Saturday, August 27, 2011

Richard Holbrooke and Thoracic Aortic Disease Awareness
I followed news reports of Richard Holbrooke's hospitalization closely last December. The valiant fight began on  Friday, the 10th, and unfolded over the week end - two surgeries, the first an incredible 20 hours long, no doubt accompanied by state-of-the-art medical treatment. It ended in tragedy. He died on Monday, December 13th.

I remembered years earlier standing at a bedside in cardiac ICU,  holding the hand of a survivor of an aortic dissection. I knew he had come close to death. His name was Richard too.

And I thought, as I have many times, of how important it is to detect aortic disease earlier, before it becomes life threatening. It is not always so difficult, as is sometimes implied, to find it before an emergency happens. True, not everything is known. But with what is known today, if risk factors and warning signs are heeded, technology can be used to find aortic disease in the chest.  The information on this center's website, Risk Stratification: Is My Aneurysm at Risk of Rupture , especially number 2 under risk assessment, describes the implications of milder symptoms in those with aneurysms.

I wondered if there were any warning signs before the crisis in this case. So I searched online for medical clues about Richard Holbrooke prior to December 10th, and this is what I found from April of that same year:

From Reuters,  U.S. envoy Richard Holbrooke to have heart treatment .

From The Voice in the Ear Stent Blog, by Burt Cohen, an article explaining Richard Holbrooke's angriogram. It mentions that no significant problem was found with the arteries of his heart, and he was cleared for overseas travel.

From, Holbrooke cleared to travel . It ends with the words "good news on the Holbrooke health front." It was good news on the arteries of his heart - but that is not the only reason there can be something wrong  in the chest.  I found no public record of any further searching for a problem.

Just 8 months later, on Friday, December 10th, Richard Holbrooke, while at work at the State Department, became ill. He was, in fact, suddenly in very serious trouble.

From ABC, Ambassador Richard Holbrooke Hospitalized . This article mentions being treated by medical personnel at the State Department before going to George Washington University Hospital for a blood clot. Blood clot in the lung (pulmonary embolism) is one reason for symptoms in the chest. There is no mention of any thought of his aorta at this point.

At the hospital the tearing of his aorta was found, and  many articles describe what followed - the first long surgery, his critical condition, another surgery, and his death on Monday, December 13th.

I wish I could write about a different outcome for Richard Holbrooke - that finding the arteries of his heart were normal, tests were then scheduled to look at his aorta. Testing revealed an aneurysm, his blood pressure was strictly controlled with medicine, and he was scheduled for aortic surgery. Out of the hospital in a few days, recovering over perhaps a few months, and then a return to full activity. This is the outcome that others have been fortunate to have. I know some of them, because the Foundation had a role in their search for information and help.

Each year since 2005,  the Bicuspid Aortic Foundation has chosen September as a special month - a time to raise awareness and bring attention to the aorta in the chest. We do so again this year. Why? Quite simply, thoracic aortic disease (TAD) is a killer. Despite efforts to estimate how many die from it, no one knows exactly how many there are. It should not be allowed to kill anyone.

Please help us, in September and every month, every day of the year, share information about thoracic aortic disease with others.

 It could save their lives.  

And if something is just "not right" in your chest, and doctors say it is not your heart, seek out someone with the expertise to check your aorta.

It could save your own life.

Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation

Sunday, August 7, 2011

Phill Michael - Mountain Climber Born with Bicuspid Aortic Valve - Dies

Infection presents particular risks for those born with bicuspid aortic valves. I remember being cautioned years go, when my husband's BAV was diagnosed and replaced, that at the sign of any fever we were to contact our doctor. Why? Because of the risk of infection to the heart - the medical term is endocarditis.

I remembered that today, as I read about a strong, vibrant man - Phill Michael - born with a bicuspid aortic valve. According to the press account, his BAV was replaced at the age of 32. He died July 30, 2011, at age 40, because of infection.

His friend, Chris Stanko, described what happened here: In Loving Memory: Phill Michael - 1970 - 2011 - an open wound, infection lodging in his heart, then reaching his brain. Doctors wrote about endocarditis long before there were any antibiotics to treat it. It is sobering that today, despite powerful medications, lives are still lost.

Phil's blog is a wonderful sharing of his life - scrolling down on the left, I found his Tears of Joy  about his BAV replacement experience.

Those with BAV are often described as active and athletic - and it is very true. Looking at the pictures on Phill's blog, I am reminded of all the others with BAV I know - exceptional athletes in their chosen sports and activities. They are generally healthy and very high energy, but they have a defect - a "fish-mouth" valve in their  heart - that makes them vulnerable to infection (before and after valve replacement), to heart valve failure, to aneurysms (aortic and brain).

Every time I learn of a loss such as this, I know there is much more to be done. At the Foundation, we invite you to join us in pressing for a better day for those with BAV, when the lives of these special people are no longer at risk.

My deepest sympathy to all who knew and loved Phill; it is clear as I read about him that he truly had a special heart.

Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation


Thursday, August 4, 2011

Living with Bicuspid Aortic Valve Disease

Carrie Mettler is a member of the Board of Directors of the Bicuspid Aortic Foundation. At the 2010  Conference, she spoke about what living with complex bicuspid aortic valve disease is like for her.

Wednesday, August 3, 2011

Echocardiograms - Using Sound Waves to Look at your Heart

DeAnne Paul will tell you that she loves her job - and it is a very special one. She spends her days looking at hearts. DeAnne is like a very special photographer - called an echosonographer because instead of light, she uses sound waves to see inside the chest and capture the motion of the beating heart. Below we share DeAnne's presentation (Parts One and Two) from the Foundation's 2010 Conference.

Derek Owens - Hope for Teens with Bicuspid Aortic Valves

Derek Owens loves basketball! Confronted with the need for surgery while still in high school, his dream was to play again - something that he had been told might not be possible.

In this video you will see Derek living his dream - back out on the court again. Thank you, Derek, for sharing your story and giving hope to us all.

Tuesday, August 2, 2011

Jen Lopez - Familial Thoracic Aortic Disease

Jen and Her Dad - One Family's Story

Jen's High School Graduation
On this happy day, there was no inkling
of Thoracic Aortic Disease and what it would later mean.
Jen Lopez

Jen Lopez was at work, catching up on her email that morning, when she felt the first stirrings of something wrong. Mild at first, her symptoms progressively became worse.  It was hours later, in the ER, that Jen learned what it was. Her descending aorta had torn. The aorta is the largest artery in the body, coming directly out of the heart. The aorta is normally strong and elastic, but if its tissue becomes weak and fragile it is prone to tearing and rupture. There are families in which there are no signs or risk factors for a diseased aorta other than sudden deaths in multiple family members, often at young ages. This condition is called familial TAAD (Thoracic Aortic Aneurysm and Dissection). On the day it sent Jen to the ER, it had already claimed the lives of her Dad and her Grandmother.
Jen's Dad, Louie

In 1995, Jen's Dad, Louie, was 47 years old and in great shape. Outgoing, gregarious, and active, he often was found on the basketball court or the softball field. From all appearances, Louie could reasonably expect to enjoy many more years with his daughter, Jen, and wife, Terry. A successful oldest son, he was the center of his extended family, which included his mother, father, brother and sister and their families.

During a rare doctor's visit in September of that year to stitch up a soft-ball injury, Louie's doctor recommended a chest x-ray based on the fact that Louie had never had one as an adult.  The chest x-ray revealed a 5.6 cm aneurysm in his aorta and a previous Type B dissection in his descending aorta.         
Waiting for Surgery

Louie was referred to a cardiologist who recommended he wait to schedule surgery until the aneurysm had grown to 6.0 cm.  In the meantime, he was put on blood pressure lowering medication and instructed not to lift anything heavy. 

Six months later, while at his office on a Friday morning, he experienced searing pain in his mid-section and abdomen.  Within half an hour he was at the Emergency Room of The Queen's Hospital in Honolulu.  A CT scan showed a Type B re-dissection of the aorta (Louie knew nothing about the original dissection, including when it may have occurred).  This re-dissection went further this time, extending down into the abdomen, and the aneurysm had grown from 5.6 cm to 5.8 cm.  The cardiologist said he could not perform surgery yet because of the new aortic tear.  He wanted to wait at least a week, hoping for some healing during that time. 

Forty-eight hours later, in the hospital, as Louie was talking to the cardiac surgeon, the aneurysm ruptured.  He was rushed to the operating room, where three and a half hours of surgery could not save his life.  An acute Type A dissection of the ascending aorta had occurred concurrently with rupture of the aneurysm. The dissection disrupted blood flow to the brain and caused an extensive heart attack.
Five Months Later, Jen's Grandmother

Five months after her son's funeral, still reeling from this sudden loss, Louie's mother (Jen's grandmother), age 67, passed out on the grass while playing golf.  At the emergency room of a hospital she was pronounced dead.  An autopsy revealed that she too had died from TAAD. Like her son, Louie, her aorta had given way.

And then the family started remembering . . . .

             • Uncle Sonny, Louie's mother's brother. Dead at 53 from a sudden blood vessel bursting.

             • Papa, Louie's mother's father, father also to Uncle Sonny. 
               Dead at 55 reportedly of a burst blood vessel in his head.

             • And now, Louie and his mother.

Early, sudden death in a man, 2 of his 4 children, and his grandson - what was going on? There were no clear answers.  Some form of familial aortic disease was the reason behind these deadly events.

Very special Mother and Daughter
Their sparkling brown eyes give no hint
of all they have been through together.
Jen in 2003

Now we fast forward 7 years to 2003. Jen was living in the Washington, D.C. area, working for the Department of Justice, F.B.I., and traveling about half of each month.  In September she accepted a one-month assignment to Saudi Arabia.  Arriving back home on a Sunday in November, Jen was back in the office at her desk on Monday morning, but not feeling well. 

Thinking perhaps it was heartburn from the daily coffee, she ignored the discomfort, but soon experienced back pain, then, shortness of breath.  After several hours with no relief, her in-office medical personnel called an ambulance, and Jen was on her way to George Washington University Hospital (GWUH).   The pain never let up and the shortness of breath continued while ER personnel worked to understand what was going on. After several hours, a CT scan with contrast was ordered.  It revealed a Type B acute Aortic Dissection.  Jen's first thought on hearing that was, "That can't be right.  That's what my Dad had.  And he died." 

Jen's descending type B aortic dissection started just below the aortic arch and extended thru the abdominal aorta and on down into the left and right iliac arteries.  And, though she had never had high blood pressure before, there in the hospital her blood pressure and heart rate were high and hard to control, even with blood pressure lowering medication delivered by IV. Blood flow to Jen's kidneys was normal, although one kidney was being fed from the dissected portion of aorta.

Type B descending aortic dissections typically can be treated with medicine, not surgery. The initial concern was to control Jen's blood pressure and heart rate to prevent further propagation of the dissection and also prevent compromise of the renal and mesenteric arteries. In type B dissection, the hope is that with lowered blood pressure the dissection will not progress.

Four days after Jen's admission, an aortogram was performed. This procedure has historically been used to visualize the aorta, but is no longer used by aortic physicians, especially in cases such as Jen's. 

Jen stabilized and after 2 weeks in the hospital, she was released with instructions for care and appointments with a renal specialist. 
Chest and Back Pain - Again

Only 22 hours later, after just one night at home in her own bed, Jen was having lunch with her cousin and a friend when she started to feel ill. She collapsed on the sidewalk outside the restaurant with intense chest and back pain.  An ambulance returned her to GWUH, where cardiac catheterization revealed a new dissection, but not in Jen's aorta. 

This time, one of her coronary arteries, the left main, had dissected back to the 'mouth' of the artery. There was no blood flow in the anterior descending portion of the artery.  A surgeon patched the 'mouth' end of the artery with a pericardial patch and used an internal mammary artery to by-pass the anterior descending, restoring blood flow to Jen's heart.  

Heart Attack During Surgery

At some point during surgery, Jen had a myocardial infarction (MI), a heart attack. It was later discovered that it was centered in that lower anterior part of her heart where the blood flow was compromised.  

Jen survived the surgery but her recovery was complicated by congestive heart failure as a result of the MI.  The heart failure eventually necessitated the implantation of a Bi-V ICD (bi-ventricular cardiac synchronization device and defibrillator) in her left chest. 

But Jen is alive!  And over the next 3 years her health stabilizes.

Jen in September 2006

Three years later found Jen living with the dissection in her descending aorta, remaining on blood-pressure lowering medication and a heart medicine to battle the ongoing heart failure.   Because of her youth, the hope was that her heart muscle might yet re-model and grow stronger. Two of the four valves of her heart might need to be replaced eventually. 

Jen and Terry traveled to southern California in September 2006. It was intended to be just a brief visit. In addition to attending Bicuspid Aortic Foundation activities for TAAD Awareness 2006, Jen also had follow up tests and appointments. One of those tests, a 64 slice CT scan, revealed a new development - another dissection, this time in her other coronary artery. When told of the new tear, Jen and Terry knew when it must have happened. On the evening of August 31st Jen felt a slight pain in her chest. Locally, nothing was found. Without the fast camera and high resolution of the relatively new 64 slice CT, this new tear would not have been found. But now the approach to treating Jen had to change. She would not survive another tear in the arteries of her heart. Jen needed a new heart.
Waiting for A New Heart, December 2006

Jen was asked to remain in Los Angeles and undergo evaluation for a heart transplant rather than returning to Hawaii. A trip of a few days turned into months as she remained in southern California, waiting for a new heart. 

December 20, 2006
Jen ready to leave the hospital with her new heart

On the morning of December 20th, the phone rang. There was a heart for Jen. With that call Jen's plans for breakfast with Terry, whose plane from Hawaii had just landed, quickly changed. They would  meet at the hospital instead.

While waiting in a hospital room, someone asked Jen if she was nervous. She calmly said that this was so much easier than before, when she was rushed to the hospital in pain. For Jen, who has survived three dissections, this was the first time she would undergo surgery by choice, not as a life-threatening emergency.

Those who watched this brave young woman wheeled through the doors to surgery saw in that moment the same courage and gentle strength with which Jen has traveled the world, protecting the freedoms of us all.

Waiting on the other side of those doors was a new heart with healthy blood vessels and the skilled hands of the transplant team. Among the hands there to give Jen a new heart were those of the aortic surgeon who had steadfastly championed Jen's cause, determined that Jen and others with TAAD be given every opportunity to triumph over it.  

It seemed very fitting that it was in December, the month of Christmas and also her birthday, that Jen received the gift of life, a new heart. Somewhere, in the midst of their sorrow, may a family be comforted in knowing they have given the precious gift of life to another.  The heart of their loved one has a new home. It beats now in Jen's chest.  

Jen continues to do well today. In the video below, Jen and Dr. Sharo Raissi, who opened many doors of hope for her, tell her story.

Monday, August 1, 2011

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About Us

Bicuspid Aortic Foundation Board of Directors

Bicuspid Aortic Foundation Mission Statement

The Bicuspid Aortic Foundation's mission is to:

• promote the well being and longevity of those affected by bicuspid aortic valve disease, thoracic aortic aneurysm and dissection, and any and all related conditions

• provide information and educational resources for patients, their families, and the general public

• encourage and support medical professionals and research scientists in exchanging information and advancing understanding of bicuspid aortic valve disease and thoracic aortic aneurysm and dissection, their underlying causes, and the optimization of care for these conditions

• seek research funding for bicuspid aortic valve and thoracic aortic aneurysm and dissection conditions