Tuesday, August 2, 2011

Jen Lopez - Familial Thoracic Aortic Disease

Jen and Her Dad - One Family's Story

Jen's High School Graduation
On this happy day, there was no inkling
of Thoracic Aortic Disease and what it would later mean.
Jen Lopez

Jen Lopez was at work, catching up on her email that morning, when she felt the first stirrings of something wrong. Mild at first, her symptoms progressively became worse.  It was hours later, in the ER, that Jen learned what it was. Her descending aorta had torn. The aorta is the largest artery in the body, coming directly out of the heart. The aorta is normally strong and elastic, but if its tissue becomes weak and fragile it is prone to tearing and rupture. There are families in which there are no signs or risk factors for a diseased aorta other than sudden deaths in multiple family members, often at young ages. This condition is called familial TAAD (Thoracic Aortic Aneurysm and Dissection). On the day it sent Jen to the ER, it had already claimed the lives of her Dad and her Grandmother.
Jen's Dad, Louie

In 1995, Jen's Dad, Louie, was 47 years old and in great shape. Outgoing, gregarious, and active, he often was found on the basketball court or the softball field. From all appearances, Louie could reasonably expect to enjoy many more years with his daughter, Jen, and wife, Terry. A successful oldest son, he was the center of his extended family, which included his mother, father, brother and sister and their families.

During a rare doctor's visit in September of that year to stitch up a soft-ball injury, Louie's doctor recommended a chest x-ray based on the fact that Louie had never had one as an adult.  The chest x-ray revealed a 5.6 cm aneurysm in his aorta and a previous Type B dissection in his descending aorta.         
Waiting for Surgery

Louie was referred to a cardiologist who recommended he wait to schedule surgery until the aneurysm had grown to 6.0 cm.  In the meantime, he was put on blood pressure lowering medication and instructed not to lift anything heavy. 

Six months later, while at his office on a Friday morning, he experienced searing pain in his mid-section and abdomen.  Within half an hour he was at the Emergency Room of The Queen's Hospital in Honolulu.  A CT scan showed a Type B re-dissection of the aorta (Louie knew nothing about the original dissection, including when it may have occurred).  This re-dissection went further this time, extending down into the abdomen, and the aneurysm had grown from 5.6 cm to 5.8 cm.  The cardiologist said he could not perform surgery yet because of the new aortic tear.  He wanted to wait at least a week, hoping for some healing during that time. 

Forty-eight hours later, in the hospital, as Louie was talking to the cardiac surgeon, the aneurysm ruptured.  He was rushed to the operating room, where three and a half hours of surgery could not save his life.  An acute Type A dissection of the ascending aorta had occurred concurrently with rupture of the aneurysm. The dissection disrupted blood flow to the brain and caused an extensive heart attack.
Five Months Later, Jen's Grandmother

Five months after her son's funeral, still reeling from this sudden loss, Louie's mother (Jen's grandmother), age 67, passed out on the grass while playing golf.  At the emergency room of a hospital she was pronounced dead.  An autopsy revealed that she too had died from TAAD. Like her son, Louie, her aorta had given way.

And then the family started remembering . . . .

             • Uncle Sonny, Louie's mother's brother. Dead at 53 from a sudden blood vessel bursting.

             • Papa, Louie's mother's father, father also to Uncle Sonny. 
               Dead at 55 reportedly of a burst blood vessel in his head.

             • And now, Louie and his mother.

Early, sudden death in a man, 2 of his 4 children, and his grandson - what was going on? There were no clear answers.  Some form of familial aortic disease was the reason behind these deadly events.

Very special Mother and Daughter
Their sparkling brown eyes give no hint
of all they have been through together.
Jen in 2003

Now we fast forward 7 years to 2003. Jen was living in the Washington, D.C. area, working for the Department of Justice, F.B.I., and traveling about half of each month.  In September she accepted a one-month assignment to Saudi Arabia.  Arriving back home on a Sunday in November, Jen was back in the office at her desk on Monday morning, but not feeling well. 

Thinking perhaps it was heartburn from the daily coffee, she ignored the discomfort, but soon experienced back pain, then, shortness of breath.  After several hours with no relief, her in-office medical personnel called an ambulance, and Jen was on her way to George Washington University Hospital (GWUH).   The pain never let up and the shortness of breath continued while ER personnel worked to understand what was going on. After several hours, a CT scan with contrast was ordered.  It revealed a Type B acute Aortic Dissection.  Jen's first thought on hearing that was, "That can't be right.  That's what my Dad had.  And he died." 

Jen's descending type B aortic dissection started just below the aortic arch and extended thru the abdominal aorta and on down into the left and right iliac arteries.  And, though she had never had high blood pressure before, there in the hospital her blood pressure and heart rate were high and hard to control, even with blood pressure lowering medication delivered by IV. Blood flow to Jen's kidneys was normal, although one kidney was being fed from the dissected portion of aorta.

Type B descending aortic dissections typically can be treated with medicine, not surgery. The initial concern was to control Jen's blood pressure and heart rate to prevent further propagation of the dissection and also prevent compromise of the renal and mesenteric arteries. In type B dissection, the hope is that with lowered blood pressure the dissection will not progress.

Four days after Jen's admission, an aortogram was performed. This procedure has historically been used to visualize the aorta, but is no longer used by aortic physicians, especially in cases such as Jen's. 

Jen stabilized and after 2 weeks in the hospital, she was released with instructions for care and appointments with a renal specialist. 
Chest and Back Pain - Again

Only 22 hours later, after just one night at home in her own bed, Jen was having lunch with her cousin and a friend when she started to feel ill. She collapsed on the sidewalk outside the restaurant with intense chest and back pain.  An ambulance returned her to GWUH, where cardiac catheterization revealed a new dissection, but not in Jen's aorta. 

This time, one of her coronary arteries, the left main, had dissected back to the 'mouth' of the artery. There was no blood flow in the anterior descending portion of the artery.  A surgeon patched the 'mouth' end of the artery with a pericardial patch and used an internal mammary artery to by-pass the anterior descending, restoring blood flow to Jen's heart.  

Heart Attack During Surgery

At some point during surgery, Jen had a myocardial infarction (MI), a heart attack. It was later discovered that it was centered in that lower anterior part of her heart where the blood flow was compromised.  

Jen survived the surgery but her recovery was complicated by congestive heart failure as a result of the MI.  The heart failure eventually necessitated the implantation of a Bi-V ICD (bi-ventricular cardiac synchronization device and defibrillator) in her left chest. 

But Jen is alive!  And over the next 3 years her health stabilizes.

Jen in September 2006

Three years later found Jen living with the dissection in her descending aorta, remaining on blood-pressure lowering medication and a heart medicine to battle the ongoing heart failure.   Because of her youth, the hope was that her heart muscle might yet re-model and grow stronger. Two of the four valves of her heart might need to be replaced eventually. 

Jen and Terry traveled to southern California in September 2006. It was intended to be just a brief visit. In addition to attending Bicuspid Aortic Foundation activities for TAAD Awareness 2006, Jen also had follow up tests and appointments. One of those tests, a 64 slice CT scan, revealed a new development - another dissection, this time in her other coronary artery. When told of the new tear, Jen and Terry knew when it must have happened. On the evening of August 31st Jen felt a slight pain in her chest. Locally, nothing was found. Without the fast camera and high resolution of the relatively new 64 slice CT, this new tear would not have been found. But now the approach to treating Jen had to change. She would not survive another tear in the arteries of her heart. Jen needed a new heart.
Waiting for A New Heart, December 2006

Jen was asked to remain in Los Angeles and undergo evaluation for a heart transplant rather than returning to Hawaii. A trip of a few days turned into months as she remained in southern California, waiting for a new heart. 

December 20, 2006
Jen ready to leave the hospital with her new heart

On the morning of December 20th, the phone rang. There was a heart for Jen. With that call Jen's plans for breakfast with Terry, whose plane from Hawaii had just landed, quickly changed. They would  meet at the hospital instead.

While waiting in a hospital room, someone asked Jen if she was nervous. She calmly said that this was so much easier than before, when she was rushed to the hospital in pain. For Jen, who has survived three dissections, this was the first time she would undergo surgery by choice, not as a life-threatening emergency.

Those who watched this brave young woman wheeled through the doors to surgery saw in that moment the same courage and gentle strength with which Jen has traveled the world, protecting the freedoms of us all.

Waiting on the other side of those doors was a new heart with healthy blood vessels and the skilled hands of the transplant team. Among the hands there to give Jen a new heart were those of the aortic surgeon who had steadfastly championed Jen's cause, determined that Jen and others with TAAD be given every opportunity to triumph over it.  

It seemed very fitting that it was in December, the month of Christmas and also her birthday, that Jen received the gift of life, a new heart. Somewhere, in the midst of their sorrow, may a family be comforted in knowing they have given the precious gift of life to another.  The heart of their loved one has a new home. It beats now in Jen's chest.  

Jen continues to do well today. In the video below, Jen and Dr. Sharo Raissi, who opened many doors of hope for her, tell her story.

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