September Awareness 2021 - TAD and the Tip of the Iceberg

 

The Tip of the Thoracic Aortic Disease Iceberg?
Photo by Danting Zhu on Unsplash

At BAF we have spent the month of September 2021 focusing on those who need life-long thoracic aortic care. 

It is so encouraging to see the increased global awareness of thoracic aortic disease. It has come a long way in the 20 years since my personal experience with my husband began. In the closing hours of this month, I am caused to reflect with gratitude on that which is seen and known - that which is above the water, so to speak.

Below the Water Line

However, those unknown, unseen, "below the water line", are those with unmet TAD challenges, those not receiving life-long aortic care. Who are some of these people? 

• the undiagnosed
• diagnosed with bicuspid aortic valve (BAV), aorta not evaluated and monitored
• replaced BAV, prosthetic valve monitored but not aorta
• TAV relative of someone with BAV, aorta not baselined or monitored
• prior elective aortic procedure, such as replacement of a portion of the ascending aorta, remaining aorta not monitored
• prior elective aortic surgery for coarctation, not receiving effective ongoing monitoring

What about those who survive aortic dissection?

Are those who survive an aortic dissection ever presumed to be "fixed"? Do they become "lost to follow up"? Those we have met at BAF are receiving careful monitoring, perhaps because of their physicians' appreciation for their very survival and the injury done to their aorta. If any of them are not receiving ongoing monitoring and additional intervention as needed, they are also at increased risk, below the water line.

The BAV/TAD Population - 6.7 Million People in the US Alone

The BAV population is large: estimated at up to 1 in 50 people (2 %). Checking the census bureau population clock, it displayed the number of people in the United States as 332,800,350 people. Two per cent, or 1 in 50, is 6,656,007 people born with BAV in the United States alone. 

Whenever I am in a group of 50 people or more, I wonder about those around me, more frequently they are males, born with a two-leaflet aortic valve in their heart - possibly already replaced. I have met some of them over the years, sometimes in such unexpected settings.

Some statistics mention that 5% of those with BAV dissect. Five percent sounds small. In the financial world, it's only 5 cents out of a US dollar. When applied to a large number however, such as 6.7 million, that relatively small percentage yields a six-figure result: 335,000.  

When applied to humanity, think of the tremendous number of BAV lives with a torn aorta in the United States alone.  However, this is a global, not just a United States, problem. 

The BAV/TAD View in 2021 

Twenty years ago, it was very difficult to find information and help for an "ascending aortic aneurysm", but we did it.  Knowing how challenging it was, we were moved to help others, many who also no doubt thought they were "fixed for life" by a BAV replacement.

There are still those today with a replaced BAV and presumably receiving good ongoing care, who have no idea their aorta is bulging, endangering them. I spoke to one of them quite recently, whose BAV was replaced 9 years ago. This person is above the TAD water line now, the bulging aorta exposed, receiving comprehensive aortic care.

I ponder once more how many with BAV/TAAD die undiagnosed, uncounted, a "natural" death without autopsy; perhaps an in-hospital death due to aortic dissection, but the aortic valve condition unspecified. How large is the iceberg beneath the water?

In 2021, we are in a better place in many ways, but not yet where we aspire to be in terms of BAV/TAD. We look forward to new advancements that will come through the efforts of gifted physicians, surgeons, and researchers around the world. 

As those affected 

are included

 in the heart/aorta teams of today,

 together we will advance,

 lowering the water line,

more receiving life-long aortic care,

Creating a climate of hope,

~Arlys Velebir, Bicuspid Aortic Foundation

 

September Awareness - Lifelong Aortic Care after Elective Surgery

Diseased Thoracic Aorta

One Man and his Aorta - Years after Surgery, Another Aneurysm!

This September I am remembering one particular man and his wife. Although I only met them once, well over a decade ago, their experience remains with me. I share it this September, in terms of the aorta and life-long aortic care.

He appeared to be a strong man, in the prime of his life, who had not been given any specific "label" for his TAD. His wife was with him, deeply concerned about her husband, as together they sought more information. He had already had a voluntary aortic surgery years before, when quite young. His aorta had never torn (dissected).

Aortic Arch Aneurysm - "Maybe it has always been this way!"  

 I recall his wife saying those words -  hoping against hope that this recent picture of his bulging aortic arch was just the way his aorta had always been - that nothing was actually wrong. TAD, silently progressing over years, makes it far too easy to spend time in denial. 

This reference describes isolated aortic arch aneurysm as "uncommon": Aortic Arch Aneurysm . 

Uncommon, perhaps, but it had happened to him. He had perhaps a little knowledge of what his aorta was like right after it was repaired. However he had not had "aortic pictures" taken from time to time since then - pictures that could have captured the changes in his aortic arch that now had become this pronounced bulge. Whatever had prompted the recent imaging, these pictures very clearly had captured the aneurysm of his aortic arch.

In time, as they continued their search, they may have come to realize that, however unwelcome the discovery, it gave them the opportunity to avoid an aortic crisis - time for opinions from surgeons who would assess his aorta, time for them to ultimately choose an aortic surgeon to skillfully repair his arch while protecting his brain. I hope so.

Ongoing Monitoring, Baseline After Surgery, Lifelong Aortic Care

Reflecting on the aortic care my family has received over the past 20 years, I am thankful. Once an aortic enlargement or aneurysm in the chest was found, there has been ongoing monitoring, taking those "aortic pictures". 

The importance of blood pressure monitoring at home and medication as appropriate has been emphasized.

After surgery, we understood the extent of aortic repair, and a baseline image of the repaired aorta was captured for future reference. Then, back to appropriate, periodic imaging.

This is the essence of the Aortic ABC's

We do not outgrow the Aortic ABC's after Surgery.

 Surgery sometimes includes both aorta and BAV;
and then, back to life-long follow up care.

The Bicuspid Aortic Foundation defines the "Aortic ABC's" as follows:

• Accurate diagnostic testing
• Blood pressure management
• Comprehensive, individualized Care

There is great concern for those who do not  receive ongoing aortic care after a planned, successful surgery - perhaps stemming from the thought that aortic surgery is a one-time, life-long "fix".

Elective surgery may have repaired the aorta. How long will that repair last? Will some other portion of the aorta, years later, require attention?

 For a long life,

 as long as we live: 

Life-long Aortic Care,

Creating a Climate of Hope,

~ Arlys Velebir, Bicuspid Aortic Foundation

September TAD Awareness - Life-long Aortic Care for Coarctation

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

Heart emergency - right care at right time

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

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

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

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

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

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

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

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

Current management of coarctation of the aorta 

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

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

The knowledge exists.  Are people receiving the care?

Life-Long Aortic Care and Coarctation

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

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

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

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

For those with coarctation,

Life-Long Aortic Care

is

 "the right care at the right time"

~Arlys Velebir, Bicuspid Aortic Foundation

September Awareness 2021 - Why "TAD" Awareness

2001  and "TAA" (Thoracic Aortic Aneurysm)

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

2005 and Thoracic Aortic Aneurysm and Dissection (TAAD) Awareness

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

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

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

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

2021 and Thoracic Aortic Disease (TAD) Awareness

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

One Man's Life-Long Aortic Journey Until Now 

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

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

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

Journey Beginning  from Age 2 

Hidden Danger of Patched Coarctation

"So, life is precious"

 as Father Prodromos wrote,

and life can be extended 

for those with TAD

when they receive

 Life-Long Aortic Care.

~Arlys Velebir, Bicuspid Aortic Foundation

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

Misdiagnosis and Disease - A BAV Perspective

Misdiagnosis and the "Big Three" Categories: 75% of "Serious Harms" , 50% from 15 Diseases 

Two papers published in the journal Diagnosis in July 2019 and May 2020 seek to shed light on the harm done by misdiagnosis of known diseases. These open-access papers are available online, as listed below (1) (2).  The authors identify three major categories and five diseases in each of them that are estimated to account for about 50% of serious injury and/or death: vascular events, infections, cancers. 

A Bicuspid Aortic Valve Perspective

Of the three categories, both the vascular and infection disease categories are of particular importance to those with BAV. Clinical significance of the bicuspid aortic valve , published in 2000, states that BAV may be responsible for more death and morbidity than all other congenital heart defects combined. Perhaps it is not surprising then, that the diseases listed include several vascular and infectious diseases known to cause injury or death in some who were born with BAV.

Diseased aorta in the chest

In the vascular events category the 5 diseases are:

• Aortic aneurysm and dissection
• Arterial thromboembolism
• Venous thromboembolism
• Stroke
• Myocardial infarction (heart attack)

In the infection category the 5 diseases are:

• Sepsis
• Meningitis and Encephalitis
• Spinal abscess
• Pneumonia
• Endocarditis

From the authors' conclusion: "We estimate that roughly one in 10 patients* with a dangerous 'Big Three' disease is misdiagnosed, and roughly half of those misdiagnosed die or are permanently disabled as a result." (1)  They go on to note that there is no indication that the error and harm rates are declining. They also mention there has been improvement in those diseases that have received actual sustained emphasis over several decades. 

BAV and These Diseases

Reading these papers I reflected on my late BAV husband's experience with 5 of the 10 diseases above at different points in his life, and the challenges we faced with diagnosis and treatment. He survived these serious threats to his life until intractable bacterial endocarditis ended it. His doctors marveled at how much he was able to overcome, which is the case with so many other "BAVers" I know.

Each person with BAV has their own journey. Seeking help may bring them to a physician's office, the
ER, perhaps both. Once there, even at the very first indication of an issue, will the disease be found in time to prevent disability or death?

What Can We Do? Be Informed, Be Proactive!

What can we do when we need help? We can be aware of these too often-missed diseases - the possible presence of an aortic aneurysm, the pain of an aortic dissection crisis, the potentially deadly infections - sepsis, endocarditis, or even pneumonia.

 With that awareness and realizing that these diseases may be missed, we can ask questions and whenever possible get additional opinions from the best expertise available. 

We can pursue answers,

 without embarrassment when discounted and misunderstood,

realizing the consequences to our future

if potentially deadly conditions are not found in time. 

Perspective from Sir William Osler in 1907 - Diagnosing Aneurysm of the Aorta

These diseases have long plagued humanity, and the challenges in finding them are not new either. The following words were written long ago by the renowned physician, William Osler, regarding diagnosis and aneurysm of the aorta (3), 

"The well-known dictum of Jenner may be taken as text: 'More mistakes are made by not looking than not knowing.' A majority of aneurism of the thoracic aorta present suggestive features to the eye, but the inspection must be made with care." 

Writing of having missed aortic diagnoses, Osler mentions an example of someone named McKinley. He continues with the often quoted:

"There is no disease more conducive to clinical humility than aneurism of the aorta."

Continuing, his words at the close of the page are compelling, where he quotes Pirogoff:

"Mistakes occur with the most careful and most skilful. Sometimes the diagnosis is beyond our art; more often it is not made because of the carelessness that so easily besets us in our work. The confession of the great Pirogoff always seems to me most touching: 'There are in everyone's practice moments in which his vision is holden, so that even an experienced man cannot see what is nevertheless perfectly clear, at least I have noticed this in my own case. An over weaning self-confidence and pre-conceived opinion, rarely a weariness, are the causes of these astonishing mistakes.'" 

It is over 100 years since Osler wrote about diagnosing diseases of the aorta and aneurysms, citing Jenner and Pirogoff (4).  The tools of modern technology have not solved the diagnosis challenges. The authors of these recent papers, several from Johns Hopkins where Osler came as one of the founders in 1888, are bringing much needed focus to the harms done when certain diseases are not found or not found quickly enough. May the transparency and humility of Osler, Jenner, and Pirogoff, coupled with courage in the face of deadly diseases, be inspirations today to overcome limitations and find mechanisms to reduce misdiagnosis.

The Challenge in 2021

Doug Grieshop's Diagnosis,
 BAV with Aortic Dissection/Rupture,
 Came too Late, During an Autopsy

Consistently diagnosing just a small number of diseases in time (aortic aneurysm and dissection, endocarditis, and sepsis among them), would drastically alter the ability of these diseases to disrupt and destroy lives, some of whom were born with bicuspid aortic valves. Someone with BAV has too often been discounted with "anxiety", sent home when a heart attack was ruled out. Some are disabled, some have died. 

The crisis eventually passes,

 but the scars linger.

May the new light these papers are shining

 be a springboard to improve diagnosis,

significantly reducing injury and death.

 ~Arlys Velebir,  Bicuspid Aortic Foundation

(1) Newman-Toker, David E., Schaffer, Adam C., Yu-Moe, C. Winnie, Nassery, Najlla, Saber Tehrani, Ali S., Clemens, Gwendolyn D., Wang, Zheyu, Zhu, Yuxin, Fanai, Mehdi and Siegal, Dana. "Serious misdiagnosis-related harms in malpractice claims: The “Big Three” – vascular events, infections, and cancers" Diagnosis, vol. 6, no. 3, 2019, pp. 227-240. https://doi.org/10.1515/dx-2019-0019

(2) Newman-Toker, David E., Schaffer, Adam C., Yu-Moe, C. Winnie, Nassery, Najlla, Saber Tehrani, Ali S., Clemens, Gwendolyn D., Wang, Zheyu, Zhu, Yuxin, Fanai, Mehdi and Siegal, Dana. "Serious misdiagnosis-related harms in malpractice claims: The “Big Three” – vascular events, infections, and cancers" Diagnosis, vol. 6, no. 3, 2019, pp. 227-240. https://doi.org/10.1515/dx-2019-0019

(3) Modern Medicine Its Theory and Practice, Volume IV, Diseases of the Circulatory System - Diseases of the Blood - Diseases of the Spleen, Thymus and Lymph-Glands, Chapter XI Aneurism,William Osler, MD, FRS, p472.

(4) The Life and Work of Nikolai Ivanovich Pirogov (1810-1881): An Outstanding Anatomist and Surgeon

*Bolding added to quotations through out.

BAV Lifelong Care - Carrie Mettler's Journey - 2016 to 2021

May 2021 - Life is good!

Background - Carrie's BAV Discovered in Early 1990's

Carrie was born with BAV, but she did not know this until she volunteered to be a guinea pig for a new echocardiogram machine in the early 1990's. There it was - a two-leaflet aortic valve! The next year, after her sister's diagnosis included not just BAV but an ascending aortic aneurysm, Carrie had another echo which showed that her ascending aorta was enlarged also.  

Carrie already had begun her heart journey with the implantation of her first pacemaker in 1989, when she was only 29 years old. In the following years, her pacemaker journey alone has required multiple interventions due to infection, broken pacing leads, and device upgrades. In 2014, open heart surgery was required to address complications caused by pacemaker leads! Carrie had developed Superior Vena Cava (SVC) syndrome, which is obstruction of the blood flow though the superior vena cava. The cause was old pacemaker leads located there and scarring that had developed in this blood vessel over time. During surgery, Carrie's own pericardial tissue was used to enlarge her right atrium and patch her superior vena cava, and the abandoned pacemaker leads were removed.

Here, we focus especially on the most recent five years of Carrie's journey, from 2016 to the present. Carrie began experiencing chest pain/discomfort that year, causing her to seek out the expertise of the Cleveland Clinic. The remainder of this post are are in Carrie's voice, with paragraph captions added.

October 28, 2016 - Chest Pain Prompts a Visit to Cleveland

So off I went for evaluation at Cleveland Clinic.  I flew to Chicago where my little sister, Theresa, picked me up.  We then took an all-day road trip to Ohio.  We spent several days there.  I had an echocardiogram, a CT, labs and EKG.  Once all the tests were completed we met with a cardiologist as well as Dr. Eric Roselli, a cardiothoracic surgeon.  Dr. Roselli viewed the CT result with us, where we were able to view the actual scan.  My aorta was stable and they all felt that it was reasonable for me to continue on with the watch and wait approach.  The chest pain issue was still unsolved so I just had to back off on my running and switch to walking.

 So, over the next several years I kept up to date with my local cardiologist, where serial imaging of my aorta and bicuspid aortic valve remained relatively stable.  Chest discomfort remains unsolved.

March 2019 - Diving Competition, Bee Sting and  Breast Cancer

During a dock diving competition where my little dachshund was competing I was stung by a bee on my left wrist.  Initially the sting was painful but I continued on with the day just feeling tired.  Then I developed a large localized reaction which spread up past my elbow.  After seeing my primary care provider he instructed me that if the redness continued going up my arm that I should go to the ER.  I did end up going to the Emergency Room where I was given appropriate medications and steroids.  The “cellulitis” finally resolved.  It was also deemed that I am allergic to bee venom.

Bee sting reaction!

But something wasn’t right.  I just kept feeling like I had an ache in my left armpit area for a while…then I started feeling pain in my left breast. 

I was watching a nightly national news program one evening and they aired a segment about women with fibrous breast tissue should be screened with 3D mammography.  After seeing this and knowing my mother passed away after a battle with metastatic triple negative breast cancer…I called my PCP and asked if he would order this mammogram for me. 

 I went in for the mammogram the end of April.  The next day my doctor’s office called to tell me that I had a suspicious lesion in my left breast and that I will be scheduled for an ultrasound.   After the ultrasound my PCP called me to let me know I needed to have a biopsy. 

The biopsy was done on May 2^nd.  Four days later, I got the call…You have cancer!  My knees buckled and I felt sick and so frightened.  I got my wits together and went out to where my husband was working in our yard.  It was all so surreal.  Right up there with when I found out I had a brain aneurysm.  All of the results came back, and I indeed had the same cancer that my mother had.  Triple negative breast cancer.

Cancer Surgery and Chemo

There were many appointments and consultations, then finally I had surgery to remove both breasts June 24, 2019.  I spent a few days in the hospital and had the support of my husband, Vic, my sister Theresa and several of my dearest friends.  I actually felt pretty well and was optimistic as no cancer was found in my lymph nodes and the tumor wasn’t too large.  But it was still considered a high grade tumor and I had to be scheduled for 16 weeks of chemotherapy.   Prior to this I had to have a pre-chemo echo…where everything appeared stable.

After discussing everything with my oncologist and surgeon they agreed that I could take the RV trip we planned to our favorite park on the Metolius River in Oregon.  I was very grateful for this time to reflect and be with my husband and my sister by heart, Judi and her husband Greg.  We were also joined by two more dear friends one of which was a retired nurse.  I was in good hands and I felt the trip to be restorative.  

January 2020 - Last chemo treatment

August 5^th 2019     I received my first round of chemo.  Judi was with me for every infusion from then on.  I developed most of the nasty side effects.  Anemia, neutropenia, oral thrush, nausea and hair loss.  We had to postpone several treatments until my labs rebounded.  I also had to have several blood transfusions. 

I was pretty much exhausted most of the time but pushed myself to walk my dogs as much as possible.    In October I had another echo, and my cardiologist told my aortic valve was now mildly stenotic.  Fast forward to December and the aortic valve showed moderate stenosis.  I became more fatigued and short of breath and even after chemo ended in January of 2020 I still could not regain my stamina.  My cardiologist recommend that I work out but I could barely climb a flight of stairs.

 March 2020 - COVID, Fatigue, BAV Severe Stenosis, A New Door Opens!

The Covid 19 Pandemic lock down started.  I was supposed to have the tissue expanders removed and proceed with breast reconstructive surgery.  This procedure was deemed elective and my case was cancelled indefinitely.  I still felt short of breath, and fatigued along with exertional chest discomfort.  Not to mention how uncomfortable the tissue expanders were. Then in May my cardiologist ordered another echo.  My aortic valve stenosis was now severe.  I had mild swelling of my ankles and was becoming depressed and very frustrated. 

One day in August, my husband and I were driving down our road and stopped in to see our relatives that were out in their yard.  This was one of those divine intervention moments.   You see, this family member also has severe aortic stenosis!  Two of us, living on the same road, with the same problem. We talked for a while, and she told me that she had become discouraged with her local cardiologists and finally went to UC Davis in Sacramento, where she felt she receives superior attention.  I told her that I was also frustrated with my care and that my cardiologist of many years was set to retire.  I asked her to get me some contact information the next time she went.  A few days later she stopped by with the TAVR (Transcatheter Aortic Valve Replacement) Nurse coordinator’s card and said “she knows you”!  I looked at the name on the card and couldn’t believe that it was someone I used to work with in a cardiology office where I worked for many years.  She actually helped train me in pacemaker and ICD follow up….and we even had golfed together!  She was expecting my call!  I felt so relieved and thankful! 

 September 1^st 2020 - BAV Needs Replacing, Stable Aorta

Carrie's aorta 
2016 at Cleveland Clinic

Judi picked me up and off we went to UC Davis, where I had another echo and was evaluated by the cardiologist specializing in TAVR. I was also evaluated by a cardiothoracic surgeon and many medical students.

They all agreed that my aortic valve needed to be replaced, but they needed more time to go over the echocardiogram results and the recent CT scan.  Because I had previous open heart surgery for SVC syndrome and also have an ascending aortic aneurysm, I was considered high risk.

I felt very comfortable and confident in my new care team, knowing they would take time to make a treatment plan. Two days later I received a call from the TAVR nurse.  They determined that I was a candidate for TAVR and that my aorta was considered stable. 

 October 5, 2020 - TAVR in Native BAV

I had a transcatheter aortic valve replacement (TAVR) using an Edwards Lifesciences Bovine valve.  Everything went very well and I felt the difference immediately!  My physicians are optimistic that with my new valve, some of the pressure will be taken off of my ascending aorta.  We will continue to monitor my prosthetic valve along with my aneurysm. 

June 2021 - Blessed in the Face of Adversity

Today, I feel very well!  I was finally able to have my breast reconstructive surgery… also at UC Davis.  In fact, I have transferred all of my care to UCD.  My care has been amazing!  Yes the past few years have been a challenge…as they have for all…but again, I would not change a thing.

Feeling blessed in the face of adversity is truly a gift…and I will always be thankful for the bee and the cow that helped save my life! 

Carrie has been with us at BAF since our beginning days,

 and it is our privilege to walk with her always. 

Thank you, Carrie,

 for once again sharing your courage and strength with the world,

and through each experience,

 Creating a Climate of Hope

~Arlys Velebir

                    Bicuspid Aortic Foundation

Also see Carrie Mettler - A Wonderful Athlete with BAV   and 

Carrie in 2016 - Seeking Opinions for a Complex Situation

I Need to Find Somebody Who Cares

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

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

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

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

"I Need to Find Somebody That Cares"

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

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

A Day in April 2020 - Lost

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

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

Above all, Mathew needed "somebody that cares". 

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

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

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

A Day in March 2021 - Strength

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

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

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

How do we at BAF know this? Experience.

Mathew has done this, these recent months. 

Many others have done it too.

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

Moving from fear and confusion 

to accurate information,

being in touch with those who

 understand and care, 

Living our lives,

Creating a Climate of Hope,

~ Arlys Velebir

                        Bicuspid Aortic Foundation

Pursuing Answers in Memory of Those We Have Lost - Heart Month 2021

Red Jasper

 On this first day of February, designated as Heart Month 2021, at the Bicuspid Aortic Foundation we begin by remembering those we have lost, for it is in remembering them that we are confronted by one of the most compelling reasons we exist, the persistent, tragic loss of life, too often in the young. Despite advances in diagnosis and treatment, we continue to lose them. We begin by remembering some young men in their 30's.

Forever 33, Almost 34, in 2020

Michael Anthony Smith was born in August, 1986. Just under a week before his 34th birthday, in August 2020, he passed away in a health center in Gainesville, Florida. 

Michael's memorial page, including a video tribute, are located here. 

Those who visit the link above and watch the video will recognize a strong, active, vibrant young man. There are no words to adequately convey the tragedy of his loss, the pain and emptiness of those love him.  

Forever 33 in 2004, Forever 34 in 2012, Forever 30 in 2013

Over the years we have heard of other young men who succumbed unexpectedly to BAV/TAD complications. Doug Grieshop turned 33 the day before his death in 2004; an autopsy revealed a ruptured aorta and an undiagnosed BAV.

Chuck Doherty was 34 that day in 2012 when he collapsed in the driveway of his home as he returned from a run; Chuck's BAV and aortic stenosis had been diagnosed, but that was insufficient to protect him from sudden death.

Chad Rogers was 30 when he failed to return from his run on Monday evening, July 22, 2013. His body was not found until Friday afternoon. Chad's BAV was known since infancy, but similar to Chuck, his physical activity had not been restricted. 

Doug Grieshop's family shared his life initially here, and there is more in this follow up, Forever 33.

Chuck Doherty is remembered here.

An account of Chad Rogers' cause of death is here.

There are others, also young, also athletic, also suddenly taken, whose families have come to us after their loss. As I type this, I remember an autopsy report sent to BAF years ago; it described the examination of a healthy young man's body. He had dropped dead while playing, if I recall the sport correctly, volleyball with his friends. The report detailed his body, healthy and normal with just one exception. The only finding was a bicuspid aortic valve; despite CPR immediately, his heart could not be restarted, his death perhaps due to an abnormal heart rhythm. 

And so we begin Heart Month 2021 remembering this group of young men, healthy appearing, athletic, vibrant, often in their 30's. 

It is approaching 17 years since Doug Grieshop died, and in the intervening years other young men in their 30's have died also. How many, we do not know. How can we see beyond their glowing, healthy appearance and identify those at risk of a deadly event (aorta, aortic valve, arrhythmia) before they are prematurely taken, leaving loved ones to go on somehow, deprived of their gifts, energy, and  many contributions to our world?

In their memory, we pursue the answers,

in February 2021, 

Creating a Climate of Hope,

~ Arlys Velebir

Bicuspid Aortic Foundation