Thursday, December 26, 2013

Bicuspid Aortic Valve and Life on the Edge

BJ Sanders at Machu Picchu
7,970 feet above sea level

Those born with  BAV are
 typically high energy adventurers.
BAVers live their lives on the edge!
Yes, the view is fantastic.
But an aortic crisis lies
 just over that edge.  
The challenge is to keep them 
far enough from the edge to be safe,
and still allow them to enjoy
active, vibrant lives.
The problem is that no one knows
exactly where the edge is!

My dear friend, BJ, recently shared the story of her path to surgery 
after 10 years of watching and waiting. 
I have been privileged to walk along with her 
during this time in her life. 
Below I share my side of the story.

 Earlier this Year

BJ, Arlys, Carrie Mettler (l to r)
I love this picture from a BAF
Conference a few years ago!
Both of these ladies are pictures
 of health, and expert at throwing
 curve balls at their doctors!
"Good morning Arlys....Since today is Sunday I hate to bother you....only if you get a chance could you give me a call....if not I will try to catch you on Monday....Thanks...BJ"

Uh, oh! I sensed trouble behind that message and quickly called my friend. She told me that she had experienced major chest pain. Immediately Dr. Raissi helped her lower her blood pressure, and she began to arrange for an imaging study locally. (It shouldn't have been difficult, but just getting the testing became a major challenge! Not a good situation for a BAVer with symptoms!)

BJ was still experiencing episodes of chest pain, and had made plans to come to LA for a consult (prepared to stay for surgery) when I received the following:

"Hi Arlys, 
I wanted to share with you that I had another episode of chest pain. It scares me that it could possibly be aortic pain. Now I am actually afraid. It is a terrible feeling. I have not lived in fear for a decade. I am really grateful to be coming to LA.... Love you, BJ"

Now, her pain was happening at rest, with low blood pressure, once even waking her out of sleep!

Arlys to BJ:
"Just in case have a plan to say the right thing to get help there in case of emergency. I texted Dr. Raissi as want him to know."

Arlys to herself: 
"BJ is complicated - delicate tissue, diabetes, partially paralyzed diaphragm, possible blood clotting issues . . .  She has got to get here for surgery! She will not survive surgery back there, especially an emergency! We all love her, and her family needs her so..." 

Arlys to Dr. Raissi:
"BJ had another pain episode lasted about 12 minutes and bp was "normal".... Wonder if ... room for even lower pressure? could she come here now . . . .?" Dr. Raissi adjusted her medication yet again, driving her blood pressure even lower. The chest pain did not reappear in the short time before her surgery. I believe strictly lowering her blood pressure kept BJ back from that dangerous edge of aortic tearing/rupture until she had surgery. 

How close to that edge did BJ come?
No one really knows.

BJ saw a physician after she returned home who told her that she really was on the "low end" of the numbers that are used to guide the surgery decision. He implied that maybe she didn't really need surgery yet. I mention this because those guidelines are for patients without chest pain. BJ had chest pain, and the numbers don't matter when the aorta begins "talking". This paper from Yale discusses this: Symptoms Plus Family History Trump Size in Thoracic Aortic Aneurysm

Would BJ have fallen over the edge if she had met and listened to this kind of thinking in her search for help? Remember, it is easy to be in denial. When physicians do not recognize aortic pain, perhaps labeling it anxiety, how close to the edge do people come? I have heard of some that fell over. 

Surgery At Last

BJ's ascending aneurysm
I arrived very early on the morning of BJ's surgery. We had spoken, but I was anxious to see her. She looked just wonderful, her expressive brown eyes bright and sparkling.  Even I, who know better, marveled at the need for this surgery. She looked so perfectly healthy! Looks are deceiving when it comes to those with BAV. With beautiful skin and vibrant energy, their youthful appearance portrays someone glowing with health. I reminded myself of what was inside her chest, threatening her life. We had to do this!

Pre-Op and Waiting

For years BJ shared the message of Hope with others. 
Now Hope kept her company after surgery!
In pre-op, BJ had her own little cubicle. I was glad for that private time together. She told me she was at peace, and I knew she was. I was so thankful we had safely reached this moment in her journey. We stood on the threshold of the door to her future, engulfed in a Climate of Hope!  

We waited through the hours of her surgery - her husband, Ron, her son, Judd, and I there at the hospital, knowing many others were there in thought with us. BJ has a lovely family, and it was very easy to be there with them. 

Never Trust a Bicuspid!

At some point, "Never Trust a Bicuspid"  has become my motto. Despite efforts to predict what they will do, people with BAV excel in throwing curve balls! Walking in to see BJ that first time after surgery, I was wondering just what surprises she might have in store. 

Dr. Raissi's patients generally look very good after surgery, even in ICU, but I did not quite like my first glimpse of BJ's face. It wasn't long before I knew why, as she threw that first curve. Bright red blood came pouring from her drain tubes! She chose the right time to do this, with her surgeon standing right there to expertly catch it. What would be next?

The next morning, the word initially was good. BJ was off the respirator and sitting up, right on schedule. I decided to wait until afternoon to see her. I should have known better! It wasn't long until I heard from her son, telling me that BJ was going back on the respirator. My heart dropped all the way to my toes!

I had been afraid of problems with BJ's lungs. Some years before, she had been injured during a surgical procedure on her shoulder, resulting in paralysis on one side of her diaphragm. As I drove those miles to the hospital, I tried not to think of the horror stories about respirators and infection.

So what did I find on arrival? Another curve ball! BJ was sitting up, breathing on her own. As long as I have memory, I will never forget that moment. This was one to be thankful for!
Dr Raissi with BJ in ICU

Seconds before they were to put that tube in, BJ somehow turned a corner! Later, Dr. Raissi told us he could see in BJ's eyes that she was better, despite her "numbers", and asked them to wait. Somehow, in that moment physician and patient understood each other, and BJ dodged the respirator! An indescribable moment. 

When we compare memories now, BJ remembers me being there that day, holding her hand. I remember vividly how happy she was to see me walk in. I also remember her saying how tough it had been that morning.  "It was so hard to breathe...."

I waited for more curves -  problems with healing due to her diabetes perhaps. Thankfully that did not materialize. She did manage to find some other bumps on her road to recovery. It was her very own journey, and like many with BAV, she chose her own path! Being under the care of someone used to catching BAV curve balls made all the difference.

I am very grateful as I write this, retracing her BAV journey, that she cleared every hurdle beautifully. I remember watching her walk while still in ICU, and thinking how strong she is. She needed to be.

Who Are these Complex BAVers?

There are variations in those with BAV, and I believe BJ is one of the more complicated. Perhaps what she has deserves its own name, beyond BAV disease, but no one has such a name. The rods that keep her spine straight, her flexible joints, those difficult pregnancies, volatile blood pressure... all tell us her body is special. It concerns me that all these factors may not be taken into consideration in those with BAV. Someday, perhaps, that will change. For now, BJ received the individualized care that her very special body needed. 

How many others need, but do not receive, that care?

In the final few days of 2013, I am happy to share my view of BJ's experience, and to remember with joy my special friend, safely home with her family and friends once again!

Thank you, 
dear BJ,
 for sharing your life 
with us all,
Creating a 
Climate of Hope.

      - Arlys Velebir