The Third Man

Thirty Years

As 2020 comes to a close, today I reflect on the experience of three men who each had aortic valve replacement surgery from a thirty year perspective.

What was it like for people who needed aortic valve replacement in 1990? 

Full open heart surgery was required to replace the aortic valve. Surgical replacement with an artificial heart valve had about a thirty year history then, dating back to roughly 1960. 

What kind of artificial valve would a given person receive? It seemed straight forward at that time. There were two general kinds of artificial heart valves: mechanical and tissue.

For younger people, with the exception of women who wished to become pregnant, a mechanical valve would be used, requiring anticoagulation (warfarin) to prevent blood clots and strokes. Taking warfarin required periodic blood tests. Warfarin patients were also given dietary restriction counseling.

Older people were offered tissue valves projected to last about 10 years, allowing them to avoid living with warfarin. 

Thirty Years Later, 2020

These three men all received mechanical aortic valves, valves that were intended to be a lifelong, durable solution for each of them. After healing from surgery, all three men resumed their lives.

Two of these men continue their lives with those original mechanical aortic valves. They have reached "old age" now and have developed other health problems. However, their original mechanical valves continue, just as intended, to open and close with each beat of their hearts.

The third man's experience turned out to be very different. 

The Third Man

The third man lived for 27 years following his first surgery. Between 1990 and 2017, he underwent a total of four open heart surgeries and experienced a major stroke due to valvular strands and pannus that had formed on that original mechanical aortic valve.

He lost his life following a tremendous fight with sepsis and persistent endocarditis due to resistant bacteria introduced into his blood stream at the time of the fourth open heart (third valve) surgery.

Was there something different about this man, the third man? Yes, he was born with a bicuspid aortic valve (BAV). 

How do I know so much about him? When I  met and married a man with a heart murmur, the future unknown to us then as it is for us all, I married the third man.

Much of what I have learned about BAV and thoracic aortic disease (TAD) is from what became our relentless pursuit of answers and help, as we faced together yet another battle, too often an ambush, when various complications confronted us. Far from being "fixed for life" as we happily believed when told this in 1990, we would become, out of necessity, readers of the medical literature, keepers of medical records, and searchers for answers along with the physicians and surgeon who bravely helped us.

We remained hopeful that each intervention would be the last, but over time our "heart journey" was much more like a convoluted roller coaster than the hoped for predictable path forward. 

My beloved husband's life ended that way, after months of precipitous ups and downs, with so many unanswered questions about the mysteries, the most obvious clue being that two-leaflet aortic valve replaced in 1990 and the large ascending aortic aneurysm "discovered" in 2001. There were other congenital "anomalies", somehow part of the package accompanying being born with a bicuspid aortic valve in this man. Eventually, I have recognized some of those things in others who also have been born with BAV.

2020: What has Changed?

It is three years now since this one man's last battle ended, but the fight continues for others living with BAV/TAD and their physicians. Many questions, that persist since at least the days of Abbott and Osler, remain today for pursuit by those in research.

Some born with BAV, like my husband, will take their physicians far beyond what is known, out on the frontier so to speak, where unexpected battles are fought and only sometimes won.

At this time, the big change regarding aortic valve disease is the availability of TAVR, which I first learned about in the 2005/2006 timeframe. My family and others I know have clearly benefited from this technological innovation. However, along with this new option come numerous questions to be carefully considered for those with aortic valve disease, perhaps most especially younger people born with BAV. 

What Will Another Thirty Years Bring?

In the context of 60 years of history, what does the future hold? 

In our family and so many others around the world, there are those with more severe, not readily apparent BAV/TAD variations. Given the prevalence of BAV in the population, their actual numbers likely are not small globally. What does the future hold for them? 

One of my greatest hopes for the future is that there will be the ability to proactively identify "the third man", those with complexities in their bodies that challenge the most skilled medical and surgical solutions. Yes, I continue to hope that in my lifetime, there will be greater understanding, more answers, and increasingly better treatment options than we have today. 

Best wishes for the New Year,

~ Arlys Velebir

Aortic Dissection - They Cannot Find What They're Not Looking For

Diseased aorta in the chest, before dissection/rupture
There is aneurysmal enlargement (root and ascending),
 abnornal lengthening(elongation)
 and curving (tortuous)

Disclaimer: This is my personal opinion only. It is information only, not medical advice, and does not represent the Bicuspid Aortic Foundation in any way.

I receive a daily alert on certain key words, including "aortic dissection". Recently, the headline on one of them was "Calif. Hospital Preserves Win in ER Patient Death Suit". Reading the published court proceedings, I was heart broken. A man was dead, sent home from an Emergency Room with an undetected aortic dissection, and the headline was about a hospital winning.... legal winning, yes, but in terms of human life, it was only loss.

When an aortic dissection is not found in life,

if there can be any so-called "winner" at all,

 it is the disease itself.

Why is aortic disease, in its most deadly state,

still not being diagnosed?

Through the courageous and persistent efforts of Jennifer Simon, following the failure to find aortic dissection in the ER where her husband Ken died, I heard about changes that were made and how others with aortic dissections were diagnosed through her efforts there.  Jennifer spoke about what happened to Ken here. As Jennifer says in the video, "They cannot find what they're not looking for".

Ken Simon died in Washington state in 2003.

 John Ritter died in southern California that same year.

They both made it to a hospital.

In June 2020, 

 I am reading about another man,
 who also made it to a hospital.

 His dissection was not found during the hours he spent there.
Why not?

Dissection was a word I first read in medical papers as I struggled to understand the aneurysm in my late husband's chest in 2001. It wasn't until September 2003, while driving one morning, that I heard a news report saying John Ritter had died of an aortic dissection. It was the first time I heard the word dissection used to inform the public. I remember exactly where I was when I heard it. That will soon be 17 years ago, and yet in June of 2020, I am reading an announcement of  a hospital winning (again) a lawsuit on appeal. A lawsuit about a man who came to their emergency room at 4:03 AM and was later discharged, leaving at 11:16 AM. When he left, he had less than 8 hours to live; not nearly long enough to follow discharge instructions to see his doctor and/or a cardiologist the next day.

At that moment, no one knew it, but aortic disease had already won.

All else was lost.

The opportunity to save a man's life was lost.

All hope of this man having emergency aortic surgery was lost.

Ultimately his life was lost.

His family lost precious time, their future, 

 with their husband and father.

The tearing of the aorta is the critical end state of disease in this blood vessel.

There is still hope for life to win, through surgery,

as long as the strong outer layer of the aorta holds together, 

but only if the dissection is found.

Reading the court proceedings, remembering my own visits to ER's with my late husband for various reasons, I recognize what was done: asking questions, taking vital signs, chest x-ray, EKG, multiple blood draws (including looking for the enzyme that indicates a heart attack, troponin). A heart attack likely would have been found, but that is not what was happening. No CT scan, no ultrasound tests were done. A cardiologist was not called. They did not find his torn aorta.

 As I recall reading the public information about John Ritter, he had a chest x-ray too, but not a CT scan. CT scanners were and are widely available, typically located very near the emergency department, since this test is both so fast and so useful. Among the many virtues of a CT scan, it makes a torn aorta visible to a radiologist.

Many years apart, in different hospitals, I suspect that both of these men were approached similarly in the emergency setting. They did not have heart attacks. Despite the many medical papers published, guidelines written, and efforts to raise awareness about aortic disease in the chest since 2003, many years later another man still wasn't given a chance to have surgery, a chance to at least fight, along with surgeons, for his life. 

The "Big Three" Killers in the Chest:

 Heart Attack, Aortic Dissection, Pulmonary Embolism

 Perhaps there are many reasons for the chest pain that causes someone to seek emergency care. However, medical literature states  there are only three immediately life-threatening conditions that cause chest pain: heart attack, aortic dissection, and pulmonary embolism.I do not understand why it would be hard to remember just three things. Perhaps it is not so much memory but focus that is the issue. If it's not a heart attack, why not check the aorta?

It is very hurtful for me to read, in the court record, the medical history of this man, documented from a prior hospitalization. He had a heart murmur. He had high blood pressure. He had a history of smoking. Even prior hernia surgery gets my attention.  There is no record of anyone ever connecting any of those dots regarding aortic disease. He was also described as being overweight and having high cholesterol. That morning in the ER he may have appeared to be a candidate for classic "heart disease" that is the leading cause of death. It is indeed important to find a heart attack, of that there is no doubt. When a heart attack was not found, what about the other deadly killers in the chest? What about the aorta?

The court document mentions his symptoms in the ER.  It is beyond sad to read what he was feeling as the chaos caused by  aortic dissection disrupted vital blood flow in his body. A radiologist's description of his chest x-ray is in the court document too, mentioning a mildly enlarged heart. Again, I wonder about his heart murmur. 

Hope 

I found some hope in a new paper available here (June 1, 2020) about a 65 year old woman who initially had chest and abdominal pain, but upon arrival at the emergency department, the pain was in her leg. She had an aortic dissection; the paper says her dissection was found, she was transferred to another hospital for surgery, and recovered well.

Despite successfully saving her, the writers (all listed as working in Emergency Medicine) discuss the focus on her leg pain initially, and the importance of the medical history, from both the family and the emergency medical personnel, so that important information, such as the original chest pain, is not missed. The use of ultrasound as an alternative to CT to find the dissection is also mentioned. I am encouraged by their focus on continuously learning and improving in this paper, so that more people may receive the correct diagnosis and are sent for surgery as quickly as possible. The passage of time is on the side of the disease, not the fight for life. 

In the ER with Chest Pain 

Writing this, Doug Grieshop is on my mind and in my heart. He is the first person, killed by aortic disease, that I came to know through his family after his death. Like so many with BAV, he was energetic and very active, and looked so vibrant and healthy on the outside. He was a loving husband and father, in the prime of life. You can meet him here: Doug Grieshop - Forever 33  Between the time he went to the ER with chest pain and the day he suddenly died, many months went by. There was time, missed opportunities to find his BAV/aortic disease and have surgery. He died almost 16 years ago, in September 2004. His children are teenagers now.

As Jennifer Simon said regarding what happened to Ken,"They cannot find what they're not looking for". Each of us may need to be the one that remembers our aorta, in the presence of chest symptoms:

When doctors say it's not a heart attack,

insist that they check your aorta!

For anyone interested, here is the recently published court document. Among other things, it sheds light on the legal view of a patient's written acknowledgement that a physician is a contractor, not an employee, of a hospital.

I close with this link to "Blowin in the Wind" performed by Peter, Paul and Mary, and these haunting words

"How many deaths will it take til he knows

 that too many people have died?

The answer my friend is blowin' in the wind.

The answer is blowin in the wind."

We no longer live in the days of the brilliant Osler and Abbott, when autopsies confirmed aortic disease diagnoses only after death. There is an answer to finding aortic dissection in the emergency room in 2020. Is the answer still just randomly blowing in the wind, some victims of dissection getting diagnosed and having surgery while others lose their chance to fight for life?

~  Arlys Velebir

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

Reflections on Life - Ordinary Days, Tubes, Pressure, Tears, and Ruptures

Any ordinary day can become a crisis in a just a moment of time. 
Recent research about the reason for weakness of the BAV aorta
reminded me of this experience with my car!

My weak, bulging, torn, ruptured radiator hose!

It was just like any other morning. I pulled into a parking spot and switched off the ignition - an ordinary motion, on an ordinary day. Immediately, to my shock, great white billows emerged from under the hood of my car! Getting out, I could tell it was steam rather than smoke - no fire at least, that was a relief!

Eventually the steam subsided, but not before attracting some attention, while I called my wonderful local mechanic and arranged to transport the car to his garage. When he arrived, the flatbed truck driver looked under the hood and pointed out the likely culprit, a hose. Riding in the cab of that huge flat bed truck, I wondered how bad it was. I knew one thing - I was extremely glad that it happened when it did, parked, and with the engine shut off. What if it had happened while driving in that rush hour traffic - not a safe place to break down?!

The human aorta has three layers - the outer one is very strong.
That strong outer layer can save lives when it holds on long
enough to get to surgery, after the inner layers tear.
I don't know how many layers of materials this hose had,
or how well designed it was to handle pressure. 

I left my car in excellent hands, knowing it would get a thorough check up. Later, the call came, confirming the problem. There was indeed a torn, ruptured main radiator hose. The hose had become weakened and bulged out over time (the clamped ends were still the normal size). I do not know why it chose that precise moment when I shut off the engine to tear and burst. Was there an extra surge of pressure just then? 

Diseased, Bulging Aortas are Dangerous!

When my "car doctor" called to tell me what had been found, it reminded me so much of the way aortic surgeons describe the diseased aorta in the chest - weakened and bulging! 

When the aortic tissue becomes weak and bulges, it is also prone to tearing and rupture. If it tears, causing bleeding in the wall of the aorta, it is called dissection. Quickly getting into surgery can save lives, as long as the outer aortic wall holds. When the aorta completely breaks open, the massive, uncontrolled bleeding is rapid and deadly.

Aortic aneurysm and bicuspid aortic valve

Check Your Hoses - Replace if Necessary!
Unfortunately, it is easier to replace the radiator hose on my car than it is to replace a weak section of aorta inside the chest. However, it can be and is safely done! The best time to do it, like it should have been for my car's radiator hose, is before it tears or breaks. 

Torn and completely ruptured, radiator fluid was soon all gone
When the aorta ruptures, it is blood that escapes rapidly,
 until life can no longer be supported.

So have that CT scan or MRI and follow the size of the aorta! Have it measured and interpreted by expert eyes. Then have open, frank discussion with your doctor about when to replace that most vital of "hoses", the ascending aorta in the chest.

I am forever grateful that the aneurysm in my husband's chest was found, medicine was prescribed to lower the pressure on his weak, bulging tissue, and it was safely and expertly removed before it could hurt him. There were other things - aortic valve failures and infection - that did hurt him, but he never had to experience aortic dissection or rupture. Too many others have suffered this aortic tragedy. Let us hope that the recently published research, Defusing the cardiac time bomb, will truly usher in the dawn of a better day for those with BAV and aneurysm.

Best wishes to all,

Arlys Velebir

More About the Infection Fight - Little Green Caps and Lines in Your Blood Stream (PICC)

It seems I have opened up my Pandora's box of memories, and several things have come tumbling out. 

One of them is my fight for little green caps!!

I will share it here. I hope it helps someone.





I was in a hospital room in LA in February, and there they were. Little green caps! Not spending a lot of time in hospitals, most of us don't fully appreciate some of the things in that setting. I know I didn't. I learned though! Seeing those green caps again brought our life with PICC lines back to me.

When it was clear he would need ongoing administration of vancomycin to fight the infection in his blood and heart, we were told it could be done through a PICC line, at home. (PICC stands for Peripherally Inserted Central Catheter.)

I hated the thought of putting a long plastic tube into his arm and threading it up until the tip was near his heart. I believed he got the methicillin resistant staph epidermidis (MRSE) that way in the first place, through a line put into his blood stream. (Yes, I am finally writing the name, unmasking the monster that eventually killed him.)

For long term medicine delivery into the blood, there was no other way. And vancomycin has to go into the blood - it just cannot be taken by mouth. In addition, the blood levels of vancomycin need to be kept within a certain range in order to be effective. (That struggle is a story for another day!!)

The supplies were delivered by a pharmacy that specializes in PICC line drugs and the "equipment" that goes with it. They deliver directly to the home.

I learned about the little green caps from the first home health nurse we met. In the hospital, they are mandatory. However, what I found out is that the pharmacy may try to provide less than the best to you at home!!! It should not be allowed, especially when you are fighting a killer.

It is important to have a clean, sterile "tip" or port that you connect to when giving medicine, or any other reason the PICC is being accessed. After all, one is trying to fight germs, not introduce more!

In hospitals, they use the little green caps to keep the port sterile. At home, the pharmacy may try to just provide alcohol swabs - no doubt they are cheaper!

Can you believe it? Yes, maybe you can. Saying I was unhappy when I found this out would be an understatement.

I asked the nurse to just order the little green caps, and I remember distinctly what she said. "If I ask, they will tell me that the alcohol swab is sufficient. But if you call and ask, they will send it."

I called!

They had to make a special delivery, just for those green caps. I told them several times, whenever supplies were sent, that we must have the green caps. As I remember, they eventually realized I would not relent, and began automatically sending them.

Just so you know why hospitals use them, the difference they make is well proven:

Use of Disinfection Cap to Reduce Central-Line–Associated Bloodstream Infection and Blood Culture Contamination Among Hematology–Oncology Patients

Here is a page from 3M with information about them

If you are going home with a PICC line and there are unused little green caps in your hospital room, take them with you. You paid for them. And make sure the pharmacy sends them to you, every time.

Someone told me that these caps were invented by the parent of a child with infection. I have not been able to verify if that is the case or not. I am just grateful for them.

I call them "little" green caps. There really are no little things when it comes to infection. Everything matters in the fight for life.

As a reminder, these are my personal experiences and opinions, and not connected in any way to the  Bicuspid Aortic Foundation.

Best wishes to all who read this,

Arlys Velebir

Fire in the Blood, Fire in the Heart - Sepsis and Endocarditis

I miss our walks together on the beach. It can never be again. 

Now I must find my path forward, alone. What should that path be?

In this second year, I am beginning to understand that I should face some of the things that happened to us during that last fight for his life. 

Indirectly it was artificial heart valve issues, but directly, it was sepsis and endocarditis that took him from me. For so long I focused on heart valves and aneurysms. Then infectious demons tortured him and eventually took him from me.

Perhaps these reflections, still almost too painful for me, will lead me to my next step. No doubt, there is is still a fight to wage. Infection is a vicious killer. Surely, we can do better than what happened to him . . . .  

There is a nonprofit, Committee to Reduce Infection Deaths, that among other things provides vital information to help the public regarding hospital-acquired infections, including practical advice for anyone going into the hospital. 

Disclaimer: This is my personal blog, and in no way reflects the position of the Bicuspid Aortic Foundation.




Two years ago at this time, we were so happy together, thinking we were recovering successfully once again following open heart surgery - the fourth one. I remember it now as a blissful interlude, as serene as the beautiful Pacific on a calm day. Suddenly, it would explode into disaster.


Something is Terribly Wrong

Recovering at home after surgery, he began to feel a little off. I noted a rash on his face. I will not share all the details here, but it was the beginning of going into Septic Shock. Overnight, he got extremely ill. By the time I found an ambulance that would transport him back to the hospital where he had surgery, he was conscious but not much more. I sat at his head in the ambulance, thinking I might lose him on that endless journey on the freeway.

We made it to the hospital.

The ER doctor and nurses leaped into action. His kidneys were completely shut down, his body terribly acidic. When they unsuccessfully attempted to insert a central line, I heard his scream of pain through the walls to the far side of the ER waiting room where I sat. I have never seen such horrific suffering.

He was in septic shock, but it was never called that while he was there. They were unable to culture any bacteria from his blood. I remember one nurse telling me that "sepsis patients present like this". She was right.

Along with other things, he was treated with antibiotics as a precaution. Amazingly, his kidneys and general health recovered. Unappreciated, the vancomycin was key to his improvement. We went home again, still without any explanation. 

Mercifully, later he had no memory of the worst of that episode. I remember. Over and over he told me, "I can't go on."  I told him then that he did not need to go on, to fight anymore. I loved him too much to ask him to stay in that ravaged body. Slowly the suffering diminished. I am thankful we had more time, although it was at a very high price to him.

Finally, A Diagnosis

Returning home again, we arranged follow up care with an excellent, caring local doctor. First he developed terrible shoulder pain, the first sign of the resurgence of the infection, although we did not know it. In the doctor's office one afternoon, he had a soaring fever and the tremors we first had seen right after surgery. We were sent directly to the ER of our local hospital, where blood was drawn to culture, and he was admitted and again vancomycin was started.

Fire in his Blood, Fire in his Heart

This time, the blood cultures revealed an antibiotic-resistant bacteria. Vancoymcin was continued, to battle it again, this time knowing it was there. Yes there was a bacterial "fire" in his blood, coursing through his body, potentially contaminating and damaging everywhere it went.

The bacteria was methicillin resistant staphylococcus epidermidis, which is found on the skin.

This villainous bacteria is among those that do not fight fair, not at all. They are particularly attracted to artificial devices in the body. They secrete a kind of slime that is difficult for drugs to penetrate, and embed in the nooks and crannies that are hard to reach. His brand new bovine aortic valve was like a magnet to them.

They did an echo at his bedside in the hospital. When I heard that the new aortic valve showed some damage, I fled his hospital room to cry alone in anguish. A beautiful new bovine aortic valve which he had risked so much to have was being destroyed by infection. I had feared endocarditis all these years. Sometimes our fears do come true.

Why Call This Fire?

I refer to this infection as fire because of how destructive it is, and because of the tremendous  inflammation it causes in the body. 

Over time, even after the septic crisis, many immune markers in his blood were so high they were "off the charts".  If he had lived, chronic joint pain would have remained with him, damage from that "hot" immune response within. This strong man began using a walker to steady himself when the stabbing pains in his low back and hips would strike.

Acquired at the Time of Surgery

The first hours and days after surgery had been difficult, but we did not know why. He told me later that he knew something was terribly wrong the moment he woke up. He felt instinctively he might not make it.

We noticed an involuntary tremor in his shoulder and arm that came and went. Why? Three prior open hearts had never been like this one.

In agony, in ICU he told me he wanted to go to heaven. So soon after successful surgery, I begged him to fight, to stay with me. We had love, prayer, special songs of praise and thankfulness to sing softly, and the comfort of scripture. Having those things, we had everything. It was the doctors who did not have everything they needed, to prevent, to understand, to treat.

After some rough days and nights in ICU, the suffering and tremors subsided. Unknown to anyone, it was the vancomycin given as a precaution following surgery that was working on the bacteria in his bloodstream. As it began to work, he improved. And so, we went home.

Yes, today, I know what happened. An antibiotic resistant bacteria had entered his blood stream at the time of his fourth heart surgery. When? It was present when he awoke in ICU. How? The infection was in his bloodstream, pointing to someone's actions introducing this deadly bacteria through one of the lines inserted directly into his bloodstream. From there it traveled the nutrient-rich super highway of his blood, causing trouble as it would, but particularly attracted to that beautiful new bovine aortic valve.

In ICU after surgery, the anesthesiologist came in and found something not to his liking with one of the lines. I remember the nurse resisting doing anything about it, using an excuse that the line would soon come out, but he insisted that it be corrected immediately.

 Another nurse present made the comment to me that they were "learning something new" from the doctor. I recall that now with cynicism. After all, would they admit to me if they had done something less than properly sterile? Of course not.

Was that how the bacteria got into his blood? There is no way to know, to prove anything after the fact, but I do not forget. He entered the hospital with clean blood. He left with killer bacteria in his blood.

Fighting with Antibiotics - What Could Medicine Do?

After all this, another surgery to change the aortic valve and clean out the infection was too dangerous for him. The fourth surgery had been high risk in and of itself. At this stage in his journey, he would not survive another open heart.

Ultimately, over months we went to the local ER for admission a total of three times. There were PIC lines at home, nurses coming and going. Blood cultures that would be clean, and then later show the infection was present again.

Reviewing what happened, there was a pattern. After about 10 to 14 days without the antibiotics, the bacteria would take control to the point it was obvious, he was sick again. A full body scan showed the infection source was the aortic valve in his heart. The antibiotic had been able to destroy it every place else.

Medicine and Infectious Disease

I was shocked to read that vancomycin is a very old drug. It was the only thing that worked at all. It is the drug of last resort. What happens when and if it does not work either?

After starting with vancomycin, one infectious disease doctor tried a different drug, easier on the kidneys but with less success against the bacteria. How much time and ground was lost trying it, only to return to vancomycin?!

Finally, after he was admitted yet again for another PIC line to deliver further antibiotic treatment, suddenly in the afternoon his pulse dropped dramatically. The infection had "taken out" the AV node near his aortic valve. After that, the only reason he was alive at all was because of a "trickle" electrical impulse getting through to the left ventricle, telling it to squeeze. I thank God for that design, because it kept his heart beating long enough for him to return home for his last hours, his dearest wish.

I still find it strange that on Monday morning, as we were arranging to take him home, an infectious disease doctor I had never seen came in and spoke of trying different drugs. Where had he been all this time? Hadn't he read the chart or spoken with our doctor first?  Why put us through the anguish of that conversation? We had already made the decision to go home and cherish our last hours together. And that is what we did.

Playing with Fire?

Endocarditis has been known to attack those born with BAV for a very long time (I love your published work so long ago, Dr. Abbott! I wish they would quote you more often!) . When surgery and artificial heart valves came along, endocarditis was recognized as a risk with artificial valves.

Today, there is a debate about whether or not antibiotics should be given when certain invasive procedures are done that may introduce bacteria into the blood stream. At one time, antibiotics were given to those with BAV. Currently the guidance is to use antibiotics only for those with artificial heart valves.

This greatly troubles me. I recently read this paper.  Risk and outcomes of aortic valve endocarditis among patients with bicuspid and tricuspid aortic valves  

From the paper, I have bolded some words (IE is infective endocarditis):

"This study does not provide guidance on the use of antibiotics in patients with BAV. However, we did observe a very high risk of IE in patients with BAV, thus establishing their potential value as a target population for randomised trials of prophylactic antibiotics in procedures prone to bacteraemia. The recent change in guidelines was largely motivated by the attempt to provide antibiotic prophylaxis to the highest-risk patients undergoing the highest-risk procedures. Although to this day, there is no randomised controlled study that tested the efficacy of antibiotic prophylaxis, BAV clearly increases the risk of IE in the otherwise healthy patient population. Our study suggests re-examination of congenital valvular heart disease among risk groups but does not provide any information regarding prophylactic administration of antibiotics prior to at-risk procedures. Patients with BAV should be encouraged to report symptoms of IE early to potentially reduce the risk of aortic root abscess. This study also highlights the risk of IE from intravenous drug use in patients with BAV."

What would I personally do if I had a bicuspid aortic valve?   I would talk to my dentists and doctors, frankly and honestly, and ask for preventive antibiotics before procedures.  I would never wish to "play with fire".  I have seen the destruction, when infection does its worst. It is relentless, right to the last breath.

Infection and the Future
 Are we doomed to return to the days when bacterial infections killed so many? Where is the next generation of drugs, that will defeat these aggressive bacteria? These are questions I ponder. Perhaps that is part of my path forward, focusing on those in search of the answers.

Gazing at the Sunset

Until now it has been too painful for me to think about, to remember, these things. It hurts to face the reality that this deadly bacteria present on someone's skin killed him, as expressed by one of his doctors, "because someone didn't wash their hands." Perhaps writing this is the next step in my personal journey forward, advocating for those attacked by infections.

Now, it is time for me to stop gazing at the sunset. 

Just beyond the horizon is tomorrow, an opportunity to make a difference.

 

Originally posted in 2019, I have updated and republished this again on 04/05/2021.

Best wishes to all who read this,

Arlys Velebir

Speaking Up about BAV - Find Your Voice!

Red Jasper
The "warrior" stone

I spent many years, from 2001 onward, reading and questioning, devouring everything I could find about those born with bicuspid aortic valves.  Initially it was trying to understand how someone supposedly "fixed for life" by aortic valve replacement surgery 11 years earlier had somehow developed a large ascending aortic aneurysm.

The reading and questioning no doubt made a difference. I know we avoided some pitfalls. Others ensnared us, diminishing our quality of life.

Fluoroquinolone Warning
Today I am moved to write and encourage BAV individuals and families to find their voice and speak with their doctors about medications after reading the December 20, 2018 FDA warning about fluoroquinolone drugs. The following is from the announcement:

 "Health care professionals should avoid prescribing fluoroquinolone antibiotics to patients who have an aortic aneurysm or are at risk for an aortic aneurysm, such as patients with peripheral atherosclerotic vascular diseases, hypertension, certain genetic conditions such as Marfan syndrome and Ehlers-Danlos syndrome, and elderly patients.  Prescribe fluoroquinolones to these patients only when no other treatment options are available.  Advise all patients to seek immediate medical treatment for any symptoms associated with aortic aneurysm. "

The large population (1 in 50) of those with BAV, known to be vulnerable to aortic aneurysm in the chest, is not listed as one of the patient examples. This leaves something for us, the patient and family members, to do. We can speak up and mention it to our doctors.

From reading, I knew that the medical literature had begun to report on this class of drugs and the risk to the aorta. Two different times, with two different doctors, I told them about this and asked that an antibiotic from a different group of drugs be given to my husband. The doctors were not aware of the information in the literature, but they listened and found another option. This was well before there were any official warnings. I will always be glad we avoided any potential complications - he already had more than his share of issues without anything more.

Blood Pressure Medications
Recently letters have come from a pharmacy addressed to my late husband, warning of contamination of blood pressure medications he had been taking. He is still in the pharmacy database. I was aware of this long before those letters arrived and would have taken steps to protect him if he were still alive. 

Ultimately, I lost him to a raging infection in his heart. I cannot begin to tell you how much it still hurts. I can tell you that it would hurt even more if we had not done what we could all those years to protect him.

I often say that those with BAV are warriors. For those still in the battle, please read, ask questions, and speak up for yourselves. It could make all the difference in the quality and length of your life! 

With very best wishes

 for peace and joy 

this holiday season,

 and a safe, healthy 2019

~Arlys Velebir