Sunday, May 12, 2013

Marfan Syndome, Aortic Aneurysm and Every Parent's Worst Nightmare

Every so often Ruairi, our soon to be 16 year old teen son will walk up to me and say, 'Dad, my chest hurts'.

#Marfan Syndrome, Our son, Ruairi's aorta is already beginning to dilate
Those words are every parent's nightmare and though I take beta-blockers to keep adrenalin surges from hitting my heart muscles quickly, the unexpected fright response kicks in.

As a loving mother, Judy my wife has the same reaction.  Sometimes our as our eyes meet, though our facial expressions calm as ever, we share deep, silent anxiety.

Of course we pay close attention to what our teens, Jincy and Ruairi say about any pain.  Aortic aneurysms and Marfan Syndrome issues have taken too many young children who were in or just approaching the prime of their life.

Out of God's grace and the superb medical attention I received in the Emergency Room at Jacksonville's Memorial Hospital the November night of 2011, I survived a complete root to foot, Type A aortic dissection.  I now have a St. Jude mechanical aortic valve and a Dacron graft for an ascending aorta.

One month later my mother has the same surgery and she too has the mechanical valve and Dacron aorta.

Genetics play a controlling role in Marfan Syndrome, connective tissue disorders and aortic dissections.  Unfortunately many people, including medical professionals do not recognize the symptoms or traits of this potentially deadly connective tissue disorder.  However, outreach efforts focused on education the public and medical professionals alike are beginning to appear around the globe today in response to too many untimely connective tissue related deaths.

Living in Flagler County, Florida provides our family with the opportunity to enjoy some of the worlds beaches, renown for surfing, fishing and coquina shell sand and surf.  Marfan Syndrome and aortic aneurysms are not however limited to older people living here in retirement.  Children and youth too are impacted by this devastating condition.  Sadly, several years ago a young twenty four year old local surfer, Tommy Tant passed away in his sleep from an aortic aneurysm in the prime of his life.  Today the Flagler Beach Community celebrates the life of the young surfer with the annual Tommy Tant Surfng Classic event to raise awareness of just how aortic aneurysms and Marfan Syndrome can affect youth.

Additionally, the National Marfan Foundation is a resource for individuals and families faced with the challenges of connective tissue disorders.

Yet young people are still unexpectedly dying, leaving this world at way too young an age.

Today more and more medical institutions are participating in research and providing health care for those afflicted with connective tissue disorders.  Stanford Hospital and Clinic, Boston's Children Hospital, and Cleveland Clinic are just a few of the hospitals with Marfan and connective tissue related disorders specialty units.

Yet just because these medical units are available today, the anxiety of being a parent to children diagnosed with cardiovascular issues doesn't diminish.  Those words, 'my chest hurts', are truly one of every parent's greatest concerns.

I personally know what a severe aortic dissection feels like.  I vividly remember the searing pain and can to this day recall how what it felt like.  Sometimes my experience lulls me into a false sense of security with the teens as I question them when they tell me something hurts.  I think I would recognize a really serious event.  That approach is wrong.  A really serious event would require immediate medical intervention.  And so I always take seriously what the teens are saying about their 'pains'.

Regular medical checkups that include CT scans, echo-cardiograms and other tests to make sure the teen's condition is not worsening are some of the proactive actions we as parents can do.  Of course we all have adopted lifestyle modifications including medications, diet, proper physical activity and blood pressure monitoring.

There are support groups too.  Facebook offers several Marfan Syndrome support groups where one can connect with others facing similar crises.  The support groups are so very valuable a place with respect to learning about available therapies, medications and of course how others deal with connective tissue disorders.  Worldwide, the Marfan community shares knowledge, tragedies, encouragement and success stories.

But heartbreak comes from these support groups too as members pass away unexpectedly, as happened again this week.  Always death is always sad, yet sometimes the lethal aneurysm or myocardial infarction comes at way too young of an age.

If the aneurystic event is severe enough there is not really any time to respond.  I was lucky.  My aneurysm dissected only through the first layer of my aorta's wall, giving me enough time to drive myself to the hospital ER.

Life is full of challenges.  There is plenty of pain and suffering throughout the world, much of it unnecessary.

Understanding the importance of cardiovascular health issues is vitally important, especially to parents of children with connective tissue challenges.

Knowledge is probably one of the most important life saving tools for families coping with Marfan Syndrome.

Those teen words will come.  It is not a matter of if but when.  Develop a relationship with a primary care physician, pediatric cardiologist and hospital that understands connective tissue challenges.  Those words, 'my chest hurts', are truly one of every parent's greatest concerns.  Know what to do when you hear those words.

Life is such a challenge.

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