TAD Awareness Walk with BJ Sanders - TAVR to the Rescue

BJ Sanders shares her life story
on this virtual walk,
raising awareness of valve-in-valve TAVR

As you walk along with BJ, she begins with her life at age 12. Before your walk is over, she will tell you that today she is counted among those with a valve-in-valve TAVR and a grateful, happy heart!

1966 - A hint of what is to come

At age twelve my connective tissue disorder was emerging in the form of a severe scoliosis requiring six years of orthotics and finally culminating in a surgical procedure, Harrington Rod spinal fusion to straighten my spine at age twenty three. Despite the scoliosis I led a very active and healthy lifestyle!

1976 - Emerging labile hypertension

This is sometimes associated with Bicuspid Aortic Valve (BAV).

1981-1986 - Premature deliveries of my children

Once again connective tissue issues appeared in my three pregnancies. As a result of undiagnosed and misunderstood heart issues, I was placed on long periods of mandatory bed rest. Despite the bed rest, I had three premature deliveries.

1998 – No worries!

Despite multiple connective tissue concerns I had a very full and active life free of worry.

However, at this stage in my life I developed a prolapsed uterus, urinary incontinence and a rectocele all requiring repairs.

2000 – Searching for answers

Arrhythmias and visual concerns led to two echocardiograms, which the internist said were of no concern! Due to financial/insurance concerns, I requested my own copy of my medical records and noted that my aorta appeared to have expanded between echoes. I no longer trusted my internist!

Thus I began my long arduous Bicuspid Aortic Valve (BAV) and Thoracic Ascending Aortic Aneurysm (TAAA) journey.

Over a span of two plus years, I searched earnestly for accurate knowledge to lead me to a physician with medical expertise in BAV and TAAA. There were many obstacles along the way, but the stars aligned the day I met Arlys Velebir in cyberspace. This eventually led to my introduction to Dr. Sharo Raissi, MD and the subsequent formation of the Bicuspid Aortic Foundation.

2002 – Watching and waiting

I entered into the watch and wait period under Dr. Raissi’s expert care. My quest for answers and understanding of my ascending aortic aneurysm and BAV were always met and my fears melted away knowing that I had found the expertise and compassionate care for which I had longed. HOPE had overcome FEAR!

 2013 – Time for Surgery!

Thirteen years into the BAV journey brought unexpected chest pain associated with a significant size increase in my ascending aorta. Dr. Sharo Raissi performed a TAAA reconstruction, a bovine artificial aortic valve replacement due to calcification of my BAV, and closed the left atrial appendage via a clip. Closing the left atrial appendage eliminates it as a potential source of embolic stroke in the future.

When I awoke from the surgery I distinctly remember Dr. Raissi saying, “In 10-15 years when this valve needs replacing, Transcatheter Aortic Valve Replacement (TAVR) should be available in the United States!” He also explained that he put in the largest artificial aortic valve possible for me; so, I could have a future TAVR!  At that moment I was not interested in 10-15 years from then. I had truly been given a new lease on life! After all, I no longer had an ascending aortic aneurysm and I had a brand new healthy aortic valve! I felt indestructible! I believed with all of my being that my new valve would last at least 15 years +! A TAVR seemed like light years away! I didn’t even bother to ask what the acronym stood for!

What I didn’t understand was that data is beginning to reveal that a new tissue surgical aortic valve replacement has a 20% chance of failure within the first 5 years!

In the Valve-in-Valve International Data registry, 20% of patients had their surgical aortic valves replaced within the 5 years prior to the TAVR. It is a bell-shaped curve.  Some patients with transcatheter aortic valves degenerate earlier, and that is true also for those with the surgical valve -  Dr. Raj Makkar Medscape interview March 25, 2019.

2016 - The honeymoon is over so soon???

The fall of 2016 brought the beginning of the end to my honeymoon with my “new” aortic valve. My scan showed the first signs of calcification!

2017 – Just “lazy”!!

2017 revealed further immobility of the “new” valve.

I discounted my increasing fatigue, believing I was just “lazy”.

I was unable to clean my house and became short of breath when making my bed. I avoided steps whenever possible. I developed a rectal prolapse with complications requiring surgery and a subsequent surgical procedure to repair another rectocele.

 2018 – Denial can be dangerous!

2018 brought frequent episodes of angina requiring nitroglycerin, increasing SOB with simple activities, debilitating fatigue. I was in complete “DENIAL” still believing my “new” valve could not possibly be the problem. Dr. Raissi spoke to me by phone and said, “BJ, I am worried about you”. I said, “Don’t worry”. He quickly responded,” I do worry”! I finally heard his concern and boarded a plane.

Happiness after TAVR with my brave, loving husband and son!

Flying from the East Coast to LA, I began to have difficulty breathing. Once again I was in complete “DENIAL”! Once my son and I arrived in LA we walked from our hotel to the Tar Pit Museum in a heat wave! I became faint and needed assistance to sit to prevent me from falling! “DENIAL”! My breathing was so compromised that I required extra pillows during the CT scan to breathe. The CT revealed severe aortic stenosis.

Dr. Raissi called me following my CT scan, and said I should call go immediately to see a TAVR specialist, Dr. Raj Makkar, MD!

No more severe stenosis!
Sweet heart sounds after valve-in-valve TAVR

Disbelief, shock and denial continued in my head… I requested that I visit Dr. Raissi first. He firmly said, “BJ you need an immediate AVR and surgery is too risky with your co-morbitities. Go to Dr. Makkar’s office at Cedar Sinai.” Mercifully, Dr. Makkar was in town and not traveling, and I was given priority and worked into their extremely busy schedule.

07-11-2018 – From grieving over one valve to welcoming another

After extensive testing, insurance approval and TAVR education, reality finally started to sink into my being. To me it was no longer the loss of my “new” surgical valve. I began to sense how fortunate I was to have the stars align once again with a life-saving procedure.

 I was especially grateful for the Sentinel Cerebral Protection System that Dr. Raj Makkar utilizes to reduce stroke risk during a TAVR procedure. One study shows that Sentinel CPS captured visible embolic debris headed toward the brain in 99% of TAVR cases. Studies show more than a 60% reduction in TAVR-related strokes when Sentinel CPS was used noted by the “Society of NeuroInterventional Surgery” and “American Association of Neurological Surgeons”.

I can breathe!

07-13-2018 – After valve-in-valve TAVR “I can breathe….!”

7:00 a.m.-TAVR procedure with the expert hands of Dr. Raj Makkar and his amazing staff!

10:00 a.m.- I awoke to my family and Arlys Velebir by my side!

My first words were, “ I can breathe lying flat!”

07-14-2018 – Out of the hospital 30 hours later!

I was discharged approximately 30 hours from my TAVR once again feeling as if I had a new lease on life!!! I was at the right place at the right time and my stars aligned…truly a miracle!

09-08-2019 – BJ today - Very grateful!!

I become more GRATEFUL each day I have to live. I no longer focus on how long my valve will last or “what ifs”.

BJ (with Dr Makkar)
after TAVR
Happy heart, happy life!!!

As I continue to discover new ways to care for my body, I am finding improved health, vigor and contentment. I no longer eat processed foods and am limiting my carbohydrates which have resulted in a 35 pound weight loss. I was an insulin dependent and insulin resistant diabetic prior to making these changes. I no longer need insulin after 17 years!

I try to live each day by the words Dr. Makkar blessed me with upon my discharge. 

He was very encouraging …

He gifted me with these words….

"Go and Live!…Go to the Beach!…

Travel!…Have Fun!…Live your Life!...

Happy Life!…Happy Heart!!!”

From one BAVer/TAD to another:

Unlike me,

please  be prompt to share your symptoms

 with your trusted physician

 or confidant

 and

 resist the temptation of DENIAL….

May your stars align on your BAV or TAD Journey,

 all the days of your life!

~ BJ Sanders

Reflections on Life - Ordinary Days, Tubes, Pressure, Tears, and Ruptures

Any ordinary day can become a crisis in a just a moment of time. 
Recent research about the reason for weakness of the BAV aorta
reminded me of this experience with my car!

My weak, bulging, torn, ruptured radiator hose!

It was just like any other morning. I pulled into a parking spot and switched off the ignition - an ordinary motion, on an ordinary day. Immediately, to my shock, great white billows emerged from under the hood of my car! Getting out, I could tell it was steam rather than smoke - no fire at least, that was a relief!

Eventually the steam subsided, but not before attracting some attention, while I called my wonderful local mechanic and arranged to transport the car to his garage. When he arrived, the flatbed truck driver looked under the hood and pointed out the likely culprit, a hose. Riding in the cab of that huge flat bed truck, I wondered how bad it was. I knew one thing - I was extremely glad that it happened when it did, parked, and with the engine shut off. What if it had happened while driving in that rush hour traffic - not a safe place to break down?!

The human aorta has three layers - the outer one is very strong.
That strong outer layer can save lives when it holds on long
enough to get to surgery, after the inner layers tear.
I don't know how many layers of materials this hose had,
or how well designed it was to handle pressure. 

I left my car in excellent hands, knowing it would get a thorough check up. Later, the call came, confirming the problem. There was indeed a torn, ruptured main radiator hose. The hose had become weakened and bulged out over time (the clamped ends were still the normal size). I do not know why it chose that precise moment when I shut off the engine to tear and burst. Was there an extra surge of pressure just then? 

Diseased, Bulging Aortas are Dangerous!

When my "car doctor" called to tell me what had been found, it reminded me so much of the way aortic surgeons describe the diseased aorta in the chest - weakened and bulging! 

When the aortic tissue becomes weak and bulges, it is also prone to tearing and rupture. If it tears, causing bleeding in the wall of the aorta, it is called dissection. Quickly getting into surgery can save lives, as long as the outer aortic wall holds. When the aorta completely breaks open, the massive, uncontrolled bleeding is rapid and deadly.

Aortic aneurysm and bicuspid aortic valve

Check Your Hoses - Replace if Necessary!
Unfortunately, it is easier to replace the radiator hose on my car than it is to replace a weak section of aorta inside the chest. However, it can be and is safely done! The best time to do it, like it should have been for my car's radiator hose, is before it tears or breaks. 

Torn and completely ruptured, radiator fluid was soon all gone
When the aorta ruptures, it is blood that escapes rapidly,
 until life can no longer be supported.

So have that CT scan or MRI and follow the size of the aorta! Have it measured and interpreted by expert eyes. Then have open, frank discussion with your doctor about when to replace that most vital of "hoses", the ascending aorta in the chest.

I am forever grateful that the aneurysm in my husband's chest was found, medicine was prescribed to lower the pressure on his weak, bulging tissue, and it was safely and expertly removed before it could hurt him. There were other things - aortic valve failures and infection - that did hurt him, but he never had to experience aortic dissection or rupture. Too many others have suffered this aortic tragedy. Let us hope that the recently published research, Defusing the cardiac time bomb, will truly usher in the dawn of a better day for those with BAV and aneurysm.

Best wishes to all,

Arlys Velebir

More About the Infection Fight - Little Green Caps and Lines in Your Blood Stream (PICC)

It seems I have opened up my Pandora's box of memories, and several things have come tumbling out. 

One of them is my fight for little green caps!!

I will share it here. I hope it helps someone.





I was in a hospital room in LA in February, and there they were. Little green caps! Not spending a lot of time in hospitals, most of us don't fully appreciate some of the things in that setting. I know I didn't. I learned though! Seeing those green caps again brought our life with PICC lines back to me.

When it was clear he would need ongoing administration of vancomycin to fight the infection in his blood and heart, we were told it could be done through a PICC line, at home. (PICC stands for Peripherally Inserted Central Catheter.)

I hated the thought of putting a long plastic tube into his arm and threading it up until the tip was near his heart. I believed he got the methicillin resistant staph epidermidis (MRSE) that way in the first place, through a line put into his blood stream. (Yes, I am finally writing the name, unmasking the monster that eventually killed him.)

For long term medicine delivery into the blood, there was no other way. And vancomycin has to go into the blood - it just cannot be taken by mouth. In addition, the blood levels of vancomycin need to be kept within a certain range in order to be effective. (That struggle is a story for another day!!)

The supplies were delivered by a pharmacy that specializes in PICC line drugs and the "equipment" that goes with it. They deliver directly to the home.

I learned about the little green caps from the first home health nurse we met. In the hospital, they are mandatory. However, what I found out is that the pharmacy may try to provide less than the best to you at home!!! It should not be allowed, especially when you are fighting a killer.

It is important to have a clean, sterile "tip" or port that you connect to when giving medicine, or any other reason the PICC is being accessed. After all, one is trying to fight germs, not introduce more!

In hospitals, they use the little green caps to keep the port sterile. At home, the pharmacy may try to just provide alcohol swabs - no doubt they are cheaper!

Can you believe it? Yes, maybe you can. Saying I was unhappy when I found this out would be an understatement.

I asked the nurse to just order the little green caps, and I remember distinctly what she said. "If I ask, they will tell me that the alcohol swab is sufficient. But if you call and ask, they will send it."

I called!

They had to make a special delivery, just for those green caps. I told them several times, whenever supplies were sent, that we must have the green caps. As I remember, they eventually realized I would not relent, and began automatically sending them.

Just so you know why hospitals use them, the difference they make is well proven:

Use of Disinfection Cap to Reduce Central-Line–Associated Bloodstream Infection and Blood Culture Contamination Among Hematology–Oncology Patients

Here is a page from 3M with information about them

If you are going home with a PICC line and there are unused little green caps in your hospital room, take them with you. You paid for them. And make sure the pharmacy sends them to you, every time.

Someone told me that these caps were invented by the parent of a child with infection. I have not been able to verify if that is the case or not. I am just grateful for them.

I call them "little" green caps. There really are no little things when it comes to infection. Everything matters in the fight for life.

As a reminder, these are my personal experiences and opinions, and not connected in any way to the  Bicuspid Aortic Foundation.

Best wishes to all who read this,

Arlys Velebir

Fire in the Blood, Fire in the Heart - Sepsis and Endocarditis

I miss our walks together on the beach. It can never be again. 

Now I must find my path forward, alone. What should that path be?

In this second year, I am beginning to understand that I should face some of the things that happened to us during that last fight for his life. 

Indirectly it was artificial heart valve issues, but directly, it was sepsis and endocarditis that took him from me. For so long I focused on heart valves and aneurysms. Then infectious demons tortured him and eventually took him from me.

Perhaps these reflections, still almost too painful for me, will lead me to my next step. No doubt, there is is still a fight to wage. Infection is a vicious killer. Surely, we can do better than what happened to him . . . .  

There is a nonprofit, Committee to Reduce Infection Deaths, that among other things provides vital information to help the public regarding hospital-acquired infections, including practical advice for anyone going into the hospital. 

Disclaimer: This is my personal blog, and in no way reflects the position of the Bicuspid Aortic Foundation.




Two years ago at this time, we were so happy together, thinking we were recovering successfully once again following open heart surgery - the fourth one. I remember it now as a blissful interlude, as serene as the beautiful Pacific on a calm day. Suddenly, it would explode into disaster.


Something is Terribly Wrong

Recovering at home after surgery, he began to feel a little off. I noted a rash on his face. I will not share all the details here, but it was the beginning of going into Septic Shock. Overnight, he got extremely ill. By the time I found an ambulance that would transport him back to the hospital where he had surgery, he was conscious but not much more. I sat at his head in the ambulance, thinking I might lose him on that endless journey on the freeway.

We made it to the hospital.

The ER doctor and nurses leaped into action. His kidneys were completely shut down, his body terribly acidic. When they unsuccessfully attempted to insert a central line, I heard his scream of pain through the walls to the far side of the ER waiting room where I sat. I have never seen such horrific suffering.

He was in septic shock, but it was never called that while he was there. They were unable to culture any bacteria from his blood. I remember one nurse telling me that "sepsis patients present like this". She was right.

Along with other things, he was treated with antibiotics as a precaution. Amazingly, his kidneys and general health recovered. Unappreciated, the vancomycin was key to his improvement. We went home again, still without any explanation. 

Mercifully, later he had no memory of the worst of that episode. I remember. Over and over he told me, "I can't go on."  I told him then that he did not need to go on, to fight anymore. I loved him too much to ask him to stay in that ravaged body. Slowly the suffering diminished. I am thankful we had more time, although it was at a very high price to him.

Finally, A Diagnosis

Returning home again, we arranged follow up care with an excellent, caring local doctor. First he developed terrible shoulder pain, the first sign of the resurgence of the infection, although we did not know it. In the doctor's office one afternoon, he had a soaring fever and the tremors we first had seen right after surgery. We were sent directly to the ER of our local hospital, where blood was drawn to culture, and he was admitted and again vancomycin was started.

Fire in his Blood, Fire in his Heart

This time, the blood cultures revealed an antibiotic-resistant bacteria. Vancoymcin was continued, to battle it again, this time knowing it was there. Yes there was a bacterial "fire" in his blood, coursing through his body, potentially contaminating and damaging everywhere it went.

The bacteria was methicillin resistant staphylococcus epidermidis, which is found on the skin.

This villainous bacteria is among those that do not fight fair, not at all. They are particularly attracted to artificial devices in the body. They secrete a kind of slime that is difficult for drugs to penetrate, and embed in the nooks and crannies that are hard to reach. His brand new bovine aortic valve was like a magnet to them.

They did an echo at his bedside in the hospital. When I heard that the new aortic valve showed some damage, I fled his hospital room to cry alone in anguish. A beautiful new bovine aortic valve which he had risked so much to have was being destroyed by infection. I had feared endocarditis all these years. Sometimes our fears do come true.

Why Call This Fire?

I refer to this infection as fire because of how destructive it is, and because of the tremendous  inflammation it causes in the body. 

Over time, even after the septic crisis, many immune markers in his blood were so high they were "off the charts".  If he had lived, chronic joint pain would have remained with him, damage from that "hot" immune response within. This strong man began using a walker to steady himself when the stabbing pains in his low back and hips would strike.

Acquired at the Time of Surgery

The first hours and days after surgery had been difficult, but we did not know why. He told me later that he knew something was terribly wrong the moment he woke up. He felt instinctively he might not make it.

We noticed an involuntary tremor in his shoulder and arm that came and went. Why? Three prior open hearts had never been like this one.

In agony, in ICU he told me he wanted to go to heaven. So soon after successful surgery, I begged him to fight, to stay with me. We had love, prayer, special songs of praise and thankfulness to sing softly, and the comfort of scripture. Having those things, we had everything. It was the doctors who did not have everything they needed, to prevent, to understand, to treat.

After some rough days and nights in ICU, the suffering and tremors subsided. Unknown to anyone, it was the vancomycin given as a precaution following surgery that was working on the bacteria in his bloodstream. As it began to work, he improved. And so, we went home.

Yes, today, I know what happened. An antibiotic resistant bacteria had entered his blood stream at the time of his fourth heart surgery. When? It was present when he awoke in ICU. How? The infection was in his bloodstream, pointing to someone's actions introducing this deadly bacteria through one of the lines inserted directly into his bloodstream. From there it traveled the nutrient-rich super highway of his blood, causing trouble as it would, but particularly attracted to that beautiful new bovine aortic valve.

In ICU after surgery, the anesthesiologist came in and found something not to his liking with one of the lines. I remember the nurse resisting doing anything about it, using an excuse that the line would soon come out, but he insisted that it be corrected immediately.

 Another nurse present made the comment to me that they were "learning something new" from the doctor. I recall that now with cynicism. After all, would they admit to me if they had done something less than properly sterile? Of course not.

Was that how the bacteria got into his blood? There is no way to know, to prove anything after the fact, but I do not forget. He entered the hospital with clean blood. He left with killer bacteria in his blood.

Fighting with Antibiotics - What Could Medicine Do?

After all this, another surgery to change the aortic valve and clean out the infection was too dangerous for him. The fourth surgery had been high risk in and of itself. At this stage in his journey, he would not survive another open heart.

Ultimately, over months we went to the local ER for admission a total of three times. There were PIC lines at home, nurses coming and going. Blood cultures that would be clean, and then later show the infection was present again.

Reviewing what happened, there was a pattern. After about 10 to 14 days without the antibiotics, the bacteria would take control to the point it was obvious, he was sick again. A full body scan showed the infection source was the aortic valve in his heart. The antibiotic had been able to destroy it every place else.

Medicine and Infectious Disease

I was shocked to read that vancomycin is a very old drug. It was the only thing that worked at all. It is the drug of last resort. What happens when and if it does not work either?

After starting with vancomycin, one infectious disease doctor tried a different drug, easier on the kidneys but with less success against the bacteria. How much time and ground was lost trying it, only to return to vancomycin?!

Finally, after he was admitted yet again for another PIC line to deliver further antibiotic treatment, suddenly in the afternoon his pulse dropped dramatically. The infection had "taken out" the AV node near his aortic valve. After that, the only reason he was alive at all was because of a "trickle" electrical impulse getting through to the left ventricle, telling it to squeeze. I thank God for that design, because it kept his heart beating long enough for him to return home for his last hours, his dearest wish.

I still find it strange that on Monday morning, as we were arranging to take him home, an infectious disease doctor I had never seen came in and spoke of trying different drugs. Where had he been all this time? Hadn't he read the chart or spoken with our doctor first?  Why put us through the anguish of that conversation? We had already made the decision to go home and cherish our last hours together. And that is what we did.

Playing with Fire?

Endocarditis has been known to attack those born with BAV for a very long time (I love your published work so long ago, Dr. Abbott! I wish they would quote you more often!) . When surgery and artificial heart valves came along, endocarditis was recognized as a risk with artificial valves.

Today, there is a debate about whether or not antibiotics should be given when certain invasive procedures are done that may introduce bacteria into the blood stream. At one time, antibiotics were given to those with BAV. Currently the guidance is to use antibiotics only for those with artificial heart valves.

This greatly troubles me. I recently read this paper.  Risk and outcomes of aortic valve endocarditis among patients with bicuspid and tricuspid aortic valves  

From the paper, I have bolded some words (IE is infective endocarditis):

"This study does not provide guidance on the use of antibiotics in patients with BAV. However, we did observe a very high risk of IE in patients with BAV, thus establishing their potential value as a target population for randomised trials of prophylactic antibiotics in procedures prone to bacteraemia. The recent change in guidelines was largely motivated by the attempt to provide antibiotic prophylaxis to the highest-risk patients undergoing the highest-risk procedures. Although to this day, there is no randomised controlled study that tested the efficacy of antibiotic prophylaxis, BAV clearly increases the risk of IE in the otherwise healthy patient population. Our study suggests re-examination of congenital valvular heart disease among risk groups but does not provide any information regarding prophylactic administration of antibiotics prior to at-risk procedures. Patients with BAV should be encouraged to report symptoms of IE early to potentially reduce the risk of aortic root abscess. This study also highlights the risk of IE from intravenous drug use in patients with BAV."

What would I personally do if I had a bicuspid aortic valve?   I would talk to my dentists and doctors, frankly and honestly, and ask for preventive antibiotics before procedures.  I would never wish to "play with fire".  I have seen the destruction, when infection does its worst. It is relentless, right to the last breath.

Infection and the Future
 Are we doomed to return to the days when bacterial infections killed so many? Where is the next generation of drugs, that will defeat these aggressive bacteria? These are questions I ponder. Perhaps that is part of my path forward, focusing on those in search of the answers.

Gazing at the Sunset

Until now it has been too painful for me to think about, to remember, these things. It hurts to face the reality that this deadly bacteria present on someone's skin killed him, as expressed by one of his doctors, "because someone didn't wash their hands." Perhaps writing this is the next step in my personal journey forward, advocating for those attacked by infections.

Now, it is time for me to stop gazing at the sunset. 

Just beyond the horizon is tomorrow, an opportunity to make a difference.

 

Originally posted in 2019, I have updated and republished this again on 04/05/2021.

Best wishes to all who read this,

Arlys Velebir

Speaking Up about BAV - Find Your Voice!

Red Jasper
The "warrior" stone

I spent many years, from 2001 onward, reading and questioning, devouring everything I could find about those born with bicuspid aortic valves.  Initially it was trying to understand how someone supposedly "fixed for life" by aortic valve replacement surgery 11 years earlier had somehow developed a large ascending aortic aneurysm.

The reading and questioning no doubt made a difference. I know we avoided some pitfalls. Others ensnared us, diminishing our quality of life.

Fluoroquinolone Warning
Today I am moved to write and encourage BAV individuals and families to find their voice and speak with their doctors about medications after reading the December 20, 2018 FDA warning about fluoroquinolone drugs. The following is from the announcement:

 "Health care professionals should avoid prescribing fluoroquinolone antibiotics to patients who have an aortic aneurysm or are at risk for an aortic aneurysm, such as patients with peripheral atherosclerotic vascular diseases, hypertension, certain genetic conditions such as Marfan syndrome and Ehlers-Danlos syndrome, and elderly patients.  Prescribe fluoroquinolones to these patients only when no other treatment options are available.  Advise all patients to seek immediate medical treatment for any symptoms associated with aortic aneurysm. "

The large population (1 in 50) of those with BAV, known to be vulnerable to aortic aneurysm in the chest, is not listed as one of the patient examples. This leaves something for us, the patient and family members, to do. We can speak up and mention it to our doctors.

From reading, I knew that the medical literature had begun to report on this class of drugs and the risk to the aorta. Two different times, with two different doctors, I told them about this and asked that an antibiotic from a different group of drugs be given to my husband. The doctors were not aware of the information in the literature, but they listened and found another option. This was well before there were any official warnings. I will always be glad we avoided any potential complications - he already had more than his share of issues without anything more.

Blood Pressure Medications
Recently letters have come from a pharmacy addressed to my late husband, warning of contamination of blood pressure medications he had been taking. He is still in the pharmacy database. I was aware of this long before those letters arrived and would have taken steps to protect him if he were still alive. 

Ultimately, I lost him to a raging infection in his heart. I cannot begin to tell you how much it still hurts. I can tell you that it would hurt even more if we had not done what we could all those years to protect him.

I often say that those with BAV are warriors. For those still in the battle, please read, ask questions, and speak up for yourselves. It could make all the difference in the quality and length of your life! 

With very best wishes

 for peace and joy 

this holiday season,

 and a safe, healthy 2019

~Arlys Velebir

Thanksgiving Morning - Heart Sounds of Love

A favorite picture of Peter Timneh!

This Thanksgiving Day morning, with the help of my sweet feathered child, I find there is a continuing story of love and thankfulness to share. These are the heart sounds of love between an African Grey parrot and his human Mom, that I have to share this Thanksgiving. 



The two of us have gone through some long, sad days and nights together in the last year, after parting with our beloved "Daddy Bird".

Today we are both thankful to be together, to be alive!

And we are thankful for everyone that make our lives so very special.

Another Fight to Live - The Unthinkable Has Happened!
The setting today in our home is a familiar one. There is very special medication in the refrigerator, which must be given properly and on time, twice a day. Medication that is fighting a terrible infection.
In November 2017 we lost the fight with infection - sepsis and endocarditis - that parted us from our beloved "Daddy Bird". There was no more need for medication, nurses, and so many things that were part of that fight.

This year, the medication is for my beloved forever child, and I am the nurse. He is, of course, the most beautiful, intelligent, African Grey Timneh ever hatched into this world! Above all, he is the most loving creature on earth!

Those who do not experience it may find it difficult to understand the beautiful bond between an African Grey parrot and his human family. I can only tell you that no one could love more intensely - it is impossible. Perhaps we can aspire to equal it. In the wild, the bond between these bird pairs, as well as the greater flock behaviors that enable them to live and thrive, must be truly magnificent.

Peter Timneh came to live with us when he was almost two. His first home had become a disaster, and it took a long time, years, to undo the damage and completely trust each other. African Greys are highly intelligent and intuitive. They are not cuddly creatures, but we found our way to a deep and enduring bond of respect and love. I remember how thrilled I was when he snuggled his head into my hand the first time.

Another Fight with Infection

State of the Art Avian Clinic
Dr. Larry Nemetz

There is an avian specialist in this area, one of the few in the country, Dr. Larry Nemetz. We had been to him once for help early on, but over the years all had seemed well. In late October, I called for an appointment for a check up, thinking something was a little off, but not serious. However, a few days later, I called urgently - something was terribly wrong! There had just been a cancellation!


I was not at all prepared for what the doctor told me. X-rays showed severe pneumonia in those little lungs. Yes, another fight with infection. I was deeply shocked. I managed not to faint. The first dose of medicine was given by the doctor. We managed to drive home safely!



Love and Medicine

The medicine is given by a syringe into his mouth. The doctor showed me how to do it, restraining him in a towel! I had never ever held my beloved baby that way, having read he would never forgive me for such a terrible violation of his person! In the early years, I believe this would have been the case.

Now, we had no choice. Twice a day, twelve hours apart, the drug must be given. How much did we love and trust?!

One night  his breathing was so labored, holding him in that towel, I feared he would die in my arms. It passed over and the medicine went in! His doctor has now told me that having survived more than two weeks from the diagnosis, he should do well.

We have learned together how to take this medicine because we discovered the secret - the most important part is love! Soft words of love and reassurance help us both be calm enough to take the medicine, despite that terrible towel! Then, once the medicine is down, the magic words "All Done" are said, the towel goes away, and the best reward of all, a kiss!

When birds are upset and emotional, they may bite out of fear. I do not suggest that anyone else do this with their feathered family member.

When I mentioned how we manage the medicine, the lovely woman who works at the Bird Clinic told me that most of the time she hears horror stories about trying to give parrots their medicine....  and so I know, we have a very special relationship.... I am comforted that he has a wonderful doctor, the medicine is working, and he loves me enough to forgive me for what I must do to him....

Parallels Between Parrots and BAVers

I have been struck about some parallels between my feathered family member

 and those with bicuspid aortic valves and thoracic aortic disease. 

Resigned to going back for a checkup!

Unintentionally, they both are very capable of "hiding" a serious condition until it is critical. Somehow their bodies compensate until they are right on the brink of disaster. I will not count just now the times that "suddenly" we fell off a cliff into crisis with BAV over the years.

My feathered child has a doctor who understands this. He has told me more than once that a) this had been coming on for awhile, but not visible to me b) symptoms and behavior cannot be used to guide the treatment - xrays and testing must be used to understand the true condition!  How I wish that more human physicians understood this about BAVers!

Fear in the doctor's office!
Visits to the doctor, even though he is very smart,
 understands what is wrong and is saving my life,
 are stressful!  BAVers can relate!

The popular understanding of how long they live is not accurate. I had been thinking that at 24, my sweet bird still has many years, perhaps double his age now, before him. His doctor is telling me that is not the case. At 24 my beloved parrot is already very vulnerable to health issues.  No doubt there are a number of reasons for this, including diet and a weakened immune system. As we extend the lives of BAVers, some of them may develop other vulnerabilities also. We need to understand so much more about them!

Safely home from my check up - get me inside fast!

One thing we can and are doing is improving his diet. This is a good idea for us all! It gives us every advantage when the time comes to fight a disease. He cannot have any fruit at all or anything with sugar due to the infection. And long term he needs to give up seeds and nuts for specially formulated pellets! He is not enjoying this, but at his recent checkup, he has gained 20 grams!



Heart Sounds of Love
Above all, those who seem so strong and healthy and actually have something wrong need extra special love in their lives! It is my greatest wish for all with BAV and TAD - that their loved ones walk beside them, every step of the way, no matter where it leads. I have no doubt it will lengthen their lives!

This Thanksgiving morning as I write this, he has chosen a soft perch with a cheerful view. The sun is shining after the rain. And there is thankfulness and love in our hearts, for each and every one who we are blessed to have in our lives.

There is much more recovery ahead;
 we know that love will get us through it!

Happy Thanksgiving with love,

Arlys and Peter Timneh

Broken Hearts this February - Remembering Bill Paxton and Those Who Grieve

Red Jasper
The Warriors' Stone

In February . . .

as children, we gave valentines to our classmates.

growing up, we learned about "Heart Month" - a time to focus on heart disease.

feeling invincible, we found our very own heart-mate, our one and only valentine.

later, we learned about BAVs and aneurysms, and wondered why we had never heard of it.

one went into the OR and the other waited as our heart-mate had open heart surgery.

some remained in the fight to overcome BAV and aneurysm.

And, in February, some lost their heart battle.

Yes, February is still about valentines, chocolate and flowers.
For some, it may be the month their hearts were saved.
And, for some, this month our hearts are broken.

Bill Paxton

We were almost ready to leave the hospital a year ago, following my husband's third aortic valve and fourth open heart surgery, when Bill Paxton's death became public. He should have had what all BAVers should have, a successful surgery, his first. What could have possibly gone wrong?

When someone with BAV is lost, we all lose. 

I did not know Bill Paxton, but there are things about him I know well. Like my husband he had rheumatic fever as a child. They also were both born with BAV and over time an aortic aneurysm developed. Like the majority with BAV, he was high energy and gifted, described here by co-star Mary Kay Place.

Video clips show him as the youthful picture of health, almost too perfect, that is typical of BAVers. Others may not notice this detail, seen in some BAVers, but in his smile I see the slight gap in his front teeth. My husband's gap was more pronounced.

If it hurts me to look at video clips of this vibrant man, what indescribable grief Bill Paxton's family must be living through now, the time of the one year anniversary of his surgery and then 11 days, one by one, until his death.

It is extremely painful to read the law suit just filed by his wife and children, grieving the loss of their husband and father. The words take us into a place of horror, with no escape for Bill and his family. 

The Right Hands, Not the Right Buildings

There is no time we are more vulnerable than when signing the papers, giving our hearts, our lives, into the hands of a surgeon and all the others involved. There are up to 100 "Does" listed in this lawsuit, their names unknown at this time. 

Having been through 4 open hearts with my husband, two of them in that hospital, I can easily picture the physical setting. How much of what is written in the lawsuit can be proven in a court of law? I do not know. I do know what is alleged is indescribably horrifying and tragic.

It reminds me of what I heard once - that buildings don't provide care, people do. The absolute greatest challenge for those with BAV is to find, not the right buildings, but the right people to undertand us, to save and extend our lives.

It is a challenge that my own family and other dear friends continue to face.

My February Memories

Memories may not comfort those who grieve at all. They haunt us. I have my own February memories, including two open heart surgeries in this month. We fought for about 8 months after that last February surgery before losing the battle. 

Others tell me the "firsts" are the hardest. I cannot tell you whether this is the case. It is too soon.

I can tell you that this is my first February without my husband, and I am among those with a broken heart. 

This February

may I rise beyond

 the grief and sadness,

and be

more understanding,

more empathetic,

and more caring.

                    ~ Arlys Velebir

.