Sunday, December 7, 2014

Why Do We Need Heart Valve Research?

Heart valve research?  Why?
I thought that was solved a long time ago!

 Can it be true that with all the modern advancements,
people are still suffering, still dying?

Young, strong, otherwise healthy people?

Yes, sadly, it is true.

Won't you help us fund badly needed research?

The Big Three

Perhaps the "Big Three" dangers for someone born with BAV are 
  • bicuspid aortic valve stenosis, regurgitation, or both
  • aortic aneurysm which may lead to tearing (dissection) or rupture
  • endocarditis - infection of the heart
While some may avoid all of them, others may find themselves in a fight with one, two, or all three at various points in their life. It is well to remember that as we admire their athletic abilities and glowing, healthy appearance.

Why Fund Valve Research? To Save Lives

Sudden death is a risk for someone with a diseased aortic valve - whether that valve has stenosis (narrowing), regurgitation (leaking), or both. 

Chuck Doherty 
Chuck Doherty, BAV Stenosis
Chuck Doherty, age 34, died suddenly at the end of a routine 8 mile run. His BAV had been diagnosed. Despite being under medical care, Chuck lost his life. Why? Were they waiting for symptoms that never happened? Did someone fail to appreciate the severity of Chuck's stenosis, especially in someone so young? Where is the information that would help a young man and his doctors make the right choices, so that Chuck would still be alive today? Questions without answers.

Chad Rogers, BAV Regurgitation
How could someone like Chad, diagnosed with BAV as a baby, suddenly lose his life at age 30? No, it was not an aneurysm that tore or ruptured. It was his bicuspid aortic valve itself that led to his sudden death.

His family has told us that Chad was very active in both cross country running and baseball in high school. The last few years, he ran marathons, completing about 12 full marathons and 3 half marathons. Over all those years, he had no restrictions on his physical activity. He had no obvious symptoms and appeared in good health until the day he died. Why? Why did this happen? How can it be prevented?

Why Fund Valve Research? To Have Better Lives

How Many Surgeries?

Robby Benson - First Surgery at 29
Robby Benson had his first BAV surgery at the age of 29, and in the years since has had three more. Here is a blog post with more information and references to his book.

Derek Owens - First Surgery in High School

What does the future hold for those whose first surgeries are at young ages? How many surgeries will they ultimately face? Can we find better valve solutions for them? There are good choices today, but a replacement solution as good as their own valve should have been still eludes us. Can we find that "ideal" valve solution?

Other BAV Complications

My husband's BAV was replaced in his late 40's. It saved his life then. There was no recognition that he might be subject to complications because he was born with BAV. Fifteen years later, the valve that saved his life was the source of a terrible injury to him. No one understands why his body formed not only scar tissue but fine strands on that mechanical valve. Some of the strands broke off one day, showered his right brain with particles, and changed his life forever. 

Some Cannot Have Surgery at All 

In addition, those with BAV who are not well enough to survive surgery still face a gradual decline resulting in death due to their diseased BAV. Here is the story of one courageous woman unable to have surgery: Lily's story.  

With Your Help, We Can Do Better

I hope haring these stories has helped explain why the Bicuspid Aortic Foundation has chosen to fund research of the bicuspid aortic valve itself. 

Your donation in any amount can help us make a difference in BAV lives everywhere. You can donate on our special page, Take the BAV Challenge, or by mailing a check to the Bicuspid Aortic Foundation. Here is our mailing address: 

Bicuspid Aortic Foundation
30100 Town Center Drive, Suite O-299
Laguna Niguel, CA  92677

We are an entirely volunteer organization, and 100% of all funds donated for valve research will go directly to that effort.

Together, through funding research,
may we create a climate of hope.

Best wishes,
Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation

Sunday, September 21, 2014

Bicuspid Aortic Valve and Aneurysms - Ten Years Later

Doug in September 2004
Ten Years Ago, in 2004

Ten years ago, on September 20, 2004, Doug Grieshop died when his aorta ruptured. He had an undiagnosed bicuspid aortic valve and an aortic aneurysm in his chest.

Eighteen months earlier, Doug went to the ER with chest pain. Nothing was found then. It was left for the coroner, after his death, to find the scar of an old tear, next to the rupture site.

A weakened, diseased aorta may "hang on" for some time, because it has an outer layer designed to be stronger than the others. For Doug, that outer layer held on for a year and a half. When it broke, there was no time to save him. He was instantly gone.

What would happen ten years later, in 2014?

If Doug went to the ER, would they still check him for a heart attack, find he did not have one, and stop there?

Or would they go further? Would they find his bicuspid aortic valve, his aorta bulging dangerously?

Would today's technology be able to "see" the small tear in his aorta? If not, would they still take his chest pain and aneurysm seriously?

Would they ask him about his family history? His uncle died in the same way. Family who saw them say that in death they both looked like they had "exploded". A horrifying memory.

Would they refer him for surgery in time?
 Ten years later, would someone save Doug?

In some communities in the United States, we know what happens today. Some aortic emergencies are being found in time to save lives. But we hear of the dying. Still. In 2014.

For one man, it happened like this.

Always healthy and active, he arrives in the ER. He tells them about a feeling of pressure and burning in his chest. He is short of breath. They check for a heart attack. Negative.

They know there is another killer in the chest - pulmonary embolism (blood clot in the lung). They rule that out too.

They keep him in the hospital, continuing to speculate. The family hears words like acid reflux/heart burn. They even hear about sleep apnea! Eventually they hear he has a bicuspid aortic valve that might need surgery "some day".

Although BAV is a red flag for aneurysm in the chest, finding it did not raise the alarm. Was it because there is a persistent impression that bicuspid aortic valves are relatively harmless?

For this man and his family, many hours went by, spent guessing about conditions that are not immediately deadly.

At times, he felt better. But all of a sudden, something terrible happened inside. At that moment, it was already too late. Many hours were spent desperately trying to save him. It was impossible. Time had run out.

He had bled massively inside his chest. The respirator would continue to breathe for him. His brain was dead.

Is this the best we can do, 10 long years later?

There are many unanswered questions and conflicting guidelines for those with bicuspid aortic valve.
We know we need more answers.

However, we ask for those with BAV what we ask for everyone else. We ask that this often varied condition be viewed with respect for its potential deadliness.

Please do not forget that the diseased aorta is right up there with heart attack and pulmonary embolism.
It is a killer.

There are little children growing up without Daddy,
 grieving wives, heart broken parents.
 Brothers and sisters wondering if this will strike them also.

For everyone with a bicuspid aortic valve
 who will never have an aortic emergency,
 we rejoice. 

But we are called to speak
 for those vulnerable to tragedy.

His daughter was born 4 months after Doug's death
Doug's children in 2010

Their children should not grow up without them.

And their family members should not live in the shadow of fear
 that some day this could happen to them also.

We are
continuing to tell their stories,
raising awareness of the danger,
while living
in a climate of hope.

- the Bicuspid Aortic Foundation

Sunday, September 14, 2014

My Love Will Find You - Families with Bicuspid Aortic Valve

This is for everyone who loves someone with Bicuspid Aortic Valve or any form of aortic disease in the chest.

Derek Owens' parents were told about his BAV at birth
He was 16 when he first had surgery

This is especially for Mothers. Mothers of all ages.  Right from the start, they know how special, how talented their child is. At some point, they may learn there is something not quite right inside the heart of their beautiful child. They agonize through the doctor visits, the surgeries. Some Mothers hear those most dreadful words. Their beloved child is gone. Rest assured, there is nothing you could have done to prevent a bicuspid aortic valve. Above all, you give your child what no physician ever can. You give them your love. It is the most wonderful treatment in the world, and you can increase the dose at any time without harm. Yes, it is the greatest healer there is. Your love. Unlimited. Always.

Following are the words of a childrens' book by Nancy Tillman. The pictures are from BAV families.

Wherever You Are, My Love Will Find You

By Nancy Tillman

I wanted you more
 than you ever will know,
 so I sent love to follow
 wherever you go.

It's high as you wish it. It’s quick as an elf.
You'll never outgrow it... 

it stretches itself!

So climb any mountain...

BJ Sanders at Machu Pich
climb up to the sky!

My love will find you.

BJ's dear Mother, who lovingly
follows her daughter's adventures
My love can fly!
BJ celebrating her birthday in 2014. She had surgery a year earlier.

Make a big splash! Go out on a limb!

My love will find you. My love can swim!

Scott Nichols (in blue) was lost to aortic dissection, January 2014

It never gets lost, never fades, never ends...

if you're working...

or playing...

or sitting with friends.

You can dance 'til you're dizzy...
paint 'til you're blue...

There's no place, not one,
that my love can't find you.

Derek Owens has always loved basketball!

And if someday you're lonely,

or someday you're sad,

or strike out at baseball,

or think you've been bad...

just lift up your face, 

Derek in high school
feel the wind in your hair.

Derek Owens and his Mom, Laura
That's me, my sweet baby, my love is right there.

Chuck Doherty lost his life in April 2012
 due to BAV complications

In the green of the grass... in the smell of the sea...

in the clouds floating by... at the top of a tree...

in the sound crickets make at the end of the day...
“You are loved. You are loved. You are loved,” they all say.

Carrie Mettler running a half marathon
Carrie Mettler's beloved Mother, who knew the reality of
having two daughters with BAV
My love is so high, and so wide and
 so deep, it's always right there, even
 when you're asleep.

So hold your head high
 and don't be afraid
Bob Gies following his surgery in 2010.
to march to the front
 of your own parade.

Doug Grieshop on his wedding day 

Doug Grieshop at 2 months
If you're still my small babe
 or you're all grown,

my promise to you
 is you're never alone.

Doug had an undiagnosed BAV. He lost his life to aortic aneurysm
 rupture 10 years ago, on September 20, 2004.

You are my angel, my darling,
 my star... 

and my love will find you

wherever you are.

You are loved.

Saturday, September 6, 2014

Scott Nichols - Bicuspid Aortic Valve and Dissection

Scott Nichols with his son, Carson, on the golf course
Words do not come easily when trying to capture the essence of Scott Nichols. In the prime of his life at age 41, he was a husband to his wife Jennifer, father to his daughter and young sons, friend to many, a special education teacher, and a coach.

Scott coached a high school hockey team as well as being the goalie coach for the NAHL Port Huron Fighting Falcons. He had a lifelong love of sports and continued to play many himself - hockey three times a week, softball, and golf.

Yes, Scott was a very special man, known to so many in his community and state.

There was something else special about Scott. Something that no one, not even Scott himself, knew. He had been born with a bicuspid aortic valve.

A Snow Day in Michigan and Chest Pain
Even in Michigan, where those who live there are prepared for winter, some days the weather is just too severe, and schools are closed. These "snow days" are unexpected free time for students and teachers alike. On a snow day in late January, 2014, Scott Nichols made the most of this unexpected gift of time, packing it with activity. He played basketball with some of the other teachers, and then went on to coach two different hockey teams. Later that evening, he was out on the ice playing hockey himself when he felt the chest pain. 

No Prior Warning
There was no warning that this strong, active man had anything wrong until chest pain forced him off the ice and to the local emergency room. There his heart was checked, and a heart attack was ruled out. The next day his bicuspid aortic valve (BAV) was found. He continued to get worse. What was happening inside Scott? More testing found it. Like many with BAV, Scott's aorta above his BAV had been bulging dangerously (aneurysm). The weak, bulging tissue had torn (dissection). Scott was bleeding inside.

Life Flight to University of Michigan
Scott needed to reach a medical center equipped to perform emergency surgery on his aorta. He was flown to the University of Michigan in Ann Arbor. There, as his family waited, surgeons battled for 14 hours to save him, repairing the terrible injury inside him. But the damage was too great. It had been too long without blood flow to vital places inside him. On January 31st, Scott Nichols was declared brain dead.

Disbelief, Shock, Mourning
Scott (in blue) at the golf course with friends

Scott's family, students, fellow teachers, and friends all descended into shock and deep mourning. This article from the The Voice, February 3, 2014, Scott Nichols Impact Felt, describes the anguish and tremendous sense of loss. As mentioned in the article, with hearts broken the Port Huron Hockey team decided they would play their game the next day in his honor. In the words of Coach Pionk, " ... people like Scott Nichols don't come around very often."

September is Chest Aneurysm and Dissection Awareness Month
The Bicuspid Aortic Foundation observes Chest Aneurysm and Dissection Awareness Month in September.  This same month is observed for brain aneurysms, which also occur in some individuals with BAV. This year, we are deeply grateful to Jennifer Nichols for bravely sharing what happened to her husband just a few brief months ago. She does so in the hope that it may save the lives of others. BAV is estimated to exist in 1 of every 50 people, predominantly in males. 

How many other athletic men in their prime
 are at risk from a bulging, weak aorta?

We do not know. 
We do know that finding a BAV is a red flag 
  • to look further 
  • to find the hidden danger 
  • and to have surgery before a life-threatening emergency.

We hope that through the tragedy of what happened to Scott, the life of someone reading this will be saved. Maybe someone strong and healthy like Scott. Someone who feels great, but gets his heart checked, and finds out he was born with a bicuspid aortic valve. 

In raising awareness
of the risk and the danger,
we  seek to create
a lifesaving
 climate of hope.
From all who volunteer
with the Bicuspid Aortic Foundation.

Saturday, August 2, 2014

There Are No Perfect Numbers - Making Decisions

In mathematics, there are perfect numbers.
The first one happens to be 6.
 But in aortic disease, there are no perfect numbers.

Just 2 millimeters more
How big is 2 millimeters? It is the thickness of a US dollar coin. Looking at it, one wonders how such a small distance could make so much difference. For one man, it was the difference between having surgery or not. His aorta was 5.8 cm. The doctors told him surgery is done at 6.0 cm. The difference - just 2 mm - the thickness of a coin.

When "6" was the Number
For Louie Lopez, this tiny length became a distance too far, a point never reached. He was in the hospital, talking with the surgeon, when his aorta tore and fully ruptured. Massive bleeding, massive damage. Rushed to surgery, he had no chance. The year was 1995. Yes, "6" was "the number" for aortic aneurysm surgery back then.

Not long ago, Terry Lopez talked about that time, almost 20 years ago, when she lost her husband, Louie. "They were waiting for just 2 mm more. Just 2 mm....surely, 20 years later they can do better than that."

Reading papers, watching medical conference videos from thought leaders in thoracic aortic disease, I marvel at the paucity of answers, the preponderance of uncertainty still, as they continue to debate aneurysm size and the timing of surgery. There is still more unknown than known. Aortic disease remains a more than worthy opponent for those who do battle with it.

The Numbers
Physicians look for numbers to guide them in making decisions about aortic disease. For aneurysms, the most prominent number is the size (diameter) of the aneurysm. There is a great deal of debate about the number, the size when surgery should be done. Some argue to protect from what can be serious complications of surgery, urging that patients wait for the aorta to grow larger. Others promote earlier surgery, at smaller sizes, to protect from life-threatening, deadly events.

The following paper from 2013 is an example of the debate, giving pro and con perspectives from physicians around the globe, Germany, the US, and Australia:

"Aortic Surgery for Ascending Aortic Aneurysms Under 5.0 cm in Diameter in the Presence of Bicuspid Aortic Valve",  authored by Klaus Kallenbach, MD, PhD,, Thoralf M. Sundt, MD, and Thomas H. Marwick, MD, PhD, MPH. 

In this paper, it is noted that very few patients were studied (with differing underlying causes of aortic disease), in coming up with the number for surgery, although used to guide the care of millions.  "The number" has generally been coming down over the years as surgery became safer, but the uncertainty of what the number should be remains.

Some individuals suffer aortic tearing, perhaps death, at smaller sizes. On the other hand, surgery has risk, and offering surgery at smaller sizes may injure someone whose aorta would not have torn or ruptured if allowed to grow larger.

 The size of the aneurysm alone is just not enough. Other measures, other ways to understand, are needed, but not available today.

If there were "perfect numbers" for the aorta, our friend Richard would not have dissected at about the same age and the same aneurysm size as my husband (who did not dissect, but had preventive surgery at 5.2 cm). Richard barely survived and has paid a high price in additional surgery because of his original dissection 10 years ago.

For the narrowed aortic valve, aortic stenosis, there are also numbers: the size of the valve opening and the pressure build up. There are measures for the leaking aortic valve also. Are they perfect numbers, that each individual can trust with their life? Is there a promise that nothing bad will happen before you reach the "guideline" numbers? Nothing bad will happen until you have obvious symptoms?

If there were "perfect numbers" for aortic valves, Chuck Doherty would still be with his family. So would  Chad Rogers.

Guidelines, Not Guarantees 
Those with BAV and other forms of aortic disease need to understand there are no guarantees, no solid promises. There are guidelines based on information available today. To our surprise we may find them quite limited, once we understand what they are based upon. As a patient, you need to research for yourself, understanding as much as you can.

Making Decisions
Part of that research can be searching for and choosing physicians/surgeons with a philosophy and approach to risk that is in agreement with your own. There are things to think about, such as your lifestyle, frequency of foreign travel, and general comfort with the risk of surgery versus the risk of waiting. These decisions should be individualized within the context of guidelines and statistics; decisions made in conjunction with physicians whose philosophy and approach to life and risk are in harmony with your own. Physicians who clearly share their outcome statistics with you.

Handling Uncertainty, Owning Decisions
In 2013, Aortic Valve and Ascending Aorta Guidelines for Management and Quality Measures were published. Including references, there are 66 pages. In the summary on page 54 are these words,"The choice of the best procedure or valve is dependent on many factors as discussed above and no procedure or device is ideal. Ultimately it is up to the patient, the cardiologist, and surgeon to reach a decision on appropriate treatment." 

As these guidelines tell us, it is very important that you, the patient, are a partner in these decisions, and that you understand the basis on which you and your physicians together are making these decisions.

Wouldn't it be wonderful if there were perfect numbers, perfect devices, and simple decision making? Yes, but as in much in life, that is rarely the case. There are pros and cons, trade offs, in most decisions that must be made. But we only have one heart, one aorta, so these are very important decisions.

The more you know, the more you understand the pros and cons, you can make decisions that belong to you, that you can own. Not knowing leaves us vulnerable, caught by surprise. Perhaps many of us have already been there, rudely discovering the imperfections and limitations regarding aortic valve options.

Over 20 years ago, the mechanical valve that saved my husband's life was called a lifetime solution. The night I walked into our home and found my fallen husband, terribly injured by that valve and its complications, I began to understand the terrible depths of untruth in that "promise" that we had happily once believed. I will never forget him saying to me, "I did everything I was supposed to do."

If you understand there are no "perfect numbers", no "ideal" devices and solutions, it will at least help you to be proactive, to question, to research, to seek more opinions when things don't make sense, and to partner with your physicians in the decisions that are made.

And when something does not feel right, when an echo or other test results do not make sense, don't let it go. Keep asking questions, seeking answers.

No one cares more than you do.
 Because it is you, the patient,
 above all others,
 who will "own" the outcome.

May this help you to read,
 and make informed decisions,
while living in a climate of hope.

Best wishes to all,
Arlys Velebir
Bicuspid Aortic Foundation

Sunday, July 13, 2014

Ellie - A Life at Risk

Ellie (not her real name), this is for you, 
and every other person in the world
 with a heart murmur, chest pain, and aortic aneurysm,
 who is not receiving the attention and care they need. 

Ellie lives in a small city in the eastern United States. Ellie has health insurance. But Ellie is not receiving the care she needs. Her life depends on her own strength and determination, her willingness to push through ignorance and find the expertise she needs.

Ellie sought help because of chest pain. Her blood pressure was also high. She has known about a heart murmur for about 15 years. She also knows that her father died suddenly of a "burst heart". He was younger than she is now.

Heart murmur, chest pain, high blood pressure, early sudden death in the family - any alarm bells going off yet?

Ellie had both an echocardiogram and a CT scan. But she did not hear back from the cardiologist who ordered them for 6 weeks. Her calls to him were not returned. In desperation, she asked for an appointment with another doctor, this time the heart surgeon at her local medical center.

From the meeting with the surgeon, Ellie came away with at best confusing and at worst, potentially deadly information.

Ellie was told that she was not a candidate for surgery yet because her aortic aneurysm was not big enough. Ellie is a petite person, but even more importantly, she has chest pain and "heart-related" sudden death in her family.

The following paper was published in 2005.
Why wasn't Ellie told about this?

Perhaps it was when she pressed him, hoping to prevent a catastrophe like her father's, that she was told she "would not come off the table alive" if she had surgery now. Really? What would make the surgery safer later, if Ellie is still alive when it reaches a larger size? At that point, Ellie felt she was given a death sentence.

She was also told that if her blood pressure would come down, her aneurysm could completely go away. Assuming she has BAV, there is nothing in the medical literature that supports this. Where did the surgeon get this misinformation? There is a recent article about a class of blood pressure medication (ACE Inhibitors) given to those with BAV:
The article concludes "In an adolescent and young adult population with an isolated BAV, there is no proven benefit to ACE inhibitor use with regard to slowing rate of ascending aortic dilation."  There is a need to keep looking for medical answers, but it is important to know that at this time, nothing has been found to prevent or make an aneurysm go away in those with BAV. If that day comes, it will be shouted from the rooftops, and just in the US alone, sales to the millions with BAV will skyrocket.

Does Ellie have BAV? Well, no doctor has explained that to her clearly either. Everything about her suggests it. Why didn't someone explain what is going on with her aortic valve to her?

Ellie's local center does not do complex aortic surgery. Why wasn't she referred to a major center that does do it? Instead, Ellie was advised to go the local ER if she has chest pain again. She was given papers that describe how life-threatening aortic aneurysm in the chest can be. One wonders if that is the handiwork of lawyers, for the protection of the physicians and the hospital. Documentation that the patient was warned of the seriousness of this. It is difficult to see how words on paper can protect Ellie. If Ellie's aorta tears, could she make it to the local ER, have a CT to find the problem, survive a life flight to another center, and make it through emergency surgery? 

What Ellie needs is real help before something deadly happens. So far, she is not getting it. May she find the strength within herself to go to a major center on her own. She needs to find someone who understands her, cares, and has the skill to save her. Until then, may that weak, bulging tissue hang on. If Ellie's aneurysm should tear or burst, with every heart beat she will bleed uncontrollably inside.

Ellie, there are answers and treatment for you.
Please don't give up until you have them.

Ellie happened to cross paths and share her story with someone who volunteers with the Bicuspid Aortic Foundation. Otherwise, we would not know about her. It is an important reminder, lest we forget, that those like Ellie are vulnerable everywhere, despite high tech advances in the treatment of those with aortic disease.

Sunday, May 18, 2014

Escaping Dr. Hodad

Dr. Marty Makary, in his book Unaccountable, writes about Dr. Hodad in the very first chapter. Reading about this doctor, I realized that I had heard of him more than once, in more than one hospital. I just did not know his last name, given to him by surgical residents - HODAD - Hands of Death and Destruction. His name says it all. If you are introduced to him, sign no papers. Escape!Run!

Escaping Death

"I thought you would be dead now."
How could a physician, trusted to protect life and do no harm, look directly at her and say this to Rose? But he did. Who was he? He was not a surgeon. He was a medical doctor. But he was part of the team in a terrible surgical drama that played out in Rose's chest, and he did nothing, nothing at all, to help her.

Yes, Rose fell into the hands of Dr. Hodad and those who collaborated with him. But she did not die. From her story, we can learn why it is worth doing everything possible to avoid the hands of death and destruction in the first place.

Every day, somewhere in the world, someone is having a surgery like Rose needed. There are surgeons who have spent many years perfecting the most complex surgery in the chest, aortic surgery, along with replacing the aortic valve. Rose had been born with BAV, and she also had an ascending aortic aneurysm.

Referred from doctor to doctor in her local city, Rose trusted the doctors she met, trusted them with her life. She believed that she would be referred to the right surgeon, with the right skill. She entered the hospital and had surgery. It was far from ideal, but she came through the complications. With time, she should have been feeling better and better.

Something is Terribly Wrong

But Rose was getting worse. She did not need anyone to tell her that something must be terribly wrong. And it was. Eventually Rose learned that her aortic aneurysm surgery had been botched. Normally a Dacron graft is used to replace the bulging section of aorta that is called an aneurysm. But not in the hands of Dr. Hodad. He had replaced her BAV with a pig's valve, which included part of the pig's aorta above the valve. Dr. Hodad simply cut out Rose's aneurysm, and instead of using Dacron, pulled Rose's own remaining aorta down until it met the pig's above the valve. What went wrong? There was too much tension on her aortic tissue. Rose's stretched aortic tissue began to pull away from the stitches holding it to the pig valve, and blood began to leak out around her valve with every beat of her heart. 

I am amazed at the depths of courage and strength Rose found within her. She fought for her life by researching and reaching out to others, asking questions. She trusted people she did not know. They helped her find her way to an experienced aortic surgeon in another state. She put her life in his experienced hands. Rose's first surgery, in those experienced hands, would have been easy.  This surgery was a fight for life. It took all day. He saved her. 

Escaping Destruction
I remember being told, I think by the anesthesiologist, that the surgeon who would replace my husband's critically narrowed BAV was a good technician. That was over 20 years ago. I didn't appreciate it then as much as I do now. Knowing now how complex that surgery was, I know that the surgeon was at least good enough to get through the challenges - a man in heart failure with a critically narrowed, extremely calcified, and abnormally small BAV. I can imagine a very different outcome with Dr. Hodad. Although, as Dr. Makary writes, the best technician may not be charming like Dr. Hodad, it is important to get the very best skill available to you to save your life.

Years later, my husband had aneurysm surgery. I remember a nurse telling me that his surgeon had "golden hands" and would be her choice. I didn't fully understand the importance of what was said. This was not just being polite. This was someone who worked there and saw the outcomes, day after day. Dr. Makary writes about asking the "insiders" about the doctor you are considering. Those who work there will have more information than you do, if you can find a way to tap into their world.

Finding "Dr. Right"
Often the Bicuspid Aortic Foundation is asked about physicians who can help those with BAV. This is such a difficult dilemma for us all. We do not have enough information to help find Dr. Right in the many places where he is so needed. What we can do is encourage everyone to do everything they can to find Dr. Right and flee from Dr. Hodad.

For those with BAV, medication cannot solve their problems, and surgery and surgeons are extremely important. Thinking about Rose (not her real name), my own family, and the larger genetically related BAV family whose lives all depend on surgical skill, there are things we can do to help ourselves:

  • Referrals given may be to Dr. Hodad, not to Dr. Right; you can refer yourself to surgeons in order to find Dr. Right
  • Educate yourself about the procedure you need and ask the surgeons you consider many questions 
  • Think in terms of the best skill based on results, not the nicest personality 
  • Other patients may not know enough about what happened to them; ask the "insiders"
We are
Creating a 
Climate of Hope

Arlys Velebir
Bicuspid Aortic Foundation

Sunday, April 20, 2014

Lily's Story - Bicuspid Aortic Valve Stenosis

Broken Heart Lily
 (picture from David's Garden Diary Blog)
I have never met Lily in person.
I have never seen her picture. 
I know the sound of her voice.
And I know her heart.

I will never forget her.

Meeting Lily
Lily contacted the Bicuspid Aortic Foundation one day. She told me that she had severe stenosis (narrowing) of her bicuspid aortic valve (BAV), as well as an aortic aneurysm starting. The narrowing was because over time Lily's bicuspid aortic valve leaflets had become calcified and stiff. They do not open widely any more, and are getting worse.

Yes, Lily was born with a bicuspid aortic valve. But that was not all. Like others with BAV, she entered the world with "delicate tissue". Numerous health issues have surfaced over her lifetime, at least some genetically based. Yes, whatever is underlying it all, Lily has lived with the pronounced effects through out her body. And yet, Lily married and had a family. She has clearly been an active woman with many interests in her life.

Lily sent me a list of her medical conditions. There are over 30!

Yet, of all her many medical conditions, it is this tight, narrow aortic valve that now threatens her life.

Lily's doctors spoke to her of options, but there are not many. She is very high risk for surgery. Lily wanted very much to know about someone else like her, someone who could not have surgery. This is the longing to be understood, to not be so terribly alone, to know someone cares.

Could TAVI Help Lily? (transcatheter aortic valve insertion)
What about the new option of inserting a tissue valve inside an existing calcified aortic valve without opening the chest? It is meant for those, like Lily, who are too high risk for surgery. But in the United States it is not approved for those, also like Lily, who have a bicuspid aortic valve.

Recently, Lily wrote that she was going to investigate a clinical trial for TAVI in those with BAV. She was seen quickly, as her symptoms are getting worse.

Just two days ago, Lily wrote again, explaining that she does not qualify for this either because of the shape of her particular bicuspid aortic valve,

 ". . . I needed to have a tricuspid valve that two flaps had fused together instead of a true bicuspid that is missing one of the three flaps altogether making it a mouth shape instead of a 3 points shape. It needs the extra point to hold it in."  

In BAV, A Disease of the Elderly in the Young 
Aortic stenosis is a terrible scourge on society. One doctor wrote an editorial calling severe aortic stenosis a "neglected malignant disorder". It often is thought of as a condition of the elderly. However, BAV hearts are prone to it at younger ages. BAV is the most common reason for aortic stenosis in those under the age of 65. Usually, the narrowing of the valve is caused by gradual calcification. However, there are some infants and children with BAVs narrowed abnormally by tissue, right from birth.

In the United States alone, statistics showed there were 300,000 individuals in need of surgery for aortic stenosis, but in one year only 80,000 received it. (There is no medicine to cure this.) Without some kind of intervention, the rest will die. Once it reaches a critical point, half are dead within two years. (View the videos in this post from 2011 for more information.)

Broken Heart Lily
A double oriental lily
whose fragrance
 is exceptionally sweet
Lily's Courage
Lily's symptoms are getting worse. She is no longer allowed to drive. She ended her note like this,
"I feel I have exhausted all my options and at peace that I have done all I can do. I am enjoying every day with blessed hope and contentment.
Love n Prayers, Lily"

Lily is one of those thousands in the United States
for whom there is no answer. I have called her Lily, which is not her real name. She is a courageous, strong woman, too young and too beloved to be taken from her family.

In honor of Lily's courage and strength, let us continue to raise awareness and to press for ways to mend beautiful, broken hearts like hers.

Love to you and your family, dear Lily,
Arlys Velebir
Bicuspid Aortic Foundation

Sunday, March 23, 2014

Bicuspid Aortic Valve and Aortic Disease Answers Are Blowing in the Wind

Kyle Wilson
sudden death
BAV and aortic dissection
Chuck Doherty
sudden death
BAV & aortic stenosis

"Yes, how many deaths will it take 'til he knows

That too many people have died?

The answer my friend is blowin' in the wind,
The answer is
blowin' in the 
                                  - Bob Dylan, 1962

Doug Grieshop
sudden death
BAV and aortic rupture

Friday, we received that most dreaded message once again.

Because we know those with BAV very well,
reading the words,
we feel we know this person, born with BAV.

". . .  very athletic."
". . .  so healthy."

We hold our breath . . . .

"He is dead."

And then we cry.

How many, oh, how many, must die?

Sometimes we read of their tragedy, although we may never meet these families. 

Sometimes, a bicuspid aortic valve has been found,
 and the person was under medical care.

Sometimes the BAV had not been diagnosed.

In both scenarios, there is death.

Why, oh why?

Sahib Singh Vaseer
death from
BAV and endocarditis
We are left to ponder very serious questions.

Where are the answers?

They are as ethereal 
as wispy white clouds,
driven by the wind.

Other families, like mine, avoid death
 but cope with life-long injuries,
both physical and emotional.

That is why Robby Benson, 
a veteran of four open heart surgeries,
entitled his book

A recent paper from a consortium of centers studying BAV, with authors from around the world, is aptly titled, "Surgical treatment of bicuspid aortic valve disease: Knowledge gaps and research perspectives."

We simply cannot tolerate, cannot bear the tremendous ache with every injury, every death in BAV families. Yes, their trileaflet aortic valve family members also are at risk. These knowledge gaps are not small things.

Those pictured here speak to us of major complications that may strike down even the young and strong - the aortic valve itself, aortic dissection, aortic rupture, and infection (endocarditis).

To remind us all of the tremendous problem, and our shared responsibility to conquer this centuries-old monster, this blog has adopted "Blowin' in the Wind" as our theme song. 

Glimpses of small 
numbers of people, 
watched only
for a few short years,

Fragments of information
blowing in the wind,

Will not answer our questions,
will not satisfy our needs.

May we all resolve
to take action,
to free humanity at last
from this plague.

                                                                                                 - Arlys Velebir
                                                                                                   Bicuspid Aortic Foundation