Oliver before his first surgery

Caleb and Oliver, November 2006

Oliver Leo came into this world on Sunday, July 31, 2006 at 9:50 P.M. He is our second child. My husband and I put a lot of thought into the decision to have a second child. Our older son, Caleb James has DiGeorge Syndrome and Tetralogy of Fallot with Absent Pulmonary Valve Syndrome along with a list of other ?minor? diagnoses.

We spoke in depth with the doctors regarding the risk of having another child with congenital anomalies. Given the fact that Caleb does not have the typical chromosome deletion associated with DiGeorge Syndrome, they saw no real increased risk for us.

On Thanksgiving Day 2005 we were overjoyed but hesitant to discover that we were pregnant. Everything progressed normally other than really bad morning sickness. All through the pregnancy I just knew something wasn?t ?quite right?. Everything went through my mind from a cleft lip to multiple digits to a ?minor? heart defect. At 17 weeks gestation we had a high level ultrasound with a perinatologist to make sure everything was ?ok?. We thought we would never hear those words again ?We think there is an abnormality with the baby?s heart.? It was just as devastating to hear it for the second time as the first. The next day we had an echo with our cardiologist. He agreed there was a heart defect, however since it was so early in the pregnancy the diagnosis was not clear. He thought it was either Tetralogy of Fallot with Pulmonary Atresia with MAPCAS (Major Aorto-Pulmonary Collateral Arteries) or Truncus Arteriosus. About a month later the cardiologist was pretty sure it was Truncus Arteriosus. One month later he was positive. After a five year relationship, Dr. Younaszai, our cardiologist told us he was leaving the area. As sad as we were to no longer work with Dr. Younaszai, he recommended, and we chose a wonderful doctor to take over the care of our children. After our first meeting with Dr. Qureshi we knew we had made the right choice.

We began preparing ourselves for the birth of our baby and the open heart surgery to follow soon after. Our cardiothoracic surgeon, Dr. Roger Mee had retired in December of 2005. This was difficult for us at that time knowing we would need his skillful services for Caleb again. Now this loss became more significant knowing both of our children would require open heart surgery throughout their entire lives. After careful investigation, we met with Dr. Brian Duncan to discuss the potential course of treatment for our baby. We liked his approach. He had worked with Dr. Mee for the last five years and was at the same hospital.

Just before a scheduled induction, Oliver decided to make his entrance. We held him briefly in the delivery room then he was whisked away to be assessed in the Neonatal Intensive Care Unit. An echo confirmed that Oliver had Truncus Arteriosus. Otherwise he appeared healthy. Cord blood was taken at delivery for numerous tests to determine if there were other anomalies or syndromes present. Following in his big brothers footsteps, these tests showed Oliver had a T cell deficiency and did not have the chromosome deletion associated with DiGeorge Syndrome. Oliver was doing great except for feeding difficulties so a nasogastric tube was inserted within the first day to help him eat. He continued to do well except for the feedings. Knowing that our time was limited before heart failure would begin and lead to his first surgery, we were anxious to bring him home and enjoy our time as a family of four. After five days Oliver came home. He had an apnea monitor and NG feedings.

We were able to enjoy four wonderful weeks at home. Oliver?s heart failure then became so significant that he needed to be hospitalized and prepared for his first open heart surgery. Everything went pretty smoothly until the night before surgery. Oliver?s most recent echo showed anomalies of his coronary arteries. The doctors thought this may make a ?complete repair? impossible at this time. The extent of the coronary artery anomalies could not be completely determined until they were performing surgery. Needless to say, this was shocking to us.

On September 7, 2006 Oliver underwent his first open heart surgery. After eight hours in the operating room we were able to see him in the Pediatric Intensive Care Unit. Thankfully, Dr. Duncan was able to do a ?complete repair?. He separated Oliver?s pulmonary artery from his aorta, inserted a conduit homograft valve in the pulmonary artery, closed a large atrial septal defect and a ventricular septal defect. Oliver?s recovery went very well. He was in the PICU for six days. He was extubated from the ventilator after three days post operatively. Oliver had significant stridor with his breathing after extubation as many children do. However, after several days this did not improve. Vocal cord paralysis was suspected. A nasal scope of Oliver?s throat was done. His vocal cords looked great but the ENT doctors noted subglottic stenosis (narrowing of the airways just below the vocal cords caused by a buildup of scar tissue). This was causing the stridor. By evening Oliver was gasping for breath. He had been scheduled for discharge the next day. Instead he would be going into the operating room for an emergency bronchoscopy. The goal was to remove some of the scar tissue and open the airways in the hopes of avoiding a tracheotomy. After the bronchoscopy, the ENT doctors believed they were successful in removing the scar tissue. After a brief stay in the PICU we were back on the floor preparing to be discharged home. Oliver came home after a total of sixteen days in the hospital. He was still on a feeding tube since weight gain was an issue and an apnea monitor due to the subglottic stenosis. We were overjoyed to be home and terrified to be adjusting to a life with two special little boys.

From a cardiac standpoint Oliver was doing well. Within a week of discharge he went back to the OR for another bronchoscopy. Since weight gain was still a concern, his calories and volume were monitored closely. After a lot of hard work and allowing Oliver to prove himself, Oliver is eating straight breast milk and gaining weight on his own. At his six week post op appointment everything looked great. He still has a small VSD and some truncal valve leakage. He is on digoxin and Lasix. ENT will continue to watch him closely for a year or two. He will go back into the OR for another bronchoscopy in December. To prevent reflux and protect his airways, Oliver is also on Pepcid. Oliver has been receiving speech therapy to assist with his feeding issues. He is currently being evaluated for concerns regarding developmental delays. We are also working with Genetics and Immunology to try to determine the extent/cause of Oliver?s issues.

We are slowly adjusting to family life. Things can get very busy and hectic with two children with multiple needs. We are hoping that in time we will all settle down into a routine. I would like to give our thanks to a few people. First I want to thank all of our family and friends for their prayers, positive support, and the courage to continue to get through these trying times. Second I want to thank The Cleveland Clinic Foundation, the dedicated P.I.C.U. staff and the step-down unit. Words cannot express my gratitude to the skillful Dr. Duncan and the compassionate and understanding Dr. Qureshi for the comprehensive care given to Oliver and our entire family. Last but certainly not least, all of the professionals everywhere whose efforts help to give our children and others like them the healthiest and most normal life possible have a special place in my heart.

Nothing gives me greater joy than being a mother to Oliver and Caleb. It is also my greatest heartache. The most beautiful sight I have ever witnessed is these two brothers together. Their bond is like no other.

Please see Caleb James? story