Friday, March 8, 2013

Carrie Mettler - A Wonderful Athlete with BAV

Carrie with her husband, Vic
A Wonderful Athlete

During her school years Carrie Mettler was a wonderful athlete. She and her classmates would all have been amazed if they had been told then that Carrie had a condition that would one day threaten her life. Carrie had been born with bicuspid aortic valve disease (BAVD), which is often far from obvious. On the contrary, many of those who have it seem to be models of health and fitness, and in many ways they are. But that picture of health can be dangerously deceptive.

Professional Fire Fighter Training

After graduation Carrie continued her active lifestyle, including running, cycling, skiing, backpacking, and skydiving! Carrie had always dreamed of being a professional firefighter. So at the age of 27, while working as an international customer service representative for a major airfreight company, she joined the Foster City Volunteer Fire Department.  She became a certified EMT during that time, while also holding down her full time airline job.

Carrie had lots of energy and was doing what she loved. A typical day found her working eight hours at the airline, then heading to the fire station where she sometimes remained all night, returning to the airport the next morning.  She was also on the Airport Safety Committee at San Francisco Airport and always found time to fit in her workouts.
Something is Terribly Wrong

Carrie describes this as a very special time in her life. "Everything seemed to be going my way.  After passing written tests, physical agility tests and panel interviews, I was on three different cities' lists to be hired as a full time fire fighter. But then, one day out of the blue, I passed out."

Looking at her, it was hard to believe that anything could be seriously wrong. But this first incident was just the beginning. "These fainting episodes started happening more frequently," Carrie remembers, "usually starting with a feeling of impending doom.  I went through a battery of tests.  But none of them could explain my fainting spells… until one day…I felt as if I was going to die.  I kept passing out and yelled to my roommate.  'Lauren, I think I am going to die.'  I will never forget her face…it was sheet white.  She and her boyfriend took me to the emergency room where they found my pulse at 16 beats per minute.  Once I was given medication to increase my heart rate I was admitted to the CCU for observation.  That evening I felt that terrible feeling again…but even before I could reach for the button to call the nurse they were running into my room with a crash cart.  Being an EMT….I knew something was seriously wrong…then I passed out.  When I came to I was told that my heart had stopped beating for 10 seconds.  The diagnosis was sinus node dysfunction." Carrie received her first pacemaker then, ending her dream of becoming a fire fighter. She was 29 years old.

Carrie, Theresa, and Bicuspid Aortic Valve Disease

A few years later Carrie met and married her husband, moving from the Bay Area to his hometown further inland. She became interested in cardiology and began working in the business office of the practice where she was a "heart" patient.  After taking classes on EKG interpretation she went on to become a cardiology technician.  Then the pacemaker technician job opened up, and Carrie was sent for training. Here she found what she was meant to do. Having a pacemaker in her own chest gave her great empathy for others. It was comforting and encouraging for patients to know that Carrie truly understood. Eventually Carrie was recruited to work for a leading pacemaker company.

One day, Carrie's youngest sister, Theresa, called to say that she had been having heart palpitations. Given Carrie's heart history, a number of tests were done on Theresa also.  They found that Theresa had a bicuspid aortic valve and an aneurysm of the ascending aorta.  Theresa had surgery in the summer of 2000 at the age of 36.  Her aneurysm had quickly ballooned to about 6.0cm.   She received a mechanical valve fitted into a Dacron conduit, replacing both her failed bicuspid aortic valve and dilated aorta.  Subsequently Theresa developed heart block, and she too was given a pacemaker.

Carrie already knew that she had a bicuspid aortic valve, and because of Theresa's aneurysm it was decided to check her aorta also.  There it was….bulging right at the same place as Theresa's.  Carrie's aorta was not as big as her sister's and has remained stable to this day.  But after all she had experienced, including frequent bouts of pericarditis, Carrie and her husband decided that she would retire from a very demanding career and work from home on their Internet Service Provider business.  Stress is known to raise blood pressure and high blood pressure can be lethal when there is a weak artery.

In Search of an Aortic Surgeon and BAVD Expertise
Carrie and Arlys

During one of her office visits in 2002, Carrie's cardiologist showed her an article that he had read in an AHA journal, "Clinical and Pathophysiological Implications of a Bicuspid Aortic Valve".  Even though her aorta was "borderline" in size, her cardiologist suggested a consult with an aortic surgeon, especially since Theresa had already had surgery. Carrie describes what happened next. "I went to a major medical center to be evaluated at their Marfan's clinic, where surgeons specialize in aortic aneurysms.  I did not meet the criteria for Marfan's and actually felt like they wondered why I was there. Lucky for me, prior to this consultation I posted a question on a forum to a group of 'heart specialists', noting the article mentioned above, explaining my history, and asking what they thought of all of this. There was a prompt but conservative answer, and  'Good Luck'. But the most important thing that came from that post was a comment posted by a woman whose husband had already had surgeries for both his bicuspid aortic valve and his ascending aortic aneurysm. She had searched for the article I mentioned, was unable to find it on the web, and was wondering if I could share it with her. This was the beginning of a most treasured friendship."

When Carrie met Arlys Velebir through that BAVD paper in the medical literature, neither of them knew that someday they would find themselves together on the Board of Directors of the Bicuspid Aortic Foundation. The Foundation was in its embryonic stage already; that was the year it began. They did know that day in 2002 that there was very little information about bicuspid aortic valve disease, that it could be life threatening, and that it was important to find expertise. Having only spoken with Arlys on the phone, Carrie traveled to southern California and together they went to Carrie's consultation with Dr. Sharo Raissi, the aortic surgeon who cared for Arlys' husband. For the first time, Carrie got the answers she needed about bicuspid aortic valve disease and her aorta. As Carrie remembers that time, " I have to say that to those I told about flying to meet a woman I had never met in person, staying at her home, and making an appointment with a heart surgeon that she so highly recommended, it sounded at the very least….bizarre!  But this was one of those times…. I knew in my gut, this was the right thing to do.  Everything went without a hitch!  And she was right, this surgeon was the one for me. If and when I need surgery, Dr. Raissi is the one."

Something is Terribly Wrong Again

During the summer of 2003, Carrie developed a ringing in her right ear and started having "funny spells".  She would feel like she might pass out, and then get a strange déjà vu type aura.  Her heart and pacemaker were checked to make sure all was in order, and everything was fine. Around this time, Arlys brought a paper in the medical literature to Carrie's attention. This paper indicated that BAVD also is associated with cerebral aneurysms.  In November of that year Carrie actually lost consciousness. With her local doctor out of town, Carrie went to see the Nurse Practitioner about this most recent frightening episode. She agreed to order a head CT. Carrie forwarded this test to Dr. Raissi in Los Angeles, who asked his neurosurgeon colleague to review it. This neurosurgeon, 

Dr. Wouter Schievink, was the author of the paper on BAVD and cerebral aneurysms!  The test done locally seemed to show that all was normal, but the specialists in LA were not satisfied with it because the test was done without contrast. A test without contrast might detect bleeding, but an aneurysm will not show up. Carrie needed another test, this time with contrast, to check for aneurysms in her brain.

"You Have an Aneurysm Behind Your Left Eye"

Carrie with Dr. Wouter Schievink

Some days are forever seared in the memory; they can never be forgotten. The day Carrie flew to LA to be screened for a brain aneurysm is one of them.  It was a beautiful December morning in LA, sunny and clear, when Arlys met Carrie at LAX.  First on the agenda that day was a CT angio of Carrie's brain, where an intravenous contrast agent would give a clear view of the blood vessels in her head.  Next was an appointment with a neurologist. Due to a power failure there was a delay in getting Carrie's final CT results, but preliminary indications from the neurologist were that all appeared normal.  Carrie's last appointment that day was with the neurosurgeon, Dr. Schievink. Was it really so necessary to see a neurosurgeon after all? But Arlys said, "You are here, and you need to see him."  The experience Arlys and her husband had the year before with his aortic aneurysm was still very fresh. Until they found Dr. Raissi, they did not get the answers they needed. Arlys wanted to be sure that surgical expertise would evaluate the blood vessels in Carrie's brain. At the very least, since Carrie had BAVD, she could explore the potential risks of developing brain aneurysms with this specialist.

Carrie had a return flight out of LAX that same evening, and as they waited to see Dr. Schievink, Arlys and Carrie were thinking it might be tight getting to the airport on time in rush hour traffic. Then the doctor walked in. Carrie says, "I really didn't anticipate the news I was about to receive when the neurosurgeon came in and said, 'YOU HAVE AN ANEURYSM BEHIND YOUR LEFT EYE'.  It was surreal.  I just crumbled…and started to cry…. Surely you must have the wrong patient. But Dr. Schievink had questioned and double-checked the results himself before I had even seen him. It was me, and now I knew I had an aneurysm in my brain as well as my chest." Dr. Schievink held Carrie's hand as he gently told her it was safe for her to fly home that night, that he would reconfirm the findings himself, and call her soon about her treatment plan.

This was exactly one week before Christmas.  Not wanting to worry her family needlessly, Carrie had only told Theresa about this visit to LA. She asked Theresa not to say anything until she spoke to the neurosurgeon again. The next day, Carrie's heart would sink each time the phone rang. Then the call came. Carrie did need surgery.  Although Carrie had lost her father earlier in 2002, she has her Mom, another sister between herself and Theresa in age, and an older brother. Theresa  told them all that Carrie would be having brain surgery soon.

Carrie didn't waste any time.  Surgery was scheduled for January 14, 2004.  Theresa flew out from her home in the Midwest, and Carrie's husband drove them to LA the day prior to surgery.  Carrie's dear friend from northern California came down, and Arlys met them at the hospital early on the morning of surgery.  Carrie's procedure was the second one of the day.  There was a long wait; the first case took longer than planned. Finally Carrie's name was called.

During Carrie's surgery, a clip was placed at the base of her "berry" aneurysm. Carrie says, "I remember my first waking moment…thanking God.  Once I was able to understand…my neurosurgeon spoke with me and told me he found another aneurysm behind the one they knew about."  Yes, Dr. Schievink also took care of another small aneurysm that had been there, too small for the CT scan to detect.

Theresa with Carrie

Today, Carrie speaks of how lucky she is. Lucky…?  Yes, Carrie says that is the only way to feel. Because she has seen the pictures taken during surgery, and she knows how very thin the walls of the aneurysms were.  She knows that without surgery, it was just a matter of time before something would have happened in her brain. In the end, it seems her original symptoms were not related to the aneurysms, but those symptoms prompted the investigation that found them and saved her life.

What is life like for Carrie now? "Frequent echocardiograms and CT scans are part of my care.  For the most part I try not to worry about my BAVD…but I do admit feeling anxious every time a test is done…just hoping there are no changes. Today I continue to stay active. My husband and I enjoy country living amongst the grapevines and cherry trees with our dog, Monty, and our cats. We both have overcome some pretty big obstacles but we still appreciate life and make the most of it…enjoying the little things and taking it day by day."

Carrie's Mom
Carrie's family has had yet another member afflicted by aortic disease. Her Mother was diagnosed with an ascending aortic aneurysm.

The Foundation is in touch with other BAV families who also have members with brain aneurysms. As with many aspects of BAV, much more data and research are needed to understand this more fully. But as Carrie knows so well, it is best to look inside and either rule out or find an aneurysm, in both the head and the chest. It must be high-quality diagnostic testing evaluated by experts, though. Had Carrie accepted the test results done locally, a very different story might be told here now.

Screening for aneurysms in Carrie's brain and the subsequent surgery saved her life in 2004. Ongoing monitoring is important to keep her safe. Carrie is sharing a precious, costly gift, her own experience, with others through the Bicuspid Aortic Foundation. She is sharing her life, hoping to save lives.

2016 Update from Carrie

Carrie's story continues here, Carrie in 2016 - Seeking Opinions for A Complex Situation

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