Friday, January 31, 2014

My Distal Aortic Dissection, NIH Treatment Discussion

I added a photo of my existing dissected distal aorta to the upper right hand corner of my Marfan blog here, to remind me everyday is an important day in the battle against hypertension.
My dissected distal aorta - existing unrepaired
If you look closely at the above photo you will see two ovals in the center of the echocardiography.  There should only be one oval.

The top oval is the false lumen, or dead end ripped out channel.  The bottom oval is the true lumen or open channel in the aorta.

The line between the two channels is the intima tear - of inner lining of my aorta, ripped out from the aorta wall and 'floating' in the middle of the aorta.  This phenomena is know as a 'dissected aorta'.

The vessel walls are much weaker than a normal, non-torn aorta and are subject to rupture or aneurysm.  As stated in the article published in the U.S. National Library of Medicine's National Institutes of Health, aggressive hypertension treatment is about the only non-surgical treatment option for this condition.

Surgical replacement of the distal aorta is highly risky.  Stents are not accepted as a treatment option at this time.  My cardiologist says 'we don't want to open a can of worms', and one of my Facebook Marfan Syndrome friends recently did not make it through her descending dissection repair in Denver this last month.

I am placing this photo here to remind me of not stressing, of remaining calm, not worrying, not picking up anything over two or so pounds as my doctor recommends.

Our body is amazing.  And it continues to function sometimes even when the 'experts' shake their head and wonder how.

Wednesday, January 29, 2014

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.

Monday, January 13, 2014

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.

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…..

Wednesday, January 1, 2014

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, 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?