Sunday, December 25, 2016

The Gift of Pain

Father Prodromos

Father Prodromos has had four surgeries, the most recent one a second repair of his aortic coarctation, which was first treated in childhood. The incision to access this part of the aorta is on the side, underneath the arm. He has lived with chronic pain since that surgery. Here are his reflections on his life now, written on this Christmas Day, December 25, 2016, from the monastery in Cyprus where he lives.

As I see it, one of human’s most common weaknesses is obliviousness. We tend to forget even the greatest gifts life has given us throughout our journey in time. So, it is no wonder that I honestly thank God for an unusual gift I was given by His all-caring and fatherly providence: pain!

Pain really is a mystery. Why some people find themselves in chronic pain after a surgery and some not? Why some people struggle all throughout their lifetime against this uninvited friend and some never even get the chance to meet him? The answer is never to be answered. One needs to find his own, very personal way to get along with the lifelong reality of pain. And for me, St Paul has portrayed the most blessed approach to this phenomenon. He writes:
“Therefore, in order to keep me from becoming conceited, I was given a thorn in my flesh… Three times I pleaded with the Lord to take it away from me. But he said to me, ‘My grace is sufficient for you, for my power is made perfect in weakness.’ Therefore, I will boast all the more gladly about my weaknesses, so that Christ’s power may rest on me. That is why, for Christ’s sake, I delight in weaknesses… in hardships… in difficulties. For when I am weak, then I am strong.”
Mrs Arlys Velebir was the one to correlate this verse by the Apostle to my state. She did that when at my first year’s anniversary after my surgery in 2015 I wrote to her the following about my pain: “every day we all face what our Lord allows to come in our lives, trying to make the best of it for the benefit of our eternal existence: perfect love to God and our neighbour under any circumstances. So, glory be to God for all things!”

Yes, I would forget about that wonderful miracle that took place in the operating room in January 2015. If you read my story on the BAF’s site, you will easily understand that what happened on that day was nothing usual. If I was in the hands of any other surgeon on that day it is really doubtful whether I would be now sitting and writing this. Not to mention that I would have never even made it to surgery. So, I had to remember; I have to always remember. But for this I needed this tiny little thorn. To others such a thorn might feel like a sharp blade. Not to me. To me is a wondrous reminder. It constantly reminds me that gratefulness is human’s most noble expression of love and understanding. I am so very grateful for this friend I was given, to walk along with him the remaining of my life and every single day to acquire the most precious knowledge from my relationship with him: “when I am weak, then I am strong!”

My dear friends, I have undergone 4 open-heart surgeries up until now with the possibility of even more to come. As the second anniversary of my last surgery is approaching, this is my message to all of you for new year: accept pain, every and any kind of pain -spiritual, sentimental, psychological or physical- with one thought: Pain makes us human. And only human can love. Therefore, the more we hurt, the more we love; and love is our destiny.

I trust that since this comes from someone who really knows what pain is, it should then count a little something to you! 

God’s love be with you all. Happy New Year!

Father Prodromos

Read the story of Father Prodromos' redo coarctation surgery experience here. 

Sunday, December 4, 2016

Urgent Call to Support BAV Research

Introducing Some Special BAV Families 
I do not have any pictures of them to share. Perhaps you can imagine them. 
They seemed to have such active, busy lives. Until suddenly, unexpectedly, they died. "Spontaneous deaths" they were called.

When I heard their stories, I cried. 

Why did they die like this?

We are fortunate that someone looked so carefully inside, seeking to answer that question. In their hearts they found something we at the Foundation have come to know all too well, an aortic valve with only two leaflets.
Human Bicuspid
Aortic Valve

Yes, they all were from BAV families.

They reminded me of  many we have met over the years, fellow travelers along the lifelong journey with BAV.

Before going further, let me explain why they are so special. They are families of mice, engaged in research. They live in the Molecular Genetics and Cardiac Regeneration Laboratory at the University of Ottawa.  Please click on the name to visit this laboratory dedicated to understanding our hearts.

Mice? Yes, they are BAV mice, mice with specific, known, genetic abnormalities. They are the first in a laboratory to consistently have bicuspid aortic valves and so strikingly mirror our human experience.

Just like us, these BAV mice families are far from straight forward. Some family members have obvious BAV, some do not. Some suffer tragic complications and premature deaths, others do not.

Our Doctors Simply Do Not Know Enough Today
Today, so little is known that doctors cannot predict very well how severe the consequences will be in the lives of those in BAV families. This is why some of us discover with time that their predictions turn out to be incorrect.

Professor Mona Nemer and the researchers in her laboratory aim to change that! They are learning so much from these wonderful little creatures and their special hearts and bodies.

The most immediate impact from their research will be the identification of biomarkers, predictive tools that can be used to test for and prevent tragedies, to distinguish between those who will have a more normal life and those who face major risks and need individualized, proactive monitoring and care.

Someone Who Understands 
As I listened to Professor Nemer speak recently, I was thrilled to hear a scientifically-based, compassionate discussion that described the BAV challenge so well. I credit these mice for being amazing teachers, representing us so accurately to these also amazing and talented researchers who seek only to understand, and in understanding, help alleviate human suffering. They have just begun to shed light on the answers our doctors so desperately need. This even includes the labile blood pressure issues that plague some BAVers during their lifetimes.

You Can Help Too!

This is the first research effort that the Bicuspid Aortic Foundation has found so compelling that we are directly fundraising in support of it.

Every 12 seconds, somewhere in the world, a child is born with BAV. Today, I have fresh hope that a new day is dawning, and their future, and that of all those living today, need not be so uncertain.

The most powerful thing that we can do today is support Professor Nemer's work.

This holiday season, 
may you know the joy and peace 
that comes to those who give from the heart,
Creating a Climate of Hope.

~ Arlys Velebir
                          Bicuspid Aortic Foundation

Sunday, September 18, 2016

September Awareness - Forever 33 - BAV, Aortic Dissection, Rupture

Doug a few days before his 33rd birthday
Forever 33

Sometimes it seems so cold
 to just write about someone.
 Somehow it feels right to address
 this personally to Doug Grieshop.

Dear Doug,

Tomorrow, September 19th, is your birthday. Can it be 12 years now since you celebrated turning 33 with your family? You should be with them still. Tomorrow you would be 45.  Instead, in your pictures and in loving memories, you are forever that strong, vibrant young man of 33.

We still have the first email Stacey wrote to the Bicuspid Aortic Foundation. The title "My Husband" did not prepare us for those first tragic words "My husband passed away unexpectedly...." It is the message of unspeakable loss that we hope never, ever to receive at the Bicuspid Aortic Foundation. For those who do not know, they can read what happened to you here, Bicuspid Aortic Valve and Aortic Rupture .

Doug's children remember
 Daddy's heavenly birthday
I know that knowledge is powerful, but when I remember you, Doug, I realize just how much power it has. As you took those last steps before you collapsed, you had never heard about bicuspid aortic valves and aneurysms of the aorta. You had never heard of  aortic dissection and rupture in the chest.

It seems so wrong that for months you were fighting something very real that no one understood or named, although it should be well known. Recently Stacey was mentioning what happened once again. She wrote of the multiple specialists you saw. There was a stress test. A chest x-ray, but no CT scan. You were given anti-anxiety medication. Of course, those things did not in any way help unmask the enemy in your chest. It hurts so much to realize that the technology and ability were there. In your own state is a world renowned center for treating this. You just did not know.

Doug, it would have been so wonderful to talk to you on the phone, to meet you in person, as we eventually did with Stacey and your family. We would tell you about your aorta in time, so you could get help.

We cannot change what happened, I know. I just want to tell you that because of you, we have the courage to tell others to persist in getting the help they need, to move on and get other opinions when doctors don't understand.

Doug, you inspire us to save others. 
This is your forever legacy. 

This September, 
we continue
to share knowledge 
Creating a Climate of Hope.

With our love,
Arlys Velebir and all the volunteers
at the Bicuspid Aortic Foundation

Saturday, September 17, 2016

September 2016 Awareness - Learning about Aortic Coarctation

What Did Sarah Have? Coarctation and BAV 
Sarah would be 20 years old now, a young woman. When I spoke to her she was just half that age and facing surgery. Sarah's Mom contacted the Bicuspid Aortic Foundation when they learned that Sarah was going to need surgery. A bright, athletic girl, Sarah began to have headaches. Doctors found she had high blood pressure, and further searching found both a bicuspid aortic valve and coarctation of her aorta. Sarah's Grandfather also had coarctation, they were not sure if had a BAV or not.

Here is a video from Children's Hospital of Cincinnati that explains what coarctation of the aorta is:

I spoke to Sarah's Mom first, and then she asked me if I would speak to Sarah too. What did I say to this beautiful, brave 10 year old girl before her surgery? I told her that she would be just fine, and that she was very special.  I also remember speaking to Sarah after she was out of the hospital. She did indeed do well, very seriously telling me that she was in the hospital for a very long time. If it is an eternity for adults, what must it seem to a child. They all so desperately want to go home.

I have not heard from this family for 10 years now, but I remember them fondly. I hope Sarah as an adult is experiencing all that life should hold for a bright, active young woman. I hope she has wonderful doctors and keeps her check up appointments faithfully, both for her aorta and her BAV.

Father Prodromos and Coarctation

More recently, Father Prodromos sought help from the Bicuspid Aortic Foundation. He was just a tiny tot when he had his first surgery, for coarctation of the aorta. In the intervening years, he has had three additional surgeries: BAV replacement, prosthetic valve and aneurysm replacement, and repeat surgery on the coarctation site.  Father Prodromos' last two surgery experiences are described at the links below.

Father Prodromos - My Journey of the Heart

Father Prodromos - My Journey of the Heart Continues

It is very common for those born with coarctation to also have BAV. May these accounts of those needing help first as children give hope to those today who must cope with this, no matter what their ages.

Sharing our lives
Together We Are
Creating a Climate of Hope.

All Best Wishes,
Arlys Velebir
Bicuspid Aortic Foundation

September Awareness 2016 - Learning About the Aorta

Richard Houchin (right) running along the route of the LA Marathon
following survival of aortic dissection
"Aorta? What's my Aorta?"

 As he lay on a stretcher, helpless and vulnerable, Richard Houchin remembers thinking this as he heard voices around him in the ER talking about him. Something was terribly wrong with his aorta, whatever that was!  Click here for details of what happened to Richard that day.

This was the way Richard learned not only about his aorta, but that he had been born with a bicuspid aortic valve. It is not the way anyone should learn this.One of the goals of this awareness month is to raise public awareness of the aorta, and how aortic disease can be detected before it threatens someone's life.

Following are a series of pages on the Bicuspid Aortic Foundation to help learn about this great blood vessel, truly a river of life in the body.

1. What is the Aorta?

2. What is a Thoracic Aortic Aneurysm?

3. What is Aortic Dissection?

4. What is Aortic Rupture? 

Along with these pages, here is a video from the Nucleus Medical YouTube Channel which describes the thoracic aorta and aortic dissection.

Learning together, we can be prepared to discuss not just our heart, not just our heart valves, but also our aorta with our physicians.

As we learn,
and are prepared to speak
with our doctors, 
we are
Creating a Climate of Hope.

Monday, September 5, 2016

September Awareness 2016 - An Interview with Aorta and Heart

An Interview with Aorta and Heart

The aorta is the large artery seen rising up from the heart,
 with arteries branching upward to the head, then curving down
Two vital members of our body, Aorta and Heart, have agreed to be interviewed for Thoracic Aortic Disease Awareness Month 2016, hoping to set the record straight. They have so much to say, but for now agree to each limit this interview to their top three concerns. Aorta, being less famous, humbly lets Heart start first.

 Heart's Top Three

  1. I am being confused with aortic disease in the chest.  
  2. Aorta, you are so important! If you start bleeding around me, I may stop beating.
  3. If it's not the Heart, check the Aorta!

Let me tell you and everyone, Aorta, being famous is not such a great thing. Have you done a search online lately, Aorta? The headlines scream about "Heart Problems", when you, Aorta, are the one with the problems. In these articles, aneurysm and dissection are mentioned - this is about you, Aorta, not me. How confusing is that! I know we are both in the chest, but in the year 2016 we should know better. There are enough problems that do belong to me, the Heart, without confusing me with aortic disease in the chest too. This is not just bad for my reputation, it is dangerous for the whole Body. We need to be very clear - is it a Heart attack, or is it the Aorta? Sometimes, in death, only an autopsy can tell for sure. I, the Heart, do not want to be blamed for deaths caused by Aorta!

Healthy Aorta
Aorta's Top Three

  1. Nobody knows who I am. 
  2. If they do know who I am, they seem to either not respect me or be terrified of me. 
  3. Please, fix me before I break. 
People don't draw pictures of me, and I don't have a month with valentines, chocolates, and flowers dedicated to me. I guess no one ever thought that love was in their Aorta! Once you get to know me though, I am pretty amazing in my own right. There is just a lot of misunderstanding about me. Those who know me best do respect me and want to help me.

I have no desire to tear inside, or worse yet, fully break open. I'll admit, I'm not easy to work on, but it's going to be a lot worse for everyone involved if I start to bleed.  I'm so sorry that no one knows just when I may finally reach my breaking point. There are some clues, but knowing when to fix me is still a big challenge today. Maybe if I become a little more famous, more research will finally be funded. 

Thank you for giving me a special month of my own. I don't need the chocolates and flowers, just some attention! Please let me be clear, Heart does have a lot of problems. Just don't forget about me, your Aorta.  I hope September Awareness 2016 will help everyone learn more about me!

End of the Interview

The interview ended with Heart and Aorta acknowledging how close they are, special friends in the chest one might say. In health, they work together beautifully. They each just want their separate identities and problems to be understood, respected, and treated. They both deserve help and attention when they are diseased. After all, no one wants to be known as a killer. 

Before they left, Heart and Aorta enthusiastically repeated together, along with the interviewer, 

If it's not the Heart, check the Aorta! 

Learning Together
We are
Creating a Climate
of Hope!
                                           - Bicuspid Aortic Foundation

Wednesday, August 31, 2016

September 2016 Awareness Month - It's All About the "Big A" in the Chest!

Normal Aorta

The aorta is so important,
 we call it the "Big A"! 

To baseball fans in the US, the Big A means Angels Stadium in southern California. At the Bicuspid Aortic Foundation, we mean something else; to us, the Big A is the aorta in the chest. It is the largest artery in our bodies, and it's proper functioning is without question vital to our lives.

Why does the aorta need its own awareness month, September?
Maybe the aorta could just share February with the heart?

Tragedies happen when no one thinks about the aorta. In the emergency room (ER), someone with chest pain will be checked for a heart attack. The blood tests come back normal, so is the EKG. It's not the heart.

No one thinks of the aorta.

The minutes go by, turning into hours. Sometimes the person is still in the ER, sometimes they are back home. Suddenly, they are dead. The opportunity to help them was missed. 

All because no one thought about the aorta.

This is one reason that the aorta needs its own month - just to help everyone think about it in time.

Aorta with aneurysm
Another reason is that aortic disease is very different from what is typically called heart disease. The treatment is different too.

Aortic disease is not heart disease!
If it's not the heart, check the aorta! 

Let's Learn All About the Big A this September!
In a nutshell, this large, high pressure artery should not be confused with the heart. Having its own month provides a time to learn about the aorta in the chest: what it does, the risks aortic disease presents to us, and the treatment available when needed. 

At the Bicuspid Aortic Foundation, we will spend this September featuring articles on the following topics:
  • Thoracic Aorta - what it is, what it does
  • Thoracic Aortic Disease
  • Thoracic Aortic Aneurysm
  • Thoracic Aortic Dissection
  • Thoracic Aortic Coarctation
We will also honor the memory of those who were cruelly taken from us by thoracic aortic disease, and the courage of those who live with injury caused by thoracic aortic disease.

Shining a spotlight on 
the Big A,
Together we are
Learning, Sharing,
Empowering each other,
Creating a Climate of Hope.

Saturday, August 20, 2016

"No Big Deal" - When Will BAV Be Respected?

This child did not require treatment
 until an adult, but that is not always
the case in those with BAV.
The email below was received
by the Bicuspid Aortic Foundation today.

After so many years of trying to raise awareness,
once again we must ask, how much longer
will it take for the bicuspid aortic valve 
to be taken seriously?

"My son was diagnosed with BAV when he was 10 months old, which was 5 years ago.  At that time they basically told us that it was no big deal, and he would just need to get checked every 5 years.  They told us that people go their whole lives not having any issues.

He just had his 5 year follow up Echo with a new cardiologist and his ascending aorta z score is 3.24, which we were told is moderately enlarged.  When the doctor told me this I just started crying.  How does this diagnosis go from no big deal to now his Aorta is enlarged?

The doctor really didn’t tell me much because he didn’t know how fast his aorta is growing since his last echo was 5 years ago.  He basically said to bring him back if he was complaining of chest pain.

We have so many questions and no answers.

I don’t know how concerned I should be, or if I need to restrict him from doing anything.  He is such an active kid, and it is impossible for him to stay still.

I don’t know what it means for his aorta to be enlarged like this at his age.  I have searched the web but I really can’t find a lot of information about children with BAV.  I am just trying to find out everything I can so I have a better understanding of how to help my son.  If there is any information you can give me I would greatly appreciate it."

Tears for Our Children, Our Loved Ones

I understand this Mother's tears. Those of us who volunteer with the Bicuspid Aortic Foundation have heard this too many times and have experienced it in our own families.

It is hard to take in adults, unbearable in a child, to go from "no big deal" to a bulging aorta with an unknown growth rate, to be told to watch for "chest pain"!

BAV May Be a Very "Big Deal" 

We all desperately want to believe that it truly will be "no big deal" in us. That we will go our whole lives without any issues. Yes, some people do.

No one can promise that to someone with BAV. 
Time reveals what BAV means in each person.
What we need to know is what happens in some people with BAV:
  • at some time in their lives, the BAV may leak, narrow, or both and need some kind of repair or replacement
  • BAVers are at greater risk for infection of the heart and BAV 
  • the aorta above the BAV may bulge (dilate) and over time become an aneurysm
We need to hear that we need lifelong follow up care, and that keeping copies of our records will help the doctors we see, who will care for us over many years. This way we give ourselves the best possible life, as knowledge and treatment get even better than today!

This Mom has excellent questions. The Foundation has tried to help her start learning about BAV. There are some physicians and centers that write about their experience with BAV children. We wish there were more who seek to better understand and care for children with BAV.

Here we share one such program for BAV children in Chicago that shares information with the public:   Children's Hospital of Chicago BAV Program .

Here is a page of medical information about children on the BAF website: BAV in Children .

And here are the stories of two little boys born with BAV: Emerson and Lincoln .

Our children
 deserve much more than
"it's no big deal",
much more than 
 being sent away for five years.

Together, we can share information
to help our children and 
their entire families.

Yes, together we are
Creating a Climate of Hope.

          - Arlys Velebir
                                     Bicuspid Aortic Foundation

Saturday, August 13, 2016

NHLBI Strategic Ten Year Vision Includes Aortic Disease!

The anxiously anticipated final strategic vision research topics from the National Heart Lung and Blood Institute (NHLBI)have been announced.

Last year the Bicuspid Aortic Foundation participated in the invitation from the NHLBI for public input into strategic research topics for the next 10 years. Preliminary topics were announced previously. We have now been notified of the final decisions. 

The final version can be found at this link:

At the Bicuspid Aortic Foundation we are very pleased to see that two selections of particular importance to us remain in the final topics for research funding. Below, they are taken directly from the research section of the NHLBI strategic vision website:

Critical ChallengeIn patients with an aortic aneurysm, better tools are needed to determine which patient phenotypes and disease characteristics could best predict who would benefit from a repair. Examples of such tools include animal models that reflect human pathology and biomarkers/molecular imaging tools that are predictive of rupture or dissection.(4.CC.02)
Yes, this is a critical challenge. Sadly, at BAF we know of far too many examples of trauma and death associated with the thoracic aorta. Those who should have surgery before they dissected or ruptured.

Compelling QuestionWhat is the optimal clinical management approach for patients with severe calcific aortic stenosis but with minimal symptoms?(5.CQ.09)

This question must be answered, and it is within reach to do so. It is beyond description to lose the otherwise healthy who have BAV and diagnosed aortic valve disease. This topic speaks directly to what happened to Chuck Doherty. Chuck's family is not the only one we have met who have suffered sudden loss due to this. We would like to see aortic regurgitation addressed also, but this is a start!

Here is Chuck's story:  

In both of these areas, the aortic valve and the aorta, there is treatment available today. It is beyond tragic when that treatment is not offered in time. Those who are granted the privilege of funding to do this research will receive a great trust, to positively impact the timing of elective treatment and prevent untimely injury, suffering, and death. 

At the Bicuspid Aortic Foundation, we will be very interested to see where the research funding is granted and will continue to advocate for meaningful research and treatment options for those with BAV and other "nameless" forms of aortic disease in the chest. 

Together, we continue
 to keep the spotlight
on aortic disease in the chest,
Creating a Climate of Hope.

Saturday, June 4, 2016

Aortic Aneurysm and Dissection Risk from Fluoroquinolone Drugs (Cipro, Levaquin, etc.)

Aortic Aneurysm and Dissection and Fluoroquinolone Drugs 

Raising Awareness - Why?

There have been some additional questions about recent medical papers and the FDA warning about this group of drugs, which the public recognizes by names such as Cipro and Levaquin. Some may have wondered why the Bicuspid Aortic Foundation is raising awareness about this. They may wonder if it has something to do with their heart. It is not the heart, it is the aorta that is getting attention, along with tendons, muscles, and other areas of the body.

To be clear, these warnings are for the general public, for everyone. 
It is helpful to tell everyone about this.

The reason the Bicuspid Aortic Foundation is putting so much emphasis on this is because we advocate for a special group within the public at large, those who already are prone to developing aneurysms of the aorta in their chest.

Quite simply, there are important questions that we must ask:

  • If it puts the general population at risk of aneurysm and dissection, what about those who already have enlarged aortas or full blown aneurysms?
  • Would this group of drugs make already enlarged, bulging aortas even worse, more dangerous, more quickly? 

Medical Papers in 2015  and the aorta.

In November 2015, two papers were published in medical journals by doctors in Taiwan and Toronto, Canada. Then in May 2016, the FDA issued a new warning.

Here is the abstract of the paper from Taiwan:

Risk of Aortic Dissection and Aortic Aneurysm in Patients Taking Oral Fluoroquinolone

Here is the paper from Toronto:
Fluoroquinolones and collagen associated severe adverse events: a longitudinal cohort study

This paper gives details about what happens to the collagen in the aortic wall:

" In the aortic wall, type I and type III are also the dominant forms of collagen,12 thereby suggesting that a medication contributing to tendon ruptures could also lead to aortic aneurysms. Indeed, pathological sections of aortic aneurysms and aortic dissections demonstrate abnormalities of collagen content, concentrations and ratios.13 Although aortic aneurysms typically develop slowly, our data suggest that fluoroquinolone prescriptions can contribute acutely to aneurysm progression and rupture."

Aneurysms Grow and Rupture More Quickly

Please note that the last sentence, which says that these drugs cause the aneurysm to grow quickly "acute .... progression" and then rupture.  So the general thought that aneurysms do not need to be checked that often, because they grow very slowly, may be dangerous for someone who has taken these drugs.

FDA Warning May 2016

Once again, here is the FDA warning from May, 2016:
Here are the first two paragraphs:

"The U.S. Food and Drug Administration is advising that the serious side effects associated with fluoroquinolone antibacterial drugs generally outweigh the benefits for patients with acute sinusitis, acute bronchitis, and uncomplicated urinary tract infections who have other treatment options. For patients with these conditions, fluoroquinolones should be reserved for those who do not have alternative treatment options.
An FDA safety review has shown that fluoroquinolones when used systemically (i.e. tablets, capsules, and injectable) are associated with disabling and potentially permanent serious side effects that can occur together. These side effects can involve the tendons, muscles, joints, nerves, and central nervous system."
While mentioning tendons, which are high in collagen, this statement does not mention the aorta. The papers from Taiwan and Toronto do.

Talking with Doctors

Taken together, these three references help me when talking to doctors about my own family. They help me remember that I should tell them if I or a loved one has ever taken one of these drugs. It also helps me ask for the safest possible drug, if I or my loved one should need an antibiotic. I hope this information helps us all. 

Staying informed together,
Asking our questions,
We are all 
Creating a Climate of Hope.

~ Arlys Velebir
                          Bicuspid Aortic Foundation

Monday, May 30, 2016

Talk to Your Doctor! Fluoroquinolones (Cipro, Levaquin, etc), Collagen Damage Warnings

Classic picture of aortic disease:
 bulging, stretching, curving due to weak connective tissue
There is never a good time to be sick, but why is it that it always seems to happen at the worst time - in this case, a long holiday week end? Yes, this Memorial Day week end my husband's painful left ear sent us to urgent care for help. While there, we had a very important conversation about the right antibiotics for him.

Ear Infection

We don't know why, but this is the second year that my husband has developed ear infection for no apparent reason. He did not suffer with this in childhood, as many children do. Now with an impressive number of birthdays to his credit, what is this all about? He was born with a bicuspid aortic valve, but more than that, there are subtle signs of other "special" things through out his body. No doubt this will move us to investigate further, looking for a reason why this is happening.

Urgent Care

After examining my husband, the doctor explained to us what he saw inside the ear. His stethoscope to my husband's chest had already alerted him to the special sound of the artificial tissue valve in my husband's chest, and he understood the special risk of infection because of that valve. In our experience since the first valve was implanted 26 years ago, this is something that has always been well understood by the doctors he has seen.

"This is in the Cipro family"

As before, both an oral antibiotic and antibiotic drops were suggested. Two choices were mentioned regarding the drops. My husband asked a second time about this. And it was then that the doctor said those words, "This is in the Cipro family"!! I immediately mentioned the recent strengthening of the FDA warning announced on May 12, 2016.  I also said that I had recently discussed this with his aortic disease doctor. We did not want anything from this family of drugs! We settled on the same drops he had received previously, from a "mycin" family, which had not been a problem for him.

Speaking Up

Likely at the pharmacy, but certainly at home, we would have read the labels and caught this. It was so much better to have the discussion directly with the doctor and avoid dealing with it after the fact. It is important to realize that this family of drugs has been available for a long time, and the latest, strongest warning was just issued May 12th. So it is important for us, as the consumers, to be informed and speak up!

My husband is very careful about the medication given to him. Ten years ago, following his last surgery, despite being very unwell in many ways, he asked the name of the medication he was being given in the hospital each time, before taking it. Human beings are fallible, prone to accidentally making mistakes.

Be Informed

Read the May 12th FDA warning.

There is a full paper available to us, published by doctors from Toronto last fall. Those with BAV and other forms of TAD already have issues with the collagen damage/weakness in their aorta and other areas of their bodies. What would taking this drug do to them?

Read it yourself! Print it out! Share it with your doctors!

Fluoroquinolones and collagen associated severe adverse events: a longitudinal cohort study

Sharing information is powerful,
Creating a Climate of Hope.

~ Arlys Velebir
                        Bicuspid Aortic Founation

Thursday, May 12, 2016

What Happened to Amy?

This is Amy's story. Only her name has been changed, to protect privacy.

Where is Amy?

"It's just not like her, Amy would have let us know if she couldn't come!" And so a "welfare check" was requested. Her home was silent. There was no response to the doorbell, the knocks, the calls of her name. Something was terribly wrong!

They found Amy inside her home, lifeless. Apparently there had been no opportunity to even call for help.

What Happened to Amy?

Her family members were notified. The police arranged for her body to be taken directly to a local mortuary. Deeply shocked, her adult children needed answers. Their mother had been an active, independent, and apparently healthy woman. She did not even have high blood pressure. They thought she would be with them for many years to come. How could this happen, without warning?

 They needed answers.

In the place where Amy lived, no one offered to find those answers. After all, there was no evidence of a crime, no villain for the police to pursue. The death was clearly due to "natural causes". But, why? Is it really "natural" to die like this, without warning, when someone is so healthy?

Why Did Amy Die?

Amy's daughter told me that not knowing was unbearable to her family, much worse than the thought of an autopsy would be. Despite their tremendous shock at her sudden death, Amy's family found the strength and the financial resources to request a private autopsy. The local medical examiner was not available, and so Amy's family found their way to someone they describe as special, a compassionate, caring physician who carefully examined their mother's body and helped them understand.  What that physician found inside would have been a gruesome sight.

Quite simply, Amy bled to death. Unknown to her, she had an aortic aneurysm in her chest just above a bicuspid aortic valve in her heart. Because there was an autopsy, Amy's death certificate is accurate, stating that death was caused by the rupture of an aortic aneurysm in the presence of a bicuspid aortic valve (BAV).

Courage to Press for Answers

Sometimes families cannot bear the thought of an autopsy on their loved one.  There also may be veiled opposition, if the death occurs in a hospital, from a medical establishment that fears malpractice lawsuits. Sadly, the days of Dr. M.E. Abbott and Sir William Osler, who learned from autopsies and published extensively about aortic disease in the chest, are gone. As with Amy's family, it is the survivors' legal right to have an autopsy, but they may have to pay for it.

Knowledge - a Gift of Life 

Amy's family was left with the sad, painful task of sorting through her things, closing her home. She was an avid reader, and they found many articles about keeping healthy, preventing disease. Among them may have been the usual warnings about preventing stroke and "heart disease". Like many with BAV, Amy's arteries in her heart were perfectly clean. She was not at risk of a heart attack. Sadly, there was nothing about BAV and aortic aneurysm, the condition that suddenly robbed her of life. Amy would not have even heard about what snatched her life away. If her family had not arranged a private autopsy, the reason for her death and the potential threat to their own lives would never have been known by anyone at all.

Knowledge like this comes at an unspeakable price. It is a price too high, too great to pay. It should not happen.  There is a well known medical center not far from where Amy lived that could have performed her aneurysm surgery. They do this surgery frequently, perhaps almost every day.

Amy's blood relatives are educating themselves, being checked for BAV and/or aortic aneurysm. No one needs to tell them why now.

They know.

The Uncounted

The Bicuspid Aortic Foundation remains extremely concerned about the uncounted deaths due to BAV and aneurysm complications. Amy could easily have been one of those uncounted deaths, if her family had not had the courage and ability to seek the truth. Sudden deaths are too often simply recorded as due to natural causes, perhaps assumed to be due to a heart attack, but without any proof. 

The lack of understanding and recognition of aortic dissection/rupture deaths in the presence of BAV, as well as sudden aortic valve related deaths has consequences not just for those who die, but for the living.

Other blood relatives may be at risk. But there are also consequences for the entire BAV population. Because these people are uncounted, they are not found in the statistics, not included in the medical papers published about patients who receive treatment. How can we understand those most at risk, those who may never even reach a hospital, if we don't learn from them? We don't even know why they died! And so, the statistics upon which treatment of the living is based can not include these unknowns, perhaps the most vulnerable, the most fragile. Must they continue to die?

 Who Will Speak for Me?
Amy began this year, 2016, as we all did, with plans and hopes for her future. They were never to be realized, because she had been born with a bicuspid aortic valve, and over time an aneurysm developed. Today her voice is silenced. Who will speak for her, and the others we lose? 

At the Bicuspid Aortic Foundation we can help give them a  voice and bring into focus these most vulnerable. We are enlisting the aid of pathologists to do so.
They are the physicians who speak for the dead, and in doing so, may save the living. We are also encouraging families where sudden death has occurred to take courage and inspiration from Amy's story, and seek the answers they need.

In giving a voice to Amy,
And others we have lost,
May we help the living
And in their memory, 
Create a Climate of Hope.

- Arlys Velebir

Tuesday, February 2, 2016

BAV Awareness and Benefit Concert, February 27th

Wesley and Heidi Ridenour with their daughter Lochlynn and son Lincoln.
Lincoln's doctor heard a faint murmur at his 3 month checkup,
 leading to the discovery of his BAV.
In the United States, a child with a bicuspid aortic valve (BAV) is born every 7 minutes. Wesley and Heidi Ridenour's two year old son, Lincoln, is one of them. His BAV was discovered when Lincoln's pediatrician heard a faint murmur at his 3 month appointment and sent him for an ultrasound of the heart (called an echocardiogram). This test revealed the aortic valve had only two cusps or leaflets. (Normal aortic valves have three.) Wesley and Heidi are so thankful Lincoln is doing well. He will have his yearly screening again in February.

 On February 27th  at 7:00 pm, Wesley and Heidi will be giving a Benefit Concert at Little Sandy Church of the Nazarene in Bruceton Mills, WV. The talented concert pianist Tatyana Titova Heis will be accompanying, and she will also be playing a few pieces! Wesley will play classical, contemporary, jazz, and electric guitar, and Heidi will be singing.  Kate Heaney will also join Heidi for a duet.

Wesley and Heidi Ridenour are local musicians and teachers in their community. Wesley teaches K-8 at Bruceton School as well as private guitar lessons. Heidi teaches private voice and piano lessons and serves as the Pastor's Associate of Worship at Little Sandy Church of the Nazarene. They love spending time with their two children Lochlynn and Lincoln. They both have Music Education Degrees from Mount Vernon Nazarene University in OH. Wesley earned his Masters in Guitar performance from Bowling Green State University in Ohio. Heidi earned her Masters in Music Education and Kodaly Certificate from Capital Conservatory of Music in Columbus OH.   

We hope that those reading this in the Bruceton Mills area will come out and enjoy an evening featuring a wide variety of music, learn about BAV, and support a worthy cause. Donations will be accepted at the door to benefit  the Bicuspid Aortic Foundation. 

Thank you, Wesley and Heidi,
for raising awareness of BAV,
supporting our mission,
 and helping us
Create a Climate of Hope.

- Arlys Velebir
                         Bicuspid Aortic Foundation

Saturday, January 30, 2016

Carrie in 2016 - Seeking Opinions for a Complex Situation

Carrie Running with Shadow and Stella
Spring 2014

For many with BAV, years may go by with seemingly little or no change. But at some point, it is realized that something was indeed changing inside. It was just that no one, not that person, not their doctors, could detect it. Until one day, whatever was happening can finally be seen. And so it has been for Carrie Mettler.

Carrie in 2014

Carrie first shared her BAV journey with the world here, Carrie Mettler - A Wonderful Athlete with BAV . Beginning with her athletic high school days, through the implantation of a pacemaker in her late 20's, discovery of her BAV and aortic aneurysm, and then her brain aneurysm in late 2003, we read of her amazing triumphs. For the next 10 years after her brain aneurysm surgery in early 2004, Carrie faithfully kept her follow up appointments.  And then it was 2014.

Time for a New Pacemaker

In 2014 Carrie needed to have her pacemaker replaced.  It was decided at that time to upgrade from a single chamber device to a dual chamber model.  They moved the pacemaker from the right side to the left side of her chest, then added two new leads, cutting and capping the old atrial lead.

Carrie tried to resume her normal activity and had difficulty exercising.  It then became difficult for her to even lie flat or bend over.  She felt as if she was being held upside down.  There was obvious neck vein distension along with some facial swelling.  Carrie was sent for a cardiac catheterization, where it was determined that her Superior Vena Cava (SVC) was almost completely blocked.

Carrie Proudly Displaying Ribbons at a Splash Dogs Event
A bulging vein on the left side of her neck is clearly visible.
Carrie underwent surgery to reconstruct her SVC along with the right atrium.  Carrie did remarkably well and was active just 3 weeks post op, walking long distances with her dogs and then running once again a short time later. 

Carrie's Aortic Aneurysm

Then it was time for her next aortic checkup, a routine echocardiogram.  The echo indicted that her aortic aneurysm had enlarged.  Why now, after so many years of being stable? Changes in aortic aneurysms can be unpredictable, which is why it is so important to faithfully keep imaging appointments. 

Could it be due to the increased blood flow after her SVC was repaired?  The surgeon said that the thrombosis and scarring on the lead had been  blocking blood flow for some time. Adding the additional leads caused near complete blockage, giving her the symptoms of Superior Vena Cava Syndrome.  Once this was repaired the blood flow is likely more forceful.

After many years of low blood pressure, Carrie has also had bouts of high blood pressure that have been difficult to control with medication. Could this high pressure also be contributing to a dilating aorta?

Carrie has had some episodes of chest, back and neck discomfort with and without exertion. It is very important to understand what is happening inside.

Carrie in 2016

Carrie, outwardly glowing with health and vitality, is very complex inside. With the encouragement of doctors who have followed her through the years, she is now arranging review of her records by physicians at the Cleveland Clinic. 

Thank you, dear Carrie,
 for sharing your journey with us.

Here at the Bicuspid Aortic Foundation,
 we promise to share each step with you,
 and surround you with a Climate of Hope.

- Arlys Velebir
                         Bicuspid Aortic Foundation