Monday, February 2, 2009

Medical Monday: Cardiomyopathy (Updated)

Doctor Connor is in the house! Dr. Connor declares my glasses and stethoscope to be the height of hilarity. Also he demands chocolate pudding.

Because Connor has so many conditions and I refer to them all the time without proper explanations, I figured I'd declare Mondays to be Medical Mondays from now on. I'll blog each Monday about a different condition Connor has and then post a link to it on the side bar as I go. This way people can get a better idea of what Connor deals with on a daily basis and his family and friends can understand what I'm saying when I call and spout ridiculously long medical terms.

DISCLAIMER: I would like to emphasize for those people who stumble upon this blog that I'm not a doctor, and I have no medical experience other than the approximately 900 doctor's appointments I've been to in the past two years. I just know about these things in relation to how they apply to Connor and not anyone else's child, so if you want accurate info on this sort of thing, please please please ask your doctor or go to the medical library instead of looking it up on the Internet. The library is your friend.
UPDATE: As undeniable proof that you need to get your info from the doctor and not me, a very nice person from the HCMA let me know today that you can only have LVNC or HCM-- not both as Connor's diagnosis sheet from his coordinating care doctor reads. I went back through my files and then called Connor's cardiologist for a double-check. Evidently somewhere along the line one of Connor's coordinating care doctors read LVNC with symptoms of HCM in a report from one of Connor's cardiologists and interpreted it as LVNC and HCM, which then found its way into Connor's permanant records and will now be almost impossible to change. Thanks, HCMA. Post corrected.

So that's that. Today's topic is cardiomyopathy.

I was checking out the news today online and came across an article about the Williams family. Louise and her twin daughters, Katie and Lauren all have dilated cardiomyopathy: a rare heart condition in which the heart is enlarged and weakened. I'd encourage you to stop by their website and leave some words of encouragement.

While Connor does not have dilated cardiomyopathy, he does have a very similar condition-- left ventricular noncompaction. I'm no expert on this sort of thing, but here is how it's been explained to me by several cardiologists:

The condition called left ventricular noncompaction (LVNC) is very rare and little understood. The muscle of the heart, known as myocardium, starts out during fetal development as a very spongy and spread-out material. As the heart begins to develop further, this material draws closer and closer together, becoming the dense, extremely strong muscle needed to be such an efficient pumping mechanism. With LVNC, the myocardium stays spongy, especially in the left ventricle, and so the heart is weakened and can't pump as efficiently. The difference in the heart muscle causes very deep nooks and crannies in the wall of the left ventricle, known as trabeculations. Trabeculations cause issues because blood can pool and form clots in these nooks and crannies, greatly increasing the risk of stroke. Connor takes aspirin every day to reduce the risk of this happening, and also takes another medication to help his heart pump more efficiently.

Because of how hard it has to work, Connor's left heart wall has become thicker over time. This is a condition that can cause serious problems if the thickness of the walls cuts off the blood flow in the heart. However, in Connor's case, the atrial septum-- the wall that divides the left and right ventricles of the heart-- is not becoming thicker and so he has no problems with blood flow. Other than not being able to play any contact sports or be in the military (I'm just heartbroken over that, I tell you) his heart issues shouldn't otherwise limit what he can do as long as they're well-managed.

The nature of Connor's heart defects are one reason why he is considered to have a shortened life span-- we've been told somewhere between five to fifteen years. However, most people who have been discovered with LVNC are diagnosed because they are going into heart failure, and so understandably the mortality rate is pretty high. It's likely that there are many, many people walking around with LVNC who have no symptoms and live a normal lifespan-- they just aren't ever diagnosed with LVNC and so the statistics don't reflect them. It's also likely that if Connor didn't have so many other things going on, his heart defects would never have been found, as he shows no outward symptoms at all. Besides, the doctors don't exactly have the best track record on the whole Connor-kicking-the-bucket thing. They've already declared him doomed on four previous occasions, and he's still his happy little very alive self.

Like Louise, Katie, and Lauren, Connor's heart condition cannot be fixed by surgery. If he were to have issues with his heart, he would have to have a heart transplant. He is at risk for sudden death, heart attack, congestive heart failure, and stroke. Luckily, Connor's heart thus far has caused him no problems and we hope this will be the case for a long, long time to come.

Here's some good links involving LVNC:



Ellen said...

Jess, thank you for this, you explained it all so clearly. We do become quite the medical experts with our kids, don't we? I remember that after Max was born, I was giving a physical therapist his background info and she asked if I was a doctor, because I'd learned all the vocabulary.

HCMA said...

I am with the HCMA (we are linked on your article).
I just wanted to offer a little more info and an offer to help if you need it.
HCM - hypertrophic cardiomyopathy and LVNC - Left Ventricular Non Compaction are two seperate conditions and you either have one or the other, not both - although often time one is diagnosed, then changed to the other after more evaluation.
Many children with syndromes have the appearance of HCM or LVNC but the actual cause of this is a different genetic muation then causes other forms of HCM.
For more information about HCM please visit our website - and BEST WISHES to Connor and his amazing mom!

Connor's Mom said...

Thanks Ellen! I've received that question quite a bit as well. I obviously still have quite a bit to learn, as the HCMA demonstrates!

Connor's sheet from the doctor's office says LVNC and HCM. I'm assuming that our coordinating care doctor read LVNC with additional symptoms similar to HCM in a report from our cardiologist and condensed it to both. I've often found that the stuff that comes from our specialists tends to mutate as it passes from doctor's office to doctor's office-- kind of like a game of telephone. I'll have to call and get that cleared up. Thanks!

Brate said...

Exercise has been really a very important factor for the people of today’s generation. And especially exercise really helps heart patient more than anything. I have got an enlarged heart because of inability of pumping. I was also having a defective valve, which led me to be a sinus tachycardia patient. I got to know at my medical checkup at my campus. And being very young to face all this, I was really frightened regarding all these health issues. I need someone to monitor my health and keep an eye on my health as well as daily health issues. Getting an internist hired was just not the solution to the problem. I got to know about some kind of wellness program from elite health ( Medical Service Provider Company. I got enrolled in it, as they were providing me 24/7 access to the doctors. Especially, I got one unexpected and quite a surprising opinion from their health executive who used to monitor my health and guide me the appropriate dietary solutions. He told me to have a regular exercise daily. I thought he is really mad, or planning to kill me. Ha Ha. .. But my regular exercise! Not so heavy, the results came out to be positive. I was really feeling better and healthier as compared to previous conditions. So, indirectly, exercise has really helped me suppress my health issues, especially the problems we generally face while having heart failure.

maria said...

jess,thank you for all that you have shared.I also have lvnc and indeed was told that i had LVH,but 11 years later was told that i have LVNC by emory in atlanta ga.please keep your faith because i am now 41 and i do have trouble daily,but by gods grace i get through each day! not yet ready for transplant,but headed that way!! maria in south carolina! i know it has been along time since you shared,but i hope the best for conner and will pray daily for him!!

Robert Jaron said...

I wish Connor a long life. He may be lucky like me. I am a husband,father, US Army Veteran, and a Grandfather of 2 boys, 5 & 2. I am 55 years old. Never new I had LVNC my whole life. Was diagnosed in Dec. of 2011. I had a defibrillator implanted due to the condition of sudden cardiac death. I am told I am very lucky to have lived a full long life. I wish Conner the same! God Bless. R.Jaron

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