By Ann Yurcek
From the very earliest of moments with my daughter Becca, her heart was “at the heart” of her issues.
When she was just two-days-old, a knock on the front door with a police officer informing us to call the hospital signaled the pounding and terror of my heart. As I raced up the steps, barely touching any, grabbing the phone I called the number on the piece of paper handed to me by the police officer. The first words from the nurse were, "She is alive . . . but her heart is causing issues and she is being airlifted to the Children's Hospital of St. Paul". Thus began our “Journey of the Heart”.
Doctors found out that Becca's heart had Pulmonary Valve Stenosis, and a huge ASD (early on she had only a little thickening from Hypertrophic Cardiomyopathy). From August to October, her stay in the NICU was marked by figuring out all the other things wrong with tiny Becca. They felt she had some unusual form of Noonan Syndrome because she was just too severe. Her immune problems, her high white blood counts, her constant thrush and staph infections puzzled the best professionals.
At nearly 3 months, they had placed her gastrostomy, repaired her malrotated intestines and with a shot of Fentanyl for the pain Becca went into respiratory and cardiac arrest. For 13 minutes, tiny Becca's heart was not beating. Seeing my daughter in deep shades of blue, I ran from the NICU, ran from the trauma and only adept nurses would corner me into reality and calm my racing heart. The doctors came to me saying, “We were able to restart her heart, but we do not know how much brain damage she suffered, only time will tell”.
The NICU staff were talking about discharge by the end of October; however, I was seeing something in Becca. I told them of my concern and the staff told me that it was probably fear of bringing Becca home. The panic caused my heart to race. Maybe they were right, I was afraid of the responsibility, I was afraid of the future, and of my life racing seriously out of control. On Halloween, Becca played tricks on her staff with her heart and soon the Pediatric Cardiologist was called back in. They came to me with the news that Becca's heart was worsening. I was not just a panicking mother, I knew in my heart that something was wrong; her spark, her eyes told me. My mother’s intuition was right and thus began my own struggle with that intuition and not wanting to see anything more wrong with my tiny daughter.
With Hypertrophic Cardiomyopathy, Pulmonary Stenosis, an ASD and all the other things wrong with Tiny Becca, I knew that my daughter was really sick. They started Lasix to relieve the fluid that was filling in her tiny body. She was in the beginnings of Congestive Heart Failure. Heart failure . . . caused this Mom's heart to worry.
Thanksgiving arrived and the hospital sent our tiny daughter home to make memories . . . Because they didn't know how long Becca would live. They were always honest about her poor prognosis. But for us, we learned to accept that every day with Becca was gift. Time home would be limited, and I learned very quickly that I could not do this alone. I had a critically ill baby and five other children under of 11. I had to learn to accept help and open my heart to sharing our struggles and journey with those in the power to make decisions and find resources.
Christmas came and went, and the miracles of the season changed our hearts and gave us HOPE. (Read the Christmas Story here) Becca's heart was in serious trouble and by February, we were given a two-day pass home before her heart catherization. I sat with Mary the social worker and realized that Becca's six-month-old birthday was on the third and it may be the only birthday we would ever be able to celebrate. I had few memories, we had few moments of home and we needed a celebration. My camera had been stolen and the only pictures we had were snapped by the staff of the NICU. Mary had me call Make-A-Wish and I told them of our wish to borrow a video camera so we could record memories of my dying baby.
Make-a-wish requires a wish to be that of a child, not of the family. Becca would never live to be old enough to get a wish of her own. I thanked them and went on to planning a special Heart Party for Becca. (Read the Valentines Story here ). The house was decorated in hearts of all colors, friends, family and others all arrived and that evening would be documented as a camera crew surprised us and showed up at the house to record Becca's party of the heart. We were not alone in our journey, Becca moved many people’s hearts.
The next day, we were off to the hospital for her cardiac catherization, as I ran the stairways of the hospital to relieve the stress of waiting my heart raced. As my heart raced, in the cath lab she heart scared the doctors with going into Ventricular tachycardia. It was too close. But Becca hung on but the news was not good. They couldn't balloon the valve and her heart was thickening even more. They told us that she would be ineligible for a heart transplant due to her immune and hematologic status, but they would present her case to a team of Cardiologists and see what they. That February was a month of waiting for news for Becca's heart.
The team decided to try to fix the parts of her heart they could and see if that would buy her more time. But it was risky with her severe biventricular hypertrophic cardiomyopathy. The day before the surgery would arrive and so would her exposure to Chicken pox. Ian had broke out and the phone call to the doctors would push the surgery back. Her heart was pounding so badly that her tiny chest shook from the force.
No stethoscope needed to count her heart rate. Becca was blue, often on oxygen, and she was having spells of oxygen not reaching her brain. They thought the pulmonary valve was intermittently clamping down leaving her for a few seconds with eyes rolling back into her head.
March 27, 1990 Becca faced open heart surgery and our church had people signed up to pray in 15 minute spots around the clock for the day of her surgery and the days that followed. We knew that she may not survive the operation, but we no choice but this last ditch effort to save our dying baby.
Seeing her after the surgery was awful as she was swollen and she was clearly not doing well. A high fever signaled a staph infection, her chest had filled in with lymph which tests later revealed her immune system was non-existent. Slowly, with prayers, Becca's status improved. One day I arrived in the Pediatric Intensive care unit to see her up and out of bed in a baby swing dressed in little angel heart pajamas. My daughter's heart was mending.
Time would heal the repairs on her heart, and within months Becca would have new strength. By her first birthday she sat up for the very first time and she would no longer be blue when we tried to give her a bath. Time on oxygen was limited and with being on IVIG (to boost her immune system) Becca was beginning to thrive. Equipment and 24 hour staffing arrived in our home, and some days it was a full-time job for the nurse and I to manage her complex care. But I didn't care because my daughter was still here.
I would put my hand on her tiny chest and marvel at how quiet her heart was now. The hypertrophic cardiomyopathy was still a concern, but for today my daughter's heart was quietly beating in her chest.
Every visit to the Cardiologist would send my heart into panic. At first, every time I would go, it was always more news that things were deteriorating. However, as time would move on, the visits caused less panic and her heart became more stable. No more congestive heart failure.
As time wore on, the visits revealed a lessening of the thickening in the walls of her heart. By age 7 the Pediatric Cardiologist told me that they could see no residual Hypertrophic Cardiomyopathy. After the visit I stood on the top of the parking garage looking up over downtown, looking at the blue sky with scattered clouds while my heart soared, I yelled out at the top of my lungs,“Thank you for the miracle”.
Becca’s heart can scare her and medications have caused her heart to race. A third degree heart block at age nine caused her heart to scare us again. However, at age twenty-one an atrial fibulation, found during a visit to the Adult Congenital Heart Defect Clinic, would give us news from the Cardiac MRI that her heart was functioning much better than they originally thought. Now we just wait, continue to follow-up and pray for Becca’s heart.
Hearts .... Valentines Day... Congenital Heart Awareness Day, Heart Month, Noonan Syndrome Awareness Month. Only appropriate.
For Becca we got a miracle and we know that every day with Becca a miraculous gift. A confirmation that resonated with me after listening to the RASopathies Foundation’s webinar, presented by Dr. Gelb, last night about the prognoses of some of the sickest wee ones with Noonan Syndrome. (Click Here To See This Webinar: http://www.youtube.com/watch?v=g6mJ9nnD2pg)
I am honored to be involved with the RASopathies Foundation and we pray that doctors will learn more to help those with Noonan Syndrome and other RASopathies.
About Ann: Ann is the author of the book Tiny Titan which you can purchase at the RASopathies Foundation’s online store www.shopteamras.com. Proceeds go to the RASopathies Foundation for research and/or educational services. For more information on the RASopathies, please visit www.teamrasopathies.org or for more information on Noonan syndrome, please visit www.teamnoonan.org. To become a guest writer, please email firstname.lastname@example.org.