Sunday, January 5, 2014

Marfan Syndrome Parenting of Teens, My Fears

Being a Marfan parent is hard not only because of the day to day pain of connective tissue problems but also because of the emotional and physical intensity required to mitigate fear of an untimely dissection or other serious health issue in affected children.
Surfer swim-out tribute to Tommy Tant - Marfan Syndrome
I know I am not supposed to worry, but as someone who has a Type A personality I not only worry about my existing dissection, I also worry about what could go wrong in my children's already compromised arterial systems.

My mom underwent aortic valve replacement and has a Dacron ascending aorta.  She had her replacement one month after mine, though unlike mine her procedure was planned.

Over the past two years since both of our surgeries I have talked to her and dad almost every afternoon on the phone.  When I worried about what a loud racket my valve was making, she could relate.  When she fought vertigo, I knew my dizzy spells were not unique to me.  I found much needed inspiration and hope in watching her slow but steady progress with recovery.   She reminds me to check my INR regularly, tells me to avoid grapefruit and checks on new food I mention with respect to blood clotting interactions.

Those days when my St. Jude valve is clicking louder than ever and I worry about it blowing out of my chest, hearing her tell me she is experiencing the same loud aortic noises works better than any medication at calming my fears.

So I want to be the same bastion of security for my children. I don't want them to feel alone in this fight for life against connective tissue health issues.  But learning to be a wise Marfan parent has not been easy for me.

Judy and I do make sure the teens have annual aortic echocardiograms and other health checkups.  The teens are also involved in local community events raising awareness of Marfan Syndrome and aortic dissection and aneurysm issues.  As with any potentially life threatening challenge, awareness has its upside but also can lead to worry in youth.  Finding the right balance with the teens is hard on them and also very hard on us.

There are those who knew of Marfan issues before they had children and decided against raising kids and there are those who made the decision to go ahead and raise children regardless.  I found out about Marfan after Judy and I already had a boy and girl challenged with connective tissue issues.  So now we must make the best of what is and what we are dealt with.  And as most any proud parent would be, I am so very thankful for the our two wonderful teenagers, regardless of Marfan challenges we now face.

But there is a delicate balancing act we must participate in, one with life and death implications.

The awareness activities we encourage our teens to participate in do help them realize the importance of understanding and proper routine medical vigilance.  All this focus on what could happen also sometimes promotes certain fears in both them and in us as parents.

However dealing with fear but having children who are alive is so much better than not knowing about connective tissue issues and loosing a child to a dissection or aneurysm.  My heart cries out for all the parents who have lost children this way.

This past November our teens worked in the Marfan Foundation booth at the Tommy Tant Classic, a national surfing event sponsored to raise awareness of Marfan and aortic dissection.  Tommy Tant died in his sleep at the age of 24 from an aortic aneurysm.  Tommy's mother, Mrs. Tant and his brother Will, and others sponsoring the event have made a huge, positive and important impact on both Ruairi and Jincy, our teens.

The entire event is really a celebration of life and one of the most moving, emotional moments of the event occurs when all the surfers, professional and amateur alike paddle out past the breakers and form a circle in the ocean.

I have been there, done that with the dissection thing and know that even though my descending aorta is still ripped deep down into my kidneys and legs, I am living another day.  However to Ruairi and Jincy the awareness of what could happen has created unnerving uncertainty.

They now come to me periodically, but on a regular enough basis, holding their chest and telling me they hurt.  Yes both are strongly self-contained however try as they may they cannot hide the fear in their eyes.  This always scares me.

"OK", I say.  "Where does it hurt?  Tell me more about how you feel."   I am not a doctor but I am a dissection survivor.  I remember in detail how the tear felt.

Even though I feel I've developed a personal sense of what is cardiovascular related and what is not, I take each complaint of chest pain very, very seriously.  But I do not take them to the emergency room each time these chest pain incidents occur.

Having to make health decisions that the life or death of their children could potentially pivot upon is not easy for a parent.  In fact, it is not something I signed up for.  However it is something now I will without hesitation take on.

I wish on no one the path I went down during my dissection and subsequent heart infection.  I hate the thought that my kids may have to walk the surgery path that my mom and I have both walked.

This Marfan journey has been surreal to say the least and for those of you who have read previous posts here on this blog know of my 'encounters' with this surreal. From guardian angels and near death out of body experience to stunningly vivid, colorful and wildly animated dreams, my life is filled with events I constantly question with respect to levels of reality.

Regardless of just how real some of what I see, hear or perceive is, my world view and my love for the two teens here are inextricably woven together.

Like this morning, when I woke early and wanted to write, first thing I did was clean the grounds out the glass coffee press.  So I carried the carafe out the front door at four A.M. to empty those grounds around the rosemary bushes.  To the left of the herbs there was a little wren holding on to the screen outside Jincy's window, singing a soft,  beautiful song.  I froze. The wren kept singing.  I walked closer and the wren didn't fly away; it kept singing and singing.   The pretty little bird sang for me for until I walked back into the house.  I turned and walked back out to see if the bird was still there.  It was, and singing even louder.

I turned and went back inside.  Shivering from the early morning chill I headed straight for Jin's room.  I know she thinks I am an overly cautious parent because I am always checking on her and her brother while they sleep but I could not see the rise and fall of her chest so I reached and turned on the hall light.  The glare prompted a wince from her sleepy eyes and an "I'm O.K., Dad" half whisper.

I sighed heavily, turned down the light, closed her door and shuffled back into the kitchen to grind dark roast coffee beans.

This morning I fought the fear that the little bird may really have be the spirit of my child singing, leaving her body.  The fear was real, very, very real.  Being a parent is so hard sometimes.

Now, when my son or daughter come to me with a look of concern on their face and hands over their stomach or chest, we do the 'checklist' diagnosis routine.

"Dad, my chest hurts really bad."

I pause, look them in the eyes and ask, "O.K.  Where exactly does it hurt?"

"Here in my chest."  They usually run their fingers up from their stomach to their neck and back down.

First question I come back with is, "Does your jaw hurt?  What about your back?"

"No, it is not my jaw or back," they respond.

"O.K., good", I tell myself.  The thing I remember most about my dissection was jaw and back pain, though caution is appropriate here as these may not be symptoms presented during a dissection or aneurysm.

"Just really hurts deep in my chest," they usually continue.

"What have you eaten today?" We then go talk about the types of foods they've ingested recently.  I key in on any sugary or acidic foods. "Could you have acid reflux?" I may ask.  "What about exercise or sports?"  Sometimes a strenuous activity may be the trigger.  "Could it be a pulled muscle?"

If we cannot quickly pinpoint a food or activity as a potential cause I will take their blood pressure.  Caution is appropriate again here too because unusual blood pressure is not usually an indicator of dissection or aneurysm.  My blood pressure actually dropped during my dissection.  Yet I still like to keep track of their blood pressure and pulse when they start complaining of chest pains.

I'm mostly encouraged when I see systolic and diastolic values of  around 95/60.  Dissection can be, though not always, aggravated by high blood pressure or adrenalin surges.  Knowledge that their aortas are only moderately dilated combined with a low blood pressure reading is somewhat statistically reassuring but still dreadfully disconcerting.

One can never be sure with this connective tissue health problem.

Usually the teen's pain will subside over time.  So far to date, I've not actually taken them to the emergency room for chest pain issues.  And I hope I never have to.  But when all this happens I am participating in a calculated risk with my children's health and lives.

There have been those days where the pain lingered longer than normal.  The teens then expect me to check on them more than once during the night.  And when I do the rise and fall of the sheet tells me they are O.K. for the moment.

The wren outside my daughter's window may really be just a wayward singing bird.  But I still worry and need to make sure Jincy is in her room, sleeping, breathing, and alive.

No matter what happens in our connective tissue torn lives I will be grateful for the friends within the Marfan support community I've found over the past several years.  There are those who have already lost children, spouses, parents, family and friends.  There are those who are presently fighting for their lives and those who have survived the worst.  Groups like The Marfan Foundation offer resources and serve as a much needed safe haven of knowledge from fear of not-knowing how, when or why this serious health issue manifests itself.

But even with all the family, friends and community support, it is still hard to know just what to do when someone who is Marfan challenged, especially when that person is a child comes to you and complains of a hurt in their chest.

I love the mystical beauty of the wren singing on my daughter's window in early morning hours.  I'm sure I'd enjoy the sweet bird call more though if I wasn't so worried about my daughter's Marfan issues.

Personal Marfan challenges are tough enough to deal with.  But I've momentarily beat my dissection.  Being a parent of a Marfan child, though, well that is a whole different song…..

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