Josh’s Heart Story
| Told by his mom Robyn.
Diagnosis: Tetralogy of Fallot  It was obvious to me when we went to see the pediatric cardiologist that Friday that he knew he was looking to confirm that something was wrong. When he finished the ultrasound, he took us into an office next door and explained that our baby had Tetralogy of Fallot, a heart defect that is characterized by 4 abnormalities in the heart; a ventricle septal defect (VSD) or a hole between the two ventricles, an enlarged aorta that was shifted to lie over the hole, the right ventricle is more muscular than normal, and pulmonary atresia (his pulmonary arteries were very narrow). He drew for us the configuration of a healthy heart and then the configuration of our baby’s heart. The doctor further explained that our baby would need open heart surgery to place a shunt between his aorta and pulmonary artery a day or two after he was born and then he would have another surgery, probably before he turned 1, to repair his heart. We were both in total shock. We were not expecting our lives to be completely thrown off balance in that one hour visit. I remember getting into the elevator to leave the hospital and just bursting into tears! This was our first baby! How could this be happening? As the pregnancy continued, we saw our pediatric cardiologist each month for an ultrasound. We were put in touch with the Prenatal Center at Columbia Presbyterian Hospital in NYC. The remainder of the pregnancy included many trips to Columbia where we were switched to a high-risk pregnancy obstetrician, saw a pediatric cardiologist, met with a genetics counselor, had an amniocentesis, took our labor class, toured the Neonatal Intensive Care Unit (NICU), met with the surgeon, etc. Since we now knew our baby’s condition we tried to do everything we could to prepare for a delivery and stay at Columbia. I wanted to be comfortable and familiar with the hospital and doctor that would be delivering my baby so since we were close enough to the city, each month we would drive down to see the high-risk OB-GYN. The Prenatal Center at Columbia was very helpful and even set up a tour of the delivery floor and NICU for us. Delivery was induced two days before the due date because my amniotic fluid was low. Josh was born on March 1, 2008 at 8 lbs 10 oz and 18 ½ inches long. We were not able to see Josh after he was born, as the doctors immediately took him for evaluation. My husband was able to see him within the hour, and I was able to meet Josh for the first time later that evening when he was moved to the NICU. I didn’t care how may monitors he was hooked up to when I first saw him. To me that just made me feel comfortable that they were keeping a very close eye on him! Josh was under constant supervision and the monitors indicated his oxygen saturation levels were at 80% to 90% (normal is 100%). Three teams of doctors performed echocardiograms (echos) on Josh to figure out how his lungs were receiving oxygen. Finally, the last team found many collateral vessels, which usually disappear during development, that were still present and were carrying blood to Josh’s lungs to be oxygenated. His body knew what to do to makeup for his defect! These vessels were hard to see, even during the echo, so that is why doctors were not able to detect them inutero. Josh was taken off a hormone that kept the ductus open where they were going to place the shunt, and he was eventually taken off the respirator, his IV line was removed, and he started feeding. By the end of the week the doctors told us that Josh did not require immediate surgery and they wanted to discharge him by the weekend! We were thrilled, but at the same time we were also scared. We were going to take a new baby home for the first time and we were worried how we would be able to take care of him, especially when he was no longer on all the monitors! Before we could take Josh home we had to learn how to give him two oral medicines and how to place his feeding tube, as his condition made him too tired to finish his bottles. Initially this was intimidating, but we were both determined to take Josh home if that was the only thing keeping him in the hospital. At 1 month old Josh had a diagnostic catheter procedure to measure the sizes, the blood flow, and the pressures in his collateral vessels. This was followed by another catheter at 3 months old where 2 stents were places in Josh’s pulmonary artery to open it up where the undersized valve was located. Then at 5 months old, a 4 hour catheter was performed to open the stents further and to do a complete diagnostic map of all the collateral vessels for the surgical team. After the third catheter, the medical team decided they would give Josh two more months to grow and recover from the catheter procedure and then he would be ready for his repair surgery. At 7 months Josh was a very strong little man! He still used the feeding tube to supplement some of his meals. This means each feeding usually takes about an hour to complete. I have heard people have problems with children using feeding tubes, however it is difficult for Josh to gain weight even with the tube so we think it was definitely the appropriate choice for him. He would no doubt be much smaller and not as healthy if he did not have it. There were some weeks when Josh didn’t gain weight, but he has never lost weight. Aside from his feeding tube, it would be difficult to tell from his appearance that he has a heart defect. In preparation for Josh’s open heart surgery, a physical therapist from our county came to our house twice a month to make sure his development kept on track with the goal of teaching him how to crawl before his first open heart surgery. This was proactive on our part. We felt that however we could help Josh we would! The physical therapist said that if he already knew how to crawl before the surgery, he would start crawling and be able to continue with his development better after the surgery because he wouldn’t have to learn how to do it when the movement might not feel comfortable. Instead Josh learned to crawl on his own, just with some extra pointers on how to do it! Just before he was 8 months old, Josh had his first open heart surgery. This surgery rerouted the collateral vessels that were going from his heart to his lungs to go from his pulmonary artery to the lungs. Dr. Chen also added a patch to his pulmonary artery to make it a larger outflow. He did not close the hole between his two ventricles because that would be too much for one surgery. As it was, we were away from Josh for a total of 6 hours. Amazingly, with family beside us in the waiting room, it did not feel that long.  After we brought Josh home from his surgery, he was back to laughing and playing with his toys right away! He hesitated at crawling for a few days, but then was off again! And we NEVER put the feeding tube back in! Josh was able to start finishing his bottles on his own! He never showed any resistance to drinking them or to solid food when we started introducing that. Josh’s next procedure was a catheter at 14 months old to see how everything was working and to balloon open any narrowings in his vessels if possible to help him be at his best for his 2nd open heart surgery. This surgery will be to close the whole between his two ventricles and open up the flow to his main pulmonary outflow. We are hoping this surgery will go as smoothly as the first one and Josh will be bounce back quickly again! We are definitely not looking forward to the time we will be spending at Columbia again with Josh, however we are very much looking forward to our son being able to move on. With the support and friendships we have made through It’s My Heart, our family has been able to cope with Josh’s condition and realize that we are not alone in our journey. |
~Robyn, July 2009 |