February is heart month. I never knew this before I had my son David Joseph. But now, February is a special month... a special time to remember my son has a very special heart.
DJ was born with Left Isomerism with Polysplenia. It is a strange heterotaxy syndrome that impacts where internal organs are placed. Simply put, DJ's body tried to create two left sides, instead of a right and a left side.
DJ is very lucky, while he has many anomalies regarding his spleen, intestines, lungs, stomach, and heart, most are congenitively corrected. Which means that they are different in size, location and function, but they are working well enough not to be a problem.
The major causes of mortality and morbidity in the heterotaxy syndromes are undoubtedly the cardiac malformations that typically occur in these conditions. In one large retrospective review from Canada, the 1-year mortality rate was 32% and the 15-year mortality rate was 49%.
We have been very lucky. While DJ's heart is very different, and should technically not work as well as it does, he has never had a heart surgery. (knock on wood) In the future, he may need a pacemaker - but for now, he is doing great.
Here is an image of a "ordinary" heart beside an picture of DJ's heart that the heart specialist gave us when he was 21 days old. I have stitched them together so that you can compare the differences.
Click image to enlarge
Here is the list of DJ's heart anomalies:Dextrocardia - this means that his heart is in the right-side of his chest.
Transposition of the Great Arteries - DJ has cognitively corrected TGA, this means that while the main arteries have switched, they are functioning on their own and no medical intervention is required.
Pulmonary Stenosis - this means that he has a heart murmur where a section of the pulmonary valve is blocked.
Interruption of the Inferior Vena Cava with Azygos Continuation - with DJ, the large vein that carries de-oxygenated blood from the lower half of the body into the heart is completely blocked. Technically, his heart should not work, and he should have passed in utero, but the azygos vein took over the function of the IVC. Incredible!
We like to tell DJ that his heart is perfectly imperfect! He is truly a very special medical miracle. It is rare to have this syndrome, but it is more rare to be so healthy, strong and cognitively corrected.
In the past few months I have written a little bit on DJ's medical condition, on how his uniqueness as impacted our lives and how I experienced my first Mommy Moment in the ER when he was just 9 days old.
I am not sure I will ever tire of writing about his medical condition. My fascination is partly due to the fact that I need a means to understand all the medical terms. Each entry allows me a reason to do more research. My fascination also allows me to raise awareness of congenital heart defects. My fascination also allows me to remind myself that while DJ is healthy and strong, I need to be prepared for anything. And finally, my fascination allows me to expel my energy in a positive way, instead of sadly dwelling on the many sad stories I hear about on a regular basis. I have become on-line friends with many other families who have children impacted by heterotaxy syndromes and very rarely do their stories have the hope, blessing and wonderful prognosis as DJ. Just last week a young girl, just 2 weeks older than DJ, died waiting for a heart and lung transplant due to having right isomerism. My heart goes out to every family faced with any congenital heart defect.
February is heart month. I hope with all my heart, that some of you reading this, or even just one other person reading this blog, will decide to donate any amount of money to the Heart Institute of Canada or to any foundation or charity that deals with heart defects or heart disease.
To my dear DJ, i HEART u with all my heart.