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A loving husband and father, a strong young man, was struck down in his prime. Charles "Chuck" Doherty was just 34 years old when he lost his life without warning. Why? Chuck was among the children born in 1977 with a bicuspid aortic valve. He died from complications related to that valve following what was for him an ordinary eight mile run.
You can read more about him and his family here, at the Charles A. Doherty Memorial Foundation. Those of you reading this who have a bicuspid aortic valve yourselves will recognize that you share some things with him - his love of athletics and activity in general are a common theme in those with BAV.
Reading about him, we would all have loved to know Chuck. We can never meet him now. In a moment of time, he was taken.
There is something we can do. We can tell his story, and remind doctors that valvular heart disease is not just a risk to the elderly. It can take the young and the strong.
We can also raise funds that can be applied to research. There are questions that need answers. Do those with bicuspid aortic valves need special guidelines for monitoring and intervention for their BAV? After all, those with BAV are extraordinarily active and athletic. Given their unusual endurance and strength, could at least some of them follow a different path than their tri-leaflet counterparts? Could their first obvious symptom be sudden death?
Chuck Doherty lost his own life, but through those who love him, he will save the lives of others. Let's join them in the quest to Conquer Aortic Disease. Register for the race, or donate here.
Perhaps the "Big Three" dangers for someone born with BAV are
bicuspid aortic valve stenosis, regurgitation, or both
aortic aneurysm which may lead to tearing (dissection) or rupture
endocarditis - infection of the heart
While some may avoid all of them, others may find themselves in a fight with one, two, or all three at various points in their life. It is well to remember that as we admire their athletic abilities and glowing, healthy appearance.
Why Fund Valve Research? To Save Lives
Sudden death is a risk for someone with a diseased aortic valve - whether that valve has stenosis (narrowing), regurgitation (leaking), or both.
Chuck Doherty
Chuck Doherty, BAV Stenosis
Chuck Doherty, age 34, died suddenly at the end of a routine 8 mile run. His BAV had been diagnosed. Despite being under medical care, Chuck lost his life. Why? Were they waiting for symptoms that never happened? Did someone fail to appreciate the severity of Chuck's stenosis, especially in someone so young? Where is the information that would help a young man and his doctors make the right choices, so that Chuck would still be alive today? Questions without answers.
Chad Rogers, BAV Regurgitation
How could someone like Chad, diagnosed with BAV as a baby, suddenly lose his life at age 30? No, it was not an aneurysm that tore or ruptured. It was his bicuspid aortic valve itself that led to his sudden death.
His family has told us that Chad was very active in both cross country running and baseball in high school. The last few years, he ran marathons, completing about 12 full marathons and 3 half marathons. Over all those years, he had no restrictions on his physical activity. He had no obvious symptoms and appeared in good health until the day he died. Why? Why did this happen? How can it be prevented?
What does the future hold for those whose first surgeries are at young ages? How many surgeries will they ultimately face? Can we find better valve solutions for them? There are good choices today, but a replacement solution as good as their own valve should have been still eludes us. Can we find that "ideal" valve solution?
Other BAV Complications
My husband's BAV was replaced in his late 40's. It saved his life then. There was no recognition that he might be subject to complications because he was born with BAV. Fifteen years later, the valve that saved his life was the source of a terrible injury to him. No one understands why his body formed not only scar tissue but fine strands on that mechanical valve. Some of the strands broke off one day, showered his right brain with particles, and changed his life forever.
I hope haring these stories has helped explain why the Bicuspid Aortic Foundation has chosen to fund research of the bicuspid aortic valve itself.
Your donation in any amount can help us make a difference in BAV lives everywhere. You can donate on our special page, Take the BAV Challenge, or by mailing a check to the Bicuspid Aortic Foundation. Here is our mailing address:
Bicuspid Aortic Foundation
30100 Town Center Drive, Suite O-299
Laguna Niguel, CA 92677
We are an entirely volunteer organization, and 100% of all funds donated for valve research will go directly to that effort.
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.
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.
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?
If you want to find someone with BAV (bicuspid aortic valve), head out to a sporting event or go where there is some form of physical activity. Exercise seems to draw them like a magnet, and they are exceptionally good at whatever their favorite sport or activity may be!
Derek Owens, who currently plays
for the University of Hawaii, Hilo
Brent Warren hitting a homer!
Whether on the basketball court or the baseball diamond, these athletes play with all their hearts!
Along with team sports, over the years we have heard from many others.... runners, swimmers, bicyclists, rock climbers, wrestlers, body builders, triathletes... even race car drivers!
The challenge we face with BAV is keeping these vibrant, gifted people safe, because some of those sports enthusiasts we can never meet in person. We heard from their families when they died suddenly, without an opportunity to have lifesaving surgery.
BAVers of all ages
remain very active!
BAV triathlete
At the Foundation, we talk about comprehensive, individualized care. We believe that if BAV individuals and families received that kind of care, it could greatly minimize the risk of untimely death or injury. Given their extraordinary gifts and physical strength, it is very possible that those with BAV could exceed the average lifespan of the general population. However, they are at risk of some very serious complications: endocarditis (infection), aortic valve failure (stenosis/narrowing, regurgitation/leaking, or both), thoracic aortic aneurysm, thoracic aortic dissection, and in some instances brain aneurysm. There is a great deal still not known, and untimely injury and death continue to occur in the United States and around the world, despite the aid of modern technology.
Chuck Doherty Running
Marathon in 2010
One of those who tragically lost his life, cut down in his prime due to BAV complications, is Chuck Doherty. On September 15th, a 5K Run is being held in his memory. There is still time to register for the run, come out and cheer on the runners, or simply donate in Chuck's memory at the 5K for Conquering Aortic Disease page.
We are very grateful for donations and support that will help keep the hearts of those with BAV beating and healthy! There are physicians in centers who need our financial support to conduct research. Visit the BAF website to find out how you can donate to the cause.
The anxiously anticipated final strategic vision research topics from the National Heart Lung and Blood Institute (NHLBI)have been announced. Last year the Bicuspid Aortic Foundation participated in the invitation from the NHLBI for public input into strategic research topics for the next 10 years. Preliminary topics were announced previously. We have now been notified of the final decisions.
At the Bicuspid Aortic Foundation we are very pleased to see that two selections of particular importance to us remain in the final topics for research funding. Below, they are taken directly from the research section of the NHLBI strategic vision website:
Critical Challenge
In patients with an aortic aneurysm, better tools are needed to determine which patient phenotypes and disease characteristics could best predict who would benefit from a repair. Examples of such tools include animal models that reflect human pathology and biomarkers/molecular imaging tools that are predictive of rupture or dissection.(4.CC.02)
Yes, this is a critical challenge. Sadly, at BAF we know of far too many examples of trauma and death associated with the thoracic aorta. Those who should have surgery before they dissected or ruptured.
Compelling Question
What is the optimal clinical management approach for patients with severe calcific aortic stenosis but with minimal symptoms?(5.CQ.09)
This question must be answered, and it is within reach to do so. It is beyond description to lose the otherwise healthy who have BAV and diagnosed aortic valve disease. This topic speaks directly to what happened to Chuck Doherty. Chuck's family is not the only one we have met who have suffered sudden loss due to this. We would like to see aortic regurgitation addressed also, but this is a start!
In both of these areas, the aortic valve and the aorta, there is treatment available today. It is beyond tragic when that treatment is not offered in time. Those who are granted the privilege of funding to do this research will receive a great trust, to positively impact the timing of elective treatment and prevent untimely injury, suffering, and death.
At the Bicuspid Aortic Foundation, we will be very interested to see where the research funding is granted and will continue to advocate for meaningful research and treatment options for those with BAV and other "nameless" forms of aortic disease in the chest.
We simply cannot tolerate, cannot bear the tremendous ache with every injury, every death in BAV families. Yes, their trileaflet aortic valve family members also are at risk. These knowledge gaps are not small things.
Those pictured here speak to us of major complications that may strike down even the young and strong - the aortic valve itself, aortic dissection, aortic rupture, and infection (endocarditis).
To remind us all of the tremendous problem, and our shared responsibility to conquer this centuries-old monster, this blog has adopted "Blowin' in the Wind" as our theme song.
But in aortic disease, there are no perfect numbers.
Just 2 millimeters more
How big is 2 millimeters? It is the thickness of a US dollar coin. Looking at it, one wonders how such a small distance could make so much difference. For one man, it was the difference between having surgery or not. His aorta was 5.8 cm. The doctors told him surgery is done at 6.0 cm. The difference - just 2 mm - the thickness of a coin.
When "6" was the Number
For Louie Lopez, this tiny length became a distance too far, a point never reached. He was in the hospital, talking with the surgeon, when his aorta tore and fully ruptured. Massive bleeding, massive damage. Rushed to surgery, he had no chance. The year was 1995. Yes, "6" was "the number" for aortic aneurysm surgery back then.
Not long ago, Terry Lopez talked about that time, almost 20 years ago, when she lost her husband, Louie. "They were waiting for just 2 mm more. Just 2 mm....surely, 20 years later they can do better than that."
Reading papers, watching medical conference videos from thought leaders in thoracic aortic disease, I marvel at the paucity of answers, the preponderance of uncertainty still, as they continue to debate aneurysm size and the timing of surgery. There is still more unknown than known. Aortic disease remains a more than worthy opponent for those who do battle with it.
The Numbers
Physicians look for numbers to guide them in making decisions about aortic disease. For aneurysms, the most prominent number is the size (diameter) of the aneurysm. There is a great deal of debate about the number, the size when surgery should be done. Some argue to protect from what can be serious complications of surgery, urging that patients wait for the aorta to grow larger. Others promote earlier surgery, at smaller sizes, to protect from life-threatening, deadly events.
The following paper from 2013 is an example of the debate, giving pro and con perspectives from physicians around the globe, Germany, the US, and Australia:
In this paper, it is noted that very few patients were studied (with differing underlying causes of aortic disease), in coming up with the number for surgery, although used to guide the care of millions. "The number" has generally been coming down over the years as surgery became safer, but the uncertainty of what the number should be remains.
Some individuals suffer aortic tearing, perhaps death, at smaller sizes. On the other hand, surgery has risk, and offering surgery at smaller sizes may injure someone whose aorta would not have torn or ruptured if allowed to grow larger.
The size of the aneurysm alone is just not enough. Other measures, other ways to understand, are needed, but not available today.
If there were "perfect numbers" for the aorta, our friend Richard would not have dissected at about the same age and the same aneurysm size as my husband (who did not dissect, but had preventive surgery at 5.2 cm). Richard barely survived and has paid a high price in additional surgery because of his original dissection 10 years ago.
For the narrowed aortic valve, aortic stenosis, there are also numbers: the size of the valve opening and the pressure build up. There are measures for the leaking aortic valve also. Are they perfect numbers, that each individual can trust with their life? Is there a promise that nothing bad will happen before you reach the "guideline" numbers? Nothing bad will happen until you have obvious symptoms?
If there were "perfect numbers" for aortic valves, Chuck Doherty would still be with his family. So would Chad Rogers.
Guidelines, Not Guarantees
Those with BAV and other forms of aortic disease need to understand there are no guarantees, no solid promises. There are guidelines based on information available today. To our surprise we may find them quite limited, once we understand what they are based upon. As a patient, you need to research for yourself, understanding as much as you can.
Making Decisions
Part of that research can be searching for and choosing physicians/surgeons with a philosophy and approach to risk that is in agreement with your own. There are things to think about, such as your lifestyle, frequency of foreign travel, and general comfort with the risk of surgery versus the risk of waiting. These decisions should be individualized within the context of guidelines and statistics; decisions made in conjunction with physicians whose philosophy and approach to life and risk are in harmony with your own. Physicians who clearly share their outcome statistics with you.
Handling Uncertainty, Owning Decisions
In 2013, Aortic Valve and Ascending Aorta Guidelines for Management and Quality Measures were published. Including references, there are 66 pages. In the summary on page 54 are these words,"The choice of the best procedure or valve is dependent on many factors as discussed above and no procedure or device is ideal. Ultimately it is up to the patient, the cardiologist, and surgeon to reach a decision on appropriate treatment."
As these guidelines tell us, it is very important that you, the patient, are a partner in these decisions, and that you understand the basis on which you and your physicians together are making these decisions.
Wouldn't it be wonderful if there were perfect numbers, perfect devices, and simple decision making? Yes, but as in much in life, that is rarely the case. There are pros and cons, trade offs, in most decisions that must be made. But we only have one heart, one aorta, so these are very important decisions.
The more you know, the more you understand the pros and cons, you can make decisions that belong to you, that you can own. Not knowing leaves us vulnerable, caught by surprise. Perhaps many of us have already been there, rudely discovering the imperfections and limitations regarding aortic valve options.
Over 20 years ago, the mechanical valve that saved my husband's life was called a lifetime solution. The night I walked into our home and found my fallen husband, terribly injured by that valve and its complications, I began to understand the terrible depths of untruth in that "promise" that we had happily once believed. I will never forget him saying to me, "I did everything I was supposed to do."
If you understand there are no "perfect numbers", no "ideal" devices and solutions, it will at least help you to be proactive, to question, to research, to seek more opinions when things don't make sense, and to partner with your physicians in the decisions that are made.
And when something does not feel right, when an echo or other test results do not make sense, don't let it go. Keep asking questions, seeking answers.
This is written in remembrance of Julie . . . a wife, mother, grandmother, and so much more... I am calling her Julie here, not her real name, to preserve her privacy and that of her family.
She passed away suddenly, unexpectedly, about a month ago, on a day in early January 2022, not very long after saying goodbye to her own fragile, elderly mother in December.
She had no inkling that her own life would not be such a long one, that it would end suddenly that January day. Seemingly neither did anyone else.
Her husband of over forty years was right there with her when her heart stopped, never to beat again despite all efforts. His love for her and their family enabled him, despite the tremendous shock and grief, to obtain the best private autopsy available.
Among the findings listed, the pathologist examining her heart found her severely calcified stenotic bicuspid aortic valve and the enlarged left ventricle that had worked so hard to push her blood out to her body.
No one, not Julie, not any of the physicians she had ever seen, knew this about her.
There is one mention in her medical records, in 2018, of a heart murmur - seemingly heard just that once. No echocardiogram was done.
Other than being a "little tired" sometimes, so easily dismissed, there were no symptoms that might have prompted further investigation of her heart.
The autopsy report includes advice to her family to be screened since other blood relatives may be affected - a lifesaving gift to this family from their dearly beloved mother and courageous father, who must carry on without his soulmate. Along with Julie's BAV AS (aortic stenosis), all of the findings the autopsy reveals will help her family understand as much as possible about what caused her sudden death that day.
It is not the first time I have spoken with someone shocked and grief-stricken by the sudden death of their loved one, where the bicuspid aortic valve, not the aorta is implicated. Sometimes, as in Chuck Doherty's Sudden Death, Age 34, the presence of a bicuspid aortic valve was known to them and their physicians, but for others like Julie and her family, it remained unknown until after death.
May these deaths inspire us all to continue pressing for diagnosis in the living and greater understanding of those with BAV.
This is for everyone who loves someone with Bicuspid Aortic Valve or any form of aortic disease in the chest.
Derek Owens' parents were told about his BAV at birth
He was 16 when he first had surgery
This is especially for Mothers. Mothers of all ages. Right from the start, they know how special, how talented their child is. At some point, they may learn there is something not quite right inside the heart of their beautiful child. They agonize through the doctor visits, the surgeries. Some Mothers hear those most dreadful words. Their beloved child is gone.Rest assured, there is nothing you could have done to prevent a bicuspid aortic valve. Above all, you give your child what no physician ever can. You give them your love. It is the most wonderful treatment in the world, and you can increase the dose at any time without harm. Yes, it is the greatest healer there is. Your love. Unlimited. Always.
Following are the words of a childrens' book by Nancy Tillman. The pictures are from BAV families.
Wherever
You Are, My Love Will Find You
By Nancy Tillman
I
wanted you more
than you ever will know,
so I
sent love to follow
wherever you go.
It's
high as you wish it. It’s quick as an elf.
You'll
never outgrow it...
it stretches itself!
So
climb any mountain...
BJ Sanders at Machu Pich
climb
up to the sky!
My
love will find you.
BJ's dear Mother, who lovingly
follows her daughter's adventures
My
love can fly!
BJ celebrating her birthday in 2014. She had surgery a year earlier.
Make
a big splash! Go out on a limb!
My
love will find you. My love can swim!
Scott Nichols (in blue) was lost to aortic dissection, January 2014
It
never gets lost, never fades, never ends...
if
you're working...
or
playing...
or
sitting with friends.
You
can dance 'til you're dizzy...
paint
'til you're blue...
There's
no place, not one,
that
my love can't find you.
Derek Owens has always loved basketball!
And
if someday you're lonely,
or someday you're sad,
or
strike out at baseball,
or
think you've been bad...
just
lift up your face,
Derek in high school
feel the wind in your hair.
Derek Owens and his Mom, Laura
That's
me, my sweet baby, my love is right there.
Chuck Doherty lost his life in April 2012
due to BAV complications
In
the green of the grass... in the smell of the sea...
in
the clouds floating by... at the top of a tree...
in
the sound crickets make at the end of the day...
“You
are loved. You are loved. You are loved,” they all say.
Carrie Mettler running a half marathon
Carrie Mettler's beloved Mother, who knew the reality of
having two daughters with BAV
My
love is so high, and so wide and
so deep, it's always right there, even
when you're asleep.
So
hold your head high
and don't be afraid
Bob Gies following his surgery in 2010.
to
march to the front
of your own parade.
Doug Grieshop on his wedding day
Doug Grieshop at 2 months
If
you're still my small babe
or you're all grown,
my
promise to you
is you're never alone.
Doug had an undiagnosed BAV. He lost his life to aortic aneurysm
rupture 10 years ago, on September 20, 2004.
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