I am scheduled to meet a new pulmonologist tomorrow, so I've been going over my medical history in preparation for the appointment. Most people know of the health complications with my pregnancy, that I have a pacemaker, and that I had surgeries as an infant. But I've recently realized that most of my family and even closest friends do not know the full story.
I like to make jokes that I'm a "miracle baby". I also jokingly blame my health problems for anything I am unable to do (i.e. I cannot swim, nor whistle, nor flip a coin- all because of my poorly functioning heart) I will vaguely mention things like "I was born with heart disease" or "I have bad lungs", but never expand on it, simply because it's too long and complicated to explain. I don't want to bore anyone with the details.
The truth, though, is that the full story is pretty incredible. I am slightly ashamed that I've had this testimony and never shared it before. And, regardless of your beliefs, it's still really interesting from a medical stand point. So here it is....
I am not a medical professional, but tried my hardest to make sure all information was correct. Please excuse any mistakes. I used information gathered from my medical records, The Annals of Thoracic Surgery, and the book "Running The Midnight Marathon" by Craig Brian Larson (at the time of my heart surgeries, my parents' pastor came to visit and pray with them at the hospital through every surgery. Years later he wrote a book on faith and perseverance, and used my story as one of the examples)
Excerpts from Running the Midnight Marathon:
"On July 26th 1984 Terri gave birth to their second child, a girl, whom they named Rebekah. The delivery had gone beautifully, and Rebekah appeared to be in perfect condition. As the weeks passed she continued to appear healthy, except for some discoloration in her skin, thought to be jaundice. At her 6 week check-up, however, the doctor, noticing a slight blue tint to her lips, became immediately alarmed, and ordered that she be taken to the hospital.
The terrifying diagnosis: Rebekah was in heart failure. She had only days to live. 'Her heart' said the cardiologist, 'is a mess' The four pulmonary veins that pipe clean, oxygenated blood from the lungs to the heart were detached, dangling behind her heart. There were two holes in her heart allowing clean and dirty blood to mix. Also, instead of left and right ventricular outlets, she had two right outlets, and no left. More revealing than the surgical report was the cardiologist's grim face.
Open-heart surgery was scheduled. The doctors planned to do just enough to keep her alive: attach those four dangling pulmonary veins. Her other defects would have to wait until her body was
Even doctors know their limitations. At the beginning of the crisis, as Vince and Terri conferred with the surgeons, Vince asked point-blank what Rebekah's chances were. The doctor pointed to the ceiling, looked up, and said, 'It's not in my hands'
Vince recalls, 'He said that he had only seen or read about 10 cases like Rebekah's in the last 10 years. I asked how many had survived. He wouldn't even answer'
For those who like technical terms and details (as I do) here are the clinical terms of my defects and explanation:
1. total anomalous pulmonary venous return(TAPVR- also called total anomalous pulmonary connection): all four pulmonary veins are malpositioned. Normally, your pulmonary veins take blood from the lungs back to the heart through the left atrium. In TAPVR the pulmonary veins connect to the right atrium, hepatic portal vein, superior vena cava, or some other vein.
The point being that they are not connected correctly to the heart, therefore, not bringing oxygenated blood back to the heart properly.
But here is what made my condition different than most cases they see:
Usually in TAPVR the 4 pulmonary veins are connected to some vein (not to where they are normally supposed to be attached, but still some sort of connection...somewhere)
All 4 of my pulmonary veins coming from the lungs were not attached to anything. They were just hanging, draining blood down towards my diaphragm. So there was no oxygenated blood coming back to my heart.
This is why my diagnosis is called TAPVD: total anomalous pulmonary venous drainage (not return/connection)
2. atrial septal defect : this is a type of "hole in the heart" While babies are in the womb there is an opening between the upper chambers of the heart, but it should close before birth. Atrial septal defect is actually beneficial to TAPVR, because the hole allows small amounts of blood to kind of leak into the heart; this hole is what keeps an infant with TAPVR alive at birth.
3. double outlet right ventricle: Normally the aorta connects to the left ventricle, and the pulmonary artery is connected to the right ventricle.
With Double Outlet Right Ventricle both pulmonary artery and aorta flow from the right ventricle.
No arteries were connected to the left ventricle (the chamber that normally pumps blood back to the body)
The doctors were unable to explain how I had survived for 6 weeks with all pulmonary veins detached from my heart, and malpositioned arteries. The atrial septal defect allows a small amount of blood to leak into the heart, but certainly not enough to sustain my entire body for 6 weeks.
"Rebekah's frail body was deathly weak when I went to visit her prior to her first surgery. After donning a sterile yellow gown and scrubbing my hands with a plastic brush and brown, disinfectant soap, I pressed with Vince into the thick tension of the intensive care unit of Children's Memorial Hospital in Chicago.
I greeted Terri, whose face was taut with strain, and then turned my attention to Rebekah. She lay unconscious, her eyes moist and swollen. She was wrapped, not in pink baby blankets, but in technology. A clear plastic, saucer-like bed, a welter of wires and tubes networked her tiny body, and around the bed stood monitors and IVs and shelves filled with medical supplies. Two feet overhead a light radiated heat. On the screen, I observed the slow fluttering record of her heartbeat, now in such jeopardy.
Then I noticed something with a human touch. Taped above Rebekah's head was a hand written note.
"My flesh and my heart may fail," it said, "but God is the strength of my heart and my portion forever Psalm 73:26"
"While I was in bed last night," Terri told me "I prayed, 'God you have to tell me if she's going to live, or if she will die. You have to tell me. I just can't stand the not knowing' Then I opened the Bible and read that scripture. My eyes fell right to it. So I wrote it out, ran to the ICU and taped it to her bed"
I survived the 1st surgery.
The next two weeks lasted forever. "The recovery involved constant monitoring," says Vince. "She could have died at any second. My wife lived at her bedside. Rebekah couldn't eat for three days. She couldn't drink for three days. We watched tears pour down her face. After a day's worth of crying, all we saw were tears, and her mouth open but no sound came out; her voice was gone. So it was horrible. It was just horrible."
Nevertheless, she made it. And after 2 weeks they brought Rebekah home.
Unfortunately, when I was 7 months old a cardio catheterization revealed pulmonary stenosis. The repaired pulmonary veins were closing, and the damage to my lungs was severe. The doctors suspected I may have interstitial lung disease. If that were the case, it would be irreversible.
They would try to reopen her pulmonary veins, and if she was not too weak, get a lung biopsy. They braced Vince and Terri for nothing but negative: even if they could reopen the veins, there's a chance they could close up again; more critical, if the lung biopsy showed lung disease, death was inevitable.
...The surgery was scheduled for the next day. Vince and Terri went home to pack a suitcase for the hospital. While there, Terri opened her scripture calendar to the next day's date:
February 22, 1985: "A new heart also will I give you, and a new spirit I will put within you; I will take away the stony heart out of your flesh, and I will give you a new heart of flesh" Ezekiel 36:26
Terri says, "I'm not into fortune cookies, or superstitions, but when I turned the calendar to that date, I just stood there crying. I yelled to Vince 'You have to see this. You have to see this' The next day I taped that to her bed when she went into surgery. I knew God was speaking to me"
The surgery was successful, and they were able to collect a sample from the lungs to biopsy. They had to wait a week for the results.
During this waiting period Terri says "While the lung biopsy was in route, you see her struggle; you see them put a chest tube in her, and all of a sudden your mind starts flip flopping. But even in moments of disappointment, there was a peace that would come over me. I didn't flip out or say "God, you lied to me" Instead, my heart would say "It's going to be okay. God's just going to do it in a different way"
Terri's peace was justified. The biopsy report finally returned-negative. Eight month old, brown-eyed Rebekah, still weighing only 8 pounds, had bounded over another huge hurdle.
Vince and Terri sat down with the heart surgeon after the 2nd operation for a briefing on Rebekah's future. "We'll have to wait several months and see what happens to the pulmonary veins. If they begin to close again, there's no sense in attempting the final surgery"
Over the following weeks, Terri often wrestled with the feeling that time was running out. She would walk into the nursery, see her baby sleeping, and think of the painful procedure ahead and the risks. Frequently she knelt by the crib and after crying for a while would pray "Touch her heart and let everyone see your power"
Months passed, and finally the doctors scheduled Rebekah's 4th cardio cath. The procedure was supposed to take 2 hours. After the 3rd hour passed without report, tension mounted. For 3 hours they had been trying to insert the testing instrument into her veins; first in one leg, then the other, then her arm - all without success. They had to call in another cardiologist.
The next day they tried again, and were finally able to get through.
With few words and no emotion, the cardiologist informed them that the veins were open, and they were clear to proceed with the 3rd surgery. He knew, all too well, that what lay ahead was nothing to get excited about.
Finally it was the night before surgery. Before putting Rebekah to bed, Terri anxiously danced with her in their hospital room, quoting scriptures, and singing praises.
The nurses wheeled 11 month old Rebekah - weighing only 10 pounds, but now with blondish brown curly hair - down to the second-floor operating room with her parents by her side. They had taped a new scripture to her bed:
"I will not die but live, and I will proclaim what the Lord has done" Psalm 118:17
"I will not die but live, and I will proclaim what the Lord has done" Psalm 118:17
Aside from the fact that I am alive today, and survived 3 open heart surgeries, the thing that strikes me as a "miracle" is that I was alive for 6 weeks with no immediate sign of heart disease. Yes, sometimes infants can go months before discovery of a hole, or a common heart defect. But it is pretty miraculous that my body somehow sustained itself for 6 full weeks, without any medical intervention, with all 4 pulmonary veins detached and just draining, and the great arteries misplaced.
I absolutely believe God sustained me those first 6 weeks of life. I absolutely believe in miracles.
Recently my father sent me an article from the "The Annals Of Thoracic Surgery: Surgical Management of Infants with Complex Cardiac Anomalies Associated with Reduced Pulmonary Blood Flow and Total Anomalous Pulmonary Venous Drainage" dated April 1986
I am patient #2 in the study. Within the last 10 yrs the hospital had seen only 8 patients with a similar combination of cardiac anomalies. 4 patients died during surgery, 3 were alive and well, and 1 was alive but doing poorly. I was one of the 3 that were living and doing well at 18 months old.
Interesting fact, the other 2 patients who were alive and doing well had their first surgery within the 1st week of birth; one at 4 days old, the other at 7 days old.
Another interesting thing was that all 4 children who died had shunts put in prior to attempted correction of TAPVD. There were two points that this article makes:
1. Time was crucial. The sooner the TAPVD was repaired the greater the survival rate
2. Shunts should be placed after the repair of TAPVD, as fatality rate was higher when shunts were placed first.
I was first seen at one hospital and then transferred to Children's Memorial hospital. The doctors at the previous hospital could not see my pulmonary veins clearly through the cardio cathetizeration, and therefore did not know I had TAPVD. Their plan was to place a shunt. If it wasn't for the incredible doctors at Children's Memorial Hospital in Chicago, who did a second cardio cath with pulmonary angiography upon my arrival, which showed my pulmonary veins draining down towards the diaphragm, I would have had an unnecessary pulmonary shunt placed, and an even greater chance of death.
My father is very intelligent, rational and analytical. He was the one who asked the doctors every possible question, and he memorized every detail of my conditions. He researched to find the top heart surgeon in Chicago. He was the one who requested that I be transferred to Children's Memorial, and have Dr. Michel Albawi perform my surgeries. When he was told that they would not accept me as a patient, because of the type of insurance we had, he went up to the hospital and confronted the director of the hospital. They made an exception. If it wasn't for his persistence, I would have never been transferred, and I would have had a shunt placed at the first hospital.
I am eternally grateful to my parents; my father's intelligence and persistence, and my mother's wisdom, prayers and unyielding faith.
I am eternally grateful to my surgeon, Dr. Michel Albawi, and every doctor and nurse that cared for me.
I've had issues with my health since those surgeries. I've had a pacemaker since 8 yrs old, and I've had 3 replacements. I also developed aorto-pulmonary collaterals during my first pacemaker surgery, which required coil embolization. I formed those collaterals again during my pregnancy. It was life threatening and required an 8 hour coiling procedure. I have come close to death multiple times, but each time God has sustained me. I may have complications in my future, and that is okay.
Because when I read a report of 8 infants (simply known as Patient 1, Patient 2, Patient 3, etc...) and then I scroll across to the final column of the report which states "Outcome", and read next to each infant the words "died" or "alive", it puts everything into perspective.
Patient 2: Alive. I've lived 30 yrs more than predicted. 30 years more than Patient 3, 4, 7, and 8. And for that I am forever grateful.
Even if I were to die tomorrow, it would not be a tragedy. Every day on this earth is an incredible blessing that I may have never known.