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.