Friday, 13 July 2012

Eight Weeks, One Day: Causes of Death

I might post an entry to my blog on a Monday or a Tuesday, and Wednesdays are nice. Fridays are Ok and either day of the weekend is fine. But never on a Thursday. I won't do Thursdays because that is the day the wounds open up and Roni needs my support completely. Posting on a Thursday would feel like walking on Her ashes.

I know my title suggests that I might know something today that I did not know before, but that would be lying. We still have no idea what killed Calista and we probably never will. Idle speculation and suspicions are just the results of a grief stricken fool grasping at any straws he can to find meaning in his barren life.  On the other hand, I have had some good suggestions from wonderful people.

I have bandied around the idea that Calista was taken by one of these rare cardiac arrhythmias that we see in young people periodically. Typically the story runs something like this "Young star athlete collapses suddenly while jogging back down the soccer field".  Usually the story has a special section explaining where the funeral/ memorial is to be held and where to send the donations or flowers. Once in a rare while there is a happy ending because a good EMT or a great Doctor recognised the symptoms of acute exercise induced cardiac arrhythmia and pulled out the defibrillator. These arrhythmias can occur at any time, but typically occur when the young person is active or under the influence of adrenalin. The most common arrhythmia (or the one I found the most references on) was something called Long QT syndrome.

I am not sure if I had any awareness of "Long QT" syndrome until one of my colleagues at a private veterinary web forum suggested that Calista might have died from this uncommon problem, but I vaguely knew about athletes dying young suddenly. Here is my round-about, highly inaccurate description of the heart cycle and how Long QT might kill your child.

If you can think of the heart as just a big electrical pump that contracts as a wave of electricity moves through the pump sequentially, this will work well for you. Picture a crowd of fans at a Saskatchewan Roughrider football game (I show my bias) and they all decide to cheer their team by doing "the wave". The fans all start by standing up (relaxing the heart muscle) and then the fans at the North end of the stadium sit down quickly first, followed row by row until the wave reaches the South end of the stadium and they are all seated (heart muscle contraction). Then everybody stands up again almost at the same time (heart muscle relaxation). The rate of the wave travelling down the stadium (always in the same direction) depends on how long people remains standing between "waves". When the wave is going slow (slow heart rate) the fans stay standing for longer, when the wave is going very fast, they are basically just jumping up and down. The rate of standing up and sitting down always stays about the same, it's the amount of time the fans stay "relaxed" that determines the rate of the successive "waves" (heart rate). 

Now here is some important points (not related to Calista, but interesting none the less). The only time the fans get to breath, eat and drink beer are when they are standing, relaxed. If they are jumping up and down constantly, they go hungry and get out of breath. Doing the wave rapidly for long periods of time just tires out the fans and, of course, beer sales plummet. The situation is bad for everyone all the way around.  Now, take this analogy a little farther. Maybe the fans start drinking early and get quite drunk by half-time (middle aged man?) and now the wave is getting pretty sloppy as fans start falling over drunk. This would be somewhat comparable to cardiomyopathy. On the other hand, maybe the game started with a few renegade Winnipeg Blue-Bomber fans mixed in with the hometown crowd; these guys are intent on screwing up the "wave" cheer, but they are just biding their time, waiting for a likely opportunity to strike. Either  way, they are planning on destroying the organised wave pattern by hopping around randomly and disrupting the orderly cheer. Maybe their best time to strike is when things are really chaotic and exciting in the stands, like at the one yard line with only one down left. This is comparable to Long QT syndrome: a congenital abnormality you harbour for your whole life, waiting for an opportune time to strike and ruin the organised wave of your heart's contractions.

Doctors record the electrical wave through the heart with an electrocardiogram (ECG or EKG depending on whether you insist on traditional German being used). The ECG is broken into the "p" wave, the "QRS" wave, and the "T" wave, which basically translate to "trigger", "contraction" and "relaxation" (not exactly by any means but who wants to go back to school now?). The spaces between these waves are "the intervals". The QT interval is the space between the beginning of the ventricular (bottom of the heart) contraction and the relaxation of the whole heart.  Now remember back when I compared this whole thing to a crowd sitting down and standing up: the time it took to do both was always constant no matter how fast the wave was cheering. The innate intervals of the heart cycle are usually constant, its the time the heart remains relaxed that determines the heart rate. If the innate intervals alter or change sporadically, then the heart gets out of proper synchronisation and pumps that aren't synchronised don't work.

Obviously this is a pretty simplistic view of cardiology, but I'm not exactly Einstein, so it's the best you are going to get. 

Long QT syndrome is characterised by,,you guessed it...a long QT interval.  The syndrome is usually congenital, passed from parent to child genetically, but it is not truly just one disease. There are many variations on the Long QT syndrome, each with it's own inheritance.  Almost all the syndromes are associated with sudden death in people under thirty, and many or most of those deaths occur during athletic activity. It seems that excitement with the accompanying natural secretion of adrenalin increases the likelyhood of the prolonged QT interval inducing chaos in the natural synchronisation of the heart and inducing a potentially fatal bout of ventricular tachycardia. The version of "V-Tach" they see is my favourite (because it sounds so exotic) "Torsade de Points", or, in English, "Fence of Points". An  ECG that looks like a fence of points is a clinician's nightmare; the patient is about to die without immediate intervention.  You might get that intervention on a soccer pitch or at a hockey rink with thousands of spectators, but it's just not happening at a private house in the early morning hours surrounded by a few friends with no medical training.

Diagnosing Long-QT syndrome is not easy in the least.  Prolonged QT intervals are not always apparent in a patient with the syndrome and not all patients that demonstrate prolonged QT intervals have the syndrome. Furthermore, while the disease is known to be inheritable, a parent does not always show the DNA markers for any one of the known Long QT abnormalities.  Many of the people will have some symptoms of heart disease such as sudden weakness or fainting spells, but just as many people collapse or die suddenly with no warning whatsoever.  My research shows that the specialist will typically take all the symptoms and the family history into account, assign a point value to each factor in the work-up and diagnose on the "score" produced.  Its basically a statistical crap-shoot with the penalty for a mistake being death.  Cardiac Russian Roulette as it were.

So, why would I write about this here today? Well, because it fits quite well and it makes my alternative of "something really bad happened" less likely.  My daughter was an athlete and she was noted to have collapsed suddenly for no apparent cause, followed rapidly by death.  Certainly she was not active when she collapsed, but that is not a consistent finding with Long QT syndrome (common yes, but not consistent). Many of those affected with the syndrome just die without warning while not doing anything much at all.  My daughter also did have a couple of unexplained incidents of fainting when she was growing up. We investigated those fainting spells, but I am not sure the doctor ever actually checked her ECG. Calista was amazingly healthy growing up and she never actually saw a doctor on a regular basis. Most of the time we were seeing the doctor for  minor stuff and once for an allergic reaction to laundry detergent. I am not sure that any of the doctors we ever saw would have known how to turn an ECG machine on much less how to diagnose Long QT. There is one more thing though: my own history.

I have always had an unnaturally low heart rate and very low blood pressure. I always assumed those two abnormalities to be a good thing, at least until last December.  In early December, about two days after I had some minor surgery, I collapsed suddenly in my bathroom while putting some eye drops in. It was first thing in the morning and all I remember is things suddenly getting real black and then waking up when an ambulance driver rolled me over onto my fat ass. I was covered in a cold sweat and pretty much unresponsive on the floor of my bathroom. The ambulance crew had a heck of a time finding a pulse or managing to find a vein on me and I am pretty sure my systolic blood pressure never got above 80 for three or four hours. At that time my ECG was normal, but nobody has any idea what it looked like when I collapsed and I'm not sure that an emergency room clinician would necessarily recognise Long QT syndrome (or even look for it). To this day it remains a bit of a mystery what happened, but you can bet that everyone is running around trying to solve that mystery now that my Calista, my only child, decided to take a permanent nap 8 weeks ago.

Its kind of like closing the barn door after the horse has been pulled down and eaten by wolves; too little too late.  At this point I'm not sure either Roni or I really care if my petty little health problem is diagnosed now that a long life just means a longer sentence in Hell. On the other hand, that brings up another issue altogether.

Perhaps if I had not been a typical man and decided to avoid doctors like the plague they would have discovered a possible familial disease. Maybe if I had not been such a jackass about my own health my daughter would have been screened for Long QT syndrome and diagnosed in time to save her life. Maybe my silly male pride killed my only child. Now there is something to think about for the rest of my life.

Sometimes it really is important to take care of yourself. If not for the obvious, do it for your kids.  I keep on reminding myself that I have numerous nieces and nephews, the majority of which are under thirty. I am pretty sure the screening process is relatively easy (but, yes, not very accurate). For everyone needing more information on Long QT screening, check out this website:

The wee bit of research I did on Long QT syndrome made me pretty sure that it is what took my girl. I will now play "What If" for the rest of my life. Wonderful.

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