Reflections on Life - Ordinary Days, Tubes, Pressure, Tears, and Ruptures

Any ordinary day can become a crisis in a just a moment of time. 
Recent research about the reason for weakness of the BAV aorta
reminded me of this experience with my car!

My weak, bulging, torn, ruptured radiator hose!

It was just like any other morning. I pulled into a parking spot and switched off the ignition - an ordinary motion, on an ordinary day. Immediately, to my shock, great white billows emerged from under the hood of my car! Getting out, I could tell it was steam rather than smoke - no fire at least, that was a relief!

Eventually the steam subsided, but not before attracting some attention, while I called my wonderful local mechanic and arranged to transport the car to his garage. When he arrived, the flatbed truck driver looked under the hood and pointed out the likely culprit, a hose. Riding in the cab of that huge flat bed truck, I wondered how bad it was. I knew one thing - I was extremely glad that it happened when it did, parked, and with the engine shut off. What if it had happened while driving in that rush hour traffic - not a safe place to break down?!

The human aorta has three layers - the outer one is very strong.
That strong outer layer can save lives when it holds on long
enough to get to surgery, after the inner layers tear.
I don't know how many layers of materials this hose had,
or how well designed it was to handle pressure. 

I left my car in excellent hands, knowing it would get a thorough check up. Later, the call came, confirming the problem. There was indeed a torn, ruptured main radiator hose. The hose had become weakened and bulged out over time (the clamped ends were still the normal size). I do not know why it chose that precise moment when I shut off the engine to tear and burst. Was there an extra surge of pressure just then? 

Diseased, Bulging Aortas are Dangerous!

When my "car doctor" called to tell me what had been found, it reminded me so much of the way aortic surgeons describe the diseased aorta in the chest - weakened and bulging! 

When the aortic tissue becomes weak and bulges, it is also prone to tearing and rupture. If it tears, causing bleeding in the wall of the aorta, it is called dissection. Quickly getting into surgery can save lives, as long as the outer aortic wall holds. When the aorta completely breaks open, the massive, uncontrolled bleeding is rapid and deadly.

Aortic aneurysm and bicuspid aortic valve

Check Your Hoses - Replace if Necessary!
Unfortunately, it is easier to replace the radiator hose on my car than it is to replace a weak section of aorta inside the chest. However, it can be and is safely done! The best time to do it, like it should have been for my car's radiator hose, is before it tears or breaks. 

Torn and completely ruptured, radiator fluid was soon all gone
When the aorta ruptures, it is blood that escapes rapidly,
 until life can no longer be supported.

So have that CT scan or MRI and follow the size of the aorta! Have it measured and interpreted by expert eyes. Then have open, frank discussion with your doctor about when to replace that most vital of "hoses", the ascending aorta in the chest.

I am forever grateful that the aneurysm in my husband's chest was found, medicine was prescribed to lower the pressure on his weak, bulging tissue, and it was safely and expertly removed before it could hurt him. There were other things - aortic valve failures and infection - that did hurt him, but he never had to experience aortic dissection or rupture. Too many others have suffered this aortic tragedy. Let us hope that the recently published research, Defusing the cardiac time bomb, will truly usher in the dawn of a better day for those with BAV and aneurysm.

Best wishes to all,

Arlys Velebir

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.

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!

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

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