SADS Foundation :: Sudden Arrhythmia Death Syndromes Foundation

February 2, 2007 3:45 a.m.: I woke up with a frantic twitch and a numbing, stiff arm under the weight of my body. I had only dozed off for a few lousy minutes but it felt like hours. If only the last 24 hours were a dream, I thought. The couch in the waiting area was uncomfortable and I couldn’t help but think of all the other people who had slept here before which gave way to a wave of nausea. I had no blanket and it was freezing. The  “timer” lights would not shut off and were bright as the sun on a summer day. But this was the dead of winter. As I lay there, I prayed to God to let my baby boy live. I missed my wife. We were not used to sleeping apart – especially under conditions such as this. I needed her to be here with me. All I could think about was how much she needed me to say, “everything is going to be alright” – but I couldn’t say that because I didn’t know. Yesterday, she gave birth to the most perfect baby I’d ever seen – no wrinkles or that alien-like look that newborns have. No, Danny was perfect. Well, at least on the outside. We didn’t know it at the time, but Danny was about to have many earthquakes behind the walls of his heaving chest inside his tiny heart. Torsades DePointes… what the hell is that? We found out the next day when my wife saw his heart-rate jumping from one extreme to the next, his rhythm looked like lightning bolts on the overhead monitor. Danny, however, was calm, sleeping, as a newborn should, deceptively peaceful. The doctor told us he was experiencing something called Torsades de Pointes, a French word, he said, meaning “twisting of the point”, a phrase used to describe deadly ventricular tachycardia – a sign that our little bundle of joy may have something we had never heard of  - Long QT syndrome.

I should tell you that this was the end of one mystery and the beginning of another. You see, at 30 weeks, my wife’s OBGYN, for no apparent reason on this earth, advised her to get an ultrasound, “...just to see if everything is developing as it should” he said. Did he see or hear something? Or was this Divine intervention? As it turned out, the ultrasound revealed that our baby’s heart rate had dropped significantly to 50-60 beats per minute. My wife was immediately sent to a high-risk doctor, who wanted, at that moment, to take the baby out of his mother’s loving womb. But the cardiologist advised that the baby was moving normally and the heart’s structure was fine – it would be better to monitor my wife and baby for a few more weeks, as he did not think the baby was in danger – as long as his mother felt him kicking. So, for 8 weeks, my patient wife went to 3 different doctors 3 times a week, and continued working full time, all the while holding her breath and her stomach. The baby punched and kicked and hiccupped, and let her know he was not going down without a fight despite what the ultrasounds read. When he was finally born on January 30, 2007 (induced), Danny was immediately taken out of his mother’s reach to the NICU for monitoring. His first EKG looked normal, but the next day everything changed….

When the cardiologist was able to diagnose Danny’s Long QT, he advised that our baby would have to be moved to a hospital where a Dr called an Electro-physiologist would be able to treat Danny with the proper therapy. So, as the hours passed and we waited for the transport from the quiet suburban streets of NJ to the madness of NYC, our little helpless wonder was being pumped with beta- blockers to keep his heart rate down. But how were we going to keep our heart rates from racing? Our two hearts were sinking and breaking and somehow my wife and I had to split ourselves apart as she was ordered back to bed for one more night before being discharged from the maternity ward. She had hardly any face time with her son -she wasn’t even allowed to hold him. I gathered all the strength I could muster to drive myself to the hospital at 11pm, still in my days work clothes to meet up with my little boy, who at any given moment could go into cardiac arrest at the tender age of 1 day old. I can’t remember how I got to the hospital, where I parked, how I even found my child in that giant building on that cold windy street at 168^th and Broadway. But I did find him and when I did he found me with his senses, as if he knew I was there and that I was his daddy and that he was loved so much already. My heart ached for my wife that she was away from this moment when I finally got to really see this amazing creature we created together - the boy with the earthquakes in his chest. He was the most mesmerizing site I had ever seen. If I was a hurricane then he was the calm eye of the storm. He didn’t cry under those heat lamps, naked and pale, wires everywhere. He gazed blindly around the room with eyes like a searchlight looking for something, or, someone, probably the one who carried him all those months and loved him more than life itself – even though her time with him outside the womb was suddenly cut short. But what if he died right here, right now, and his mommy was miles and miles away? These were the thoughts that breathed in and out of my lungs, as if I was the one on the table who could die. And then it hit me – this baby was a part of me like no other possession in my life and his heart was made from me – what if I caused this? There’s no rationale for selfish thinking in times of grief. It is what it is.

The next 69 days were spent at NY Presbyterian Children’s Hospital and our child was under the care of a brilliant young physician, who never gave up on our son, and does not to this day. We learned much about ourselves in those hours spent nestling our little boy in between entangled wires and central lines (his groin, his arm, his head). He could not eat or taste the sweet milk welling up in his mother’s breasts – which she pumped every three hours to store away in hopes of one day being able to feed her son. This is love as unconditional as it gets. But we got a sense from Danny, even in his worst state (2 weeks in the hospital he contracted NEC, a GI infection which prolonged his food intake, his cardiac care and coming home) that he was a child who always gave us hope even when science made no sense. His eyes, so soft and sweet, determined and alert – it’s unfair to have to live your first days like this. The hospital, especially at night (Long QT3’s effect on the heart usually comes in the middle of a deep, late night slumber) was chaotic, the beeping monitors and late night visits from the “vampires.” But he endured it like a gladiator. It was an hourly occurrence that someone was rushing into that room for some emergency situation and if you left to go to the bathroom, it was 9 out 10 times, the alarms going off were for Danny. Even when having his extremities stuck with needles, or early morning x-rays, our sweet Danny, [namesake of the Biblical Daniel], remained strong and silent, inside his own lion’s den.

We learned what Long QT is, but what was so vexing to us was the mystery of why –why our son? And how? DNA testing revealed that his heart had a malfunction in it’s sodium channels causing the ventricle to pause between pumps and that wait, or wave elongation, throws the normal sinus rhythm into a frenetic pattern akin to a volcanic rupture. The blood stops flowing to the necessary places and sudden death is probable if not shocked by a defibrillator.  So, what to do with a baby less than 8 lbs? We were advised that this baby could not leave the hospital without an implanted defibrillator and proper oral medication (as he was surviving on IV for weeks).  Danny’s genetic mutation did not come from me or my wife, it is a de novo, or new, mutation born inside our child – and no other mutation or genetic code like his could be found anywhere on this earth. This is hard to swallow. What does this mean? It simply means, Danny’s care comes without much reference or any other cases to compare to – his doctor only has hypotheses to derive his treatment for our son in an area of science still relatively new.  Unfortunately, an implant of this nature had never been done before on a child this size – the surgeon would like to have waited until Danny is at least 12 lbs. Time was running out. And he was not gaining weight as quickly as needed. By the end of the 3rd week, the Torsades were coming on like machine-gun fire and it was clear that Danny needed that defibrillator sooner than later. Amazingly, the surgery was going to happen.  My wife and I surrendered our selves to God, offering up to Him our son as we lived in the moment of “whatever is to be, will be.” Life sometimes leads you to places you are not prepared to go, places of weakness and desperation, where all your strength and power and even faith cannot possibly control what the outcome will be. And so, we waited in this place while they cut open our boy’s chest cavity to do the unthinkable- implant a defibrillator the size of a cell-phone and connect the lead wires to the surface of the heart of an 8 lb baby. If this surgery were to be successful, it would be a medical first. We never imagined our marriage of 3 years bringing us to this moment – where life and death converged on a high wire with no safety net below. If there is a fall – there is death. Will Danny live? And how will we live without Danny? These are not everyday questions. But this was our reality. Hope never died within us despite the weight of these questions of life and death.  It didn’t really matter what it meant, or even why this was happening. We certainly were not the only parents in a situation like this – but this was our life and our son in there – and no matter what it means to someone else, how we accept the unknown truly measures the kind of human beings we are. Is it possible that God gave us Danny because of who we are? If a baby were to be born like Danny, what kind of parents would he need? Was this our calling in life? Was God entrusting us with this little boy because He felt we could handle it? Seems like a tall order. But as we heard the surreal sound of the doctor’s voice saying “Danny is alive” and that the surgery was without complications – we felt that calling so loud and so clear. We would love this boy with all the strength in our own hearts that God has granted us. Love, as we have come to reflect upon in our own marriage, is the only way out of situations like this. It keeps us afloat when everything is sinking.   

So where are we now? Daniel Matthew is exactly 28 months old and is the older brother of Robert James (“Robby”), who is 9 weeks old. For all the pain and physical suffering Danny has endured in his short life, one would think he would be a difficult 2-year old.  But he is not. He wakes up smiling and goes to bed with his thumb in his mouth (most nights without a fight). In between he keeps us entertained as he has developed a personality full of love and life and laughter and beijinhos (Portuguese for little kisses). He looks like a little boy now, with his cropped dirty blonde locks, long torso and legs, and that face – where did he get those amazing ocean colored green eyes? He is beautiful in every sense of the word. After his third surgery before he was 5 months old (his first, the ICD implant; second, a colon resection from scarring caused by the NEC infection; third, sympathectomy at the Mayo Clinic, Rochester, MN), we began to see what life with Danny was going to be – a roller coaster ride, or perhaps that high wire act, where we walk that thin line between life and possible sudden death imminently looming over our heads and deep inside our consciousness. There were many late night screams from the crib - and mad dashes to be there when he was shocked. You see, at home there are no monitors - just the sound of Danny being awakened by his defibrillator or his heart combusting – we’ll never really know which one makes him scream like that. And we hold him in the aftermath (as he got older, he actually would faint prior to a shock if already awake), his face sinking into our arms, as we fight back the tears until falls back into his angelic slumber. We eventually learned to fall asleep, too, and to not allow our emotions to get the best of us. It is a massive calling to be a parent and to be a parent of a child with Long QT is a test of character and will. We are like soldiers. Everyday is unpredictable, sudden attacks one minute, sniper fire the next -and then, moments of peace and joy. It is these moments we live for, but we are prepared at any given moment for disruption – it’s just the way it is.  Danny is tolerating his meds well (anti-arrhythmic and beta-blocker every 8 hours), his pacing set at 110 beats per minute (his ICD doubles as a pace maker), and his defibrillator hasn’t shocked him in almost 3 months – this is a very good thing. The battery life should give him another year, if we are lucky, then it will have to be surgically replaced with a new ICD, hopefully a smaller one. But he’s grown into his current one nicely. It sits in his abdomen and is clearly visible with his shirt off – to him another organ. To us, the tiny box that keeps our baby boy alive. Thank God for medical science and doctors like Robert Pass, who are breaking new ground in the field of Electrophysiology and more importantly, saving babies.

Danny’s story continues…we hope for a very long time…

                                                            ----- Steevan Mars 5/4/2009

Steevan Mars (real name Stephen Mauriello) is a singer/songwriter/guitarist, based in Morristown, NJ). You can find him on the web at www.steevanmars.com , CDBaby, iTunes, Facebook, Myspace.