Home of the Four Trans

Justin’s first birthday bash plans are underway. The invitation promises of a party “fit for a king.” A lion and crown adorn the tangerine and green card. I’m excited. I love parties, love my Justin, still can’t believe he’s turning one. There’s still 3 more weeks till the big day that I am almost afraid of believing it. Anything can–wait, I don’t want to say it.He has this cold that went away and came back. I’m assuming that he got it from Nathan. But, I’m still concerned. Called the cardiologist yesterday because his eyes had been getting progressively puffier for 3 days. But, then, it went away. I guess Justin didn’t want to take that extra dose of Lasix the doc ordered for him.

 Today, I read through a blog about a mother trying to find answers to her 5-year old daughter’s heterotaxy condition. This child was not diagnosed until she was 5 years old. After pushing for 3 years, this mother finally got a diagnosis for her daughter’s, Chloe, condition. Fortunately for Chloe, she has a good prognosis and does not show any signs of congenital heart disease that is often associated with heterotaxy. I read through this blog and left inspired.

Justin has heterotaxy. This was diagnosed by the wonderful Dr. Michael Rebolledo while inutero. The early diagnosis saved us a lot of heartache and emotional turmoil because we were allowed some time to grieve this loss of a physically perfect child before he came into this world. When he came into this world, we were prepared, not shocked. Our minds were on straight, enabling us to make the best decisions for his health and well being. We knew that with this complex heterotaxy condition came a set of congential heart defects. We knew the set of congenital heart defects he may be born with: double outlet right ventricle, unbalanced atrioventricular canal, pulmonary valve atresia, situs inversus with levocardia. We knew that he would need open heart surgery in the first week of life to have a modified Blalock-Taussig shunt put in. We knew that it would be likely that he’d need a minimum of 3 heart surgeries to maintain his life, including the Glenn and Fontan; heart transplant will eventually be part of the plan when his own heart could no longer work for him. We knew that his complex heterotaxy would mean his stomach was on the left side as was his heart. He had the potential of having malrotated intestines (which was confirmed through a GI exam at CHLA after his first heart surgery, and has been “corrected” with the LADDS procedure). He would be asplenic (no spleen).

For the past year and a half, we have learned so much about Justin’s condition. In addition to those stated above, Justin’s general surgeon, Dr. James Stein, also found that he had a preduodenal portal vein and an annular pancreas. His liver is larger than normal. We also learned that Justin’s asplenic condition would have him with double AV (atrialventricular) nodes, while a normal person would have one. Having 2 AV nodes would lessen his chances of getting an artificial pacemaker put in because when one node doesn’t work properly, the other would kick in–not in Justin’s case. He developed arrhythmia sometime between his first heart surgery and second. He ended up needing an artificial pacemaker put in during his Glenn procedure.

Loi and I have accumulated more medical jargon than we had bargained for. We’ve developed a greater compassion for children and the disabled. We’ve learned of the various heart foundations and where to donate to. We’ve experienced the hospital life. We’ve met other heart families and joined in their journeys. We’re learning about the “system” and how to get it working for us in getting therapy and aid for Justin.

We’re moving along, getting through day to day, changing, trying to make a difference, to spread awareness…Yet, I realize, we’re still where we started from…clueless. Chloe’s blog reminded me that there is so little known about the heterotaxy condition. I’ve realized this, but have somehow placed it secondary to Justin’s heart defects, as those raised an immediate threat to his life; without proper function of his heart, he’d cease to exist.

But, this heterotaxy condition is no less important. It is the very basis of his heart condition. Somewhere during the embryonic development, something went horribly awry (but what?) that caused the rest of his development to mess up. In Chloe’s case, there may be a genetic link. In Justin’s case, we’re still not quite sure. We’ve been told that it’s not genetic. We’ve had genetic testing. But, I’m considering more.

Whenever I mention heterotaxy to the doctors, I’m made to feel like it’s not a big deal, that I should just concentrate on his heart. But, with heterotaxy, there are a group of health problems that I should be aware of depending on whether he’s situs inversus or situs ambiguous. Knowing which category he falls  in would better direct me to find answers.  According to my readings, Justin’s heart anomalies correspond with the situs ambiguous group.

Because Justin’s condition is so complex, I will be contacting a specialist in Immunology, Infectious Diseases, and possibly Pulmonary to obtain a better understanding of Justin’s condition and how to keep him healthy. While I agree that Justin’s heart is priority, I also believe that I need to know as much as I can about how the rest of his body works. How the rest of his body works affects how his heart works and vice versa. I need to know if his colds are normal and with a child with his set of anomalies, what is normal, for him?

His birthday party is approaching. I was looking forward to this lull in the year prior to his Fontan. Yet, I know, in this life with Justin, there is no such thing as a lull. I’m forging forward to find some answers for my baby boy. 

This entry was posted on Saturday, July 5th, 2008 at 3:08 am and is filed under Heterotaxy, Justin. You can follow any responses to this entry through the RSS 2.0 feed. You can leave a response, or trackback from your own site.