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.



Fever

After what we call the "kidney crisis" at the end of March, we returned home with hope in our hearts that the worst was behind us. After only about a week, he developed shoulder pain, which gradually became worse and reached the point where we were working on seeking help. Before that could happen, he spiked a fever!
Walking through the ER door once again, 27 years later, with a fever.
Fortunately, by this time we had met with a local physician who is very familiar with his heart issues and had arranged for him to take my husband as a patient. That day, we had an appointment just as the fever peaked. We were sent directly from his office to our local hospital, where the battle was fought.

The parking area also remains unchanged.
He was admitted to the hospital and started on powerful IV antibiotics.

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

Sepsis

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

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

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

Echocardiogram - Is the New Aortic Valve All Right?

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

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

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

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

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

Final Results

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

As we returned home, it was to see this lovely planting, a reminder of endurance over many years.
This plant, a gift at the time of his first surgery,
faithfully blossoms year after year.
It is a gentle reminder
to keep hope
 in our hearts,
always!



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.

Grateful

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
EMERGENCY!

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.