written by Alaina, Lydia’s mother
October 3rd, 2011 started like any other day. I went to work at an acute care hospital in Omaha where I was an occupational therapist. I took a late lunch to go to a 2 pm doctor’s appointment, keeping my pager with me for when I would return later in the day. Little did I know, I wouldn’t return to work 6 months and my life would never be the same.
I was 30 weeks pregnant with our first child. I had miscarried a year earlier, so we were very grateful for the life growing inside of me. My pregnancy had been uneventful and we were patiently waiting for December when we would get to meet our new baby girl.
My OB/GYN was behind schedule as usual, as I sat feeling our little girl do flips inside of me. When the OB finally arrived we chatted and she put the Doppler on me to listen to our babies’ heartbeat. After several attempts and hearing a few beats here and there, she told me I needed to go to the hospital to get a fetal non-stress test. She assured me not to worry; she just couldn’t get a consistent heart rate and wanted to err on the side of caution.
At the hospital, I was escorted into a delivery room. I still hadn’t called my husband, thinking everything was fine. A nurse came in and attempted to put the fetal monitor on me and again she couldn’t find a heartbeat. I turned from side to side at her request. She pushed and prodded to get our baby girl to wake up. But nothing was working. More people started to enter the room. Two, three, five, ten, fifteen. Suddenly it was packed and someone uttered the words “You need to call your husband. There is something wrong. We are going to deliver your baby.” I couldn’t think; I couldn’t dial his number; I couldn’t speak. My baby still needed ten more weeks in my womb, what was happening?
A nurse called my husband. Oxygen was put over my face. Needles were stuck in my arms. Blood squirted on the floor. Clothes ripped off my body. As they wheeled me down the hall to the surgery room, one kiss from my husband, and a final prayer as I closed my eyes
“God, please let me wake up from this and please let my baby be ALIVE.”
When I woke up from the c-section an hour later, our daughter was already transferred to the NICU at the Nebraska Medical Center and my husband gave me another kiss before he followed our daughter to the highest level NICU in Omaha. There still were no answers. Nobody knew why her heart rate was in the 50s when it should have been 140-160 bpm. She was struggling to breathe even on the ventilator. She was alive, but nobody promised us anything. Laying in my recovery room alone that night, I knew my life had changed forever.
Over the next few days, a multitude of heart tests determined that our baby girl, who we named Lydia, had Complete Congenital Atrioventricular Block, a dilated ascending aorta, an enlarged left ventricle, and an open Patent Ductus Arteriosus (PDA) and Patent Foramen Ovale (PFA). She was also struggling with many preemie issues due to her premature birth, including scaring of her lungs, difficulty breathing, regulating body temperature, and simply growing. Her body was using so much energy just to stay alive when it should have still been in my womb while also having a heart rate a third of what it should have been. There were so many questions, but so few answers. How are we going to fix her heart rate? Can she live like this? What will happen?
On Lydia’s third day of life, the answers started to come. A blood draw showed that she tested positive for the SSA and SSB antibodies. The only way for a baby to get antibodies is through the placenta, meaning I had transferred them to her. These antibodies are known to cause irreversible damage in-utero to the atrioventricular node (AV node), tissue which regulates the heart beat transfer from the atria to the ventricles. Without it, there is no heartbeat conducted from the atria down to the ventricles. The only way the body survives is by the ventricles spontaneously beating by themselves, which occurs erratically, very slowly, and cannot sustain life long-term. The positive antibody test results also indicated that I had Lupus and Sjogren’s Syndrome, two lifelong degenerative autoimmune diseases. How could this be? How could I have a disease I didn’t know I had? How could my body attack my innocent child? How would I ever be able to deal with this? The pain, fear, and guilt was overwhelming. I wept and wept.
Even with the definitive diagnosis, things were still uncertain for a long period of time. Congenital complete AV block is one of the most rare heart defects. In mothers with known lupus, it only occurs in 2% of pregnancies. There are no statistics on mothers without known lupus. It’s that rare. It is estimated that in the United States there are only 100 children born with complete AV block per year. But even though physicians know the cause, treatment and long-term prognosis can be difficult. At 3 lbs, 4 oz at birth, Lydia was not a candidate for a pacemaker. She was too small, too weak and too fragile. Therefore, our job became to keep her alive. To help her lungs heal. To get her to eat. To help her to grow. All made more difficult by a heart rate so low it was difficult for her to do anything.
By her seventh day of life we were able to hold her for the first time. She was so fragile they only allowed us to hold her for four hours per day. My husband and I would alternate days, sitting undisturbed skin-to-skin for four hours breathing in every ounce of her life and spirit. Every day we poured our love into her, hoping and praying she would make it. By day 58, we convinced the doctors to let us take her home. She hadn’t been growing or developing as the doctors had hoped, but my husband was deploying overseas in two days and we didn’t want our only memories as a family to be in the hospital. So after some convincing, a quick welcome home and a long good-bye, I sat at home holding Lydia, knowing that my sole job for the next three months until my husband returned was to keep her alive. Life had never been so important.
For the next three months, I held her. I held her while she slept in 20-minute increments until her heart rate got too low and she woke up again (an amazing survival technique). I held her while she attempted to eat between labored breaths. I held her while I showered. I held her while I walked laps around the house, trying to calm her constant cry from the sheer discomfort her body felt. I held her and willed her to grow – willed her to grow so she could get her pacemaker and have a chance at living. We left the house only for doctor’s appointments, which were typically three times a week. If I was feeling upbeat, we even occasionally stopped by the drive-thru of Jimmy Johns on the way home. Generous friends brought us food and shoveled snow off our driveway. And we all prayed my husband would return safely from his deployment as a pilot for the United States Air Force, with a daughter to hold.
The days and months were long, but on April 26th, 2012, shortly after Corey returned from Qatar, a barely 8-lb. Lydia received her pacemaker and a new lease on life. For the first time in her life, she slept, breathed when she ate, and became calm. It was as though she had been waiting for this peace and comfort her whole life. Instead of struggling to live on an insufficient heart rate, like she was out of breath 24/7, she opened her eyes to the world and was able to do more than just survive. All was not smooth sailing and recovery included two additional surgeries to fix hernias due to her body being too small for this massive pacemaker in her abdomen, but we were beyond grateful. For the first time we had real hope for her future.
Lydia is now a joyful four and a half-year-old who is generous, kind, independent, thoughtful and incredibly brave. She has had other issues including sleep apnea, lung scarring and difficulty growing, but they are minor compared to the gift we have been given. She will be dependent on a pacemaker for every beat of her heart for the rest of her life. She will continue to have ECHOs, EKG’s, pacemaker checks, x-rays, and surgeries consistently for the rest of her life. There is no cure or treatment for damaged electrical cells. Pacemakers can never mimic an intact heart and therefore at times her heart rate does not match her emotions, activity, or regulate her body temperature like it should. The antibodies also caused damage to her aorta and she is at risk for developing cardiomyopathy, but we will take the challenges as they come. For now, we are letting her live and enjoy life as much as possible. She loves to swim, run, dance, ice skate, read and learn. She is also an incredible sister to her two-year old brother, Lincoln. After four and a half years, we also recently met the next baby born in Omaha with complete heart block and Lydia is looking forward to being an amazing mentor and friend.
For myself, the antibodies and autoimmune disease have gotten worse and will now always be a constant struggle in my life. With Lincoln there was a 20% chance of heart block occurring in him as well. However, we enrolled in a research study and we were able to prevent a recurrence of heart block using a trial medication. He is heart healthy!
Through the past five years my faith has been tested beyond measure, but it has allowed me to grow and our family to grow together. We have created a tight-knit family across the world of heart block families whom we gain support and strength from. Lydia has learned to use her pacemaker and her differences as a source of strength and she lights up a room like no one else I know.
Her heart, her bravery and her compassion shine like a beacon to all of those around her.
My heart is full of gratitude everyday for the life we get to share with her. And our mission now is to spread that love to others.