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 

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 with Bob Gies

An actual walk with Bob would be a fast one! Like many with BAV/TAD, his entire life has been on the go, pushing to the max, always at high speed!  

Ready for a ride, September 2019
A typical ride is at least 12 miles, sometimes up to 20

He agreed to pause and share this virtual walk with us, where we learn that despite having just celebrated his 75th birthday, Bob is still going strong! Ten years ago, he wasn't sure he would see this day. And if he was still alive, would he still be doing the things he loved?

A Gifted Athlete with a Heart Murmur

Bob remembers hearing doctors talk with his parents when he was very young, talking about his heart murmur. When he was ready for his freshman year of high school, more than anything else, he wanted to play football. A physical was required, and he still remembers what the doctor said to him, "You have a heart murmur, kid. But you're a healthy specimen, you'll be all right!"  Yes, the doctor let him play! Bob remembers thinking that this heart murmur must be something very special. Looking back, he never had any vision of ever having  a serious heart problem, not ever! 

Football in the Fall, Track in the Spring

In addition to high school football, which he played all four years, Bob excelled at track. He was a fast sprinter, winning both the 100 and 220 yard dashes, and also ran the low hurdles.  His junior year, at the state track meet, Bob scored more points than the rest of their team combined. A sprained ankle kept him off the track his senior year.

Somehow, Bob seemed to be just made for football. He says that great runners get all the glory in this game. Fast on the track, Bob was fast on the football field as well, thrilling the crowd each time he broke loose and raced down the field for a touchdown! Pointing out that avoiding being caught and tackled was an extra incentive, Bob says "If I ever got two steps ahead, they could never catch me."

Championship Game 1963

Bob is number 24

In the fall of 1963, his senior year, Bob describes the Huskies as a terrific football team, with lots of good blockers, everyone working together. A newspaper clipping from that time mentions that the backfield - Gies, Koontz, and Becker - was possibly one of the greatest combinations in the history of any Class C school. They were the dream team! What happened that year is well remembered to this day. They went into the final, championship game undefeated. They were also determined to always have the lead, to never fall behind, just as they had done all season! The game started with a kick off to the opposing team, who pushed them back to the 30 yard line. On their first play, Bob broke loose and carried the ball all 70 yards - touch down! That first play set the tone for the entire game. They were ahead from the start, and they stayed ahead, all the way to victory!

Greatest Of All Time (GOAT) Football Record 1963

Athletic Hall of Fame
Honors for Bob
 in 2016

Bob returned to his high school to be named to their Athletic Hall of Fame in 2016.

In 2018, a local paper wrote  about some of the football history for his high school.

There is Bob's name, along with two of his team mates, Koontz and Becker, on the GOAT list.

Bob still holds the record for rushing more than one mile his senior year and scoring 136 points that season. He did that while playing both offense and defense.

Bob on Sheep Mountain
About 30 Years Ago
3.5 hours to ski up,
 a fast 40 minutes back down!

Outdoor Sports After High School

Bob turned to outdoor, individual sports after high school: cross country skiing, biking, fishing, and generally enjoying the outdoors under that big Montana sky! There was no reason to remember that "heart murmur" of his youth!

He felt he was slowing a bit, fatigue and naps entered the picture at around age 60. Read here how his bicuspid aortic valve and aneurysm were discovered and about the surgery that followed. 





September 2019

This September finds Bob still busy in his bountiful garden, which he describes as a lot of work and great exercise!

He maintains his weight within a pound or two of what he was in high school. His step counter averages around 6,000 a day, with a peak of 24,000. Looking at his number during the day motivates him to get busy and do more! And then there are the miles on his bike, the miles on skis. . . .

It is just over 9 years since Bob had his life-saving BAV and aneurysm surgery. His bovine aortic valve and aorta are looking good at the nine year mark!

When asked how life has changed at this age, Bob says he misses pushing his body to the absolute limit, going flat out to the wall . . . his body just won't let him do that anymore!

His lifelong craving for exercise is still there, however. Exercise still makes him feel good!

Thank you, Bob, for sharing your life

on this walk with us,

Inspiring us, 

and

Creating a Climate of Hope,

  ~ Arlys Velebir,  BAF

                       

TAD Awareness 2019 - A Virtual Walk with Larry Abramson

September is Thoracic Aortic Disease Awareness Month
In 2019 BAF is raising awareness about Thoracic Aortic Disease through a "virtual" walk. From time to time this month we will invite you to walk along with someone with TAD, through reading their story.
Here we invite you to come and walk alongside Larry. He has an amazing story to tell, beginning at a very young age! Today he continues to live his very active, adventurous life! We hope that "walking" with him and "listening" to his story will inspire you to do the same! 

Age 4 - Diagnosis, Age 11 - First Valve Surgery

Larry Abramson

My BAV was discovered during a routine exam in 1959 when I was 4 years old. I was monitored every 6 months and restricted to "no sudden exertion or contact sports". But, life went on until Dec 1966 when I started having some issues with shortness of breath and actually fell while swinging on a rope hung from a tree. In Feb 1967, because of a thickening of the valve where the 3rd leafet would have been, the day after my 12th birthday, I went in to the hospital where they performed an aortic commissurotomy (opening up the valve to allow the valve to function better) but was told that the repair would probably only last about 7-8 years.

Age 20 - Second Valve Surgery

In 1975, I once again started having issues with shortness of breath and this time, at age 20, I went in and had a mechanical valve put in my heart at Bryn Mawr Hospital in PA. It was amazing to see the difference in the procedures in the 8 years since my first surgery. After the first surgery, I woke up naked, laying in an oxygen tent, on a bed of ice with the oxygen running through ice so they could bring my body temperature back up to normal very slowly. 3 days later, they finally allowed me to start eating a normal diet. This time, my body temp was already back to normal and, had I not accidentally pulled one of the drain tubes loose which collapsed my lung, I would have been back in my room the next day. From that day forward, I had a fairly normal life with no issues, and was just monitored every 6 months, though of course my Pro-time (now measured differently and called INR) was checked monthly.

Age 53 - Aneurysm Diagnosis and Third Surgery 

Larry and Michele in NYC in 2008, 10 days after surgery!

In 2008, I saw my cardiologist in June and was told everything was fine. In early July, I was browsing the internet and came across The Bicuspid Foundation website. While looking over the site, I found that they had recently discovered that people with BAV were developing aneurysm's in the ascending aorta. Well, I had just seen my cardiologist and he didn't seem concerned, so maybe it wasn't a big deal. But...the more I thought about it, the more concerned I became so I emailed the Bicuspid Foundation website to inquire about it. The next day, I received a message on my cell phone from Dr Sharo Raissi who was the medical advisor for the website and the Director of Thoracic Surgery at Cedar Sinai Hospital in L.A. The message said "this is my personal phone number...you need to call me...we need to talk!". Well, this was certainly disconcerting.

So, I called Dr Raissi and we discussed the fact that I had also had some hernia issues, which he explained were also connective tissue and that I should probably have an Echo or a TEE to get a baseline measurement. So, I called my cardiologist and we discussed it. At that time, he had not heard about the aneurysms but was willing to look into it. He called me back about a week later and agreed to an Echo. On July 25, 2008 I had an Echo which, according to the radiologist, revealed a 50mm (5.0cm) aneurysm in 2 places in the mid-to-distal ascending aorta. My cardiologist determined that I probably should see a surgeon, though he also admitted that there probably wasn't anyone in the Memphis area that could do it.

I saw a surgeon here, and my wife and I decided that he wasn't the one to do my surgery. So, I got copies of my films and did much research on the internet as to the best surgeons to perform this type of surgery; we sent them to a number of surgeons across the U.S. including Dr Raissi, the Mayo Clinic, Massachusetts General, Weill Cornell Medical/NY Presbyterian and a few others. Dr Raissi said that, in his opinion, the aneurysm looked to be about 5.4cm, and I got similar opinions from the other doctors.

After much discussion, we decided to go to NYC and NY Presbyterian to have my surgery done by Dr Leonard Girardi and on September 11, 2008, my aorta was repaired/replaced along with replacing the valve again because even though the original mechanical valve was working great, it was determined to be easier to put a one piece aorta and valve in vs trying to connect an artificial aorta to a mechanical valve.

Today

Now...eleven years later and 52 years after my first heart surgery, I'm still here kicking though granted, not as hard as I used to. I thought I would share this for those of you who are just finding out about your BAV, or that your child has BAV. It's a shock to find this out to begin with, but life goes on and you, or your child, can lead a normal life (yes, normal...I spent 32 years as a Federal Law Enforcement Officer, 19 years as a volunteer firefighter, I scuba dive, I rode a motorcycle for 20+ years (yeah, I know...not too smart being on Warfarin), and generally enjoy life. 

Hang in there...it will get better!  ~  Larry Abramson

Thank you, Larry, for sharing your life with us,

and

Creating a Climate of Hope,

~ Arlys Velebir

                          Bicuspid Aortic Foundation

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

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 

September 2016 Awareness Month - It's All About the "Big A" in the Chest!

Normal Aorta

The aorta is so important,

 we call it the "Big A"! 

To baseball fans in the US, the Big A means Angels Stadium in southern California. At the Bicuspid Aortic Foundation, we mean something else; to us, the Big A is the aorta in the chest. It is the largest artery in our bodies, and it's proper functioning is without question vital to our lives.

Why does the aorta need its own awareness month, September?

Maybe the aorta could just share February with the heart?

Tragedies happen when no one thinks about the aorta. In the emergency room (ER), someone with chest pain will be checked for a heart attack. The blood tests come back normal, so is the EKG. It's not the heart.

No one thinks of the aorta.

The minutes go by, turning into hours. Sometimes the person is still in the ER, sometimes they are back home. Suddenly, they are dead. The opportunity to help them was missed. 

All because no one thought about the aorta.

This is one reason that the aorta needs its own month - just to help everyone think about it in time.

Aorta with aneurysm

Another reason is that aortic disease is very different from what is typically called heart disease. The treatment is different too.

Aortic disease is not heart disease!

If it's not the heart, check the aorta! 

Let's Learn All About the Big A this September!

In a nutshell, this large, high pressure artery should not be confused with the heart. Having its own month provides a time to learn about the aorta in the chest: what it does, the risks aortic disease presents to us, and the treatment available when needed. 

At the Bicuspid Aortic Foundation, we will spend this September featuring articles on the following topics:

• Thoracic Aorta - what it is, what it does
• Thoracic Aortic Disease
• Thoracic Aortic Aneurysm
• Thoracic Aortic Dissection
• Thoracic Aortic Coarctation

We will also honor the memory of those who were cruelly taken from us by thoracic aortic disease, and the courage of those who live with injury caused by thoracic aortic disease.

Shining a spotlight on 

the Big A,

Together we are

Learning, Sharing,

Empowering each other,

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

Talk to Your Doctor! Fluoroquinolones (Cipro, Levaquin, etc), Collagen Damage Warnings

Classic picture of aortic disease:
 bulging, stretching, curving due to weak connective tissue

There is never a good time to be sick, but why is it that it always seems to happen at the worst time - in this case, a long holiday week end? Yes, this Memorial Day week end my husband's painful left ear sent us to urgent care for help. While there, we had a very important conversation about the right antibiotics for him.

Ear Infection

We don't know why, but this is the second year that my husband has developed ear infection for no apparent reason. He did not suffer with this in childhood, as many children do. Now with an impressive number of birthdays to his credit, what is this all about? He was born with a bicuspid aortic valve, but more than that, there are subtle signs of other "special" things through out his body. No doubt this will move us to investigate further, looking for a reason why this is happening.

Urgent Care

After examining my husband, the doctor explained to us what he saw inside the ear. His stethoscope to my husband's chest had already alerted him to the special sound of the artificial tissue valve in my husband's chest, and he understood the special risk of infection because of that valve. In our experience since the first valve was implanted 26 years ago, this is something that has always been well understood by the doctors he has seen.

"This is in the Cipro family"

As before, both an oral antibiotic and antibiotic drops were suggested. Two choices were mentioned regarding the drops. My husband asked a second time about this. And it was then that the doctor said those words, "This is in the Cipro family"!! I immediately mentioned the recent strengthening of the FDA warning announced on May 12, 2016.  I also said that I had recently discussed this with his aortic disease doctor. We did not want anything from this family of drugs! We settled on the same drops he had received previously, from a "mycin" family, which had not been a problem for him.

Speaking Up

Likely at the pharmacy, but certainly at home, we would have read the labels and caught this. It was so much better to have the discussion directly with the doctor and avoid dealing with it after the fact. It is important to realize that this family of drugs has been available for a long time, and the latest, strongest warning was just issued May 12th. So it is important for us, as the consumers, to be informed and speak up!

My husband is very careful about the medication given to him. Ten years ago, following his last surgery, despite being very unwell in many ways, he asked the name of the medication he was being given in the hospital each time, before taking it. Human beings are fallible, prone to accidentally making mistakes.

Be Informed

Read the May 12th FDA warning.

There is a full paper available to us, published by doctors from Toronto last fall. Those with BAV and other forms of TAD already have issues with the collagen damage/weakness in their aorta and other areas of their bodies. What would taking this drug do to them?

Read it yourself! Print it out! Share it with your doctors!

Fluoroquinolones and collagen associated severe adverse events: a longitudinal cohort study

Sharing information is powerful,

Creating a Climate of Hope.

~ Arlys Velebir

                        Bicuspid Aortic Founation

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