Down Right Beautiful

Sofia had another echo this morning. The fluid that was around her heart has NOT returned since we stopped the steroids last Friday. YAY!!!! ... AND... much to the surprise of even the cardiologist, her heart has now returned to normal size!!  This was a huge concern. They were afraid the heart would not be able come back down to size after it had enlarged so much from the pressure of the major gush of blood coming through that leaking valve. BUT it DID... and so quickly!! YAY!!!! ... AND... in three days we will be able to stop giving Sofia one of her heart medications (lasix) that she has been taking twice a day since she was 2 weeks old when she went into heart failure!!  Incredible! They are hoping in maybe a year to be able to discontinue the other one. He said it is highly unlikely the fluid around her heart will come back, but we still need to watch for signs. We also have to watch for puffy eyes, hands, etc after we stop the lasix. But all these are just tiny annoyances in the big picture.

This is all too amazing for us to comprehend at this point. Really. I don't even know how to express it all.

She has 3 more weeks of recovery and we hope it keeps going as well as this. Then she will get her flu shots and a few extra vaccinations...  AND THEN... SHE IS READY TO TAKE ON THE WORLD... WITH NO RESTRICTIONS!!!!!!!!!!!!! And go to school!!!!!!!!!  And travel!!!!!  And just be a kid for the first time in her life!

It's been 3 1/2 years that we've been helping our lilttle girl battle this Congenital Heart Defect (CHD) since before she was even born, and we never thought we would reach this point. Dennis and I need to learn how to exhale now, and figure out how to live a normal life again. Live a normal life with our beautiful little girl for the first time. Life is Beautiful.

A mother's heart has been healed
Because a daughter's heart has been fixed.
.

Home sings me of sweet things,
Life there has its own wings.
My little girl is home again.
-- Dennis Pepe (aka daddy)

Oh... how sweet it is! We came home from the hospital this afternoon after blood cultures showed nothing after 48 hours. No temperatures since we were admitted. They said maybe her body just took a little extra time getting over the trauma of her 3rd open-heart surgery, or perhaps she caught a little bug and she's over it now. Either way, our little girl is home, and happy, singing in front of the mirror, and dancing with her shadow. The universe is in balance again.

We see the cardiologist Friday for an echo and see how the fluid around her heart is doing with the steroids she is on. It seemed to be shrinking in the last echo, and we hope it continues to do so and does not return.

Thank you to friends and family who sent us notes and e-mails, and to all those people we've never even met who took the time to contact us and share their journeys with us and gave us encouragement. It warms my heart to find out there are hundreds of people out there with our little girl on their minds and in their hearts. We have truly been blessed to be in this circle of love. Our little girl has touched many lives, and we are the luckiest parents in the world.

Today was a good day. Started feeding her again and she's tolerating it very well. She's looking more like herself except for that central line with about 5 ports and wires hanging out of her neck. She's been disconnected from all machines and just gets hooked up every few hours to check her heart rate, breathing rate, oxygen levels, blood pressure, and temperature. And all numbers are good. She had her first post-op echo today. It's a good day when the cardiologist says, "Her heart looks spectacular." He said she has a really funky looking mitral valve, but it's a functioning valve. There is a minor leak that they expected, but the major leak is all patched. It's an even better day when he says it looks like this is her last surgery and she should be off all heart meds within a year. WHAT?! We NEVER expected to hear those words. It's a new life for our beautiful, strong, brave little girl! Watch out world.... here comes Sofia! 

Wed Aug 11 - We check into the hospital at 6am tomorrow, and expect surgery to begin at 7:30am. It will take about an hour to prep her with all the IVs, tubes, monitors, and such before the operation actually begins. Then they will open her rib cage, hook her up to heart/lung bypass, a machine will be breathing for her, they will stop her heart, drain the blood, and begin the repair. The cardiologist will update us every step of the way as the surgeon tries to repair the tare by the patch that was put in at 5 months of age, and attempt to repair her mitral valve using her own tissue along with pig intestine. We pray that he will not have to insert an artificial mitral valve and put her on blood thinners. The next step is to hope her enlarged heart will return to normal size and function properly once the valve is repaired/replaced. The chamber of the heart that is enlarging is used to all the pressure from the leak, and they hope it can readjust to the lower pressure and continue to pulsate normally.

Another angel is on the way
    We were thrilled when we found out we were going to have a baby… I was 4 weeks pregnant..  We were even more thrilled when we found out we were going to have a little baby girl… I was 20 weeks pregnant.  Somewhere between this time period, fear entered our hearts and confusion entered our minds.  
    We found out at 9 weeks pregnant that there was a 1/11 chance of our baby having Down syndrome.  I was scared and devastated.  We had planned on having just one child and this was not the child we had planned.  We tried to put the possibility out of our minds, but I was 38 years old and I knew the statistics that came along with being a pregnant woman of “advanced maternal age,” as all my paperwork so clearly pointed out.  We decided not to have further testing because it did not matter what the results would be.  I had waited my entire life to have a daughter and I was going to enjoy the pregnancy and love my daughter no matter what.  So we decided to just enjoy watching her grow through the sonograms and look for the typical markers for Down syndrome.    

   It was the week before Christmas and we were excited to find out the sex of our baby, the day had finally come for the sonogram.  After telling us that it was a girl, there was an eerie silence as the technician continued scanning the sonogram. Our moment of excitement suddenly turned into fear, and everything from this point forward seemed to become a blur.  In a very thick accent she mumbled something about the heart and a hole.  We could not understand what she was saying, and before we had time to process her words, she left the room to get the neonatologist.  As we waited for the specialist, time stood still and I could feel the fear and tears building from deep within my soul. The doctor took a close look at the sonogram and told us that our daughter had a huge hole in her heart.  She said this type of Congenital Heart Defect (CHD) was very common for babies with Down syndrome and combined with a few other things she saw on the sonogram, she said our baby most likely had Down syndrome. 
   This was all too much information to process at once.  We feared for the life of our unborn child and wanted to do everything we possibly could to help her and improve her chances during the pregnancy and delivery.  In our moment of panic, fear, and ignorance, we agreed to have an amniocentesis right there on the spot.  We thought if this could help the doctors be prepared for any complications along the way and during delivery, we should go ahead and find out for sure.  By the time we got the call a few days later confirming that our daughter did indeed have that extra 21st chromosome, it didn’t even matter.  All we were concerned about was her heart.  We didn’t know what any of this meant. All we knew was that our little girl had a big problem.  
    We were immediately sent to our obstetrician who was full of condolences. We also met with a genetic counselor that offered us a simple genetic definition and diagrams of that extra chromosome. She also told us that we can expect our child to have a wide variety of developmental delays of unknown levels. What our obstetrician offered us was her cell phone number so we could call her over the weekend with our “decision," as she assumed we would terminate the pregnancy because all of her previous patients had. We were 20 weeks pregnant and had a very small window of time should we also choose that route.  
    What we were not offered was what it meant to have a child with Down syndrome.  We were not offered any real counseling or information on children with Down syndrome.  We were not offered contact information to families or support groups who could share their experience and firsthand knowledge of what it means to have a child with Down syndrome. This is what we needed most, not pressure to abort our beautiful baby, and not just a clinical genetic definition.  We felt completely isolated and alone.
   One week later, the day before Christmas, we took our first step toward becoming members of a wonderful new community of support and inspiration.  We discovered the Down Syndrome Connection web site and contacted Kathy Getman.  One month later, and six months pregnant, we attended our first DSC gathering at Peter Piper Pizza.  We met some beautiful children and wonderful families.  Many parents had heard about us and were excited to share the stories of their babies and their heart defects.  Almost immediately these moms pulled up their children’s shirts and so proudly showed us the scars from their heart surgeries.  I wouldn’t understand this show of pride until months later after our own baby had her surgery scar... its like a badge of honor, warn very proudly, showing that they made it through the dark times and were able to stand strong on the other side.
    The Down Syndrome Connection made a world of difference in my family’s journey with Sofia, especially in the beginning when we were entering new and unknown territory and didn’t even have our beautiful baby in our arms yet!  They were a voice of acceptance and assurance in a time of confusion and fear. They were warm and welcoming and were the first ones to say “Congratulations on your little baby girl” and really mean it.  They were open arms giving us a big loving hug full of joy and celebration.


Sofia’s Journey of Courage
        Our daughter’s journey of courage began with her very first echocardiogram when I was 20 weeks pregnant. We had just found out that her tiny heart had a huge hole and was missing a valve (complete AVCanal - AVSD). We were terrified. She was born two weeks early on April 15, 2007 weighing only 5 pounds 6 ounces.  The cardiologist said her heart looked very strong though and there was no need to worry about the possibility of heart failure for at least three or four months. We remained in the NICU, however, until she was able to suck and eat on her own. Two weeks later, we were finally able to take her home and help her get strong enough for her scheduled surgery at five months of age.

Just two days after we got her home, she went into heart failure. We returned to the hospital for another three weeks and watched as she lost weight while the doctors tried to stabilize her tiny heart and deal with various complications brought on by heart failure.
    Because her heart required all of her energy to keep it pumping, she didn’t even have the strength to eat, so when she was just four weeks old and weighed less than six pounds, she was operated on and had a feeding tube put directly into her stomach through her belly.  Once they found the right combination of heart medications to ensure her heart was stable, and she could tolerate food being directly pumped into her stomach through the g-tube, we were finally able to take our sweet baby home again.  By now she was almost six weeks old, but had spent only two days of her life at home.  
    The following four months we struggled to keep her healthy and help her gain the weight and strength she desperately needed to endure her open-heart surgery. Those months were filled with weekly doctor appointments, blood tests, echocardiograms, sleepless nights, and many tears. Unfortunately, she stopped gaining weight when she reached nine pounds. The doctors had wanted her to weigh thirteen pounds before they operated, but she was five months old and could not wait any longer.  She was four pounds short of her goal when the date of surgery arrived in September.

We thought we were prepared for her surgery with all the research we had done and by talking with other parents who had already reached the other side of what we were just beginning to embark upon, but nothing can really prepare you for seeing your tiny baby lay in the pediatric intensive care unit after major open-heart surgery.  She was recovering quite well after surgery and came off the ventilator that very same day, in fact, the doctors said she was recovering faster and better than most babies, but everything changed very quickly.  Sofia went into heart failure just two days before we were supposed to take her home. Our daughter’s heart had torn apart, the stitches did not hold. As her heart split open again, so did mine. I was terrified that neither one of us would recover.
    Sofia, our amazing daughter however, gathered the strength to pull through. This tiny baby had been cut open twice, was bruised and battered, a machine was breathing for her and pacing her heart, she had more tubes and wires and IVs in her than you would think such a small baby could endure, but she eventually healed. It seemed she was the strongest of us all. She was the one who carried us through those dark days and eventually into the light again. It was her joyful smile, just a few days after her second surgery, that let the sun shine into our lives again.

The following links will take you to articles from three wonderful organizations containing more information on Sofia's journey:
Beads of Courage   Mended Little Hearts  La Leche League

If you would like to read a touching story about another parent's journey with thier daughter, here is an article written by a friend of mine that was published in Washington Parent magazine, November 2006 (click on the article title below to access it). Adrienne gave birth to her daughter Clare and found out hours later that she had Down syndrome and a heart defect. She was devestated and struggled to come to terms with what it meant for their future to have a child with Down syndrome.  To quote from her article... "Almost four years later, I remember just how desperate I felt at the time, and I wish the 'me' I am today could go back in time and tell the 'me' back then just how wrong I was." .... " I believe with all my heart and soul that Clare is exactly as God intended her to be. Given the choice, we wouldn’t change a single thing about Clare. We think she’s perfect. Just perfect."  - from Days and Dreams Filled with Promise by Adrienne Vadell Sturges.