73 percent

Long QT Syndrome and Christina

Seventy three percent. This is the number of undiagnosed children with "long QT" syndrome who die the first time their condition manifests itself. Without any warning, a child may develop a ventricular cardiac arrhythmia, which quickly degrades so that the heart goes into Ventricular fibrillation and eventually full cardiac arrest. If medical help is not obtained very soon, brain damage occurs very quickly after the onset of ventricular fibrillation, due to the lack of oxygen to the brain, because the heart is not pumping blood. A significant number of children who survive their first episode have brain damage or renal failure as a result of the anoxia they have endured.

I knew nothing of "long qt" syndrome, which my daughter was diagnosed with, or percentages for survival from the first "attack", and could barely read an EKG before Wednesday, last week. I know a lot now, but the other lessons I learned are much more valuable.

We who are Christians and live as such, putting our children in the hands of God. He giveth, and He taketh away, and He is always merciful. We believe these simple facts, and conform our lives to them. I have always done so, and I have rarely felt fear. Wednesday and Thursday I knew fear, and I also knew peacefulness and rest. The rest was in God's will, and certainly not for my body, as I am still exhausted from the previous week's vigils.

The following is a story of God's mercy, and how He cares even for sinners.

On Wednesday afternoon, my daughter Christina, 11, was dropped off for the third day of a five day basketball camp. On the way into the building, while my wife was STILL IN THE PARKING LOT, she developed a cardiac arrhythmia, with preventricular contractions (PVC's). This arrhythmia was the genesis of an even deadlier rhythm, called "Torsade Depointe" before or during which she lost consciousness and fell face down on a concrete sidewalk, about ten feet away from an outside door.

She was discovered, outside, by the maintenance man perhaps 5 to 10 minutes later. By God's marvelous allowance, he was headed out for a late lunch, which he needed badly, as he is hypoglycemic. He was in a great rush, since he had missed the usual time for his meal, and therefore, exited from the side door of the facility, although he usually goes out the back, and tells the secretary he is going out. When he found her, he had the presence of mind to make sure 911 was called, then he rushed back to her side. Christina's breathing was shallow, and she was either in ventricular fibrillation or close to it. When the paramedics arrived, she was definitely in ventricular fibrillation, had stopped breathing, and was blue. She was worked on for ten minutes in the ambulance, and taken to the nearby hospital, only known as Jane Doe, since she had no identification, as she was in gym clothes.

My wife found out she was in the emergency room when she went back to the gym before she would normally go, because she had a bad feeling. Christina had already been in emergency treatment for about an hour. I was called, and rushed to the hospital, not knowing if she was alive or dead. Many things go through your mind at times like these. I remember noticing a mile marker, 34. It seemed like ten minutes passed, and I saw mile marker 36. It made me angry. I decided right then and there to get a numeric pager, so that I would never be out of touch. I lamented that I did not have an epatrachial or cuffs, and did not have the canon that is said at the departing of the soul from the body. Mostly, I prayed the Jesus Prayer: "Lord Jesus Christ, Son of God, have mercy on Christina". I prayed aloud, in a hoarse whisper, which is atypical for me. Interspersed with my prayer were waves of guilt. I wondered if my daughter would die not knowing how much I loved her. She is a sensitive girl, and I sometimes overwhelm her. I often am too busy, even though I am not really that busy. I did not make any promises with God (in "exchange" for her life), but I vowed to stop my sinful way of life, and to live with my daughter in such a way that I would not have any regrets, if God gave us more time.

Before I entered the emergency room, I got a prayer book out of the glove compartment. When I saw her I could tell that she was not dead, as there was a technician compressing an ambu bag, and the activity around her was tense, but under control. I saw that her pupils were "fixed and dilated" and totally unresponsive to light. My wife was crying, I was dry eyed. I could not feel my feet. I was right at her side, looking at her sweet face, and the multiple contusions on her, the result of her fall and probable seizure, brought on by the apoxia. I asked about her condition, and received unsatisfying answers. They did not know how long she was unconscious. They were testing her for drugs of abuse, and were trying to evaluate if she had a seizure or cardiac problem. I did not waste any effort telling them that there were not drugs in her - I knew they would check anyway. Somebody asked loudly if this kid was still "Jane Doe" or not. The resident gently told her name. I noticed in myself an amazing rage, and I had to will myself not to snap at the man. The language would not have been pleasant. I thought to myself : "you got mad because any emotion is preferable to this terrible fear".

I was praying the Jesus prayer, sometimes without audible words, sometimes in a whisper, sometimes louder. I think I said "Most Holy Theotokos Save us" at odd intervals. A little bit later, I opened the prayer book, and started the supplicatory canon to the Theotokos. I know the translation from Holy Transfiguration Monastery almost by heart, and sing all the Byzantine melodies. I only had the "Old" Jordanville prayer book, and I could not sing. I tried to sing the first ode, but got too mixed up with the different translation, and gave up. I could not remember a word of the translation I knew so well. Singing this canon has always been a great consolation to me, and I felt disappointed. Then I was disappointed that I was disappointed. I completed the canon, with hope in God.

They had decided to transport Christina by helicopter to Children's Medical Center in Dallas. It is a 54 minute drive, even when one goes 75 or 80 miles an hour on the toll road. I have no idea why I timed it. My children were with me. My wife (erroneously) had planned to fly in the chopper with Christina. She was later taken by a friend, as no one would let her drive. On the way, Jenny sang the Akathist to the Mother of God, then started the canon of repentance. I told her to sing the one to Sweetest Jesus instead. I remember praying "Thy will be done", then admitting I did not really "mean it". I wanted to mean it, no matter what, but I wanted my daughter to live.

Christina was in the pediatric ICU. She was comatose, but her pupils responded to light. She was on a ventilator, which was breathing for her eighty percent of the time (she was able to breathe a little bit). She was in critical condition, and was receiving intravenous lidocaine, which kept her heart in the proper rhythm. We began the longest portion of my life. No one knew if Christina was brain damaged or not. She had received some potent medicines, and we did not know how long she had been without oxygen. By 6:30 PM, she was completely weaned off the ventilator, but was still comatose. I stayed all night in her room, waiting for her to awaken. She looked "different" than she looks when she is just asleep. My wife and I prayed. Christina woke up several times through that longest of nights. Her eyes were like those of a scared animal, and she would not squeeze our hand, or respond to any commands. I wanted her to talk so badly! In the morning, she awakened somewhat more, and I asked he some question. She croaked out an almost inaudible, almost unintelligible answer. It was three words. I don't remember what they were.

Thursday was a rough day. The specter of serious brain damage loomed large. In the afternoon, I left for church, as I had promised to serve a moleiban to St. John for several who are sick. During that time, she sat up in bed, talked a little bit, but was incoherent, confused and very combative. Concurrently, I visited Bonnie, a good friend of my good friend, Michael. She is Orthodox, and attends St. Seraphim's Cathedral in Dallas. She is probably dying of type C hepatitis. I brought myrrh from an icon of St. John that is currently weeping, and anointed her. Upon arrival back at the hospital, I encountered my wife "on the ragged edge". She could hardly stand, or think. She told me that Christina was talking "like a three year old". This terrified me, but when I went to her, she was not as bad as I feared. Michael, more an angel in the flesh than a man to me, and Marina's mother (she had immediately flown in from Florida when she heard the news) and I went to a nearby restaurant to eat. We had to force her to eat. I was ravenous, and thought it very strange.

Christina continued to improve through Thursday and Friday, with intermittent periods of memory lapse, confusion, and what the medical people call "inappropriate" behavior. She asked the same questions over and over: "What day is it?", "Where am I", etc. Saturday, she was "almost herself", and had lost most of the flat affect that people often have when they come out of a coma. On Saturday, I served the vigil as usual, preceded with another moleiban to St. John. On Sunday, after the liturgy, I went to the Hospital in full vestments with the Holy chalice, which still contained a portion of the Holy mysteries, and communed Christina and my wife. I was pleased to hear Christina say the Trisagion prayers.

On Monday, Christina went home. She cannot play competitive sports, and this is a big disappointment to her. We are trying to continue to trust God, who saved her from certain death. Even so, the first night I checked her in her bed three times.

I have not yet assimilated all that has happened. I am so thankful to God that he preserved my "Teen la dean".

Glory be to God for all things.
July 25, 1995

Christina returned yesterday from the hospital, having had a successful procedure completed which inserted a pacemaker/defribrillator near her heart. After her first attack, she was placed on a beta blocker medicine (Atenelol), but it gradually lost some of it's effectiveness, and she almost had another cardiac incident at the end of December. The doctors told us that in a significant minority of Long QT patients, the medicine must be augmented by the pacemaker/defribillator.

The device is about the size of a pager, and is inserted above the heart. An electrical lead is then passed into the heart, and after a little bit of trial and error, the doctors affixed this lead directly to the heart by screwing it in ! The surgery is reasonably quick, and non-invasive. Christina just has a 2.5 inch scar in her upper left chest. the device will pace her heart if it drops below a certain rate, and in the unlikely but certainly fatal if uncorrected even of a cardiac arrythmia that leads to ventricular fibrillation, she will be defibrillated by a large shock from the device. This "resets" the heart, so it can recover, and beat normally.

Bonnie (also named Christina), whom I mentioned in the first installment, above, died of hepatitis C, and her passing was heroic and edifying. I would appreciate it is all Orthodox who read this would commemorate Christina in their dyptichs for the reposed, and also my "Teen La Dean" in the dyptichs for the living.

I thought about my "73 percent" article when Christina came home yesterday, and decided to read it again. It came from my heart at the time, and is more true now than it was then.

Priest Seraphim Holland, the grateful father of Christina

An Overview of the Inherited Long QT Syndrome

THE LONG QT SYNDROME
From the Sudden Arrhythmia Death Syndromes Foundation

Cardiac Arrhythmias Research and Education Foundation, Inc.

The European Long QT Syndrome Information Center