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

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 

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

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

Aneurysm Surgery - When to Have It

Diseased aorta, including ascending aneurysm

Avoiding the Controversies

I will always be thankful that there are two controversial issues in aortic surgery that can never hurt my husband. (He has found enough complications on his own, without anything more!)

One of those controversies is when to have surgery!

It may surprise you that there is a great deal of debate among physicians about some things that are often said to patients with great confidence, as if there were no questions at all. 

One of them is the size of the aorta that justifies surgery.

Two Men, Real Life

I will never forget Richard. He is close in age to my husband.  His aortic aneurysm was close in size to my husband's too.

I held Richard's hand the very first time I met him. I held it to comfort him. He was a patient in ICU that day. He was a miracle to me then. He still is. Richard is a survivor of aortic dissection.

Richard Houchin (in blue) running again
 after surviving aortic dissection

Why did one man's aorta tear,
 and not the other?

Why did Richard's aorta tear,
 nearly killing him,
 and my husband's did not?

No one can answer that question.

Clearly, it was more than just the size
 of the aneurysm.

When to Have Aneurysm Surgery

When it was discovered, my husband's aneurysm was measured at 5.2 cm at the widest part. He had an echo first, than a CT, and also an MRI. How we hoped there had been some mistake, or that it would somehow shrink. There was no mistake. And aneurysms do not shrink.

We had read before we met Dr. Raissi that aortic surgery had been done at 6.0 cm historically, when the surgery was still quite risky. As the surgery became safer, it was being done at smaller sizes. Based on what we had read, we believed my husband's aneurysm qualified for surgery in properly skilled hands.

One of the papers I read at that time, from Mt. Sinai in New York, spoke about when and how aortic surgery should be done. The paper includes those born with BAV.  I was very grateful for this paper, presented at the Aortic Surgery Symposium in 1998 and then published:  Surgical treatment of the dilated ascending aorta: when and how?  I remain truly grateful for Dr. Griepp and all the great work done at Mt. Sinai guided by his vision, skill, and compassion.

We were glad that when we found our way to Dr. Raissi, he did not hesitate to offer aneurysm surgery to my husband. Neither did he immediately rush him into the operating room. He did treat his blood pressure immediately, adding medications and adjusting dosage until it was well under control. During this time, we chose a surgery date. How was his surgery and recovery? Everything went perfectly. My husband's aorta remains in excellent condition, time tested and monitored for almost 14 years now! (His valve experience is a different story, which will be told later!)

Sadly, despite continued discussion over the years since my husband's surgery, today there remains a great deal of conflicting thinking about when an aortic aneurysm should be replaced in someone with BAV.

What's the Clinician to Do?

In a recent editorial in Current Opinion in Cardiology, Dr. Alan Braverman discusses the variations in recommendation in recent guidelines: Guidelines for management of bicuspid aortic valve aneurysms: what's the clinician to do?

Notice the differences in the guidelines in the chart.

Dr. Braverman writes: "I do not believe 'one size fits all' when it comes to decision making about the timing of aortic aneurysm surgery. One has to individualize management based on each patient's characteristics."

Yes! This is comprehensive, individualized care! This is what we need!

He further writes, "However, I am concerned that for some bicuspid aortic valve patients the strategy of observing the dilated aorta (greater than 5 cm) while waiting for the diameter to exceed 5.5 cm before recommending surgery will be associated with an unacceptable risk of aortic dissection."

However well meant, confident statements about waiting for surgery until a specific aneurysm size is reached, implying safety to the patient until then,  may be followed by tragedy for some people. People who believed they were safe until the moment their aorta ripped inside them. These are the people exposed to unacceptable risk when told to wait for a certain size aneurysm to develop.

What about the risk of the surgery? To me, this is a moral issue. Complex aortic surgery will be safest in the most skilled hands. These are the hands I would want if I were to have this surgery.

The moral issue is that patients need to have surgery in those most skilled hands, but may not be helped to reach them.

I personally would not want to have my aorta tear while waiting for a larger size aneurysm because I may be injured or die from the surgery. I am being forced to wait because the surgeon may hurt me before the aneurysm will!

I would feel I am being asked to gamble, trying to have as many good, quality days of life as possible before one or the other ( the aneurysm or the surgeon) injures me or worse!

There are highly skilled aortic surgeons. I should be able to go to one of them. I should not have to gamble with my life.

Dr. Braverman concludes his article with: "In discussions with cardiologists and surgeons who have expertise in managing the aortopathy of bicuspid aortic valve disease, it is my impression that they will use many factors other than size alone to inform the decision as to when to go forward with aortic surgery. We all should!"

Well said, Dr. Braverman!

What's the Patient To Do?

This debate and uncertainty makes it very difficult for someone born with BAV who has an aneurysm. We are expecting trustworthy advice about when to have surgery that is based on solid knowledge, with the assurance nothing "bad" is going to happen to us until then.

After all, what is the point of knowing you have an aneurysm, living each day in the shadow of that knowledge, only to suffer injury or death from it?

We need to know what is known.

We need to know what is NOT known.

We need to know and be part of the decision making process. Knowing so many with BAV and their families, I believe we can handle the truth.

So, what is the patient to do? Find someone who will tell us everything.

Find someone who will tell us all about what is known about size. Tell us that the larger the aneurysm, the riskier it is. But tell us that smaller sizes are not safety guarantees! Tell us that some aneurysms tear at sizes smaller than 5.5 cm. My friend Richard certainly knows that!  Tell us that some aneurysms tear at sizes smaller than 5.0 cm too!

And then tell us more.

Tell us, as Dr. Braverman describes, all the factors, the pros and cons, that apply to us as an individual.

Help us decide, thinking about many things and not just size alone, when to have surgery.

We need to find the hands that are expert in aortic surgery, with the best possible outcomes. Will someone tell us who they are? We are on the brink of greater transparency in medicine in the US. My hope is that will help us, the patients, search out the right hands.

We are the ones that live each day with an aneurysm in our chests. The risk is ultimately ours.

Please, tell us everything.

We can handle it.

Just don't let us die

 without the best possible chance

 to win this aneurysm fight. 

                        

I believe this is what my husband has had. 

I wish the same for everyone.

                                   - Arlys Velebir

BAV and Aneurysm - The Search for Help

"If you cannot do great things, do small things in a great way."  - Napoleon Hill

The Search for Help

Diseased aorta, including ascending aortic aneurysm

bicuspid aortic valve ascending aortic aneurysm
That is what I typed in the search engine in 2001. I found important answers from a somewhat surprising source. Williamsport, Pennsylvania, is a town of about 30,000 people. It is not the home of any famous heart centers. I am not aware of any well known heart specialists who practice there.  There would not be any large research grants or costly, multi-year studies of patients happening there. But in 1998, cardiologists there published a paper that is very important. In 2001, it was this paper that helped us understand what no physician had explained to us: that someone born with BAV could have an aneurysm, and get into trouble, whether their valve has been replaced or not. Someone like my husband. After his successful surgery, I wrote to Dr. John Burks to request the full paper and thank him. I am forever grateful and will treasure this paper always. Just one paper, perhaps it may seem small, but it was done in a great way. I like to think it awakened others and spurred them to do more for those born with BAV. 

Gone Fishing

Here I will spend a moment on our local experience. Once the CT confirmed the aneurysm finding of the echo, our internist suggested we contact the surgeon who had replaced the valve about 11 years earlier. Although we continued to search online, I did go ahead and call the surgeon's office. I remember telling the woman I spoke with that my husband had an aneurysm. I am not sure if she was a nurse or not, but there was a serious tone in her voice after she heard me say those words. She told me that the surgeon was out of town, on a fishing trip with the cardiologist that we also had met 11 years earlier.

This was a good thing.

We did not waste any further time with them. Today I know that there is nothing in their backgrounds that would particularly equip them to handle what some call the most difficult surgical procedure in the chest: aortic surgery. Years later, someone locally told me that this same cardiologist first referred them (adult man) to a surgeon whose specialization was in pediatric heart surgery! Then they were given another name, in Houston, of an aortic surgeon who had been dead many years. I suppose I was not surprised, but I was certainly saddened, at such inappropriate "help" being suggested.

Still today, I rather marvel that the internist did not have more knowledge or take more time with us. He was in many ways a very kind man, but clearly not equipped in the face of BAV and aortic disease. Kindness alone cannot save lives.

Regardless, it was time to move on. We were on our own.

Searching online, I discovered PubMed, the online US National Library of Medicine. What a treasure! I read that the aorta is replaced by a "tube" made of Dacron. I read about the aneurysm sizes at which the aorta is replaced. I tried out a calculation based on my husband's height and weight versus aneurysm size. Most of all, I searched for surgeons who did aortic aneurysm surgery in the chest.

Meant to Be

Did we really know how to look for help? Not very well, but we could follow clues. Today, when I think back to that time and all that has followed, the phrase "it was meant to be" comes to mind. We did not know then so many things, that eventually we would learn.

We did not know about the pioneering work of Dr. Randall Griepp in New York, and his collaboration with Dr. Nicholas Kouchoukos in St. Louis, among others. We did not know that Dr. Griepp had mentored Dr. Sharo Raissi in aortic surgery, and that Dr. Raissi had brought his exceptional aortic surgery skills quite near to us.

At a time when the bulging aorta, and worse yet, the horrendous bleeding emergency of aortic dissection, were viewed by many physicians with helplessness and dread, these skilled, courageous, and compassionate surgeons ran not away from the fight, but directly into it. They are the "special forces" of heart surgery - the most skilled, the bravest, the best. The biggest challenge yet today for those with BAV and aneurysm is getting themselves to skilled hands like these.

Perhaps some angels paved the way to Dr.Raissi's office that day. We were rather terrified really, consulting a surgeon on our own after being told years before "never ask a surgeon if you need surgery"!  The aneurysm terrified us more.

About half way through that appointment with Dr. Raissi, I felt the icy fear begin to melt. We went home with blood pressure medication prescriptions and strict instructions to start them that very night! Wonderful! At last we had found someone who understood and cared.

Regardless of how it came to be, we know how hard it can be still today to find help. We share our story to help others who also must travel this road after us.

Here is a video where I describe our search and aneurysm surgery experience.

 I also mention here that this aneurysm surgery was not the end. My husband would need another valve surgery just 5 years later, in 2006. Perhaps it has taken all these years, and we are still learning today, the reality that some born with BAV are never "done"!

Best wishes to all who read this, in this New Year of 2015. May you have happiness, good health, and be led to those who can help you in your time of need.

                                                                                       - Arlys Velebir