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

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

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

Broken Hearts this February - Remembering Bill Paxton and Those Who Grieve

Red Jasper
The Warriors' Stone

In February . . .

as children, we gave valentines to our classmates.

growing up, we learned about "Heart Month" - a time to focus on heart disease.

feeling invincible, we found our very own heart-mate, our one and only valentine.

later, we learned about BAVs and aneurysms, and wondered why we had never heard of it.

one went into the OR and the other waited as our heart-mate had open heart surgery.

some remained in the fight to overcome BAV and aneurysm.

And, in February, some lost their heart battle.

Yes, February is still about valentines, chocolate and flowers.
For some, it may be the month their hearts were saved.
And, for some, this month our hearts are broken.

Bill Paxton

We were almost ready to leave the hospital a year ago, following my husband's third aortic valve and fourth open heart surgery, when Bill Paxton's death became public. He should have had what all BAVers should have, a successful surgery, his first. What could have possibly gone wrong?

When someone with BAV is lost, we all lose. 

I did not know Bill Paxton, but there are things about him I know well. Like my husband he had rheumatic fever as a child. They also were both born with BAV and over time an aortic aneurysm developed. Like the majority with BAV, he was high energy and gifted, described here by co-star Mary Kay Place.

Video clips show him as the youthful picture of health, almost too perfect, that is typical of BAVers. Others may not notice this detail, seen in some BAVers, but in his smile I see the slight gap in his front teeth. My husband's gap was more pronounced.

If it hurts me to look at video clips of this vibrant man, what indescribable grief Bill Paxton's family must be living through now, the time of the one year anniversary of his surgery and then 11 days, one by one, until his death.

It is extremely painful to read the law suit just filed by his wife and children, grieving the loss of their husband and father. The words take us into a place of horror, with no escape for Bill and his family. 

The Right Hands, Not the Right Buildings

There is no time we are more vulnerable than when signing the papers, giving our hearts, our lives, into the hands of a surgeon and all the others involved. There are up to 100 "Does" listed in this lawsuit, their names unknown at this time. 

Having been through 4 open hearts with my husband, two of them in that hospital, I can easily picture the physical setting. How much of what is written in the lawsuit can be proven in a court of law? I do not know. I do know what is alleged is indescribably horrifying and tragic.

It reminds me of what I heard once - that buildings don't provide care, people do. The absolute greatest challenge for those with BAV is to find, not the right buildings, but the right people to undertand us, to save and extend our lives.

It is a challenge that my own family and other dear friends continue to face.

My February Memories

Memories may not comfort those who grieve at all. They haunt us. I have my own February memories, including two open heart surgeries in this month. We fought for about 8 months after that last February surgery before losing the battle. 

Others tell me the "firsts" are the hardest. I cannot tell you whether this is the case. It is too soon.

I can tell you that this is my first February without my husband, and I am among those with a broken heart. 

This February

may I rise beyond

 the grief and sadness,

and be

more understanding,

more empathetic,

and more caring.

                    ~ Arlys Velebir

.

Reasonable Hope - Infection and Heart Sounds

His nurse was listening to his lungs recently. It was a relief to hear her say the lung sounds were normal. And then she said, "You have a heart murmur though." 

It was not the first time, of course, we have heard those words. My husband has lived with them from childhood. Over the years, there have been surgeries to address that murmuring aortic valve. Three of them. (A fourth surgery addressed an ascending aortic aneurysm.) The murmur would improve, then worsen again as the man-made solutions, mechanical and tissue, failed over time and in different ways.

Recent echo with DeAnn Paul, who has expertly
imaged his heart for many years. There are videos of her explaining echo's
on the Bicuspid Aortic Foundation YouTube channel.

 And so, here we are again, despite so many efforts to have a working aortic valve. We know why the bovine aortic valve, just a few months old, is murmuring now. There is infection in his heart, caused by bacteria acquired in a hospital.

Courage, In the Presence of Fear

I had watched the latest echocardiogram, heard what his doctor said. Somehow, though, when his nurse said "heart murmur", the all-too-familiar icy fingers of fear grabbed my own heart once more. It is the cost, those moments of fear, of loving and caring for someone complicated, someone who was born with a bicuspid aortic valve. It is at such times we find within us the depths of love and courage we need, in spite of the fear. Perhaps those depths are never realized, except in experiences such as these.

Vancomycin - Packaged for home use

Reasonable Hope 

One of his doctors used these words recently, "reasonable hope".  Hope is a special word, and this time it needed to be qualified as "reasonable". What is this reasonable, medical hope? The hope is that a long series of vancomycin treatments will help him.

Several weeks of another drug, daptomycin, failed to clear the infection. In the struggle to understand and treat this, at least there is no debate about that drug's failure. The recent echo clearly showed infection still present.

This leaves only the drug vancomycin, administered intravenously also, through a catheter called a PICC line. That in itself is not without risk, another catheter dwelling in his veins, ending near his heart.

 A high enough dose of "vanco", maintained for a long enough time, is what we pursue today.  The treatment is somewhat complex. The drug has significant side effects, and the level in the blood must be measured to insure there is enough present to kill the bacteria.

We can do this! 

Hospital Acquired Infection

This infection is the result of bacterial contamination of his blood, most likely through one of the catheters inserted into his veins at the time of his surgery.  This unwelcome bacterial invader  took time to clearly reveal itself, however. It nearly cost his life more than once. Who knew that sepsis and then later endocarditis can be so difficult to diagnose, at least in my husband?

The Cleveland Clinic has established an Endocarditis Center. There are two videos at this link where doctors describe their approach, along with text explaining about infection in the heart. 

This article, also from the Cleveland Clinic, sheds some light on infections in the heart: Infective endocarditis


A War with Many Battles

There is so much still not understood about the bodies of those born with abnormal aortic valves. It leaves them vulnerable to aortic stenosis, aortic insufficiency. aortic aneurysm, aortic dissection/rupture, and endocarditis. My husband has had all of those things except dissection/rupture. In addition, valvular strands on his mechanical valve caused a stroke.

His aneurysm was discovered and "disarmed" in time, before it could tear or fully rupture. We thought that was the last big battle. It was not. There have been many since.

This is a lifelong war with many battles. He remains in the fight.

The Struggle: BAV, Medicine, and Mystery

Medicine -  what is it, really?

According to Merriam-Webster online, one meaning is  "the science and art dealing with the maintenance of health and the prevention, alleviation, or cure of disease".

I have my own definition, based on years of walking beside my husband as well as others with bicuspid aortic valve (BAV) and thoracic aortic disease (TAD). Here it is:

"Medicine is the struggle to discover the secrets, to solve the mysteries, and unmask the disease processes in the human body, in the hope of preventing or relieving injury and suffering."

Having spent a great deal of time with doctors and hospitals again these last months, the struggle is very real. For some like my husband, and his doctors, it is a lifelong struggle, a lifelong fight. Where, oh where, are the answers?

Hasn't Modern Medicine Solved the Mysteries?

When we are healthy, reports about the latest discoveries and breakthroughs, often described in glowing, wondrous words, can give the impression that much of human disease and suffering has been solved and eliminated. As we learn about BAV, we learn there is treatment for it. Later we may learn it is rather generic, one-size-fits-all treatment. Sadly, those with BAV may too often find that their experience, their bodies, are not understood. There are no wonders, no miracles, for the most complex among us in their time of need. Instead, there are many questions, many challenges.

The Challenge of the Unknown

I remember talking once with a young pre-med student about this. He was volunteering in a major medical center, where his eyes were opened to the many unknowns in aortic disease in the chest. We both agreed that day that there is still so much, too much, about aortic disease that is unknown. He is learning to be a surgeon now. I hope and believe he will always remember what he learned then, that there are many challenges awaiting and needing his skills - the challenge of the unknown.

Vulnerable, Fearful, Uncomfortable 

For about 16 years, I have been trying to understand what being born with BAV means for my husband and others like him. I am extremely uncomfortable with not knowing, because it means there may continue to be surprises - unpleasant, ugly surprises that bring suffering, pain, and potentially untimely death.

Treat the Symptoms, Save the Life, but What about the Cause?

I have written before about being told that with the mechanical aortic valve implanted in his heart in 1990, my husband was "fixed for life". Since then, there have been more heart surgeries (a total of four!), a major stroke, and now infection in his blood stream (sepsis) and heart (endocarditis). In hindsight, it is clear how little has been understood about this one man, born with a bicuspid aortic valve.

Imagine multiplying that across every 1 in 50 people, across the entire world. The issues are massive.

There is a great deal of uncertainty in medicine.

This is why I call it a struggle. 

When It Doesn't Make Sense 

Over a decade ago, a man born with BAV had a stroke, just a few hours after his ascending aneurysm surgery. I will call him Matt. The doctors looked for the explanation, but there was no obvious reason for it. The stroke was relatively mild, and Matt went forward and recovered well.

Matt had the same mechanical valve and the same aortic aneurysm surgery as my husband. I always remembered that he had a stroke that "didn't make sense". Why did Matt have a stroke? Everyone moved on, the question not answered.

Sometime later, a shower of particles injured multiple areas of my husband's right brain. Locally, the doctors had no explanation for it. It was another stroke that "didn't make sense". We persisted in seeking the reason, and this time we found the answer through a test called TEE (transesophageal echo). There were still strands of tissue, hanging off the mechanical valve, floating in the blood
stream. There was no doubt that some strands had already broken off and gone to his brain. (There were a few medical papers, very few, that supported this.) We had found the answer! It was important to tell others!

I told Matt and his wife that we had an explanation for my husband's stroke, in case it might apply to him. It did! Matt also had strands on his mechanical valve. Reviewing images from the time of his surgery, the strands were there but not noticed, not understood. Strands were the cause of his stroke right after surgery. Matt had the valve with its strands removed before it could injure him further.

If the reason for Matt's stroke had been found sooner,

 it could have protected others, including my husband, 

as well as Matt himself.

Unanswered questions are dangerous.

After my husband, strands on other valves were found by his surgeon, but always after a stroke. I had always wished that the strands could be found before they hurt the brain. My wish was granted when Father Prodromos had aneurysm surgery, and the strands on his mechanical valve were found and addressed before they could hurt him!

Understanding why

makes all the difference!

Prevention of injury
and providing proper treatment 
depend on it! 

Somewhere, out on the horizon,
there are answers for those with BAV!
We just need to find them!

My husband continues to experience "fall out"/complications following his most recent surgery. Among the biggest challenges has been to make his body give up its secrets, to fully understand and fully treat what has been happening inside him. 

Who Will Unravel the Mystery?
Researchers at Northwestern University and the University of Alberta have been awarded funds to work on the mystery of BAV:  3 Million Dollar Grant Brings Precision to Heart Patients

 Answers cannot come too soon. I wish these researchers well and look forward to their work changing in a positive way the lives of those with BAV.

After centuries of pain, suffering, and death,

may this be the time when BAV remains a mystery no longer.

Emergency Again!

A Lifelong Journey

The drive to this local hospital remains unchanged. It's ownership and name have changed with time, but we are very thankful that the small hospital nearest to us, where my husband was first hospitalized 27 years ago, is still there for us today. 

A beautiful natural setting, this hospital overlooks the Pacific. 

His first echocardiogram, later his first CT scan, were done here. We had no idea, so long ago, that important battles in my husband's fight to live would need to be fought here once again in 2017.

When we made the decision to have surgery once again at the end of February, our hope was to have a recovery similar to his other surgeries. Some things have been different with each surgery. 

No doubt the easiest and best recovery was in 2001, just 16 years ago now. We were admitted to the hospital on Memorial Day. This surgery removed his ascending aortic aneurysm, removing the danger of aortic dissection or rupture. He was strong, healthy and discharged on the third day after surgery. He healed well, and never looked back!

 Recovery from his first surgery, in 1990, and this last one have been the most difficult.
 Both times, he was in varying degrees of heart failure.

 His surgeon tells us his tissue is very fragile now. I truly hope that doctors will recognize the connective tissue aspects of those with BAV that genetics
 are beginning to reveal.
  It would help so much in understanding the whole person.

We repeat history, returning  to the same Emergency Room decades later.

Fever

After what we call the "kidney crisis" at the end of March, we returned home with hope in our hearts that the worst was behind us. After only about a week, he developed shoulder pain, which gradually became worse and reached the point where we were working on seeking help. Before that could happen, he spiked a fever!

Walking through the ER door once again, 27 years later, with a fever.

Fortunately, by this time we had met with a local physician who is very familiar with his heart issues and had arranged for him to take my husband as a patient. That day, we had an appointment just as the fever peaked. We were sent directly from his office to our local hospital, where the battle was fought.

The parking area also remains unchanged.

He was admitted to the hospital and started on powerful IV antibiotics.

I found comfort in the words of his doctor, "It may take some time, but we will get to the bottom of this."

Sepsis

He had been treated with powerful, IV antibiotics immediately that are known to be most effective, while waiting for blood culture results. Then one day we were told the something was growing in the blood cultures!

Sepsis is bacterial infection in the blood. The blood should be sterile, no bacteria should be there. It is to be especially feared in someone born with BAV or who has an artificial heart valve. They are magnets for bacteria!

I had horrifying visions of his new bovine valve, just two months old, being damaged by some vicious bacteria. If the bacteria should settle on his valve or tissue lining his heart, the infection would be called endocarditis. This should always be feared. They had told us this 27 years ago, with that first prosthetic valve. It was a known risk long ago for those born with BAV, long before there were antibiotics. It remains a danger today for native BAVs as well as artificial valves. What is worse, in today's world many antibiotics no longer work against bacteria. Infection can indeed be a deadly killer once again.

Echocardiogram - Is the New Aortic Valve All Right?

My husband had paid a high price already to receive a new aortic valve, first the surgery and recovery, followed by the kidney crisis. It was unbearable to think that the valve might be damaged and fail all over again.

When his doctor reviewed the first echocardiogram done at his bedside, it did not show the aortic valve clearly enough. The next day something very special happened at my husband's bedside. His cardiologist personally came, along with his echosonographer (who has become our dear friend, looking out for his heart for so many years). Together they persisted until his aortic valve was clearly seen.

I will never forget them, and what they did for us that day. Only the most dedicated, the most compassionate, do such things. I wish every person could receive that kind of care.

Initially it appeared that the new aortic valve might have developed a significant leak. I felt my own heart breaking. This was among the lowest points in our recent battles. In my moments alone, I cried over that bovine valve, still so new and possibly already damaged. It seemed too much, that he would lose the function of the valve he had braved so much to have.

After more review and discussion with his surgeon, it was decided that the new valve was not damaged after all. We could breathe again!

Final Results

At last the bacteria from the blood culture had developed well enough to determine what it was. He was to continue the IV antibiotic, a PICC line was inserted, and we were allowed to go home with assistance from home health!

As we returned home, it was to see this lovely planting, a reminder of endurance over many years.

This plant, a gift at the time of his first surgery,
faithfully blossoms year after year.
It is a gentle reminder
to keep hope
 in our hearts,
always!

Surgery Number Four - The Longest Day

The heart is a Red Jasper stone,
 known as the "warrior stone"

 We had been asked to come to the hospital early, and we were right on time. The clock said 5 AM sharp as we signed in at the front desk. It was the beginning of the longest day, his fourth surgery. . . .

I was prepared for this day to be a long one, especially from that moment when we parted at the "Kissing Door". 

After 27 years, some memories of his first surgery day have faded, but some never will. I remember the shock I felt, going in to see him in "recovery" in the afternoon. Later, I was there when  he awakened from the anesthesia with a great commotion of alarms blaring at 6 pm that evening. As soon as he opened his eyes, we connected again, although he could not speak yet. This is the most wonderful moment for me, when I feel we communicate again - we don't need words!

Until this recent surgery, that first one had been the longest - the longest period of time when I felt separated from him. The elapsed time of the two surgeries that followed (aneurysm in 2001, replacement of mechanical aortic valve in 2006) were shorter, and he woke from anesthesia quickly.

Why so long?

Why was this this surgery going to take so long? There are two main reasons: 1) scar tissue and 2) the delicacy of my husband's own tissue. I have been convinced for some time that in our family as well as many others, BAV is a sign of something that involves the body's tissue more broadly, not just the aortic valve and aorta.

1) Scar Tissue

After a first heart surgery (and he had 3 prior!), the body forms scar tissue, not just on the outside of the chest where we can see it, but on the inside. This scarring has to be gently and carefully navigated.  "Rise of the Redo" , an article from Royal Brompton and Harefield hospitals in the UK, describes the challenges.

2) Delicate Tissue

It was the next morning, in ICU, before my husband was even allowed to wake up and breathe on his own, that the surgeon told me about how very delicate his tissue is inside now. It had been 11 years since these same hands had last operated on my husband.  Now, his tissue generally is so much more fragile, only the gentlest touch would not damage him! In the wrong hands, this surgery would have been a disaster. 

Many BAVers, including my family members, have delicate tissue that manifests in various ways, and it seems to become more so with time.

A few weeks ago now, I heard Professor Mona Nemer describe her work with BAV mice families. I was thrilled to hear her say that the genetic defects in these mice, so like their human counter parts, are defects of the tissue. BAV has not been called a "connective" or other kind of tissue defect historically. Professor Nemer's work is vital to looking beyond a malformed aortic valve alone, and accurately characterizing those with BAV.

Progress, One Step at a Time 

The most lovely liaison nurse went into surgery periodically and then updated me on the progress through out the day. It was just after noon when she told me that there was lots of scar tissue, but the old valve was out! 

It was great progress, and I felt relief flow through me for a moment. Then I realized that he had no aortic valve at all now, until a new one was safely anchored in place! 

It took the remainder of the day to place a new valve, slowly rewarm him, and close the chest. The surgeon came out to tell me about the surgery around 6 pm.

I will write separately about what the surgeon said about the old valve. What was it really like? How well had the echocardiograms and CTA tests prior to surgery portrayed what was happening inside my husband's chest? Learning from this is extremely important, given that these tests, along with symptoms, help guide the decision to have surgery or to wait longer. I will just say now, the tests and symptoms were accurate enough for us understand that waiting was not an option for my husband, if he was to have this surgery at all. 

More Waiting 

Yes, surgery was over, but my husband would not be allowed to wake up as quickly as he had with his past surgeries. Before surgery, his right heart had been overworked by the failing bovine valve, and the long surgery time had also been hard on it. He was to remain sedated while his right heart recovered. Thankfully, his left heart, the main pumping chamber, was strong! 

It was a long night, thinking about that right heart. By 8 the next morning, I was told that his right heart had completely recovered during the night. Soon, he was awake and then breathing all on his own once again!

Family Friendly Care 

I was allowed to remain with my husband in ICU from that very first night, which was wonderful! This is generally not the case in ICU's. We would spend a total of four nights there. I remember that first night, looking at all the technology surrounding his bed, lights glowing in the darkened room. It had a surreal quality, the feeling of another world, another universe.

Not everyone may find that they can do this. All the machines, the tubes, and the sight of their loved one can be more than they can bear. This hospital is new, 'state of the art", and the nurses so supportive, that I was indeed comfortable there. I remember the warm blankets brought for me to  rest under, and the hot tea given to me at 3 am that first morning. These are beautiful touches of care, not to be forgotten, in the midst of all that technology. A century ago, those kind of comforts were all that could be done for the patients themselves - none of the drugs, the surgical and intensive care expertise and equipment, existed.

The Heart of a Warrior,
Healing Once Again,
27 Years after His First Battle

Post Surgery Day 16 - Recovery at Home

Today is the 16th day following the day of surgery. With the attentive care of his surgeon and a home health nurse coming in, I am comfortable caring for him as he recovers. We just need to stay vigilant lest any complications should develop. From our own family and others, we know how nasty some of these complications can be! 

Overall the hospital was a great place to be as long as he needed that level of care, but there is just no place like home for recovery! 

I am not a nurse, and I admired many of the things nurses did for him in the hospital. However, I do tell my dear husband that no one else could ever put so much love into his care!