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

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

Aneurysm - A Medical Term

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

We do not want anyone to experience an aortic emergency, 

we do not want to lose anyone else,

which is why there needs to be more awareness. 

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

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

Thank you for walking with me, 

Remembering those we have lost,

Others who were saved ,

As together, we create a Climate of Hope,

~ Arlys Velebir

                          Bicuspid Aortic Foundation

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

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

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

1966 - A hint of what is to come

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

1976 - Emerging labile hypertension

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

1981-1986 - Premature deliveries of my children

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

1998 – No worries!

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

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

2000 – Searching for answers

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

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

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

2002 – Watching and waiting

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

 2013 – Time for Surgery!

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

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

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

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

2016 - The honeymoon is over so soon???

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

2017 – Just “lazy”!!

2017 revealed further immobility of the “new” valve.

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

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

 2018 – Denial can be dangerous!

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

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

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

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

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

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

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

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

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

I can breathe!

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

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

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

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

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

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

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

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

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

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

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

He was very encouraging …

He gifted me with these words….

"Go and Live!…Go to the Beach!…

Travel!…Have Fun!…Live your Life!...

Happy Life!…Happy Heart!!!”

From one BAVer/TAD to another:

Unlike me,

please  be prompt to share your symptoms

 with your trusted physician

 or confidant

 and

 resist the temptation of DENIAL….

May your stars align on your BAV or TAD journey,

 all the days of your life!

~ BJ Sanders

TAD Awareness 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