Monday, May 30, 2016

Remembering the Unknown Bicuspid on Fifteen Year Anniversary of Aneurysm Removal, May 30th

In 2001, May 30th was a Wednesday. Early that morning, my husband had surgery to remove the dangerous bulge above his heart. It is a day to remember with thankfulness.

Fifteen years ago, the Memorial Day holiday was two days earlier, on May 28th. We had agreed to do something unusual that day: check into a hospital.
My husband had stopped the coumadin (warfarin) required for his mechanical aortic valve, so that his blood clotting would return to normal. He was to check into the hospital at noon on Memorial Day, the 28th, where he would go on a heparin drip to prevent clots from forming and also have the typical pre-surgery tests.

Memories

We each have our own memories of that experience, but one thing is certain. Today, we remember and remain very thankful for the safe and complete removal of that aneurysm, up to and underneath part of his arch. It is a difficult surgery, and was done flawlessly. Fifteen years later, the repair and the aorta itself remain excellent.

Today, we are most grateful to his own gifted surgeon. We also understand that the pioneering work of aortic surgeons laid the foundation upon which his surgery was possible. The surgery my husband had that day remains perhaps the most complex in the chest, involving completely stopping blood flow at cold temperatures. Only some surgeons have this skill. We still marvel that we found such hands.

The Unknown Bicuspid

There is someone else that I remember today. I do not know his name. His identity is lost, except perhaps to his family. I have searched the medical papers, to see if anything might have been written during that era, that might be about him. I cannot find it.

Today I remember him because, like my husband and many others, one day he went into the hospital to have BAV surgery.

He never came back. He died from a torn aorta. 

It happened sometime after his valve surgery was over, during that first night. The doctors covering that night saw his kidneys weren't working. They did not know why. They did not know his aorta was torn, bleeding inside. The blood flow was cut off from his kidneys, so they could not work. By the time, the next morning, someone figured it out, it was too late. He could not be saved.

Lying there, perhaps the respirator tube still down his throat, helpless, his aorta ripping apart in his chest. It is a horrendous scene. I hope the drugs were strong enough to block the pain.  I hope he did not suffer.

It could have happened to my husband. His records show that his aorta was bulging at the time his BAV was removed. Somehow his aorta withstood the clamps, the cutting and sewing, when his BAV was removed. I don't know why it did.

There was a young surgeon in training then, who saw this happen. He never forgot it. From that day forward, he was to save many of those with BAV from a similar fate. Years later, he removed the aneurysm from my husband's chest on May 30th, 2001.

And so today, I remember this young man, and others like him. They are the Unknown Bicuspids. Some of them died of massive aortic bleeding, sometimes under the mistaken label of a massive heart attack. Some of them died because of complications from their BAV.

Today I remember their lives, cut short fighting aortic disease. 

Yes, we remember these unknown, who lost their fight much too soon. In their memory, those like my husband continue to live, to fight. They are all heart warriors. They have their own battles to fight.

Best wishes to all,
~ Arlys Velebir

Tuesday, May 10, 2016

Imperfect Heart Valves - Treasure the Ordinary Day

Background


With every beat of his heart, my husband's bovine aortic valve leaflets open and close. He received that valve 10 years ago. Statistics gave us hope then that, given his age, this valve might last about 20 years.

 Statistics are just that, nothing more.
 They are not promises.

 After just 8 years, there was evidence that one leaflet had begun to deteriorate. And we have been told that artificial tissue valves can fail suddenly.

Can We Avoid An Emergency?
Mechanical valve removed from my husband in 2006

We have already been through urgent situations because of his aortic valve. First it was his own BAV that put him abruptly into pneumonia and heart failure.

 Then, 15 years later, it was complications from scar tissue (pannus) and strands on his mechanical valve that injured him, sending particles to his brain.

 Each time, we were blindsided by the suddenness. Now, with this tissue valve showing a problem, is there anything we can do to be better prepared?

Sudden Failure

I am thinking of two people with bovine valves whose symptoms became severe suddenly. They found themselves urgently needing another surgery. One, a woman, had "redo" surgery locally to replace the valve. She was too sick to travel anywhere else. The recovery was not easy, but her life was saved.

 And then there is a young man. He developed severe symptoms, in a place far from the surgeon who performed his first surgery. He had to make a difficult decision. Step on a plane and fly for hours to reach his surgeon again, or entrust himself to whoever was local. He stepped on the plane. I still remember the relief I felt when I knew his plane had landed.
A failed bovine valve that required urgent removal

Knowing this, I wonder, can we know ahead of time, before it is urgent? We have been told that one reason for sudden problems is calcification buildup at the "hinges", the bottom of the leaflets where the tissue bends as the leaflets move. And when the valve leaks (regurgitation, insufficiency) for whatever reason, severe trouble can develop. These are two key things (the hinges, the leakage) that help me understand, removing some of the fear that comes with uncertainty and mystery.

Keeping Watch

When he first received this bovine valve, because of the severity of what happened with his mechanical valve, he was watched closely. Then it was checks just once a year, as things continued to look good. Just when we might begin to think all was well, there was the damaged leaflet!

These last two years, his bovine valve has been watched more closely again. The interpretation of his regular echocardiogram was rather alarming two years ago. A TEE (transesophageal echo, which requires anesthesia) was more re-assuring. The damage to that  leaflet is at the top, not at the hinges!

This leaflet does not move far enough to open as fully as it should, but the other two leaflets do. All three of them close well, so there is no significant leaking.

Another echo followed by a CT scan recently showed the valve about the same. Good news, for now!

Imperfect, Life Extending Solutions

I remember again how we were told 26 years ago that he was fixed for life. Today we know that for my husband, these artificial valves, first a mechanical and then one made of bovine tissue, have been life saving but imperfect solutions.

Coping in Our Three-Foot World

In the book No Hero, Mark Owen writes in chapter three about fear and the three-foot world. It is something he learned in rock climbing training. Not to look down, not to look off in the distance, not even to look for help from others. Focus on the things within reach, things that you can do. We can do that!

In our three-foot world, we do not have to live in fear of things in the distance that no one can control. Here is what we can control:

  • living a healthy lifestyle
  • monitoring blood pressure and taking blood pressure medication accordingly 
  • listening to his body, knowing that even mild symptoms are messengers
  • keeping appointments to check on the valve
  • learning from information as it emerges about artificial heart valves 

Treasure the Ordinary Day

This thought comes from a Swedish proverb. It would be so easy to forget that every ordinary day  is special, a treasure. For us, a day when, with every heart beat, an imperfect valve opens and closes well enough to make it a perfect day!

With very best wishes to all who,
like we do,
 live each day with an imperfect heart valve.

 May you have many perfect days.

~ Arlys Velebir














Saturday, January 2, 2016

Hope for the New Year

 Hope for the New Year


Amaryllis  Fantastica

Special Holidays This Year

This year when asked about plans for the holidays,
 I said that I had very special plans indeed! 
What were they? 
I was spending them with my husband.

Only those who can never do that again, this year or any other, truly understand how special that is. Their lives have been forever changed by the loss of their loved one to bicuspid aortic valve disease  My thoughts go to them, in many states and around the world. How much they would give for just one more moment together.

Flowers for the Holidays - An Impulse Buy

It was an impulse buy. While shopping in November I saw these square boxes with gorgeous flowers pictured on the outside. Amaryllis. I remembered my Mom having this plant around the holidays one year. It had seemed rather exotic to me. I do not have her green thumb and had no idea how much she coddled and coaxed it to produce those huge, gorgeous flowers. But, how I would love to enjoy those beautiful blooms once again!

The instructions seemed simple, and the picture on the box charmed me. Hoping I was not consigning it to a terrible fate, into my shopping cart it went.

The box promised blooms in about 6 weeks. If I started right away, there might be flowers for the holidays. Inside the box were just three things: a rather plain pot, a compact ball of soil, and a dead looking bulb. I followed the instructions. The soil did expand in the pot when water was added, as promised, and into it I placed the bulb.

At first, not much happened. I tried to keep the soil moist but not too wet. But I wondered if this dead looking bulb, partly exposed above the soil, could ever come to life, let alone produce something beautiful.

Then one day, I noticed the bulb was not brown any more. It had a green color to it. Life! Hope came alive in me too.

I'm sorry I don't have pictures of those early changes. I suppose I still did not have very much hope. But then something started to shoot up from the top of the bulb. Slender, green leaves! It was still a long way from beautiful flowers, but I found more hope stirring in my heart.

Maybe it would do better in a different location, with a bit more December sun. I moved it, watched it, and tried to keep the soil moist without drowning it! It was alive, that was clear, but I doubted there would be anything special happening any time soon.

Wonders Over Night

 Then, in just three days, wonders happened!

December 29th
On December 29th the outer casing popped open, with hints of the beauty within. December 30th was an amazing day!
December 30th






December 30th















Just Spending Time Together
And so, we have these holidays now, special, uninterrupted moments together. I find myself clinging to them, wanting to slow the passing of time. Soon they will be over.

The Amaryllis has so beautifully rewarded my rather doubtful efforts, producing beautiful flowers for the new year. In this new year, check ups await him once again. How is his prosthetic aortic valve doing? It needs to be checked soon. Last year, we learned it is not holding up as well as we would like. How much has it changed? His mitral valve tends to leak, but has remained unchanged for several years. He is to have a follow up with an eye specialist, too. We need to make sure nothing can rob him of his vision. Oh, the delicate tissue, the possible complications, for someone born with BAV.

This new year holds heart and aortic check ups for so many. Some face serious decisions about when and where to have surgery. Too often, good answers are hard to find.There are no labels, no names, other than bicuspid aortic valve or thoracic aortic disease, for many with complex issues through out their bodies. Quite simply, too little is known, too little understood.

I cannot bear to think about those so apparently healthy, so talented and active, who are unaware that they are in danger from their BAV or aneurysm. We need ways to find them before tragedy strikes.

May the new year be a fresh start in search of answers.

These are my thoughts on the eve of the New Year. And then, the New Year arrives!


January 1, 2016
Two fully open flowers, two buds

January 2, 2016
The Third Bud Begins to Open
January 2, 2016
 Something in Common - An Amaryllis and BAV??

Watching it change and bloom, I find this amazing plant has reminded me of the BAV experience. After long periods of quietness, BAV may change so quickly, so dramatically.  Doctors call it "latent". One interesting definition of that word is "hidden" or "dormant". For many years, BAV may not express itself. Then, often very quickly, there is important change. A valve needs surgery, an aneurysm is dangerously bulging. My husband's experience has been like that.

It is so hard for those who know, who must watch and wait. Like my Amaryllis bulb, it seems nothing is likely to happen. But then it does. Often then, things move quickly. It is so easy to be in denial.

Can I see the beauty, find the wonder, as life unfolds?

January 2, 2016
My husband has had three surgeries. When he had aneurysm surgery in 2001, I stayed in a nearby hotel until he was out of ICU, when I could be with him at night. That high risk time, right after surgery, is not easy. In that room was a single rose bud. Each evening when I returned to rest, it had opened a little more. Like my husband's recovery, it unfolded beautifully. I am glad I have that memory of beauty and hope from that time. The surgery was a marvelous success. Something I experienced almost 15 years ago still remains as a comforting memory. Today, the Amaryllis reminds me again that I can find beauty, even in the dark times. Beauty unfolds through the help and kindness of others, the repair of the broken, the miracle of healing.



Another Flower Stem Has Appeared
Hope for the Future
January 1, 2016
Yes, new experiences lie before us in this new year. I remind myself, whatever the future holds, we can do this! One step at a time, we can do this.

We can have those check ups, ask our questions, get multiple opinions. We can make decisions.

Along the way, I will look for the beauty in each day. 

So much awaits us all. May the beauty in the promise of help and healing sustain us. Let us focus on hope.


With all best wishes in this new year,

                          -  Arlys Velebir





Friday, December 25, 2015

Thank You on Christmas, Dear Mom and Dad

Montana in Winter
Dear Mom and Dad,

You did not know that you would become the parents of a little boy with a heart murmur.

Many years later, you would learn that he was born with something called a bicuspid aortic valve.

Others in your family were ultimately affected also.

 Mom, you heard his first cries before they rushed him away. (Dads were not allowed anywhere near in those days!) You could not see or hold him, your first born, for those first 24 hours. He was a little blue. There was a faint heart murmur. That is all you knew.

Whatever was wrong inside, your son was a fighter. He turned a beautiful pink (did something close inside?). Maybe the heart murmur was nothing? It was so faint, after all.

So off you went, home from the hospital together. His baby pictures tell me how much you loved him, how well you took care of him. He was a beautiful baby. (Of course, I am biased.)

It wasn't until he went to school that you were told about his eyes. From the day he was born, the world had been a fuzzy place to him, unless he held something very close. How could he know that the world wasn't like that for everyone? And so, he became the little boy with the big, thick glasses. A very active boy, with lots of energy.

There were so many busy, ordinary days. Two more children came along to complete your family. There was no reason to think about that faint heart murmur anymore. And then, suddenly, your oldest son and his sister, both under 10, were very sick. Off to the hospital they went, both of them. You heard the words "rheumatic fever". After that, doctors very clearly heard a murmur in his heart, and they restricted him from playing sports. Your daughter seemed to be fine.

And so, the years went by, and this boy became a man. I wonder now, about all the times you must have worried about his heart.

Decades went by, and both of you traveled to us when he had his first heart surgery. His aortic valve had to be replaced. I will never forget the two of you, no longer young, going into ICU to see him the first time. You heard about "bicuspid aortic valve" for the first time.

In the years after that first surgery, Dad, you always asked about his heart. We used to tell you there was nothing to worry about any more, that with the mechanical valve he had been "fixed" for life. But you must not have believed it. You kept asking. You were right, Dad, to be on guard. Eleven years later, your son needed aneurysm surgery. Dad, we never told you this, because you had already left us, two years earlier. We went home for your funeral service, just before Christmas that year.

Mom, you were with us for that aneurysm surgery. A big part of recovery is walking. You went with him on those walks, as he recovered. It was a precious time, the last we spent together. Less than a year later, clogged arteries in your own heart took you from us.

And so, Mom and Dad, you have not been with us these last years, as more has unfolded. You see, it was not just your first born who has been affected. One of the next generation has been found to have a BAV. (So far it has not caused any problems at all!) Your oldest son had a very bad stroke, caused by strands of tissue that had formed on the mechanical valve. He had a high risk surgery to get that mechanical valve out. Then, three years later, your daughter, who appeared to have a "normal" aortic valve all those years, became very sick. Both her aortic valve and an aneurysm of her aorta were replaced. It was a very high risk surgery, but she came through and returned to her busy life.

You would be amazed to learn, like we were, that the neighborhood boy who spent so much time at your house with your youngest son, also was born with bicuspid aortic valve. He had his valve and aneurysm surgery the year after your daughter! You would be glad to know that he too has returned to his very active life!

Our dear Mom and Dad, all of your lives as a family, you were surrounded by children with this condition, two of your own and the best friend of your younger son! They all love you so much. On this Christmas Day, so many years after you have left us, we all have special memories of how you made this a special holiday.

You are not here with us today, so that we can tell you that only special parents are entrusted with children with special hearts. You gave them the gift of living normal lives,  the best possible gift of all. As long as you lived, you loved them and were with them through the rough patches.

I hope you won't mind us sharing this with other parents. Parents like you, who have children born with bicuspid aortic valves. You would understand their feelings. But you can also tell them, that no matter what, your children are not defined by anything unusual about their hearts. They are whole and complete in all the ways that truly matter.

I, who write this, know that too. I am the woman who married him, your oldest son, the man with a heart murmur. Through all the years, your son's heart is, and will always be, just perfect to me.

For young parents today, please remember above all that your son or daughter has so many talents to give, to share. Some day, someone else will love them too. Someone will love them for all the wonderful things that they are. The number of leaflets that their aortic valve has will not matter at all.

With  love to you this Christmas, Mom and Dad, 
and to parents of those with bicuspid aortic valves everywhere.

                                                - Arlys Velebir







Saturday, November 14, 2015

Bicuspid Aortic Valve Disease - Facing Our Fears, Living with Love and Hope

Fall on Palomar Mountain, November 2005
The Days . . . .

Fall is in the air on this November day in 2015, the sky clear, the sun shining. On a sunny mid November day just like this one, ten years ago, we visited the Palomar Observatory. There were four of us in the car that day; we had family visiting from out of state. My husband drove those winding mountain roads to the Observatory, enjoying the handling of his car. It was a golden fall day, a beautiful memory to treasure always.

"And each day was filled with silver
For you gilded each moment . . .
And dazzled I joined your journey


Days like sand flow from our hands
If you doubt, then feel
One like the other goes with the wind
And the time we have goes there

Promise that you'll stay with me all your days
Yes, we made that promise together . . . . "
                                    
                                                                from the song "Dagane" (The Days)*
                                                                         
And now, come with me to a day about two weeks later, November 30th. It began like any other week day for us, with busy work schedules. We had plans to meet after work at a church service, as we had done many times before. He was not there when I arrived. I waited, then finally went in, saving a seat beside me. Still, he did not come. Finally, someone sat beside me as the seats all filled. To this day, I cannot remember who I sat beside that night. The memory is obliterated by the shock that followed.

"And outside sneaks the ugly 
and separates you from me"

                                 from the song "Dagane" (The Days)*
                 

I drove home afterwards, thinking (hoping?) that he had just been delayed too long by work or traffic, not an unusual thing, and had just gone directly home.

Please, let me be wrong . . . .


I try not to live in fear of this BAV condition, about which far too little is known. It is always there, from the day of birth. When your husband has already had two surgeries for valve and aneurysm, when you know that the valve he has may need replacing again, icy fingers of fear come easily. I felt them grip me as I neared home. I reminded myself of how many times I had feared the worst, only to find I was wrong, that he was fine. 

Please, let me be wrong once again. 

His car was in the driveway, but there were clues something was wrong. The house was mostly dark and silent, with just a faint glow coming from the back. Without the welcoming lights we always put on for each other, I hurried to the door, fumbling in the dark with the key in the lock. Fear had turned my heart into an icy rock now. 

I walked down the hall, turned on the kitchen light, and found him. 

This time, the fear had not been wrong.

He was down on the floor in a most pitiable condition. Only the paramedics and I need ever know just how terrible it was. I am so thankful that those images have never once visited me in my dreams. 

It seemed to me I screamed those words, "you have had a stroke", as I grabbed the phone, calling 911. I had thought he was dead, until he moved his head. As I spoke to the dispatcher, this pitiful apparition of my husband, helpless on the floor, asked me "Why are you calling them?"

The fire station and paramedics are less than a mile away. I believe they arrived quickly, although I was in a shocked world where time has little meaning. Our beloved African Grey parrot must have been screaming as they came in to help us. I have no memory of hearing our sweet parrot's cries, but I do remember one of them asking me about him. I have no idea what I answered.

The paramedics did some things to assess him, including having him repeat some words, "the sky is blue in Cincinnati", which he remembers to this day. (He thinks it should have been "the sky is blue in Montana", Big Sky Country!) Yes, it was apparent he had had a stroke. He was helpless, with no control of his left side at all. He was soon on his way to the waiting ambulance on a stretcher. 

I made one other quick call, to his surgeon, who has courageously followed him through thick and thin, since we first came to him in 2001 with an aneurysm. Then I drove behind the ambulance to the hospital. In the car, I called two dear friends. I asked one to remember us with prayers. The other joined me at the hospital.

If only we had known . . . .

"And if we had known,
we would have taken off

And I'd have carried you on my arm
through forest and over mountain

And you'd have held my hand
through gorge and dark ravine

And I'd have reached out for you
And you'd have reached out for me."
                                  from the song "Dagane" (The Days)*

                                                        
I  have tried not to spend time dwelling on what cannot be changed. I do believe we can learn from the past and in learning, help others also. We would have done anything to get that valve out before it could hurt his brain the way it did, if we had known.  "We would have taken off" indeed, where ever we needed to go, to find help. 

My husband had received a mechanical valve in the spring of 1990. We had been made aware then that stroke is a risk, due to blood clots forming on the valve. I assumed this was the reason for his stroke that night. It would be many weeks before we would find out the true underlying cause. It was not as simple as blood clotting on that valve. 

It comforts me today that what has been learned from my husband's injury (due to strands and panus on his mechanical valve) has helped others. Most of them had already had at least a small stroke before the problem was found. In one of them, Father Prodromos, who shares his two recent surgery experiences on the Foundation website, the valve was removed before it could injure him.

Some are called to be pioneers, out on the frontier, before knowledge arrives to help.  We understand that my husband has been one of them. May good continue to come from this, through preventive help for others.

Facing Our Fears, Living with Hope and Love

I did not know very much about strokes. I did know that brain injury was the one thing I dreaded above all else. I had seen it in others. It was the one thing I thought I could never face in someone I love. Today, I am so glad I was wrong about that!

Sometimes we are spared our greatest fears. When we are not, we ask for the grace to live through them, to prove a Power much greater than ourselves, to find blessing in adversity, in experiencing things we would never choose. 

We have been doing that now, for ten years.  Always with hope, finding God's best for us each day, in each experience. 

And so it has been, one day at a time. Hope, love, and healing.
  •  Do not let a stroke intimidate you. The brain loves to heal! 
  •  Do not despair. Your loved one is still there, no matter how impaired.
  •  Do not let medical professionals limit you, by telling you that after 6 months, improvement stops. Remember, the brain loves to heal, and it will, for months and years!
The Days . . . Finding Them Again . . . .

For anyone reading this who is dealing with brain injury in your loved one, may you live each day with hope and love. Ten years later, I can tell you there is hope. There is recovery. And a depth of love that is so precious, so rare, because it comes only at a great price. 

Who we are is much more than the function of our brain.

 Just follow your hearts together, your eyes on a future day, where you will find your dreams fulfilled once again  . . . . You may need to change those dreams a little, but who doesn't? Never give up on your hopes, your dreams. May you be blessed together, as you find them again . . . .

"So far away
So far away
So far as the sun can follow the blue waves
There will I follow you, my friend
There will we find them again"
                                   
                                        from the song "Dagane" (The Days)*
        
                                   
                                    
                               *Dagane by Odd Nordstoga
Translation from Norwegian courtesy of Kaare K. Johnsen on YouTube
Music player version from Kaare K. Johnsen's YouTube Channel,
of a live performance of Dagane by Sissel Kyrkjebo and Odd Nordstoga 

Friday, July 3, 2015

Bicuspid Aortic Valve and Big Hearts, Hearts of Champions

Our wedding anniversary is this month. 

Perhaps that is why I am moved to write about special hearts,
 as we celebrate the day my heart was joined 
with a very special one!

BAV and Big Hearts

My husband was born with a bicuspid aortic valve, and while no "condition" should ever define someone, in the case of those with BAV, there are some wonderful traits that seem to come along with this "defect".  Those with BAV seem to have large, strong hearts, that far exceed the norm. How much do their "big hearts" have to do with the energetic, active lives they typically lead? Perhaps a great deal.

My husband always loved physical activity. Forbidden to play rigorous sports because of his heart murmur, he turned to weight lifting with great intensity. Of course no one knew he had BAV then. Even after his BAV was removed, lifting weights was not forbidden. Today, it would be. And so I married a strong fit man, who exercised with discipline and rigor! Little did I know then that I would meet so many others with that same intense love of physical activity.

Secretariat - an Extraordinary Heart

My family no longer kept horses by the time I was born, but I have always loved them! I am fascinated by the legendary Secretariat. Secretariat had a very special heart! No, it does not seem to have been abnormal in any way. But it was extremely large! He also had tremendous muscular strength throughout his body. In the video below, he is 12 years old, which would be late 40's in human years.



I found it very interesting that a great deal is understood about the genetic lineage of Secretariat's heart.  "Research indicates that the X chromosome is responsible for the large heart found in outstanding race horses". BAV is an inherited condition, but although genetic studies are underway, there is so much complexity and few, if any, answers.

BAV and "Generous Hearts"

Reading about Secretariat's heart reminded me of this presentation from one of the Bicuspid Aortic Foundation conferences. Those born with bicuspid aortic valves have large, strong hearts!



A few months ago now, my husband had a thorough cardiac check up, including blood work. The cardiologist was amazed that his heart muscle is so strong, given that he has had three open heart surgeries and is no longer a young man! That strong heart muscle, as well as strength through out his body, has carried him not only through those three surgeries but the damage from a major right brain injury almost 10 years ago.

Others with BAV also receive these glowing assessments. Recently a young man who has undergone 4 surgeries in his short life was told that there are virtually no traces of injury, none of the detectable "damage" usually seen after open heart surgery.

Yes, these are special hearts!

Those with BAV are at risk from infection, heart valve disease, and aneurysms. However, they have other characteristics that help them handle and recover from those threats in ways that the "average" person may not.

A young woman who had her first BAV and aneurysm surgery over a decade ago recently was diagnosed with a terrible heart infection from a seemingly rare source, Q Fever. She has undergone two major surgeries (heart valve and aorta) in the last few months. Seeing her today, there are no visible signs from the terrible infection in her heart, the multiple surgeries that would have felled someone less strong. From her medical history, doctors would expect to see someone disabled, if still alive at all. But no, those wonderful muscles, including those of her heart, respond so well in cardiac rehab. What a wonderful, special heart!

Defying the Odds, BAVers Not Average

And so when I read accounts saying that those with BAV generally live as long as the general population, I know that it is because BAVers are special. The "average" person is not typically as well equipped, as strong and vibrant, going into surgery, fighting infection, etc. And yet, some of them lose their lives, always much too young.

BAVers should actually live much longer than "average", because they have so many advantages, if only they can avoid or overcome the threats from infection, their heart valves, and aneurysms. They are not "average".

Despite his special heart, Secretariat only lived to age 18. He developed laminitis, and it could not be cured.





This July, as we celebrate another year together, despite all the threats to his life, my dearly beloved husband continues to defy the odds.  And so, we have something to share from our life's experience, a message of hope and courage:

To those born with BAV
and to those who love you,
always remember,
you have a big heart,
the heart of a champion!

Indeed, you are very special,
and so is your heart!

With all best wishes,
Arlys Velebir


Saturday, January 3, 2015

Aneurysm Surgery - How Much Aorta Was Left Behind?

Another Controversy

It was hard to believe that my husband had such a major surgery. Of course that wound down his chest, along the same scar line as his first surgery, proved that his chest indeed had been opened again. 

The procedure was Wednesday morning, by Friday he was asking to go home, and Saturday morning he was discharged! Amazing - the third morning post op! He healed and recovered well.

But what had actually been done inside his chest? Would it stand the test of time? Later I would learn that there are things about aortic surgery that are debated too. I mentioned previously that there are two controversies that my husband escaped. One was the size of the aneurysm when surgery should be done. Here I write about the second one: whether or not to remove the entire ascending aorta using total circulatory arrest. Today I am so thankful that, in the right hands, my husband's entire ascending aorta was removed!

23 Minutes of Circulatory Arrest

The surgeon spoke to us after my husband's surgery, before I went in to ICU to see him. I wrote down something about 23 minutes, but at the time I did not really understand what it meant.

Later I would learn more about this. I would realize that my beloved's head was packed in ice during this time. Extra protection during a time when his brain waves went to zero! Nothing, no brain activity at all! I would eventually learn something about the history of this. And one day, I would go into surgery and watch it done to someone else's husband.

How Much Aorta Was Left Behind?

Perhaps there are many debates about the exact "how" of aortic surgery. This one is particularly meaningful and important to me.

The largest "bulge" of the aneurysm for those with BAV is usually in  the middle of the ascending aorta. Of course, there are exceptions to this, as there are exceptions in all things BAV!

The simplest, easiest surgery is just to cut out that piece of the aorta that is obviously bulging, leaving behind the part of the ascending aorta closest to the arch (the curve of the "candy cane") or so-called top part of the ascending aorta.

Why is this simpler, easier? Because the surgeon simply puts a clamp on the top of the ascending aorta, allowing the heart lung machine to keep circulating blood while cutting away the aortic tissue lower down.

Very Cold, No Blood Flow ( Deep Hypothermic Total Circulatory Arrest)

But what if the surgeon removes all of the ascending aorta, right up to the arch? There is no place to put a clamp. How can this be done, if the aorta is wide open, without blood pouring out of that opening?

Well, the flow of blood has to temporarily be stopped (circulatory arrest). That can be done by stopping the heart lung machine, but how can a patient survive that? Every cell in their body needs proper blood flow.

People can survive temporary blood flow interruption if they are kept very cold. The term used is deep hypothermic total circulatory arrest. As you can imagine, this is not easy to do without injuring the person whose life you are trying to save from the aneurysm.

My husband had this procedure. This was the 23 minutes, when his brain waves went to zero, he was very cold, and no blood circulated in his body. Yes, his head was packed in ice for extra brain protection. Unaware of all this, I sat in the waiting area. I was waiting to hear those wonderful words "the aneurysm is removed." At last, I heard them, some of the most special words ever spoken to me! A tremendous feeling of relief flowed through me at that moment. I remember that feeling, that moment to this day. Those words, that feeling, are tucked away among the special "sounds" in my heart.

When someone was talking to me about this part of surgery, they called this the part of the operation "where they deaded me"! We might think this sounds like death, and it would be at room temperature perhaps. But when very cold, the brain is protected, and comes back beautifully when slowly re-warmed.  This is what prevents brain and other organ damage to those who fall through the ice in frigid northern climates. They come out just fine, if they are rescued from the icy waters in a reasonable time.

Every time I think of my husband's aorta, I am very thankful that his entire ascending aorta is all gone! There is actually a "tongue" of Dacron underneath his arch, as his tissue there was not "good". So, it is a very good thing for him that the hands he trusted had the skill to do what was needed and nothing was left behind.

Not All Surgeons Do Circulatory Arrest and Take the Entire Ascending Aorta

There is a man, still young today, who lives in a rural setting. I picture him and his family enjoying those wonderful, wide open spaces. He does not live near any major medical center. As a young husband and father, he had his BAV and ascending aneurysm replaced. He and his wife traveled to a major medical center, far from home, for this surgery.

I well remember asking them how many minutes he had been under circulatory arrest. I wondered how long it had taken. My understanding was that up to 30 minutes was generally safe. Much to my surprise, they were told that it had not been necessary to do it, that the upper part of his ascending aorta was "fine". The years have passed. What about the aorta that was left behind? What does it mean for him? It is something to be watched. I think of him and his family often.

Since then, I have heard of many surgeries where some part of the upper ascending aorta was left behind. In that moment, done by those hands, perhaps it was the safest that could be done. But what about these people in the long term?


Thankfulness but Not Forgetfulness

Today my husband and I are grateful for life-extending surgery. Without it, his own and so many precious lives would end much too soon. But none of us can forget about aortic disease, once it enters our lives. There are things we can understand to help us live more confidently, although we may have aortic disease.

Before Aneurysm Surgery

I was given some good advice about surgery once. I was told never to urge a surgeon to do a specific technique on me, in order to have me as a patient. Surgeons will offer their patients what they do best. Just listen and try to understand. I was told not to push them for something else. If I did, I might be the first patient they try it on! Especially with something as complicated as what is described here, if the surgeon did not offer it to me, I would never ask for it. I would continue my search for help.

If a surgeon did offer it to me, I would ask how many of this exact procedure his hands do on a yearly basis. I would also ask how many complications (bleeding, stroke) there were. I would ask how many deaths. It is important to ask these questions.  It is my life, my future at stake.

I would also try to find someone who works at the hospital, who knows this surgeon's work. I am thinking of a woman who did this. She found out that the number of procedures that the surgeon quoted her was not for the heart procedure she needed at all. She had been given confusing, if not outright misleading, information. Being both brave and intelligent, she continued her research and ultimately traveled out of state, where expert hands performed this technique during her surgery.

After Aneurysm Surgery

If I already had my aneurysm removed, I would try to learn how much of the ascending aorta was left behind. One way to do this is to get my surgery report from the hospital medical records, and read it. Usually the surgeon's report describes in detail what was done.

And then, every few years, I would have my aorta scanned. It is better that I leave nothing to chance. It is best to know if my aorta has changed or not, since my surgery and since the last scan I had.

Time Tests the Surgeon's Work

Yes, surgeons may continue to debate the best techniques in aortic surgery for years to come. While they are debating, men, women, even children live with their work inside their chests. Time tests each surgeon's work.

I am very grateful that this is one debate that does not touch us. His entire ascending aorta is all gone. It cannot hurt him now. Something else did hurt him. It hurt him very badly. I will share that next.

May all with aneurysms be kept safe,
 both before and after their surgery,
                - Arlys Velebir