Choosing Doctors

My husband's Subaru WRX STI

We need to choose doctors and our health care with
the same intensity as our cars!

Intensely Interested in Cars

My husband has always been intensely interested in cars. Airplanes too, but his severe nearsighted eyes, let alone his heart murmur, were enough to prevent him from becoming a test pilot. I have always been glad he could not pursue that high risk dream!

He has purchased reference manuals for the cars he has owned over the years. He can tell you minute details about the engine, brakes, tires, and the fluids he uses. No detail is too small to be ignored! He has meticulously maintained his vehicles over the years, and he has the records to prove it. The reward has been the outcome, hundreds of thousands of miles of trouble free driving.

We discovered a real gem, an independent garage that is just great! A visit to them is always a special treat, where there are others as intensely interested in motor vehicles as he is! What a difference from the more typical, often stressful experience, where no one cares very much about the car or the customer, but everyone definitely knows how to present you with numerous charges for parts and labor, whether you are satisfied with the outcome or not.

What About Our Bodies, Our Hearts?

Dr. Marty Makary, in his book Unaccountable, writes about this in Chapter 6, Navigating the System. He also uses the analogy of buying a car. "Second opinions are always possible in America, even when you are admitted to a hospital. When your health is on the line, don't take chances with your life and wellness that you wouldn't even take buying a car." I highly recommend Dr. Makary's book. Much of what he writes, we learned the hard way. We certainly did not start out understanding what we do today.

Second Opinions

If you have followed our story from the beginning, you know that we went from an ER referral list of doctors to an internist, an internist to a cardiologist, and the cardiologist chose the surgeon. We did not seek a second, independent opinion. We were rather like little lambs. I believe one of the reasons was that there was no mystery regarding what was wrong and what the solution was. My husband was also very sick. We trusted the doctors we had met, believing they would have referred us elsewhere if that were necessary. I will have more to say about referrals another time.

While we were waiting, there would have been opportunity for another opinion. Someone who worked with my husband asked him if he would have his surgery at one of the major medical centers in the area. We knew about those centers, but did not consider them seriously. It might have been possible, but there was no real internet presence then, no easy way to research medical centers and physicians online. And some centers and physicians, even to this day, will not accept a patient unless they are referred by another physician. No doubt they have their reasons, but I view this as an obstructive approach that is not pro-patient and simply remove them from my list of options. I would make an exception only if I believed they were the best/only expert, and I must see them. Then I would find a way to be referred.

Checking Out Doctors and Medical Centers 

Dr. Makary writes about transparency in medicine. Transparency has begun to happen and will continue to unfold. The New England Journal of Medicine held a web event recently called "Innovation in Health Care Leadership. Transparency in Quality Data, Pricing, and Medical Records". The announcement includes the words, "Transparency is coming. This is not up for debate." The speakers listed included Dr. Cosgrove, a heart surgeon who heads the Cleveland Clinic.

Yes, things are changing. 

It should become easier and easier

to find out what you need to know,

to make wise decisions about your body. 

You may have many cars over your life time,

 but you will have only one body.

Inside Information

The internet was not there to help us. We went to the local library and looked up the surgeon. We found out that he was trained in Boston, certainly a city well known for heart surgery. We did not know anyone who worked at that hospital, but if we had, we would have asked them questions. We asked our friend, who once was a nurse and knows many people through working in real estate, what she could find out about these doctors. Word came back that the cardiologist had a terrible bedside manner but was thought to be smart, good at what he did. The cardiologist had chosen the surgeon for us, telling us he was the best. I assume he meant the best among the heart surgeons in the group who practiced there.

We Chose the Anesthesiologist!

It amazes me now that the one doctor we chose ourselves was the anesthesiologist. I knew that the anesthesiologist holds life in their hands while the surgeon works. I wanted to know who would hold my husband's life in his hands while that new valve was being installed!

The cardiologist's assistant was very helpful to me, answering practical questions for me during this time. I wanted to know how things would happen, such as pre-surgical testing, hospital admission, etc. One day I asked her about anesthesiologists, and she told me there were several there that worked in heart surgery. I asked her which one she would use, and she was willing to tell me! She told me the name of the one she had chosen when she had her hysterectomy. She had been very pleased. She helped arrange that he would be the one who would hold my husband's life in his hands as the surgeon worked. To this day, both my husband and I have very fond memories of him. I will call him Dr. T.

Time for Surgery

Yes, now it was time for my husband's surgery. We had done what we could to prepare. In all this time, we had never met the surgeon, the man who would open my husband's chest and work on his failing heart.

Today patients and their families arrive very early on the morning of the event, but back then they admitted patients the day before surgery. He was admitted in the afternoon. The anesthesiologist, Dr. T., had already come by and met us for the first time. It was later, towards evening, that we heard heavy footsteps on the hard surface of the hallway floor, getting louder, coming toward us. Thud, thud, thud. . . . They stopped outside my husband's room. Into his room came the heart surgeon. He wore a kind of hard-soled boot that had announced his presence before we saw him. My husband would be his first case in the morning.

It was the first time we had met him.

My husband has had a total of three open heart surgeries. This was the first one, and the last time we would ever allow anyone to perform surgery on him without being truly informed of everything, well in advance.

This is not a criticism of the surgeon. He saved my husband's life. It is a statement of the importance of being well informed, of having all our questions answered, and getting additional opinions.

My husband would never have bought a car this way!

With all best wishes,

- Arlys Velebir

Critical Aortic Stenosis and Heart Failure

"Sunset scene from a little hill . . . ."
                                                                          - Seven Angels by Paul Bernhard Rogers, sung by Sissel

I mentioned that I was not overly afraid of a heart murmur. 

But I was not the one who had grown up in its shadow.
Perhaps only those who live with this knowledge from a young age understand.

My husband was not allowed to play sports with his classmates. He was on the sidelines, as the team manager, when he wanted to be out there playing. Yes, he had lived with the thought through out his life that his heart was less than perfect. But he never let it stop him from living his life. As a young man with BAV who had surgery in his teens told us, "I refuse to live in fear."

For decades my husband had proved that it was entirely possible to live a healthy, active lifestyle. In those years, if he had seen a doctor, which he did not, they might easily have told him not to think about it. He might never need anything done.

But never is a long, long time.

I avoid the words always and never when talking about bicuspid aortic valves.

Now, it could not be denied. His aortic valve was barely opening, and his heart could no longer compensate. He was in what is called congestive heart failure. It affects your whole body.

What is Congestive Heart Failure Like?

It was as though he had aged decades in the wink of an eye. An active, strong man had turned into an elderly shadow of himself almost overnight. With the pneumonia, he had lost between 10 and 20 pounds of muscle. What do I remember about him? He had no strength, no energy. He was short of breath, even at rest, and could not breathe lying flat. Going for a gentle walk on a flat surface, he had to sit down and rest. He had to sit down to tie his shoes; bending over took too much effort.

His face had no color whatsoever. They checked his oxygen levels many times, thinking they would be low. The levels were fine, although he was so pale he looked like he had no blood at all. Our friends who saw him at that time never forgot that deathly looking pallor he had. He was on a diuretic to keep the fluid out of his lungs. Interestingly, although fluid wanted to gather in his lungs, he never had even a hint of swelling in his ankles. He never had any pain (some people do, it is called angina).

I never said it out loud, but icy fingers of fear gripped me. Oh yes, I was afraid now. Was this what happened to someone with a heart murmur? They had told us his heart muscle had distorted itself from working too hard. He now had a boot-shaped heart.

Over the years I have asked others with aortic valve stenosis about their experience, looking for someone whose heart failed like my husband's. I found two of them, both men, similar to his experience. And about 19 years later, his own sister went into heart failure suddenly, just like her brother. Again, there is variation in how each person's body reacts to a narrowed aortic valve.

What the Angiogram Showed

We did not understand the details of it all very well back then. I am looking at the report of his angiogram, written in two parts, by both the cardiologist and radiologist. This test is what the internist told us was the "gold standard" to understand what was going on inside him. Today there are CT angiograms, which may provide enough information without putting a catheter up into the heart.

• His aortic valve narrowing was described as critical, the opening calculated at 0.5 cm squared, and the valve was heavily calcified.
• He had moderate to severe pulmonary hypertension.
• His overworked, thickened left ventricle had global mild to moderate hypokenesis. The muscle was under-active, not squeezing as vigorously as it should. 
• There are two different numbers for his ejection fraction, 30% and 40%, measuring how much blood is "ejected" with each squeeze. Regardless of which was correct, not enough blood was getting through.
• Pressure build up across the valve was 75 mm with a mean of 57 mm.

All of this proved why his body was screaming. His left heart muscle was getting too weak to push his blood out through a narrow, highly calcified valve to circulate through his body. His lungs were under pressure.

There was some good news, his coronary arteries were normal, with no signs of blockage! Of the many bicuspids I have met since, this has usually been the case. It is a great thing to be spared the trouble of clogged arteries of the heart, which is what is usually called heart disease, and the main reason for heart attacks. Bicuspids have enough to cope with, without that also. (However, remember, there are always exceptions!) Each person's body will ultimately tell them and their doctors just who they are in the spectrum of possibilities for those with BAV.

There is one other thing noted in the reports by both the radiologist and the cardiologist: post stenotic dilatation. The cardiologist called it "significant". The radiologist writes it was "of the aortic root". Nothing was ever said to us about this. These many years later, I have learned why. More about this later.

Waiting for Surgery

It seems ridiculous to me now, but he briefly went back to work before he had surgery. The doctors did not suggest that he stay home, and we did not think to ask. In hindsight, we were not thinking very clearly! One day he called me, telling me that he had some kind of "episode". We did not bother to ask, but made the decision right then - no more work until after surgery. One day, still at home waiting, his heart rate jumped up. We called the cardiologist for guidance, who was not concerned unless it went even higher. Thankfully, it calmed down on its own.

I have spoken with three women who suddenly lost their husbands to stenosis of their bicuspid aortic valves. Two of them knew they had it, and were under some level of medical care. Each one of these families have gone through shocking tragedies. It should not have happened to them. All these men needed was surgery. 

No one warned us those many years ago about the risk of sudden death from aortic stenosis. I reflect on where we were at that time, and I realize that my husband too must have been at risk of his heart just stopping. I wonder why quite so much time went by before his surgery, which was scheduled just over 5 weeks after that first echocardiogram. If I could go back to that time today, I would question the delay. 

And so it was high time for surgery. He would not get any better, only continue to decline. It was a relief to at last have him safely admitted to the hospital, the night before surgery. Would his heart recover its strength? Could he come back to being that same strong man in the prime of life once again? That is the next part of the story.

A Cardiology Referral

Spirit of Dana Point sailing off the southern California coast
"Life without love is like a ship without sails" - Yogi Tea

If you started reading at the beginning, 

you know that what seemingly was just a flu bug

 ended up with my husband in the hospital, a very sick man.

 In time we learned his problems stemmed from his aortic valve in his heart. 

Now, the internist was referring us to a cardiologist.

Time for Aortic Valve Replacement

True to what he said when we first met him, the internist ordered that first echo while my husband was in the hospital. He knew it was important to understand my husband's heart murmur. (If it sounded terrible to my ear on his chest, I am sure it must have sent volumes of rather shocking information through a stethoscope into a physician's ear.) 

Now the pneumonia was cleared, but my husband was in congestive heart failure. I promise to tell you what that was like for him, what it can do to a man in his prime, a little later. 

Knowing from the echo that his aortic valve was severely narrowed and calcified, and his left ventricle (the main pumping chamber of the heart) was in trouble, the internist was the first one to tell us that my husband would need his aortic valve replaced. I did know that heart valves could be replaced. My frame of reference was a data point of one, our dear family friend, whose mitral valve had been replaced with a pig's valve. 

It was not hard to accept the idea of needing surgery. My husband definitely had symptoms now! The internist said he would refer us to a cardiologist affiliated with a local hospital where open heart surgery was performed. 

Never Ask a Surgeon if You Need Surgery?!

One thing the internist said to us then left a deeply lasting impression. It also is very telling regarding how the practice of medicine and surgery evolved once open heart surgery became possible. 

We innocently asked why we needed to see a cardiologist. Why wouldn't we go directly to see the surgeon? I will never forget his answer, "Oh, you never ask a surgeon if you need surgery!" It conjured up images of knife wielding doctors in white coats, lurking near operating room doors, luring their innocent victims swiftly inside where they would soon be helplessly unconscious. Our vivid imaginations created those nightmarish scenes, of course. But his meaning was clear. Surgeons like to do surgery and are not to be trusted! 

It is a bias that has apparently developed over time between medicine and surgery. The medical branch should be the "gatekeepers" of patients. 

Years later, when my husband's aneurysm was discovered, we went on our own to see a surgeon. The office was inside a hospital, right on the floor where surgery was done. So many years later, those words still haunted us. We were doing what we had been told was at best very unwise. We were going directly to see a surgeon. Time has proven that for us, in that situation, it was the best thing we could possibly have done! 

What Would the Cardiologist Be Like?

We had only met one cardiologist prior to this, while living on the east coast. He was a wonderful, caring, compassionate man, who took beautiful care of my mother. Mom had just come to visit us when she was stricken one Saturday morning. The ambulance took her to our local hospital, and this man was in the hospital when they arrived. It is still not clear to me what exactly precipitated her crisis. She was on several medications, including a beta blocker. On the monitor in ICU, I remember how slow her heart was, how widely spaced the signals on the monitor. This kind physician accepted my mother as his patient from the moment he met her. He changed all her medications, and eventually discharged her with follow up care. It was many months before she was well enough to travel home, and I have always been grateful to him for saving and extending her life. 

If we had not moved, I am sure when my husband's valve failed we would have turned to him. It never occurred to us to consult him about his heart murmur in those days. After all, he had lived with it since childhood and felt very well.

With that background, we went to see the cardiologist recommended. I don't remember very clearly the nurse or office staff during that visit. An ekg and blood pressure reading were likely done. They had the report from the echo, so already a great deal was known. And by now, my husband looked as sick as he truly was.

Indelible Memories

There are some indelible memories from that office visit. We sat in chairs side by side, across the desk from the cardiologist. I remember it being quite a nice office. There were framed diplomas on the wall. But this man was nothing like my mother's cardiologist. In fairness, this was a practice supporting an apparently busy cardiac program that included open heart surgery. His pager went off, and I remember him speaking briefly on the phone, apparently about an urgent situation.

Born with a Bicuspid Aortic Valve?

The first indelible memory we have is when he told us that my husband had been born with something called a "bicuspid aortic valve". We had never heard of such a thing. Despite my husband's childhood hospitalization, he was sure that his aortic valve was bicuspid. One of the reasons he gave was that BAVs fail at earlier ages. My husband was too young. I tucked those three words, bicuspid aortic valve, away in my own heart, to ponder over later. Had my husband really been born with such an exotic thing? I was inclined not to believe it.

Fixed for Life!

I recognize now that the doctor ran through the typical information about aortic valve stenosis with us. He spoke about replacement options, and said that mechanical valves were used in younger people, as tissue valves would not last very long. The exception was in the case of young women who wanted to have children. They could not take an anticoagulant. Mechanical valves required taking an anticoagulant, coumadin, to prevent blood clots from forming. Mechanical valves lasted essentially forever. So it would be a mechanical valve and coumadin. 

He would be fixed for life!

My husband and I remember different things about that office visit. One thing we both remember. It was when the doctor was talking about the survival statistics for people with aortic stenosis. He said that without surgery, my husband would "terminate" in 18 - 24 months. And then he looked at me and said, "You don't look very happy." 

My husband was in heart failure. We knew surgery was his only hope. To this day, I do not believe that he would have survived any length of time at all without intervention, certainly not as long as those general statistics. Of course, I was not happy. He was talking about the love of my life.

My husband would need an angiogram before surgery. The cardiologist would get in touch with the surgeon. And so we went home.

Checking Back with the Internist

Our internist was a very kind, calm man, easy to talk to, and he had just saved my husband from virulent pneumonia. We were trusting his judgment regarding this cardiologist being the right one for my husband. He described the life of a cardiologist as a tough one.  I remember him telling us about the saying, "Mothers, don't let your sons become cardiologists." 

We asked him why an angiogram was necessary, since the echocardiogram had already proven that surgery was the only answer. He assured us that the angiogram was the "gold standard", and would justify the surgery. It would also reveal any problems with the arteries of my husband's heart.

It revealed something more, documented in the report. Something we were never told about. That is why I tell everyone to get copies of all their reports, and read them yourself.

I will tell you about that later, when I tell you about his aneurysm.

Now, my husband just needed to stay alive and get stronger following the pneumonia, if he could. Then he would have surgery. 

This was almost 25 years ago. Perhaps not so very much has changed for someone still quite young and in trouble, like my husband, because they were born with a bicuspid aortic valve.

With very best wishes to you all,

- Arlys Velebir

Heart Sounds and Bicuspid Aortic Valves - My Beginning

Fall on Palomar Mountain

As I begin this blog, it is fall.
My thoughts go back to another time today.
Sometimes I think it was just yesterday,
but decades have gone by. 
 I am remembering the time when 
I met and married a man with special heart sounds.

 And that was my "heart sounds" beginning.

Loving a Man with a Heart Murmur

When do we lose that invincible feeling of our youth? I do not know, but I do know it is a good thing to have that courageous feeling, that one can face anything, especially together with the love of our life. We might miss out on a great deal of life otherwise.

When we first met, my husband told me that he had a  heart murmur. Over the years he has smiled at me and reminded me that there was full disclosure of his "bad heart" from the beginning.

I, on the other hand, have always known he has a heart of gold.

Is a Heart Murmur Really that Bad?

No, a heart murmur did not frighten me at all. It did not seem "bad" to me. He and his sister were both in the hospital as children, and rheumatic fever was named as the culprit. One of my sisters had that too, leaving her with a little murmur, and to this day it has not affected her. A special friend to our family, a woman, was quite elderly in my eyes (in her 70's!), when her mitral valve was replaced due to rheumatic fever damage. I was sure we would have many blissful decades together before reaching such an exalted age, and by then, who knew what miracles might be possible.

No Signs of Trouble?

Yes, there was full disclosure of his heart murmur. It could not be hidden. My ear to his chest was all that was needed to hear a tremendous rumbling there. Sometimes I look back to our early life together, wondering if there were any signs of what was to come. Like many others born with a "special" aortic valve, he appeared very healthy, took good care of himself, exercised, and was high energy. Even a slight decline would still leave him far beyond "average". (Those born with bicuspid aortic valves are far beyond "average" in many ways, but we had never heard those three words back then.)

A few years went by, and we relocated to a completely new area. I well remember at that time thinking I could not bear to listen to his heart anymore, the roaring was so dramatic. He seemed very healthy still. There seemed to be no reason to visit a doctor. We should have, but we did not know it.

The only hint, a very slight one, was when we were went for a walk, exploring our new neighborhood. It was a hilly area, and coming up a fairly steep incline, he told me he felt something in his chest. Nothing more bothered him, and we did not think any more of it. (Many years later, the same thing would happen with a mechanical valve inside his heart. That is a story for another time.)

All of a Sudden, Big Trouble

It was in the winter, and I was recovering from the flu. I was also about to start a new job, with a new company. When my husband got sick too, we thought he had caught the same bug from me. If only it had been that simple. We did not have a doctor. We were still quite new in the area and had not needed one.

He seemed to get better, but a nasty cough persisted. During that time, someone gave us the name of a doctor. We went. There was no help there at all.

All of a sudden he had terrible, soaring fever. He would shake violently as it rose. This kind of crisis always seems to happen at night and on week ends! We went to the emergency room of the hospital near us. He hated to go, sick as he was. I re-assured him that they likely would not need to keep him, just give him some medicine.

I remember them asking him why he was there. He was muscular, strong, in his prime. Anyone could see he was in good health! (I didn't know then that this is a common problem for those born with bicuspid aortic valves. They generally look very healthy, even when they are not. It is not an advantage in getting the attention of physicians used to seeing obviously sick people.)

They did chest x-rays and blood work. We were told there was a "little pneumonia" in one lung, and given a prescription for an antibiotic. They also gave us a list of local doctors and told us to follow up with one of them.

Searching for Help

The antibiotic from the ER was not helping. On Monday, one of the doctors on the referral list could see him quickly. I am forever grateful for the carefulness of that doctor. He called the hospital and had the x-rays re-read and the blood work reviewed. My husband had full blown pneumonia in both lungs and an astronomically high white blood cell count. He told us he was surprised anyone that sick was allowed to leave the hospital. He prescribed erythromycin to fight the pneumonia, warning us it causes nausea in some people. He also asked my husband about his heart murmur - did he know which valve it was? My husband said no. He gently told us that it is always a good thing to know about the cause of a heart murmur.

Our First Fight for Life

This is the first fight for life that we shared together. The erythromycin was helping, but gave him horrendous nausea. We were told there was only one other antibiotic, if this one did not work. The doctor admitted him to the hospital, where the drug was given by IV initially, until red streaks went up his arm. They found a combination of drugs to fight the side effects so he could take the antibiotic. I remember the kindness of the nurse who let me rest in the other bed in his room. This hospital has a gorgeous ocean view and fantastic food for the patients. It was mostly lost on us.

It is approaching 25 years ago now. I remember it like yesterday. It taught me to respect infection, particularly pneumonia. Jim Henson, creator of the Muppets, died of pneumonia that same year.

My husband had his first echocardiogram in that hospital. At that time, echo machines had sound, and I listened to his heart sounds as the technician worked. The doctor wanted to know more about his murmur. We were not told just then that very likely it was at the root of all his troubles.

He was released from the hospital, a shadow of himself. The bulging muscles had melted away almost overnight. It was a relief to be home, on the road to recovery. Or so I thought. He was not there beside me when I awoke in the morning. 

He was sitting up in a chair, so he could breathe.

The doctor was not surprised when I called. My husband was in full blown congestive heart failure. He was going to need heart valve surgery. The doctor sketched a picture of the heart and its valves, teaching us about the aortic valve and what happens if it does not work well. Blood was not getting through properly. It helped us understand. He sent us to a cardiologist. And in that cardiologist's office, we heard the words "bicuspid aortic valve" for the very first time.

That is the next part of my husband's story. A story for another day.

For now, his story has a simple message: heart murmurs are important to understand, and aortic valve stenosis in those with bicuspid aortic valve may not express itself in the typical way. Don't wait until there is a crisis to find out about your heart. My husband did not have the symptoms they often describe, like shortness of breath or chest pain. Later we would learn that his left ventricle was greatly over taxed, fluid was quietly gathering in his lungs. A person in this condition would be expected to have symptoms. My husband dd not, until rabid pneumonia and a rapid descent into full blown heart failure struck him.

 Coach John Fox also mentioned that he did not have the symptoms he was told about. Here is that interview, which is very enlightening, about his experience with bicuspid aortic valve.

In another interview, Coach Fox talks about his decision to delay his surgery, and the certainty he came close to death on the golf course. 

In future posts, I will tell you what his first heart valve replacement surgery was like.

Until then,

best wishes to those
 with "special" heart valves,

and all who love them,

- Arlys Velebir