Thursday, December 26, 2013

Bicuspid Aortic Valve and Life on the Edge

BJ Sanders at Machu Picchu
7,970 feet above sea level

Those born with  BAV are
 typically high energy adventurers.
BAVers live their lives on the edge!
Yes, the view is fantastic.
But an aortic crisis lies
 just over that edge.  
The challenge is to keep them 
far enough from the edge to be safe,
and still allow them to enjoy
active, vibrant lives.
The problem is that no one knows
exactly where the edge is!

My dear friend, BJ, recently shared the story of her path to surgery 
after 10 years of watching and waiting. 
I have been privileged to walk along with her 
during this time in her life. 
Below I share my side of the story.

 Earlier this Year

BJ, Arlys, Carrie Mettler (l to r)
I love this picture from a BAF
Conference a few years ago!
Both of these ladies are pictures
 of health, and expert at throwing
 curve balls at their doctors!
"Good morning Arlys....Since today is Sunday I hate to bother you....only if you get a chance could you give me a call....if not I will try to catch you on Monday....Thanks...BJ"

Uh, oh! I sensed trouble behind that message and quickly called my friend. She told me that she had experienced major chest pain. Immediately Dr. Raissi helped her lower her blood pressure, and she began to arrange for an imaging study locally. (It shouldn't have been difficult, but just getting the testing became a major challenge! Not a good situation for a BAVer with symptoms!)

BJ was still experiencing episodes of chest pain, and had made plans to come to LA for a consult (prepared to stay for surgery) when I received the following:

"Hi Arlys, 
I wanted to share with you that I had another episode of chest pain. It scares me that it could possibly be aortic pain. Now I am actually afraid. It is a terrible feeling. I have not lived in fear for a decade. I am really grateful to be coming to LA.... Love you, BJ"

Now, her pain was happening at rest, with low blood pressure, once even waking her out of sleep!

Arlys to BJ:
"Just in case have a plan to say the right thing to get help there in case of emergency. I texted Dr. Raissi as want him to know."

Arlys to herself: 
"BJ is complicated - delicate tissue, diabetes, partially paralyzed diaphragm, possible blood clotting issues . . .  She has got to get here for surgery! She will not survive surgery back there, especially an emergency! We all love her, and her family needs her so..." 

Arlys to Dr. Raissi:
"BJ had another pain episode lasted about 12 minutes and bp was "normal".... Wonder if ... room for even lower pressure? could she come here now . . . .?" Dr. Raissi adjusted her medication yet again, driving her blood pressure even lower. The chest pain did not reappear in the short time before her surgery. I believe strictly lowering her blood pressure kept BJ back from that dangerous edge of aortic tearing/rupture until she had surgery. 

How close to that edge did BJ come?
No one really knows.

BJ saw a physician after she returned home who told her that she really was on the "low end" of the numbers that are used to guide the surgery decision. He implied that maybe she didn't really need surgery yet. I mention this because those guidelines are for patients without chest pain. BJ had chest pain, and the numbers don't matter when the aorta begins "talking". This paper from Yale discusses this: Symptoms Plus Family History Trump Size in Thoracic Aortic Aneurysm

Would BJ have fallen over the edge if she had met and listened to this kind of thinking in her search for help? Remember, it is easy to be in denial. When physicians do not recognize aortic pain, perhaps labeling it anxiety, how close to the edge do people come? I have heard of some that fell over. 

Surgery At Last

BJ's ascending aneurysm
I arrived very early on the morning of BJ's surgery. We had spoken, but I was anxious to see her. She looked just wonderful, her expressive brown eyes bright and sparkling.  Even I, who know better, marveled at the need for this surgery. She looked so perfectly healthy! Looks are deceiving when it comes to those with BAV. With beautiful skin and vibrant energy, their youthful appearance portrays someone glowing with health. I reminded myself of what was inside her chest, threatening her life. We had to do this!

Pre-Op and Waiting

For years BJ shared the message of Hope with others. 
Now Hope kept her company after surgery!
In pre-op, BJ had her own little cubicle. I was glad for that private time together. She told me she was at peace, and I knew she was. I was so thankful we had safely reached this moment in her journey. We stood on the threshold of the door to her future, engulfed in a Climate of Hope!  

We waited through the hours of her surgery - her husband, Ron, her son, Judd, and I there at the hospital, knowing many others were there in thought with us. BJ has a lovely family, and it was very easy to be there with them. 

Never Trust a Bicuspid!

At some point, "Never Trust a Bicuspid"  has become my motto. Despite efforts to predict what they will do, people with BAV excel in throwing curve balls! Walking in to see BJ that first time after surgery, I was wondering just what surprises she might have in store. 

Dr. Raissi's patients generally look very good after surgery, even in ICU, but I did not quite like my first glimpse of BJ's face. It wasn't long before I knew why, as she threw that first curve. Bright red blood came pouring from her drain tubes! She chose the right time to do this, with her surgeon standing right there to expertly catch it. What would be next?

The next morning, the word initially was good. BJ was off the respirator and sitting up, right on schedule. I decided to wait until afternoon to see her. I should have known better! It wasn't long until I heard from her son, telling me that BJ was going back on the respirator. My heart dropped all the way to my toes!

I had been afraid of problems with BJ's lungs. Some years before, she had been injured during a surgical procedure on her shoulder, resulting in paralysis on one side of her diaphragm. As I drove those miles to the hospital, I tried not to think of the horror stories about respirators and infection.

So what did I find on arrival? Another curve ball! BJ was sitting up, breathing on her own. As long as I have memory, I will never forget that moment. This was one to be thankful for!
Dr Raissi with BJ in ICU

Seconds before they were to put that tube in, BJ somehow turned a corner! Later, Dr. Raissi told us he could see in BJ's eyes that she was better, despite her "numbers", and asked them to wait. Somehow, in that moment physician and patient understood each other, and BJ dodged the respirator! An indescribable moment. 

When we compare memories now, BJ remembers me being there that day, holding her hand. I remember vividly how happy she was to see me walk in. I also remember her saying how tough it had been that morning.  "It was so hard to breathe...."

I waited for more curves -  problems with healing due to her diabetes perhaps. Thankfully that did not materialize. She did manage to find some other bumps on her road to recovery. It was her very own journey, and like many with BAV, she chose her own path! Being under the care of someone used to catching BAV curve balls made all the difference.

I am very grateful as I write this, retracing her BAV journey, that she cleared every hurdle beautifully. I remember watching her walk while still in ICU, and thinking how strong she is. She needed to be.

Who Are these Complex BAVers?

There are variations in those with BAV, and I believe BJ is one of the more complicated. Perhaps what she has deserves its own name, beyond BAV disease, but no one has such a name. The rods that keep her spine straight, her flexible joints, those difficult pregnancies, volatile blood pressure... all tell us her body is special. It concerns me that all these factors may not be taken into consideration in those with BAV. Someday, perhaps, that will change. For now, BJ received the individualized care that her very special body needed. 

How many others need, but do not receive, that care?

In the final few days of 2013, I am happy to share my view of BJ's experience, and to remember with joy my special friend, safely home with her family and friends once again!

Thank you, 
dear BJ,
 for sharing your life 
with us all,
Creating a 
Climate of Hope.

      - Arlys Velebir

Thursday, November 28, 2013

A Dream Come True - My Life with Bicuspid Aortic Valve Disease

My Journey Through Fear, Complacency and Peace

November 2013
My aortic journey began 13 years ago when I found out that my aorta was growing. I began to search for centers that had aortic programs. I became more confused as I went from center to center. I was initially misdiagnosed as having Marfan syndrome and surgery was immediately recommended. I was very afraid of aortic surgery which required hypothermic circulatory arrest (lowering body temperature and arresting circulation); therefore, I continued to search for an expert opinion. I was finally diagnosed with a bicuspid aortic valve. It seemed like no center knew what to recommend to someone with a bicuspid aortic valve and ascending aortic aneurysm.

For three years I lived in fear of my aorta rupturing. I had been told different levels of lifting at each center. This was a concern since my profession as a physical therapist required heavy lifting daily. I had labile high blood pressure that was never addressed. To my surprise I met a kindred soul on the internet whose husband had previously had surgeries for his bicuspid aortic valve and ascending aortic aneurysm. She was extremely knowledgeable, and I clung to her every word. I had truly met my Aortic Angel, Arlys Velebir. Little did I know that very soon over the next decade she would become the Aortic Angel for hundreds of people! Once again I began my search on the internet and to my surprise I found a new website from a hospital in Los Angeles that actually acknowledged bicuspid valves.

The website said to leave a message. I have never been so surprised when an aortic surgeon was the one to respond to the message. He spent a longer time on the phone with me than all of my appointments put together. I remember thinking maybe this surgeon needs more business! The first thing he did was to address my blood pressure and assist me in lowering it. I learned more about my condition in that one phone call than I did throughout my entire three year journey! The surgeon was Dr. Sharo Raissi who would follow my scans for the next decade and eventually perform an aortic aneurysm repair and a bicuspid aortic valve replacement for me.

Shortly before traveling
to LA for my surgery
A decade ago I was blessed to be invited to participate with Arlys Velebir, Carrie Mettler, Fiorenza Courtright-Lucas and Dr.Sharo Raissi in establishing the Bisuspid Aortic Foundation. Our most urgent dream was to have an organization that would assist those with bicuspid aortic valve disease to find accurate information about their condition and to help them locate expert care. Throughout a decade I believed firmly in practicing the ABC’s for Aortic Care (A. Accurate Testing, B. Blood Pressure Control and C. Comprehensive, Individualized, Care). But I gradually slipped into complacency with my aortic care. My aorta was stable for a decade. I attributed this to excellent blood pressure control and began to believe that perhaps my aorta was not fragile and would always be stable. 

I mentioned to Dr. Raissi that I was late in getting my MRI, but that I was not worried because my blood pressure was so low. He quickly and as always kindly responded, “It is good to have a low blood pressure, but it is no substitute for good surveillance.” This statement immediately set me into action.

I was also in denial about the pain I was having in my chest which radiated to my jaw. When I experienced this pain I did have fear, but as the pain subsided I would choose not to report the pain to anyone. As the pain became more frequent, I did share about it with my dear friend, Arlys. She kept saying to me that aorta’s can "talk to you". She shared with me numerous stories she had heard through other patients. Sometimes they would go to the ER, and it would be dismissed as anxiety! I am so grateful for Arlys, who continually expressed her concern about my chest pain. Almost simultaneously my MRI was read by Dr. Raissi, during my time of increasing aortic pain.

Years ago I prayed that when Dr. Raissi said it was time for me to have the surgery I would have a sense of peace and accept his advice. To be honest, it did catch me totally off guard, but I knew I had the best expert care possible. I had so many questions, but when he so gently said, “BJ, we just don’t want to lose you” God gave me that sense of peace that I had prayed for so long ago. My husband and I quickly made arrangements to fly 3,000 miles from coast to coast, meet with Dr. Raissi to ask our questions, and make our final decision. Dr. Raissi answered all of our questions and calmed any fears.  I knew I was in the hands of a blessed cardiothoracic surgeon.

In ICU, with Dr. Raissi,
September 2013
I always knew that Dr. Raissi was a special man and medical caregiver. For many years he always instilled confidence in me during my “watch and wait” phase for my aorta. But it was not until I experienced his servant hood during my surgical repair of my ascending aorta and bicuspid aortic valve replacement that I truly know the depth of his heart.  He always has a way of helping you see a complication in a positive light. His intelligence and patient instruction alleviates any fear that might arise.  His genuine compassion is so comforting especially during a complication or crisis.  In all situations he is calm and decisive. He follows you throughout pre-op, surgery and post op. He stays with you throughout any complication. He gives superb care post hospitalization and is always readily accessible when you need his advice. When patients tell him how grateful they are for him, he quickly responds, “No, it is the other way…I am grateful for you! I am grateful that God can still use me to help others!” He epitomizes humility. He does not have an ounce of arrogance. Truly he is a blessed servant to all of his patients.

Now in my third month after surgery,
November 2013
It is my hope that my story will inspire people with bicuspid aortic valve disease to avoid becoming neglectful of the ABC’s of Bicuspid Aortic Care.  Always insure that you have A. Accurate Testing, B. Blood Pressure Control and C. Comprehensive, Individualized, Care. Please don’t let yourself drift into complacency with this “OTHER KILLER IN THE CHEST”!!!

                      B.J. Sanders
                      Board Member, 
                      Bicuspid Aortic Foundation

Sunday, October 6, 2013

Emerson - Our Miracle Boy

Baby Emerson was diagnosed with BAV and AS at 11 weeks
Statistics say that a baby with BAV enters the world every 12 seconds. We also know that they are more likely to be a little boy rather than a girl. Some of them may live many years before their BAV is even discovered. Today, there is increased focus on these adults with BAV and their families.

But there is a group of babies born with BAV who need attention and intervention while still infants or children. They are very much in need of understanding, care, and advancements to help them also.

Who are these little ones, and what is done to help them? 

Statistics may help put the frequency in context, but numbers can be so cold and impersonal. They can never convey what it means to these little ones and their families to face seemingly endless doctor visits and surgery. In order to express the true meaning of BAV in infants and children, we must share their stories.

And so, with the help of his parents, here we share the story of a beautiful little baby, born just a few months ago - Emerson - Our Miracle Boy

Thank you, Nicole and Kyle, for sharing him with us.

 May we all learn the lessons
 that Emerson has to teach us
 about little ones affected by BAV,
Creating a Climate of Hope.

Journey of the Heart

Dr. Sharo Raissi, Lia Nicandrou, Arlys Velebir, Father Prodromos
At the Bicuspid Aortic Foundation, we are very touched and encouraged whenever we hear from those dealing with BAV and the complications of thoracic aortic disease. We take our mission very seriously, knowing that all around the world we share a common experience with families who have BAV and variations of thoracic aortic disease.

The story we share here, of one young man's journey seeking answers and help, spans the many miles between the island of Cyprus and southern California. It would seem highly unlikely that a young man living in a monastery high in the mountains of Cyprus would find his way to the Bicuspid Aortic Foundation. It would seem even less likely that doors would open that would make it possible for his surgery to be performed here. And critically important for this young man, he would find himself in hands that not only could deal with his ascending aortic aneurysm, but also with the strands of tissue clinging to his 13 year old mechanical valve.

His story, in his own words, is on the BAF website - Journey of the Heart .

We are very grateful that Father Prodromos has so graciously shared his experience with others,

Helping us in our mission, 
and creating a climate of hope,

Arlys Velebir
Chairman and President,
Bicuspid Aortic Foundation

Wednesday, August 28, 2013

Keep Your Heart in the Game!

If you want to find someone with BAV (bicuspid aortic valve), head out to a sporting event or go where there is some form of physical activity. Exercise seems to draw them like a magnet, and they are exceptionally good at whatever their favorite sport or activity may be! 

Derek Owens, who currently plays
for the University of Hawaii, Hilo

Brent Warren  hitting a homer!

Whether on the basketball court or the baseball diamond, these athletes play with all their hearts! 

Along with team sports, over the years we have heard from many others.... runners, swimmers, bicyclists, rock climbers, wrestlers, body builders, triathletes... even race car drivers! 

The challenge we face with BAV is keeping these vibrant, gifted people safe, because some of those sports enthusiasts we can never meet in person. We heard from their families when they died suddenly, without an opportunity to have lifesaving surgery. 

BAVers of all ages
remain very active!
BAV triathlete 
At the Foundation, we talk about comprehensive, individualized care. We believe that if BAV individuals and families received that kind of care, it could greatly minimize the risk of untimely death or injury. Given their extraordinary gifts and physical strength, it is very possible that those with BAV could exceed the average lifespan of the general population. However, they are at risk of some very serious complications: endocarditis (infection), aortic valve failure (stenosis/narrowing, regurgitation/leaking, or both), thoracic aortic aneurysm, thoracic aortic dissection, and in some instances brain aneurysm. There is a great deal still not known, and untimely injury and death continue to occur in the United States and around the world, despite the aid of modern technology.

Chuck Doherty Running
Marathon in 2010
One of those who tragically lost his life, cut down in his prime due to BAV complications, is Chuck Doherty. On September 15th, a 5K Run is being held in his memory. There is still time to register for the run, come out and cheer on the runners, or simply donate in Chuck's memory at the 5K for Conquering Aortic Disease page.

We are very grateful for donations and support that will help keep the hearts of those with BAV beating and healthy! There are physicians in centers who need our financial support to conduct research. Visit the BAF website to find out how you can donate to the cause.

Join us in Creating a Climate of Hope!

Monday, May 27, 2013

Alicia's Story - From Fatigued and Fainting to a New Life!

Alicia, 3 months after surgery, hiked 3.1 miles!
Her Mom remembers Alicia as a healthy baby. At the age of 7 she developed asthma and was also hospitalized with pneumonia, suffering staff infections. From then on, all was well as she grew and went through her teen age years.

But things changed for her at the age of 20, following the birth of her first child, when she began to have complications.

It was during a routine exam that her doctor heard a heart murmur and suggested she see a cardiologist. At her first cardio exam, she was diagnosed with a bicuspid aortic valve. She was told she was fine and would need to be monitored annually. But she had some difficulties during her pregnancy - dizzy spells and fainting. She gave birth to her daughter in 2009.

Her baby daughter was very ill during her first year of life and was ultimately diagnosed with a PDA (patent ductus arteriosis). At one year of age, the PDA was closed, resulting in a very healthy child ever since.

But for Alicia, all was not well. She tired easily, was short of breath, and often felt faint. When she visited her cardiologist in May of 2011, she was told that nothing had changed.

She was also told that she likely would not need heart surgery until she was in her 40's.

How could she face the next 20 years feeling like this? 

The new year, 2012, found this young mother lightheaded, tiring easily, sometimes fainting, and suffering from migraine head aches. Her cardiologist told her she needed to live with these symptoms, and that she was too young to have surgery. She was also cautioned not to have more children.

The months passed, and the symptoms persisted. Alicia was extremely fatigued and fainted often, also suffering frequent migraines. Her cardiologist prescribed metroprolol for her migraines, which lowered her blood pressure and made her feel worse than ever. Alicia had chronically low blood pressure, and the metroprolol took away what little energy she had.

Their search for help led this family to the Bicuspid Aortic Foundation, looking for information, support, and hope. Alicia sought a second opinion from Dr. Sharo Raissi, who chairs the Foundation's Scientific Advisory Board. She underwent testing that revealed she had an aortic aneurysm along with her BAV. It was clear that Alicia continued to feel unwell, and she could not keep up with her very active toddler. In the fall of 2012, after having further tests, plans were put in place for surgery to remove the aortic aneurysm. The bicuspid aortic valve would be replaced if necessary. Amazingly, in December Alicia completed an extensive pharmacy technician program, despite feeling very tired and weak.

Alicia had surgery on January 16, 2013, at St. John's Health Center. The aneurysm was removed and her bicuspid aortic valve replaced with a bovine prosthetic valve. She now looked forward to feeling better, as her body healed.

By March, she was doing very well indeed. Alicia proved her new found energy and stamina by completing a 3.1 mile hike just three months after surgery (pictured above). What a dream come true! Today she feels great and is so happy. She now knows what it feels like to be the healthy woman she was meant to be! And her daughter notices it too. For the first time in her three years of life, she has a healthy, energetic Mom. This is a very happy family!

Alicia is now well informed about her health. She knows that some day the bovine prosthetic valve will require intervention. In the mean time, she is doing all she can to take charge of her health and her life!

May Alicia's story inspire others to listen to their bodies, and seek the help they need until they find it.

Thank you, Alicia, for sharing your story, and creating a climate of hope.

Sunday, May 26, 2013

Paying It Forward - Touching Many Hearts

From One Heart
To Another

"When you helped me, you helped many people......"
Pay It Forward!
Invisible Bonds of the Heart
How do you describe those that have walked together through life and death decisions? I doubt that there is a word in any language. They are connected in a very special way, through an invisible thread between them. Each end of this thread is tied with a very special knot, deep inside their beings. It is a bond that cannot be broken. Over time, as one person helps someone, and they in turn help others, hearts bond together in an unbreakable web. Each one of us can be a part of this wonderful connection. All by paying forward what someone else has done for us.

I have a connection like this to one particular woman in the mid western United States. Her bicuspid aortic valve and aneurysm surgery failed, and only a few months after her surgery she was in serious trouble. It is beyond my comprehension why her physicians did not help her get to a major heart center. Perhaps they thought she was beyond hope. Apparently one of her doctors thought that, because he later told her he expected she would have been dead rather than coming to see him in his office again.

She was not beyond hope! I helped her go to a well known aortic surgeon in another state, and she is not only alive today, she pays the gift of life forward at every opportunity!

Recently, I visited a young man in the hospital. He had just had a much needed surgery. The valve and aneurysm that both threatened him can no longer harm him! He found his way to help not through referrals from physicians, but through his own diligent research and because this special woman reached out to him and pointed the way to help.

This young man is one of several that she has helped. In a recent email, she told me about them. She closed her email this way,

"These are all people who have a chance to live long lives, 
because they are getting expert care.
That's what you taught me, Arlys, that there's care,
 and then there's expert care.
I wouldn't be alive right now, if I hadn't had expert care, so thank you."

You too can pay it forward.
After first getting expert help yourself, share it with another. 

In this way, all around the world
 we join together in this
 indescribable connection of hearts,
Creating a Climate of Hope.

Best wishes,
Arlys Velebir
Bicuspid Aortic Foundation

Sunday, April 21, 2013

Come Out and Run! The 5K for C.A.D.: Conquering Aortic Disease

Chuck Doherty Running in 2010
On April 15, 2012, the unthinkable happened. 

A loving husband and father, a strong young man, was struck down in his prime. Charles "Chuck" Doherty was just 34 years old when he lost his life without warning. Why? Chuck was among the children born in 1977 with a bicuspid aortic valve. He died from complications related to that valve following what was for him an ordinary eight mile run.

You can read more about him and his family here, at the Charles A. Doherty Memorial Foundation. Those of you reading this who have a bicuspid aortic valve yourselves will recognize that you share some things with him - his love of athletics and activity in general are a common theme in those with BAV.

Reading about him, we would all have loved to know Chuck. We can never meet him now. In a moment of time, he was taken.

There is something we can do. We can tell his story, and remind doctors that valvular heart disease is not just a risk to the elderly. It can take the young and the strong.

We can also raise funds that can be applied to research. There are questions that need answers. Do those with bicuspid aortic valves need special guidelines for monitoring and intervention for their BAV? After all, those with BAV are extraordinarily active and athletic. Given their unusual endurance and strength, could at least some of them follow a different path than their tri-leaflet counterparts? Could their first obvious symptom be sudden death?

Chuck Doherty lost his own life, but through those who love him, he will save the lives of others. Let's join them in the quest to Conquer Aortic Disease. Register for the race, or donate here.

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