Thoracic Aortic Disease Awareness September 2023 - A Time to Remember

"Try to remember when life was so tender

That no one wept except the willow"

"Deep in December our hearts should remember

And follow, follow, follow" 

How long is 22 years?

I do remember a time of innocence in terms of aortic disease in the chest. It is 22 years now since a large aortic aneurysm was discovered around and above the mechanical aortic valve in my husband's heart. It changed our world. It became clear after his successful surgery that others needed to be warned, to receive help. How long has time been since then? Very long. And very short. Time is like that, its length defined by what happens in the moments of our lives.

Remembering our roots at the Bicuspid Aortic Foundation, this year we mark the passage of time since becoming conscious of the tremendous implications of this disease and the need for awareness and advocacy. Those issues and needs, along with unanswered questions, persist today despite progress made.

At the Bicuspid Aortic Foundation it is a privilege to once again focus on the aorta with September Awareness 2023. It is indeed gratifying to see many global efforts to raise awareness, support individuals and families, and continue progress through research and advancements in surgery.

This September we remember...

• We remember those who died.
• We remember those who survive with injuries.
• We remember all those with thoracic aortic disease, too often undiagnosed.
• We remember the great pioneering medical knowledge and surgical progress that saves lives through out the world today.

At the Bicuspid Aortic Foundation we are
Remembering this September;
And along with our memories,
Creating a Climate of Hope,
~Arlys Velebir
                                          President, Bicuspid Aortic Foundation

September Awareness 2022 - Larry Abramson 1955 - 2021

Larry and his wife in 2008,
after aneurysm/valve surgery

In September 2019, Larry gave the following to the Bicuspid Aortic Foundation, to encourage others and raise awarness about the aorta in the chest. It is painful to realize that just two years later, on September 22, 2021, he did not survive a 4th open heart surgery, a fight forced by infection.

September Awareness 2019 - Larry Abramson 

I remember with sadness the last time I heard Larry's voice, no longer strong and clear, the tone roughened by his illness.

 Larry was very unwell by then, after days and weeks of ups and downs - testing, searching for answers, reaching dead ends - had become months.

At last, the villain was cultured in his blood, the infection’s damage to his heart became clear, and one more surgery, his 4th, was his only hope.

Larry did not survive that last surgery. The infection’s damage was too severe.

Devastating bacteria have evolved, with the ability to conceal their presence and aggressively attack the heart and its valves until extensive damage is done. Such an infection ended the life of this amazing man, born with BAV. 

It was in the "early days" of heart surgery that he had his first, at age 11. From his second surgery at age 20 onward, he lived with mechanical aortic valves, a span of 44 years. This account by his family speaks to the vibrant, active man he was, until those last weeks. 

One year later, 

the Bicuspid Aortic Foundation remembers Larry, 

who lived so vibrantly with "great heart",

 and was taken much too soon.

September Awareness 2022 - The Aorta is Not the Heart!

 

When the Bicuspid Aortic Foundation first chose an Awareness Month for the Thoracic Aorta, it was important to designate a time widely separated from February, "Heart Month". September is widely separated from February on the calendar.

Quite simply, the reason for this was to educate individuals and families about their aorta in the chest as a separate, unique, and vital entity in its own right.  

Especially in emergency situations, but also in office visit settings, when the focus has been on the heart primarily, there have been far too many "near misses" and tragic loss of lives. 

Too often, including the current era, some articles for the general public confuse the heart with aortic disease in the chest. There are inherent dangers in such confusion. The tests and treatment for thoracic aortic disease are very different from that for typical heart disease, which involves blockage of the arteries of the heart. 

It is encouraging in 2022 that there are global efforts to inform the public about aortic disease in the chest.

Once again, in September 2022,

 we applaud and join with the efforts of all

 who accurately educate and inform

 the public about the aorta in the chest,

Creating a Climate of Hope,

~ The Bicuspid Aortic Foundation 

September TAD Awareness - Life-long Aortic Care for Coarctation

The Right Emergency Care at the Right Time
An account published earlier this year describes what happened in 2018 along a stretch of Interstate 90, as one man returned home from vacation with his family. I share the article here:

Heart emergency - right care at right time

Twenty four years after his last aortic surgery, now age 44,  an aortic aneurysm was dangerously bulging in his chest. Who can say why, at that precise moment, his abnormal aortic tissue could no longer hold together and contain the blood pumped with each heart beat? 

In the article, Dr. Jason Knutson describes many things that went right, one after another, in saving his life. He mentions not knowing that the odds of survival were about 2% in a hospital, less out on a highway!  

Yes, he received the right emergency assessment and surgery at just the right time. Reading this article again now, for September Awareness 2021, my initial question remains: why didn't this man have ongoing aortic care and a scheduled, elective surgery to address his aortic aneurysm? 

In the article, Dr. Knutson mentions that after that first surgery, thinking everything was ok while growing up, an athletic heart screening revealed that another surgery was needed; the patch placed on his aorta at age 6 had not grown with him. He had surgery again at age 20. 

Father Prodomos' coarctation
"redo" surgery, patch
and rupture visible

Patching of coarctation was also the initial surgical relief that Father Prodromos Nikolaou had at age 2 in 1981, not repaired again until over thirty years later in 2015 as he writes here: The Hidden Danger: A Patched Coarctation . How long had it seriously endangered him before being addressed?  As pictured, surgery removed that section of aorta completely. It is important to warn others of such life-long risks!

Life-long Aortic Care - "the right care at the right time"

For those wishing more detail, this full paper from 2015 covers coarctation, including BAV and aortic aneurysms also.

Current management of coarctation of the aorta 

Regarding surgical solutions, the paper states that the patch solution is "avoided whenever possible due to the frequent occurrence of aortic aneurysm and rupture".

Prior to their conclusion, the authors write of this as a "lifelong disease" and the importance of "life-long surveillance", whether the coarctation has been repaired or not. 

The knowledge exists.  Are people receiving the care?

Life-Long Aortic Care and Coarctation

In the early years of  BAF, we came to know a talented young athlete who was diagnosed with BAV and coarctation. He had surgery while still in his teens. His family became well informed and understood the importance of ongoing vigilance.

We were also contacted by a very worried Mom. She had taken her 10 year old daughter to the doctor because of headaches. I will call this little girl Sally. The bottom line, following testing: Sally's aorta was narrowed, just past the arch (coarctation), restricting blood flow and building up pressure in the vessels feeding her head.

After I spoke with her Mom, she put Sally on the phone. I still hear her sweet voice across the years. I spoke to her once more, after she was out of the hospital. She told me in a very serious tone that she had been in the hospital "a long time" - not so long really, but it is always too long, even for adults - how much more at age 10!

Her Mom shared Sally's picture with us at BAF. A lovely young girl then, Sally would be in her 20's now. I hope she is living the active, vibrant life that awaited her then. I would be so happy to hear all about her life now. Most of all, I want to know: is Sally receiving Life-Long Aortic Care? 

For those with coarctation,

Life-Long Aortic Care

is

 "the right care at the right time"

~Arlys Velebir, Bicuspid Aortic Foundation

September Awareness 2021 - Why "TAD" Awareness

2001  and "TAA" (Thoracic Aortic Aneurysm)

My personal involvement with aortic aneurysm in the chest began 20 years ago, in 2001. There was so little public information then. My late husband's physicians, although carefully monitoring his anticoagulation levels, had not  followed his aorta in the years following his BAV replacement. Believing a mechanical valve equaled "fixed for life", we were comfortable with only a very few echocardiograms in the following years. We were spared an aortic catastrophe when one of those echocardiograms, to check on the 11-year-old mechanical aortic valve, "discovered" the bulging aneurysm above it. 

2005 and Thoracic Aortic Aneurysm and Dissection (TAAD) Awareness

We read medical references in our search for information and help in 2001, where we found an unfamiliar term, "aortic dissection".  By 2005, when the Bicuspid Aortic Foundation first observed awareness for the aorta in the chest, we had learned about aortic dissection, the "D" in TAAD.

We learned the medical definitions, yes, but much more, we learned the meaning in human terms, for individuals and families. We do not forget those we met in those beginning days.

It is the human experience that ultimately defines these medical words:

 those who die, 
those who survive with injury,
 those who have elective surgery with desired outcomes.

2021 and Thoracic Aortic Disease (TAD) Awareness

With the passing of the years, at BAF we met others with additional aortic complexities such as coarctation. Again, we had learned the terms first, but their meaning comes from meeting those who have them - from children to adults. Consequently, we have adopted an umbrella term - "Thoracic Aortic Disease" to include them as well. 

One Man's Life-Long Aortic Journey Until Now 

Father Prodromos Nikolau's life experience with TAD began at age 2 in 1981, and thus far has included the following:

• coarctation repair (1981)
• BAV replacement (2000)
• "Thinking Everything is Fixed" (after BAV replacement, annual cardiology visits)
• discovery of Aortic Root Aneurysm (2007)
• root and ascending aortic aneurysm repair and replacement of prosthetic aortic valve due to strands (2013)
• "redo"of original coarctation repair (2015)
• ongoing checkups, life-long aortic care

 At the following two links are the details, in Father Prodromos' own words:

Journey Beginning  from Age 2 

Hidden Danger of Patched Coarctation

"So, life is precious"

 as Father Prodromos wrote,

and life can be extended 

for those with TAD

when they receive

 Life-Long Aortic Care.

~Arlys Velebir, Bicuspid Aortic Foundation

I Need to Find Somebody Who Cares

Every Month is Heart Month, Every Day is Heart Valve Day

February 2021 is just a memory now, the calendar has turned to March. Heart Month 2021 and Heart Valve Day 2021 are over.

The need for accurate information, greater awareness, and compassionate care remains just as intensely, for it is always there!

The increased focus, still so fresh from recent February activities, can be the launch pad for maintaining focus and resolve to better meet the needs of those with BAV and associated TAD. Above all, at BAF we are reminded of the need to care, every month, every day.

"I Need to Find Somebody That Cares"

In 2020, the Bicuspid Aortic Foundation heard from a man, (I will call him Mathew, not his real name) who had been told a few months earlier that he had a bicuspid aortic valve. He was also told that he had an aortic aneurysm whose diameter was just under 5 cm. He mentioned several questions he had, as he pondered his active life and what options for surgical repair one day might mean to his future.

He closed that first message by saying that he felt quite lost as he worked through what these discoveries about his heart and aorta meant. And then he wrote, "I need to find somebody that cares."

A Day in April 2020 - Lost

It wasn't Heart Valve Day, it wasn't Heart Month, and it wasn't Thoracic Aortic Disease awareness month (which BAF observes in September). The COVID-19 pandemic was very real, but that is not what weighed so heavily on him, moving him to contact the Bicuspid Aortic Foundation.

It was a day in April, 2020, when this man, needing information, compassion, understanding, and hope, reached out across many miles and borders. There are no borders when it comes to this!

Above all, Mathew needed "somebody that cares". 

How do I know so well what Mathew needed? Experience. The myriad of emotions from the time when we desperately searched for information and help for a bulging aneurysm above my husband's heart are not forgotten as I write this twenty years later.

Not the least of those feelings were the icy tentacles of fear, not inappropriate when confronting a reputed killer. That icy fear began to melt in a surgeon's office, in the presence of accurate information, a plan for treatment, and from that moment forward, lifelong, compassionate care.

Yes, we had a great deal in common with Mathew, having never met him, on that April day in 2020.

A Day in March 2021 - Strength

Not quite a year later, in March 2021, what is it like for Mathew, who was lost and needed someone to care?

He was moved to reach out again, from a very different place. There is no mention of feeling lost, but rather a tone of strength. "I am doing well", followed by beautiful words of encouragement, which we all need so much! 

When this happens, whenever we move from fear and confusion to the place of informed empowerment, we have strength to fully live, making informed decisions for ourselves and sharing our strength and courage together.

How do we at BAF know this? Experience.

Mathew has done this, these recent months. 

Many others have done it too.

At BAF, we are here to walk with you, whenever you would like.

Moving from fear and confusion 

to accurate information,

being in touch with those who

 understand and care, 

Living our lives,

Creating a Climate of Hope,

~ Arlys Velebir

                        Bicuspid Aortic Foundation

Doing Good in the World of BAV and Thoracic Aortic Disease

BAV and aorta research in Ottawa involves mice like this one

My thoughts return often to the research in progress at the University of Ottawa.

 It is hard to find words that will convey what the efforts there mean to those of us with BAV and thoracic aortic disease.

I will try. 

One of the memories I have from my visit to Ottawa is holding the upper half of a human heart in my gloved hands. The lower half had been cut away. As I looked inside at the upper chambers and valves, to my surprise I saw this was a heart whose aortic valve had been replaced. The biological aortic valve that had been so carefully stitched in place by a surgeon's hands was still firmly in place.

I cannot describe the emotions that flowed through me then, and once again now, thinking of an unknown someone whose heart somehow came to be donated to medicine, to be studied after their life ended. I think of Dr. Abbott's work with preserved hearts in what is today the Maude Abbott Medical Museum in Montreal. There are hearts with BAV there. I hope to see them one day.

The cost of progress that others might live longer and more fully can indeed be very high. The cost of someone else's life. An even greater cost, a tragedy, occurs when lives are lost without learning from them how to help others. It means others will continue to suffer and die.

Everything about these mice, including diet, is meticulously tracked

Mice Hearts and BAV - Families Just Like Ours

We need not learn from our human families alone. At the University of Ottawa there are two different mice groups with specific genetic deficiencies that produce BAV. Like human families, some have BAV, some do not. Each one has their only individual experience, although having the same genetics. They are teaching researchers there about BAV, and they are very good teachers, accurately representing the variability of BAV and aortic aneurysm.  It was a great thrill to visit them and listen to what researchers are learning from them. This research is performed in the Nemer Lab, which is contained within the Department of Biochemistry, Microbiology, and Immunology. 

MRI for research in Ottawa
(Note the small, mouse-size opening!)

Echocardiogram machine
just right for mouse hearts!

Research is Costly

Dr. Sharo Raissi (BAF) with Dr. Daniel Figeys

Dr. Daniel Figeys heads the Department of Biochemistry, Microbiology, and Immunology within the School of Medicine at the University of Ottawa. This department includes the Nemer Lab and BAV research. Dr. Figeys very kindly gave us a tour of the research facilities.  It was impressive to see so much equipment in this facility that is solely aimed at understanding disease and alleviating human suffering.

 

 Doing Good in Our World

Dr. Sharo Raissi (BAF), Arlys Velebir (BAF), Dr. Daniel Figeys

At the Bicuspid Aortic Foundation, thanks to donations from the public, we have been able to fund scholarships to support BAV research in the Nemer Lab in Ottawa. They are adding important understanding to the knowlege Dr. Abbott established so long ago.
Some of us with BAV in our families will be called upon to teach the doctors who care for us more about BAV and TAD than they currently know. Those who have wise, skilled and compassionate physicians to walk beside them will indeed add to medical knowledge as their experiences unfold.

However, anyone so moved can make a contribution to help. In these last days and weeks of 2018, our thoughts may turn  to many things, including where we might give financially that will truly do good in our world.

This is to tell you that you can do good in the world of those with BAV and thoracic aortic disease through a donation to BAF, as we collaborate with those working to understand BAV and thoracic aortic disease. You may donate online at MightyCause 

Thank you for joining with us and

Creating a Climate of Hope,

~ Arlys Velebir, Chairman

        Bicuspid Aortic Foundation 

Unlocking the Mysteries - BAV and TAD September Awareness 2018

Still struggling with mysteries of the heart
  202 years since its invention,
 the stethoscope remains an important device
 for probing mysteries of the heart
   

"The practice of medicine is the struggle to unlock the mysteries within the body, to understand them in the hope of applying treatments to save life...."

This is my very own definition for the practice of medicine, reflective of the many years I have lived with struggling to understand and thwart BAV and TAD. I said this to a beloved cardiologist last year. He has been practicing for a very long time, and he did not disagree with me.

No one will ever convince me it is not a struggle. On the surface, what is known today was known 100 years ago. Do we have treatments now? Yes, but there is such a struggle to know what is happening, when and how to apply treatment, and will the treatment work . . . .?!

Too many times, there is no clear understanding. 

Too many times, there is suffering, injury, death. 

I was with one of our BAF board members recently for a consult with a new doctor. He remarked with astonishment that her medical records are a saga that rivals the novel War and Peace! So many different specialties, all searching for keys to understand her. We can only hope this particular specialist will be successful with his chapter of her story!

As September begins, I am looking forward to traveling to the University of Ottawa later this month, to visit the  Molecular Genetics and Cardiac Regeneration Laboratory . Dedicated to studying congenital heart defects, about half of their work is dedicated to unlocking the mysteries surrounding bicuspid aortic valves. They are co-located with the University's medical school and the Ottawa hospital. I am so grateful to be welcomed by them in our shared quest, our search for answers.

I invite you to share my visit to the University of Ottawa. Through this blog, I will have pictures, information, and stories to share. One thing I already know, I will be with those intensely focused on unlocking mysteries about the hearts and entire bodies of those born with BAV, and their trileaflet aortic valve family members too. I will be with those who understand the quest, the struggle, and are up for the challenge of this fight for life.

This September, 

there is no quest more worthy,

 no struggle more worthwhile,

 than seeking to unlock the mysteries

of BAV and TAD in our families,

Creating a Climate of Hope,

~ Arlys Velebir

Bicuspid Aortic Foundation