This Is The Day The Lord Has Made

THIS IS THE DAY - Day 45, Heart Transplant 1A List, New Heart Day

 

It's a beautiful day in Rochester.
Mark is inside this hospital in surgery, receiving the gift of life. 

This is the day the Lord has made; Let us rejoice in it and be glad. Psalms 118:24 

This Bible verse has always been special to me.  It was one of the first I memorized as a child.  I still have a small white slip of wide-ruled, notebook paper, stuck between the pages of my Bible. On the paper is this verse written in my 1^st grade handwriting.  It’s the way we start every day at the Johnson household and we have for years.  Some days we sing it on the way to school, some days we recite it.  None the less, it is always present.  It is my verse.  It is now my family’s verse.  Some days I recite it to myself a hundred times a day, when I feel myself stepping outside the joy or gladness of the situation I find myself. And, today…TODAY it has an especially sweet ring to it. 

TODAY IS THE DAY.  It is the day we get another chance at life.  At healing. At being complete with my other half living life beside me. For my girls, having Daddy at home to watch them grow and change.  It is a day we have prayed to come quickly for years.  We have waited and watched.  We have walked through the valley of the shadow of death.  We have cried out that we needed help, an answer. We  have had doctors tell us this transplant was the only way Mark would survive, then tell us his case was too complex and that there was nothing they could do.  We have prayed over this decision.  Make him comfortable?  Do we listen?  Do we believe the doctors?  Do we have faith that they are wrong?  Do we split our family up for a chance of another chapter in our story?  Our only answer has been to boldly step out in faith, taking the tough days and decisions head on.

God led us to Mayo Clinic.  God walked beside us every step of the way.  God is leading us today.  He is holding the hands of the surgeon who is holding Mark’s hearts—the one he was born with and the new one.  He has His arms around me and is gently reminding me to breathe.  (That hasn’t been so easy for me.)  I keep finding myself holding my breath.  Breathing deep is difficult when so many thoughts are rushing through your head.  Minutes seem like hours…Hours seem like days…Weeks seem like years.

Trying to pass the time as I fly somewhere over the great United States, I fall back to one of my joys—the peace I find in the written word.  This is a very long post and one that I have been working on for the past few weeks.

I hope you enjoy reflecting on the journey—and clearly see God’s hand on our lives, as we do.

An overview!  So many people ask why Mark is waiting for a heart.  Most assume this is all new to us.  It isn't new at all.  Yes, physically being on the heart transplant list is somewhat new.  But the cardiac issues are not new at all.

Mark was born with aortic stenosis—a narrowing of the aorta of his heart.  He was born on March 12, just two days before me.  At first all was fine.  He went home from the hospital as a normal newborn. He was the first child of a wonderful young couple.  His mom was just 21 years old, not realizing how strong she would have to soon be.  In the beginning of April, his mom took him to the doctor sensing something was not right.  He was congested and just seemed like he was struggling.  The doctor told her Mark just had developed a simple cold and would soon be fine.  Her maternal instinct knew that this was more than a cold and proceeded to take him to doctor after doctor looking for answers.  Her persistence led her to Duke and ultimately to the realization that their one-month-old little baby needed open-heart surgery to stretch the narrowing of his aorta.  Mark survived his first surgery and so began a life-long adventure.  Many years later Dr. Armstrong, his primary cardiologist, told me that he should never have survived that first surgery, based on medical science.  She acknowledged that he was a miracle from the beginning.  Over the years, over and over, one miracle after another would be realized.  Mark's life has always been a powerful testimony of God's grace, provision and mercy.

His next open-heart surgery was at seven-years-old.  He was one of the first pediatric patients to ever receive a titanium Saint Jude's valve, rather than a pig valve.  At seven your heart is almost full size and thus the reason they waited for him to reach this milestone.  At this point, a pace maker was also connected to keep track of the electronic pulses of his heart function. For the remaining childhood and teen years, Mark continued to be followed by his primary cardiologist (Dr. Brenda Armstrong) and his electrophysiologist (Dr. Ron Kanter).  Both of these amazing doctors have become like family, following Mark's success and cheering him along the way.  Fine-tuning was done, but nothing major until he began having issues while in grad school.  During his post-grad years several new diagnosis were added and multiple heart procedures - heart catheterizations, electrophysiology (EP) studies, ablations, etc. Over the years he has undergone 40+ heart procedures.

Several years then went by without too many significant changes. In January of 2001, I entered the picture.  We quickly knew that we were destined to be together.  We were inseparable from the beginning. We had been set-up on a blind date by a close friend who had spent 3 years trying to convince us both that we needed to meet the other.  He finally wore us down.  And, we are so thankful for his persistence and the great care he took at keeping us uber informed on the other.  When we met, it felt like we already had known each other for years.  The moment our eyes met, I knew he would be my husband.  We both were smitten from the beginning.  Just a couple of weeks into our dating relationship, I started working at Duke Children's Hospital, where Mark had been treated since he was one-month-old.

Through those years, I had many chances to see heart patients at all stages of their journey. In high school during Summer Ventures in Science in Math, I got to witness a cardiac catheterization on a one-month-old baby.   At Duke Children's, my first communications assignment was with a local reporter covering a baby after open-heart surgery.  I got to see first-hand what Mark had been through.  I kept up with this family for years and watched their son grow into a young man. (Years later, I also watched proudly as my Make-A-Wish team got to grant that little boy's wish —in his teenage years.) 

God has placed me in unique circumstances throughout my years in children's healthcare to fully experience what Mark has endured.  I have seen first-hand how Mark's upbringing allowed him to flourish and not use his illness as a crutch.  His parents treated him as if he was normal—not as if he were fragile.  And, because of that, I am eternally grateful.  Because of the way they raised Mark he was able to fully live life and reach his academic, career goals, get married and have a beautiful family.  Many children, in similar circumstances, are shielded and protected and never fully live life.  I have personally witnessed how very different life is, when a child is taught to allow their circumstances to define their future.  

Our first heart procedure together came just a few months after we started dating.  Mark had a cardiac catheterization in May, we were engaged to be married in July,  and then later that year he had an Implantable Cardioverter Defibrillator (ICD) placed, replacing his pacemaker.  Just a few months later, the battery drained unexpectedly and he had surgery to replace the faulty device.  

A year later on December 6, 2003, Mark's life was spared again.  Because he was already followed by cardiology, his doctor was able to catch an unrelated aortic aneurysm before it ruptured.  Most people do not survive an aortic aneurysm because it kills them instantly.  I will never forget getting the call from Mark's doctor on a Wednesday evening that he would likely not survive through the weekend.  She told me to get Mark to the hospital as soon as possible.  An ice storm was coming and the doctors wanted to get Mark in surgery before the aneurysm had chance to rupture.  I distinctly remember getting the call, falling to my knees in hysterical crying, on our stairs and our brand new puppy Sam, coming to comfort me. That would be the first of many times, poignant memories of Mark's heart journey intertwined with memories of Sam.  [We said good-bye to Sam just a few weeks ago.  He was a surprise to Mark—a companion for him to get through a major heart surgery.  Sam, a Jack Russell Terrier, had a white heart marking on his head and was our first baby.]

The doctors were successful in grafting a prosthetic aortic root to Mark's heart and proactively replaced his valve with a larger version.  The first open-heart surgery in Mark's adult years was definitely a learning experience, but we survived and we're a stronger couple for getting through the rough , both physically and emotionally grueling, days that we walked through.

Mark's health journey slowed to a dull pace and in 2005 we welcomed our first baby girl, Alivia Kristen!  That summer Mark had surgery to replace the battery in his ICD.  The following year we welcomed Avery Kamdyn in 2006, followed three years later by Aleah Kathryn in 2009 and three years later by Ardyn Kennedy in 2012. Our biggest struggle during these years were Aleah's severe lung infection and beginnings in the NICU for the first nine days of life; followed by Mark's esophagus tear (discussed below) and Ardyn's brain surgery scare.  [Looking back we have had a number of frightening medical situations with our family, but none compare overall with Mark's life-long struggle.]   

We were busy.  Busy living life, enjoying our girls and watching them reach milestones.  And thankfully, Mark's health was mostly mere routine check-ups during these years of our young family.  Mark and I were both focused on our family and growing our busy careers.  Mark was advancing through growth in health care administration, managing wound care clinics in multiple local and regional hospital systems and I transitioned from Duke Children's to Make-A-Wish Eastern North Carolina.

Life would take a new turn in February 2013 when Mark had his first TIA —a stroke. That's another call I will never forget.  Mark was working at a hospital in Rocky Mount at that time.  I got out of a lunch meeting to see I had missed multiple calls from him.  Just as I was calling him back the phone rang.  It was Mark's phone number but another voice on the other end.  A nurse who worked with Mark told me that Mark had suffered a stroke and was being transported by ambulance from the hospital in Rocky Mount to the ED at Duke.  And, so started a new chapter.  We would soon be dealing with neurology specialists on top of all the cardiology specialists we routinely see.  Mark went in the hospital for a few days. Everything checked out and after the weekend, Mark seemed fine and returned to work on Monday, mere days from the excitement. That weekend began 18 weeks of chaos.  Mark worked all week, came home immediately and went to sleep.  On the weekends, he would go to sleep Friday afternoon and sleep solidly through the entire weekend.  At first, we thought he was exhausted.  We had never dealt with strokes and were sure his body was just catching up after the stress of the weekend.  After several weeks and the girls passing around crude, we chalked it up to an illness - a cold, the flu, we came up with a million excuses.  

By June, we were realizing this could not be normal.  We questioned this strange pattern of more sleep than normal with his cardiologist. She decided to run tests.  Before those tests could be run, we found ourselves back in the ED at Duke.  Heart Failure.  Another defining moment.  Mark was diagnosed with heart failure. The extreme sleep was his body compensating for the stress of heart failure.  This led to a hospitalization and a battery of additional tests.  One of those tests was to have a TEE.  Unfortunately, this test was anything but routine. Mark returned to the room after the procedure complaining with severe pain. The doctor's dismissed it as being normal.  For several hours, he continued in pain.  

This was early July and the house staff at Duke were all in new rotations.  The hospital had just converted to EPIC (the electronic medial records system) and the nurses, doctors and pharmacists on the unit were just not prepared.  Mark begged for relief. A Fellow proceeded to try to send us home, despite Mark's complaints of pain. Mark has an extremely high tolerance for pain and I knew to believe him at his word.  I had enough and had to step in and tell the Doctor that we were not leaving the hospital until we had answers.  Mark was in severe pain and this was not normal.  Had we left the hospital I am certain he would not be alive today.  Just minutes later things got real, fast.

Several years before we had been through an unrelated scary trip to the emergency room, after Mark had torn his esophagus on a jagged nut encrusted piece of cereal and was transferred from the ED at REX to Duke by ambulance and was in critical care for several days.  Mark claimed this was the same burning pain as that tear.  Soon he started throwing up blood—one-third of his body's blood content to be exact.  They rushed him back to the OR and sure enough, Mark was right.  The medical team had unknowingly torn his esophagus during the TEE and he was bleeding internally.  The scope with the camera on the tip ripped open Mark's esophagus.  It was able to be repaired but he was once again in critical condition, this time in a medically induced coma to let his body heal.  

The trauma was as extreme as you can imagine.  We truly did not know if he would make it from hour-to-hour.  By the grace of God, his life was spared again.  His recovery was slow and led to a month-long hospitalization to heal from the trauma.  In early August, we were released to return home only to be back within days.  Mark's care had transitioned to life-preservation rather than addressing the heart failure and reason we originally were seeking answers. Another couple of weeks, more tests and very little answers started the next phase of Mark's journey.

Mark was placed on medical leave and would ultimately transition to full-time disability. At first we thought this would be for six weeks, then eight weeks, then six-months and then permanently. From the medical trauma of that summer, added with a lifetime of medical issues, these life experiences and traumas led to post traumatic stress syndrome (PTSD) and months of struggling with panic attacks and the emotional highs and lows that followed.  In December Mark had another TIA, this time going to his eye rather than his brain.

Over the next 4 years, we would go to countless specialists to try to understand what Mark was dealing with.  At first the doctors seemed like they knew which direction to seek answers.  Over the years, the answers became fewer and fewer. When Mark began having issues with strokes and heart failure, his care was transitioned from pediatrics (whom had followed him wonderfully his entire life) to adult care.  The results were disappointing.  The adult doctors were not as caring as we had been accustomed to in pediatrics.  They didn't have the benefit of knowing all the nuisances of caring for Mark.  It often seemed like all they knew how to handle was textbook cases.  They seemingly had no idea how to function in the complex world of Mark.  I often have told new doctors to expect the unexpected with Mark’s heart. 

Question marks were everywhere.  In addition to normal cardiology appointments, we saw specialists for neurology, eye specialists, genetic specialists, kidney specialists—the list just kept growing.  We went through genetic testing. The tests came back to show Mark did not have the suspected disorder that would explain his very rare medical abnormalities. Mark was checked out from head to toe.  We were finally referred to the transplant team at Duke in the fall of 2014.  We went through the battery of tests pre-transplant evaluation and the doctors were hopeful.  

For the first time we had a doctor who told us that without a new heart Mark would only live 2-3 years.  Yet, again...another defining moment.  That moment would transition our adult lives. In the back of our heads we always knew a transplant may be an option down the road.  We had no idea we would be facing this reality so soon.

After the initial evaluation appointments, we were led to believe the transplant was a viable option, needed and that it would likely happen very soon.   The transplant seemed like a no-brainer, like it would help and give Mark his future back. All the doctors and specialists seemed to be on-board until the prerequisite heart cath.  

And another defining moment.  The doctor completing the cath came out to tell us the news.  She looked at me and said, "You should expect him to die in his sleep at anytime."  According to her assessment, his sleep apnea was so severe that she said she had never seen anything like it.  Immediately after going to sleep, his heart pressures would go from normal to off the chart.  Suddenly this became a major road block.  Mark was denied for listing and told to focus on losing weight and getting his sleep apnea under control.  They said the transplant was too risky. 

For the next two years, every six months we were reevaluated. Mark’s decline was so apparent to those close to him, yet the doctors seemed to not know what to do, nor acknowledged the constant decline.   He was sleeping in long segments again for no apparent reason.  When he was in a deep sleep he could not be awakened—sometimes for days.  It was devastating to watch—and extremely frustrating.  Mark would soon be missing family events more and more often.  He would go through cardiac rehab twice and have several more TIAs and ocular strokes.  It was to the point that I knew I was watching him die.  Every time I saw an ambulance I would call him as quickly as I could to be sure he was not headed to the hospital.  I have vivid memories of the girls waving goodbye to daddy as he was whisked away on the ambulance.  That’s a scene I hope I never have to experience again.

In 2016, he was in the ED at Duke 26 times in six months.  After that, I lost count.  He was admitted some of those hospital trips and others sent home with medication changes.  He was taking so much medication that his kidneys, at times, seemed to be shutting down.  At one point, there was talk of a possible kidney and lung transplant, too.  He came to the point where the doctors looked to him for direction on what they should do and changes in medication.   Mark would titrate his meds based on the level of fluid he was retaining.

Unfortunately, the tests the Duke doctors were offering only saw his heart pressures at rest and not in motion.

By last fall, we were told that the team at Duke thought Mark's heart condition was too complex and questioned whether a new heart would solve the issues from the symptoms.  We repeatedly were told that his severe symptoms did not make since and did not match his test results.  he Duke heart team was baffled and honestly seemed not to know how to help Mark.

[We would later find out that Mark's heart failure was HFpEF (Heart Failure with preserved Ejection Fracture) also known as diastolic systolic heart failure.  Most heart failure is systolic— the exact opposite. Mayo Clinic has the world-leading heart program addressing HFpEF and thus the reason Mark has had much success in the program.]

One doctor, who we have known for years through both pediatric and adult EP talked with us as friends.  He listened intently as we shared our frustrations and what we were seeing at home.  A light bulb moment happened.  On a whim, he connected Mark’s ICD to the wireless monitor and had him walk down the hall.  Mark didn’t even get to the next exam room door before Dr. Carboni’s month dropped open and he responded, ‘OH, MY!”  Mark’s heart with any exertion, even walking a few feet, caused his heart to go out of rhythm and drop beats.  Suddenly we had proof of what was happening and a new direction that would ultimately led us to Mayo Clinic. 

Soon we would hear of a clinical trial that Mayo was completing at a few centers across the US and Duke happened to be one of them.  Mark qualified for the trial and started last fall.  It was remarkable.  The study was with an investigational drug and a double blind placebo.  Patients would be given the drug for several weeks and on the placebo for several weeks..  We knew immediately when Mark was taking the real investigational drug.  The effect was staggering.  He had energy and seemed to be himself again.  On the placebo, he would crash and crash hard.  At the end of the study, his overall decline had progressed by 18%.

Dr. Barry Borlaug was leading the study and we knew we needed to reach out to him.  He has dedicated his life to studying the exact HFpPF diagnosis that Mark has and its effects and treatments.  Just before Christmas Mark found his email and sent him a message.  Within days, we were sitting in his clinic.  We fully believe God ordained this meeting.  We were blown away with the knowledge, reassurance that Mark’s symptoms were absolutely a direct result of his advanced heart failure and the care and compassion. Finally, we had someone willing to look at the whole picture and offer guidance and medical expertise that would address all that was happening.  In healthcare you need a quarterback—someone to guide you through, navigate the process with the overall goal in mind—and we finally found that at Mayo.  We have been overwhelmed with the caliber of the Mayo experience.

Over the past six-seven months, Mark has been welcomed with open arms and cheered all along the way.  The medical team and their comprehensive approach to care is better than anything I have ever seen or can imagine.  We have had an excellent experience.  It has not been easy, but he has worked hard, completed the second phase of the clinical trial, lost 57 pounds, endured 2 heart surgeries, 2 heart caths, is currently in open-heart surgery, receiving the gift of life and made life-long friends. 

Good is good and faithful.  We are forever grateful to have made it this far and we can't wait to see what the next chapter holds.

To God Be the Glory for the Great Things He Hath Done!