Make A Child Smile

John Nicholas' Story
written by mom Jessica

John Nicholas was born on March 19th, 1999 after a difficult pregnancy. Our family rejoiced at the birth of our seemingly healthy baby boy. The morning following his birth, things went very wrong. My husband went to the nursery to bring John Nicholas to my room, but when the nurse went to his bassinet to bring him to Tim, he was still and gray. She quickly alerted his pediatrician who worked to stabilize him and transfer him to a neonatal ICU in Memphis, TN.

Later that night, we received a diagnosis of Transposition of the Great Arteries and were told John Nicholas would need surgery within a week to survive. He had his 1st heart catheterization at only 4 days old to tear a hole between the two atriums of his heart to buy him more time before his operation as we waited to be transferred to LeBonheur Children's Medical Center. He was finally transferred at 5 days old. John Nicholas had his 1st open-heart operation when he was 6 days old. In the 11-hour operation, his aorta and pulmonary artery were disconnected from their wrong positions and moved to the right ones, his coronary arteries were moved, and the two holes in his heart that had been opened by medicine and during the 1st heart cath were sutured closed. In that moment, his heart was perfect.

After surgery, he recovered well and we were able to go home just one week later. He was on 4 medications which had to be given every 2 hours! During a cardiologist appointment at one month post-op, we were told there was a slight complication, but that it was nothing to be concerned about. When we visited the cardiologist when John Nicholas was almost 6 months old, the tests showed that his heart had suddenly gotten much worse. A 2nd heart cath determined that he needed surgery urgently. His 2nd open-heart surgery was performed just a few days later to remove massive scar tissue from his pulmonary artery, to enlarge his pulmonary artery, and also to remove his pulmonary valve. There were more complications this time and he was in the hospital for over 2 weeks after the operation.

Shortly after we came home from the hospital, John Nicholas began having seizures which required another hospitalization and medicine for months. Within a few months, he began to turn blue for no apparent reason which led to more hospital stays. He also had frequent respiratory infections and ear infections that first winter. He had his 3rd heart cath shortly before his 1st birthday, but a reason for the blue spells could not be found.

Through the summer, we continued to struggle with ear infections. When John Nicholas was 17 months old, an ENT decided to place ear tubes, but first needed clearance from his cardiologist for the surgery. During an exam by his cardiologist, it was determined his heart was struggling again. His cardiologist allowed the ear tubes to be placed, but also scheduled his 4th heart cath for the day after the ear tubes were inserted. Miraculously the heart cath showed that his heart was stable. His ears, however, did not improve staying infected for 8 weeks following the tubes being placed. This led his ENT to choose to perform a 2nd surgery to place larger tubes in his ears and to remove his tonsils and adenoids.

In the months after this, we had relative calm as far as his heart and ears were concerned. We took him to a new cardiologist at a different hospital shortly before his 2nd birthday. The new cardiologist's approach was less aggressive which we appreciated very much. John Nicholas went nearly 2 years with no major cardiac interventions as a result of his cardiologist's "watch and wait" type of practice. It was a welcomed break and opportunity for him to grow. When he was 3½ years old, his cardiologist felt it was time for his 5th heart cath to evaluate the size of his pulmonary arteries. There were problems with the cameras in the cath lab which lengthened his cath considerably and prevented the cardiologist from being able to place stents although he did perform angioplasty to enlarge John Nicholas's pulmonary branches. John Nicholas struggled terribly with nausea and arrhythmias after this heart cath and spent several days in the hospital being monitored. He did very well for a few months, but then began to complain of chest pain and we noticed more shortness of breath. His cardiologist could not find an obvious reason for this.

John Nicholas continued to experience chest pain as well as shortness of breath with activity both of which were getting worse. Our LeBonheur surgeon referred us to a new cardiologist who could see us quickly. The new cardiologist scheduled John Nicholas for his 6th heart cath with the plan to place two stents in the branches of his pulmonary artery. When the cath was performed, his cardiologist determined that the stents would be too little too late. He needed a 3rd open-heart surgery to give him a new pulmonary valve. The cath procedure irritated his heart so he actually was worse after the cath than he had been before. His heart needed time to heal and his surgeon needed time to fight with the FDA.

Let me explain... His surgeon felt the best course of action for John Nicholas was to use the Contegra conduit to replace not only his pulmonary valve, but also a large section of his pulmonary artery. The problem was that the Contegra conduit had not yet been given approval by the FDA for use outside of a clinical trial. It took nearly 2 months for the approval to come through. John Nicholas had his 3rd open-heart surgery at age 4½, in October of 2003. The surgery was a success and he spent only one week in the hospital. A few days later, he had to be re-admitted to the hospital for several days because he developed a post-op syndrome where the heart became inflamed as a result of the handling it received during the operation. He recovered well and even got to play t-ball the following spring.

His cardiologist decided late in the summer that his 7th heart cath was necessary to evaluate the conduit which had been placed during the 3rd surgery. In late August of 2004, this cath was performed. The cardiologist was very disappointed in what he found. He had to perform angioplasty and placed a stent in one of his pulmonary branches and the integrity of the conduit was in question due to outpouchings in its walls. John Nicholas was started on daily baby aspirin while the cardiologists and surgeon tried to decide what to do. They decided to wait and watch the situation and hope it improved. Amazingly, it did improve.

John Nicholas had his 8th heart cath in October of 2005 at which time his cardiologist performed angioplasty on his right pulmonary artery and also on the stent in his left pulmonary artery. His cardiologist continues to very closely monitor the conduit for changes so they can best determine when it will need to be replaced.

He has begun to experience terrible migraines in the past 8 months which have been extremely difficult for him to cope with because of the pain and nausea. It was during the work-up for the migraines that his neurologist discovered a cyst in the center of his brain. It is stable at this time so there are no plans to do anything about it other than to monitor it for changes in the size. Through everything, John Nicholas keeps a smile on his face that lights up a room. I think he knows somehow that life is fragile and precious, so he lives every day to the fullest. He is our little hero.