September TAD Awareness 2021and Life-Long Aortic Care

 September - A Month for the Aorta!

Today BJ Sanders 
 Lives her Best Life Story
 through Proactive
Life-Long Care 

Since 2005, BAF has observed September as  Awareness Month for the aorta in the chest.

 Although those who have bicuspid aortic valves and their family members have a great deal of interest in the heart, whose month is February, we have learned that the aorta is vitally important also. We need them both to receive care to live our best life story! We also know that still today, Thoracic Aortic Disease (TAD) is too often overshadowed by the heart or just ignored altogether.

This September, BAF remains convinced that the aorta is more than worthy of  it's own month, distanced from February and "Heart Month" to avoid confusion.  At BAF we are delighted by the various days and weeks also designated this September that focus on the aorta! The aorta needs all the global attention it can get! 

This year BAF particularly highlights living your best life story through proactive Life-Long Aortic Care.

Life-Long Care and the Aorta

BJ Sanders is among those with BAV/TAD who receive Life-Long monitoring and care, including follow up imaging for both her heart and aorta. Described by one cardiologist at a major center as "complicated", she knows the importance of being proactive in order to achieve her best life story. 

The Aorta is Not the Heart! 

First of all, let's get better acquainted with a vital part of us that we may not have heard much about - our aorta! What is it, and how do various diseases affect it?

Diseased aorta in the chest

While there are some articles that describe the aorta and aortic dissection in the press, the aorta and the heart are sometimes blurred, potentially confusing the reader. Much more concerning and dangerous to life itself is confusing the heart and the aorta in a medical setting, often an Emergency Room(ER).

When doctors say our pain is not from the heart, we can ask them to check the aorta. In the United States, CT imaging is widely available and will find aortic aneurysm/dissection.

Very often there is no second chance to prevent an aortic catastrophe.

 At BAF, this September we do not forget Doug Grieshop and his family. This September19th would have been Doug's 50th birthday.

Doug Grieshop - Forever 33

 It remains particularly hurtful that, having checked him for signs of "heart disease" and failing to look further (at his aorta)  for a reason for his chest pain, this strong young man was given anxiety medication, which he took faithfully including the day his aorta ruptured. It was an autopsy that told the truth about his aorta, previously scarred by a small tear and now fully ruptured, his life story had ended much too soon. The diseased aorta remains among the subset of vascular diseases most frequently contributing to serious injury and death.

The Aorta is Not a Tube!

The authors of this recent paper from Italy begin by introducing the aorta:

Pathology of the Aorta and Aorta as Homograft

They open with "The aorta is not a rigid tube, it is an 'organ'....", and go on to beautifully describe it.

The aorta is not "just" a tube, any more than the heart is "just" a pump. The aorta is a vital entity in its own right, and prone to various disease conditions, as these authors describe.

We continue to press for the aorta to be included in proactive diagnosis and ongoing care for all those who need it so much. In a series of articles throughout September, we will share examples of those who would benefit from and need Life-Long Aortic Care. Sharing information is an avenue to empowering the public to advocate for their own best care.

TAD is often silent, taking years to suddenly explode into a bleeding emergency in the chest. Our theme is simple this September:

 Receiving Life-Long Aortic Care

we can

Live our best life stories,

 Creating a Climate of Hope, 

~ 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

 

 

 

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 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.

Aortic Aneurysm and Dissection Risk from Fluoroquinolone Drugs (Cipro, Levaquin, etc.)

Aortic Aneurysm and Dissection and Fluoroquinolone Drugs 

Raising Awareness - Why?

There have been some additional questions about recent medical papers and the FDA warning about this group of drugs, which the public recognizes by names such as Cipro and Levaquin. Some may have wondered why the Bicuspid Aortic Foundation is raising awareness about this. They may wonder if it has something to do with their heart. It is not the heart, it is the aorta that is getting attention, along with tendons, muscles, and other areas of the body.

To be clear, these warnings are for the general public, for everyone. 

It is helpful to tell everyone about this.

The reason the Bicuspid Aortic Foundation is putting so much emphasis on this is because we advocate for a special group within the public at large, those who already are prone to developing aneurysms of the aorta in their chest.

Quite simply, there are important questions that we must ask:

Ascending
Aortic
Aneurysm

• If it puts the general population at risk of aneurysm and dissection, what about those who already have enlarged aortas or full blown aneurysms?

• Would this group of drugs make already enlarged, bulging aortas even worse, more dangerous, more quickly? 

Medical Papers in 2015  and the aorta.

In November 2015, two papers were published in medical journals by doctors in Taiwan and Toronto, Canada. Then in May 2016, the FDA issued a new warning.

Here is the abstract of the paper from Taiwan:

Risk of Aortic Dissection and Aortic Aneurysm in Patients Taking Oral Fluoroquinolone

Here is the paper from Toronto:
Fluoroquinolones and collagen associated severe adverse events: a longitudinal cohort study

This paper gives details about what happens to the collagen in the aortic wall:

" In the aortic wall, type I and type III are also the dominant forms of collagen,12 thereby suggesting that a medication contributing to tendon ruptures could also lead to aortic aneurysms. Indeed, pathological sections of aortic aneurysms and aortic dissections demonstrate abnormalities of collagen content, concentrations and ratios.13 Although aortic aneurysms typically develop slowly, our data suggest that fluoroquinolone prescriptions can contribute acutely to aneurysm progression and rupture."

Aneurysms Grow and Rupture More Quickly

Please note that the last sentence, which says that these drugs cause the aneurysm to grow quickly "acute .... progression" and then rupture.  So the general thought that aneurysms do not need to be checked that often, because they grow very slowly, may be dangerous for someone who has taken these drugs.

FDA Warning May 2016

Once again, here is the FDA warning from May, 2016: http://www.fda.gov/Drugs/DrugSafety/ucm500143.htm

 

Here are the first two paragraphs:

"The U.S. Food and Drug Administration is advising that the serious side effects associated with fluoroquinolone antibacterial drugs generally outweigh the benefits for patients with acute sinusitis, acute bronchitis, and uncomplicated urinary tract infections who have other treatment options. For patients with these conditions, fluoroquinolones should be reserved for those who do not have alternative treatment options.

An FDA safety review has shown that fluoroquinolones when used systemically (i.e. tablets, capsules, and injectable) are associated with disabling and potentially permanent serious side effects that can occur together. These side effects can involve the tendons, muscles, joints, nerves, and central nervous system."

While mentioning tendons, which are high in collagen, this statement does not mention the aorta. The papers from Taiwan and Toronto do.

Talking with Doctors

Taken together, these three references help me when talking to doctors about my own family. They help me remember that I should tell them if I or a loved one has ever taken one of these drugs. It also helps me ask for the safest possible drug, if I or my loved one should need an antibiotic. I hope this information helps us all. 

Staying informed together,

Asking our questions,

We are all 

Creating a Climate of Hope.

~ Arlys Velebir

                          Bicuspid Aortic Foundation

What Happened to Amy?

This is Amy's story. Only her name has been changed, to protect privacy.



Where is Amy?

"It's just not like her, Amy would have let us know if she couldn't come!" And so a "welfare check" was requested. Her home was silent. There was no response to the doorbell, the knocks, the calls of her name. Something was terribly wrong!

They found Amy inside her home, lifeless. Apparently there had been no opportunity to even call for help.

What Happened to Amy?

Her family members were notified. The police arranged for her body to be taken directly to a local mortuary. Deeply shocked, her adult children needed answers. Their mother had been an active, independent, and apparently healthy woman. She did not even have high blood pressure. They thought she would be with them for many years to come. How could this happen, without warning?

 They needed answers.

In the place where Amy lived, no one offered to find those answers. After all, there was no evidence of a crime, no villain for the police to pursue. The death was clearly due to "natural causes". But, why? Is it really "natural" to die like this, without warning, when someone is so healthy?

Why Did Amy Die?

Amy's daughter told me that not knowing was unbearable to her family, much worse than the thought of an autopsy would be. Despite their tremendous shock at her sudden death, Amy's family found the strength and the financial resources to request a private autopsy. The local medical examiner was not available, and so Amy's family found their way to someone they describe as special, a compassionate, caring physician who carefully examined their mother's body and helped them understand.  What that physician found inside would have been a gruesome sight.

Quite simply, Amy bled to death. Unknown to her, she had an aortic aneurysm in her chest just above a bicuspid aortic valve in her heart. Because there was an autopsy, Amy's death certificate is accurate, stating that death was caused by the rupture of an aortic aneurysm in the presence of a bicuspid aortic valve (BAV).

Courage to Press for Answers

Sometimes families cannot bear the thought of an autopsy on their loved one.  There also may be veiled opposition, if the death occurs in a hospital, from a medical establishment that fears malpractice lawsuits. Sadly, the days of Dr. M.E. Abbott and Sir William Osler, who learned from autopsies and published extensively about aortic disease in the chest, are gone. As with Amy's family, it is the survivors' legal right to have an autopsy, but they may have to pay for it.

Knowledge - a Gift of Life 

Amy's family was left with the sad, painful task of sorting through her things, closing her home. She was an avid reader, and they found many articles about keeping healthy, preventing disease. Among them may have been the usual warnings about preventing stroke and "heart disease". Like many with BAV, Amy's arteries in her heart were perfectly clean. She was not at risk of a heart attack. Sadly, there was nothing about BAV and aortic aneurysm, the condition that suddenly robbed her of life. Amy would not have even heard about what snatched her life away. If her family had not arranged a private autopsy, the reason for her death and the potential threat to their own lives would never have been known by anyone at all.

Knowledge like this comes at an unspeakable price. It is a price too high, too great to pay. It should not happen.  There is a well known medical center not far from where Amy lived that could have performed her aneurysm surgery. They do this surgery frequently, perhaps almost every day.

Amy's blood relatives are educating themselves, being checked for BAV and/or aortic aneurysm. No one needs to tell them why now.

They know.

The Uncounted

The Bicuspid Aortic Foundation remains extremely concerned about the uncounted deaths due to BAV and aneurysm complications. Amy could easily have been one of those uncounted deaths, if her family had not had the courage and ability to seek the truth. Sudden deaths are too often simply recorded as due to natural causes, perhaps assumed to be due to a heart attack, but without any proof. 

The lack of understanding and recognition of aortic dissection/rupture deaths in the presence of BAV, as well as sudden aortic valve related deaths has consequences not just for those who die, but for the living.

Other blood relatives may be at risk. But there are also consequences for the entire BAV population. Because these people are uncounted, they are not found in the statistics, not included in the medical papers published about patients who receive treatment. How can we understand those most at risk, those who may never even reach a hospital, if we don't learn from them? We don't even know why they died! And so, the statistics upon which treatment of the living is based can not include these unknowns, perhaps the most vulnerable, the most fragile. Must they continue to die?


 Who Will Speak for Me?
Amy began this year, 2016, as we all did, with plans and hopes for her future. They were never to be realized, because she had been born with a bicuspid aortic valve, and over time an aneurysm developed. Today her voice is silenced. Who will speak for her, and the others we lose? 


At the Bicuspid Aortic Foundation we can help give them a  voice and bring into focus these most vulnerable. We are enlisting the aid of pathologists to do so.
They are the physicians who speak for the dead, and in doing so, may save the living. We are also encouraging families where sudden death has occurred to take courage and inspiration from Amy's story, and seek the answers they need.

In giving a voice to Amy,
And others we have lost,

May we help the living
And in their memory, 

Create a Climate of Hope.

- Arlys Velebir