Mirabel Atkinson

Nine-year-old twins, Mirabel and Sofia, share a special bond.  Born at 32-weeks, they were more exceptional than most identical twins in that they shared a placenta and an amniotic sac.  But one significant difference they have is that Mirabel was born with a congenital heart defect, or several to be more precise, including hypoplastic right heart, transposition of the great arteries and a couple others.  We were fortunate to know about all of these complications before our girls were born, but that didn’t change how challenging the pregnancy and early years have been.

Being born early, they both required time in the NICU.  Sofia mostly just needed to grow and was discharged at about one month.  Mirabel’s case was different and the doctors anticipated her needing the first of three staged surgeries (the Norwood, the Glenn and the Fontan) within days of birth.  But because she was so small, we all waited and watched—through many good and bad days.  And then at two months, when the doctors finally determined that she could hold off on the Norwood procedure and have any necessary changes to her heart combined with her Glenn, they discharged her.  We frequented the cardiology clinic from that point on, often with several visits a week.

Mirabel had her first heart catheterization at about 6 months.  I thought it was the end of the world when we handed her over to the anesthesiologist and I watched her terrified eyes silently asking me what was happening.  But I learned that wasn’t anything compared to surgery; she had her first open heart, the Glenn, at 8 ½ months.  Mirabel’s strength and tenacity had already been proven and she did remarkably well during her Glenn and was discharged after only 6 days.  We maintained regular weekly and then monthly visits to her cardiologists.  She had a second catheterization, mostly for diagnostic purposes in preparation for her next surgery, at about 15 months old and then the Fontan surgery at age two.  Once again, she responded well and was discharged after only 8 days.

We knew Mirabel wasn’t “cured” at that point.  The regular cardiology check-ups, her slower growth (compared to her identical sister), daily medications, how quickly she caught colds and the flu, and her general lack of energy all served as reminders to us that she was different.  But we tried to act and live as normally as we could.

And then at age 5 she needed another heart catheterization to close a fenestration (vent hole) in the hope of improving her oxygen saturation levels.  We had always been open about her heart condition and frequently looked at photos, but she didn’t remember any of the previous interventions, and so when we told her she needed to have the cath, she didn’t take the news well.  It was really hard listening to her ask questions like, “how big of a knife are they going to use to cut me open…will it be like the ones in the kitchen?”  But, guided by an excellent social worker, we made it through with only a few minor complications.  Her kindergarten class made a giant heart card with well wishes and all of their signatures.   And we thought we were done for a LONG time.

Then during the summer when she was 6, we visited the cardiologist for a regular follow-up and he told us that she needed another “surgical intervention” right away.  Eleven days later she was in surgery to enlarge a hole (VSD)—a “minor” open-heart surgery, as they go.  Mirabel was the calmest of anyone in the family; Sofia struggled the most.  Sofia didn’t like the attention Mirabel was getting and was scared about what she was hearing.  We all had to work through this together as a family.  She healed a little slower than the previous two open-hearts, but was still only in the hospital 8 days.  And she started first grade four weeks to the day after her surgery.   But it takes time for everyone to recover from something like this.

Two and a half years later, Mirabel is doing well and we are relatively “normal” again.  Her last surgery granted her more strength and energy than we had ever seen.  She has been well enough to play soccer with her sister in a neighborhood league, a far distance from when eating or crying too hard would wear her out and she would turn blue.

While Mirabel’s road hasn’t been easy, and her journey to keep her heart working as best as possible isn’t over, we have met many exceptional people and made some wonderful friendships with those having similar experiences.  We know what a courageous little girl she is and when we see fits of stubbornness we are quickly reminded that her stubborn attitude is what has kept her going through rough times.  Sofia has been her constant cheerleader and their bond and love they share will remain strong forever.

Rayna

Rayna Schuh

In June of 2006 my husband Tim and I found out that our family would be blessed with the gift of baby. As you can imagine we were excited and anxiously awaiting that first Dr.’s appointment to hear our new baby’s heartbeat. We had no idea if our baby was a boy or girl. Nor, that the heartbeat we heard at that appointment was coming from a heart that had a critical congenital heart defect.

Rayna was born February 16, 2007. She was perfect, or so we thought. We took Rayna home and just thought she was a tiny little girl who tired easily and didn’t gain any weight. After many trips to the doctor we were reassured she was fine and I was a paranoid mom.

In the summer of 2010, Rayna was diagnosed with strep throat and the doctor heard a disturbing heart murmur. I thought, “Well, everyone has a heart murmur.” After some thought, we took her back 2 weeks later and requested that her heart be checked. Rayna’s little brother had a hole in his heart that we needed to check, and I had that “mom feeling” that something was not right. That appointment would become the beginning of our journey into the world of Congenital Heart Defects.

Rayna had many appointments to determine what her defect was. In September of 2010 she underwent a sedated MRI and we were told she has a heart defect known as Total Anomalous Pulmonary Venous Return -TAPVR – and a very large ASD or hole in her heart. Her defect is usually fatal if not detected in the first few weeks of life but she was 4 ½ years old. We were told that this is unheard of and left the hospital wondering and questioning everything we did. At one of her first cardiology appointments I had a doctor take my hand and look me in the eye saying, “Mom you did not do anything wrong to cause this. By the time you heard her heartbeat for the first time, her heart already had this defect.” These words still echo in my heart. On October 17, 2011 we set out for the hospital and it was the longest ride of my life. From the backseat my beautiful girl called for me and as I turned around and our eyes locked her words got me through the day: “Mommy today is the day they are going to fix my heart and I am going to feel all better.” We turned our beautiful girl over to an amazing team that afternoon. As they wheeled her away, the most wonderful nurse looked at my husband and I and said to us “I will protect as if she were my own.” 7 ½ hours later she kept that promise and we saw our little girl with tubes, needles and hoses everywhere, but she was out and doing well. The relief in my heart and mind was indescribable.

Rayna is now 6. She is stronger inside and out and continues to be our driving force to find the beauty in this world of heart defects and doctors appointments. As each appointment nears we still wait with excitement to hear her heartbeat, just like we did the first time.

Rayna’s heart is mended but we are forever changed. The sound of that heartbeat is priceless.