Thursday, November 16, 2017

Until We Meet Again, My Love - the Last Heart Sound

Under the wide and starry sky
Dig the grave and let me lie.
Glad did I live and gladly die,
And I laid me down with a will.

This be the verse you grave for me;
"Here he lies where he longed to be,
Home is the sailor, home from sea,
And the hunter home from the hill." 
-                                               -  Requiem by Robert Louis Stevenson

Somehow these words of the poet have come to me, perhaps from school days, as I made the arrangements for my husband's body to finally rest under the beautiful Big Sky in Montana.

Red Jasper
"The Warrior Stone"
Yes, the last battle has been fought in a war that he and his courageous, wonderful physicians ultimately could not win. It became clear that it was no longer necessary for him to hunt, to seek more help from medicine or another procedure. He could return from the sea of life, where in 2017 he was hospitalized five times, and take his last breaths as he desired, in his own home.

On February 28th, he underwent a high risk, technically challenging fourth surgery to replace his failing bovine aortic valve once again. The surgery was perfect, he did well, and came home to begin what seemed like a routine recovery. However, he abruptly went into kidney failure a little over two weeks after surgery, coming to the brink of death before fighting back and returning home. There was no explanation for the kidney crisis then, but in retrospect I believe it was the extreme response of his immune system to a foreign invader, bacteria that entered his blood stream at the time of surgery. In the course of time, the bacteria was identified and he underwent multiple rounds of treatment, most recently about 8 weeks continuously. Removing the infected valve surgically was not an option - he would not come off the operating table alive if it were attempted again. He was still in the fight, however, and an expert in valve-in-valve TAVI offered him a procedure to insert a new valve into the infection-damaged one. However, it became apparent that the infection was still present, and it viciously destroyed the electrical node near his aortic valve. He was in complete heart block, and it was not hard to understand it was time to leave the battle to others with BAV.  This heart warrior, who underwent 4 open heart surgeries and suffered a major stroke from valvular strands, had been given permission to leave the fight.

Since he has left me, our beloved Dr. Rose and many others have remarked on how amazing it was that he was able to go through so much: 4 open heart surgeries and the stroke over a period of 27 years. He was hospitalized 5 times in 2017. It is true that he was gifted with great physical strength, and his heart muscle was strong. Above all, he had learned that his help and strength came from God, who had gifted him with life, breath, and all things. Over and over, I saw him rise above the pain from the infection and the painful things done to him, things necessary in order to help him. 

But they that wait upon the Lord shall renew their strength;
 they shall mount up with wings as eagles;
 they shall run, and not be weary;
 and they shall walk, and not faint.
                                                - Isaiah 40:31

This verse from the Christian Bible has beautiful imagery, as does this picture of a soaring eagle. As I walked beside my husband every step of this journey, every battle he fought, I know that these words are not just beautiful, they are the answer to how he continued to cherish the life God gave him, regardless of what happened to him, until the moment it was time to go Home. I write this here because I know he would want to tell you this, if you should ask how he was able to do the things he did.

The bacterial infection caused his new bovine aortic valve to leak, and once again he had a heart murmur. When at last his heart stopped beating, the murmur stopped too, never to be heard again. 

My beloved, the battles are over,
 the murmur is gone, forever.
  Soon your body will rest
 under the wide and starry sky,
 as you wished.

Sunday, August 6, 2017

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.

Saturday, July 8, 2017

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.

Saturday, June 3, 2017

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.


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."


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,

Sunday, April 9, 2017

When a Heart Valve Fails

It is 27 years this month since my husband's own bicuspid aortic valve was replaced. It had completely failed him, causing his entire heart to fail too.

Since that time, we know now that his body has struggled with the artificial valves that have kept him alive, aortic valves made by man. First a mechanical valve. Then a bovine tissue vale. And now, it is almost 6 weeks since he received his third artificial aortic valve.

Why another surgery? He was, quite simply, on the brink of losing his life. The 11 year old bovine prosthetic valve in his heart was failing.

The 2 leaflets at the bottom were completely frozen,
only the 1 leaflet at the top was moving to let blood flow
Tissue Valve Deteriorating at Eight Years
At 8 years, we were told that one of the leaflets was not moving well. Only 8 years?

We had hoped that he would be like many in his age group, whose bovine pericardial valves still function at 20 years.

Inside his body, for whatever the reason, the valve is considered a foreign invader, something that needs to be attacked or healed!

I had read that tissue valves like this last about 6 years in children, and that was the case for one teenager we know.

He had a transesophageal echo, and those findings was more hopeful. The one leaflet in trouble was moving enough to close completely, it just could not open all the way. Maybe it wasn't too bad, and would still last quite a long time.

Another Leaflet in Trouble

Late in 2016, with the valve approaching 11 years old, a "regular" echocardiogram, through the chest wall, showed that two leaflets were in trouble now. We had to face that it wasn't just one leaflet, and that this valve continued to deteriorate.

Surgery the Only Hope

My husband was not a candidate for TAVI (TAVR), the procedure where another valve is threaded up and inserted inside the old, failing valve. One of the reasons for that was that his aortic valve diameter is too small, only 21 mm, to accept another valve inside it.

There was another reason also, that would have prevented TAVI from helping him. On the bottom, or "intake" side of the valve, my husband had grown scar tissue, called pannus all around the ring of the valve. It is pictured on the left. Most of the tissue was cut away and sent to pathology, so only a small part of the softer tissue remains attached.

This tissue was also blocking blood flow. In order to have full blood flow, those failing valve leaflets, and the scar tissue attached to the valve ring, had to come out. There was no other way.

Today, understanding these things, I am surprised that my husband's heart and body had coped as long as it had.

I found a paper from surgeons in Japan, Subvalvular Pannus Overgrowth after Mosaic Bioprosthesis Implantation in the Aortic Position, that discusses this happening in some of their patients. There is a picture, Figure 1, that shows the ring of scar tissue below the valve. In the conclusion, it mentions that preventing this scar tissue formation is unsolved.

My husband also grew pannus and strands of tissue on his first prosthetic valve, a mechanical valve. My personal feeling is that there is something about his body's reaction to these "foreign" valves that causes it to want to heal. However, in the body's attempts to heal, it has hurt him.

I do not know how many people form scar tissue on their artificial valves. Perhaps some of them do not live long enough.

In any case, this is why time was no longer our friend, and the surgery door was our door of hope. 

No Solutions?

And that, in this year 2017, remains the challenge. There is still far too little that is understood about the tissue in some of those who are apparently most complex, like my husband, who were born with a bicuspid aortic valve. For the sake of generations following us, we need answers to these mysteries.

I am glad that physicians such as those in Japan have seen and published about this. I refuse to believe that there are no solutions to these problems, if only someone will look for them! 

Monday, April 3, 2017

An Unplanned Door of Hope - Emergency!

An Unplanned Door of Hope
 The emergency entrance of the hospital
where my husband had his recent heart surgery.

As we passed the three week milestone after his surgery, there were some signs that my husband was not progressing as he should. Nothing, however, prepared us for his sudden decline. In the early hours of the morning, I was online and on the phone, searching for an ambulance to return him to the hospital where his surgery was performed. 

The Nearest Hospital

I could have called 911, the emergency telephone number here in the US. If I had done so, an ambulance and fire truck would have come quickly, from less than a mile away.

Why didn't I just do that - call 911? From previous experience, I knew that our local service would take him to the nearest hospital that in their judgment had the expertise he needed. If you search online, you will find articles that describe how this works, in my area as well as most of the US. 

Continuity of Care

Quite simply, I didn't call them because I felt strongly that my husband needed to be back under the care of the same team that had so recently cared for him during and following his heart surgery. They were not the physicians nearest to us, but they were best equipped to understand and help him.

In the medical world, they call this continuity of care.

Finding an Ambulance

Some companies would only transport him to the nearest facility. I found one service that would not do it, because it had to be a distance of at least 300 miles, and we only needed to go about 75! 

Who would do it? I found an "inter facility transportation" service. Yes, they would take him from our home to the Emergency Room of the hospital where he had surgery. 

I am forever grateful to this company.

Decision in My Hands

My husband was too sick by this time to know anything except that he was very sick, but I was confident that I knew what he would want.

We were taking a risk to do this, rather than go to a local hospital. 

Each person and their loved ones must make
 what they believe is the right decision,
and be prepared to accept the consequences, whatever happens. 

I rode in the ambulance with him, seated at his head, steadfast in the conviction that he needed to return to these specific doctors. I did  not know what was at the root of this. However, especially if he had succumbed to an infection, I wanted him in the hands of his surgeon, who has a stellar track record at conquering post operative infections.

Mercifully, his vital signs remained stable, and what seemed an endless ride at last brought us to the Door of Hope pictured above. He remembers almost nothing of any of this - the ambulance ride, the experience in the Emergency Department itself, and some of the time in Intensive Care. I am so glad. It is enough that I must remember it. 

In the Doctors' Hands 

I stayed in the Emergency area to answer questions and sign any permissions needed to treat him. I can never express my gratitude to the Emergency doctor, in my memory a lovely woman with dark hair, and the two nurses that worked on him. It was not long before the Pulmonary specialist who had cared for him appeared. I am sure he was shocked to see the change in a man who had looked so well when discharged. I know I was crying as I told him that my husband was just "a mess"! Later his surgeon arrived, and no doubt others.

It was up to these doctors now, to do what they could. And they did!

Acute Renal Failure

The Mayo Clinic website states that this can happen rapidly, can be deadly, and requires intense treatment. I certainly saw the reality of those words! 

In that cubicle in the Emergency Department, and then in Intensive Care and through out his hospitalization, the treatment was indeed intense.

I have not counted the number of doctors who cared for him.  There were many.


Today we are grateful for many things, especially the expert and compassionate treatment and care. His doctors and nurses gave him the best possible treatment and recovery. We are home together again, no dialysis was ever needed!

Surgery Far From Home

Out of four heart surgeries, only one was done nearby our home. From past experience, I knew that it might be necessary to return to his doctors and hospital if there were complications. In 2006, we had signed out of our local hospital, who wanted to admit him, and I drove him back to the hospital where he had surgery myself. He was stable enough and well enough to ride in the car that time.

However, this time, he was too ill, too quickly, for me to drive him there. What I discovered is that, even if paid privately, cash up front, many ambulance services will not transport someone from their home to the hospital they request. 

If it should be necessary again, I will always have the phone number of that inter facility transport service in my area at hand and be prepared to call them. 
The 911 service responds to everyone, and takes them to the nearest hospital equipped to help them. For example, in the case of a stroke, to the nearest stroke designated hospital. Generally this makes sense.

However, in a case such as this, wishing to return after a major heart surgery to his doctors was a challenge. It was necessary to pay the full ambulance fee up front in order to make it happen. I understand that may be beyond what many people could do. In that case, the next best thing is to get to the local hospital via 911.

Let Others Help You

Normally, when we are well, we are used to being independent. Going through this crisis, there were so many who were so thoughtful and helped so much. We can never adequately thank those who were there in our hour of need.

I think now of my dear friend and her husband, who drove my car to the hospital that day while I rode in the ambulance, and stayed with me in those first terrible hours at the hospital. Others came later to be with me. A very dear friend of the heart, on the other side of the world, spoke to me by phone at midnight that night. A very brave young woman came daily to support and cheer us. There were so many messages of love and encouragement.

For all those who sent thoughts and prayers on our behalf, we are forever grateful.

Plan for the Worst, Hope for the Best!

Thinking about it now, I could have identified the ambulance company ahead of time. I just did not realize many would not choose to be hired privately. And so, in the future for us, when our doctors are not nearby, I will plan for the worst case, and hope for the best!

And that worst case would include knowing ahead of time how to request an ambulance to take us back to the doctors and hospital we need.

Above all, especially in emergencies,
keep hope in your heart!
There is a Door of Hope waiting to receive you,
a door with big red letters that say

Best wishes to all who follow us on this journey,
~ Arlys Velebir

Thursday, March 16, 2017

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!

Friday, March 10, 2017

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

Saturday, March 4, 2017

Making Progress in Our "Three Foot World"

I took this picture years ago. Looking at it today, I feel my husband and I were in that place on the water where the sun shines through the clouds.  There has been just enough light and hope to guide us and help us once again.

In May 2016 I wrote about our challenges with prosthetic aortic valves. (See "Imperfect Heart Valves" post, May 10, 2016).  Today, less than a year later, the concept of the three foot world shared by Navy SEAL Mark Owen is very helpful once again.

What is it like in our three foot world today? I marvel at all that is within our reach. Specially trained cardiac nurses, medicines and monitors, tests of all sorts.... Things I don't begin to truly appreciate. There is no place more detail oriented, more proactive. All are here to help my husband recover.

Even in this very special place, my husband is rather unusual. As one of the doctors said this morning, most people don't have even one heart surgery and now he has had four!

He is making good progress here, and all signs are that the clouds are being banished, many things being discontinued and taken away. They are no longer needed as he progresses toward moving to a "normal" hospital room soon!

The picture below, clear blue skies and calm seas, symbolizes our goal as we think of returning to our life again, a priceless gift of more time together.

Wednesday, March 1, 2017

Recovering in Intensive Care

My husband was very brave as he went through the door to the operating room. This was as far as I could go with him. Heart warriors and their surgeons know this is their battle, and it is for  loved ones to stand by as bravely as they can, waiting to welcome them again when surgery is over.

It was a long, complicated surgery, as we understood it would be. It is no small thing just to safely open the chest in someone who has had three prior heart surgeries, let alone deal with the issues once inside.

It was also a marvelously successful surgery. Full blood flow was restored with a new valve in place, and as night turned to morning, his heart recovered beautifully from the surgery. As I write he is breathing on his own, awake and alert. There are more milestones of recovery to achieve, but this is everything we hoped for at this point.

Once removed, it was clear that the failing bovine valve only had one leaflet still functioning. In addition,  some kind of scar tissue had grown around the intake side of the valve. This also obstructed the flow. Clearly his heart had valiantly coped with this restricted blood flow, but had reached its limit, making surgery urgent if it was to be at all.

Today, on the other side of that Door of Hope, we are together once more, still in intensive care, but we dare to dream of futures days together once more.

Monday, February 27, 2017

The Door of Hope - Surgery Once Again

Beautiful Pacific Coastline, Southern California

There are always memories, whenever we have reason to visit our local hospital. This hospital happens to have a beautiful natural setting, overlooking the sparkling waters of the Pacific.  My husband's heart experience began there so long ago now, in 1990. His first ever echocardiogram was done there at that time. 

Last Monday, February 20th, was such a day of memories, as once again we drove there and parked. It was time for another echocardiogram (ultrasound) of his heart. We are so grateful for a very special sonographer there, who over the years has so carefully taken the images of his heart. Once again she did her excellent work that afternoon and coordinated getting the results read and the doctors connected.

On Tuesday evening, the 21st, the results were explained to us, and it was clear that it is time to intervene once again. After 11 years, the artificial tissue aortic valve is severely narrowed and also leaking. The heart, especially the right side, is working very hard, there is back pressure affecting the lungs, and there may be some risk of another stroke if we wait! The good news is that his heart muscle remains strong and vigorous, and placing a new aortic valve will allow blood to flow properly again and address all these issues. If the mitral valve needs any attention, it will be addressed as well.

And so it is time for surgery once again. Having done this three times previously, we know what this means, but of course each time the body may respond somewhat differently. We look forward with hope that he will soon feel better with his heart and blood flow working properly again.

We understand that it is wise to do this soon and eliminate the threat of further injury. The arrangements are all in place for his surgery, 24 hours from now. All that is left is just to get to the hospital on time!

Without intervention, in 1990 my husband would have lost his life to heart failure. He was incredibly sick, with critical BAV stenosis at that time. We had never even heard of BAV until then, although we knew he had a heart murmur! 
Keep Hope in Your Heart!

Since then there have been 27 precious years, thanks to his heart surgeries. It is a great comfort that once again when he needs it so much, this surgical door of hope is open to him. 

This is why I write so often about hope. As my dear friend BJ shared with me once again with the picture here, always "Keep Hope in Your Heart".  I know there are so many in BAV families like ours, some just newly diagnosed, others facing another surgery. On the threshhold of my husband's surgery, his Door of Hope, may all who need it find their door of hope and receive the help they need.

Keep Hope in Your Heart,

~ Arlys Velebir

Monday, January 2, 2017

Bicuspid Aortic Valve - Our Dreams for 2017

On December 20th, 2016, the Dalton Daily Citizen brought back this column from 1990.  Lewis Grizzard: A Christmas dream    When Lewis Grizzard wrote this, his mother, father, and some of the others in his family he writes about were no longer alive. Yes, this family gathering for Christmas was only something he could dream about in 1990.

Lewis Grizzard was born with a bicuspid aortic valve. It is deeply touching to me to read this part of his dream :

 "I've got so much to be thankful for, this and every other Christmas.
 I've got my health. When I was 15, the doctors discovered a heart murmur.
 But it didn't turn out to be anything serious.
 I can still boast of the fact I've never spent one night in the hospital."

This was also only fantasy, just a dream. Lewis Grizzard had indeed spent nights in hospitals by then. He had already had two heart surgeries.  His first aortic valve surgery had been in 1982, with another just 3 years later. It had turned out to be "something serious" indeed.

During his life span, Lewis Grizzard would have a total of 4 open heart surgeries within 12 years, from 1982 to 1994. 

In this article announcing his death, Lewis Grizzard, Southern Humor Columnist, Dead at 47, his first heart valve surgeries, in 1982 and again in 1985 are mentioned. In 1993, he received a mechanical valve, as well as aorta repair and coronary artery bypass, a very large surgery. In February 1994, it was reported that he was hospitalized in Florida to deal with an aneurysm of the right kidney:  Lewis Grizzard in Critical Care at Florida Hospital February 1994 .  In March 1994, a very high risk surgery was done to deal with a "life threatening mass" in his aorta. This time an aortic valve from a human donor would be used (perhaps to be less prone to infection). Lewis Grizzard did not leave the hospital alive. His brain was too injured, due to lack of oxygen at some point. Perhaps particles from that "mass" had escaped during surgery and blocked blood flow in his brain? 

Lewis Grizzard Obituary (died March 20, 1994) following 4th heart surgery

Dreams for 2017

Just for  a moment, we can give ourselves permission to dream a "heart dream" too. Dream that our lives are not touched by bicuspid aortic valves, aneurysms, and surgeries.

There is no harm in dreaming, but we cannot linger there too long, in our dream world of the heart.

In these first days of the new year, let us resolve to do all we can to live fully, including being fully informed, as we embark upon the next part of our journey with BAV.

In the reality of our journey with BAV, some of us will be called upon to make important decisions this year. All of us in BAV families must take the very best care of ourselves possible, stay informed about our own bodies and about the research in progress that may change our futures, and seek the answers we need.

Living with a "special" heart does not mean that our dreams of time spent with loving family, of achieving things important to us, cannot come true. The number of leaflets of an aortic valve need not rob us of so many important joys and goals in life. May we find, after all, that even though we have imperfect heart valves and delicate tissue, that our dreams do come true in 2017!

Wishing everyone courage, strength, and joy in 2017,
~ Arlys Velebir