TAD Awareness Walk with BJ Sanders - 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 Ascending Aortic 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

The 11th Day - Reflections on BAV and the Fight for Life

 ICU at daybreak, on the first morning after surgery.
My husband was still sedated and on the respirator.
I was allowed to be with him,
resting on a padded bench beneath the window,
 all through the night.

                   

Today, March 10th, is the 11th day since my husband's fourth heart surgery.

Bill Paxton died on the 11th day after his first heart surgery, on February 25th.

Based on press reports, Bill Paxton was born with BAV and had an aneurysm. He also had rheumatic fever in his youth. 

My husband was born with BAV, had an aneurysm, and had rheumatic fever as a child.


We made the decision to fight one more time, have one more surgery, on February 21st. It had seemed unthinkable, that roughly 27 years after the first surgery, no longer young or even middle aged, my husband would have surgery again. We decided to fight this, one more time. This was a very personal decision, made with his surgeon, who knew his own skill as well as his patient, and above all, with the help of God.

Today, my husband's body fights to heal, one step at a time. All is going well, one step at a time. I am so blessed to be with him, every step of this fight. We are in a different kind of fight, but the words of a Navy SEAL come to mind:

 "There's a storm inside of us, a burning river, a drive.

You push yourself further than anyone could think possible.

 You are never out of the fight." 

- Marcus Luttrell

Our deepest sympathy, beyond words to express,

 to all those who mourn the loss of Bill Paxton.

 Our deepest sympathy also, to those we may not know about,

 who mourn the loss of their loved ones,

in the BAV battle for life.

~ Arlys Velebir

Remembering the Unknown Bicuspid on Fifteen Year Anniversary of Aneurysm Removal, May 30th

In 2001, May 30th was a Wednesday. Early that morning, my husband had surgery to remove the dangerous bulge above his heart. It is a day to remember with thankfulness.

Fifteen years ago, the Memorial Day holiday was two days earlier, on May 28th. We had agreed to do something unusual that day: check into a hospital.
My husband had stopped the coumadin (warfarin) required for his mechanical aortic valve, so that his blood clotting would return to normal. He was to check into the hospital at noon on Memorial Day, the 28th, where he would go on a heparin drip to prevent clots from forming and also have the typical pre-surgery tests.

Memories

We each have our own memories of that experience, but one thing is certain. Today, we remember and remain very thankful for the safe and complete removal of that aneurysm, up to and underneath part of his arch. It is a difficult surgery, and was done flawlessly. Fifteen years later, the repair and the aorta itself remain excellent.

Today, we are most grateful to his own gifted surgeon. We also understand that the pioneering work of aortic surgeons laid the foundation upon which his surgery was possible. The surgery my husband had that day remains perhaps the most complex in the chest, involving completely stopping blood flow at cold temperatures. Only some surgeons have this skill. We still marvel that we found such hands.

The Unknown Bicuspid

There is someone else that I remember today. I do not know his name. His identity is lost, except perhaps to his family. I have searched the medical papers, to see if anything might have been written during that era, that might be about him. I cannot find it.

Today I remember him because, like my husband and many others, one day he went into the hospital to have BAV surgery.

He never came back. He died from a torn aorta. 

It happened sometime after his valve surgery was over, during that first night. The doctors covering that night saw his kidneys weren't working. They did not know why. They did not know his aorta was torn, bleeding inside. The blood flow was cut off from his kidneys, so they could not work. By the time, the next morning, someone figured it out, it was too late. He could not be saved.

Lying there, perhaps the respirator tube still down his throat, helpless, his aorta ripping apart in his chest. It is a horrendous scene. I hope the drugs were strong enough to block the pain.  I hope he did not suffer.

It could have happened to my husband. His records show that his aorta was bulging at the time his BAV was removed. Somehow his aorta withstood the clamps, the cutting and sewing, when his BAV was removed. I don't know why it did.

There was a young surgeon in training then, who saw this happen. He never forgot it. From that day forward, he was to save many of those with BAV from a similar fate. Years later, he removed the aneurysm from my husband's chest on May 30th, 2001.

And so today, I remember this young man, and others like him. They are the Unknown Bicuspids. Some of them died of massive aortic bleeding, sometimes under the mistaken label of a massive heart attack. Some of them died because of complications from their BAV.

Today I remember their lives, cut short fighting aortic disease. 

Yes, we remember these unknown, who lost their fight much too soon. In their memory, those like my husband continue to live, to fight. They are all heart warriors. They have their own battles to fight.

Best wishes to all,

~ Arlys Velebir

Hope for the New Year

 Hope for the New Year

Amaryllis  Fantastica

Special Holidays This Year

This year when asked about plans for the holidays,

 I said that I had very special plans indeed! 

What were they? 

I was spending them with my husband.

Only those who can never do that again, this year or any other, truly understand how special that is. Their lives have been forever changed by the loss of their loved one to bicuspid aortic valve disease  My thoughts go to them, in many states and around the world. How much they would give for just one more moment together.

Flowers for the Holidays - An Impulse Buy

It was an impulse buy. While shopping in November I saw these square boxes with gorgeous flowers pictured on the outside. Amaryllis. I remembered my Mom having this plant around the holidays one year. It had seemed rather exotic to me. I do not have her green thumb and had no idea how much she coddled and coaxed it to produce those huge, gorgeous flowers. But, how I would love to enjoy those beautiful blooms once again!

The instructions seemed simple, and the picture on the box charmed me. Hoping I was not consigning it to a terrible fate, into my shopping cart it went.

The box promised blooms in about 6 weeks. If I started right away, there might be flowers for the holidays. Inside the box were just three things: a rather plain pot, a compact ball of soil, and a dead looking bulb. I followed the instructions. The soil did expand in the pot when water was added, as promised, and into it I placed the bulb.

At first, not much happened. I tried to keep the soil moist but not too wet. But I wondered if this dead looking bulb, partly exposed above the soil, could ever come to life, let alone produce something beautiful.

Then one day, I noticed the bulb was not brown any more. It had a green color to it. Life! Hope came alive in me too.

I'm sorry I don't have pictures of those early changes. I suppose I still did not have very much hope. But then something started to shoot up from the top of the bulb. Slender, green leaves! It was still a long way from beautiful flowers, but I found more hope stirring in my heart.

Maybe it would do better in a different location, with a bit more December sun. I moved it, watched it, and tried to keep the soil moist without drowning it! It was alive, that was clear, but I doubted there would be anything special happening any time soon.

Wonders Over Night

 Then, in just three days, wonders happened!

December 29th

On December 29th the outer casing popped open, with hints of the beauty within. December 30th was an amazing day!

December 30th

December 30th

Just Spending Time Together
And so, we have these holidays now, special, uninterrupted moments together. I find myself clinging to them, wanting to slow the passing of time. Soon they will be over.

The Amaryllis has so beautifully rewarded my rather doubtful efforts, producing beautiful flowers for the new year. In this new year, check ups await him once again. How is his prosthetic aortic valve doing? It needs to be checked soon. Last year, we learned it is not holding up as well as we would like. How much has it changed? His mitral valve tends to leak, but has remained unchanged for several years. He is to have a follow up with an eye specialist, too. We need to make sure nothing can rob him of his vision. Oh, the delicate tissue, the possible complications, for someone born with BAV.

This new year holds heart and aortic check ups for so many. Some face serious decisions about when and where to have surgery. Too often, good answers are hard to find.There are no labels, no names, other than bicuspid aortic valve or thoracic aortic disease, for many with complex issues through out their bodies. Quite simply, too little is known, too little understood.

I cannot bear to think about those so apparently healthy, so talented and active, who are unaware that they are in danger from their BAV or aneurysm. We need ways to find them before tragedy strikes.

May the new year be a fresh start in search of answers.

These are my thoughts on the eve of the New Year. And then, the New Year arrives!

January 1, 2016
Two fully open flowers, two buds

January 2, 2016

The Third Bud Begins to Open
January 2, 2016

 Something in Common - An Amaryllis and BAV??

Watching it change and bloom, I find this amazing plant has reminded me of the BAV experience. After long periods of quietness, BAV may change so quickly, so dramatically.  Doctors call it "latent". One interesting definition of that word is "hidden" or "dormant". For many years, BAV may not express itself. Then, often very quickly, there is important change. A valve needs surgery, an aneurysm is dangerously bulging. My husband's experience has been like that.

It is so hard for those who know, who must watch and wait. Like my Amaryllis bulb, it seems nothing is likely to happen. But then it does. Often then, things move quickly. It is so easy to be in denial.

Can I see the beauty, find the wonder, as life unfolds?

January 2, 2016

My husband has had three surgeries. When he had aneurysm surgery in 2001, I stayed in a nearby hotel until he was out of ICU, when I could be with him at night. That high risk time, right after surgery, is not easy. In that room was a single rose bud. Each evening when I returned to rest, it had opened a little more. Like my husband's recovery, it unfolded beautifully. I am glad I have that memory of beauty and hope from that time. The surgery was a marvelous success. Something I experienced almost 15 years ago still remains as a comforting memory. Today, the Amaryllis reminds me again that I can find beauty, even in the dark times. Beauty unfolds through the help and kindness of others, the repair of the broken, the miracle of healing.

Another Flower Stem Has Appeared
Hope for the Future
January 1, 2016

Yes, new experiences lie before us in this new year. I remind myself, whatever the future holds, we can do this! One step at a time, we can do this.

We can have those check ups, ask our questions, get multiple opinions. We can make decisions.

Along the way, I will look for the beauty in each day. 

So much awaits us all. May the beauty in the promise of help and healing sustain us. Let us focus on hope.


With all best wishes in this new year,

                          -  Arlys Velebir