I was asked to write a blog posts for the Rasopathies Foundation in February. February is Heart Month and now Noonan Syndrome Awareness month. For me February and heart month brings me back heart fears and heart miracles with my journey with my daughter Becca. (Written for the Rasopathies Foundation and Noonan Syndrome Awareness Month).
My Daughter, My Heart
From the very earliest of moments with my daughter Becca, her heart was at the heart of her issues.
When she was just 2 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.
They found out that Becca's heart had Pulmonary 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, but she was just too severe. Her immune problems, her high white blood counts, her constant thrush and staph infections puzzled the best of the professionals.
At nearly 3 months, they had placed her gastrostomy and repaired her malrotated intestines and with a shot of Fentanyl for the pain she 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 with we were able to restart her heart, but we do not know how much brain damage she suffered, only time would tell.
The NICU staff were talking about discharge by the end of October, and I was seeing something in Becca. I told them of my concern and the staff told me that it was probably fear of bringing home Becca. The panic caused my heart to race. Maybe they were right, I was afraid of the responsibility, I was afraid of the future, 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 mothers intuition was right and thus began the struggle with my mothers intuition and not wanting to see anything more wrong with my tiny daughter.
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 would arrive and the hospital sent our tiny daughter home to make memories as they had told us that they didn't know how long she 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 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 I 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 6 month birthday was on the 3rd 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 peoples 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 and 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 thought. 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 they could 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 intermittantly clamping down leaving her for a few seconds with eyes rolling back into her head. A Zoster booster shot to give her the immunities to the Chicken pox and all but Kristy left for Grandma's as none of the rest had had the pox.
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 they warned us, but we no choice but this last ditch effort to save our dying baby.
Seeing her after the surgery was awful. She was swollen, she was 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 had 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, my daughter was still here.
I would put my hand on her tiny chest and marvel at how quiet her heart was now. But the hypertrophic cardiomyopathy was 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. Every time I would go for many months prior it was always more news that things were deteriorating. As time would move on, the visits caused less panic as they talked of her heart being 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.
Her heart can scare her, medications have caused her heart to race. Third degree heart block at age 9 caused her heart to scare us. At 21 atrial fibulation found at a visit to the Adult Congenital Heart Defect Clinic would give us a news from the Cardiac MRI that her heart was functioning much better than they thought. Now we just wait, follow-up and pray for her heart.
Hearts .... Valentines Day... Congenital Heart Awareness Day, Heart Month, Noonan Syndrome Awareness Month. Only appropriate.
For Becca we got a miracle. We know that there are chances that the thickening in her heart may return as miraculously as it went away. The biopsies of her heart muscle showed us that she has unusual muscle fibers. We know that heart repairs done on these wee ones can wear out, we also know that her right ventricle has to be watched and her missing pulmonary valve may need replacing some day. We know that the lymph after her first heart surgery may be a problem for the next time. We know that every day with Becca a miraculous gift.
A confirmation after listening to Dr. Gelb last night about the prognoses of some of the sickest wee ones with Noonan Syndrome I think about all those we have lost over the years and wonder why is it that Becca survived and they didn't? A bittersweet reminder to my heart of all the Mom's and Dad's who lost their wee ones leaving them with a broken heart. It could have been me.
I am honored to be involved with the Rasopathies Foundation and we pray that the doctors will learn more to help those with Noonan Syndrome and other Rasopathies.