And Now My First Serious Post

This week is Congenital Heart Defect Awareness Week. CHDs entered my life about 6 years ago when a good friend's daughter was born a micro-preemie with an assortment of heart defects that made up Tetralogy of Fallot. She had a long road ahead of her and overcame numerous health issues and surgeries. Unfortunately, with CHDs you're never really "cured" and nobody really knows what the future holds for her and how many more trials she'll have to face.

CHDs hit even closer to home for me Dec. 21st 2007. My nephew was born at home on Dec. 6th and he seemed a healthy baby, albeit a very sleepy baby who had trouble nursing and would only sleep in 45 minute stretches round the clock. His first pediatrician appointment at 2 weeks old turned our lives upside down. The doctor heard a heart murmur and was concerned enough to send them directly to the hospital for an ultrasound. That evening, after a specialist reviewed the scans my sister received a phone call urging her to pack for 2 weeks and head straight to Texas Children's Hospital 2 hours away. The preliminary diagnosis was transposition of the great arteries and pulmonary atresia.

Knowing very little about the conditions I hopped online to learn what I could. The TGA seemed fairly easy to deal with, all things considered, but the information I read on PA wasn't comforting at all. My nephew was a sensation at the hospital as it was unheard of for PA babies to make it to 2 weeks without intervention and doctors and nurses kept dropping into the room to look him over. More thorough tests were run and his diagnosis was changed, thankfully, to tricuspid atresia, transposition of the great arteries, pulmonary stenosis, ventricular septal defect and atrial septal defect. It sounds silly to say "thankfully" the diagnosis was changed and then post a laundry list of defects but I have my reasons.

So it was decided that he was "balanced," meaning a good ratio of oxygenated to unoxygenated blood was being pumped through his body and he could bypass the first of 3 surgeries that is standard procedure for correcting his particular defects. That surgery is usually done at birth. After a week in the hospital he was able to come home with special equipment to monitor his oxygen levels and his family settled into their new routine of dealing with a special needs baby. Gone were the days of packing up and going shopping or out to eat or even having guests over as he could not afford to get even a little cold. My sister had to quit her job as he could never go into daycare. She also had to begin pumping breastmilk as nursing from the breast was too taxing for him and he would get sweaty and exhausted from the effort.

Before we knew it the time for his first surgery had come. They generally wait as long as possible as the bigger and stronger the babies are the better the odds are of a good outcome. His condition began to deteriorate sooner than we had hoped and a week before he turned 5 months old he had his first open heart surgery. It's called the Hemi-Fontan and re-routes the blood flow from the upper body directly to the lungs which reduces the amount of work the right ventricle has to do. So now blood goes into the lungs to be oxygenated, back into the heart and then pumped to the rest of the body.

The surgery went extremely well and as hard as it was to prepare for this and to see the aftermath I can't imagine how we would have made it through if there had been complications. He spent a week in the hospital afterwards and another week at The Ronald McDonald House nearby. After 2 weeks he was home again and began his new life. Of course, we still had to be cautious and life certainly didn't go back to "normal" but he was a whole new baby. He began to eat better, sleep better, catch up on his milestones that were delayed because he had been simply trying to survive. We were all astounded at how much better his color was and horrified that we didn't notice how bad it was until we saw what he was supposed to look like. His older sister could finally start to really play with him as he began to have longer periods where he was awake.

He is now 14 months old and is the handsomest, happiest, best natured baby. He is still carefully monitored and his body will let us know when his next and, hopefully final, surgery will need to be performed. They like to get to 4 years old, ideally, for this one although if it becomes necessary before then, that's what will have to be. It's called the Fontan and will re-route the blood from his lower body directly to the lungs to further reduce the burden on his heart. From that point on his heart will only be responsible for pumping blood to the body and not to the lungs. I am dreading the surgery because it's surgery, and open heart at that, but I'm also excited in a way to see what this means for him. He changed SO much after being halfway "repaired" that it seems like the sky's the limit for him after he's fully "repaired."

There isn't much data on there about long-term prognosis for babies with his defect(s) as the procedure used to correct the defects is new. New as in only a few decades old. So there aren't many older kids and adults out there who had these defects and this repair to let us know what's in store for him. However, my sister has become acquainted with a wonderful girl named Lauren who is 21, has TA and has never had a transplant. She's still living with her heart that was repaired using the Hemi-Fontan and Fontan and gives my sister a lot of hope that her son will have just as positive an outcome as Lauren has. We're also supremely thankful to live so near to Texas Children's Hospital where there are so many world-renowned specialists who are on the cutting edge of research and technology and innovation. We know he's in the best hands he could be in.

Lauren created this video to raise awareness of CHDs and the impact they have on our lives. My nephew is featured briefly at 1:53 and at 3:22. You can also go here and view my nephew's CarePage. My sister is also raising money for CHD awareness this month and if you would like to donate the information as to how to do that is also in his latest CarePage entry.