Sunday, April 20, 2014

Lily's Story - Bicuspid Aortic Valve Stenosis

Broken Heart Lily
 (picture from David's Garden Diary Blog)
I have never met Lily in person.
I have never seen her picture. 
I know the sound of her voice.
And I know her heart.

I will never forget her.

Meeting Lily
Lily contacted the Bicuspid Aortic Foundation one day. She told me that she had severe stenosis (narrowing) of her bicuspid aortic valve (BAV), as well as an aortic aneurysm starting. The narrowing was because over time Lily's bicuspid aortic valve leaflets had become calcified and stiff. They do not open widely any more, and are getting worse.

Yes, Lily was born with a bicuspid aortic valve. But that was not all. Like others with BAV, she entered the world with "delicate tissue". Numerous health issues have surfaced over her lifetime, at least some genetically based. Yes, whatever is underlying it all, Lily has lived with the pronounced effects through out her body. And yet, Lily married and had a family. She has clearly been an active woman with many interests in her life.

Lily sent me a list of her medical conditions. There are over 30!

Yet, of all her many medical conditions, it is this tight, narrow aortic valve that now threatens her life.

Lily's doctors spoke to her of options, but there are not many. She is very high risk for surgery. Lily wanted very much to know about someone else like her, someone who could not have surgery. This is the longing to be understood, to not be so terribly alone, to know someone cares.

Could TAVI Help Lily? (transcatheter aortic valve insertion)
What about the new option of inserting a tissue valve inside an existing calcified aortic valve without opening the chest? It is meant for those, like Lily, who are too high risk for surgery. But in the United States it is not approved for those, also like Lily, who have a bicuspid aortic valve.

Recently, Lily wrote that she was going to investigate a clinical trial for TAVI in those with BAV. She was seen quickly, as her symptoms are getting worse.

Just two days ago, Lily wrote again, explaining that she does not qualify for this either because of the shape of her particular bicuspid aortic valve,

 ". . . I needed to have a tricuspid valve that two flaps had fused together instead of a true bicuspid that is missing one of the three flaps altogether making it a mouth shape instead of a 3 points shape. It needs the extra point to hold it in."  

In BAV, A Disease of the Elderly in the Young 
Aortic stenosis is a terrible scourge on society. One doctor wrote an editorial calling severe aortic stenosis a "neglected malignant disorder". It often is thought of as a condition of the elderly. However, BAV hearts are prone to it at younger ages. BAV is the most common reason for aortic stenosis in those under the age of 65. Usually, the narrowing of the valve is caused by gradual calcification. However, there are some infants and children with BAVs narrowed abnormally by tissue, right from birth.

In the United States alone, statistics showed there were 300,000 individuals in need of surgery for aortic stenosis, but in one year only 80,000 received it. (There is no medicine to cure this.) Without some kind of intervention, the rest will die. Once it reaches a critical point, half are dead within two years. (View the videos in this post from 2011 for more information.)

Broken Heart Lily
A double oriental lily
whose fragrance
 is exceptionally sweet
Lily's Courage
Lily's symptoms are getting worse. She is no longer allowed to drive. She ended her note like this,
"I feel I have exhausted all my options and at peace that I have done all I can do. I am enjoying every day with blessed hope and contentment.
Love n Prayers, Lily"

Lily is one of those thousands in the United States
for whom there is no answer. I have called her Lily, which is not her real name. She is a courageous, strong woman, too young and too beloved to be taken from her family.

In honor of Lily's courage and strength, let us continue to raise awareness and to press for ways to mend beautiful, broken hearts like hers.

Love to you and your family, dear Lily,
Arlys Velebir
Bicuspid Aortic Foundation