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

 

Remembering the Unknown Bicuspid on Fifteen Year Anniversary of Aneurysm Removal, May 30th

In 2001, May 30th was a Wednesday. Early that morning, my husband had surgery to remove the dangerous bulge above his heart. It is a day to remember with thankfulness.

Fifteen years ago, the Memorial Day holiday was two days earlier, on May 28th. We had agreed to do something unusual that day: check into a hospital.
My husband had stopped the coumadin (warfarin) required for his mechanical aortic valve, so that his blood clotting would return to normal. He was to check into the hospital at noon on Memorial Day, the 28th, where he would go on a heparin drip to prevent clots from forming and also have the typical pre-surgery tests.

Memories

We each have our own memories of that experience, but one thing is certain. Today, we remember and remain very thankful for the safe and complete removal of that aneurysm, up to and underneath part of his arch. It is a difficult surgery, and was done flawlessly. Fifteen years later, the repair and the aorta itself remain excellent.

Today, we are most grateful to his own gifted surgeon. We also understand that the pioneering work of aortic surgeons laid the foundation upon which his surgery was possible. The surgery my husband had that day remains perhaps the most complex in the chest, involving completely stopping blood flow at cold temperatures. Only some surgeons have this skill. We still marvel that we found such hands.

The Unknown Bicuspid

There is someone else that I remember today. I do not know his name. His identity is lost, except perhaps to his family. I have searched the medical papers, to see if anything might have been written during that era, that might be about him. I cannot find it.

Today I remember him because, like my husband and many others, one day he went into the hospital to have BAV surgery.

He never came back. He died from a torn aorta. 

It happened sometime after his valve surgery was over, during that first night. The doctors covering that night saw his kidneys weren't working. They did not know why. They did not know his aorta was torn, bleeding inside. The blood flow was cut off from his kidneys, so they could not work. By the time, the next morning, someone figured it out, it was too late. He could not be saved.

Lying there, perhaps the respirator tube still down his throat, helpless, his aorta ripping apart in his chest. It is a horrendous scene. I hope the drugs were strong enough to block the pain.  I hope he did not suffer.

It could have happened to my husband. His records show that his aorta was bulging at the time his BAV was removed. Somehow his aorta withstood the clamps, the cutting and sewing, when his BAV was removed. I don't know why it did.

There was a young surgeon in training then, who saw this happen. He never forgot it. From that day forward, he was to save many of those with BAV from a similar fate. Years later, he removed the aneurysm from my husband's chest on May 30th, 2001.

And so today, I remember this young man, and others like him. They are the Unknown Bicuspids. Some of them died of massive aortic bleeding, sometimes under the mistaken label of a massive heart attack. Some of them died because of complications from their BAV.

Today I remember their lives, cut short fighting aortic disease. 

Yes, we remember these unknown, who lost their fight much too soon. In their memory, those like my husband continue to live, to fight. They are all heart warriors. They have their own battles to fight.

Best wishes to all,

~ Arlys Velebir

Imperfect Heart Valves - Treasure the Ordinary Day

Background


With every beat of his heart, my husband's bovine aortic valve leaflets open and close. He received that valve 10 years ago. Statistics gave us hope then that, given his age, this valve might last about 20 years.

 Statistics are just that, nothing more.

 They are not promises.

 After just 8 years, there was evidence that one leaflet had begun to deteriorate. And we have been told that artificial tissue valves can fail suddenly.

Can We Avoid An Emergency?

Mechanical valve removed from my husband in 2006

We have already been through urgent situations because of his aortic valve. First it was his own BAV that put him abruptly into pneumonia and heart failure.

 Then, 15 years later, it was complications from scar tissue (pannus) and strands on his mechanical valve that injured him, sending particles to his brain.

 Each time, we were blindsided by the suddenness. Now, with this tissue valve showing a problem, is there anything we can do to be better prepared?

Sudden Failure

I am thinking of two people with bovine valves whose symptoms became severe suddenly. They found themselves urgently needing another surgery. One, a woman, had "redo" surgery locally to replace the valve. She was too sick to travel anywhere else. The recovery was not easy, but her life was saved.

 And then there is a young man. He developed severe symptoms, in a place far from the surgeon who performed his first surgery. He had to make a difficult decision. Step on a plane and fly for hours to reach his surgeon again, or entrust himself to whoever was local. He stepped on the plane. I still remember the relief I felt when I knew his plane had landed.

A failed bovine valve that required urgent removal

Knowing this, I wonder, can we know ahead of time, before it is urgent? We have been told that one reason for sudden problems is calcification buildup at the "hinges", the bottom of the leaflets where the tissue bends as the leaflets move. And when the valve leaks (regurgitation, insufficiency) for whatever reason, severe trouble can develop. These are two key things (the hinges, the leakage) that help me understand, removing some of the fear that comes with uncertainty and mystery.

Keeping Watch

When he first received this bovine valve, because of the severity of what happened with his mechanical valve, he was watched closely. Then it was checks just once a year, as things continued to look good. Just when we might begin to think all was well, there was the damaged leaflet!

These last two years, his bovine valve has been watched more closely again. The interpretation of his regular echocardiogram was rather alarming two years ago. A TEE (transesophageal echo, which requires anesthesia) was more re-assuring. The damage to that  leaflet is at the top, not at the hinges!

This leaflet does not move far enough to open as fully as it should, but the other two leaflets do. All three of them close well, so there is no significant leaking.

Another echo followed by a CT scan recently showed the valve about the same. Good news, for now!

Imperfect, Life Extending Solutions

I remember again how we were told 26 years ago that he was fixed for life. Today we know that for my husband, these artificial valves, first a mechanical and then one made of bovine tissue, have been life saving but imperfect solutions.

Coping in Our Three-Foot World

In the book No Hero, Mark Owen writes in chapter three about fear and the three-foot world. It is something he learned in rock climbing training. Not to look down, not to look off in the distance, not even to look for help from others. Focus on the things within reach, things that you can do. We can do that!

In our three-foot world, we do not have to live in fear of things in the distance that no one can control. Here is what we can control:

• living a healthy lifestyle
• monitoring blood pressure and taking blood pressure medication accordingly 
• listening to his body, knowing that even mild symptoms are messengers
• keeping appointments to check on the valve
• learning from information as it emerges about artificial heart valves 

Treasure the Ordinary Day

This thought comes from a Swedish proverb. It would be so easy to forget that every ordinary day  is special, a treasure. For us, a day when, with every heart beat, an imperfect valve opens and closes well enough to make it a perfect day!

With very best wishes to all who,

like we do,

 live each day with an imperfect heart valve.

 May you have many perfect days.

~ Arlys Velebir

Hope for the New Year

 Hope for the New Year

Amaryllis  Fantastica

Special Holidays This Year

This year when asked about plans for the holidays,

 I said that I had very special plans indeed! 

What were they? 

I was spending them with my husband.

Only those who can never do that again, this year or any other, truly understand how special that is. Their lives have been forever changed by the loss of their loved one to bicuspid aortic valve disease  My thoughts go to them, in many states and around the world. How much they would give for just one more moment together.

Flowers for the Holidays - An Impulse Buy

It was an impulse buy. While shopping in November I saw these square boxes with gorgeous flowers pictured on the outside. Amaryllis. I remembered my Mom having this plant around the holidays one year. It had seemed rather exotic to me. I do not have her green thumb and had no idea how much she coddled and coaxed it to produce those huge, gorgeous flowers. But, how I would love to enjoy those beautiful blooms once again!

The instructions seemed simple, and the picture on the box charmed me. Hoping I was not consigning it to a terrible fate, into my shopping cart it went.

The box promised blooms in about 6 weeks. If I started right away, there might be flowers for the holidays. Inside the box were just three things: a rather plain pot, a compact ball of soil, and a dead looking bulb. I followed the instructions. The soil did expand in the pot when water was added, as promised, and into it I placed the bulb.

At first, not much happened. I tried to keep the soil moist but not too wet. But I wondered if this dead looking bulb, partly exposed above the soil, could ever come to life, let alone produce something beautiful.

Then one day, I noticed the bulb was not brown any more. It had a green color to it. Life! Hope came alive in me too.

I'm sorry I don't have pictures of those early changes. I suppose I still did not have very much hope. But then something started to shoot up from the top of the bulb. Slender, green leaves! It was still a long way from beautiful flowers, but I found more hope stirring in my heart.

Maybe it would do better in a different location, with a bit more December sun. I moved it, watched it, and tried to keep the soil moist without drowning it! It was alive, that was clear, but I doubted there would be anything special happening any time soon.

Wonders Over Night

 Then, in just three days, wonders happened!

December 29th

On December 29th the outer casing popped open, with hints of the beauty within. December 30th was an amazing day!

December 30th

December 30th

Just Spending Time Together
And so, we have these holidays now, special, uninterrupted moments together. I find myself clinging to them, wanting to slow the passing of time. Soon they will be over.

The Amaryllis has so beautifully rewarded my rather doubtful efforts, producing beautiful flowers for the new year. In this new year, check ups await him once again. How is his prosthetic aortic valve doing? It needs to be checked soon. Last year, we learned it is not holding up as well as we would like. How much has it changed? His mitral valve tends to leak, but has remained unchanged for several years. He is to have a follow up with an eye specialist, too. We need to make sure nothing can rob him of his vision. Oh, the delicate tissue, the possible complications, for someone born with BAV.

This new year holds heart and aortic check ups for so many. Some face serious decisions about when and where to have surgery. Too often, good answers are hard to find.There are no labels, no names, other than bicuspid aortic valve or thoracic aortic disease, for many with complex issues through out their bodies. Quite simply, too little is known, too little understood.

I cannot bear to think about those so apparently healthy, so talented and active, who are unaware that they are in danger from their BAV or aneurysm. We need ways to find them before tragedy strikes.

May the new year be a fresh start in search of answers.

These are my thoughts on the eve of the New Year. And then, the New Year arrives!

January 1, 2016
Two fully open flowers, two buds

January 2, 2016

The Third Bud Begins to Open
January 2, 2016

 Something in Common - An Amaryllis and BAV??

Watching it change and bloom, I find this amazing plant has reminded me of the BAV experience. After long periods of quietness, BAV may change so quickly, so dramatically.  Doctors call it "latent". One interesting definition of that word is "hidden" or "dormant". For many years, BAV may not express itself. Then, often very quickly, there is important change. A valve needs surgery, an aneurysm is dangerously bulging. My husband's experience has been like that.

It is so hard for those who know, who must watch and wait. Like my Amaryllis bulb, it seems nothing is likely to happen. But then it does. Often then, things move quickly. It is so easy to be in denial.

Can I see the beauty, find the wonder, as life unfolds?

January 2, 2016

My husband has had three surgeries. When he had aneurysm surgery in 2001, I stayed in a nearby hotel until he was out of ICU, when I could be with him at night. That high risk time, right after surgery, is not easy. In that room was a single rose bud. Each evening when I returned to rest, it had opened a little more. Like my husband's recovery, it unfolded beautifully. I am glad I have that memory of beauty and hope from that time. The surgery was a marvelous success. Something I experienced almost 15 years ago still remains as a comforting memory. Today, the Amaryllis reminds me again that I can find beauty, even in the dark times. Beauty unfolds through the help and kindness of others, the repair of the broken, the miracle of healing.

Another Flower Stem Has Appeared
Hope for the Future
January 1, 2016

Yes, new experiences lie before us in this new year. I remind myself, whatever the future holds, we can do this! One step at a time, we can do this.

We can have those check ups, ask our questions, get multiple opinions. We can make decisions.

Along the way, I will look for the beauty in each day. 

So much awaits us all. May the beauty in the promise of help and healing sustain us. Let us focus on hope.


With all best wishes in this new year,

                          -  Arlys Velebir