Monday, March 11, 2013

Bicuspid Aortic Valve Awareness Campaign


The calendar may say March, but it is still “Heart Month” at the Bicuspid Aortic Foundation. In fact, it is always Heart Month here! About every 7 minutes, someone in the United States is born with a bicuspid aortic valve in their heart. There is never a time when their hearts aren’t in the forefront of everything we do. Haven’t heard of a bicuspid aortic valve (BAV) before? A major part of our mission is to raise public awareness of BAV, the most common congenital heart defect, and its associated risks.

Bicuspid Aortic Valve

Awareness Campaign 

Laguna Niguel, CA (March 11, 2013) – 

Doug with his son, Grant
When Doug Grieshop left for work early that September morning in 2004, he had no inkling he would never walk through the door of his home again. He collapsed suddenly at work later that morning, and nothing could be done to save him. It was the day after his 33rd birthday. Doug was born with a bicuspid aortic valve, but he did not know it. He also did not know that he had an aortic aneurysm bulging dangerously just above his heart. If only he had known. If he had, it is highly likely he would have had successful surgery, like his cousin did, and still be with his family today.

Most people have not heard of a bicuspid aortic valve (BAV), but the Bicuspid Aortic Foundation is out to change that. It is common, existing in an estimated 1 in 50 people, and is more often found in males. Complications may happen when someone has BAV. They include the valve leaking and/or not opening properly, and the aorta bulging dangerously (aneurysm), tearing, or rupturing. There is also increased risk of infection in the heart (endocarditis), and some people may have a narrowing of the aorta called coarctation. Blood relatives of someone with BAV may also develop an aortic aneurysm in their chest, even if their aortic valve looks normal.

While the list of possible complications may sound frightening, everyone’s experience is different. Knowing about BAV can alert the entire family to be checked for BAV and/or aneurysms. For those who need it, there are excellent solutions to help them. The important thing is to get out in front and prevent any surprises, finding those at risk early so they can receive the care they need. Many with BAV are active, athletic, and talented, contributing a great deal to society. Finding BAV early and screening family members is important to help them continue their active, productive lives. Their lives should never be lost because of BAV.

Wondering if BAV could be in your own family, or that of someone you know? Since it is so common, it is very possible. Finding BAV and aneurysms saves lives! To learn more, please visit the Bicuspid Aortic Foundation website at . And tell a friend to check it out too. You may save someone’s life.

Saturday, March 9, 2013

Dianna Rose and John Johnston - BAV in Families

While both Dianna and her brother John were born with bicuspid aortic valves, it is important to remember that when one person has a BAV, other family members may develop an aneurysm even though their aortic valve may appear normal (three leaflets).

Brian Cleary and BAV- A Life Saver is Saved Himself!

February 2004
Disbelief flashed through Brian Cleary's mind as he watched Richard Houchin stagger and fall. Brian was the contractor who had just had lunch with Richard and was talking with him when he suddenly collapsed that day in February 2004. 

For the tiniest fraction of a second, Brian thought Richard was joking. After all, this was Richard. Brian had known him for years and thought Richard was one of the healthiest guys around. He had been fine all through lunch. Whatever was Richard doing, kidding around like this?

No, this was not a joke. Something was terribly wrong, and Brian's finger was pressing 9-1-1 on his cell.  At that moment, neither of them knew that Richard had an aortic aneurysm tearing in his chest. And neither of them knew that as Brian made that call, there was an aneurysm in his own chest as well.

Brian Cleary (left) and Richard Houchin
September Awareness 2006, Marina del Rey, California

 Brian is "Bicuspid"!
Through the days and weeks of Richard's recovery, Brian visited him often in the hospital and then later at home. In those first foggy days after his surgery, there was so much for Richard to take in - learning that he had been born with a bicuspid aortic valve, that his aorta had enlarged silently until the day it began to tear as he stood talking to Brian, and that he had suffered a stroke. So Richard doesn't remember Brian telling him then that he too had a bicuspid aortic valve. It had been replaced years before. 

It wasn't until months later that the topic came up again, when Richard and Brian were again having lunch one day. This time, Brian gave Richard the shock of his life. He just casually brought up his own heart surgery, saying that his bicuspid aortic valve had been taken out over 10 years before. Unlike Richard, who never knew he had a bicuspid aortic valve, Brian had known about his BAV since he was about 30. When it began to fail, Brian's aortic valve was replaced with a human donor valve, a homograft.

Brian's MRI
Richard will tell you that he almost fell off his chair in the restaurant! Brian was "bicuspid" too! And then Richard became very afraid for this man sitting with him at the table - afraid because he knew that just having valve surgery doesn't take care of the aorta if you are bicuspid. Richard needed to persuade Brian to have his aorta checked. He told Brian he needed an MRI of his aorta.

You can imagine their conversations . . . talking through what is just beginning to be appreciated by physicians - that bicuspid aortic valves may be associated with abnormal tissue leading to aneurysms, especially of the ascending aorta. Brian knew that his donor valve was leaking and might need to be addressed at some point. But the idea that he might have an aneurysm too just seemed hard to believe.  Could Richard possibly be right?

If you have seen someone collapse and then fight to recover from both aortic dissection and stroke, you have a pretty good idea what you could be facing if it happens to you. You also know you might not make it at all. So Brian had an MRI, and there it was - an aneurysm. Brian's ascending aorta was bulging to just over 5 cm, roughly the same size as Richard's had been when it dissected.

Brian's Surgery - March 2005
Brian didn't hesitate scheduling his surgery, choosing a date in March 2005. This was a second surgery for Brian, what surgeons call a "redo". Brian's homograft valve was leaking and needed to come out, along with removal of his ascending aorta. Redo operations involving homografts can be complicated, but Brian's surgery and recovery went well. He emerged from surgery sporting a Dacron graft and a new bovine valve. Now it was Richard's turn to visit Brian.  

The Background
Brian Cleary and Richard Houchin have known each other a long time. Born across the world from each other, Brian in Australia and Richard in Ohio, they now know that they both began life with a bicuspid aortic valve. Later they both found their way to southern California. It's over 20 years ago since the day Richard asked an architect who he should get to work on an industrial building he was planning. He was told Brian Cleary was the one, so Richard contacted him. Richard found Brian to be a perfectionist about his work, and since then they have worked together on a number of building projects over the years. Today no one would doubt what their greatest project is. It is the project they never planned at all. It began in February 2004, a task they would never have thought to undertake - saving each other's lives.

Brian and Richard in 2006
How do you thank someone for calling 911 at the most precarious moment of your life? No doubt Richard Houchin thinks about that when he thinks about Brian Cleary.

How do you thank someone for insisting you have your chest scanned for an aneurysm when your doctors had never suggested it? Brian Cleary probably thinks about that when he thinks about Richard Houchin.

So, how do you thank someone? Well, you might take them to lunch, but if Brian and Richard do that, they joke that they might as well split the tab. Despite the fact that their most dramatic moments seem to happen around lunch time, Brian and Richard still get together often at local restaurants. And when they do, beneath their banter and laughter is an indescribable bond. Listening to them talk is like being with those who have gone through war together. There is a sense of something that only they have lived through, something only they know. They both have scars on their chests and Dacron inside, evidence of winning their fight to live.

Brian and Richard are grateful to many people who played such important parts in their experience - paramedics, their surgeon, their families.  .  .  .  But they both know that the reason they are still here begins with the two of them.

"It could be the guy standing next to you." 
For Brian and Richard, that was the case many times. When they were together, each of them was right next to someone who had an aneurysm in his chest. Neither one of them knew it.

And that is their message,  for September Awareness and for every day of the year: it is much more common than we think.

Bicuspid aortic valves are estimated to exist in up to 2% of the population, more frequently found in men than women.  Along with the bicuspid aortic valve may be abnormal aortic tissue, which can result in thoracic aortic aneurysm and dissection or rupture.

So go ahead, talk to the guy standing next to you about BAV and TAD. Sometimes, two lives may be saved.

Richard Houchin - BAV Dissection Survivor

Richard Houchin (right) joins Jan Van Noord 
along the route of the Los Angeles Marathon,  March 6, 2005

March 2005 
At the beginning of 2004 in Los Angeles, Richard Houchin was an active 62 year old, running 5 miles with his jogging partners and regularly working out at the gym. When time allowed, he ran half marathons (13 miles).

February 4, 2004, began as an ordinary day for Richard. Around noon he and his wife, Mary, had lunch in a restaurant with a contractor doing work on their rental property, an industrial building. Mary left them at 1 pm, and the two men walked back to the nearby property. As Richard stood talking with the contractor, he suddenly collapsed. (The contractor's name is Brian Cleary. Read Brian's story on this blog also!)

An ordinary day was now a crisis: the call to 911, paramedics, the emergency room of the local hospital, the message for Mary to rush to Richard's side, testing, then a CT scan and a diagnosis.

No, it was not a heart attack. Richard's ascending aorta had dissected.

Written here in a few brief words are events that took place over several hours. During that time, Richard's aorta was torn inside, holding together by its thin outer layer.

At about 7:30 pm, Richard was transferred to Cedars-Sinai Medical Center in critical condition. Dr. Sharo Raissi performed emergency surgery, replacing Richard's entire ascending aorta and repairing his bicuspid aortic valve.  

When paramedics first arrived on the scene, they noticed Richard could not move his left side. During the dissection, Richard had suffered a stroke.  Richard not only had to recover from the surgery to repair his aorta, he also had to overcome the affects of the stroke.Today, no one looking at him would imagine that Richard experienced a life-threatening aortic dissection and stroke just over a year ago. He feels slightly off balance when he stands, and there is some numbness in his left hand and foot.

Richard's positive focus on his recovery
 and the support of his family and friends brought him through the months of healing and rehabilitation with flying colors. Nine months after his dissection, Richard returned to running, beginning with short distances and gradually increasing the length. On the one year anniversary of his dissection, he ran 5 miles with his friend and jogging partner, Jan Van Noord. About a month later, on March 6th, 2005, Richard ran with Jan along part of the route of the LA Marathon.

Richard and Jan following the LA Marathon
In speaking with Richard, if you should ask him if he is having a good day, he will tell you that every day is a good day. Every day is a day he might not have had

Richard and his family hope that sharing their experience will bring greater awareness of the nature of bicuspid aortic valve disease. His bicuspid aortic valve has had essentially normal function throughout his life, both before and after his dissection. What endangered his life that February day in 2004 began with thin weak tissue in his ascending aorta, silently bulging to more than 5 cm in diameter.

Richard's family history holds a clue to his own condition. His father died suddenly at the age of 57, and it is possible that Richard inherited bicuspid aortic valve disease from him.

September 2005

Mary Houchin, Arlys Velebir, Richard Houchin, Jan Van Ord
at Culver City for TAAD Awareness Proclamation
On September 12, 2005, Culver City, California  proclaimed September as Thoracic Aortic Aneurysm and Dissection (TAAD) Awareness Month. This city is where Richard collapsed with his dissection, and it was the Culver City Fire Department paramedics who responded to the 911 call. During the proclamation ceremony, Richard spoke about the crucial role the paramedics played in saving his life. Although advised by a local medical center to treat Richard for a heart attack, they thought there might be an aneurysm in Richard's chest. The approach to heart attack victims is just the opposite of what is desirable for someone like Richard, whose aorta has torn and may be on the verge of rupture. Instead of thinning his blood and raising his blood pressure, they quickly transported him to the local hospital and later moved him to Cedars-Sinai. In expressing his gratitude, Richard said "Your paramedics saved me........I shouldn't be here."

Richard in 2013

Living as a post-dissection survivor, Richard continues to do well today. And he still has his bicuspid aortic valve, which was spared during the operation that saved his life!

Chuck and Cheri Sheridan - Aortic Dissection and Hope

 by Marie LoParcoCheri wrote that this artist's proof, created by her friend, is to be shared with others as an expression of hope. It is fitting that a picture of it be shared here, with their story.
 This story of hope was written in  May 2007 by Cheri Sheridan about what she and her husband, Chuck, experienced almost three years earlier. 

Before you read Cheri's words, I will add a little background to this story. At the time Cheri writes about in 2004, I was privileged to work with Dr. Raissi on a limited basis in his Aortic Surgery Program. And that is how I know Cheri and Chuck.

I still have not met them in person, but I know Cheri especially well. We have walked in the same shoes. The first time I heard Cheri's voice, I recognized in it the timbre of fear that has at times gripped me also.

 Aortic disease does that. An icy fear penetrates to our very core. 

It is the fear of losing our loved one in a fight with an enemy we scarcely understand. Cheri mentions being "frozen with fear". 

She was afraid there was no hope

This is one of the reasons that the Bicuspid Aortic Foundation emphasizes creating a climate of hope. Hope empowers us to keep seeking  help. Cheri and Chuck did find hope. They found choices they never knew existed and skilled hands to save Chuck's life.  

Today, as you are about to read, Chuck and Cheri "have their life back". Thank you, Cheri and Chuck, for sharing your lives here and giving hope to others. We all need hope so much. - Arlys Velebir

The rich, warm colors that the artist chose for this work,
seen in this close up, remind us that icy fear melts away in the warmth of hope.
                                                                         May 2007
                                                         Here is Chuck and Cheri's story . . . .

I am writing this almost 3 years after the fact: I would like to express my feelings about the compassion, encouragement, and care that we personally received from Arlys and Dr.Raissi at the Cedars Sinai Hospital. My husband had had 2 surgeries after having an aortic dissection while swimming in 1996. It was a miracle that he survived his first episode. Several years later after another operation repairing another segment of his aorta his yearly CAT scan showed that he was in need of yet a third operation. His medical reports were sent to Cleveland Clinic without our knowledge and a physician's secretary left a message on our answering machine saying that my husband needed to get in touch with that doctor immediately for a consultation.

We did and that doctor told us that my husband needed to have 2 sections of his aorta that were previously repaired redone in two more radically invasive operations, to be scheduled a month or two apart. When my husband asked what his options were, he was asked by the doctor if the word mortality meant anything to him. It was a very long drive home to central NY from Cleveland, Ohio. I was frozen with fear, believing that my husband would not be able to "get his head around" two more invasive surgeries and that I would loose him.

Fortunately for us, when we were at the Cleveland Clinic that day, waiting to see the doctor, we met a nurse who had had the nearly same experience that my husband had had in the same year. We spent the entire day together waiting to see the doctor. It was through her, after comparing similar experiences that we finally learned what much of the medical jargon meant. She gave specific details about stroke, heparin causing bleeding ulcers, and how low her blood pressure was being kept and what specific medications/doses that she was taking. I wrote everything down as I had been doing with each person that my husband had met within the 2 days of testing/evaluating procedures prior to our meeting with the doctor.

Still not knowing what to do, several days later I found the Cedars Sinai Web page and discovered it to be very easy to navigate. It even had a space to write in a specific aortic question. It also offered a page that you could go to that would answer general questions that were asked about aortic medical problems and I knew that each aortic dissection survivor has different/individual circumstances. So I wrote in something about our situation; it was processed and shortly I received an email that said a Cedars Sinai Hospital liaison person would be in touch with me within 24 hours.

That happened! Arlys called me and she asked me how she could be of help. I told her our situation and I said to her that if we were "just tilting at windmills and didn't have any hope" that someone should tell us and that we would just live out our lives in the time that we had. She said,"Oh no! You are only looking for the least invasive procedure that you can find for your husband…you have so much hope and many possibilities!" She gave me her work number and her cell number and remained in constant contact with us and introduced us to Dr. Raissi. 

We sent all of the medical reports that we had received about my husband's condition to him. He spoke personally to my husband, our local family doctor, and me. Over a period of 3 months he got my husband's blood pressure to a safer level and referred us to medical help on the East coast near our children who both lived in NYC. Arlys and Dr. Raissi never let go of us! We also knew that if we didn't find the medical care that we were looking for in NYC that Dr. Raissi would do the operation. We felt their care and concern immediately from the first time I heard Arlys' voice.

We had the surgery in NYC and it proved to be successful. Arlys and Dr. Raissi directed and guided us through a very rough time in our lives and held us close. My husband is back in the pool for exercise, just won his division in our local Canoe/Kayak Race, and works full time. We will forever be grateful that we have our lives back!  - Chuck and Cheri Sheridan

Living with BAV - Hope for Family Members

I took this picture at Sea World in San Diego. We may never
stand on the nose of a whale, but we may find ourselves
doing amazing things of a different kind when our loved one
has BAV disease.
My husband has bicuspid aortic valve disease. On at least five separate occasions, it has seriously threatened his life.  Like most people, I had encountered serious illness before, through my family and friends. But nothing prepared me for bicuspid aortic valve disease. Nothing. Perhaps it is the ability to suddenly create very dangerous situations in seemingly strong, healthy people that sets BAV disease apart. It has injured my husband, despite our best efforts to protect him. It has broken my own heart, only to find that my husband's smile could heal it again.

November 2007

Darkness comes early in November. Walking with my husband in the evening, twinkling lights outline the communities around us.  My eyes linger on one large building, its glow standing out from the general glitter.  Recognizing our local hospital, I remember again one particular night in November - the night when I drove toward those lights, following an ambulance. Inside that ambulance was my husband. An agonizing distance of just five miles, it was the beginning of an unplanned journey into the world of brain injury.  I realize now that it is more than time for me to say something important.
No matter what happens, keep hope with you always.

My last memories of my husband without injury to his brain are happy ones. There was no warning, no sense of impending disaster that morning. Later, while alone in our home, he fell repeatedly, unable to keep his balance. Finally, he could no longer stand at all. I found him on the floor hours later - alive, but disoriented and completely helpless. Paramedics came quickly. And so, I followed an ambulance into the unknown on a dark November night.
A Bolt of Lightning

The symbol for stroke is a lightning bolt. It is an accurate depiction of a "brain attack"; victims are stricken suddenly, without warning. The injured person often has no idea what is happening. In concept, an embolic stroke is similar to a heart attack. However, it is different also. Unlike heart muscle, there are no pain receptors in the brain itself. The cells do not cry out as they die. My husband remembers falling. He had no idea why.  He felt no pain at all.
"Creating a Climate of Hope"

"Creating a Climate of Hope" I love these words, the theme of the Bicuspid Aortic Foundation. In the years leading up to 2005, through my husband's critically severe pneumonia, congestive heart failure, and two open-heart surgeries,  I had already proven that hope is vital when confronted by aortic disease. But on that November night, I was about to learn something more about living in hope - this time, in the context of strokes.

From the moment I found my husband, helpless and paralyzed, I was shocked into action. No matter how much it hurt, this was no time to despair. As I write this now, I can think of many times when fear gripped me, but I cannot think of even one time when hope was not there.
Valvular Heart Disease and Stroke

"Progress in Medicine is often triggered by emotional circumstances. As a resident in cardiac surgery in the early sixties at the Hôpital Broussais in Paris, I was struck by an artist suffering from a valvular disease whose valve had been replaced by a valvular prosthesis. Three months after the operation, he presented with a severe brain damage due to the migration of a clot formed in the contact of the prosthesis. He couldn't paint anymore. The same valve, which had saved his life, had now definitely impaired his quality of life. At this very moment, I decided to devote my research to this problem."

   - Dr. Alain Carpentier's Acceptance Speech
    The Lasker Foundation 2007 Clinical Medical Research Award 
I was deeply touched by these words of Dr. Carpentier's. I did not know until recently, when I read Dr. Carpentier's acceptance speech, that he was so moved by a stroke victim that he devoted his work to perfecting bioprosthetic heart valves. Similar to the artist Dr. Carpentier mentioned, the mechanical valve that so clearly at one point saved my husband's life later became the source of serious injury to him - injury to his brain.
20% of Left-Heart Prosthetic Valve Patients
Have Strokes Within 15 Years

A paper from the University of Ottawa, 
"Late incidence and determinants of stroke after aortic and mitral valve replacement", studied those with left-heart prosthetic valve replacements. Published in 2004, the abstract states that about 20% of those who have a prosthetic valve experience an embolic stroke within 15 years after valve replacement.   

My husband had lived without incident for over a decade with a mechanical valve. Following the diagnosis and timely, marvelously successful removal of his ascending aortic aneurysm,  I dared to hope that we had conquered the worst this disease could do. However, I realize now that his stroke experience fits into these statistics reasonably well - my husband's valve had been in place just over 15 years when he suffered a massive right brain injury. 

A Shower of Particles to His Brain

As Dr. Carpentier mentioned, a blood clot is the likely suspect when someone with a mechanical valve suffers a stroke. In my husband's case, this explanation did not fit. Locally, I was told that they could not explain what had happened - the reason could not be found.  He essentially had multiple strokes at once - a shower of particles had reached his brain. Why?

It took careful investigation to find the explanation.  We learned that fine, hair-like strands of tissue had formed on the intake side of the valve. Pieces of these strands had broken off and traveled quickly through that efficient plumbing system, designed to keep the brain well supplied with oxygenated blood.  Reaching his brain, these fragments blocked blood vessels. Cells without blood flow cannot live very long.

Today, I wonder how many individuals have strokes, not from blood clots, but from valvular strands. There are only a few papers in the literature. My husband was the first case that his doctors had seen. In the months since his diagnosis, they have found these strands in four others. Valvular strands are only found by transesophageal echocardiograms (TEE). On the screen, they are small white dots that move quickly across the visual field. Physicians and patients are on the frontier of knowledge here,  learning together about valvular strands.
A Devastating Complication

The paper from Ottawa begins by describing stroke
as a "devastating complication".
It is.
But never let anyone take hope from you.

Brain injury was completely foreign to me as I entered the emergency room that night, totally unprepared. Sadly, it is not a stranger in that setting. Stroke is the leading cause of disability and ranks as the third cause of death in the United States. Hearing "Code Stroke" over the hospital loud speaker always filled me with tremendous sadness. In addition, motorcycle and car accidents, falls, and many other catastrophes result in "TBI", traumatic brain injury. We found ourselves in this world, where young and old alike desperately seek help, where ever it may be found.

I have read the reports now, including the technical description of the damage to multiple lobes of my husband's brain. Words on paper, had I seen them then, in those early hours, they would not have mattered to me. I, who knew him best, was focused on any evidence, any tiny sign, that my husband was still there.

He was! But like the landscape after an earthquake, there was damage. Vital bridges were out. Some structures had crumbled, others partly collapsed. I remember watching as the ER doctor asked him to wiggle his toes, move his left foot, leg, arm, fingers - nothing. Paralyzed on his left side, I also thought he was blind in his left eye. Later I would understand it was not his eye - it was damage to the visual centers in his brain.

With time I would find that many things were still intact. But vital links and functions were gone - things we so easily take for granted. What we both needed then above all else was the conviction that what was lost could return again. We needed hope.

There is a common thought that the first 6 to 12 months are generally when recovery occurs after a stroke. We have long ago passed that time frame now, and we know from experience that the first year is not the end of improvement. Perhaps for some things, it is still just the beginning. 
It is important not to under estimate the ability of the brain to heal and repair itself. There is a term used to describe this ability of the brain to adapt and change - neuroplasticity. The brain is indeed capable of recovery.
It would be a tragedy to limit the brain by giving up.
Hope will never let that happen.

Tread Mills and Roller Coasters

What is it like to go through a major stroke with your loved one? It is a complex experience, and a book with many chapters would be required to cover all that should be said.

Along with my husband I suddenly became part of a chain of events beyond our control.  Imagine that you both have stepped on a treadmill that never stops. There is no way for either one of you to get off. Your only choice is to keep going, one step at a time. That is what the intensity of the come back from a stroke has been like. There are no days off, and no vacations, from brain injury.

My husband's experience was complicated by two things: 1) there were "multiple infarctions" or areas of injury to his brain, 2) shortly after his stroke, he underwent his third open-heart surgery to replace the mechanical valve. Both of these made the experience especially challenging. At times, it has felt more like a roller coaster - there have been definite ups and downs, but the trend always upward over time.

Along with the stroke came a confusing deluge of papers and forms - insurance, disability claims - that must be dealt with. At times it was over whelming, and I learned to prioritize. Researching options for treatment and therapies - and a relentless round of doctor appointments, lab work, medication adjustments, and therapy sessions - all were part of the experience, especially in the early months. We both look back now and wonder how we did it. It must have been because hope was always there . . . . 
When exhaustion sets in, hope is there too.
Reminding you that you still have each other.
You can keep going, because you are together.
Together, you can make it.

The key for us was and still is in surrounding ourselves with positive people, working with those who are fully committed to the concept that the brain does indeed come back. We are fortunate to live in an area where there are a number of choices and to have the ability to take advantage of them.
Courage is doing what needs to be done,
when it needs to be done.

Someone described courage this way, and  it has helped me very much. I do not always feel very strong or very brave. I have found that this condition that affects my husband  has at times terrified me, and I know it may do so again. But it need not  affect my courage. And I would like to share that courage, because I believe it can help someone else. It is just a matter of doing what is before you at this moment, today. It is not about what has happened in the past or what waits in the future. It is simply doing now whatever it is that needs to be done.
You will find that you can do amazing things.

There are so many  things you can do if you have courage. You can search for the best information and expertise. You can pursue every option in understanding what has happened and what choices are available. When time for surgery comes, you can wait for an eternity while it is performed. Later your touch, the sound of your voice, your love - these are powerful medicines, indespensible "critical care" that you alone can give.

Yes, you can smile when your own heart is breaking . . .
                           and bring hope with you always . . .  
                                      because your loved one
                                               needs your strength,
                                                          your reassurance.

               Later,  you may cry, all the tears you have  . . . .

And Hope Will Bring You to a Brighter Day
December 2007

Two years ago in December I pushed my husband in a wheel chair -- to his meals in the hospital rehab dining room, sometimes outside for a breath of fresh air on a sunny afternoon. This year, what a different setting - December found us enjoying a day at Sea World in San Diego! Walking everywhere together on a glorious sunny day!

Along with hope, I have found other treasures on this journey. Costly ones. Perhaps they would not have come to me in any other way. Love . . . faith . . . comfort . . . peace . . . strength . . . courage . . . the caring of  friends . . . the kindness of strangers . . .  my husband's smile as we mark another milestone together . . . .
These jewels sparkle in my memory . . . priceless gifts . . . .  

Yes, we have come to a much brighter day,
and hope continues to whisper in my ear,
telling me of wonderful possibilities still ahead,
because the body loves to heal.

For anyone who wishes to reach me, you may do so by emailing the Bicuspid Aortic Foundation. - Arlys Velebir

Friday, March 8, 2013

BJ Sanders - Bicuspid Aortic Valve and Blood Pressure

BJ Sanders
BAF Board Member

BJ Sanders is a member of the Board of Directors of the Bicuspid Aortic Foundation. Here she speaks at a BAF Conference about her experience with Bicuspid Aortic Valve disease.

Carrie Mettler - A Wonderful Athlete with BAV

Carrie with her husband, Vic
A Wonderful Athlete

During her school years Carrie Mettler was a wonderful athlete. She and her classmates would all have been amazed if they had been told then that Carrie had a condition that would one day threaten her life. Carrie had been born with bicuspid aortic valve disease (BAVD), which is often far from obvious. On the contrary, many of those who have it seem to be models of health and fitness, and in many ways they are. But that picture of health can be dangerously deceptive.

Professional Fire Fighter Training

After graduation Carrie continued her active lifestyle, including running, cycling, skiing, backpacking, and skydiving! Carrie had always dreamed of being a professional firefighter. So at the age of 27, while working as an international customer service representative for a major airfreight company, she joined the Foster City Volunteer Fire Department.  She became a certified EMT during that time, while also holding down her full time airline job.

Carrie had lots of energy and was doing what she loved. A typical day found her working eight hours at the airline, then heading to the fire station where she sometimes remained all night, returning to the airport the next morning.  She was also on the Airport Safety Committee at San Francisco Airport and always found time to fit in her workouts.
Something is Terribly Wrong

Carrie describes this as a very special time in her life. "Everything seemed to be going my way.  After passing written tests, physical agility tests and panel interviews, I was on three different cities' lists to be hired as a full time fire fighter. But then, one day out of the blue, I passed out."

Looking at her, it was hard to believe that anything could be seriously wrong. But this first incident was just the beginning. "These fainting episodes started happening more frequently," Carrie remembers, "usually starting with a feeling of impending doom.  I went through a battery of tests.  But none of them could explain my fainting spells… until one day…I felt as if I was going to die.  I kept passing out and yelled to my roommate.  'Lauren, I think I am going to die.'  I will never forget her face…it was sheet white.  She and her boyfriend took me to the emergency room where they found my pulse at 16 beats per minute.  Once I was given medication to increase my heart rate I was admitted to the CCU for observation.  That evening I felt that terrible feeling again…but even before I could reach for the button to call the nurse they were running into my room with a crash cart.  Being an EMT….I knew something was seriously wrong…then I passed out.  When I came to I was told that my heart had stopped beating for 10 seconds.  The diagnosis was sinus node dysfunction." Carrie received her first pacemaker then, ending her dream of becoming a fire fighter. She was 29 years old.

Carrie, Theresa, and Bicuspid Aortic Valve Disease

A few years later Carrie met and married her husband, moving from the Bay Area to his hometown further inland. She became interested in cardiology and began working in the business office of the practice where she was a "heart" patient.  After taking classes on EKG interpretation she went on to become a cardiology technician.  Then the pacemaker technician job opened up, and Carrie was sent for training. Here she found what she was meant to do. Having a pacemaker in her own chest gave her great empathy for others. It was comforting and encouraging for patients to know that Carrie truly understood. Eventually Carrie was recruited to work for a leading pacemaker company.

One day, Carrie's youngest sister, Theresa, called to say that she had been having heart palpitations. Given Carrie's heart history, a number of tests were done on Theresa also.  They found that Theresa had a bicuspid aortic valve and an aneurysm of the ascending aorta.  Theresa had surgery in the summer of 2000 at the age of 36.  Her aneurysm had quickly ballooned to about 6.0cm.   She received a mechanical valve fitted into a Dacron conduit, replacing both her failed bicuspid aortic valve and dilated aorta.  Subsequently Theresa developed heart block, and she too was given a pacemaker.

Carrie already knew that she had a bicuspid aortic valve, and because of Theresa's aneurysm it was decided to check her aorta also.  There it was….bulging right at the same place as Theresa's.  Carrie's aorta was not as big as her sister's and has remained stable to this day.  But after all she had experienced, including frequent bouts of pericarditis, Carrie and her husband decided that she would retire from a very demanding career and work from home on their Internet Service Provider business.  Stress is known to raise blood pressure and high blood pressure can be lethal when there is a weak artery.

In Search of an Aortic Surgeon and BAVD Expertise
Carrie and Arlys

During one of her office visits in 2002, Carrie's cardiologist showed her an article that he had read in an AHA journal, "Clinical and Pathophysiological Implications of a Bicuspid Aortic Valve".  Even though her aorta was "borderline" in size, her cardiologist suggested a consult with an aortic surgeon, especially since Theresa had already had surgery. Carrie describes what happened next. "I went to a major medical center to be evaluated at their Marfan's clinic, where surgeons specialize in aortic aneurysms.  I did not meet the criteria for Marfan's and actually felt like they wondered why I was there. Lucky for me, prior to this consultation I posted a question on a forum to a group of 'heart specialists', noting the article mentioned above, explaining my history, and asking what they thought of all of this. There was a prompt but conservative answer, and  'Good Luck'. But the most important thing that came from that post was a comment posted by a woman whose husband had already had surgeries for both his bicuspid aortic valve and his ascending aortic aneurysm. She had searched for the article I mentioned, was unable to find it on the web, and was wondering if I could share it with her. This was the beginning of a most treasured friendship."

When Carrie met Arlys Velebir through that BAVD paper in the medical literature, neither of them knew that someday they would find themselves together on the Board of Directors of the Bicuspid Aortic Foundation. The Foundation was in its embryonic stage already; that was the year it began. They did know that day in 2002 that there was very little information about bicuspid aortic valve disease, that it could be life threatening, and that it was important to find expertise. Having only spoken with Arlys on the phone, Carrie traveled to southern California and together they went to Carrie's consultation with Dr. Sharo Raissi, the aortic surgeon who cared for Arlys' husband. For the first time, Carrie got the answers she needed about bicuspid aortic valve disease and her aorta. As Carrie remembers that time, " I have to say that to those I told about flying to meet a woman I had never met in person, staying at her home, and making an appointment with a heart surgeon that she so highly recommended, it sounded at the very least….bizarre!  But this was one of those times…. I knew in my gut, this was the right thing to do.  Everything went without a hitch!  And she was right, this surgeon was the one for me. If and when I need surgery, Dr. Raissi is the one."

Something is Terribly Wrong Again

During the summer of 2003, Carrie developed a ringing in her right ear and started having "funny spells".  She would feel like she might pass out, and then get a strange déjà vu type aura.  Her heart and pacemaker were checked to make sure all was in order, and everything was fine. Around this time, Arlys brought a paper in the medical literature to Carrie's attention. This paper indicated that BAVD also is associated with cerebral aneurysms.  In November of that year Carrie actually lost consciousness. With her local doctor out of town, Carrie went to see the Nurse Practitioner about this most recent frightening episode. She agreed to order a head CT. Carrie forwarded this test to Dr. Raissi in Los Angeles, who asked his neurosurgeon colleague to review it. This neurosurgeon, 

Dr. Wouter Schievink, was the author of the paper on BAVD and cerebral aneurysms!  The test done locally seemed to show that all was normal, but the specialists in LA were not satisfied with it because the test was done without contrast. A test without contrast might detect bleeding, but an aneurysm will not show up. Carrie needed another test, this time with contrast, to check for aneurysms in her brain.

"You Have an Aneurysm Behind Your Left Eye"

Carrie with Dr. Wouter Schievink

Some days are forever seared in the memory; they can never be forgotten. The day Carrie flew to LA to be screened for a brain aneurysm is one of them.  It was a beautiful December morning in LA, sunny and clear, when Arlys met Carrie at LAX.  First on the agenda that day was a CT angio of Carrie's brain, where an intravenous contrast agent would give a clear view of the blood vessels in her head.  Next was an appointment with a neurologist. Due to a power failure there was a delay in getting Carrie's final CT results, but preliminary indications from the neurologist were that all appeared normal.  Carrie's last appointment that day was with the neurosurgeon, Dr. Schievink. Was it really so necessary to see a neurosurgeon after all? But Arlys said, "You are here, and you need to see him."  The experience Arlys and her husband had the year before with his aortic aneurysm was still very fresh. Until they found Dr. Raissi, they did not get the answers they needed. Arlys wanted to be sure that surgical expertise would evaluate the blood vessels in Carrie's brain. At the very least, since Carrie had BAVD, she could explore the potential risks of developing brain aneurysms with this specialist.

Carrie had a return flight out of LAX that same evening, and as they waited to see Dr. Schievink, Arlys and Carrie were thinking it might be tight getting to the airport on time in rush hour traffic. Then the doctor walked in. Carrie says, "I really didn't anticipate the news I was about to receive when the neurosurgeon came in and said, 'YOU HAVE AN ANEURYSM BEHIND YOUR LEFT EYE'.  It was surreal.  I just crumbled…and started to cry…. Surely you must have the wrong patient. But Dr. Schievink had questioned and double-checked the results himself before I had even seen him. It was me, and now I knew I had an aneurysm in my brain as well as my chest." Dr. Schievink held Carrie's hand as he gently told her it was safe for her to fly home that night, that he would reconfirm the findings himself, and call her soon about her treatment plan.

This was exactly one week before Christmas.  Not wanting to worry her family needlessly, Carrie had only told Theresa about this visit to LA. She asked Theresa not to say anything until she spoke to the neurosurgeon again. The next day, Carrie's heart would sink each time the phone rang. Then the call came. Carrie did need surgery.  Although Carrie had lost her father earlier in 2002, she has her Mom, another sister between herself and Theresa in age, and an older brother. Theresa  told them all that Carrie would be having brain surgery soon.

Carrie didn't waste any time.  Surgery was scheduled for January 14, 2004.  Theresa flew out from her home in the Midwest, and Carrie's husband drove them to LA the day prior to surgery.  Carrie's dear friend from northern California came down, and Arlys met them at the hospital early on the morning of surgery.  Carrie's procedure was the second one of the day.  There was a long wait; the first case took longer than planned. Finally Carrie's name was called.

During Carrie's surgery, a clip was placed at the base of her "berry" aneurysm. Carrie says, "I remember my first waking moment…thanking God.  Once I was able to understand…my neurosurgeon spoke with me and told me he found another aneurysm behind the one they knew about."  Yes, Dr. Schievink also took care of another small aneurysm that had been there, too small for the CT scan to detect.

Theresa with Carrie

Today, Carrie speaks of how lucky she is. Lucky…?  Yes, Carrie says that is the only way to feel. Because she has seen the pictures taken during surgery, and she knows how very thin the walls of the aneurysms were.  She knows that without surgery, it was just a matter of time before something would have happened in her brain. In the end, it seems her original symptoms were not related to the aneurysms, but those symptoms prompted the investigation that found them and saved her life.

What is life like for Carrie now? "Frequent echocardiograms and CT scans are part of my care.  For the most part I try not to worry about my BAVD…but I do admit feeling anxious every time a test is done…just hoping there are no changes. Today I continue to stay active. My husband and I enjoy country living amongst the grapevines and cherry trees with our dog, Monty, and our cats. We both have overcome some pretty big obstacles but we still appreciate life and make the most of it…enjoying the little things and taking it day by day."

Carrie's Mom
Carrie's family has had yet another member afflicted by aortic disease. Her Mother was diagnosed with an ascending aortic aneurysm.

The Foundation is in touch with other BAV families who also have members with brain aneurysms. As with many aspects of BAV, much more data and research are needed to understand this more fully. But as Carrie knows so well, it is best to look inside and either rule out or find an aneurysm, in both the head and the chest. It must be high-quality diagnostic testing evaluated by experts, though. Had Carrie accepted the test results done locally, a very different story might be told here now.

Screening for aneurysms in Carrie's brain and the subsequent surgery saved her life in 2004. Ongoing monitoring is important to keep her safe. Carrie is sharing a precious, costly gift, her own experience, with others through the Bicuspid Aortic Foundation. She is sharing her life, hoping to save lives.

2016 Update from Carrie

Carrie's story continues here, Carrie in 2016 - Seeking Opinions for A Complex Situation