Sunday, September 21, 2014

Bicuspid Aortic Valve and Aneurysms - Ten Years Later

Doug in September 2004
Ten Years Ago, in 2004

Ten years ago, on September 20, 2004, Doug Grieshop died when his aorta ruptured. He had an undiagnosed bicuspid aortic valve and an aortic aneurysm in his chest.

Eighteen months earlier, Doug went to the ER with chest pain. Nothing was found then. It was left for the coroner, after his death, to find the scar of an old tear, next to the rupture site.

A weakened, diseased aorta may "hang on" for some time, because it has an outer layer designed to be stronger than the others. For Doug, that outer layer held on for a year and a half. When it broke, there was no time to save him. He was instantly gone.

What would happen ten years later, in 2014?

If Doug went to the ER, would they still check him for a heart attack, find he did not have one, and stop there?

Or would they go further? Would they find his bicuspid aortic valve, his aorta bulging dangerously?

Would today's technology be able to "see" the small tear in his aorta? If not, would they still take his chest pain and aneurysm seriously?

Would they ask him about his family history? His uncle died in the same way. Family who saw them say that in death they both looked like they had "exploded". A horrifying memory.

Would they refer him for surgery in time?
 Ten years later, would someone save Doug?

In some communities in the United States, we know what happens today. Some aortic emergencies are being found in time to save lives. But we hear of the dying. Still. In 2014.

For one man, it happened like this.

Always healthy and active, he arrives in the ER. He tells them about a feeling of pressure and burning in his chest. He is short of breath. They check for a heart attack. Negative.

They know there is another killer in the chest - pulmonary embolism (blood clot in the lung). They rule that out too.

They keep him in the hospital, continuing to speculate. The family hears words like acid reflux/heart burn. They even hear about sleep apnea! Eventually they hear he has a bicuspid aortic valve that might need surgery "some day".

Although BAV is a red flag for aneurysm in the chest, finding it did not raise the alarm. Was it because there is a persistent impression that bicuspid aortic valves are relatively harmless?

For this man and his family, many hours went by, spent guessing about conditions that are not immediately deadly.

At times, he felt better. But all of a sudden, something terrible happened inside. At that moment, it was already too late. Many hours were spent desperately trying to save him. It was impossible. Time had run out.

He had bled massively inside his chest. The respirator would continue to breathe for him. His brain was dead.

Is this the best we can do, 10 long years later?

There are many unanswered questions and conflicting guidelines for those with bicuspid aortic valve.
We know we need more answers.

However, we ask for those with BAV what we ask for everyone else. We ask that this often varied condition be viewed with respect for its potential deadliness.

Please do not forget that the diseased aorta is right up there with heart attack and pulmonary embolism.
It is a killer.

There are little children growing up without Daddy,
 grieving wives, heart broken parents.
 Brothers and sisters wondering if this will strike them also.

For everyone with a bicuspid aortic valve
 who will never have an aortic emergency,
 we rejoice. 

But we are called to speak
 for those vulnerable to tragedy.

His daughter was born 4 months after Doug's death
Doug's children in 2010

Their children should not grow up without them.

And their family members should not live in the shadow of fear
 that some day this could happen to them also.

We are
continuing to tell their stories,
raising awareness of the danger,
while living
in a climate of hope.

- the Bicuspid Aortic Foundation

Sunday, September 14, 2014

My Love Will Find You - Families with Bicuspid Aortic Valve

This is for everyone who loves someone with Bicuspid Aortic Valve or any form of aortic disease in the chest.

Derek Owens' parents were told about his BAV at birth
He was 16 when he first had surgery

This is especially for Mothers. Mothers of all ages.  Right from the start, they know how special, how talented their child is. At some point, they may learn there is something not quite right inside the heart of their beautiful child. They agonize through the doctor visits, the surgeries. Some Mothers hear those most dreadful words. Their beloved child is gone. Rest assured, there is nothing you could have done to prevent a bicuspid aortic valve. Above all, you give your child what no physician ever can. You give them your love. It is the most wonderful treatment in the world, and you can increase the dose at any time without harm. Yes, it is the greatest healer there is. Your love. Unlimited. Always.

Following are the words of a childrens' book by Nancy Tillman. The pictures are from BAV families.

Wherever You Are, My Love Will Find You

By Nancy Tillman

I wanted you more
 than you ever will know,
 so I sent love to follow
 wherever you go.

It's high as you wish it. It’s quick as an elf.
You'll never outgrow it... 

it stretches itself!

So climb any mountain...

BJ Sanders at Machu Pich
climb up to the sky!

My love will find you.

BJ's dear Mother, who lovingly
follows her daughter's adventures
My love can fly!
BJ celebrating her birthday in 2014. She had surgery a year earlier.

Make a big splash! Go out on a limb!

My love will find you. My love can swim!

Scott Nichols (in blue) was lost to aortic dissection, January 2014

It never gets lost, never fades, never ends...

if you're working...

or playing...

or sitting with friends.

You can dance 'til you're dizzy...
paint 'til you're blue...

There's no place, not one,
that my love can't find you.

Derek Owens has always loved basketball!

And if someday you're lonely,

or someday you're sad,

or strike out at baseball,

or think you've been bad...

just lift up your face, 

Derek in high school
feel the wind in your hair.

Derek Owens and his Mom, Laura
That's me, my sweet baby, my love is right there.

Chuck Doherty lost his life in April 2012
 due to BAV complications

In the green of the grass... in the smell of the sea...

in the clouds floating by... at the top of a tree...

in the sound crickets make at the end of the day...
“You are loved. You are loved. You are loved,” they all say.

Carrie Mettler running a half marathon
Carrie Mettler's beloved Mother, who knew the reality of
having two daughters with BAV
My love is so high, and so wide and
 so deep, it's always right there, even
 when you're asleep.

So hold your head high
 and don't be afraid
Bob Gies following his surgery in 2010.
to march to the front
 of your own parade.

Doug Grieshop on his wedding day 

Doug Grieshop at 2 months
If you're still my small babe
 or you're all grown,

my promise to you
 is you're never alone.

Doug had an undiagnosed BAV. He lost his life to aortic aneurysm
 rupture 10 years ago, on September 20, 2004.

You are my angel, my darling,
 my star... 

and my love will find you

wherever you are.

You are loved.

Saturday, September 6, 2014

Scott Nichols - Bicuspid Aortic Valve and Dissection

Scott Nichols with his son, Carson, on the golf course
Words do not come easily when trying to capture the essence of Scott Nichols. In the prime of his life at age 41, he was a husband to his wife Jennifer, father to his daughter and young sons, friend to many, a special education teacher, and a coach.

Scott coached a high school hockey team as well as being the goalie coach for the NAHL Port Huron Fighting Falcons. He had a lifelong love of sports and continued to play many himself - hockey three times a week, softball, and golf.

Yes, Scott was a very special man, known to so many in his community and state.

There was something else special about Scott. Something that no one, not even Scott himself, knew. He had been born with a bicuspid aortic valve.

A Snow Day in Michigan and Chest Pain
Even in Michigan, where those who live there are prepared for winter, some days the weather is just too severe, and schools are closed. These "snow days" are unexpected free time for students and teachers alike. On a snow day in late January, 2014, Scott Nichols made the most of this unexpected gift of time, packing it with activity. He played basketball with some of the other teachers, and then went on to coach two different hockey teams. Later that evening, he was out on the ice playing hockey himself when he felt the chest pain. 

No Prior Warning
There was no warning that this strong, active man had anything wrong until chest pain forced him off the ice and to the local emergency room. There his heart was checked, and a heart attack was ruled out. The next day his bicuspid aortic valve (BAV) was found. He continued to get worse. What was happening inside Scott? More testing found it. Like many with BAV, Scott's aorta above his BAV had been bulging dangerously (aneurysm). The weak, bulging tissue had torn (dissection). Scott was bleeding inside.

Life Flight to University of Michigan
Scott needed to reach a medical center equipped to perform emergency surgery on his aorta. He was flown to the University of Michigan in Ann Arbor. There, as his family waited, surgeons battled for 14 hours to save him, repairing the terrible injury inside him. But the damage was too great. It had been too long without blood flow to vital places inside him. On January 31st, Scott Nichols was declared brain dead.

Disbelief, Shock, Mourning
Scott (in blue) at the golf course with friends

Scott's family, students, fellow teachers, and friends all descended into shock and deep mourning. This article from the The Voice, February 3, 2014, Scott Nichols Impact Felt, describes the anguish and tremendous sense of loss. As mentioned in the article, with hearts broken the Port Huron Hockey team decided they would play their game the next day in his honor. In the words of Coach Pionk, " ... people like Scott Nichols don't come around very often."

September is Chest Aneurysm and Dissection Awareness Month
The Bicuspid Aortic Foundation observes Chest Aneurysm and Dissection Awareness Month in September.  This same month is observed for brain aneurysms, which also occur in some individuals with BAV. This year, we are deeply grateful to Jennifer Nichols for bravely sharing what happened to her husband just a few brief months ago. She does so in the hope that it may save the lives of others. BAV is estimated to exist in 1 of every 50 people, predominantly in males. 

How many other athletic men in their prime
 are at risk from a bulging, weak aorta?

We do not know. 
We do know that finding a BAV is a red flag 
  • to look further 
  • to find the hidden danger 
  • and to have surgery before a life-threatening emergency.

We hope that through the tragedy of what happened to Scott, the life of someone reading this will be saved. Maybe someone strong and healthy like Scott. Someone who feels great, but gets his heart checked, and finds out he was born with a bicuspid aortic valve. 

In raising awareness
of the risk and the danger,
we  seek to create
a lifesaving
 climate of hope.
From all who volunteer
with the Bicuspid Aortic Foundation.