Saturday, May 25, 2013

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.




6 comments:

  1. Great entry, Kevin. More people need to know about the risk factors for aortic disease and the signs of dissection. This includes more doctors. And the folks in the hospital emergency departments need to be aware, not only of the signs, but also how to diagnose quickly. Every hour wasted increases the patient's chance of death. Thanks for sharing your story and your words of wisdom.

    Eileen Masciale
    National Marfan Foundation

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  2. Very informative and well written! Thank you for sharing your experience and looking forward to reading your future posts :)
    Brandy B. Evitts

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  3. I enjoyed reading your article very much. I was diagnosed with Marfan's when I was 20. At age 28, I was at work, and experienced severe chest pain that radiated into my left arm. I had trouble seeing and breathing became difficult. The ER doctor couldn't find anything, and admitted me overnight for observation. Once the on-call cardiologist saw me, he ordered a TEE and immediately saw my dissection of the aortic valve and I was rushed into surgery. I now am a proud owner of a St. Jude valve. I've not had many issues since, and am under constant supervision of my cardiologist. I've been learning so much more about Marfan's in the last couple of years, as there wasn't much known about it when I was diagnosed. Your story mirrors my own, and I'm thankful that you have chosen to speak out and educate. Bless.

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  5. Yes I know Marfan Syndrome. I am a 41yr old African American woman who was diagnosed with Marfan Syndromebat the age of 12. I have undergone emergency retinal detachment surgery, mitral valve repair, by the Grace of God I survived an aortic rupture and dissection, descending thoracic aorta graft replacement, and about to have surgery for another TAA. Living with Marfan syndrome has controlled my life all my life, but I refuse to "Give Up or Give In" to this condition..

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