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

.

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!

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

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

                   

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

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

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

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


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

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

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

You push yourself further than anyone could think possible.

 You are never out of the fight." 

- Marcus Luttrell

Our deepest sympathy, beyond words to express,

 to all those who mourn the loss of Bill Paxton.

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

 who mourn the loss of their loved ones,

in the BAV battle for life.

~ Arlys Velebir

Discovery of an Aneurysm - Keeping Hope in Our Hearts

Our beloved African Grey Timneh parrot, "P.T."

Although my ancestry is from Africa, I was hatched in America, never knowing my bird parents. I was almost two before I met my human Mom and Dad. I had been returned to the same bird store that I left as a baby, when I went to live with a teenage girl and her family. Things just did not work out there. It was a disaster for me. The best thing that happened was when I was returned to the bird store. But I still had hope that one day I would find my forever family and go home with them. After all, stores are not places to live. So when Mom and Dad walked through the door one day, there I was! It was love at first sight. I am twenty years old now. Dad's first heart surgery was before I was even hatched, but I remember the time they found an aneurysm in his chest. I want to tell you from my own experience and my Dad's, keep hope in your heart always. Your dreams will come true.

                              - P. T. (Peter Timneh) Velebir

Hope is a thing with feathers

That perches in the soul

And sings the tune

Without the words

And never stops at all

                                      - Emily Dickinson

Fear

Aneurysm. It is just a word. Dictionaries tell us this word comes from the Greek, and means dilated or to dilate. For so many, including me, this word brings fear. When a blood vessel weakens and bulges, it is a condition to be respected and not to be ignored.Without treatment, it may create uncontrolled bleeding in the body if it tears or ruptures.

In my heart I remember a country cemetery, just outside a small town. Years before I met my husband, I said goodbye to a dear friend there. She was a young wife, the mother of a baby boy. Her life ended suddenly when a brain aneurysm ruptured and she was gone, snatched away in the midst of her happy, busy life. I have never forgotten her little boy, held in his father's arms, crying for his mother. He was far too young to understand what we as adults could scarcely comprehend. His beautiful, loving mother was gone, never to hold him, never to comfort him again.

For many years that is what aneurysm meant to me. It was a word tinged with icy terror and dread. A word without hope. A killer. So when I heard that my husband had an aneurysm in his chest, icy fear, real terror, gripped my heart. I didn't even know there could be aneurysms in the chest. 

Eleven years earlier, I had almost lost him to his heart valve complications. Now I thought I would lose him for sure. But not without a fight! Was there anything that could be done? If there was, we would do it, no matter where we might have to go to find help. 

Fear can be a paralyzing  heart sound. We must not be so frozen by fear that we could not focus, could not think. Yes, the seriousness of his aneurysm would motivate us to fight, but it must not paralyze us. We had to be able to focus.

Hope and Comfort

There is another heart sound that helped us through this difficult time. Hope. It dulled the fear, so we could focus on getting help.

 Carry hope with you always.

 Never let it go.

I want to tell you now that there is surgery that can successfully remove aortic aneurysms in the chest.  Looking back to that time, after coming through those two surgeries successfully, one for his valve and then for his aneurysm, we found that hope turned into joy, almost as though "a thing with feathers" took wing and soared within.

Perhaps while reading this, a feathered thing called hope will softly perch in the soul and sing to those who need it. May you have hope today and joy tomorrow.

In my heart I find that comfort is also a thing with feathers. If all hope had slipped away during or after those surgeries, I knew that somehow comfort would have come and gently perched where hope had been, just as sweetly as my beloved feathered baby perches on my hand. For some who read this, if it should be that the very best of surgery and medical care can no longer bring hope to you, may comfort gently wrap its soft wings around you.

Treated Just Like Other Valve Patients!

When my husband's BAV was replaced, he was sent home to heal and return to a normal life. There were no restrictions on his activity. He was told that he could do any exercise he wanted, including lifting heavy weights, which he had done all his life! And so he did.

Although he was born with an abnormal aortic valve, and his valve failed at a much younger age than the other, mostly elderly valve patients we saw at that time, he was treated just the same. In short, he was treated like everyone else, regardless of the reason why their heart valves had failed them. This is one-size-fits-all thinking. It is not good for someone born with BAV and their families.

Nothing was said to us about a finding noted in his medical reports called "post stenotic dilatation". Dilatation? Hmm. What was that Greek word again? 

For eleven years, we lived in ignorance that my husband's aorta was bulging in his chest. The bulging was gradually getting worse. He was taking coumadin (warfarin) to prevent blood clots forming on his valve. In an emergency, he would bleed more than normal. Ignorance is not a good thing.

Stumbling on an Aneurysm in His Chest

My husband did not have very many echocardiograms after his heart valve was replaced. One day the local internist, who had managed his blood levels over the years, suggested he come in for a visit in his office. He wrote out a prescription for an echo to check on his aortic valve, which seemed a good idea, even though this mechanical heart valve was supposed to be indestructible. 

His echo was done at the beginning of January, 2001. I remember a friend of ours asking about the results, and I told her we had not heard anything just yet but were not concerned. It was just routine.

There was one niggling thing bothering me. His blood pressure had been high in the internist's office. The doctor had not offered any medication, perhaps thinking it was just a one time occurrence. I believe today he should have known better. My husband's blood pressure was not just a little high - it was extreme. The "top" number or systolic was approaching 200, the "bottom" number or diastolic was over 100. 

But my husband didn't "feel" any differently, even with those high pressures. Shopping in a local store, I saw a home blood pressure monitor and bought it. These monitors were not that common then, and it stayed in the box, unused. I did not want to nag him about his blood pressure! We would see what the doctor said when we received the echo results.

Bad News Doesn't Always Travel Fast

It was three weeks before we heard anything about the echo results. Returning home from work, there was a message on the answering machine, telling us to call the doctor's office but not saying anything else. Not a good sign. When all was well, we were used to messages from the doctor telling us that. Now, he wanted to talk to us. Why?

On the phone, the internist said what he would not leave in a message. I still have the notes I made, the words I scribbled down that day. The aorta looked "a little dilated" above the valve. He did not use the word aneurysm. Perhaps he was afraid of that word too?

There had been no symptoms in his chest to indicate the presence of the aneurysm. Yes, we were shocked, but with time came to realize that since it was there, finding it was a good thing . We had an opportunity to deal with the aneurysm in a non-emergency setting, before it could rupture or tear.

A CT scan was scheduled at the local hospital. It confirmed the presence of the aneurysm, 5.2 cm in diameter, in his ascending aorta. There is nothing little about that size! The local internist had never followed up with us about my husband's blood pressure, never offered any medication to lower it. He just suggested that we contact the surgeon who had performed my husband's valve replacement surgery. From that time onward, I do not recall ever speaking to him again.

This was early 2001, and I turned to the internet in search of answers and help. I quickly learned that there was surgery for this. Basically the aneurysm was cut out, and a tube made of Dacron put in its place. Yes, there was something that could be done. No matter where we had to go to have surgery, we would do it. I expected we might have to travel far from home.

What Aren't Your Doctors Telling You? Get Your Medical Records and Find Out!

At this time, we became very disillusioned with our local physicians. We had trusted them with my husband's life. Now, he was on what is commonly called a "blood thinner", his aorta was bulging and could rupture in his chest, and he had also developed high blood pressure, which obviously was putting strain on that weak, bulging tissue. 

My husband was faithful in testing his blood to keep the anticoagulant levels within range. We were always on guard for infection, and he took antibiotics before any dental procedures. These were important things they told us. But nothing more. This is advice given to anyone with a mechanical prosthetic valve. Since he was supposedly "fixed for life", we believed that was all that would ever be necessary. It was not true.

No one had warned us. My husband had never known anyone with an aneurysm, but I had. I remember telling him not to lift those heavy weights out in the garage.

Yes, disillusioned may be putting it mildly. I thought that it was just our local physicians who didn't understand, especially when I learned online from medical papers that those born with BAV are prone to aneurysms in the ascending aorta. Later I would learn that these doctors were probably just like many other physicians. They had been taught that having BAV was really nothing of significant concern, and that "post stenotic dilatation" was quite harmless, especially after the BAV was replaced. I will write more about this separately. It is an example of how theory that is taken as fact endangers lives. While an idea may be attractive as an explanation, if it is not pursued carefully and thoughtfully questioned, it can be deadly.

In case you think it is different today, that your doctors are telling you everything,  I want to warn you. Just a few days ago, I spoke to a 33 year old man who lives in upstate New York. His doctors had known for years that he had a bicuspid aortic valve. They never told him. The week before we spoke, he had a burning sensation in his chest. He wondered why his doctor became so concerned, sending him to the local ER and insisting on a CT scan. He knows why now. They were afraid his aorta might be tearing in his chest. Today, he is educating himself. He is also looking for new doctors. Doctors that take an interest and are willing to learn along with their patients. Doctors that understand what is known and what still remains unknown about those with BAV. Doctors that will truly care for someone born with a bicuspid aortic valve. I sincerely wish him well in his search.

Fear and Your Three-Foot World

I am currently reading Mark Owen's new book, No Hero (1). I love it, because in each chapter he writes about a single topic, such as purpose, trust, and communication, using examples from his life as a Seal. There is no doubt Navy Seals need coping mechanisms as they go through so many extraordinary, life-threatening experiences. Those in BAV families may find themselves in tough situations also, and coping mechanisms help us through those hard times in our lives.

In chapter 4, he writes about fear. He describes the advice he was given by an instructor during a difficult rock climbing exercise (he has a fear of heights). Looking down and thinking about how far he would fall, looking across the horizon to a city in the distance . . . none of this would help him. He was told to focus on things within three feet of himself. And so he did, and he made it to the top!

Perhaps another way to say this comes from my mother's generation. She was descended from sturdy Norwegian pioneers who faced many challenges, and there was no going back to Norway!

  "By the yard, life is hard. By the inch, life's a cinch." 

Just stay focused on what you can do, step by step, to get through the crisis. You will find you can do a lot!

One day, I spoke to a woman with BAV who was symptomatic, fearful, and whose doctors were not listening to her. She was so discouraged she told me, she felt like giving up. I realized that she was seriously depressed. I tried to gently help her focus on what she could do, one step at a time. And I quoted my mother, encouraging her she could do this, one inch at a time.

None of us are powerless. If you or your loved one has just learned there is an aneurysm, it is all right to feel afraid. Just don't let the fear control you. Accurate information will give you hope, as you focus on what can be done, one step at a time.

Get your records and read them. Look up words you don't know. Ask your doctors questions. And if they are not helping you, find someone who will. 

You will be surprised what you can do in your three-foot world, one inch at a time.

You can save your own life!

With very best wishes to all,

Arlys Velebir

(1) No Hero, the Evolution of a Navy Seal by Mark Owen with Kevin Maurer, Dutton, 2014.

Surgery Day - Getting a New Heart Valve

Getting a new heart valve is a special journey.

Just focus on your destination, that high point across the valley.

You will soon be there!

The Kissing Place

Outside the operating room door there is always a special place. The exact location varies in different hospitals, but it is generally as far as you can go with your loved one before surgery. This is the kissing place. I hope no one ever goes into the operating room, which I will call the "OR" from now on, without that expression of love and support. 

This is not a "last kiss".

Don't ever think it!

 It is a "see you soon", 

"I'll be here when you wake up" kiss. 

I had given my husband that kiss and had been shown the small waiting room. This was the place where a nurse would come out and update me from time to time.  I had dear friends with me, standing by us both so loyally that morning. It was very early still. My husband was the first case of the day. I always recommend that if possible. One hopes the surgeon is nice and fresh, well rested, the first thing in the morning! 

Our Special Friend in the OR

Yes, I thought I had gone as far as I could that morning with my husband. But that was not the case. I have written about specially requesting the anesthesiologist, Dr. T. We had met him the day before, and he spent lots of time with us. He took my husband's blood pressure, which was very high. I will always remember his words, "If the pressure is that high in his arm, imagine how high it is in his heart." All because of a severely narrowed, plugged up aortic valve. I was so glad we were within hours of removing that obstacle to freely flowing blood once again.

There is an area where the anesthesiologist and others will do their preparation work before the person goes into the OR itself. In this hospital it was a fairly large room, and Dr. T. invited me to come there and sit beside my husband while he worked on him. There were no other patients, and I have very nice memories of the atmosphere there. Dr. T. was a calm man with a serious, confident manner. He had some sort of board underneath my husband's arm, there to stabilize it as he attached various lines to him. Whatever drugs were going in were definitely relaxing my husband, as he chatted with Dr. T. At one point, he flinched at something, so of course I did too. Dr. T. just looked across at me and said he was almost done! And soon he was. He asked my husband what he thought of his handiwork. Very seriously, my husband moved his arm with all the tubing very close so he could see (no glasses, and he is extremely "blind" without them), and inspected it carefully. He very seriously told Dr. T. it would be fine as long as it all was functional. Ah, those drugs were at work, as he calmly approved the doctor's work!

No Ordinary Days

At some point, others who would work in the OR that day also came into this room. I do not know what their roles were. I distinctly remember them chatting rather lightly about their social lives, sharing pictures with each other. I remember thinking, don't they know what a special, momentous day this is?! How could they be talking about parties?

Of course, surgery was something they did every day, an ordinary day for them.

An ordinary day for me would have found me at my new job. Yes, I had changed companies in the middle of all this. My manager and the company were very kind to me and found a way for me to have the time away I needed for my husband's surgery. Today, my workaday world seemed as far away as another universe.

Somehow I hope that those in medicine, and especially in surgery, never lose a feeling of wonder about what they do. There are others who spend their days at work making heart valves. Others are involved with pacemakers. All these lifesaving devices for the heart. I hope the same for them. The jobs they do can never be just ordinary jobs, ordinary work. Every day is special when you are saving lives.

I Will Take Good Care of Him for You

Now, it really was time for my husband to go through that one last door, into the OR. There I could not go. When Dr. T. told me, "I will take good care of him for you", those were not just words. I knew he meant it. Off they went together, but I knew he went with a friend, Dr. T. He had told us that the surgeon was a good technician. Now it was up to the skill of the surgeon's hands, his eyes, his judgment, to give him a new valve. 

Most of the time, we do not get to know the anesthesiologist very well, but they are a very important member of the surgical team. There are no office appointments with them before or after the surgery. Another time perhaps I will spend more time on their role, and how important they are, although we typically do not know them well at all.

I tell those I meet that surgery day is a good day.

Not an easy day, but a good day.

It is always a good day when life is saved.

Waiting

Waiting. This is what makes this such a hard day. Your loved one is in that drug-induced sleep, blissfully unaware of what is being done to save their life. But you are wide awake, adrenalin coursing through you, prepared for whatever may come. 

The nurse came out and updated us at key points. Generally, her updates were encouraging, telling me things were going as planned. In my experience, nurses give updates like this, meant to reassure. It is for the surgeon, later, to tell more of the story. 

I remember asking her once how the surgeon was doing, and she smiled and told me he was listening to "cowboy music". Ah, so they have music in the OR. Whatever kind of music helped them do their work was just fine with me! 

My friends encouraged me to walk around a bit, between updates. I did a little, but just could not stray very far from that little room in case they needed to find me. (This was not the day of cell phones, as it is today!)

During this waiting period, a frightening thought dawned on me. They would stop my husband's heart. His heart had been failing. What if it didn't start again? I had never let my mind go there before, but now I waited rather desperately to hear that his heart was beating on its own again.

At last, she told me that the new valve was installed. Then, that his heart was beating on its own once more! Oh what a wonderful heart, to take so much abuse and come back to beat strongly once again!

Let me say now, the heart and the brain both love to heal. They will fight to come back. In these years since, we have proved this more than once. Do not let anyone in medicine discourage you in the fight! Your own body will tell you. You will get better. You can have your life back!

Surgery Ends

I was not a good judge of time that day. I just know it did finally pass. The nurse was done now. The next update would be from the surgeon, and then I could see him.

And then, into the waiting room came our friend, Dr. T. How sweet of him! Other than his kindness, what stays with me now is his description of my husband's valve. He said it was the worst case of calcification on an aortic valve he had ever seen. Describing it, he said the calcification looked like cauliflower. Leave it to my husband to have the very worst! Well, it was gone now. It could not hurt him any more.

Dr. T. left. I have never seen him again. He has a special place in our heart sounds always. May everyone have an anesthesiologist like him.

When the surgeon came, he had more details for me. It is the surgeon's job to tell you everything. Your loved one is his patient first and foremost, although other doctors are involved. The surgeon's name is on that wrist band the patient wears.

He told me that the new mechanical valve fit well into my husband's heart, and that his heart had started up again well. Then he began to tell me about the severe calcification too. He told me that he had been very careful, but there was so much calcification that there was a risk that some of it broke off and got into my husband's blood stream. Any loose particles could either go to his brain, where it would cause a stroke, or travel to his finger tips or toes. The bottom line: my husband might have had a stroke during surgery, and we would not know until he woke up.

Yes, it is the surgeon who tells you both the good and the bad news, after the surgery is over. I wondered why they had not warned us about this before hand. My husband had gone to sleep without being warned of this high risk of stroke.

That First Look

Your loved ones do not see themselves, and even after they wake up, the drugs may still prevent them from remembering those early hours later. It is a good thing for them. But they need you to look at them, touch them, be there when they awake. Don't worry. Your loved one is still there, no matter how they look at that first glimpse.

A nurse took me back to some kind of holding area. I was glad my dear friend, who had years of nursing experience, went with me. I thought it was a terrible place!

Several people were apparently also coming out of various kinds of surgery, all of them lying on some kind of stretchers. This was a recovery area, where they kept patients before moving them to intensive care (ICU) or somewhere else.  My eyes searched, and then I saw him.

He was lying there motionless, on his back. Of course there were many tubes and lines. He could not breathe on his own. What shocked me most was his color. He was a bright red! He had been so terribly pale, no color at all, before surgery. Now rather than just normal color, his skin had the appearance of a boiled lobster. He has had two additional open heart surgeries, and I have never seen that again. I have never had an explanation for it.

This is the time to have a strong, sturdy friend with you. She quickly said in a positive voice, "Oh, look how pink he is now!" Yes, no doubt his blood was flowing much better now. Next they would take him to ICU. I could see him there, about 15 minutes every hour. 

Waiting for Him to Wake Up

My husband's parents were flying that day of his surgery. We had planned it so that they need not endure the hours of waiting with me at the hospital, but would arrive that evening and be with us for whatever would unfold next. Not an easy thing for them to do, traveling to be with this oldest child and first son of theirs, who had lived from childhood with a heart murmur.

Now I wondered how the timing would work, bringing them from the airport. I wanted to be there when my husband first opened his eyes. I especially wanted to see for myself what he would be like. Would there be any signs of a stroke?

At some point, I encountered the cardiologist. He told me that he had seen the valve that was taken out of my husband's heart. The way he said it implied it was in terrible condition, which I already had been told. The one thing I wanted to know, "Was it bicuspid?" "Yes."  There was no doubt now that my husband had been born with an aortic valve with only two leaflets. I believed it now, and never forgot it.

Intensive Care (ICU)

I am not very brave at all. Intensive care is so good for my loved one, I know, but it not easy for me to take those steps and enter there. I do much better once I see my loved one.

My first experience with this was with my father. He had cancer and radiation was tried as one of his last treatments. Aimed at destroying the cancer, it also caused him to bleed suddenly, and he was rushed to ICU. Yes, it was a good setting for him then. Each patient in their own little cubicle, all in a semicircle around a desk area in the center for the nurses. But the gravity of the illness of each patient overshadowed attempts to make it feel close to a pleasant setting.

Now my husband was in ICU, with tubes of various kinds, a machine breathing for him. It was the first time since our marriage that I felt truly separated from him, although I stood there beside the bed and touched him.

I may not be very brave, but I am convinced of the importance of being positive. No one, including myself, shall be negative at all when someone is fighting to reclaim their life after surgery.

From the first moments, before they are awake, I do not want any trace of negativity to come near them. They just need loving reassurance that they are doing wonderfully, that all is well. I have done my best to do this. I need hope myself, and I would never rob another person of that precious lifeline.

It was a time for patience. Waiting for him to come back to me.

And then, there he was! He announced his awakening by setting off loud beeping, blaring alarms to alert the nurse. He was trying to move. There were his eyes, open at last!

We were connected again! I don't remember all I told him. I do remember the intelligent look in his eyes. He could not speak with the respirator tube still in place, but he nodded when I mentioned his parents arriving that evening. He has always had an amazing memory, and at least recent events were still there.

At times like this, you really want to know each other. That unspoken communication is priceless. I was satisfied. I did not need to wait for the doctors to tell me. If there was any damage at all, I thought it would be minor.  I just wanted him to still know and love me! He did!

Later, I was there when the cardiologist came by. He asked my husband to move his legs and wiggle his toes, which he did beautifully! My husband still had the tube in and could not talk, but I read his mind. He was wondering what was wrong with the doctor! The surgery was on his heart, not his legs and feet! He had not been told yet how worried they were about a possible stroke.

God Will Guide the Surgeon's Hands

My initial assessment was right. There was no stroke at all. He just needed to heal and get strong again. Before surgery, my mother told me something very special. She told me that God would guide the surgeon's hands. None of us knew how challenging my husband's situation would be, with the terrible excessive calcification of his valve. I was comforted then, by that thought, and grateful for those hands and how they were guided. If that had not been the case, how different the outcome might have been.

He spent two days in ICU. It should have been just one, that was the goal. But he was not strong enough that first day. I walked in once as they were trying to get him to sit up. "Oh, look, there is your wife. Sit up for her!" His head wobbled on his neck like that of a baby bird, too heavy to hold up!

I had to sign in each time I visited ICU. A volunteer was there with the list of ICU patients. One time, as the volunteer went down the list of names, I noticed what was written down for my husband. His condition was serious! I was shocked. Everyone was being so positive and encouraging. I thought he would at least be considered good!

He had a mechanical valve now. I heard the cardiologist and the surgeon discussing when to start the coumadin that would prevent blood clots from forming on this pristine, new valve. The cardiologist pressed for starting soon. The surgeon did not want any uncontrolled bleeding. They worked it out. My husband cooperated by not bleeding!

Despite any labels used to describe him, he did get stronger, and it was time for him to go out on the floor.

No Private Room Available?!

Having a private room after open heart surgery is so important. Some hospitals have been built in such a way that the rooms are essentially private but can be billed as semi-private. Those are technicalities, but a good thing to understand. We were willing for extra cost to have a private room.

But, this hospital did not have any private rooms open when it was time to leave ICU. His first room on the floor was with an older man who had a terrible cough. I was so upset, afraid that my husband was being exposed to some sort of lung infection right after surgery, and having survived pneumonia himself. They told me that the other patient was not contagious. He had aspirated fluid into his lungs somehow.

A private room did become available soon, and he made good progress. We walked the halls together, working on getting strong enough to go home. Our local internist came every day that he was in the hospital. Much later, I realized that there were never any bills from him for those visits. He told our friend that most of his patients were not as sick as my husband. No doubt he wondered if my husband would survive, and if he did, what he would be like.

No hospital experience is complete without mentioning nurses. They took good care of him. Perhaps that in itself is remarkable. Our later adventures in hospitals cannot be so glowingly described.

Going Home

We were told that after surgery, nothing tastes very good. But I did not have surgery and can vouch that the hospital food was terrible. At home, his mother would be there, cooking for him.  Both his parents were there while I returned to work. It was not easy for his parents, no longer young, to do this. Seeing their son, whose heart murmur must have weighed on them over the years, go through this hurt them too. But they saw him become strong again. We have always been thankful for their loving support. They did not live to see the day when their daughter would have a high risk surgery too.

We were discharged on the 7th day. There would be one follow up with the surgeon. Our local internist was given the job of monitoring my husband's coumadin levels.

This is a long post. Surgery day is a long day! I have shared details here in hopes of giving a glimpse into what is a foreign world for most of us. We enter that world of hospitals, operating rooms, nurses, and doctors only when something is wrong, when we need help. My husband has had two additional surgeries and a stroke since that time. I have gone through surgery with others also. Some I have known well, others not as well. There are heart sounds in those stories, too, still to be shared.

For now, I leave you here, with my husband's heart strongly beating once again, as we resumed our normal life.

We were told he was fixed!

 Fixed for life! 

We believed it.

If only it had been true.

Best wishes to all,

        - Arlys Velebir

Life Savers - Heart Valves Made by Man

Starr-Edwards ball and cage valves                                                    
I took this picture on a tour of the valve                                                    
 museum at Edwards Lifesciences                                                      

Thirty years before my husband's crisis, surgeons began implanting artificial valves in place of damaged, failing valves like his. Pictured here are some of those early ball and cage valves. We did not know very much about mechanical heart valves. We did know that there were newer versions with movable leaflets. 

Now that we were meeting the surgeon, we had one very important question to ask him.


Frightening Headlines in the Local Paper about  Bjork-Shiley Valves

While we were waiting for my husband's surgery, some very alarming stories appeared in our local newspaper. Mechanical valves had been breaking inside people's hearts! People were dying. Others were having surgery to replace these valves before they could break.

Imagine having a valve like this in your heart!

Having seen these articles, we wanted to be sure that the surgeon was well aware of any problems with mechanical valves. He was. I realize now that every heart surgeon in the world would be well aware of these problems. I remember him telling us that certain sizes of the Bjork-Shiley valves had the problem. While these valves were no longer allowed to be used, we did not want anything even remotely like this to happen to  my husband!

I also remember being told, perhaps by the cardiologist, that the valve my husband would receive had been tested in the laboratory and proven reliable. It would last far beyond a human life span.

We had no choice. Tissue valves wouldn't last very long. My husband would die, like untold numbers before him who needed help prior to 1960 had done, if his valve was not replaced. And so we placed our trust in the surgeon's judgment regarding the valve he would implant in my husband's heart.

Ultimately, that is what you do when you sign the papers.

 You trust the surgeon with your heart, your life.

Encouragement from Others

I have always been grateful to one of my sisters. She told me about friends of her family who had gone through this. This man had recently had aortic valve surgery and was doing well. He had been offered a tissue valve locally, and had gone elsewhere, to another state, to a surgeon who would implant a mechanical valve instead.

Not long ago I asked her about this man. For him, the mechanical valve implanted about 25 years ago now has worked out very well. He still has it. Perhaps it will be a lifetime solution for him. I believe his heart valve had been damaged by rheumatic fever. We know another man who had his aortic valve replaced also, around the same time. I saw him perhaps a year ago. He too still has his valve. So far, it has served him well. I am so glad when it works out this way. This is the dream we all have.

Yes, this is the dream.

A valve solution that lasts a lifetime.

But too often it remains an elusive one. 

On the eve of his surgery, talking to the surgeon, no one knew that the same valve that would save my husband's life now, would later become the source of a terrible injury to him.

Today, the number of mechanical versus tissue valves being used is an interesting comparison. Tissue valves now far exceed the number of mechanical valves being implanted. In 2011, 16,780 mechanical valves were sold in the United States, compared to 75,734 for tissue valves. There are a number of reasons for this, a topic for another time. (See reference 1 below)

I am remembering the first time, years later, that I heard a surgeon tell someone that there are no perfect valve solutions, and then go on to discuss options in detail. It is a more realistic, much better informed experience than what we experienced so long ago. Today the patient is much more a part of the valve decision making process.

Perhaps it is fair to say there are no perfect solutions,

 but there are certainly good, life-saving ones!

There is a great deal of work going on as I write this,
aimed at new solutions already being made available to high risk individuals.

Saying Goodnight

At 10 pm, the hospital told me that I had to leave. Worse than that, I could not call him later. The hospital switchboard would not accept calls to patient rooms this late. And so I drove the few miles home. Neither of us could sleep! My husband was not to be defeated! I remember the phone ringing, around midnight or was it 1 am? I could not call in, but my husband could call out! After that call, we both slept. The next thing I knew, in the wee hours of the morning I was up and back at the hospital. The nurse was helping him prepare for surgery when I arrived.

Waiting for this day, I remembered how I thought we would be old before he would need anything done to his heart. That had been wrong. Our wedding anniversary would be in the summer. Now, I wanted so much just to have that 10th anniversary together. This was the surgery that would make that possible!

With all best wishes,

- Arlys Velebir

Reference 1 - Aortic Valve and Ascending Aorta Guidelines for Management and Quality Measures