Over the weekend, Tiernan began to struggle more and more with his eating. By early Sunday morning he was unable to maintain a latch for more than a few seconds. Around 7 am on Sunday August 30th, his crying had changed to a very high pitched squeal and his color was becoming more and more gray. Jason and I packed up both boys and rushed to our local ER. An x-ray showed his heart was quite enlarged and his lungs were wet. They also could not get a pulse in his lower limbs. They suspected a congenital heart defect (CHD) and transported him to Mary Bridge Children's Hospital in Tacoma.
Following an echocardiogram, he was diagnosed with a severe and complex combination of defects and complications:
- Complete Atrioventricular Canal (hole through all 4 chambers)
- Coarctation of the Aorta (CoA-extremely narrowed aortic arch)
- Mildly Hypoplastic Left Ventricle (too small)
- Hypoplastic Aorta (too small)
- Mitral Valve Regurgitation (allows blood to leak back through in the wrong direction)
- Congestive heart failure (CHF) due to blood going into his lungs at a rate of 5:1 over the rest of his body since it could not get through the CoA.
Because Dr. Woods, the only pediatric cardio-thoracic surgeon at Mary Bridge was going on vacation, we were transported up to Seattle Childrens Hospital where Dr. Cohen and Dr. Permut reconstructed his CoA and put a band on his pulmonary artery to restrict the blood flow to his lungs and encourage it to go through his newly enlarged aorta.
Tiernan was discharged from SC 2 weeks later with a nasogastric (NG) feeding tube. He learned to eat on his own after a few weeks though so that was that. Or so we thought.
The day after Thanksgiving, T was once again showing work of breathing. Our pediatrician sent us over to Mary Bridge to have him examined. They admitted him when his x-ray showed his lungs were a little wet again. They mistook this for a mild case of pneumonia and treated him accordingly. During this time he was also showing signs of reflux. He was given Zantac but it continued. After about 10 days he was still not improving and was, in fact, getting worse. He was admitted to the PICU and finally diagnosed with CHF once again. A heart catheterization showed his CoA had returned and so now his heart was struggling to pump against a double obstruction and had, in the process become very thick and hypertrophic. It became clear he needed to have his second surgery, the DKS and Glenn. Dr. Woods and Dr. Welke performed this surgery on Christmas Eve 2009. It was a difficult recovery, but not as bad as expected. Sometime in early January it was decided he needed to have a Gastro-Jejunal (GJ) tube surgically placed in his stomach. He was severely underweight and any reflux or aspiration could jeopardize the surgical reconstruction of his heart.
Tiernan was discharged with his GJ tube on continuous 24 hour drip feeds on January 28th, 2010. From the start he did not deal well with the GJ. He vomited upwards of 30+ times a day and gagged and wretched constantly. It was pulled out accidentally on several occasions, despite our focused attention to being extremely careful. He gained weight though and by his birthday we got him down to a much shorter time on the feeds and were much more successful with oral feeds. His "reflux" continued until we began feeding him into his Gastric port (directly into his tummy and not his intestine as the GJ does). November of 2010 we switched completely over to straight G tube feeds and have seen a vast improvement in his reflux and he has a better appetite.
In May of 2010 we put an end to his night feeds and made a partial switch to the blended diet. When I didn't feed him food I had blended myself we fed him a formula made from real food, rather than chemicals. We only did this for a short time because within a few weeks he began to drink whole milk. That was the extra caloric intake needed to try a wean. We began a full wean in June and, although it was a struggle, he began to eat an average of 1000 calories a day! All by himself.
By August through discussions with his GI doctor, Cardiologist and dietitian, we decided it was time to remove the tube altogether. He had significant chronic tissue breakdown around the tube and due to the risk of infection, keeping an unused tube was just not a good option. So on August 24, 2011, we pulled out the tube and waited for the stoma to close. After a month it still had not done so. On September 23, 2011, Tiernan had a surgery to close the site for good.
