel dizzy.
• My Florida drivers license is medically revoked so I have had to learn to get around on my bike or through rides from family and friends.  I try to keep asking for a ride to a minimum because I do not want to be a burden.  Loosing my license after driving for years was a mental challenge.
• Depression is still a looming specter and I deal with it through prayer and spending lots of time outdoors, especially at the ocean and on my bicycle.
• I self-adjusted my Losartan dosage - doubling it from 50mg to 100mg daily because I could not keep my blood pressure down on 50mg.  After a month or two at 150+systolic I upped the daily dose to one 50mg tab in the am and another just before bed.  This has brought my systolic back down to 115.  I could not afford to go to the cardiologist to discuss, and will discuss with him in May - though we have discussed before.  Please do not do this yourself.  

And that's about it for my life with Marfan and other CTD issues.

Stay tuned for more as it happens!

Living with a Dissected Aorta, Good and Bad News, Depression and Hope.

I am so confused and can't figure much of this Marfan challenge out.

Some days I could care less about my dissected aorta.  Usually on those days the sun is shining and I go for a bike ride, keeping my physical exertion to a minimum but distracting my mind from the torn vessel from inside my chest.

Other days, like today are rainy and generally blah.

Yes, this is all perspective, I remind myself.  I could have been born in a much poorer place of the world and be dead now because of a lack of access to hospitals.  I could have been born during the Inquisition period too, or any other horrid point in history.  Instead I am here today with hot water, electricity, Facebook and grocery store food.  So I should be thankful instead of gloomy.

But I am gloomy.  And it didn't help that I awoke this morning to an article in my email inbox about the FDA approving Medtronic's thoracic stent graft system for people like me living with a dissected descending aorta.

Ugh.  The article starts out in the first couple sentences stating, "Expand Treatment Options for Patients with Dangerous Tears in Upper Segment of Body's Main Aorta".  Hey, my tear goes all the way to my feet so is that dangerous times three for me? Ugh again.

Then the article talks about my condition as a "serious cardiovascular condition associated with high morbidity and mortality in which the upper segment  of the body's main artery has become torn along the innermost layer of the vessel wall."

Using the words, 'morbidity' and 'mortality' is such an endearing writing strategy for those of us living with dissections.

I am so glad the medical profession is working to find solutions.  Without such I'd be dead now of a ruptured ascending aorta.  And I am not wanting to live like an ostrich with my head stuck underground so I won't see or hear (or read) the 'fear' out there.

Yes too I am so thankful for all the blessings in my life.

But chronic pain is real and so is fear of dying way too early in life from a dissected aorta.

I know I am not alone in this struggle.

Hopefully by writing these brief words, someone else out there suffering in a similar fashion will too know they are not alone.

O.K., now that I have had my pity party I will finish reading the article.  Maybe it ends in a note of hope.

Living in the Moment, Family Life and Aortic Dissection

Life is predictably arbitrary.

Dolphin swimming with the current, Mantanzas River

Just when we think we've have mastered our existing set of challenges, other issues capriciously appear out of nowhere, either 'upsetting the apple-cart' or broadening our wisdom, depending on how we want to view these unexpected chapters of our lives.

Yesterday four kids and a dolphin swimmingly through the Mantanzas River's swift currents taught me more about life.

Most days it is all I can do to ponder how I am going to make it through another day with this Marfan Syndrome painted body.  Will my damaged aorta hold out another moon's orbit?    It is so easy for me to become totally absorbed in my own personal health drama.

Don't get me wrong!  Each heartbeat is a gift for me and I know I must be very careful with everything I do from lifting anything over a couple pounds to coping with low pressure weather systems that wreck havoc on my bionic heart parts.

But it is easy to think; me, me, me; my problems.

Compound that with my wife Judy's serious and chronic health issues and I quickly think I am in 'cope ability' overload.

This past month Judy's second eldest daughter suffered a brain hemorrhage, a very serious aneurysm leaving her almost blind.  She is a single mother of two high powered teenagers.  But then again, most all teenagers are 'high powered'.

Judy spent the entire last month sleeping in a hospital room chair, by her daughter's side, every night guiding her through immense periods of pain, helping her daughter navigate the unknown of cerebral bleeding.

I spent the month tending to our two teens, cooking, cleaning, and all the things two parents normally do.

I always say, "my doctor tells me my number one job is to stay alive".  I love this mantra because it allows me to hide inside myself; a place I am familiar with, a place I mostly control and a place where I can placate my self absorbedness.

"It's difficult!" I would proudly and without humility tell those who ask how Judy and I are holding up as we extended ourselves out past our comfort zone.  I loved the attention and I was truly looking forward to her finally coming back to our home once the doctors had stabilized her daughter's bleeding and rehabilitation had begun.

Our two teens are tough enough for two relatively healthy parents to properly raise.  But parenting them all by myself for a short time was taking its toll and I was ready for my wife to come back home from out of town.

Judy's daughter is now recuperating and working with rehabilitation.  Her vision has been seriously impacted, however we are so glad she is alive.  She has a very long road ahead of her with respect to being able to take care of herself again.

Her two teens coming to live with Judy and I, their 'Nana' and 'Papa K', is the best solution until their mom's healing is complete.  Now we have four teens, two teens of our own and two grandchildren teens.

Just the physical logistics of this family integration seemed complicated and overwhelming at first.  But in fact it was a blessing.

Yes the laundry requirements have jumped, as did the amount of food to prepare and the energy output on our part as parents.

But I am learning so much about life from all four teens and also from the dolphin we saw yesterday while out on a picnic down by the beach.

Yes, there are strong currents.  The waters in Mantanzas Inlet are swift.  But if you learn not to fight them, you can go so many new places.  So it is with the many unexpected challenges we face from time to time.

There may be lots more work but there is also lots more laughter, smiles and insights and understanding about our human spirits, like when Dylan ran up to Judy the other day and gave her a really big joyful hug.   Perhaps at the end of the day that is all life is really about.

Yesterday, taking the four teens to Mantanzas National Monument we rode the ferry boat across the river to the fort.  Dolphins swam playfully alongside the craft.  In the middle of the swift flowing potentially treacherous waters, one dolphin rolled up across a wave's crest and loudly pulled in a deep breath.  Our two grandchildren exclaimed loudly pointing, smiling, exclaiming, exuding a sense of wonder and amazement for life in the midst of their deep fear and concern for their mother.

Mantanzas River, Kayaker and Dolphin

Breathing deeply, swimming with the currents, laughing, hugging and living in the moment despite whatever comes our way sure beats that depressing mantra "your number one job is to stay alive".

I think it is time for me to adapt a new mantra.  I am not sure how to word it yet but the thoughts of 'love', 'hope', 'swimming with the currents' and 'living and breathing in the moment' will be what it is based upon.

Maybe my number one job is to breathe deeply, live in the moment and show love and be loved, despite whatever swift currents may drag and pull.  I'll swim with the flow.

Dissected Aorta, Connected Tissue Disorders and 2014

It has been an entire month since I last published a blog post.

Dissected aorta or not, must keep going!

Pain and depression I've battled lately have discouraged me from writing. However I know that I need to keep pushing my partially functioning brain.  Last thing I want is to end up with moss and fungus growing all over and inside my head.

The memory loss and recall issues is so very frustrating. My conversations are sometimes filled with nonsensical terms as I reach into once word filled but now empty grey matter chasms.

And my body hurts so bad.  Last night I had promised others I'd show up for New Year's eve fun.  However after taking the teens out for pizza I felt the imminent crash of pain and exhaustion and had to have Jincy take me home.  Stumbling into the bedroom I fell on the floor and passed out into unaware oblivion until the midnight neighborhood fireworks rocked the house.

There are no words to truly describe the feeling of muscles shredding, pulling apart, unraveling, burning with uncoolable heat.  Curling into a fetal position I lay still for hours, until I fell asleep once more, the rest a gift of mercy from my guardian angel and from God.

It is so easy to feel sorry for myself.  With a dissected aorta, an obnoxiously loud aortic valve, chronic fatigue, the worry and pressures of living life as a disabled person without a driver's license, watching my wife nurse her second oldest daughter laying in the hospital with a cerebral hemorrhage, two teenage additions to our family (who are truly blessings) experiencing the turmoil of their sick mother and having to be transferred to a different school midyear, the physical demands of keeping up with the parenting and energy input requirements of four teens, and all the other 'stuff' that happens to us all, well....it is easy to become self-absorbed in pity.

I can say I will choose the higher path, one with love, care and concern for others above myself.  That sounds so good.  But I know I am only human and will soon fall back into the narrowly focused pit of pain and hurt, because I really do hurt!

But I am going to try.  I will fight off the ... (I cannot think of the appropriate word for the state of being where depression and pain are so friggin bad - maybe - monumental BLAHS) and commit to trying, at least for a day.

So I will be once more posting diet and blood pressure and Marfan Syndrome - Dissected Aorta life notes here on the blog.

Maybe if I can get through today I will do the same again tomorrow.

Yes, I am thankful too.  I do have a wonderful family, wife and children.  That loud aortic valve means I am alive.  My friends are so encouraging.  My guardian angel sits faithfully outside my window and follows me wherever I go.  There is a marvelous organic garden outside.  Sidewalks bring beautiful and adventurous pathways for miles along most of the roads here.  Wildlife and wildflowers inspire haiku and poetry and life's beauty is inspiring.

But the depression of hurt sucks.  I am not going to lie to myself about this.

Just going to focus on making it through today.

It is going to be a good one.  Despite.  Hallelujah, right?

Descending Aortic Dissection, Sonogram and video view of a Marfan Syndrome Leg Aorta

Made it through my periodic sonogram check of the dissected descending aorta in my right leg yesterday at the cardiologist office in St. Augustine.

Technology is so advanced these days and what used to be rather dull, uninteresting paper and pencil diagrams or x-rays are now colorful real-time views of body parts.

Take for example, my dissected descending aorta.  The cardiologist wanted to update the visuals of the dissection down through my leg and look at the vein sufficiency at the same time.  He orders these tests run on a regular basis to make sure the aorta is not in active, uncontrollable aneurysm mode, to ensure there are no clots and keep a check on the status of the torn (dissected) intima layer of the large blood vessel.

Several weeks ago he instructed me to have a CT scan completed on my heart section.  The CT scan is a highly accurate test that provides detailed views of the heart, aorta and other body parts he may be interested in viewing.

However the CT scan did not record images of the aorta passing through my lower abdomen or leg.  So the cardiologist decided to use a sonogram test to view the blood vessels in the lower part of my body.  This sonogram test is generally the same test used that we are all familiar with from visits to the obstetrician when someone is pregnant.  The test uses sound waves to create an image of the body's organs, muscle and tissue.  Also referred to as ultrasound and sometimes echogram, the test can document conditions of the body with relative accuracy.

To run the test, the technologist applies a gel across the area to be viewed and holds the sonogram receiver against the body.  Images are recorded according to the receiver position and angle.

Ultrasound/sonogram test used to check the dissected aorta in my right leg

The tests I had run yesterday included gathering images of my veins, the valves within the veins, my arteries and specifically cross-sectional views of my artery depicting the torn inner layer (dissection).

Interestingly, the ultrasound test gives one a clear view of what the body's blood vessels look like.

My dissected aorta should be a hollow vessel with one open interior diameter.  However the torn inner layer is instead flapping about, having created a false channel similar to a sock, inside the aorta's interior.  This false channel is like a dead end pocket for blood to flow into but offering no way out other than back out through the tear hole into which the blood flowed in the first place as shown in the following video.

In my case the false channel takes up almost half of the aorta's interior flow space, limiting blood flow to my leg and foot. 

One of the reasons my cardiologist wanted to look at the aorta was because my right foot recently started to grow - he refers to the term as swelling - and was wondering if a change to the leg blood flow was occurring.

The radiological technician yesterday did congratulate me on having the 'longest dissection' she had ever seen, and I did take the compliment with pride.  After all, if you have to have a medical issue it is always good I suppose to have a rare condition rather than the standard run-of-the-mill malady.

Anyway,  for those of you who have never seen a cross section of a dissected aorta from someone who is still alive, today is your opportunity to see an example in black and white and living color!

Awareness and education are always critically important and I hope if you haven't taken your blood pressure or seen your cardiologist lately this post may encourage you to do so soon.

In the meantime, the web is full of useful information about the heart, the cardiovascular system, Marfan Syndrome and other connective tissue disorders.  Think Heart Health!

Is My Aorta Still Dilating? #Marfan #Aorta Dissection

How many times during the day do I ask myself this very question?

Flagler Hospital's GE Lightspeed CT Scan Unit

Everytime I reach to hold an object or lift something, my mind shifts to the wondering and questioning mode. Whenever I strain, holding by breath, even for a second I wonder if the now very thin walled vessel will hold or pop. Looking at my teens and my wife the same question flashes through my mind. What is in store for my aorta?

Even watching the few television episodes I waste my time on, the rupture of an aorta is always lurking somewhere in the background. Such was the case when we were watching the season finale of Revenge and the character Dechlan died of an aorta rupture. Another character, Nolan (explaining through tears to Dechlan's brother Jack) referred to the event as a rupture of the 'aorta artery', leading me to believe the script writers had to include the term 'artery' because most viewers would have never asked if their aorta was dilating or unhealthy, and for that matter even know the aorta was an artery, let alone the largest artery in our body.

So every so often my cardiologist also begins to wonder what is happening in my thoracic cavity (chest) and orders a CT Scan event, complete with iodine for better contrast.

I really like Flagler Hospital in St. Augustine. The stormwater pond is a marvelous example of as to how truly green infrastructure should be designed. Birds from around the world come to Flagler Hospital, not of course for the medical expertise but because the big stormwater pond has plenty of shallow water roosts where the two wings can perch, poop and wait for a fish to swim by. I see roseate spoonbills, all types of herons, the goofy anhingas, ducks galore and more. Moreover, the hospital boasts some of the loveliest living walls around, flowering vines releasing their lovely fragrance that unfortunately has to mix with the exhaust of the laundry vents.

Flagler's staff are always so welcoming and friendly (and cute too!). Most are experts at cracking the lamest jokes in an attempt to turn my dour smirk into a smile.

Last time the inside of my elbows looked like a chickenpox battlefield after the nurse couldn't find the vein. Finally they had to get someone else to try. This time the nurse got the IV attached effortlessly and without any pain, making me very happy and keeping my blood pressure at reasonable levels.
Being a Marf is always a challenge for many reasons. The first reason is always having to explain just what a 'Marf' is, the whole connective tissue disorder and aortic dissection thing. I always know at what point in the story their eyes widen to the size of a raccoon's and the words, 'wow, you are so lucky to be alive!' are uttered.

After determining my kidneys are borderline strong enough to handle the contrast dye they tell me about what the IV's iodine rush will like. One never forgets the warm flush as the iodine flows from the arm into the heart down the aorta into the abdomen area, very similar to the wonderful feeling of finally finding a toilet a couple hours after eating a big plate of chopped, steamed okra. I always manage to hold it in, but notice the retractable bed is covered in plastic causing me pause to think of others not so disciplined.

The 'nitro' pill is like afib in a bottle. Because my pulse usually stays around the low 40s, the CT techs always administer nitroglycerin either before or during the test. This time they used a spray, telling me to lift my tongue before they coated the inside of my mouth with a fine mist that makes your heart stand up, salute and sing the Star Spangled Banner.

GE should use a larger font for the 'Do Not Stare at the Laser' warning sign placed adjacent the unit's laser opening. I found myself staring over and over again at the opening and sign trying to read the text. Then there are those ugly little green and yellow heads with noses as long as Pinocchio's that light up when you are supposed to breath or exhale. It is so much easier to interpret to color of the evil grinning heads than understand what the CT techs are saying over the very crackly and way too loudspeaker.

All the stretching and yoga poses I've practiced paid off nicely, for when the CT Techs placed my arms straight out above my head so I'd fit through the CT unit's orifice, the techs did not have to tie down my wrists and the IV stayed in place. The entire test took about twenty or so minutes and, except for the hospital gown flapping about, went smoothly.

The CT Techs and nurses were speechless when I asked, after the test was complete, if I could take a photo of the CT Unit with my Ipad for the Marfan Blog.

Another CT Scan completed I walked down the hall of the lovely hospital, admiring the beauty of the courtyard's waterfall and stunning planted gardens, finding a place to sit and make sure I was not to dizzy to walk out.

My cardiologist will read the scans and compare to the last set of test results and I've an appointment to discuss the findings with him next week.

In the meanwhile, I'll still be asking myself the question, 'I wonder if my aorta is still dilating'. Such is the life of someone who deals with a connective tissue disorder such as Marfan Syndrome.

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Marfan Life, Another Iodine CT Scan Today, Flagler Hospital, St Augustine

Flagler Hospital hospital is just twenty minutes north up US 1.  Today I'll be having another Cardiac and Abdominal CT Scan.

So I wrote a haiku for myself.

super hero's cloak
won't stay tied and so so cold,
blue hospital gown


 Those flimsy blue hospital gowns are just plain unstylish.  Being so tall they never fit me right anyway and make one feel, as Miami Herald humor writer says, "more naked than nakeed".

But I always worry how the iodine dye they use to get better contrast on the CT scan, will do to  my kidneys.  Yes, I do probably worry more than others, but that is my nature.  It doesn't help though that Judy's parents have some friends, one of whom just passed away from iodine dye invoked kidney failure. 

Looking, on the bright side, the cardiologist will, after today's test, be able to tell if my descending aortas dissection has stabilized, shrunk or (gasp) grown in size.

I don't like to think about it growing in size because I don't like to think about bad things and I don't really want to go through another surgery so soon.

There is a medication that I read last night about, called N-acetylcysteine and supposed a twenty five cent 500mg tablet can protect the kidneys from potential dye damage.  I am definitely going to ask about this drug today.  There is also a very interesting on the potentially life saving drug in the National Review of Medicine from a couple years ago, which you can access here.

Updates tomorrow.  In the meantime, I hope the Hospital Gown stays tied today!

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.