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 - Breaking the Pattern of Sudden Death

This September 2022, we reflect on the 20 year experience of one man. Despite the odds against him, he is not only still alive but fully enjoying life in his early 70's! While there is special focus on the aorta in September, his experience is a reminder that there can be multiple dangers, both heart and aorta, to someone's health and life. 

Family History - 14 Men, Sudden Death Before Age 65

Willie, not his real name, has a formidable history of sudden death in his father's family. Fourteen men "dropped dead" before the age of 65. They never reached the hospital alive.

What was it about these men that doomed them? What took them suddenly? Was it a massive heart attack? Could it have been something very different, a torn or ruptured aorta in the chest? What about sudden death from undiagnosed aortic valve disease? Willie has managed to proactively prevent harm from all of those things: aortic valve, aortic aneurysm, coronary artery blockage.

It could have been very different, as it was for many in his family.

Willie was born with BAV, but like many he had no awareness of it. Discovered at around age 50, his BAV was not working well and his aorta was bulging above his heart and needed surgery. Fast forward 20 years after successful aortic valve and aneurysm surgery, and his biological aortic valve was in major trouble.  He also had developed CAD (coronary artery disease)! 

All of these reached a point where he needed treatment, but all of them were also "silent" in his body. No symptoms. When asked how he feels now, after the most recent treatments, Willie says he feels well but adds that he has never felt unwell. No chest pain, no shortness of breath, nothing....

Today, he is a triumphant example of living well despite these silent threats, due to:

• proactive diagnosis 
• seeking information and second opinions 
• timely treatment
• ongoing care 

Breaking the Pattern of Sudden Death

Willie has overcome that horrendous history, and is grateful to be alive and well now, as he enters his early 70's! Even without any physical warning signs,  he was treated in time to prevent injury or sudden death from:

• bicuspid aortic valvular disease
• ascending aortic aneurysm
• prosthetic aortic valve failure
• coronary artery blockage

It is very special to share in his joy in being alive and well.

During September Awareness 2022,

 we share his proactive triumph

over BAV/TAD

and CAD,

Creating a Climate of Hope, 

                    ~ The Bicuspid Aortic Foundation

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

TAD Awareness September 2020 - More Awareness, Many Challenges Today

On this last day of September 2020, where are we in terms of awareness of aortic disease in the chest, both for the public and for medical professionals? 

Are We Still Learning "the Hard Way"?

Once the aorta threatens or takes away someone's life there is painful "awareness". It is a "hard way" to learn about a potentially deadly condition. For physicians, missed or delayed diagnoses and possibly loss of a patient are also learning "the hard way"; that is, if the diseased aorta is actually ever discovered at all. It is possible, in the absence of autopsies, that deaths due to aortic disease in the chest remain hidden, uncounted.

Would it Be Different Today?

At the time Michael Kirk went to the ER with symptoms, he was a cardiac intensive care nurse at that very hospital.  No one thought of his aorta, although over an agonizing 68 hours, they thought of many other things, including anxiety! The medical team ultimately learned from Michael, and he survived the crisis.  Michael shared his experience with BAF here. Thinking of Michael's experience, would it be better in 2020? Would his family members, assumed to have died suddenly of "heart disease" be checked for aortic disease today?

In 2020, Someone Still Needs to Think of the Aorta in the ER

There is a marker in the blood, troponin, that indicates heart muscle injury (typically signs of a heart attack) that appears to be broadly understood and used. There is no simple equivalent for the aorta today. Currently, someone needs to think of the aorta, to rule it in or out with imaging, as one of the major killers in the chest.

 However, a paper published in July 2020 offers hope for additional diagnostic help in the future. Imaging and Biomarkers in Acute Aortic Syndromes: Diagnostic and Prognostic Implications :  "It is expected, in the near future, the development of serologic and imaging biomarkers able to early detect clinically-silent pathologic changes in the aorta wall before (primary prevention) and after (secondary prevention) the acute index event."

This is hopeful news! However, checking for these biomarkers would still require that someone thinks of the aorta, not just the heart!

This court case is an example of death after being sent home from the ER, when the aorta was not considered. 

Richard Houchin, whose aorta dissected in 2004,  remembers hearing someone say "aorta" while in the ER, and thinking fuzzily, what is my aorta? Richard had a number of things "go right" that day, beginning with the paramedics who thought of his aorta, despite instructions from a major medical center to treat him as a heart attack victim.

When it is not a heart attack, we can help physicians think of the aorta in the chest by bringing it up to them.

In 2020, Someone Needs to Care for Aortic Dissections Survivors

An article published this September has this poignant title: 

Am I going to die now? Experiences of hospitalisation and subsequent life after being diagnosed with aortic dissection

The full article is not freely available to the public. However, as the abstract indicates, this is an effort to understand and improve the experience of those who survive aortic dissection. 

In 2020, Someone Needs Answers and Support When Thoracic Aortic Disease Causes Sudden Death

There are no words to describe the devastation left behind among the living who suddenly lose a loved one. 

How many times is aortic disease in the chest the actual culprit, not a "heart attack"? 

Amy's family had the courage and resources to arrange for a private autopsy, which unmasked the undiagnosed BAV and diseased aorta that abruptly took her life. Here is Amy's story.

In 2020, Focus is Still Needed on the Aorta, Not Just the Heart

For those with BAV, they may only be told about their aortic valve and heart. I recently spoke to the mother of a wonderful, active boy, age 14. While hospitalized at a major medical center for another reason, the presence of a bicuspid aortic valve was discovered. The initial information given was not overly concerning. When following up with a local cardiologist later, however, this boy and his parents were shocked to be told that his aorta was enlarged, and his physical activity should be limited. 

How could that be possible? His aorta had not even been mentioned, and all of a sudden, it is an issue important enough to restrict his sports participation? This is devastating to anyone, let alone an active, strong teenager.

It was not easy in my own family when a large ascending aortic aneurysm was "discovered", years after being told all was safely fixed following BAV replacement surgery. Later we learned the aorta was already enlarged at the time of the BAV replacement and had quietly continued to grow! 

No, it is not easy to work through the emotions and lifestyle changes that may follow abruptly learning about an enlarged/aneurysmal aorta. Acceptance of physical restrictions and lifechanging adjustments are not trivial for those whose bodies are seemingly so healthy, often naturally gifted athletes who are typically involved in numerous activities and sports. 

Parents, families, and each one with BAV deserve compassionately shared accurate information about their aorta along with their BAV/heart, ultimately enabling them to live with confidence, supported by their medical care team.

The following paper published in 2020 provides international information from multiple medical centers, regarding 2,122 BAV children; half of them had an enlarged aorta. Their average age was 10.2 years.

Predictors of Bicuspid Aortic Valve-Associated Aortopathy in Childhood: A Report From the MIBAVA Consortium

Keeping Our Focus on the Aorta Every Day, Every Month, All Year Long

At BAF we have been observing September TAD Awareness for a number of years. We find in 2020 that the aorta in the chest still presents many challenges. The various efforts to raise awareness this year all help the public understand the importance of their aorta, this large candy-cane shaped artery that begins with their heart. May we maintain this focus every day, every month, all year long. 

 On this last day of September,

 we can help everyone we meet

to remember the aorta and

seek accurate information,

empowering them to live confidently,

 Creating a Climate of Hope,

~Arlys Velebir

                         Bicuspid Aortic Foundation

Thoracic Aortic Disease Awareness September 2020 - TAD in the Midst of COVID-19

 How many have been injured or died from a diseased aorta in the chest (thoracic aortic disease (TAD)), not receiving treatment for various reasons during the COVID-19 pandemic?

Did the pandemic come between those with a torn aorta in the chest and the emergency physicians and skilled surgeons who could save them? It is reasonable to believe it did. The following is written by physicians in New York:

The COVID-19 Pandemic and Acute Aortic Dissections in New York

A Matter of Public Health

In the above, a "significant and precipitous drop" was identified in the number of surgeries for acute aortic dissection. The best hope for someone with acute type A dissection, which involves the ascending aorta and strikes without warning, is coming to the ER, being diagnosed, and receiving emergency surgery. How many were denied that opportunity due to the pandemic?

As the above concludes:

"It is critical, as we adjust to the pandemic, to balance the public health imperative of social distancing with the individual need to consult in the presence of sudden severe symptoms. Furthermore, additional resources, ranging from telemedicine to numbers of first responders, should be greatly increased. This serves as a word of caution for cities yet to experience a surge in COVID-19 cases, as well as for future similar events."

Inspiration and Also A Warning

The following is an account of a professor from Idaho who traveled to New York for life-saving complex aortic surgery during the pandemic. It is hopeful and inspiring, while at the same time containing a warning about the vital importance of lifelong monitoring of the aorta in those born with bicuspid aortic valves.

BACK IN BUSINESS: CEED Director undergoes life-saving heart surgery in New York amid pandemic

One of the surgeons who saved Jeff Street, pictured with him in the article, is Dr. El-Hamamsy, who is also among the authors in the first article above. He is among the skilled surgeons that could have helped those living nearby also, if only they had reached him.

Regarding the warning in this man's experience, it is important to avoid unnecessary, life-threatening aortic dissection or rupture in the first place. How did this man's aorta reach this very fragile state undetected? 

Jeff Street had surgery to address his bicuspid aortic valve 23 years earlier. Presumably he had cardiac care over those years, but what about his aorta?

Only when he became symptomatic this year, the article states, was an aneurysm "discovered". More concerning still, was the actual condition of his aorta when fully exposed during surgery on June 9th (quoting from the article):

" “Absolutely it was a life-saving surgery,” Street said. “I did not know that my aorta already had a small leak on it. The only thing holding it together was the scar tissue from my previous surgery 23 years ago.”"

Words like "miracle" come to mind as one reads about this man's experience - fragile tissue somehow holding together until he was in the hands of a surgeon with the skills to save his life.

And then, thoughts turn to those whose lives have ended abruptly, unknown and uncounted.

For those in BAV families, aortic valves are unquestionably important and vital. It would be beyond a tragedy to provide state-of-the-art treatment and care for their aortic valves, but not their aortas. 

This September 2020, once again it is important to raise awareness of the aorta in the chest in those born with bicuspid aortic valves and their family members.

In the midst of  this pandemic,

We again highlight the aorta in the chest, 

Raising awareness

and  

 Creating a Climate of Hope,

  ~Arlys Velebir,

                           Bicuspid Aortic Foundation

 

 

 

TAD Awareness 2019 - A Virtual Walk about BAF's Beginnings

September is Thoracic Aortic Disease (TAD) Month. What is it about?  Do we need a month just for TAD? Maybe we can explore this together, on our virtual walk today. This beach is a great place to walk - ocean breezes, blue skies, and the sand beneath our feet! Let me start by telling you my own experience with aneurysms and how the Bicuspid Aortic Foundation was born.

Aneurysm - A Medical Term

 There are technical words -  medical terms - used to accurately describe, communicate, and treat conditions and diseases. Medical textbooks and papers are appropriately written with such words. Thoracic aortic disease has these words, and I try to be very careful to use them properly. The visible indication of disease of the aorta in the chest is the medical term aneurysm, taken from the Greek, meaning widening out or dilatation. Some day maybe there will be other markers to find disease in the aorta even earlier!

My Definition of Aneurysm
For me, the most important meaning is the human experience. And so, on this walk, it is not the technical meaning I want to explore, but the meaning as defined by the lives who have been touched by it. Let me tell you about some of them.

Aneurysm and My Friend
Aneurysm, not in the chest, but in the brain was my first actual experience, my first real understanding, of this word. It entered

Decades have come and gone since
Saying goodbye at my friend's graveside here
She remains in my memories always,
Where she is forever 33

my life on a Sunday afternoon, with a phone call. The message was unbelievable - my friend was hospitalized with bleeding in her brain. Over the next few hours, there was more bleeding, nothing could be done, and gradually her still young body began to fail. She was gone. My memories of something so shocking have not faded. I still hear a little boy, scarcely more than a baby, crying for his mother. I still feel the grass beneath our feet as we walked to the graveside for that final goodbye. My definition of aneurysm stems from this experience - a crying child, a devastated husband - broken hearts forced to go on without someone so loved, so needed. Nothing could be more cruel than such a sudden, deadly killer.

Aneurysm Where?
I continued to be aware of brain aneurysms because of the loss of my friend. Did you know that aneurysms could be in the chest? I didn't. I thought they were in the brain.

Shocked and Terrified - an Aneurysm Above His Heart
When they stumbled upon the aneurysm above my husband's heart, I was shocked. I was terrified. I didn't even know it was possible! Deep inside was that real life definition of aneurysm I had learned years before. I thought this was going to kill him!

How could this be? He was supposedly fixed for life by his valve replacement surgery. I thought somehow they injured him when his BAV was replaced. After all, no one was saying anything to BAVers about aneurysms back then (1990). (They should have, as there were medical papers that associate BAV and aortic aneurysm in the chest - my favorite is Dr. Abbott's paper published in 1928!)

I kept my worst fears inside as we searched for answers. It was difficult, but we found our way to help.

Killer Disarmed by Successful Surgery
Can you imagine the feeling of relief when you are told that a ruthless killer has been disarmed? It was a wonderful, physical sensation that flowed though me, when the nurse told me that the aneurysm was removed, that the surgery had gone very well! Every step to reach that point, to find someone to help us, was so worth it! The aneurysm was gone. I would not find myself prematurely weeping at a graveside because of an aneurysm!

I knew how hard it had been to find that help. My only thought was to make it easier for others to learn about BAV and aneurysms - because when found in time, this killer could be disarmed before causing terrible bleeding inside the chest (dissection, rupture). I had seen it done!

BAF is Born
In time, out of the conviction that accurate information and knowledge can empower us to find help, the Bicuspid Aortic Foundation came into being.

After officially becoming a nonprofit, the first donation the Bicuspid Aortic Foundation received was a check in memory of Doug Grieshop. Doug's widow was the first family to contact us about untimely death from an aneurysm.  Doug had just turned 33 . He left behind his wife, young son and unborn daughter, other family members, and friends. Aneurysms, at their worst, take one life without warning and cause indescribable pain and suffering to those left behind. It is 15 years this September since they lost Doug, in 2004.

BAV and Brain Aneurysm
I know this is supposed to about aneurysms in the chest, this month of  September. Somehow, as we walk together, I am thinking of my friend and fellow board member at BAF, Carrie Mettler. Clicking here,you will find her story about BAV and brain aneurysm.   The discovery and successful treatment of her brain aneurysm is proof that those with aneurysms of the brain need not be terribly injured and lost either, when it is found in time!

Why TAD Awareness?
Doug Grieshop's family was the first to contact us about an aneurysm causing sudden death, but sadly, many others have followed.

We do not want anyone to experience an aortic emergency, 

we do not want to lose anyone else,

which is why there needs to be more awareness. 

 It is still a problem for those at risk to receive proper imaging of their aorta! Less than a year ago, I urged a family friend to request a CT scan of his chest (an echo had found a BAV). The cardiologist refused, saying it was unnecessary! Can you imagine saying it is not necessary to unmask and disarm a killer?! When physicians may not know or understand, it is important to keep seeking help through other opinions. Our friend eventually received the expert screening he needed, elsewhere!

This has turned into a long walk, so let's enjoy the sunset before we leave! 

Thank you for walking with me, 

Remembering those we have lost,

Others who were saved ,

As together, we create a Climate of Hope,

~ Arlys Velebir

                          Bicuspid Aortic Foundation

TAD Awareness 2019 Virtual Walk with BJ - TAVR to the Rescue

BJ Sanders shares her life story
on this virtual walk,
raising awareness of valve-in-valve TAVR

As you walk along with BJ, she begins with her life at age 12. Before your walk is over, she will tell you that today she is counted among those with a valve-in-valve TAVR and a grateful, happy heart!

1966 - A hint of what is to come

At age twelve my connective tissue disorder was emerging in the form of a severe scoliosis requiring six years of orthotics and finally culminating in a surgical procedure, Harrington Rod spinal fusion to straighten my spine at age twenty three. Despite the scoliosis I led a very active and healthy lifestyle!

1976 - Emerging labile hypertension

This is sometimes associated with Bicuspid Aortic Valve (BAV).

1981-1986 - Premature deliveries of my children

Once again connective tissue issues appeared in my three pregnancies. As a result of undiagnosed and misunderstood heart issues, I was placed on long periods of mandatory bed rest. Despite the bed rest, I had three premature deliveries.

1998 – No worries!

Despite multiple connective tissue concerns I had a very full and active life free of worry.

However, at this stage in my life I developed a prolapsed uterus, urinary incontinence and a rectocele all requiring repairs.

2000 – Searching for answers

Arrhythmias and visual concerns led to two echocardiograms, which the internist said were of no concern! Due to financial/insurance concerns, I requested my own copy of my medical records and noted that my aorta appeared to have expanded between echoes. I no longer trusted my internist!

Thus I began my long arduous Bicuspid Aortic Valve (BAV) and Thoracic AscendingAortic Aneurysm (TAAA) journey.

Over a span of two plus years, I searched earnestly for accurate knowledge to lead me to a physician with medical expertise in BAV and TAAA. There were many obstacles along the way, but the stars aligned the day I met Arlys Velebir in cyberspace. This eventually led to my introduction to Dr. Sharo Raissi, MD and the subsequent formation of the Bicuspid Aortic Foundation.

2002 – Watching and waiting

I entered into the watch and wait period under Dr. Raissi’s expert care. My quest for answers and understanding of my ascending aortic aneurysm and BAV were always met and my fears melted away knowing that I had found the expertise and compassionate care for which I had longed. HOPE had overcome FEAR!

 2013 – Time for Surgery!

Thirteen years into the BAV journey brought unexpected chest pain associated with a significant size increase in my ascending aorta. Dr. Sharo Raissi performed a TAAA reconstruction, a bovine artificial aortic valve replacement due to calcification of my BAV, and closed the left atrial appendage via a clip. Closing the left atrial appendage eliminates it as a potential source of embolic stroke in the future.

When I awoke from the surgery I distinctly remember Dr. Raissi saying, “In 10-15 years when this valve needs replacing, Transcatheter Aortic Valve Replacement (TAVR) should be available in the United States!” He also explained that he put in the largest artificial aortic valve possible for me; so, I could have a future TAVR!  At that moment I was not interested in 10-15 years from then. I had truly been given a new lease on life! After all, I no longer had an ascending aortic aneurysm and I had a brand new healthy aortic valve! I felt indestructible! I believed with all of my being that my new valve would last at least 15 years +! A TAVR seemed like light years away! I didn’t even bother to ask what the acronym stood for!

What I didn’t understand was that data is beginning to reveal that a new tissue surgical aortic valve replacement has a 20% chance of failure within the first 5 years!

In the Valve-in-Valve International Data registry, 20% of patients had their surgical aortic valves replaced within the 5 years prior to the TAVR. It is a bell-shaped curve.  Some patients with transcatheter aortic valves degenerate earlier, and that is true also for those with the surgical valve -  Dr. Raj Makkar Medscape interview March 25, 2019.

2016 - The honeymoon is over so soon???

The fall of 2016 brought the beginning of the end to my honeymoon with my “new” aortic valve. My scan showed the first signs of calcification!

2017 – Just “lazy”!!

2017 revealed further immobility of the “new” valve.

I discounted my increasing fatigue, believing I was just “lazy”.

I was unable to clean my house and became short of breath when making my bed. I avoided steps whenever possible. I developed a rectal prolapse with complications requiring surgery and a subsequent surgical procedure to repair another rectocele.

 2018 – Denial can be dangerous!

2018 brought frequent episodes of angina requiring nitroglycerin, increasing SOB with simple activities, debilitating fatigue. I was in complete “DENIAL” still believing my “new” valve could not possibly be the problem. Dr. Raissi spoke to me by phone and said, “BJ, I am worried about you”. I said, “Don’t worry”. He quickly responded,” I do worry”! I finally heard his concern and boarded a plane.

Happiness after TAVR with my brave, loving husband and son!

Flying from the East Coast to LA, I began to have difficulty breathing. Once again I was in complete “DENIAL”! Once my son and I arrived in LA we walked from our hotel to the Tar Pit Museum in a heat wave! I became faint and needed assistance to sit to prevent me from falling! “DENIAL”! My breathing was so compromised that I required extra pillows during the CT scan to breathe. The CT revealed severe aortic stenosis.

Dr. Raissi called me following my CT scan, and said I should call go immediately to see a TAVR specialist, Dr. Raj Makkar, MD!

No more severe stenosis!
Sweet heart sounds after valve-in-valve TAVR

Disbelief, shock and denial continued in my head… I requested that I visit Dr. Raissi first. He firmly said, “BJ you need an immediate AVR and surgery is too risky with your co-morbitities. Go to Dr. Makkar’s office at Cedar Sinai.” Mercifully, Dr. Makkar was in town and not traveling, and I was given priority and worked into their extremely busy schedule.

07-11-2018 – From grieving over one valve to welcoming another

After extensive testing, insurance approval and TAVR education, reality finally started to sink into my being. To me it was no longer the loss of my “new” surgical valve. I began to sense how fortunate I was to have the stars align once again with a life-saving procedure.

 I was especially grateful for the Sentinel Cerebral Protection System that Dr. Raj Makkar utilizes to reduce stroke risk during a TAVR procedure. One study shows that Sentinel CPS captured visible embolic debris headed toward the brain in 99% of TAVR cases. Studies show more than a 60% reduction in TAVR-related strokes when Sentinel CPS was used noted by the “Society of NeuroInterventional Surgery” and “American Association of Neurological Surgeons”.

I can breathe!

07-13-2018 – After valve-in-valve TAVR “I can breathe….!”

7:00 a.m.-TAVR procedure with the expert hands of Dr. Raj Makkar and his amazing staff!

10:00 a.m.- I awoke to my family and Arlys Velebir by my side!

My first words were, “ I can breathe lying flat!”

07-14-2018 – Out of the hospital 30 hours later!

I was discharged approximately 30 hours from my TAVR once again feeling as if I had a new lease on life!!! I was at the right place at the right time and my stars aligned…truly a miracle!

09-08-2019 – BJ today - Very grateful!!

I become more GRATEFUL each day I have to live. I no longer focus on how long my valve will last or “what ifs”.

BJ (with Dr Makkar)
after TAVR
Happy heart, happy life!!!

As I continue to discover new ways to care for my body, I am finding improved health, vigor and contentment. I no longer eat processed foods and am limiting my carbohydrates which have resulted in a 35 pound weight loss. I was an insulin dependent and insulin resistant diabetic prior to making these changes. I no longer need insulin after 17 years!

I try to live each day by the words Dr. Makkar blessed me with upon my discharge. 

He was very encouraging …

He gifted me with these words….

"Go and Live!…Go to the Beach!…

Travel!…Have Fun!…Live your Life!...

Happy Life!…Happy Heart!!!”

From one BAVer/TAD to another:

Unlike me,

please  be prompt to share your symptoms

 with your trusted physician

 or confidant

 and

 resist the temptation of DENIAL….

May your stars align on your BAV or TAD journey,

 all the days of your life!

~ BJ Sanders

TAD Awareness September 2019 - A Virtual Walk to Raise Awareness

TAD Awareness 2019 - A Virtual Walk to Raise Awareness of Thoracic Aortic Disease

Perhaps we can imagine a virtual walk together along the beach!

A Virtual Walk Together
Individuals and families impacted by thoracic aortic disease (TAD) are found all over the world. There are readers of this blog in many different countries, and it is likely we may never meet in person. However, this month we would like to take a "virtual walk" together with all who read here, through sharing stories of those with a form of TAD. We invite you to imagine walking with them for a few moments, as you read about them..

Background

Our imaginary walk together might take us here!

There was no awareness month for the aorta in the chest when the Bicuspid Aortic Foundation was formed. We believe it is important that, separate and distinct from what is commonly referred to as "heart disease", there is greater awareness of this major, vital artery that rises from the heart and the potential for life-threatening emergencies when it enlarges due to disease (aneurysm), tears (dissection), or ruptures. For those born with an abnormal aortic valve, such as those with bicuspid aortic valves (BAVs), they may at some point need both  their BAV, which is the doorway between the heart and the aorta, and an aortic aneurysm addressed. And so, we chose the month of September, a month far away from February, to distance thoracic aortic disease (TAD) from heart attacks and any confusion with what is commonly known as "heart disease", whose month is February. The aorta is worthy of having a month of its own!

You Are Not Alone

We need not walk alone with TAD!

We hope that in sharing real life stories this month, they will both inform and inspire all who read them, no matter where we may find ourselves in our personal journey with BAV and other forms of TAD.

There are many uncertainties and each each journey may be a little different, but we need not walk alone!  If we at BAF can share information with you, or just be there to listen and care, don't hesitate to reach out and contact us!

Walking together in September 2019

We are

Creating a Climate of Hope,

 ~ Arlys Velebir

                            Bicuspid Aortic Foundation

BAV Aortic Aneurysm Research Break Through

Diseased Aorta - Enlarged and Twisted Shape

This week we have exciting news to share about BAV aortic aneurysm research.

Desperate Need to Understand and Help
We are in desperate need to better detect, understand and appropriately help those with BAV and aortic aneurysms and their families.

Writing this, I remember  Doug Grieshop,  Forever 33, who left a young son and unborn daughter the day that his aorta ruptured. His BAV was never diagnosed, his aneurysm never found in life - only by autopsy.

Researchers Are Unmasking the Villains
At BAF we are very excited by published research, highlighted in the press this week, describing cellular villains in the aortic wall that destroy tissue around them and weaken the aortic wall.

This is work lead by Dr. Pickering, a research scientist, and Dr. Chu, an aortic and cardiac surgeon, who have collaborated to study aortic tissue removed from human patients. They have published their findings, reporting on the existence and trouble caused by abnormal smooth muscle cells in the wall of the aorta. 

According to Dr. Pickering, these abnormal cells produce an enzyme that eats away at the tissue around them, destroying and weakening the aorta. 

Following are press release and links with more information
Exchange Magazine  

Robart's Research

Dr. Pickering's background.

Dr. Chu's background.

Research Paper: Seno-destructive smooth muscle cells in the ascending aorta of patients with bicuspid aortic valve disease

Light Breaking Through the Mysteries 

Can Light Shed by Research Translate to Better Care?

This give us tremendous hope, but there is much more to do.

We know from the work done at Dr. Mona Nemer's Lab on GATA about the genetic abnormalities and BAV in mice families, who are so very much like our human families.

There are studies of abnormal blood flow through BAVs, of aortic wall stiffness.

There are studies about the leaflet configuration abnormalities of BAVs.

There are many things to study!

Now we know something important about the aorta tissue - that these abnormal smooth muscle cell secretions are breaking down the aortic wall!

There is not just one thing, but many things, to think about! What else is happening, that we do not know today? No doubt there is more to understand.

Light is just beginning to shine on multiple factors, some combination of which is making each BAVer experience somewhat unique, but with common tendencies and themes.

Aneurysms and Abnormal Smooth Muscle Cells
If we can identify these smooth muscle cell villains, can we get rid of them before they cause trouble?

Can we find out what causes these smooth muscle cells to become abnormal and prevent that from happening?

The Challenge of BAV Misunderstandings
It is a very difficult challenge when someone is the picture of glowing health on the outside, vibrant and active, but with a serious vulnerability on the inside - prone to aortic valve failure, aneurysm, infections.

Anything that sheds light on what is wrong on the inside helps both patients and physicians believe the reality of the BAV experience.

In the future, if aortic aneurysm disease were better understood, it might help physicians think about and look for BAV and an aneurysm in someone young and healthy-appearing with symptoms, like Doug Greishop, in time to save them. He lived in a state with world-renowned aortic surgical capability. It is tragic that he did not know, that no one knew in time, that he needed that expertise.

While we rejoice over the research and wait for it to translate into better care, we are very grateful for every successful aortic surgery that saves someone born with BAV and their TAV family members! And we do not forget those we have lost.

Today, we see light breaking through, but BAVers remain vulnerable. 

Someday, there will be more light than shadows in the experience of BAV families!

Answers are beginning to come,

Creating a Climate of Hope!

~ Arlys Velebir

                           Bicuspid Aortic Foundation

 

Questions in Search of Answers in 2018

Questions Without Answers

Why do some BAVers tear/rupture their aorta?

Why do some BAVers have aneurysms that do not tear or rupture?

Why are there BAV/aneurysm and  TAV/aneurysm in the same family?

Why do some BAVs calcify and narrow?

Why do some BAVs leak?

Why do some BAVs do both: calcify/narrow and leak?

Why do BAVers get infection (endocarditis) in their hearts? 
Why do some BAVers develop blood pressure issues?

Why are many BAVers so athletic and energetic?
Why do many BAVers (and their TAVer family members) have "delicate tissue", joints prone to injury, "bad eyes", and other issues through out their bodies?
Why can't my doctor tell me ......?

Why............?

Why............?

Why............?

There are so many unanswered questions when it comes to the individualized care that each person with BAV and their blood relatives should have.

As this year comes to a close, we invite you to support our search for answers. Many of those answers may be found in the mice families being studied at the University of Ottawa. With your help, the Bicuspid Aortic Foundation wishes to continue to support this vital work. Online donations through Razoo may be made here. 


Thank you for joining us in our search for answers.

Best wishes in 2018,

~ Arlys Velebir

                       Bicuspid Aortic Foundation 

September Awareness - Forever 33 - BAV, Aortic Dissection, Rupture

Doug a few days before his 33rd birthday

Forever 33

Sometimes it seems so cold

 to just write about someone.

 Somehow it feels right to address

 this personally to Doug Grieshop.

Dear Doug,

Tomorrow, September 19th, is your birthday. Can it be 12 years now since you celebrated turning 33 with your family? You should be with them still. Tomorrow you would be 45.  Instead, in your pictures and in loving memories, you are forever that strong, vibrant young man of 33.

We still have the first email Stacey wrote to the Bicuspid Aortic Foundation. The title "My Husband" did not prepare us for those first tragic words "My husband passed away unexpectedly...." It is the message of unspeakable loss that we hope never, ever to receive at the Bicuspid Aortic Foundation. For those who do not know, they can read what happened to you here, Bicuspid Aortic Valve and Aortic Rupture .

Doug's children remember
 Daddy's heavenly birthday

I know that knowledge is powerful, but when I remember you, Doug, I realize just how much power it has. As you took those last steps before you collapsed, you had never heard about bicuspid aortic valves and aneurysms of the aorta. You had never heard of  aortic dissection and rupture in the chest.

It seems so wrong that for months you were fighting something very real that no one understood or named, although it should be well known. Recently Stacey was mentioning what happened once again. She wrote of the multiple specialists you saw. There was a stress test. A chest x-ray, but no CT scan. You were given anti-anxiety medication. Of course, those things did not in any way help unmask the enemy in your chest. It hurts so much to realize that the technology and ability were there. In your own state is a world renowned center for treating this. You just did not know.

Doug, it would have been so wonderful to talk to you on the phone, to meet you in person, as we eventually did with Stacey and your family. We would tell you about your aorta in time, so you could get help.

We cannot change what happened, I know. I just want to tell you that because of you, we have the courage to tell others to persist in getting the help they need, to move on and get other opinions when doctors don't understand.

Doug, you inspire us to save others. 

This is your forever legacy. 

This September, 

we continue

to share knowledge 

Creating a Climate of Hope.

With our love,
Arlys Velebir and all the volunteers
at the Bicuspid Aortic Foundation

September Awareness 2016 - Learning About the Aorta

Richard Houchin (right) running along the route of the LA Marathon
following survival of aortic dissection

"Aorta? What's my Aorta?"

 As he lay on a stretcher, helpless and vulnerable, Richard Houchin remembers thinking this as he heard voices around him in the ER talking about him. Something was terribly wrong with his aorta, whatever that was!  Click here for details of what happened to Richard that day.

This was the way Richard learned not only about his aorta, but that he had been born with a bicuspid aortic valve. It is not the way anyone should learn this.One of the goals of this awareness month is to raise public awareness of the aorta, and how aortic disease can be detected before it threatens someone's life.

Following are a series of pages on the Bicuspid Aortic Foundation to help learn about this great blood vessel, truly a river of life in the body.

1. What is the Aorta?

2. What is a Thoracic Aortic Aneurysm?

3. What is Aortic Dissection?

4. What is Aortic Rupture? 


Along with these pages, here is a video from the Nucleus Medical YouTube Channel which describes the thoracic aorta and aortic dissection.

Learning together, we can be prepared to discuss not just our heart, not just our heart valves, but also our aorta with our physicians.

As we learn,

and are prepared to speak

with our doctors, 

we are

Creating a Climate of Hope.