The Waiting Game

After June was pulled off the ventilator, we were able to cuddle with her (well, as much as you can with all the tubes, cords and drains June had attached to her). Then the waiting game began...

Each esophagram (she had five on July 31, August 3, 13, 20 and 26) showed that June still had a leak at the repair site of her TEF, which meant that she could not feed. After a couple esophagrams, the doctors thought that the JP drain (which was pulling all of the fluid leaking from around the repair site out of her body) might be too close to the repair to actually let it heal.  Surgery reopened part of her incision site to move the drain away from the repair. The good news is that within a week June's leak became minimal.

During this time, June encountered a few complications. Her trachea was weak and breathing was labored because of her VSD. There was a lot of pressure on her lungs, and she had a few spells where she quit breathing or couldn't get enough oxygen to her lungs. She also had lots of secretions that were hard for her to swallow because of the narrowing in her esophagus. The doctors felt that it was best for her to lay on her stomach so that her trachea would stop collapsing.

By the fourth esophagram (August 20) the doctors felt that the leak was restricted enough to start feeding. We were very excited about her finally getting some milk!

Unfortunately, with hours of them starting to feed June, all the milk was coming back up - both out of her drainage tubes and her mouth. The explanation for the milk coming out of her drainage tubes was that June had developed a chylothorax. It is a rare condition but commonly develops in babies with TEF. It results from either disruption (surgery) or obstruction of the thoracic duct. I'll try my best to explain it in layman's terms (this should take you back to freshman biology):

Your arteries and veins carry blood to and from your heart, lungs and tissue with "good" and "bad" blood. The third system involved is your lymphatic system, which takes the used fats from the tissue back to the circulatory system. The doctors thought June's system was either disrupted or not fully formed because those fats were leaking out into the chest cavity instead, which was part of the reason she was having trouble breathing. They switched June to a formal designed for babies with chylothorax, but again, everything that was fed to her leaked right back out.

If the lymphatic system was disrupted from surgery, it would take some time for her to heal (meaning more time without food). The other possibility was that her lymphatic system hadn't formed correctly. If this was the case, we would need to wait at least six weeks for the system to grow a new route. For the time being, feeding would stop and be tested later to see if her chylothorax had resolved itself or needed more time. Feeding is one of the keys for June to heal and go home, so this was disappointing.

The other concern was that June was refluxing the milk and formula too. With the last esophagram (August 26), the doctors found that there was no longer a leak; however, they noticed esophageal dysmotility (meaning the way her esophagus pushed food to her stomach was irregular). There is talk about dilating her esophagus to help, but that will be several weeks down the road so that the repair site isn't reopened.

During the last esophagram is when the doctors also noted that there was blockage from her stomach to intestine, a duodenum atresia (the A in VACTRL association that I wrote about in the previous post). At this point, the doctors felt the chylothorax was a complication of the previous surgery, and we would not have to wait for her lymphatic system to regrow. They now had a definite explanation as to why milk and formula was refluxing out her drainage sites. Chylothorax is a complication that continues to be monitored, but, at this point, one hurdle we hope June is over.

June is the first baby the doctors had seen with an atresia at both the top and bottom of the GI track. At the end of August, she was headed back into surgery to repair the blockage, and hopefully, move forward.