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

Sunday, December 18, 2016

Can the Mice Save Our Families?

Still Not Understood
Although at times the BAV journey is lonely,
always remember to see the beauty along the way!

I was chatting with a member of our family not long ago. I never actually expected to have to say to this person what I have told so many others, those who have contacted the Bicuspid Aortic Foundation over the years: 

"The doctors will not understand you.
 There are no convenient labels or terms to describe you in medicine.
 Don't be discouraged, and don't let them tell you it is anxiety.
 Your symptoms are real. 
It is just that no one understands it all."

The doctors had already offered anti-anxiety medication which, in this case, was very wisely refused. We have a plan on how to move forward, but it is not an easy path. Just saying that we come from a BAV family does not help at all. BAV is still looked at primarily as an isolated defect in the heart.

What About Our Tissue?

Those with BAV, at least until now, are not generally referred to as having a tissue disorder. I have believed for a long time that they do, but without proof. It was based on my own husband, others I have met, and over time, other members of our family. Unlike some other conditions, BAV families blend into the general population very well. If anything, they are over-achievers and at periods in their lives at least, glowing with health, vibrant and full of life.

And then, at some point, and to varying degrees, they fall apart. Maybe it is their bicuspid aortic valve, maybe an aortic aneurysm, or both, maybe the "electrical" side of their heart, maybe other parts of their bodies - eyes, joints, GI tract - give them trouble. I will just mention two areas on my mind because of our own family right now.

The Eyes

Ever since I heard Ken Simon's story, I have worried about my husband's eyes. Before he lost he life to aortic dissection, Ken had an eye emergency - retinal detachment. My husband has had very poor eyesight from birth, and I have always wondered about his eyes. We have had his eyes examined carefully by multiple specialists, trying to proactively guard his sight. Some of those specialists were incredibly arrogant, but we persevered because eyesight is such a precious thing. He has been told that he has thin areas of the cornea in both eyes, and that he was "probably born that way".  So far at least, my husband has avoided a detached retina. Much to my shock though, it happened to a member of our family not long ago. Someone with thin areas of the cornea also. Someone with other characteristics through out the body that are a lot like my husband.

The Electrical Side - Abnormal Heart Rates and Rhythms

Sometimes they have heart rates that sky rocket without warning - again, I am thinking of my own family and also others I know well. Why? On the other hand, my husband's heart rate can drop like a rock on beta blocker medication, so he has to watch the dose carefully.

Mice Just Like Us to the Rescue! 

It is hard to describe the longing to at least be believed, if not understood, by physicians. Listening to Professor Mona Nemer recently describe the families of mice with BAV in her laboratory at the University of Ottawa was indescribably wonderful. These mice sound so much like my own family, and other families I know. And Professor Nemer and those in her laboratory are making great progress in understanding them!

Some of them have BAV's, but not all of them - at least not obvious leaflet malformations. Some of them have aortic aneurysms, some have malformations of the aortic arch. It varies, just like in human families.

There are issues with their blood pressure! And some of them develop "electrical problems" with their hearts as they get older. Yes, just like in human families!

It Is the Tissue!

These precious mice are teaching the researchers about us. I am moved beyond words by their contributions. I was beyond thrilled when Professor Nemer said that it is a problem with the tissue, based on a genetic defect! Yes, at last, someone is seeing the big picture throughout the body!

Personally I am very grateful to these little mice and the researchers who study them. I hope to live to see the day that conversations like the one I mentioned above are no longer necessary. As the year closes, I want to remind everyone that donations to the Bicuspid Aortic Foundation will directly support the research being done with these mice, who are so very much just like us!
Amaryllis Fantastica

Best wishes to all through
 out this holiday season,
 and may this New Year of 2017 bring 
more answers, 
more understanding,
and more hope
 for families with BAV.

~ Arlys Velebir