First published in Children's Heart Network's Heart Matters Newsletter. Written by Khol's mom, Melissa Martz
When my husband and I decided to have a baby we never dreamed that our first born would have a congenital heart defect. When I went for my routine ultrasound, the technician showed my husband and I our baby, pointing out the feet, the hands, and the heartbeat. She printed off the sonogram pictures for us and I couldn’t wait to show the family. I never had a clue something was wrong.
The next day I received a call from a doctor whom I didn’t really know, my regular doctor being on holidays. The doctor tried to explain that they noticed that the baby’s heart was on the wrong side of the body, called dextrocardia. He said some babies can live normal lives with this, while others will have more heart defects. I would have to go to BC Women’s Hospital for a fetal echo and more investigation.
I was in shock, in total disbelief. I was supposed to be leaving within a couple hours for a weekend away with my sister, so I called and told her I was not up to going because something was wrong with the baby—and then the tears started. I wasn’t sure how to get hold of my husband because he had just started a new job, but after some time my mom got hold of him and he promptly left work to come home. My mom then called the doctor back to ask more questions because I was in shock and didn’t understand what was going on.
I cried and cried, but held on to the hope that my baby would be okay. Over the next while I prayed and did Internet research. The next week they scheduled a fetal echo at BC Women’s Hospital in Vancouver. At the appointment the cardiologists studied the echo for what felt like forever, and it’s no wonder, as our baby had a very complex heart: dextrocardia, double outlet right ventricle, VSD, and ASD. They weren’t able to tell how well his pulmonary artery and surrounding vessels had formed. They gave us the option of terminating the pregnancy, but that wasn’t a choice for us. Then they explained a three-stage surgical “palliative” procedure that the baby would need.
After that I had fetal echoes monthly so the cardiologists were able to develop a plan: a BT shunt operation at birth, a Glenn shunt at 4 to 8 months, and a Fontan between 2 to 3 years. All of this information was shocking and difficult to deal with at times, but we have a great support system that helped us through the difficult times. We found out we were having a boy, which really helped us get excited for the birth and helped us prepare for our son. We decided to name him Khol Wally Martz.
The cardiologists decided I needed to move closer to BC Children’s when I was 37 weeks pregnant, so I moved to the Easter Seals House. I was supposed to be induced December 8, which didn’t happen because there were no ICU beds available. It kept being delayed due to a lack of ICU beds, with the hospital considering transferring me to Alberta and Seattle—but still, no beds. They finally found a bed for Khol at Legacy Emmanuel Hospital in Portland, Oregon. What a shock! I was devastated at first.
We had our passports rushed out to us by a family member, we quickly packed a small bag, and that evening we were transferred to Portland via ambulance and airplane. Now not only was I away from my family and support system, but I had no idea about this hospital and its staff. But after we arrived, I felt better right away—it’s a gorgeous hospital with incredible staff. Some of our family drove out to be with us the following day. The evening of December 12 I was induced and by 2:13 the next day Khol was born. He was so precious, my husband and I just couldn’t stop looking at him! I was able to hold him for a few minutes before they took him to get assessed, with my husband accompanying him.
The next few days we spent staring at him and being by his side. We were able to hold him with the help of the nurses. His official diagnosis is dextrocardia, pulmonary atresia, single ventricle, levo-transposition of the great arteries, double outlet ventricle, and a severely narrowed and deformed left pulmonary artery.
At 3 days old on Khol had his BT shunt operation. What an emotional day! It was hard to see him with all the tubes, drains, and wires, but we were prepared and thankful he was alive. The next morning they were able to extubate him, and the doctors and nurses were all surprised at how well he was doing. Three days later we were able to come back to Canada, staying at BC Children’s until December 23.
The Glenn surgery would be May 28, 2010. I prayed and prayed, putting my trust in God; I kept telling myself that God couldn’t take him away from us after such a short time. Some days I felt at peace about it and other days I felt like a ball of nerves!
On May 27 we got up bright and early and checked him in for surgery. What a terrible feeling to hand your baby to the nurse for surgery! Khol was smiling and laughing at the nurse as they walked down the hall. Hours later, at around 1 p.m., I called the ICU to see if Khol was there, but not yet. Finally, around 2 p.m. a friend who had her daughter in ICU came and told me Khol was there. Then the surgeon, Dr Campbell, came in a few minutes later to tell us how it all went. He said it went pretty well, but since Khol’s heart is on the right side the surgery was a bit more complicated. Also Khol’s pulmonary arteries were very small and Dr Campbell did extensive repairs by attaching cow tissue to these areas. Once again our baby was hooked up to many machines, tubes and wires, but he was a fighter and he was able to go home 6 days later.
Over the next couple years Khol developed quite normally; he crawled at around 9 months and started walking at around 14 months old. As he started to walk and then run we noticed that living with a lower oxygen level was affecting him and he had to pace himself. Khol became a big brother in August of 2010.
When he was around 2 1/2 years old we started to notice more bluing around his lips and darkness under his eyes, and the cardiac team confirmed that his oxygen level was getting too low and it was time for the next step—the Fontan surgery!
The Fontan surgery was booked for August 17, 2011. Once again we put all our trust in Dr Campbell and the surgical team, and prayed for another miracle. The surgery took around 5 hours and when we saw him, Dr Campbell confirmed that all went well.
Khol’s recovery was a bumpy one, with lots of pain and lots of fluid in his lung. Khol ended up having his chest tubes in for almost 3 weeks due to excessive drainage. It was so discouraging; every day I would wake up and watch for less fluid in his drains, and I felt like it was never going to stop! Khol started to hate the nurses, hate anyone that entered his room. He hated his tubes being stripped, and his vitals being taken. He would scream and fight with the staff, he wouldn’t sleep. I had to ask the staff not to disturb him and that I personally would do his vitals and stripping. Once he was up to it we took him out of his room in his wheelchair as much as possible. We walked laps around that hospital, so grateful we had nice weather! Finally on September 3 we were discharged, but had to check back in a couple days to make sure his lungs stayed dry. Once home Khol started to get back to himself and started to smile again, eat again, and gain weight back.
Since then Khol has not had any further surgeries. At some point he may need a pacemaker, but currently we are just monitoring him. We go regularly to BCCH, but at our last appointment was cleared for a full year! Khol is now 6 years old, and in kindergarten. He is currently doing his second year of kindergarten; he has shown some developmental delays. He has also been diagnosed with low muscle tone and some sensory processing difficulties. Khol has a teacher’s aide at school that assists him a couple hours a day.
At home Khol is a very happy boy—he loves life. He has two younger brothers and a puppy that he loves a bunch. He loves to be outside, be with his friends, play Minecraft, and do Lego. He plays hockey in a non-competitive league and is in jiu-jitsu. He is not very athletic, but still enjoys being in these sports. We took him and his brothers to Disneyland in October and it was a fabulous trip! He is an amazing child.
We have lots of hopes and dreams for Khol; we pray for him daily and we try to live life to the fullest. We have no idea how long he will be with us; we pray that it is a long time. Everything we have been through has been worth every second.