On January 30th, 2007 at 1:01 PM, our precious baby-boy, Daniel Matthew Mauriello was born. Our lives for the previous 40 days were filled with a range of emotions; awe, confusion, horror and wonder.
In my wife's 30th week, it was detected by ultrasound that our baby had a lower than normal heart rate (2:1 block with an average of 60 beats per minute). My wife was monitored very closely by Dr. John Fernandes, pediatric cardiologist, and other high-risk physicians at St. Barnabas Hospital in Livingston, New Jersey. Everything otherwise appeared normal and the baby grew inside his mother's womb until her 38th week when she was admitted. Labor was induced and amazingly, my wife pushed 6 times and little Daniel popped out in less than 15 minutes. That wonderful act probably saved his life.
The next day we learned about the arrhythmia, Torsade de Pointes, of which Daniel was having frequent episodes, some as long as 3 minutes. Dr. Fernandes immediately put him on beta-blockers after detecting prolonged QT intervals on Daniel's ECG. With the diagnosis of Long QT Syndrome, Daniel was moved to New York’s Presbyterian Children's Hospital at Columbia University.
The night that Daniel was transferred by ambulance to Children’s was the longest, emotionally draining night of my life. My wife was still at St. Barnabas and I made the hour-long drive to New York’s premier children's hospital alone with a lump in my throat. What was happening in the ambulance? What is Torsades and what does Long QT mean? At the Neonatal Intensive Care Unit, my baby slept under a heat lamp and the first of many blood draws was taken. I sat and watched the heart monitor for waves that looked like lightening bolts - this would be the first of many days just watching.
The next day, my wife arrived. We met Dr. Robert Pass, a pediatric electrophysiologist. He explained what LQTS was and that Daniel was prone to lethal arrhythmias called Torsade de Pointes, French for “twisting around a point”. We were told that he would need an implanted defibrillator (ICD) to survive these arrhythmias if they could not be controlled with medication.
For the next four weeks, Daniel was arrhythmia-free on a combination of heart medications including lidocaine, esmolol, and flecainide. Unfortunately, when the time came to switch from IV to oral doses of flecainide and inderal, he began experiencing episodes of Torsades again, the last one requiring 2 shocks from an external defibrillator. Despite his small size of just 8 pounds, it was decided that an ICD must be implanted and dual chamber pacing would be necessary, as his medications were not sufficiently controlling his arrhythmias. On Sunday, March 3rd, Drs. Henry Spotnitz and Jonathan Chen performed brilliant surgery to implant Daniel’s ICD in his abdomen. Dr. Pass programmed and tested the device.
Daniel is now weighing in at 9 lbs and 11 ounces (2.8 ounces of ICD) and is recovering well. The pacing seems to be the biggest help at this point and we look forward to feeding him, getting him off IV medications/nutrition and taking him home for the first time.
Preliminary genetic testing confirmed Dr. Pass’ original notion that Daniel’s Long QT was due to a malfunction in the sodium channels, indicative of Long QT Type 3. My wife and I have no history of heart problems or unexplained sudden deaths in our families. After weeks and weeks of ICU living, our little Daniel is still calm, cool and collected AND living….thanks to some really amazing scientists, nurses, surgeons, and of course, those little miracles that science cannot prove.