What a difference a year makes

Jul 11, 2010

I can’t believe that a year ago today, our daughter Sophie was in the hospital after her 3rd open heart surgery. This time to replace her mitral valve with a mechanical one. Sophie’s biggest concern that morning was giving up her sleeper pajamas to change into the hospital pajamas. I had no idea what things would be like after this surgery since an artifical valve meant going on anticoagulants. I was doubly concerned since just three months prior, this surgery was attempted and had to be aborted due to abnormal bleeding which took everyone by surprise and the cause still unknown. We went thru countless blood draws and tests in the three months leading up to the surgery with nothing coming up abnormal. But it’s like Sophie’s anesthesiologist said, Sophie likes to throw us curve balls. Let me tell you more of her story.

I was born with Holt Oram Syndrome which is a hand/heart related defect that presents itself in various forms. I was born with curved arms, no radius bone and 4 fingers on each hand (no thumbs). I also had a VSD that closed on it’s own yet was monitored by a cardiologist. As I grew up I never had a name for it, it was just how I was born. I met my husband, Colin, and 3 years after we were married I became pregnant with my first child. There was some concern that I might pass on something genetic, so we scheduled a level 2 ultrasound and a meeting with a perinatologist. The ultra sound came out fine and my son Connor Daniel was born with a 9/10 apgar score. When I got pregnant with my second child, we had no worries of passing on any of my birth defects. Things progressed as normal until I got a call that my quad screen marker blood test came back with a high risk of down syndrome, we were then sent back to the perinatologist and geneticist. I remember laying on the ultra sound table forever and knowing something was not right when they kept scanning over the heart and leaving the room and coming back. They finally told me there was something wrong with the baby’s heart & referred me to a cardiologist. They also wanted me to have amniocentesis done and discussed possible syndromes that are usually associated with heart defects. I declined the amniocentesis and was committed to continue with the pregnancy no matter what. After meeting with the cardiologist we were told Sophie had a very large VSD that had a good chance of needing surgery.

Sophie was born February 21st, 2006, 7lbs, 4oz via scheduled c-section. They flashed her in front of me & then rushed her to the NICU for oxygen. She had swallowed too much amniotic fluid. I was able to meet up with her about 4 hrs later in a wheelchair and then she was brought to our room than night. The next day we met with countless doctors and had xrays, echos, etc run. She was noticed to have smaller shoulders and her thumb on her right hand was underdeveloped and webbed to the hand. We later found out after genetic testing that she too, had Holt Oram. The first few days with Sophie were rough. She did not eat a lot at a feeding & started to breath faster, get paler, etc. We got her to the doctor as soon a we could and we were told she may not make it long. They started her on lasix, but said it might not help much. We sure were in for a shock when we had full diapers every hr. We woke up every 4 hrs to feed her and saw the cardiologist on a monthly basis. They finally said that they wanted to schedule her surgery for the beginning of August and would go in and patch her VSD. However, when checking with my insurance, was informed that the surgery would only be covered at 50% due to being out of network, but a partner facility. I was forced to switch doctors and hospitals, but am now so thankful I did. The team at Legacy Emanuel was wonderful and got her set for surgery at the end of July. They informed me that not only did she have a VSD, there was a small ASD as well and her heart was very enlarged. If she had been in their care from the start, she would have had surgery much sooner.

So on July 27th, 2006, Sophie had her 1st surgery. We were told that if they were to successfully patch the VSD and stitch closed the ASD that she may be done with surgery. That wasn’t how it was to be. We get word from the surgeon that it was a very tricky surgery and they had trouble getting her heart started again. They also said that after they closed her up, her mitral valve was leaking. They were confident, however that she would be able to go home on some medications and they would attempt to repair it around age 2 or 3. We filled prescriptions for lasix, digoxin, and captopril & after her recovery, were sent home. We struggled with giving her medication 3 times a day that she kept chocking & gaging on & throwing up her food which we spent so long getting her to eat. Everyday I had an uneasy feeling every morning I left for work. I came home the night of Aug 8th to find her breathing very fast and retracting her chest. I said we are taking her to the ER. We arrived and they got us right in and determined she was in severe heart failure. They rushed us up in an ambulance to Portland, a good 2 hr drive away. Her next open heart surgery was Aug 11th, just two weeks after the 1st.

This time they had to leave her chest open a few days due to swelling. They said that they got a pretty good repair at first, but as the days went on, the valve started leaking more, but she was stable. We adjusted her medication and eventually switched to pills which we crushed. It was nice not having as much liquid for her to swallow. We were sent home and followed up with her cardiologist every 6 months. Sophie started to grow more and sat up at 13 months and learned to walk at 21 months of age. All of this was with help from EC Cares/Early Intervention program.

The next blow came to us when her pediatrician mentioned that her head size seemed abnormally large. We were sent for an MRI and told there was fluid in her head. The thought of her needing head surgery as well seemed more than we could take. We took the 2 hr drive up to Portland to meet with the neurosurgeon. Our minds were full of questions. She said that it was extra axial fluid and due to recent research found that surgery was not necessary.

Life was falling into a comfortable routine, the time between doctor visits was getting longer. Then in January 2009 we get a call out of the blue from her cardiologist. He had consulted with the surgeon and due to the fact that Sophie’s heart was still enlarged they wanted to replace/repair her mitral valve within the next few months. We set her surgery for March 28th since it would be during Spring Break since my husband was finishing school. The week prior to surgery, she got very sick with a respiratory virus, tested negative for RSV, but was not 100% well by the 28th, so surgery got moved to April 10th.

This time we knew the routine. We knew where they wheeled her back from surgery and everything. We had difficulty getting her blood drawn during the pre-op, so we were OK when they said they would just take what they needed after they put her under anesthesia since all her labs for her prior surgeries turned out fine. We were at the Ronald McDonald House taking a short break when I get a call on my phone thinking it was a little earlier for an update. We were told to proceed directly to the hospital ASAP. Surgery had been aborted and the surgeon and doctor needed to speak with us. A lump rose in my throat as we rushed across the street. They said they were about to make the 1st incision, but noticed abnormal bleeding from her arterial line in her leg. They ran tests and her blood would not clot. They swore up and down that they did not give an overdose of heparin. We rushed into see her. She still had to be extubated & there were about 5 nurses around her all applying pressure. A huge bruise was forming in her groin area and down her leg. We meet with the hematologist and after several hours and a substance called factor 7, we got the bleeding to stop. My husband and I were nervous wrecks. We were now no closer to having the surgery behind us and had to figure out what happened before we could proceed. We worked with the hematologist for about 3 months and it was deemed safe to proceed with the surgery, yet with an unsolved mystery of what happened that day. I told my daughter that she was not allowed to meet any more specialists at the hospital after visiting the cardiologist, geneticist, neurologist, and hematologist.

Now I think we are full circle from the start of this post about Sophie’s surgery a year ago today. We know the journey is not over and we will face more surgeries along the way. I however have meet some wonderful people along the way and I am thankful of their support. Our last visit to her cardiologist did show that her heart is still enlarged and there was no improvement in her heart function. She is thriving and doing great, yet follow up this Wednesday to see if any changes have occurred. Our cardiologist said if things don’t improve or decline further, we may have to discuss transplant. Not the words I wanted to hear, but in the meantime I enjoy, marvel and love every second I have with Sophie.