Saturday, October 18, 2014

Surgery Day - Getting a New Heart Valve

Getting a new heart valve is a special journey.
Just focus on your destination, that high point across the valley.
You will soon be there!

The Kissing Place

Outside the operating room door there is always a special place. The exact location varies in different hospitals, but it is generally as far as you can go with your loved one before surgery. This is the kissing place. I hope no one ever goes into the operating room, which I will call the "OR" from now on, without that expression of love and support. 

This is not a "last kiss".
Don't ever think it!
 It is a "see you soon", 
"I'll be here when you wake up" kiss. 

I had given my husband that kiss and had been shown the small waiting room. This was the place where a nurse would come out and update me from time to time.  I had dear friends with me, standing by us both so loyally that morning. It was very early still. My husband was the first case of the day. I always recommend that if possible. One hopes the surgeon is nice and fresh, well rested, the first thing in the morning! 

Our Special Friend in the OR

Yes, I thought I had gone as far as I could that morning with my husband. But that was not the case. I have written about specially requesting the anesthesiologist, Dr. T. We had met him the day before, and he spent lots of time with us. He took my husband's blood pressure, which was very high. I will always remember his words, "If the pressure is that high in his arm, imagine how high it is in his heart." All because of a severely narrowed, plugged up aortic valve. I was so glad we were within hours of removing that obstacle to freely flowing blood once again.

There is an area where the anesthesiologist and others will do their preparation work before the person goes into the OR itself. In this hospital it was a fairly large room, and Dr. T. invited me to come there and sit beside my husband while he worked on him. There were no other patients, and I have very nice memories of the atmosphere there. Dr. T. was a calm man with a serious, confident manner. He had some sort of board underneath my husband's arm, there to stabilize it as he attached various lines to him. Whatever drugs were going in were definitely relaxing my husband, as he chatted with Dr. T. At one point, he flinched at something, so of course I did too. Dr. T. just looked across at me and said he was almost done! And soon he was. He asked my husband what he thought of his handiwork. Very seriously, my husband moved his arm with all the tubing very close so he could see (no glasses, and he is extremely "blind" without them), and inspected it carefully. He very seriously told Dr. T. it would be fine as long as it all was functional. Ah, those drugs were at work, as he calmly approved the doctor's work!

No Ordinary Days

At some point, others who would work in the OR that day also came into this room. I do not know what their roles were. I distinctly remember them chatting rather lightly about their social lives, sharing pictures with each other. I remember thinking, don't they know what a special, momentous day this is?! How could they be talking about parties?

Of course, surgery was something they did every day, an ordinary day for them.

An ordinary day for me would have found me at my new job. Yes, I had changed companies in the middle of all this. My manager and the company were very kind to me and found a way for me to have the time away I needed for my husband's surgery. Today, my workaday world seemed as far away as another universe.

Somehow I hope that those in medicine, and especially in surgery, never lose a feeling of wonder about what they do. There are others who spend their days at work making heart valves. Others are involved with pacemakers. All these lifesaving devices for the heart. I hope the same for them. The jobs they do can never be just ordinary jobs, ordinary work. Every day is special when you are saving lives.

I Will Take Good Care of Him for You

Now, it really was time for my husband to go through that one last door, into the OR. There I could not go. When Dr. T. told me, "I will take good care of him for you", those were not just words. I knew he meant it. Off they went together, but I knew he went with a friend, Dr. T. He had told us that the surgeon was a good technician. Now it was up to the skill of the surgeon's hands, his eyes, his judgment, to give him a new valve. 

Most of the time, we do not get to know the anesthesiologist very well, but they are a very important member of the surgical team. There are no office appointments with them before or after the surgery. Another time perhaps I will spend more time on their role, and how important they are, although we typically do not know them well at all.

I tell those I meet that surgery day is a good day.
Not an easy day, but a good day.
It is always a good day when life is saved.


Waiting. This is what makes this such a hard day. Your loved one is in that drug-induced sleep, blissfully unaware of what is being done to save their life. But you are wide awake, adrenalin coursing through you, prepared for whatever may come. 

The nurse came out and updated us at key points. Generally, her updates were encouraging, telling me things were going as planned. In my experience, nurses give updates like this, meant to reassure. It is for the surgeon, later, to tell more of the story. 

I remember asking her once how the surgeon was doing, and she smiled and told me he was listening to "cowboy music". Ah, so they have music in the OR. Whatever kind of music helped them do their work was just fine with me! 

My friends encouraged me to walk around a bit, between updates. I did a little, but just could not stray very far from that little room in case they needed to find me. (This was not the day of cell phones, as it is today!)

During this waiting period, a frightening thought dawned on me. They would stop my husband's heart. His heart had been failing. What if it didn't start again? I had never let my mind go there before, but now I waited rather desperately to hear that his heart was beating on its own again.

At last, she told me that the new valve was installed. Then, that his heart was beating on its own once more! Oh what a wonderful heart, to take so much abuse and come back to beat strongly once again!

Let me say now, the heart and the brain both love to heal. They will fight to come back. In these years since, we have proved this more than once. Do not let anyone in medicine discourage you in the fight! Your own body will tell you. You will get better. You can have your life back!

Surgery Ends

I was not a good judge of time that day. I just know it did finally pass. The nurse was done now. The next update would be from the surgeon, and then I could see him.

And then, into the waiting room came our friend, Dr. T. How sweet of him! Other than his kindness, what stays with me now is his description of my husband's valve. He said it was the worst case of calcification on an aortic valve he had ever seen. Describing it, he said the calcification looked like cauliflower. Leave it to my husband to have the very worst! Well, it was gone now. It could not hurt him any more.

Dr. T. left. I have never seen him again. He has a special place in our heart sounds always. May everyone have an anesthesiologist like him.

When the surgeon came, he had more details for me. It is the surgeon's job to tell you everything. Your loved one is his patient first and foremost, although other doctors are involved. The surgeon's name is on that wrist band the patient wears.

He told me that the new mechanical valve fit well into my husband's heart, and that his heart had started up again well. Then he began to tell me about the severe calcification too. He told me that he had been very careful, but there was so much calcification that there was a risk that some of it broke off and got into my husband's blood stream. Any loose particles could either go to his brain, where it would cause a stroke, or travel to his finger tips or toes. The bottom line: my husband might have had a stroke during surgery, and we would not know until he woke up.

Yes, it is the surgeon who tells you both the good and the bad news, after the surgery is over. I wondered why they had not warned us about this before hand. My husband had gone to sleep without being warned of this high risk of stroke.

That First Look

Your loved ones do not see themselves, and even after they wake up, the drugs may still prevent them from remembering those early hours later. It is a good thing for them. But they need you to look at them, touch them, be there when they awake. Don't worry. Your loved one is still there, no matter how they look at that first glimpse.

A nurse took me back to some kind of holding area. I was glad my dear friend, who had years of nursing experience, went with me. I thought it was a terrible place!

Several people were apparently also coming out of various kinds of surgery, all of them lying on some kind of stretchers. This was a recovery area, where they kept patients before moving them to intensive care (ICU) or somewhere else.  My eyes searched, and then I saw him.

He was lying there motionless, on his back. Of course there were many tubes and lines. He could not breathe on his own. What shocked me most was his color. He was a bright red! He had been so terribly pale, no color at all, before surgery. Now rather than just normal color, his skin had the appearance of a boiled lobster. He has had two additional open heart surgeries, and I have never seen that again. I have never had an explanation for it.

This is the time to have a strong, sturdy friend with you. She quickly said in a positive voice, "Oh, look how pink he is now!" Yes, no doubt his blood was flowing much better now. Next they would take him to ICU. I could see him there, about 15 minutes every hour. 

Waiting for Him to Wake Up

My husband's parents were flying that day of his surgery. We had planned it so that they need not endure the hours of waiting with me at the hospital, but would arrive that evening and be with us for whatever would unfold next. Not an easy thing for them to do, traveling to be with this oldest child and first son of theirs, who had lived from childhood with a heart murmur.

Now I wondered how the timing would work, bringing them from the airport. I wanted to be there when my husband first opened his eyes. I especially wanted to see for myself what he would be like. Would there be any signs of a stroke?

At some point, I encountered the cardiologist. He told me that he had seen the valve that was taken out of my husband's heart. The way he said it implied it was in terrible condition, which I already had been told. The one thing I wanted to know, "Was it bicuspid?" "Yes."  There was no doubt now that my husband had been born with an aortic valve with only two leaflets. I believed it now, and never forgot it.

Intensive Care (ICU)

I am not very brave at all. Intensive care is so good for my loved one, I know, but it not easy for me to take those steps and enter there. I do much better once I see my loved one.

My first experience with this was with my father. He had cancer and radiation was tried as one of his last treatments. Aimed at destroying the cancer, it also caused him to bleed suddenly, and he was rushed to ICU. Yes, it was a good setting for him then. Each patient in their own little cubicle, all in a semicircle around a desk area in the center for the nurses. But the gravity of the illness of each patient overshadowed attempts to make it feel close to a pleasant setting.

Now my husband was in ICU, with tubes of various kinds, a machine breathing for him. It was the first time since our marriage that I felt truly separated from him, although I stood there beside the bed and touched him.

I may not be very brave, but I am convinced of the importance of being positive. No one, including myself, shall be negative at all when someone is fighting to reclaim their life after surgery.

From the first moments, before they are awake, I do not want any trace of negativity to come near them. They just need loving reassurance that they are doing wonderfully, that all is well. I have done my best to do this. I need hope myself, and I would never rob another person of that precious lifeline.

It was a time for patience. Waiting for him to come back to me.

And then, there he was! He announced his awakening by setting off loud beeping, blaring alarms to alert the nurse. He was trying to move. There were his eyes, open at last!

We were connected again! I don't remember all I told him. I do remember the intelligent look in his eyes. He could not speak with the respirator tube still in place, but he nodded when I mentioned his parents arriving that evening. He has always had an amazing memory, and at least recent events were still there.

At times like this, you really want to know each other. That unspoken communication is priceless. I was satisfied. I did not need to wait for the doctors to tell me. If there was any damage at all, I thought it would be minor.  I just wanted him to still know and love me! He did!

Later, I was there when the cardiologist came by. He asked my husband to move his legs and wiggle his toes, which he did beautifully! My husband still had the tube in and could not talk, but I read his mind. He was wondering what was wrong with the doctor! The surgery was on his heart, not his legs and feet! He had not been told yet how worried they were about a possible stroke.

God Will Guide the Surgeon's Hands

My initial assessment was right. There was no stroke at all. He just needed to heal and get strong again. Before surgery, my mother told me something very special. She told me that God would guide the surgeon's hands. None of us knew how challenging my husband's situation would be, with the terrible excessive calcification of his valve. I was comforted then, by that thought, and grateful for those hands and how they were guided. If that had not been the case, how different the outcome might have been.

He spent two days in ICU. It should have been just one, that was the goal. But he was not strong enough that first day. I walked in once as they were trying to get him to sit up. "Oh, look, there is your wife. Sit up for her!" His head wobbled on his neck like that of a baby bird, too heavy to hold up!

I had to sign in each time I visited ICU. A volunteer was there with the list of ICU patients. One time, as the volunteer went down the list of names, I noticed what was written down for my husband. His condition was serious! I was shocked. Everyone was being so positive and encouraging. I thought he would at least be considered good!

He had a mechanical valve now. I heard the cardiologist and the surgeon discussing when to start the coumadin that would prevent blood clots from forming on this pristine, new valve. The cardiologist pressed for starting soon. The surgeon did not want any uncontrolled bleeding. They worked it out. My husband cooperated by not bleeding!

Despite any labels used to describe him, he did get stronger, and it was time for him to go out on the floor.

No Private Room Available?!

Having a private room after open heart surgery is so important. Some hospitals have been built in such a way that the rooms are essentially private but can be billed as semi-private. Those are technicalities, but a good thing to understand. We were willing for extra cost to have a private room.

But, this hospital did not have any private rooms open when it was time to leave ICU. His first room on the floor was with an older man who had a terrible cough. I was so upset, afraid that my husband was being exposed to some sort of lung infection right after surgery, and having survived pneumonia himself. They told me that the other patient was not contagious. He had aspirated fluid into his lungs somehow.

A private room did become available soon, and he made good progress. We walked the halls together, working on getting strong enough to go home. Our local internist came every day that he was in the hospital. Much later, I realized that there were never any bills from him for those visits. He told our friend that most of his patients were not as sick as my husband. No doubt he wondered if my husband would survive, and if he did, what he would be like.

No hospital experience is complete without mentioning nurses. They took good care of him. Perhaps that in itself is remarkable. Our later adventures in hospitals cannot be so glowingly described.

Going Home

We were told that after surgery, nothing tastes very good. But I did not have surgery and can vouch that the hospital food was terrible. At home, his mother would be there, cooking for him.  Both his parents were there while I returned to work. It was not easy for his parents, no longer young, to do this. Seeing their son, whose heart murmur must have weighed on them over the years, go through this hurt them too. But they saw him become strong again. We have always been thankful for their loving support. They did not live to see the day when their daughter would have a high risk surgery too.

We were discharged on the 7th day. There would be one follow up with the surgeon. Our local internist was given the job of monitoring my husband's coumadin levels.

This is a long post. Surgery day is a long day! I have shared details here in hopes of giving a glimpse into what is a foreign world for most of us. We enter that world of hospitals, operating rooms, nurses, and doctors only when something is wrong, when we need help. My husband has had two additional surgeries and a stroke since that time. I have gone through surgery with others also. Some I have known well, others not as well. There are heart sounds in those stories, too, still to be shared.

For now, I leave you here, with my husband's heart strongly beating once again, as we resumed our normal life.

We were told he was fixed!
 Fixed for life! 
We believed it.
If only it had been true.

Best wishes to all,
        - Arlys Velebir

Tuesday, October 14, 2014

Life Savers - Heart Valves Made by Man

Starr-Edwards ball and cage valves                                                    
I took this picture on a tour of the valve                                                    
 museum at Edwards Lifesciences                                                      

Thirty years before my husband's crisis, surgeons began implanting artificial valves in place of damaged, failing valves like his. Pictured here are some of those early ball and cage valves. We did not know very much about mechanical heart valves. We did know that there were newer versions with movable leaflets. 

Now that we were meeting the surgeon, we had one very important question to ask him.

Frightening Headlines in the Local Paper about  Bjork-Shiley Valves

While we were waiting for my husband's surgery, some very alarming stories appeared in our local newspaper. Mechanical valves had been breaking inside people's hearts! People were dying. Others were having surgery to replace these valves before they could break.

Imagine having a valve like this in your heart!

Having seen these articles, we wanted to be sure that the surgeon was well aware of any problems with mechanical valves. He was. I realize now that every heart surgeon in the world would be well aware of these problems. I remember him telling us that certain sizes of the Bjork-Shiley valves had the problem. While these valves were no longer allowed to be used, we did not want anything even remotely like this to happen to  my husband!

I also remember being told, perhaps by the cardiologist, that the valve my husband would receive had been tested in the laboratory and proven reliable. It would last far beyond a human life span.

We had no choice. Tissue valves wouldn't last very long. My husband would die, like untold numbers before him who needed help prior to 1960 had done, if his valve was not replaced. And so we placed our trust in the surgeon's judgment regarding the valve he would implant in my husband's heart.

Ultimately, that is what you do when you sign the papers.
 You trust the surgeon with your heart, your life.

Encouragement from Others

I have always been grateful to one of my sisters. She told me about friends of her family who had gone through this. This man had recently had aortic valve surgery and was doing well. He had been offered a tissue valve locally, and had gone elsewhere, to another state, to a surgeon who would implant a mechanical valve instead.

Not long ago I asked her about this man. For him, the mechanical valve implanted about 25 years ago now has worked out very well. He still has it. Perhaps it will be a lifetime solution for him. I believe his heart valve had been damaged by rheumatic fever. We know another man who had his aortic valve replaced also, around the same time. I saw him perhaps a year ago. He too still has his valve. So far, it has served him well. I am so glad when it works out this way. This is the dream we all have.

Yes, this is the dream.
A valve solution that lasts a lifetime.
But too often it remains an elusive one. 

On the eve of his surgery, talking to the surgeon, no one knew that the same valve that would save my husband's life now, would later become the source of a terrible injury to him.

Today, the number of mechanical versus tissue valves being used is an interesting comparison. Tissue valves now far exceed the number of mechanical valves being implanted. In 2011, 16,780 mechanical valves were sold in the United States, compared to 75,734 for tissue valves. There are a number of reasons for this, a topic for another time. (See reference 1 below)

I am remembering the first time, years later, that I heard a surgeon tell someone that there are no perfect valve solutions, and then go on to discuss options in detail. It is a more realistic, much better informed experience than what we experienced so long ago. Today the patient is much more a part of the valve decision making process.

Perhaps it is fair to say there are no perfect solutions,
 but there are certainly good, life-saving ones!

There is a great deal of work going on as I write this,
aimed at new solutions already being made available to high risk individuals.

Saying Goodnight

At 10 pm, the hospital told me that I had to leave. Worse than that, I could not call him later. The hospital switchboard would not accept calls to patient rooms this late. And so I drove the few miles home. Neither of us could sleep! My husband was not to be defeated! I remember the phone ringing, around midnight or was it 1 am? I could not call in, but my husband could call out! After that call, we both slept. The next thing I knew, in the wee hours of the morning I was up and back at the hospital. The nurse was helping him prepare for surgery when I arrived.

Waiting for this day, I remembered how I thought we would be old before he would need anything done to his heart. That had been wrong. Our wedding anniversary would be in the summer. Now, I wanted so much just to have that 10th anniversary together. This was the surgery that would make that possible!

With all best wishes,
- Arlys Velebir

Reference 1 - Aortic Valve and Ascending Aorta Guidelines for Management and Quality Measures

Saturday, October 11, 2014

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

Friday, October 10, 2014

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.

Sunday, October 5, 2014

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

Dear Dr. Reynard

Beauty of the southern California coast,
in the general area of these heart sounds 

My husband's very first echocardiogram,
 and others performed over the years in that hospital,
 were read and the report signed by J.S. Reynard, MD, FACC.

  These are our heart sounds of gratitude to him. 

Dear Dr. Reynard,

We have never met you, but you have a very special place in my husband's heart story. This is written to thank you and to pay tribute to your dedication to understanding and accurately describing the human heart.

At some very critical moments in my husband's life, your accurate assessment of his heart was vital to keeping him alive.

As I write, I am looking at our copy of an echocardiogram report. In a few months, it will be 25 years old. It was  my husband's first echocardiogram. The diagnosis listed is pneumonia. My husband was in the hosptial at that time. This report is the first time anyone ever described the reason for my husband's heart murmur - aortic stenosis.  No doubt you knew, looking at the images of his heart, how sick he was.  Aortic valve replacement surgery saved his life. He did well after surgery, and his left ventricle fully recovered normal function!

The next critical moment in his life was about 11 years later. This time, as you looked at the images, you noted an ascending aortic aneurysm above my husband's prosthetic aortic valve. It was 5.2 cm. He had a CT locally and later an MRI elsewhere. All confirmed your finding and measurement. He had surgery to remove it. A Dacron graft took its place, and continues to serve him well.

Thank you so much for seeing that dangerous bulge. He had no symptoms whatsoever. I fear it would have continued to grow silently until one day it ended in tragedy. I cannot imagine life without him.

We continue to have his echoes done there at the hospital. We have come to know DeAnne Paul well and request that she perform the test each time. She has told us about working closely with you, and how  you mentored her over the years. She has done echoes on a number of our family members now. There is no doubt we continue to benefit today, along with all the others who come to DeAnne needing answers about their hearts.

DeAnne has spoken at Bicuspid Aortic Foundation conferences, and those videos are available to the world. All the wisdom you invested in DeAnne continues today. I am sure she shares her knowledge with the students who are so fortunate to undergo training there. The lifesaving gifts of skilled, dedicated medical professionals go on and on, beyond measure.

As I write, I find there are no words to express our gratitude to you. We just want you to know that you are and will always remain entwined in the heart sounds of our lives.

With gratitude,
The Velebir Family

Saturday, October 4, 2014

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